腹腔镜辅助胆道造影在幼婴延迟性黄疸诊断中的价值

目的探讨腹腔镜辅助胆道造影在幼婴延迟性黄疸诊断中的价值。方法首先脐部置镜观察胆囊及肝脏情况,如果胆囊大小正常或基本正常,通过右肋缘下trocar孔拖出胆囊底部,部分患儿需游离胆囊系膜。切开胆囊,插管行胆道造影。如果胆囊呈纤维闭锁状态,不能拖出腹腔外,中转开腹。切开胆囊插管失败,放弃造影。结果12例患儿直接从拖出胆囊行胆道造影显示婴儿肝炎综合征或胆汁淤积8例;2例显示为胆管发育不良;2例为肝内胆管闭锁。5例从肝脏游离胆囊系膜后拖出造影,2例显示为胆管发育不良,3例提示胆道闭锁。其余21例患儿胆囊呈条索状,不同程度肝硬化,放弃胆道造影,诊断为胆道闭锁。所有胆道闭锁患儿均行Kasai手术。结论腹腔镜探查、胆道造影是诊断胆道闭锁、鉴别幼婴延迟性黄疸简便、准确、安全的方法,能够显示整个胆树的解剖结构,避免过大或不必要的创伤。     

Renal acid excretion in early infancy

In early infancy, complex disorders of acid base metabolism are more frequent than in any other age group, with a predisposition to metabolic acidosis due to an age-related low renal capacity for acid excretion and an unphysiologically high actual renal acid load in nutrition with common formulas. Recently in preterm and small-for-gestational-age infants, persistent maximum renal net acid excretion (NAE) with subnormal or normal blood acid base status, impaired weight gain, and adaptive hormonal reactions have been observed. Incipient late metabolic acidosis is one example of a mixed disorder of acid base metabolism with maximum renal NAE in early infancy. Alkali therapy is highly effective and can be realized both on an individual basis, using urine pH screening as a diagnostic criterium for maximum renal acid stimulation, or on a general preventive level using modified standard formula with a reduced actual renal NAE similar to that seen on alimentation with human milk. From an integrated point of view, the low glomerular filtration rate and renal capacity for acid excretion beyond the developmental age of more than 44 weeks, may well be interpreted as the result of a specific adaptation to breast feeding sparing energy, and thus an evolutionary advantage for the survival of mother and child. Received July 10, 1996; received in revised form and accepted October 7, 1996     

33例婴幼儿复发性喉乳头状瘤的观察与护理

目的探讨婴幼儿复发性喉乳头状瘤的观察与护理。方法对33例婴幼儿复发性喉乳头状瘤患儿密切观察病情,以赢取抢救机会;进行有效的术前术后护理,包括完善术前准备和进行术前护理评估及术后并发症的预防、气管切开术后呼吸道管理等。结果33例中31例均好转出院,2例病情变化抢救无效死亡。结论婴幼儿复发性喉乳头状瘤患儿的术前、术后观察与护理对及时发现患儿病情变化,进行有效的抢救措施至关重要。     

婴幼儿患者手术的护理要点

目的进一步认识婴幼儿患者实施外科手术的危险性及规范手术配合程序的重要性,提高配合质量,提出手术配合要点。方法回顾近几年168例婴幼儿患者外科手术护理配合,同时结合自己的临床实际经验和手术配合中的体会,提炼出婴幼儿患者手术的护理配合要点。结果婴幼儿患者手术的配合要做好术前评估,掌握患者心肺主要脏器的功能情况,做好充分准备,手术室感染控制,术中的各项检测,监测输液量、速度及体温维持情况。结论掌握婴幼儿患者手术的护理配合要点,可提高手术配合质量和手术成功率。     

极低体质量近存活期分娩儿一例的出生后早期管理

近存活期分娩（PVB）儿各组织系统发育极不成熟,易发生各种器官功能损害和并发症,预后常不佳。目前关于PVB儿出生后早期管理的报道不多,诸多尚待解决的问题仍是围产医学所面临的挑战。经有效的新生儿复苏和呼吸循环支持、积极的营养支持和喂养、防治感染、内分泌和代谢性疾病管理、积极处理早产儿相关并发症,以及发育支持护理、家庭参与式护理等一系列个体化管理和精细化护理,四川省医学科学院·四川省人民医院成功救治了1例胎龄23周,出生体质量（BW）为450 g的PVB儿。本文通过总结1例PVB儿的出生后早期管理,并结合文献复习进行归纳总结,以期为提高BW<500 g的PVB儿存活率并获得良好预后提供经验及借鉴。     

Nasotracheal intubation using fiberoptic bronchoscopy in infants and children

Journal of Anesthesia -     

新生儿G6PD缺陷病和晚发性维生素K缺乏症的特点与预防

目的 探讨新生儿C6PD缺陷病和晚发性维生素K缺乏症的危害与预防措施。方法 回顾分析1995-2000年儿内科住院的1周-2月(不含2月)的婴儿3104例次，其中病死56例。结果 1周-2月的小婴儿占住院患儿的19．34％，其中新生儿G6PD缺陷病239例，占7．70％；晚发性维生素K缺乏症92例，占2．96％。死因的第2、3位分别是晚发性维生素K缺乏症(13例，占23．21％)和新生儿C6PD缺陷病(12例，占21．43％)，两者的病死率分别为14．13％和5．02％，极显著高于(x^2＝17．59，P＜0．01)或相近于(x^2＝0．88，P＞0．05)肺炎的3．57％。新生儿G6PD缺陷病合并感染占38．49％、低氧血症占23．35％、低血糖占19．25％、酸中毒占15．90％，继发胆红素脑病占13．81％。晚发性维生素K缺乏症出现抽搐占90．22％、胃肠、注射部位出血占60．89％；CT证实颅内出血占98．91％。结论 1周-2月的小婴儿约占住院患儿的两成，新生儿G6PD缺陷病和晚发性维生素K缺乏症的病死率均很高，两者是除肺炎外最主要的死因。提议制定并推广预防这2种疾病的常规措施，并参照国内外相应的现状拟出其具体内容。     

婴儿测试行为与智力发展相关性的研究

目的：分析自行编制的一套婴幼儿测试行为评定量表集中反映了哪些行为倾向，并探讨测试行为与智力与运动能力之矧的关系。方法：对象为227名6—12个月的正常婴儿（男130名，女97名），采用贝利婴幼儿发展量表（BSID）和自编的婴儿测试行为评定量表作为研究工具。应用因子分析、方差分析和相关分析的统计学方法进行统计分析。结果：提取出目标定向性，觉醒状态，负性情绪，正性情绪，反应性，活动量共6个因子，方差贡献率为65．43％。目标定向性、觉醒状态、正性情绪、反应性与智力发育指数（MDI）存在明显的相关，目标定向性，正性情绪，反应性与运动发育指数（PDI）存在明显的相关。结论：婴儿测试行为与智力发展相关。     

The development of goal-directed reaching in infants: hand trajectory formation and joint torque control

Nine young infants were followed longitudinally from 4 to 15 months of age. We recorded early spontaneous movements and reaching movements to a stationary target. Time-position data of the hand (endpoint), shoulder, and elbow were collected using an optoelectronic measurement system (ELITE). We analyzed the endpoint kinematics and the intersegmental dynamics of the shoulder and elbow joint to investigate how changes in proximal torque control determined the development of hand trajectory formation. Two developmental phases of hand trajectory formation were identified: a first phase of rapid improvements between 16 and 24 weeks of age, the time of reaching onset for all infants. During that time period the number of movement units per reach and movement time decreased dramatically. In a second phase (28–64 weeks), a period of fine-tuning of the sensorimotor system, we saw slower, more gradual changes in the endpoint kinematics. The analysis of the underlying intersegmental joint torques revealed the following results: first, the range of muscular and motiondependent torques (relative to body weight) did not change significantly with age. That is, early reaching was not confined by limitations in producing task-adequate levels of muscular torque. Second, improvements in the endpoint kinematics were not accomplished by minimizing amplitude of muscle and reactive torques. Third, the relative timing of muscular and motion-dependent torque peaks showed a systematic development toward an adult timing profile with increasing age. In conclusion, the development toward invariant characteristics of the hand trajectory is mirrored by concurrent changes in the control of joint forces. The acquisition of stable patterns of intersegmental coordination is not achieved by simply regulating force amplitude, but more so by modulating the correct timing of joint force production and by the system's use of reactive forces. Our findings support the view that development of reaching is a process of unsupervised learning with no external or innate teacher prescribing the desired kinematics or kinetics of the movement.     

ADRENOCORTICAL CARCINOMA IN INFANCY

A case of adrenocortical carcinoma associated with congenital heart defect in a 6-month-old Japanese girl is reported. A fist-sized tumor was incidentally noted in the right hypochondrium upon admission for cardiac surgery. No clinical endocrinopathy was evident in this case. The resected tumor was encapsulated with smooth surface and no invasion to adjacent tissues or organs was observed. Histologically, the tumor was composed of small cells with granular or clear cytoplasm, and occasional giant cells with single or multiple nuclei. By electron microscopy, the tumor cells showed various nuclear contours with distinct nucleoli and had a moderate amount of cytoplasm containing abundant rough endoplasmic reticulum and mitochondria with variable-sized electron-dense granules. Intercellular desmosome-llke junctions were observed in some tumor cells. Immunohistochemlcally, the tumor cells contained granules positive for estriol, progesterone and Cortisol. These morphological findings including electron microscopic features suggested that the tumor cells had a malignant character.