Miquel Gussinyer

Cambios antropométricos, dietéticos y psicológicos tras la aplicación del programa «Niñ@s en movimiento» en la obesidad infantil

26.5 (3.6) kg/m 2 (p < 0.001) and from 3.3 (1,4) to 2.6 (1.2) kg/m 2 (p < 0.001). Diary fruit intake increased from 63.3% to 82.7% of patients (p < 0.001) and greens from 45.6% to 88,2% of patients (p < 0.001). Breakfast intake of industrial cakes decreased from 17.7% to 1.3% of patients (p < 0.001) and the number of patients who skipped breakfast changed from 36.7% to 11.7% (p < 0.001). Anxiety and depression scores fell from 53.46 (27.69) to 47.22 (26.3) (p < 0.03) and from 29.68 to 16.88 (p < 0.001), respectively. The relative risks of suffering a depressive or anxiety disorder dropped from 38.8% to 22.5% (p < 0.001) and from 15% to 8.2% (p = 0.01) of the patients respectively. CONCLUSIONS: Application of the «Nin@s en movimiento» programme leads to a decrease in BMI and in anxiety and depression scores and an increase in Mediterranean diet score.

Macroorchidism and Panhypopituitarism: Two Different Forms of Presentation of FSH-Secreting Pituitary Adenomas in Adolescence

Background: FSH-secreting pituitary adenomas are extremely rare in children and are seldom associated with clinical manifestations of high serum gonadotrophin levels. Thus, most patients have a late presentation, usually as macroadenomas. Case Reports: Two different clinical forms of presentation of FSH-secreting pituitary adenomas are reported: one in a 12-year-old boy with macroorchidism due to a pituitary microadenoma, probably FSH-secreting, and the other in a 15-year-old boy with panhypopituitarism due to an FSH-producing macroadenoma. Both patients presented slightly high or high FSH with low LH and high inhibin B levels. In the first case, the microadenoma was treated medically with cabergoline, which failed to reduce FSH and inhibin B levels. No radiological progression has been observed despite increasing testicular volume. In the second case, surgery was performed on the macroadenoma, leading to a decrease in FSH and inhibin B levels. The patient developed severe hypothalamic obesity and is currently under treatment with somatostatin. Conclusions: FSH-secreting pituitary tumors have an extremely variable clinical expression. The discrepancy between normal or slightly increased FSH and low LH values, together with high inhibin B levels, strongly suggests FSH hypersecretion which should be studied.

Trastornos de la conducta alimentaria en adolescentes jóvenes con diabetes mellitus tipo 1

Fundamento y objetivo: Determinar la prevalencia de trastornos de la conducta alimentaria (TCA) en pacientes con diabetes mellitus tipo 1. Sujetos y metodo:Se ha incluido a adolescentes de ambos sexos de 12 a 16 anos, 98 con diabetes mellitus tipo 1 y 575 sin ella (grupo control), a los que se aplico la version espanola validada del Eating Attitudes Test-40. Un grupo de sujetos fue posteriormente evaluado mediante la entrevista Eating Disorders Examination. Se registraron el peso, la talla, el indice de masa corporal, la hemoglobina glucosilada y la manipulacion de insulina como control de peso. Resultados: La prevalencia de TCA no especificados fue mayor en adolescentes diabeticos (en varones, el 1,7 frente al 0,9%; odds ratio [OR] = 1,7; intervalo de confianza [IC] del 95%, 0,2-17,6; en mujeres, el 5,3 frente al 1,6%; OR = 3,2; IC del 95%, 0,6-17,2). En varones diabeticos los TCA subclinicos fueron mas frecuentes que en los no diabeticos (el 10% frente al 4,4%; OR = 2,4; IC del 95%, 0,9-6,6), aunque sin diferencia estadisticamente significativa. Los valores de hemoglobina glucosilada fueron significativamente mayores (p = 0,49) en adolescentes diabeticos con TCA no especificados y TCA subclinicos (9,7% [1,5%] y 5,6 [2,8], respectivamente; n = 13) que en aquellos sin trastornos (8,4% [1,5%] y 5,1 [2,7]; n = 85). Nueve pacientes (9,1%) manipulaban dosis de insulina para controlar el peso. Conclusiones: La prevalencia de TCA no especificados en adolescentes diabeticos de ambos sexos fue mayor que en los adolescentes no diabeticos. La prevalencia de TCA subclinicos fue mayor en varones con diabetes mellitus tipo 1 que los no diabeticos.

Glycogen Storage Disease Type III with Hypoketosis

ABSTRACT A rare case of glycogen storage disease type III with unusually absent ketone body production during hypoglycemia is presented. A 10-month-old boy presented with asymptomatic hepatomegaly. GOT/GPT 2555/ 1160 IU/L, CK 302 IU/L, triglycerides 1223 mg/dL, cholesterol 702 mg/dL and uric acid 7.9 mg/ dL. After a 9-hour fast, glucose was 27 mg/dL and adequate lipolysis without ketogenesis was observed (total/free carnitine 34.5/20 μmol/L, free fatty acids 1620 μmol/L and β-hydroxybutyrate 172 μmol/L). Result of MCT (medium-chain triglycerides) load test: basal β-hydroxybutyrate 29 μmol/L rose to 5748 μmol/L. Treatment with a fat-restricted diet supplemented with formula containing MCT was initiated and the patient presented a satisfactory initial evolution. Three months later, CK were 3000 IU/L. Muscle biopsy was diagnostic of glycogenosis. Enzymatic activity in skin fibroblasts was 0% for amylo-1,6-glucosidase. The diagnosis of glycogenosis type III was established. Echocardiography performed at that time showed non-obstructive ventricular hypertrophy. Until now hypoketosis during hypoglycemia has only been described in glycogenosis type I.