Spectrum of Hepatic Presentation of Wilsons Disease in Children Attending A Tertiary Care Centre of Dhaka City

Bayes, Farhana; Karim, Asm Bazlul; Helaly, Laila; Bayes, Fahmida; Rukunuzzaman,; Ahmed, Sanjida; Alam, Shamsul

doi:10.3329/bjch.v38i2.21142

Cited by 7 publications

(6 citation statements)

References 22 publications

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“…Acute liver failure (ALF) (8-20%) 22,28 is predominantly seen in childhood and adolescents. It is usually associated with Coombs-negative non immune intravascular hemolysis.…”

Section: Acute Liver Failurementioning

confidence: 99%

“…Patients may present with complications of cirrhosis (35%-60%), 22,25,28 including ascites (spontaneous bacterial peritonitis), encephalopathy, and renal failure (including hepatorenal syndrome) or portal hypertension (variceal bleeding). 36 In adults, although neurological manifestations may dominate the underlying liver is usually cirrhotic.…”

Section: Cirrhosismentioning

confidence: 99%

See 1 more Smart Citation

Wilson's Disease: Clinical Practice Guidelines of the Indian National Association for Study of the Liver, the Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition, and the Movement Disorders Society of India

Nagral

Sarma

Matthai

et al. 2019

Journal of Clinical and Experimental Hepatology

103

157

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Clinical practice guidelines for Wilson's disease (WD) have been published by the American Association for the Study of Liver Diseases and European Association for the Study of the Liver in 2008 and 2012, respectively. Their focus was on the hepatic aspects of the disease. Recently, a position paper on pediatric WD was published by the European Society of Pediatric Gastroenterology Hepatology and Nutrition. A need was felt to harmonize guidelines for the hepatic, pediatric, and neurological aspects of the disease and contextualize them to the resource-constrained settings. Therefore, experts from national societies from India representing 3 disciplines, hepatology (Indian National Association for Study of the Liver), pediatric hepatology (Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition), and neurology (Movement Disorders Society of India) got together to evolve fresh guidelines. A literature search on retrospective and prospective studies of WD using MEDLINE (PubMed) was performed. Members voted on each recommendation, using the nominal voting technique. The Grades of Recommendation, Assessment, Development and Evaluation system was used to determine the quality of evidence. Questions related to diagnostic tests, scoring system, and its modification to a version suitable for resource-constrained settings were posed. While ceruloplasmin and 24-h urine copper continue to be important, there is little

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“…Acute liver failure (ALF) (8-20%) 22,28 is predominantly seen in childhood and adolescents. It is usually associated with Coombs-negative non immune intravascular hemolysis.…”

Section: Acute Liver Failurementioning

confidence: 99%

Section: Cirrhosismentioning

confidence: 99%

Wilson's Disease: Clinical Practice Guidelines of the Indian National Association for Study of the Liver, the Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition, and the Movement Disorders Society of India

Nagral

Sarma

Matthai

et al. 2019

Journal of Clinical and Experimental Hepatology

103

157

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“…In 10-20% of individuals, WD manifests as ALF primarily in children and young adults. 103,104 Most individuals presenting as ALF-WD have cirrhosis on explant biopsy. 105,106 ALF with Coomb's negative haemolytic anaemia can help to diagnose WD.…”

Section: Wilson Disease (Wd)mentioning

confidence: 99%

Epidemiology of liver failure in Asia‐Pacific region

Jindal

Sarin

2022

Liver International

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It is important to understand the concept of liver failure because it can manifest in a number of phenotypes. If the onset of liver dysfunction or jaundice is accompanied by encephalopathy within days or weeks, it is called acute liver failure (ALF). 1 If chronic liver disease is rapidly worsened (within 4 weeks) by an acute event such as a hepatitis B or autoimmune flare or excessive alcohol intake, this is called acute-on-chronic liver failure (ACLF). [2][3][4] Early recognition of ACLF before the onset of sepsis and extrahepatic insults (such as renal, circulatory and respiratory failure) is important to help prioritize organ-specific interventions. 5 The syndrome of ACLF is potentially reversible 2 and with early attenuation of acute precipitating event, liver reserve improves, fibrosis regresses and portal pressure decreases. The clinical course and outcomes of ACLF patients are well differentiated from decompensated cirrhosis patients

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“…WD can present as ALF-WD predominantly in childhood or adolescence in 8-20% of patients. 163,164 The presentation starts as acute hepatitis but may rapidly deteriorate with deep jaundice, coagulopathy, ascites, and encephalopathy. Majority of patients presenting with clinical "ALF" have been found to have cirrhosis on explants and therefore are not true ALFs.…”

Section: Wilson Diseasementioning

confidence: 99%

Indian National Association for the Study of the Liver Consensus Statement on Acute Liver Failure (Part 1): Epidemiology, Pathogenesis, Presentation and Prognosis

Anand

Nandi²,

Acharya

et al. 2020

Journal of Clinical and Experimental Hepatology

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Acute liver failure (ALF) is an infrequent, unpredictable, potentially fatal complication of acute liver injury (ALI) consequent to varied etiologies. Etiologies of ALF as reported in the literature have regional differences, which affects the clinical presentation and natural course. In this part of the consensus article designed to reflect the clinical practices in India, disease burden, epidemiology, clinical presentation, monitoring, and prognostication have been discussed. In India, viral hepatitis is the most frequent cause of ALF, with drug-induced hepatitis due to antituberculosis drugs being the second most frequent cause. The clinical presentation of ALF is characterized by jaundice, coagulopathy, and encephalopathy. It is important to differentiate ALF from other causes of liver failure, including acute on chronic liver failure, subacute liver failure, as well as certain tropical infections which can mimic this presentation. The disease often has a fulminant clinical course with high short-term mortality. Death is usually attributable to cerebral complications, infections, and resultant multiorgan failure. Timely liver transplantation (LT) can change the outcome, and hence, it is vital to provide intensive care to patients until LT can be arranged. It is equally important to assess prognosis to select patients who are suitable for LT. Several prognostic scores have been proposed, and their comparisons show that indigenously developed dynamic scores have an edge over scores described from the Western world. Management of ALF will be described in part 2 of this document.

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Spectrum of Hepatic Presentation of Wilsons Disease in Children Attending A Tertiary Care Centre of Dhaka City

Cited by 7 publications

References 22 publications

Wilson's Disease: Clinical Practice Guidelines of the Indian National Association for Study of the Liver, the Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition, and the Movement Disorders Society of India

Wilson's Disease: Clinical Practice Guidelines of the Indian National Association for Study of the Liver, the Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition, and the Movement Disorders Society of India

Epidemiology of liver failure in Asia‐Pacific region

Indian National Association for the Study of the Liver Consensus Statement on Acute Liver Failure (Part 1): Epidemiology, Pathogenesis, Presentation and Prognosis

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Spectrum of Hepatic Presentation of Wilsons Disease in Children Attending A Tertiary Care Centre of Dhaka City

Cited by 7 publications

References 22 publications

Wilson's Disease: Clinical Practice Guidelines of the Indian National Association for Study of the Liver, the Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition, and the Movement Disorders Society of India

Wilson's Disease: Clinical Practice Guidelines of the Indian National Association for Study of the Liver, the Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition, and the Movement Disorders Society of India

Epidemiology of liver failure in Asia‐Pacific region

Indian National Association for the Study of the Liver Consensus Statement on Acute Liver Failure (Part 1): Epidemiology, Pathogenesis, Presentation and Prognosis

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Product

Resources

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Spectrum of Hepatic Presentation of Wilsons Disease in Children Attending A Tertiary Care Centre of Dhaka City