Adequate Chelation and Cupriuresis in Hepatic Wilson Disease Patients Under Combination (Chelator + Zinc) Therapy at 2 Years of Follow-up

Panda, Kalpana; Lal, Bikrant B.; Sood, Vikrant; Khanna, Rajeev; Alam, Seema

doi:10.1016/j.jceh.2023.09.005

Cited by 2 publications

(2 citation statements)

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“…The inclusion criteria of the present study were designed drawing insights from an earlier study including our pediatric Wilson cohort, where 80% of compliant patients achieved "Criteria for adequate chelation (CAC)" within 1-2 years of combination therapy. 34 By choosing those "CAC," as inclusion criteria, it was ensured that all recruited patients had been adequately chelated before D-pen stoppage. Children with mildly elevated liver enzymes (40-60 U/L) were also included (only if they fulfilled all other criteria) to ascertain whether transaminase levels at D-pen stoppage have predictive value for relapse and if so, then to establish a cut-off for such prediction.…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Relapse following withdrawal of D‐penicillamine from combination (D‐penicillamine + zinc) therapy in hepatic Wilson disease

Panda,

Lal,

Sood

et al. 2024

J. pediatr. gastroenterol. nutr.

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ObjectivesLong‐term D‐penicillamine (D‐pen) therapy in Wilson disease (WD) has numerous adverse effects which advocates its withdrawal, but with an inherent risk of relapse. This prospective observational study was conducted with the objective of evaluating incidence of relapse following withdrawal of D‐pen from combination (D‐pen + zinc) therapy in maintenance phase of previously symptomatic hepatic WD.MethodsHepatic WD patients <18 years of age and on combination therapy for >2 years with 6 months of biochemical remission were included. Biochemical remission was defined as achievement of (i) aspartate aminotransferase (AST) and alanine aminotransferase (ALT) ≤1.5 times upper limit of normal (ULN), (ii) serum albumin >3.5 g/dL, international normalized ratio (INR) <1.5 and (iii) 24‐h urinary copper excretion (UCE) <500 mcg/day, nonceruloplasmin‐bound‐copper (NCC) <15 mcg/dL. After D‐pen withdrawal, monthly liver function test (LFT) and INR and 3 monthly UCE and NCC were done till 1 year or relapse (elevation of AST/ALT/both >2 times ULN or total bilirubin >2 mg/dL), whichever occurred earlier.ResultsForty‐five patients enrolled with median combination therapy duration of 36 months. Sixty percent of them had their index presentation as decompensated cirrhosis. Fourteen patients (31.8%) relapsed (cumulative incidence: 4 at 3 months, 11 at 6 months, and 14 at 12 months after D‐pen discontinuation). All relapsers had index presentation as decompensated cirrhosis. On cox‐regression, ALT at D‐pen withdrawal was an independent predictor of relapse (hazard ratio [HR]: 1.077, 95% confidence interval [CI]: 1.014–1.145, p = 0.017) with AUROC of 0.860. ALT ≥40 U/L predicted risk of relapse with 85.7% sensitivity, 70.9% specificity.ConclusionIncidence of relapse after withdrawal of D‐pen from combination therapy is 31.8% in hepatic WD. ALT ≥40 U/L, at the time of D‐pen stoppage, predicts future relapse.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%