[Clinical Characteristics and Bone Marrow Histopathology Features in Essential Thrombocythaemia Patients with Different Gene Mutation in China].

OBJECTIVE: To investigate the clinical characteristics, laboratorial and bone marrow pathological features of primary thrombocytopenia (ET) patients with different mutations of CALR, JAK2 and MPL genes. METHODS: The chinical data of 120 cases of ET in Jiangsu provincial people's hospital/ The First Affiliated Hospital of Nanjing Medical University from January 2015 to December 2017 were collected and analyzed, including 76 cases with JAK2 gene mutation, 40 cases with CALR gene mutation, 2 cases with MPL gene mutations, 2 cases without gene mutation. RESULTS: Among the ET patients, compared with the JAK2 gene mutation, CALR gene mutation showed statistically significant deareament of white blood cells and hemoglobin (P=0.001, P=0.01) and the male platelets in CALR group showed significant increament (P=0.04). Fourthermore, the average number of megakaryocytes and its cluster numbers in each hight power field of vision showed statistically significant decreament in CALR group as compared with JAK2 group (P=0.001, P=0.001), and thrombotic events in CALR group were signicantly lower than those in JAK2 group (7.5% vs 18.4%) (P=0.03). CONCLUSION: Mutations of CALR, JAK2 have different clinical characteristics and blood pathological changes of Chinese ET patients, and their clinical significance is worth to explore.

[Mucosal malignant melanoma of the nasal cavity and paranasal sinuses: two case reports of nonspecific clinical features].

Mucosal malignant melanoma of the nasal cavity and paranasal sinuses are rare. Because symptoms and radiological examinations of this disease are generally nonspecific, the diagnosis is often difficult. Diagnosis can be confirmed by the immunohistochemical profile of staining with HMB-45 and S-100. This study deals with two cases of nonspecific clinical features. The factors of misdiagnosis were discussed and the lesson was summarized.

[A case of facial palsy as a initial sign of Wegener's granulomatosis].

Wegener's granulomatosis (WG) is a multi-system disease characterized by granuloma formation and necrotizing vasculitis. It typically involves the upper and lower respiratory tract and kidney. Otologic involvement may occasionally be the initial symptom of WG. A case report of WG was described, which first presented as facial nerve palsy. The combination of facial nerve palsy, sensorineural hearing loss and otitis media was unusual. We should raise suspicion. Early diagnosis is vital if unnecessary surgical exploration is to be avoided. The diagnosis of a WG is made clinically based on clinical findings, histologic confirmation and titres of cytoplasmic pattern antineutrophil cytoplasmic autoantibodies (c-ANCA)/ Anti-proteinase 3 (Anti-PR3). Immunosuppressive therapy with steroids, cyclophosphamide (CTX) is required for relief. A delay in diagnosis may lead to devastating sequelae, such as facial nerve palsy and hearing loss. WG is a challenging disease for otorhinolaryngologist.

[Clinical study on acupotomy of occipitalia on the treatment of cervicogenic headache].

OBJECTIVE: To observe the effects of acupotomy on cervicogenic headache and explore the function of cutaneous nerve entrapment in the mechanism of cervicogenic headache. METHODS: From October 2008 to June 2009, 82 patients with cervicogenic headache were treated with acupotomy. There were 23 males and 59 females, ranging in age from 17 to 73 years (averaged 41.57 years). The course of disease ranged from 0.5 to 50 years,with an average of 10.4 years. The location of treatment was occipitalia, both of left and right side: altogether were 8 points, including tenderness point of postmastoid; the mid-point between mastoid and C2 spinous process; the internal 1/3 attachment between occipital protuberance and mastoid process; the posterior midline of C2 spinous process open to 1.5-2 cm. The chief complaint of all patients was headache. PPI assessment rating was observed. RESULTS: The total effective rate at 1 month after treatment was 81.70% (67/82). Recurrence of headache within 3 months after treatment was obvious. However, 17.07% (14/82) patients did not reoccur at 6 months after treatment. CONCLUSION: 17.07% patients with cervicogenic headache recovered by acupotomy, so it shows cutaneous nerve entrapment plays an important role in the mechanism of cervicogenic headache. For the patients whose headache recurred at 3 months after treatment, increasing the treatment time and therapeutic range is suggested.

[Comparative observation of efficacy on lumbar disc herniation treated with acupotomology and operation].

OBJECTIVE: To compare the efficacy difference between acupotomology and operation in the treatment of lumbar disc herniation (LDH). METHODS: One hundred and eighty-four cases were randomized into an acupotomology group (109 cases) and an operation group (75 cases). In acupotomology group, acupotomology was applied to relevant local soft tissues in LDH and the reactive points in the distribution of superior gluteal nerves, at the outlet of sciatic nerve and in the nerve innervate area of the lower extremities separately. In operation group, the small-windowed discectomy through small incision was adopted. The clinical efficacies were assessed between two groups. RESULTS: In acupotomology group, the clinical cured rate was 82.6% (90/109) and the effective rate was 96.3% (105/109). In operation group, they were 86.7% (65/75) and 97.3% (73/75) respectively. There were no significant differences in statistics between two groups (both P > 0.05). CONCLUSION: Acupotomology is definitely effective in the treatment of lumbar disc herniation and its efficacy is similar to that of operation.

[A comparative study of complications in transoral CO2 and Nd:YAG laser surgery for glottic carcinoma].

OBJECTIVE: To compare the complications in transoral CO2 and Nd:YAG laser surgery for the treatment of laryngeal carcinoma. METHODS: Retrospective analysis of 83 cases of glottic laryngeal carcinoma treated with laser surgery from January 1, 1999 to December 31, 2008 was carried out. Thirty-two cases were treated with the CO2 laser, including Tis (2 cases), T1N0M0 (21 cases), T2N0M0 (8 cases), and T3N0M0 (1 case). Fifty-one cases were treated with the Nd:YAG laser, including Tis (3 cases), T1N0M0 (36 cases), T1N2M0 (3 cases), and T2N0M0 (9 cases). RESULTS: Four complications (12.5%) occurred in the CO2 laser group. There was 1 local infection (3.1%), 1 numbness of the tongue (3.1%), 1 odontoseisis (3.1%), and 1 subcutaneous emphysema (3.1%). Twenty-seven complications (52.9%) occurred in 19 patients in the Nd:YAG laser group. There were postoperative bleeding 2 (3.9%), dyspnea 5 (9.8%), local infection 7 (13.7%), aspiration pneumonia 4 (7.8%), numbness of the tongue 2(3.9%), pharyngeal cutaneous fistula 1 (2.0%), vocal cord fixation 4 (7.8%), and laryngostenosis 2 (3.9%). CONCLUSION: More complications were observed in the patients with Nd:YAG laser surgery when compared to the patients with CO2 laser surgery.

[Surgical treatment of lymphatic malformation located in head and neck in children].

OBJECTIVE: To evaluate the surgical approaches and therapeutic effect of lymphatic malformations located in head and neck in children. METHODS: Eleven cases of lymphatic malformations in the region of head and neck in children encountered between Jan. 1998 and Dec.2008 in Peking University First Hospital were retrospectively analyzed. Initial diagnosis was made based on the physical examination and then confirmed by MR and Enhanced CT imaging. Surgical therapy was used for patients with lymphatic malformation which exceeds 4 cm. The operative technique was as follows: mass resection and superficial parotidectomy (4 cases), mass resection and total parotidectomy (2 cases), mass resection with neck dissection (2 cases), mass resection with neck dissection and sternotomy (1 case), marginal mandibular branch of facial nerve dissection and mass resection (2 cases). Dissection outside the false capsule was applied during the operation and facial nerve was dissected from bole to terminal arborization. RESULTS: The mass was completely removed in all 11 cases without organ dysfunction and obvious disfigurement. The cure rate was 100%. Three cases suffered from a branch of facial nerve paralysis because of tension and 1 case had a Horner's syndrome after operation. One case needed a blood transfusion (150 ml) during the operation. All cases have been followed up with excellent results from 6 to 121 months, 32 months of the median, no mass recurrence. CONCLUSIONS: Dissection outside the false capsule of mass and dissection of facial nerve were applied in the surgical treatment of huge lymphatic malformations. These methods are effective in the preservation of function and avoidance of abnormality.

[Study on hemorrhage following coblation assisted uvulopalatopharyngoplasty].

OBJECTIVE: To compare the postoperative hemorrhage between standard uvulopalatopharyngoplasty (UPPP) and coblation assisted UPPP, and to evaluate the related risk factors and preventive measures. METHODS: Five hundreds and ninety seven patients with obstructive sleep apnea hypopnea syndrome (OSAHS) underwent UPPP and coblation assisted UPPP between January 1, 1999, and September 30, 2009 were reviewed retrospectively. Two hundred and sixty three patients with coblation assisted UPPP and 334 patients with standard UPPP were treated respectively. Single factor statistic analysis, multiple factors Logistic regress statistic analysis and Wilcoxon test method for related risk factors were applied. RESULTS: A total of 42 patients (7.0%) experienced postoperative bleeding. Among them, 24 patients with coblation assisted UPPP (9.1%) and 18 patients with UPPP (5.4%) had postoperative hemorrhage. Significant difference was not found in the degree of hemorrhage (z = 0.784, P > 0.05), hemorrhage site(χ(2) = 1.387, P > 0.05) and postoperative hemorrhage rates (χ(2) = 3.14, P > 0.05) between the two surgical techniques. Significant difference was found in the interval of hemorrhage after surgery between the two surgical techniques (χ(2) = 9.25, P < 0.01). History of hypertension, smoking, hepatic dysfunction was found to be correlated with the postoperative hemorrhage (Odd-ratio were respectively 7.326, 3.674, 2.707). CONCLUSION: Coblation technique did not significantly increase UPPP postoperative hemorrhage.

[Selective neck dissection on the treatment of neck metastases in 63 patients with squamous cell carcinoma of hypopharynx].

OBJECTIVE: To analyze the prevalence and distribution of cervical node metastases in squamous cell carcinoma of hypopharynx and evaluate the efficacy of selective neck dissection (SND) on the treatment of the neck metastases. METHODS: A retrospective review was performed for 63 patients with squamous cell carcinoma of hypopharynx from January 1990 to December 2004. Lymph nodes were clinically negative in 17 patients and positive in 46 patients. There were 15 unilateral SND, 22 bilateral SND and 16 modified radical neck dissections (MRND). Ipsilateral MRND or radical neck dissections (RND) and contralateral SNDs were performed in 10 cases. Forty-eight patients were followed up and the follow-up interval ranged from 24 to 143 months and the median was 41 months. RESULTS: Lymph nodes were pathologically negative (pN0) in 22 and positive (pN +) in 41 patients. A total of 106 positive nodes were found in 95 neck dissections. The distribution of the positive nodes were as follows: Level II 47.2% (50/106), Level III33.0% (35/106), Level IV11.3% (12/106), Level V2.8% (3/106), Level VI 5.7% (6/106). Contralateral nodal metastases and recurrence were observed in both cN0 and cN + cases. There were 18 neck recurrent cases. High incidence of neck recurrence after SND was found in level II and III. The 3-year estimated survival rate analysis were 58.1% for the pN0 and 44.9% for pN1 and 41.1% for pN2 patients. Cox regression analysis suggested N stage was the most important prognostic factor. The risk ratio was 1.7 in pN1 and 2.2 in pN2. CONCLUSIONS: Lymphatic metastases was the most significant prognostic factor of hypopharyngeal squamous cell carcinoma. Bilateral selective neck dissection, when carefully indicated, offers functional advantages without oncologic compromise.

[Otologic manifestations of Wegener's granulomatosis].

OBJECTIVE: To evaluate the clinical features, pathologic diagnosis and laboratory test of otologic manifestation in Wegener's granulomatosis (WG). METHODS: Fourteen eases of WG with ear involvement were reviewed. The clinical course, method of treatment and outcomes in all cases were evaluated. Diagnosis of WG was made when the patients had clinical findings, clear histologic findings and a positive titer of antineutrophil cytoplasmic autoantibodies (ANCA). RESULTS In all 14 cases, the most frequent finding was chronic otitis media Sensorineural hearing loss was present in 1 case and facial nerve paralysis was present in 2 cases separately. There were 5 patients whose otologic manifestations were the primary involvement of WG. Fourteen cases had positive histologic diagnosis of WC while 13 cases were confirmed positive for ANCA. All patients were treated with glucocorticoids, immunosuppressive drugs, and finally got marked improvement. CONCLUSIONS: WG should he included in the differential diagnosis in cases of atypical inflammatory states of the ear, facial nerve paralysis and sensorineural hearing loss. Early diagnosis was made basing on the histologic findings and a positive titer of ANCA. Appropriate treatment were important to improve the otologic manifestations of WG.

[Managements of masked mastoiditis].

OBJECTIVE: To appreciate clinically of masked mastoiditis to explored how to reduce the incidence and associated morbidity of otogenic complications. METHODS: Between January 1998 and February 2005, 11 cases of masked mastoiditis were collected retrospectively. Their clinical presentation, results of investigations, and response to treatment were reviewed. RESULTS: Of 11 cases, there were 8 cases with hearing impairment, 5 cases with otalgia, 4 cases with facial nerve palsy, one patient with postauricular subperiosteal abscess, one case with meningitis, and one with thrombosis of the lateral sinus. Computed tomography (CT) scan revealed blurring (haziness) of the mastoid air-cells. After admission, intravenous antibiotics were prescribed and antro-mastoidectomy or mastoidectomy was performed for eradication of infection source. The predominant finding at mastoidectomy was granulation tissue filling the mastoid cavity and antrum. A varying amount of pus and osteitis was found in the 5 cases. The granulations into the antrum were severe, obstructing the drainage into the attic and the middle ear. The mastoid tip cells were filled with granulation tissue which spared the antrum. The patients recovered excellently postoperative, without facial palsy, vertigo or other complications. CONCLUSIONS: To be a contemporary otologist, such severe complications of otologic diseases should not be overlooked. Appropriate intravenous antibiotics and adequate surgeries, as soon as possible, were recommended. Advanced CT scans of the temporal bone were necessary. Failure to identify associated concomitant pathology might result in treatment failure or persistent neurological deficit.

[CO2 laser endoscopic subtotal arytenoidectomy for bilateral median vocal cord paralysis].

OBJECTIVE: To delineate the surgical procedures and correlated techniques for endoscopic subtotal arytenoidectomy, as well as to discuss their applications and clinical outcomes. METHODS: CO2 Laser endoscopic unilateral arytenoidectomy was performed in eight cases of bilateral median vocal cord paralysis combined with one stage of mucosal micro-anastomosis. All patients suffered from dyspnea in some extent, of which 5 had the history of thyroidectomy and 2 had traumatic causes following esophagectomy and tracheal surgery respectively. One of patient had unknown cause. Six patients had undergone tracheotomy prior to operation or before their referral to our hospital. The airway was evaluated via fibro-optic laryngoscopy, and the voice quality was assessed subjectively by the patients and the surgeon before and after surgery. RESULTS: Following 5-43 months after the surgery, in all cases the function of airway as well as the acceptable voice quality was successfully restored. The tracheotomy done before operation in six patients was decannulated within the mean time of 44.2 days post-operation. CONCLUSION: The endoscopic approach for CO2 laser unilateral arytenoidectomy may lead to better restoration of an adequate airway and satisfying phonation without postoperative aspiration. Mucosal micro-anastomosis can prevent the formation of granulation or scar tissue thus promotes the healing processes. This procedure is simpler than other ordinary surgical methods, and could be a satisfactory alternation of treatment for bilateral median vocal cord paralysis.