Byler's disease (familial fibrogenic cholestasis in children). Apropos of 7 cases

Seven cases presenting with the features of Byler's disease (BD) are reported. The diagnosis of BD can only be made after exclusion of other causes of cholestasis. It seems likely that BD is a syndrome and that more accurate biological and histological investigations will allow to differentiate several entities within this affection. At the present, liver transplantation appears to be the only rational treatment.     

Mast cell disease: surgical and anesthetic implications

Mast cell disease (MCD) is a clonal disorder of the mast cell and its precursor cells. Cardiac surgery in MCD is rarely described. We report an unusual case of a 14-year-old girl who was admitted for atrial septal defect closure and incidentally found to have hepatosplenomegaly with lymphadenopathy who underwent a successful open-heart surgery, after a diagnosis of MCD.     

Pediatric obstructive sleep apnea syndrome and anesthetic management

Sleep-related breathing disorders require special attention in children who spend a considerable time sleeping. Obstructive sleep apnea syndrome is characterized by episodes of upper airway obstruction during sleep. Symptoms include hyperactivity, enuresis, headache, failure to thrive, and increased respiratory effort and total sleep time. The most common cause is adenotonsillar hypertrophy. Coexisting diseases are obesity, neuromuscular and craniofacial anomalies, and Down's syndrome. Early diagnosis is important to minimize neurocognitive, cardiac and developmental complications. Polysomnography is the gold standard for diagnosis. Although the features of pediatric obstructive sleep apnea syndrome are distinctly different from that in adults, it may predispose to the adult type of the syndrome. As therapy concerns several surgical approaches as well as conservative techniques, anesthetic management calls for particular attention. Pre- and postoperative sedation must be performed cautiously and patients must be watched closely with respect to airway obstruction and hypoventilation. Difficult intubation must always be considered.     

从先天性心脏病治疗方法的发展历史中学习正确的临床思维和规范的医疗行为

先天性心脏病(先心病)的诊断治疗,在儿科学和心脏外科学中占有重要地位,近年来已成为小儿心内外科的联合医疗任务,随着医疗设备和技术的不断发展,先心病的治疗效果不断提高.先心病的外科治疗开始于1938年,Gross及Hubbard首先成功地结扎未闭动脉导管,1953年Gibbon首次使用体外循环于房间隔缺损(ASD)手术,近60余年先心病的外科治疗发展迅速.     

A consideration of neonatal resuscitation

There are currently two major areas of resuscitation of the newborn which have come into question: the use of intermittent positive pressure ventilation and the use of oxygen. There is evolving evidence that volutrauma associated with IPPV, especially in the premature infant, may induce changes in the lung which can lead to chronic lung disease. There is reason to believe that the use of continuous positive airway pressure in premature infants who are making respiratory efforts may be less harmful than the use of IPPV. With regard to the use of oxygen, it is clear that most infants can be successfully resuscitated with room air. Although we can identify markers for oxidative stress in newborns when resuscitated with 100% oxygen, the clinical importance of these markers remain an open issue. If the presence of these markers after resuscitation is shown to relate to clinical problems, then the use of oxygen may need to be considered.     

Freeman-Sheldon syndrome: clinical manifestations and anesthetic and surgical management

We report a new sporadic case of Freeman-Sheldon syndrome. The parents were not blood relatives. The boy showed characteristic deformities of distal arthrogryposis in the hands and feet, as well as the typical features of "whistling face syndrome". In addition, the patient showed other clinical manifestations such as a large bilateral inguinal hernia and thoracic cage abnormalities. The latter abnormality led to serious episodes of bronchopneumonia that delayed the surgical repair of bilateral inguinal hernia. Knowledge of the sonographic characteristics of deformities of the extremities is essential to reach an early prenatal suspected diagnosis of Sheldon-Freeman syndrome, especially in families with a history of the syndrome. We describe the preanesthetic management, anesthetic method and surgical technique performed when the child was aged 9 months. The delay was due to recurrent episodes of bronchopneumonia.     

Perioperative anesthetic and analgesic management of newborn bladder exstrophy repair

ObjectiveReconstruction of bladder exstrophy in newborn infants requires immobilization, sedation and pain management to prevent distracting forces from compromising the repair. We present a 6-year review of our experience.Subjects and methodsWe reviewed the perioperative management of newborn infants undergoing reconstruction between November 1999 and October 2006. Data are presented as means ± SD.ResultsTwenty-three newborn infants underwent surgery under a combined epidural and general anesthetic technique. Tunneled caudal epidural catheters were inserted in all patients and intermittently injected with 0.25% bupivacaine with 1:200,000 epinephrine. Postoperatively, a continuous infusion of 0.1% lidocaine, 0.8–1 mg/kg/h was administered for 15 ± 8 (range 4–30) days. Children were sedated with diazepam for 20 ± 13 (range 2–40) days. Central venous catheters were maintained for 20 ± 9 (range 1–34) days for fluids, drug administration and blood sampling. No patient experienced bladder prolapse or wound dehiscence.ConclusionPerioperative management with tunneled epidural and central venous catheters in newborn infants with bladder exstrophy facilitates immobilization, analgesia and sedation, resulting in an excellent cosmetic repair with no case of bladder prolapse or wound dehiscence.     

Surgical and anesthetic decisions for children with terminal illness.

Terminal conditions such as congenital anomalies and cancer are a significant source of infant and childhood mortality. Many terminally ill children are considered for operative procedures each year. These procedures may be palliative or elective, and the prognosis and natural course of the terminal illness play a significant role in determining the appropriateness of the surgical procedure. Providing anesthesia to a terminally ill patient is a complex task requiring an appropriate balance between adequate anesthesia and hemodynamic normality. Some children with a terminal condition will have a standing "Do-Not-Resuscitate" order that should not necessarily be reversed. Surgeons, anesthesiologists, and family members must consider a number of factors when determining the appropriateness of an operation for a dying patient, including the rights of the child.     

脓毒症抗凝治疗成功与失败对我们的启示

脓毒症尤其是重症脓毒症仍是危重症抢救中的临床难点问题,有较高的发病率及病死率.目前的观点认为脓毒症是由于机体过度的炎症反应所致,这其中包括大量炎症介质的释放、补体系统及凝血纤溶系统、氧化系统的激活等.严重脓毒症导致凝血系统紊乱早已被人们注意到,但近年才对其在严重脓毒症发生发展及影响预后方面有一定的认识.本文仅就脓毒症抗凝治疗方面的进展做一回顾.     

Effects of topical anesthetic agents on Campylobacter pylori

The susceptibility of Campylobacter pylori was determined for the two topical anesthetic agents commonly used prior to gastroscopy. Campylobacter pylori proved to be extremely sensitive to the anti-infective activity of benzocaine, the active ingredient in most commonly used topical anesthetic agents, with minimum inhibitory concentrations of 0.14 mg/ml. However, lidocaine-containing agents did not interfere with the growth of this microorganism. Specifically, in patients with histologic evidence of C. pylori, the bacterium was recovered from significantly more patients anesthetized with lidocaine than with benzocaine. Thus, the use of benzocaine-containing topical anesthetic agents limits recovery of Campylobacter pylori from clinical specimens and might account for the low colonization rates reported in some recent publications.     

儿童院前急救转运系统的现状和思考

根据国内外转运系统现状,本文在简要介绍不同转运模式的基础上重点介绍儿童转运系统的范围、措施、质量控制、制度、相关培训和流程;分析儿童转运系统存在的主要问题和原因,就目前医院间院前转运的体系管理、重症儿童风险评估、区域性危重患儿转运网络的建立、现场急救与院前转运的关系等进行了讨论,也提出了儿童急救转运的发展模式.