BACKGROUND: Pulmonary Langerhans cell histiocytosis (PLCH) is characterized by a proliferation of Langerhans cells and this results in granulomas that involve multiple organs of the body. Because the incidence of PLCH is very low in Korea and worldwide, collecting the clinical data of patients with PLCH nationwide is needed to determine the clinical features of Korean patients with PLCH. METHODS: The patients with PLCH confirmed by biopsy at any body site were included and the patients should have lung lesions present. A questionnaire that had items on the symptoms, lung function tests, the roentgenographic findings and the treatment was collected retrospectively at a Korean ILD Research Meeting. RESULTS: A total of 56 cases were collected. The number of males and females was 48 and 8, respectively, and their median age was 43 years (range: 18~67 years). The patients were current or ex-smokers in 79% of the cases. The most frequent symptom was coughing (39%), followed in decreasing order by dyspnea (38%), sputum (20%) and chest pain (20%). Pneumothorax was observed in 16 (29%) patients. Lung function tests showed a normal, restrictive, mixed or obstructive pattern in 26 (61%), 7 (16%), 7 (16%) and 3 patients (7%), respectively. Nodular-cystic lesion was most frequently observed in 59% of the patients on HRCT. The lung lesions were located in the middle and upper lobes in almost the cases. The median follow-up period was 90 months (range: 1~180 months) and only two patients died during this period. CONCLUSION: This study provides a national survey of the patients with PLCH during a long follow-up period.
Biopsy ; Chest Pain ; Cough ; Dyspnea ; Female ; Follow-Up Studies ; Granuloma ; Histiocytosis ; Histiocytosis, Langerhans-Cell ; Humans ; Incidence ; Korea ; Langerhans Cells ; Lung ; Lung Diseases ; Male ; Pneumothorax ; Surveys and Questionnaires ; Respiratory Function Tests ; Retrospective Studies ; Sputum

PURPOSE: The aim of this study was to investigate the epidemiologic status of Kawasaki disease (KD) in infants < or =6 months of age. METHODS: For the epidemiologic study of KD in Korea, data from 22,674 KD patients were collected from 1997 to 2005 on a 3 -year basis by a retrospective survey. From this survey, data of 1,739 KD patients < or =6 months of age were analyzed and compared with those of KD patients >6 months of age. RESULTS: A total of 1,739 patients < or =6 months of age, including 1,088 males and 651 females, represented 7.7% of total KD patients. These subjects included 22 patients aged <1 month, 171 patients aged 1-2 months, 304 patients aged 2-3 months, 407 patients aged 3-4 months, 372 patients aged 4-5 months, and 463 patients aged 5-6 months. Their mean age of onset was 4.3 months, and the male-to-female ratio was 1.67 :1 . Incidences of coronary arterial (CA) abnormalities (21.0% versus 18.7%) and CA aneurysms (4.7% versus 3.1%) detected by echocardiography showed differences between patients with KD younger and older than 6 months, respectively. CONCLUSION: Comparison of data from KD patients >6 months old with data from 1,739 KD patients < or =6 months old showed significantly higher incidences of CA abnormalities and CA aneurysms in the younger patients.
Age of Onset ; Aged ; Aneurysm ; Coronary Aneurysm ; Echocardiography ; Epidemiologic Studies ; Female ; Humans ; Incidence ; Infant ; Korea ; Male ; Mucocutaneous Lymph Node Syndrome ; Retrospective Studies

PURPOSE: To determine the diagnostic accuracy of four different sequences : moderately T2 weighted, two heavily T2-weighted single shot turbo spin-echo sequence and breath-hold axial-2D balanced turbo field-echo sequence(bFFE) for characterization of focal lesions. MATERIALS AND METHODS: During the 3-month period between June and August 2005, seventy-six patients were proved to have ninety-three focal hepatic lesions on MR imaging. The patients consisted of 49 men and 27 women (age range, 15-75 years; mean age, 56.23 years). All MR images were acquired on a 1.5-T MR using the following sequences: 1. A breath-hold axial T2-weighted single shot turbo spin-echo sequence, 2. a breath-hold axial-2D balanced turbo field-echo sequence. Two radiologists performed quantitative analysis. Another radiologist measured the lesion-to-liver contrast-to-noise ratio at the region-of-interest in the four sequences. RESULTS: There was no significant difference in inter-observer variability between the four sequences. The accuracy for both cyst and malignancy of moderate T2 weighted MRI (echo time: 80 msec) was also highest. There was significant difference for lesion characterization between moderate T2 weighted MRI and balanced steady state procession (p-value: 0.004) in the second reader. For longer echo time, the CNR of cystic lesions were markedly increased in comparison to lesions of other component. CONCLUSION: The accuracy and inter-observer variability of single shot turbo spin echo T2 weighted sequence was higher than bFFE. Although there was no statically significant difference, moderate T2 weighted MRI (echo time: 80 msec) was more accurate than heavily T2 weighted sequence (echo time: 300 msec). If the results for lesion characterization is equivocal in TE 80, the addition of heavily T2 weighted MRI (echo time: 180 msec) can be helpful.
Female ; Humans ; Liver* ; Magnetic Resonance Imaging ; Male ; Observer Variation

PURPOSE: The aim of this study was to investigate the epidemiologic and clinical profiles of Kawasaki disease (KD) in children 8 years of age and older. METHODS: For the epidemiologic study of KD in Korea, data of total 15, 692 KD patients were collected from 1994 to 2002 on a 3 year basis, by the retrospective survey. Among them, data of 211 KD patients 8 years of age and older were analyzed and compared with those of the KD patients less than 8 years of age. RESULTS: 211 patients 8 years of age and older (1.3% of total KD patients) with the mean age of 9.8 years, included 142 cases aged 8-10 years, 42 cases aged 10-12 years, and 27 cases aged 12 years and older. The male-to-female ratio was 1.9: 1, the recurrence rate was 3.8%, and the prevalence of cases in sibling was zero. Coronary arterial (CA) abnormalities were detected by echocardiography in 25.4% of the cases, including 7.6% of CA aneurysm cases. There was 1 case complicated by myocardial infarction. CONCLUSION: Comparing with the data of the KD patients less than 8 years of age, data of 211 patients 8 years of age and older showed significantly higher incidences of CA abnormalities (25.4%: 19.8%) and CA aneurysms (7.6%: 4.0%).
Aneurysm ; Child* ; Coronary Aneurysm ; Echocardiography ; Epidemiologic Studies* ; Epidemiology ; Humans ; Incidence ; Korea ; Mucocutaneous Lymph Node Syndrome* ; Myocardial Infarction ; Prevalence ; Recurrence ; Retrospective Studies ; Siblings

Human follicular fluid (HFF) includes various biologically active proteins which can affect follicle growth and oocyte fertilization. Thus far, these proteins from mature follicles in human follicular fluid have been poorly characterized. Here, two-dimensional polyacrylamide gel electrophoresis (2-DE) with matrix-assisted laser desorption/ionization-mass spectrometry (MALDI-MS) was used to identify new proteins in HFF. Mature follicular fluids were obtained from five females after oocyte collection during in vitro fertilization (IVF). We directly rehydrated HFF samples, obtained high-resolution 2-DE maps, and processed them for 2-DE and MALDI-MS. One hundred eighty spots were detected and 10 of these spots were identified. By the 2-DE database, six of them had been reported, as proteins already existing in HFF. Hormone sensitive lipase (HSL), Unnamed protein product 1 (UPP1), Unnamed protein product 2 (UPP2), and apolipoprotein A-IV precursor were newly detected. HSL and apolipoprotein A-IV participate in lipid metabolism. UPP1 has a homology with selenocysteine lyase. We found by RT-PCR that these genes are expressed from human primary granulosa cells. The proteins identified here may emerge as potential candidates for specific functions during folliculogenesis, hormone secretion regulation, or oocyte maturation. Further functional analysis of these proteins is necessitated to determine their biological implications.
Adult ; Electrophoresis, Gel, Two-Dimensional/*methods ; Female ; Follicular Fluid/*chemistry/metabolism ; Gene Expression ; Granulosa Cells/metabolism ; Humans ; Ovarian Follicle/*chemistry/metabolism ; Proteins/*analysis/genetics ; RNA, Messenger/genetics/metabolism ; Research Support, Non-U.S. Gov't ; Reverse Transcriptase Polymerase Chain Reaction ; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization

BACKGROUND: This study compared the bronchodilator efficacy and safety of tiotropium inhalation capsules (18microgram once daily) with a ipratropium metered dose inhaler (2 puffs of 20microgram q.i.d.) in patients with chronic obstructive pulmonary disease (COPD). METHOD: After the initial screening assessment and a two-week run-in period, patients received either tiotropium 18microgram once daily or ipratropium 40microgram four times daily over a period of 4 weeks in a double blind, double dummy, parallel group study. The outcome measures were the lung function, the daily records of the peak expiratory flow rate (PEFR), the patients' questionnaire, and the use of concomitant salbutamol. The forced expiratory volume in one second (FEV1) and the forced vital capacity (FVC) were measured 5 minutes before inhalation, and 0.5, 1, 2 and 3 hours after inhaling the study drug on days 0, 14 and 28. RESULT: In 16 centers, 134 patients with a mean (SD) age of 66 (7) years and a predicted FEV1 of 42 (12)% were analyzed. The trough FEV1 response was significantly higher in the tiotropium group than in the ipratropium group after a four-week treatment period. The weekly mean morning PEFR of the tiotropium group was consistently higher than that of the ipratropium group during the 4-week treatment period with differences ranging from 12.52 to 13.88 l/min, which were statistically significant. Tiotropium was well tolerated by the COPD patients during the 4-week treatment period and had a similar safety profile to ipratropium. CONCLUSION: This study shows that tiotropium administrated once daily has a superior bronchodilator effect with a similar safety profile in treating COPD patients compared with ipratropium, inhaled four times daily.
Adult* ; Albuterol ; Bronchodilator Agents ; Capsules ; Forced Expiratory Volume ; Humans ; Inhalation ; Ipratropium* ; Lung ; Mass Screening ; Metered Dose Inhalers ; Outcome Assessment (Health Care) ; Peak Expiratory Flow Rate ; Pulmonary Disease, Chronic Obstructive* ; Surveys and Questionnaires ; Vital Capacity ; Tiotropium Bromide

PURPOSE: High dose methotrexate (HDMTX) dramatically increases survival rate, which is related to the histologic response of tumornecrosis rate in the treatment of osteosarcoma. But, the study of tumor necrosis rate after preoperative chemotherapy with MTX has notbeen systemically evaluated. We analysed tumor necrosis rate versus related parameters such as the MTX serum peak level, age, sex, location, pathologic type under two chemotherapeutic protocols (modified T10 & T20). MATERIALS AND METHODS: From June 1995 to June 2001, 44 cases of Enneking stage IIB osteosarcoma patients (29 males, 15 females, mean age 18) were treated with two similar neoajuvant chemotherapeutic protocols involving intravenous injections of MTX, ifosphamide, adriamycin and the intra-arterial injection of cisplatin preoperatively. The histologic response to the chemotherapy was graded as complete(100% necrosis) or incomplete (less than 100%). RESULTS: Of 44 patients, 26 showed serum MTX concentrations greater than 1, 000 micromol/L. Complete response was seen in 9 patients with a peak serum MTX level of 1, 000 micromol/L or greater. Complete response was seen in 34.6% of patients with 1, 000 mol/L orgreater MTX serum peak level (p=0.031). Seven of 15 female patients showed complete response. Three of 29 male patients showed complete response. The complete response rate of females was significantly higher than that of males (p=0.019). Histological response was not significantly correlated with other variables. CONCLUSION: In this study, the rate of complete necrosis was significantly higher in patients with a preoperative peak serum MTX levelover 1, 000 micromol/L, especially in females, than in those with a serum MTX below this level. We suggest the preoperative dose of MTXserum peak level should be maintained at over 1, 000 micromol/L, and suggest that the preoperative MTX dose should be maintained at ahigh level in order to increase the tumor necrosis rate.
Cisplatin ; Doxorubicin ; Drug Therapy* ; Female ; Humans ; Injections, Intra-Arterial ; Injections, Intravenous ; Male ; Methotrexate ; Necrosis* ; Osteosarcoma* ; Survival Rate

Pancreas divisum is the most common congenital anomaly of the pancreas. Whether pancreas divisum is the cause of chronic abdominal pain, acute pancreatitis, or chronic pancreatitis is a matter of controversy yet. We recently experienced a case of acute pancreatitis due to an impacted stone at the orifice of the minor papilla in a 58 year-old man with pancreas divisum who showed clear radiologic evidences of dorsal chronic pancreatitis. The bout of acute pancreatitis recovered rapidly after endoscopic treatment (minor papilla sphincterotomy, stone removal, and endoscopic nasopancreatic drainage). We herein report an unusual manifestation of pancreas divisum in which endoscopic treatment was effective.
Abdominal Pain ; Humans ; Middle Aged ; Pancreas* ; Pancreatitis* ; Pancreatitis, Chronic

Alfa-interferon(IFN) has become the major therapeutic modality for chronic viral hepatitis. The spectrum of side effects is one of the main drawbacks of this treatment. Alfa-INF is known to lead to neuropsychiatric sym ptoms. Neuropsychiatric toxicity, including cognitive dys function, irritability, personality changes and emotional instability cause interpersonal problems, discontinuation of work and domestic discord. Other more serious IFN- induced signs of neurotoxicity include delirium, depres sion and seizures. The psychiatric side effects fell into three categories: an organic personality syndrome charac terized by irritability, and short temper; an organic affective syndrome marked by extreme emotional lability, depression and tearfulness; and a delirium marked by clouding of consciousness, agitation, paranoia, and suicial potential. These complications are thought to be revers ible with dose reduction or cessation of therapy. There fore it is important that physicians, patients and their families are informed about the potential risk of the emotional and psychiatric disturbances that can occur during alfa-INF therapy. We report three patients who had neuropsychiatric complications during long-term interferon alfa therapy.
Consciousness ; Delirium ; Depression ; Dihydroergotamine ; Hepatitis* ; Humans ; Interferon-alpha* ; Interferons* ; Paranoid Disorders ; Seizures

Ebstein's anomaly is a complex malformation that can be treated by various surgical techniques, either repair or replacement of the abnormal tricuspid valve, with variable results. The essence of the malformation is the downward displacement of the septal and posterior leaflets into the ventricle, resulting in the formation of an atrialized portion of the right ventricle. The aim of surgical repair is to correct the tricuspid valve dysfunction and to plicate the atrialized portion of the right ventricle A 12-months old female was admitted with the diagnosis of Carpentier type A of Ebstein's anomaly with severe tricuspid regurgitation. She successfully underwent operation with vertical plication of right ventricle and reimplantation of tricuspid leaflets. Postoperatively cardiac size was significantly reduced and tricuspid regurgitation was trivial in echocardiography. She was diacharged the 14th postoperative day.
Diagnosis ; Ebstein Anomaly* ; Echocardiography ; Female ; Heart Ventricles ; Humans ; Infant ; Replantation ; Tricuspid Valve ; Tricuspid Valve Insufficiency