Search

We report a case of genetic Creutzfeldt-Jakob disease (gCJD), which has a clinical phenotype that is highly similar to Fatal Family Insomnia (FFI) and has a triad of Wernicke-Korsakoff syndrome (WKs) at the developmental stage of the disease. The 51-year-old male complained of sleep disorder and imbalance who had visited five different hospitals before diagnosed. A neurological examination revealed a triad of symptoms characteristic for WKs such as gaze paresis, ataxia of limbs and trunk, and memory disturbances. The disturbances increased during the course of the disease, which led to the death of the patient 18 months after the appearance of the signs. Although the patient show negative in brain magnetic resonance imaging (MRI) and 14-3-3 protein of cerebrospinal fluid (CSF), he was finally diagnosed with gCJD disease by the human prion protein (PRNP) gene mutations.

Effect of lignin preoxidation on subsequent radical-mediated pyrolysis was discussed in this study. Technical hot-water-extracted lignin was preoxidized by 2,3-dichloro-5,6-dicyano-1,4-benzoquinone in diverse degrees and pyrolyzed under different temperatures. Characterizations indicated that preoxidation increased lignin oxygen contents and converted α-hydroxyls to α-carbonyls. These structural modifications caused by preoxidation reduced the thermal stability and pyrolysis reactivity of lignin, shifting lignin thermal decomposition to the low temperature region and inhibiting lignin pyrolysis into bio-oil fractions. However, recognition of species and yields of specific compounds via analytical pyrolysis declared that although preoxidation reduced product yields, it did not alter the reaction pathways. The fixed bed experiments proved the above findings and gave the gas compositions, mainly CO

Whether lignin benzyl hydroxyl shielding could promote its pyrolysis to phenolic compounds was investigated in this paper. Lignin benzyl hydroxyl was first preoxidized by 2,3-dichloro-5,6-dicyano-1,4-benzoquinone and stabilized by propionaldehyde respectively, then pyrolysis was conducted with milled wood lignin as a control. Organic stable radicals in pyrolytic chars were further detected to reveal lignin pyrolysis chemistry. Results showed that benzyl hydroxyl shielding process weakened lignin thermal stability, and decreased liquid yields regardless of the frequency of lignin β-O-4 linkages. In addition, char yield grew after benzyl hydroxyl shielding. Radical concentration was inversely proportional to β-O-4 content which indicated the non-negligible impact of shielded benzyl hydroxyl on lignin pyrolysis. Furthermore, gases from propionaldehyde stabilized lignin quenched its radicals. This work confirmed that lignin β-O-4 linkages and shielded benzyl hydroxyl both played the great role in radical-mediated pyrolysis, but the enhancement of liquid products could not be achieved via benzyl hydroxyl shielding.

To investigate the value of cementless total hip arthroplasty (THA) for the treatment of Crowe type Ⅲ developmental dysplasia of hip (DDH) in adults.Between September 2013 and September 2015, 50 patients (51 hips) with Crowe type Ⅲ DDH were treated. There were 20 males (20 hips) and 30 females (31 hips), with the average age of 39 years (range, 19-55 years). The left side was involved in 34 cases, the right side in 15 cases, and both sides in 1 case. All patients had the symptoms of limp walking and hip pain. The disease duration was 10-47 months (mean, 26 months). The sign of "4" number test and Trendenleburg sign were positive; the Harris score was 38.9±7.1. The bilateral lower extremities discrepancy was 2.5-4.0 cm (mean, 3.3 cm) before operation. All the patients underwent cementless THA, and acetabulum by structural femoral head autograft was performed in 28 cases (28 hips).After operation, the incision healed by first intention. Only 2 patients (2 hips) had femoral nerve palsy, and 7 patients (7 hips) had leg swelling, which were cured after symptomatic treatment. All the patients were followed up 6-18 months (mean, 10 months). The sign of limp walking was improved after operation, hip pain was relieved in 46 patients (46 hips) and only 4 patients (5 hips) still had mild pain. The X-ray films showed bony healing at 3-6 months (mean, 5 months) after operation. At last follow-up, the patients had equal limb length with the discrepancy less than 1 cm (mean, 0.4 cm). At last follow-up, the Harris score was significantly increased to 91.2±2.8 (The cementless THA is an effective method to treat Crowe type Ⅲ DDH in adults.探讨生物型人工全髋关节置换术（total hip arthroplasty，THA）治疗Crowe Ⅲ型成人髋关节发育不良（developmental dysplasia of hip，DDH）的疗效。.2013年9月-2015年9月，收治50例（51髋）Crowe Ⅲ型DDH患者。男20例（20髋），女30例（31髋）；年龄19～55岁，平均39岁。左髋34例、右髋15例，双髋1例。患者均伴明显跛行步态，患侧髋关节疼痛明显。临床症状出现至入院就诊时间为10～47个月，平均26个月。患髋“4”字征、Trendenleburg征均呈阳性，髋关节Harris评分为（38.9±7.1）分。单髋患者双下肢长度相差2.5～4.0 cm，平均3.3 cm。患者均采用生物型假体行THA，其中28例（28髋）行自体股骨头结构性植骨。.术后患者切口均Ⅰ期愈合。2例（2髋）出现股神经牵拉麻木疼痛，7例（7髋）出现患肢肿胀，均对症处理后症状消失。50例均获随访，随访时间6～18个月，平均10个月。患者跛行步态均明显改善；4例（5髋）髋关节疼痛较术前明显缓解，46例（46髋）髋关节无疼痛。X线片复查示，髋关节外上方结构性植骨均在术后3～6个月达骨性愈合，平均5个月；随访期间假体均无松动、下沉；末次随访时单髋患者双下肢长度相差0～1 cm，平均0.4 cm，视为双下肢等长。末次随访，髋关节Harris评分为（91.2±2.8）分，与术前比较差异有统计学意义（对于Crowe Ⅲ型成人DDH患者，采用生物型THA治疗可获得较好疗效。.

The new tunable filter technology is used here to build full band (60 MHz) 9 pole dielectric resonator (DR) filters tuned at two different ranges separated by 200 MHz, PCS band (1930-1990 MHz) and UMTS band (2110-2170 MHz). All the measurements are taken in the same housing and the same resonators. The double conical resonators when put in proximity, provide the required strong couplings for the full band filter. The gap between the two conical parts that make the resonator, regulates the frequency over a very wide range without affecting the quality factor and helps provide a safe spurious free band. The fine tuning of the frequency is achieved with metal tuning plugs. Two different dielectric constants are used epsivr=35 and epsivr=45. In both cases, a typical 0.4 dB insertion loss is achievable for the 9 pole filter. An optimization of the design can push it very well near 0.3 dB for Er=35 or even lower. The design is patent pending and to our knowledge this full band DR filter tuned over such a wide range is presented for the first time.