Acute intermittent porphyria after gastroplasty

Abstract

A 28-year-old female underwent gastroplasty for obesity in an uneventful procedure. This was followed within five days by persistent abdominal pain associated with weakness that progressed to generalized flaccid quadriparesis. She developed respiratory distress, and required intubation. Cerebrospinal fluid (CSF) revealed a normal cell count and elevated total protein. Her folate and vitamin B12 levels were normal. Electromyography (EMG) and nerve-conduction studies (NCS) showed acute generalized motor-sensory demyelinating neuropathy predominantly in the lower limbs. A diagnosis of Guillain-Barré syndrome was made, and a five-day course of immunoglobulin was begun, which did not produce any improvement.Three months later, she still had not improved and she came into our service looking for a diagnosis.The patient reported that seven relatives had lower limb weakness, and that five of them died due to respiratory failure after the onset of neurological manifestations. Neurological examination showed moderate quadriparesis, with proximal weakness greater than distal weakness. Muscle reflexes were absent, and no pathological reflexes were demonstrated. The patient's sense of feeling was normal.The blood tests were normal. We repeated the EMG and NCS, which showed pure motor axonal polyradiculoneuropathy.The porphobilinogen (PBG) and δ-aminolevulinic acid (ALA) levels in urine were high: 32.4 mg/24h (normal range: 0-2 mg/24h) and 26.2 mg/g, (normal value: 4.5 mg/g) respectively, thus indicating the diagnosis of acute intermittent porphyria.Six months later, the patient returned complaining of a burning feeling in her lower abdomen and upper limbs. Two days after this, she became weak again and developed quadriparesis. She was then treated with intravenous glucose (300 g per day), with a slight improvement. Hematin was not used because it was not available at our service.Because the patient's condition continued to worsen, we decided to reverse the gastric bypass, given that this could decrease the risk of further attacks. Within a couple days, she got better and her muscle strength gradually improved. DISCUSSIONThe case reported above is important because it provides the description of a porphyria crisis subsequent to bariatric surgery in a patient without any previous medical history. The first acute episode was triggered by gastroplasty, and recurrence of the attack was probably due to the persistent nutritional disorder caused by surgery. The low-calorie diet (600 kcal/day) that the patient had been following since the gastroplasty could have been the precipitating factor for the second crisis.With regard to correlations between polyneuropathy and gastroplasty, Abarbanel et al. studied 500 patients with gastroplasty but only 16 (3.2%) were identified as presenting polyneuropathy 1 . Thaisetthawatkul et al found that 71 patients (16%) presented polyneuropathy out of 435 patients who underwent bariatric surgery. Such cases of neuropathy have multifactorial causes, but malnutrition may be the most important risk fac...

Acute intermittent porphyria after right hemi-colectomy

“…These neuro-visceral and psychiatric manifestations are most likely due to involvement of the autonomic, peripheral and central nervous system secondary to elevated PBG and ALA [1], [2], [3]. Similar to others, family history of AIP in our patient was positive [3], [5], [11]. But at times, patients give no family history of AIP [1], [3].…”

“…Acute intermittent porphyria after surgery is very rare with only few case reports in the literature [9], [10], [11], [12], [13]. We present what we believe is the first case of AIP after right hemi-colectomy.…”

“…She had a smooth postoperative course. Most likely, her attack of AIP was precipitated by a combination of (1) hormonal changes during menstruation, (2) epidural analgesia of lidocaine and fentanyl, (3) low-calorie intake during the postoperative period (510 Kcal/day) and (4) surgical site pain after cessation of epidural analgesia [1], [11]. Medline search revealed only five cases of AIP after surgery (Table 1) [9], [10], [11], [12], [13].…”

“…Most likely, her attack of AIP was precipitated by a combination of (1) hormonal changes during menstruation, (2) epidural analgesia of lidocaine and fentanyl, (3) low-calorie intake during the postoperative period (510 Kcal/day) and (4) surgical site pain after cessation of epidural analgesia [1], [11]. Medline search revealed only five cases of AIP after surgery (Table 1) [9], [10], [11], [12], [13]. Other triggers that increase the demand for heme and hence induce AIP include porphyrinogenic drugs, infections, smoking, alcohol intake, fasting, psychological stress, and hormonal changes associated with menstruation and ovulation [1], [2], [3].…”

“…Medline search revealed only five cases of AIP after surgery (Table 1) [9], [10], [11], [12], [13]. Other triggers that increase the demand for heme and hence induce AIP include porphyrinogenic drugs, infections, smoking, alcohol intake, fasting, psychological stress, and hormonal changes associated with menstruation and ovulation [1], [2], [3].…”