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Introduction: The use of continuous subcutaneous insulin infusion therapy in type 1 diabetes mellitus has increased due to its benefits on glycemic control and on the lifestyle flexibility. The aim of this study was to assess the impact of continuous subcutaneous insulin infusion therapy on glycemic control, body mass index, total daily dose of insulin and complications associated with this therapy, during 20 years of experience in Centro Hospitalar e Universitário de Coimbra., Material and Methods: This retrospective study included patients with type 1 diabetes mellitus who started continuous subcutaneous insulin infusion therapy up until 2005, followed at Centro Hospitalar e Universitário de Coimbra. Glycated hemoglobin A1c, body mass index, total daily dose of insulin and acute complications associated with continuous subcutaneous insulin infusion therapy were evaluated immediately prior to initiation of continuous subcutaneous insulin infusion therapy with follow-up at six months, one year, five, 10, 15 and 20 years. The frequency of acute complications associated with this type of therapy was also evaluated., Results: This study included 20 patients (seven males, 13 females) with mean disease duration up to the start of continuous subcutaneous insulin infusion therapy of 16.1 ± 7.9 years, mean age of onset of continuous subcutaneous insulin infusion therapy of 31.1 ± 8.4 years and follow-up during 13.2 ± 2.3 years. The reasons for initiating pump therapy were: inadequate metabolic control in 15 patients, history of asymptomatic or severe hypoglycemia in four patients, and pregnancy/pregnancy planning in one patient. The previous median of glycated hemoglobin A1c was 9.3% (6.5 - 16.0) and, at six months, decreased to the minimum value of 7.2% (5.3 - 9.8); p < 0.0125. The reduction of glycated hemoglobin A1c remained statistically significant in the first 10 years of follow-up. There was a statistically significant difference in the body mass index variation at 10 years with continuous subcutaneous insulin infusion therapy compared to previous body mass index; 24.7 kg/m2 (18.9 - 31.8) vs 25,5 kg/m2 (18.9 - 38.9), p <0.0125. Daily insulin requirements were reduced from 56.5 U (32.0 - 94.0) to 43.8 U (33.0 - 64.0) (p < 0.0125) at six months and no statistical differences were found in the remaining follow-up. There were two severe episodes of hypoglycemia (incidence 0.0095/patient/year), five episodes of diabetic ketoacidosis (0.0238/patient/year) and no infections at the site of catheter insertion., Discussion: This study shows that continuous subcutaneous insulin infusion therapy improved glycemic control, especially during the first 10 years of follow-up and allowed a significant decrease in total daily dose of insulin in the first six months. The rate of acute complications was low., Conclusion: Treatment with continuous subcutaneous insulin infusion therapy seems effective in achieving metabolic control in selected patients with type 1 diabetes mellitus.

Introduction: An early investigation and management op cryptorchidism are essential for the diagnosis of associated pathologies, because intra-abdominal testis are associated with infertility and neoplasic risk. The authors describe a case of late presentation of cryptorchidism associated with genetic disorder., Case Report: Adolescent male with 17 years old, referred to Adolescent Clinic for short stature and bilateral cryptorchidism. Hypertension, short stature and bilateral cryptorchidism were detected in clinical evaluation. Bone age is coincident with chronological age and testosterone was slightly below normal values. The study of secondary hypertension was negative. He underwent bilateral orchidopexy, and histological analysis revealed severe bilateral testicular atrophy. The karyotype showed 45,X[15]/46,X,+mar.ish der(Y)(SRY+)[117] mosaicism., Comments: With this case report the authors intend to enhance the need of early management of cryptorchidism and short stature, in order to improve the prognosis.

The deficiency of the enzyme 21-hydroxylase (21-HO) is responsible for about 90% to 95% of all cases of congenital adrenal hyperplasia (HCSR). This disorder is one of the most frequent hereditary illnesses of autosomal recessive trait. The illness can be presented in two clinical forms: 1 - classic, subdivided in saltwasting form and simple virilizing form; 2 - nonclassic or late-onset. The severity of the illness is correlated with the degree of enzymatic activity of 21-HO, which depends on the type of mutation that occurs in gene CYP21A2. The late onset congenital adrenal hyperplasia is that one where the enzymatic blockade is less intense. The clinical is variable: precocious pubarche, polycystic ovaries, hirsutism, oligoamenorrhea, acne and infertility. Gold standard for the diagnosis of late onset congenital adrenal hyperplasia consists on the test of the tetracosactide, considering itself diagnostic positive when 17-hidroxiprogesterona (17-OHP) is higher of 10-15 ng per mL. Many patients don't need treatment; however, if necessary, such a treatment essentially consists on the Administration of glucocorticoids and antiandrogens. Considering that affected individuals and the carriers may have a severe mutation in CYP21A2, they can have descendents with the classic form (if their partner is also a carrier of a severe mutation), it is mandatory the genetic study of the couple. The authors illustrate the importance of this genetic study through five clinical cases, whose common link is the desire to have children, as well as the presence on the feminine element of late onset congenital adrenal hyperplasia. The genetic study of the couple allows adequate pre-conception counseling and also prevents the use of corticoids throughout the pregnancy (if there's no risk of descendents being affected with the classic form). This aspect must be had in account in programming the pregnancy, in order to prevent therapeutics and unnecessary distrusts.

Aim: To evaluate the impact of the aging of our population in the growing of diabetes prevalence, among patients treated inward at our department., Material and Methods: The clinical files of 242 diabetics older than eighty years, treated between 1999 and 2002 were studied retrospectively. Demographic (sex, age, year of first contact), clinical (causes of admission, co-morbidities, duration of diabetes, previous hypoglycaemic treatment, duration of hospitalisation and evolution) and biochemical (glycaemia, HbA1c, lipaemia and C-peptide) data were recorded., Results: A progressive rising in the number of aged patients was found during this period (seven in 1999 to 55 in 2002), with a large proportion of women (73%). The more frequent causes of in-hospital treatment were hyperglycaemia (20.6%) and feet ulcers (septic or necrotic) (16.5%). In nearly 42% of the cohort an acute infection was found. Diabetes was unknown in 13% of cases and less than ten years of duration in 38%. In this cohort most of the patients (77%) were treated with oral hypoglycaemic drugs, mostly sulfonylureas. HbA1c mean value was 10.4 +/- 2.2%; C -peptide was measured in a subset of cases (n=79), suggesting deficient insulin secretion in 18% of them. The global mean duration of hospitalisation was eleven days. Nineteen patients (8%) died during the hospitalization., Conclusions: In this study, we concluded that diabetes in people older than 75 years, is being progressively more often diagnosed. The general metabolic control was bad (HbA1c mean value--10.4 +/- 2.2%), with a significant number of patients presenting a deficient C-peptide secretion, worsened by frequent coexisting infections. So, a strengthened clinical care, directed to a careful diagnosis and treatment, may effectively contribute to a better prognosis and quality of life of aged diabetic patients.

Aim: To evaluate the characteristics and outcomes of the patients diagnosed as ACTH-dependent Cushing syndrome, registered in the department., Material and Methods: We reviewed the files of forty-three patients followed from 1974 to 2002., Results: A progressive rise in the number of patients diagnosed was found, being 80% females. Clinical suspicion was based mostly on the typical fat distribution; hirsutism and amenorrhoea were important in women. The more reliable diagnostic tests were: 11 pm cortisol, day curve of ACTH and cortisol, and dexamethasone suppression tests. The ACTH response to CRH during inferior petrosal sinus sampling permitted the diagnosis of ectopic source. In thirty-seven patients a pituitary adenoma was diagnosed. The three patients diagnosed before 1985 went for bilateral adrenalectomy (Nelson's syndrome in two); the others were submitted to transsphenoidal pituitary adenomectomy, obtaining remission in twenty six at the first operation and in two others at the second. Three patients had a recidive. Of the six patients with persistent disease (all treated with metyrapone or ketoconazole), three were submitted to radiotherapy, two to bilateral adrenalectomy, and one was waiting for surgery. Four patients had a bronchial carcinoid, successfully removed in three. One patient was lost to follow-up and another was still being evaluated., Conclusions: We found a positive evolution in the capacity to diagnose and treat these patients. Neurosurgical ability to achieve remission was 80% in the operated cases. More effective technical methods and drugs, as well as a multidisciplinary and dedicated medical team, lead to long lasting remissions in most of the patients.

A clinic case of simultaneous pancreas/kidney transplantation on a type 1 diabetic patient is described. A six years follow-up was made with both organs functionating and a good quality of live. Then the authors make a review of the available surgical techniques, indications and complications of this form of treatment. A comparison between the Portuguese and the International experience was discussed.

Leptin is a product of the ob gene. It is syntetized by the adipocytes. This review will summarize the current findings of the physiopathology and actions of leptin.

The aim of this study is to evaluate the use of conventional external radiotherapy in patients with pituitary adenomas. Between October 1970 and May 1998, 27 patients with pituitary adenoma were followed at the Department of Endocrinology and Diabetes of the Hospitais da Universidade de Coimbra. They received radiation therapy at Instituto Português de Oncologia. Seven of those tumors were classified as nonfunctioning adenomas, 17 as growth-hormone-secreting adenomas, 2 as prolactinomas and 1 as adrenocorticotropic adenoma. Twenty-six patients received radiation as adjuvant therapy after incomplete resection and one patient as primary treatment. The majority of these cases were treated using the parallel opposed-field technique with a total dose between 45 and 52 Gy. The patients were submitted, before and after radiotherapy, to a protocol in order to assess the efficacy of this treatment. Some of the results were analyzed. Reduction of tumor mass was achieved in 66.6% of nonfunctioning tumors and in 25% of the secreting ones. Reduction or stabilization of hormonal levels was achieved in 55% of the cases and normalization in 30%. The average duration of follow-up was 126.3 months. Complications observed: hypopituitarism, stroke (3 patients), cerebral edema (1 patient), memory loss (2 patients) and hearing loss (2 patients). None of the patients developed brain tumors.

Between May 1990 and October 1998, 67 diabetic patients with end-stage renal disease, on dialysis, were submitted to a standardized protocol in order to assess the coexistence and degree of other diabetic and nondiabetic complications that could affect transplantation. Some of the results were analysed. Type 2 diabetic patients had more abnormal results on the lower limbs doppler ultrasound and on the lower limbs arteriography than type 1 (p < 0.05). Type 2 diabetic patients had more cardiovascular complications so the decision to transplant should be taken on a case by case basis.

A total of 618 patients with end-stage renal disease received kidney transplants between 1980 and September 1996. Twenty eight of them were diabetics. Better results were achieved for type 1 diabetic patients than for type 2 (mortality: 5.9% vs 27.3%; functioning graft: 88.2% vs 72.7%). The morbility was also higher in those patients (infections: 81.8% vs 29.4%; vascular complications: 45.5% vs 17.6%). Actuarial patient and graft survival were lower for type 2 than for non diabetic patients. For type 1 diabetics the results are similar to those for non diabetics. Better results can probably be achieved by restricting the selection criteria. The decision to transplant or maintain on dialysis should be made on a case by case basis.

A case of severe ulcerative colitis refractory to steroid therapy is reported. The authors look at cyclosporine use in this clinical setting and make a short review of cyclosporine usefulness and major side effects.