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🏥 PEDIATRIC JAUNDICE IN OLDER CHILDREN - EXAM CRASH COURSE

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📌 PART 1: TABLE 362-2 PDF

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🔴 JAUNDICE IN OLDER CHILDREN - OVERVIEW

• Jaundice = total bilirubin >1 mg/dL
• Much less common in older children vs neonates
• Two types:

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🟡 UNCONJUGATED HYPERBILIRUBINEMIA

Gilbert Syndrome

• Rare autosomal recessive
• Defect in UDPGT (UDP glucuronosyl transferase)
• Mild unconjugated bili (usually ≤5 mg/dL); otherwise healthy
• Triggered by: infection, fasting, vigorous exercise

Crigler-Najjar Syndrome

• Type 1: Complete absence of UDPGT - severe, life-threatening
• Type 2: Limited activity of UDPGT - less severe
• Phenobarbital can induce the enzyme in Type 2 (not Type 1!)

💡 MCQ trick: Phenobarbital works in Crigler-Najjar Type 2 only

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🟠 VIRAL HEPATITIS

Key Distinguishing Features (HIGH-YIELD!)

HAV Key Points

• RNA virus, Picornavirus family
• Most common cause of viral hepatitis
• Asymptomatic in <5 yr old, symptomatic in older
• Communicable: 2 weeks BEFORE to 7 days AFTER jaundice
• Duration: 7-14 days
• Dx: Anti-HAV (detectable when symptoms appear; positive for 4-6 months)
• Vaccine: at 12 months, booster at least 6 months after
• Complications: Acute liver failure, prolonged cholestatic syndrome

HBV Key Points

• dsDNA virus, Hepadnaviridae family; 8 genotypes (A-H); B & C prevalent in Asia
• Perinatal transmission: up to 90% if mother is HBeAg-positive and untreated
• Does NOT spread by breastfeeding, kissing, hugging, or sharing utensils
• Pathogenesis: immune-mediated (predominant in HBV)
• Chronic treatment: only in immune-active form (elevated ALT/AST + progressive fibrosis)

Lab findings in Viral Hepatitis

• ALT/AST elevated - does NOT correlate with severity
• Rapidly falling ALT + rising PT = POOR outcome!
• Conjugated bilirubin normalizes first (before ALT/AST)
• ALP, 5-nucleotidase, GGT, urobilinogen elevated during acute phase

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🔵 AUTOIMMUNE HEPATITIS (AIH)

Type 1 vs Type 2 - Classic MCQ!

Diagnosis Criteria

• Positive: Female + elevated transaminases (NOT ALP) + elevated γ-globulin + autoantibodies
• Negative: no viral markers, no drug/blood exposure, negligible alcohol

Labs

• Aminotransferase: 100-300 IU/L (up to 1,000 IU/L in symptomatic)
• Bilirubin: 2-10 mg/dL
• IgG >16 g/L (hypergammaglobulinemia)
• Associated HLA: DR3, DR4, DR7

Treatment

• Prednisone 1-2 mg/kg/day, then taper to 0.1-0.3 mg/kg/day
• Poor responders add: Azathioprine or 6-mercaptopurine
• Goal: ALT <2x upper limit of normal

• 75% remission on initial therapy; 50% relapse after withdrawal

• Progression to cirrhosis → liver transplant

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🟢 DRUG-INDUCED LIVER INJURY (DILI) - Table 363-1

Drug Patterns (HIGH-YIELD TABLE!)

CYP450 Inducers (memorize!)

DILI Patterns

• Hepatitic: fatigue, anorexia, vomiting; isolated aminotransferase elevation
• Cholestatic: jaundice, pruritus; elevated ALP & GGT; mild aminotransferases
• Mixed: both
• Drug hypersensitivity: fever, rash, eosinophilia, lymphadenopathy, multi-organ

Treatment

• Acetaminophen → N-acetylcysteine (within 16 hr!)
• Valproic acid → IV L-carnitine

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🟣 WILSON'S DISEASE

• Autosomal recessive copper storage disease
• Defect in ATP7B gene → defective ceruloplasmin incorporation → copper accumulates
• Hepatolenticular degeneration
• Hepatic involvement precedes neurologic manifestation by up to 10 yr
• Acute liver failure: 3x more common in girls
• Manifests as early as 2-3 yr (complete gene absence)

Think Wilson's when:

Treatment

• Dietary copper restriction <1 mg/day (avoid liver, shellfish, nuts, chocolate)
• D-Penicillamine (chelation)
• Trientine (alternative chelation)
• Zinc 25 mg 3x/day in children >5 yr (adjuvant/maintenance/primary)
• Liver transplant: 85-90% survival, curative

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🟤 CHOLEDOCHAL CYST

• Congenital dilation of biliary tree
• 75% cases manifest during childhood
• Classic triad in older children: Abdominal pain + Jaundice + Mass
• Infants: cholestatic jaundice (dark urine, acholic stool)
• Acute cholangitis: fever + RUQ pain + jaundice + leukocytosis
• Dx: Ultrasonography or MRCP
• Treatment: Primary excision + Roux-en-Y choledochojejunostomy

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⚪ LIVER ABSCESS

• Usually seen <6 yr old
• 70% solitary; 75% in right lobe
• Most common organism: S. aureus, Streptococcus spp., E. coli, Klebsiella, Salmonella
• In endemic areas: Entamoeba histolytica (amoebic abscess)
• 50% are polymicrobial; cryptogenic = often monomicrobial
• Imaging: Elevated hemidiaphragm on CXR; Ultrasound/CT preferred
• Treatment:
  • Broad-spectrum antibiotics IV 2-3 weeks, then oral to complete 4-6 weeks
  • Amoebic: Metronidazole + Paromomycin
  • Multiple/large abscess: CT-guided aspiration or surgical drainage

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🔴 ACUTE ACALCULOUS CHOLECYSTITIS

• Uncommon in children; no gallstones
• Most common cause: Infection (Streptococci groups A & B, gram-negative - Salmonella, Leptospira; Parasites - Ascaris, Giardia)
• Dx: US - enlarged, thick-walled gallbladder without calculi; daily US recommended (can become gangrenous)
• Treatment: Antibiotics + Cholecystectomy

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📌 PART 2: TYPES OF STONES / CHOLELITHIASIS PDF

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💛 CHOLELITHIASIS (Gallstones)

• Rare in healthy children; common with predisposing factors
• 70% pigment stones (most common in children!)
• 15-20% cholesterol stones
• <15% mixed

Pathophysiology

• Cholesterol stones: gallbladder stasis + excess cholesterol → supersaturation → crystallization
• Co-factors: mucin, calcium, apolipoprotein, lecithin
• Biliary pseudolithiasis: Calcium-Ceftriaxone salt precipitates
  • Prolonged high-dose Ceftriaxone (>40% treated ≥10 days)
  • Presents with jaundice, abdominal pain
  • Spontaneously resolves within several months

Clinical Presentation

• >50% have symptoms
• 18% present with complications (pancreatitis, choledocholithiasis, acute cholecystitis)
• Key feature: recurrent colicky pain in RUQ
• Older child: intolerance for fatty foods

Diagnosis

• Ultrasonography = method of choice
• Plain x-ray: only radiopaque (calcified) stones visible; radiolucent = not seen
• Hepatobiliary scintigraphy (HIDA scan): adjunct; shows cholecystitis

Treatment

• Symptomatic: Laparoscopic cholecystectomy
• Operative cholangiography: at surgery to detect unsuspected CBD stones
• ERCP with extraction: older children and adolescents
• Asymptomatic: May resolve spontaneously; advise parents

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🔴 QUICK COMPARISON TABLE (HIGH-YIELD!)

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🟠 ACUTE PANCREATITIS

Etiology (Children)

• Most common pancreatic disorder in children
• Top causes:
  1. Blunt abdominal injuries (most common cause)
  2. Hemolytic uremic syndrome (HUS)
  3. Inflammatory bowel disease
  4. Biliary stones / microlithiasis
  5. Drug toxicity: valproic acid, L-asparaginase, 6-mercaptopurine, azathioprine

Pathophysiology

• Ductal disruption → premature activation of trypsinogen → trypsin → activates proenzymes → autodigestion

Severity Classification (HIGH-YIELD!)

💡 Cullen sign = periumbilical bluish discoloration; Grey Turner = flanks - both = Severe pancreatitis

Diagnosis: Lipase vs Amylase

• CT scan for severe: pancreatic enlargement, hypoechoic edematous pancreas, fluid collections, abscess
• MRCP/ERCP: recurrent or non-resolving pancreatitis

Treatment

• Prognosis: Uncomplicated → recovery in 4-5 days

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🔵 PORTAL HYPERTENSION

• Portal pressure >10-12 mm Hg (normal: 7 mm Hg)

Causes by Location

💡 Suspect portal vein obstruction in a child <6 yr with isolated splenomegaly who had a complicated neonatal course

Clinical Manifestations

1. Most common: Esophageal variceal bleed (hematemesis/melena)
   • Hard to control if secondary to cirrhosis
2. 2nd most common: Splenomegaly (with/without hypersplenism)
3. Ascites (more common in intrahepatic disease)
4. Growth retardation (cirrhosis > extrahepatic)
5. Hepatopulmonary syndrome: ≥10% of patients; arterial oxygenation defect from intrapulmonary microvascular dilation

Diagnosis

• Doppler US: reversal of portal flow (hepatofugal flow) = variceal bleeding
• Endoscopy: most reliable for detecting esophageal varices + source of GI bleed
• Best noninvasive predictors: Platelet count + spleen length + serum albumin
• Contrast echocardiography: for hepatopulmonary syndrome - delayed appearance of microbubbles in left heart

Treatment - Acute Variceal Bleed

• Fluid resuscitation → RBC transfusion
• IV H2 blocker or PPI
• Vitamin K + platelets/FFP (if coagulopathy)
• Vasopressin → increases splanchnic vascular tone → decreases portal blood flow
• Octreotide → decreases splanchnic blood flow
• Endoscopic: rubber band ligation (more effective) or sclerotherapy
• Sengstaken-Blakemore tube: if persistently bleeding

Surgical Options

• TIPS (transjugular intrahepatic portosystemic shunt): risk for hepatic encephalopathy
• Distal splenorenal shunt: for esophageal variceal bleed; best with well-preserved liver
• Rex shunt: for portal vein thrombosis; restores physiologic flow; improves growth and cognition
• Liver transplant: for intrahepatic disease / cirrhosis / hepatopulmonary syndrome
• β-blockers (non-selective): long-term; reduce heart rate by ≥25%

Prognosis

• Intrahepatic: poor prognosis
• Portal vein obstruction: >50% experience bleeding during adolescence; can develop portal biliopathy (bile duct compression from collaterals)

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🟣 FULMINANT HEPATIC FAILURE (Acute Liver Failure)

Definition (Pediatric ALF)

• Acute liver injury <8 weeks duration
• No chronic liver disease
• Coagulopathy:
  • PT >15 sec or INR >1.5 not corrected by Vit K + encephalopathy, OR
  • PT >20 sec or INR >2 (regardless of encephalopathy)

Causes

• Viral: HAV, HBV, HCV, HEV + EBV, HSV, CMV, Adenovirus
• Drugs: Acetaminophen = most common
• Autoimmune hepatitis
• Metabolic: Wilson disease, Galactosemia, Hereditary tyrosinemia
• Vascular/ischemic

Warning Signs

• Rapid decrease in liver size without clinical improvement = OMINOUS sign
• Progressive jaundice, fetor hepaticus, fever, ascites, diathesis

Multi-Organ Involvement (Memorize!)

• Brain: hepatic encephalopathy, cerebral edema, intracranial hypertension, seizures
• Kidney: hepatorenal syndrome
• Lungs: ARDS, hepatopulmonary syndrome
• Heart: high output state
• Bone marrow: anemia, thrombocytopenia
• Liver: hypoglycemia, hyperammonemia, coagulopathy, lactic acidosis

Labs

• Bilirubin + aminotransferases: markedly elevated
• Aminotransferase decreases as patient deteriorates (bad sign!)
• PT/INR prolonged (often don't normalize)
• Hypoglycemia, hypokalemia, hyponatremia, metabolic acidosis

Treatment - Specific Antidotes (HIGH-YIELD!)

• Liver transplant: hepatic encephalopathy stages III-IV

Prognosis

• 90% survival: Acetaminophen OD + Fulminant Hepatitis A
• 40% survival: Idiopathic + acute-onset Wilson disease
• Extremely poor: stage IV coma
• Associated with mortality: Age <1 yr, Stage 4 encephalopathy, INR >4

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🟡 ASCITES

• Hallmark: abdominal distention
• Signs: bulging flanks, shifting dullness, fluid wave, puddle sign
• Moderate: early satiety, dyspnea
• Tense: umbilical herniation

SERUM-ASCITES ALBUMIN GRADIENT (SAAG)

Treatment

• Sodium restriction + Spironolactone + Furosemide
• Albumin infusion
• Large volume paracentesis or TIPS
• Risk: Spontaneous bacterial peritonitis (SBP)

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🔴 PERITONITIS

Primary (Spontaneous Bacterial) Peritonitis

• No intraabdominal source
• Most common in: Cirrhosis and Nephrotic syndrome
• Pathogen: Pneumococci most common; also Gr A strep, E. coli, Klebsiella
• Paracentesis: WBC ≥250 cells/mm³ with >50% PMN
• Treatment: Cefotaxime (broad spectrum initially); vancomycin for resistant pneumococci; 10-14 days
• Monomicrobial

Secondary Peritonitis

• Has an intraabdominal source
• Most common: Perforated appendix
• In neonates: Necrotizing enterocolitis
• Paracentesis: WBC ≥250 + elevated protein (>1 g/dL) + low glucose (<50 mg/dL)
• X-ray: Free air in peritoneal cavity
• Treatment: Polymicrobial coverage
  • Lower GIT perforation: Ampicillin + Gentamicin + Clindamycin/Metronidazole
  • PD catheter-related: Intraperitoneal Cefepime or Cefazolin + Ceftazidime
• Surgery once stabilized

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⚡ RAPID MCQ FIRE - KEY NUMBERS

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🔑 TOP MCQ TRIGGERS - WHAT DIAGNOSES WHAT?

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