• A questionnaire survey of 375 patients with cystic fibrosis was performed to seek evidence of pulmonary hypertrophic osteoarthropathy (PHOA). Three hundred responses were obtained. Forty-five patients (15%) who described long-bone or joint pain met clinical criteria for the diagnosis of PHOA, 25 of whom had roentgenographic evidence of periostitis. Compared with an age-and sex-matched control group (group 3), the patients with cystic fibrosis and PHOA as well as roentgenographic evidence of periostitis (group 1) had worse Shwachman scores and pulmonary function and a significantly increased mortality rate (36%). The patients with PHOA but no roentgenographic evidence of periostitis (group 2) had Shwachman scores intermediate between groups 1 and 3. We believe PHOA is more common than previously suspected. Its incidence appears related to severity of disease.
Cohen AM, Yulish BS, Wasser KB, Vignos PJ, Jones PK, Sorin SB. Evaluation of Pulmonary Hypertrophic Osteoarthropathy in Cystic Fibrosis: A Comprehensive Study. Am J Dis Child. 1986;140(1):74–77. doi:10.1001/archpedi.1986.02140150076042
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