![]() ![]() Osteonecrosis was found in patients as young as 5 years old. Patients with the hemoglobin SS genotype and alpha-thalassemia and those with frequent painful crises were at highest risk. (1991) found an overall prevalence of osteonecrosis of the femoral head of about 10%. ![]() In a large study involving 2,590 patients over 5 years of age at entry and followed for an average of 5.6 years, Milner et al. He had had cholecystectomy for asymptomatic cholelithiasis at the age of about 47. Steinberg (1989) described a 73-year-old black man in Mississippi who had hematologically and genotypically typical sickle cell anemia with, however, very mild clinical manifestations. They found a correlation between urine concentrating ability and the percentage of sickle hemoglobin, which was highest in the individuals with normal alpha-globin genotype and lowest in those homozygous for the deletion. (1991) studied urine concentrating ability in 3 classes of A/S individuals, those with a normal alpha-globin genotype and those who were either heterozygous or homozygous for the gene-deletion type of alpha-thalassemia. Reasoning that the severity of the concentration defect might be related to the percentage of sickle hemoglobin present in erythrocytes, Gupta et al. ![]() The defect in urine concentrating ability in persons with sickle cell trait is thought to result from intracellular polymerization of Hb S in erythrocytes, leading to microvascular occlusion, in the vasa recta of the renal medulla. However, MRI series demonstrated 11 to 24% of cerebral vascular accidents in patients with sickle cell disease, indicating a large proportion of silent infarctions. Previous studies had shown clinically evident cerebral vascular disease in 7 to 8% of cohorts followed during the first 2 weeks of life. (1991) concluded that despite current rates of mortality and morbidity with bone marrow transplantation, a substantial minority of parents of children with sickle cell disease would consent to bone marrow transplantation for their children.Īdams (1995) reviewed the literature on sickle cell disease and stroke. The studies showed a downward age-related trend in hemoglobin and platelets and falling reticulocyte count consistent with progressive bone marrow failure which could not be explained by renal impairment. (1991) reported hematologic findings in 181 patients, aged 40 to 73 years, with hemoglobin SS disease. The routine use of prophylactic penicillin therapy in infants and children with SC disease remained controversial. (1994) concluded that pneumococcal vaccine should be administered in all children with SC disease. Autopsy findings in 5 cases included splenic congestion without infarction in 5, splenomegaly in 4, and bilateral adrenal hemorrhage in 3. Erythrocyte pit counts in 2 patients were 40.3 and 41.7%, respectively (normal, less than 3.6%). All 7 children had an acute febrile illness and rapid deterioration despite parenterally administered antibiotic therapy and intensive medical support. Only 1 child had received pneumococcal vaccine or prophylactic penicillin therapy. ![]() The earliest death occurred in a 1-year-old child who had cyanotic congenital heart the other children were aged 3.5 to 15 years. (1994) reported 7 fatal cases of pneumococcal septicemia in children with SC disease. Less is known about splenic dysfunction and the risk of overwhelming sepsis in children with sickle cell-hemoglobin C disease (see HbC 141900.0038), although functional asplenia has been documented by radionuclide liver-spleen scans in some adult patients ( Ballas et al., 1982) and an elevated erythrocyte pit count, a finding that indicates functional asplenia in children with sickle cell anemia, also has been found in some children with SC disease ( Pearson et al., 1985). The risk of septicemia in sickle cell anemia is greatest during the first 3 years of life and is reduced markedly by prophylactic penicillin therapy. In many children with sickle cell anemia, functional asplenia develops during the first year of life and septicemia is the leading cause of death in childhood. Scriver and Waugh (1930) concluded that large aggregations of sickle cells seen in sinuses, vessels, and organs of sickle cell patients at autopsy reflected lowered oxygen tension resulting from death. Increased sickling was observed when oxygen pressure fell below 40 to 45 mm Hg. In a series of clever experiments that involved taking venous blood from the arm under different circumstances, the authors showed a correlation between oxygen tension and sickling of the red blood cells in vivo. Her main complaints were cough, night sweats, vague pains in the legs and joints, occasional abdominal pain, poor appetite, and increasing fatigue. Scriver and Waugh (1930) reported detailed studies of a 7-year-old child with sickle cell anemia. ![]()
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