关键词: Alport syndrome Case report Fetal outcome Maternal outcome Alport syndrome Case report Fetal outcome Maternal outcome

Mesh : Adult Cesarean Section / adverse effects Female Humans Hypertension / complications Infant, Newborn Nephritis, Hereditary / complications Pregnancy Pregnancy Complications / etiology Proteinuria / etiology

来  源:   DOI:10.1186/s13256-022-03565-7

Abstract:
BACKGROUND: Alport syndrome is a rare inherited disorder affecting the glomerular basement membrane, manifested by hematuria and proteinuria that is commonly associated with ocular and hearing defects. There is limited information about the maternal and fetal outcomes of Alport syndrome in pregnancy.
METHODS: We describe a smooth course of pregnancy, a good maternal outcome, and a good fetal outcome in three consecutive pregnancies for a 35-year-old Mediterranean woman with Alport syndrome over a 10-year duration. Although there was a nephrotic range of progressive proteinuria in all her pregnancies, there was a prompt drop in proteinuria within 2 weeks of her deliveries. She has constantly shown a normal serum creatinine level and a normal serum protein level in all her pregnancies. Apart from a single episode of asymptomatic hypertension in her second pregnancy at 34 weeks of gestation that returned to a normal range immediately after delivery, she was normotensive antenatally and postnatally. She gave birth by cesarean section to three healthy newborns.
CONCLUSIONS: A normal prepregnancy creatinine level and a mild range of proteinuria in a patient with normotension, who is not on any medication, are associated with good maternal and fetal outcomes. Furthermore, successful pregnancy that is followed by a normal renal function test might suggest a favorable outcome for any future pregnancy.
摘要:
背景:Alport综合征是一种罕见的遗传性疾病,影响肾小球基底膜,表现为血尿和蛋白尿,通常与眼和听力缺陷有关。关于妊娠Alport综合征的母体和胎儿结局的信息有限。
方法:我们描述了一个顺利的怀孕过程,良好的母性结果,一名35岁的地中海女性患有Alport综合征,持续10年,连续三次怀孕,胎儿结局良好。尽管她的所有怀孕都有肾病性的进行性蛋白尿,在分娩后2周内蛋白尿迅速下降.在所有怀孕中,她一直显示出正常的血清肌酐水平和正常的血清蛋白水平。除了在妊娠34周时第二次怀孕时一次无症状高血压发作外,分娩后立即恢复到正常范围,她产前和产后血压正常。她剖腹产了三个健康的新生儿。
结论:正常孕前肌酐水平和轻度蛋白尿的患者,没有服用任何药物的人,与良好的母体和胎儿结局有关。此外,成功妊娠后再进行正常肾功能检查,可能提示未来妊娠结局良好.
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