MRI findings of hypoxic cortical laminar necrosis in a child with hemolytic anemia crisis

European Radiology - We present magnetic resonance imaging findings of a 5-year-old girl who had a rapidly installing hemolytic anemia crisis induced by trimethoprim–sulfomethoxazole,...     

Economic impact of juvenile idiopathic arthritis and familial Mediterranean fever

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?±?5?years; FMF:12?±?4?years) and mean disease duration (JIA:5?±?3?years; FMF: 4?±?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 (€3,994?±?4,101) was considerably higher than that of FMF (€162?±?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 (€6,045?±?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.     

Coronary artery calcifications in children with end-stage renal disease

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<0.001), calcium-phosphate (CaxP) product (P=0.012), intact parathyroid hormone (P=0.010), vitamin B₁₂ levels (P=0.010), the amount of cumulative calcium-containing oral phosphate binders (OBPs) (P<0.001), and calcitriol intake (P<0.001), and had lower serum hemoglobin level (P=0.014). Interventricular septum systolic thickness (P=0.033) was significantly higher, relative wall thickness (P=0.062) tended to be higher, and flow-mediated endothelium-dependent dilatations (P=0.071) were lower without reaching statistically significant levels in those with CAC. A stepwise logistic regression analysis revealed that serum phosphorus (P=0.018) and the cumulative exposure to calcium-containing OPBs (P=0.016) were the most significant independent predictors in the development of CAC. These results indicate that even adolescents and children with ESRD may have coronary calcifications. We concluded that impaired divalent ion metabolism is the main factor in the formation of CAC in this age group.     

The Pediatric Rheumatology International Trials Organization criteria for the evaluation of response to therapy in juvenile systemic lupus erythematosus: prospective validation of the disease activity core set

OBJECTIVE: To validate and promulgate a core set of outcome measures for the evaluation of response to treatment in patients with juvenile systemic lupus erythematosus (SLE). METHODS: In 2001, a preliminary consensus-derived core set of measures for evaluating the response to therapy in juvenile SLE was established. In the present study, the core set was validated through an evidence-based, large-scale data collection process that led to the enrollment of 557 patients from 39 different countries. Consecutive patients with active disease were assessed at baseline and after 6 months. The validation procedures included assessment of feasibility, responsiveness, discriminant and construct ability, agreement in the evaluation of response to therapy between physicians and parents, redundancy, internal consistency, and ability to predict a therapeutic response. RESULTS: The following clinical measures were found to be feasible and to have good construct validity, discriminative ability, and internal consistency; furthermore, they were not redundant, proved responsive to clinically important changes in disease activity, and were associated strongly with treatment outcome and thus were included in the final core set: 1) physician's global assessment of disease activity, 2) global disease activity measure, 3) 24-hour proteinuria, 4) parent's global assessment of the patient's overall well-being, and 5) health-related quality of life assessment. CONCLUSION: The members of PRINTO propose a core set of criteria for the evaluation of response to therapy that is scientifically and clinically relevant and statistically validated. The core set will help standardize the conduct and reporting of clinical trials and assist practitioners in deciding whether a patient with juvenile SLE has responded adequately to therapy.     

MRI findings of hypoxic cortical laminar necrosis in a child with hemolytic anemia crisis

We present magnetic resonance imaging findings of a 5-year-old girl who had a rapidly installing hemolytic anemia crisis induced by trimethoprim-sulfomethoxazole, resulting in cerebral anoxia leading to permanent damage. Magnetic Resonance imaging revealed cortical laminar necrosis in arterial border zones in both cerebral hemispheres, ischemic changes in subcortical white matter of left cerebral hemisphere, and in the left putamen. Although cortical laminar necrosis is a classic entity in adulthood related to conditions of energy depletions, there are few reports available in children. A wide review of the literature is also presented.     

Acute phase response in familial Mediterranean fever

OBJECTIVE: To test the hypothesis that not all acute phase reactants respond in the same way during attacks of familial Mediterranean fever (FMF) and that there is a subclinical acute phase response (APR) in a proportion of patients during the interval between attacks. METHODS: Blood and urine samples were obtained from 49 patients with FMF during an attack and the attack-free period that followed, to test for erythrocyte sedimentation rate, C reactive protein (CRP), fibrinogen, white blood cell count, platelet count, factor VIII related antigen, haptoglobin, protein electrophoresis, ferritin, proteinuria, and haematuria. Control groups comprised 29 patients with juvenile idiopathic arthritis, 10 patients with various infectious diseases, and 19 healthy subjects. RESULTS: A marked APR was seen during the FMF attacks which was comparable with that obtained in the diseased control groups. CRP was the only acute phase protein that was raised during all attacks. Neither thrombocytosis nor an increase in ferritin levels (except one) was noted in any attack. Serum albumin levels remained unchanged. In two thirds of the patients with FMF a continuing APR was seen in between the attacks. CONCLUSION: Platelet, ferritin, and albumin responses are not part of the significant APR seen during short lived attacks of FMF, and inflammation continues in about two thirds of the patients during an attack-free period.