243例健康人血红细胞悬浮液射频介电特性的研究

本文应用同轴传输线反射法建立的细胞悬浮液介电测量系统，测量了２４３例健康人（男１２０，女１２３名）血红细胞悬浮液在１～５００ＭＨｚ频率范围的介电常数和电导率。结果表明不同性别和不同血型之间的红细胞悬浮液的介电参数无显著差异。将１９～８０岁之间的受试者按１０岁年龄间隔分成六个组，探讨年龄差别对介电参数的影响；发现５０岁左右是人红细胞介电参数有显著差异的临界年龄，４９岁后三个高年龄组的介电参数显著地低于４９岁前的三个低年龄组的数值（Ｐ＜０．０５）。本文的结果证明年龄对人血红细胞的介电参数有明确的影响。     

Quantitative Correlation between Steric Defects and Thermal Behavior in Highly Syndiotactic Polystyrene: A Study Based on DSC and 13C NMR Spectroscopy

Several syndiotactic polystyrene (sPS) samples have been synthesized by using different catalytic systems. Their stereochemistry has been determined by ¹³C NMR spectra in both the aliphatic CH₂ and aromatic C₁ resonance regions. The observed peaks have been unambiguously assigned to specific hexads and heptads, respectively, and their intensities have been used to draw the percent of defects (meso dyads) in the polymer chains. On the hypothesis that chain defects are at the origin of chain folding and thus determine the thickness of crystalline lamellae, we performed differential scanning calorimetry (DSC) analysis on the same samples, and their thermal parameters were measured. A model was developed to determine the amount of steric defects from the DSC melting‐peak profiles, and the results obtained were compared with the NMR results. A satisfactory agreement was found (correlation factor 0.96) in the explored range of defect concentrations (up to 2.5% of meso dyads). The possible influence of the extraction procedure of the amorphous fraction was found to be negligible. Thus, information on stereochemistry can be obtained from DSC experiments starting from as‐prepared (not extracted) samples.

     

A patient with novel MBOAT7 variant: The cerebellar atrophy is progressive and displays a peculiar neurometabolic profile

Mutations in the MBOAT7 gene have been described in 43 patients, belonging to 18 families, showing nonspecific clinical features (intellectual disability [ID], seizures, microcephaly or macrocephaly, and mild to moderate cerebellar atrophy) that make the clinical diagnosis difficult. Here we report the first Italian patient, a 22.5‐year‐old female, one of the oldest reported, born to apparently consanguineous parents. She shows severe ID, macrocephaly, seizures, aggressive outbursts, hyperphagia. We also documented progressive atrophy of the cerebellar vermis, that appeared not before the age of 7. The whole‐exome sequencing of the trio identified a novel homozygous variant c.1057_1058delGCinsCA (p.Ala353His) in the MBOAT7 gene. The variant is considered to be likely pathogenic, since it is absent from population database and it lies in a highly conserved amino acid residue. This disorder has a neurometabolic pathogenesis, implicating a phospholipid remodeling abnormalities. A brain hydrogen‐magnetic resonance spectroscopy (H‐MRS) examination in our patient disclosed a peculiar neurometabolic profile in the cerebellar hemispheric region. This new finding could address the clinical suspicion of MBOAT7‐related disorder, among the wide range of genetic conditions associated with ID and cerebellar atrophy. Moreover, the documented progression of cerebellar atrophy and the worsening of the disease only after some years open to the possibility of a therapeutic window after birth.     

Renpenning syndrome in an Indian patient

Renpenning syndrome is one of the well‐characterized causes of X‐linked intellectual disability and is associated with microcephaly and various visceral malformations. Face is considered characteristic but the dysmorphism is subtle. Here we report an Indian adult with a very lean habitus, progressive atrophy of the upper back muscles, microcephaly, loss of cervical lordosis, and upper thoracic scoliosis. Using whole‐exome sequencing, a hemizygous deletion was identified in PQBP1 that leads to a frameshift and premature termination of translation. The loss of normal curvatures of cervical and upper thoracic spine due to muscular atrophy is a characteristic feature, though it may be age dependent.     

壳聚糖与硫酸软骨素共混膜性质的研究

以壳聚糖和硫酸软骨素按一定比例制备出共混膜，研究了膜片的透光性、含水量、渗透性、力学性质、表面结构、生物降解性、生物相容性等性质。结果表明该共混膜具有较好的透光性、通透性、生物降解性和生物相容性，膜表面较粗糙。以此共混膜为载体培养兔角膜基质细胞，发现细胞在此共混膜上生长良好。制备膜片随着加入CaSO4量的增加，膜的通透性也随之增加。     

Tensile Tests of Collagen Fibers Obtained from the Rabbit Patellar Tendon

Tensile properties of collagen fibers of approximately 1 m in diameter were determined using a newly developed micro tensile test system for cells and fine fibrous biological tissues. The test system consists of a thermostatic test chamber, an inverted microscope, micromanipulators, a direct drive linear actuator, a cantilever-type load cell, and a video dimension analyzer (VDA). The fibers were isolated with a mechanical method from collagen fascicles (approximately 300 m in diameter) cut out from the rabbit patellar tendon. The ends of each fiber were attached to the tips of a pair of glass microtubes (15 to 20 m in outer diameter) using a cyanoacrylate adhesive. One of the microtubes was attached to the load cell; the other one was connected to the linear actuator which was utilized to stretch the fiber. Load applied to the fiber was measured with the load cell, while its elongation was determined with the VDA using the images of the edges of the adhesive as markers. Tangent modulus, tensile strength, and strain at failure of the tested fibers were 54.3± 25.1 MPa, 8.5± 2.6 MPa, and 21.6± 3.0%, respectively. These values were much different from those of collagen fascicles (300 m in diameter) cut out from the rabbit patellar tendon and also from those of the bulk patellar tendon (Trans. ASME, J. Biomech. Eng. 121, 124–294, 1999); for example, tensile strength and strain at failure of the fibers were approximately 50 and 200% of those of the fascicles, respectively. These results suggest that the mechanical interactions between fibers and between fibers and ground substances contribute much to the mechanical properties of collagen fascicles and bulk tendons.     

Determinants of pulmonary blood volume. Effects of acute changes in pulmonary vascular pressures and flow

To examine the effects of pulmonary vascular pressures and flow on pulmonary blood volume (PBV), experiments were performed at constant heart rate and zone 3 conditions (mean left atrial pressure (LAP) above airway pressure) in six anesthetized, open-chest dogs. PBV was calculated as the product of electromagnetic aortic flow and pulmonary mean transit time for ascorbate, obtained without blood withdrawal by polarographic recording of aortic ascorbate changes. In three series of experiments LAP was raised similarly in three steps, from 4.5 to 14.8 mmHg: by mitral constriction which reduced pulmonary blood flow, by blood volume expansion which more than doubled pulmonary blood flow, or by a combination of the two procedures which kept pulmonary blood flow constant. In all three series, LAP and mean pulmonary arterial pressure (PAP) rose in proportion, but PBV was better correlated to PAP (r=0.87±0.02) than to LAP (r=0.66±0.09). These experiments suggest that PAP is the most important factor in determining PBV under zone 3 conditions, whether PAP is raised by increasing pulmonary blood flow or by mitral constriction.     

Effect of a Polymeric Additive on the Pore‐Size Distribution and Shrinking Process of a Hydrogel Network

A systematic comparison of the effect of architectural modifications to the network structure on the internal microstructure of N‐isopropylacrylamide (NIPA) based hydrogels showed that the addition of a second component to the network significantly increased the proportion of macropores in the network. The second components considered were short poly(N‐isopropylacrylamide) (PNIPAM) chains grafted to the network backbone, high‐molecular‐weight polyacrylamide (PAM) chains, or microsphere particles of PNIPAM. Structures are proposed for each of the modified gel networks taking into account the new structural information. Through a combination of the pore size and network structure data reported here, and with the shrinking data obtained previously, shrinking mechanisms are proposed for each of the modified network structures. In all cases, the enhanced shrinking rates were directly caused by the presence of the second component, which acted as nuclei for shrinking (graft‐PNIPAM and PNIPAM microspheres) or as water‐release channels (PAM gel), and indirectly caused by the second components via their affect on the network microstructure.

Proposed structures for the architecturally modified gels based on the pore‐size information. Graft‐PNIPAM gel. The freely mobile graft chains prevent chains from meeting resulting in larger pores.     

桡动脉与冠状动脉几何性状和显微结构成份的比较

目的：通过对桡动脉与冠状动脉几何性状和显微结构成份的比较研究，为桡动脉应用于冠状动脉搭桥术提供依据。方法：非心脏病死亡的尸体，在其桡动脉的上端、中点、下端及冠状动脉主干根部，横断取材制作切片，光镜下观察并用图像分析仪对其显微结构成份进行定量研究。结果：桡动脉的内、外径与冠状动脉的前室间支、旋支、右冠状动脉接近，但它的内膜面积和管腔狭窄率均明显低于冠状动脉。桡动脉与冠状动脉的显微结构成份相似；管壁平滑肌的含量二者无显著差异。结论：桡动脉是冠脉搭桥术可供选择的较理想的移植血管。