Çocukluk çağında idiyopatik interstisyel pnömoniler: Tanı tedavi ve izlem

Abstract

Aim: There is not an exact consensus about the terminology classification therapy and follow up of childhood interstitial lung diseases because of its rarity and wide clinical spectrum The aims of this study are to describe the clinical features and follow up of our patients with idiopathic interstitial pneumonia on an appropriate terminology and popularize this group of illness because of having difficulties on diagnosis and therapy Material and Method: We investigated the files of patients who were diagnosed as interstitial lung diseases with lung biopsy between 1985 and 2009 retrospectively and the group that is classified as ldquo;idiopathic interstitial pneumonia rdquo; according to the European Respiratory Society Task Force is included Results: Ten patients 6 females 4 males with median age 10 8 years were diagnosed as idiopathic interstitial pneumonia with lung biopsies The most common complaintments were cough sputum expectoration failure to thrive cyanosis dyspnea and reduction on physical exertion Five patients had systemic steroid two patients had systemic steroid and hydroxychloroquine two patients had systemic steroid and cyclophosphamide and one patient had systemic steroid and azathioprine therapies Eight patients could be followed up regularly; three patients improved two patients are stable two patients had recurrence and one patient who had been diagnosed lately has end stage pulmonary disease Conclusions: Any child with a normal birth history presenting with the signs and symptoms suggestive of interstitial lung diseases lasting for three months should be evaluated for this disease Early diagnosis and treatment of childhood ILD has an important effect on prognosis Lung biopsy is necessary for histopathologic diagnosis; but it rsquo;s rarely performed The number of patients will increase with awareness of this disease To constitute appropriate diagnosis therapy and follow up consensus multicenter studies must be done Turk Arch Ped 2013; 48: 281 7