Maternal diabetes is one of the most common and dangerous risk factors during pregnancy, as often there are no generalized signs. Diabetic fetopathy is a severe, poorly defined complication of gestational diabetes or preexisting maternal diabetes mellitus, with an ill-defined histological spectrum of changes. Herein we report a case of severe diabetic fetopathy diagnosed upon autopsy of a recently miscarried fetus. On histology, the liver revealed severe generalized macrovesicular steatosis and number of small cysts. The pancreas revealed not only Langerhans isle hyperplasia, but also Langerhans amyloidosis, evident of the severity of maternal diabetes and fetal hyperglycemia. The adrenal glands revealed hyperplasia in zona glomerulosa, due to aldosterone overproduction, evident of fetal hypertension. The current case is an extreme example of an undiagnosed and untreated gestational diabetes mellitus. The severity of histological changes, in this case, is suggestive of further extension of the diagnostic criteria of diabetic fetopathy to include more subtle changes that can be observed clinically and even a combination of maternal-newborn factors.

Rare endocrine conditions present specific diagnostic and management challenges to healthcare providers, one of which is the understudied transition of care. Despite the need for guidance regarding transition, consensus on structured and protocolled approaches are lacking. Therefore, we aimed to map the current clinical practice, and identify unmet needs regarding transition of care for patients with pituitary disease in the reference centers (RCs) of the European Reference Network on Rare Endocrine Conditions (Endo-ERN). A survey-based, cross-sectional study using the EU Survey tool was performed, and completed by 46 physicians (n) from 30 RCs (N). Transition is a common practice among RCs (n=44/46), usually accomplished by a multidisciplinary team meeting (N=20/30). Criteria for start and end of transition were defined in half of the RCs, with 16.7% of centers providing dissimilar answers. Transition readiness was assessed by >75% of the RCs, mostly by unvalidated means (e.g. subjective opinions, informal consultations). Pituitary-specific transition assessment tool was applied in one RC only. Transition protocols were present in only 9% of RCs, while in many RCs transition decisions were taken in combined adult-pediatric meetings, or based on clinicians’ personal judgment. A minority of physicians evaluated the effectiveness of transition-related interventions (n=11/46), or medical outcomes (n=8/46). Patient-reported outcome measures were infrequently used (n=4/46). Identified unmet needs included development of guidelines (n=5/46), and EU-wide approach (n=2/46). This study exemplifies the unmet needs for a structure the definition of the transition period and transition management for patients with rare hypothalamic and pituitary conditions from healthcare providers perspective.

AIM: The human laryngeal complex has been an endoscopic sight since the late 19 th century, however despite the technological advances little has changed in the standard observational methodology, with optical distortion of the image being a major setback in all endoscopic studies. The aim of this study is to evaluate different endoscopes and image correction algorithms in the endoscopic imaging of the laryngeal complex. METHODS AND MATERIALS: Conventional rigid 0 0 , 30 0 , 70 0 and 90 0 endoscopes and a flexible otorhinolaryngoscope with two-dimensional imaging capabilities, a digital camera and a direct laryngoscope were used together with an intubation mannequin to obtain endoscopic images of the laryngeal complex. Images of a custom made lens distortion grid were taken to compare the different optical systems and used as a benchmark for image correction. Both sets of images were superimposed in order to digitally correct for angle and lens distortion and to show the true size and proportions of the human laryngeal complex, so far seen only during an intubation or autopsy. RESULTS: After comparing the fiber optic and rigid endoscope obtained images, the rigid endoscopes proved to have better imaging qualities and therefore a better potential for future in depth study of laryngeal anatomy. The rigid 70 0 endoscope, compared to other rigid systems, allowed for an atraumatic perpendicular to the glottis view of the laryngeal complex, best suited for the study, despite the higher image distortion. CONCLUSION: Based on the collected data the 70 0 rigid otorhinolaryngoscope together with a standard software correction is an affordable, atraumatic and reliable method for anatomical and proportionate measuring of the human laryngeal complex in a model study.