Cephalometric craniofacial features of patients with Sagliker syndrome: a primary analysis of our experience.

BACKGROUND: Sagliker syndrome (SS) is characterized by a severe uglifying facial appearance resulting from untreated or inadequately treated secondary hyperparathyroidism (SHPT). To date, the craniofacial morphology of patients with SS has yet to be analyzed. The present research sought to cephalometrically evaluate the craniofacial features of patients with SS and to perform an in-depth analysis of their serum biochemical parameters, with the aim of furthering the theoretical basis for the early diagnosis and prevention of SS. METHODS: A retrospective chart review of 9 patients who fulfilled the diagnostic criteria for SS were included in this study, and their serum biochemical parameters were collected. After subjecting standard lateral cephalometric radiographic images to correction for distortions caused by magnification followed by digitization, we conducted a cephalometric analysis. Student's two-tailed t tests or Mann-Whitney U tests were used to analyze the data. Thirty-three patients with patients with SHPT alone were also included as controls. RESULTS: The lower anterior facial height (ANS-ME) and total anterior facial height (N-Me) measurements of patients with SS were significantly increased compared to those of the controls. The angles between the Frankfort horizontal, palatal, and occlusal planes and the mandibular plane, were greater in the SS group than in the control group, as was the gonial angle. Patients with SS also exhibited a significantly larger maxillary protrusion angle and relative position of the maxilla to the mandible. Most patients with SS had class II malocclusion, whereas most of the controls exhibited normal occlusion. Soft tissue largely followed the same pattern as craniofacial changes. Our investigation also showed that among patients with SHPT, female sex, longer duration of dialysis, and higher serum levels of alkaline phosphatase and intact parathyroid hormone were associated with development to SS. CONCLUSIONS: Patients with SS show facial and biochemical differences compared to patients with SHPT. Female sex, long dialysis duration, and high serum levels of intact parathyroid hormone and alkaline phosphatase may be potential risk factors for SS.

Retrospective analysis of prognostic factors in 205 patients with laryngeal squamous cell carcinoma who underwent surgical treatment.

OBJECTIVES: To investigate the most important factors affecting the prognosis of the patients with squamous cell carcinoma (SCC) of the larynx. METHODS: Based on the clinical and follow-up data, 205 patients with SCC of the larynx receiving total laryngectomy, partial laryngectomy, or CO2 laser surgery in GuangDong General Hospital were retrospectively analyzed. A survival analysis was performed by the Kaplan-Meier method and a multivariable analysis of prognostic factors was carried out using the Cox proportional hazard model. RESULTS: Subtypes of carcinoma included 69.8% glottic and 30.2% supraglottic. Most patients were in N0 stage (77.6%), and 22.4% patients were in N1∼N3 stage. Over half of the patients were in T1∼T2 stage (55.1%), 20.0% in T3, and 24.9% in T4. Mean follow-up duration was 49.2 months. The survival rates 1, 2, and 3 years after the surgery were 99.0%, 91.7%, and 81.5%, respectively. The survival rate for those patients with clinical stage IV was significantly lower than for those with clinical stage I and II (p<0.001 and p = 0.013, respectively). The disease-free progression rates 1, 2, and 3 years after the surgery were 83.9%, 74.6%, and 71.2%, respectively. Futhermore, those patients with a Charlson score of 1 to 2 and ≥3 had higher risk of mortality than those with a Charlson score of 0 (hazard ratios of 1.8 and 2.41 p = 0.042 and p = 0.008). Multivariable analysis revealed that clinical stage, surgical margin, and comorbidity were significantly associated with both mortality and disease-free progression. CONCLUSION: The surgical resection margin, clinical stage, and comorbidity were independent factors affecting the laryngeal cancer prognosis. The survival rates were lower for patients with advanced laryngeal cancer, positive surgical margins, or severe comorbidity, suggesting the importance of early diagnosis, early treatment, negative surgical margins, and conditions of comorbidity.

Supracricoid partial laryngectomy cricohyoidoepiglottopexy (SCPL-CHEP) versus vertical partial laryngectomy for the treatment of glottic carcinoma.

The study aimed to compare the survival rate and functional outcome of supracricoid partial laryngectomy cricohyoidoepiglottopexy (SCPL-CHEP) and vertical partial laryngectomy (VPL) in the treatment of glottic carcinoma. Thirty-nine patients underwent SCPL-CHEP and 45 patients underwent VPL between 2002 and 2007. Post-operative clinical staging were performed according to the UICC staging criteria (2002). The primary endpoints were survival time and recurrence rate. The secondary endpoints were the laryngeal functions including pronunciation, swallowing, and respiration. Overall, the estimated mean survival time was 75.6 months. There was no significant difference in survival times between the two groups (P = 0.496). The SCPL-CHEP group had a significantly lower post-operative local recurrence rate than VPL group (2.6 vs. 17.8 %, P = 0.033). Significantly longer nasogastric tube removal (22 vs. 14 days, P < 0.001) and tracheostomy decannulation (38.5 vs. 15 days, P < 0.001) times in SCPL-CHEP group was observed with the VPL group, respectively. Significantly higher aspiration rates were observed at 3, 6, and 12 months in the SCPL-CHEP group compared with the VPL group (P < 0.001, P < 0.001, P < 0.05, respectively). Although the survival rate was not significantly different between the two groups, the local regional control rate and organ preservation in the supracricoid partial laryngectomy cricohyoidoepiglottopexy group was better than those observed in the vertical partial laryngectomy group.

[Activity of bilateral posterior cricoarytenoid muscle satellite cell after denervation or reinnervation with ansa in dogs].

OBJECTIVE: To investigate the activity of bilateral posterior cricoarytenoid muscle satellite cell after denervation or reinnervation with ansa cervicalis. METHODS: Twenty four dogs were randomly divided into 3 groups. The bilateral laryngeal recurrent nerves were cut in group one in all dogs. The bilateral laryngeal recurrent nerves were anastomosed with ansa cervicalis after incision in group two in all dogs. The dogs in group three were used as control. Nine weeks after surgery, the electromyography was used to test the regeneration of the nerve. The posterior cricoarytenoid muscles biopsy were collected. The expression of mRNA of Myogenin, Myf5, and Pax7 was assayed by realtime RT-PCR after total RNA isolation. RESULTS: Two dogs died after surgery in incision and anastomose group. The electromyography suggested that the RLN of all dogs had denervated in the incision group and had reinnervated in the anastomose group after 9 weeks. Myogenin mRNA from RLN incision dogs PCA muscles had greater expression versus controls (Z = 1.42, P < 0.01) or anastomosed dogs (Z = 1.38, P < 0.01). Myf5 mRNA expression from RLN incision dogs PCA muscles had significant increase versus control dogs (Z = 1.66, P < 0.01) or anastomosed dogs (Z = 1.69, P < 0.01). Pax7 mRNA expression from RNL incision dogs had significant increase compared with control (Z = 1.66, P < 0.01) or anastomosed animals (Z = 1.42, P < 0.05). There was no significant difference in Myogenin (Z = 1.34, P > 0.05), Myf5 (Z = 0.54, P > 0.05) and Pax (Z = 0.54, P > 0.05) mRNA expression between controls and anastomosed animals. CONCLUSIONS: The bilateral denervation of RLN cause significantly increasing in dog PCA muscle satellite cell proliferation and differentiation. The bilateral reinnervation of RLN cause PCA muscle satellite cell come back nonproliferative, quiescent state in dog.

Model of evoked rabbit phonation.

OBJECTIVES: We describe a method for eliciting phonation in an in vivo rabbit preparation using low-frequency, bipolar pulsed stimulation of the cricothyroid muscles with airflow delivered to the glottis. METHODS: Ten New Zealand White breeder rabbits weighing 3 to 5 kg were used in this study. The cricothyroid muscles were isolated bilaterally, and separate pairs of anode-cathode hooked-wire electrodes were inserted into each muscle. A Grass S-88 stimulator and 2 constant-current PSIU6 isolation units were used to deliver bipolar square wave pulses to each cricothyroid muscle, with airflow delivered to the glottis through a cuffed endotracheal tube. RESULTS: Phonation was evoked with a 50-Hz, 4-mA stimulus train of 1-ms pulses delivered to each cricothyroid muscle. The pulse trains were on for 2 seconds and were repeated every 5 seconds over a period of 180 minutes. Airflow was delivered at 143 cm3/s, producing phonation measuring 71 to 85 dB sound pressure level. CONCLUSIONS: Evoked phonation is feasible in rabbits by use of bipolar stimulation of the cricothyroid muscles with airflow delivered to the glottis. The in vivo rabbit preparation described may provide a useful small animal option for studies of evoked phonation. From the level and consistency of the adduction observed, we hypothesize that current spreading to the underlying adductor muscles and nerves resulted in neural pathway involvement beyond discrete activation of the cricothyroid muscle, providing sufficient approximation of the vocal folds for phonation.

Extracellular matrix gene expression after vocal fold injury in a rabbit model.

OBJECTIVES: We determined the expression of matrix metalloproteinase (MMP)-1, MMP-9, collagen types I and III, and fibronectin from rabbit vocal folds after injury. METHODS: Thirty rabbits were involved in this study. Five animals were assigned to each time period. Noninjured vocal fold specimens were collected as a control. Gene expression was analyzed at 2, 4, 8, 24, 48, and 72 hours after injury by use of real-time polymerase chain reaction. RESULTS: Compared to 2 hours after injury, MMP-1 expression was increased at 4, 8, 24, 48, and 72 hours. Compared to 4 hours, MMP-1 expression was increased at 8, 24, 48, and 72 hours. Compared to the control specimens, MMP-9 expression was increased at 4, 8, 24, 48, and 72 hours. Compared to 2 hours, MMP-9 expression was increased at 4, 8, 24, 48, and 72 hours. Compared to 2 and 4 hours, collagen type I expression was increased at 72 hours. Collagen type III expression was increased at 72 hours compared to 2, 4, and 8 hours. Compared to 2 hours, fibronectin expression was increased at 24, 48, and 72 hours. CONCLUSIONS: Results revealed time-dependent changes in expression of MMP-1, MMP-9, collagen types I and III, and fibronectin from rabbit vocal folds after injury. Future experiments are planned to investigate the effects of phonation on expression of these genes after injury.

[Effects of CD4+ T cell transplantation on facial motoneuron survival in nude mice model with facial nerve axotomy].

OBJECTIVE: To investigate the specific T cell subpopulation and the relationship with facial motoneuron in immune deficiency mouse model with facial nerve paralysis, so as to find information for new strategy of facial palsy treatment. METHODS: Firstly, purifying the CD4+ T cell from wild type mouse and reestablishing the immune function of nude mouse by infusing the CD4+ T cell through the tail vein a week before the surgery. Then the all nude mouse (BALB/c background) and wild type mouse (BALB/c background) were subjected to a right facial nerve axotomy. Then the mouse was studied by application and assessment with fluorogold retro tracer at specific time. After collecting the slices of brain stem three days post the operation, the facial motoneurons was observed under fluorescence microscope, then analyzed and counted with the software Image Pro Plus5. 1. RESULTS: The number of survival facial motoneuron in the group with CD4+ T cell transplantation and control group was (3444.5 +/- 84.2, x +/- s) and (3013.2 +/- 65.3) respectively. There was significant difference of the number of survival facial motoneurons between nude mouse transplanted with CD4+ T cell and PBS at three days post the operation (t = 5.52, P = 0.0003). But there was no significant difference of survival facial motoneurons between nude mouse transplanted with CD4+ T cell and wild type mouse three days post the operation (t = 0.49, P = 0.6347). It was the transplantation of CD4+ T cell that rescued the survival facial motoneuron to the level of wild type. CONCLUSIONS: CD4+ T cell have the ability to rescue the injuring facial motoneuron from death. It may suggest that there is a critical role of the specific T cell subpopulation in facial nerve repair and regeneration.

[Pharyagoesophageal sphinoter myotomy for voice rehabilitation after total laryngectomy].

OBJECTIVE: In those patients received total laryngectomy, Blom-Singer prosthesis speech appears to be the more effective form of rehabilitation. To reduce the effect of pharyngoesophageal sphincter on the Blom-Singer prosthesis voice restoration following total laryngectomy, the pharyagoesphageal sphincter myotomy was designed and carried out. METHODS: After total laryngectomy, the cricopharyngeus muscle stricture or spasm appears to inhibit the air flowing and prevent the speech. The pharyngoesophageal sphincter is now defined as the area including the cricopharyngeus with a portion of inferior constrictor, as well as some of the upper cervical esophagus. The pharyngoesophageal sphincter were incised intra- and post- operation of total laryngectomy, the dissection was approximately 5 cm in vertical length and 1 cm in width. RESULTS: Among the 33 patients who received pharyngoesophageal sphincter myotomy following total laryngectomy, voice restoration was achieved in 32 cases, the success rate was 97%. 25 patients had survived for 3 years, 18 patients survived for 5 years. CONCLUSION: Pharyagoesophageal sphincter myotomy can raise the success rate of rehabilitation of the voice by Blom-Singer prosthesis.

[The use of muscle autograft denatured by microwave for repair of gaps in removal of facial neuromas].

OBJECTIVE: To study the clinical value of muscle autograft denatured by microwave for repair of gaps in removal of facial neuromas. METHODS: Two cases of patients with facial nerve Schwann cell neuromas were reported. The operations for removal of facial neuromas were completed, and the gaps of the nerves were repaired with muscle autograft denatured by microwave of 250 W for 120 sec. RESULTS: The patients were followed up for two years, and the recovery of facial function on the affected sides were satisfactory. CONCLUSION: Muscle autograft denatured by microwave technique is convenient, highly efficient for repairing facial nerve gap after removal of facial neuroma.

[Serum levels of macrophage colony stimulating factor in the patients with Alzheimer's disease].

OBJECTIVE: To investigate the availability of serum level of macrophage clony stimulating factor (M-CSF) as a marker for early diagnosis of Alzheimer's disease (AD). METHODS: The serum levels of M-CSF in 70 patients with AD, 52 healthy controls, 22 patients with VAD (vascular dementia) were measured and the serum levels of IL-1 beta, IL-6, TNF-alpha in 32 patients with AD and 20 controls were measured as well. RESULTS: Serum levels of M-CSF were significantly elevated in patients with AD when compared with healthy controls (P < 0.01) and VAD controls (P < 0.05) respectively. At the early stage of mild dementia and middle dementia, serum levels of M-CSF were significantly elevated, but at the later stage of severe dementia, they returned to normal level. Serum levels of IL-1 beta were significantly elevated in AD patients compared with controls (P < 0.05), and serum levels of TNF-alpha and IL-6 were within the normal range in patients with AD. CONCLUSIONS: The results suggest that serum M-CSF level may provide a convenient and sensitive means for the early diagnosis of Alzheimer's disease.

[The use of muscle autograft denatured by microwave for repair of gap in removal of facial neuroma].

OBJECTIVE: To study the clinical value of muscle autograft denatured by microwave for repair of gap in removal of facial neuroma. METHODS: A case of patient with a larger facial nerve Schwann cell neuroma was reported. Based on the good results of our experimental research on rats recently, we completed the operation by transmastoid approach for removal of facial neuroma, and the gap of the nerve was repaired with muscle autograft denatured by microwave. RESULTS: The patient was followed up for one year, and her facial function recovery on the affected side was satisfactory. CONCLUSION: Muscle autograft denatured by microwave technique is convenient, highly efficient for repairing facial nerve gap after removal of facial neuroma.