Severe pulmonary hypertension associated with the acute motor sensory axonal neuropathy subtype of Guillain-Barré syndrome.
OBJECTIVE
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.


METHODS
Case report.


METHODS
Tertiary care pediatric intensive care unit.


METHODS
Three-yr-old female patient.


METHODS
None.


RESULTS
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.


CONCLUSIONS
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.
Rooney
Kris A
KA
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.
Thomas
Neal J
NJ
D002675:Child, Preschool; D005260:Female; D020275:Guillain-Barre Syndrome / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D015278:Intensive Care Units, Pediatric; D010523:Peripheral Nervous System Diseases / Q000503:physiopathology*; D012720:Severity of Illness Index