LIVER TRANSPLANTATION FOR HEREDITARY HAEMORRHAGIC TELANGIECTASIA (RENDU-OSLER DISEASE) WITH HEPATIC INVOLVEMENT: INDICATION AND LONG TERM RESULTS IN 10 PATIENTS.
Bibliographic record
Abstract
P568 Aims: Hepatic involvement in hereditary haemorrhagic telangiectasia is common but often asymptomatic. However, in some cases, the vascular lesions that involve the liver may lead to high- output cardiac failure and pulmonary hypertension and hepatobiliary manifestations requiring liver transplantation (LT). We report the long term results of LT in this indication. Patients & Methods: Between 1993 and 2003, ten patients with hereditary haemorrhagic telangiectasia and hepatic involvement received transplants. Indication for LT was right-sided heart failure (n=10) with pulmonary hypertension (n=8), biliary abcesses (n=1), and/or hemobilia (n=1). Left-to-right intrahepatic shunt output was estimated to range between 34% and 57.5% of cardiac output. Results: The patients were 9 women et 1 men, with a median age of 53 years (range 36-64). Operating time was 225 to 510 minutes (median : 260 minutes). Transfusion requirement was 0 to 16 blood units (median : 4 blood units). Hyperdynamic circulation disappeared after liver transplantation in all patients. Results of computed tomography and right-sided heart catheterization performed 6 months post-LT were normal and each patient continues to be asymptomatic. Median follow-up period currently is 48 months (range 12-122). Several complications occured during follow-up : digestive bleeding requiring transfusion and endoscopic treatment (n=2), epistaxis requiring transfusion (n=2), hepatic artery aneurysm from the native common hepatic artery requiring surgical treatment (n=1) and cerebral hemorrhage from aneurysm rupture (n=1). Overall survival was 90% (one patient died at one month post-LT from cardiac failure). Conclusions: Our report strongly suggests that LT can be considered as the best curative treatment that may prevent the irreversible evolution of cardiopulmonary disease in patients with hereditary haemorrhagic telangiectasia, but long term prognosis is related to other organ involvement of the disease.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.000 |
| Open science | 0.000 | 0.001 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".