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Glucosa-6-Phosphate Delydrogenase Deficiency (G6PD)

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Glucosa-6-phosphate Dehydrogenase (G6PD) deficiency is the most common human enzyme deficiency effecting approximately 400 milllons people worldwide, and also the most imprtant disease of the hexose monophaspate pathway G6PD deficiency makes eythrocytes more vulnerable to oxidative stress, such as infections, oxidative drugs or, rarely fava beans; this is why the G6PD deficiency is also know as favism. G6PD deficiiency as an x-chromosome linked inbom emor is most commonly found in males of African, Asian, Mediterranean, or middle Eastem descent. Homozygotes and heterozygotes both can be symptomatic, may be more severe in the homozygotes. The clinical presentations include acute hemodlytic anemia, chronic hemolutic anemia, neonatal hyperbilirubinemia or absence of clinical synptoma. There is an advantage of resistance to Falcoparum malaria inheterozygous females. Basically, the main treatment is avoidance of oxicdative stressors that induces hemolysis. Acute hemolysis is mostly self-limited, but in rate cases it can be so severe that blood transfusion is required. Neonatal hyperbilirubinemia may be trasted with phototherapy or exchange trans fusion to prevent kemicterus.

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Ketersediaan

Informasi Detail

Judul Seri

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No. Panggil

Cermindnia Kedokteran;38(1)2011: 34-36

Penerbit

Jakarta : Kable Farma., 2011

Deskripsi Fisik

2p.

Bahasa

ISBN/ISSN

0125-913X

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Tipe Isi

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Tipe Media

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Tipe Pembawa

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Edisi

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Pernyataan Tanggungjawab

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