John A Dodge

TL;DR: The conclusions of a consensus conference to address the use and interpretation of CF mutation analysis in clinical settings are described, including the use of CFTR genotype for prediction of prognosis in people with CF at the time of their diagnosis is not recommended.

TL;DR: There is great heterogeneity in the clinical manifestations of cystic fibrosis (CF).

TL;DR: A proposal for consensus guidelines on cystic fibrosis transmembrane conductance regulator ( CFTR)-related disorders (CFTR-RDs), reached after expert discussion and two dedicated workshops is presented.

TL;DR: Draft guidelines for diagnosis, evaluation of organ and system abnormalities, and treatment of hematologic, pancreatic, dietary, dental, skeletal, and neurodevelopmental complications for Shwachman‐Diamond syndrome are provided.

TL;DR: Before starting nutritional supplementation, it is important to rule out pathological conditions that may have a deleterious effect on nutritional status: persistent exocrine pancreatic insufficiency, chronic bacterial pulmonary colonization, impaired glucose tolerance, specific nutritional deficits and associated disorders leading to a decrease of energy intake.