RESUMEN
INTRODUCTION: SBS is a complex entity with high morbimortality. Survival following extent intestinal resection during the neonatal period is higher than 90%. Nutritional support is paramount during the periods of high growth. OBJECTIVE: To assess growth of children with neonatal SBS neonatal having received during the first two years of life. MATERIAL AND METHODS: A retrospective study was done on patients assisted at the Nutrition Department of the Children's Hospital of La Plata. The following parameters were gathered: gestational age (GA), diagnosis, length of the remnant bowel, liver impairment, duration of PN and EN, and socio-economical profile of the patients. Growth assessment was done by: weight (W), height (H) at two years (Control I), and at the last follow-up visit done at the Department (Control II), using the NCHS tables. Weight and height were expressed as the Z score (Z) and the percentage of W/H, and were related to need of long-term NS. Patients were categorized into three groups according to NS requirement at the time of Control II: PN-dependent, EN-dependent, and those not requiring nutritional support (WOS). RESULTS: Eighteen patients were included. Length of the remnant bowel was: 45 cm. (r 10-80 cm.), length was < 40 cm in 11 pts. (61%) (massive resection). The ileocecal valve was resected in 9 pts. (50%) and 3 pts. (16 %) required partial colon resection and 2 pts. (11%) total resection. Mean Z scores for W/A, H/A, %W/H, Z W/H yielded values < -2 SD, and the percentage of W/H appropriateness was > 90% in both controls. Twenty seven percent of the patients at two years of age, and 33% at the last control showed height < -2 SD. Two patients (11%) died while on PN from liver failure, both with 10 cm of remnant bowel and without VIC. At Control II, 4 patients (22%) remained PN-dependent. Mean length of the remnant bowel in this group was 33 cm (r: 17-50) and mean follow-up duration with PN was 2176 days (r: 750-4380). Six patients (33%) remained EN-dependent at Control II, with a mean intestinal length of 41 cm (r: 20-75) and 2 out of 6 pts. did not have VIC. This group of patients required PN as the initial therapy with a mean duration of 629 days. Follow-up time while on EN was 627 days (r: 210-3010). Six patients (33%) achieved nutritional support independence (WOS) with a mean intestinal length of 60 cm (r: 27-80) after a mean duration of NS of 791 days, being assisted with dietary recommendations and vitamins and minerals supplementation. The group showing the greatest growth impairment was the EN-dependent group since we consider that early withdrawal of PN was decided based on inappropriate socio-environmental conditions. CONCLUSIONS: According to the present study, we conclude that is may be possible to achieve a normal growth in children with neonatal SBS under nutritional support and that complications related to this nutritional therapy or difficulties for adequately implementing it at home may affect the final height.
Asunto(s)
Trastornos del Crecimiento/etiología , Complicaciones Posoperatorias/etiología , Síndrome del Intestino Corto/complicaciones , Argentina/epidemiología , Comorbilidad , Nutrición Enteral , Femenino , Estudios de Seguimiento , Enfermedades Gastrointestinales/congénito , Enfermedades Gastrointestinales/cirugía , Edad Gestacional , Trastornos del Crecimiento/epidemiología , Humanos , Recién Nacido , Ictericia Neonatal/epidemiología , Hígado/fisiopatología , Masculino , Estado Nutricional , Apoyo Nutricional , Nutrición Parenteral , Complicaciones Posoperatorias/epidemiología , Estudios Retrospectivos , Síndrome del Intestino Corto/epidemiología , Síndrome del Intestino Corto/terapia , Factores SocioeconómicosRESUMEN
La linfangiectasia intestinal congénita, enteropatía perdedora de proteínas de aparición esporádica que afecta fundamentalmente a niños y adultos jóvenes, se inicia por lo general con edemas y diarreas. Mostramos el caso de un lactante que presentó ascitis en ausencia de edemas, así como los hallazgos clínicos, de laboratorio e histológicos y la respuesta al tratamiento con triglicéridos de cadena media(AU)