Trait

Annular pancreas

A congenital anomaly in which the pancreas completely (or sometimes incompletely) encircles the second portion of duodenum and occasionally obstructs the more proximal duodenum.

HPO HP:0001734

Body system: Abnormality of the digestive system

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26 genes linked to Annular pancreas

Changes in these genes cause rare conditions in which Annular pancreas is seen. Most people with this trait have none of those conditions, and a gene on this list says nothing about anyone's own genome.

Gene	How often it is seen	Conditions
RFX6regulatory factor X6	Often (30–79%)	In 1 condition
SIK3SIK family kinase 3	Often (30–79%)	In 1 condition
CASZ1castor zinc finger 1	Sometimes (5–29%)	In 1 condition
FBXO22F-box protein 22	Sometimes (5–29%)	In 1 condition
FLI1Fli-1 proto-oncogene, ETS transcription factor	Sometimes (5–29%)	In 1 condition
FOXF1forkhead box F1	Sometimes (5–29%)	In 2 conditions
GABRDgamma-aminobutyric acid type A receptor subunit delta	Sometimes (5–29%)	In 1 condition
HSPG2heparan sulfate proteoglycan 2	Sometimes (5–29%)	In 1 condition
KCNAB2potassium voltage-gated channel subfamily A regulatory beta subunit 2	Sometimes (5–29%)	In 1 condition
LUZP1leucine zipper protein 1	Sometimes (5–29%)	In 1 condition
MMP23Bmatrix metallopeptidase 23B	Sometimes (5–29%)	In 1 condition
PDPNpodoplanin	Sometimes (5–29%)	In 1 condition
PRDM16PR/SET domain 16	Sometimes (5–29%)	In 1 condition
PRKCZprotein kinase C zeta	Sometimes (5–29%)	In 1 condition
REREarginine-glutamic acid dipeptide repeats	Sometimes (5–29%)	In 2 conditions
SETBP1SET binding protein 1	Sometimes (5–29%)	In 1 condition
SKISKI proto-oncogene	Sometimes (5–29%)	In 1 condition
SPENspen family transcriptional repressor	Sometimes (5–29%)	In 1 condition
STRA6signaling receptor and transporter of retinol STRA6	Sometimes (5–29%)	In 1 condition
TELO2telomere maintenance 2	Sometimes (5–29%)	In 1 condition
UBE4Bubiquitination factor E4B	Sometimes (5–29%)	In 1 condition
WNT7BWnt family member 7B	Sometimes (5–29%)	In 1 condition
ASXL1ASXL transcriptional regulator 1	Rarely (1–4%)	In 1 condition
FANCD2FA complementation group D2	Rarely (1–4%)	In 1 condition
MYCNMYCN proto-oncogene, bHLH transcription factor	Not recorded	In 1 condition
RECQL4RecQ like helicase 4	Not recorded	In 1 condition

How often is among people with one of the gene's conditions, the most often across them, as the Human Phenotype Ontology records it. These pages describe traits and the research behind them; they are not a diagnosis, and a doctor or genetic counsellor is the person to ask about symptoms.

Broader traits

Abnormally shaped pancreas

Where this comes from

The Human Phenotype Ontology, release 2026-09-01: the trait, its names, its definition and the genes linked to it. Find out more at hpo.jax.org.
Data release 2026.10.16-c6eea6bf.

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