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Glucose-6-phosphate dehydrogenase (G6PD) deficiency, the most common enzymopathy worldwide, is an insufficient amount of the G6PD enzyme, which is vital to the protection of the erythrocyte. Deficient enzyme levels lead to oxidative damage, hemolysis, and resultant severe hyperbilirubinemia. If not promptly recognized and treated, G6PD deficiency can potentially lead to bilirubin-induced neurologic dysfunction, acute bilirubin encephalopathy, and kernicterus. Glucose-6-phosphate dehydrogenase deficiency is one of the three most common causes for pathologic hyperbilirubinemia. A change in migration patterns and intercultural marriages have created an increased incidence of G6PD deficiency in the United States. Currently, there is no universally mandated metabolic screening or clinical risk assessment tool for G6PD deficiency in the United States. Mandatory universal screening for G6PD deficiency, which includes surveillance and hospital-based risk assessment tools, can identify the at-risk infant and foster early identification, diagnosis, and treatment to eliminate neurotoxicity.
Chylothorax, a lymphatic flow disorder characterized by an abnormal circulation of lymph fluid into the pleural cavity, is the most common cause of pleural effusions during the neonatal period. This condition affects 1/15,000 neonates every year. Affected neonates often manifest with respiratory distress, electrolyte imbalances, sepsis, and even immunodeficiencies. Mortality risk is highest among neonates undergoing cardiac surgery as well as those with associated hydrops fetalis. Conservative treatment options include bowel rest with administration of parenteral nutrition, followed with medium-chain triglyceride enteral feedings, and octreotide therapy. Severe or persistent cases require surgical intervention. This can involve a unilateral or bilateral pleurectomy and thoracic duct ligation, with or without pleurodesis. Early identification and successful treatment of this condition is contingent upon awareness of the most current evidence and a timely cross-disciplinary approach to care.
- Go to article: Congenital Diaphragmatic Hernia: Interprofessional Care of the Neonate and Family Grounded in Swanson’s Theory of Caring
Congenital Diaphragmatic Hernia: Interprofessional Care of the Neonate and Family Grounded in Swanson’s Theory of Caring
Congenital diaphragmatic hernia (CDH), a major congenital defect, occurs in 3.3–5 per 10,000 live births. It is associated with significant morbidity and mortality risks, with lifelong implications imposed on survivors. The pathophysiology, diagnostic tools, and treatment modalities for CDH must be understood to provide timely, holistic care to the infant and family. Swanson’s theory of caring can effectively guide the interprofessional perinatal/neonatal team in the prenatal and postnatal care of the infant and family.
- Go to article: Use of Placental/Umbilical Blood Sampling for Neonatal Admission Blood Cultures: Benefits, Challenges, and Strategies for Implementation
Use of Placental/Umbilical Blood Sampling for Neonatal Admission Blood Cultures: Benefits, Challenges, and Strategies for Implementation
Placental blood remains an underused resource for early neonatal care despite ample evidence that placental blood provides the same clinical decision making information without the need for painful, invasive blood sampling procedures. Potential benefits of placental/umbilical blood sampling (PUBS) for neonatal admission labs include decreases in pain reactivity, rates of anemia, need for blood transfusions, use of vasopressors, and rates of intraventricular hemorrhage. Here, we present a unique case study of a critically ill infant with contradictory blood culture results from PUBS and direct infant sampling. A negative admission direct sample blood culture result compared with a positive admission PUBS blood culture result suggests that infection may have been missed in the direct infant sample. Relevant placental embryology and circulation is also described, as well as the benefits of PUBS for neonatal admission labs (with focus on the blood culture), challenges associated with PUBS practice, and strategies for implementation of PUBS.
Down syndrome (DS) is a well-known genetic disorder that affects 700–1,000 infants per year. One particular comorbidity of DS is transient myeloproliferative disorder (TMD), a disease characterized by leukocytosis with elevated blast counts. Approximately 10 percent of DS infants develop TMD, which usually manifests during the first week of life and can lead to an extended hospitalization in a NICU. In addition to hallmark hematologic findings, other manifestations include jaundice, conjugated hyperbilirubinemia, hepatomegaly, and pericardial or pleural effusions. TMD generally resolves spontaneously in the first three months of life with the provision of timely medical management; however, survivors are at increased risk of developing acute myeloid leukemia (AML). Neonatal nurses need to have knowledge of this disorder to facilitate screening of DS infants and optimize family education and coordination of care.
Relative adrenal insufficiency (AI) is a disease process commonly associated with preterm birth and critical illness. Further, the incidence of AI is inversely proportional to gestational age. The incidence of AI is likely underreported; however, it is reported to occur in 150–280/1,000,000 live births worldwide. Functional development of the adrenal gland does not occur until after 30 weeks of gestation; however, advances made in neonatal care increase the survivability of infants born well before this period. Among infants with AI, the adrenal gland is transiently incapable of secreting physiologic levels of cortisol in response to stressors. Common and nonspecific signs include hypotension, poor perfusion, and dysregulation of fluid, electrolytes, and euglycemia. Recognition, diagnosis, and steroid therapy is critical, as inappropriately managed AI can lead to an adrenal crisis, shock, and death. Understanding the presentation and common risk factors for developing relative AI is crucial for quick diagnosis and timely management to prevent morbidity and mortality in this vulnerable population.
Pulmonary hemorrhage (PH) is a pathology associated with significant morbidity and mortality, particularly among preterm infants in the NICU. The diagnosis is made when hemorrhagic secretions are aspirated from the trachea concurrent with respiratory decompensation that necessitates intubation or escalated support. The implementation of mechanical ventilation and widespread exogenous surfactant administration have significantly reduced respiratory morbidities. However, when PH develops, death remains the most common outcome. Treatment for PH remains primarily supportive; thus, a thorough understanding of underlying disease processes, manifestations, diagnostic testing, and current evidence is vital to enable early identification and proactive management to reduce morbidity and mortality.
Congenital diaphragmatic hernia (CDH) is a developmental defect in the diaphragm that allows abdominal viscera to herniate into the thoracic cavity. Pulmonary hypoplasia and pulmonary hypertension are consequences of this disease process. The incidence is approximately 2.4–4.1/10,000 births, and survival rate is estimated at 70–90 percent. To avoid potentially devastating delays in care, it is crucial that neonatal nurses and care providers in both tertiary and nontertiary care centers be familiar with the pathogenesis of CDH and the standard of care for initial stabilization of the neonate. Novel fetal and postnatal surgical repair techniques are also described here.
Neuroblastoma represents approximately 6 to 10 percent of childhood cancers, yet is one of the most common solid tumors observed in neonates; approximately 700 cases are reported in the United States each year. Neuroblastoma occurs secondary to oncogene mutations that cause abnormal proliferation of neural crest cells and tumor formation anywhere along the spinal cord. Visible manifestations include a blueberry rash and subcutaneous skin nodules. Common histologic findings include multifocal, small, round, blue cell tumors. Cytogenetics testing differentiates aggressive versus nonaggressive forms of neuroblastoma. Treatment ranges from supportive care to surgery and chemotherapy; targeted molecular therapies and immunotherapy offer opportunity to individualize treatment. Morbidity and mortality are contingent upon age at diagnosis and genetic abnormalities. Neonatal clinicians must establish and maintain active knowledge of the current science pertaining to this neoplasm to assist in early identification and timely initiation of medical management. This article presents a case report and comprehensive discussion of the state of the science on metastatic familial (congenital) neuroblastoma.