COMPASSIONATE USE OF AN INTRAVENOUS FAT EMULSION COMPRISED OF FSIH OIL IN THE TR

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 我们建议提供omega-6为主的脂肪输注(Omeaven)给严重的肠外营养相关胆汁淤积症(结合胆红素和GT；6)的患者在同情的基础上使用。我们将在最长5个月的干预期间监测安全参数。Omeaven将以每天1克/公斤的剂量静脉滴注，直到婴儿脱离肠外营养，持续时间不超过5个月。这是H-21344议定书的延续。 为有肠外营养(PN)相关性胆汁淤积的危重婴儿提供一种机制，以便在没有令人满意的替代治疗的情况下接受Omeaven的体恤治疗。 肠外营养(PN)为继发于肠道长度或功能不足而无法吸收足够的肠道营养物质的患者提供静脉营养补充。PN以其最基本的形式(氨基酸和葡萄糖)包含人类饮食中的大量营养素，通常与脂肪乳剂一起使用，以避免必需的脂肪酸缺乏，并提供高热量的非蛋白质热量来源。此外，PN含有必需的微量营养素(电解质、微量元素和维生素)，以提供最佳的营养方案。在1960年末S发展为PN之前，胃肠吸收功能不足的患者通常死于饥饿和随后的营养不良并发症(1，2)。今天，30,000多名患者永久依赖肠外营养生存。然而，PN仍然与以不可预测的速度发生的肝损伤有关，包括生化，即血清转氨酶和碱性磷酸酶升高，以及组织学变化，如脂肪变性、脂肪性肝炎、脂肪沉积、胆汁淤积、纤维化和肝硬变(3，4)。这些异常在儿科人群中更为普遍，可能会随着PN给药时间的延长而恶化。这种情况的其他危险因素包括早产、低出生体重、长期使用PN、缺乏伴随的肠道摄入、败血症和多次手术(5)。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. We propose to provide an omega-6 predominant lipid infusion (Omegaven) to patients with severe parenteral nutrition associated cholestasis (conjugated bilirubin > 6) on a compassionate use basis. We will monitor for safety parameters during the maximum 5 months of intervention. The Omegaven will be intravenously infused at a dose of 1gm/kg/day until the infant is weaned from parenteral nutrition and for no longer than 5 months. This is a continuation of protocol H-21344. To provide a mechanism for critically ill infants with parenteral nutrition (PN) associated cholestasis to receive Omegaven for compassionate use situations for which there are no satisfactory alternative treatments. Parenteral nutrition (PN) provides intravenous nutritional supplementation for patients unable to absorb adequate enteral nutrients secondary to insufficient intestinal length or function. PN contains the macronutrient building blocks of the human diet in their most elemental forms (amino acids and dextrose) and is commonly administered with a lipid emulsion to avoid essential fatty acid deficiency and to provide a calorically dense source of non-protein calories. In addition, PN contains the essential micronutrients (electrolytes, trace elements, and vitamins) to provide an optimal nutritional regimen. Before the development of PN in the late 1960 s, patients with insufficient gastrointestinal absorptive function commonly died of starvation and subsequent complications of malnutrition (1, 2). Today, more than 30,000 patients are permanently dependent on parenteral nutrition for survival. However, PN continues to be associated with hepatic injury that occurs at an unpredictable rate and includes both biochemical, i.e., elevated serum aminotransferase and alkaline phosphatase, and histologic alterations such as steatosis, steatohepatitis, lipidosis, cholestasis, fibrosis, and cirrhosis (3, 4). These abnormalities, which may worsen with the duration of PN administration, is more prevalent in the pediatric population. Additional risk factors for this condition include prematurity, low birth weight, long-term use of PN, the lack of concomitant enteral intake, sepsis, and multiple operative procedures (5).