A syndrome of acute interstitial nephritis and anterior uveitis

A syndrome of acute interstitial nephritis (AIN) and anterior uveitis is described in two children and the literature is reviewed. These disorders appear to improve, in uncontrolled studies, with systemic and topical ophthalmic corticosteroid treatment. Although the renal and ocular prognoses appear good, it is important to recotnize that patients with AIN are at risk for uveitis and if present, consultation with an ophthalmologist is recommended.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47832/1/467_2004_Article_BF00852531.pd

Dialysis therapy for children with acute renal failure: survey results

We surveyed 123 pediatric nephrologists to investigate the current dialytic management of acute renal failure (ARF) in children. Data collected from 92 responding physicians revealed that hemodialysis (HD), peritoneal dialysis (PD), and continuous renal replacement therapy (CRRT) are currently used as the primary means of acute renal replacement therapy in a nearly equal percentage of centers. The preferential use of CRRT appears to be increasing, while PD usage is decreasing except for the youngest infants and those patients likely to develop end-stage renal disease (ESRD). Additional data correlating patient outcome to dialytic modality should be collected to compare the efficacy of the three techniques.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/42302/1/467-15-1-2-11_00150011.pd

Invited Review Recognition and management of angiotensin converting enzyme inhibitor fetopathy

Angiotensin converting enzyme (ACE) inhibitors are extensively used for the treatment of hypertension, to decrease proteinuria, and to mitigate hyperfiltration. These drugs now have been shown to be fetotoxic causing profound fetal hypotension, renal tubular dysplasia, anuria-oligohydramnios, growth restriction, hypocalvaria, and death when used in the second and third trimesters of pregnancy. We recommend that ACE inhibitors not be used in pregnancy. However, if a child is born with ACE inhibitor fetopathy, aggressive therapy with dialysis to remove the inhibitor may mitigate the profound hypotensive effects. Therapy will depend on the specific ACE inhibitor, and care recommendations cannot be generalized for the entire class of drugs as their protein binding and volume of distribution differ substantially.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47835/1/467_2005_Article_BF02254221.pd

Treatment of vancomycin overdose using high-efficiency dialysis membranes

Two children underwent acute hemodialysis using high-efficiency dialysis membranes for vancomycin intoxication (plasma levels 238 µg/ml and 182 µg/ml). During a 3-h treatment, plasma vancomycin removal was on average 60%, with a calculated vancomycin half-life ( t 1/2 ) of 2 h. This is in contrast to a recent report using charcoal hemoperfusion for vancomycin intoxication (plasma level of 137 µg/ml), which resulted in a 40% relative plasma clearance and a calculated vancomycin t 1/2 of 12.5 h for a 4-h treatment. The choice of optimal modality for clearing a toxin should take into account the availability of equipment, protein or lipid binding of the toxin, and inherent risks of charcoal hemofiltration (large extracorporeal circuit, reversible hypocalcemia, heat loss, reversible coagulation defects) versus risks of high-efficiency hemodialysis (large extracorporeal circuit).Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/42299/1/467-13-9-773_90130773.pd

Use of mycophenolate mofetil in steroid-dependent and -resistant nephrotic syndrome

Cyclosporin (Cs-A) is an effective treatment for difficult cases of nephrotic syndrome (NS), but its use can be complicated by renal toxicity and a high incidence of relapses after withdrawal. We reviewed the charts of 10 Cs-A-dependent patients and 4 patients with steroid-dependent nephrotic syndrome (SDNS) not previously treated with Cs-A therapy. All patients had persistent NS, even after prior treatment with oral cyclophosphamide. Of 10 patients treated with Cs-A, 4 had surveillance renal biopsies consistent with Cs-A toxicity, and 8 of 10 had interstitial fibrosis prior to mycophenolate mofetil (MMF). Patients were treated with MMF, at 1,200 mg/m 2 per day, in an attempt to allow weaning of Cs-A and/or steroid therapy, and reduce the frequency of relapses. Overall, a significant decrease in frequency of relapses was noted after initiation of MMF therapy. In addition, 5 patients were weaned off Cs-A by 1–2 years of follow-up. One patient was weaned off Cs-A and MMF, and remained in complete remission. However, the subgroup of patients with frequently relapsing SDNS not treated with Cs-A appeared to have a reduction in the number of relapses while on MMF that did not reach statistical significance. Two patients with intractable steroid-resistant NS continued to relapse repeatedly on MMF and Cs-A therapy. We conclude that in this small, single-center, uncontrolled experience, MMF therapy in patients with Cs-A-dependent NS appears to be effective in reducing Cs-A exposure. In addition, MMF appears to significantly decrease the frequency of relapses in this patient population. Further controlled studies are warranted to better define the potential efficacy and side effects of long-term MMF therapy in this setting.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47822/1/467_2003_Article_1175.pd

Quantitative morphometry of renal biopsies prior to cyclosporine in nephrotic syndrome

Use of cyclosporine (CsA) in the management of children with steroid-resistant (SRNS) and steroid-dependent (SDNS) nephrotic syndrome has become increasingly popular in recent years. Although most children receive a renal biopsy prior to initiation of CsA, the relationship between initial renal histology and the subsequent clinical response to CsA is not known. We analyzed the correlation between pre-CsA segmental and global glomerular scarring and interstitial fibrosis and the subsequent response to CsA in 23 children (5.6±1.0 years, Mean±SEM) with SDNS ( n =8) and SRNS ( n =15) treated with CsA for 24.2±3.8 months and followed for 28.0±4.1 months. Complete remission was obtained in 78% of patients within 67.6±16 days, while 18% had a partial response and 4% no response. Quantitative histological analysis revealed a trend toward partial rather than complete response with increasing segmental glomerular ( P =0.13), global glomerular ( P =0.05), and interstitial ( P =0.08) scarring, and among patients with minimal change nephrotic syndrome versus IgM nephropathy versus focal segmental glomerulosclerosis. Among complete responders, linear regression analyses revealed no correlation between time to response and pre-CsA glomerular or interstitial scarring. We conclude that increased glomerular or interstitial scarring on a pre-CsA renal biopsy tends to correlate with a partial, rather than complete, response to CsA in childhood nephrotic syndrome.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/42297/1/467-12-9-737_80120737.pd

Sonographic evaluation of renal artery aneurysm in childhood

We report a child presenting with renovascular hypertension and sonographic evidence of a renal artery aneurysm (RAA). The diagnosis of RAA was made sonographically by demonstrating vascular flow in an aneurysmal segment adjacent to but continuous with the right renal artery and externally compressing the inferior vena cava. Comparison of the sonographic studies and an abdominal angiogram illustrate the sensitivity of sonography in diagnosing this condition. We suggest that with renal doppler sonography, RAA may be diagnosed less invasively and possibly with greater frequency yet believe that the gold standard of angiography is necessary prior to surgical intervention.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/46697/1/247_2005_Article_BF02018637.pd

Medication errors and patient complications with continuous renal replacement therapy

Continuous renal replacement therapy (CRRT) is commonly used for renal support in the intensive care unit. While the risk of medication errors in the intensive care unit has been described, errors related specifically to CRRT are unknown. The purpose of this study is to characterize medication errors related to CRRT and compare medication errors that occur with manually compounded solutions versus commercially available solutions. We surveyed three separate internet-based, pediatric list serves that are commonly used for communications for programs utilizing CRRT. Data regarding CRRT practices and medication errors were recorded. Medication errors were graded for degree of severity and compared between programs using manually compounded dialysis solutions versus commercially available dialysis solutions. In a survey with 31 program responses, 18 reported medication errors. Two of the 18 were related to heparin compounding, while 16/18 were due to solution compounding errors. Half of the medication errors were classified as causing harm, two of which were fatal. All medication errors were reported by programs that manually compounded their dialysis solutions. Medication errors related to CRRT are associated with a high degree of severity, including death. Industry-based, commercially available solutions can decrease the occurrence of medication errors due to CRRT.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/45869/1/467_2006_Article_49.pd

Pediatric acute renal failure: outcome by modality and disease

Two hundred and twenty-six children who underwent renal replacement therapy (RRT) from 1992 to 1998 were retrospectively reviewed. The mean age, at the onset of RRT, was 74±11.7 months and weight was 25.3±9.7 kg. RRT therapies included hemofiltration (HF; n =106 children for an average of 8.7±2.3 days), hemodialysis (HD; n =61 children for an average of 9.5±1.7 days), and peritoneal dialysis (PD; n =59 children for an average of 9.6±2.1 days). Factors influencing patient survival included: (1) low blood pressure (BP) at onset of RRT (33% survival with low BP, vs 61% with normal BP, vs 100% with high BP; P <0.05), (2) use of pressors anytime during RRT (35% survival in those on pressors vs 89% survival in those not requiring pressors; P <0.01), (3) diagnosis (primary renal failure with a high likelihood of survival vs secondary renal failure; P <0.05), (4) RRT modality (40% survival with HF, vs 49% survival with PD, vs 81% survival with HD; P <0.01 HD vs PD or HF), and (5) pressor use was significantly higher in children on HF (74%) vs HD (33%) or PD (81%; P <0.05 HD vs HF or PD). In conclusion, pressor use has the greatest prediction of survival, rather than RRT modality. Patient survival in children with the need for RRT for ARF is similar to in adults and, as in adults, is best predicted by the underlying diagnosis and hemodynamic stability.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/42305/1/467-16-12-1067_10161067.pd

Continuous arterial-venous diahemofiltration and continuous veno-venous diahemofiltration in infants and children

Continuous arterial-venous diahemofiltration and continuous veno-venous diahemofiltration [CAVH(D)/CVVH(D)] in the infant and pediatric population is increasingly being utilized in the child needing renal replacement therapy (RRT). Difficulties with infant- and pediatric-specific equipment remains a limitation. The availability of techniques and equipment in this unique population is addressed. Use of this form of RRT as opposed to hemodialysis or peritoneal dialysis is discussed. The decision for CAVH(D) or CVVH(D) remains an individual choice.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47833/1/467_2004_Article_BF00868282.pd