GI 3 Flashcards
Criteria for autoimmune hepatitis
- increased transaminases - usually 500-1000
- hypergammaglobulinemia
- positive NA Aand anti Sm antibodies (70%), antiliver-kidney antibodies in some
- liver biopsy - hepatic lobules and portal areas
Treatment of autoimmune hepatitis
1st line steroids and azathioprine
2nd line: cyclosporine A, tacrolimus, mycophenolate mofetil
transplant for patients who progress to end stage
rate of remission 75% in 1-3 months, lots will relapse , can get recurrence in transplanted liver
Wilson disease findings
LVR: asymptomatic hepatomegaly, subacute or chronic hepatitis, fulminant hepatic failure
Neuropsych: behavioural changes, deterioration in school or job performance, abnormal speech, tremors/dystonia
Rando: Kayser-Fleischer rings, hemolytic anemia, Fanconi syndrome
Diagnosis of wilson disease
low ceruloplasmin
elevated serum and/or urine copper
increased ALP/AST but can be low with fulminant disease
Treatment of wilson disease
chelation therapy - penicillamine restrict copper intake zinc to impair copper absorption liver transplant if severe without treatment it is fatal , fulminant ALF have poor prognosis
Reye syndrome - which infections classically associated
influenza or varicella
often 1 week after
often associated with aspirin use
can progress to really bad neuro symptoms
labs: LFTs, NH3, prolonged PT, and/or hypoglycemia
Treatment of nonalcoholic steatohepatitis
fatty infiltration of the liver in children with obesity and type II diabetes
usually asymptomatic, picked up on screening labs for obese patients
AST/ALT usually < 1, may or may not have hepatomegaly
diagnosis by liver US or biopsy
treatment by diet and exercise
History of acute hepatic crisis precipitated by intercurrent illness, what diagnosis do you think about?
tyrosinemia (hereditary)
autosomal recessive
results from deficient activity of fumarylacetoacetate hydrolase, causes progressive dysfunction of LVR/KDN/Peripherap nervous system
labs: increased serum tyrosine, mehionine, elevated serum AFP
increased risk of hepatocellular carcinoma
Treatment of tyrosinemia
dietary modifications, tyrosine degradation inhibitor, LVR transplanted if needed
alpha 1 antitrypsin - how does it present in children?
neonatal cholestasis
childhood/adolescence- cirrhosis
diagnosis is by genetic testing
Drugs which can cause cholestasis
chlorpromazine
erythromycin
estrogens
(table 12.14 in Oski) has other drugs too
Drug which can cause biliary sludge
ceftriaxone
Drugs that cause liver disease
acetaminophen, valproic acid, isoniazid, sulfonamids, phenytoin, methotrexate
OCP, steroids (can cause cancer)
Causes of portal hypertension
extrahepatic obstruction - ie portal vein thrombosis
intrahepatic obstruction: hepatocellular disease, cirrhosis, Budd-Chiari syndrome, veno-occlusive disease, Alagill syndrome
idiopathic
definition: elevated by 10-12 mmHg of portal pressure
Treatment of portal hypertension
treat underlying disease
meds to decrease portal pressure - beta blocker, vasopressin
shunt/liver transplant
treat complications - esophageal varices
Most common cause of SBP
#1 E coli #2 Klebsiella, treat with IV cefotaxime while awaiting disease ID causes of ascites in children: LVR, heart filaure, protein losses, malnutrition, infectious, chylous, VP shunt dysfunction, hypo T and malignancy
Definition of fulminant hepatic failure
- biochemical evidence of acute liver injury
- no evidence of chronic liver disease
- hepatic based coagulopathy
(PT >15 seconds, INR >1.5 not corrected by vitamin K in the presence of encephalopathy OR APT >20 seconds or INR >2 regardless of presence of clinical encephalopathy)
**need all 3 criteria
cause :50% idiopathic, rest are ingetsions, infections, other causes of injury
management of patient in fulminant hepatic failure
neuro: avoid sedatives, limit protein, lactulose, ICP monitoring
Resp: intubation may be needed
GI: antacids, glucose control
renal: avoid hypovolemia
heme: vitamin K, FFP, plasmapheresis, platelets, liver transplantation
mortality 70% without transplant
most common cause of cholestasis in 0-3 month term babies
biliary atresia
thought to be acquired disorder
HIDA scan - no excretion of tracer from liver into intestines , liver biopsy is definitive diagnosis
initial treatment: Kasai procedure, 80% ened liver transplant
Alagille syndrome - 6 features
- intrahepatic bile duct paucity
- facial features - triangular shaped
- ocular (posterior embryotoxon)
- cardiac (pulmonary valve stenosis, peripherl pulmonic stenosis)
- vertebral arch - butterfly vertebrae
- renal anomalies
(don’t need all of them)
Inheritance of Alagille syndrome
autosomal dominant
15-50% are spontaneous mutations
severity of liver disease is variable
treatment mainly supportive
liver disease in born marrow transplant, mechanism?
GVHD, veno-occlusive disease
liver disease in sickle cell mechanism
gallstones, hemosiderosis (chronic transfusions)
Are cholodochal cysts more common in females or males?
4x more common in females
usually present in first 6 years of life
type 1 is the most common
Long term risk of cholodochal cyst
increased risk of cholangiocarcinoma
Conditions associated with gall bladder hydrous
- hemoglobinopathies
- Kawasaki
- fasting
- systemic infections
Medical conditions associated with gallstones
rare in children, usually associated with risk factor
- hemolytic disease
- hypercholesterolemia
- obesity
- CF
- biliary tract malformations
patients with distal obstruction from gallstones can have pancreatitis at the same time
A patient with IBD presents with abdo pain, fever, jaundice, pruritus and hepatomegaly. ERCP shows a beaded appearance of biliary tree. What is the diagnosis
primary sclerosing cholangitis
direct hyperbili, transaminitis, increased serum Ig, + ANA, anti smooth muscle Ab
treatment: liver transplant not curable, poor prognosis due to increased risk of cholangiocarcinoma (50% 10-12 year survival)
<10% of IBD patients, rare in childhood
What is Charcot’s triad and Reynol’d pentad?
signs for ascending cholangitis (which is treated with NPO, Abx, stone removal/dilation via ERCP vs PTC
Charcots triad: fever, jaundice, RUQ pain
Reynod’s pentad: fever, jaundice, RUZ pain, hypotension, altered mental state
Which of the following symptoms has the highest risk of colorectal cancer?
a) familial adenomatous polyposis
b) Peutz-Jeghers
c) juvenile polyposis
a) FAP - 100% risk of colon cancer
poly - adenomals in large intestines (vs others which are large and small)
onset 2nd decade
rectal bleeding, abdo pain, bowel obstruction
**All the syndrome listed in Oski are autosomal dominant
Which of the following is not a feature of Peutz Jegher syndrome
a) adenoma
b) intussusception
c) mucosal pigmentation of lips
d) rectal bleeding
a) NOT adenoma, it’s hamartomas of small and large intestines
the others are features
risk of colorectal cancer is 40%
Name 7 conditions which increase the risk of lymphoma
- AIDS
- ataxia telangiectasia
- Wiscott-Aldrich syndrome
- agammaglobulinemia
- SCID
- bone marrow/solid organ transplant
- long standing celiac disease
most common GI malignancy in children
consider lymphoma in child >2 year old with intussusception
What type of tumour is often found incidentally during an appendectomy?
carcinoid tumour
What is carcinoid syndrome?
caused by carcinoid tumours outside the appendix which metastasize to the liver
diarrhea, vasomotor disturbances, bronchoconstriction, and/or right heart failure
dx: elevated urine serotonin metabolite - 5-hydroxyindoleacetic acid (5HIAA)
Please name the GI manifestations of CF
intestines - mec ileus, DIOS, GERD
pancreas - exocrine deficiency (malabsorption of fats, proteins, and lesser extent carbs; diabetes
Nutrition/Metabolism - vitamin and mineral deficiencies, edema and hypoproteinemia, salt loss
Hepatobiliary: focal biliary cirrhosis (pathognomonic for CF, can be as early as 3day, astymptomatic to hepatic failure), primary sclerosing cholangitis, portal hypertension
gallbladder - galstones - 1/3 of adult patients
Treatment of SMA syndrome
resolves when feeds are given in a way which bypasses the duodenum (i.e. NJ feeds) -in SMA syndrome the SMA loses it fat pad and compresses onto the duodenum
prone/lateral positioning
prokinetics
surgery in refractory cases
can confirm dx: with upper GI will demonstrate abrupt duodenal cutoff to the right of the midline
consequences of untreated meckel diverticulum
diverticulitis
intussusception
volvulus
difference between acrodermatitis enteropathica and dermatitis herpetiformis
dermatitis herpetiformis - in celiac disease
acrodermatitis enteropathica - AR form and also form related to zinc deficiency
perianal fistula in a 1 year old?
relatively common in heath children of that age
usually heals on its own
if severe symptoms then might need fistulotomy and other treatment for kids > 2 years, more likely to have predisposing condition (immunocompromise, Crohn’s, prior rectal surgery), may need antibiotics, surgery
3 possible consequences of choledochal cyst
cholangiocarcinoma
malrotation
cirrhosis
when can you start refeeding a patient with pancreatitis
when clinical symptoms better and amylase is decreasing
lipase elevated for 8-14 days so don’t use as a marker to start feeds
clear diet then advance
True or false - Kayser -Fleischer rings are only caused by wilson disease
nope, other causes of cirrhosis with kayser Fleischer rings are:
1. autoimmune hepatitis
2. viarl hepatitis
3. alpha1 antitrypsin
4. drug toxicity
but most commonly associated with wilson disease
What are the findings of peritoneal fluid analysis in patients with exudative process causing peritonitis
increased total protein increased ascites:plasma protein ratio elevated ascites plasma LDH low serum:ascites albumin WBC > 500 with >50% pmns