Study of serum syndecan-1 levels in a group of Egyptian juvenile systemic lupus erythematosus patients.

The aim of the study was to assess the serum levels of Syndecan-1 in a group of Egyptian juvenile systemic lupus erythematosus (JSLE) patients and to study any possible associations with disease activity, renal activity and organ damage. Serum level of Syndecan-1 was assessed in 60 Egyptian JSLE patients and 30 apparently healthy age and gender matched children using ELISA. SLE Disease Activity Index-2000 (SLEDAI-2K), renal SLEDAI-2K, renal activity score and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index were assessed for all patients. Serum SDC-1 levels were higher in patients with JSLE than in healthy controls (p<0.001) and were positively correlated with SLEDAI-2K (p<0.001), with renal SLEDAI score (p=0.008) and renal activity score (p=0.04). So, Syndecan-1 might be used as a marker for disease activity and renal activity in JSLE patients.

Detection of Mediterranean fever gene mutations in Egyptian children with inflammatory bowel disease.

AIM: The aim of the current study is to investigate the prevalence of familial Mediterranean fever gene (MEFV) mutations in a cohort of Egyptian children with inflammatory bowel disease (IBD), and to characterize familial Mediterranean fever (FMF)-IBD patients, helping better understanding of IBD pathogenesis. METHODS: The study enrolled 17 patients with ulcerative colitis (UC), 15 with Crohn's disease(CD), 10 with indeterminate colitis (IC) and 33 healthy children as controls. All cases and controls were tested for 12 FMF gene mutations by reverse hybridization after multiplex polymerase chain reaction amplification and DNA sampling. RESULTS: Eighty-eight percent of the IBD patients carried the mutations, with Sequence variant V627A being the commonest versus 42.4% of controls. No associations were found between MEFV gene mutations, and phenotypic characteristics of IBD patients. CONCLUSION: IBD patients, in populations with a high background carrier rate of MEFV variants, should be screened for MEFV gene mutations, especially those diagnosed as indeterminate colitis. Testing larger numbers of healthy Egyptian children for MEFV gene mutation is important to further determine the allele frequency in Egypt.

Renal involvement in childhood-onset systemic lupus erythematosus in Egypt.

Lupus nephritis has been described as the most serious complication of systemic lupus erythematosus (SLE) and the strongest predictor of poor outcome. While the incidence of childhood SLE is relatively low, renal involvement appears to be more common and more severe in childhood SLE. This study aims to characterize the features and outcome of renal involvement in childhood-onset SLE based on a study of 100 Egyptian patients (mean age at diagnosis 10.1 years, range 2-17 years). Initial data regarding disease manifestations and biopsy findings were reviewed. Disease activity was assessed using SLEDAI scores. Follow-up data (mean duration 6 years) were noted regarding specific treatment, response, complications and renal survival. Initial renal involvement was present in 78 patients, including 66 with hypertension and 23 with renal impairment. Pathologically, class IV nephropathy was found in 18 patients, class V in 9 and low-grade lesions (class II-III) in 49. Twenty patients required follow-up biopsy, and all transformations were observed. SLEDAI scores significantly decreased from initial (mean ± SD) of 21.4 ± 7.3 to 13.4 ± 7.8, in association with response to therapy (P < 0.0001). Poor response was associated with initial hypertension and renal impairment but not with initial SLEDAI score or pathological class. The projected renal survival was 82.4 and 64.7% 5 and 10 years from diagnosis. Early renal involvement in childhood SLE is common, serious and requires proper evaluation and management.

Systemic lupus erythematosus in Egyptian children.

The aim of the study was to study the characteristics of systemic lupus erythematosus (SLE) in the Egyptian population, comparing it to other populations. We retrospectively studied 207 patients with SLE diagnosed between 1990 and 2005. We obtained clinical features and laboratory data and analyzed them statistically. We studied 151 female and 56 male SLE patients. The female to male ratio was 2.7 to 1 and the mean age at presentation was 10 +/- 2.7 years (range 2-16). The mean disease duration was 6.47 +/- 3.74 years. At diagnosis, musculoskeletal, constitutional and mucocutaneous manifestations were the commonest features. During follow-up, the prevalence of nephritis (67%), hematological manifestations (44.9%), photosensitivity (44%), arthritis (39%), malar rash (38.2%), serositis (32.9%) and neuropsychiatric manifestations (24.25%) increased significantly. Those whose age of onset of the disease was