Bone changes in sickle cell anaemia.

Analysis of available radiographs of the skeletal system in 50 patients suffering from sickle cell anaemia in the Assir Region (South Western height and valley) of the Kingdom of Saudi Arabia taken over a three year period showed a wide spectrum of bone changes. Both infarctions and medullary hyperplasia were common, producing the usual previously reported changes. Spinal changes, mostly osteoporosis and vertebral end plate depression were more prominent in the younger age group. Avascular necrosis of femoral head was common and occurred earlier than in previous reports in the literature. Humeral head and radial head necrosis were also recorded. The overall findings agree with recent reports from both the Eastern and Western regions of the Kingdom that bone changes in SCA are common and could be more severe than in other countries. Complications such as osteomyelitis and fractures were not common.

Urinary hydroxyproline and serum alkaline phosphatase in sickle cell disease.

Serum alkaline phosphatase, alkaline phosphatase isoenzymes, and urinary hydroxyproline excretion were studied in 20 young adult sickle cell patients and 58 matching normal controls. Total alkaline phosphatase was significantly higher in the sickle cell patients than in controls. Heat inactivation test and isoenzyme electrophoresis indicated that bone is the predominant isoenzyme in patients. Hydroxyproline excretion was significantly higher in the sickle cell patients than in controls. Serum total alkaline phosphatase correlated well with urinary hydroxyproline excretion in sickle cell patients (r = 0.73). Both alkaline phosphatase and hydroxyproline increased with age in the sickle cell patients. This study suggests that delayed growth and/or bone destruction may contribute to the elevated levels of alkaline phosphatase and urinary hydroxyproline.

Cerebrovascular accidents (strokes) in children with sickle cell disease residing at high and low altitudes of Saudi Arabia.

In a 3-year period, eight cases of cerebrovascular accident were seen out of 400 patients with sickle cell disease residing at high and low altitudes of Asir Province of Saudi Arabia. They were all Saudi nationals and comprised five boys and three girls of ages ranging from 3.5 to 9 years. They had varied clinical presentations of their cerebrovascular accident. Cranial computed tomography in seven of them showed abnormality in the region of the middle cerebral circulation. Management and some suggested factors contributing to the occurrence of the cerebrovascular accident are discussed.

Effect of dietary nitrogen on urinary excretion of non-protein nitrogen in adolescent sickle cell patients.

The nitrogen economy of sickle cell patients has been found to be FRpaired due to several factors including poor absorption of protein products and higher than normal urinary nitrogen excretion. The increased urinary loss of nitrogen is probably due to increased protein catabolism as a result of inadequate intestinal supply of nitrogen and its poor utilization. The relationship between urinary nitrogen excretion and dietary nitrogen intake was investigated. The results confirmed that there was higher than normal nitrogen excretion in the sicklers at all levels of nitrogen intake. Creatinine excretion, expectedly, was lower in the sicklers due to their smaller physical stature. Uric acid excretion was higher in the sicklers, and so was the mean urine acid to creatinine ratio (0.64 in the sicklers and 0.34 in the normals). The ratio was not affected by the diet, indicating that there was increased purine metabolism in the sicklers.

Total body water, total exchangeable sodium and related variables in the Ghanaian.

1. Standard radioisotope dilution techniques employing [3H]water and [22Na]sodium chloride have been used to determine the total body water and total exchangeable sodium of 20 male and 10 female normal Ghanaians (Africans) aged 19--25 years. 2. Lean body mass and total body fat are calculated as a percentage of body weight; the total exchangeable sodium values have been expressed in relation to lean body mass. 3. Comparison of the data for Ghanaian subjects with published figures for Caucasian subjects of similar age shows that the Ghanaian men have much less total body fat and the women a little less total body fat than their Caucasian counterparts. 4. Total exchangeable sodium expressed in terms of lean body mass shows close agreement in both men and women.