ACTA ENDOCRINOLOGICA (BUC)

Background. Ganglioneuroma (GN) is a rare, benign, neuroblastic tumor. It may arise from the sympathetic plexus or adrenal medulla. Its most common location is the posterior mediastinum (41.5%), while in approximately 21% of patients the tumor is located in the adrenal gland. Median age of adults at diagnosis is approximately 40 to 50 years of age. Since GN is most commonly asymptomatic, the diagnosis is usually accidental, i.e. when radiological examinations are performed due to other indications. This slow-growing tumor can reach a large size. In a small percentage of patients the tumor secretes catecholamines. We present five ganglioneuroma patients, who were hospitalized in our Department from 1993 through 2012. Their mean age was 31 years. In four cases the tumor was located in the adrenal gland and in one - extra-adrenally, which was revealed during surgery. In three out of five presented cases pathological hormone secretion by the tumor was confirmed in laboratory results.

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Introduction. The Antley-Bixler syndrome (ABS) is an extremely rare syndrome characterized by congenital\r\ncraniosynostosis and radiohumeral synostosis. Many patients demonstrate defects in steroidogenesis and disturbances\r\nof sexual development.\r\nAim: To present the first documented case of a Romanian patient with ABS.\r\nMaterial and Methods. Alexandru, a 3 year old child, was referred to our department for elucidating a complex\r\nmalformative syndrome that consisted of ambiguous genitalia and skeletal anomalies. We performed a complete assessment that ranged from obtaining a detailed medical history to gene sequencing. The hospital?s Ethical Committy gave\r\nthe authors its approval for using the medical data concerning the case, after the parents signed an informed consent.\r\nResults. Medical history. We note 4 previous admissions to our hospital during which efforts were made to diagnose the\r\nchild?s disorder of sexual development. At 3 months Edwards syndrome was suspected, which was later infirmed. During the third and fourth admission (8 months, 10 months respectively) the suspicion of congenital adrenal hyperplasia (CAH) was raised and then confirmed (impaired steroidogenesis, 46 XX karyotype). The patient was lost to\r\nfollow-up since then, probably due to the parents? inability to cope with the implications of their child?s condition.

Introduction. Bartter’s Syndrome is a renal tubular defect characterized with low or normal blood pressure, hypokalemic metabolic alkalosis and hyperreninemic hyperaldosteronism due to renal sodium loss. Herein we reported a case who presented with growth retardation, growth hormone deficiency previously treated with growth hormone for a period of 6 months in mind and with hypopituitarism similar findings such as primary amenorrhea, fatigue, weakness and just diagnosed as Bartter’s syndrome. Case report. A 18 year-old female patient was referred to our hospital for amenorrhea, growth retardation and fatigue. Her history revealed that she was diagnosed with growth hormone deficiency two years ago and given growth hormone treatment for 6 months. Physical examination showed growth retardation. Laboratory tests revealed that serum potassium was 2,3 mmol/L. Further investigations demonstrated metabolic alkalosis, increased urinary potassium excretion and hyperreninemic hyperaldosteronism. The patient was considered as Bartter syndrome and spironolactone, indomethacin and potassium chloride were initiated. While she had growth retardation and primary amenorrhea, we searched basal hormone levels and performed stimulation tests for evaluating pituitary reserve. Insulin tolerance test showed that serum growth hormone and cortisol levels were 10 mcg/dL and 19 mcg/dL respectively during hypoglycemic period. It demonstrated that both of the axes were intact. Luteinizing hormone releasing hormone test showed that hypothalamo pituitary gonadal axis was also intact. Conclusions. Bartter syndrome can lead to growth retardation that mimicking hypopituitarism in case of delay in diagnosis.

The COVID-19 pandemic affected 62 million people and caused more than 1.4 million deaths globally till the end of November 2020. Our study aimed to explore the association between obesity and the negative outcomes of COVID-19 worldwide. The prevalence of obesity and overweight per country was extracted from the World Health Organization information system and, for measuring the COVID-19 negative outcomes, we calculated the cumulated notification rate and the mortality for the interval since starting of pandemic till the 29th of November 2020. We explored the linear relationship between COVID-19 and obesity by a bivariate Spearman rank correlation, interpreted using the rule of thumb for 95% level of confidence. We found globally a positive, moderate, and statistically significant correlation between prevalence of obesity and overweight and the negative outcomes of COVID-19. Some of the results found at global level were confirmed in the disaggregated analysis per WHO regions. Due to the linear association of obesity with the COVID-19 negative outcomes, effective actions are needed for keeping the health systems resilience during the COVID-19. Preventing the obese and overweight people to be infected, prioritizing access to vaccination and to adequate care and treatment could be effective.

Objective. Unilateral adrenalectomy (UA) is an alternative for treatment in bilateral adrenal incidentaloma (AI) to avoid possible long-term risks of bilateral adrenalectomy. In this study, we aimed to evaluate the effectiveness of UA in bilateral AI patients with subclinical hypercortisolemia (SH). Method. A total of 35 patients were included in this study. The patients were divided into two groups; those who underwent UA (n=27) and patients without adrenalectomy (PWA) (n=8). Hormone tests related to cortisol mechanism were reviewed to analyze results at the time of diagnosis compared to the latest available results to figure out any changes in cortisol mechanism and determine whether SH has recovered or not. Results. Median age of PWA group were higher compared to UA group (p=0.03). Median duration of followup in groups were similar (p=0.3). In the PWA group, none of the patients recovered from hypercortisolemia during their follow-up. In UA group 92.6% of the patients went into remission, whereas during follow-up 3.3% had recurred and another 3.3% were found to have post-adrenalectomy persistent SH. Patients in UA group had lower final cortisol level following dexamethasone suppression (p=0.003) and higher final adrenocorticotrophic hormone (ACTH) levels (p=0.001) than patients in PWA group. In UA group, final basal cortisol level (p=0.009) and final cortisol level after 1 mg dexamethasone suppression test (DST) (p=0.004) were lower than corresponding levels at the time of diagnosis. Discussion. Our study demonstrates unilateral adrenalectomy targeting the side with the larger lesion is an effective approach to reduce excess cortisol levels in bilateral AI patients with SH.

Context. Prognostic considerations include assessing the risk of liver fibrosis in people with nonalcoholic fatty liver disease (NAFLD). Objectives. This study evaluates the use of hematologic and metabolic parameters regarding liver steatosis and fibrosis scores (FLI and Fib-4) in non-obese type 2 diabetes mellitus (t2DM) patients with NAFLD. Methods. Subjects underwent abdominal ultrasound examinations, and FLI and Fib-4 scores were calculated to evaluate liver steatosis and the risk of liver fibrosis non-invasively: 61 non-obese NAFLD subjects with t2DM were included in the cohort study and were divided into 2 groups depending on the t2DM treatment regimen. Results. Fib-4 and WBC count demonstrated a significant inverse correlation (OR = 0.509, p = 0.007). WBC count had an R2 of 0.237, indicating that this marker could account for up to 23.7% of a variation in Fib-4. Fib- 4 and FFA had positive correlation which did not achieve statistically significant prediction (OR=7.122, p=0.062). Additionally, a significant prediction of HbA1c (OR=1.536, p=0.016) and haemoglobin (OR=1.071, p=0.020) for FLI was revealed. Conclusion. HbA1c and other haematological and metabolic parameters, such as haemoglobin and WBC, may be another non-invasive tool for determining whether nonobese NAFLD patients with t2DM are at risk of developing liver steatosis and fibrosis.

Context. Different vitamin D analogs might have advantages over calcitriol. Objective. To evaluate the effects of paricalcitol vs. calcitriol based vitamin D receptor activators on calcium-phosphate metabolism and pulse wave velocity in hemodialysis patients. Design. Observational, cross-sectional and 1 year follow-up study. Subjects and Methods. 181 hemodialysis patients were enrolled in this study as divided in to 5 groups based on vitamin D therapy. Baseline and 12th month data on blood biochemistry, pulse wave velocity and cumulative dose of treatments were compared in each study group as well as in overall paricalcitol vs. calcitriol-based treatment groups. Results. From baseline to 12th month, significant improvement in pulse wave velocity and parathyroid hormone was shown in paricalcitol-based treatment group without a significant change in calcium, phosphate, alkaline phosphatase. A significant increase in pulse wave velocity, serum phosphate levels, calcium x phosphate product and serum alkaline phosphatase levels were noted in calcitriolbased treatment group with no significant change in serum calcium and parathyroid hormone levels. Conclusion. Our findings revealed superiority of paricalcitol than calcitriol based vitamin D receptor activator therapy in terms of serum phosphate levels, CaxP product, dose requirement for vitamin D and the control of pulse wave velocity.

Background. Patients with acromegaly (caused by growth-hormone-secreting pituitary adenomas) are at increased risk of hypopituitarism, in particular hypogonadotropic hypogonadism, before and after multimodal therapy. In affected women of reproductive age, fertility is impaired and complex fertility treatments are needed to achieve conception. C ase presentation. We present the case of a young woman with acromegaly caused by a GH-secreting macroadenoma with suprasellar and bilateral cavernous sinus extension; hypogonadotropic hypogonadism and secondary hypothyroidism were present from the initial evaluation. Neurosurgical intervention was repeatedly recommended but the patient refused it initially; also she was non-compliant to the medical treatment of acromegaly. Transsphenoidal tumor debulking with adjuvant gamma-knife radiotherapy was eventually performed. Following treatment persistent active acromegaly and hypogonadotropic hypogonadism were diagnosed. Under chronic estroprogestative replacement therapy, the patient conceived and delivered a full-term healthy newborn without any complications. Possible mechanisms are discussed. Conclusions. Secondary hypogonadotropic hypogonadism in pituitary patients, even when considered permanent (after surgery and radiotherapy), can exceptionally allow spontaneous conception and normal course of pregnancy.