We developed a low-intensity 10-min resistance exercise program for nonalcoholic fatty liver disease (NAFLD). We report a case of NAFLD with elevated hepatic fibrosis indices, which were improved by a 60-week daily exercise program. A 71-year-old female patient with NAFLD whose hepatic fibrosis stage corresponded to F2 was referred to our hospital. She performed the exercise once a day with no changes in other lifestyle habits and medications. The homeostasis model assessment-insulin resistance value and NAFLD-liver fat score, the Hepamet fibrosis score, and the enhanced liver fibrosis score decreased. The FIB-4 index and serum levels of Mac-2 binding protein glycosylation isomer decreased to the reference values. We investigated the changes in chemokines/cytokines. The serum granulocyte-colony stimulating factor level was increased, and serum interferon-gamma-induced protein-10 and platelet-derived growth factor-BB levels were decreased. Our program may be beneficial for improving hepatic fibrosis in patients with NAFLD.

Hepatocyte nuclear factor 1B (HNF1B) is a member of the homeodomain-containing family of transcription factors located on 17q12. HNF1B deficiency is associated with a clinical syndrome (kidney and urogenital malformations, maturity-onset diabetes of the young, exocrine pancreatic insufficiency) and to an underdiagnosed liver involvement. Differently from HNF1A, the correlation between hepatocellular carcinoma (HCC) and germline HNF1B deficiency has been poorly evaluated.
Here, we report a novel case of a syndromic HNF1B-deficient paediatric patient that developed HCC with unique histopathological features characterised by neoplastic syncytial giant cells, which was observed only in one additional case of paediatric cholestatic liver disease of unknown origin.
Our case highlights the influence of HNF1B deficiency in liver disease progression and its putative association with a rare yet specific HCC histotype. We hypothesised that HCC could be secondary to the repressive effect of HNF1B variant on the HNF1A transcriptional activity.

暂无摘要。

Polysplenia syndrome represents a type of left atrial isomerism characterized by multiple small spleens, often associated with cardiac malformations and with situs ambiguus of the abdominal organs. The case presented is of a one-month-old male infant, weighing approximately 3000 g, born at the County Clinical Emergency Hospital of Sibiu, who was hospitalized from birth until death. The patient suffered cardio-respiratory arrest due to severe hypoxia and septicemia on the background of a series of complex cardiac malformations associated with congenital abdominal organ anomalies. Examination of the body revealed a common atrium with complete atrioventricular canal defect, left ventricular hypertrophy, right ventricle hypoplasia, truncus arteriosus, superior vena cava duplication, bilobation of the lungs, situs ambiguous of the abdominal organs with right-sided stomach, a midline liver, gall bladder agenesis, multiple right-sided spleens and complete inversion of the intestines and pancreas. Histopathology concluded that the patient suffered cardiac lesions consistent with infantile lactic acidosis, as well as pulmonary modifications suggesting congenital alveolar dysplasia and altered hepatic architecture compatible with fibrosis.

UNASSIGNED: To evaluate the clinical significance of Mac-2 binding protein glycosylation isomer (M2BPGi), we investigated the relationship between M2BPGi and clinicopathological and surgical parameters and posthepatectomy complications.
UNASSIGNED: We examined M2BPGi in 115 patients with hepatic malignancies undergoing hepatectomy. Significance as an independent prognostic marker was determined with multivariate logistic regression analysis.
UNASSIGNED: The mean serum M2BPGi level was 1.14 ± 1.03 C.O.I. (range 0.2-5.79). M2BPGi in the chronic viral hepatitis group (1.42 ± 1.25) was significantly higher than that in the other disease groups (p < 0.05). The M2BPGi level correlated negatively with platelet count, LHL15 and GSA-Rmax (r = -0.36, -0.69 and -0.56, respectively; p < 0.01) but correlated positively with serum hyaluronate level (fibrotic marker), ICGR15 and HH15 (r = 0.52, 0.63 and 0.57, respectively; p < 0.01). In 53 patients examined for histological hepatic fibrosis, the M2BPGi level was highest for hepatic fibrosis stage 4, indicating cirrhosis (2.15 ± 1.56), and was significantly higher than that for stages 0-2 (p < 0.05). M2BPGi level did not correlate significantly with any surgical parameters. The preoperative level correlated significantly only with increased alanine aminotransferase level (r = -0.21, p < 0.05) and was significantly higher in patients with (1.35 ± 0.78) than without (1.11 ± 1.07) hepatectomy-related complications (p < 0.05). Area under the ROC curve analysis for prediction of hepatic fibrosis score 4 showed a cut-off value of 0.78 for M2BPGi to have high sensitivity (90%) and specificity (58%). For postoperative hepatectomy-related complications, only the M2BPGi level (at a cut-off value 0.90) tended to show significance (p = 0.06).
UNASSIGNED: The non-invasively measured serum level of M2BPGi reflected impaired liver function or cirrhosis and hepatectomy-related complications after surgery, making it potentially useful as a complementary parameter accompanying other liver function parameters.

We describe the first case of biliary cirrhosis in Japanese macaque. Clinical signs had not been detected. The liver was nodular. Histopathologically, portal-to-portal pattern of fibrosis might have indicated chronic cholestasis. Fibrotic septa were infiltrated with inflammatory cells. Therefore, this case could be diagnosed as active incomplete biliary cirrhosis.

Schistosomiasis is a parasitic disease caused by Schistosoma species. Intestinal and hepatic schistosomiases are the most common forms of chronic disease. We describe a case of a 26-year old patient from Eritrea who was referred to our hospital with abdominal pain and diarrhea. The diagnosis of hepatosplenic schistosomiasis was made by liver biopsy and the patient was treated with praziquantel. Hepatic schistosomiasis is characterised by deposition of schistosomal eggs in the liver which results in a host cell immune response and leads to granuloma formation and neoangiogenesis. This is hallmarked by different grades of periportal fibrosis with portal hypertension leading to splenomegaly. Normal liver architecture is preserved and periportal fibrosis can be reversible if treated adequately and timely. With a recent native schistosomiasis cluster report from France and the expected influx to Europe of persons from regions endemic for schistosomiasis, increased awareness of this disease in healthcare practitioners is needed. We review the epidemiology, pathogenesis, clinical presentation and treatment of schistosomiasis.

BACKGROUND: This study aimed to investigate the association between non-alcoholic steatohepatitis (NASH) and intrahepatic cholangiocarcinoma (ICC).
METHODS: This was a case control study of patients who underwent surgical resection either for ICC or for a metastatic liver tumor (the control group). We assessed their clinical characteristics, pathological findings, and the prevalence of known ICC risk factors. For patients without known risk factors, we compared other factors including the prevalence of NASH.
RESULTS: In the patients without known risk factors, 15 of 34 patients in the ICC group and 13 of 69 patients in the control group were diagnosed with NASH. Univariate analysis showed significantly higher values in the ICC group for age (P = 0.0478), prevalence of obesity (P = 0.0365) and NASH (P = 0.0078), and serum levels of albumin (P = 0.0051), and gamma-glutamyl transpeptidase (γ-GTP) (P = 0.0006) compared with the control group. Multivariate analysis showed that age and serum levels of γ-GTP and NASH were independent risk factors for ICC. In patients with NASH, the proportion of patients with hepatic fibrosis was significantly higher in the ICC group than in the control group (P = 0.0014).
CONCLUSIONS: NASH is a possible risk factor for ICC development. J. Surg. Oncol. 2016;113:779-783. © 2016 Wiley Periodicals, Inc.

暂无摘要。

Hepatic involvement in aggressive systemic mastocytosis (ASM) is relatively common, and the main clinical features of this disease include hepatomegaly, portal hypertension, ascites, and fibrosis. Cirrhosis is a rare ASM symptom. We report an ASM case that initially mimicked cirrhosis based on clinical and radiographic analyses. The portal tract was expanded by mononuclear inflammatory cells, and an increase in collagen amount was observed in routine histological sections of the biopsied liver. A diagnosis of systemic mastocytosis (SM) was made after ancillary tests for mast cells using bone marrow aspirates. Extensive involvement of the liver and gastrointestinal tract was observed. Clinicians and pathologists need to consider ASM as a diagnosis or differential diagnosis in a clinical case of cirrhosis with unknown etiology. The diagnosis can be confirmed or disregarded by immunohistochemical staining and molecular analysis.