Congenital Hyperinsulinism, Hyperinsulinemic Hypoglycemia, Persistent, Persistent Hyperinsulinemic Hypoglycemia of Infancy (PHHI)
Conditions
Keywords
Hyperinsulinemic hypoglycemia, PET/CT, PET/MRI, [18F]-DOPA, PET/MR, Congenital Hyperinsulinism
Brief summary
The primary objective of this study is to evaluate the utility of \[18F\]-DOPA PET to provide improved presurgical planning and distinguish between focal and diffuse forms of HI. The investigators will perform descriptive analysis, relying on visual analysis to diagnose and localize a focal lesion. Our findings will be compared to surgical histopathology to determine sensitivity and specificity or this technique. The investigators will also track patient surgical outcomes, specifically whether the patient is surgically cured or still requires medical management to control residual hypoglycemia.
Detailed description
Hyperinsulinemic hypoglycemia is caused by low glucose due to excessive insulin secretion and remains difficult to treat because of the requirement for extensive amounts of glucose and the lack of effective long-term medical therapy. Correct diagnosis, localization, and limited excision of the focal lesion will result in a complete cure of the patient. In contrast, medically unresponsive diffuse disease requires a near total pancreatectomy, greatly increasing the risk of future diabetes mellitus. Fortunately, \[18F\]-DOPA PET has been shown to be a useful noninvasive imaging method for distinguishing between focal and diffuse forms of hyperinsulinemic hypoglycemia. In this study, the investigators seek to validate the effectiveness of using PET/MR and PET/CT with F-DOPA to accurately and reliably detect and localize disease.
Interventions
DRUG18 F-DOPA
Subjects will undergo PET imaging with \[18F\]-DOPA. All PET imaging will be performed after administration of a single dose (0.08 - 0.16 mCurie/kg) of \[18F\]-DOPA. PET imaging can be performed on either Philips Ingenuity TF PET/MRI or PET/CT.
Sponsors
Baylor College of Medicine
Eligibility
Sex/Gender
ALL
Age
No minimum to 64 Years
Inclusion criteria
* All Patients clinically diagnosed with Hyperinsulinemia of any age * Patients in need of PET scan * Patients that require sedation * Patients that do not require sedation
Exclusion criteria
Subjects presenting with any of the following will not be included in the study: * Patients without Hyperinsulinemia * Patients who are or may be pregnant * Serious intercurrent medical illness other than hypoglycemia that precludes having the scan either because of patient instability or concerns about potential toxicity. * Patient's requiring emergency surgical intervention that would be inappropriately delayed by \[18F\]-DOPA PET imaging.
Countries
United States
Outcome results
None listed