title: Avascular necrosis of the femoral head in adult Kuwaiti sickle cell disease patients.
abstract: 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.
authors.affiliation: Department of Pathology, Faculty of Medicine, Kuwait University, Safat, Kuwait.
authors.forename: R
authors.initials: R
authors.lastname: Marouf
authors.forename: R
authors.initials: R
authors.lastname: Gupta
authors.forename: M Z
authors.initials: MZ
authors.lastname: Haider
authors.forename: H
authors.initials: H
authors.lastname: Al-Wazzan
authors.forename: A D
authors.initials: AD
authors.lastname: Adekile
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000755:Anemia, Sickle Cell / Q000453:epidemiology*; D005260:Female; D005271:Femur Head Necrosis / Q000453:epidemiology* / Q000473:pathology*; D006801:Humans; D007730:Kuwait; D008279:Magnetic Resonance Imaging; D008297:Male; D015995:Prevalence