Guidelines for evaluating and managing children born with disorders of sexual Development

Pediatric Annals

Volumn 41, Issue 4, 2012, Pages

  Douglas, Ganka ^a   Axelrad, Marni E ^a   Brandt, Mary L ^a   Crabtree, Elizabeth ^a   Dietrich, Jennifer E ^a   French, Shannon ^a   Gunn, Sheila ^a   Karaviti, Lefkothea ^a   Lopez, Monica E ^a   Macias, Charles G ^a   Mccullough, Laurence B ^a   Suresh, Deepa ^a   Austin, Elise ^a   Sutton, V Reid ^a  

^a TEXAS CHILDREN S HOSPITAL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ANDROGEN RECEPTOR; ANDROSTANOLONE; ANDROSTENEDIONE; CHORIONIC GONADOTROPIN; CORTICOTROPIN; CORTODOXONE; ELECTROLYTE; FLUDROCORTISONE; FOLLITROPIN; GLUCOSE; HYDROCORTISONE; LUTEINIZING HORMONE; MINERALOCORTICOID; PRASTERONE; STEROID 5ALPHA REDUCTASE; TESTOSTERONE; THYROTROPIN; THYROXINE;

ADOLESCENT; ANDROGEN INSENSITIVITY SYNDROME; ANXIETY; CANCER RISK; CAREGIVER; CHILD; CLINICAL EXAMINATION; CLINICAL PRACTICE; COMPARATIVE GENOMIC HYBRIDIZATION; CONGENITAL ADRENAL HYPERPLASIA; CONSENSUS DEVELOPMENT; CONTINUING EDUCATION; CRYPTORCHISM; DECISION MAKING; DEPRESSION; ENZYME DEFICIENCY; EVIDENCE BASED PRACTICE; FAMILY HISTORY; FEMALE; FEMINIZATION; FLUORESCENCE IN SITU HYBRIDIZATION; GENE MUTATION; GONADAL DYSGENESIS; GONADECTOMY; GYNECOLOGIST; HEALTH CARE PERSONNEL; HORMONE DETERMINATION; HORMONE SUBSTITUTION; HUMAN; HUMAN GENETICS; HYPOGONADOTROPIC HYPOGONADISM; HYPOPITUITARISM; HYPOSPADIAS; HYPOTHALAMUS HYPOPHYSIS ADRENAL SYSTEM; INFANT; KARYOTYPE; LABORATORY TEST; MALE; MASCULINITY; MEDICAL ETHICS; MEDICAL SPECIALIST; MICROPENIS; NEPHROBLASTOMA; ORCHIDOPEXY; PARENT; PEDIATRIC SURGERY; PEDIATRICIAN; PHENOTYPE; PLASMA RENIN ACTIVITY; PRACTICE GUIDELINE; PSYCHOLOGICAL ASPECT; PSYCHOLOGIST; PUBERTY; REVIEW; SEX DIFFERENTIATION; SEX DIFFERENTIATION DISORDER; SEX TRANSFORMATION; SRY GENE; SURGEON; TREATMENT INDICATION; TURNER SYNDROME; UROLOGIST; VIRILIZATION; Y CHROMOSOME;

CHILD, PRESCHOOL; CONSENSUS; DISORDERS OF SEX DEVELOPMENT; HUMANS; INFANT; TEXAS;

EID: 84860152241     PISSN: 00904481     EISSN: None     Source Type: Journal    
DOI: 10.3928/00904481-20120307-09     Document Type: Review

References (27)

1. Chumpitazi CE, Barrera P, Macias CG. "Diagnostic accuracy and therapeutic reliability in Pediatric Emergency Medicine: The role of evidence-based guidelines." Clin Pediatr Emerg Med; 2011. Available at: www.clinpedemergen-cymed.com/article/S1522-8401(11)00016-4/abstract. Accessed Feb. 28, 2012.
2. Douglas G, Axelrad ME, Brandt ML, et al. Consensus in Guidelines for Evaluation of DSD by the Texas Children's Hospital Multidisciplinary Gender Medicine Team. Int J Pediatr Endocrinol. 2010;2010:919707.
3. Blizzard RM. Intersex issues: a series of continuing conundrums. Pediatrics. 2002;110(3):616-621.
4. Thyen U, Richter-Appelt H, Wiesemann C, Holterhus PM, Hiort O. Deciding on gender in children with intersex conditions: considerations and controversies. Treat Endocrinol. 2005;4(1):1-8.
5. Pescovitz OH, Eugster EA. "Disorders of sexual differentiation." Pediatric Endocrinology: Mechanisms, Manifestations and Management. Philadelphia PA: Lippincott Williams & Wilkins; 2004:243-253.
6. Houk CP, Lee PA. Intersexed states: diagnosis and management. Endocrinol Metab Clin North Am. 2005;34(3):791-810.
7. Diamond M. Pediatric management of ambiguous and traumatized genitalia. J Urol. 1999;162(3Pt2):1021-1028.
8. Diamond DA, Burns JP, Mitchell C, Lamb K, Kartashov AI, Retik AB. Sex assignment for newborns with ambiguous genitalia and exposure to fetal testosterone: attitudes and practices of pediatric urologists. J Pediatr. 2006;148(4):445-449.
9. Brett AS, McCullough LB. When patients request specific interventions: Defining the limits of the physician's obligation. N Engl J Med. 1986;315(21):1347-1351.
10. Hughes IA. Minireview: sex differentiation. Endocrinology. 2001;142(8):3281-3287.
11. Speiser PW, Azziz R, Baskin LS, et al. Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2010;95(9):4133-4160.
12. Axelrad ME, Berg JS, Coker LA, et al. The Gender Medicine Team: "It takes a village." Adv Pediatr. 2009;56:145-164.
13. Sultan C, Paris F, Jeandel C, Lumbroso S, Galifer RB, Picaud JC. Ambiguous genitalia in the newborn: diagnosis, etiology and sex assignment. Endocr Dev. 2004;7:23-38.
14. White PC, Speiser PW. Congenital Adrenal Hyperplasia due to 21-hydroxylase Deficiency. Endocr Rev. 2000;21(3):245-291.
15. Grumbach MM. A window of opportunity: the diagnosis of gonadotropin deficiency in the male infant. J Clin Endocrinol Metab. 2005;90(5):3122-3127.
16. Davenport M, Brain C, Vandenberg C, et al. The use of the hCG stimulation test in the endocrine evaluation of cryptorchidism. Br J Urol. 1995;76(6):790-794.
17. Cai LQ, Zhu YS, Katz MD, et al. 5 alphareductase-2 gene mutations in the Dominican Republic. J Clin Endocrinol Metab. 1996;81(5):1730-1735.
18. Deeb A, Jääskeläinen J, Dattani M, Whitaker HC, Costigan C, Hughes IA. A novel mutation in the human androgen receptor suggests a regulatory role for the hinge region in aminoterminal and carboxy-terminal interactions. J Clin Endocrinol Metab. 2008;93(10):3691-3696.
19. Parisi MA, Ramsdell LA, Burns MW, et al. A gender assessment team: experience with 250 patients over a period of 25 years. Genet Med. 2007;9(6):348-357.
20. Lu XY, Phung MT, Shaw CA, et al. Genomic imbalances in neonates with birth defects: high detection rates by using chromosomal microarray analysis. Pediatrics. 2008;122(6):1310-1318
21. Speiser PW, White PC, Congenital Adrenal Hyperplasia. N Eng J of Med. 2003;8:776-788.
22. Schaeffer TL, Tryggestad JB, Mallappa A, et al. An evidence-based model of multidisciplinary care for patients and families affected by classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Int J Pediatr Endocrinol. 2010;2010:692439.
23. Reiner WG, Gearhart JP. Discordant sexual identity in some genetic males with cloacal exstrophy assigned to female sex at birth. N Engl J Med. 2004;350(4):333-341.
24. American Academy of Pediatrics. Timing of elective surgery on the genitalia of male children with particular reference to the risks, benefits, and psychological effects of surgery and anesthesia. Pediatrics. 1996;97(4):590-594.
25. McCullough LB. A framework for the ethically justified clinical management of intersex conditions. Adv Exp Med Biol. 2002;511:149-165.
26. Wiesemann C, Ude-Koeller S, Sinnecker GH, Thyen U. Ethical principles and recommendations for the medical management of differences of sex development (DSD)/intersex in children and adolescents. Eur J Pediatr. 2010;169(6):671-679.
27. Hampson JG, Money J, Hampson JL. Hermaphrodism: recommendations concerning case management. J Clin Endocrinol Metab. 1956;16(4):547-556.