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Porphyria can have nervous and cutaneous manifestations

What is porphyria?

Group of disorders characterized by nervous or dermatological manifestations is known as porphyria disease. This happens due to abnormal metabolism of heme which leads to build up of heme precursors in the body. The cause is inherited gene defects. Porphyria herbal product by natural herbs clinic is recommended for its treatment.

Porphyria symptoms:

Porphyria symptoms can be divided into two types, nervous symptoms which appear in case of acute disease includes chest pain, abdominal pain, often severe, increased heart rate and blood pressure. Limb and back pain, muscle weakness, tingling, loss of sensation, cramping, vomiting and constipation, personality changes or mental disorders, agitation, confusion, and seizures. Hypothermia can also be observed during the acute episode which can last from days to weeks. Cutaneous form of the disease effect skin of the forearms, hands, face, ears and neck on which symptoms like blisters, itching, pain , swelling, increased hair growth and hyper pigmentation along with thickening of skin can occur.

What is Acute Intermittent Porphyria?

Acute Intermittent Porphyria condition is associate deficiency of the enzyme hydroxymethylbilane synthase (also called porphobilinogen deaminase).

What is porphyria cutanea tarda?

Porphyria cutanea tarda (PCT) is the most familiar kind of porphyria.

Porphyria cause:

experts at natural herbs clinic observed that all kinds of porphyrias emerge due to defect in heme metabolism. It occurs due to inherited gene defect which is autosomal dominant in nature but can also be autosomal recessive. Porphyria cutanea tarda is a kind of porphyria which is acquired. The triggers of the acute attack can be several this includes alcohol consumption, excessive iron intake, infections such as hepatitis C, fasting, smoking stress and sun exposure.

Porphyria diagnosis:

porphyria diagnosis is done on the basis of blood stool and urine analysis which will show elevated levels of porphoblinogen or its derivatives.

Porphyria differential diagnosis:

porphyria need to be differentiated from acute lymphoblastic leukemia, acute myelocytic leukemia, chronic and acute anemias, generalized anxity disorder, panic disorder, diabetic ketoacidosis, dubin-jhonson syndrome, hepatitis B and C, Hodgkin disease, hydrocarbon inhalation injury, myocardial infarction in childhood, pheochromocytoma and tyrosinemia.

Porphyria complication:

porphyria complication includes chronic renal insufficiency, repeated acute attacks increases the chances of neuropsychiatric symptoms. Experts at natural herbs clinic observed that chances of hepatocellular carcinoma also increase. In around 8% of the women pregnancy can precipitate an acute attack after which termination of pregnancy becomes necessary. Permanent skin damage can also occur in case of cutaneous disease.

Porphyria prognosis:

porphyria can be considered a lifelong disease. Some types effects the quality of life more immensely then others. Proper treatment and avoiding the triggers can increase the time span between the attacks.

Porphyria treatment:

porphyria treatment can be done by hematin, analgesics, propranolol, beta carotene supplements, and chloroquine but the best treatment presently available in the market is porphyria herbal product by natural herbs clinic.

Porphyria prevention:

the development of the symptoms can be delayed by taking some steps this include avoiding the medications which trigger the attack, avoid alcohol, eat healthy and don’t go for severe caloric restrictions, avoid smoking, minimize sun exposure as much as possible, timely treatment of infections and reduction of stress. Genetic testing can confirm if an individual have a potential of developing this condition.

Causes of Porphyria, Symptoms, Diagnosis, Treatment of Porphyria

Porphyria, a rare disorder involving certain enzymes in the body required for the production of heme pigment and porphyrins. It may be inherited or acquired. It is derived from a Greek word which means ‘purple pigment’ owing to discoloration of urine and feces when exposed to light during the attack. Two type of porphyria disease are present, which are cutaneous and acute. Cutaneous is associated with the skin involvement while acute produces neurological complications. These porphyrias may sometimes be present together simultaneously in the same person.

Porphyria cutanea tarda:

Porphyria cutanea tarda (PCT) is a term encompassing a collection of acquired and familial disorders in which movement of the heme synthetic enzyme uroporphyrinogen decarboxylase (UROD) is deficient.

Acute intermittent porphyria:

Acute intermittent porphyria (AIP) is an uncommon autosomal dominant [1] metabolic disease affecting the creation of heme, the oxygen-binding prosthetic collection of hemoglobin.

CAUSES OF PORPHYRIA:

Porphyrias of at least eight different types exist owing to defect in different enzymes of the body responsible for heme production. Heme is an important iron containing pigment present in erythrocytes responsible for the transport and delivery of oxygen to different organs and tissues. Series of eight different steps involving different enzyme in each step, are responsible for normal production of heme in liver and bone marrow. Defect or deficiency of these enzymes disrupts the process leading to abnormal accumulation of large amount of porphyrins in the body. Porphyria Causes include inherited or acquired mutation in one or more genes. Most common pattern is transmission of faulty gene from parents to offspring. Acquired mutations result from a number of environmental factors. These include alcohol abuse, dieting and fasting, cigarette smoking and drugs such as sedatives, birth controls, transquilizers and barbiturates. Long term exposure to sunlight, hormones, chemicals, stress, excessive dietary iron intake and infections with HBV also trigger the mutations.

PORPHYRIA SYMPTOMS:

Porphyria symptoms depend upon its type. Erythropoietic or cutaneous porphyria is the type which is associated with skin disorders especially when exposed to sun. Skin may swell, blisters form, photodermitis, darkening and thickening of skin along with increased hair growth on forehead is seen. In acute or hepatic type of porphyria, neurological symptoms result. The onset is abrupt and last for several days or week. Due to faulty electrolyte balance, signs and symptoms of increased blood pressure, heart rate, severe abdominal pain, back, limb and chest pain along with tingling, vomiting, numbness, seizures, psychosis, agitation and confusion are found.  Urine on exposure to the sun, changes color to dark brown or dark red owing to the presence and oxidation of abnormal constituents (heme precursors) present in it in different forms of porphyria.

PORPHYRIA DIAGNOSIS:

Porphyria diagnosis is based upon the biochemical findings and analysis of patient’s urine, stool and blood. Porphobilinogen level in urine is elevated in case of acute porphyria.

PORPHYRIA TREATMENT:

In case of acute fatal attack, patient should be hospitalized immediately and treatment should be initiated. No complete cure is present but symptomatic management of the condition prevents the disease progression. Analgesics and anti-emetics are prescribed for pain and vomiting. In porphyria treatment, patient is advised to eat carbohydrate enriched diet. In acute type, treatment with heme arginate and hematin is prescribed. For long term cimetidine is prescribed as prophylactic. Porphyria herbal product made up of rich herbs and plants cure the condition proficiently without any side effects which are normally present with allopathic medications. Herbal mixture improves the condition within a short period of time by curing all the symptoms of the disease.