Low phospholipid-associated cholelithiasis syndrome (LPAC), a not uncommon cause of biliary problems and hospital admissions.

BACKGROUND AND AIMS: Biliary diseases are a major source of morbidity and mortality for patients and a burden for the healthcare system. The genetic syndrome LPAC (low phospholipid-associated cholelithiasis) is a little known and rare entity whose treatment with bile salts avoids symptoms, admissions and the need for surgery. Our aim is to determine its incidence and characteristics in our center. METHODS: Prospective study between February 2021 and September 2022. LPAC was diagnosed if (at least two): onset of biliary problems <40 years of age, recurrence of symptoms after cholecystectomy, ultrasound image of hepatolithiasis (multiple echoic foci, comet-tail images, hepatolithiasis with acoustic shadow). Demographic, clinical, genetic (analysis of MDR3 gene mutations) and ultrasound characteristics were analyzed, as well as their incidence in hospital admissions for biliary causes. RESULTS: 36 patients with LPAC were identified. Of these, 6 were among 237 admissions for biliary causes in the previous 9 months, with an incidence of 2.5% (95%CI 1.17-5.41). By age subgroup, the incidence was 16.7% in those admitted <40 years and 9.1% in those <50 years. Considering women only, the incidence was 21% in those admitted <40 years and 15.8% in those <50 years. All patients remained asymptomatic after treatment with ursodeoxycholic acid and there were no new admissions. CONCLUSIONS: LPAC syndrome is not as uncommon as it may appear, especially in women <50 years of age admitted with biliary problems. Its correct diagnosis based on simple criteria would avoid a significant number of hospital admissions and unnecessary cholecystectomies.

Use of abdominal ultrasound in the diagnosis of hepatic sinusoidal obstruction syndrome (SOS) in a patient receiving a hematopoietic-cell transplant.

The sinusoidal obstruction syndrome (SOS) is an uncommon form of portal hypertension that occurs in hematopoietic-cell transplant recipients who receive intense conditioning treatments. The diagnosis is clinical and it is usually delayed, because in these patients there are many causes that can damage the liver and can delay its detection. We present the case of a 47-year-old man, whose diagnosis was made with clinical and analytical data supported by ultrasound signs that showed hepatic congestion. After treatment with defibrotide the patient improved clinically and analytically. Hepatic vascularization and perfusion also improved. Therefore, abdominal doppler ultrasound is shown as a noninvasive exploration useful for the clinical management of SOS and as an early marker of good clinical evolution.

A bronchobiliary fistula treated with cyanoacrylate glue in a patient with liver cirrhosis.

A 60-year-old male was operated upon in 2002 for liver hydatidosis, which included partial right hepatectomy with cholecystectomy and bilioduodenal anastomosis. He then developed liver cirrhosis secondary to left hepatic duct stricture. He presents at the emergency room with dry cough, which he had for a month and then became associated with yellowish, bitter-tasting "fluid" expectoration. A chest-abdominal CT scan revealed a 6 x 5-cm collection roughly located somewhere between the middle pulmonary lobe and subphrenic area . A fistula was suspected, which prompted a sputum biochemistry test that was positive for bilirubin. Given the patient's impaired liver function because of his liver disease conservative treatment was initiated with an inner-outer drain under transparietal hepatic cholangiography (TPHC) to promote bile outflow via the duodenum. Subsequently, TPHC was used to locate the point where contrast leaked from the biliary tree into a pleural cavity, and the fistula was sealed using cyanoacrylate glue, which resulted in improved symptomatology.