ADAMTS-13 antigen and activity levels in thrombocytopenic disorders including thrombotic thrombocytopenic purpura in Kuwait.

BACKGROUND/AIMS: While deficiency of a disintegrin and metalloprotease with thrombospondin-1-like domains (ADAMTS-13) was reported as the basis for the pathogenesis of thrombotic thrombocytopenic purpura (TTP), low levels have also been found in other thrombocytopenic disorders. This study was conducted to characterize the activity and antigen levels of ADAMTS-13 and von Willebrand factor (vWF) in patients with different thrombocytopenic disorders in Kuwait. METHODS: Forty healthy subjects and 41 patients with different thrombocytopenic disorders were recruited for this study. ELISA tests were used to measure ADAMTS-13 and vWF activity and antigen levels in patients and controls. RESULTS: All TTP patients had severely deficient ADAMTS-13 activity (<5%), which was significantly lower than that of controls (p < 0.001). Severe deficiency of ADAMTS-13 was also found in some, but not all, patients with idiopathic thrombocytopenic purpura, acute leukemia and sepsis. CONCLUSIONS: This study is the first to report ADAMTS-13 levels in this part of the world. ADAMTS-13 was found to be severely deficient in TTP patients. We present evidence that significantly lower levels of ADAMTS-13 were not specific for TTP and can be found in other thrombocytopenic disorders. We also hypothesize that clinical manifestation of TTP may not be solely due to ADAMTS-13 deficiency, and there might be other contributing factors, since the deficiency was also found in some healthy controls.

Comparison of renal function markers in Kuwaiti patients with sickle cell disease.

BACKGROUND: Proteinuria is a common manifestation of renal disease which is a significant cause of morbidity in patients with sickle cell disease (SCD). OBJECTIVE: To evaluate and compare cystatin C, beta(2)-microglobulin, and creatinine as markers of renal disease in relation to the degree of proteinuria and other complications of SCD. METHODS: 24 h urine collections were used for estimation of urine protein and creatinine clearance in 59 patients with SCD. Results were correlated with plasma cystatin C, beta(2)-microglobulin, creatinine, glomerular filtration rate (GFR; derived from plasma creatinine by Cockcroft-Gault, MDRD formulae, and calculated cystatin C clearance), and clinical and haematological variables. RESULTS: Comparing the different methods of GFR, the proportion of patients with hyperfiltration (GFR >140 ml/min) were 30.5% (MDRD), 44.1% (Cockcroft-Gault), and 10.2 % (calculated cystatin C clearance). Cystatin C was the most consistent marker of hyperfiltration. The endogenous markers of GFR showed an increasing trend with increasing proteinuria, but haematological variables were not correlated with cystatin C, beta(2)-microglobulin, or plasma creatinine. Urine protein excretion was correlated with age (r = 0.33) and significant proteinuria was present in 13.6% of patients. Patients with proteinuria had lower haemoglobin concentration (p = 0.027) than those without proteinuria but HbF was not related to the degree of proteinuria or to markers of GFR. CONCLUSIONS: Markers of GFR show variable ability to identify hyperfiltration in patients with SCD, but cystatin C is the best endogenous marker. Proteinuria is associated with age, haemoglobin, and abnormalities of GFR. Routine screening is recommended to allow for early detection and intervention.

Avascular necrosis of the femoral head in adult Kuwaiti sickle cell disease patients.

While sickle cell disease (SCD) is generally mild in most Kuwaitis, because of their elevated fetal Hb levels, avascular necrosis of the femoral head (AVNFH) appears to be a common complication. It was recently documented in 26.7% of Kuwaiti children with SCD. There have, however, been no previous studies of adult patients. This is a 1-year study of consecutive, steady-state SCD patients seen in the hematology clinic of Mubarak Al-Kabeer Hospital. The patients' charts were reviewed for frequency of hospitalizations, any documented complications and steady-state complete blood count (CBC). MRI was performed using T1- and T2-weighted FATSAT sequences in coronal and axial planes with 4-mm-thick slices on a 1.5-tesla GE super-conducting magnet. Thirty-five patients were studied, consisting of 25 SS and 10 Sbeta(0)Thal patients aged between 17 and 44, with a mean age of 26.7 +/- 9.3 years. Seventeen (48.6%) had varying degrees of AVNFH; among the 70 hips examined, 29 (41.1%) were affected. Of the 17 patients affected, 11 (64.7%) were SS, while 6 (35.3%) were Sbeta(0)Thal. There were 14 (82.4%) males and 3 (17.6%) females (chi(2) = 8.6, p < 0.01). The mean age of those affected, 27.5 +/- 10.7 years, was not significantly higher than that of the unaffected (26.3 +/- 8.0 years). Eleven (64.7%) of those affected had a history of frequent vaso-occlusive crisis. No significant differences could be demonstrated in the mean CBC and Hb F values of the two groups; coexistent alpha-thal trait was not a factor in the SS group. Male gender was the only significant predisposing factor identified. While more patients with frequent vaso-occlusive crises were affected, the difference was not significant. AVNFH is, indeed, quite common among Kuwaiti SCD patients and there is a need for early institution of preventive and therapeutic protocols.

Ethnic differences in contraceptive use in Kuwait: a clinic-based study.

The Arab, Muslim, oil-rich nation of Kuwait has achieved unusually high levels of knowledge and use of contraception for a developing, high fertility country. Almost all women know of contraceptive pills without prompting, and 57-86% report having ever used a method (usually the oral pill or IUD) in recent studies. Based on a survey of six randomly selected clinics the present study compares the knowledge and use levels of two major ethnic groups--the Beduins and non-Beduins. It also analyses preference for various contraceptive methods and probable reasons for this. While Census or Survey data do not provide information about the size of ethnic groups analyzed in this paper, it is estimated that at least one-third of the population of Kuwait is Beduin. There is a significant difference between the levels of knowledge and use of contraception between the Beduin and non-Beduin women; current use being 42% and 65%, respectively. The differentials between the two groups are particularly marked among women of lower socioeconomic status, and tend to reduce notably once variables such as education and income are controlled. Within the subgroup of non-Beduins, socioeconomic differences in contraceptive use have virtually disappeared; the illiterate and relatively less affluent women are as likely to use a contraceptive method as the university educated, and richer women. However, among the Beduins, the usually expected differences by socioeconomic characteristics still persist. The oral pill is the best known and most commonly used method. Male sterilization is the least known and not practiced at all.(ABSTRACT TRUNCATED AT 250 WORDS)