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THE ADRENOGENITAL SYNDROME

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Abstract

HE adrenogenital syndrome due to congenital adrenocortical hyperplasia is another example of an inborn error of metabolism in man, which has enjoyed closer scrutiny and etiological clarification in recent years. This condition is hereditary and behaves as a Mendelian recessive characteristic. Its imprint is heavier on female children, in whom it produces masculinization of the external genitalia owing to the initiation of the disorder during fetal life. This disease constitutes one of the commonest causes of female pseudohermaphroditism. The incrimination of the adrenal cortex in this syndrome has long been a matter of morphologic record—in patients who died, this gland was many times normal size. Since the availability of the Zimmermann reaction for the measurement of urinary 17-ketosteroids, it has repeatedly been shown that the androgens (of adrenal origin) are regularly elevated in patients with this condition. In recent years, the administration of hydrocortisone or its many analogs has been

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HE adrenogenital syndrome due to congenital adrenocortical hyperplasia is another example of an inborn error of metabolism in man, which has enjoyed closer scrutiny and etiological clarification in recent years. This condition is hereditary and behaves as a Mendelian recessive characteristic. Its imprint is heavier on female children, in whom it produces masculinization of the external genitalia owing to the initiation of the disorder during fetal life. This disease constitutes one of the commonest causes of female pseudohermaphroditism. The incrimination of the adrenal cortex in this syndrome has long been a matter of morphologic record—in patients who died, this gland was many times normal size. Since the availability of the Zimmermann reaction for the measurement of urinary 17-ketosteroids, it has repeatedly been shown that the androgens (of adrenal origin) are regularly elevated in patients with this condition. In recent years, the administration of hydrocortisone or its many analogs has been

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Available abstract

HE adrenogenital syndrome due to congenital adrenocortical hyperplasia is another example of an inborn error of metabolism in man, which has enjoyed closer scrutiny and etiological clarification in recent years. This condition is hereditary and behaves as a Mendelian recessive characteristic. Its imprint is heavier on female children, in whom it produces masculinization of the external genitalia owing to the initiation of the disorder during fetal life. This disease constitutes one of the commonest causes of female pseudohermaphroditism. The incrimination of the adrenal cortex in this syndrome has long been a matter of morphologic record—in patients who died, this gland was many times normal size. Since the availability of the Zimmermann reaction for the measurement of urinary 17-ketosteroids, it has repeatedly been shown that the androgens (of adrenal origin) are regularly elevated in patients with this condition. In recent years, the administration of hydrocortisone or its many analogs has been

Key concepts: Adrenogenital syndrome, Medicine, Congenital adrenal hyperplasia, Adrenal cortex, Etiology, Urinary system, External genitalia, Endocrinology

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