A combined vascular surgical and clinical genetics approach to diffuse aneurysmal disease

We report two patients who presented with extensive aneurysmal disease, in association with minimal external physical signs. Patient 1 remained genetically undiagnosed despite multiple structural, biochemical and genetic investigations. He made a good recovery following surgery for popliteal and left axillary artery aneurysms. Patient 2 was diagnosed with vascular type Ehlers–Danlos syndrome, associated with a high degree of tissue and blood vessel fragility, and is being managed conservatively. Early multidisciplinary assessment of such patients facilitates accurate diagnosis and management.     

经胫骨隧道与前内入路建立股骨骨道早期变化的差异

目的：比较分析经胫骨隧道与前内入路两种方式建立股骨骨道早期变化的差异,探讨相关的影响因素。方法对94例患膝关节在关节镜下单束重建前交叉韧带,A 组（42例）经胫骨隧道建立股骨隧道,B 组（52例）经前内侧入路建立股骨隧道。重建后用相同的方法进行康复锻炼,术后1周和6个月复查 X 线片了解骨道情况。结果A 组38例、B 组42例完成 X 线检查。术后骨道增宽的发生率两组比较差异无统计学意义（P＞0．05）;术后骨道增宽的程度 A 组较 B 组明显,差异有统计学意义（P ＝0．001）。结论两种入路建立的股骨股道术后早期均具有较高的骨道扩大发生率,经前内侧入路行 ACL 重建更有利于减轻股骨骨道的扩大。     

Adherent cell inhibition of human leukemic in vitro agar clonal growth by short-term cell-to-cell contact

The effects of peripheral blood adherent cells from normal donors on human myeloid leukemic cluster growth in agar were studied. A prior co- incubation of nonadherent leukemic cells with adherent cell monolayers from 9 out of 10 donors in liquid cultures over a 4-hr period was sufficient to reduce subsequent leukemic growth in semisolid agar cultures. Inhibition was seen with adherent to leukemic cell ratios of as low as 0.5:1. Conversely, identical numbers of adherent cells in agar cultures but separated from the leukemic cells enhanced growth more than the cultures containing human placental conditioned media alone. Because leukemic cell exposure to adherent cells was brief, a cytotoxic mechanism appeared likely; however, this could not be detected by 51Cr release. Human peripheral blood adherent cells not activated by any in vitro mechanism suppress clonal growth of human myeloid leukemic cells by a mechanism requiring cell to cell contact. Examination of the inhibition of clonal growth appears to be more sensitive than 51Cr release as an indicator of adherent cell effects on myeloid leukemia.     

Pelvic ultrasound measurements in normal girls

Pelvic ultrasound scans were carried out in 153 normal girls aged between 3 days and 14.9 years, in order to obtain reference data for ovarian volume, uterine length and uterine configuration. The right ovary was significantly larger than the left (by about 17%). Ovarian volume increased exponentially with age, over this age range. No relationship with pubertal stage (independent of age) could be demonstrated. Uterine length decreased from birth to 4 years, before steadily increasing. The fundalcervical ratio (FCR) decreased initially then increased to lie above 1.0 by 15 years of age. A midline endometrial echo was seen in half of the subjects aged less than 6 months, but otherwise it was not seen before 11.8 years of age or at Tanner stage B2. Smoothed reference centile curves for uterine length, right and left ovarian volume were produced, allowing z scores (or SD scores) to be calculated for each measurement.     

Genetic testing for maturity onset diabetes of the young in childhood hyperglycaemia

Mild hyperglycaemia is a common finding during minor illness in children. The differential diagnosis includes maturity onset diabetes of the young (MODY), which can be a difficult diagnosis to make clinically. As most genes resulting in MODY have been identified, it is possible to make a firm diagnosis using mutation detection. A case is reported of a 4 year old girl in whom a diagnosis of MODY2 was established by the finding of a heterozygous missense mutation in exon 7 of the glucokinase gene, resulting in the substitution at codon 259 of alanine by threonine (A259T). Observations from other glucokinase families suggest that hyperglycaemia in this child is likely to be stable and will not require intensive medical follow up, whereas other forms of MODY (1, 3, and 4) might carry a different prognosis.     

Dexamethasone and bacterial meningitis in Pakistan

The objective of this study was to assess, in a developing country setting, the effect of dexamethasone therapy on bacterial meningitis outcomes. A prospective double blind placebo controlled trial was conducted in 89 children aged from 2 months to 12 years suffering from bacterial meningitis. Neurological, developmental, and hearing assessments were conducted at one, four, and 12 months after discharge. Forty eight patients received dexamethasone and 41 placebo. Initial antimicrobial drugs used were ampicillin and chloramphenicol. For all patients at the time of admission the mean duration of illness was 5.7 days; 47% had had seizures and 56% had impaired consciousness. Seventeen of 89 (19%) patients died. The mortality for the dexamethasone group was 25% as compared with 12% in the group receiving placebo. Presentation to the hospital after four days of symptoms and with impaired conscious state were independent predictors of death. Of the dexamethasone group survivors, 26.5% had neurological sequelae and 42.3% had hearing impairment, whereas in the placebo group it was 24% and 30% respectively. Altered state of consciousness was a predictor of neurological sequelae. The presence of neurological sequelae and high cerebrospinal fluid protein independently predicted hearing loss. No beneficial effect of dexamethasone was observed on morbidity or mortality of this group of patients with bacterial meningitis. Dexamethasone is therefore not useful in developing countries as adjunctive treatment in patients seriously ill with bacterial meningitis, who present late for treatment and have been partially treated.     

机器人辅助腹腔镜下膀胱癌根治术后早期并发症及其危险因素

目的：探索机器人辅助腹腔镜下膀胱癌根治术后早期并发症发生的危险因素,为预防术后早期并发症 提供参考。方法：选择2016年1月至2018年1月在中南大学湘雅二医院和湘雅三医院行机器人辅助腹腔镜下膀胱癌 根治术(robot-assisted laparoscopic radical cystectomy,RARC)的膀胱癌患者90例,收集患者基本信息、术前检查及术 后90 d内随访资料。对术后早期并发症的危险因素行单因素和多因素logistic回归分析。结果：术后总体并发症发 生率为48.9%(44/90),其中Clavien分级1级9例,2级17例,3级4例,4级12例,5级2例。术后90 d内并发症以急性肾 功能损伤(22.2%)、肠梗阻(16.7%)、尿路感染(14.4%)、淋巴漏(10.0%)最为常见。术后90 d内死亡2例(2.2%)。术前 BMI(OR=1.16,95% CI：1.02~1.32)、术后30 min内血肌酐值(OR=1.02,95% CI：1.00~1.03)和pT分期(OR=1.67,95% CI：1.05~2.68)为RARC术后早期并发症的危险因素。结论：RARC术后早期并发症发生率较高,术前血液透析,纠正 贫血,术中保护肾功能,快速康复治疗等有助于预防术后早期并发症的发生。     

Perspectives of Screening‐Eligible Women and Male Partners on Benefits of and Barriers to Treatment for Precancerous Lesions and Cervical Cancer in Kenya

Background

Cervical cancer is the leading cause of female cancer mortality in Kenya. Kenya's National Cervical Cancer Prevention Program Strategic Plan outlines efforts to reduce the burden; however, treatment services remain limited. This study identified male and female perspectives regarding benefits, facilitators, and barriers to treatment for precancerous lesions and cervical cancer.

Materials and Methods

Ten focus groups were conducted in Nairobi and Nyanza in 2014 with females aged 25–49 years (n = 60) and male partners (n = 40). Participants were divided into groups dependent on screening status, sex, language, and geographic location. Qualitative analytic software was used to analyze transcribed and translated data.

Results

Treatment was endorsed as beneficial for the prevention of death and the improvement of wellness, quality of life, symptoms, and family life. Barriers reported by males and females included the following: (a) concerns about side effects; (b) treatment‐related fear and stigma; (c) marital discord; (d) financial and access issues; (e) religious and cultural beliefs; and (f) limited knowledge. Male endorsement of wanting to improve knowledge and communication with their partners, in spite of stigmatizing beliefs and misperceptions regarding females with abnormal screening results or those who have been diagnosed with cancer, was novel.

Conclusion

Incorporating qualitative data on benefits of and barriers to treatment for precancerous lesions and cervical cancer into Kenya's national priorities and activities is important. These findings can be used to inform the development and successful implementation of targeted, region‐specific community outreach and health messaging campaigns focused on alleviating the country's cervical cancer burden.

Implications for Practice

This article provides important insight into female and male partner perspectives regarding benefits, facilitators, and barriers to treatment for precancerous lesions and cervical cancer. These novel research findings can inform the development of targeted community health interventions, educational messages, and resources and aid stakeholders in strengthening strategic plans regarding treatment coverage and cervical cancer prevention. Because several treatment barriers identified in this study are similar to barriers associated with cervical cancer screening in low‐ and middle‐resourced countries, effective messaging interventions could address barriers to receipt of both screening and treatment.     

Coronary Interventions in Congenital Heart Diseases: From Preterm to Young Adult Patients

Background

The small vessel size of infants and children makes interventional treatment of impaired coronary perfusion, such as stenoses, complete occlusions, and fistulae, demanding. Materials and techniques appropriate for this young age group have to demonstrate their ability to effectively treat these lesions.

Methods and Results

Between 2004 and 2011, 14 patients with an age of 9 days to 25 years (median 4.6 years) and a bodyweight of 1.7–65 kg (median 14 kg) underwent coronary intervention. In 3 cases, emergency revascularization of the left coronary artery (CA) was performed successfully, followed by stent implantation in 1 patient. Embolization of coronary arterial fistulae with coils and vascular plugs was effective in 10 patients. An antegrade, retrograde or combined approach to achieve the most distal device placement preserved all side branches. One infant with pulmonary atresia and an intact ventricular septum was prepared for biventricular repair by step‐by‐step closure of the right ventricular to the CA connections. No procedure‐related deaths occurred.

Conclusion

Congenital and post‐procedural coronary obstructive lesions can be considered for effective treatment with balloon dilation at any age as a salvage procedure. In coronaries impaired by external compression, stent implantation can restore perfusion, but long‐term results are missing. Interventional closure of coronary fistulae has shown improvement of coronary arterial perfusion. The latter techniques can be used to close right ventricular to CA connections in patients with pulmonary atresia to prepare for biventricular repair, but bail‐out strategies should be planned in all coronary interventions. (J Interven Cardiol 2013;26:287–294)