Child ; Cleidocranial Dysplasia ; diagnosis ; diagnostic imaging ; Humans ; Male ; Radiography

Aim To establish an animal model of uterine leiomyoma for pathogenesis research and drug screen analysis.Methods Guinea pigs were treated with estradiol benzoate 100 ?g?d~(-1),twice a week,by intramuscular injection for 12 weeks.After the animal were killed,the uteri were dissected out and ultrathin sections were obtained.Compare to that of human being,pathohistologic characteristics were observed under the microscope.Results The uteri treated with estrogen were proliferated and deformated.Uterine nodules were observed in many of the tested animals.According to the results of pathohistology,these cells demonstrate the features of smooth muscle-like cells.Conclusion The uterine leiomyoma induced by estrodiol in guinea pig is similar to that of human being.It has been suggested that the animal model is valuable for pathogenesis research and drug screen analysis,although it still needs more evidence to be demonstrated.

Clinical studies have shown that trastuzumab combined with chemotherapy can significantly improve survival in treatment of HER2-positive metastatic breast cancer as well as in the neoadjuvant and adjuvant therapy for HER2-positive early breast cancer. In patients with HER2-positive and hormone receptor-positive metastatic breast cancer, adding trastuzumab to endocrine therapy improves treatment efficacy. Resistance to trastuzumab may be reversed by incorporating other targeted therapeutic drugs. Patients can still benefit from continuing trastuzumab after their disease progresses.

Adult ; Ear Canal ; pathology ; Ear Neoplasms ; Female ; Humans ; Neurofibromatoses

[Objective]To sum up Mr. Zhu Xiankang’s clinical experience in treating children multiple tourette syndrome with nourishing kidney to soothe liver and relieving dizziness to remove sputum. [Method] By expounding the reason and mechanism, treating rules and formula of children multiple tourette syndrome, as wel as analyzing typical clinical cases, it expounds Pro. Zhu Xiankang ’s experience in treating the said disease with his method. [Re-sult] In his view, the disease owes to excessive five sensations, wind sputum accumulated inside, with mechanism of deficient kidney and hyperactivity of liver, wind sputum blocking col aterals, should take nourishing kidney to soothe liver and relieving dizziness to remove sputum as basic method;he applied revised Dingchou Decoction to treat 1 case of multiple tourette syndrome; after 3m, the un-automomous spasm disappeared gradual y, the patient had good appetite and sleep, al symptoms were relieved, and he was cured. [Conclusion] Mr. Zhu Xiankang ’s clinical experience in treating children multiple tourette syndrome has enlightenment to pediatrics clinic.

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Newborn due to low birth weight caused by preterm or fetal growth restriction will have an adverse effect on kidney development. In adulthood,the long-term adverse effects of low birth weight are associ-ated with a variety of kidney disease. Current studies suggest that low birth weight may participate in a variety of kidney disease development and progression by affecting the nephron number,the function of the vascular struc-ture and the endocrine level. This paper mainly reviews the relationship between low birth weight and kidney dis-ease.

Objective To report a pedigree of generalized atrophic benign epidermolysis bullosa (GABEB),a special type of junctional epidermolysis bullosa(JEB). This is the first case report in China. Methods The clinical, histologic, electron microscopic, and immunofluorescence changes of the disease were evaluated. Results Besides the symptom of inherited epidermolysis bullosa, the patient had special clinical manifestations such as atrophic alopecia and dental dysplasia. The disease was inherited in an autosomal recessive mode in this pedigree. Electron－ microscopy found the fissure in lamina lucida, accompaning with dysplasia and sparse hemidesmosome. Immunofluorescence study did not show BPAg2 in the basement membrane zone, indicating that mutation in the gene encoding BPAg2 leading to the disease in this family. Conclusions The case is diagnosed as GABEB, a special type of JEB. Although GABEB has its characteristic, clinical manifestations, electron microscopy and immunofluorescence study are important for confirmative diagnosis and classification, and will guide the study of mutation detection.

Gallstone disease is highly prevalent in clinic,particularly in women and some specific ethnic groups.The formation of water-insoluble cholesterol crystals is due to a misbalance between the three major lipids present in the bile:cholesterol,bile salts,and phospholipids.Many proteins implicated in biliary lipid secretion in the liver are regulated by several transcription factors,including nuclear receptors LXR and FXR.Human and murine genetic,pathophysiological evidence is consistent with the relevance of these nuclear receptors in gallstone formation.In addition,there is emerging data that also suggests a role for estrogen receptor ESR1 in abnormal cholesterol metabolism leading to gallstone disease.A better comprehension of the role of nuclear receptor function in gallstone formation may help doctors to design new and more effective therapeutic strategies for this highly prevalent disease condition.

Sepsis is a major cause of secondra y pancreatic injury in critically ill children.A lagr e number of inflammatoyr m ediators werer elesa ed after acute ijn ury of the pancreas,which may aggravate the inflammatory reaction and the stress reaction.Nuclear factor E2-related factor 2(Nrf2),an important protec-tive transcription factor,could activate the expression of more than 500 genes.Most of the genes have the function of cytoprotection.Studise have dem onts rated that Nrf2 plays a certain role in cytoprotection,such as regulating the inflammatory response,anti-oxidative stress,improving mitochondrial functoi n and decreasing toxic protein aggregation.This review explored the protective mechanism of Nrf2 in pancreatic injury caused by sepis s.