About this trial
Low blood sugars are known to cause brain damage in newborn babies. One of the most common causes of low blood sugars persisting beyond the new born period is a condition called congenital hyperinsulinism (HI). This is a disease whereby the pancreas secretes too much insulin and causes low blood sugars. Twenty to forty percent of these babies will have brain damage. There are two forms of this disease. In one form only a small part of the pancreas makes too much insulin (focal HI) and in the other, the whole pancreas make too much insulin (diffuse HI). Another very similar disease is insulinoma which occurs after birth, but also causes hyperinsulinism. If a surgeon could know which part of the pancreas has the focal lesion he could remove it and cure the patient.
The purpose of this study is to investigate whether a new investigational drug called Fluorodopa F 18, when used with a PET scan, can find the focal lesion and guide the surgeon to remove it, thus curing the patient and preventing further brain damage.
Eligibility criteria
Qualifiers
Patients with HI attending the Cook Children's Congenital Hyperinsulinism Center and being treated by an Endocrinologist which may be the PI or a partner of this clinician.
Hypoglycemia (blood glucose <70 m/dL) on a single measure despite the use of anti-hypoglycemic medications, if applicable to the individual patient, including and limited to diazoxide or octreotide
Inability to fast, defined as the inability to maintain a blood glucose >50 mg/dL for: 1) more than 12 hours for infants < 1 year of age; 2) more than 15 hours 1-3 years of age; 3) more than 18 hours over 3 years of age
no genetic testing results (e.g., due to insurance denial or parental refusal)
Disqualifiers
Patients who do not have a diagnosis of HI
Patients with genetic evidence of diffuse HI
Patients who are pregnant
Nursing mothers who are unwilling to discontinue breastfeeding their infant for 48 hours after Fluorodopa F 18 injection
Trial design
Treatments tested in this trial
- Fluorodopa F 18