Abstract：
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.
摘要：
维生素K在凝血级联中充当几种蛋白质的γ-羧化的辅因子。维生素K缺乏症（VKD）的临床谱可以无症状到明显的出血。VKD在新生儿中很常见。然而,这可能会在有营养欠佳等风险的患者中表现出来，脂肪吸收不良,包括抗生素在内的药物。一名17岁男性，患有脊髓性肌萎缩症（SMA）1型，气管造口术与呼吸机依赖，胃肠病学家在治疗小肠细菌过度生长（SIBO）后观察到胃造瘘管喂养。调查显示凝血病，随后他被转移到儿科ICU。实验室显示凝血酶原时间（PT）114s[正常9.4-12.5s]，INR（国际标准化比率）12.6[正常<1.1]和部分凝血活酶时间（PTT）90s[正常25.1-36.5s]。混合研究和凝血测定与VKD(低因子VII和因子IX与正常因子V)一致。他的家庭混合喂养方案满足了热量需求，但没有足够的维生素K和其他矿物质摄入量（AI）值。他连续五天接受静脉注射维生素K（植物地己酮），凝血障碍消退（PT13.2s，PTT37.1s,1.2卢比)。在营养师的饮食审查后，患者接受肠内维生素K和其他补充剂出院。临床医生应认识到接受混合管饲料的患者可能不符合足够摄入量（AI）目标的VKD。在未接受营养完整配方或接受量不足的患者中，重要的是监测宏观和微量营养素。
