Nodular regenerative hyperplasia of the liver

Summary

Nodular regenerative hyperplasia of the liver (MONDO:0018835) is a disease and 10 clinical trials. Top therapeutic interventions include propranolol and dexpropranolol. A subtype of liver disorder — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: 1-9 / 100 000 (Spain) [Orphanet-validated]
• Phenotypes (HPO): 2
• Clinical trials: 10

Clinical features

Epidemiology

Prevalence records

2 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 1 000 000	0.34	Spain	Validated
Point prevalence	1-9 / 100 000	3.1	Spain	Validated

Signs & symptoms

Clinical features (HPO)

2 HPO clinical features (Orphanet curated; top 2 by frequency):

HPO ID	Term	Frequency
HP:0006707	Abnormality of the hepatic vasculature	Very frequent (80-99%)
HP:0001409	Portal hypertension	Occasional (5-29%)

Identifiers

Disease identifiers

Field	Value
Canonical name	nodular regenerative hyperplasia of the liver
Mondo ID	MONDO:0018835
Orphanet	48372
ICD-11	1642018758
SNOMED CT	715140008
UMLS	C5779783
MedGen	1830387
GARD	0010929
Is cancer (heuristic)	no

Also known as: miliary hepatocellular adenomatosis · nodular regenerative hyperplasia · non-cirrhotic nodular transformation · non-cirrhotic nodulation · non-cirrhotic portal hypertension

Disease family

This is a subtype of liver disorder. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › digestive system disorder › hepatobiliary disorder › liver disorder › nodular regenerative hyperplasia of the liver

Related subtypes (31): polycystic echinococcosis, autosomal dominant polycystic liver disease, hepatorenal syndrome, hepatitis, hepatic vascular disorder, hepatic porphyria, hepatopulmonary syndrome, fatty liver disease, cirrhosis of liver, drug-induced liver injury, perinatal jaundice due to hepatocellular damage, Aagenaes syndrome, transient familial neonatal hyperbilirubinemia, hyperbiliverdinemia, transient infantile hypertriglyceridemia and hepatosteatosis, idiopathic copper-associated cirrhosis, familial intrahepatic cholestasis, bile duct cyst, hepatoportal sclerosis, primitive portal vein thrombosis, glycogen storage disease due to liver phosphorylase kinase deficiency, liver and intrahepatic bile duct neoplasm, alcoholic liver disease, early-onset familial noncirrhotic portal hypertension, liver failure, fibrotic liver disease, intestinal failure–associated liver disease, liver abscess (disease), membranous obstruction of inferior vena cava, liver disease, severe congenital, cystic fibrosis-related liver disease

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 10.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	9
PHASE3	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT01000779	PHASE3	COMPLETED	Comparison of Endoscopic Variceal Ligation (EVL) With Propranolol in Non Cirrhotic Portal Hypertension (NCPH)
NCT02417740	Not specified	RECRUITING	Natural History of Noncirrhotic Portal Hypertension
NCT04578301	Not specified	RECRUITING	Predicting Acute-on-Chronic Liver Failure After Surgical Intervention in Chronic Liver Disease
NCT05782556	Not specified	RECRUITING	Freiburg TIPS Registry
NCT06500403	Not specified	RECRUITING	Non-invasive Tools for PSVD Diagnosis
NCT07424963	Not specified	RECRUITING	Hepatic Venous Pressure Gradient Versus Endoscopic Ultrasound-Guided Portal Pressure Gradient - Comparative Analysis (HEPCA)
NCT03541057	Not specified	UNKNOWN	Vienna Vascular Liver Disease Study
NCT05719857	Not specified	UNKNOWN	Hepatic Venous Pressure Gradient and Elastography in Porto-sinusoidal Vascular Disorder
NCT06054451	Not specified	UNKNOWN	Clinical Diagnosis and Pathological Spectrum of Porto-sinusoidal Vascular Disease in India
NCT06145100	Not specified	COMPLETED	Prediction of Portal Hypertension in Patients With CVID (CVID-pHT)

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
PROPRANOLOL	4	3
DEXPROPRANOLOL	2	1