Doença de Wilson em crianças e adolescentes: diagnóstico e tratamento

Sócio, Stephania de Andrade; Ferreira, Alexandre Rodrígues; Fagundes, Eleonora Druve Tavares; Roquete, Mariza Leitão Valadares; Pimenta, Júlio Rocha; Campos, Lilian de Faria; Penna, Francisco José

doi:10.1590/s0103-05822010000200002

Cited by 12 publications

(7 citation statements)

References 15 publications

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“…Although reports on pediatric WD patients from other countries have been published, only one study has been published on Brazilian children (13). Machado et al (2006) evaluated 119 Brazilian patients, mostly adults, with a mean age at diagnosis of 19.3 [7–37] and reported that the major initial clinical manifestation was neurological (12).…”

Section: Discussionmentioning

confidence: 99%

“…Studies of WD in childhood are limited because the number of patients available to compose a relevant study is frequently low, which precludes large cohort studies or randomized controlled trials. Although reports on pediatric WD patients from other countries have been published, only one study has been published on Brazilian children ( 13 ). Machado et al (2006) evaluated 119 Brazilian patients, mostly adults, with a mean age at diagnosis of 19.3 [7–37] and reported that the major initial clinical manifestation was neurological ( 12 ).…”

Section: Discussionmentioning

confidence: 99%

“…Few reports have evaluated the clinical course of adult Brazilian patients with WD ( 12 ), and only one study preliminarily evaluated pediatric patients ( 13 ). The aim of this study was to analyze the clinical and laboratory evolutions of WD and the effects of the standard treatments in Brazilian children with WD.…”

Section: Introductionmentioning

confidence: 99%

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Wilson's disease: an analysis of 28 Brazilian children

Kleine¹,

Mendes²,

Pugliese³

et al. 2012

Clinics

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OBJECTIVES:Clinical-laboratory and evolutionary analysis of twenty-eight patients with Wilson's disease.METHODS:Twenty-eight children (twelve females and sixteen males) with Wilson's disease were evaluated retrospectively between 1987 and 2009, with a follow-up of 72 months (1 – 240 months). The clinical, laboratory, and histologic features at diagnosis were recorded at the end of the study.RESULTS:The median age at diagnosis was 11 years (2 – 18 years). Twelve patients were asymptomatic, seven had hepatitis symptoms, five had raised aminotransferase levels, three had hepatomegaly associated with neurological disorders, one had fulminant hepatitis with hemolytic anemia, and six patients presented with a Kayser-Fleischer ring. A histological analysis revealed that six children had chronic hepatitis, seven had cirrhosis, two had steatosis, one had portal fibrosis, and one had massive necrosis. The treatment consisted of D-penicillamine associated with pyridoxine for 26 patients. Adverse effects were observed in the other two patients: one presented with uncontrollable vomiting and the other demonstrated elastosis perforans serpiginosa. At the end of the study, all 26 treated patients were asymptomatic. Twenty-four of the patients were treated with D-penicillamine and pyridoxine, and two were treated with trientine and zinc sulfate. A liver transplant was performed in one patient with fulminant hepatitis, but the final patient died 48 hours after admission to the intensive care unit.CONCLUSIONS:Family screenings associated with early treatment are important in preventing Wilson's disease symptoms and potentially fatal disease progression. The study suggests that Wilson's disease must be ruled out in children older than two years presenting with abnormal levels of hepatic enzymes because of the heterogeneity of symptoms and the encouraging treatment results obtained so far.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Wilson's disease: an analysis of 28 Brazilian children

Kleine¹,

Mendes²,

Pugliese³

et al. 2012

Clinics

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“…Like zinc, copper is an essential element for human beings. However, levels above the maximum permissible limits may induce neurological, psychiatric, hematological, or hepatic effects (Sócio et al, 2010). Chromium is an important causative agent of contact dermatitis among workers, and it can also cause chronic skin ulcers and nasal septum perforations due to its corrosive properties.…”

Section: Introductionmentioning

confidence: 99%

Risk of exposure to metals in soil contaminated by steel industry waste for a population in Volta Redonda, RJ

et al. 2021

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The aim of this study was to identify the risk of population’s exposure, by different exposure routes, to Zn, Ni, Cu, Cr, and Pb in the soil of a condominium. Six sampling points in three campaigns provided thirty-six soil samples, collected at two depths, one superficial, 0.20 m, and the other underground, between 2.00 and 3.00 m. The results show that the levels of metals in the condominium's soil are generally high. Children cannot withstand the same doses as adults since they are more susceptible and risk greater damage to health. The non-carcinogenic risk based on the hazard quotient (HQ) and the health index (HI) values for each exposure pathway and for each metal were observed to be less than (<) 1. The results showed an immediate carcinogenic risk by chromium ingestion for children. Nevertheless the combined effect for each exposure pathway (TCRI) for adults is close to becoming unacceptable after exposure to Cr by ingestion. The results suggest that contamination by metals is more likely to occur among children than adults at similar exposure levels. Thus, the condominium built on an area contaminated by steel industry waste raises concern, requiring the continuous monitoring of its population.

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“…A impregnação do metal na córnea, o anel BRASIL, 2013). A literatura aponta uma predominância de manifestações neste órgão na faixa etária pediátrica, como evidenciado por estudo realizado porSócio et al (2010) cujo 65% das apresentações eram predominantemente hepática. O paciente em questão foi de encontro ao descrito, pois este apresentava provas de função hepática completamente normais em mais de uma dosagem.…”

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Neurosarcoidose: Relato De Caso Em Paciente Com Rebaixamento Do Sensório Associado a Neuropatia De Nervo Craniano, Cefaleia E Vasculopatia

Hämmerle¹,

Souza²,

Botelho³

et al. 2020

Frente Diagnóstica E Terapêutica Na Neurologia 2

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Todo o conteúdo deste livro está licenciado sob uma Licença de Atribuição Creative Commons. Atribuição 4.0 Internacional (CC BY 4.0). O conteúdo dos artigos e seus dados em sua forma, correção e confiabilidade são de responsabilidade exclusiva dos autores. Permitido o download da obra e o compartilhamento desde que sejam atribuídos créditos aos autores, mas sem a possibilidade de alterá-la de nenhuma forma ou utilizá-la para fins comerciais.

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Doença de Wilson em crianças e adolescentes: diagnóstico e tratamento

Cited by 12 publications

References 15 publications

Wilson's disease: an analysis of 28 Brazilian children

Wilson's disease: an analysis of 28 Brazilian children

Risk of exposure to metals in soil contaminated by steel industry waste for a population in Volta Redonda, RJ

Neurosarcoidose: Relato De Caso Em Paciente Com Rebaixamento Do Sensório Associado a Neuropatia De Nervo Craniano, Cefaleia E Vasculopatia

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