Gastrointestinal Stromal Tumor of the Rectum: An Analysis of Seven Cases

Purpose Gastrointestinal stromal tumors (GISTs) rarely originate in the rectum. We investigated the clinicopathologic characteristics of rectal GISTs. Methods We analyzed the medical records of seven patients who underwent surgery for GIST of the rectum between 1998 and 2003. Results There were two men and five women with a median age of 55 years (range, 41–72 years) at the time of diagnosis. The median follow-up period was 23 months (range, 7–75 months). The chief symptoms were hematochezia, constipation, and anal pain. All patients underwent curative resection; in the form of abdominoperineal resection in five patients, transanal excision in one, and Hartmann's operation with prostatectomy in one. The median tumor size was 6.6 cm (range, 1–12 cm). Four patients received adjuvant radiation therapy. Local recurrence developed in two patients; 54 months and 23 months after surgery, respectively. Conclusion The common symptoms of rectal GIST were the same as those of other rectal tumors. Curative surgical resection should be done, but further studies are necessary to investigate better adjuvant treatment strategies for patients with rectal GISTs     

Etiology and outcome of chronic renal failure in Indian children

A prospective analysis of all new pediatric cases of chronic renal failure (CRF) was performed at our hospital over a 1-year period. The diagnosis of CRF was based on serum creatinine >2 mg/dl with supportive clinical, laboratory, and radiological findings. There were a total of 48 patients with CRF with a median age of 13 years (range 10 days to 16 years). The causes of CRF included glomerulonephritis (37.5%), obstruction and interstitial (52%), hereditary (6.3%), and undetermined (4.2%). Patients were symptomatic for a mean of 33.2 months (range 10 days to 11 years) at presentation. Eight patients (16.7%) had acute reversible deterioration of renal function at presentation. This was due to accelerated hypertension in 2, infection in 3, volume depletion in 2, and nonsteroidal antiinflammatory drugs in 1 patient. At presentation, 22 (46%) children had mild to moderate renal failure and 26 (54%) had end-stage renal disease. Twenty-one children (43.7%) had associated illness at presentation. Mean follow-up was 22.9 weeks (range 2–126 weeks). At the end of the study period, 10 (21%) patients were on conservative treatment, 7 (14.6%) on maintenance dialysis, 8 (16.7%) patients had functioning allografts, 4 (8.3%) patients had died, and 19 (39.6%) opted against further therapy. We conclude that CRF in Indian children carries a poor prognosis due to late referral and the limited availability and high cost of renal replacement therapy. Received: 31 July 1998 / Revised: 7 December 1998 / Accepted: 13 December 1998     

Prevalence of Reduced Bone Mineral Density in Patients with Anti-neutrophil Cytoplasmic Antibody Associated Vasculitis and the Role of Immunosuppressive Therapy: A Cross-Sectional Study

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a relapsing-remitting disease, which is treated with corticosteroids (CS) in combination with cyclophosphamide. One of the major side-effects of this treatment is osteoporosis, which may result in the increased occurrence of fractures. In the present study we measured the prevalence of reduced bone mineral density (BMD) in a cross-sectional cohort of patients and correlated BMD findings with cumulative doses of CS and/or cyclophosphamide. BMD was measured by dual-energy X-ray absorptiometry (DXA) of the lumbar spine, radius and proximal femur between January 1998 and December 1999. Cumulative doses of CS and cyclophosphamide were calculated by chart review. Ninety-nine consecutive patients (48 men, 51 women) aged 55 ± 16 years (mean ± SD) were studied 50 months (median; range 0–400 months) after a diagnosis of ANCA-associated vasculitis had been made. Sixty-nine patients were treated with 10.7 g (median cumulative dose; range 0.4–67.2g) of CS, and 88 patients were treated with 34.1 g (median cumulative dose; range 0.8–324.3g) of cyclophosphamide. Fifty-seven percent of the patients had osteopenia (T-score: –1 to –2.5 SD), and 21% had osteoporosis (T-score: <−2.5 SD) at least at one site. Thirty-four of 37 (92%) postmenopausal women, 9 of 14 (64%) premenopausal women, and 34 of 48 (71%) men had either osteopenia or osteoporosis. The mean age- and sex-adjusted BMD (Z-score) of the proximal femur in men was found to be significantly lower than zero. Cumulative dose of CS therapy showed an inverse relation with Z-scores at the lumbar spine (p= 0.035) and proximal femur (p = 0.011). Cumulative dose of cyclophosphamide was not correlated with Z-scores. Osteopenia and osteoporosis are thus frequently observed in patients with ANCA-associated vasculities. However, only in men is the mean Z-score significantly lower than zero. Cumulative dose of CS therapy is significantly associated with bone loss at the spine and femur. Received: 26 March 2001 / Accepted: 1 August 2001     

Post graft development of short children treated with growth hormone before kidney graft

Sixteen prepubertal patients with chronic renal failure (CRF) were given daily recombinant human growth hormone (rhGH) treatment (1.2 IU/kg per week) for 2.6±1.6 years until kidney transplant. Therapy was then discontinued and the patients followed for a further 3.5±1.4 years. During treatment, mean height increased from –3.0±0.9 standard deviation score (SDS) to –1.9±1.4 SDS (P<0.001) at the time of transplantation, corresponding to a mean height gain of +1.2±0.9 SDS. After discontinuation of rhGH therapy, prepubertal children continued a partial catch-up growth with a height gain of +0.5±0.8 SDS for the follow-up period. Conversely, negative changes of height were observed in pubertal transplanted children: –0.5±0.4 SDS in patients grafted at early stages of puberty (P2–P3) and –0.15±0.9 SDS in patients grafted at late stages of puberty (P4–P5). These data confirmed the benefit of rhGH therapy in CRF patients. Nevertheless, only early initiation of rhGH treatment led some of these patients to their target height at transplantation, thus preserving their potential growth. Reinitiation of rhGH therapy after transplantation should be considered in order to complete catch-up growth to target height in prepubertal children. Received: 23 July 1998 / Revised: 8 December 1998 / Accepted: 13 December 1998     

Histologic and clinical outcome after laparoscopic Nissen fundoplication for gastroesophageal reflux disease and Barrett’s esophagus

Background The effectiveness of laparoscopic Nissen fundoplication for the regression of Barrett’s esophagus in gastroesophageal reflux disease remains controversial. The aim of this study, therefore, was to review endoscopic findings and clinical changes after laparoscopic Nissen fundoplication for gastroesophageal reflux disease, particularly for patients with Barrett’s esophagus. Methods From September 1995 through June 2004, 127 patients with gastroesophageal reflux disease underwent laparoscopic Nissen fundoplication. All the patients had clinical and endoscopic follow-up evaluation. We further analyzed the course of 37 consecutive patients with Barrett’s esophagus (29% of all laparoscopic fundoplications performed in our institution) using endoscopic surveillance with appropriate biopsies and histologic evaluation. The median follow-up period for all the patients after fundoplication was 34 months (range, 3–108 months). The median follow-up period for the patients with Barrett’s esophagus was 19 months (range, 3–76 months). Results During the 9-year period, 70 women (55 %) and 57 (45%) men were treated with laparoscopic Nissen fundoplication. The median age of these patients was 42 years (range, 7–81 years). The clinical results were considered excellent for 67 patients (53%), good for 51 patients (40%), fair for 7 patients (6%), and poor for 2 patients (1%). Endoscopic surveillance showed regression of the macroscopic columnar segment in 23 patients with Barrett’s esophagus (62%). Regression at a histopathologic level occurred for 15 patients (40%). The histopathology remained unchanged for 14 patients with Barrett’s esophagus (38%). Conclusion Laparoscopic Nissen fundoplication effectively controls intestinal metaplasia and clinical symptoms in the majority of patients with Barrett’s esophagus.     

Renal replacement therapies in pediatric multiorgan dysfunction syndrome

Both peritoneal dialysis (PD) and continuous hemodiafiltration (CHDF) techniques are used in children who develop acute renal failure as part of multiorgan dysfunction syndrome (MODS). An important goal of renal support in MODS is treatment and prevention of fluid overload. This report describes an experience with PD and CHDF in children with MODS and presents an analysis of fluid balance for each modality. Medical records of patients with MODS treated with PD/CHDF were reviewed. Fluid balance was studied only in patients with documented fluid overload treated with PD/CHDF for more than 24 h. Successful fluid control was defined as more fluid output than input over the course of PD/CHDF. CHDF was used in 37 patients, median age 47 months (range 0.2–284 months), for a mean of 110 h (range 4–733 h). PD was initiated in 25 patients, median age 4 months (range 0.1–156 months), for a mean of 145 h (range 7–992 h). Successful fluid control was achieved in 17 of 26 (65%) CHDF patients and in 5 of 14 (36%) PD patients (P<0.01, chi-squared). In conclusion, CHDF is more effective than PD in treating and preventing fluid overload in children with MODS. Received: 14 July 1998 / Revised: 13 January 1999 / Accepted: 13 January 1999     

Conservative treatment for chronic renal failure from birth: a 3-year follow-up study

Fifteen children with chronic renal failure from early infancy who did not require renal replacement therapy were followed for 3 years. Chronic renal failure was defined as a serum creatinine at or above 1 mg/dl for the entire 1st year of life. These patients were treated conservatively with diet and supplements of sodium bicarbonate and sodium chloride, calcium and vitamin D. Erythropoietin was given to 5 patients. Neither nasogastric nor gastrostomy tube feeding was used, and none of the patients received recombinant human growth hormone. We analyzed length, weight, and head circumference at 3, 12, 24, and 36 months of age. All three variables displayed a significant drop in the first 3 months, but remained stable for the whole observation period thereafter. At the age of 3 years, the patients’ mean values of length, weight, and head circumference standard deviation score were –1.96, –1.37, and –1.07, respectively. Height velocity during the 1st, 2nd, and 3rd year was 22.2, 10.9, and 7.6 cm per year, respectively. The first two figures and the cumulative height velocity are significantly better than those from a large cohort of chronic renal failure patients collected by the European Study Group for Nutritional Treatment of Chronic Renal Failure in Childhood; here the corresponding figures of height velocity were 12.3, 8.3, and 7.6 cm per year. Median serum calcium, phosphate, parathyroid hormone, and albumin levels remained within normal limits for the entire study period. Therapy-resistant hyperparathyroidism occurred in 1 patient and radiological signs of renal osteodystrophy in 4 patients. Kidney length, as measured by ultrasonography, showed almost no growth. Received: 17 December 1998 / Revised: 17 May 1999 / Accepted: 21 May 1999     

Conversion from cyclosporin (Neoral®) to tacrolimus (Prograf®) in renal allograft recipients with chronic graft nephropathy: results of an observational study

To evaluate the role of tacrolimus in the treatment of Chronic Graft Nephropathy (CGN), a pilot cross-sectional study was performed on 14 patients with deteriorating renal function and biopsy-proven CGN. Maintenance therapy was switched from cyclosporin to tacrolimus, and results of conversion on allograft function were assessed by estimated glomerular filtration rate (GFR) and clinical outcome. Minimum follow-up was 15 months. Two distinctive response patterns emerged: (i) continuing deterioration of renal function with no apparent benefit over the projected trend of GFR (nine patients), and (ii) unequivocal change in the GFR trend line equation with reduced rate of deterioration in one patient and sustained improvement of GFR in four patients (reversal of downward trend). Five out of 14 patients (36 %) benefited from replacing Neoral with Prograf. All five patients exceeded their estimated time of return to dialysis by a median of 41 weeks (range: 29–52) and their grafts continue to function. Received: 6 July 1998 Received after revision: 8 December 1998 Accepted: 18 December 1998     

Renal transplantation in children under 5 years of age

Between March 1987 and December 1997, 59 renal transplants [49 cadaveric, 10 live related (LRD)], were performed in 54 children aged 5 years and younger. Six children required a second transplant. The median (range) age of the recipients was 2.9 (1.4–5.0) years; mean weight was 12.6 kg (7.4–23) and donor age 11 (2–50) years. Immunosuppression was cyclosporin or FK506, prednisolone, and azathioprine. Antithymocyte globulin was given as induction therapy for second transplants. Patient survival was 98.3%; 1 patient died from upper gastrointestinal haemorrhage. Graft survival was 67.7% at 1 year, 57.4% at 5 years, and 45.2% at 10 years. No LRD graft was lost during 7 years of follow-up. Thrombosis was the main cause of graft loss (10 cases) followed by vascular rejection (2 cases). There was no significant difference in graft survival between recipients aged less than 2, 2–3, and 3–5 years. The height standard deviation score (±SD) improved from –2.1±1.3 at transplantation to –1.0±1.3 at 1 year, –1.1±1.5 at 5 years, and to –0.14±1.1 at 10 years. Received: 19 August 1998 / Revised: 18 November 1998 / Accepted: 18 November 1998     

The 1998 report of the Japanese National Registry data on pediatric end-stage renal disease patients

We carried out a nationwide survey on patients less than 20 years of age with pediatric chronic end-stage renal disease (ESRD) in Japan for the year 1998. There were 582 patients who had started on renal replacement therapy before 1998, and 105 patients who had been newly introduced to renal replacement therapy in that year. The prevalence rate of the ESRD patients already on treatment was 22 per million population (aged 0–19 years) in 1998. Older patients had a higher prevalence rate than younger ones. There were 345 patients on dialysis as of 1 January 1998, and 237 patients with transplants. The major diseases causing ESRD were renal hypoplasia/dysplasia and focal segmental glomerulosclerosis. Of the 237 patients (46.9%) who had received renal transplants before 1 January 1998, 262 patients (96%) received their transplants from living kidney donors. The incidence rate for the new ESRD patients was 4 per million population (aged 0–19 years) in 1998. Older patients had a slightly higher incidence rate than younger ones. Peritoneal dialysis was used more frequently than hemodialysis under 15 years (85%–95% and 39% respec-tively), especially in very young patients. The major diseases causing ESRD were the same as in the patients already on treatment. The transplant rate for the year 1998 was 10 per 100 dialysis patient-years (patients aged 0–19 years) with 9 living kidney donors. The death rate was 15.6 per 1,000 dialysis patient-years (patients aged 0–19 years); the major causes of death being cardiovascular diseases and infections. Received: 30 January 2001 / Revised: 3 January 2002 / Accepted: 4 January 2002     

A review of Takayasu’s arteritis in children in Gauteng, South Africa

We have reviewed 31 patients with Takayasu’s arteritis followed at two pediatric nephrology units in Gauteng, South Africa over a 15-year period. There were 25 black patients, 4 white, and 2 of mixed race. The mean age at diagnosis was 8.42±3.59 (range 2.4–14.5, median 8) years. The most common presenting sign was hypertension, followed by cardiac failure, bruits, and absent pulses. The Mantoux test was strongly positive in 27 patients (90%, control population 5%). Markers of activity included a raised erythrocyte sedimentation rate (23 patients) or Gallium single photon emission tomography (positive in 12 of 16 patients). Angiography revealed type II (abdominal aorta) and III (arch plus abdominal aorta) lesions to be most common (11 in each group). All patients received antituberculous therapy and most low-dose aspirin for its antithromboxane effect. Corticosteroids and further immunosuppression were used to control disease activity. We added total lymphoid irradiation (TLI) or cyclophosphamide. Twenty-six patients in all received further immunosuppression, with 13 patients in each group. Results were similar in the two groups, with similar pre- and post-therapy systolic blood pressures and creatinine clearances. Two patients in each group relapsed, 3 died in the TLI group and 2 in the cyclophosphamide group. Surgical intervention, usually in the quiescent phase, consisted mainly of renal autotransplantation. Because of the problems with TLI and 2 patients with papillary carcinoma of the thyroid with long-term follow-up, we no longer use TLI. We have shown that with active medical and surgical intervention the aggressive course of this disease in children can be modified. Received December 10, 1997; received in revised form March 13, 1998; accepted March 16, 1998     

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     

Human immunodeficiency virus-associated nephropathy (HIVAN) in Nigerian children

Human immunodeficiency virus-associated nephropathy (HIVAN) has rarely been reported in African children. In this single-center study, we analyzed ten children diagnosed with HIVAN from January 2000 to October 2006. There were eight boys and two girls, with a male:female ratio of 4:1. Their ages were from 5 months to 15 years (mean 6.8 ± 6.2 years), with a peak age of 5–9 years. The presenting complaints included generalized edema (60%) and hypertension (50%). All patients had proteinuria on urine dipstick, with four (40%) at nephrotic range (proteinuria ≥500 mg/dl). Nine (90%) patients were in renal failure, with elevated serum creatinine (6.3–24 mg/dl) and serum urea (70–120 mg/dl). Renal disease was the first manifestation of HIV infection in six patients, whereas the diagnosis was made on autopsy in three. The duration from HIV infection to development of HIVAN ranged from 5 months to 10 years. CD4⁺ cell count, done in only three patients due to financial constraints, was below 200/mm³. The kidneys were hyperechoic on abdominal ultrasound in all patients, and three (30%) showed grossly enlarged kidneys. Histology of renal tissues available by autopsy in three patients showed mainly collapsing focal segmental glomerulosclerosis. Treatments given were angiotensin-converting enzyme (ACE) inhibitors and highly active antiretroviral therapy (HAART) in four and two patients, respectively, and one patient underwent peritoneal dialysis. On outcome analysis, seven (70%) patients died, two were lost to follow-up, and one was alive on HAART therapy at the writing of this article. In conclusion, HIVAN occurs in Nigeria children, and the mortality is very high from uremia.     

Outcomes of Cocaine-Induced Gastric Perforations Repaired With an Omental Patch

Crack cocaine has been associated with acute gastric perforation. The appropriate surgical treatment and long-term outcomes remain unclear. A retrospective chart review of all gastroduodenal perforations associated with crack cocaine use was performed. Data abstracted included details of short- and long-term outcomes. Kaplan–Meier methods were used to evaluate surgical outcomes. Over the 14-year period ending December 2005, 16 cases of crack-induced gastric perforations were identified. Most (75%) were treated with an omental patch. The other patients underwent a formal antiulcer operation, including one vagotomy and pyloroplasty (V&P), one vagotomy and antrectomy, one subtotal gastrectomy, and one ulcer excision and V&P. All patients after antiulcer procedures were followed for a median of 63 months (range 27–120) with no recurrences. Follow-up data were available in 75% of the omental patch patients. Recurrence of disease was observed in 56% of these omental patch patients at a median of 20 months (range 11–39). Those without recurrence were followed for a median of 67 months (range 12–96). The recurrence rate was borderline lower in the antiulcer group (P = 0.072). Omental patch closure results in a recurrence rate over 50% compared with no recurrence for formal antiulcer procedures. Presented as a poster at the 48th annual meeting of The Society for Surgery of the Alimentary Tract. Washington, DC, May 19–23, 2007.     

The clinical features of anti-neutrophil cytoplasmic antibody-associated systemic vasculitis in Chinese children

Anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis is reported mainly in adults. Studies in children are limited. The current study retrospectively analyzed the clinical characteristics and pathology of ANCA-associated systemic vasculitis in children in our hospital during the past 7 years. Twenty-four pediatric patients were diagnosed as having ANCA-associated systemic vasculitis, including 19 patients with microscopic polyangiitis (MPA), one with Wegener’s granulomatosis (WG), three with propylthiouracil (PTU)-induced ANCA-positive vasculitis and one with anti-glomerular basement membrane (GBM) disease. Of patients with primary ANCA-associated systemic vasculitis (MPA and WG), with an average age of 10.8±2.8 (6–14) years, 18 patients (90%) were female and two (10%) were male. Nineteen patients (95%) were p-ANCA/MPO-ANCA positive and one (5%) was c-ANCA/PR3-ANCA positive. The interval between onset and diagnosis was 8.5±24.3 (0.2–108) months. The majority of the patients (85%) had multi-organ involvement. All patients had clinical evidence of renal involvement and presented with hematuria and proteinuria. Of 20 patients, 16 (80%) also had acute renal failure, and five patients were dialysis dependent. Nine patients underwent renal biopsy and were diagnosed with necrotizing and crescentic glomerulonephritis. However, six biopsies showed immune complex deposition. All patients received immunosuppressive therapy including prednisone and cyclophosphamide, and ten patients also received intravenous administration of methylprednisone pulse therapy according to their clinical situation and renal pathology. Sixteen patients achieved clinical remission, and four patients presented as treatment failure. Patients were followed up for 12.3±5.1 months (median 12 months; range 1 to 91 months). Ten patients maintained their clinical remission, and ten progressed to renal failure requiring dialysis. Our study showed that the clinical features and pathology of primary ANCA-associated systemic vasculitis in children were similar to those of adults, but there were a predominance of female patients and late diagnoses. We suggest that early recognition and prompt aggressive treatment might improve outcome.     

Long-term enteral nutrition in infants and young children with chronic renal failure

An inadequate nutritional intake is common in infants and young children with chronic and end-stage renal failure (CRF/ESRF), causing poor weight gain and growth retardation. In a programme of enteral feeding (EF), growth, nutritional intake and outcome for oral feeding were evaluated in 35 children with CRF/ESRF, mean (range) age 1.6 (0–4.9) years at start of EF for 30 (12–60) months. Twenty-nine had a glomerular filtration rate of 12.1 (6–26) ml/min per 1.73 m² and 6 were on peritoneal dialysis. Mean (SD) weight standard deviation scores (SDSs) in the 0 to 2-year age group (n=26) were –3.3 (1.0) 6 months before EF, –3.1 (1.3) at the start, –1.7 (1.4) at 1 year, (P=0.0003) and –1.4 (1.8) at 2 years, (P=0.0008). Height SDSs were –2.9 (0.7), –2.9 (1.2), –2.2 (1.2) (P=0.008) and –2.1 (1.3) (P=0.004). Weight SDSs in the 2 to 5-year age group (n=9) were –2.3 (1.2), –2.0 (1.1), –1.1 (1.3) (P=0.002) and –0.9 (1.0) (P=0.04). Height SDSs were –2.8 (0.6), –2.3 (0.7), –2.0 (0.7) and –2.0 (0.8). There was no change in energy intake as a percentage of the estimated average requirement, nor was this exceeded. Percentage energy from the EF in the 0 to 2 year age group remained unchanged despite an absolute increase in energy intake with age. Twenty-one have had renal transplants, of whom 86% eat and drink normally. Long-term EF prevents or reverses weight loss and growth retardation in children with CRF/ESRF, with the achievement of significant catch-up growth if started before age 2 years. Received: 27 July 1998 / Revised: 19 November 1998 / Accepted: 20 November 1998     

Long-term maintenance of therapeutic cyclosporine levels leads to optimal graft survival without evidence of chronic nephrotoxicity

Since the introduction of cylcosporine into clinical use, a major area of concern within the transplant community has been the fear of chronic nephrotoxicity. Although progressive renal damage does appear to occur in native kidneys of heart and liver transplant patients receiving cyclosporine, it has been our contention that its use is not a major cause of deterioration in renal allografts. We therefore undertook a study of 91 consecutive renal transplants performed over a three-year period with a minimum graft survival of 1 year and a follow-up of 7–9 years. Serial serum creatinine values, iothalamate clearances and cyclosporine levels were obtained at 3 months after transplantation and yearly thereafter. Biopsies were performed on all grafts that had failed as well as on the majority of patients with deteriorating renal function, and were interpreted by two nephropathologists. As measured by iothalamate clearances, 65 % of the patients in this series exhibited absolutely stable renal function despite the maintenance of cyclosporine levels of more than 200 ng/ml for 7–9 years. Since these stable patients did not reveal any decline in renal function, it therefore follows that they did not experience chronic cyclosporine nephrotoxicity. Furthermore, none of the patients with declining renal function or with failed grafts showed any evidence of nephrotoxicity on biopsy. Chronic cyclosporine nephrotoxicity may be a cause of declining function or graft loss with renal transplant recipients, but if so, it is exceedingly rare. Received: 17 March 1998 Received in revised form: 8 October 1998 Accepted: 18 December 1998     

Donor age and graft function

We evaluated survival and renal function of cadaveric donor grafts according to donor age. The median age of the pediatric donors was 7.0 (0.7 – 16) years in 46 patients [median age 11.8 years (range) 3 – 16.8 years]. The median age of the adult donors was 34.4 (19 – 54) years in 59 patients [median age 12.1 years (range) 7 – 17.3 years]. Thirty patients were treated with azathioprine and prednisolone and 75 with cyclosporine A and prednisolone. The glomerular filtration rate (GFR) and the effective renal plasma flow (ERPF) were determined by the clearances of ⁵¹chromium-EDTA and ¹²⁵iodine-hippurate 1 – 48 months after kidney transplantation. There was no difference in graft survival between pediatric and adult grafts. There were also no differences in GFR in patients receiving grafts from pediatric or adult donors; 2 – 3 months after transplantation the GFR in recipients of pediatric grafts was 62±20 ml/min per 1.73 m² compared with 61±21 in those receiving adult grafts. The ERPF in recipients of adult grafts was significantly higher in the 1st month after transplantation: 486±239 versus 362±158 ml/min per 1.73 m². From the 4th to the 6th month after transplantation this difference disappeared: the ERPF of grafts from pediatric donors was 279±131 ml/min per 1.73 m² compared with 273±123 ml/min per 1.73 m² in grafts from adult donors. Using the single-kidney GFR and ERPF on an age-matched group of probands with minor diseases as references, 2 – 3 months after transplant the mean GFR of grafts from pediatric donors increased to 118%±51%, whereas the GFR of adult donor grafts fell to 60%±22% over the same period. After 4 – 6 months the ERPF in pediatric grafts was 96%±55% compared with 50%±22% in adult grafts. We conclude that graft survival and function in children with either a pediatric or an adult graft may not differ because graft function adapts to the requirement of the recipient. Received December 6 1995; received in revised form March 19 1996; accepted March 22 1996     

Short-term outcomes of severe lupus nephritis in a cohort of predominantly African–American children

Renal involvement is one of the major determinants of the outcome in patients with systemic lupus erythematosus. Although African–American ethnicity has been suggested to be a poor prognostic factor in severe lupus nephritis in adult patients, information on outcomes of African–American children with this disease is still very limited. We retrospectively studied the patients diagnosed with severe lupus nephritis by renal biopsy at Le Bonheur Children’s Medical Center from January 1990 to December 2003. All patients were below the age of 18 years at the time of biopsy. Clinical features assessed included age, gender, race, estimated glomerular filtration rate (GFR), presence of hypertension, gross hematuria, degree of proteinuria, complement 3 and 4 levels, serum albumin, renal histology and dose of oral prednisone. Forty-four patients were studied: 82% were African–American and 89% were female. Mean age at biopsy was 14.2±3 years (median 15.0 years; range 4.7 years to 17.0 years). Renal biopsies were assessed according to the WHO classification. Twenty-seven percent, 43%, and 30% were in class III, IV and V, respectively. At presentation, 55% had hypertension and 23% had a history of macroscopic hematuria. The patients had varying degrees of proteinuria, including 18% with nephrotic syndrome. Eighteen percent had moderate renal insufficiency with estimated GFRs less than 50 ml/1.73m² body surface area per minute. All the patients were treated with corticosteroids. Sixty-eight percent also received cyclophosphamide and 20% received either mycophenolate mofetil (MMF) or azathioprine (AZA). Two patients developed end stage renal disease and required chronic dialysis within 12 months of biopsy. At the 12-month follow-up visit, 23% of patients had complete remission and 48% had partial remission. The mean estimated GFR had increased from 96.0 ml/1.73m² per minute to 124 ml/1.73m² per minute (P=0.03). Mean serum creatinine levels decreased from 1.62 mg/dl to 0.91 mg/dl (P=0.03). Complement 3 levels increased from 54.3 mg/dl to 90.3 mg/dl (P<0.01). Mean serum albumin levels also increased from 2.8 mg/dl to 3.6 mg/dl (P<0.01) and urine protein-to-creatinine ratio decreased from 5.8 to 1.0 (P<0.01). The average prednisone dose decreased from 0.96 mg/kg per day to 0.41 mg/kg per day (P=0.64). In our center, with predominantly African–American children, patients with lupus nephritis presented similarly to those in other studies with predominantly Caucasian patients, and short-term renal outcomes were not different.     

Renal involvement in polyarteritis nodosa: evaluation of 26 Turkish children

Renal involvement is common in childhood polyarteritis nodosa (PAN). We report a retrospective analysis of the presentation and clinical course of 26 patients with PAN and renal involvement. The mean age was 9.3 years (range 1–14 years) and there were 12 boys and 14 girls. Renal symptoms at presentation were as follows: 3 had isolated proteinuria, 9 had nephritic syndrome, 2 had nephritic and nephrotic components, and 10 had renal failure with one of the above features. Two patients with isolated hypertension were diagnosed by angiography and classified as classical PAN. Patients either received prednisone p.o. alone (n=9), or prednisone plus cyclophosphamide p.o. (n=11), or pulse steroids with prednisone p.o. and cyclophosphamide (n=2); 4 did not receive any treatment. Patients who were given cyclophosphamide had a significantly better outcome than those who did not. We suggest that oral cyclophosphamide therapy and corticosteroids are effective in the treatment of PAN. The overall 1-year and 5-year survival rates of the patients were 72.5% and 60%, respectively. In conclusion, renal disease is a serious manifestation of PAN necessitating prompt and aggressive treatment. Received: 23 January 1997 / Revised: 28 June 1999 / Accepted: 1 July 1999