Bembi B, Cerini E, Danesino C, Donati M A, Gasperini S, Morandi L, Musumeci O, Parenti G, Ravaglia S, Seidita F, Toscano A, Vianello A
Regional Coordination Centre for Rare Diseases, University Hospital Santa Maria della Misericordia of Udine, Udine, Italy.
Neurology. 2008 Dec 2;71(23 Suppl 2):S12-36. doi: 10.1212/WNL.0b013e31818da93f.
Glycogenosis type II is a multisystem disorder that requires management by a multidisciplinary team. The team should include several specialists, such as a metabolic disease specialist or biochemical geneticist, cardiologist, pulmonologist, neurologist, neuromuscular specialist, intensivist, orthopedist, respiratory therapist, physical therapist, occupational therapist, otolaryngologist speech therapist, audiologist, genetic counselor, and a metabolic dietician, who, as a team, will be capable of addressing the different manifestations of the condition. Aspects of functional assessment, rehabilitation, nutritional management, care coordination, nursing, genetic counseling, prenatal diagnosis, and screening are discussed in this article. In addition, treatment of glycogenosis type II is reviewed with attention to emerging therapeutic options.
II型糖原贮积病是一种多系统疾病,需要多学科团队进行管理。该团队应包括几位专家,如代谢疾病专家或生化遗传学家、心脏病专家、肺病专家、神经科医生、神经肌肉专家、重症监护专家、骨科医生、呼吸治疗师、物理治疗师、职业治疗师、耳鼻喉科言语治疗师、听力学家、遗传咨询师和代谢营养师,作为一个团队,他们将有能力应对该疾病的不同表现。本文讨论了功能评估、康复、营养管理、护理协调、护理、遗传咨询、产前诊断和筛查等方面。此外,还对II型糖原贮积病的治疗进行了综述,并关注新兴的治疗选择。