Abstract | PURPOSE: To study the gender assignment and hormonal treatment advocated for disorders of sexual differentiation (DSD). METHODS: A study was done on patients who were reviewed in the Pediatric Intersex Clinic to evaluate the pattern of gender assignment and hormonal treatment advocated. RESULTS AND CONCLUSION: The patients included male pseudohermaphrodite (MPH) 169; congenital adrenal hyperplasia (CAH) 91; mixed gonadal dysgenesis (MGD) 29; true hermaphrodite (TH) 25; pure gonadal dysgenesis ( PGD) 2; persistent mullerian duct syndrome (PMDS) 2 and others ( micropenis, severe hypospadias with cryptorchidism, 46XX male) 39. Out of 91 cases of CAH, 70 (76.9%) were on steroids ( prednisolone, hydrocortisone) and/or mineralocorticoids (fluoricortisone) for adrenal suppression. Out of 146 cases of male pseudohermaphrodite and 21 cases of true hermaphrodite and 3 cases of mixed gonadal dysgenesis reared as males, testosterone was given for local application for phallic growth in 101 and/or as systemic injection for mental makeup after puberty in 41 cases. Systemic testosterone injection was also given for 7 cases of CAH reared as males. Out of 26, 15 cases with mixed gonadal dysgenesis and one out of 2 cases of pure gonadal dysgenesis that attained puberty after being reared as females, after female genitoplasty, were given conjugated oestrogen ( Premarin) supplemented with progesterone, as the uterus was preserved. For 12 post-pubertal cases of complete androgen insensitivity syndrome (AIS), only premarin was given as there was no uterus. Growth hormone and Gn RH analogue was given in 2 patients with CAH to tide over the early bone maturation induced by hormones with equivocal results. Thus judicious hormonal supplementation based upon type of DSD and gender assigned can provide a psychological and cosmetic benefit to patients with DSD.
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Authors | Shilpa Sharma, D K Gupta |
Journal | Pediatric surgery international
(Pediatr Surg Int)
Vol. 24
Issue 10
Pg. 1131-5
(Oct 2008)
ISSN: 0179-0358 [Print] Germany |
PMID | 18704450
(Publication Type: Journal Article)
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Chemical References |
- Adrenal Cortex Hormones
- Androgens
- Estrogens
- Estrogens, Conjugated (USP)
- Progestins
- Human Growth Hormone
- Gonadotropin-Releasing Hormone
- Testosterone
- Progesterone
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Topics |
- Adrenal Cortex Hormones
(therapeutic use)
- Algorithms
- Androgens
(therapeutic use)
- Disorders of Sex Development
(therapy)
- Estrogens
(therapeutic use)
- Estrogens, Conjugated (USP)
(therapeutic use)
- Female
- Gender Identity
- Genitalia
(surgery)
- Gonadotropin-Releasing Hormone
(analogs & derivatives)
- Human Growth Hormone
(therapeutic use)
- Humans
- Male
- Progesterone
(therapeutic use)
- Progestins
(therapeutic use)
- Testosterone
(therapeutic use)
- Urologic Surgical Procedures
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