دورية أكاديمية
أعرض في EDS

Clinical profile of adult and pediatric patients with hepatic Wilson's disease.

التفاصيل البيبلوغرافية
العنوان: Clinical profile of adult and pediatric patients with hepatic Wilson's disease.
المؤلفون: Kumar S; Department of Gastroenterology, Seth G S Medical College and K E M Hospital, Gastroenterology Office, 9th Floor, New Building, Parel, Mumbai, 400 012, India., Irtaza M; Department of Gastroenterology, Seth G S Medical College and K E M Hospital, Gastroenterology Office, 9th Floor, New Building, Parel, Mumbai, 400 012, India., Patra BR; Department of Gastroenterology, Seth G S Medical College and K E M Hospital, Gastroenterology Office, 9th Floor, New Building, Parel, Mumbai, 400 012, India., Rao PK; Department of Gastroenterology, Seth G S Medical College and K E M Hospital, Gastroenterology Office, 9th Floor, New Building, Parel, Mumbai, 400 012, India., Gopan A; Department of Gastroenterology, Seth G S Medical College and K E M Hospital, Gastroenterology Office, 9th Floor, New Building, Parel, Mumbai, 400 012, India., Kale AP; Department of Gastroenterology, Seth G S Medical College and K E M Hospital, Gastroenterology Office, 9th Floor, New Building, Parel, Mumbai, 400 012, India., Shukla A; Department of Gastroenterology, Seth G S Medical College and K E M Hospital, Gastroenterology Office, 9th Floor, New Building, Parel, Mumbai, 400 012, India. drakashshukla@yahoo.com.
المصدر: Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology [Indian J Gastroenterol] 2024 Apr; Vol. 43 (2), pp. 425-433. Date of Electronic Publication: 2024 May 10.
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: Springer India Country of Publication: India NLM ID: 8409436 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 0975-0711 (Electronic) Linking ISSN: 02548860 NLM ISO Abbreviation: Indian J Gastroenterol Subsets: MEDLINE
أسماء مطبوعة: Publication: India : Springer India
Original Publication: Bombay : Indian Society of Gastroenterology,
مواضيع طبية MeSH: Hepatolenticular Degeneration*/complications , Hepatolenticular Degeneration*/mortality , Hepatolenticular Degeneration*/diagnosis, Humans ; Male ; Adolescent ; Child ; Female ; Adult ; Child, Preschool ; Young Adult ; Infant ; Prognosis ; Age Factors ; Liver Cirrhosis/complications ; Liver Cirrhosis/etiology ; Liver Cirrhosis/mortality ; Liver/pathology ; Acute-On-Chronic Liver Failure/etiology ; Acute-On-Chronic Liver Failure/mortality ; Acute-On-Chronic Liver Failure/diagnosis
مستخلص: Background: The clinical profile varies in patients with Wilson's disease (WD). There is paucity of data regarding adult and pediatric patients with hepatic WD.
Methods: As many as 140 consecutive patients diagnosed with hepatic WD between December 2006 and January 2021 were included in the study. Data was collected regarding the demographic parameters, clinical presentation, extrahepatic organ involvement, liver histology and laboratory investigations. Adult and children (0-14 years) with hepatic WD were compared regarding these features.
Result: Eighty-eight adults and 52 children were included in the study. The median age of presentation was 17 years (range: 1.1-42 years). Male preponderance was seen (adult 68/88, 69%; children 40/52, 77%). Adults as compared to children presented more commonly as cirrhosis (52/88 vs. 15/52, p = 0.0005) and with hepatic decompensation (35/88 vs. 9/52, p = 0.005). Presentation with acute-on-chronic liver failure (ACLF) was more common in children (10/52 vs. 2/88, p = 0.0005). Twenty-eight-day mortality was 50% (5/10) in children and none in adults presenting with ACLF. Nazer's Prognostic Index (≥ 7) and New Wilson Index were more accurate in predicting mortality among children with ACLF with AUROC 1, while AARC (APASL ACLF Research Consortium) was less accurate with AUROC 0.45. Liver histology findings were similar in adults and children. Extrahepatic involvement was also similar. (8/88 in adults vs. 3/52 children, p value 0.48).
Conclusion: Most patients with WD present as cirrhosis in adulthood. ACLF is more common in children. Nazer's prognostic index and new Wilson Index score are accurate in predicting mortality in children with ACLF.
(© 2024. Indian Society of Gastroenterology.)
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فهرسة مساهمة: Keywords: Clinical profile; Diagnosis; Paediatrics; Prognosis; Wilson disease
تواريخ الأحداث: Date Created: 20240510 Date Completed: 20240526 Latest Revision: 20240527
رمز التحديث: 20240527
DOI: 10.1007/s12664-024-01586-2
PMID: 38730078
قاعدة البيانات: MEDLINE
الوصف
تدمد:0975-0711
DOI:10.1007/s12664-024-01586-2