Voorhoeve P G, van Wijk J L, Delemarre-van de Waal H A
VU Medisch Centrum, afd. Kindergeneeskunde, Postbus 7057, 1007 MB Amsterdam.
Ned Tijdschr Geneeskd. 2004 Jan 17;148(3):125-9.
Persistent hyperinsulinemic hypoglycaemia in infancy (PHHI) presents a diagnostic and therapeutic challenge for the treating physician: increased glucose requirements, detectable insulin levels at the point of hypoglycaemia, inappropriately low blood levels of free fatty acids and ketone bodies are characteristic of this condition. Despite recent developments in understanding its pathophysiology, treatment remains difficult and there are many long-term complications. Adequate treatment strategies are needed to prevent severe neurological damage. As there is a high possibility that hyperinsulinism may only be transient, aggressive pharmacological treatment is necessary for 4 to 6 weeks before moving on to surgical intervention. In the light of recent knowledge, routine subtotal pancreatectomy in children is no longer justifiable. An attempt should first be made to differentiate between focal and diffuse hypersecretion of insulin by using interventional radiology techniques, notably pancreatic venous sampling. This then enables targeted partial pancreatectomy.
婴儿持续性高胰岛素血症性低血糖症(PHHI)给治疗医生带来了诊断和治疗方面的挑战:葡萄糖需求量增加、低血糖时可检测到胰岛素水平、游离脂肪酸和酮体的血液水平异常降低是这种病症的特征。尽管在理解其病理生理学方面有了最新进展,但治疗仍然困难,且存在许多长期并发症。需要有适当的治疗策略来预防严重的神经损伤。由于高胰岛素血症很可能只是短暂的,因此在进行手术干预之前,需要进行4至6周的积极药物治疗。根据最新知识,儿童常规次全胰腺切除术已不再合理。应首先尝试通过介入放射学技术,尤其是胰腺静脉采血,来区分胰岛素的局灶性和弥漫性分泌过多。这样就能进行有针对性的部分胰腺切除术。