Nutritional and Metabolic Diseases

All Conditions

Digestive System Diseases

Diseases and Abnormalities at or Before Birth

Neoplasms

Gland and Hormone Related Diseases

Rare Diseases

Hypoglycemia

Congenital Hyperinsulinism

Nesidioblastosis

Insulinoma

Hyperinsulinism

Metabolic Diseases

Glucose Metabolism Disorders

Adenoma

Adenoma, Islet Cell

Neoplasms, Glandular and Epithelial

Neoplasms by Histologic Type

Pancreatic Neoplasms

Gastrointestinal Neoplasms

Digestive System Neoplasms

Endocrine Gland Neoplasms

Gastrointestinal Diseases

Pancreatic Diseases

Endocrine System Diseases

Infant, Newborn, Diseases

Pancreatic Cancer

as in a single-arm before-and after study, the only one arm will receive intervention of low GI diet with supplementation of RCS.

Patients will be guided with a low GI diet with 60%\~70% of energy from carbohydrates. RCS constituted 30%\~50% of daily carbohydrate, and was supplemented as snacks every 4\~6 hours (25g per time) especially at night.

low GI diet with regular supplementation of raw corn starch

Rongrong Li

This will be a prospective single-arm before-and-after clinical trial in which raw corn starch (RCS) will be first applied on patients with unoperated insulinoma. Nutritional intervention with supplementation of RCS will be initiated in 20 patients with suspected insulinoma to improve their hypoglycemia before the surgery. Duration of nutritional intervention, fasting blood glucose, lipid profile, weight change, BMI and other metabolic indices will be recorded and compared before and after the intervention.

Insulinoma is a rare disease which can cause recurrent hyperinsulinemic hypoglycemia, subsequent hyperphagia as well as weight gain. As only 50% \~ 60% of pharmacological therapy (diazoxide, somatostatin, etc) is effective to elevate blood glucose, the nutritional interventions play a role as an important supportive treatment to maintain glycemic stablization and control weight gain before the curative surgery. As the essential management for patients with glycogen storage disease (GSD), the extended release cornstarch has been proved superior to prevent hypoglycemia since 1980s. Its application has elevated GSD patients' quality of life and prolonged their survival. In view of its charicteristic of chronic release in gastrointestinal tract, we will for the first time try to utilize the raw corn starch (RCS) on patients with suspected insulinoma and evaluate its efficacy of improving hypoglycemia in such patients.

This will be a prospective single-arm before-and-after clinical trial. Nutritional intervention with supplementation of RCS will be initiated in 20 patients with suspected insulinoma to improve their hypoglycemia before the surgery. Duration of nutritional intervention, fasting blood glucose, lipid profile, weight change, BMI and other metabolic indices will be recorded and compared before and after the intervention.

In vie of the fact that insulinoma is a rare disease and it will be difficult to enroll enough cases in this study, a prospective single-arm before-and-after study will be performed.

Application of Raw Corn Starch on Patients With Insulinoma

Application of Raw Corn Starch on Patients With Unoperated Insulinoma is Helpful to Decrease Risk of Hypoglycemia

the change of mean fasting blood glucose before and after nutritional intervention

the change of requency of hypoglycemia onset before and after nutritional intervention

the change of glycated albumin before and after nutritional intervention

the change of self-reported satiety score before and after nutritional intervention. Satiety will be measured using a graphic rating scale that combined verbal descriptors on a scale graded 0-6 (0 = no sensation, 1 = just noticeable/threshold, 2 = very mild, 3 = mild, 4 = moderate, 5 = fullness, 6 = pain)

the change of weight / BMI before and after nutritional intervention

the change of body fat mass (rate of fat mass) before and after nutritional intervention, through measurement by bioelectrical impedence analysis

the change of total triglycerides, total cholesterol, LDL-C, HDL-C and free fatty acid befor and after nutritional intervention, through measurement by lipid profiles

the change of uric acid before and after nutritional intervention

Peking Union Medical College Hospital

Symptoms and General Pathology

Syndrome

Beckwith-Wiedemann Syndrome

Congenital Abnormalities

Abnormalities, Multiple

Chromosome Disorders

Genetic Diseases, Inborn

Fetal Thalidomide Syndrome

All Drugs and Chemicals

Cardiotonic Agents

Dihydroxyphenylalanine

Dopamine

Dopamine Agents

Neurotransmitter Agents

18F-DOPA

Susan Becker, RN, BSN, CCRC

Lisa States, MD

This purpose of this study is to determine the ability of an 18F-fluoro-L-dihydroxyphenylalanine (18F-DOPA) PET (Positron Emission Tomography) scan to detect a focal lesion of hyperinsulinism and determine the location in patients with congenital hyperinsulinism, Beckwith Wiedemann Syndrome and suspected insulinoma. Safety data will be collected.

Patients with hyperinsulinemic hypoglycemia are at risk for neurological damage due to hypoglycemia. Early surgical intervention can be preventative. F-DOPA PET may be able to detect of a focal lesion prior to surgical intervention and be used during surgical planning to guide the surgical procedure.

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization	Link
Application of Raw Corn Starch on Patients With Insulinoma	NCT03930368	Hyperinsulinemi... Insulinoma Raw Corn Starch	low GI diet wit...	18 Years -	Peking Union Medical College Hospital
18F-L-Fluoro-DOPA PET/CT Scan Localization of Focal Pancreatic Lesions in Subjects With Hyperinsulinemic Hypoglycemia	NCT01916148	Congenital Hype... Beckwith-Wiedem... Insulinoma	18F-DOPA	-	Children's Hospital of Philadelphia

Spots Global Cancer Trial Database for hyperinsulinemic hypoglycemia