Glucose-6-Phosphate Dehydrogenase Deficiency and Neonatal Hyperbilirubinemia in Chinese

葡萄糖六磷酸脫氫酶缺乏與中國新生兒黃疸

陳森輝(Shen-Hui Chen) ； 林國信(Kuo-Sin Lin) ； 李鎡堯(Tzu-Yao Lee) ； 陳烱霖(Chiung-Lin Chen)

《Acta Paediatrica Sinica》 27卷4期 (1986/08) Pp. 286-293

摘要

為了解新生兒紅血球G-6-PD缺乏症之發生率及其影響黃疸發生之關係。本文針對在臺大醫院出生之1,675位男嬰及1,596位女嬰中用Methemoglobin reduction法作篩檢，再以Methemoglobin elution法和酶定量法確認。結果1,675位男嬰中之51位(3.05%)為G-6-PD缺乏半合子，1,596位女嬰中58例(3.63%)及2(0.13%)各為G-6-PD缺乏異合子和純合子。45位未有母子血型不合或其他引起黃疸因素之G-6-PD缺乏嬰見中13位(28.9%)發生高膽紅素血症，其黃疸之發生率顯然比正常嬰兒(7.6%)及G-6-PD缺乏異合子(11.3%)為高。G-6-PD缺乏異合子與正常嬰兒之高膽紅素血症發生情形並無統計學上之差異。1979至1983年在臺大醫院出生之13,768位嬰兒中，66例發生厲害黃疸以致需要換血，66例中之6例(9.1%)乃由G-6-PD缺乏所致。由本文可見G-6-PD缺乏能引發國人新生兒黃疸，在新生兒期有必要做G-6-PD缺乏症之篩檢及注意黃疸產生之情形。

並列摘要

Three thousand two hundred and seventy-one infants, including 1675 males and 1596 females, were screened for Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency: 3.05% of males, 3.63% and 0.13% of females were hemizygous, heterozygous and homozygous for G-6-PD deficiency, respectively. G-6-PD deficiency is associated with neonatal hyperbilirubinemia. Thirteen of 45 (28.9%) G-6-PD deficient infants, free of isoimmunization and other possible cause of jaundice, developed hyperbilirubinemia. Six of 66 (9.1%) severely icteric, hospitalborn infants who received exchange transfusion were G-6-PD deficient. The incidence of jaundice in females heterozygous for G-6-PD deficiency was not significantly different from normal infants. As G-6-PD deficiency is associated with greater risk of severe jaundice, screening of G-6-PD deficiency, close surveillance and discharge of infants only with careful instructions for care after the jaundice subsides are recommended.

並列關鍵字

G-6-PD deficiency ； phenotype ； hyperbilirubinemia ； newborn