|
 Previous Article | Table of Contents | Next Article 
Blood, 1 May 2002, Vol. 99, No. 9, pp. 3144-3150
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Moyamoya syndrome in childhood sickle cell disease: a predictive
factor for recurrent cerebrovascular events
Scott R. Dobson,
Kenton R. Holden,
Paul J. Nietert,
Joel K. Cure,
Joseph H. Laver,
Deborah Disco, and
Miguel R. Abboud
From the Departments of Neurology, Pediatrics, and
Radiology and the Center for Health Care Research, Medical University
of South Carolina, Charleston, SC; and the Department of Pediatrics,
Medical College of Virginia, Richmond, VA.
We conducted a retrospective study to determine whether the
presence of moyamoya collaterals influenced the risk of recurrence of
cerebrovascular events (CVEs: stroke or transient ischemic attack) in
patients with sickle cell disease placed on chronic transfusions after
a stroke. Forty-three patients with homozygous sickle cell anemia
(HbSS) and 1 with HbSOArab (16 females, 28 males) who had
suffered strokes while under the age of 18 were studied. All patients
had been on transfusions aimed at maintaining the sickle hemoglobin
(HbS) level below 30%. They were followed for a mean of 6.6 years (2.2 to 20.4 years). The presence of collaterals was diagnosed based on
either magnetic resonance angiography or conventional angiography.
Eighteen (41%) of the 44 patients suffered recurrent CVEs. Nineteen
(43%) (6 females, 13 males) patients had moyamoya collaterals. Eleven
(58%) of these 19 experienced 21 total recurrent CVEs, including 4 strokes in 4 patients (21%). In comparison, 7 (28%) of 25 patients
without moyamoya collaterals experienced 9 recurrent CVEs
(P < .05) with only 1 recurrent stroke (4%). Moyamoya
patients were also more likely to have 2 recurrent CVEs (42% vs 8%,
P < .05) as well as poorer neuropsychological testing
results. A proportional hazards regression analysis indicated that
patients with moyamoya were more than twice as likely to incur a
subsequent CVE (hazard ratio, 2.40; 95% confidence interval, 0.85, 6.75). We conclude that up to 41% of patients with sickle cell disease
experience recurrent CVEs after an initial stroke despite chronic
transfusions and that the risk of recurrence is significantly higher
for those who have moyamoya collaterals.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
A. Skardoutsou, K. A. Voudris, S. Mastroyianni, E. Vagiakou, G. Magoufis, and P. Koukoutsakis
Moya Moya Syndrome in a Child With Pyruvate Kinase Deficiency and Combined Prothrombotic Factors
J Child Neurol,
April 1, 2007;
22(4):
474 - 478.
[Abstract]
[PDF]
|
|
|
|
|
|
|
V. Ganesan, M. Prengler, A. Wade, and F. J. Kirkham
Clinical and Radiological Recurrence After Childhood Arterial Ischemic Stroke
Circulation,
November 14, 2006;
114(20):
2170 - 2177.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. J. Strouse, M. L. Hulbert, M. R. DeBaun, L. C. Jordan, and J. F. Casella
Primary Hemorrhagic Stroke in Children With Sickle Cell Disease Is Associated With Recent Transfusion and Use of Corticosteroids
Pediatrics,
November 1, 2006;
118(5):
1916 - 1924.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. T. Lee, S. Piomelli, S. Granger, S. T. Miller, S. Harkness, D. J. Brambilla, R. J. Adams, and for the STOP Study Investigators
Stroke Prevention Trial in Sickle Cell Anemia (STOP): extended follow-up and final results
Blood,
August 1, 2006;
108(3):
847 - 852.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
O. S. Platt
Prevention and Management of Stroke in Sickle Cell Anemia
Hematology,
January 1, 2006;
2006(1):
54 - 57.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
L. I. Kornblihtt, S. Cocorullo, C. Miranda, P. Lylyk, P. G. Heller, and F. C. Molinas
Moyamoya Syndrome in an Adolescent With Essential Thrombocythemia: Successful Intracranial Carotid Stent Placement
Stroke,
August 1, 2005;
36(8):
e71 - e73.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. Jabbour, A. Taher, A. Shamseddine, and S. F. Atweh
Moyamoya Syndrome With Intraventricular Hemorrhage in an Adult With Factor V Leiden Mutation
Arch Neurol,
July 1, 2005;
62(7):
1144 - 1146.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. Lottenberg and K. L. Hassell
An Evidence-Based Approach to the Treatment of Adults with Sickle Cell Disease
Hematology,
January 1, 2005;
2005(1):
58 - 65.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. C. Driscoll, A. Hurlet, L. Styles, V. McKie, B. Files, N. Olivieri, C. Pegelow, B. Berman, R. Drachtman, K. Patel, et al.
Stroke risk in siblings with sickle cell anemia
Blood,
March 15, 2003;
101(6):
2401 - 2404.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
