Good's syndrome is extremely rare. This adult-onset condition is characterized by a thymoma with immunodeficiency, low B- and T-cell counts, and hypo-gammaglobulinemia. The initial clinical presentation is either a mass-lesion thymoma or a recurrent infection. Patients with Good's syndrome are very susceptible to infections; common respiratory and opportunistic infections can be life-threatening. There are no reports of granulomatous lung disease in patients with Good's syndrome, although it has been observed in patients with common variable immunodeficiency, of which Good's syndrome is a subset. We describe a 53-year-old male thymoma patient who presented with respiratory symptoms caused by granulomatous lung disease and an opportunistic infection. He died of uncontrolled fungal infection despite repeated intravenous immunoglobulin and supportive care. Clinicians should look for evidence of immunologic dysfunction in thymoma patients presenting with severe recurrent infections, especially opportunistic infections.
Fatal Outcome ; Granuloma, Respiratory Tract/diagnosis/*etiology/therapy ; Humans ; Immunologic Deficiency Syndromes/*complications/immunology/pathology ; Lung Diseases/diagnosis/*etiology/therapy ; Male ; Middle Aged ; Thymoma/*complications/immunology/pathology ; Thymus Neoplasms/*complications/immunology/pathology

A coronary arteriovenous (AV) fistula consists of a communication between a coronary artery and a cardiac chamber, a great artery or the vena cava. It is the most common anomaly that can affect coronary perfusion. Yet bilateral involvement of a coronary fistula, constitutes an uncommon subgroup of coronary AV fistulas. We herein report on a case of bilateral coronary AV fistula that was coexistent with variant angina originating from the distal right ventricular branch of the right coronary artery and the distal septal branch of the left anterior descending artery, and the latter drained into the right ventricle.
Angina Pectoris, Variant/*etiology/radiography ; Coronary Vessel Anomalies/*complications/*diagnosis ; Female ; Heart Ventricles/*abnormalities ; Humans ; Middle Aged ; Vascular Fistula/complications/*congenital/*diagnosis

Infiltration of sarcoid granuloma in old cutaneous scars is one of the uncommon cutaneous manifestations of sarcoidosis. Here, we report the case of a 47-year-old female who presented with swelling and irritation in 5 old scars. She had acquired these scars 9 years ago in a traffic accident. An incisional scar biopsy revealed noncaseating granulomas consistent with sarcoidosis. High-resolution CT (HRCT) revealed right paratracheal, both hilar, paraaortic, and subcarinal lymphadenopathy without any nodular densities in both lung fields. Successful regression of cutaneous inflammation was achieved using a short course of oral steroids.
Cicatrix/*pathology ; Female ; Humans ; Middle Aged ; Sarcoidosis/*diagnosis/therapy ; Skin Diseases/*diagnosis/therapy

Blue rubber bleb nevus syndrome is a rare disorder that is characterized by multiple recurrent vascular malformations, such as hemangioma, and these primarily involve the skin and the gastrointestinal tract. It may also involve the brain, liver, lungs, and skeletal muscles. A 14-year-old female visited our hospital with a chief complaint of dizziness; upon examination, we found multiple recurrent hemangiomas on the skin and gastrointestinal tract. We were able to diagnose her as suffering from blue rubber bleb nevus syndrome and we treated her with methylprednisolone (2 mg/kg/day for 1 month and 1 mg/kg/day for additional 3 months). We report on this case along with a review of the literature.
Adolescent ; Female ; Gastrointestinal Neoplasms/*diagnosis/therapy ; Hemangioma/*diagnosis/therapy ; Humans ; Nevus, Blue/*diagnosis/therapy ; Skin Neoplasms/*diagnosis/therapy ; Syndrome

BACKGROUND/AIMS: Vascular access dysfunction is an important cause of morbidity and mortality in hemodialysis (HD) patients. Recent studies have shown that a klotho gene mutation is related to endothelial dysfunction, thrombosis, and arteriosclerosis, which are regarded as causes of vascular access dysfunction. We investigated the relationship between the klotho G-395A polymorphism and early dysfunction in vascular access in HD patients. METHODS: Patients who underwent vascular access operations between 1999 and 2002 were enrolled (n=126). Genotyping was performed by allelic discrimination using a 5'-nuclease polymerase chain reaction assay. Clinical data that could be relevant to access dysfunction were obtained from medical records. Early dysfunction of vascular access was defined as the need for any angioplastic or surgical intervention to correct or replace a poorly or nonfunctioning vascular access within 1 year and at least 8 weeks after initial access placement. RESULTS: Of the 126 patients, the genotype frequency of G-395A was 72.2% for GG (n=91), 24.6% for GA (n=31), and 3.2% for AA (n=4), and the frequency of minor allele was 0.155. Clinical data were similar between the two groups, divided according to the status of the A allele. Early dysfunction occurred in 34 (27.0%) of patients, but it occurred at a significantly higher rate in A allele carriers (45.7%, 16/35) than in noncarriers (19.8%, 18/91; p=0.003). CONCLUSIONS: Our results suggest that the klotho G-395A polymorphism could be a risk factor for early dysfunction of vascular access in HD patients.
Aged ; *Arteriovenous Shunt, Surgical ; Catheters, Indwelling ; Cohort Studies ; Female ; Glucuronidase/*genetics ; Humans ; Kidney Failure, Chronic/complications/*genetics/therapy ; Male ; Middle Aged ; Polymorphism, Genetic/*genetics ; Promoter Regions, Genetic/genetics ; *Renal Dialysis ; Vascular Diseases/complications/genetics ; Vascular Patency/*genetics

BACKGROUND/AIMS: Brain natriuretic peptide (BNP) levels are known to be elevated in patients with chronic kidney disease (CKD) and normal heart function. Therefore, the present study was performed to examine the effectiveness of BNP level in diagnosing heart failure in patients with CKD and to determine its effects on survival rate and prognosis. METHODS: A total of 182 patients with CKD who visited the hospital due to dyspnea of NYHA class II were included in the study. BNP levels were measured and echocardiography was performed to divide the subjects into groups with and without heart failure. Their BNP levels, clinical courses, and survival rates were analyzed retrospectively. RESULTS: When BNP level was > or =858.5 pg/mL in CKD patients, heart failure could be diagnosed with sensitivity and specificity of 77% and 72%, respectively. Survival rate of the group with BNP levels of > or =858.8 pg/mL was significantly lower than that of the group with BNP level below this threshold (p=0.012) and multivariate analysis showed that BNP level, age, and sex affected survival rate in the group with BNP level > or =858.8 pg/mL. CONCLUSIONS: BNP levels of patients with CKD showed a positive correlation with creatinine levels, and the critical point of BNP level for diagnosis of heart failure was 858.5 pg/mL. As the survival rate in patients with BNP level above the critical point was significantly low, this level was a useful indicator for predicting their prognosis. Care should be taken in interpreting BNP level because patients with stage 5 CKD may show a high concentration of BNP without heart failure.
Aged ; Cohort Studies ; Female ; Heart Failure/blood/complications/*diagnosis ; Humans ; Kidney Diseases/*blood/complications/mortality ; Male ; Middle Aged ; Natriuretic Peptide, Brain/*blood ; Predictive Value of Tests ; Retrospective Studies ; Survival Rate

BACKGROUND/AIMS: Diffuse large B-cell lymphoma (DLBCL) in Koreans is frequently accompanied by extranodal (EN) disease at the time of autologous stem cell transplantation (ASCT). We sought to determine whether high EN involvement affected survival following ASCT in Koreans. METHODS: We reviewed 27 patients who had DLBCL with residual disease at ASCT: 13 with residual disease at nodal site(s) only and 14 with nodal and EN disease. RESULTS: Univariate analysis showed that disease status, lactate dehydrogenase (LDH), and performance status at ASCT were predictors of survival following ASCT. The number of EN sites, as categorized by the International Prognostic Index system, had no prognostic significance. When EN involvement at ASCT was classified as negative or positive, the 2-year overall survival for the negative group was 64%, significantly better than the 14% for the positive group (p=0.021), and the event-free survival for the negative group was 62%, significantly better than the 14% for the positive group (p=0.02). CONCLUSIONS: Patients who had DLBCL with residual EN involvement at ASCT showed worse outcomes following ASCT compared to those without EN disease.
Adolescent ; Adult ; Aged ; Cohort Studies ; Female ; Humans ; Korea ; Lymphoma, Large B-Cell, Diffuse/mortality/*pathology/*therapy ; Male ; Middle Aged ; Neoplasm, Residual ; Retrospective Studies ; *Stem Cell Transplantation ; Survival Rate ; Treatment Outcome ; Young Adult

BACKGROUND/AIMS: The androgen dehydroepiandrosterone (DHEA) attenuates allergic inflammatory airway reactions by down-regulating the Th2 response in mice. The purpose of this study was to investigate whether DHEA suppresses Th2 cytokine production in cultured peripheral blood mononuclear cells (PBMCs) from asthmatic patients. METHODS: Sixty-one consecutive suspected asthmatic or non-asthmatic men underwent tests for asthma. PBMCs from each subject were cultured with and without DHEA (0.01~10 micrometer) for 48 h. The concentrations of interferon (IFN)-gamma, interleukin (IL)-5, and IL-10 in the culture supernatant were measured via an enzyme-linked immunosorbent assay. RESULTS: In PBMCs from subjects exhibiting methacholine airway hyperresponsiveness (AHR), DHEA significantly suppressed IL-10, IL-5, and IFN-gamma production in a dose-dependent manner (all p<0.001) and tended to increase the IFN-gamma/IL-5 ratio (p=0.087). DHEA (10 micrometer) suppressed cytokine production to a greater degree in subjects with AHR compared with those without AHR (IL-5: 24.0+/-7.8% vs. 40.9+/-3.6%, p<0.01; IFN-gamma: 29.7+/-7.0% vs. 54.5+/-5.1%, p<0.01). Cytokine suppression was significantly related to AHR, serum total IgE levels, and skin reactivity to house dust mites. CONCLUSIONS: DHEA suppressed both Th1 and Th2 responses, with a Th1 bias, and the degree of suppression was associated with the severity of AHR or atopy. Therefore, DHEA may be a useful therapy for asthma.
Adjuvants, Immunologic/*pharmacology ; Adolescent ; Adult ; Asthma/*pathology ; Cell Culture Techniques ; Cytokines/*metabolism ; Dehydroepiandrosterone/*pharmacology ; Humans ; Male ; Th2 Cells/*drug effects/*metabolism ; Young Adult

No abstract available.
Aged, 80 and over ; Biopsy ; Carcinoma, Papillary/chemistry/*secondary ; Female ; Humans ; Immunohistochemistry ; Liver Neoplasms/chemistry/*secondary ; Pancreatic Neoplasms/chemistry/*pathology ; Tumor Markers, Biological/analysis

No abstract available.
Diagnosis, Differential ; Endosonography ; Female ; Humans ; Incidental Findings ; *Intestinal Mucosa/pathology/radiography/surgery ; Intestinal Polyps/*diagnosis/pathology/radiography/surgery ; Middle Aged ; Neoplasms, Fibroepithelial/*diagnosis/pathology/radiography/surgery ; Predictive Value of Tests ; Rectal Neoplasms/*diagnosis/pathology/radiography/surgery ; Tomography, X-Ray Computed