Treatment And Prevention Of Acute Porphyria Attack | Pediatric Oncall
PORPHYRIA
Share
Last Updated : 10/1/2014
Ira Shah
More..
Treatment of acute attack
Treatment consists of both supportive and specific therapy. Any patient in an acute attack requires symptomatic treatment for the following conditions:

Pain: When mild, it can be controlled with aspirin, paracetamol or dihydrocodeine. For severe pain, pethidine or morphine may be required. Sleep diminishes pain, hence chlorpromazine with analgesics may be useful. Patients should be left undisturbed in a darkened room.

Nausea, vomiting and constipation: They can be controlled with chlorpromazine, prochlorperazine. If constipation is severe enough to cause obstipation, neostigmine may be useful.

Tachycardia & Hypertension: They are due to sympathetic overactivity and can be controlled by propranolol. If there is evidence of cardiovascular instability, continuous cardiac monitoring is required.

Convulsions: Hyponatremia should be corrected by restricting fluid intake. It could also be a sign of hypertensive encephalopathy and blood pressure should be corrected. Convulsions are usually present only during an attack and remit as the symptoms resolve.

Peripheral neuropathy: Watch should be kept for respiratory fatigue. The expiratory peak flow rate should be monitored. If there is any decrease in this rate, the blood gases should be checked and patient should be in ICU with facilities for assisted ventilation. Proper physiotherapy should be maintained in a paralyzed patient.

Specific therapy consists of:

Hematin therapy: Hematin is the end product of the heme pathway. Thus, it acts by supplementing the depleted heme pool and repressing the activity of the initial enzymes of heme biosynthesis, thus reducing the overproduction of porphyrins. In the acute attack it is given intravenously. There are no major side effects except for phlebitis around the injection site. This can be prevented by giving hematin with human albumin solution to which it will bind. It may also cause prolongation of prothrombin and partial thromboplastin times, which revert back to normal on stopping the drug. In patients with renal failure, the dose of drugs should be reduced.

High Carbohydrate intake: Most patients have nausea and vomiting in the attack and their poor carbohydrate intake further aggravates the disease. Hence, glucose should be supplemented either orally or intravenously.

Prevention of an acute attack
By maintaining a regular diet, avoiding alcohol and certain drugs, most of the attacks can be prevented. For patients who have attacks premenstrually, prophylactic LHRH analogues can be tried long term. Patients who have acute attacks, should avoid pregnancy till they are free of symptomatic attacks for at least 18 months as pregnancy may precipitation an acute attack.

To avoid the photosensitivity seen in VP and HCP, Beta-Carotene treatment may be useful.

Prevention of skin problems
The best way to treat the skin condition is to prevent it. Avoiding sunlight is the most beneficial therapy. Sun-exposed areas can be protected by wearing long sleeves, gloves and a hat. Patient should go out only in early morning or late afternoon. Sunscreens, which can help, are opaque zinc or titanium oxide, however, they are thick and greasy.

Non-acute porphyria
There is no cure for porphyria. However, it can be made less severe. Patients with PCT should avoid alcohol. If skin conditions remain bad, venesection is indicated. This removes about 10cc\kg\body weight of patient's blood at regular intervals usually fortnightly for about eight weeks. Patients with EPP should be regularly tested for liver dysfunction.



Contributor Information and Disclosures

Ira Shah
Consultant Pediatrician, B.J.Wadia Hospital for Children, Mumbai, India


First Created : 2/28/2001
Last Updated : 10/1/2014

References

Advertisements by :    Mega-CV by Aristo IndoIV by Sun Pharma
Disclaimer: The information given by www.pediatriconcall.com is provided by medical and paramedical & Health providers voluntarily for display & is meant only for informational purpose. The site does not guarantee the accuracy or authenticity of the information. Use of any information is solely at the user's own risk. The appearance of advertisement or product information in the various section in the website does not constitute an endorsement or approval by Pediatric Oncall of the quality or value of the said product or of claims made by its manufacturer.
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.