Results of growth trophic therapy in children with short bowel syndrome.

Purpose: In addition to the structural, ultrastructural, and functional changes that occur after extensive enterectomy or in utero bowel loss that results in short bowel syndrome (SBS), a complex array of humoral responses take place that may also affect adaptation of the remaining small intestine as well as nutritional status or growth. These include alterations in the levels of circulating hormones and trophic substances such as growth hormone (GH) and insulinlike growth factors (IGF-1 and IGFBP-3). The purpose of this investigation is to report on the management/treatment of 3 children with SBS (>4 years in duration) and growth failure.

Methods: Serum measures of growth factors and the response to GH stimulation after an arginine insulin tolerance test (AITT) were determined. Weight and height z-scores as well as linear growth velocity were calculated annually pre- and postinitiation of medication therapy.

Results: Patient 1 (boy, 8.5 years old, midgut volvulus, 18-cm bowel) was found to be GH deficient, whereas patients 2 (girl, 12.5 years old, gastroschisis, 70-cm bowel) and 3 (boy, 9 years old, jejunal atresia, 21 cm bowel) were found to have limited GH responsiveness. Subsequently, treatment with GH (1) and growth releasing factor (GRF; 2 & 3) was prescribed. Z-scores for both weight and height improved over time. Positive linear growth velocity was observed from initiation of therapy (<0.5 cm/yr for all) to more than 3 years of treatment (mean 1, 4.7 cm/yr; 2, 8.7 cm/yr; 3, 5.0 cm/yr [age adjusted normals >4.5, >8.5, and >4.9 cm/yr, respectively]). All patients received a regular diet with oral supplements, whereas 2 received parenteral nutrition support for about 1 year.

Conclusions: In children with medically refractory SBS, it is not only important to offer trophic factors but also essential that sufficient nutrient substrate be provided to achieve adequate growth.