Maintenance zinc therapy after initial penicillamine chelation to treat symptomatic hepatic Wilson’s disease in resource constrained setting
Background Experience with zinc in treating symptomatic hepatic Wilson’s disease (WD) is limited. Aim To study the efficacy of Penicillamine followed by zinc in treating symptomatic hepatic Wilson’s disease. Methods We retrospectively analyzed case records of 31 symptomatic hepatic WD patients for w...
Ausführliche Beschreibung
Gespeichert in:
| Autor*in: |
Gupta, Piyush [verfasserIn] |
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| Format: |
E-Artikel |
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| Sprache: |
Englisch |
| Erschienen: |
2018 |
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| Schlagwörter: |
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| Anmerkung: |
© Indian Society of Gastroenterology 2018 |
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| Übergeordnetes Werk: |
Enthalten in: Indian Journal of Gastroenterology - Springer-Verlag, 2009, 37(2018), 1 vom: Jan., Seite 31-38 |
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| Übergeordnetes Werk: |
volume:37 ; year:2018 ; number:1 ; month:01 ; pages:31-38 |
| Links: |
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| DOI / URN: |
10.1007/s12664-018-0829-x |
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SPR026661489 |
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| 520 | |a Background Experience with zinc in treating symptomatic hepatic Wilson’s disease (WD) is limited. Aim To study the efficacy of Penicillamine followed by zinc in treating symptomatic hepatic Wilson’s disease. Methods We retrospectively analyzed case records of 31 symptomatic hepatic WD patients for whom disease severity scores (Child’s, model for end-stage liver disease (MELD), Nazer’s, and New Wilson Index (NWI) score) and 24-h urinary copper were compared at 3-time points—baseline at presentation, at transition from penicillamine to zinc and at end of follow up. Results Thirty-one patients (median age 11 [5–24] years) with symptomatic hepatic WD were studied; ten had associated neuropsychiatric manifestations of WD. Penicillamine was changed to zinc sulfate either due to financial constraints (28 patients) or due to adverse effects of penicillamine (3 patients). At presentation (baseline), six patients belonged to Child’s class A, five to Child’s B, and 17 to Child’s C. Duration of initial penicillamine chelation therapy was 134 (2–320) weeks, and of subsequent zinc therapy was 363 (35–728) weeks. There was a significant improvement in liver function tests and disease severity scores (Child’s, MELD, Nazer’s, and NWI score) at the transition from penicillamine to zinc compared to baseline. This improvement was maintained until the end of study period with 90% survival at 10 (2–20) years. Fifteen of the 17 Child’s C cirrhotic patients showed significant improvement in disease severity scores from baseline until end of follow up. Conclusions Penicillamine followed by zinc may be a safe and effective treatment in resource-constrained setting for symptomatic hepatic WD patients in all grades of baseline disease severity. Some patients with decompensated cirrhosis due to WD may be managed with medical treatment, avoiding liver transplantation. | ||
| 650 | 4 | |a Hepatic Wilson’s disease |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Penicillamine |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Symptomatic Wilson’s |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Wilson’s disease |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Zinc |7 (dpeaa)DE-He213 | |
| 700 | 1 | |a Choksi, Mehul |4 aut | |
| 700 | 1 | |a Goel, Ashish |4 aut | |
| 700 | 1 | |a Zachariah, Uday |4 aut | |
| 700 | 1 | |a Sajith, Kattiparambil Gangadharan |4 aut | |
| 700 | 1 | |a Ramachandran, Jeyamani |4 aut | |
| 700 | 1 | |a Chandy, George |4 aut | |
| 700 | 1 | |a Kurian, George |4 aut | |
| 700 | 1 | |a Rebekah, Grace |4 aut | |
| 700 | 1 | |a Eapen, Chundamannil Eapen |4 aut | |
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10.1007/s12664-018-0829-x doi (DE-627)SPR026661489 (SPR)s12664-018-0829-x-e DE-627 ger DE-627 rakwb eng Gupta, Piyush verfasserin (orcid)0000-0002-3670-2800 aut Maintenance zinc therapy after initial penicillamine chelation to treat symptomatic hepatic Wilson’s disease in resource constrained setting 2018 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Indian Society of Gastroenterology 2018 Background Experience with zinc in treating symptomatic hepatic Wilson’s disease (WD) is limited. Aim To study the efficacy of Penicillamine followed by zinc in treating symptomatic hepatic Wilson’s disease. Methods We retrospectively analyzed case records of 31 symptomatic hepatic WD patients for whom disease severity scores (Child’s, model for end-stage liver disease (MELD), Nazer’s, and New Wilson Index (NWI) score) and 24-h urinary copper were compared at 3-time points—baseline at presentation, at transition from penicillamine to zinc and at end of follow up. Results Thirty-one patients (median age 11 [5–24] years) with symptomatic hepatic WD were studied; ten had associated neuropsychiatric manifestations of WD. Penicillamine was changed to zinc sulfate either due to financial constraints (28 patients) or due to adverse effects of penicillamine (3 patients). At presentation (baseline), six patients belonged to Child’s class A, five to Child’s B, and 17 to Child’s C. Duration of initial penicillamine chelation therapy was 134 (2–320) weeks, and of subsequent zinc therapy was 363 (35–728) weeks. There was a significant improvement in liver function tests and disease severity scores (Child’s, MELD, Nazer’s, and NWI score) at the transition from penicillamine to zinc compared to baseline. This improvement was maintained until the end of study period with 90% survival at 10 (2–20) years. Fifteen of the 17 Child’s C cirrhotic patients showed significant improvement in disease severity scores from baseline until end of follow up. Conclusions Penicillamine followed by zinc may be a safe and effective treatment in resource-constrained setting for symptomatic hepatic WD patients in all grades of baseline disease severity. Some patients with decompensated cirrhosis due to WD may be managed with medical treatment, avoiding liver transplantation. Hepatic Wilson’s disease (dpeaa)DE-He213 Penicillamine (dpeaa)DE-He213 Symptomatic Wilson’s (dpeaa)DE-He213 Wilson’s disease (dpeaa)DE-He213 Zinc (dpeaa)DE-He213 Choksi, Mehul aut Goel, Ashish aut Zachariah, Uday aut Sajith, Kattiparambil Gangadharan aut Ramachandran, Jeyamani aut Chandy, George aut Kurian, George aut Rebekah, Grace aut Eapen, Chundamannil Eapen aut Enthalten in Indian Journal of Gastroenterology Springer-Verlag, 2009 37(2018), 1 vom: Jan., Seite 31-38 (DE-627)SPR02665167X nnns volume:37 year:2018 number:1 month:01 pages:31-38 https://dx.doi.org/10.1007/s12664-018-0829-x lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA AR 37 2018 1 01 31-38 |
| spelling |
10.1007/s12664-018-0829-x doi (DE-627)SPR026661489 (SPR)s12664-018-0829-x-e DE-627 ger DE-627 rakwb eng Gupta, Piyush verfasserin (orcid)0000-0002-3670-2800 aut Maintenance zinc therapy after initial penicillamine chelation to treat symptomatic hepatic Wilson’s disease in resource constrained setting 2018 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Indian Society of Gastroenterology 2018 Background Experience with zinc in treating symptomatic hepatic Wilson’s disease (WD) is limited. Aim To study the efficacy of Penicillamine followed by zinc in treating symptomatic hepatic Wilson’s disease. Methods We retrospectively analyzed case records of 31 symptomatic hepatic WD patients for whom disease severity scores (Child’s, model for end-stage liver disease (MELD), Nazer’s, and New Wilson Index (NWI) score) and 24-h urinary copper were compared at 3-time points—baseline at presentation, at transition from penicillamine to zinc and at end of follow up. Results Thirty-one patients (median age 11 [5–24] years) with symptomatic hepatic WD were studied; ten had associated neuropsychiatric manifestations of WD. Penicillamine was changed to zinc sulfate either due to financial constraints (28 patients) or due to adverse effects of penicillamine (3 patients). At presentation (baseline), six patients belonged to Child’s class A, five to Child’s B, and 17 to Child’s C. Duration of initial penicillamine chelation therapy was 134 (2–320) weeks, and of subsequent zinc therapy was 363 (35–728) weeks. There was a significant improvement in liver function tests and disease severity scores (Child’s, MELD, Nazer’s, and NWI score) at the transition from penicillamine to zinc compared to baseline. This improvement was maintained until the end of study period with 90% survival at 10 (2–20) years. Fifteen of the 17 Child’s C cirrhotic patients showed significant improvement in disease severity scores from baseline until end of follow up. Conclusions Penicillamine followed by zinc may be a safe and effective treatment in resource-constrained setting for symptomatic hepatic WD patients in all grades of baseline disease severity. Some patients with decompensated cirrhosis due to WD may be managed with medical treatment, avoiding liver transplantation. Hepatic Wilson’s disease (dpeaa)DE-He213 Penicillamine (dpeaa)DE-He213 Symptomatic Wilson’s (dpeaa)DE-He213 Wilson’s disease (dpeaa)DE-He213 Zinc (dpeaa)DE-He213 Choksi, Mehul aut Goel, Ashish aut Zachariah, Uday aut Sajith, Kattiparambil Gangadharan aut Ramachandran, Jeyamani aut Chandy, George aut Kurian, George aut Rebekah, Grace aut Eapen, Chundamannil Eapen aut Enthalten in Indian Journal of Gastroenterology Springer-Verlag, 2009 37(2018), 1 vom: Jan., Seite 31-38 (DE-627)SPR02665167X nnns volume:37 year:2018 number:1 month:01 pages:31-38 https://dx.doi.org/10.1007/s12664-018-0829-x lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA AR 37 2018 1 01 31-38 |
| allfields_unstemmed |
10.1007/s12664-018-0829-x doi (DE-627)SPR026661489 (SPR)s12664-018-0829-x-e DE-627 ger DE-627 rakwb eng Gupta, Piyush verfasserin (orcid)0000-0002-3670-2800 aut Maintenance zinc therapy after initial penicillamine chelation to treat symptomatic hepatic Wilson’s disease in resource constrained setting 2018 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Indian Society of Gastroenterology 2018 Background Experience with zinc in treating symptomatic hepatic Wilson’s disease (WD) is limited. Aim To study the efficacy of Penicillamine followed by zinc in treating symptomatic hepatic Wilson’s disease. Methods We retrospectively analyzed case records of 31 symptomatic hepatic WD patients for whom disease severity scores (Child’s, model for end-stage liver disease (MELD), Nazer’s, and New Wilson Index (NWI) score) and 24-h urinary copper were compared at 3-time points—baseline at presentation, at transition from penicillamine to zinc and at end of follow up. Results Thirty-one patients (median age 11 [5–24] years) with symptomatic hepatic WD were studied; ten had associated neuropsychiatric manifestations of WD. Penicillamine was changed to zinc sulfate either due to financial constraints (28 patients) or due to adverse effects of penicillamine (3 patients). At presentation (baseline), six patients belonged to Child’s class A, five to Child’s B, and 17 to Child’s C. Duration of initial penicillamine chelation therapy was 134 (2–320) weeks, and of subsequent zinc therapy was 363 (35–728) weeks. There was a significant improvement in liver function tests and disease severity scores (Child’s, MELD, Nazer’s, and NWI score) at the transition from penicillamine to zinc compared to baseline. This improvement was maintained until the end of study period with 90% survival at 10 (2–20) years. Fifteen of the 17 Child’s C cirrhotic patients showed significant improvement in disease severity scores from baseline until end of follow up. Conclusions Penicillamine followed by zinc may be a safe and effective treatment in resource-constrained setting for symptomatic hepatic WD patients in all grades of baseline disease severity. Some patients with decompensated cirrhosis due to WD may be managed with medical treatment, avoiding liver transplantation. Hepatic Wilson’s disease (dpeaa)DE-He213 Penicillamine (dpeaa)DE-He213 Symptomatic Wilson’s (dpeaa)DE-He213 Wilson’s disease (dpeaa)DE-He213 Zinc (dpeaa)DE-He213 Choksi, Mehul aut Goel, Ashish aut Zachariah, Uday aut Sajith, Kattiparambil Gangadharan aut Ramachandran, Jeyamani aut Chandy, George aut Kurian, George aut Rebekah, Grace aut Eapen, Chundamannil Eapen aut Enthalten in Indian Journal of Gastroenterology Springer-Verlag, 2009 37(2018), 1 vom: Jan., Seite 31-38 (DE-627)SPR02665167X nnns volume:37 year:2018 number:1 month:01 pages:31-38 https://dx.doi.org/10.1007/s12664-018-0829-x lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA AR 37 2018 1 01 31-38 |
| allfieldsGer |
10.1007/s12664-018-0829-x doi (DE-627)SPR026661489 (SPR)s12664-018-0829-x-e DE-627 ger DE-627 rakwb eng Gupta, Piyush verfasserin (orcid)0000-0002-3670-2800 aut Maintenance zinc therapy after initial penicillamine chelation to treat symptomatic hepatic Wilson’s disease in resource constrained setting 2018 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Indian Society of Gastroenterology 2018 Background Experience with zinc in treating symptomatic hepatic Wilson’s disease (WD) is limited. Aim To study the efficacy of Penicillamine followed by zinc in treating symptomatic hepatic Wilson’s disease. Methods We retrospectively analyzed case records of 31 symptomatic hepatic WD patients for whom disease severity scores (Child’s, model for end-stage liver disease (MELD), Nazer’s, and New Wilson Index (NWI) score) and 24-h urinary copper were compared at 3-time points—baseline at presentation, at transition from penicillamine to zinc and at end of follow up. Results Thirty-one patients (median age 11 [5–24] years) with symptomatic hepatic WD were studied; ten had associated neuropsychiatric manifestations of WD. Penicillamine was changed to zinc sulfate either due to financial constraints (28 patients) or due to adverse effects of penicillamine (3 patients). At presentation (baseline), six patients belonged to Child’s class A, five to Child’s B, and 17 to Child’s C. Duration of initial penicillamine chelation therapy was 134 (2–320) weeks, and of subsequent zinc therapy was 363 (35–728) weeks. There was a significant improvement in liver function tests and disease severity scores (Child’s, MELD, Nazer’s, and NWI score) at the transition from penicillamine to zinc compared to baseline. This improvement was maintained until the end of study period with 90% survival at 10 (2–20) years. Fifteen of the 17 Child’s C cirrhotic patients showed significant improvement in disease severity scores from baseline until end of follow up. Conclusions Penicillamine followed by zinc may be a safe and effective treatment in resource-constrained setting for symptomatic hepatic WD patients in all grades of baseline disease severity. Some patients with decompensated cirrhosis due to WD may be managed with medical treatment, avoiding liver transplantation. Hepatic Wilson’s disease (dpeaa)DE-He213 Penicillamine (dpeaa)DE-He213 Symptomatic Wilson’s (dpeaa)DE-He213 Wilson’s disease (dpeaa)DE-He213 Zinc (dpeaa)DE-He213 Choksi, Mehul aut Goel, Ashish aut Zachariah, Uday aut Sajith, Kattiparambil Gangadharan aut Ramachandran, Jeyamani aut Chandy, George aut Kurian, George aut Rebekah, Grace aut Eapen, Chundamannil Eapen aut Enthalten in Indian Journal of Gastroenterology Springer-Verlag, 2009 37(2018), 1 vom: Jan., Seite 31-38 (DE-627)SPR02665167X nnns volume:37 year:2018 number:1 month:01 pages:31-38 https://dx.doi.org/10.1007/s12664-018-0829-x lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA AR 37 2018 1 01 31-38 |
| allfieldsSound |
10.1007/s12664-018-0829-x doi (DE-627)SPR026661489 (SPR)s12664-018-0829-x-e DE-627 ger DE-627 rakwb eng Gupta, Piyush verfasserin (orcid)0000-0002-3670-2800 aut Maintenance zinc therapy after initial penicillamine chelation to treat symptomatic hepatic Wilson’s disease in resource constrained setting 2018 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Indian Society of Gastroenterology 2018 Background Experience with zinc in treating symptomatic hepatic Wilson’s disease (WD) is limited. Aim To study the efficacy of Penicillamine followed by zinc in treating symptomatic hepatic Wilson’s disease. Methods We retrospectively analyzed case records of 31 symptomatic hepatic WD patients for whom disease severity scores (Child’s, model for end-stage liver disease (MELD), Nazer’s, and New Wilson Index (NWI) score) and 24-h urinary copper were compared at 3-time points—baseline at presentation, at transition from penicillamine to zinc and at end of follow up. Results Thirty-one patients (median age 11 [5–24] years) with symptomatic hepatic WD were studied; ten had associated neuropsychiatric manifestations of WD. Penicillamine was changed to zinc sulfate either due to financial constraints (28 patients) or due to adverse effects of penicillamine (3 patients). At presentation (baseline), six patients belonged to Child’s class A, five to Child’s B, and 17 to Child’s C. Duration of initial penicillamine chelation therapy was 134 (2–320) weeks, and of subsequent zinc therapy was 363 (35–728) weeks. There was a significant improvement in liver function tests and disease severity scores (Child’s, MELD, Nazer’s, and NWI score) at the transition from penicillamine to zinc compared to baseline. This improvement was maintained until the end of study period with 90% survival at 10 (2–20) years. Fifteen of the 17 Child’s C cirrhotic patients showed significant improvement in disease severity scores from baseline until end of follow up. Conclusions Penicillamine followed by zinc may be a safe and effective treatment in resource-constrained setting for symptomatic hepatic WD patients in all grades of baseline disease severity. Some patients with decompensated cirrhosis due to WD may be managed with medical treatment, avoiding liver transplantation. Hepatic Wilson’s disease (dpeaa)DE-He213 Penicillamine (dpeaa)DE-He213 Symptomatic Wilson’s (dpeaa)DE-He213 Wilson’s disease (dpeaa)DE-He213 Zinc (dpeaa)DE-He213 Choksi, Mehul aut Goel, Ashish aut Zachariah, Uday aut Sajith, Kattiparambil Gangadharan aut Ramachandran, Jeyamani aut Chandy, George aut Kurian, George aut Rebekah, Grace aut Eapen, Chundamannil Eapen aut Enthalten in Indian Journal of Gastroenterology Springer-Verlag, 2009 37(2018), 1 vom: Jan., Seite 31-38 (DE-627)SPR02665167X nnns volume:37 year:2018 number:1 month:01 pages:31-38 https://dx.doi.org/10.1007/s12664-018-0829-x lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA AR 37 2018 1 01 31-38 |
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Aim To study the efficacy of Penicillamine followed by zinc in treating symptomatic hepatic Wilson’s disease. Methods We retrospectively analyzed case records of 31 symptomatic hepatic WD patients for whom disease severity scores (Child’s, model for end-stage liver disease (MELD), Nazer’s, and New Wilson Index (NWI) score) and 24-h urinary copper were compared at 3-time points—baseline at presentation, at transition from penicillamine to zinc and at end of follow up. Results Thirty-one patients (median age 11 [5–24] years) with symptomatic hepatic WD were studied; ten had associated neuropsychiatric manifestations of WD. Penicillamine was changed to zinc sulfate either due to financial constraints (28 patients) or due to adverse effects of penicillamine (3 patients). At presentation (baseline), six patients belonged to Child’s class A, five to Child’s B, and 17 to Child’s C. Duration of initial penicillamine chelation therapy was 134 (2–320) weeks, and of subsequent zinc therapy was 363 (35–728) weeks. There was a significant improvement in liver function tests and disease severity scores (Child’s, MELD, Nazer’s, and NWI score) at the transition from penicillamine to zinc compared to baseline. This improvement was maintained until the end of study period with 90% survival at 10 (2–20) years. Fifteen of the 17 Child’s C cirrhotic patients showed significant improvement in disease severity scores from baseline until end of follow up. Conclusions Penicillamine followed by zinc may be a safe and effective treatment in resource-constrained setting for symptomatic hepatic WD patients in all grades of baseline disease severity. 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Gupta, Piyush Choksi, Mehul Goel, Ashish Zachariah, Uday Sajith, Kattiparambil Gangadharan Ramachandran, Jeyamani Chandy, George Kurian, George Rebekah, Grace Eapen, Chundamannil Eapen |
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maintenance zinc therapy after initial penicillamine chelation to treat symptomatic hepatic wilson’s disease in resource constrained setting |
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Maintenance zinc therapy after initial penicillamine chelation to treat symptomatic hepatic Wilson’s disease in resource constrained setting |
| abstract |
Background Experience with zinc in treating symptomatic hepatic Wilson’s disease (WD) is limited. Aim To study the efficacy of Penicillamine followed by zinc in treating symptomatic hepatic Wilson’s disease. Methods We retrospectively analyzed case records of 31 symptomatic hepatic WD patients for whom disease severity scores (Child’s, model for end-stage liver disease (MELD), Nazer’s, and New Wilson Index (NWI) score) and 24-h urinary copper were compared at 3-time points—baseline at presentation, at transition from penicillamine to zinc and at end of follow up. Results Thirty-one patients (median age 11 [5–24] years) with symptomatic hepatic WD were studied; ten had associated neuropsychiatric manifestations of WD. Penicillamine was changed to zinc sulfate either due to financial constraints (28 patients) or due to adverse effects of penicillamine (3 patients). At presentation (baseline), six patients belonged to Child’s class A, five to Child’s B, and 17 to Child’s C. Duration of initial penicillamine chelation therapy was 134 (2–320) weeks, and of subsequent zinc therapy was 363 (35–728) weeks. There was a significant improvement in liver function tests and disease severity scores (Child’s, MELD, Nazer’s, and NWI score) at the transition from penicillamine to zinc compared to baseline. This improvement was maintained until the end of study period with 90% survival at 10 (2–20) years. Fifteen of the 17 Child’s C cirrhotic patients showed significant improvement in disease severity scores from baseline until end of follow up. Conclusions Penicillamine followed by zinc may be a safe and effective treatment in resource-constrained setting for symptomatic hepatic WD patients in all grades of baseline disease severity. Some patients with decompensated cirrhosis due to WD may be managed with medical treatment, avoiding liver transplantation. © Indian Society of Gastroenterology 2018 |
| abstractGer |
Background Experience with zinc in treating symptomatic hepatic Wilson’s disease (WD) is limited. Aim To study the efficacy of Penicillamine followed by zinc in treating symptomatic hepatic Wilson’s disease. Methods We retrospectively analyzed case records of 31 symptomatic hepatic WD patients for whom disease severity scores (Child’s, model for end-stage liver disease (MELD), Nazer’s, and New Wilson Index (NWI) score) and 24-h urinary copper were compared at 3-time points—baseline at presentation, at transition from penicillamine to zinc and at end of follow up. Results Thirty-one patients (median age 11 [5–24] years) with symptomatic hepatic WD were studied; ten had associated neuropsychiatric manifestations of WD. Penicillamine was changed to zinc sulfate either due to financial constraints (28 patients) or due to adverse effects of penicillamine (3 patients). At presentation (baseline), six patients belonged to Child’s class A, five to Child’s B, and 17 to Child’s C. Duration of initial penicillamine chelation therapy was 134 (2–320) weeks, and of subsequent zinc therapy was 363 (35–728) weeks. There was a significant improvement in liver function tests and disease severity scores (Child’s, MELD, Nazer’s, and NWI score) at the transition from penicillamine to zinc compared to baseline. This improvement was maintained until the end of study period with 90% survival at 10 (2–20) years. Fifteen of the 17 Child’s C cirrhotic patients showed significant improvement in disease severity scores from baseline until end of follow up. Conclusions Penicillamine followed by zinc may be a safe and effective treatment in resource-constrained setting for symptomatic hepatic WD patients in all grades of baseline disease severity. Some patients with decompensated cirrhosis due to WD may be managed with medical treatment, avoiding liver transplantation. © Indian Society of Gastroenterology 2018 |
| abstract_unstemmed |
Background Experience with zinc in treating symptomatic hepatic Wilson’s disease (WD) is limited. Aim To study the efficacy of Penicillamine followed by zinc in treating symptomatic hepatic Wilson’s disease. Methods We retrospectively analyzed case records of 31 symptomatic hepatic WD patients for whom disease severity scores (Child’s, model for end-stage liver disease (MELD), Nazer’s, and New Wilson Index (NWI) score) and 24-h urinary copper were compared at 3-time points—baseline at presentation, at transition from penicillamine to zinc and at end of follow up. Results Thirty-one patients (median age 11 [5–24] years) with symptomatic hepatic WD were studied; ten had associated neuropsychiatric manifestations of WD. Penicillamine was changed to zinc sulfate either due to financial constraints (28 patients) or due to adverse effects of penicillamine (3 patients). At presentation (baseline), six patients belonged to Child’s class A, five to Child’s B, and 17 to Child’s C. Duration of initial penicillamine chelation therapy was 134 (2–320) weeks, and of subsequent zinc therapy was 363 (35–728) weeks. There was a significant improvement in liver function tests and disease severity scores (Child’s, MELD, Nazer’s, and NWI score) at the transition from penicillamine to zinc compared to baseline. This improvement was maintained until the end of study period with 90% survival at 10 (2–20) years. Fifteen of the 17 Child’s C cirrhotic patients showed significant improvement in disease severity scores from baseline until end of follow up. Conclusions Penicillamine followed by zinc may be a safe and effective treatment in resource-constrained setting for symptomatic hepatic WD patients in all grades of baseline disease severity. Some patients with decompensated cirrhosis due to WD may be managed with medical treatment, avoiding liver transplantation. © Indian Society of Gastroenterology 2018 |
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