Pulmonary hypertrophic osteoarthropathy (PHOA) in cystic fibrosis has not been previously described in the English language, except for one patient mentioned briefly by Caffey.1 It is our purpose to present two patients with cystic fibrosis and symptomatic PHOA, and to give a preliminary report on the incidence of asymptomatic PHOA in cystic fibrosis. A brief discussion as to possible etiological factors of PHOA will also be given.
PHOA is extremely rare in children; only ten cases were reported in the American and British literature up to 19602; since then none has been reported. Clubbing is a common finding in cyanotic congenital heart disease and bronchiectasis, but PHOA is rarely associated with the former3 and only occasionally with the latter. Clubbing and PHOA are felt by many to have a similar pathogenesis, and the former represents a more advanced state of the process.2,4,5 While it is true
GROSSMAN H, DENNING CR, BAKER DH. Hypertrophic Osteoarthropathy in Cystic Fibrosis. Am J Dis Child. 1964;107(1):1-6. doi:10.1001/archpedi.1964.02080060003001