Antifungal Agents ; therapeutic use ; Female ; Humans ; Lung ; microbiology ; pathology ; Lung Diseases, Fungal ; drug therapy ; etiology ; Middle Aged ; Schizophyllum ; drug effects ; pathogenicity ; Voriconazole ; therapeutic use

OBJECTIVETo analyze clinical and imaging features and genetic characteristics of Leigh syndrome with emergent pulmonary edema.

METHODThe clinical features and imaging data of 2 cases (1 male, 1 female) seen in Anhui Provincial Children's Hospital from 2012 to 2014 were analyzed and summarized. Venous blood samples were sent to Guangzhou Jinyu Medical Examination Center for genetic analysis. Peripheral blood DNA was extracted and amplified, then sent to a sequencing facility for presence of genetic mutation by comparing with the reference sequence (NC_012920.1).

RESULT(1) The first patient was a 7 months old boy. The second patient was a 7 months and 21 days old girl. They were presented with abnormal respiration and pulmonary hemorrhage required mechanical ventilation. The first patient had a similar attack after 4 months of his birth, whose psychomotor development was normal, and no abnormal neurological findings. The value of blood lactate was 1.58 mmol/L. The value of pyruvic acid was 0.25 mmol/L. The value of cerebrospinal fluid lactate was 6. 4 mmol/L, which was an abnormal increase. The second patient had abnormal nervous system development, which included motor development retardation and hypotonia. The value of blood lactate was 6. 8 mmol/L, pyruvic acid was 0.31 mmol/L. Cerebrospinal fluid lactate was 8.2 mmol/L. (2) Imaging data: chest X-ray revealed double lung effusion. Bilateral caudate nucleus and lentiform nucleus had high signal, and bilateral internal capsule forelimbs were affected in DWI sequence of head MRI. Hemispheres, basal ganglia, cerebral peduncle, cerebellum, pons, and splenium of corpus callosum had multiple abnormal signals in head MRI of the second patient. NAA peak showed significantly reduced lesion area in magnetic resonance blood-flow scanning, and Cho peak increased significantly, which were double lactate-peak. (3) Genetic testing: ATPase6 m.9185 t > C mutation was found in case 1 that was consistent with Leigh syndrome pathogenesis. Hybrid mutations (m. 10191 t > C) in mitochondrial DNA was found in case 2. Two cases with the diagnosis of Leigh syndrome was clear. They were given combined therapy, such as mechanical ventilation, limited fluid to alleviate lung exudation, coenzyme Q10, and L-carnitine. The illness of case 1 relapsed after discharge. But in case 2, there was no improvement. They both died after treatment was given up.

CONCLUSIONNeurological symptoms were common in Leigh syndrome, in which acute lung hemorrhage was rarely reported. Timely ventilator support can temporarily save lives, but fatality rate is high and prognosis is poor.

Brain ; pathology ; Carnitine ; therapeutic use ; DNA, Mitochondrial ; Female ; Genetic Testing ; Hemorrhage ; etiology ; Humans ; Infant ; Lactic Acid ; Leigh Disease ; complications ; genetics ; Lung Diseases ; etiology ; Magnetic Resonance Imaging ; Male ; Mutation ; Pyruvic Acid

OBJECTIVETo discuss the clinical features of Indium-related lung diseases.

METHODSWe searched database of Chinese and Pubmed, Embase, Web of Science to collect research data of indium-related lung diseases from Jan. 1998 to Aprl. 2014. Case reports, exposure histories and lab results were analysed and summarized.

RESULTS1998 to Mar 2010, ten cases of indium-related lung diseases were published. Seven cases of interstitial pneumonia were reported in Japan, two cases of pulmonary alveolar proteinosis (PAP) were reported in the USA and one case of PAP reported in China. Chest computer tomography (CT) showed diffuse or local ground glass appearance (GGA) in 8 cases, 3 of which also showed centrilobular nodules; Pulmonary function test were normal only in one out of 8 cases. Cholesterol clefts were found in 4 cases of interstitial pneumonia. 3 cases died among 6 cases who were followed-up.

CONCLUSIONSOccupational exposure to indium compounds are contributory to different pulmonary diseases, which are composed of interstitial pneumonia and pulmonary alveolar proteinosis. The relationships between In-C, In-S and these pulmonary diseases are unclear.

China ; Glass ; Humans ; Indium ; toxicity ; Japan ; Lung Diseases, Interstitial ; etiology ; pathology ; Occupational Exposure ; adverse effects ; Pulmonary Alveolar Proteinosis ; etiology ; pathology ; Respiratory Function Tests ; Tomography, X-Ray Computed

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

OBJECTIVEWe sought to master the clinical characteristics and prognosis of hard mental lung disease, improving this disease's diagnosis and treatment quality.

METHODSWe recruited two suspected patients with hard mental lung disease and collected their occupational history, examination results of occupational health, and past medical records. By virtue of laboratory tests, high Kv chest radiography, CT and HRCT of chest, fiberoptic bronchoscopy and ECG examination, diagnostic report was synthesized respectively by respiratory physicians and pathologist from three different agencies. Then the report was submitted to diagnosis organizations of occupational disease, and diagnostic conclusion of occupational disease was drawn after discussion by at least three diagnosticians of occupational disease.

RESULTSWe found that both of the two suspected patients were exposed to dusts of hard metal, and length of exposure service ranged from 8 to 9 years. Clinical manifestations were dominated by dry cough, wheezing after activities, and pathological manifestation was characteristic giant cell interstitial pneumonia. The prognosis and outcome of the disease were different.

CONCLUSIONAccording to exact occupational exposure history, clinical manifestations, combined with the results of high Kv chest radiography, CT of chest and pathological manifestation, it can be diagnosed with hard mental lung disease.

Alloys ; adverse effects ; Bronchoscopy ; Cobalt ; adverse effects ; Dust ; Humans ; Lung ; pathology ; Lung Diseases ; diagnosis ; etiology ; Occupational Diseases ; diagnosis ; etiology ; Occupational Exposure ; adverse effects ; Tungsten ; adverse effects

The bronchoarterial (BA) ratio measured with computed tomography is widely used in human medicine to diagnose bronchial dilation or collapse. Although use of the BA ratio in veterinary medicine has been recently studied, this has not been evaluated in brachycephalic dogs predisposed to bronchial diseases including bronchial collapse. The purpose of this study was to establish BA ratios for brachycephalic dogs and compare the values with those of non-brachycephalic dogs. Twenty-three brachycephalic dogs and 15 non-brachycephalic dogs without clinical pulmonary disease were evaluated. The BA ratio of the lobar bronchi in the left and right cranial as well as the right middle, left, and right caudal lung lobes was measured. No significant difference in mean BA ratio was observed between lung lobes or the individual animals (p = 0.148). The mean BA ratio was 1.08 +/- 0.10 (99% CI = 0.98~1.18) for brachycephalic dogs and 1.51 +/- 0.05 (99% CI = 1.46~1.56) for the non-brachycephalic group. There was a significant difference between the mean BA ratios of the brachycephalic and non-brachycephalic groups (p = 0.00). Defining the normal limit of the BA ratio for brachycephalic breeds may be helpful for diagnosing bronchial disease in brachycephalic dogs.
Animals ; Bronchial Arteries/*anatomy & histology ; Craniosynostoses/pathology/radiography/*veterinary ; Dog Diseases/pathology/*radiography ; Dogs ; Female ; Lung Diseases/etiology/*veterinary ; Male ; Reference Values ; Tomography, X-Ray Computed/*veterinary

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

BACKGROUNDGiant cell interstitial pneumonia (GIP) was a rare form of pneumoconiosis, associated with exposure to hard metals, which had been reported mostly as isolated case reports. We described eight cases of GIP diagnosed in our hospital during the past seven years, with particular reference to new findings.

METHODSEight patients with GIP confirmed by biopsy in the Nanjing Drum Tower Hospital affiliated to Medical School of Nanjing University from 2005 to 2011 were retrospectively analyzed. For each patient, the occupy histories and medical records were thoroughly reviewed and clinic data were extracted. Two radiologists, without knowledge of any of the clinical and functional findings, independently reviewed the HRCT scans of all patients. Follow-up data were collected.

RESULTSAmong the eight patients, seven had a history of exposure to hard metal dusts, one denied an exposure history. The most common manifestations were cough and dyspnea. One patient initiated with pneumothorax and another pleural effusion, both of which were uncommon to GIP. The main pathologic appearances were the presence of macrophages and multinucleated giant cells in the alveolar space. The clinical symptoms and radiographic abnormalities were obviously improved after cessation of exposure and receiving corticosteroid treatments, recurrences were observed in two patients when they resumed work. In spite of exposure cessation and corticosteroid treatment, one patient developed pulmonary fibrosis at seven years follow-up.

CONCLUSIONSAwareness of the patients' occupational history often provided clues to the diagnosis of GIP. Histopathologic examinations were necessary to establish the right diagnosis. Exposure cessation was of benefit to most patients; however, pulmonary fibrosis was possible in spite of exposure cessation and corticosteroid treatment. Better ways should be found out to improve the outcome and quality of life.

Adult ; Alloys ; toxicity ; Cobalt ; toxicity ; Female ; Humans ; Lung ; pathology ; Lung Diseases, Interstitial ; diagnosis ; etiology ; Male ; Middle Aged ; Pulmonary Fibrosis ; diagnosis ; etiology ; Retrospective Studies ; Tungsten ; toxicity ; Young Adult

OBJECTIVETo identify the risk factors which will indicate the Pneumocystis carinii (Pc) infection in children with systemic lupus erythematosus (SLE) and investigate the clinical features and to elevate the level to find out the high-risk patients and make early diagnosis and treatment.

METHODThe characteristics, clinical features, laboratory examinations, treatment and prognosis of Pneumocystis carinii pneumonia (PCP) in children with SLE under 18 years of age treated in our hospital between January 2000 and January 2013 were prospectively reviewed. A comparison was made with the 26 cases of SLE children without PCP who were matched for gender, age and course, and a literature review was made.

RESULTS(1) Five cases were enrolled, 3 were male and 2 female. Their age range was 13-17 (14.0 ± 1.6) years. All the children had kidney involvement. The courses were from 3 months to 4.5 years. All patients were receiving daily glucocorticoid therapy and immunosuppressive drugs before the diagnosis of PCP.Four patients were in the inactive phase of SLE (SLEDAI 2-4 points), and the fifth case was in active phase (SLEDAI 8, low complement 2 points, anti-dsDNA antibody positive 2 points, urine-protein 4 points). (2) Besides the clinical manifestations of SLE, most patients had progressive dyspnea, fever and dry cough at onset of PCP. Two children accepted mechanical ventilation because of respiratory failure. The mean duration of the symptoms to diagnosis was 10-30 (17.6 ± 7.8) days. Lactose dehydrogenase (LDH) was elevated more or less, median was (700 ± 263) U/L. Lymphocyte count were (0.3-1.4)×10(9)/L (median 0.5×10(9)/L), and three children had CD4 T lymphocyte count <0.3×10(9)/L. Arterial blood gas analyses showed severe hypoxemia. Chest radiographs showed in all cases diffuse interstitial infiltration. Pc was positive in the sputum. All patients were treated with trimethoprim-sulfamethoxazole and corticosteroids.

CONCLUSIONWhen SLE children are treated with corticosteroids and immunosuppressive drugs, low lymphocyte count is the risk factor for Pc infection.It is essential to monitor lymphocyte count.We should pay more attention to fever, dry cough and hypoxemia. Chest radiologic examination may help diagnose the PCP in SLE children.It may be helpful for SLE children whose CD4T lymphocyte was below 0.3×10(9)/L to take trimethoprim-sulfamethoxazole for PCP prophylaxis.

Adolescent ; Anti-Infective Agents ; adverse effects ; therapeutic use ; Case-Control Studies ; Child ; Female ; Glucocorticoids ; adverse effects ; therapeutic use ; Humans ; Immunosuppressive Agents ; adverse effects ; therapeutic use ; Kidney Diseases ; etiology ; Lung ; pathology ; Lupus Erythematosus, Systemic ; complications ; drug therapy ; Lymphocyte Count ; Male ; Opportunistic Infections ; drug therapy ; epidemiology ; Pneumonia, Pneumocystis ; drug therapy ; epidemiology ; Prognosis ; Retrospective Studies ; Risk Factors ; Trimethoprim, Sulfamethoxazole Drug Combination ; therapeutic use

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