1970Archives of Disease in ChildhoodOpen access

A Disorder of Tryptophan Metabolism in Chronic Granulomatous Disease

A. F. Heeley, M E Heeley, John Hardy, J F Soothill

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Abstract

5 children with chronic granulomatous disease had a disorder of tryptophan metabolism, resulting in excretion of gross excess of hydroxykynurenine, kynurenine, and xanthurenic acid, with or without tryptophan loading. Hydroxykynurenine predominated, and in this and other ways the findings were different from those present in most defects of tryptophan metabolism, but were similar to those in pyridoxine deficiency. Oral pyridoxine 30 mg./day corrected this defect, but had no effect on the nitroblue tetrazolium reaction, or bacterial killing by polymorphonuclear leucocytes.

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5 children with chronic granulomatous disease had a disorder of tryptophan metabolism, resulting in excretion of gross excess of hydroxykynurenine, kynurenine, and xanthurenic acid, with or without tryptophan loading. Hydroxykynurenine predominated, and in this and other ways the findings were different from those present in most defects of tryptophan metabolism, but were similar to those in pyridoxine deficiency. Oral pyridoxine 30 mg./day corrected this defect, but had no effect on the nitroblue tetrazolium reaction, or bacterial killing by polymorphonuclear leucocytes.

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Available abstract

5 children with chronic granulomatous disease had a disorder of tryptophan metabolism, resulting in excretion of gross excess of hydroxykynurenine, kynurenine, and xanthurenic acid, with or without tryptophan loading. Hydroxykynurenine predominated, and in this and other ways the findings were different from those present in most defects of tryptophan metabolism, but were similar to those in pyridoxine deficiency. Oral pyridoxine 30 mg./day corrected this defect, but had no effect on the nitroblue tetrazolium reaction, or bacterial killing by polymorphonuclear leucocytes.

Key concepts: Xanthurenic acid, Tryptophan, Kynurenine, Pyridoxine, Tryptophan Metabolism, Chronic granulomatous disease, Medicine, Excretion

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