Value of Serum Zinc in Diagnosing and Assessing Severity of Liver Disease in Children With Wilson Disease

Sintusek, Palittiya; Kyrana, Eirini; Dhawan, Anil

doi:10.1097/mpg.0000000000002007

Cited by 12 publications

(8 citation statements)

References 43 publications

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“…A broadly modified version of the Leipzig score has recently been developed as a diagnostic scoring system specifically for children. [246] Its rationale is the need to avoid delayed diagnosis when there are difficulties getting ATP7B genotyping and/or liver parenchymal copper measurement. Histochemical assessment for hepatic parenchymal and a "triad" of biochemical parameters are substituted.…”

Section: Problemaɵcsee Tablementioning

confidence: 99%

A multidisciplinary approach to the diagnosis and management of Wilson disease: Executive summary of the 2022 Practice Guidance on Wilson disease from the American Association for the Study of Liver Diseases

et al. 2022

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This is the complete version of the American Association for the Study of Liver Diseases (AASLD) 2022 Guidance on Wilson disease (WD). It provides a contemporary approach to diagnosis and management of WD. Guidance documents, developed by a panel of experts, are formulated as recommendations to help clinicians implement the most current strategies for diagnosis and management. This Guidance was developed with the support and oversight of the AASLD Practice Guidelines Committee. The AASLD Practice Guidelines Committee chose to commission a guidance, rather than a guideline, because of the paucity of randomized controlled trials on this topic. AASLD Guidelines are supported by systematic reviews of the literature, formal ratings of evidence quality and strength of recommendations, and, if appropriate, meta-analysis of results using the Grading of Recommendations Assessment Development and Evaluation system. In contrast, this narrative review and its guidance statements are based on (1) formal review and analysis of the recently published international literature on WD, (2) guideline policies of the AASLD, and (3) the authors' experience. This practice Guidance is intended for use by physicians and other health professionals. As clinically appropriate, guidance statements should be tailored to the needs of individual patients. Specific guidance statements are evidence based whenever possible. Where such evidence is unavailable or inconsistent, guidance statements are based on expert consensus opinion. An executive summary providing a convenient overview is available (

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Section: Problemaɵcsee Tablementioning

confidence: 99%

A multidisciplinary approach to the diagnosis and management of Wilson disease: Executive summary of the 2022 Practice Guidance on Wilson disease from the American Association for the Study of Liver Diseases

et al. 2022

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“…Moreover, serum zinc is low in WD-induced ALF, thereby it appearing to be a novel parameter for diagnosis correlated with WD severity. 64 Likewise, a ratio of alkaline phosphatase to total bilirubin <4 and an AST to ALT ratio > 2.2 are regarded as highly sensitive and specific predictors of fulminant WD. 65 WD along with hepatitis B and AIH consist of the 3 exclusions, wherein an underlying preexisting hepatic condition should be ruled out before ALF is diagnosed.…”

Section: Clinical Presentation At Emergency Roommentioning

confidence: 99%

Acute Liver Failure

Doulberis¹,

Kotronis

Gialamprinou

et al. 2019

Journal of Clinical Gastroenterology

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Acute liver failure is a rare hepatic emergent situation that affects primarily young people and has often a catastrophic or even fatal outcome. Definition of acute liver failure has not reached a universal consensus and the interval between the appearance of jaundice and hepatic encephalopathy for the establishment of the acute failure is a matter of debate. Among the wide variety of causes, acetaminophen intoxication in western societies and viral hepatitis in the developing countries rank at the top of the etiology list. Identification of the clinical appearance and initial management for the stabilization of the patient are of vital significance. Further advanced therapies, that require intensive care unit, should be offered. The hallmark of treatment for selected patients can be orthotopic liver transplantation. Apart from well-established treatments, novel therapies like hepatocyte or stem cell transplantation, additional new therapeutic strategies targeting acetaminophen intoxication and/or hepatic encephalopathy are mainly experimental, and some of them do not belong, yet, to clinical practice. For clinicians, it is substantial to have the alertness to timely identify the patient and transfer them to a specialized center, where more treatment opportunities are available. FIGURE 1. The 3 main well-established classifications for ALF. (i) Williams-Schalm and O'Grady system (ii) Bernuau system, and (iii) Mochida (Japanese) system. ALF indicates acute liver failure.

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“…Most of the study showed low serum Zinc level in Wilson disease children. A study conducted by Palittiya Sintusek et al found serum Zn level was significantly lower in WD with acute liver failure patients compared to those with WD-non-ALF and ALF from indeterminate causes even when corrected serum Zn for low albumin & regardless of presentation, Zn levels showed a negative correlation with the disease severity [7].…”

Section: Introductionmentioning

confidence: 97%

Assessment of Serum Zinc Level in Relation to Different Presentation of Wilson Disease in a Tertiary Care Hospital

Alam

Haq

Begum³

et al. 2023

J. Bangladesh Coll. Phys.

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Background:Wilson disease (WD) is an autosomal recessive disorder of copper metabolism with diverse clinical manifestations. Zinc (Zn) has been used for treatment of WD. Recent studies showed low serum zinc level in patients suffering from WD than the normal. Objective: This cross sectional observational study has been designed to compare the serum Zinc level in children suffering from different presentation of WD before started treatment. Methods: This work was carried out at the department of Pediatric Gastroenterology and nutrition, BSMMU, Dhaka between July’ 2018 to June2019. Total 27 children diagnosed as WD disease, aged between three to eighteen years were included in this study. The patients of WD were divided into four groups according to their presentation as acute hepatitis, decompensated liver disease, acute liver failure and Wilson disease with neurological manifestation. Informed written consent were obtained from all patients for participation in this study. Along with other physical findings and laboratory investigations 3 ml of venous blood were collected for estimation of serum Zinc level. After estimation of serum Zinc level results were analyzed statistically. The difference in serum zinc levels were compared between the groups. Results: Serum Zinc level was founded low in all Wilson disease patients (43.8 ±19.7 [13-83] µg /dl) compared to normal value (64-124µgm. /dl). Among the patients, Serum Zinc level was significantly lower in those who presented as CLD (18 cases, 38.4±17.4µg/dl) and acute liver failure (4 cases, 33.1±3.7µg/dl) compared to those presented as acute hepatitis (4cases, 71.8±4.3 µg/dl) and p value was 0.001 and <0.001 respectively. Mean serum Zinc level was low in 4 patients suffering from acute liver failure (33.1±3.7µg/dl) but was not significant compared to those (23) who presented as Wilson disease non-ALF (45.7±20.8 µg/dl) (P= 0.013) and as Wilson disease CLD (38.4±17.4µg/dl) (p=.25). Conclusion: Serum Zinc level was lowered in patients of Wilson disease. Serum level of zinc was significantly low in patients presented as CLD and acute liver failure in comparison to patients with acute hepatitis. J Bangladesh Coll Phys Surg 2023; 41: 126-131

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Value of Serum Zinc in Diagnosing and Assessing Severity of Liver Disease in Children With Wilson Disease

Abstract: Serum Zn is a novel parameter for diagnosis and correlates with severity of WD. The Proposed WD diagnostic score is useful while awaiting ATP7B mutation analysis.

Cited by 12 publications

References 43 publications

A multidisciplinary approach to the diagnosis and management of Wilson disease: Executive summary of the 2022 Practice Guidance on Wilson disease from the American Association for the Study of Liver Diseases

A multidisciplinary approach to the diagnosis and management of Wilson disease: Executive summary of the 2022 Practice Guidance on Wilson disease from the American Association for the Study of Liver Diseases

Acute Liver Failure

Assessment of Serum Zinc Level in Relation to Different Presentation of Wilson Disease in a Tertiary Care Hospital

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