Kühl P G, Ulmer H E, Schmidt W, Wille L
Klin Padiatr. 1985 Jul-Aug;197(4):282-7. doi: 10.1055/s-2008-1033984.
With the advent of prenatal diagnosis and the feasibility of intrauterine treatment, non-immune hydrops fetalis (NHIF) is gaining increasing importance. 14 patients are reported 10 of whom having been diagnosed prenatally. 13 were born prematurely severe asphyxia being a frequent occurrence. 7 out of 14 infants survived. In 5 cases no definite etiology could be established. In 7 patients NHIF was attributable to cardiovascular diseases, predominantly associated with brady- or tachycardia. A survey of the literature reveals that etiology remains unidentified in about 40%. For the rest fetal, maternal or placental diseases are the primary causes. The abundance of underlying diseases are operative via three pathophysiological pathways: hemodynamic disturbances, decreased oncotic pressure and increased capillary permeability. By ultrasound examination, NHIF can be detected prenatally permitting intrauterine treatment in selected cases. In combination with improved postnatal management, this might contribute to a better outcome of NIHF which is still associated with a high mortality rate. Meticulous diagnostic work-up facilitates genetic counseling and the pursuit of prenatal diagnosis in subsequent pregnancies.
随着产前诊断的出现以及宫内治疗的可行性,胎儿非免疫性水肿(NHIF)正变得越来越重要。报告了14例患者,其中10例为产前诊断。13例早产,重度窒息很常见。14例婴儿中有7例存活。5例无法确定明确病因。7例患者的NHIF归因于心血管疾病,主要与心动过缓或心动过速有关。文献调查显示,约40%的病因仍不明。其余病例中,胎儿、母体或胎盘疾病是主要原因。大量潜在疾病通过三种病理生理途径起作用:血液动力学紊乱、胶体渗透压降低和毛细血管通透性增加。通过超声检查,可在产前检测到NHIF,从而在特定病例中进行宫内治疗。结合改进的产后管理,这可能有助于改善NHIF的预后,NHIF的死亡率仍然很高。细致的诊断检查有助于遗传咨询以及后续妊娠中进行产前诊断。