One line questions - curiosities Sunday, September 9, 2018 12:22 - acinar cell carcinoma of pancreas. Panniculitis, second most common, rx surgery poor outcome AIP - type 1, sausage shaped pancreas, not duct centric, path LPSP, relapses, systemic, igG4 elevated 65% time, stringy duct, (may have clinical scenario or parotid affected) Type 2. Duct centric, local, IgG4 normal, bx granulocyte epithelial lesion, associated with UC, low recurrence rate, rx steroids MCT- women, 50,s, size critical 3cm, calcification in wall, If communicates with duct IPMN. high cea in MCT Serous cystadenoma - central scar in 20% of cases Fertility Workup and pancreatitis, CYSTIC fibrosis, recessive and sweat chloride test PRSS or abn cationic trypsinogen gene, hereditary pancreatitis, AD (autosomal dominant). SPINK - tropical pancreatitis AR Schwachman Diamond syndrome SDS - second most common inherited pancreatic insufficiency, respiratory tract infection and bone marrow issues including AML. AR Bilirubin more than 4 and dilated ducts ERCP Skin lesion plus hypervascular mass - glucaganoma of pancreas - gallium scan, glucagon level, necrolytic migratory erythema is characteristic (but not specific) Pancreatic cancer screening in PJ syndrome, lynch, BRAC mutations with family history (one FDR or 2 any family), p16 with FDR Hypercontractile esophagus - DCI between 5000 to 8000 rx SSRI or TCA Diffuse esophageal spasm,- normal IRP, but low latency and high DCI and few normal peristalsis Gastric band - pseudoachalasia Pancreatico - biliary maljunction - do cholecystectomy. (pancreas takes off from distal cbd). Slightly higher cholangiocarcinoma also. Simvastatin protects against gallstones formation Bouveret syndrome - gb-duodenal fistula. Heals on its own. Endoscopic removal of stone AIDS choangiopathy - do ERCP, stent placement Hep C is RF for cholangioca Thailand - parasite Opsithorcosis and Chlonorsis, cholangio ca Milwaukee classification for SOD - type 1 ( typical pain, ALT x2x ULN and cb more than 8 - Rx ercp Type 2 - 2/3 do manometry and ercp if abnormal Type 3 functional S typhi chronic - rf for gb cancer Endoscopic ampullectomy can be done for ampullary polyps upto 4 cm Porcelain GB - incomplete calcification GB cancer risk. Complete calcification does not have gb cancer risk HIV choangiopathy is from HIV, cryptosporidium (commonest), microsporidium, cyclospora and MAI PSC strictures - FISH more than once with polysomy is concerning, not if just once GB polyps - commonest cholesterol - size less than 1 cm. Size more than 1 cm - adenomyomatosis nd maybe carcinogenic. Adenoma is last type and always carcinogenic. Carolis disease. Type 5 - just has hepatic -intra and extrahepatic - dot sign - dots in cbd. Carolis syndrome when extra hepatic manifestations and have hepatic fibrosis. Rxx of carolis is liver transplant or resection to prevent cancer Type 1 or type 4 Rx is reux hepaticojejunostomy high risk of cancer (extrahepatic cyts and other is both intra and extra hepatic cysts) Type 3 less risk of cancer, presents as pancreatitis Type 2 - is a diverticulum, rx is diverticulectomy Multiple bile duct strictures post OLT - hepatic artery compromised.)usually in first 3 months PBC - worse outcomes in younger age, males and fatigue Pediatric liver conditions Criggler Najjar 1 - high bilirubin, neonates Rx OLT fatal, and from UGT1A1 abnormality, unconjugated bilirubin Type 2 rx barbiturates, can be older rx is stimulation of UGTA1 activity Progressie familial intrahepatic cholestasis PFIC - 3 types. Type 3 is in adults, genetic ABCB4, duct proliferation on bx of liver and high GGT. Type 1 and 2 younger age and normal GGT. Type 1 is Bylers disease and homozygous for ATP1B8. BRIC is heterozygous for ATP1B8, milder and normal GGT. Common in AMISH Gallstones - 3 types cholesterol - commonest Black pigment - hemolysis, cirrhosis, ileal resection. Radioopaque Brown pigment - radiolucent, biliary strictures, chlonorchis infections Alagille syndrome - JAG 1 gene, characteristics skeletal, cardiac and facial features with jaundice and ductopenia Biliary cyst adenoma - rx is surgery, turns into cancer, ovarian tissue lining, calcifications in lining (similar to MCT?) Rx for biliary infections - chlonorchis - praziquental Strongyloides - Ivermectin Fasciola hepatica - triclabendazole Ascariasis - albendazole Cryptosporidium - nitazoxanide HCV - highest risk of cirrhosis and cancer type 3 IBD rx immuno suppression - HBV - HB sAg positve and HBc Ab + - highest risk. Moderate risk 1-10% reactivation - HBcA+ and HBsAg - - treat with tenofovir. Prednisone more than 20 mg for more than 4 weeks is also a high risk. AZT and methotrexate use is low risk less than 1 %. Once on treatment continue for 12 more months after the offending medication is not used In solid organ transplant Rx HEV IgG due to relapse. HEV is from game meat, pork and mussels, Rx is ribavarin Hep B with PAN (polyarteritis nodosa), - arthritis, myalgia, hypertension, rash and renal issues. Rx tenofovir if mild. If moderate steroids and severe cyclophosphamide. ANCA positive HCV - lichen planus, lymphoma, diabetes,cholangioca, PCT HBV with cirrohsis - Rx no matter what ALT or DNA level is. Risk of lactic acidosis is there HCC screening in HBV - every 6 months. Asian male more than 40, asian female more than 50 and african americans more than 20 age HSV hepatitis - ground glass appearance and history of steroid / immunosuppression use EBV infection and splenomegaly - avoid sports for 4 weeks. HIV and HBV together - treat both AFLP - LCAD def. Mother is heterozygous and fetus is homozygous. Microvesicular steatosisj (other causes are tetracycline, reyes syndrome, valproic acid). Reyes syndrome - MCAD medium chain fatty disorder precipitated with flu and salicylate Alcoholic hepatitis - worst prognosis with hepatic encephalopathy. Also they can get HRS without cirrhosis Best transplant survival is in PBC. AIH dosing - prednisone 60 mg or 30 mg with AZT 50 mg Africa american patient with elevated ACE level, high alk phos - sarcoidosis Tegretol decreasis the efficacy of OLT drugs (calcineurin). Causes acute cellular rejection High Hb is PV and which leads to BCS (budd chiari) Budd chiari treatment - anticoagulation, then thrombolysis, then TIPS and then OLT Acute onset hepatomegaly,ascites, edema and jaundice - can be SOS (sinusoidal obstruction syndrome or veno occlusive disease, post liver transplant, herbal medications. Similar to budd chair but obstruction is at sinusoids and venules and not in hepatic vein or IVC as in BCS). Azathioprine can do it and treatment for Bone marrow transplant, HH - target ferritin 50-100 and do liver biopsy if ferritin is more than 1500 (to rule out cirrhosis) Alcoholic hepatitis - good response to prednisone is drop in Bil at day 7 or lille score less than 0.45 CHF - cholestsis of liver High isolated AST - rhabdo PSC - can be large duct or small duct. Small duct needs liver biopsy Gilberts mild def of bilirubin UDP glucoronotransferase. Conjugated bilirubin abnormalities are dubin johnson and rotors syndrome CREST is higher in PBC (not any other autoimmune) PBC - fatigue not improved with treatment, osteoporosis is common, hypothyroidism, and high cholesterol (not TG). Rx for pruritis is questran, rifampicin and naltrexone (naltrexone can cause chronic pain like syndrome, rare hepatotoxicity). Uros does not help itching. AMA targets enzymes that catalyze oxidative decarboxylation of keto acid GVHD - close HLA match causes it. So liver is spared but develop immune reaction in skin, pancytopenia etc However AGVHD with bone marrow transplant affects the liver - attacking the bile duct and cholestatic picture AATD - PAS diastase resistant globules. Classical case emphysema also. However if A1AT is null / null, no liver damage since the abnormal protein is totally absent (so no abnormal A1AT polymerization in the liver cells)Be careful about checking A1AT levels after transfusion. False neg. Ground glass in liver cells is the hep B Glycogen storage disease presents as hypertension and can cause HCC. Rx is starch Only type 3 causes cirrhosis, others do not Gauchers disease - erlenmeyer flask deformity and glucocerebrosidease def leading to its deposition in the macrophages. NASH biggest mortality is from cardiovascular abnormality Non cirrhotic portal hypertension common in HIV treatment esp with didoinosine drugs, higher chance of variceal bleed. It is presinusoidal Large EGV - do not use beta blocker if pt is diabetic or has asthma TIPS mortality high if bilirubin more than 4, meld more than 15-18, advanced age, low sodium Hospital acquired SBP - use zosyn or extended spectrum carbapenem Increasing creatinine in cirrhosis - hold diuretics, volume expansion with albumin. If Cr does not improve or Cr rises more than 1.5, then type 1 HRS. Then consider octreotide and midodrine. HE - sodium benzoate can be considered. Clubbing and hypoxemia in cirrhosis - hepatopulmonary syndrome from shunting, do contrast echo. Portopulmonary HTN has vascular resistance more than 240, Pa pressures more than 25 and wedge less than 15. Occurs from splanchnic vasoconstrictors bypassing the liver, loud P2 and no hypoxia Post OLT - hepatic artery thrombosis is in 7 days, 2-12% of cases and if with high lactate, AST more than 3000, need another liver immediately High cancers post transplant include skin, respiratory and lymphoma Ascites - fluid restriction if sodium low. Restrict salt, new ascites occurs about 7% per year. A1AT does not recur after OLT Best regimen is reduced dose tacrolimus with