To investigate the clinical manifestation, clinicolpathological diagnosis and treatment of Castleman disease (CD) in the neck. The data of 2 patients with CD in the neck were reviewed retrospectively, together with literature summary, in order to analyze its clinical manifestation, pathological characteristics, clinical and pathological types, imaging characteristics and treatment. Two cases presented as solitary, painless neck masses and met the criteria of localized CD, and also had histological evidence of the hyaline vascular variant. Complete surgical resection was the first choice of treatment. One patient was alive without evidence of recurrence for ten years. The another patient had masses recurred in the neck one month after operation and followed to undergo curative chemotherapy, no recurrence in one-year follow-up. CD in the neck is a rare disease. The diagnosis for the disease is mainly based on pathological examination. Surgical resection is the main treatment, if necessary, need combination with radiotherapy and chemotherapy.
Castleman Disease ; Child ; Female ; Humans ; Male ; Middle Aged ; Neck

Objective To explore the deafness-causing underlying mechanisms of CX26 gene recessive mutations through functional analyzing nine missense mutations (p.S19T,p.R32H,p.E47K,p.V84L,p.V95M,p.R143W,p.R165W,p.S199F,p.L214P) in exogenous expression system Hela cells.Methods The nine recessive missense mutations of CX26,which are in the different domains of CX26 protein,and the wild type CX26 were subcloned into pEGFP-N1 vector directively,following to transfect into HeLa cells by the liposome complex method.The expressions of the mutated proteins were analyzed using western blot method.The localizations of the mutated proteins and whether there were gap junction-plaques formation were observed under confocal microscopy with immunofluorescence technique.Results The nine constructs were all expressed in HeLa cells.In which,the mutated proteins of p.S19T,p.E47K,p.V84L,p.V95M and p.R165W localized at the cytoplasmic membrane of HeLa cells and formed gap junction-plaques at contact points between two cells,and the mutated proteins of p.R32H,p.R143W,p.S199F and p.L214P accumulated and localized only intracellularly and did not form gap junction-plaques on cell membrenes.Conclusion The mutations of p.S19T,p.E47K,p.V84L,p.V95M and p.R165W do not interfere the mutated connxins trafficking and inserting into the plasma membrane.The mutations of p.R32H,p.R143W,p.S199F and p.L214P impared the proteins trafficking to the cell surface.The deafness-causing mechanisms of different missense mutations might not be identical and no correlation could be observed between the mutation and the topological domain of the mutated protein.

BACKGROUND: Rat models induced by unilateral nephrectomy combined with adriamycin are widely used in screening anti-drugs of glomerular sclerosis. However, few reports concerning this method on inducing rabbit model of glomerular sclerosisOBJECTIVE: To establish a rabbit model of glomerular sclerosis, and to observe the renal function and histopathological changes during model preparation. DESIGN, TIME AND SETTING: Randomized controlled animal experiment was performed in Laboratory Animal Center of Kunming General Hospital of Chengdu Military Region between December 2008 and May 2009. MATERIALS: Twenty-five Japanese big-ear rabbits, weighing 1.75-2.25 kg, half males and half females, were selected to establish glomerular sclerosis models, and randomly divided into normal (n=10) and model (n=15) groups. METHODS: The left kidney of rabbits in the model group was removed under anesthetized with 30g/L saline solution of sodium pentobarbital (1 mL/kg). Rabbits in the normal group underwent a similar surgical procedure without kidney removing. At 1 week after operation, 5 mg/kg adriamycin was injected into rabbits in the model group, 3 mg/kg adriamycin was reinjected 2 weeks later. Same volume of physiological saline was injected in the normal group. MAIN OUTCOME MEASURES: The renal blood biochemical indexes were detected prior to and at weeks 4, 6, and 8 after model preparation. One nephridial tissue was harvested from each group to undergo pathological observation at week 8 after the second medication. RESULTS: ①Kidney in the normal group presented slightly white, slightly tough texture with smooth surface. ②Pathological features of focal segmental glomerulosclerosis was showed in the model group under a light microscope, presented as extracellular matrix hyperplasia, mesangial region expansion, renal glomerular capillary wall ball mix adhesion, tubule degeneration, or even interstitial fibrosis, as well as interstitial infiltration of inflammatory cells. ③Compared to the normal group, the total protein and albumin was and decreased, triglyceride, total cholesterol, low density lipoprotein cholesterol, urea nitrogen, and creatinine were significantly increased in the model group. The urine protein content of rabbits in the model group was obviously increased at 4 weeks, gradually reached a platform after 8 weeks, which still greater than that of the normal group. ④SPECT showed that the glomerular filtration rate of the model group was notably decreased (33 mL/min) than the normal group (92.6 mL/min).CONCLUSION: The unilateral nephrectomy combined with adriamycin in rabbits results in the formation of glomerular sclerosis, renal function and 24 h urinary protein is more visible in the phase change.

Objective To investigate the affecting factors on auditory and speech performances in preschool children with unilateral cochlear implantation (CI) .Methods The clinical data of the preschool children (n=165) with unilateral cochlear implantation in the Second Xiangya hospital from January 2006 to April 2013 were collected . These children received rehabilitation according to the method recommended by the China Rehabilitation Research Center for Deaf Children ,and the data were analyzed retrospectively .The categories of auditory performance (CAP) and speech intelligibility rating (SIR) were used to assess their auditory and speech performances .The relationships between the performance and gender ,implanted age ,genotype ,inner ear malformation ,history of hearing aid were evaluated .Results Implanted ages and genotypes were associated with the auditory and speech performance of par‐ticipants (P<0 .05) ,while genders ,hearing aid experience ,and inner ear malformations(enlarged vestibular aque‐duct syndrome ,EVAS)were not significant related (P<0 .05) .Children were found to have achieved better CAP and SIR growths when CI was implanted during 1~3 years old and 2~4 years old ,respectively (P<0 .05) .The outcomes of CI recipients with GJB2 mutation were significantly better than those of the GJB2-nonrelated CI recipi‐ents (P<0 .05) .Conclusion This study provides evidence that CIs during first 1~3 years old having better auditory rehabilitation results than those of during 4~6 years old ,and CIs during 2~4 years old obtaining a better speech development in the first 12 months after operation .Deaf children with GJB2 mutation show better auditory and speech performances after CIs than those of the peers without GJB2 mutation .CIs can be effectively performed in deaf children associated with EVAs as in those without EVAS .

Child ; Cord Blood Stem Cell Transplantation ; methods ; Histocompatibility Testing ; standards ; Humans ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; therapy ; Transplantation, Homologous ; methods ; Treatment Outcome

OBJECTIVE: To investigate the expressions of tumor suppressor gene CX26 mRNA and coding protein in laryngeal squamous cell carcinoma, and to explore the relationship between CX26 gene and the biological behaviors of laryngeal squamous cell carcinoma for understanding the tumorigenicity and development of laryngeal squamous cell carcinoma. METHOD: Laryngeal carcinoma tissues (studying group), which takeda from the center of tumors and laryngeal normal tissues (control group) takeda at the place of 1.0 cm out of the edge of the tumors, were took from 38 patients with laryngeal squamous cell carcinoma while they were in operation. Semi-quantitative reverse transcriptase polymerase chain reaction (RT-PCR) was used to analyze the expression level of CX26 mRNA, and immunohistochemical staining (frozen section) was used to detect the expression of CX26 protein in laryngeal carcinoma tissues and laryngeal normal tissues of 38 cases, respectively. RESULT: mRNA of CX26 gene was all positively expressed in laryngeal carcinoma tissues and laryngeal normal tissues of 38 cases by RT-PCR. However, CX26 mRNA was obviously down-regulated in laryngeal carcinoma tissues than that in laryngeal normal tissues (P < 0.05). Immunohistochemical staining showed CX26 protein was strong-positively expressed in laryngeal normal tissues in 34 cases (89.5%), while it was positively expressed in laryngeal carcinoma tissues in 18 cases (47.4%), and with the location alteration of CX26 protein in laryngeal carcinoma cells. There was significant difference between the expression rate of CX26 protein in laryngeal carcinoma tissues and in laryngeal normal tissues (P < 0.05). Meanwhile, the expression level of CX26 mRNA and the positive-expressed rate of CX26 protein of the laryngeal carcinoma tissues in the advanced stage patients group (III stage and IV stage) were significantly lower than these in the early stage patients group (I and II) (P < 0.05), and it was significantly lower in those who have a cervical lymph node metastasis than those without metastasis. (P < 0.05). Moreover, the expression level of CX26 mRNA and the positive-expressed rate of CX26 protein reduced along with the reduction of pathological differentiation, and there was significant difference among the well-differentiated group, moderately-differentiated group and poorly-differentiated group (P < 0.05). CONCLUSION CX26 gene may play an important role in the pathogenesis and development of laryngeal carcinoma and may be related to its prognosis.
Adult ; Aged ; Carcinoma, Squamous Cell ; metabolism ; pathology ; Connexin 26 ; Connexins ; metabolism ; Humans ; Laryngeal Neoplasms ; metabolism ; pathology ; Male ; Middle Aged ; Neoplasm Staging ; RNA, Messenger ; genetics

OBJECTIVE: To summarize the clinical features and the experiences of diagnosis and treatment of extramedullary plasmacytomas (EMPs), and to enhance the cure rates of EMPs. METHOD: Clinical data of 8 patients with EMPs in head and neck, who treated in our hospital from Jan. 1990 to Dec. 2004, were reviewed. Of 8 cases, 3 cases with the tumors occurred in nasal cavities, 1 in maxillary sinus, 2 in nasopharynx, 1 in posterior wall of oropharynx. RESULT: Eight patients who were all pathologic confirmed EMP accepted surgical resections of the tumors, and 4 of 8 cases were boosted radiation therapy post-operation. Four cases were still alive disease-free for more than 1, 5, 10 and 12 years after treatments, respectively. One died of local recurrence in 2 years, 1 died of multiple myeloma in 3 year, and 1 died of heart attack in 2 years after treatments, respectively. One lost follow-up. CONCLUSION EMPs in head and neck are low potential malignancy tumors. The diagnosis of EMPs mainly depends on clinical manifestations and pathological results. Surgery and radiation therapy are the main treatments for EMPs in head and neck.
Adult ; Aged ; Female ; Head and Neck Neoplasms ; diagnosis ; therapy ; Humans ; Male ; Middle Aged ; Plasmacytoma ; diagnosis ; therapy ; Retrospective Studies