Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0022672 (acute tubular necrosis)
 2,175 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The purpose of this study was to characterize the spectrum of renal lesions associated with plasma cell dyscrasias from a population of patients who had renal disease identified by kidney biopsy. Thirty-six patients (2.6% of 1361 kidney specimens examined over 6 years) had evidence of monotypical light chain with or without concomitant heavy chain deposition. A variety of lesions was found, including (a) AL-amyloid and glomerular nonamyloid light chain deposition manifesting as nodular, membranoproliferative, mesangioproliferative, and "minimal-change" glomerulopathies; (b) fibrillary glomerulopathy; (c) tubulointerstitial lesions (cast nephropathy, acute tubular necrosis, and tubulointerstitial nephritis); and (d) vascular (arterioles and small and medium-sized arteries) lesions. AL-amyloid was the most common renal lesion (39%), nonamyloid deposition occurred second most commonly (33%), and cast nephropathy ("myeloma kidney") was third most frequent (14%). Clinical and laboratory manifestations of a plasma cell dyscrasia were frequently subtle. Immunoelectrophoresis of both serum and urine did not demonstrate a monotypical light chain or immunoglobulin in almost 35% of this population. Thus, the correct diagnosis was not considered in the majority of these patients before biopsy. Progressive deterioration of renal function was common with all of the lesions, except for proximal tubule injury, which tended to improve over the period of study. Renal biopsy with careful examination for monotypical light chain with or without associated heavy chain deposition using immunofluorescence or immunoelectron microscopy was crucial in identifying and characterizing the varied lesions associated with lymphoplasmacytic disorders.
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PMID:Spectrum of glomerular and tubulointerstitial renal lesions associated with monotypical immunoglobulin light chain deposition. 190 26

Renovascular disease in a solitary kidney is a difficult and challenging problem. In six patients, with a mean age of 62 years, revascularisation was undertaken. In four of them, one to three attempts at PTA were made before the operation but all were unsuccessful. The preoperative mean serum creatine (Cr) was 3.52 mg% (range 2.5-5.5). The stenoses were caused by atherosclerosis in five cases and fibromuscular dysplasia (FMD) in one. Two methods of revascularisation were used: aortorenal bypass with saphenous vein (three patients) or PTFE (two patients), and transaortic renal endarterectomy with a venous patch (one patient). One patient with an aortic aneurysm underwent simultaneous aortic repair with a PTFE graft. There was no mortality or major complications in our series and no patient suffered acute tubular necrosis (ATN) after surgery. The mean follow-up period was 51 months (15-84 months). Postoperative Cr and blood pressure improved in all patients. Four patients no longer required any medication and the other two only needed reduced doses of antihypertensive drugs. Based on our experience of patients with poor single kidney function, we do not recommend PTA in this specific group of patients and suggest an aggressive surgical approach, which can effectively improve solitary kidney function, thus eliminating further haemodialysis.
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PMID:Revascularisation for a poorly functioning solitary kidney. 191 7

This report describes a 7-year experience with acute peritoneal dialysis in 31 neonates and infants less than 60 days of age. There were 20 boys and 11 girls, ages 3 to 60 days. Tenckhoff catheters of modified length were placed in the newborn intensive care unit (ICU), pediatric ICU, or surgery suites, and hourly exchanges (20 cc/kg) were started immediately postoperatively. Diagnoses included congenital metabolic disorders (11), acute tubular necrosis (6), postcardiopulmonary bypass with renal failure (5), renal cortical necrosis (5), obstructive uropathy (2), renal agenesis (1), and bilateral renal dysplasia (1). Complications included: peritonitis (4), bowel perforation (1), exit site infection (3), leaking dialysate (4), catheter obstruction (2), inguinal hernias (3), umbilical hernia (1), and retroperitoneal hemorrhage (1). There were 19 deaths (61.3%) from 1 to 90 days postinsertion in this high risk group. The (1), and post liver transplant (1). Effective dialysis (lowering of blood urea nitrogen (BUN) or ammonia, correction of acidosis, decrease in fluid overload) was possible in all cases. Five of the 12 survivors remain on chronic dialysis awaiting renal transplantation. Peritoneal dialysis is effective in the newborn period in the management of metabolic disturbances as well as renal failure. Morbidity and mortality (61.3%) is related to the near-morbid condition of the baby at the time of insertion and the severity of the complex underlying diagnosis often associated with multiorgan failure.
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PMID:Peritoneal dialysis in the first 60 days of life. 229 35

To determine the effect of diabetes mellitus on gentamicin nephrotoxicity we treated male F344 rats with streptozotocin 22 mg/kg (DM rats). DM rats were compared to controls (C) and nondiabetic rats ingesting the osmotic diuretic isosorbide administered to simulate glycosuric diuresis (C/I). Base-line C/I renal function and histology did not differ from C. However, in DM rats base-line inulin clearance (CIN) was 20% lower, and renal cortical slice uptake of p-aminohippurate was reduced compared to C and C/I. DM rats also had foci of renal tubular epithelial dysplasia not seen in C or C/I. Gentamicin was administered at 40 mg/kg-day to C and C/I and 32 mg/kg-day to DM rats to adjust for base-line CIN. Acute tubular necrosis, associated with depression of CIN and renal cortical p-aminohippurate and N-methylnicotinamide uptake, developed in all three groups. There were no differences between C and C/I. However, the degree of acute tubular necrosis and dysfunction was less in DM rats than C and C/I. Renal cortical gentamicin accumulation was also slower in DM than either C or C/I, and changes in renal cortical gentamicin over time followed a different pattern in DM rats. These results indicate that 1) attenuation of gentamicin injury in DM rats may be related to reduced accumulation of gentamicin by the renal cortex, 2) this reduced accumulation may be due to subtle baseline tubular injury mediated by streptozotocin or the diabetic state, and 3) osmotic diuresis does not account for attenuation of renal injury in DM.
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PMID:Experimental gentamicin nephrotoxicity: effect of streptozotocin-induced diabetes. 315 84

Ex vivo renal artery surgery has been reported by several investigators and has extended the role of revascularization in the treatment of lesions previously managed by nephrectomy alone. Several techniques are available for use, and selection of the most appropriate method can be tailored to the specific anatomy being managed. Our total experience included 27 kidneys that have been managed by ex vivo renal artery reconstruction. Lesions managed in this manner include two kidneys with renal artery stenosis and renal tumors, one kidney with a congenital branch arteriovenous malformation, and 24 kidneys with branch occlusive or aneurysmal disease from fibromuscular dysplasia. Postoperative angiography was performed in 22 cases and defined successful revascularization without technical error in 20 cases. One operative death occurred as a result of myocardial infarction. One patient required reoperation to control bleeding, and two patients had temporary acute tubular necrosis during the postoperative period. Techniques employed included ex vivo repair with autotransplantation to the iliac system (six kidneys), mobilization and perfusion without transection of the renal vein (10 kidneys), and ex vivo perfusion and repair with replacement into the original renal fossa (11 kidneys). The authors believe this latter technique of reconstruction to be preferable to autotransplantation for the usual patient undergoing ex vivo repair of complex renovascular lesions.
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PMID:Ex vivo renal artery reconstructions: indications and techniques. 378 29

Scant information exists on the prognosis of infants with renal failure who receive peritoneal dialysis in the first month of life. We reviewed the outcome of 23 such patients 1 year after the onset of renal failure. Diagnoses included acute tubular necrosis (11 infants), renal dysplasia (5), obstructive uropathy (4), polycystic kidney disease (1), renal vein thrombosis (1), and renal artery thrombosis (1). Seven of the eleven patients with acute tubular necrosis had had cardiac surgery. At 1 year, eight (35%) of the patients had died, six (26%) had a full recovery, seven (30%) were receiving long-term dialysis awaiting a transplant, and two (9%) had chronic renal failure. Effective dialysis, characterized by the reversal of metabolic disturbances or attainment of fluid balance, was accomplished in all patients. The mean duration of dialysis was 4.5 months (range, 0.1 to 12 months). The most common complications of dialysis were peritonitis and catheter exit site infection. Despite the provision of supplemental calories via nasogastric tube, the majority of patients receiving long-term dialysis showed impaired growth and mild developmental abnormalities. Peritoneal dialysis is an effective means of renal replacement therapy in the neonatal period; however, the morbidity and mortality rate for this population remains high.
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PMID:Peritoneal dialysis in the neonatal period: outcome data. 844 49

We report two cases of non-cardiogenic pulmonary edema as a complication of basiliximab induction therapy in young pediatric renal transplant patients identified following a retrospective review of all pediatric renal transplant cases performed in the National Paediatric Transplant Centre, Childrens University Hospital, Temple Street, Dublin, Ireland. Twenty-eight renal transplantations, of which five were living-related (LRD) and 23 were from deceased donors (DD), were performed in 28 children between 2003 and 2006. In six cases, transplantations were pre-emptive. Immunosuppression was induced pre-operatively using a combination of basiliximab, tacrolimus and methylprednisolone in all patients. Basiliximab induction was initiated 2 h prior to surgery in all cases and, in 26 patients, basiliximab was re-administered on post-operative day 4. Two patients, one LRD and one DD, aged 6 and 11 years, respectively, developed acute non-cardiogenic pulmonary edema within 36 h of surgery. Renal dysplasia was identified as the primary etiological factor for renal failure in both cases. Both children required assisted ventilation for between 4 and 6 days. While both grafts had primary function, the DD transplant patient subsequently developed acute tubular necrosis and was eventually lost within 3 weeks due to thrombotic microangiopathy and severe acute antibody-mediated rejection despite adequate immunosuppression. Non-cardiogenic pulmonary edema is a potentially devastating post-operative complication of basiliximab induction therapy in young pediatric patients following renal transplantation. Early recognition and appropriate supportive therapy is vital for patient and, where possible, graft survival.
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PMID:Basiliximab induced non-cardiogenic pulmonary edema in two pediatric renal transplant recipients. 1964 61

Wolcott-Rallison syndrome is a rare disease presenting with insulin-dependent diabetes mellitus (DM) before 6 months old, skeletal dysplasia after 6 months old, and liver failure. Other manifestations are renal failure, microcephaly, epilepsy, central hypothyroidism, neutropenia, and dental and dermal problems. The cases were 2 patients from 2 different states of Iran (Khoozestan and Fars) who had developed DM before 6 months old. The first one was a 7-month-old infant who was healthy; in the genetic study (screening), autosomal recessive pattern and novel deletion in EIF2AK3 were reported; her sister had died at 5.5 years old due to diabetic ketoacidosis (DKA) that was associated with liver and renal failure. The second patient had developed DKA at 45 days old, which was associated with mild acute tubular necrosis and abnormal coagulation tests at onset clinical presentation, which were then resolved. He was treated with insulin, and at follow-up, the laboratory data are normal; in the genetic study, EIF2AK3 nonsense homozygous mutation was diagnosed. Genetic study of patients with insulin-dependent DM before 6 months old, especially those with DKA and associated with or without other disorders; attention to novel deletion of in EIF2AK3 gene; screening for skeletal dysplasia after 1 year old; and renal, liver, pancreatic, and thyroid function tests are recommended.
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PMID:Wolcott-Rallison Syndrome With Different Clinical Presentations and Genetic Patterns in 2 Infants. 3100 90