Aspectos clinicos e laboratoriais da deficiencia de desidrogenase de 6 fosfato de glicose (G-6-PD) em recem-nascidos brasileiros




The etiological association of neonatal jaundice G-6-PD deficiency due to the African variant or A- variant has not been defined in previous works and Brazilian studies done in São Paulo and Bahia with different methodologies showed discordant results. The purpose of this work was to estabilish this association in a prospective study of 697 newborn infants excluding those presenting newborn blood Incompatibility, infections or prematurity. All icteric newborn infants had negative Coombs test. The G-6-PD deficiency investigated in umbilical cord blood by screening tests (methemoglobin reduction test and brilliant cresyl blue dye test) and confirmed by enzymatic activity assay and starch gel electrophoresis. Twenty five newborn infants had G-6-PD deficiency. The enzymopeny frequencies were 9,5% in the negroid boys, 2% in the caucasoid boys and 2,3% in the negroid girls. All of the G-6-PD deficient infants examined had the mild African or A- type of G-6-PD deficiency with exception of one Caucasoid boy who showed an enzyme with electrophoretic pattern highly suggestive of the Mediterranean type. The infants were clinically followed in the nursery for detection of Jaundice. Jaundice was classified as mild or moderate. No infants had severe Jaundice. The incidence of moderate jaundice was significantly higher (p<<<0,00l) in the enzymopenic infants but the same was not observed (0,3


desidrogenação neonatologia hematologia pediatrica

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