Hamaoka K, Nakagawa M, Furukawa N, Sawada T
Division of Pediatric Medicine, Children's Research Hospital, Kyoto, Japan.
Pediatr Cardiol. 1990 Jan;11(1):54-6. doi: 10.1007/BF02239550.
Two cases of pulmonary hypertension associated with type I glycogen storage disease (type I GSD) are reported. Before the development of pulmonary hypertension, patient 1 had been treated with dietary therapy with nocturnal gastric-drip infusion and zyloric therapy. Patient 2 had received a shunt operation between the intestinal vein and inferior vena cava, as well as dietary therapy. Both patients died of progressive heart failure due to pulmonary hypertension despite many attempts at drug therapy. There was no evidence in either case of a disorder that could have been the cause of the pulmonary hypertension. In case 1, the autopsy revealed a vasoconstrictive type of pulmonary hypertension with plexiform vascular lesions.
报告了两例与I型糖原贮积病（I型GSD）相关的肺动脉高压病例。在肺动脉高压出现之前，患者1接受了夜间胃内滴注饮食疗法和痛风利仙治疗。患者2接受了肠静脉与下腔静脉分流手术以及饮食疗法。尽管进行了多次药物治疗尝试，但两名患者均死于肺动脉高压导致的进行性心力衰竭。两例均无证据表明存在可能是肺动脉高压病因的疾病。在病例1中，尸检显示为伴有丛状血管病变的血管收缩型肺动脉高压。
