Endokrinologikum

The care of patients with rare diseases in Germany poses a challenge; endogenous Cushing's syndrome highlights this issue. An analysis of the care situation aims to identify areas for improvement. A systematic literature search on the care situation was compared with prescription data from Germany and France as well as physician statistics from various European countries. In Germany, there is a low density of endocrinologists, a lower treatment prevalence than in France and a longer time to diagnosis than in other countries with well-developed healthcare systems. In order to guarantee need-based care for rare diseases, investments in the training and further education of specialists, such as endocrinologists, and in new, cross-sectoral forms of care are necessary.

In Denmark, the parathyroidectomy rate due to primary hyperparathyroidism has increased over the last decade. Primary hyperparathyroidism leads to a range of diffuse neuromuscular symptoms, bone loss and kidney stones. Tertiary hyperparathyroidism can develop in secondary hyperparathyroidism from chronic renal failure. The predominant definitive treatment for both primary and tertiary hyperparathyroidism is surgery, generally preceded by localization imaging and usually with intraoperative parathyroid hormone assessment. This publication summarizes in this review the updated management of these diseases.

Kırk iki yaş erkek hasta, bilateral sürrenal hematom nedeniyle yapılan incelemede bilateral adrenal kitle saptanması nedeniyle endokrinoloji kliniğinde tetkik edildi. Eş zamanlı HIV pozitifliği ve patolojik lenf nodları da saptanan olguya nonhodgkin lenfoma ve adrenal yetmezlik tanısı konuldu. Takibinde hidrokortizon başlanan, HIV tedavisi düzenlenen ve kemoterapisi tamamlanan hastada adrenal yetmezliğin de düzeldiği görüldü. Bu makalede bilateral adrenal kanamaya bağlı adrenal yetmezlik gelişen bir olgu anlatılacaktır.

Introduction: The endovascular surgical approach provides a minimally invasive treatment of abdominal aortic aneurysms with less perioperative morbidity. This technique depends heavily on imaging to diagnose, plan surgical treatment, conduct the surgical procedure, and follow-up with patients. Image fusion technique was proposed to decrease radiation and injected contrast exposure; however, the accuracy of image fusion still needs to be improved. One of the major causes of this inaccuracy is the displacement of visceral arteries during respiration. Objective: To analyze the physiological respiration-induced movements of the abdominal aortic side branches. Materials and Methods: Thirty healthy volunteers were studied using noncontrast-enhanced MRI. Images were acquired in expiratory and inspiratory forced breath-holds. The displacements were studied by calculating 3-dimensional expiration-inspiration vectors of measured points; diaphragmatic domes, origins of the celiac trunk (CTA), superior mesenteric artery (SMA), right renal artery (RRA), and left renal artery (LRA) as well as the kidneys hila. Results: For all measured points, the superior-inferior displacements were the most important component of movement vectors, and they were statistically significant. The right hemidiaphragm moved by 33.35 mm P < .001, the left hemidiaphragm by 32.4 mm P < .001, the right kidney moved by 19.56 mm P < .001, the left kidney moved by 23.64 mm P < .001, the origin of CTA moved by 3.41 mm P < .001, the origin of SMA moved by 3.54 mm P < .001, the origin of RRA moved by 3.03 mm P < .001, and the origin of moved LRA by 3.17 mm P < .001. Conclusion: We found significant displacements of abdominal aortic branches from forced expiratory to forced inspiratory position. There is a positive correlation between diaphragmatic movement and all visceral arteries orifices displacements; however, this correlation is not high enough to be relayed upon for image fusion correction. Considering only the level of diaphragm to predict the level of abdominal branches origins is insufficient to correct image fusion inaccuracy. We suggest using a dynamic preoperative study as fusion model. Finally, this analysis protocol should be reapplied to a sample of patients with abdominal aneurysm to evaluate respiration-induced movements of the abdominal aortic side branches in pathological condition.Clinical ImpactAlthough it is known that visceral arteries move with respiration, the specific patterns and influencing factors remain unclear. No large-scale studies have quantified this motion under physiological conditions, and current understanding relies mainly on anatomical assumptions rather than detailed evidence.Understanding and predicting this movement has clear clinical implications. Accurate characterization of respiratory-induced arterial motion could improve image registration and fusion techniques, enhance navigation during endovascular or surgical interventions, and reduce errors related to motion artifacts. Ultimately, building predictive models of arterial dynamics may lead to safer procedures, more precise device deployment, and better patient outcomes.

Somapacitan is a long-acting GH approved for once-weekly treatment of GH deficiency (GHD). This study aims to evaluate the efficacy and tolerability of somapacitan after 3 years of treatment and 2 years after switch from daily GH in children with GHD. Randomized, multi-national, open-labelled, active-controlled parallel-group phase 3 trial, with a 52-week main phase and 3-year safety extension (NCT03811535). Treatment-naïve children with GHD were randomized (2:1) to continuous somapacitan (0.16 mg/kg/week; "soma/soma" group) or daily GH (Norditropin®; 0.034 mg/kg/day) followed by somapacitan (0.16 mg/kg/week; "switch" group). Of 200 participants, 188 completed 3 years of treatment. Sustained growth was observed in both groups. At week 156, mean (SD) height velocity (HV) between weeks 104 and 156 was 7.4 (1.5) cm/year in the soma/soma group and 7.8 (1.4) cm/year in the switch group. At week 156, the soma/soma and switch groups had reached a mean (SD) height SD score (HSDS) of -0.95 (0.98) and -1.08 (0.93), respectively, and were approaching the mean mid-parental HSDS of -0.74 (for both groups). Mean total insulin-like growth factor I (IGF-I) SDS during year 3 was similar between groups and within normal range (-2.0 to +2.0). Bioactive IGF-I and bioactive IGF-I to IGF-I ratio were similar between groups. Somapacitan was well tolerated, with low proportions reporting injection-site reactions. Sustained efficacy and tolerability were observed for continuous somapacitan treatment for 3 years, and for 2 years after the switching from daily GH treatment. HSDS in both groups was approaching mean mid-parental HSDS. NCT03811535.