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

Tilbery, Charles P.; Sanvito, Wilson L.; Espíndola, Edna S.

doi:10.1590/S0004-282X1979000200005

Resumos

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.

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).

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

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

Charles P. Tilbery^I; Wilson L. Sanvito^II; Edna S. Espíndola^III

^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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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.

1. ATSMON, A.; BLUM, I. & FISCHL, J. - Treatment of an acute attack of porphyria variegata with propanolol. S. Afr. Med. J. 46:311, 1972.
2. BEATTIE, A. D.; MOORE, M. R.; GOLDBERG, A. & WARD, R. L. - Acute intermittent porphyria: response of tachycardia and hypertension to propanolol. Brit. Med. J. 3:257, 1973.
3. DAROCHA, T. & GREGOR, A. - Acute intermittent porphria in Poland. S. Afr. Med. J. 204, 1971.
4. NOVAICHAT, F.; BABINET, P.; MANKO, E.; COMBES, A.; BOCHEREAU, G. & GOULON, M. - Manifestation neurologiques graves des porphyries. In GOULON, M. et col. - Reanimation et Medecine D'Urgence. L'Expansion, Paris, 1974, pp. 19-34.
5. KERR, G. D. - Acute intermittent porphyria and inappropriate secretion of antidiuretic hormone in pregnancy. Proc. Roy. Soc. Med. 66:763, 1973.
6. KRULIK, M.; RENOUX, M. & DEBRAY, J. - L'Hypersecretion inappropriée d'hormones anti-diurétiques (S.I.A.D.H.): a propos d'un cas observe an cours d'une porphyrie aiguë intermittent tardive de l'adulte. Sem. Hôp. Paris 48:3149, 1972.
7. LUDWIG, G. D. & GOLDBERG, M. - Hyponatremia in acute intermittent porphyria probably resulting from inappropriate secretion of antidiuretic hormone. Ann. N. Y. Acad. Sci. 104:710, 1963.
8. NAEF, R. W.; BERRY, R. G. & SCHEZINGER, N. S. - Neurological aspects of porphyria. Neurology (Minneapolis) 9:313, 1959.
9. NIELSEN, B. & THORN, N. A. - Transient excess urinary excretion of antidiuretic material in acute intermittent porphyria with hyponatremia and hypomagnesemia. Am. J. Med. 38:345, 1965.
10. PERLROTH, M. G.; TSCHUDY, D. P.; MARVER, H. S.; BERARD, C. W.: ZEIGEL, R. F.; RECHCIGLI, M. & COLLINS, A. - Acute intermittent porphyria: new morphological and biochemical findings. Am. J. Med. 41:149, 1966.
11. RIDLEY, A.; HIERONS, R. & CAVANAGH, J. B. - The tachycardia and the neuropathy of porphyria. Lancet 2:708, 1968.
12. STEIN, J. A. & TSCHUDY, D. P. - Acute intermittent porphyria: a clinical and biochemical study of 46 patients. Medicine 49:1,, 1970.
13. TILBERY, C. P.; SANVITO, W. L.; CAMPANA, J. O. & LANCELLOTTI, C. L. P. - Forma respiratória da porfiria aguda intermitente: a propósito de dois casos com tetraparesia. Neurobiologia (Recife) 88:199, 1975.
14. WALDENSTROM, J. & HAEGER-ARONSEN, B. - Different patterns of human porphyria. Brit. Med. J. 15:272, 1963.
15. WALDENSTROM, J. - The porphyrias as errors of metabolism. Am. J. Med. 22:758, 1957.
16. WATSON, C. J. - Hematiun and porphyria. N. Engl. J. Med. 293:605, 1975.
17. WATSON, C. J.; PIERACH, C. A.; BOSSENMAIER, I. & CARDINAL, R. -Postulate deficiency of hepatic heme and repair by porphyries. Proc. Nat. Acad. Sci. 74:2118, 1977.

Datas de Publicação

Publicação nesta coleção
24 Ago 2012
Data do Fascículo
Jun 1979

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. ATSMON, A.; BLUM, I. & FISCHL, J. - Treatment of an acute attack of porphyria variegata with propanolol. S. Afr. Med. J. 46:311, 1972.

[2] 2. BEATTIE, A. D.; MOORE, M. R.; GOLDBERG, A. & WARD, R. L. - Acute intermittent porphyria: response of tachycardia and hypertension to propanolol. Brit. Med. J. 3:257, 1973.

[3] 3. DAROCHA, T. & GREGOR, A. - Acute intermittent porphria in Poland. S. Afr. Med. J. 204, 1971.

[4] 4. NOVAICHAT, F.; BABINET, P.; MANKO, E.; COMBES, A.; BOCHEREAU, G. & GOULON, M. - Manifestation neurologiques graves des porphyries. In GOULON, M. et col. - Reanimation et Medecine D'Urgence. L'Expansion, Paris, 1974, pp. 19-34.

[5] 5. KERR, G. D. - Acute intermittent porphyria and inappropriate secretion of antidiuretic hormone in pregnancy. Proc. Roy. Soc. Med. 66:763, 1973.

[6] 6. KRULIK, M.; RENOUX, M. & DEBRAY, J. - L'Hypersecretion inappropriée d'hormones anti-diurétiques (S.I.A.D.H.): a propos d'un cas observe an cours d'une porphyrie aiguë intermittent tardive de l'adulte. Sem. Hôp. Paris 48:3149, 1972.

[7] 7. LUDWIG, G. D. & GOLDBERG, M. - Hyponatremia in acute intermittent porphyria probably resulting from inappropriate secretion of antidiuretic hormone. Ann. N. Y. Acad. Sci. 104:710, 1963.

[8] 8. NAEF, R. W.; BERRY, R. G. & SCHEZINGER, N. S. - Neurological aspects of porphyria. Neurology (Minneapolis) 9:313, 1959.

[9] 9. NIELSEN, B. & THORN, N. A. - Transient excess urinary excretion of antidiuretic material in acute intermittent porphyria with hyponatremia and hypomagnesemia. Am. J. Med. 38:345, 1965.

[10] 10. PERLROTH, M. G.; TSCHUDY, D. P.; MARVER, H. S.; BERARD, C. W.: ZEIGEL, R. F.; RECHCIGLI, M. & COLLINS, A. - Acute intermittent porphyria: new morphological and biochemical findings. Am. J. Med. 41:149, 1966.

[11] 11. RIDLEY, A.; HIERONS, R. & CAVANAGH, J. B. - The tachycardia and the neuropathy of porphyria. Lancet 2:708, 1968.

[12] 12. STEIN, J. A. & TSCHUDY, D. P. - Acute intermittent porphyria: a clinical and biochemical study of 46 patients. Medicine 49:1,, 1970.

[13] 13. TILBERY, C. P.; SANVITO, W. L.; CAMPANA, J. O. & LANCELLOTTI, C. L. P. - Forma respiratória da porfiria aguda intermitente: a propósito de dois casos com tetraparesia. Neurobiologia (Recife) 88:199, 1975.

[14] 14. WALDENSTROM, J. & HAEGER-ARONSEN, B. - Different patterns of human porphyria. Brit. Med. J. 15:272, 1963.

[15] 15. WALDENSTROM, J. - The porphyrias as errors of metabolism. Am. J. Med. 22:758, 1957.

[16] 16. WATSON, C. J. - Hematiun and porphyria. N. Engl. J. Med. 293:605, 1975.

[17] 17. WATSON, C. J.; PIERACH, C. A.; BOSSENMAIER, I. & CARDINAL, R. -Postulate deficiency of hepatic heme and repair by porphyries. Proc. Nat. Acad. Sci. 74:2118, 1977.