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Abnormal glucose tolerance and diabetes mellitus are a common complication of cystic fibrosis (CF). Cystic fibrosis related diabetes (CFRD) is due to decreased insulin secretion, secondary to pancreatic insufficiency. Patients with CFRD have increased morbidity and mortality and are subject to the same microvascular complications as patients with other types of diabetes. Prompt diagnosis and aggressive management of CFRD is important.A consensus conference on CFRD was held in Madrid in May 2000to define the current standards for the diagnosis and management of this disease in Spain.

To evaluate the effect of bone marrow transplantation during childhood on gonadal function in postpubertal patients.We studied 19 post-pubertal patients (13 males, 6 females) aged 13-20 years, 2-9 years after bone marrow transplantation for diverse diseases. Fifteen patients had received multi-agent cytotoxic treatment and 6 had received cranial irradiation prior to transplantation; eleven patients received total body irradiation. Gonadal function was assessed by typifying Tanner's stages of pubertal development and by measuring testicular volume in males (Prader's orchidometer). Serum concentrations of luteinizing hormone, follicle-stimulating hormone, testosterone and 17-beta-estradiol were also measured.Twelve patients were found to have gonadal dysfunction of gonadal origin. Of these, three had not received total body irradiation.The results obtained show that gonadal dysfunction is frequent in patients treated with bone marrow transplantation. This damage can be attributed to both chemo- and radiotherapy but a synergistic effect between these treatments could not be excluded. The prepubertal status of patients at the moment of transplantation was not a protective factor against chemo- or radiotherapy-induced gonadal damage in our series.

Authors describe one case of Cushing's disease in a old eight-year boy, with growth deficiency, pubic hair and obesity. He had hypercortisolism unresponsive to dexamethasone suppression (1 mg). The more interesting fact for localization of the lesion was the more than 50% suppression with 8 mg of dexamethasone, while tomographic studies of sella turcicaland CAT were normal. A 3 mm microadenoma was removed at transsphenoidal surgery. After surgery the patient had diabetes insipida and adrenal insufficiency. One year later all endocrinologic studies were normal. This fact underlines the importance that transsphenoidal surgery can have in the treatment of Cushing's disease.

Authors describe clinic and therapeutic results in seven hyperthyroid Graves' disease patients between ages of three and a half an 15 and a half (x: 11.2) years relating their results with those of literature. Most peculiar aspects of these cases were pretibial myxedema in two patients and lack of goiter in one of them. Results include T4 greater than 18.5 mcg/100 ml in five, T3 greater than 645 ng/100 in three and a relationship between T3/T4 greater than or equal to 20 in five. These results have been evaluated as signs of a bad prediction. After a two years treatment with antithyroid drugs remission was obtained in five cases, two of which relapsed again (40%). Authors recommend as initial treatment anti-thyroids drugs in high dosages with an individual lasting period of treatment using specific markers of remission (antibodies directed the receptor for TSH, triiodothyronine suppression test, relationship okt4/okt8).

Descent of the testes is essential for normal function, with cryptorchidism being associated with defective spermatogenesis. The postpubertal function of cryptorchid testicles seems to be related to the original location of the undescended testis and the age at the moment of treatment. The objective of this study was to evaluate the pubertal development and gonadal function in postpubertal patients treated for cryptorchidism during childhood.Twenty postpubertal males with a mean age of 17.35 years (range: 15-21 years) and treated for cryptorchidism during childhood were evaluated for pubertal development and gonadal function. A hormonal study which included basal determinations of testosterone, luteinizing hormone (LH) and follicle-stimulating hormone (FSH), testicular volume assessed by Prader's orchidometer and semen analysis was performed on each patient.Complete virilization was observed in all patients. The start and development of puberty were normal in all cases (except one patient that started puberty at 10 years of age). Basal studies in all patients showed normal levels of LH and testosterone. FSH levels were increased in 3 patients and normal in the other 17 patients. Fourteen patients achieved normal spermatogenesis with more than 20 million spermatozoa/ml. In the other 7 patients (35%), 5 with unilateral cryptorchidism and 2 with bilateral cryptorchidism, the sperm count remained below 20 million with a range of 0.8 to 18.4 x 10(6) spermatozoa/ml. The three males with elevated levels of FSH also presented oligospermia.The results obtained show that pubertal development is normal after cryptorchidism. Impaired spermatogenesis is a major factor in undescended testes. Basal SH levels can be useful in predicting germinal damage secondary to cryptorchidism.

Girls diagnosed of type 1 diabetes mellitus during the prepubertal stage may present reduced longitudinal growth and have a tendency to be overweight after puberty starts. Our objective was to evaluate the growth, pubertal growth spurt, final height and weight gain of diabetic girls diagnosed of insulin dependent diabetes during the prepubertal period.We evaluated retrospectively the longitudinal growth and body mass index (BMI) of 17 girls diagnosed of type 1 diabetes before puberty, from the time of diagnosis until final height. The effects of age at diagnosis, evolution time, insulin therapy (conventional or intensified), insulin dose and metabolic control on growth were analyzed.The final height attained was 161 +/- 4 cm, which is within the target height range. The height at diagnosis (0.31 +/- 0.71) was normal in relationship to the reference population, showing a significant reduction upon reaching the final height (-0.18 +/- 0.77). The BMI was significantly increased when it was compared at final height (0.99 +/- 0.79) with the index obtained one year after the onset of diabetes (0.04 +/- 0.63). The insulin therapy regimen did not affect the evolution of the patients and no significant relationship existed between the age at diagnosis, duration of diabetes, daily insulin dosage or metabolic control and height or BMI.The final height of type 1 diabetic girls is reduced and adolescent diabetic girls have a tendency to be overweight. Metabolic control (for the range observed in these patients) or insulin dosage does not seem to affect the growth of diabetic girls.

We report the cases of 6 patients, all younger than 14 years of age, with differentiated thyroid carcinoma. None of the patients had a previous history of radiation exposure. All patients presented with an enlarged thyroid gland as a solitary nodule, with or without cervical nodes. The fine-needle aspiration cytological examination was found to be the most sensitive and specific, evidencing 5 pure papillary adenocarcinoma and 1 follicular. Our standard preoperative evaluation included thyroid scintiscan and ultrasound examination, laboratory studies of thyroid function and serum calcitonin, chest x-ray, fine needle aspiration and vocal cord examination. The treatment was total thyroidectomy and bilateral modified neck dissection. A whole body scan (WBS) with 131I was performed 6 weeks after surgery, followed by radioiodine therapy for ablation of thyroid remnants and treatment of metastases when present. Patients then began thyroid replacement treatment. The follow-up of the patients consisted of thyroglobulin and WBS. The microscopic carcinoma was found in the contralateral lobe in 100%. Lung metastases were detected in 2 patients. All of the patients have survived during a follow-up period ranging from 6 to 108 months.