Alday L E, Moreyra E
Am Heart J. 1984 Oct;108(4 Pt 1):996-1000. doi: 10.1016/0002-8703(84)90466-6.
In 12 infants and children with hypertrophic cardiomyopathy (HCM), seen during a 5-year period, the incidence of the idiopathic or primary form was similar to that secondary to systemic disease. Five of the six patients with secondary HCM are reported. Pompe's disease, Friedreich's ataxia, and Noonan's syndrome were seen in one each. There were two patients with lentiginosis. The diagnosis of HCM and of the metabolic disorder was made simultaneously in the patient with Pompe's disease. In two patients, one with Friedreich's ataxia and the other with lentiginosis, the diagnosis of HCM preceded the manifestations of the associated disorder. The other two patients were referred to rule out heart disease. The diagnosis of HCM in infancy and childhood should be considered with the possibility of being a secondary form, and patients with known associations with HCM should be referred for early diagnosis of heart conditions.
在5年期间诊治的12例婴幼儿肥厚型心肌病(HCM)患者中,特发性或原发性HCM的发病率与继发于全身性疾病的发病率相似。报告了6例继发性HCM患者中的5例。庞贝病、弗里德赖希共济失调和努南综合征各有1例。有2例雀斑样痣综合征患者。庞贝病患者同时诊断出HCM和代谢紊乱。在2例患者中,1例患有弗里德赖希共济失调,另1例患有雀斑样痣综合征,HCM的诊断先于相关疾病的表现出现。另外2例患者因排除心脏病前来就诊。婴幼儿期HCM的诊断应考虑其为继发性的可能性,已知与HCM有关联的患者应转诊以尽早诊断心脏疾病。