Hepatic hemobilia: Hemorrhage from the intrahepatic biliary tract,a review

Hepatic hemobilia is defined as hemorrhage arising from pathological changes in the intrahepatic biliary tract. The main causes are iatrogenic trauma, cholangitis, tumors, and coagulopathy. The salient features of the hemobilia syndrome are described and their causes explained. The treatment, when necessitated by hemorrhage or clot formation, is either resection of the liver or occlusion of the responsible artery by ligature or embolization. The iatrogenic trauma may be operative, resulting from instrumental lesion of the bile ducts, needle biopsy, transhepatic cholangiography, biliary tract prosthesis, or inlaying hepatic artery catheters. Among the inflammatory etiologies, special attention is given to nematodes in the ducts, the tropical hemobilia. Spontaneous hemobilia may, just as nose bleeds or hematuria, result from treatment with anticoagulants.

---

Résumé L'hémobilie d'origine hépatique répond à l'hémorragie qui provient de lésions situées au niveau des voies biliaires intra-hépatiques. Les causes principales en sont le traumatisme, l'angiocholite, les tumeurs et les troubles de la coagulation. Les caracteres saillants de l'hémobilie sont décrits ainsi que ses causes. Le traitement quand il devient nécessaire en raison de l'importance de l'hémorragie ou de la formation de caillots consiste à réséquer une partie du parenchyme hépatique ou à obtenir l'occlusion de la plaie vasculaire par ligature ou par embolisation.Le traumatisme peut être d'origine iatrogène, qu'il soit le fait d'une lésion instrumentale des canaux biliaires hépatiques, d'une biopsie du foie à l'aiguille, de la cholangiographie transhépatique, ou encore de la présence d'une prothèse au niveau des voies biliaires ou d'un cathéter artériel.Parmi les causes inflammatoires une attention spéciale doit être accordée à l'existence de nématodes au niveau des canaux biliaires intra-hépatiques qui sont à l'origine de l'hémobilie tropicale.L'hémobilie spontanée, comme l'épistaxis ou l'hématurie, peut être la conséquence d'un traitement anti-coagulant.ResumenLa hemobilia hepática se define como hemorragia proveniente de alteraciones patológicas en el tracto biliar intrahepatico. Las causas principales son el trauma iatrogénico, la colangitis, los tumores y la coagulopatía. Se describen las características sobresalientes del síndrome de hemobilia y se explican sus causas. El tratamiento, cuando se hace necesario por hemorragia o por formación de coágulos, es la resección del hígado o la oclusión de la arteria responsable por ligadura o por embolización. El trauma iatrogénico puede ser de origen operatorio, como resultado de lesión instrumental de los canales biliares, biopsia por aguja, colangiografía transhepática, prótesis en el tracto biliar y catéteres colocados en la arteria hepática. Entre las etiologías de naturaleza inflamatoria se presta atención especial a la presencia de nemátodos en los canales, la hemobilia tropical. Al igual de lo que ocurre con las epistaxis o las hematurias, la hemobilia espontanea puede ser consecuencia de tratamientos con anticoagulantes.

     

Emergency portacaval shunt for control of hemorrhage from a parenchymal fracture after adult-to-adult living donor liver transplantation

As more adults undergo transplantation with partial liver grafts, the unique features of these segments and their clinical significance will become apparent. A patient presented with life-threatening hemorrhage from an iatrogenic laceration to a right lobe graft 11 days after transplantation. The creation of a portacaval shunt effectively controlled the bleeding, allowing more elective replacement of the organ with another right lobe graft. The regeneration process combined with increased portal blood flow and relative outflow limitation may have set the stage for this complication. Any disruption of the liver parenchyma during transplantation should be securely repaired and followed cautiously. Portacaval shunting is an option for controlling hemorrhage from the liver in transplant recipients. The timely availability of a second organ was likely the ultimate determinant of survival for this patient.     

Superoxide dismutases in chronic gastritis

Human gastric diseases have shown significant changes in the activity and expression of superoxide dismutase (SOD) isoforms. The aim of this study was to detect Mn‐SOD activity and expression in the tissue of gastric mucosa, primarily in chronic gastritis (immunohistochemical Helicobacter pylori‐negative gastritis, without other pathohistological changes) and to evaluate their possible connection with pathohistological diagnosis. We examined 51 consecutive outpatients undergoing endoscopy for upper gastrointestinal symptoms. Patients were classified based on their histopathological examinations and divided into three groups: 51 patients (archive samples between 2004–2009) with chronic immunohistochemical Helicobacter pylori‐negative gastritis (mononuclear cells infiltration were graded as absent, moderate, severe) divided into three groups. Severity of gastritis was graded according to the updated Sydney system. Gastric tissue samples were used to determine the expression of Mn‐SOD with anti‐Mn‐SOD Ab immunohistochemically. The Mn‐SOD expression was more frequently present in specimens with severe and moderate inflammation of gastric mucosa than in those with normal mucosa. In patients with normal histological finding, positive immunoreactivity of Mn‐SOD was not found. Our results determine the changes in Mn‐SOD expression occurring in the normal gastric mucosa that had undergone changes in the intensity of chronic inflammatory infiltrates in the lamina propria.     

Clinical and histologic spectrum of nonalcoholic fatty liver disease associated with normal ALT values

A retrospective study was performed to (1) characterize the clinical and histologic features of those with nonalcoholic fatty liver disease (NAFLD) and normal alanine aminotransferase (ALT) values, (2) compare the spectrum of NAFLD associated with normal versus elevated ALT levels, and (3) determine whether there were differences in the clinical or histologic spectrum of NAFLD between those with a low normal versus high normal ALT value. A total of 51 subjects with NAFLD and normal ALT were identified and compared with 50 consecutive subjects with NAFLD and elevated ALT. The major indications for liver biopsy in those with normal ALT were unexplained hepatomegaly (n = 21) and evaluation as a potential donor for living donor liver transplantation (n = 16). The 2 groups were comparable with respect to age, gender distribution, and ethnicity. Approximately 80% of cases in both groups had at least 1 feature of the metabolic syndrome, the major risk factor for NAFLD. The 2 groups were also comparable with respect to the grade of the individual histologic parameters of NAFLD. A total of 12 subjects with normal ALT levels had bridging fibrosis, whereas 6 had cirrhosis. Diabetes was the only factor independently associated with an increased risk of advanced fibrosis (bridging fibrosis or cirrhosis) by multivariate analysis (relative risk: 2.3, P <.01). The mean steatosis (1.6 vs. 2.16, P <.04) and perisinusoidal fibrosis scores (0.35 vs. 0.9, P <.049) were lower in those with low normal (<30 IU/L) ALT versus high normal ALT. However, the prevalence of advanced fibrosis was similar (5 of 15 vs. 13 of 36, respectively). In conclusion, (1) the entire histologic spectrum of NAFLD can be seen in individuals with normal ALT values, (2) the histologic spectrum in these individuals is not significantly different from those with elevated ALT levels, and (3) a low normal ALT value does not guarantee freedom from underlying steatohepatitis with advanced fibrosis.     

Chronic hepatitis C infection in patients with end stage renal disease: characterization of liver histology and viral load in patients awaiting renal transplantation

OBJECTIVES: Hepatitis C virus (HCV) is common in patients with end stage renal disease (ESRD) awaiting renal transplantation (RT). However, few data are available on the liver histology and viral titer in these patients relative to patients with HCV and normal renal function. The aims of this study were to assess liver histology, quantitative HCV-RNA titer, and alanine aminotransferase (ALT) levels in patients with ESRD awaiting RT, and to identify clinical predictors of histological progression to advanced bridging fibrosis and/or cirrhosis. METHODS: A total of 50 consecutive patients (mean age 42 yr, 62% male) with ESRD and HCV, who were awaiting RT, underwent liver biopsy. Two HCV populations, one with persistently normal ALT and another with elevated ALT, both with normal renal function, served as controls. HCV-RNA titer was assessed by quantitative PCR. RESULTS: Of the patients with ESRD, 94% had normal ALT. Log HCV RNA titer was significantly higher in patients with ESRD (5.8+/-0.3) than in either normal ALT (5.4+/-0.1) or elevated ALT (5.3+/-0.1) controls (p < 0.05). Knodell Histological Activity Index (HAI) in patients with ESRD was similar to that observed in control patients with normal ALT (4.8+/-0.4 vs 4.9+/-0.4) but significantly less (p < 0.05) than that observed in control patients with elevated ALT (8.4+/-0.5). The percentage of patients with bridging fibrosis or cirrhosis was similar in patients with ESRD and controls with persistently normal ALT (22% vs 13%) but significantly less (p < 0.001) than that observed in control patients with elevated ALT (48%). No significant differences in ALT, HCV-RNA titer, duration on hemodialysis, or time from first possible exposure was observed between ESRD patients with advance fibrosis (n = 11) and those with mild disease (n = 39). CONCLUSIONS: Our data suggest that liver biopsy is necessary to exclude significant liver pathology in patients with HCV and ESRD, and to help define those patients in whom interferon treatment might be helpful.     

Nutrition and bone health in children and adolescents

Bone accretion during childhood is proportional to the rate of growth. During this time, interval height velocity is relatively slow for both boys and girls. As a direct consequence of this, calcium retention in the body of an average child is lower than the calcium retention in an adolescent. Bone size, bone mass, and bone mineral areal density of the regional skeletal sites increase on average by about 4%/yr from childhood to late adolescence and young adulthood, when most of the bone mass is accumulated. Calcium needs are greater during adolescence (pubertal growth spurt) than in childhood or adulthood. According to calcium balance studies, the threshold in take for adolescents is about 1500 mg/d. Inadequate calcium intake during growth may increase the risk of childhood fractures and predispose certain individuals to a lower peak bone mass.     

Genomic diagnostics in polycystic kidney disease: an assessment of real-world use of whole-genome sequencing

Autosomal Dominant Polycystic Kidney Disease (ADPKD) is common, with a prevalence of 1/1000 and predominantly caused by disease-causing variants in PKD1 or PKD2. Clinical diagnosis is usually by age-dependent imaging criteria, which is challenging in patients with atypical clinical features, without family history, or younger age. However, there is increasing need for definitive diagnosis of ADPKD with new treatments available. Sequencing is complicated by six pseudogenes that share 97% homology to PKD1 and by recently identified phenocopy genes. Whole-genome sequencing can definitively diagnose ADPKD, but requires validation for clinical use. We initially performed a validation study, in which 42 ADPKD patients underwent sequencing of PKD1 and PKD2 by both whole-genome and Sanger sequencing, using a blinded, cross-over method. Whole-genome sequencing identified all PKD1 and PKD2 germline pathogenic variants in the validation study (sensitivity and specificity 100%). Two mosaic variants outside pipeline thresholds were not detected. We then examined the first 144 samples referred to a clinically-accredited diagnostic laboratory for clinical whole-genome sequencing, with targeted-analysis to a polycystic kidney disease gene-panel. In this unselected, diagnostic cohort (71 males :73 females), the diagnostic rate was 70%, including a diagnostic rate of 81% in patients with typical ADPKD (98% with PKD1/PKD2 variants) and 60% in those with atypical features (56% PKD1/PKD2; 44% PKHD1/HNF1B/GANAB/ DNAJB11/PRKCSH/TSC2). Most patients with atypical disease did not have clinical features that predicted likelihood of a genetic diagnosis. These results suggest clinicians should consider diagnostic genomics as part of their assessment in polycystic kidney disease, particularly in atypical disease.Subject terms: Genetics research, Polycystic kidney disease, Genetic testing