Showing papers on "Sickle cell anemia published in 1975"
TL;DR: Results of a controlled trial with zinc supplementation in 14 to 19 year old sickle cell anemia subjects who were retarded in growth show that zinc supplementation significantly improved longitudinal growth and body weight.
Abstract: Clinical similarities between patients with sickle cell anemia and zinc-deficient subjects suggested a secondary zinc deficiency in sickle cell anemia. Zinc was assayed in various biological fluids and tissues by atomic absorption spectrophotometry. Zinc in the plasma, erythrocytes, and hair was decreased and urinary zinc excretion was increased in anemia patients as compared to controls. Erythrocyte zinc and daily urinary zinc excretion were inversely correlated in the anemia patients ( r = -.63, P < 0.05), suggesting that hyperzincuria may have caused zinc deficiency in these patients. Carbonic anhydrase, a zinc metalloenzyme, correlated significantly with erythrocyte zinc ( r = +0.94, P < 0.001). Plasma RNase activity was significantly greater in anemia subjects than in controls. We administered zinc sulfate, 660 mg per day, orally, to seven men and two women with sickle cell anemia. Two 17-year-old males gained 5 cm and 7 cm in height during 49 and 42 weeks of zinc therapy, respectively. All but one patient gained weight (0.5 kg to 4.1 kg). Five of the males showed increased growth of pubic, axillary, facial, and body hair, and in one a leg ulcer healed in six weeks on zinc and in two others some benefit of zinc therapy on healing of ulcers was noted.
156 citations
TL;DR: Significant additional data are needed to validate the preliminary hypothesis that transient impairment in gonadal function may, in part, account for the variation in sexual maturation seen in subjects with sickle cell anemia.
71 citations
TL;DR: The data are interpreted as suggesting that the elevated platelets and megathrombocytes in asymptomatic sickle cell patients result from lack of splenic sequestration.
54 citations
TL;DR: The present review examines some of the older as well as the more recent information on the morphology of the kidney in sickle cell disease, the functional changes which occur, aswell as the clinically significant syndromes with which the patient may present.
51 citations
TL;DR: It was concluded that in this community the incidence of G6PD deficiency in sickle cell anemia was not greater than would be expected by chance, and there was no evidence that the coexistence of the GdA- gene in SS patients ameliorated their disease.
49 citations
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47 citations
TL;DR: An increase in urate secretion permits most young adults with urate overproduction due to sickle cell anemia to remain normouricemic and may account for the low frequency of secondary gout in this disease.
38 citations
TL;DR: In Ibadan, Nigeria, 28 patients with sickle cell disease (hemoglobin SS and SC) died and were autopsied, and the type and frequency of intercurrent disease appears to be related to the environment.
Abstract: In Ibadan, Nigeria, 28 patients with sickle cell disease (hemoglobin SS and SC) died and were autopsied. Fifty percent of these had died by the age of 5 years, as did 21 (68%) with homozygous hemoglobin SS disease. No death occurred in infancy or childhood among the patients with hemoglobin SC disease. Serious intercurrent disease that could have been responsible for death was present in ten patients (36%). The type and frequency of intercurrent disease appears to be related to the environment. (JAMA233:889-890, 1975)
26 citations
TL;DR: The present data suggest that the serum alkaline phosphatase level may be an additional indicator of the degree, frequency, and persistence of tissue injuries that occur in sickle cell anemia.
Abstract: Alkaline phosphatase isoenzymes in sera from patients with sickle cell anemia were separated by electrophoresis on starch gel. Physical and biochemical criteria identified bone alkaline phosphatase as the principal, although not necessarily the sole, enzyme fraction that increases during symptomatic sickle cell crises. Moreover, there appeared to be concordance between crisis severity, serum levels of alkaline phosphatase, and isoenzyme patterns; electrophoretic and biochemical abnormalities could be detected even when the patients were asymptomatic. The present data suggest that the serum alkaline phosphatase level may be an additional indicator of the degree, frequency, and persistence of tissue injuries that occur in sickle cell anemia. ( JAMA 232:738-741, 1975)
22 citations
TL;DR: In this paper, a case of multiple myeloma in a patient with sickle cell anemia was described, where the increased viscosity of the patient's whole blood as compared with that of the control patient with SCL was mainly due to erythrocytic interaction with the circulating abnormal immunoglobulin.
TL;DR: It seems likely that properties of S-C red cells other than their relative ease of sickling contribute significantly to their rate of hemolysis.
TL;DR: Androgenic hormone therapy may be useful for selected adult patients with sickle cell disease when severe anemia contributes to disease morbidity and less marked changes followed lower oxymetholone doses.
TL;DR: Pulmonary angiographic findings in patients with sickle cell states might add information about the pathogenesis of this disorder since vascular occlusion appears to play a major role.
TL;DR: It is found that alkaline phosphatase scores are often lower than control values in patients with sickle-cell anemia and this difference is especially marked in such patients with leukocytosis.
Abstract: LEUKOCYTE alkaline phosphatase scores are often lower than control values in patients with sickle-cell anemia.1 , 2 This difference is especially marked in such patients with leukocytosis, in whom ...
TL;DR: Findings in four patients with hemophilia and nonobstructive renal enlargement are described, one factor common to all of these patients and patients with sickle cell anemia or thalassemia with large kidneys is multiple blood transfusions.
Abstract: Various renal abnormalities have been reported in patients with hemophilia. Findings in four patients with hemophilia and nonobstructive renal enlargement are described. Three of these patients also had pseudotumors of the iliac bone. The one factor common to all of these patients and patients with sickle cell anemia or thalassemia with large kidneys is multiple blood transfusions.
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Journal Article•
TL;DR: Special emphasis has been placed on electroencephalographic changes which occur during CNS complications in sickle cell anemia, and the severity of the observed EEG abnormalities which, at times, stood in contrast with a milder degree of clinical symptomatology.
Abstract: Introduction Neurological complications of sickle cell anemia (SS Hemoglobinopathy) are well known, and the first report by Sydenstricker et a l l . has been followed by a fair number of further observation^^-^. The CNS complications tend to occur episodically and are characterized mainly by obtundation, coma, epileptic seizures and, above all, stroke-like acute hemiplegic deficits. These neurological manifestations have been ascribed to small necrotic and necrobiotic lesions of diffuse distribution which preferentially are located at the border between cortex and underlying white matterlo. In addition to widespread small vessel stasis the major cerebral arteries may also become occluded9, perhaps as a result of vasa vasorum involvement and intimal proliferation. In this report, particular emphasis has been placed on electroencephalographic changes which occur during CNS complications in sickle cell anemia. A special motive for the present report w a s the severity of the observed EEG abnormalities which, at times, stood in contrast with a milder degree of clinical symptomatology.
TL;DR: Sickle cell disease affects approximately one in 400 to 500 blacks, and the carrier state, sickle cell trait, occurs in about 8% to 10% of the black population in the western hemisphere, and in up to 25% of black people of western Africa.
Abstract: Sickle cell disease affects approximately one in 400 to 500 blacks, and the carrier state, sickle cell trait, occurs in about 8% to 10% of the black population in the western hemisphere, and in up to 25% to 30% of black people of western Africa. Our knowledge of the molecular pathology, genetics, clinical manifestations, and natural history of sickle cell disease has expanded dramatically since J. B. Herrick's original observation in 1910 of "peculiar elongated and sickle shaped red blood corpuscles in a case of severe anemia." Until recently, a fashionable viewpoint suggested that sickle cell anemia has been a neglected disease and that we know little about its clinical manifestations or natural history. Yet, the classical breakthrough achieved by Linus Pauling and Vernon Ingram in unraveling the molecular structure of sickle cell hemoglobin led to a massive body of information on the structure and function of normal and other
Journal Article•
TL;DR: The NBT test did not differentiate well between sickle-cell anemia with bacterial infection and without bacterial infection.
Abstract: Forty-four patients who had sickle-cell anemia were examined to evaluate the reliability and usefulness of the nitroblue tetrazolium (NBT) test. The patients with sickle-cell anemia in painful crisis with bacterial infection often had low percentages of NBT reduction or negative results. The NBT test did not differentiate well between sickle-cell anemia with bacterial infection and without bacterial infection.
TL;DR: An 11-year-old black boy with sickle cell anemia developed profound pancytopenia during the course of his disease, but fully recovered therefrom.
Abstract: • An 11-year-old black boy with sickle cell anemia developed profound pancytopenia during the course of his disease, but fully recovered therefrom. The patient was receiving anticonvulsant drugs for a seizure disorder secondary to a "stroke," and, therefore, a drug-related marrow aplasia cannot be ruled out. ( Am J Dis Child 129:1195-1196, 1975)