Abstract Introduction Girls with classical congenital adrenal hyperplasia (CAH) are exposed to excess fetal androgens in-utero, and often born masculinised genitalia. They conventionally reared as females, but show more “boyish” gender-role behaviour (GRB) gender-identity (GI) issues in childhood adolescence. Male-rearing is also reported mainly due delayed treatment and/or socio-cultural facto...

46XY women is a label that gathers together a number of different conditions for which the natural history in to adult life is still only partially known. A common feature is the difficulty that many women encounter when approaching clinicians. In this review we assemble medical, surgical and psychological literature pertaining adult 46XY women together with our experience gained from an adult ...

The National Hospital of Pediatrics (NHP), Hanoi, Vietnam is an 1200 bed tertiary referral centre servicing approximately 30 million people from northern provinces of Vietnam. This audit was undertaken to analyze anecdotal reports of increasing patient numbers. Retrospective review of all CAH patients registered at NHP from 19995.2014. Ethical clearance was granted by the NHP Directorate. At th...

BACKGROUND Congenital adrenal hyperplasia with apparent combined P450C17 and P450C21 deficiency is associated with accumulation of steroid metabolites, indicating impaired activity of 17alpha-hydroxylase and 21-hydroxylase. However, no mutations have been reported in the CYP17 and CYP21 genes, which encode these P450 enzymes. Affected girls are born with ambiguous genitalia, but their circulati...

Introduction: Steroid cell tumors (SCTs) constitute less than 0.1% of all ovarian tumors. They are divided into 3 categories according to cell of origin: Stromal Luteoma arising from stromal cells of the ovary, Leydig cell tumor arising from Leydig cells, and SCT not otherwise specified (NOS) when the origin of the tumor is not defined. Case Presentation:...

Leydig cell tumours represent more than 75% of all testosterone-secreting ovarian masses. These benign tumours are frequently occult or very small, but cause dramatic virilization. Chronic hyperandrogenism can also induce systemic complications, which increase morbidity and mortality risk. One of the most obvious effects of increased testosterone levels is polycythemia, a complication which ind...

Congenital adrenal hyperplasia (CAH) is a common hereditary disease in the National hospital of pediatrics (NHP). CAH is treated with hormone replacement therapy for life. It is necessary to evaluate treatment outcome in order to have monitoring method and further treatment plan. Thus we study the treatment results and factors affecting treatment outcome of children with CAH being treated in th...

L'hyperplasie congénitale des surrénales par déficit en 11ß hydroxylase se voit dans 5 à 10 % des cas et est diagnostiquée, habituellement, devant des signes de virilisation d'un nouveau né ou d'un fœtus 46 XX et plus tard devant une HTA avec une hypokaliémie. Le pronostic de taille chez ces patients est très souvent compromis. Nous rapportons le cas d'un déficit en 11ß hydroxylase chez une enf...

BACKGROUND Physiological functioning of the testes is important for cardiac health besides for virilisation, physical strength, behavior and reproduction; moreover, hypogonadism has been demonstrated as a significant risk marker of increased all-cause and cardiovascular mortality. CASES PRESENTATION We reported two cases of long-standing hypogonadotropic hypogonadism presenting with wasting, ...

Adrenocortical tumour is rare in children. We report on a female infant with adrenocortical carcinoma presenting with pseudoprecocious puberty at the age of two. She had a history of gradually increasing public hair growth after birth. Physical examination showed signs of virilisation such as pubic hair growth and hirsutism with evidence of facial hair growth. On biochemical evaluation, DHEA-S,...