<prompt> 1 month old newborn presents with conjugated Hyperbilirubinemia, intrahepatic cholestasis and high alkaline phosphatase. Eosinophilic, PAS positive cytoplasmic granules are seen in liver biopsy specimen. Diagnosis is?Alpha 1 AT deficiencyCong hepatic fibrosisExtrahepatic biliary atresiaDuctal plate defect  <response>: a. Alphal AT deficiencyRef: Nelsons 20e p 871-879, Ghai 8e p 172-176The given clinical features investigation findings are suggestive of Alphal Antitrypsin deficiency.Alphal AT deficiencyIt usually presents as neonatal cholestasis or later-onset childhood cirrhosisal -Antitrypsin, a protease inhibitor synthesized by liver, protects lung alveoli from destruction by neutrophil elastaseNormal phenotype is PiMM; In affected patients with PiZZ, polymers of, AT are retained in the ER of hepatocytesIn liver biopsy, periodic acid-Schiff PAS-positive diastase-resistant globules are seen, primarily in periportal hepatocytes 