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Type of congenital adrenal hyperplasia with hypertension worry about 4 minutes (content expertise) to edit entries Abstract Congenital adrenal hyperplasia type of hypertensionAlso known as congenital adrenal hyperplasia (congenitaladrenalhy-perplasia) initially with the discovery of virilization of female fetuses was increased awareness of the adrenal. Adrenal was called metamorphosis syndrome. However, the name reflects only the variation can cause the adrenal cortical hyperplasia or tumor. And this disease is not caused by certain types of abnormal sexual differentiation or function, it is now commonly known as congenital adrenal hyperplasia, which refers to the synthesis of adrenal corticosteroids during the congenital absence of an enzyme, interfering with some of the steroids hormone synthesis and secretion of ACTH due to an increase in secondary adrenal hyperplasia caused. An autosomal recessive hereditary metabolic disease. Edit this section | Back to the top of the pathological description of
In the adrenal cortex, cortisol, aldosterone, testosterone biosynthesis requires many enzymes, if due to genetic reasons, the relative or absolute lack of an enzyme, the biosynthesis of the corresponding blocked, the accumulation of the results of the material before the occlusion, blood, urine in concentration, but decreased after blocking the material. If such damage can lead to decreased cortisol synthesis, is reduced because the feedback inhibition of pituitary ACTH se


cretion increases, resulting in adrenal hyperplasia. Complete lack of the enzyme is not fully compensated or not treated early often leads to death. If the enzyme deficiency is not complete, is not life-threatening, the first symptom may occur only after the age of 15, known as late onset. Due to the accumulation of clinical manifestations or lack of body steroids different substances are different. Such as cortisol decrease in performance caused by adrenal insufficiency, androgen can cause an increase in masculine, hairy, primary amenorrhea, mineralocorticoid excess can cause hypertension and hypokalemia. Congenital adrenal hyperplasia often caused by enzyme deficiency due to the following five: 21 - hydroxylase, 11 -hydroxylase, 3 -hydroxysteroid dehydrogenase, 17 -hydroxylase, a simple 18 - and 20,22 carbon chain lyase (Table 15-15). Hydroxylase or 18-hydroxyl dehydrogenase deficiency does not cause adrenal hyperplasia.
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Diagnosis is confirmed to be an early application of hydrogen's loose or prednisone, one can substitute its own lack of glucocorticoid synthesis, also inhibit pituitary release of adrenocorticotropic hormone, thereby inhibiting excessive production of adrenal androgens, stop masculine development. If used properly, patients can maintain normal growth and development, and life. The beginning of a larger dose should be 1 to 2 weeks after the steroids in urine emission to be controlled to a satisfactory level has been, can be reduced to a physiological dose: General 2.5 mg per day oral prednisone infants, children 2.5 to 5 mg, 7.5 adolescence 10 mg dose of steroids under the urinary excretion of ketones 17 and alignment. Orally twice daily volume, the last one to be taken in the evening.
Should insist on lifelong medication. In the stress case, the hormone necessary to increase the amount of times to maintain in case of severe stress conditions or the occurrence of acute adrenal crisis hypothyroidism, the steroid dose or even the need to increase 5 to 10 times, and using water-soluble hydrocortisone and sodium chloride infusion.
Loss of salt-type patients should be timely rescue, beginning with hydrocortisone 25 to 100 mg / day intravenous infusion of fluids to correct dehydration and sodium chloride and salt, and may also apply deoxy corticosterone acetate (DOCA) 0.5 ~ l mg / day intramuscular injection, or sodium retention hormones 90 - hydrofluoric cortisol 250 ~ 300 mg / square meter / day, three times to take. Light loss of salt may oral prednisone per day add 2 to 5 grams of salt that is able to maintain electrolyte balance.
Pseudohermaphroditism clitoris surgery in children after birth should be 2 to 4 years, surgery is not easy success too early, too late for the patient if the psychological and social impact of disadvantage. Urethral and vaginal openings in the urogenital sinus with the patient can do after menstruation urethra, vagina separation surgery in order to avoid ascending urinary tract infection. If the patient was definitely diagnosed in adolescence, its external genitalia are basically like men, are not appropriate to change the original form of the external genitalia, because patients often change sex will be a blow to the psychological and social impact of a complex can be considered for the uterus and ovaries removed, so that continued development of male secondary sexual characteristics and, based 7 urinary excretion of ketone steroids prednisone dosage adjustments to make to maintain the level in normal adult men. Edit entry Open Category:
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