Ambiguous genitalia: medical, socio-cultural and religious factors affecting management in Saudi Arabia.
Twenty-eight children with ambiguous genitalia were seen at King Khalid University Hospital over a 6-year period. The incidence of this disorder was 0.4/1000 live births. Of the total, 21 (75%) were Saudis and seven (25%) were non-Saudis. The consanguinity rate was 67.9%. Twenty-four (85.7%) were born in hospital and four (14.3%) at home. In only three (10.7%) was the news first broken to the parents by a senior doctor, in 13 (46.4%) by a junior doctor, and in 11 (39.3%) by a nurse. Ambiguous genitalia were observed in 22 (78.6%) at birth and in six (21.4%) were picked up later. Owing to a lack of immediate investigative facilities and for some socio-cultural reasons, 19 of the latter groups were assigned sex without prior investigations. There was an obvious preference to assign male sex. On investigation, 13 (46.4%) had XX chromosomes, 11 (39%) XY and one (3.6%) XO: in three (10.7%), chromosomal results were not available. There were 14 cases (50%) of congenital adrenal hyperplasia, two of 5-alpha reductase deficiency (7.1%), and five of testicular feminization syndrome (17.9%), in addition to others. After investigation, five (17.9%) of the children needed sex reassignment. This was accepted by two and rejected for socio-cultural reasons by three. The opinion of the religious leaders was obtained. Some recommendations on management of these cases are made, based on our local experience.
Abdullah
M A
MA
Department of Paediatrics, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Katugampola
M
M
al-Habib
S
S
al-Jurayyan
N
N
al-Samarrai
A
A
Al-Nuaim
A
A
Patel
P J
PJ
Niazi
M
M
D012734:Disorders of Sex Development / Q000453:epidemiology / Q000523:psychology* / Q000601:surgery; D005260:Female; D005835:Genitalia / Q000002:abnormalities*; D006801:Humans; D007231:Infant, Newborn; D007514:Islam / Q000523:psychology; D008297:Male; D011593:Psychology, Social; D012068:Religion and Medicine; D012529:Saudi Arabia / Q000453:epidemiology