Hepatopatía crónica asociada a Fontan: a propósito de dos casos y revisión de la literatura / Fontan-associated liver disease: report of two cases and literature review

La técnica quirúrgica de Fontan consiste en transmitir el flujo de la vena cava inferior a través de una anastomosis cavopulmonar para evitar que se mezcle la sangre oxigenada con la no oxigenada, en caso de atresia tricuspídea asociada a ventrículo hipoplásico. Las alteraciones fisiológicas que produce esta nueva circulación tienen varias consecuencias a nivel cardiaco y extracardiaco, incluido el hígado, con múltiples descripciones de hepatopatía crónica de origen vascular, e incluso de carcinoma hepatocelular en hígado cirrótico a edades tempranas. Con el objetivo de conocer un poco más sobre las afectaciones hepáticas que produce este procedimiento, se describen dos casos clínicos y se realiza una revisión de la literatura. A pesar de que la hepatopatía asociada a Fontan es una entidad poco frecuente, los hepatólogos deben tenerla presente y aprender a reconocerla, ya que un adecuado seguimiento puede ser la diferencia al momento de seleccionar de manera eficaz, aquellos pacientes que se beneficiarían de un trasplante cardiaco único o combinado de hígado y corazón, además de permitir la detección de manera oportuna de complicaciones tan devastadoras como un carcinoma hepatocelular en estadios avanzados.

Fontan surgery diverts the flow of the inferior vena cava through a cavopulmonary anastomosis, to avoid mixing the oxygenated and non-oxygenated blood, in the presence of tricuspid atresia associated with hypoplastic ventricle. The physiological changes produced by this new circulation have several consequences at cardiac and extracardiac level, including the liver, with multiple descriptions of chronic liver disease of vascular origin, and even hepatocellular carcinoma in cirrhotic liver at an early age. With the aim of a better understanding of liver complications caused by this procedure, two clinical cases are described and a literature review is presented. Despite the fact that Fontan-associated liver disease is a rare entity, hepatologists must keep it in mind and learn how to recognize it, since an adequate follow-up can make the difference when effectively selecting those patients who would benefit from a single or combined liver and/or heart transplant, as well as allowing the early detection of devastating complications such as advanced stage hepatocellular carcinoma.

Diagnóstico de hepatitis autoinmune / Diagnosis of autoimmune hepatitis

La hepatitis autoinmune es una enfermedad inflamatoria del hígado de etiología desconocida, que se caracteriza por la presencia de autoanticuerpos y niveles altos de globulinas séricas. Afecta a ambos sexos, a todos los grupos de edad, y se ha descrito en todas las razas. Tiene una amplia variedad de presentación clínica, lo que la convierte en un reto diagnóstico para el médico. La forma de presentación más común es la hepatitis aguda; sin embargo, un porcentaje no despreciable de pacientes es asintomático y hasta el 40% de los casos tiene cirrosis al momento del diagnóstico. El diagnóstico se basa en: 1) la medición de autoanticuerpos, como los antinucleares (ANA), los antimúsculo liso (ASMA), y los antimicrosomales de hígado y riñón (anti-LKM-1); 2) la detección de niveles séricos elevados de globulinas (especialmente IgG); 3) descartar otras etiologías de hepatitis crónica; y, 4) los hallazgos histológicos, los cuales además de confirmar el diagnóstico, ayudan a establecer la severidad. Se han desarrollado escalas y puntajes diagnósticos que permiten una mejor aproximación a dicha patología sin reemplazar el juicio del médico, por lo que deben ser interpretados bajo un adecuado contexto clínico. Un diagnóstico apropiado puede permitir el inicio oportuno de tratamiento con el fin de disminuir la progresión y el desarrollo de complicaciones.

Autoimmune hepatitis is an inflammatory disease of the liver of unknown etiology. It is characterized by the presence of autoantibodies and elevated levels of serum globulins. It affects both genders, all age groups and has been described in all races. It has a wide variety of clinical manifestations, so it remains a diagnostic challenge for physicians. The most common form of manifestation is acute hepatitis, however a non-negligible percentage of patients is asymptomatic, and up to 40% of cases have cirrhosis at the time of diagnosis. The diagnosis is based on: 1) measurement of autoantibodies, such as antinuclear antibodies (ANA), anti-smooth muscle antibodies (ASMA), and liver/kidney microsomal type 1 antibodies (anti-LKM-1); 2) detection of elevated serum levels of globulins (especially IgG); 3) exclusion of other causes of chronic hepatitis; and, 4) histological findings, which also confirm the diagnosis and help determine its severity. Diagnostic scales and scores have been developed to allow a better approach to this pathology without replacing the clinical judgment, so they must be interpreted under an adequate clinical context. A proper diagnosis can allow a timely initiation of treatment in order to decrease the progression and the development of complications.

Formation mechanism of neutrophil extracellular traps and their role in liver diseases / 临床肝胆病杂志

Neutrophil extracellular traps (NETs) have recently been found to be a new immune mechanism for neutrophils to kill pathogens. The main component of NETs is DNA, with granular proteins of different sizes attached to the surface. NETs play different roles in various liver diseases such as hepatic ischemia-reperfusion injury, chronic hepatitis B, liver cirrhosis, and liver cancer. This article reviews the formation mechanism, structure, and role of NETs in liver diseases.

Clinico-bacteriological profile of spontaneous bacterial peritonitis in cirrhosis of liver with ascites

Background: A good and an effective empirical treatment of SBP is not possible unless bacteriological spectrum and their antibiogram is clear. This becomes more important in country like India due to high antibiotic resistance.  Authors aim was to assess the prevalence of SBP, clinical and microbiological profile of its variants, in patients with cirrhosis seeking care at a tertiary care teaching hospital.Methods: Patients of liver cirrhosis with ascites seeking care at study subject during January- December 2018 formed the study population. Diagnosis was based on clinical examination, biochemical investigation and ultrasonography. Diagnostic abdominal paracentesis was performed on subjects within 24 hours of admission. Ascitic fluid was aspirated. It was sent for microbiological examination.Results: Prevalence of SBP was 16.12%. Out of total ten subjects with Spontaneous Bacterial Peritonitis (SBP), six subjects were of Culture Negative Neutrocytic Ascites (CNNA). Thus most common (60%), variant of Spontaneous Bacterial Peritonitis (SBP) was Culture Negative Neutrocytic Ascites (CNNA). Prevalence of Mono-microbial Non-neutrocytic Bacterascites (MNBA) and Classic-Spontaneous Bacterial Peritonitis (C-SBP) was 20%.  Two subjects found positive for MNBA, Staphylococcus aureus and Acinitobacter spp. was detected in each patient. Klebseila pneumonia, Escherichia coli and Coagulase negative Staphylococcus were found in ascitic fluid culture of C-SBP. In clinical variants of SBP, 80% subject’s complaint about abdominal pain. Hepatic-encephalopathy and fever was seen in 7(70%) patients. Rebound tenderness was seen among 60%.Conclusions: Diagnostic paracentesis should be advised to all cirrhotic patients with ascites. Ascitic fluid analysis should be sent for better selection of antibiotics thus better outcome of cirrhotic patients.

HEPATITIS В, С VIRUS INFECTION AND M2BPGI LIVER CIRRHOSIS MARKER CORRELATION / Шинэ санаа Шинэ нээлт

@#BACKGROUND Mostly of liver cancer in the world is caused by hepatitis B and C virus. Liver cancer occurs within 10-29 years after the virus is infected. If have liver cirrhosis, you can develop cancer after 5-10 years. According to the study in Mongolia 2014, C virus infection is 9.5% and B virus infection is 10.6%, high prevalence of hepatitis B, C and liver cirrhosis. Designed by Sysmex Corporation of Japan, possible analyze from blood, non invasive, sensitive and specific, M2BPGi liver cirrhosis marker. It is necessary to investigate the relationship between hepatitis B and C viruses and M2BPGi liver cirrhosis marker. METHODS M2BPGi liver cirrhosis marker measured total patients number 283. Of this patients 172 cases infected hepatitis B and C viruses, 78 patients with hepatitis B virus infection and 94 patients hepatitis C virus infection. All tests performed by full automatic analyzers Sysmex HISCL-5000, JEOL BM-6010, in the Laboratory department, Medipas Hospital, Orkhon province. RESULT Of the 283 patients who received the M2BPGi screening, 33% had C virus, 28% B virus, 4% had B and C virus co-infected, and 35% had no virus. Of the 172 patients infected with hepatitis B and C virus, man 97 (56%), woman 75 (44%). The majority of patients (72%) have liver function abnormality. Of patients with B and C viruses 115 (67%) were positive for M2BPGi liver cirrhosis marker. M2BPGi positive patients with 68 (59%) had C virus, 47 (41%) had B viruses. CONCLUSION Men are more likely to be infected with hepatic viruses. 67% of patients with hepatitis B and C viruses have M2BPGi liver cirrhosis marker positive. The likelihood of a change M2BPGi liver cirrhosis marker, more likely associated hepatitis C virus infection, than B virus infection. The presence of liver cirrhosis in adults under 45 years of age B virus is relatively high compared to C virus infection.

Cardiac changes in hepatic cirrhosis in Government Dharmapuri Medical College Hospital, Dharmapuri

Introduction: Cirrhosis liver, is characterized by diffuse destruction and regeneration of hepatic parenchymal cells leading to deposition of connective tissue with resulting disorganization of the lobular and vascular architecture. Despite the remarkable regenerative capacity of the liver, once hepatic parenchymal reserve is exceeded, clinically overt or decompensated cirrhosis ensues. Portal hypertension develops due to resistance to blood flow through the liver resulting increase in portal venous pressure leading to diversion of blood flow through low resistance portosystemic collaterals thereby bypassing the liver. The current study was designed to precisely evaluate the cardiovascular system in a group of patients with hepatic cirrhosis based on clinical examination. Aim of the study: To clinically evaluate patients with hepatic cirrhosis with respect to changes in heart rate, blood pressure, mean arterial pressure, ECG jugular venous pressure and precordial examination. Materials and Methods: 50 patients of cirrhosis liver were selected for the study. These patients were admitted in the general medical wards. All patients were subjected to routine investigations. All patients were subjected to ultrasound scan abdomen to confirm the diagnosis of cirrhosis. Patients with ascites underwent abdominal paracentesis and fluid was analyzed for protein content and cells. All patients were then subjected to electrocardiography, chest X-ray and M-mode 2-Dimensional echocardiography. Results: Out of the 50 patients studied 35 (70%) were males and 15 (30%) were females. The age of the patients ranged from 19 years to 75 years. 5 Patients (10%) were alcoholics, 14 patients (28%) had past history of jaundice or, 8 patients (16%) presented with haemetemesis. Among this 4 patients were P. Elango, G. Indumathi. Cardiac changes in hepatic cirrhosis in Government Dharmapuri Medical College Hospital, Dharmapuri. IAIM, 2017; 4(9): 19-24. Page 20 HbsAG+ (8%) and 2 patients were diabetics 4%. All patients had sonographic evidence of cirrhosis.with portal hypertension. Out of 50 patients, 3 patients had elevated blood pressure. Previous studies show that the systolic blood pressure more than 160 mmHG and diastolic blood pressure more than 95 mmHg are the range for hypertension in cirrhotic patients. The electro cardiac gram showed an average heart rate of 82/ m. The low QRS voltage in chest leads and limb leads were found in 10 patients. T wave inversion was found in chest leads (V1 to V3 / V6) in 4 (8%) patients, in II, III avf in 7 patients (14%). The chest roentgenogram showed Hepatic Hydrothorax in 5 patients (10%). Cardio megaly was evident in chest X – ray in 11 patients (22%). Conclusion: The result of this study clearly showed that a large number of patients with hepatic cirrhosis are asymptomatic (40%) about cardiovascular system, have evidence of cardiac involvement in electrocardiography and echo cardiograph. cardiac decompensation in cirrhosis is rare despite the high output state and its presence as indicated by left ventricular systolic dysfunction.

Perfil transfusional dos cem primeiros pacientes submetidos a transplante hepático em Fortaleza / Transfusion profile of the first one hundred patients undergoing liver transplant in Fortaleza / Perfil de transfusión de los primeros cien pacientes sometidos a trasplante de hígado en Fortaleza

Objetivo: Traçar o perfil transfusional da primeira centena de pacientes submetidos a transplante hepático em um hospital escola, em Fortaleza, Ceará. Método: Trata-se de um estudo observacional, analítico e retrospectivo. Resultados: Dentre os cem pacientes inicialmente incluídos no estudo, houve dez retransplantes. Um paciente foi excluído do estudo por impossibilidade de acesso ao prontuário. Por esses motivos, 89 prontuários foram analisados. Conclusão: Os pacientes, em sua maioria, eram adultos com idade média de 47 anos, do gênero masculino e com grande prevalência de cirurgias abdominais prévias. O grupo sanguíneo mais prevalente foi o grupo A, e a principal causa do transplante, a cirrose por álcool. Em média, os pacientes receberam seis unidades de hemocomponentes, sendo o concentrado de hemácias o mais frequentemente transfundido.

Objective: To identify the profile of the first hundred transfusion patients undergoing liver transplantation at a university hospital in Fortaleza, Ceará. Method: An observational, analytical, and retrospective study was performed. Results: There were 10 retransplantations among the 100 patients initially enrolled in the study. One patient was excluded from the study owing to the inability to access the medical records. For this reason, 89 medical records were analyzed. Conclusion: Most patients were male adults with an average age of 47 years, and a high prevalence of previous abdominal surgeries were observed among them. The most common blood group was group A, and the leading cause of transplantation was cirrhosis induced by alcohol. On average, patients received 6 units of blood components, and the most frequently transfused were packed red blood cells.

Objetivo: Trazar el perfil de transfusión de los cien primeros pacientes sometidos a trasplante de hígado en un hospital universitario en Fortaleza, Ceará, Brasil. Método: Se realizó un estudio de observación, analítico y retrospectivo. Resultados: Entre los 100 pacientes incluidos inicialmente en el estudio, hubo 10 re-trasplantes. Se excluyó un paciente del estudio porque no había acceso a sus registros médicos. Por estas razones, se analizaron 89 registros médicos. Conclusión: La mayoría de los pacientes eran adultos con una edad promedio de 47 años, sexo masculino y con un alto predominio de cirugías abdominales previas. El grupo sanguíneo más frecuente fue el grupo A y la principal causa del trasplante fue la cirrosis inducida por alcohol. En promedio, los pacientes recibieron seis unidades de componentes sanguíneos, siendo el concentrado de glóbulos rojos el más frecuentemente transfundido.

Clinical value of serum HDL-C in the evaluation of synthetic function of the liver / 中国基层医药

Objective To evaluate the clinical value of serum high density lipoprotein cholesterol (HDL-C)in the evaluation of synthetic function of the patients with liver diseases.Methods Abbott Aeroset automatic biochemical analyzer was used to detect serum HDL-C level in control group (30 cases) and liver disease group(87 cases).T test was used to analyze the differences of serum HDL-C level in liver disease group and control group,different liver disease groups and control group,and different liver disease groups.Results HDL-C level was (1.28 ±0.20)mmol/L in the control group,(0.77 ± 0.15)mmol/L in the liver disease group,and (0.81 ± 0.13)mmol/L in the hepatitis group,(0.68 ± 0.14)mmol/L in the liver cirrhosis group,(0.54 ± 0.05)mmol/L in the liver cancer group.The HDL-C level in liver disease group and hepatitis group,liver cirrhosis group,liver cancer group was significantly lower than that in the control group(t =15.5569,14.2463,45.4393,25.6344,all P ＜ 0.01).The HDL-C level in liver cirrhosis group and liver cancer group was significantly lower than that in hepatitis group (t =3.6583,16.9057,all P ＜ 0.01).The HDL-C level in liver cancer group was significantly lower than that in liver cirrhosis group(t =4.4103,P ＜ 0.01).Conclusion Serum HDL-C level can be used to evaluate synthetic function of the liver,the lower of serum HDL-C level,the more serious liver synthesis function is impaired.

A Comparative Cross-sectional Study of the Development of Hepatocellular Carcinoma in Patients with Liver Cirrhosis Caused by Hepatitis B Virus, Alcohol, or Combination of Hepatitis B Virus and Alcohol / 대한소화기학회지

BACKGROUND/AIMS: Alcohol may be a cocarcinogen in patients with chronic viral hepatitis. We investigated the effect of alcohol on the development of hepatocellular carcinoma (HCC) in liver cirrhosis (LC) caused by hepatitis B virus (HBV). METHODS: All patients with LC or HCC associated with HBV or alcohol, admitted between March 2001 and June 2005, were included. Patients were divided into three groups according to the etiology of LC: Alcohol (AL), HBV, or HBV+alcohol (HBV+AL). Age and laboratory data at the enrollment of study were analyzed. The logistic regression coefficiency for the prevalence of HCC was calculated by using variables such as age, gender, serologic markers, and etiology of LC. RESULTS: In LC patients (n=342), the proportions of AL, HBV, and HBV+AL groups were 44%, 39%, and 17%, respectively. The proportions of HCC in AL, HBV and HBV+AL groups were 17%, 55%, and 76%, respectively. Age at the diagnosis of HCC was younger in HBV+AL than in AL group (p=0.036). In logistic regression analysis for the risk factor of HCC, odds ratio of age was 1.056 (p<0.001). Odds ratios of HBV and HBV+AL group comparing AL were 8.449 (p<0.001) and 17.609 (p<0.001), respectively. Therefore, old age and chronic alcohol intake in patients with HBsAg were the risk factors of HCC. CONCLUSIONS: Chronic alcohol intake may be an additive factor for the development of HCC in patient with LC caused by HBV. However, a prospective cohort study is needed to confirm these findings.

Prevalence and Clinical Impact of a Subclinical Intrapulmonary Right to Left Shunt Diagnosed by Contrast Echocardiography in Patients with End Stage Hepatic Disease

BACKGROUND AND OBJECTIVES: The clinical impact of an intrapulmonary right to left shunt, without hypoxia (subclinical IPS), has not been sufficiently evaluated. We investigated the prevalence and clinical impact of a subclinical IPS in patients with end stage hepatic disease. SUBJECTS AND METHODS: Contrast echocardiography, with hand-agitated saline, was performed in 72 consecutive candidates for a liver transplantation between April 2001 and November 2001. A positive contrast echocardiography indicated an intrapulmonary right to left shunt, and was defined as the delayed appearance of microbubbles in the left side of the heart (3 to 6 beats after the initial appearance in the contrast in the right side of the heart). We compared the clinical events of the patients both with and without a shunt, i.e. death, spontaneous bacterial peritonitis, sepsis, hepatic encephalopathy or variceal bleeding. RESULTS: A subclinical IPS was detected in 19 of the 72 candidates for a liver transplantation (26.3%). All these candidates were Child class C liver cirrhosis. There were no differences in the baseline characteristics between the patients and those without a shunt in the Child class C (n=57). The mean PaO2 value of the patients with at least a 3+ left ventricular opacification (3 to 4+, n=6) was significantly lower than those with a 1+ to 2+ left ventricular opacification (n=13) (76+/-10 mmHg vs. 103+/-13 mmHg, p< 0.05). The mortality was not significantly difference between the patients with (5%, 1/19) or without (21%, 8/38, p=0.24) a shunt. Also, there were no significant differences in the clinical events during the mean follow-up period of 7+/-3 months (68.4% vs. 83.5% p=0.12). CONCLUSION: Subclinical IPS's are not uncommon in patients with end stage hepatic disease. The extent of shunting correlates with the level of arterial oxygenation. However, a subclinical IPS is not associated with the mortality or clinical events of the patient selected.

Mechanism of Ruangan Granule in Counteracting Immune Liver Fibrosis in Rats / 广州中医药大学学报

[Objective] To study the mechanism of Ruangan Granule (RG) in counteracting the experimental liver fibrosis and cirrhosis in SD rats. [Methods] As compared with colchicines, effect of RG in small and large dosages on serum transforming growth factor ? (TGF ?1 ), hydroxyproline (Hyp), collagen Ⅰ and Ⅲ , hepatic function and hepatic pathology were observed in rats. [ Results ] Both RG and colchicines could decrease serum levels of alanine aminotransferase (ALT) and alkaline phosphatase (AKP) and increase serum levels of total protein and albumin in rats with liver fibrosis induced by bovine serum albumin (P 0.05). Effect of large-dosage RG in improving hepatic pathology was better than that of colchicines. [Conclusion] RG has an inhibitory effect on the formation of liver fibrosis and cirrhosis. Its mechanism may be related to the improvement of hepatic function, decrease of serum TGF ft , collagen Ⅰ and Ⅲ and Hyp levels.

Experimental Studies on Ruangan Kangxian Prescription in Preventing Rats Liver Fibrosis Induced by Carbon Tetrachloride / 广州中医药大学学报

[Objective] To investigate the effect of Ruangan Kangxian Prescription (RKP), a prescription mainly composed of Radix Salviae Miltiorrhizae, Radix Paeoniae Rubra, Catechu, Radix Stephaniae Tetrandrae and Polyporus, for rats liver fibrosis induced by carbon tetrachloride (CC14) and to explore its mechanism. [Methods] Rats with liver fibrosis were induced by CC14 and were randomized to RKP group, normal group, Biejia Ruangan Tablet (BRT) group and model group. Serum levels of alanine aminotransferase (ALT), aspartate aminotransferase (AST), hyaluronic acid (HA), laminin (LN) , procollagen Ⅲ (PCⅢ ) were detected; hepatic histopathology and immunohistochemical result of transforming growth factor ?1 (TGF ?1 ) were also examined. [ Results ] Death rate of rats was reduced; serological parameters of hepatic function and liver fibrosis were improved and liver fibrosis showed by immunohistochemical method, HE staining and Masson staining was inhibited in RKP and BRT groups (P 0.05). [ Conclusion ] RKP and BRT have the similar effect in preventing CC14-induced liver fibrosis in rats and its mechanism may be related to the counteraction of expression of TGF ?1 .

Blood bilirubin determinations as an aid in the differentiations between peptic ulcer and portal cirrhosis in gastric hemorrhage

1. Seven cases with massive gastric hemorrhage resulting in profuse hematemesis form the basis of this report. Four of these were admitted in the Medical Wards of the Philippine General Hospital, two were patients admitted in the Pay Section of the same Hospital, and the sixth was an American internee admitted in a special ward for internees of the same hospital2. Four of these patients died, and a complete pathologic report for each was available. Two were operated on and accurate operative findings were also available for these patients. One had no operative or autopsy verification of the clinical impression. But this appears well supported by clinical laboratory and X-ray findings3. Four of these patients were found suffering from peptic ulcer and the other three from portal chirrhosis. The patients with peptic ulcer showed practically no bilirubin in the blood (Whether B-I or B-II) while those with portal cirrhosis showed significant amounts of B-I4. When clinical, laboratory , or x-ray findings are not sufficiently distinct for the differentiation of these two conditions in cases of massive gastric hemorrhage, the bilirubin (B-I) content of the blood may help in the differentiation. Peptic ulcer is not accompanied by B-I in the blood, while portal cirrhosis is always accompanied by functional disturbances of the liver. This is shown by the constant presence of B-I in the blood. (Conclusion)

Parasitic cirrhosis among Filipinos

1. The single leading cause of death among 81 autopsied cases of parasitic cirrhosis caused by Schistosoma Japonica was massive upper gastrointestinal hemorrhage from ruptured gastroesophageal varices. Only one patient died from hepatic insufficiency. The changes seen in the liver of autopsy were those of fibrosis of the portal tracts, with very minimal parenchymal damage and distortion of the lobular architecture2. The clinical features, liver function tests, hepatic wedge and splenic pulp pressures, splenoportography, and liver biopsies of 16 patients with parasitic cirrhosis secondary to Schistosoma Japonica were reviewed. Clinically, these patients presented the problem of portal hypertension. Standard liver function tests revealed very insignificant impairment of parenchymal hepatic function. The wedged hepatic vein pressures were found to be normal, while the splenic pulp pressures were markedly elevated. A significant pressure gradient between the splenic pulp and hepatic wedge pressures was demonstrated. Splenoportography revealed evidences of portal hypertension, with no obstruction of the extrahepatic portal venous system. Liver biopsy showed deposition of Schistosoma ova with varying degrees of portal and periportal fibrosis3. A correlation between the clinical, hemodynamic and morphologic changes in parasitic cirrhosis was made. The morphologic aberration in parasitic cirrhosis was demonstrated to be fibrosis of the intrahepatic radicles of the portal vein, resulting in intrahepatic presinusoidal portal hypertension. There was very minimal damage to the liver cells, and hence hepatic perenchymal function remained essentially intact. (Summary)