Research progress of Hutchinson-Gilford progeria syndrome / 中华实用儿科临床杂志

ABSTRACT

Hutchinson-Gilford progeria syndrome (HGPS) is a rare disease mainly characterized by accele-rated aging, with an incidence rate of 1 in 8 million to 1 in 4 million.It can affect the skin, fat, cardiovascular, bone and other organ systems.Most HGPS children can only live to 6-20 years old, with an average life expectancy of only 14.6 years.HGPS has distinctive clinical features, such as severe growth retardation, special skin manifestations, and craniofacial manifestations.The prognosis of this disease is poor, and no treatment has been proven effective so far.Upon the diagnosis, the progress of the disease should be observed and monitored via long-term and careful follow up, so as to extend the life span of the children as much as possible.In this article, the disease type, clinical manifestations, pathogenesis and clinical examination of HGPS were reviewed.