Autosomal dominant polycystic kidney disease is a systemic disorder with cystic manifestations in the kidneys, liver, pancreas, seminal vesicles, and meninges; its noncystic manifestations affect mostly the vascular, cardiac, and connective tissues. Cardiovascular abnormalities, including mitral and aortic valvular prolapse and regurgitation and annuloaortic ectasia, have been considered important extrarenal manifestations of autosomal dominant polycystic kidney disease. But there were no reports with dilated cardiomyopathy in patients with polycystic kidney disease yet. We have experienced a case of dilated cardiomyopathy that is associated by autosomal dominant polycystic kidney disease in 40 year old male patient. Abdominal ultrasonography revealed variable sizes of multiple cysts in both kidneys and echocardiography showed the marked dilatation of left ventricle and severely depressed left ventricular systolic function (ejection fraction=19%). He was treated with diuretics intravenously and orally. Then dyspnea and abdominal distension was improved. This is the first case of dilated cardiomyopathy with autosomal dominant polycystic disease in Korea.
Adult ; Cardiomyopathy, Dilated* ; Cardiovascular Abnormalities ; Connective Tissue ; Dilatation ; Dilatation, Pathologic ; Diuretics ; Dyspnea ; Echocardiography ; Heart Ventricles ; Humans ; Kidney ; Korea ; Liver ; Male ; Meninges ; Pancreas ; Polycystic Kidney Diseases ; Polycystic Kidney, Autosomal Dominant* ; Prolapse ; Seminal Vesicles ; Ultrasonography

Cytomegalovirus (CMV) is a ubiquitous virus and its infections occur commonly after renal transplantation and immunosuppressive therapy. Early and accurate laboratory diagnosis of CMV infection in renal transplant is necessary but often difficult. To find optimal diagnostic methods for CMV infection, we compared shell vial culture and polymerase chain reaction (PCR) and Southern blot of PCR products. A total of 301 specimens of urine, blood neutrophils, tissues, or body fluids were obtained from 75 renal transplant recipients and were submitted to shell vial culture for CMV as well as DNA PCR using primers for immediate early(IE) gene of CMV. The human fibroblast cell line (MRC-5) was used to culture CMV and were examined with immunofluorescence staining using monoclonal antibody to the early antigen of CMV. The PCR products (274 and 379 bp) were detected by gel electrophoresis and ethidium bromide staining. When PCR products were not clearly visible on electrophoresis, PCR products were analyzed by Southern blot using IE gene probe. Sixty four(85.3%) of 75 renal transplant recipients showed CMV infection as analyzed by PCR and Southern blot as well as shell vial culture. On shell vial culture, CMV were detected in 81 specimens from 30(40%) renal transplant recipients in viremic state. On PCR and Southern blot analysis CMV were detected in 55 and 26 specimens, respectively from 59 patients. The sensitivity of culture and PCR to detect CMV infection were 42.4% and 83.3%, respectively. The results of two studies were concordant in 48%. PCR and Southern blot did not detect CMV in 10 and 5 culture proven CMV positive samples, respectively. Mutant CMV were found in 3 patients which showed 5-10 bp deletion in IE gene. Moreover, DNA sequencing analysis showed 5 mutant strains among 11 strains which appeared same by PCR prodcut. These results suggest that PCR followed by Southern blot may be more sensitive, but less specific than shell vial culture in the diagnosis of CMV disease. PCR followed by Southern blot may not detect mutant CMV. Combined analysis using both shell vial culture and PCR followed by Southern blot may be necessary to diagnose CMV infection in renal transplant recipients.
Blotting, Southern ; Body Fluids ; Cell Line ; Clinical Laboratory Techniques ; Cytomegalovirus Infections* ; Cytomegalovirus* ; Diagnosis ; DNA* ; Electrophoresis ; Ethidium ; Fibroblasts ; Fluorescent Antibody Technique ; Humans ; Kidney Transplantation ; Neutrophils ; Polymerase Chain Reaction ; Sequence Analysis, DNA ; Transplantation*

No abstract available.
Histamine* ; Pruritus*

No abstract available.
Bacteremia* ; Humans ; Renal Dialysis*

No abstract available.

No abstract available.

No abstract available.
Peritoneal Dialysis, Continuous Ambulatory* ; Peritonitis*

No abstract available.
Catheterization* ; Catheters* ; Edema* ; Subclavian Vein* ; Upper Extremity*

As craniopharyngioma is histologically benign, recent trend of managing this tumor has been complete surgical removal without adding any adjuvant therapy. But because of its close relation with surrounding vital structures, total removal sometimes results in unacceptable neurologic sequelae. To avoid these serious complications various management options have been suggested. Among these, bleomycin injections into the cystic cavity have been sporadically reported with satisfactory results. The authors report a 50-year-old woman presented with visual symptoms, who was found to have a largely cystic craniopharyngioma. Because the boarder between the tumor and hypothalamus was ill defined, intracystic bleomycin injection followed by delayed surgery was scheduled. A total of 80mg bleomycin was given over the 8 days. After the treatment high fever, skin rash and mental change developed but these symptoms were gradually subsided and the cysts were shrunken with surrounding infarction. During the follow-up period, visual symptoms became rapidly worse for which surgery was undertaken. Optic nerve was severely compressed by the underlying solid tumor and overlying A1 portion of the anterior cerebral artery. The tumor was near totally removed without any vascular insult. After the operation, the patient remained drowsy and lapsed into coma 6 days later and died. CT scan just before her death showed an infarct in the right ACA and MCA territories suggesting ICA occlusion. The cause of ICA occlusion remained to be unsolved.
Anterior Cerebral Artery ; Bleomycin* ; Coma ; Craniopharyngioma* ; Exanthema ; Female ; Fever ; Follow-Up Studies ; Humans ; Hypothalamus ; Infarction ; Middle Aged ; Optic Nerve ; Tomography, X-Ray Computed

BACKGROUND: An initiating time of renal replacement therapy (RRT) for patients with end-stage renal disease (ESRD) has great influence on prognosis of the patients, however, there are currently no accurate guidelines for initiation of renal replacement therapy. Traditionally, nephrologists usually initiate RRT on the basis of observation of uremic symptoms and changes of laboratory parameters, such as serum creatinine concentration and/or glomerular filtration rate (GFR). DOQI guidelines suggest weekly Kt/Vurea < 2.0 or nPNA < 0.8 g/kg/day for an objective index of the initiation of dialysis. Then, we designed to formulate KP index {(weekly Kt/Vurea + 2.5 X nPNA) X 1/2} using two indices above as a clinically useful objective index to determine the initiation of RRT for patients with ESRD. METHODS: Patients with ESRD having the weekly Kt/Vurea index below 3.0 were selected from 186 patients who came to renal unit of Soonchunhyang Bucheon hospital. The patients having the weekly Kt/Vurea index between 1.0 and 2.0 were classified into two groups, KP index > 2.0 and KP index < 2.0. The groups were compared and analyzed via renal function, biochemical index and the number of patients starting a first RRT. Further, the correlations between KP index and other indices were analyzed over all patients. Then, the numbers per group of patients starting RRT were compared after all patients were divided into two groups, one of which is between 1.5 and 2.0 and the other is between 2.0 and 2.5 by weekly Kt/Vurea index and KP index. RESULTS: The group of KP index < 2.0 indicated significantly lower indices in weekly Kt/Vurea, nPNA (B2) and % LBM (%) than those of the group of KP index > 2.0, while no significant differences between the groups were in serum albumin concentration, serum creatinine concentration, FFEFBM and RRF. And the frequency of patients starting RRT was significantly higher in the group of KP index < 2.0 rather than the group of KP index > 2.0 in statistics. There was a significant correlation between KP index and other indices in all patients. In comparing and analyzing the number of patients starting RRT, weekly Kt/Vurea index did not demonstrate significant differences between two groups of 1.5 < weekly Kt/Vurea < 2.0 and 2.0 < weekly Kt/Vurea < 2.5, but the frequency of patients in the group of 1.5 < KP index < 2.0 was significantly higher than that in the group of 2.0 < KP index < 2.5. CONCLUSION: It is considered that KP index is an index clinically useful for ESRD patients to determine an appropriate timing for the initiation of RRT, and that the timing should be fixed with regard for other various indices and clinical features for advisable prognosis of the patients. In addition, accurate guideline to determine such an appropriate time needs to be suggested by further new studies.
Creatinine ; Dialysis* ; Glomerular Filtration Rate ; Gyeonggi-do ; Humans ; Kidney Failure, Chronic* ; Prognosis ; Renal Replacement Therapy ; Serum Albumin