Klinefelter' syndrome is a disorder of sexual differentiation in males, characterized by the presence of two or more X-chromosomes, hypogonadism, and lack of secondary sexual characteristics. The association between Klinefelter' syndrome and glomerulonephritis has been reported, while cases of glomerulonephritis associated with Klinefelter' syndrome are rare. We report the Korean case: a 31-year-old man with Klinefelter' syndrome who developed glomerulonephritis. The patient's urine analysis shows microscopic hematuria and the result of kidney biopsy was minimal change disease. The onset and course of his disease might have been influenced by the sex hormone imbalance.
Biopsy ; Glomerulonephritis ; Hematuria ; Humans ; Hypogonadism ; Kidney ; Male ; Nephrosis, Lipoid ; Sex Differentiation

Peritonitis is a common and potentially serious infection in patients undergoing continuous ambulatory peritoneal dialysis (CAPD). The most common organisms usually associated with CAPD peritonitis are Staphylococcus aureus and Staphylococcus epidermidis. Rarely, aerobic gram negative bacilli have been the causative agents of CAPD peritonitis. The treatment of CAPD peritonitis requires removal of the peritoneal catheter and treatment with parenteral antibiotics active against the causative pathogen. While hospitalized for CAPD peritonitis, a 55-year-old man on CAPD had nosocomial peritonitis secondary to infection by ESBL-producing E.coli, that was sensitive to imipenem and meropenem. He was treated successfully with a 4-week course of intraperitoneal meropenem therapy without subsequent relapse, loss of peritoneal catheter, ultrafiltration failure, or dialysis inadequacy.
Anti-Bacterial Agents ; Catheters ; Dialysis ; Escherichia coli ; Humans ; Imipenem ; Peritoneal Dialysis ; Peritoneal Dialysis, Continuous Ambulatory ; Peritonitis ; Recurrence ; Staphylococcus aureus ; Staphylococcus epidermidis ; Thienamycins ; Ultrafiltration

BACKGROUND: Detection of antibodies to extractable nuclear antigens (ENAs) and dsDNA is needed for the diagnosis of and predicting prognosis in systemic autoimmune diseases. Recently introduced line immunoassay (LIA) has the advantage of detecting several autoantibodies simultaneously, and we evaluated its usefulness in the diagnosis of autoimmune diseases in comparison with enzyme-linked immunosorbent assay (ELISA). METHODS: Samples were collected from 437 patients referred by rheumatologists. FANA (fluorescent antinuclear antibody) test and LIA for the detection of 13 different autoantibodies, including 6 ENAs and dsDNA were performed. LIA-positive samples for ENA or dsDNA antibodies were further tested with ELISA. Final diagnosis was made by rheumatologists according to the diagnostic criteria. Agreement of results between LIA and ELISA was analyzed in 53 selected patients with systemic autoimmune diseases. RESULTS: The LIA detected antibodies to ENA and dsDNA in 118 and 22 patients, respectively, and ELISA detected 70.3% (83/118) and 45.5% (10/22) of LIA positive samples. Especially, 60.2% (71/118) of patients with positive ENA antibody on LIA was diagnosed as systemic autoimmune diseases. Patients having strong FANA titer and homogenous/speckled pattern showed higher prevalence of autoantibodies, but a small proportion of FANA negative patients also showed positive reactivity (LIA 10.8%, ELISA 5.2%). LIA showed a good agreement with ELISA for the anti-ENA antibodies (> or =80%), and a lower agreement for the anti-dsDNA antibody (67.9%). CONCLUSIONS: LIA detecting several autoantibodies simultaneously might replace ELISA for anti-ENA antibodies, but not for anti-dsDNA antibodies. When LIA is performed considering clinical manifestations and FANA, it could contribute to the diagnosis of systemic autoimmune disease.
Antibodies, Antinuclear/*analysis ; Antigens, Nuclear/immunology ; Autoimmune Diseases/*diagnosis ; DNA/*immunology ; Enzyme-Linked Immunosorbent Assay ; Female ; Fluorescent Antibody Technique, Indirect ; Humans ; *Immunoassay ; Male ; Middle Aged ; Reagent Kits, Diagnostic ; Reproducibility of Results

OBJECTIVES: To introduce a new injection material for vocal fold diseases, which could be readily translated to clinical practice, we investigated the effectiveness of platelet-rich plasma (PRP) injection on the injured vocal fold in terms of histological recovery. METHODS: Blood samples were drawn from New Zealand White rabbits and PRP was isolated through centrifugation and separation of the samples. Using a CO2 laser, we made a linear wound in the 24 vocal fold sides of 12 rabbits and injected each wound with PRP on one vocal fold side and normal saline (NS) on the other. Morphologic analyses were conducted at 2, 4, and 12 weeks after injection, and inflammatory response, collagen deposit, and changes in growth factors were assessed using H&E and masson trichrome (MT) staining and western blot assay. RESULTS: PRP was prepared in approximately 40 minutes. The mean platelet concentration was 1,315,000 platelets/mm3. In morphological analyses, decreased granulation was observed in the PRP-injected vocal folds (P<0.05). However, the irregular surface and atrophic change were not difference. Histological findings revealed significant inflammation and collagen deposition in NS-injected vocal folds, whereas the PRP-injected vocal folds exhibited less (P<0.05). However, the inflammatory reaction and fibrosis were not difference. In western blot assay, increased amounts of growth factors were observed in PRP-injected vocal folds. CONCLUSION: Injection of injured rabbit vocal folds with PRP led to improved wound healing and fewer signs of scarring as demonstrated by decreased inflammation and collagen deposition. The increased vocal fold regeneration may be due to the growth factors associated with PRP.
Blood Platelets ; Blotting, Western ; Centrifugation ; Cicatrix ; Collagen ; Fibrosis ; Inflammation ; Intercellular Signaling Peptides and Proteins ; Lasers, Gas ; Platelet-Rich Plasma* ; Rabbits ; Regeneration ; Vocal Cords* ; Wound Healing* ; Wounds and Injuries*

Hypercalcemia is a common complication in CAPD patients treated with calcium-containing phosphate binders and using the standard dialysate(Ca++ : 3.5 mEq/L). Furthermore, the high calcium concentration in standard dialysate may have a suppressive effect on parathyroid hormone(iPTH) level, contributing to the high prevalence of low-urnover bone disease. We studied the effect of low calcium dialysate(Ca++ : 2.5 mEq/L) for those patients with high risk of low- turnover bone disease. Among 386 patients(1996. 1.- 1999. 12.) who had been stable on CAPD for at least 3 months, 46 patients were included in this study. The patients were divided into 3 groups on the basis of the iPTH levels(<150 pg/mL) and/or corrected serum calcium levels(>10 mg/dL) before the conversion to low calcium dialysate. Group 1(n=29), iPTH <150 pg/mL and Ca++>10 mg/dL; Group 2 (n=14), iPTH <150 pg/mL and Ca++<10 mg/dL; Group 3(n=3), iPTH >150 pg/mL and Ca++ >10 mg/ dL. During a 2-month run-in period, those patients were treated with standard dialysate. After that, a 12-month therapy with low calcium dialysate was followed. Biochemical data including calcium, phosphorus, iPTH and alkaline phosphatase were measured regularly and daily phosphate binder and calcitriol intake(pill counting) were assessed during the run-in and therapy period. We obtained the following result: the prevalence of hypercalcemia(Ca++>10.5 mg/dL) was 5.7%(22/ 386 patients). Serum calcium levels decreased during the therapy period(12 months)(10.5+/-1.4 vs 9.4+/-1.3 mg/dL, p<0.05). Serum phosphorus levels remained unchanged. Mean serum alkaline phosphatase level increased(203.0+/-92.9 vs 257.2+/-103.4 U/L, p<0.05). Serum iPTH levels increased (92.7+/-128.8 vs 225.3+/-237.3 pg/mL,p<0.05). The mean intake of oral phosphate binders was not significantly different between run-in period and therapy period. But calcitriol doses increased 0.038+/-0.087 at run-in period to 0.158+/-0.288 tablets/person/day at therapy period(p<0.05). In the six patients, low calcium dialysate was converted to standard dialysate due to high iPTH level (n=3), symptomatic hypo calcemia(n=2), and uncontrolled edema(n=1). In conclusion, in the study of 46 patients over 12 month period, the usage of 2.5 mEq/L calcium dialysate resulted in a significant decrement in calcium levels and increased iPTH levels. Therefore, we propose that dialysis with a low calcium dialysate is an acceptable form of therapy for the patients with high risk of low-turnover bone disease showing hypercalcemia and low iPTH level. However, further study will be needed for evaluating the effect of low calcium dialysate in low-turnover bone disease.
Alkaline Phosphatase ; Bone Diseases* ; Calcitriol ; Calcium* ; Dialysis ; Humans ; Hypercalcemia ; Peritoneal Dialysis, Continuous Ambulatory* ; Phosphorus ; Prevalence

OBJECTIVES: Nasal septal perforation is an anatomic defect of the cartilaginous and bone tissues of the nasal septum. Many approaches and techniques to repair nasal septal perforations have been reported on. The purpose of this paper is to report on our surgical technique and the results of the treatment for nasal septal perforations. METHODS: From May 2001 to March 2008, 14 patients (12 males and 2 females; mean age: 41.3 yr) were enrolled. The mean perforation size was 15 mm, and all the perforations were located at the cartilaginous portion. Our surgical technique is based on an endoscope-assisted endonasal approach, with dissection of unilateral advanced mucosal flaps with using a temporalis fascia graft. The follow-up periods ranged from 3 to 23 months (mean follow-up period: 8 months). RESULTS: Using our surgical technique on 14 patients, 12 cases (85.7%) of septal perforation were closed without complication. The remaining two patients (14.3%) had incomplete closures (about 2-3 mm) without any significant symptoms related to the remaining perforation. CONCLUSION: Our technique is a viable procedure with a high success rate for achieving closure of nasal septal perforations. It has the advantages of shortening the operative time, no external incision and avoiding any other perforation during the operation. Therefore, we consider it to be a good alternative for repairing nasal septal perforations.
Bone and Bones ; Fascia ; Follow-Up Studies ; Humans ; Male ; Nasal Septal Perforation ; Nasal Septum ; Operative Time ; Transplants

BACKGROUND/AIMS: Cholestatic jaundice caused by a juxtapapillary diverticulum can be treated by excision of the diverticula or endoscopic sphincterotomy(EST). The aim of this study is to evaluate the effectiveness of EST for cholestatic jaundice in patient with juxtapapillary diverticulum with absence of common bile duct stones. METHODS: We recruited patients who underwent Endoscopic Retrograde Cholangio Pancreatography(ERCP) between September 2000 and May 2005. The presence of Juxtapapillary diverticula was seen in 92(29.3%)) out of 315 patients underwent ERCP. Cholestatic jaundice associated with juxtapapillary diverticula was considered when patients have jaundice, elevated serum bilirubin and alkaline phosphatase more than normal, and there are no other obstructive lesion on abdominal ultrasonography or computed tomography(CT) scan. The number of the patients were 13 who had cholestatic jaundice associated with juxtapapillary diverticula without common bile duct stones. All 13 patients underwent EST and had no complications. The patients were followed up to visit outpatient department or interviewed on the telephone. RESULTS: Of the 13 patients, 10 were male and 3 were female. The mean age was 70.4 years and mean duration of follow-up were 28.9 months. All 13 patients who had gallbladder presented RUQ discomfort and jaundice. Seven patients presented gallstone pancreatitis with jaundice. Nine patients had GB stone, 1 patients had GB sludge. In the follow-up period, from 1month to 46 months after EST, 1 patient died of unknown cause within 1 year. One patient had recurred gall stone pancreatitis with jaundice 12 months later. CONCLUSIONS: EST can be considered as a useful therapeutic modality especially in eldery patients with cholestatic jaundice and gallstone pancreatitis associated with juxtapapillary diverticulum.
Alkaline Phosphatase ; Bilirubin ; Cholangiopancreatography, Endoscopic Retrograde ; Common Bile Duct* ; Diverticulum* ; Female ; Follow-Up Studies ; Gallbladder ; Gallstones ; Humans ; Jaundice ; Jaundice, Obstructive* ; Male ; Outpatients ; Pancreatitis ; Sewage ; Telephone ; Ultrasonography

BACKGROUND: Lipoprotein (a) concentration is mainly determined by apo (a) genotype, but elevated in the atherosclerotic vascular disease more than in normal group with the same apo (a) phenotype. It has been known that Lp (a) has independent metabolism in contrast with other lipoproteins and that the use of cholesterol lowering agent such as HMG-CoA reductase inhibitor for 6 months does not change the level of Lp (a). The results of several studies suggests that Lp (a) may be related to inflammation of atherosclerotic plaque and therefore, long term use of cholesterol lowering agents make plaque stable by reduction of inflammation at plaque. We hypothesized that there is a relationship between long term use of HMG-CoA reductase inhibitor and change of Lp (a) level. We prospectively measured Lp (a), lipids and inflammatory markers before and after long term use of HMG-CoA reductase inhibitor to examine our hypothesis. METHODS: Forty-nine subjects (M:F=28:21, age=59.1+/-12.0) with hyperlipidemia were administered HMG-CoA reductase inhibitor for 15 months (minimum 6 months, maximun 44 months), and Lp (a), lipids and inflammatory markers were measured before and after use of the HMG-CoA reductase inhibitor. In control group (ninty-nine subjects, M:F=60:39, age=61.2+/-9.2), these parameters were measured more than 6 months. RESULTS: In the hyperlipidemia group who were given HMG-CoA reductase inhibitor, baseline levels of total cholesterol, TG, LDL were significantly elevated more than those of the control group, but Lp (a) and inflammatory markers were not significantly different. After use of HMG-CoA reductase inhibitor, the level of Lp (a) was reduced significantly (before 28.9+/-29.3 mg/dl, after 20.0+/-19.0 mg/dl, p=0.009), but not significantly in the control group. There was a minimal relation between baseline Lp (a) levels and percent changes of Lp (a) levels. Total cholesterol and LDL levels reduced significantly after use of the drug, but inflammatory markers did not. CONCLUSION: These data showed that Lp (a) level in the hyperlipidemia group after the long term use of HMG-CoA reductase inhibitor decreased significantly. We suggest that these changes of Lp (a) level may be one of reliable markers for plaque stability in atherosclerotic vascular disease.
Atherosclerosis ; Cholesterol ; Genotype ; Hyperlipidemias ; Inflammation ; Lipoprotein(a) ; Lipoproteins ; Metabolism ; Oxidoreductases* ; Phenotype ; Plaque, Atherosclerotic ; Prospective Studies ; Vascular Diseases

Burkitt's lymphoma is a distinct clinical entity classified as an undifferentiated lymphoma of B-lymphocytic origin. Burkitt's lymphoma cell leukemia occurs when Burkitt's lymphoma cells represent more than 20% of marrow cells and exceed about 5000/ul in blood. We report a case of Burkitt's lymphoma cell leukemia in primary gastric lymphoma in 55-year-old man with 5kg of weight loss for 2 months and epigastric pain for 3 days. Primary gastric lymphoma was diagnosed by biopsies with gastrofiberoscopy and upper abdominal CT scan in this patient. During admission, peripheral blood smear revealed sudden increase in abnormal blasts with multiple vacuoles. Burkitt's lymphoma cell leukemia was diagnosed by peripheral blood smear, bone marrow aspiration and biopsy, immunophenotyping and cytogenetic analysis in this patient. For its great rarity, we report this case with review of literature.
Biopsy ; Bone Marrow ; Burkitt Lymphoma* ; Cytogenetic Analysis ; Humans ; Immunophenotyping ; Leukemia* ; Lymphoma* ; Lymphoma, Non-Hodgkin ; Middle Aged ; Tomography, X-Ray Computed ; Vacuoles ; Weight Loss

Cases with extranodal Hodgkin's disease located in sites outside those defined as lymphatic structure are quite rare. The exact incidence of this localized form of Hodgkin's disease is unknown, but it is estimated to be only 0.25% for overall incidence of Hodgkin's disease. Localized primary extranodal Hodgkin's disease seems to have an excellent prognosis in some cases including skin, tongue, lung, stomach, small bowel, and thyroid. We report a case of primary gastrointestinal Hodgkin's disease involved in the stomach and terminal ileum in 48-year-old man with 7 kg of weight loss for 3 months, fever with chills for 1 week, and intermittent epigastric pain for 1 year. The histologic type of this case was lymphocyte predominance, which was diagnosed by biopsy of the stomach and terminal ileum with immnophenotyping study. And the stage of this case was IIE. We tried combination chemotherapy with adriamycin, bleomycin, vinblastin, and dacabazine with excellent response. For its rarity, we report this case with review of literatures.
Biopsy ; Bleomycin ; Chills ; Doxorubicin ; Drug Therapy, Combination ; Fever ; Hodgkin Disease* ; Humans ; Ileum* ; Incidence ; Lung ; Lymphocytes ; Lymphoma ; Middle Aged ; Prognosis ; Skin ; Stomach* ; Thyroid Gland ; Tongue ; Weight Loss