Research progress on Melkersson-Rosenthal syndrome / 浙江大学学报·医学版

Melkersson-Rosenthal syndrome (MRS) is a rare neuro-muco-cutaneous syndrome, which is characterized by recurrent orofacial swelling, recurrent facial paralysis and fissured tongue. It has a high prevalence in young adults. Up to now, the etiology of MRS is still not clear, it may related to infection, immune deficiency and hereditary factors. The pharmacological therapy and surgery are the main treatment. Corticosteroids seems to be the drug of choice for MRS patient, but the specific dosage and therapeutic effect have not yet been determined. Surgeries of lips provide excellent results in persistentlip edema MRS cases. This article reviews the research progress on MRS, focusing on its epidemiology, etiology, histopathological characteristics, clinical manifestations, classification, diagnostic criteria, differential diagnosis and treatment, to provide information for its early diagnosis and appropriate treatment.

Application of medical magnifying loupes in diagnosis of oral mucosal diseases / 浙江大学学报·医学版

: To evaluate the application of medical magnifying loupes in diagnosis of oral mucosal diseases. Twenty-four patients with plaque-type oral lichen planus or homogeneous oral leukoplakia were inspected by naked eyes or assistance with magnifying loupes. Histopathological results were used as the gold standard to evaluate the sensitivity, specificity and accuracy of the two methods in clinical diagnosis. Questionnaires were used to evaluate the subjective effect of magnifying loupes on the diagnosis efficiency of oral mucosal diseases and to explore the most suitable parameters for application. The sensitivity, specificity and accuracy of medical magnifying loupes for the identification of plaque-type oral lichen planus and homogeneous oral leukoplakia were 94.74%, 100.00% and 95.83%, respectively, which were significantly higher than those of naked eye inspection (89.47%, 80.00% and 87.50%). The effective rate of magnifying loupes assisted diagnosis was 91.76% according to physicians' subjective evaluation. The most suitable parameters were 3.5 times magnification and working distance. The medical magnifying loupes can effectively improve the efficiency of the inspection and diagnosis of oral mucosal diseases, and have the characteristics of convenience and real-time. The recommended clinical parameters are 3.5 times magnification and working distance.

Relationship between joints bleeding and joint assessment in children with severe haemophilia A / 中华实用儿科临床杂志

Objective:To understand the relationship between joint bleeding and joint disease in hemophilia children, and to provide a theoretical basis for clinical treatment and prognosis.Methods:The patients with severe hemophilia A between 1 and 7 years old and with relevant nodal bleeding records were selected.All the patients admitted in Beijing Children′s Hospital, Capital Medical University, and Chengdu New Century Women′s and Children′s Hospital since June 2016 to January 2017.All the joint bleeding of each child was taken as the study joint, and the joint bleeding was collected during the last 3 months.The joints were assessed by using ultrasound, X-ray, magnetic resonance imaging(MRI) and Hemophilia Joint Health Score (HJHS) scoring systems.The correlation analysis was conducted between the joint bleeding, ultrasound, X-ray, MRI and HJHS scores.The correlation analysis was conducted for baseline ultrasound, X-ray, MRI and HJHS scores.Results:(1) There were 18 patients enrolled.The mean age was (5.6±1.8) years old.There were 30 joints bleeding in the observation period in total, with the annul median joint bleeding times of 4 (4-16 times), and the annul median target joint bleeding times of 8 (4-16 times). (2) Joint bleeding times of was correlated with ultrasound and X-ray evaluation ( r=0.390, P=0.033; r=0.517, P=0.008), and not correlated with HJHS or MRI(all P>0.05). (3) There was significantly positive correlation among ultrasound, X-ray, HJHS and MRI [ r=0.815(ultrasound vs.X-ray), r=0.510(ultrasound vs.HJHS), r=0.812(ultrasound vs.MRI), r=0.666(X-ray vs.HJHS), r=0.911(X-ray vs.MRI), r=0.781(HJHS vs.MRI), all P<0.01]. (4) There was no correlation between times and assessment for joints whose ultrasound and /or MRI in joints with abnormal ultrasound and /or MRI evaluation( P>0.05). Conclusions:The results of joint bleeding and joint evaluation are inconsistent.Joint bleeding can not truly reflect the situation of joint diseases.The assessment of hemophilia should include comprehensive evaluation of joint structure, function, activity ability and other aspects to guide the treatment of haemophi-lia children.

Analysis of the arthropathies on no-bleeding history joints in pre-school age severe hemophilia A children / 中华儿科杂志

Objective@#To detect the arthropathies on no bleeding history joints in pre-school hemophilia A children in order to provide evidence for further prevention and control of joint disease in children with hemophilia A.@*Methods@#This study was a cross-sectional study based on China Hemophilia Individualized prophylaxis study (CHIPS). The basic data of outpatients with hemophilia in Beijing Children's Hospital and Chengdu Women's and Children's Central Hospital between August 2016 and June 2017 were collected and a three-month follow-up was conducted. The target joints (six joints of bilateral elbows, knees and ankles) of thirty-four children aged 1-7 years old with severe hemophilia A were examined by ultrasound, X-ray and joint function examination (4-7 years old, hemophilia joint health score (HJHS)). To find out whether there are arthropathies in patient's joints with no bleeding history and analyze the relevant factors by chi-square test, rank sum test and other statistical methods.@*Results@#There were 32 analyzable cases with 112 no-bleeding history target joints, 42.9% (48/112) were elbow joints. Arthropathies were revealed in 34.8% (39/112) of them by joint structural and functional examination and 46.2% (18/39) were ankles (χ2=8.379, P=0.015) . Ultrasound showed abnormalities in 18.3% (20/109) joints, X-ray showed abnormalities in 3.8% (3/79) joints and HJHS showed abnormalities in 25.3% (20/79) joints. There was no correlation between ultrasound and HJHS (r=0.015, P=0.895), no correlation was found between X-ray and HJHS (r=-0.101, P=0.390) either, which suggested that joint structural and functional examination could not replace each other. The related risk factors of arthropathies in this group were >4.91 years old (OR=3.917, 95%CI:1.610-9.528) and combining with target joint (OR=3.530, 95%CI:1.316-9.465).@*Conclusions@#Detecting the joint structure and function on no bleeding history joints in pre-school hemophilia A children could reveal the arthropathies and majority of them were ankle arthropathies. Joint structural and functional examinations could not replace each other. For patients more than 5 years old and those with target joints, the joints with no complaint of bleeding should be examined regularly to reveal the arthropathies in time.

Investigation and Analysis of Pregnancy Contraindication in Package Inserts of 210 Kinds of Chinese Pat-ent Medicines / 中国药房

OBJECTIVE:To provide reference for drug consultation of Chinese patent medicine(CPM)in pregnancy. METH-ODS:Package inserts of CPM in our hospital during Jan.-Jun. 2016 were collected. Referring to the prohibited,contraindicated and caution materials and decoction pieces in Chinese Pharmacopoeia(part 1,2015 edition),based onnoteitem in Chinese Pharma-copoeia and Clinical Application Guidelines(2010 edition),problems existing in pregnancy contraindication labeling of package in-serts were compared and analyzed. RESULTS:There were 99 kinds of prohibited,contraindicated and caution materials and decoc-tion pieces in Chinese Pharmacopoeia. There were 210 package inserts of CPM except for the special medicine for children,in which 32 contained prohibited materials,61 contained caution materials,only 21 were in line with the contained materials. Among the package inserts of 93 CPM containing prohibited or caution materials,27 were included in Chinese Pharmacopoeia,13 in Clini-cal Application Guidelines only,17 in both,but only 6 had the same contraindication labeling. Among the package inserts of 32 CPM containing prohibited materials,6 were explicitly labeled prohibited,17 labeled contraindicated or caution,and 9 labeled none. Among the package inserts of 61 CPM containing caution materials,29 were labeled prohibited or contraindicated,15 la-beled caution,and 17 labeled none. Among the package inserts of 117 CPM containing no prohibited,contraindicated or caution materials,8 were labeled contraindicated in pregnancy,and 18 labeled prohibited in pregnancy. CONCLUSIONS:The pregnancy contraindications of most CPM are not normative,showing poor consistency with Chinese Pharmacopoeia or Clinical Application Guidelines. Except for providing drug consultation by complying with Chinese Pharmacopoeia,Clinical Application Guidelines and package insert,pharmacists can judge the CPM without labeled pregnancy contraindication by analyzing its classification of con-tained materials. Using TCM does not indicate safety drug use,in addition,some CPM contain western medicine ingredients. There-fore,pharmacists should conduct medication education for patients who used CPM in pregnancy. Considering the new and severe adverse reactions of TCM injections are more,its adverse reactions exist unpredictability,so that pregnant patients should be sug-gested to avoid using TCM injections by pharmacists.

Patient with pemphigus vulgaris complicated with dysfunctional uterine bleeding upon glucocorticoid usage: a case report / 华西口腔医学杂志

Pemphigus is a severe chronic autoimmune mucocutaneous bullous disease. Glucocorticoids are considered as the first line of treatment for this disease. Dysfunctional uterine bleeding is also observed as a result of hypothalamic-pituitary-ovary axis dysfunction. This study reported one female patient with pemphigus vulgaris complicated with dysfunctional uterine bleeding upon systemic glucocorticoid usage. Before this disease was diagnosed, the patient experienced normal menstruation. The mechanism of dysfunctional uterine bleeding triggered by glucocorticoids is elucidated on the basis of case studies and literature review.