MEDICAL INSIDER
DR. RIA MASLOG
FOR AUG 27, 2023-08-25
Glucose-6-Phosphate Dehydrogenase (G-6-PD)
Deficiency
This condition is part of the newborn screening done on
newborn babies who are 24 hours old.
And, G-6-PD deficiency is the most important disease of the
pentose phosphate pathway and is responsible for two
clinical periods. These two clinical periods are:
- Episodic hemolytic anemia
- Spontaneous chronic nonspherocytic hemolytic anemia
Clinically, those children with G-6-PD deficiency will develop
symptoms 24-48 hours after ingestion of a substance with
oxidant properties like:
- Sulfonamides
- Trimethoprine
- sulfamethoxazole
- nalidixic acid
- Chloramphenicol
- antimalarial medications
- Vit K analogs
- Methylene blue
- Acetylsalicyslic acid
- fava beans
The onset of acute hemolysis results in the drop of the
hemoglobin and hematocrit levels.
In severe cases, the hemoglobin binding proteins are
saturated and free hemoglobin may appear in the plasma
and the urine.
Affected individuals may also have infections as a result of
hemolysis, hyperbilirubinemia and potential kernicterus.
The availability of the Newborn Screening is really a
breakthrough because we can detect the condition on a very
early stage of affected babies. Similarly, parents and
physicians will also know what to avoid and what medicines
are not to be given to avoid hemolysis in G-6-PD deficienct
children.