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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.37 no.2 São Paulo June 1979

http://dx.doi.org/10.1590/S0004-282X1979000200005 

Aspectos disautonômicos da porfiria aguda intermitente: a propósito de seis casos

 

Disautonomic aspects of the acute intermittent porphyria: a six cases report

 

 

Charles P. TilberyI; Wilson L. SanvitoII; Edna S. EspíndolaIII

IProfessor-Assistente. Departamento de Medicina da Faculdade de Ciências Médicas da Santa Casa de São Paulo
IIProfessor Pleno. Departamento de Medicina da Faculdade de Ciências Médicas da Santa Casa de São Paulo
IIIResidente de 3º ano. Departamento de Medicina da Faculdade de Ciências Médicas da Santa Casa de São Paulo

 

 


RESUMO

Foram estudados seis casos de porfiria aguda intermitente, sendo enfocados os aspectos disautonômicos apresentados durante a longa permanência hospitalar dos doentes (média de 64 dias). Foram observados taquicardia sinusal e hipertensão arterial (4 casos), parada cardíaca (3 casos) e depressão respiratória (5 casos). Os autores tecem comentários a propósito da fisiopatologia destas alterações e chamam a atenção sobre o prognóstico sombrio da porfiria aguda intermitente.


SUMMARY

The study of six patients with acute intermittent porphyria is reported. Clinical and biochemical studies have been performed during the long hospitalization of the patients. The main disautonomic aspects were the arterial hypertension and the tachycardia in four patients, cardiac arrest in three patients and respiratory arrest in five patients. The cause of this problems is probably the involvment of the autonomic adrenergic system. The authors observed also three patients with hyponatremia probably resulting from inappropriate secretion of antidiuretic hormone. The prognosis of the acute intermittent porphyria is worse if the patients have disautonomic symptoms; three patients died and two had neurological sequalae (motor tetraparesis).


 

 

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REFERENCIAS

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Trabalho da Disciplina de Neurologia do Departamento de Medicina da Faculdade de Ciências Médicas da Santa Casa de São Paulo.
Disciplina de Neurologia, Departamento de Clinica Médica - Faculdade de Ciências Médicas da Santa Casa de São Paulo - Rua Cesdrio Motta Jr. 112 - 01221 São Paulo, SP - Brasil.

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