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A total of 66 children and adolescents with chronic kidney disease (CKD) (20 pre-dialysis patients and 46 chronic dialysis patients) were evaluated to address the prevalence of abnormalities in glucose and insulin metabolism and their association with cardiovascular disease. Glucose intolerance was assessed using an oral glucose tolerance test; insulin resistance was estimated by the homeostasis model assessment of insulin resistance (HOMA-IR). Carotid artery intima-media thickness (IMT) and left ventricular hypertrophy (LVH) were examined as early markers of cardiovascular disease. Thirty-four patients (7 pre-dialysis, 27 dialysis) exhibited an abnormal glucose tolerance; however, ten patients (7 pre-dialysis, 3 dialysis) were insulin-resistant. Height-specific standard deviation scores of carotid artery IMT were above normal in three of the pre-dialysis patients (15%) and in 34 of the dialysis patients (74%). LVH was present in seven pre-dialysis (35%) and 34 dialysis patients (74%). In addition, two of the pre-dialysis patients (10%) and 18 of the dialysis patients (39%) had severe LVH. The prevalence of both increased carotid artery IMT and LVH were similar in patients with or without glucose intolerance in both groups, but severe LVH was more prevalent in pre-dialysis patients with glucose intolerance (p = 0.042). The multivariate analyses showed that neither carotid artery IMT nor LVM index was predicted by serum glucose levels or HOMA-IR. In conclusion, children with CKD are at a high risk of glucose intolerance and also have a greater risk of subclinical cardiovascular disease (CVD). However, the presence of glucose intolerance does not appear to be an independent risk factor for increased carotid artery IMT or LVH.

To determine the incidence of latent tuberculosis infection and evaluate the follow-up protocol of the patients diagnosed with juvenile idiopathic arthritis (JIA) and other chronic rheumatologic diseases treated with anti-TNF-α treatment (etanercept, infliximab, adalimumab) in Turkey, 144 patients were evaluated retrospectively for the development of tuberculosis. Patients were evaluated every 6 months for tuberculosis using history, physical examination, tuberculin skin test (TST), chest radiographs, and, when required, examination of sputum/early morning gastric aspirates for acid-fast bacilli and chest tomography. A tuberculin skin test over 10 mm induration was interpreted as positive. Patients were diagnosed with JIA (n = 132), enthesitis-related arthritis (ERA; n = 14), juvenile psoriatic arthritis (JPsA; n = 4), chronic idiopathic uveitis (n = 4), and chronic arthritis related to FMF (n = 8). Mean age was 12.25 ± 3.96 years (4.08-19.41 years), mean duration of illness was 5.86 ± 3.77 years (0.66-15 years), and the mean duration of anti-TNF-α treatment was 2.41 ± 1.47 years (0.6-7 years). Anti-TNF-α agents prescribed were etanercept (n = 133), infliximab (n = 30), and adalimumab (n = 6). When unresponsive to one anti-TNF-α therapy, patients were switched to another. There was no history of contact with individuals having tuberculosis. During follow-up, seven patients (4.8%) with positive TST were given INH prophylaxis. One oligoarticular JIA patient (0.69%) diagnosed with secondary uveitis who had been followed for 5 years and had been using infliximab for 2 years, developed a positive Quantiferon-TB test while on INH prophylaxis. He was started on an anti-tuberculosis drug regimen. In conclusion, anti-TNF-α treatment in children with chronic inflammatory disease is safe. Follow-up every 6 months of children on anti-TNF-α treatment with respect to tuberculosis by the pediatric infectious disease department is important to prevent possible complications.

The aim of the study was to determine the economical impact of juvenile idiopathic arthritis (JIA) and familial Mediterranean fever (FMF) in Turkey. A total of 100 patients (69 F/31 M) with JIA and 100 with FMF (68 F/32 F) who were consecutively seen in the outpatient clinic of the pediatric rheumatology department at Cerrahpasa Medical School between August 2008 and January 2009 were studied. Cost data were collected through a questionnaire filled out by the parents. The mean age (JIA: 11 +/- A 5 years; FMF:12 +/- A 4 years) and mean disease duration (JIA:5 +/- A 3 years; FMF: 4 +/- A 3 years) of the patients were similar. JIA patients were assigned to 5 subtypes (polyarticular: n = 45, oligoarticular: n = 30, systemic onset: n = 13, psoriatic: n = 6, and enthesopathy-related JIA: n = 6). Forty-nine percent of the patients with JIA were treated with anti-TNF drugs and 61% with DMARDs. All patients with FMF were using colchicine. The total annual cost of JIA (a,not sign3,994 +/- A 4,101) was considerably higher than that of FMF (a,not sign162 +/- A 77) (P < 0.001). Medication fee was the major determinant of total costs in both diseases constituting 85% in JIA and 39% in FMF. Among the subtypes of JIA, total annual costs were the highest among patients with polyarticular type (a,not sign6,045 +/- A 4,078). Medications especially anti-TNF drugs were the major contributor among all determinants of costs in JIA. The low costs of health care system and prominent changes in the health care policies for the last 5 years in Turkey might have played role in our findings.

The aim of this study was to evaluate longitudinal changes in dialysate cancer antigen 125 (dCA125) levels over time and to analyze relationships between dCA125 and peritoneal glucose exposure (PGE) in children undergoing long-term peritoneal dialysis (PD). The study group included seven boys and four girls (mean age 13 ± 5.1 years) with a mean PD duration of 84.0 ± 1.1 months. A peritoneal equilibration test (PET) was performed, and dCA125 levels were measured in all patients. Peritoneal appearance rates (AR) of dCA125, the velocity of the decrease in dCA125AR values, and annual PGE levels were also calculated. The final tests were performed at a mean of 63.3 ± 3.5 months after the initial ones. Both dCA125 and dCA125AR levels showed statistically significant decrements during the follow-up period (p = 0.003), with the velocity of decrease in dCA125AR found to be 52.6 ± 19.4%. There were no significant differences in peritoneal transport parameters between the beginning and end of the study period. PGE values were significantly higher in the last year of the study than in the first year (p = 0.014), but the velocity of the decrease in dCA125AR levels was not related to total PGE. In conclusion, a significant decline was found in dCA125 and CA125 AR levels, reflecting mesothelial cell mass, in children undergoing long-term PD (>5 years), but these were not related to PGE.

We report a rare case of polyarteritis nodosa (PAN) presenting in childhood. The child had multiple visceral aneurysms and later developed ascending colitis and jejunitis. The diagnosis was established with multidetector CT and CT angiography.

Coronary artery calcification (CAC) is common in adults with chronic kidney disease (CKD) and progresses with time. However, data are limited for younger patients. We have previously reported CAC in eight of 53 children with CKD. After 2 years, CAC evaluation was repeated in 48 patients. The median CAC score (CACS) increased from 101.3 (1473.6 +/- 1978.6, range 8.5-4332) to 1759.2 (2236.4 +/- 2463.3, range 0-5858) Agatston units (AU). When the individual changes in CACS were evaluated one by one, we showed a mild decrease in two patients on hemodialysis (HD) and in one transplant (Tx) recipient, a moderate increase in one patient on HD, one on peritoneal dialysis (PD) and one Tx recipient, and a large increase in one HD patient. Also, CAC disappeared in one HD patient. All patients with no calcification at baseline remained calcification-free at follow-up. To obtain the individual cumulative exposure, we calculated time-averaged mean values, using the laboratory values from the beginning of dialysis to the first and second multidetector spiral computed tomography (MDCT) scans (baseline and final values, respectively). Final CACS was positively related to final calcium-phosphorus (CaxP) product, while CAC progression was inversely associated with final serum albumin level. This report is the first study with the largest number and the youngest cohort to document the natural history of coronary calcification.

Cardiovascular complications are common in patients with end-stage renal disease (ESRD). We aimed to investigate left ventricular (LV) function and carotid intima-media thickness (cIMT) in children and adolescents with ESRD. This study included 38 ESRD patients (15 hemodialysis and 23 peritoneal dialysis) and 17 age- and sex-matched healthy subjects. The ESRD patients had significantly lower mean mitral E/A ratio, and higher left ventricular mass index (LVMI) and cIMT than the control group. Compared with PD patients, HD patients had worse LV diastolic function. In stepwise linear regression analysis, LVMI (P = 0.043) and hemoglobin (P = 0.015) turned out to be independent variables for predicting diastolic dysfunction (reduced E/A ratio), and the only significant predictor of cIMT was indexed diastolic blood pressure (DBP) (P = 0.035). Cardiovascular structure and function abnormalities are also common in pediatric dialysis patients, as in adults. Furthermore our data indicated that hemodialysis was disadvantageous for preserving LV diastolic function as compared with peritoneal dialysis.

The aim of our study was to show how the progression and severity of Familial Mediterranean Fever (FMF) is affected by procoagulant activity and alterations in the markers of thrombosis and fibrinolysis. The study cohort comprised 64 FMF patients who were classified as attack-free patients (Group 1; n = 34 patients, aged 3-19 years) and attack patients (Group 2; n = 30 patients, aged 3-21 years). All patients were on colchicine treatment with the exception the newly diagnosed patients in Group 2. A total of 14 healthy subjects between 5-12 years of age were enrolled as controls (Group 3). Laboratory tests, including leukocyte and thrombocyte counts, erythrocyte sedimentation rate, CRP, fibrinogen, PT, aPTT, Factor VIII, vW factor, D-dimer, P-selectin, tPA and PAI-1, were carried out on all patients. Inflammation continued both during the attack and attack-free period in FMF. The prolongation of PT was observed during attacks (PT = 13.6 s in Group 2, and PT = 12.6 s in Group 3; p = 0.002). tPA levels increased in FMF patients (tPA levels of group 1, 2 and 3 were 12.6, 13.2 and 9.7 ng/ml, respectively; p = 0.01). P-selectin was lower in both patient groups than in the control group. During attack periods PAI-1 levels increased (PAI-1 level of Group 1: 89.6 ng/ml and PAI-1 level of Group 2: 335.7 ng/ml, p = 0.000). Inflammation with increased acute phase reactants continued during both attack and attack-free periods in FMF patients. Prolongation of PT and differences in tPA and P-selectin levels suggest that hypercoagulability may have a role in the etiopathogenesis of FMF. It may be possible to use PAI-1 as a marker for the attacks of FMF.

Coronary artery calcification (CAC) is common in adults with end-stage renal disease (ESRD), but little is known about the prevalence and the extent of it in children. We used multidetector spiral computed tomography (MDCT), echocardiography, and carotid and brachial high-resolution ultrasonography to screen for the presence and predisposing factors of CAC in 53 children with ESRD [15 hemodialysis (HD) patients, 24 peritoneal dialysis (PD) patients, and 14 renal transplant (rTx) recipients]. CAC was present in 15% of patients (three HD patients, three PD patients, and two rTx). The mean age of the patients with CAC was 16.4 years (range: 11.0–21.2 years), and their median CAC score was 101.3, ranging from 8.5 to 4,322 according to the Agatston method. The patients with CAC had longer duration of total dialysis (P=0.005), had higher time-integrated serum phosphorus (P

This prospective study was conducted to assess dialysate cancer antigen 125 (dCA125) levels in pediatric peritoneal dialysis (PD) patients and to investigate whether it exhibits any alterations during or after recovery from peritonitis, and also to analyze the relationships between dCA125 and age, duration of PD treatment, peritonitis incidence, time passed since the last episode of peritonitis, PD prescription parameters, and peritoneal transport parameters. Forty-seven standardized 4-h peritoneal equilibration tests (PET) were performed in 38 children (mean age 11.6+/-4.7 years) on PD (duration 20.9+/-14.3 months). Thirty-two of the patients were in stable condition at the time of PET (stable group). Six patients were included in the study only during a peritonitis episode, and two of the stable patients were reevaluated during a peritonitis episode afterwards (peritonitis group). Seven out of a total of eight patients with acute peritonitis were reexamined after recovery (recovery group). CA125 levels were measured in 188 samples at 0-, 1-, 2-, and 4-h dwells. Peritoneal appearance rates (AR) were calculated. Mean dCA125 (4 h) and AR CA125 values were 5.6+/-5.3 U/ml [median 4.15 U/ml/4 h (range 0.5-25.9)] and 50.1+/-45.6 U/min/1.73 m2[median 37.91 U/ml/1.73 m2 (range 3.61-223.39)]. AR CA125 levels did not correlate with age, PD duration, peritonitis incidence, time passed since the last peritonitis episode, exchange volume used per m2 per day, or peritoneal transport properties in the stable patients' group. Although stable patients using hypertonic PD solutions (n=16) had slightly lower AR CA125 levels (p=0.04), multivariate analysis showed no influence of hypertonic dextrose solutions on mesothelial CA125 secretion (p=0.4). During acute peritonitis, CA125 concentrations showed a reversible threefold increase [AR CA125: stable 37.9 vs. peritonitis 101.2 U/ml/1.73 m2 (p=0.001)]. No difference could be found between the stable group and the recovery group. We conclude that changes in the peritoneal mesothelial cell mass cannot be assessed by determining CA125 in a cross-sectional way and that longitudinal determinations could be more valuable in the follow-up of patients.

The aim of this study was to investigate the adequacy of the following criteria for the classification of juvenile-onset spondyloarthropathies (JSpA): European Spondyloarthropathy Study Group criteria (ESSGCr) and Amor criteria (ACr) (proposed for adult SpA), Garmisch-Partenkirchen (G-PCr), seronegative enthesopathy and arthropathy syndrome (SEACr), and atypical spondyloarthropathies classification criteria (ASpCr) (proposed for JSpA). Sixty-two patients with JSpA (48 male and 14 female) and 64 with juvenile idiopathic arthritis (27 male and 37 female) (excluding enthesitis-related and psoriatic arthritis) were enrolled in the study group. Twenty-nine of the patients with JSpA were diagnosed with definite JSpA and the remaining 35 with undifferentiated JSpA. One hundred six patients in the study group were evaluated by one investigator, who was unaware of the diagnosis, according to the following: ESSGCr, ACr, G-PCr, ASpCr, and SEACr. Analysis of the patients diagnosed with JSpA showed 83.9%, 82.3%, 95.2%, 61.3%, and 62.9% sensitivity and 87.5%, 95.3%, 78.1%, 98.4%, and 92.2% specificity for the ESSGCr, ACr, G-PCr, ASpCr, and SEACr sets, respectively. None of the criteria evaluated above is sufficient for the classification of JSpA. There is a definite need for a new set of criteria with high specificity and sensitivity for early recognition and classification.

Results There was no statistically significant difference in between the disease subtype groups and between the pain groups as to the sociodemographic features. The frequency of depression and anxiety diagnosis which were made according to the DSM-IV criteria was %31 and %17.7 in 45 JIA patients. For the 45 patients the average CDI score was 7.60 and the average Trait Anxiety Inventory score was 34.31. For the 42 patients which were aloocated into pain groups, the average CDI score was 7.67 and the average Trait Anxiety Inventory score was 34.10. There was no statistically significant difference between the groups as to the depression and anxiety scale scores. Pain was not correlated with depression and anxiety scores.

A 10-year-old-boy with acute acalculous cholecystitis due to polyarteritis nodosa of the gallbladder with limited involvement of this organ is reported. It is suggested that a search for other lesions is indicated when polyarteritis nodosa of the gallbladder has been diagnosed, but that no treatment is indicated when additional lesions are not found.