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Results for “Hemoglobin SC Disease”

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At least 19 recordsLinked to original sources
PubMed1978

Myocardial necrosis following general anesthesia in hemoglobin SC disease.

A 4-year-old girl with hemoglobin SC disease died following general anesthesia. Autopsy showed widespread intravascular sickling; staining with hematoxylin-basic fuchsin-picric acid demonstrated newly developed massive myocardial necrosis, a rarely documented finding. Anesthesia may produce conditions that provoke "crises" in patients with sickle hemoglobinopathies. Preoperative identification of sickle states and careful attention to hydration and oxygenation may minimize anesthetic risks in these patients.

Anemia, Sickle Cell↗
PubMed1978

Partial exchange transfusion as treatment for hemoglobin SC disease in pregnancy.

Serious infarctions and embolic events can complicate the course of pregnant patients with hemoglobin SC disease. In two cases, partial exchange transfusion preceded recovery in severely ill pregnant women with hemoglobin SC disease. There seem to be pathophysiological correlations for the observed clinical findings, and there are potential beneficial effects of partial exchange transfusion. Based on our experience, partial exchange transfusion should be considered as a means of reversing the often fatal complications attending hemoglobin SC disease and pregnancy. The exchange should be of sufficient volume to ensure a postexchange level of hemoglobin A of at least 30%.

Adult↗
PubMed1979

Posterior pole neovascularization in a patient with hemoglobin SC disease.

A 33-year-old black woman with hemoglobin SC disease and a history of photocoagulation for peripherally located retinal neovascularization had a neovascular frond at the temporal border of her right macula. Multiple zones of black sunburst hyperpigmentation were located nearby. Fluorescein angiography showed that the lesions were closely related to an extensive zone of avascular retina in the posterior pole. The perifoveal vasculature was uninvolved, and the patient was unaware of any visual deficit. Although sickle cell maculopathy is a well-recognized entity, there have been no published reports, to the best of our knowledge, of posterior pole neovascularization in patients with sickle cell hemoglobinopathy. The fluorescein angiographic findings in our case provide further support for the hypothesis that retinal hypoxia is an important stimulus for retinal neovascularization.

Adult↗
PubMed1978

Optic disk neovascularization in hemoglobin SC disease.

A 37-year-old black man had a clinically documented case of optic disk neovascularization associated with hemoglobin SC disease. Through medical evaluation, we ruled out other disorders associated with proliferative retinopathy. Extensive areas of retinal nonperfusion were associated with vasoproliferative retinopathy at the junction of the perfused and nonperfused retina; and a delicate network of neovascularization extended from the optic disk into the vitreous gel in one eye.

Adult↗
PubMed2026

Is There a Difference in Occurrence of Complications Between Adults With Hemoglobin SS and Hemoglobin SC Disease: An Extended Systematic Review.

Sickle cell disease (SCD) is characterized by both acute and chronic complications. The clinical manifestation of these complications differs between genotypes. Given the large amount of research already published, this systematic review aims to offer a complete overview of types of sickle cell complications between adults in the most common genotypes Hemoglobin SS (HbSS) and Hemoglobin SC (HbSC), putting options for further research into perspective. An extensive literature search was performed to study all available evidence on these complications. This review was performed according to the "Preferred Reporting Items for Systematic Reviews and Meta-Analyses" (PRISMA) statement guidelines, and was performed on January 2, 2024. A total of 710 references were identified. After careful screening, 521 records were excluded based on title and abstract and other exclusion criteria. In total, 158 articles were excluded after full-text assessment. Our analysis of 31 studies highlights key differences in complications between HbSS and HbSC genotypes in sickle cell disease (SCD). Vaso-occlusive crises (VOCs) remain the most common acute complication in both genotypes. HbSS patients experience more frequent VOCs, while HbSC patients generally have a milder clinical course when it comes to acute complications. Chronic complications, particularly in the ocular and pulmonary systems, are more prevalent in HbSC patients. However, as acute complications are more common in HbSS and chronic complications more common in HbSC, both genotypes face progressive organ damage due to recurrent ischemic injury and inflammation.

Humans↗
PubMed1979

Severe mycoplasma pneumonia with pleural effusions in a patient with sickle cell-hemoglobin C(SC) disease. Case report and review of the literature.

We describe a patient with sickle cell hemoglobin C(SC) disease in whom severe pneumonia developed complicated by large bilateral pleural effusions and respiratory failure. Mycoplasma infection was not initially suspected but was subsequently proved serologically. The course of the illness was unusually long. A review of the literature showed occurrence of large pleural effusions to be infrequent for pulmonary infection with Mycoplasma in adults, with only eight such cases previously reported. The possibility of Mycoplasma pneumonia should not be dismissed merely because of the severity of the illness or the presence of pleural effusions.

Adolescent↗
PubMed1979

Disseminated intravascular coagulation in sickle cell crisis.

We conclude that DIC can occur as a result of sickle cell crisis in the absence of sepsis and we recommend that patients with sickle cell disease, particularly those with hemoglobin SC disease, presenting in crisis should be considered at risk for the development of disseminated intravascular coagulation. With symptomatic treatment and improvement of the crisis, our patient's coagulopathy resolved.

Adult↗
PubMed1978

Relationship of density distribution and pyruvate kinase electrophoretic pattern of erythrocytes in sickle cell diseases and other disorders.

The density distributions of red cells from sickle cell disorders and other hematological diseases were determined. In sickle cell anemia there was an increase in the proportion of cells in both the heaviest and lightest fractions. In hemoglobin SC disease, a small fraction was shifted to heavier cells. Sickle cell trait blood had a normal density pattern. In hereditary spherocytosis an increase in the number of heavy cells was observed. Thin-layer polyacrylamide gel electrophoresis of red cell pyruvate kinase demonstrated that the pyruvate electrophoretic pattern was related to density distribution.

Anemia↗
PubMed1977

Hemoglobin SC, SS/GPhiladelphia and SOArab diseases diagnostic importance of an integrative analysis of clinical, hematologic and electrophoretic findings.

Hemoglobin studies are presented on two children in whom electrophoretic analysis of hemolysates at alkaline pH initially suggested the diagnosis of hemoglobin SC disease. In both patients the course of the disease was unusually severe, blood smears contained irreversibly sickled cells, and crystal formation did not occur when red cells were incubated in 3 per cent sodium chloride solution. Each of these findings is inconsistent with the interpretation of the electrophoretic patterns. Family studies demonstrated that one of the patients had sickle cell anemia and was also a carrier of an alpha chain variant; citrate agar electrophoresis at pH 6.2 of the second child's hemolysate revealed that the "C-like" hemoglobin was not hemoglobin C. Tryptic peptide analyses of the nonhemoglobin S fractions showed that the first child has SS/GPhiladelphia disease and that the second child has SOArab disease. These observations emphasize the importance of integrating the clinical and hematologic data with the results of electrophoretic procedures in order to define precisely the hemoglobin abnormality in patients with sickling disorders.

Anemia, Sickle Cell↗
PubMed1977

Evolution of neovascularization in sickle cell retinopathy. A prospective fluorescein angiographic study.

With prospective observations we have documented by fluorescein angiography the development of retinal neovascularization in the eyes of patients with sickle cell-hemoglobin C disease (hemoglobin SC). In each patient, arteriolarvenular (AV) anastomoses occurred before the development of neovascular sea fans. To our knowledge, this report constitutes the first such documentation in the literature. New sea fans from AV anastomoses appeared throughout an average interval of 18 months (range, 8 to 36 months) in approximately 21% of young adults with hemoglobin SC. Each year approximately 14% of young patients with hemoglobin SC may develop retinal neovascularization.

Adolescent↗
PubMed1976

The iduronidase-deficient mucopolysaccharidoses: clinical and roentgenorgraphic features.

Hurler and Scheie syndromes, two of the six clinically distinct mucopolysaccharidoses, are deficient in the same lysosomal enzyme, alpha-L-iduronidase. A third group of iduronidase-deficient patients can now be identified during the pediatric years using clinical and radiographic criteria. Based on inferential evidence for allelism between the Hurler and Scheie genes, the occurrence of genetic compounds which simultaneously carry both mutant alleles may be predicted to occur. This can be considered analogous to the structural gene mutations leading to hemoglobin SC disease. Four patients with phenotypes intermediate between Hurler and Scheie syndromes are flet to represent genetic compounds of this type. Both clinical and roentgenographic features are helpful in distinguishing these patients from those with Hurler syndrome or Scheie syndrome. Fibroblast correction characteristics identical to those of Hurler syndrome and Scheie syndrome and absence of consanguinity are additional features which favor classification as genetic compounds. The possibility of a third mutant allele at the Hurler-Scheie locus or of extreme phenotype variation are not considered likely alternative explantations. Depending on the frequency of the Scheie syndrome and the Hurler syndrome, genetic compounds may occur with an intermediate frequency or may be more common than either homozygous condition.

Adolescent↗
PubMed1979

The diagnosis and treatment of secondary glaucoma after hyphema in sickle cell patients.

Four patients with sickle cell hemoglobinopathies (one sickle cell hemoglobin C disease (SC); three sickle cell trait (AS)) and hyphemas had a higher percentage of erythrocytes sickled in their anterior chambers than in their circulating venous blood. Intraocular pressure (IOP) was severely increased, despite relatively small amounts of intracameral blood. Systemic hypotensive agents were not always successful in reducing IOP, and in patients with sickle cell hemoglobinopathy, are probably contraindicated in high or repeated dose regimens. Moderate increase of IOP in sickle cell hemoglobinopathy patients may produced rapid deterioration of visual function, because of a greater than usual effect on vascular perfusion in the central retinal artery and optic nerve. Early anterior chamber paracentesis may be the best treatment for this type of hyphema-induced secondary glaucoma.

Acetazolamide↗
PubMed1977

Hemoglobinopathies in pregnancy.

Sickle cell trait is associated with an increased incidence of asymptomatic bacteriuria, low birth weight infants and premature rupture of membranes. Hemoglobin SS and hemoglobin SC in pregnancy result in significantly increased maternal morbidity and mortality and reproductive wastage. Heterozygous thalassemias in pregnancy carry increased risks but to a lesser degree than hemoglobins SS and SC. Partial exchange transfusion can provide effective prophylaxis for many of the maternal and fetal problems of sickle cell disease.

Anemia, Sickle Cell↗
PubMed1977

Secondary gout in hemoglobinopathies: report of two cases and review of the literature.

Although patients with hemolytic hemoglobinopathies characteristically are over-producers of urate, and hyperuricemia is frequently recognized, clinical gout has rarely been reported in such patients. Our evaluation of 2 premenopausal women with gout led to the diagnosis of previously unrecognized hemoglobinopathies (SC disease and CC disease). Investigation of these 2 patients and review of the reported cases of gout in patients with hemoglobin S or C disorders suggest that relatively minor abnormalities of renal function in these patients may lead to early development of significant hyperuricemia. With increasing lifespan of patients with hemolytic hemoglobinopathies and the likelihood of increased occurrence of renal function abnormalities, it is anticipated that gout will more frequently be responsible for joint symptoms in such patients.

Adolescent↗