Search Results - "Banugaria, Suhrad"

Algorithm for the early diagnosis and treatment of patients with cross reactive immunologic material-negative classic infantile pompe disease: a step towards improving the efficacy of ERT by Banugaria, Suhrad G, Prater, Sean N, Patel, Trusha T, Dearmey, Stephanie M, Milleson, Christie, Sheets, Kathryn B, Bali, Deeksha S, Rehder, Catherine W, Raiman, Julian A J, Wang, Raymond A, Labarthe, Francois, Charrow, Joel, Harmatz, Paul, Chakraborty, Pranesh, Rosenberg, Amy S, Kishnani, Priya S

“…Although enzyme replacement therapy (ERT) is a highly effective therapy, CRIM-negative (CN) infantile Pompe disease (IPD) patients typically mount a strong…”
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Skeletal muscle pathology of infantile Pompe disease during long-term enzyme replacement therapy by Prater, Sean N, Patel, Trusha T, Buckley, Anne F, Mandel, Hanna, Vlodavski, Eugene, Banugaria, Suhrad G, Feeney, Erin J, Raben, Nina, Kishnani, Priya S

“…Pompe disease is an autosomal recessive metabolic neuromuscular disorder caused by a deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA). It has…”
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The impact of antibodies on clinical outcomes in diseases treated with therapeutic protein: Lessons learned from infantile Pompe disease by Banugaria, Suhrad G, Prater, Sean N, Ng, Yiu-Ki, Kobori, Joyce A, Finkel, Richard S, Ladda, Roger L, Chen, Yuan-Tsong, Rosenberg, Amy S, Kishnani, Priya S

“…Purpose: Enzyme replacement therapy with rhGAA (Myozyme®) has lead to improved survival, which is largely attributable to improvements in cardiomyopathy and…”
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Neuroimaging findings in infantile Pompe patients treated with enzyme replacement therapy by McIntosh, Paul T., Hobson-Webb, Lisa D., Kazi, Zoheb B., Prater, Sean N., Banugaria, Suhrad G., Austin, Stephanie, Wang, Raymond, Enterline, David S., Frush, Donald P., Kishnani, Priya S.

“…Recombinant human acid α-glucosidase (rhGAA) enzyme replacement therapy (ERT) has prolonged survival in infantile Pompe disease (IPD), but has unmasked central…”
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The emerging phenotype of long-term survivors with infantile Pompe disease by Prater, Sean N., Banugaria, Suhrad G., DeArmey, Stephanie M., Botha, Eleanor G., Stege, Erin M., Case, Laura E., Jones, Harrison N., Phornphutkul, Chanika, Wang, Raymond Y., Young, Sarah P., Kishnani, Priya S.

“…Purpose: Enzyme replacement therapy with alglucosidase alfa for infantile Pompe disease has improved survival creating new management challenges. We describe…”
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The impact of antibodies in late-onset Pompe disease: A case series and literature review by Patel, Trusha T., Banugaria, Suhrad G., Case, Laura E., Wenninger, Stephan, Schoser, Benedikt, Kishnani, Priya S.

“…Pompe disease (glycogen storage disease type II, GSD II) is an autosomal recessive disease caused by a deficiency of acid α-glucosidase (GAA), leading to…”
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Predicting cross-reactive immunological material (CRIM) status in Pompe disease using GAA mutations: Lessons learned from 10 years of clinical laboratory testing experience by Bali, Deeksha S., Goldstein, Jennifer L., Banugaria, Suhrad, Dai, Jian, Mackey, Joanne, Rehder, Catherine, Kishnani, Priya S.

“…Enzyme replacement therapy (ERT) for Pompe disease using recombinant acid alpha‐glucosidase (rhGAA) has resulted in increased survival although the clinical…”
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Enhanced efficacy of enzyme replacement therapy in Pompe disease through mannose-6-phosphate receptor expression in skeletal muscle by Koeberl, Dwight D., Luo, Xiaoyan, Sun, Baodong, McVie-Wylie, Alison, Dai, Jian, Li, Songtao, Banugaria, Suhrad G., Chen, Y.-T., Bali, Deeksha S.

“…Enzyme replacement therapy (ERT) with acid α-glucosidase has become available for Pompe disease; however, the response of skeletal muscle, as opposed to the…”
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Persistence of high sustained antibodies to enzyme replacement therapy despite extensive immunomodulatory therapy in an infant with Pompe disease: Need for agents to target antibody-secreting plasma cells by Banugaria, Suhrad G., Patel, Trusha T., Mackey, Joanne, Das, Stuti, Amalfitano, Andrea, Rosenberg, Amy S., Charrow, Joel, Chen, Y.-T., Kishnani, Priya S.

“…With the advent of enzyme replacement therapy (ERT) with alglucosidase alfa (rhGAA, Myozyme®) for Pompe disease, the clinical course of the disease has…”
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Bortezomib in the rapid reduction of high sustained antibody titers in disorders treated with therapeutic protein: lessons learned from Pompe disease by Banugaria, Suhrad G., Prater, Sean N., McGann, Judeth K., Feldman, Jonathan D., Tannenbaum, Jesse A., Bailey, Carrie, Gera, Renuka, Conway, Robert L., Viskochil, David, Kobori, Joyce A., Rosenberg, Amy S., Kishnani, Priya S.

“…Purpose: High sustained antibody titers complicate many disorders treated with a therapeutic protein, including those treated with enzyme replacement therapy,…”
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Oropharyngeal Dysphagia in Infants and Children with Infantile Pompe Disease by Jones, Harrison N., Muller, Carolyn W., Lin, Min, Banugaria, Suhrad G., Case, Laura E., Li, Jennifer S., O’Grady, Gwendolyn, Heller, James H., Kishnani, Priya S.

“…Pompe disease is a rare genetic progressive neuromuscular disorder. The most severe form, infantile Pompe disease, has historically resulted in early…”
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Immune modulation in Pompe disease treated with enzyme replacement therapy by Banugaria, Suhrad G, Patel, Trusha T, Kishnani, Priya S

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Basilar artery aneurysm: A new finding in classic infantile pompe disease by Patel, Trusha T., Banugaria, Suhrad G., Frush, Donald P., Enterline, David S., Tanpaiboon, Pranoot, Kishnani, Priya S.

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Clinical and histologic ocular findings in pompe disease by Yanovitch, Tammy L, Banugaria, Suhrad G, Proia, Alan D, Kishnani, Priya S

“…Limited information is available on the ocular findings in patients with Pompe disease. This study summarizes this information with a systematic literature…”
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Letter to the Editors: Concerning “CRIM-negative Pompe disease patients with satisfactory clinical outcomes on enzyme replacement therapy” by Al Khallaf et al by Prater, Sean N., Banugaria, Suhrad G., Morgan, Claire, Sung, Crystal C., Rosenberg, Amy S., Kishnani, Priya S.

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Atypical immunologic response in a patient with CRIM-negative Pompe disease by Abbott, Mary-Alice, Prater, Sean N., Banugaria, Suhrad G., Richards, Susan M., Young, Sarah P., Rosenberg, Amy S., Kishnani, Priya S.

“…We report the clinical course of a patient with severe infantile onset Pompe disease [cross-reactive immunologic material (CRIM) negative, R854X/R854X] who was…”
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Hypovitaminosis D in glycogen storage disease type I by Banugaria, Suhrad G., Austin, Stephanie L., Boney, Anne, Weber, Thomas J., Kishnani, Priya S.

“…Glycogen storage disease type I (GSD I) is caused by inherited defects of the glucose 6-phosphatase complex, resulting in fasting hypoglycemia, lactic…”
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Non-depleting anti-CD4 monoclonal antibody induces immune tolerance to ERT in a murine model of Pompe disease by Sun, Baodong, Banugaria, Suhrad G, Prater, Sean N, Patel, Trusha T, Fredrickson, Keri, Ringler, Douglas J, de Fougerolles, Antonin, Rosenberg, Amy S, Waldmann, Herman, Kishnani, Priya S

“…Approximately 35-40% of patients with classic infantile Pompe disease treated with enzyme replacement therapy (ERT) develop high, sustained antibody titers…”
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Improvement of Bilateral Ptosis on Higher Dose Enzyme Replacement Therapy in Pompe Disease by Yanovitch, Tammy L, Casey, Robin, Banugaria, Suhrad G, Kishnani, Priya S

“…Pompe disease is a lysosomal disorder caused by deficiency of acid alpha -glucosidase (GAA) that leads to accumulation of glycogen in multiple tissues. Enzyme…”
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The Use of a Reduced-Intensity Conditioning (RIC) Regimen in Patients Ages 2-30 Undergoing Allogeneic Transplantation for Sickle Cell Disease by Banugaria, Suhrad, MD, Gillio, Alfred P., MD, Haugh, Jeanette, RN, APN, CCRC, Durning, Nancy, APN, CBMT, Nycz, Melanie, APN, CPHON, CPNP-PC, Adler-Brecher, Barbara, MS, Krajewski, Jennifer, MD

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