Coagulopathy in Zellweger spectrum disorders: a role for vitamin K.
J Inherit Metab Dis
; 41(2): 249-255, 2018 03.
Article
em En
| MEDLINE
| ID: mdl-29139025
ABSTRACT
INTRODUCTION:
Zellweger spectrum disorders (ZSDs) are caused by an impairment of peroxisome biogenesis, resulting in multiple metabolic abnormalities. This leads to a range of symptoms, including hepatic dysfunction and coagulopathy. This study evaluated the incidence and severity of coagulopathy and the effect of vitamin K supplementation orally and IV in ZSD.METHODS:
Data were retrospectively retrieved from the medical records of 30 ZSD patients to study coagulopathy and the effect of vitamin K orally on proteins induced by vitamin K absence (PIVKA-II) levels. Five patients from the cohort with a prolonged prothrombin time, low factor VII, and elevated PIVKA-II levels received 10 mg of vitamin K IV. Laboratory results, including thrombin generation, at baseline and 72 h after vitamin K administration were examined.RESULTS:
In the retrospective cohort, four patients (13.3%) experienced intracranial bleedings and 14 (46.7%) reported minor bleeding. No thrombotic events occurred. PIVKA-II levels decreased 38% after start of vitamin K therapy orally. In the five patients with a coagulopathy, despite treatment with oral administration of vitamin K, vitamin K IV caused an additional decrease (23%) of PIVKA-II levels and increased thrombin generation.CONCLUSION:
Bleeding complications frequently occur in ZSD patients due to liver disease and vitamin K deficiency. Vitamin K deficiency is partly corrected by vitamin K supplementation orally, and vitamin K administered IV additionally improves vitamin K status, as shown by further decrease of PIVKA-II and improved thrombin generation.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Vitamina K
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Deficiência de Vitamina K
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Coagulação Sanguínea
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Transtornos da Coagulação Sanguínea
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Síndrome de Zellweger
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Suplementos Nutricionais
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Hemorragia
Tipo de estudo:
Diagnostic_studies
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Incidence_studies
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Observational_studies
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Prognostic_studies
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Risk_factors_studies
País como assunto:
Europa
Idioma:
En
Ano de publicação:
2018
Tipo de documento:
Article