GI Flashcards
Recurrent abdominal pain definition
Once/week for last 2 months
What meds should you consider for functional abdomial pain
Anticholinergic, amitriptyline, SSRI
Definition of IBS
- Pain and relief with defection or onset associated with change in for or frequency of bowel movements
- At least once per week over the course of 2 months
- Tends to occur in families
- No organic cause
Rx for IBS
Reduce sorbitol, fructose, cruciferous veggies, increasing soluble fiber, SSRIs/TCAs
Pattern: Pain in upper abdomen, no signs of gastritis on endocsopy, esophageal pH testing
Non-ulcer dyspepsia
Pattern: acute, incapacitating, periumbilical pain; anorexia, nausea, vomiting, HA, pallor, photophobia, symptom-free periods
abdominal migraine
Test for Sphincter of Oddi Dysfunction
ERCP manometry, HIDA scan
What is the cause of familial mediterranean fever
serositis causing abdominal pain
Vomiting and fever
infection, inflammation
Vomiting and tenderness/guarding
inflammation
vomiting and tachycardia, loss of skin turgor, dry mucous membranes
Dehydration
vomiting and sclerodactylyl
scleroderma
vomiting and loss of dental enamel
GERD, bulimia
Vomiting and adenopathy
neoplasm
vomiting and high pitched bowel sounds
obstruction
vomiting and absent bowel sounds
ileus
asterixis
uremia, liver failure
Vomiting and air-filled distended loops
ileus, pseudo-obstruction
vomiting and retained food in upper endoscopy
gastroparesis
What evaluation would you do for dysphagia?
upper GI, endoscopy, esophageal manometry
4 anatomic congenital causes of dysphagia
Esophageal web, tracheobronchial remnant, vascular ring, Schatzki ring (ring of mucosa/muscular tissue in distal esophagus)
What can esophageal strictures that cause dysphagia be caused by?
caustic ingestion or chronic GERD
Ingestion of alkalis can cause what injury
deep liquefaction necrosis through all layers
Ingestion of acids cause what injury
Thick eschar that limits depth of injury, bitter taste limits ingested volume
Pattern: salivation, refusal to drink, nausea and vomiting, epigastric pain, burns or ulcerations to lips or mouth, fever and leukocytosis
Caustic ingestions
Rx of caustic ingestions
oral administration of water or milk to dilute, intubation if airway edema, EGD within 48 hours to assess burns, broad spectrum antibiotics for suspected perforation/mediastinitis
How to detect radiolucent foreign bodies in esophagus
endoscopy
Within how many hours can button batteries cause perforation?
4 hours
When does GERD peak
4 months
Risk factors for GERD
Down syndrome, MRCP, prematurity
Test for gastroparesis
nuclear med gastric emptying
Pattern: baby fails to pass meconium (multiple)
Hirschsprung, anal stenosis, intestinal peudo-obstruction, hypoTH, meconium plug
What genetic disorder predisposed to Hirschsprung?
Down
What are dx tools for Hirschsprung
unprepared barium enema, full thickness biopsy and do ACh staining
Rx for Hirschsprung’s
Colonic resection, endorectal pull-through
Pattern: delayed passage of meconium, signs of obstruction, FTT, enterocolitis, soiling rare, anal fissure/fecal impaction rare, behavioral problems rare, small stool caliber
Hirschsprungs
Pattern: frequent soiling, difficulty toilet training, stool in the rectal vault on PEx, very large stools, no FTT
Functional constipation
GI bleeding in infant
milk protein allergy
GI bleeding in child
anal fissure, juvenile polyp, swallowed epistaxis, PUD, intussusception
GI bleeding in adolescent
IBD, bacterial enteritis, anal fissure, Mallory-Weiss, polyp
Pattern: 16yo with colonic adenomas
Familial adenomatous - polyposis APC gene mutation, 100% risk ofCA
Pattern: small bowel and colonic adenomas and supranumerary teeth
Gardner’s syndrome, APC mutation, 100% risk of colon CA
Pattern: hamartomatous polyps, lip/gum freckling, requiring polypectomy
Peutz-Jegher’s; sl increase in colon CA
Pattern: hamartomatous polyps in small bowel and colon
Juvenile Polyposis Syndrome PTEN, SMAD4, BMPR1 gene mutations, 10% risk of colon CA
Pattern: colonic adenomas, endometrial/renal/GBM
Hereditary non-polyposis colon cancer
Pattern: 2.5 yo with intermittent painless rectal bleeding that can become infected, followed by intussusception and can cause obstruction
Meckel’s Diverticulum
What is the diagnosis for Meckel’s diverticulum?
Technetium-pertechnetate scan, increased sensitivity with ranitidine
What tests to do to confirm serologic H pylori cause of peptic ulcer dx?
urea breath hydrogen testing or stool H pylori antigen testing
Caveat to interpreting serologic H pylori testing
May be old infection
Rx for H pylori
PPI plus two antibiotics (amoxicillin and clarithromycin) for 14 days
What are causes of secretory diarrhea?
cholera, toxigenic e. coli, C. difficile, cryptosporidiosis
What are causes of inflammatory diarrhea
salmonella, shigella, staph aureus, yersinia, campylobacter, B cereus
Do you need to treat salmonella?
No. carriers are typically asymptomatic
Do you need to treat shigella?
Bactrim, pt always symptomatic, person to person transmission
Diarrhea and reactive arthritis
salmonella, shigella, yersinia, campylobacter
Diarrhea and bloody diarrhea
HUS
Diarrhea and short incubation
C. perfringens, B. cereus
Diarrhea and seizures
shigella
Pattern: diarrhea for >1 week, stool cultures negative
parasitic - giardia, cyclospora, isospora
Pattern: fever 1-2 days, vomiting followed by 5 to 7 days diarrhea, no stool wbcs
Viral - rota adeno astro noro
Causes of Diarrhea >1 day in first month of life
Cow’s milk protein allergy, sucrase-isomaltase, gluc-galact malabsorption, pancreatic insufficiency from Schwachman-Diamond syndrome
Causes of diarrhea in 1 month to 2 years of age
Toddler’s diarrhea, post-infectious enteropathy, parasitic (giardia lamblia), rota no nror and adeno
Suspicious of diarrhea after camping/daycare
stool antigen for giardia and treat with metronidazole
Causes of diarrhea in 2 to 18
Celiac sprue, post-infectious, excessive juice, IBS, IBD, laxative abuse
Pattern: small volume of diarrhea, bloody, mucoid, urgency and tenesmus
colitis, UC or infectious
Pattern: diarrhea, distension, gas, explosive
giardiasis or sorbital excess
Dx for celiac disease
EGD with biopsies
Dx for IBD
colonoscopy with biopsies
Dx for colitis
stool WBC’s
Dx for milk protein allergy
stool eosinophils
Dx for CF, Schwachman-Diamond
Fecal fat
Dx for giardia
stool antigen
Dx for carbohydrate malabsorption
stool reducing substances
Pattern: protracted diarrhea of infancy - longer than 2 weeks in infant (multiple)
chronic enteropathies, microvillus, inclusion disease, tufting enteropathy
Pattern: protracted diarrhea in childhood (multiple)
autoimmune enteropathy, celiac disease, post-infectious diarrhea
Best oral rehydration
pedialyte
Pattern: 10wk old with 2wk h/o watery, mucoid stools with strongs of bright red blood, gassy.
Milk protein allergy
Soy has what percentage cross over allergen with cow’s milk
40%
Can cow’s milk protein ingested by mom cross into breastmilk?
yes
What are two protein-losing enteropathy?
Lymphangiectasia, Crohn’s disease
What is Toddler’s diarrhea?
malabsorption of sorbitol and fructose
Pattern: steatorrhea, acanthocytosis
abetalipoproteinemia
Pattern: PLE, hypogamm, steatorrhea, lymphedema, lymphopenia
lymphangiectasia
What is malabsorbed in lysinuric protein intolerance
dibasic AAs
What is malabsorbed in Harnup disease
free neutral AAs
What is malabsorbed in blue diaper syndrome
tryptophan
What is malabsorbed in acrodermatitis enteropathica
zn
What is malabsorbed in Menke’s disease
cu
What is malabsorbed in terminal ileal disease (Crohn’s)
Folate, B12
Pattern: diarrhea, abdominal distension, proximal muscle wasting, weight loss, anorexia, abdominal pain
Celiac disease
Pattern: diarrhea, anemia, dermatitis herpetiformis or other immune disease
Celiac disease
What HLA class I molecule is associated with celiac disease?
gliadin, DQalphaBeta, haplotypes found in >95% of patients
Celiac disease is associated with what other immune disease
IgG deficiency and hypogammaglobulinemia
How would you screen for celiac disease?
Tissue transglutaminase IgA, antiendomysial antibodies (anti-gliadin not sensitive or specific)
gold standard for celiac disease diagnosis
upper endoscopy with biopsy
Dx for lactase deficiency
lactose breath hydrogen testing, lactase activity on duodenal biopsy or simple challenge/withdrawal
Pattern: Eskimo child, diarrhea, abdominal pain, bloating with pureed fruits or juices or with starch/cornstarch
sucrase-isomaltase deficiency
Dx of sucrose-isomaltase deficiency
acid hydrolysis of stool first, hydrogen breath testing, enzyme activity in biopsy during upper endoscopy
Rx for sucrose-isomaltase deficiency
Restriction of sucrose containing foods, enzyme replacement with yeast enzyme sacrosidase (Sucraid)
Inheritance of sucrose-isomaltase and glucose-galactose deficiency
AR
Pattern: diarrhea at birth, dehydration and metabolic acidosis increase and lactose containing formula fed infant
Glucose-galactose malabsorption
Rx for glucose-galactose malabsorption
fructose only carbs pt can eat
What % of CF pt has pancreatic insufficiency?
85-90% by one year
How do you monitor pancreatic insufficiency in CF patient?
Fecal elastase
Pattern: pancreatic insufficiency, bone marrow failure (cyclic neutropenia, defect in neutrophil chemotaxis, thrombocytopenia, anemia) metaphyseal dysostosis, FTT, short stature, oily, sticky foul smelling stools
Schwachman-Diamond Syndrome
Differences between Schwachman-Diamond and CF
nl sweat test, no CF mutation, metaphyseal lesions, fatty pancreas on imaging, infection more generalized and not just primarily pulmonary
Major cause of morbidity and mortality for Schwachman-Diamond patient
pyogenic infections
Crohn’s or UC?
Transmural, skip lesions
Crohn’s
Crohn’s or UC?
Bimodal age, first peak during adolescence
Crohn’s
Crohn’s or UC?
obstructive small bowel disease, RLQ pain, fibrostenotic
Crohn’s
Crohn’s or UC?
cramping, diarrhea, bleeding colonic diseae
Crohn’s
Crohn’s or UC?
inflammatory becomes structuring, perforating
Crohn’s
Crohn’s or UC? systemic signs, malaise, anorexia, growth failure/weight loss, pubertal delay
Crohn’s
Crohn’s or UC? perianal disease, skin tags, fistulae
Crohn’s
Crohn’s or UC? fistulae between loops of bowel (bowel to vagina or bladder or bowel to skin)
Crohn’s
Crohn’s or UC? erythema nodosum, arthritis, aphthous ulcers, clubbing, episcleritis/uveitis, renal stones and gallstones
Crohn’s
Crohn’s or UC? acute abdomen
Crohn’s
Crohn’s or UC? anemia, thrombocytosis, hypoalbuminemia
Crohn’s
Crohn’s or UC? Anti-saccharomyces
Crohn’s
Crohn’s or UC? fecal calprotectin and lactoferrin (neutrophil proteins shed in stool)
Crohn’s
Crohn’s or UC? non-caseating granulomas on upper endoscopy and colonscopy
Crohn’s
Crohn’s or UC? on upper GI/SBFT, thumbprinting, string sign, cobblestoning
Crohn’s
Rx for Crohn’s
corticosteroids, aminosalicylates, flagyl, immunomodulators, anti-TNF biologics infliximab
Crohn’s or UC?
Limited to mucosal layer and to colon sparing upper GI
UC
Crohn’s or UC? bloody, mucoid, purulent diarrhea, tenesmus, urgency, frequency, cramping, nocturnal stools
UC
Crohn’s or UC? pyoderma gangrenosum, PSC, chronic active hepatitis, anklyosing spondylitis (HLA B27)
UC
Crohn’s or UC? risk of colon CA, requiring surveillance colonoscopies every 1-2 years for dysplasia
UC
Crohn’s or UC? crypt abscesses, crypt branching on biopsy
UC
Crohn’s or UC? pseudopolyps, continuous erythema, loss of vascularity
UC
Crohn’s or UC? toxic megacolon, fevers, hypoalbuminemia
UC
Rx for UC
5-ASA mesalamine
Crohn’s or UC? pouchitis
UC
Crohn’s or UC? cured by surgical excision
UC
Pattern: weakness, rhabdomyolysis, neutrophil dysfunction, arrhythmia, seizure, AMS, sudden death in child with malnutrition just started feeds again
Hypophosphatemia
Pattern: arrhythmias, weakness, nausea and vomiting with refeeding
hypokalemia
Pattern: arrhythmias, tetany, nausea, vomiting
hypomagnesemia
What vitamin deficiency is a part of refeeding syndrome?
thiamine
What can happen with serum glucose in refeeding syndrome?
hyperglycemia
Daily fluid requirement for infants and children
100ml/first 10kg + 50ml/next 10kg + 20ml/remainder
How much water is in human milk or formula?
89%
How much caloric requirement for 20kg?
20kg – 1500kcal+20kcal/kg for each kg above 20kg
Additional calories required for: hypermetabolic state due to recent surgery
20%
Additional calories required for: multiple trauma
25%
Additional calories required for:Severe infection
50%
Additional calories required for: uncomplicated starvation
50%
Additional calories required for: third degree burns
100%
Predominant protein in breast milk
Whey
What three proteins in human milk protect child again local infection?
IgA, lactoferrin, lysozyme
How much protein is needed in infants
1) during first month
2) by 6th month
3) premature infants
1) 2 - 2.4g/kg/day
2) 1.5g/kg/day
3) 3.5g/kg/day
What are three major categories of lipids in Western diet?
triglycerides
phospholipids
sterols (cholesterol)
How many carbons on
1) SCTriglyceride
2) MCT
3) LCT
1) 12
Which are essential fatty acids?
Linoleic
Linolenic
Pattern: scaly dermatitis, hair loss, diarrhea, poor wound healing, growth failure
Essential fatty acid deficiency
How soon can deficiency in essential FAs occur in infants?
Within 2 weeks
Pattern: premature infant, scaly skin, diarrhea, poor wound healing, growth failure?
EFAD
Pattern: child with hepatobiliary or pancreatic disease with sacly skin, growth failture
EFAD
Pattern: Child on long term TPN without IV lipids, diarrhea, poor wound healing
EFAD
What is daily recommended % of calories of EF needed to prevent deficiency?
2-4%
How much Linoleic acid is in
1) Human milk
2) commercial formulas
3) cow’s milk
1) 3-7%
2) 10%
3) 1%
Recommendation for fat intake in children.
1) % total fat
2) % saturated
3) cholesterol
1)
What milk type has most whey?
Breast milk
What milk type has most casein?
Cow’s milk
What LCFAs are found in highest concentration in breast milk in 1st month?
DHA, ARA
What are DHA and ARA LCFA’s useful for?
Improving vision and cognition
What triglyceride is best for premature infants? Why?
MCT because they have decreased amount of intraluminal bile acids and decreased absorption of LCFA.
What vitamin deficiency is this?
CHF, tachycardia, peripheral edema
B1 wet
What vitamin deficiency is this?
Neuritis, peripheral paresthesia, irritability, anorexia
B1 dry
What vitamin deficiency is this?
Confusion, ataxia, opthlamoplegia
B1 Wenicke-Korsakoff
What vitamin deficiency is this?
Seborrheic dermatitis, angular stomatitis, photophobia, loss of visual acuity, burning an ditching of eyes, corneal vascularization, glossitis, poor growth
B2
What vitamin deficiency is this?
Preemie on prolonged phototherapy
B2
What vitamin deficiency is this?
Peripheral neuritis in child with suspected TB, seb derm, cheilosis, sideroblastic anemia
B6 - needed for iron utilization for hemoblogin synthesis
What vitamin deficiency is this?
Breast fed infants of vegan mothers, macrocytic anemia, hypersegmented neutrophils, ataxia, periphral neuropathy
B12
What vitamin deficiency is this?
Pt s/p distal small bowel resection
B12
What vitamin deficiency is this?
Petechiae, poor wound healing, easy fractures of bone, friable bleeding gums with loose teeth, microcytic anemia
C - scurvy
What vitamin deficiency is this?
Dermatitis of sun-exposed skin
B3
What vitamin deficiency is this?
macrocytic anemia/leukopenia, poor growth, imparied cellular immunity, glossitis, diarrhea, neural tube defects
B9 (folate)
Goat’s milk is deficient in what?
Folate
Pattern: dermatitis, anorexia, glossitis, muscle pain, insomnia, alopecia
biotin
What are 3 water-soluble vitamins that can cause toxicity?
Niacin, Vitamin B6, Vitamin C
Pattern: skin flushing, tingling, itching, dizziness, nausea, liver test abnormalities
Niacin toxicity
Pattern: sensory neuropathy, progressive ataxia, altered sense of touch and pain
B6 toxicity
Pattern: nausea, diarrhea, cramps, kidney stones (oxalate and cysteine nephrocalcinosis)
Vit C
3 types of patients at high risk for fat sol vitamin deficiency
chronic liver disease, pancreatic insufficiency, short bowel syndrome
Antibiotic therapy can cause deficiency of what vitamin?
vit K
Renal disease could cause deficiency of what vitamin?
vit D
Pattern: night blindness, retinal degeneration, xerophthalmia, photophobia, conjunctivitis, keratomalacia, follicular hyperkeratosis, poor growth, impaired resistance to infection
Vit A deficiency
Pattern: enlargement of costochondral junctions, chostochondral beading widening of epiphyses with thickening of the wrists and ankles, bowing, craniotabes
Vit D deficiency
Pattern: hemolytic anemia in premature infants, ataxia, hyporeflexia, decreased vibratory and position sensation, proximal muslce weakness, ophthlamoplegia/retinal dysfunction, neurocognitive changes
Vit E deficiency
Pattern: easy bruisability/soft-tissue hemorrhage, hemorrhagic disease, prolonged PT and PTT, defective bone and collagen production
Vit K deficiency
Pattern: dry, rough cracking skin and alopecia, carotenemia, pseudotumor cerebri, hepatosplenomegaly, bone pain in children
Vit A toxicity
Pattern: hypercalcemia, anorexia, vomiting, abdominal pain, constipation, convulsions
Vit D toxicity
Pattern: tired and weak, decreased work and school performance, slow cognitive and social development during childhood, difficulty maintaining body temperature, decreased immune function, glossitis
Iron deficiency
Pattern: Myositis, cardiomyopathy, macrocytic anemia, loss of skin and hair pigmentation
selenium deficiency
Pattern: rash, poor wound healing, impaired cellular immunity, anorexia, diarrhea, growth failure, low alkaline phosphatase
Zinc deficiency
Pattern: bullous and pustular lesion of oral, anal, genital areas and extremities, alopecia, diarrhea, opthalmologic problems (blepharitis, conjunctivitis, photophobia, corneal opacities)
Acrodermatitis enteropathica, AR disorder defect in zinc absorption
Pattern: microcytic anemia which does not respond to Fe supplementation, neutropenia, depigmentation of hair and skin, bone abnormalities
Cooper deficiency
Pattern: 2-3 mo male with loss of milestones/neurodegenerative disease, truncal hypotonia/seizures, FTT, abnormal, lightly pigmented, kinky hair, eyebrows, and eyelashes, connective-tissue and skeletal abnormalities
Menkes
What is the mutation in Menkes disease
X-linked (mutation of Xq13.3 gene) of impaired tranport of dietary Cu from the intestine
Major difference b/w Kwashiorkor and marasmus
Kwashiorkor is deficiency of protein. Marasmus inadequate caloric intake
Pattern: lethargy, apathy, irritability, anorexic, poor growth, loss of muscular tissue, immunodeficiency, edema, alopecia, scaly, erythematous dermatitis with darkening at sites of irritation
Kwashiorkor
Pattern: failure to gain weight, loss of fat and muscle, wasted, emaciated appearance, hypothermia, bradycardia, no hepatomegaly
marasmus
Difference in Kwashiorkor and Marasmus
Kwashiorkor - hepatomegaly, edema, anorexia; marasmus none of that
Which vitamins are typically deficient in Crohn’s patients?
iron, folate, vitB12, zinc, selenium, ADEK, ca, mg
Pattern: newborn with fulminant hepatitis
metabolic, tyrosinemia, galactosemia, fructosemia
Reducing substances in urine and fulminant hepatitis
galactosemia
liver failure after introduction of fruit juices
fructosemia
liver failure and succinylacetone in urine
tyrosinemia
Pattern: liver failure, jaundice, increase conjugated bilirubin, inc AST/ALT
Alpha-1 antitrypsin deficiency
Pattern: fetal hydrops, profound cholestasis and early death, iron deposition in liver
Neonatal iron storage disease, familial, increased iron and ferritin; due MRI of abdomen
3 causes of neonatal hepatitis
Tyro, galacto, fructosemia
Alpha-1 antitrypsin deficiency
Iron storage disease
Most common cause of neonatal hepatitis
Infection
TORCHES, adeno, echo, coxsackie
Pattern: liver problem at birth, SGA, sepsis, enlarged liver, spleen, rashes
hepatitis from infection
Why don’t you always see elevated conjugated bili in newborns?
Liver is still so immature
What lab work is typically associated with neonatal liver infection?
high WBC, low platelets
Dx galactosemia
reducing substance in urine
Dx fructosemia
liver failure after fruit juices
Dx tyrosinemia
urine succinylacetone
Pattern: increased conjugated bilirubin with elevated AST/ALT, jaundice resolves in first 6 months
alpha-1 antitrypsin deficiency
Dx alpha-1 antitrypsin
alpha-1 antitrypsin, genotype ZZ
Pattern: fetal hydrops, profound cholestatis, early death, iron deposition in liver and OTHER organs, present very sick
neonatal iron storage disease, increased iron, increased ferritin, MRI T2
Cause of neonatal iron storage
familial
Only hep virus that is DNA
Hep B
Transmission of HepA
poor hygiene, contaminated food and water
Pattern: child between 5 to 14 years, low grade fever, RUQ pain, jaundice, increased liver size, ANOREXIA
Hep A
If you suspect fulminant hepatitis, what tests should you order
PT/PTT, protime, serum ammonia, low serum glucose
Does Hep A cause chronic hepatitis?
No
What test do you order for Hep A?
antibody only, look at IgM
Which hep has the longest incubation?
HepB
Which hep can be spread horizonally
Hep B
Pattern: arthritis, rash, arthalgyria, liver function impairment
Hep B
Who can clear HepB virus - infected neonates or pt that acquire as adult?
Neonates will not clear
Consequences with HepB chronic.
Chronic active hepatitis, cirrhosis, hepatocellular carcinoma
Acute HepB serology
HB surface antigen, HBE antigen
What is the only protective HepB antibody
HB surface antibody
What is the serology for HepB patient who is clearing infection?
HB surface antigen not detectable, IgM HBcore high, no surface antibody yet - that is the window
Which Hep is orally transmitted?
A/E (vowels)
Which Hep is blood transmitted?
B/C (consonant)
Pattern: acute disease asymptomatic, chronic hepatitis
HepC
Which 2 hep can cause cancer?
B/C (consonants)
Is HepC viral antibody protective?
No
Dx for Hep C
PCR antigen
Rx Hep C
Interferon and ribavirin
Pattern: pt with hepB, eastern european, mediterranean
Hep D
Dx HepD
IgM to HepD
Pattern: teenager with HepE during pregnancy
fulminant liver failure
Pattern: child with liver AND spleen enlarged, adenopathy, jaundice and other hep signs
EBV virus
Which viruses tend to cause acute hep in immunocompromised children?
CMV, herpes, varicella
Drugs that can cause acute hepatitis
acetaminophen, INH, valproate, MTX, 6MP, halothane
What is characteristic lab work pattern in child with acetaminophen overdose?
Elevated AST/ALT, no hyperbili, increased PT/INR, increased ammonia
Rx for drug toxicity acute hepatitis?
N-acetyl cysteine
Mushroom - red cap with white spots
amanita phalloides
What combination of drug and infection can lead to Reye and Reye-like syndromes?
aspirin and varicella
What industrial toxins can cause acute hep
glue sniffing, polyvinyl alcohol, CCL4
Which Heps can cause chronic hep?
HepB/C
Rx of chronic hep?
Fluid and sodium restrction, treatment of encephalopathy, transplatnation
If you are suspicious of chronic hepatitis but bilirubin and/or AST/ALT are normal, what should you check?
PT, albumin
Pattern: HB surface antigen positive, no HB surface antibody
Chronic HepB, no clearance
Dose core antigen and antibody matter with HepC
Nope, it does nothing really
What are signs of active replicating HepB?
HBeAg +/- DNA PCR
Rx HepB chronic
Interferon and antiviral
Time course of organ involvement in alpha-1 antitrypsin
Liver in childhood
Lung disease in childhood or adulthood
Pattern: child with cirrhosis and variceal bleeding?
Alpha-1 antitrypsin deficiency
when in childhood do you see cirrhosis and portal HTN in CF?
mid-childhood
Rx hepatitis in CF
shunt procedure then transplant
What organs accumulate cooper in Wilson’s
Liver, CNS, kidney, eye
Dx Wilson
low serum copper, ceruloplasmin, high 24hr urine copper, liver copper on biopsy
Pattern: acute fulminant hepatitis and hemolytic anemia
Wilson’s disease, liver accumulates, liver damage and die, copper released from dying cells and RBCs lyse
Rx for Wilson’s
Penicillamine, trientene, transplantation
Fulminant liver disease with tyrosinemia; what is the worst sequelae?
Hepatocellular carcinoma
Rx tyrosinemia
NTBC - stops tyrosine degradation, can stop HCC risk
Pattern: chronic hepatitis signs, jaudice, mainly cholestasis and really bad pruritus
Progressive familial intrahepatic cholestatic disease
What is progressive familial intrahepatic holestatic disease
defect of bile acid metabolism/excretion
Pattern: pt with short gut syndrome getting parenteral feeds, elevated liver enzymes
parenteral nutrition induced
push enteral, and omega 3 intralipid
Pattern: child with arthryalgia, arthritis, IBD or DM, signs of chronic liver disease, serum protein highly elevated, but serum albumin is low
Hypergammaglobulinemia in autoimmune chronic hepatitis
Rx for autoimmune
steroids, 6MP, azathioprine
Pattern: Full-term infant with CONJUGATED hyperbilirubinemia w/in first 4 weeks of life
Biliary atresia
Prog of baby with biliary atresia who does not get biliary drainage?
Death within 1-2 years of life
All jaundice baby should get what test?
Total bili and direct to look for hyper conjugate bili for biliary atresia workup
When does Kasai procedure need to be done?
Pattern: biliary atresia baby with Kasai, but now having recurrent cholangitis, jaundice, portal HTN, cirrhosis
failing biliary drainage
Pattern: pulmonary stenosis, short stature, triangular facies, butterfly vertebrate, jaundice, xanthromas, pruritus
Alagille
Pruritus should make you think of what
Alagille (paucity of intrahepatic bile ducts) or familial intrahepatic cholestatic disease
Pattern: female, school-aged, abdominal mass, RUQ pain, intermittent jaundice and fever
Choledochal cyst
What is sclerosing cholangitis?
chronic inflammation causing focal areas of fibrotic narrowing and dilation of the intra and extrahepatic bile ducts
Liver problems associated with UC
Autoimmune chronic hepattitis and primary sclerosing cholangitis
Pt with primary sclerosing cholangitis, what can you tell parents who don’t want to treat?
end-stage liver disease and cholangiocarcinoma can develop
Pattern: pt with Langerhan’s cell histiocytosis and signs of liver problem
secondary sclerosing cholangitis
Sequelae of frequent cholelithiasis
cholangitis, intrahepatic abscesses, pancreatitis
pattern: hi alk phos and GGT and intermittent abdominal pain
cholithiasis
Rx of cholelithisis
ursodeoxycholic acid, removal
Pattern: female, colicky, RUQ pain referred to right scapula, fever, jaundice, increased bili and alk P
cholecystitis
What supervening bacterial infection can be seen with cholecystitis?
E.coli, enterococcus
Pattern: pt has no stones, salmonella/shigella prior
non-calculus cholecystitis
Pattern: epigastric pain with vomiting, increased amylase and lipase, enlarged pancreas on imaging of abdomen
pancreatitis
Why pancreatitis could lead to jaundice?
Swollen pancreas pushes on 2nd or 3rd part of duodenum and compresses segment that common bile duct and pancreatitic duct enters jointly as ampulla of vater
What is location of liver cancer that causes jaundice?
Porta hepatis
Which liver cancer is common in children
hepatoblastoma
Pattern: jaundice, abscess
amoebic abscess,
