https://www.journal-of-hepatology.eu/article/0168-8278(95)80194-4/pdf

Liver transplantation for Wilson's disease

The most common hepatic complications of cystic fibrosis (CF) are steatosis, fibrosis, biliary cirrhosis, atretic gallbladder, cholelithiasis, and sclerosing cholangitis. Cholestatic liver disease is a slow progressive disorder, but will stabilize for many patients. CF patients may suffer from the consequences of their liver disease and without liver transplantation, variceal hemorrhage, malnutrition, or end-stage liver disease can lead to death. Prospective data were collected and reviewed on 311 liver transplants performed in 283 patients at the Children's Medical Center of Dallas between October 1984 and November 2000. Ten children received an orthotopic liver transplant (OTLX) for end-stage liver disease associated with cystic fibrosis. Pulmonary function tests were obtained preoperatively in all cases. There were nine boys and one girl. Six are currently alive, and four are dead. Both patient and graft survival was 5.75 yr. Among those currently alive, mean patient and graft survival is 7.71 yr (range 0.10-12.62 yr). Mean patient and graft survival of those who died was 2.35 yr (range 0.78-5.33 yr). No survivor required re-transplantation and currently, all have normal serum aminotransferase values. Chronic sinusitis was not a significant pre- or post-transplant morbidity, although systematic radiographic evaluation of the sinuses did not occur. Pulmonary deaths occurred in three patients from pulmonary hemorrhage, pulmonary infection with Aspergillus and Candida glabrata, and acute bronchopneumonia associated with polymicrobial sepsis because of Pseudomonas, Klebsiella, and Candida albicans 1.44, 0.78, and 1.83 yr, respectively, after transplantation. The fourth death was associated with chronic rejection, and occurred 5.33 yr after transplantation. All non-survivors were below the 5th percentile for height and weight at the time of liver transplantation. Mean age at transplantation was 9.72 yr (range 1.23-19.09, median 9.61). Survivors were transplanted at a younger age than non-survivors (mean of 9.21 yr vs. 10.66 yr), and had shorter waiting times from diagnosis of end-stage liver disease to transplantation (6.87 months vs. 13.83 months). Eighty percentage (n = 8) of patients had pretransplant variceal bleeds (83% of survivors, 75% of non-survivors). While all non-survivors had a history of meconium ileus and preoperative need of pancreatic enzymes, only 67% of those alive experienced these complications. Preoperative forced vital capacity FVC was 103% for survivors and 95% for non-survivors. The corresponding numbers for forced expiratory flow (FEF) 25-75 were 74-84% respectively. Preoperative Aspergillus was identified in 30% of patients (n = 3). Two of these patients are alive. Cystic fibrosis constitutes an indication for 3.5% of pediatric liver transplants. Evaluation and transplantation for end-stage liver disease associated with cystic fibrosis should be undertaken at an early age. Most deaths were associated with pulmonary/septic events, and occurred less than 2 yr after OLTX. Those children who did not survive had poor growth and nutrition, prolonged waiting times prior to transplantation, were transplanted at an older age, and had a higher incidence of pancreatic insufficiency and meconium ileus. The presence of Aspergillus in the sputum does not constitute a contraindication for OLTX.

Liver transplantation for cholestasis associated with cystic fibrosis in the pediatric population.

The efficacy of orthotopic liver transplantation (OLT) in the management of more common liver-based metabolic disorders associated with severe liver damage, alpha-1-antitrypsin deficiency (PIZZ), Wilson disease and tyrosinaemia has been demonstrated and indications defined. An early mortality in excess of 15% and finite resources limit its use. Phenotypic heterogeneity make the precise indication in other disorders less certain. In disorders in which endstage liver disease is less frequent such as cystic fibrosis, haemochromatosis and galacosaemia it has been a very effective therapy. It has been used with encouraging results in disorders in which the liver is structurally normal such as Crigler-Najjar type I, primary hyperoxaluria type I and primary hypercholesterolaemia. In these it should be performed before there is permanent damage to brain, kidneys or heart. OLT in the short term prevents hyperammonaemic coma in urea cycle defects and may prevent extrahepatic disease in glycogen storage disease type IV. Its limitation in reversing all metabolic effects in these and other disorders is discussed. It is ineffective in protoporphyria or Niemann Pick disease type II (Sea Blue Histiocyte syndrome) in which the transplanted liver acquires the lesions of the initial disorder and extrahepatic features progress. Early referral provides optimum circumstances to assess the benefits of OLT as compared with those of other forms of management and to achieve transplantation at the ideal time. The place of OLT in management will require constant review as metabolic disorders are better defined, new forms of therapy evolve and as techniques of liver transplantation and modes of immunosuppression improve.

Orthotopic liver transplantation in liver-based metabolic disorders.

Objective Ta evaluate the effectiveness and morbidity of endoscopic variceal ligation in secondary prevention of recurrence bleeding by ruptured esophageal varices at the cirrhotic in CHU Tlemcen. Materials and methods: Between January 2004, starting date of this study retro and forward-looking and June 2007 date point, patients having received at least a ligature were upheld. Resuits Fifty-six cirrhosis patients, including 40 post-viral hepatitis B or C having least one ligature: 29 men and 27 women, average age 52.8 years, (10 Child-Pugh A, 36 B and 10 C), with Varicose stage Il (n = 24) and stage III (n = 32). The average decline was 21.07 months. The eradication of esophageal varices was obtained in 78.6% of our patients after an average of 2.7 meetings. At an average period of 18.3 months, reanastomosis was detected in 38.1% 0f patients. A recurrence 0f bleeding was found in 23% of patients. The size of varices (Grade III) and the score Child Pugh C are factors that are significantly predictive of reanastomosis in our study, however neither sex nor age seem to play a role. At least one complication was observed in 17% of our patients. The mortality rate was 14.3% unrelated to the technique. Conclusion: The endoscopic variceal ligation is an effective technique, with 10w morbidity and easy achievement. It appears as a method of choice in the secondary prophylaxis of recurrence esophageal varice bleeding in the cirrhotic. The interest of the association of a beta blocker observed in our series is ta a high light.

/pdf/ministere-de-l-enseignement-superieur-et-de-la-recherche-19wcv8eoaz.pdf

Ministere de l'enseignement superieur et de la recherche scientifique

Liver transplantation for metabolic disorders

We report the case of a 12 year-old boy with Wilson's disease, presenting with decompensated liver cirrhosis. Medical treatment failed to prevent fulminant liver failure in less than one month. Emergency liver transplantation was successfully performed. This report led us to review the prognosis of Wilson's disease with liver failure according to the present results of liver transplantation.

[Indications for liver transplantation in Wilson's disease].

Indications de la transplantation hépatique au cours d'une maladie de Wilson

Resume La transplantation hepatique est une solution therapeutique proposee chez les patients atteints de mucoviscidose presentant une insuffisance hepatique terminale. Lorsque ces patients sont porteurs chroniques de Pseudomonas aeruginosa , se pose la question du choix entre une transplantation hepatique isolee (TRH) et combinee foie-poumon. Observation. Une adolescente de 15 ans atteinte de mucoviscidose a beneficie d'une TRH en raison d'une cirrhose au stade terminal. Elle presentait depuis 3 ans un portage chronique de pyocyanique traite par cures antibiotiques intravcineuses systematiques. Il existait une insuffisance pancreatique exocrine. Il etait note une intolerance glucidique moderee lors des surinfections. Les suites operatoires immediates ont ete simples. Elle a presente par la suite un diabete necessitant une insulinotherapie pendant 9 mois. Il a ete constate des troubles majeurs de l'absorption de la ciclosporine orale ameliores secondairement par la prise d'une nouvelle forme hydrosoluble (Neoral ® ). Il n'y a pas eu de surinfection pulmonaire severe due au portage chronique du pyocyanique. Il a ete note un rattrapage du developpement pubertaire. Conclusion. Ce recul de 3 ans confirme que la TRH est une bonne alternative therapeutique pour les patients atteints de mucoviscidose presentant une insuffisance hepatique terminale et une atteinte pulmonaire moderee.

Transplantation hépatique chez une adolescente atteinte de mucoviscidose

Handle everyday research tasks with reliable, citation-backed results

Your personal Research Agent to handle research tasks with citation-backed results

Popular Tasks used by Researchers

How can I help with your research?

Meet SciSpace

Get more enhanced response by uploading the PDFs you want me to reference.

No relevant PDFs in your library

SciSpace is the AI research assistant for academics. Run systematic literature reviews on 280M+ papers, and write papers with cited sources. Trusted by 1M+ students, PhDs & researchers.

SciSpace | AI for Research

Analyze PDFs

Code & Manuscripts

Funding & Grants

Literature & Patents

Medical & Clinical Data

Systematic Review

Visualize & Present

Web & Data

Build a Google Scholar-like website for your research.

Build a website

Create charts and images for your research

Create a Chart

Write a paper for submission to a journal

Draft a manuscript

Patent Search

Design eye-catching scientific posters in minutes.

Scientific Poster Generation

Systematic Literature Review

One task is running at the moment. Your messages will be shown right after.

Drag and drop or click here to browse

Loved by <highlight>1 million+</highlight> researchers

Extract a list of specific topics and their sources from unstructured text

Topics

Compare and analyze relevant papers that matches with your search

Papers

Get insights from PDFs and bookmarked papers from your library

My library

Recent searches

Try searching for:

Catch AI-generated content in scholarly and non-scholarly content

{ai} Detector

Ai Writer

Get PDF Summaries, highlighted text explanations 

Chat with PDF

Effortlessly create in-text citations and bibliographies in APA and 2,500 other formats

Citation generator

Get explanations, summaries, and answers on academic papers

Ease up your research workflow with {scispace}'s cohort of exciting AI tools

Elevate your academic writing skills and convey your ideas the way you want

Paraphraser

Explore our range of reading and writing tools

Your file is being prepared and should be ready in a few minutes. If it's a large file, it might take a bit longer. You can close this window, and we'll email you the file when it's done.

You have reached a maximum limit of <strong>{limit}</strong> columns in the table. Remove at least <strong>1</strong> column to add or create another one.

M. Razemon

Author Tools

Chat about Author

Papers

[Indications for liver transplantation in Wilson's disease].

Indications de la transplantation hépatique au cours d'une maladie de Wilson

Transplantation hépatique chez une adolescente atteinte de mucoviscidose