21-羟化酶缺乏症儿童日间护理手术的麻醉问题。

Rajashekar R. Mudaraddi, Yashodanand Kumar Areti
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引用次数: 0

摘要

21-羟化酶缺乏引起的先天性肾上腺增生症是最常见的常染色体隐性遗传病之一。皮质醇合成障碍导致促肾上腺皮质激素(ACTH)对肾上腺的过度刺激,肾上腺增生和雄激素合成过度。超过90%的先天性肾上腺增生是由21-羟化酶缺乏引起的,在1:10 000至1:15 000活产婴儿中发现。小儿先天性肾上腺增生由于21-羟化酶缺乏提出手术是非常罕见的。在这里,我们提出一个15岁的男孩与经典的21-羟化酶缺乏症,谁是成功地管理作为一个一天的情况下,全麻选择性兰花切除术。该病例的出版是由手术后孩子的母亲获得的。
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Anaesthetic Concerns In A Child With 21-Hydroxylase Deficiency For A Day Care Surgery.
Congenital adrenal hyperplasia due to 21-hydroxylase deficiency is one of the most common autosomal recessive hereditary diseases. The impairment of cortisol synthesis leads to excessive stimulation of adrenal gland by adrenocorticotropic hormone(ACTH), adrenal hyperplasia and excessive androgen synthesis. More than 90% of congenital adrenal hyperplasia is caused by 21-hydroxylase deficiency which is found in 1:10,000 to 1:15,000 live births. Children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency presenting for surgeries is very unusual. Here we are presenting a fifteen year old male child with classical 21-hydroxylase deficiency, who was successfully managed as a day case for elective orchidopexy under general anaesthesia. The consent for publication for the case was obtained by mother of child after surgery.
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