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November 1966

Gout and Glycogen Storage Disease in Preadolescent Brothers

Arch Intern Med. 1966;118(5):471-477. doi:10.1001/archinte.1966.00290170059011

GOUT in children is a rare disease and provides a challenge to demonstrate more clearly the basic cause of gout. The isolated case reports which appear in the literature, however, only underline its rarity and do not contribute significantly to a better understanding of the disease. More recently, reports have been published of hyperuricemia and gout appearing in one adolescent and several adults with glycogen storage disease of the von Gierke or glucose-6-phosphatase deficient type often called type I1-8 and hereafter referred to as glycogenosis. Even if a study of these cases does not clarify the cause of primary gout it still illuminates some of the several factors promoting hyperuricemia and may point to the basic mechanism responsible for the development of gout in these patients.

The following is a report of gout occurring in two preadolescent male siblings with glycogenosis.

Report of Cases 

Case 1.  —Patient (Fig 1)