ABSTRACT
Pulmonary function testing is a vital tool in evaluation and management of adult ILD patients and is rarely overlooked during workup. However, there is paucity of data regarding its usefulness in management of infants with suspected interstitial lung disease. In this paper, we present the contribution of infant pulmonary function testing (iPFT) to the management of two infants with biopsy confirmed chronic pneumonitis of infancy due to surfactant protein C mutation. We have productively and safely used serial iPFT for decision making both during diagnosis and follow-up of these infants.
Subject(s)
Lung Diseases, Interstitial/diagnosis , Mutation , Pneumonia/diagnosis , Pulmonary Surfactant-Associated Protein C/genetics , Respiratory Function Tests , Female , Humans , Infant , Lung Diseases, Interstitial/genetics , Lung Diseases, Interstitial/physiopathology , Pneumonia/genetics , Pneumonia/physiopathologyABSTRACT
Interstitial lung disease, mostly chronic lung disorder, is usually characterized by diffuse infiltrates and disordered gas exchange, is a very rare disease in children. Chronic pneumonitis of infancy (CPI) is a recently described entity representing a distinct form of interstitial lung disease affecting infants and young children. The histologic findings suggest marked alveolar septal thickening, striking alveolar pneumocyte hyperplasia, and alveolar exudates containing numerous macrophages and foci of eosinophilic debris. A 3-year-old boy visited a local clinic with cough and tachypnea for a duration of 3 weeks. His clinical symptoms were aggravated and he was referred to our hospital. He was diagnosed with chronic pneumonitis of infancy after undergoing open lung biopsy. We herein present the case with review of literature.
Subject(s)
Child , Child, Preschool , Humans , Infant , Male , Biopsy , Cough , Eosinophils , Exudates and Transudates , Hyperplasia , Lung , Lung Diseases, Interstitial , Macrophages , Alveolar Epithelial Cells , Pneumonia , Rare Diseases , Strikes, Employee , TachypneaABSTRACT
Chronic pneumonitis of infancy (CPI) is a very rare interstitial lung disease. Its pathological features differ from other types of interstitial pneumonia that occur in adults and children. The mortality rate of CPI is high, even with treatment. We report a case of a 3 month old girl diagnosed with CPI after an open lung biopsy who improved after proper treatment.
Subject(s)
Female , Humans , Infant , Biopsy , Lung Diseases, Interstitial/diagnosis , Pneumonia/diagnosis , Tomography, X-Ray ComputedABSTRACT
The interstitial lung diseases(ILD) comprises a large, heterogeneous group of disorders characterized by derangement of alveolar walls and alveolar capillary unit causing restrictive pulmonary dysfunction and disordered gas exchange. It is rare, especially in children, and the opinions on investigation and treatments are controversial. The classification of adult ILD is applied to that of children. But considering occurrence during the developing state of lung and immune systems, we should be more cautious because the clinical manifestations and prognosis will be different from those of adults. In 1995, Katzenstein named a unique type of ILD, chronic pneumonitis of infancy (CPI) for the first time. CPI is characterized by marked alveolar septal thickening, striking type II pneumocyte hyperplasia, and eosinophilic alveolar exudate containing numerous macrophages. We report a 14 month-old girl, who had continuing respiratory difficulty and cyanosis after upper respiratory infection, could not sustain oxygen saturation and was finally diagnosed as CPI by histologic examination.
