PMID- 36331262
OWN - NLM
STAT- MEDLINE
DCOM- 20230209
LR  - 20230209
IS  - 1440-1746 (Electronic)
IS  - 0815-9319 (Linking)
VI  - 38
IP  - 2
DP  - 2023 Feb
TI  - Experience on switching trientine formulations in Wilson disease: Efficacy and 
      safety after initiation of TETA 4HCl as substitute for TETA 2HCl.
PG  - 219-224
LID - 10.1111/jgh.16050 [doi]
AB  - BACKGROUND AND AIM: This retrospective, multicenter study aims to assess the 
      efficacy and safety in Wilson disease (WD) patients treated with trientine 
      tetrahydrochloride (TETA 4HCl) after switch from trientine dihydrochloride (TETA 
      2HCl). METHODS: In total, 68 WD patients with stable copper metabolism were 
      identified to receive TETA 4HCl (Cuprior) after previous treatment with TETA 
      2HCl. We analyzed biochemical markers such as urinary copper, serum copper, 
      non-coeruloplasmin bound copper (NCC), and transaminases as well as clinical 
      scores (APRI; FIB-4 score) at baseline with a follow-up (FU) of 12 months. Safety 
      of TETA 4HCl treatment was based on reported adverse events (AEs). RESULTS: The 
      study cohort reflects a common WD cohort with a mean age of 20.3 years at 
      diagnosis and 38.3 years at baseline. There are no significant differences 
      concerning serum copper, NCC, transaminases, APRI, and FIB-4 score in the 3-month 
      FU. Six-month FU revealed a decreased AST (P = 0.008), APRI (P = 0.042), and 
      FIB-4 score (P = 0.039). GGT varied only borderline significantly in the 3-month, 
      but not in the 6-month FU. Comparison of urinary copper within the subsets did 
      not reveal a difference to baseline in all FUs, suggesting stable control of 
      copper metabolism. Few AEs during TETA 4HCl treatment were reported, most 
      commonly gastrointestinal discomfort. Only three treatments with TETA 4HCl were 
      discontinued. CONCLUSION: Copper parameters and liver function were stable after 
      treatment switch to TETA 4HCl. Treatment with TETA 4HCl was generally well 
      tolerated. This study indicates that the switch from TETA 2HCl to TETA 4HCl is 
      safe and viable.
CI  - (c) 2022 Journal of Gastroenterology and Hepatology Foundation and John Wiley & 
      Sons Australia, Ltd.
FAU - Mohr, Isabelle
AU  - Mohr I
AUID- ORCID: 0000-0001-7354-0241
AD  - Department of Internal Medicine IV, Department of Gastroenterology, University 
      Hospital Heidelberg, Heidelberg, Germany.
FAU - Bourhis, Helene
AU  - Bourhis H
AD  - Medizinische Klinik und Poliklinik II, Hospital of the Ludwig-Maximilians 
      University Munich, Munich, Germany.
FAU - Woimant, France
AU  - Woimant F
AD  - Department of Neurology, Hopital Lariboisiere, Paris, France.
FAU - Obadia, Mickael Alexandre
AU  - Obadia MA
AD  - Department of Neurology, Rothschild Foundation Hospital, National reference 
      center for Wilson disease, Paris, France.
FAU - Morgil, Muzeyyen
AU  - Morgil M
AD  - Department of Internal Medicine, Salem Hospital Heidelberg, Heidelberg, Germany.
FAU - Morvan, Erwan
AU  - Morvan E
AD  - Department of Neurology, Rothschild Foundation Hospital, National reference 
      center for Wilson disease, Paris, France.
FAU - Merle, Uta
AU  - Merle U
AD  - Department of Internal Medicine IV, Department of Gastroenterology, University 
      Hospital Heidelberg, Heidelberg, Germany.
FAU - Denk, Gerald
AU  - Denk G
AD  - Medizinische Klinik und Poliklinik II, Hospital of the Ludwig-Maximilians 
      University Munich, Munich, Germany.
FAU - Poujois, Aurelia
AU  - Poujois A
AD  - Department of Neurology, Rothschild Foundation Hospital, National reference 
      center for Wilson disease, Paris, France.
FAU - Weiss, Karl Heinz
AU  - Weiss KH
AD  - Department of Internal Medicine, Salem Hospital Heidelberg, Heidelberg, Germany.
LA  - eng
GR  - Orphalan S.A./
PT  - Journal Article
PT  - Multicenter Study
DEP - 20221112
PL  - Australia
TA  - J Gastroenterol Hepatol
JT  - Journal of gastroenterology and hepatology
JID - 8607909
RN  - SJ76Y07H5F (Trientine)
RN  - 789U1901C5 (Copper)
RN  - 0 (Chelating Agents)
RN  - EC 2.6.1.- (Transaminases)
SB  - IM
MH  - Humans
MH  - Young Adult
MH  - Adult
MH  - *Trientine/adverse effects
MH  - *Hepatolenticular Degeneration/drug therapy
MH  - Copper
MH  - Retrospective Studies
MH  - Chelating Agents/adverse effects
MH  - Transaminases
OTO - NOTNLM
OT  - Wilson disease
OT  - trientine dihydrochloride (TETA 2HCl)
OT  - trientine tetrahydrochloride (TETA 4 HCl)
EDAT- 2022/11/05 06:00
MHDA- 2023/02/10 06:00
CRDT- 2022/11/04 09:33
PHST- 2022/09/27 00:00 [revised]
PHST- 2022/03/06 00:00 [received]
PHST- 2022/11/01 00:00 [accepted]
PHST- 2022/11/05 06:00 [pubmed]
PHST- 2023/02/10 06:00 [medline]
PHST- 2022/11/04 09:33 [entrez]
AID - 10.1111/jgh.16050 [doi]
PST - ppublish
SO  - J Gastroenterol Hepatol. 2023 Feb;38(2):219-224. doi: 10.1111/jgh.16050. Epub 
      2022 Nov 12.