• Neonatal Medicine
• /
• v.17 no.1
• /
• pp.109-115
• /
• 2010

Persistent pulmonary hypertension in newborns (PPHN) is a disorder of the vascular transition from fetal to neonatal circulation. It results in cyanosis due to right-to-left shunting of the blood through the ductus arteriosus and/or foramen ovale manifesting as hypoxemic respiratory failure. We managed two cases of PPHN after meconium aspiration with high frequency oscillating ventilators and inhaled nitric oxide. They did not respond to conventional management. Veno-venous extracorporeal membrane oxygenation (ECMO) was provided, and ECMO weaning was possible resulting survivals in two cases. We report two PPHN cases, which were treated successfully with veno-venous ECMO for the first time in Korea.

• Tuberculosis and Respiratory Diseases
• /
• v.46 no.1
• /
• pp.110-115
• /
• 1999

Pulmonary histiocytosis X is a granulomatous disorder of the lung of unknown cause. Patients with this disease often complain of cough, dyspnea on exertion and, occasionally, chest pain from pneumothorax or bone involvement. However, DI is uncommon in these patients. We report a case of primary pulmonary histiocytosis X with central diabetes insipidus. A 23-year-old man presented with dyspnea suffered from dry cough, exertional dyspnea, polydipsia and polyuria for 4 months. He was a heavy smoker. He was found to have reticulonodular interstitial opacities on chest X-ray film. High-resolution computed tomography revealed thin-walled cysts of various sizes in both lungs. Open lung biopsy was done. On light microscopic examination revealed proliferation and infiltration of Langerhans cells. Immunohistochemically, Langerhans cells showed strong cytoplasmic staining with S-100 protein and electronmicroscopic examination showed Birbeck granules in Langerhans cells. Water deprivation test showed central-type diabetes insipidus and brain MRI showed no abnormal lesion on suprasellar region. Smoking cessation was recommended. He was treated with oral desmopressin.

• Tuberculosis and Respiratory Diseases
• /
• v.65 no.4
• /
• pp.323-327
• /
• 2008

Systemic lupus erythematosus (SLE) is a multisystem disorder where the etiology is not clearly known. Symptomatic chronic interstitial pneumonitis is an uncommon manifestation, with a reported prevalence of 3~13%. Achalasia is rare disease that presents with failure in the relaxation of the esophagus sphincter. A 22-year-old woman was admitted to our hospital because of fever, cough and dyspnea. The patient had a history of pericardial effusion and Raynaud's phenomenon. The results of laboratory tests indicated the presence of lymphopenia and included positive antibody tests for antinuclear antibody and anti Sm antibody. A chest X-ray demonstrated the presence of peribronchial infiltration on both lung fields. A Chest CT image showed interlobar septal thickening, ground-glass opacity and a honeycomb appearance in both lung fields and esophageal dilatation with air fluid level. An esophagogram showed the presence of dilated esophagus ends that represented the non-relaxed lower esophageal sphincter. Manometry demonstrated incomplete sphincter relaxation. The case was diagnosed as systemic lupus erythematosus associated with interstitial pneumonia and achalasia.

• Tuberculosis and Respiratory Diseases
• /
• v.48 no.6
• /
• pp.973-979
• /
• 2000

A glycogen storage disease(GSD) type I is a metabolic disease caused by a deficiency in one of the components of the glucose-6-phosphatase(G-6-Pase) system. This disorder results in hypoglycemia, hepatomegaly, lactic acidemia, hyperlipidemia, and hyperuricemia. Comon long(-)term complications include growth retaradation, gout, hepatic adenomas, osteoporosis and renal disease. However the cardiovascular system is rarely involved, and only six cases of pulmonary hypertension associated with GSD I have been reported in the literature. We experienced a case of pulmonary hypertension with type I GSD. A 31-year-old rnan, who had discovered type I GSD and received portocaval shunt operation 22 years ago, was admitted to the hospital with the chief complaint of dyspnea. Echocardiographic examination and cardiac catheterization revealed severe pulmonary hypertension. Nitric oxide and oral prostacycline derivative(beraprost) were tried without acute favorable response. After one year with beraprost, dyspnea, exercise capacity and hemodynamic parameters were improved. We report this case with a review of the literature.

• Tuberculosis and Respiratory Diseases
• /
• v.57 no.1
• /
• pp.55-60
• /
• 2004

Anticonvulsant hypersensitivity syndrome (AHS) is an uncommon, but potentially fatal and mutilsystemic disorder that occurs after exposure to the arene oxide-producing anticonvulsants-carbamzepine, phenobarbital and phenytoin. The multisystemic reactions include fever, skin eruptions, lymphadenopathy, hematologic abnormality and hepatitis. The diagnosis of AHS is made by history of drug exposure and clinical course. No specific treatments are proved as benefit except discontinuing the offending drug and trying the steroids in some severe cases. We report a case of carbamazepine induced anticonvulsant hypersensitivity syndrome characterized by skin rash, eosinophilia, subcarinal lymphadenopathy and eosinophilic pneumonia. The patient was resolved completely after only discontinuing carbamazepine.

• Tuberculosis and Respiratory Diseases
• /
• v.62 no.2
• /
• pp.119-124
• /
• 2007

Sarcoidosis is a multi-system granulomatous disorder of an unknown etiology and affects individuals worldwide. It is characterized pathologically by the presence of non-caseating granulomas in more than one involved organ. However, pleural involvement of sarcoidosis is rare and there are no reported cases in Korea. Traditionally, sarcoidosis has often been treated with systemic corticosteroids or cytotoxic agents. In particular, chylothorax with sarcoidosis is usually treated with corticosteroid for approximately 3~6 months, followed by repeated therapeutic thoracentesis, talc pleurodesis, dietary treatment, or thoracic duct ligation where needed. We encountered a 46 years old female patient presenting with cough, dyspnea and both hilar lymphadenopathy (stage I) on chest radiograph. The patient was diagnosed with a non-caseating granuloma, sarcoidosis by a mediastinoscopic biopsy. For one month, she had suffered from dyspnea due to right side pleural effusion, which was clearly identified as a chylothorax on thoracentesis. Corticosteroid therapy with dietary adjustment was ineffective. She was treated successfully with a subcutaneous injection of octreotide for 3 weeks and oral corticosteroid. We report a case of successful and rapid treatment of chylothorax associated with sarcoidosis using octreotide and oral corticosteroid.

• Tuberculosis and Respiratory Diseases
• /
• v.54 no.6
• /
• pp.628-634
• /
• 2003

Bronchiectasis is defined as an abnormal, irreversible dilatation of the bronchi, which may result from a number of possible causes, and the recognition of these causes may lead to a specific management strategy. Immunodeficiency is known as one of the conditions associated with bronchiectasis. X-linked agammaglobulinemia is a rare inheritable immunodeficiency disorder, caused by a differentiation block, leading almost to the complete absence of B lymphocytes and plasma cells. The affected protein is a cytoplasmic protein tyrosine kinase, Bruton's tyrosine kinase. The early detection and treatment with immunoglobulin replacement are most important for the management of recurrent infections and for reducing severe complications. We report a 20-year-old male patient, with X-linked agammaglobulinemia associated with bilateral bronchiectasis, carrying a missense mutation(R520P) in the BTK gene.

• Tuberculosis and Respiratory Diseases
• /
• v.67 no.5
• /
• pp.422-429
• /
• 2009

Background: The discomfort caused by chronic cough, that is persistent for more than 3 weeks, causes a number of patients to seek medical attention. However, the underlying disorder often remains undetermined despite thorough examinations, and is considered to be idiopathic. This study compared the efficacy of inhaled corticosteroid with conventional cough suppressants on chronic idiopathic cough. Methods: Eligible patients with chronic idiopathic cough were randomly assigned to either the inhaled fluticasone group or the codeine plus levodropropizine oral administration group. The subjects in each group took their planned medication for 2 weeks. After the trial, comparative analyses of outcomes were performed in terms of the remnant cough (%) at the end of treatment, drug compliance, and adverse drug events. Results: Seventy-seven patients were enrolled in this randomized trial; 38 to the inhaled fluticasone group and 39 to the codeine plus levodropropizine group. The remnant cough was 41.0${\pm}$35.8% in the inhaled fluticasone group, and 32.4${\pm}$32.0% in the codeine+levodropropizine group (p=0.288). Drug compliance was 95.4${\pm}$7.4% and 81.8${\pm}$18.6% in the inhaled fluticasone and the codeine+levodropropizine group, respectively (p<0.001). Nine patients had adverse drug events in the codeine+levodropropizine group compared to one in the inhaled fluticasone group (p<0.001). Conclusion: Short-term inhaled corticosteroid is not inferior to conventional antitussive agents in controlling chronic idiopathic cough without significant adverse events.

• Tuberculosis and Respiratory Diseases
• /
• v.69 no.2
• /
• pp.119-123
• /
• 2010

Bronchobiliary fistula is a rare disorder consisting of an abnormal communication between the bronchial tree and the biliary duct. In Western countries, trauma, postoperative biliary stenosis, and biliary lithiasis are the predominant causative factors of bronchobiliary fistula. Bilioptysis (bile stained sputum) is a pathognomic finding for bronchobiliary fistula. To date, there are just a few reported cases of bronchobiliary fistula after sclerosis of a liver cyst. We describe the case of a 74-year-old woman who developed bronchobiliary fistula after sclerosing therapy of a liver cyst. The diagnosis was confirmed by the presence of bilioptysis and the chest and dynamic liver CT findings. The patient was successfully treated with antibiotics and percutaneous transhepatic catheter drainage.

• Biomolecules & Therapeutics
• /
• v.32 no.6
• /
• pp.744-758
• /
• 2024

Asthma is a chronic inflammatory disorder of the lungs that results in airway inflammation and narrowing. BS012 is an herbal remedy containing Asarum sieboldii, Platycodon grandiflorum, and Cinnamomum cassia extracts. To elucidate the anti-asthma effect of BS012, this study analyzed the immune response, respiratory protection, and changes in metabolic mechanisms in an ovalbumin-induced allergic asthma mouse model. Female BALB/c mice were exposed to ovalbumin to induce allergic asthma. Bronchoalveolar lavage fluid and plasma were analyzed for interleukin and immunoglobulin E levels. Histological analyses of the lungs were performed to measure morphological changes. Apoptosis-related mediators were assayed by western blotting. Plasma and lung tissue metabolomic analyses were performed to investigate the metabolic changes. A T-helper-2-like differentiated cell model was used to identify the active components of BS012. BS012 treatment improved inflammatory cell infiltration, mucus production, and goblet cell hyperplasia in lung tissues. BS012 also significantly downregulated ovalbumin-specific immunoglobulin E in plasma and T-helper-2-specific cytokines, interleukin-4 and -5, in bronchoalveolar lavage fluid. The lungs of ovalbumin-inhaled mice exhibited nerve growth factor-mediated apoptotic protein expression, which was significantly attenuated by BS012 treatment. Ovalbumin-induced abnormalities in amino acid and lipid metabolism were improved by BS012 in correlation with its anti-inflammatory properties and normalization of energy metabolism. Additionally, the differentiated cell model revealed that N-isobutyl-dodecatetraenamide is an active component that contributes to the anti-allergic properties of BS012. The current findings demonstrate the anti-allergic and respiratory protective functions of BS012 against allergic asthma, which can be considered a therapeutic candidate.