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1.
Kingston; s.n; 2000. 55 p. tab, gra.
Tese em Inglês | MedCarib | ID: med-486

RESUMO

Sickle cell disease is a global health problem. It is an inherited blood disorder affecting the red blood cells. There are different types of sickle cell disease. Homozygous sickle cell (SS) disease, results when the infant inherits two abnormal sickle genes, one from each parent. In Jamaica SS disease affects approximately 1 in 300 births and some form of sickle cell disease, 1 in 150 births. Complications of sickle cell disease include acute splenic sequestration. The cause and triggers of acute splenic sequestration (ASS) are largely unknown. The spleen becomes acutely enlarged, trapping a proportion of the red cell mass and leading to acute anaemia, circulatory failure and sometimes death. Emergency blood tranfusion is extremely important in the management of acute splenic sequestration. Parental education may allow prevention of death from ASS by early transfusion. Parents need to be taught how to diagnose splenic enlargement, and to recognize the signs and symptoms of increasing anaemia characterized by pallor. It is important for mothers to examine splenic size on a regular basis and when the child looks ill. At the Jamaican Sickle Cell Unit, parents are instructed to examine the spleen at least once a day. The aim was to identify the parents' and guardians' knowledge of ASS, their attitudes towards the examination of the abdomen and practices in detecting an enlarging spleen. The objectives were to determine the percentage of parents and guardian who regularly examined the abdomen to determine spleen size; who had detected an enlarging spleen and the outcome; who were aware of the proper technique for examining the spleen; who were aware of what to do if an enlarging spleen was found; and identify the barriers that prevent regular examination for splenic enlargement. The study was carried out on the parents and guardians of children four years and younger, with homozygous sickle cell disease, attending the sickle cell unit. At the time of the study, the population of children four years and younger with SS disease was 23.7. A questionnaire was designed based on the objectives of the study, to solicit data from the partcipants. The researcher administered the questionnaire to 60 parents and guardians who attended the clinic over a two-month period- March and April 2000. The participants were also asked to demonstrated their competence in examining the spleen.(Au)


Assuntos
Adulto , Criança , Pré-Escolar , Pessoa de Meia-Idade , Idoso , Humanos , Lactente , Adolescente , Esplenopatias/diagnóstico , Anemia Falciforme/complicações , Conhecimentos, Atitudes e Prática em Saúde , Jamaica , Baço/anormalidades , Pais/educação , Hiperesplenismo , Esplenopatias/prevenção & controle , Estudos de Coortes
2.
Eur J Clin Nutr ; 50(10): 672-5, Oct. 1996.
Artigo em Inglês | MedCarib | ID: med-2134

RESUMO

OBJECTIVE: To determine whether, in the same individual, an observed fall in whole body protein turnover following splenectomy in children with hypersplenism and homozygous sickle cell (SS) disease is associated with a measurable fall in resting metabolic rate (RMR) and an increase in rate of growth. SUBJECTS: Six children (5 SS disease, 1 S beta degree thalassaemia), aged 68 to 126 months, were studied before and after splenectomy for hypersplenism. DESIGN: Protein turnover was measured by the end product method using prime/intermittent oral doses of 15N-glycine and RMR by indirect calorimetry before preoperative transfusion and repeated at least eight weeks after splenectomy. Height and weight velocities were measured over six month periods before and after splenectomy. SETTING: University Hospital of the West Indies in Jamaica and the Medical Research Laboratories (Jamaica). RESULTS: After splenectomy protein turnover fell significantly by 30 percent and RMR by 34 kJ/kg/d. Mean weight velocity which was below normal before surgery, z = -2.3, improved significantly after surgery, z = 0.7, (p = 0.03). Height velocity increase in two children but the mean height velocity did not change following splenectomy. The reduction in protein turnover was estimated to account for 62 percent of the fall in RMR. CONCLUSION: This study confirms that there is a significant reduction in energy expenditure following splenectomy for hypersplenism in SS disease. A reduction in protein turnover was a major contributor to the saving in energy, although it is not clear whether it accounted for all. In the present group of children the energy saved was associated with an improvement in the wasting present before splenectomy.(AU)


Assuntos
Criança , Feminino , Humanos , Masculino , Anemia Falciforme/cirurgia , Metabolismo Basal , Hiperesplenismo/cirurgia , Proteínas/metabolismo , Esplenectomia , Metabolismo Energético , Antropometria , Anemia Falciforme/genética , Anemia Falciforme/metabolismo , Hiperesplenismo/genética , Hiperesplenismo/metabolismo , Consumo de Oxigênio
3.
Arch Dis Child ; 72(3): 227-9, March 1995.
Artigo em Inglês | MedCarib | ID: med-5894

RESUMO

Chronic hypersplenism in homozygous sickle cell (SS) disease markedly increases haemolysis and the resulting erythropoietic expansion is likely to have a high metabolic cost. Splenectomy for hypersplenism in SS disease is followed by highly significant changes in haematological indices and an increase in height, but not weight, velocity. This pattern is similar to that observed in the trichuris dysentery syndrome after treatment, and differs from the increase in both height and weight velocity that follow nutritional supplementation of severely malnourished children. It is postulated that accelerated linear growth after the reduction in erythropoietic stress may implicate a specific nutrient deficiency in hypersplenic children with SS (AU)


Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Crescimento , Hiperesplenismo/cirurgia , Traço Falciforme/complicações , Peso Corporal , Estatura , Índices de Eritrócitos , Testes Hematológicos , Hiperesplenismo/sangue , Hiperesplenismo/fisiopatologia , Traço Falciforme/sangue , Traço Falciforme/fisiopatologia , Traço Falciforme/cirurgia , Esplenectomia
4.
West Indian med. j ; 36(Suppl): 46, April 1987.
Artigo em Inglês | MedCarib | ID: med-5983

RESUMO

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 Falciforme
5.
Lancet ; 1(8368): 88-91, Jan. 14, 1984.
Artigo em Inglês | MedCarib | ID: med-12148

RESUMO

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 Tempo
6.
West Indian med. j ; 32(Suppl): 31, 1983.
Artigo em Inglês | MedCarib | ID: med-6134

RESUMO

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 , Hiperesplenismo
7.
Arch Dis Child ; 56(10): 765-9, Oct. 1981.
Artigo em Inglês | MedCarib | ID: med-14375

RESUMO

A cord blood screening programme initiated in June 1973 had screened 68 000 normal deliveries by February 1979 with the detection of 216 cases of homozygous sickle cell disease. Regular review of these children in the Medical Research Council paediatric clinic has identified acute splenic sequestration as a major cause of morbidity and mortality in the first 5 years of life. In addition to classical episodes characterised by peripheral circulatory failure, minor episodes of increasing anaemia associated with an enlargening spleen and an active marrow were also common. These minor episodes appeared to have predictive value in children who later developed severe life-threatening episodes ofacute splenic sequestration. Sustained hypersplenism was also appreciably more common in children developing minor or major episodes of acute splenic sequestration compared with those without such a history. It is proposed that the classification of acute splenic sequetration be expanded to include these minor episodes, and that consideration be given to prevention of recurrences by splenectomy particularly in patients who also develop sustained hypersplenism (Summary)


Assuntos
Humanos , Lactente , Pré-Escolar , Masculino , Feminino , Anemia Falciforme/complicações , Esplenopatias/etiologia , Anemia Falciforme/mortalidade , Infecções Bacterianas/complicações , Hiperesplenismo/etiologia , Hiperesplenismo/mortalidade , Esplenopatias/mortalidade
8.
West Indian med. j ; 19(4): 260, Dec. 1970.
Artigo em Inglês | MedCarib | ID: med-6356

RESUMO

Traditionally sickle cell anaemia is characterised by splenic atrophy in adult life but occasional persistence of splenomegaly is now well recognized. Splenomegaly is not present at birth and reached a maximum incidence of 60-70 percent at the age of 18 months. There is then a gradual disappearance of splenomegaly which falls to 35 percent at the age of 12 years, 19 percent at the age 18 and finally to 9 percent in patients over the age of 25 years. Since the persistence of splenomegaly is frequently associated with a benign clinical course, it was felt important to find what factors favoured its persistence in some patients. High levels of foetal haemoglobin and low counts of irreversibly sickled cells (ISCs) were two factors found to be associated with persistence of splenomegaly. Low ISC counts have perviously been observed in splenomegaly and attributed to sieve like removal of these cells by the spleen. Evidence presented here suggests that low ISCs may be the cause of splenomegaly and the result. The practical implications of these observations are threefold. Firstly, it is the first evidence that ISCs may be related to tissue infarction. Secondly, occasionally splenectomy indicated on other grounds, may be deferred for fear that removal of the sieve like action of the spleen will be followed by an increased in ISCs and perhaps thrombatic phenomena. The evidence is against this. Thirdly, in children with hypersplenism, the decision to perform splenectomy or await spontaneous atrophy may be a relevant problem. It is suggested that since spontaneous atrophy is more common in cases with low Hb F levels, conservative therapy may be preferred (AU)


Assuntos
Humanos , Criança , Esplenomegalia , Anemia Falciforme/complicações , Hiperesplenismo
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