Acalculous Cholecystitis as a Complication of Primary Epstein-Barr Virus Infection: A Case-Based Scoping Review of the Literature.

Primary Epstein-Barr virus (EBV) infection manifests with diverse clinical symptoms, occasionally resulting in severe complications. This scoping review investigates the rare occurrence of acute acalculous cholecystitis (AAC) in the context of primary EBV infection, with a focus on understanding its prevalence, clinical features, and underlying mechanisms. The study also explores EBV infection association with Gilbert syndrome, a condition that potentially exacerbates the clinical picture. Additionally, a case report of an 18-year-old female presenting with AAC and ascites secondary to EBV infection enhances the review. A comprehensive literature review was conducted, analyzing reported cases of AAC secondary to EBV infection. This involved examining patient demographics, clinical presentations, laboratory findings, and outcomes. The search yielded 44 cases, predominantly affecting young females. Common clinical features included fever, cervical lymphadenopathy, tonsillitis/pharyngitis, and splenomegaly. Laboratory findings highlighted significant hepatic involvement. The review also noted a potential link between AAC in EBV infection and Gilbert syndrome, particularly in cases with abnormal bilirubin levels. AAC is a rare but significant complication of primary EBV infection, primarily observed in young females, and may be associated with Gilbert syndrome. This comprehensive review underscores the need for heightened clinical awareness and timely diagnosis to manage this complication effectively.

Portopulmonary hypertension and serum endothelin levels in hospitalized patients with cirrhosis.

BACKGROUND: Cirrhosis is associated with several extrahepatic manifestations including portopulmonary hypertension (PPHT). Recent data suggest that endothelins (ETs) are related to the pathophysiology of PPHT. The study aimed to measure serum ET levels in hospitalized cirrhotic patients and to determine their association with PPHT and patient outcome. METHODS: Fifty-seven cirrhotic patients [43 males; median age 58 (28-87) years] underwent Doppler echocardiography. Patients with systolic pulmonary arterial pressure ≥40 mmHg and pulmonary acceleration time <100 ms were deemed to have PPHT. ET-1, 2, and 3 serum levels were measured with an ELISA assay. All-cause mortality was recorded over a median period of 24 months. RESULTS: Nine out of 57 patients (15.8%) had PPHT. Among various clinical variables, only autoimmune hepatitis was associated with PPHT (OR=11.5; 95% CI, 1.58-83.4; P=0.01). ET-1 levels [9.1 (1.6-20.7) vs 2.5 (1.4-9.2) pg/mL, P=0.02] and the ET-1/ET-3 ratio [4.73 (0.9-22.4) vs 1.6 (0.3-10.7), P=0.02] were significantly higher in patients with PPHT than in those without. ET-2 and ET-3 levels did not differ between the two groups. There was no difference in survival between the two groups, although ET-1 levels were associated with an adverse outcome in Cox regression analysis (HR=1.11; 95% CI, 1.02-1.22; P=0.02 per unit increase in ET-1). CONCLUSION: Our data suggest that ET-1 and the ET-1/ET-3 ratio are elevated in patients with PPHT and that ET-1 is associated with a poor outcome irrespective of PPHT.

Acute-phase proteins as indicators of bacterial infection in patients with cirrhosis.

BACKGROUND: Cirrhosis is associated with elevated levels of acute-phase proteins (APP), irrespective of the presence of infection. This condition limits the clinical application of APP determination in cirrhotic patients with bacterial infections. AIMS: To weigh the diagnostic value of several APP in cirrhotics with or without bacterial infection, and to correlate them with the clinical outcome. METHODS: We investigated 88 consecutive cirrhotic patients (67 males, 21 females; range 28-85 years) with mean age (SD) 58.9 (13.8) on admission, according to a standard protocol for infection. We measured the following APP: C-reactive protein (CRP), fibrinogen (FIB), haptoglobin (Hpt), ferritin (Fer), beta2-microglobulin (beta2-mg), C3, C4 and C1 inhibitor. RESULTS: From the 88 patients, 19 (21.6%) had documented infection at the entry based on clinical, radiological and microbiological data. This group of patients did not differ in basic demographics from those without infection. CRP [17.5 (20.7) vs 77.1 (43.9), P<0.001], beta2-mg [4.4 (4.1) vs 5.6 (2.2), P<0.001] and ferritin [461.2 (776.4) vs 825.8 (870), P=0.03] were significantly higher in infection, whereas C3 was significantly lower. No significant differences were noted in the remaining APP levels between the two groups. After receiver operating characteristic curves were fitted, CRP was the best diagnostic test for infection (area under the curve 0.91), followed by beta2-mg, ferritin, FIB, C1 inhibitor, C4, Hpt and C3. CONCLUSIONS: Serum CRP is the best test, among the examined APP, to discriminate bacterial infection in cirrhotics. A cut-off value of >55.8 mg/L has high sensitivity (79%) and specificity (96%), with the best diagnostic accuracy (92%).

Prevalence and longterm course of primary biliary cirrhosis in primary Sjögren's syndrome.

OBJECTIVE: To study the prevalence of primary biliary cirrhosis (PBC) and its progression in patients with primary Sjögren's syndrome (SS). METHODS: We investigated 410 patients with primary SS, without history of liver disease, for the presence of PBC based on a retrospective review of clinical, biochemical, immunologic, and histologic data. RESULTS: Thirty-six (8.8%) patients had cholestatic liver biochemistry. Of them, 21 (5.1%) had positive antimitochondrial autoantibodies (AMA) detected by indirect immunofluorescence, while 15 were AMA-negative. Ten of the 21 AMA-positive patients and 7 of the 15 AMA-negative patients were further investigated with liver biopsy, the result of which was compatible with PBC in all but one (AMA-negative) patient. Overall, 27 (6.6%) patients had definite (n=10), probable (n=11), or AMA-negative (n=6) PBC. Pathologically, most PBC lesions were stage 1. Five patients had a second liver biopsy, with no significant histological deterioration. CONCLUSION: PBC is a rather uncommon development in patients with primary SS. The disease appears to be pathologically mild, with a propensity for slow progression, as assessed clinically, biochemically, and histologically.