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Pedia April30
Pedia April30
Coagulation Cascade
● Series of steps that are activated so that there will be stabilization
of the platelet plug
● When there is damage to blood vessels → chemical signals sent
by the bloodstream → first clotting factor that would be activated
is the factor 12. Like lock and key mechanism that would activate TYPES OF HEMOPHILIA
the factor 11 → factor 9 → factor 8 → factor 10 → factor 5 →
A B C
thrombin → fibrinogen
○ The fibrinogen, when it is divided into several It is the most common It is the second most It is a mild form of
components, it becomes fibrin type of hemophilia common type of hemophilia
○ Fibrin is responsible for making the platelet plug stable (severe) hemophilia (moderate) (mild)
(added layer of security for our platelet plug)
● If one of the clotting factors would decrease or would be missing X-linked recessive X-linked recessive Autosomal recessive
on the cascade then there is no fibrin that would result in that disorder (only males will disorder (only males disorder (need 2 copies
cascade. manifest s/sx) will manifest s/sx) of the gene for disorder
○ Sometimes, on the thrombin level, it would already to manifest; affects both
stop → nothing will make that plug stable causing the males & females)
uncontrollable bleeding in hemophilia It is also known as It was originally named Deficiency of factor XI
factor VIII deficiency or “Christmas disease”.
classic hemophilia Caused by factor IX
deficiency
80% of the cases would
be the classic The first person to have
hemophilia this disease is Stephen
Christmas → christmas
disease
Hemophilia
CLINICAL MANIFESTATIONS
● Has a missing clotting factor depending on the type of hemophilia
● Children usually do not manifest s/sx until after 6 months of age (begin
● X-linked recessive disorder
moving around, losing teeth)
○ X chromosome would be affected that would contain the
○ Because at 6 months of age, the child begins to crawl, walk,
mutated gene → only the males will have the manifestation
climb stairs
of the disease while the females are the carrier
■ Ex. in crawling, the knees are stressed → easy
○ Only males would have manifestations because they only
bruising on the knees.
have one X chromosome (same with baldness because it is
■ Ex. mild bump of the elbow on the table → easy
also x-linked recessive disorder)
bruising on the elbow or sometimes swelling
■ Has a significant decrease of clotting factors that
● Spontaneous bleeding
would affect the coagulation cascade resulting to
uncontrollable bleeding ○ In some countries or even in the PH, early circumcision is
○ Females are more of a carrier because they have 2 X being practiced. So as soon as the baby boy is born →
chromosomes
TREATMENT
● Supportive therapy
○ Nurses address the symptoms patient is experiencing
● IV Fluids
○ Dehydration due to plasma leakage → provide isotonic IV
fluids and sometimes colloids
○ Monitor patient because of increased risk for circulatory
SIGNS AND SYMPTOMS
NOTE: Signs and symptoms of DHF depends on which phase the patient is in overload due to plasma leakage
1. Febrile Phase ○ Plasma leakage can cause dehydration and circulatory
overload at the same time
● N/V ● Bone pain
● High grade fever (2-4 ○ DHF also called ● Monitor BP (narrowed pulse pressure)
days) breakbone fever ○ Patient may be experiencing internal hemorrhage or shock
● Severe headache because of the pain ○ Narrow pulse pressure: <20 mmHg → check if patient is
● Retro-orbital pain ● Maculopapular rash experiencing other symptoms of shock
● Joint pain (arthralgia) ○ Petechiae (bleeding or ● No aspirin for fever
● Muscle pain (myalgia) bursting within
○ Drug of choice for fever is paracetamol
capillary) or purpura
○ Aspirin is anticoagulant → increases risk for bleeding since
rash (sign of bleeding
under the skin, the platelets for patients with DHF are already low
palpable, elevated) ● Plasma expander and platelet concentrate (below 20,000)
○ Nursing responsibilities are the same with blood transfusion
■ Stay with the patient
■ Monitor VS every 15 mins, every 30 mins, and
every hour
■ Check for transfusion reactions
2. Critical Phase
○ We transfuse platelet concentrates when platelet value falls
● Most patients would recover but if patient doesn’t, they can
below 20,000
have plasma leakage (usually happens when fever subsides or
● Oxygen PRN
defervescence)
○ Administered if there are signs of shock or plasma leakage
● Begins after defervescence (24-48 hours)
has affected lungs (pleural effusion)
○ After 24-48 hrs of defervescence, patient may
● DAT (except dark colored food) - Diet As Tolerated
recover completely or progress to a more severe type
○ Dark colored foods may mask the internal bleeding
of dengue called DHF which would lead to plasma
○ Ex. Patient ate tocino/pusit → vomit is dark → we may not
leakage or capillary leak syndrome
recognize if it’s the food or blood
○ The exact mechanism of the increased permeability
● Best treatment is PREVENTION
of capillaries is not known, but it may be due to the
○ Instruct patients to remove stagnant water
reactions of the capillary walls to the enzymes
released by the virus
● Plasma leakage - severe dengue Leukemia (Blood Cancer)
● Cancer of blood-forming cells that usually involves bone marrow and
● Reappearance of fever (saddleback fever)
lymphatics
○ On and off fever
● There are many types but the most common in pediatrics: Acute
● Could lead to shock
Lymphocytic Leukemia (ALL) and Acute Myeloid Leukemia (AML)
NOTE: In capillary leak syndrome, only the plasma leaks out of the
capillary, the RBCs will stay in the capillary → plasma goes out to
ANATOMY & PHYSIOLOGY REVIEW
lungs, heart, peritoneal membrane causing accumulation of fluid in ● Stem cells gives rise to different blood components
these organs → pleural effusion, cardiac overload, ascites → end ● Before they become mature, stem cells produce the immature cells
result is hemoconcentration (high hematocrit, but capillaries are full ○ megakaryoblast (immature) → platelets (mature)
of RBCs) ○ erythroblasts → RBC
NOTE: In DHF, platelets are affected so patients also have ○ myeloblast (immature granulocyte) → eosinophil, basophil,
thrombocytopenia. The exact mechanism of this is also unknown, neutrophil, monocyte
but one explanation is the virus has high affinity to platelets → ■ In AML, there’s increased number of myeloblast in
attach to platelets and replicate there → platelets with virus attacks bloodstream (no mature granulocytes → no
normal platelets → death of platelets → drastic drop of platelets function)
○ Lymphoblast → lymphocyte
3. Convalescent Stage
● Patient is recovering
DIAGNOSIS
● CBC
○ All blood components are decreased
● Peripheral Blood Smear
○ Checking the number of a blood component in relation to
other blood components & morphology
○ Reveals a lot of “-blasts” or immature cells
● Bone Marrow biopsy
○ Reveals a lot of immature cells in the system but specifically
Agranulocytes (affected in ALL) in the bone marrow
● lymphocyte and monocyte ○ Normal site: iliac crest (based on book)
● Lymphocytes aka Agranulocytes (B lymphocytes, T lymphocytes, ○ However, pediatricians often use sternum as site of
NK cells) are important in fighting abnormal cells aspiration
○ When they detect an abnormal cell in bloodstream, these ■ Ideally should be on iliac crest so that it won’t be
mature cells release chemicals that cause cell death on traumatic on child bc in sternum they can see
abnormal cells what’s happening
○ After killing it, they will proceed to finding more abnormal ○ Child is sedated
cells and completely remove them from the bloodstream ● Lumbar puncture
○ They can only function if they are mature ○ Aka lumbar tap or spinal tap
○ NK cells are important in detecting and fighting tumor or ○ If suspected of CNS involvement
cancer cells → since this is missing/deficient, it is easy for ○ Patient in side-lying knee-chest position to widen the space
cancer cells to proliferate in leukemia (ALL) between the vertebrae
■ Easier to locate the site to aspirate CSF (between
Granulocytes (affected in AML) L3 & L4)
● Myeloblast will not develop into mature granulocytes (basophil, ■ Spinal cord ends at L2 and we don’t want to hit
eosinophil, neutrophil) the spinal cord when we aspirate CSF
○ Basophils - largest granulocyte cells important in immune / ■ Can also be L4 & L5 or L5 & S1 but L3 & L4 most
allergic reactions of the time
■ These granules release histamine to counteract ○ Side effect: spinal headache
effects of allergic reaction ■ Advise patient to remain flat and still to avoid
○ Neutrophils - responsible for phagocytosis spinal leakage
■ When they detect a pathogen, they extend a part
of their cell to phagocyte the pathogen →
resolves invasion of pathogen
○ Eosinophils - responsible for parasitic infections,
inflammation
■ Through degranulation (release of granules), they
are able to kill the intestinal worm
SIGNS AND SYMPTOMS
Acute Lymphocytic Leukemia ● NOTE: S/sx are related to pancytopenia
● Involves lymphoblasts (immature lymphocytes) ● Normally, the first report of parents is on and off fever
○ Rapid proliferation of lymphoblasts (acute because they ● Extreme fatigue
rapidly multiply in bloodstream) → outnumber normal cells ● Headache
(RBC, WBC, platelets) → bone marrow stops producing ● Seizures
○ CNS involvement; a lot of immature cells in brain
● The manifestation of ALL and AML are the same (attributed to Can a female with hemophilia transfer it to her SON?
pancytopenia of the patient) ● Yes. 50% because one of the 2 son’s chromosome will be affected
● But since ALL involves the lymphatics, there’s more incidence of
lymphadenopathy and bone and muscle joint pain
● With AML, since what’s greatly affected is the bone marrow, and the
occurrence of lymphadenopathies is decreased and not the common
compared to ALL
● According to age, ALL are common among children while AML is more
common in adults or in adolescents
● Hepatosplenomegaly is less common in AML because of the
involvement of the lymphatics. When there is an involvement of the
lymphatics in ALL, hepatosplenomegaly is more common to them and
because of hepatosplenomegaly in patient will ALL, they have this loss
of appetite (anorexia)
● Gum hypertrophy is more common in AML because the bone marrow is
affected. When there is bone marrow hyperactivity or increased
stimulation of the bone marrow, the bones (esp. facial bones) are
affected → gum hypertrophy
● The cells that are rampant or increase in AML would be myeloblast or
the myeloid while in ALL the increase would be lymphoblast
TREATMENT
● Chemotherapy
○ In ALL, there are different phases of chemotherapy:
induction, consolidation, and maintenance
○ In AML, the chemotherapy starts in induction phase but
there is no maintenance phase
■ Once there is remission after the induction phase,
→ proceed with bone marrow transplant ideally
so that complete remission could happen → life
expectancy will increase and the prognosis would
be good
● Bone marrow transplant