Chronic granulomatous disease: a case report.

Chronic granulomatous disease is one form of the phagocyte function disorder. Unlike most patients with chronic granulomatous disease who develop signs and symptoms of chronic and recurrent pyogenic infections during the first 2 years of life, patients with mild forms of the disease may not present until the teenage years or even adulthood. Thus, the diagnosis in these mild-form patients is often delayed. This paper describes a patient with the mild form of chronic granulomatous disease. A 7-year-old boy was admitted to our ward with intermittent high fever and a left neck mass present for about 1 week. He had a history of persistent infection in the bilateral lower face lasting for about 1 year during his fourth year of life. Family history was unremarkable except that the patient's elder sister had a history of persistent oral mucosal wound infection for about 1 year during the fifth year of life. On physical examination, there were scars over the patient's bilateral lower face. Bacterial culture of pus drained from the neck mass revealed Burkholderia cepacia, a rare species in patients without immunodeficiency. A series of antibiotics, including oxacillin, clindamycin, and piperacillin, was given, and two incision operations for drainage and debridement were performed. The neck mass resolved completely about 1.5 months later. This history indicated that the patient might have chronic granulomatous disease. A definite absence of superoxide activity in the patient's granulocytes detected by chemiluminescence and nitroblue tetrazolium dye test confirmed this diagnosis.     

Bethlem myopathy in a Taiwanese family.

We report three cases of Bethlem myopathy from three consecutive generations of a Taiwanese family, including one woman aged 70, one man aged 40, and a boy aged 8. The clinical features of the patients included autosomal dominant inheritance, childhood or adolescent onset, mainly proximal and extensor involvement, early diffuse joint contractures, and absence of cardiac involvement. These features fulfilled the diagnostic criteria for Bethlem myopathy. Though the clinical course of the disease was once thought to be benign, our female patient became wheelchair-bound at the age of 53. This suggests that the disease process in Bethlem myopathy is slow but ongoing.     

Effectiveness of postoperative adjuvant therapy in improving reproductive outcome of endometriosis-associated infertility.

BACKGROUND AND PURPOSE: Treatment of endometriosis-associated infertility has not yet become standardized. Various protocols including surgical treatment, medical therapy, and a combination of both have been suggested but their use remains controversial. The objective of the present study was to determine whether postoperative adjuvant therapy for endometriosis is effective in improving reproductive outcome. METHODS: Medical records of infertile patients with newly diagnosed endometriosis treated in a university teaching hospital during a 50-month period were reviewed. After exclusion of patients with other major infertility factors, a total of 209 patients were included in the retrospective analysis. These patients were divided into those receiving (n = 78) or not receiving (n = 131) peri- or postoperative adjuvant medical therapy. The adjuvant therapies included danazol (n = 62), gonadotropin releasing hormone analogues (n = 11), progestins (n = 3), oral contraceptives (n = 1), and mixed treatment (n = 1). RESULTS: The pregnancy rate was lower in those receiving adjuvant therapy, although this result was not significant (32.1% vs 45.8%; p = 0.05). When patients using postoperative danazol therapy were considered alone, the pregnancy rate in patients receiving adjuvant therapy was significantly lower than that in patients not receiving it (p = 0.047). When the stage of endometriosis was considered, the pregnancy rate in patients receiving adjuvant therapy was again lower than in those not receiving it in patients with minimal or mild endometriosis (42.9% vs 60%; p = 0.043). However, in patients with moderate or severe endometriosis, the pregnancy rate was not different in the two groups (31% vs 36%; p = 0.56). Postoperative assisted reproductive techniques (ART) including controlled ovarian hyperstimulation/intrauterine insemination (COH/IUI) and in vitro fertilization (IVF) were effective in improving the pregnancy rates for all patients (53.9% with ART vs 33.1% without; p = 0.003) and for patients with advanced endometriosis (47.7% with ART vs 27.2% without; p = 0.016). CONCLUSIONS: Our results suggest that postoperative adjuvant therapy is ineffective in improving reproductive outcome in patients with either early (minimal or mild) or advanced (moderate and severe) endometriosis. This finding suggests that if fertility is the goal of treatment, adjuvant therapy may be unnecessary after surgery. In contrast, our data suggest that empirical ART, including COH/IUI or IVF, may be a better alternative to improve the pregnancy outcome after surgery.     

香茅醇亚微乳的处方工艺优化研究

目的:优化香茅醇亚微乳的制备工艺。方法:采用高效液相色谱法测定香茅醇亚微乳中的香茅醇含量;采用高速剪切分散-高压均质法制备香茅醇亚微乳,以离心稳定常数(ke)、粒径为指标,对其处方及工艺进行优化并进行验证;测定所得制剂的载药量的包封率。结果:香茅醇检测质量浓度的线性范围为4～64μg/mL(R²=0.999 9);精密度、稳定性(24 h)、重复性试验的RSD均小于3%;加样回收率为97.64%～101.97%(RSD=2.28%,n=3)、97.71%～99.50%(RSD=1.29%,n=3)、96.87%～101.48%(RSD=2.86%,n=3)。最优处方为大豆油+中链甘油三酯(1∶1,g/g)总质量3.75 g,1.2%大豆磷脂0.6 g,胆固醇0.06 g,香茅醇1.25 g,0.6%油酸钠0.3 g,15-羟基硬脂酸聚乙二醇酯0.75 g,泊洛沙姆188 0.75 g,加水至50 mL。最优工艺为于4℃下以13 000 r/min高速剪切5 min制得初乳后,经稀盐酸调p H至7,再以600 Bar高压均质5 min。按最优处方及工艺制备的3批香茅...     

MiR-767和CYLD在皮肤恶性黑色素瘤中的表达和意义

目的探讨MiR-767和圆柱瘤基因(CYLD)在皮肤恶性黑色素瘤(CMM)的表达情况和意义。方法采集45例CMN患者肿瘤组织及配对的瘤旁正常组织进行研究,应用实时荧光定量PCR(qRT-PCR)检测肿瘤组织及瘤旁组织MiR-767和CYLD表达水平,分析二者相关性及对CMM预后的临床意义。结果肿瘤组织中MiR-767表达水平明显高于瘤旁正常组织(P<0.05),CYLD表达水平显著低于瘤旁正常组织(P<0.05);MiR-767表达在不同性别间比较,差异无统计学意义(P>0.05),与肿瘤分期、淋巴结转移、肿瘤厚度密切相关(P<0.05),CYLD表达在不同性别、肿瘤分期差异无统计学意义(P>0.05),与淋巴结转移、肿瘤厚度密切相关(P<0.05);随着CMM肿瘤组织中MiR-767表达水平升高,CYLD表达呈明显降低趋势,MiR-767与CYLD表达呈明显负相关(r=-0.791,P<0.001);MiR-767高表达组预后5年存活率显著低于低表达组存活率(P<0.05);CYLD高表达组预后5年存活率显著高于低表达组存活率(P<...     

颅内肿瘤术后主要安全隐患及护理对策

分析颅内肿瘤术后安全隐患及其发生原因,探讨安全防护措施,旨在为相关护理过程中实行护理安全管理,杜绝医患纠纷的发生,确保患者生命安全提供参考。     

大批量特重度烧伤病人的护理

目的：讨论大批量特重度烧伤病人的护理。方法：对7例特重度烧伤病人精心治疗和护理。结果：7例病人经过全科人员的共同努力,无1例发生休克、创面感染、褥疮及其他并发症,均先后治愈出院。结论：及时有效的治疗和精心的护理能弥补条件的不足,促进病人的康复,并减少并发症的发生。     

咽痛冲剂治疗急性咽炎疗效观察

目的：探讨咽痛;中剂治疗急性咽炎的临床疗效。方法：将120例急性咽炎患者随机分为两组,治疗组予咽痛;中剂治疗,对照组口服抗生素治疗。结果：治疗组总有效率为93．33％,对照组总有效率为81．66％,两组比较,差异有显著性（P〈0．05）。结论：咽痛冲剂治疗急性咽炎疗效佳。     

结肠癌Colo205细胞系SP细胞分子标志的初步观察

目的：寻找结肠癌Colo205细胞株中SP（side population）亚群细胞的特异性分子标志。方法：结肠癌Colo205细胞经Hoechest33342染色处理后固定,后续PI复染对其中的SP细胞进行定位,并通过荧光免疫细胞化学染色比较SP细胞与非SP细胞在DNA-PKcs、p16、FHIT、NF-κB／p65及Id2中表达的异同。结果：结肠癌Colo205细胞中Ho—echst33342低染的SP细胞比例的均数为7％;结肠癌Colo205 SP细胞的胞核体积较大,呈圆形或椭圆形,符合干细胞的形态学特点;SP细胞DNA—PKcs表达定位于整个胞核,而非SP细胞定位于核膜及其邻近核质。SP细胞DNA—PKcs表达的阳性强度明显高于非SP细胞;SP细胞Id2表达的阳性强度明显高于非SP细胞;SP细胞的p16表达的阳性强度明显低于非SP细胞;SP细胞FHIT的表达为阴性或微弱阳性;SP细胞NFκB／p65表达的阳性强度明显低于非SP细胞。结论：DNA-PKcs和Id2可能为结肠癌Colo205细胞中SP细胞的阳性分子标志,而p16、NF-κB／p65和FHIT可能为结肠癌SP细胞的阴性分子标志。     

苦参素注射液治疗晚期消化道恶性肿瘤40例

目的:观察苦参素注射液(天晴复欣)治疗晚期恶性消化道肿瘤的疗效。方法:将60例晚期恶性消化道肿瘤随机分为治疗组40例,用苦参素注射液每次100ml,每日1次,连用30d;对照组20例,单用最佳支持治疗。治疗前后分别采用恶性肿瘤生活质量评分表,KPS评分表进行评估。结果:苦参素组能明显改善晚期恶性消化道肿瘤病人生活质量,无明显毒副作用。