Marked cardiomegaly in a patient with familial amyloidotic polyneuropathy after orthotopic liver transplantation: a case study.
Pathol Int
; 63(5): 260-5, 2013 May.
Article
en En
| MEDLINE
| ID: mdl-23714253
ABSTRACT
Hepatocyte-derived mutant amyloidogenic transthyretin (ATTR) causes familial amyloidotic polyneuropathy (FAP), for which orthotopic liver transplantation is an established curative treatment. However, some patients with FAP have cardiac amyloidosis after transplantation. Here, we describe a man with an autonomic disorder diagnosed as FAP ATTR Val30Met and marked cardiomegaly after liver transplantation. He underwent orthotopic liver transplantation at 49 years of age and was prescribed prednisolone to prevent graft rejection. Two years later, autonomic dysfunction and severe heart failure gradually developed. He died suddenly at 59. The autopsy revealed marked cardiomegaly (heart weight 1020 g). Histological and ultrastructural examinations demonstrated massive amyloid deposition and unusual myocardial hypertrophic injury associated with nuclear translocation of the glucocorticoid receptor (GR). No other FAP patients without heart failure showed GR nuclear translocation. GR is a nuclear transcription factor that leads to myocardial hypertrophy, and cumulative prednisolone doses may promote marked cardiomegaly and severe cardiac amyloidosis.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Contexto en salud:
1_ASSA2030
/
2_ODS3
/
6_ODS3_enfermedades_notrasmisibles
Problema de salud:
1_doencas_nao_transmissiveis
/
2_muertes_prematuras_enfermedades_notrasmisibles
/
6_cardiovascular_diseases
/
6_endocrine_disorders
/
6_other_circulatory_diseases
Asunto principal:
Polineuropatías
/
Trasplante de Hígado
/
Cardiomegalia
/
Neuropatías Amiloides Familiares
/
Amiloidosis
Límite:
Humans
/
Male
/
Middle aged
Idioma:
En
Revista:
Pathol Int
Asunto de la revista:
PATOLOGIA
Año:
2013
Tipo del documento:
Article
País de afiliación:
Japón