Management and Controversies in Pediatric Pulmonary Hypertension.
Management and Controversies in Pediatric Pulmonary Hypertension.
复制标题
小儿肺动脉高压的管理和争议。
DOI：
10.1089/pai.2009.2204.rt
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发表时间：
2009
期刊：
影响因子：
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通讯作者：
Farber,HaroldJ
中科院分区：
文献类型：
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作者：
MalloryJr,GeorgeB;Hanna,BrianD;Ivy,DDunbar;Shardonofsky,Felix;Farber,HaroldJ
MALLORY ET AL. 140从症状开始到做出PH诊断可能要经过很长时间，而从告知患者诊断结果到开始治疗又要经过一段时间。我的感觉是，在儿科，我们做得比这更好。GEORGE B. MALLORY：也可以说，在8岁时出现酸碱度症状的儿童，其病程往往比35岁才确诊酸碱度的妇女要快得多。每年至少有一次，我们会接到同事打来的电话，说有一个患有酸碱度晚期的孩子在重症监护室，询问我们的肺移植中心是否可以接受这个孩子进行肺移植。在这种情况下，通常为时已晚，做任何事情，不幸的是，当他们的临床表现最终导致诊断为博士时，儿童的疾病已经非常严重，这并不罕见。我不认为我们有足够的关于儿科患者的数据来确定从症状开始到诊断为ph的时间。从我自己的经验来看，我认为这取决于患者的年龄，高血压的背景和表现症状。通常情况下，病人会出现晕厥，这是一种非常戏剧性的表现，他们会被神经科医生诊断为癫痫发作，而我们无法治疗这个病人。最后病人才会去看心脏病专家或肺科专家。另一方面，也有患者在用力时出现呼吸困难。我记得有一些患者，他们6、7岁，有3、4年不能参加运动或身体表现不佳的病史，最终被诊断为PH。所以我认为从PH的症状出现到诊断之间可能要经过很长一段时间。
MALLORY ET AL. 140 a long time can elapse from the beginning of symptoms to the time that a diagnosis of PH is made, and a further period can then pass from the time that the patient is told of the diagnosis to the beginning of therapy. My feeling is that in pediatrics we do a better job than that. GEORGE B. MALLORY: It could also be argued that the child who becomes symptomatic with PH at age 8 often has a far more accelerated course than the lady whose PH diagnosis is made at the age of 35. At least once a year we get a call from a colleague about a child who is terminally ill with PH and in an intensive care unit asking whether our lung transplant center can accept the child for lung transplantation. In that situation it is usually too late to do anything, and unfortunately it is not rare for children to have very advanced disease by the time their clinical manifestations finally lead to a diagnosis of PH. FELIX SHARDONOFSKY: I don’t think that we have sufficient data about pediatric patients to establish a time from the beginning of symptoms to the diagnosis of PH. From my own experience I think that depends on the age of the patient, the context of the hypertension, and the presenting symptoms. Often, the patient presents with syncope, which is a very dramatic presentation, is referred to a neurologist with a presumption of seizures, and we don’t get to treat that patient. Only eventually is the patient seen by a cardiologist or a pulmonologist.On the other hand, there are patients who have dyspnea on exertion. I can remember instances of patients who were 6 or 7 years old and had a 3-or 4-year history of inability to participate in sports or poor physical performance, and who were ultimately diagnosed as having PH. So I think that a long time can pass between the presentation of symptoms of PH and its diagnosis.