Hepatic Sarcoidosis with Portal Hypertension. A Report of Seven Cases with a Review of the Literature

We have reviewed the clinical, histological and hemodynamicfeatures of sarcoidosis complicated by portal hypertension inseven patients and in 40 previously reported cases. Young blackpatients of either sex and white females over 40 years wereselectively affected. In 12 of these 47 patients, portal hypertensionappeared to be a consequence of cirrhosis due to longstandingintrahepatric cholestasis; in white patients, this conditionwas clinically, histologically, and serologically indistinguishablefrom primary biliary cirrhosis. In most of the other patients,portal hypertension was the predominant and often the presentingsymptom of hepatic sarcoidosis; in these patients portal hypertensionwas due to a presinusoidal block probably determined by portalgranulomas, with or without superimposed sinusoidal block determinedby fibrosis. Corticosteroids did not prevent the developmentof portal hypertension.     

Allergy to suxamethonium: persisting abnormalities in skin tests, specific IgE antibodies and leucocyte histamine release

Twenty-one patients, who had previously experienced an anaphylactic reaction to suxamethonium during general anaesthesia, were selected for this study. Initially, skin tests with muscle relaxants were carried out in the twenty-one patients, detection of specific anti-choline IgE in nineteen, and leucocyte histamine release in seventeen. These three tests were then repeated between 1 year and 4 years after the initial evaluation. In the majority of patients, sensitization to the muscle relaxants persisted for more than 1 year after the anaphylactic reaction. Only three patients out of twenty-one (4%) had negative skin tests when retested 1–4 years later. A reduction in leucocyte histamine release was noticed in one of the seventeen retested patients (6%). Modifications of anti-choline IgE were observed in five of nineteen patients (26%). The persistence of sensitization to suxamethonium may result from repeated stimulation by occasional contacts with quaternary ammonium compounds. This study demonstrates the reliability of skin tests, leucocyte histamine release and detection of anti-choline IgE to diagnose allergic reactions to suxamethonium, even when they are performed a long time after the initial anaphylactic reaction.     

One‐Year Results of the CRISTAL Trial,a Randomized Comparison of Cypher Sirolimus‐Eluting Coronary Stents versus Balloon Angioplasty for Restenosis of Drug‐Eluting Stents

Objectives: We compared the efficacy of the Cypher Select? (Cordis Corporation, Bridgewater, NJ, USA) sirolimus‐eluting stent (SES) versus balloon angioplasty (BA) in in‐stent restenosis (ISR) of Taxus? or Taxus Liberté? paclitaxel‐eluting stents (PES; Boston Scientific, Natick, MA, USA) or Cypher/Cypher Select SES. Background: Optimal treatment strategies have not been identified for drug‐eluting stent (DES) ISR. Methods: Patients with a native coronary artery SES or PES ISR were randomized to SES or BA. In addition, a control group included BMS ISR treated with SES. Angiographic control was performed at 12 months. Results: 281 patients were enrolled. Significant differences favoring SES over BA were noted in immediate and net gain (1.39 ± 0.51 vs. 0.97 ± 0.54 mm, P < 0.0001 and 1.07 ± 0.69 vs. 0.49 ± 0.67 mm, P < 0.0001), 12‐month mean luminal diameter (MLD; 2.14 ± 0.62 vs. 1.71 ± 0.55 mm, P < 0.0001) and percent diameter stenosis (%DS; 21 ± 19.24 vs. 29.82 ± 18.47, P = 0.001). There was no significant difference at 12 months between SES and BA in the primary end‐point late lumen loss (LLL; 0.37 ± 0.57 vs.0.41 ± 0.63, P = 0.73) and in in‐stent binary restenosis (11.1% vs. 14%, P = 0.59). Target‐lesion revascularization (TLR) was numerically lower in patients treated with SES (5.9% vs. 13.1%, P = 0.097). There was no difference according to the initial DES. In contrast, significantly higher immediate and net gains and MLD were noted in the BMS control group treated by SES. Conclusions: In this angiographic randomized trial comparing SES and BA in SES or PES restenosis, 12 month MLD, immediate and net gain, and %DS favored SES whereas no difference was noted in LLL. Condensed Abstract Optimal treatment strategies have not been identified for sirolimus‐ (SES) or paclitaxel‐eluting stent (PES) in‐stent restenosis (ISR). We randomized patients with a native coronary artery SES or PES ISR to SES or BA. In addition, a control group included BMS ISR treated with SES. There was no difference in the primary end‐point, late lumen loss (LLL) at 12 months between the SES and BA groups. However, follow‐up MLD and immediate and net gain favored SES. (J Interven Cardiol 2012;25:586–595)     

Inadvertent Implantation of an ICD Lead in the Great Cardiac Vein

We report an implantable cardioverter defibrillator (ICD) implanted with a single lead inadvertently introduced in the great cardiac vein. No venous lesion was caused by the shocks and the position of the lead remained stable. This case emphasizes the usefulness of different fluoroscopic views during ICD implantation.     

Severe and Early Alteration of Action Potential During Acute Cardiac Rejection in Rats

Alteration of Cardiac Action Potential . Introduction: Alteration of cardiac action potential and its adaptation to heart rate could contribute to cardiac dysfunction and arrhythmias during acute cardiac rejection.
Methods and Results: Heterotopic heart transplantation was performed in allogeneic and syngeneic rats in which the action potentials of right and left ventricles were measured at 1, 2.5, 3.3, and 5.7 Hz successively using standard microelectrode techniques and compared with nontransplanted hearts. For each frequency, we measured action potential amplitude, action potential duration, transmembrane resting potential, and Vmax. In the right ventricle, at 1 Hz in the presence of rejection (n = 40), a significant increase was observed in action potential duration at 20%, 50%, and 70% repolarization (82.5%, 75.6%, and 70.8%, respectively) and in action potential amplitude (+17.9 mV), and the resting potential was decreased (-5.3 mV). A lack of adaptation of action potential duration to the driving frequency was observed in the rejecting heart group in contrast to controls (n = 20) and nourejecting hearts (n = 13). Similar results were observed in the left ventricle and surprisingly in the native hearts (n = 11) of recipients with allografted rejecting hearts in the abdominal position.
Conclusion: Action potential and its adaptation to the driving frequency is considerably altered during acute rejection. A humoral factor could contribute to cardiac dysfunction.     

Direct evidence for dissociated megakaryocytic chimaerism in a Wiskott-Aldrich patient successfully allografted

Summary. We report a Wiskott-Aldrich patient who underwent allogeneic bone marrow transplantation from his HLA-identical sister at the age of 25. Conditioning regimen consisted of cyclophosphamide (180mg/kg) and thoracoabdominal irradiation (6 Grays). Cytogenetic follow-up revealed rapid and complete lymphoid chimaerism, but prolonged mixed bone marrow chimaerism. Correlative interphase cytogenetics performed on bone marrow smears using dual-colour fluorescence in situ hybridization with × and Y specific probes showed that the proportion of donor cells was significantly higher within megakaryocytes than in other lineages. This patient therefore presented with dissociated lineage engraftment, which is not exceptional in congenital diseases and aplastic anaemia, but has not previously been described in Wiskott-Aldrich syndrome. Bone marrow transplantation was successful despite this delayed engraftment which ensured adequate production in the involved cell lines.