TABLE 1.
The characteristics of the included studies.
| Author | Year | Country | Study design | Patient population | Treatment | Outcome | Patients (n) (original size) |
|---|---|---|---|---|---|---|---|
| Couchonnal (Couchonnal et al., 2021) | 2021 | France | Retrospective and prospectively study | WD diagnosis 1995–2019 | 1. d-Penicillamine | Side effects (1) | 17 [131] |
| Age: 10.7 ± 4.2 (1–18) | 2. Zinc salts | Side effects (2) | 0 [24] | ||||
| Presentation: neurologic; hepatic symptoms; asymptomatic | 3. Trientine | Side effects (3) | 0 [17] | ||||
| Improved (1) | 109 [131] | ||||||
| Improved (2) | 10 [18] | ||||||
| Zhang (Zhang et al., 2020) | 2020 | China | Retrospective study | WD diagnosis 2015–2018 | 1. d-Penicillamine(5 mg/kg/d) | Neurological improved (1) | 37 [65] |
| Age: 24.47 ± 8.00 | |||||||
| Presentation: neurologic | |||||||
| Zhou (Zhou et al., 2020) | 2020 | China | Prospectively study | WD diagnosis 2015–2019 | 1. d-Penicillamine | Side effects (1) | 13 [38] |
| Presentation: neurologic; hepatic symptoms | 2. Zinc gluconate | Side effects (2) Neurological improved (1) | 5 [10] | ||||
| 13 [38] | |||||||
| Mayr (Mayr et al., 2021) | 2020 | Germany | Retrospective study | WD diagnosis 1980–1992 | 1. d-Penicillamine | Side effects (1) | 24 [26] |
| Age: <18 | 2. Trientine | Side effects (2) | 10 [27] | ||||
| Presentation: neurologic; hepatic symptoms | |||||||
| Litwin (Litwin et al., 2015) | 2015 | Poland | Retrospective study | WD diagnosis 2005–2009 | 1. d-Penicillamine | Neurological deterioration (1) | 12 [42] |
| Presentation: neurologic | 2. Zinc sulfate | Neurological deterioration (2) | 4 [28] | ||||
| Kalita (Kalita et al., 2015) | 2015 | India | Cohort study | Age: 11 (5–37) | 1. d-Penicillamine (750 mg/day) | Neurological deterioration (1) | 12 [42] |
| Presentation: neurologic | 2. Elemental zinc (150 mg/day) | Neurological deterioration (2) | 0 [9] | ||||
| Członkowska (Członkowska et al., 2014) | 2014 | Poland | Retrospective study | WD diagnosis 2005–2009 | 1. d-Penicillamine | Neurological deterioration (1) | 4 [35] |
| Presentation: neurologic; hepatic symptoms | 2. Zinc sulfate | Neurological deterioration (2) | 1 [21] | ||||
| Neurological improved (1) | 29 [35] | ||||||
| Neurological improved (2) | 15 [21] | ||||||
| Hepatic improved (1) | 34 [36] | ||||||
| Hepatic improved (2) | 48 [51] | ||||||
| Sini (Sini et al., 2013) | 2013 | Italy | Cohort study | WD diagnosis 1970–2000 | 1. d-Penicillamine (600–1,200 mg/day) | Hepatic improved (1) | 12 [13] |
| Presentation: hepatic symptoms | 2. Elemental zinc | Hepatic improved (2) | 3 [3] | ||||
| (150–300 mg/day) | |||||||
| Rodriguez (Rodríguez et al., 2012) | 2012 | Spain | Retrospective study | Age: 22 (6–50) | 1. d-Penicillamine | Side effects (1) | 4 [18] |
| Presentation: neurologic; hepatic symptoms | 2. Zinc salts | Side effects (2) | 0 [2] | ||||
| improved (1) | 11 [18] | ||||||
| improved (2) | 2 [2] | ||||||
| Weiss (Weiss et al., 2011) | 2011 | Germany | Retrospective cohort study | Presentation: neurologic; hepatic symptoms; asymptomatic | 1. d-Penicillamine | Side effects (1) | 99 [313] |
| 2. Zinc salts | Side effects (2) | 10 [88] | |||||
| 3. Trientine | Side effects (3) | 9 [102] | |||||
| Neurological deterioration (1) | 22 [243] | ||||||
| Neurological deterioration (2) | 9 [95] | ||||||
| Masebas (Maselbas et al., 2010) | 2010 | Poland | Retrospective study | Presentation: neurologic; hepatic symptoms; asymptomatic | 1. d-Penicillamine | Neurological deterioration (1) | 6 [55] |
| 2. Zinc sulfate | Neurological deterioration (2) | 3 [47] | |||||
| improved (1) | 44 [55] | ||||||
| improved (2) | 37 [47] | ||||||
| Yokoyama (Cope-Yokoyama et al., 2010) | 2010 | Italy | Cohort study | WD diagnosis 1981–2006 | 1. d-Penicillamine | Hepatic improved (1) | 3 [5] |
| Age: 17.3 (6–35) | 2. Zinc sulfate | Hepatic improved (2) | 3 [7] | ||||
| Presentation: hepatic symptoms | |||||||
| Bruha (Bruha et al., 2011) | 2010 | Czech Republic | Retrospective study | WD diagnosis 1965–2008 | 1. d-Penicillamine (600–1,200 mg/day) | Neurological deterioration (1) | 2 [50] |
| Age: 38.5 ± 11 (16–63) | 2. Elemental zinc | Neurological deterioration (2) | 0 [3] | ||||
| Presentation: neurologic; hepatic symptoms; asymptomatic | (150 mg/day) | Neurological improved (1) | 36 [50] | ||||
| Neurological improved (2) | 2 [3] | ||||||
| Hepatic improved (1) | 32 [40] | ||||||
| Hepatic improved (2) | 7 [8] | ||||||
| Merle (Merle et al., 2007) | 2007 | Germany | Cohort study | WD diagnosis 2000–2005 | 1. d-Penicillamine (900–1,800 mg/day) | Side effects (1) | 78 [138] |
| Age: 20.4 ± 10.6 (4–56) | 2. Trientine | Side effects (2) | 4 [140] | ||||
| Presentation: neurologic; hepatic symptoms; asymptomatic | (900–2,100 mg/day) | Side effects (3) | 19 [140] | ||||
| 3. Elemental zinc | Neurological deterioration (1) | 19 [138] | |||||
| (150–250 mg/day) | Neurological deterioration (3) | 6 [140] | |||||
| Medici (Medici et al., 2006) | 2006 | Italy | Retrospective study | WD diagnosis 1980–2005 | 1. d-Penicillamine (600–1,800 mg/day) | Neurological deterioration (1) | 6 [8] |
| Age: 15.5 ± 7.2 (4–35) | 2. Zinc sulfate | Neurological deterioration (2) | 1 [10] | ||||
| Presentation: neurologic; hepatic symptoms | (660–880 mg/day) | Neurological improved (1) | 2 [8] | ||||
| Neurological improved (2) | 9 [10] | ||||||
| Hepatic improved (1) | 7 [17] | ||||||
| Hepatic improved (2) | 10 [20] | ||||||
| Czlonkowska (Czlonkowska et al., 1996) | 1996 | Poland | Retrospective study | WD diagnosis 1980–1992 | 1. d-Penicillamine (1,000–1,500 mg/day) | Side effects (1) | 15 [34] |
| Presentation: neurologic; psychiatric; hepatic symptoms; asymptomatic | 2. Zinc sulfate | Side effects (2) | 4 [33] | ||||
| (600–800 mg/day) | Neurological deterioration (1) | 3 [34] | |||||
| Neurological deterioration (2) | 2 [33] | ||||||
| improved (1) | 13 [19] | ||||||
| improved (2) | 23 [29] |