Term
| Factors dependent on Vitamin K |
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Definition
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Term
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Definition
Most common: Blind upper esophageal pouch with lower fistula
Next common: Esophageal atresia without fistula, flat gasless stomach
Least common: H type, often presents later in life |
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Term
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Definition
Mid esophagus commonly
Associated with tetracyclines, NSAIDs, bisphosphonates, potassium
Adhere to esophagus causing local irritation |
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Term
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Definition
Strongly linked to PUD
Test only with urea breath test or endoscopy, not serum testing
Treat with acid blockers, clarithromycin and amoxicillin or metronidazole
Treat only for PUD or MALT |
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Term
| Defects associated with short gut |
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Definition
Duodenum: iron, folate, calcium
jejunum: no serious effects
ileum: fluid loss, bile salts, B12 |
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Term
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Definition
Defect at the umbilicus containing bowel and organs, covered by membrane
Associated with Beckwith-Wiedemann and trisomies
Can result in midgut volvulus |
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Term
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Definition
| AD Pulmonary artery stenosis, cholestasis, butterfly vertebrae, radius/ulna abnormalities, characterstic facies, elevated conjugated bilirubin, intrahepatic duct paucity |
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Term
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Definition
RNA virus
fecal-oral transmission, IgM for acute infx, IgG for previous, complications rare |
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Term
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Definition
DNA virus
HBs IgG antibody indicates immunity and is first marker to appear
HBcAg antibody (IgM) present during window;indicates previous exposure and is not conferred by the vaccine
HBs antibody appears last
Associated with polyarteritis nodosa, Hep D infection, associated with acute illness, chronic disease can lead to hepatocellular cancer |
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Term
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Definition
RNA virus
Most common cause of liver disease and transplant
acute infection usually asymptomatic, chronic form is common and can be asymptomatic, carrier or chronic
Associated with PCT, mixed cryoglobulinemia
Anti-HCV does not confer immunity; confirm with RNA viral load |
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Term
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Definition
Benign alteration in UDP-GT
Mild indirect hyperbili < 5
Stress, fasting, illness
No treatment required |
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Term
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Definition
More severe form of UDP-GT inactivity
Severe indirect hyperbili, kernicterus
Treat with photothx, liver transplant |
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Term
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Definition
Defect in conjugated bilirubin transport
Mild conjugated hyperbili
Does not require therapy |
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Term
| Alpha-1-antitrypsin deficiency |
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Definition
| Porgressive liver disease, cirrhosis, cancer, cholestatic jaundice, emphysema |
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Term
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Definition
AR
Copper metabolism disorder
Eyes, liver, kidneys, brain
Hepatomegaly, hepatitis,neurological symptoms, behavioral changes
Low serum ceruloplasmin, 24 hour urine copper, liver bx (gold standard)
Penicillamine increases copper excretion |
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Term
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Definition
Excessive iron storage
Liver, heart, pacreas, gonads, skin, joints
Adult cirrhosis, bronze diabetes
Treat with phlebotomy |
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Term
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Definition
MCC of pediatric liver transplant
Week 2-3 present with acholic stool,dark urine, hepatomegaly, hyperbilirubinemia, elevated alk phos and liver enzymes
Dx with liver biopsy and cholangiogram
Tx with Kasai procedure
At risk for liver failure, ascending cholangitis |
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Term
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Definition
Treat with bowel rest
Associated with severe illness, burn |
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Term
| Hydrops of the gallbladder |
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Definition
Associated with Kawasaki, GAS, fasting, TPN, HSP
Treat the underlying condition |
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Term
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Definition
Usually benign
Most common are hemangiomas, adenomas
Hepatoblastoma most common malignant and is assoc with elevated AFP |
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