半规管阻塞术与迷路切除术治疗70例晚期顽固性梅尼埃病的疗效

目的 比较半规管阻塞术与迷路切除术治疗晚期顽固性梅尼埃病的远期疗效。 方法 回顾性分析2015年3月至2016年3月接受半规管阻塞术(n=56)和迷路切除术(n=14)治疗的70例晚期顽固性梅尼埃病患者的临床资料,比较半规管阻塞术(半规管阻塞术组)和迷路切除术(迷路切除术组)两种手术方式的治疗效果。 结果 半规管阻塞术组眩晕总控制率为96.4%(54/56),其中A级控晕率为80.4%(45/56)、B级控晕率为16.1%(9/56);迷路切除组眩晕总控制率为100%(14/14),所有患者均为A级控制;两组眩晕控制有效率差异无统计学意义(P>0.05)。半规管阻塞术组听力下降率为28.6%(16/56),迷路切除组听力损失率为100%(14/14),半规管阻塞术组听力损失率低于迷路切除组(P<0.001)。半规管手术组手术前、后颈源性/眼源性前庭肌源性电位(c/oVEMP)的异常率差异无统计学意义(P均>0.05)。迷路切除术组手术前、后颈源性/眼源性前庭肌源性电位的异常率差异有统计学意义(P=0.014;P=0.006)。半规管阻塞术组、迷路切除组的平衡恢复时间分别为(16.5±5.19)d、(25.07±7.87)d,差异有统计学意义(P<0.001)。 结论 半规管阻塞术治疗晚期顽固性梅尼埃病的疗效优于迷路切除术。     

耻骨上前列腺切除术后排尿困难的原因

目的　探讨耻骨上前列腺切除术后排尿困难的原因 ,为预防提供依据。方法　对 1 6例耻骨上前列腺切除术后排尿困难的临床资料进行分析。结果　后尿道狭窄 9例 ,前尿道狭窄 3例 ,腺体残留 2例 ,不稳定膀胱 1例 ,腺体增生复发 1例。结论　尿道狭窄为耻骨上前列腺切除术后排尿困难的主要原因 ,采用提高手术技巧 ,预防尿路感染 ,术前尿动力学测定是预防这类并发症的有效措施     

Speech and language development in toddlers with and without cleft palate

OBJECTIVE: The effect of early palate closure on speech and language development in children with cleft palate. DESIGN: Comparative study. SETTING: University Medical Center Groningen, Cleft Palate Team (The Netherlands). MATERIALS AND METHODS: Forty-three toddlers with cleft palate and thirty-two toddlers without cleft palate were analyzed with standardized tests for language comprehension and language production. Moreover articulation and hyper nasality were examined by trained speech therapists. RESULTS: For language comprehension, language production and articulation there were no significant differences between the children with and without cleft lip and/or palate. This is despite the high percentage of conductive hearing loss (55%) in children with clefts. Significant difference was found for hyper nasality (mean: 35% vs. 0%, p=0.001). In both groups articulation problems raise to a higher percentage than language production problems (63-20%; 24-4%). CONCLUSIONS: Early surgical treatment is effective for a part of the communicative development, i.e. language development and articulation. Besides conductive hearing loss hyper nasality remains a serious problem in 30-50% of the children with cleft palate. Therefore, speech therapy and pharyngoplasty also are part of the treatment procedure. Because of the high amount articulation problems in all children, standards for articulation development are perhaps too strict. Future research should be carried out after normal variations in articulation development.     

Investigation of underlying primary immunodeficiencies in patients with severe atopic dermatitis

BackgroundPrimary immunodeficiency diseases (PIDs) are a group of heterogeneous inherited disorders, characterised by recurrent infections, autoimmunity and malignancy. Some PIDs such as hyper IgE syndrome (HIES) and Wiskott–Aldrich syndrome (WAS) may be initially presented as atopic dermatitis (AD), especially in its severe form, resulting in diagnostic delay and poor prognosis of patients.ObjectiveThe aim of this study was to evaluate the frequency of PIDs among patients with severe AD and to determine factors that can help to raise suspicion towards these disorders.MethodsSeventy-five patients with a well-established diagnosis of severe AD were enrolled in this study. Initial immunological evaluations, including humoral and cellular investigation, were performed in all individuals. Patients underwent further investigations in a case of suspicion of a probable PID.ResultsAmong all patients with severe AD, five (6.6%) were diagnosed with HIES and one (1.3%) with WAS. Family history of PIDs, family history of death in early infancy, positive history of recurrent infections such as skin and respiratory infections, otitis media and sinusitis were observed significantly higher in patients with a diagnosis of PID.ConclusionsThe presence of an underlying PID could explain the poor prognosis and refraction to the treatment of some patients with severe AD. Several clinical and laboratory findings can help the physicians to focus towards PIDs which are more serious. Delay in diagnosis of PID cases with skin manifestation of AD without proper management may result in lower quality of life and higher morbidity and mortality rates.     

Hyper IgE syndrome: review and future directions

Hyper IgE syndrome, or Job’s syndrome, is an immunodeficiency syndrome characterized by eczema, recurrent infections and elevated serum immunoglobulin E levels. Individuals tend to have frequent staphylococcal abscesses and pneumonias early in life, often developing pneumatocoeles that predispose them to later fungal and Gram-negative bacterial infections. In addition to the immunologic abnormalities, hyper IgE syndrome is now recognized as a multisystem disorder with skeletal and morphologic features such as characteristic facies, osteopenia, scoliosis, fractures and retention of the primary teeth. Therapy of hyper IgE syndrome involves careful skin care, antimicrobial prophylaxis and aggressive treatment of infections, as well as treatment of some of the complicating abnormalities, such as scoliosis. The genetic etiology of hyper IgE syndrome remains unknown, however, it continues to be investigated. Identification of the genetic etiology or etiologies of this syndrome will allow for targeted therapies as well as great advances in understanding the connections between immunologic function and connective tissue.     

A huge low-grade fibromyxoid sarcoma of small bowel mesentery simulating hyper immune splenomegaly syndrome: a case report and review of literature

Introduction

Low-grade fibromyxoid sarcoma (LGFMS) is a rare non epithelial tumour. It usually arises from the smooth muscles of the extremities. It is, however, occasionally reported to arise from other regions of the body.

Case report

We report the case of a 32 year old man who complained of a progressive abdominal swelling of 4 months duration. There was associated abdominal discomfort and weight loss. Abdominal examination revealed a non-tender intra abdominal mass filling the abdomen completely. Abdominal ultrasound suggested a massive splenomegaly. Abdomina Computerized Tomography (CT) scan was not done due to financial constraints. At laparotomy, a large, pearl-coloured mass was found within the mesentery of the proximal jejunum, with dilated, tortuous vessels. It was resected along with the overlying 60cm of jejunum. It weighed 7.5kg. Histology and immunohistochemistry confirmed the diagnosis of lowgrade fibromyxoid sarcoma. Post-operative period was uneventful and there were no features of recurrent after 2 year of follow up.

Conclusion

LGFMS may cause a diagnostic dilemma, especially in a third world setting where preoperative diagnosis is hampered by lack of facilities and poverty. A high index of suspicion is needed for preoperative diagnosis, which is necessary for proper planning of the operation.     

可逆性脑血管收缩综合征2例的临床及影像学特点

目的 探讨可逆性脑血管收缩综合征(reversible cerebral vasoconstriction syndrome,RCVS)的临床及影像学特点.方法 回顾性分析2例RCVS患者的临床资料.结果 2例患者的触发因素分别为子痫和吉兰巴雷综合征.临床表现包括癫痫、意识障碍和视觉缺失.影像学表现包括凸面蛛网膜下腔出...     

Removal of biofilm is essential for long-term ventilation tube retention

BACKGROUND Although long-term retention of a ventilation tube is required in many ear diseases,spontaneous removal of conventional ventilation tube is observed in patients within 3 to 12 mo.To address this issue,we aimed to determine a new method for long-term retention of the ventilation tube.AIM To explore the value of removing the biofilm for long-term retention of tympanostomy ventilation tubes.METHODS A case-control study design was used to evaluate the safety and effectiveness of long-term tube retention by directly removing the biofilm(via surgical exfoliation)in patients who underwent myringotomy with ventilation tube placement.The patients were randomly divided into two groups:Control group and treatment group.Patients in the treatment group underwent regular biofilm exfoliation surgery in the clinic,whereas those in the control group did not have their biofilm removed.Only conventional ventilation tubes were placed in this study.Outcome measures were tube position and patency.Tube retention time and any complications were documented.RESULTS Eight patients with biofilm removal and eight patients without biofilm removal as a control group were enrolled in the study.The tympanostomy tube retention time was significantly longer in the treatment group(43.5±26.4 mo)than in the control group(9.5±6.9 mo)(P=0.003).More tympanostomy tubes were found to be patent and in correct position in the treatment group during the follow-up intervals than in the control group(P=0.01).CONCLUSION Despite the use of short-term ventilation tubes,direct biofilm removal can be a well-tolerated and effective treatment for long-term tube retention of tympanostomy ventilation tubes in patients who underwent myringotomy.     

Ménétrier病1例报道

Ménétrier病是一种临床罕见的、特殊类型的肥厚性胃炎,主要表现为胃体、胃底黏膜皱襞粗大、肥厚,扭曲成脑回状,同时伴有胃酸分泌减少及低蛋白血症。本文报道1例患者临床资料,并结合国内外文献报道,探讨本病的病因、发病机制、临床表现、诊断及治疗,以提高临床医师对该病的认识水平。