- Occurs in coinfection with Hepatitis B --> worse course - Eastern Europe and Mediterranean - Diagnosis = IgM to Hepatitis D

- Very similar to Hepatitis A - Short incubation period - Epidemics/endemics with contaminated water - Developing countries - No chronic disease - Hepatitis E Ab test through CDC

Gastroenterology Flashcards by Khristine Bautista

+ reducing substances in urine, fulmninant neonatal hepatic failure

Galactosemia

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Liver failure after introduction of fruit juices

Fructosemia

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Fulminant neonatal hepatic failure + succinylacetone in urine

Tyrosinemia

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Test for diagnosing hepatitis A

Hepatitis A IgM

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Percentage of children infected with Hepatitis A that go on to fulminant liver failure

<1%

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Extrahepatic manifestations like rash and arthralgias

Think Hepatitis B

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Percentage of neonates that will not clear the hepatitis B antigen

90% versus <10 adults

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This chronic hepatitis carrier state has a high association with cirrhosis and hepatocellular carcinoma

Hepatitis B

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Positive HepBsAg

Acute infection

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HBeAg

All about level of viral replication

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HepBsAb

Only Ab that gives protection from the Hepatitis B virus

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HepBcAb

Rises early in course of Hepatitis B, before HepBsAb

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Hepatitis A and B treatment

Symptomatic management

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Hepatitis B prevention in children born to mothers with Hepatitis B

Hepatitis B vaccine + HBIg shortly after birth

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Which of the Hepatitis Viruses is a DNA virus?

Hepatitis B

Hepatitis A + C are RNA viruses

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Percentage of children exposed to Hepatitis C that develop chronic disease

75%

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These two hepatitides have a high rate of cirrhosis and hepatocellular carcinoma

Hepatitis B + C

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Gold standard for Hepatitis C diagnosis

Hepatitis C DNA PCR

Hepatitis C Ab has a high rate of false positive an negative results

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Treatment for chronic Hepatitis C

Ribavirin and Interferon

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Hepatitis D

Occurs in coinfection with Hepatitis B –> worse course
Eastern Europe and Mediterranean
Diagnosis = IgM to Hepatitis D

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Hepatitis E

Very similar to Hepatitis A
Short incubation period
Epidemics/endemics with contaminated water
Developing countries
No chronic disease
Hepatitis E Ab test through CDC

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Other viruses that cause hepatitis in childhood…

EBV –> transaminitis, +/- jaundice, adenopathy + hepatoSPLENOmegaly
CMV, HSV, Varicella in immunocompromised pts

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Drugs associated with hepatotoxicity

Acetaminophen
Isoniazid
Anticonvulsants - Valproic Acid
Anesthetics - Halothane
Onc - MTX, mercaptopurine, 6MP

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This mushroom, when ingested, leads to hepatitis

Amanita phalloides

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- Viral illness - Aspirin use - Acute liver failure - Cerebral edema

Reye's Syndrome

Cutoff for chronic hepatitis

6 months

Management of chronic liver failure

- Symptomatic - Ascites --> fluid + sodium restriction - Encephalopathy --> limit protein to 1-1.5g/kg/day, lactulose, rifaximime - Liver transplantation ultimately

Chronic hepatitis B treatment

- Interferon alone | - +/- Lamiduvine - develop resistance

Chronic hepatitis C treatment

Ribavirin and Interferon - combination therapy better

Liver disease in childhood, lung disease in adulthood

Alpha 1 antitrypsin | - ZZ phenotype

Findings in Wilson's disease

- Deposition of copper in CNS, liver, kidney - Increased: 24 urine Cu exretion, copper content in liver on biopsy - Decreased: serum Cu, serum ceruloplasm

Teenager with acute liver failure and hemolytic anemia or...chronic hepatitis leading to cirrhosis Can also present with neurological symptoms - personality changes

Wilson's disease Kayser Fleisher rings - (liver dz with eye image -- think Wilson's or Alagille's Syndrome [posterior endotoxin])

Treatment for Wilson's

Penicillamine or Trientene

Tyrosinemia

- AR - can present as acute liver failure in an infant - untreated, by age 3, high percentage will develop hepatocellular carcinoma - May have neurologic symptoms and renal impairment (lose phosphorus in urine) - Rx: limit tyrosine in diet, NTBC stops degradation of tyrosine into toxic metabolites - Children with cirrhosis, transplant?

Progressive Familial Intrahepatic Cholestasis

Defects of bile acid metabolism or secretion - mimic chronic hepatitis - PRURITUS significant (also in Alagille's) - Rx: symptom management, liver transplantation

Adolescent females | - Elevated total protein with normal/low albumin

Autoimmune hepatitis | Rx: immunosuppression, steroids for induction, mercaptopurine or azothioprine for maintenance

Most common form of obstructive jaundice occuring in the neonatal period

Biliary Atresia - 1:10,000 - Full term infants with conjugated hyperbilirubinemia in 1st month of life - Classic: complete atresia of external biliary system (bile ducts draining liver are not formed properly) - Jaundice >2 weeks of life must be worked up - Rx: Kasai or portoenterostomy procedure - must be done before 3 months of life because have higher success rates

Percentage of biliary atresia pts, even with Kasai procedures, that go on to require liver transplantation

75%

- Paucity of intrahepatic bile ducts - within liver parenchyma (portal triads 50% missing bile duct) - Cardiac anomalies - PPS, TOF - Short stature - Typical facies - triangular, elongated facies - Butterfly vertebrae, especially in thoracic region

Alagille's Syndrome - JAG1 mutation

School-aged female + abdominal mass + RUQ pain + intermittent jaundice +/- conjugated hyperbilirubinemia

Choledochal cyst

Chronic inflammation around bile ducts --> focal areas of fibrosis --> narrowing and dilation of bile ducts - rare in children - associated with UC - may present with fever, abd pain +/- jaundice - liver may be enlarged

Sclerosis cholangitis | Dx: percutaneous cholangiogram or ERCP --> see "beads on a string"

Individuals with sclerosing cholangitis are at increased risk of developing this cancer

Cholangiocarcinoma

Gall stones in children

R/o hemolytic disorders, think about CF

Colicky RUQ pain referred to right scapula + jaundice + elevated bili + elevated AP

Cholecystitis - E.coli, enterococcus - Dx: US (thickening of gall bladder wall) or HIDA scan

Acalculous cholecystitis, associations...

- Systemic infections (enteric infections - salmonella/shigella, viral) - Rx: cholecystectomy one inflammation improves

Neonatal metabolic diseases that may present as fulminant liver failure

Tyrosinemia, Fructosemia, Galactosemia

Epigastric pain, vomiting, elevated amylase + lipase

Pancreatitis

Basal energy needs are MOST ACCURATELY calculated by...

BSA

The predominant protein in BM that is easily digestible, promotes gastric emptying and is a protein with high quality biologic value.

Whey

Protein content in human milk

9g/L --> 7.2g/L nutritionally available | - IgA, lactoferrin and lysozyme (30%)

This type of triglyceride does not require intraluminal digestion or bile salts for absorption

MCT (8-10 carbons)

Essential fatty acids

1. Linoleic 2. Linolenic Both needed to make arachnodonic acid

1. Scaly dermatitis 2. Hair loss 3. Diarrhea 4. Poor wound healing 5. Growth failure

Essential Fatty Acid Deficiency (EFAD) In infants, can present within 2 weeks of not receiving EFAs

Patients at high risk for EFAD

1. Premies who receive inadequate linoleic acid (no IL) 2. Children with fat malabsorption from hepatobiliary or pancreatic disease 3. Children receiving long term TPN without IL

What is the protein content in breastmilk

60 whey:40 casein - mature BM 90 whey:10 casein - early BM [cow's milk formula 40:60] [cow's milk 20:80] BM IS THE WHEY TO GO!

1. CHF 2. Tachycardia 3. Peripheral edema

Wt beriberi = fluid overload THIAMIN (B1) DEFICIENCY

1. Neuritis 2. Peripheral paresthesia 3. Irritability 4. Anorexia

Dry beriberi = more neurologic THIAMIN (B2) DEFICIENCY Seen in chronic EtOH related encephalopathy

1. Seborrheic dermatitis 2. Angular stomatitis 3. Photophobia 4. Loss of visual acuity 5. Burning and itching of eyes 6. Corneal vascularization 7. Glossitis 8. Poor growth

Riboflavin (B2) deficiency [described in premies on prolonged phototx]

1. Newborns --> convulsions (1951-infant formula prep destroyed B6) 2. Older children --> peripheral neuritis (INH administration), seb deem, cheilosis, SIDEROBLASTIC ANEMIA 2/2 failure of iron utilization for Hgb synthesis

Pyridoxine (B6) Deficiency

1. Macrocytic anemia/hypersegmented neutrophils | 2. CNS changes (ataxia, peripheral neuropathy)

B12 CYANOCOBALAMIN DEFICIENCY High risk: BF infants of vegan mothers, Patients s/p distal sm bowel resection (B12 absorbed in ileum)

1. Bleeding problems (petechiae) 2. Poor wound healing (collagen synthesis) 3. Easy fractures of bone 4. Friable bleeding gums with loose teeth 5. Microcytic anemia (aids in Fe absorption)

ASCORBIC ACID (C) DEFICIENCY/SCURVY

3D's - dermatitis (sun-exposed skin), diarrhea, dementia

NIACIN (B3) DEFICIENCY/PELLAGRA

1. Macrocytic anemia/leukopenia 2. Poor growth 3. Impaired cellular immunity 4. Glossitis 5. Diarrhea 6. Neural Tube Defects

FOLATE (B9) DEFICIENCY

Goat's milk is deficient in this

Folate (B9) | Also Methotrexate impairs folate absorption

1. Dermatitis 2. Anorexia 3. Glossitis 4. Muscle pain 5. Insomnia 6. Alopecia

BIOTIN DEFICIENCY [seen in infants given TPN free of biotin]

Only 3 water-soluble vitamins that cause toxicity when given in excess:

1. Niacin 2. Vitamin B6/pyridoxine 3. Vitamin C

1. Skin flushing 2. Tingling 3. Itching 4. Dizziness 5. Nausea 6. Liver test abnormalities

Niacin toxicity

1. Sensory neuropathy 2. Progressive ataxia 3. Altered sense of touch & pain

Vitamin B6 (pyridoxine) toxicity

1. Nausea 2. Diarrhea 3. Cramps 4. Kidney Stones (oxalate and cysteine nephrocalcinosis)

Vitamin C toxicity

1. Eye symptoms - NIGHT BLINDNESS, retinal degeneration, xerophthalmia (dryness), photophobia, conjunctivitis, keratomalacia, BITOT's SPOTS 2. Follicular hyperkeratosis 3. Poor growth 4. Impaired resistance to infection

VITAMIN A (RETINOL) DEFICIENCY

1. Widening of epiphyses with thickening of wrists and ankles 2. Chostochondral beading 3. Bowing 4. Craniotabes (persistently open anterior fontanelle/softening of the skull

VITAMIN D DEFICIENCY/RICKETS

1. Hemolytic anemia in premature infants 2. Neurological changes - ataxia, hyporeflexia, decreased vibratory and position sensation, proximal muscle weakness, ophthalmoplegia/retinal dysfunction, neurocognitive changes

VITAMIN E DEFICIENCY

1. Easy bruisability/soft-tissue hemorrhage | 2. Defective bone & collagen production

VITAMIN K DEFICIENCY II, VII, IX, X, protein C&S

- HA - Anorexia - N/V - increased ICP (pseudotumor cerebri) - HSM - Bone pain - On retinoic acid

Vitamin A toxicity (can be teratogenic)

- Hypercalcemia - Anorexia - Vomiting, abd pain, constipation - Convulsions

Vitamin D

1. Myositis 2. Cardiomyopathy 3. Macrocytic anemia 4. Loss of skin and hair pigmentation

SELENIUM DEFICIENCY

1. Rash 2. Poor wound healing 3. Impaired cellular immunity 4. Anorexia 5. Diarrhea 6. Growth failure 7. Co-factor for many enzymes (low alk phos in malnourished pt)

ZINC DEFICIENCY

SKIN - bullous and pustular lesions of oral, anal, genital areas & extremities Alopecia Diarrhea EYES - blepharitis, conjunctivitis, photophobia, corneal opacities

ACRODERMATITIS ENTEROPATHICA - AR - Defect in zinc absorption

1. Microcytic anemia which does not respond to Fe supplementation 2. Neutropneia 3. Depigmentation of hair and skin 4. Bone abnormalities

COPPER DEFICIENCY

2-3m/o M with: - loss of milestones/neurodegenerative disease/truncal hypotonia/seizures - FTT - Lightly pigmented kinky hair - Connective tissue and skeletal abnormalities

Menkes Disease - X-linked - Impaired transport of dietary Cu from the intestine

This type of malnutrition results from a severe deficiency of protein and less than adequate caloric intake

Kwashiorkor

This type of malnutrition results from inadequate caloric intake only

Marasmus

What nutrients are deficient in Crohn's disease?

- Iron - Folic acid - Vitamin B12 - Zinc - Selenium - Vitamin ADEK - Calcium - Magnesium

Premature neonates should consume formula with increased ________-chain FA

Medium

Breath hydrogen test

- One of the best ways to detect carbohydrate malabsorption - Breath sampled after patient ingests carbohydrate substrate - Rise > 10 to 20ppm in expired hydrogen = carbohydrate malabsorption

Small intestinal biopsy

Detects celiac disease

Colonoscopy

Detection of IBD

Juices with high sorbitol

Prune Apricot nectar Apple Pear nectar

Presents at birth with diarrhea, dehydration and metabolic acidosis in breast and lactose containing formula fed infants

Congenital glucose-galactose malabsorption - AR - Fructose = only carbohydrate tolerated

- 10% eskimos have it - diarrhea, abd pain and or bloating with ingestion of sucrose - starch and cornstarch (isomaltose) also cause symptoms - symptoms variable in onset, depends on time of introduction of juices, fruits, cereals

Congenital sucrase isomaltase deficiency | - AR

Gastroenterology Flashcards

(90 cards)