Idiopathic Disease

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William W. Huang - One of the best experts on this subject based on the ideXlab platform.

  • Pyoderma gangrenosum: a review of pathogenesis and treatment.
    Expert Review of Clinical Immunology, 2018
    Co-Authors: Christine S. Ahn, Deborah Negus, William W. Huang
    Abstract:

    Introduction: Pyoderma gangrenosum (PG) is a complex neutrophilic dermatosis that can occur as an Idiopathic Disease, in association with systemic conditions such as inflammatory bowel Disease, as ...

  • pyoderma gangrenosum a review of pathogenesis and treatment
    Expert Review of Clinical Immunology, 2018
    Co-Authors: Deborah Negus, William W. Huang
    Abstract:

    ABSTRACTIntroduction: Pyoderma gangrenosum (PG) is a complex neutrophilic dermatosis that can occur as an Idiopathic Disease, in association with systemic conditions such as inflammatory bowel Disease, as part of an inherited inflammatory syndrome. It can be challenging to treat, as it occurs in a wide variety of clinical settings and there is a lack of a standardized treatment approach. The main limitations to treatment have been an incomplete understanding of the pathogenesis. However, recent advances have been made in understanding the pathogenesis of this condition, and PG is now considered an autoinflammatory Disease process.Areas covered: This review discusses the newest studies that further define our understanding of this Disease and the relevant literature on treatment options for pyoderma gangrenosum.Expert commentary: The presence of abnormal neutrophils and T-cells lead to immune dysregulation, leading to lesions of PG. Increased levels of inflammatory mediators including IL-1β, IL-8, IL-17, a...

Mohammed Al-qahtani - One of the best experts on this subject based on the ideXlab platform.

  • Individualized medicine enabled by genomics in Saudi Arabia
    BMC Medical Genomics, 2015
    Co-Authors: Muhammad Abu-elmagd, Mourad Assidi, Hans-juergen Schulten, Ashraf Dallol, Peter Natesan Pushparaj, Farid Ahmed, Stephen W Scherer, Mohammed Al-qahtani
    Abstract:

    The biomedical research sector in Saudi Arabia has recently received special attention from the government, which is currently supporting research aimed at improving the understanding and treatment of common Diseases afflicting Saudi Arabian society. To build capacity for research and training, a number of centres of excellence were established in different areas of the country. Among these, is the Centre of Excellence in Genomic Medicine Research (CEGMR) at King Abdulaziz University, Jeddah, with its internationally ranked and highly productive team performing translational research in the area of individualized medicine. Here, we present a panorama of the recent trends in different areas of biomedical research in Saudi Arabia drawing from our vision of where genomics will have maximal impact in the Kingdom of Saudi Arabia. We describe advances in a number of research areas including; congenital malformations, infertility, consanguinity and pre-implantation genetic diagnosis, cancer and genomic classifications in Saudi Arabia, epigenetic explanations of Idiopathic Disease, and pharmacogenomics and personalized medicine. We conclude that CEGMR will continue to play a pivotal role in advances in the field of genomics and research in this area is facing a number of challenges including generating high quality control data from Saudi population and policies for using these data need to comply with the international set up.

Roham T Zamanian - One of the best experts on this subject based on the ideXlab platform.

  • characterization of connective tissue Disease associated pulmonary arterial hypertension from reveal identifying systemic sclerosis as a unique phenotype
    Chest, 2010
    Co-Authors: Lorinda Chung, Lori Parsons, Paul M Hassoun, Michael D Mcgoon, David B Badesch, Dave P Miller, Mark R Nicolls, Roham T Zamanian
    Abstract:

    Background REVEAL (the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management) is the largest US cohort of patients with pulmonary arterial hypertension (PAH) confirmed by right-sided heart catheterization (RHC), providing a more comprehensive subgroup characterization than previously possible. We used REVEAL to analyze the clinical features of patients with connective tissue Disease-associated PAH (CTD-APAH). Methods All newly and previously diagnosed patients with World Health Organization (WHO) group 1 PAH meeting RHC criteria at 54 US centers were consecutively enrolled. Cross-sectional and 1-year mortality and hospitalization analyses from time of enrollment compared CTD-APAH to Idiopathic Disease and systemic sclerosis (SSc) to systemic lupus erythematosus (SLE), mixed connective tissue Disease (MCTD), and rheumatoid arthritis (RA). Results Compared with patients with Idiopathic Disease (n = 1,251), patients with CTD-APAH (n = 641) had better hemodynamics and favorable right ventricular echocardiographic findings but a higher prevalence of pericardial effusions, lower 6-min walk distance (300.5 ± 118.0 vs 329.4 ± 134.7 m, P = .01), higher B-type natriuretic peptide (BNP) levels (432.8 ± 789.1 vs 245.6 ± 427.2 pg/mL, P lco ) (44.9% ± 18.0% vs 63.6% ± 22.1% predicted, P lco (41.2% ± 16.3% predicted), and poorest 1-year survival (82% vs 94% in SLE-APAH, 88% in MCTD-APAH, and 96% in RA-APAH). Conclusions Patients with SSc-APAH demonstrate a unique phenotype with the highest BNP levels, lowest D lco , and poorest survival of all CTD-APAH subgroups. Trial registry ClinicalTrials.gov ; No.: NCT00370214 ; URL: clinicaltrials.gov

  • characterization of connective tissue Disease associated pulmonary arterial hypertension from reveal identifying systemic sclerosis as a unique phenotype
    Chest, 2010
    Co-Authors: Lorinda Chung, Lori Parsons, Paul M Hassoun, Michael D Mcgoon, David B Badesch, Dave P Miller, Mark R Nicolls, Roham T Zamanian
    Abstract:

    Background REVEAL (the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management) is the largest US cohort of patients with pulmonary arterial hypertension (PAH) confirmed by right-sided heart catheterization (RHC), providing a more comprehensive subgroup characterization than previously possible. We used REVEAL to analyze the clinical features of patients with connective tissue Disease-associated PAH (CTD-APAH). Methods All newly and previously diagnosed patients with World Health Organization (WHO) group 1 PAH meeting RHC criteria at 54 US centers were consecutively enrolled. Cross-sectional and 1-year mortality and hospitalization analyses from time of enrollment compared CTD-APAH to Idiopathic Disease and systemic sclerosis (SSc) to systemic lupus erythematosus (SLE), mixed connective tissue Disease (MCTD), and rheumatoid arthritis (RA). Results Compared with patients with Idiopathic Disease (n = 1,251), patients with CTD-APAH (n = 641) had better hemodynamics and favorable right ventricular echocardiographic findings but a higher prevalence of pericardial effusions, lower 6-min walk distance (300.5 ± 118.0 vs 329.4 ± 134.7 m, P = .01), higher B-type natriuretic peptide (BNP) levels (432.8 ± 789.1 vs 245.6 ± 427.2 pg/mL, P lco ) (44.9% ± 18.0% vs 63.6% ± 22.1% predicted, P P P = .03). Compared with patients with SSc-APAH (n = 399), those with other CTDs (SLE, n = 110; MCTD, n = 52; RA, n = 28) had similar hemodynamics; however, patients with SSc-APAH had the highest BNP levels (552.2 ± 977.8 pg/mL), lowest D lco (41.2% ± 16.3% predicted), and poorest 1-year survival (82% vs 94% in SLE-APAH, 88% in MCTD-APAH, and 96% in RA-APAH). Conclusions Patients with SSc-APAH demonstrate a unique phenotype with the highest BNP levels, lowest D lco , and poorest survival of all CTD-APAH subgroups. Trial registry ClinicalTrials.gov ; No.: NCT00370214 ; URL: clinicaltrials.gov

Deborah Negus - One of the best experts on this subject based on the ideXlab platform.

  • Pyoderma gangrenosum: a review of pathogenesis and treatment.
    Expert Review of Clinical Immunology, 2018
    Co-Authors: Christine S. Ahn, Deborah Negus, William W. Huang
    Abstract:

    Introduction: Pyoderma gangrenosum (PG) is a complex neutrophilic dermatosis that can occur as an Idiopathic Disease, in association with systemic conditions such as inflammatory bowel Disease, as ...

  • pyoderma gangrenosum a review of pathogenesis and treatment
    Expert Review of Clinical Immunology, 2018
    Co-Authors: Deborah Negus, William W. Huang
    Abstract:

    ABSTRACTIntroduction: Pyoderma gangrenosum (PG) is a complex neutrophilic dermatosis that can occur as an Idiopathic Disease, in association with systemic conditions such as inflammatory bowel Disease, as part of an inherited inflammatory syndrome. It can be challenging to treat, as it occurs in a wide variety of clinical settings and there is a lack of a standardized treatment approach. The main limitations to treatment have been an incomplete understanding of the pathogenesis. However, recent advances have been made in understanding the pathogenesis of this condition, and PG is now considered an autoinflammatory Disease process.Areas covered: This review discusses the newest studies that further define our understanding of this Disease and the relevant literature on treatment options for pyoderma gangrenosum.Expert commentary: The presence of abnormal neutrophils and T-cells lead to immune dysregulation, leading to lesions of PG. Increased levels of inflammatory mediators including IL-1β, IL-8, IL-17, a...

Muhammad Abu-elmagd - One of the best experts on this subject based on the ideXlab platform.

  • Individualized medicine enabled by genomics in Saudi Arabia
    BMC Medical Genomics, 2015
    Co-Authors: Muhammad Abu-elmagd, Mourad Assidi, Hans-juergen Schulten, Ashraf Dallol, Peter Natesan Pushparaj, Farid Ahmed, Stephen W Scherer, Mohammed Al-qahtani
    Abstract:

    The biomedical research sector in Saudi Arabia has recently received special attention from the government, which is currently supporting research aimed at improving the understanding and treatment of common Diseases afflicting Saudi Arabian society. To build capacity for research and training, a number of centres of excellence were established in different areas of the country. Among these, is the Centre of Excellence in Genomic Medicine Research (CEGMR) at King Abdulaziz University, Jeddah, with its internationally ranked and highly productive team performing translational research in the area of individualized medicine. Here, we present a panorama of the recent trends in different areas of biomedical research in Saudi Arabia drawing from our vision of where genomics will have maximal impact in the Kingdom of Saudi Arabia. We describe advances in a number of research areas including; congenital malformations, infertility, consanguinity and pre-implantation genetic diagnosis, cancer and genomic classifications in Saudi Arabia, epigenetic explanations of Idiopathic Disease, and pharmacogenomics and personalized medicine. We conclude that CEGMR will continue to play a pivotal role in advances in the field of genomics and research in this area is facing a number of challenges including generating high quality control data from Saudi population and policies for using these data need to comply with the international set up.