ESPE Abstracts

Malnutrition, including that caused by malabsorption, is the most common cause of growth failure in children. On the other hand, in children who receive total parenteral nutrition due to extremely severe forms of malabsorption disorders, which involves almost continuous intravenous infusion of nutrients (including glucose), a persistent, functional reduction in growth hormone secretion occurs. A female patient with extreme short bowel syndrome, fed almost entirely parenterally, was found to have severe growth deficiency (-5.6SDS) with bone age delay (at 3 years and 8 months, bone age was 1 year and 8 months). The girl was born via cesarean section at 35 weeks of gestation, with birth weight 2700 g, length 48 cm, head circumference of 32 cm, and an APGAR score 10 points. Due to congenital evisceration, the child underwent extensive intestinal resection, intestinal lengthening using the STEP method, and two bowel plication, resulting in short bowel syndrome, intestinal dysfunction due to impaired intestinal transit and backlogging of intestinal contents in significantly dilated intestinal loops, and chronic dysbacteriosis of the intestinal flora. Despite an optimal caloric balance, achieved with parenteral nutrition provided since birth, the growth deficit steadily increased. The thyroid and adrenal dysfunction were excluded, the karyotype was normal female, MRI examination did not reveal any pituitary abnormalities. The IGF -1 level was low (41.1 ng/mL; N: 42-276 for chronological age, N: 45-361 for bone age). The maximum concentration of growth hormone obtained by stimulation tests was 5.57 ng/mL, which led to the diagnosis of isolated inorganic growth hormone deficiency. At the age of 4 years and 11 months, the patient was qualified for treatment with human recombinant growth hormone. At the same time, a marginal ileostomy with the placement of a continuous bowel drain was performed, which improved gastrointestinal motility, reduced the backlog of intestinal contents and improved function (improved tolerance of trophic oral feeding). In the first year of treatment growth velocity reached 12.6 cm/year, and the growth deficit decreased to (-)3.55SDS; the bone age was 3 years and 6 months. No negative and adverse effects of therapy were observed in the patient during treatment.

Conclusion: Growth disorders in children with extreme short bowel syndrome are common despite optimal nutritional treatment. In such cases, it is worth considering the possibility of growth hormone deficiency as a consequence of nutritional treatment, including continuous intravenous glucose infusion. Substitution with human recombinant growth hormone may be an effective treatment.