The unstable Hb Hammersmith or alpha 2 beta 2(42)(CD1)Phe----Ser observed in an Indian child; identification by HPLC and by sequence analysis of amplified DNA.

We have identified the unstable hemoglobin variant present in a Chipewayan Indian patient with severe hemolytic anemia as Hb Hammersmith or alpha 2 beta 2(42)(CD1)Phe----Ser. Her parents were normal. Identification was greatly facilitated by the use of reversed phase high performance liquid chromatography for the isolation of the beta X chain and its tryptic fragments, and of sequence analysis of amplified DNA which readily identified a TTT(Phe)----TCT(Ser) mutation at codon 42.

Hereditary anomaly of neutrophil granulation in Birman cats.

A hereditary anomaly of neutrophil granulation in purebred Birman cats was described with respect to genetic, electron microscopic, histochemical, and functional characters. The trait was inherited in an autosomal recessive manner and was prevalent in the population studied. Affected cats had fine eosinophilic granules in the cytoplasm of neutrophils. The granules had normal morphology as determined by electron microscopy and did not stain for acid mucopolysaccharide. Bactericidal activity, phagocytosis, and oxidative function of affected neutrophils were not different from those of unaffected neutrophils. The anomaly was concluded to be an alteration in the content of lysosomal granules with increased affinity for acidic dyes.

Early acute rejection in renal homotransplantation. Angiographic-pathologic correlation.

Early acute rejection occurs between two and ten days after renal homotransplantation and has been recognized in eight of 156 renal transplants. Four of the eight patients developed spontaneous renal rupture unrelated to biopsies. Pathologically, there was acute passive congestion with areas of tubular necrosis, venous thrombi and perivenous lymphoplasia. Glomerular and arterial immunogenic injury was slight. Radiologically, the kidneys were enlarged with stretching of the interlobar and segmental arteries, decreased peripheral filling and a homogeneous nephrogram. Renal vein filling defects were a frequent feature.

Counting cells in cerebrospinal fluid collected directly on membrane filters.

Accurate enumeration of cells in the cerebrospinal fluid is not feasible with the current method of counting cells in the Fuchs Rosenthal or Neubauer chamber when the count is near normal since the volume of fluid examined is too small. A sample of adequate volume to permit such accurate assessment may be collected from the lumbar puncture needle into a syringe through a ruled membrane filter in a Swinney cartridge. A study of 291 samples shows that the upper limit of the normal cerebrospinal fluid count is 2000 cells/ml (2/cmm) and not 5000/ml (5/cmm) as is currently accepted.

The electrophoretic pattern of hemoglobin in newborn babies, and abnormalities of hemoglobin F synthesis in adults.

On routine electrophoretic analyses on filter paper and starch gel in an alkaline or neutral medium, no abnormal hemoglobin fractions were found in the blood of 600 newborn infants or their mothers. Trace amounts of hemoglobin Barts were noted in many of the blood samples from newborns when the starch gels (phosphate buffer pH 7.0) were stained with a benzidine/H(2)O(2) reagent. In one infant, precocious cessation of synthesis of hemoglobin F was postulated to account for the small amounts of this hemoglobin found in a cord-blood specimen. Analysis of 15,000 blood samples from adults revealed two instances in which the hemoglobin F level was 20 and 35%, respectively. The former was attributed to a hereditary persistence of hemoglobin F, while the latter was associated with acute leukemia.In an addendum, the finding of an infant with an abnormal hemoglobin variant, resembling in many of its properties hemoglobin F Texas, is reported.