Structural and functional cardiac changes in myotonic dystrophy type 1: a cardiovascular magnetic resonance study

Background

Myotonic dystrophy type 1 (MD1) is a neuromuscular disorder with potential involvement of the heart and increased risk of sudden death. Considering the importance of cardiomyopathy as a predictor of prognosis, we aimed to systematically evaluate and describe structural and functional cardiac alterations in patients with MD1.

Methods

Eighty MD1 patients underwent physical examination, electrocardiography (ECG), echocardiography and cardiovascular magnetic resonance (CMR). Blood samples were taken for determination of NT-proBNP plasma levels and CTG repeat length.

Results

Functional and structural abnormalities were detected in 35 patients (44%). Left ventricular systolic dysfunction was found in 20 cases, left ventricular dilatation in 7 patients, and left ventricular hypertrophy in 6 patients. Myocardial fibrosis was seen in 10 patients (12.5%). In general, patients had low left ventricular mass indexes. Right ventricular involvement was uncommon and only seen together with left ventricular abnormalities. Functional or structural cardiac involvement was associated with age (p = 0.04), male gender (p < 0.001) and abnormal ECG (p < 0.001). Disease duration, CTG repeat length, severity of neuromuscular symptoms and NT-proBNP level did not predict the presence of myocardial abnormalities.

Conclusions

CMR can be useful to detect early structural and functional myocardial abnormalities in patients with MD1. Myocardial involvement is strongly associated with conduction abnormalities, but a normal ECG does not exclude myocardial alterations. These findings lend support to the hypothesis that MD1 patients have a complex cardiac phenotype, including both myocardial and conduction system alteration.     

Improved surgical mesh integration into the rat abdominal wall with arginine administration

Prosthetic meshes are used as the standard of care in abdominal wall hernia repair. However, hernia recurrences and side effects remain unsolved problems. The demand by health care providers for increasingly efficient and cost-effective surgery encourages the development of newer strategies to improve devices and outcomes. Here, we evaluated whether l-arginine administration was able to ameliorate long-term polypropylene prostheses incorporation into the abdominal wall of Sprague-Dawley rats. Meshes were placed on-lay and continuous l-arginine was administered. In vivo biocompatibility was studied at 7, 25 and 30 days post-implantation. Effectively, l-arginine administration in combination with mesh triggered subtle changes in ECM composition that impinged on critical biochemical and structural features. Lastly, tensile strength augmented and stiffness decreased over the control condition. This could help to restructure the mechanical load transfer from the implant to the brittle surrounding tissues, i.e., impact load and fatigue load associated with mechanical tensions could be distributed between the mesh and the restored tissue in a more balanced manner, and ultimately help to reduce the incidence of loosening, recurrences, and local wound complications. Since the newly formed tissue is more mechanically stable, this approach could eventually be introduced to human hernia repair.     

Observations of crypt neuron-like cells in the olfactory epithelium of a cartilaginous fish

A new receptor neuron (RN) type was recently described in bony fish olfactory epithelium (OE): the crypt receptor neuron. This name is due to its main feature: the presence, at the apical part, of a deep invagination into which cilia protrude. The presence of this receptor neuron type is well documented in different species of bony fishes but it has never been described in cartilaginous fishes. In this study we demonstrate that crypt neuron-like cells are present in the olfactory epithelium of the elasmobranch Scyliorhinus canicula (Linnaeus, 1758). Histological observations allowed us to detect the presence of a few egg-shaped cells, characterized by a crypt like zone; alpha-tubulin immunoreactivity suggested the presence of cilia in the same area; fluorocrome conjugated lectin bindings suggested a distinctive mucus composition inside the presumptive crypt. The possible presence of crypt neuron-like cells in chondrichthyes would represent an interesting common feature between bony and cartilaginous fishes.     

Rosuvastatin protects against podocyte apoptosis in vitro

BACKGROUND: Clinical studies suggest that statins reduce proteinuria and slow the decline in kidney function in chronic kidney disease. Given a rich literature identifying podocyte apoptosis as an early step in the pathophysiological progression to proteinuria and glomerulosclerosis, we hypothesized that rosuvastatin protects podocytes from undergoing apoptosis. Regarding a potential mechanism, our lab has shown that the cell cycle protein, p21, has a prosurvial role in podocytes and there is literature showing statins upregulate p21 in other renal cells. Therefore, we queried whether rosuvastatin is prosurvival in podocytes through a p21-dependent pathway. METHODS: Two independent apoptotic triggers, puromycin aminonucleoside (PA) and adriamycin (ADR), were used to induce apoptosis in p21 +/+ and p21 -/- conditionally immortalized mouse podocytes with or without pre-exposure to rosuvastatin. Apoptosis was measured by two methods: Hoechst 33342 staining and fluorescence-activated cell sorting (FACS). To establish a role for p21, p21 levels were measured by western blotting following rosuvastatin exposure and p21 was stably transduced into p21 -/- mouse podocytes. RESULTS: Rosuvastatin protects against ADR- and PA-induced apoptosis in podocytes. Further, exposure to rosuvastatin increases p21 levels in podocytes in vitro. ADR induces apoptosis in p21 -/- mouse podocytes, but rosuvastatin's protective effect is not seen in the absence of p21. Reconstituting p21 in p21 -/- podocytes restores rosuvastatin's prosurvival effect. CONCLUSION: Rosuvastatin is prosurvival in injured podocytes. Rosuvastatin exerts its protective effect through a p21-dependent antiapoptotic pathway. These findings suggest that statins decrease proteinuria by protecting against podocyte apoptosis and subsequent podocyte depopulation.     

血小板输注:将来的方向

引言过去几年中,血小板输注治疗已取得了显著进展。首先,已有两种降低浓缩血小板细菌污染率的方法,即灭活病原体和细菌检测。但两类方法均未被管理机构完全接受。一旦这些技术被常规运用,可能延长的血小板保存期超过目前批准的5天, 以及保存期延长血小板的质量得以维持。就血小板输注指征而言,几项临床试验已证实血小板输注阈值从以前的20000/μL降至10000／μL是安全有效的。进一步     

Comparison of bacteria growth in single and pooled platelet concentrates after deliberate inoculation and storage

BACKGROUND: The ability to store pools of platelet concentrates (PCs) for extended periods would provide logistical flexibility. However, reports of severe adverse reactions due to the transfusion of contaminated PCs led to an examination of whether the total bacteria levels after storage of pools containing a deliberately inoculated platelet unit would be significantly different than the levels in paired unpooled concentrates. STUDY DESIGN AND METHODS: A single PC was deliberately inoculated on Day 0 with one of three bacterial species (0.1–8.0 colony-forming units/mL). On Day 1, the deliberately inoculated PC was divided into three equal parts and either 1) pooled with 5 half-volume, ABO- and Rh-identical PCs; 2) similarly pooled and white cell reduced; or 3) kept as a control. Sterile connections were used during pooling; modified storage containers were used to ensure the correct surface-to-volume ratio of the single unit. RESULTS: Between Day 2 and Day 5 of storage, in 26 of 36 paired samples, nonfiltered pools containing Escherichia coli had greater total numbers of bacteria than did the paired single PCs. Day 2 pools had total bacteria levels approximately five times higher (colony-forming units/mL × container volume) than those in single units (p < 0.05). There was rapid growth of Staphylococcus aureus by Day 2 in pooled and unpooled PCs; by Day 3, total bacteria levels were approximately five times higher in pools than in single units (p < 0.05). Between Days 3 and 5 of storage, in 23 of 27 paired samples, nonfiltered pools containing S. aureus had greater total bacteria levels than the single PCs. By Day 5, 15 of 16 non-white-cell reduced pools had total levels of Staphylococcus epidermidis bacteria approximately five times those in the paired single PCs. Greater total bacteria levels in pooled units than in single units generally occurred when bacteria in pools reached the stationary phase of growth (when bacteria concentration became constant), and they were well correlated with the sixfold volume of pooled units. White cell reduction did not substantially affect the time required to attain stationary phase. CONCLUSION: The potential during storage for greater total bacteria levels in pools than in single PCs is a consequence of the greater volume of the pool.     

Side-Effects After IPL Photodepilation

BACKGROUND: Intense pulsed light (IPL) has been successfully used as an efficient hair removal system; however, possible side-effects have been not specifically addressed in the literature. OBJECTIVE: To assess all possible side-effects after IPL hair removal in a series of 49 females with facial hirsutism during a total of 390 treatment sessions of IPL photodepilation. METHODS: Immediate post-treatment clinical, photography evaluation, and a two-month post-treatment questionnaire were done in 49 females with facial hirsutism submitted to photodepilation with an IPL source (EpiLight trade mark, ESC, Israel). RESULTS: Side-effects observed were: transient erythema (n = 30), late evanescent erythema (n = 3), mild pain (n = 43), moderate pain (n = 6), crust formation (n = 9), superficial burning (n = 1), isolated vesicles (n = 3), transient hyperpigmentation (n = 8), transient hypopigmentation (n = 1), paradoxical effect (n = 5), persistent local heat sensation (n = 1), and minimal scar (n = 1). CONCLUSION: Even though common, most side-effects secondary to IPL photodepilation are mild and transient. Permanent side-effects such as scars are unlikely but they may occur. Growth of new, fine and dark hair may be seen in untreated areas in close proximity to the treatment area, especially in the neck, a side-effect that is reported for the first time in the literature.     

核素肺灌注显像对小儿先天性心脏病肺动脉高压肺血管病变的评价

核素肺灌注显像对小儿先天性心脏病肺动脉高压肺血管病变的评价田月琴史蓉芳余坚芳王道宇潘世伟阮英茆刘秀杰附表各组肺灌注显像各项参数对比组别例数年龄（岁）ＬＲＣＲＵＬＲ左肺右肺肾显像阳性对照组轻中度病变组重度病变组６１２１１５５±２３４１±２０５?..