N-terminal pro-B-type natriuretic peptide diagnostic algorithm versus American Heart Association algorithm for Kawasaki disease.

Background: Diagnosis of Kawasaki disease (KD) can be challenging in the absence of a confirmatory test or pathognomonic finding, especially when clinical criteria are incomplete. We recently proposed serum N-terminal pro-B-type natriuretic peptide (NT-proBNP) as an adjunctive diagnostic test.

Methods: We retrospectively tested a new algorithm to help KD diagnosis based on NT-proBNP, coronary artery dilation (CAD) at onset, and abnormal serum albumin or C-reactive protein (CRP).

Hereditary Heterozygous C2 Deficiency: Variable Clinical and Serological Manifestations Among Three Sisters.

The causal link between inherited complement deficiencies and systemic lupus erythematosus (SLE) has been well established, although it remains a rare cause of the disease. We present the case of three biological sisters with hereditary heterozygous C2 deficiency, but who differ widely in their clinical and serological manifestations. Patient 1 is 25 years old and was diagnosed with SLE at the age of 12.

Rapidly progressive aortic aneurysmal dilation in a child with systemic lupus erythematosus: too early too severe.

About 10-20% of systemic lupus erythematosus cases occur in children, often with more severe features at onset and more active disease over time compared with adults. Cardiovascular complications are common in this population but thoracic aortic aneurysms rarely occur. Although the pathophysiology of this complication remains unclear, vasculitis seems to play an important role, leading to degeneration and fibrosis of the media and formation of the aneurysm.

Glucocorticoid-related changes in body mass index among children and adolescents with rheumatic diseases.

Objective: To examine the temporal and dose-related effects of glucocorticoids (GCs) on body mass index (BMI) in children with rheumatic diseases.

Methods: Children initiating GCs for a rheumatic disease (n = 130) were assessed every 3 months for 18 months. BMI, weight, and height Z score trajectories were described according to GC starting dosage in prednisone equivalents: high (≥1.

Value of amino-terminal pro B-natriuretic peptide in diagnosing Kawasaki disease.

Background: The aim of the present study was to investigate the diagnostic value of the N-terminal B-type natriuretic peptide (NT-proBNP) in acute Kawasaki disease (KD) given that the clinical criteria and the current basic laboratory tests lack the necessary specificity for accurate diagnosis.

Methods: Basic biological tests and serum NT-proBNP levels obtained from acute KD patients were compared to that of febrile controls. NT-proBNP was considered abnormal based on the following definitions: above a cut-off determined on receiver operator characteristic (ROC) analysis, above the upper limit for age, or above 2 SD calculated from healthy children.

Incident vertebral fractures among children with rheumatic disorders 12 months after glucocorticoid initiation: a national observational study.

Objective: To determine the frequency of incident vertebral fractures (IVF) 12 months after glucocorticoid (GC) initiation in children with rheumatic diseases and to identify children at higher risk.

Methods: Children with rheumatic diseases initiating GC were enrolled in a prospective observational study. Annual spine radiographs were evaluated using the Genant semiquantitative method.

High prevalence of primary immune deficiencies in children with autoimmune disorders.

Objectives: Autoimmunity is a well-recognised manifestation of primary immunodeficiency disorders. However, the prevalence of primary immunodeficiency among children with autoimmune diseases is not well characterised. The objective of this retrospective study was to describe the prevalence of primary immunodeficiency disorders in a paediatric population with autoimmune diseases.

Fatal group A Streptococcus purpura fulminans in a child receiving TNF-α blocker.

Inhibition of tumor necrosis factor alpha (TNF-α) is effective in the treatment of many pediatric autoimmune diseases and inflammatory conditions. Commonly available biologic agents blocking TNF-α are infliximab, etanercept, and adalimumab. These agents have changed the management of rheumatic diseases in the adult population and are being used more and more in pediatric patients as safety and efficacy have been demonstrated.

Early outcomes and improvement of patients with juvenile idiopathic arthritis enrolled in a Canadian multicenter inception cohort.

Objective: To determine early outcomes and early improvements in a prospective inception cohort of children with juvenile idiopathic arthritis (JIA) treated with current standard therapies.

Methods: Patients selected were enrolled in an inception cohort of JIA, the Research in Arthritis in Canadian Children Emphasizing Outcomes Study. The juvenile rheumatoid arthritis core criteria set measures were completed at enrollment and 6 months later.

Predictors of early inactive disease in a juvenile idiopathic arthritis cohort: results of a Canadian multicenter, prospective inception cohort study.

Objective: To determine early predictors of 6-month outcomes in a prospective cohort of patients with juvenile idiopathic arthritis (JIA).

Methods: Patients selected were those enrolled in an inception cohort study of JIA, the Research in Arthritis in Canadian Children Emphasizing Outcomes Study, within 6 months after diagnosis. The juvenile rheumatoid arthritis core criteria set and quality of life measures were collected at enrollment and 6 months later.

Natriuretic peptide as an adjunctive diagnostic test in the acute phase of Kawasaki disease.

Coronary arteritis rather than myocardial involvement is typically emphasized in Kawasaki disease (KD). Moreover, the criteria and the usual biological markers oversee the importance of cardiac-specific markers in diagnosing this disease. We sought to study the clinical usefulness of measuring B-type natriuretic peptide (BNP) and its N-terminal moiety (NT-proBNP) at the onset of KD.

Renal granuloma and immunoglobulin M-complex glomerulonephritis: a case of common variable immunodeficiency?

Common variable immunodeficiency (CVID) is characterized by reduced serum immunoglobulin levels and recurrent bacterial infections. Granulomatous infiltrations are occasionally found in the lymphoid or solid organs of affected patients, but renal involvement is rare. We present a case of possible CVID with interstitial noncaseating granuloma and immunoglobulin (IgM)-complex glomerulonephritis with a membranoproliferative pattern and with a favorable response to corticosteroids, intravenously administered immunoglobulins (IVIGs) and rituximab.