Platelet-derived growth factor inhibition--a new treatment of pulmonary hypertension in congenital diaphragmatic hernia?

J Pediatr Surg. 2008 Oct;43(10):1928-31. doi: 10.1016/j.jpedsurg.2008.07.022.

Abstract

Increased pulmonary vascular resistance causing pulmonary artery hypertension is a major problem in the treatment of congenital diaphragmatic hernia with a strong association to mortality. We here report a patient with intractable pulmonary hypertension at 4 weeks of age unresponsive to conventional treatment. After administration of the platelet-derived growth factor (PDGF) receptor antagonist imatinib, pulmonary artery pressure gradually decreased to acceptable levels and the patient's clinical condition gradually improved.

Publication types

  • Case Reports

MeSH terms

  • Benzamides
  • Bosentan
  • Combined Modality Therapy
  • Continuous Positive Airway Pressure
  • Diuretics / therapeutic use
  • Enteral Nutrition
  • Extracorporeal Membrane Oxygenation
  • Heart Failure / etiology
  • Hernia, Diaphragmatic / complications*
  • Hernia, Diaphragmatic / surgery
  • Hernias, Diaphragmatic, Congenital
  • Humans
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / etiology
  • Hypoxia / drug therapy
  • Hypoxia / etiology
  • Hypoxia / therapy
  • Iloprost / therapeutic use
  • Imatinib Mesylate
  • Infant, Newborn
  • Male
  • Nitric Oxide / therapeutic use
  • Piperazines / therapeutic use*
  • Protein Kinase Inhibitors / therapeutic use*
  • Purines / therapeutic use
  • Pyrimidines / therapeutic use*
  • Receptors, Platelet-Derived Growth Factor / antagonists & inhibitors*
  • Sildenafil Citrate
  • Sulfonamides / therapeutic use
  • Sulfones / therapeutic use

Substances

  • Benzamides
  • Diuretics
  • Piperazines
  • Protein Kinase Inhibitors
  • Purines
  • Pyrimidines
  • Sulfonamides
  • Sulfones
  • Nitric Oxide
  • Imatinib Mesylate
  • Sildenafil Citrate
  • Receptors, Platelet-Derived Growth Factor
  • Iloprost
  • Bosentan