Congenital Anomalies of the Kidneys and Urinary Tract.

Congenital anomalies of the kidneys and urinary tract encompass the spectrum of disorders that include the kidneys, ureters, bladder, and urethra. These abnormalities often lead to altered renal size and location, dysplastic changes in the kidney parenchyma, and anomalies in the collecting system. Though the etiology of each of these conditions can be variable, it is known that the collection of these defects represent 40% to 50% of all pediatric end-stage renal disease worldwide. The multifaceted management of these conditions is aimed at preserving kidney function and ultimately delaying the need for transplantation. With the advancement of prenatal ultrasonographic techniques, these conditions are more likely to be diagnosed before birth, which often leads to rapid postnatal intervention and better outcomes.

Hyperbilirubinemia and Choledocholithiasis in an Extremely Premature Neonate.

Cholestasis affects 2% of newborns admitted to the neonatal intensive care unit and 20% of premature infants and requires a thoughtful evaluation and diagnostic workup.There may be a single responsible etiology, or its development may be multifactorial. Premature neonates are especially predisposed because of their increased risk of infections and acute illness, need for parenteral nutrition, and exposure to certain medications. Clinically, an infant may present with jaundice, evidence of hepatic injury, or worsening hepatic function. Diagnosis may be made in consultation with various pediatric subspecialists including gastroenterology, genetics, and surgery. Treatment depends on the etiology but may include medications or surgical interventions. Timely recognition and intervention improve outcomes. [Pediatr Ann. 2023;52(8):e297-e302.].

Relative Adrenal Insufficiency in the Preterm Infant.

Identifying relative adrenal insufficiency in the critically ill preterm neonate is not always clear-cut. Preterm infants with vasopressor-resistant shock may have persistent cardiovascular insufficiency, which can result in rapid decompensation. After attempts of resuscitation with fluids and inotropes, these infants are often found to respond to glucocorticoids. This raises the important question of how prevalent adrenal insufficiency is in the preterm population. This article reviews the development and role of the adrenal glands, defines relative adrenal insufficiency in the preterm population, discusses barriers to determining this diagnosis, and describes treatment options.

Ureasplasma and Its Role in Adverse Perinatal Outcomes: A Review.

Human Ureaplasma species are the most common microbes found in amniotic fluid and in the placenta after preterm birth, and have previously been correlated with chorioamnionitis, preterm labor, and bronchopulmonary dysplasia, among other adverse birth and neonatal outcomes. Although these correlations exist, there still remains little explanation as to whether Ureaplasma plays a pathogenic role in the development of neonatal disease. In addition, Ureaplasma species are not usually identified on routine culture as they require special culture methods because of their fastidious growth requirements. Treatment of Ureaplasma with macrolides has been shown to effectively eradicate the bacteria in pregnant women and infants. However, it is unclear whether this leads to improved neonatal morbidity and mortality, or whether these generally represent commensal organisms. This review will synthesize the current perspectives about the proposed mechanisms of pathogenicity of Ureaplasma bacteria, its links to poor neonatal outcomes, and the role of screening and treatment in current clinical practice.

Maternal-to-Fetal Transmission of Syphilis and Congenital Syphilis.

Between 2012 and 2018, rates of congenital syphilis increased by 291% in the United States. In 2018, the rate of congenital syphilis was the highest it has been since 1995. Given these concerning epidemiologic trends, this review seeks to summarize the maternal-to-fetal transmission of syphilis to ensure adequate care of affected mothers and their infants. It also serves as a call to reinvest public health resources and reestablish infrastructure to ensure reversal of this concerning trend to stop preventable perinatal deaths, associated morbidities, and long-term consequences of congenital syphilis.

Nasal high-frequency jet ventilation (NHFJV) as a novel means of respiratory support in extremely low birth weight infants.

OBJECTIVE: Describe our experience of successfully using nasal high-frequency jet ventilation (NHFJV) in extremely low birth weight infants with respiratory failure. STUDY DESIGN: A retrospective review was conducted on 16 infants with birth weights <1000 g who received NHFJV from 2015 to 2019. Successful use was defined as avoiding intubation for at least 72 hours and demonstrating tolerance after being placed on NHFJV. RESULTS: Median gestational age was 24.5 weeks (IQR 24, 25), and weight at the start of NHFJV 1090 g (IQR 905, 1250). NHFJV was used successfully in 13/16 (81%) infants with a median duration of 7 days (IQR 3, 12). Days on invasive (30 vs. 186) and noninvasive (46 vs. 81) ventilation were shorter when compared to those who failed the use of NHFJV. CONCLUSION: This is the first reported case series for the successful use of NHFJV. Our study highlights the feasibility of a potential new mode of noninvasive respiratory support.

Congenital Cervical Spondyloptosis in the Neonate: A Prenatal Diagnosis.

Congenital spine abnormalities are rare in the fetus and neonate. The illustrative case described in this article is unique as it depicts a neonate with prenatally diagnosed cervical spondyloptosis. Vertebral instability at any level of the spine, regardless of its etiology, is dangerous as it has the potential for neurologic involvement-making an early diagnosis and treatment paramount. Proper stabilization in the delivery room, transfer to the neonatal intensive care unit, and establishment of a multidisciplinary treatment plan are the mainstays of therapy. Diagnosis is usually obtained through computed tomography and magnetic resonance imaging performed during the fetal or, more commonly, neonatal period. Successful management is often accomplished in consultation with different pediatric subspecialists, particularly orthopedists and neurosurgeons. The definitive therapy is surgical intervention. Prognosis of this condition is dependent upon the severity of the malformation, time to stabilization, successful orthopedic and neurosurgical intervention, and proper adherence to follow-up. [Pediatr Ann. 2020;49(7):e313-e318.].

Extremely Preterm Infant Born to a Mother With Severe COVID-19 Pneumonia.

Little is known about the effects of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) on pregnant women, fetuses, and neonates, especially when the virus is contracted early in pregnancy. The literature is especially lacking on the effects of SARS-CoV-2 on extremely preterm (<28 weeks gestation) infants who have underdeveloped immune systems. We report the case of an extremely preterm, 25-week 5-days old infant, born to a mother with severe COVID-19 (coronavirus disease-2019) pneumonia. In this case, there is no evidence of vertical transmission of SARS-CoV-2 based on reverse transcription-polymerase chain reaction testing, despite extreme prematurity. However, it appears that severe maternal COVID-19 may have been associated with extremely preterm delivery, based on observed histologic chorioamnionitis. This is the first reported case of an extremely preterm infant born to a mother with severe COVID-19 pneumonia who required intubation, and was treated with hydroxychloroquine, azithromycin, remdesivir, tocilizumab, convalescent plasma, inhaled nitric oxide, and prone positioning for severe hypoxemic respiratory failure prior to and after delivery of this infant. The infant remains critically ill with severe respiratory failure on high-frequency ventilation, inotropic support, hydrocortisone for pressor-resistant hypotension, and inhaled nitric oxide for severe persistent pulmonary hypertension with a right to left shunt across the patent ductus arteriosus and foramen ovale. Pregnant women or women planning to get pregnant should take all precautions to minimize exposure to SARS-CoV-2 to decrease adverse perinatal outcomes.

Newborn Sleep: Patterns, Interventions, and Outcomes.

Sleep is a necessary function of life. Fetuses and neonates spend most of their day sleeping, making it paramount to place emphasis on adequate and optimal sleep. As the current body of literature continues to expand, we have increased our understanding of sleep and its role in development. Sleep disturbances, particularly early in life can affect all aspects of health such as neurological development, emotional well-being, and overall growth. This article aims to provide a primer on sleep development from fetal life into the neonatal period, discuss sleep in both the home and hospital settings, explore the tools used to measure sleep, and review common interventions applied to those infants experiencing poor sleep. Lastly, there is a mention of long-term outcomes and how early recognition and implementation of measures could help to improve overall growth and development throughout childhood. [Pediatr Ann. 2020;49(2):e82-e87.].

Value Narratives: A Novel Method for Understanding High-Cost Pediatric Hospital Patients.

OBJECTIVES: To delineate the drivers of cost associated with the most-costly inpatients in a tertiary pediatric hospital. METHODS: We identified the 10% most-costly inpatients treated at a large regional children's hospital in 2010. From this group we randomly selected, within representative specialties, 2 groups of 50 inpatients for detailed chart review. By using daily cost data and clinical records, 2 independent reviewers examined the clinical course of each patient to identify events that drove cost beyond that expected for standard of care. By using an iterative process, these events were grouped into themes or "cost drivers." Linear regression was used to measure the association of number of cost drivers and total 2010 inpatient cost. RESULTS: We identified 7 cost drivers: medical complications (49%), futile treatment (6%), failure to identify family care preferences (9%), system errors (65%), preventable admissions (21%), complex family dynamics (11%), and expensive diagnosis with no other cost driver (15%). Cost drivers were associated with increased total costs. CONCLUSIONS: We developed a novel method for understanding high-cost inpatients. This method allowed a more detailed understanding of cost drivers than could be achieved with administrative data alone. Many of these cost drivers were related to problems with communication.

The Perinatal Origins of Cardiovascular Disease.

In recent decades, with advances in neonatal intensive care, extremely premature infants are now surviving into adulthood. Epidemiologic data on the health of these ex-premature infants have begun to reveal a concerning motif-that is, prematurity, in and of itself, seems to be a risk factor for cardiovascular and metabolic disease in later adulthood. The mechanisms underlying this increased risk are unclear, but it is believed that both adverse fetal environment and postnatal exposures for a premature infant likely contribute to the developmental programming of disease by altering the normal trajectory of maturation and aging of multiple organ systems. This article specifically focuses on perinatal factors that may affect risk for cardiovascular disease.

Vein of Galen Arteriovenous Malformation in a Neonate.

The vein of Galen is the most common type of arteriovenous malformation in the fetus and neonate. Most vein of Galen arteriovenous malformations (VGAMs) are diagnosed in the neonatal period, with the remainder being identified in early childhood, typically via computed tomography scan. The VGAM is found in five different patterns where the vein of Galen and straight sinus extending to the torcula Herophili are dilated. This dilation can lead to significant compression of the posterior fossa structures. Clinically, the infant with this malformation can present with seizures or most commonly, high output cardiac failure. It is important, however, to keep a broad differential diagnosis as more prevalent neonatal conditions arise similarly. These conditions can include developmental delay, cerebral palsy, epilepsy, superior vena cava syndrome, hemangioendothelioma, and other arteriovenous fistulae. Treatment begins with early diagnosis and testing of initial sequelae. This is often accomplished in consultation with different pediatric subspecialists, particularly neurologists and cardiologists. The mainstay of therapy is with neurosurgical intervention. Although the mortality of a fetus or neonate with VGAM is very high, prognosis is dependent on the size of the malformation, age at diagnosis, and successful neurosurgical outcome.