Why does porphyria patient have abdominal pain?

summary

Hematoporphyrin pathogen is called purpura disease, which is a rare disease. Most of the diseases are caused by the lack of enzymes related to heme synthesis pathway due to genetic defects, resulting in the disorder of porphyrin metabolism. The main clinical manifestations were photosensitive skin damage, abdominal pain, neuropsychiatric symptoms and increased blood pressure. According to the location of porphyrin metabolic disorder, it can be divided into erythropoietic porphyria and hepatic porphyria. So let's take a look at why patients with porphyria have abdominal pain? What about it?

Why does porphyria patient have abdominal pain?

First, acute intermittent porphyria is more common, which is an autosomal dominant genetic disease caused by the deficiency of PBG deaminase (uroporphyrinogen synthetase). This defect causes the decrease of PBG conversion into uroporphyrin III in liver, resulting in the disorder of heme synthesis, which strengthens the function of ALA synthetase, increases the synthesis of ALA and PBG and increases their excretion from urine.

Second: abdominal pain is the most important and prominent symptom. Although the paroxysmal colic can be very mild sometimes, it is mostly serious. The pain can be localized, spread to the whole abdomen, or radiate to the back or waist, and may be accompanied by nausea and vomiting. Often have stubborn constipation. There was no obvious tenderness in the abdomen.

Third: there are many symptoms of the nervous system, such as limb neuralgia, hypoalgesia or numbness. There may be muscle weakness of single limb until flaccid paralysis of limbs, and there may be severe muscle pain before or at the same time of paralysis, especially in the lower leg. Tendon reflexes often decrease or disappear. Abdominal, intercostal or diaphragmatic muscle weakness can lead to respiratory paralysis and life-threatening. Also can appear optic atrophy, ophthalmoplegia, facial paralysis, dysphagia and vocal cord paralysis and other symptoms.

matters needing attention

1. Avoid inducements such as overwork, mental stimulation, hunger and infection, and stop using drugs that may induce disease attack immediately. 2. It is suitable to have a high sugar diet. In case of acute attack of alcohol prohibition, intravenous drip of 10% glucose solution combined with high sugar diet can quickly relieve the symptoms. Patients with impaired glucose tolerance can be treated with insulin. 3. Endocrine therapy for a few cases with acute attack and menstrual cycle, GnRH analogues can be considered to inhibit menstruation. 4. For symptomatic treatment, it is necessary to avoid using drugs that aggravate the disease. 5. Heme is an effective method to rescue critical acute porphyria.