Jahed Hamdan

A Saudi child with homozygous sickle cell disease (SS) presented with bilateral periorbital swelling, right-sided proptosis, skull bone infarcts, and retro-orbital and epidural hematomas. The findings of skull bone infarcts, retro-orbital and epidural hematomas are rare in patients with sickle cell disease.

Comprehensive screening programs for congenital diseases of newborn infants are lacking at a national or regional level. A comprehensive newborn screening program modified to the needs and resources available was established in ARAMCO Dhahran Health Center. This program includes screening for congenital hypothyroidism, phenylketonuria, abnormal hemoglobins, glucose-6-phosphate dehydrogenase deficienc, and blood group incompatibilities. Several problems were encountered during the operation which required several modifications of the program. The organization and procedures of the program are described. Since the program was started in 1980, more than 70,000 newborn infants have been screened. Valuable epidemiological data have been collected and necessary information for direct clinical use was obtained. A national program to screen all neonates in the Kingdom of Saudi Arabia is achievable and urgently needed. Recommendations based on ARAMCO experience are given.

In a 5 1/2-year retrospective study of hospitalized children with osteomyelitis who had technetium bone scans, we found that 32 of 38 (84%) had increased radionuclide uptake, 4 (11%) had normal uptake and 2 (5%) had decreased uptake. The two patients with decreased uptake had a stormy course with extensive bone destruction and pathologic fractures in spite of prolonged courses of antibiotic therapy. Decreased uptake on bone scan in patients with clinical evidence of osteomyelitis could indicate a poor prognosis and early surgical drainage is indicated in order to decrease morbidity.