Acute porphyric attack with cold sweating and low blood pressure

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American Journal of Emergency Medicine

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Acute porphyric attack with cold sweating and low blood pressure?,??


Overproduction of toxic heme precursors leads to neurovisceral manifestations in porphyria. Porphyria can affect the autonomic, peripheral, and central nervous systems. Many drugs and steroids can induce an acute attack of porphyria. Because of its rarity and ability to mimic other diseases, porphyrias are often overlooked and misdiag- nosed. Hypertension and tachycardia are reported in patients with acute porphyric attack and are associated with increased Sympathetic activity. Here we reported an interesting case with intermittent cold sweating and shock in acute porphyric attack. A 52-year-old woman presented with dysuria, cold sweating, intermittent low blood pressure, occasional ventricular premature beats, and dark-colored urine. Metabolic disease was concerned in this patient due to the intermittent course of illness. Autonomic neuropathy due to por- phyria was suspected because of dysuria and dark urine. Her symptoms improved after intravenous glucose injection. Porphyrin level evaluation showed that she had experienced an acute porphyria attack. This is the first case report of acute porphyria presenting with intermittent cold sweating and shock. In a sudden onset of shock and arrhythmia of unknown etiology, metabolic disease such as porphyria should be considered.

A 52-year-old woman was admitted due to dysuria for 2 weeks. She reported that for 2 weeks before admission, she had had poor appetite, headache, nausea, vomiting, and constipation. She took nalidixic acid, trichlormethiazide, estradiol, norethindrone, calcium carbonate, and antacid from a local clinic, but her symptoms persisted. The patient had visited the emergency department (ED) 2 days before admission. At that time, urine examination showed white blood cell count of 5 to 10/high-power field. Treatment with trimethoprim- sulfamethoxazole was started, and she was discharged. She com- plained of generalized weakness after taking the medication, and she was admitted via the ED.

Intermittent cold sweating developed in the first hour of admission. She had excessive sweating. Her blood pressure dropped from 120/70 to 60/40 mm Hg. Her heartbeat was 70 to 90 beats per minute. She did not develop hematemesis, hematochezia, melena, or diarrhea. Cold sweating and low blood pressure occurred intermittently under rapid normal saline infusion. She remained lucid and conscious throughout. She had normal motor strength and reflexes. She denied chest pain. Electrocardiography showed normal

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sinus rhythm with occasional ventricular premature beats. Chest radiography, serum cardiac enzyme, and cardiac did not reveal significant abnormality.

Metabolic disease was suspected in this patient due to the intermittent course of illness. She reported that her urine was dark colored. Because she had dysuria and dark urine, autonomic neuropathy due to porphyria was suspected. We prescribed 10% glucose water. The attacks of cold sweating and low blood pressure occurred only once after glucose-water injection. The frequency of ventricular premature beats gradually decreased and disappeared on the second day of admission. We administered 300 mg intravenous glucose daily for 5 days. Her symptoms of dysuria improved after therapy.

The patient’s results for 24-hour urine coproporphyrinogen levels, which demonstrated a urine coproporphyrin level of 1060 nmol (reference range, 0-245 nmol/24 hours), proved that the patient had an acute porphyric attack.

Porphyria is an inherited Metabolic disorder, resulting from a partial deficiency of a specific enzyme in the heme biosynthesis pathway [1]. Porphyria can affect the autonomic, peripheral, and central nervous systems [2]. Hypertension and tachycardia are reported in patients with acute porphyric attack and are associated with increased sympathetic activity [3]. A prolonged low-carbohy- drate intake along with estradiol, norethindrone, and nalidixic acid prescribed at a local medical clinic and trimethoprim-sulfamethox- azole prescribed at the ED may have contributed to the acute porphyric attack in this patient [4,5]. Early suspicion and prompt management prevent other severe complications of porphyria such as lethal cardiac arrhythmia [6].

neurologic symptoms are always intermittent in acute porphyric attack [7]. Cold sweating may have been due to an increase of sympathetic activity. The exaggerated fall in blood pressure may have been caused by blood volume loss due to cold sweating. The patient’s blood pressure did not increase after normal saline infusion, implying that low blood pressure may be compounded by decreased splanchnic capacitance, which normally acts to maintain venous return and stroke volume. In the setting of a generalized increase in sympathetic activity, sustained VAsoconstriction of the splanchnic circulation may decrease the capacitance of the venous reservoir and exaggerate the fall in stroke volume during rapid changes in central blood volume [8].

The challenge of correctly diagnosing this patient’s condition mainly involved extracting important details from a large number of Nonspecific complaints. To our knowledge, this is the first report on an acute porphyric attack with intermittent cold sweating and low blood pressure. We believe that the findings of this report will help others clinicians learn from our experience.

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Yuag-Meng Liu MD

Department Infectious Disease, Changhua Christian Hospital

Changhua, Taiwan Graduate Institute of Clinical Medical Science

China Medical University

Taichung, Taiwan E-mail address: [email protected]


  1. Kauppinen R. Porphyrias. Lancet 2005;365:241-52.
  2. Kang SY, Kang JH, Choi JC, et al. posterior reversible encephalopathy syndrome in a patient with acute intermittent porphyria. J Neurol 2010;257:663-4.
  3. Ridley A, Hierons R, Cavanagh JB. Tachycardia and the neuropathy of porphyria. Lancet 1968;2:708-10.
  4. Bonkovsky HL, Siao P, Roig Z, et al. Case records of the Massachusetts General Hospital. Case 20-2008. A 57-year-old woman with abdominal pain and weakness after gastric bypass surgery. N Engl J Med 2008;358:2813-25.
  5. Meyer UA, Schuurmans MM, Lindberg RL. Acute porphyrias: pathogenesis of neurological manifestations. Semin Liver Dis 1998;18:43-52.
  6. Sassa S. Modern diagnosis and management of the porphyrias. Br J Haematol 2006;135:281-92.
  7. Laiwah AC, Macphee GJ, Boyle P, et al. Autonomic neuropathy in acute intermittent porphyria. J Neurol Neurosurg Psychiatry 1985;48:1025-30.
  8. Griffith JC, Jardine DL, Bailey W, et al. Variegate porphyria presenting with acute Autonomic dysfunction, intussusception and Renal infarction. Scand J Gastroenterol 2004;39:500-3.

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