Descripción de un caso de angioedema hereditario dependiente de estrógenos / A case description of oestrogen-dependent inherited angioedema

Introducción: El angioedema hereditario es una enfermedad genética rara que carece de tratamiento específico. Principales síntomas: Se caracteriza por la aparición recurrente de episodios de edema que afectan fundamentalmente a piel y mucosa. Diagnósticos principales: Existen tres tipos de angioedema hereditario, habiéndose relacionado el tipo III con situaciones que presentan niveles elevados de estrógenos. Intervenciones terapéuticas: Exponemos el caso de una paciente con angioedema hereditario tipo III, que presenta crisis de angioedema coinciciendo con el periodo periovulatorio y premenstrual. Ante dicha relación hormonal, se pautó terapia con gestágenos para intentar reducir el número de ovulaciones. Resultados: Tras varios meses en tratamiento con desogestrel la paciente refiere disminución del número y gravedad de las crisis. Conclusión: La terapia con gestágenos parece resultar útil en el control de los episodios de angioedema hereditario tipo III.(AU)

Introduction: Hereditary angioedema is a rare genetic disease without any specific treatment. Main symptoms: It is characterized by recurrent episodes of skin and mucous oedema. Main diagnoses: There are three types of angioedema and type III has been related to high-level oestrogen conditions. Therapeutic interventions: We describe the case of a patient with hereditary angioedema type III, who had an episode of angioedema associated with the periovulatory and premenstrual period. Due to this hormonal influence, we used gestagen therapy to attempt to reduce the number of ovulations. Results: After several months of treatment with desogestrel, the patient reports a decrease in the number and severity of episodes. Conclusion: Gestagen therapy seems to be useful for controlling episodes of hereditary angioedema type III.(AU)

Effect of angiotensin-converting enzyme inhibitor on renal function in ovarian hyperstimulation syndrome in the rabbit.

OBJECTIVE: To investigate renal function and whether captopril prevents alterations in the handling of sodium and water in the ovarian hyperstimulation syndrome (OHSS) in the rabbit. DESIGN: Experimental study SETTING: Physiology laboratory. ANIMAL(S): Six female New Zealand white rabbits were used as controls, and 13 were hyperstimulated with gonadotropins. INTERVENTION(S): Saline or captopril. MAIN OUTCOME MEASURE(S): Renal excretory and hemodynamic variables. RESULT(S): The 3% extracellular volume expansion in OHSS animals induced a significant elevation in mean arterial pressure by 27%, although increments in natriuresis and diuresis were similar to those observed in controls. The OHSS group had impaired pressure-natriuresis sensitivity compared with controls (0.36 +/- 0.07 microEq/min/g of Na excreted per mm Hg vs. 1.74 +/- 0.45 microEq/min/g of Na excreted per mm Hg; P<.05. Captopril significantly reduced mean arterial pressure (P<.05) and shifted the pressure-natriuresis response to the left by 0.85 +/- 0.17 microEq/min/g of Na excreted per mm Hg (P<.05). CONCLUSION(S): In OHSS in the rabbit model, pressure-natriuresis sensitivity is impaired. Angiotensin II may play a significant role in this phenomenon, since angiotensin-converting enzyme inhibition normalized the pressure-natriuresis relationship.

17beta-estradiol prevents oxidative stress and decreases blood pressure in ovariectomized rats.

In this study, we tested whether estrogen deficiency is associated with oxidative stress and decreased nitric oxide (NO) production, which could be responsible for an increased blood pressure in ovariectomized rats. Hemodynamic studies were performed on conscious, chronically instrumented rats. Chronic estrogen replacement on ovariectomized rats lowered blood pressure approximately 13 mmHg, from 119 +/- 3 mmHg in ovariectomized rats to 106 +/- 3 mmHg in ovariectomized-treated rats; it was also accompanied by an increase in cardiac index and vascular conductance, achieving hemodynamic values similar to those shown by sham-operated rats. N(G)-nitro-L-arginine methyl ester administration lowered significantly less the vascular conductance (0.14 +/- 0.01 vs. 0.22 +/- 0.03 and 0.26 +/- 0.01 ml. min(-1). mmHg(-1)/100 g; P < 0.05) in ovariectomized rats than in the sham-operated and estrogen-treated ovariectomized rats, respectively. Estrogen replacement prevented the lower plasma levels of nitrites/nitrates observed in ovariectomized rats. The lower plasma total antioxidant status and reduced thiol groups and the increase in plasma lipoperoxides presented in ovariectomized animals were reestablished with the estrogen treatment. These results show that estrogen administration decreases blood pressure and increases vascular conductance in ovariectomized rats. This effect may be related to an increase in NO synthesis and/or preventing oxidative stress, then improving endothelial function.