003 1-399819313305-0000$03.00/0 Vol. 33, No. 5, 1993 PEDIATRIC RESEARCH Printed in U.S.A. Copyright O 1993 International Pediatric Research Foundation, Inc.

Fourth Joint LWPESJESPE Meeting 1993 Final Program

FRIDAY, JUNE 4,1993

MORNING 9. Hormonal Monitoring and Side Effects of 8: 15 Opening ceremony Prenatal Dexamethasone Treatment for 21 - Fairmont Grand Ballroom Hydroxylase Deficiency Congenital Adrenal Melvin M. Grumbach, Chairman, Hyperplasia Organizing Committee SongyaPang Jo Anne Brasel, President, LWPES Department of Pediatrics Ieuan A. Hughes, President, ESPE University of Illinois College of Medicine, Chicago, IL PLENARY SESSION, I Fairmont Grand Ballroom 10. Prenatal Treatment of Congenital Adrenal 8:30 1. Lawson Wilkins Lecture: Hyperplasia Due to 21 -Hydroxylase Mellitus, Type I - Islet Cell Deficiency: European Experience in 223 Destruction and Growth Studied in Pregnancies at Risk Transgenic Animal Models M. G. Forest Nora Sarvetnick I.N.S.E.R.M., Hopital Debrousse, Lyon, Department of Neuropharmacology France Scripps Research Institute, and LaJolla, CA H. G. Dorr Klinik fur Kinder and Jugendliche, 9:30 BREAK University Erlangen-Nurnberg, Erlangen, Germany CONCURRENT SYMPOSIA, I 11. Prenatal Treatment of Congenital Adrenal 10:OO-11:30 A.M. Hyperplasia SESSION A: Prenatal Diagnosis and P. Speiser Treatment of CAH Department of Pediatrics Fairmont Grand Ballroom The New York Hospital 10:OO 7. Molecular Pathology of 21 -Hydroxylase Cornell Medical Center, Deficiency New York, NY Tom Strachan Division of Human Genetics SESSION B: IDDM - Pharmacologic and University of Newcastle upon Tyne, UK Transplant Approaches to Therapy 10:20 8. Prenatal Diagnosis of Congenital Adrenal Mark Hopkins Peacock Court Hyperplasia Due to 21 -Hydroxylase 10:OO 12. Design and Use of Cellular Implants for Deficiency Treatment of Diabetes (IDDM) Yves Morel David Scharp I.N.S.E.R.M. Department of Surgery Hopital Debrousse, Washington University, Lyon, France St. Louis, MO

10:40 Prenatal Treatment Round Table Discussion Chair: Walter L. Miller Discussants: Drs. Dorr, Forest, Morel, Pang, Speiser, and Strachan 10:30 13. Antigen Directed Therapies to Prevent 297. Spontaneous Growth and Final Height in SGA Insulin Dependent Diabetes Infants Noel K. Maclaren J. Karlberg and K. Albertsson-Wikland Department of Pathology and 385. Insulin-Like Growth Factors (IGFs) Stimulate Laboratory Medicine VLA-4 Expression in University of Florida, D~fferentiatingMouse Skeletal Muscle Cells Gainesville, FL S. M. Rosenthal and D. Hsiao 322. Increased Chromosome Fragility in Lymphocytes 11:00 14. Role of Glucose in Diabetic Complications of Short Normal Children Treated with Anthony Cerami Recombinant Human Growth The Picower Institute for Medical Research G. L. Spadoni, B. Tedeschi, M. L. Sanna, Manhasset, NY P. Vemole, D. Caporossi, S. Cianfarani, B. Nicoletti, B. Boscherini 11:30 LUNCHIPOSTERSIEXHIBITS 270. Radioimmunologic Determination of GHBP in Sera of Children with Shorl Stature AFTERNOON J. Kratzsch, W. F. Blum, T. Selisko, and E. Keller POSTER SYMPOSIUM 189. Advantages of the Computer-Aided Image 12:OO - 1:15 P.M. Analysis System for Estimating TW Skeletal Genibnormalities of the Axis Maturity: Increased Reliability and a Continuous Stanford Court India Suite Scale 17 1. Exclusion of the GHRH Gene as a Candidate for J. M. Tanner, R. D. Gibbons, and R. D. Bock Most Cases of Familial Isolated Growth Hormone 359. Low Proconvertin (Factor VII) and Impaired Deficiency Blood Clotting Due to Growth Hormone L. A. Perez-Jurado, J. D. Cogan, J. A. Phillips, 111, Deficiency in the Rat and U. Francke L. S. G. Savendahl, K. G. Engstrom, and 137. Detection of Heterogeneous Growth Hormone K. Grankvist (GH)Gene Splicing by DNA Analysis of Dried Blood Spots From GH Deficient Subjects. SESSION B: NeuroendocrineIPituitary S. Miller-Davis, J. Cogan, J. A. Phillips, 111, Mark Hopkins Peacock Court R. D. G. Milner, A. Al-Ashwal, and N. A. Sakati 154. Characterization of the Molecular Defect in the 340. Spectrum of Growth Mutations -Neurophysin II Gene in a Family and Associated Haplotypes in Laron Syndrome with Autosomal Dominant Neurohypophyseal S. Amselem, F. Dastot, P. Duquesnoy, M-L. Sobrier, S. Vallex, B. Duriez, and D. R. Repaske and J. E. Browning M. Goossens 184. N-Methyl-D-Aspartate (NMDA)Stimulates LHRH 339. Molecular Analysis of Two Families with Laron Release via the NMDA Receptor in an LHRH Syndrome and Positive Growth Hormone Binding Neuronal Cell Line (GTl-I) P. ~ahachoklertwattana,J. Sanchez, P. Duquesnoy, M-L. Sobrier, C. R. Buchanan, M. M. Grumbach, S. L. Kaplan, and B. Duriez, H. G. Maheshwari, M. 0. Savage, S. M. Rosenthal M-C. Postel-Vinnay, M. Norman, A. M. Cotterill, 153. Inhibition of GnRH Secretion by IGF-1 M. A. Preece, M. Goossens, and S. Amselem Degradation into a Sub-product Antagonist at 191. A New Type of Inherited Growth Hormone NMDA Receptors: An Effect Developing After the Deficiency: A Compound Heterozygote of a 6.7 Onset of KB Deletion, Including the GH-l Gene, and Two J. P. Bourguignon, A. Gerard, Base Deletion in the Third Exon of the GH-I Gene M. L. Alvarez Gonzalez, and P. Franchimont Y.Igarashi, T. Kamijo, M. Ogawa, Y. Nishi, 148. PIT-I Gene Expression in the is N. Iwatani, H. Kono, T. Masumura, and J. Koga Modulated by Changes in Circulating Levels of Testosterone 221. Molecular Analysis of a Large Bedouin Kindred J. Argente, S. Gonzalez-Parra, J. A. Chowen, and with IGHD Type IB L.M. Garcia-Segura E. Leiberman, R. Carmi, Y. Limoni, H. Abdul 144. Sexual Dimorphism of Galanin Gene Expression Latif, M.R. Brown, and J.S. Parks in Growth Hormone-Releasing Hormone Neurons of the Rat During Development CONCURRENT ORAL PRESENTATIONS H. A. Delemarre-van de Waal, K. A. Burton, 1:30 - 3:00 p.m. E. B. Kabigting, D. K. Clifton, and R. A. Steiner SESSION A: Growth, Growth Factors, Growth Hormone, I Fairmont Grand Ballroom 11 2:45 150. Neonatal Testosterone Modulates the Number and 17. Therapeutic Response to Recombinant IGF-I Responsivity of Growth Hormone-Releasing in Thirty-Two Patients with Growth Hormone (GHRH)Neurons Hormone Insensitivity J. A. Chowen, S. Gonzalez-Parra, M. 0. Savage L. M. Garcia-Segura, and J. Argente St. Bartholomew's Hospital, London, UK SESSION C: Fetal, Neonatal, Pregnancy Stanford Court Ballroom 18. One Year Treatment with IGF-I of Children 1:30 405. Pregnancy Luctogens in the Rat Conceptus: with Laron Syndrome Circulating Levels, Distribution of Binding, and Expression of Receptor mRNA Children's Medical Center of M. S. Freemark, K. Kirk, K. Pihoker, P. Driscoll, Petah Tikva, Israel and M. Robertson Effects of Prolonged IGF-I Treatment in 1 :45 408. Anti-Mullerian Hormone in Early Human Development Children with Growth Hormone Insensitivity N. Josso, I. Lamarre, and J.Y. Picard Syndrome (GHIS) Louis Underwood 2:00 413. Protein Kinase C Isotype Expression During Division of Pediatric Early Pituitary Development L. Cuttler, M. Axline, and A.P. Fields University of North Carolina, Chapel Hill, NC SESSION C(cont'd): Calcium/Phosphorus, Bone 2: 15 470. Nephrocalcinosis (NC)Due to 4:30 Discussion Hyperparathyroidism in X-Linked Hypophosphatemic Rickets (XLHR)-Results of a SESSION B: Neuroendocrinology of Retrospective Study in 155 Children and Reproduction Adolescents Stanford Court Ballroom K. Mohnike, K. Knye, M. Cagnoli, K. Kruse, 19. Regulation of GnRH Release from GnRH E. Schoenau, and the German Phosphate-Diabetes Neuronal Cell Lines Study Group Richard I. Weiner 2:30 473. VGR-l/BMP-6 Induces Osteogenic Differentiation Reproductive Endocrinology Center in Mesenchymal Cells University of California, S. E. Gitelman, M. Kirk, A. J. Kahn, and San Francisco, CA R. Derynck 20. : A Defect in Neuronal 2:45 467. Ultrasound Velocity in the 0s Calcis, Thumb, and Patella During Childhood as Indicator of Bone Target Recognition Density Andrea Ballabio E. Schoenau, A. Rademacher, K. Klein, and Institute for Molecular Genetics Baylor D. Michalk College of Medicine, Houston, TX CONCURRENT SYMPOSIA, II Spatio-Temporal Patterns of Hormone and 3:OO-4:30 P.M. Hormone Transcription Factor Gene SESSION A: Insulin-Like Growth Factors Expression During the Development and Fairmont Grand Ballroom Mature Function of the Neuroendocrine 3:00 15. In Vivo Metabolic Actions of IGF-l System Jurgen Zapf Lany W.Swanson Department of Medicine Department of Biological Sciences University Hospital, University of Southern California, , Los Angeles, CA

Round Table Discussion: IGF-1 Treatment of SESSION C: Abnormalities of Steroidogenesis Growth Hormone Insensitivity and Metabolism Mark Hopkins Peacock Court 3:30 16. Changes in Body and Bone Composition in 3:OO 22. Molecular Basis of Congenital Adrenal Adolescent Hypelplasia Due to 3beta-Hydroxysteroid Deficient (GHRD)Patients Receiving rhIGF- Dehydrogenase Deficiency I and a GnRH Analog Jacques Simard Jaime Guevara-Aguirre MRC Group in Molecular Endocrinology IEMIR CHUL Research Center, Quito, . . . Quebec, Canada 111 3:30 23. Ilbeta-Hydroxylase and Hypertension 4:30 BREAK P. C. White Cornell University PLENARY SESSION, II Medical College, Fairmont Grand Ballroom New York, NY 5:00 2. PIT-1 and Its Naturally Occurring Genetic Mutations: Answers and Questions about the Steroid Salpha-Reductase Type 2 Deficiency 4:00 24. Molecular Biology of Pituitary Development David W. Russell and Disease Department of Molecular Genetics Holly A. Ingraham University of Texas Southwestern Medical Department of Obstetrics, Gynecology and Center, Reproductive Sciences Dallas, TX University of California, San Francisco, CA

6:00 ADJOURN

SATURDAY, JUNE 5,1993

MORNING 11:00 27. Molecular Genetics of Early-Onset Non- Insulin Dependent (Type 2) Diabetes Mellitus PLENARY SESSION, Ill Fairmont Grand Ballroom Graeme I. Bell Howard Hughes Medical Institute 8:30 3. G in Transmembrane Signaling The University of Chicago, Henry R. Bourne Chicago, IL Departments of Pharmacology and Medicine University of California, San Francisco, CA SESSION B: FETAL AND PLACENTAL ENDOCRINOLOGY 9:30 BREAK Stanford Court Ballroom 10:OO 28. The Role of Insulin-Like Growth Factors in CONCURRENT SYMPOSIA, Ill Normal and Abnormal Fetal Growth 10:OO-11:30 A.M. Peter D. Gluckman SESSION A: Transmembrane Signaling Research Centre for Developmental Diseases Medicine and Biology University of Mark Hopkins Peacock Court Auckland, Auckland, 10:OO 25. The McCune Albright Syndrome: A New Zealand Geneticallv Determined Signal- Transduction Disorder 10:30 29. Placental Growth Hormone and Related Allen M. Spiegel Proteins NIDDWNIH, Nancy E. Cooke Bethesda, MD Departments of Medicine and Genetics The University of Pennsylvania, 10:30 26. Growth Hormone Insensitivity Syndrome Philadelphia, PA Uta Francke Howard Hughes Medical Institute and 11:00 30. Placental Epidermal Growth Factor Departments of Genetics and Pediatrics, Receptors: From Physiology to Pathology Stanford University, ~ Daniele Evain-Brion Stanford, CA Physiopathology of Development Laboratory C.N.R.S.-E.N.S., Paris, France SESSION C: IDDM - Etiology, Genetics, and CONCURRENT ORAL PRESENTATIONS Environmental Factors 1 :30 - 3:00 p.m. Fairmont Grand Ballroom SESSION A: Growth, Growth Factors, Growth 10:OO 3 1. Immunogenetics of Type 1 Diabetes Mellitus Hormone, I1 Massimo Trucco Fairmont Grand Ballroom Department of Pediatrics 1:30 203. A Role for IGF-1 and Its Binding Proteins in University of Pittsburgh, Neuronal Rescue Following Asphyxia1 Injury Pittsburgh, PA P. D. Gluckman, J. Guan, E. Bilharz, E. Sirimanne, 0. Miller, and C. Williams 10:30 32. Population Screening for Risk of IDDM Edwin A. M. Gale 1:45 294. Interleukin-1 Differentially Stimulates Insulin- Department of Diabetes and Metabolism Like Growth Factor Binding Protein 3 Release St. Bartholomew's Hospital, from Human Articular Chondrocytes London, UK R. C. Olney, M. Mohtai, D. M. Wilson, and R. L. Smith 11:OO 33. Incidence Variation and Etiology of Type 1 Diabetes 2:00 41 1. Lack of 150kDa Ternary Complex Formation in Claire Levy-Marchal Serum From the Human Fetus In Utero: Hopital Robert Debre, Increased Bioavailability of Circulating IGF-I Paris, France P. Bang, R. G. Rosenfeld, M. Stangenberg, and M. Westgren 2: 15 235. Is Immunoreactive IGFBP-3 in the Urine an Indicator of Growth Hormone Deficiency (GHD)? AFTERNOON S. E. Gargosky, K. F. Wilson, H. Anhalt, POSTER SYMPOSIUM M. MacGillivray, and R. G. Rosenfeld 12:OO - 1:15 P.M. 2:30 292. Insulin-Like Growth Factor Binding Proteins from Diabetes Mellitus/Fuel Metabolism Prima~yCultures of Rat Ovarian Theca- Mark Hopkins Room of the Dons Interstitial (ROTI) Cells: Regulation by LH and 404. Glucose Entry into the Fetal Hepatocyte (FH) IGF-I Requires Non-Insulin Dependent GLUT-I and J. F. Cara and J. Fan GLUT-2 0.Zheng, L. Levitsky, D. Rhoads, and K. Mink 2:45 237. Characterization and Regulation of IGFBP-4 in Cultured Mouse Leydig Cells (TM-3) 445. Elevated Anti-Bovine Serum Albumin (BSA) T. Hasegawa, P. Cohen, P. J. Fielder, Antibodies Predict Development of Type I Y. Hasegawa, and R. G. Rosenfeld Diabetes in Siblings of Diabetic Children J. Karjalainen, M. Knip, H.-K. Akerblom, H.-M. SESSION B: Steroids, Adrenals, Gonads, I Dosch, and the Study Group of Childhood Diabetes in Finland Mark Hopkins Peacock Court 1:30 72. Identification of Gene and 43 1. Anti-Retrovirus Antibodies in Insulin-Dependent Future Therapy Diabetic Children at Diagnosis and During 1-2.6 P. Aubourg, J. Mosser, A. M. Douar, C. Sarde, Years Follow-up J. L. Chaussain, and J. L. Mandel R. Lorini. L. Cortona. A. Scaramuzza. G. Achilli. E. Cattaneo, P. Ferrante, and F. Severi ' 1 1:45 121. Abundant Adrenal-Specific Transcription of the P450c21A Pseudogene-Implications for Beta-Cell Destruction in Insulin-Dependent 424. Congenital Adrenal Hyperplasia Diabetes Mellitus -A Stepwise Series of Events J. D. Bristow, S. E. Gitelman, M. K. Tee, and B. G. Cuartero, D. Hougaard, F. Pociot, W. L. Miller J. Argente, L. I. Larsson, and J. Nerup Recipients of the HENNING ANDERSEN PRIZE awarded by the ESPE Council for the best-rated 418. Nicotinamide and Interleukin-1 (IL-I)Effects on abstract Human Pancreatic Islet Function 2:00 124. Germ Cell Localization of Testicular Growth U. Zumsteg, M. Brendel, R. Alejandro, and Hormone Releasing Hormone (GHRH) J. Nerup P. R. Breyer, C. Srivastava, M. Peredo, 417. Identification qf a Marker to Enrich Populations I J. Rothrock, M. Collard, and 0. H. Pescovitz of Endocrine Cell Precursors from the Human 2: 15 66. A Point Mutation of the ACTH Receptor in Fetal Pancreas Familial Deficiency A. Hayek, G.M. Beattie, M.I. Mally, A. J. L. Clark, A. Weber, A. Grossman, and T. Otonkoski, J.S. O'Brien, and F. Levine M. 0. Savage 120. Homozygous Deletion of Amino Acids 487-489 in 4:00 36. Growth Hormone (GH)Deficiency in P450cl7 Causes Severe 17alpha-Hydroxylase Adulthood: Short and Long Term Effects of (I 70H)Deficiency GH Substitution C. E. Fardella, L. Zhang, P. Mahachoklertwattana, Jens Otto Lunde Jorgensen D. Lin, and W. L. Miller Medical Department, 126. Activating Mutation in the Stirnulatory Division of Endocrinology and Diabetes Gene in an Infant with Adrenocorticonodular Aarhus Kommunehospital, Dysplasia Aarhus, Denmark B.A. Boston, M. Bliziotes, S. Mandel, and S. LaFranchi SESSION B: Neurosteroids Mark Hopkins Peacock Court SESSION C: Thyroid Neurosteroid Biosynthesis: for Stanford Court Ballroom 3:00 37. Adrenal Steroidogenic Enzymes are 536. Antibodies Blocking Thyroid Growth and Function in Patients with Permanent Congenital Expressed Regionally in the Rat Brain Hypothyroidism-In Vitro and In Vivo Study Synthia H. Mellon A. Gruters, P. M. Schumm-Draeger, U. Bogner, Department of Obstetrics, Gynecology, and K. P. Liesenkotter, and H. Helge Reproductive Sciences 542. Thyroid Autoimmunity is Associated with University of California, Cognitive Impairment in Turner Syndrome San Francisco, CA J. M. Watkins, T. E. Elliott, E. K. Neely, 3:30 38. Neuroactive Steroids R. L. Hintz, R. G. Rosenfeld, and B. Lippe Steven M. Paul 525. Long-Term Neurodevelopmental Correlates of Section on Molecular Pharmacology, Treatment Adequacy in Screened Hypothyroid Clinical Neuroscience Branch Children National Institute of Mental Health, J. F. Rovet, R. M. Ehrlich, and E. Donner Bethesda, MD 543. Pattern of lntrathyroid Subsets of Lymphocytes and Its Correlation with Serum Thyroid 4:00 39. GABAa Receptors and Actions of Antibodies (Ab) in Juvenile Autoimmune Thyroid Neurosteroids Disease (ATD) Maria Dorota Majewska S. Iorcansky, V.Herzovich, J. Rossi, and Medications Development Division J. Goldberg National Institute on Drug Abuse, NIH, 552. Thyroid Autoimmunity in Childrenfrom an Area Rockville, MD Contaminated After the Chernobyl Accident I. I. Dedov, V. A. Peterkova, A. N. Tiul'pakov, SESSION C: Bone and Mineral Metabolism T. V. Semicheva, V. I. Kandror, V. P. Fedotov, Stanford Court Ballroom and V. F. Stepanenko 3:00 40. The Role of TGF-beta Family Members in 529. Normal Serum T3 Postnatal Surge and Increased Mesenchymal Dzfferentiation Reverse T3 Levels in Selenium-Deficient Rat Pups Rik Derynck J. P. Chanoine, S. Alex, S. Stone, S. L. Fang, Departments of Growth and Development, J. L. Leonard, and L. E. Braveman Anatomy, and Pediatrics University of California, CONCURRENT SYMPOSIA, IV San Francisco, CA 3:OO-4:30 P.M. 3:30 41. Effects of 17J-Estradiol on Human Cartilage SESSION A: Growth Hormone Fairmont Grand Ballroom Cell Proliferation and Dzfferentiation M. T. Comol 34. Molecular Basis of Growth Hormone Action I.N.S.E.R.M. Christin Carter-Su Hopital Enfants Malades, Department of Physiology University of Michigan Medical School, Paris, France Ann Arbor, MI 4:00 42. Bone Mass Growth During Normal Pubertal Development 35. Structure and Functional Analysis of the G. E. Theintz Human Growth Hormone Receptor Division of Biology of Growth and William I. Wood Department of Molecular Biology Reproduction Genentech, Inc., Department of Pediatrics University Hospital, South San Francisco, CA Geneva, Switzerland vi 4:30 BREAK

PLENARY SESSION, IV Fairmont Grand Ballroom 5:00 4. The Genetics and Biochemistry of Sex Determination Peter N. Goodfellow Department of Genetics University of Cambridge, Cambridge, UK

6:00 ADJOURN

SUNDAY, JUNE 6,1993

MORNING PLENARY SESSION, V Masonic Auditorium AFTERNOON 8:30 5. Difisible Factors and Cell Differentiation J. B. Gurdon POSTER SYMPOSIUM Wellcome CRC Institute of Cancer and 12:OO - 1:15 p.m. Developmental Biology Androgen Action Cambridge, UK Mark Hopkins Room of the Dons 123. Molecular-Phenotypic Correlations in the 9:30 BREAK Androgen Insensitivity Syndrome SYMPOSIUM, V C. A. Quigley, A. Debellis, M. K. El-Awady, 10:OO-11:30 A.M. E. M. Wilson, and F. S. French Final and Near Height Symposium 96. Receptor Accessory Factor (R4F)Enhances Masonic Auditorium Specific DNA Binding of Androgen and 10:OO 43. Final Height Attainment in Normal Children Glucocorticoid Receptors with Treated with Growth S. R. Kupfer, K. B. Marschke, E. M. Wilson, and Hormone F. S. French Harvey Guyda 60. Clinical, Biochemical and Genetic Studies in Division of Endocrinology and Metabolism Androgen Insensitivity Syndrome: A Two Year Montreal Children's Hospital, Study Montreal, Canada M. N. Patterson, H. R. Davies, J. A. Batch, D. M. Williams, B. D. Brown, and I. A. Hughes 10:30 44-45.Height Development in Turner Syndrome 95. Improved Detection of Point Mutations in the (TS): Results of an International Survey Human Androgen Receptor Gene by Denaturing Conducted by ESPE/LWPES Gradient Gel Electrophoresis of DNA Michael B. Ranke Heteroduplexes Under Stringent Denaturing Section for Paediatric Endocrinology Conditions University Children's Hospital, P. Ghirri and T. R. Brown Tuebingen, Germany 114. Androgen Receptor (AR)Gene Mutations in 6 11:OO 46. Final and Near Height of Children with True Families with Androgen Insensitivity Syndrome J. M. Lobaccaro, S. Lumbroso, Ch. Belon, Precocious Puberty Treated with GnRH J. L. Chaussain, J. E. Toublanc, B. Leheup, and (LRF)Agonists Ch. Sultan Robert P. Kelch Department of Pediatrics 73. High Frequency of Carcinoma-in-Situ and Gonadoblastorna in Children and Adolescents University of Michigan Medical Center, with Androgen Insensitivity (AI) and Gonadal Ann Arbor, MI Dysgenesis (GD) J. Muller and N. E. Skakkebaek vii 82. Partial Androgen Insensitivity Due to VAL - 520. Evidence that Neuropeptide Y (NPY) Could LEU Substitution in Codon 866 of the Androgen Represent A Neuroendocrine Inhibitor of Sexual Receptor Can Be Treated with High Doses of Maturation in Unfavorable Metabolic Conditions Testosterone in the Rat G.H.G. Sinnecker, 0. Hiort, C. Brack, E. Nitsche, N. M. Gruaz, D. D. Pierroz, F. Rohner- and K. Kruse Jeanrenaud, P. C. Sizonenko, and M. L. Aubert 116. XX Males and Ambiguous Genitalia: A Link 501. Dzfferences in Inhibin and Gonadotropin Between SRY and Androgen Receptor (AR) Gene? Secretion Between Prepubertal and Late Pubertal J.M. Lobaccaro, B. Leheup, N. Boucekkine, S. Females Lumbroso, J.E. Toublanc, M. Fellous, and Ch. P. K. Manasco, S. M. Muly, D. C. Godwin, and Sultan M. D. Culler 59. A New Point Mutation of SRY Gene in Two Sisters CONCURRENT ORAL PRESENTATIONS With 46 XY Gonadal Dysgenesis 1:30 - 3:00 p.m. T. Tajima, N. Shinohara, J. Nakae, and K. Fujieda SESSION A: Growth, Growth Factors, and Growth Hormone, I11 SESSION C: Diabetes MellituslFuel Metabolism Masonic Auditorium Stanford Court Ballroom 328. Both the Low and the High AfJinity Nerve Growth 400. from Lactate in Fasting Infants Factor Receptors (NGF-R)Are Expressed in N. Nurjhan, F. Rocchiccioli, J. Zeller, and P.F. Pancreatic Beta Cell Lines Bougneres A. Tazi, R. Scharfmann, M. Asfari, M. Polak, and 428. Time Course of Increased Lipid and Glucose P. Czemichow Oxidation During the Early Phase of Childhood 253. Gene Knockout Mice Demonstrate that IGF-1 Is Essential for Normal Muscle Development C. Le Stunff and P.F. Bougneres L. Powell-Braxton, P. Hollingshead, N. Gillett, S. 444. Growth, Development and Carbohydrate Pitts-Meek, and T.A. Stewart Tolerance of Formerly Small for Gestational Age 381. GH Preincubation Fails to Induce Resistance to Infants With and Without Glucose Infusion the Mitogenic Action of Insulin in a Pygmy T-cell Therapy Line E. Keller, E. Mehlhorn, E. Patz, and H. Willgerodt M.E. Geffner, N. Bersch, R.C. Bailey, and D.W. 440. (SRIH)Increases Glomerular PG E2 Golde Production in Diabetic and Normal Rats 308. Human Growth Hormone Increases the D. A. Nickels and M. Poth Intracellular Free Calcium in Cultured Human 434. Differential Effects of Hypo- & Hyperglycemia on IM-9 Lymphocytes Cognitive Function and Pancreatic Polypeptide M. M. Ilondo, P. Bouchelouche, and P. DeMeyts (PP)Responses 264. Gonadal Steroids Are Capable of Acting Within S. Gschwend, C. Ryan, T. Williams, S. Arslanian, the to Modulate Somatostatin (SS) J. Atchison, and D. Becker Gene Expression in Pubertal Aged Rats 429. The Effects of rhIGF-1 on GH, IGF-BP3 and IGF- P. M. Martha, Jr., J. A. Brewer, and E. 0. Reiter BPI Concentrations and Insulin Sensitivity in 200. The Effects of Zinc on Lactogen Stimulation of Adolescents with Insulin Dependent Diabetes Nb2 Cells Using an Eluted Stain Assay (ESTA) Mellitus (IDDM) M. T. Dattani, T. Weir, P.C. Hindmarsh, T. D. Cheetham, J. M. P. Holly, A. M. Taylor, C. G. D. Brook, and N. J. Marshall S. C. Cwyfan-Jones, J. Jones, D. Hanis, and D. B. Dunger SESSION B: Steroids, Adrenals, Gonads, I1 Mark Hopkins Peacock Court CONCURRENT SYMPOSIA,VI 56. Mutational Spectrum of the Steroid 21- 3:OO-4:30P.M. Hydroxylase Gene SESSION A: Pituitary and Hypothalamic A. Wedell, E. M. Ritzen, and H. Luthman Tumors 122. Quantitation of Cortisol (F)Production and Masonic Auditorium Elimination Rates in Premature and Term 47. Pituitary Adenomas in Childhood Neonates: Evidence for Pulsatile Secretion Charles B. Wilson D. L. Metzger, N. M. Wright, A. D. Rogol, Department of Neurosurgery J. D. Veldhuis, and J. R. Kenigan University of California, 112. The Adrenal Autoantigen in APSI is the Side- San Francisco. CA Chain Cleavage Enzyme 0.Winqvist, J. Gustafsson, F.A. Karlsson, and 0. Kampe 3:30 48. Hypothalamic Surgery and Chemotherapy SESSION C: New Genetics and Endocrinology Michael S. B. Edwards Mark Hopkins Peacock Court Department of Neurosurgery 3:00 53. Non-Traditional Forms of Inheritance That Division of Pediatric Neurosurgery Are Relevant for Pediatric Endocrinology University of California, Judith G. Hall San Francisco, CA Departments of Pediatrics and Medical Genetics 4:00 49. Endocrine Outcome University of British Columbia, S.M. Shalet Vancouver, BC, Canada Department of Endocrinology Christie Hospital, 3:30 54. X-Inactivation Manchester. UK Larry J. Shapiro Department of Pediatrics SESSION B: Resistance Syndromes - Nuclear University of California, Receptors San Francisco, CA Stanford Court Ballroom 4:00 55. Genetically Engineered Mice to Study 3:00 50. Nuclear Hormone Receptors: Regulators of Endocrine Disorders Gene Expression Edward M. Rubin A. 0. Brinkmann Lawrence Berkeley Laboratory Department of Endocrinology and University of California, Reproduction Berkeley, CA Erasmus University, Rotterdam, The Netherlands 4:30 BREAK 3:30 5 1. Mutations in the Androgen Receptor Gene Causing Androgen Resistance PLENARY SESSION, VI Michael J. McPhaul Masonic Auditorium Department of Internal Medicine , 5:00 6. Andrea Prader Lecture: TBA I University of Texas 6:00 Closing Southwestern Medical Center 1993 Organizing Committee and E. Martin Dallas, TX Ritzen, Chair, 1997 Committee 4:00 52. Mineralocorticoid Receptors John W. Funder 6:15 ADJOURN Baker Medical Research Institute, Melbourne, Australia