Osteoporosis: A New Challenge in Cystic Fibrosis
Osteoporosis: A New Challenge in Cystic Fibrosis
The increased life expectancy of patients with cystic fibrosis (CF) may lead to medical complications such as osteoporosis. Based on data collected through a MEDLINE search (1985-May 1999) and review of references for additional relevant articles, nutrition status, weight, and disease severity are factors most highly correlated with osteopenia. Links also were noted with calcium and vitamin D intake, hypogonadism, chronic inflammation, and age, but findings in these areas are not consistent from one report to the next. Increased fracture rates and kyphosis are consequences of osteoporosis. Simple measures such as compliance with recommended nutrition guidelines and restrictions in corticosteroid therapy could be considered first-line management options. Further studies must be conducted to clarify factors involved in the etiology of osteoporosis in patients with CF and to identify the best treatment and prevention methods.
For many years, cystic fibrosis (CF) was considered a childhood disease because of its high rate of infant mortality and short life expectancy, chiefly due to pulmonary infection complications. In Canada and the United States, the disease occurs in about 1/2000-2500 live births in the Caucasian population (personal communication, Canadian Association of Cystic Fibrosis, 1999). The frequency decreases in other groups, to approximately 1/17,000 among blacks and 1/90,000 among Asians. Publicity helped increase public awareness of what is the most common genetically inherited disease in North America. As a result, more research has been done on CF, and therapies and diagnostic techniques have been developed, increasing the life expectancy of patients who now can survive through adulthood.
With increasing age and long-term pharmaco-therapy, complications such as diabetes, liver disease, infertility, and osteoporosis may develop (with their own complications), affecting as many as 10% of patients. Osteoporosis in this patient population, which until now was recognized only by researchers working in CF, is gaining the attention of clinicians and is likely to continue to do so as the population ages.
Osteoporosis is a condition in which bone frailty results from low bone mass. It may be secondary to osteopenia, a clinically significant decrease in bone mass compared with expected values adjusted for gender and age. Osteomalacia is another disorder characterized by deficient bone mineralization and reduced bone strength. The word osteoporosis is used often to refer to both osteopenia and osteoporosis in the literature, thus it will be used similarly here.
A MEDLINE search of the literature from 1985-May 1999 was conducted to summarize knowledge of the development of osteoporosis in patients with CF. An initial data analysis was carried out, and references were reviewed for additional relevant articles.
The increased life expectancy of patients with cystic fibrosis (CF) may lead to medical complications such as osteoporosis. Based on data collected through a MEDLINE search (1985-May 1999) and review of references for additional relevant articles, nutrition status, weight, and disease severity are factors most highly correlated with osteopenia. Links also were noted with calcium and vitamin D intake, hypogonadism, chronic inflammation, and age, but findings in these areas are not consistent from one report to the next. Increased fracture rates and kyphosis are consequences of osteoporosis. Simple measures such as compliance with recommended nutrition guidelines and restrictions in corticosteroid therapy could be considered first-line management options. Further studies must be conducted to clarify factors involved in the etiology of osteoporosis in patients with CF and to identify the best treatment and prevention methods.
For many years, cystic fibrosis (CF) was considered a childhood disease because of its high rate of infant mortality and short life expectancy, chiefly due to pulmonary infection complications. In Canada and the United States, the disease occurs in about 1/2000-2500 live births in the Caucasian population (personal communication, Canadian Association of Cystic Fibrosis, 1999). The frequency decreases in other groups, to approximately 1/17,000 among blacks and 1/90,000 among Asians. Publicity helped increase public awareness of what is the most common genetically inherited disease in North America. As a result, more research has been done on CF, and therapies and diagnostic techniques have been developed, increasing the life expectancy of patients who now can survive through adulthood.
With increasing age and long-term pharmaco-therapy, complications such as diabetes, liver disease, infertility, and osteoporosis may develop (with their own complications), affecting as many as 10% of patients. Osteoporosis in this patient population, which until now was recognized only by researchers working in CF, is gaining the attention of clinicians and is likely to continue to do so as the population ages.
Osteoporosis is a condition in which bone frailty results from low bone mass. It may be secondary to osteopenia, a clinically significant decrease in bone mass compared with expected values adjusted for gender and age. Osteomalacia is another disorder characterized by deficient bone mineralization and reduced bone strength. The word osteoporosis is used often to refer to both osteopenia and osteoporosis in the literature, thus it will be used similarly here.
A MEDLINE search of the literature from 1985-May 1999 was conducted to summarize knowledge of the development of osteoporosis in patients with CF. An initial data analysis was carried out, and references were reviewed for additional relevant articles.
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