Objective To investigate the clinical application and effecacy of ulnar-sided thumb ladder progressive flap in repairing grade Ⅰ and Ⅱ thumb oblique wound.Methods Between October 2009 and October 2013,ulnar-sided thumb ladder progressive flap with double blood supply was transferred to cover the grade Ⅰ and Ⅱ thumb oblique wound in 24 patients (12 males and 12 females).Mean age was 30 years (range,10-40 years).Mechanism of injury was machine twist injury in 8 patients,machine cutting injury in 6,sharp weapon injury in 6 and injury by a heavy object in 4.Twelve patients had grade Ⅰ defect and twelve patients grade Ⅱ defect.Flap ranged in size from 1.0 cm ×3.0 cm to 1.5 cm × 3.5 cm.Flap advancement distance was 1.5-2.5 cm.Donor area was sutured directly.Wound healing and color,swelling and temperature of the flap were observed after operation.Finger function was assessed with the upper limb function evaluation standard set up by hand surgery branch of Chinese Medical Association.Results Postoperatively,the flap survived and the fingertip had protective sensation.The wound healed by first intention.Two patients developed distal interphalangeal joint stiffness,and restored to normal after rehabilitation.All patients were followed up for 1-36 months (mean,20 months).Finger color,temperature,texture and pulp were restored.Finger function recovered satisfactorily,with excellent results in 22 patients and good results in 2 patients.Conclusion The procedure is easy and reliable,has affirmative effect and achieves maximal preservation of finger length and function.

Objective To investigate the clinical effect of inherent artery dorsal perforator flaps of thumb radial palmar for coverage of ipsilateral thumb degree Ⅰ and Ⅱ oblique defect of the fingertip.Methods The study included 6 males and 6 females,aged 10-40 years (mean 30 years),with thumb fingertip Ⅰ and Ⅱ degree defect treated between October 2009 and October 2013.Injury resulted from machinery cutting injury in 4 patients,machinery twist injury in 3,cutting by sharp weapons in 3,and crush injury in 2.There were 6 patients with Ⅰ degree defect and 6 patients with Ⅱ degree defect.Defects were all oblique involving more in the radial side rather than in the ulnar side and reconstructed with thumb radial palmar artery perforator flaps.Flap ranged in size from 1 cm × 2 cm to 1.5 cm × 2.5 cm.Donor site was covered with skin grafts.Observation indexes were wound healing condition,flap color,flap swelling degree,and flap temperature.Finger function was measured with upper extremity scoring system formulated by Hand Surgery Branch of Chinese Medical Association.Results All flaps survived and achieved good protective sensation.Wound healed primarily.Color,temperature and texture of the flap returned to almost normal.Moderate swelling of the flaps was detected and subsided around one week.All patients were followed up for 1-36 months (mean 20 months).At the final follow-up,two-point discrimination was 9-11 mm (mean 10 mm).Two patients developed distal interphalangeal joint stiffness and recovered after rehabilitation exercise.Finger function was rated as excellent in 10 cases and good in 2.Conclusion Inherent artery dorsal perforator flap of thumb radial palmar has affirmative effect and allows maximum preservation of finger length and function when applied to repair ipsilateral thumb degree Ⅰ and Ⅱ oblique defect of the fingertip.

OBJECTIVEFatal familial insomnia (FFI) is an autosomal dominant prion disease characterized clinically by inattention, sleep loss, dysautonomia, and motor signs. This study is aimed to investigate clinical and familial characteristics of ten Chinese Patients with FFI.

METHODSWe identified ten FFI cases from the surveillance network for Creutafeldt-Jakob disease (CJD) in China. Final diagnosis of FFI cases was made in accordance with the WHO criteria for CJD. The main clinical features and family histories of these ten FFI cases were analyzed.

RESULTSThe median age of ten cases at onset was 38 years (from 19 to 55). The foremost symptoms seemed to be various, including sleep disturbances, vision disorder, dizziness and anorexia. Sleep disturbances appeared in all cases and lasted in the whole clinical courses. Progressive sympathetic symptoms, memory loss, movement disturbances, myoclonus and hypertension were also frequently observed. The median duration of the disease was 9.5 months. EEG and MRI did not figure out special abnormality. 14-3-3 protein in CSF was positive in five out of eight tested patients. Clear family histories were identified in 8 patients.

CONCLUSIONThe data from our study confirm that the Chinese FFI cases have similar clinical characteristics as that of the Caucasian cases. Compared with other genetic CJD associated mutations, the genetic frequencies of D178N in PRNP are apparently high among the Chinese cases.

Adult ; Asian Continental Ancestry Group ; Female ; Humans ; Insomnia, Fatal Familial ; pathology ; physiopathology ; Male ; Middle Aged ; Young Adult

The two injuries of fluid and qi are the pathological basis of atrophy syndrome,and also the result of the imbalance of"yang transforming into qi,yin forming shape".Deficiency of yang transforming into qi,imbalance of yin forming shape would result in dysfunction of visceral functions,internal generation of water,phlegm,and blood stasis,and loss of nourishment in limbs and nine orifices,and then later result in atrophy syndrome.Based on the theory of"yang transforming into qi,yin forming shape",this article discussed Professor Gao Ying's treatment of myasthenia gravis with insufficient yang transforming into qi by using the method of promoting yang transforming into qi;the method of tonifying the kidney and regulating the shape could be used to treat multiple sclerosis with imbalance in yin forming shape;yin-yang tonifying method could be used to treat motor neuron disease with both yin and yang damaged.In the treatment of atrophy syndrome,it is necessary to examine the syndrome and seek the cause,distinguish between yin and yang,adjust biases,and provide TCM treatment in the early stages of the disease to delay the progression of the disease and improve prognosis.

OBJECTIVETo break immune tolerance to prion (PrP) proteins using DNA vaccines.

METHODSFour different human prion DNA vaccine candidates were constructed based on the pcDNA3.1 vector: PrP-WT expressing wild-type PrP, Ubiq-PrP expressing PrP fused to ubiquitin, PrP-LII expressing PrP fused to the lysosomal integral membrane protein type II lysosome-targeting signal, and PrP-ER expressing PrP locating the ER. Using a prime-boost strategy, three-doses of DNA vaccine were injected intramuscularly into Balb/c mice, followed by two doses of PrP protein. Two weeks after the last immunization, sera and spleens were collected and PrP-specific humoral and cellular immune responses evaluated by ELISA and ELISPOT tests.

RESULTSHigher levels of serum PrP antibodies were detected in mice vaccinated using the strategy of DNA priming followed by protein boosting. Of these, WT-PrP, Ubiq-PrP, and PrP-LII induced significantly higher humoral responses. ELISPOT tests showed markedly increased numbers of IFN-γ-secreting T cells in mice vaccinated using the strategy of DNA priming followed by protein boosting after stimulation with recombinant PrP23-90 and PrP23-231. PrP-ER induced the strongest T-cell response.

CONCLUSIONPrion vaccines can break tolerance to PrP proteins and induce PrP-specific humoral and cellular immune responses.

Animals ; Antibodies ; immunology ; CHO Cells ; COS Cells ; Cercopithecus aethiops ; Cricetinae ; Cricetulus ; Enzyme-Linked Immunosorbent Assay ; Female ; HeLa Cells ; Humans ; Immune Tolerance ; Interferon-gamma ; immunology ; Lysosomal-Associated Membrane Protein 2 ; genetics ; immunology ; Mice ; Mice, Inbred BALB C ; Peptide Fragments ; immunology ; Prions ; genetics ; immunology ; Receptors, Peptide ; genetics ; immunology ; Recombinant Fusion Proteins ; genetics ; immunology ; Recombinant Proteins ; immunology ; Transfection ; Ubiquitin ; genetics ; immunology ; Vaccines, DNA

Objective: To search for biomarkers for human familial prion disease. Methods: Two-dimensional differential gel electrophoresis (2D-DIGE) proteomic analysis has been performed in frontal lobe tissues of 3 patients suffering from human familial prion disease (PrP) and 3 age-and sex-matched patients suffering from sudden death due to heart failure without neurological disease. Results: The maps revealed 14 polypeptide chains differentially modulated in the PrP samples, among those, 7 could be identified upon digestion and MALDI-TOF/MS analysis, of which 6 appeared to be up-regulated, 1 being down-regulated. Conclusions We highlight Galectin-1(Gal-1), ryanodine receptor 2 (RyR2), ubiquitin, Rab-interacting lysosomes protein-like protein 1 (RILPL-1) profillin 2 (PFN2), in the differential map. These proteins are related to neurogenesis, the clearance of misfolded proteins, stasis of calium channel, myoclonus and so on. These proteins are potential biomarkers or targets for treatment of prion disease.