Probability of urinary tract infection in infants with ureteropelvic junction obstruction: is antibacterial prophylaxis really needed?

In infants with ureteropelvic junction obstruction (UPJO), the risk of urinary tract infection (UTI) is unknown, and there is a lack of prospective studies showing definitive evidence regarding the benefits and necessity of antibiotic prophylaxis. The aim of this study was to assess the risk of UTI in infants with UPJO and to determine whether the risk varies according to the degree of hydronephrosis. Infants with hydronephrosis detected prenatally or within the postnatal 28th day and who had no previous history of UTI were followed prospectively without antibacterial prophylaxis. Imaging studies were performed according to our Pediatric Uro-Nephrology Study Group protocol. Dimercaptosuccinate (DMSA) scintigraphy was performed in all infants at the end of 1 year of follow-up. Eighty-four infants (56 boys, 28 girls) were included in the study. The distribution of patients in each hydronephrosis grading group was incidentally similar. Within a median follow-up period of 18 (12-24) months, none of the patients had UTI. Furthermore, no pyelonephritic scar was found on DMSA scans in any patient. We conclude that prophylactic antibiotic usage is not indicated in infants with UPJO, regardless of the severity of hydronephrosis, as the risk of UTI is minimal in this population.

Hereditary renal tubular disorders in Turkey: demographic, clinical, and laboratory features.

BACKGROUND: The Turkish Renal Tubular Disorders Working Group aimed to form a patient registry database and gathered demographic, clinical, and laboratory data in various hereditary renal tubular disorders (HRTDs). METHODS: A questionnaire comprising HRTDs was sent to the centers. The cohort was composed of 226 patients (109 girls, 117 boys). RESULTS: The distribution of patients according to HRTD was as follows: 45.6% distal renal tubular acidosis (dRTA), 26.6% proximal RTA (pRTA), 3.5% type IV RTA, 21.7% Bartter's syndrome, and 2.6% Gitelman's syndrome. Cystinosis was the most common cause for renal Fanconi syndrome. Age at diagnosis was between 1 month and 16 years. Overall consanguinity rate was as high as 72%. Rate of affected siblings was 28.5%. pRTA and type IV RTA were more common in males. Most common presenting symptoms were failure to thrive, lack of appetite, and vomiting. Nephropathic cystinosis was the most common HRTD leading to renal failure, followed by dRTA. Hearing loss was present in 23% of patients with dRTA and 6.3% of patients with Bartter's syndrome. No other patient had hearing loss. Convulsions were noted in Bartter's syndrome patients with failure to thrive, especially in those with height below 3%. Polyuria and nephrocalcinosis were more common in dRTA patients with deafness compared with patients without deafness. CONCLUSIONS: This data reflected a high number of HRTDs as a result of high consanguinity rate in Turkey. Our data serve as a database of demographic, clinical, and laboratory features of this rare disease group.

Can serum cystatin C reflect the glomerular filtration rate accurately in pediatric patients under chemotherapeutic treatment? A comparative study with Tc-99m DTPA two-plasma sample method.

OBJECTIVE: It was assessed whether cystatin C (cysC) could be used as a marker of glomerular filtration rate (GFR) by considering the technetium-99m diethylenetriamine penta-acetate (Tc-99m DTPA)-two blood sample method (GFRTc-99m DTPA) as the reference in pediatric patients under chemotherapeutic treatment. METHODS: The chemotherapy group (CG) consisted of 31 patients (21 females, 10 males median age: 8.2 years; range: 2-16 years) who had been planned to receive allogenic hematopoietic stem cell transplantation. All patients in the CG received conditioning regimen (includes chemotherapy protocol) before hematopoietic stem cell transplantation. In addition, 21 patients (14 females, seven males median age: 9.5 years; range: 4-16 years) without any chemotherapy (nonchemotherapy group: nCG) were also prospectively investigated. Serum cysC, serum creatinine, GFRTc-99m DTPA, and GFR with a cysC-based formula (GFRcysC) were analyzed. Tubular function was also assessed. RESULTS: Although we found good correlation between GFRTc-99m DTPA and cysC (r = -0.78), GFRTc-99m DTPA and GFRcysC (r = 0.91), cysC and creatinine (r = 0.91) in nCG, the same correlations were poor in CG (r = -0.42, r = 0.43, r = 0.46, respectively). Tubular function was impaired after chemotherapy. Bias+/-1.96 SD values were -6+/-15.7 and -3+/-54.8 ml/min/1.73 m in nCG and CG, respectively. Precision was also better in nCG (10 ml/min/1.73 m) than in CG (27.6 ml/min/1.73 m). CONCLUSION: Serum cysC and GFRcysC cannot reflect GFR accurately in pediatric patients under chemotherapeutic treatment. Tubular cell damage induced by chemotherapeutics could be a responsible factor through the impairment of tubular absorption and metabolism of cysC.

Urolithiasis in the first year of life.

Data on urolithiasis (UL) in infancy are limited. The objective of this study was to increase awareness of infant UL and to investigate the influence of possible risk factors in this very specific age group. Nonfasting, second-voiding urine samples were obtained to test for urinary excretions of calcium, oxalate, citrate, magnesium, uric acid, and creatinine. Blood analysis included calcium, phosphate, magnesium, uric acid, creatinine, sodium, potassium, chloride, and alkaline phosphatase. Patients received follow-up testing every 1-2 months; serial ultrasonography was used to track UL status. Fifty infants with a median age of 5 months were enrolled in the study. Hypercalciuria was detected in 9/47, hyperoxaluria in 5/39, hypocitraturia in 4/31, and cystinuria in 2/50 infants. We identified at least one metabolic abnormality in 46% of our patients; no metabolic abnormality was identified in 27 infants. Within a mean follow-up period of 14 months, 17 infants became stone free, stones increased in number in ten patients and decreased in number in 16, and recurrence was detected in seven. This study showed that UL could be detected in very early life, even in the newborn period, and could be the source of late childhood/adulthood UL. Infants with nonspecific symptoms such as restlessness may have UL and should undergo ultrasonographic examination. Metabolic evaluation of UL in this specific age group carries some diagnostic challenges, e.g. unsatisfactory data regarding normal ranges of urinary mineral excretion, and collection of 24-h urine samples.

Renal function after hematopoietic stem cell transplantation in children.

OBJECTIVES: The aim of this study was to assess glomerular and tubular renal function after HSCT in children in a prospective trial. METHODS: Renal function was assessed prospectively before HSCT (on day -10), on days +30, +100, and at least 6 months after transplantation in 34 patients (21 females/13 males) with a mean age of 8.2 years. The following parameters were investigated: glomerular filtration rate (GFR) by creatinine clearance (CrCl), cystatin C (CysC)-based formula and plasma clearance of radiolabeled diethylenetriaminepentaacetic acid ((99m)Tc-DTPA), urinary excretion of beta(2)-microglobulin (beta(2)M), beta-N-acetylglucosaminidase (beta-NAG), fractional excretion of sodium (FE(Na)) and fractional tubular phosphate reabsorption (TP/CrCl). RESULTS: Nine patients (26.4%) suffered from acute renal insufficiency within the first 100 days after transplantation. All patients who developed acute renal insufficiency were treated successfully without renal replacement therapy. Age, sex, primary diagnosis, sepsis, veno-occlusive disease, acute graft versus host disease, and use of vancomycin were not significant risk factors for the development of acute renal insufficiency. The medians (99m)Tc-DTPA-based GFR of patients after HSCT showed a statistically significant decrease when compared with pre-transplant values. beta-NAG excretion was significantly elevated in the first 30 days after HSCT. CONCLUSION: Acute and chronic renal impairment can be developed in patients who undergo HSCT even though the pre-transplant renal function is in normal limits and the conditioning regimen does not include TBI. Both glomerular and tubular renal function evaluation should be part of a long-term follow-up in children following HSCT.

Beneficial effect of triple treatment plus immunoglobulin in experimental nephrotic syndrome.

Combinations of antiproteinurics, including angiotensin I-converting enzyme inhibitors + angiotensin II receptor antagonist + statins, are promising choices in the treatment of steroid-resistant nephrotic syndrome. We aimed to investigate the effects of high doses of immunoglobulin in addition to these combinations in rats with adriamycin-induced nephrosis. The study included 40 rats allocated into five groups: control, nephrotic syndrome without treatment, dual therapy (DT) with enalapril + losartan, triple therapy (TT) with enalapril + losartan + simvastatin, and quadruple therapy (QT) with enalapril + losartan + simvastatin + a high dose of immunoglobulin. The proteinuria levels were not statistically different between DT, TT and QT groups at weeks 5, 8, 12 and 16. At week 16, serum creatinine levels in the QT group were significantly lower than those in the control, DT and TT groups. The glomerulosclerosis index in the DT group was significantly lower than in the TT and QT groups. The scores for interstitial fibrosis and TGF-beta staining were similar among treatment groups. In conclusion, we showed that quadruple therapy including immunoglobulin had a beneficial effect on renal function in the late phase, but it had no additional effects in reducing proteinuria or in glomerulosclerosis score in experimental nephrotic syndrome. Further studies with angiotensin I-converting enzyme inhibitors (ACEIs), angiotensin II receptor antagonists (AIIRAs) and immunoglobulin combinations would offer some benefits in the treatment of nephrotic syndrome.

Evaluation of reproductive functions in male adolescents following renal transplantation.

The aim of this study was to analyze the semen variables and hormone profiles in kidney transplanted male adolescents. Eight post-pubertal male patients who underwent successful renal tx during the peripubertal period and who had ESRD during childhood were enrolled in the study. Patients who underwent tx before 14 yr old (group I) and patients who underwent tx after 14 yr old (group II) were evaluated separately. Semen was collected and analyzed. Serum levels of LH, FSH, and testosterone were measured and found to be normal in all patients except one. The mean age at the diagnosis of CKD was six yr and 13 yr in groups I and II, respectively. The mean age at the time of tx was 12 yr in the first and 17.8 yr in the second group. The patients in group I had received prednisone, cyclosporine A and azathioprine with a longer duration of time compared with patients in group II. Sperm counts (15.5 +/- 15.7 vs. 82.3 +/- 64.2 millions/mL) and sperm motilities (37.8 +/- 30.9 vs. 57.8 +/- 22.1%) were lower in group I than group II. Only one patient in group II had normal sperm parameters and azospermia was observed in one patient from group I. We conclude that the earlier onset and the longer duration of uremia, the more impairment of reproductive function. Also, it seems that duration of exposure to corticosteroids or cyclosporine combined with azathioprine contribute to sperm dysfunction in peripubertal transplanted boys.

Screening for hypercalciuria in schoolchildren: what should be the criteria for diagnosis?

The methodologies to diagnose hypercalciuria have not yet been standardized. The aims of this study were to assess the correlation between urinary calcium/creatinine ratio (UCa/Cr) > or = 0.21 (mg/mg) and 24 h urinary calcium excretions and to determine the reference values of the UCa/Cr ratio among a large population of schoolchildren in southern Turkey. Non-fasting, second morning urine samples were collected from 2,143 children aged 7-14 years. In children with suspected hypercalciuria [UCa/Cr > or = 0.21 (mg/mg)], 24 h urine samples were collected. The 95th percentile values of the UCa/Cr ratio for each age were calculated and showed a decrease in value with advancing age. In all, 269 (12.5%) of the children had UCa/Cr > or = 0.21 (mg/mg), of whom 66 (24.5%) had daily urinary calcium excretion > or =4 mg/kg per day. A weak correlation was found between spot UCa/Cr ratios and daily urinary calcium excretions in children with UCa/Cr > or = 0.21 (r = 0.27). We conclude that a spot UCa/Cr ratio of 0.21 (mg/mg) as the upper limit of normal cannot be used universally to define hypercalciuria. Age-specific reference values for UCa/Cr should be established for each population, to be used as a screening test for hypercalciuria, and the definite diagnosis should be established with 24 h urinary calcium excretion whenever possible.

Adjuvant effect of vitamin A on recurrent lower urinary tract infections.

BACKGROUND: The purpose of the present paper was to investigate the effects of vitamin A supplementation on recurrent lower urinary tract infections (RUTI). METHODS: Twenty-four patients with non-complicated RUTI were included in a placebo-controlled, double-blinded study. Twelve patients received a single dose of 200,000 IU vitamin A in addition to antimicrobial therapy. Patient and control groups (each containing 12 patients) were followed for up to 1 year and were evaluated for eradication and frequency of lower urinary tract infections (UTI). Serum levels of vitamin A and beta-carotene were determined periodically. RESULTS: During the first 6 months follow-up period the infection rate of the vitamin A-supplemented group reduced from 3.58 to 0.75 per 6 months, and in the subsequent 6 months the infection rate was 1.75 per 6 months. These values were calculated as 2.75, 2.83 and 2.66, respectively, in the placebo group. CONCLUSION: Vitamin A supplementation may have an adjuvant effect on the treatment of RUTI.

Case of Bartter syndrome presenting with hypokalemic periodic paralysis.

Hypokalemic periodic paralysis can occur secondarily to excessive potassium loss. Thyrotoxicosis, diuretic ingestions, hyperaldosteronism, barium poisoning, Gitelman syndrome, and Bartter syndrome are among the disorders causing secondary hypokalemic periodic paralysis. Clinical presentation of Bartter syndrome with hypokalemic periodic paralysis is rare. A 12-year-old boy was admitted to our hospital because of transient paralysis. He had been suffering from transient weakness attacks for 2 years and had had a total of 10 attacks, lasting 1 to 3 days. He had growth retardation, polyuria, and polydipsia. Laboratory examinations revealed hypokalemic alkalosis, normomagnesemia, hypercalciuria, and hyperaldosteronism. The clinical and laboratory findings were in accordance with Bartter syndrome. He has been followed up for 6 months and has suffered no further paralytic attacks under indomethacin therapy. This case highlights the importance of blood pH measurement in patients with hypokalemic periodic paralysis; it might prevent misdiagnosis and mismanagement in such diseases.

Value of the urine strip test in the early diagnosis of bacterial peritonitis.

BACKGROUND: In an effort to detect the presence of leukocytes in the peritoneal dialysate fluid (PDF) a urine dipstick may be practical for the early detection of peritonitis in peritoneal dialysis patients. METHODS: The study was performed in 44 samples of four children with peritonitis. The total counts of white blood cell (WBC) and polimorphonuclear neutrophils (PMNs) were found using both a hemocytometer (CELDYN 3700 R) and a microscopic method. The existence of leukocytes was investigated by urine dipstick tests. RESULTS: The dipstick test was correlated with both hemocytometer and microscopic methods (r = 0.537, P = 0.001; r = 0.560, P = 0.0001, respectively). Our results revealed no false negative values in all strip categories. At the proposed cut-off point (> 100/mm3 of WBC count), a 3+ reading on the strip test reached a sensitivity of 100% for the detection of peritonitis with a specificity of 100%. A 2+ reading reached a sensitivity of 100% with lower specificity (71.4%) at the same cut-off point. The dipstick test correlated significantly with the total counts of PMNs (r = 0.80, P = 0.0001). All positive strip categories had more than 50% of PMNs with a low PMN percentage of negative strip category in PDF samples. CONCLUSION: It is proposed that the strip test might be a valuable test to diagnose bacterial peritonitis through the detection of both WBC and PMN in peritoneal dialysis patients.

Chronic peritoneal dialysis in Turkish children: a multicenter study.

Chronic peritoneal dialysis (CPD) has been utilized in the treatment of children since 1989 in Turkey. The aims of this study were to summarize our experience with CPD in children and to establish a pediatric registry data system in Turkey. Standard questionnaires were sent to all pediatric CPD centers. 514 patients treated between 1989 and 2002 in 12 pediatric centers were enrolled in the study. Reflux nephropathy was the most common (18.1%) cause of renal failure. Mean age at dialysis initiation was 10.1+/-4.6 years. Mean duration of dialysis was 24.1+/-20.5 months. Continuous ambulatory peritoneal dialysis (CAPD) was the first CPD modality for 476 (92.6%) patients, 142 of whom switched to automated peritoneal dialysis (APD) during follow-up. Currently, 47.3% of the patients are still on CPD, 15.4% were transplanted, 13.2% switched to hemodialysis, 16.7% died. The patient and technique survivals were 90% and 95% at one year and 70% and 69% at five years, respectively. The survival was significantly shorter in the youngest age group (0-24 months) compared to those in older age groups (p=0.000). We herein report the first results of the TUPEPD study providing information on demographic data and survival of pediatric CPD patients. As opposed to clear recommendations in favor of APD, there is a clear preponderance of CAPD in our pediatric CPD population. That vesicoureteral reflux (VUR) is still the leading cause of renal failure is a distressing finding. Remarkably lower survival rates and transplantation ratios are as striking and distressing as the high incidence of VUR among the causes of ESRD. We conclude that we must make a great effort to achieve better results and to change these undesirable events.

Mercury intoxication resulting from school barometers in three unrelated adolescents.

UNLABELLED: Three adolescents with severe hypertension due to mercury intoxication are presented. Two of them had skin rash, signs and symptoms of central nervous system involvement, peripheral neuropathy and mild-to-moderate proteinuria in addition to hypertension. All three patients had a history of exposure to mercury, the source being broken barometers taken from school laboratories 2-4 months previously. Urine and blood mercury levels were consistent with mercury intoxication. The patients were treated with chelation therapy. One of them died; the others recovered over a period of 1-4 months. CONCLUSION: mercury intoxication should be considered in any child with signs and symptoms of hypertension, skin rash, peripheral neuropathy and behavioural changes. The parents and school administrators, as well as paediatricians, should be aware of the potential risks of mercury and should be encouraged to avoid mercury-containing devices in schools and households.

Rapid decline of anti-pneumococcal antibody levels in nephrotic children.

Nephrotic children are at increased risk for pneumococcal infections. Antibody responses to the currently recommended pneumococcal polysaccharide vaccine have been variable and maintenance of adequate antibody levels over time has not been well documented. In this study, we determined total IgG antibody levels against pneumococcal polysaccharides before and 1, 6, 12 and 36 months after 23-valent pneumococcal polysaccharide vaccine (PPV) administration in nine children with steroid-responsive nephrotic syndrome during remission while off corticosteroids. The baseline antibody levels were between 4 and 86 mg/l. Four weeks after vaccination, the titer increased at least twofold in all patients with a mean arithmetic value of 165.4 mg/l. At the 6th month, the levels decreased in six out of nine subjects to a mean of 94.6 mg/l. At the 36th month, the control antibody levels were below the baseline or below the early postvaccination values in four out of nine subjects. Only two patients had stable high concentrations through the study period. Our data show that nephrotic patients may not retain their antibody levels despite reasonably good initial responses to the pneumococcal vaccine and that susceptibility to infections may continue in vaccinated children.

Visceral Kaposi's sarcoma with intracranial metastasis: a rare complication of renal transplantation.

The incidence of Kaposi's sarcoma (KS) has increased in solid organ transplantation recipients. This type of KS tends to be aggressive, involving lymph nodes, mucosa and visceral organs in about half of patients, sometimes in the absence of skin lesions. Brain involvement of KS has rarely been reported. A 16-yr-old Turkish boy underwent renal transplantation from his mother. The immunosuppressive regimen included prednisolone, cyclosporin A and azathioprine. Fourteen months later the azathioprine was changed to cyclophosphamide (3 mg/kg/day) because of the development of a nephrotic syndrome. After 12 weeks, the cyclophosphamide was changed to mycophenolate mofetil (MMF) to control the nephrotic syndrome. At this time his serum creatinine level rose to 2.1 mg/dL. Polyclonal or monoclonal antibodies were never given. Multiple intra-abdominal lymphadenopathy was detected on abdominal tomography at the 32nd month after renal transplantation. Kaposi's sarcoma was diagnosed via laparotomy and biopsy. He had a generalized tonic and clonic seizure and contrast enhanced cranial tomography showed two intracranial masses which had an abundant vascular component which caused a mild shift. One of the masses was removed via a burr-hole with the aim of diagnosis and treatment of the shift. A pathologic examination of the intracranial lesion was also reported as Kaposi's sarcoma. Herpes virus-8 DNA was detected by PCR in the intracranial lesion.

Late diagnosis of severe colchicine intoxication.

A 4-year-old Turkish girl was referred to our hospital with the findings of encephalopathy and pancytopenia. She had a history of severe abdominal cramps and gastrointestinal bleeding. A confused state, muscle pain and weakness, erythema-bullous and erythema-nodosum-like skin lesions, and alopecia were observed at her hospitalization. All of these symptoms resolved on follow-up. On laboratory investigation severe thrombocytopenia and leukopenia, mild anemia, a moderate increase in aspartate aminotransferase and alanine aminotransferase levels were detected. After reevaluating her medical history, it was learned that she had accidentally taken 1.3 to 1.5 mg/kg of colchicine 3 to 4 days before her first hospitalization. The possibility of misdiagnosis of colchicine intoxication should be borne in mind, and pediatricians must be aware of its toxic effects, especially in areas where patients with familial Mediterranean fever are present.

IgG and IgG subclasses deficiency in children undergoing continuous ambulatory peritoneal dialysis and its provocative factors.

BACKGROUND: Low levels of serum IgG or IgG subclasses may be responsible for the defective peritoneal defense and for peritonitis attacks in continuous ambulatory peritoneal dialysis (CAPD) children. Malnutrition, peritoneal loss or frequent peritonitis may lead to IgG or IgG subclasses deficiency. METHODS: Levels of IgG subclasses were determined in 12 children undergoing CAPD treatment. Radial immunodiffusion technique was used for determination. Patients were aged from 6 to 16 years (mean age 12.3 years) and had been on CAPD for 11-26 months (mean duration 19.4 months). We evaluated whether IgG and IgG subclasses deficiency are related to malnutrition, the peritonitis rate and the duration of CAPD using the SPSS program. RESULTS: Serum total IgG levels were found to be low in eight out of 12 patients. Eight patients showed low levels of IgG1, four patients IgG2, one patient IgG3 and three patients IgG4. Total IgG values were found to be positively correlated to malnutrition status, peritonitis rate and duration of CAPD. The IgG2 values were found to be related to the duration of CAPD. The IgG4 values were found to be correlated to the peritonitis rates. CONCLUSIONS: The IgG and IgG subclasses deficiency may develop in children while on CAPD treatment. Periodical determinations of either serum IgG or the subclasses may be useful in the follow-up of these patients.