ACTA ENDOCRINOLOGICA (BUC)

Aim. Posttransplant diabetes mellitus (PTDM) is a metabolic complication that usually occurs after liver transplantation (LT) due to immunosuppression. In this study, our aim was to identify PTDM incidence after LT in our center and the potential risk factors. Materials and Methods. In this study, 238 adult LT patients were evaluated in terms of PTDM development. Results. Of 238 patients included in the study, 170 (71.4%) were male, 68 (28.6%) were female and the mean age was 43.5± 13.7 years. Of all patients, PTDM developed in 24 (10.1%). Transient-Hyperglycemia (t-HG) was detected in 31 (13%) patients. PTDM and t-HG patients had a greater body weight than non-PTDM patients (BMI kg/ m2 : 27.6± 5.3, 25.8± 4.3and 23.9± 3.3, respectively p<0.001 p= 0.028). PTDM and t-HG patients mean age was higher than non-PTDM patients (51.5± 9.68, 48.2± 11.1 and 41.5± 14 years, respectively, p= 0.002 p= 0.023). In the univariate analysis, the only independent risk factor for PTDM was age (OR 1.93, 95% CI 1.31-2.97). Conclusion. Age is the most important risk factor for PTDM development after LT. PTDM was found more common in the patient group with greater body weight. Patients with older age and greater body weight should be examined more carefully for PTDM before LT.

Background. Relative adrenal insufficiency (RAI) is the inadequate production of cortisol due to dysfunction of the hypothalamic–pituitary–adrenal (HPA) axis during a severe illness. We evaluated the HPA axis and RAI in a tertiary pediatric intensive care unit (PICU). Methods. A total of 100 PICU patients were included in this prospective cohort study. Basal serum levels of adrenocorticotropic hormone (ACTH), cortisol values were compared with those in the control group. A low-dose ACTH stimulation test was performed in patients with basal cortisol levels below 18 μg/dL. Results. The basal cortisol levels of the PICU patients were significantly higher than those of the control group (P < 0.05). All tested patients (n= 24) had delta cortisol levels > 9 μg/dL and a peak cortisol response > 18 μg/dL. Basal cortisol levels were positively correlated with Pediatric Risk of Mortality (PRISM) III scores (P < 0.05; r = 0.363). The basal or stimulated cortisol levels of the patients who received glucocorticoid treatment were higher than the cut-off levels. Conclusions. High basal or stimulated cortisol levels are indicative of disease severity in the acute phase of stress. Patients with very high cortisol levels should be particularly carefully monitored because of the high mortality risk.

Context. We report the use of subcutaneous somatostatin injection three times a day to decrease hypercortisolism in a patient who had Cushing’s syndrome induced by bronchial carcinoid tumour progressive pneumonia due to immune suppression. Subject and Method. A 46-yearold man with 7-month history of DM type-2, hypertension and cerebrovasculardisease, vertebral compression-fracture was admitted to our clinic. Physical examination was consistent with Cushing’s syndrome. Laboratory results revealed hyperglycemia (143 mg/dL; reference range, <100 mg/dL) and hypokalemia (2.29 mEq/L; reference range, 3.5-5.1 mEq/L). His morning serum cortisol was 40 μg/dL (reference range 6.7- 22.6 μg/dL), urine cortisol-excretion was 2245 μg/24 hours (reference range 58-403 μg/24 hours), after 1 mg dexamethasonesuppression test serum cortisol was 28 μg/dL (6.7-22.6 μg/dL) and ACTH 354 pg/mL (reference range 7.9-66 pg/mL). Adrenal CT and hypophyseal MRI were normal. An ectopic source was searched for Cushing’s syndrome. Chest CT scan of the right lung showed 12x9 mm nodule. High fever cough occurred on the followp. Chest radiograph revealed diffuse pneumonic infiltration. Despite 3-drug antibiotic combination therapy, infection did not improve. Subcutaneous injection of octreotide 3x100 μ/g was initiated to decrease hypercortisolism. The infection improved rapidly after the therapy. The morning serum cortisol, urine cortisol-excretion, ACTH was at the upper normal range (77.1 pg/mL, reference range 7.9-66 pg/mL) on 10th day of treatment. The patient was a consulted for surgery and the nodule was excised. The pathology was consistent carcinoid tumor. Conclusion. Subcutaneous octreotide treatment may be helpful to gain time for exploring the focus in ectopic cushing’s syndrome and to control the serious infections due to hypercortisolism.

Context. Children having gonadal tumors and disorder of sex differentiation (DSD) are rare. Objective. To investigate the presentation of DSD children with malignant gonadal tumors. Methods. A retrospective study from 2010- 2020, that evaluated 17 children with DSD, including 13 females, eight months to 16 years, with congenital adrenal hyperplasia, 5-alpha reductase deficiency, androgen insensitivity syndrome, Turner, Sywer, and Klinefelter syndromes. Results. Ten children had malignant gonadal tumor; nine had germ cell tumors and one person granulosa cell tumors, while seven children with non-malignant tumor had gonadoblastoma, cystadenoma (five children), and cysts. Systemic malformations, obesity, elevated tumor markers, and psychosocial issues were observed in 90%, 90%, 70%, and 50% of children with malignancy unlike 28.6%, 42.9%, 14.35%, and 57.1% children without malignancy respectively. Most (9/10) children >12 years, had psychosocial issues, unlike 0/7 children ≤12 years. From 8/17 children presenting with symptoms suggestive of tumor, 75% had malignancy, while from 9/17 children with DSD presentation, 44% had malignant tumors. Malignancy was observed in 3/10 children between eight months to age six, while 7/10 children had stage 1-2 tumors. We reported a child, identified as female, aged 13 years, with partial androgen insensivity syndrome (PAIS) 46,XY, and testicular papillary serous cystadenoma with genomic variant AR NM_000044.4:c.2750del. p.(F917Sfs*27) chromosome Xq12, never published in people with PAIS nor population databases (GnomAD). Conclusion. DSD diagnosis raises numerous challenges. People with DSD have increased risk of malignancy, especially when obesity and, systemic malformations are present; also, psychosocial issues in these children are associated with postpubertal age.

Background. Antineutrophil cytoplasmic antibodies (ANCA) positivity is usually determined in vasculitis of medium and large arteries. In literature, data related to the prevalence of ANCA positivity and the development of antibodies after antithyroid therapy in Graves’ disease are quite rare. Aim. To investigate the titers of myeloperoxidase (MPO)-ANCA and proteinase 3 (PR3)-ANCA in Graves’ patients treated with propylthiouracil (PTU) and to determine the factors that may contribute to ANCA positivity. Subjects and Methods. Fifty-two Graves’ patients treated with propylthiouracil (PTU) were included into the study. The control group consisted of 37 healthy subjects. MPO-ANCA and PR3-ANCA titers were measured in both groups. Results. Mean titer of PR3-ANCA in Graves’ group was significantly higher than in controls (p=0.025), but no significant difference was found in the titer of MPOANCA between two groups (p=0.060). A positive correlation was observed between PR3-ANCA titer, and anti-thyroid peroxidase antibody and anti-thyroglobulin antibody levels in Graves’ patients (p=0.001, r=0.447 and p=0.030, r=0.310, respectively). PR3-ANCA titer in anti-thyroglobulin antibody positive patients was higher than those with negative antibody (p=0.018). A positive correlation was detected between the duration of treatment and PR3-ANCA titer (p=0.024, r=0.314). Both MPO-ANCA and PR3-ANCA were positive in two Graves’ patients, while only MPO-ANCA was positive in two patients. No signs of vasculitis in ANCA positive patients were observed. Conclusion. Propylthiouracil (PTU) may cause ANCA positivity, but no vasculitis may develop in most of the cases. A correlation was determined between PR3- ANCA titer, and thyroid autoantibodies and the duration of treatment.

Objective. The aim of this study was to compare the clinical effects of a triple oral antidiabetic combination versus basal insulin and metformin combination treatment in patients with poorly controlled type 2 diabetes.\r\nMethods. Eighty patients with type 2 diabetes, who were treated by metformin and sulphonylurea combination, and had\r\nHbA1c values between 7.5 and 10 % (58 and 86 mmol/L), were randomized into two groups. The first group was given triple oral antidiabetic therapy (pioglitazone, metformin, and sulphonylurea) and the second group was given metformin and a bedtime basal insulin (insulin detemir) combination for 12 weeks. Metabolic parameters were evaluated.\r\nResults. The mean fasting plasma glucose and HbA1c levels decreased in both groups. The decrease in HbA1c was slightly\r\nhigher in triple oral antidiabetic group (p=0.046). The patients in triple oral combination group gained 0.2 kg (p=0.881) and those in the metformin-insulin detemir combination group lost 1.7 kg (p=0.001) in 12 weeks (p=0.29 between groups). The frequency of hypoglycemia was higher in\r\ntriple oral antidiabetic group (11 vs. 2 episodes, respectively).\r\nConclusion. Both sulphonyureametformin-pioglitazone and insulin detemir-metformin therapies provided significant improvements in glycemic control. However, sulphonylurea,\r\npioglitazone and metformin combination led to more frequent hypoglycemic events, and weight management seemed in favor of insulin detemir-metformin combination.