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1. Childhood and Adolescent Obesity: A Review

Author

Alvina R. Kansra, Sinduja Lakkunarajah, and M. Susan Jay

Subjects
obesity, childhood, review (article), behavior, adolescent, Pediatrics, RJ1-570
Abstract

Obesity is a complex condition that interweaves biological, developmental, environmental, behavioral, and genetic factors; it is a significant public health problem. The most common cause of obesity throughout childhood and adolescence is an inequity in energy balance; that is, excess caloric intake without appropriate caloric expenditure. Adiposity rebound (AR) in early childhood is a risk factor for obesity in adolescence and adulthood. The increasing prevalence of childhood and adolescent obesity is associated with a rise in comorbidities previously identified in the adult population, such as Type 2 Diabetes Mellitus, Hypertension, Non-alcoholic Fatty Liver disease (NAFLD), Obstructive Sleep Apnea (OSA), and Dyslipidemia. Due to the lack of a single treatment option to address obesity, clinicians have generally relied on counseling dietary changes and exercise. Due to psychosocial issues that may accompany adolescence regarding body habitus, this approach can have negative results. Teens can develop unhealthy eating habits that result in Bulimia Nervosa (BN), Binge- Eating Disorder (BED), or Night eating syndrome (NES). Others can develop Anorexia Nervosa (AN) as they attempt to restrict their diet and overshoot their goal of “being healthy.” To date, lifestyle interventions have shown only modest effects on weight loss. Emerging findings from basic science as well as interventional drug trials utilizing GLP-1 agonists have demonstrated success in effective weight loss in obese adults, adolescents, and pediatric patients. However, there is limited data on the efficacy and safety of other weight-loss medications in children and adolescents. Nearly 6% of adolescents in the United States are severely obese and bariatric surgery as a treatment consideration will be discussed. In summary, this paper will overview the pathophysiology, clinical, and psychological implications, and treatment options available for obese pediatric and adolescent patients.

Published
2021
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2. Contributors

Author

Omar Ali, Louella B. Amos, Bethany Auble, Donald Basel, Shannon H. Baumer-Mouradian, Ashley Beattie, Geetanjali Bora, Brett J. Bordini, Brian R. Branchford, Amanda M. Brandow, Ryan Byrne, Gisela G. Chelimsky, Thomas C. Chelimsky, Paula Cody, Gary Cohen, Deborah M. Costakos, Emily M. Densmore, John C. Densmore, Patricia A. Donohoue, Amy L. Drendel, Garrett Elsner, Raquel Farias-Moeller, Shayne D. Fehr, Susan Feigelman, Veronica H. Flood, Jessica Francis, Julia Fritz, Sandra Gage, Bhaskar Gurram, Matthew M. Harmelink, Kristen E. Holland, Stephen R. Humphrey, Anna R. Huppler, Susan L. Jarosz, S. Anne Joseph, Alvina R. Kansra, Virginia Keane, Robert M. Kliegman, Julie M. Kolinski, Chamindra G. Konersman, Kathleen A. Koth, Katja Kovacic, Amornluck Krasaelap, John V. Kryger, Sara M. Lauck, Tracey H. Liljestrom, Ahmad Marashly, Seema Menon, Adrian Miranda, Michelle L. Mitchell, Amy Moskop, Michael Muriello, James J. Nocton, Joshua Noe, Cynthia G. Pan, Andrew N. Pelech, Brittany Player, Jacquelyn M. Powers, Angela L. Rabbitt, Amanda Rogers, John M. Routes, Mark Simms, Rajasree Sreedharan, Alyssa Stephany, Julie Talano, Grzegorz W. Telega, Heather Toth, Scott K. Van Why, Sarah Vepraskas, James W. Verbsky, Bernadette Vitola, Kevin D. Walter, Michael Weisgerber, Peter M. Wolfgram, Sarah C. Yale, and Alicia C. Zolkoske

Published
2023
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4. Contributors

Author

Omar Ali, Louella B. Amos, Donald Basel, Brett J. Bordini, Amanda M. Brandow, Ryan Byrne, Yvonne E. Chiu, Dominic Co, Paula Cody, Gary Cohen, Deborah M. Costakos, Emily M. Densmore, John C. Densmore, Patricia A. Donohoue, Shayne D. Fehr, Susan Feigelman, Veronica H. Flood, Jessica Francis, Julia Fritz, Sandra Gage, Bhaskar Gurram, Kristen E. Holland, Stephen R. Humphrey, Alvina R. Kansra, Virginia Keane, Kirstin Kirschner, Julie M. Kolinski, Chamindra Konersman, John V. Kryger, Jacquelyn C. Kuzminski, Sara M. Lauck, Patricia S. Lye, Seema Menon, Adrian Miranda, Priya Monrad, James Nocton, Joshua Noe, Richard J. Noel, Cynthia G. Pan, Andrew N. Pelech, Brittany Player, Angela L. Rabbitt, Amanda Rogers, John R. Routes, J. Paul Scott, Anjali Sharma, Mark Simms, Paula J. Soung, Raji Sreedharan, Robert R. Tanz, Grzegorz W. Telega, John G. Thometz, Heather Toth, Scott K. Van Why, Sarah Vepraskas, James W. Verbsky, Bernadette Vitola, Michael Weisgerber, and Peter M. Wolfgram

Published
2018
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6. Stable Stretched Suspended DNA Molecules

Author

Trapane, Pamela L, James C Hyl, and Alvina R. Kansra

Subjects
Genetics, business.industry, 02 engineering and technology, General Medicine, 010402 general chemistry, 021001 nanoscience & nanotechnology, Infantile hypophosphatasia, Omics, 01 natural sciences, 0104 chemical sciences, Novel gene, Mutation (genetic algorithm), Medicine, Presentation (obstetrics), 0210 nano-technology, business
Published
2016
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7. Comparison of Clinical and Biochemical Parameters in Adolescent Girls with Polycystic Ovary Syndrome in Different Clinical Settings

Author

Marquart K and Alvina R. Kansra

Subjects
Gynecology, Pediatrics, medicine.medical_specialty, business.industry, Clinical settings, medicine.disease, Omics, Polycystic ovary, Adolescent medicine, medicine, In patient, Metabolic syndrome, business, Body mass index, hirsutism
Abstract

Objective: The aim of this study is to identify different polycystic ovary syndrome (PCOS) phenotypes in adolescent girls presenting to different clinical subspecialties and assess the metabolic syndrome (MS) among these phenotypes. Design: Retrospective chart review of Adolescent girls with PCOS seen in Pediatric Endocrine (PEndo), Pediatric Adolescent Medicine (PAMed) clinics. Main outcome measure: Compare the clinical and laboratory hallmarks for PCOS and evaluate for MS among adolescent populations presenting in sub-specialty clinics. Results: One hundred and sixty two charts from PEndo, PAMed clinics on post-menarchal girls with PCOS diagnosis were reviewed. Adolescent girls presented in PEndo clinic have distinct PCOS phenotype that showed statistically significant free testosterone (FT) (p=0.0257) with possibly more hirsutism. In addition 17 hydroxyprogesterone (17 OHP) levels were higher (p=0.0257) in patients from PEndo clinic as compared to other clinic. To quantify the risk of MS, we regrouped patients having body mass index (BMI)>90 percentile from both the clinics and divided them in hyper-androgenemia (HA) if FT ≥ 4.0 pg/mL and non-HA phenotype. 35.9% (28/78) met the criteria for MS in HA phenotype. When compared, HA phenotype had higher rate of MS as compared to non- HA (35.9% Vs 0.1%). Conclusion: Adolescent girls with PCOS presenting in the sub-specialty clinics are likely to have different phenotypes. HA phenotype had increased rate of MS syndrome. Understanding the heterogeneous nature of this disorder will address specific health needs of an individual patient and help us tailor appropriate medical therapy.

Published
2016
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9. Pseudohypoparathyroidism Type Ia-Clinical Case with a Novel Mutation of GNAS1 Gene

Author

Steven J Steinberg, Alvina R. Kansra, and Hanaa Zidan

Subjects
musculoskeletal diseases, endocrine system, medicine.medical_specialty, biology, business.industry, Parathyroid hormone, General Medicine, Growth hormone–releasing hormone, medicine.disease, Short stature, Endocrinology, Internal medicine, medicine, GNAS complex locus, biology.protein, Pseudopseudohypoparathyroidism, medicine.symptom, Luteinizing hormone, business, Pseudohypoparathyroidism, Hormone
Abstract

Pseudohypoparathyroidism (PHP) is a rare autosomal dominant disorder resulting from loss of function mutations in the GNAS gene. Several forms of PHP are noted. PHP type 1a occurs most commonly and is characterized by physical features termed Albright’s Hereditary Osteodystrophy (AHO), a constellation of physical features which may include short stature, obesity, round facies, heterotopic ossification, brachydactyly and mental retardation, and increased levels of parathyroid hormone (PTH) due to the end organ hormone resistance to its action. Here we report a new GNAS mutation in a 3.5 years old African American female patient with a history of round facies, developmental delays, obesity and seizure disorder; she was admitted for apneic episode and noted to have prolonged QTc interval on cardiac monitor. A lab evaluation showed severe hypocalcaemia, hyperphosphatemia, high PTH with normal magnesium and alkaline phosphatase levels. She also had slightly elevated Thyroid Stimulating Hormone (TSH) levels indicative of type 1 a PHP where resistance to multiple Gs protein-coupled hormones (e.g. PTH, TSH, Luteinizing Hormone (LH), Follicular Stimulating Hormone (FSH), and Growth Hormone Releasing Hormone (GHRH)) is present. Full genomic DNA sequencing of the exons and adjacent intronic regions of the GNAS gene revealed a novel heterozygous mutation in intron 7, c.585+1G>A, in both the patient and her mother.

Published
2013
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11. IGF receptor gene variants in normal adolescents: effect on stature

Author

Ranjan Deka, Alvina R. Kansra, Lisa J. Martin, Lawrence M. Dolan, and Steven D. Chernausek

Subjects
medicine.medical_specialty, Adolescent, Genotype, Cross-sectional study, Endocrinology, Diabetes and Metabolism, Population, IGFBP3, Enzyme-Linked Immunosorbent Assay, Biology, medicine.disease_cause, Receptor, IGF Type 1, Loss of heterozygosity, Endocrinology, Internal medicine, medicine, Humans, Insulin-Like Growth Factor I, education, Gene, Insulin-like growth factor 1 receptor, education.field_of_study, Mutation, General Medicine, Body Height, Cross-Sectional Studies, Insulin-Like Growth Factor Binding Protein 3
Abstract

Objective: IGF1 is essential for human growth and mediates its effects through the type 1 IGF receptor (IGF1R). Our objective was to determine the frequency of certain previously reported IGF1R gene variants in the normal population and their effect on stature. Design: A cross-sectional study was conducted in a population of 2500 children enrolled in public school grades 5 through 12 for whom DNA and anthropometric data were available. Subjects were genotyped at five previously reported loci that affect receptor abundance or function. Methods: The frequency of the following IGF1R variants Arg108Gln, Lys115Asn, Arg59stop, Arg481Gln, and Arg605His was measured by a PCR-based assay. Circulating concentrations of IGF1 or IGF binding protein-3 (IGFBP3) were measured by ELISA in those affected and matched controls. Results: A scan of 1300 subjects detected none with Arg108Gln, Lys115Asn, or Arg59stop mutations. In contrast, nucleotide changes leading to heterozygosity at codon 605 were identified in nine of 2500 subjects and six of 1800 subjects at codon 481. These individuals were, on average, 4 cm shorter than the others. There were no differences in circulating concentrations of IGF1 or IGFBP3 between those with the gene variants and controls matched for sex, ethnicity, age, and BMI. Conclusion: Rare IGF1R variants exerting a moderate effect on stature are present in the general population, supporting the importance of IGF1R function in growth control and indicating that variation in height within healthy individuals may be explained, in some cases, by larger effects of a small subset of gene variants.

Published
2012

13. PCOS: perspectives from a pediatric endocrinologist and a pediatric gynecologist

Author

Alvina R. Kansra and Seema Menon

Subjects
Infertility, medicine.medical_specialty, Adolescent, MEDLINE, Testosterone blood, Acneiform Eruptions, Pediatric endocrinologist, Physicians, medicine, Prevalence, Humans, Testosterone, Obesity, Gynecology, business.industry, General Medicine, Luteinizing Hormone, medicine.disease, Atherosclerosis, Polycystic ovary, Adolescent population, Early Diagnosis, Adolescent Health Services, Pediatrics, Perinatology and Child Health, Childbearing age, Female, Follicle Stimulating Hormone, business, Hyperandrogenism, Infertility, Female, Polycystic Ovary Syndrome
Abstract

Polycystic ovary syndrome is the most common endocrinopathy recognized in women of childbearing age with a prevalence of 4-12%. The prevalence of the disorder in adolescent population is poorly defined. The pathogenesis as well as the management of this disorder is widely debated.

Published
2012

15. Contributors

Author

Jon S. Abramson, Mark J. Abzug, John J. Aiken, H. Hesham A-kader, Cezmi A. Akdis, Harold Alderman, Ramin Alemzadeh, Evaline A. Alessandrini, Omar Ali, Namasivayam Ambalavanan, Karl E. Anderson, Peter M. Anderson, Kelly K. Anthony, Alia Y. Antoon, Stacy P. Ardoin, Carola A.S. Arndt, Stephen S. Arnon, Stephen C. Aronoff, David M. Asher, Barbara L. Asselin, Joann L. Ater, Dan Atkins, Erika F. Augustine, Marilyn Augustyn, Ellis D. Avner, Parvin H. Azimi, Carlos A. Bacino, Robert N. Baldassano, Christina Bales, William F. Balistreri, Robert S. Baltimore, Manisha Balwani, Shahida Baqar, Christine E. Barron, Dorsey M. Bass, Mark L. Batshaw, Richard E. Behrman, Michael J. Bell, John W. Belmont, Daniel K. Benjamin, Michael J. Bennett, Daniel Bernstein, Jatinder Bhatia, Zulfiqar Ahmed Bhutta, Leslie G. Biesecker, James Birmingham, Samra S. Blanchard, Ronald Blanton, Archie Bleyer, C.D.R. Lynelle M. Boamah, Steven R. Boas, Thomas F. Boat, Walter Bockting, Mark Boguniewicz, Daniel J. Bonthius, Laurence A. Boxer, Amanda M. Brandow, David Branski, David T. Breault, Rebecca H. Buckley, Cynthia Etzler Budek, E. Stephen Buescher, Gale R. Burstein, Amaya Lopez Bustinduy, Mitchell S. Cairo, Bruce M. Camitta, Angela Jean Peck Campbell, Rebecca G. Carey, Waldemar A. Carlo, Robert B. Carrigan, Mary T. Caserta, Ellen Gould Chadwick, Lisa J. Chamberlain, Jennifer I. Chapman, Ira M. Cheifetz, Wassim Chemaitilly, Sharon F. Chen, Yuan-Tsong Chen, Russell W. Chesney, Jennifer A. Chiriboga, Robert D. Christensen, Andrew Chu, Michael J. Chusid, Theodore J. Cieslak, Jeff A. Clark, Thomas G. Cleary, John David Clemens, Joanna S. Cohen, Mitchell B. Cohen, Pinchas Cohen, Michael Cohen-Wolkowiez, Robert A. Colbert, F. Sessions Cole, Joanna C.M. Cole, John L. Colombo, Amber R. Cooper, Ronina A. Covar, Barbara Cromer, James E. Crowe, Natoshia Raishevich Cunningham, Steven J. Czinn, Toni Darville, Robert S. Daum, Richard S. Davidson, H. Dele Davies, Peter S. Dayan, Michael R. DeBaun, Guenet H. Degaffe, David R. DeMaso, Mark R. Denison, Arlene E. Dent, Nirupama K. DeSilva, Robert J. Desnick, Gabrielle deVeber, Esi Morgan DeWitt, Chetan Anil Dhamne, Anil Dhawan, Harry Dietz, Lydia J. Donoghue, Patricia A. Donohoue, Mary K. Donovan, John P. Dormans, Daniel A. Doyle, Jefferson Doyle, Stephen C. Dreskin, Denis S. Drummond, Howard Dubowitz, J. Stephen Dumler, Janet Duncan, Paula M. Duncan, LauraLe Dyner, Michael G. Earing, Elizabeth A. Edgerton, Marie Egan, Jack S. Elder, Sara B. Eleoff, Dianne S. Elfenbein, Stephen C. Eppes, Michele Burns Ewald, Jessica K. Fairley, Susan Feigelman, Marianne E. Felice, Eric I. Felner, Edward Fels, Thomas Ferkol, Jonathan D. Finder, Kristin N. Fiorino, David M. Fleece, Patricia M. Flynn, Joel A. Forman, Michael M. Frank, Melvin H. Freedman, Melissa Frei-Jones, Jared E. Friedman, Sheila Gahagan, Paula Gardiner, Luigi Garibaldi, Gregory M. Gauthier, Abraham Gedalia, Matthew J. Gelmini, Michael A. Gerber, K. Michael Gibson, Mark Gibson, Francis Gigliotti, Walter S. Gilliam, Janet R. Gilsdorf, Charles M. Ginsburg, Frances P. Glascoe, Donald A. Goldmann, Denise M. Goodman, Marc H. Gorelick, Gary J. Gosselin, Jane M. Gould, Olivier Goulet, Dan M. Granoff, Michael Green, Thomas P. Green, Larry A. Greenbaum, Marie Michelle Grino, Andrew B. Grossman, David C. Grossman, Alfredo Guarino, Lisa R. Hackney, Gabriel G. Haddad, Joseph Haddad, Joseph F. Hagan, Scott B. Halstead, Margaret R. Hammerschlag, Aaron Hamvas, James C. Harris, Mary E. Hartman, David B. Haslam, Fern R. Hauck, Gregory F. Hayden, Jacqueline T. Hecht, Sabrina M. Heidemann, J. Owen Hendley, Fred M. Henretig, Gloria P. Heresi, Andrew D. Hershey, Cynthia E. Herzog, Jessica Hochberg, Lauren D. Holinger, Jeffrey D. Hord, B. David Horn, William A. Horton, Harish S. Hosalkar, Hidekazu Hosono, Peter J. Hotez, Michelle S. Howenstine, Heather G. Huddleston, Vicki Huff, Denise Hug, Winston W. Huh, Carl E. Hunt, Anna Klaudia Hunter, Patricia Ibeziako, Richard F. Jacobs, Peter Jensen, Hal B. Jenson, Chandy C. John, Michael V. Johnston, Richard B. Johnston, Bridgette L. Jones, James F. Jones, Marsha Joselow, Anupama Kalaskar, Linda Kaljee, Deepak Kamat, Alvina R. Kansra, Sheldon L. Kaplan, Emily R. Katz, James W. Kazura, Virginia Keane, Gregory L. Kearns, Desmond P. Kelly, Judith Kelsen, Kathi J. Kemper, Melissa Kennedy, Eitan Kerem, Joseph E. Kerschner, Seema Khan, Young-Jee Kim, Charles H. King, Stephen L. Kinsman, Adam Kirton, Priya S. Kishnani, Nora T. Kizer, Martin B. Kleiman, Bruce L. Klein, Bruce S. Klein, Michael D. Klein, Robert M. Kliegman, William C. Koch, Patrick M. Kochanek, Eric Kodish, Stephan A. Kohlhoff, Elliot J. Krane, Peter J. Krause, Richard E. Kreipe, Steven E. Krug, John F. Kuttesch, Jennifer M. Kwon, Catherine S. Lachenauer, Stephan Ladisch, Stephen LaFranchi, Oren Lakser, Marc B. Lande, Philip J. Landrigan, Gregory L. Landry, Wendy G. Lane, Philip S. LaRussa, Brendan Lee, Chul Lee, K. Jane Lee, J. Steven Leeder, Rebecca K. Lehman, Michael J. Lentze, Norma B. Lerner, Steven Lestrud, Donald Y.M. Leung, Chris A. Liacouras, Susanne Liewer, Andrew H. Liu, Stanley F. Lo, Franco Locatelli, Sarah S. Long, Anna Lena Lopez, Steven V. Lossef, Jennifer A. Lowry, Kerith Lucco, G. Reid Lyon, Prashant V. Mahajan, Akhil Maheshwari, Joseph A. Majzoub, Asim Maqbool, Ashley M. Maranich, Mona Marin, Joan C. Marini, Morri Markowitz, Kevin P. Marks, Stacene R. Maroushek, Wilbert H. Mason, Christopher Mastropietro, Kimberlee M. Matalon, Reuben K. Matalon, Robert Mazor, Susanna A. McColley, Margaret M. McGovern, Heather S. McLean, Rima McLeod, Peter C. Melby, Joseph John Melvin, Diane F. Merritt, Ethan A. Mezoff, Marian G. Michaels, Alexander G. Miethke, Mohamad A. Mikati, Henry Milgrom, E. Kathryn Miller, Jonathan W. Mink, Grant A. Mitchell, Robert R. Montgomery, Joseph G. Morelli, Anna-Barbara Moscicki, Hugo W. Moser, Kathryn D. Moyer, James R. Murphy, Timothy F. Murphy, Thomas S. Murray, Mindo J. Natale, William A. Neal, Jayne Ness, Kathleen A. Neville, Mary A. Nevin, Jane W. Newburger, Peter E. Newburger, Linda S. Nield, Zehava Noah, Lawrence M. Nogee, Robert L. Norris, Stephen K. Obaro, Makram Obeid, Theresa J. Ochoa, Katherine A. O'Donnell, Robin K. Ohls, Jean-Marie Okwo-Bele, Keith T. Oldham, Scott E. Olitsky, John Olsson, Susan R. Orenstein, Walter A. Orenstein, Judith A. Owens, Charles H. Packman, Michael J. Painter, Priya Pais, Cynthia G. Pan, Vijay Pannikar, Diane E. Pappas, Anjali Parish, John S. Parks, Laura A. Parks, Maria Jevitz Patterson, Pallavi P. Patwari, Timothy R. Peters, Larry K. Pickering, Misha L. Pless, Laura S. Plummer, Craig C. Porter, Dwight A. Powell, David T. Price, Charles G. Prober, Linda Quan, Elisabeth H. Quint, C. Egla Rabinovich, Leslie J. Raffini, Denia Ramirez-Montealegre, Giuseppe Raviola, Ann M. Reed, Harold L. Rekate, Megan E. Reller, Gary Remafedi, Jorge D. Reyes, Geoffrey Rezvani, Iraj Rezvani, A. Kim Ritchey, Frederick P. Rivara, Angela Byun Robinson, Luise E. Rogg, Genie E. Roosevelt, David R. Rosenberg, Melissa Beth Rosenberg, David S. Rosenblatt, Cindy Ganis Roskind, Mary M. Rotar, Ranna A. Rozenfeld, Sarah Zieber Rush, Colleen A. Ryan, H.P.S. Sachdev, Ramesh C. Sachdeva, Mustafa Sahin, Robert A. Salata, Denise A. Salerno, Edsel Maurice T. Salvana, Hugh A. Sampson, Thomas J. Sandora, Tracy Sandritter, Wudbhav N. Sankar, Ajit Ashok Sarnaik, Ashok P. Sarnaik, Harvey B. Sarnat, Minnie M. Sarwal, Mary Saunders, Laura E. Schanberg, Mark R. Schleiss, Nina F. Schor, Bill J. Schroeder, Robert L. Schum, Gordon E. Schutze, Daryl A. Scott, J. Paul Scott, Theodore C. Sectish, George B. Segel, Kriti Sehgal, Ernest G. Seidman, Janet R. Serwint, Dheeraj Shah, Raanan Shamir, Bruce K. Shapiro, Richard J. Shaw, Bennett A. Shaywitz, Sally E. Shaywitz, Meera Shekar, Elena Shephard, Philip M. Sherman, Benjamin L. Shneider, Scott H. Sicherer, Richard Sills, Mark D. Simms, Eric A.F. Simões, Thomas L. Slovis, P. Brian Smith, Mary Beth F. Son, Laura Stout Sosinsky, Joseph D. Spahn, Mark A. Sperling, Robert Spicer, David A. Spiegel, Helen Spoudeas, Jürgen Spranger, Rajasree Sreedharan, Raman Sreedharan, Shawn J. Stafford, Margaret M. Stager, Sergio Stagno, Virginia A. Stallings, Lawrence R. Stanberry, Charles A. Stanley, Bonita F. Stanton, Jeffrey R. Starke, Merrill Stass-Isern, Barbara W. Stechenberg, Leonard D. Stein, William J. Steinbach, Nicolas Stettler, Barbara J. Stoll, Gregory A. Storch, Ronald G. Strauss, Frederick J. Suchy, Karen Summar, Moira Szilagyi, Norman Tinanoff, James K. Todd, Lucy S. Tompkins, Richard L. Tower, Riccardo Troncone, Amanda A. Trott, David G. Tubergen, David A. Turner, Ronald B. Turner, Christina Ullrich, George F. Van Hare, Jakko van Ingen, Heather A. Van Mater, Dick van Soolingen, Scott K. Van Why, Pankhuree Vandana, Douglas Vanderbilt, Jon A. Vanderhoof, Andrea Velardi, Elliott Vichinsky, Linda A. Waggoner-Fountain, Steven G. Waguespack, David M. Walker, Heather J. Walter, Stephanie Ware, Kimberly Danieli Watts, Ian M. Waxman, Debra E. Weese-Mayer, Kathryn Weise, Martin E. Weisse, Lawrence Wells, Jessica Wen, Steven L. Werlin, Michael R. Wessels, Ralph F. Wetmore, Randall C. Wetzel, Isaiah D. Wexler, Perrin C. White, John V. Williams, Rodney E. Willoughby, Samantha L. Wilson, Glenna B. Winnie, Paul H. Wise, Laila Woc-Colburn, Joanne Wolfe, Cynthia J. Wong, Laura L. Worth, Joseph L. Wright, Peter F. Wright, Terry W. Wright, Eveline Y. Wu, Anthony Wynshaw-Boris, Nada Yazigi, Ram Yogev, Marc Yudkoff, Peter E. Zage, Anita K.M. Zaidi, Lonnie K. Zeltzer, Maija H. Zile, Peter Zimmer, and Barry Zuckerman

Published
2011
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17. Abnormalities in glucose tolerance are common in children with fanconi anemia and associated with impaired insulin secretion

Author

Deborah A. Elder, David A. D'Alessio, Franklin O. Smith, Ori Eyal, Susan R. Rose, Alvina R. Kansra, and Robin Mueller

Subjects
Male, medicine.medical_specialty, Adolescent, Carbohydrate metabolism, Insulin resistance, Fanconi anemia, Internal medicine, Diabetes mellitus, Insulin Secretion, medicine, Humans, Insulin, Child, Glycemic, Glucose tolerance test, medicine.diagnostic_test, business.industry, nutritional and metabolic diseases, Infant, Hematology, Metabolism, Glucose Tolerance Test, medicine.disease, Endocrinology, Fanconi Anemia, Glucose, Oncology, Child, Preschool, Pediatrics, Perinatology and Child Health, Homeostatic model assessment, Female, Insulin Resistance, business
Abstract

BACKGROUND To determine prevalence of abnormal glucose metabolism in Fanconi Anemia (FA). PROCEDURE Thirty-nine children with FA underwent 2-hr oral glucose tolerance test (OGTT). Reference lean adolescents (REF) were older than FA patients (mean +/- SD: FA 8.6 +/- 3.9 years, REF 19.8 +/- 0.3 years, P < 0.001), but comparable in BMI Z-scores (FA 1.25 +/- 0.58, REF -0.02 +/- 0.24; P = 0.24). Patients had normal glucose tolerance (NGT) or abnormal glucose metabolism (AGM) by American Diabetes Association Criteria. Insulinogenic index estimated beta-cell function. Insulin resistance estimation used homeostatic model assessment (HOMA-IR). Insulin secretion estimation relative to insulin sensitivity used disposition index (DI). RESULTS Among FA patients, 46% had AGM. Compared to REF, there were significant differences in glycemic responses (area under curve: FA-NGT 344 +/- 42, FA-AGM 596 +/- 35, REF 208 +/- 25 mM, P < 0.0001) and insulinogenic index (FA-NGT 105 +/- 29, FA-AGM 44 +/- 8, and REF 173 +/- 41 pM/mM, P < 0.05). Insulin sensitivity did not differ among NGT, AGM, and REF (HOMA-IR: FA-NGT 1.9 +/- 0.4, FA-AGM 2.2 +/- 0.5, REF 1.3 +/- 0.2, P = NS). However, DI was significantly lower in both FA groups than REF [NGT 63.6 +/- 16.5 vs. AGM 26.4 +/- 3.5 (P < 0.048); REF 132.6 +/- 24.5 (NGT and AGM vs. REF, both P < 0.0002)]. CONCLUSION Abnormalities in glucose metabolism are frequent in young FA patients without prior diagnosis of diabetes, and are associated with marked defects in insulin secretion.

Published
2008

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