case_images: PMC6041848_01
case_images: (a) Patient at diagnosis with cushingoid face and torso and hepatomegaly. (b) Delayed puberty at diagnosis (insulin pump is attached to the abdomen). (c) The boy 1 year after continuous insulin delivery:normal appearance, no hepatomegaly.
case_images: 10.1177_2050313X18785510-fig1.jpg
case_images: PMC6041848_01_10.1177_2050313X18785510-fig1.jpg
case_images: fig1-2050313X18785510
case_images: Physical examination showed short stature at -0.1% (-3.3 standard deviation (SD) below the mean), no central puberty, cushingoid appearance with a weigh at 22%, and liver enlargement (6 cm below the right costal margin; Figure 1).
case_images: (a) CGMS at diagnosis with extremely large glycemic variations (HbA1c 11.9%-105 mmol/mol), several hypoglycemic events per day. (b) CGMS after 1.5 years showing less glycemic variations (HbA1c 8.4%-68.3 mmol/mol), good overnight control, higher daily values, and no hypoglycemia. Daily levels still need improvement.
case_images: 10.1177_2050313X18785510-fig2.jpg
case_images: PMC6041848_01_10.1177_2050313X18785510-fig2.jpg
case_images: fig2-2050313X18785510
case_images: Continuous glucose monitoring (CGM) showed severe peaks of hyperglycemia but also large variations with hypoglycemic periods (Figure 2(a)). The follow-up CGM after 18 months showed 50% of measurements within the range (Figure 2(b)).