Posterior urethral valves: prognostic factors

OBJECTIVE: To determine which variables besides bladder dysfunction can help to predict the outcome of renal function in boys with posterior urethral valves (PUV). PATIENTS AND METHODS: All 40 patients with PUV in this retrospective study were diagnosed and began treatment in our hospital within the first 3 months of life, and have had >or= 5 years of follow-up. At the time of diagnosis, 33 were in renal insufficiency (RI) and seven had normal renal function (RF). At the time of the study 16 were in chronic renal failure (CRF) and 24 had normal RF. We compared their RF (initial and during follow-up), vesico-ureteric reflux (VUR), urinary tract infection (UTI), proteinuria, hypertension, renal echogenicity, final patient age and initial management. RESULTS: The mean serum creatinine values before and after initial treatment were worse in boys who developed CRF than in those who did not (P = 0.08); the mean glomerular filtrate rate (GFR) at 1 year old was 52 mL/min/1.73 m2 in the former and 102 in the latter (P < 0.001). Proteinuria was present during the follow-up in 79% of patients in CRF and in only 17% of those with normal RF. All patients who developed CRF had echogenic renal changes while only 53% of the others had (P < 0.01). Other variables showed no statistically significant differences (VUR, UTI, hypertension and final patient age). Of 33 patients in RI at diagnosis, nine were treated by valve ablation and 24 by temporary pyelo-ureterostomy. The initial mean serum creatinine value was worse in the latter than in the former (20.8 vs 13.0 mg/L). However, at 1 year old the mean GFR was better in the latter than in the former (P < 0.05). These GFR differences persisted during the first years of life but had disappeared by the fifth. CONCLUSIONS: The most significant prognostic factor for the future development of CRF is the GFR at 1 year old. The onset of proteinuria during the follow-up is associated with a worse prognosis. Echogenic renal changes may help to identify those dysplastic kidneys that will develop RI. Neonatal boys in RI who underwent pyelo-ureterostomy had better RF during the first years of life than those who underwent valve ablation.     

Long-term prognosis of hemolytic uremic syndrome and effective renal plasma flow

The long-term prognosis of diarrhea-associated hemolytic uremic syndrome (D+ HUS) was evaluated in a cohort of 127 of 149 children who had survived the acute phase. Glomerular filtration rate (GFR) and effective renal plasma flow (ERPF) were estimated by serial ⁵¹Cr-EDTA and ¹²³iodine-hippurate clearances. All children had acute renal failure during the initial phase and 74% of patients were dialyzed. During the 1st year, mean GFR and ERPF increased continuously until a plateau was reached. In the 2nd year after the diagnosis of HUS, GFR was below 80 and ERPF below 515 ml/min per 1.73 m² in 16% and 47% of patients, respectively. At the end of a median follow-up of 5.0 (range 2.0–13.2) years, the proportion of children with renal sequelae such as proteinuria ≥300 mg/l, hypertension, or a GFR <80 ml/min per 1.73 m² was 23%. Anuria of more than 7 days’ duration and hypertension during the acute phase were statistically significant risk factors for an unfavorable outcome. A reduced ERPF in the 2nd year was found in 93% of patients with sequelae. Mean filtration fraction (SD) in these patients was 0.26 (±0.07) versus 0.19 (±0.05) in patients without sequelae (P<0.0001). These data suggest that loss of nephrons during the acute phase may implicate hyperfiltration in the residual functioning kidney mass leading to progressive renal disease. ERPF in the 2nd year after D+ HUS may serve as an excellent parameter to detect patients with a high risk of an unfavorable long-term outcome. Received: 17 September 1998 / Revised: 31 March 1999 / Accepted: 1 April 1999     

Duration of oliguria and anuria as predictors of chronic renal-related sequelae in post-diarrheal hemolytic uremic syndrome

Prior long-term retrospective studies have described renal sequelae in 25-50% of postdiarrheal hemolytic uremic syndrome (HUS) survivors, but the ability to predict the likelihood of chronic renal-related sequelae at the time of hospital discharge is limited. We surveyed 357 children in our HUS registry who survived an acute episode of post diarrheal HUS (D+HUS) and were without end-stage renal disease (ESRD) at the time of hospital discharge. Of the 357 patients surveyed, 159 had at least 1 year (mean 8.75 years) of follow-up. Of these, 90 individuals were identified as having had at least 1 day of oliguria, with 69 individuals having had at least 1 day of anuria. The incidences of renal-related sequelae [proteinuria, low glomerular filtration rate (GFR), and hypertension] were determined among experimental groups based on oliguria and anuria duration. One or more sequelae (e.g. proteinuria, low GFR, hypertension) was seen in 25 (36.2%) of those who had no recorded oliguria and 34 (37.8%) of those with no recorded anuria. The prevalence of chronic sequelae increased markedly in those with more than 5 days of anuria or 10 days of oliguria, with anuria being a better predictor than oliguria of most related sequelae. A particularly high incidence of hypertension was seen in patients with > 10 days of anuria (55.6%) in comparison with those with no anuria (8.9%) [odds ratio (OR) 12.8; 95% confidence interval (CI) 2.9-57.5]. Patients with > 10 days of anuria were also at substantially increased risk for low GFR and proteinuria (OR 35.2; 95% CI 5.1-240.5). These findings may help identify children who need periodic and extended follow-up after hospital discharge.     

Long-term follow-up of Czech children with D+ hemolytic-uremic syndrome

Fifty-seven children (f/m=31/26) who survived diarrhea (D) + hemolytic uremic syndrome (HUS) were evaluated. The examinations were performed 1–27 years (median 7 years) from the onset of the acute disease. Patients aged 2.3–27 years (median 10 years) were allocated to three groups: Recovery (R, complete recovery), Residual renal symptoms (RRS, hematuria and/or proteinuria and/or hypertension with glomerular filtration rate (GFR) >80 ml/min/1.73 m², or moderate renal insufficiency with slightly decreased GFR to 60–80 ml/min/1.73 m² with or without residual renal symptoms), and Chronic renal insufficiency/failure (CRI/F, dialysis, transplantation – GFR <60 ml/min/ 1.73 m²). Results from 18 patients who survived more than 10 years after HUS demonstrated a high prevalence of renal damage. Only 6/18 patients were in group R, 7/18 patients were in group RRS and 5/18 patients were in group CRI/F. An early onset of HUS (36 patients between 0 and 2 years) was associated with a better prognosis when compared with late onset (21 patients aged more than 2 years), P=0.009. Serology typing of Human leukocyte antigens (HLA) classes I and II in 64 patients revealed a significantly higher frequency of DR9 antigen (P=0.0037) and a lower frequency of DQ1 antigen (P=0.009) in D+HUS patients compared with healthy Czech blood donors. Conclusion: Our study demonstrates a high prevalence of late renal damage in Czech patients surviving after D+HUS. The HLA typing in our group revealed a significantly higher rate of HLA DR9 haplotypes in D+HUS patients. Received: 4 January 2001 / Revised: 9 October 2001 / Accepted: 2 January 2002     

Neuropathic bladder as a cause of chronic renal failure in children in developing countries

Neuropathic bladder is considered a threat to the kidneys if not managed appropriately. In this study, we report our experience with neuropathic bladder at King Abdulaziz University Hospital (KAUH) as a cause of chronic renal failure (CRF) in the pediatric age group. This retrospective study included all children diagnosed with neuropathic bladder who presented with moderate or severe CRF over a 4-year period from December 2000 to December 2004 [glomerular filtration rate (GFR) at presentation <50 ml/min per 1.73 m²]. Fifteen patients were diagnosed with neuropathic bladder; group A consisted of ten patients with spina bifida and one with sacral agenesis and group B consisted of four patients with nonneurogenic neurogenic bladders (NNNB). The mean age±SD at presentation was 6.2±3.8 years, GFR was 24.2±12.4 ml/min per 1.73 m², and creatinine was 289.9±253.2 μmol/l. There were no differences in the age at presentation to a pediatric nephrologist or the degree of renal failure at presentation between the two groups. Clean intermittent catheterization (CIC) was not started in all patients before presentation to KAUH, except in two children. Five children required dialysis as they were in end-stage renal failure (ESRF). All except one received peritoneal dialysis (PD). Their mean age at the start of dialysis was 10.8±1.7 years. Neuropathic bladder due to spina bifida or NNNB is an important cause of CRF in developing countries. There was a considerable delay in the diagnosis of NNNB and a significant delay in starting CIC in all neuropathic patients.     

Renal failure and dialysis therapy in children with hepatic failure in the perioperative period of orthotopic liver transplantation

Severe renal failure (GFR less than or equal to 20 ml/min/1.73 m2) complicated the clinical course in 27 of 146 children (18.5%) admitted for orthotopic liver transplantation (OLT). Hepatorenal syndrome (HRS) was the cause of renal failure in 12 of 15 patients in whom renal failure preceded OLT while acute tubular necrosis, pre-renal factors and cyclosporine nephrotoxicity were the major causes of renal failure post-OLT. Eight patients died from hemorrhage, infection or other complications of hepatic failure before OLT could be performed. Survival in the remaining 19 patients undergoing OLT was significantly lower compared to 114 patients with OLT and no renal failure (53% vs 81%, p less than 0.025). Dialysis therapy in 13 of the 27 patients with renal failure (10 hemodialysis and 3 peritoneal dialysis) was frequently complicated by severe gastrointestinal hemorrhage and hypotension, and directly contributed to the death of two patients prior to OLT. Among the 19 patients with renal failure who were actually transplanted, the survival rate was similar whether dialysis was used or not (5/10 vs 5/9) even though the mean GFR was significantly lower in dialyzed patients (p less than 0.05). However, although small patient numbers precluded meaningful statistical analysis, dialysis appeared to be beneficial for the subgroup of 12 patients with HRS, 4 of whom had complete recovery of renal function after successful OLT. We conclude that, renal failure is common in children with advanced liver failure; dialysis in such patients may increase morbidity and does not improve overall mortality; and dialysis support may improve survival in the subgroup of patients with HRS.     

The spectrum of chronic renal failure among Jordanian children

BACKGROUND: The causes of chronic renal failure (CRF) vary from one country to another. In this study we reviewed our experience with the different types of renal disorders leading to CRF in Jordanian children. METHODS: We investigated CRF in 202 Jordanian children (113 males and 89 females) who presented to the Jordan University Hospital, Amman, in the period from July 1988 to April 2001. The mean age at onset of CRF was 7.5 +/- 3.9 years. Patients were followed for 0.6-12.6 years (mean 6.3 years). RESULTS: The causes of CRF included urological abnormalities and malformations (42.1%), hereditary renal disorders (29.7%), glomerulonephritis (GN)(14.4%), renal hypo- or dysplasia (5%), hemolytic uremic syndrome (HUS) (4.5%), and idiopathic (4.5%). Forty-nine patients required renal replacement therapy, most of them with peritoneal dialysis. Nine patients have undergone renal transplantation. We estimated the prevalence of CRF in children in Jordan to be 51 per million population, and the incidence as 10.7 new cases per million-child population per year. CONCLUSION: The high rate of hereditary disorders in our series is attributed to the high prevalence of parental consanguinity in our community. There was a striking number of patients with non-neurogenic neurogenic bladder in our study. The relative incidence of GN leading to CRF in Jordan is lower than in Europe and North America. The relative incidence of the other causes of CRF in our series is similar to many other countries. The incidence and prevalence of CRF in children in Jordan is high compared to other countries.     

Differential effects of recombinant human growth hormone on glomerular filtration rate and renal plasma flow in chronic renal failure

In normal subjects recombinant human growth hormone (rhGH) increases glomerular filtration rate (GFR) and effective renal plasma flow (ERPF) through the action of insulin-like growth factor-I (IGF-I). We have measured clearance of inulin and para-aminohippuric acid in 18 children with chronic renal failure (CRF) during their 1st year of rhGH treatment to look at the immediate (first 3 h), short-term (1 week) and long-term (1 year) effects of treatment. On day 1 mean (range) age was 9.1 (4.9–13.9) years, GFR 19 (9–58) and ERPF 77 (34–271) ml/min per 1.73 m². During treatment height velocity increased from 4.5 (1.7–6.5) to 9.5 (4.8–12.7) cm/year (P<0.0001). Two children required dialysis after 0.75 years and 1 child was electively transplanted after 0.5 years. There were no other serious adverse events. GFR and ERPF were unchanged in the 3 h following rhGH. GFR remained constant on day 8, 22 (6–56) and after 1 year, 20 (9–59) ml/min per 1.73 m². ERPF increased to 96 (33–276) ml/min per 1.73 m² on day 8P=0.005), and remained elevated, but not significantly so, at 99 (24–428) ml/min per 1.73 m² at 1 year. Fasting IGF-I increased from 147 (46–315) ng/ml to 291 (61–673) by day 8P<0.003), and to 341 (101–786) ng/ml at 1 year. There was no correlation between the change in IGF-I and renal function. Blood pressure, albumin excretion and dietary protein intake were unchanged by treatment. The significance of increased ERPF after 1 week of rhGH in CRF is unclear, but long-term follow-up of renal function is indicated.     

Long-term outcomes of Shiga toxin hemolytic uremic syndrome

Shiga toxin-producing Escherichia coli (STEC) hemolytic uremic syndrome (HUS) is an important cause of acute kidney injury (AKI). The outcomes of STEC HUS have improved, and the acute mortality rate in children is 1–4 %. About 70 % of patients recover completely from the acute episode and the remainder have varying degrees of sequelae. Only a few retrospective studies have reviewed these patients over long periods. Methodological flaws include a lack of strict definitions, changing modes of treatment, ascertainment bias and loss of subjects to follow-up. The kidneys bear the brunt of the long-term damage: proteinuria (15–30 % of cases); hypertension (5–15 %); chronic kidney disease (CKD; 9–18 %); and end-stage kidney disease (ESKD; 3 %). A smaller number have extra-renal sequelae: colonic strictures, cholelithiasis, diabetes mellitus or brain injury. Most renal sequelae are minor abnormalities, such as treatable hypertension and/or variable proteinuria. Most of the patients who progress to ESKD do not recover normal renal function after the acute episode. Length of anuria (more than 10 days) and prolonged dialysis are the most important risk factors for a poor acute and long-term renal outcome. After the acute episode all patients must be followed for at least 5 years, and severely affected patients should be followed indefinitely if there is proteinuria, hypertension or a reduced glomerular filtration rate (GFR).     

The Role of Plasma Exchange in the Treatment of Severe Forms of Hemolytic-Uremic Syndrome in Childhood

Abstract: Therapeutic plasma exchange (PE) or plasma-pheresis has been used in recent years in the treatment of severe hemolytic uremic syndrome (HUS) in children. We analyzed the benefit of PE and peritoneal dialysis (PD) in 9 children, 6 boys and 3 girls, aged 1–10 years, from 1983–1993. All children came from different geographical regions, and all had the sporadic form of the illness. Three patients had the gastrointestinal form, 5 had respiratory prodromes while 1 child developed HUS during the course of varicella. Seven children were hypertensive, but only in 3 was hypertension persistent. The child with varicella had a transient complement decrease. Five children were treated with PE. In 4 children, fresh frozen plasma (FFP) was used as replacement fluid, and human albumin was used in 1 child. Four children were treated with PD and infusions of FFP. Rapid recovery of renal function was observed in 5 patients whereas in 2 oliguric children the recovery of renal function ensued within 1 and 2 months, respectively. Two children developed terminal renal failure (TRF) (in 1 child the treatment was very delayed, and in the other child HUS developed following varicella). Only 1 boy had relapses of the disease followed by impairment of renal function from which he gradually recovered. During the 3–10 year follow-up period, only the child with relapses was hypertensive while the others had normal clinical and laboratory parameters. We suggest that PE plays an important role in the early treatment of severe forms of HUS in children.     

Darbepoetin alfa (Aranesp) in children with chronic renal failure

BACKGROUND: Darbepoetin alfa use has been reported in 7 children with chronic renal failure (CRF). Our objective was to evaluate the efficacy and safety of darbepoetin and determine a therapeutic dose in a larger sample of children with CRF. METHODS: Twenty-six children with chronic renal insufficiency (CRI) GFR <30 mL/min/1.73 m(2), on peritoneal dialysis (PD) or hemodialysis (HD) entered a prospective, open-label study of darbepoetin. Seven ineligible children who underwent the same evaluation were analyzed retrospectively. The starting dose was 0.45 microg/kg/week. IRB/REB approval and informed consent were obtained. The primary outcome measure was hemoglobin (Hb) response within a target range of 10.0 to 12.5 g/dL between 8 and 12 and 20 and 28 weeks. RESULTS: Thirty-three children (15 CRI, 9 HD, 9 PD; aged 1-17 years) were enrolled in the study. Ten patients dropped out (3 before 12 weeks and 7 before 28 weeks), none due to darbepoetin. Mean Hbs were 11.8 and 11.4 between weeks 8 and 12 and 20 and 28, respectively; the proportion of patients with Hb values >10.0 g/dL was 97% and 91% in the same intervals. No effect of grouping patients into CRI, HD, or PD or prospective versus retrospective was observed. One of 13 serious adverse events (hypertension) was possibly related to darbepoetin; 8/14 children reported injection-site pain. At 12 and 28 weeks, respectively, 73% and 87% were receiving darbepoetin less than once weekly. CONCLUSION: A dose approximating 0.5 microg/kg/week of darbepoetin effectively treats anemia in children with chronic renal failure; for many, this may be proportionately increased and injected less than once weekly.     

What do we know about chronic renal failure in young adults? II. Adult outcome of pediatric renal disease

Congenital abnormalities of the kidney and urinary tract (CAKUT) account for more than half of all renal failure in children. For young adults with CAKUT two questions are paramount: what is the prognosis and what is the best management to improve outcome? The paediatric literature shows that prognostic factors are glomerular filtration rate (GFR) and the presence of proteinuria. We reviewed data from 101 young adult patients with either primary vesico-ureteric reflux and renal dysplasia or obstructive uropathy. Patients had an estimated GFR (eGFR) of ≤60 ml/min per 1.73 m² body surface area and had had at least 5 years of follow up (median 162 months). There was a strong correlation between the amount of proteinuria at the start and overall rate of decline. Angiotensin-converting enzyme inhibitors (ACEIs) slowed declining renal function at all levels of function, but this only had a significant effect on renal outcome when eGFR was >35 ml/min. The ACEI benefit increased with time. Rate of decline was slower than reported for other diseases and was only ?2.4 ml/min per year for those reaching the start of dialysis. Outcome is predictable by the level of residual renal function (GFR). Nevertheless, function remains stable while proteinuria is minimal. Short-term studies overestimate rates of deterioration.     

Risk factors for renal failure in children with non-glomerular nephropathies

The aim of the present study was to analyze the progression of chronic renal failure (CRF), the effects of modification of risk factors for disease progression, and to formulate a theoretical model of CRF progression in an unselected group of children with CRF. The study was a cross-sectional, retrospective analysis of 92 patients aged 9.2+/-5.8 years with CRF and low-level proteinuria [glomerular filtration rate (GFR) 43+/-18 ml/min per 1.73 m(2), proteinuria 0.57 g/day, range 0-3.9 g/day]. Inclusion criteria were an established diagnosis of CRF and completion of any surgical treatment. The etiology of CRF in the majority of patients was congenital uropathy. Sixty-nine patients observed for longer than 3 years were divided into two groups according to progression of CRF or improvement of GFR. Forty-three patients were on renoprotective therapy. Over a 3-year period GFR decreased in 39 children and improved in 30 children. There were no differences between the groups in the etiology of CRF. Patients with progression of CRF were older ( P<0.08), grew faster ( P<0.004), had higher blood pressure ( P<0.01), and were more often proteinuric ( P<0.03). Arterial hypertension in patients with progression of CRF was resistant to therapy and these patients needed more intensive treatment. Renoprotective therapy led to improvement of kidney function in 50% of patients, and resistance to renoprotective therapy was correlated with increased body mass and height. Patients who received renoprotective drugs showed stabilized kidney function ( P<0.007) and decreased proteinuria ( P<0.05) and blood pressure ( P<0.02), despite higher basal values. In patients on renoprotective therapy in whom CRF progressed despite treatment, proteinuria was persistent in contrast to patients with improvement ( P<0.02). The best model of CRF progression in the path diagram included systolic blood pressure and anthropometric parameters. In conclusion, in unselected patients with CRF of non-glomerular origin and nil-to-moderate proteinuria the main risk factors for CRF progression are rapid somatic growth, age, and blood pressure. Arterial hypertension and proteinuria, even of mild intensity, differ significantly between patients with progression of CRF and those with stable or improved renal function. Renoprotective therapy is related to significant slowing of CRF progression, but the risk factors for resistance to therapy include persistent proteinuria and somatic growth.     

A prospective cohort study of incident maintenance dialysis in children: an NAPRTC study

BACKGROUND: Prior studies of dialysis practices and outcomes have included children with varied duration of end-stage renal disease (ESRD). This study evaluated dialysis characteristics, complications, practices, and outcomes in an incident pediatric cohort. METHODS: The cohort was limited to 1992 subjects enrolled in the North American Pediatric Renal Transplant Cooperative Study registry, starting hemodialysis (HD) or peritoneal dialysis (PD) between 1992 and 1998, without prior dialysis or transplantation. RESULTS: At dialysis initiation, the median glomerular filtration rate (GFR; Schwartz formula) was 6 to 11 mL/min/1.73 m2, and 90th percentile was 14 to 25 mL/min/1.73 m2. GFR was not associated with age or race. PD was used in 97% of infants, 70 to 80% of children and 59% of adolescents. Blacks were significantly less likely to be started on PD than whites. Twenty percent of patients switched dialysis modality, largely due to infection, inadequate access or family choice. Younger children received HD almost exclusively through percutaneous catheters, while 57% of children more than six years old were dialyzed with fistula or graft after six months on HD. The prevalence of anemia (Hct <33%) still exceeded 40% after six months of dialysis. The median interval to transplantation was 1.4 years, and was significantly greater in non-white, young, and female patients. Mortality rates (deaths/1000 patient-years) varied with age, from 13.6 in infants to 2.2 in adolescents. CONCLUSION: These data demonstrate considerable variability in patient management across pediatric centers. Prospective studies are needed to determine the optimum adequacy of care among children on dialysis and to identify populations at risk.     

Important differentiation of factors that predict outcome in peritoneal dialysis patients with different degrees of residual renal function.

BACKGROUND: Residual renal function (RRF) is an important predictor of outcome in peritoneal dialysis (PD) patients. Whether results from survival studies in dialysis patients with RRF can also be extrapolated to anuric patients remains uncertain. In this observational study, we examined the characteristics of PD patients with a residual glomerular filtration rate (GFR) > or =1 ml/min per 1.73 m2 vs those with complete anuria and differentiated factors that predict outcome in the two groups of patients. METHODS: Two hundred and forty-six continuous ambulatory peritoneal dialysis (CAPD) patients (39% being completely anuric) were recruited from a single regional dialysis centre. Assessments of haemodynamic, echocardiographic, nutritional and biochemical parameters and indices of dialysis adequacy were done at study baseline and were related to outcomes. RESULTS: During the prospective follow-up of 30.8+/-13.8 (mean+/-SD) months, 28.0% of patients with residual GFR > or =1 ml/min per 1.73 m2 vs 50.5% of anuric patients had died (P = 0.005). The overall 2 year patient survival was 89.7 and 65.0% for patients with GFR > or =1 ml/min per 1.73 m2 and anuric patients, respectively (P = 0.0012). Compared with patients with GFR > or =1 ml/min per 1.73 m2, anuric patients were dialysed for longer (P<0.001), were more anaemic (P<0.005), and had higher calcium-phosphorus product (P<0.01), higher C-reactive protein (P<0.001), lower serum albumin (P<0.05), greater prevalence of malnutrition according to subjective global assessment (P<0.05) and more severe cardiac hypertrophy (P<0.001) at baseline. Using multivariable Cox regression analysis, serum albumin, left ventricular mass index and residual GFR were significant factors associated with mortality in patients with GFR > or =1 ml/min per 1.73 m2, while increasing age, atherosclerotic vascular disease and higher C-reactive protein were associated with greater mortality in anuric PD patients. CONCLUSIONS: Our study demonstrates more adverse cardiovascular, inflammatory, nutritional and metabolic profiles as well as higher mortality in anuric PD patients. Furthermore, factors associated with mortality are also not equivalent for PD patients with and without RRF, suggesting that patients with and without RRF are qualitatively different.     

Chronic kidney disease--a common and serious complication after intestinal transplantation

BACKGROUND: Chronic kidney disease after organ transplantation is a serious complication that negatively impacts on long-term patient survival. We describe long-term renal function after intestinal transplantation by serial measurements of glomerular filtration rates (GFR) with Chromium EDTA clearance. MATERIALS AND METHODS: Ten patients with at least 6 months survival form the basis of this report. Glomerular filtration rate measurements were performed at baseline, 3 months posttransplantation, and yearly thereafter. Median follow-up time for the cohort was 1.5 years (0.5-7.8 years). Tacrolimus (Prograf) was discontinued in four patients because of impaired renal function. These four patients were switched to sirolimus (Rapamune) at 11, 18, 24, and 40 months posttransplantation. RESULTS: Median baseline GFR was 67 (22-114) mL/min/1.73 m. In the adult patients, GFR 3 months posttransplantation had decreased to 50% of the baseline. At 1 year, median GFR in the adult patients was reduced by 72% (n=5). Two patients developed renal failure within the first year and required hemodialysis. One of the pediatric patients fully recovered her renal function, the second pediatric patient lost 20% of her baseline GFR at 6 months posttransplantation. Glomerular filtration rate calculated with the modified diet in renal disease formula consistently overestimated GFR by approximately 30% compared with measured GFR. CONCLUSION: Chronic kidney disease and renal failure are common after intestinal transplantation. These two factors significantly contribute to poor long-term survival rates. Measurements of GFR may help to identify those individuals at risk for developing chronic kidney disease to implement renal sparing strategies.     

The long-term prognosis of patients with focal segmental glomerulosclerosis.

Forty consecutive patients whose biopsies showed focal and segmental sclerosis were studied for 6 to 16 years to establish the long-term prognosis of this group of patients. By the end of a decade 21 were dead, on regular dialysis or transplanted; only one death was unrelated to renal failure. A further 8 patients had a GFR of less than 60 ml/min/1.73 m2. Only 11 patients had normal renal function, and of these eleven only four had no urinary abnormality. Actuarially calculated survival was 75% at 5 years, 50% at 10 years, and 38% at 15 years. There was no difference between the 28 adults and the 12 children in terms of evolution. Patients with a nephrotic syndrome at presentation had a poorer prognosis than those never nephrotic. Twenty nephrotic patients were treated with prednisone, and 14 of these with cyclophosphamide in addition. One patient responded with loss of proteinuria within two months to both drugs, and another lost proteinuria when treated with cyclophosphamide. Thirteen patients received allografted kidneys; a nephrotic syndrome recurred in three patients, with histological evidence of recurrent disease in two.     

The effect of triple therapy on rapidly progressive type of Henoch-Schönlein nephritis

Twelve patients with Henoch-Schönlein purpura, aged 6–14 years (mean 10.3 years), presenting with rapidly progressive glomerulonephritis (RPGN) were investigated prospectively. Analysis of the initial clinical features revealed: oedema (8 patients), hypertension (7 patients), gross haematuria (11 patients), oliguria (5 patients) and a decreased glomerular filtration rate (GFR) (<40 ml/min per 1.73 m², 8 patients). Renal biopsies were available in 9 patients and revealed focal necrotising and a fibroepithelial type of crescentic glomerulonephritis (with 60%–90% crescent formation). The remaining 3 patients fulfilled the clinical criteria of RPGN. Two patients who were in the acute stage required peritoneal dialysis for a period of 2 weeks. The treatment protocol in all patients consisted of intravenous pulse methylprednisolone (3 days), oral cyclophosphamide (2 months), oral dipyridamole (6 months) and oral prednisolone (3 months). At the end of triple therapy, GFR returned to normal in all but 1 patient. During a follow-up period of 9–39 months, 7 patients achieved complete remission, while 4 patients showed partial remission, 3 of whom had persistent proteinuria and haematuria and 1 microscopic haematuria only. One patient had persistent nephropathy with decreased GFR and macroscopic haematuria and nephrotic-range proteinuria. His renal biopsy, performed 30 months after the onset of the disease, showed chronic diffuse sclerosing glomerulonephritis and intratubular severe IgA deposition. Although our patient group was small, this type of intensive treatment appears to be effective; further studies are needed.     

Microalbuminuria three years after recovery from Escherichia coli O157 hemolytic uremic syndrome due to municipal water contamination

BACKGROUND: Knowledge of the long-term renal prognosis of diarrhea associated hemolytic uremic syndrome (HUS) is important for patient counseling and follow-up. However, estimates of long-term risk are highly variable, with previous studies not using a healthy control group. METHODS: A municipal water system in the small rural town of Walkerton, Ontario, became contaminated with Escherichia coli O157:H7 in 2000. A cohort of 19 children who recovered from HUS was randomly age- and sex-matched to 38 children with no symptoms at the time of the outbreak. Both groups had detailed renal function testing 3 years after the outbreak, including a random urine albumin to creatinine, glomerular filtration rate estimated by Schwartz formula, and automated and manual blood pressure measurements. RESULTS: There were no baseline differences between the groups with respect to age (mean 4.8 years, range 1 to 15), sex, or birth weight (mean 3.4 kg). In follow-up there were no differences between the groups in body surface area (mean 1.0 m(2)), or in the methods by which renal function was assessed. Compared to the group with no symptoms, patients with HUS demonstrated more microalbuminuria [32% vs. 5%, relative risk 4.8 (95% CI 1.1 to 22.0)], a nonsignificant trend toward lower GFR (124 vs. 134 mL/min per 1.73 m(2)), and no difference in blood pressure. CONCLUSION: Children may demonstrate microalbuminuria 3 years after recovering from HUS. Longer follow-up is needed to determine if this finding has clinical relevance and utility.     

Early prognostic factors of infants with chronic renal failure caused by renal dysplasia

Renal dysplasia (RD) is a common cause of chronic renal failure (CRF) in children. The evolution towards end-stage renal failure is unpredictable due to the paucity of early prognostic factors. In order to identify early prognostic clinical criteria, we have retrospectively analyzed renal function and growth in 11 infants with RD and CRF from birth up to 4 years of age. Children with obstructive RD were not included. Glomerular filtration rate (GFR) was estimated from Schwartz formula. In infants with a GFR below 15 ml/min per 1.73 m² at 6 months of age (group A, n=5), kidney function did not further improve; 4 reached end-stage renal failure between 8 months and 6 years of age. In contrast, infants with a GFR above 15 ml/min per 1.73 m² at 6 months of age (group B, n=6) experienced a significant improvement in renal function during follow-up, and none required renal replacement therapy. During the first 3 months of life all infants with RD and CRF developed severe growth retardation. Between 6 months and 4 years of age, children from group B grew significantly better than those from group A. In conclusion, our experience suggests that GFR, estimated from Schwartz formula at 6 months of age, is a useful prognostic factor in infants with RD and CRF. Infants with a GFR below 15 ml/min per 1.73 m² are at risk of severe growth delay and the need for early renal replacement therapy, whereas those with a GFR above 15 ml/min per 1.73 m² have a relatively favorable long-term prognosis. Received: 4 October 1999 / Revised: 26 October 2000 / Accepted: 26 October 2000