OBJECTIVETo investigate the etiology and clinical manifestation of hemoptysis in children.

METHODA retrospective analysis was performed for 106 cases of hemoptysis who were admitted to The Second Affiliated Hospital & Yuying Children's Hospital of Wenzhou Medical University from January 2005 to December 2014.The clinical information including laboratory tests and image data were collected and analyzed.

RESULTA total of 106 patients (50 males and 56 females) were identified. The median age was 9.1 years (range 2 months to 18 years). Pneumonia (35, 31.1%) was the most common etiology of hemoptysis, which included bacterial pneumonia (27 cases), mycoplasmal pneumonia(4 cases), chlamydial pneumonia (3 cases), and influenza pneumonia(1 case). Other causes included bronchitis(15, 14.2%), pulmonary tuberculosis (11, 10.4%), bronchiectasis (11, 10.4%), diffuse alveolar hemorrhage (8, 7.5%), idiopathic pulmonary hemosiderosis(6, 5.7%), cardiovascular dysplasia(6, 5.7%), pulmonary contusion (4, 3.8%), foreign body in bronchus (2, 1.9%), allergic bronchopulmonary aspergillosis (2, 1.9%). Eighty-six patients manifested mild hemoptysis; moderate and massive hemoptysis were found in nine and eleven patients, respectively. Pneumonia accounted for 33.7% of mild hemoptysis and 45.5% of massive hemoptysis were due to bronchiectasis; 80.2% were treated with antibiotics and 41.5% were given hemostatic agents; 8.5% received lobectomy. Ninety-six patients (90.6%) were cured and parents gave up treatment in 4 cases (3.8%). Six patients (5.7%) suffered from recurrent hemoptysis.

CONCLUSIONHemoptysis mainly occurred in children who were older than 6 years, the most common cause of hemoptysis was respiratory tract infection. In most cases, the amount of hemoptysis was small and the overall prognosis was good.

Adolescent ; Bronchiectasis ; complications ; Bronchitis ; complications ; Child ; Child, Preschool ; Female ; Foreign Bodies ; complications ; Hemoptysis ; diagnosis ; etiology ; therapy ; Hemosiderosis ; complications ; Humans ; Infant ; Influenza, Human ; complications ; Lung Diseases ; complications ; Lung Injury ; complications ; Male ; Pneumonia, Bacterial ; complications ; Prognosis ; Retrospective Studies ; Tuberculosis, Pulmonary ; complications

To compare clinical features, diagnosis and therapeutic effect between pulmonary histoplasmosis and progressive disseminated histoplasmosis.
 Methods: A retrospective analysis for 12 cases of hospitalized patients with histoplasmosis, who was admitted in Xiangya Hospital, Central South University during the time from February 2009 to October 2015, was carried out. Four cases of pulmonary histoplasmosis and 8 cases of progressive disseminated histoplasmosis were included. The differences of clinical features, imaging tests, means for diagnosis and prognosis were analyzed between the two types of histoplasmosis.
 Results: The clinical manifestations of pulmonary histoplasmosis were mild, such as dry cough. However, the main clinical symptoms of progressive disseminated histoplasmosis were severe, including recurrence of high fever, superficial lymph node enlargement over the whole body, hepatosplenomegaly, accompanied by cough, abdominal pain, joint pain, skin changes, etc.Laboratory examination showed pancytopenia, abnormal liver function and abnormal coagulation function. One pulmonary case received the operation of left lower lung lobectomy, 3 cases of pulmonary histoplasmosis and 6 cases of progressive disseminated histoplasmosis patients were given deoxycholate amphotericin B, itraconazole, voriconazole or fluconazole for antifungal therapy. One disseminated case discharged from the hospital without treatment after diagnosis of histoplasmosis, and 1 disseminated case combined with severe pneumonia and active tuberculosis died ultimately.
 Conclusion: As a rare fungal infection, histoplasmosis is easily to be misdiagnosed. The diagnostic criteria depends on etiology through bone marrow smear and tissues biopsy. Liposomeal amphotericin B, deoxycholate amphotericin B and itraconazole are recommended to treat infection for histoplasma capsulatum.
Abdominal Pain ; etiology ; Amphotericin B ; therapeutic use ; Antifungal Agents ; therapeutic use ; Biopsy ; Cough ; epidemiology ; Death ; Deoxycholic Acid ; therapeutic use ; Diagnostic Errors ; Drug Combinations ; Fever ; etiology ; Hepatomegaly ; etiology ; Histoplasma ; Histoplasmosis ; complications ; diagnosis ; mortality ; therapy ; Humans ; Invasive Fungal Infections ; complications ; diagnosis ; therapy ; Itraconazole ; therapeutic use ; Lung ; microbiology ; surgery ; Lung Diseases, Fungal ; diagnosis ; surgery ; therapy ; Pneumonia ; complications ; mortality ; Recurrence ; Retrospective Studies ; Splenomegaly ; etiology ; Treatment Outcome ; Tuberculosis ; complications ; mortality

BACKGROUND/AIMS: To evaluate the impact on mortality of anti-tumor necrosis factor (anti-TNF) treatment of rheumatoid arthritis-associated interstitial lung disease (RA-ILD). METHODS: We retrospectively reviewed the medical records of 100 RA-ILD patients who visited our tertiary care medical center between 2004 and 2011, identified those treated with an anti-TNF agent, divided patients into non-survivor and survivor groups and evaluated their clinical characteristics and causes of death. RESULTS: A total of 24 RA-ILD patients received anti-TNF therapy, of whom six died (25%). Mean age at initiation of anti-TNF therapy was significantly higher in the nonsurvivor versus survivor group (76 years [range, 66 to 85] vs. 64 years [range, 50 to 81], respectively; p = 0.043). The mean duration of anti-TNF treatment in the non-survivor group was shorter (7 months [range, 2 to 14] vs. 23 months [range, 2 to 58], respectively; p = 0.030). The duration of anti-TNF therapy in all nonsurviving patients was < 12 months. Pulmonary function test results at ILD diagnosis, and cumulative doses of disease-modifying drugs and steroids, did not differ between groups. Five of the six deaths (83%) were related to lung disease, including two diffuse alveolar hemorrhages, two cases of acute exacerbation of ILD, and one of pneumonia. The sixth patient died of septic shock following septic arthritis of the knee. CONCLUSIONS: Lung complications can occur within months of initial anti-TNF treatment in older RA-ILD patients; therefore, anti-TNF therapy should be used with caution in these patients.
Adult ; Aged ; Aged, 80 and over ; Antirheumatic Agents/adverse effects/*therapeutic use ; Arthritis, Rheumatoid/complications/diagnosis/*drug therapy/immunology/mortality ; Female ; Humans ; Lung Diseases, Interstitial/diagnosis/etiology/*mortality ; Male ; Middle Aged ; Republic of Korea ; Retrospective Studies ; Risk Assessment ; Risk Factors ; Tertiary Care Centers ; Time Factors ; Treatment Outcome ; Tumor Necrosis Factor-alpha/*antagonists & inhibitors

Amphotericin B ; administration & dosage ; therapeutic use ; Antifungal Agents ; administration & dosage ; therapeutic use ; Biomarkers ; blood ; Child ; Cough ; diagnosis ; drug therapy ; etiology ; Cryptococcosis ; Fever ; diagnosis ; drug therapy ; etiology ; Fluconazole ; administration & dosage ; therapeutic use ; Humans ; Lung ; diagnostic imaging ; pathology ; Lung Diseases, Fungal ; complications ; diagnosis ; drug therapy ; Male ; Radiography, Thoracic ; Tomography, X-Ray Computed

OBJECTIVETo explore the diagnostic and therapeutic methods for perioperative children with congenital heart disease (CHD) with airway stenosis in pediatric intensive care unit (PICU).

METHODFiberoptic bronchoscopy was used for the diagnosis of 100 CHD cases in PICU who were clinically considered to have possible airway malformation because of complicated difficult-to-control lung infection, atelectasis and failure with the ventilator after surgery from January 2010 to October 2011. Cases who were confirmed to have severe airway stenosis by bronchoscopy and weaning from the ventilator after surgery were treated with balloon expandable stents into the desired position in the bronchoscopy.

RESULTThere were 73 cases (73%) of CHD patients with airway abnormalities, including 31 cases of severe stenosis (31%), moderate stenosis in 29 cases (29%), mild stenosis in 13 cases (13%). Nine of the 10 children in whom the mechanical ventilation was hard to be stopped after surgery because of severe airway stenosis were weaned from mechanical ventilation successfully by fiberoptic bronchoscopy, while one case died from primary disease with severe sepsis after the placement of bronchial stents.

CONCLUSIONCHD children with difficult-to-control lung infection, atelectasis and failure with ventilator after surgery are often complicated with airway abnormalities. The therapeutic bronchoscopy with airway stent can be used for cases with weaning from the ventilator because of severe airway stenosis.

Airway Obstruction ; diagnosis ; etiology ; therapy ; Bronchoscopy ; methods ; Constriction, Pathologic ; Female ; Follow-Up Studies ; Heart Defects, Congenital ; complications ; diagnosis ; surgery ; Humans ; Infant ; Infant, Newborn ; Intensive Care Units, Pediatric ; Lung Diseases ; diagnosis ; etiology ; therapy ; Male ; Perioperative Period ; Respiration, Artificial ; Stents ; Trachea ; abnormalities ; Tracheal Stenosis ; diagnosis ; etiology ; therapy ; Treatment Outcome

OBJECTIVETo explore the clinical and laboratory features and the prognosis of juvenile dermatomyositis (JDM) complicated with interstitial lung disease (ILD).

METHODData of 39 cases of JDM complicated with ILD hospitalized in Beijing Children's Hospital from January 2005 to December 2011 were collected. The clinical features, laboratory data and prognosis of these children were analyzed.

RESULTOf the 39 cases studied, 16 were boys, and 23 girls. The average age of onset was 5.6 years, and 61.5% of the patients' age of onset (24 cases) was under 6 years. Rashes (17 cases, 43.6%), simultaneous eruption of rashes and muscle weakness (14 cases, 35.9%), fever (4 cases, 10.1%), or muscle weakness (3 cases, 7.7%) were common initial symptoms of the disease. Only 51.3% of the patients (20 cases) had the symptoms of respiratory system, but (24 cases) 61.5% were complicated with that of the gastrointestinal system; (27 cases) 69.2% had at the same time electrocardiographic and echocardiographic abnormalities. The chest high resolution computed tomography (HRCT) showed cord or band-like shadows in their lungs of more than half of the cases (25 cases, 64.1%), and other changes included ground glass-like shadow (10 cases, 25.6%), net and lineation-like shadow (9 cases, 23.1%), nodular change (5 cases, 12.8%). The patients complicated with lung essential infiltration accounted for as high as 71.8% (28 cases). These imaging changes were largely seen on both dorsal sides of their lungs. Severe patients also had mediastinal emphysema, pneumothorax, pneumorrhagia or aerodermectasia. Twenty-four patients underwent pulmonary function examination, and 62.5% of the patients' pulmonary function (15 cases) was abnormal. The fatality rate of the cases studied was 10.1%.

CONCLUSIONThe imaging changes of patients suffering from JDM with ILD were often more severe as compared to the clinical symptoms, and were often complicated with damages to other systems and organs. The prognosis of those patients was poorer than others. Patients with JDM especially at a younger age of onset and with various organ damages should be examined with chest HRCT examinations as early as possible.

Child ; Child, Preschool ; Dermatomyositis ; complications ; diagnosis ; drug therapy ; Female ; Glucocorticoids ; administration & dosage ; therapeutic use ; Humans ; Immunosuppressive Agents ; administration & dosage ; therapeutic use ; Lung ; diagnostic imaging ; pathology ; Lung Diseases, Interstitial ; diagnosis ; drug therapy ; etiology ; Male ; Methotrexate ; administration & dosage ; therapeutic use ; Muscle Weakness ; diagnosis ; epidemiology ; etiology ; Prognosis ; Respiratory Function Tests ; Retrospective Studies ; Tomography, X-Ray Computed

OBJECTIVETo review the pathophysiology, evaluation, management, and outcomes of children with inherited disorders of surfactant metabolism due to mutations in the genes encoding surfactant proteins-B or -C (SFTPB, SFTPC), ATP binding cassette member A3 (ABCA3), and thyroid transcription factor (NKX2.1).

DATA SOURCESReview of the literature, previous work from the author's and collaborators' laboratories, St. Louis Children's Hospital Lung Transplant Database.

STUDY SELECTIONKey articles in the field, author's work.

RESULTSInherited disorders of surfactant metabolism present as acute, severe respiratory dysfunction in the neonatal period (SFTPB, ABCA3, NKX2.1) or as chronic respiratory insufficiency in later infancy and childhood which is of variable onset, severity, and course (SFTPC, ABCA3, NKX2.1). Diagnosis is established with sequencing the relevant genes; lung biopsy with electron microscopy is a useful adjunct. For surfactant protein-B and ABCA3 deficiency presenting with acute neonatal disease, treatment options are limited to lung transplantation or compassionate care. For the more chronic presentations of surfactant protein-C, ABCA3, and NKX2.1 associated disease, the natural history is variable and therefore individualized, supportive care is appropriate,

CONCLUSIONSInherited disorders of surfactant metabolism are rare, but informative diseases that provide unique opportunities for understanding mechanisms of respiratory disease in newborns and children.

ATP-Binding Cassette Transporters ; genetics ; Humans ; Infant, Newborn ; Lung Diseases ; diagnosis ; etiology ; therapy ; Lung Transplantation ; Mutation ; Pulmonary Surfactant-Associated Protein B ; deficiency ; genetics ; Pulmonary Surfactant-Associated Protein C ; genetics ; Pulmonary Surfactants ; metabolism

Dermatomyositis ; complications ; Humans ; Lung Diseases, Interstitial ; diagnosis ; etiology ; therapy

Microscopic polyangiitis (MPA) is a systemic vasculitis affecting small vessels. To determine the clinical features and outcomes of MPA in Korean patients, we retrospectively reviewed the medical records of patients diagnosed with MPA at a single medical center in Korea between 1989 and 2006. The 18 patients who met the Chapel Hill criteria for MPA had a mean (+/-SD) age at the time of diagnosis of 62.4+/-12.7 yr. Renal manifestations and general symptoms were the most common features of MPA, with lung involvement also very common. Antineutrophil cytoplasmic antibodies (ANCA) were present in 17 of the 18 patients (94%). Of 17 patients treated with steroids and cyclophosphamide, 11 (65%) had stable or improved course. One patient treated with steroids without cyclophosphamide showed disease progression. Ten of the 18 patients (56%) died at a median follow-up of 8 months. MPA in Korean patients was distinguished by a higher rate of lung involvement, especially alveolar hemorrhage, which was the leading cause of death in our patients. Korean patients were also older at MPA onset and were more likely positive for ANCA. Other overall clinical manifestations did not differ significantly.
Adult ; Age Factors ; Aged ; Aged, 80 and over ; Antibodies, Antineutrophil Cytoplasmic/blood ; Cyclophosphamide/therapeutic use ; Drug Therapy, Combination ; Female ; Hemorrhage/etiology ; Humans ; Kidney Failure/etiology ; Korea ; Lung Diseases/etiology ; Male ; Middle Aged ; Polyarteritis Nodosa/*diagnosis/drug therapy/mortality ; Pulmonary Alveoli/blood supply/pathology ; Retrospective Studies ; Steroids/therapeutic use ; Survival Analysis ; Treatment Outcome

Adult-onset Still's disease (AOSD) is an inflammatory disease that presents with a variety of clinical symptoms. Pulmonary involvement is well-known in AOSD and is seen in up to 53% of AOSD cases, with the most common pulmonary diseases being pleural effusion and transient pulmonary infiltrates. We present the first case of chronic AOSD complicated with diffuse alveolar hemorrhage during the acute flare of the disease.
Adult ; Ankylosis/diagnosis ; Anti-Inflammatory Agents/administration & dosage ; Diagnosis, Differential ; Female ; Fever of Unknown Origin/diagnosis ; Hemorrhage/*diagnosis/drug therapy/etiology ; Humans ; Lung Diseases/*diagnosis/drug therapy/etiology ; Methylprednisolone/administration & dosage ; *Pulmonary Alveoli ; Still's Disease, Adult-Onset/complications/*diagnosis/drug therapy/radiography ; Tomography, X-Ray Computed