Low-phospholipid-associated cholelithiasis syndrome: Prevalence, clinical features, and comorbidities.

Background & Aims: Low-phospholipid-associated cholelithiasis (LPAC) syndrome, a rare genetic form of intrahepatic cholelithiasis in adults, is still poorly understood. We report the results of the largest-ever case-control study of patients with LPAC syndrome aiming to assess the prevalence, clinical features, and comorbidities of the disease.

Methods: We included all LPAC cases diagnosed between 2001 and 2016 in 11 French centres.

Prevalence of low phospholipid-associated cholelithiasis in young female patients.

Background And Aims: We evaluated the prevalence of low phospholipid-associated cholelithiasis, a specific form of cholelithiasis associated with at least 2 of the 3 following criteria: first symptoms before the age of 40; intrahepatic comet tail artefacts, sludge or microlithiasis on ultrasound imaging; and recurrence of symptoms after cholecystectomy.

Methods: We prospectively studied the cases of 60 consecutive female patients under 30 with symptomatic cholelithiasis.

Results: A diagnosis of low phospholipid-associated cholelithiasis was made in 14/60 patients (23%).

[Difficulty in diagnosing acute appendicitis in the presence of acute portal vein thrombosis: the role of CT scan. Report of 2 cases].

Acute appendicitis can cause of portal vein thrombosis. Diagnosis of appendicitis can be difficult because its symptoms may be masked by those of acute portal vein thrombosis. We report 2 cases of appendicitis associated with acute portal vein thrombosis whose diagnosis was delayed by several months.

[A new cause of intra and extrahepatic cholangitis: the drug hypersensitivity syndrome or DRESS (Drug Rash with Eosinophilia and Systemic Symptoms)].

The DRESS (Drug Rash with Eosinophilia and Systemic Symptoms) corresponds to a drug reaction generally including cutaneous eruption, fever, hematologic abnormalities such as eosinophilia and atypical lymphocytosis and one or more specific visceral lesions specially in the liver. We report a case of drug hypersensitivity syndrome or DRESS associated with intra and extra-hepatic biliary lesions. This syndrome was associated with sulfasalazine and naproxene therapy.