BOLILE PULMONARE INTERSTIŢIALE LA COPIL.

Interstitial lung diseases (ILD) is a heterogenous group of disorders defined by histological alterations in alveolar structures. ILD is a rare group of conditions in children, with a prevalence estimated at 0,36/100.000. ILD in children can be divided into three groups: ILD with known cause, ILD from systemic disorders and ILD with unknown causes. Some formes are encountered unique in children, many of them with hereditary forms and familial clustering. The classic concept regarding the development of ILD combines an individual/genetic predisposition with an initiating agent. Recently the paradigm has shifted away from inflammation to one of tissue injury with aberrant wound healing resulting in collagenous fibrosis. The median age at onset is 8 month. Physical examination may reveal tachypnea, cyanosis, dry cough, retractions, clubbing, fever, wheezing, chest pain, weight loss, signs of pulmonary hypertension and so on. Auscultation may be normal or reveal dry crackles. ILD should be suspected when respiratory symptoms, diffuse infiltrates on the chest radiograph and/or restrictive ventilatory abnormalities with impaired gas exchange persist for more than three month. Chest radiography may be normal in active disease, but typically there are five possible abnormal patterns: ground glass, reticular, nodular, reticulonodular and honeycomb. HRCT has become an integral part of the evaluation of the patient with ILD. Pulmonary function testing are consistent with restrictive physiology. Lung biopsy may become necessary in some cases, but when the HRCT and clinical picture are typical, biopsy is not recommended. The diagnostic begins with history, physical examination, chest radiograph, and lung function tests. On the basis of this information, the patients may be divided into two groups: cases with known causes, and cases that do represent idiopathic ILD. For the "chronic pneumonitis" of unknown cause prednisolone and an antifibrogenic drug like is hidroxycloroquine (Plaquenil) are the treatment of choice. In children, mortality for all types of chronic ILD is 11% and for ILD of unknown origin is 43%. [ABSTRACT FROM AUTHOR]