Huntington Disease in Asia

Objective:

The objective was to review the major differences of Huntington disease (HD) in Asian population from those in the Caucasian population.

Data Sources:

Data cited in this review were obtained from PubMed database and China National Knowledge Infrastructure (CNKI) from 1994 to 2014. All the papers were written in English or Chinese languages, with the terms of Asia/Asian, HD, genotype, epidemiology, phenotype, and treatment used for the literature search.

Study Selection:

From the PubMed database, we included the articles and reviews which contained the HD patients’ data from Asian countries. From the CNKI, we excluded the papers which were not original research. Due to the language''s restrictions, those data published in other languages were not included.

Results:

In total, 50 papers were cited in this review, authors of which were from the mainland of China, Japan, India, Thailand, Taiwan (China), Korea, and western countries.

Conclusions:

The lower epidemiology in Asians can be partly explained by the less cytosine-adenine-guanine repeats, different haplotypes, and CCG polymorphisms. For the physicians, atypical clinical profiles such as the initial symptom of ataxia, movement abnormalities of Parkinsonism, dystonia, or tics need to be paid more attention to and suggest gene testing if necessary. Moreover, some pathogenesis studies may help progress some new advanced treatments. The clinicians in Asian especially in China should promote the usage of genetic testing and put more effects in rehabilitation, palliative care, and offer comfort of patients and their families. The unified HD rating scale also needs to be popularized in Asia to assist in evaluating the progression of HD.     

Multifocal pheochromocytoma-paraganglioma in a 29-year-old woman with cyanotic congenital heart disease

Background

Multifocal pheochromocytoma/paraganglioma presenting at an early age is commonly associated with a hereditary syndrome.

Midterm Outcomes of the Dissected Aorta Repair Through Stent Implantation Trial

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Arrhythmias induced by exercise in paced patients

Seventy-nine per cent of a series of 48 patients with implanted pacemakers developed arrhythmias during exercise. There were 23 patients without known previous competition, and in 69.5 per cent of them arrhythmias developed during exercise.No serious arrhythmias developed, but there were three cases of repetitive firing or short bursts of ventricular tachycardia, five instances of pacing on the apex of the T wave, and one instance of R-on-T phenomenon. None of these cases of ventricular tachycardia was preceded by spike-on-T or R-on-T stimulation.Based on these observations, recommendations for selection of the proper pacemaker have been made.