Disease relevance of Hemoglobin SC Disease

• The concentrations of C-reactive protein and serum amyloid A were increased above 10 mg/L and 5 mg/L, respectively (our definition of an acute-phase response) in 18% (26/143) of subjects with SS disease even when they were symptom free, in 17% (6/35) of subjects with SC disease, and in 1% (1/80) of AA controls (p < 0.001) [1].
• This is a unique instance of fatal fat embolism secondary to bone marrow necrosis in a patient with sickle cell-hemoglobin C disease, systemic lupus erythematosus, and Sjögren's syndrome [2].
• To detect and quantitate temporal variations of the hemolytic rate in sickle cell disease, the authors measured endogenous carbon monoxide (CO) production in five normal subjects, nine patients with sickle cell anemia (SS) in steady clinical state, and two patients with sickle cell-hemoglobin C (SC) disease in and out of pain crises [3].
• 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) [4].
• Studies in adults with Hb SC disease suggested that hydroxyurea reduced hemolysis and increased red cell hydration [5].

High impact information on Hemoglobin SC Disease

• Functional asplenia in hemoglobin SC disease [6].
• Only biopsy Gleason score 7 (p = 0.0006) and PSA 10-15 ng/ml (p = 0.04) were significant predictors of SC disease [7].
• Cellular effects of hydroxyurea in Hb SC disease [8].
• Serum transferrin receptor (TfR) levels were measured in 182 children with homozygous sickle cell (SS) disease, 47 with sickle cell-haemoglobin C (SC) disease and 41 normal (AA) controls on their eighth birthday [9].
• Arterial blood lactate concentrations obtained on seven black males with hemoglobin sickle cell disease (SC) before, during, and after graded bicycle exercise up to exhaustion were compared with those of seven untrained (HU) and seven trained (HT) healthy males of the same ethnic origin [10].

Chemical compound and disease context of Hemoglobin SC Disease

• Nineteen percent of patients with hemoglobin SS disease had avascular necrosis of the femoral head (ANF) while a 9% incidence was found in hemoglobin SC disease [11].
• Hemolytic anemia associated with a radiopaque contrast agent in a patient with hemoglobin SC disease [12].
• Ceftriaxone-induced hemolytic anemia and hepatitis in an adolescent with hemoglobin SC disease [13].
• Many variables were assessed in our study before and after administration of L-carnitine to randomly selected 37 SC disease (SCD) children for a period of 6 months [14].

Biological context of Hemoglobin SC Disease

• There was no evidence that hydroxyurea was associated with increased urine concentrating ability in children with Hb SC disease [5].
• The association of hemoglobin SC disease and haptoglobin phenotypes [15].
• The age at menarche in girls with SS disease (mean (SD) 15.4 (1.3) years) was significantly later than girls with SC disease (13.7 (1.7) years) and those with AA haemoglobin (13.1 (1.3) years) but these genotype differences were no longer significant after controlling for the delay in the adolescent growth spurt [16].

Gene context of Hemoglobin SC Disease

• In a selected sample of patients with sickle cell-haemoglobin C (SC) disease, proliferative retinopathy (PSR) occurred in 90/243 (37%) patients, developed most frequently between the ages of 20 and 30 years, and affected 68% patients aged 45 years or over [17].
• Sudden sensorineural hearing loss in haemoglobin SC disease [18].
• The whole blood oxygen affinity of a Negro carrier of SC disease was found to be characterized by some right-shifted p50 and clearly increased Bohr effect, whereas the isolated and purified Hb-S and Hb-C exhibited slight deficiencies mainly of the Bohr effect [19].

Analytical, diagnostic and therapeutic context of Hemoglobin SC Disease

• To address these issues, we conducted a prospective, multicenter study of splenic function in persons with hemoglobin SC disease [6].
• Children with homozygous sickle cell (SS) disease and with sickle cell-haemoglobin C (SC) disease, aged 6 1/2 to 8 1/2 years, were examined by fluorescein angiography/angioscopy to determine the presence of retinal nonperfusion [20].
• We report here our experience with an adult patient with severe SC disease who developed symptomatic splenomegaly requiring splenectomy while being treated with hydroxyurea [21].

References