Department of Pediatrics, East Tennessee State University, Johnson City, TN, USA.
Dow Medical College, Dow University of Health Sciences, Karachi, Pakistan.
J Diet Suppl. 2023;20(4):543-549. doi: 10.1080/19390211.2022.2026545. Epub 2022 Jan 11.
Vitamin K acts a cofactor for the gamma-carboxylation of several proteins in the coagulation cascade. The clinical spectrum of vitamin K deficiency (VKD) can be asymptomatic to a significant bleeding. VKD is classically seen in newborns. However, this can manifest later in patients with risks such as sub-optimal nutrition, fat malabsorption, medications including antibiotics. A 17-year-old male with spinal muscular atrophy (SMA) Type 1, tracheostomy with ventilator dependent, gastrostomy tube feeding was seen by the gastroenterologist following treatment for small intestinal bacterial overgrowth (SIBO). Investigations showed coagulopathy following which he was transferred to the Pediatric ICU. Labs revealed prothrombin time (PT) 114 s [Normal 9.4-12.5 s], INR (International normalized ratio) 12.6 [Normal < 1.1] and partial thromboplastin time (PTT) 90 s [Normal 25.1-36.5 s]. Mixing studies and coagulation assays were consistent with VKD (low Factor VII and Factor IX with normal Factor V). His home blenderized feeding regimen met the caloric requirement but not the adequate intake (AI) values for vitamin K and other minerals. He received intravenous vitamin K (phytonadione) for five consecutive days with resolution of the coagulopathy (PT 13.2 s, PTT 37.1 s, INR 1.2). The patient was discharged on enteral vitamin K and additional supplements following dietary review by a nutritionist. Clinicians should be cognizant of VKD in patients on blenderized tube feeds which may not meet the adequate intake (AI) goals. In patients who are not receiving nutritionally complete formulas or receiving inadequate volumes, it is important to monitor macro and micronutrients.
维生素 K 作为凝血级联中几种蛋白质的γ-羧化作用的辅助因子。维生素 K 缺乏症 (VKD) 的临床表现从无症状到明显出血不等。VKD 在新生儿中很常见。然而，在营养摄入不足、脂肪吸收不良、包括抗生素在内的药物等风险因素存在的情况下，这种情况可能会在以后出现。一名 17 岁男性，患有脊髓性肌萎缩症 (SMA) 1 型，行气管切开术并依赖呼吸机，通过胃造口管进行肠内喂养，因小肠细菌过度生长 (SIBO) 接受了胃肠病学家的治疗。检查发现凝血功能异常，随后他被转入儿科重症监护病房。实验室检查显示凝血酶原时间 (PT) 为 114 秒 [正常值为 9.4-12.5 秒]，国际标准化比值 (INR) 为 12.6 [正常值<1.1]，部分凝血活酶时间 (PTT) 为 90 秒 [正常值为 25.1-36.5 秒]。混合研究和凝血检测结果一致提示 VKD（VII 因子和 IX 因子低，V 因子正常）。他的家用搅拌机配方满足热量需求，但维生素 K 和其他矿物质的摄入量未达到足够的摄入量 (AI)。他接受了连续 5 天的静脉注射维生素 K（叶绿醌）治疗，凝血功能异常得到纠正（PT 为 13.2 秒，PTT 为 37.1 秒，INR 为 1.2）。患者出院后接受肠内维生素 K 治疗，并在营养师进行饮食回顾后补充其他补充剂。临床医生应注意接受搅拌机管饲的患者中可能存在 VKD，这些患者可能无法达到足够的摄入量 (AI) 目标。对于未接受营养全面配方或接受量不足的患者，监测宏量和微量营养素非常重要。
