Evaluation of SICKLEQUIK, a differential solubility test for hemoglobin S.

Researchers evaluated a differential tube solubility test's ability to detect Hemoglobin S (Hb S) in sickle cell conditions. Six hundred twenty-nine blood samples, including 190 S hemoglobinopathies, were used to assess test performance. Alkaline cellulose acetate electrophoresis was used to confirm all results. The test correctly indicated the presence of Hb S in every case in which Hb S was found on electrophoresis. Six of the 198 specimens containing Hb S incorrectly differentiated the heterozygous from the homozygous state (two with Hb AS, three with Hb SF, and one with Hb SC). Electrophoresis later showed four of these samples to be inappropriate for solubility testing. Three of these specimens contained Hb SF and the other specimen contained Hb SC. One specimen from a patient with polycythemia gave a false positive result. Results were compared using different methods of centrifugation. This solubility test was found to be dependable as a screening method provided the manufacturer's directions are followed and all positive results are confirmed by electrophoresis.

[Familial hemophagocytic reticulosis (Farquhar) (author's transl)]. / Familiäre hämophagozytierende Retikulose (Farquhar).

An account is given of six cases of familial hemophagocytic reticulosis (familial lymphohistiocytosis). After a period of illness varying from 2 weeks to 7.5 months the infants studied died with pancytopenia, fever and hepatosplenomegaly. Histologic examination revealed three characteristic features: Lymphocyte infiltration of the organs, reduction of the lymphatic and hematopoetic system, histiocytosis with erythrophagocytosis. The etiology of this disease remains obscure. Congenital allogeneity may be a possibility, but further immunologic investigations would be necessary to support this hypothesis.

"Fast isoamylases" in the parotid saliva of children with cystic fibrosis and heterozygous carriers.

On electrophoresis, parotid saliva always exhibits a basic pattern of 6 isoamylases. Additional faster migrating isoamylases occur in varying numbers. These "fast isoamylases" are generated, at least in part, by deamidation. Compared with juvenile and adult controls, a significantly greater number of "fast isoamylases" was found in the parotid saliva of children with cystic fibrosis and their healthy heterozygous parents. A shift in the equilibrium between amidation and deamidation is discussed in terms of its possible connection with the metabolic defect responsible for cystic fibrosis.

[Fast isoamylases in saliva from mucoviscidosis patients (author's transl)]. / Uber das Auftreten schneller Isoamylasen im Mukoviszidosespeichel

Human parotid saliva is characterized by a basic pattern of 6 isoamylases; during ageing, it contains additional isoamylases which migrate faster towards the anode. An increasing number of 'fast isoamylases' are also found in saliva from mucoviscidosis patients. Their possible symptomatological significance for the pathogenesis of mucoviscidosis which is as yet not solved is being discussed.

'Fast isoamylases' in parotid saliva of heterozygous carriers of cystic fibrosis.

An increased number of 'fast isoamylases' is found in the parotid saliva of children with cystic fibrosis and their heterozygous parents who are clinically healthy. 'Fast isoamylases' are generated, at least in part, from the 6 isoamylases of the basic pattern by deamidation of asparagine and glutamine residues. This basic pattern is present in the parotid saliva of all subjects we have tested so far. A shift in the equilibrium between amidation and deamidation processes is discussed in terms of its possible significance for the pathogenesis of cystic fibrosis and for ageing.