Low phospholipid-associated cholelithiasis syndrome
Citation, DOI, disclosures and article data
Citation:
Knipe H, Low phospholipid-associated cholelithiasis syndrome. Reference article, Radiopaedia.org (Accessed on 30 Mar 2025) https://doi.org/10.53347/rID-52070
Permalink:
rID:
52070
Article created:
Disclosures:
At the time the article was created Henry Knipe had no recorded disclosures.
View Henry Knipe's current disclosures
Last revised:
Disclosures:
At the time the article was last revised Henry Knipe had the following disclosures:
- Radiopaedia Events Pty Ltd, Speaker fees (past)
- Integral Diagnostics, Shareholder (ongoing)
- Micro-X Ltd, Shareholder (ongoing)
These were assessed during peer review and were determined to not be relevant to the changes that were made.
View Henry Knipe's current disclosures
Revisions:
3 times, by
1 contributor -
see full revision history and disclosures
Systems:
Sections:
Synonyms:
- LPAC
Low phospholipid-associated cholelithiasis (LPAC) syndrome is one of the syndromes associated with ABCB4/MDR3 mutation. Characteristics of this syndrome include 1,2:
- intrahepatic microlithiasis/sludge
- symptomatic cholesterol stones with early onset (<40 years)
- recurrent symptoms post cholecystectomy
- successful treatment (i.e. symptom resolution) with ursodeoxycholic acid (UDCA)
References
- 1. Pasmant E, Goussard P, Baranes L et al. First Description of ABCB4 Gene Deletions in Familial Low Phospholipid-Associated Cholelithiasis and Oral Contraceptives-Induced Cholestasis. Eur J Hum Genet. 2011;20(3):277-82. doi:10.1038/ejhg.2011.186 - Pubmed
- 2. Sundaram S & Sokol R. The Multiple Facets of ABCB4 (MDR3) Deficiency. Curr Treat Options Gastroenterol. 2007;10(6):495-503. doi:10.1007/s11938-007-0049-4 - Pubmed
- 3. Rosmorduc O & Poupon R. Low Phospholipid Associated Cholelithiasis: Association with Mutation in the MDR3/ABCB4 Gene. Orphanet J Rare Dis. 2007;2(1):29. doi:10.1186/1750-1172-2-29 - Pubmed
Incoming Links
Related articles: Pathology: Hepato-Pancreato-Biliary
- liver
- depositional disorders
- infection and inflammation
- liver abscess
- hepatic hydatid infection
- cirrhosis
- hepatitis
- cholecystitis
- cholangitis
- malignancy
- liver and intrahepatic bile duct tumors
- benign epithelial tumors
- hepatocellular hyperplasia
- hepatocellular adenoma
- hepatic/biliary cysts
- benign nonepithelial tumors
- primary malignant epithelial tumors
- hepatocellular carcinoma
- hepatocellular carcinoma variants
-
cholangiocarcinoma
- intra-hepatic
- mass-forming type
- periductal infiltrating type - Klatskin tumors
- intraductal growing type
- extra-hepatic/large duct type
- intra-hepatic
- biliary cystadenocarcinoma
- combined hepatocellular and cholangiocarcinoma
- hepatoblastoma
- undifferentiated carcinoma
- primary malignant nonepithelial tumors
- hematopoietic and lymphoid tumors
- primary hepatic lymphoma
- hepatic myeloid sarcoma (hepatic chloroma)
- secondary tumors
- miscellaneous
- adrenal rest tumors
- hepatic carcinosarcoma
- hepatic fibroma
- hepatic hemangioma
- hepatic Kaposi sarcoma
- hepatic lipoma
- hepatic mesenchymal hamartoma
- hepatic myxoma
- hepatic rhabdoid tumor
- hepatic solitary fibrous tumor
- hepatic teratoma
- hepatic yolk sac tumor
- inflammatory myofibroblastic tumor (inflammatory pseudotumor)
- nodular regenerative hyperplasia
- pancreatic rest tumors
- primary hepatic carcinoid
- benign epithelial tumors
- liver and intrahepatic bile duct tumors
- metabolic
- trauma
-
vascular and perfusion disorders
- portal vein related
- hepatic artery related
- hepatic veins related
- inferior vena cava related
- other
- third inflow
- liver thrombotic angiitis
- infra diaphragmatic total anomalous pulmonary venous return (TAPVR)
- hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease)
- pancreas
-
pancreatic neoplasms
- cystic neoplasm (cystic pancreatic mass differential diagnosis)
- solid neoplasm
- non-epithelial pancreatic neoplasms
- others
- simple pancreatic cyst
-
pancreatitis (mnemonic for the causes)
- acute pancreatitis
- chronic pancreatitis
- Ascaris-induced pancreatitis
- tropical pancreatitis
- autoimmune pancreatitis
- emphysematous pancreatitis
- hypertriglyceridemia-induced pancreatitis
- hereditary pancreatitis
- pancreatitis associated with cystic fibrosis
- pancreaticopleural fistula
- segmental pancreatitis
- pancreatic atrophy
- pancreatic lipomatosis
- pancreatic trauma
- pancreatic transplant
-
pancreatic neoplasms
- gallbladder and biliary
- congenital malformations and anatomical variants
- gallstones
- gallbladder inflammation
- bile ducts inflammation
- gallbladder wall abnormalities
- other gallbladder abnormalities
- bile duct dilatation (differential)
- bile duct wall thickening (differential)
- bile ducts neoplasms