ACTA ENDOCRINOLOGICA (BUC)

Denosumab,a monoclonal IgG2 antibody, is used as neoadjuvant therapy for giant cell bone tumors, particularly in inoperable or metastatic cases. It targets the receptor activator of nuclear factor kappa-β ligand (RANKL), which is overexpressed in tumor stromal cells. However, denosumab treatment can lead to side effects such as hypocalcemia during treatment and rarely but malignant hypercalcemia after discontinuation. The unpredictable onset time and persistent course of hypercalcemia attacks increase the duration of hospitalization and the risk of complications. Case. A 9-year-old girl with a giant cell bone tumor was treated with denosumab for diffuse tumor recurrence. Severe hypercalcemia occurred four months after completing therapy. Evaluation suggested "rebound-linked" hypercalcemia following denosumab discontinuation. IV bisphosphonate treatment normalized calcium levels initially, but hypercalcemia recurred, requiring repeated IV bisphosphonate administration. Oral alendronate was used weekly to prevent further attacks, resulting in stable calcium levels during follow-up. Results. Rebound hypercalcemia, as an unpredictable recurrent episode at any time, is a potential complication of denosumab cessation, and requires close monitoring post-treatment. Children may be at higher risk due to their rapid bone cycle. In long-term follow-up, IV and oral bisphosphonates can be used effectively in the management of especially life-threatening recurrent attacks.

Background. Iodine deficiency and/or thyroid autoimmunity are the most common causes of hypothyroidism development among pregnant women. In this study, we aimed to investigate the effect of iodine consumption and thyroid autoimmunity on TSH levels and abortus. The study sample consisted of 104 patients, 79 in abortus and 25 in control groups. TSH, free T4, free T3, anti-TPO, anti-Tg, spot urinary iodine concentrations, and thyroid volumes of the cases were measured by ultrasonography. The spot urine concentration was below 100 μg/L in 93% of the cases included in the study. The TSH levels of the abortus group cases were significantly higher than those of the controls (p=0.025). The percentage of subclinical hypothyroid cases were significantly higher among the cases evaluated due to abortus compared to the control group (p<0.001). Abortus and control groups did not differ statistically with respect to the presence of autoimmune thyroid diseases (p=0.424). Spot urine iodine concentrations of abortus cases with subclinical hypothyroid were significantly lower than those with TSH levels below the defined range (p=0.001). Spot urine iodine concentrations of the cases with subclinical abortus with negative thyroid autoantibodies were also significantly lower than those with TSH levels below the defined range (p=0.017). TSH levels above 1 μIU/mL for the first trimester and 2 μIU/mL for the second trimester may be indicators of iodine nutrition in pregnancy losses.

Objective. This study was undertaken to examine prognostic factors in patients with pancreatic neuroendocrine tumors (PNET) undergoing surgical treatment to evaluate the prognostic value of recently introduced immunohistochemical staining methods of CD10 and cytokeratin 19. Materials and Methods. Tumors were classified on the basis of 2004 WHO Classification Guidelines and European Neuroendocrine Tumor\r\nSociety (ENETS) grading system. Immunohistochemical staining with Ki- 67, CD10 and cytokeratin 19 was performed. Results. A total of 36 patients with a mean age of 53.7 ? 12.0 years were included. Overall, 33 patients had a long-term follow-up with 10 patients (30.3%) experiencing recurrence. Seven\r\npatients (21.1%) died. Clinical parameters that were associated with recurrence included liver metastasis at the time of surgery and extra-pancreatic invasion (p < 0.005). Positive surgical margins, extra-pancreatic invasion, and multi-focal disease were associated with reduced survival (p < 0.05). In addition, there was an association between\r\nsurvival and WHO 2004 classification (p < 0.05).\r\nConclusions. Although vascular and peripancreatic invasion showed increased risk of recurrence, they were unrelated to survival. Of the histopathological examinations, Ki-67\r\nand mitotic activity showed a correlation with both recurrence and survival, while immunohistochemical\r\nstaining with cytokeratin 19 and CD 10 did not provide adequate prognostic information.

Background. HDR syndrome is an autosomal dominant disorder characterized by hypoparathyroidism, sensorineural deafness, and renal dysplasia caused by mutation of the GATA3 gene. Case report. We describe a case of HDR syndrome diagnosed in a 46-yearold male patient. He had hand numbness, intermittent dizziness, and imbalance due to hypocalcemia. Physical examination was slim in appearance, had marfanoid features, and presented the typical apathetic face. He had a narrow rib cage and pectus excavatum deformity. Laboratory findings included serum calcium of 7.4 mg/dL, phosphorus of 4.87 mg/dL, albumin of 4.5 g/dL, intact parathormone (iPTH) of 13.3 pg/mL, 25(OH) D3 vitamin of 16.3 μg/L, daily urinary calcium excretion of 23.4 mg/day. Noncontrast computerized tomography (CT) of the brain showed bilateral basal ganglia calcification. Abdominal ultrasonography and CT showed the absence of renal tissue in the left kidney site and pelvis (left renal aplasia). Audiometry revealed mild sensorineural hearing loss at high frequencies. Conclusions. The patients with HDR syndrome are often diagnosed when they are examined for symptomatic hypoparathyroidism and deafness. To the best our knowledge, the present case is the second case of Barakat Syndrome reported from Turkey. The treatment of HDR syndrome is focused on clinical symptoms. The prognosis of the disease correlates with hypoparathyroidism and the severity of renal disease. HDR syndrome has an autosomaldominant inheritance, screening of family members is recommended for early diagnosis and treatment.