General Abdo Flashcards
PRIMARY SCLEROSING CHOLANGITIS is what
clinical features
Progressive cholestasis with bile duct inflammation and strictures.
Clinical features: jaundice, steatorrhoea, pruritus, fatigue weight loss, malabsorption of fat Ca.
Associations: ♂sex, HLA-A1, AIH, IBD, especially ULCERATIVE COLITIS. Bile duct, gallbladder, liver and colon cancers are more common → do yearly colonoscopy + ultrasound; consider cholecystectomy for gallbladder polyps.
test results for PSC
Tests: ↑Alk phos, then ↑bilirubin; hypergammaglobulinaemia;
AMA–ve, but ANA, SMA, and pANCA may be +ve;
ERCP distinguishes large duct from small duct disease. Liver biopsy shows a fibrous, obliterative cholangitis. Screen for cholangiocarcinoma (CA19-9)
PSC typical epidemiology/associations
ssociations: ♂sex, HLA-A1, AIH, IBD, especially ULCERATIVE COLITIS.
Bile duct, gallbladder, liver and colon cancers are more common → do yearly colonoscopy + ultrasound; consider cholecystectomy for gallbladder polyps.
Ulcerative colitis is associated with what
PSC
PRIMARY BILIARY CIRRHOSIS (PBC) is what + feautres
Interlobular bile ducts are damaged by chronic granulomatous inflammation (biliary epithelial cells lining the small intrahepatic bile ducts.) causing progressive cholestasis, cirrhosis, portal HTN
Often asymptomatic
Lethargy and pruritus may occur, and can precede jaundice by months to years.
Signs: Jaundice; skin pigmentation; xanthelasma; xanthomata; hepatomegaly; and splenomegaly
PBC complications
Osteoporosis is common.
■Malabsorption of fat-soluble vitamins due to cholestasis and ↓ bilirubin in the gut lumen results in osteomalacia and coagulopathy
■portal hypertension; ascites; variceal haemorrhage; hepatic encephalopathy;
■HCC.
PBC associations, cause
■Thyroid disease ■Rheumatoid arthritis ■Sjögren’s syndrome ■Keratoconjunctivitis sicca ■Systemic sclerosis ■Renal tubular acidosis ■Membranous glomerulo-nephritis
?autoimmune response triggered by environmental factors, with genetic predisposition. ♀:♂≈9:1. Prevalence: ≤4/100,000. Peak presentation: ~50yrs old.
is PBC more common in men or women?
women (PBC)
is PSC more commmon in men or women?
men (PSC)
tests in PBC
●AMA M2 subtype (Antimitochondrial antibodies) are hallmark of PBC.
●Immunoglobulins are ↑ (esp. IgM).
●TSH & cholesterol ↑ or ↔. ●↑Alk phos, ↑γgt, and mildly ↑ast & alt; late disease: ↑bilirubin, ↓albumin, ↑prothrombin time.
what could have a picture of AMA M2 and high Ig (esp IgM
PBC
is PSC AMA positive?
No
AUTOIMMUNE HEPATITIS (AIH) is what and clinical features
An inflammatory liver disease of unknown cause: ■suppressor t-cell defects with autoAb against hepatocyte surface antigens ■Three types AIH
■predominantly young & middle-aged women.
■25% present with acute hepatitis and signs of autoimmune disease, eg fever, malaise, urticarial rash, polyarthritis, pleurisy, pulmonary infiltration, or glomerulonephritis.
■remainder present insidiously or are asymptomatic and diagnosed incidentally with signs of chronic liver disease.
■Amenorrhoea is common and disease tends to attenuate during pregnancy.
AIH tests show
Abnormal LFT (ast, alt, serum bilirubin and alk phos are ↑ in most), hypergammaglobulinaemia (especially IgG), \+ve autoantibodies (ANA, SMA, or LKM1).
■Other autoantibodies, eg anti-soluble liver antigen (sla) and antimeasles virus may be seen.
■Anaemia, WCC↓, and platelets↓ indicate hypersplenism.
how can definitive diagnosis of AIH be made?
Liver biopsy required for Dx: mononuclear infiltrate of portal and periportal areas & piecemeal necrosis, fibrosis, or cirrhosis.
What causes air in the biliary system?
& Previous surgery or endoscopy procedures (most common)
& Penetrating ulcers
& Erosion of gallstone into the bowel lumen
& Traumatic fistula
& Neoplasms
& Bowel obstruction
caecal tumour - what type of resection
right hemicolectomy
FELTY’S SYNDROME
splenomegaly and neutropenia in a patient with rheumatoid arthritis (RA).
Case: abdo pain, vomiting and jaundice. On examination she has tender hepatosplenomegaly and ascites. She has a historyof recurrent miscarriages
what can it be?
BUDD–CHIARI SYNDROME is a condition characterized by obstruction to hepatic venous outflow. It usually occurs in a patient with a hypercoagulative state (e.g. antiphospholipid syndrome, use of oral contraceptive pill, malignancy) but can also occur due to physical obstruction (e.g. tumour). The venous congestion can lead to enlargement of the spleen as well as the liver. The history of recurrent miscarriages suggests that there may be an underlying disorder (e.g. antiphospholipid syndrome) and this should be investigated thoroughly.
ASCENDING CHOLANGITIS signs and cause
secondary to bacterial contamination of an obstructed biliary system. The classical description of fever with rigors, right upper quadrant pain and jaundice is known eponymously as Charcot’s triad
biliary colic characteristic and how does it differ from acute cholecystitis>
BILIARY COLIC Is a constant right upper quadrant pain (unlike renal colic) as it results from the spasm of gallbladder muscle against a stone lodged in Hartmann’s pouch (at neck of gallbladder) or the cystic duct. Acute cholecystitis presents with biliary colic symptoms. The key difference is that in ACUTE CHOLECYSTITIS there is development of inflammation due to the mechanical obstruction that can result in superimposed bacterial infection. These patients will therefore show signs of inflammation/infection (pyrexia, tachycardia, increased white cell count).
MUCOCOELE
dilatation and enlargement of the gallbladder without infection, secondary to prolonged obstruction of the cystic duct. There is progressive distension over a period of time due to the accumulation of mucus secreted by the epithelial cells. The condition is usually asymptomatic. On clinical examination a visible, non-tender mass is palpable in the right upper quadrant
markers for pancreatic carcinoma
CA19-9
ZOllinger-Ellison syndrome typical presentation, patho and associations, investigation of choice
patient with Hx of multiple ulcers resistant to anti-ulcer Tx
gastrin secreting tumour usually in the pancreas).
Associated with MEN1 – ie also a risk of parathyroid hyperplasia.
Ix: nuclear medicine (somatostatin receptor scintigraphy is the most sensitive)
METAPLASIA definition
transformation of one fully differentiated cell type into another fully differentiated cell type
ANAPLASTIC CARCINOMA of the THYROID description, associations
aggressive tumour which usually affects the more elderly patients. A rapidly invasive thyroid tumour. It may arise from a pre-existing papillary/follicular carcinoma and is also closely associated with endemic goitre. Treatment involves surgical debulking of tumour and is usually a palliative procedure due to the aggressiveness of the tumour.
ANAPLASTIC CARCINOMA of thyroid is associated with PHAEOCHROMOCYTOMA.
