Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A multitude of general disorders of the vascular system may also affect the blood circulation of the cochlea and cause symptoms such as fluctuating or permanent hearing loss. Such is the case for arteriosclerosis combined with hypertension or hypotension, collagenosis, and diabetes. Blood disorders, like leukemia, sickle cell anemia, and polycythemia, and infectious diseases involving the blood vessels, such as lues, may also present their primary symptoms in the ear. The otorhinolaryngologist must be able to establish the correct diagnosis and refer patients requiring more general treatment to other specialists. The use of specific vasoactive treatment should be continued to those patients with symptoms of acute or fluctuating hearing loss, vertigo, or tinnitus who exhibit no other signs. Modern techniques for cochlear blood flow measurements have verified that several of the treatment protocols in use, which have a sound theoretical background, do indeed increase cochlear blood flow.
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PMID:Clinical treatment of vascular inner ear diseases. 306 94

Sickle cell anemia is frequently associated with auditory dysfunction. However peripheral vestibulopathy is uncommon. The purpose of this report is to describe a patient who presented repeated episodes of severe vertigo attributable to sickle cell disease. Discussion of this case includes a review of the literature.
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PMID:[Sickle cell disease and vertigo: a possible diagnosis in metropolitan France. Review of the literature and case report]. 1261 47

Piracetam, a derivative of the neurotransmitter gamma-aminobutyric acid (GABA), has a variety of physiological effects that may result, at least in part, from the restoration of cell membrane fluidity. At a neuronal level, piracetam modulates neurotransmission in a range of transmitter systems (including cholinergic and glutamatergic), has neuroprotective and anticonvulsant properties, and improves neuroplasticity. At a vascular level, it appears to reduce erythrocyte adhesion to vascular endothelium, hinder vasospasm, and facilitate microcirculation. This diverse range of physiological effects is consistent with its use in a range of clinical indications. Its efficacy is documented in cognitive disorders and dementia, vertigo, cortical myoclonus, dyslexia, and sickle cell anemia. While high doses are sometimes necessary, piracetam is well tolerated.
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PMID:Piracetam: a review of pharmacological properties and clinical uses. 1600 38

Patients with superior dehiscence (SCD) syndrome present with vertigo and oscillopsia evoked by loud sounds and changes in middle ear or intracranial pressure. The first objective of this retrospective cohort study is to demonstrate that thin-section computed tomography (CT) scans reformatted in the plane of the superior semicircular canal (SSC) overestimate this anomaly compared to pathologic studies. The second objective of this study is to re-evaluate the positive predictive value of temporal bone scanning. All temporal bone CT scans with 0.55-mm collimation and reconstruction in the SSC plane performed over a 1-year period were analysed at a tertiary referral centre. CT-positive cases had their clinical data reviewed and patients were re-examined, if available. A total of 581 temporal bone CT-scans were analysed. A dehiscent-appearing superior canal was seen in 4.0% of studies while published pathologic studies report that only 0.5% of temporal bones SSCs have a dehiscence (P < 0.001). Of the 21 patients with positive temporal bone CTs, only 1 presented with sufficient clinical dues to identify the syndrome. Three additional patients did not have symptoms consistent with the diagnosis, but had surgery for a dehiscence of the tegmen mastoideum. When our findings are added to published data, the positive predictive value of temporal bone CT-scanning drops from 93 to 57%. The prevalence of dehiscent-appearing superior canal on thin-section temporal bone scanning with reformation in the SSC plane is much higher than anticipated by pathologic studies. Even with 0.55 mm-collimated helical CT and reformation in the SSC plane, the risk of overdiagnosis is present.
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PMID:Superior semicircular canal dehiscence: positive predictive value of high-resolution CT scanning. 1841 14

Vestibular syndrome is not frequently described in patients with sickle cell disease. We report the case of a teenager with sickle cell disease who had a vestibular syndrome with vertigo that successfully responded to exchange transfusion. We discuss guidelines and review the literature in view of this case report. Sensorineural disorders should be considered as stroke syndromes. They require urgent treatment consisting of exchange transfusion or maintaining optimal hydration associated with blood withdrawal. Treatment of vestibular syndrome in sickle cell disease is urgent.
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PMID:[Vestibular neuronitis in a teenager with sickle cell disease. Treatment is urgent]. 1867 41

The rise in non-communicable diseases in Africa presents challenges for health systems that are burdened by infectious diseases. Gout is one of those diseases that has seen an increase in numbers worldwide, including Africa. Gout is commonly associated with comorbidities and mortality. It directly impacts the quality of life, increases health costs, decreases physical function, and significantly increases the time from work, much of which is potentially avoided if treatment is instituted early. Despite advances in understanding the pathophysiology and outcomes of gout, the quality of care delivered to patients in Africa is still suboptimal. Existing data on gout in Africa reveals a general low index of suspicion due to limited knowledge of the disease by healthcare workers resulting in late diagnosis, with severe polyarticular tophaceous gout being a common presenting feature. These late presentations are associated with avoidable disability and increase the direct and indirect costs of managing gout. The challenges are related to lack of government budgetary support for staff training, infrastructure for diagnosis, and availing medicines. The picture of gout in Africa largely mirrors the west concerning risk factors, comorbidities, and burden of disease, but with some unique presentations seen in HIV, sickle cell disease, and vertigo. We discuss the challenges of gout diagnosis and management in Africa and propose a roadmap to improve gout outcomes across Africa.
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PMID:The management of gout in Africa: challenges and opportunities. 3294 84