【Objective】 To investigate the influencing factors behind the follow-up compliance of patients with low/no phenylketonuria (PKU) for special medical use, in order to provide a basis for regulating the follow-up of PKU patients and ensuring the effectiveness of special diet treatment. 【Methods】 A survey was conducted on PKU patients treated in Urumqi Maternal and Child Health Hospital for over 1 year, from January 2010 to December 2020. Interviews and questionnaires were conducted with their caregivers to collect and analyze the current status of PKU patients undergoing special diet treatment, and to identify the influencing factors behind their compliance with follow-up treatment. 【Results】 Patients who had received neonatal disease screening, neonatal gene diagnosis, and maternal Down′s screening during pregnancy had better compliance, with statistically significant differences (χ²=5.753, 10.993, 9.189, P<0.05). PKU children with parents who had a college education or above showed significantly higher adherence to special diet treatment (χ²=8.321, 7.415, P<0.05). PKU children with parents having a fixed occupation also showed higher compliance, with a statistically significant difference (χ²=20.626, 7.895, P<0.05). Patient age, interval of buying special diet, number of blood samples sent and enrollment of normal age, all had a significant impact on the follow-up compliance of PKU patients with special diet (χ²=19.443, 8.090, 69.482, 12.001, P<0.05). 【Conclusions】 PKU is a treatable genetic metabolic disease. Strengthening health education, formulating standardized follow-up plans and procedures, and improving follow-up treatment compliance are crucial in enhancing the treatment and follow-up effectiveness of PKU patients.

Hepatic fibrosis (HF) is the common pathological process of various chronic liver diseases and is associated with the progression of liver diseases, and it is also a key factor affecting the outcome of liver diseases and the risk of hepatocellular carcinoma (HCC). The early process of HF is reversible, but without timely intervention and treatment, HF may gradually aggravate and progress to liver cirrhosis and even HCC, which will endanger people's health. Therefore, it is of great significance to actively prevent and treat HF; however, due to the complex pathogenesis of HF, Western medicine treatment is limited and there is still a lack of effective and widely accepted anti-fibrotic drugs, and thus there is a huge demand for medical treatment. With the efforts of scholars in China and globally, traditional Chinese medicine treatment has become a hotspot and a key breakthrough point in reversing HF and preventing its progression, with the advantages of multiple targets and pathways. This article elaborates on the advantages and characteristics of TCM treatment of HF from the aspects of syndrome differentiation, method of treatment, drug compatibility, and modern molecular mechanism.

A 40-year-old male patient presented with an unexplained pericardial effusion and was treated with antituberculosis therapy. This patient showed a transient improvement, but then progressively worsened to develop constrictive pericarditis, multiple plasma chamber effusions, and venous thrombosis. The patient was transferred to Zhongshan Hospital, Fudan University. Through a pleural histopathological biopsy, this patient was diagnosed with pleural mesothelioma and secondary malignant tumor of the pericardium. Later, clinical the manifestations of patient progressed rapidly, resulting in the death of the patient. Mesothelioma is a very rare group of malignant tumors originating from the pleura and other tissues. Patients are mostly advanced at the time of initial diagnosis, with limited treatments and short median survival. It is important to strengthen the understanding of mesothelioma, pay attention to all medical and technical examinations, especially dynamic follow-up of changes in the disease for early diagnosis of this disease.

Congenital hydrocephalus is a major neurological disorder with high rates of morbidity and mortality; however, the underlying cellular and molecular mechanisms remain largely unknown. Reproducible animal models mirroring both embryonic and postnatal hydrocephalus are also limited. Here, we describe a new mouse model of congenital hydrocephalus through knockout of β-catenin in Nkx2.1-expressing regional neural progenitors. Progressive ventriculomegaly and an enlarged brain were consistently observed in knockout mice from embryonic day 12.5 through to adulthood. Transcriptome profiling revealed severe dysfunctions in progenitor maintenance in the ventricular zone and therefore in cilium biogenesis after β-catenin knockout. Histological analyses also revealed an aberrant neuronal layout in both the ventral and dorsal telencephalon in hydrocephalic mice at both embryonic and postnatal stages. Thus, knockout of β-catenin in regional neural progenitors leads to congenital hydrocephalus and provides a reproducible animal model for studying pathological changes and developing therapeutic interventions for this devastating disease.
Animals ; Disease Models, Animal ; Hydrocephalus/genetics* ; Mice ; Mice, Knockout ; Neurons ; beta Catenin/genetics*

Objective:To analyze the epidemiological characteristics and trend of stroke death among registered residents in Hexi District of Tianjin from 2007 to 2019，and to provide scientific basis for evaluation of comprehensive prevention and control strategies and measures of stroke. Methods:All death monitoring data of the residents was collected and the crude mortality， standardized mortality and annual percent change （APC） of stroke were obtained by statistical treatment. Results:A total of 15 235 stroke deaths were reported in Hexi District of Tianjin from 2007 to 2019，with an average annual mortality rate of 144.59/10⁵. The rate was higher in males than in females （169.02/10⁵ in males and 121.04/10⁵ in females， P<0.001）. The crude mortality rate showed an upward trend with the year （APC=1.51%， P<0.05）. The standardized mortality rate showed a downward trend （APC=-1.39%， P<0.05）. The crude mortality of ischemic stroke showed an upward trend with the year （APC=3.15%， P<0.01）， and the trend change in standardized mortality rate had no statistical significance. The crude mortality and standardized mortality of hemorrhagic stroke showed a downward trend，with the mortality rate decreased at 2.43% per year and the standardized mortality rate decreased at the 4.50% per year （all P<0.05）. Conclusion:Stroke is the main disease affecting the health of residents in Hexi District of Tianjin. Targeted measures should be taken to reduce the mortality of stroke. Stroke prevention and control should focus on winter and spring seasons， males， elderly people and patients with ischemic stroke.

Objective: To study the effects of nerve growth factor (NGF) on the proliferation, osteogenic differentiation and mineralization of type 2 diabetic mice bone marrow stromal cell (BMSC), providing basis for clinical application of NGF. Methods: Three 8-week-old male db/db mice and two 8-week-old male C57BL/6J mice were used in the study. BMSC derived from femur were cultured though adherence method. BMSC of C57BL/6J mice and db/db mice was divided into normal group and diabetic group to conduct the osteogenic potential experiment, named experiment one. In experiment two, diabetic BMSC was divided into 3 groups: diabetic control group, NGF group, and K252a+NGF group [K252a was the inhibitor of tyrosine kinase A (TrkA), which was the high affinity receptor of NGF], to investigate effect of NGF on osteogenic potential of diabetic mice BMSC. After seeding BMSC, K252a was added into K252a+NGF group, then NGF was added 30 min later. NGF was added into NGF group and K252a+NGF group, but not diabetic control group. The proliferation of BMSC at 1, 3, 5 and 7 d in experiment one and the proliferation of BMSC at 1, 2 and 3 d in experiment two were evaluated through methyl thiazolyl tetrazolium, and the level of alkaline phosphatase (ALP) at 3, 5 and 7 d in both experiments were measured. After being osteogenic induced for 14 d, mineralized nodules in both experiments were quantitated by alizarin red calcium stain. Five holes were set in every group, and all experiments were repeated 3 times. Results: The BMSC proliferation of diabetic group was significantly higher than that of the normal group at 3, 5 and 7 d (P<0.05). After being osteogenic inducted for 3, 5 and 7 d, ALP level of diabetic group were significantly lower than that of normal group (P<0.05). After being osteogenic inducted for 14 d, calcium nodule count of diabetic group [(23.1±6.4) nodule/field] were significantly lower than that of normal group [(36.9±7.9) nodule/field](P<0.05). At 1, 2 and 3 d, BMSC proliferations of diabetic control group, NGF group and K252a+NGF group were not statistically different (P>0.05). After being osteogenic inducted for 3 and 5 d, ALP level of NGF group was significantly higher than that of diabetic control group (P<0.05). After being osteogenic inducted for 3, 5, and 7 d, ALP level of K252a+NGF group was significantly lower than that of NGF group (P<0.05) and diabetic control group (P<0.05). After being osteogenic induced for 14 d, calcium nodule count of NGF group [(45.2±6.8) nodule/field] was significantly more than that of diabetic control group [(23.1±6.4) nodule/field](P<0.05); while calcium nodule count of K252a+NGF group [(18.0±4.5) nodule/field] was significantly less than that of NGF group (P<0.05) and diabetic control group (P<0.05). Conclusions The differentiation and mineralization of type 2 diabetic mice BMSC was significantly reduced. NGF promoted the osteoblastic differentiation and mineralization of diabetic mice BMSC in viro though combining with TrkA.

Objective To study the expression of lipoic acid synthase(LIAS)in the liver and kidney of Leprdb/db mice with deficient leptin receptor. Methods Eight 10-week old male Leprdb/ +mice and Leprdb/dbmice were included in this study. The body weight of rats in the two groups was measured. Fasting blood glucose(FPG)was measured with blood glucose test strips for all mice after fasting for 8 hours. Blood samples were obtained from the abdominal aorta and the animals were sacrificed. The liver and kidney were weighed. The right lobe of liver and the left kidney samples were fixed in 4% paraformaldehyde for pathological examination. Serum samples were separated and the sereum contents of CHO, TG,HDL and LDL were detected. The mitochondria of liver and kidney tissues were extracted with a mitochondrial isolation kit, and the protein was extracted. The expression of LIAS protein was detected by western blot. Results Histopathological observation showed that the liver and kidney tissues of Leprdb/ +mice have intact and clear structure. But the liver tissue of Leprdb/dbmice showed fatty degeneration, the kidney tissue showed glomerular hypertrophy, basement membrane thickening, mesangial area widened, including mesangial cells and mesangial matrix increased. The GLU,CHO,TG,LDL and AST of Leprdb/dbmice were significantly increased compared with those of Leprdb/ +mice(P<0.05). Compared with Leprdb/ +mice,the LIAS protein expression was significantly increased in the liver and kidney mitochondria of Leprdb/dbmice(P<0.05). Conclusions There is impaired glucose and lipid metabolism in the Leprdb/dbmice which has defect leptin receptor,and the expression of LIAS protein in liver and kidney of the Leprdb/dbmice is higher than that of Leprdb/ +mice.

Objective: To study the quality testing method for the seeds of Silybum marianum, so as to provide the basis for the development of testing procedures and quality grading standard for the seeds of S. marianum. Methods: Refering to "The International Seed Testing Rules" and "China Crop Seed Testing Rules" to carry out the quality testing for the seeds of S. marianum. Results: The seed cleanliness was analyzed by winnowing method; The authenticity was identified by morphological appearance compared with 1000 grain weight by 1000 grain weight determination method; The germination conditions for seed germination were washed with running tap water before 2 h, the double filter paper was used as germination bed, and the seeds were incubated at 20 ℃, 8000 lx light, counting time for 4-14 d; The viability was determined by electrical conductivity method. Conclusion: The method is simple and reliable to the quality testing for the seeds of S. marianum.

Objective To study the effects of improved Wright-Giemsa staining. Methods 40 semen samples by both traditional and improved Wright-Giemsa staining.The morphological characteristics stained by two methods was ob-served. Results The defective rates of the middle and tail parts of sperm were 7.02%± 2.4%and 11.02%± 2.03%respec-tively in improved staining group;while those were 5.48%±2.8%and 8.05%±2.56%in traditional staining group. There was statistical significance between two staining methods (P < 0.05). There was no significant difference of normal sperm and sperm head between these two staining methods;however, improved staining method showed a much clearer nucleus and ac-rosome staining than that of traditional methods. Improved Wright-Giemsa staining is better for sperm, vaginal discharge and Trichomonas vaginalis. More importantly, axostyle and flagella were clearly stained with the improved method. Conclusion Improved Wright-Giemsa staining is simple and can be used in clinical diagnosis.

In natural conditions, fully ripe Cynomorium songaricum seeds parasitize in Nitraria tangutorum or N. sphaerocarpa or N. sibirica or Zygophyllum xanthoxylom and Peganum harmala, were used in this study to research the morphological characteristics, embryo rate, seed viability, 1 000-grain weight, purity, water content and the seeds of different host parasitic relationship with each other. The results showed that the morphology, color and surface characteristics of the C. songaricum seeds are very similar in different hosts. According to the seed morphology can not be judged on its host. For the host to N. tangutorum or Peganum harmala or N. sibirica, we should choose the round hole screen less than 0.923 1 mm and larger than 1.066 2 mm to cleaning seeds. For the C. songaricum seeds parasitic in N. sphaerocarpa, the choice of slightly less than 0.926 1 mm and larger than 0.985 3 mm round hole screen to cleaning. For the parasitic seeds in Z. xanthoxylom, less than 0.751 3 mm and slightly larger than 1.035 3 mm round hole screen could be used. Highy significant correlation was found among the morphological indexes in C. songaricum seeds (P < 0.01). Morphological indexes and 1 000-grain weight were significantly correlated (0.01 < P < 0.05), but with the seed viability and the embryo rate were not found significant correlation. Grain weight is not related with the seed viability and the Fully mature C. songaricum seed viability is high and water content is low. The difference of the habitats and the host plants should be considered in the seed quality assessment and classification. The C. songaricum seeds on host plants are not selective, and the C. songaricum seeds from the host plants could be parasitized in other host plants.
China ; Cynomorium ; chemistry ; growth & development ; physiology ; Ecosystem ; Host Specificity ; Magnoliopsida ; physiology ; Seeds ; chemistry ; growth & development ; physiology