RESUMEN
Insulinoma is a rare neuroendocrine tumor that causes hypoglycemia due to unregulated insulin secretion. Blood glucose management during insulinoma resection is therefore challenging. We present a case in which real-time subcutaneous continuous glucose monitoring (SCGM) in combination with intermittent blood glucose measurement was used for glycemic control during surgery for insulinoma resection. The SCGM system showed the trends and peak of interstitial glucose in response to glucose loading and the change of interstitial glucose before and after insulinoma resection. These data were helpful for adjusting the glucose infusion; therefore, we think that an SCGM system as a supportive device for glucose monitoring may be useful for glucose management during surgery.
RESUMEN
Mucolipidosis (ML) II alpha/beta is an autosomal recessive disease caused by reduced enzyme activity of N-acetylglucosamine-1-phosphotransferase. Clinical symptoms of ML II are severe psychomotor delay and dysostosis multiplex; death usually occurs by 5-8 years of age from cardiopulmonary complications. Allogeneic hematopoietic stem cell transplantation (HSCT) has been attempted for ML; however, few reports have documented the detailed outcomes of HSCT for ML. A 26-month-old girl received a human leukocyte antigen 3/6-allele-matched transplant from cord blood. The preparative regimen consisted of fludarabine, cyclophosphamide, 6-Gy total body irradiation, and rabbit antithymocyte globulin. Although comparing before and after cord blood transplantation results, we observed that lysosomal enzyme activities in the plasma decreased by approximately 20-40%. Low serum levels of immunoglobulin A, G2, and G4 were also observed before HSCT; however, these values normalized after transplantation. Despite undergoing HSCT, she was treated twice for bacterial pneumonia with acute respiratory distress syndrome at ages 37 and 38 months. Although HSCT effects on the clinical manifestations were limited, laboratory data including plasma lysosomal enzyme activities and serum levels of immunoglobulin showed improvement.