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Management of critically ill very low-birth-weight infants with the clinical picture of vasopressor-resistant shock in the neonatal intensive care unit is a complex issue. Once airway and breathing have been stabilized and hypotension has been treated with volume and vasopressors with no improvement, the issue of cardiovascular insufficiency must be addressed. After cultures have been performed and antibiotic therapy started, the question of adrenal insufficiency arises. This article discusses of the development of the hypothalamic-pituitary-adrenal axis during gestation, aspects of what is considered normal function, and the diagnosis and management of the entity of relative adrenal insufficiency in the premature infant.

Background Literature suggests a paucity of formal training in end-of-life care in contemporary American medical education. Similar to trainees in adult medicine, paediatric trainees are frequently involved in end-of-life cases. Objective To determine current experience and comfort levels among paediatric trainees when caring for dying patients with the hypothesis that more clinical experience alone would not improve comfort. Methods Paediatric residents, subspeciality fellows and programme directors at the University of Chicago completed a voluntary electronic needs assessment in June and July 2013. Ten question pairs determined frequency of experiencing various aspects of end-of-life care in clinical practice and comfort levels during these encounters. Results 118 respondents participated (63.8 % response rate): 66.4 % were female; 53 % had previous education in end-of-life care. The proportion of those with experience in end-of-life care increased through the third year of training, and remained at 1.0 thereafter. Conversely, positive comfort scores increased gradually throughout all six years of training to a maximum proportion of 0.45. Comfort in many specific aspects of care lagged behind experience. Previous education had a significant positive effect on comfort levels of most, but not all, aspects of care. 58 % or more of trainees desired further education on specific end-of-life topics. Conclusions Paediatric trainees are often involved in end-of-life care but may not be comfortable in this role. More experience alone does not improve comfort levels; however, there is a positive correlation with comfort and previous education. Trainees had a strong interest in further education on a variety of end-of-life care topics. Electronic supplementary material The online version of this chapter (doi: 10.1007/s40037-015-0161-4) contains supplementary material, which is available to authorized users.

BACKGROUND: Since publication of the sentinel antenatal late preterm steroids clinical trial, the use of antenatal steroids has become a routine aspect of the management of pregnancies at risk for late preterm delivery. However, in practice, the administration of antenatal corticosteroids in the late preterm period is widely varied across provider and institution, and the process of implementation of this new practice as well as outcomes associated with implementation are not well understood. OBJECTIVE: The objective was to evaluate institutional adherence to an antenatal late preterm corticosteroid protocol and to assess neonatal outcomes associated with its introduction. STUDY DESIGN: This is a retrospective cohort study of all women with singleton pregnancies admitted between 34 to 36 5/7 weeks who presented in the year before (“pre-protocol”: November 2012 to October 2013) and after implementation (“post-protocol”: April 2016 to March 2017). The protocol recommends corticosteroid administration to women 34 to 36 5/7 weeks gestation at risk for preterm birth who have not received prior corticosteroids. Women with fetal anomalies or pregestational or gestational diabetes were excluded from analysis. The frequency with which eligible women received corticosteroids and ineligible women were appropriately excluded (adherence) was calculated on a monthly basis. Neonatal outcomes of interest were hypoglycemia, receipt of dextrose, birth weight, 5 minute Apgar less than 7, receipt of surfactant, respiratory distress syndrome, transient tachypnea of the newborn, neonatal intensive care unit length of stay, intraventricular hemorrhage, necrotizing enterocolitis, culture positive sepsis, bronchopulmonary dysplasia, and death. Bivariable and multivariable analyses were used to compare neonatal outcomes between 1) all women in the post-protocol cohort to those in the pre-protocol cohort and 2) only women who received adherent care in the post-protocol cohort to all women in the pre-protocol cohort. RESULTS: A total of 452 women were included in the pre-protocol cohort and 451 in the post-protocol cohort. The majority of the post-protocol women (N=366, 81.2%) received adherent care. Women in both cohorts were similar with the exception that women in the post-protocol cohort were more likely to be nulliparous (p=0.013). Compared to the pre-protocol period, neonates of women in the post-protocol period had significantly higher odds of hypoglycemia

A male infant is born at 324/7 weeks of gestation with a birth weight of 2,870 g. He is born to a 27-year-old gravida 2, para 1001 mother who received appropriate prenatal care. The infant is noted to have severe isolated abdominal ascites on a 21-week ultrasonogram, which is confirmed with subsequent ultrasonography. No pleural or pericardial effusion, skin edema, or structural abnormalities in the heart are noted. Doppler ultrasonography reveals normal middle cerebral artery velocity. The mother’s blood group is O+, antibody test result is negative, and serologic test results for hepatitis B, human immunodeficiency virus, and syphilis are negative. The mother is rubella immune and tests group B Streptococcus negative. The results of Neisseria gonorrhea and Chlamydia tests are negative. IgM test results are negative for toxoplasma, parvovirus, varicella zoster virus, and cytomegalovirus. The mother presents with premature rupture of the membranes, which occurs 16 hours before delivery, and receives 2 doses of betamethasone. Before an elective low transverse caesarean delivery, 1.5 L of clear fluid is removed through amniocentesis and 180 mL through paracentesis secondary to concerns of fetal lung immaturity and abdominal distension. The infant is electively intubated at delivery in anticipation of respiratory distress from extensive ascites, administered one dose of surfactant, and given mechanical ventilatory assistance. Apgar scores are 6 and 8 at 1 and 5 minutes, respectively. The infant passes urine and meconium soon after birth. On physical examination, the infant is noted to have a distended abdomen and bilateral scrotal edema. Cord arterial and venous blood gas …

Aim To determine the boundaries of the grey zone of discretionary resuscitation over the past 20 years. Background As the likelihood of survival improves over time, the BW- and GA-specific boundaries of discretionary nonresuscitation should fall. Hypothesis Between 1988 and 2008 reductions in BW- and GA-specific mortality would drive a parallel reduction in BW and GA boundaries of discretionary resuscitation. Methods We determined the likelihood of resuscitation and survival to NICU discharge for all infants born

Atrial flutter (AFL) is the second most common type of tachyarrhythmia in the fetus and neonate. An atrial rate of 240 to 360 beats per minute, 2:1 atrioventricular conduction, and a “saw tooth” appearance on electrocardiogram (ECG) are characteristic. On echocardiogram, bilateral atrial dilatation is the most common finding. Treatment is dependent on the severity of symptoms; delivery is usually indicated in the case of fetal heart failure or hydrops fetalis, whereas postnatal AFL is most commonly treated with direct current cardioversion (DCC). This article presents an illustrative case in which the patient presented antenatally via abnormal nonstress testing and subsequent fetal echocardiogram that was concerning for AFL. Postnatal ECG confirmed this diagnosis and the patient received DCC on the day of birth, followed by digoxin and propranolol as maintenance therapy. [ Pediatr Ann . 2015;44(10):e247–e250.]

To examine the extent to which weight gain and weight status in the first 2 years of life relate to the risk of neurodevelopmental impairment in extremely preterm infants.In a cohort of 1070 infants born between 23 and 27 weeks' gestation, we examined weight gain from 7-28 days of life (in quartiles) and weight z-score at 12 and 24 months corrected age (in 4 categories:-2; ≥-2,-1; ≥1,1; and ≥1) in relation to these adverse neurodevelopmental outcomes: Bayley-II mental development index55, Bayley-II psychomotor development index55, cerebral palsy, Gross Motor Function Classification System ≥1 (cannot walk without assistance), microcephaly. We adjusted for confounders in logistic regression, stratified by sex, and performed separate analyses including the entire sample, and excluding children unable to walk without assistance (motor impairment).Weight gain in the lowest quartile from 7-28 days was not associated with higher risk of adverse outcomes. Children with a 12-month weight z-score-2 were at increased risk for all adverse outcomes in girls, and for microcephaly and Gross Motor Function Classification System ≥1 in boys. However, excluding children with motor impairment attenuated all associations except that of weight z-score-2 with microcephaly in girls. Similarly, most associations of low weight z-score at 24 months with adverse outcomes were attenuated with exclusion of children with motor impairment.Excluding children who have gross motor impairment appears to eliminate the association of low weight status with neurodevelopmental impairments at 2 years in extremely preterm infants.

In lieu of traditional training of examiners to identify cerebral palsy on a neurologic examination at age 1 year, we proposed an alternative approach using a multimedia training video and CD-ROM we developed after a two-step validation process. We hypothesized that use of CD-ROM interactive training will lead to reliable and valid performance of the neurologic examination by both pediatric neurologists and nonpediatric neurologists. All examiners were asked to take one of six interobserver variability tests found on the CD-ROM on two occasions. In the first interobserver variability evaluation, 89% (531 of 594) of the responses agreed with the gold standard responses. Following annotated feedback to the examiners about the two items that had a 60% correct rate, the correct response rate rose to 93% (114 of 123). In the second interobserver variability evaluation, 88% (493 of 560) of the responses agreed with the gold standard responses. Following annotated feedback to the examiners about the four items that had a 70% correct rate, the correct response rate rose to 96% (104 of 108). Interactive CD-ROM examination training is an efficient and cost-effective means of training both neurologists and non-neurologists to perform structured neurologic examinations in 1-year-old children. It provides an effective means to evaluate interobserver variability, offers a route for feedback, and creates an opportunity to reevaluate variability, both immediately and at periodic intervals. ( J Child Neurol 2005;20:829—831).

OBJECTIVES. For a cohort of extremely premature, ventilated, newborn infants, we determined the power of either serial caretaker intuitions of “die before discharge” or serial illness severity scores to predict the outcomes of death in the NICU or neurologic performance at corrected age of 2 years.METHODS. We identified 268 premature infants who were admitted to our NICU in 1999–2004 and required mechanical ventilation. For each infant on each day of mechanical ventilation, we asked nurses, residents, fellows, and attending physicians the following question: “Do you think this child is going to live to go home or die before hospital discharge?” In addition, we calculated illness severity scores until either death or extubation.RESULTS. A total of 17066 intuition profiles were obtained on 5609 days of mechanical ventilation in the NICU. One hundred (37%) of 268 profiled infants had ≥1 intuition of die before discharge. Only 33 infants (33%) with an intuition of die actually died in the NICU. Of 48 infants with even 1 day of corroborated intuition of die in the NICU, only 7 (14%) were alive with both Mental Developmental Index and Psychomotor Developmental Index scores of >69, and only 2 (4%) were alive with both Mental Developmental Index and Psychomotor Developmental Index Scores of >79 at corrected age of 2 years. On day of life 1, the Score for Neonatal Acute Physiology II value for nonsurvivors (38.2 ± 18.1) was significantly higher than that for survivors (26.3 ± 12.7). However, this difference decreased steadily over time as scores improved for both groups.CONCLUSIONS. Illness severity scores become progressively less helpful over time in distinguishing infants who will either die in the NICU or survive with low Mental Developmental Index/Psychomotor Developmental Index scores. Serial caretaker intuitions of die before discharge also fail to identify prospective nonsurviving infants. However, corroborated intuitions of die before discharge identify a subset of infants whose likelihood of surviving to 2 years with both MDI and PDI >80 is approximately 4%.

OBJECTIVE. We assessed physician preferences and physician prognostic abilities regarding delivery room management of exceedingly low birth weight/short gestation infants.METHODS. We surveyed US neonatologists to assess their behavior in the delivery room when confronted with infants with gestational ages of 22 to 26 weeks. We identified 102 infants in our NICU with birth weights/gestational ages of 400 g/23 weeks to 750 g/26 weeks, whose follow-up care was ensured because of their participation in ongoing clinical trials. We determined 4 proxy measures for “how the infant looked” in the delivery room (Apgar scores at 1 and 5 minutes and heart rates at 1 and 5 minutes) and assessed the predictive value of each marker for subsequent death or neurologic morbidity.RESULTS. For infants with birth weights of 600 g and gestational ages of 25 weeks, >90% of neonatologists considered resuscitation obligatory. For infants with birth weights of 500 to 600 g and gestational ages of 23 to 24 weeks, only one third of neonatologists responded that parental preference would determine whether they resuscitated the infant in the delivery room. The majority wanted “to see what the infant looked like.” For 102 infants with birth weights of ≤750 g, Apgar scores at 1 and 5 minutes and heart rates at 1 and 5 minutes were neither sensitive nor predictive for death before discharge, survival with a neurologic abnormality, or intact neurologic survival.CONCLUSIONS. The “gray zone” for delivery room resuscitation seems to be between 500 and 600 g and 23 and 24 weeks. For infants born in that zone, neonatologists' reliance on accurate prediction of death or morbidity in the delivery room may be misplaced.

In this issue of Pediatric Annals, the articles discuss newborns who present with common neonatal symptomology, but have rare diagnostic outcomes. In the first article, Drs. Alison Chu and Harvey K. Chiu discuss tachypnea and hypoglycemia, commonly presenting symptoms during the newborn period. In the illustrated case, the early and persistent hypoglycemia was a clue to the newborn’s underlying endocrinopathy, a known risk factor for necrotizing enterocolitis in a full-term newborn. In the next article, Dr. Theodore De Beritto and colleagues present an article discussing a vein of Galen arteriovenous malformation. The authors describe a newborn delivered secondary to decreased fetal movements, decreased fetal heart rate variability, and late decelerations. After delivery, the newborn had unusual physical findings including bilateral neck swelling, a varicosity over the left carotid area, a fontanel with a bruit, and an active precordium. Next, Dr. Joyce Woo and colleagues discuss tachycardia in a newborn. Tachycardia in the fetus that persists in the newborn period warrants prompt evaluation. A lack of treatment can result in up to a 20% mortality rate. The most common causes of fetal and neonatal tachycardia are sinus tachycardia and supraventricular tachycardia. The final article, by Dr. Owais A. Khan and colleagues, describes acute renal failure (ARF) in the neonate. Their illustrative case details a neonate with ARF secondary to renal vein thrombosis, which is an unusual cause of intrinsic ARF that can be serious, often leading to a need for dialysis. Neonatal symptomology often leads to common neonatal diagnoses. In the articles presented in this issue, the common symptoms of tachypnea, hypoglycemia, fetal distress, tachycardia, and the signs of acute renal failure ultimately lead the physicians to much less common diagnoses.

To assess the predictive value of early therapy for ventilated extremely low birth weight (ELBW) infants beyond information available at delivery.Prospective, single-center cohort analysis of 177 ventilated ELBW infants. We collected information known at delivery (gestational age, birth weight, singleton, sex, antenatal steroids) and additional information while infants were mechanically ventilated (head ultrasound scanning, clinician intuitions of death before discharge). An adverse outcome was defined as mortality or Bayley Mental Developmental Index or Psychomotor Developmental Index70 at 2 years. We compared the predictive ability of clinical variables separately, in combination, and in addition to information available at delivery.A total of 77% of infants survived to follow-up; 56% of survivors had Bayley Mental Developmental Index and Psychomotor Developmental Index ≥ 70. A total of 95% of infants with both abnormal head ultrasound scanning results and predicted death before discharge had an adverse outcome, independent of gestational age. Conversely, 40% of infants with normal head ultrasound scanning results and no predicted death before discharge had an adverse outcome, independent of gestational age. After adjusting for variables known at birth, predicted death before discharge and abnormal head ultrasound scanning results added significantly to the ability to predict outcomes.Information gained early in the neonatal intensive care unit improves prediction of mortality or neurodevelopmental impairment in ventilated ELBW infants beyond information available in the delivery room.

Objectives: To assess how p53 gene mutations and microvessel density (MVD) may be used as prognostic markers for the study and management of head and neck squamous cell carcinomas and to investigate putative associations between p53 gene mutations and MVD and the relationship of these factors to tumor response to radiotherapy and/or chemotherapy at 6 weeks. Patients and Design: Thirty-nine patients with squamous cell carcinoma of the head and neck, stages I to IV, who were examined at Rush-Presbyterian-St Luke's Medical Center, Chicago, 111, and its affiliated hospitals between 1993 and 1995 were monitored. Mutations in the p53 gene were identified by microdissection of tumor cells on frozen sections, followed by single-strand conformation polymorphism analysis of the products of polymerase chain reaction amplification of exons 5 to 9. The microvessels were immunostained with monoclonal antibodies to factor VIII and/or CD31. Microvessel counts were done by 2 investigators blinded to each other's counts and to the p53 gene status. Intratumoral or peritumoral microvascular hot spots were assessed and counts were done with an ocular grid in 3 X 200 fields of hot spots by each investigator. The mean of the highest values was considered. Statistical analysis was done with the Wilcoxon rank sum test, the log-rank test, and proportional hazard models. Results: Of the 39 patients, 13 had mutations in exons 5 to 9. Mutations in the p53 gene were associated with unfavorable overall (P=.003) and disease-free (P=.02) survival. A strong inverse relationship was seen between MVD and p53 mutations (P=.01). No statistically significant relationship was seen between mean MVD and overall and disease-free survival. The response to therapy differed significantly (P=.03) by p53 mutations, whereas there was no statistical significance with MVD counts. Conclusion: In this study a strong inverse relationship was seen between MVD and p53 mutations. p53 Mutations in exons 5 through 9 were associated with unfavorable survival, whereas MVD showed no association with survival.