患有短肠综合征的母亲的儿子在新生儿中缺乏维生素 K,Pediatrics International

当前位置： X-MOL 学术 › Pediatr. Int. › 论文详情

Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)

患有短肠综合征的母亲的儿子在新生儿中缺乏维生素 K
Pediatrics International ( IF 1.0 ) Pub Date : 2018-10-01 , DOI: 10.1111/ped.13684
Mariko Ashina ₁ , Kazumichi Fujioka ₁ , Kosuke Nishida ₁ , Kazumoto Iijima ₁

Affiliation

维生素 K 缺乏性出血 (VKDB) 是新生儿的一种毁灭性疾病，尤其是对于母乳喂养的婴儿。建议使用通用维生素 K (VK) 预防剂来预防 VKDB。VK 从母体到胎儿的转运机制尚不清楚，但脂溶性维生素（如 VK）的转运速度比水溶性维生素慢得多。因此，新生儿易患母体 VK 缺乏症。据报道，新生儿 VKDB 伴有基于潜在疾病（如克罗恩病）的母亲营养不良。我们报告了一名新生儿 VK 缺乏症的无症状患者，其母亲因先天性巨结肠疾病相关疾病而患有全结肠切除术后短肠综合征。一名接受过短肠综合征治疗的 21 岁初产妇在妊娠 33 周时被转诊至本中心进行围产期管理。由于先天性巨结肠疾病相关疾病，她从新生儿期开始接受肠造口术和肠外营养治疗。6 岁时，她接受了全结肠切除术和腹肛门直通切除术。15 岁时，由于疑似炎症性肠病，开始口服美沙拉嗪。从 19 岁开始，她就没有肠外营养，情况稳定，成分营养和 iv 电解质校正（根据需要）。经与孕前相同的治疗，孕程控制良好，能正常进餐，胎儿生长正常。她在 33 周时入院，怀孕 0 天进行围产期管理并每天接受细胞外液治疗。每日口服美沙拉嗪、一水磷酸二氢钠、葡萄糖酸钾、法莫替丁、氧化镁、酪酸梭菌MIYAIRI 588益生菌（日本东京MIYARISAN制药）细颗粒，分娩前体重38.9 kg（体质量指数：17.5）。33周6天时因肾积水加重腹痛，导致饮食摄入减少。在第 34 周零 0 天放置输尿管支架。母亲逐渐发烧，并在 35 周 1 天时因怀疑尿路感染接受了抗生素治疗。然而，她的全身状况恶化，由于血清炎症标志物和凝血功能障碍，她被诊断为临床败血症。所以，在全身麻醉下通过紧急剖宫产终止妊娠。一名男婴（出生体重，2,314 克）出生时因母亲的麻醉而没有呼吸，并且在 1 分钟和 5 分钟时的 Apgar 评分分别为 2 和 7。他出生后不久就进行了气管插管和机械通气，但在出生后 8 h 苏醒后拔管。常规超声和 X 射线正常，但初始血液检查显示大量凝血功能障碍（活化部分凝血活酶时间延长和凝血酶原时间减少百分比），由维生素 K 缺失或拮抗剂-II（PIVKA-II；表 1）引起的蛋白质大大升高。根据母亲的病史和婴儿的数据，我们将这种情况诊断为无症状的 VK 缺乏症，并在出生时静脉注射维生素 K2（甲萘醌，2 毫克），以及在出生后 2 小时和 8 小时后连续进行凝血试验结果。出生后24小时内凝血功能迅速改善，无出血。他于第 20 天出院，没有任何后遗症。在常规维生素 K2 治疗（第 4 天）后，PIVKA-II 在第 4 天下降 44%，并在出生后第 17 天恢复正常。该婴儿的母亲患有疑似败血症的短肠综合征。随后，由于出生时 VK 缺乏，他出现了明显的凝血功能障碍，没有任何出血症状。我们之前曾报道过一例因凝血功能障碍导致的急性期后脑室内出血病例，尽管接受了单一 VK 治疗，但在一名患有克罗恩病的母亲的早产儿中。我们建议仔细筛查因肠内吸收不良而营养不良的母亲分娩的新生儿的凝血功能障碍。西口等人。在他们的基于人群的研究中推荐多剂量 VK，其中 0.19% 的婴儿肝素试验低 (<40%)，即使在生命的第一周口服 VK 两次后也是如此。在本病例中，我们预计产前母亲 VK 吸收不良会导致凝血功能障碍，并成功使用多剂量 VK 治疗婴儿，没有发生任何出血事件。为了检测 VK 缺乏症，我们通过电化学发光免疫测定法测量了血清 PIVKA-II，发现 PIVKAII 与 VK 治疗后的凝血状态相关。通讯作者： Kazumichi Fujioka，医学博士，神户大学医学研究生院儿科系，7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan。电子邮件：fujiokak@med.kobe-u.ac.jp 2018 年 5 月 10 日收到；2018 年 6 月 18 日修订；2018 年 8 月 17 日接受。doi：10.1111/ped.13684 即使在出生后的第一周内两次口服 VK 后，仍有 19% 的婴儿肝素酶检测结果偏低 (<40%)。在本病例中，我们预计产前母亲 VK 吸收不良会导致凝血功能障碍，并成功使用多剂量 VK 治疗婴儿，没有发生任何出血事件。为了检测 VK 缺乏症，我们通过电化学发光免疫测定法测量了血清 PIVKA-II，发现 PIVKAII 与 VK 治疗后的凝血状态相关。通讯作者： Kazumichi Fujioka，医学博士，神户大学医学研究生院儿科系，7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan。电子邮件：fujiokak@med.kobe-u.ac.jp 2018 年 5 月 10 日收到；2018 年 6 月 18 日修订；2018 年 8 月 17 日接受。doi：10.1111/ped.13684 即使在出生后的第一周内两次口服 VK 后，仍有 19% 的婴儿肝素酶检测结果偏低 (<40%)。在本病例中，我们预计产前母亲 VK 吸收不良会导致凝血功能障碍，并成功使用多剂量 VK 治疗婴儿，没有发生任何出血事件。为了检测 VK 缺乏症，我们通过电化学发光免疫测定法测量了血清 PIVKA-II，发现 PIVKAII 与 VK 治疗后的凝血状态相关。通讯作者： Kazumichi Fujioka，医学博士，神户大学医学研究生院儿科系，7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan。电子邮件：fujiokak@med.kobe-u.ac.jp 2018 年 5 月 10 日收到；2018 年 6 月 18 日修订；2018 年 8 月 17 日接受。doi：10.1111/ped.13684 我们预计会出现产前母体 VK 吸收不良引起的凝血功能障碍，并成功使用多剂量 VK 治疗婴儿，没有发生任何出血事件。为了检测 VK 缺乏症，我们通过电化学发光免疫测定法测量了血清 PIVKA-II，发现 PIVKAII 与 VK 治疗后的凝血状态相关。通讯作者： Kazumichi Fujioka，医学博士，神户大学医学研究生院儿科系，7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan。电子邮件：fujiokak@med.kobe-u.ac.jp 2018 年 5 月 10 日收到；2018 年 6 月 18 日修订；2018 年 8 月 17 日接受。doi：10.1111/ped.13684 我们预计会出现产前母体 VK 吸收不良引起的凝血功能障碍，并成功使用多剂量 VK 治疗婴儿，没有发生任何出血事件。为了检测 VK 缺乏症，我们通过电化学发光免疫测定法测量了血清 PIVKA-II，发现 PIVKAII 与 VK 治疗后的凝血状态相关。通讯作者： Kazumichi Fujioka，医学博士，神户大学医学研究生院儿科系，7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan。电子邮件：fujiokak@med.kobe-u.ac.jp 2018 年 5 月 10 日收到；2018 年 6 月 18 日修订；2018 年 8 月 17 日接受。doi：10.1111/ped.13684 我们通过电化学发光免疫测定法测量了血清 PIVKA-II，发现 PIVKAII 与 VK 治疗后的凝血状态相关。通讯作者： Kazumichi Fujioka，医学博士，神户大学医学研究生院儿科系，7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan。电子邮件：fujiokak@med.kobe-u.ac.jp 2018 年 5 月 10 日收到；2018 年 6 月 18 日修订；2018 年 8 月 17 日接受。doi：10.1111/ped.13684 我们通过电化学发光免疫测定法测量了血清 PIVKA-II，发现 PIVKAII 与 VK 治疗后的凝血状态相关。通讯作者： Kazumichi Fujioka，医学博士，神户大学医学研究生院儿科系，7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan。电子邮件：fujiokak@med.kobe-u.ac.jp 2018 年 5 月 10 日收到；2018 年 6 月 18 日修订；2018 年 8 月 17 日接受。doi：10.1111/ped.13684

"点击查看英文标题和摘要"

Neonatal vitamin K deficiency in the son of a mother with short bowel syndrome

Vitamin K deficiency bleeding (VKDB) is a devastating morbidity in newborns, especially for breast-fed infants. Universal vitamin K (VK) prophylaxis is recommended to prevent VKDB. The mechanism of VK transportation from mother to fetus is unclear, but fat-soluble vitamins, such as VK, have a much slower transfer rate than water soluble vitamins. Therefore, newborns are susceptible to maternal VK deficiency. Neonatal VKDB accompanied by maternal malnutrition based on underlying diseases, such as Crohn’s disease, has been reported. We report an asymptomatic patient with neonatal VK deficiency who was delivered from a mother with post-total colectomy short bowel syndrome because of Hirschsprung disease-related disorders. A 21-year-old primiparous woman who had been treated for short bowel syndrome was referred to the present center for perinatal management at 33 weeks’ gestation. She had been managed with enterostomy and parenteral nutrition because of Hirschsprung disease-related disorders since her neonatal period. At 6 years, she received total colectomy and abdomino-anal pull-through resection. At 15 years, oral mesalazine was started because of suspected inflammatory bowel disease. From 19 years of age she had been free from parenteral nutrition and was in a stable condition with component nutrition and i.v. electrolyte correction as needed. Her pregnancy course was well controlled on the same treatment as before pregnancy, she could take regular meals, and fetal growth was normal. She was admitted at 33 weeks, 0 days of pregnancy for perinatal management and treated with daily extracellular fluid. She took oral mesalazine, monobasic sodium phosphate monohydrate, potassium gluconate, famotidine, magnesium oxide, and Clostridium butyricum MIYAIRI 588 probiotic (MIYARISAN Pharmaceutical Co., Ltd. Tokyo, Japan) fine granules daily, and her bodyweight before delivery was 38.9 kg (body mass index: 17.5). At 33 weeks, 6 days, abdominal pain was exacerbated because of hydronephrosis, resulting in a decrease in dietary intake. A ureteral stent was placed at 34 weeks, 0 days. The mother gradually developed fever and was treated with antibiotics for suspected urinary tract infection at 35 weeks, 1 day. Her general condition worsened, however, and she was diagnosed with clinical sepsis on increased serum inflammatory markers and coagulopathy. Therefore, the pregnancy was terminated via emergency cesarean section under general anesthesia. A male infant (birthweight, 2,314 g) was born not breathing because of the mother’s anesthesia and had Apgar scores of 2 and 7 at 1 and 5 min, respectively. He was tracheally intubated soon after birth and mechanically ventilated, but was extubated 8 h after birth after awakening. Routine ultrasound and X-ray were normal, but initial blood tests showed massive coagulopathy (activated partial thromboplastin time prolongation and % prothrombin time decrease) with greatly elevated protein induced by vitamin K absence or antagonist-II (PIVKA-II; Table 1). Based on the maternal history and the infant’s data, we diagnosed this condition as asymptomatic VK deficiency and gave vitamin K2 (menatetrenone, 2 mg) i.v. at birth, and at 2 and 8 h after birth following consecutive results of a coagulation test. Coagulopathy was improved promptly in 24 h after birth with no bleeding. He was discharged on day 20 without any sequelae. PIVKA-II decreased by 44% on day 4, and normalized on day 17 after birth, following routine vitamin K2 treatment (day 4). This infant was born from a mother with short bowel syndrome with suspected sepsis. He then developed marked coagulopathy because of VK deficiency at birth without any bleeding symptoms. We have previously reported a case of post-acute-phase intraventricular hemorrhage due to coagulopathy, despite single VK treatment, in a preterm infant with maternal Crohn’s disease. We propose careful screening for coagulopathy in newborns delivered from mothers with malnutrition via enteral malabsorption. Nishiguchi et al. recommended multiple doses of VK in their population-based study, in which 0.19% of infants had low hepaplastin test (<40%), even after oral VK twice in the first week of life. In the present case, we expected coagulopathy caused by prenatal maternal VK malabsorption, and successfully treated the infant with multiple doses of VK without any bleeding incidents. To detect VK deficiency, we measured serum PIVKA-II on electro-chemiluminescence immunoassay, and found that PIVKAII correlated with coagulation status after VK treatment. Correspondence: Kazumichi Fujioka, MD PhD, Department of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan. Email: fujiokak@med.kobe-u.ac.jp Received 10 May 2018; revised 18 June 2018; accepted 17 August 2018. doi: 10.1111/ped.13684

更新日期：2018-10-01

点击分享查看原文

点击收藏

阅读更多本刊新发论文本刊介绍/投稿指南