Division of Critical Care Medicine, Department of Pediatrics, Penn State Center for Host defense, Inflammation, and Lung Disease, Penn State Children's Hospital and Pennsylvania State University College of Medicine, Hershey, PA, USA.
Pediatr Crit Care Med. 2010 Jan;11(1):e16-9. doi: 10.1097/PCC.0b013e3181b0133d.
To evaluate pulmonary hypertension associated with acute motor sensory axonal neuropathy subtype of Guillain-Barré syndrome. Guillain-Barré syndrome consists of a group of autoimmune disorders that generally manifest as symmetric, progressive, ascending paralysis. There are five subtypes of Guillain-Barré syndrome, and autonomic involvement has been described in all subtypes, including cardiovascular, vasomotor, or pseudomotor dysfunction of both the sympathetic and parasympathetic systems.
Case report.
Tertiary care pediatric intensive care unit.
Three-yr-old female patient.
None.
Serial measurements of pulmonary artery pressure. We report the case of a young girl with acute motor sensory axonal neuropathy who presented with severe cardiovascular collapse secondary to severe pulmonary hypertension. In this patient, multiple factors may have played a role in the development of pulmonary hypertension including autonomic dysfunction, hypoventilation, and immobility as a risk for thrombosis and pulmonary emboli.
It is possible that many other individuals suffering from severe forms of Guillain-Barré syndrome, especially those with significant autonomic dysfunction, may actually have undiagnosed and therefore untreated pulmonary hypertension. Therefore, it is recommended that clinicians caring for critically ill children with Guillain-Barré syndrome have a high index of suspicion for pulmonary hypertension and consider echocardiography if there are clinical signs of this potentially fatal process.
评估吉兰-巴雷综合征急性运动感觉轴索性神经病型伴发的肺动脉高压。吉兰-巴雷综合征是一组自身免疫性疾病，通常表现为对称、进行性、上升性瘫痪。吉兰-巴雷综合征有 5 种亚型，所有亚型均有自主神经受累，包括交感和副交感两个系统的心血管、血管运动或假性运动功能障碍。
病例报告。
三级儿科重症监护病房。
3 岁女性患者。
无。
肺动脉压的系列测量。我们报告了一例急性运动感觉轴索性神经病的年轻女孩病例，她因严重肺动脉高压导致严重心血管衰竭。在该患者中，多种因素可能在肺动脉高压的发展中起作用，包括自主神经功能障碍、通气不足和因活动受限而容易发生血栓形成和肺栓塞。
许多其他患有严重吉兰-巴雷综合征的患者，尤其是那些有明显自主神经功能障碍的患者，可能实际上患有未确诊和未经治疗的肺动脉高压。因此，建议照顾吉兰-巴雷综合征重症儿童的临床医生对肺动脉高压保持高度怀疑，并在有这种潜在致命过程的临床迹象时考虑进行超声心动图检查。
