RESUMO
OBJECTIVE: To determine whether children with homozygous sickle cell (SS) disease and splenectomy are at greater risk of death, overwhelming septicemia, or other complications. METHODS: A total of 130 patients with SS treated by splenectomy (46 recurrent acute splenic sequestration, 84 chronic hypersplenism) over a 22.5-year period at the Sickle Cell Clinic of the University Hospital of the West Indies, Kingston, Jamaica, were compared with a control group matched for sex, age, and duration of follow-up in a retrospective review. Deaths and bacteremias were examined over the whole study period. Painful crises, acute chest syndromes, and febrile episodes were compared in the 90 patients completing 5 years of postsplenectomy follow-up. FINDINGS: Mortality and bacteremic episodes did not differ between the splenectomy and control groups. Painful crises were more common in the splenectomy group than in the control group (P =.01) but did not differ between splenectomy indications. Acute chest syndrome was more common in the splenectomy group than in the control group (P <.01) and was more common in the acute splenic sequestration group than in the hypersplenism group (P =.01). Febrile events did not differ between the groups or between the indications for splenectomy. CONCLUSION: Splenectomy does not increase the risk of death or bacteremic illness in patients with SS disease and, if otherwise indicated, should not be deferred for these reasons.
Assuntos
Anemia Falciforme/complicações , Homozigoto , Esplenectomia , Adolescente , Adulto , Anemia Falciforme/mortalidade , Anemia Falciforme/cirurgia , Antibioticoprofilaxia , Bacteriemia/epidemiologia , Bacteriemia/prevenção & controle , Estudos de Casos e Controles , Causas de Morte , Distribuição de Qui-Quadrado , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Jamaica/epidemiologia , Modelos Logísticos , Masculino , Penicilinas/uso terapêutico , Estudos Retrospectivos , Fatores de Risco , Esplenectomia/estatística & dados numéricosRESUMO
The dumping syndrome in childhood is an uncommon complication of gastro-oesophageal surgery, principally Nissen fundoplication. A Jamaican child developed the syndrome after fundoplication and pyloroplasty to relieve gastro-oesophageal reflux complicating the repair of a congenital tracheo-oesophageal fistula. He developed marasmus and failed to gain weight on the standard remedial milk-based high-energy diet. An oral glucose tolerance test confirmed the diagnosis of dumping syndrome. A low sugar low milk diet based on adult type meals with continuous nibbling of fried dumplings relieved his diarrhoea and hypoglycaemia and he gained weight. This is a cheaper and more practical dietary therapy than the regimens described previously.
Assuntos
Humanos , Lactente , Síndrome de Esvaziamento Rápido/etiologia , Antro Pilórico/cirurgia , Fundoplicatura/efeitos adversos , Jamaica/epidemiologia , Nefropatias/cirurgia , Síndrome de Esvaziamento Rápido/dietoterapia , Síndrome de Esvaziamento Rápido/epidemiologiaRESUMO
Data were collected prospectively on 57 Jamaican children presenting with 62 episodes of acute intussusception over a two year period, for whom operative and hydrostatic methods of reduction were employed. 31 (54%) of 57 episodes were reduced successfully using barium (42), saline (11) and air (4) hydrostatically. Among the 31 other episodes, 15 had ileo-colic intussusception, seven caeco-colic, six ileo-ileo-colic and one ileo-ileal. Two patients had spontaneous reduction discovered at surgery. There were two episodes of barium hydrostatic perforation of the colon leading to death in one patient. Hydrostatic reduction is recommended as the first therapeutic option for acute intussusception because it spares the patient a major operative procedure when successful.
Assuntos
Intussuscepção/terapia , Irrigação Terapêutica , Doença Aguda , Sulfato de Bário/uso terapêutico , Enema , Feminino , Humanos , Pressão Hidrostática , Lactente , Intussuscepção/etiologia , Masculino , Estudos Prospectivos , Cloreto de Sódio/uso terapêuticoRESUMO
Data were collected prospectively on 57 Jamaican children presenting with 62 episodes of acute intussusception over a two year period, for whom operative and hydrostatic methods of reduction were employed. 31 (54) of 57 episodes were reduced successfully using barium (42), saline (11) and air (4) hydrostatically. Among the 31 other episodes, 15 had ileo-colic intussusception, seven caeco-colic, six ileo-ileo-colic and one ileo-ileal. Two patients had spontaneous reduction discovered at surgery. There were two episodes of barium hydrostatic perforation of the colon leading to death in one patient. Hydrostatic reduction is recommended as the first therapeutic option for acute intussusception because it spares the patient a major operative procedure when successful.
Assuntos
Humanos , Masculino , Feminino , Lactente , Irrigação Terapêutica , Intussuscepção/terapia , Cloreto de Sódio/uso terapêutico , Doença Aguda , Enema , Estudos Prospectivos , Intussuscepção/etiologia , Pressão Hidrostática , Sulfato de Bário/uso terapêuticoRESUMO
The dumping syndrome in childhood is an uncommon complication of gastro-oesophageal surgery, principally Nissen fundoplication. A Jamaican child developed the syndrome after fundoplication and pyloroplasty to relieve gastro-oesophageal reflux complicating the repair of a congenital tracheo-oesophageal fistula. He developed marasmus and failed to gain weight on the standard remedial milk-based high-energy diet. An oral glucose tolerance test confirmed the diagnosis of dumping syndrome. A low sugar low milk diet based on adult type meals with continuous nibbling of fried dumplings relieved his diarrhoea and hypoglycaemia and he gained weight. This is a cheaper and more practical dietary therapy than the regimens described previously.
Assuntos
Síndrome de Esvaziamento Rápido/etiologia , Síndrome de Esvaziamento Rápido/dietoterapia , Síndrome de Esvaziamento Rápido/epidemiologia , Fundoplicatura/efeitos adversos , Humanos , Lactente , Jamaica/epidemiologia , Nefropatias/cirurgia , Antro Pilórico/cirurgiaRESUMO
Portal hypertension and bleeding from oesophageal varices in children remain a difficult medical problem. The clinical course and management of children with portal hypertension seen over a 14-year period was reviewed. There were 5 females and 2 males with a mean age of 3.6 years at presentation. Five patients presented with severe upper gastrointestinal bleeding and two with severe hypersplenism. All patients had extra-hepatic portal hypertension. Five patients were treated with endoscopic sclerotherapy, including one who had bleeding five years post-splenectomy. A mean of 9 sclerotherapy sessions was performed in each patient. Complete obliteration of varices was not achieved in any patient and a single rebleeding episode occurred in four. Three children underwent operative management consisting of splenectomy in two and splenectomy and central spleno-renal shunt in one. There was no mortality in either group after a mean follow-up of 4.3 years. Sclerotherapy may not be totally successful in long-term management of childhood portal hypertension. Surgical therapy or a combination of sclerotherapy and surgery may be the best approach.
Assuntos
Hipertensão Portal/epidemiologia , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Jamaica/epidemiologia , Estudos Longitudinais , Masculino , Estudos RetrospectivosRESUMO
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 increases 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.
Assuntos
Crescimento , Hiperesplenismo/cirurgia , Traço Falciforme/complicações , Estatura , Peso Corporal , Criança , Pré-Escolar , Índices de Eritrócitos , Feminino , Testes Hematológicos , Humanos , Hiperesplenismo/sangue , Hiperesplenismo/fisiopatologia , Lactente , Masculino , Traço Falciforme/sangue , Traço Falciforme/fisiopatologia , Traço Falciforme/cirurgia , EsplenectomiaRESUMO
Of the twenty-nine children with solid renal tumours treated at the University Hospital of the West Indies (UHWI) between January, 1972 and December, 1991, there were twenty-eight cases of nephroblastoma and one of mesoblastic nephroma. Peak incidence was between the ages of two and four years. Twenty-five children had radical nephrectomy while one had bilateral partial nephrectomy. In thirteen cases, pre-operative chemotherapy +/- radiotherapy was used. Post-operative chemotherapy and radiotherapy were used in 24 and 13 cases, respectively. Stage of the tumour was the most decisive factor influencing the outcome. Whereas there was a 100% cure rate in Stages I and II, Stage III had only a 55.5% survival rate and none of the Stage IV survived. Bilateral (Stage V) tumours are curable if individual tumours are localised, as in one of the two cases. The benign mesoblastic nephroma, in a one-month-old infant, was cured by nephrectomy alone. While the present therapy of radical nephrectomy along with combination chemotherapy is satisfactory for early stages, more aggressive adjuvant therapy is needed for improving the results in Stages III and IV.
Assuntos
Países em Desenvolvimento , Neoplasias Renais/mortalidade , Nefroma Mesoblástico/mortalidade , Tumor de Wilms/mortalidade , Criança , Pré-Escolar , Terapia Combinada , Feminino , Seguimentos , Humanos , Lactente , Jamaica/epidemiologia , Neoplasias Renais/patologia , Neoplasias Renais/terapia , Masculino , Estadiamento de Neoplasias , Nefroma Mesoblástico/patologia , Nefroma Mesoblástico/terapia , Taxa de Sobrevida , Tumor de Wilms/patologia , Tumor de Wilms/terapiaRESUMO
A retrospective analysis of 26 children with neuroblastic tumours treated at the University Hospital of the West Indies (UHWI) between 1970 and 1991 was undertaken to evaluate factors affecting prognosis. The peak incidence was between 48 months and 60 months of age, and 75% of the deaths occurred in children older than 3 years. The abdomen was the most common site of the tumours 79% of which were from the adrenal gland and carried a dismal outcome. Other sites were thoracic, pelvic and cervical. Extra-adrenal tumours have a good outcome even when the histology is unfavourable; 92% of the tumours were in Stages III or IV at the time of initial presentation. Stage IV disease accounted for all but one of the deaths. Of the 8 children with favourable histology (ganglioneuroma or well-differentiated ganglioneuroblastoma), only one (12.5%) died, wheras of the remaining 18 cases with unfavourable histology (neuroblastoma and undifferentiated ganglioneuroblastoma), eleven (61.1%) died. When managed by surgery alone or with adjuvant chemo- and/or radio-therapy, only 36.4% succumbed while all 4 children with chemotherapy only died. Ten children are alive without disease for more than three years post-therapy. A scoring system was designed which takes into account the factors influencing the outcome in neuroblastic tumours, namely, age, location, stage and histological types of the tumours and therapy. All the children with a score of 21 or less survived, whereas all those with scores of 22 or above succumbed. This underlines the multifactorial influences on the final outcome of neuroblastic tumours.
Assuntos
Neuroblastoma , Neoplasias Abdominais/mortalidade , Fatores Etários , Criança , Pré-Escolar , Análise Fatorial , Feminino , Humanos , Masculino , Neuroblastoma/mortalidade , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do TratamentoRESUMO
The Rapunzel Syndrome, a rare manifestation of trichobezoar, occurs when bolus gastrointestinal obstruction is produced by an unusual trichobezoar with a long tail that extends to or beyond the ileocaecal valve. A five-year-old Jamaican girl presented with this abnormality and was found at laparotomy also to have an ileo-ileal intussusception. For the Rapunzel Syndrome, we recommend bezoar extraction at laparotomy via multiple enterotomies. In addition, psychiatric evaluation and therapy is essential due to the commonly associated finding of underlying emotional stress.