Interstitial lung abnormalities (ILAs) refer to the subtle or mild signs of ILAs pulmonary parenchyma on chest HRCT scans, which are not yet sufficient to diagnose a certain interstitial lung disease, may be potentially compatible an early stage of the diseases. The signs of ILAs usually includes ground-glass opacities, reticular abnormakicies, honeycombing, traction bronchiectasis or non-emphysematous cysts. This article reviews the research progreses in the definition and classification, risk factors, prognosis, comorbidities and management of ILAs in combination with domestic and foreign literatures.
Humans ; Lung/diagnostic imaging* ; Tomography, X-Ray Computed ; Lung Diseases, Interstitial/diagnosis* ; Prognosis ; Diagnosis, Differential

Diagnostic Imaging ; Humans ; Lung/diagnostic imaging* ; Lung Diseases, Interstitial/diagnosis*

BACKGROUND: Diffuse interstitial lung diseases (DILDs) form a part of a heterogeneous group of respiratory diseases. Bronchoalveolar lavage (BAL) analysis has been used for differential diagnosis of DILDs, but their clinical usefulness is controversial. The aim of this study was to investigate the clinical usefulness of BAL cellular analysis with lymphocyte subsets for the differential diagnosis of DILDs. METHODS: A total of 69 patients diagnosed with DILDs were enrolled. Basic demographic data, BAL cellular analysis with lymphocyte subsets, histology, and high resolution computed tomogram (HRCT) findings were analyzed and compared as per disease subgroup. RESULTS: Significant differences were found between groups in the proportion of neutrophils (P=0.0178), eosinophils (P=0.0003), T cells (P=0.0305), CD4 cells (P=0.0002), CD8 cells (P<0.0001), and CD4/CD8 ratio (P<0.0001). These findings were characteristic features of eosinophilic pneumonia and sarcoidosis. Other parameters were not significantly different between groups. At the cut-off value of 2.16 for sarcoidosis, CD4/CD8 ratio showed sensitivity of 91.7% (95% CI, 61.5-98.6%) and specificity of 84.2% (95% CI, 72.1-92.5%). CONCLUSIONS: Routine analysis of BAL lymphocyte subset may not provide any additional benefit for differential diagnosis of DILDs, except for conditions where BAL is specifically indicated, such as eosinophilic pneumonia or sarcoidosis.
Aged ; Aged, 80 and over ; Area Under Curve ; Bronchoalveolar Lavage Fluid/*cytology ; CD4-CD8 Ratio ; Demography ; Eosinophils/cytology ; Female ; Humans ; Immunophenotyping ; Lung Diseases, Interstitial/*diagnosis/diagnostic imaging ; Lymphocyte Subsets/*cytology ; Male ; Middle Aged ; Neutrophils/cytology ; ROC Curve ; Sarcoidosis/diagnosis ; T-Lymphocytes/cytology ; Tomography, X-Ray Computed

Bronchopulmonary Dysplasia ; diagnostic imaging ; pathology ; therapy ; Female ; Glycogen Storage Disease ; diagnosis ; pathology ; therapy ; Humans ; Infant ; Infant, Newborn ; Lung ; diagnostic imaging ; growth & development ; pathology ; Lung Diseases, Interstitial ; classification ; diagnosis ; pathology ; therapy ; Male ; Neurosecretory Systems ; pathology ; Pediatrics ; Tomography, X-Ray Computed

OBJECTIVETo investigate the high-resolution computed tomographic (HRCT) features of infants with diffuse lung disease (DLD) for improving the diagnostic accuracy clinically.

METHODTotally 75 infants under 2 years of age with DLD (2010-2013) were involved in this study. Among them, 56 were males and 19 females, aged from 2 days to 24 months (mean age was 10.9 months). According to the clinical or pathological data, the cases were enrolled into three groups, including systemic diseases-associated infantile DLD (30 cases), alveolar structure disorders-associated infantile DLD (23 cases), and infantile DLD specific to infancy (22 cases). Retrospectively, HRCT images, from the three groups respectively, were analyzed and compared. HRCT presentations including airway disorders, interstitial disorders and air space disorders were reviewed. Inter-reviewers consistency check was performed, the consistency between reviewers was good (K = 0.64;P = 0.03, < 0.05), as well as χ(2) test.

RESULTAmong the three groups, some of the HRCT sings (bronchiectasis, thickened bronchiolar wall, mosaic sign, reticular, intralobular nodules and consolidations) had significant differences (χ(2) = 24.52, 6.08, 18.00, 12.56, 9.11 and 11.50, P < 0.05) .

CONCLUSIONThe HRCT features of infantile pulmonary DLD/interstitial LD with different causes were as follows, compared to the other two groups, intralobular nodules was the main feature of the systemic diseases-associated infantile DLD, thickened bronchiolar wall, mosaic sign and consolidations were rare as well. Meanwhile, bronchiectasis was more common in alveolar structural disorders-associated infantile DLD, and reticular opacity was rarely seen. Associated clinical data, the HRCT presentations would help clinicians to make accurate diagnosis.

Bronchial Diseases ; diagnostic imaging ; pathology ; Child, Preschool ; Diagnosis, Differential ; Female ; Humans ; Infant ; Infant, Newborn ; Lung ; diagnostic imaging ; pathology ; Lung Diseases, Interstitial ; diagnostic imaging ; pathology ; Male ; Pulmonary Alveoli ; diagnostic imaging ; pathology ; Retrospective Studies ; Tomography, X-Ray Computed ; methods

OBJECTIVETo investigate the association of ATP-binding cassette transporter A3 (ABCA3) gene mutations with severe neonatal respiratory distress syndrome (NRDS) and lung disease in children.

METHODThirty-eight children hospitalized with respiratory disorders in Children's Hospital of Chongqing Medical University from January 2010 to December 2011 were screened. Two mutations (E292V, G1221S) in the ABCA3 gene were identified. Interstitial lung disease (ILD) was present in 10 cases, NRDS was found in 23 and congenital pulmonary dysplasia in 5 cases. There were 24 males and 14 females, with an age range of 1 hour to 15 years. Genomic DNA was prepared from blood samples and sequences were analyzed by polymerase chain reaction (PCR). Clinical feature, imaging characteristics and the results of gene detection were retrospectively analyzed.

RESULTFour cases with ABCA3 gene mutations were found; 2 patients (case 2 and case 4) had the heterozygous mutation of ABCA3 E292V. One was a 3-hour-old girl and another was a 52-day-old boy, 2 patients (case 1 and case 4) had the heterozygous mutation of ABCA3 G1221S. One was a 78-day-old boy and another was a girl, 15 years and one month old. The family history was negative for respiratory disease. Three patients (case 1, 2, 4 ) had NRDS and 2 (case 1, 2) of them were premature. One patient (case 3) had normal growth and development. She was diagnosed clinically as interstitial lung disease (ILD) after admission. The clinical outcomes of 4 cases were various. Case 1 had recurrent wheezing and inhaled corticosteroid was needed. Case 2 died because she failed to wean from mechanical ventilator. Case 3 was discharged with improvement but lost to follow-up. Case 4 grows normally.

CONCLUSIONGenetic variants within ABCA3 may be the genetic causes or background of a contributor to some unexplained refractory NRDS, and chronic lung disease developed in latter childhood. Identification of ABCA3 genetic variants in NRDS infants is important to offer genetic counseling, as well as early prognosis estimation and intervention in pediatric chronic lung disease.

ATP-Binding Cassette Transporters ; genetics ; Adolescent ; Child ; Child, Preschool ; DNA Mutational Analysis ; Exons ; Female ; Heterozygote ; Humans ; Infant ; Infant, Newborn ; Lung ; diagnostic imaging ; pathology ; Lung Diseases, Interstitial ; diagnosis ; genetics ; Male ; Mutation ; genetics ; Radiography ; Respiratory Distress Syndrome, Newborn ; diagnosis ; genetics

Acetates ; therapeutic use ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Hyperplasia ; Infant ; Lung ; diagnostic imaging ; pathology ; physiopathology ; Lung Diseases, Interstitial ; diagnosis ; drug therapy ; physiopathology ; Methylprednisolone ; therapeutic use ; Neuroendocrine Cells ; pathology ; Quinolines ; therapeutic use ; Tomography, X-Ray Computed

Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Humans ; Infant ; Lung ; diagnostic imaging ; pathology ; Lung Diseases, Interstitial ; diagnostic imaging ; pathology ; Male ; Pediatrics ; Pulmonary Fibrosis ; diagnostic imaging ; pathology ; Radiation Dosage ; Radiographic Image Enhancement ; Tomography, X-Ray Computed

OBJECTIVETo report a case of pulmonary surfactant protein (SP) gene mutation associated with pediatric interstitial lung disease, and study the clinical diagnosis process and review of related literature, to understand the relationship between interstitial lung disease and SP gene mutation in infants and children.

METHODThe clinical, radiological, histological, and genetic testing information of a case of SP gene mutation related pediatric interstitial lung disease were analyzed and related literature was reviewed.

RESULTA 2-year-old girl without a history of serious illness was hospitalized because of the shortness of breath, cough, excessive sputum, and the progressive dyspnea. Physical examination on admission revealed tachypnea, slight cyanosis, and the retraction signs were positive, respiratory rate of 60 times/minute, fine crackles could be heard through the lower lobe of both lungs; heart rate was 132 beats/minute. No other abnormalities were noted, no clubbing was found. Laboratory test results: pathologic examination was negative, multiple blood gas analysis suggested hypoxemia. Chest CT showed ground-glass like opacity, diffused patchy infiltration. Bronchoalveolar lavage fluid had a large number of neutrophils, and a few tissue cells. Eosinophil staining: negative. Fluconazole and methylprednisolone were given after admission, pulmonary symptoms and signs did not improve, reexamination showed no change in chest CT. Then lung biopsy was carried out through thoracoscopy. Histopathology suggested chronic interstitial pneumonia with fibrosis. The heterozygous mutation of R219W in the SFPTA1 and the S186N in SFTPC were identified by SP-related gene sequencing. The review of related literature showed that polymorphisms at the 219th amino acid in SP-A1 allele were found in adults with idiopathic pulmonary fibrosis (IPF), but there is no related literature in pediatric cases. The patient in this report had a mutation at the SP-A1 allele consistent with related literature. Data of 17 young children with mutation in SP-C gene showed that all the 17 cases had dyspnea and tachypnea, chest CT revealed diffuse opacities in lungs, the pathology of lungs was NSIP and CPI. There were 17 kinds of mutation and the common mutation was I73T. The mutation of S186N in SFTPC in our case has never been shown in previously published literature.

CONCLUSIONA case of interstitial lung disease with S186N gene mutation in SFTPC was preliminarily diagnosed in an infant. The SP-C gene mutations and polymorphisms are associated with pediatric interstitial lung disease.

Biopsy ; Child, Preschool ; DNA Mutational Analysis ; Dyspnea ; diagnosis ; pathology ; Female ; Humans ; Infant ; Lung ; diagnostic imaging ; pathology ; Lung Diseases, Interstitial ; diagnosis ; genetics ; pathology ; Male ; Mutation ; Pulmonary Surfactant-Associated Protein C ; genetics ; Tomography, X-Ray Computed

Algorithms ; Biopsy, Needle ; Child ; China ; Humans ; Lung ; diagnostic imaging ; pathology ; Lung Diseases, Interstitial ; classification ; diagnosis ; etiology ; Mass Screening ; Societies, Medical ; Tomography, X-Ray Computed