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Peritonitis is a common complication of end stage renal failure (ESRF) patients receiving continuous ambulatory peritoneal dialysis (CAPD). Peritoneal macrophage may participate in the activation of specific T cells and in the generation of local cell-mediated immunity to various pathogens. The purpose of this study is to investigate the possible role of macrophage in CAPD patients with peritonitis.We evaluated the expression of Fas receptor (CD95), ICAM-1 (CD54), CD25, and CD69 by two-color flow cytometry on extravasted macrophages from 16 ESRF patients on CAPD with peritonitis (peritonitis-positive) and compared them to 11 ESRF patients on CAPD without peritonitis (peritonitis-negative) and normal controls.We found an increased expression of CD95, CD54, and CD25 on macrophage in peritonitis-positive group compared to controls (all p0.001). In the peritonitis-positive group, the CD95 expression was significantly higher than that of the peritonitis-negative group (p0.001). The expression of CD54, CD25, and CD69, however, was not significantly different between the peritonitis-positive and peritonitis-negative CAPD subgroups.We found an abnormally increased percentage of macrophage-expressing Fas receptor and ICAM-1, and the percentage of CD95+ macrophage, but not those of other markers, were increased among the subset of CAPD patients with peritonitis. The later finding suggests that this macrophage phenotype is associated with peritonitis occurring in CAPD.

The prognosis of uremia patients on continuous ambulatory peritoneal dialysis (CAPD) is related to frequent peritonitis rate. Frequent peritonitis will lead to peritoneum failure, making CAPD unfeasible. We have performed proteomic profiling of peritoneal dialysis effluent samples from a cross-section of CAPD patients with and without peritonitis in order to identify biomarkers of peritonitis. We performed 2D gel electrophoresis and surface-enhanced laser esorption/ionization time of flight mass spectrometry (SELDI-TOF MS) on peritoneal dialysis effluent from 16 subjects with peritonitis. A genetic algorithm search of principal component space revealed a group of a peak distinguishing peritonitis-positive subjects, with mass/charge (m/z) values of 11,117.4. Our analyses identified the peak at m/z 11,117.4 with an accuracy of 95% for classifying peritonitis. Mass spectrometric analysis of peritonitis PDE samples identified the 11,117.4 protein as beta2-microglobulin (B2M). Using an unbiased protein profiling approach, we have validated previously reported findings of B2M as a biomarker associated with CAPD peritonitis. Prospective studies are warranted to establish additional biomarkers that would be predictive of peritoneal dialysis peritonitis. Besides, extending the study to a larger number of patients with subgroup analyses may yield additional information of the peritoneal dialysate proteins in association with dialysis adequacy, residual renal function, nutritional status, and risk of peritoneal infection.

Methylmalonic academia (MMA) is a rare inborn error of branched-chain amino acid metabolism. Therapy consists of a special formulated protein diet, carnitine supplementation, and emergent detoxification during acute decompensation. Continuous hemodiafiltration is a modality choice to treat acute metabolic decompensation in inborn error of metabolism. We report the successful use of continuous hemodiafiltration in the management of acute decompensation in patients with methylmalonic academia. Three male patients were diagnosed with methylmalonic academia with the initial presentation of hyperammonemia. Continuous hemodiafiltration was utilized to treat acute decompensation of metabolic crisis. This approach results in a rapid reduction of systemic toxin levels. Continuous hemodiafiltration should be considered as a treatment modality for symptomatic neonates with MMA where hemodialysis is not feasible.

To evaluate the organ system failures hospital mortality predictions in critically ill patients with acute renal failure requiring dialysis.Prospective, cohort study.Intensive care units in a tertiary care university hospital in Taiwan.A total of 112 patients admitted to the intensive care units with acute renal failure who required dialysis from January 1999 through December 1999.Collection of information necessary to compute the number of failed organs.Of the 112 patients studied, 75 were men and 37 were women. The mean age of survivors and non-survivors was 58.59 +/- 19.91 years and 58.76 +/- 19.62 years. The overall mortality rate was 67%. There were no significant differences between survivors and non-survivors in terms of age, gender, or indication for dialysis. The cause of death in the majority of patients was related to organ system failure during the 24 hours immediately preceding the initiation of acute hemodialysis, and carry mortality rates exceeding 83% with the coexistence of four or more failed organs. The area under the organ system failures prediction model receiver operating characteristic curve equaled 0.772 +/- 0.046.We conclude that mortality rate for acute renal failure in intensive care unit patients continues to be high. Organ system failures prediction model performed well and simple in its ability to identify patients who die in hospital. Mortality rate increases as number of failed organ increases.

Acute hyperammonemia caused by urea cycle disorder is a medical emergency for which immediate managements should be taken to minimize permanent brain damage. Among different enzyme defects, ornithine transcarbamylase deficiency (OTC) is one of the most common enzyme defect in urea cycle disorders. We utilized continuous renal replacement therapy techniques in the acute treatment of hyperammonemia due to ornithine transcarbamylase deficiency.Three male neonates with elevated serum ammonia levels were shown, based on urine organic acid analysis and serum amino acid studies, to have OTC deficiency. Administration of sodium benzoate and sodium phenylacetate for activating alternative nitrogen waste pathway were used associated with protein restriction. Other modalities, including blood exchange transfusion, peritoneal dialysis, continuous renal replacement therapy were utilized in an attempt to lower serum ammonia concentration.We report the successful use of continuous arteriovenous hemofiltration (CAVH), continuous arteriovenous hemodialysis (CAVHD), continuous arteriovenous hemodiafiltration (CAVHDF) in the acute management of hyperammonemia due to OTC deficiency. We also compared the ammonia clearance between peritoneal dialysis, exchange transfusion, CAVH, CAVHD and CAVHDF. It demonstrated the evidence that CAVHDF provides the best ammonia clearance.Continuous renal replacement therapy including CAVH, CAVHD, and CAVHDF may be the alternative techniques for acute management of hyperammonemia in inborn error of metabolism when dialysis machine is not available. Our data suggests CAVHDF provides the best ammonia clearance.

Vancomycin-related nephrotoxicity typically manifests as acute tubulointerstitial nephritis. The drug does appear, however, to increase the nephrotoxicity of concurrently administered aminoglycosides. The extent of direct tubular toxicity attributable to vancomycin, especially in the absence of aminoglycoside treatment, does not appear to have been previously described. We report a case of biopsy-proven acute tubular necrosis secondary to vancomycin toxicity in a 13-year-old boy where there was no likely alternate explanation for toxic or ischemic injury. No hemodialysis was required, and the patient made a full recovery with subsequently regained renal function.

Essential mixed cryoglobulinemia (type II) has turned out to be secondary to hepatitis C virus (HCV) infection in the large majority of patients. Interferon might be anticipated to be effective only in HCV-associated cryoglobulinemias. We found that interferon was highly effective in an HCV-positive patient with true essential type II mixed cryoglobulinemia. The patient presented with symptomatic cryoglobulinemic vasculitis without underlying immunologic, infectious, or neoplastic diseases. Tests for HCV viremia, a reverse-transcriptase-polymerase-chain-reaction (RT-PCR) assay, and anti-HCV antibodies (third-generation assays) were positive before therapy. The patient had severe cryoglobulinemic vasculitis with purpura, peripheral neuropathy, and membranous proliferative glomerulonephritis. The cryocrit before therapy was 6 percent in the patient. Recombinant interferon alfa-2a (Roferon-A, Hoffmann-LaRoche, Basel, Switzerland) was administered at a dose of 3 million units per day for three months and 3 million units every other day for the subsequent nine months, a protocol adopted for HCV-associated cryoglobulinemia. The patient had a complete clinical response, with the disappearance of serum cryoglobulins and all signs of cutaneous vasculitis and with the normalization of kidney-function results and urinary values in the patient with nephropathy. The patient has remained in complete remission for more than one year since the withdrawal of therapy. True essential mixed cryoglobulinemia with HCV infection complicated with glomerulonephritis represents a therapeutic challenge.

Carcinosarcoma is a malignancy that occurs very rarely in the renal pelvis, and only a very limited number of cases has been documented. These tumors are composed of both malignant mesenchymal and epithelial elements. Unlike the cases described so far, this carcinosarcoma of the renal pelvis was characterized by quick recurrence, following complete surgical resection. Carcinosarcomas of the renal pelvis are known to be rapid in progression and associated with a poor prognosis. They usually metastasize soon after surgery, but very little information is available on the histologic types that characterize the recurrent tumors. We presented a histologically proven case of renal carcinosarcoma extending from renal pelvis. Convalescence was uneventful, and fifteen months after the operation, he is alive with no recurrence or metastasis. If a rapidly growing tumor is detected that seems to originate from the kidney with extension to ureter and bladder, carcinosarcoma should be included in the differential diagnosis.

Valproate-induced hyperammonemic encephalopathy is an unusual but serious complication that may occur in people with normal liver-associated enzyme levels, despite normal therapeutic doses and serum levels of valproate. Here, we describe an adolescent girl who had absence seizure and complained about progressive dizziness and general malaise several days after restarting valproate. Then, she presented vomiting and decreased consciousness three weeks after valproate use. Notably, her serum ammonia level was five times the upper limit of normal (184 micrommol/L), with normal liver-associated enzyme and supra-therapeutic valproate level. EEG showed continuous generalized slowing. The tandem mass analysis revealed carnitine deficiency. Consciousness improved after emergent hemodialysis. Ammonia level and EEG also returned to normal. Possible mechanisms, risk factors and the treatments of valproate-induced hyperammonemic encephalopathy are described. Physicians should consider this possibility when consciousness disturbance occurs in patients treated with valproate.

We report a case of primary Sjogren's syndrome (SS) with cutaneous leukocytoclastic vasculitis and IgA nephropathy. The accurate diagnosis of SS was established based on objective signs and symptoms of ocular and oral dryness, a characteristic appearance of a biopsy sample from a minor salivary gland, and the presence of anti-SS-A autoantibody. A second autoimmune disorder was not present, so the diagnosis of primary SS was established. A histologic finding of skin biopsy of purpuric lesion was typical for leukocytoclastic vasculitis. Renal biopsy was performed for nephrotic range proteinuria. The pathologic finding of renal biopsy was IgA glomerulonephritis with crescent formation. The patient was treated with small doses of glucocorticoids and maintenance hemodialysis. Leukocytoclastic vasculitis is one of the most characteristic extraglandular manifestations of SS. However, IgA nephropathy associated with SS and leukocytoclastic vasculitis is a rare finding. SS patients with glomerulonephritis present a more diverse outcome, even requiring hemodialysis. Therefore, renal biopsy is warranted in SS with glomerulonephritis and systemic vasculitis.

Background Hemodialysis dependence is an independent risk factor for hematogenous complication, including distant metastatic infection and osteomyelitis. Chronic osteomyelitis is a serious disease that fails to respond to aggressive medical and surgical treatment. Hyperbaric oxygen therapy has been proved to enhance bone and soft tissue healing in many studies. This article presents the preliminary result of hyperbaric oxygen therapy in hemodialysis-dependent patients with chronic osteomyelitis. Materials and methods Ten hemodialysis dependent patients who were diagnosed with chronic diffuse osteomyelitis were treated prospectively with adjunctive hyperbaric oxygen therapy, in addition to aggressive surgical debridement and antibiotic treatment. Results The hyperbaric oxygen therapy averaged 20 daily sessions. Successful treatment was achieved in eight patients (80%). The mean length of treatment was 21 days. The preliminary results are comparable with other series. Conclusion Hyperbaric oxygen is effective as an adjunct to aggressive medical and surgical treatment in chronic refractory osteomyelitis among hemodialysis-dependent patients.

Background: Hyperammonemia caused by ornithine transcarbamylase (OTC) deficiency can be properly managed by continuous arteriovenous hemodiafiltration (CAVHDF).) Removal of amino acids (AA) during CAVHDF has not been thoroughly investigated. AA losses in patients with urea cycle defects due to ornithine transcarbamylase deficiency treated by CAVHDF were analyzed. Methods: Twelve neonates with elevated serum ammonia levels, proven through urine organic acid analysis and serum amino acid studies, were documented to have OTC deficiency. CAVHDF was administered in an attempt to lower serum ammonia concentration. Amino acid analysis of ultrafiltration and serum were performed by liquid chromography. Results: Serum levels of leucine, isoleucine, methionine, phenylalanine and tyrosine were significantly lower in the patients than acceptable levels. Glutamine was the only amino acid that increased significantly, which is clinically relevant to OTC enzyme deficiency. Although the mean serum concentrations of tyrosine and glutamine concentrations were lower in dialysate, the serum and dialysate concentrations of other amino acids did not differ. Conclusion: CAVHDF may induce changes in amino acid metabolism and distribution as well. The requirement of aminogram monitor for amino acid supplementation in urea cycle defect patients is important.

Continuous hemofiltration has been used with increasing frequency for treating volume overload and acute renal failure in critically ill, hemodynamically unstable pediatric patients. This retrospective report investigates continuous hemofiltration in pediatric patients, and their survival rate. Sixty children treated between 1999 and 2001 with a diagnosis of acute renal failure and requiring continuous hemofiltration were admitted to this study to determine if pediatric risk of mortality III (PRISM III) scores were an accurate prediction of mortality. PRISM III scores were calculated on the day continuous hemofiltration commenced; mean PRISM III scores of non-survivors were significantly higher than mean scores of survivors. PRISM III scores may be a useful indicator of outcome in children receiving continuous hemofiltration.

Acute physiology, age, chronic health evaluation II and III (APACHE II and III) scoring systems obtained on the day of the initiation of dialysis were compared the mortality rate among in critically ill patients with acute renal failure requiring dialysis.Retrospective study.Intensive care units in a tertiary care university hospital in Taiwan.100 patients diagnosed with acute renal failure and requiring dialysis were admitted to intensive care units from January 1997 through December 1998.Information deemed necessary to compute the APACHE II and APACHE III score on the day of dialysis initiation was collected.The overall hospital mortality rate was 71%. The relationship between APACHE II and APACHE III scores for patients was linear and correlated significantly in all subgroups. Goodness-of-fit was good for APACHE II and APACHE III models. Both reported good areas under receiver operating characteristic curve. Death in most patients was related to a higher APACHE II or APACHE III score during the 24 h immediately preceding the initiation of acute hemodialysis. Our results indicated a significant rise in mortality rates associated with higher APACHE II or III scores among all patients. Although less than 60%, the mortality rates markedly increased extent when APACHE II score of 24 or higher or APACHE III score above 90 had mortality rates exceeding 85%.Both predictive models demonstrated a similar degree of overall goodness-of-fit. Although APACHE II showed better calibration, APACHE III was better in terms of discrimination. The prediction accuracy of the APACHE II score for extremely high-risk patients is further enhanced by specific utility of APACHE III scoring as a second prediction model when the AII score is 24 or higher.