Diarrhea-associated hemolytic uremic syndrome(D+ HUS) is induced by enterohemorrhagic Escherichia coli(EHEC) and is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. The disease is usually transmitted by meat and water contaminated by excreta of domestic animals. We report a son and his mother with diarrhea-associated hemolytic uremic syndrome that spread within the family.
Acute Kidney Injury ; Anemia, Hemolytic ; Animals, Domestic ; Enterohemorrhagic Escherichia coli ; Escherichia ; Hemolytic-Uremic Syndrome* ; Humans ; Meat ; Mothers ; Thrombocytopenia

PURPOSE: There is increasing consideration about the preemptive transplantation, transplantation without any preceding dialysis, as a one of options of a renal replacement therapy (RRT). This study evaluates a beneficial effect on recipient and allograft survival of preemptive transplantation and compares the outcome to that according to the dialysis modality and duration. METHODS: All patient who had received a kidney transplant from a living donor in the Seoul National University Hospital (SNUH) between January 1990 and October 2004 are included in this retrospective study. Patients were subdivided into three groups; preemptive transplant group (group 1, n=47), hemodialysis group (group 2, n=307) and peritoneal dialysis group (group 3, n=52). RESULTS: The characteristics of each groups were not statistically different except recipient age. Ten-year patient survival was 97.8% in PE group, but is not significantly higher than in HD (89.4%) and in PD (90.7%) groups. However, 10-year graft survival was higher in PE group than in HD group (p<0.05; 100%, 74.7% respectively). The differential effect of pretransplant dialysis duration on graft survival was prominent if the patients had been on the pretransplant dialysis for more than 42 months (p<0.05; 10-year graft survival; PE, 100% and dialysis more than 42 months, 77.9% respectively) Compared with HD group as a pre-transplant dialysis modality, PD group showed better patient and graft survival rate, but not statistically significant. CONCLUSION: Depending on the above results, we may suggest PE or PD being a superior pre-transplant modality than HD. And we should be considerate of choosing treatment modality and duration before transplantation.
Allografts ; Dialysis* ; Graft Survival* ; Humans ; Kidney Transplantation* ; Kidney* ; Living Donors* ; Peritoneal Dialysis ; Renal Dialysis ; Renal Replacement Therapy ; Retrospective Studies ; Seoul ; Transplants*

BACKGROUND: Darbepoetin alfa is a new erythropoietic agent with a three fold longer terminal half-life than recombinant human erythropoietin (r- HuEPO). The aim of this randomized, open-label study is to determine whether darbepoetin alfa is as effective as r-HuEPO for the treatment of anemia in hemodialysis patients when administered at a reduced dosing frequency. METHODS: A total 74 Korean hemodialysis patients receiving r-HuEPO therapy by either the intravenous (IV) or subcutaneous (SC) route were randomized to continue r-HuEPO or to receive an equivalent dose of darbepoetin alfa at a reduced dosing frequency. Patients receiving r-HuEPO once weekly changed to once every other week darbepoetin alfa, and those receiving r-HuEPO two or three times weekly changed to once-weekly darbepoetin alfa. The initial dose of darbepoetin alfa was based on the r-HuEPO dose at the time of entry into the study, using a formula equating the peptide mass of the two molecules (200 IU r-HuEPO=1 microgram darbepoetin alfa). The doses of r-HuEPO and darbepoetin alfa were titrated to maintain hemoglobin concentrations within -1.0 to +1.5 g/dL of patients' baseline values and within a range of 8.0 to 13.0 g/ dL for up to 20 weeks (16-week dose-titration period followed by a 4-week evaluation period). The primary end point was change in hemoglobin level between baseline and the evaluation period. RESULTS: The mean change in hemoglobin from baseline to the evaluation period was similar in the darbepoetin alfa (-0.03+/-0.19 g/dL) and r-HuEPO (0.27+/-0.20 g/dL) groups, and the difference between the two treatments was -0.30 g/dL (95% CI, -0.84 to 0.23). This was not a statistically significant or clinically relevant difference, despite the reduced frequency of darbepoetin alfa administration. The safety profiles of darbepoetin alfa and r-HuEPO were similar. CONCLUSION: This study suggests that darbepoetin alfa maintains hemoglobin as effectively as r- HuEPO, but with reduced dose frequency.
Anemia* ; Erythropoietin ; Half-Life ; Hemoglobin A ; Humans ; Renal Dialysis* ; Darbepoetin alfa

BACKGROUND: Diagnosis of RTA (renal tubular acidosis) is not easy due to its nonspecific and various manifestations. To find out the clues to diagnosis, we investigated initial manifestations, laboratory features and clinical course of RTA patients. METHODS: Thirty-seven patients with RTA type I or II, whose follow-up period was over 6 months were included in the study. We reviewed their medical records retrospectively. RESULTS: Male to female ratio was 5:32 and the average age at the time of diagnosis was 38.7 (15~60). Twenty-five patients had RTA type I, nine had type II, and three had both. The average follow-up period was 6.4 years. Initial manifestations were asthenia (54%), nausea (46%), urinary stone (24%), paresthesia (24%), lower extremity weakness (22%), and paralysis (11%). Underlying diseases at the time of diagnosis include Sjogren's syndrome (14%), SLE (8%), drug-induced nephropathy (11%), diabetic nephropathy (5.4%), Sjogren's syndrome combined with SLE (2.7%), and medullary sponge kidney (2.7%). Laboratory tests revealed acidosis with hypokalemia (59%), acidosis without hypokalemia (14%), and hypokalemia without acidosis (24%). The level of total CO2 was 22 mmol/L or lower in 27 patients. The Na:Cl ratio on the average was 1:1.26 and for 33 patients below 1:1.35. Renal function deteriorated in 8 patients and 7 of them had underlying diseases. Urinary stone developed in 2 patients with RTA type I. CONCLUSION: When patients with nonspecific symptoms show decreased levels of serum total CO2, potassium, or Na:Cl ratio, RTA should always be considered.
Acidosis ; Acidosis, Renal Tubular* ; Asthenia ; Diabetic Nephropathies ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Hypokalemia ; Lower Extremity ; Male ; Medical Records ; Medullary Sponge Kidney ; Nausea ; Paralysis ; Paresthesia ; Potassium ; Retrospective Studies ; Sjogren's Syndrome ; Urinary Calculi

BACKGROUND: The proinflammatory cytokine, interleukin-6(IL-6), seems to be involved in the pathogenesis and progression of IgA nephropathy. The aim of this study is to elucidate the relationship between the intrarenal expression of IL-6 and the clinicopathological findings in IgA nephropathy, and to reveal whether allele-frequency differences of -174 G/C polymorphism of IL-6 gene promoter region exist between IgA nephropathy patients and the normal population. METHODS: The total RNA was extracted from renal tissue of 56 IgA nephropathy patients. Semiquantitative reverse-transcriptase polymerase chain reactions(RT-PCR) using the internal competitors were done for the quantification of IL-6 transcripts. Using PCR-RFLP, we examined the -174 G/C polymorphism in IgA nephropathy patients and in 53 of the normal Korean population. RESULTS: In RT-PCR, the degree of intrarenal IL-6 expression was not related with any clinicopathological characteristics of IgA nephropathy patients. The degree of IgA deposition in glomeruli was correlated with the expression of IL-6, but the correlation was not statistically significant. Among the 56 IgA nephropathy patients studied, 55 carried the GG wild type and only 1 carried the GC genotype. Among 53 normal controls studied, only 1 carried the GC genotype and the rest carried GG wild type(C allele frequency=0.009). CONCLUSION: These results suggest that IL-6 is not related with the pathophysiology of IgA nephropathy, and the -174 G to C polymorphism of IL-6 promoter region is very rare in Koreans. And, the IL-6 polymorphism at -174 is unlikely to contribute significantly to susceptibility to or the progression of IgA nephropathy in Koreans.
Alleles ; Genotype ; Glomerulonephritis, IGA* ; Humans ; Immunoglobulin A* ; Interleukin-6* ; Promoter Regions, Genetic ; RNA

BACKGROUND: Recently, it was reported that the incidence of FSGS has increased and that the corticosteroid treatment could improve its prognosis in the case of the adult patients. However, little is known about the clinical outcome and prognosis of patients with FSGS especially in Korean Adults. We performed a retrospective clinical study in 92 Korean adults with primary FSGS to determine clinical factors that have influence on the prognosis. METHODS: The subjects of this study were all adult patients(age >or=16 years) who had been diagnosed as primary FSGS through a renal biopsy between 1985 and 1999, and the patients affected by the secondary cause were excluded. We analyzed the clinical manifestation of all patients at that time of renal biopsy. In addition, in the case of patients who were followed more than 10 weeks, the rate of deterioration of renal function was analyzed retrospectively. RESULTS: Of the 92 patients, 47(51%) patients were nephrotic and 45(49%) were non-nephrotic. In terms of gender, 58(63%) of the patients were male, and 34(37%) were female. At that time of renal biopsy, 51% were in the state of renal insufficiency, and 45% were hypertensive. There was no significant difference in clinical findings of nephrotic and non-nephrotic patients at biopsy except the amount of proteinuria. Among them, 88 patients were followed up at least 10 weeks. The median of the follow-up was 37 months(from 2.5 to 185). Of the 88 patients, the renal function of 14 patients deteriorated during the follow-up; at least 50% increment of plasma creatinine over the baseline values. There was no significant difference in clinical characteristics between 14 patients and the others. The result of a multivariate analysis on clinical factors(age, hypertension, the degree of proteinuria, advanced renal insuffiency, steroid therapy) showed that nephrotic range of proteinuria, advanced renal insufficiency, and no steroid treatment were independent predictors for poor renal outcome. Overall, 80% of the 88 patients maintained stable state of a renal function for five years irrespective of steroid treatment. In the case of nephrotics, 34 patients(77%) were treated with corticosteroid. Only one out of 34 patients experienced the deterioration of renal function. On the other hand, 6 out of 10 nephrotic patients who did not launch steroid treatment, revealed worsening of renal function. CONCLUSION: The above findings suggest that corticosteroid treatment would be helpful for nephrotic adults with FSGS. Furthermore, these findings underscore the need for a controlled trial in patients with FSGS to confirm the responsiveness of corticosteroid treatment and to establish guidelines of therapy.
Adult* ; Biopsy ; Creatinine ; Female ; Follow-Up Studies ; Glomerulosclerosis, Focal Segmental ; Hand ; Humans ; Hypertension ; Incidence ; Korea* ; Male ; Multivariate Analysis ; Plasma ; Prognosis ; Proteinuria ; Renal Insufficiency ; Retrospective Studies

BACKGROUND: Common complications after hematopoietic stem cell transplantation(HCT) include sepsis, graft versus host disease(GVHD), veno-occlusive disease(VOD), drug-induced nephrotoxicity, and acute renal failure(ARF). Prior studies report that the presence of ARF affects prognosis. However, we are unaware of such reports on the incidence of ARF after HCT in Koreans, and whether or not the development of ARF is related to prognosis. The purpose of our study was to investigate the cause of ARF after HCT and its relation to prognosis. METHODS: 163 patients received HCT at Seoul National University Hospital since 1985, of which, 107 were available for review. RESULTS: ARF after HCT developed in 52 patients (48.6%). In the three clinical causes, VOD, sepsis, and GVHD, risk factor related to the development of ARF was preexisting VOD. Logistic regression confirmed this association(odds ratio 4.4). The causes of ARF were different according to the periods it developed, and cyclosporin nephrotoxicity was the main cause through the whole period after HCT. The overall survival was worse in the ARF group(60 vs 73 %; p < 0.05). ARF group was split into two groups : patients whose peak serum creatinine levels were below 3.0 mg/dL(mild ARF group) and those who were above 3.0 mg/dL(severe ARF group). Severe ARF group had worse survival than mild ARF group and patients without ARF(p < 0.01). CONCLUSION: VOD, sepsis, GVHD after HCT increase the risk of the deveolopment of ARF, but cyclosprin nephrotoxicity is the main cause of ARF. Severe ARF is a factor influencing the prognosis of patients who received HCT.
Acute Kidney Injury* ; Cell Transplantation* ; Creatinine ; Cyclosporine ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells ; Humans ; Incidence ; Logistic Models ; Prognosis* ; Renal Insufficiency ; Risk Factors ; Seoul ; Sepsis ; Transplants*

PURPOSE: This study was implemented to investigate the prevalence of proteinuria and its combined morbidity in apparently normal adults. METHODS: We examined the mass screening data of Health Promotion Center in Seoul National University Hospital from May 1, 1995 to February 11, 2000. The random urine samples of all screenees were examined by dipstick test. Among them 22,595 adults(men 11,737 and women 10,858) who didn't take anti- hypertensive medication and whose fasting blood sugar <126 mg/dL were included in this analysis. RESULTS: The prevalence of proteinuria was 6.7% in men and 3.6% in women. Risk factors for proteinuria by simple correlation analysis were age, sex, body weight, systolic blood pressure, diastolic blood pressure, fasting blood sugar, blood urea nitrogen, serum creatinine, total cholesterol and smoking. As the degree of proteinuria increased, the systolic and diastolic blood pressures also increased significantly and creatinine clearance significantly decreased above the '++' level of proteinuria. Probability of proteinuria was calculated at each blood pressure level graded by JNC VI. With the increase of the level of blood pressure, the probability of proteinuria increased significantly between normal and high normal, high normal and hypertension1, and hypertension 2 and hypertension 3 level. Creatinine clearance and blood pressure level showed negative correlation. When total screenees were divided to proteinuria and no proteinuria groups, proteinuria group showed significant decrease of creatinine clearance in high normal and hypertension 1 level. CONCLUSION: Our results suggest that proteinuria in the apparently normal adults is not a benign condition, and it can be accompanied by significantly increased blood pressures and decreased renal function.
Adult* ; Blood Glucose ; Blood Pressure ; Blood Urea Nitrogen ; Body Weight ; Cholesterol ; Creatinine ; Fasting ; Female ; Health Promotion ; Humans ; Hypertension ; Male ; Mass Screening ; Prevalence* ; Proteinuria* ; Risk Factors ; Seoul ; Smoke ; Smoking

BACKGROUND: Autosomal dominant polycystic kidney disease(ADPKD) is the most common hereditary renal disease in adults, and its major complaints include pain and abdominal fullness due to cyst expansion. So far, for the control of these symptoms, cyst ablation with ethanol or tetracycline, laparoscopic manipulations and surgical marsupialization have been used. METHODS: We used conventional ethanol(n=9) or n-butyl cyanoacrylate(NBCA) plus lipiodol solution (n=18) or both(n=3) for separate cysts as the sclerosing agent in 24 adult Korean ADPKD patients. And their clinical courses after treatment were evaluated. RESULTS: The male to female ratio was 8 : 16 and the mean age at the treatment was 50 yrs(S.D. 13.1). Causes for aspiration were pain in 14 and abdominal fullness in 7 patients and the range for the cyst diameters aspirated were 5-16 cm. Flank pain or discomfort were decreased subjectively in most cases except two. Mean arterial pressures(S.D.) (mmHg) before and after procedure were as follows 112(11.1)(basal), 96(9.6)(1 month) and 98(9.7)(6 month)(p < 0.05, paired-t test). Blood urea nitrogen levels(mg/dL) were not changed 6 month later[24 (12.1) vs. 22(14.6)]. There was no major complication such as bleeding or infection and no death and associated with procedure. There was no difference of therapeutic effect according to sclerosing agent. CONCLUSION: NBCA was as effective as conventional ethanol for sclerotherapy in ADPKD and cyst ablation therapy showed a BP-lowering effect in short-term period.
Adult ; Blood Urea Nitrogen ; Ethanol ; Ethiodized Oil ; Female ; Flank Pain ; Hemorrhage ; Humans ; Male ; Polycystic Kidney, Autosomal Dominant* ; Sclerotherapy* ; Tetracycline

PURPOSE: Fungal peritonitis is a fatal disease with a high mortality and morbidity to the peritoneal dialysis(PD) patients. This study was implemented to provide a guideline for the prevention and treatment of fungal peritonitis in PD patients by analyzing the clinical and microbiologic features of fungal peritonitis cases. METHODS: We analyzed retrospectively into the 15 cases(14 patients) of fungal peritonitis among 376 end stage renal disease(ESRD) patients who newly started PD in the Seoul National University Hospital from Jan. 1991 to Dec. 1999. RESULTS: The patients' age was 53.6+/-11.6 years (mean+/-standard deviation) and their male to female ratio was 12:3. They have been on PD for 29.2+/-27.7 months before the fungal peritonitis developed. Candida species was the most common etiologic agent, accounting for 10(62.5%) out of the 16 fungal organisms isolated from our patients. Among others were two Aspergillus, one Cryptococcus, one Penicillium, one Torulopsis, and one Trichosporon beigelii cases. Bacterial agents were isolated simultaneously in five fungal peritonitis cases. Peritoneal catheters were all removed no later than 72 hours after the diagnosis was made. Patients were given a single or combined therapy with amphotericin B, fluconazole, or flucytosine on the physician's choice. The outcomes of fungal peritonitis were as follows; 20% continued PD, 60% converted to HD and 20% died of fungal peritonitis. We made a comparative analysis between the fungal and bacterial peritonitis cases which developed in the same 5-year period, which showed significantly higher catheter removal and technique failure rates in the fungal cases. CONCLUSION: Fungal peritonitis is a rare but a fatal disease with a high mortality and a technique failure rate. Candida species was the most prevalent microorganism in our study.
Amphotericin B ; Aspergillus ; Candida ; Catheters ; Cryptococcus ; Diagnosis ; Female ; Fluconazole ; Flucytosine ; Fungi ; Humans ; Male ; Mortality ; Penicillium ; Peritoneal Dialysis* ; Peritonitis* ; Retrospective Studies ; Seoul ; Trichosporon