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  Vol. 152 No. 7, July 1998 TABLE OF CONTENTS
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Picture of the Month

Masayuki Sasaki, MD; Hiroshi Aikoh, MD; Kenji Sugai, MD; Hiroshi Yoshida, MD; Walter W. Tunnessen, Jr, MD
From the Department of Child Neurology, National Center hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry (Drs Sasaki, Aikoh, and Sugai) and Department of Pediatrics, Shonai Hospital (Dr Yoshida), Tokyo, Japan; and the American Board of Pediatrics, Chapel Hill, NC (Dr Tunnessen).

Arch Pediatr Adolesc Med. 1998;152:707-708.

Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

A 13-MONTH-OLD infant with methylmalonic acidemia presented with an erythematous, desquamating rash that had begun in the diaper area and progressively spread to involve parts of the face, extremities, and trunk (Figure 1 and Figure 2). As part of his treatment for methylmalonic acidemia, dietary protein was restricted (1.2 g/kg per day). Before the rash began, he had gastroenteritis and was not able to tolerate this diet for 3 days.


Figure 1.


Figure 2.


Denouement and Discussion: Cutaneous Lesions Associated With Isoleucine Deficiency

Figure 1 and Figure 2. The rash is erythematous, desquamating, and most prominent in the diaper area where it began. The perioral area is spared in this infant.

Methylmalonic aciduria (MMA) is a disorder of branched-chain amino acid metabolism in which methylmalonic acid accumulates in body fluids. Infants with this disorder usually present in the first . . . [Full Text of this Article]







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