Pediatric Hematology Very important is the history and physical examination.

After that you

(Dra. Fajardo Online class, 2020) already have 2/3 of your diagnosis. The laboratory test will just confirm your
diagnosis
1. Anemia
2. Hemolytic Anemia Pertinent data in the history
3. Bleeding Disorders
Onset of anemia
4. Clinical Assessment of the Child with Suspected Cancer
5. Hematologic Aspect of Dengue Hemorrhagic Fever Family history
6. Appropriate Use of Blood Diet
7. Neonatal Erythropoiesis Ethnicity
8. Hemorrhagic Disease of the Newborn Gender
9. Neonatal Jaundice Age
ANEMIA Drug intake
Pallor = anemia Menstrual history
Neonatal history
Factors affecting Skin Color
Infection
Hemoglobin concentration
Diarrhea
State of constriction
Very important is the onset of anemia; is it already chronic or is it only first
Dilatation of peripheral vessels time. This can help you to narrow down your differential diagnosis. If it is
Pigmentation and subcutaneous fluid already chronic, you can think of hereditary anemia or hereditary form of
Not only secondary of hemoglobin concentration. In other words, in cold bleeding disorder. Family history of hemolytic anemia (G6PD), bleeding
weather patients may look pale. For those who are edematous anasarcous, disorders (von willebrand disease or hemophilia). Diet is also important
will also look pale. because this will rule out nutritional cause of anemia like iron deficiency
anemia or megaloblastic anemia. Age is important because different age
ANEMIA
group has different causes of anemia. Drug intake, particularly aspirin, will
reduction in red cell mass inhibit platelet aggregation
reduction in blood hemoglobin concentration
Physical Findings Helpful in the Diagnosis
Function of the red cell
Pallor and jaundice
to deliver and release adequate quantities of oxygen to the
Organomegaly and lymphadenopathy
tissues to meet their metabolic demands
Oral lesions
Criteria for identifying children with low Hgb and Hct values` Tachycardia, tachypnea and cardiac murmurs
HEMOGLOBIN HEMATOCRIT Bloody urine and stools
AGE MEAN LOWER MEAN LOWER
Stunted growth
LIMIT LIMIT
0.5 – 1.9 12.5 11.0 37 33 Skull structural defects
2–4 12.5 11.0 38 34 Very important is when you are dealing with pallor and jaundice. In jaundice,
5-7 13 11.0 39 35 this is unconjugated type of bilirubinemia (B1). This is encountered in
hemolytic anemia. Because of the hemolysis you develop pallor and
8 – 11 13.5 12.0 40 36
jaundice. When you develop organomegaly and lymphadenopathy, you want
12 – 14
to rule out infection, leukemia and iron deficiency anemia. When patient
Female 13.5 12.0 41 36
become tachycardic and tachypnic this is because of very low hemoglobin
Male 14.0 12.0 41 36 and hematocrit level. The tendency of your heart is to compensate.
15 – 17 Difference in difficulty in breathing and tachypnea. In difficulty in breathing
Female 14.0 12.0 41 36 you have different retraction (supraclavicular, intercostal and subcostal). As
Male 15.0 13.0 46 38 doctors, we must memorize the normal value of children in each age group.
18-49 In cardiac murmur, there could be high output failure already because of
Female 14.0 12.0 42 37 severe anemia. The problem when you have red blood cells in the urine you
Male 16.0 14.0 47 40 have bleeding, but when you have hemoglobin in the urine you have
*Hemoglobin and hematocrit in newborn is much higher hemolysis. Bleeding is different from hemolysis but both of them can
produce anemia. When you have fresh blood from the stool (hematochezia),
Hemoglobin Level and Symptoms the bleeding is lower GI (below the ligament of treitz). When you have black
Hgb (g/dl) symptoms stool (melena), the bleeding is upper GI. When the patient has stunted
9-11 little to no dysfunction growth, the patient experiencing chronic problem already. Skull structural
5-7 exertion dyspnea defects can also be seen like lytic lesions, frontal bossing, this is secondary to
6.0 some weakness extramedullary hematopoiesis. This is usually seen in patients with
3.0 dyspnea at rest thalassemia or congenital spherocytosis, with chronic hemolysis.
2-2.5 cardiac failure Complete Blood Count
Depending on the level of hemoglobin, you will have the following level of hgb/hct
symptoms. If it is severe, patients will develop tachycardia or even cardiac
differential count
failure. They can even develop hymnic murmur
platelet count
Evaluation of Anemic patient You look at the differential count because viral and bacterial infection can
history produce pallor and anemia. There will be ineffective erythropoiesis and
physical examination decrease utilization of iron, and decrease erythropoietin or increase plasma
level of erythropoietin. Part of the complete complete blood count is the
laboratory test reticulocyte distribution width (RDW), this will tell you the difference or the
o cbc variety of sizes of the red blood cells. In iron deficiency you have high RDW.
o rbc indices In thalassemia, you have normal RDW. Both IDA and thalassemia has
o reticulocyte count microcytic type of anemia. You have to differentiate them base on clinical
o peripheral smear findings and the RDW
Red Blood Cell Indices II. Based on Size
A. Microcytic anemias
MCV= volume of prc (hct) x 1000
1. iron deficiency (chronic bld. loss, nutritional)
rbc count
2. chronic lead poisoning
normal = 80 – 100 fl
This well tell you the size of the red blood cell. The size must be the same as
3. thalassemia syndromes
the nucleus of the lymphocyte (7 – 10 um). Morphology or size is important 4. sideroblastic anemias
because it will narrow down your differentials. Normocytic type anemia are 5. chronic inflammation
all the acute anemia. In microcytic type anemia are all the chronic anemia. In 6. congenital hemolytic anemias with unstable hgb
macrocytic type anemia, it can be seen in megaloblastic or pernicious B. Macrocytic anemias
anemia. Also in the newborn period until the second day of life, you can have 1. With megaloblastic bone marrow
macrocytic type of anemia. a) Vitamin b12 deficiency
MCH = ____Hgb____ b) Folic acid deficiency
rbc count c) Hereditary orotic aciduria
normal = 27-34 pg d) Thiamine responsive anemia
MCH will tell you the degree of hemoglobinization. Normally in the red blood 1. Without megalobastic bone marrow
cell, the central pallor will be occupied by the central quadrant. If it goes a) Aplastic anemia
beyond the central quadrant, you label that as mild, moderate or severe b) Diamond-blackfan syndrome
hypochromia. c) Hypothyroidism
MCHC = _____hgb_____ d) Liver disease
volume of prc e) Bone marrow infiltration
normal = 32-36 % f) Dyserythropoietic anemias
C. Normocytic anemias
Corrected RC or Reticulocyte Count Index 1. Congenital hemolytic anemias
_Actual Hct_ x observed RC (%) a. Hemoglobin mutants
DESIRED hct b. RC enzyme defects
normal = 1-1.5% c. Disorders of rc membrane
2. Acquired hemolytic anemias
Reticulocytosis a. Antibody mediated
Blood loss b. Microangiopathic hemolytic anemias
Hemolytic anemia c. Secondary to acute infections
After a therapeutic trial of iron 3. acute blood loss
Reticulocytopenia 4. splenic pooling
bone marrow failure 5. chronic renal disease
aplastic anemia
IRON DEFICIENCY ANEMIA
leukemia
When you are dealing with patients with anemia you always have to request Most common cause of anemia
for reticulocyte count together with your CBC. It is mandatory to request for Prevalence:
reticulocyte. Those beyond 1.5%, you label that as reticulocytosis. Condition o 6-12 mos –46 –53 %
with reticulocytosis, acute blood loss, acute hemolytic anemia and after o Pregnant and lactating mother
therapeutic trial with iron. Three days after the iron preparation, you will o 44 and 43%
have reticulocytosis. When you have reticulocytosis, the bone marrow is Common in ages 6-24 months
responding to the anemia. There is an unimpaired bone marrow response.
When you have less than 1%, reticulocytopenia, your bone marrow is not Etiology of iron deficiency
responding. You have bone marrow failure. Condition such as leukemia and A. Inadequate supply of iron
aplastic anemia. 1. Lack of iron stores at birth
o Low birth weight, preterm, twin or multiple births,
Peripheral Smear severe IDA in mother, fetal blood loss, bleeding
Hypochromia, microcytosis from the 1st few days of life
Anisopoikilocytosis – non-specific sign of anemia 2. Inadequate intake-deficient dietary iron
Target cells – seen in chronic hemolysis B. Impaired absorption – absorb iron in ileum
Thrombocytosis 1. chronic or recurrent diarrhea
Thrombocytopenia 2. malabsorption syndrome
In counting platelet, one platelet is equal to 10,000. You must count in 5 – 10 3. GI abnormalities
high power fields. Signs of hemolysis like burr cells C. Excessive demands from iron for growth in preterm, low
birth weight infants, adolescent and pregnancy
Classification of Anemia D. Blood Loss
1. Acute or chronic hemorrhage
I. Physiologic
2. Parasitic infection – anal pruritus in night to know.
A. Disorder of RC production
1. Marrow failure Factors that Modify Iron Absorption
2. Ompaired erythropoietin production o physical state (bio availailty)
B. Disorder of erythroid maturation and ineffective  heme > fe2+ > fe3+ (give the ferrous sulfate)
erythropoiesis o Inhibitors
C. Hemolytic anemias  Iron Overload
 o Competitors Treatment of iron deficiency anemia
 Cobalt, Lead Determine the cause
o Facilitators Correct the abnormality
 Abcorbate , Citrate, Amino Acids
I. Oral Iron
BURDEN OF IDA – WORLDWIDE Oral Ferrous Sulfate: 6 Mg/kg/day (6 - 8 Wks After Normal
Hgb value Is attained) – meaning you have already attained
STAGES OF IRON DEFICIENCY
the normal value, you still have to give iron for 6 – 8 weeks
I. Prelatent iron deficiency
Older Children: 100 200 mg/day of elemental iron
depleted stores without a change in hematocrit or serum
When you are dealing with IDA, you give ferrous sulfate. But when you are
iron levels
dealing with hemolytic process, you give folic acid.
rarely detected
Serum ferritin is already low. Rarely detected because the patient is Poor Response To Oral Iron
still pinkish. No pallor yet Incorrect Diagnosis
Non Compliance
II. latent
Ongoing Blood Loss
Decreased serum iron level
Insufficient duration of therapy
TIBC increases without a changes in Hct
high gastric pH
Decreased transfusion saturation
inhibitors of iron absorption and utilization
III. Frank iron deficiency You will know if the patient is complying if the color of the feces is black.
Associated with erythrocyte microcytosis and hypochromia When you have patient that have immune thrombocytic purpura or
menorrhagia. No matter how much you give iron, this patient will still
Your patient will already look pale. You can have iron deficiency already
develop IDA because of chronic blood loss. The inhibitors of iron absorption
even without anemia. If you are presented with patient with risk are infection or malignancy. If there is an infection, you must control the
factors, you already have to give iron supplementation to prevent IDA. infection first before correcting IDA. Those patient who undergoes dialysis or
chronic renal failure, we usually give blood transfusion or erythropoietin
Effects of Iron Deficiency Anemia
subcutaneously.
Reactive thrombocytosis
Impairs tissue Oxygen, weakness, fatigue, palpitation and Inhibitors of Iron Absorption
lightheadedness lead intoxication
Upon correction of your anemia, your platelet level will return to aluminum intoxication
normal. chronic inflammation
neoplasm
I. Growth and Development
Impairs neurologic functions (behavioral abnormalities, II. Blood Transfusion Indication
motor incoordination and seizure) severe anemia
II. Epithelial changes debilitated from infection
Angular stomatitis signs of cardiac decompensation
You give your packed red blood cell transfusion
Glossitis
Spooning of the fingernails Effect On The Fetus Of Maternal Iron Deficiency
You usually see this in patient with IDA already especially those with “Maternal iron status determine the iron stores of the neonate”
malnutrition If your mother is iron deficient, so the iron the iron stores from the baby will
be depleted earlier than 6 months. Usually it will decline at 4 months of age.
III. Miscellaneous Early for those who are premature.
pica (ice and soil)
massive hepatosplenomegaly ANEMIA OF CHRONIC INFLAMMATION
poor wound healing ineffective iron utilization
bleeding diathesis (epistaxis or hematoma) low plasma levels
zinc deficiency Consequences of Iron Overload
lead intoxication 1.heart
pseudotumor cerebri (headache or vomiting) 2.liver
3.endocrine
Diagnosis When treating IDA, you can only give iron for 3-4 or 2-3 months. You only
in infants: high index of suspicion have to give supplement after you attained normal Hgb value for age and for
1.prematurity another 6 – 8 weeks
2.blood loss
3.fed exclusively on milk ANEMIA DURING THE NEONATAL PERIOD
4.chronic diarrhea hemorrhage: acute or chronic
hemolysis: congenital or acquired (isoimmunization)
Prevention failure of RC production
administration of iron to expectant mother ANEMIA OF PREMATURITY
early introduction of solid food Due to:
supplemental iron: 10 –15 mg/kg (elemental iron) for 6-8 o preterm infants deprived of 3rd trimester
weeks hematopoiesis and iron transport
If you have the risk factors, you have to give supplemental irons already o decreased RC production (preterm infants have low
to your patient epc levels with a nadir between 7 and 50 days)
 o shorter RC life span HEMOLYTIC ANEMIA
o increased blood volume with growth results from an increased rate of red cell destruction
o marked blood sampling in relation to their weight
unimpaired bone marrow response
You don’t have to transfuse blood. Whenever you are extracting blood,
*When you do your reticulocyte index, you will have reticulocytosis
when you reach 10% of blood volume, you must transfuse your patient
already. Site of Hemolysis
normocytic, normochromic intravascular – predominantly within the circulation
reduced bm erythropoietin activity extravascular – within tissue macrophages
low reticulocyte count Manifestation of intravascular hemolysis, you hemoglobinuria and
hemoglobinemia. One good example if G6PD. In the manifestation of
compounded by folic acid, vitamin e and iron deficiency and
extravascular hemolysis, you have organomegaly and more pronounced
frequent blood sampling jaundice. One example is collagen diseases
physiologic anemia
Inheritted Hemolytic Disease
PHYSIOLOGIC ANEMIA A. Defects in the erythrocyte membrane
in utero –fetal 02saturation is 70% (hypoxic levels) 1. Hereditary spherocytosis
-stimulates erythropoietin 2. Hereditary elliptocytosis
-produces reticulocytosis (3-7%) B. Deficiency of erythrocyte glycolytic enzymes
-increases rc production 1. Pyruvate kinase deficiency
-high hgbat birth C. Abnormalities of erythrocyte nucleotide metabolism
after birth –02saturation is 95% 1. Adenylate kinase deficiency
-erythropoietin is undetectable D. Deficiencies of enzymes involved in the pentose
-rc production by day 7 is 10% phosphate pathway and in the glutathione metabolism
-hgb level falls 1. Glucose -6-phosphate dehydrogenase ( G6PD)
-nadir at 8 -12 wks 2. Glutathione reductase
-02delivery is impaired E. Defects in globinstructure and synthesis
-erythropoietin stimulated 1. Unstable HgbDisease
-rc production increases 2. Sickle cell anemia
Clinical and Laboratory Evaluation of Anemia In The New Born 3. Hemoglobinopathies
History 4. Thalassemiamajor
o Obstetrical History 5. HgbH disease
o Family History Acquired Hemolytic Disease
Physical Examination A. Immunohemolytic anemias
Laboratory Test 1. Transfusion of incompatible blood
o CBC 2. Hemolytic disease of the newborn
o RC 3. Autoimmune hemolytic anemia due to warm reactive
o Blood Smear antibodies
o Antiglobulin Test  virus and mycoplasmainfection
o Blood Type of Baby And Mother  lymphosarcoma, chronic lymphocytic leukemia
o Bilirubin Level  other malignant disease
o Studies of Neonatal Infection  immune-deficiency states
o Ultrasound of The Head  Systemic lupus erythematosusand other autoimmune
o RC Enzyme Assays (If Clinically Indicated) disorders
o Bone Marrow  drug-induced
Include the family history hemolytic disorders. Patients with G6PD can 4. Autoimmune hemolytic anemia due to coldreactive
develop hemolysis at 2nd or 3rd day of life. The antiglobulin test is important
antibodies
to know if there is an immunologic cause of hemolysis. You have to take note
the blood type of the mother and the baby because you can have ABO or Rh B. Traumatic and microangiographic hemolytic anemias
incompatibility. Infection can also produce anemia. Ultrasound of the head 1. Prosthetic valves and other cardiac abnormalities
to see if there is intracranial bleeding from hemorrhagic disease. Who are at 2. Hemolytic – uremic syndrome
risk from hemorrhagic disease of the newborn? Those patients who are born 3. Thrombotic thrombocytopenic purpura
at home that are not given vitamin K. You can also have bone marrow 4. Disseminated intravascularcoagulation
examination to know if there is a neonatal leukemia. It can happen at birth 5. Associated with immunologic phenomena (graft rejection)
up to 6 weeks of age. C. Infectious agents
When To Suspect Malignancy In Patients With Anemia? 1. Protozoa: malaria, toxoplasmosis
Affecting > 2 cell lineage 2. Bacteria: clostridial infection, cholera, typhoid fever and
Bone Marrow involvement others
Immunologic Disorder D. Chemicals, drugs and venoms
Sequestration of cells 1. associated with hemodialysisand uremia
2. venoms
E. Physical agents
1. Thermal injury
F. Hypophosphatemia
G. Paroxysmal nocturnal hemoglobinuria
H. Spur –cell anemia in liver disease
I. Vitamin E deficiency in newborns
 When we say hemostasis, it means maintaining the blood in its liquid form. It
Clinical Manifestaion is the balance between formation of fibrin clot and lysis of this clot
(fibrinolysis). Any problem in the hemostasis, from vasoconstriction up to the
Chronic Congenital Hemolytic Anemia lysis of clot, there will be bleeding or thrombosis
Degree of anemia
Components of Hemostasis
Jaundice
Vascular Phase
Crises
Platelet Phase
Splenomegaly
Plasma Phase
Cholelithiasis
Leg ulcers
Classification of Disorders of Hemostasis:
Skeletal abnormalities (tower-shaped and skull thickening
and striation of the frontal and parietal bones A. Purpuraswithoutmajorcoagulationfactors
1. With low platelet count
Acquired Hemolytic Anemia a) Acquired thrombocytopenia (ITP)
pains in the back , abdomen or limbs b) Hereditary thrombocytopenia (Fanconi anemia)
headache , malaise , vomiting , chills andfever c) Neonatal(Isoimmunepurpura)
prostration and shock 2. With normal platelet count
pallor , jaundice ,tachycardia a) Qualitative
Acquired thrombocytopathies (Aspirin)
Laboratory Signs of Accelerated red cell Destruction Heritable thrombocytopathies (Glanzmann
Decreased erythrocyte life span Dse)
Increased catabolism of heme b) Vascular Disorders
Increased serum lactate dehydrogenase activity 1.Acquired (HSP)
Absence of serum haptoglobin 2.Heritable (Ehlers-Danlos Syndrome)
Reduced glycosylated hemoglobin B. Major Coagulation Disorders
Signs of intravascular hemolysis 1. Decreased Activity of One or More Coagulation Factors
a) Heritable (Hemophilia)
Immune Hemolytic Anemias b) Acquired (DIC)
Alloimmune hemolytic anemias c) Neonatal (Hemorrhagic Dse of the Newborn)
o Hemolytic transfusion reactions 2. With Normal or Increased Coagulation Factors
o Hemolytic disease of the newborn a) Heritable thrombotic
Autoimmune hemolytic anemia b) Disorders (Deficiency of Antithrombin III)
o Warm antibody type c) Acquired (Oral Contraceptive Drugs)
o Cold antibody type
COAGULATION FACTORS
Drug-induced
o Penicillin type I Fibrinogen
*ABO and Rh incompatibility are alloimmune II Prothrombin
III Tissue Thromboplastin
BLEEDING DISORDERS IV Calcium
V Proaccelerin, Labile Factor
What happens after the injury to the blood vessel? Your vessel will VI
aggregate, after that you will form your platelet adhesion and aggregation,
VII Proconvertin, Serum Prothrombin Conversion
and will form unstable platelet clot. After that you activate the coagulation
pathway.
Accelerator (Spca), Stable Factor
VIII Antihemophilic Globulin(Ahg), Antihemophilic
You have your intrinsic pathway, involved here are factors VIII, IX, XI and XIII.
Factor(AHF)
Now how will you know if there is a problem in your intrinsic pathway. You
look at your activated partial thromboplastin time (aPTT). aPTT is prolonged IX Christmas Factor
if it is more than 20 seconds that of the control. It is very sensitive to X Stuart-Power Factor
deficiency of factor 8. For the extrinsic pathway, the factor involved are the XI Plasma Thromboplastin Antecedent (PTA)
vitamin K dependent factors; II, V, VII and IX. Prothrombin Time (PT) is very XII Hageman Facto
sensitive to the factor VII. When do you say when the PT is prolonged? You XII Fibrin-Stabilizing Factor (FSF)
look at the INR (International Normalize Ratio). The normal INR is 1 – 1.5%. If
it goes beyond 1.5%, you label that as prolonged. Both PT and PTT, you don’t HEMOSTATIC MECHANISM
look at the %activity. Primary:
Now we go now to common pathway, with factor X. Then to the final Vasoconstriction
product from fibrinogen, you form your fibrin thrombus, which is your stable Formation of Platelet
fibrin clot. If the body needs to lyse this clot, you have your natural
Plug (Adhesion & Aggregation)
anticoagulant, such as anti-thrombin III, protein C and protein S, to lyse the
clot to prevent hemostasis
Secondary:
Intrinsic & Extrinsic Pathway –Formation of Fibrin Thrombus
Hemostasis Vascular Phase
The sum total of those specialized functions within the Scurvy
circulating blood & its blood vessels that are designed to stop Vasculitis
hemorrhage Henoch Schonlein Purpura
Platelet Phase or PTT. You don’t request for all bleeding parameters. For examples, dengue
Thrombocytopenia fever, you don’t need to request bleeding time anymore.
Destruction
o Acquired Primary vs Secondary Hemostatic Defects: Clinical Manifestations
 ITP, Infection, Renal failure Characteristics Primary Secondary
o Hereditary Onset of bleeding Immediate Delayed
 Wiskott Aldrich Syndrome Bleeding from:
 Fanconi Syndrome Abrasions Yes No
Decreased Production Lacerations Yes No
o Aplastic Anemia Surgery Yes No
o Leukemia Type of bleeding
Thrombasthenia Petechiae Yes No
o Acquired: Ecchymoses Small, multiple Large, spreading
 Infection, Renal Failure Hematoma sometimes Yes
o Hereditary Hemarthrosis No Yes
 Glanzmann Thrombasthenia
 Bernard Soullier Syndrome IMMUNE THROMBOCYTOPENIC PURPURA
Plasma Phase acquired hemorrhagic disease
Anti-Prothrombin Complex Deficiency characterized by thrombocytopenia,
Hemophilia purpura, absence of other causes of
DIC thrombocytopenia
Liver Disease no organomegaly
When you are confronted with a patient with bleeding problems, you have abundant megakaryocytesin the
to think whether you are dealing with a vascular, platelet or plasma phase. In BM aspirate
the vascular phase, the problem is the fragility of the blood vessels. This is Acute >2X Chronic
the reason why you have positive tourniquet test in dengue fever. In dengue Acute: Peak Incidence is 2 -6 yrs
fever, it is not only thrombocytopenia which is the cause of bleeding, you
Chronic:PeakIncidence is 10 -30 yr
also have vasculopathy. For the platelet phase, the test for it is bleeding
time. There is prolonged bleeding time in thrombocytopenia and Etiology: Autoimmune Disorder
thrombastenia. In thrombastenia, you have normal count of platelet but it Females >3x Males
not functional. When do you request for bleeding time? You don’t need to
request bleeding time in patient with thrombocytopenia because it is already Classification of Immune Thrombocytopenic Purpura
prolonged. You will only request for bleeding time when there is platelet A. Acute ITP: complete &sustained remission within 6 months from
dysfunction (thrombastenia). onset
Before you can diagnose Immune Thrombocytic Purpura, you have to rule 1. single episode, variable severity
out other causes of thrombocytopenia. You also have thrombocytopenia in 2. recurrent, biphasic
infection, renal failure, in cardiac diseases, in collagen disease and those with B. Chronic ITP: sustained remission not achieved within 6 months
malignancy. from onset
In the APCD, PT and aPTT are both prolonged. You don’t have to request for 1. continuous episode, variable severity
bleeding time. Because the problem in plasma phase are the coagulation 2.recurrent, multiphasic
factors. Hemophila A, B and C have deficiency in factors VIII, IX and XI,
HEMORRHAGIC DISEASE OF THE NEWBORN
respectively. Only aPTT will be prolonged, not PT. In DIC, all the phases are
affected. In dengue fever, all the phases are affected. The expected result of Self-limited disease
aPTT and PT in liver disease are both prolonged. 1st-5thday of life
deficiency of vit K dependent clotting
Prevention of Formation of Platelet & Fibrin Clot factors II, VII, IX & X
Prostacyclin less common in full term infants
o Vasodilatation bleeding is rarely massive
o Prevents platelet aggregation
prolonged pt,ptt
Receptors for thrombin which
treatment: vit K
o Neutralizes the clotting
o Activity: DISSEMINATED INTRAVASCULAR COAGULOPATHY
 Anticoagulant-Protein C Most common form of consumption coagulopathy in children
 Antithrombin3 Presents with fever from multiple sites
Diagnosis Bleeding from skin and mucous membranes, GU, GI, & CNS
history You treat the underlying cause. The patient who are more prone to
physical examination DIC are those with shock, infection, burned and stressed patients
laboratory tests
o CBC, APC Management for DIC
o bleeding time treatment of underlying disease
o prothrombin time
general supportive care
o partial thromboplastin time
blood transfusion: hypovolemia& shock
Very important is the history and PE. In history, you will ask history of
trauma (child abuse), you have to rule out child abuse. In medication Control of temperature abnormalities
(aspirin). Family history of bleeding disorder and blood cancer. Base from the Management of organ failures
phase that you suspected, you request for bleeding time, prothrombin time Specific measures to control DIC
 CLINICAL ASSESSMENT OF THE CHILD WITH SUSPECTED CANCER Thoracic mass
Bone and joint pain
Incidence Signs of increased intracranial pressure and neurologic
1% increase /year overall deficits
2% increase /year in brain tumors leading cause of disease – Ophthalmic manifestations
related mortality in children Abdominal mass
Annual incidence: 15/ 100,000 Bleeding
Cytopenias
Children at Risk
Genetic predisposition: Down Syndrome, Neurofibromatosis First is recurrent, unexplained and prolonged fever. Lymphadenopathy is
and Beckwith-Wiedemann Syndrome from malignancy if it is matted, non-movable, non-tender, generalized and
Preterm infants - Hepatoblastoma we you see it in supraclavicular area. In thoracic mass, initially it will present
Ebstein-Barr virus infection: B-cell lymphoma (BL), T-cell as consolidation, pneumonia or because of thymus. In bone and joint pain,
lymphoma, HL, HLH and Nasopharyngeal Ca initially they are seen by orthopedics or rheumatologist. In signs of increased
intracranial pressure and neurologic deficits, like recurrent headache in the
Hepatitis B and C – Hepatocellular Ca
morning relieve by vomiting. Neurologic deficits like paresis. Ophthalmic
Survivors of ALL –Brain tumors, AML manifestation like blurring of vision or nystagmus are presented in patients
Hodgkin Lymphoma – secondary AML, NHL with retinoblastoma. Bleeding can be presented by patients with leukemias.
And cytopenias, when there are two or more cell lineages that are affected,
Signs and Symptoms Of Childhood Cancer Mimicking Normal consider blood cancer
Childhood Disease
Generalized malaise, fever, adenopathy Unusual Presentation of Childhood Neuroblastoma
o Lymphoma, Leukemia, EWS, NBL Not related directly to tumor growth:
Head and Neck o chronic diarrhea, polymyoclonus
o Headache, N/V - Brain tumor Leukemia o cogwheel erythrocytes, failure to thrive
o Febrile seizure - Brain tumor o Cushing’s syndrome
o Earache and rhinitis and pharyngitis-STS Related directly to tumor growth:
o Epistaxis – Leukemia o Horner’s syndrome
o Adenopathy–NBL, Thyroid, STS, Lymphoma, o Superior vena cavalsyndrome
Leukemia o Hydrocephalus
Thorax o Meningealinvolvement
o Extrathoracic o Cavernous or lateral sinus involvement
 Soft tissue mass - STS, PNET o Blindness
 Bone mass –EWS, NBL o Subcutaneous nodules
o Intrathoracic o Leukemoidreaction
 Adenopathy-Lymphoma, leukemia o Myasthenia gravis
Abdomen Acute lymphoblastic leukemia:
o External: Soft tissue -STS, PNET o hypercalcemia, cyclic neutropenia eosinophilia,
o Internal: diarrhea, vomiting, organomegaly pulmonary nodules, lupus erythematosus,
 -NBL, Lymphoma, liver tumor leukemia rheumatoid arthritis, skin nodules, pericardial
Genitourinary effusion, aplasticanemia, vertebral collapse,
o Hematuria - Wilms‘ tumor, STS hypoglycemia, solid tumor
o Trouble voiding – Bladder STS Acute lymphoblastic leukemia:
o Vaginitis/Peritesticular mass-STS o hypercalcemia, cyclic neutropenia eosinophilia,
Musculoskeletal pulmonary nodules lupus erythematosus,
o Soft tissue mass-RMS, STS, PNET rheumatoid arthritis, skin nodules, pericardial
o Bony mass/ pain –Osteosarcoma, EWS effusion, aplasticanemia, vertebral collapse,
o NHL, NBL, Leukemia hypoglycemia, solid tumor
Acute myelogenousleukemia:
THE TIME TO DIAGNOSIS IS VERY CRUCIAL o Myelofibrosis, mediastinal mass, clitorism, ovarian
mass, pericarditis, chloroma, bone fractures
Delays Lymphoma:
Disease o Nephroticsyndrome, pruritus, acute dysautonomia,
Patient / Parent dermatomyositis
Health care related Germ cell tumor:
It could be the disease itself. For example, in Wilms’ Tumor, it will present o Parinaudsyndrome
only with unilateral flank mass. It will be accidentally palpated by the mother Thymoma:
during bathing. It could be secondary to the patient or the parent The parent
o Myasthenia gravis
could bring the baby first to the albularyo or manghihilot. It could be health
care related as well. Either they do not have access to the health care CNS tumor:
facilities or because of the first doctor. o Diencephalicsyndrome
Wilms’ tumor:
When to Suspect o Hypoglycemia, uterine mass
Fever o IVC thrombosis, anemia
Tumor / Swelling Liver Ca:
Lymphadenopathy o Erythrocytosis, thrombocytosis
 Ewing’s sarcoma: Hemostasis
o SVC syndrome, septic arthritis 1. Vascular
Rhabdoid tumor: 2. Platelet
o Pericardial effusion 3. Plasma
Rhabdomyosarcoma:
Bleeding Diathesis
o Pulmonary bronchial cyst, cardiac mass, leukemia 1. vasculopathy
Chronic myelogenousleukemia: 2. thrombocytopenia
o Priapism 3. platelet dysfunction
Predominant Pediatric Malignant Tumors by Age and site 4. coagulopathy
Leukemia: Vasculopathy
o < 1 yr: Congenital leukemia, AML, CML Direct effect of dengue virus
o 1-3 yr: ALL, AML, CML Seen in the 1stfew days of illness during the viremic phase
o 3-11 yr: ALL, AML Ms: (+) Tourniquet Test
o 12-21 yr: ALL, AML *In doing the tourniquet test, you are not supposed to use the
Lymphomas: tourniquet. You use your BP cuff
o < 1 yr: very rare
o 1-3 yr: NHL Thrombocytopenia
o 3-11 yr: NHL, HL Starts during the febrile stage
o 12-21 yr: HL, NHL Most prominent during the toxic stage (preceed plasma
CNS: leakage)
o < 1 yr –3 yr: Medulloblastoma, ependymoma Mechanism: Decreased platelet production and increased
astrocytoma/glioma, choroid plexus papilloma peripheral destruction (megakaryocytic suppression)
o 3-11 yr Marked hypocellularity with a decreased megakaryocyte,
erythroblast and myeloid precursors
HEMATOLOGIC ASPECT OF DENGUE HEMORRHAGIC FEVER This is the why you have the typical blood picture of dengue. Which
is low platelet count, anemia even without bleeing and leukopenia
Clinical spectrum Increased peripheral destruction is prominent 2 days before
Asymptomatic infection defervescence
Undifferentiated fever Hemophagocytosis of young, mature erythroid and myeloid
Dengue fever cells, lymphocytes and platelets
Dengue hemorrhagic fever Survival of patients’ and transfused platelets was markedly
Dengue hemorrhagic fever decreased because of immune-mediated injury of platelets
Decline in platelet counts preceed plasma leakage
sustained high fever for 2-7 days
Even if the patient is bleeding and have low platelet count, we don’t
bleeding diathesis (+ TT, petechiae, epistaxis and
hematemesis) transfuse platelet concentrate because there might be immune
mediated injury of platelets.
thrombocytopenia
plasma leakage (hemoconcentration pleural effusion and Thrombastenia
ascites) Exhaustion from platelet activation triggered by immune
complexes containing dengue antigen
Pathophysiology
Absence of adenosine diphosphate (ADP) release in patients
activation of the T cells
with DHF during convalescent stage
production of cytokines
Impaired platelet aggregation response to ADP during febrile
disturbance of hemostaticsystem
and early convalescent stages that returned to normal
Increased apoptosis and endothelial cell dysfunction
response 2-3 wks later
Stages of Clinical Presentation Bone marrow examinations showed hypocellularity early in
1. Febrile the disease, followed by hypercellularity during recovery and
2. Toxic normocellularity in convalescence
3. Convalescent
Coagulopathy
Bleeding Manifestations Among 257 children with DHF During the acute febrile stage:
1. Petechiae 42.7 % (100) mild prolongation of the PT and PTT
2. Epistaxis 22 .6 % (53) reduced fibrinogen levels
3. GI bleeding 15.4 % (36) variable reductions in the activities of different coagulation
4. Menorrhagia 10.7 % (25) factors like prothrombin, factors V, VII, VIII, IX and X
5. Gum bleeding 6.0 % (14) low levels of protein C and S and anti-thrombin III
6. Ecchymosis 1.7 % (4) Direct activation of fibrinolysis by the
7. Hematuria 0.9% (2) dengue virus
Pathophysiology Focal necrosis of the hepatic cells
Evidence of plasma leakage (tachycardia, hypotension , PE , Two-threefold elevation of the transaminases
ascites)
High Risk Patients
Bleeding tendency
Prolonged shock
RISING HCT NOT ALWAYS EVIDENT Massive bleeding
 Obesity Republic Act 7719 (National Blood Services Act of 1994)
Infants less than 1 year of age To ensure safe and efficient blood banking and transfusion
If patient is experiencing prolonged shock, there will be DIC. Those with practices in the Philippines.
massive bleeding will be having end organ failure. For those kilos and above,
you use the ideal body weight. For those below 50 kilos, you use actual body The Philippine Clinical Practice Guidelines
weight. Infants less than 1 year of age are at risk because they usually
For the rational use of blood products and strategies for
present with atypical presentation
implementation in 2009.
Unusual Manifestation Aims to develop and implement national policies for
Patients with underlying disease: promoting the rational use of blood and blood products.
1. Thalassemia
2. Iron Deficiency Anemia Unique aspects of Red Blood Transfusion in Pediatric patients
3. G6PD 1. Age - dependent physiology of anemia
4. Hemophilia 2. Age - dependent signs and symptoms of anemia
5. CRF 3. Normal hemoglobin levels for age
Thalassemia you have chronic hemolysis. G6PD, you also have hemolysis 4. Hemoglobin threshold for transfusion
These patients will not be hemoconcentrate. When you have
hemoconcentration, you have to increase your fluid. Blood Component Therapy
Complications Giving only the particular blood fraction the patient needs
1. liver failure leaving the rest of the components for other purposes and
2. DIC avoiding waste. (PRBC, washed PRBC, platelet concentrate
3. encephalopathy cryoprecipitate, cryosupernate and fresh frozen plasma)
*You only use what is needed. 1 unit of blood can be given to 5 person
4. myocarditis
5. Acute renal failure
6. Hemolytic uremic syndrome Fresh Whole Blood
*Usually seen in severe dengue The use of FWB should be discouraged and patients should
What are signs and symptoms of myocarditis? Bradycardia, you can be given the specific blood products as indicated.
also have tachycardia, arrhythmia and muffled heart sound. When Indications
you get your cardiac rate, you need to get it for 1 full minute 1. Exchange transfusion
2. Acute blood loss (in the absence of blood
Management components)
Symptomatic Dose:
Control of shock and hemorrhage o 20 ml/kg
*Patients who do not receive a proper treatment usually die within o Always regulate IVF while on blood transfusion
12-24 hrs after shock ensues When you fresh whole blood, this are unbanked blood for less than 24 – 48
hours. So some of the components are not viable anymore if it is not fresh.
When do we suspect internal Bleeding? In children, you always regulate the IV fluid. You can give it with side drip
1. Patients with refractory shock, hct less than 40% or rapid with plain NSS. What we mean about regulate is we subtract the rate of the
drop in Hct blood from the rate of the main IV line. Usually in children, blood transfusion
2. Normal BP but pulse is rapid should be given for 2 hours.
3. Drop in Hct of more than 10% with 10 hrs of fluid
replacement The criteria for PRBC transfusion should be based on:
1. Hemoglobin level
Indication for blood transfusion 2. Patient's clinical condition
1. Platelet concentrate in controlling massive bleeding. 3. Risk for inadequate oxygenation / greater requirement
Dose: 0.2-0.4 units /kg with maximum of 8-10 units Patients who are intubated, with cardiac problems specially those patients
2. Packed red blood cell to increase the carrying capacity with congestive heart failure, with neurologic problems, usually require a
Dose: Desired hct–actual hctx wt 9 kg ) higher hemoglobin level for better oxygen requirement
3. Fresh frozen plasma in bleeding due to coagulopathyor
circulatory failure non-responsive to IV crystalloid PRBC (Concentrated RC or Plasma reduced blood )
replacement Indications for restoring tissue oxygenation:
Dose: 10-15ml /kg 1. Anemia (infection, malignancy, CRF, uremia, BMF )
4. Cryoprecipitate in patients with DIC 2. Pre —surgical anemia
3. Acute blood loss
APPROPRIATE USE OF BLOOD
Dose:
Who was the 1st person to do blood transfusion? o Desired Hct — Actual Hct x wt. (kg) = Deficit
In 1818 , British obstetrician, James Blundell - successfully o Acute anemia: 10 ml / kg
transfused human blood to a patient who had hemorrhage o Chronic anemia: 5 ml/ kg
during childbirth.  10 -15 ml /kg of rc will raise the hgb by 2.3 g/dl
In 1901, Karl Lansteiner an Australian physician discovered  Regulate IVF while on blood transfusion
the first human blood groups, which helped transfusion to Dose is computed, not always 10 cc/kg. You have to compute first for the
become a safer practice. deficit. After you got the deficit, you will have to divide it accordingly. If you
are dealing with acute anemia, give 10ml/kg. You can give it for every 6-8
hours. For chronic anemia, give 5ml/kg. You give it every 12 or 24 hours.
Depending on how low your hemoglobin is
 o Hereditary: Ganzmann's thrombasthenia , Bernard-
Washed RC Soullier Syndrome
To remove the majority of plasma, proteins, antibodies and
electrolytes Monitoring of patients
Depleted of plasma, platelets and leukocytes For each unit of blood transfused monitor at the transfusion
Indications: ff. stages (WHO 1997 )
1. Confirmed deficiency of immunoglobulin A 1. Before starting transfusion
2. Recurrent severe allergic type of adverse events 2. As soon as transfusion is started
(fever, generalized urticarial and dyspnea) 3. Every 15 minutes after starting the transfusion for the 1st
* Regulate IVF while on blood transfusion hour
We wash the red cells to remove the antibodies. In children, we usually wash 4. Every 30 minutes during transfusion on completion of
the red cells when the children had previous allergic or anaphylactic transfusion
reaction. We use plain NSS to wash the red cell. But if you do that, it is 5. 4 hours after completion of transfusion
already an open system. So you have to transfuse the blood already Usually after 2 hours, we monitor every hour for late transfusion reaction
Monitor and Record the ff:
Indications for leukocyte —reduced RC transfusion
1. General appearance
1. Immunocompromised patients
2. Patients for long —term RC support (thalassemia) 2. Temperature
3. RR, PR, BP
3. Hematopoietic cell transplantation
4. Fluid balance
5. Subjective complaints
Indications for Fresh Frozen Plasma transfusion
How will you know the fluid balance? Aside for looking if the patient is
1. Active bleeding or with coagulopathy undergoing surgical
dehydrated or not, you also need to monitor the urine output of the patient
procedure and other losses such as vomiting.
2. Support in the treatment of DIC
3. Replacement in patients with factor deficiency if specific What should be recorded?
factor concentrate is NOT available 1. Time the transfusion is started
4. Replacement fluid for shock 2. Volume and type of all products transfused
5. Factor VIII and IX deficiency 3. Donation number
 Dose: 10 -15ml /kg for 2 hours 4. Adverse events
 Regulate IVF while on blood transfusion Premedication
For patients with hemophilia, where they cannot afford factor 1. Hydrocortisone /IV - 5mkday q 6h
concentrate, you can give this every 12 hours. 2. Diphenhydramine /IV - 1mkdose q 6h
3. Oral Paracetamol - 10 mkdose q 6h lrasita
Cryoprecipitate * All medications should be given 30 minutes prior to blood
Contains Factor VIII and fibrinogen transfusion.
Indication : Secure Informed Consent – you have to tell everything to the parents.
1. Hemophilia A You have to explain to them why blood transfusion must be given even
2. VWD if there are side effects.
3. Hypofibrinogenemia
4. DIC Restrictive Transfusion Strategy (Rational)
Dose : Restrictive transfusion thresholds (triggers) are an effective
o 1-2 units / 10 kg or 5-10 ml/kg method of reducing and conserving RBC use.
o Neonates: 2m1/kg to 1 unit/7 kg RBC transfusion should NOT be dictated by hemoglobin
 Each unit = increase fibrinogen level by 5-10mg/dI 'trigger' alone.
 Regulate ivf while on blood transfusion A Clinical Practice Guidelines: Red Blood Cell Transfusion Thresholds
This is the first or priority blood component in patients with DIC.  A restrictive transfusion threshold is safe in most clinical
Because in DIC, it is fibrinogen which is the first factor to be settings.
deficient. In older children, you can give this on fast drip. Usually it is
only less than 50mL per bag. Summary of transfusion considerations in Burned Children
1. Children have greater body surface area than adults
2. Cardiac function, MBV and hgb levels are age- dependent in
Cryosupernate
children = higher blood transfusion / unit volume ratio
contains all coagulation factors except those present in
3. The optimal Hgb threshold has not been defined.
cryoprecipitate
4. Potassium levels should be monitored in children receiving
cryo — poor plasma
>20m1 / kg transfusion outcome.
Indication: Hemophilia B
5. The maximum allowable blood loss (MABL) without
transfusion is:
Platelet concentrate
a. MABL = ( Hct start — Hct target ) / Hct start ] x EBV
Thrombocytopenia:
o Acquired: infection, BMF , chronic disease , CRF Blood Transfusion Delay and Outcome in country in Kenya
o Hereditary: Wiskott — Aldrich Syndrome, Fanconi's  Significant delays between blood transfusio and actual
anemia transfusions are associated with pool outcomes.
Thrombasthenia How can we avoid delay in blood transfusions? In emergency room,
o Acquired: infection, BMF, chronic disease, CRF you must immediately request for CBC, reticulocyte count, blood
 typing and save serum for cross matching. If you are entertaining a 20 - < 30 consider for clinical reasons:
hemolytic process, you request coomb’s test. with any of the ff: coagulation
disorder, invasive procedure
Pediatric massive transfusion
30 -50 1st week of life < 1000 g,
Definition:
cerebral, hemorrhage, sepsis
1. Packed red blood cell (PRBC) transfusion of 50 % of the total
1. blood volume in 3 h Cryoprecipitate
2. PRBC transfusion of 100 % TBV in 3h 1. Microvascular/ active bleeding
3. PRBC transfusion of > 10 % of TBV in a minute 2. Massive bleeding
Characteristics of Neonatal Erythrocytes 3. Fibrinogen deficiency
 Dose : 1 unit / 5-10 kg
1. Life span is 60- 70 days (preterm 35 -50 days) : increased
rigidity Nonpharmalogical blood conservation strategies and techniques for
2. Higher MCV: more susceptible to oxidant induced injury newborns
3. High frequency of dysmorphology. 1. Increasing the hemoglobin endowment and its circulating
4. Hemoglobin switching from Hb F to Hb A occurs in the 1st blood volume at birth.
week of life 2. Removing less blood for laboratory testing.
5. Rate of Hgb synthesis and RBC production decreases sharply 3. Maintaining optimal nutrition.
during the 1st few days of life.
6. Iron homeostasis is different in neonates with lower hepcidin Hazards of Blood Transfusion
 The incidence of acute blood transfusion reactions among
levels.
pediatric patients: UP - PGH Experience
Perioperative neonatal and pediatric blood transfusion  The incidence of acute transfusion reaction among pediatric
Indications for Fresh Frozen Plasma patients is 20. 7 % and were all nonhemolytic transfusion
1. Liver disease reactions. Most of these reactions are associated with
2. Vit K deficiency platelet transfusions.
3. DIC Association between RBC Transfusion and Bronchopulmonary
4. Dilutional coagulopathy Dysplasia in Preterm Infants
5. VWD The incidence of BPD was higher with earlier PRBC
6. Hemophilia transfusions (within 3 weeks of life).
 Dose: 1 unit / 10 kg or 10- 15 ml/kg Greater numbers of PRBC transfusions also increased the
If you know you are dealing with hemophilia A, you can just give
incidence of BPD and its severity.
cryoprecipitate because it already has factor VIII. Or much better, you can
give factor concentrate, if your patient can afford. Packed red blood cell transfusions as a risk factor for parenteral
nutrition associated liver disease in premature infants
Infants of < 4 months of age require RBC transfusion if:
1. Hgb < 12g/di in the 1st 24 h of life Repeated RBC transfusions increase the risk of PNALD in the
2. Hct < 20 % with symptoms of anemia NICU infants.
3. Hct < 30 % on oxygen therapy or clinical signs of apnea, Transfusion reactions in pediatric compared with adult patients: a
bradycardia , tachycardia or low weight gain look at rate, reaction type and associated products
4. Hct > 45 % in the presence of cyanotic heart disease Transfusion and reaction rate
5. Blood loss > 10 %. Pediatric Adult
Criteria for Administering Packed RCT In patients 28, 702 77,624
Level Transfusions/ 1000 610/ 1000 1500/ 1000
Anemia 1st 24 h Hgb <12 g/dl in patients
Cumulative blood loss in 1 wk 10 % BW Reactions/ 1000 in 39/1000 39/1000
receiving Intensive care patients
Neonate receiving intensive care Hgb < 12g/dl Transfusions / 1000 39/1000 39/1000
Acute blood loss 10 % BW visits
Chronic oxygen dependency Hgb < 12g/dl Reactions/ 1000 0.15/ 1000 0.094/ 1000
Late anemia Hgb < 7g /dI visits
Reactions / 1000 6.16/1000 2.38/ 1000
transfusion
Volume 56, March 2015 TRANSFUSION

Red blood cell transfusion guidelines for premature neonates

Maintain hemoglobin (g/dI ) threshold based on clinical scenario: Blood Transfusion Reactions (more frequent in children)
1. > 11-15 for severe cardiopulmonary disease 1. Hypocalcemia
2. > 10 for moderate cardiopulmonary disease 2. Hyperkalemia
3. > 10 for major surgery 3. Hypomagnesemia
4. > 7-9 for symptomatic anemia 4. Acid-base disorders
5. > 7 for asymptomatic anemia 5. Hypothermia
6. Infectious disease transmission
Indications for platelet transfusion in neonates
Platelet count (x10/m1) Indication
< 20 always transfuse
 NEONATAL ERYTHROPOEISIS Polycythemia
Characteristics: Venipuncture hematocrit > 65 %
1. Different membrane properties Causes
2. Different hemoglobins 1. Maternal –fetal transfusion
3. Unique metabolic profile 2. Delayed umbilical cord clamping
4. Shorter RC lifespan 3. Twin–twin transfusion syndrome (the donor will have
Interaction factors: anemia, and the recipient will have polycythemia)
1. Genetics 4. Intrauterine hypoxia
2. Acquired disease of the newborn 5. Maternal diabetes
3. Maternal factors Effects
Development of Erythropoiesis 1. Seen in SGA and Intra Uterine Growth Restriction
14th day AOG: Onset of blood formation 2. hemolysis
15th –16th week AOG: Blood formation in the liver 3. Infants of diabetic mothers
3RD–6THmonths AOG: Primary site is the liver 4. Hyperviscosity: stroke, seizure, NEC, renal vein thrombosis
1st week postnatal life: Observed in the spleen Signs and symptoms
thereafter: primary site is the bone marrow 1. lethargy
2. irritability
Erythropoiesis After Birth
3. jitteriness
first few days after birth: rate of rc production decreases
4. tremors
dramatically
5. cerebrovascularaccidents
by: factor of 2-3: 1stfew days factor of 10: after the 1stweek
6. seizure
sudden and marked decrease in RC production is initiated -> 7. oliguria
increase in tissue oxygen levels at birth -> virtual 8. respiratory distress
disappearance of erythropoietin in the plasma
Treatment
Rate of production
For asymptomatic: Fluid boluses with crystalloid with hct of
1. Reaches a minimum on the 2ndweek of life
65% -75%
2. Shorter lifespan of the neonatal erythrocyte
For symptomatic: Partial exchange transfusion
Hematologic values at birth
Clinical spectrum of G6PD deficiency
VARIABLES:
1. Acute Hemolytic Anemia – Usually seen in older children
1. Gestational age
2. Neonatal Jaundice – seen during the 2nd to 3rd day of life
2. Conduct of labor
3. Congenital Nonspherocytic Hemolytic Anemia – at birth
3. Treatment of umbilical veins
4. Time of sampling Neonatal Jaundice
5. Site of sampling rarely present at birth
Can be dictated by gestational age. If you have a preterm patient, peak incidence: between day 2 and day 3
you have lower, means there might be bleeding. jaundice > anemia
Ms. Subclinical to threat to kernicterus
Anemia in neonate
1 Blood loss Prompt recognition is important to prevent neurologic
3. Hemolysis sequelae
4. Less than normal RC production Polycythemia
Erythropoietin 15% - SGA
1. Polypeptide hormones which acts via surface receptors 2% - AGA
2. Amplify RC production Summary
3. Prevents apoptosis 1. Monitor neuro developmental growth
Physiologic anemia 2. Advice and regular ff-up for the G6PD Deficiency
hemoglobin starts to drop on the 3rd day of life until the nature of the disease
2ndor 3rd month of life complications
Causes : plan (understanding of the disease process is
1. relative decrease in BM erythropoeitic activity mandatory)
2. increase in the rate of hemolysis
3. hemodilution due to rapid increase in blood volume HEMORRHAGIC DISEASE OF THE NEWBORN
You are not supposed to transfuse blood in patient with just physiologic Hemostatic System:
anemia. Because you will be sending negative feedback on your bone
marrow
begins in utero until after birth
functional levels of procoagulants coagulation inhibitors,
Hypoxia fibrinolytic components and platelet associated factors differ
induces a transcriptional increase in the expression of from older children and adults
erythropoietin “adult values” are reached until 6 months of age
In hypoxia you can have more production of red blood cell, you will
have polycythemia. Because you stimulate your erythropoietin
Summary of Hemostatic Differences between Neonates, Older o cephalhematoma , subgaleal , GI umbilicus,
Children and Adults: intraabdominal
1. Decreased plasma concentrations of procoagulantproteins ( Etiology
factors II, VII, IX , X , XI and XII ) , prekallikreinand kininogens o maternal ( Pb , phenytoin , warfarin rifampicin , INH
2. A unique fetal glycoformof fibrinogen — interfere with vit K ) inherited coagulopathy
3. Decreased plasma concentrations of the coagulation Prevention
inhibitors AT III, heparin cofactor II, TFPI, protein C & S & o Vit K to infant at birth or to mother before birth
slower rate of thrombin inhibition Incidence
4. Plasminogenless converted to plasmin o very rare
5. Elevated D-dimerlevels until at least 3 days after birth
6. Increased plasma VWF concentration & elevated levels of Classic Disease:
ULVWF multimers 2 - 7 days’ old
7. Transient hyporesponsivenessof platelets to agonists but exclusively breastfed infants
increased agglutination home delivered (no vit k at birth)
site of hemorrhage: early onset — umbilicus late — onset: 1-
Clinical Aspects of Developmental Hemostasis: 6 months (IC, GI, cutaneous mucosal, injection sites, thoracic)
acquired disorders are more common Cholestasis (malabsorption of vit K) abetalipoprotein
severe forms of congenital factor deficiencies and platelet deficiency, idiopathic in breastfed Asian infants, warfarin
disorders in healthy infants who are bleeding ingestion
15-30% of inherited bleeding disorders presents with prevented by parenteral & high dose oral vit K during
bleeding in the NB period malabsorption & cholestasis
Ms. dependeny on the primary disease
oozing from umbilicus
bleeding into the scalp, large cephalhematoma, bleeding Late — onset Disease:
after circumcision, phlebotomy sites bleeding into the skin, after 2 weeks of life
ICH cholestatic liver disease, pancreatic disease intestinal
Sick infants can bleed from: disorder (celiac sprue, inflammatory bowel disease, short
o mucous membrane, bladder, sites of invasive bowel syndrome)
procedures, joint bleeding (rare) management: Prophylaxis — 1 mg vit K1 / IM within 1 hour of
Healthy infants: birth or 2mg of vitkl orally at birth at 4-6 days and at 4- 6
o thrombocytopenia secondary to passage of weeks or 2mg of vitkl at birth and the wkly if 1mg for 3
maternal antiplatelet antibody, VK deficiency, months (IM is preferable
congenital coagulation factor deficiency VKDB: 1-2 mg vit k1 / slow iv or sc infusion
severe bleeding may require FFP
Laboratory Examinations:
1. Work –up for sepsis plus: Policy became less clear for the ff. reasons:
a. PT 1. Plasma concentrations of other coagulation proteins in
b. APTT addition to prothrombinwere low in newborns.
c. Thrombin clotting time (TCT) 2. Increasing recognition that bleeding in neonates was often
d. Fibrinogen level not due to VK deficiency
e. Platelet count 3. Administration of high amounts (50-70 mg) of water soluble
2. If with abnormality -specific factor assay form of VK resulted in hemolytic anemia with kernicterusin
some infants.
Screening Coagulation Tests in Neonates: 4. Clinicians suggested that VK prophylaxixwas not needed for
Plasma clotting activity are all prolonged during fetal development in all healthy FT Iinfants
neonates:
1. PT Prophylactic Vitamin K Administration:
2. APTT Forms:
3. TCT 1. VK1 (phytonadione) –present in leafy green vegetables
2. VK2 (menaquinone) - synthesized in the intestinal bacterial
Bleeding Time: flora
shorter than in adults in the 1stweek of life : 3. VK3 –can cause hemolytic anemia
increased plasma concentration of VWF *What we usually use is phytonadione
enhanced function of VWF
large RC, high hct
Recommendations for VK prophylaxis:
Hemorrhagic Disease of the Newborn: 1. VK -1-5 ug/ kg in newborns
Hemorrhage on days 1 to 5 of life from multiple sites in 2. SD of 0.5 –1 mg IM or Oral dose of 2-4 mg at birth with
otherwise healthy infants . subsequent dosing for breastfed infants
3. Repeated administration of oral VK or continuous low-dose
Early — Onset Disease: VK supplementation to prevent late VK deficiency.
Age 4. Additional for risk groups
o 0 — 24 hour infants with antitrypsin deficiency
Site chronic diarrhea
 cystic fibrosis marker of VK deficiency using maternal venous & cord blood,
celiac disease is common among mothers & babies ( 22 %) in Uganda
5. Pregnant women receiving oral anticonvulsant therapy -10 No predictors of NB insufficiency Universal administration
mg of oral VK1 daily from 36 wks gestation of NB VitK prophylaxis to prevent VKDB

Recommendations by the American Academy of Pediatrics (2003) NEONATAL JAUNDICE

1. Vitamin K should be given to all newborns as a single IM dose
of 0.5 to 1 mg. NORMAL VALUES IN TERM INFANT:
2. Additional research should be conducted on efficacy, safety Hgb
(g/dl
Hct
(%)
RBC
count
MCV
(fl)
MCH
(pg)
MCHC
(g/dl )
Reticul
ocytes
Nucleated
RBCs (mm3)
Platelet
(1000/mm
and bioavailability of oral formulations and optimal dosing ) (x106/ (%) 3
)
mm3)
regimens of vitamin K to prevent late VKDB. Cord 16.8 53 5.25 107.0 34 31.7 3-7 500 290.0
3. Health care professionals should promote awareness among blood
Day 1 18.4 58 5.8 108.0 35 32.5 3-7 200 192.0
families of the risks of late VKDB associated with inadequate Day 3 17.8 55 5.6 99.0 33 33 1-3 0–5 213
vitamin K Prophylaxis from current oral dose regimens, in Day 7 17.0 54 5.2 98.0 32.5 33 0-1 0 248
Day 14 16.8 52 5.1 96.0 31.5 33 0-1 0 252
newborns who are breastfed exclusively.
Emphasis on reticulocyte count. In the newborn, it can go as high as 3% up to
International Journal of Current Pharmaceutical and Clinical Research day 3. For example, you look at retics count for a 1-day old baby and it is at
(2015) 7%, it is still normal.
Late Hemorrhagic Disease should be considered after 6
months of age specially if predisposing factors like exclusive Diagnostic Approach to Anemia in The Newborn:
breastfeeding, cholestasis, cystic fibrosis and hepatitis is 1. anemia (< 13.5 g Hb/dl)
present. 2. reticulocyte count
3. Direct Antiglobulin Test
Journal of Pediatric Neurology (2014) o this will tell you if hemolysis is secondary to
Because of the potential consequences of ICH, infants immunologic process
strongly suspected to have VKDB should promptly be given 1 4. RBC Indices
mg of IV vitamin K (phytonadione) even before laboratory 5. Peripheral smear (normal)
results are unavailable. o blood loss or hemolysis
In life-threatening hemorrhage or surgical intervention, other
treatment like FFP FWB or rFvII can be given. Hemolysis:
Congenital RBC enzyme defects
Journal of Midwifery & Women’s Health (2016) o Pyruvic Kinase
Vitamin K Deficiency Bleeding in Infants o Hexokinase
Type Age at Onset Incidence o G6PD
Early 0 -24 hrs dependent on o Other
maternal condition Infection
Classic 1-7 days 0.4-1.7/100b o Bacterial
Late 2-12 wks ( up to 6 4.4-7.2/ 100T o Viral (CMV, rubella, HSV, coxsackie, adenovirus)
mos) o Toxoplasmosis
o Fungal
Vitamin K Deficiency Bleedingin Infants: Others:
Type Risk Factors Typical Ms o galactosemia
Early Seizure and antiTB Severe bleeding o hypothyroidism
Classic Bruising & umbilical
bleeding Causes of Unconjugated Hyperbilirubinemia
Late Breastfeeding ICB –60 % Increases the load of bilirubin
No vit K o hemolytic anemia
o polycythemia
o immaturity
Eastern Journal of Medicine (2015)
o increased enterohepatic Circulation
1. Classical and late onset type of HDN can be prevented to a
o infection
great extent by administration of VitK as per recommentations
of the AAP and local authorities. In immaturity, because you have less of the glucoronic transferase that is
2. Insufficient to prevent the development of early type of HDN. used in conjugation.
3. For prevention of early type HDN, vitK may be administered to
all pregnant women at least 2-3 days prior to delivery,
regardless of whether they carry a risk. thereby preventing all Damages or reduces the activity of enzymes
types of HDN o genetic deficiency
o hypoxia
Prevalence and Predictors of Functional Vitamin K Insufficiency in o infection
Mothers and Newborns in Uganda (2015) o thyroid deficiency
functional VK insufficiency evidenced by raised PIVKA-II For example, you have hypoxic patient. You have more pronounced
(immunoassay of uncarboxylated prothrombin) –sensitive jaundice.
 Competes or blocks the enzyme
o drugs and other substances influencing glucoronic
acid conjugation
Absence or decrease amounts of enzymes or reduction of
bilirubin uptake by the liver
o genetic defects
o prematurity

When To Investigate For The Cause of Jaundice?

1. It appears in the 24 -36 hours of life
2. Serum bilirubin is rising at a rate faster than 5 mg /dl/24 hr
3. Serum bilirubin is > 12 mg/dl in full terms infants
4. Jaundice persists after 10 -14 days of life
5. Direct acting bilirubin is >2mg/dl

If pathologic jaundice usually occurs at first 24 hours of life, there is ABO or

Rh incompatibility. If the direct bilirubin is > 2mg/dL, you think of biliary
atresia. Especially if the patient has dark colored urine and alkaline
phosphatase is high.

Other factors:
Family history of hemolytic disease
pallor, organomegaly
Failure of phototherapy to lower bilirubin
Vomiting, lethargy, poor feeding, apnea
excessive weight loss, abnormal VS
light –colored stool, dark urine, signs of kernicterus

Who are the patients wherein phototheraphy is indicated? It is for

unconjugated bilirubinemia. Because phototheraphy helps in conjugation of
bilirubin
 PEDIATRICS II

SEM 1P
HEMATOLOGY
DR: FAJARDO
Why? For us to narrow down the
FACTORS IN AFFECTING SKIN COLOR differential diagnosis ƒ
 Hemoglobin concentration  We have to compute for the
 State of constriction reticulocyte count index
 Dilation of peripheral vessels Actual hematocrit x retic count
 Pigmentation and SCT fluid % Desired hematocrit for age
 NORMAL RDW – Thalassemia
ANEMIA  INCREASE RDW - IDA
 Reduction in red cell mass
 Reduction in blood hemoglobin concentration RBC INDICES

CRITERIA FOR IDENTIFYING CHILDREN WITH LOW HGB &

HCT VALUES
HGB SYMPTOMS
9 -11 Little to no dysfunction Normal value: 80 – 100 fl
5-7 Extertional dyspnea
 Normal value of MCV: 80-100 femtoliters (fL) –
6.0 Some weakness
normocytic
3.0 Dyspnea at rest
 >100 fL – macrocytic
2-1.5 Cardiac failure  < 80 fL – microcytic
 Newborn until 2nd day of life is macrocytic

EVALUATION OF THE ANEMIC PATIENT

 History
o Onset of anemia
o Diarrhea
o History of travel  MCH – tells you the degree of hemoglobinization
o Menstrual history of your RBC
o Neonatal history  NV = 27 – 34 pg
o Infection  normally, the central pallor will occupy the
o Diarrhea central portion. So, if it goes beyond the central
o Nutritional history quadrant, you label that as mild, moderate or
o History of trauma severe hypochromia
o Family history of bleeding disorder or
hemolytic anemia
 PE
o Emphasize in looking in palpebral
conjunctiva
o Pallor RETICULOSYTOSIS
o Jaundice  When the reticulocyte index more than 1.5%, it
o Organomegaly means that the bone marrow is compensating for
o Lymphadenopathy the anemia reticulocytosis
o Oral lesions  What are the conditions which can give you
o Tachychardia reticulocytosis?
o Tachypnea o ACUTE BLOOD LOSS o
o Cardiac murmur o HEMOLYTIC ANEMIA o
o Bloody urine and stool o AFTER A THERAPEUTIC TRIAL OF IRON
o Stunted growth o when you are suspecting patients with
o Skull structural deficit IDA, you can give ferrous sulfate, and
 Laboratory test (Confirmatory) then, after 3 days, you have
o CBC - You want to see the Hgb, Hct level reticulocytosis
o RBC indices
o Reticulocyte count IRON DEFECIENCY ANEMIA
o Peripheral smear  Most common cause of anemia
o Platelet count – It could be a cause of  Common ages 6-24 months
bleeding (NV= 150 – 450 K)
o Reticulocyte
 young RBC ƒ you have to
request a retic count in all ETIOLOGY OF IRON DEFECIENCY ANEMIA
patients presenting with anemia.  Inadequate supply of iron
Page 1 of 8
 SEM 1P
HEMATOLOGY

o Lack of iron stores at birth o (formation of postcricoid esophageal

 LBW web)
 PT o Koilonychia or spooning of the fingernails
 Twin or multiple births  Miscellaneous
 Severe IDA in mother o pica (consume laundry starch, ice and
 Fetal blood loss soil clay)
 Bleeding from the 1st few days of o Massive hepatosplenomegaly
life o poor wound healing and bleeding
o Inadeuqate intake diathesis
 Defeciency dietary iron o zinc deficiency
 Impaired absorption o lead intoxication
o Chronic or recurrent diarrhea o Pseudotumor cerebri
o Malabsorption syndrome
o Gastrointestinal abnormalities STAGES OF IRON DEFECIENCY ANEMIA
 Excessive demands for iron for growth  Prelatent iron deficiency
o Pt  latent iron deficiency
o lbw  frank iron deficiency
o infants
o adolescent  PRELATENT IRON DEFICIENCY
o pregnancy o depleted stores without a change in hct
 Blood loss or serum iron levels
o Acute or chronic hemorrhage o Rarely detected
o Parasitic infection (hookworm trichuris  LATENT IRON DEFICIENCY
trichiura) o decreased serum iron level
o total iron - binding capacity increases
FACTORS THAT MODIFY IRON ABSORPTION without a change in the hct
 Physical state o Decreased transferrin saturation
o (bioavailability) heme > fe 2+ > fe 3 +  IRON DEFICIENCY ANEMIA
 Inhibitors o associated with erythrocyte,
o Phytates microcytosis and hypochromia
o tannins
o Soil laundry starch DIAGNOSIS
o iron overload  In infants: high index of suspicion
 Competitors o prematurity
o Cobalt o blood loss
o lead o fed exclusively on milk
o Strontium o Chronic diarrhea
 Facilitators
o Ascorbate PREVENTION
o Citrate 1. Administration of iron to expectant mothers
o amino acids 2. Early introduction of solid food
3. Supplemental iron : 1o – 15 mg of elemental iron / day (
CONSEQUENCE OF IRON DEFECIENCY ANEMIA 6 -8 wks of age )
 Anemia
o impairs tissue oxygen TREATMENT OF IRON DEFECIENCY ANEMIA
o weakness, fatigue, palpitations and  Diagnostic approach
lightheadedness o demonstrate the presence of anemia
o Reactive thrombocytosis o Determine the cause
 Growth and developmental retardation o correct the abnormality
o Growth and developmental o how do you treat patients with iron
abnormalities deficiency?
o impairs neurologic functions (behavioral  Give iron supplementation o in
abnormalities, motor incoordination and patients who are preterm, we
seizure) start iron preparation at 2 weeks
 Epithelial changes of age.
o angular stomatitis  in patients who are full term, you
o Glossitis start your iron preparation at 4-5
o flattened and atrophic lingual months
o Papillae  why? because the iron stores
o plummer- vinson are already decreasing

Page 2 of 8
 SEM 1P
HEMATOLOGY

CONSEQUENCE OF IRON OVERLOAD  Other malignant disease ƒ

 Heart  Immune-deficiency states ƒ
 Liver  Systemic lupus erythematosus
 Endocrine and other autoimmune disorders
ƒ
HEMOLYTIC DISORDERS  Drug-induced
o Autoimmune hemolytic anemia due to
HEMOLYTIC ANEMIA cold-reactive antibodies
 Results from an:  Traumatic and microangiographic hemolytic
o Increased rate of red cell destruction anemias
o Unimpaired bone marrow response o Prosthetic valves and other cardiac
SITE OF HEMOLYSIS abnormalities
o Hemolytic –uremic syndrome o
INTRAVASCULAR EXTRAVASCULAR Thrombotic thrombocytopenic purpura
HEMOLYSIS HEMOLYSIS o Disseminated intravascular coagulation
(w/in the circulation (w/in Tissue macrophage) o Associated with immunologic
Destruction of RBC within Reticuloendothelial cell phenomena( graft rejection)
BV  Infectious agents
Jaundice Organomegaly (Spleen, o Protozoa ƒ
Hemoglobinuria Liver)  Malaria ƒ
Hemoglobinemia More pronounced  Toxoplasmosis
jaundice o Bacteria ƒ
 Clostridial infection ƒ
Hemolytic anemia  Cholera ƒ
Infection  Typhoid fever and others
Malignancy o Chemicals , drugs and venoms
Collagen diseases  Associated with hemodialysis
and uremia
INHERITED HEMOLYTIC DISORDERS  Venoms
 Defects in the erythrocyte membrane o Physical agents
o Hereditary spherocytosis o  Thermal injury
o Hereditary elliptocytosis o Hypophosphatemia
 Deficiency of erythrocyte glycolytic enzymes o Paroxysmal nocturnal hemoglobinuria
o Pyruvate kinase deficiency o Spur –cell anemia in liver disease
 Abnormalities of erythrocyte nucleotide o Vitamin E deficiency in newborns
metabolism
o Adenylate kinase deficiency CLINICAL MANIFESTATION
 Deficiencies of enzymes involved in the pentose CHRONIC CONGENITAL HEMOLYTIC ANEMIA
phosphate pathway and in the glutathione  Degree of anemia
metabolism  Jaundice
o Glucose -6- phosphate dehydrogenase (  Crises
G6PD)  Splenomegaly
o Glutathione reductase  Cholelithiasis
 Defects in globin structure and synthesis  Leg ulcers
o Unstable Hgb Disease  Skeletal abnormalities ( tower-shaped and skull
o Sickle cell anemia thickening and striation of the frontal and
o Hemoglobinopathies parietal bones)
o Thalassemia major
o Hgb H disease Acquired hemolytic anemia
 Pains in the back , abdomen or limbs
ACQUIRED HEMOLYTIC DISORDERS  Headache , malaise , vomiting , chills and o
 Immunohemolytic anemias  Fever
o Transfusion of incompatible blood  prostration and shock
o Hemolytic disease of the newborn  Pallor , jaundice ,tachycardia
o Autoimmune hemolytic anemia due to
warm-reactive antibodies ƒ
 Virus and mycoplasma infection LABORATORY SIGNS OF ACCELERATED RED CELL
ƒ DESTRUCTION
 Lymphosarcoma ƒ
 Chronic lymphocytic leukemia ƒ  Decreased erythrocyte life span

Page 3 of 8
 SEM 1P
HEMATOLOGY

 Increased catabolism of heme  Platelet Phase – secondary hemostasis

 Increased serum lactate dehydrogenase activity  Plasma Phase – secondary hemostasis
 Absence of serum haptoglobin
 Reduced glycosylated hemoglobin
 Signs of intravascular hemolysis ¾
 Hemoglobinemia
 Hemoglobinuria
 Hemosiderinuria
 Fall in Hgb level greater than 1g/dl/wk

IMMUNE HEMOLYTIC ANEMIA

 Alloimmune hemolytic anemias
o Hemolytic transfusion reactions
o Hemolytic disease of the newborn
o Blood group incompatibility COAGULATION FACTORS
 Autoimmune hemolytic anemia I – FIBRINOGEN
o Idiopathic II – PROTHROMBIN
 Sec to underlying disorder III - TISSUE THROMBOPLASTIN
 Passive Transfer of Maternal Ab IV - CALCIUM
o Warm antibody type o Cold antibody V - PRO-ACCELERIN / LABILE FACTOR
type VI - NONE
 Drug- induced VII - PRO-CONVERTIN SERUM PROTHROMBIN CONVERSION
o Penicillin type ACCELERATOR (SPCA) STABLE FACTOR
VIII - ANTI-HEMOPHILIC GLOBULIN (AHG) ANTI -
BLEEDING DISORDERS HEMOPHILIC FACTOR (AFG)
 What is the most common cause of hemolysis in IX - CHRISTMAS FACTOR PLASMA THROMBOPLASTIN
the newborn period? COMPONENT
o RH Incompatibility X - STUART-POWER FACTOR
 What are the factors involved in your intrinsic XI - PLASMA THROMBOPLASTIN ANTECEDENT XII -
pathway? HAGEMAN FACTOR
o Factors 8,9,11,12. XIII - FIBRIN STABILIZING FACTOR
 What is the laboratory examination that could
give a rough estimate of your intrinsic pathway?
o aPTT HEMOSTATIC MECHANISM
 What is its significance? ƒ PRIMARY SECONDARY
o Significant in patients with more than 20 Vasoconstriction Intrinsic and extrinsic
seconds compared to the control. ƒ Platelet formation pathway
o For example, the control is 85 seconds, Plug (Adhesion & (Formation of Fibrin
and the patients level is 5 minutes. PTT is Aggregation) thrombus)
prolonged
 What does it mean if you have a prolonged PTT? DIAGNOSIS
o There is deficiency in your intrinsic  History
pathway factors especially your factor 8.  Physical Examination
 What are the factors in your extrinsic pathway?  Laboratory Test
o 2,5,7,9 o CBC, APC
 What is the test for your extrinsic pathway? o Bleeding Time - rough estimate (IVY upto
o PT. 10min) ƒ
o In the PT, what you look at is the INR or  prolonged in Thrombocytopenia
the international normalized ratio. o o Prothrombin Time
When it is >1.5, you have a prolonged o Partial Thromboplastin Time
prothrombin time, meaning, you have a
deficient factor especially your factor 7

HEMOLYSIS
 Sum total of those specialized functions within the
circulating blood and its blood vessels that are
designed to stop hemorrhage

PHASES OF HEMOSTASIS
 Vascular Phase – primary hemostasis

Page 4 of 8
 SEM 1P
HEMATOLOGY

 Patients who do not receive a proper treatment

usually die within 12- 24 hrs after shock ensues .----
---- Journal Compilation 2006

WHEN DO WE SUSPECT INTERNAL BLEEDING

 Patients with refractory shock , hct less than 40%
or rapid drop in hct
 Normal Bp but pulse is rapid
 Drop in hct of more than 10% with 10 hrs of fluid
replacement

INDICATIONS FOR BLOOD TRANSFUSION

 Platelet concentrate in controlling massive
bleeding.
o Dose : 0.2-0.4 units /kg with maximum of
8-10 units
 Packed red blood cell to increase the carrying
IMMUNE THROMBOCYTOPENIC PURPURA capacity
 Acquired hemorrhagic disease o Dose : Desired hct – actual hct x wt 9 kg )
 Characterized by:  Fresh frozen plasma in bleeding due to
o Thrombocytopenia coagulopathy or circulatory failure non-
o Purpura responsive to Iv crystalloid replacement
o Absence of other causes of o Dose: 10-15ml /kg
thrombocytopenia  Cryoprecipitate in patients with DIC
o No organomegaly  BLOOD TRANSFUSION IS NOT A TOTALLY SAFE
 BM aspirate : abundant Megakaryocytes PROCEDURE
 Acute >2x Chronic
 Etiology : Autoimmune Disorder TREATMENT OPTIONS
 Females >3x Male  Recombinant activated factor VII :
1. Enhances thrombin generation
HEMORRHAGIC DISEASE OF THE NEWBORN 2. Enhances activity and function of
 Self-limited disease patients’ and transfused platelets
 1st - 5th day of Life 3. Direct activation of factor X
 Deficiency of Vitamin K Dependent Clotting 4. Down- regulation of fibrinolysis
Factors II, VII, IX, X 5. Dose : 60-90 ug/kg
 Less common in Full-term infants
 Bleeding RARELY MASSIVE RATIONAL/APPROPRIATE USE OF BLOOD:
 Prolonged PT, PTT A REVIEW AND CURRENT PRESPECTIVE IN BLOOD
 Mgt: Vitamin K TRANSFUSION
 Republic act 7719 (National Blood services act of
HEMATOLOGIC ASPECT OF DENGUE FEVER 1994)
o To ensure safe and efficient blood
BLEEDING DIASTHESIS banking and transfusion practices in the
 vasculopathy Philippines
 thrombocytopenia  The Philippine clinical practice guidelines:
 platelet dysfunction o For the rational use of blood products
 coagulopathy and strategies for implementation in 2009
o Aims to develop and implement national
HIGH RISK PATIENTS policies for promoting the rational use of
 Prolonged shock blood and blood products.
 Massive bleeding
 Obesity UNIQUE ASPECT OF RED BLOOD TRANSFUSION IN
 Infants less than 1 year of age PEDIATRIC PATIENTS
 Age – Dependent of physiology of anemia
MANAGEMENT  Age- Dependent signs and symptoms of anemia
 Normal hemoglobin levels for age
 Symptomatic  Hemoglobin threshold for transfusion
 Control of shock and hemorrhage
BLOOD COMPONENT THERAPHY

Page 5 of 8
 SEM 1P
HEMATOLOGY

 Giving only the particular blood fraction the

patient needs leaving the rest of the component
for other purposes and avoiding waste. (PRBC,
Washed PRBC, Platelet concentrate
cryoprecipitate, cryosupernate and fresh frozen
plasma)
FRESH WHOLE BLOOD
 The use of FWB should be discouraged and
patients should be given the specific blood
products as indicated
 Indications:
o Exchange transfusion
o Acute blood loss
 Dose:
o 20 ml/ kg
*ALWAYS REGULATE IVF WHILE ON BLOOD TRANSFUSION
THE CRITERIA FOR PRBC TRANSFUSION SHOULD BE BASED
ON
 Hemoglobin level
 Patients clinical condition
 Risk for inadequate oxygenation/ greater
requirement.
PRBC (Concentrated RC or Plasma reduced blood)
 Indications for restoring tissue oxygenation
o Anemia (Infection,
malignancy,CRF,Uremia,BMF)
o Pre- surgical anemia
o Acute blood loss
 Dose: Desired HCT – Actual HCT x Wt. (kg) =
deficit
o Acute anemia: 10 ml/kg
o Chronic anemia: 5 ml/Kg
 10 -15 ml/kg of rc will raise the hgb by 2.3 g/dl
 Regulate IVF while on blood transfusion
WASHED RC
 To remove the majority of plasma, proteins,
antibodies and electrolytes
 Depleted of plasma, platelets and leukocytes
 Indications:
o Confirmed deficiency of immunoglobulin
A o RR,PR,BP
o Recurrent severe allegic type of adverse
events (Fever, generalized urticarial
dyspnea)
INDICATIONS FOR FRESH FROZEN PLASMA TRANSFUSION
 Active bleeding or with coagulopathy
undergoing surgical procedure
 Support in the treatment in DIC
 Replacement in patients with factor deficiency if
specific factor concentrate is NOT available
 Replacement fluid for shock
 Factor VIII and IX deficiency
 Dose: 10 – 15 ml/kg
o Regulate IVF while on blood transfusion
CRYOPRECIPITATE
 Contains F8 and fibrinogen
 Indication:
o Hemophilia A
o VWD
o Hypofibrinogenemia
o DIC
 Dose: 1-2 units / 10 kg or 5 – 10 ml/kg
 Neonates: 2 ml/kg to 1 unit/7 kg
o Each unit = Increase fibrinogen level by
5-10 mg/dl
o Regulate ivf while on blood transfusion
CRYOSUPERNATE
 Contains all coagulation factors except those
present in cryoprecipitate
 Cryo – poor plasma
o Indication: Hemophilia B
PLATELET CONCENTRATE
 Thrombocytopenia
o Acquired: Infection, BMF, chronic
disease, CRF
o Hereditary: Wiskott – Aldrich syndrome,
fanconi’s anemia
 Thrombasthenia
o Acquired; Infection, BMF, Chronic
disease, CRF
o Hereditary: Ganzmanns thrombasthenia,
Bernard soullier syndrome
MONITORING OF PATIENTS
 For each unit of blood transfused monitor at the
transfusion the ff stages ( WHO 1997):
o Before starting transfusion
o As soon as transfusion is started
o Every 15 minutes after starting the
transfusion for the 1st hour
o Every 30 minutes during transfusion on
completion of transfusion 5. 4 hours after
completion of transfusion
 Monitor and record the ff:
o General appearance
o Temperature
o Fluid balance
o Subjective complaints
 What should be recorded
o Time the transfusion is started
o Volume and type of all products
transfused
o Donation number
o Adverse events
 Premedication:
o Hydrocortisone/IV – 5 mkday q 6h
o Diphenhydramine/IV – 1 mkdose q 6h
o Oral paracetamol – 10 mkdose q 6h
* All medications should be given 30 mins prior to blood
transfusion

Page 6 of 8
 SEM 1P
HEMATOLOGY

CLINICAL IMPLICATIONS o Chemicals

 In critically ill patients, a restrictive transfusion o Drugs
strategy should be employed o Genetics (somatic genetic alteration >
 RBC transfusion should not be dictated by a hgb Inherited)
“trigger” alone, but should be based on  Genetics:
assessment of the patient’s clinical status o If 1st twin develops leukemia during the 5
 Where indicated, transfusion of a single unit of years life of life -> The risk of the 2nd twin is
RBC followed by clinical assessment to determine 20%
the need for further transfusion is appropriate. o Sibling of leukemias patients -> 4x
Greater than general population
CHARACTERISTICS OF NEONATAL ERYTHROCYTES o Chromosomal abnormalities:
 Life span is 60 – 70 days ( pre term 35 – 50 days) :  Trisomy 21 – 1 in 95
increased rigidity  Bloom syndrome – 1 in 8
 Higher MCV: more susceptible to oxidant –  Fanconi anemia – 1 in 12
induced injury  Increased incidence with:
 High frequency of dysmorphology o Congenital agammaglobulinemia
 Hemoglobin switching from Hb f to HB A occurs in o Poland syndrome
the 1st week of life o Shwachman – diamond syndrome
 Rateof hgb synthesis and RBC production o Ataxia telangiectasia
decreases sharply during the 1st few days of life. o Li-Fraumeni syndrome
 Iron homeostasis is different in neonates with o Neurofibromatosis
lower hepcidin levels. o Diamond Blackfan anemia
o Kostmann disease
INDICATIONS FOR FRESH FROZEN PLASMA o Bloom syndrome
 Liver disease
 Vit. K Def CLINICAL FEATURES OF ACUTE LYMPHOBLASTIC LEUKEMIA
 DIC  General systemic effects
 Dilutional coagulopathy o Fever (60%)
 VWD o Lassitude (50%)
 Hemophilia o Pallor (40%)
 Dose: 1 Unit/10 kg or 10-15 ml/kg  Hematologic effects (bone marrow invasion)
o Anemia, neutropenia,
BLOOD TRANSFUSION REACTIONS ( MORE FREQUENT IN thrombocytopenia
CHILDREN) o Pancytopenia -> Acute leukemia ( Single
 Hypocalcemia cytopenia hypocellular BM, no
 Hyperkalemia organomegaly, diagnosis 1-9 mos after
 Hypomagnesemia onset of symptoms)
 Acid base disorders  Clinical manifestation from lymphoid system
 Hpothermia infiltration:
 Infectious disease transmission o Lymphadenopathy (mediastinal -> SVC
syndrome) – common in T cell leukemia
o Splenomegaly
LEUKEMIA o Hepatomegaly
 Acute Leukemia
o Clonal expansion and arrest at a specific CLINICAL MANIFESTATION OF EXTRAMEDULLARY INVASION
stage of normal myeloid or lymphoid  Central nervous system
hematopoiesis o 5% of children at initial diagnosis
o 97% of all childhood leukemias o S/Sx of increased ICP
 ALL – 75% o S/sx of parenchymal involvement
 AML (ANLL) – 20% (Ataxia hypotonia and hyperflexia)
 Acute undifferentiated leukemia o Hypothalamic syndrome (Polyphagia,
– 0.5% hirtuism)
 Acute mixed - lineage o Diabetes insipidus
 Chronic leukemia o Chloromas
o Chronic myelogenous leukemia – 3% o Hemorrhage (AML>ALL) : caused by
 Incidence: leukostasis, thrombocytopenia and
o Peak: 2-5 years of age coagulopathy
o 25 – 30% of all childhood cancers  Genitourinary involvement ( Testicular
 Etiology: Involvement)
o Ionizing radiation o Painless enlargement

Page 7 of 8
 SEM 1P
HEMATOLOGY

o 10-23% ( median time – 13 months) o CNS 2 (<5WBCs/mm3, blast on

o Riskfactors: T-cell ALL, leukocytosis at cytocentrifuge)
diagnosis (>20,000/mm3). Mediastinal o MDR – 0.01% to 0.99%
mass, moderate – severe organomegaly  Unfavorable
and lymphadenopathy and o < 1 year old
thrombocytopenia (<30,000/mm3) o Hypodiploid
 Ovarian involvement: Very rare o CNS 3 (>5 WBCs/MM3, blast
 Paraprism: Rare cytocentrifuge)
 Renal involvement o >/= 1 %
o Hematuria, hypertension and renal
failure INFANT LEUKEMIA
o More common in T cell ALL or mature B-  Infant ALL accounts for 2-5% of childhood
cell ALL leukemia
 Gastrointestinal (typhitis and bleeding)  Poor prognostic features:
 Bone and joint involvement o High initial WBC
o 25% as initial symptoms o Massive organomegaly
o Radiologic changes: o Thrombocytopenia
 Osteolytic lesions o Thrombocytopenia
 Transverse metaphyseal o CNS leukemia
readiolucent bands o Failure to achieve complete remission by
 Transverse metaphyseal lines of day 14
increased density (growth arrest
lines) ACUTE MYELOID LEUKEMIA
 Subperiosteal new bone  Age incidence peak in neonatal adolescence
formation  Predisposing factors
 Skin involvement o Down syndrome
o Occurs occasionally in neonatal o Fanconi anemia
leukemia or AML o Kostmann syndrome
 Cardiac involvement o Bloom syndrome
o Symptomatic heart disease in less than o Diamond – blackfan anemia
5% cases  Secondart AML can evolve from: Myelodysplastic
 Lung involvement syndromes and myeloproliferative syndromes
o Is uncommon
ACUTE MYELOGENOUS LEUKEMIA
ACUTE LYMPHOBLASTIC LEUKEMIA  Poor prognosis
 Diagnostic o WBC >100,000/mm3
o Blood count o Monosomy 7
o Bone marrow o Secondary AML
o Chest radiograph o Minimal residual disease after induction
o Blood chemistry
o Cerebrospinal fluid CONGENITAL LEUKEMIA
o Coagulation profile  Diagnosed from birth to 6 weeks of age
o Cardiac function  Rare disease
o Infectious Disease profile  Unknown etiology
 Associated with: Trisomt 21, turner syndrome,
PROGNOSTIC FACTORS IN CHILDHOOD ACUTE mosaic trisomy 9, mosaic monosomy 7
LYMPHOBLASTIC LEUKEMIA  Clinical features:
 Favorable o Nodular skin infiltrates ( bluish, fibroma –
o 1-9 years tumors lerkemia cutis)
o WBC = <50 (X 10 9/ L) o Hepatosplenomegaly
o Immunophenotype – precursor B cell o Lethargy, poor feeding, pallor
o Hyperdiploidy o Purpura/Petechiae
o CNS 1 (5<WBCs/mm3, no blast on o Respiratory disease
centrifuge)  Laboratory studies:
o MRD (end of induction) - <0.01% o Usually monocytic subtype at ANLL (pre-
 Intermediate B immunophenotype)
o >/= 10 years
o WBC >/ = 50% (x109/L)
o T cell
o Diploid

Page 8 of 8
 PEDIA
HEMATOLOGY
DOC FAJARDO

Neonatal Erythropoiesis 4. Enterohepatic recirculation of bilirubin

Interaction of the factors Bilurubin formation
1. Genetics 1. Sequestration and destruction of senescent red
2. Acquired disease of the newborn blood cells in the reticuloendothelial cells
3. Maternal factors 2. Hemoglobin catabolism – indirect bilirubin
Anemia in the Neonate 3. Indirect bilirubin is bound by albumin for transport
1. Blood loss to the liver
2. Hemolysis 4. Indirect bilirubin is dissociated from the carrier
3. Less than normal RC production protein at the liver membrane, then transferred
Physiologic Anemia into the hepatic cell in unbound form
 Hemoglobin starts to drop on the 3rd day of life
until the 2nd or 3rd month of life Overproduction of bilirubin
 Causes: 1. Intravascular Hemolysis
o Relative decrease in BM erythropoeitic  Isoimmunization due to fetal-maternal blood
activity group incompatibilities (ABO and Rh)
o Increase in the rate of hemolysis  Maternal-fetal or feto-fetal transfusion
o Hemodilution due to rapid increse  Chemical and bacterial hemolysis
Hypoxia  Structural abnormalities of rbc (hereditary
 Induces a transcriptional increase in the spherocytosis and pyknocytosis)
expression of EPO  Enzymatic deficiencies of the rbc (G6PD, pyruvic
Polycythemia kinase)
 Venipuncture haematocrit >65% 2. Extravascular hemolysis
Causes:  Petechial hemorrhages
 Maternal fetal transmission  Enclosed hematomas
 Delayed umbilical cord clamping Under secretion of Bilirubin
 Twin-twin trabsfusion syndrome 1. Enzymatic Deficiency
 Intrauterine hypoxia  Glucuronyl transferase
 Maternal diabetes
 Uridine diphosphopyridine glucose
Effects:
dehydrogenase
1. Seen in SGA and IUGR
2. Hormonal suppression
2. Hemolysis
 Pregnanediol (breast milk jaundice)
3. Infants of diabetic mothers
 Thyroxine deficiency
4. Hyperviscosity: stroke, seizure, NEC, renal vein
3. Inhibition of conjugation
thrombosis
Treatment:  Galactose 1 phosphate uridyl transferase
 For asymptomatic: Fluid boluses with crystalloid deficiency (galactosemia)
with HCT of 65% - 75%  Drugs (sulphonamides, vit k)
 For symptomatic: Partial exchange transfusion  Lucey – Driscoll syndrome
Clinical Spectrum of G6PD Deficiency: 4. Hepatic cell injury due to infections
1. Acute Hemolytic Anemia  Coliform bacteria, cytomegalic virus,
2. Neonatal Jaundice toxoplasmosis, syphilis, viral hepatitis herpes
3. Congenital nonspherocytic Hemolytic Anemia simplex
Neonatal Jaundice 5. Substrate deficiency (hypoglycemia)
 Rarely present at birth
 Peak incidence: between day 2 and day 3 6. Circulatory insufficiency
 Jaundice>ANEMIA  Dehydration
 Ms. Subclinical to threat to kernictus  Anoxia
 Prompt recognition is important to prevent  Pyloric stenosis
neurologic sequelae Mechanical Obstruction
1. Biliary atresia
Hyperbilirubinemia in the Newborn 2. Hepatitis
 Enterohepatic circulation of bilurubin 3. Mucoviscidosis
1. Overproduction of bilirubin
2. Undersecretion of bilirubin Isoimmune Hemolytic Anemia
3. Mechanical obstruction of the bile ducts

Page 1 of 3

HEMATOLOGY
 Arise from blood group incompatibility between
fetus and mother which results in formation by
the mother of antibodies against her infants red
cells
Rh Incompatibility
 Occurs in Rh negative mother who has an Rh
positive infant
 Premature born very pale edematous infant
(severe anemia resulting in compensatory
tachycardia and subsequent high output
congestive failure)
Clinical
1. Frequency – unusual
2. Occurrence in 1st born – 5%
3. Severe 1st next pregnancies – usually
4. Stillbirths and/or hydrops – occasional
5. Pallor – marked
6. Jaundice – marked
7. Organomegaly – marked
8. Incidence of late anemia - common
Laboratory Findings
1. Blood type mother – Rh negative
2. Blood type infant – Rh positive
3. Antibody type – incomplete (7s)
4. DAT – positive
5. IAT – positive
6. HEmoglobin level – very low
7. Serum bilirubin – markedly elevated
8. RC morphology – nucleated RBCs
Prevent or Minimize
1. Injection of antibody (Rhogam) – effective
against Rh-positive cells
2. Itrauterine blood transfusion
Treatment
1. Need for antenatal management - YEs
2. Exchange transfusion
 Frequency – 2:3
 Donor blood type – Rh negative group specific
when possible
ABO Incompatibility
 Type O mother – anti A and anti B agglutinins
which transverse the placenta to enter the fetal
circulation
 Fetal red cells – agglutinogen A and
agglutinogen B
 Hemolysis
Clinical
1. Frequency – common
2. Occurrence in 1st born – 40 – 50%
3. Severe 1st next pregnancies – noooo
4. Stillbirths and/or hydrops – rare
5. Pallor – minimal
6. Jaundice – minimal (occ. Marked)
7. Organomegaly – minimal
8. Incidence of late anemia - uncommon
SEM 1P
Laboratory Findings
 Blood type mother - O
 Blood type infant – A or B or AB
 Antibody type – immune (7s)
 DAT – usually positive
 IAT – usually positive
 Hemoglobin level – moderately low
 Serum bilirubin – variably elevated
 RC morphology – spherocytes
Treatment
1. Need for antenatal management - NO
2. Exchange transfusion
 Frequency – 1:10
 Donor blood type – Rh same as infant group O
only
Kernicterus
 High level of unconjugated bilirubin deposited in
the brain cells
 Rare in healthy term infants
 Bilirubin toxicity starts at levels as low as 8-12
mg/dl in sick and very low birth weight premature
 Ms: lethargy, hypotonia, poor feeding,
generalized spasticity, irregular respirations
 Post kernicterus syndrome: deafness, cerebral
palsy
Management
 Double volume exchange transfusion (donors
blood should match the mothers blood)
 Phenobarbital and phototherapy
Hemmorhagic Disease of the Newborn
 Hemmorrhage on days 1-5 of life from multiple
sites in otherwise healthy infants – Townsend in
1894
 Early onset disease
 Age – 0-24hr
 Site – cephalhematoma, subgaleal, GI umbilicus,
intrabdominal
 Etiology – maternal (Pb, phenytoin, warfarin,
rifampicin, INH – interfere with vit k), inherited
coagulopathy
 Prevention – Vit K to infant at birth or to mother
before birth
 Incidence – very rare
Classic Disease
 2-7 days old
 Exclusively breasfed infants
 Home delivered (no vit K at birth)
 Site of haemorrhage:
 early onset – umbilicus
 late onset – 1-6 months (IC, GI, cutaneous
mucosal, injection sites, thoracis)
 Cholestasis (malabsorption of vit K )
abetalipoprotein deficiency , idiopathic in
breastfed infants, warfarin ingestion
Page 2 of 3
 SEM 1P
HEMATOLOGY

 Prevented by parenteral and high dose oral vit K b. APTT

during malabsorption and cholestasis c. Thrombin clotting time (TCT)
 Dependency on the primary disease d. Fibrinogen level
Late onset disease e. Platelet count
 After 2 weeks of life 2. If with abnormality specific factor assay
 Cholestatic liver disease, pancreatic disease, 3. Bleeding Time
intestinal disorder (celiac sprue, inflammatory a. Shorter than in adults in the 1st week of
bowel disease, short bowel syndrome) life:
 Management: prophylaxis – 1mg vit K1/IM within i. Increased plasma concentration
1hr of birth or 2mg vit K1 orally at birth at 4-6days of VWF
and at 4-6weeks or 2mg of vitK1 at birth and the ii. Enhanced function of VWF
weekly if 1mg for 3 months (IM is preferable) iii. Large RC, high hct
 VDKB: 1-2mg vit K1/ slow IV or SC infusion
 Severe bleeding may require FFP
Prophylactic Vitamin K administration:
 Forms:
o VK1 (phytonadione) – present in leafy
green vegetables
o VK2 (menaquinone) – synthesized in the
intestinal bacterial flora
o VK3 – can cause haemolytic anemia
Recommendations for VK prophylaxis
1. VK – 1-5ug/kg in newborns
2. SD of 0.5-1mg IM or oral dose of 2-4mg at birth
3. Repeated administration of oral VK or continuous
low dose VK supplementation to prevent late VK
deficiency

Hemostatic System
 Begins in utero until after birth
 Functional levels of procoagulants coagulation
inhibitors, fibrinolytic components and platelet-
associated factors differ from older children and
adults
 “adult value” are reached until 6 months of age
Clinical Aspects of Developmental Hemostasis
 Acquired disorders are more common
 Severe forms of congenital factor deficiencies
and platelet disorders in healthy infants who are
bleeding
 15 – 30% of inherited bleeding disorders presents
with bleeding in the NB period
 Oozing from umbilicus
 Bleeding into the scalp, large cephalhematoma,
bleeding after circumcision, phlebotomy sites,
bleeding into the skin, ICH
 Sick infants can bleed from:
o Mucous membrane, bladder, sites of
invasive procedures, joint bleeding (rare)
 Healthy infants: thrombocytopenia 2nd to
passage of maternal antiplatelet antibody, VK
deficiency, congenital coagulation factor
deficiency
Laboratory Examinations
1. Work up for sepsis plus:
a. PT
Page 3 of 3
 PEDIATRICS II
PEDIATRIC GASTROENTEROLOGY
09/22/2020 – Dr. Elino T. Lim
RED – lecture notes (take note of words in bold)
GASTROENTEROLOGY I
GIT
• Starts from the mouth à stomach à intestine à
anus + liver, pancreas, gallbladder, biliary tree
• GI complaints are a significant part of ambulatory
pediatrics
• Determine if symptoms are due to GIT problem or
related to a systemic disease, example:
o UTI, meningitis can present as diarrhea
o Pharyngitis/diabetes can present as
abdominal pain
Abdominal Pain
• Most frequent GI complaint
Pain Types
1. Visceral – vague, poorly localized pain, from
distention of wall
o Stretching of capsule, inflammation,
ischemia
o Dull, aching pain
o Dermatomal
• Site of pain/organs associated:
o Epigastrium – liver, hepatobiliary,
stomach, pancreatic and gastroduodenal
o Periumbilical – small and large intestines,
appendix
o Suprapubic – distal large bowel, urinary
tract, pelvic organs, transverse colon
2. Somatic/Parietal pain – secondary to an inflamed
viscous or peritonitis
o More localized and intense than visceral
o More sharp, constant and localized to
area of viscous
o Abdominal rigidity, muscle guarding,
rebound tenderness
3. Referred pain – extraintestinal locations
o E.g. pneumonia – possible referred
parietal pain to the GIT
• No specific lab tests
o Results may direct away from
intraabdominal cause (misdiagnosis)
o With abdominal pain, you NEED an
impression then ask for laboratory to
confirm
• Acute abdominal pain – usually organic cause
(there is an actual infection)
• Chronic or recurrent – often functional (no
pathology, purely physiologic alteration, not
dangerous)
• Non intestinal causes – needs to be ruled out (r/o)
• Chronic abdominal pain – Irritable Bowel
Syndrome
• Acute abdominal pain – r/o Acute Appendicitis
1.2
Diarrhea
• Increase number or fluid content of stool
o Loss of fluids and electrolytes in stool
• Infants may have 10-12 bowel movements each
day as long as there’s no watery margin around
the stool
nd
• 2 most common cause of death in pediatric
st
patients (1 is pneumonia)
• Infectious and non infectious
• Note: Frequency, consistency, gross blood, color
hyperthyroidism, etc.
• Acute diarrhea <2 weeks
• Chronic diarrhea > 2 weeks
Mechanism
• Secretory – large volume, persist with fasting
o Ex. Cholera à rice watery stool (bacterial
toxin)
• Osmotic – lesser volume, stops on fasting
o Ex. Malabsorption syndrome, lactose
deficiency
• Motility – hyper or hypomotility
o Ex. Irritable bowel syndrome,
pseudoobstruction
• Mucosal invasion or decrease surface area
o Ex. Rotavirus, Shigella, short bowel
syndrome
• Assess degree of dehydration – main problem
o Anterior fontanel (sunken = diarrhea)
o Mucus membrane ( (+) tears or saliva)
o Skin turgor (skin remains wrinkled)
o Pulses (blood pressure goes down, heart
rate goes up)
o Capillary refill and urine output
• Stool analysis for blood, culture and sensitivity,
serum electrolytes
• Acute diarrhea – infectious, overfeeding, food
poisoning, systemic infection, antibiotics
Management
• Replace fluid and electrolyte loss
• Antispasmodic and antidiarrheal is
contraindicated
• Antibiotic must be individualized
Chronic Diarrhea
• Chronicity doesn’t imply organic disease
• Dysmotility with normal absorption
• Chronic infection
• Severe intestinal mucosal damage
• Nutrient malabsorption and diminished growth
• Chronic diarrhea with normal weight and length is
unlikely to have a serious organic cause
• Can lead to failure in pediatric drive
1 of 13
 Vomiting • Infants often hungry after the episode
• Violent contraction of GIT smooth muscle • MC manifestation of gastroesophageal reflux
• Vomiting reflex is effortful (reflux is passive) • Due to incompetent or immature lower
• Emetic or vomiting center – medulla activated esophageal sphincter (normal for infants –
• Bile stained – obstruction below 2nd portion of physiologic regurgitation)
duodenum • Resolves upon maturity
• Common causes: (diagnosis is age specific)
o Infants – gastroenteritis, reflux, Dysphagia
overfeeding, obstruction, systemic • Difficulty in swallowing (disordered swallowing)
infection • Odynophagia – painful swallowing
o Child – gastroenteritis, systemic infection, • Globus – sensation that something is in the
toxic ingestion, pertussis, medication esophagus
o Adolescents – inflammatory bowel • Oral or esophageal – coordinated process
disease, appendicitis, migraine, • Affected by
pregnancy, meds, bulimia o Mechanical obstruction (strictures,
congenital web, tumor, vascular ring,
Non-GIT Causes motility abnormalities)
• Increase intracranial pressure o Neuromuscular disorder (cerebral palsy,
• Inborn error of metabolism botulism, diphtheria, Guillain Barre
• Meds – chemotherapy syndrome)
• UTI
• Pregnancy Anorexia
• Psychogenic • Loss of appetite for prolonged period
• Toxins • Common symptom of many disease processes
• Adrenal insufficiency including GIT
• Hypothalamus – hunger and satiety center
Cyclic Vomiting Syndrome • Eating till “full” à distend stomach and upper
• With numerous episodes of vomiting interspersed bowel à signal sent to sensory afferent and
with well intervals chemoreceptors à stop eating
• 12 episodes per year, each lasting 2-3 days
• “Abdominal migraine” Gastrointestinal Bleeding
• 2-5 years old • Hematemesis
• Not due to another disorder (make sure that there o Vomit fresh blood esophageal (severe
is no other problem) esophagitis or varices), upper GI bleed,
stomach, duodenum
• Triggered by – stress, infection, excitement
• Melena
• DDx – GIT, CNS, endocrine, metabolic disorders
o Dark or black stools
• Choice of Labs – endoscopy, contrast GIT
o Upper GI bleeding (gastritis, ulcer,
radiogram, brain MRI, metabolic studies
swallowed blood)
• Hydration, antiemetics, treat underlying cause o Mild to moderate bleeding above distal
ileum
Complications o Cause major bleeding even in duodenum
• Metabolic • Hematochezia
• Nutritional o Fresh blood in stool
• Mallory Weiss tear – erosion due to protracted o Due to lower GI bleeding (polyp, Meckels
vomiting diverticulum), distal bleeding site or may
• Esophagitis be from upper GIT if increased motility
• Aspiration – when vomiting goes into lungs • Symptoms are age specific
• Shock
• Pneumomediastinum, pneumothorax *Doc showed the table just for reference, he didn’t really
• Petecchial / Retinal discussed it

Regurgitation
• “Spitting up or lungad” – in infants
• Effortless movement of stomach contents into the
esophagus and mouth, no retching
2 of 13
 • Apthous ulcer or canker sores-painful,
circumscribed lesions, recurrences
• Herpetic gingivostomatitis
• Herpangina – painful lesions confined to soft
palate and oropharynx
• Cheilitis / Cheilosis
• Ankyloglossia

Gingivostomatitis (Herpetic)
• Vesicles on mucocutaneous
borders, painful, febrile
• Patient can’t open his mouth

Diagnostic Procedures Cheilitis

• Endoscopy – normally used
o Esophagogastroduodenoscopy
o Capsule endoscopy – swallow of capsule
with camera
• Colonoscopy – lower GI bleeding
• Tag RBC scan – nuclear medicine in the RBC
o Used in Meckel’s diverticulum
• Chemical
o Guiac test for occult (can be seen with the
eyes) blood-hemoglobin
§ Oxidizing potential – blue color
• Apts test – determine if maternal or fetal blood
o 10% NaOH
o Adult Hb – brown; Fetal – pink
Complications
• Anemia
• Shock
• Increased bilirubin from breakdown of blood
(jaundice)
Constipation
• Functional (non-organic) – usually by lack of fiber
• Organic – intestinal
o Drugs (use dietary or fluid management
for pediatrics, shy away from laxatives as
much as possible)
o Metabolic – dehydration, cystic fibrosis,
hypothyroidism, etc.
o Neuromuscular
o Psychiatric – anorexia nervosa
• Note: Stool consistency, frequency, difficulty, age
• Encopresis – fecal incontinence, soiling of
formed/semi-formed stool in underwear in >4yo
MOUTH CONDITIONS
*Doc: I won’t dwell on this too much
• Dental caries, teething
• Oral thrush – esp. immunodeficient and diabetics
• Dryness of lips, cracking, scaling,
burning sensation
• Due to contact sensitivity, vitamin
deficiency, weak immunity,
infection
• Seen in cold weather or high fever
Cheilosis
• Involves angle of the lips
• Fissuring of the lips esp. the
angle of the mouth (bilateral)
• Due to vitamin B2 (riboflavin)
deficiency
Apthous stomatitis (Canker Sores)
• Painful circumscribed ulcers with white necrotic
base surrounded by red halo
• Allergy, immunologic, stress, tissue injury
GASTROENTEROLOGY II
ESOPHAGUS
Achalasia and Chalasia
• (L) Achalasia – lower esophageal sphincter fails
to relax with swallowing (always close)
o Birds beak
o Symptoms include difficulty swallowing
and regurgitation
• (R) Chalasia (Gastroesophageal reflux)
o Transient relaxation
3 of 13
 o Reflux across a dilated lower esophageal • Common in infants
sphincter made worse by increased • Peak at 1-4mos old
abdominal pressure or delayed gastric • Incompetence of lower esophageal sphincter
emptying. • Symptoms – due to regurgitation, esophagitis,
respiratory, neurobehavioral
Cleft Lip and Palate • Tests – scintigraphy, barium, endoscopy, biopsy,
• Cleft lip – failure of fusion of fronto-nasal and manometry (measures the coordination of
maxillary processes swallowing and motility of the intestine)
o More common in boys • Signs and symptoms
o (Unilateral or bilateral) – occur with or
without cleft palate
• Cleft palate – failure of fusion of palatine process
and nasal septum, midline
• Causes:
o Multiple genetic or environmental factors
(maternal drug exposure, etc.)
• Problem – speech or swallowing/feeding problem
– long soft nipple
• Management:
o Surgical closure cleft lip – done by 3mos
old upon demonstrating steady weight
gain followed by closure of cleft palate
before 1yo. (9-12mos old)

Gastroesophageal Reflux (GER – Physiologic)

Management
• All supportive, no definitive management
• Left side supine position (upright positions),
thickened formula
• Pharmacologic
o Prokinetic – Betanechol, Domperidone,
Metoclopramide
• Proton pump inhibitor – most potent anti-reflux
drug acid control
o Cimetidine, Ranitidine, Antacids
• Surgery – fundoplication
• Complication – stricture, esophagitis, Barretts
• Normal in infants (maximum 2y.o) esophagus
• There should be NO signs and symptoms
• If with signs and symptoms à Gastroesophageal Esophageal Atresia / Tracheoesophageal fistula
Reflux Disease • Congenital defect, more common in premature
• Manifestations:
Gastroesophageal Reflux Diseases (GERD) o Maternal polyhydramios
• Return of gastric contents to esophagus o Failure to pass NGT into stomach
• Physiologic or pathologic (increasing times)
4 of 13
 o Excessive oral secretions • Cytoprotectives (sucralfate, misoprostol)
o Choking • H2 blockers
o Cyanosis • Proton pump inhibitor
o Others depend on type of defect • Anti-infective
• Tests – plain x-ray – contrast shows blind pouch, • Triple therapy – PPI + 2 antibiotics (amoxicillin,
bronchoscopy for H type fistula clarithromicin, PPI) – for H. pylori
• Management – surgical correction (no other way) • Endoscopic cautery
• Preop – NPO, prone position, suction, IV fluids • Surgical if with complications
and nutrition
Omphalocoele
Tracheoesophageal Fistula Types
• 5 anatomic types (A to E)

• Abdominal wall defect

• Herniation of content outside of the abdomen,
covered with a membrane (midline)
• Should be differentiated with gastroschisis
o Hole is on the lateral side, no membrane
• Type C (EA with distal TEF) – most common
• Moisten via application of fluid
o DO NOT APPLY DRESSING!
Infant Colic
• Management – surgery
• Spells of irritability, fussing, crying (RULE OF 3)
o > 3 hours a day
Umbilical Hernia
o > 3 days a week
o > 3 weeks
rd
o During 3 month of life
• Peaks at 6-8 weeks old and subsides
• Etiology:
o Nutritional
o Swallowed gas – failure to burp
o Maternal psyche – stressed mother
• Surgical management not necessary – only if with
• Establish the diagnosis, r/o pathologic or organic
strangulation
cause
• Close on its own by 1 year old if there is no other
problem
Gastritis / Peptic Ulcer Disease
• Primary - more common in children
Inguinal Hernia
o 10 yo. – Helicobacter pylori in 10%
• Luslos
• Secondary
• Indirect more common that direct and femoral
o <10yo. – due to stress (sepsis, asphyxia
hernia
– usually due to neonatal problem),
excess acid, drugs • Embryologic – slippage of bowel thru inguinal ring
• Manifestations depend on age group • Males > females, premature
• Tests – upper GI series, UGI endoscopy (gold • Patent processus vaginalis – processus herniates
standard), plain x-ray: for perforation, thru abdominal wall with hydrocoele into canal
angiography: bleeding site, H. pylori testing • Inguinal / scrotal bulges on straining or crying ()on
(hydrogen breath test, stool antigen) the inguinal area (hydrocoele remains same size)
o Inguinal hernia – content is gut
Management o Hydrocoele – no bulging, content is water
• Dietary and health habits • Complications – incarceration (insides decay)
• Acid control agents • Surgical repair

5 of 13
 Irritable Bowel Syndrome • Blocked or closed chloride channel
• Most common cause of chronic abdominal pain • Pancreaticin sufficiency, malnutrition, FTT,
• Affects a very large number of children and adults steatorrhea
(absences) • Complications – hemorrhagic diasthesis, rickets,
• Poorly understood neurologic annormalities
• Triggers – endogenous and exogenous factor
(infection, teething, stress, diet) à GI Dysmotility
• Routine GI evaluation/tests – all normal
o Variations found in electrophysiological
studies
• Characteristics
o Diarrhea or constipation w/ or w/o
abdominal pain
o Infants – episodes of colic or chronic
diarrhea
o Teens – vague abdominal pain, symptoms
may increase with physical or emotional
stress
• No specific manifestation
• Management is non-specific

Inflammatory Bowel Disease

• Genetic susceptibility and environmental triggers
Other GIT Disorders associated with CF
• Peak incidence – preadolescents and adolescents
Ulcerative Colitis
• Typically present with bloody, loose stools with
urgency and tenesmus
Crohn’s Disease
• May present with symptoms ranging from isolated
growth failure and perianal abscess to diarrhea,
weight loss and fever
• (Regional enteritis) – small bowel series may
show narrowing and nodularity of bowel loops,
esp. in terminal ileum
o Inflammation tends to be patchy
*Doc: I won’t dwell on this too much
• Complications – intraabdominal abscess,
intestinal obstruction, bleeding, perforation
• Treatment
o Medical – nutritional rehab, promote
growth and development, psychological
assessment, anti-inflammatory therapy for
mucosal healing (ASA, corticosteroids,
antibiotics, chemotherapeutics, biological)
o Surgical – for complications
Cystic Fibrosis
• Major cause of pancreatic insufficiency
• Life threatening, inherited dse. primarily of whites
• Disease of exocrine glands that causes viscid
secretions
o GIT and pulmonary systems most
severely affected
• Meconium ileus
• Intestinal impaction
• Intussusception
• Rectal prolapse
• Liver disease – neonatal cholestasis, fatty liver,
biliary cirrhosis
• Abnormal gall bladder function and cholelithiasis
Pancreatitis
• Present as epigastric pain accompanied by
elevated serum amylase and lipase
• Usually seen in adults (alcoholic, obesity)
• Acute, chronic, necrotic, hemorrhagic
• Causes:
o Trauma, infection (mumps, mycoplasma),
meds, anatomic variants, systematic and
metabolic diseases and gall stone
• Characteristics
o Sudden epigastric pain (severe), sharp,
constant, RUQ or periumbilical, eating
triggers pain
• Grey Turner Sign – bluish flanks (signs of
hemorrhage_
• Cullen Sign – bluish periumbilical area
• Complications
o Hypocalcemia, hyperglycemia, pseudocyst,
phlegmon, peritonitis
Management / Laboratories
• Elevated serum amylase and lipase – screening
test (this is very high)
6 of 13
 • Abdominal Ultrasound – imaging test of choice, Figure 3. Upper GI
KUB (kidney, ureter, bladder) showing “string
• Supportive management sign” in 4-week old
o Pancreatic rest (no feeding), pain relief infant with pyloric
stenosis
Peritonitis
• Inflammation of peritoneal lining of abdominal
cavity Duodenal Obstruction
• Fever, severe abdominal pain, vomiting • Types – duodenal atresia, annular pancreas,
• Abdominal paracentesis – reveals organism and duodenal webs
leucocytes • Vomiting, billous or non billous
• Primary/Spontaneous – source of infection is • “Double bubble sign” – distention of stomach
outside the peritoneal cavity and duodenum with constricting pylorus between
o Reaches it by hematogenous or lymphatic them
spread
o Most cases are nephrotic syndrome and
liver cirrhosis
• Secondary – from inside the peritoneum
o Rupture of abdominal viscus, spillage of
abscess, trauma or vascular accident
• Manifestation – acute abdomen
o Abdominal rigidity, muscle guarding, direct
& rebound tenderness of the abdominal wall • Treatment – surgical-bypass the obstruction

GASTROENTEROLOGY III Duodenal Atresia

Pay attention on how the defect cause the problem à
easily be able to absorb related information

Congenital Intestinal Obstruction

Pyloric Stenosis
• Most common congenital obstruction of stomach
and intestines (stenosis = narrowing)
• More common in male infants
• Characteristics Jejunal, Ileal or Colon Obstruction
nd rd
o Projectile vomiting 2 -3 week of life Meconium Ileus
o Hypertrophy and hyperplasia of antrum of • Most commonly associated with cystic fibrosis
stomach • Thick meconium is trapped in terminal ileum
o Appear wasted, palpable “olive” (à which • Abdominal distention, vomiting, constipation
causes the stenosis), peristaltic waves
• “Ground glass appearance” in RLQ or
seen in abdomen
calcifications
• Electrolytes; profound hypochloremic metabolic
• Gastrograffin enema (insert something to make it
acidosis
come out) or surgical if refractory
•
• Large gastric bubble with paucity of bowel gas
distal to obstruction
• Differential Diagnosis:
o Chalasia, hiatal hernia, duodenal
stenosis, adrenal insufficiency, inborn
error of metabolism
• Treatment – surgical pylorotomy (Ramstedt
procedure)
• Management – surgical

7 of 13
 o 2% symptomatic
Hirschsprung Disease • May mimic acute appendicitis
(Congenital Aganglionic Megacolon) • Serve as lead point for intussusception
• Most common cause of lower intestinal > During surgery, the appendix is sometimes removed
obstruction in neonates even if without appendicitis à for financial purpose (so
• Uncommon in premature that you won’t go through another set of surgery if you’ll
• (Aganglionic) = Absence of ganglion cells in part have appendicitis)
or whole colon à chronic contraction
• 80% is recto-sigmoid colon Management
• Characteristics • Meckel scan – technitium 99m localizes to gastric
o Constipation or fail to pass meconium mucosa (which is the site of the bleeding)
o Imperforated anus • X-ray and barium no role
o Abdominal distention • Surgical resection
o Billous or feculent vomiting
o “Ribbon like stools” *Doc didn’t dwell on this too much
• Differential Diagnosis: Acquired Obstruction
o Meconium plug • Paralytic ileus
o Intestinal atresia • Electrolyte imbalance
• Uremia
Management • Intussusception
• Barium enema – dye in the proximal megacolon • Cystic fibrosis
• Punch or rectal suction biopsy – gold standard • Roundworm bolus
• Management (is surgery) – laparoscopic single • Foreign bodies
stage endorectal pull thru procedure • Bezoars
• Other operative: Swenson – excise aganglionic • Tumors
portion and anastomose
Intussusception
• Segment of bowel telescopes into another distal
segment
• Most common cause of obstruction between 3
months to 6 years old
• Most often ileocolic or ileoileocolic (ileum enters
ileum à enters colon)
• Aperistaltic area is abutted:
o Diverticulum, tumors, hematoma
• Manifestations:
o Sudden onset paroxysmal colicky
abdominal pain
o Vomiting progress to shock-like
Meckel’s Diverticulum o Bloody currant jelly stools – coagulated
• Blind pouch blood
• Remnant of vitello- • PE – sausage shaped mass (represents the
intestinal duct telescopic movement) right upper abdomen, coil
spring sign
• May lead to
diverticulitis (if • Recurrence is possible
inflamed),
intussusception,
hernia
• Most common complication – painless rectal
bleeding-gastric acid secreting cells
• RULE OF 2
o Present in first 2 years of life
o 2 inches
o 2% of population
o Usually under 2 years old
8 of 13
 Management
• Barium enema – “coiled spring” appearance (for
diagnosis)
• Hydrostatic reduction, if unsuccessful à surgical
• Left untreated à perforation, peritonitis, death
Malrotation of the Intestine / Volvulus
• Non rotation or incomplete rotation of intestine
around superior mesenteric artery during
embryologic development leads to obstruction
and ischemia (blood supply is compromised)
• Non rotation – midgut volvulus
• Incomplete – duodenal obstruction
• Acute or chronic manifestations – obstructive,
abdominal pain, infarction/necrosis/peritonitis
• Corkscrew sign
• Double bubble sign (enlarged stomach and
proximal duodenum with little gas in remainder of
bowel)
• Upper GI series – investigation of choice to detect
volvulus/obstruction
• Treatment – surgery (Ladd’s procedure),
reduction of volvulus
Acute Appendicitis
• Most common cause of abdominal surgery in
childhood
o Must always be considered in children
with acute abdominal pain
o Diagnosis is very important in pediatrics
• Obstruction of lumen, inspissation of mucus,
ischemia of mucosa, bacterial invasion
o Obstructed by fecoliths, calculi
• Periumbilical pain moving over to McBurneys
point (RLQ) or if retrocecal,
o (+) Pain on palpation rectal exam
o (+) Psoas or Obturator sign – signs of
peritoneal irritation
Diagnostics
• Abdominal ultrasound
• X-ray not helpful
• High WBC count, urinalysis (to r/o UTI)
• In teenage girls r/o gynecological problems (OB
Gyne clearance)
• Complications – rupture and peritonitis
• Postop – wound infection and intestinal adhesion
• Appendectomy, IV antibiotics
Necrotizing Enterocolitis (NEC)
• Also known as acute intestinal necrosis syndrome
• Major GIT cause of morbidity in NICU
• Common in NICU
• Risk factors:
o Prematurity – most important risk factor
o Umbilical cannulation
• Roles of infection, enteral alimentation,
mesenteric ischemia, tissue hypoxia
• Signs and symptoms variable
o Feeding intolerance or residuals, to
fulminant intra-abdominal catastrophe
o Triad – abdominal distention, GI bleeding,
air within intestinal wall
*Doc showed this but didn’t discuss it
Bell staging
• I – suspect – non specific manifestations
• IIA – definite manifestations with pneumatosis
intestinalis on X-ray
• IIB – moderate – with systemic toxicity showing
mild acidosis, low platelet, abdominal wall edema
• IIIA – advanced – resp. and metabolic acidosis,
low BP and urine output, DIC
• IIIB – advanced – deteriorating vital signs and lab
indices, shock syndrome, electrolyte imbalance
“Pneumatosis Intestinalis”
Management
• Laboratory
o “Pneumatosis intestinalis” – radiologic
hallmark
o Others are bubbles of layers of gas in the
wall of the bowel as well as portal venous
gas. free air in peritoneum is evidence of
perforation
• Blood, stool, exam
• Medical management
o NPO with nasogastric suction
decompression, IV resuscitation, nutrition,
antibioticsl, surgical referral
• Best way to prevent NEC is avoid premature birth
9 of 13
 Malabsorption Syndromes Bilirubin Conjugation Pathway
• Causes osmotic diarrhea
• Stool studies for fat, carbohydrate, pH, reducing
substances, and alpha one antitrypsin should be
performed
• Specific causes:
o Cows milk protein intolerance
o Celiac disease (gluten sensitive
enteropathy)
§ Autoimmune disorder of proximal
small intestine
§ Characterized by intolerance to
gluten (gliadin) – results in mucosal
damage
§ Usually present bet. 6mos-2yo.
§ Gluten is found in wheat, rye, Hepatitis
barley, oats • Acute hepatic inflammation
o Infectious and non infectious
Short Bowel Syndrome • Primary hepatotrophic virus (A-E)
• Lactose intolerance – congenital or secondary o A and E – transmitted through feco-oral
§ Hepa A – most common cause of
o Deficient lactase in intestinal brush border
infectious jaundice
• This is more common; malabsorption of milk
o B, C, D – percutaneous or mucosal
• Causes sour acidic stool, anal fissure or
exposure to body fluids and vertical
abdominal discomfort
transmission
• Loose, watery, frothy stools following ingestion of o B, C – can cause chronic hepatitis à
milk, with perianal excoriations cirrhosis à carcinoma
• At risk:
o A and E – travel, poor sanitation, contact
with other children from day care
o B, C, D – IV drug user, multiple sex
partners, blood transfusion recipient
• Clinical signs of acute hepatitis
o Low grade fever, jaundice, dark urine,
abdominal pain, diarrhea
o B and C – usually silent (can be
asymptomatic)
• PE – scleral icterus and jaundice, hepatomegaly,
right upper quadrant tenderness (this can’t
GASTROENTEROLOGY IV pinpoint which hepatitis is present)
LIVER AND GALLBLADDER • Clinically similar, serology needed to secure
Cholestasis accurate diagnosis
• Reduced bile flow and characterized by elevation • Serologic Tests:
of direct bilirubin à leading to jaundice o Hepa A – anti-HAV IgM
• Distinguished from ordinary neonatal jaundice o Hepa B – HBsAg, HBcAg, HBeAg, anti-
where direct bilirubin (conjugated form) is never HbS, anti HBc, anti HBe
elevated o Hepa C – anti-HCV ab, HCV-PCR
• Unconjugated hyperbilirubinemia – immature • Only A and B have vaccines
hepatocellular excretory function, hemolysis • If vaccinated with B, you also can’t have D
o If unconjugated à Indirect
hyperbilirubinemia Hepatitis A
• Direct hyperbilirubinemia – elevated in • RNA picornavirus
obstructive, infectious, metabolic, toxic, idiopathic, • Subclinical, anicteric, symptomatic
autoimmune causes • Feco-oral, some percutaneous
• HAV IgM – acute infection
• HAV IgG – previous infection
10 of 13
• Characteristics
o Icteric, nausea, vomiting, malaise,
anorexia, cholestatic jaundice, pruritus
• Signs and symptoms is self limiting, no chronicity
• No therapy indicated
o Almost all patients recover without
fulminant infection or chronicity

Hepatitis B
• DNA hepadnavirus
• Most often parenteral or sexual transmission (ex.
intimate contacts, needle stick exposure,
perinatal)
• HBsAg – acute infection if it goes down
nd
• Anti HBc – 2 marker to confirm diagnosis of
acute infection when HBsAg level wanes
• HBeAg – (enveloped antigen) marker of infectivity
or viral replication
• HBsAg+, antiHBs (-) >6months – chronic carrier
• Serum sickness-like form, Fulminant form Chronic Hepatitis
• No specific treatment • Inflammation of liver > 6months
o Interferon alfa, lamivudine • HBV, HCV, HBD
o Other virus: HIV, CMV, EBV, rubella
Hepatitis C • Drugs – Isoniazid, Nitrofurantoin
• So RNA flavivirus, NANB hepatitis,transfusion • Other metabolic diseases
related hepatitis o Inflammatory bowel disease
• Predominantly parenteral • Chronic persistent – benign, most common form,
• Sexual, perinatal,household contacts self limited
• Anti HCV-infection, not immunity • Chronic active – continuing inflammation
• Chronicity progressing to severe destruction
• Interferon alfa, ribavirin
Liver Abscess
Hepatitis D
• Delta hepatitis; Parenteral • Pyogenic abscess –
• Anti-HDV – highest titers are found in patients from bacterial seeding
with chronic hepatitis D via portal vein or
ascending cholecystitis,
Hepatitis E burns, pyodermas,
• Feco-oral route osteomyelitis
• Solitary and found in right hepatic lobe –
percutaneous aspiration can be done (but only if
solitary)
• Multiple abscess – associated with severe sepsis
• More prone – children on anti-inflammatory,
immunosuppressives & defects in WBC function
• Amebic abscess – rare in children
• (+) Travel to endemic area

Clinical Findings
• Pyogenic – fever, shaking chills, malaise,
abdominal pain, weight loss, jaundice
• Dominant complaint is dull pain over an enlarged
liver that is tender to palpation
• Elevated hemi-diaphragm with reduced/ absent
resp. excursion
11 of 13
 • Resp. symptom – rupture to R chest encephalopathy – first indication of underlying
(consolidation R lower lobe) liver disease
• CBC – leukocytosis, at times, anemia • Hepatosplenomegaly, spider angioma, palmar
• Liver tests – mild hepatitis erythema, clubbing
• Ultrasound liver – most useful test, nuclear, CT • Small shrunken liver, resonant on percussion or
scan slightly enlarged, firm to hard and irregular edge
• Ultrasound guided needle aspiration (antibiotic • Biliary cirrhosis – jaundice, dark urine, pruritus,
can’t be used if there is pus, clean it out first) hepatomegaly, xanthomas, failure to thrive,
undernutrition, steatorrhea
*Doc: I’m not gonna go into this, skipped
Differential Diagnosis Laboratory
• Hepatitis, hepatoma, hydatid cyst, gall bladder • Mild increase ALT, AST
disease, biliary tract infection can mimic liver • Decreased albumin
abscess • Prolonged pro-time unresponsive to vit. K
• Others – subphrenic abscess, empyema, • May be present despite normal blood test
pneumonia • Hepatic abscess or CT – abnormal hepatic texture
• Complications – spontaneous rupture of abscess and nodules
with extension, bronchopleural fistula, secondary • Biliary cirrhosis – abnormalities of biliary ducts
bacterial infection of amebic abscess, metastatic
hematogenous spread to lungs and brain Complications
Treatment • Nutritional
• Ultrasound or CT guided percutaneous needle • Hormonal
aspiration, catheter for drainage • Portal hypertension
• Antibiotics (esp. multiple abscess) • Hepatocellular CA
• Surgical – rupture, enterohepatic fistula • Liver failure

Cirrhosis Management
• No proven treatment but with identification of
• Histologic defined primary cause or offending agent, progression
condition of liver may be altered – immunosuppressives –
characterized by autoimmune
diffuse hepatocyte • Surgical correction – biliary tract abnormalities
injury and
• Liver transplant – continuing or unmanageable
regeneration
Gallbladder Diseases
• Characteristics:
• Rare in pediatrics
o Bridging fibrosis, disorganized lobular and
• Cholelithiasis and cholecystitis
vascular architecture (regenerative nodules
o Relatively rare in healthy children
– macro or micronodular)
o Cholelithiasis most commonly seen in
o Portal hypertension
hemolytic anemias
o Liver disease progress to fibrosis, insidious,
o Cholesterol cholelithiasis commonly affects
may be with no icteric phase
§ Obese adolescent girls
• In children, most common are:
§ With impaired enterohepatic
o Post-necrotic (giant cell, viral, autoimmune,
circulation of bile acids
drug induced)
• Right upper quadrant pain with food intake
o Biliary (congenital duct anomalies and
tumors, paucity of intrahepatic bile ducts, • Abdominal ultrasound
cystic fibrosis, sclerosing cholangitis) • Management – Cholecystectomy & antibiotics
• Parasites – Opistorchis, Fasciola, Ascaris
Biliary Atresia
• May be active or quiescent with normal liver
function tests • Progressive fibrosclerotic disease affecting
extrahepatic biliary tree
Clinical Manifestation • Etiology – unknown
• Non-specific • Present with jaundice, dark urine, pale or acholic
• Malaise, loss of appetite, failure to thrive stool (white color)
• Ascites, GI hemorrhage or hepatic • Rapid progression with bile duct obstruction at
12 of 13
 4mos age
• Hepatosplenomegaly, ascites, poor growth, ANORECTAL ABNORMALITIES
steatorrhea Imperforated Anus
• No anal opening

Atresia or Stenosis
• Low (infralevator) – intestine terminates below
pelvic floor, anal site with skin tag. anus covered
by skin
Management:
o Mgt – excise cover and anal dilatation
• Abdominal ultrasound
• High (supralevator) – intestine terminate above
• Radionuclide imaging (ex. HIDA Scan – shows pelvic floor, either blind or w/ fistula
non-visualizing gallbladder, suggestive for biliary
o Mgt – transverse colostomy and elective
atresia)
pull through 6-12 mos. later
• Liver biopsy
• Intraoperative cholangiogram with laparotomy – Clues for Anorectal Malformations
examine biliary tree confirmatory • Clues – failure to pass meconium
• Kasai portoenterostomy – damaged bile duct • Perineal inspection
remnant is removed and replaced with a loop of
• Digital exam
jejunum
• VACTERL Syndrome
o Must be done before 3 months old
o Vertebral defects
o Initial but just a temporizing management
o Anal atresia
• Liver transplant (definitive), supportive, nutritional o Cardiac defects
o Tracheo-esophageal fistula
Choledochal Cyst o Renal anomalies
• Bile collects in the extra pouches à jaundice o Limb abnormalities
• Dilatation of the biliary system which may affect Laboratory
extrahepatic and intrahepatic bile ducts • Plain X-ray sacrum
• Unknown etiology • Abdominopelvic UTZ
• Characteristics: • Pass NGT
o Weakness of wall of bile duct, distal • 2D Echo
obstruction
o Infants – obstructive jaundice, pale stools, Anal Fissures
palpable mass right hypochondrium
• Perianal fissures/ulcers are the most common
o Older children – intermittent mild
cause of non-massive bright red anal bleeding in
jaundice, abdominal pain, palpable mass
children
• Tests
• Blood streaked stools – caused by constipation
o Abdominal Ultrasound
• Large stool or straining
o DISIDA scan
o CT/MRI scan • Skin tags
o Barium meal • Management
o ERCP o Ensure soft stool (dietary, behavioral,
• Treatment – surgery à excise cyst and stool softeners. increase water intake
hepaticojejunostomy o Sitz bath (for older children) – sit down in
a bucket of warm water à cause
vasodilation of vessels and promote
circulation

13 of 13
 PEDIATRICS II 1.4
NEWBORN (SYNCHRONOUS)
10/20/2020 – Dr. Cabarles

RED – Lecture 3 Fetal Shunts

TOPIC OUTLINE • Located intracardia (DA
- Fetal / Newborn Physiology & FO)
- Newborn Assessment • Shunt – opening /
- Common neonatal problems communication bet. two
chambers
FETAL / NEW BORN PHYSIOLOGY • Ductus arteriosus –
• Fetal physiology is quite distinct from the neonate, bet. pulmonary artery &
both structural and functional aspects arch or aorta
• Developing fetus has unique physiologic needs • Foramen Ovale – bet
right & left atrium
• Fetus is well-adapted to the relatively hypoxemic
intrauterine environment • Ductus venosus – bet.
umbilical vein and IVC
• Transition from intra- to extrauterine life requires
rapid, complex and well-orchestrated steps to
ensure neonatal survival Scheme of Fetal Circulation

FETAL CIRCULATION
• Begins when the heart first beats at approximately
22 days of gestation
• Gas exchange
o Initially provided by yolk sac and placenta
o Until the placenta becomes dominant at
10 weeks’ gestation
• Why does the fetus thrives in a hypoxemic
environment?
o Because oxygenated maternal blood
mixes with poorly oxygenated blood
within the free-flowing placental space
o Oxygen content of blood provided to the • Trace of blood:
fetus is lower than the maternal uterine o Where oxygenated blood comes from –
arterial blood placenta
o In IVC – mixture of oxygenated and
deoxygenated blood
Lun 65 o Preferential flow of blood
§ Oxygenated blood in the right
atrium shunted through the patent
FO à body
§ Unoxygenated blood in the r.
HEART
atrium à r. ventricle à aorta
LIVER
(doesn’t include lungs bec. not
yet functional) à internal iliac
arteries

PLACENTA
• As fetal lungs do not contribute to intrauterine
oxygenation, there are several intrauterine shunts
designed to direct blood away from the fetal lungs
Pulmonary Development
• 2nd trimester → formation of the gas-exchanging
portions of the airway
• 24 weeks → alveolar ductal development
o Production of surfactant
• 36 weeks’ gestation → septation of the air sacs
• During both phases of development
o Distal pulmonary epithelial cells – actively
secrete a chloride-rich fluid into the
bronchial tree
o Results in accumulation of fluid within the
fetal airways

1 of 11
 o Compared to postnatal lungs: fetus’ lungs
are hyperexpanded
• Elevated intrapulmonary vascular pressures as a
result of fluid distension contribute to increased
pulmonary vascular resistance
• Low oxygen à pulmonary vessels constrict à
increase pulmonary vascular resistance
o Contributory is the fluid distention brought
about by the chloride rich fluid
Fetal Physiology: Unique Characteristics
Right-to-Left Shunts
• Intracardaic shunts
o Foramen ovale
o Patent ductus arteriosus
• Relative hypoxemic environment
• High-resistance, low-flow pulmonary circulation
• Pulmonary epithelial cells actively secrete chloride
à leading to fluid accumulation w/n fetal airways
• Fetal erythropoiesis – occurs in liver until 3
rd
trimester when transitions to bone marrow
o Initial site: yolk sack à liver à BM
o Potent stimulus: hypoxemia
o Reason why CBC hemoglobin of
newborns range from 16-18
• Fetal hemoglobin, allowing for oxygen uptake in
the lower oxygenated placental vascular bed
T OXYGEN AFFINITY
BIRTH PROCESS
• At the time of birth, multiple changes occur to
transition from fetal circulation to infant circulation
• Structures unique to fetal circulation are no longer
necessary and undergo changes to reflect this
Circulation After Birth
• Changes occur in:
o Pulmonary circulation (lungs functional)
§ Lungs expand
§ Pulmonary vessels dilate
§ Low-resistance, high-flow
pulmonary circulation
o Systemic circulation (placenta removed)
§ Increased
• Resulting in closure of: foramen ovale, ductus
arteriosus, and ductus venosus
o Primary stimulus for closure:
yr AFTER BIRTH
o FO – once left atrial pressure becomes
greater than the right
o DA – response to increased oxygen
§ Reason why premature babies
are prone to develop patent DA
(primary problem is pulmonary)
3- 7 DAYS
o DV – as soon as the cord is clamped
Major Physiologic Changes At Delivery
• Elimination of placenta
• Initiation of respiration
• Initiation of neonatal/adult circulation
NEONATAL CIRCULATION
• At birth, fetal circulation must immediately adapt
to extrauterine life
o Gas exchange is transferred from
placenta to lung
• Onset of spontaneous respiration → expands the
lung and brings O2 to pulmonary alveoli
• With elimination of placenta → increases SVR →
stimulates baroreceptors → slow heart rate
Pulmonary
• 1st weeks of life
o Remodeling of pulmonary vasculature
o Thinning of vascular smooth muscle
o Recruitment of new vessels
• Decrease in PVR influences the timing of clinical
appearance of many congenital heart lesions
• Left to right shunt through a VSD may be minimal
in 1st week after birth, when PVR is still high
2 of 11
 • Umbilical arteries → median umbilical ligament • LA pressure becomes higher than RA pressure,
• Left umbilical vein → ligamentum teres of liver functional closure of FO
• Ductus venosus → ligamentum venosum • When SVR > PVR, DA functionally closes
• Ductus arteriosus → ligamentum arteriosum • Establishing neonatal adult “in series” circulation

Persistent Fetal Circulation Physiology of Birth

• Or Persistent Pulmonary Hypertension • First breath causes rapid expansion of lung
• In the presence of certain stimuli • Molecular pumps rapidly pull fluid out of airspace
o Pulmonary arterioles constrict à lead to through osmosis and into interstitium
increase in Pulmonary Vascular • Oxygen causes rapid dilatation of pulmonary
Resistance (PVR) arteries, decreasing pulmonary vascular
• These stimuli include: resistance
o Hypoxia • PDA and patent FO close over first 1-2 days
o Hypercarbia • Differences in vascular resistance results in
o Acidosis transformation from fetal to adult circulation patter
o Cold • Sympathetic nervous system increases systemic
• ↑ PVR favors right to left shunt and foramen ovale vascular resistance
and ductus arteriosus subsequently remain patent
Absence of a placenta for gas exchange à can
•
lead to a catastrophic outcome in the newborn
NEW BORN ASSESSMENT
ESSENTIAL NEWBORN CARE (ENC)
• Time- bound care (1st 90 minutes of life)
• Done to majority who DO NOT need resuscitation

4 Core Steps of ENC

1. Immediate drying
2. Skin-to-skin contact
3. Proper cord clamping and cutting
4. Non-separation of newborn and mother

Immediate and Thorough Drying

• Immediate drying:
Important Physiologic Changes During Transition to o Stimulates breathing
Extrauterine Life o Prevents hypothermia
• Increased systemic vascular resistance with • Hypothermia can lead to
separation from the low-resistance placental o Infection
vasculature o Coagulation defects
• Closure of right-to-left shunts o Acidosis
o Foramen ovale – closes when left atrial o Delayed fetal to newborn circulatory
pressure greater than right atrial pressure adjustment
o Ductus arteriosus – left-to-right flow within o HMD
mins. of ventilation à closure over days o Brain hemorrhage
• Rapid lowering of pulmonary vascular resistance • If baby is not breathing, STIMULATE by DRYING!
with onset of ventilation • Do not ventilate unless the baby is floppy/limp and
• Clearance of fluid from airways via active sodium not breathing
absorption and changes in airway pressure due to • Routine suctioning NOT recommended unless the
ventilation mouth/nose are blocked
• Increased metabolic rate à leading to higher
glucose needs Skin-To-Skin Contact
• Increased catecholamine levels to support blood • Mother-baby bonding
pressure • Other benefits:
o B – Breastfeeding success
What Happens After the First Breath? o L – Lymphoid tissue system stimulation
• Lung expansion: increase in oxygenation o E – Exposure to maternal skin flora
• Decrease in PVR: increase in blood flow to PV-LA o S – Sugar (protection from hypoglycemia)
o T – Thermoregulation
3 of 11
 Properly Timed Cord Clamping
• Reduction in anemia I-
3 MINUTES AFTER BIRTH

o 80% in term newborns

o 51% in preterm newborns
• Reduction in brain hemorrhage by 41% in preterm
• No significant impact on incidence of post-partum
hemorrhage in the mother
o When do we clamp? – as soon as
pulsation stops

Non-Separation for the Early Initiation

of Breastfeeding
• Early first breastfeed seems to help with
increased milk supply in the first few days of life Rapid Aging Following Delivery Using
o Earlier passage of meconium 5 Physical Parameters
o Greater likelihood of continued
• Hair
breastfeeding
• Ears
• Breast
What Newborn Care Practices In The Delivery Room
Should No Longer Be Continued? • Genitalia
• Manipulation • Plantar creases
o Such as routine suctioning of secretions if • Note: Skin is the least helpful
the baby is crying and breathing normally
o Doing so may cause trauma or introduce Route Care
infection • Prophylactic eye care against Gonococcal
• Putting the newborn on a cold or wet surface infection
• Wiping or removal of vernix caseosa if present o 1% Silver nitrate drops
o Some studies showed it helps in o 0.5% Erythromycin ointment – drug of
thermoregulation choice
• Foot printing o 1% Tetracycline ointment
• Bathing earlier than 6 hours of life o 2.5% Povidone-iodine solution
• Unnecessary separation of newborn, primarily for
Prophylaxis for VKDB
o Weighing
o Anthropometric measurements • Parenteral Vitamin K (for clotting factors 2, 7, 9,
o Intramuscular administration of vitamin K 10) – to prevent hemorrhagic disease
o Hepatitis B vaccine and BCG vaccine o 0.5mg if <1500gms
• Transferring of the newborn to the nursery or o 1.0mg if >1500gms
neonatal intensive care unit without any indication
Order of Examination
EXAMINATION OF NEWBORN • General appearance
• APGAR Score • Head and neck region, Trunk, Extremities
o Appearance (color) • Neurologic exam
o Pulse (heart rate) • Head circumference and length
o Grimace (reflex irritability) • Genitalia / Anal patency
o Activity (muscle tone) • Eye exam
o Respirations
• Done at 1, 5 mins Newborn Examination
o Every 5 until 20min or two successive • Done within 24hrs after birth
scores of 7 • Purpose: To determine overall well being and
• Later scores of 0-3 at 10, 15, 20 min have higher normality
correlation with future neurological handicaps but • Includes the following:
still not specific to diagnose asphyxia in the o Vital signs
absence of other clinical evidence of o PE
encephalopathy o Neurological exam
o Estimation of gestational age

4 of 11
 Vital Signs Gestational Age
• Temperature • Preterm < 37 wks
o Should be taken in axillary area • Late preterm 34-36 wks
o Normal range: 36.5-37.2°C • Full term 37-42 wks
o Axillary temp is 0.5-1°C lower than rectal • Postterm > 42 wks
• Heart rate
o Should be obtained by auscultation and Birth Weight
counted for a full minute when infant is • LBW <2500g
not crying or moving vigorously • VLBW <1500g
o Normal: 120-160 beats/min
• ELBW <1000g
o Maybe <100 beats/min when asleep
• Respiratory rate
Appropriateness of Weight for Gestational Age
o Normal: 40-60/min
• AGA – appropriate for gestational age
o Obtained by observation for a full minute
o Newborns have periodic breathing • SGA – small for gestational age
• Blood pressure • LGA – large for gestational age
o Not measured routinely, only when
suspecting cardiac problems/critically ill Weight Percentiles
§ Coarctation of aorta
o Normal values vary with gestational and
postnatal ages
o BP in upper extremities is higher than
lower ex.
• Capillary refill time
o Normal: <3 seconds over the trunk
o Maybe as long as 4sec. on extremities

Physical Examination
st
• 1 examination in DR or ASAP after delivery
•
nd
2 and more detailed after transition • Lubchenco Chart
•
rd
3 Discharge examination • AGA – 10th-90th percentile for GA
• SGA – < 10th percentile for GA
Measurements • LGA – > 90th percentile for GA
• Weight
o For FT at birth: 2.6-3.8kgs COMPLETE PHYSICAL EXAMINATION
o Babies < 2.5kgs are considered LBW OF THE NEWBORN
o Normal: lose 5-10% of birth weight few *check appendix for table
days after birth and regain it by 7-10 days
(Physiologic weight loss: caused by Key Messages
diuresis, expulsion of meconium, & • Complete newborn examination – tool that
withholding of water and calories) identifies danger signs that threaten the life of
o Weight gain: 15-20gms/day newborn
• Length • Examination should be thorough, systematic and
o Crown to heel length complete from “head to toe”
o Normal range: 48-52cm • It is critical to know normal newborn behavior in
• Head circumference order to recognize abnormality and correctly
o Measure the most prominent parts of the prescribe further tests and/or treatment
occiput and frontal bones • A routine newborn examination is performed at a
o Acceptable values at birth in term NB: time convenient for the newborn, the parents and
33-38cm the health worker
• Environment influences the baby’s behavior
CLASSIFICATION OF NEWBORN during assessments
• By gestational age
• By birth weight
• By appropriateness of weight for gestational age
• By weight percentiles
5 of 11
COMMON NEONATAL PROBLEMS • Since the eye on the affected side cannot be
• Birth injuries closed completely, it is covered with an eye shield
to prevent drying of the conjunctiva and cornea.
• Respiratory problems
• Disorders related to infectious process
Erb’s Palsy (ERB – Duchenne Paralysis)
o Sepsis
• Associated with stretching or pulling head away
o Necrotizing enterocolitis
from shoulder during delivery
• Hyperbilirubinemia
• Signs:
• Disorders related to maternal condition
o Flaccid arm
o Infants of diabetic mothers
o Elbow extended
o Hand rotated inward
BIRTH INJURIES
o Moro and grasp reflexes absent on
• Head injuries affected side
o Caput succedaneum
o Cephalhematoma
• Fractures
• Facial paralysis
• Erb’s / Brachial palsy

Head Injuries
• Caput Succedaneum
o Edema of the scalp at the neonate’s
presenting part of the head
o Often appears over the vertex of the head
as a result of pressure against mother’s
cervix during labor RESOLVES OVER 3 -
4 DAYS
o Edema crosses the suture lines
• Cephalhematoma
o Collection of blood between periosteum of
a skull bone and the bone itself
o Occurs in one or both sides of the head
o Swelling is not present at birth, rather it
develops w/n the first 24-48hrs after birth
o Has clear edges that end at the suture
lines
RESOLVES OVER 9- 4 WEEKS

Fractured Clavicle
• Bone most frequently fractured during delivery
• Associated w Cephalopelvic Disproportion (CPD)
• Signs: ⑧ KLUMP KÉ 's

o Limited ROM
o Crepitus RESPIRATORY PROBLEMS
o Cries of pain when arm is moved Respiratory Distress Syndrome (RDS)
o Absent Moro reflex on Affected side • Also known as Hyaline Membrane Disease (HMD)
• Heals quickly, handle gently, immobilize arm, • Most common cause of preterm neonatal mortality
eliciting scarf sign is contraindicated • RDS related to gestational age and birth weight
• Any newborn that weighs more than 3855g and is GESTATIONAL AGE PERCENTAGE
delivered vaginally should be evaluated for a Less than 28 weeks 60-80%
fractured clavicle 32-36 weeks 15-30%
37-39 weeks 5%
Facial Palsy
• From pressure on facial nerve during delivery Etiology and Pathophysiology
• Affected side unresponsive when crying • Primary cause: surfactant deficiency
• Resolves in hours/days • Low levels of surfactant cause high surface
• Feedings may be given by gavage in order to tension
prevent aspiration

6 of 11
• High surface tension → makes it hard to expand
the alveoli
• Tendency of affected lungs to become atelectatic
at end-expiration when alveolar pressures are too
low to maintain alveoli in expansion
• Lead to failure to attain an adequate lung inflation
o Betamethasone 12mg IM every 24hrs for
two days (better, but hard to find locally
and is more expensive), or
o Dexamethasone 6mg IM four doses at an
interval of 12hrs
• Surfactant replacement therapy (intra-tracheal) IF BATED
lntu

and therefore reduced gaseous exchange • Acid-base parameters should be monitored

• Unmonitored oxygen levels may lead to
Clinical Manifestation retinopathy of prematurity to oxygen toxicity
• Tachypnea
• Nasal flaring Transient Tachypnea of Newborn
• Intercostal, sternal recession
• Grunting; closure of glottis
during expiration
• Cyanosis

• These symptoms may begin at birth or within 6

hours of birth
•
Chest X-Ray

• Bell-shapedthorax
• ↓ Lung volume
• Air bronchogram extended
beyond cardiac border
• Absolutely opaque lung
(whiteout lung)

Risks
• Risk Factors
o Prematurity
o Maternal diabetes • Wet lung
o Multiple births • Respiratory distress shortly after delivery
o Elective cesarean section without labor • Term babies
o Perinatal asphyxia • Due to retained lung fluid
o Cold stress o Cesarean section
o Genetic disorders o No vaginal squeeze
• Decreased risks when: o Decreased function of pulmonary
o Chronic intrauterine stress capillaries and lymphatics
o Prolonged rupture of membranes
o Antenatal steroid prophylaxis Congenital Pneumonia
• Acquired trans placentally or perinatally
L/S Ratio • Accounts for >50% of cases of RD in the new
• Pulmonary immaturity of the fetal lungs can be born
assessed by determination of lecithin/ • Risk factor:
sphingomyelin ratio in the amniotic fluid o PROM (>18hrs)
• 2 or more suggestive of adequate lung maturity o Prolonged labor (> 24hrs)
• Ratio of less than 1.5 is often associated w HMD o Unclean vaginal examinations
o Foul smelling amniotic fluid
Management o Maternal fever
• Premature labor should be arrested by • Causative Organisms:
appropriate tocolytic therapy to gain pulmonary o Group B streptococcus
maturity o Gram negative organisms:
• When premature labor below 34 weeks of § E.coli
gestation is unavoidable, mother should be given § Klebsiela
7 of 11
 § Pseudomonas Clinical Features
§ Staph aureus • Respiratory distress – onset soon after birth if
§ Listeria monocytogene mother is with meconium stained amniotic fluid
• Clinical Manifestations • Hyper inflated chest
o Respiratory distress soon after birth • Meconium stained skin and cord
o Recurrent apneic attack • Urine may appear dark and brown
o Often asphyxiated and sick at birth
o Prolonged capillary filling time Chest X-Ray
o Hypothermia
o Cough is rare • Hyperinflation
• Bilateral fluffy shadows
Investigations
• Evidence of air leak
• CBC, esp. ANC
• Gastric aspirate for PMN
• Blood culture
• CXR (infiltrates, lobar Management
consolidation, interstitial • Prevention of meconium aspiration
reticular opacities) • Prevention of Intrauterine hypoxia
• Postnatal suctioning
Management o Thick meconium:
• Basic supportive therapy § Direct laryngoscopy & tracheal
• Specific suction
o Empirical broad spectrum antibiotics – § Stabilize the baby
Penicillin/Ampicillin § Stomach wash
§ Penicillin 100mg/kg in 2 divided § Work up for sepsis
doses § Antibiotics can be started
§ Gentamicin 4mg/kg q24hr or 2.5 o Thin meconium
mg/kg q12hr § Depressed baby – do everything like
thick meconium
Meconium Aspiration Syndrome § Active baby – no tracheal suctioning
• Respiratory distress in newborn infants born and need close observation
through meconium stained amniotic fluid whose
symptom cannot be otherwise explained DISORDERS RELATED TO INFECTIOUS PROCESS
• Incidence: Necrotizing Enterocolitis
o 10-15% of births – meconium stained • Characterized by necrosis of intestinal wall
amniotic fluid • Serious life threatening condition that is being
o 5% – meconium aspiration pneumonia diagnosed with increasing frequency in premature
↳§ 30% Require mechanical ventilation infants
§ 3-5% Expire ← • Factors that place the infant at risk of this disease
include:
Risk Factors o Perinatal asphyxia
• Placental dysfunction o Low apgar score
• Fetal hypoxia o IRDS
• Ante partum hemorrhage o Sepsis
• Post maturity or SGA o Enteral feedings
• Listeriosis o Congenital cardiac disease
• Breech delivery o Relative ischemia of the intestinal tract
that is due to hypotension
Pathophysiology o Use of umbilical catheters
• Fetal hypoxia – peristalsis and intra uterine o Exchange transfusion
passage of meconium
• Meconium stained amniotic fluid
o Meconium aspiration
o Peripheral & proximal air way obstruction,
inflammatory & chemical pneumonitis

8 of 11
 Clinical Manifestations
• Abdominal distention
• Decreased bowel sounds
• Poor feeding
• Increased gastric residuals
• Blood streak bile vomiting
• Bloody or mucoid stools
Neonatal Sepsis
• Early onset: usually happens in first 72hrs of life
o From birth to 7 days
o Mainly due to organisms present in the:
§ Genital tract
§ In the labor room or in the OR
• Late-onset:
o From 7-28 days
o Caused by the organisms thriving in
external environment
o Infection often transmitted by care givers
DISORDERS RELATED TO MATERNAL CONDITION
Infants of Diabetic Mothers (IDM)
Clinical Manifestation
• Large for gestational age
• Very plump and full faced
• Abundant vernix caseosa
• Plethora
• Listlessness and lethargy
• Large placenta and umbilical
cord
Management
• Careful monitoring of serum glucose levels
• Observation for accompanying complications
such as RDS
o Studies confirm that maintaining blood
glucose level >50mg/dl in IDMs with
hypoglycemia prevent serious
neurological conditions
• Oral and IV backup may be titrated to maintain
adequate blood glucose levels.

Clinical Manifestations ⑧ HYPER BILIRUBIN EM 1A

• Often vague and nonspecific demanding high > 4mg 1dL
index of suspicion for early diagnosis PEDI A 5

• Any alteration in feeding patterns

• Active baby suddenly becoming lethargic
• Hypothermia in preterm and fever in term babies
especially in association with gram positive
infections and meningitis
• Diarrhea, vomiting and abdominal distention
• Jaundice & hepatosplenomegaly may be present
• Episodes of apneic spells with cyanosis

9 of 11
 PEDIATRICS II 1.4
NEWBORN (SYNCHRONOUS)
10/20/2020 – Dr. Cabarles

SPECIFICS USUAL FINDINGS VARIATIONS ABNORMALITIES

HEAD
- Symmetrical
- Molded – with vaginal birth - Severe molding
- Round – if cesarean birth - Asymmetry due to molding - Depressed fontanel (dehydration)
- Palpable anterior and posterior - Scalp lesions or abrasion - Full bulging fontanels (intracranial
fontanels and sutures - Cephalohematoma pressure)
Shape - Palpate the Anterior fontanel (can be - Caput succedaneum - Depressed skull fracture
expected to be slightly depressed when - Posterior fontanel may be difficult to - Unusually large or small head in
child is in sitting position) palpate if sutures are overriding relation to body size
- Sutures are normally felt as ridges overriding of the sutures (observe) - Unusual hair pattern or texture
immediately after birth or as - Subgaleal hemorrhage
depressions within a day
EYES
- Correct placement on face in relation
Symmetry - Centered or deviated to right or left
to one another
- Edema
Appearance - Blink reflex present
- Fused < 25 weeks approx
Discharge - None - Purulent
- Hemorrhage
Sclera - Bluish white
- Jaundice
EARS
- Preauricular papillomas (ear tags)
- Well-formed
may be present
- Cartilage present - Malformations
Shape - Amt. of cartilage varies (less with
- Upper part of ear should be on same - Low placement
prematurity)
plane or above angle of eye
- May be folded or creased
NOSE
Symmetry - Midline of face - Deviated to the right or left
Shape - Appears flattened - Malformation or unusual flattening
- Should breathe easily through nose - Some mucous present in nares may - Flaring of nares
Patency
when mouth is closed interfere with breathing - Stenosis of naris (choanal atresia)
MOUTH
Lips - Pink, Rooting reflex - Transient circumoral-cyasnosis - Cleft, Persistent cyanosis
- Trush, Protrusion
- Pink
- Frenulum linguae (tongue tie) – may
Tongue - Positioned inside mouth
interfere with sucking
- Normal size
- Large and thick
Palate - Pink and well formed - Epstein’s pearls - Cleft
- Rear gums whitish
- Can appear jagged
Gums - Pink
- Teeth can be present or inclusion
cysts (whitish tooth-like cysts)
NECK
- Short - Masses (cystic hygroma)
Appearance
- Straight - Distended veins or edema
- Webbing, Congenital torticollis,
Motion - Unrestricted range of motion
Opisthotonus
CHEST
Shape - Almost circular - Barrel
- Straight - Fractures (crepitations, tenderness,
Clavicles - Depressed sternum
- Smooth palpable mass)
- Symmetry of movement with - Asymmetrical movements (e.g.
Expansion
respirations diaphragmatic hernia, pneumothorax)
- Laboured breathing
- Grunting on expiration
Respirations - Rate 40-60/min
- Retractions with respirations
- Tachypnea
Breast - Excessive amount of breast tissue
- Present in both sexes
Tissue - Breast engorgement
Nipple - Symmetrical placement of nipples - Presence of super numerary nipples - Asymmetrical placement of nipples

10 of 11
CIRCULATION
- Rate 100-160 bpm - Heart sounds heard on right side
Heart Rate - Rate increased following physical or (dextrocardia)
emotional stimulus - Tachycardia
Capillary
- Less than 3 seconds - > 3-4 seconds (poor perfusion)
Refill
- Persistent
- Intermittent during first few days of
Murmur - Absent - Muffled heart sounds
life
- Extra sound
- Absent or weak
Pulses - Equal bilaterally - Bounding (PDA)
- Upper > lower (coarctation)
ABDOMEN
- Asymmetry
- Distention
- Contour cylindrical and relatively
- Gastroschisis, Bladder extrophy
Shape prominent
- Localized bulging (e.g. hernia)
- Soft
- Scaphoid abdomen (e.g.
diaphragmatic hernia)
- Abnormal redness
- Bluish white
Umbilical - Umbilical hernia may be present and - Bleeding
- 3 vessels
Stump is usually insignificant - Foul smelling
- Dry within several hours after birth
- Omphalocele
Bowel
- Transient in first 24hrs - Intermittent - Hyperactive
Sounds
ANUS
- Normal position
Patency - imporforated anus
- Dimpled/Puckered appearance
EXTREMITIES
- Acrocyanosis may last for several - Difference in color or temp. between
Color
hours after birth the extremities
- Limitation of movement in any joint
(e.g. fractures, paralysis)
- Generally flexed but can be passively - May retain in utero position when
Appearance - Presence of defect or missing parts of
put through a full range of motion sleeping
all extremities (club feet, webbing,
palmar simian crease, extra digits)
SKIN
- Hemangiomas, Lanugo, Milia, Vernix - Pallor
caseosa, Peeling, Birthmarks, - Jaundice in 1st 24hrs of life central
- Pink
Appearance Mongolian spots, Petechiae (rapid cyanosis (e.g. cardiac, neurological or
- Varies w race and ethnic origin
delivery), Meconium stained, respiratory problem)
Erythema, Bruising - Pustules, Abrasions, Lacerations
GENITALIA
FEMALE
- Labia minora quite prominent and
protrude over labia majora - Excessive vaginal bleeding
MALE - Malformations (e.g. epispadias,
Appearance - Prepuce usually adherent to glands - Undescended testicles on either side hypospadias, phimosis)
- Testicles usually in scrotum - Hydrocele
- Scrotum small and firm or fairly loose, - Ambiguous (both male/ female)
relaxed and pendulous
- Meatal opening appear as a slit
SPINE
- Malformations (e.g. spina bifida)
- Pilonidal dimple over coccygeal area - Abnormal curvature of spine
- Spine straight, closed and easily
Appearance - Lanugo over shoulders and back, - Pilonidal cyst or sinus
flexed
especially in preterm infants - Tufts of hair anywhere over the spine,
esp. the sacrum (e.g. spina bifida)

11 of 11