Giant cell arteritis in a 12-year-old girl presenting with nephrotic syndrome.

Giant cell arteritis (GCA) is rare in children. The kidneys are generally spared. We present a case of GCA in a 12-year-old girl with severe headache and tender scalp especially over the right temporal area. The right superficial temporal artery was cord like and nodular and the pulsations were barely felt. Several small tender nodular swellings were felt in the occipital area. She had been previously diagnosed as a case of nephrotic syndrome due to underlying membranoproliferative glomerulonephritis. This report is aimed at drawing attention to this rare form of vasculitis in children aiming at decreasing its morbidities.

Screening for B- and T-cell defects in Egyptian infants and children with suspected primary immunodeficiency.

BACKGROUND: Primary immunodeficiency diseases are underdiagnosed in developing countries. The aim of this study was to identify primary B- and T-cell immune defects in Egyptian infants and children with clinical criteria indicating primary immunodeficiency disease. MATERIAL/METHODS: We enrolled 100 consecutive infants and children clinically suspected to have primary immunodeficiency disease. Subjects were evaluated with respect to immunodeficiency-related score, complete blood count, erythrocyte sedimentation rate, serum immunoglobulin (Ig) A and tetanus IgG antibody estimation, and Candida and tuberculin intradermal testing. Subjects showing IgA deficiency underwent serum total IgG, IgM, and IgE measurement, and lymphopenic patients underwent lymphocyte subset counting by flow cytometry. RESULTS: Thirty-five subjects showed laboratory evidence of T- and/or B-cell immunodeficiency and showed significantly higher immunodeficiency-related scores, a greater frequency of hospitalization, and were more likely to have no bacillus Calmette-Guérin scar and a negative Candida skin test. Laboratory evaluation revealed evidence of predominant B-cell defects in 19 subjects, T-cell defects in 8, and combined immunodeficiency in 8. An immunodeficiency-related score of > or =6 was associated with 71% of the immune defects, and a score > or =8 was predictive of significant immune derangement. The most helpful screening tests for B-cell defects were those for serum IgA and antitetanus IgG, whereas those for T-cell immunodeficiency were peripheral blood lymphocyte count and Candida sensitivity skin test. CONCLUSIONS: Our screening procedures allowed for identification of most of the subjects requiring further evaluation of primary immunodeficiency disease in this study. Widescale screening of neonates and older children for primary immunodeficiency disease is indicated.

Serum melatonin in juvenile rheumatoid arthritis: correlation with disease activity.

The study was conducted to investigate the abnormalities in early morning serum melatonin among patients with Juvenile Rheumatoid Arthritis (JRA) and to outline its relation to disease activity and severity. Twenty one patients with JRA and twenty healthy age and sex matched controls were enrolled in the study. Fifteen patients had polyarticular JRA, 3 had oligoarticular and 3 had systemic onset JRA. Evaluation was carried out clinically, functionally and radiologically by using disease activity score, Juvenile Arthritis Functional Assessment Report for Children (JAFAR-C score) and modified Larsen score, respectively. Laboratory investigations included Complete Blood Picture (CBC), The Erythrocyte Sedimentation Rate (ESR), C-Reactive Protein (CRP), classic IgM Rheumatoid Factor (RF), Anti-nuclear Antibodies (ANA) and melatonin estimation in serum. The serum levels of melatonin were significantly increased in JRA patients (mean +/- SD = 13.9 +/- 8 pg mL(-1)) as compared to healthy controls (mean +/- SD = 8.1 +/- 2.7 pg mL(-1), p < 0.01). A significant positive correlation could link serum melatonin levels to disease activity scores and ESR (r = 0.91, p < 0.001 and r = 0.55, p < 0.01, respectively). No significant correlation was found between melatonin and either Larsen or JAFAR scores (r = 0.19, r = 0.15, respectively). According to melatonin levels, there were 2 groups of patients: Group I with elevated melatonin level (more than 11 pg mL(-1)) (n = 15) and group II with normal melatonin level (less than 11 pg mL(-1)) (n = 6). Patients with elevated melatonin levels had higher ESR (p < 0.05), higher disease activity scores (p < 0.01) and Larsen scores (p < 0.05), than the group of patients with normal serum melatonin. The results of GAFAR scores were comparable between the two groups (p > 0.05). Hence the study conclude that the elevated melatonin levels among JRA patients with active synovitis and its close relation to disease activity rather than disease severity suggests that melatonin might play a promoting role in rheumatoid arthritis. Hence, inhibition of its synthesis and/or action by specific antagonists may be of therapeutic value.