Onset, progression, and plateau of skeletal lesions in fibrous dysplasia and the relationship to functional outcome.

Most lesions in FD and their attendant functional disability occur within the first decade; 90% of lesions are present by 15 years, and the median age when assistive devices are needed is 7 years. These findings have implications for prognosis and determining the timing and type of therapy. INTRODUCTION: Fibrous dysplasia of bone (FD) is an uncommon skeletal disorder in which normal bone is replaced by abnormal fibro-osseous tissue. Variable amounts of skeletal involvement and disability occur. The age at which lesions are established, the pace at which the disease progresses, if (or when) the disease plateaus, and how these parameters relate to the onset of disability are unknown. To answer these questions, we performed a retrospective analysis of a group of subjects with FD. MATERIALS AND METHODS: One hundred nine subjects with a spectrum of FD were studied for up to 32 years. Disease progression was assessed in serial (99)Tc-MDP bone scans by determining the location and extent of FD lesions using a validated bone scan scoring tool. Physical function and the need for ambulatory aids were assessed. RESULTS: Ninety percent of the total body disease skeletal burden was established by age 15. Disease was established in a region-specific pattern; in the craniofacial region, 90% of the lesions were present by 3.4 yr, in the extremities, 90% were present by 13.7 yr, and in the axial skeleton, 90% were present by 15.5 yr. Twenty-five of 103 subjects eventually needed ambulatory aids. The median age at which assistance was needed was 7 yr (range, 1-43 yr). The median bone scan score for subjects needing assistance was 64.3 (range, 18.6-75) compared with 23.1 (range, 0.5-63.5) in the unassisted subjects (p < 0.0001). Among subjects needing assistance with ambulation, 92% showed this need by 17 yr. CONCLUSIONS: The majority of skeletal lesions and the associated functional disability occur within the first decade of life. The implication is that the window of time for preventative therapies is narrow. Likewise, therapeutic interventions must be tailored to where the patient is in the natural history of the disease (i.e., progressive disease [young] versus established disease [older subjects]). These findings have implications for prognosis, the timing and type of therapy, and the development of trials of new therapies and their interpretation.     

泰安市RhD抗原阴性无偿献血者表型结果分析

目的:建立泰安市RhD抗原阴性无偿献血者表型库,保证临床需求.方法:应用微板血型血清学方法对献血者进行RhD抗原筛选,采用试管法进行RhD抗原阴性确认,用分型血清进行RhD阴性表型分型.结果:89 860名献血者中检出RhD抗原阴性者352名,占0.39%,RhD抗原变异者10名,占0.01%.d基因频率为0.062 6,D基因频率为0.937 4,以ccdee所占比例最多,为57.39%,cde基因频率为0.047 4,其次为Ccdee占33.80%,其他表型则少见或罕见,其分布特征为ccdee＞Ccdee＞CCdee＞ccdEe＞CcdEe＞CCdEe＞ccdEE.结论:进行RhD抗原阴性无偿献血者表型分型,对于安全输血具有重要意义.     

Fabrication of porous hollow silica nanoparticles and their applications in drug release control.

Preparation and characterization of porous hollow silica nanoparticles (PHSN) for controlled release applications were investigated. Through orthogonally designed experiments, the optimal synthesis conditions for the preparation of PHSN were obtained and the produced PHSN were characterized by BET, SEM, TEM and IR. Scanning and transmission electron microscopy images revealed their hollow shell-core structure and also demonstrated that the size and shape of PHSN are determined by the templating CaCO3 nanoparticles. The produced PHSN were applied as a carrier to study the controlled release behaviors of Brilliant Blue F (BB), which was used as a model drug. Being loaded into the inner core and on the surfaces of the nanoparticles, BB was released slowly into a bulk solution for about 1140 min as compared to only 10 min for the normal SiO2 nanoparticles, thus exhibited a typical sustained release pattern without any burst effect. In addition, higher BET of the carriers, lower pH value and lower temperature prolonged BB release from PHSN, while stirring speed showed little influence on the release behavior. It showed that PHSN have a promising future in controlled drug delivery applications.     

Late-onset and Recurrent Neonatal Group B Streptococcal Disease Associated with Breast-milk Transmission

The purpose of the study was to determine the epidemiological relationships in three unrelated cases of neonatal late-onset Group B streptococcal (GBS) disease and maternal breast-milk infection with GBS. All deliveries were by cesarean section; case 1 was at term, and cases 2 and 3 were at 32- and 33-wk gestation, respectively. Case 1 relates to a mother with clinical mastitis and recurrent GBS infection in a 20-day-old male infant. Following antibiotic therapy and cessation of breast-feeding, the infant recovered without sequelae. Case 2 refers to a mother with clinical mastitis and the occurrence of late-onset GBS disease in 5-wk-old male twins. Despite intervention, one infant died and the second became ill. Following antibiotic therapy and cessation of breast-feeding, the surviving infant recovered without sequelae. Case 3 refers to a mother with sub-clinical mastitis and late-onset GBS infection occurring in a 6-day-old female twin. Following intervention, the infant recovered but suffered a bilateral thalamic infarction resulting in developmental delay and a severe seizure disorder. Following recovery of GBS from an inapparent mastitis and cessation of breast-feeding, the second infant remained well. Blood cultures from all affected infants and maternal breast milk were positive for GBS. Epidemiological relationships between neonatal- and maternal-derived GBS isolates were confirmed by a random amplified polymorphic DNA polymerase chain reaction assay (RAPD-PCR). This study is significant in that it has demonstrated that maternal milk (in cases of either clinical or sub-clinical mastitis) can be a potential source of infection resulting in either late-onset or recurrent neonatal GBS disease.