This study is sponsored by Children's Hospital of Philadelphia Department of Radiology. The purpose of this research is to study 18F-L-Fluoro-DOPA (F-DOPA), a tracer, which is used to take pictures of the pancreas. This imaging test is called an F-DOPA PET scan. The F-DOPA tracer is used to highlight areas of the pancreas that make too much insulin on the PET scan images. The test includes a CT scan (computerized tomography) done at the same time on the same machine, to help define exactly where the highlighted area is located within the pancreas and determine the location of blood vessels and other vital structures. Who can participate:

• Infants, children and adults with hyperinsulinemic hypoglycemia, diagnosed by a fasting test and/or response to glucagon stimulation
• Any age, from birth on
• Requiring surgery to remove part or all of the pancreas

The goal of the study extension is to see whether the incretin hormone GIP increases insulin secretion in individuals with CF and normal glucose tolerance compared to individuals without CF and normal glucose tolerance. This study includes 3 or 4 visits.

The purpose of this research study is to examine and understand the various mechanisms that contribute to CFRD and gain a better understanding of potential means to treat CFRD. Participation will include 2-3 visits, home monitoring, and will last about 2 years.

NAD+ (nicotinamide adenine dinucleotide) is an important substance found in the body that plays a role in energy metabolism and overall health. The purpose of this research study is to test the performance of a new magnetic resonance imaging (MRI) technique (spectroscopy) to measure NAD+ metabolism in brain and skeletal muscle. MRI provides pictures of the inside of the body and information about chemicals the body makes.We are seeking to enroll males and females with Friedrichs Ataxia (FA) as well as healthy matched volunteers, 18 years of age and above. Pregnant females excluded for safety purposes.

The purpose of the study is to determine changes in lactate and creatine in calf muscle of healthy adult volunteers. Males and females of different ages (18 - 30 years, 45-55 years, and 70-80 years) will be asked to attend a single in-person study visit. Prior to the completing the MRI scan, the study team will review the health status of individuals.

Our team is working on a study to better understand neurological problems, including seizures and developmental delays, that occur in children with hyperinsulinism, including HI/HA syndrome, and type 1 diabetes mellitus. The study involves a one-day visit to The Children's Hospital of Philadelphia and The University of Pennsylvania for a specialized magnetic resonance imaging scan (MRI) of the brain, an electroencephalogram (EEG), blood draw, and completion of questionnaires to evaluate development and behavior. The entire study visit is expected to take about five hours. If you are interested in learning more, please contact us at HIResearch@email.chop.edu.

Insulin therapy, commonly given as multiple daily injection therapy, is the only recommended treatment for Cystic Fibrosis Related Diabetes (CFRD). Traditional therapy for CFRD requires an intense daily effort related to diabetes care on top of the already burdensome management of Cystic Fibrosis. In this study, we will assess the safety and effectiveness of the iLet Bionic Pancreas, an automated insulin delivery system, which uses a continuous glucose monitor (CGM), an insulin pump, and a control formula that activates insulin delivery based on CGM glucose data.