先天性心脏病儿童白细胞介素2活性及膜表面白细胞介素2受体的研究

目的 研究先天性心脏病（先心病）患儿的免疫功能状态。方法 采用间接免疫荧光检测膜表面白细胞介素2受体（Tac）、植物血凝素刺激后的膜表面白细胞介素2受体（Tac-a),MTT比色法检测白细胞介素2活性（IL－2）。结果 与健康儿童比较,先心病患儿IL－2活性和Tac-a都显著降低（P＜0．01）;与非紫绀型先心病患儿比较,紫绀型先心病患儿IL－2活性显著降低（P＜0．01）。结论 先心病患儿免疫功能缺陷可能是其易患感染性疾病的原因之一,增强免疫治疗对预防先心病患儿感染十分必要。     

先天性心脏病伴肺动脉高压患儿血浆神经肽Y、降钙素基因相关肽的研究

目的研究降钙素基因相关肽(CGRP)、神经肽Y(NPY)与先心病肺高压的关系,分析两者是否具有相关性.方法采用放射免疫法测定80例先天性心脏病(13例非肺高压,67例肺高压)血清NPY、CGRP的浓度,比较两组间不同采血部位及手术前后水平变化.结果血浆NPY及CGRP浓度非肺高压组与轻、中、重度肺高压组均存在显著性差异;手术前后轻、中度肺高压组存在显著性差异,而重度肺高压组差异不明显;NPY与CGRP存在负相关关系.结论NPY及CGRP在先天性心脏病肺动脉高压的发生机制中有重要的作用,NPY/CGRP比值可作为估测肺动脉压力和判断先心病预后的重要指标.     

Use of ivabradine for treatment of junctional ectopic tachycardia in post congenital heart surgery

Cardiac surgeries especially involving crux of the heart as performed in tetralogy of Fallot (TOF) and pulmonary stenosis are mainly responsible for junctional ectopic tachycardia (JET). Diversified antiarrhythmic agents have been used in an impressive way to treat JET but showed suboptimal efficacy and varied associated adverse effects. But, ivabradine has proved as final crusader for its treatment. We report our initial experience of 4 cases in last 6 months with ivabradine in the management of postoperative JET. Encouraged by various reports and our increasing experience with ivabradine in heart failure population, we have moved to ivabradine as the first drug of choice for postoperative JET. Bradycardia was the only significant adverse effect in our series. The availability of atrial and ventricular pacing wires or at least transvenous temporary pacing should be ensured before starting ivabradine.     

Migraine-like headaches associated with nickel allergy requiring removal of atrial septal defect closure device

There is a deficit of literature regarding the association between nickel allergy–induced symptoms and implanted devices. This report describes a case of nickel allergy causing debilitating migraine-like symptoms, failing to resolve with medical therapy, requiring surgical removal of the device and repair of the defect.     

Congenital Pseudarthrosis of Femur and Sciatic Nerve Palsy with Congenital Constriction Band Syndrome: Report of the First Case in Literature

Congenital constriction band syndrome has varied clinical presentations ranging from small, incomplete skin deep constriction band to in utero amputation. Pseudarthrosis of underlying bone most commonly tibia has been reported by many authors. We report the first case of congenital pseudarthrosis of the femur with congenital constriction band syndrome. Nine-day-old female presented with the constriction band in the left thigh with open pseudarthrosis of the femur. The left femur had gross recurvatum deformity and the posterior apex of the pseudarthrosis was exposed via skin ulceration. She had an ipsilateral paralytic clubfoot. She was treated with single-stage excision of constriction band and Z-plasty. Spontaneous union of the femur was achieved at 3 months. Procurvatum deformity of the femur improved gradually over 3 years. This happens to be the first and only reported case of congenital pseudarthrosis of the femur with sciatic nerve palsy due to congenital constriction band.     

Cross-cultural adaptation,validity and reliability of Turkish version of Oxford Ankle Foot Questionnaire for children with congenital talipes equinovarus

BackgroundThe purpose of the study was to evaluate the reliability and validity of the Turkish version of the Oxford Ankle Foot Questionnaire (OxAFQ) to provide cultural adaptation.MethodsThis study involved translation, back translation, and cross-cultural adaptation. Forty-nine patients with congenital talipes equinovarus were evaluated using the Turkish version of OxAFQ. Turkish version of the Childhood Health Assessment Questionnaire (CHAQ) was used as a gold standard to validate the Turkish version of the OxAFQ. The validation was assessed with Spearman correlation analysis by using CHAQ. The reliability of the questionnaire was assessed with Cronbach alpha (internal consistency) and exploratory factor analysis.ResultsHigh validity was found between OxAFQ and CHAQ (r = -0.422?0.292) (p < 0.01). Reliability analysis showed that OxAFQ had a high level of Cronbach alpha (α = 0.88?0.96) and internal consistency (ICC = 0.90?0.96).ConclusionThe Turkish version of OxAFQ is a valid, reliable and useful quality of life questionnaire in patients with congenital talipes equinovarus and it is proper for use by health professionals and researchers.     

Retrorectal tumors: A challenge for the surgeons

Retrorectal or presacral tumors are rare lesions located in the presacral area and considered as being derived from multiple embryological remnants. These tumors are classified as congenital, neurogenic, osseous, inflammatory, or miscellaneous. The most common among these are congenital benign lesions that present with non-specific symptoms, such as lower back pain and change in bowel habit. Although congenital and developmental tumors occur in younger patients, the median age of presentation is reported to be 45 years. Magnetic resonance imaging plays a crucial role in treatment management through accurate diagnosis of the lesion, the evaluation of invasion to adjacent structures, and the decision of appropriate surgical approach. The usefulness of preoperative biopsy is still debated; currently, it is only indicated for solid or heterogeneous tumors if it will alter the treatment management. Surgical resection with clear margins is considered the optimal treatment; described approaches are transabdominal, perineal, combined abdominoperineal, and minimally invasive. Benign retrorectal tumors have favorable long-term outcomes with a low incidence of recurrence, whereas malignant tumors have a potential for distant organ metastasis in addition to local recurrence.