OBJECTIVE: To observe the application of the tragus cartilage in tympanoplasty. METHOD: Thirty-eight patients with tympanoplasty carried out tragus cartilage-perichondrium repairing tympanic membrane. The air-bone gap changes and the recovery of patients'tympanic membrane were evaluated at pre-operative and two months after the operation. RESULT: Thirty-one patients finishing follow-up visit showed tympanic membrane healed and looked like normal appearance in 1 to 3 months after the operation. The postoperation narrow of the air-bone gap >18 dB, tragus looked normal. CONCLUSION Tympanic membrane reconstruction using the tragus cartilage-perichondrium is feasible for tympanoplasty, and the survival rate and postoperative hearing would be acceptable.
Cartilage ; transplantation ; Ear Auricle ; Hearing ; Hearing Tests ; Humans ; Postoperative Period ; Reconstructive Surgical Procedures ; Tympanic Membrane ; Tympanic Membrane Perforation ; Tympanoplasty ; methods

Objective To study the diagnosis and treatment of hyperthyroidism companied with thyroid carcinoma . Methods The clinical data of 11 cases of hyperthyroidism with thyroid carcinoma were retrospectively analysed. Results 9 of 11 cases were diagnosed preoperatively,and comfirmed by frozen section intra-operatively and underwent suitable operation. In the other two cases the final diagnosis was made by pathological examination postoperatively, and re-operation was performed on 1 case . All the patients were followed up for 1～16 years and neither hyperthyroidism nor thyroid carcinoma recurred. Conclusion It is difficult to make diagnosis of hyperthyroidism with thyroid carcinoma preoperatively. B mode ultrasonography may find some nodes in enlarged thyroid; but fine needle aspisation biopsg(FNA) has high false negative diagnostic rate. Intra-operative frozen section examination is important in the diagnosis of hyperthyroidism with thyroid carcinoma .

In this study, the effect of heparin-derived oligosaccharide (HDO) on platelet-derived growth factor (PDGF) induced vascular smooth muscle cells (VSMCs) proliferation and the related signal transduction mechanisms were investigated. MTT assays were used to measure VSMCs proliferation. Cell cycle distribution was analyzed by flow cytometry. The level of key regulatory proteins in PKC, MAPK and Akt/PI3K pathways were determined by RT-PCR, Western blot and immunocytochemical methods. Meanwhile, mRNA expressions of some proto-oncogenes were assayed by RT-PCR method. Our data showed that HDO (0.01, 0.1 and 1 μmol · L(-1)) inhibited 30 ng · mL(-1) PDGF-induced VSMCs proliferation in a dose-dependent manner, blocked the G1/S transition and inhibited the level of key regulatory proteins and some proto-oncogenes (P < 0.05). The results showed that HDO may decrease the key regulatory proteins expression, hence suppress the transcription of proto-oncogene and G1/S transition, finally inhibiting VSMCs proliferation.

Objective To understand the type and frequency of the gene mutation in exon 6 of phenylalanine hydroxylase(PAH) in the children of Ningxia,in order to provide foundation for phenylketonuria(PKU) gene diagnosis and prenatal diagnosis.Methods The exon 6 and flanking introns of 73 cases of classic PKU patients in Ningxia[all confirmed at Ningxia Neonatal Screening Center from Jan.2010 to Jun.2013,and distributed in Ningxia 22 County (city,district),aged from 15 days to 13 years,including 38 male cases,35 female cases,and Hui 39 cases,Han 34 cases] as well as 100 healthy newborn babies(Hui 50 cases;Han 50 cases) were sequentially analyzed by using the approach of PCR direct sequencing.Results There were 6 kinds of mutations detected,including EX6-96A ＞ G(6.85％),Q232X(2.74％),D222G(1.37％),V2301 (1.37％),R176X (0.68％) and N223I (0.68％).Mutation detection rate of exon 6 was 13.70％,and there were 3 mutation types:50.0％ missense mutation (3 types) ;33.3 ％ nonsense mutation (2 types) ;16.7％ cleavage site mutation(1 type).After reviewing the previous studies,the researchers had found out that EX6-96A ＞ G,Q232X and R176X were ever reported in China,and V2301 and D222G had been reported in our country for the first time but N223I was a new kind of PAH gene mutations were not been reported in the world.Conclusions It has defined the gene type and frequency of PAH gene mutations in exon 6 in the children of Ningxia and it will enrich the research of PKU in this area,and provide the basis for the development of gene diagnosis of PKU.

Deafness ; diagnosis ; genetics ; Genetic Counseling ; Humans

Objective To explore the relationship between serum uric acid and pulmonary hypertension (PH) in patients with systemic sclerosis (SSc). Methods The echocardiography, electrocardiogram, nailfold videocapillaroscopy and laboratory parameters of 62 patients with SSc were retrospectively analyzed . Patients were divided into two groups according to presence of PH . Statistical analysis was performed using SPSS 17 software . Results Compared to patients without PH , patients with PH had significantly higher serum uric acid levels ( P < 0 . 01 ) , systolic pulmonary arterial pressure ( P < 0 . 01 ) , abnormality of electrocardiogram (P < 0.01), abnormality of nailfold video capillaroscopy and lower serum albunin levels (P < 0.01). Systolic pulmonary arterial pressure had correlation with Serum UA ( r = 0 . 26 , P < 0 . 01 ) as well as serum ablumin (r = -0.28, P < 0.03). Moreover, the mean value of serum UA was significantly different in two ECG groups (P < 0.01) and two nailfold videocapillaroscopy groups (P < 0.01). At the cutoff level of 374 μmol/L, serum uric acid had reasonable accuracy for predicting the presence of PH in SSc patients ( sensitivity 66 . 7% and specificity 84 . 0%) . Conclusion The serum uric acid may be useful as a practicable marker for predict PH in patients with SSc .

Objective To observe the change of intestinal microflora on the process of nonalcoholic steatohepatitis(NASH),and to explore the synbiotics therapeutic effect on NASH.Methods Rats were administrated with high fat diet to establish NASH model.In the process of NASH rats modeling,the level of triglyceride (TG), total cholesterol (TC), high-density lipoprotein (HDL), low density lipoprotein (LDL), fasting blood sugar (FBS) and fasting insulin (FINS) was dynamically tested by automatic biochemical analyzer.The change of main intestinal flora was detected by 16 S rRNA fluorescence quantitative polymerase chain reaction.NAFLD activity score was calculated.HE staining was used to observe the hepaticpathological changes and the TLR4 expression was detected by using enzyme-linked immunosorbent assay and immunohistochemical method.Until the 4th,8th,10th weekin the process of NASH modeling, 10 rats were feeded with synbiotics for 2 weeks, and all of above indicators were tested and observed.Results 1)With the extension of a high-fat diet feeding time, the degree of hepatocyte steatosis obviously increased.NAFLD score was significantly heightened(P<0.01).2)Number of independent activities of rats significantly increased, the serological level of TG, TC, LDL, FBS and FINS were lower significantly after intervention with synbiotics for 2 weeks(P<0.05).3)Synbiotics intervention for two weeks significantly increased the amount of bifidobacterium and lactobacillus and decrease the amount of enterococcus significantly(P<0.05).4)The expression of TLR4 was gradually increased in the process of NASH rats modeling(P<0.05),but decreased after 2 weeks of the synbiotics-intervention (P<0.05).Conclusions Intestinal microecology change is closely related to the development of NASH,therefor, synbiotics could improve the quality of life and biochemical indicators of NASH rats through adjusting intestinal microecology and the expression level of TLR4 protein might been involved.

Objective: Diffuse muscular calcification was rare myopathological change due to abnormal metabolism of calcium, which was mainly found in dermatomyositis and myositis ossificans progressiva. Here we reported a case of diffuse muscular calcification that clinically mimicked myositis ossificans progressiva. The disease might be a new type of congenital calcium metabolic disease. Methods:A 15-year-old girl developed subcutaneous cysts in the wrist and ankle when she was 1 year old. At the age of 9, she developed recurrent fever with myalgia, fatigue and diffuse muscular calcification. It was difficult for her to squat, run or walk. Protuberance presented in the subcutaneous tissue of her trunk. Some nodules ruptured with outflow of chalky material. ESR, ENA, RF, CRP, PTH, CK were in normal limits. EMG was unremarkable. X-ray confirmed diffuse calcification in the muscle and subcutaneous tissues. Biceps muscle biopsy was performed. Results:Numerous inflammatory cells infiltrated around vessels in the perimyosium with perifascicular muscle fiber atrophy and degeneration. Many RRF and SDH positive fibers were also observed. EM showed tubular reticular inclusions in vascular endothelium. Conclusion: Diffuse muscular calcification indicated existence of systemic calcium metabolic abnormality. As the clinical symptoms and distribution pattern of calcification were different from dermatomyositis with subcutaneous calcification and myositis ossificans progressiva, our case might be a new type of disease. The microvascular changes might result in the lesion of muscle fibers.

To report the clinical, radiological and neuropathological findings of a patient with rheuma-toid meningitis. The patient was a 71-year-old Chinese man with a two-year history of rheumatoid arthritisand no other significant medical history, who presented to our hospital recurrent weakness of his left ex-tremities, dysarthria and a continuous bilateral hand tremor. Cerebrospinal fluid (CSF) and serumexam-inations were normal apart from a mildly raised serum perinuclear antineutrophil cytoplasmic autoantibody(p-ANCA). Brain magnetic resonance imaging (MRI) showed leptomeningeal enhancementin both fron-tal and parietal lobes, in addition to several old white matter infarcts. Meningeal biopsy showed numerousinfiltrating macrophages and lymphocytes within the leptomeninges. The patient responded clinically andradiologically to corticosteroid and cyclophosphamide therapy. The patient subsequently developed herpeszoster over his left chest as a complication of his immunosuppressive treatment. His cyclophosphamidewas ceased and intravenous immunoglobulin (IVIG) therapy was commenced, with good clinical responseto both the herpes zoster and meningitis. According to the result of the biopsy, aseptic meningitis wasconsidered the MRI results and the patient’s clinical history were given, and a diagnosis of rheumatoidmeningitis was made. The patientwas p-ANCApositive. Although there was no evidence for cerebral vas-culitis on biopsy, it remains a possibility that the patient’s recurrent minor cerebral infarcts visible onMRI were vasculitic in nature.

Objective To evaluate the methods of postoperative respiratory management and complications prevention of patients with myasthenia gravis who received thymectomy. Methods According to the accumulated scores of myasthenic crisis prediction, the patients with myasthenia gravis who underwent thymectomy in the past 5 years were divided into 2 groups: high risk group(12, n=11). The time of mechanical ventilation, restoration of muscle strength and spontaneously breathing during extubation, results of arterial blood gas analysis, body temperature, chest X-ray examination and sputum culture of each patient were analyzed. Results The time of mechanical ventilation in high risk group (18~30 h, 26 h in average) was longer than that in control group(4~28 h, 14 h in average)(P