A Case of Primary Hyperoxaluria with Renal Allograft Dysfunction / 대한신장학회지

Primary hyperoxaluria is a rare disorder of glyoxylate metabolism in which hepatic enzyme deficiencies result in overproduction of oxalate. The resulting elevation of urinary oxalate excretion leads to recurrent urolithiasis and progressive nephrocalcinosis. End-stage renal disease frequently occurs and is accompanied by systemic oxalate deposition along with its harmful effects. With the rarity and various clinical heterogeneity of the disease, the high proportion of patients in whom diagnosis is made after advanced renal failure have developed it. On account of its high rate of graft loss associated with primary hyperoxaluria, isolated kidney transplantation has been replaced by combined liver/kidney transplantation. In this report, we describe a case of primary hyperoxaluria with kidney graft failure who had a history of recurrent renal stones.

Outcome of Cadaveric Kidney Transplantation from Expanded Criteria Donors / 대한내과학회지

BACKGROUND/AIMS: The Korean Network for Organ Sharing (KONOS) was established in 2000, and the KONOS criteria for expanded-criteria donors (ECD) have since been applied to kidney allocation from deceased donors. The outcome of cadaveric kidney transplantation from ECD according to KONOS criteria has not been investigated. METHODS: Seventy-seven cadaveric kidney transplants from January 2003 to December 2009 were recruited retrospectively. Factors that influence the glomerular filtration rate (GFR) of graft kidneys up to 36 months after transplantation were evaluated. Postoperative renal function and allograft and patient survival in the ECD group (n = 28) were compared with those in the standard-criteria donor (SCD) group (n = 49). RESULTS: The GFR of graft kidneys was different according to donor GFR, age, hypertension history, and cause of brain death. In the ECD group, GFR was lower than that in the SCD group by KONOS criteria. No differences in allograft or patient survival were observed until 3 years after kidney transplantation. CONCLUSIONS: Cadaveric kidney transplantation using the ECD by KONOS criteria is acceptable in term of graft and 3-year patient survival, although the GFR was lower in the ECD than in the SCD group.

Sustained Low-Efficiency Dialysis as an Alternative Therapy to Continuous Renal Replacement Therapy in Critically Ill Patients with Acute Kidney Injury / 대한신장학회지

PURPOSE: Although continuous renal replacement therapy (CRRT) is commonly used as a renal replacement therapy in critically ill patients with acute kidney injury, it has some disadvantages such as inconvenience, intensive labor, expensiveness and high bleeding risk. Recent studies have shown that sustained-low efficiency dialysis (SLED) can overcome these shortages of CRRT and also has the advantages of CRRT. We prospectively compared the efficiency, safety, cost and convenience between SLED and CRRT, and evaluated whether SLED could be a complementary substitute to traditional CRRT. METHODS: Forty-six critically ill patients with acute kidney injury (AKI) from 2003 to 2005 were treated with SLED (n=25) and CRRT (n=21). The modality was tended to be selected randomly and based largely on availability of equipments and not on the clinical status of patients. Mann-Whitney rank-sum test, Fisher's exact test and chi-square test were used for statistics, and data were described as median value, range from 25th to 75th. RESULTS: The Acute Physiology and Chronic Health Evaluation (APACHE) II score at the point of ICU admission was 27 for SLED (range 17-32, 25-75th percentile) and 26 for CRRT group (range 19-31) (p=NS). There were no significant differences between the two groups in mean arterial blood pressure when measured pre-dialysis (83 for SLED vs. 85 for CRRT; p=NS), mid-dialysis (90 vs. 84; p=NS) and at the end of treatment (88 vs. 80; p=NS). Incidences of hypotension also did not differ between the two groups (p=NS). Ultrafiltration volume per treatment day was similar in two treatment modalities (2,000 mL for SLED vs. 2,400 mL for CRRT; p=NS). Heparin was used to thirty-one patients (SLED: 18 patients, CRRT: 13 patients). The dosage of heparin tended to be lower in SLED (2,900 unit per day for SLED vs. 6,000 unit per day for in CRRT, p=0.065). Total clotting number was 4 for SLED and 11 for CRRT (p=NS). There was also no significant difference in hospital mortality between the two groups (56% for SLED vs. 42.9% for CRRT; p=NS). SLED was superior to CRRT in the aspect of cost and convenience. CONCLUSION: Our data suggest that SLED can be used as a useful substitute to traditional CRRT in critically ill patients with AKI.

Clinical characteristics of p-ANCA (anti-neutrophil cytoplasmic antibody)-related nephritis: a single center experience / 대한내과학회지

BACKGROUND/AIMS: We evaluated the clinical characteristics and prognostic value of the clinical, laboratory, pathologic features, at time of diagnosis, and the renal survival of patients with ANCA (anti-neutrophil cytoplasmic antibody)-related nephritis. METHOD: We retrospectively analyzed 17 patients who were diagnosed with ANCA-related nephritis at a single center. The risks of progression to ESRD or death according to the clinical parameters, the ANCA pattern and the renal pathologic findings were evaluated. RESULTS: The major symptoms were hematuria (100%), proteinuria (100%), uremic symptoms (41.2%), edema (35.5%), upper respiratory symptoms (29.4%) and oliguria (23.5%), which were not correlated with renal survival. All the patients showed a p-ANCA pattern. The BUN level (p=0.032) and GFR (p=0.023) at the time of diagnosis were different between the improved and the progressed patients in terms of renal function. The pathology indices were not predictive factors of both renal and patient survival. Eight patients (47.1%) were treated with steroid IV pulse, 4 (23.5%) with steroid IV pulse and cyclophosphamide IV pulse, 2 (11.8%) with steroid IV pulse, cyclophosphamide IV pulse and plasma exchange, and 2 (11.8%) with steroid IV pulse and plasma exchange. Fourteen patients (82.4%) needed hemodialysis. There were 3 (17.6%) disease-related deaths, 13 patients (76.5%) reached ESRD and 4 (23.5%) showed recovery of renal function. The mean percent of patients who survived was 80.2% and the mean percent of renal survival was 33.3% at the 1st and 3rd year, respectively. CONCLUSIONS: Poor renal function at presentation was associated with a high risk for disease progression, but age, gender, the clinical patterns of presentation and the pathologic findings were not associated with the prognosis. Early diagnosis and treatment seems to be essential to improve the renal outcomes.

Clinical factors affecting long-term patency after percutaneous transluminal angioplasty of insufficient hemodialytic arteriovenous fistula / 대한내과학회지

BACKGROUND: The aim of this study is to evaluate success rate and patency rate after percutaneous transluminal angioplasty (PTA) and to determine the factors affecting patency rate afterPTA in the management of insufficient arteriovenous fistula of hemodialysis patients. METHODS: Ninety-two cases of insufficient arteriovenous fistulae in 73 hemodialysis patients underwent angiography of the fistula and were treatedby PTA (native AVF: 67 cases, AV graft: 10 cases, central vein: 15 cases). The initial success rate and complications of PTA were evaluated. All patients were divided into two groups according to the age, sex, the duration of hemodialysis, a history of diabetes mellitus, the numbers of AVF operation, and the presence or absenceof thrombus. The patency rate was compared with the Kaplan-Meier method with log-rank test. To determine the clinical factors that affect vascular access failure rate, Cox regression method was used. RESULTS: The initial success rate of PTA was 78.2% (72/92). The patency rate after successful PTA was 80.0% at 6 months, and 73.2% at 12 months. The patency rate after successful PTA was significantly lower in cases which had two or more AVF operation (62.3% and 48.5% at 6 and 12 months) than just one (87.8% and 80.5% at 6 and 12 months). The most common PTA related complications were vessel spasm (3.9%) and hematoma (3.9%). CONCLUSIONS: The numbers of AVF operation was the only risk factor which affected the patency rate.

Gadolinium-enhanced Three-dimensional Magnetic Resonance Angiography in the Evaluation of Potential Renal Donors for Laparoscopic Donor Nephrectomy: Is it Alternative or Supplementary Tools to Intraarterial Digital Subtraction Angiography? / 대한신장학회잡지

BACKGOUND: The assessment of renal vasculature should be performed by an appropriate imaging technique before kidney transplantation. The current standard technique is intra-arterial digital subtraction angiography. Digital subtraction renal angiography (DSA) is associated with multiple problems. Three dimensional (3D) gadolinium enhanced magnetic resonance angiography (Gd-enhanced MRA) has been suggested as a potential technique due to its noninvasive nature. The aim of this study was to evaluate the accuracy of Gd-enhanced MRA of potential live donors before renal transplantation. METHODS: From September 2002 to September 2004, forty potential live kidney donors were evaluated using Gd-enhanced MRA and intraarterial DSA. Concordance rate and kappa were calculated in 38 transplantations except 2 patients who could not be operated after preoperative evaluation. Gd-enhanced MRA findings were compared with conventional DSA and surgical findings as the reference method respectively. RESULTS: In five donors, the findings at surgery were discrepant with Gd-enhanced MRA findings (concordance rate=86.8%) and conventional DSA findings (concordance rate=86.8%). Two accessory arteries initially were missed by Gd-enhanced MRA. They were depicted retrospectively by discussion with a radiologist, which was radiologic reporting error. There were no adverse events during the Gd-enhanced MRA procedure. None of the findings missed by Gd-enhanced MRA resulted in deleterious consequences at laparoscopic nephrectomy for the donor and graft. CONCLUSION: We demonstrated that Gd-enhanced MRA is an accurate, minimally invasive technique for assessing the live donors before renal transplantation. Clinical feedback and optimization of imaging techniques should be continued to improve accuracy rate in preoperative assessment of potential live donors who are candidates for laparoscopic nephrectomy.

A Case of Cytomegalovirus (CMV) Panniculitis after Renal Transplantation / 대한이식학회지

Cytomegalovirus (CMV) remains an important pathogen in organ transplant patients. However, cutaneous lesions are rare manifestation of systemic CMV infection. We report a case of CMV panniculitis in a 47-year-old renal transplant recipient. She admitted due to fever, local pain in the left pelvic area, and erythematous tender palpable mass for 2 weeks. Twenty years ago, she underwent renal transplantation and had taken azathioprin 100 mg and prednisone 10 mg daily. Serum creatinine had been preserved between 2.5 and 3.5 mg/dL. Bacterial, fungal, and AFB cultures of skin lesion and drained pus were negative. CMV antigenemia was noted. Skin biopsy revealed multinucleated dermal histiocytes and positive for CMV antibody by immunohistochemical staining. Polymerase chain reaction testing of skin tissue and drained pus revealed CMV DNA. She was treated with ganciclovir for four weeks and skin lesion was completely resolved without recurrence.

Long Term Effects of Dialysis Adequacy, Residual Renal Function, Nutritional Indices on CAPD Patient Outcome / 대한신장학회잡지

PURPOSE: Several factors including dialysis adequacy are now known to be related to clinical outcomes of CAPD patients. In our study, the long term effects of dialysis adequacy, residual renal function, nutritional indices, and peritoneal membrane transport characteristics on the outcomes of CAPD patients were evaluated. METHODS: Prevalent or incident 127 patients were enrolled from February 1998 to January 1999. Follow-up was terminated in January 2003. In 41 patients, parameters of adequacy were reevaluated, and the effects of parameter change over time on patient outcomes were analyzed. RESULTS: Seventy-two were male. Twenty-nine (23%) were diabetic. Mean age was 47+/-2.9 years, and the mean follow up period was 45+/-.8 months. The baseline serum albumin was 3.7+/-.5 g/dL. nPCR was 0.8+/-.2 g/kg/d, and %lean body mass was 68.4+/-4.3. Kt/V was 2.0+/-.7, and Ccr was 64.5+/-8.8 L/wk/1.73 m2. The estimated GFR was 1.1+/-.3 mL/min. The multivariated analysis demonstrated that diabetes, serum albumin, and the patent residual renal function were independent factors of the patient and technique survival. Membrane transport characteristic was a predictor of technique survival. Among patients for whom the parameters of adequacy were reevaluated, a more rapid decrease of serum albumin and a more rapid increase of membrane transport characteristics were associated with increased risk of either death or technique failure. CONCIUSION: Nutritional indices and presence of residual renal function have a significant impact on the clinical outcome. The decrease of serum albumin and the increase of membrane transport characteristic have negative impacts on patient and technique survival.

Long Term Effects of Dialysis Adequacy, Residual Renal Function, Nutritional Indices on CAPD Patient Outcome / 대한신장학회잡지

PURPOSE: Several factors including dialysis adequacy are now known to be related to clinical outcomes of CAPD patients. In our study, the long term effects of dialysis adequacy, residual renal function, nutritional indices, and peritoneal membrane transport characteristics on the outcomes of CAPD patients were evaluated. METHODS: Prevalent or incident 127 patients were enrolled from February 1998 to January 1999. Follow-up was terminated in January 2003. In 41 patients, parameters of adequacy were reevaluated, and the effects of parameter change over time on patient outcomes were analyzed. RESULTS: Seventy-two were male. Twenty-nine (23%) were diabetic. Mean age was 47+/-2.9 years, and the mean follow up period was 45+/-.8 months. The baseline serum albumin was 3.7+/-.5 g/dL. nPCR was 0.8+/-.2 g/kg/d, and %lean body mass was 68.4+/-4.3. Kt/V was 2.0+/-.7, and Ccr was 64.5+/-8.8 L/wk/1.73 m2. The estimated GFR was 1.1+/-.3 mL/min. The multivariated analysis demonstrated that diabetes, serum albumin, and the patent residual renal function were independent factors of the patient and technique survival. Membrane transport characteristic was a predictor of technique survival. Among patients for whom the parameters of adequacy were reevaluated, a more rapid decrease of serum albumin and a more rapid increase of membrane transport characteristics were associated with increased risk of either death or technique failure. CONCIUSION: Nutritional indices and presence of residual renal function have a significant impact on the clinical outcome. The decrease of serum albumin and the increase of membrane transport characteristic have negative impacts on patient and technique survival.

The Relationship between the Histological Findings and beta ig-h3 in IgA Nephropathy / 대한신장학회잡지

BACKGROUND: TGF-beta is involved in the pathogenesis of various kidney diseases characterized by glomerulosclerosis and tubulointerstitial fibrosis. It is reported that urinary TGF-beta reflects the grade of interstitial fibrosis in glomerular disease. Here, we evaluated the relationship between the histological findings and beta ig-h3 in IgA nephropathy. METHODS: In patients with IgA nephropathy, we measured blood pressure (BP), serum creatinine, 24-hour urinary protein excretion (UTp), creatinine clearance (Ccr), serum and urine beta ig-h3 levels, and urine TGF-beta levels at the time of renal biopsy. Histologic findings were semiquantitively scored according to the extent of glomerulosclerosis (GG), tubulointerstitial fibrosis (TIG) and hyaline arteriolosclerosis (HA) by the criteria suggested by To. Semiquantitive scoring of immunohistochemistry for beta ig-h3 was done. RESULTS: Mean BP 95.4+/-14.5 mmHg, serum creatinine 1.06+/-0.35 mg/dL, 24-hour UTp 1, 423+/-1, 439 mg/day, and Ccr was 97.84+/-59.73 mL/min. The number of patients that showed GG 3 were 5, GG 2 was 1, GG 1 were 12. And, the number of patients that showed TIG 3 were 2, TIG 2 were 5, TIG 1 were 11. HA was shown in 4 patients. beta ig-h3 immunostaining was observed in glomerular Bowman's capsules and basement membrane of proximal tubules. The degree of beta ig-h3 immunostaining was positively correlated with the degree of glomerulosclerosis (r=0.72, p<0.001), interstitial fibrosis (r=0.91, p<0.001), serum creatinine (r=0.592, p<0.05) and Ccr (r=-0.626, p<0.05), but not with 24-hour UTp. Serum and urine beta ig-h3 levels did not correlate with any of these parameters. CONCLUSION: Renal beta ig-h3 expression in patients with IgA nephropathy may be related to glomerulosclerosis and interstitial fibrosis. However, urinary beta ig-h3 levels did not represent the pathologic changes of IgA nephropathy. Long-term study to measure renal beta ig-h3 expression and urinary beta ig-h3 is required to elucidate the roles of beta ig-h3 in IgA nephropathy.