Alveolar adenoma is a very rare benign intraparenchymal lung tumor originating from type II pneumocytes. It can be mistaken for other benign tumors or lung cancer in radiological images. It is especially difficult to distinguish alveolar adenoma from sclerosing hemangioma. A small aspiration biopsy specimen, such as with percutaneous needle aspiration, is insufficient for a pathological diagnosis. Surgical resection is the only method by which a pathological diagnosis can be made and the disease treated. An alveolar adenoma presenting as multiple nodules is very rare and has to our knowledge not been reported in Korea previously. Here, we report a case of alveolar adenoma in multiple nodules in a 57-year-old female and review the literature.
Adenoma* ; Biopsy, Needle ; Diagnosis ; Female ; Histiocytoma, Benign Fibrous ; Humans ; Korea ; Lung Neoplasms ; Lung* ; Middle Aged ; Needles ; Pneumocytes

We evaluated the potential ability of germanium biotite (GB) to stimulate the production of antibodies specific for foot-and-mouth disease virus (FMDV). To this aim, we measured the total FMDV-specific antibody responses and IgM production after vaccination against FMD both experimentally and in the field. GB supplementation with FMDV vaccination stimulated the production of anti-FMDV antibodies, and effectively increased IFN-gamma and TNF-alpha levels. These results suggest that GB may be a novel alternative feed supplement that can serve as a boosting agent and an immunostimulator for increasing the efficacy of FMDV vaccination in pigs.
Adjuvants, Immunologic/therapeutic use ; Aluminum Silicates/*therapeutic use ; Animals ; Antibodies, Viral/*immunology ; Antibody Formation/drug effects ; *Dietary Supplements ; Ferrous Compounds/*therapeutic use ; Foot-and-Mouth Disease/*immunology/prevention & control ; Foot-and-Mouth Disease Virus/immunology ; Germanium/*therapeutic use ; Swine ; Swine Diseases/immunology/prevention & control/*virology

Niemann-Pick disease (NPD) is an inherited metabolic disorder caused by a deficiency of the enzyme acid sphingomyelinase coded by SMPD1 gene. In contrast with type A NPD, a severe neurodegenerative disease of infancy, type B NPD patients have little or no neurodegeneration, and frequently survive into adulthood. Although over 100 mutations have been found within the SMPD1 gene causing NPD, there was only one report about SMPD1 mutation status of a Korean NPD patient. We report a case of a 32-yr-old female, who presented with thrombocytopenia without any neurologic involvement. Hepatosplenomegaly was detected by both physical examination and imaging studies, and a thoracic radiograph examination showed a pattern of interstitial lung disease. Biochemical tests revealed increased liver enzymes, cholesterol, triglyceride, and LDL-cholesterol, and decreased HDL-cholesterol. Sea-blue or foamy vacuolated histiocytes occurred in bone marrow and liver. Sequencing analysis of SMPD1 using genomic DNA from peripheral leukocytes identified a compound heterozygote of two mutations at exon 2: p.E246K and p.A357V. The former is a known mutation in an Italian patient, and the latter has not been reported yet. She has received oral rosuvastatin to treat hyperlipidemia at a dose of 10 mg per day for 4 months. This is the second report in which the mutation of SMPD1 gene was detected in a Korean NPD patient. The active genetic analysis of SMPD1 gene in patients with typical findings of type B NPD would enable us to facilitate diagnosis as well as to accumulate data on molecular characteristics of Korean NPD patients.
Adult ; Base Sequence ; Bone Marrow Cells/pathology ; Female ; Humans ; Korea ; Liver/pathology ; Niemann-Pick Disease, Type B/*diagnosis/genetics/radiotherapy ; Pregnancy ; Sea-Blue Histiocyte Syndrome/diagnosis/pathology ; Sequence Analysis, DNA ; Sphingomyelin Phosphodiesterase/genetics ; Tomography, X-Ray Computed

Henoch-Schonlein purpura (HSP) is an immunologically mediated systemic vasculitis of small blood vessels that primarily involves the skin, gastrointestinal tract, joints and kidneys. HSP is a common vasculitic syndrome in children who, in most cases, achieve complete recovery. Pulmonary hemorrhage is a very rare manifestation of HSP. The authors present a case of a 46-year-old male presenting with pulmonary hemorrhage and renal involvement and the diagnosis of HSP. The patient responded to prednisolone therapy.
Blood Vessels ; Child ; Gastrointestinal Tract ; Hemorrhage ; Humans ; Joints ; Kidney ; Male ; Middle Aged ; Prednisolone ; Purpura, Schoenlein-Henoch ; Skin ; Systemic Vasculitis

OBJECTIVES: The aim of this study was to evaluate the reliability and validity of the Korean Version of the Diagnostic Interview Schedule for Children Version IV(DISC-IV), a highly structured diagnostic interview used to assess more than 30 psychiatric disorders in children and adolescents. METHODS: A total of 91 study subjects, including 67 subjects who visited the child and adolescent psychiatry outpatient clinic at our institution and 24 community-based subjects, were assessed using the Korean Version of the DISCIV. Clinical diagnosis was used as a gold standard for the examination of the validity of the DISC-IV. Forty-four of the study subjects were randomly selected for test-retest reliability measurement. RESULTS: The validity of the Korean Version of the DISC-IV showed kappa values ranging from 0.25 to 0.40 in the clinical sample and 0.65 to 1.00 in the community sample. The sensitivities varied according to the diagnostic categories, but the specificities were excellent for all diagnostic entities. CONCLUSION: The Korean Version of the DISC-IV showed good reliability and validity in Korean children and adolescents. The Korean Version of the DISC-IV might be a useful tool for assessing psychiatric disorders in children and adolescents.
Adolescent ; Adolescent Psychiatry ; Ambulatory Care Facilities ; Appointments and Schedules* ; Child* ; Diagnosis ; Humans ; Reproducibility of Results*

Anticonvulsant hypersensitivity syndrome(AHS) is an uncommon, but potentially fatal, multi-systemic disorder that occurs after exposure to phenytoin, carbamazepine, phenobarbital. Clinical features and laboratory data are diverse and variable. The multi-systemic reaction presents as fever, skin eruptions, lymphadenopathy, hematologic abnormality, and hepatitis. It is postulated that this mechanism can cause deficient enzymatic reduction by epoxide hydrolase. The diagnosis of AHS is made by reviewing the history of drug exposure and clinical course. It is important to discontinue use of the offending drug suspected for AHS and to closely observe patients with anticonvulsant therapy. We experienced a case of anticonvulsant hypersensitivity syndrome developed by carbama-zepine, presented with pseudolymphoma in lymph node biopsy and improved by discontinuing the drug and implementing steroid treatment. We report this case with pathologic findings and a brief review.
Biopsy ; Carbamazepine* ; Diagnosis ; Fever ; Hepatitis ; Humans ; Hypersensitivity* ; Lymph Nodes ; Lymphatic Diseases ; Phenobarbital ; Phenytoin ; Pseudolymphoma* ; Skin

BACKGROUND: Bronchial asthma is characterized by a reversible airway obstruction, airway hyperresponsiveness, and eosinophilic airway inflammation. The bronchodilator response(BDR) after short acting beta agonist inhalation and PC20 with methacholine inhalation are frequently used for diagnosing bronchial asthma, However, the relationship between the presence of a bronchodilator response and the degree of airway hyperresponsiveness is uncertain. Therefore, the availability of a eosinophil cationic protein(ECP) and a correlation ECP with a bronchodilator response and airway hyperresposiveness was investigated. METHOD: A total 71 patients with a moderate to severe degree of bronchial asthma were enrolled and divided into two groups. 31 patients with a positive bronchodilator response and 38 patients with a negative bronchodilator response were evaluated. In both groups, the serum ECP, peripheral blood eosinophil counts, and total IgE level were measured and the methacholine bronchial provocation test was examined. RESULTS: There were no differences observed in age, sex, atopy, and baseline spirometry in both groups. The peripheral eosinophil counts showed no difference in both groups, but the ECP level in group 1 (bronchodilator responder group) was higher than in group 2(non-bronchodilator responder group) (22.4±20.7 vs 14.2±10.4, mean±SD). The PC20 in group 1 was significantly lower than in group 2 (1.14±1.68 vs 66±2.98). There was a significant positive correlation between the BDR and ECP, and a negative correlation between the bronchial hyperresponsiveness and ECP. CONCLUSION: The bronchodilator response significantly correlated with the bronchial hyperresponsiveness and serum ECP in the moderate to severe asthma patients. Hence, the positive bronchodilator response is probably related with active bronchial inflammation and may be used as a valuable index in treatment, course and prognosis of bronchial asthma.
Airway Obstruction ; Asthma ; Bronchial Provocation Tests ; Eosinophil Cationic Protein* ; Eosinophils ; Humans ; Immunoglobulin E ; Inflammation ; Inhalation ; Methacholine Chloride ; Prognosis ; Spirometry

Recently we have experienced one case of pulmonary lymphangioleiomymatosis(LAM). A 49 year-old woman visited the outpatient department complaining of longstanding dyspnea, which was aggravated by exercise. Although the chest PA film showed nothing more than a slight increase in interstitial marking, a lung HRCT revealed multiple cystic lesions of a similar size that were scattered through out the whole field in both lungs. An abdominal CT detected an angiomyolipoma located in the midbody of the left kidney. Video-assisted thoracic surgery(VATS) was performed for the pathologic diagnosis. On gross examination of the biopsy lung, a pulmonary LAM was confirmed by a finding of smooth muscle proliferation in the interstitum of the lung. After the final diagnosis, oral medroxyprogesterone was prescribed and she is presently in a stable condition.
Angiomyolipoma* ; Biopsy ; Diagnosis ; Diagnosis, Oral ; Dyspnea ; Female ; Humans ; Kidney ; Lung ; Lymphangioleiomyomatosis* ; Medroxyprogesterone ; Muscle, Smooth ; Outpatients ; Thoracic Surgery, Video-Assisted ; Thorax ; Tomography, X-Ray Computed

Membranous fat necrosis is a variant of fat necrosis characterized by the presence of membranocystic foci which is lined by eosinophilic, homogeneous and crenulated membrane that has pseudopapillary projections. Membranous fat necrosis may be idiopathic or has been associated with many local and systemic diseases. The pathogenesis of membranous fat necrosis is uncertain but trauma may be suspected in our case. We describe a case of lipoma with membranous fat necrosis.
Eosinophils ; Fat Necrosis* ; Lipoma* ; Membranes

BACKGROUND: The outcome of hematopoietic stem cell transplantation (HSCT) for patients with severe aplastic anemia (SAA) in Seoul National University Hospital was analyzed retrospectively. METHODS: Between January, 1990 and March, 1999, 25 patients with SAA underwent HSCT. Their medical records were reviewed. Statistical analyses were done about survival and complication after HSCT. RESULTS: The median age of patients was 22 (range, 14~43) and male to female ratio was 18 : 7. Twenty two were HLA matched non- identical siblings. Three were one identical twin, one one-locus mismatched father and one HLA matched unrelated donor, respectively. Conditioning regimens were CY/TLI (cyclophosphamide, total lymphoid irradiation) for 18 patients, CY/ATG (CY, antithymocyte globulin) for 3, CY/ buffy (CY, unirradiated buffy- coat) for 2, CY/ ATG/TLI for 1, BU/CY (busulfan, CY) for 1. For prophylaxis of graft-versus-host disease (GVHD), cyclosporine and methotrexate were used in all patients except for identical twin. The median nucleated cell dose given to patients was 4.5x108/kg (range, 2.0~5.9). All evaluable patients achieved absolute neutrophil count of 500/microliter after median 17 days of HSCT (range, 12~27) and untransfused platelet count over 20,000/microliter after median 21 days of HSCT (range, 13~67). Six patients (24%, grade I : 3, II : 1, III : 1, IV : 1) developed acute GVHD and 8 (32%, limited : 4, extensive : 4) developed chronic GVHD. Hepatic venoocclusive disease (VOD) occurred in 2 patients (8%). Rejection occured in 4 patients (16 %), but among 22 allogeneic transplant recipients from HLA matched siblings, only one (5%) lost graft. After a median follow-up of 32 months (range 9~120 months), 5 year overall survival of all patients was 87%, and that of 22 allogeneic recipients from HLA matched sibling donors was 95%. Four patients (16%) died. Causes of death were VOD in one case, rejection with pneumonia one, acute GVHD one. One died from traffic accident in a cured state. CONCLUSION: Experiences from our center suggest that HSCT is an effective treatment for patients with severe aplastic anemia. Long- term survival is especially excellent for patients who have matched related donors.
Accidents, Traffic ; Anemia, Aplastic* ; Cause of Death ; Cyclosporine ; Fathers ; Female ; Follow-Up Studies ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Humans ; Male ; Medical Records ; Methotrexate ; Neutrophils ; Platelet Count ; Pneumonia ; Retrospective Studies ; Seoul ; Siblings ; Tissue Donors ; Transplantation ; Transplants ; Twins, Monozygotic ; Unrelated Donors