Moyamoya disease associated with hemoglobin Fairfax and beta-thalassemia

Pediatric Neurology
Franklin A MardenJay Greenberg

Abstract

Moyamoya disease is a rare cerebrovascular condition characterized by steno-occlusive disease of the major intracranial arteries at the base of the brain, and the appearance of innumerable, tiny, arterial collaterals that resemble a "puff of smoke." Although it has been associated with hematologic diseases such as sickle-cell disease, the association with other hemoglobinopathies is less frequently observed. We describe the association of a unique hemoglobinopathy (hemoglobin Fairfax) with beta-thalassemia and moyamoya disease in a 9-year-old girl with a history of stroke. To our knowledge, this is the first report of this unstable hemoglobin with moyamoya disease, and it emphasizes the potential for cerebral infarction due to the severe anemia of hemolytic disease.

References

Oct 26, 1972·The New England Journal of Medicine·J A StockmanF A Oski
Jul 31, 1982·Lancet·E D ThomasR Storb
May 1, 1995·Neurology·S G PavlakisA D Auerbach
Apr 16, 1998·Pediatric Radiology·A HolzE Lane
Jan 5, 2002·Blood·Amiram Eldor, Eliezer A Rachmilewitz
Aug 13, 2005·Journal of Pediatric Hematology/oncology·Knut BrockmannArnulf Pekrun
Mar 23, 2006·Journal of Child Neurology·Masafumi SanefujiToshiro Hara

❮ Previous
Next ❯

Citations

Jul 5, 2012·Cold Spring Harbor Perspectives in Medicine·Khaled M MusallamEliezer A Rachmilewitz
Jun 6, 2013·Blood Cells, Molecules & Diseases·Adlette InatiAhmad Beydoun
Aug 4, 2012·Thrombosis Research·Khaled M MusallamEliezer A Rachmilewitz
Apr 3, 2012·Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie·M NassafM Bouskraoui
Feb 2, 2013·International Journal of Laboratory Hematology·R AgrawalT I George
May 17, 2019·American Journal of Hematology·Alexander A BoucherCharles T Quinn
Feb 25, 2020·Journal of Child Neurology·Andrew R PinesRadhika Dhamija
Dec 24, 2019·NeuroRehabilitation·Khushnoo K IndorewallaIrene Piryatinsky
Aug 20, 2019·Journal of Neurology·Shambaditya DasMarkus Kraemer
Feb 17, 2021·Annals of Hematology·Linet NjueAlicia Rovó

❮ Previous
Next ❯

Related Concepts

Related Feeds

Anemia

Anemia develops when your blood lacks enough healthy red blood cells. Anemia of inflammation (AI, also called anemia of chronic disease) is a common, typically normocytic, normochromic anemia that is caused by an underlying inflammatory disease. Here is the latest research on anemia.

Blood And Marrow Transplantation

The use of hematopoietic stem cell transplantation or blood and marrow transplantation (bmt) is on the increase worldwide. BMT is used to replace damaged or destroyed bone marrow with healthy bone marrow stem cells. Here is the latest research on bone and marrow transplantation.

Brain Ischemia

Brain ischemia is a condition in which there is insufficient blood flow to the brain to meet metabolic demand. Discover the latest research on brain ischemia here.

Related Papers

Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia
Basak Karakurum GökselSuheyl Asma
Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
H OillicP Vic
Pediatric Blood & Cancer
Torrey M ParkerRobert J Klaassen
© 2021 Meta ULC. All rights reserved