Multicentre, retrospective study to assess long-term outcomes of chelator based treatment with trientine in Wilson disease patients withdrawn from therapy with d -penicillamine.

Author: DhawanAnil, FerenciPeter, KruseCarlot, ManolakiNina, WeissKarl Heinz, WijnbergLarissa, ZuinMassimo, de KoningConstance E, van ScheppingenDaphne

Paper Details 
Original Abstract of the Article :
OBJECTIVES: Trientine dihydrochloride (TETA-2HCl) has been used for the treatment of Wilson disease for over 30 years. The current study was designed to systematically evaluate existing data to further define the long-term outcome of the efficacy and tolerability of TETA-2HCl in Wilson disease patie...See full text at original site
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引用元:
https://pubmed.ncbi.nlm.nih.gov/35482910

データ提供:米国国立医学図書館(NLM)

Trientine Dihydrochloride: A Long-Term Assessment for Wilson Disease

This study delves into the long-term efficacy and safety of trientine dihydrochloride (TETA-2HCl) in the management of Wilson disease, a genetic disorder characterized by copper accumulation in the body. The study retrospectively analyzed the medical records of 77 Wilson disease patients who had switched from D-penicillamine to TETA-2HCl treatment, examining their hepatic and neurological symptoms, copper homeostasis, and adverse events over a period of 48 months. The findings revealed that TETA-2HCl was well-tolerated and effective in maintaining copper homeostasis, preventing progression to acute hepatic failure and liver transplantation. While some patients experienced gastrointestinal and nervous system disorders, these were manageable and did not lead to treatment discontinuation in most cases.

Long-Term Management of Wilson Disease: A Steady Path Through the Sand

This study provides valuable insights into the long-term use of TETA-2HCl for managing Wilson disease. The sustained efficacy and favorable tolerability observed in this cohort are encouraging, suggesting that TETA-2HCl can provide a stable and effective therapeutic option for patients with Wilson disease. It's like a steady path through a vast and unforgiving desert, offering a reliable route to navigate the challenges of this complex disorder.

Navigating the Challenges of Wilson Disease: A Collaborative Effort

This study highlights the importance of collaborative efforts between clinicians and patients in managing chronic conditions like Wilson disease. The sustained monitoring of patients receiving TETA-2HCl is crucial for ensuring optimal treatment outcomes and minimizing potential adverse events. It's like a caravan traversing a desert, where each member plays a vital role in ensuring the safety and well-being of the entire group.

Dr.Camel's Conclusion

This research underscores the value of long-term studies in understanding the true impact of therapeutic interventions. The findings offer hope for patients with Wilson disease, demonstrating the sustained efficacy and safety of TETA-2HCl. It's a reminder that with careful monitoring and a collaborative approach, we can navigate the challenges of chronic conditions and find solutions that lead to a better future.

Date :
  1. Date Completed 2022-08-03
  2. Date Revised 2022-08-28
Further Info :

Pubmed ID

35482910

DOI: Digital Object Identifier

00042737-202209000-00007

Related Literature

SNS
PICO Info
in preparation
Languages

English

Positive IndicatorAn AI analysis index that serves as a benchmark for how positive the results of the study are. Note that it is a benchmark and requires careful interpretation and consideration of different perspectives.

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