Metabolic Perspectives for Non-classical Congenital Adrenal Hyperplasia With Relation to the Classical Form of the Disease

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Non-Classical Congenital Adrenal Hyperplasia in Childhood

Congenital adrenal hyperplasia (CAH) is classified as classical CAH and non-classical CAH (NCCAH). In the classical type, the most severe form comprises both salt-wasting and simple virilizing forms. In the non-classical form, diagnosis can be more confusing because the patient may remain asymptomatic or the condition may be associated with signs of androgen excess in the postnatal period or in...

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Endocrinology and auxology of sibships with non-classical congenital adrenal hyperplasia.

The symptoms, auxological characteristics, and stimulated 17-hydroxyprogesterone (17-OHP) concentrations in a group of patients with non-classical 21-hydroxylase deficiency (NCCAH) were compared with those of their siblings. Ten index cases consisting of nine females and one male patient aged 3-33 years and 16 siblings were studied. In the sibling group five subjects were slightly virilised and...

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A girl with steroid cell ovarian tumor misdiagnosed as non-classical congenital adrenal hyperplasia.

Ovarian steroid cell tumors are rarely encountered in prepubertal girls. The majority of these tumors produce hormones, testosterone being the leading one. These tumors may either coexist with or imitate congenital adrenal hyperplasia (CAH). We present a 13-year-old female patient who was diagnosed with non-classical CAH at six years of age while being investigated for premature pubarche. She w...

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Perianal hair as an unusual presentation of non-classical congenital adrenal hyperplasia.

To cite: Gopal-Kothandapani JS, Petkar A, O’Shea E, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/ bcr-2013-010123 DESCRIPTION A 6-year-old girl was referred with a 1 year history of ‘strings in her bottom’. Her parents were unrelated and white Caucasian. Pregnancy and birth history were unremarkable. Postnatal examination had shown normal female genitalia. A...

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Alternative Strategies for the Treatment of Classical Congenital Adrenal Hyperplasia: Pitfalls and Promises

Despite decades of different treatment algorithms, the management of congenital adrenal hyperplasia (CAH) remains clinically challenging. This is due to the inherent difficulty of suppressing adrenal androgen production using near physiological dosing of glucocorticoids (GC). As a result, alternating cycles of androgen versus GC excess can occur and may lead to short stature, obesity, virilizat...

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ژورنال

عنوان ژورنال: Frontiers in Endocrinology

سال: 2019

ISSN: 1664-2392

DOI: 10.3389/fendo.2019.00681