Bone mineral density in Egyptian children with familial mediterranean fever

Background: Familial Mediterranean fever [FMF] has episodic or subclinical inflammation that may lead to a decrease in bone mineral density [BMD]. The objective of this study was to assess BMD in Egyptian children with FMF on genetic basis

Methods: A cross sectional study included 45 FMF patients and 25 control children of both sexes in the age range between 3-16 years old. The patients were reclassified into two groups, namely group I[A] with 23 cases using colchicine for 1 month or less, and group I[B] with 22 cases using colchicine for more than 6 months. For both the patients and control groups, MEFV mutations were defined using molecular genetics technique and BMD was measured by DXA at the proximal femur and lumbar spines

Results: Four frequent gene mutations were found in the patient group E148Q [35.6%], V726A [33.3%], M680I [28.9%], and M694V [2.2%]. There were also four heterozygous gene mutations in 40% of the control children. Patients receiving colchicine treatment for less than 1 month had highly significant lower values of BMD at the femur and lumbar spines than the control children [P=0.007, P<0.001]. Patients receiving colchicine treatment for more than 6 months had improved values of BMD at femur compared with the control, but there were still significant differences between them in lumbar spine [P=0.036]. There were insignificant effect of gene mutation type on BMD and the risk of osteopenia among the patients

Conclusion: FMF had a significant effect on BMD. However, regular use of colchicine treatment improves this effect mainly at the femur

value of insulin-like growth factor binding protein-3 in growth assessment of juvenile rheumatoid arthritis patients

This study was carried out on 40 children having juvenile rheumatoid arthritis [JRA], their mean age was 8.5 +/- 2.8 with a range of 3-18 years. Two hundred and ninety healthy children, age and sex matched with the patients, were included as controls. History, clinical examination and full anthropometry were performed. The basal levels of insulin like growth factor 1 [IGF1] and IGF binding protein 3 [IGFBP3] were assessed. Growth hormone assessment was performed in patients with compromised growth and in those having low IGF1 values. Mean height standard deviation score [SDS] of the patients was significantly lower compared with the controls. Mean IGF1 and IGFBP3 values as well as their Z scores were significantly lower in patients than in controls whether pre or post pubertal. IGF1 Z scores were positively correlated to IGFBP3 Z score in a statistically significant manner. IGF1 Z score was also positively correlated to height SDS and negatively correlated to steroid therapy. The group of patients who received glucocorticoids was shorter, showed less IGF1 Z score and IGFBP3 Z score than the group who did not receive steroid therapy. GH stimulation tests in patients with compromised growth showed normal values [mean +/- SD was 21.0 +/- 5.7 ng/ml, with a range of 10-30]. IGF1 and IGFBP3 Z scores were less in the group with compromised growth than in that with fair growth [-2.40 + 1.58 and -2.34 + 1.85 versus -1.41 + 0.86 and -1.11 + 1.49, respectively] and the difference was statistically significant