Drug boosts bone density in cystic fibrosis
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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...
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]
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.