Bennett Erin L
University of Pennsylvania, Philadelphia, PA, USA.
J Pediatr Oncol Nurs. 2011 Mar-Apr;28(2):67-74. doi: 10.1177/1043454210382421. Epub 2010 Nov 22.
Sickle-cell disease is the most prevalent genetic hematologic condition in the United States. Numerous studies have demonstrated poor growth and delayed maturation in children with homozygous sickle-cell disease; however, the pathophysiology remains inadequately understood. Affected children have normal weight and length at birth, and then around 6 months of age their growth patterns begin to diverge from the norm. The growth deficits experienced by these children remain a problem with clinical significance and intangible consequences. A review of literature has provided insight into the multifactorial basis of the growth failure experienced by this population. It is important that nurses and health care providers are familiar with the growth patterns unique to sickle-cell disease and recognize their role in clinical practice.
镰状细胞病是美国最常见的遗传性血液疾病。众多研究表明，患有纯合子镰状细胞病的儿童生长发育不良且成熟延迟；然而，其病理生理学仍未得到充分理解。患病儿童出生时体重和身长正常，然后在大约6个月大时，他们的生长模式开始偏离正常水平。这些儿童所经历的生长缺陷仍然是一个具有临床意义和无形后果的问题。文献综述为该人群生长发育失败的多因素基础提供了见解。护士和医疗保健提供者熟悉镰状细胞病特有的生长模式并认识到他们在临床实践中的作用非常重要。
