Sickle cell patients develop splenic dysfunction early in the course of their disease as shown by failure to visualize the organ on technetium‐99m colloid scintigraphy. However, preliminary studies from our center have shown that, when the spleen is not demonstrable on colloid uptake, it may be visualized on technetium‐99m heat‐denatured RBC scintigraphy. With time, however, the spleen can no longer be visualized with both tests in many patients. We have studied 46 patients aged 2 to 16 years, which included 36 SS, 7 Sβ0 thal, and 3 SD. Eighteen (39.1%) had normal splenic colloid uptake (Group 1), 15 (32.6%) had partial uptake (Group 2), and 13 (28.3%) had absent uptake (Group 3). When the patients in Group 1 were compared to those in the two other groups, there was no significant difference in the mean age and Hb F values. However, the mean Hb of 10.2 g/dl in Group 1 was significantly higher than the value of 9.0 g/dl in the other two groups. In addition, 60% of the SS patients with normal uptake and 40% of those with partial or absent uptake had co‐existing α‐thal trait; the difference in this proportion is significant (χ2 = 85, P < 0.0001). Heat‐denatured RBC scintigraphy was carried out on five patients in Group 2, and the spleen was visible in all, while of 12 children in Group 3, the spleen was visible only in 4 patients. This study demonstrates that the phagocytic function of the spleen, which is tested by colloid uptake, is the first to be lost while the filtration function, tested by denatured RBC uptake, persists for much longer. This finding may have significant implications for the clinical symptomatology and therapeutic strategies of sickle cell disease. Am. J. Hematol. 69:23‐27, 2002. © 2002 Wiley‐Liss, Inc.

Spleen function was studied in a group of 20 Kuwaiti SS patients (aged 2-12 years), using 99mTc-labeled tin colloid scintigraphy. They were screened for the a-thalassemia determinants which are prevalent in the Arabian Peninsula [-a (3.7 kb) deletion, (~2-globin gene polyadenylation signal (AATAAA --f AATAAG) mutation, and 5' IVS-I splice junction pentanucleotide (GAGGTGAGG -+ GAGG) deletion] with a combination of polymerase chain reaction and allele-specific oligonucleotide (ASO) hybridization techniques. The patients were divided into three groups depending on the result of their colloid uptake. Group I consisted of 7 patients (35.0%) with normally visualized spleens, Group II consisted of 5 (25.0%) with partial visualization, and in Group 111 there were 8 (40.0%) in whom the spleen was not visualized at all. The significant distinguishing features among those in Groups I and 111 were mean corpuscular volumes (MCVs) of 74.1 2 5.1 and 90.1 & 6.6 f l (P < 0.0001) and mean corpuscular hemoglobins (MCHs) of 22.4 2 2.7 and 27.5 2 4.0 pg (P < 0.05), respectively. The overall frequency of a-thalassemia determinants in the study was 35.0%; however, the frequencies in Groups I, II, and 111 were 57.1, 30.0, and l8.8%, respectively. mu-Thalassemia trait, therefore, appears to be associated with normal splenic function in these patients. o 19% Wiley-Liss, Inc.

Spleen function was studied in a group of 20 Kuwaiti SS patients (aged 2-12 years), using 99mTc-labeled tin colloid scintigraphy. They were screened for the alpha-thalassemia determinants which are prevalent in the Arabian Peninsula [-alpha (3.7 kb) deletion, alpha2-globin gene polyadenylation signal (AATAAA => AATAAG) mutation, and 5' IVS-I splice junction pentanucleotide (GAGGTGAGG => GAGG) deletion] with a combination of polymerase chain reaction and allele-specific oligonucleotide (ASO) hybridization techniques. The patients were divided into three groups depending on the result of their colloid uptake. Group I consisted of 7 patients (35.0%) with normally visualized spleens, Group II consisted of 5 (25.0%) with partial visualization, and in Group III there were 8 (40.0%) in whom the spleen was not visualized at all. The significant distinguishing features among those in Groups I and III were mean corpuscular volumes (MCVs) of 74.1 +/- 5.1 and 90.1 +/- 6.6 fl (P<0.0001) and mean corpuscular hemoglobins (MCHs) of 22.4 +/- 2.7 and 27.5 +/- 4.0 pg (P<0.05), respectively. The overall frequency of alpha-thalassemia determinants in the study was 35.0%; however, the frequencies in Groups I, II, and III were 57.1, 30.0, and 18.8%, respectively. alpha-Thalassemia trait, therefore, appears to be associated with normal splenic function in these patients.

ObjectiveThis study aimed to measure bone mineral density (BMD) of the lumber spine and femur regions in healthy adult Kuwaiti women, and to evaluate the influence of body size on BMD results.MethodsBone mass measurement was performed by dual-energy X-ray absorptiometry at the lumbar spine (L2-L4) and femur (four regions) of healthy ambulatory Kuwaiti females aged between 20 and 80 years. Body size measurements and a detailed questionnaire on past medical and gynecologic history, as well as lifestyle factors, were administered to all subjects. After excluding those with apparent or suggested abnormalities affecting bone mass, only 805 women from 4,887 were included and served as the target subjects.ResultsThe spine BMD values for Kuwaiti women up to 69 years old were lower than their US counterparts; the peak value was established in the fifth decade. Femur neck BMD was relatively stable up to menopause. Spine osteoporosis was prevalent among 10% of the subjects, compared with 4% in the femoral neck. Regression analysis revealed that each kilogram of body weight has a change of 0.3% in premenopausal women and 0.5–0.6% in the older group. In multiple regressions that included the three factors (height, weight and age), the effect of age superseded that of weight in the older group of women, where there was a detrimental annual effect on spine and femur (neck and total hip) BMD by 0.9%, while each kilogram of body weight had a constructive effect by 0.4 and 0.3%. In the premenopausal women, the positive effect of 1 kg of body weight was equivalent to about 1 year of aging in the trochanter region and half a year in the total hip region.ConclusionThe BMD value of healthy Kuwaiti women tend to be lower than the US reference at the lumber spine in all age groups but showed higher values for femur neck only in the age group of late 30s through 60 years. Weight has a stronger effect on BMD than does the height. The prevalence of osteoporosis in the lumber spine was more than double that in the femur neck in postmenopausal women.