Gastroenterology Flashcards by Jen Lim

Liver failure after introduction of juices

Fructosemia

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Reducing substances in urine

Galactosemia

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Succinylacetone in urine

Tyrosinemia

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Antibody that appears early and then fades off

IgM

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Positive HB surface antigen +/- positive HB envelope antigen

Acute Hep B infection

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Positive HB surface antibody

Previous Hep B infection

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May be the only positive marker in a patient infected with Hep B who is going to clear the virus but has not yet developed the protective antibody (anti-Hep B surface antigen)

IgM anti-Hep B core antibody

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Occurs only with co-infection with hepatitis B

Delta Hepatitis

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Type of hepatitis associated with fetal loss and higher risk of fulminant liver failure in pregnant woman

Hepatitis E

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TB drug that can cause acute hepatitis

INH

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Anticonvulsant that can cause acute hepatitis

Valproate

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Tx for acetaminophen OD

N-acetyl cysteine

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Most common mushroom to cause acute hepatitis

Amanita phalloides

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Negative Hep B surface antibody

Hep B chronic carrier state

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Indicates active Hep B replication

Positive Hep B envelope antigen

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Low serum copper and ceruloplasmin. Elevated 24 hr urine copper and liver copper content

Wilson’s disease

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Kayser-Fleischer rings

Wilson’s disease

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Conjugated hyperbilirubinemia in first 4 weeks of life (MUST evaluate any baby jaundiced beyond two weeks of life)

Biliary atresia

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Paucity of the intrahepatic bile ducts

Alagille’s syndrome

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Syndrome associated with congenital heart disease (especially pulmonary stenosis), short statute, triangular facies, butterfly vertebrae, xanthomas

Alagille’s syndrome

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Most common infections in calculus cholecystitis

E. Coli & enterococcus

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85 to 90% of kids with this will have a soy allergy

Cows milk allergy

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If fructose to glucose ratio is increased, there is a higher chance of developing

Watery diarrhea

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Present in fruit juices, excessive consumption causes diarrhea

Sorbitol

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The HLA class 1 molecule associated with Celiac

The HLA class II molecules associated with celiac

DR3 and DR7

Foamy macrophages, caused by tropherma whipplii

Whipple's Disease

Pancreatic insufficiency from deficiency in pancreatic enzyme production + bone marrow failure, cyclic neutropenia

Schwachman-Diamond Syndrome

Of the three, which has greatest likelihood of steatorrhea: 1) cystic fibrosis 2) celiac sprue 3) Schwachman Diamond

Cystic fibrosis

1) absence of ganglion cells | 2) hypertrophied nerve fibers

Hirschsprungs Disease

% body water in infants

70 to 75%

% body water in young adults

50 to 60%

Basal energy needs are most accurately calculated by:

Body surface area

Premature infants who receive inadequate linoleic acid are at high risk for what? (E.g children receiving long term TPN but no lipids)

Essential fatty acid deficiency

60% whey, 40% casein

Mature breast milk

40% whey, 60% casein

Cow's milk formula

Best formula to use in premature infants

One containing medium chain triglycerides (preterm infants have decreased amount of intraluminal bile acids and decreased absorption of LCFA and fat soluble vitamins)

Vitamin deficiency that causes macrocytic anemia (hyper segmented neutrophils) and CNS changes

B12 deficiency

Breast fed infants of vegan mothers are at high risk for

Cyanocobalamin deficiency

Patients a/p distal small bowel resection are at increased risk for what deficiency

B12 (it is absorbed in the illeum)

Goat's milk is deficient in?

Folate

Methotrexate impairs the absorption of

Folate

Vitamin K dependent coagulation factors

2, 7, 9, 10, protein C & S

Syndrome with: - Enlargement of costochondral junctions - Widening of epiphyses - Thickening of wrists - Chostochondral beading - Bowing of long bones - Softening of skull (craniotabes)

Rickets

- dry skin - alopecia - carotenemia

Vitamin A Toxicity

Paresthesias, foot and wrist drop, Wernicke encephalopathy (opthalmaplegia, ataxia, confusion)

Beriberi (Thiamin/B1 deficiency)

Cheilosis, sore tongue

Riboflavin B2 deficiency

Pellagra - dermatitis, dementia, diarrhea

Niacin B3 deficiency

Seizures

Pyridoxine B6 deficiency

Megaloblastic anemia

Cobalamin B12 and folate deficiency

Scurvy

Vitamin C deficiency

Neural tube defects

B12 deficiency

Spina bifida

Folate deficiency

Painful brittle bones, loose teeth, bleeding gums

Vitamin C deficiency (needed for collagen, affects vessels and bone junctions)

Decreased reflexes, weakness, limited ability to look upward

Vitamin E deficiency

Hemolysis, reticulocytes, nerve signs

Vitamin E deficiency

Vitamin K is necessary for which factors

2, 7, 9, 10

Measures extrinsic pathway (factor VII)

Pat

Night blindness, immune dysfunction

Vitamin A deficiency

Scaly skin, pseudotumor cerebri, hepatomegaly

Vitamin A excess

Macrocephaly, pigemented penile lesions, hamartomatous polyps

Ruvalcaba Myhre Smith

STK11 gene, hamartomatous polyps, mucho cutaneous hyper pigmentation, associated with other cancers

Peutz Jeghers

Rectal prolapse can be associated with which disorder

Pancreatic insufficiency, AR, skeletal abnormalities, neutropenia

Shwachman Diamond

Arteriohepatic dysplasia

Alagille Syndrome

AD, paucity of bile ducts, triangular face, peripheral pulmonary stenosis, posterior embryotoxon in eyes, butterfly vertebrae

Alagille Syndrome

Requires coexistent Hep B infection

Hepatitis D

20% fatality rate from this fulminate hepatitis in third trimester of pregnancy

Hepatitis E

Jaundice when ill, defect in promoter for UDP-GT (which conjugated bilirubin) so less enzyme is made to conjugate with. Result = increased level of indirect bili. No tx needed.

Gilbert Syndrome

Complete absence of UDP-GT so they don't make any conjugated bilirubin. Tx: lifetime PTX or liver transplant

Crigler Najjar Type 1

Defect in gene for conjugated bilirubin transporter resulting in mild conjugated hyper bili, no hepatocyte injury

Dubin Johnson

Partial activity of UDP-GT, does not require therapy

Crigler Najjar Syndrome Type 2

Disease of copper accumulation

Wilson Disease

Kayseri Fleischer rings, liver disease, hemolysis, psych illness, behavior changes

Wilson Disease

Tx: penicillamine

Wilson Disease

Oral contraceptives associated with hepatic vein thrombosis and liver tumors

Buds Chiari Syndrome

AR, liver disease in Norwegian newborns, severe cholestasis with lower extremity lymphedema

Aagenaes Syndrome

Jaundice at one month of life, clay colored stools, dark urine, no gallbladder on ultrasound

Biliary atresia

Definitive test for biliary atresia

Intraoperative cholangiogram

Tx for biliary atresia

Kasai procedure

Gallbladder hydrops seen in:

``` Kawasaki Strep pharyngitis Prolonged fasting TPN HSP ```

Second most common cause of exocrine pancreatic insufficiency (after CF)

Schwachman Diamond syndrome. AR