Dr. Dilip Nazareth

ECFS standards of care on CFTR-related disorders: Identification and care of the disorders

1. CFTR-related disorders (RD) are a group of conditions caused by CFTR protein dysfunction, but to a level of dysfuncton not satisfying the diagnostic crtieria for cystic fibrosis (CF). 2. This paper discusses the individual disorders, including congenital absence of the vas deferens (CAVD), diffuse bronchiectasis, chronic or acute recurrent pancreatitis, allergic bronchopulmonary aspergillosis, chronic rhinosinusitis, primary sclerosing cholangitis and aquagenic wrinkling of the palms. 3. The CFTR functional and genetic evidence in support of each condition being a CFTR-RD are discussed and guidance for reaching the diagnosis is provided. 4. Gaps in our knowledge and future areas of research, including the role of CFTR modulators, are highlighted.

Dynamic chest radiography in the workup of lung cancer

Dynamic chest radiography is a low-dose X‑ray technique that captures moving images of the chest and can be applied to several heart and lung imaging tasks. It can measure regional lung blood flow during breath hold, in which case it is termed dynamic perfusion digital radiography. The method uses standard chest-like views taken with the patient standing or lying down, and the images can double as routine chest films before automated analysis. It can also be applied to estimate lung volume compartments, evaluate diaphragm motion, assess ventilation, and track the movement of lung nodules, suggesting a potential role in the imaging assessment of people with lung cancer.

Continuous glucose monitoring systems for monitoring cystic fibrosis-related diabetes

Cystic fibrosis‑related diabetes is a serious complication of cystic fibrosis, but there is still no evidence from suitable trials on how best to use continuous glucose monitoring to guide insulin treatment. The review found no eligible randomised controlled studies, so the benefits or risks of continuous glucose monitoring for this group remain unclear. One relevant trial is in progress and another study is awaiting classification, so future updates may provide answers.