RESUMO
OBJECTIVE: To determine the frequency with which inflammatory bowel disease (IBD) is diagnosed in persons with Hirschsprung disease in population-based datasets from 3 Canadian provinces. STUDY DESIGN: In study I, Ontario data were used to assess the incidence of IBD in a birth cohort of children with Hirschsprung disease relative to children without Hirschsprung disease. In study II, a case-control design was used in Alberta and Manitoba to determine the frequency of previously diagnosed Hirschsprung disease in persons with IBD, compared with the frequency of Hirschsprung disease in matched controls. Validated algorithms for Hirschsprung disease and IBD were applied to each provincial health registry. RESULTS: In study I, of the 716 children diagnosed with Hirschsprung disease in Ontario since 1991, 18 (2.5%) ultimately developed IBD (168.8 per 100 000 person-years), compared with 7109 of 3 377 394 children without Hirschsprung disease (0.2%, 14.2 per 100 000 person-years). The percentage of males with post-Hirschsprung disease IBD was 77.8%. The incidence rate ratio was 11.9 (95% CI, 7.5-18.8). In study II, the OR of having had Hirschsprung disease before a diagnosis of IBD compared with controls was 74.9 (95% CI, 17.1-328.7) in Alberta and 23.8 (95% CI, 4.6-123) in Manitoba. Crohn's disease was more common after Hirschsprung disease than ulcerative colitis. CONCLUSIONS: IBD can emerge in more than 2% of patients with Hirschsprung disease and, like Hirschsprung disease itself, is more common in males. IBD is much more common after a diagnosis of Hirschsprung disease than in the general population.
Assuntos
Doença de Hirschsprung/epidemiologia , Doenças Inflamatórias Intestinais/epidemiologia , Adolescente , Canadá/epidemiologia , Estudos de Casos e Controles , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Distribuição por SexoRESUMO
OBJECTIVE: To characterize the natural history of intestinal failure (IF) among 14 pediatric centers during the intestinal transplantation era. STUDY DESIGN: The Pediatric Intestinal Failure Consortium performed a retrospective analysis of clinical and outcome data for a multicenter cohort of infants with IF. Entry criteria included infants <12 months receiving parenteral nutrition (PN) for >60 continuous days. Enteral autonomy was defined as discontinuation of PN for >3 consecutive months. Values are presented as median (25th, 75th percentiles) or as number (%). RESULTS: 272 infants with a gestational age of 34 weeks (30, 36) and birth weight of 2.1 kg (1.2, 2.7) were followed for 25.7 months (11.2, 40.9). Residual small bowel length in 144 patients was 41 cm (25.0, 65.5). Diagnoses were necrotizing enterocolitis (71, 26%), gastroschisis (44, 16%), atresia (27, 10%), volvulus (24, 9%), combinations of these diagnoses (46, 17%), aganglionosis (11, 4%), and other single or multiple diagnoses (48, 18%). Prescribed medications included oral antibiotics (207, 76%), H2 blockers (187, 69%), and proton pump inhibitors (156, 57%). Enteral feeding approaches varied among centers; 19% of the cohort received human milk. The cohort experienced 8.9 new catheter-related blood stream infections per 1000 catheter days. The cumulative incidences for enteral autonomy, death, and intestinal transplantation were 47%, 27%, and 26%, respectively. Enteral autonomy continued into the fifth year after study entry. CONCLUSIONS: Children with IF endure significant mortality and morbidity. Enteral autonomy may require years to achieve. Improved medical, nutritional, and surgical management may reduce time on PN, mortality, and need for transplantation.
Assuntos
Enteropatias/epidemiologia , Enteropatias/terapia , Enterocolite Necrosante/epidemiologia , Feminino , Gastrosquise/epidemiologia , Doença de Hirschsprung/epidemiologia , Humanos , Lactente , Atresia Intestinal/epidemiologia , Enteropatias/mortalidade , Enteropatias/cirurgia , Volvo Intestinal/epidemiologia , Intestinos/transplante , Masculino , Nutrição Parenteral , Prognóstico , Estudos RetrospectivosRESUMO
Estudo retrospectivo realizado entre janeiro de 1986 e junho de 1998 no Hospital Universitário da Universidade Federal de Sergipe com o objetivo de analisar os dados epidemiológicos, quadro clínico, meios diagnósticos, opçöes terapêuticas e índice de morbimortalidade de pacientes portadores da Doença de Hirschsprung que foram submetidos a tratamento cirúrgico. Foram avaliados 29 pacientes com predomínio do sexo masculino(80 por cento) e incidência maior em pacientes abaixo de um ano de idade (72,5 por cento). As manifestaçöes clínicas mais freqüentes foram a distensäo abdominal (48,3 por cento) seguida da obstruçäo intestinal (38 por cento), constipaçäo (34,5 por cento), toque retal com fezes explosivas (31 por cento) e vômitos biliosos (31 por cento). Os exames complementares mais utilizados foram o enema opaco (58,6 por cento), seguido da radiografia simples de abdome (48,3 por cento) e da biópsia de cólon (45 por cento). O procedimento cirúrgico definitivo mais executado foi a cirurgia de Duhamel modificada (44,5 por cento). Em 27,6 por cento dos pacientes houve relato de algum tipo de complicaçäo pós-operatória. A infecçäo de ferida operatória foi a complicaçäo mais freqüente nos pacientes submetidos à cirurgia definitiva (37,5 por cento). O procedimento cirúrgico que apresentou maior índice de morbidade foi a cirurgia de Duhamel Haddad (67 por cento). Ocorreram 2 óbitos, ambos em pacientes com enterocolite que foram colostomizados. A morbidade do procedimento cirúrgico definitivo foi zero. De acordo com os dados encontrados, a cirurgia de Duhamel mostrou-se vantajosa e eficaz no tratamento da Doença de Hirschsprung. O índice de morbidade da cirurgia definitiva foi alto, porém, foram complicaçöes de fácil correçäo e sem mortalidade
Assuntos
Humanos , Doença de Hirschsprung/cirurgia , Doença de Hirschsprung/diagnóstico , Doença de Hirschsprung/epidemiologia , Megacolo/cirurgia , Estudos Retrospectivos , Procedimentos Cirúrgicos OperatóriosRESUMO
Se estudiaron 29 casos clínicos de megacolon congénito en Nariño en un período de 1984 a 1988 en cinco instituciones médicas, encontrándose una incidencia de 1-5.000 nacidos vivos, con una relación de 4:1 con predominio del sexo masculino, sin asociaciones con anomalías congénitas, un compromiso predominante de segmentos ultracorto y corto de 96.5 por ciento; se hizo el diagnóstico con base en la clínica colon por enema y biopsia clásica; para segmento ultracorto se empleó como técnica de elección la rectomiectomía Lynn y para segmento corto y largo la técnica de Soave y de Ikeda; obteniéndose mejores resultados y una marcada disminución de complicaciones con el último método
Assuntos
Humanos , Doença de Hirschsprung , Doença de Hirschsprung/diagnóstico , Doença de Hirschsprung/epidemiologia , Doença de Hirschsprung/terapia , IncidênciaRESUMO
Four decades ago, the mortality of congenital aganglionosis of the distal part of the colon (Hirschsprung's disease) approached 100 per cent. The development of diagnostic methods and curative operations lowered the mortality to 5 to 10 per cent. Enterocolitis remained the major cause of death. In this series of 18 children with Hirschsprung's disease, the majority were diagnosed as neonates. Although enterocolitis was present at diagnosis in four (22 per cent), all children survived after prompt surgical therapy. Neonatal diagnosis and treatment may prevent the development of fatal enterocolitis, virtually eliminating the mortality of Hirschsprung's disease.
Assuntos
Doença de Hirschsprung/diagnóstico , Sulfato de Bário , Biópsia , Colectomia , Colostomia , Diagnóstico Diferencial , Feminino , Doença de Hirschsprung/epidemiologia , Doença de Hirschsprung/cirurgia , Humanos , Lactente , Recém-Nascido , Masculino , New Mexico , Reto/patologia , Fatores de TempoRESUMO
From 1958 to 1979, 55 children with Hirschsprung's disease have been treated at the University Hospital of the West Indies. Delay in passage of meconium, abdominal distension, vomiting and constipation, were the main symptoms. Bowel obstruction (28) and chronic constipation (27) were the common modes of presentation. Although symptoms had their onset within the first six months in 43 children, the diagnosis was established before six months in only 18. Barium Enema failed to demonstrate a narrow segment in 21 cases often leading to delay in establishing the correct diagnosis. It was also misleading in the assessment of the extent of the aganglionosis, only one out of eight long segment types being identified radiologically. Rectal biopsy by punch technique was successfully employed in 41 cases to establish the diagnosis. Forty-four children had pull-through operations (31 Duhamel, 10 Modified Soave, 3 Swenson). Four had posterior excisional anorectal myectomies, while three are awaiting definitive surgery following colostomy. Eleven of the definitive procedures were done in one stage while six were done in two stages and 27 in three stages. Stenosis (10) cases pelvic abscesses (7) persistent septa (6) and cuff revision of pull-through. There were eight deaths of which three were before surgery, one after colostomy and four after definitive surgery. Three of these four post pull-through deaths were in one stage procedures. Thirty children have been followed up for periods of one year to ten years. Seventeen have excellent results with normal continence, growth and development. Eleven have fair results with five of them requiring laxatives and six having soiling. Two have poor results. Of the seven children followed up for more than five years after surgery, six have excellent results. With early diagnosis and adequate staged definitive surgery, the long term results for Hirschsprung's disease are excellent (AU)