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22 Cards in this Set
- Front
- Back
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What is the most likely cause of indirect hyperbilirubinemia in a child? Direct?
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Indirect- physiologic reason due to increased production of bilirubin or decreased conjugation.
Direct- pathologic reason due to obstruction. |
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Why are newborns predisposed to jaundice?
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Increased RBC mass
Decreased RBC life span Decreased conjugation lower hepatic perfusion Lower biliary excretion |
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What role does breast milk play in physiologic jaundice?
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Beta-glucuronidase in the breast milk unconjugates bilirubin so that it can be reabsorbed and recycled.
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What are risk factors for neonatal jaundice?
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Male
Bruising from delivery Asian Maternal diabetes Permaturity |
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Why does breastfeeding jaundice result? What are associated symptoms? What is the time frame?
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Ineffective breastfeeding leading to excessive weight loss, hypovolemia, and hypernatremia.
2-3rd day |
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What causes breast milk jaundice?
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Increased concentrations of beta-glucuronidase in milk.
2-3rd week |
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What are risk factors for severe hyperbilirubinemia?
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Jaundice in first 24 hours
ABO incompatibility Excessive bruising Exclusive breastfeeding Asian Prematurity Polycythemia Infection |
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When is jaundice concerning?
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Present at birth or within 24 hours.
Serum bilirubin increases more than 5 mg/dl/24 hours Direct bilirubin > 2mg/dl Persists longer than 2 weeks Vomiting, lethargy, poor feeding, weight loss, apnea, bradycardia. |
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How does unconjugated bilirubin cause toxicity?
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Bilirubin can be released from albumin by drugs (Bactrim).
Crosses blood brain barrier and causes encephalopathy. Kernicterus results from deposition of bilirubin in basal ganglia and brainstem nuclei. |
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What are symptoms of bilirubin toxicity?
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Early: lethargy, poor feeding, hypotonia, seizures.
Later: abnormal rigidity, hypertonia, shrill cry. Chronic: choroid movements, hearing loss, squinting, mental retardation. |
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What are inherited causes of unconjugated hyperbilirubinemia? Conjugated?
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Unconjugated- Gilbert's, Crigler-Najjar
Conjugated- Dubin-johnson; Rotor syndrome |
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What diseases can cause conjugated hyperbilirubinemia?
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Extrahepatic biliary atresia
alpha-1 antitrypsin deficiency Idiopathic hepatitis Infection, drugs, choledochal cyst |
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What is extrahepatic biliary atresia? How is it diagnosed in neonates? What surgical treatment is performed? When does this need to be performed?
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Obliteration of entire biliary tree.
Diagnosed by absent gallbladder on ultrasound, no excretion on scintigraphy. Kasai portoenterostomy performed by connecting the small intestine to the liver. Must be done before 2 months old. |
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How does alpha-1 antitrypsin deficiency cause liver problems? What is the presentation?
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Accumulation of misfolded protein in the liver.
Presentation: jaundice, acholic stools, hepatomegaly, elevation of conjugated bili and transaminases. |
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What is idiopathic neonatal hepatitis? How does it present?
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Prolonged conjugated bili and inflammation of liver without obvious etiology.
Presents with vomiting, poor feeding, elevated LFTs |
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What is Reye's syndrome? What is the presentation?
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Encephalopathy and hepatic failure caused by viral illness treated by aspirin.
Presentation: altered mental status, elevated ICP, increased liver enzymes, hyperammonemia |
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What is the presentation of annular pancreas? What is it associated with?
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Presents with high level obstruction at the duodenum.
Associated with maternal polyhydramnios, Down syndrome, imperforate anus. |
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How is pancreatic exocrine function determined?
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Fecal fat measure by sudan stain
Fecal elastase |
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What are respiratory manifestations of cystic fibrosis?
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Bronchectasis develops early.
Persistent bronchial obstruction leads to infection by S aureus or P aeruginosa. Sinusitis |
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What are gastrointestinal manifestations of cystic fibrosis?
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GERD
Chronic pancreatitis, diabetes Pancreatic obstruction- steatorrhea Meconium ileus Cholestasis Distal ileum obstruction Rectal prolapse |
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What is meconium ileus? What is it associated with?
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Obstruction in terminal ileum by thick meconium with microcolon after the obstruction.
90% associated with CF. |
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What are symptoms of meconium ileus? What is the appearance on xray? How is it treated?
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Failure to pass meconium within first day of life; abdominal distension, bilous emesis.
Xray: dilated loops of bowel, soap bubble appearance, microcolon. Tx: enema |
