Menni F, de Lonlay P, Sevin C, Touati G, Peigné C, Barbier V, Nihoul-Fékété C, Saudubray J M, Robert J J
Fédération de Pédiatrie, Diabète-Métabolisme-Neurologie, Centre Hospitalier Universitaire Necker-Enfants Malades, Paris, France.
Pediatrics. 2001 Mar;107(3):476-9. doi: 10.1542/peds.107.3.476.
To evaluate the neurologic outcomes of neonates and infants suffering from persistent hyperinsulinemic hypoglycemia of infancy (PHHI).
The neurologic development of 90 PHHI patients was studied retrospectively. Sixty-three patients were treated surgically and 27 were treated medically. Fifty-four patients were neonates, of whom 8 were treated medically and 46 were operated on (19 for a focal adenomatous hyperplasia and 27 for diffuse hyperinsulinism). Thirty-six patients had infancy-onset hyperinsulinism, of whom 19 were treated medically and 17 underwent pancreatectomy (10 patients for a focal adenomatous hyperplasia and 7 for diffuse hyperinsulinism).
Severe psychomotor retardation was found in 7 patients, 6 with neonatal-onset PHHI. Intermediate psychomotor disability existed in 12 patients; epilepsy existed in 16. Neonatal-onset was the main risk factor for severe retardation or epilepsy. Medically treated patients were less severely affected than those treated by surgery, and there was no difference between the diffuse and focal forms of hyperinsulinism.
Neonatal hyperinsulinemic hypoglycemia is still a severe disease with an important risk to rapidly develop severe mental retardation and epilepsy.
评估患有持续性婴儿高胰岛素血症性低血糖症（PHHI）的新生儿和婴儿的神经学预后。
对90例PHHI患者的神经发育进行回顾性研究。63例患者接受手术治疗，27例接受药物治疗。54例患者为新生儿，其中8例接受药物治疗，46例接受手术（19例为局灶性腺瘤样增生，27例为弥漫性高胰岛素血症）。36例患者为婴儿期起病的高胰岛素血症，其中19例接受药物治疗，17例接受胰腺切除术（10例为局灶性腺瘤样增生，7例为弥漫性高胰岛素血症）。
7例患者出现严重精神运动发育迟缓，6例为新生儿期起病的PHHI。12例患者存在中度精神运动障碍；16例患者患有癫痫。新生儿期起病是严重发育迟缓或癫痫的主要危险因素。接受药物治疗的患者受影响程度低于接受手术治疗的患者，弥漫性和局灶性高胰岛素血症形式之间无差异。
新生儿高胰岛素血症性低血糖症仍是一种严重疾病，有迅速发展为严重智力发育迟缓及癫痫的重要风险。
