Vol. 4 Issue 4
On the cover: Official Journal of the Mexican Association of Hepatology
Recent findings have led to major advances in our understanding of genetics and pathophysiology of hereditary hemochromatosis. Many crucial genes and molecules have come to light, and the complex interrelationships between them are being studied. However, several questions still remain unanswered. Availability of genotyping has changed the approach to diagnosis, and serum markers hold promise for prognostication. However, the effectiveness of population screening continues to be an area of controversy. Finally, there is a promise of development of newer therapeutic modalities based on our understanding of the mechanism of iron absorption. In this review, we describe the current state of understanding in the clinical features, pathophysiology and treatment of hereditary hemochromatosis.
Despite the public concern about the controversial use and abuse of marijuana, the scientific community has focused on the therapeutic potentials of cannabinoid compounds and had highlighted the importance of endocannabinoids and their receptors in physiology and disease. Endocannabinoids have been shown to be important mediators in neuroendocrine and psychiatric processes such as food intake, drug reward and energy metabolism. Cannabinoid receptors are expressed by several cell lines in the liver, such as hepatocytes, myofibroblastic cells, endothelial cells and probably cholangiocytes. A perpetuating role in liver damage for the endocannabinoid system has been proposed in several steps of chronic liver disease progression. Being a major cause of death worldwide, chronic liver disease is an important problem. New therapies are needed in order to stop or slow damage progression. This review summarizes the results of experimental studies involving the endocannabinoid system in liver disease and their clinical and therapeutical implications in hepatology.
Introduction: Autoimmune type chronic active hepatitis (AI-CAH) is a rare pediatric disorder whose principal characteristics include hepatocellular dysfunction and active tissue damage with evolution to cirrhosis in 25–30% of cases. Objective: Our aim was to ascertain the evolution of 23 children with AI-CAH treated between 1978 and 2004 at the Hospital Infantil del Estado de Sonora in the Sonora State capital of Hermosillo in northern Mexico. Materials and methods: We conducted a retrospective review that included the following variables: age; sex; personal antecedents; signs and symptoms; laboratory and medical office studies; histologic tissue pattern obtained by biopsy; treatment; evolution, and mortality. Results: Thirteen males and 10 females participated in the study; predominant signs were hyporexia, fatiguehepatomegalia, and icterus. Ten patients presented moderate anemia and six patients, leukopenia and thrombocytopenia. Eighteen patients presented hypergammaglobulinemia. Twenty three patients had percutaneous (p.c.) liver biopsy, and we observed the following histologic pattern: rupture of the limiting plate; necrotic foci; cholangiolar proliferation, and fibrotic bridges; in addition, four patients demonstrated precirrhotic changes. Twenty one children received prednisone for periods of between 1 and 12 years; in seven cases, children were administered azatioprine. Six children presented histologic, biochemical, and clinical remission, nine experienced biochemical remission but presented inflammatory activity, five abandoned treatment, and three died due to cirrhosis, liver insufficiency, and other complications. Discussion: To date, AI-CAH etiology continues to be unknown, while treatment has not been importantly modified in 50 years and continues to consist of prednisone alone or in association with azathioprine.
The Liver is one of the organs involved in the multiorgan failure that occurs in sickle cell disease, the pathophsiology of liver disease in this condition is complex because of the interrelated multifactorial causes. Liver dysfunction was assessed in both paediatric and adult sickle cell disease patients in the steady state. The transaminases and alkaline phosphatase were analysed by automation while coagulation studies were done manually. The mean (range) of Alanine transaminase (ALT), Aspartate transaminase (AST) and alkaline phosphatase (ALP) were 23.0 (2-77) IU, 48.5 (15-120) IU, 227.5 (37-1200) IU respectively. ALT and AST levels were less than 100 IU in over 95% of the patients. The gender or age of the patients did not significantly affect the level of these three enzymes. There was close association between the liver size and elevation of the liver enzymes except for alkaline phosphatase (ALT = .017, AST = .009, ALP = .056). Twenty-five percent of the patients had normal enzymes while 13% had derangement of the three enzymes, 19%, 50% and 74% had abnormal ALT, AST and ALP respectively. Only 22% and 5% had deranged PT and APTT respectively. In conclusion minimal elevation of the tramsaminases which is not gender or age dependent were observed in steady state sickle cell disease, higher levels of alkaline phosphatase may be due to associated vasoocclussive crises involving the bones rather than a pathology of the liver.
Our aims were to validate the Brazilian Portuguese version of the Liver Disease Quality of Life Questionnaire instrument (LDQOL1.0) and evaluate this tool in non-cirrhotic patients. Methods: The LDQOL1.0 consists of the SF-36 generic measure of health-related quality of life (HRQOL) and 12 disease-specific dimensions for patients with liver disease. The Brazilian Portuguese version instrument was administered to 103 patients with liver disease. Reliability was analyzed by studying the internal consistency of the individual disease- specific dimensions of the LDQOL, as well as by studying the test-retest reliability in a sub-group of 74 patients who completed the questionnaire on two occasions 1 to 2 weeks apart. Validity was analyzed by determining the instrument's ability to discriminate between groups of patients according to severity of the liver disease. Results: Internal consistency in the disease- specific dimensions was good (CACs 0.66-0.94), as well as test-retest reliability in all dimensions (ICCs of 0.64-0.96) p < 0.05. We found statistically significant differences in six domains when compensated cirrhotic patients, Child A, were compared with non-cirrhotic patients. We also found statistically significant differences when we compared cirrhotic patients evaluated assessed by Child-Pugh and Meld classifications. Patients with Child C and Meld ≥ 15 showed worse quality of life. Moderate ceiling and floor effects were found in some disease-specific dimensions. In Conclusions: the Brazilian Portuguese version of the LDQOL 1.0 is very useful for measuring HRQOL in liver diseases.
Background: Liver is the most common site of infection and several methods of surgery have been described to treat this common disease. In this study we aim to compare the results of two common methods of surgery; simple drainage versus omentoplasty. Methods: In this prospective study 65 patients with hepatic hydatid cyst underwent surgery from 10 May 1995 to 1 July 2002. 35 patients were treated with omentoplasty (group I) and 30 of them were treated with drainage (group II). The results of surgery including mortality, complications and recurrences were recorded. Results: There was no case of mortality in each group of patients. Postoperative complications were seen in 5.7% of group I, 16.6% of group II patients. The mean duration of hospital stay was 6.5 and 15.6 days in group I and group II patients. During a mean period of 18.6 month follow up there was no recurrence in each group of patients. Conclusion: According to the results of this study we suppose that omentoplasty of cyst cavity –if feasible– is preferred to tube drainage.
Nucleic acid-amplification testing (NAT) is not routinely practiced in blood banks from most low-income countries. We did an exploratory comparison of the performance of the standard immunoassay-based screening tests for the hepatitis B (HBV) and C (HCV) viruses with that of NAT, in blood donors. From January 1999 to March 2005, 94,806 blood donors were screened for anti-HCV antibodies and for hepatitis B surface antigen (HBsAg). Also, an exploratory period of molecular screening was carried out on 100 consecutive blood donors to detect HBV DNA and HCV RNA by home-made PCR techniques without sera pooling. In the 75-month period of serologic screening, HBsAg was detected in 219 donors (0.23%; 95% CI, 0.20– 0.26%) and anti-HCV antibodies in 922 (0.97%; 95% CI, 0.90–1.03%). The annual trend for HBsAg prevalence had a decreasing pattern over the years (p < 0.001), whereas that for anti-HCV did not (p = 0.19). In the molecular screening cohort, HBV DNA was detected in one donor (1%; 95% CI, 0–6%) and HCV RNA in another (1%; 95% CI, 0–6%). All these 100 donors tested negative to HBsAg and anti-HCV. Thus, the prevalence of positive results for HBV and HCV did not differ if considering immunoassays or NAT; nevertheless, these methods did not coincide in detecting HBV or HCV in the molecular screening cohort. In conclusion, NAT can detect cases of HBV and HCV infections that standard immunoassay techniques can not, even in a highly selected population at low risk, like blood donors. Large-scale studies are warranted for NAT to be considered as a systematic method for screening of HBV and HCV in Mexican blood banks.
Background: Elevated ALT is an indirect marker of NAFLD in patients with non-alcohol abuse and without other known causes of chronic hepatitis. Obesity, type 2 diabetes and some dyslipidemias are associated to this condition. The purpose of this study was to determine the frequency of increased aminotransferases and associated metabolic anomalies among overweight and obese children. Methods: Children from an elementary school with obesity or overweight were included. Medical history and anthropometrics measurements were recorded and serum liver function tests, lipid profile, glucose and insulin levels, and HOMA index were determined. NAFLD diagnosis was considered in those children with ALT > 40 U/L and AST/ALT ratio < 1 after exclusion of other causes of chronic hepatitis. Results: Increase ALT levels (> 40 U/L) were found in 34/ 80 (42%) obese and overweight children; mean age was 9.5 ± 1.1 years and mean BMI of 25.8 ± 3. The metabolic abnormalities in the study group were similar, there were no differences in insulin concentration, insulin resistance determined by HOMA-IR Index, serum lipid profile and serum glucose between children with or without increased ALT. Conclusions: The frequency (42%) of elevated ALT levels in children with exces body weight in this study was greater to those reported in other pediatric populations. There were no differences among the metabolic alterations with or without increased ALT; these findings support that the principal pathogenic factor involved in the development of the hepatic injury may be located in the liver.
Portal biliopathy is a rare condition that is usually not diagnosed and only in few cases causes symptoms. Those symptoms are caused by vascular obstruction of the biliary tree in patients with portal hypertension. We report a case of a 29 years man who presented with history of intermittent jaundice, persistent elevation of hepatic function test and hematemesis as a manifestation of portal hypertension without liver damage. We present the clinical, radiological and pathological characteristics and literature review of the cases that had been reported, their diagnoses, treatment and clinical implication.