hereditary chronic pancreatitis
Findings
No curated finding names hereditary chronic pancreatitis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Hereditary chronic pancreatitis (HCP), a rare inherited form of pancreatitis is defined as recurrent acute pancreatitis and/or chronic pancreatitis in two first-degree relatives or 3 or more second-degree relatives in 2 or more generations, for which no predisposing factors are identified. HCP is characterized by irreversible damage to both exocrine and endocrine components of the pancreas.
Definition from the Mondo Disease Ontology (MONDO:0008185), read 2026-09-29. CC BY 4.0.
Features
9 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abdominal painHPOHP:0002027
- Very frequent (80% to 99% of cases)
- Elevated circulating C-reactive protein concentrationHPOHP:0011227
- Very frequent (80% to 99% of cases)
- Increased total leukocyte countHPOHP:0001974
- Very frequent (80% to 99% of cases)
- Recurrent pancreatitisHPOHP:0100027
- Very frequent (80% to 99% of cases)
- Abnormal circulating enzyme concentration or activityHPOHP:0012379
- Frequent (30% to 79% of cases)
- Diabetes mellitusHPOHP:0000819
- Occasional (5% to 29% of cases)
Genes
7 genes
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- CTRCHGNC:2523
- Definitive · Ambry Genetics · Autosomal dominant · 2023
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2021
- PRSS1HGNC:9475
- Definitive · Ambry Genetics · Autosomal dominant · 2024
- Definitive · ClinGen · Autosomal dominant · 2020
- Definitive · Illumina · Autosomal dominant · 2020
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2023
- Strong · PanelApp Australia · Autosomal dominant · 2025
- Supportive · Orphanet · Autosomal dominant · 2021
- CPA1HGNC:2296
- Strong · Ambry Genetics · Autosomal dominant · 2018
Where it sits
- Narrower terms (2)
Other names
1 name
Resolves to: hereditary chronic pancreatitis
- Also called
- hereditary pancreatitis