Clinical Characteristics and Treatment of External Auditory Canal Cholesteatoma / 听力学及言语疾病杂志

Objective To investigate the clinical characteristics and treatment of external auditory canal chol‐esteatoma (EACC) .Methods The clinical data of 38 cases(39 ears)with external auditory canal cholesteatoma from August 2006 to December 2014 were retrospectively analyzed .Results All the cases of EACC in this study had the external ear canal full of impacted squamous material or granulation tissue .The Holt and CT imaging examinations disclosed that 10 ears were phase I ,lesions were confined to the external auditory with no bone destruction .There were 23 ears were phase II ,the lesions were located in external auditory meatus with destruction of bone ,but with no involvement of the middle ear .There were 6 ears were phase III ,showing the lesions with disrupt external audi‐tory meatus and involvement of the middle ear ( mastoid and/or tympanic sinus) .Hearing impairment and aural fullness were the most common symptoms .The phase I cases were treated by removing cholesteatomas from the ex‐ternal auditory canal .Canaloplasty and/or tympanoplasty were performed in phase II cases .The phase III cases were successfully managed by modified radical mastoidectomy and/or tympanoplasty .There were 30 ears of tympan‐ic membrane were perfect and invaginate .There were 4 ears of ossicular chain were disrupted with one ear of facial nerve exposed .All surgeries were performed at once .No recurrence except in one patient was found during the fol‐low -up period .Conclusion The clinical stages can help identify the primary lesions and determine the choice of the best surgical approach .

Molecular pathogenesis of Waardenburg syndrome type II resulting from SOX10 gene mutation / 中华医学遗传学杂志

<p><b>OBJECTIVE</b>To explore the molecular mechanism of Waardenburg syndrome type II (WS2) resulting from SOX10 gene mutation E248fs through in vitro experiment.</p><p><b>METHODS</b>293T cells were transiently transfected with wild type (WT) SOX10 and mutant type (MT) E248fs plasmids. The regulatory effect of WT/MT SOX10 on the transcriptional activity of MITF gene and influence of E248fs on WT SOX10 function were determined with a luciferase activity assay. The DNA binding capacity of the WT/MT SOX10 with the promoter of the MITF gene was determined with a biotinylated double-stranded oligonucleotide probe containing the SOX10 binding sequence cattgtc to precipitate MITF and E248fs, respectively. The stability of SOX10 and E248fs were also analyzed.</p><p><b>RESULTS</b>As a loss-of-function mutation, the E248fs mutant failed to transactivate the MITF promoter as compared with the WT SOX10 (P<0.01), which also showed a dominant-negative effect on WT SOX10. The WT SOX10 and E248fs mutant were also able to bind specifically to the cattgtc motif in the MITF promoter, whereas E248fs had degraded faster than WT SOX10.</p><p><b>CONCLUSION</b>Despite the fact that the E248fs has a dominant-negative effect on SOX10, its reduced stability may down-regulate the transcription of MITF and decrease the synthesis of melanin, which may result in haploinsufficiency of SOX10 protein and cause the milder WS2 phenotype.</p>

Experimental Studies on Regulation of PAX3 on Transcriptional Activities of Target Gene MITF / 听力学及言语疾病杂志

Objective To investigate the impact of pair box 3 (PAX3) gene mutations on transcriptional ac‐tivity of target gene microphthalmia -associated transcription factor (MITF) and the role it plays in the pathogene‐sis of Waardenburg syndrome type I .Methods The 293T cells were transient transfected with wild type (WT ) PAX3 and mutant type (M T ) H80D ,H186fsX5 plasmids .We observed and analysed the regulation effects of WT/MT PAX3 on the transcriptional activities of MITF and the influence of the two mutants on WT PAX3 function u‐sing luciferase activity assays ,detect DNA binding capacity of WT/MT PAX3 to MITF gene promoter using a bioti‐nylated double - stranded oligonucleotide probe containing PAX3 binding motif ATTAAT to precipitate PAX3 , H80D and H186fsX5 respectively .Results H80D mutant was partially functional and was able to transactivate the MITF promoter in part ,but it was dramatically reduced as compared with WT PAX 3(P0 .05) .WT PAX3 and H80D mutant were able to bind specifically to the ATTAAT motif on the MITF promoter ,whereas H186fsX5 PAX3 lost the DNA -binding ability .Conclusion The mutations H80D and H186fsX5 made down-regulation of MITF transcription and decrease syn‐thesis of melanin ,which resulted in haploinsufficiency of PAX3 protein and caused mild phenotypes of WS1 .

Diagnosis and treatment of primary ectopic thyroid carcinoma: report of 3 cases and literature review / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#Discuss the clinical features of primary ectopic thyroid carcinoma.@*METHOD@#The clinical data of three cases diagnosed of primary ectopic thyroid carcinoma in our department since 1990 were analyzed ret respectively and related literature was reviewed.@*RESULT@#All three patients were young females that had cervical lumps before surgery. The pathology results confirmed the diagnosis of ectopic thyroid papillary carcinoma. All cases had followed up till now and no relapse signs occurred.@*CONCLUSION@#Ectopic thyroid tissue is resulting from developmental defects at early stages of thyroid gland embryogenesis. They can cancerization, the treatment of ectopic thyroid carcinoma is also controversial. Here,we report 3 cases of primary ectopic thyroid papillary carcinoma, all of which were removed by surgery, take thyroxin orally after surgery and have a great prognosis.

Detection of Runx2 mRNA expression using relatively real-time RT-PCR in papillary thyroid carcinoma / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To relatively detect the Runx2 mRNA expression in papillary thyroid carcinoma (PTC) and thyroid adenoma, then to investigate the role of Runx2 in the development and progression in PTC and the relationship with the micro calcification in PTC.@*METHOD@#The expression of Runx2 mRNA in 14 samples of PTC and 14 samples of thyroid adenoma was examined by relatively real-time RT-PCR.@*RESULT@#The deltaCT value of the carcinoma group and adenoma group was 2.395 +/- 0.302 and 5.028 +/- 1.179 respectively (P0.05), and the adenoma group as well. The carcinoma group was divided into two groups by the size of carcinoma ( or = 1 cm). The deltaCT value was 2.629 +/- 0.300 and 2.212 +/- 0.124 respectively (P0.05).@*CONCLUSION@#The expression of Runx2 mRNA was high in PTC, and was related to the size of carcinoma which was higher in bigger size carcinoma. The role of Runx2 may contribute to the formation of the micro-calcification and the development and progression in PTC and other malignant tumors, such as breast cancer, prostatic carcinoma and osteosarcoma.

Adjustment of levothyroxine during pregnancy in women undergoing thyroid carcinoma surgery / 中华内分泌外科杂志

Objective Hypothyroidism during pregnancy has been associated with impaired cognitive development and increased fetal mortality.In this retrospective study we attempt to identify the timing and adjustment of levothyroxine during pregnancy.Methods 20 women planning pregnancy after thyroidectomy because of thyroid cancer were observed before and throughout their pregnancies.Their thyroid function was measured before conception,and approximately every 4 weeks during pregnancy.The dose of levothyroxine was increased to maintain the thyrotropin concentration at preconception values throughout pregnancy.Results 21 pregnancies occurred in the 20 women and resulted in 20 full-term births.One woman had abortion.Their basal dose was all 100 μg.The dose increased by 12.5 μg a time to target the thyrotropin concentrations (0.1-0.25 mU/ml).The mean levothyroxine requirement increased 50 percent during the first half of pregnancy and plateaued by the 16th week.This increased dose was required until delivery.All the newborns and their mothers were healthy according to the follow-up.Conclusions We propose that women after thyroid cancer surgery should increase levothyroxine dose as soon as pregnancy is confirmed.Thereafter,serum thyrotropin level should be monitored and the levothyroxine dose adjusted accordingly.The adjustment dose is set at 12.5 μg and the maternal thyrotropin concentration is between 0.10 and 0.25 mU/ml,which guarantees healthy growth of fetus while suppresses thyroid tumor growth simultaneously.

Clinical analysis of granulomatous capillary hemangioma of the larynx / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To improve the acknowledgement of clinician on granulomatous capillary hemangioma of the larynx.@*METHOD@#Collect patients in our hospital who were treated with granulomatous capillary hemangioma of the larynx from 2006 to 2011 and the clinical data, treatment and follow-up consequence were retrospectively analyzed.@*RESULT@#There are 10 cases of laryngeal granulomatous capillary. Eight patients with hoarseness as the chief complaint, in the eight patients three cases associated with foreign body sensation in the throat. One patient with hemoptysis as the main performance. The left one patient to hospital because of pharyngeal foreign body sensation associated with blood in the sputum. All were treated with surgery, there are several cases supplemented by CO2 laser treatment, so far no case of postoperative recurrence.@*CONCLUSION@#Granulomatous capillary hemangioma is a benign, vascular proliferation of the skin or mucosa, rather than ture tumor. Recurrence is rare if completely excised. The prognosis is encouraging.

The significance of calcification in the thyroid papillary carcinoma / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To study the significance of calcification in the thyroid papillary carcinoma.@*METHOD@#Retrospectively analyze 88 cases of thyroid papillary carcinoma.@*RESULT@#There was no association between calcification with age,TSH and TNM. But calcification was related to the size of tumor (P < 0.05). In addition, the level of TSH has no relationship with the size of tumor.@*CONCLUSION@#Calcification especially microcalcification may have significant relationship with thyroid papillary carcinoma and be directly related to the size of tumor. The larger size of tumor implies the higher possibility of calcification.

The significance of middle thyroid vein in the VI region neck dissection / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate the effect of VI region neck dissection in thyroid papillary carcinoma surgery and to prompt the key matters in such surgery.@*METHOD@#Retrospectively analyzed 213 cases of thyroid papillary carcinoma treated by Renji ENT Department from 2003 to 2009. Under each case, we implemented the VI region neck dissection after marking recurrent laryngeal nerve with middle thyroid vein.@*RESULT@#Among the 213 cases of thyroid papillary carcinoma performed with VI region neck dissection, 82 cases were positive, 3 cases incurred recurrent laryngeal nerve injury with 2 cases of permanent RLN injury and 2 case of temporary injury, 2 cases showed permanent Hypoparathyroidism, and 6 cases showed temporary Hypoparathyroidism. In 3 cases the metastasis of neck lymph arose within 3 years.@*CONCLUSION@#Marking recurrent laryngeal nerve with middle thyroid vein not only helps to lower the possibility of injuring RLN and parathyroid glands in thyroidectomy, but also makes the VI region neck dissection an feasible treatment for thyroid papillary carcinoma.

Significance of modified radical neck dissectionin papillary thyroid carcinoma / 临床耳鼻咽喉头颈外科杂志

Objective:To investigate the recurrence of the neck and survival incidence of clinically possible pos-itive lateral cervical nodes in patients with well-differentiated papillary thyroid cancer and the essentiality of the performation of modified radical neck dissection. Method: Retrospective datum of 267 cases of papillary thyroid car-cinoma were collected and ananlyzed. They were divided into two groups with time; selective neck dissection with VI area was performed in 151 cases of group A and selective neck dissection was performed without VI area in 116 cased of group B. They were received the same treatment in the thyroid-total thyroidectomy on initial part and subtotal thyrodectomy on the other, and total thyroidectomy on both parts if both were invated. Incidence of posi-tive lateral cerivcal nodes of VI area in group A and the recurrence in the neck and survival incidence in groups B were analyzed. Result: Fiftynine patients in group A were treated with the modified radical neck dissection, and 22 patients of them were found metastasis in lateral cervical nodes with Ⅵ area. The other 92 patients were treated with neck dissection of Ⅱ,Ⅲ,Ⅳ,Ⅵ VI area, and 31 patients weren't metastasis, 33 patients were metastasis in both Ⅵ area and the others(35.8%), 17 patients were metastasis only in Ⅵ area(18.4%), 11 patients weren't metastasis only in Ⅵ area(11.9%). Thus, the incident of metastasis in Ⅵ area was 47.70% in group A(72/151). 47 patients in group B had metastasis in lateral cervical nodes though without performing VI area neck dissection (40.5%), and the survival rate of 5 years was 99.3%. The recurrence rate in the neck of group two was 6.0% (7/116). The metastasis rate of neck lymph node was higher in group A (54.9%) than group B(40.5%). Conclu-sion : Pretracheal and peripheral recurrent nerve lymph node are very susceptible to the metastasis of well-differenti-ated papillary thyroid cancer. The neck dissection of VI area could be performed as routine.

The significance of p63 expression in thyroid neoplasm / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To explore the significance of p63 expression in thyroid carcinoma, thyroid papillary carcinoma, thyroid follicular carcinoma, squamous cell carcinoma and medullary thyroid carcinoma in order to find the possible causes of such thyroid-related diseases and if there is some kind of relation among them.@*METHOD@#The expression of p63 was examined in 10 thyroid carcinomas, 20 thyroid papillary carcinomas, 4 thyroid follicular carcinomas, 2 squamous cell carcinomas and 2 medullary thyroid carcinomas using direct immunofluorescence.@*RESULT@#It was shown that p63 expressed in all the thyroid-related diseases mentioned above. In squamous cell carcinoma, p63 has the highest expression and the expression of p63 in thyroid papillary carcinoma has no obvious relationship with the patients age, sex, the size and location of tumor and neoplasm metastasis.@*CONCLUSION@#The p63 masculine stem cells in thyroid could be one of the causes of some thyroid papillary carcinomas and thyroid follicular carcinomas. Thyroid papillary carcinoma, thyroid follicular carcinoma and squamous cell carcinoma may have similar origins.

Clinical analysis of 122 cases of non-thyroid related neck cyst / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate the characteristic of non-thyroid-related neck cyst for the purpose of im proving the accuracy of diagnosis of neck cyst preoperatively.@*METHOD@#Retrospectively analyzed 122 cases of non thyroid related neck cyst during 1999 2005 in our department.@*RESULT@#Congenital mass (n=84) constitute the main part of total cases (n 122), among which thyroglossal cyst is the most common reason. Infective cyst is the second cause of neck cyst (n=30), among which the number of submaxillary gland cyst is 25. Tumor is the most rare cause of neck cyst (n=8). Timing and position of cysts, auxiliary examination of physical sign and puncture cytological analysis lead to more accurate diagnosis preoperatively.@*CONCLUSION@#Accurate diagnosis of neck cyst reduce the blindness and minimize the risk preoperatively.

Management of the sentinel lymph node of papillary thyroid carcinoma in surgery / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate the value of sentinel lymph node of papillary thyroid carcinoma exploring and measuring in the metastasis of the cervical lymph node of thyroid carcinoma.@*METHOD@#The sentinel lymph node were investigated by injecting the methylene blue around 25 cases of thyroid tumors. The lymph nodes dyed in blue and obtained from selective lymph node dissection were examined by pathology, respectively. The value of the sentinel lymph nod were evaluated.@*RESULT@#The sentinel lymph nodes dyed in blue were identified in 22 cases which account for 88.0% of all the 25 cases. Nineteen cases of the sentinel lymph node were positive by pathology, of whom 13 cases were verified positive in other area. Six cases were negative.@*CONCLUSION@#It is of great value that sentinel lymph node of thyroid carcinoma can indicate the metastasis of the cervical lymph node of thyroid carcinoma.