Glycogen storage disease type I (GSD-I) is a group of autosomal recessive disorders with an incidence of 1 in 100,000. The two major subtypes are GSD-Ia, caused by a deficiency of glucose-6-phosphatase (G6Pase), and GSD-Ib, caused by a deficiency of glucose-6-phosphate transporter (G6PT). We report that a substantial improvement was achieved following several infusions of hepatocytes in a patient with GSD-Ib. Hepatocytes were isolated from the unused cadaveric whole livers of two donors. At the first transplantation, approximately 2 x 10(9) cells (2% of the estimated recipient's total hepatocytes) were infused. Seven days later 1 x 10(9) (1% of liver mass) cryopreserved hepatocytes from the same donor were infused, and an additional 3 x 10(9) (3% of liver mass) cells from the second donor were infused 1 month after the second transplantation. After the hepatocyte transplantation, the patient showed no hypoglycemic symptoms despite the discontinuation of cornstarch meals. Liver biopsies on posttransplantation days 20 and 250 showed a normal level of glucose-6-phosphatase activity in presolubilization assay that was very low before transplantation. This was the first and successful clinical hepatocyte transplantation in Korea. In this study, hepatocyte transplantation allowed a normal diet in a patient with GSD-Ib, with substantial improvement in their quality of life. Hepatocyte transplantation might be an alternative to liver transplantation and dietary therapy in GSD-Ib.

This study was aimed to evaluate the prevalence of soy protein hypersensitivity in cow's milk protein-sensitive children in Korea. A total of 1,363 patients with atopic dermatitis, urticaria, enterocolitis syndrome, bronchial asthma or allergic rhinitis were recruited. First, we estimated the prevalence of sensitization to soy in children sensitized to cow's milk. Specific IgE levels > 0.7 kU/L by CAP assay were considered positive. Next, the prevalence of soy allergy in cow's milk allergy (CMA) patients was investigated. Those children whose parents agreed to participate the open challenge test with soy had a convincing history of allergic reactions elicited by cow's milk and these symptoms were relieved by elimination. All of them had negative soy-specific IgE. Patients with positive soy-specific IgE accounted for 18.3% of 224 children sensitized to cow's milk protein. The prevalence of sensitization to soy decreased with age (36.8% in the first year of life, 16.4% in the second year, and 13.7% in the third year). Of 21 CMA patients, 42.9% (n=9) were determined to have soy allergy (mean age 10.3 months). Our results suggest that soy protein formula should be carefully used as a substitute for cow's milk in CMA patients, especially during infancy.

Cherry angiomas are a common cutaneous vascular proliferation which manifests as single or multiple spots and occurs predominantly on the upper trunk and arms. They typically appear as round-to-oval, bright, red, dome-shaped papules and pinpoint macules measuring up to several millimeters in diameter. The histopathologic findings of a cherry angioma are consistent with a true capillary hemangioma, which is formed by numerous, newly developed capillaries with narrow lumens and prominent endothelial cells arranged in a lobular fashion in the papillary dermis. Herein, we report a case of multiple cherry angiomas on the scalp, an uncommon location for cherry angiomas.