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Pulmonary arterial hypertension and type-I glycogen-storage disease: the serotonin hypothesis

¹ Unité Propre de Recherche de l'Enseignement Supérieur (UPRES) 2705, Service de Pneumologie, Centre des Maladies Vasculaires Pulmonaires and ² UPRES 2704, Service de Pédiatrie, Hôpital Antoine Béclère, Université Paris Sud, Clamart, ⁴ Centre Hépato-Biliaire, Hôpital Paul Brousse, Villejuif and ⁶ Service d'Immuno-hématologie and ³ Service de Biochimie, Hôpital Lariboisière, Assistance Publique, Hôpitaux de Paris, Paris, France. ⁵ Division of Medical Genetics, Depts of Medicine and Genetics, University of Leicester, Leicester, UK

CORRESPONDENCE: M. Humbert, Service de Pneumologie, Centre des Maladies Vasculaires Pulmonaires, Hôpital Antoine Béclère, 157 rue de la Porte de Trivaux, 92140, Clamart, France. Fax: 33 146303824. E-mail: humbert@ipsc.u-psud.fr

Keywords: pulmonary hypertension, serotonin, type-I glycogen-storage disease

Received: July 6, 2001
Accepted January 7, 2002

This study was supported by grants from Universite Paris-Sud, Institut National de la Santé et de la Médicale (INSERM) and Association Francaise contre les Myopathies (AFM).

A case of pulmonary arterial hypertension in a patient with type-Ia glycogen-storage disease, a rare autosomal recessive disorder caused by a deficiency of glucose-6-phosphatase is reported in this study. It has been suggested that the occurrence of pulmonary arterial hypertension in type-Ia glycogen-storage disease could be due to an abnormal production of vasoconstrictive amines such as serotonin.

To test this hypothesis, plasma serotonin concentrations were prospectively measured in 13 patients with type-Ia glycogen-storage disease, one patient with severe pulmonary hypertension and type-Ia glycogen-storage disease, 16 patients displaying severe pulmonary arterial hypertension, and 26 normal healthy controls.

Elevated plasma serotonin concentrations were found in patients with either severe pulmonary arterial hypertension (38.8±7.3 nmol·L^–1) or type-Ia glycogen-storage disease (36.8±11.5 nmol·L^–1), as compared with controls (8.8±0.6 nmol·L^–1, p<0.001). Plasma serotonin was dramatically elevated in the patient with type-Ia glycogen-storage disease and pulmonary arterial hypertension (113.4 nmol·L^–1).

It is concluded that type-Ia glycogen-storage disease may be another condition in which abnormal handling of serotonin is one event in a multistep process leading to severe pulmonary arterial hypertension.

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