Abstract

BackgroundCystic fibrosis is the most common fatal autosomal recessive genetic disease in the Caucasian population. Osteoporosis is increasingly being recognised as an important complication in people with cystic fibrosis.MethodsA descriptive study of adult cystic fibrosis patients receiving care at a Canadian tertiary care hospital was conducted to evaluate the prevalence of osteoporosis, the prevalence of non-vertebral fractures, and the change in bone mineral density during the course of a year. Data on bone mineral density were obtained for 40 adult cystic fibrosis patients by reviewing dual x-ray absorptiometry scans taken at baseline (when annual scans became standard clinical practice) and one year prior to baseline. Data on prevalent fractures were obtained by reviewing all available patient charts. Clinical and laboratory data were collected from an existing clinic database.ResultsOver half of the 40 patients had reduced T- and Z-scores at baseline. For the 27 patients who had data available one year prior to baseline, total hip and lumbar spine bone mineral density had decreased by 3.04% and 0.86% after one year while total body bone mineral density had not changed significantly. Four prior non-vertebral fractures were reported in three patients (1,146 patient-years).ConclusionThis study confirms that osteoporosis is a significant problem in adult cystic fibrosis patients, and constitutes the first published evidence of cystic fibrosis bone disease in Canadians.

Highlights

  • Cystic fibrosis is the most common fatal autosomal recessive genetic disease in the Caucasian population

  • Advances in medical science have increased life expectancy for Cystic fibrosis (CF) patients and as a result long-term sequelae of the disease such as osteoporosis and liver disease are becoming apparent in late adolescence and into adulthood

  • Study Population dual x-ray absorptiometry (DXA) scans are routinely performed on all patients attending the adult CF clinic

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Summary

Introduction

Cystic fibrosis is the most common fatal autosomal recessive genetic disease in the Caucasian population. Osteoporosis is increasingly being recognised as an important complication in people with cystic fibrosis. Cystic fibrosis (CF) is the most common fatal autosomal affecting 30,000 Americans and about the same number (page number not for citation purposes). Advances in medical science have increased life expectancy for CF patients and as a result long-term sequelae of the disease such as osteoporosis and liver disease are becoming apparent in late adolescence and into adulthood. There is an increased prevalence of osteopenia and osteoporosis in CF patients compared with the general population. Studies have estimated that between 24 to 58% of adult CF patients are osteopenic [6,7,8,9,10,11,12]

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