We have investigated the levels of Th1 (IL-2 and IFN-γ) and Th2 (IL-4) cytokines in the plasma and supernatants following peripheral blood mononuclear cell culture and mitogen stimulation in a group of 39 patients with sickle cell disease (SCD) made up of 29 SS, 8 Sβ-thal and 2 Hb SD in steady state. Five SS patients were studied during 7 episodes of vaso-occlusive crisis. Twenty-four control (3 Hb AS and 21 Hb AA) were also studied; 10 were acutely ill while 14 were healthy at the time of the study. The plasma levels of IL-2 and IFN-γ were similar in the patients and the controls. However, plasma IL-4 was significantly higher among the steady-state SS patients than in the controls. While there was no significant difference in cytokine levels following mitogen stimulation in the different groups, plasma IL-2 to IL-4 and IFN-γ to IL-4 ratios were significantly lower among the steady-state SS patients, indicating a possible Th2 bias in our sickle cell patients and suggesting a possible mechanism to explain the predisposition of SCD patients to bacterial infections. However, SS patients with good splenic function showed a relative Th1 bias, which may be an additional explanation for the protection against bacterial infections in such patients.

Objectives: To analyze the results of hemoglobin electrophoresis (HE) in the routine laboratory of a tertiary hospital in Kuwait and to review the common types of hemoglobinopathies prevalent in the country. Methods: This was a prospective study of HE performed on 2,386 samples in Mubarak Al-Kabeer Hospital, which serves more than 30% of the population of Kuwait, from June 1997 to May 1998. Results: Of the 2,386 HE tests, only 561 (23.5%) had abnormal hemoglobin genotypes. The most commonly identified hemoglobinopathies were β-thalassemia minor (14%), sickle cell trait (6%), sickle cell anemia (0.9%), Sβ⁰thal (0.8%) and Sβ⁺thal (0.8%). Two rare hemoglobin variants, Hb D_Punjab and Hb E, were encountered. Conclusion: HE yielded only 23.5% abnormal results, thus indicating the need to streamline requests for the test. The test should be limited to patients with hematological and clinical features suggestive of hemoglobinopathies or to individuals with a positive family history.

Steady-state sickle cell disease (SCD) patients may have increased plasma levels of acute phase reactants and pro-inflammatory cytokines because of subclinical inflammation. We have estimated TNF-levels in the plasma and in supernatants following peripheral blood mononuclear cell (PBMC) activation with phytohemagglutinin (PHA) in a group of Kuwaiti SCD patients using ELISA. The group consisted of 28 SS, 8 S-thal, and 2 SD patients all in steady state; 5 SS patients were studied during 7 episodes of painful crisis. The subjects were aged 2 to 16 years, with a mean of 7.3 ± 3.5 years. The S-globin gene cluster haplotype,-tha1 status, and spleen function were determined in the SS group using standard techniques. Most (82%) were homozygous for the Saudi Arabia/ India haplotype and had elevated Hb F levels ranging from 15% to 35%. There were 24 controls (Hb AA or AS), of whom 14 were healthy and 10 were acutely ill at the time of the study. None of the children with SCD (either in steady state or crisis) had detectable plasma TNF-, but four controls (3 acutely ill and one healthy) had levels ranging from 61.7 to 249.8 pg/mL. Following PHA stimulation most subjects responded with high levels of TNF-, with the median level among the steady-state SS patients being significantly higher than that in the controls (both the acutely ill or healthy). It therefore appears that because of the mild disease among our Arab SS children, TNF-is not detectable in their plasma in steady state; these children, however, had a significantly higher response than controls following PBMC activation. Am. J. Hematol. 64:91-94, 2000.

α-Thalassemia is very common in the Kuwaiti population, but its influence on anemia of pregnancy has not been previously investigated. We have screened a group of 59 anemic (Hb <11 g/dl) pregnant women for the α-thal-2 (–α-3.7 kb) deletion which is the commonest α-thal allele in this community, using a polymerase chain reaction method. A control group of 35 nonanemic (Hb ≥11 g/dl) pregnant women was studied for comparison. All the women were in the second or third trimester of pregnancy. Among the 94 women in both groups, 69 (73.4%) had a normal complement of α-globin genes (αα/αα), 18 (19.1%) were heterozygotes (–α/αα) and 7 (7.4%) were homozygotes (–α/–α) giving an allele frequency of 17.0%. Among the anemic group, there were 44 (74.6%) individuals with a normal genotype, 9 (15.3%) heterozygotes and 6 (10.2%) homozygotes. In the nonanemic group, the corresponding prevalence figures were 25 (71.4%), 9 (25.7%) and 1 (2.9%), respectively. The difference between these distributions was statistically significant (χ² = 37.5, p < 0.0001). However, the mean Hb values were similar in heterozygotes, homozygotes and normal individuals. We, therefore, conclude that while the α-thal trait affects the prevalence of anemia among pregnant Kuwaiti women, it does not affect its severity.

Objective: To study the attitudes of different groups of physicians toward management of acute pyelonephritis in children and the impact of such attitudes on the long-term sequelae of this problem. Materials and Methods: A questionnaire, describing a case history of a child with acute pyelonephritis, was distributed to general practitioners working in 12 primary health care centers and pediatricians working in the pediatric departments of the 6 main hospitals in Kuwait. The responses of the two groups were analyzed regarding their views toward hospitalization of the child, investigations requested, the choice of antibiotics, its route of administration, duration of treatment and the need for long-term follow-up. Results: A total of 83 general practitioners (group A) and 65 hospital pediatricians (group B) responded to the questionnaire. Hospitalization of the child was thought to be essential by 19% of group A compared to 89% of group B (p < 0.0001). Only 25% of group A decided to fully investigate the child compared to 58% of group B (p < 0.0001). Treatment with a single antibiotic was preferred by 72% of group A compared to 80% of group B. Only 6% of group A, compared to 74% of group B favored parenteral antibiotics (p < 0.0001). A 7-day course of treatment was considered by 70% of group A and 52% of group B (p < 0.02). Long-term follow-up was thought to be necessary by 63% of group A and 75% of group B. Conclusion: A marked discrepancy existed in the management of acute pyelonephritis between the two groups, indicating the need of a unified policy. Children presenting with such a problem can be managed by general practitioners on an outpatient basis provided that proper antibiotics are used, compliance is ensured and appropriate investigations and follow-up are provided.