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Jia-Woei Hou, MD; Tso-Ren Wang, MD; Walter W. Tunnessen, Jr, MD

Arch Pediatr Adolesc Med. 1996;150(2):219-220.

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

A 14-YEAR-OLD BOY with long-standing growth retardation (Figure 1) developed arthritis and the presence of firm, irregular, subcutaneous nodules over the dorsum of his right hand (Figure 2). He has a history of sweating and rapid respirations, particularly before meals. On physical examination his face appeared full, with prominent cheeks (Figure 3). His liver was palpable 12 cm below the right costal margin. The skin over his buttocks and posterior thighs had diffuse, firm, yellowish papules and nodules, some of which appeared excoriated and secondarily infected (Figure 4).

Laboratory studies revealed the following abnormal results: aspartate aminotransferase, 677 U/L; alanine aminotransferase, 434 U/L; triglycerides, 8.77 mmol/L (777 mg/dL); total cholesterol, 5.04 mmol/L (195 mg/dL); uric acid, 916 µmol/L (15.4 mg/dL); glucose, 2.2 mmol/L (40 mg/dL); and lactate, 5.0 mmol/L. A prolonged bleeding time, prothrombin time, and partial thromboplastin time were also present.

Denouement and Discussion

Glycogen Storage Disease Type la . . . [Full Text PDF of this Article]

Author Affiliations
From the Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan (Drs Hou and Wang), and the American Board of Pediatrics, Chapel Hill, NC (Dr Tunnessen).

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