Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The cause of Moyamoya disease is still unknown. We made a research about the factors closely related to Moyamoya disease through a case-control study. The number of cases studied was 66. Controls were selected from among patients' friends, matched as to sex, age and residential area. Questionnaires were sent to the cases and the respective controls by mail. The questions were about past history, developmental history, school records, habitual factors, dietary habits and pets. The first symptoms and age at onset were also surveyed in the cases. The response rate was 84.8% (56 cases) of the cases and 76.5% (101 cases) out of the 132 controls. There was no significant difference in the prevalence of tonsillitis, conjunctivitis, otitis media, and bronchitis. Odds ratio of fever of unknown origin is 2.793 and X2 is 7.213. Diseases whose odds ratio was over 1 were herpes, appendicitis, bronchitis, asthma, anemia, dental caries, head injury and drug allergy. But all of them were not significantly prevalent. Odds ratios of school records were 4-9 and X2 were 4-17 from elementary to junior high school. Odds ratio of western dishes was 2.709 and X2 was 5.189. There was no significant difference as to pets kept. We could not find overt relationships between Moyamoya disease and diseases of head and neck like tonsillitis.
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PMID:[Case-control study of moyamoya disease]. 280 21

Sickle cell (drepanocytic) anemia is a hereditary blood disease occurring very rarely in Mexico. A 13-year-old Mexican boy with sickle cell anemia eventually died of a cerebrovascular accident of the brain stem, as shown by computerized tomography (CT). A characteristic moyamoya-like angiographic pattern was demonstrated on bilateral carotid and left vertebral arteriograms. Moyamoya disease has no known etiology, but the characteristic angiographic features of moyamoya have been observed in conjunction with some other disease of known origin (including sickle cell anemia). It is therefore my belief that these latter cases should be referred to as pseudomoyamoya and not true moyamoya.
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PMID:Pseudomoyamoya in sickle cell anemia. 716 62

We report two patients with Fanconi anemia (FA) and moyamoya disease taken from a clinical database composed of 434 FA patients. Both are compound heterozygotes for the 322delG and R185X mutations in the FA complementation group C (FACC) gene. This combination of mutations is not found in any other of the 174 FA families screened. Either the 322delG or R185X mutation alone or in combination may predispose to primary, possibly congenital, vascular anomalies.
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PMID:Fanconi anemia and moyamoya: evidence for an association. 774 24

In purpose of clarifying the risk factors of perioperative ischemic complications in cases with moyamoya disease, a retrospective study was conducted on the perioperative care of 36 patients who underwent vascular reconstruction for moyamoya disease. Following surgery, 8 patients developed ischemic complications (5 with minor completed strokes and 3 with transient ischemic attacks). Three factors including the presence of a preoperative low density area (LDA), intra-operative urinary output, and a reduction in the hematocrit significantly differed with complications and those without. In an evaluation of individual cases, intra-operative hypercapnia, hypotension, and a reduction in the circulating blood volume also were identified as potential factors associated with complications. On the basis of these findings, it was concluded that it is necessary to infuse an adequate amount of fluids, maintain normocapnic and normothermic states, and actively correct anemia in patients undergoing vascular reconstruction for moyamoya disease. It is also necessary to pay particular attention to perioperative care in patients in whom a LDA is detected by preoperative CT.
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PMID:Perioperative factors related to the development of ischemic complications in patients with moyamoya disease. 910 39

Cerebral infarction is a frequent, severe complication of sickle cell anaemia. During childhood, most strokes are due to infarction with the majority resulting from occlusion of the large cerebral arteries. Risk factors include transient ischaemic attacks, acute chest syndrome, severe anaemia and elevated blood pressure. Less certain is the association with leucocytosis, or protection provided by alpha-thalassaemia or fetal haemoglobin. Children who have one stroke are at significant risk for having subsequent events that can be substantially reduced by maintaining haemoglobin S below 30%. It has not yet been possible to identify individuals for whom transfusion can be safely stopped. Haemosiderosis is a consequence of intensive and long term transfusion therapy, which requires chelation with deferoxamine. Iron accumulation can be minimised using erythrocytapheresis but this is technically difficult in children, expensive and results in increased donor exposure. In addition to lesions associated with strokes, an additional 17% of patients can be shown to have clinically silent cerebral infarcts. Although these are termed 'silent', those affected have mild neuropsychological deficits. Their relationship to stroke or risk for recurrence is unknown. Transfusion therapy has been shown to provide primary stroke prevention for children who have elevated cerebral artery velocity. Finally, intracranial haemorrhages, more commonly found in adults, also affect children. Subarachnoid haemorrhage is frequently found to result from cerebral artery aneurysms. A condition that mimics the moyamoya syndrome radiographically, as well as for its risk of haemorrhage, can be found in children with partly occluded cerebral arteries either as a result of stroke or silent infarct.
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PMID:Stroke in children with sickle cell anaemia: aetiology and treatment. 1143 87

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.
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PMID:Moyamoya disease associated with hemoglobin Fairfax and beta-thalassemia. 1820 95

This retrospective study assessed the long-term effect of transfusional exchange therapy on MRA/MRI abnormalities in 24 homozygous sickle-cell anemia (HbSS) children presenting with abnormal brain MRA. The median time elapsed from baseline to last available MRA was 29 months. Follow-up MRAs showed improvement, stabilization or worsening of cerebrovascular lesions in 11, 6 and 7 patients respectively. Complete normalization of MRA was observed in 6 patients within a mean time of 1.4 years, but stenosis recurred at the same location in the 4 patients in whom transfusion therapy was discontinued. Baseline severe stenosis/occlusion of large cerebral arteries and occurrence of moyamoya syndrome were significantly associated with an absence of improvement of the cerebral vasculopathy. These data emphasize the heterogeneity of the course of cerebrovasculopathy in SS children receiving chronic transfusion. Further studies are needed to determine whether different therapeutic approaches have to be considered according to these different evolutive patterns in SS children.
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PMID:Effect of transfusion therapy on cerebral vasculopathy in children with sickle-cell anemia. 1905 38

Moyamoya disease is a rare chronic cerebrovascular occlusive disease diagnosed by angiogram with primary features consisting of bilateral occlusion of the internal carotid arteries and of the vessels forming the circle of Willis, and subsequent development of small collateral vessels. These collateral vessels may rupture, leading to the clinical presentation of transient ischemic attacks and intracerebral hemorrhage. We describe a young Korean woman with known moyamoya disease presenting with her second major vascular event who was also found to have active, undiagnosed systemic lupus erythematosus (SLE). SLE, with or without anti-cardioli-pin, or anti-cardiolipin antibody syndrome, can be associated with "pseudo-moyamoya" (unilateral central nervous system disease). SLE, anti-cardiolipin antibody syndrome, pseudo-moyamoya, or moyamoya disease can all present in young women with intracerebral events, making diagnosis difficult.Our patient presented with fatigue, anemia, leukopenia, and a positive anti-nuclear antibody. Her physicians initially entertained a diagnosis of SLE, but when she developed a thalamic infarct, this diagnosis was abandoned, because a cerebral angiogram revealed a bilateral "cigarette smoke" appearance of blood vessels characteristic of moyamoya disease rather than the similar unilateral findings more typical of pseudo-moyamoya. Subsequently, it became clear that our patient had full-blown SLE and moyamoya disease. The clinician should be aware of the diagnosis of moyamoya because patients with this disease may present with intracerebral events associated with features of autoimmune disease, even in the absence of SLE. The concurrent development of SLE may confound the diagnosis even further.
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PMID:Systemic lupus erythematosus associated with moyamoya disease case report and review of the literature. 1907 32

Moyamoya syndrome (MMS) is a progressive disorder. We report a 19-year-old boy with beta-thalassemia who presented with a left hemiparesis. Brain MRI showed old middle cerebral artery and left frontal subcortical white matter infarcts. Brain magnetic resonance angiography and digital subtraction angiography revealed occlusion of the bilateral internal carotid arteries with a rich network of basal collateral vessels. To our knowledge this is the third report of beta-thalassemia intermedia and MMS, and the first report of a patient in Turkey. It emphasizes the potential for cerebral infarct due to anemia, protein S and thrombocytosis.
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PMID:Beta-thalassemia intermedia associated with moyamoya syndrome. 2039 63

A 3-year-old girl who presented with anemia, thrombocytopenia, and recurrent strokes is described. The cerebral angiography revealed moyamoya vasculopathy. Her younger brother also had anemia and thrombocytopenia but no neurologic abnormalities. Both children had severe deficiency of ADAMTS13 (A Disintegrin And Metalloprotease with Thrombospondin like domain activity) confirming the diagnosis of congenital thrombotic thrombocytopenic purpura. The children responded well to regular fresh-frozen plasma infusions. This report expands the spectrum of hematologic diseases associated with moyamoya syndrome. Unexplained thrombocytopenia, especially in the presence of neurologic symptoms, should prompt an evaluation for ADAMTS13deficiency. The diagnosis has significant implications not only for therapy but also for genetic counseling.
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PMID:Congenital thrombotic thrombocytopenic purpura associated with moyamoya syndrome in a 3-year-old girl: a case report. 2237 76


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