第80例——青年女性,坐立不耐受伴进食后呕吐和躯体无汗2个月余

The eightieth case： young women, sitting and standing intolerance with vomiting after eating and body sweating for two months

患者青年女性,表现为急性发病的直立性低血压及胃肠动力障碍体检及辅助检查均提示广泛的自主神经神经病变.患者血清抗核抗体及抗干燥综合征抗原A抗体水平升高,唇腺活体组织检查（活检）提示干燥综合征,神经活检提示无髓神经纤维大量丢失.给予静脉丙种球蛋白及糖皮质激素治疗与对症治疗后,患者直立性低血压症状明显缓解,但胃肠动力障碍无明显好转.

The patient is a young woman,manifested as orthostatic hypotension and gastrointestinal motility disorders in acute onset.The physical examination and laboratory test suggested disorders of wide range of autonomic neuropathy.The levels of serum antinuclear antibody and SSA antibody were elevated.The biopsy of lip gland suggested Sjogren＇s syndrome.Nerve biopsy showed loss of a large number of unmyelinated nerve fibers.After the treatment of intravenous gamma globulin and glucocorticoid and symptomatic treatment,the symptoms of orthostatic hypotension were significantly relieved,but the gastrointestinal motility was not significantly improved.