Mayr, Toni published the artcileOptimized Trientine-dihydrochloride Therapy in Pediatric Patients With Wilson Disease: Is Weight-based Dosing Justified?, Recommanded Product: N1,N1′-(Ethane-1,2-diyl)bis(ethane-1,2-diamine) dihydrochloride, the publication is Journal of Pediatric Gastroenterology and Nutrition (2021), 72(1), 115-122, database is CAplus and MEDLINE.
The aim of the study was to investigate the efficacy and safety of trientine-dihydrochloride (TD) in pediatric patients with Wilson disease (WD) and the effect of different weight-based dosages on their clin. and biochem. outcome. We retrospectively reviewed the clin. data of 31 children with WD receiving TD therapy ages under 18 years at the time of diagnosis. Outcome measures included parameters of copper metabolism and liver function tests. To examine the impact of different weight-based dosages, 2 dosage subgroups were analyzed. Group 1 received less than 20mg/kg TD per day, group 2 more than 20mg¡¤kg-1¡¤day-1. Median follow-up was 60 (5-60) months in the total study group. During TD therapy, nonceruloplasmin-bound copper was reduced from mean 1.53 (0.01-6.95) at baseline to 0.62 (0.01-4.57) ¦Ìmol/l. 24h-urinary copper excretion diminished to 1.85 (0.8-9.6) ¦Ìmol/day approximating the therapeutic goal of 1.6¦Ìmol/day. Seven of 31 patients (22.6%) required discontinuation of TD treatment, in 4 cases it was because of adverse events (ulcerative colitis, gingival and breast hypertrophy, hirsutism, elevation of transaminases). Investigations about weight-based dosage showed no significant difference of any laboratory parameter between the 2 cohorts. But in terms of clin. safety, adverse effects because of TD were only found in 6.7% of children in group 1 (<20mg¡¤kg-1¡¤day-1, median follow-up 60 [14-60] months), it was 63.6%. TD proves to be an efficacious alternative chelating agent for children with WD. Weight-based dosages above the recommended 20mg¡¤kg-1¡¤day-1 may increase the rate of adverse effects in pediatric patients.
Journal of Pediatric Gastroenterology and Nutrition published new progress about 38260-01-4. 38260-01-4 belongs to catalysis-chemistry, auxiliary class Chelating Agents, name is N1,N1′-(Ethane-1,2-diyl)bis(ethane-1,2-diamine) dihydrochloride, and the molecular formula is C6H20Cl2N4, Recommanded Product: N1,N1′-(Ethane-1,2-diyl)bis(ethane-1,2-diamine) dihydrochloride.
Referemce:
https://courses.lumenlearning.com/boundless-chemistry/chapter/catalysis/,
Catalysis – Wikipedia