Diagnosis of acute intermittent porphyria in a renal transplant patient:A case report

BACKGROUND Acute intermittent porphyria(AIP)is an inherited disorder of porphyrin metabolism with a worldwide distribution and a prevalence ranging from 1 to 9 per million population.AIP is caused by an autosomal dominant-inherited mutation of low penetrance resulting in a deficiency of porphobilinogen deaminase(PBGD)activity.Acute attacks are provoked by stressors such as certain medications,alcohol,and infection.We herein present the first case report of AIP detected in a post-renal transplant patient.CASE SUMMARY The patient was a 65-year-old man who underwent transplantation 2 years previously for suspected nephroangiosclerosis and chronic interstitial nephropathy.He subsequently developed diabetes mellitus which required insulin therapy.He had been treated in the recent past with local mesalamine for proctitis.He presented with classic but common symptoms of AIP including intense abdominal pain,hypertension,and anxiety.He had multiple visits to the emergency room over a 6-mo period for these same symptoms before the diagnosis of AIP was entertained.His urinary postprandial blood glucose level was 60 mg/24 h(normal,<2 mg/24 h).He was placed on a high carbohydrate diet,and his symptoms slowly improved.CONCLUSION This case report describes a common presentation of an uncommon disease,in which post-transplant complications and medications may have contributed to precipitating the previously undiagnosed AIP.We hypothesize that the lowcarbohydrate diet and insulin with which our patient was treated may have led to the attacks of AIP.Alternatively,our patient’s mesalamine treatment for proctitis may have led to an acute AIP crisis.A high index of suspicion is needed to consider the diagnosis of a heme synthesis disorder,which presents with the common symptoms of abdominal pain,high blood pressure,and anxiety.

Exploring the role of prophylactic levosimendan in coronary surgery

Background:The role of prophylactic levosimendan in coronary surgery has not been established conclusively.Methods:Postoperative outcomes of 139 patients(mean age,68.2±9.6 years)having preoperative left ventricular ejection fraction(LVEF)≤40%and undergoing isolated coronary surgery(2013-2017)were reviewed retrospectively.In 42(30.2%)patients(L-group),an intravenous infusion of levosimendan was started 24 hours before operation.The remaining 97(69.8%)patients were the control group(C-group).A comparison between the two groups regarding outcome of surgery was performed also after propensity matching.Results:Although the risk profile in L-patients was higher than in C-patients(median European System for Cardiac Operative Risk Evaluation II,10.5%vs.6.5%,P=0.013)due to higher prevalence of New York Heart Association class III-IV,LVEF≤30%,and preoperative intra-aortic balloon pump,in-hospital mortality was equivalent(4.8%vs.3.1%,P=0.48).However,low cardiac output,multiple blood transfusion,and any major complication early after surgery were more frequent in L-patients.After one-to-one propensity matching,which resulted in 15 pairs with similar baseline characteristics the use of levosimendan was associated with a trend towards an increased blood use(P=0.077),a higher frequency of any major complication(P=0.053),and lower peak serum levels of cardiac troponin I(P=0.088).No intergroup differences concerning mid-term survival or outcomes were found even for matched patients.Conclusions:When compared with traditional inotropes alone,prophylactic use of levosimendan showed clear benefits/drawbacks neither concerning immediate nor mid-term outcomes after coronary surgery.There could be any advantage in terms of myocardial preservation.