Acute Bowel Ischemia in a Premature Neonate with Miller-Dieker Syndrome and Anomalous Right Coronary Artery From the Pulmonary Artery.

Miller-Dieker syndrome (MDS) is a rare disease characterized by type I lissencephaly, craniofacial dysmorphisms, intellectual disability, seizures, and death in early childhood. We report a case of a premature infant with MDS with an anomalous right coronary artery from the pulmonary artery who developed sudden bowel ischemia. This case prompts the reconsideration of cardiovascular involvement in patients with MDS. In addition, this review highlights key clinical features and reviews the critical manifestations of MDS that persist into childhood. [Pediatr Ann. 2023;52(8):e283-e291.].

Increased prevalence of severe neonatal hyperbilirubinaemia during social distancing.

AIM: Social distancing guidelines implemented with the COVID-19 pandemic impacted health-care utilisation and disrupted critical social supports. Resurgence of highly transmissible strains has resulted in revisiting restrictions with potential impacts on newborn health. With concerns for inadequate post-partum support, we sought to determine if social distancing correlated with increased rates of readmission for hyperbilirubinaemia. METHODS: Retrospective chart review identified all readmissions for hyperbilirubinaemia between 1/18 and 4/20 in Western New York. Infant/maternal demographics and data on hospital course were collected on control (1/1/18-31/1/20) and social distancing (1/2/20-30/4/20) cohorts. Nineteen outpatient clinics were surveyed regarding lactation support. RESULTS: Monthly readmissions for hyperbilirubinaemia nearly tripled during social distancing (0.90 ± 0.91 vs. 2.63 ± 2.29 per 1000 births during early COVID, P = 0.015). Comparable severity of disease at readmission was observed with no difference in the need for therapies (phototherapy, intravenous immunoglobulin or exchange transfusion) or length of hospital stay. Mothers were younger (25.8 ± 3.3 vs. 31.3 ± 4.7 years; P = 0.005) with higher rates of primiparity and exclusive breastfeeding than national norms, however not significantly higher than controls in our small cohort (62.5 vs. 37.0% for primiparity; 87.5 vs. 81.5% for breastfeeding). Of 19 clinics surveyed, only six confirmed a telemedicine option for lactation support. CONCLUSIONS: Rates of readmission for hyperbilirubinaemia increased during social distancing. Younger maternal age with high rates of primiparity and exclusive breastfeeding raise concern for inadequate social and/or lactation support. Proactive identification of mothers at risk and expansion of remote lactation services may be indicated with recurrent waves of the pandemic.

Central line-associated bloodstream infection among children with biliary atresia listed for liver transplantation.

BACKGROUND: Pre-transplant nutrition is a key driver of outcomes following liver transplantation in children. Patients with biliary atresia (BA) may have difficulty achieving satisfactory weight gain with enteral nutrition alone, and parenteral nutrition (PN) may be indicated. While PN has been shown to improve anthropometric parameters of children with BA listed for liver transplantation, less is known about the risks, particularly infectious, associated with this therapy among this specific group of patients. AIM: To describe the incidence, microbiology, and risk factors of central line-associated bloodstream infection (CLABSI) among children with BA listed for liver transplantation. METHODS: Retrospective review of children aged ≤ 2-years of age with BA who were listed for primary liver transplantation at Texas Children's Hospital from 2008 through 2015 (n = 96). Patients with a central line for administration of PN (n = 63) were identified and details of each CLABSI event were abstracted. We compared the group of patients who experienced CLABSI to the group who did not, to determine whether demographic, clinical, or laboratory factors correlated with development of CLABSI. RESULTS: Nineteen of 63 patients (30%, 95%CI: 19, 43) experienced 29 episodes of CLABSI during 4800 line days (6.04 CLABSI per 1000 line days). CLABSI was predominantly associated with Gram-negative organisms (14/29 episodes, 48%) including Klebsiella spp., Enterobacter spp., and Escherichia coli. The sole polymicrobial infection grew Enterobacter cloacae and Klebsiella pneumoniae. Gram-positive organisms (all Staphylococcus spp.) and fungus (all Candida spp.) comprised 9/29 (31%) and 6/29 (21%) episodes, respectively. No demographic, clinical, or laboratory factors were significantly associated with an increased risk for the first CLABSI event in Cox proportional hazards regression analysis. CONCLUSION: There is substantial risk for CLABSI among children with BA listed for liver transplantation. No clinical, demographic, or laboratory factor we tested emerged as an independent predictor of CLABSI. While our data did not show an impact of CLABSI on the short-term clinical outcome, it would seem prudent to implement CLABSI reduction strategies in this population to the extent that each CLABSI event represents potentially preventable hospitalization, unnecessary healthcare dollar expenditures, and may exact an opportunity cost, in terms of missed allograft offers.