Glucagon-like peptide1 receptor agonist treatment of cystic fibrosis-related diabetes complicated by obesity: A cases series and literature review.

Cystic fibrosis-related diabetes (CFRD) is the most common non-pulmonary comorbidity in people with cystic fibrosis (CF). Current guidelines recommend insulin therapy as the treatment of choice for people with CFRD. In the past, obesity and overweight were uncommon in individuals with CF.

Experimental pharmacological approaches to reverse impaired awareness of hypoglycemia-a review.

The colossal global burden of diabetes management is compounded by the serious complication of hypoglycemia. Protective physiologic hormonal and neurogenic counterregulatory responses to hypoglycemia are essential to preserve glucose homeostasis and avert serious morbidity. With recurrent exposure to hypoglycemic episodes over time, these counterregulatory responses to hypoglycemia can diminish, resulting in an impaired awareness of hypoglycemia (IAH).

Population Pharmacokinetic Model of N-Acetylcysteine During Periods of Recurrent Hypoglycemia in Healthy Volunteers.

Recurrent hypoglycemia leads to impaired awareness of hypoglycemia where the blood glucose threshold that elicits the counterregulatory response is lowered. Hypoglycemia-induced oxidative stress is hypothesized to contribute to impaired awareness of hypoglycemia development and hypoglycemia-associated autonomic failure. Our group conducted a randomized, double-blinded, placebo-controlled, crossover study in healthy individuals undergoing experimentally induced recurrent hypoglycemia to evaluate the impact of intravenous N-acetylcysteine (NAC) during experimental hypoglycemia to preserve the counterregulatory response to subsequent hypoglycemia.

A rare case of isolated IgG4-related hypophysitis with Rathke's cleft cyst presenting as panhypopituitarism.

Summary: We report a rare case of biopsy-proven isolated immunoglobulin G4 (IgG4)-related hypophysitis and Rathke's cleft cyst (RCC) presenting as panhypopituitarism. A 54-year-old Caucasian female presented with symptoms of slurred speech, altered mental status, polyuria and polydipsia and was found to have panhypopituitarism. Brain MRI showed a suprasellar mass with suspected intralesional hemorrhage.

The impact of elexacaftor/tezacaftor/ivacaftor on body composition in a small cohort of youth with cystic fibrosis.

Background: The effects of elexacaftor-tezacaftor-ivacaftor (ETI) on body composition in people with CF (pwCF) are unknown.

Methods: Dual-energy X-ray absorptiometry fat-free mass and fat mass adjusted for height (FMI) as well as oral glucose tolerance test derived measures of insulin secretion and sensitivity were compared before and after ETI initiation in eight pwCF.

Results: Patients median age: 22 years interquartile range (IQR: 16-28), 87.

Glycemia and β-cell function before and after elexacaftor/tezacaftor/ivacaftor in youth and adults with cystic fibrosis.

Background: Diabetes is prevalent among people with CF (PwCF) and associated with worse clinical outcomes. CFTR modulators are highly effective in improving the disease course of CF. However, the effects of elexacaftor/tezacaftor/ivacaftor (ETI) on glucose metabolism in PwCF are unclear.

Clinical Presentation and Diagnostic Approach to Hypoglycemia in Adults Without Diabetes Mellitus.

Objective: To review the clinical presentation, causes, and diagnostic approach to spontaneous hypoglycemia in adults without diabetes mellitus.

Methods: A literature review was performed using the PubMed and Google Scholar databases.

Results: Hypoglycemia is uncommon in people who are not on glucose-lowering medications.

Coronary artery disease in patients with cystic fibrosis - A case series and review of the literature.

Progressive obstructive pulmonary disease is the primary life-shortening complication in people with Cystic Fibrosis (CF); improvement in life expectancy has led to increased prevalence of non-pulmonary complications. Patients with CF are considered to be at low risk for coronary artery disease (CAD). We report here a case series of six patients with CF with and without known cystic fibrosis related diabetes (CFRD) who had acute myocardial infarction (AMI) requiring coronary stent placement.

Hospital-Care Utilization and Medical Cost Patterns Among Patients With Insulin-Dependent Diabetes.

Objective: Using claims data from an integrated payer-provider, we compared costs incurred by patients with insulin-dependent diabetes mellitus (IDDM) who received Hospital Inpatient/Observation/EmeRgency Department care (HIghER care) for diabetes-related events with those who did not receive such care to identify a target population for interventions in future studies.

Methods: A retrospective study pooled real-world claims data for IDDM (type 1 or type 2) between July 1, 2018, and June 30, 2019. Medical claims were used to calculate the total and diabetes-related allowed medical costs to the enterprise and per member per month costs.

Relationship Between Hypoglycemia Awareness Status on Clarke/Gold Methods and Counterregulatory Response to Hypoglycemia.

Context: Impaired awareness of hypoglycemia (IAH) is characterized by the diminished ability to perceive symptoms of hypoglycemia. Gold and Clark questionnaires are commonly used to identify patients with IAH. The relationship between IAH status on questionnaires and a person's symptom and epinephrine responses to hypoglycemia are not well understood.

Heterogeneity in Epinephrine Response to Experimental Hypoglycemia in Type 1 Diabetes and Controls.

Context: The epinephrine response (Epi) to a first episode of hypoglycemia (HG) has been proposed to be predictive of Epi in subsequent HG and to provide insight into the risk for developing HG-associated autonomic failure (HAAF) in healthy controls (HCs).

Objective: To determine if Epi and symptom response (SR) to the first episode of HG predicts who will develop HAAF after exposure to recurrent HG in volunteers with type 1 diabetes (T1D) and in HCs.

Design: Review of data collected between 2013 and 2019.

New Concepts in the Pathogenesis of Cystic Fibrosis-Related Diabetes.

Context: Cystic fibrosis-related diabetes (CFRD) is the most common extrapulmonary complication of cystic fibrosis (CF). Approximately 40% of people with CF who are older than 20 years have CFRD. Presence of CFRD is associated with poor health outcomes in people with CF.

The Effect of Control IQ Hybrid Closed Loop Technology on Glycemic Control in Adolescents and Adults with Cystic Fibrosis-Related Diabetes.

Cystic fibrosis-related diabetes (CFRD) is associated with pulmonary decline, compromised nutritional status, and earlier mortality. Although diabetes technology is increasingly being used in individuals with CFRD, there is a paucity of data investigating the impact of hybrid closed loop (HCL) technology on glycemia in this patient population. In this multicenter retrospective study of 13 adults and adolescents with CFRD, 14 days of continuous glucose monitor data were analyzed at baseline, 1 and 3 months after transition to the Tandem t:slim X2 pump with Control IQ™ technology, a HCL system.

Different FreeSurfer versions might generate different statistical outcomes in case-control comparison studies.

Purpose: Neuroimaging pipelines have long been known to generate mildly differing results depending on various factors, including software version. While considered generally acceptable and within the margin of reasonable error, little is known about their effect in common research scenarios such as inter-group comparisons between healthy controls and various pathological conditions. The aim of the presented study was to explore the differences in the inferences and statistical significances in a model situation comparing volumetric parameters between healthy controls and type 1 diabetes patients using various FreeSurfer versions.

Obesity in cystic fibrosis.

The prevalence of obesity in patients with cystic fibrosis (CF) is increasing and around one-third of adults with CF are now overweight or obese. The causes of excess weight gain in CF are likely multifactorial, including: adherence to the high-fat legacy diet, reduced exercise tolerance, therapeutic advances, and general population trends. Increased weight has generally been considered favorable in CF, correlating with improved pulmonary function and survival.

Spontaneous and iatrogenic hypoglycemia in cystic fibrosis.

Spontaneous episodes of hypoglycemia can occur in people with cystic fibrosis (CF) without diabetes, who are not on glucose lowering medications. Spontaneous hypoglycemia in CF could occur both in the fasting or postprandial state (reactive hypoglycemia). The pathophysiology of fasting hypoglycemia is thought to be related to malnutrition and increased energy expenditure in the setting of inflammation and acute infections.

Monitoring the Neurotransmitter Response to Glycemic Changes Using an Advanced Magnetic Resonance Spectroscopy Protocol at 7T.

The primary excitatory and inhibitory neurotransmitters glutamate (Glu) and gamma-aminobutyric acid (GABA) are thought to be involved in the response of the brain to changes in glycemia. Therefore, their reliable measurement is critical for understanding the dynamics of these responses. The concentrations of Glu and GABA, as well as glucose (Glc) in brain tissue, can be measured using proton (H) magnetic resonance spectroscopy (MRS).

Lumacaftor/ivacaftor therapy fails to increase insulin secretion in F508del/F508del CF patients.

Background: Glucose tolerance abnormalities including cystic fibrosis related diabetes (CFRD) are common in patients with cystic fibrosis (CF). The underlying pathophysiology is not fully understood. Emerging evidence suggests that CFTR dysfunction may directly or indirectly impact β-cell function, offering the potential for improvement with CFTR modulator therapy.

Structural Alterations in Deep Brain Structures in Type 1 Diabetes.

Even though well known in type 2 diabetes, the existence of brain changes in type 1 diabetes (T1D) and both their neuroanatomical and clinical features are less well characterized. To fill the void in the current understanding of this disease, we sought to determine the possible neural correlate in long-duration T1D at several levels, including macrostructural, microstructural cerebral damage, and blood flow alterations. In this cross-sectional study, we compared a cohort of 61 patients with T1D with an average disease duration of 21 years with 54 well-matched control subjects without diabetes in a multimodal MRI protocol providing macrostructural metrics (cortical thickness and structural volumes), microstructural measures (T1-weighted/T2-weighted [T1w/T2w] ratio as a marker of myelin content, inflammation, and edema), and cerebral blood flow.

Infusion of N-acetyl cysteine during hypoglycaemia in humans does not preserve the counterregulatory response to subsequent hypoglycaemia.

Aim: Administration of N-acetyl cysteine (NAC) during hypoglycaemia will preserve the counterregulatory response to subsequent hypoglycaemia in healthy humans.

Methods: This was a randomized double-blind cross over study where humans were given either a 60-minute infusion of NAC (150 mg/kg) followed by a 4-hour infusion of NAC (50 mg/kg) or saline starting 30 minutes before the initiation of a 2-hour hypoglycaemic (HG) clamp at 8 am. After rest at euglycaemia for ~2 hours, subjects were exposed to a 2nd HG clamp at 2 pm and discharged home in euglycaemia.

Prevalence and factors associated with overweight and obesity in adults with cystic fibrosis: A single-center analysis.

Background: The relation between malnutrition and pulmonary death in patients with cystic fibrosis (CF) has resulted in intensive nutritional intervention over the last few decades, leading to a significant decline in underweight and the emergence of overweight/obesity as a potential new problem.

Methods: We performed a cross-sectional database analysis of 484 adults with CF seen at the University of Minnesota CF Center between January 2015-January 2017, to determine the prevalence and pulmonary/cardiovascular risk factors associated with overweight and obesity in this population.

Results: Mean age was 35.

Cystic fibrosis related diabetes: Pathophysiology, screening and diagnosis.

Cystic fibrosis related diabetes (CFRD) is a distinct form of diabetes that is associated with significantly increased morbidity and mortality in the CF population. The primary etiology is relative insulin insufficiency secondary to destruction of pancreatic islets, and to other factors that affect the function of the remaining beta cells. The prevalence of CFRD increases markedly with age and more than half of CF adults develop the disease.

Continuous glucose monitoring in cystic fibrosis - A practical guide.

Our ability to monitor blood glucose levels has become increasingly accurate over the last few decades. Continuous glucose monitoring (CGM) technology now allows providers and patients the ability to monitor glucose levels retrospectively as well as in real-time for diabetes management. CGM also provides the ability to study glucose patterns and trends for insight into the pathophysiology and natural history of disease.

Hypoglycemia in cystic fibrosis: Prevalence, impact and treatment.

Hypoglycemia is a common and feared complication of insulin therapy. As in type 1 and type 2 diabetes, people with cystic fibrosis related diabetes are also at risk for hypoglycemia related to insulin therapy. Spontaneous hypoglycemia is also common in patients with CF without diabetes, who are not on glucose lowering medications.

Cystic fibrosis related diabetes: Medical management.

Cystic Fibrosis Related Diabetes Mellitus (CFRD) drives excess pulmonary morbidity and mortality in patients with cystic fibrosis (CF). The recommended treatment is insulin therapy. Insulin therapy in CF should be customized to the specific patient.