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Julian L. Allen - One of the best experts on this subject based on the ideXlab platform.

  • Pulmonary complications of sickle cell Disease in children.
    Current opinion in pediatrics, 2008
    Co-Authors: Jason B. Caboot, Julian L. Allen
    Abstract:

    PURPOSE OF REVIEW: Sickle cell Disease is one of the most prevalent genetic Diseases, affecting 1 in 600 African-Americans. The lung is one of the major organs affected in sickle cell Disease and the pulmonary complications of sickle cell Disease result in significant morbidity and mortality in children. RECENT FINDINGS: Recent literature has provided evidence supporting the strong association between asthma and airway hyperreactivity and sickle cell Disease, as well as a link with acute chest syndrome and vaso-occlusive crisis. Additionally, children with sickle cell Disease who have acute chest syndrome episodes have worse pulmonary function than those who have not experienced acute chest syndrome episodes. An increasing amount of literature highlights the link between pulmonary hypertension and sickle cell Disease, and multiple mechanisms have been invoked to explain why patients with sickle cell Disease are prone to the development of pulmonary hypertension. Previous and current studies have also highlighted the manifestations of sleep disordered breathing in children with sickle cell Disease. SUMMARY: The pulmonary complications of sickle cell Disease include airway hyperreactivity, acute chest syndrome, chronic sickle lung Disease, pulmonary hypertension, and sleep disordered breathing. Further understanding of the interrelationships between these disorders will lead to improved therapies.

Jason B. Caboot - One of the best experts on this subject based on the ideXlab platform.

  • Pulmonary complications of sickle cell Disease in children.
    Current opinion in pediatrics, 2008
    Co-Authors: Jason B. Caboot, Julian L. Allen
    Abstract:

    PURPOSE OF REVIEW: Sickle cell Disease is one of the most prevalent genetic Diseases, affecting 1 in 600 African-Americans. The lung is one of the major organs affected in sickle cell Disease and the pulmonary complications of sickle cell Disease result in significant morbidity and mortality in children. RECENT FINDINGS: Recent literature has provided evidence supporting the strong association between asthma and airway hyperreactivity and sickle cell Disease, as well as a link with acute chest syndrome and vaso-occlusive crisis. Additionally, children with sickle cell Disease who have acute chest syndrome episodes have worse pulmonary function than those who have not experienced acute chest syndrome episodes. An increasing amount of literature highlights the link between pulmonary hypertension and sickle cell Disease, and multiple mechanisms have been invoked to explain why patients with sickle cell Disease are prone to the development of pulmonary hypertension. Previous and current studies have also highlighted the manifestations of sleep disordered breathing in children with sickle cell Disease. SUMMARY: The pulmonary complications of sickle cell Disease include airway hyperreactivity, acute chest syndrome, chronic sickle lung Disease, pulmonary hypertension, and sleep disordered breathing. Further understanding of the interrelationships between these disorders will lead to improved therapies.

Jannet F. Lewis - One of the best experts on this subject based on the ideXlab platform.

  • Pulmonary hypertension in sickle cell Disease
    The American journal of cardiology, 1994
    Co-Authors: Laddeus L. Sutton, Oswaldo Castro, Diane J. Cross, Jane E. Spencer, Jannet F. Lewis
    Abstract:

    Abstract Pulmonary hypertension is not uncommon in the echocardiograms of patients with sickle cell Disease. Although a substantial number of these patients may not have symptoms of cardiac dysfunction, mortality is significantly increased compared with patients with sickle cell Disease hut without pulmonary hypertension.

Fernando D. Testai - One of the best experts on this subject based on the ideXlab platform.

  • Neurologic Complications of Sickle Cell Disease
    Current Neurology and Neuroscience Reports, 2019
    Co-Authors: Shama Farooq, Fernando D. Testai
    Abstract:

    Purpose of Review Sickle cell anemia is a multiorgan Disease with acute and chronic complications. Involvement of the central nervous system (CNS) is associated with increased mortality and morbidity. This review highlights the broad spectrum of neurological complications seen in patients with sickle cell Disease. Recent Findings Increasing recognition of neurological complications has led to improved diagnostic and treatment options throughout the years. Neurologic complications in sickle cell Disease include silent cerebral ischemia, ischemic/hemorrhagic stroke, moyamoya syndrome, posterior reversible encephalopathy syndrome, cerebral fat embolism, and cerebral venous sinus thrombosis. Treatment varies depending on the neurological complication. Summary Sickle cell Disease is the most common hereditary anemia with increasing global Disease burden. Early recognition and treatment is imperative.

Renee Gardner - One of the best experts on this subject based on the ideXlab platform.

  • Sickle Cell Disease: Advances in Treatment
    The Ochsner journal, 2018
    Co-Authors: Renee Gardner
    Abstract:

    Background Sickle cell Disease causes significant morbidity and mortality and affects the economic and healthcare status of many countries. Yet historically, the Disease has not had commensurate outlays of funds that have been aimed at research and development of drugs and treatment procedures for other Diseases. Methods This review examines several treatment modalities and new drugs developed since the late 1990s that have been used to improve outcomes for patients with sickle cell Disease. Results Targeted therapies based upon the pathophysiologic mechanisms of sickle cell Disease that result in organ dysfunction and painful episodes include hydroxyurea, L-glutamine, crizanlizumab, and other drugs that are currently on the market or are on the verge of becoming available. These agents have the potential to improve survival and quality of life for individuals with sickle cell Disease. Also discussed is stem cell transplantation that, to date, is the only curative approach for this Disease, as well as the current status of gene therapy. Conclusion These examples demonstrate how the current knowledge of sickle cell Disease pathophysiology and treatment approaches intersect. Although interest in sickle cell research has blossomed, many more clinical trials need to be initiated and subjected to more strenuous examination and analysis than have been used in the past.