Endoscopic features of low-phospholipid-associated cholelithiasis syndrome: A retrospective cohort study.

BACKGROUND AND OBJECTIVE: LPAC (low phospholipid-associated cholelithiasis) syndrome is a rare genetic form of cholelithiasis. ERCP (endoscopic retrograde cholangiopancreatography) is often used to remove gallstones in the bile duct. No published data is available on the role of ERCP in LPAC syndrome. PATIENTS AND METHODS: In this retrospective cohort study, we included patients diagnosed with LPAC syndrome in a single tertiary referral center between 2009 and 2021. Our aim was to assess the frequency, indications, modalities, results, and complications of ERCP, as well as predictive factors for ERCP, in LPAC syndrome. Independent factors associated with ERCP occurrence were identified using a multivariable Cox regression analysis. RESULTS: ERCP was required in 31.2 % of the 269 patients included for analysis. Among patients who required ERCPs, 78.6 % had the procedure before diagnosis (i.e., starting UDCA). Most common indications were choledocholithiasis (53.6 %) and acute cholangitis (29.5 %). Post ERCP pancreatitis, perforation and bleeding rates were 7.2 %, 2.6 %, and 1.3 %, respectively. Age and history of cholelithiasis in first-degree relatives were associated with a higher risk of ERCP (Hazard-ratio [HR]=1.30 [95 %confidence-interval [CI] 1.04-1.62] and HR=1.88 [95 %CI 1.15-3.07] respectively). Female gender and UDCA intake ≥ 1 year were associated with a lower risk of ERCP (HR=0.49 [95 %CI 0.29-0.82] and HR=0.44 [95 %CI 0.22-0.90] respectively). Median follow-up was 10.8 years. CONCLUSION: One-third of patients with LPAC syndrome undergo sphincterotomy. However, most procedures are performed before diagnosis and UDCA is associated with a lower risk of endoscopic procedure. Earlier diagnosis and treatment with UDCA may further reduce the need for ERCP in patients with LPAC syndrome.

Mottling score predicts survival in septic shock.

BACKGROUND: Experimental and clinical studies have identified a crucial role of microcirculation impairment in severe infections. We hypothesized that mottling, a sign of microcirculation alterations, was correlated to survival during septic shock. METHODS: We conducted a prospective observational study in a tertiary teaching hospital. All consecutive patients with septic shock were included during a 7-month period. After initial resuscitation, we recorded hemodynamic parameters and analyzed their predictive value on mortality. The mottling score (from 0 to 5), based on mottling area extension from the knees to the periphery, was very reproducible, with an excellent agreement between independent observers [kappa = 0.87, 95% CI (0.72-0.97)]. RESULTS: Sixty patients were included. The SOFA score was 11.5 (8.5-14.5), SAPS II was 59 (45-71) and the 14-day mortality rate 45% [95% CI (33-58)]. Six hours after inclusion, oliguria [OR 10.8 95% CI (2.9, 52.8), p = 0.001], arterial lactate level [<1.5 OR 1; between 1.5 and 3 OR 3.8 (0.7-29.5); >3 OR 9.6 (2.1-70.6), p = 0.01] and mottling score [score 0-1 OR 1; score 2-3 OR 16, 95% CI (4-81); score 4-5 OR 74, 95% CI (11-1,568), p < 0.0001] were strongly associated with 14-day mortality, whereas the mean arterial pressure, central venous pressure and cardiac index were not. The higher the mottling score was, the earlier death occurred (p < 0.0001). Patients whose mottling score decreased during the resuscitation period had a better prognosis (14-day mortality 77 vs. 12%, p = 0.0005). CONCLUSION: The mottling score is reproducible and easy to evaluate at the bedside. The mottling score as well as its variation during resuscitation is a strong predictor of 14-day survival in patients with septic shock.