Effect of 18 months elexacaftor-tezacaftor-ivacaftor on body mass index and glycemic control in adults with cystic fibrosis.

BACKGROUND & AIMS: Malnutrition and cystic fibrosis related diabetes (CFRD) are common comorbidities in cystic fibrosis (CF). Cystic fibrosis transmembrane regulator (CFTR) modulators have shown beneficial effects on respiratory status. This study aims to determine the effect of elexacaftor-tezacaftor-ivacaftor (ETI) on body mass index (BMI) and glycemic control. METHODS: A retrospective, observational study of a cohort of 17 adult CF patients was conducted at the CF reference center of Ghent University Hospital. BMI evolution was analyzed 18 months before and 0, 3, 6, 12 and 18 months after the start of ETI. The evolution of insulin dependence and the 2 h oral glucose tolerance test (OGTT) results were described until 36 months after start of ETI, in a small subgroup of ten patients with CFRD or impaired glucose tolerance (IGT). RESULTS: A significant increase in mean BMI of 1.2 kg/m2 (±1.3 SD) was observed. Most weight gain was observed in the first 3 months after starting treatment. This effect was sustained during the observed period of 18 months. Six patients had insulin dependent CFRD, of which three were able to stop insulin after starting ETI. Two patients with CFRD treated with dietary measures showed an initial normalization of the 2 h OGTT, but deterioration at 36 month follow-up. CONCLUSIONS: After initiation of ETI an increase in BMI was observed in adults with CF. ETI can have a beneficial impact on glucose metabolism in patients with CFRD, leading to a possible need for reduction or cessation of insulin therapy.

Metastatic malignant struma ovarii: a case report and review of the literature on the management of malignant struma ovarii.

OBJECTIVES: To present a case of metastatic struma ovarii, to review the literature on malignant struma ovarii and to discuss the management in locoregional and metastatic disease. METHODS: We present a case of an 82-year-old patient with a malignant struma ovarii and liver metastasis. The patient was treated with pelvic surgery, total thyroidectomy, radioactive iodine therapy and TSH suppression therapy with levothyroxine. We performed a PubMed search for case reports of metastatic struma ovarii. RESULTS: 43 cases of metastatic struma ovarii were identified. 53.5% of patients presented with metastatic disease at diagnosis. Mean time to development of metastasis was 6.9 years in the group with initial locoregional disease. First-line treatment was pelvic surgery in all patients. Thyroidectomy was performed in 83.7% of patients, subsequent radioactive iodine therapy in 79.1%, followed by TSH suppression therapy in 46.5% of patients. Mean time of follow-up after diagnosis of metastases was 3.6 years, ranging from 0.5 to 24 years. At the end of the follow-up, 51.1% of patients were free of disease, 34.9% were alive with disease, 7.0% died of disease and 7.0% were lost to follow-up. CONCLUSION: The majority of patients with metastatic struma ovarii were treated with pelvic surgery, total thyroidectomy and radioactive iodine therapy. Suppression of TSH with levothyroxine was given in less than half of the patients. In non-metastatic setting, the same approach could be considered depending on the patient profile.