Fatal cholestatic hepatitis and multisystem failure associated with nitrofurantoin.
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Adolescent↗
Biomedical subjects
A E Mulberg
Publications and source records attributed to A E Mulberg.
Modulation of coated vesicle chloride channel activity and acidification by reversible protein kinase A-dependent phosphorylation.
We have previously shown that activity of a Cl- channel is required for acidification of clathrin-coated vesicles by the coated vesicle (H+)-ATPase (Arai, H., Pink, S. and Forgac, M. (1989) Biochemistry 28, 3075-3082). We demonstrate that activity of the coated vesicle Cl- channel is modulated by phosphorylation. Cl- conductance was measured in a reconstituted preparation of coated vesicle membrane proteins using the Cl(-)-sensitive fluorescence probe, 6-methoxy-N-(3-sulfopropyl)quinolinium. Treatment of coated vesicle membranes with alkaline phosphatase resulted in a 25 +/- 5% decrease in Cl- channel activity. A parallel decrease in ATP-dependent acidification of coated vesicles was also observed. The decrease in Cl- conductance and ATP-dependent acidification was reversed by treatment with protein kinase A and MgATP; the alkaline phosphatase inhibitor, sodium orthovanadate, blocked the inhibition of acidification. These results indicate that Cl- conductance in coated vesicles is modulated by a protein kinase A-dependent phosphorylation and that this modulation in turn affects ATP-dependent acidification.
Adenosine Triphosphate↗
Autoimmune liver disease and sickle cell anemia in children: a report of three cases.
PURPOSE: We wish to alert clinicians to the possible association between Sickle cell anemia (SCA) and autoimmune liver disease (AIL). METHODS: AIL was diagnosed by serologic, histologic and/or cholangiographic studies in patients with known SCA. RESULTS: Three schoolaged children with SCA were investigated for increased levels of transaminases and/or worsening jaundice. All were diagnosed to have AIL. Two patients had autoimmune hepatitis and have responded to immunosuppressive therapy, and I had primary sclerosing cholangitis and is stable with therapy. CONCLUSIONS: Autoimmune hepatitis and sclerosing cholangitis are rare liver disorders of childhood, but may occur with greater frequency in patients with SCA. Early diagnosis and treatment is essential to prevent progression to liver cirrhosis. This association may provide a clue to the etiology of AIL and warrants further investigation.
Adolescent↗