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PubMed1975

[Dyserythropoiesis and dyserythropoietic anemias].

The expression "congenital dyserythropoietic anemia" (CDA) has been used to characterize the kinetic and morphological aberrations in the proliferation and maturation compartment of erythropoiesis, occurring in a group of hereditary anemias of unknown pathogenesis. The main symptoms of these disorders are moderate or mild anemia, increased hemoglobin turnover, ineffectiveness of erythropoiesis, striking morphological aberrations of the erythroblasts and tendency to secondary hemochromatosis. To date, three types have emerged from this group that may be hereditary nosological entities. They are distinguished not only on a morphological basis, but also by different modes of inheritance and immunological properties of the red cell membrane. A number of additional cases or families have been described which could not be attributed to one of these three types. Comparative investigations of morphological, biochemical and immunological details in CDA on the one hand, and other forms of ineffective erythropoiesis (e.g. thalassemia, refractory anemia) on the other demonstrate the lack of specificity of many of the single changes observed in CDA. These changes may well be secondary phenomenon of intramedullary cell destruction brought about by different underlying pathogenetic mechanisms.

Anemia, Aplastic↗
PubMed1975

Isolation and properties of poly(A)-containing RNA from the cytoplasm of rat liver cells.

1. The described technique for preparative isolation of poly(A)-containing RNA by complexing with poly(U)-cellulose and elution at 45 degrees C, is simple, reproducible, and the poly(U)-cellulose preparation is suitable for repeated use. 2. The poly(A)-containing RNA isolated from the poly(U)-cellulose complex is still contaminated by ribosomal RNA which can be removed by recomplexing. The purified preparation is heterogeneous on polyacrylamide-gel electrophoresis and is located over the region from 5-6S to 28S. Poly(A) segments of cytoplasmic RNA show heterogeneous size distribution. 3. In acidic medium, the poly(A) segments form ordered double-stranded structure. 4. The rapid labelling of rat liver and fibroblast cytoplasmic poly(A)-containing RNAs and their base composition resemble closely the corresponding properties of mRNA.

Adenine Nucleotides↗
PubMed1987

Diagnosis of urea cycle disorders.

Hyperammonemia in pediatrics can be due to a number of causes (defects of urea cycle enzymes or transport of its metabolites, organic acidurias, acyl-CoA dehydrogenase or carnitine deficiency, liver bypass or nonspecific insufficiency) requiring differentiated rapid treatment for a satisfactory prognosis. The specific diagnosis cannot be established by clinical means. Thus the work-up rests on biochemical analyses. The methods used are detailed and their interpretation discussed. An algorithm for the interpretation of the data which can easily be computerized is presented. The procedure has proven practicable in 126 patients with urea cycle disorders.

Algorithms↗