Transient left bundle branch block and left ventricular dysfunction in a patient with NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome

The NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome is a rare novel autoinflammatory disorder. Cardiac involvement has not been previously reported. We present a 12-year-old girl with NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome who was diagno...

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Bibliographic Details
Published inCardiology in the young Vol. 29; no. 3; pp. 435 - 438
Main Authors Garrelfs, Mark R., Hoppenreijs, Esther, Tanke, Ronald B.
Format Journal Article
LanguageEnglish
Published Cambridge, UK Cambridge University Press 01.03.2019
Subjects
Abdomen
Arthritis
Brief Report
Cardiac function
Cardiology
Cardiovascular disease
Coronary artery disease
Coronary vessels
Cytokines
Dyskeratosis
Electrocardiography
Electrolytes
Fever
Girls
Heart diseases
Heart failure
Laboratories
Methylprednisolone
Mutation
Patients
Pediatrics
Sepsis
Ventricle
United States > US
Netherlands
NLRP1-associated autoinflammation with arthritis and dyskeratosis
autoinflammation
cardiac dysfunction
left bundle branch block
autoimmune
cardiac involvement
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Summary:The NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome is a rare novel autoinflammatory disorder. Cardiac involvement has not been previously reported. We present a 12-year-old girl with NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome who was diagnosed with severely impaired left ventricular function and complete left bundle branch block during an exacerbation of the disease. Cardiac dysfunction proved to be rapidly reversible after initiation of high-dose methylprednisolone.
Bibliography:ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 23
ISSN:1047-9511
1467-1107
DOI:10.1017/S1047951118002342