心导纳微分环,肺导纳微分环最大向量正常值及其临床意义

本文报道３－５０岁２１４例心导纳微分环（ＣＡＤＬ）和肺导纳微分环（ＰＡＤＬ）最大向量振幅及方位正常值，结果随增龄变化振幅递减，方位递增，多数男＞女，部分组间有差异。４８例左向右分流先心病环体最大向量与肺动脉压之间呈平行关系，并探讨其临床意义。     

Percutaneous transcatheter occlusion of coronary artery fistulas using detachable balloons

Three pediatric patients underwent successful transcatheter coronary artery fistula occlusion using the Debrun system. This latex balloon system offers several advantages over other occlusion systems. First, the balloon delivery and release is controlled. Second, “test occlusions” can be performed that allow simultaneous balloon inflation, coronary cineangiography, and electrocardiographic monitoring. Third, because the balloons are flow-directed, they are easily positioned in properly chosen locations. Finally, the balloons can be constructed to suit the size of the fistula. In this study, two patients received only one balloon; in the other patient two balloons were placed in the same fistula. All fistulas drained into either the right atrium or ventricle and were successfully occluded. After a follow-up period of up to 3 years, no local or systemic reactions to the balloons were recognized. We conclude that detachable balloon occlusion of coronary artery fistulas is a safe, effective alternative to surgical ligation in selected pediatric patients.     

Congenital cervical atresia: Report of seven cases and review of the literature

OBJECTIVE: Our aim was to evaluate the clinical course and management of congenital cervical atresia. STUDY DESIGN: This retrospective analysis included 7 patients referred to our clinic and a review of the medical literature. RESULTS: Including this case series, 58 cases of congenital cervical atresia have been reported in the literature. Forty-eight percent of patients had isolated congenital cervical atresia with a normal vagina whereas the remainder had either complete or partial vaginal atresia (“shortened blind vaginal pouches”). Surgical management has included abdominal hysterectomy or uterovaginal cannulation with or without vaginoplasty. In 59% of patients who underwent uterovaginal canalization procedures (23/39), normal menstrual bleeding was achieved. Four of these patients subsequently became pregnant and were delivered at term. CONCLUSION: Surgical canalization in selected patients with congenital cervical atresia can be successfully performed to provide patients an opportunity for conservative management, resulting in normal menstrual bleeding, resolution of cyclic pelvic pain, and some potential (albeit limited) for fertility.(Am J Obstet Gynecol 1997;177:25)     

Blood gas and ventilatory parameters in predicting survival in congenital diaphragmatic hernia

Blood gas analysis can be used to reliably predict outcome in infants with congenital diaphragmatic hernia (CDH) both before and after surgical repair, providing these values are indexed to some measurement of alveolar ventilation. Until recently there has been difficulty in interpreting some of the published data because of differing sampling sites and the fact that ventilatory parameters, which have major influences on all blood gas values in this anomaly, were not included. Application of this information enables us to identify infants with similar degrees of severity of CDH in order to evaluate the efficacy of novel forms of therapy and to determine whether they represent a genuine advance in management where more conventional forms of treatment have failed.     

医原性(足母)内翻的外科治疗

目的探讨医原性躅内翻矫形手术治疗的经验与防治方法。方法1989年7月至2005年5月治疗医原性躅内翻27例（28足），男4例（4足），女23例（24足）；年龄24～62岁，平均51.3岁。采用Hiss手术13例（14足）、myerson手术10例（10足）、跖骨远端截骨术4例（4足）。术前对每个病例进行临床、x线片与CT检查，分析其躅内翻的发病原因。术后对所有病例进行随访，按美国足踝外科协会maryland百分制评分系统，对疼痛、功能、体征进行评分。结果27例（28足）获得随访，随访时间1年～17年2个月，平均5．7年。术前评分平均为52．4（41～57）分，术后平均为89（64～100）分。疗效优13足（46_4％），良11足（39．3％），可4足（14-3％），优良率85．7％。施行Hiss手术者中1例术后13年发生蹿长伸肌腱轻度挛缩，采用myerson手术者术后背伸较术前平均减少9.5°（8°。～12．5°。）。医原性躅内翻的发生原因有5个，其中最常见的是在躅外翻矫形时跖骨头内侧骨赘切除过多，本组有14足（50％），然后依次为内侧关节囊过度重叠紧缩缝合5足（17．9％），矫形时过度纠正IMA4足（14-4％），术后长时期固定3足（10．8％），术后躅趾外展与内收肌力失衡2足（6．9％）。结论躅外翻矫形并发躅内翻最常见的原因是对跖骨头内侧骨赘切除过多。术者在治疗医原性躅内翻时，应根据各个病例的不同情况选择不同的术式进行治疗。采用Hiss、Myerson软组织重建术与Chevron跖骨远端截骨术矫正医原性跨内翻切实可行、疗效可靠。     

小儿体外循环采用中度低温低流量灌注技术的临床研究

目的研究先天性心脏病患儿体外循环手术中采用中度低温合并低流量灌注技术的可行性。方法３０例手术患儿体外循环降温至肛温２５．６±０．８４℃，食管温度２４．１±１．３２℃，鼓膜温度２３．８±１．４１℃，维持流量１．２３±０．０９Ｌ／ｍｉｎｍ２，转流时间９５．４±３４．６分钟，主动脉阻断时间５１．４±２０．２分钟，低流量维持时间４５．７±２２．４分钟。结果低流量灌注时静脉血氧饱和度均在８０％以上，除１例外，其余均无缺氧酸中毒的血气表现，血乳酸值也未升高，术后恢复良好。结论中度低温体外循环选用低流量灌注可满足机体代谢的需要，有利于减少长时间高流量灌注的不良反应。     

Successful management of laryngotracheosophageal cleft through an anterior approach

Laryngotracheoesophageal cleft is an uncommon disease that is difficult to diagnose and treat. Repair of the cleft depends on length and localization of the defect as well as the associated anomalies. A successful repair of a type II cleft is reported in this paper. An anterior split of the larynx and trachea was used and provided excellent exposure and safe repair without injury to the neurovascular structures. This is the best approach and should be used to correct all type II defects.     

Ischemic stroke in infancy,childhood, and adolescence

The authors studied 34 patients with juvenile ischemic cerebrovascular disease over a 15-year period. Of the 34 patients, 23 had intracranial occlusions attributed to cerebral thrombosis or embolism and 11 had occlusions resulting from moyamoya disease. Clinicopathological features were evaluated in the 23 cases with ischemic stroke, but not those with moyamoya disease. The cause of the arterial occlusion remained undetermined in 11 patients and was found to be an embolism based on congenital heart disease in 8, on trauma in 3, and on infection in 1. Cerebral angiography was performed in 21 patients. Of these, 17 had stenoses or occlusions corresponding to their symptoms. CT scans were performed in 10 patients; the lesion in question showed no stenosis or occlusion with cerebral angiography. With regard to prognosis, patients with unknown etiology had good outcomes compared with those with congenital heart disease. With respect to acute infantile hemiplegia, 10 patients had convulsive seizures and 4 had a history of an earlier infection. Angiography and CT scans in patients with congenital heart disease demonstrated arterial occlusive sites in the middle cerebral artery region. Three patients had abscesses after their ischemic lesions.     

Genetics of congenital hyperinsulinism

Congenital hyperinsulinism (CHI) is a clinically and genetically heterogeneous entity and causes severe hypoglycemia in neonates and infants. The clinical heterogeneity is manifested by severity ranging from extremely severe, life-threatening disease to very mild clinical symptoms, which may even be difficult to identify. Furthermore, clinical responsiveness to medical and surgical management is extremely variable. Recent discoveries have begun to clarify the molecular etiology of this disease in about 50% of cases. Mutations in five different genes have been identified in patients with this clinical syndrome. Most cases are caused by mutations in the genes ABCC8 and KCNJ11 coding for either of the two subunits of the beta-cell KATP channel (SUR1 and Kir6.2). Recessive mutations of the beta-cell K(ATP) channel genes cause diffuse HI, whereas loss of heterozygosity together with inheritance of a paternal mutation causes focal adenomatous HI. In other cases, CHI is caused by mutations in genes coding for the beta-cell enzymes glucokinase (GK), glutamate dehydrogenase (GDH), and SCHAD. However, for as many as 50% of the cases, no genetic etiology has yet been determined. The study of the genetics of this disease has provided important new information regarding beta-cell physiology.     

Infected infradiaphragmatic retroperitoneal extralobar pulmonary sequestration: a case report

Infradiaphragmatic extralobar pulmonary sequestration is an extremely rare congenital malformation. It is more frequently diagnosed in the antenatal period due to routine ultrasonic examination of the fetus or in the first 6 months of life, though on rare occasions it is discovered incidentally in adults. A 32-yr-old man presenting with epigastric discomfort and fever was referred. Computed tomographic scanning showed that a 16-cm, multiseptated, dumbbell-shaped, huge cystic tumor was located beneath the diaphragm. On the next day, 850 mL of thick yellowish pus was drained by sonography-guided fine needle aspiration for the purpose of infection control and diagnosis, but no microscopic organisms were found in repeated culture studies. Surgical removal of the cyst was performed through thoracoabdominal incision and most of these pathologic lesions were removed but we could not find the feeding arteries or any fistulous tract to surrounding structures. Histopathologic study revealed that it was extralobar pulmonary sequestration and culture study showed that many WBC and necrotic materials were found but there were no microorganisms in the cystic contents. We report the first case of an infected infradiaphragmatic retroperitoneal extralobar sequestration which was administered a staged management and achieved an excellent clinical course.