Atrioventricular nodal re-entrant tachycardia with QRS voltage and cycle length alternation and aberrant conduction due to two distinct antegrade slow pathways.

QRS voltage and cycle length alternation can be seen during supraventricular re-entrant tachycardias, especially in atrioventricular (AV) re-entrant tachycardia. We present a case of a 20-year-old man, in which AV nodal re-entrant tachycardia (AVNRT) shows alternation of QRS voltage and cycle length, as well as right bundle branch block aberration due to a re-entrant circuit using two distinct, beat-to-beat alternating slow AV nodal pathways antegradely and a single fast pathway retrogradely. Although more than one antegrade slow pathway exists, creation of a single lesion at the right posterior atrial septum using the conventional right-sided approach successfully eliminated AVNRT.     

Fibrin glue does not improve the fixation of press-fitted cell-free collagen gel plugs in an ex vivo cartilage repair model

Purpose  

Adequate graft fixation over a certain time period is necessary for successful cartilage repair and permanent integration of the graft into the surrounding tissue. The aim of the present study was to test the primary stability of a new cell-free collagen gel plug (CaReS^?-1S) with two different graft fixation techniques over a simulated early postoperative period.     

Penile revascularization in vasculogenic erectile dysfunction (ED): long-term follow-up

Study Type – Therapy (case series) Level of Evidence 4 What’s known on the subject? and What does the study add? This study reflects the second largest series of revascularization operations performed in different etiologic and risk groups with the longest follow up. Beyond that this is the only study in which CC‐EMG techniques were used preoperatively to exclude some patients and to predict postoperative success in penile revascularization.

OBJECTIVE

? To determine the overall long‐term success of penile revascularization surgery in the treatment of vasculogenic erectile dysfunction (ED) and also to investigate the effect of risk factors on the results of a modified Furlow–Fisher technique.

PATIENTS AND METHODS

? Between 1999 and 2010, 125 men with a mean (sd , range) age of 43.2 (11.3, 23–69) years underwent penile revascularization surgery. In all, 110 men completed the long‐term follow‐up with a mean follow‐up of 73.2 months. ? Diagnostic evaluations, penile colour Doppler ultrasonography, corpus cavernosum electromyography, and cavernosometry, were performed in all the men before surgery. ? The efficacy of the surgery was assessed as improvement or failure according to the change in the five‐item version of the International Index of Erectile Function (IIEF‐5). A ≥5 point increase in the IIEF‐5 score during the latest patient visit after surgery compared with that before surgery was regarded as improvement (surgical success).

RESULTS

? The mean (sd ) IIEF‐5 score was 7.3 (3.2) before surgery and at the end of the follow‐up periodit was 16.8 (3.1). ? The success rates were 81.8% at 3 months, 77.2% at 1 year, 70% at 2 years, 66.3% at 3 years and 63.6% at 5 years after surgery in the men who achieved a no‐ED threshold score of >26 in the IIEF‐15. ? The success rate was the highest in the men with no risk factors (92.8%). ? Seven patients (6.36%) showed signs of glans hypervascularization as a major complication.

CONCLUSIONS

? Penile revascularization surgery has not been widely used by urologists probably due to the technical difficulties and the use of phosphodiesterase type 5 inhibitors. ? However, with reported high rates of noncompliance or failure of oral pharmacotherapy it seems likely that this surgery will become more popular in the near future.     

Scimitar Syndrome and Azygos Continuation of the Inferior Vena Cava Diagnosed in an Adult—An Unusual Association

Anormal drainage of the pulmonary veins into the inferior vena cava is known as scimitar syndrome. Scimitar syndrome often presents during infancy and rarely during adulthood, and the adult patients are mostly asymptomatic. It is usually in association with dextrocardia, hypoplasia of right lung, and congenital heart defects. However, interruption of inferior vena cava with azygous continuation is rarely associated with this syndrome.     

The clinical significance of microvascular impairment in patients with pure uncomplicated diabetes mellitus

Pure uncomplicated type 2 diabetes mellitus is 'coronary artery disease equivalent' in view of microvascular impairment in myocardium. We believe that the current study reemphasized clinical significance of coronary microvasculature assesment in patients with diabetes mellitus even though they had normal luminogram.     

Autoimmune liver diseases in the Asia–Pacific region: Proceedings of APASL symposium on AIH and PBC 2016

During the 25th annual meeting of the Asia–Pacific Association for the Study of the Liver (APASL 2016) in Tokyo, we organized and moderated an inaugural satellite symposium on the autoimmune liver diseases, autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC). Following the keynote lecture by John M. Vierling (USA), speakers from the Asia–Pacific region provided an up-to-date perspective on the epidemiology, clinical practice and research in AIH and PBC in the Asia–Pacific region. Although epidemiology and clinical features of AIH seem to be similar in East Asia compared to those in western countries, the majority of patients with AIH are detected at an advanced stage and have higher mortality rates in South Asia, indicating an unmet need for earlier diagnosis and the initiation of appropriate immunosuppressive treatment. PBC is more commonly seen in Australia and East Asia. As of 2016, clinical practice guidelines (CPG) for PBC have been published in Japan and China. Ursodeoxycholic acid (UDCA) is recommended as a first-line therapy by both CPG. Nevertheless, one of the unmet therapeutic needs in PBC is the treatment of patients refractory to or intolerant of UDCA. It is of interest that the prevalence of chronic hepatitis B (CHB) in PBC patients was low in Taiwan and mainland China where the prevalence of CHB is very high. In this review, we overview this exciting and epoch-making symposium.     

Clinical phenotype,biochemical profile,and treatment in 19 patients with arginase 1 deficiency

Background

Arginase 1 (ARG1) deficiency is a rare urea cycle disorder (UCD). This hypothesis-generating study explored clinical phenotypes, metabolic profiles, molecular genetics, and treatment approaches in a cohort of children and adults with ARG1 deficiency to add to our understanding of the underlying pathophysiology.

Methods

Clinical data were retrieved retrospectively from physicians using a questionnaire survey. Plasma aminoacids, guanidinoacetate (GAA), parameters indicating oxidative stress and nitric oxide (NO) synthesis as well as asymmetric dimethylarginine (ADMA) were measured at a single study site.

Results

Nineteen individuals with ARG1 deficiency and 19 matched controls were included in the study. In patients, paraparesis, cognitive impairment, and seizures were significantly associated suggesting a shared underlying pathophysiology. In patients plasma GAA exceeded normal ranges and plasma ADMA was significantly elevated. Compared to controls, nitrate was significantly higher, and the nitrite:nitrate ratio significantly lower in subjects with ARG1 deficiency suggesting an advantage for NO synthesis by inducible NO synthase (iNOS) over endothelial NOS (eNOS). Logistic regression revealed no significant impact of any of the biochemical parameters (including arginine, nitrates, ADMA, GAA, oxidative stress) or protein restriction on long-term outcome.

Conclusion

Three main hypotheses which must be evaluated in a hypothesis driven confirmatory study are delineated from this study: 1) clinical manifestations in ARG1 deficiency are not correlated with arginine, protein intake, ADMA, nitrates or oxidative stress. 2) GAA is elevated and may be a marker or an active part of the pathophysiology of ARG1 deficiency. 3) Perturbations of NO metabolism merit future attention in ARG1 deficiency.