Drug boosts bone density in cystic fibrosis
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We examined whether desaturation during 6 minute walk test (6MWT) was associated with increased osteoporosis and hospitalization in NCFBr.We enrolled 49 NCFBr and measured FEV1, 6MWT, and oxygen saturation by pulse oximetry (SpO2) during 6MWT at baseline and every 3-6 moths for 5 years. Desaturation group was defined as a fall in SpO2≥10% or
ConclusionIn our cohort of children and teenagers with good nutritional and lung function status, bone microstructure evaluated with HR-pQCT was not severely affected. Minimal microstructure abnormalities observed at the tibial level may be related to the cystic fibrosis transmembrane conductance regulator defect alone; the long-term consequences of such impairment will require further evaluation.
CONCLUSIONS: The clinical and laboratory variability of CF were associated with the variants in the genes of SLC family in our sample. PMID: 28756021 [PubMed - as supplied by publisher]
The objectives of this study were, in a current adult CF population, to assess the prevalence of bone disease, to examine its relationship with infections and inflammation, and to characterize the bone microarchitecture using high resolution peripheral scanner (HR-pQCT). Methods Fifty-six patients (52 % men, 26 ± 7 years) were assessed in clinically stable period, during a respiratory infection, and finally 14 days after the end of antibiotic therapy. At each time points, we performed a clinical evaluation, lung function tests, and biochemical test...
Optimal growth and nutrition are essential for effective treatment of individuals with cystic fibrosis (CF). However, the maldigestion and malabsorption of classic CF coupled with increased energy needs of pulmonary disease and complicated by comorbidities such as CF-related diabetes, osteoporosis, liver disease, and/or depression can result in challenges for the implementation of a successful nutrition management plan. Nutrition therapy of this complex and progressive disease has evolved along with the overall treatment over the 8 decades since CF was first recognized.
We report a case of a young woman with CF, lung transplantation and low bone mass developing long-term leukopenia after treatment with zoledronic acid. The leukopenia, with a strong affection of the neutrocytes, lasted for 4 months and the condition only went into remission after granulocyte-colony stimulating factor (G-CSF) treatment. It is important to be aware of symptomatic leukopenia in immunosuppressive patients after treatment with zoledronic acid.
CONCLUSIONS: There is evidence to indicate an increase in bone mineral density at the femoral neck, lumbar spine and forearm after administration of bisphosphonates and at the lumbar spine and hip after zinc sulphate supplementation. The authors recommend that further long-term randomised control trials on different bisphosphonates and zinc supplementation therapies in people with beta-thalassaemia and osteoporosis are undertaken. PMID: 26964506 [PubMed - as supplied by publisher]
Abstract Summary Young adults with cystic fibrosis have compromised plate-like trabecular microstructure, altered axial alignment of trabeculae, and reduced connectivity between trabeculae that may contribute to the reduced bone strength and increased fracture risk observed in this patient population. Introduction The risk of fracture is increased in patients with cystic fibrosis (CF). Individual trabecular segmentation (ITS)-based morphological analysis of high-resolution peripheral quantitative computed tomography (HR-pQCT) images segments trabecular b...
Conclusions Male sex, short stature, and low lean mass are associated with low BMD in CF. Greater adiposity and lower lung function are predictors of negative change in BMD Z-score over 2 years.