The outcome of Iranian children on continuous ambulatory peritoneal dialysis: the first report of Iranian National Registry.

BACKGROUND: Continuous ambulatory peritoneal dialysis is not a very common modality to treat Iranian children with end-stage renal disease; however, there is sometimes no choice but to offer this therapy to salvage the patient. Obviously, promotion in each program needs re-evaluation to find the pitfalls. Therefore, a nation-wide survey on pediatric continuous ambulatory peritoneal dialysis was conducted to find the cause of death or termination of dialysis. METHODS: All children, younger than 14 years old, treated by continuous ambulatory peritoneal dialysis in nine main pediatric nephrology wards in Iran between 1993 and 2006 were included in this historical cohort study. Patient and technique survival rates were determined. Kaplan-Mayer and Cox-regression analysis were used to compare the survival. 2 x 2 table was used to calculate the risk ratio. A P<0.05 was considered significant. RESULTS: One hundred twenty children with a mean age of 47.6 months were on continuous ambulatory peritoneal dialysis. The most frequent cause of renal failure was hereditary-metabolic-cystic disease. One hundred eighty-two peritoneal dialysis catheters were inserted surgically. The median first catheter exchange was 0.74 year (95%CI: 0.5 - 0.98). The most frequent cause of catheter replacement was catheter outflow failure due to displacement, adhesion, and infection (persistent peritonitis or tunnel infection). The mean patient survival was 1.22 years (95%CI: 0.91 - 1.53). The mortality rate was 55% before 1997, and 60% between 1998 and 2001, which declined to 23% after 2002 (P<0.05). Young age (<24 months) was the only independent factor that predicted mortality (P<0.05). The outcome of children was as follows: recovery of renal function (6.7%), renal transplantation (8.3%), switch to hemodialysis (16.7%), still on continuous ambulatory peritoneal dialysis (23.3%), death (43.3%), and lost to follow-up (1.7%). CONCLUSION: The mortality is still high among Iranian children on peritoneal dialysis. Young age is the most important factor influencing on survival and mortality.

The neonatal outcome in twin versus triplet and quadruplet pregnancies.

BACKGROUND: To assess the risk of neonatal mortality and morbidity in twin, triplet and quadruplet pregnancies. METHODS: In a retrospective study, the neonatal outcome of all twin, triplet and quadruplet gestations delivered from October 2001 to September 2006 was reviewed. The neonatal outcome of triples and quadruplets was compared with a matched group of twins for gestational age. RESULTS: During a 5-year period, 511 sets of twin pregnancies, 42 sets of triplet and 5 sets of quadruplet pregnancies were studied. The mean of gestational age for twins, triplets and quadruplets were 33.92 ± 3.5 weeks, 30.92 ± 3.8 weeks and 31.60 ± 2.0 weeks, respectively, (P = 0.0001). Triplets and quadruplets weighed less than twins, (P = 0.0001). Neonatal mortality was 13.5% for twins, 26.8% for triplets and 30% for quadruplets. In vitro fertilization, use of ovulation induction agents, and cesarean delivery in the women with triplet and quadruplet were significantly higher than in those with twin pregnancies, (P = 0.0001). The mean age of mothers with triplets and quadruplets was significantly higher than with twins (P = 0.026). There was not a significant difference in respiratory and non-respiratory short outcomes between triplets, quadruplets and twins when matched for gestational age. Apgar score at 1 and 5 minutes was significantly lower in triplets and quadruplets than twins. There was no influence of birth order on neonatal mortality of triplet pregnancy. Neonatal mortality of triplet births was significantly decreased over the 5 years of the study period. CONCLUSIONS: Triplets and quadruplets have a similar neonatal outcome as twins when matched for gestational age. There is no influence of birth on the neonatal mortality of triplet pregnancy. It appears that outcome is mainly dependent on gestational age.

The metabolic syndrome in survivors of childhood acute lymphoblastic leukemia in Isfahan, Iran.

BACKGROUND: To determine the prevalence of metabolic syndrome in survivors of childhood leukemia in Isfahan, Iran. METHODS: During a 4-year period (2003 to 2007), 55 children (33 male and 22 female) diagnosed with ALL at Unit of Hematology/ Oncology, Department of Pediatrics, Isfahan University of Medical Science, were enrolled in this cross-sectional study. Metabolic syndrome was defined using the modified version of Adult Treatment Panel (ATP III) crite-ria. Insulin resistance was defined based on the homeostasis model assessment index (HOMA-IR). RESULTS: The mean age of participates was 10.4 years (range 6-19 years) and the mean interval since completion of chemotherapy was 35 months. Twenty percent (11/55) of survivors (10 male, 1 female) met criteria for diagnosis of metabolic syndrome. Obesity was observed in one forth of patients and nearly 3/4 of obese patients had metabolic syndrome. High serum insulin levels were found in 16% of participants and in 63% of obese survivors. The mean insulin levels in survivors with metabolic syndrome was three-times more than those without (28.3 mu/l vs. 9.57 mu/l, p = 0.004). Insulin resistance was detected in 72.7% of survivors with metabolic syndrome and it was positively correlated with serum triglycerides (0.543, p ≤ 0.001), systolic and diastolic BP (0.348, p = 0.01 and 0.368, p = 006 respectively), insulin levels (0.914, p < 0.001) and blood sugar (0.398, p = 003). CONCLUSIONS: The prevalence of metabolic syndrome in survivors of childhood leukemia in Iran is higher than developed countries. Nearly all of the obese patients had metabolic syndrome. Weight control and regular physical exercise are recommended to the survivors.

New treatment protocol for primary nocturnal enuresis in children according to ultrasound bladder measurements.

OBJECTIVE: To evaluate the response rate of various modalities of therapy in primary nocturnal enuretic children according to the ultrasound bladder volume and wall thickness index (BVWI) measurements. METHODS: From February 2006 to November 2007, a total of 31 children, aged 6-12 years old were enrolled in a clinical trial. Based on BVWI they were divided into 3 groups as follows: Group 1 (BVWI <70%) was treated with oral desmopressin and oxybutynin; Group 2 (BVWI 70% to <130%) was treated with oral desmopressin. Group 3 (BVWI >130%) was treated with oral desmopressin accompanied by double-voiding technique and scheduled voiding. All of them were treated for 3 months. RESULTS: Significant reductions in mean bed-wetting frequency before and after first treatment cycle were observed in all groups (p<0.05). The complete response rate was 70% in Group 1, 25% in Group 2, and 20% in Group 3. Overall, the complete and partial response rate was 9/10 (90%) children in Group 1, 13/16 (81%) in Group 2, and 3/5 (60%) in Group 3. Bedwetting frequency significantly decreased at the first and second treatment cycles in Group 2 (p<0.05) for each pair wise comparison. CONCLUSION: The proposed treatment representation according to ultrasound BVWI measurements achieves favorable response rates in children with PNE. We suggest that this treatment should be used to develop the management of enuresis in children.

Etiology and pathology of enuresis among primary school children in Isfahan, Iran.

OBJECTIVE: To determine the etiology and pathogenesis of enuresis among primary school children by using a special ultrasound (US) protocol for the assessment of bladder dysfunction and to compare excretion of urinary sodium and calcium in enuretic children. METHODS: We conducted this cross sectional study on 66 enuretic children aged 6-12 years from September 2005 to January 2006 in Isfahan University of Medical Sciences, Iran. Ultrasound (US) was designed for the evaluation of bladder parameters using bladder volume and wall thickness index (BVWI%), and expected percentage bladder volume index for kidney volume. RESULTS: Sixty children (90.9%) had nocturnal enuresis, 5 (7.5%) had diurnal enuresis and one child (1.6%) had nocturnal and diurnal enuresis. Urinary infection was detected in one child (1.5%). The incidence of urinary system abnormalities was 10.6% in all enuretic children. Hypercalciuria was seen in 9.2% and natriuresis in 20.3%. Normal bladder function (BVWI 70% to <130%) was seen in 67%, small bladder with a thick wall (BVWI <70%) in 27% and large bladder capacity with a thin wall (BVWI >130%) was seen in 6% of children with primary nocturnal enuresis (PNE). There was a significant difference in BVWI between children with PNE and secondary nocturnal enuresis (p=0.01). CONCLUSION: Enuresis is a common problem among school children and associated urinary abnormalities are not uncommon. Our results show that US measured bladder parameters can provide useful clues for the underlying bladder dysfunction and may help to guide clinical management.

An epidemiological study of enuresis among primary school children in Isfahan, Iran.

OBJECTIVE: To estimate the prevalence of enuresis in primary school children in Iran and to determine the factors associated with this disorder. METHODS: A cross-sectional time-ordered study was performed at the Faculty of Medicine, Isfahan Medical University, Isfahan, Iran from September 2005 to January 2006. A total of 4500 self-administered questionnaires were distributed to parents of children aged 6-12 years attending 30 primary schools. RESULTS: From an overall response rate of 69.9%, enuresis was reported in 216 children (7%), comprising 6.2% for nocturnal enuresis according to ICD10 and 3.3% according to DSM IV, 0.5% for diurnal enuresis and 0.8% for combined day and night wetting. Primary nocturnal enuresis was reported in 166 children (5.3%). Seventy-one (50.7%) of the 140 children with nocturnal enuresis had > or =3 wet nights per week. A positive family history in father and mother was seen in 51% and 39% of children with primary nocturnal enuresis respectively. Using logistic regression analysis, younger age (p < 0.002), gender (p < 0.0001) and low level of education of mother (p < 0.028) were significant predictors of enuresis. Positive history of enuresis in father was a significant predictor of primary nocturnal enuresis (p < 0.012). CONCLUSION: The prevalence of nocturnal enuresis in Iran is lower than those reported in western countries, however, higher percentage demonstrated severe enuresis. The prevalence of diurnal enuresis is lower than previous studies. Age, gender and the educational level of the mother are the main risk determinants of enuresis and the prevalence of primary nocturnal enuresis appears to be significantly related to positive history of enuresis in father.

Value of sonography in the diagnosis of mild, moderate and severe vesicoureteral reflux in children.

This study was conducted to determine the positive predictive value (PPV) of gray scale sonography in the diagnosis of mild, moderate and severe vesicoureteral reflux (VUR). This cross-sectional descriptive analytic study was conducted in the University Clinic of Isfahan University of Medical Sciences in 2008. The study was performed on children aged two to 12 years; female children with febrile urinary tract infection (UTI) and clinical suspicion of VUR and males with history of UTI and VUR who attended follow-up during the six months study period were selected. Non-cooperative patients were excluded from the study. A total of 90 patients were studied. The data gathering method was sequential. The following sonographic parameters were assessed in all the study patients: antero-posterior pelvic diameter, distal ureteric diameter and stasis of urinary system, which were measured at rest and during and after the Valsalva maneuver. Also, the vesicoureteral junction distance and distance of vesicoureteral junction to the midline were measured on both sides at rest. We then referred them for radionuclide cystography (RNC) and the results of the two methods were compared. Data were analyzed with SPSS program and t-test and chi square formulas were used. The sensitivity of ultrasound in the diagnosis of VUR was 70.9% and the specificity was 51.4%. Thus, the PPV was 69.64% and the negative predictive value was 52.94%. Although the most definite diagnosis of VUR is made with micrurating cystoureterography or RNC, sonography may be used as the first step in evaluation for VUR, especially in high-grade VUR.

Pathological diagnosis of antibody-mediated rejection in renal allograft without c4d staining, how much reliable?

BACKGROUND: C4d as a part of complement activation process is a marker for detecting antibody-mediated rejection (ABMR) and its positivity accompanied by positive donor specific antibody (DSA), and morphologic view of humoral rejection has been suggested to detect ABMR since 2003. MATERIALS AND METHODS: 41 specimens of transplanted kidney biopsies gathered from 2006 to 2008 were evaluated for morphological changes on light microscopy, and nephro-pathologist made distinct diagnosis for all of specimens then c4d staining was done for all of them. The association between primary diagnosis without c4d staining and c4d scoring on peritubular capillaries and glomerular capillaries were evaluated to determine whether morphological changes were enough for distinct diagnosis or not. RESULTS: Acute tubular necrosis (ATN) 27%, interstitial fibrosis and tubular atrophy (IF&TA) 17%, and T cell mediated rejection (TCMR) 22% were the commonest diagnosis on light microscopy, and 17% of all biopsies had diffuse positive c4d staining. There was not any report of ABMR in morphological evaluation while c4d positive staining was seen in some specimens (17%). It may result from masking of ABMR by other morphological changes such as TCMR and no specific histologic changes for ABMR on light microscopy. CONCLUSION: We would like to emphasize that c4d staining should be done for all of renal allograft biopsies, and pathologists all over the world should consider the probability of ABMR masked by other morphological changes on light microscopic evaluation.

Fractional excretion of magnesium (FEMg), a marker for tubular dysfunction in children with clinically recovered ischemic acute tubular necrosis.

Among the different etiologies of acute renal failure (ARF), acute tubular necrosis (ATN) is one of the most common causes. There is no consensus on the duration of follow-up needed among these patients and also on choosing a reliable screening test to recognize early signs of chronic kidney injury that may ensue. The aim of this study was to evaluate the clinical and biochemical findings in children with a history of clinically recovered ischemic ATN, to detect the patients who may be at risk of ensuing chronic kidney disease. A cross-sectional study was carried out on 20 children between six months and 10 years of age, admitted at St. Al Zahra Hospital and Amin Children's Hospital, Isfahan, Iran, with a past history of ischemic ATN. Eighteen patients were evaluated between 12 and 24 months, and two patients were evaluated at 30 months. The second sample of urine while still fasting was used for assessing urinary sodium, creatinine and magnesium. The mean ages for study and control groups were 3.4 ± 1.3 years and 4.5 ± 1.1 years, respectively. Glomerular filtration rate, urinary magnesium, fractional excretion of magnesium (FEMg), urinary sodium and fractional excretion of sodium (FENa) were significantly higher in the study group compared to those in the control group. No significant differences were demonstrated in systolic and diastolic blood pressures between the two groups. Since FEMg can reflect tubular function for both the ability of tubules for reabsorption of the filtered magnesium and for retaining the intracellular magnesium, FEMg can be used as a marker to detect early stages of chronic renal injury. However, further studies with larger number of cases are needed to evaluate the sensitivity of this test.

Nephrocalcinosis in very low birth weight infants.

To determine the incidence and risk factors of nephrocalcinosis in preterm infants, we studied in a prospectively 64 preterm infants of birth weight :5 1500 g from February 2006 to November 2007. Data were collected on gestation, birth weight, gender and family history of renal calculi, respiratory support, and use of nephrotoxic drugs. The parameters of mineral metabolism were assessed in blood and spot urine samples at the end of 2 nd and 4 th weeks of age. Forty-nine babies completed the study, and nephrocalcinosis was observed in 13 (26.5%) babies and was bilateral in 7 (14.3%) infants. The mean age of diagnosis of nephrocalcinosis was 52.58 days (range 30-123 days). Gestational age, birth weight, and sex were not significantly associated with increased risk of nephrocalcinosis. The mean duration of ventilation was significantly less in babies with than without nephrocalcinosis (P= 0.020), and the mean levels of urine calcium and phosphate at 4 weeks of age, respectively (P= 0.013, P= 0.048). There were also significant differences in urine calcium/creatinine ratio (P= 0.001), mean plasma levels of calcium at 2 weeks of age (P= 0.047) and plasma levels of phosphate at 4 weeks of age (P= 0.016) between babies with and without nephrocalcinosis. Using logistic regression analysis, family history of renal stone (P= 0.002) and urine calcium/creatinine ratio (P= 0.011) were significant predictors of nephrocalcinosis. However, there were no significant differences in the length of stay in the intensive care unit, duration of total parenteral nutrition, and duration and cumulative doses of nephrotoxic drugs between these two groups. We conclude that the incidence of nephrocalcinosis was similar in our population to the previous studies. Family history of renal stone and urine calcium/ creatinine ratio are the major risk factors of nephrocalcinosis in very low birth weight neonates.

Post transplant anaplastic large T-cell lymphoma.

Post transplant lymphoproliferative disorders (PTLD) are a heterogeneous group of lymphoid proliferation that ranges from polyclonal hyperplasia to monoclonal malignant lymphoma. We report a 13-year-old boy who was diagnosed with PTLD in February 2007 after 3 1/2 years of deceased renal transplantation. We treated him with an adapted ACVBP (doxorubicin, cyclo-phosphamide, vincristine, bleomycin and prednisone) regimen. He responded well to the chemo-therapy without deterioration of graft function.

Prevalence and severity of anemia in pediatric hemodialysis patients.

OBJECTIVE: To determine the prevalence and severity of anemia, and to identify independent predictors for anemia in children on hemodialysis. METHODS: We conducted this cross sectional study on 25 children aged 7-20 years receiving hemodialysis from September 2005 to January 2006 in Isfahan University of Medical Sciences, Isfahan, Iran. RESULTS: A total of 22 (82%) patients had hemoglobin (Hb) level of <11 g/dL (anemia) and 12 (48%) patients had Hb values <8 g/dL (severe anemia). The mean age was 15.5 +/- 3.7 years. Mean time on hemodialysis was 20.44 +/- 15.25 months. Anemia was more common and severe among children who were on dialysis <6 months. There was an inverse relation between severity of anemia and duration of hemodialysis (r=-0.465, p=0.019). Nearly all patients were treated with recombinant human erythropoietin (rHuEPO). Children with severe anemia received a slightly higher dose of erythropoietin (r=0.202 p=0.09). There was a correlation between serum albumin and Hb level (r=0.511, p=0.01). Intact parathyroid hormone (iPTH) levels were >200 pg/mL in 16 patients (66%) and >400 pg/mL in 9 patients (37.5%). There was a reverse correlation between iPTH level >200 pg/mL and Hb level (r=-0.505, p=0.046). CONCLUSION: The prevalence of anemia in our study appears to be higher than that reported in the other studies in spite of extensive use of rHuEPO and iron supplementation. We found this to be especially true for patients who were on dialysis <6 months and with low albumin and severe hyperparathyroidism.