Patient: 3 years old boy
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Source: Canada, 1978
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| Presenting features: |
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Child was healthy and thriving, requesting for thorough health check.
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| When examined: |
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The 3-year-old boy was in excellent health with no abnormal features. |
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In view of the severe anemia and jaundice before death occurring in his
elder brother, hemolytic condition induced by infection and precipitated
by drugs was suspected in this family.
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| Relevant Investigations: |
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Both the boy and his mother were found to have G6PD deficiency.
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| Background: |
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Elder brother of 5 just passed away following a short illness, with "acute
urinary tract infection" for which he was treated with septrin and
aspirin. By third day, he developed rapidly progressive jaundice and became
increasingly drowsy and lethargic. He was rushed to the A&E department
and collapsed within 10 minutes of arrival. Child was noted also to be markedly
pale necessitating transfusion with un-cross-matched blood. He died quickly
within the hour of arrival, with tentative diagnosis of "Reye Syndrome". |
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Reports on the 5 year old brother were requested which failed to reveal
brain pathology typical of Reye Syndrome, but he showed Hb 3 gm/dl and no
external blood loss. G6PD status was NOT checked. |
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( On retrospect, he was most likely G6PD deficient, since both his mother
and brother were G6PD deficient. )
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| Lessons: |
| 1) |
G6PD deficiency is highly prevalent
among southern Chinese ( 4.42% in boys; 0.45% homozygous and 8.4% heterozygous
in girls ) |
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2)
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Acute hemolytic jaundice occurs readily
in certain acute infections, and provoked also by some drugs ( sulfa and
aspirin as in this case ) and several herbs. |