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Hereditary Hemorrhagic Telangiectasia with Unusual Associations

We describe a report of an elderly lady who was hospitalized with progressive worsening of breathlessness and fatigue of one month's duration. Clinical evaluation of the patient revealed hereditary hemorrhagic telangiectasia, interstitial lung disease, pulmonary hypertension without left heart...

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Detalles Bibliográficos
Autores principales: Jain, Dheeraj, Viswanathan, Stalin, Ramasamy, Chandramohan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4494561/
https://www.ncbi.nlm.nih.gov/pubmed/26180702
http://dx.doi.org/10.7759/cureus.278
Descripción
Sumario:We describe a report of an elderly lady who was hospitalized with progressive worsening of breathlessness and fatigue of one month's duration. Clinical evaluation of the patient revealed hereditary hemorrhagic telangiectasia, interstitial lung disease, pulmonary hypertension without left heart failure, and bilateral gluteal calcinosis cutis. Initially, CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome was considered in view of the telangiectasia and calcinosis cutis, but a strong autosomal inheritance pattern and endoscopies (nasal and upper gastrointestinal) favored a diagnosis of hereditary hemorrhagic telangiectasia with rare associations.