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.

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.

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.

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.

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.

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.

Protective effects of bilirubin in an experimental rat model of pyelonephritis.

OBJECTIVE: To investigate the effects of bilirubin in a rat model of pyelonephritis. METHODS: Experimental pyelonephritis was induced in 32 wistar rats and 4 groups were formed: group 1 (no treatment), group 2 (antibiotic), group 3 (bilirubin), and group 4 (antibiotic + bilirubin). Antibiotic was given on days 3 to 8, and bilirubin was administered between days 0 and 8 of bacterial inoculation. Half of the rats were killed on the 9th day (early period) and histopathological parameters, immunohistochemical renal fibrosis markers, apoptosis, and oxidant/antioxidant system activities were evaluated. The rest of the rats were killed at the 6th week of the study and evaluated for histopathological parameters and renal fibrosis markers. RESULTS: Inflammatory activity was significantly lower in rats treated with antibiotic + bilirubin vs no treatment group both in the early and late periods. In the late period, inflammatory cell intensity was lower in rats treated with bilirubin vs no treatment and the antibiotic + bilirubin groups. Interstitial fibrosis/tubular atrophy was lower in the antibiotic + bilirubin group vs the no treatment and antibiotic groups, and in bilirubin vs antibiotic group. Tissue inhibitor of metalloproteinase-1 expression was lower in the bilirubin vs antibiotic group. Terminal deoxynucleotidyl transferase mediated 2'-deoxyuridine, 5'-triphosphate nick end labeling(+) cells were significantly lower in bilirubin and antibiotic + bilirubin groups vs no treatment group. Malondialdehyde levels were significantly lower in the antibiotic + bilirubin vs the no treatment group and superoxide dismutase activity was significantly higher in the antibiotic and antibiotic + bilirubin groups vs the no treatment group. CONCLUSION: Bilirubin may have protective effects on pyelonephritis-associated inflammation in both early and late periods in addition to fibrosis and apoptosis when applied with antibiotics. When used alone, bilirubin may also prevent inflammation (in the late period) and apoptosis.

Two adolescent girls with keratinizing squamous metaplasia of the bladder.

Squamous metaplasia is a proliferative lesion characterized by the replacement of the transitional epithelium with stratified squamous cells. In the urinary system, it is mostly seen in the bladder. It can be nonkeratinized or keratinized. We report the cases of 2 adolescent girls with keratinizing metaplasia, 1 of whom presented with difficulty with indwelling catheterization and 1 with final terminal hematuria. The predisposing factors were recurrent urinary tract infection and additional catheterization in 1 of the patients. The diagnosis was confirmed by histologic examination in both patients. We report on these cases to draw attention to this rare entity in children.

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.

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.

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.

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

Ahead of Print article withdrawn by publisher.

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.

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.

Skin and muscle involvement as presenting symptoms in four children with familial Mediterranean fever.

Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterized by recurrent attacks of fever and polyserositis. It is the most frequent periodic fever syndrome. In FMF, sterile peritonitis, pleuritis and arthritis are frequently seen in addition to recurrent febrile attacks. Skin and muscle involvement is less common. Here, we report four patients presented with skin lesions or myalgia. Most striking findings in those patients are the absence of other major criteria for FMF and dominancy of skin lesions or myalgia. All four patients had MEFV gene mutations on both alleles. In patients with erysipelas-like lesions or erythema nodosum along with arthritis/arthralgia or recurrent myalgia, FMF should be kept in mind.

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.

Protective effect of Epo on oxidative renal injury in rats with cyclosporine nephrotoxicity.

The aim of our study was to determine the effect of recombinant human erythropoietin (rhEPO) on cyclosporine (CsA) nephrotoxicity. Twenty-six female Wistar rats were injected with 15 mg/kg subcutaneous CsA and intraperitoneal saline/rhEPO for 28 days. Four groups were formed: Group 1 (n = 5), a control group; Group 2 (n = 7), CsA + saline; Group 3 (n = 7), CsA + low dose (20 U/kg per day) rhEPO; Group 4 (n = 7), CsA + high dose (100 U/kg per day) rhEPO. Body weights, creatinine clearance, urinary protein/creatinine, hematocrit, serum creatinine levels, histopathological parameters, apoptosis and lipid peroxidation tests were compared between the three groups. Body weights and renal functions were similar in Groups 2, 3 and 4 rats but significantly lower than the values found for the control group at the end of the study. The hematocrit was significantly different between the four groups, showing a positive association with the strength of the injected rhEPO doses. Tubular and arteriolar damage was significantly lower in Groups 3 and 4 rats than in Group 2 rats, while chronic changes were similar between the three groups. TUNEL-positive cells and thiobabarbituric acid reacting substance (TBARS) levels were significantly higher in Group 2 rats, whereas superoxide dismutase levels were significantly lower in Group 2 rats than in those of the other three groups. Low or high dose rhEPO had no significant protective effects on body weight, renal functions, chronic fibrotic changes, but both doses reduced tubular and arteriolar changes, apoptotis and oxidative stress.