Çocukluk Çağı Diffüz Parankimal Akciğer Hastalıkları: Tanı, Tedavi ve Izlem

Abstract

Childhood diffuse parenchymal lung diseases are a group of heterogeneous disorders that have common histologic features with a chronic course and associated with high morbidity and mortality. To date, there is not an exact consensus about the terminology, classification, therapy and follow up of this disease because of its rarity and wide clinical spectrum. In this study, clinical features, laboratory, radiological and histopathological findings, therapy and outcome of 130 pediatric DPLD cases who were followed up at Hacettepe University Pediatric Pulmonology Department between 1974 and June 2012, are retrospectively evaluated. We also tried to classify these patients according to the last classification scheme recommended in the literature. All clinical and laboratory features and radiological findings at first admission, bronchoscopy and BAL findings, histopathological investigations of any organ biopsies, given therapies, therapy duration and outcome of all patients are recorded. Our childhood DPLD series is the largest one in the literature that’s reported from a single center. Sixteen distinct disease groups are discussed under the DPLD title. Fifteen patients, older than 2 years of age, with idiopathic interstitial pneumonia could not be classified in the last classification scheme recommended by Children’s Interstitial Lung Disease (chILD) Network. Also our patients with idiopathic pulmonary hemosiderosis and pulmonary alveolar microlithiasis stayed out of this classification. A newly defined congenital lung disease, diffuse chondroid malformation of the lung, was not defined in the available classification scheme. These results indicate that a new classification system involving all these patients is required. Our patients’ mean age at diagnosis was 7,7 years. The most frequent symptoms were cough and dyspnea in all disease groups. The diagnosis of patients were delayed between median 5,5 months and 3 years in all groups; and these patients were treated as if they had recurrent pneumonia, tuberculosis and asthma. As the presenting clinical manifestations are non-specific, every child, with a history of normal birth, who has signs and symptoms suggesting DPLD for more than 3 months should be investigated in terms of DPLD.