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home - Liver - Miscellaneous - Alpha 1 Antitrypsin Deficiency Presentation Written by Dr Sebastian Zeki

Recognises the importance of sepsis as a complication
Aware of the differential diagnosis and management of sepsis and its
possible sequelae

Knows the appropriate use of the appropriate antibiotics and their
complications Aware of prevention of nosocomial infection

Understands the principles and practice of diagnosis and treatment of

Prepared to involve and liaise with specialist sepsis support



Knows the importance of clinical nutrition and its disturbances in
patients with acute and chronic liver disease

Appreciates indications for enteral or parenteral support and
understanding of limitations of these interventions

Shows ability to make careful nutritional assessment
Can liaise with nutritional support team where appropriate


Understands prognostic scoring systems including Child - Pugh
MELD UKELD Maddrey and disease-specific scoring systems where
they exist

Builds the use of accredited quantitative scoring systems into routine
clinical liver practice clinical colleagues and junior staff

Shows consistent application of evidence-based in the
evaluation of liver disease and the determination of prognosis

Alpha 1 Antitrypsin Deficiency Presentation

AAT AAT AAT AAT AAT AAT AAT AAT AAT AAT AAT AAT AAT Written by Dr Sebastian Zeki Alpha 1 anti-trypsin deficiency- Clinical Presentation Other manifestations:Vascular problems.Intrcranial haemorrhages/ fibr-muscular dysplasia.Inflammatory Bowel Disease. Risk Factors For Emphysema:Cigarette smoking (esp PiSZ).Dusty occupational exposure.A parental history of COPD.Personal history of Chronic bronchitis / Asthma/ Pneumonia. MZ causes no CLD unless have other liver conditions (eg ETOH) GlomerulonephritisMembranoproliferative type is rarely associated with PI*ZZ phenotype.IgA nephropathy occurs in one third of cirrhosis as kuppfer cells can't clear IgA. PiZZ Neonatal hepatitis with cholestasis up to 12 months 25% 11% 25% 25% 25% Liver disease in adults Beyond childhood, individuals with at-risk alleles may develop adult-onset cirrhosis or hepatocellular carcinoma.Adult onset cirrhosis can often occur without antecedent childhood liver disease. Liver disease is caused by pathologic polymerization of the variant AAT in the rER, resulting in intrahe-patocyte accumulation of AAT molecules, rather than a proteolytic mechanism (=Z-type molecules) .So no liver disease in PI*Null-Null individuals Hepatocytes that stain positively with periodic acid-Schiff (PAS) reagent but resist digestion by diastase. Mechanism unknown Need a second hit to explain why only some PiZZ get damaged livers- second hit is unknown -Development of cirrhosis between age 6m and 17 years, often causing death from complications of end-stage liver disease. -Histologic evidence of cirrhosis but with survival through the first decade with few sequelae. -Persistent elevation of LFTs without cirrhosis. PIZZ causes panacinar emphysema in about 60% Resolution of hepati-tis by ages 3 to 10 without hepatomeg-aly or splenomegaly.