Outcome of Wilson’s disease in Bangladeshi children: a tertiary center experience
Abstract Background Wilson disease (WD) is an inherited disorder of copper metabolism commonly involving the liver, cornea, and brain. Its incidence is increasing day by day worldwide. Early diagnosis and prompt treatment are the key for best outcome. Material and methods A cross-sectional descripti...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
SpringerOpen
2022-11-01
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| Series: | Egyptian Liver Journal |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s43066-022-00228-6 |
| _version_ | 1811189400268177408 |
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| author | Salahuddin Mahmud Jahida Gulshan Madhabi Baidya Rafia Rashid Farhana Tasneem Ahmed Rashidul Hasan Tanzila Farhana Syed Shafi Ahmed |
| author_facet | Salahuddin Mahmud Jahida Gulshan Madhabi Baidya Rafia Rashid Farhana Tasneem Ahmed Rashidul Hasan Tanzila Farhana Syed Shafi Ahmed |
| author_sort | Salahuddin Mahmud |
| collection | DOAJ |
| description | Abstract Background Wilson disease (WD) is an inherited disorder of copper metabolism commonly involving the liver, cornea, and brain. Its incidence is increasing day by day worldwide. Early diagnosis and prompt treatment are the key for best outcome. Material and methods A cross-sectional descriptive study was done from January 2014 to December 2019. Sixty children of both genders between 3 and 18 years were diagnosed by clinical and laboratory profile meeting selected criteria. Results Mean age was 8.42 ± 2.6 years and male female ratio was 1.5:1. Consanguinity of marriage was found in 38.3% cases. Seventy percent of cases were hepatic, 16.7% were neuropsychiatric, 5.0% were hepatic with neuropsychiatric, and 8.3% cases were manifested asymptomatically. Asymptomatic and hepatic WD were reported between 3 and 10 years and most of the neuropsychiatric and hepatic with neuropsychiatric manifested after 10 years of age. More than 50% cases improved, a little more than 20% children died, 18.4% were unchanged and 6.6% were hepatic added neuropsychiatric manifestations. Most of the asymptomatic (100%) and hepatic (61.9%) cases improved. High mortality was found with 76.9% cases of acute liver failure (ALF), 7.7% case of chronic liver disease (CLD) and 25% cases of CLD with portal hypertension (CLD and PH). Most of the neuropsychiatric cases (90.0%), and approximately two-third (66.6%) of hepatic with neuropsychiatric cases remained unchanged. Neuropsychiatric manifestations were added in 15.4% cases of CLD and 25% cases of CLD with PH patient. The treatment was well tolerated in 66% children without any side effects. Low WBC (6.3%) and platelet count (4.3%), vomiting (6.3%), anorexia (4.3%), loss of taste (4.3%), rash (4.3%), and proteinuria (2.1%) were found in few cases. Conclusion Majority of the children were presented with hepatic manifestations. More than half of patients with WD treated by D-penicillamine (DP) were improved. Significant mortality was found in acute liver failure whereas neuropsychiatric presentations had persistent abnormalities. No major side effects of DP was observed in most of the cases. Early diagnosis and prompt treatment were crucial for better outcome. |
| first_indexed | 2024-04-11T14:35:11Z |
| format | Article |
| id | doaj.art-69fa0b3ccb9d45f094c98409073f0040 |
| institution | Directory Open Access Journal |
| issn | 2090-6226 |
| language | English |
| last_indexed | 2024-04-11T14:35:11Z |
| publishDate | 2022-11-01 |
| publisher | SpringerOpen |
| record_format | Article |
| series | Egyptian Liver Journal |
| spelling | doaj.art-69fa0b3ccb9d45f094c98409073f00402022-12-22T04:18:21ZengSpringerOpenEgyptian Liver Journal2090-62262022-11-0112111210.1186/s43066-022-00228-6Outcome of Wilson’s disease in Bangladeshi children: a tertiary center experienceSalahuddin Mahmud0Jahida Gulshan1Madhabi Baidya2Rafia Rashid3Farhana Tasneem4Ahmed Rashidul Hasan5Tanzila Farhana6Syed Shafi Ahmed7Bangladesh Shishu Hospital & InstituteBangladesh Shishu Hospital & InstituteBangladesh Shishu Hospital & InstituteBangladesh Shishu Hospital & InstituteBangladesh Shishu Hospital & InstituteBangladesh Shishu Hospital & InstituteBangladesh Shishu Hospital & InstituteBangladesh Shishu Hospital & InstituteAbstract Background Wilson disease (WD) is an inherited disorder of copper metabolism commonly involving the liver, cornea, and brain. Its incidence is increasing day by day worldwide. Early diagnosis and prompt treatment are the key for best outcome. Material and methods A cross-sectional descriptive study was done from January 2014 to December 2019. Sixty children of both genders between 3 and 18 years were diagnosed by clinical and laboratory profile meeting selected criteria. Results Mean age was 8.42 ± 2.6 years and male female ratio was 1.5:1. Consanguinity of marriage was found in 38.3% cases. Seventy percent of cases were hepatic, 16.7% were neuropsychiatric, 5.0% were hepatic with neuropsychiatric, and 8.3% cases were manifested asymptomatically. Asymptomatic and hepatic WD were reported between 3 and 10 years and most of the neuropsychiatric and hepatic with neuropsychiatric manifested after 10 years of age. More than 50% cases improved, a little more than 20% children died, 18.4% were unchanged and 6.6% were hepatic added neuropsychiatric manifestations. Most of the asymptomatic (100%) and hepatic (61.9%) cases improved. High mortality was found with 76.9% cases of acute liver failure (ALF), 7.7% case of chronic liver disease (CLD) and 25% cases of CLD with portal hypertension (CLD and PH). Most of the neuropsychiatric cases (90.0%), and approximately two-third (66.6%) of hepatic with neuropsychiatric cases remained unchanged. Neuropsychiatric manifestations were added in 15.4% cases of CLD and 25% cases of CLD with PH patient. The treatment was well tolerated in 66% children without any side effects. Low WBC (6.3%) and platelet count (4.3%), vomiting (6.3%), anorexia (4.3%), loss of taste (4.3%), rash (4.3%), and proteinuria (2.1%) were found in few cases. Conclusion Majority of the children were presented with hepatic manifestations. More than half of patients with WD treated by D-penicillamine (DP) were improved. Significant mortality was found in acute liver failure whereas neuropsychiatric presentations had persistent abnormalities. No major side effects of DP was observed in most of the cases. Early diagnosis and prompt treatment were crucial for better outcome.https://doi.org/10.1186/s43066-022-00228-6BangladeshChildWilson’s diseaseHepatic presentationNeurological presentation D-Penicillamine |
| spellingShingle | Salahuddin Mahmud Jahida Gulshan Madhabi Baidya Rafia Rashid Farhana Tasneem Ahmed Rashidul Hasan Tanzila Farhana Syed Shafi Ahmed Outcome of Wilson’s disease in Bangladeshi children: a tertiary center experience Egyptian Liver Journal Bangladesh Child Wilson’s disease Hepatic presentation Neurological presentation D-Penicillamine |
| title | Outcome of Wilson’s disease in Bangladeshi children: a tertiary center experience |
| title_full | Outcome of Wilson’s disease in Bangladeshi children: a tertiary center experience |
| title_fullStr | Outcome of Wilson’s disease in Bangladeshi children: a tertiary center experience |
| title_full_unstemmed | Outcome of Wilson’s disease in Bangladeshi children: a tertiary center experience |
| title_short | Outcome of Wilson’s disease in Bangladeshi children: a tertiary center experience |
| title_sort | outcome of wilson s disease in bangladeshi children a tertiary center experience |
| topic | Bangladesh Child Wilson’s disease Hepatic presentation Neurological presentation D-Penicillamine |
| url | https://doi.org/10.1186/s43066-022-00228-6 |
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