The autosomal recessive multicentric osteolytic disorders of childhood-Torg, Winchester, and François syndromes-predominantly affect the carpal, tarsal, and interphalangeal joints, and their progressive bone loss and crippling arthritic deformities mimic severe juvenile rheumatoid arthritis. In a consanguineous Saudi Arabian family two affected sibs with facial anomalies and short stature displayed a distal arthropathy of the metacarpal, metatarsal, and interphalangeal joints starting in the first few months of life that eventually progressed to the proximal joints and resulted in crippling ankylosis and severe generalized osteopenia. Facial changes included proptosis, a narrow nasal bridge, bulbous nose, and micrognathia. In addition, they had large, painful fibrocollagenous palmar and plantar pads and mild body hirsutism. Affected individuals were of normal intelligence and had normal renal function. Routine hematologic, chemistry, and rheumatoid studies were within normal limits. Histologic examination of bone marrow and an interphalangeal joint biopsy were not informative. The autosomal recessive inheritance, clinical, and radiologic characteristics of the affected sibs suggested that they had a form of multicentric osteolysis most closely resembling the Torg syndrome, but with a unique facial appearance, fibrocollagenous pads, and body hirsutism not noted in the original description of the syndrome.
Objective: Screening of kidney diseases by urinalysis in school children has been approved in many parts of the world with inexpensive tools such as urinary dipsticks. In this study, we investigated the prevalence of hematuria in a large sample of 6 to 15-year-old school students in Makkah and Baha, Saudi Arabia. Design: A total population of 12,347 students in primary and middle schools in Makkah and Baha, Saudi Arabia, were investigated for hematuria during 2007. There were 7,299 students in Makkah (5,007 boys and 2,292 girls) and the remaining 5,048 were in Baha (2,734 boys and 2,314 girls), aged from 6 to 15 years old. All the studied children were apparently healthy and asymptomatic. Parents' consents were taken prior to the test. A random fresh mid-stream urine was collected to complete the urine analysis (by dipstick and microscopy) to detect microscopic hematuria, albuminuria or pus cells. Measurements of the height and weight were also done and blood pressure was measured for each student. Results: Among the 12,347 students screened and urine samples provided, 2,745 (22.23%) had hematuria, 1,822 (14.76% of the total population) from Makkah and 923 (7.48%) from Baha. Analysis of the prevalence of hematuria in students in Makkah and Baha showed that there was a very highly significant difference between the two cities (P value = 0.0000). There were 842 students (7% of the total population) had positive hematuria and hypertension and 2,678 students (22%) had negative hematuria and hypertension with a highly statistically significant difference between positive hematuria students and negative students in the prevalence of hypertension (P value = 0.0044). Conclusion: Asymptomatic hematuria might be detected by the school screening program and should be considered as an inexpensive way for early management of some renal diseases.
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