Canakinumab in patients with systemic juvenile idiopathic arthritis and active systemic features: results from the 5-year long-term extension of the phase III pivotal trials.

Objectives: To evaluate the long-term efficacy and safety of canakinumab in patients with active systemic juvenile idiopathic arthritis (JIA).

Methods: Patients (2-19 years) entered two phase III studies and continued in the long-term extension (LTE) study. Efficacy assessments were performed every 3 months, including adapted JIA American College of Rheumatology (aJIA-ACR) criteria, Juvenile Arthritis Disease Activity Score (JADAS) and ACR clinical remission on medication criteria (CR).

A propensity-matched comparison of hard outcomes in children on chronic dialysis.

Unlabelled: Data concerning outcomes of children on hemodialysis (HD) and peritoneal dialysis (PD) are scarce and frequently derived from single-center experiences. We sought to compare survival and transplantation rates in a large cohort of PD and HD patients. We extracted all patients initiating dialysis under 16 years of age between 2004 and 2013 from the Italian Registry of Pediatric Chronic Dialysis.

JAG1 Loss-Of-Function Variations as a Novel Predisposing Event in the Pathogenesis of Congenital Thyroid Defects.

Context: The pathogenesis of congenital hypothyroidism (CH) is still largely unexplained. We previously reported that perturbations of the Notch pathway and knockdown of the ligand jagged1 cause a hypothyroid phenotype in the zebrafish. Heterozygous JAG1 variants are known to account for Alagille syndrome type 1 (ALGS1), a rare multisystemic developmental disorder characterized by variable expressivity and penetrance.

[Osteomyelitis: a probable, uncommon etiology agent].

The relation of infectious agents to arthritis is an area of great interest to the rheumatologist. Septic arthritis of bacterial origin accounts for approximately 6.5% of all childhood arthritides.

High frequency of Merkel cell polyomavirus DNA in the urine of kidney transplant recipients and healthy controls.

Background: Polyomavirus (PyV) infection is common, ranging from 60% to 100% depending on the virus. The urinary excretion rates of JC virus (JCV) and BK virus (BKV) have been extensively studied, but less is known about the more recently discovered PyVs.

Objectives: To investigate the urinary excretion of Merkel cell PyV (MCPyV), which is associated with Merkel cell carcinoma (MCC), in kidney transplant recipients and healthy subjects, as well as those of lymphotropic polyomavirus (LPV), which was isolated from the B-cells of African green monkeys but has also been found in human blood, JCV and BKV.

Clinical dehydration and glomerular filtration rate in acute paediatric gastroenteritis.

Aim: To evaluate changes in glomerular filtration rate in acute gastroenteritis.

Methods: The correlation between two clinical diagnostic scales and glomerular filtration rate has been investigated in 113 children with acute gastroenteritis in a paediatric emergency setting.

Results: A significant reduction of GFR was found in 10% children less than, and 5% children higher than, 2 years of age with acute gastroenteritis.

A comprehensive cooperative project for children with renal diseases in Nicaragua.

In low-income countries renal diseases generally and chronic kidney disease (CKD) in particular represent a wide-spread and often underdiagnosed clinical problem. The aim of the cooperative project between the pediatric nephrology units of Milan, Italy, and Managua, Nicaragua was to improve the diagnosis and treatment of renal diseases and CKD in Nicaraguan children. When the project started, in 2000, there were many constraints in human and material resources in the Children's Hospital in Managua.

Renal vein obstruction and orthostatic proteinuria: a review.

Objectives: The cause of orthostatic proteinuria is not clear but may often relate to obstruction of the left renal vein in the fork between the aorta and the superior mesenteric artery (=renal nutcracker). However, reports dealing with proteinuria only marginally refer to this possible cause of orthostatic proteinuria. We analysed the corresponding literature.

Postural proteinuria associated with left renal vein entrapment: a follow-up evaluation.

Imaging studies show entrapment of the left renal vein in the fork between the aorta and proximal superior mesenteric artery in most cases of isolated postural proteinuria. Therefore, it has been postulated that partial obstruction to the flow in the left renal vein in the upright position is a cause of this form of proteinuria. In a girl with isolated postural proteinuria, kidney ultrasonic imaging and Doppler flow scanning showed left renal vein entrapment.

Superior palatability of crushed lercanidipine compared with amlodipine among children.

Aims: To compare the taste of equivalent doses of pulverized amlodipine and lercanidipine, two calcium channel blockers, among children with kidney disease.

Methods: Each child received a test dose of 1 mg of amlodipine besylate and 2 mg of lercanidipine in a single-blinded fashion. Children indicated their preference by pointing to the appropriate face on a visual analogue scale (VAS) that depicts five degrees of pleasure.

Assessment and monitoring of nutrition status in pediatric peritoneal dialysis patients.

Abnormalities of nutrition status are a common problem in children on peritoneal dialysis (PD) and a source of significant morbidity and mortality. The state of decreased body protein mass and fuel reserves (body protein and fat mass) common in PD patients is now better known as protein-energy wasting (PEW). Protein-energy wasting is a slow, progressive process in chronic kidney disease.

Left renal vein entrapment: a frequent feature in children with postural proteinuria.

In most Asian subjects with postural proteinuria, ultrasonic imaging and Doppler flow scanning disclose entrapment of the left renal vein in the fork between the aorta and the superior mesenteric artery. Little information is available on the possible occurrence of left venal rein entrapment in European subjects with postural proteinuria. Renal ultrasound with Doppler flow imaging was therefore performed on 24 Italian or Swiss patients with postural proteinuria (14 girls and ten boys, aged between 5.

[Hepatitis type A with atypical course].

Hepatitis A virus infection is usually asymptomatic in children. Classic symptomatic forms and atypical clinical manifestations are known. We report a paediatric case of hepatitis A with marked cholestasis, treated with steroids, and with an unusual prolonged course.

Knee synovectomy in children with juvenile idiopathic arthritis.

The purpose of the study was to assess the outcomes of knee synovectomies in children with juvenile idiopathic arthritis. Thirty-one arthroscopic synovectomies were performed in 19 children (six oligoarthritis, 20 polyarthritis, five psoriatic arthritis). The percentage of recurrence in the group with oligoarthritis was 67%, in the group with polyarthritis was 95%, whereas all psoriatic arthritis recurred.

Prevalence and outcome of juvenile idiopathic arthritis-associated uveitis and relation to articular disease.

Objective: To determine prevalence and complications of juvenile idiopathic arthritis (JIA)-associated uveitis, and to evaluate risk factors for uveitis and its relation to articular disease.

Methods: Records of 309 patients with JIA (244 female, 65 male, mean age at onset 4.9 yrs) were retrospectively reviewed.

[Arthritis and infections].

Arthritis caused by infectious agents can be secondary to direct invasion of the joint space or to immune mechanisms (subsequent to or concomitant to an infection). Septic arthritis refers to a situation when bacteria can be cultured in synovial fluid. Arthritis can complicate for example meningococcemia or infection by Neisseria gonorrhoeae or Haemophilus influenzae.

Clivus osteomyelitis secondary to Enterococcus faecium infection in a 6-year-old girl.

A 6-year-old girl was diagnosed to suffer from clivus osteomyelitis secondary to Enterococcus faecium infection. On the basis of the magnetic resonance image, the abscess was drained via the posterior wall of the pharyngeal tract immediately. Subsequent antibiotic therapy allowed rapid improvement and long-term healing of the osteomyelitic process without any side effect.

Anomalies of the kidney and urinary tract are common in de Lange syndrome.

Sixty-one patients affected by de Lange syndrome underwent a careful renal and urological evaluation including family and personal history, physical examination, urinalysis, renal tract ultrasonography, and serum creatinine. A voiding cystourethrography was performed in patients with urinary tract infections, in patients with renal ectopy, and in patients with small kidneys. Structural anomalies of the kidney and urinary tract were detected either by ultrasound or voiding cystourethrography in 25 patients (41%): absent or poor corticomedullary differentiation (N = 8; 13%), pelvic dilation (N = 6; 10%), vesicoureteral reflux (N = 5; 8%), small kidney (N = 3; 5%), isolated renal cyst (N = 3; 5%), and renal ectopia (N = 2; 3%).

Additional case of Tsukahara's syndrome or new syndrome: further delineation of the association of microcephaly and radio-ulnar synostosis.

In 1994, Giuffré et al. reported two unrelated families in which some of the members had microcephaly and radio-ulnar synostosis, suggesting a new condition. Since this first report, Tsukahara et al.

Proteinuria as a predictor of disease progression in children with hypodysplastic nephropathy. Data from the Ital Kid Project.

Little is known about the role of proteinuria in the progression of childhood renal diseases. We analyzed the decline in creatinine clearance ( C(Cr)) and kidney survival in 225 children (185 males) with chronic renal failure (CRF) due to isolated hypodysplasia or hypodysplasia associated with urological abnormalities. The data were based on the information available in the Italian Pediatric Registry of CRF (ItalKid Project), which includes patients from all of Italy aged <20 years with C(Cr )levels of <75 ml/min per 1.