Deficiencia somatotrófica en niños: características clínicas y bioquímicas según la etiología en 75 casos / Somatotrophic deficiency in children: report of 75 cases

Somatotrophic deficiency (SDMT) can be due to a deficiency of growth hormone releasing hormone(GHRH), growth hormone (GH) or insulin like growth factor I (IGF-I). Although its clinical features have been thoroughly described, the diagnosis is still controversial. Now there is an effective treatment with GH or IGF-I for these patients. AIM: To analyze the main clinical, etiological and laboratory characteristics of 75 SD patients (44 males), aged 9.4 + 4.5 years, with severe growth retardation. The diagnosis was confirmed by the lack of response to two GH stimulation tests (Clonidine, Glugagon or Insulin) and low levels of IGF-I or insulin-like growth factor binding protein- 3 (IGFBP-3). RESULTS: In 34 patients (46 percent), the cause of DSMT was considered idiopathic (DSMT-I), in 31 (41 percent) there was an organic cause (DSMT-O), most commonly caused by malformations or pituitary tumors and in 10 (13 percent), it was genetic (DSMT-G) (three patients with Laron's Syndrome, five with mutations of GH gene and 2 with probable mutations of Prop-1 and Pit-1 genes). IGF-1 levels, were significantly lower in DSMT-O and DSMT-G thanin DSMT-I (21.2 +/- 46.1, 23.4 +/-30.3 ng/mL and 50.2 +/- 48.3 ng/mL, respectively). The lowest height score corresponded to DSMT-G, compared to DSMT-O and DSMT (­5.7 +/- 0.9, -4.0 +/- 1.6 and ­4.3 +/- 1.2 DS, respectively) CONCLUSIONS: The high percentage of organic and genetic etiologies in our patients can be due to the systematic search of these diseases. DSMT-G (Laron, mutations in GH and Pit-1 genes) had the most severe growth retardation.

Resultados de un programa multidisciplinario de tratamiento intensificado de la diabetes mellitus tipo 1 (DM1) en un Hospital Público / Results of a multidisciplinary and intensified treatment program for type 1 Diabetes Mellitus in a Chilean Public Hospital

BACKGROUND: During the last decade, the importance of glycemic control in the prevention of the microvascular complications of type 1 Diabetes Mellitus (DM1) was clearly demonstrated. AIM: To evaluate the metabolic and anthropometric results of a multidisciplinary intensified treatment program of DMI in children and adolescents. PATIENTS AND METHODS: Report of 54 patients treated during 2001. The intensified treatment consisted of: multiple daily doses of insulin, frequent glycemic control, nutritional, psychological and educational support, and permanent availability of a diabetes nurse for telephonic support. RESULTS: Thirty one patients were female, their mean age was 10.4 +/- 0.5 years old and 52 per cent were experiencing puberty. Fifty three percent of the patients used 3 insulin doses per day, 95 per cent changed rapid insulin dose based on glucose levels and 18 per cent considered carbohydrates in their rapid insulin dosing. Mean glycosilated hemoglobin was 8.18 +/- 0.23 per cent without differences by sex or pubertal status. Sex, pubertal stage and the number of insulin doses did not contribute to glycosilated hemoglobin changes. There were no differences in weight or BMI, but there was a decrease in height Z score from the admission to the program until the last control (0.1 +/- 0.1 vs--0.3 +/- 0.1 DS; p < 0.01). CONCLUSIONS: A modified intensified modality of DM1 therapy for pediatric patients in a public hospital in Chile is feasible, achieving similar metabolic control, compared to international large centers.