35 results about "PRIMARY BILIARY ATRESIA" patented technology

The invention relates to a serum marker MMP-7-based biliary atresia diagnosis kit. The diagnosis kit comprises an anti-human MMP-7 monoclonal antibody coated ELISA plate, a negative control solution,a positive control solution, an enzyme labeling reagent, an enzyme substrate solution, a blocking solution, a sample diluent, a washing solution and a stopping solution. The diagnosis kit is a new sensitive, safe, reliable and easily-operated commercial kit. Quantitative detection of the level of MMP-7 in human serum is helpful for early diagnosis of BA; the BA diagnosis sensitivity of the serum biomarker protein MMP-7 is 100%, and the BA diagnosis specificity is 95.6%, so the kit has the characteristics of high specificity and high sensitivity; and the kit improves the early diagnosis rate ofBA, reduces misdiagnosis, and improves the self liver survival rate of the BA.

The invention relates to applications of serum taurochenodeoxycholic acid and chenodeocycholic acid as diagnostic markers in the preparation of medical instruments for diagnosing biliary atresia of newborns. The diagnostic markers of the medical instruments are content ratio of the taurochenodeoxycholic acid and the chenodeocycholic axid in serum. The invention also provides the application of chenodeocycholic acid. The application of diagnostic markers in biliary atresia of the newborns has the advantages that the application of the taurochenodeoxycholic acid and the chenodeocycholic acid as diagnostic markers of biliary atresia is proposed for the firstly time, so that the biliary atresia can be distinguished from other cholestasis diseases of newborns, the best operation time of children can be strived for, and therefore, the operation effect can be improved.

The invention relates to an alpha crystal form of obeticholic acid and a preparation method thereof. The preparation method comprises the following steps: (a) fully dissolving obeticholic acid under a backflow state in a mixed solvent of toluene or toluene and another kind of solvent forming a homogeneous system; (b) cooling the obtained obeticholic acid solution, so the alpha crystal form of the obeticholic acid precipitates out from the solution; (c) separating the alpha crystal form of the obeticholic acid out from the solution; and (d) placing the alpha crystal form of the obeticholic acid under 30-70 DEG C and drying for 3-30 hours at reduced pressure or ordinary pressure. The prepared alpha crystal form of obeticholic acid has the characteristics of better stability, higher purity, stable moisture content and the like. Furthermore, the invention also relates to a medicine composition of the alpha crystal form and the application of the alpha crystal form in preparing medicines for treating hepatic diseases such as biliary atresia, cholestatic liver disease, chronic liver disease, primary biliary cirrhosis, alcoholic fatty liver, non-alcoholic fatty liver disease and the like.

The invention discloses an efficient amplifying and culturing method for biliary epithelial cells in rabbit livers and belongs to the technical field of biological medicines. The efficient amplifying and culturing method comprises the following steps of: (1) separating, purifying and culturing the epithelial cells; (2) detecting cell growth lines; and (3) identifying the epithelial cells. The efficient amplifying and culturing method for the biliary epithelial cells, disclosed by the invention, has the advantages as follows: through a multi-step separating method, biliary epithelial cells with higher purities are obtained and a long-time multiplication culturing system for the biliary epithelial cells is established; a fact that the multiplication of the biliary epithelial cells in the livers can be obviously promoted through an Rho kinase inhibitor Y-27632 is first found; and the efficient amplifying and culturing method for the biliary epithelial cells, disclosed by the invention, has the advantages of simpler operation and the like, is suitable for promotion and application and can provide a platform for researches on a plurality of disease mechanisms including biliary atresia, primary biliary cirrhosis and the like.

The invention relates to a construction method of an animal model for biliary atresia of chronic liver disease firstly utilizing a Gr-1 antibody to construct the construction method of the animal model for biliary atresia of chronic liver disease. The method comprises following steps: injecting rhesus monkey rotavirus within the 24 hours of neonatal mouse to induce a classic animal model for biliary atresia, firstly injecting the Gr-1 antibodies before rhesus monkey rotavirus is injected; injecting the Gr-1 antibodies multiple times after mouse are born on the 14th day to maintain the effective concentration range of the Gr-1 antibodies in the mouse; and injecting 5-20ng / 1.6g of Gr-1 antibodies to the mouse each time. The construction method of an animal model for biliary atresia of chronic liver disease has following beneficial effects: the model is capable of simulating the process of biliary atresia, development and prognosis, especially in the end-stage hepatocirrhosis phase.

The invention discloses a primer set for detecting plasma miR-140-3p of a child patient with biliary atresia. The primer set comprises a reverse transcription primer shown in SEQ ID NO:1, a forward upstream primer as shown in SEQ ID NO:2, and a reverse primer as shown in SEQ ID NO:2. The invention further provides a detection kit containing the primer set. The primer set and the kit of fluorescentquantitative PCR for detecting the miR-140-3p, provided by the invention, have high specificity and sensitivity, and can be applied to rapid and specific detection of the miR-140-3p content in plasmain the fluorescent quantitative PCR.

The invention provides application of CD177 in preparation of a product for diagnosing biliary atresia, and relates to the technical field of molecular diagnosis. According to the invention, by detecting a large number of biliary atresia child patient samples and combining animal experiments, it is proved that the number of CD177+ neutrophile granulocytes in biliary atresia peripheral blood and liver tissue can serve as a powerful index for evaluating the liver injury degree at first; and therefore, detection of CD177 genes, CD177 protein or the CD177+ neutrophile granulocytes can serve as a more convenient, direct and accurate method for clinically evaluating the illness state of biliary atresia child patients. The invention also proves that CD177 has a pro-inflammatory effect in biliaryatresia; and the inhibitor of CD177 can be used for research and development of drugs for treating biliary atresia.

The invention provides a biliary atresia evaluation system, a method for detecting MMP-7, a kit and application. The biliary atresia assessment system is a biliary atresia risk assessment system based on MMP-7 and bile acid spectra, and the biliary atresia assessment system takes the concentration of MMP-7 in a subject sample and the concentration of bile acid in the subject sample as input and takes a risk assessment result as output. By adopting the technical scheme, the infantile biliary atresia can be conveniently, efficiently and accurately evaluated, screened and / or diagnosed, the diagnosis accuracy of the biliary atresia is remarkably improved, and non-invasive accurate diagnosis is realized.

The invention belongs to the field of biological medicine and molecular biology, relates to a novel biliary atresia diagnostic marker, and more specifically provides applications of a set of microRNAs in preparing biliary atresia diagnostic preparations. The microRNA is composed of hsa-miR-150, hsa-miR-4689, hsa-miR-92a-3p, and hsa-miR-4429. It is confirmed by experiments that, in biliary atresia serum, expression amount of the miRNA possesses significant difference on the statistical significance with expression amount of a corresponding miRNA of cholestatic infant hepatitis syndrome. The invention also provides a detection method of the set of miRNAs. Compared with the prior art, the detection method is capable of saving time, and is high in sensitivity and accuracy, low in cost, and / or high in efficiency. And in addition, the invention also provides kits and chips relative to the applications.

The invention belongs to the field of biomedicine and molecular biology, and relates to a method for establishing a disease-related factor scoring parameter analysis model, in particular to a method for establishing a biliary atresia column diagram prediction model and an application thereof. The method comprises the steps: employing an rms packet to establish a Nomogram diagnosis column diagram based on multiple logistic regression analysis, wherein the Nomogram diagnosis column diagram comprises the selection of independent variables and quantification of weight scores of all factors in theindependent variables, and the independent variables comprise sex, weight, DB, logarithm (ALP) and logarithm (GGT). The established Nomogram diagnosis column diagram based on multivariate logistic regression analysis is helpful for determining the risk degree of biliary atresia according to scores in clinical practice. Furthermore, the established Nomogram diagnosis column diagram based on multiple logistic regression analysis can be used for analyzing scoring parameters of serum markers of biliary atresia and cholestatic infant hepatitis syndrome, and is beneficial to identification of BA andother forms of neonatal cholestasis (NC) with different causes of diseases.

The invention discloses application of a newborn biliary atresia diagnostic marker in preparation of a tool for evaluating and diagnosing newborn biliary atresia. The diagnostic product can be a medical instrument, a kit, test paper, a medical device and the like. The invention also provides a kit for detecting the biomarker and a use method of the kit. The invention provides an effective means for early screening of the risk of neonatal biliary atresia and diagnosis different from cholestasis caused by other reasons, improves the diagnosis efficiency and diagnosis accuracy of the neonatal biliary atresia, strives for time for taking treatment measures in time, and is beneficial to improving the treatment effect and prognosis of the neonatal biliary atresia.

The invention provides a composition comprising a butyric acid compound and an application of the composition. The butyric acid compound is used for preventing and curing biliary atresia and the complications. The composition comprising the butyric acid compound comprises the butyric acid compound and a nutrition agent or a pharmaceutical aid, and the concentration of the butyric acid compound is5-70 mM. The intervention technique generally acting on the intestine is creatively used to prevent and cure the biliary atresia and the complications. As a safe effective preventing and curing method, the butyric acid compound is directly used or added in the nutrition agent and used for pregnant and newborn individuals having no symptoms, so that the occurrence and development of the biliary atresia is prevented and cured fundamentally. The composition solves the technical problems of prevention and cure of complications of the biliary atresia and hepatic fibrosis at the same time, and a safe economical effective method is provided for the prevention and cure of biliary atresia and hepatic fibrosis.

The invention belongs to the technical field of medicines, and relates to Biliatresone separated and purified from plants, as well as a preparation method, medicinal composition and application thereof. The Biliatresone further comprises cis-trans-isomer, enantiomer, diastereoisomer, raceme, solvate, hydrate or pharmaceutically acceptable salt and ester. Bioactivity experimental tests indicate that the compound can be used for remarkably inhibiting zebrafish larvae from gallbladder dysplasia, and prove that the compound Biliatresone has bioactivity, can be further used as biliary epithelia-philic medicines for studying biliary atresia inducing animal models.

The invention relates to the field of medical diagnostics, in particular to a biliary atresia diagnostic kit, which comprises a CD177 + neutrophil sorting reagent and a reagent for observing mitochondrial morphology and / or judging whether mitochondria has dysfunction or not. According to the kit provided by the invention, a specimen required for detection is obtained during normal examination, sothat a patient does not need to be additionally subjected to traumatic detection, and the kit is beneficial to popularization and application in the field of biliary atresia detection.

Keratin 8 and 18 (K8 / K18) mutations are shown to be associated with a predisposition to liver or biliary tract disease, particularly noncryptogenic hepatobiliary disease. Unique K8 / K18 mutations are shown in patients with diseases including but without limitation to viral hepatitis, biliary atresia, alcoholic cirrhosis and other acute or chronic toxic liver injury, cryptogenic cirrhosis, acute fulminant hepatitis, autoimmune liver disease, cystic fibrosis, primary biliary cirrhosis, primary sclerosing cholangitis, diseases that are linked with cryptogenic cirrhosis, such as nonalcoholic steatohepatitis, and the like. Livers with keratin mutations had increased incidence of cytoplasmic filamentous deposits. Therefore, K8 / K18 are susceptibility genes for developing cryptogenic and noncryptogenic forms of liver disease. Mutant alleles are associated with disease susceptibility, and their detection is used in the diagnosis of a predisposition to these conditions.

The invention relates to applications of serum taurochenodeoxycholic acid and chenodeocycholic acid as diagnostic markers in the preparation of medical instruments for diagnosing biliary atresia of newborns. The diagnostic markers of the medical instruments are content ratio of the taurochenodeoxycholic acid and the chenodeocycholic axid in serum. The invention also provides the application of chenodeocycholic acid. The application of diagnostic markers in biliary atresia of the newborns has the advantages that the application of the taurochenodeoxycholic acid and the chenodeocycholic acid as diagnostic markers of biliary atresia is proposed for the firstly time, so that the biliary atresia can be distinguished from other cholestasis diseases of newborns, the best operation time of children can be strived for, and therefore, the operation effect can be improved.

Disclosed are methods and compositions for treatment of a subject having a biliary disorder. The methods include administering a therapeutically effective amount of a serine protease inhibitor to a subject in need thereof. The biliary disorder include biliary atresia, a biliopathy, Primary Biliary Cirrhosis (PBC), Primary Sclerosing Cholangitis (PSC), and combinations thereof. In certain aspects, the serine protease inhibitor may be a protease inhibitor rC1 Inhibitor.

The invention belongs to the fields of biomedicine and molecular biology, and relates to a new diagnostic marker for biliary atresia. Specifically, the application of a group of microRNAs in the preparation of biliary atresia diagnostic preparations is provided, and the microRNAs are composed of hsa-miR-150, hsa-miR-4689, hsa-miR-92a-3p and hsa-miR-4429. Experiments have shown that in the serum of biliary atresia, there is a statistically significant difference in the expression levels of the above miRNAs and the corresponding miRNAs of cholestatic infantile hepatitis syndrome. The present invention also provides a detection method for this group of miRNAs, which saves time, has higher sensitivity and accuracy, lower cost, and / or is more efficient than prior art detection. In addition, the present invention also provides kits and chips related to these applications.

Disclosed are methods and compositions for treatment of a subject having a biliary disorder. The methods include administering a therapeutically effective amount of a serine protease inhibitor to a subject in need thereof. The biliary disorder include biliary atresia, a biliopathy, Primary Biliary Cirrhosis (PBC), Primary Sclerosing Cholangitis (PSC), and combinations thereof. In certain aspects, the serine protease inhibitor may be a protease inhibitor rC1 Inhibitor.

The invention provides application of N-acetylcysteine in preparation of a product for treating biliary atresia and a medicine for treating biliary atresia, and relates to the technical field of disease treatment. According to the application of N-acetylcysteine in preparation of the product for treating the biliary atresia, the research of the inventor finds that after N-acetylcysteine is administered to treat children with biliary atresia, the number of CD177<+> neutrophils and cell viability (ROS level, mitochondrial level and extracellular trap level) of the children with biliary atresia are obviously reduced, direct bilirubin and total bilirubin are obviously reduced, N-acetylcysteine has an improvement effect on treatment of biliary atresia, and the N-acetylcysteine is reliable in safety and can be used for preparing products for treating biliary atresia. The medicine for treating the biliary atresia comprises N-acetylcysteine, and has a treatment effect on the biliary atresia.

Compounds of general formula (I): wherein R2a, R2b, R3a, R3b, R5, Y and R7 are as defined herein are selective agonists at the FXR receptor and are useful for the treatment or prevention of diseases and conditions including nonalcoholic steatohepatitis (NASH); primary biliary cirrhosis; primary sclerosing cholangitis; biliary atresia; cholestatic liver disease; hepatitis C infection; alcoholic liver disease; fibrosis; or liver damage arising from fibrosis.

The invention discloses a porta hepatis retractor for biliary atresia porta hepatis intestinal anastomosis. The porta hepatis retractor comprises a retractor handle, a front retractor plate and a rearretractor plate, wherein the front retractor plate is arranged on the upper portion of one end of the retractor handle; the front retractor plate is perpendicular to the retractor handle; the rear retractor plate is arranged on the lower portion of the other end of the retractor handle; the rear retractor plate is perpendicular to the retractor handle; and the retractor handle, the front retractor plate and the rear retractor plate are of an integrated structure. The shape of the retractor is designed according to the form of the biliary atresia hypertrophic liver quadrate lope, hollow partsare arranged in the front retractor plate and the rear retractor plate, or the top end of the front retractor plate is provided with a protrusion bent towards one side of the retractor handle, and thebottom of the rear retractor plate is provided with a protrusion bent towards one side of the retractor handle, so that the stability of liver tissue traction is improved, and the optimal surgical exposure effect is achieved.

The invention provides an application of neutrophil elastase in the preparation of a product for diagnosing biliary atresia, and relates to the technical field of molecular diagnosis. The invention firstly confirms that by detecting a large number of BA patient samples and combining in vivo and in vitro experiments The expression of NE in liver tissue and peripheral blood of BA can be used as a powerful indicator to improve the diagnosis of BA and evaluate the degree of liver injury. Therefore, detecting the expression of NE can provide a more convenient, direct and accurate method for clinical diagnosis and evaluation of the condition of children with BA. method. In addition, the present invention also confirms that the NE inhibitor has a therapeutic effect in animal experiments and can be used in the development of drugs for the treatment of BA.

The invention provides an application of neutrophil elastase in preparation of product for diagnosing biliary atresia and relates to the technical field of molecular diagnosis; by detecting a large number of BA child patient samples and combining in-vivo and in-vitro experiments, firstly, it is proved that expression of NE in BA liver tissue and peripheral blood can be used as a powerful index forimproving BA diagnosis and evaluating the liver injury degree, and therefore detection of expression of NE can be used as a more convenient, direct and accurate method for clinically diagnosing and evaluating the illness state of BA children patients; in addition, the invention also proves that the NE inhibitor plays a treatment role in animal experiments, and can be used for research and development of medicines for treating BA.

The present invention relates to the field of neonatal cholestasis, in particular the treatment of neonatal biliary atresia or diseases associated with biliary system destruction. Also, the invention relates to methods and / or compounds for treating or preventing biliary atresia or disease associated with biliary system destruction. It further relates to methods and / or compounds for slowing the progression of biliary atresia or disease associated with biliary system destruction.

Provided are novel diagnostic and prognostic methods for biliary atresia using amyloid precursor protein.

The invention provides application of a biomarker. The biomarker is characterized in that the biomarker is selected from one or more of the following specific biomarkers: A. Glutamic acid; B, indoleacetic acid (Indoleaceticacid); C, [alpha]-ketoisovalerate (alpha-ketoisovalerate), and C, [alpha]-ketoisovalerate ([alpha]-ketoisovalerate); D, ketoleucine (Ketoleucine) is used as a raw material; E. 2-hydroxyglutaric acid (2-hydroxyglutaric acid), and E. 2-hydroxyglutaric acid (2-hydroxyglutaric acid); F, taurohyocholic acid (THCA); G, taurocholic acid (TCA); and H, glycocholic acid (GCA). The application is used for preparing a screening kit for detecting early risk of neonatal biliary atresia.

The invention relates to a construction method of an animal model for biliary atresia of chronic liver disease firstly utilizing a Gr-1 antibody to construct the construction method of the animal model for biliary atresia of chronic liver disease. The method comprises following steps: injecting rhesus monkey rotavirus within the 24 hours of neonatal mouse to induce a classic animal model for biliary atresia, firstly injecting the Gr-1 antibodies before rhesus monkey rotavirus is injected; injecting the Gr-1 antibodies multiple times after mouse are born on the 14th day to maintain the effective concentration range of the Gr-1 antibodies in the mouse; and injecting 5-20ng / 1.6g of Gr-1 antibodies to the mouse each time. The construction method of an animal model for biliary atresia of chronic liver disease has following beneficial effects: the model is capable of simulating the process of biliary atresia, development and prognosis, especially in the end-stage hepatocirrhosis phase.

The invention discloses a method based on a gene / protein as a diagnostic marker and a therapeutic target for biliary atresia and application thereof. Notch3 signaling pathway and remodeling of hepaticartery system mediated thereby are first proposed in the context of biliary atresia (BA), and constitute a perfection to the existing theoretical mechanism. The Notch3-Hey1 gene / protein, which is highly expressed in liver tissue, can be converted into a BA diagnostic marker which is helpful to prediction of prognosis in children and selection of surgical surgical methods as an auxiliary means. Incurrent clinical practice, anti-inflammatory and anti-fibrosis treatment cannot achieve ideal effect, and Kasai operation can not completely stop the progression of BA; however, a new adjuvant therapy strategy is provided by Notch3 signaling pathway inhibitors, thereby beneficially improving hypoxia and bile duct injury in portal area, slowing down disease progression and raising survival rates of patients with BA liver