Long-term survival without surgical intervention in a patient with a natural history of a single right ventricle: A case report.

Single ventricle (SV), a complex cardiac anomaly, if left untreated, is thought to lead to a poor prognosis. Herein, we report the case of a long-term survivor with an unrepaired SV with right ventricle (RV) morphology. A 55-year-old man presented with a SV with RV morphology, with a double outlet, large atrial septal defect, common atrioventricular valve, pulmonary valve stenosis (PS), and dextrocardia. Because the native PS provided adequate restriction of the pulmonary blood flow, he did not develop pulmonary hypertension; however, he had severe cyanosis. In patients with SV and moderate PS, even if the SV has RV morphology, long-term survival may be possible without surgical intervention. .

Pacemaker Treatment for Apical Hypertrophic Cardiomyopathy in the Setting of an Apical Ventricular Aneurysm.

We describe the case of a patient with apical hypertrophic cardiomyopathy with concomitant apical aneurysm. We measured the aneurysmal cavity pressure using the pressure guidewire system. The patient underwent implantable cardioverter-defibrillator treatment successfully to reduce the pressure gradient between the aneurysmal cavity and the true left ventricle. (Level of Difficulty: Intermediate.).

[Role of IL-33 in bronchial asthma].

IL-33 is a member of the IL-1 family and has been identified as an agonist of ST2L. IL-33 drives the production of Th2-associated cytokines and IgE, and IL-33 administration induces eosinophilia and hypertrophy of bronchial epithelial cells, as well as mucus secretion in vivo. Such changes resemble pathological findings in bronchial asthma (BA). In this study, we investigated the relationship between IL-33 and BA by evaluating serum IL-33 levels. Serum was obtained from BA patients (n = 20), emphysema patients (n = 5) and from non-smoking healthy controls (n = 8). IL-33 levels were assayed by enzyme-linked immunosorbent assay. Then, we divided BA patients according to 5 factors; (1) IgE concentration, (2) eosinophil count, (3) current treatment, (4) classification of severity, and (5) smoking status. Atopic BA patients showed significantly higher IL-33 levels than non-atopic patients. IL-33 was significantly higher in untreated patients, and in the moderate and severely affected groups. Smoking BA and emphysema patients had lower levels than nonsmoking BA patients. Eosinophil counts were not related to IL-33 levels. The present study suggests that IL-33 is closely associated with IgE levels and the exacerbation of BA. We speculated that IL-33 elevation is responsible for the maintenance of airway inflammation and hypersensitivity. It is possible that low IL-33 levels in smokers are caused by the deterioration of the airway epithelium and endothelium.

Reversible lesion in the splenium of the corpus callosum in a patient with chronic alcoholism.

Marchiafava-Bignami disease (MBD) is often diagnosed in chronic alcoholics. The disease processes typically involve the corpus callosum and clinically presents with various manifestations on the basis of clinical condition, extent of the splenium of the corpus callosum involvement at brain magnetic resonance imaging (MRI), and prognosis. We report a patient affected by MBD, who presented an isolated reversible splenial lesion at brain MRI and achieved a favorable recovery.

Opening of ligand-gated cation channel families by calpain inhibitors.

The class of Ca2+-permeable cation channels is composed of large families with six transmembrane segments including transient receptor potential, vanilloid receptor (VR), polycystin, epithelial calcium channels and melastatin (MLS). However, most of them are functionally silent and unexpressed in mammalian cells. An investigation of associated proteins made us believe that the blockade of calpain opens the silent channels. Using 1 microM of blockers in whole cellular patch pipette fill we measured currents of Chinese hamster ovary cells transfected by VR-like 1 and 2, polycystin-2, or a MLS-like new member (MLS3S). Significant conductance of every clone with a characteristic rectification by blockers was demonstrated. The permeability of Ca2+ to them is similar to that reported. Western blot suggested that blockers did not affect the assembly of the protein but enabled its cleavage. Therefore, investigation of these families with the blockers may boost our knowledge of electrophysiologic function.

A single amino acid mutation results in a rapid inactivation of epithelial calcium channels.

Epithelial Ca(2+) channel (ECaC1 and 2 = CAT1) molecules are characterized by properties including inward rectification and Ca(2+)-dependent fast and slow inactivation. To elucidate the electrophysiological differences based on the amino acid residues, we compared human and rodent ECaC1, and ECaC2 alignments, made mutants, and investigated their function in Xenopus and mammalian cells. Expression of the ECaC1 mutant Q579H and a H587Q mutation in ECaC2 in Xenopus oocytes resulted in a possible change in the rate of fast decay. Currents of H587C and H587N were not detected, and the H587R diminished the rate of rapid decay. Treatment of the oocytes with BAPTA magnified the amplitude of the current and abolished the decay. The expressions of mutants, therefore, implied that H587 in ECaC2 is a position related to the mechanism of the rapid decay rather than the magnitude of the current or the slow decay. Decay measurements were carefully performed in mammalian cells by tight-seal patch clamping. The rapid decay was exaggerated in H587C and H587N mutants but was undetectable in the H587R mutant. The results indicate that the amino acid 579Q of ECaC1, corresponding to 587H of ECaC2, is of primary importance in the structure for the fast inactivation by intracellular Ca(2+).