A novel two‐nucleotide deletion in the ornithine transcarbamylase gene causing fatal hyperammonia in early pregnancy

U Schimanski, D Krieger, M Horn, W Stremmel, B Wermuth, L Theilmann – 1 December 1996 – Ornithine transcarbamylase (OTC) deficiency shows X‐linked inheritance. Typically, symptomatic females (who constitute 15%‐20% of all carriers) have markedly reduced enzyme activity and show first symptoms in late infancy or early childhood. Here we present the case of a previously asymptomatic 24‐year‐old woman who died of severe hyperammonemia associated with orotic aciduria but normal OTC activity in the fourth month of pregnancy.

Autoreactive CD4+ LKM‐specific and anticlonotypic T‐cell responses in LKM‐1 antibody‐positive autoimmune hepatitis

H F Lohr, J F Schlaak, A W Lohse, W O Bocher, M Arenz, G Gerken, K M Buschenfelde – 1 December 1996 – Peripheral blood mononuclear cells (PBMC) of patients with autoimmune hepatitis (AIH) and controls were studied for their proliferative response to six overlapping synthetic peptides covering the 33‐amino acid immunodominant region of cytochrome P450IID6, the main target antigen of LKM‐1 antibody‐positive type II AIH.

Serum hyaluronate reflects hepatic fibrogenesis in alcoholic liver disease and is useful as a marker of fibrosis

A Pares, R Deulofeu, A Gimenez, L Caballeria, M Bruguera, J Caballeria, A M Ballesta, J Rodes – 1 December 1996 – The high levels of hyaluronic acid (HA), a glycosaminoglycan of the liver extracellular matrix, which is synthesized and degraded in the liver sinusoidal cells, have been related with a decreased function of the endothelial sinusoidal cells.

A Hitchhiker's guide to antisense and nonantisense biochemical pathways

A D Branch – 1 December 1996 – Antisense pharmaceutical research has sought to provide drugs that would yield effective therapies for diseases resulting from the production of deleterious proteins. The original concept was straightforward: eliminate production of unwanted proteins, such as oncogenic proteins, by blocking the function of their mRNAs; and block their mRNAs by adding “antisense” nucleic acids that bind them through complementary base pairing. However, it has proven difficult to develop clinically useful antisense strategies.

Humoral and cellular immune responses to hepatitis B vaccination in hepatitis B surface antigen‐carrier children who cleared serum‐ hepatitis B surface antigen

H Hsu, M Chang, R Hsieh, Y Ni, W Chi – 1 December 1996 – The immune responses to hepatitis B vaccine were studied in 11 hepatitis B surface antigen (HBsAg) carrier children who had cleared HBsAg but failed to develop hepatitis B surface antigen antibodies (anti‐HBs) in sera (group 1), 5 HBsAg carrier children who had cleared HBsAg and developed detectable anti‐HBs in sera (group 2), and 5 healthy subjects seronegative for all hepatitis B virus (HBV) markers (group 3). After receiving three doses of HB vaccine, group 1 subjects failed to develop detectable anti‐HBs.

Stimulation of bile acid independent bile flow with bromo‐cyclic guanosine monophosphate

M V St‐Pierre, J Dufour, I M Arias – 1 December 1996 – The second messenger, cyclic guanosine monophosphate (cGMP), mediates the actions of nitric oxide, natriuretic peptides, and microbial toxins on cellular contractility and electrolyte movement. Because both hepatocellular contractility and electrolyte secretion participate in bile formation, we investigated the actions of cGMP on this process in intact liver. In rat liver perfused with 8‐bromo‐cyclic GMP (bcGMP) at 0.5 and 3 micromol/min, bile flow increased by 5% and 31%, respectively.

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