Bone health in children with recurrent and chronic pancreatitis: A multi-center cross sectional analysis.

Background/objectives: Bone health of children with acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) is not well studied.

Methods: This retrospective study was performed at three sites and included data from INSPPIRE-2.

Results: Of the 87 children in the study: 46 had ARP (53%), 41 had CP (47%).

Patient and Caregiver Perceptions of Airway Clearance Methods Used for Cystic Fibrosis.

Introduction: Cystic Fibrosis Foundation guidelines recommend people with CF perform daily airway clearance. This can be difficult for patients, as some find it time consuming or uncomfortable. Data comparing airway clearance methods are limited.

A provider survey of cystic fibrosis related diabetes screening and management practices at North American CF centers.

Background: Cystic Fibrosis Foundation (CFF) Guidelines recommend annual screening for cystic fibrosis related diabetes (CFRD) with an oral glucose tolerance test (OGTT). However, screening rates remain consistently low. We conducted surveys of 1) US CF center directors and 2) Endocrinologists affiliated with the CFF-sponsored EnVision program to characterize CFRD screening practices, describe provider perceived barriers to screening, and identify strategies for improving screening.

Cystic Fibrosis-Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes.

Cystic fibrosis (CF) is a recessive disorder arising from mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. CFTR is expressed in numerous tissues, with high expression in the airways, small and large intestine, pancreatic and hepatobiliary ducts, and male reproductive tract. CFTR loss in these tissues disrupts regulation of salt, bicarbonate, and water balance across their epithelia, resulting in a systemic disorder with progressive organ dysfunction and damage.

Cystic Fibrosis-Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes.

Cystic fibrosis (CF) is a recessive disorder arising from mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. CFTR is expressed in numerous tissues, with high expression in the airways, small and large intestine, pancreatic and hepatobiliary ducts, and male reproductive tract. CFTR loss in these tissues disrupts regulation of salt, bicarbonate, and water balance across their epithelia, resulting in a systemic disorder with progressive organ dysfunction and damage.

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.

The role of modulators in cystic fibrosis related diabetes.

The development and introduction of modulator therapies have completely shifted the paradigm for the treatment of cystic fibrosis (CF). Highly effective modulator therapies have driven marked improvements in lung function, exacerbation rate, weight and quality of life in CF patients. However, their effect on CF related diabetes (CFRD) is not well delineated.

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.

Endocrine Complications of Cystic Fibrosis: A Multisystem Disease of the Endocrine Organs.

Cystic fibrosis (CF) is one of the most common life-limiting genetic disorders. Although CF is typically considered primarily as a pulmonary disease, the CF conductance transmembrane regulator is present throughout the body. From an endocrine perspective, this multisystem disease manifests primarily in the pancreas as a unique form of diabetes (CF-related diabetes mellitus), as bone disease, and as reproductive health issues in people with CF.

Survival in a bad neighborhood: pancreatic islets in cystic fibrosis.

In cystic fibrosis (CF), ductal plugging and acinar loss result in rapid decline of exocrine pancreatic function. This destructive process results in remodeled islets, with only a modest reduction in insulin producing β cells. However, β-cell function is profoundly impaired, with decreased insulin release and abnormal glucose tolerance being present even in infants with CF.

Incretin dysfunction and hyperglycemia in cystic fibrosis: Role of acyl-ghrelin.

Background: Insulin secretion is insufficient in cystic fibrosis (CF), even before diabetes is present, though the mechanisms involved remain unclear. Acyl-ghrelin (AG) can diminish insulin secretion and is elevated in humans with CF.

Methods: We tested the hypothesis that elevated AG contributes to reduced insulin secretion and hyperglycemia in CF ferrets.

CFTR Influences Beta Cell Function and Insulin Secretion Through Non-Cell Autonomous Exocrine-Derived Factors.

Although β-cell dysfunction in cystic fibrosis (CF) leads to diabetes, the mechanism by which the cystic fibrosis transmembrane conductance regulator (CFTR) channel influences islet insulin secretion remains debated. We investigated the CFTR-dependent islet-autonomous mechanisms affecting insulin secretion by using islets isolated from CFTR knockout ferrets. Total insulin content was lower in CF as compared with wild-type (WT) islets.

Abnormal Glucose Tolerance in Infants and Young Children with Cystic Fibrosis.

Rationale: In cystic fibrosis, abnormal glucose tolerance is associated with decreased lung function and worsened outcomes. Translational evidence indicates that abnormal glucose tolerance may begin in early life.

Objectives: To determine whether very young children with cystic fibrosis have increased abnormal glucose tolerance prevalence compared with control subjects.

A Transient Metabolic Recovery from Early Life Glucose Intolerance in Cystic Fibrosis Ferrets Occurs During Pancreatic Remodeling.

Cystic fibrosis (CF)-related diabetes in humans is intimately related to exocrine pancreatic insufficiency, yet little is known about how these 2 disease processes simultaneously evolve in CF. In this context, we examined CF ferrets during the evolution of exocrine pancreatic disease. At 1 month of age, CF ferrets experienced a glycemic crisis with spontaneous diabetic-level hyperglycemia.

Quantifying insulin sensitivity and entero-insular responsiveness to hyper- and hypoglycemia in ferrets.

Ferrets are an important emerging model of cystic fibrosis related diabetes. However, there is little documented experience in the use of advanced techniques to quantify aspects of diabetes pathophysiology in the ferret. Glycemic clamps are the gold standard technique to assess both insulin sensitivity and insulin secretion in humans and animal models of diabetes.

Decelerated early growth in infants of overweight and obese mothers.

Objective: To investigate the relationship between maternal prepregnancy body mass index and early infant growth and body composition.

Study Design: Prospective cohort study performed at a university hospital/surrounding community. Ninety-seven nondiabetic mothers with singleton, term, healthy infants completed study visits at 2 weeks and 3 months of age.

Impaired fasting glucose in cystic fibrosis.

Objective: While glucose tolerance abnormalities are common in cystic fibrosis (CF), impaired fasting glucose (IFG) has scarcely been explored. No studies have examined the relation between IFG and clinical status.

Research Design And Methods: Data were retrieved from the University of Minnesota CF database on oral glucose tolerance tests (OGTTs) performed in 1996-2005.