Osteoporosis in severe congenital neutropenia treated with granulocyte colony-stimulating factor

Recombinant human granulocyte colony-stimulating factor (G-CSF) has substantially improved life expectancy for children with severe congenital neutropenia (SCN). Severe osteoporosis, reported in this population, may relate to the disease process, or be a therapeutic side-effect. This report details bone loss, quantitated absorptiometrically and histomorphometrically, in a child with SCN and vertebral collapse, and the positive response to anabolic steroid and bisphosphonate therapy.     

The dysmorphic lung: imaging findings

Congenital lung malformations are not infrequent and can be discovered in adults. It is, therefore, necessary to know their radiological manifestations in order to avoid diagnostic errors. We classify the congenital lung malformations in two main groups: dysmorphic lung and focal pulmonary malformations. We review the radiological spectrum of dysmorphic lung, based on a classification that emphasises the pulmonary abnormality, adding variants when diaphragmatic or venous abnormalities are present. In our opinion this approach allows for a rational use of advanced imaging techniques (CT, MRI). Correspondence to: Josep M. Mata.     

Type classification of anomalous pancreaticobiliary junction by ERCP

The role of endoscopic retrograde cholangiopancreatography (ERCP) in the preoperative assessment of anomalous pancreaticobiliary junction was retrospectively evaluated in 74 consecutive patients (19 males and 55 females; aged 0–80 years). Sixty-three patients had congenital biliary dilatation and 11 did not. Type classification of congenital biliary dilatation was possible by ERCP alone in 45 patients (71%). The main causes of classification failure were previous bilio-enteric anastomosis and restriction of postural changes during ERCP due to general anesthesia in pediatric patients. Classification of anomalous junction was possible in 69 patients (93%). Technical difficulty in ERCP caused classification failure in 5 patients. Neoplastic lesions were found in 12 patients (16%) and all but 1 were correctly diagnosed by ERCP. We conclude that ERCP plays an important role in the preoperative diagnosis and type classification of anomalous pancreaticobiliary junction and congenital biliary dilatation.     

小儿先天性巨结肠42例手术疗效分析

手术治疗小儿先天性巨结肠４２例，其中Ｄｕｈａｍｅｌ术９例，Ｓｗｅｎｓｏｎ术１１例，Ｓｏａｖｅ术６例，Ｒｅｈｂｅｉｎ术１０例，直肠后壁肌切除４例，直肠肛管纵切心形斜吻合术２例。术后污粪、便秘、小肠结肠炎及肛门狭窄发生率２１％。认为：手术疗效是与术前充分准备，选择适宜术式，术中精细操作，术后加强护理，精心喂养等直接相关     

Revascularization of a thrombosed aortopulmonary shunt with the use of the AngioJet thrombectomy system.

We report the use of the AngioJet F140 rheolytic catheter to recannalize an acutely thrombosed aortopulmonary shunt in a 21-year-old female with palliated, complex congenital heart disease. After extracting the thrombus that filled the entire length of the shunt, three stents were placed at sites where the lumen was compromised by distortion or thrombus. Unobstructed flow was restored to the left pulmonary artery that persisted at 1-year follow-up.     

Congenital esophageal atresia successfully treated by early ligation of a tracheoesophageal fistula and delayed repair of the esophagus in a premature infant: Report of a case

We report herein the case of a premature infant with esophageal atresia (EA) and a tracheoesophageal fistula (TEF) associated with cardiac anomalies who was successfully treated by an early ligation of the TEF following gastrostomy, and delayed repair of the esophagus. A 1212-g male was born prematurely at 31 gestational weeks, at which time he was diagnosed as having EA with TEF and patent ductus arteriosus (PDA), ventricular septal defect (VSD), and atrial septal defect (ASD). A gastrostomy was initially performed but following extubation he gradually became tachypneic. A chest roentogenogram revealed atelectasis and ground-glass appearance, and reintubation was required. Ligation of the TEF was performed 53h after his birth. Following the improvement of his respiratory condition through ventilatory support and the intratracheal administration of pulmonary surfactant, he underwent repair of the esophagus on the 6th day of life. Postoperatively, he suffered from heart failure, but was treated with peritoneal dialysis and pharmacological closure of the PDA. Weaning the infant from the ventilator proved difficult, but it was finally achieved when he had reached a weight of 2268g at 3 months of age by enteral feeding. Our experience of this case demonstrates that early ligation of TEF should be performed for a premature infant with EA and TEF before respiratory distress syndrome (RDS) has developed. If a gastrostomy is required to prevent gastric distention, it should be followed by simultaneous or immediate ligation of the TEF.     

先天性心脏病患儿术后多脏器功能障碍的预后分析

目的 探讨小儿先天性心脏病(先心病)术后多脏器功能障碍(MODS)预后情况及其影响因素,为对此类患儿进行针对性护理提供依据.方法 收集先心病术后并发MODS 77例患儿的临床资料.结果 11例放弃治疗出院,66例中44例救治存活,22例死亡.出现时间最早、累及最多的脏器为心脏;病死率最高的为累及中枢神经系统的患儿(57.69%),其次是累及血液系统的患儿(55.56%);患儿的病死率与累及脏器的数量呈显著正相关(P＜0.01).死亡患儿手术体外循环时间和主动脉阻断时间显著长于存活患儿(均P＜0.05),术中意外及术后心肺复苏发生率显著高于存活患儿(均P＜0.05).结论 先心病术后患儿应加强心功能监护,特别是体外循环时间＞120 min,主动脉阻断时间＞60 min及术中发生过意外情况、术后采取过心肺复苏术的患儿;尽早采取有利措施避免其他脏器功能受损是提高患儿存活率的关键.     

Ross procedure in an infant weighing 4.5 kg

A 6 month-old male infant (weight: 4.5 kg) with congenital aortic stenosis underwent aortic valve replacement with a pulmonary autograft (Ross procedure). The right ventricular outflow tract (RVOT) was reconstructed with a polytetrafluoroethylene (PTFE)-valved equine pericardial conduit. At the age of 5, re-RVOT reconstruction with an equine pericardial patch bearing a PTFE monocusp was required because of severe pulmonary stenosis resistant to 2 attempts of percutaneous transluminal pulmonary valvotomy. Currently, at the age of 8, the degree of aortic regurgitation is trivial and the pulmonary autograft is free of functional deterioration despite somatic growth.     

Pemberton手术为主多种辅助手段治疗大龄儿童先天性髋脱位

目的　应用Pemberton手术为主多种辅助手段治疗大龄儿童先天性髋脱位 ,从而降低术后髋关节脱位、僵硬和股骨头坏死发生率。方法　自 1989年 8月 - 1999年 8月 ,用Pemberton手术为主多种辅助手段治疗大龄儿童 (7～ 14岁 )先天性髋脱位 4 6例 (5 8髋 ) ,随访 2～ 9年 ,平均 5年 3个月。辅助手段包括 :①髂腰肌及内收肌切断 ,股骨髁上骨牵引 ;②股骨粗隆下旋转加短缩截骨 ;③单髋人字石膏制动 ;④髋关节早活动 ,晚负重 ;⑤术后被动活动髋关节加主动功能锻炼。结果　髋关节完全复位率10 0 % ,髋关节屈曲小于 90°者 4髋 ,占 6 9% ,按Salter提出的股骨头坏死诊断标准 4 6例 (5 8髋 )中 ,股骨头坏死 6髋 ,占 10 3% ,明显降低了大龄儿童先天性髋脱位术后髋关节僵硬、股骨头坏死率。结论　Pemberton手术为主多种辅助手段治疗大龄儿童先天性髋脱位疗效确切 ,优良率高。