The Experts below are selected from a list of 123 Experts worldwide ranked by ideXlab platform

Rüdiger Von Kries - One of the best experts on this subject based on the ideXlab platform.

  • Vitamin K Deficiency and Breast-Feeding
    Perinatal Thrombosis and Hemostasis, 1991
    Co-Authors: Rüdiger Von Kries
    Abstract:

    A dietary component which is essential for hemostasis was detected some fifty years ago and was called vitamin K. The clinical relevance of this vitamin in pediatrics was studied during this period [1]. Classical hemorrhagic disease of the newborn characteristically presents with gastrointestinal, nasal, skin and circumcision bleeding during the first 7 days of life [2,3]. For many years vitamin K deficiency bleeding beyond the neonatal period appeared to be related to malabsorption and cholestasis syndromes, such as celiac disease [2], Bile Duct Atresia, [4] and cystic fibrosis [5] only. Bleeding in these cases may be observed at any time during the course of the underlying disease unless sufficient vitamin K supplements are given [6]. An early infantile hemorrhagic syndrome due to vitamin K deficiency, was not recognized until 1970 [7,8]. This hemorrhagic syndrome is characterized by intracranial hemorrhage which accounts for more than 50% of cases and is observed mainly in the fourth to sixth week of life [2,3].

P. Schweizer - One of the best experts on this subject based on the ideXlab platform.

  • Pathogenesis of extrahepatic Bile Duct Atresia (EHBA): comprehension from a surgical point of view.
    European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie, 2005
    Co-Authors: P. Schweizer, M. Petersen, U. Drews, C. Bachmann, M. Schweizer
    Abstract:

    IntroDuction: Le but de cette etude est de mieux comprendre la pathogenie de l'atresie biliaire et d'expliquer a la fois la pathomorphologie variable et repetitive ainsi que les decouvertes histologiques. Materiel et Methodes: La pathomorphologie et les decouvertes histologiques de 223 patients avec une EHBA histologiquement evidente etaient revues retrospectivement (72 patients) ou prospectivement (151 patients). Ces decouvertes etaient comparees avec une etude histologique chez des embryons humains et de rats. Resultats: 1) Les decouvertes pathomorphologiques retrouvees chez les patients avec EHBA etaient aussi trouvees au cours de l'embryogenese normale du systeme biliaire chez les embryons humains et de rats. 2) Les resultats histologiques dans l'atresie biliaire correspondent a une interruption du developpement normal de l'embryon humain et de rat. 3) Les decouvertes chez les patients et embryons peuvent etre expliquees completement par un trouble de l'interaction intrinseque epithelium/mesoderme. 4) Quelques decouvertes dans l'atresie des voies biliaires ne peuvent etre expliquees facilement par l'action d'un facteur intrinseque. Conclusion: Il n'y a pas de decouverte concernant l'atresie des voies biliaires qui ne puisse etre completement expliquee par un defaut du developpement intrinseque, probablement du a des anomalies ou a une interruption de l'interaction epithelium/mesoderme durant l'embryogenese.

  • Prognosis of extrahepatic Bile-Duct Atresia after hepatoportoenterostomy
    Pediatric Surgery International, 2000
    Co-Authors: P. Schweizer, M. Schweizer, K. Schellinger, H. J. Kirschner, C. Schittenhelm
    Abstract:

     Clinical and histologic findings from 206 patients operated upon for extrahepatic biliary Atresia (EHBA) are analyzed in order to define the prognosis of patients with EHBA. The prospective study took into consideration both initial fibrosis of the liver and the morphology of the porta hepatis (PH) at surgery. Kaplan-Meier survival estimates and statistical calculations demonstrated a relationship between long-term survival and histologic findings in the liver and porta hepatis. The efficacy of HPE is significantly influenced by the morphology of the PH and to a lesser extent by the initial liver fibrosis. Surgery should thus achieve pattern 1 morphology of the PH, but this is problematic because of the close relationship of the vascular and biliary structures in its two lateral zones.

  • extrahepatic Bile Duct Atresia how efficient is the hepatoporto enterostomy
    European Journal of Pediatric Surgery, 1998
    Co-Authors: P. Schweizer, K Lunzmann
    Abstract:

    But: Etude prospective de 151 enfants atteints d'atresie biliaire extra-hepatique operes entre 1972 et 1997 et en particulier de 108 patients qui ont ete operes depuis 5 ans et plus, de facon a preciser l'efficacite de l'hepatico-porto-enterostomie (HPE). Methodes: L'evolution a ete evaluee de facon prospective d'apres un programme conditionne pour des enfants operes depuis 5 ans et plus. Les parametres objectifs ont ete l'histologie hepatique au moment de la chirurgie et la modification de ces parametres suivis pendant 5 ans et plus. Ils sont en accord avec la secretion biliaire et l'âge du patient. Resultats: 108 patients ont ete operes depuis 5 ans et plus: 62 sont parfaitement bien portants (57 %), 58 enfants sont anicteriques. Les resultats suivants peuvent etre etablis: 1. Il y a une relation etroite entre l'âge de patient et l'importance de la fibrose hepatique au moment de la chirurgie. 2. Il y a une relation etroite entre l'âge et le niveau de fibrose hepatique au moment de la chirurgie d'une part et le pourcentage de survie a long terme, l'absence d'ictere et la qualite de la fonction hepatique d'autre part. 3. Il y a une relation etroite entre l'âge et le niveau de fibrose hepatique au moment de la chirurgie d'un cote et le flux biliaire permis chirurgicalement de l'autre. 4. La morphologie et les parametres biophysiques du hile du foie dans la region portale de meme que les cholangites post-operatoires ont une influence negative sur le pronostic alors s qu'une fibrose peu intense au moment de la chirurgie, un flux biliaire post-operatoire important ont une influence positive u sur l'evolution. Conclusion: L'efficacite de l'HPE depend de l'âge et (en correlation avec) du niveau de la fibrose hepatique au moment de l'intervention chirurgicale; elle depend egalement de la morphologie et des proprietes biophysiques de la region portale hepatique et des episodes de cholangite post-operatoire. Le niveau de fibrose de la triade de Glisson au moment de l'intervention chirurgicale, les parametres morphologiques et biophysiques du hile du foie sont consideres comme des causes de defaillance du flux biliaire apres HPE et de la progression de la fibrose. En l'absence de cause precise, on etablit que l'obliteration primitive des voies biliaires traduit une evolution de la maladie primaire. L'efficacite de HPE est resumee dans les resultats: sur 108 enfants qui ont ete operes depuis plus des 5 ans, 62 sont parfaitement bien portants, 58 sont anicteriques, 25 ne presentent pas de progression de la fibrose et 46 sur 82 qui initialement etaient sains, ne presentent pas non plus de fibrose actuellement.

  • Extrahepatic Bile Duct Atresia. Comparison of surgical and non-surgical therapy
    Monatsschrift Kinderheilkunde : Organ der Deutschen Gesellschaft fur Kinderheilkunde, 1992
    Co-Authors: M. Schweizer, P. Schweizer, R. Knupfer, Klaus Dietz
    Abstract:

    In recent years hepatoportoenterostomy according to Kasai undoubtedly was the only successful therapy for extrahepatic biliary Atresia. Since liver-transplantation becomes more successful than previously, the question arises if the Kasai procedure is still justified. From this point of view a prospective study was started based on the following findings in 15 EHBA-children after hepatoportoenterostomy compared to those in 15 patients without the Kasai procedure: the survival rate at the end of two years of life; the development of ascites until the end of the first year of life; the weight and length percentiles at the end of the first year of life; and the activity of serum cholinesterase. In order to calculate significant differences in weight and length percentiles, the exact Fisher test was used. Serum cholinesterase activities of both the above mentioned groups were compared with each other, as well as with the normal values of a third group of 50 healthy children, by analyzing variances and then comparing one by one with adjustment for multiple comparisons. There was a significant difference between the operated and not-operated group, and in addition between the non-operated and the healthy group. In contrast, no significant difference was detected between the operated and the healthy group. These differences were best demonstrated by the findings of weight and length percentiles and the activity of serum cholinesterase. From this we conclude sofar hepatoportoenterostomy undoubtedly is standard procedure in the treatment of EHGA. However, if the Kasai procedure fails or the liver disease progresses to a greater extent, liver transplantation will become the method of choice.

M. Schweizer - One of the best experts on this subject based on the ideXlab platform.

  • Pathogenesis of extrahepatic Bile Duct Atresia (EHBA): comprehension from a surgical point of view.
    European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie, 2005
    Co-Authors: P. Schweizer, M. Petersen, U. Drews, C. Bachmann, M. Schweizer
    Abstract:

    IntroDuction: Le but de cette etude est de mieux comprendre la pathogenie de l'atresie biliaire et d'expliquer a la fois la pathomorphologie variable et repetitive ainsi que les decouvertes histologiques. Materiel et Methodes: La pathomorphologie et les decouvertes histologiques de 223 patients avec une EHBA histologiquement evidente etaient revues retrospectivement (72 patients) ou prospectivement (151 patients). Ces decouvertes etaient comparees avec une etude histologique chez des embryons humains et de rats. Resultats: 1) Les decouvertes pathomorphologiques retrouvees chez les patients avec EHBA etaient aussi trouvees au cours de l'embryogenese normale du systeme biliaire chez les embryons humains et de rats. 2) Les resultats histologiques dans l'atresie biliaire correspondent a une interruption du developpement normal de l'embryon humain et de rat. 3) Les decouvertes chez les patients et embryons peuvent etre expliquees completement par un trouble de l'interaction intrinseque epithelium/mesoderme. 4) Quelques decouvertes dans l'atresie des voies biliaires ne peuvent etre expliquees facilement par l'action d'un facteur intrinseque. Conclusion: Il n'y a pas de decouverte concernant l'atresie des voies biliaires qui ne puisse etre completement expliquee par un defaut du developpement intrinseque, probablement du a des anomalies ou a une interruption de l'interaction epithelium/mesoderme durant l'embryogenese.

  • Prognosis of extrahepatic Bile-Duct Atresia after hepatoportoenterostomy
    Pediatric Surgery International, 2000
    Co-Authors: P. Schweizer, M. Schweizer, K. Schellinger, H. J. Kirschner, C. Schittenhelm
    Abstract:

     Clinical and histologic findings from 206 patients operated upon for extrahepatic biliary Atresia (EHBA) are analyzed in order to define the prognosis of patients with EHBA. The prospective study took into consideration both initial fibrosis of the liver and the morphology of the porta hepatis (PH) at surgery. Kaplan-Meier survival estimates and statistical calculations demonstrated a relationship between long-term survival and histologic findings in the liver and porta hepatis. The efficacy of HPE is significantly influenced by the morphology of the PH and to a lesser extent by the initial liver fibrosis. Surgery should thus achieve pattern 1 morphology of the PH, but this is problematic because of the close relationship of the vascular and biliary structures in its two lateral zones.

  • Extrahepatic Bile Duct Atresia. Comparison of surgical and non-surgical therapy
    Monatsschrift Kinderheilkunde : Organ der Deutschen Gesellschaft fur Kinderheilkunde, 1992
    Co-Authors: M. Schweizer, P. Schweizer, R. Knupfer, Klaus Dietz
    Abstract:

    In recent years hepatoportoenterostomy according to Kasai undoubtedly was the only successful therapy for extrahepatic biliary Atresia. Since liver-transplantation becomes more successful than previously, the question arises if the Kasai procedure is still justified. From this point of view a prospective study was started based on the following findings in 15 EHBA-children after hepatoportoenterostomy compared to those in 15 patients without the Kasai procedure: the survival rate at the end of two years of life; the development of ascites until the end of the first year of life; the weight and length percentiles at the end of the first year of life; and the activity of serum cholinesterase. In order to calculate significant differences in weight and length percentiles, the exact Fisher test was used. Serum cholinesterase activities of both the above mentioned groups were compared with each other, as well as with the normal values of a third group of 50 healthy children, by analyzing variances and then comparing one by one with adjustment for multiple comparisons. There was a significant difference between the operated and not-operated group, and in addition between the non-operated and the healthy group. In contrast, no significant difference was detected between the operated and the healthy group. These differences were best demonstrated by the findings of weight and length percentiles and the activity of serum cholinesterase. From this we conclude sofar hepatoportoenterostomy undoubtedly is standard procedure in the treatment of EHGA. However, if the Kasai procedure fails or the liver disease progresses to a greater extent, liver transplantation will become the method of choice.

V. Bhatnagar - One of the best experts on this subject based on the ideXlab platform.

Peter Van Eyken - One of the best experts on this subject based on the ideXlab platform.

  • Extrahepatic Bile Duct Atresia from the pathologist’s perspective: pathological features and differential diagnosis
    2014
    Co-Authors: Peter Van Eyken, Daniela Fanni, Gavino Faa
    Abstract:

    Extrahepatic biliary Atresia (EHBA) refers to stenosis or Atresia of the extrahepatic biliary tree. It accounts for 25-30% of cases of neonatal cholestasis. If left untreated, EHBA progresses to biliary cirrhosis and is universally fatal within the first 2 years of life. Early diagnosis is crucial since surgical treatment (Kasai procedure) is the only treatment option. Histopathologic examination of liver biopsy specimens is a key element in the diagnostic work-up of infants with suspected EHBA. Pathologic diagnosis aims at excluding non-surgically correctable causes of neonatal cholestasis thereby leading to surgical exploration for confirmation of the diagnosis. All published data indicate that pathologists can diagnose EHBA with high sensitivity, high specificity and reasonable interobserver agreement. The most useful histologic features in the diagnosis of EHBA are portal tract changes including Ductular proliferation and Bile plugs in Ducts and Ductules. These lesions are not pathognomonic but can be seen in extrahepatic obstruction of any cause. Total parenteral nutrition (TPN)-associated cholestasis and alpha1-antitrypsin (A1AT) deficiency cannot be differentiated from EHBA without access to clinical data and may lead to false-positive diagnosis. False-negative interpretation may be caused by early age at diagnosis or by small/indequate specimens. The pathologist also plays a role in the examination of the resected fibrotic segment and of explant specimens. Histopathology can yield prognostic information, being also an indispensable tool in research for the possible pathogenesis of this disease. A well-coordinated, multidisciplinary approach is required in the assessment of suspected cases of EHBA. Proceedings of the International Course on Perinatal Pathology (part of the 10 th International Workshop on Neonatology · October 22 nd -25 th , 2014) · Cagliari (Italy) · October 25 th , 2014 ·  The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken

  • extrahepatic Bile Duct Atresia from the pathologist s perspective pathological features and differential diagnosis
    Journal of Pediatric and Neonatal Individualized Medicine (JPNIM), 2014
    Co-Authors: Peter Van Eyken, Daniela Fanni, Gavino Faa
    Abstract:

    Extrahepatic biliary Atresia (EHBA) refers to stenosis or Atresia of the extrahepatic biliary tree. It accounts for 25-30% of cases of neonatal cholestasis. If left untreated, EHBA progresses to biliary cirrhosis and is universally fatal within the first 2 years of life. Early diagnosis is crucial since surgical treatment (Kasai procedure) is the only treatment option. Histopathologic examination of liver biopsy specimens is a key element in the diagnostic work-up of infants with suspected EHBA. Pathologic diagnosis aims at excluding non-surgically correctable causes of neonatal cholestasis thereby leading to surgical exploration for confirmation of the diagnosis. All published data indicate that pathologists can diagnose EHBA with high sensitivity, high specificity and reasonable interobserver agreement. The most useful histologic features in the diagnosis of EHBA are portal tract changes including Ductular proliferation and Bile plugs in Ducts and Ductules. These lesions are not pathognomonic but can be seen in extrahepatic obstruction of any cause. Total parenteral nutrition (TPN)-associated cholestasis and alpha1-antitrypsin (A1AT) deficiency cannot be differentiated from EHBA without access to clinical data and may lead to false-positive diagnosis. False-negative interpretation may be caused by early age at diagnosis or by small/indequate specimens. The pathologist also plays a role in the examination of the resected fibrotic segment and of explant specimens. Histopathology can yield prognostic information, being also an indispensable tool in research for the possible pathogenesis of this disease. A well-coordinated, multidisciplinary approach is required in the assessment of suspected cases of EHBA. Proceedings of the International Course on Perinatal Pathology (part of the 10 th International Workshop on Neonatology · October 22 nd -25 th , 2014) · Cagliari (Italy) · October 25 th , 2014 ·  The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken

  • 10 questions: a Belgian pathologist, Peter Van Eyken, on the future of pathology. Interview by Sonia Nemolato
    2013
    Co-Authors: Peter Van Eyken, Sonia Nemolato
    Abstract:

    Prof. Peter Van Eyken, a renowned Belgian pathologist, answers to the following 10 questions: 1. You are a pupil of Valeer Desmet, one of the leading liver pathologists in the world. What was it like spending so many years with him? 2. Your first research project focused on cytokeratins: how has your research changed liver biopsy interpretation in clinical practice? 3. What are the most important innovations in pathology of the last years? What is changing in your approach to histology and to cytology? 4. What is the role of pathologists in pediatric pathology? How is their relationship with pediatricians is changing? 5. How is the role of the pathologist changing in neonatal and in perinatal medicine? 6. As a young researcher, you published many articles on liver development and on Bile Duct Atresia: did your studies change the way you approach liver biopsy interpretation in a newborn? 7. After years of involvement in kidney biopsy interpretation in adults, recently, you became involved in studies on nephrogenesis. Which is the relationship between renal development and adult kidney pathology? 8. What is your opinion on networks in medicine? Should pathology be integrated with “omic” sciences and informatics ? 9. What about the future of pathology? New techniques or new eyes in the interpretation of H&E-stained sections? What is the role of immunohistochemistry? And of of molecular pathology? 10. Could you advise young medical doctors to become pathologists? What are your suggestions?