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Background Chronic granulomatous disease (CGD) is a rare disease in China, and very little large‐scale studies have been conducted to date. We aimed to investigate the clinical and genetic features of CGD in Chinese pediatric patients. Methods Pediatric patients with CGD from Beijing Children's Hospital, Capital Medical University, China, were enrolled from January 2006 to December 2016. Results A total of 159 pediatric patients with CGD were enrolled. The median age of clinical onset was 1.4 months, and 73% (116/159) had clinical onset symptoms before the 1 year of age. The most common site of invasion was the lungs. The lymph nodes, liver, and skin were more frequently invaded in X‐linked (XL) CGD patients than in autosomal recessive (AR) CGD patients (P

Local activated macrophages derived from infiltrating monocytes play an important role in the damage and repair process of spinal cord injury (SCI). The present study investigates the dynamic change of classically activated proinflammatory (M1) and alternatively activated anti-inflammatory (M2) cells in a rat model with contusive SCI by flow cytometry (FCM) and immunohistochemistry. The macrophage subsets were immunophenotyped by using antibodies against cluster of differentiation (CD)-68, C-C chemokine receptor type 7 (CCR7), CD163, and arginase 1 (Arg1). The CD68(+) CD163(-) and CD68(+) CCR7(+) cells were determined to be M1 subsets, whereas the CD68(+) CD163(+) and CD68(+) Arg1(+) cell subpopulations represented M2 cells. The subsets of macrophages in the injured spinal cord at 1, 3, 5, 7, 14, and 28 days postinjury (dpi) were examined. In the sham-opened spinal cord, few M1 or M2 cells were found. After SCI, the phenotypes of both M1 and M2 cells were rapidly induced. However, M1 cells were detected and maintained at a high level for up to 28 dpi (the longest time evaluated in this study). In contrast, M2 cells were transiently detected at high levels before 7 dpi and returned to preinjury levels at 14 dpi. These results indicate that M1 cell response is rapidly induced and sustained, whereas M2 induction is transient after SCI in rat. Increasing the fraction of M2 cells and prolonging their residence time in the injured local microenvironment is a promising strategy for the repair of SCI.

Amorphous Cr2O3 materials with high surface areas were prepared via a precipitation process with CrCl3 · 6H2O as the raw material, (NH4)2CO3, NH3 · H2O, and KOH as precipitating agents. Calcination of Cr(OH)3 precipitation at low temperatures (300–400 °C) resulted in amorphous Cr2O3 materials with high surface areas, while high-temperature calcination (500–600 °C) led to crystalline Cr2O3. The hydrogen-adsorption phenomenon was clearly observed in a hydrogen atmosphere on the amorphous Cr2O3 materials with high surface area. The adsorbed hydrogen desorbed at about 550 °C due to the phase transition from amorphous to crystalline. However, this phenomenon was not observed for crystalline Cr2O3.

Changes of different chromium oxide species [Cr(VI), Cr(V) and Cr(III)] were investigated using in situ Raman spectroscopy in an anhydrous hydrogen fluoride (AHF) atmosphere. It was found that the Cr(VI) (CrO3) and Cr(V) (YCrO4) species were easy to fluorinate, and generated CrO2F2 or/and CrOF3, while the Cr(III) (Cr2O3) species was difficult to fluorinate. Moreover, the CrO2F2 and CrOF3 species were stable in the AHF atmosphere, but they easily transform into CrO3 in N2 atmosphere and further decomposed to Cr2O3 at 400 °C. Moreover, Cl/F exchange reaction of CF3CH2Cl to CF3CH2F over these Cr species indicated that the high-oxidation-state CrOxFy (CrO2F2 and CrOF3) species exhibited much higher reactivities than Cr2O3, suggesting that these species (CrO2F2 and CrOF3) may be the active phases of the reaction. Copyright © 2010 John Wiley & Sons, Ltd.

Mixed crystals of K1-x(NH4)xH2PO4(KADP) were grown from KDP (KH2PO4) dominated mixed solutions with varying molar proportion of ADP (NH4H2PO4) addition. It was found that, as the increase of ADP molar concentration, the growth rate along z-axis of KADP crystal decreased rapidly. The structure of KADP crystals was investigated by powder XRD and the lattice parameter was calculated. The results showed that the lattice parameter c of KADP crystal increased with the molar concentration of ADP. The optical homogeneity of grown KADP crystals was determined with a differential phase-shifting interferometry. Frequency dependences of the dielectric constant and dielectric loss of KADP crystals were measured at room temperature (290 K). The dielectric constants of KADP crystals were almost invariant with the increase of frequency. In the region of 102∼104Hz, the values of the dielectric loss reduced with the increase of frequency. The piezo-resonance coupling effect still exists in KADP crystals at room temperature, but shifted to low frequency band. (© 2010 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim)

Two novel metal–organic coordination polymers, [Cd(bqdc)(phen)2]n (1), [Cd(bqdc)(phen)]n (2), (H2bqdc = 2,2′-biquinoline-4,4′-dicarboxylic acid, phen = 1,10-phenanthroline) were obtained with different metal/ligand ratios through hydrothermal method. Their structures were determined by single-crystal X-ray diffraction. In two complexes, central Cd2+ ions and (bqdc)2− ligands display completely different coordination modes. 1 is a 1-D zigzag chain, two phen ligands chelate to one cadmium atom, which is rare in similar polymers. 2 is a 3-D framework, exhibits an unusual eight-coordinate structure (CdN2O6) and the coordination polyhedron can be described as distorted monocapped pentagonal bipyramid. 1 and 2 are stable in air, and show blue photoluminescence at room temperature.

In order to provide some useful data for studies on radiative and chemical processes in the stratosphere, the vertical distribution of trace gases (NO, NO2, HCl, HF, CH4, H2O and O3) and their variation trends in the stratosphere over China have been analyzed by using the HALOE data from 1992 to 2005. Besides the mixing ratio of the traces averaged over China, the values averaged over Qinghai-Xizang Plateau are also given. The analyses show that the mixing ratios for various trace gases distribute vertically in different patterns in stratosphere, the distribution and seasonal variation of the traces near tropopause are much different from those in stratosphere, The analyses also show that the seasonal variation, the quasi-biennial oscillation (QBO) signals and the long-term trends are clear for all the traces, and the trends of the traces gases in various levels of stratosphere are different. In middle stratosphere, the mixing ratios of NO, NO2, HCl and H2O are all increased before 1998 and then decreased after 1998, while it is contrary for mixing ratio of O3 which decreased evidently before 1998 and the decrease trend is not significant after 1998. That means the decrease of those traces lead to the ozone depletion retarded in middle stratosphere in recent years. While in the lower stratosphere, the ozone depletion is still significant.

Objectives: To determine the heritability of BMI and to examine the mode of inheritance of BMI variation in Chinese. Research Methods and Procedures: Familial correlation and complex segregation analyses for BMI were undertaken in a Chinese sample composed of 392 nuclear families, with 1190 total individuals. Results: A moderate heritability was found for BMI (h2 = 0.419-0.492). The obtained results do not support a major gene for BMI in our samples. BMI may be inherited in a complex and non-Mendelian manner in Chinese. Discussion: The findings of this study suggest that identification of specific genes for BMI in Chinese, at least within the same data set, is a serious challenge because of the lack of evidence of a major gene for BMI in our Chinese sample.

PBD is an important determinant of osteoporotic fractures. Few studies were performed to search for genes underlying PBD variation in Chinese populations. We tested linkage and/or association of the estrogen receptor alpha gene polymorphism with PBD in 401 Chinese nuclear families. This study suggests the ER-alpha gene may have some minor effects on PBM variation in the Chinese population. Low peak bone density (PBD) in adulthood is an important determinant of osteoporotic fractures in the elderly. PBD variation is mainly regulated by genetic factors. Extensive molecular genetics studies have been performed to search for genes underlying PBD variation, largely in whites. Few studies were performed in Chinese populations. In this study, we simultaneously test linkage and/or association of the estrogen receptor alpha (ER-alpha) gene polymorphism with PBD in 401 Chinese nuclear families (both parents plus their female children) of 1260 subjects, with the 458 children generally between 20 and 40 years of age. All the subjects were genotyped by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) at polymorphic PvuII and XbaI sites inside the ER-alpha gene. Bone mineral density was measured at the lumbar spine (L1-L4) and hip (femoral neck, trochanter, and intertrochanteric region). Raw bone mineral density values were adjusted by age, height, and weight as covariates. We detected marginally significant results for within-family association (transmission disequilibrium; p = 0.054) between the spine bone mineral density variation and the ER-alpha XbaI genotypes. For the hip bone mineral density variation, significant (p < 0.05) linkage results were generally found for the two intragenic markers. Analyses of the haplotypes defined by the two markers confer further evidence for linkage of the ER-alpha with the hip PBD variation. In conclusion, this study suggests that the ER-alpha gene may have minor effects on PBD variation in our Chinese population.

Osteoporosis is a major public health problem defined as a loss of bone strength, of which bone size is an important determinant. In the present study, familial correlation and segregation analyses for the spine and hip bone sizes were performed for the first time in a Chinese sample composed of 393 nuclear families with a total of 1,193 individuals. The results indicate a major gene of codominant inheritance for spine bone size; however, there is no evidence of a major gene influencing hip bone size. Significant familial residual effects are found for both traits, suggesting their polygenic inheritance. Heritability estimates (±SE) for spine and hip bone size were 0.62 (0.13) and 0.59 (0.12), respectively. Sex and age differences in genotype-specific average bone size were observed. Compared with our previous study on bone mineral density (BMD) in the same population, this study suggests that genetic determination of bone size may be different from that of BMD, and thus studying bone size as one surrogate phenotype for osteoporotic fractures may be necessary. Am. J. Hum. Biol. 16:68–77, 2004. © 2003 Wiley-Liss, Inc.