90K immunostimulatory glycoprotein in children with juvenile idiopathic arthritis.

OBJECTIVES: To assess whether circulating levels of 90K glycoprotein are increased in children with juvenile idiopathic arthritis (JIA) at different stages of the disease, compared to healthy controls and to evaluate potential over time changes in its concentrations following treatment with the antitumor-necrosis factor (TNF) drug etanercept. METHODS: 90K glycoprotein, C-reactive protein, erythrocyte sedimentation rate, TNF, antinuclear antibodies, rheumatoid factor and the Juvenile Arthritis Disease Activity Score were assessed in 71 children: 23 with newly diagnosed JIA, 23 with established and active JIA and 25 healthy controls. Patients, eligible for anti-TNF treatment, underwent a similar clinical/laboratory assessment after 6- and 12-month etanercept therapy. RESULTS: At baseline, significant differences were found in 90K levels between the three study groups: JIA at onset (157.7 [131.4-241.5] µg/ml), JIA on treatment (90.0 [68.8-120.2] µg/ml) and control group (58.0 [44.5-79.0] µg/ml), (p for trend <.001), with the JIA at onset group showing the highest values. In the JIA on treatment group, following one-year etanercept treatment, a significant reduction in 90K was detected already at 6 months (74.3 [56.0-104.1] µg/ml p = .001) and a further decline was observed at 12 months (49.3 [46.0-67.6] µg/ml p < .001). CONCLUSION: Our study showed that 90K glycoprotein levels are increased in JIA children compared to healthy controls, suggesting a potential pathogenetic role in the JIA. Besides, 12 months of therapy with etanercept can reduce 90K levels.

Clinical practice guidelines: what they are, why we need them and how they should be developed through rigorous evaluation.

The number of available clinical practice guidelines has grown enormously in the recent years, therefore requiring a correct approach and use of them. We present a revision of what guidelines are and serve, how to correctly develop and find them, and how to develop and evaluate them through rigorous scientific methods. Limits and benefits of guidelines are also discussed. An overview about the use of paediatrics' guidelines is finally reported.

Role of urinary NGAL and KIM-1 as biomarkers of early kidney injury in obese prepubertal children.

Objectives Childhood obesity is an important cause of end-stage renal disease. To date, available markers do not characterize kidney changes, especially in the early stages. kidney injury molecule-1 (KIM-1) and neutrophil gelatinase-associated lipocalin (NGAL) are already detected before the onset of proteinuria or alterations of glomerular filtration rate and thus might represent biomarkers that directly reflect kidney injury. Methods We characterize kidney injury in a group of 40 obese-prepubertal children compared to 29-healthy age- and gender matched-peers. Anthropometric measurements and body composition were determined. Fasting blood samples were collected for measurement of insulin, glucose, lipid profile, transaminases, cystatin C and creatinine. Urine samples were collected to assess urinary NGAL, KIM-1 and urinary isoprostanes. Kidney length was measured with ultrasound evaluation. Differences between the two groups were evaluated by Mann-Whitney U test, and Spearman correlation analysis was used to explore relationship between variables. Results Triglycerides, alanine transaminase (ALT), glucose, insulin, homeostasis model assessment insulin resistance, triglycerides/high-density lipoprotein (HDL)-cholesterol ratio and cystatin C values were significantly higher in obese children than normal weight peers. Creatinine values were normal and similar between the two groups, while isoprostanes were higher in obese. Obese children had larger kidney sizes, indicating organ hypertrophy. NGAL and KIM-1 were increased in obese children compared to controls. A significant association between NGAL and KIM-1 with adiposity indices, insulin status and markers of oxidative stress postulated a possible effect of obesity in inducing kidney abnormalities. KIM-1 and NGAL are directly related respectively to cystatin C and isoprostanes, supporting the ability of these biomarkers in reflecting early kidney damages in obese subjects. Conclusions These findings suggest that obese subjects exhibit a certain degree of renal damage before kidney function loss.

Protein-losing enteropathy in an infant with rotavirus infection.

Protein-losing enteropathy (PLE) is a rare gastro-intestinal complication characterised by intestinal loss of proteins with consequent hypoproteinaemia and generalised oedema. Rotavirus infection associated with PLE in children has rarely been reported. A 6-month-old girl presented with diarrhoea, fever and generalised oedema. Total serum proteins were 34 g/L (61-79) and plasma albumin 16.8 g/L (40-50), serum sodium was 126 mmol/L and there was mild metabolic alkalosis (pH 7.46). Stool for alpha-1 antitrypsin was >1.2 mg/g (<0.6) which supported the diagnosis of PLE. Stool examination demonstrated the presence of rotavirus antigen by the rapid immunochromatographic test. Abdominal ultrasound showed bowel distension and intestinal wall thickening with a small amount of ascites. Echocardiography excluded pericardial effusion. Two albumin infusions (1 g/kg) were required to sustain normal serum albumin levels. Over the next 2 weeks, there was gradual normalisation of stools and progressive reduction of oedema. In children with acute and symptomatic PLE, rotavirus should be considered in the differential diagnosis. The availability of the rapid immunochromatographic test facilitates the diagnosis. In most cases, supportive care alone is sufficient, but albumin infusions may be required in more severely affected children.