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At least 19 recordsLinked to original sources
PubMed1988

Sickle cell heart disease. Two-dimensional echo and Doppler ultrasonographic findings in the hearts of adult patients with sickle cell anemia.

Sickle cell anemia (SCA) is a commonly diagnosed condition in the black population in the United States. With better medical management, patients with SCA are living longer and what were previously uncommon sequelae are being recognized frequently, including those involving the cardiovascular system. Previous reports on the cardiovascular effects of SCA have focused predominantly on children, but the conclusions remain controversial. To study this question further, 40 adult patients with documented SCA were examined using two-dimensional and Doppler cardiac ultrasound. Valvular structures were normal despite an increased incidence of flow murmurs. Abnormalities were found more frequently in the left heart than the right, as manifested primarily by increased left ventricular mass, and left ventricular and left atrial dilatation with preservation of systolic function. Pulmonary hypertension, which was present in two thirds of the sample, was minimal to moderate as assessed by our Doppler technique. The effects of SCA on the heart seem to be minimal and similar to those of other anemias, predominantly confined to the left atrium and ventricle with passive elevation of pulmonary pressures. Clinical murmurs were most often physiologic; there was no association with myxomatous valvular degeneration or mitral valve prolapse.

Adult↗
PubMed1979

Microcytosis associated with sickle cell anemia.

Sickle cell (Hb SS) anemia is considered a normochromic-normocytic hemolytic disorder. In 53 patients with Hb SS (mean reticulocyte values 16.8%), the authors observed that mean corpuscular hemoglobin (MCH) was 29.8 +/- 2.4 mu microgram and mean corpuscular hemoglobin (MCV) was 88.1 +/- 6.8 cu micrometers. In contrast, patients in a comparable hemolytic-disease group unrelated to hemoglobinopathies (mean reticulocyte count = 15.7%) had a higher MCH (33.0 +/- 1.8 mu microgram) and larger MCV (97 +/- 5.3 cu micrometers). These data indicate that Hb SS disease is associated with "relative microcytosis," presumably a consequence of reduced hemoglobin production.

Anemia, Sickle Cell↗
PubMed1976

[Sickle cell anemia].

Sickle cell disease is a hereditary chronic hemolytic anaemia mostly found in negroes. The blood of these patients contains hemoglobin S. Absence of oxygen determines the sickling to the red blood cells. In the heterozygous state clinical manifestations usually are absent. The homozygous form shows different symptoms. The progressive course with hemolytic, aplastic or thrombotic crises mostly ends in an early death in infancy or childhood. Treatment is largely palliative. Transfusions, treatment of the crises and general measures play the most essential role.

Africa↗
PubMed1989

The presentation and management of the acute abdomen in the patient with sickle-cell anemia.

Sickle crises frequently manifest as abdominal pain that may simulate intra-abdominal infection. To establish parameters to distinguish these, we retrospectively studied 53 patients with sickle-cell anemia who had abdominal pain (genotype SS 62%, SC 15%, SA 11%, S-other 11%; 30% men and 70% women; mean age 23). A vaso-occlusive crises was responsible for the pain in 57 per cent; 23 per cent had a surgical entity and 20 per cent had a nonsurgical genitourinary disorder. Of the surgical conditions, 9 of 12 patients (95%) had cholecystitis and 4 of 12 patients (33%) had acute appendicitis (one patient had both). Vaso-occlusive crises were diffuse in 15 of 30 patients (50%), compared with proven surgical conditions, and was more often associated with remote pain such as limbs and chest (23 of 30 [77%] P less than 0.005). The pain of vaso-occlusive crises simulated prior crises in 21 of 30 patients (70%) compared with 1 of 12 patients (8%) who had surgical abdominal pain (P less than 0.005). A precipitating event (especially upper respiratory infection) was found in 50 per cent of abdominal vaso-occlusive crises versus 0 per cent of surgical abdomens (P less than 0.010). The pain was relieved with hydration and oxygen in 97 per cent of sickle crises within 48 hours versus 0 per cent of surgical abdomens (P less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Abdomen, Acute↗
PubMed1978

Hepatic bilirubin UDP-glucuronyltransferase in patients with sickle cell anemia.

In sickle cell anemia the shortened survival of red blood cells presents the liver with an augmented load of bilirubin for hepatic clearance. To determine the effects of this excessive bilirubin load on the microsomal conjugating enzyme, hepatic bilirubin UDP-glucuronyltransferase, levels of this enzyme were measured in liver biopsies from patients with sickle cell anemia and several comparison groups. UDP-glucuronyltransferase activity in 14 patients with sickle cell anemia was 2-fold greater (P less than 0.005) than in 14 nonjaundiced comparison patients without liver disease. The elevated UDP-glucuronyltransferase activity in sickle cell anemia was similar to that found in 10 patients who chronically ingested drugs (barbiturates or estrogens) known to increase UDP-glucuronyltransferase activity. These observations suggest enhanced conjugation of bilirubin in patients with sickle cell anemia may result from substrate (bilirubin) induction of UDP-glycuronyltransferase.

Adolescent↗
PubMed1977

Differential diagnosis of some simple osseous lesions associated with sickle-cell anemia.

Sickle-cell disease is characterized by chronic hemolytic anemia, rapid destruction of defective erythrocytes, with consequent circulatory disturbances. Thromboses and infarctions with resultant osseous manifestations tend to be localized in the ends of the long bones or in the medullary area, the latter often becoming apparent in the jaws. Three case reports are presented to illustrate osseous changes observed in the jaws of these sickle-cell patients. The radiographic findings demonstrate the adaptive mechanisms of bone metabolism in response to vascular infarction, infection, or both in some sickle-cell anemia patients. A differential diagnosis is suggested for osseous lesions observed in the jaws of these patients.

Adolescent↗
PubMed1992

[Genetic counseling and sickle cell anemia].

In sickle cell anaemia genetic counselling benefits from the easy diagnosis and, as early as the foetal stage, from the heterozygosity (AS) and the major syndromes. Its main objective is to inform as many as possible of the subjects concerned and in particular couples with genetic risk. Counselling gives them all the elements they require to decide freely about their offsprings. It is part of the management of sickle cell anaemia patients and is often associated with the neonatal diagnosis. The ever increasing number of justified requests for a prenatal diagnosis and the early time of these requests during pregnancy show that the information has really been transmitted. Actions remain to be developed in certain medical groups and in the populations at risk. A sickle cell anaemia centre is necessary for a better rationalization of genetic counselling.

Anemia, Sickle Cell↗
PubMed1992

[Natural history of sickle cell anemia].

Sickle-cell anaemia is characterized by three categories of clinical signs: anaemia, vaso-occlusive phenomena and infective complications, which are described here according to age. The natural history of the disease can be divided into four periods: the neonatal period which is asymptomatic but important to organize an effective protection; the first 5 years of life are characterized by a high risk of mortality, a high level of morbidity due to severe infections, episodes of acute anaemia and painful crises typical of that age-group; the life of older children and adolescents is dotted with painful crises; it is in this period that degenerative tissue pathology begins; in adulthood, the acute episodes are less frequent, but multiple complications develop. Some of them (cerebral vascular accidents or lung diseases) may be fatal, while others are the source of chronic and disabling lesions, notably ocular, orthopaedic and renal lesions, which affect the functional prognosis. Pregnancy remains a high risk. There is, therefore, a striking contrast between the basic physiopathological mechanism (polymerization of haemoglobin S) and the various clinical manifestations which depend on the type of haemoglobin, on the social and sanitary conditions in each country and on other reasons which remain to be elucidated.

Adolescent↗
PubMed1992

Hypothyroidism in adults with sickle cell anemia.

Persons with sickle cell anemia have several indications for transfusion of red blood cells. One of the complications of transfusion of red blood cells is iron overload. Iron overload has been associated with multiple endocrine abnormalities. We report herein three cases of hypothyroidism in adult individuals with sickle cell disease. All three patients were over the age of 45 years at the time of the diagnosis and had received multiple units of transfused red blood cells and had serum ferritin levels of greater than 6,000 ng/mL. All patients were diagnosed during times when they were critically ill. Replacement therapy was instituted in all cases; however, all three patients died shortly after the diagnosis of hypothyroidism was made. Congestive heart failure appeared to be a primary cause of death in all three patients. In the one patient in whom a postmortem examination was done, there was evident extensive fibrosis of the thyroid gland as well as extensive deposition of iron in the cells lining the thyroid follicles. We believe that this represents the first report of clinical hypothyroidism in patients with sickle cell anemia who have received multiple transfusions. Awareness of this condition is especially important given that congestive heart failure is common in sickle cell disease.

Anemia, Sickle Cell↗
PubMed1989

Increased incidence of bacteremia in Haitian children with sickle cell anemia.

Children with sickle cell anemia who were less than 5 years old were observed over a three-year period. Those born to recent Haitian immigrants accounted for 108.4 patient-years. They had seven episodes of Streptococcus pneumoniae bacteremia and five due to Haemophilus influenzae for rates of 6.5 and 4.6 episodes per 100 patient-years, respectively. Children born to American parents were observed for 131.3 patient-years and had three episodes and one episode, respectively, for rates of 2.3 and 0.76 episodes per 100 patient-years. The differences in the overall rate of bacteremia were significant (P less than .015). Leukocyte counts and percentage of erythrocytes with pits, indicating decreased splenic function, were similar for the two groups. The Haitian families had lower annual incomes, but values for both groups were so low that their difference is unlikely to be related to the increased infection rate. Although no cause for the higher rate of bacteremia could be found, the approach to febrile illness in Haitian children with sickle cell anemia should be even more aggressive than usual. In addition to Haemophilus influenzae immunization, antibiotics effective against penicillin-resistant species should be used in the initial antibiotic coverage of their febrile illnesses.

Anemia, Sickle Cell↗
PubMed1992

Hydroxyurea: effects on hemoglobin F production in patients with sickle cell anemia.

Patients with sickle cell anemia were treated with daily doses of hydroxyurea, to assess pharmacokinetics, toxicity, and increase in fetal hemoglobin (Hb) production in response to the drug. Plasma hydroxyurea clearances were not a useful guide to maximum tolerated doses of the drug. The mean daily single oral dose that could be maintained for at least 16 weeks was 21 mg/kg (range, 10 to 35 mg/kg). Among 32 patients, last HbF levels were 1.9% to 26.3% (mean, 14.9%) with increases in HbF over initial values of 1.4% to 20.2% (mean, 11.2%). The most significant predictors of last HbF were last plasma hydroxyurea level, initial white blood count and initial HbF concentration. Last HbF was not related to beta globin haplotype or alpha globin gene number. No serious toxicity was encountered. Clinically significant bone marrow depression was avoided, and chromosome abnormalities after 2 years of treatment were no greater than those observed before treatment. The period of observation has been too short to evaluate the risk of carcinogenesis. Patient's red cells developed striking macrocytosis. Median red cell Hb concentrations did not change. Hb concentrations increased, on average 1.2 g/dL, but serum erythropoietin levels increased. Patients' body weights increased, and some returned to work or school, but no conclusions regarding therapeutic efficacy could be drawn from this uncontrolled open-label study.

Adult↗
PubMed1987

HTLV-I antibody and cell-mediated immunity status in sickle cell anemia.

Patients with sickle cell anemia (SCA) had an abnormal susceptibility to infections. In Martinique (French West Indies), a human T-cell leukemia/lymphoma virus type I (HTLV-I) endemic area, we found that 17 (10%) of 173 SCA patients had antibodies to HTLV-I. The possible relationship between HTLV-I seropositivity and altered immunity was studied in 13 SCA patients with HTLV-I antibodies compared with 13 matched SCA patients without HTLV-I antibodies. The immunological results, as evaluated by the T-cell subsets analysis, the lymphocyte proliferation responses analyzed after activation with concanavalin A, phytohemagglutinin, or pokeweed mitogen, and the natural killer activity were not statistically different in these two groups of patients (SCA HTLV-I positive vs SCA HTLV-I negative). These data suggest that HTLV-I infection did not result in a major alteration of cellular immunity in this population.

Anemia, Sickle Cell↗
PubMed1987

Sickle cell anemia.

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Anemia, Sickle Cell↗