Lopes Daniela von Ah, Valle Marcelo Araújo do, Taguti Jéfferson, Taguti Regina Celli Thomé Castro, Betônico Gustavo Navarro, Medeiros Fabiana Clemente
Unidade de Terapia Intensiva, Hospital IAMADA, Presidente Prudente, SP, Brasil.
Serviço de Cirurgia, Hospital IAMADA, Presidente Prudente, SP, Brasil.
Rev Bras Ter Intensiva. 2008 Dec;20(4):429-34.
Acute intermittent porphyria is an unusual pathology with potentially severe consequences when not early detected. Among the possible causes of porphyric crises decrease of caloric intake has been described. A case of acute intermittent porphyria in the late postoperative period of a bariatric surgery performed for treatment of obesity is reported. A review of the diagnostic aspects and management of this pathology in the intensive care unit follows. A 31 year old woman was admitted in the intensive care unit three weeks after a bariatric surgery, with decreased level of consciousness and respiratory distress. The patient evolved with psychomotor agitation, mental confusion, abdominal pain and proximal tetraparesis. Diagnosis investigation disclosed severe hyponatremia (92mEq/L), hypomagnesemia, hypophosfatemia and hypocalcemia and cloudy urine without hematuria. Acute porphyria was suspected and the urine test detected high delta amino-levulinic acid and porphobilinogen. Treatment consisted of a correction of electrolyte disturbances and high carbohydrate intake. Hematin and heme arginate were not used, due to the difficulty to acquire the medication. After 8 months the patient progressed with full recovery of muscle strength and a clinical improvement. Acute intermittent porphyria has signs and symptoms common to several clinical, neurological, psychiatric and gastroenterological pathologies, which complicate diagnosis. Therefore, acute intermittent porphyria should be included in the differential diagnosis of neurological, psychiatric and gastroenterological alterations when results of all other exams are normal. Attention must be given to patients undergoing surgery mainly bariatric that, in addition to procedure stress, substantially limit the total caloric intake, potentially triggering crises. Review of literature did not disclose any report of acute intermittent porphyria crisis induced by bariatric surgery.
急性间歇性卟啉病是一种特殊的病理状况，若不及早发现可能会导致严重后果。在卟啉病发作的可能原因中，热量摄入减少已被提及。本文报告了一例在肥胖症治疗性减重手术后晚期出现急性间歇性卟啉病的病例。接下来将对该病症在重症监护病房的诊断要点及治疗进行综述。一名31岁女性在减重手术后三周因意识水平下降和呼吸窘迫入住重症监护病房。患者出现精神运动性激越、精神错乱、腹痛和近端四肢轻瘫。诊断检查发现严重低钠血症（92mEq/L）、低镁血症、低磷血症和低钙血症，尿液浑浊但无血尿。怀疑为急性卟啉病，尿液检测发现高浓度的δ-氨基-γ-酮戊酸和卟胆原。治疗包括纠正电解质紊乱和高碳水化合物摄入。由于难以获取药物，未使用血红素和精氨酸血红素。8个月后患者病情好转，肌肉力量完全恢复，临床症状改善。急性间歇性卟啉病具有一些常见于多种临床、神经、精神和胃肠病症的体征和症状，这使得诊断变得复杂。因此，当所有其他检查结果正常时，急性间歇性卟啉病应纳入神经、精神和胃肠改变的鉴别诊断中。对于接受手术（主要是减重手术）的患者必须予以关注，因为除了手术应激外，这类手术还会大幅限制总热量摄入，有可能引发发作。文献综述未发现任何关于减重手术诱发急性间歇性卟啉病发作的报告。
