Article Figures & Data
Figures
- FIGURE 1.
(A) Diazoxide-responsive β-cell with normal KATP channel (orange) shows diazoxide keeping channel open and causing hyperpolarization of membrane and inhibition of insulin release. (B) Diazoxide-unresponsive β-cell with failure of KATP channel assembly and tracking to plasma membrane because of ABCC8 or KCNJ11 mutation allows depolarization of membrane with opening of calcium channel causing influx of calcium and unregulated release of insulin. (Courtesy of Serene McLaughlin.)
- FIGURE 2.
Indications for 18F-FDOPA PET/CT using genetic analysis.
- FIGURE 3.
Specificity (A) and sensitivity (B) of 18F-FDOPA PET/CT for detection of focal lesions.
- FIGURE 4.
Sample imaging protocol. CECT = contrast-enhanced CT; NCCT = noncontrast CT.
- FIGURE 5.
Focal disease. 3-mo-old female with diazoxide-unresponsive HI with ABCC8 mutation. 18F-FDOPA 3-dimensional MIP image at 50 min shows 2 focal lesions, 1 within head (arrow) and other at pancreatic body/tail junction (arrowhead). Lesions were excised with 10% pancreatectomy. Finding 2 lesions is rare occurrence.
- FIGURE 6.
Focal lesion in pancreatic body. 3-mo-old female with ABCC8 mutation and paternal mutation. 18F-FDOPA 3-dimensional MIP image shows mild increased activity in pancreatic head and small lesion (arrowhead) in pancreatic body. Focal lesion was found in pancreatic body, abutting vasculature and requiring 50% pancreatectomy.
- FIGURE 7.
BWS. 1-mo-old male with suspected BWS and presenting with HI. 18F-FDOPA 3-dimensional MIP image at 10 min shows uptake within markedly enlarged pancreas, typical of BWS. Increased uptake within enlarged kidneys is also related to patient’s syndrome. Patient underwent 95% pancreatectomy.
Tables
No. of patients Undergoing surgery With focal PET/histology results With diffuse PET/histology results No. of atypical cases Accuracy of localization (%) Study Otonkoski et al. (25) (2006; Finland) 9 5/5 4/4 100 Ribeiro et al. (32) (2007; France) 24 15/15 8/9 3 atypical 92 Hardy et al. (19) (2007; United States) 50 18/24 26/26 1 large focal; 2 localized islet nuclear enlargement 100 Barthlen et al. (33) (2008; Germany) 11 9/9 1/2 1 atypical 100 Masue et al. (26) (2011; Japan) 12 6/9 3/3 3 large focal 33 Zani et al. (49) (2011; United Kingdom) 19 14/14 5/5 1 large focal 79 Laje et al. (28) (2013; United States) 105 45/53 50/52 100 Meintjes et al. (15) (2013; United Kingdom) 8 5/5 3/3 100 Christiansen et al. (35) (2018; Denmark) 34 22/22 12/12 1 atypical; 1 normal; 1 ectopic 91 Gubaeva et al. (50) (2019; Russia) 25 14/14 11/11 1 giant 100 Ni et al. (51) (2019; China) 14 12/12 2/2 Atypical 100 Total 311 165/182 (91%) 125/129 (97%)
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