• Tuberculosis and Respiratory Diseases
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• v.65 no.5
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• pp.410-415
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• 2008

Bronchiolitis obliterans (BO) is a serious noninfectious complication following an allogeneic bone marrow transplant (BMT). A 21-year-old female received an allogeneic BMT as a treatment for myelodyplastic syndrome. Four months after the BMT, progressive dyspnea developed and BO was also diagnosed by a lung biopsy. The patient was administered steroid and immunosuppressive agents for 1 year but there was no improvement in pulmonary function. Azithromycin was prescribed (500 mg q.d. for 3 days followed by 250 mg three time a week) because macrolides might decrease the inflammatory reaction leading to BO. The patient's pulmonary function improved after administration of azithromycin for 1 year. The forced expiratory volume in a one second ($FEV_1$) increase was 220 mL (28.2%) and the forced vital capacity (FVC) increase was 460 mL (25.7%). We report the improvement in the pulmonary function after the administration of azithromycin for 1 year in a patient with BO after a BMT.

• Tuberculosis and Respiratory Diseases
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• v.44 no.5
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• pp.1083-1093
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• 1997

Background : So far, there have been numerous reports on organ damage due to cryptococcosis, however, cases of lung localization have been infrequently reported. Recently pulmonary cryptococcosis has been reported more frequently than before due to enhanced diagnostic techniques and increased underlying diseases. Method : The author, therefore, analyzed the clinical manifestations of 5 cryptococcosis cases that we experienced at Hanyang University Hospital from 1985 to 1996 and 9 cases reported in Korea from 1984 and 1996 retrospectively. The following results were obtained. Results : Cryptococcosis occurred frequently over sixth decade and the male to female ratio was 3.6 : 1. Underlying diseases included acute rejection after kidney transplantation, rheumatoid arthritis, autoimmune hepatitis, diabetes mellitus and state of bilateral adrenalectomy. Remaining 8 cases had no evidence of an underlying disease. Because the symptoms were subacute & nonspecific, and not improved by conventional antibiotics, 6 patients of 14 pulmonary cryptococcosis patients were treated as pulmonary tuberculosis before correct diagnosis was made. There were three asymptomatic cases. According to the results of CXR, solitary alveolar consolidation was the most common finding(8 cases) followed by diffuse infiltration(5 cases). It also showed pleural effusion, hilar lymphadenopathy and cavity formation that was rarely reported in world literature. The diagnasis was made through fine needle aspiration biopsy in 10 cases, open thoracotomy in 2 cases, transbronchial lung biopsy in 1 case. and thoracentesis with pleural biopsy in 1 case. Only one case showed positive result in sputum stain and culture, serum latex agglutination test for cryptococcus neoformans. Treatment modalities were various such as fluconazole, amphotericin B, flucytosine, ketoconazole, surgery and it's combination. After 1990 year, there was a trend that fluconazole or ketoconazole are more used than other therapeutic modalities. Conclusion : Because the symptoms are subacute & nonspecific and not improved by conventional antibiotics, pulmonary cryptococcosis is likely to misdiagnosis as pulmonary tuberculosis in Korea. Because the diagnosic yield of sputum stain, culture and serologic test for pulmonary cryptococcosis is low, histologic diagnosis is need in most pulmonary cryptococcosis.

• Journal of Life Science
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• v.16 no.6
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• pp.1060-1065
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• 2006

Non-invasive evaluation of liver function in animal models remains a challenge. Hepatoscintigraphy provides information about changes in liver size and shape, and enables to understand general liver function. Futhermore it is readily used to diagnosis complications of liver transplantation like hepatitis, rejections and biliary complications. In this study, we investigated the usefulness of evaluating the liver function in miniature pigs with $^{99m}Tc-Tin$ colloid and $^{99m}Tc-DISIDA$ which are the most commonly used radiopharmaceuticals in human medicine. In result, $^{99m}Tc-Tin$ colloid was uptaked in lung, liver, gastric wall and kidney in miniature pigs. And $^{99m}Tc-DISIDA$ showed continuous uptake images of heart, lung, liver, gallbladder and duodenum, and it was similar to human's. Therefore we could conclude $^{99m}Tc-Tin$ colloid would not be suitable for evaluating hepatic function because of it's nonspecific affinity, however $^{99m}Tc-DISIDA$ scintigraphy would be an effective method for detecting hepatobiliary function in miniature pigs.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.1
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• pp.24-33
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• 2016

Mucopolysaccharidosis type VI (MPS VI) is a rare disease caused by the mutation of ARSB with prevalence range from 1/5,000 in northeast Brazil to 1/2,057,529 births in Czech Republic. In Asia, there is only one published figure in Taiwan of about 1/833,000 births. The exact prevalence in the Korean population is unknown, but we estimated the incidence of MPS VI is about 0.03/100,000 live births. Enzyme replacement therapy (ERT) with recombinant human Arylsulfatase B (rhASB) is a modality for the treatment of MPS VI that reduces the excretion of urine glycosaminoglycan (GAG) and improves joint motion, pulmonary function, and endurance. We presented the clinical features, molecular analysis and outcome of ERT in three Korean MPS VI patients. All patients had the typical characteristic clinical features of MPS IV. Short stature, dysostosis multiplex, corneal opacity and valvular heart disease were found at first presentation, while restrictive lung disease and carpal tunnel syndrome developed later in all patients. Molecular analysis demonstrated novel missense and nonsense mutation in the patients, including p.Ile 67Ser, p.Gly328Arg, $p.Arg191^*$, p.Asp352Asn, and p.Gly17Asp. After ERT, urine GAG was decreased in all patients. Skeletal involvement, corneal opacity, heart valve abnormalities and pulmonary function were not improved with ERT, but it had a better outcome on regarding joint motion and endurance. One patient underwent allogeneic bone marrow transplantation (BMT) prior to ERT, but their clinical response was not improved much after BMT. This study demonstrates clinical phenotypes and molecular analysis of the severe form of MPS VI in Korean patients.

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.42-50
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• 2003

Purpose : The acute respiratory distress syndrome(ARDS), an acute form of severe alveolar-capillary injury evolving after a direct or indirect lung insult is thought to be a common cause of respiratory failure though not many clinical studies on the subject have been made yet. Methods : Between January 1992 and December 2001, we conducted a retrospective study on 33 children who fulfilled the definition of the ARDS recommended by the American-European Consensus Conference in 1994. Results : A total of 33 patients(20 boys and 13 girls) were selected. Their age ranged from 4 months to 12 years with seven children younger than 1 year. The overall mortality rate was 78.8% and no significant difference was noted based on age or sex. Concerning seasonal variation, incidence of the ARDS increased in spring, especially in May(21.2%). Pneumonia(66.7%) was found to be the most common risk factor of the ARDS followed by sepsis(24.2%) and aspiration(3.0%). In immune compromised children(six cases), including a recipient of bone marrow transplantation, the mortality rate was 100%. Compared with children with multiple organ failure recording a 83.3% mortality rate, those with isolated respiratory failure, showed a lower mortality rate of 33.3%, although stastistically insignificant. Between survivor and non-survivor groups, significant differences were shown in hematocrit, $PaO_2$, $PaCO_2$, PEEP, and $PaO_2/FiO_2$ on the seventh day after the onset of the ARDS. Conclusion : According to our study, respiratory failure proved to have a great effect on mortality rate in the ARDS. More aggressive intervention and further studies on this subject should be done to improve the survival rate.