ABSTRACT
The clinical features, imaging findings and pathological manifestations of children diagnosed with acute interstitial pneumonia (AIP) in the Department of Respiratory, Beijing Children′s Hospital, Capital Medical University from January 2016 to December 2017 were retrospectively analyzed.One patient was a girl aged 8 years and 4 months, and the other patient was a boy aged 1 year and 11 months.Both of them had cough and tachypnea for 20 days with transient afebrile.They were diagnosed as Mycoplasma pneumonia and viral pneumonia, respectively, in other hospitals, but the treatment effect was poor.The physical examination results at admission suggested tachypnea, three depression sign (+ ), cyanosis of lips and fingers, no acropachia, and no rales.No abnormality in cardio abdominal and nervous system was detected.Both patients had hypoxemia.The partial pressure of carbon dioxide was normal.The investigations of pathogen were negative.Autoantibody and antineutrophil cytoplasmic antibodies were negative.High resolution CT (HRCT) showed reduced light transmittance of both lungs (especially the lower lung), diffuse bilateral ground glass opacities, consolidation, and traction associated bronchiectasis.The pulmonary histopathology showed di-ffuse alveolar damage, thickened alveolar septum and fibrous tissue in the alveolar cavity.The hyaline membrane was observed in the girl patient.Both patients were treated with corticosteroid.The girl patient had nasal cannula oxygenation, while the boy patient received nasal continuous positive airway pressure (NCPAP) support.They were followed up with improvement.The course of corticosteroid was taped for 8 months and 1 year in the girl patient and boy patient, respectively.After treatment, lung lesions were basically absorbed.
ABSTRACT
Objective@#To summarize the clinical characteristics, virological and histopathological features, clinical outcome of Epstein-Barr virus-positive lymphoproliferative disease (EBV+LPD) in children.@*Methods@#The clinical and follow-up data of 13 children histopathologically diagnosed as EBV+LPD in the Department of Infectious Disease of Beijing Children's Hospital between January 2011 and December 2016 were summarized.@*Results@#Of the 13 patients, 5 were males and 8 females. The median age of disease onset was 6.0 years (range 1.3 to 15.0 years). The median duration between disease onset and diagnosis was 3 months (range 1 to 24 months). All the 13 patients had fever, 9 cases had hepatosplenomegaly and lymphoadenopathy, 4 cases had only lymphoadenopathy, 7 cases had reduced peripheral blood cells, 7 cases had lung involvement, 3 cases had central nervous system involvement, 3 cases had cardiac involvement, 3 cases had intestinal involvement, 2 cases had skin involvement and 1 case had abdominal mass. All the 13 patients underwent whole blood EBV-DNA PCR examination and the copies ranged from 1×108/L to 1×1011/L. Pathology of lymph node confirmed 6 cases, skin pathology confirmed 2 cases, lung pathology, ileum mucosa pathology, liver pathology, abdominal mass pathology and bone marrow pathology confirmed 1 case each. Among 13 patients, 9 cases presented with EBV-positive T cell lymphoproliferative disease(EBV+ T-LPD), 2 cases with hydroa vacciniforme (HV) and 2 cases with EBV-positive diffuse large B-cell lymphoma (EBV+ DLBCL) . All the patients were followed up for 2 days to 65 months after discharge. Among 9 cases of EBV+T-LPD, 1 case died in a short time, 1 case died after evolved to T-cell lymphoma, 2 cases recovered after hematopoietic stem cell transplantation, 1 case recovered after the chemotherapy of hemophagocytic lymphohistiocytosis(HLH) 2004 protocol and 4 cases were stable now. Of 2 cases of HV patients, 1 case died after evolved to HV like lymphoma and the other still have symptoms. Among 2 cases of EBV+ DLBCL, 1 case died shortly after discharge and the other was still stable after chemotherapy.@*Conclusions@#Chronic recurrent fever, lymphadenopathy and hepatosplenomegaly are the most common clinical manifestations in children with EBV+LPD. Involvement of lung, central nervous system, intestinal tract, skin and other organs are also involved frequently. For children with chronic fever of unknown cause and accompanied by lymphadenopathy and (or) hepatosplenomegaly, EBV + LPD should be considered highly when the whole blood EBV-DNA load continues to increase significantly, early biopsy of the proliferative lesion should be performed to make a definite diagnosis. The prognosis of EBV + LPD is poor, and some evolve to lymphoma, hematopoietic stem cell transplantation is an effective way to treat this disease.