Progress report on multiple endocrine neoplasia type 1.

Multiple endocrine neoplasia type 1 (MEN1) syndrome is an autosomal dominant disorder caused by a germline pathogenic variant in the MEN1 tumor suppressor gene. Patients with MEN1 have a high risk for primary hyperparathyroidism (PHPT) with a penetrance of nearly 100%, pituitary adenomas (PitAd) in 40% of patients, and neuroendocrine neoplasms (NEN) of the pancreas (40% of patients), duodenum, lung, and thymus. Increased MEN1-related mortality is mainly related to duodenal-pancreatic and thymic NEN.

NET Models Meeting 2024 white paper: the current state of neuroendocrine tumour research models and our future aspirations.

Current models for the study of neuroendocrine tumours (NETs) are severely limited. While (e.g.

The effect of Mankai plant consumption on postprandial glycaemic response among patients with type 2 diabetes: A randomized crossover trial.

Aim: To explore the effect of Mankai, a cultivated aquatic duckweed green plant, on postprandial glucose (PG) excursions in type 2 diabetes (T2D).

Methods: In a 4-week, randomized crossover-controlled trial, we enrolled 45 adults with T2D (HbA1c range: 6.5%-8.

Simplified Meal Management in Adults Using an Advanced Hybrid Closed-Loop System.

The advanced hybrid closed-loop (AHCL) algorithm combines automated basal rates and corrections yet requires meal announcement for optimal performance, which poses a challenge for some. We aimed to compare glucose control in adults with type 1 diabetes (T1D) using the MiniMed 780G AHCL system, utilizing simplified meal announcement versus precise carbohydrate (CHO) counting. In a study involving 14 adults with T1D, we evaluated glycemic control during a 13-week "precise phase," followed by two 3- to 4-week simplified meal announcement phases: "fixed one-step" (preset of one personalized fixed CHO amount) and "multistep" (entry of multiples of one, two, or three of these presets depending on meal size estimate).

Long-term metabolic effects of non-nutritive sweeteners.

Objective: Excessive consumption of added sugars has been linked to the rise in obesity and associated metabolic abnormalities. Non-nutritive sweeteners (NNSs) offer a potential solution to reduce sugar intake, yet their metabolic safety remains debated. This study aimed to systematically assess the long-term metabolic effects of commonly used NNSs under both normal and obesogenic conditions.

Mid-Treatment Response to 177-Lutetium Dotatate Predicts Overall Outcome in Patients With Advanced Neuroendocrine Tumors.

Purpose: Patients with advanced, well-differentiated neuroendocrine tumors (WD-NETs) often require both peptide receptor radionuclide therapy (PRRT) and subsequent chemotherapy. However, no mid-PRRT predictors are available to identify patients who will not benefit from subsequent PRRT to limit their radiation exposure. Our aim is to characterize patients for whom subsequent PRRT is less efficacious on the basis of mid-PRRT evaluation.

Role of epigenetic regulation on catecholamine synthesis in pheochromocytoma and paraganglioma.

Introduction: Pheochromocytomas and paragangliomas (PPGLs) typically secrete catecholamines and their metabolites (metanephrines [MN] and normetanephrine [NMN]). Catecholamines are synthesized by several enzymes: phenylalanine hydroxylase (encoded by PAH), tyrosine hydroxylase (TH), aromatic L-amino acid decarboxylase (DDC), dopamine β-hydroxylase (DBH), and phenylethanolamine N-methyltransferase (PNMT). MN/NMN secretion varies between anatomical and molecular subgroups.

Somatic USP8 alteration affects the immune landscape of corticotroph pituitary adenomas- a pilot study.

Introduction: Somatic mutations in ubiquitin-specific protease-8 (USP8), encoding a deubiquinating protein, are found in approximately 30% of corticotroph-derived pituitary adenomas (CPAs). Stratifin, a protein encoded by SFN, inhibits USP8 catalytic activity. USP8 has immunomodulating properties that have been demonstrated in non-tumoral diseases.

An Automated Insulin Delivery System with Automatic Meal Bolus Based on a Hand-Gesturing Algorithm.

Carbohydrate counting (CC) and meal announcements, before eating, introduce a significant burden for individuals managing type 1 diabetes (T1D). An automated insulin delivery system with automatic bolusing that eliminates the need for CC and premeal bolusing (i.e.

Noninvasive Real-Time Glucose Monitoring Is in the Near Future.

Since the introduction of continuous glucose monitoring (CGM) technology, developers have rigorously researched the feasibility of creating a noninvasive glucose monitoring device. In a recent pilot study, investigators reported a strong correlation between glucose values obtained from novel noninvasive monitoring device (GWave) values to venous and capillary glucose measurements. We investigated whether the level of accuracy observed in the pilot study could be reproduced in a larger cohort, using a smaller third-generation manufacturable device (Gen III GWave) containing a standardized sensor chip that can be mass produced for commercial use.

Glycemic outcomes and patient satisfaction and self-management improves in transition from standard to virtual multidisciplinary care.

Aims: With advances in cloud-based technologies, there has been a rise in remote T1D care. We hypothesized that transitioning T1DM care to a virtual, multidisciplinary clinic could improve measures beyond HbA1c.

Methods: To assess the impact of transitioning from standard to virtual T1DM care, we evaluated glycemic measures and patient reported outcomes.

Adipose tissue-derived FABP4 mediates glucagon-stimulated hepatic glucose production in gestational diabetes.

Aims: One of the most common complications of pregnancy is gestational diabetes mellitus (GDM), which may result in significant health threats of the mother, fetus and the newborn. Fatty acid-binding protein 4 (FABP4) is an adipokine that regulates glucose homeostasis by promoting glucose production and liver insulin resistance in mouse models. FABP4 levels are increased in GDM and correlates with maternal indices of insulin resistance, with a rapid decline post-partum.

Expectations from a Home Cooking Program: Qualitative Analyses of Perceptions from Participants in "Action" and "Contemplation" Stages of Change, before Entering a Bi-Center Randomized Controlled Trial.

Home cooking is an emerging strategy to improve nutrition; however, the literature lacks reports about patient expectations from culinary interventions. Personalized medicine utilizes knowledge about a person's genes; yet, behavioral factors, such as participant "readiness" to make a change, may also impact treatment preferences and outcomes. The purpose is to explore the expectations of participants in different stages of change from a home cooking intervention.

Trabecular Bone Score Change Is Not Predicted by Bone Turnover: Short-term Sequential Follow-up.

Background: Trabecular bone score (TBS) reflects vertebrae microarchitecture and assists in fracture risk assessment. The International Society of Clinical Densitometry postulates that the role of TBS in monitoring antiresorptive therapy is unclear. Whether changes in TBS correlate with bone resorption measured by bone turnover markers is not known.

Unannounced Meal Challenges Using an Advanced Hybrid Closed-Loop System.

The advanced hybrid closed-loop (AHCL) algorithm combines automated basal rates and corrections yet requires meal announcement for optimal outcomes. We aimed to compare the performance of the MiniMed™ 780G AHCL algorithm with and without meal announcement. In a single-arm study involving 14 adults with type 1 diabetes, we evaluated the safety and efficacy of AHCL when meals were not announced.

Glucose intolerance in pregnancy and risk of early-onset type 2 diabetes: a population-based cohort study.

Background: The risk of type 2 diabetes among women with glucose intolerance during pregnancy that does not meet gestational diabetes criteria requires further investigation. We aimed to explore the associations between various degrees of gestational glucose intolerance and the risk of type 2 diabetes in young adulthood.

Methods: For this population-based cohort study, the national Israeli conscription database was linked to Maccabi Healthcare Services (MHS), the second-largest state-mandated health provider in Israel.

Non-Interventional Management of Advanced Pancreatic Neuroendocrine Neoplasms in Patients with von Hippel-Lindau Disease.

Von Hippel-Lindau (VHL) is a rare autosomal dominant hereditary cancer predisposition syndrome. Patients with VHL have a high risk for developing retinal and central nervous system hemangioblastoma, pheochromocytoma, renal cell carcinoma, and pancreatic neuroendocrine neoplasms (PNEN). About a fifth of patients with VHL will develop PNEN, and only a tenth of them will develop metastatic or unresectable (advanced) PNEN requiring medical intervention.

Unique Characteristics of Patients with Von Hippel-Lindau Disease Defined by Various Diagnostic Criteria.

Von Hippel-Lindau (VHL) disease diagnosis is based on two criteria sets: International criteria (IC, two hemangioblastomas, one hemangioblastoma plus one visceral lesion, or VHL family history/pathogenic variant plus hemangioblastoma/visceral lesion); or Danish criteria (DC, two clinical manifestations, or VHL family history/pathogenic variant plus hemangioblastoma/visceral lesion). We aimed to compare the characteristics of patients with VHL-related pancreatic neuroendocrine tumor (vPNET) meeting either the clinical Danish criteria only (DOC) or IC to those with sporadic PNET (sPNET). The cohort included 33 patients with VHL (20 vPNETs) and 65 with sPNET.

Design of a stable human acid-β-glucosidase: towards improved Gaucher disease therapy and mutation classification.

Acid-β-glucosidase (GCase, EC3.2.1.

A Case of Metastatic Thymoma Responsive to Treatment With 177 Lu-DOTATATE.

We describe a case of a 74-year-old woman with germline BRCA2 mutation, with an incidental diagnosis of metastatic thymoma presenting as a mediastinal mass with cardiac muscle and lymph node involvement. Despite surgical and radiotherapy treatment, there was marked advancement with new lung and liver metastases. All lesions demonstrated 68 Ga-DOTATATE PET/CT uptake, and the patient received 4 peptide receptor radionuclide therapy cycles with 177 Lu-DOTATATE, with pronounced reduction in the size of the liver, cardiac, and pleural lesions.

A Multidisciplinary Thyroid Eye Clinic: A One-stop Shop.

Background: Little is known about the success of multidisciplinary thyroid eye disease (TED) clinic.

Objectives: To present the characteristics, treatments, and outcomes of patients treated in a multidisciplinary TED clinic.

Methods: A medical record review of all patients who attended a TED clinic was performed.