OBJECTIVEAcute interstitial pneumonia (AIP) is a rare lung interstitial disease in children. This study was conducted to understand the clinical features of the AIP in children.

METHODThe data of the three cases with AIP admitted to our hospital from March 2008 to November 2009 were reviewed. Of the 3 cases, 2 were male, one was female. Their age ranged from 1 year and 4 months to 10 years. The clinical manifestation, pulmonary function test and the high resolution computed tomography (HRCT) and pathology of the lung were studied retrospectively. Tissue specimens of the lung were obtained by video-assisted thoracoscopic biopsy. Viral etiologic examinations for the respiratory syncytial virus, adenovirus, influenza virus, parainfluenza viruses, EB virus, cytomegalovirus, enterovirus and herpes simplex virus were performed. The IgM antibody to Mycoplasma pneumoniae in the serum was also detected.

RESULTAll the 3 cases rapidly developed respiratory failure of unknown origin, none of these cases had failure of any other organs. All three cases had cough and dyspnea. No case had the rales and digital clubbing. The examinations for viruses, bacteria and Mycoplasma pneumoniae infection were all negative. No evidence for the diagnosis of connective tissue disease was obtained. The HRCT of the chest showed diffuse alveolar consolidation, air bronchogram and ground glass appearance in the bilateral lungs, and the traction-associated bronchiectasis in areas. All the three cases had the histological proof of diffuse alveolar damage by the biopsy. All the three cases were treated with CPAP and corticosteroid. Two cases were treated with corticosteroid in early stage of the disease, the condition of these cases were improved obviously. The third case was treated with high-dose steroid pulse therapy days, the condition of this case was improved slightly in a month. One year follow-up showed that case 1 and case 2 had no hypoxemia and the HRCT of the chest showed obvious improvement. The pulmonary function of case 2 had restrictive deficiency.

CONCLUSIONAIP has a rapidly progressive clinical course leading to respiratory failure. The HRCT of the chest showed alveolar consolidation and ground glass-like change. The pathology of the lung includes diffuse alveolar damage. The prognosis of the AIP in children may be improved by the treatment with respiratory assistance and corticosteroids.

Acute Disease ; Child ; Child, Preschool ; Female ; Humans ; Lung Diseases, Interstitial ; diagnosis ; pathology ; therapy ; Male ; Respiratory Insufficiency

No abstract available.
Diagnosis, Differential ; Doxycycline/therapeutic use ; Female ; Guillain-Barre Syndrome/*diagnosis/pathology ; Humans ; Immunoglobulins/therapeutic use ; Male ; Middle Aged ; Respiration, Artificial ; Respiratory Insufficiency/*diagnosis/pathology ; Scrub Typhus/*diagnosis/pathology

OBJECTIVETo summarize the clinical characteristics, diagnosis, treatments and outcomes of perinatal autosomal recessive polycystic kidney disease.

METHODSThe clinical data of one case with infantile polycystic kidney disease diagnosed in perinatal stage and the reports of 11 cases seen in the past 15 years searched in Pubmed, OVID and Elsevier and CNKI, Wanfang database by using the polycystic kidney disease, infant, perinatal, autosomal recessive and case report as keyword were reviewed and analyzed.

RESULTSThe infant was characterized by huge kidneys, severe respiratory and renal compromise. The kidneys were symmetrically enlarged and highly echogenic by ultrasonographic examination and showed high-signal intensity on T2-weighted images by MRI. Histologic analysis showed pulmonary hypoplasia, numerous dilated and elongated tubular structures in the kidney and dilated intrahepatic biliary ducts. Among the 12 cases, 8 cases' presumptive diagnosis was made by prenatal ultrasound revealed enlarged kidneys and oligohydramnios. All cases suffered respiratory distress after birth, and 5 cases complicated pneumothorax. 6 cases died in neonatal stage because of respiratory failure.1 case died 2 m after birth because of renal failure. Five cases are alive and underwent dialysis, nephrectomy or renal transplant.

CONCLUSIONNewborn infants with perinatal autosomal recessive polycystic kidney disease often have poor outcome and died from respiratory and renal failure. Aggressive respiratory support and renal replacement therapy (including nephrectomy, dialysis and transplantation) may give these infants a favorable outcome.

Bronchopulmonary Dysplasia ; etiology ; pathology ; Fatal Outcome ; Humans ; Infant, Newborn ; Infant, Newborn, Diseases ; diagnosis ; genetics ; pathology ; Kidney ; pathology ; Male ; Perinatology ; Polycystic Kidney, Autosomal Recessive ; complications ; diagnosis ; genetics ; pathology ; Renal Dialysis ; Renal Insufficiency ; etiology ; pathology ; Respiratory Insufficiency ; etiology ; pathology ; Retrospective Studies ; Ultrasonography

We reviewed the data of 38 neonates who died of respiratory failure. Paraffin sections of the autopsy lung samples were examined with HE staining or immunolabeling for CD34, CD68 and CK to observe the development of the pulmonary vessels and detect potential pulmonary vascular diseases (PVDs). Five cases were identified to have PVDs, including pulmonary hypertensive vascular remodeling in 3 cases and alveolar capillary dysplasia in 2 cases. The result indicated that PVD was one of the important reasons for respiratory failure in these neonates.
Death ; Humans ; Infant, Newborn ; Lung ; pathology ; Lung Diseases ; diagnosis ; Persistent Fetal Circulation Syndrome ; pathology ; Pulmonary Alveoli ; abnormalities ; pathology ; Respiratory Insufficiency ; mortality ; Vascular Diseases ; diagnosis ; Vascular Remodeling

BACKGROUND: The diffuse infiltrative lung disease requires surgical lung biopsy for its final diagnosis. We evaluated the effect of surgical lung biopsy for final diagnosis of duffuse interstitial lung disease and compared video assisted thoracoscopic lung biopsy (TLB) with open lung biopsy (OLB). MATERIAL AND METHOD: We evaluated the patients who underwent surgical lung biopsy from March 2000 from December 2005, retrospectively. We divide to two groups (OLB and TLB group) and compared them. RESULT: There were 36 patients and cough was the most common pre- operative symptom. Surgery time, anesthetic time, hospital stay, duration of chest tube indwelling, specimen volume and the rate of post-operative complication were not significantly different between two groups. Histologic diagnosis was confirmed in all cases. There was one post-operative death who had suffered from respiratory failure since pre-operative period. CONCLUSION: Surgical lung biopsy is effective method in final diagnosis for diffuse infiltrative lung disease. Video assisted thoracoscopic lung biopsy is lesser invasive method than open lung biopsy and provide similar results, so it is basic diagnostic method of surgical lung biopsy.
Biopsy* ; Chest Tubes ; Cough ; Diagnosis ; Humans ; Length of Stay ; Lung Diseases* ; Lung Diseases, Interstitial ; Lung* ; Pathology ; Respiratory Insufficiency ; Retrospective Studies

OBJECTIVETo study the pathology, imaging and clinical features of a child with trisomy 21 syndrome associated interstitial lung disease.

METHODData of a case with trisomy 21 syndrome associated interstitial lung disease confirmed by lung imaging and pathology were collected, analyzed and the related reports in literature were reviewed.

RESULTThe patient was a one year and 7 months old boy who suffered from severe pneumonia and recurrent infection during his hospital stay. When his disease was stable, he did not have shortness of breath and cyanosis, but a chest computed tomography (CT) showed ground-glass opacity, regional emphysema, band-like change in lung parenchyma, which indicated interstitial lung diseases. Unequal air inflation in bilateral lungs and diffuse over-distension of peripheral air spaces in lung surface were seen through thoracoscope. Pathological examination indicated that alveolar, alveolar ducts and alveolar sac were enlarged, alveolar septa was expanded. There were two reports in lung pathology of trisomy 21 syndrome, alveolar growth abnormalities was seen in 86%-88% cases. The multiple subpleural cysts in chest CT was characteristic. Clinically, trisomy 21 syndrome had high morbidity of respiratory tract infection and progress to respiratory failure frequently. Prolonged postoperative desaturation was constant which required long duration of respiratory support.

CONCLUSIONTrisomy 21 syndrome associated alveolar growth abnormalities were confirmed, which manifest as alveolar simplification in pathology and interstitial lung diseases in imaging. The risk of respiratory failure in these cases caused by infection and surgery should be considered.

Cysts ; pathology ; Down Syndrome ; complications ; Humans ; Infant ; Lung ; pathology ; Lung Diseases, Interstitial ; diagnosis ; etiology ; Male ; Postoperative Period ; Pulmonary Alveoli ; pathology ; Respiratory Insufficiency ; Respiratory Tract Infections ; Tomography, X-Ray Computed

We report a patient who suffered from bronchiolitis obliterans organizing pneumonia(BOOP) after Ivor Lewis operation for esophageal cancer. The patient presented low grade fever, dry cough and mild dyspnea at 4 day after operation. Chest roentgenograms and chest CT revealed bilateral patchy and infiltrative shadows. The respiratory symptoms worsened and respiratory failure developed with mild elevation of WBC count despite of conservative treatment. An open lung biopsy was done and the biopsy specimen showed bronchiolitis obliterans organizing pneumonia(BOOP). After several weeks of steroid therapy, there were marked clinical, physiological and roentgenographic improvements. Our experience suggests that BOOP may be one of the underlying pathology in a number of patients presenting with ARDS after thoracotomy. Since steroid therapy may improve survival in these patients, thoracic surgeons should heighten their index of suspicion for this entity. Early histologic diagnosis should be considered in patients with treatment-resistant ARDS after thoracotomy.
Biopsy ; Bronchiolitis Obliterans* ; Bronchiolitis* ; Cough ; Cryptogenic Organizing Pneumonia* ; Diagnosis ; Dyspnea ; Esophageal Neoplasms ; Fever ; Humans ; Lung ; Pathology ; Respiratory Insufficiency ; Thoracotomy* ; Thorax ; Tomography, X-Ray Computed

In 2011, a cluster of peripartum patients were admitted to the intensive care unit of a tertiary hospital in Seoul with signs and symptoms of severe respiratory distress of unknown etiology. Subsequent epidemiological and animal studies suggested that humidifier disinfectant (HD) might represent the source of this pathology. Epidemiological studies, animal studies, and dose-response analysis demonstrated a strong association between HD use and lung injuries. The diagnostic criteria for HD-associated lung injury (HDALI) was defined on the basis of the clinical, pathological, and radiological attributes of the patients. The clinical spectrum of HDALI appears to range from asymptomatic to full-blown acute respiratory failure, and some patients have required actual lung transplantation for survival. The overall mortality of the exposed population was not significant, although peripartum patients and children who were admitted to the intensive care unit did show high mortality rates. Persistent clinical findings such as diffuse ill-defined centrilobular nodules and restrictive lung dysfunction were observed in some of the survivors. The findings of this review emphasize the importance of assessment of the level of toxicity of chemical inhalants utilized in a home setting, as well as the need to identify and monitor afflicted individuals after inhalational injury.
Animals ; Child ; Diagnosis ; Disinfectants ; Epidemiologic Studies ; Humans ; Humidifiers* ; Intensive Care Units ; Lung Injury* ; Lung Transplantation ; Lung* ; Mortality ; Pathology ; Peripartum Period ; Prognosis ; Respiratory Insufficiency ; Seoul ; Survivors ; Tertiary Care Centers

Aorta, Thoracic ; abnormalities ; Humans ; Infant ; Magnetic Resonance Angiography ; Male ; Pulmonary Artery ; abnormalities ; Recurrence ; Respiratory Insufficiency ; etiology ; Respiratory Sounds ; etiology ; Subclavian Artery ; abnormalities ; Vascular Diseases ; complications ; congenital ; diagnosis ; pathology ; physiopathology

INTRODUCTIONWe report angioedema as a rare presentation leading to a diagnosis of systemic lupus erythematosus (SLE).

CLINICAL PICTUREA diagnosis of angioedema was delayed in a patient presenting with limb and facial swelling until she developed acute upper airway compromise. After excluding allergic and hereditary angioedema, acquired angioedema (AAE) was suspected, possibly precipitated by respiratory tract infection. Associated clinical and laboratory features led to a diagnosis of SLE.

TREATMENTManagement proved challenging and included high dose steroids and immunosuppressants.

OUTCOMEThe patient responded to treatment and remains in remission without recurrence of the angioedema.

CONCLUSIONAAE occurs due to the acquired deficiency of inhibitor of C1 component of complement (C1 INH). Lymphoproliferative disorders and anti-C1 INH antibodies are well-described associations. However, one should also consider the possibility of SLE.

Angioedema ; blood ; etiology ; physiopathology ; therapy ; Antiphospholipid Syndrome ; diagnosis ; etiology ; Brain ; pathology ; Complement C1 Inactivator Proteins ; analysis ; deficiency ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; diagnosis ; etiology ; Magnetic Resonance Imaging ; Middle Aged ; Respiration, Artificial ; Respiratory Insufficiency ; etiology ; therapy