ACTA ENDOCRINOLOGICA (BUC)

The introduction of tumor necrosis factor-alpha (TNF-α) targeting drugs has given new opportunities in the treatment of various inflammatory rheumatic diseases and has been the most important development in the treatment of ankylosing spondylitis (AS). However, the increasing use and longer follow-up periods of treatment also pose risks of developing various adverse effects, ranging from common ones, including infections, to uncommon thyroid neoplasms. Adalimumab (ADA), a fully human monoclonal antibody targeted against TNF-α, is indicated for AS. We describe the case of a patient with AS who developed a papillary thyroid carcinoma (PTC) while on therapy with ADA. Cervical lymphadenopathy and a heterogeneous nodule in the right thyroid lobe were detected in the 48-year-old female patient who had been using ADA (40 mg subcutaneously every 2 weeks) for 2 years with the diagnosis of AS. Fine-needle aspiration cytology confirmed PTC. ADA treatment was discontinued, and a total thyroidectomy surgery was performed. We believe that the thyroid gland should also be taken into consideration while screening for malignancy before and during TNF inhibitors (TNFi).

Context. The metabolic effects of primary hyperparathyroidism (PHPT) causing increased cardiovascular morbidity have begun to gain importance in medical science, and the number of studies investigating glucose metabolism disorders in asymptomatic PHPT patients is rare. Objective. To evaluate the relationship between glucose metabolism disorders and calcium, phosphorus and parathyroid hormone concentrations in asymptomatic PHPT patients. Subjects and Methods. Fifty-five asymptomatic PHPT patients were included into the study. Control group consisted of 55 normocalcemic cases. Oral glucose tolerance test (OGTT) of 75 g was performed with patients and controls. Insulin resistance was calculated by HOMA index. Results. No significant difference was present between groups regarding fasting plasma glucose, basal insulin and HOMA levels. Glucose levels measured at minutes 30, 90 and 120 after OGTT were higher in patients than in controls (p=0.041, p=0.025 and p=0.001, respectively). No individuals in both groups were diagnosed with diabetes mellitus. While impaired glucose tolerance was detected in six patients with asymptomatic PHPT, no impaired glucose tolerance was determined in controls. A positive correlation was found between serum calcium levels, and fasting plasma glucose and OGTT glucose levels were measured at minutes 60, 90 and 120. Mean fasting plasma glucose was significantly higher in patients with serum calcium levels ≥ 10.5 mg/dL than those with serum calcium levels <10.5 mg/dL (p=0.008). No significant correlation was detected between serum phosphorus and parathyroid hormone levels, and glucose levels were determined in OGTT and HOMA index. Conclusion. Increased levels of serum calcium affect glucose metabolism, so leading to glucose intolerance.

Introduction. Ganglioneuromas are rare, benign tumors that arise from the neural crest tissue and are usually asymptomatic and discovered accidentally. GN have good prognosis with surgical removal and the standard treatment for such lesions is laparoscopic adrenalectomy. Case report. We report the case of a 13 year old patient who presented to our hospital with abdominal pain. The ultrasound and the CT scan confirmed the presence of a well-defined mass situated in the right renal space. Taking into account the size of the tumor, its position and the fact that CT scan showed no local signs of invasion, the decision was taken to perform a laparoscopic removal of the right adrenal mass. After dividing the main adrenal vein and while dissecting the adrenal gland, we noticed an intraoperative bleeding due to the fact that one of the clips used to ligate the vein had slipped. Because we were unable to control the intraoperative bleeding we decided to convert to open surgery. The histopathological result showed that the adrenal lesion was a ganglioneuroma. Conclusion. While the role of minimal invasive adrenalectomy in patients with larger lesion is still controversial, advantages such as reduced hospital stay and postoperative morbidity, lower rate of complications, have resulted in the laparoscopic approach for adrenal pathology becoming the choice procedure in the removal of adrenal lesion.

Lingual thyroid is a rare developmental disorder, and it is the result of failure of the thyroid gland to descend from the tongue root to its normal site. The ectopic thyroid with a functioning gland is even rarer. In this case, we present a 44-year-old female patient with a complaint of foreign body sensation, progressive dysphagia and dyspnea due to ectopic thyroid tissue. Her complaints were totally resolved after L-thyroxine treatment. Here we report a patient with functioning ectopic thyroid tissue who had had bilateral subtotal thyroidectomy 10 years ago due to multinodular goiter. In conclusion, if progressive\r\ndysphagia, dyspnea, and foreign body sensation occurs in a patient who had thyroidectomy and living in an endemic goiter region, lingual thyroid may be underlying disorder.

Context. Pheochromocytomas are very rare but important and potentially life-threatening sources of ectopic ACTH secretion (EAS), and the diagnosis of Cushing’s syndrome due to adrenocorticotropic hormone (ACTH)- producing pheochromocytoma needs a high index of suspicion. Case presentation. Herein, we present a rare case of catastrophic Cushing ‘s syndrome due ACTH-producing pheochromocytoma in a 59-year-old woman, which was characterized by severe hypercortisolism, markedly elevated ACTH levels and rapidly progressed and persisting metabolic derangements, and complete resolution of symptoms and signs after adrenalectomy, despite no biochemical evidence of pheochromocytoma and the coexisting adrenal cortical adenoma. The timely recognition of findings sufficient to raise the suspicion of an ACTH-producing pheochromocytoma is crucial to plan surgical resection of the adrenal mass which is the only curative option enabling quick recovery with complete amelioration of symptoms and signs and restoration of organ functions. Conclusions. In this regard, our case highlights the likelihood of severe hypercortisolism even in the absence of typical Cushingoid features, and the consideration of suspected diagnosis of ACTH-releasing pheochromocytoma even in the absence of biochemical evidence on catecholamine hypersecretion when workup is suggestive of an ectopic source along with an adrenal mass on imaging.

Context. Recent studies have demonstrated a strong association between cardiorespiratory fitness (CRF) and mortality, but bias due to differences in the distribution of baseline variables has not been adequately considered. We studied a cohort of veterans with and without Type-2 diabetes using a propensity score matching method. Methods. Males with (n=592) and without (n= 6,167) Type-2 diabetes were studied. Propensity scores were used to balance covariate distributions between groups with and without Type-2 diabetes. All-cause mortality was the end point. Results. Predictors of mortality included hypertension, smoking, Type-2 diabetes, BMI and CRF. For each 1 MET increase in CRF in the unmatched group, the adjusted HR was 0.83 in those with diabetes (95% CI 0.77- 0.89; p<0.0001) compared to 0.87 in those without diabetes (95% CI 0.86-0.89; p<0.0001). Similar trends were observed for the matched dataset: the adjusted HRs were 0.83 (95% CI 0.77-0.90; p<0.0001) and 0.88 (95% CI 0.82-0.94; p<0.0001) for those with and without diabetes, respectively. Conclusions. CRF is a strong predictor of mortality in veterans with and without Type-2 diabetes. Although the trend in the association between CRF and all-cause-mortality was similar for matched and unmatched data, the mortality risks were relatively inflated when using unmatched data.

Familial hypercholesterolemia (FH) is a genetic disease with autosomal dominant transmission, characterised by high blood cholesterol levels. The evolution of this disease leads to primary atherosclerosis and cardiovascular disease. Patients with HF develop atherosclerosis by the age of 20 and usually do not survive past the age of 30. We present the case and oro-dental aspects of a preschooler that was diagnosed at the age of 4 with FH, compound heterozygote (mutation/genotype1 LDLR: C20IX, exon 4; mutation/ genotype2 LDLR: G571E, exon 12) and the experience of our clinic in the management of this patient that received offlabel treatment with statins. When diagnosed, his cholesterol level was 932 mg/dL and his LDL-cholesterol level was 792 mg/dL. Treatment with rosuvastatin and ezetimibe was prescribed. Both substances (rosuvastatin and ezetimibe) are not approved for children under the age of 6 in Europe. Taking into considerations the diagnosis and prognosis for unfavorable evolution, treatment with statins was started at the age of 5 years.

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Context. Immunoassays are vulnerable to occasional analytical errors despite their sensitivity and specificity. Adrenocorticotropic hormone (ACTH) is among hormones which are vulnerable to assay interference. Objective. In this case report, we aimed to present a case of ACTH interference in a patient with subclinical hypercortisolism. Case report. We present a case of subclinical hypercortisolism with unexpectedly elevated levels of ACTH in whom interference was shown by different biochemical methods. It is important for the clinician to keep in mind the possibility of interference when clinical and laboratory results are discordant. The communication between the clinician and laboratory staff is important while interpreting results. This case report shows that ACTH interference should be considered in patients with subclinical hypercortisolism who have unexpectedly high levels of adrenocorticotropic hormone.