Acute Porphyria And Acute Attack | Pediatric Oncall
PORPHYRIA
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Last Updated : 10/1/2014
Ira Shah
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Acute porphyria
Acute porphyrias vary in their clinical presentation. During an attack the affected patient may suffer from abdominal pain, cramps, constipation, nausea or vomiting. In addition they may have altered behavior. Severe cases may have weakness and paralysis due to peripheral neuropathy. There may be autonomic dysfunctions like tachycardia, hypertension, postural hypertension, profuse sweating, pallor and pyrexia. Severe hyponatremia may be present due to SIADH, which may cause convulsions.

In patients with VP and HCP, there may also be additional skin involvement and solar photosensitivity.

How do acute attacks occur?
Most of the patients who inherit the disorder enjoy normal health, However, they are at a risk of developing an attack if exposed to various precipitating factors such as alcohol, infection, dieting and drugs. Pregnancy and oral contraceptives may also precipitate attacks. Some women may experience regular attack, one week premenstrual.

The drugs commonly causing the acute attack are:
- Diuretics like frusemide, hydrochlorothiazide
- Antihypertensives like Alpha Methyl Dopa, Enalapril, Hydralazine,Lisinopril, Nifedipine, Verapamil
- Antihistamines like Dimenhydrinate, Terfenadine.
- Hypnotics like Amylobarbitone, Diazepam, Flurazepam.
- Anticonvulsants like Barbiturates, carbamazepine, ethosuximide, Hydantoins, Phenytoin.
- Antibiotics: Chloramphenicol, Erythromycin, Cloxacillin, Griseofulvin, Pyrazinamide, Sulphonamides.
- Miscellaneous-Oral contraceptives, Sulphonylureas, Ergotamine.

Skin problems in porphyria
Skin problems in porphyria are usually acquired conditions. Patients with VP, HC, CP and PCT have a very sensitive skin, which is easily damaged. Sunlight often causes the skin to become fragile particularly in those areas exposed to light like hands, faces, neck, legs and feet. Their skin develops blisters and sores. Long term, their skin become thin, dark, scarred and often hairy. EPP and CP present at much younger age and have severe symptoms.



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

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