Spectrum of Disease in Hospitalized Newborns with Congenital Micrognathia: A Cohort of 3,236 Infants at North American Tertiary-Care Intensive Care Units.

OBJECTIVE: To describe the spectrum of disease and burden of care in infants with congenital micrognathia from a multicenter cohort hospitalized at tertiary care centers. STUDY DESIGN: The Children's Hospitals Neonatal Database was queried from 2010 through 2020 for infants diagnosed with micrognathia. Demographics, presence of genetic syndromes, and cleft status were summarized. Outcomes included death, length of hospitalization, neonatal surgery, and feeding and respiratory support at discharge. RESULTS: Analysis included 3,236 infants with congenital micrognathia. Cleft palate was identified in 1266 (39.1%). A genetic syndrome associated with micrognathia was diagnosed during the neonatal hospitalization in 256 (7.9%). Median (IQR) length of hospitalization was 35 (16, 63) days. Death during the hospitalization (n = 228, 6.8%) was associated with absence of cleft palate (4.4%, P < .001) and maternal Black race (11.6%, P < .001). During the neonatal hospitalization, 1289 (39.7%) underwent surgery to correct airway obstruction and 1059 (32.7%) underwent gastrostomy tube placement. At the time of discharge, 1035 (40.3%) were exclusively feeding orally. There was significant variability between centers related to length of stay and presence of a feeding tube at discharge (P < .001 for both). CONCLUSIONS: Infants hospitalized with congenital micrognathia have a significant burden of disease, commonly receive surgical intervention, and most often require tube feedings at hospital discharge. We identified disparities based on race and among centers. Development of evidence-based guidelines could improve neonatal care.

Clinical identification of feeding and swallowing disorders in 0-6 month old infants with Down syndrome.

Feeding and swallowing disorders have been described in children with a variety of neurodevelopmental disabilities, including Down syndrome (DS). Abnormal feeding and swallowing can be associated with serious sequelae such as failure to thrive and respiratory complications, including aspiration pneumonia. Incidence of dysphagia in young infants with DS has not previously been reported. To assess the identification and incidence of feeding and swallowing problems in young infants with DS, a retrospective chart review of 174 infants, ages 0-6 months was conducted at a single specialty clinic. Fifty-seven percent (100/174) of infants had clinical concerns for feeding and swallowing disorders that warranted referral for Videofluroscopic Swallow Study (VFSS); 96/174 (55%) had some degree of oral and/or pharyngeal phase dysphagia and 69/174 (39%) had dysphagia severe enough to warrant recommendation for alteration of breast milk/formula consistency or nonoral feeds. Infants with certain comorbidities had significant risk for significant dysphagia, including those with functional airway/respiratory abnormalities (OR = 7.2). Infants with desaturation with feeds were at dramatically increased risk (OR = 15.8). All young infants with DS should be screened clinically for feeding and swallowing concerns. If concerns are identified, consideration should be given to further evaluation with VFSS for identification of dysphagia and additional feeding modifications.

An evidence-based pilot study exploring relationships between psychologic and physiologic factors in post-lung-transplant adolescents with cystic fibrosis.

After using an evidence-based approach to address clinical practice questions, a pilot study was designed to explore the relationships among anxiety and depression self-report data, cyclosporine (CSA) trough values, and biopsy rejection grades within a posttransplant sample of adolescents with cystic fibrosis. Thirteen study participants completed the Revised Children's Manifest Anxiety Scale (RCMAS) and the Children's Depression Inventory (CDI), and had CSA trough levels and biopsy data collected. There was a statistically significant correlation between depression and inflammatory rejection levels. Adolescents with benign rejection scores had lower depression scores than adolescents with higher levels of rejection scores. The data also showed a statistically significant inverse correlation between CSA trough levels and inflammatory rejection levels. Posttransplant success depends on compliance with a strict, antirejection regimen. Adolescence is a turbulent time when many stressors can result in risk-taking behavior such as medication noncompliance and anxiety and depression. Regular psychologic monitoring of posttransplant adolescent patients may be warranted to identify increasing psychologic distress and proactive opportunities for health care intervention. This pilot study is an example of a clinical research question that evolved from a practice situation in which nurses caring for posttransplant adolescents with cystic fibrosis surmised that there were many psychologic issues influencing successful transplant outcomes. Nursing observations suggested a strong connection between poorer medication control, greater risk for rejection, and signs of depression and/or anxiety. The nurse researchers were interested in identifying significant relationships between psychologic factors and physiologic factors, and in discovering objective ways to better assess the psychologic needs of this patient population. A thorough search was conducted of the research literature, and the best clinical practices of other institutions were investigated. There was a lack of transplant research literature on adolescents, and although excellent ideas were generated from inquiries to other institutions, there was a lack of best evidence to answer the clinical questions about this group of patients. The initial clinical question became a research question to determine more about this specific patient population. This article is an example of a clinical research pilot study that resulted from an evidence-based practice process. Pilot studies are springboards for other, more refined research: They help to point the way, or to clarify what the real issues may be. For nurses, they serve as an opportunity to hone critical thinking skills and to contribute to the scientific foundation of our profession.