Premature pubarche: distinguishing between nonclassic congenital adrenal hyperplasia and idiopathic premature adrenarche

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Precocious pubarche: distinguishing late-onset congenital adrenal hyperplasia from premature adrenarche.

CONTEXT Because precocious pubarche (PP) reveals late-onset congenital adrenal hyperplasia (LO-CAH) in 5 to 20% of cases, an adrenal stimulation test is recommended in all patients presenting with it. This test is stressful and expensive, and results are normal in more than 80% of cases. OBJECTIVE Our objective was to identify clinical and plasma predictors of LO-CAH among patients presenting...

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Prevalence of Nonclassic Congenital Adrenal Hyperplasia in Turkish Children Presenting with Premature Pubarche, Hirsutism, or Oligomenorrhoea

Background. Nonclassic congenital adrenal hyperplasia (NCAH), caused by mutations in the gene encoding 21-hydroxylase, is a common autosomal recessive disorder. In the present work, our aim was to determine the prevalence of NCAH presenting as premature pubarche (PP), hirsutism, or polycystic ovarian syndrome (PCOS) and to evaluate the molecular spectrum of CYP21A2 mutations in NCAH patients. M...

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Nonclassic Congenital Adrenal Hyperplasia

PURPOSE OF REVIEW Late-onset or nonclassic congenital adrenal hyperplasia (NCAH) due to 21-hydroxylase deficiency is one of the most common autosomal recessive disorders. Reported prevalence ranges from 1 in 30 to 1 in 1000. Affected individuals typically present due to signs and symptoms of androgen excess. The purpose of this review is to provide current information regarding the pathophysiol...

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Premature pubarche in an infant: nonclassical congenital adrenal hyperplasia or mini-puberty variant?

A previously healthy, 7-mo-old male infant presented with a 3-mo history of pubic hair. He was a healthy, term newborn who was born to nonconsanguineous parents, and had appropriate weight and length. Physical examination revealed thick and long pubic hair on the scrotum, without hyperpigmentation or enlargement of the penis (Fig. 1a). No other signs of virilization or secondary sexual developm...

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[Premature pubarche].

Premature adrenarche refers to the appearance of public hair before age 8 years in girls and 9 years in boys, without other signs of puberty or virilization. Growth velocity may be increased and slightly advanced bone maturation is often present and is usually well correlated with the height age. The transient acceleraration of growth and of bone maturation have no negative effects on the onset...

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ژورنال

عنوان ژورنال: Endocrine Abstracts

سال: 2013

ISSN: 1479-6848

DOI: 10.1530/endoabs.32.p796