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.

Evaluation of non-infectious complications of peritoneal dialysis in children: a multicenter study.

BACKGROUND: Peritoneal dialysis (PD) is the most common kidney replacement therapy in children. Complications associated with PD affect treatment success and sustainability. The aim of this study was to investigate the frequency of PD-related non-infectious complications and the predisposing factors. METHODS: Retrospective data from 11 centers in Turkey between 1998 and 2018 was collected. Non-infectious complications of peritoneal dialysis (NICPD), except metabolic ones, in pediatric patients with regular follow-up of at least 3 months were evaluated. RESULTS: A total of 275 patients were included. The median age at onset of PD and median duration of PD were 9.1 (IQR, 2.5-13.2) and 7.6 (IQR, 2.8-11.9) years, respectively. A total of 159 (57.8%) patients encountered 302 NICPD within the observation period of 862 patient-years. The most common NIPCD was catheter dysfunction (n = 71, 23.5%). At least one catheter revision was performed in 77 patients (28.0%). Longer PD duration and presence of swan neck tunnel were associated with the development of NICPD (OR 1.191; 95% CI 1.079-1.315, p = 0.001 and OR 1.580; 95% CI 0.660-0.883, p = 0.048, respectively). Peritoneal dialysis was discontinued in 145 patients; 46 of whom (16.7%) switched to hemodialysis. The frequency of patients who were transferred to hemodialysis due to NICPD was 15.2%. CONCLUSIONS: Peritoneal dialysis-related non-infectious complications may lead to discontinuation of therapy. Presence of swan neck tunnel and long duration of PD increased the rate of NICPD. Careful monitoring of patients is necessary to ensure that PD treatment can be maintained safely.

Association of procalcitonin with acute pyelonephritis and renal scars in pediatric UTI.

BACKGROUND AND OBJECTIVE: Urinary tract infections (UTIs) are common childhood bacterial infections that may involve renal parenchymal infection (acute pyelonephritis [APN]) followed by late scarring. Prompt, high-quality diagnosis of APN and later identification of children with scarring are important for preventing future complications. Examination via dimercaptosuccinic acid scanning is the current clinical gold standard but is not routinely performed. A more accessible assay could therefore prove useful. Our goal was to study procalcitonin as a predictor for both APN and scarring in children with UTI. METHODS: A systematic review and meta-analysis of individual patient data were performed; all data were gathered from children with UTIs who had undergone both procalcitonin measurement and dimercaptosuccinic acid scanning. RESULTS: A total of 1011 patients (APN in 60.6%, late scarring in 25.7%) were included from 18 studies. Procalcitonin as a continuous, class, and binary variable was associated with APN and scarring (P < .001) and demonstrated a significantly higher (P < .05) area under the receiver operating characteristic curve than either C-reactive protein or white blood cell count for both pathologies. Procalcitonin ≥0.5 ng/mL yielded an adjusted odds ratio of 7.9 (95% confidence interval [CI]: 5.8-10.9) with 71% sensitivity (95% CI: 67-74) and 72% specificity (95% CI: 67-76) for APN. Procalcitonin ≥0.5 ng/mL was significantly associated with late scarring (adjusted odds ratio: 3.4 [95% CI: 2.1-5.7]) with 79% sensitivity (95% CI: 71-85) and 50% specificity (95% CI: 45-54). CONCLUSIONS: Procalcitonin was a more robust predictor compared with C-reactive protein or white blood cell count for selectively identifying children who had APN during the early stages of UTI, as well as those with late scarring.

Procalcitonin is a predictor for high-grade vesicoureteral reflux in children: meta-analysis of individual patient data.

OBJECTIVE: To assess the predictive value of procalcitonin, a serum inflammatory marker, in the identification of children with first urinary tract infection (UTI) who might have high-grade (≥3) vesicoureteral reflux (VUR). STUDY DESIGN: We conducted a meta-analysis of individual data, including all series of children aged 1 month to 4 years with a first UTI, a procalcitonin (PCT) level measurement, cystograms, and an early dimercaptosuccinic acid scan. RESULTS: Of the 152 relevant identified articles, 12 studies representing 526 patients (10% with VUR ≥3) were included. PCT level was associated with VUR ≥3 as a continuous (P = .001), and as a binary variable, with a 0.5 ng/mL preferred threshold (adjusted OR, 2.5; 95% CI, 1.1 to 5.4). The sensitivity of PCT ≥0.5 ng/mL was 83% (95% CI, 71 to 91) with 43% specificity rate (95% CI, 38 to 47). In the subgroup of children with a positive results on dimercaptosuccinic acid scan, PCT ≥0.5 ng/mL was also associated with high-grade VUR (adjusted OR, 4.8; 95% CI, 1.3 to 17.6). CONCLUSIONS: We confirmed that PCT is a sensitive and validated predictor strongly associated with VUR ≥3, regardless of the presence of early renal parenchymal involvement in children with a first UTI.

Prediction of high-grade vesicoureteral reflux after pediatric urinary tract infection: external validation study of procalcitonin-based decision rule.

BACKGROUND: Predicting vesico-ureteral reflux (VUR) ≥3 at the time of the first urinary tract infection (UTI) would make it possible to restrict cystography to high-risk children. We previously derived the following clinical decision rule for that purpose: cystography should be performed in cases with ureteral dilation and a serum procalcitonin level ≥0.17 ng/mL, or without ureteral dilatation when the serum procalcitonin level ≥0.63 ng/mL. The rule yielded a 86% sensitivity with a 46% specificity. We aimed to test its reproducibility. STUDY DESIGN: A secondary analysis of prospective series of children with a first UTI. The rule was applied, and predictive ability was calculated. RESULTS: The study included 413 patients (157 boys, VUR ≥3 in 11%) from eight centers in five countries. The rule offered a 46% specificity (95% CI, 41-52), not different from the one in the derivation study. However, the sensitivity significantly decreased to 64% (95%CI, 50-76), leading to a difference of 20% (95%CI, 17-36). In all, 16 (34%) patients among the 47 with VUR ≥3 were misdiagnosed by the rule. This lack of reproducibility might result primarily from a difference between derivation and validation populations regarding inflammatory parameters (CRP, PCT); the validation set samples may have been collected earlier than for the derivation one. CONCLUSIONS: The rule built to predict VUR ≥3 had a stable specificity (ie. 46%), but a decreased sensitivity (ie. 64%) because of the time variability of PCT measurement. Some refinement may be warranted.

Urinary uric acid : creatinine ratios in healthy Turkish children.

BACKGROUND: Determining uric acid : creatinine ratios in random urine samples may be useful to assess the excretion of uric acid in children. Because it was shown that urinary uric acid excretion varies with age and geographic area, it is important to have accurate reference values of uric acid excretion. The aim of the present study was therefore to obtain regional reference values for urinary uric acid : creatinine ratios in healthy Turkish children. METHODS: A total of 1306 children aged 1 month-15 years were analyzed for uric acid and creatinine, and urinary uric acid : creatinine ratios were determined from each sample. The second non-fasting morning urine samples were taken from all the children. Urine samples were analyzed for uric acid using the uricase method, and for creatinine with the Jaffe reaction. RESULTS: The mean +/- SD and 5th-95th percentiles of urinary uric acid : creatinine ratios (mg/mg) were 1.09 +/- 0.48 and 0.27-1.87 at 1-6 months, 0.86 +/- 0.41 and 0.19-1.64 at 7-12 months, 0.76 +/- 0.32 and 0.32-1.43 at 1-3 years, 0.63 +/- 0.29 and 0.20-1.23 at 4-6 years, 0.44 +/- 0.24 and 0.14-0.93 at 7-11 years, and 0.30 +/- 0.14 and 0.12-0.62 at 12-15 years. Uric acid : creatinine ratios were not significantly different between boys and the girls except at 12-15 years. Girls aged 12-15 years had higher urinary uric acid : creatinine ratio when compared with boys (P < 0.05). There was no correlation between urinary uric acid : creatinine ratio and protein intake. CONCLUSIONS: Urinary uric acid : creatinine ratio changes with age. When assessing urinary uric acid : creatinine ratio, the clinician should consider the age of the child.

Pediatric urolithiasis: an 8-year experience of single centre.

OBJECTIVE: The objective was to investigate the clinical features and metabolic and anatomic risk factors for kidney stone formation in our patient group. METHODS: Between 1998 and 2005, 179 children (94 girls, 85 boys) followed in our department because of urolithiasis were enrolled to participate in our study. Clinical presentation, urinary tract infection, stone localisation, positive family history, stone composition, presence of anatomic abnormalities and urinary metabolic risk factors, and treatment modality were evaluated retrospectively. RESULTS: The mean age at diagnosis of stone disease was 4.5 years (range 0.25-15.3 years). The mean follow-up duration was 8 months (range 1-98). The major clinical presentations of our patients were abdominal pain and/or flank pain in 100 children (55.9%) and macroscopic hematuria in 25 (14%). Urinary tract infection was detected in 20% of patients on admission. Forty-three children (24%) had a urinary tract abnormality and ureteropelvic junction obstruction was the most common abnormality. A family history of stone disease was recorded in 98 patients (54.7%). Stones were located within the renal parenchyma in 90 patients. Hypercalciuria and hyperuricosuria were detected in 42.3 and 54.8% respectively. Stone analysis was performed in 63 children and calcium oxalate was a major mineral. Surgical treatment was performed in 49 children and extracorporeal shock wave lithotripsy (ESWL) in 41 children. CONCLUSION: We think that urolithiasis remains a serious problem in children in our country. Family history of urolithiasis, urologic abnormalities (especially under the age of 5 years), metabolic disorders and urinary tract infections tend to indicate childhood urolithiasis.

Plasma ghrelin levels in various stages of development of iron deficiency anemia.

OBJECTIVES: Ghrelin stimulates food intake and induces metabolic changes leading to an increase in body weight and body fat mass. Iron-deficiency anemia (IDA) is the most frequently seen cause of nutritional anemia, that is a type of starvation. There is no available study related to levels of ghrelin in IDA. The aim of this study is to show an association with ghrelin levels and iron deficiency and to demonstrate whether changes seen in iron deficiency (ID) are explained by ghrelin, as opposed to whether ghrelin levels correlate with ID. MATERIALS AND METHODS: The study group was consisted of children who were admitted in the outpatient clinic of pediatrics. Control group (C) was defined as cases with normal hemoglobin (Hb), serum iron (SI), transferrin saturation (TS), and ferritin (F) (>12 ng/mL) values; group hypoferritinemia (IDec) Hb: N, SI: N, TS: N, F<12 ng/mL; group iron deficiency (IDef), Hb: N, SI: decreased, TS

Proinflammatory cytokines and leptin are increased in serum of prepubertal obese children.

It has not yet been shown in prepubertal children how cytokines, leptin, and body mass, as well as parameters of obesity are interrelated. The aim of this study was to explore the relation between circulating levels of some cytokines with leptin and body mass index. A case control study was carried out in obese children of both sexes. An obese group was carried out with 63 school prepubertal children and a control group comprised the same number of nonobese children paired by age and by sex. Mean serum leptin concentration was significantly higher in the obese children at 19.9 +/- 7.4 ng/mL, than the control group (7.9 +/- 5.1 ng/mL). Serum IL-1beta, IL-6, and TNF-alpha levels were also significantly higher in the obese group than controls (33.0 +/- 8.9, 45.2 +/- 11.8, and 9.2 +/- 2.3 pg/mL, versus 3.6 +/- 1.0, 13.1 +/- 3.9, and 3.9 +/- 1.0 pg/mL, resp). In controversy, serum IL-2 level was diminished in the obese group as 0.4 +/- 0.1 versus 0.9 +/- 0.1 U/L. Obesity may be a low-grade systemic inflammatory disease. Obese prepubertal children have elevated serum levels of IL-1beta , IL-6, and TNF-alpha which are known as markers of inflammation.

Acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage associated with herpes simplex virus type I.

Acute hemorrhagic leukoencephalitis (AHL) is a rare and usually fatal disorder characterized by an acute onset of neurological abnormalities. It may occur in association with a viral illness or vaccination. Radiology and brain biopsy are essential for the diagnosis. We present a case of AHL mimicking or manifesting as intracerebral hemorrhage associated with herpes simplex virus. A 3-year-old boy was admitted to our hospital with cerebral hemorrhage. The findings of cerebrospinal fluid, MRI and electroencephalogram indicated acute hemorrhagic leukoencephalitis associated with herpes simplex virus involving the left parietal cortex (hemorrhage) and the right temporal lobe (encephalitis). The patient received intravenous dexamethasone (1.5 mg/kg/24 h divided q6 (six times a day) for 5 days, tapered slowly to discontinuation over the next 5 days) and aciclovir (15 mg/kg/every 6 h for 14 days). His clinical condition markedly improved, and after 3 weeks he was discharged from the hospital with moderate right hemiparesis. Mild hemiparesis with normal psychometric testing was determined when he was checked 6 months later. AHL remains misdiagnosed as viral encephalitis because it produces different protean clinical forms. Pediatric patients admitted with cerebral hemorrhage and infarct should be evaluated for presence of hemorrhagic encephalitis-causing agents, especially for herpes simplex. Since, as in our case, treatment with corticosteroid and acyclovir markedly improves conditions of herpes simplex-caused AHL, we suggest that early diagnosis and treatment is of importance for the prognosis.