Neonatal Hemochromatosis, Renal Tubular Dysgenesis, and Hypocalvaria in a Neonate

We report a neonate with neonatal hemochromatosis (NH), renal tubular dysgenesis (RTD), and hypocalvaria. NH is a fatal condition of the newborn, characterized by severe idiopathic liver failure of intrauterine onset and siderosis, intra- and extrahepatic, with sparing of the reticuloendothelial system. RTD is characterized by short, abnormally developed cortical tubules that lack proximal tubule differentiation. Although both NH and RTD have been reported as entities with a genetic component, similar findings can be secondary to in utero insults. Hypocalvaria has been reported in association with fetal hypoxia including that secondary to angiotensin converting enzyme inhibitors. This 38-week-old infant died at 8.5 h. The small nodular liver weighed 44 g. Grossly, the kidneys were normal. Hypocalvaria was present. Microscopically, the hepatic parenchyma was distorted by fibrous tracts, proliferation of bile ducts, and abundant iron deposition in hepatocytes. Extrahepatic siderosis in the pancreas, myocardium, and other organs was consistent with NH. Proximal convoluted tubules were not seen on routine stains and markers for proximal tubules were negative. Previous reports have linked NH with RTD and RTD with hypocalvaria. This infant had all three of these rare conditions, which have been hypothesized or shown to be due to genetic factors, hypoxia, or drugs. The etiology in this case is unknown. Received May 20, 1997; accepted August 15, 1997.     

血糖水平对缺氧缺血新生大鼠脑内GLUT3合成的影响

目的 探讨血糖水平对缺氧缺血新生大鼠脑内葡萄糖转运蛋白3(glucose transporter 3，GLUT3)合成的影响。方法 在成功建立了缺氧缺血合并高，低血糖新生大鼠模型的基础上，应用免疫组化方法定量检测新生大鼠脑内海马和皮质部位GLUT3的合成。结果 正常情况下GLUT3随日龄增加而合成增加；缺氧缺血可引起GLUT3合成在短期内明显增加，但在HI后期降至较低的水平；HI前低血糖使GLUT3合成在HI后期更进一步降低；HI前重度高血糖则可引起各时段GLUT3合成明显增加，尤其在HI后期仍保持一定的水平。结论 在缺氧缺血前预先补充足量葡萄糖，有可能在一定程度上提高脑内应对缺氧缺血的侵袭、改善缺氧缺血程度的能力。     

纳络酮对缺氧缺血性脑病新生儿血浆神经肽Y和β-内啡肽的影响

目的探讨新生儿缺氧缺血性脑病(HIE)血浆神经肽Y(NPY)、β-内啡肽(β-EP)的变化及纳络酮治疗后对其的影响。方法将34例中、重度HIE患儿随机分成常规治疗组(18例),纳络酮治疗组(16例),以14例正常新生儿为对照组,纳络酮治疗组入院后在常规治疗的基础上给予纳络酮治疗,连用3天。HIE患儿组治疗前、治疗3d后各采血收集标本一次,采用放射免疫法测定NPY、β-EP。结果①HIE患儿血浆NPY、β-EP水平为(174.23±18.31)ng／L、(123.36±16.42)ng／L均显著高于正常对照组(87.19±12.95)ng／L、(63.27±12.65)ng／L(P<0.01)。HIE急性期NPY与β-EP呈正相关(r=0.347,P<0.05)。②HIE常规组、纳络酮组治疗3d后血浆NPY、β-EP水平均较治疗前显著降低(P<0.01)。治疗后HIE纳络酮组NPY、β-EP水平均显著低于常规组(P<0.01)。结论NPY、β-EP共同参与了HIE的病理生理过程,在HIE发病机制中可能起重要作用;纳络酮能显著降低NPY、β-EP水平,减轻脑损伤。     

Current controversies regarding pain assessment in neonates

Although over 40 methods of pain assessment in infants are available for use in clinical practice, unrecognized and under-treated pain remains one of the most commonly reported problems within the Neonatal Intensive Care Units. A number of factors have been found to account for differences in the robustness of the pain response in neonates of varying gestational ages. Discrepancies between behavioral and physiological pain indicators have also been reported. With newer technologies, there is an opportunity not only to verify infant pain perception, but these tools may allow an identification of which of the observed indicators are most sensitive in particular clinical situations. The current controversies regarding pain assessment in preterm and term infants are reviewed to define the most important issues and to develop a dialogue for future directions.     

Premedication for tracheal intubation in neonates: confusion or controversy?

Tracheal intubation is performed frequently in the NICU and delivery room. This procedure is extremely distressing, painful, and has the potential for airway injury. Premedication with sedatives, analgesics, and muscle relaxants is standard practice for pediatric and adult intubation, yet the use of these drugs is not common for intubation in neonates. The risks and benefits of using premedications for intubating unstable newborns are hotly debated, although recent evidence shows that premedication for non-urgent or semi-urgent intubations is safer and more effective than awake intubations. This article reviews clinical practices reported in surveys on premedication for neonatal intubation, the physiological effects of laryngoscopy and intubation on awake neonates, as well as the clinical and physiological effects of different drug combinations used for intubation. A wide variety of drugs, either alone or in combination, have been used as premedication for elective intubation in neonates. Schematically, these studies have been of three main types: (a) studies comparing awake intubation versus those with sedation or analgesia, (b) studies comparing different premedication regimens comprising sedatives, analgesics, and anesthetics, and (c) case series of neonates in which some authors have reported their experience with a specific premedication regimen. The clinical benefits described in these studies and the need for pain control in neonates make the case for using appropriate premedication routinely for elective or semi-urgent intubations. Tracheal intubation without the use of analgesia or sedation should be performed only for urgent resuscitations in the delivery room or other life-threatening situations when intravenous access is unavailable.     

无创持续气道正压通气治疗新生儿呼吸衰竭的临床研究

目的：探讨采用无创持续气道正压通气（CPAP）治疗新生儿呼吸衰竭的临床疗效。方法选择2011年1月至2013年9月我院收治的呼吸衰竭新生儿168例,按照随机数字表法分为观察组和对照组各84例。观察组采用新生儿无创呼吸机进行经鼻CPAP治疗,对照组采用头罩法吸氧。观察两组治疗前和治疗1 h后的动脉血气分析变化,评价临床疗效,统计转有创机械通气率。结果观察组和对照组临床总有效率分别为84.5%和67.9%,观察组明显高于对照组,差异具有统计学意义（P＜0.05）;观察组和对照组转有创机械通气率分别为15.5%和32.1%,观察组明显低于对照组,差异具有统计学意义（P＜0.05）;治疗前两组PaO2、SaO2、PaCO2水平比较差异均无统计学意义（P＞0.05）;治疗后,两组PaO2、Sa O2均较治疗前明显升高,PaCO2较治疗前明显降低,观察组改善程度明显优于对照组,差异具有统计学意义（P＜0.05）;两组pH值治疗前后比较差异无统计学意义（P＞0.05）。结论采用无创CPAP治疗新生儿呼吸衰竭临床疗效显著,可迅速改善缺氧状态,降低有创机械通气率,值得临床推广应用。     

美罗培南治疗新生儿耐碳青霉烯类肺炎克雷伯菌败血症27例分析

目的 探讨美罗培南治疗新生儿耐碳青霉烯类肺炎克雷伯菌（CRKP）败血症的疗效及其影响因素,为临床合理使用抗生素提供参考依据。方法 采用回顾性研究方法,收集2014年6月至2018年6月在上海交通大学附属儿童医院新生儿科住院的27例CRKP败血症患儿的临床资料,分析美罗培南在27例患儿中的治疗效果。根据治疗效果将患儿分为美罗培南单药治疗有效组和美罗培南单药治疗无效需联合治疗组,比较两组患儿围产因素、感染CRKP前接受碳青酶烯类抗生素暴露等临床特点的差异。结果 美罗培南单药治疗新生儿CRKP败血症有效率为48.1%（13/27）,采用联合治疗后总体有效率为74.1%（20/27）。美罗培南单药治疗无效需联合治疗组患儿存在外科手术后开放性伤口（7/14 vs 1/13）、感染性休克（7/14 vs 1/13）、无菌体腔液（脑脊液及腹水）培养阳性（6/14 vs 0/13）、感染时需有创机械通气（10/14 vs 1/13）的患儿比例高于单药治疗有效组（P均<0.05）,纸片扩散法药物敏感性试验中美罗培南抑菌圈直径小于单药治疗有效组[（9.14±3.37）mm vs（12.85±5.27）mm,P<0.05]。结论 美罗培南单药治疗新生儿CRKP败血症具有一定疗效。当CRKP败血症患儿存在外科手术后开放性伤口、感染性休克、无菌体腔液培养阳性、感染时需有创机械通气及药物敏感性试验中美罗培南抑菌圈直径偏小时需联合治疗,以提高治疗有效率。     

Predictability model of the need for extracorporeal membrane oxygenation in neonates with meconium aspiration syndrome treated with inhaled nitric oxide

Background

As the use of inhaled nitric oxide (iNO) resulted in a decline in the need for extracorporeal membrane oxygenation (ECMO) in neonates with hypoxic respiratory failure, iNO has become an accepted treatment modality even in non-ECMO centers. However, because not all neonates respond to iNO, the timely identification and transfer of nonresponders to an ECMO center are important.

Objectives

The objective of this study was to identify the risk factors predictive of the need of ECMO in neonates with hypoxic respiratory failure after the first 6 hours of iNO treatment in an ECMO center.

Methods and Patient Population

Forty-nine patients with hypoxic respiratory failure transferred for iNO therapy and potential ECMO during a 2-year period were identified in this retrospective study. None of the patients had received iNO before admission. Strict clinical guidelines were used to standardize lung inflation, cardiovascular support, and iNO administration and weaning and to define treatment failure. The relationship between treatment failure (ie, the need for ECMO) and a set of suspected risk factors after 6 hours of iNO administration was examined by logistic regression analysis.

Results

Twenty-two neonates responded to iNO (non-ECMO group) whereas 27 neonates failed and met ECMO criteria (ECMO group). There was no difference between the 2 groups in demographic data, ventilatory support, air leak syndrome at 6 hours of iNO treatment, and survival to discharge. However, the dose and duration of iNO therapy were predictive of the need for ECMO with an adjusted odds ratio of 1.12 (95% CI, 1.01-1.25; P = .04) and 0.45 (95% CI, 0.27-0.65; P = .0002), respectively.

Conclusions

By the end of the first 6 hours of iNO treatment and under the specific conditions established by the use of the clinical guidelines, the dose and the duration of iNO administration were predictive of the probability for the need of ECMO in this patient population. Thus, one can establish a center-specific predictability model for the need of ECMO in neonates with hypoxic respiratory failure treated with iNO if strict clinical guidelines for iNO administration and weaning and respiratory and cardiovascular support are used in the given center.     

Remifentanil for sedation and analgesia in a preterm neonate with respiratory distress syndrome

We present the efficacy and safety of the use of remifentanil for intubation, sedation and analgesia in a preterm infant during mechanical ventilation for respiratory distress syndrome. A 34-week-old baby, born by cesarean delivery that developed respiratory distress, required intubation and ventilatory support. For intubation, the baby was given midazolam (0.2 mg.kg(-1)) and remifentanil (1 microg.kg(-1)). The intubation conditions were assessed and classified as excellent. The remifentanil infusion was started at dose 0.75 microg.kg(-1).min(-1) and the dose adjustments were made depending on the neonatal infant pain scale (NIPS), hemodynamic and respiratory changes or the presence of spontaneous movements. Pulse oximetry, respiratory rate, ECG and invasive blood pressure were continuously monitored. He was given surfactant within 2.5 h of life after which ventilator parameters could be progressively decreased. Three hours later, the remifentanil infusion was decreased to 0.5 microg.kg(-1).min(-1), and he remained sedated (NIPS < 2). Six hour after surfactant administration, blood gases and chest X ray were normal. The remifentanil infusion was then discontinued and 30 min later the baby was awake and extubated with success. There were no side effects after intubation or during the continuous infusion. The profile of remifentanil allowing a rapid recovery, the absence of side effects and a good level of sedation and analgesia support the choice of this opioid for sedation in the NICU.     

Ethical concerns in the management of pain in the neonate

The debate about the management of pain in the neonate has continued to evolve over the past 30 years. This controversy can be understood as evolving through now three eras of thought about the effect of pain and its management in newborns and infants. The first generation was characterized by a widespread belief that newborns lacked the complete development of the neuroanatomical and neuroendocrine components necessary to perceive pain. During this period, newborns often received inadequate anesthesia and analgesia for painful procedures, if not no treatment at all. The second generation was heralded by research that demonstrated that newborns did demonstrate similar or even exaggerated physiological and hormonal responses to pain compared with those observed in older children and adults and that exposure to prolonged or severe pain may increase neonatal morbidity. Controversy in this generation focused around the dosage of analgesia to newborns as well as the risks and benefits of pain management techniques. We are now in a third generation of thought about pain in the neonate, defined by intense debate over the significance of a growing number of studies in immature animal models that demonstrate degenerative effects of several anesthetics on neuronal structure. The challenge of this era is to integrate the advances in diagnosis and treatment achieved in previous generations with ongoing adaptation of clinical practice as dictated by research advances in the field. In this review, we examine the evolution of medical thought and ethical concerns regarding pain treatment in the neonate.