Drug boosts bone density in cystic fibrosis

Current treatments not enough to stave off osteoporosis and fracturesRelated items from OnMedicaAntibiotic fails to improve lung function in children with CFMacrolide antibiotics improve symptoms in bronchiectasisBetter use should be made of current cystic fibrosis treatments Lung transplantation leads to good outcome for cystic fibrosis Cystic fibrosis bug spreads from patient to patient
Source: OnMedica Latest News - Category: UK Health Source Type: news

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ABSTRACT Objective: Bone disease is a common comorbidity in patients with cystic fibrosis (CF). We sought to determine risk factors and identify potential biochemical markers for CF-related bone disease (CFBD) in a unique cohort of CF patients with end-stage lung disease undergoing lung transplantation (LTx) evaluation. Methods: All of the CF patients who were evaluated for LTx at our center between November of 1992 and December of 2010 were included in the study. Clinical data and biochemical markers of bone turnover, as well as bone mineral density (BMD) at the lumbar spine and femoral neck, were evaluated. Spearman &rsq...
Source: Jornal Brasileiro de Pneumologia - Category: Respiratory Medicine Source Type: research
AbstractSummaryPatients with cystic fibrosis awaiting lung transplantation for end-stage respiratory failure have high prevalence of reduced bone mineral density and fragility fracture. Suboptimal 25-hydroxyvitamin D levels could significantly contribute to the development of cystic fibrosis-related bone disease.IntroductionThe assessment of the prevalence of cystic fibrosis-related bone disease (CFBD) and its associated risk factors in young adults with cystic fibrosis (CF) awaiting lung transplantation for end-stage respiratory failure.MethodsClinical characteristics, bone mineral density (BMD), the parameters of calcium...
Source: Osteoporosis International - Category: Orthopaedics Source Type: research
Osteoporosis is a common comorbidity in non-cystic fibrosis (non-CF) bronchiectasis patients. We determined whether desaturation during 6-min walk test (6MWT) can be a predictor for osteoporosis risk.
Source: BMC Pulmonary Medicine - Category: Respiratory Medicine Authors: Tags: Research article Source Type: research
Rationale: Although Cystic Fibrosis (CF) with residual function mutations (RFM) can be associated with less severe disease, no firm conclusions are available on long-term outcomes. Aim of the study was to investigate long-term outcomes in CF adults with ≥1 RFM.Methods: A secondary analysis of the Milan Adult CF Database was performed: it includes consecutively enrolled adults diagnosed following the 2017 CF Foundation guidelines. Patients were referred between 2006 and 2016 to the Adult CF Center of the Policlinico Hospital, Milan, Italy. Patients with ≥1 RFM (Group 1) and with F508del homozygosis or ≥1 class I mu...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Cystic fibrosis Source Type: research
Purpose of review Pancreatic exocrine insufficiency (PEI), defined as a secretion of pancreatic enzymes and bicarbonate insufficient to maintain a normal digestion, is a frequent but frequently underdiagnosed and undertreated condition. PEI may be secondary to different pancreatic diseases and extrapancreatic conditions. Recent data support the high clinical relevance of PEI and its treatment. Recent findings Together with symptoms of maldigestion, PEI is associated with nutritional deficiencies leading to osteoporosis, low-trauma fractures, sarcopenia and increased mortality. No single widely available test allows to...
Source: Current Opinion in Gastroenterology - Category: Gastroenterology Tags: PANCREAS: Edited by Timothy B. Gardner Source Type: research
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Poster Sessions Source Type: research
Conclusions: Osteoporosis in CF occurs with deteriorating clinical status while the role of calcium metabolism seems minor. Gender specific and dysglycemic impact on bone status should be investigated further. PMID: 29780417 [PubMed]
Source: International Journal of Endocrinology - Category: Endocrinology Tags: Int J Endocrinol Source Type: research
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
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Rehabilitation and Chronic Care Source Type: research
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.
Source: Osteoporosis International - Category: Orthopaedics Source Type: research
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]
Source: Gene - Category: Genetics & Stem Cells Authors: Tags: Gene Source Type: research
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