纤维支气管镜下带囊支架置入术治疗儿童气管狭窄2例

目的通过纤维支气管镜下带囊支架置入术治疗儿童气管狭窄,观察近期疗效和并发症。方法例1,女,4月龄,因＂发现心脏杂音3个月余,咳嗽10 d渐加重,伴发热、喘息3 d＂入院,诊断为膜周部＋肌部室间隔缺损,房间隔缺损（继发孔型）,肺动脉高压,主气道下段及左右主支气管变形狭窄。行肺动脉环缩术,房间隔、室间隔缺损修补术＋主动脉悬吊术成功后,术后2个月内3次撤离呼吸机均未成功。例2,女,1岁10个月,因＂咳嗽1个月余＂入院,诊断为左主支气管狭窄,EBV相关性噬血细胞淋巴组织细胞增生症。左侧支气管狭窄致使排痰困难,肺部反复感染,左肺气肿。2例患儿家长在充分了解纤维支气管镜下带囊支架置入术可能的风险后签署知情同意书。采用＂边麻边进＂方法行气道黏膜表面麻醉,例1和例2选择带囊支架长度分别为24和29 mm,直径均为4 mm（均经雷帕霉素处理）,由支架导入器送入纤维支气管镜,在纤维支气管镜直视下释放带囊支架,然后退出支架导入器。结果 2例患儿置入带囊支架后狭窄段气管扩张良好,带囊支架放置2~3个月随访,未见肉芽组织增生等并发症。结论纤维支气管镜下带囊支架置入术治疗气管狭窄近期疗效较好,远期疗效尚待观察。     

Familial ureteric bud anomalies.

A family is described in which various renal anomalies occurred. The condition is considered to represent an autosomal dominantly inherited 'instability' of the ureteric bud.     

Uncommon ureteric ectopias: embryological implications

We herein report two rare ureteric ectopias, one in the uterus, the second in the rectum. These kinds of ectopias are not easily explained with regard to the classic embryological theories of the too-cranial or too-caudal origin of the ureteric bud on the mesonephric duct. We thus set out new explanations consistent with embryological studies on the narrow contact between the developing paramesonephric and mesonephric ducts, and on the so-called cloacal septation.     

Intracellular and extracellular regulation of ureteric bud morphogenesis

The urinary collecting duct system of the permanent kidney develops by growth and branching of an initially unbranched epithelial tubule, the ureteric bud. Formation of the ureteric bud as an outgrowth of the wolffian duct is induced by signalling molecules (such as GDNF) that emanate from the adjacent metanephrogenic mesenchyme. Once it has invaded the mesenchyme, growth and branching of the bud is controlled by a variety of molecules, such as the growth factors GDNF, HGF, TGFβ, activin, BMP-2, BMP-7, and matrix molecules such as heparan sulphate proteoglycans and laminins. These various influences are integrated by signal transduction systems inside ureteric bud cells, with the MAP kinase, protein kinase A and protein kinase C pathways appearing to play major roles. The mechanisms of morphogenetic change that produce branching remain largely obscure, but matrix metalloproteinases are known to be necessary for the process, and there is preliminary evidence for the involvement of the actin/myosin contractile cytoskeleton in creating branch points.     

Intracellular and extracellular regulation of ureteric bud morphogenesis

The urinary collecting duct system of the permanent kidney develops by growth and branching of an initially unbranched epithelial tubule, the ureteric bud. Formation of the ureteric bud as an outgrowth of the wolffian duct is induced by signalling molecules (such as GDNF) that emanate from the adjacent metanephrogenic mesenchyme. Once it has invaded the mesenchyme, growth and branching of the bud is controlled by a variety of molecules, such as the growth factors GDNF, HGF, TGFβ, activin, BMP‐2, BMP‐7, and matrix molecules such as heparan sulphate proteoglycans and laminins. These various influences are integrated by signal transduction systems inside ureteric bud cells, with the MAP kinase, protein kinase A and protein kinase C pathways appearing to play major roles. The mechanisms of morphogenetic change that produce branching remain largely obscure, but matrix metalloproteinases are known to be necessary for the process, and there is preliminary evidence for the involvement of the actin/myosin contractile cytoskeleton in creating branch points.     

Clinical characteristics and rate of dilatation in Turner syndrome patients treated for aortic dilatation

Turner syndrome is associated with an increased risk of aortic aneurysms and dissection. Recent 2017 clinical care guidelines recommend medical therapy to treat aortic dilatation, although whether this slows dilatation is unknown. We aimed to describe a pre‐guideline cohort of Turner syndrome patients with aortic dilatation, the rate of dilatation following diagnosis, and post therapy dilatation rates. We conducted a retrospective review of Turner syndrome patients with a dilated aortic root or ascending aorta by current definitions. In total, 40 patients were included with 22 treated patients. Most patients had 45,X karyotype, were white, non‐Hispanic, and received both growth hormone and estrogen. Except for hypertension, there were no differences in risk factors among treated and untreated groups. Bicuspid aortic valve was very common. Treatment group patients had significantly more dilated ascending aortas by absolute measurements and aortic size index. In an adjusted model, there was minimal change in aortic measures over time and this was not associated with medication use. In conclusion, in this cohort, Turner syndrome patients with aortic dilatation were more likely to be treated if they had hypertension and if they met multiple dilatation criteria. Further study is needed to establish medical therapy efficacy on dilatation progression.     

Comparison of Paclitaxel-Coated Balloon Treatment and Plain Old Balloon Angioplasty for De Novo Coronary Lesions

PurposeThis study compared the angiographic outcomes of paclitaxel-coated balloon (PCB) versus plain old balloon angioplasty (POBA) treatment for de novo coronary artery lesions. At present, there is no available data comparing the efficacy of PCB versus POBA for the treatment of de novo coronary lesions.ResultsA total of 72 patients (49 receiving PCB and 23 receiving POBA) were enrolled in this study. Late luminal loss was -0.12±0.30 mm in the PCB group and 0.25±0.50 mm in the POBA group (p<0.001). There was a higher percentage of binary restenosis (diameter stenosis ≥50%) in POBA, compared to PCB (30.4%, n=7 vs. 4.1%, n=2, p<0.001). Target vessel revascularization was higher in the POBA group (13.0%, n=3 vs. 0%, p=0.033).ConclusionPCB treatment of de novo coronary lesions showed better 9-month angiographic outcomes than POBA treatment alone.     

Balloon cell nevus of the iris

Balloon cell nevus is a rare histopathological lesion characterized by a predominance of large, vesicular and clear cells, called balloon cells. There is only 1 case of balloon cell nevus of the iris reported in the literature.Case reportA 55 year-old man presented a pigmented elevated lesion in the right iris since the age of 12 years old. The lesion had been growing for the past 2 years and excision was performed. Histopathological examination showed a balloon cell nevus composed of clear and vacuolated cells without atypia. A typical spindle cell nevus of the iris was also observed. The differential diagnosis included xanthomatous lesions, brown adipocyte or other adipocytic lesions, clear cell hidradenoma, metastatic clear cell carcinoma of the kidney and clear cell sarcoma. The tumor was positive for Melan A, S100 protein and HMB45.ConclusionBalloon cell nevus of the iris is rare but should be considered in the differential diagnosis of melanocytic lesions of the iris.