PORFIRIAS

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	PORFIRIAS
	*PORFIRIAS*

PORFIRIAS

Porfirias

Group of rare hereditary diseases that the excessive formation and excreción of porfirinas are characterized by (present chemical substances in all alive thing).

Affected parts

Often these affections are attributed erroneously to emotional disorders. According to the type, they are affected

the central nervous system and/or

the skin and/or

the liver and/or

the alimentary canal.

Prognosis

This disease considers incurable, but many patients live with her during years. The symptoms can be alleviated or be controlled.

They continue the investigations to obtain an effective treatment.

Complications

A mortal attack of porfiria can appear during the pregnancy.

The medical attention is necessary before and after the childbirth.

The mistaken diagnosis as psychological or emotional problem can delay to the diagnosis and the suitable treatment.

Addiction to narcotics.

Aesthetic deformations.

Diagnosis and treatment

Diagnosis

Consultation and physical examination by a doctor.

Laboratory tests to measure the porfirinas in the blood, tinkle and lees.

Treatment

General measures

Protéjase of intense the solar light.

Medication

It does not take any medication until consulting with its doctor.

It can prescribe to him:

tranquilizers to reduce the anxiety. The clorpromazina usually is effective.

Analgesic and other yes an attack takes place. Avi him to it if his it tinkles she is dark (according to porfiria in question) or other symptoms of an attack reproduce.

Activity

Without restrictions.

Diet

Carbohydrate discharge.

Prevention

They do not have prevention.

If you are woman and its disease is severe, the pregnancies are not recommendable.

Any person with familiar antecedents of porfiria must look for genetic advising before beginning a family.

Perhaps in the future it is possible to be resolved by means of genetic engineering to replace the affected enzyme.

In order to reduce to the frequency and severity of the attacks:

Avoid all drugs, including medicines without prescription until consulting its doctor.

It does not take contraceptive pills.

Avoid intense the solar light.

Factors of risk

Familiar antecedents of porfiria.

The drug use like contraceptive pills; alcohol, barbiturates, do not cause the disease but it can cause attacks.

Signs and symptoms

Signs

Variables, since according to the passage of the metabolism, diverse types of porfirinas are accumulated, each one affecting different organs.

In most of the cases attacks take place more alternating with rested periods.

Between that they appear in some, several or all porfirias, we emphasized:

Torácico or abdominal pain.

Mental upheavals like depression or deviation.

Dermatological disorders that include picores and blisters.

Pain in the legs.

Muscular cramps and weakness.

Entumecimiento and creeps in feet and hands.

Causes

Hereditary disorder of the metabolism of the porfirinas.

The porfirinas are products of the metabolism of the hemoglobina, among other corporal proteins, and these diseases cause their accumulation.

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