Objective To develop a scale to assess Kennedy's disease (KD) based on patients' clinical characteristics and to validate its application in patients.Methods We developed KD1234 Scale following the model of Amyotrophic Lateral Sclerosis-Functional Rating Scale.Using this new scale,we assessed patients with genetic diagnosis of KD and evaluated the reliability and validity of the new scale.Reliability was analyzed using Cronbach' s α coefficient and split-half reliability.Validity was analyzed by content validity and structure validity.Results The Cronbach' s α coefficient of the total scale was 0.789 and split-half reliability coefficient was 0.868.The α of breathing with total score was 0.3-0.4,α of written,language and swallowing with the total score was 0.4-0.5,α of other items with the total score was ＞ 0.5 (P ＜ 0.01 in each of above α).Four common factors extracted by exploratory factor analysis had well correspondence between the scale construction and the theoretical construction.Factor loading was ranged from 0.541 to 0.864 for each item.The duration of the disease showed some correlation with the score of KD1234 Scale.Conclusions The KD1234 Scale demonstrates high reliability and validity.The duration of the disease indicates negative correlation with KD1234 Scale scores.The KD1234 Scale can be used in clinical research to evaluate the condition of KD patients quantitatively.

Objective To explore the significance of serum myoglobin detection in the diagnosis and evaluation of Kennedy`s disease (KD).Methods The level of serum myoglobin (Myo) was detected in 60 KD patients and 30 amyotrophic lateral sclerosis (ALS) patients.Results The serum Myo level and abnormal rate in KD group were significantly higher than those in ALS group (all P<0.001).The serum Myo level was positively related with the disease course of KD (r=0.665,P<0.01).There was no significant difference of serum Myo level and disease course of ALS (r=-0.047,P>0.05).There was no overlap of serum Myo level between the two groups.There was no significant difference of serum Myo level and CAG repeated number of ALS (r=-0.193,P>0.05).Conclusion Myo is likely as an easy and sensitive biomarker, used to identify the KD and special type of ALS, and used in the evaluation of the KD condition in the future.

OBJECTIVETo explore the curative effect of surgery and selerotherapy for massive venous malformations of the tongue.

METHODSFrom January 2005 to December 2014, subtotal resection or debulking for 15 cases of massive venous malformation in the tongue was undertaken with multiple sessions of pre- and post-operative injection therapy of pingyangmycin, lauromacrogol and absolute ethanol.

RESULTSAll signs associated with the lesions including eating, sleep and speech disorders disappeared after treatment. Complete or near complete resolution was achieved in 9 cases, and a significant reduction in size in a further 6 cases after surgical excision and peri-operative sclerotherapy.

CONCLUSIONSFor massive venous malformations of the tongue, surgical excision combined with multiple sessions of sclerotherapy is a good treatment option.

Bleomycin ; analogs & derivatives ; therapeutic use ; Combined Modality Therapy ; methods ; Ethanol ; therapeutic use ; Humans ; Injections, Intralesional ; Polyethylene Glycols ; therapeutic use ; Sclerosing Solutions ; therapeutic use ; Sclerotherapy ; Tongue ; blood supply ; Treatment Outcome ; Vascular Malformations ; therapy ; Veins ; abnormalities

OBJECTIVETo investigate the clinical effect of Halo-vest head ring in the treatment of replantation of total scalp avulsion.

METHODSWe treated 11 cases of total scalp avulsion with the anastomosis of arteriovenous vessels and Halo-vest head ring from December 2006 to February 2015.

RESULTSOne patient's replanted scalp got necrosis because of serious contusion which was healed without hair growth after free skin graft and dressing. All the scalp flaps in the other 10 patients survived. After 3-96 months follow-up, the wound completely healed, the scalp and hair grew well with satisfactory appearance.

CONCLUSIONSThe use of Halo-vest head ring for replantation of total scalp avulsion can effectively improve the survival rate and survival area.

Anastomosis, Surgical ; methods ; Bandages ; Graft Survival ; Humans ; Lacerations ; surgery ; Necrosis ; etiology ; Prostheses and Implants ; Replantation ; methods ; Scalp ; injuries ; pathology ; surgery ; Skin Transplantation ; methods ; Surgical Flaps

Traumatic brain injury (TBI)-induced coagulopathy has increasingly been recognized as a significant risk factor for poor outcomes, but the pathogenesis remains poorly understood. In this study, we aimed to investigate the causal role of acrolein, a typical lipid peroxidation product, in TBI-induced coagulopathy, and further explore the underlying molecular mechanisms. We found that the level of plasma acrolein in TBI patients suffering from coagulopathy was higher than that in those without coagulopathy. Using a controlled cortical impact mouse model, we demonstrated that the acrolein scavenger phenelzine prevented TBI-induced coagulopathy and recombinant ADAMTS-13 prevented acrolein-induced coagulopathy by cleaving von Willebrand factor (VWF). Our results showed that acrolein may contribute to an early hypercoagulable state after TBI by regulating VWF secretion. mRNA sequencing (mRNA-seq) and transcriptome analysis indicated that acrolein over-activated autophagy, and subsequent experiments revealed that acrolein activated autophagy partly by regulating the Akt/mTOR pathway. In addition, we demonstrated that acrolein was produced in the perilesional cortex, affected endothelial cell integrity, and disrupted the blood-brain barrier. In conclusion, in this study we uncovered a novel pro-coagulant effect of acrolein that may contribute to TBI-induced coagulopathy and vascular leakage, providing an alternative therapeutic target.

Traumatic brain injury (TBI) triggers the activation of the endogenous coagulation mechanism, and a large amount of thrombin is released to curb uncontrollable bleeding through thrombin receptors, also known as protease-activated receptors (PARs). However, thrombin is one of the most critical factors in secondary brain injury. Thus, the PARs may be effective targets against hemorrhagic brain injury. Since the PAR1 antagonist has an increased bleeding risk in clinical practice, PAR4 blockade has been suggested as a more promising treatment. Here, we explored the expression pattern of PAR4 in the brain of mice after TBI, and explored the effect and possible mechanism of BMS-986120 (BMS), a novel selective and reversible PAR4 antagonist on secondary brain injury. Treatment with BMS protected against TBI in mice. mRNA-seq analysis, Western blot, and qRT-PCR verification in vitro showed that BMS significantly inhibited thrombin-induced inflammation in astrocytes, and suggested that the Tab2/ERK/NF-κB signaling pathway plays a key role in this process. Our findings provide reliable evidence that blocking PAR4 is a safe and effective intervention for TBI, and suggest that BMS has a potential clinical application in the management of TBI.

Clinical advances in the treatment of intracranial hemorrhage (ICH) are restricted by the incomplete understanding of the molecular mechanisms contributing to secondary brain injury. Acrolein is a highly active unsaturated aldehyde which has been implicated in many nervous system diseases. Our results indicated a significant increase in the level of acrolein after ICH in mouse brain. In primary neurons, acrolein induced an increase in mitochondrial fragmentation, loss of mitochondrial membrane potential, generation of reactive oxidative species, and release of mitochondrial cytochrome c. Mechanistically, acrolein facilitated the translocation of dynamin-related protein1 (Drp1) from the cytoplasm onto the mitochondrial membrane and led to excessive mitochondrial fission. Further studies found that treatment with hydralazine (an acrolein scavenger) significantly reversed Drp1 translocation and the morphological damage of mitochondria after ICH. In parallel, the neural apoptosis, brain edema, and neurological functional deficits induced by ICH were also remarkably alleviated. In conclusion, our results identify acrolein as an important contributor to the secondary brain injury following ICH. Meanwhile, we uncovered a novel mechanism by which Drp1-mediated mitochondrial oxidative damage is involved in acrolein-induced brain injury.