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TOP SI Pediatrics PDF
TOP SI Pediatrics PDF
Pediatrics
N. Srikanchanawat
1
Developmental
Milestone
Pediatrics
Nithiwat Srikanchanawat
1. Frog position 1. 1. 1.
2. 2 2. 8-9 2. 2. >
Newborn 3. Moro, Placing, 3. Grasping 3.
Rooting, reflex
Suckling reflex
1. 1. 1. 1. Eye regard
2. Head up midline ()
1 Mo. 3. ATNR (Fencing 2. Tight grasp 2. Social smile
position) () ()
**Placing reflex gone
(1-1 mo.)
1. Head up 45 1. 1. Vowel sound 1.
() midline () 2.
2 Mo. 2. () 2.
1. Head up 90 1. Grasp rattle 1. Coo
2. Stepping reflex () ()
3 Mo. **Rooting reflex **Grasping
gone reflex gone
1. 1. Pincer grasp
**
10 Mo.
4 yr. 50 cm
10 yr. 55 cm
(4-10-4-10 5 cm; 3-9-3-9)
- Fontanelles
1. Posterior fontanelle (PF) 3 , ; 1 mo.
2. Anterior fontanelle (AF) 4 , 4-6 cm; 1 yr.
( PF AF, )
- Chest circumference (CC)
NB: CC < HC (CC NB = 33 cm; CC = HC-2)
Age -1 yr.: CC = HC ()
Age > 1 yr: CC > HC ()
- Upper:Lower segment (U:L) ratio : lower segment fix pubic symphysis
lower segment upper segment Ht. - lower segment
U:L ratio = 0.9-1;
Age U:L ratio
NB 1.7
6 mo. 1.6
1 yr. 1.5
2 yr. 1.4
3 yr. 1.3
4 yr. 1.25
5 yr. 1.2
10 yr. 1
- Arm span ( 2 ) ~ Height 5 cm
Marfan syndrome arm span
- Onset of puberty
Boy: puberty 9-14 yr.; 1st sign Testicular enlargement
Girl: puberty 8-13 yr. (); 1st sign Breast enlargement
- Tanner stage of Breast
Stage 1
Stage 2 Breast bud
Stage 3 1
Stage 4 2 (areola )
Stage 5 Nipple protrude ( 1 stage 3)
- breast menses ~ 3
- Total pubertal growth puberty
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Boy: 25-30 cm
Girl: 20-25 cm
- Midparental height Ht. ( 100% factor)
(Ht .+)+13
Boy: 7cm
2
Ht . 13
Girl: 7cm
2
- Ht. < P3 & = Short stature
(, , ) Ht. < P3 ( genetic ) = Familial short stature
delayed puberty, bone age = chronological age
- rate curve P3, delayed puberty, bone age = height age (
chronological age) = Constitutional delay (rate curve delay )
- Ht. > P97 & = Tall stature
- Weight for height (%W/H)
Failure to thrive (FTT)
- FTT < 5 yr.; Dx
1. BW < P3 ( BW ; Ht. normal < P3 )
2. Growth curve 2 (major percentile lines)
- FTT BW Ht. Ht. = chronic
- FTT
1. Organic cause
1) GI: Cleft lip / Cleft palate, Pyloric stenosis, GERD, Cow milk allergy, Hepatitis, Cirrhosis, Diarrhea
2) Renal: UTI, RTA, Renal failure
3) CVS: CHD
4) RS: OSA
5) Brain: CP, MR
6) Endocrine: GH deficiency, Hypothyroid, Cushing syndrome, Hypopituitarism, Delayed puberty
( Hx Menstruation, Tanner stage), Polyuria, Polydipsia
7) Genetic: DS, Turner syndrome, Skeletal dysplasia
2. Non-organic cause
1) Normal variation
2) Inadequate calories ( Hx , solid
food infant 6-7 mo.)
3) Psychosocial (environmental) factor < 3yr.
- Investigation
1. CBC Anemia, Malnutrition
2. ESR Chronic inflammation / infection
3. Electrolyte Metabolic acidosis / alkalosis
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- Development ---
- Frog position NB
- Head up 1
- Head up 45 2
- Head up 90 3
- Hold head up 4
- Pull to sit, Roll over 5
- Sit w/ support 6
- Sit w/o support, 7
- 8
- Pull to stand (), 9
- 10
- 1 1
- 15
- 1
- 2
- 3
- 6
- Development -
- 15
- 1 1
- 2
- , 3
- 4
- Development
- 2 (jump) 2
- (hop) 4
- (skip), 5
- Development
- Grasping reflex NB
- Tight grasp 1
- Loose grasp 2
- Grasp rattle, Grasping reflex gone 3
- Hand together 4
- Palmar grasp 5
- Transfer object hand to hand 6
- 2 , 8
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- 9
- Pincer grasp 10
- Release object on demand, 12
- 2 , , 15
- 3 , 1
- 6 2
- 8 3
- 4
- 6
- Development
- 1
- 2
- 3
- , 4
- , 5
- 6
- Development
- 8
- 1
- (parallel play) 2
- 3
- 4
- 6
- Development
- (babble), jargon 9
- (single word) 1
(Delayed speech Dx 15 mo. babble, 2 yr. single word)
- 1
- 2-3 2
- , , 4
- 5
- , , 6
- Development
- 6
- 9
- 2
- 3
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Neonatology
General considerations in neonatology
- 7 ( round NB )
1. Temperature keep core T ( rectum) 37C0.2 (36.8-37.2C)
(: thermometer 3 cm (term), 2.5 cm (preterm) 3 min)
LR: T 25-26C
Nursery: T 26-28C
2. Respiration keep RR 40-60/min, airway , Sao2 92-95% ( 95%
O2 toxicity e.g. ROP esp. Preterm, MAS keep Sao2 > 98%)
2 hr. RR > 60/min
3. Nutrition / IV fluid
4. Infection
5. Specific treatment (e.g. Early neonatal sepsis ATB: Ampicillin + Gentamicin 10-14 d)
6. Bonding ,
7. Development
- Neonatal period = D.0-28 (4 wk.);
1. Early = D.0-7 (Wk.1)
2. Late = D.8-28 (Wk.2-4)
- Term: GA 37 wk. ( 40 wk.)
Preterm: GA < 37 wk.
Postterm: GA 42 wk.
- DOL = 1 (DOL = day of life)
- Apgar score 1, 5 min
Score 0 1 2
Appearance (Color) All blue, pale Pink body, Blue extremities All pink
Pulse (HR) Absent < 100/min > 100/min
Grimace (Response to nasal None Grimace Sneeze, Cough
catheter / tactile stimulation)
Activity (Muscle tone) Limp Some flexion of extremities Active
Respiration Absent Irregular, slow Good, crying
At 5 min: Score 7-10 Normal; Score 4-6 Intermediate; Score 3 Low
Intermediate & Low (At 5 min: Score < 7) = Perinatal depression 5 min
score 7
Perinatal depression NPO organ (
vital organ ) Bowel ischemia infection NEC
- Severe asphyxia 4
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5. No ANC
2 Neuromuscular Physical score 2
Physical x2
Day 1 2 3 4 5 6 7 8
Term 65 100 120 150
65 80 150
Preterm 60 80 100 120
(D.1 65 60; D.2-3 ; D.4-7 1 step)
D.1-2 10%D/W ( Electrolyte shock load NSS
isotonic )
(10%D/W 100 cc glucose 10 g 40 kcal)
> D.3 10%D/N/5
- + IV fluid volume , TV TV
IV fluid
volume (20-30 cc/kg/d term) D.3
volume TV
Strength Rate
- Glucose production rate (GPR) =
6 BW
(Strength %D, Rate cc/hr., BW kg)
Term: GPR = 4-6 ( / IV GPR 4 F/U DTX
Hypoglycemia GPR 15)
Preterm: GPR = 6-8
- GPR 2 () Strength & Rate
Strength %D peripheral line 12.5% central line
(UVC)
Rate volume limit
- order NB
One day Continue
Hct, DTX, BS Routine newborn care
BM/SI (20 kcal/oz) __ cc x 8 feeds (TV = __ cc/kg) Record V/S q 4 hr.
- Monitor SBP 60 mmHg ( BW )
- T 36.8-37.2C
- RR 40-60/min
- HR 120-160/min
- Sao2 > 95%
Record BW OD
Record Lt, HC once a week
- Primitive reflex incomplete myelination
Demyelination Persistent primitive reflex ( Myelination
Primitive reflex)
- scalp: Cephalhematoma, Subgaleal hematoma, Caput succedaneum
1. Cephalhematoma
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- suture
- Soft, fluctuation, (subperiosteal hemorrhage)
- ! introduce infection , counseling , F/U
2. Subgaleal (Subaponeurotic) hematoma
- suture
- Soft, fluctuation, aponeurosis ( & space
)
- 90% vacuum
- Head trauma (ICH, Skull Fx) 40%
- 12-72 hr. , Hemorrhagic shock
3. Caput succedaneum
- suture
- Tense, fluid
- pressure
- skull bone = Molding
- Cephalhematoma, Subgaleal hematoma anemia, Hyperbilirubinemia
Phototherapy / Exchange transfusion
- Molding Craniosynostosis
Molding bone
Craniosynostosis bone
- NB 95% meconium 1 d, 99% meconium 2 d
- NB 95% void 1 d, 99% void 2 d
Normal findings in the newborn
- NB
1. Vernix caseosa = heat loss;
source of infection
2. Erythema toxicum neonatorum (ETN) = erythematous macule () , , ,
()
- , hypersensitivity reaction
- ~ D.10, 5 d, 2 wk.
- confirm ETN vesicle vesicle Wrights stain
Eosinophil; G/S negative
3. Mongolian spot = hip/coccyx; ~ 4 yr.
4. Desquamation (Skin peeling) = skin term D.1-2 , ;
2-3 d
Preterm: Desquamation , GA
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2. Symptomatic: 10%D/W 2 cc/kg IV push = Minibolus F/U DTX 30 min, 10%D/W IV, keep GPR
4-6; F/U DTX GPR 2 q 30 min ( F/U DTX ), F/U DTX
GPR hypoglycemia; DTX , GPR 1 ( 1-2
)
- GPR 12 hypoglycemia ( LGA )
Hydrocortisone
- Persistent hypoglycemia 7 d
Neonatal polycythemia
- Polycythemia NB = Hct 65%
- Dx Polycythemia Partial exchange ( UVC NSS
dilute )
1. Hct 65% + S&S of Hyperviscosity syndrome ( active (lethargy), cyanosis, respiratory
distress, plethora, seizure, feeding problems, signs of poor tissue perfusion, etc.)
2. Hct 70%
- Hct Partial exchange 50-55%
- Volume Partial exchange = (Hct Hct ) x Blood volume; cycle 3-5 cc/kg,
15-30 min ( Hypovolemia, BP drop )
(Blood volume = 0.8 x BW)
e.g. 2 kg, Hct = 70% Partial exchange = (70-50) x 0.8 x 2 = 32 cc, cycle 3-
5 cc/kg = 6-10 cc/cycle 4-5 cycles
Neonatal hyperbilirubinemia
- Neonatal jaundice
1. Physiologic jaundice 24 hr. (~ D.2-3) liver
2. Pathologic jaundice 24 hr. , , ,
: MB rate > 5 /d > 0.5 /hr
: onset 24 hr.
: > 7 d (term), > 14 d (preterm)
- MB : ()
5
10
15
20
NB indirect () direct ();
direct: TB DB 20%
- Hct, MB; plot MB graph zone : LR/LI/HI/HR, on Phototherapy /
Exchange transfusion
(LR = low risk, LI = low intermediate, HI = high intermediate, HR = high risk)
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1. Tone ()
2. HR ()
3. RR ()
vigorous
- Nasal obstruction: test nasal airflow
obstruction respiratory distress
turn Obstructive apnea
Nasal obstruction: NSS Iliadin (Oxymetazoline) q 8 hr.
Iliadin decongestant Imidazoline derivative 3
Rebound congestion & Rhinitis medicamentosa 3 1 )
- Choanal atresia Obligate nose breather =
Choanal atresia
- Hyperoxia test cyanosis cardiac cause (Cyanotic heart disease)
( non-cyanotic Hyperoxia test )
- C:T ratio NB = 0.55 ( 1 yr. 0.5 adult)
- Cause of respiratory distress NB 4
1. Postnatal adaptation
2. Transient tachypnea of the newborn (TTN)
3. Meconium aspiration syndrome (MAS)
4. Respiratory distress syndrome (RDS, Hyaline membrane disease, HMD)
Postnatal adaptation
- Respiratory distress Postnatal adaptation 2 hr. ( LR ) ,
Transient tachypnea of the newborn (TTN)
- TTN delayed absorption pulmonary fluid ( fluid lung ) respiratory
distress 2 hr.
- Risk factor 4
1. C/S (esp. Elective C/S labor pain)
2. Prolonged labor
3. Maternal DM
4. Maternal Asthma
- PE: Breath sounds
- CXR: Sunburst appearance ( perihilar 2 ), Hyperaeration, fluid
Minor fissure, air bronchogram (esp. ), F/U film absorb fluid
pattern clear , ( lymphatic system)
- Management: Supportive, absorb fluid ; 3-5 d
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- Neuro IICP
- Gut obstruction ( opening of bile duct) Intestinal atresia, Malrotation, Meconium
plug syndrome, Hirschsprungs disease
3. blood
- Blood Coagulopathy GI bleeding / , NEC, Gastric volvulus,
irritate stomach (e.g. NSAIDs)
- Blood ( Apt test), cracked nipple
- Initial management
1. PE: V/S (Blood loss, Infection), Abdomen (Mass, Gut obstruction, Bowel sounds; Hyperactive
bowel sounds surgical condition), Neuro (Meningeal irritation, IICP, Bulging fontanelle)
2. position: aspiration
3. NPO + + OG/NG decompression
4. IV fluid
5. Investigation: Acute abdomen series, CBC, Apt test
- Definite treatment:
Abdominal distension
- Abdominal distension ( normal ,
stool )
- cause
Gut obstruction ( distal) Hx delayed pass meconium in 48 hr., NEC, Sepsis, GI perforation
Organ PKD, Neuroblastoma, Hepatosplenomegaly
- Initial management
1. PE: V/S, Abdomen
2. NPO + OG/NG decompression ()
3. IV fluid
4. Investigation: Acute abdomen series, etc.
Necrotizing enterocolitis (NEC)
-
1. Intestinal ischemia (Preterm, IUGR, Perinatal asphyxia, Polycythemia)
2. Abnormal bacterial flora ( Broad-spectrum ATB)
- NEC DOL 1-2 (term), DOL 7-10 (preterm)
( Preterm Preterm NPO DOL
7-10 step )
- Common VLBW (< 1,500 g)
- common Terminal ileum & Ascending colon
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Cardiology
- pulse brachial a. femoral a.
- Active precordium NB sign PDA
- Femoral pulse NB: CoA; PDA
- Innocent murmur (Functional murmur) = murmur flow
1. Systolic murmur Venous hum innocent murmur Continuous murmur
( Diastolic / Pansystolic / Late systolic / Continuous murmur Venous hum)
2. Gr. 3
3. heart
4.
- Venous hum = blood flow Internal jugular v. Continuous murmur
Venous hum PDA
: murmur Jugular v. murmur
Venous hum ( PDA murmur )
Venous hum supine position murmur
- Hemic murmur = innocent murmur anemia, anemia
: Anemia (Hct ) Blood viscosity , TPR SV & C.O. , Velocity flow
Turbulent flow (esp. Aortic valve & Root of ascending aorta velocity
flow ) soft SEM
Congenital heart disease (CHD)
- Approach
1. Heart
2. Heart Congenital Acquired
( Acquired heart disease RF, Myocarditis, KD, etc.)
3. Congenital ( present 1-2 yr. ) Cyanotic Non-cyanotic
4. Cyanotic Refer
- Cyanotic heart diseases: 5T
1. Tetralogy of Fallot (TOF)
2. Transposition of great arteries (TGA)
3. Tricuspid atresia (TA)
4. Truncus arteriosus
5. Total anomalous pulmonary venous connection (TAPVC)
6. Common ventricle
- Non-cyanotic heart diseases 3
1. Lt.-to.Rt. shunt: ASD, VSD, PDA, PAPVC HF
2. Obstructive disease: AS, PS, CoA CoA
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Endocarditis Murmur
Myocarditis Clinical HF, TnT, CK-MB
Pericarditis Pericardial effusion Pericardial rub, Distant heart sound, Cardiac tamponade
4. Polyarthritis
5. Erythema margitanum
Minor criteria
Clinical
1. Fever
2. Arthralgia
3. Hx of RF of RHD
Laboratory
1. ESR, CRP (Acute phase reaction)
2. Prolonged PR interval
- Supporting evidence of GAS infection ( RF sequelae GAS infection)
1. ASO titer, Anti-DNaseB
2. Throat swab for C/S ( 20% GAS carrier)
3. Recent Scarlet fever
- Investigation: CXR, EKG, Echo.
- Prophylaxis Dx RF
1. Carditis 5 yr. 21 yr.
2. Carditis + no residual 10 yr.
3. Carditis + residual (valve ) > 10 yr. 40 yr. life-long
Kawasaki disease (KD)
- Kawasaki disease (KD, Mucocutaneous lymph node syndrome) criteria Dx
1. Fever 5 d
2. 4/5
1) Bilateral nonexudative conjunctival injection
2) Changes in lips, tongue, or oral mucosa (e.g. Injection, Drying, Fissuring, Strawberry tongue)
3) Changes in peripheral extremities (e.g. Edema, Erythema, Desquamation)
4) Polymorphous truncal exanthem
5) Cervical Lymphadenopathy (at least 1 node 1.5 cm)
Criteria KD Mucocutaneous lymph node syndrome
Muco (2 ) = ,
Cutaneous (2 ) = -//,
Lymph node (1 ) = LN
4 5 criteria + 5 d Dx KD
- infant & pre-school
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- Onset
- Hx bile Enterohepatic circulation
absorb bile Jaundice
Dx Breastfeeding jaundice Breastfeeding
supplement
2. Breast milk jaundice
- Onset mo.
- Hx Glucuronyl transferase inhibitor conjugated IB DB
IB Jaundice
- Breastfeeding & Breast milk jaundice Phototherapy, Phenobarbital
- IV fluid
Replace bile NSS
Replace gastric content N/2
Short bowel syndrome
- Dx SB < 50%
- : Peptamen junior MCT
Protein-energy malnutrition (PEM)
- Waterlow classification malnutrition Weight for height (%W/H) & Height for age (%H/A)
Normal Mild Moderate Severe
%W/H 100 10 (90-110) < 90 < 80 < 70
%H/A 100 5 (95-105) < 95 < 90 < 85
Acute PEM %W/H = Wasting
Chronic PEM %H/A = Stunting (Nutritional dwarf)
- Gomez classification malnutrition Weight for age (%W/A)
Mild Moderate Severe
%W/A < 90 < 75 < 60
- Welcome classification malnutrition %W/A & Edema ( hypoalbuminemia
protein)
%W/A No edema Edema
60-80 Undernutrition Kwashiorkor
< 60 Marasmus Marasmic kwashiorkor
- Marasmus: common < 2 yr.; %W/A < 60
1. Skin and bone appearance
2. Monkeys face appearance : , , ,
subcutaneous fat
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Respiratory Medicine
- Dose
1. Paracetamol syrup (120 mg/5 ml = 1 tsp) 10-15 MK/dose
(e.g. 10 kg 100-150 mg/dose = 1 tsp)
2. Ventolin 0.03 MK/dose
(e.g. 10 kg 0.3 mg/dose Ventolin 0.3 mg NSS 3 cc q 4/6/8 hr.
severity)
3. Amoxicillin (125 mg/5 ml = 1 tsp) 40-50 MKD tid
(e.g. 10 kg 400-500 mg/day = 1 tsp tid)
4. Cloxacillin syrup (125 mg/5 ml = 1 tsp) 50-100 MKD qid
(e.g. 10 kg 500-1,000 mg/day qid = 1 tsp qid)
5. Bactrim 8 MKD qid
6. Pseudoephedrine 4 MKD
7. Carbocysteine ( 10 kg tsp)
- poor air entry () sign upper airway obstruction
- (high CO2) , aspirate
= Sudden infant death syndrome (SIDS)
- FB aspiration 1-3 yr. ()
- oral T 5 yr. ()
Upper respiratory tract infections (URI)
- URI typical (3-5-7)
3 d
5 d
7 d
> 2 wk. Sinusitis
Hx URI 1 mo. URI
( Ag ;
1 mo. Dx URI )
-
+ Rhinopharyngitis
Epiglottitis
- Epiglottitis: X-ray Thumb sign
Croup
- Croup (Acute laryngotracheobronchitis) PIV 1, 2, 3; triad
1. Hoarseness
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2. Inspiratory stridor
3. Barking cough
- 6 mo. 6 yr.
- Incubation period: 2-6 d, () 12-72 hr.
- DDx: Acute epiglottitis, Bacterial tracheitis, FB aspiration, RPA, Diphtheria
- X-ray: Pencil (Steeple) sign
- Downes croup score: C2RSI
Score 0 1 2
Cough Hoarseness Backing cough
Cyanosis Cyanosis in RA Cyanosis in O2 40%
Retraction & No Nasal flaring & 1 + Infrasternal &
Nasal flaring Suprasternal retraction Intercostal retraction
Stridor Inspiratory Inspiratory & Expiratory
Inspiration Normal Harsh with rhonchi
Score 0-3: Mild Supportive, , Advice
Score 4-7: Moderate Admit
Score 8-10: Severe on ETT
- Admission criteria: Progressive stridor, Severe stridor at rest, Respiratory distress, Hypoxia, Cyanosis,
Depressed mental status, Need for reliable observation
- Management
1. O2 + Mist therapy
2. IV fluid (Hydration)
3. ATB ( bacterial infection)
4. Closed observation, Downes croup score
5.
6. Steroid severe
7. Epinephrine (1:1,000) inflammation larynx
Acute Bronchiolitis
- 6 mo. 2 yr.; RSV
- S&S: , Wheezing, Increased AP diameter ( air trapping), Liver (
)
- CXR: Hyperaeration
Pneumonia
- Pneumonia criteria Dx
1. Fever ( 38C)
2. Tachypnea
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Nephrology
Fluid replacement
- Fluid replacement = Maintenance (M) + Deficit (D) + Ongoing Loss (L)
- Maintenance (M) = fluid metabolism + + loss urine,
feces, insensible loss (ISL = 300 cc/m2/d)
admit replace ISL & urine output admit urine
output Oliguric urine output = 240 cc/m2/d)
- H2O Holliday-Segar law
10 kg 100 cc/kg
10 kg 50 cc/kg
10 kg 20 cc/kg
- Na+ 2-3 mEq/kg, K+ 1-2 mEq/kg
- M 1 3 8 hr.
- Deficit (D) severity dehydration
- Mild Flat fontanelle
- Moderate Marked dry lip/tongue, Sunken eyeball, Decreased urine output, Decreased skin
turgor, Orthostatic hypotension
- Severe BP drop, Anuria
- < 1 yr. deficit 5-10-15% severity
1 yr. deficit 3-6-9% severity
- isotonic dehydration: 100 cc H2O deficit Na+ 8 mEq/kg
- Deficit 1 8 hr. 16 hr.
- fluid replacement
Case: 2 15 kg 10 1 2 ,
last void 8 hr. .., PE: afebrile, P 130/min, BP 85/50 mmHg, marked dry lip, dry tongue, sunken
eyeball IV rate & strength admit
Maintenance
H2O: 15 kg 1,000 + 250 = 1,250 cc
Na+: 3 mEq/kg = 3 x 15 mEq = 45 mEq
Deficit
H2O: moderate dehydration, 1 yr. 6% = (6/100) x 15 x 1,000 ( L
cc x 1,000) 6 x 15 x 10 ( % x BW x 10) = 900 cc
Na+: 8 mEq/kg 100 cc H2O deficit H2O deficit 900 cc Na+ 8 x 9 (8 x H2O deficit/100) =
72 mEq
H2O M 1,250 cc, D 900 cc
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Potassium disorders
- HypoK+ K+ 1-2 mEq/kg
KCL 1 cc K+ 2 mEq ( IV)
- IV 1 K+ Max = 40 mEq/L Phlebitis central line
- Rate K+ Max = 0.5 mEq/kg/hr rate
Elixir KCl 15 cc K+ 20 mEq ()
(e.g. K+ = 3.1 () : K+ 1 mEq/kg, 10 kg K+ 10 mEq = Elixir KCl 7.5
cc)
- HyperK+ guideline
K+ 5-6 K+ intake < 0.5 mEq/kg/d & Lasix
K+ 6-6.5 add NaHCO3-, -agonist, Kalimate
K+ > 6.5 Ca gluconate, Glucose & RI, consider Dialysis
Acid-base imbalance
- acidosis urine urine RTA
(e.g. Proximal RTA PCT absorb HCO3- )
- RTA type 1 (Distal RTA) Distal tubule secrete H+ ; Nephrocalcinosis, U/S: Calcification
- RTA type 2 (Proximal RTA) Proximal tube reabsorb HCO3-
- Metabolic acidosis (e.g. MMA) FTT ()
Nephrotic syndrome (NS)
- NS 4
1. Nephrotic-range proteinuria
1) Urine protein > 40 mg/m2/hr > 50 MKD
2) Urine protein:Cr > 2 ( < 0.2)
2. Hypoalbuminemia Plasma albumin < 2.5 g/dl
3. Generalized edema
4. Hyperlipidemia Cholesterol > 250 mg/dl
- Sign Puffy eyelid ()
- urine sp.gr. < 1.015 protein 2+ significant
urine sp.gr. > 1.015 protein 3+ significant
- spot urine ( Urine protein:Cr) Morning urine Orthostatic
proteinuria (esp. )
- Nephrotic syndrome IgG, IgA IgE, IgM
- Complications: THAI+P
1. Thrombosis
2. Hypovolemia/Hypotension
3. Acute renal failure (ARF)
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4. Infection (peritonitis)
5. Protein depletion
- Management
1. Prednisolone 2 MKD (Max = 60 mg/d = 12 tab/d; 5 mg/tab) OD 4 wk. urine protein -ve 3
d ( 8 wk.)
m2 Prednisolone 60 mg/m2/d
2. Taper dose 2 MK AD ()
3. Taper dose off 6 mo.
- 90% responder remission (urine protein < 4 mg/m2/hr) 10-15 d
- 2 MKD OD 4 wk. remission = Steroid resistance (Steroid unresponsive)
urine protein remission +ve (e.g. 4+ 3+) = Late responder
- treat urine protein -ve +ve = Relapsed
relapsed 2 /6 mo. 4 /yr. = Frequent relapsed
- taper dose steroid (e.g. taper dose urine protein ) = Steroid dependent
treat urine protein negative try taper steroid taper urine protein positive
= Frequent relapsed Steroid dependent
- Late responder & Steroid resistance W/U Infection
Treat infection
add Cyclophosphamide (Endoxan) 2 MKD 12 wk. 3 MKD 8 wk.
- Renal biopsy
1. Steroid resistance
2. Frequent relapsed
3. Atypical case
4. start immunosuppressant Cyclosporine
Renal biopsy NS Minimal change disease (MCD)
Renal biopsy 4
- Albumin NS I/C
1. Intravascular volume depletion
2. S&S of volume overload e.g. Respiratory distress Pulmonary edema, Ascites)
( Albumin oncotic P intravascular)
3. Genitalia swelling Torsion testis, Infection
4. Acute renal failure (ARF)
- volume 5% Albumin
volume 20% Albumin
- NS Ascites Peritonitis most common S. pneumonia
Pneumococcal vaccine Dx NS
Peritonitis
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- I/C refer NS
1. Age < 1 yr. > 10-12 yr.
2. Persistent HT
3. Gross hematuria
4. Low C3 / C4
5. Steroid resistance
Glomerulonephritis (GN)
Acute post-streptococcal glomerulonephritis (APSGN)
- APSGN Pharyngitis 1-2 wk., Ecthyma 3-6 wk.
- APSGN clinical + (e.g. ) hematuria (
microscopic hematuria)
- Investigation
1. U/A, Serum complement (C3)
C3 Active SLE & APSGN
2. Supporting evidence
1) ASO titer ( Pharyngitis), Anti-DNaseB ( Pharyngitis & Ecthyma)
ASO, Anti-DNaseB > 20% UNL
2) Throat swab for C/S ( 20% GAS carrier)
Ecthyma swab C/S
3. Complication: CXR, BUN/Cr, Serum electrolyte, CBC
4. SLE ANA, Anti-dsDNA
- Management
1. ATB: Amoxicillin 10 d ( Macrolides)
2. decreased GFR
- HT Lasix 1-2 mg/kg IV Short-acting anti-HT (Hydralazine 0.1 mg/kg IV
Nifedipine 0.25 mg/kg SL)
- Pulmonary edema O2 + MO
3. IV fluid ISL (300-400/m2/d) Oliguric urine output (240/m2/d)
4. Restrict fluid
5. Correct electrolyte (e.g. HyperK+; K+ monitor EKG )
- F/U: Clinical 1 wk., Hematuria 2-3 wk.
F/U U/A, BUN/Cr, V/S, Sao2, Complication HT (e.g. Hypertensive retinopathy,
Hypertensive encephalopathy, Seizure, Severe headache)
Rapid progressive glomerulonephritis (RPGN)
- RPGN 3
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(3) False positive serological test for Syphilis > 6 mo. (VDRL +ve; TPHA -ve)
- DDx: Henoch-Schnlein purpura (HSP), Polyarthritis nodosa (PAN), Rheumatic fever (RF), Juvenile
rheumatoid arthritis (JRA)
- ANA sense 95% screen false positive neoplasm, chronic infection, other autoimmune
- active 2 Complement level (C3) & Anti-dsDNA
- SLE
1. Hydroxychloroquine 5-7 MKD: Skin lesion (esp. Discoid rash) / Arthritis / DLP /
Renal progression
( Eye exam Hydroxychloroquine SE: Retinopathy)
2. NSAIDs arthralgia, myalgia ASA 60-80 MKD Indomethacin 75-150 mg/d
3. Topical steroid skin involvement 0.1% 0.02% TA cream
4. Systemic corticosteroid Prednisolone
( Stool exam Prednisolone)
- Advice: , , ,
- Renal biopsy SLE I/C
1. Steroid high dose No response
2. Nephrotic nephritis
- LN (Lupus nephritis) class 3, 4 Steroid high dose + Cyclophosphamide (Endoxan)
Cyclophosphamide SE: Hemorrhagic cystitis
hydration 6 hr. Hemorrhagic cystitis IV hydration
F/U 2 wk. CBC ( Nadir period ANC
dose FN; F/U 2 wk. CMT WBC
10-14 d )
- ESR F/U ; treat ESR < 20
- U/A profile RBC proteinuria
Hypertension (HT)
- Normal BP BP < P90
- HT = BP > P95; renal cause;
1. HT stage 1: P95-99 + 5 mmHg
2. HT stage 2: > P99 + 5 mmHg
(P95 + 7 = P99)
- Pre-HT = P90-95 BP > 120/80 mmHg ( P90)
- Hypertensive urgency = P99 + 5 Management: Nifedipine (Adalat) (Short-acting CCB)
complication HT
Goal: BP 1/3 6 hr. Optimal BP 24-72 hr.
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3) Symptomatic HT
4) 2 HT
5) Target organ damage
6) DM
- DM type 2 / proteinuria -blocker
- Headache / Migraine CCB
- DM type 1 / Asthma / Heart block -blocker
- Goal:
- Essential HT complication, target organ damage (e.g. LVH) BP < P95
- HT Target organ damage / HT Chronic kidney disease / DM BP < P90
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Endocrinology
Hypoglycemia
- NB Maternal DM, Macrosomia ( hyperinsulinism); persistent
PHHI
- DDx:
1. Insulinoma
2. Pituitary tumor
3. Glycogen storage disease (type 1 severe , type 6, 9 adult, PE: Hepatomegaly)
4. Fatty acid oxidation defect (defect Lipolysis & Ketogenesis; Hx Prolonged fasting 10-12 hr. &
hypoglycemia; Hepatic failure, Cardiomyopathy)
5. Drugs
1) DM
2) HT (-blocker)
3) Salicylate ( Hepatic gluconeogenesis; Hx / Salicylate)
- Hyperinsulinism test Fasting test: fasting hypoglycemia insulin;
fasting
Age < 1 yr. Fasting 12-18 hr.
Age > 1 yr. Fasting 24 hr.
Adult Fasting 72 hr.
- Fasting test Urine ketone ketone
Hyperinsulinism ( Insulin Ketogenesis) test
Persistent hyperinsulinemic hypoglycemia of infancy (PHHI)
- PHHI most common cause persistent hypoglycemia < 1 yr.; Hx GFR
hypoglycemia 10%D/W strength central line
- Pathophysiology: PPHI K+-ATP channel -cell (
ATP K+ cell)
K+-ATP channel: Glucose GLUT2 -cell Glucose
Glycolysis ( enz. Glucokinase) ATP ATP K+-ATP channel channel K+
cell Depolarization Ca2+ channel Ca2+ cell Insulin
- PHHI Lab. Ketone ( Insulin Ketogenesis) = Nonketotic hypoglycemia
Counter-regulatory hormone (isolated) Ketone
Panhypopituitarism Ketone ve ( PHHI)
- Blood glucose < 50 mg/dl (Hypoglycemia) Insulin level < 2
Blood glucose < 50 mg/dl (Hypoglycemia) Insulin level > 2 = Hyperinsulinism
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3. Exercise
Insulin resistance Chronic hyperglycemia (insulin , -cell
insulin insulin ) liver detect insulin Gluconeogenesis
Blood sugar
- DM type 2 PE: Acanthosis nigricans ( Insulin resistance)
- Complications (DR, DN) q 1 yr.
DM type 1 : Dx 2 ; : Dx 5
DM type 2 Dx
- complication insulin: Lipohypertrophy, Lipoatrophy
- DN FSGS (Focal segmental glomerulosclerosis) Nephrotic syndrome
Diabetes ketoacidosis (DKA)
- DKA DM type 1; criteria Dx 3
1. Hyperglycemia: Plasma glucose > 200 mg/dl
2. Acidosis: HCO3- < 15 mmol/l or Venous pH < 7.3
3. Urine / Serum ketone +ve
- Severity HCO3- & Venous pH
HCO3- Venous pH
Mild < 15 < 7.3
Moderate < 10 < 7.2
Severe <5 < 7.1
- S&S: Dehydration, , N/V, Abdominal pain, , acetone , Kussmaul breathing (
acidosis)
- Pathophysiology: insulin ( blood sugar), lipolysis ( ketone) +
Precipitating cause Counter-regulatory hormone blood sugar (
Glycogenolysis + Gluconeogenesis) Hyperosmolarity Osmotic dieresis & Electrolyte
Dehydration
- Precipitating factors
1. Stress Infection, Shock
2. Poor compliance Insulin
3. Drug blood sugar
- Management: volume depletion, drip RI, add K+
1. IV fluid ( + = + +
100
100
1.6)
DKA Dehydration
- Fluid = Maintenance + Deficit (Moderate 5-7%, Severe 7-10%) 48 hr.
- 4-6 hr. NSS, then N/2
- Shock Load NSS 10-20 cc/kg in 1 hr.
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Pediatric obesity
- Weight for height (%W/H)
%W/H > 120% Overweight
%W/H > 140% Obesity
%W/H > 200% Morbid obesity
- Approach:
1. Obesity: %W/A > P97 %W/H > 140%
2. Normal Ht. velocity, Normal or higher Ht. percentile Simple obesity ()
Decreased Ht. velocity, Ht. below mid-parental Pathological obesity ()
- Metabolic syndrome criteria Dx (IDF 2006)
1. Waist circumference > P90
2. 2/4
1) TG > 150 mg/dl (or on treatment)
2) HDL < 40 mg/dl (male), < 50 mg/dl (female) (or on treatment)
3) BP > 130/85 mmHg (or on treatment of HT)
4) FPG > 100 mg/dl (or Pervious Dx DM type 2) criteria OGTT
- Investigation: FBS OGTT, Lipid profile, U/A, LFT ( NAFLD: ALT, AST:ALT < 1)
- Lipid profile
1. TC 170 200 (< 170 Normal; 170-199 Borderline; 200 Hypercholesteralemia)
2. TG 150 200 (< 150 Normal; 150-199 Borderline; 200 Hypertriglyceridemia)
3. LDL 110 120 (< 110 Normal; 110-120 Borderline; 120 High LDL)
4. HDL 40 ( 40 Desirable; < 40 Undesirable)
- Thick skin fold > P85 age & sex
- BMI obesity > P95 age & sex
- Waist circumference obesity > 90 cm (male), > 80 cm (female)
- OSA obesity CBC Polycythemia (Hct) hypoxia
- Pickwikian syndrome (Obesity hypoventilation syndrome, OHS) -
hypoxemia & hypercapnia & hypoventilation, lung volume
complications Polycythemia, PHT, Rt.-sided HF
- Puberty obesity onset of puberty fat tissue Estrogen
Tanner stage ( Genitalia & Breast )
- PCOS obesity ( obesity insulin resistance Reproductive system
); criteria Dx ( 2/3 )
1. Oligomenorrhea / Anovulation
2. Clinical and/or Biochemical signs of Hyperandrogenism , (Hirsutism),
3. Polycystic ovaries ( U/S)
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Infectious Diseases
Sepsis
- Signs of sepsis: , Hypo/Hyperthermia, Hypo/Hyperglycemia, Food intolerance
- Gram positive sepsis ATB treat 7-14 d
Gram negative sepsis ATB treat 14-21 d
- : T peripheral central ( chill )
Septic shock
- Management
1. Load NSS 20 cc/kg in 15-30 min ()
2. BP Load 3 doses ( 60 cc/kg) Load
1 hr.
3. BP Inotrope
Antibiotics
- S. pneumoniae : binding protein dose
H. influenzae, M. catarrhalis : -lactamase BL-BI
- Actinomyces israelii ATB: PGS
Nocardia asteroids ( immunocompromised) ATB: Bactrim (Co-trimoxazole)
- Meropenem E. faecalis E. faecium, Stenotrophomonas maltophilia
- Tigecycline P. aeruginosa, A. baumannii colistin
- Caspofungin Cryptococcus
Pharyngitis
- Exudative pharyngitis DDx 4
1. GAS infection
2. Infectious mononucleosis (IMN)
3. Fungal infection (Candida)
4. Diphtheria
- < 3 yr. IMN ( GAS)
> 3 yr. GAS IMN ( triad IMN, Downey cell)
treat GAS ( RF, APSGN) IMN rash Ampicillin
Amoxicillin
- DPT vaccine 3 doses diphtheria
Streptococcus group A (S. pyogenes, GAS) infection
- Non-suppurative complication GAS
1. Rheumatic fever (RF) Pharyngitis
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- 20 min
- C/I:
1. Immunocompromised host
2. MMR
3. Pregnancy ( 1 mo. )
4. IG
- Anti-TB start MMR
- PPD MMR , PPD MMR MMR
PPD 4-6 wk. MMR
6. JE (SC)
- 3 1 yr. 2 1-4 wk., 3 2 yr.
12 mo.
(Killed JE 3 Live attenuated JE 2 3 mo.)
- Booster 3-5 yr. ( 5 doses)
- Hypersensitivity reaction (Generalized urticaria, Angioedema) (rare)
- C/I:
1. Pregnancy
2. < 1 yr.
3. Adverse reaction JE vaccine
Vaccine
Newborn BCG, HBV1
2 mo. HBV2, DTPw1, OPV1
4 mo. DTPw2, OPV2
6 mo. HBV3, DTPw3, OPV3
9 mo. MMR1
1 yr. JE1, JE2 ( 1 wk.)
1 yr. DTPw4, OPV4
2 yr. JE3
4-6 yr. DTPw5, OPV5, MMR2
12-16 yr. dT (booster q 10 yr.)
- Vaccine
- VZV 2 1 yr., 4-6 yr.
- HAV 2 1 yr., 1 yr.
- Rabies
- vaccine HRIG + HDCV (Rabies
vaccine; IM) 5 D.0, 3, 7, 14, 28 Deltoid/Anterolateral thigh
- vaccine ( HRIG) HDCV 2 D.0, 3
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Dermatology
-
(e.g. Candida) Nystatin, Clotrimazole
- Diaper dermatitis VS Candida dermatitis ( well-defined erythematous patch )
Diaper dermatitis: Spare inguinal region
Candida dermatitis: spare
- SSSS (Staphylococcal scalded skin syndrome): Generalized desquamation, Erythroderma;
Nikolsky sign positive (); crusting ,
, = Sunburst appearance
- SSSS VS TEN
SSSS: involve mucosa
TEN: Involve mucosa
Streptococcus group A (S. pyogenes, GAS) infection
Scarlet fever
- Scarlet fever () PE: Fine, sandpaper-like rash, Circumoral pallor, Strawberry tongue
Eczema
- 3 acute, subacute, chronic
- Loratadine 10 mg OD (, )
Acute eczema
- PE: Vesicle, Serum oozing,
- Acute eczema lesion : lesion Wet dressing (Wet compression)
Subacute eczema
- PE: Scale, Crust, lichenification
- Management: 0.1% 0.02% TA cream ( inflammatory process)
Chronic eczema (Lichen simplex chronicus)
- PE: Lichenification ( + + increased skin marking), Hyperpigmentation, Excoriation ()
Atopic dermatitis (AD)
- AD chronic; Allergic march;
Allergic march: age of onset
( 4 )
1. Food allergy
2. Atopic dermatitis (AD)
3. Asthma
4. Allergic rhinitis (AR)
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- - = Nummular eczema AD
- Pityriasis alba I/C Hx
- AD abrasion 2 bacterial infection Abscess
Tendency toward cutaneous infection minor criteria AD
- Management
1. Hydration of skin ( AD lesion )
- /
- Lotion cream / Ointment;
( cream Fragrant-free / )
- ( )
- ( 1) , 2) occlude moisturizer
; moisturizer natural moisturizer
)
2. Topical steroid inflammation, potency , SE steroid
3. Antihistamine (AH) ( 2 bacterial infection)
Oral ( 1st Gen.
2nd Gen. 3rd Gen. )
IV
Topical AH () Desensitization ()
4. ATB 2 bacterial infection fungal infection
5. Avoid aggravating factors
Specific , infant food allergy
( hypoallergenic food
; severe food allergy e.g.
, food allergy sequence Allergic march)
Non-specific , , emotional stress ,
, cotton
- Education: , , Psychosupport
- Prognosis
- prognosis ( 2 yr., 5 yr.)
- & Adult chronic
- Severe AD control steroid Topical immunosuppressant
Topical calcineurin inhibitor (e.g. Tacrolimus, Pimecrolimus (Elidel), Cyclosporine), MTX, MMF
2nd line severe, control topical treatment
( 1st line FN )
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Hematology
- Peripheral blood smear (PBS)
Zone : RBC
Zone : RBC e.g. Polychromasia (Reticulocyte), NRC
- Physiologic anemia of infancy NCNC 2-3 mo. (term) 7 wk. (preterm)
- Parvovirus B19 = Fifth disease (Erythema infectiosum, Slapped cheek syndrome)
infect Young RBC BM Red cell aplasia
Microcytic anemia
Iron deficiency anemia
- Fe
1 d Clinical
1 wk. Reti. count
1 mo. Hb
Thalassemia
- present thalassemia > 6
- RDW CBC anisocytosis
- Hypersplenism = spleen CBC RBC, WBC, Platelet
splenectomy
Thalassemia splenomegaly ( RBC )
hypersplenism
- Splenectomy I/C Pneumococcal polysaccharide vaccine (23 serotypes)
Penicillin V prophylaxis
- CBC: Hct Hb (Hct:Hb = 4 ), MCV 60 fl HbH disease
- CBC: Target cell 3+ Homozygous HbE
- HbH disease Acid elusion test
= RBC HbH
Hemolytic anemia
- PBS Schistocyte hemolysis
- Hemolysis ABO/Rh incompatibility, G6PD, HS, TORCH infection, etc.
- Hemolysis AST ( AST RBC Liver ALT )
- Hemolysis general management
1. Hydration
2. Alkalinize urine
Hereditary spherocytosis (HS)
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Hemophilia
- Prolonged aPTT
- XR (); 80% Hemophilia A ( F8), 20% Hemophilia B ( F9)
- S&S Hemarthrosis (esp. knee jt.), Hematoma (intramuscular, retroperitoneal), Bruise, Mucosal
bleeding (GI (e.g. bleeding per gum), GU), Intracranial bleeding
Hemophilia C ( F11) mild bleeding
hemarthrosis misleading term Hemophilia C Factor
11 deficiency
- Severity
Normal factor activity
Mild 5-25%
Moderate 1-5%
Severe < 1%
- keep 30-60% 2-3 d
life-threatening (intracranial bleeding) keep 100% 1 wk.
- FFP factor; Cryoprecipitate factor 1, 8, 13, vWF
hemophilia A cryoprecipitate, hemophilia B FFP
hemophilia FFP
- F8
- 1 U/kg F8 2%, q 12 hr. ( T1/2)
- FFP 1 cc F8 1 U
- Cryoprecipitate 1 bag (1 U) F8 100 U
- F9
- 1 U/kg F9 1%, q 24 hr. ( T1/2)
- FFP 1 cc F9 1 U
Example:
1. 10 kg hemophilia A intracranial bleeding ?
: 100% F8 50 U/kg = 500 U cryoprecipitate 5 bag 12 hr.
2.5 bag 1 wk. #
2. 10 kg hemophilia () intracranial bleeding ?
: 100% F8 & F9
F9 100% F9 100 U/kg = 1,000 U FFP 1,000 cc
F8 100% F8 50 U/kg = 500 U FFP 500 cc
FFP 1,000 cc stat 12 hr. 500 cc #
- prolonged VCT Severe coagulation defect (coagulation factor < 1%)
- DDAVP (Minirin) = ADH
1. IV Mild hemophilia A F8 clotting activity
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Oncology
- Tumor Neuroblastoma ( malignant)
Tumor Wilm tumor (Nephroblastoma)
- Neuroblastoma VS Wilm tumor
Neuroblastoma Wilm tumor
Abdominal pain, Bone pain,
Symptom Symptomless
Metastasis, Pale
Consistency Firm, Irregular surface Tense cystic or firm, Smooth surface
Intra-abdominal palpable, may Bimanual palpable,
Palpable
Cross midline usually Unilateral
Calcification Common Rare
Multiple intra-abdominal
Well-circumscribed mass,
CT abdomen lymphadenopathy with
may extend to IVC
extensive organ involvement
- Tumor marker Neuroblastoma NSE (Neuron-specific enolase)
Oncologic emergencies
Febrile neutropenia (FN)
- FN criteria DX
1. T 38.5C 1 peak T 38C 2 peaks ( 1 hr.)
( Medicine 38.3C 38.5C 1 peak)
2. ANC < 500 ANC < 1,000 with predicted nadir < 500
Tumor lysis syndrome (TLS)
- TLS electrolytes PUKCa (P, Uric acid, K; Ca)
1. Hydration e.g. 1.5M; keep Urine sp.gr. < 1.010
2. Alkalinization keep urine pH 6.5-7.5
- TLS Uric acid toxic tubule ATN (
renal failure CMT , dialysis
- TLS Burkitt's lymphoma q 6 hr.
(5 CMT) TLS hydration
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Neurology
Seizure
- Seizure = Abnormal excessive neuronal discharge
- Convulsion = Seizure involuntary contraction voluntary muscles
- Epilepsy () = Unprovoked seizure 2
- Dx & DDx of seizure seizure/epilepsy ( stereotype & rhythmic
Paroxysmal non-epileptic event) cause ( Hx, PE, Lab)
- Cause of seizure ABCDE
1. Alcohol withdrawal
2. Brain tumor
3. Cerebrovascular disease (CVD)
4. Degenerative disorder, Drugs (-lactams, INH, CsA)
5. Electrolyte imbalance
- Classification of seizure
1. Generalized seizure unconscious; metabolic disorder genetic;
1) Infantile spasm (Salaam seizure)
- < 1 yr.
- Tuberous sclerosis (TS) common cause ( Hypopigmented macule;
Retinal patch fundoscopy)
-
(1) Suddenly flex arms, forward flex trunk, extend legs sec
(Cluster of repetitive seizure)
(2) EEG: Hypsarrhythmia
- Vigabatrin ( Visual field defect )
- ~ 5 yr. seizure type
2) Absence seizure
(1) Typical
(2) Atypical
3) Tonic seizure
4) Tonic-clonic seizure
5) Atonic seizure
6) Myoclonic seizure
2. Partial seizure (PS) conscious ; structural abnormality;
1) Simple PS conscious
2) Complex PS conscious complete
- PS 1) Aura, 2) Todd's paralysis
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- seizure
- 85% Remote symptomatic
- Congenital brain malformation
- Hypoxic ischemic encephalopathy (HIE)
- Tuberous sclerosis (TS)
- Metabolic
- 15% Cryptogenic
- Vigabatrin
2. Benign Rolandic epilepsy (BRE, Benign childhood epilepsy with centrotemporal spike)
- Age 3-13 yr.
- PS evolving to secondarily GTC
- ()
- , ,
- EEG: Centrotemporal spike 1 side/both sides
- AED, 15 yr.
3. Lennox-Gastaut syndrome (LGS)
- Age 3-5 yr.
-
- 25% infantile spasm
- Delayed development
- EEG: Slow spike & waves
- Remote symptomatic: Congenital brain malformation, HIE, CNS infection
- Valproate ( )
4. Childhood absence epilepsy (CAE) / Juvenile absence epilepsy (JAE)
- Age 4-8 yr. (CAE) / 8-12 yr. (JAE)
- Absence seizure (CAE GTC 40%, JAE GTC 80%)
- aura, post-ictal, automatism, 5-10 s, > 10 /
- Hyperventilation test (2-3 min)
- EEG: 3 Hz Generalized spike & waves
- Valproate (1st line) / Lamotrigine (2nd line) 2 yr.
5. Juvenile myoclonic epilepsy (JME)
- Age 12-18 yr.; FHx
- Myoclonic then GTC
- (/)
- EEG: 4-6 Hz Spike & waves + Polyspike & waves
- Valproate
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Febrile seizure
- Febrile seizure = Seizure CNS infection; 3 mo.-5 yr.
2
1. Simple GTC seizure < 15 min, D.1-2 , , post-ictal
2. Complex ( definition)
1) Focal / Partial seizure
2) Ictal phase > 15 min
3) ()
- DDx 4
1. CNS infection , , PE: Tense bulging fontanelle (), Signs of
meningeal irritation
2. Metabolic disturbance Hypoglycemia, HypoNa+, HyperNa+; Hx /Vomiting/Diarrhea
3. Intoxication Salicylate, INH
4. Epilepsy AED
- Septic W/U: PE source of infection (e.g. Pharyngitis, Otitis media, CNS infection headache, stiff
neck, ), LP R/O CNS infection (esp. infant) CSF cell count, cell diff., sugar, protein, C/S.
CBC, H/C
< 18 mo. LP ( S&S )
> 18 mo. , Simple febrile seizure, PE source of infection
LP
Electrolyte, Toxicology ; EEG Simple febrile seizure
- ER , O2, Valium 0.3 mg/kg/dose (
38.9C)
- Reassure ,
, Paracetamol
W/U , 30-50%
- Simple febrile seizure AED > 5 yr.
Valium 1 mg/kg q 8 hr.
- Complex febrile seizure Epilepsy AED prophylaxis: PNB 5 MKD Valproic
acid (Depakine) 20-60 MKD; Valium prophylaxis
Sturge-Weber syndrome (SWS)
- SWS 3 types
Type 1: Port-wine stain + Brain angioma
Type 2: Port-wine stain
Type 3: Brain angioma
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Neuromuscular diseases
- Approach
1. Hx
- Onset
Acute/Subacute: AIDP, AFP
(AIDP = Acute inflammatory demyelinating polyradiculopathy, AFP = Acute flaccid paralysis)
Chronic: DMD/BMD
- Fluctuation of symptom: MG
- Associated symptom (): Peripheral n.
- Age
Neonate: SMA
Early childhood: DMD
Late childhood: BMD, MG
- Sex
Male: DMD/BMD
- FHx
XR: DMD/BMD
AR: SMA
AD: HMSN
2. Where is the lesion? Acute / Chronic
Lesion Acute Chronic
SC ATM
AHC Poliomyelitis, SMA
Non-poliomyelitis
PN GBS HMSN
NMJ MG
Muscle Acute myositis, DMD/BMD,
Dermatomyositis Congenital myopathy
(SC = Spinal cord, AHC = Anterior horn cell, PN = Peripheral n.)
Acute transverse myelitis (ATM)
- ATM = acute inflammation SC,
- PE: Weakness at lower limb ( upper limb ), Sensory level loss (60% T, 25%
C), Bowel/bladder dysfunction, Hyperreflexia / Areflexia (Spinal shock)
- Investigation
1. LP: cell , protein
2. MRI T2W1: Increased signal inflammation
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- Management: Supportive
Poliomyelitis
- Poliomyelitis Hallmark Asymmetry of flaccid paralysis
- Dx Stool for Polio virus
- Management: Supportive
Spinal muscular atrophy (SMA)
- SMA AR; mutation gene p arm chromosome 5 Progressive
degeneration of Anterior horn cell SC Motor nuclei Brain stem
- PE: Hypotonia, Hyporeflexia, Tongue fasciculation
Anterior horn cell signs: Tongue fasciculation, 2 Tremor-like,
Minimyoclonus
- SMA 3 major types
1. Type 1 (Werdnig-Hoffman disease) = Non-sitter Age onset < 6 mo.; severe ,
, PE: Areflexia
2. Type 2 = Sitter Age onset > 6 mo.; , PE: Hypotonia, Hyporeflexia, Tongue
fasciculation
3. Type 3 = Walker Age onset > 18 mo.; Hx ()
- SMA Respiratory complication (end up tube
death )
- Investigation: SMN gene ( 95%)
- Management
1. Supportive
2. Genetic counseling
Guillain-Barr syndrome (GBS)
- GBS AFP ; Peripheral n.; Ascending paralysis
( Descending paralysis: Botulism)
- 70-80% URI / GI symptom / Vaccination , ,
/ (Glove-stocking pattern)
- PE: Symmetrical weakness, Hyporeflexia / Areflexia
- Investigation
1. LP: Cyto-albumino dissociation ( cell CSF protein )
2. PNCV (Peripheral n. conduction velocity): Demyelination
- Complication : Respiratory failure, Autonomic instability, Pressure sore, Sepsis
- Management
1. Supportive
2. IVIG Respiratory failure /
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Genetics
Down syndrome (DS)
- Down syndrome clinical manifestation
1. Skin & hair Alopecia areata (AA), Straight pubic hair, Dry skin
2. HEENT
Head Brachycephaly, Microcephaly, Flat occiput, Flat face, Facial hypoplasia
Eye Upslant palpebral fissure, Epicanthal fold
Ear Small prominent low-set ear, Hearing loss (HL)
Nose Flat nasal bridge with small nose
Mouth Protrude tongue
3. Hand Simian crease, Broad hand with short metacarpals, Clinodactyly of 5th finger
4. Foot Wide gap between 1st & 2nd toes
5. CVS Endocardial cushion defect (ECD), VSD, ASD, TOF, PDA
6. GI Esophageal/Duodenal atresia, TE fistula
7. GU Hypoplastic pelvis with wide flat iliac wings, Small penis with decrease testicular volume
8. NS Hypotonia, Moderate MR, Short stature
- Down syndrome risk
1. Atlanto-axial instability
2. Serious otitis media, Hearing loss
3. Sinus / NL duct / Pulmonary infection
4. Autoimmune, Hypo/Hyperthyroidism
5. Leukemia
6. Cataract, Myopia
7. DM
8. OSA
9. Cardiac abnormality
- F/U
1. X-ray Lateral C-spine (Flex-Extend) at 5-18 mo., 10-12 yr., 18 yr., then q 10 yr.
2. TFT at Newborn, then q 2-3 yr.
3. Eye examination at 6-12 mo., then q 4 yr.
4. Cardiac evaluation (CXR, Echo, EKG) at < 6 mo.
Turner syndrome
- webbed neck Turner syndrome (XO)
webbed neck Noonan syndrome (AD)
Neurofibromatosis (NF)
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