Govaerts L, Monnens L, Melis T, Trijbels F
Eur J Pediatr. 1984 Nov;143(1):10-2. doi: 10.1007/BF00442740.
An ACTH stimulation test was performed in six patients suffering from the cerebro-hepato-renal syndrome of Zellweger. In contrast to controls, no rise in cortisol was observed. None of these patients showed clinical symptoms of adrenal insufficiency. The sudden death, which occurs in this syndrome, can probably be explained by an impaired stress reaction. In stress situations, such as respiratory infection, corticosteroids should be administered to these patients. A striking resemblance exists between the Zwellweger syndrome and the neonatal form of adrenoleukodystrophy.
对6例患有泽尔韦格脑肝肾综合征的患者进行了促肾上腺皮质激素（ACTH）刺激试验。与对照组相比，未观察到皮质醇升高。这些患者均未表现出肾上腺功能不全的临床症状。该综合征中发生的猝死可能可以用应激反应受损来解释。在应激情况下，如呼吸道感染，应给这些患者使用皮质类固醇。泽尔韦格综合征与新生儿型肾上腺脑白质营养不良之间存在显著相似之处。
