Dietary Management Reverses Grooving And Abnormal Polarization Of Hair Shafts In Argininosuccinase Deficiency

The study observed that two untreated patients with argininosuccinic aciduria exhibited fragile hair with abnormal banding and grooves. These abnormalities were identified using polarizing and scanning electron microscopy. Despite normal amino acid analysis of the hair, the abnormalities were present until the patients were treated with a low-protein, arginine-supplemented diet, which resulted in the hair returning to a normal appearance. Five other patients who were already on the diet showed no hair abnormalities. The study suggested that the disease might produce substances that negatively impact metabolically active tissues like hair.