Effects of pentoxifylline on gentamicin-induced nephrotoxicity.

We aimed to investigate the underlying mechanisms responsible for the renoprotective effects of pentoxifylline (PTX) in gentamicin (GEN)-induced nephropathy. On this purpose, 26 female Wistar rats (200-250 g) were included and four groups were formed. The first one was the control group (n:5). The rats in other groups (n:7 for each) received 50 mg/kg twice daily intraperitoneal (i.p.) PTX, 100 mg/kg i.p. GEN and both GEN and PTX at the same doses for consecutive 8 days, respectively. Rats were weighed both at the beginning and end of the study. After the last dose, 24-hour urines were collected and the rats were sacrificed. Blood samples and kidney tissues were obtained for biochemical, histological, oxidative stress, and apoptotic parameters. Body weights were similar in all groups at the beginning of the study. Rats in GEN group had significant weight loss, tubular damage, and increased apoptosis, while GEN + PTX group had significantly better outcomes. Scr, urinary protein/creatinine, and TBARS levels were significantly higher and Ccr and SOD levels were significantly lower in GEN and GEN + PTX groups in comparison to control and PTX groups, but the levels were similar between GEN and GEN + PTX groups. In conclusion, concomitant administration of PTX provides renoprotection via suppressing apoptosis in GEN-induced nephropathy.

Neuropsychiatric involvement in juvenile-onset systemic lupus erythematosus: A multicenter study.

INTRODUCTION: Neuropsychiatric (NP) involvement is a restricted area in juvenile-onset systemic lupus erythematosus (jSLE). AIM: To investigate the prevalence, demographic and clinical features, and outcomes of the neurological involvement in the Turkish jSLE population. METHODS: This study was based upon 24 referral centers' SLE cohorts, multicenter and multidisciplinary network in Turkey. Patient data were collected by a case report form which was standardized for NP definitions according to American Collage of Rheumatology (ACR). Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) neuropsychiatric part was used to determine NP damage. Variables were evaluated Ward's hierarchical clustering analyses, univariate, and multivariate logistic regression analyses. RESULTS: A hundred forty-nine of 1107 jSLE patients had NP involvement (13.5%). The most common NPSLE findings were headache (50.3%), seizure (38.3%), and acute confusional state (33.6%). Five clusters were identified with all clinical and laboratory findings. The first two clusters involved neuropathies, demyelinating diseases, aseptic meningitis, and movement disorder. Cluster 3 involved headache, activity markers and other SLE involvements. Idiopathic intracranial hypertension, cerebrovascular disease, cognitive dysfunction, psychiatric disorders and SLE antibodies were in the fourth, and acute confusional state was in the fifth cluster. In multivariate analysis, APA positivity; OR: 2.820, (%95CI: 1.002-7.939), P: 0,050, plasmapheresis; OR: 13.804 (%95CI: 2.785-68.432), P: 0,001, SLEDAI scores; OR: 1.115 (%95CI: (1.049-1.186), P: 0,001 were associated with increased risk for neurologic sequelae. CONCLUSION: We detected the prevalence of juvenile NPSLE manifestations in Turkey. We have identified five clusters that may shed light pathogenesis, treatment and prognosis of NP involvements. We also determined risk factors of neurological sequelae. Our study showed that new definitions NP involvements and sequelae for childhood period are needed.

Concurrent protracted febrile myalgia syndrome in a child with diabetic ketoacidosis.

Familial Mediterranean Fever (FMF), characterized by recurrent attacks of inflammation in predominantly serosal and synovial membranes, is caused by MEFV gene mutations resulting in abnormal pyrin. Protracted febrile myalgia syndrome (PFMS), a kind of vasculitis requiring corticosteroid treatment, is associated with M694V mutation of MEFV gene. Here, we report a case where the patient developed PFMS leading to the diagnosis of FMF concurrently at the time of treatment for diabetic ketoacidosis (DKA) of new-onset type 1 diabetes mellitus and discuss the possible mechanisms of simultaneous DKA and FMF-associated PFMS. DKA-associated cytokine release may be a predisposing factor or trigger for FMF-associated PFMS.

Sirolimus in pediatric renal transplantation.

SRL, an mTOR inhibitor that inhibits cell cycle progression, represents an important alternative to CNIs, which are still the cornerstones of pediatric solid organ tx. Because there are still limited data on SRL use among pediatric solid organ recipients, further studies are needed to verify the efficacy and safety of SRL. It has unique pharmacokinetic characteristics concerning dosing intervals and reduction of the dose in combination with other immunosuppressants. SRL also has antineoplastic, antiviral, and antiatherogenic advantages over other immunosuppressive agents. The adverse effects of SRL including thrombocytopenia, hyperlipidemia, proteinuria, impaired wound healing, mouth ulcers, edema, male hypogonadism, TMA, and interstitial pneumonitis must be considered carefully in pediatric population. This article reviews the most recent data on SRL application in the field of pediatric renal tx.

Resolution of chyloperitoneum in a preterm with octreotide, diet and cessation of dialysis.

The diagnosis of chyloperitoneum (CP) is based on the presence of high levels of triglycerides (TGs) in the dialysate. It is a rare complication of peritoneal dialysis (PD) and even rarer in neonates. We report here the case of CP in a 1700-g male baby delivered at the 30th gestational week due to posterior urethral valve and associated oligohydramnios. On postnatal day 2, the serum creatinine (Scr) was 1.6 mg/dL, and he was anuric. PD was instituted via a Tenckhoff catheter. At the end of the second week, after the initiation of enteral feeding, the ultrafiltrate became cloudy, with a leukocyte count of 900/mm(3). A treatment regimen consisting of intraperitoneal vancomycin and ceftazidime was then started. Five days later, the fluid became milky, with a TG level of 251 mg/dL. The patient was then placed on a diet based on medium-chain triglycerides and octreotide (1 microg/kg/h; increasing up to 2 microg/kg/h over 15 days). Although the TG and leukocyte levels decreased, the milky appearance persisted. PD was stopped for 2 days when the Scr decreased to 1.7 mg/dL. When it was resumed, the fluid was totally clear, with a TG level of 7 mg/dL. The infant was discharged with a nightly intermittent peritoneal dialysis program and has had no recurrence. In summary, we report a preterm infant who developed CP during PD and recovered following treatment that included diet modification, octreotide, and temporary discontinuation of the PD.

Acute kidney injury following hypokalemic rhabdomyolysis: complication of chronic heavy cola consumption in an adolescent boy.

A 16-year-old boy presented with acute kidney injury (AKI) which was attributed to chronic heavy cola consumption. Habitual heavy cola ingestion might lead to hypokalemic rhabdomyolysis by its glycyrrhizin content. AKI has been described rarely in association with this clinical picture. It is important for physicians to keep heavy cola and other soft drink consumption in mind as a cause for hypokalemic rhabdomyolysis and AKI.

Juvenile linear scleroderma with unique forms of renal involvement.

Linear scleroderma is the most common type of juvenile localized scleroderma, which usually involves the limbs. The linear lesions may extend through the dermis, subcutaneous tissue, muscles, and the underlying bone, leading to significant functional and cosmetic deformities. Renal involvement in juvenile localized scleroderma is a rare manifestation. Here we report two cases with juvenile linear scleroderma and unique renal manifestations, including unilateral renal arterial stenosis and immunoglobulin M nephropathy.

WITHDRAWN: TRANSIENT TACHYPNEA OF THE NEWBORN: A PREDICTIVE FACTOR FOR PROLONGED TACHYPNEA.

Ahead of Print article withdrawn by publisher.

New quantitative parameters for evaluating radionuclide cystography and their value in understanding the physiology of reflux.

UNLABELLED: In this descriptive study of radionuclide cystography, time-activity curves were generated from renal pelves with reflux and evaluated to reveal the physiology of the reflux. The generated new parameters were also evaluated for any correlation with the frequency of urinary tract infections and renal scarring. METHODS: Thirty-two children with reflux to the renal pelvis (36 refluxing units) were included. Regions of interest were drawn on the pelves and bladder, and time-activity curves were generated. The first reflux phase, the bladder volume at first reflux, and the bladder volume at maximal reflux were defined, and reflux percentages were calculated. Dimercaptosuccinic acid (DMSA) scintigraphy findings and urinary tract infections were used for correlation. RESULTS: New curves from the renal pelvis during bladder filling and bladder emptying were generated and their patterns classified. Episodic reflux to the pelvis was observed on time-activity curves, which demonstrated 4 different emptying patterns during voiding. These patterns were described in detail. Visually, residual activity was present in the renal pelves in 58.8% of patients. The mean number of urinary tract infections per year was 1.07 (range, 0-4; SD, 0.88). The DMSA findings were normal in 57.1% of the patients. CONCLUSION: We obtained and categorized some new time-activity curve patterns from renal pelves with reflux on radionuclide cystography. Discussing these patterns may help physicians understand the physiology of the reflux and the relationship between infections and reflux.

The relation of IgM deposition to clinical parameters and histomorphometry in childhood mesangial proliferative glomerulonephritis.

We investigated the question of whether IgM deposition causes any difference in the histomorphometry of children with mesangial proliferative glomerulonephritis (MePGN). We retrospectively compared the urinalysis, blood pressure measurements, and serum creatinine levels of children with IgM (+) and IgM (-) MePGN. For histopathological evaluation, the outlines of Bowman's capsules and glomerular tufts of 10 glomeruli were rimmed manually, and the surrounded areas were calculated as pixels. The cells at each tuft of the largest four glomeruli were counted. Bowman's capsular areas, glomerular tuft areas, glomerular cellularities, and the number of cells per tuft area were determined for each patient. There were 24 (M/F: 14/10) patients with MePGN. Twelve of them were IgM (+). Mean age at biopsy was 6.8+/-4.1 years. IgM (+) patients mostly presented with nephrotic syndrome (NS) (n=9) and IgM (-) patients mostly with macroscopic hematuria (n=7). None of the clinical or laboratory findings was statistically different between the groups (p>0.05) except increased NS frequency in IgM (+) patients (p: 0.038). The histomorphometric parameters were similar in both groups (p>0.05). IgM (+) patients with MePGN mostly presented with NS in our study, but their histomorphometric parameters demonstrated no significant difference.

Growth in familial mediterranean fever: effect of attack rate, genotype and colchicine treatment.

We evaluated the effect of attack frequency, homozygosity for the M694V mutation and colchicine treatment on growth in children with familial Mediterranean fever (FMF). Prepubertal patients with FMF (19 M, 14 F) were evaluated retrospectively for height SDS, weight SDS and body mass index (BMI) before and after 46.2 +/- 39.8 months of colchicine therapy. Pretreatment attack frequency and acute phase markers at diagnosis were also recorded. While acute phase markers were not correlated to anthropometric variables, attack rate was negatively, albeit insignificantly, correlated to height and weight SDS. Height SDS did not change, while BMI showed a slight but significant increase during colchicine therapy (16.2 +/- 2.6 to 17.3 +/- 3.1 kg/m2, p = 0.035). Homozygosity for M694V did not affect time from the onset of symptoms to diagnosis, anthropometric variables and acute phase markers. In conclusion, pre-treatment attack rate and anthropometric development correlated negatively. Colchicine therapy improved BMI slightly, but significantly. Homozygosity for M694V had no effect on anthropometric development.

Schimke immunoosseous dysplasia: suggestions of genetic diversity.

Schimke immunoosseous dysplasia (SIOD), which is characterized by prominent spondyloepiphyseal dysplasia, T-cell deficiency, and focal segmental glomerulosclerosis, is a panethnic autosomal recessive multisystem disorder with variable expressivity. Biallelic mutations in switch/sucrose nonfermenting (swi/snf) related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like 1 (SMARCAL1) are the only identified cause of SIOD. However, among 72 patients from different families, we identified only 38 patients with biallelic mutations in the coding exons and splice junctions of the SMARCAL1 gene. This observation, the variable expressivity, and poor genotype-phenotype correlation led us to test several hypotheses including modifying haplotypes, oligogenic inheritance, or locus heterogeneity in SIOD. Haplotypes associated with the two more common mutations, R820H and E848X, did not correlate with phenotype. Also, contrary to monoallelic SMARCAL1 coding mutations indicating oligogenic inheritance, we found that all these patients did not express RNA and/or protein from the other allele and thus have biallelic SMARCAL1 mutations. We hypothesize therefore that the variable expressivity among patients with biallelic SMARCAL1 mutations arises from environmental, genetic, or epigenetic modifiers. Among patients without detectable SMARCAL1 coding mutations, our analyses of cell lines from four of these patients showed that they expressed normal levels of SMARCAL1 mRNA and protein. This is the first evidence for nonallelic heterogeneity in SIOD. From analysis of the postmortem histopathology from two patients and the clinical data from most patients, we propose the existence of endophenotypes of SIOD.

The role of calcium utilization of intestinal flora in urinary calcium excretion.

OBJECTIVE: We aimed to evaluate whether Ca(+2) utilization of intestinal flora (IF) has an effect on urinary excretion of Ca(+2) (UCaE) levels. MATERIALS AND METHODS: Fecal samples (0.1 g/mL) of children who underwent UCaE examination in the past year were implanted in broths. Labeled (45)Ca (5 muL) was added to the samples and incubated. From these samples, a 200-muL quantity was filtrated with a 0.45-micrometer membrane and was rinsed in 200 muL pure water. (45)Ca activity in the membrane was measured and defined as percent activity per bacteria ((45)Ca(act) %/CFU). Levels of aerobic and anaerobic (45)Ca(act) %/CFU and their correlations with UCaE were compared between hypercalciuric (Group I) and normocalciuric (Group II) patients. RESULTS: Levels of (45)Ca %/CFU were similar between groups (P > .05). Aerobic and anaerobic (45)Ca(act)%/CFU levels were not significantly correlated to UCaE, either in normocalciuric (P = .079, r = -0.503; P = .260, r = -0.420, stray mart respectively) or in hypercalciuric children (P = .509, r = 0.223; P = .623, r = -0.257, respectively). CONCLUSION: Similar (45)Ca(act)%/CFU levels in the 2 groups imply that calcium utilization of IF does not have a distinct effect on UCaE.

Rhabdomyolysis with different etiologies in childhood.

AIM: To investigate different etiologies and management of the rhabdomyolysis in children. METHODS: Eight pediatric rhabdomyolysis cases who applied to the Dokuz Eylul University Faculty of Medicine Department of Pediatric Nephrology with different etiologies between January 2004 and January 2012 were evaluated in terms of age, gender, admission symptoms, physical examination findings, factors provoking rhabdomyolysis, number of rhabdomyolysis attacks, laboratory results, family history and the final diagnosis received after the treatment. RESULTS: Average diagnosis ages of eight cases were 129 (24-192) ± 75.5 mo and five of them were girls. All of them had applied with the complaint of muscle pain, calf pain, and dark color urination. Infection (pneumonia) and excessive physical activity were the most important provocative factors and excessive licorice consumption was observed in one case. In 5 cases, acute kidney injury was determined and two cases needed hemodialysis. As a result of the further examinations; the cases had received diagnoses of rhabdomyolysis associated with mycoplasma pneumoniae, sepsis associated rhabdomyolysis, licorice-induced hypokalemic rhabdomyolysis, carnitine palmitoyltransferase II deficiency, very long-chain acyl-CoA dehydrogenase deficiency, congenital muscular dystrophy and idiopathic paroxysmal rhabdomyolysis (Meyer-Betz syndrome). CONCLUSION: It is important to distinguish the sporadic and recurrent rhabdomyolysis cases from each other. Recurrent rhabdomyolysis cases should follow up more regardful and attentive.

An adolescent girl with Meyer-Betz syndrome.

Idiopathic paroxysmal rhabdomyolysis indicating a classical triad of symptoms consisting of muscle pain, weakness, and discolored urine is known as "Meyer-Betz syndrome". It may result in acute renal failure due to precipitation of the myoglobin casts in the tubuli or to the direct toxic effects of myoglobin to the tubular epithelium. On the other hand, outcome may be uneventful. In this study, we reported the case of a 16-year-old girl who was admitted with red-colored urine after a slight exertion. She had tenderness and weakness in upper parts of her legs and bilateral flank pain. She had a positive urine dipstick test for heme despite absent red cells on microscopic examination. White cell count, liver function tests, serum creatine kinase (CK), lactate dehydrogenase (LDH), and urine myoglobin levels were raised. All metabolic tests were in normal ranges and EMG was normal. A muscle biopsy performed after recurrent exertional rhabdomyolysis attacks demonstrated normal findings and ruled out metabolic disorders. At the time of attacks, hydration along with alkalinization was applied and she did not experience renal failure. She was advised to avoid strenuous physical exertion and had an uneventful outcome for the last 5 months. We reported the clinical course and follow-up of an adolescent girl with Meyer-Betz syndrome.

Sarcoidosis with an uncommon presentation: apropos of a case.

A 17-year-old male presenting with chronic renal failure whose supporting clinical manifestations of the disease had appeared independently over a four-year period is reported. The renal biopsy specimen of the patient revealed tubulointerstitial nephritis and membranous glomerulonephritis. He never had hilar adenopathy, but maculopapular rashes, erythema nodosum, arthritis, chronic lymphocytopenia, hepatomegaly, splenomegaly, and lymphadenomegaly had been observed at different periods over four years. The presence of non-caseating granulomatous lesions in the liver biopsy accompanying uveitis verified the diagnosis of sarcoidosis. Low dose steroid was applied to this hepatitis-C carrier, and uveitis was suppressed. No recurrence has been observed in two-year follow-up.

A case of acute post-streptococcal glomerulonephritis that developed posterior reversible encephalopathy syndrome.

A 10-year male patient presented with swelling in the face, legs and scrotal area which developed 8 days after tonsillitis treatment. Acute post-sterotococcal glomerulonephritis (APSGN) was considered in the patient whose urinalysis revealed hematuria and proteinuria at nephrotic level, whose urea, creatinine, lipid profile and anti-streptolysine O antibody levels were increased, albumin and C3 value were decreased and whose 24-hour urine test revealed proteinuria. Renal biopsy was found to be compatible with APSGN. In the follow-up, severe headache, vomiting and convulsion were observed under antihypertensive and diuretic treatment and when the blood pressure was 130/80 mmHg (the 99(th) percentile for the patient: 129/88 mmHg). During the follow-up, the blood pressure values increased to 160/90 mmHg. The electroencephalogram (EEG) performed was found to be normal and magnetic resonance imaging (MRI) findings were compatible with posterior reversible encephalopathy syndrome (PRES). MRI was found to be normal at the first month following antihypertensive and anticonvulsive treatment. In the first year of the follow-up, the blood pressure, neurological examination and urinalysis findings were found to be normal. This patient was presented to draw attention to the fact that PRES can also present with a blood pressure tending to increase and with blood pressure values which are not so high.

Chronic kidney disease in an adolescent with hyperuricemia: familial juvenile hyperuricemic nephropathy.

Chronic kidney disease (CKD) is a life-long condition associated with substantial morbidity and premature death due to complications from a progressive decrease in kidney function. Especially in children, early diagnosis and detection of the etiologic factors are important to improve their health outcomes. Familial juvenile hyperuricemic nephropathy (FJHN) is a rare autosomal-dominant disorder characterized by hyperuricemia with renal uric acid under-excretion and CKD. Genetic studies have revealed mutations in the uromodulin (UMOD) gene. Highlighting the importance of CKD in children, a 14-year-old girl with the rare diagnosis of FJHN is reported herein.

The relationships between renal compensatory hypertrophy etiologic factors and anthropometric development in the pediatric age group.

OBJECTIVE: To evaluate the factors associated with compensatory hypertrophy in the functional kidneys of children. METHODS: The medical files of patients with a solitary functional kidney were reviewed retrospectively. Data regarding anthropometric measurements, functional renal length, functional renal length of standard deviation score (SDS) of functional kidney at diagnosis, and end of follow-up were obtained. Patients were divided into 2 groups, those with a unilateral kidney function of <10% (hypoplasia, dysplasia, and atrophy, group 1) and those with a solitary kidney (agenesis, and multicystic dysplastic kidney, group 2). RESULTS: A total of 126 patients (70 boys) were evaluated. Both the sizes of the functional kidney and functional kidney SDS values at diagnosis were greater in group 1 relative to group 2. At the end of the follow-up period, anthropometric values including functional kidney size were higher in group 2. Functional kidney size of 2 SDS above the normal was mostly predictive at age 17.5 months (odds ratio [OR] 5.06) and at a body height of 82 cm (OR 5.57). CONCLUSION: The most determining factors for renal length SDS values were age and height. Solitary kidneys complete compensatory hypertrophy by 17.5 months of age, and after that their growth continues in parallel to normal growth.