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Renal infiltration in children with acute leukemia has been reported previously; however, it has rarely been described in association with atypical hemolytic uremic syndrome (aHUS). We present a case of 9-year-old boy who developed life-threatening aHUS in the 1st week of Burkitt leukemia/lymphoma diagnosis with renal infiltration. Complete resolution of aHUS was achieved after therapeutic plasma exchange. This is an uncommon complication of Burkitt leukemia/lymphoma in a pediatric case.

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BACKGROUND/AIM: The aim of this retrospective study was to evaluate the presentation, clinical and pathological manifestations and outcome of the Henoch-Schönlein purpura (HSP) nephritis in children. METHODS: Clinical and laboratory data of 443 children with HSP nephritis aged between 3 and 16 years from 16 pediatric nephrology reference centers were analyzed retrospectively. The biopsy findings were graded according to the classification developed by the International Study of Kidney Disease in Children (ISKDC). RESULTS: Renal biopsy was performed in 179 of the patients with HSP nephritis. The most common presenting clinical finding in patients who were biopsied was nephrotic range proteinuria (25%) which was followed by nephritic-nephrotic syndrome (23.5%). The biopsy findings according to the ISKDC were as follows: class I: 8.3%; II: 44.1%; III: 36.3%; IV: 6.7%; V: 3.3%; VI: 1.1%. All of the patients who developed end-stage renal disease had nephritic-nephrotic syndrome at presentation. Of 443 patients, 87.2% had a favorable outcome and 12.8% had an unfavorable outcome. The overall percentage of children who developed end-stage renal disease at follow-up was 1.1%. Logistic regression analysis did not show any association of initial symptoms and histology with outcome. CONCLUSION: In the presented cohort, the presence of crescents in the first biopsy or presenting clinical findings did not seem to predict the outcome of HSP nephritis in children. We conclude that children with HSP nephritis even with isolated microscopic hematuria and/or mild proteinuria should be followed closely.

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Systemic donor infections especially with gram-negative organisms are regarded as an absolute contraindication to cadaveric organ donation for transplantation. This is largely due to fear of transmitting the pathogenic organisms to the immunosuppressed recipient. However, due to the current shortage of organs available for transplantation, clinicians are faced with the option to use organs from infected donors. Between 1996 to January 2006, we collected 44 solid organs. Two out of nine donors had microorganisms from blood cultured. Case 1 was of 23-year old woman whose cause of brain death was intracerebral bleeding due to a traffic accident. The donor had stayed 9 days in the intensive care unit prior to brain death. Two kidneys, two livers (split), and or heart were used. Klebsiella was the organism on blood culture. Case 2 was of 35-year-old man; cause of brain death was cerebral hematoma due to traffic accident. The donor had stayed 6 days prior to brain death onset. The liver and two kidneys were used. Acinetobacter baumannii was yielded upon blood culture. All donors were treated with appropriate antibiotics for at least 48 hours prior to organ procurement with consequent negative blood cultures, while the recipients received the same culture-specific antibiotics for 10 days following transplantation. One donor (case 1) heart and both donor corneas were not used due to infection. All patients are alive with excellent graft function at a median of 90 days following transplantation. In conclusion, our results suggested that bacteremic donors with severe sepsis under proper treatment can be considered for transplantation.

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Posttransplant renal dysfunction episodes can result from a variety of causes, including polyomavirus (BK virus)-associated nephropathy (PVAN). It is a well-recognized entity with a high incidence of graft failure. The delicate balance of viral infection and immune regulation in the transplant population would allow development of successful long-term strategies. In this presentation, we have described two PVAN cases of our institution and reviewed the literature.

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Acute rejection (AR) is a major determinant of chronic allograft dysfunction and graft survival. This study evaluated the effect of basiliximab on AR in pediatric renal transplantation on triple immunosuppression. Forty-three transplantations (25 males and 18 females; mean age 14.9 +/- 3.6 years) were performed between 1996 and 2002. Thirteen of the grafts came from cadaveric donors and 30 from living-related donors. All patients were placed on immunosuppression with prednisolone + (azathioprine or mycophenolate mofetil) + (cyclosporine [CYA] or tacrolimus). Basiliximab was also administered in 20 cases. The respective rates of biopsy-proven AR in the basiliximab group (BG) and the standard-regimen group (N-BG) were 0% vs 17.4% (P >.05) at 1 month posttransplantation; 0% vs 26.1% (P <.05) at 3 months; 0% vs 26.1% (P <.05) at 6 months, and 7.1% vs 26.1% (P >.05) at 12 months. In the N-BG group the 1- and 3-year graft survival rates were 91.3% (21/23) and 83.3% (15/18), respectively. The mean glomerular filtration rate (GFR) in the first year after the transplantation was 75 +/- 33 mL/min/1.73 m(2) in the N-BG and 98 +/- 21 mL/min/1.73 m(2) in the BG patients (P <.05). Basiliximab significantly reduced the rates of acute rejection at 3 and 6 months after pediatric renal transplantation. The GFR in the first year was significantly higher among the patients treated with basiliximab, which was well tolerated by all patients and caused no significant adverse effects. The effect of basiliximab on long-term graft survival and chronic allograft dysfunction deserves further investigation.

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Gastric emptying time (GET) appears to be a rate-limiting factor in the absorption of cyclosporine-A (CsA) and may be responsible for intra- and interpatient variability of CsA bioavailability. Few studies have assessed gastric motility after renal transplantation. The purpose of this study was to evaluate gastric emptying of semi-solid material in stable renal transplant patients with reference to blood CsA levels. The GET of semi-solids (GET t(1/2), half emptying time) was measured in 16 transplant recipients who were taking CsA (Neoral), prednisolone and azathioprine (or mycophenolate mofetil). The GET (t(1/2)) measured by radionuclide methods, was analyzed with reference to the daily CsA doses, levels of CsA (C(0)), and serum creatinine concentrations. The mean GET (t(1/2)) was 89.1 +/- 26.4 minutes. Twelve patients exhibited delayed gastric emptying with a mean CsA level of 171.8 +/- 56 ng/mL and a mean dose of 4.1 +/- 1.1 mg/kg/d. The GET (t(1/2)) was not significantly correlated with the serum creatinine levels, the time since transplantation, or the CsA concentration. In addition, the correlation between the mean daily CsA dose and the GET (t(1/2)) was only weakly positive, (r =.33, P =.2) and therefore, statistically insignificant. In conclusion, it could not be ascertained whether a higher dose of CsA delays gastric emptying or whether patients with delayed emptying require higher doses of CsA. However, it is believed that determining the GET after transplantation helps in the adjustment of immunosuppressant doses.

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A 12-year-old female patient with end-stage renal failure whose primary disease was reflux nephropathy, was first admitted for augmentation cystoplasty by using an ileum segment because of contracted urinary bladder. Four months later, she had a renal transplantation from her father on March 28th 1997. The first three days after the operation were uneventful. On the fourth day, she presented a severe rejection episode and was treated with steroid and ATG. A urinary fistula developed and she underwent surgery again on the 14th postoperative day. At surgery, apical resection + omentoplasty + nephrostomy + DJ replacement were performed. The postoperative period after the second operation was full of problems for both the patient and the transplantation team. She was discharged from hospital on the 40th postoperative day with excellent renal function (a serum creatinine level of 1 mg/dl) and with full recovery.

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The pathogenesis of Henoch-Schonlein Purpura (HSP) is still controversial. The aim of our study was to investigate the role of oxidative stress and cyclooxygenase (CO) pathway products in the pathogenesis of HSP. In order to investigate this, malondialdehyde (MDA) levels, indicating lipid peroxidation, prostaglandin E (PGE)-like activity as inflammatory mediator and vitamin E (vit-E) levels indicating anti-oxidant status were studied in a group of 10 children with HSP (five girls and five boys, aged 6-21 years, mean 10.7 years), both in the acute and recovery phase of the disease and in five age and sex-matched healthy children as a control group. The patients were also grouped into low and high clinical score groups. Plasma levels of MDA and PGE-like activity were significantly elevated in the active phase of HSP compared to the recovery phase. Vit-E levels were significantly reduced in the active phase compared to the recovery phase. The plasma levels of PGE-like activity of the patients obtained in the active phase were significantly higher than the levels of the control group, whereas the levels of the recovery phase were significantly lower than in the control group. No such difference between the controls and MDA and vit-E levels in the patient group was shown. No correlation between the clinical scores and the parameters studied could be found. Our findings indicate that oxidant stress and CO pathway products may play a role in the pathogenesis of HSP.

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As urinary prostaglandin excretion might be involved in idiopathic hypercalciuria, we studied the excretion of prostaglandin E2 and calcium together with serum prostaglandin E2, parathormone and 1,25 dihydroxyvitamin D in 20 patients and 11 controls Idiopathic hypercalciuric patients showed increased levels of urinary prostaglandin E2-like activity, which is correlated with calcium excretion. Although no change was observed in serum parathormone level in control and hypercalciuric patients, there was a positive correlation between serum 1,25 dihydroxyvitamin D and prostaglandin E2-like activity. These findings suggest that prostaglandin E2 could play a role in the hypercalciuria syndrome, possibly by increasing 1,25 dihydroxyvitamin D synthesis.

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Adhesion molecules play an important part in leucocyte transendothelial migration and thus may provide a useful marker of surface expression at inflammatory sites. In 20 patients with Henoch-Schönlein purpura serum intercellular adhesion molecule 1 (ICAM-1), E-selectin, and plasma von Willebrand factor (vWF) were determined by ELISA during the active and inactive phase of the disease. Twelve healthy children were studied as a control group. Serum ICAM-1 concentrations increased during the active phase of the disease and differed significantly compared with the inactive phase (p < 0.05). However ICAM-1 in the active phase did not differ significantly compared with controls (p = 0.08). Serum E-selectin concentrations did not differ in the active and inactive phase of the disease. By contrast, vWF increased in the active phase of the disease and differed significantly compared with inactive disease and control groups (p < 0.01). Considering the adhesion molecules and vWF, only vWF correlated well with the C reactive protein measurement in the active phase, which is considered a good marker of disease activity. These data suggest that plasma vWF is a good marker of vascular inflammation and endothelial damage. Circulating ICAM-1 might be an additional parameter in some of the patients.

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Serum levels of carboxyterminal propeptide of type I procollagen (PICP) and aminoterminal propeptide of type III procollagen (PIIINP) can be used as markers of bone formation and the evaluation of children with growth disorders. We measured the serum levels of these collagens by radioimmunoassay (RIA), insulin-like growth factor I (IGF-I) (by immunoradiometric assay) and insulin-like growth factor binding protein-3 (IGFBP-3) (by RIA) levels in 24 children aged between 4 and 14 years with chronic renal failure (CRF) (n = 12 dialysis, n = 12 non-dialysis) and 12 age-matched healthy controls, to find out whether these parameters have a prognostic or therapeutic value on monitoring of growth retardation in CRF. Mean serum IGFBP-3 and PIIINP levels in the dialysis patients were higher than the control group, the difference between the groups was statistically significant (P < 0.05). It seemed that the pubertal stage of the patients did not affect the levels of PICP, PIIINP, and IGFBP-3 while serum IGF-I levels in pubertal patients were significantly higher than those in prepubertal patients (P < 0.001). There was no significant correlation between PICP and PIIINP in any patients. Neither PICP nor PIIINP correlated with height z-score, bone age, IGF-I, or IGFBP-3. It was concluded that the increased serum IGFBP-3 and PIIINP levels in chronic renal disease are poor prognostic indicators leading to progressive renal failure and end-stage renal disease (ESRD). Further investigations into the effects of these collagens on growth failure associated with CRF are needed.

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Pubertal development has recently been evaluated from the standpoint of changes in insulin-like growth factor (IGF)-I and IGF-binding protein-3 (IGFBP-3) levels in healthy children. We studied puberty related changes in serum IGF-I and IGFBP-3 levels in 24 patients (11 prepubertal) with insulin dependent diabetes mellitus (IDDM) and 26 healthy subjects (14 prepubertal). Serum IGF-I and IGFBP-3 levels were assayed using immunoradiometric assays and radioimmunoassays, respectively. Serum IGF-I and IGFBP-3 levels in diabetics did not increase during puberty, as opposed to those in healthy children. Serum IGF-I and IGFBP-3 levels of diabetic patients were found to be lower than those of control subjects during puberty (p < 0.0001 and p < 0.05, respectively). Proteolysis is believed to be a general mechanism to increase IGF bioavailability in the presence of IGFBPs. Increased IGFBP-3 protease activity has been shown in sera of children with IDDM as well as a decrease in this activity in response to insulin therapy. Our data displaying low IGFBP-3 levels in diabetic children may be due to increased proteolysis, which also causes a shift in IGF-I to its lower molecular weight forms. Higher rate of clearance of the latter may be the reason for the low IGF-I levels we observed in children with IDDM. The moderate correlation between insulin dose and IGFBP-3 levels (r = 0.5, p < 0.01) may suggest insulin to be a contributing factor in the regulation of IGFBP-3 levels. We conclude that regulation of IGF-I and IGFBP-3 concentrations is disturbed in children with IDDM, in particular during adolescence.

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An experience with 103 children treated with extracorporeal shock wave lithotripsy (ESWL) is reviewed in this report. The success rate was 63%. The stone volume was of major importance for the result. There was a continuous decrease in success rate with increasing stone size. It was also shown that stone-free rates decreased with an increasing number of stones. Short-term complications were minor and hospitalization times were short. It is concluded that ESWL is a first-choice treatment in children with urinary calculi smaller than 200 mm2 in size.

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Serum levels of carboxyterminal propeptide of type I procollagen (PICP) and aminoterminal propeptide of type III procollagen (PIIINP) can be used as markers of bone formation and the evaluation of children with growth disorders. We measured the serum levels of these collagens with radioimmunoassay in 24 children aged between 4 and 14 years with chronic renal failure (CRF; n = 12 dialysis, n = 12 nondialysis) and 12 age-matched healthy controls, to find out whether these parameters have a prognostic or therapeutic value in monitoring the growth retardation in CRF. Mean serum PIIINP levels in the dialysis patients were higher than in the control group; the difference between the groups was statistically significant (p < 0.05). It seemed that the pubertal stage of the patients did not affect the levels of PICP and PIIINP. There was no significant correlation between PICP and PIIINP in any patients. Neither PICP nor PIIINP correlated with the height z-score or bone age. It was concluded that the increased serum PIIINP levels in renal patients might be accepted as a poor prognostic factor leading to progressive renal failure and end-stage renal disease. Further investigations into the effects of these collagens on growth failure associated with CRF are needed.

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Nephrotoxicity is a common side effect of intravascular contrast media (CM). Although nephrotoxicity of ionic CM has been widely demonstrated, recent studies suggest that newer and more costly non-ionic agents are not less nephrotoxic. We studied the hemodynamic, hematologic and nephrotoxic effects of CM prospectively in 38 patients (ages six months-16 years) with or without risk factors predisposing to nephropathy and compared ionic and non-ionic CM. We performed intravenous urography (IU) with a ionic CM, sodium meglumine diatrizoate (n = 18) and a non-ionic CM, iohexol (n = 20). The patients were divided into three groups according to glomerular filtration rate (GFR) [GFR < or = 50 (n = 9), 50-80 (n = 13), > or = 80 ml/min/1.732 (n = 26)]. Eleven patients had risk factors for nephropathy. Blood pressure, heart rate, ECG, urine and blood samples were obtained 24 hours and one hour before as well as one, 24, and 48 hours after CM infusion. Although a significant increase was found in urine specific gravity, protein/creatinine ratios and serum Na and creatinine levels, the increased levels were within normal limits. We observed a significant reduction in Hb and Htc and urinary prostaglandin E1 levels. Many of the changes observed in the urine and serum values after the use of CM were minor, insignificant and transient, later returning to their initial values. The GFR levels, the presence of risk factors and the use of ionic vs. non-ionic CM had no effect on the results. The elevated urinary basal beta-2-microglobulin levels further increased after CM in patients with low GFRs. It was concluded that non-ionic CM was not superior to ionic CM in patients with GFRs greater than 50 ml/min regardless of predisposing risk factors. One of the non-invasive radiological methods is advised instead of IU in patients with low GFRs.

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The significance of serum insulin-like growth factor I (IGF-I) and insulin-like growth factor binding protein 3 (IGFBP-3) levels in uremia is still controversial. In this study we measured serum IGF-I by immunoradiometric assay (IRMA) and IGFBP-3 levels by specific radioimmunoassay (RIA) in 28 children (aged 3-16 years) with end-stage (n = 14, on hemodialysis) and pre-terminal renal failure (n = 14) and in 15 age-matched healthy children. Thyroid function of the patients was also investigated and GH-stimulation tests with L-Dopa and insulin were performed. Neither IGF-I nor IGFBP-3 levels significantly correlated with mean height SDS for bone age or for chronological age in either non dialysis patients or pubertal (n = 10) or prepubertal patients (n = 18). These data was consistent with the concept that growth in CRF was not related to abnormalities in serum IGF-I or IGFBP-3 levels.

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We evaluated the efficacy of intestinal sonography in the diagnosis of gastrointestinal involvement of Henoch-Schönlein syndrome (HSS). Intestinal sonography was performed in 20 children who were clinically diagnosed as HSS and sonographic findings of the intestinal system were reviewed. Out of 20 patients, 10 who suffered from abdominal pain demonstrated sonographic findings consistent with small intestinal involvement (dilatation of intestinal segments, hypomotility, and eccentric thickening of the intestinal wall). Our results reveal that sonography of the intestine may be useful in the evaluation of the involvement of HSS.

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This prospective study was designed to investigate the most relevant radiological approach for the evaluation of urinary tract infections (UTI) in childhood. In the first 48 hours following the diagnosis of UTI, all patients underwent 99mTc dimercaptosuccinic acid (DMSA) scanning, ultrasonography (US) and intravenous urography (IVU). For the imaging of renal parenchymal infection the sensitivity of IVU and US as compared to DMSA scanning were found to be 9.09% and 25%, respectively. It was concluded that 99mTc DMSA, where available, should be the first step for the accurate diagnosis and follow-up of patients with UTI.

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