[1例严重营养不良伴染色体畸变患者的多器官功能衰竭]。

Anestezjologia intensywna terapia Pub Date : 2010-07-01
Roman Kaźmirczuk, Marlena Jakubczyk, Sylwia Kołtan, Piotr Brzeziński, Zbigniew Szkulmowski, Kinga Kupczyk, Piotr Skiba, Małgorzata Apanasiewicz, Mariusz Mielniczuk, Marcin Owczarek, Mieczysława Czerwionka-Szaflarska, Mariusz Wysocki, Krzysztof Kusza
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引用次数: 0

摘要

背景:根据世界卫生组织的定义,肺炎和营养不良是儿童的两个最大杀手。虽然这些情况在发展中国家很常见,但在较发达的国家也可以观察到,这是由于对残疾儿童的疏忽和缺乏适当照顾造成的。我们描述了一个严重营养不良导致多器官衰竭的病例。病例报告:一名16岁+14q染色体畸变的智障女孩,因严重贫血和呼吸困难入院。她极度营养不良。体重32公斤,身高152厘米(BMI 13.9)。血红蛋白1.12 mmol L(-1),血红蛋白7%,红细胞0.93 mmol L(-1)。输血导致输血相关性急性肺损伤(TRALI)和多器官衰竭。患者接受机械通气、肌力支持和肠外营养治疗,并发再进食综合征和胃肠道出血。恢复后,进行胃造口术,但由于胃潴留,她需要剖腹手术进行粘连松解。这名女孩已经康复,目前仍在接受家庭护理。讨论:在一个迟滞女孩的情况下,观察到多器官功能衰竭导致十年的营养不良。由于长期的体内平衡功能障碍、低蛋白血症、低磷血症和SIRS,她尤其难以治疗。这类患者需要在ICU环境中仔细治疗。
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[Multiple organ failure in a severely malnourished patient with chromosome aberration].

Background: Pneumonia and malnutrition are two of the biggest killers in childhood, as defined by the World Health Organisation. Although common in the developing world, these conditions can also be observed in more advanced countries, as a result of negligence and lack of proper care in disabled children. We describe a case in which severe malnutrition resulted in multiple organ failure.

Case report: A 16-yr-old retarded girl with +14q chromosome aberration, was admitted to hospital because of severe anaemia and dyspnea. She was extremely malnourished. Her body weight was 32 kg with a height of 152 cm (BMI 13.9). Her Hb concentration was 1.12 mmol L(-1), Ht 7%, and RBC 0.93 T L(-1). RBC transfusion resulted in transfusion-related acute lung injury (TRALI) and multiple organ failure. She was treated with mechanical ventilation, inotropic support and parenteral nutrition, complicated by the refeeding syndrome and gastrointestinal haemorrhage. After recovery, a gastrostomy was performed, but due to gastric retention she required a laparotomy for adhesiolysis.The girl recovered and remains under home care.

Discussion: In a case of a girl with retardation, multiple organ failure resulting from ten years of malnutrition was observed. She was especially difficult to treat because of a prolonged dysfunction of homeostasis, hypoproteinemia, hypophosphatemia and SIRS. Such patients require careful treatment in ICU settings.

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