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

Kris A. Rooney, Neal J. Thomas

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Objective: To evaluate pulmonary hypertension associated with acute motor sensory axonal neuropathy subtype of Guillain- Barre syndrome. Guillain-Barre syndrome consists of a group of autoimmune disorders that generally manifest as symmetric, progressive, ascending paralysis. There are five subtypes of Guillain- Barre syndrome, and autonomic involvement has been described in all subtypes, including cardiovascular, vasomotor, or pseudomotor dysfunction of both the sympathetic and parasympathetic systems. Design: Case report. Setting: Tertiary care pediatric intensive care unit. Patient: Three-yr-old female patient. Intervention: None. Measurements and Main 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. Conclusion: It is possible that many other individuals suffering from severe forms of Guillain-Barre 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-Barre syndrome have a high index of suspicion for pulmonary hypertension and consider echocardiography if there are clinical signs of this potentially fatal process.

Original languageEnglish (US)
JournalPediatric Critical Care Medicine
Volume11
Issue number1
DOIs
StatePublished - Jan 1 2010

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Guillain-Barre Syndrome
Pulmonary Hypertension
Hypoventilation
Pediatric Intensive Care Units
Tertiary Healthcare
Embolism
Critical Illness
Paralysis
Pulmonary Artery
Echocardiography
Thrombosis
Pressure
Lung

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Critical Care and Intensive Care Medicine

Cite this

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title = "Severe pulmonary hypertension associated with the acute motor sensory axonal neuropathy subtype of Guillain-Barre syndrome",
abstract = "Objective: To evaluate pulmonary hypertension associated with acute motor sensory axonal neuropathy subtype of Guillain- Barre syndrome. Guillain-Barre syndrome consists of a group of autoimmune disorders that generally manifest as symmetric, progressive, ascending paralysis. There are five subtypes of Guillain- Barre syndrome, and autonomic involvement has been described in all subtypes, including cardiovascular, vasomotor, or pseudomotor dysfunction of both the sympathetic and parasympathetic systems. Design: Case report. Setting: Tertiary care pediatric intensive care unit. Patient: Three-yr-old female patient. Intervention: None. Measurements and Main 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. Conclusion: It is possible that many other individuals suffering from severe forms of Guillain-Barre 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-Barre syndrome have a high index of suspicion for pulmonary hypertension and consider echocardiography if there are clinical signs of this potentially fatal process.",
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AB - Objective: To evaluate pulmonary hypertension associated with acute motor sensory axonal neuropathy subtype of Guillain- Barre syndrome. Guillain-Barre syndrome consists of a group of autoimmune disorders that generally manifest as symmetric, progressive, ascending paralysis. There are five subtypes of Guillain- Barre syndrome, and autonomic involvement has been described in all subtypes, including cardiovascular, vasomotor, or pseudomotor dysfunction of both the sympathetic and parasympathetic systems. Design: Case report. Setting: Tertiary care pediatric intensive care unit. Patient: Three-yr-old female patient. Intervention: None. Measurements and Main 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. Conclusion: It is possible that many other individuals suffering from severe forms of Guillain-Barre 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-Barre syndrome have a high index of suspicion for pulmonary hypertension and consider echocardiography if there are clinical signs of this potentially fatal process.

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