Maldonado Maria, Alhousseini Ali, Awadalla Michael, Idler Jay, Welch Robert, Puder Karoline, Patwardhan Manasi, Gonik Bernard
Department of Obstetrics and Gynecology, Wayne State University School of Medicine, Detroit, MI, USA.
Case Rep Obstet Gynecol. 2017;2017:5646247. doi: 10.1155/2017/5646247. Epub 2017 Jun 7.
Intrahepatic cholestasis of pregnancy is seldom associated with significant vitamin K deficiency. We report a case of a 16-year-old primigravid patient at 24 weeks and 3 days of gestation who presented with pruritus, hematuria, and preterm labor. Laboratory work-up showed severe coagulopathy with Prothrombin Time (PT) of 117.8 seconds, International Normalized Ratio (INR) of 10.34, and elevated transaminases suggestive of intrahepatic cholestasis of pregnancy. Her serum vitamin K level was undetectable (<0.1 nMol/L). Initial therapy consisted of intramuscular replacement of vitamin K and administration of fresh frozen plasma. Her hematuria and preterm labor resolved and she was discharged. She presented in active labor and delivered at 27 weeks and 1 day. Her bile acids (93 /L) and INR (2.32) had worsened. She delivered a male infant, 1150 grams with Apgar scores 7 and 9. The newborn received 0.5 mg of intramuscular vitamin K shortly after delivery but went on to develop bilateral grade III intraventricular hemorrhages by day 5. Intrahepatic cholestasis in pregnancy and nutrition issues were identified as the main risk factors for the severe coagulopathy of this patient. This case underlines the importance of evaluation of possible severe coagulopathy in patients with intrahepatic cholestasis of pregnancy in order to avoid serious maternal or fetal adverse outcomes.
妊娠期肝内胆汁淤积症很少与严重的维生素K缺乏相关。我们报告一例16岁初孕妇，妊娠24周零3天时出现瘙痒、血尿和早产。实验室检查显示严重凝血功能障碍，凝血酶原时间（PT）为117.8秒，国际标准化比值（INR）为10.34，转氨酶升高提示妊娠期肝内胆汁淤积症。她的血清维生素K水平检测不到（<0.1 nMol/L）。初始治疗包括肌肉注射补充维生素K和输注新鲜冰冻血浆。她的血尿和早产症状缓解并出院。她进入活跃期分娩，于妊娠27周零1天时分娩。她的胆汁酸（93 μmol/L）和INR（2.32）有所恶化。她产下一名男婴，体重1150克，阿氏评分分别为7分和9分。新生儿在出生后不久接受了0.5毫克肌肉注射维生素K，但在第5天时发生了双侧III级脑室内出血。妊娠期肝内胆汁淤积症和营养问题被确定为该患者严重凝血功能障碍的主要危险因素。该病例强调了对妊娠期肝内胆汁淤积症患者评估可能存在的严重凝血功能障碍的重要性，以避免严重的母婴不良结局。
