European Porphyria Initiative: www.porphyria-europe.com
Acute abdominal pain occurs in about 85-90% of attacks (1), and is neurologic in origin. The pain is usually severe, diffuse, unremitting for hours and poorly localized, but is sometimes colicky. Nausea, vomiting and constipation are common, but diarrhea is sometimes noted.
Pain in the extremities, back, chest or head is also common. Objective sensory loss may be found in up to 40% of cases (1). Peripheral motor neuropathy is an indication of a severe and potentially life-threatening attack. Neuropathy can progress to respiratory failure and bulbar paralysis in hours or days. Sudden death, presumably from cardiac arrhythmia may occur. Bladder paresis may cause dysuria and hesitancy (1).
Tachycardia and systemic hypertension are very common. Fever is absent or mild. Signs of peritoneal irritation, such as tenderness are usually not prominent, but there is often ileus with distension and decreased bowel sounds. However, at times bowel sounds are normal or increased.
Leukocytosis is usually absent or mild. Hyponatremia may reflect inappropriate antidiuretic hormone secretion. Gastrointestinal or renal sodium loss may contribute.