Lymphangioleiomyomatosis is a rare disease which mostly occurs during the reproductive ages of premenopausal women. The presenting symptoms of lymphangioleiomyomatosis include cystic lung lesions, progressive dyspnea on exertion, and recurrent pneumothorax. HRCT shows bilateral diffusely scattered tiny cysts and PFT shows obstructive airflow limitation and decreased diffusion capacity. We report a case of lymphangioleiomyomatosis which presented with recurrent pneumothorax and characteristic cysts in HRCT. The diagnosis was confirmed by pathology. This is the first case of lymphangioleiomyomatosis diagnosed for a postmenopausal woman in Korea. Lymphangioleiomyomatosis must be considered in the diagnosis for a postmenopausal woman with characteristic symptoms and radiologic findings for Korean patients.
Diagnosis ; Diffusion ; Dyspnea ; Female ; Humans ; Korea ; Lung ; Lymphangioleiomyomatosis* ; Menopause ; Pathology ; Pneumothorax* ; Rare Diseases

Respiratory bronchiolitis-associated interstitial lung disease has been described among current or former smokers and has features consistent with interstitial lung disease. Symptoms include cough, dyspnea on exertion. Lung pathology is characterized by the accumulation of pigmented macrophages within respiratory bronchioles and adjacent air spaces associated with mild thickening of the peribronchiolar interstitium. It must be separated from the other interstitial lung disease because of marked differences in treatment and prognosis. Recently we experienced a case of respiratory bronchiolitis -associated interstitial lung disease in a 48-year-old man. As far as we know, this is the first case in Korea.
Bronchioles ; Bronchiolitis ; Cough ; Dyspnea ; Humans ; Korea ; Lung ; Lung Diseases, Interstitial* ; Macrophages ; Middle Aged ; Pathology ; Prognosis ; Smoking

An 81-year-old man underwent bronchoscopy to investigate the cause of his dyspnea symptoms. A benign natured mass was observed in the bronchus and was excised. The pathology examination revealed a fibroepithelial polyp. He has been asymptomatic since the simple bronchoscopic excision. A fibroepithelial polyp is a benign tumor with a mesodermal origin. It is commonly found in the skin, urogenital area and chest, but is extremely rare in the respiratory system. We report a case of a fibroepithelial polyp in the bronchus, which was treated with a bronchoscopic excision, with a brief review of the relevant literature.
Aged, 80 and over ; Bronchi ; Bronchoscopy ; Dyspnea ; Humans ; Mesoderm ; Pathology ; Polyps* ; Respiratory System ; Skin ; Thorax

Respiratory bronchiolitis associated interstitial lung disease is a rare condition among current or ex-smokers, which has features consistent with interstitial lung disease. The presentations are non-specific, but symptoms generally include a cough and dyspnea on exertion, and its pathology is characterized by the accumulation of pigmented macrophages within the respiratory bronchioles and adjacent air spaces, and is associated with mild thickening of the peribronchiolar interstitium. Recently, the case of a 54-year-old woman passive smoker, diagnosed as having respiratory bronchiolitis associated interstitial lung disease, was experienced at our institution.
Bronchioles ; Bronchiolitis* ; Cough ; Dyspnea ; Female ; Humans ; Lung Diseases, Interstitial* ; Macrophages ; Middle Aged ; Pathology ; Tobacco Smoke Pollution*

OBJECTIVEMedically unexplained dyspnea is common in adult and accounts for 14% patients complaining of dyspnea. Its occurrence in children is seldom recognized. In the present paper, 34 children with medically unexplained dyspnea (age 10 to 18 years) seen in Peking Union Medical College Hospital from 1996 to 2002 are reported.

METHODSThe diagnosis of medically unexplained dyspnea was clinical: it was based on the presence of dyspnea and other complaints which cannot be explained by an organic disease. The patients answered Nijmegen questionnaire and state and trait anxiety (STAI), and performed hyperventilation provocation test. Twenty sessions of breathing therapy were applied and 13 out of 34 children were followed up after the therapy.

RESULTSAmong the children, 75% started to have symptoms at the age of 13 to 16 years, though the age of first episode could be as early as 8 years. In most of the cases, the course was chronic clinically. In addition to marked dyspnea, their clinical profile included symptoms of hyperventilation i.e. blurred vision, dizziness, tingling, stiff fingers or arm. The symptoms of anxiety were less frequent in children and accordingly the level of anxiety evaluated by means of STAI was lower in children compared to adult patients. The precipitating psychological factors appeared to be related to middle school competition. Pressure from exams, reprimand from stern and unsympathetic teachers coupled with high parental expectation could be emotionally damaging to psychologically susceptible children. Thirteen patients were followed up after 2-3 months of breathing therapy with emphasis on abdominal breathing and slowing down of expiration. After therapy, the sum score of the Nijmegen Questionnaire was markedly decreased. Dyspnea and symptoms of hyperventilation were improved. The level of anxiety was minimally modified.

CONCLUSIONThe cases illustrated the need for careful diagnostic evaluation and treatment because of the high rate of chronicity of the disorder.

Adolescent ; Child ; Dyspnea ; etiology ; pathology ; therapy ; Female ; Humans ; Hyperventilation ; Male ; Prognosis ; Treatment Outcome

Aged, 80 and over ; Dyspnea ; etiology ; Glottis ; pathology ; Hematoma ; complications ; Hemophilia A ; complications ; Humans ; Laryngeal Diseases ; complications ; Male

Idiopathic pulmonary hemosiderosis (IPH) is a rare respiratory disease with an unknown etiology, and is diagnosed with laboratory, radiology, and pathology tests. Chief complaints of IPH include hemoptysis, cough, and dyspnea. Since it is considered an immune-mediated disease, the first line of treatment is systemic corticosteroid therapy. The three cases reported here showed a decrease in ferritin level and improvement in the hemoglobin level with prednisolone treatment. However, long-term corticosteroid therapy may cause several side effects, particularly growth retardation and obesity, which can affect growing children. In the present study, all patients had cushingoid symptoms and obesity. Therefore, we switched to deflazacort (DFZ), which has lesser side-effects of weight gain. This report describes clinical courses of the disease and comparison of body mass index of three patients with IPH who took DFZ instead of prednisolone. DFZ was effective for IPH, and is useful for weight gain reduction.
Body Mass Index ; Child ; Cough ; Dyspnea ; Ferritins ; Hemoptysis ; Hemosiderosis ; Humans ; Obesity ; Pathology ; Prednisolone ; Weight Gain

OBJECTIVETo explore the role of rigid bronchoscope in the diagnosis and treatment of plastic bronchitis.

METHODSVarious clinical parameters, the key points of operation and postoperative recovery were analyzed in 8 patients with plastic bronchitis treated in Department and ICU using retrospective analysis.

RESULTSAll cases demonstrated bronchial lumen stenosis, mucous membrance coarse, congestion, edema in the affected side. 2 cases had follicular hyperplasia, 1 case had a few ooze after taking out the plastic type. All cases had endogenous foreign bodies, taking the shape of the bronchial tree or a funicular. Block shape and sites were as follows: right main bronchus 2 cases, superior lobe of right lung 1 case, right middle bronchial 1 case, left main bronchus 2 cases, 1 case with left lower lobe, right main bronchus and left lower lobe bronchus 1 case. The breath sounds of the affected side become more enhanced after operation, with the alliviation of dyspnea. All cases recovered after ICU treatment. The pathologic examination were all type I plastic bronchitis.

CONCLUSIONSRemovement of the endogenous foreign body via rigid bronchoscopy is the effective method in the treatment of plastic bronchitis. Plastic bronchitis is a rapid-developing critical, urgent disease.In order to reduce the mortality, early diagnosis and timely surgery are necessary.

Airway Obstruction ; surgery ; Bronchi ; pathology ; Bronchitis ; diagnosis ; surgery ; Bronchoscopes ; Bronchoscopy ; Constriction, Pathologic ; surgery ; Dyspnea ; therapy ; Foreign Bodies ; pathology ; surgery ; Humans ; Lung ; pathology ; Postoperative Period ; Retrospective Studies ; Trachea ; pathology

Objective: To analyze the clinical characteristics and prognosis of 6 children with idiopathic interstitial pneumonia (IIP). Methods: This retrospective study analyzed the clinical manifestations, examinations, treatment and prognosis of 6 children with IIP who were hospitalized in Children's Hospital of Nanjing Medical University from January 2015 to March 2020. Results: Of the 6 children, 2 were males and 4 were females, aged 4.8 to10.6 years. All children had a subacute onset, and presented with cough, shortness of breath and cyanosis. The lung high-resolution CT (HRCT) showed diffuse patchiness in bilateral lung fields in all the children and reticular pattern in 2 cases. Pulmonary function test found moderate to severe mixed defect in 5 children. Lung biopsy was performed in 4 children. All of the 6 children were treated with systemic glucocorticoids, of whom 2 cases had additional inhaled glucocorticoids. Four children were finally diagnosed as cryptogenic organizing pneumonia (COP), whose lung HRCT return to normal in 1-11 months. Two children were finally diagnosed as nonspecific interstitial pneumonia (NSIP), and had long-term residual fibrosis on lung HRCT. The 6 children were followed up for 1 year to 6 years and 5 months after discontinuation of systemic glucocorticoids, and all had no recurrence. Conclusions: The clinical characteristics of IIP in children are subacute onset presented with cough, shortness of breath, cyanosis and diffuse patchiness in bilateral lungs on HRCT. The common subtypes of IIP in children are COP and NSIP. Systemic glucocorticoid is effective for IIP in children and there is a good prognosis overall.
Child ; Cough/etiology* ; Cryptogenic Organizing Pneumonia ; Cyanosis/pathology* ; Dyspnea/pathology* ; Female ; Glucocorticoids/therapeutic use* ; Humans ; Idiopathic Interstitial Pneumonias/pathology* ; Lung/pathology* ; Male ; Retrospective Studies

Aged ; Bronchiectasis ; etiology ; pathology ; Comorbidity ; Dyspnea ; etiology ; pathology ; Female ; Humans ; Male ; Middle Aged ; Pulmonary Disease, Chronic Obstructive ; complications ; pathology ; Smoking ; adverse effects