Management of Crigler-Najjar syndrome.

Crigler-Najjar syndrome is a rare autosomal recessive inherited non-hemolytic unconjugated hyperbilirubinemia caused by UDP-glucuronosyltransferase deficiency. There are two forms of this disorder. Type 1 disease is associated with severe jaundice and neurologic impairment due to bilirubin encephalopathy that can result in permanent neurologic sequelae. Type 2 disease is associated with a lower serum bilirubin concentration and affected patients survive into adulthood without neurologic impairment. Currently, liver transplantation is the only available therapeutic method for these patients. Developing new curative approaches is a clinical need.

Gastrointestinal manifestations in hospitalized patients with chronic liver disease and COVID-19.

Background and aim: Gastrointestinal manifestations in COVID-19 have been frequently acknowledged by clinicians and scientists. However, their clinical significance and potential influence on the disease outcome is not entirely elucidated. In this study we aim to evaluate gastrointestinal involvement, both digestive symptoms and liver-related changes in hospitalized COVID-19 patients in correlation to the presence or absence of underlying liver disease and rate of mortality. Methods: We performed a retrospective cohort study of COVID-19 patients, consecutively admitted in a hospital from Chisinau, between September 3, 2020 and May 31, 2021. Data on clinical symptoms and laboratory findings were collected from electronic clinical records. The cohort was divided into two groups, with and without pre-existing liver disease. The Fisher exact, Pearson Chi-square tests were used to compare groups. Results: A total of 1835 patients were included, 108 (5.9%) with pre-existing liver disease and 1727 (94.1%) without this comorbidity. Digestive symptoms were reported by 331(18%) of the patients, diarrhea being the most common symptom 11.8% (217) and being encountered more in patients with underlying chronic liver disease. No statistical difference was identified between the groups in regard to other symptoms, comorbidities and rate of mortality. But patients with chronic liver disease had significant (P < 0.001) lower ferritin, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) in comparison with the other group. At admission, 341 (19.2%) had increased aspartate aminotransferase level (AST) and 317 (17.8%) alanine aminotransferase. The enzyme abnormalities were predominantly mild and transitory. Abnormal AST level at admission and during follow up, higher ESR, CRP, ferritin, lactate dehydrogenase (LDH) was found to correlate with higher rates of mortality. Conclusion: Digestive implications, especially diarrhea in COVID-19 patients is frequent, but do not appear to be associated with mortality. Elevated liver enzymes during hospitalization, age, high ferritin, CRP, LDH might be interpreted as risk factors for mortality in COVID-19 but further studies are needed to address this topic.

Pregnancy and COVID-19 - liver damage.

This review examines information from systematic reviews and meta-analyses, research studies, and case reports to present current knowledge about liver damage in pregnant patients having Covid-19 during pregnancy. Problems with diagnosis and differential diagnosis are examined in the context of the need to rule out other causes of liver dysfunction, including pregnancy-related liver disease. In this paper we give an overview of COVID-19 liver problems during pregnancy. Mechanisms of liver involvement in COVID-19 infection are being examined. An overview of the assessment of abnormal liver biological syndromes in pregnant patients is provided. Differential diagnostic algorithms for primary liver damage established in a pregnant woman in the context of the Covid-19 pandemic are presented. Challenges in diagnosis and etiology assessment methods and customized management options are described. The management of pregnant women with hepatic dysfunction onset on the Covid-19 background and subsequently aggravated is discussed. The importance of anticoagulant therapy as an essential measure of symptomatic management of Covid-19 in pregnant women is emphasized, as both pregnancy and COVID-19 are thrombogenic. Hypercoagulability appears to adversely affect the pregnant women liver with Covid-19 and post Covid-19 and anticoagulant therapy has benefits in the management of liver damage associated with Covid-19. The COVID-19 liver problems in a 33-year-old woman who was not vaccinated for Covid-19, without a history of chronic liver disease, was tested positive for Covid-19 at 33 weeks of gestation is discussed. The report of the diagnostics, differential diagnosis, and management questions in the context of liver dysfunction manifested by a significant increase in alanine aminotransferase cytolysis syndrome. The positive effect of anticoagulant therapy in resolving cytolytic syndrome is emphasized. The good maternal and perinatal result is also mentioned.

Acid suppression therapy, gastrointestinal bleeding and infection in acute pancreatitis - An international cohort study.

BACKGROUND: Acid suppressing drugs (ASD) are generally used in acute pancreatitis (AP); however, large cohorts are not available to understand their efficiency and safety. Therefore, our aims were to evaluate the association between the administration of ASDs, the outcome of AP, the frequency of gastrointestinal (GI) bleeding and GI infection in patients with AP. METHODS: We initiated an international survey and performed retrospective data analysis on AP patients hospitalized between January 2013 and December 2018. RESULTS: Data of 17,422 adult patients with AP were collected from 59 centers of 23 countries. We found that 23.3% of patients received ASDs before and 86.6% during the course of AP. ASDs were prescribed to 57.6% of patients at discharge. ASD administration was associated with more severe AP and higher mortality. GI bleeding was reported in 4.7% of patients, and it was associated with pancreatitis severity, mortality and ASD therapy. Stool culture test was performed in 6.3% of the patients with 28.4% positive results. Clostridium difficile was the cause of GI infection in 60.5% of cases. Among the patients with GI infections, 28.9% received ASDs, whereas 24.1% were without any acid suppression treatment. GI infection was associated with more severe pancreatitis and higher mortality. CONCLUSIONS: Although ASD therapy is widely used, it is unlikely to have beneficial effects either on the outcome of AP or on the prevention of GI bleeding during AP. Therefore, ASD therapy should be substantially decreased in the therapeutic management of AP.

Profile of hepatocellular carcinoma in the Republic of Moldova: first-hand information on the presentation, distribution and etiologies.

INTRODUCTION: Moldova is the European country with the highest incidence of hepatocellular carcinoma (HCC) in both sexes. There is, however, no data comprehensively describing the presentation and the risk factors of HCC in the country. We decided to analyze cases of HCC recently received in a tertiary healthcare Institution from Chisinau, the Moldovian capital. METHODS: A series of 148 primary liver tumors including 139 cases of HCC were retrospectively analyzed for demographic features, serological and biochemical data, and clinical presentation. RESULTS: The mean age of patients was 59 ± 10 years (range: 19-66) with a M:F sex ratio of 1.9. Tumors appeared on full-blown liver cirrhosis in 83% of cases and were composed of multiple nodules at diagnosis in 36% of patients. Serum Alpha-fetoprotein was exceeding 10ng/mL in 76% of cases. Liver tumor and hepatitis were co-discovered in 34% of cases. More than 81% of hepatocellular carcinomas were associated with at least one hepatitis virus. Carriers of anti-hepatitis C virus were predominating (55% of cases) over patients seropositive for hepatitis B virus surface antigen (36%). Half of the latter were also infected with hepatitis Delta virus. In total, dual or triple infections were present in 24% and 7% of cases. CONCLUSIONS: The burden of infections with hepatitis viruses is particularly important in Moldova and corresponds to a situation commonly observed in countries of the Southern hemisphere. A pro-active policy of screening for persistent liver infection targeting population at risk of HCC (> 50 years) and coupled with the distribution of antivirals in positive cases should be rapidly implemented in Moldova to reduce incidence or primary liver cancer.