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Presenting complaints
8 months
4 month
8 month ago developed fever vomiting &abdominal pain, admitted to hospital,diagnosed as viral hepatitis Symptoms gradually improved But continue to feel fatigue, malaise Feel tired on exertion Had never became jaundice, developed on/off episodes of itching
History cont..
Abdominal pain & discomfort epigastric pain with dyspeptic symptoms, aggravate with meal , no particular radiation or other aggravating /relieving factors Progressive darkening of skin for the last 4-5 months he has noted increased pigmentation of skin , generalized, not in skin creases or scars No H/O jaundice, skin rashes , arthritis or fever
History cont..
No recurrent diarrhea or bleeding PR or altered bowel habits No dry mouth, eye or sicca syndrome No H/O wheezing attacks, haemoptysis No history of, orthopnoea, exertional chest pain or dyspnoea No legs or body swelling
No DM , inflammatory bowel disease, Rheumatoid arthritis , Movement disorders or psychiatric illness Not on drugs, Aurvedic Rx No exposure to Organo-phosphates or organic compounds Had T. Toxoid 1 1/2 yrs ago No H/O blood transfusion
No cirrhosis , Psychiatric illness , Movement disorders or haematological disease No sexual promiscuity Unmarried security officer, 6000/= Non alcoholic, non smoker
General examination
Not pale, not icteric, not emaciated generalized pigmentation, not in skin creases or scars No chronic liver stigmata's or scratch marks No tendon or palmar xanthomas Has considerable amount of acne No finger clubbing, lymhpadinpathy, oral or genital ulcers
System examination
ABDOMAN 1cm smooth , non tender hepatomegaly. No splenomegaly or free fluid or mass CVS BP- 130/80mmHg, Clinically normal All other systems clinically normal
summery
34 yrs old male, presented with 8month H/O malaise, fatigue,epigastric pain & dyspeptic symptoms,progressive darkening of skin with preceding H/O hepatitis Not pale or icteric, no evidence of liver stigmata's 1cm hepatomegaly,no splenomegaly, free fluid or mass Other systems clinically normal
Hepatic Alcohol abuse Chronic Heb B/ C----HepBsAg/ C Ab NAFLD( NASH ) Autoimmunne hepatitis-young ,female----- S. Electroph: --^ polyclonal emmunoglobulin-- 80% Haemochromatosis-- TS >45% Wilsons -- ( <40yrs ) -S. ceruloplasmin -- 85% Alpha1 antitrypsin deficiency -S. electrophoresis-- marked reduction alpha globulin -- Phenotype ZZ
Cont..
NON HEPATIC Celiac sprue-- Anti endomyosial Ab, Antigliadin Muscle disorders -- CPK / Aldolase Strenuous Exercise
Inv.
SGOT-- >60 U/L -------------- 82 SGPT ---> 60 U/L --------------- 170 S. bilirubin 10umol/l ---------- <17 um/l Alk Phos 132 U/L ^-------------123U/L Gama GT -----------------------141 U/L ^ ( 11 - 50 ) PTT 13sec/ 12 sec ESR ----- 47mm/hr S. Protien T-8.5mg/dl Al- 4.4 HepB sAg / Hep C -- neg USS Abdomen mild hepatomegaly with normal echo pattern Rheumatoid factor ----------- Neg
Inv cont..
S. Fe 116umol/l ( N ) TIBC 320umol/l ( N ) S. Ferritin 322ng/ml ( N ) ANF ----------------------- neg Anti-smooth muscles Ab -- neg S electrophoresis --- normal S. Ceruloplasmin 25ng dl ( N ) KF rings -- neg Lipids-- TG 189 ( 157 ) Liver Bx periportal fibrosis with septa formation & mononeuclear cell infiltration. Extensive fatty changers & focal necrosis of parenchymal cells Bile duct proliferation not seen
Increasingly recognized condition may progresses to end stage liver disease Diabetic hepatitis, NASH, Fatty liver hepatitis Steatosis==> Steatohepatitis ==> Cirrhosis 10 -24 % general population The most common cause of abnormal LFT in adult Cryptogenic cirrhosis share many clinical, demographical features of NAFLD
Risk Factors
Obesity ( truncal ) 10 - 100% DM type 2 10 - 75 % Hyperlidemia ( TG ) 20 -92 % Family H/O NAFLD or cryptigenic cirrhosis Middle age women , some says male
Pathogenesis
Poorly understood, hypothetical Net retention of lipids in hepatocytes prerequisite for NAFLD synthesis *Degradation Secretion Insulin resistance is the most reproducible factor Enzyme deficiency of beta oxidation & Acytyl-coenzyme A important cause of microvesicular steatosisi
Clinical Presentation
Mostly asymptomatic at diagnosis Fatigue, malaise, fullness of abdomen Symptoms & signs of advance liver disease Only physical finding may hepatomegaly, acanthosis nigricance
Diagnosis
Suspect== increased AST/ALT, Radiological fatty liver & persistent hepatomegaly Should exclude alcohol abuse, secondary causes Histologicaly--- Liver Bx
steatosis,monocytes, neutrophils, ballooning necrosis, Mallorys bodies portal tracts relatively spared in some cirrhotic, steatosis & necroinflammation may be not seen
Laboratory investigation
AST to ALT -- <1 Alk Phosp & gama GT -- may elevated but less than alcoholic hepatitis Increased PTT, Bilirubin, reduced Alb -advance liver disease S. Ferritin increased in 50 % Increased Transferin saturation in 11% Hepatic Fe index -- normal
Investigation Cont..
USS increased diffused echogenasity similar to cirrhosis sensitivity - 89% specificity - 93% CT low density hepatic paranchyma fatty sparing focal fatty infiltration -- mimic focal mass
Natural history
Not well defined 28% progressive liver damage in 311yrs 58 % no change 13 % improves or completely resolved
Management
Good control of DM , Hyperlipaedemia Reduction of Wt Drugs No place small pilot studies-- gemfibrozil, Vit E, Metformin, Ursodiol improves LFT Liver transplant--- in end stage liver disease, but may recur
Bad prognosis
Age-- > 45 yrs Obese DM- type 2 AST/ ALT ratio > 1 -- indicate advance fibrosis Increased TG Liver Bx == Steotohepatitis or advance fibrosis