RESUMO
AIM: To investigate the relation between head growth at different periods and IQ at 8 years, and to identify factors associated with more rapid head growth. METHOD: Two parallel cohorts of term low birthweight (LBW) and appropriate birthweight (ABW) infants were enrolled at birth in northeast Brazil. Anthropometric measurements were made at birth, 2 months, 6 months, 12 months, 24 months, and 8 years. Cognition was assessed at 8 years (n=164) with the Wechsler Intelligence Scale for Children. Multivariable analysis with a two-stage residual model was used to relate head growth between successive time points with IQ. RESULTS: Mean birthweight was 2.35kg (SD 0.15) in the LBW group (33 males, 50 females), and 3.21kg (SD 0.15) in the ABW group (28 males, 53 females). Mean gestational age was 38.8 weeks (SD 1.4) and 40.0 weeks (SD 1.3) respectively. In the LBW group, head growth from birth to 2 months and from 2 to 6 months, conditional on previous size, were significant independent predictors of IQ at 8 years. Conditional head growth from 6 months to 8 years and head size at birth were unrelated to IQ. In the ABW group there was no significant relation between conditional head growth and IQ for any period. Determinants of more rapid head growth from birth to 6 months in LBW infants were maternal height and rate of infant weight gain. INTERPRETATION: Head growth from birth to 6 months in term LBW infants is more important than prenatal or later postnatal head growth in predicting IQ at 8 years.
Assuntos
Cabeça/crescimento & desenvolvimento , Recém-Nascido de Baixo Peso , Inteligência/fisiologia , Antropometria , Peso ao Nascer , Brasil , Cefalometria , Criança , Pré-Escolar , Transtornos Cognitivos/diagnóstico , Estudos de Coortes , Feminino , Seguimentos , Idade Gestacional , Humanos , Lactente , Recém-Nascido , Masculino , Fatores de Risco , Meio Social , Fatores Socioeconômicos , Escalas de WechslerRESUMO
AIM: To investigate the development and behaviour of low-birthweight (LBW) term infants compared with matched term infants of appropriate birthweight (ABW). METHODS: Two parallel cohorts of LBW infants (1500-2499 g) and ABW controls (3000-3499 g) were enrolled at birth in northeast Brazil. At 8 y, 164 children were assessed using clinical and psychological tests. RESULTS: The LBW group had lower IQ scores than ABW children on the Weschler Intelligence Scale for Children; differences were larger on the performance (5 points, p = 0.04) than the verbal scale (3 points, p = 0.11). LBW children had poorer dynamic balance (p = 0.03) and eye-hand coordination (p = 0.02), but better selective attention (p = 0.02). Hyperactivity and conduct problems were common in both groups, but fewer LBW children had peer problems (p = 0.04). After controlling for social background, IQ was not significantly associated with birthweight (p = 0.10). Significant determinants were maternal education, home stimulation and type of school attended. The effect of birthweight on coordination and selective attention remained significant. Birth head circumference and growth in head size in the first 6 mo had independent effects on IQ. CONCLUSION: After controlling for social background, LBW term infants did not differ from ABW infants in cognition at school age. Head circumference at birth and 6 mo was a better predictor than birthweight of IQ.
Assuntos
Transtornos do Comportamento Infantil/epidemiologia , Deficiências do Desenvolvimento/epidemiologia , Atenção , Brasil/epidemiologia , Criança , Transtornos Cognitivos/epidemiologia , Feminino , Seguimentos , Humanos , Recém-Nascido , Estudos Longitudinais , Masculino , Análise Multivariada , Fatores de Risco , Fatores SocioeconômicosRESUMO
In individuals with homozygous sickle cell (SS) disease, haemolysis creates a demand for increased red cell production. In children with hypersplenism haemolysis may be further increased and in such cases the excessive red cell production may be reduced by splenectomy (1). Whole body protein turnover is increased in SS disease and a large part of this increase is the consequence of increased turnover of red blood cells (2). Whole body protein turnover has therefore been measured in children with SS disease before and after splenectomy to find out if the reduction of the haemolytic rate following splenectomy was associated with a decrease in protein turnover. (AU)
Assuntos
Humanos , Lactente , Pré-Escolar , Criança , Anemia Falciforme/metabolismo , Proteínas/metabolismo , Esplenectomia , Metabolismo Basal/fisiologia , Hemólise/fisiologia , HomozigotoRESUMO
Splenomegaly in sickle-cell disease is associated with excessive red cell destruction. We previously found whole body protein turnover and energy expenditure to be significantly higher in adults patients with sickle-cell disease compared to normals. The increase could be largely due to the increased turnover of the red cell, in particular the protein haemoglobin, in this disease state. We therefore measured whole body protein turnover in four children with sickle-cell disease, before and after splenectomy, to find out if the reduction in haemolysis that follows splenectomy would be reflected in a reduction in protein turnover. Protein turnover was measured with an oral primed intermittent infusion of 15N-glycine over 12-16 hours on the standard hospital diet. Isotopic excretion in urinary urea and ammonia were used to calculate protein turnover. (Table Included) Protein turnover was reduced by 23-47 percent after splenectomy. This reduction is presumably due to the fall in the excessive haemolysis that is associated with enlarged spleen. From a regression of protein turnover against energy expenditure in our previous study, it is estimated that the reduction in turnover after splenectomy could result in a theoretical saving of energy after splenectomy could be associated with the growth spurt that generally follow operation (AU)
Assuntos
Humanos , Masculino , Feminino , Criança , Esplenectomia , Anemia Falciforme , Metabolismo EnergéticoRESUMO
Hypersplenism, arbitrarily defined as a spleen more than 3 cm below the left costal margin, associated with an haemoglobin level below 6.5 gm/dl and platelet counts below 260 x 10/1, for at least one month, occurred in 38/308 (12.3 percent) children with SS disease in a cohort study followed from birth. Comparison of clinical and haematological features in 12 patients (10 SS; 2 S§ thalassemia), with splenomegaly but without hypersplenism, indicated that hypersplenic patients had significantly shorter red cell survival (mean 3.8 days, p=0.04), lower red cell volumes (p=0.03), higher plasma volumes (p=0.003) and higher blood volumes (p=0.02). These patients also had greater diploic expansion (p = 0.05) and greater cardiomegaly (p =0.0d). Following splenectomy, there were highly significant increases in haemoglobin levels, red cell volume, red cell survival, and platelet counts, and decreases in reticulocytes and plasma volume. Height velocity over the year following splenectomy increased in 7/9 patients, with adequate data, at ages when height velocity is normally falling. Hypersplenism is characterized by severe anaemia, rapid haemolysis, and bone expansion; the metabolic cost of the erythropoietic hyperactivity may compete with the demands for normal growth. Splenectomy is the optimal method of treatment in such cases (AU)
Assuntos
Humanos , Criança , Esplenomegalia , Hiperesplenismo , Anemia FalciformeRESUMO
Changes in mean cell volume in sickle cell disease must be judged against the patient's own steady state values. Two cases are reported in which megaloblastic erythropoiesis was overlooked because of genetically determined microcytosis. The effect of megaloblastic change was to increase the mean cell volume(MCV) but values remained in the range normal for other patients (AU)
Assuntos
Adolescente , Criança , Humanos , Masculino , Anemia Falciforme/sangue , Eritrócitos Anormais/patologia , Eritropoese , Anemia Falciforme , Índices de Eritrócitos , JamaicaRESUMO
Of a cohort of 308 children with homozygous sickle cell disease diagnosed at birth. 89 experienced 132 clinically significant attacks of acute splenic sequestration (ASS) over a 10-year period. The age at first attack ranged from 3 months to 6 years. Survival curve analysis of the interval until first attack indicated a cumulative probability of 0.225 by 2 years, 0.265 by 3 years, and 0.297 by 5 years of age. Thirteen events were fatal, 11 during the first attack, and all before transfusion could be instituted. Recurrences occurred in 49 percent of survivors of the first attacks, and there were diminishing intervals between subsequent events. Respiratory symptoms were associated with 52 of 132 events, but bacterial isolates on blood culture were less frequent, and ASS was not prevented by pneumococcal vaccine or penicillin prophylaxis. A high fetal hemoglobin level protected against attacks of ASS. A parental education program aimed at early diagnosis of ASS was followed by an increase in the incidence rate for ASS from a mean of 4.6 per 100 patient-years to 11.3 per 100 patient-years, probably reflecting increased awareness of the complication. During the same periods, the fatality rate fell from 29.4 per 100 events to 3.1 per 100 events. The improvement in outcome is likely to have resulted from improvement in medical management and earlier detection of ASS (AU)
Assuntos
Humanos , Pré-Escolar , Criança , Masculino , Feminino , Anemia Falciforme/complicações , Esplenopatias/complicações , Doença Aguda , Esplenopatias/diagnóstico , Esplenopatias/mortalidadeRESUMO
Homozygous sickle cell disease is an uncommon but important cause of hemiparesis and subarachnoid haemorrhage. A 24-year review showed that 42 cases of hemiparesis and 9 cases of subarachnoid haemorrhage had been seen at the University Hospital of the West Indies. All living patients (32) were examined in 1984 for residual defects. Twenty-two males and 20 females, with a median age of 7 years (range 8 months to 36 years) had had a stroke. Most (69 percent) were under 10 years of age at the time of the first stroke. No haematological or clinical predictor for stroke was identified, although transient ischaemic episodes prior to the onset of a complete stroke were seen in one 13-year-old. Recurrence clustered within the first 30 months with 10 of the 17 cases occurring in the first year. Contralateral occurrences were commonest (14) and resulted in death in 13. Subarachnoid haemorrhage was seen in 5 males and 4 females, aged 6 to 57 years (median 19 years), and 3 patients died. At re-evaluation of 32 patients, 8 had complete recovery, 20 residual hemiplegia and 4 quadriplegia. It is concluded that cerebro-vascular accident, though uncommon in sickle cell disease, affects a young age group and is associated with devastating consequences (AU)
Assuntos
Humanos , Masculino , Feminino , Lactente , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Anemia Falciforme/complicações , Hemorragia Subaracnóidea/etiologia , Hemiplegia/etiologiaRESUMO
60 Jamaican children with homozygous sickle cell (SS) disease underwent splenectomy, 14 for prophylaxis against recurrent acute splenic sequestration and 46 for treatment of sustained hypersplenism. Age at operation varied from 9 months to 27 years (median 6 years), with a total of 369 years of patient-observation. None of the 3 patients who died, at ages 2«, 6«, and 21 years, had received prophylaxis against infection. Overwhelming sepsis was possible but not confirmed in the first two deaths which occurred 11 months and 2« years after operation; the third died from chronic renal failure 11 years after splenectomy. After operation, there were no confirmed cases of pneumococcal septicaemia or meningitis, and the commonest clinical event was the acute chest syndrome. (Summary)
Assuntos
Humanos , Pré-Escolar , Criança , Masculino , Feminino , Anemia Falciforme/complicações , Infecções Bacterianas/imunologia , Esplenectomia/efeitos adversos , Doença Aguda , Anemia Falciforme/genética , Anemia Falciforme/fisiopatologia , Seguimentos , Homozigoto , Hiperesplenismo/sangue , Hiperesplenismo/mortalidade , Hiperesplenismo/cirurgia , Complicações Pós-Operatórias/mortalidade , Recidiva , Risco , Sepse/imunologia , Fatores de TempoRESUMO
The persistence of splenomegaly in children with homozygous sickle cell (SS) disease can pose difficult management problems for the clinician. In the young child the prognosis of acute spenic sequestration (ASS) is worse if superimposed on hypersplenism and in the older child the development of chronic hypersplenism may have a wide range of detrimental effects on the child's health. Elective splenectomy abolishes the risk of further acute, or chronic, sequestration but the increased risk of fulminant sepsis following splenectomy and the known propensity to spontaneous atrophy counsels for conservative mangement. To understand better the role of splenectomy in SS disease the outcome of elective splenectomy in 60 Jamaican children is reviewed. The age at time of operation ranged from 9 months to 17 years, with a mean of 6 years and the period of follow up ranged from 1 month to 19 years. The principal indications were recurrent ASS under the age of 2 years and persistent hypersplenism in those over this age. There was no mortality or significant morbidity during the operative period. Two deaths occurred, one from pneumonia 11 months post operatively and one from unknown causes 30 months post operatively. The haematological response to splenectomy was striking, with significant sustained increases in haemoglobin, platelets and red cell survival times, and fall in reticulocyte count. In the last five years prophylaxis with penicillin and/or pneumococcal and Haemophilus influenzae vaccine has been given to most patients and may have improved the prognosis, but in 54 patients splenectomy precede this period. Elective splenectomy with modern surgical and anaesthetic techniques carries minimal morbidity and with careful patient selection (two episodes of ASS, or sustained hypersplenism) the procedure may confer considerable benefit on selected children with SS disease (AU)
Assuntos
Humanos , Lactente , Pré-Escolar , Criança , Adolescente , Anemia Falciforme/cirurgia , Esplenectomia , HiperesplenismoRESUMO
A questionnaire study of Jamaican patients with homozygous sickle cell (SS) disease indicated a 42 percent prevalence of priapism, with a median age at onset of 21 years. Two predominantly different patterns of priapism were recorded: short "stutering" episodes lasting less than three hours, with normal consequent sexual function, and severe prolonged attacks (generally more than 24 hours) commonly followered by impotence. Stuttering episodes were frequently a prodrome to a major attack. Over one fourth of those who had suffered priapism had some degree of impotence. Hematologic analysis indicated that priapism was significantly associated with low hemoglobin F levels and high platelet counts. Patients with severe attacks of priapism had lower hemoglobin F levels and reticulocyte count, and a higher mean corpuscular volume, than patients with only stuttering episodes. Priapism and impotence contribute to the morbidity of SS disease more frequently than previously recognized (AU)