Women's Corner
Women's Corner
A) GnRH-dependent(Central or true) :
1. Idiopathic
2. CNS problems :
· Congenital : hudrocephalus
· Traumatic
· Inflammatory : Postmeneingitis and postencephalitis
· Neoplastic : hypothalamic hamartoma, craniopharyngioma, teratoma, neurofibroma
· Metabolic : abnormal skull development due to rickets
· Hormonal : Primary juvenile hypothyroidism
Idiopathic (consitiutional) precocious puberty :
- The most common condition (75-90%) and diagnosed by exclusion .
-Due to premature maturation of the hypothalamo-pituitary-ovarian axis(elevated IGF-1)
- There is premature closure of the epiphysis leading to a short stature
- Fertility and age of menopause are not affected
- In consititutional cases run in families , while idiopathic cases ocurrs earlier .
Primary juvenile hypothyroidism : Postulated mechanisms are : 1) Increased TRH leads to increased production of -subunit of Gn . 2) TSH has a stimulatory effect on the prolactin and there is cross reactivity between TSH and LH . 3) Hypothyroid state leads to decreased catabolism of Gn . it is characterized by :
- Short stature due to absence of thyroxine (but not bone age acceleration)
- There is galactorrhea, scanty pubic and axillary hair and may be mental affection
- The sella turcica is frequently enlarged, but with treatment, pubertal development will stop and even regress .
B) GnRH-independent (pseudo-) :
· Estrogen producing ovarian tumor
· Estorgen producing adrenal tumor
· Iatrogenic estrogens in vulvovaginitis
· Mc-Cune Albright syndrome
· Ectopic gonadotropin production : ovarian choriocarcinoma,dysgerminoma and hepatoblastoma
Mc-Cune Albright syndrome (Albright’s polystotic fibrous dysplasia) :
- Incidence : 5% of prococious puberty
- Etiology : Recently demonstrated to be due to autonomus estrogen secretion by the ovary. This autonomous activity of the ovary and other endocrine glands is now explained by a mutaion affecting the structure of the G-protein (regulatroy subunit of the adenyl cyclase) leading to attenuation of the GTPase activity which is necessary to terminate the adenylate cyclase actvivity . Disturbance of the hypothalamus by the sclerotic bone growth on the base of the skull is an old theory
- Characters :
· Age of affection : may start at 9 months and the first sign is menarche
· Multiple cystic bone lesions with easy fractures but adult height is mormal
· Cafe au lait patches in the skin
· Endocrinal : cushing, gigantism or hyperthyroidism may be manifestations
· Fertility is not affected .
3. Heterosexual :
· Adrenal : Delayed type of CAH , cushing syndrome and virilizing adrenal tumor
· Ovarian : PCO and virilizing ovarian or adrenal tumor
· Idiopathic hirsutism
· Some cases of male pseudohermaphroditism
dr. Hisham Al-Inany Lecturer in Obstetrics and Gynecology Department, Cairo University, Egypt Dr. Amr Hussein Lecturer in Obstetrics and Gynecology Department, Cairo University, Egypt