lipoid congenital adrenal hyperplasia

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lipoid congenital adrenal hyperplasia

The most severe form of adrenal hyperplasia (OMIM:201710), which is characterised by profound adrenocortical insufficiency of neonatal onset, hyperpigmentation due to increased production of pro-opiomelanocortin, elevated plasma renin secretion due to reduced aldosterone synthesis, and male pseudohermaphroditism due to deficient foetal testicular testosterone synthesis. Affected infants are phenotypically female regardless of gonadal sex, and die in infancy if mineralocorticoid and glucocorticoid replacement is not begun immediately.

Molecular pathology
Defects of STAR, which encodes a protein that plays a key role in steroid hormone synthesis, cause lipoid congenital adrenal hyperplasia.
References in periodicals archive ?
An AH1 attack helicopter made an emergency landing on the premises of a hotel in the village of Yomitan after a warning light had come on.
El investigador Jose Arturo Martinez Orozco, encargado del Diagnostico de Influenza del instituto Nacional de Enfermedades Respiratorias (INER), dijo que la temporada de influenza 2017-2018 se preve que el virus AH3N2 pueda presentarse con mayor fuerza que el AH1 NI, debido a que asi fue el comportamiento en Australia y el brote en Estados Unidos.
Unlike India, Pakistan uses its American AH1 Cobra gunships extensively against even suspected civilians as can be seen in its Operation Zarb- e- Azb in Taliban and Baloch areas within its own country.
Boekhout AH1, Vincent AD, Dalesio OB, van den Bosch J, Foekema-Tons JH, Adriaansz S, et al.
The AH1 is in service at least with an export customer, Algeria.
The facility repairs all models of the T53 engine, as well as main transmissions and major components for helicopter models including the UH1H, 205, 212, 206, OH58 and AH1 helicopters that are used in firefighting, armed forces and law enforcement operations worldwide.
A lineal relation between the AH1 values and the hard segment content can be seen.