新生儿高胰岛素血症
Hyperinsulinism in the Neonate.
作者信息
Lord Katherine, De León Diva D
机构信息
The Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia, 3401 Civic Center Boulevard, Philadelphia, PA 19104, USA; Department of Pediatrics, The Perelman School of Medicine, University of Pennsylvania, 3401 Civic Center Boulevard, Philadelphia, PA 19104, USA.
The Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia, 3401 Civic Center Boulevard, Philadelphia, PA 19104, USA; Department of Pediatrics, The Perelman School of Medicine, University of Pennsylvania, 3401 Civic Center Boulevard, Philadelphia, PA 19104, USA.
出版信息
Clin Perinatol. 2018 Mar;45(1):61-74. doi: 10.1016/j.clp.2017.10.007. Epub 2017 Dec 6.
Hyperinsulinism (HI) is the leading cause of persistent hypoglycemia in infants. Prompt recognition and treatment, independent of whether infants have transient or permanent HI, are essential to decrease risk of neurologic damage. The most common form of congenital HI is due to inactivating mutations of the β-cell ATP-sensitive potassium (K) channel (K-HI) and is typically diazoxide unresponsive. K-HI occurs in diffuse and focal forms. Distinguishing between the 2 forms is crucial, because pancreatectomy is curative in the focal form but palliative in the diffuse form. The 18-fluoro-L-3,4-dihydroxyphenylalanine PET scan has revolutionized HI management by allowing accurate localization of focal lesions prior to surgery.
高胰岛素血症（HI）是婴儿持续性低血糖的主要原因。无论婴儿患的是暂时性还是永久性HI，迅速识别并进行治疗对于降低神经损伤风险至关重要。先天性HI最常见的形式是由于β细胞ATP敏感性钾（K）通道（K-HI）的失活突变引起，通常对二氮嗪无反应。K-HI有弥漫性和局灶性两种形式。区分这两种形式至关重要，因为胰腺切除术对局限性形式具有治愈性，但对弥漫性形式仅起姑息作用。18氟-L-3,4-二羟基苯丙氨酸PET扫描通过在手术前能够精确定位局灶性病变，彻底改变了HI的治疗方式。
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