Gender assignment in disorders of sex: An Islamic perspective from Saudi Arabia

Abstract

Genital ambiguity, is defined as a group of congenital conditions in which development of chromosomal, gonadal, or anatomic sex is atypical. It constitutes a major social emergency and the decision making in relation to sex assignment has been perceived as an extremely disturbing and difficult to both families and health care professionals. It can also be a medical emergency as it may lead to life-threatening adrenal crisis. Each patient should be evaluated individually by a well-coordinated experienced multidisciplinary team of specialists in a higher center capable of managing such disorders. The team should include a geneticist, neonatologist, endocrinologist, pediatric surgeon, urologist, psychiatrist and psychologist, a social worker, and other specialists such as a nurse, and gynecologist to be consulted whenever needed. The estimated incidence was found to be approximately 0.1% to 0.2% worldwide i.e, 1:4500-5000 live birth. The 46XX is the most common cause, while the majority of these patients congenital adrenal hyperplasia (CAH) is the most underlying aetiology. The 46XY DSD is a heterogeneous disorders that often result from a disruption in the production or response to testosterone, dihydrotestosterone, or Mullerian inhibitory substances. Chromosomal DSD includes condition resulting from abnormal meiosis, including Klinefelter syndrome (47XXY) and Turner syndrome. The evaluation of patients with sex disorders requires a through history, physical examination, karyotype, serum electrolytes, and specific hormones, such as 17-OH-progesterone.The pediatric radiologist has a major role in diagnosis. In Saudi Arabia, a general guidelines were suggested by the council of senior ulama to manage patients with DSD in accordance with the islamic laws. This should be the practice in other muslim countries. Health professionals need to be aware of the existence of such recommendation for a better care of patients.