Wilson′s disease: A study of 21 cases from north-west India

Panagariya, A.; Sureka, R. K.; Sharma, Anjani Kumar; Dev, Amit; Agarwal, Neeraj

doi:10.4103/0972-2327.37819

Cited by 15 publications

(27 citation statements)

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“…Panagariya A et al, had observed the mean duration to be 25.2 months with male preponderance (67%). 7 Hepatic and CNS involvement were seen in 73.3% and 26.7% respectively similar to Stephania et al study. 8 Presence of KF ring was seen in 53.3%, more with liver involvement (68.7%).…”

Section: Discussionsupporting

confidence: 79%

Spectrum of cutaneous manifestations in Wilson’s disease in children in a tertiary care center in South India: a prospective study

Karrunya¹,

Bavanandam²,

Dheivamani³

et al. 2017

Int J Contemp Pediatr

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Background: Wilson’s disease in children is the most common inherited disorder of copper metabolism in India with varied clinical manifestations secondary to deposition of copper in various organs including skin. Methods: The aim of the study was to study various cutaneous manifestations of Wilson’s disease in south Indian children. A prospective study for a period of one year was conducted at Dermatology and Pediatric Gastroenterology clinic in Government Rajiv Gandhi Hospital, Chennai. Clinical, demographic details, various cutaneous manifestations and results of skin biopsy were analysed. Results: Out of the total 30 children with Wilson’s disease, 93.3% had at least one cutaneous, mucosal, hair or nail findings but not statistically significant between naive and on treatment group. Cutaneous findings, mucosal involvement, hair changes and nail findings were observed in 90%, 36.7%, 46.7% and 60% respectively. Cutaneous findings were more common in liver disease (P value<0.05). Xerosis was the most common cutaneous manifestation seen in 63.3% patients, followed by pigmentation in 36.7% and infection in 30%. KF ring was noted in 53.3%. Icterus and pruritus were seen in one fifth of study children and majority had portal hypertension. Vascular changes like spider nevi, purpura and other features of portal hypertension were noted in 16.6% of study population. Trichomegaly was one of the common hair manifestation found in 9 (30% especially in children already on treatment (p value <0.05). There was no evidence of stainable copper on skin biopsy. Conclusions: The physicians should be aware of various cutaneous manifestations of Wilson’s disease in children.

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Section: Discussionsupporting

confidence: 79%

Spectrum of cutaneous manifestations in Wilson’s disease in children in a tertiary care center in South India: a prospective study

Karrunya¹,

Bavanandam²,

Dheivamani³

et al. 2017

Int J Contemp Pediatr

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“…Additionally subthalamic nuclei, globus pallidus externa are also capable of inducing tremor [27]. However recent report based on MRI has also confirmed implication of basal ganglia in inducing tremor [28]. In our series, the characteristic "wing beating" tremor of WD was present in only about 9.0% of patients as compared to 33% in other study [10]).…”

Section: Tremorsupporting

confidence: 48%

“…In the other series from India [16], the frequency of ataxia was found to be similarly low. Few series have noted ataxia to be in the range of 28-58% [19,23,28,29]. One of the causes of the smaller number of patients with ataxia may be the large number of juvenile patients in our series as ataxia is usually found in older patients.…”

Section: Ataxiamentioning

confidence: 70%

Spectrum of Abnormal Movement in a Cohort of Neuro-Wilson Patients with Radiological Associations

Bagchi¹,

Paul²

2016

J Neurol Disord

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“…8,9 In a case series from Brazil, the authors found skin hyperpigmentation in 4 out of 36 cases (11.1%). 10 In another study done in Taiwan,the authors had reported hyperpigmentation of skin in 12 out of 20 patients (60%).…”

Section: Discussionmentioning

confidence: 99%

Generalized Hyperpigmentation of Skin as an Atypical Presenting Feature in Wilson Disease

Amaan

Rukunuzzaman

Zahan³

et al. 2018

Bangladesh J Child Health

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Wilson′s disease: A study of 21 cases from north-west India

Cited by 15 publications

References 0 publications

Spectrum of cutaneous manifestations in Wilson’s disease in children in a tertiary care center in South India: a prospective study

Spectrum of cutaneous manifestations in Wilson’s disease in children in a tertiary care center in South India: a prospective study

Spectrum of Abnormal Movement in a Cohort of Neuro-Wilson Patients with Radiological Associations

Generalized Hyperpigmentation of Skin as an Atypical Presenting Feature in Wilson Disease

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