Mapping of the current transition of care practice for patients with pituitary disease at Endo-ERN reference centers.

Rare endocrine conditions present specific diagnostic and management challenges for healthcare providers, one of which is the understudied transition of care. Despite the need for guidance regarding transition, consensus on structured and protocolled approaches is 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 the development of guidelines (n = 5/46) and EU-wide approach (n = 2/46). This study exemplifies the unmet needs for a structural definition of the transition period and transition management for patients with rare hypothalamic and pituitary conditions from healthcare providers' perspective.

Histological Changes in Severe Diabetic Fetopathy: An Autopsy Case Report.

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.