Unexplained Findings of Kayser-Fleischer-Like Rings in a Patient with Cryptogenic Cirrhosis

Jawairia, Mahreema; Subhani, Miral; Siddiqui, Ghulam; Prasad, Apsara; Shahzad, Ghulamullah; Rizvon, Kaleem; Mustacchia, Paul

doi:10.1155/2012/438525

Cited by 5 publications

(4 citation statements)

References 12 publications

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“…Kayser-Fleischer rings, are also found in cases of cryptogenic cirrhosis, without other clinical features of Wilson's disease. 16,17 This exemplifies some of the variations within clinical presentation and demonstrates that the correct clinical diagnosis may be difficult, and can be delayed. On the other hand, the characteristic clinical features associated with Wilson's disease are only a benchmark; alone they are insufficient to make a reliable diagnosis.…”

Section: Wilson's Disease: An Inherited Genetic Disordermentioning

confidence: 88%

Wilson’s Disease: An Inherited, Silent, Copper Intoxication Disease

Merle

Weiskirchen

2016

EMJ Neurol

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Wilson’s disease is a rare, autosomal recessive, genetic, copper overload disease, which evokes multiple motor or neuropsychiatric symptoms and liver disease. It is the consequence of a variety of different mutations affecting the ATP7B gene. This gene encodes for a class IB, P-type, copper-transporting ATPase, which is located in the trans-Golgi network of the liver and brain, and mediates the excretion of excess copper into the bile. When functionally inactive, the excess copper is deposited in the liver, brain, and other tissues. Free copper induces oxidative stress, lipid peroxidation, and lowers the apoptotic threshold of the cell. The symptoms in affected persons can vary widely and usually appear between the ages of 6 years and 20 years, but there are also cases in which the disease manifests in advanced age. In this review, we discuss the considerations in diagnosis, clinical management, and treatment of Wilson’s disease. In addition, we highlight experimental efforts that address the pathogenesis of Wilson’s disease in ATP7B deficient mice, novel analytical techniques that will improve the diagnosis at an early stage of disease onset, and treatment results with copper-chelating agents.

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Section: Wilson's Disease: An Inherited Genetic Disordermentioning

confidence: 88%

Wilson’s Disease: An Inherited, Silent, Copper Intoxication Disease

Merle

Weiskirchen

2016

EMJ Neurol

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“…These conditions include cholestatic liver diseases like PBC, hepatitis A, autoimmune hepatitis, and cryptogenic cirrhosis. 15 , 25 – 27 In all patients with PKF rings, there is either deposition of bile pigments or even copper. In PBC, where the excretion of copper through the biliary tree is blocked, the levels of copper in the liver increase.…”

Section: Discussionmentioning

confidence: 99%

Presence of pseudo-Kayser-Fleischer rings in patients without Wilson disease: a prospective cohort study

Sabhapandit

Sharma²,

Kulkarni³

et al. 2023

Hepatology Communications

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Background and Aims: Kayser-Fleischer (KF) rings are due to copper deposition in Descemet membrane of the cornea in Wilson disease. Pseudo-KF ring (PKF), seen in patients with high bilirubin, is often misinterpreted as KF rings. These are bilirubin deposits in posterior corneal stroma. The aim of the study was to prospectively evaluate the presence of KF and PKF rings in Wilson disease and non-Wilsonian liver disease with serum bilirubin >5 mg/dL. Methods: All patients referred from the hepatology unit with serum bilirubin >5 mg/dL were assessed by slit-lamp examination (SLE) and anterior segment optical coherence tomography at baseline, 3 months, and 6 months for differences in corneal deposits between KF and PKF rings. All other clinical, laboratory, radiological, genetic, and tissue diagnoses by liver biopsy were done as required to confirm the cause of liver disease. Results: Among the 750 patients examined, corneal deposits were present in 13%, KF rings as granular deposits in 31.7%, and PKF as a posterior stromal hue in 68.3% of cases. PKF rings showed regression in 60%, disappearance in 36.6% at 3 months, and in 100% of cases at 6 months. KF ring showed regression in 10.7% and 8.3% until 6 months. Anterior segment optical coherence tomography identified KF ring as a hyperintense line on Descemet membrane in an additional 9.7% of patients compared with a scattered hyperintense hue in PKF rings. Conclusions: The presence of PKF rings in patients with jaundice is not uncommon and should be differentiated from true KF rings. Serial monitoring is essential to look for resolution, and anterior segment optical coherence tomography may be additionally helpful.

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“…[ 2 ] Correlation of increased bilirubin levels with pigmented rings was supported by normal Serum Copper studies in these patients. [ 3 ]…”

Section: Discussionmentioning

confidence: 99%

“…Laboratory investigations showed high excretion of copper in urine but it was quite less than the levels found in symptomatic cases of WD. [ 3 ]…”

Section: Discussionmentioning

confidence: 99%

Kayser-Fleischer-like rings in patients with hepatic disease

Chhikara,

2021

Indian Journal of Ophthalmology

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Purpose: The aim of this work was to study Kayser-Fleischer [KF]-like rings in patients with hepatic or cholestatic liver disease and to find out the relation between serum bilirubin level and the presence of KF like ring in these patients. Methods: In this study, we evaluated patients with hepatic and cholestatic liver diseases with total Serum bilirubin levels >10 mg/dl. These patients were evaluated for the presence or absence of KF like ring. Results: A total of 67 patients with total bilirubin >10 mg/dl were included in the study. Patients were divided into 3 groups based on total S. bilirubin level: Group 1 with S. bilirubin >30 mg/dl, Group 2 with S. bilirubin >20 - <30 mg/dl and Group 3 with S. bilirubin >10 - <20 mg/dl at baseline evaluation. On follow-up they were divided into 3 groups based on the serum bilirubin level. Group 1- >20 mg/dl, Group 2- >10 - <20 mg/dl, Group 3- <10 mg/dl. KF like ring was seen in 98.5% of patients with high total S. bilirubin level. KF like ring disappeared in 87.5% of patients with reduction in the total S. bilirubin level to less than 10 mg/dl. Conclusion: KF like ring was seen in 98.5% of patients with high total S. bilirubin, disappeared in 87.5% of patients with reduction in the total S. bilirubin level to less than 10 mg/dl. There was no significant difference between the Total S. bilirubin levels, age, gender and KF Like Ring.

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Unexplained Findings of Kayser-Fleischer-Like Rings in a Patient with Cryptogenic Cirrhosis

Cited by 5 publications

References 12 publications

Wilson’s Disease: An Inherited, Silent, Copper Intoxication Disease

Wilson’s Disease: An Inherited, Silent, Copper Intoxication Disease

Presence of pseudo-Kayser-Fleischer rings in patients without Wilson disease: a prospective cohort study

Kayser-Fleischer-like rings in patients with hepatic disease

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