Impact of inborn errors of metabolism on admission in a neonatal intensive care unit: a 4-year report

Author(s):  
Meng Meng ◽  
Yu-Ping Zhang
2021 ◽  
Vol 12 ◽  
Author(s):  
Scott M. Gordon ◽  
Amy E. O’Connell

Due to heightened awareness and advanced genetic tools, inborn errors of immunity (IEI) are increasingly recognized in children. However, diagnosing of IEI in premature infants is challenging and, subsequently, reports of IEI in premature infants remain rare. This review focuses on how common disorders of prematurity, such as sepsis, necrotizing enterocolitis, and bronchopulmonary dysplasia, can clinically overlap with presenting signs of IEI. We present four recent cases from a single neonatal intensive care unit that highlight diagnostic dilemmas facing neonatologists and clinical immunologists when considering IEI in preterm infants. Finally, we present a conceptual framework for when to consider IEI in premature infants and a guide to initial workup of premature infants suspected of having IEI.


2019 ◽  
Vol 14 (2) ◽  
pp. 184-195 ◽  
Author(s):  
Jennifer R. Charlton ◽  
Louis Boohaker ◽  
David Askenazi ◽  
Patrick D. Brophy ◽  
Carl D’Angio ◽  
...  

Background and objectivesNeonatal AKI is associated with poor short- and long-term outcomes. The objective of this study was to describe the risk factors and outcomes of neonatal AKI in the first postnatal week.Design, setting, participants, & measurementsThe international retrospective observational cohort study, Assessment of Worldwide AKI Epidemiology in Neonates (AWAKEN), included neonates admitted to a neonatal intensive care unit who received at least 48 hours of intravenous fluids. Early AKI was defined by an increase in serum creatinine >0.3 mg/dl or urine output <1 ml/kg per hour on postnatal days 2–7, the neonatal modification of Kidney Disease: Improving Global Outcomes criteria. We assessed risk factors for AKI and associations of AKI with death and duration of hospitalization.ResultsTwenty-one percent (449 of 2110) experienced early AKI. Early AKI was associated with higher risk of death (adjusted odds ratio, 2.8; 95% confidence interval, 1.7 to 4.7) and longer duration of hospitalization (parameter estimate: 7.3 days 95% confidence interval, 4.7 to 10.0), adjusting for neonatal and maternal factors along with medication exposures. Factors associated with a higher risk of AKI included: outborn delivery; resuscitation with epinephrine; admission diagnosis of hyperbilirubinemia, inborn errors of metabolism, or surgical need; frequent kidney function surveillance; and admission to a children’s hospital. Those factors that were associated with a lower risk included multiple gestations, cesarean section, and exposures to antimicrobials, methylxanthines, diuretics, and vasopressors. Risk factors varied by gestational age strata.ConclusionsAKI in the first postnatal week is common and associated with death and longer duration of hospitalization. The AWAKEN study demonstrates a number of specific risk factors that should serve as “red flags” for clinicians at the initiation of the neonatal intensive care unit course.


2019 ◽  
Vol 4 (6) ◽  
pp. 1507-1515
Author(s):  
Lauren L. Madhoun ◽  
Robert Dempster

Purpose Feeding challenges are common for infants in the neonatal intensive care unit (NICU). While sufficient oral feeding is typically a goal during NICU admission, this can be a long and complicated process for both the infant and the family. Many of the stressors related to feeding persist long after hospital discharge, which results in the parents taking the primary role of navigating the infant's course to ensure continued feeding success. This is in addition to dealing with the psychological impact of having a child requiring increased medical attention and the need to continue to fulfill the demands at home. In this clinical focus article, we examine 3 main areas that impact psychosocial stress among parents with infants in the NICU and following discharge: parenting, feeding, and supports. Implications for speech-language pathologists working with these infants and their families are discussed. A case example is also included to describe the treatment course of an infant and her parents in the NICU and after graduation to demonstrate these points further. Conclusion Speech-language pathologists working with infants in the NICU and following hospital discharge must realize the family context and psychosocial considerations that impact feeding progression. Understanding these factors may improve parental engagement to more effectively tailor treatment approaches to meet the needs of the child and family.


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