Early Hyperglycemia Is Associated with Increased Incidence of Severe Retinopathy of Prematurity in Extremely Low Birth Weight Infants.

OBJECTIVE: Preterm infants have a higher rate of hypoglycemia and hyperglycemia, both of which have been associated with increased neonatal morbidities. The aim of this study was to evaluate the incidence of abnormal glucose homeostasis during the first 72 hours of life and study its association with major morbidities in infants admitted to our neonatal intensive care unit (NICU). STUDY DESIGN: This was a retrospective single-center study. We extracted demographic, maternal, and patient information and blood glucose levels for the first 72 hours of life for all infants with birth weight ≤1,000 g admitted to the NICU from January 2017 to December 2019. Continuous data were presented as mean ± standard deviation or as median with interquartile range. Categorical data were presented as frequency and percentage. Student's t-tests and Mann-Whitney U test were used to analyze continuous data and chi-squared test or Fisher's exact test were used to analyze categorical data. Logistic regression analysis was performed to study the relation between hyperglycemia and various morbidities after controlling for gestational age (GA). Statistical significance was set at p < 0.05. RESULTS: Of the 235 infants included in the study, 49% were hypoglycemic at admission to the NICU. Infants that were small for GA and those with history of maternal ß-blocker use had a higher incidence of hypoglycemia. Hypoglycemia at admission was not associated with increased mortality or any major morbidities. Seventy-three percent infants who were hypoglycemic or euglycemic at birth developed iatrogenic hyperglycemia during the first 72 hours of life. The incidence of retinopathy of prematurity (ROP) and severe ROP was higher in infants with hyperglycemia on univariate analysis. However, on multivariate analysis, after adjusting for GA, no difference was noted in the incidence of ROP between the two groups. Multivariate analysis could not be performed for severe ROP due to inadequate sample size. CONCLUSION: Hyperglycemia in the initial 3 days of life is associated with an increased incidence of severe ROP in preterm infants. Neonatologists should aim to maintain euglycemia in these infants to decrease the risk of adverse outcomes. KEY POINTS: · Preterm infants have a high rate of both hypoglycemia and hyperglycemia.. · Majority of infants who were euglycemic or hypoglycemic at birth develop iatrogenic hyperglycemia.. · Hyperglycemia in the initial 3 days of life is associated with an increased incidence of severe ROP in preterm infants..

Neonatal Gastric Perforation: 14-Year Experience from a Tertiary Neonatal Intensive Care Unit.

OBJECTIVE: Neonatal gastric perforations (NGPs) are rare and account for 7 to 12% of all gastrointestinal perforations in the neonatal period. The etiology and prognostic factors associated with NGP remain unclear. The aim of this study is to review the cases of NGP in our neonatal intensive care unit (NICU) in the past 14 years and describe the risk factors, clinical presentation, and outcomes associated with it. STUDY DESIGN: A retrospective chart review of neonates with gastric perforation admitted to the NICU between June 2006 and December 2020 was performed. Data regarding their antenatal and neonatal characteristics, laboratory and radiological results, intra-operative findings, hospital course, and outcomes were recorded. RESULTS: We identified 350 patients with gastrointestinal perforation at our center during the study period of which 14 (4%; nine males and five females) patients were diagnosed with NGP during surgery. A total of 71% neonates were born preterm (range: 24-39 weeks, median: 34 weeks). Two neonates (14%) were SGA. Only one neonate received cardiopulmonary resuscitation at birth. In all neonates, except two, perforation occurred within the first 10 days of life (median: 4 days, range: 1-22 days). In total, 79% infants received feeds prior to perforation. Ten neonates had a feeding tube, and one neonate had a gastrostomy tube placed prior to perforation. Abdominal distension and pneumoperitoneum were present in all neonates. Majority of the babies had metabolic acidosis (64%) and elevated C-reactive protein (79%). Most (86%) neonates received surgical intervention within 12 hours. Overall survival in our study was 93%. CONCLUSION: NGP is a rare entity seen mostly in preterm infants within the first 10 days of life. Clinical presentation is similar to perforation anywhere along the gastrointestinal tract and definite diagnosis requires exploratory laparotomy. With prompt recognition and surgical intervention, the overall mortality related to neonatal gastric perforation is low. KEY POINTS: · Neonatal gastric perforation is a rare but life threatening entity with unclear etiology.. · Prematurity is associated with an increased incidence of gastric perforations in the neonate.. · Laparotomy is required for definitive diagnosis and treatment..

Improving Red Reflex Screening in a Level III NICU Through a Quality Improvement-based Approach.

BACKGROUND: Red reflex is a routine part of newborn examination in most high-income countries. It is an inexpensive, noninvasive method of detecting serious ocular abnormalities like cataracts, retinoblastoma, vitreous masses, etc. The American Academy of Pediatrics recommends red reflex examination before discharge from newborn nursery. However, the current rate of red reflex examination in the NICUs in the United States is unknown. We noted a low rate of documentation (19%) in our level III NICU, prompting us to initiate this quality improvement project to improve this rate. METHODS: We created a key-driver diagram and summarized possible interventions to achieve our aim to increase the documentation rate to >80%. We implemented various interventions over 4 plan-do-study-act cycles. Over 19 months, we educated the nurses and the providers regarding the importance of red reflex assessment, placed visual reminders to check red reflex, implemented discharge checklist for the residents, and improved the accessibility to ophthalmoscope. RESULTS: Infants discharged from our NICU during a 25-month period included 1168 infants who an ophthalmologist did not formally examine. The rate of red reflex documentation improved significantly from a baseline of 19% (6 months before the first plan-do-study-act cycle) to 89.5% (during the 19-month intervention period). One abnormal red reflex was detected during this study. CONCLUSIONS: Implementation of this project has led to a culture change at our institution, which will help prevent us from missing the diagnosis of serious visual abnormalities in the future.

Pharmacotherapy in Bronchopulmonary Dysplasia: What Is the Evidence?

Bronchopulmonary Dysplasia (BPD) is a multifactorial disease affecting over 35% of extremely preterm infants born each year. Despite the advances made in understanding the pathogenesis of this disease over the last five decades, BPD remains one of the major causes of morbidity and mortality in this population, and the incidence of the disease increases with decreasing gestational age. As inflammation is one of the key drivers in the pathogenesis, it has been targeted by majority of pharmacological and non-pharmacological methods to prevent BPD. Most extremely premature infants receive a myriad of medications during their stay in the neonatal intensive care unit in an effort to prevent or manage BPD, with corticosteroids, caffeine, and diuretics being the most commonly used medications. However, there is no consensus regarding their use and benefits in this population. This review summarizes the available literature regarding these medications and aims to provide neonatologists and neonatal providers with evidence-based recommendations.

Early-Onset Fulminant Sepsis in a Preterm Neonate due to Streptococcus gallolyticus : A Case Report and Literature Review.

Streptococcus gallolyticus is an uncommon cause of neonatal infections. We describe the first case of fulminant lethal neonatal sepsis due to S. gallolyticus reported in literature. Our patient was an extremely low birth weight premature infant born to a mother with prolonged rupture of amniotic membranes and chorioamnionitis. We also review the cases of neonatal S. gallolyticus infections reported in literature. Fifty-eight percent neonatal S. gallolyticus infections presented in the first week of life. Importantly, S. gallolyticus meningitis is more commonly reported with early-onset infections compared with group B streptococcal meningitis, which is more common with late-onset infections. Streptococcus gallolyticus should be included in differential for neonatal sepsis, particularly in the presence of meningitis in the first week of life. Most cases are sensitive to penicillin; however, cases of reduced sensitivity to penicillin have also been reported.

A novel variant in the dystonin gene causing hereditary sensory autonomic neuropathy type VI in a male infant: Case report and literature review.

The DST gene is located on chromosome 6p and encodes for a large protein. Alternative splicing of this protein produces the neuronal (a1-a3), muscular (b1-b3), and epithelial (e) isoforms. Hereditary sensory and autonomic neuropathy (HSAN) type VI is a rare autosomal recessive disorder due to mutations affecting the a2 isoform. We present a case of HSAN-VI in a male neonate born to consanguineous parents. Genome sequencing revealed a novel homozygous variant (DST_c.1118C > T; p.Pro373Leu) inherited from both parents. This case further expands the phenotype and genotype of this rare syndrome.

Case Report: An Infant With Kabuki Syndrome, Alobar Holoprosencephaly and Truncus Arteriosus: A Case for Whole Exome Sequencing in Neonates With Congenital Anomalies.

Kabuki syndrome is a rare multiple anomalies syndrome associated with mutations in KMT2D or KDM6A. It is characterized by infantile hypotonia, developmental delay and/or intellectual disability, long palpebral fissures with everted lateral third of the lower eyelids and typical facial features. Intracranial anomalies occur infrequently in patients with KS and holoprosencephaly has only been recently described. Additionally, though congenital heart diseases are common in patients with KS, to our knowledge truncus arteriosus has never been reported in a patient with KS. We present an unusual case of KS in an infant with holoprosencephaly and truncus arteriosus with partial anomalous pulmonary venous return. Duo whole exome sequencing in our patient identified a pathogenic nonsense variant in exon 10 of KMT2D (c.2782C > T; p. Gln928*) establishing the diagnosis. This report further expands the phenotypic spectrum of patients with Kabuki syndrome and emphasizes the utility of performing large scale sequencing in neonates with multiple congenital anomalies.

Kagami-Ogata Syndrome: Case Series and Review of Literature.

Kagami-Ogata syndrome (KOS) (OMIM #608149) is a genetic imprinting disorder affecting chromosome 14 that results in a characteristic phenotype consisting of typical facial features, skeletal abnormalities including rib abnormalities described as "coat hanger ribs," respiratory distress, abdominal wall defects, polyhydramnios, and developmental delay. First identified by Wang et al in 1991, over 80 cases of KOS have been reported in the literature. KOS, however, continues to remain a rare and potentially underdiagnosed disorder. In this report, we describe two unrelated male infants with differing initial presentations who were both found to have the characteristic "coat hanger" rib appearance on chest X-ray, raising suspicion for KOS. Molecular testing confirmed KOS in each case. In addition to these new cases, we reviewed the existing cases reported in literature. Presence of polyhydramnios, small thorax, curved ribs, and abdominal wall defects must alert the perinatologist toward the possibility of KOS to facilitate appropriate molecular testing. The overall prognosis of KOS remains poor. Early diagnosis allows for counseling by a multidisciplinary team and enables parents to make informed decisions regarding both pregnancy management and postnatal care.

Initial ED oxygen saturation ≤90% increases the risk of a complicated hospital course in pediatric asthmatics requiring admission.

Emergency physicians are responsible for admitting children with asthma who do not respond to initial therapy. We examined the hypothesis that an initial room air pulse oximetry ≤90% elevates the risk of a complicated hospital course in children who require admission with acute asthma. METHODS: Charts of all patients ages 2 years-17 years admitted for asthma from January 2017 to December 2017 were reviewed. An explicit chart review was performed by trained data extractors using a standardized form. RESULTS: A total of 244 children meeting inclusion criteria were admitted for asthma from the ED during the study period. All patients had an initial room air pulse oximetry documented. Sixty-five were admitted to PICU status (27%), and 179 (73%) were admitted to floor status. The relative risk of a complicated course in those patients presenting with a saturation of ≤90% was 11.3 (95% CI 3.9-32.6). The mean initial pulse oximetry on patients with a complicated course was 85% versus 93% for those without a complicated course (p < 0.005). CONCLUSION: Our data suggest that in pediatric asthmatics that require admission from the ED, those with pulse oximetry readings less than or equal to 90% on presentation are at higher risk of a complicated hospital course.