回复:请教大家一个疑难病,多谢了

来源: MDGG 2007-06-12 18:21:23 [] [博客] [旧帖] [给我悄悄话] 本文已被阅读: 0 次 (1605 bytes)
my 2 cents, without looking at his brain MRI...

1. 诊断 is the key. So they think there is a lesion is the quadrigeminal plate cistern, or the pineal region, as reported in different MRI exams. How big is the lesion? Is there enhancement after contrast (增强)? Is there also suprasellar/hypothalamic lesion besides the "empty sellar"? This is the key for this case. His series MRIs need to be looked at by a neuroradiologist or neurosurgeon. Given the symptoms of central diabetes insipidus AND precocious puberty, I suspect this is a case of Germinoma, with other differential diagnosis depending on the MRI exams. I can not say either way without his MRIs.

2. 是否一定需要药物控制尿量?可否维持液体平衡即可?----Minirin or 弥凝 which is just a structural analogue of the natural pituitary hormone arginine vasopressin. It works on the kidneys and only treat the symptoms. In theory and a perfect condition, the answer is yes.

3.有无更好的治疗方案?---- it depends upon the correct diagnosis. Germinoma is highly radiosensitive, potentially curable.

4、松果体经过观察,无明显改变 -- is it??? 此处病变可否引起尿崩?---- in pediatric pineal region lesions, germinoma is known to have associated suprasellar lesion or subependymal involvement which can cause all his symptoms.

5、还会不会引起其他后果?---- depending upon the correct diagnosis.

6、中枢性尿崩症国外有什么好的治疗方案?7、是否需要终身服药?8、是否会随年龄增长而发育好?9、为什么成人吃一段时间弥凝片后可减量,小孩一减量尿的次数就增多。
---------- see number 5.

Best of luck to the boy!
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