Living with GCGR-related hyperglucagonemia
A rare pancreas condition with very high glucagon levels and growths in the pancreas, usually found in adults.
Understanding GCGR-related hyperglucagonemia
It involves overgrowth of pancreas alpha cells, pancreatic neuroendocrine tumors, and much higher than normal levels of glucagon in the blood. It does not cause glucagonoma syndrome. It is usually noticed in adulthood.
Signs & symptoms
Symptoms vary from person to person. GARD lists these among the most common:
- High glucagon level in the blood
- Belly pain
- Pancreas tumor
- Gallstones
- Inflammation of the pancreas (pancreatitis)
- Fatigue and diarrhea
Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.
Diagnosis
- 1Talk with your doctor
Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.
- 2See a specialist
Specialists who may be involved include cancer specialists and digestive system specialists.
- 3Ask which tests can help
Your care team will decide which exams and tests can confirm the diagnosis.
Care & treatment
- Care team
- Cancer specialists (oncologists); Digestive system specialists (gastroenterologists)
- Expert care
- Ask about multidisciplinary care centers for rare conditions
- Treatment
- GARD doesn't list specific treatments for this condition. Your care team will tailor care to your symptoms.
- Research
- Clinical trials and observational studies may be options. See the research section below.
General information, not medical advice. Your care team makes the decisions.
Everyday life
Living with a rare condition is easier with support.
- Connect with Pancreatic Cancer Action Network and other patient organizations
- Keep notes on symptoms, changes and questions for appointments
- Ask about patient registries that help research
- Look into financial aid and travel resources for care visits
Quick facts
Changes in the GCGR gene cause this condition. It is inherited in an autosomal recessive way, meaning a child inherits a changed copy of the gene from each parent.
Also known as: alpha-cell hyperplasia with glucagonemia; glucagon receptor-related hyperglucagonemia; mahvash disease; mvah; nesidioblastosis alpha cell hyperplasia microglucagonoma and nonfunctioning islet cell tumor; nesidioblastosis alpha cell hyperplasia microglucagonoma and nonfunctioning islet cell tumour
Patient organizations
- Pancreatic Cancer Action NetworkPancreatic tumors · United States
- American Cancer SocietyPancreatic tumors · United States
You are more than your diagnosis
Perspectives from people navigating life with GCGR-related hyperglucagonemia.
Explore research for GCGR-related hyperglucagonemia
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- Recruiting studies from ClinicalTrials.gov
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- Eligibility is confirmed by the study team
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Powered by HealthSpark, our AI chat helper · Study information from ClinicalTrials.gov · Eligibility is confirmed by the study team.
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