RESUMEN
OBJECTIVE: To examine the association between fetal outcome and the steady state haematology of mothers with homozygous sickle cell disease. DESIGN: A retrospective observational study. The data were taken from dockets kept at the Sickle Cell Clinic and verified by interview with 45% of the patients. SETTING: The Sickle Cell Clinic at the University Hospital of the West Indies or two peripheral clinics operated by the staff of the MRC Laboratories. SUBJECTS: All women aged 14 years or older with homozygous sickle cell disease who had experienced at least one pregnancy in the period 1977 to 1986. MAIN OUTCOME MEASURES: Three fetal outcomes including miscarriages, perinatal deaths, and birthweight. RESULTS: There were 270 singleton pregnancies in 175 women with an overall fetal wastage of 32.2%. There was a significant increased risk of perinatal death with low maternal fetal haemoglobin level, but there were no haematological associations with miscarriages or birthweight. CONCLUSIONS: These data suggest that maternal steady-state haematology has little influence on fetal outcome, with the exception that mothers with high HbF levels are less prone to perinatal deaths. Further study is required to investigate acute haematological changes associated with pregnancy.
Asunto(s)
Anemia de Células Falciformes/sangre , Hemoglobina Fetal/análisis , Hemoglobinas/análisis , Complicaciones Hematológicas del Embarazo/sangre , Aborto Espontáneo/sangre , Aborto Espontáneo/epidemiología , Adolescente , Adulto , Anemia de Células Falciformes/mortalidad , Peso al Nacer , Femenino , Muerte Fetal , Humanos , Recién Nacido , Embarazo , Complicaciones Hematológicas del Embarazo/mortalidad , Estudios Retrospectivos , Factores de RiesgoRESUMEN
The effect of sectoral, scatter laser photocoagulation on proliferative sickle retinopathy (PSR) was investigated by reviewing fluorescein angiograms of 88 sickle cell-haemoglobin C patients enrolled in a controlled, randomised trial. Follow-up was for a median period of 2.9 years. Complete infarction of all PSR in an eye occurred in 7 of 74 treated eyes and 2 of 60 control eyes. Treatment resulted in significantly greater regression (decrease in number or size of PSR lesions) in eyes of patients aged < 25 years at enrollment but not in eyes of patients > or = 25 years at enrollment. Infarction of individual PSR lesions was significantly more common in treated eyes. Treated PSR was significantly more likely to infarct if small (< 15 degrees circumferential involvement) and if flat rather than elevated. New PSR was significantly less likely to develop in treated eyes.
Asunto(s)
Enfermedad de la Hemoglobina C/complicaciones , Coagulación con Láser , Retina/cirugía , Enfermedades de la Retina/cirugía , Adulto , Angiografía con Fluoresceína , Humanos , Enfermedades de la Retina/etiologíaRESUMEN
The relevance of fetal haemoglobin (HbF) concentration to the development of early clinical manifestations of homozygous sickle (SS) disease has been investigated by examining the time to first occurrence and the proportional hazard of these complications in three groups of the HbF distribution at age 5 years. HbF was significantly related to dactylitis, painful crises, acute chest syndrome, and acute splenic sequestration. The relationship suggested that a critically low HbF concentration increased the risk, little difference in risk occurring between the medium and high HbF groups. The abdominal painful crisis and hypersplenism were not related to HbF concentration suggesting that the degree of sickling may not be important in their genesis. Parental education on acute splenic sequestration should be focused on children with HbF concentrations in the lowest part of the HbF distribution for age.
Asunto(s)
Anemia de Células Falciformes/sangre , Hemoglobina Fetal , Adolescente , Factores de Edad , Anemia de Células Falciformes/genética , Anemia de Células Falciformes/mortalidad , Niño , Preescolar , Femenino , Humanos , Inflamación/etiología , Masculino , Dolor/etiología , Modelos de Riesgos Proporcionales , Factores de Riesgo , Esplenomegalia/etiología , Análisis de SupervivenciaRESUMEN
Strokes occurred in 17 of 310 children with homozygous sickle cell disease who were followed from birth, representing an incidence of 7.8% by the age of 14 years. Two children had subarachnoid hemorrhage, one having resolution of symptoms after aneurysm surgery and another dying of a presumed second hemorrhage 14 days later. The remaining 15 strokes were presumed to be cerebral infarction, although autopsy, angiographic, or computed tomographic evidence was available in only 8 children. There were 6 deaths, 2 in the acute event and 4 after recurrence, which occurred in 6 (46%) of 13 patients who survived the initial episode. There were 10 recurrent episodes at a median interval of 9 months after the initial event. Steady-state hematologic data revealed significantly higher leukocyte counts than in control subjects without strokes at age 1 year and in the last study preceding the stroke. The initial stroke coincided with an acutely lowered hemoglobin value in 5 patients (3 aplastic crises, 1 acute splenic sequestration, 1 probable pulmonary sequestration) and with painful crises in another 7 patients. We conclude that a high leukocyte count and an acute decrease of hemoglobin are risk factors for stroke in patients with homozygous sickle cell disease.
Asunto(s)
Anemia de Células Falciformes/complicaciones , Trastornos Cerebrovasculares/etiología , Adolescente , Anemia de Células Falciformes/sangre , Anemia de Células Falciformes/genética , Recuento de Células Sanguíneas , Niño , Preescolar , Estudios de Cohortes , Femenino , Hemiplejía/etiología , Hemoglobinas/análisis , Homocigoto , Humanos , Lactante , Masculino , Factores de RiesgoRESUMEN
The clinical and haematological features of homozygous sickle cell (SS) disease were compared in 30 Greek and 310 Jamacian patients. Deletional alpha-thalassaemia, which modifies SS disease, is rare among Greek patients, so only Jamacian patients with four alpha-globin genes were included in the control group. Greek patients had higher total haemoglobin concentration and red cell counts, and lower mean cell haemoglobin concentration (MCHC) and reticulocyte counts. They also had a more normal body build and more adults had persistent splenomegaly. Fewer had a history of leg ulceration or priapism but more reported acute chest syndrome. The comparatively mild disease in Greek patients is consistent with less haemolysis and sickling and therefore less bone marrow expansion. In the absence of amelioriating factors such as high HbF concentration or alpha-thalassaemia, these findings may be explained by the low MCHC.
Asunto(s)
Anemia de Células Falciformes/genética , Homocigoto , Adolescente , Adulto , Anemia de Células Falciformes/sangre , Anemia de Células Falciformes/complicaciones , Niño , Estudios Transversales , Recuento de Eritrocitos , Índices de Eritrocitos/genética , Femenino , Hemoglobina Fetal/análisis , Genotipo , Grecia , Hemoglobina A2/análisis , Hemoglobina Falciforme/análisis , Humanos , Jamaica , Masculino , Fenotipo , Reticulocitos , Estudios Retrospectivos , Talasemia/sangre , Talasemia/complicaciones , Talasemia/genéticaRESUMEN
The prevalence of nocturnal enuresis (wet at least two nights a week) was investigated in children, aged 8, who were being followed up as part of a prospective cohort study. There were 175 children with homozygous sickle cell disease, 106 with sickle cell haemoglobin C disease, and 150 controls with a normal haemoglobin genotype. In homozygous sickle cell disease, 48 boys (52%) and 31 girls (38%) were enuretic, a significantly higher prevalence than in those with sickle cell haemoglobin C disease--five boys (10%) and 11 girls (20%)--or in normal children--16 boys (22%) and 13 girls (17%). There was no significant difference between children with sickle cell haemoglobin C disease and the normal genotype. Boys with homozygous sickle cell disease were significantly more likely to be enuretic if they came from large families; there was a similar trend for girls with homozygous sickle cell disease, although it did not reach significance. Enuresis was more common in boys with homozygous sickle cell disease who had low concentrations of fetal haemoglobin and in girls with sickle cell haemoglobin C disease who had high mean corpuscular haemoglobin concentrations. Similar associations were not shown for girls with homozygous sickle cell disease or boys with sickle cell haemoglobin C disease.
Asunto(s)
Anemia de Células Falciformes/complicaciones , Enuresis/etiología , Niño , Estudios de Cohortes , Enuresis/epidemiología , Composición Familiar , Femenino , Enfermedad de la Hemoglobina SC/complicaciones , Humanos , Jamaica/epidemiología , Masculino , Prevalencia , Factores Sexuales , Factores SocioeconómicosRESUMEN
The prevalence, incidence, and risk factors associated with proliferative sickle retinopathy (PSR) were investigated in 786 patients with homozygous sickle cell (SS) disease and 533 patients with sickle cell haemoglobin C (SC) disease. PSR was more common in SC disease, in which there was a significant predominance of males, and it increased with age in both genotypes. In SC disease the risk of developing PSR was highest between 15 and 24 years in males, between 20 and 39 years in females, and in SS disease between 25 and 39 years in both sexes. PSR tended to be bilateral, especially in SC disease. There was no evidence of familial clustering of PSR in SC siblings, and insufficient numbers of SS siblings were available to test for clustering. Haematological risk factors associated with PSR in SS disease were a high haemoglobin in males and a low fetal haemoglobin in both sexes and in SC disease, a high mean cell volume, and a low fetal haemoglobin in females.