Benjamin L. Shneider, MD, Morton B. Brown, PhD, Barbara Haber, MD, Peter F. Whitington, MD, Kathleen Schwarz, MD, Robert Squires, MD, Jorge Bezerra, MD, Ross Shepherd, MD, FRACP, Philip Rosenthal, MD, Jay H. Hoofnagle, MD, NIDDK, and Ronald J. Sokol, MD, for the Biliary Atresia Research Consortium, New York, New York, Ann Arbor, Michigan, Philadelphia, Pennsylvania, Chicago, Illinois, Baltimore, Maryland, Pittsburgh, Pennsylvania, Cincinnati, Ohio, St. Louis, Missouri, San Francisco, California, Bethesda, Maryland, and Denver, Colorado

HIGH PREVALENCE OF RENAL DYSFUNCTION IN LONG-TERM SURVIVORS AFTER PEDIATRIC LIVER TRANSPLANTATION 475

Kathleen M. Campbell, MD, Nada Yazigi, MD, Frederick C. Ryckman, MD, Maria Alonso, MD, Greg Tiao, MD, William F. Balistreri, MD, Harry Atherton, MS, and John C. Bucuvalas, MD, Cincinnati, Ohio

MIXING RAPID-ACTING INSULIN ANALOGUES WITH INSULIN GLARGINE IN CHILDREN WITH TYPE 1 DIABETES MELLITUS 481

Rosanna Fiallo-Scharer, MD, Brian Horner, MS, Kim McFann, PhD, Philippe Walravens, MD, and H. Peter Chase, MD, Denver, Colorado

50 YEARS AGO IN THE JOURNAL OF PEDIATRICS— THE OCCURRENCE OF CONVULSIONS IN CHILDREN WITH SHIGELLA GASTROENTERITIS 484

Sarah S. Long, MD, Philadelphia, Pennsylvania

THERAPEUTIC EFFICACY AND ADVERSE EFFECTS OF ADRENOCORTICOTROPIC HORMONE THERAPY IN WEST SYNDROME: DIFFERENCES IN DOSAGE OF ADRENOCORTICOTROPIC HORMONE, ONSET OF AGE, AND CAUSE 485

Shin-ichiro Hamano, MD, PhD, Shintaro Yamashita, MD, Manabu Tanaka, MD, Satoshi Yoshinari, MD, Motoyuki Minamitani, MD, PhD, and Yoshikatsu Eto, MD, PhD, Saitama, Japan

SINGLE DOSE OF ANTI-D IMMUNE GLOBULIN AT 75μG/KG IS AS EFFECTIVE AS INTRAVENOUS IMMUNE GLOBULIN AT RAPIDLY RAISING THE PLATELET COUNT IN NEWLY DIAGNOSED IMMUNE THROMBOCYTOPENIC PURPURA IN CHILDREN 489

Michael D. Tarantino, MD, Guy Young, MD, Salvatore J. Bertolone, MD, Karen A. Kalinyak, MD, Frank E. Shafer, MD, Roshni Kulkarni, MD, Lisa C. Weber, Mary L. Davis, Henry Lynn, and Diane J. Nugent, MD, for the Acute ITP Study Group, Peoria, Illinois, Orange, California, Louisville, Kentucky, Cincinnati, Ohio, Philadelphia, Pennsylvania, East Lansing, Michigan, and Hong Kong

PLANT STANOLS DO NOT RESTORE ENDOTHELIAL FUNCTION IN PRE-PUBERTAL CHILDREN WITH FAMILIAL HYPERCHOLESTEROLEMIA DESPITE REDUCTION OF LOW-DENSITY LIPOPROTEIN CHOLESTEROL LEVELS 495

Lily Jakulj, MSc, Maud N. Vissers, PhD, Jessica Rodenburg, MD, PhD, Albert Wiegman, MD, PhD, Mieke D. Trip, MD, PhD, and John J.P. Kastelein, MD, PhD, Amsterdam, the Netherlands

50 YEARS AGO IN THE JOURNAL OF PEDIATRICS—PHYSIOLOGIC CONTROL OF RED CELL PRODUCTION 500

George R. Buchanan, MD, Dallas, Texas

A RANDOMIZED CLINICAL TRIAL OF DIETARY CALCIUM TO IMPROVE BONE ACCRETION IN CHILDREN WITH JUVENILE RHEUMATOID ARTHRITIS 501

Lori J. Stark, PhD, Ann McGrath Davis, PhD, David M. Janicke, PhD, Laura M. Mackner, PhD, Kevin A. Hommel, PhD, Judy A. Bean, PhD, Daniel Lovell, MD, MPH, James E. Heubi, MD, and Heidi J. Kalkwarf, PhD, Cincinnati, Ohio, Kansas, Florida, Columbus, Ohio, and Philadelphia, Pennsylvania

QUALITY-OF-LIFE IN CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS MANAGED IN BOTH REGIONAL OUTREACH AND CYSTIC FIBROSIS CENTER SETTINGS IN QUEENSLAND 508

Clare Thomas, MBBS, Penny Mitchell, BN, Peter O’Rourke, BSc, BA, PhD, GCEd, and Claire Wainwright, MBBS, MD, Brisbane, Queensland, Australia

VARIABILITY IN ASTHMA SEVERITY IN PEDIATRIC SUBJECTS WITH ASTHMA PREVIOUSLY RECEIVING SHORT-ACTING β₂-AGONISTS 517

Bradley E. Chipps, MD, Joseph D. Spahn, MD, Christine A. Sorkness, PharmD, Leslie Baitinger, BS, Laura B. Sutton, PharmD, Amanda H. Emmett, MS, and Paul M. Dorinsky, MD, Sacramento, California, Denver, Colorado, Madison, Wisconsin, and Research Triangle Park, North Carolina

CHILDHOOD PREGNANCY (10-14 YEARS OLD) AND RISK OF STILLBIRTH IN SINGLETONS AND TWINS 522

Hamisu M. Salihu, MD, PhD, Puza P. Sharma, MD, MPH, Olaniyi J. Ekundayo, MD, MPH, Sibylle Kristensen, MPH, Abiodun P. Badewa, MD, PhD, Russell S. Kirby, PhD, and Greg R. Alexander, ScD, Birmingham, and Montgomery, Alabama

RISKY BEHAVIORS AMONG MOTORCYCLING ADOLESCENTS IN ITALY 527

Claudia Pileggi, MD, Aida Bianco, MD, Carmelo G.A. Nobile, MD, and Italo F. Angelillo, DDS, MPH, Catanzaro, and Naples, Italy

ENZYME REPLACEMENT THERAPY FOR MUCOPOLYSACCHARIDOSIS VI: A PHASE 3, RANDOMIZED, DOUBLE-BLIND, PLACEBO-CONTROLLED, MULTINATIONAL STUDY OF RECOMBINANT HUMAN N-ACETYLGALACTOSAMINE 4-SULFATASE (RECOMBINANT HUMAN ARYLSULFATASE B OR RHASB) AND FOLLOW-ON, OPEN-LABEL EXTENSION STUDY 533

Paul Harmatz, MD, Roberto Giugliani, MD, PhD, Ida Schwartz, MD, Nathalie Guffon, MD, Elisa Leão Teles, MD, M. Clara Sá Miranda, PhD, J. Edmond Wraith, MD, Michael Beck, MD, Laila Arash, MD, Maurizio Scarpa, MD, Zi-Fan Yu, ScD, Janet Wittes, PhD, Kenneth I. Berger, MD, Mary S. Newman, MS, Ann M. Lowe, MD, Emil Kakkis, MD, PhD, and Stuart J. Swiedler, MD, PhD, for the MPS VI Phase 3 Study Group, Oakland, California, Porto Alegre, Brazil, Lyon, France, Porto, Portugal, Manchester, England, Mainz, Germany, Padova, Italy, Washington, DC, New York, New York, and Novato, California

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Grand rounds

MALIGNANT HYPERTENSION, POLYCYTHEMIA, AND PARAGANGLIOMAS 540

Janis M. Dionne, MD, John K. Wu, MD, Manraj Heran, MD, James J. Murphy, MD, Gareth Jevon, MD, and Colin T. White, MD, Vancouver, British Columbia, Canada

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Clinical and laboratory observations

DELAYED SEPARATION OF THE UMBILICAL CORD IN TWO SIBLINGS WITH INTERLEUKIN-1 RECEPTOR–ASSOCIATED KINASE 4 DEFICIENCY: RAPID SCREENING BY FLOW CYTOMETER 546

Hidetoshi Takada, MD, PhD, Hideto Yoshikawa, MD, PhD, Masue Imaizumi, MD, PhD, Taro Kitamura, MD, PhD, Junji Takeyama, MD, PhD, Satoru Kumaki, MD, PhD, Akihiko Nomura, MD, PhD, and Toshiro Hara MD, PhD, Kyushu, Miyagi, and Tohoku, Japan

AUTOSOMAL RECESSIVE INTERLEUKIN-1 RECEPTOR-ASSOCIATED KINASE 4 DEFICIENCY IN FOURTH-DEGREE RELATIVES 549

María Cardenes, BSc, Horst von Bernuth, MD, Ayoze García-Saavedra, BSc, Esther Santiago, STch, Anne Puel, PhD, Cheng-Lung Ku, MSc, Jean-François Emile, PhD, Capucine Picard, MD, Jean-Laurent Casanova, MD, PhD, Elena Colino, MD, Ana Bordes, PhD, Antonio Garfia, MD, PhD, and Carlos Rodríguez-Gallego, PhD, Santa Cruz de Tenerife, Las Palmas de Gran Canaria, and Seville, Spain, Paris, and Garches, France

NEONATAL HYPOGLYCEMIA AND OCCIPITAL CEREBRAL INJURY 552

Peter M. Filan, MBBChBAO, Terrie E. Inder, MD, MBChB, Fergus J. Cameron, MD, BMedSci, MBBS, Michael J. Kean, Dip App Sci, and Rod W. Hunt, BMBS, MMed, Melbourne, Victoria, Australia

VITAMIN E STATUS IN CHILDREN WITH CYSTIC FIBROSIS AND PANCREATIC INSUFFICIENCY 556

Shirley H. Huang, MD, Joan I. Schall, PhD, Babette S. Zemel, PhD, and Virginia A. Stallings, MD, Philadelphia, Pennsylvania

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Commentary

REASSURANCE CAN HURT: PARENTAL BEHAVIOR AND PAINFUL MEDICAL PROCEDURES 560

C. Meghan McMurtry, BA, Patrick J. McGrath, PhD, and Christine T. Chambers, PhD, Halifax, Nova Scotia, Canada

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Insights

NONCOMPACTION OF THE VENTRICULAR MYOCARDIUM 562

Christian Lilje, MD, Vit Rázek, MD, Hansjörg Schäfer, MD, PhD, and Jochen Weil, MD, PhD, Hamburg, Germany

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Letters

NATURAL KILLER CELL DEFICIENCIES AND SEVERE VARICELLA INFECTION 563

Jacques Zimmer, MD, PhD, François Hentges, MD, and Emmanuel Andrès, MD, Luxembourg and Strasbourg, France Luigi D. Notarangelo, MD, and Evelina Mazzolari, MD, Brescia, Italy 563

REPLY 564

Amos Etzioni, MD, Technion, Haifa, Israel

OXYGEN AND OXYGENATION IN THE DELIVERY ROOM 564

Augusto Sola, MD, and Richard Deulofeut, MD, MPH, Atlanta, Georgia

CLONIDINE EXPOSURES, NOT TOXICITY 565

Melissa Langhan, MD, and GarMing Chan, MD, New York and Manhasset, NY

VALIDITY OF HOMA-IR AS INDEX OF INSULIN RESISTANCE IN OBESITY 565

Angelika Mohn, MD, Maria Marcovecchio, MD, and Francesco Chiarelli, MD, Chieti, Italy

REPLY 566

Prabhakaran Balagopal, Donald George, Norman Patton, Hossein Yarandi, William L. Roberts, and Samuel Gidding, Jacksonville and Gainesville, Florida, Salt Lake City, Utah, and Wilmington, Delaware

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