Minimal change nephrotic syndrome showing complete remission after resection of a neurofibroma in a type I neurofibromatosis patient

No abstract available.

The Natural Course of Biopsy-Proven Isolated Microscopic Hematuria: a Single Center Experience of 350 Patients

The increasing interest in healthcare and health screening events is revealing additional cases of asymptomatic isolated microscopic hematuria (IMH). However, a consensus of the evaluation and explanation of the IMH prognosis is controversial among physicians. Here, we present the natural course of IMH together with the pathological diagnosis and features to provide supportive data when approaching patients with IMH. We retrospectively evaluated 350 patients with IMH who underwent a renal biopsy between 2002 and 2011, and the pathological diagnosis and chronic histopathological features (glomerulosclerosis, interstitial fibrosis, and tubular atrophy) were reviewed. Deterioration of renal function was examined during follow up. The patients with IMH were evaluated for a mean of 86 months. IgA nephropathy was the most common diagnosis in 164 patients (46.9%). Chronic histopathological changes were observed in 166 (47.4%) but was not correlated with proteinuria or a decline in renal function. Ten patients developed proteinuria, and all of them had IgA nephropathy. Three patients progressed to chronic kidney disease with an estimated glomerular filtration rate < 60 mL/min/1.73 m2 but none progressed to end stage renal disease. In conclusion, IMH had a generally benign course during 7-years of observation, although IgA nephropathy should be monitored if it progresses to proteinuria. Future prospective randomized studies may help conclude the long-term prognosis and lead to a consensus for managing IMH.

Benefits of a Continuous Ambulatory Peritoneal Dialysis (CAPD) Technique with One Icodextrin-Containing and Two Biocompatible Glucose-Containing Dialysates for Preservation of Residual Renal Function and Biocompatibility in Incident CAPD Patients

In a prospective randomized controlled study, the efficacy and safety of a continuous ambulatory peritoneal dialysis (CAPD) technique has been evaluated using one icodextrin-containing and two glucose-containing dialysates a day. Eighty incident CAPD patients were randomized to two groups; GLU group continuously using four glucose-containing dialysates (n=39) and ICO group using one icodextrin-containing and two glucose-containing dialysates (n=41). Variables related to residual renal function (RRF), metabolic and fluid control, dialysis adequacy, and dialysate effluent cancer antigen 125 (CA125) and interleukin 6 (IL-6) levels were measured. The GLU group showed a significant decrease in mean renal urea and creatinine clearance (-Delta1.2+/-2.9 mL/min/1.73 m2, P=0.027) and urine volume (-Delta363.6+/-543.0 mL/day, P=0.001) during 12 months, but the ICO group did not (-Delta0.5+/-2.7 mL/min/1.73 m2, P=0.266; -Delta108.6+/-543.3 mL/day, P=0.246). Peritoneal glucose absorption and dialysate calorie load were significantly lower in the ICO group than the GLU group. The dialysate CA125 and IL-6 levels were significantly higher in the ICO group than the GLU group. Dialysis adequacy, beta2-microglobulin clearance and blood pressure did not differ between the two groups. The CAPD technique using one icodextrin-containing and two glucose-containing dialysates tends to better preserve RRF and is more biocompatible, with similar dialysis adequacy compared to that using four glucose-containing dialysates in incident CAPD patients. [Clincal Trial Registry, ISRCTN23727549]

Benefits of a Continuous Ambulatory Peritoneal Dialysis (CAPD) Technique with One Icodextrin-Containing and Two Biocompatible Glucose-Containing Dialysates for Preservation of Residual Renal Function and Biocompatibility in Incident CAPD Patients

In a prospective randomized controlled study, the efficacy and safety of a continuous ambulatory peritoneal dialysis (CAPD) technique has been evaluated using one icodextrin-containing and two glucose-containing dialysates a day. Eighty incident CAPD patients were randomized to two groups; GLU group continuously using four glucose-containing dialysates (n=39) and ICO group using one icodextrin-containing and two glucose-containing dialysates (n=41). Variables related to residual renal function (RRF), metabolic and fluid control, dialysis adequacy, and dialysate effluent cancer antigen 125 (CA125) and interleukin 6 (IL-6) levels were measured. The GLU group showed a significant decrease in mean renal urea and creatinine clearance (-Delta1.2+/-2.9 mL/min/1.73 m2, P=0.027) and urine volume (-Delta363.6+/-543.0 mL/day, P=0.001) during 12 months, but the ICO group did not (-Delta0.5+/-2.7 mL/min/1.73 m2, P=0.266; -Delta108.6+/-543.3 mL/day, P=0.246). Peritoneal glucose absorption and dialysate calorie load were significantly lower in the ICO group than the GLU group. The dialysate CA125 and IL-6 levels were significantly higher in the ICO group than the GLU group. Dialysis adequacy, beta2-microglobulin clearance and blood pressure did not differ between the two groups. The CAPD technique using one icodextrin-containing and two glucose-containing dialysates tends to better preserve RRF and is more biocompatible, with similar dialysis adequacy compared to that using four glucose-containing dialysates in incident CAPD patients. [Clincal Trial Registry, ISRCTN23727549]

Efficacy of Hemocontrol Biofeedback System in Intradialytic Hypotension-Prone Hemodialysis Patients

We conducted a study to determine whether the hemocontrol biofeedback system (HBS) can improve intradialytic hypotension (IDH) in hypotension-prone hemodialysis (HD) patients compared with conventional HD. In this multicenter prospective crossover study, 60 hypotension-prone patients were serially treated by conventional HD for 8 weeks (period A), by HD with hemoscan blood volume monitoring for 2 weeks (period B0), and by HBS HD for 8 weeks (period B1). The number of sessions complicated by symptomatic IDH during 24 HD sessions (14.9+/-5.8 sessions, 62.1% in period A vs 9.2+/-7.2 sessions, 38.4% in period B1, P<0.001) and the number of IDH-related nursing interventions in a session (0.96+/-0.66 in period A vs 0.56+/-0.54 in period B1, P<0.001) significantly decreased in period B1 than in period A. Recovery time from fatigue after dialysis was significantly shorter in period B1 than in period A. The patients with higher post-dialysis blood pressure, lower difference between pre- and post-dialysis blood pressure, less frequent IDH, and higher pre- and post-dialysis body weight in period A responded better to HBS in period B1 in regard to the reduction of IDH. In conclusion, HBS may improve the patient tolerability to HD by reducing the IDH frequency and promoting faster recovery from fatigue after dialysis.

Pneumatosis Intestinalis with Hepatic Portal Venous Gas in a Hemodialysis Patient / 대한신장학회지

Pneumatosis intestinalis is an uncommon but important condition in which gas is found in a linear or cystic form in the submucosa or subserosa of the bowel wall. It occurs in several clinical settings in adults who have a wide variety of underlying disorders that determine prognosis. Especially, hepatic portal venous gas with pneumatosis intestinalis has been rarely described in chronic dialysis patients. We report a case of 53-year-old man with hemodialysis-dependent end stage renal disease who developed a pneumatosis intestinalis accompanied hepatic portal venous gas. This patient was treated conservatively with intravenous antibiotics, fluid therapy, and oxygen supply without surgical approach.

Anatomical Variation of Internal Jugular Vein in Korean Hemodialysis Patients / 대한신장학회지

PURPOSE: The internal jugular vein (IJV) is a preferred site for central cannulation for hemodialysis (HD) because of its low incidence of central vein stenosis. Although anatomically IJV is commonly located on the anterior-lateral side of the carotid artery, some patients have anatomical variation of IJV, which can lead to difficulty and complication of cannulation. This study was performed to evaMETHODS: We enrolled 358 patients receiving IJV catheter cannulation for HD using doppler ultrasonography between January 2007 and February 2009. We examined the anatomical positions of IJV in relation to the position of carotid artery (CA) and incidence of anatomical variation on both sides. We also investigated incidence of inadequate IJV for cannulation, RESULTS: The mean age of 358 enrolled patients was 57+/-15 years (14-88 years) (M:F=203:155). Anatomical variations of the left (Lt) and right (Rt) IJV position relative to the CA were found in 36.3% and 27.1%, respectively. Various anatomical variations of IJV position were discovered in the anterior side (Lt 23.7%, Rt 21.2%), anterior-medial side (Lt 7%, Rt 2.5%), and the lateral side (Lt 1.1%, Rt 1.7%) relative to CA. Inadequate Lt and Rt IJVs for cannulation, which can be too small sized or obstructed, were 6.4% and 2.8%, respectively. CONCLUSION: About one third of Korean HD patients had anatomical variations of IJV position relative to the CA. This study supports the use of doppler ultrasound guided technique for IJV cannulation in HD patients.

Clinical characteristics of bladder cancer identified by multi-detector computed tomography in patients with asymptomatic isolated hematuria / 대한내과학회지

BACKGROUND/AIMS: This report discusses the frequency and clinical characteristics of bladder cancer diagnosed with multi-detector computed tomography (MDCT) in outpatients visiting a nephrology and urology clinic with asymptomatic isolated hematuria. METHODS: MDCT was performed on outpatients who presented to the nephrology and urology clinic at the Holy Family Hospital of the Catholic University of Korea with asymptomatic isolated hematuria from January 2005 to December 2007. RESULTS: Of the 1,819 patients who underwent MDCT, the results were normal in 54.1%, showed a simple renal cyst in 25.7%, a renal calculus in 4.7%, and a malignant tumor of the urinary tract in 1.7%. Twenty-five patients (1.3%) were diagnosed with bladder cancer, including 20 (80%) men and 5 (20%) women. The average age of the patients with bladder cancer was 74.5+/-7.3 years. The 25 patients diagnosed with bladder cancer had a higher rate of gross hematuria, older age, and male gender (p<0.05) compared to the other patients. In addition, their hemoglobin and albumin levels were significantly lower (p<0.05). For all patients, the frequency of bladder cancer in those with asymptomatic microscopic hematuria, excluding those with gross hematuria, was extremely low (0.3%, 6 patients). CONCLUSIONS: MDCT may constitute an adequate diagnostic test for patients with asymptomatic hematuria. Older male patients with gross hematuria require a thorough urologic evaluation.

A Synonymous Genetic Alteration of LMX1B in a Family with Nail-Patella Syndrome

The gene responsible for nail-patella syndrome, LMX1B, has recently been identified on chromosome 9q. Here we present a patient with nail-patella syndrome and an autosomal dominant pattern of inheritance. A 17-year-old girl visited our clinic for the evaluation and treatment of proteinuria. She had dystrophic nails, palpable iliac horns, and hypoplastic patellae. Electron microscopy of a renal biopsy showed irregular thickening of the glomerular basement membrane. A family history over three generations revealed five affected family members. Genetic analysis found a change of TCG to TCC, resulting in a synonymous alteration at codon 219 in exon 4 of the LMX1B gene in two affected family members. The same alteration was not detected in an unaffected family member. This is the first report of familial nail-patella syndrome associated with an LMX1B in Korea mutation, However, we can not completely rule out the possibility that the G-to-C change may be a single nucleotide polymorphism as this genetic mutation cause no alteration in amino acid sequence of LMX1B.

Status of Initiating Pattern of Hemodialysis: A Multi-center Study

This study was to evaluate the status of initiating pattern of hemodialysis (HD). Five hundred-three patients in 8 University Hospitals were included. Presentation mode (planned vs. unplanned), and access type (central venous catheters [CVC] vs. permanent access) at initiation of HD were evaluated, and the influence of predialysis care on determining the mode of HD and access type was also assessed. Most patients started unplanned HD (81.9%) and the most common initial access type was CVC (86.3%). The main reason for unplanned HD and high rate of CVC use was patient-related factors such as refusal of permanent access creation and failure to attend scheduled clinic appointments. Predialysis care was performed in 57.9% of patients and only 24.1% of these patients started planned HD and 18.9% used permanent accesses initially. Only a minority of patients initiated planned HD with permanent accesses in spite of predialysis care. To overcome this, efforts to improve the quality of predialysis care are needed.

The Impact of the Aortic Pulse Wave Velocity on the Cardiovascular Outcomes of Hemodialysis Patients

The aims of our study were to identify the risk factors for an increased aortic pulse wave velocity (AoPWV) and to assess the impact of the AoPWV on the cerebro-cardiovascular (CV) outcomes of hemodialysis (HD) patients. Seventy two HD patients were included, and the AoPWV, the echocardiography and the biochemical parameters were measured. After dividing the patients into tertiles according to the AoPWV values, we defined the low, the middle and the high AoPWV groups. The patients in the high AoPWV group showed a significantly higher age and high-sensitivity C-reactive protein level, a greater prevalence of diabetes and statin use, left ventricular hypertrophy, average pulse pressure (PP), AoPWV and left ventricular mass index and a lower serum albumin level than those in the low AoPWV group (p<0.05). On multivariate regression analysis of the AoPWV, age and the average PP were independently related to the AoPWV (p<0.05). On the multivariate Cox analysis for CV outcomes, the AoPWV and the average PP remained significant independent predictors of CV events. Our data suggest that an increased AoPWV is an independent predictor for the CV outcomes of HD patients.

The Relation of Arterial Stiffness with Vascular Calcification and BMD in Chronic Kidney Disease / 대한신장학회지

PURPOSE:Vascular calcification with arterial stiffness as well as bone mineral density was compared in hemodialysis, peritoneal dialysis patients, and pre-dialysis patients. METHODS:The calcification level of the aorta was scored, the arterial stiffness level was examined by pulse wave velocity (PWV), and bone mineral density was measured by the use of DEXA, and analyzed. RESULTS:PWV was significantly higher in the calcification group. The systolic blood pressure and the PWV value of the HD group was r=0.566 (p<0.001), the PD group was r=0.711 (p<0.001), and the pre-dialysis patients group was r=0.461 (p=0.001), and in all groups, a high correlation was shown. In the association of the PWV value with BMD and T score, in the PD patient group, with spine BMD, it was r=-0.351 (p<0.05), femur BMD was r=-0.510 (p<0.01), and femur T score was r=-0.527 (p= 0.001). In the multivariate analysis of the PWV value, in the HD group, age and systolic blood pressure were significant and in the PD group, calcification score femur BMD, femur T score, and CRP were significant factors. In the pre-dialysis patients group, only femur T score was detected to be a significant factor for PWV. CONCLUSION:In hemodialysis patients, age and systolic blood pressure, and in peritoneal dialysis patients, vascular calcification and the BMD level were analyzed to be significant factors mediating effects on arterial stiffness.

Acquired Gitelman Syndrome

Acquired renal tubular disorder can be observed in various disease processes, especially autoimmune diseases. Gitelman syndrome is an autosomal recessive disease characterized by hypokalemic metabolic alkalosis, hypomagnesemia, and hypocalciuria. This disorder is caused by mutation in the SLC12A3 gene, which encodes the thiazide - ensitive NaCl cotransporter (NCCT). Acquired Gitelman syndrome has been reported and the majority has been associated with Sjogren's syndrome. The presence of circulating auto - antibodies to NCCT was suggested as a mechanism of acquired Gitelman syndrome. Treatment of acquired Gitelman syndrome was done with supplements of potassium and magnesium and prednisone was effective in some cases. Acquired Gitelman syndrome should be included in the differential diagnosis of renal involvement in patients with autoimmune diseases, especially Sjogren's syndrome.

A Case Report of Rhabdomyolysis Associated With Acute Renal Failure Following Laparoscopic Radical nephrectomy / 대한신장학회지

Postoperative rhabdomyolysis with acute renal failure (ARF) is a rare complication of laparoscopic urologic surgery. It is associated with lateral decubitus surgical position, long operative time, and increased body mass. We report a case of a 49-year-old-man with right renal cell carcinoma who underwent a laparoscopic right radical nephrectomy for 7 hours in left decubitus position and was complicated by ARF due to postoperative rhabdomyolysis. Laparoscopic renal surgery is performed in many surgical renal diseases and heightened suspicion may help early recognition of postoperative rhabdomyolysis. Vigorous hydration and hemodialysis are also helpful in treating ARF secondary to rhabdomyolysis

A Case of Acute Pyelonephritis Complicated with Renal Vein Thrombosis / 대한신장학회지

Acute pyelonephritis is a common disease in clinical practice. Renal vein thrombosis in acute pyelonephritis has become a rare complication at present because of the advances of antibiotics. The trend in management has shifted to non-surgical therapies, particularly systemic anticoagulation, except in highly selected group of patients. Here we report the case of a 67-year-old woman who got hospitalized for fever and chilling. Acute pyelonephritis was diagnosed by clinical manifestation and positive urine and blood cultures. Computed tomography demonstrated left pyelonephritis and ipsilateral renal vein thrombosis. She was fully recovered after treatment with antibiotics, low molecular weight heparin and warfarin for 8 weeks.

The Incidence and Clinical Course of Acute Renal Failure in Patients with Severe Acute Pancreatitis / 대한신장학회지

PURPOSE: Although acute renal failure (ARF) commonly develops in patients with severe acute pancreatitis (SAP), the impact of ARF on disease severity is rarely reported in Korea. This study was performed to compare the clinical findings, morbidity and mortality between SAP patients with and without ARF. METHODS: We retrospectively evaluated the medical records of 102 patients with SAP between january 2001 and June 2008 in 3 hospitals. We investigated the incidence and clinical course of ARF in SAP patients. Then, we compared morbidity and mortality between the patients with ARF and normal renal function (NRF). RESULTS: Of the total 102 SAP patients, ARF was observed in 39 patients (38.2%). The peak serum creatinine level in ARF patients was 4.5+/-2.3 mg/dL. Eight of the 39 ARF patients (20.5%) received hemodialysis and ten patients (25.6%) died. When compared to NRF patiens, ARF patients (n=39) had higher incidence of dyspnea (17.9% vs 3.2%, p=0.011), loss of consciousness (17.9% vs 1.6%, p=0.003), and APACHE II scores more than 8 (92.3% vs 0%, p<0.001). The ARF group had also higher incidences of sepsis (35.9% vs 7.9%, p<0.001), multiorgan failure (15.4% vs 0%, p=0.001), respiratory failure (28.2% vs 4.7%, p=0.001) and mortality (25.6% vs 3.2%, p=0.001). Multivariate analysis demonstrated thrombocytopenia, hemoconcentration, and high LDH as independent risk factors of ARF in SAP patients. CONCLUSION: The incidence of ARF was high (38.2%) and ARF patients showed higher morbidity and mortality, compared to NRF patients. We suggest that early management of ARF should be performed for reducing the mortality in SAP patients.

Natural History and Renal Pathology in Patients with Isolated Microscopic Hematuria

BACKGROUND/AIMS: No definite conclusions have been reached about the natural history of patients with isolated microscopic hematuria (IMH). In this study, we observed the natural history of patients with IMH and examined factors related to a pathologic diagnosis and subsequent prognosis. METHODS: We retrospectively evaluated 156 subjects with IMH who had a renal biopsy performed. Of the 156 subjects, 33.3% were diagnosed with IgA nephropathy, 23.7% with mesangial proliferative glomerulonephritis, 15.4% with glomerular minor lesion, and 12.8% with thin basement membrane nephropathy; 6.4% had normal biopsies. RESULTS: We followed up with 100 subjects for about 31 months. During this follow-up period, two subjects who had received a pathologic diagnosis of IgA nephropathy developed chronic kidney disease. During the course of the study, one of these subjects presented with proteinuria and hypertension and the other with proteinuria. The overall incidences of proteinuria and hypertension were 6% and 5% respectively. CONCLUSIONS: The prognosis for patients with IMH was relatively favorable, but patients developing proteinuria and/or hypertension require careful observation and management during the follow-up period.

The Clinical Differentiations between Diabetic Nephropathy and Non-diabetic Renal Disease in Type 2 Diabetic Patients / 대한신장학회지

PURPOSE: It is important to differentiate non-diabetic renal diseases (NDRD) from diabetic nephropathy (DN) in type 2 diabetes. Our study was reviewing the clinical data and treatment strategies from diabetic patients performed renal biopsy to determine the clinical indicators suggestive of NDRD METHODS: We reviewed the medical records of type 2 patients who underwent renal biopsy from Jan. 1995 to Dec. 2007. RESULTS: Seventy four patients were included. Mean age was 52.0+/-12.5 years and 41 (55%) patients were male suddenly developed. Nephrotic syndrome [34 cases (46%)] was the leading reason for renal biopsy. There were 37 cases (50%) with a pathologic diagnosis of DN, 31 (42%) with NDRD, and 6 (8%) with concurrent DN and NDRD. IgA nephropathy (35%) was the most common lesion found in patients with NDRD. Thirty one patients (84%) with DN and 26 (84%) with NDRD had follow-up periods of more than 6 months. Of 26 patients with NDRD, 12 were treated with immune suppressants and 6 achieved complete remission. Thirteen patients with DN and one with NDRD developed end-stage renal disease. Patients with NDRD tended to show shorter duration of diabetes, lower systolic blood pressure (SBP) and lower serum triglyceride, and had significantly lower incidence of diabetic retinopathy (DR). In the univariate regression analysis, diabetes duration, SBP, triglyceride and DR showed statistically significance. And SBP and DR were identified as independent correlating factors by multivariate regression analysis. CONCLUSION: In this study, the absence of retinopathy could predict the presence NDRD among NIDDM patients presenting with renal disease. And additional disease-specific therapies may be helpful for the patients with NDRD.

The Effect of Intraperitoneal Calcitriol Pulse Therapy in CAPD Patients with Secondary Hyperparathyroidism / 대한신장학회지

PURPOSE: In hemodialysis patients with secondary hyperparathyroidism, intravenous administration of calcitriol became widely utilized. In CAPD patients, however, the intravenous administration of calcitriol is not practical. The purpose of the present study was to determine the effect and safety of intraperitoneal (IP) calcitriol pulse therapy in CAPD patients. METHODS: All patients undergoing CAPD between January 2006 and January 2007 and willing to give informed consent were eligible. Inclusion criteria were age greater 18 years, on CAPD for at least 6 months, and secondary hyperparathyroidism (intact PTH >300 pg/mL). Intraperitoneal calcitriol was given by direct infusion into the dialysate (2.0 microgram) twice per week. If hypercalcemia (>10.5 mg/dL) and hyperphosphatemia (>6.5 mg/dL) developed, the patients were excluded from study. RESULTS: Eighteen patients were enrolled into the study. Among them, 16 patients completed the study period. After IP calcitriol for 3 months, there was a significant drop of iPTH level from the pretreatment level of 490+/-234 pg/mL to the level of 318+/-315 pg/mL (p<0.05). There were no definite hypercalcemia during the study period, and only 1 patient was excluded from study due to hyperphosphatemia. CONCLUSION: In CAPD patients, IP calcitriol pulse therapy is effective in treating secondary hyperparathyroidism, and that IP calcitriol pulse therapy is associated with a low incidence of hypercalcemia and hyperphosphatemia.

Association of Arterial Stiffness with the Decrease of Post-Dialysis Blood Pressure in Maintained Hemodialysis Patients / 대한신장학회지

PURPOSE: Cardiovascular disease is one of the leading causes of mortality and morbidity in hemodialysis patients. Arterial stiffness is known to be associated with vascular calcification in HD patients. Post-dialysis hypotension is a risk factor for cardiovascular mortality. We evaluated the relation between arterial stiffness and post-dialysis blood pressure in maintained HD patients. METHODS: 72 HD patients were enrolled in this study. They had been under maintenance HD for more than 3 months. We checked the biochemical data, including the troponin T, CRP and OPG (osteoprotegerin) levels before their dialysis session and the baPWV (brachio-ankle pulse wave velocity) after the session. We defined post-dialysis BP decrease as a drop of BP of more than 5% of the average MAP for 2 weeks (6 sessions). RESULTS: There were 34 and 38 patients with and without post-dialysis BP decrease, respectively. The BP decrease group had higher CRP, troponin T and OPG levels (p<0.05, 0.01 and 0.01, respectively). The PWV was higher in the BP decrease group (p<0.001). The CRP and troponin T levels were positively correlated with the PWV (r=0.26, p<0.05; r=0.31, p<0.01, respectively). The OPG level was positively correlated with the PWV (r=0.44, p<0.001). Age, pre-dialysis pulse pressure and drops in the patients MAP were correlated with the PWV (r=0.33, p<0.05; r=0.31, p<0.05; r=0.30, p<0.05, respectively). On multivariate analysis, PWV was the independent factor related to the drops in the MAP of the patients (beta=0.311, p=0.021). CONCLUSION: Arterial stiffness is associated with post-dialysis blood pressure decrease.