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DOI: 10.1055/s-2000-8139
© Johann Ambrosius Barth
Anorexia nervosa in congenital adrenal hyperplasia: long-term follow-up of 4 cases [*]
Publikationsverlauf
Submitted December 2, 1999
Accepted in revised form June 15, 2000
Publikationsdatum:
31. Dezember 2000 (online)
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Summary:
Studies which evaluate the psychosocial development and integration of adult female congenital adrenal hyperplasia (CAH) patients are rare but show that patients with the salt wasting form are significantly more virilised and more frequently single and childless. Major complaints are irregular menstruation, hirsutism, acne, obesity, deep voice, and cushingoid features. Surprisingly, a higher prevalence of psychosomatic disorders has not yet been described. Since anorexia nervosa (AN) has not yet been described in patients with CAH, we here report 4 cases of female CAH patients who developed AN during adolescence. Diagnosis of CAH was made between the age of 10 days and 3 years. Three patients suffer from the simple-virilising form of CAH, one patient has a mild salt wasting CAH. Genital malformation varied from Prader stage II to IV. All 4 patients were compound heterozygotes for mutations/deletions of the CYP21B gene. Control of substitution therapy consisting of hydrocortisone and fluorocortisone was good. AN developed at ages 12, 13, 17, and 21 years (ICD 10 criteria for AN are BMI below 17.5 kg/m2, deliberate weight loss, body image disturbance, and primary or secondary amenorrhoea). Diagnosis of AN was established by psychiatrists and/or psychologists. All four patients showed an impressive and deliberate weight loss between 13 and 20 kg within 6 months, had primary or secondary amenorrhoea, and presented with BMI between 13 and 17.9 kg/m2. All patients received psychological treatment and recovered. However, one patient had a severe relapse of AN. Two patients are now married and one has a healthy son. These cases demonstrate that the diagnosis of CAH is compatible with the development of AN and illustrate the importance of providing treatment for CAH patients that encompasses not only medical but also psychological and social care.
Key words:
Congenital adrenal hyperplasia - 21-hydroxylase deficiency - anorexia nervosa - masculinisation - body mass index
1 * Presented in part at the 37th Annual Meeting of the European Society for Paediatric Endocrinology (ESPE), Florence, Sept. 24-27, 1998
References
- 1 Anderson A E, Mickalide A E. Anorexia nervosa in the male. Psychosomatics. 24 1066-1074 1983;
- 2 Atkins D M, Silber T J. Clinical spectrum of anorexia nervosa in children. J Dev Behav Pediatr. 14 211-216 1993;
- 3 Attie I, Brooks-Gunn J. Development of eating problems in adolescent girls: a longitudinal study. Dev Psychol. 25 70-79 1989;
- 4 Cameron F J, Kaymakci B, Byrt E A, Ebeling P R, Warne G L, Wark J D. Bone mineral density and body composition in congenital adrenal hyperplasia. J Clin Endocrinol Metab. 80 2238-2243 1995;
- 5 Dittmann R W, Kappes M H, Kappes M E, Börger D, Meyer-Bahlburg H FL, Stegner H, Willig R H, Wallis H. Congenital adrenal hyperplasia II: Gender-related behaviour and attitudes in female salt wasting and simple-virilising patients. Psychoneuroendocrinology. 15 421-434 1990a;
- 6 Dittmann R W, Kappes M H, Kappes M E, Börger D, Stegner H, Willig R H, Wallis H. Congenital adrenal hyperplasia I: Gender-related behaviour and attitudes in female patients and sisters. Psychoneuroendocrinology. 15 401-420 1990b;
- 7 Ehrhardt A A, Meyer-Bahlburg H FL. Effects of prenatal sex hormones on gender-related behaviour. Science. 211 1312-1328 1981;
- 8 Feldmann W, Feldmann E, Goodman J. Culture versus biology: children's attitudes toward thinness and fatness. Pediatrics. 81 190-194 1988;
- 9 Haller E. Eating disorders - A review and update. West J Med. 157 658-662 1992;
- 10 Hartmann D. Anorexia nervosa - diagnosis, aetiology, and treatment. Postgrad Med J. 71 712-716 1995;
- 11 Holland A J, Hall A, Murray R, Russell G FM, Crisp A H. Anorexia nervosa: a study of 34 twin pairs and one set of triplets. Br J Psychiatry. 145 414-419 1984;
- 12 Hsu L KG. Epidemiology of the eating disorders. Psychiatr Clin North Am. 19 681-700 1996;
- 13 Jimerson D C, Lesem M D, Hegg AP, Brewerton T D. Serotonin in human eating disorders. Ann N Y Acad Sci. 600 532-544 1990;
- 14 Kuhnle U, Bullinger M, Schwarz H P. The quality of life in adult female patients with congenital adrenal hyperplasia: a comprehensive study of the impact of genital malformations and chronic disease on female patients life. Eur J Pediatr. 154 708-716 1995;
- 15 Langer M, Grünberger W, Ringler M. Vaginal agenesis and congenital adrenal hyperplasia. Acta Obstet Gynecol Scand. 69 343-349 1990;
- 16 Mahowald M B. To be or not to be a woman: anorexia nervosa, normative gender roles, and feminism. J Med Philos. 17 233-251 1992;
- 17 Maloney M J, McGuire J, Daniels S R, Speckler B. Dieting behaviour and eating attitudes in children. Paediatrics. 84 482-489 1989;
- 18 Meyer-Bahlburg H FL, Gruen R S, New M I, Bell J J, Morishima A, Shimshi M, Bueno Y, Vargas J, Baker S W. Gender change from female to male in classical congenital adrenal hyperplasia. Horm Behav. 30 319-332 1996;
- 19 Plante E, Boliek C, Binkiewicz A, Erly W K. Children with congenital adrenal hyperplasia. Dev Med Child Neurol. 38 423-437 1996;
- 20 Rolland-Cachera M F, Cole T J, Sempé M, Tichet J, Rossignol C, Charraud A. Body mass index variations: centiles from birth to 87 years. Eur J Clin Nutrit. 45 13-21 1991;
- 21 Sato T, Koizumi S. Effects of androgens on childhood behaviour. Acta Paediatr Jpn. 33 639-644 1991;
- 22 Sohlberg S, Strober M. Personality in anorexia nervosa: an update and a theoretical integration. Acta Psychiatr Scand 89 ((Suppl 378)) 1-15 1994;
- 23 Van der Kamp H J, Slijper F ME, Brandenburg H, de Muinck Keizer-Schrama S MPF, Drop S LS, Molenaar J C. Evaluation of young women with congenital adrenal hyperplasia: a pilot study. Horm Res 37 ((Suppl 3)) 45-49 1992;
- 24 Walsh B T, Devlin M J. Eating disorders: progress and problems. Science. 280 1387-1390 1998;
- 25 Whitaker A, Davis M, Shaffer D. The struggle to be thin: a survey of anorexic and bulimic symptoms in a non-referred adolescent population. Psychol Med. 19 143-163 1989;
- 26 Woodside D B. A review of anorexia nervosa and bulimia nervosa. Curr Probl Pediatr. 25 67-89 1995;
- 27 Yates A. Current perspectives on the eating disorders: I. History, psychological, and biological aspects. J Am Acad Child Adolesc Psychiatry. 28 813-828 1989;
- 28 Zucker K J, Bradley S J, Oliver G, Blake J, Fleming S, Hood J. Psychosexual development of women with congenital adrenal hyperplasia. Horm Behav. 30 300-318 1996;
- 29 Physical status: the use and interpretation of anthropometry: report of a WHO expert committee. WHO Tech Rep Ser 854 1-452 1995;
1 * Presented in part at the 37th Annual Meeting of the European Society for Paediatric Endocrinology (ESPE), Florence, Sept. 24-27, 1998
Prof. Dr. med. W. G. Sippell
Department of Paediatrics
Christian-Albrechts-Universität zu Kiel
Schwanenweg 20
D-24105 Kiel
Germany
Telefon: + 49-431-597-1626
Fax: + 49-431-597-1675
eMail: sippell@pediatrics.uni-kiel.de