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Objective: The European Registries for Rare Endocrine Conditions (EuRRECa, eurreb. eu) includes an e-reporting registry (e-REC) used to perform surveillance of conditions within the European Reference Network (ERN) for rare endocrine conditions (Endo-ERN). The aim of this study was to report the experience of e-REC over the 3.5 years since its launch in 2018. Methods: Electronic reporting capturing new encounters of Endo-ERN conditions was performed monthly through a bespoke platform by clinicians registered to participate in e-REC from July 2018 to December 2021. Results: The number of centres reporting on e-REC increased to a total of 61 centres from 22 countries. A median of 29 (range 11, 45) paediatric and 32 (14, 51) adult centres had reported cases monthly. A total of 9715 and 4243 new cases were reported in adults (age ≥18 years) and children, respectively. In children, sex development conditions comprised 40% of all reported conditions and transgender cases were most frequently reported, comprising 58% of sex development conditions. The median number of sex development cases reported per centre per month was 0.6 (0, 38). Amongst adults, pituitary conditions comprised 44% of reported conditions and pituitary adenomas (69% of cases) were most commonly reported. The median number of pituitary cases reported per centre per month was 4 (0.4, 33). Conclusions: e-REC has gained increasing acceptability over the last 3.5 years for capturing brief information on new encounters of rare conditions and shows wide variations in the rate of presentation of these conditions to centres within a reference network. Significance statement Endocrinology includes a very wide range of rare conditions and their occurrence is often difficult to measure. By using an electronic platform that allowed monthly reporting of new clinical encounters of several rare endocrine conditions wi thin a defined network that consisted of several reference centres in Europe, the EuRRECa project shows that a programme of e-surveillance is feasible and acceptable. The data that have been collected by the e-reporting of rare endocrine conditions (e-REC) can allow the continuous monitoring of rare conditions and may be used for clinical benchmarking, designing new studies or recruiting to clinical trials.

As the most visible and vulnerable organ of the human organism, the skin can provide an impression of its state of health. Rare forms of diabetes and endocrinopathies are often diagnosed late or primarily misinterpreted due to their rarity. Skin peculiarities associated with these rare diseases may be indicative of the underlying endocrinopathy or form of diabetes. At the same time, rare skin changes in diabetes or endocrinopathies can also be a major challenge for dermatologists, diabetologists and endocrinologists in optimal patient and therapy management. Active collaboration between these different specialist groups can therefore lead to increased patient safety, better therapeutic success and more targeted diagnostics.

There is considerable heterogeneity within the clinical phenotype of type 1 diabetes: genetic susceptibility is complex, progression rates differ markedly, as does the efficacy of therapeutic interventions. New prevention strategies should use biomarkers to lead to personalized combination therapies analogous to the approach used in pediatric oncology. It is well known that type 1 diabetes starts with autoimmunity, the detection of 2 or more persistent diabetes-associated antibodies (stage 1). Additional subclinical dysglycemia is termed stage 2, and manifest type 1 diabetes is termed stage 3. Both genetic and immunologic parameters allow early detection in children and adolescents. Because 9 out of 10 affected children come from families without other family members with type 1 diabetes, screening approaches provide an opportunity for preservation of residual insulin-producing beta cell function. Studies of the psychological burden of screening procedures and results showed that the vast majority of families were able to live relatively unburdened by the knowledge of their child's increased diabetes risk after a short period of time. The GPPAD research network is investigating primary prevention with oral insulin (POINT study) or probiotics (S1NTIA study). The European research network INNODIA is developing a standardized study protocol ("master protocol") to accelerate the investigation of new single agents and combination therapies from the age of 5 years. Secondary prevention approaches are showing promising results in initial pilot studies to prolong the remission phase or delay disease onset in at-risk populations. Intensified insulin therapy with differential substitution of basal- and bolus insulin is the gold standard also for children with type 1 diabetes. The different absorption profiles and half-lives of insulin preparations allow insulin therapy to be tailored for the individual patient. Ultra-rapid insulin analogues show improved postprandial glucose levels, while second-generation basal analogues are associated with less hypoglycemia and reduced events of hyperglycemia and ketosis as a surrogate marker of a high risk for DKA. New parameters like time in range and time below range suitable for identifying high glycemic variability as risk factor for severe hypoglycemia complement the HbA1c targets and the ambulatory glucose profile in a shared decision making on therapeutic adjustments between the diabetes team and families with a child with diabetes. Automated insulin delivery as a hybrid closed loop or dosing advice using artificial intelligence are becoming a clinical reality. However, diabetes education as a team approach, defining clear targets with outcomes evaluated in multinational registries like SWEET remain important for shaping the future of pediatric diabetology.

Short-term glycemic variability is associated with the risk of hypoglycemia and hyperglycemia in people living with type 1 diabetes and can potentially affect clinical outcomes. Continuous glucose monitoring (CGM) is of increasing importance to evaluate glycemic variability in greater detail. Specific metrics for assessing glycemic variability were proposed, such as the SD of mean glucose level and associated coefficient of variation, and time in target glucose range to guide study designs, therapy and allow people with diabetes more transparency in interpreting their own CGM data. Randomized controlled trials (RCT) and real-world evidence provide complementary information about the efficacy/effectiveness and safety of interventions. Insulin glargine 300 U/mL (Gla-300) has a longer lasting and less variable action than insulin glargine U100 (Gla-100) with a lower risk of hypoglycemia. While insulin degludec U100 (iDeg-100) was associated with lower glucose values but more time below range in one randomized study compared with Gla-300, Gla-300 was associated with a higher per cent time in range, but also above the therapeutic range. However, a real-world study did not find differences during the day between Gla-300 and iDeg-100. The upcoming InRange RCT is the first head-to-head comparison of Gla-300 with iDeg-100 using CGM in an international population using CGM metrics as the primary endpoint. The non-interventional COMET-T real-world study will determine the real-world effectiveness of Gla-300 using CGM metrics and cover a broad spectrum of clinical practice decisions irrespective of the prior basal insulin.

Introduction Empagliflozin reduced morbidity and mortality in patients with type 2 diabetes mellitus (T2DM) in clinical trials. A registry study was undertaken to describe evolution of patient characteristics and assess the real-world effectiveness/safety of empagliflozin.Research design and methods Data from the Diabetes Patienten Verlaufsdokumentation (DPV)/Diabetes Versorgungsevaluation (DIVE) registries on 9571 adults with T2DM (registered in 2014–2019) receiving empagliflozin were used. Patients were grouped according to the following: early users (group 1; n=505) received empagliflozin before the EMPA-REG OUTCOME study publication (mid-September 2015); intermediate users (group 2; n=2961) started empagliflozin after the EMPA-REG OUTCOME publication but before the European Medicines Agency label change (from mid-September 2015 to mid-January 2017); and late users (group 3; n=6105) started empagliflozin after mid-January 2017. Data on clinical and treatment characteristics were collected.Results Over time, the proportion of recipients aged

Background: An understanding of the current status of patients with type 1 diabetes mellitus (T1DM) can help to provide appropriate treatment. Methods: This was a retrospective analysis of the DIabetes Versorgungs-Evaluation (DIVE) and the Diabetes-Patienten-Verlaufsdokumentation (DPV) databases for Germany. Results: The analysis included 56,250 people with T1DM (54.2% male), a median age of 36.8 years, and a median diabetes duration of 12.4 years. 15.3% were obese (body mass index ≥ 30kg/m 2 ). Long-acting insulin analogs were used by 53.3%, short-acting analogs by 72.1%, and oral antidiabetic drugs by 4.7%. Patients had a median glycosylated hemoglobin (HbA1c) of 7.8%. There was a drop in HbA1c and an increase in the rate of hypertension, oral antidiabetic drug use, and in the rate of severe hypoglycemia (all p < 0.01) with age. Flash glucose monitoring (FGM) showed the best glucose values with fewer complications compared to other monitoring systems. HbA1c and FBG were lower in patients using a pump versus multiple daily injections (MDIs; 7.7 versus 7.9% and 7.8 versus 8.7 mmol/l; all adjusted p < 0.01). Patients had a lower risk of at least one severe hypoglycemic or DKA episode during the most recent treatment year with pump treatment compared to MDI (9.4% versus 10.5% and 4.7% versus 6.1%, both adjusted p < 0.01). Conclusion: The data demonstrated less-than-optimal glycemic control in the young, an increasing metabolic pattern in T1DM with increasing age, a benefit of FGM to improve HbA1c control and adverse effects, as well as benefits of pump treatment over MDIs.

For paediatric patients with type 1 diabetes, intensified insulin therapy with either multiple daily injection or insulin pump therapy is currently the only method of treatment. To optimize this therapy, insulin analogues are fixed parts of all therapy regimens. New ultra-rapid insulins seem to be beneficial not only in adults but also in this age group. New developments in long-acting analogues have demonstrated safety and will be regular in paediatrics, we hope, soon. Furthermore, the psychosocial approach for consideration of real-life aspects becomes more the focus of therapeutic regimens and is implemented into international guidelines. Technical improvements, such as continuous glucose monitoring, particularly in combination with pump therapy, support the great success of rapid-acting analogues by reducing hypoglycaemias. Non-insulin agents such as SGLT2-inhibitors show beneficial aspects in people with type 1 diabetes. For outpatient care with these currently off-label-used drugs, special training for measurement of ketones should be imperative.

Aims. This study assesses the impact of using an AP-system at home on fear of hypoglycaemia. In addition, satisfaction and acceptance of the new technology are evaluated. Methods. In a multicentre, multinational study of 75 patients using the MD-Logic AP during four consecutive nights in home setting 59 of them (aged 10–54 years, 54% male, HbA1c 7.89 ± 0.69% [62.72 ± 7.51 mmol/mol], diabetes duration 11.6 ± 8.4 yrs) answered standardized questionnaires (HFS, adapted TAM, and AP satisfaction) before and after using the AP. Results. After experiencing the AP in home setting worries of hypoglycaemia were significantly reduced (before 1.04 ± 0.53 versus after 0.90 ± 0.63; P=0.017). Perceived ease of use as a measure of acceptance with the AP significantly increased after personal experience (before 4.64 ± 0.94 versus after 5.06 ± 1.09; P=0.002). The overall satisfaction mean score after using the AP was 3.02 ± 0.54 (range 0–4), demonstrating a high level of satisfaction with this technology. Conclusions. The four-night home-based experience of using MD Logic AP was associated with reduced worries of hypoglycaemia, high level of satisfaction, and increased perceived ease of use of the new technology in children, adolescents, and adults.