Liver transplantation in a child with liver cirrhosis caused by langerhans cell histiocytosis: a case report

Background Langerhans cell histiocytosis (LCH) is a rare condition that has a variety of clinical manifestations. But LCH in children localized only in the hepatobiliary system is unusual. Case presentation. Here we reported a rare case of a 2-year-old boy who was serendipitously found to have elevated liver enzymes while undergoing treatment of a perianal abscess. After a period of earlier conservative treatment in another hospital, the perianal abscess had resolved but the levels of liver enzymes were still rising slowly. The child was then referred to our institution for a definitive diagnosis. After laboratory tests, imaging and pathological examinations, a diagnosis of liver cirrhosis and sclerosing cholangitis was established, although the cause was unclear. Subsequently, living-donor liver transplantation was performed due to deterioration in liver function. Following successful liver transplantation, a diagnosis of LCH localized only within the hepatobiliary system was finally confirmed, based on additional pathological and imaging investigation. Additionally, the BRAF V600E mutation in this patient was also confirmed. The child has now recovered without evidence of LCH recurrence. Conclusions LCH localized only within the hepatobiliary system is unusual. The presence of unexplainable sclerosing cholangitis and liver cirrhosis in any child should raise the suspicion of LCH.

IMBALANCED IMMUNOBIOLOGICAL REACTIVITY, INFECTION AND MUTAGENESIS FACTORS IN THE HEPATOBILIARY SYSTEM AFFECTED BY A NATURAL FOCAL HABITAT FACTOR – A TREMATODE INVASION BY OPISTHORCHIS FELINEUS

An interaction between decreasing host anti-infective defense due to long-term invasion with Opisthorchis felineus in the hepatobiliary system, duct bile colonization by microflora and revealing the endogenous mutagenesis (carcinogenesis) factor - secondary bile acids - in bile is considered in the article. The role of organism genotype in the pathogen-related immune response to Opisthorchis felineus trematode and helminth development in the hepatobiliary system has been shown. The role of dysregulated mechanisms of tissue homeostasis in induction of compensatory chronic homeostatic proliferation and somatic cell oncogenesis is discussed. The study results evidence that disturbed functioning of the regulatory T-cells, inhibition of the NK cell effector function and very high functional activity of the memory B-cells are of great importance in imbalanced host immunobiological reactivity, caused by chronic opistorchosis invasion. Decreased host anti-infective protection causes intrahepatic bile duct infection with different bacterial species. Presence of secondary bile acids in hepatobiliary system was associated with biliary bacterial strains inhabiting intestinal tract: Proteus vulgaris*, Proteus mirabilis*, Citrobacter freundii*, Bacteroides alcaligues faecalis*, Clostridium*, Streptococcus faecalis*, Еscherichia coli* (*gut microflora). Participation of microbiota in bile acid biotransformation immediately in the duct bile has been confirmed in in vitro experiments. Experimental methods on Drosophila melanogaster and Salmonella tiphimurium strains: TA 100, TA 98 allowed to find out that bile from chronic opistorchosis patients exerts higher mutagenic activity compared with control groups. Mutational events in somatic and bacterial cells depend on the presence of secondary bile acids (deoxycholic, lithocholic) in duct bile, as well as the level of total bile acid concentration. The study data confirm the concept by Professor A.A. Shain about presence of endogenous risk factor for developing primary cholangiocellular liver cancer such as secondary bile acids in the bile of chronic opistorchosis patients. A concept of cholangiocarcinogenesis, based on mutational events, is added up with disturbance of generative cycle in tissue cells and their differentiation due to decreased kylon factor activity, as well as sensitivity threshold to it. Level of investigation and understanding of mechanisms underlying cholangiocarcinogenesis during chronic opisthorchiasis invasion will allow to develop pathogenetic approaches to correct homeostasis regulation and prevention of cholangiocarcinomas.

Influence of complex treatment on the functional state of the hepatobiliary system in patients with diabetes mellitus

Liver damage in diabetes mellitus is of particular interest, since this factor significantly affects the course of the disease, the level of compensation and prognosis of the underlying disease. The aim. To study the effect of complex treatment using ursosan on the functional state of the hepatobiliary system in patients with diabetes mellitus. Materials and methods. The study included 30 patients with type 1 diabetes mellitus and 48 patients with type 2 diabetes. According to the duration of diabetes, patients were divided into three subgroups: a) up to five years; b) 5–10 years; c) more than 10 years. The number of patients was: 1a group – 13, 1b group – 10, 1c group – 7 people; Group 2a – 23, 2b group – 13, 2c group – 10 people. The control group consisted of 23 apparently healthy people. Ursosan was prescribed at a dose of 10-12 mg per 1 kg of body per day for 6 months. Clinical laboratory and instrumental research methods were used to study the functional state of the liver and gallbladder. Research results. After the course of treatment with Ursosan, patients with diabetes mellitus showed normalization of protein, pigment, enzymatic metabolism and, to a lesser extent, lipid metabolism in the liver, which led to an improvement in cellular metabolism and redox processes, providing a stable course of diabetes. Conclusions. A 6-month course of treatment with Ursosan in diabetic patients promotes long-term diabetes compensation. Patients during treatment have a decrease in cytolysis syndrome indicators (alanine aminotransferase (AlAT), aspartate aminotransferase (AsAT), lactate dehydrogenesis (LDH)) by about 1.5 times in all 3 indicators compared to the indicators before treatment. The same trend was observed in terms of gamma-glutamine transferase (GGT) and alkaline phosphatase (ALP)

Possibilities of predicting lesions of the hepatobiliary system in children with community-acquired pneumonia

Annotation. The prognostic criteria of complications of community-acquired pneumonia and the possibility of developing disorders of the hepatobiliary system (HBS) depending on the concentration in the serum of the secretory leukocyte protease inhibitor (SLPI) were studied. The data of clinical and laboratory examination of 338 children with community-acquired pneumonia aged from one to three years were analyzed. Statistical processing of the results was performed using the system “IBM SPSS Statistica” 12 using parametric and non-parametric methods. It was found that in young children with pneumonia in the serum increases the concentration of SLPI, the level of which depends on the course and severity of pneumonia. It has been shown that the development of lobar pneumonia is significantly higher in patients from the SLPI cohort IV quartile (OR – 1.986, 95% CI – 1.864-2.356), compared with children from the cohort SLPI II and III quartile (OR – 0.476, 95% CI – 0.405- 0.559, OR – 0.494, 95% CI – 10.423-0.576, respectively). At the same time, at the values of SLPI at the level of III-IV quartile (OR – 1.923, 95% CI – 1.457-1.866) there is the development of community-acquired pneumonia. It was found that the development of pathological processes in the organs of HBS is associated with increased levels of SLPI. Thus, patients with polysegmental pneumonia and SLPI III/IV quartile (OR – 2.190, 95% CI – 1.810-2,754) are twice as likely to develop pathology of the hepatobiliary system than children with SLPI I/II quartile (OR – 1.153, 95% CI – 1.071-1.527). The established fact indicates the involvement of SLPI in the pathogenesis not only of pneumonia, but also in disorders of HBS.

A rare case of double gall bladder: a case report and review of literature

Double gall bladder is a rare congenital anomaly of the Hepatobiliary system with an incidence of 1:4000 due to abnormalities resulting during the embryogenesis during fourth to sixth week of gestation. Boyden was first to describe the duplication of the gallbladder and Harlaftis classification describes three types for the duplication of the gallbladder. Preoperative diagnosis of the duplication of the gallbladder has to be confirmed to minimise the complications during the surgery and post operatively. Sometimes the duplication of the gallbladder is missed preoperatively and hence increasing the risk and complications during the cholecystectomy. MRCP is the investigation of choice for preoperative diagnosis of the duplication and the laparoscopic cholecystectomy being the treatment of choice for the double gallbladder. We encountered a 25 year old girl with duplicate gallbladder who underwent laparoscopic cholecystectomy with removal of both gallbladders successfully.

Liver diseases at new coronavirus infection in pregnant women of Samara region

The new coronavirus infection (COVID-19) is a life-threatening condition. The features of the treatment of COVID-19 in pregnant women are not sufficiently studied and contradictory, therefore, research in this area is relevant.Objective. Assess the presence of liver disease in pregnant women with COVID-19 infection during pregnancy and after childbirth, taking into account the treatment.Methods. A retrospective analysis of medical records of 43 pregnant women aged 19 to 39 years (mean age 29.1 ± 5.4 years) who were hospitalized with laboratory-confirmed COVID-19 infection was performed. The selection was carried out by the method of continuous sampling.Results. 17 women (39.5%) had an acute respiratory viral disease, 26 people (60.5%) were diagnosed with bilateral polysegmental pneumonia with pulmonary tissue damage from 5 to 25%. In the structure of extragenital pathology, 46.5% of women had anemia and 23.3% of diseases of the hepatobiliary system, including chronic non-calculous cholecystitis (4.7%), biliary dysfunction (7%), chronic viral hepatitis B (2.3%) and chronic viral hepatitis C (9.3%). 6.9% were diagnosed with cholestatic hepatosis of pregnant women before admission to the hospital. An increase in the level of transaminases above the upper normal values was noted in 69.8% of cases, alkaline phosphatase – in 76.7%. In the structure of pregnancy complications in women with COVID-19 infection, fetal hypoxia prevailed in 15 women (34.9%). In second place is premature rupture of amniotic fluid (16.3%) and fetal malnutrition (16.3%).Conclusions. Pregnant women with diseases of the hepatobiliary system and anemia are most susceptible to new coronavirus infection. They have an increase in the level of transaminases and alkaline phosphatase, which can be caused by liver damage due to the increased tropism of the virus to cholangiocytes and hepatocytes, as well as hepatotoxic drugs. The most common complications of pregnancy in women with a new infection are premature birth, fetal hypoxia, and antenatal death.

A Retrospective Analysis of the Clinical Studies of a Drug "Flakozid" in the Treatment of Diseases of the Hepatobiliary System

Introduction. The medicine "Flakozid" with hepatoprotective effect has been developed at the VILAR. The drug is presented in dosage form-tablets of 0.1 g for oral drug administrationAim. To analyze the clinical efficacy and safety of "Flakozid" therapy according to clinical laboratory methods, as well as the motility of the gallbladder and bile ducts in patients with diseases of the hepatobiliary system.Materials and methods. The results of clinical studies of "Flakozid" (0.1 g tablets) were analyzed in 99 patients with chronic active hepatitis, chronic stone-free cholecystitis and fatty liver dystrophy, conducted in 2 clinical institutions: Perm State Medical University. Academician E. A. Wagner of the Ministry of Health of Russia and the Central Research Institute of Gastroenterology. "Flakozid" was prescribed against the background of a therapeutic diet (Table No. 5) of 0.1-0.2 g 3 times a day after meals for 32 days and repeated courses (3-5) for 6-12 months. Analysis of the efficacy and safety of "Flakozid" was carried out on the basis of the results of clinical and laboratory studies: general and biochemical blood analysis, general urinalysis, electrocardiogram. To study the motility of the gallbladder and biliary tract, the method of multifractional duodenal probing was used to determine the functional state of the sphincter apparatus of the gallbladder and biliary tract. In cystic and hepatic bile, its biochemical composition was determined. All patients underwent X-ray examination of the gastrointestinal tract, liver scanning and hepatography with iodine-131-bengal-roz.Results and discussion. In chronic active hepatitis, chronic stone-free cholecystitis and fatty liver dystrophy the use of "Flakozid" orally in daily doses of 0.3-0.6 g for 25-45 days led to an improvement in the General condition of patients, a decrease in pain in the right hypochondrium, a decrease in dyspeptic disorders, and an improvement in appetite. According to cholecystography, the indicators of concentration and contractility of the gallbladder improved. In terms of the severity of the therapeutic effect, "Flakozid" was not inferior to silibor and carsil, and in some indicators (improvement of the motility of the gallbladder and biliary tract) significantly exceeded them.Conclusions. Treatment of "Flakozid" improved functional state of the liver, reducing the syndrome of cytolysis and cholestasis. "Flakozid" is recommended in clinical practice in the complex treatment of diseases of the hepatobiliary system, such as chronic active hepatitis, chronic stone-free cholecystitis and fatty liver dystrophy.