Abstract:
:Avascular necrosis (AVN) of the hip is a common cause of morbidity in sickle cell disease (SCD). Its prevalence increases with age and predisposing factors include coexistent alpha-thalassemia trait, frequent vaso-occlusive crisis and a high hematocrit (Hct). SCD is relatively mild among Kuwaiti patients because of their elevated Hb F levels, but a subset exists with severe recurrent vaso-occlusive crises. We carried out a prospective magnetic resonance imaging (MRI) study of the hip in a group of patients being followed in the Pediatric Hematology clinics of Al-Mubarak and Al-Amiri Hospitals. The association of AVN with age, frequency of hospitalization, alpha-thal trait, steady-state Hb, Hct, Hb F, WBC and platelet counts was investigated. MRI was carried out with a 1.5-tesla GE unit with a super-conducting magnet. Thirty patients (19 males, 11 females) (23 SS and 7 SbetaThal) were studied. Their ages ranged from 6 to 17 years, with a mean of 9.8 +/- 3.5 years, and Hb F from 11 to 35% with a mean of 22.8 +/- 5.7%. Among the SS patients, 11 (47.8%) had coexistent alpha-thal trait (-3.7-kb deletion). A total of 8 (26.7%) patients (6 SS and 2 SbetaThal) had varying degrees of osteonecrosis of the hip. Four (36.4%) of the 11 SS patients with alpha-thal trait and 2 (16.7%) of those without alpha-thal trait had osteonecrosis. This difference is, however, not statistically significant (chi(2) = 0.3, p = 0.5). While there was also no significant difference in the mean age and hematological parameters (Hb, Hct, Hb F, WBC, platelets), the SS patients with osteonecrosis had a significantly higher number of hospitalizations for vaso-occlusive crisis in the preceding 3 years than those without osteonecrosis.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Adekile AD,Gupta R,Yacoub F,Sinan T,Al-Bloushi M,Haider MZdoi
10.1159/000046529subject
Has Abstractpub_date
2001-01-01 00:00:00pages
27-31issue
1eissn
0001-5792issn
1421-9662pii
46529journal_volume
105pub_type
杂志文章abstract::Factor VII deficiency is the least rare among uncommon congenital coagulation disorders. The majority of cases are isolated deficiencies. In some cases, FVII deficiency has been found to be associated with the deficiency in another coagulation factor or with non-coagulation-related abnormalities or defects. The evalua...
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abstract:BACKGROUND:In 2003, oral fludarabine was introduced in the USA for the treatment of patients with hematologic malignancies as an alternative to its intravenous (i.v.) formulation; in 2008, it was introduced in México while the i.v. formulation was withdrawn. Accordingly, i.v. fludarabine had to be replaced by oral flud...
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pub_type: 临床试验,杂志文章
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更新日期:1991-01-01 00:00:00
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更新日期:2009-01-01 00:00:00
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pub_type: 杂志文章
doi:10.1159/000041005
更新日期:2000-01-01 00:00:00
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journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207923
更新日期:1976-01-01 00:00:00
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pub_type: 杂志文章
doi:10.1159/000204894
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journal_title:Acta haematologica
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更新日期:1989-01-01 00:00:00
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pub_type: 杂志文章,评审
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更新日期:1979-01-01 00:00:00
abstract::Recently, a factor was discovered in the serum of hepatectomized animals which was capable of augmenting the hepatic erythropoietin response to hypoxia when injected into normal rats. This substance was localized in the liver via an in situ perfusion technique and was termed the hepatic erythropoietic factor (HEF). Pa...
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更新日期:1983-01-01 00:00:00
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更新日期:2008-01-01 00:00:00
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更新日期:1993-01-01 00:00:00
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更新日期:2020-08-19 00:00:00