Pub Date : 2025-03-01DOI: 10.1016/j.siny.2025.101619
Jon F. Watchko , Vinod K. Bhutani
The WHO recently revised their classification schema for G6PD gene variants. Notably, the previously separate Class II (severe enzyme deficiency; <10 % normal) and Class III (moderate enzyme deficiency; 10–60 % normal) variant groups are now combined into a single new category designated as Class B. Class B variants exhibit G6PD enzymatic activity in the <45 % of normal range. This welcome and prudent reclassification far better aligns with the neonatal hyperbilirubinemia risk reported in neonates with i) former “less severe” Class III variants including G6PD A- and ii) female neonates heterozygous for deficient alleles.
世界卫生组织最近修订了 G6PD 基因变异的分类模式。值得注意的是，以前单独划分的 II 类（严重酶缺乏症，即 G6PD 基因变异）现在被划分为 G6PD 基因变异；
{"title":"Revised World Health Organization (WHO) classification of G6PD gene variants: Relevance to neonatal hyperbilirubinemia","authors":"Jon F. Watchko , Vinod K. Bhutani","doi":"10.1016/j.siny.2025.101619","DOIUrl":"10.1016/j.siny.2025.101619","url":null,"abstract":"<div><div>The WHO recently revised their classification schema for G6PD gene variants. Notably, the previously separate Class II (severe enzyme deficiency; <10 % normal) and Class III (moderate enzyme deficiency; 10–60 % normal) variant groups are now combined into a single new category designated as Class B. Class B variants exhibit G6PD enzymatic activity in the <45 % of normal range. This welcome and prudent reclassification far better aligns with the neonatal hyperbilirubinemia risk reported in neonates with i) former “less severe” Class III variants including <em>G6PD A-</em> and ii) female neonates heterozygous for deficient alleles.</div></div>","PeriodicalId":49547,"journal":{"name":"Seminars in Fetal & Neonatal Medicine","volume":"30 1","pages":"Article 101619"},"PeriodicalIF":2.9,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143537884","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01DOI: 10.1016/j.siny.2024.101547
Robert D. Christensen , Timothy M. Bahr , Robin K. Ohls , Kenneth J. Moise Jr.
Hemolysis is a pathological shortening of the red blood cell lifespan. When hemolysis occurs in a neonate, hazardous hyperbilirubinemia and severe anemia could result. Hemolysis can be diagnosed, and its severity quantified, by the non-invasive measurement of carbon monoxide (CO) in exhaled breath. The point-of-care measurement is called “End-tidal CO corrected for ambient CO” (ETCOc). Herein we explain how ETCOc measurements can be used to diagnose and manage various perinatal/neonatal hemolytic disorders. We provide information regarding five clinical situations; 1) facilitating a precise diagnosis among neonates presenting with anemia or jaundice of unknown etiology, 2) monitoring fetal hemolysis with serial measurements of mothers during pregnancy, 3) measuring the duration of hemolysis in neonates with hemolytic disease, 4) measuring neonates who require phototherapy, to determine whether they have hemolytic vs. non-hemolytic jaundice, and 5) measuring all neonates in the birth hospital as part of a jaundice-detection and management program.
溶血是红细胞寿命缩短的一种病理现象。新生儿发生溶血时，可能会导致危险的高胆红素血症和严重贫血。溶血可通过无创测量呼出气体中的一氧化碳 (CO) 来诊断，并量化其严重程度。这种护理点测量方法被称为 "根据环境 CO 校正的潮气末 CO"（ETCOc）。在此，我们将解释如何利用 ETCOc 测量来诊断和处理各种围产期/新生儿溶血性疾病。我们提供了有关五种临床情况的信息：1）便于对病因不明的贫血或黄疸新生儿进行精确诊断；2）通过对孕期母亲进行连续测量来监测胎儿溶血情况；3）测量患有溶血性疾病的新生儿的溶血持续时间；4）测量需要光疗的新生儿，以确定他们是否患有溶血性黄疸或非溶血性黄疸；5）测量出生医院的所有新生儿，作为黄疸检测和管理计划的一部分。
{"title":"Neonatal/perinatal diagnosis of hemolysis using ETCOc","authors":"Robert D. Christensen , Timothy M. Bahr , Robin K. Ohls , Kenneth J. Moise Jr.","doi":"10.1016/j.siny.2024.101547","DOIUrl":"10.1016/j.siny.2024.101547","url":null,"abstract":"<div><div>Hemolysis is a pathological shortening of the red blood cell lifespan. When hemolysis occurs in a neonate, hazardous hyperbilirubinemia and severe anemia could result. Hemolysis can be diagnosed, and its severity quantified, by the non-invasive measurement of carbon monoxide (CO) in exhaled breath. The point-of-care measurement is called “End-tidal CO corrected for ambient CO” (ETCOc). Herein we explain how ETCOc measurements can be used to diagnose and manage various perinatal/neonatal hemolytic disorders. We provide information regarding five clinical situations; 1) facilitating a precise diagnosis among <em>neonates</em> presenting with anemia or jaundice of unknown etiology, 2) monitoring <em>fetal</em> hemolysis with serial measurements of mothers during pregnancy, 3) measuring the <em>duration</em> of hemolysis in neonates with hemolytic disease, 4) measuring neonates who require <em>phototherapy,</em> to determine whether they have hemolytic <em>vs.</em> non-hemolytic jaundice, and 5) measuring <em>all neonates</em> in the birth hospital as part of a jaundice-detection and management program.</div></div>","PeriodicalId":49547,"journal":{"name":"Seminars in Fetal & Neonatal Medicine","volume":"30 1","pages":"Article 101547"},"PeriodicalIF":2.9,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142511654","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01DOI: 10.1016/j.siny.2025.101612
Sandra Juul , Kendell German
Infants born preterm or with other perinatal risk factors are at added risk for both iron deficiency and overload. Insufficient iron supplementation in the perinatal period is associated with long-term neurodevelopmental effects. Based on this, iron supplements must be targeted to infants’ individual iron needs to avoid the adverse effects of both iron deficiency and overload. Enteral iron supplements have been the gold standard in iron supplementation of neonates for many years. However, emerging parenteral formulations may provide an alternative for some infants, such as those who are unable to tolerate oral supplements or who are refractory to enteral supplementation. Optimal dosing and timing of supplementation is an area of ongoing research. In this review, we will summarize available enteral and parenteral iron formulations, review iron measurement parameters, and identify outstanding questions and ongoing research.
{"title":"Iron supplementation for infants in the NICU: What preparation, how much, and how long is optimal?","authors":"Sandra Juul , Kendell German","doi":"10.1016/j.siny.2025.101612","DOIUrl":"10.1016/j.siny.2025.101612","url":null,"abstract":"<div><div>Infants born preterm or with other perinatal risk factors are at added risk for both iron deficiency and overload. Insufficient iron supplementation in the perinatal period is associated with long-term neurodevelopmental effects. Based on this, iron supplements must be targeted to infants’ individual iron needs to avoid the adverse effects of both iron deficiency and overload. Enteral iron supplements have been the gold standard in iron supplementation of neonates for many years. However, emerging parenteral formulations may provide an alternative for some infants, such as those who are unable to tolerate oral supplements or who are refractory to enteral supplementation. Optimal dosing and timing of supplementation is an area of ongoing research. In this review, we will summarize available enteral and parenteral iron formulations, review iron measurement parameters, and identify outstanding questions and ongoing research.</div></div>","PeriodicalId":49547,"journal":{"name":"Seminars in Fetal & Neonatal Medicine","volume":"30 1","pages":"Article 101612"},"PeriodicalIF":2.9,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143524952","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
In neonatal patients, bleeding is a multifactorial event in which several factors may play a pathogenic role. Among these, thrombocytopenia is often considered a risk factor for bleeding, although a causal relationship has never been demonstrated. In fact, major bleeding mainly occurs in non-thrombocytopenic newborns and thrombocytopenic newborns rarely experience major bleeding. Therefore, parameters other than platelet count might better assess the hemostatic function and define bleeding risk. Historically, neonatologists aimed to reduce the risk of bleeding by administering platelet transfusions. However, recent studies demonstrated that transfusing newborns at higher threshold is associated with an increased risk of death, bleeding, bronchopulmonary dysplasia and neurodevelopmental impairment. The mechanism behind this association is not known and various hypotheses have been proposed, including the non-hemostatic effects of adult-derived platelets transfused into neonates. Alternatively, the rapid volume expansion caused by a platelet transfusion might cause hemodynamic instability and cardiocirculatory overload. Guidelines about platelet transfusions should now include this recent evidence and adopt more stringent thresholds. Future research should focus on finding alternative or improved transfusion products more suitable for newborns.
{"title":"Platelet transfusion and bleeding risk","authors":"Valeria Cortesi , Enrico Lopriore , Susanna Fustolo-Gunnink","doi":"10.1016/j.siny.2025.101608","DOIUrl":"10.1016/j.siny.2025.101608","url":null,"abstract":"<div><div>In neonatal patients, bleeding is a multifactorial event in which several factors may play a pathogenic role. Among these, thrombocytopenia is often considered a risk factor for bleeding, although a causal relationship has never been demonstrated. In fact, major bleeding mainly occurs in non-thrombocytopenic newborns and thrombocytopenic newborns rarely experience major bleeding. Therefore, parameters other than platelet count might better assess the hemostatic function and define bleeding risk. Historically, neonatologists aimed to reduce the risk of bleeding by administering platelet transfusions. However, recent studies demonstrated that transfusing newborns at higher threshold is associated with an increased risk of death, bleeding, bronchopulmonary dysplasia and neurodevelopmental impairment. The mechanism behind this association is not known and various hypotheses have been proposed, including the non-hemostatic effects of adult-derived platelets transfused into neonates. Alternatively, the rapid volume expansion caused by a platelet transfusion might cause hemodynamic instability and cardiocirculatory overload. Guidelines about platelet transfusions should now include this recent evidence and adopt more stringent thresholds. Future research should focus on finding alternative or improved transfusion products more suitable for newborns.</div></div>","PeriodicalId":49547,"journal":{"name":"Seminars in Fetal & Neonatal Medicine","volume":"30 1","pages":"Article 101608"},"PeriodicalIF":2.9,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143634803","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01DOI: 10.1016/j.siny.2024.101546
Timothy M. Bahr , Thomas R. Christensen , Sarah J. Ilstrup , Robin K. Ohls , Robert D. Christensen
ELGANs (Extremely-Low-Gestational-Age Neonates; those born before 28 weeks gestation) are at risk for developing significant morbidities including retinopathy of prematurity (ROP), bronchopulmonary dysplasia (BPD), and cognitive impairment. The pathogenesis of each of these morbidities is complex, but a growing literature suggests that repeated transfusions of adult donor red blood cells (RBC) conveys a propensity to develop these disorders. The biological rationale for the propensities might vary with each morbidity. For instance, hemoglobin A in adult red cells increases oxygen delivery to the developing retina, potentiating ROP, while a proinflammatory nature of adult donor RBC might potentiate BPD. It is possible that fetal RBC harvested from otherwise discarded umbilical cord blood after healthy term births would be a more physiologically appropriate transfusion product for anemic ELGANs. Such a product might result in a lower incidence or severity of the common morbidities. Herein we review our progress, and that of others, toward testing that theory.
ELGANs（极低妊娠年龄新生儿；妊娠 28 周前出生的新生儿）有罹患严重疾病的风险，包括早产儿视网膜病变（ROP）、支气管肺发育不良（BPD）和认知障碍。这些疾病的发病机制都很复杂，但越来越多的文献表明，反复输注成人供体红细胞（RBC）会导致这些疾病的发生。每种疾病的发病倾向的生物学原理可能各不相同。例如，成人红细胞中的血红蛋白 A 可增加向发育中视网膜的氧输送，从而诱发早产儿视网膜病变，而成人供体红细胞的促炎症性质可能会诱发早产儿脑瘫。对于贫血的 ELGANs 而言，从健康足月儿出生后被丢弃的脐带血中采集的胎儿红细胞可能是一种在生理上更合适的输血产品。这种产品可能会降低常见疾病的发病率或严重程度。在此，我们回顾了我们和其他人在验证这一理论方面取得的进展。
{"title":"Term umbilical cord blood, fully tested and processed, as the source of red blood cell transfusions for extremely-low-gestational age neonates","authors":"Timothy M. Bahr , Thomas R. Christensen , Sarah J. Ilstrup , Robin K. Ohls , Robert D. Christensen","doi":"10.1016/j.siny.2024.101546","DOIUrl":"10.1016/j.siny.2024.101546","url":null,"abstract":"<div><div>ELGANs (Extremely-Low-Gestational-Age Neonates; those born before 28 weeks gestation) are at risk for developing significant morbidities including retinopathy of prematurity (ROP), bronchopulmonary dysplasia (BPD), and cognitive impairment. The pathogenesis of each of these morbidities is complex, but a growing literature suggests that repeated transfusions of adult donor red blood cells (RBC) conveys a propensity to develop these disorders. The biological rationale for the propensities might vary with each morbidity. For instance, hemoglobin A in adult red cells increases oxygen delivery to the developing retina, potentiating ROP, while a proinflammatory nature of adult donor RBC might potentiate BPD. It is possible that fetal RBC harvested from otherwise discarded umbilical cord blood after healthy term births would be a more physiologically appropriate transfusion product for anemic ELGANs. Such a product might result in a lower incidence or severity of the common morbidities. Herein we review our progress, and that of others, toward testing that theory.</div></div>","PeriodicalId":49547,"journal":{"name":"Seminars in Fetal & Neonatal Medicine","volume":"30 1","pages":"Article 101546"},"PeriodicalIF":2.9,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142548567","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01DOI: 10.1016/j.siny.2025.101615
Akhil Maheshwari
Necrotizing enterocolitis (NEC) is a catastrophic inflammatory bowel necrosis of premature infants. The etiology is unknown, but 25–40 % of cases have a history of red blood cell (RBC) transfusions in the preceding 48 h. This association has been noted in retrospective case/case-control studies, and many meta-analyses, and in a murine model. However, we still need human studies with larger, adequately powered cohorts to confirm this association and determine the operant mechanisms. The murine model shows that severe anemia leads to macrophage infiltration in the gut mucosa. Subsequent RBC transfusions containing free hemoglobin, activate nuclear factor-kappa B-mediated inflammatory changes and cause NEC-like mucosal injury. This review summarizes current human and experimental data to evaluate ta-NEC and hitherto unanswered mechanistic questions. If a causal relationship between transfusions and NEC is proven, these data could help develop effective therapeutic strategies.
{"title":"Severe anemia predisposes very premature infants to transfusion-associated necrotizing enterocolitis","authors":"Akhil Maheshwari","doi":"10.1016/j.siny.2025.101615","DOIUrl":"10.1016/j.siny.2025.101615","url":null,"abstract":"<div><div>Necrotizing enterocolitis (NEC) is a catastrophic inflammatory bowel necrosis of premature infants. The etiology is unknown, but 25–40 % of cases have a history of red blood cell (RBC) transfusions in the preceding 48 h. This association has been noted in retrospective case/case-control studies, and many meta-analyses, and in a murine model. However, we still need human studies with larger, adequately powered cohorts to confirm this association and determine the operant mechanisms. The murine model shows that severe anemia leads to macrophage infiltration in the gut mucosa. Subsequent RBC transfusions containing free hemoglobin, activate nuclear factor-kappa B-mediated inflammatory changes and cause NEC-like mucosal injury. This review summarizes current human and experimental data to evaluate ta-NEC and hitherto unanswered mechanistic questions. If a causal relationship between transfusions and NEC is proven, these data could help develop effective therapeutic strategies.</div></div>","PeriodicalId":49547,"journal":{"name":"Seminars in Fetal & Neonatal Medicine","volume":"30 1","pages":"Article 101615"},"PeriodicalIF":2.9,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143587868","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-12-01DOI: 10.1016/j.siny.2024.101563
Alexander J. Rickart , Theodore Dassios , Anne Greenough
Survival rates for extremely low birth weight (ELBW) infants have improved over the recent years, yet morbidity remains high. This review explores respiratory management strategies for this unique cohort and how it may impact their long-term outcomes. Although there is a preference towards non-invasive respiratory support in less immature infants, ELBW infants often require invasive ventilation. This comes with an increased risk of bronchopulmonary dysplasia, adverse neurodevelopmental outcomes and lifelong respiratory impairment. There are a range of options available to reduce volutrauma and minimise lung injury, including volume targeted ventilation and high-frequency ventilation. In the absence of high-quality evidence focussing on ELBW infants, much of current practice is inferred from studies involving infants with a broader range of gestational ages and experiences at high-volume centres. This highlights the need for further research targeted to this specific population with a focus on long-term respiratory health.
{"title":"Optimal respiratory support for extremely low birth weight infants – do we have the answers?","authors":"Alexander J. Rickart , Theodore Dassios , Anne Greenough","doi":"10.1016/j.siny.2024.101563","DOIUrl":"10.1016/j.siny.2024.101563","url":null,"abstract":"<div><div>Survival rates for extremely low birth weight (ELBW) infants have improved over the recent years, yet morbidity remains high. This review explores respiratory management strategies for this unique cohort and how it may impact their long-term outcomes. Although there is a preference towards non-invasive respiratory support in less immature infants, ELBW infants often require invasive ventilation. This comes with an increased risk of bronchopulmonary dysplasia, adverse neurodevelopmental outcomes and lifelong respiratory impairment. There are a range of options available to reduce volutrauma and minimise lung injury, including volume targeted ventilation and high-frequency ventilation. In the absence of high-quality evidence focussing on ELBW infants, much of current practice is inferred from studies involving infants with a broader range of gestational ages and experiences at high-volume centres. This highlights the need for further research targeted to this specific population with a focus on long-term respiratory health.</div></div>","PeriodicalId":49547,"journal":{"name":"Seminars in Fetal & Neonatal Medicine","volume":"29 6","pages":"Article 101563"},"PeriodicalIF":2.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142631265","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-12-01DOI: 10.1016/j.siny.2024.101567
Olivia Ruth , Nasuh Malas
Delirium is a common and serious complication of critical illness that has been increasingly recognized in pediatric patients. There have been several published cases of delirium in newborns and infants over the last decade, though research on neonatal delirium is severely lacking. The true prevalence of delirium and its associated consequences in this population remain unknown, although the risk of delirium in this population appears to be elevated compared to older youth. The current approach to management of delirium in neonates is extrapolated from older children. In the present review, the pathophysiology and clinical presentation of delirium are outlined. Strategies for prevention, evaluation, and management of delirium in neonates are explored.
{"title":"Neonatal delirium","authors":"Olivia Ruth , Nasuh Malas","doi":"10.1016/j.siny.2024.101567","DOIUrl":"10.1016/j.siny.2024.101567","url":null,"abstract":"<div><div>Delirium is a common and serious complication of critical illness that has been increasingly recognized in pediatric patients. There have been several published cases of delirium in newborns and infants over the last decade, though research on neonatal delirium is severely lacking. The true prevalence of delirium and its associated consequences in this population remain unknown, although the risk of delirium in this population appears to be elevated compared to older youth. The current approach to management of delirium in neonates is extrapolated from older children. In the present review, the pathophysiology and clinical presentation of delirium are outlined. Strategies for prevention, evaluation, and management of delirium in neonates are explored.</div></div>","PeriodicalId":49547,"journal":{"name":"Seminars in Fetal & Neonatal Medicine","volume":"29 6","pages":"Article 101567"},"PeriodicalIF":2.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142631261","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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