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.
Biopsy ; Female ; Humans ; Infant ; Lung Diseases, Interstitial/*diagnosis/drug therapy/pathology ; Pneumonia/*diagnosis/drug therapy/pathology ; Tomography, X-Ray Computed

OBJECTIVEStreptococcus pneumoniae necrotizing pneumonia (SPNP) was reported elsewhere but not in China yet. Inappropriate treatment due to poor recognition of this disease could influence its prognosis. This paper presents the clinical characteristics, diagnosis and treatment of SPNP hoping to elevate pediatrician's recognition level for this disease.

METHODClinical manifestations, radiological findings, treatment and prognosis of 20 patients (9 boys, 11 girls) who had been hospitalized with SPNP in Beijing Children's Hospital from 2004-2011 were retrospectively analyzed.

RESULTThe patients included in this study aged from 9 months to 6 years [(27.9 ± 15.8) m] and were healthy before admission. They were febrile for 8 to 50 days [(27.7 ± 13.5) d] and hospital day was 24 - 55 days [(36.5 ± 8.3) d]. The general condition of all subjects was relatively poor and they all had fever and cough. One child had moderate fever and nineteen children had high fever. Dyspnea was found in sixteen children. Fine rales were found on auscultation in 18 children, among whom diffuse wheeze appeared in 4 children, and wheezy phlegm was found in two children. Signs of pleural effusion were discovered in all cases by physical examination and chest X-ray. White blood cell (WBC) count was 16.2 - 60.95×10(9)/L and neutrophil was 70.5% - 80.2% in peripheral blood routine test. Erythrocyte sedimentation rate (ESR) was 44 - 109 mm/h [(69.6 ± 16) mm/h]and C-reactive protein (CRP) was 80 - > 160 mg/L. The pleural effusion biochemistry and routine test revealed a WBC count of 6400×10(6)/L-too much to count, polykaryocyte of 51% - 90%, glucose of 0.02 - 1.8 mmol/L, protein of 32 - 51 g/L and LDH of 5475 IU/L-or higher. Pleural effusion culture in all cases and blood culture in 2 cases was positive for Streptococcus pneumoniae. Chest X-ray or CT revealed high density and well-distributed lobar consolidation in one lung or two lungs initially. Single or multiple low density lesions in the area of lobar consolidation were found a week later, accompanied by multiple cystic shadow or cavity at the same time or afterwards. Bulla of lung appeared later. Pleural effusions were found in all patients. Seven cases complicated with hydropneumothorax, two with otitis media, one with heart failure, one with cardiac insufficiency. Seventeen patients were treated with vancomycin or teicoplanin or linezolid two with amoxicillin and clavulanate potassium. Other two patients had been treated with meropenem and cephalosporin antibiotics respectively before admission, and they had been at recovery stage when they were hospitalized. Thoracic close drainage and thoracoscopy were performed respectively in 18 cases and 3 cases, respectively. After a follow up of more than 6 months, chest CT showed that almost all lesions in lungs recovered during 4-6 months. No one received pneumonectomy.

CONCLUSIONSPNP has special manifestations. The incidence in infants is higher. Patients' general condition is poor and febrile course is relatively long. All patients manifested fever and cough, with a presence of dyspnea in most of them. WBC, neutrophil and CRP elevated apparently. The characteristic of pleural effusion indicates empyema. In early stage, the chest X-ray and CT showed high-density lobar lesions, followed by low-density lesions and cyst gradually. Bulla of lung and/or hydropneumothorax may appear at the late stage. But if diagnosed and treated promptly, the prognosis of SPNP was relatively good.

Anti-Bacterial Agents ; therapeutic use ; Child ; Child, Preschool ; Drug Resistance, Bacterial ; Dyspnea ; diagnosis ; drug therapy ; epidemiology ; Female ; Fever ; diagnosis ; drug therapy ; epidemiology ; Humans ; Infant ; Leukocyte Count ; Lung ; diagnostic imaging ; pathology ; Male ; Methylprednisolone ; therapeutic use ; Pleural Effusion ; diagnosis ; drug therapy ; epidemiology ; Pneumonia, Pneumococcal ; complications ; diagnosis ; drug therapy ; Prognosis ; Retrospective Studies ; Streptococcus pneumoniae ; drug effects ; isolation & purification ; pathogenicity ; Tomography, X-Ray Computed ; Treatment Outcome

OBJECTIVETo analyze clinical manifestations, treatment and prognosis of 4 cases with endemic typhus.

METHODThe clinical data of four endemic typhus patients in prognosis were retrospectively analyzed. These four atypical cases of endemic typhus with pneumonia were treated in our department from October 2011 to March 2012. They were all male, with an age range of 15 months to 7 years. The four patients had long history, mild respiratory symptom and no improvement was found after treatment with cephalosporins. There were no evidences of bacterial, viral, or fungal infections and we thought they might have infection with other pathogen. Three were from rural areas. Routine blood tests, Weil-Felix reaction, blood smear (Giemsa staining) , and indirect immunofluorescence assay were performed.

RESULTBlood smear and IFA tests showed evidences for endemic typhus. The clinical presentations were atypical, the patients had no headache, but all had fever, rash, and pneumonia of varying severity. None of the patients had a severe cough, but bronchial casts were observed in one case. Recurrent fever was reported in three cases. Physical examinations showed no eschars, but one patient had a subconjunctival hemorrhage, and one had skin scratches, cervical lymphadenopathy, pleural effusion, pericardial effusion, and cardiac dilatation. Two patients had remarkably increased peripheral blood leukocyte counts; both these patients also had high alanine aminotransferase (ALT) levels and one had a high C-reactive protein (CRP) level. Weil-Felix testing was negative or the OX19 titer was low. The peripheral blood smear (Giemsa stain) showed intracellular pathogens in all four cases. After combined therapy with doxycycline and macrolide antibiotics, all four patients recovered well.

CONCLUSIONThe endemic typhus children often come from rural areas. The clinical presentations were atypical, they usually have no headache, but have fever (often Periodic fever) , rash, and pneumonia of varying severity in these four cases. Combined therapy with doxycycline and macrolide antibiotics was effective in all four patients.

Anti-Bacterial Agents ; therapeutic use ; Child ; Child, Preschool ; Doxycycline ; therapeutic use ; Fever ; diagnosis ; drug therapy ; pathology ; Fluorescent Antibody Technique, Indirect ; Humans ; Infant ; Leukocyte Count ; Lung ; diagnostic imaging ; pathology ; Macrolides ; therapeutic use ; Male ; Pneumonia ; diagnosis ; drug therapy ; pathology ; Radiography ; Retrospective Studies ; Staining and Labeling ; methods ; Typhus, Endemic Flea-Borne ; diagnosis ; drug therapy ; pathology

Aged ; Echinocandins ; administration & dosage ; therapeutic use ; Humans ; Leukemia, Myelomonocytic, Chronic ; pathology ; Lipopeptides ; Male ; Pneumonia, Pneumocystis ; diagnosis ; drug therapy ; pathology ; Trimethoprim, Sulfamethoxazole Drug Combination ; administration & dosage ; therapeutic use ; Uremia ; pathology

A case of dermatomyositis presented as bronchiolitis obliterans organizing pneumonia has been rarely reported. We describe a 46-year-old female patient with dermatomyositis without elevation of creatine kinase presented as bronchiolitis obliterans organizing pneumonia. She was treated with prednisolone and azathioprine. Over a 2-year follow-up she has had no elevation of creatine kinase. The patient remains asymptomatic and has no medication for dermatomyositis and bronchiolitis obliterans organizing pneumonia two years after initial treatment. It has been suggested that the prognosis of dermatomyositis without creatine kinase elevation may be poor. Because the prognosis of bronchiolitis obliterans organizing pneumonia is generally believed to be good, we tentatively suggest that the normal value of creatine kinase in dermatomyositis does not always seem to herald a poor prognosis, an associated malignancy or severe interstitial lung disease.
Azathioprine/administration +ACY- dosage ; Biopsy, Needle ; Bronchiolitis Obliterans Organizing Pneumonia/pathology ; Bronchiolitis Obliterans Organizing Pneumonia/diagnosis+ACo- ; Case Report ; Creatine Kinase/blood+ACo- ; Dermatomyositis/pathology ; Dermatomyositis/enzymology ; Dermatomyositis/drug therapy ; Dermatomyositis/diagnosis+ACo- ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Human ; Middle Age ; Prednisone/administration +ACY- dosage ; Tomography, X-Ray Computed

Anti-Bacterial Agents ; administration & dosage ; therapeutic use ; Anti-Inflammatory Agents ; therapeutic use ; Azithromycin ; administration & dosage ; therapeutic use ; Biomarkers ; blood ; Child ; Eyelids ; pathology ; Humans ; Immunoglobulin M ; blood ; Lip ; pathology ; Male ; Methylprednisolone ; administration & dosage ; therapeutic use ; Mucositis ; diagnosis ; drug therapy ; microbiology ; Mycoplasma pneumoniae ; drug effects ; isolation & purification ; Pneumonia, Mycoplasma ; complications ; diagnosis ; drug therapy

Various forms of hypogammaglobulinemia can occur in patients with autoimmune diseases and vice versa. We report a 13-yr-old boy with membranous nephropathy and common variable immunodeficiency. He presented with the nephrotic syndrome, pneumonia with bronchiectasis, and profound hypogammaglobulinemia. Renal biopsy showed diffusely thickened glomerular capillary walls with 'spikes' suggesting a membranous nephropathy. Secondary causes were ruled out by laboratory studies; however, heavy proteinuria persisted with steroid therapy. Cyclosporine and intravenous immunoglobulin were added, and the patient was discharged with decreased proteinuria. Hypogammaglobulinemia may have a deleterious impact on the immune dysregulation in some patients with membranous nephropathy.
Adolescent ; Bronchiectasis/etiology ; Common Variable Immunodeficiency/complications/*diagnosis/drug therapy ; Cyclosporine/therapeutic use ; Drug Therapy, Combination ; Glomerulonephritis, Membranous/complications/*diagnosis/drug therapy ; Humans ; Immunoglobulins/therapeutic use ; Immunosuppressive Agents/therapeutic use ; Injections, Intravenous ; Kidney/pathology ; Male ; Pneumonia/etiology ; Proteinuria/etiology ; Steroids/therapeutic use

Interstitial lung disease is a generic term for a heterogeneous group of lung disease that primarily affect the interstitium although the disease is not clearly restricted to the interstitium. The majority of interstitial lung diseases represent inflammatory insults to the microscopic anatomic space bounded by the basement membrane of epithelial and endothelial cells, which may occur as slowly developing process and ultimately end up as end-stage honeycomb fibrosis. The currently prevalent classification of interstitial pneumonia with practical utility and easy reproducibility pertaining only to idopathic interstitial pneumonia encompasses several different entities some of which may represent different aspects of the same condition. Honeycomb fibrosis is usually caused by a variety of pulmonary disease including chronic interstitial lung disease. It is important to recognize that usual inter-stitial pneumonia and honeycomb fibrosis are not synonymous. In the era of chemotherapy for malignant tumor, aggressive immunosuppression for autoimmune diseases and transplant recipients and acquired immunodeficiency syndrome, lung disease in the immunocompromised host has been common. Diagnostic lung biopsy becomes increasingly needed because proper treatment of interstitial lung disease relies on correct morphologic diagnosis. This review summarizes the pathologic spectrum of idiopathic interstitial pneumonias together with other inflammatory process with known or suggestive etiologies simulating interstitial pneumonias.
Acquired Immunodeficiency Syndrome ; Autoimmune Diseases ; Basement Membrane ; Biopsy ; Classification ; Diagnosis ; Drug Therapy ; Endothelial Cells ; Fibrosis ; Idiopathic Interstitial Pneumonias ; Immunocompromised Host ; Immunosuppression ; Lung ; Lung Diseases ; Lung Diseases, Interstitial* ; Pathology* ; Pneumonia ; Transplantation

OBJECTIVETo investigate clinical characteristics and changes of pulmonary imaging of mineral oil aspiration pneumonia in children.

METHODThe clinical features, CT findings, and effects of corticosteroid therapy were analyzed in 16 children with mineral oil aspiration pneumonia, who were hospitalized in our hospital from January 2003 to July 2013.

RESULTAll patients with mineral oil aspiration pneumonia had a history of mineral oil administration.Four patients had no clinical manifestations. Ten cases presented fever, and 8 of the 10 patients had fever in 4-8 h after taking mineral oil, and the temperature was between 39-40 °C. There were wheezing in 2 cases, shortness of breath in 6 cases, cyanosis in 1 case, dyspnea in 3 cases, and moaning in 2 cases, chest pain in 1 case, headache and abnormal EEG in 1 case.Six patients had rales in lungs. Peripheral blood white cells increased in 10 cases, and C- reactive protein elevated in 7 patients. Chest CT examination showed abnormal findings in 6 children, and the earliest CT was performed within 2 h after the accident. The rest 10 children got chest X-ray, and 9 of 10 children had abnormal findings. The earliest X-ray was done within 3 h after the accident. And the remaining 1 of 10 children showed no significant changes in the first chest X-ray 2-3 h after the accident until 3 days. All of the patients received corticosteroid and antibiotic treatments, 4 cases underwent bronchoalveolar lavage, 3 patients were given albumin, 6 cases received intravenous immunoglobulin. Three cases delayed in treatment with hormone because of misdiagnosis, and 2 of them had clearly secondary infections. Twelve patients recovered completely from oil aspiration pneumonia after 8 days to 5.5 months.

CONCLUSIONOil aspiration pneumonia in children occurs in almost all cases after mineral oil aspiration. Pulmonary opacities can be found by chest CT in most patients within 24 hours after mineral oil aspiration. Corticosteroids therapy was effective for patients with exogenous lipid pneumonia, which may inhibit the inflammatory response and possible pulmonary fibrosis.

Anti-Bacterial Agents ; therapeutic use ; C-Reactive Protein ; analysis ; Child, Preschool ; Diagnosis, Differential ; Female ; Fever ; diagnosis ; drug therapy ; Glucocorticoids ; therapeutic use ; Humans ; Infant ; Lung ; diagnostic imaging ; pathology ; Male ; Mineral Oil ; adverse effects ; Pneumonia, Lipid ; diagnosis ; drug therapy ; etiology ; Radiography, Thoracic ; Retrospective Studies ; Tomography, X-Ray Computed

OBJECTIVETo analyze the clinical manifestations of refractory Mycoplasma pneumoniae pneumonia (RMPP) which unresponded to methylprednisolone in the dosage of 2 mg/(kg·d) for 3 days.

METHODRetrospective analysis was performed on the clinical data of 110 children (64 boys and 46 girls) with RMPP. The patients were divided into "effective group" and "ineffective group" according to initial effect of 2 mg/(kg·d) methylprednisolone. The clinical manifestations, laboratory examination, radiological features and bronchofibroscopic findings of the children were compared. In order to seek the reference indexes which indicate nonresponsive to 2 mg/(kg·d) methylprednisolone, an ROC curve was made, of which the diagnostic cut-off was five independent correlation factors while grouping was made according to patients' different response to glucocorticosteroid.

RESULTThe effective group had 86 (86/110, 78.2%) children while ineffective group had 24 (24/110, 21.8%). The ineffective group children had the following performance: 16 children (16/24, 66.7%) in ineffective group had ultrahyperpyrexia (T ≥ 40 °C), which was significantly more severe compared to those in effective group (32/86, 37.3%, P < 0.01); the levels of white blood cell (WBC) count, percentage of neutrophils count (N), C-reactive protein (CRP), serum ferritin (SF), alanine transaminase (ALT), lactic dehydrogenase (LDH), creatine kinase isoenzyme (CK-MB) and fibrinogen (Fib) in ineffective group were significantly higher than those in effective group(P < 0.01); while percentage of lymphocyte count (L) was lower than that in effective group(P < 0.01). Proportion of mixed infection in ineffective group was higher than that in effective group (33.3% vs. 4.7%). Radiological manifestations: It was more frequently seen in ineffective group that chest CT scan indicated high density consolidation in no less than a whole pulmonary lobe and pulmonary necrosis (41.7% vs. 0%). Abundant secretions blockage (45.0% vs. 16.9%) and mucosal necrosis (37.5% vs. 8.1%) on bronchofibroscopy were more frequently seen in ineffective group. The critical values of the five independent correlation factors were CRP 110 mg/L, SF 328 mg/L, LDH 478 IU/L, N 0.78, L 0.13.

CONCLUSIONTreatment with 2 mg/(kg·d) methylprednisolone can improve clinical symptoms and radiological manifestations of most children with RMPP quickly, but it may be ineffective in some situations such as lasting high fever or ultrahyperpyrexia for more than 7 days, CRP ≥ 110 mg/L, N ≥ 0.78, L ≤ 0.13, serum LDH ≥ 478 IU/L, SF ≥ 328 µg/L, chest CT scan indicating high density consolidation in more than a whole pulmonary lobe involved and moderate-abundant pleural effusion.

Adrenal Cortex Hormones ; administration & dosage ; therapeutic use ; Anti-Bacterial Agents ; administration & dosage ; therapeutic use ; Bacterial Infections ; drug therapy ; epidemiology ; C-Reactive Protein ; analysis ; Child ; Child, Preschool ; Coinfection ; Female ; Ferritins ; blood ; Fever ; diagnosis ; drug therapy ; Humans ; Infusions, Intravenous ; Leukocyte Count ; Lung ; diagnostic imaging ; pathology ; Male ; Methylprednisolone ; administration & dosage ; therapeutic use ; Mycoplasma pneumoniae ; Pneumonia, Mycoplasma ; blood ; diagnosis ; drug therapy ; Radiography, Thoracic ; Retrospective Studies ; Tomography, X-Ray Computed ; Treatment Outcome