PDF(Pubmed)
Abstract：
BACKGROUND: Diabetes insipidus is a syndrome characterized by polyuria, which is almost always associated with polydipsia. The most frequent cause is central diabetes insipidus, which is the result of an inadequate secretion of the antidiuretic hormone, and diagnosis involves differentiating it from other causes of polyuria and polydipsia.
METHODS: Here, we present a clinical case of a previously healthy 13-year-old Nepali boy, who, in December 2022, was found to have intense polydipsia accompanied by polyuria. He had bilateral lower limb weakness at the time of presentation. Biochemical evaluation demonstrated raised serum sodium (181 mEq/L), serum creatinine (78 μmol/L), and serum uric acid (560 μmol/L) with suppressed serum potassium (2.7 mEq/L), which was the major concern to the clinicians. Further laboratory workup revealed an increased serum osmolarity (393.6 mOsm/kg) with reduced urine osmolarity (222.7 mOsm/kg). On contrast magnetic resonance imaging of the brain, a thick-walled third ventricular cyst with bilateral foramen obstruction, thin membrane-like structure at top of aqueduct of Sylvius with gross obstructive hydrocephalus (inactive), and compressed and thinned pituitary gland with no bright spot was observed. The laboratory findings, radiological findings, and case presentation provided the provisional diagnosis of diabetes insipidus due to hydrocephalus and third ventricular cyst.
CONCLUSIONS: Central diabetes insipidus due to hydrocephalus, though rare, can have serious complications including the predilection to develop a deficit of other pituitary hormones. Thus, even if hydrocephalus is dormant with normal intracranial pressure, it must be addressed during investigations of central diabetes insipidus.
METHODS: Here, we present a clinical case of a previously healthy 13-year-old Nepali boy, who, in December 2022, was found to have intense polydipsia accompanied by polyuria. He had bilateral lower limb weakness at the time of presentation. Biochemical evaluation demonstrated raised serum sodium (181 mEq/L), serum creatinine (78 μmol/L), and serum uric acid (560 μmol/L) with suppressed serum potassium (2.7 mEq/L), which was the major concern to the clinicians. Further laboratory workup revealed an increased serum osmolarity (393.6 mOsm/kg) with reduced urine osmolarity (222.7 mOsm/kg). On contrast magnetic resonance imaging of the brain, a thick-walled third ventricular cyst with bilateral foramen obstruction, thin membrane-like structure at top of aqueduct of Sylvius with gross obstructive hydrocephalus (inactive), and compressed and thinned pituitary gland with no bright spot was observed. The laboratory findings, radiological findings, and case presentation provided the provisional diagnosis of diabetes insipidus due to hydrocephalus and third ventricular cyst.
CONCLUSIONS: Central diabetes insipidus due to hydrocephalus, though rare, can have serious complications including the predilection to develop a deficit of other pituitary hormones. Thus, even if hydrocephalus is dormant with normal intracranial pressure, it must be addressed during investigations of central diabetes insipidus.
摘要：
背景：尿崩症是一种以多尿为特征的综合征，这几乎总是与多饮有关。最常见的原因是中枢性尿崩症，这是抗利尿激素分泌不足的结果，诊断包括将其与多尿和多饮的其他原因区分开来。
方法：这里，我们提供了一个以前健康的13岁尼泊尔男孩的临床病例，谁,在2022年12月，被发现有强烈的多饮并伴有多尿。他在就诊时患有双侧下肢无力。生化评估显示血清钠升高（181mEq/L），血清肌酐（78μmol/L），和血清尿酸（560μmol/L）与抑制血清钾（2.7mEq/L），这是临床医生最关心的问题。进一步的实验室检查显示，血清渗透压增加（393.6mOsm/kg），尿液渗透压降低（222.7mOsm/kg）。对比大脑的磁共振成像，伴有双侧孔阻塞的厚壁第三脑室囊肿,Sylvius输水管顶部的薄膜状结构伴严重阻塞性脑积水（不活跃），垂体压缩变薄，无亮点。实验室的发现,放射学发现，和病例介绍提供了由于脑积水和第三脑室囊肿引起的尿崩症的临时诊断。
结论：脑积水引起的中心性尿崩症，虽然罕见，可能有严重的并发症，包括其他垂体激素缺乏的倾向。因此，即使脑积水处于正常颅内压的休眠状态,在对中枢性尿崩症进行调查时必须加以解决.
方法：这里，我们提供了一个以前健康的13岁尼泊尔男孩的临床病例，谁,在2022年12月，被发现有强烈的多饮并伴有多尿。他在就诊时患有双侧下肢无力。生化评估显示血清钠升高（181mEq/L），血清肌酐（78μmol/L），和血清尿酸（560μmol/L）与抑制血清钾（2.7mEq/L），这是临床医生最关心的问题。进一步的实验室检查显示，血清渗透压增加（393.6mOsm/kg），尿液渗透压降低（222.7mOsm/kg）。对比大脑的磁共振成像，伴有双侧孔阻塞的厚壁第三脑室囊肿,Sylvius输水管顶部的薄膜状结构伴严重阻塞性脑积水（不活跃），垂体压缩变薄，无亮点。实验室的发现,放射学发现，和病例介绍提供了由于脑积水和第三脑室囊肿引起的尿崩症的临时诊断。
结论：脑积水引起的中心性尿崩症，虽然罕见，可能有严重的并发症，包括其他垂体激素缺乏的倾向。因此，即使脑积水处于正常颅内压的休眠状态,在对中枢性尿崩症进行调查时必须加以解决.
