Table 3.

Common symptoms and signs of acute porphyria.

Symptoms and SignsEstimated Incidence, %Comment
Reprinted with permission from
Anderson KE, Bloomer JR, Bonkovsky HL, et al. Recommendations for the diagnosis and treatment of the acute porphyrias.
Ann Intern Med
.
2005
;
142
:
439
–450.
 
Gastrointestinal 
    Abdominal pain 85–95 Usually unremitting (for hours or longer) and poorly localized but can be cramping. Neurologic in origin and rarely accompanied by peritoneal signs, fever, or leukocytosis. 
    Vomiting 43–88 Nausea and vomiting often accompany abdominal pain. 
    Constipation 48–84 May be accompanied by bladder paresis. 
    Diarrhea 5–12  
Neurologic 
    Pain in extremities, back, chest, neck, or head 50–70 Pain may begin in the chest or back and move to the abdomen. Extremity pain indicates involvement of sensory nerves, with objective sensory loss reported in 10%–40% of cases. 
    Paresis 42–68 May occur early or late during a severe attack. Muscle weakness usually begins proximally rather than distally and more often in the upper than lower extremities. 
    Respiratory paralysis 9–20 Preceded by progressive peripheral motor neuropathy and paresis. 
    Mental symptoms 40–58 May range from minor behavioral changes to agitation, confusion, hallucinations, and depression. 
    Convulsions 10–20 A central neurologic manifestation of porphyria or due to hyponatremia, which often results from syndrome of inappropriate antidiuretic hormone secretion or sodium depletion. 
Cardiovascular 
    Tachycardia 64–85 May warrant treatment ot control rate, if symptomatic (see text). 
    Systemic arterial hypertension 36–55 May require treatment during acute attacks, and sometimes becomes chronic. 
Symptoms and SignsEstimated Incidence, %Comment
Reprinted with permission from
Anderson KE, Bloomer JR, Bonkovsky HL, et al. Recommendations for the diagnosis and treatment of the acute porphyrias.
Ann Intern Med
.
2005
;
142
:
439
–450.
 
Gastrointestinal 
    Abdominal pain 85–95 Usually unremitting (for hours or longer) and poorly localized but can be cramping. Neurologic in origin and rarely accompanied by peritoneal signs, fever, or leukocytosis. 
    Vomiting 43–88 Nausea and vomiting often accompany abdominal pain. 
    Constipation 48–84 May be accompanied by bladder paresis. 
    Diarrhea 5–12  
Neurologic 
    Pain in extremities, back, chest, neck, or head 50–70 Pain may begin in the chest or back and move to the abdomen. Extremity pain indicates involvement of sensory nerves, with objective sensory loss reported in 10%–40% of cases. 
    Paresis 42–68 May occur early or late during a severe attack. Muscle weakness usually begins proximally rather than distally and more often in the upper than lower extremities. 
    Respiratory paralysis 9–20 Preceded by progressive peripheral motor neuropathy and paresis. 
    Mental symptoms 40–58 May range from minor behavioral changes to agitation, confusion, hallucinations, and depression. 
    Convulsions 10–20 A central neurologic manifestation of porphyria or due to hyponatremia, which often results from syndrome of inappropriate antidiuretic hormone secretion or sodium depletion. 
Cardiovascular 
    Tachycardia 64–85 May warrant treatment ot control rate, if symptomatic (see text). 
    Systemic arterial hypertension 36–55 May require treatment during acute attacks, and sometimes becomes chronic. 
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