Systemic inflammation in acute intermittent porphyria: a case-control study.

This study aimed to examine whether acute intermittent porphyria (AIP) is associated with systemic inflammation and whether the inflammation correlates with disease activity. A case-control study with 50 AIP cases and age-, sex- and place of residence-matched controls was performed. Plasma cytokines, insulin and C-peptide were analysed after an overnight fast using multiplex assay. Long pentraxin-3 (PTX3) and complement activation products (C3bc and TCC) were analysed using enzyme-linked immunosorbent assay (ELISA). Urine porphobilinogen ratio (U-PBG, µmol/mmol creatinine), haematological and biochemical tests were performed using routine methods. Questionnaires were used to register AIP symptoms, medication and other diseases. All 27 cytokines, chemokines and growth factors investigated were increased significantly in symptomatic AIP cases compared with controls (P < 0·0004). Hierarchical cluster analyses revealed a cluster with high visfatin levels and several highly expressed cytokines including interleukin (IL)-17, suggesting a T helper type 17 (Th17) inflammatory response in a group of AIP cases. C3bc (P = 0·002) and serum immunoglobulin (Ig)G levels (P = 0·03) were increased significantly in cases with AIP. The U-PBG ratio correlated positively with PTX3 (r = 0·38, P = 0·006), and with terminal complement complex (TCC) levels (r = 0·33, P = 0·02). PTX3 was a significant predictor of the biochemical disease activity marker U-PBG in AIP cases after adjustment for potential confounders in multiple linear regression analyses (P = 0·032). Prealbumin, C-peptide, insulin and kidney function were all decreased in the symptomatic AIP cases, but not in the asymptomatic cases. These results indicate that AIP is associated with systemic inflammation. Decreased C-peptide levels in symptomatic AIP cases indicate that reduced insulin release is associated with enhanced disease activity and reduced kidney function.

Tryptase levels after suxamethonium administration and defibrillation.

BACKGROUND: A more than threefold increase in tryptase, when comparing with the control sample, strengthens the diagnosis of anaphylaxis. Trauma, coronary ischaemia and non-IgE-mediated reactions to several medications have been shown to cause more than threefold rise in tryptase levels. The aim of our study was to examine whether suxamethonium or defibrillation could lead to a more than threefold increase in tryptase in the absence of signs of anaphylaxis. METHODS: S-tryptase was measured in 50 patients who had general anaesthesia with either pentothal and suxamethonium before electro convulsive therapy (ECT) to treat depression (n=31) or propofol before electro conversion to treat atrial fibrillation (n=19). Blood samples were collected minutes before and 1 h after the procedures. RESULTS: Tryptase values did not differ significantly before and after the procedures. CONCLUSION: Tryptase levels do not increase in patients undergoing elective defibrillation or ECT with the administration of suxamethonium, in absence of symptoms of anaphylaxis.