alpha 1-antitrypsin deficiency/phosphatase

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Long-term follow-up of a cohort of children with alpha-1-antitrypsin deficiency.

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We assessed lung function, liver function, and smoking attitudes and behavior in 22 adolescents with homozygous alpha 1-antitrypsin deficiency whose condition had been detected through neonatal screening in the early 1970s. All subjects had normal lung volumes, expiratory flow rates, and diffusing

Liver function in patients with pulmonary emphysema due to severe alpha-1-antitrypsin deficiency (Pi ZZ).

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Alpha 1-Antitrypsin deficiency predisposes to pulmonary emphysema, liver cirrhosis and hepatocellular carcinoma. Anecdotal evidence and a large autopsy study suggest that severe lung and liver disease rarely coexist in the same subject, but this has not been studied in patients. Therefore we

Prochlorperazine-induced cholestasis in a patient with alpha-1 antitrypsin deficiency.

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A case of a 58-year-old woman with history of bilateral lung transplant secondary to alpha-1 antitrypsin deficiency (PIZZ), who presented with a severe drug-induced cholestasis secondary to prochlorperazine is reported. After 27 months of prochlorperazine use, she developed liver failure consisting

Serum liver enzymes in Turner syndrome.

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Increased serum concentrations of liver enzymes are sometimes observed, in the absence of clinical symptoms of liver disease, in patients with Turner syndrome. The purpose of this study was to evaluate, in our Turner patients, serum liver enzyme levels and to find a cause for their increase. In 70

Acute fatty liver of pregnancy in a patient with chronic active hepatitis and associated hepatocyte alpha 1-antitrypsin inclusions.

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BACKGROUND Acute fatty liver of pregnancy is a rare, potentially fatal condition that shares many of the signs and symptoms of severe preeclampsia. Early reports of this condition noted alarmingly high fetal and maternal mortality rates. However, recent reports have described more favorable outcomes

Antineutrophil antibody: a test for autoimmune primary sclerosing cholangitis in childhood?

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The detection of an antineutrophil antibody which is highly sensitive and specific for adult primary sclerosing cholangitis using indirect immunoalkaline phosphatase has been previously described. In this study, the diagnostic potential of this method in childhood primary sclerosing cholangitis is

Frequency of urinary tract infection in pediatric liver transplantation candidates.

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An increased frequency of infections has been reported in patients with chronic liver disease. The tendency of patients in this population to acquire UTI is not completely understood. We aimed at investigating the incidence of UTI in children with cirrhosis, before liver transplantation. Twenty-six

Altered alpha-mannosidase isoenzymes in the liver in hepatic cirrhosis.

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Homogenates of liver from cases of hepatic cirrhosis due to alpha 1-antitrypsin deficiency (PiZZ) alcoholism were analyzed for their content of various lysosomal enzymes. Also determined were the specific activities of lactate dehydrogenase, glutamate-oxaloacetate transaminase, glutamate-pyruvate

A Polarizing Case of Elevated Liver Enzymes.

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A 25-year-old male was referred to hepatology clinic for abnormal liver enzymes: AST 242 IU/L, ALT 507 IU/L, alkaline phosphatase 139 IU/L, INR 1.1, bilirubin 1.3 mg/dl, and albumin 4.2 g/dL. His hemoglobin was 13.3 g/dl and platelet count 130 x10⁹ /L. The patient reported heavy alcohol

ACG Clinical Guideline: Evaluation of Abnormal Liver Chemistries.

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Clinicians are required to assess abnormal liver chemistries on a daily basis. The most common liver chemistries ordered are serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase and bilirubin. These tests should be termed liver chemistries or liver tests.

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