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Purpose: Radiographic examinations are frequently performed for diagnostic and therapeutic purposes in neonatal intensive care units (NICUs). However, concerns are emerging regarding the safety of radiation exposure, especially in vulnerable preterm infants in periods of rapid cellular division. This quality improvement (QI) project aimed to reduce radiation hazards in level-IV NICU. Methods: We established an "X-ray prescription protocol" and educated the physicians to ensure that only essential radiographs were obtained. Additionally, we discouraged full-body infantograms and emphasized the prescription of targeted radiographs, such as chest or abdominal radiographs. Furthermore, to reduce the dose-area product (DAP, Gy·cm2) values, which act as a surrogate for radiation exposure, we provided training to radiologic technologists on meticulous collimation for each radiography session. We aimed to achieve a 30% reduction in the average monthly cumulative DAP per patient, which was calculated by dividing the total monthly DAP from radiographs in the NICU by the monthly average of patient admissions. Retrospective baseline data were collected 8 months pre-intervention and prospectively for 4 months post-interventions. Results: The average monthly X-ray count per patient was 28.3 in the pre-intervention period (October 2022 to May 2023), which decreased to 25.4 in the post-intervention period (June 2023 to September 2023), reflecting a 10.2% reduction (p=0.109). The average monthly infantogram count per patient showed an 18.0% reduction (25.9% to 21.2%, p=0.016), and the proportion of infantograms in the total X-ray counts decreased from 91.5% to 83.3% (p=0.017). The DAP value per X-ray decreased by 42.6%, from an average of 0.25 to 0.14 (p=0.011). The primary outcome, the average monthly cumulative DAP value per patient, showed a substantial reduction of 48.6%, dropping from 7.00 to 3.60 (p=0.004). The baseline characteristics and short-term morbidities of the patients did not differ significantly between the pre- and post-intervention period. Conclusion: Our QI approach, which included discouraging excessive prescriptions of infantograms and promoting optimal collimation, significantly reduced the average monthly radiation exposure in the NICU, benefiting both patients and healthcare workers. [ABSTRACT FROM AUTHOR]

Introduction: Holocarboxylase synthetase deficiency (HLCS deficiency, OMIM #253270) is an exceedingly rare metabolic disorder resulting in multiple carboxylase deficiencies owing to impaired biotin cycle. Clinical manifestations include severe metabolic acidosis, hyperammonemia, tachypnea, skin rash, alopecia, feeding problems, hypotonia, developmental delay, seizures, and, in severe cases, death. Methods and Results: An 8‐day‐old female neonate presented with severe lactic acidosis, necessitating sedation and mechanical ventilation. Despite receiving supportive care, no evident clinical improvement was observed, accompanied by the onset of generalized ichthyosis. Genetic analysis of actionable metabolic disorders revealed compound heterozygous variants of HLCS (NM_000411.8), specifically c.[710T>C (p.Leu237Pro)]; [1544G>A (p.Ser515Asn)], prompting the initiation of biotin mega‐dose therapy (10 mg/day). Remarkably, dramatic clinical improvement in lactic acidosis was observed the day after initiating biotin administration, leading to the discontinuation of mechanical ventilation within 6 days. The patient remained in stable condition during follow‐up, exhibiting normal growth and development along with consistently stable laboratory findings up to 18 months of age. Conclusion: Our case highlights the significance of early genetic testing in neonates with unexplained metabolic disorders to enable timely diagnosis and therapy initiation. Biotin therapy has demonstrated remarkable efficacy in improving the clinical condition of patients with HLCS deficiency, leading to favorable outcomes. [ABSTRACT FROM AUTHOR]

Background: Pulmonary hypertension (PH) is a life-threatening condition in newborns. We aimed to assess the clinical and echocardiographic responses of term and preterm infants to treprostinil. Methods: This retrospective study included newborns diagnosed with PH and treated with treprostinil as additional therapy after inhaled nitric oxide administration in the neonatal intensive care unit of a tertiary center. Term and preterm infants were compared in terms of echocardiographic findings and clinical findings 4 weeks after treprostinil treatment. Results: During the study period, 11 term and 18 preterm infants were diagnosed with PH and received treprostinil. There were no differences in the echocardiographic findings of interventricular septal deviation, direction of shunt, and ratio of estimated pulmonary artery pressure over systolic blood pressure. Congenital diaphragmatic hernia was the most common condition occurring upon PH diagnosis among term infants, while severe bronchopulmonary dysplasia was the most common in preterm infants. Improvements in echocardiographic findings were more pronounced in term infants than in preterm infants (100% vs. 55.6%, P = 0.012). The inhaled nitric oxide dose was gradually tapered for term infants and was lower than that for preterm infants at 1, 2, and 3 weeks after treprostinil. Conclusion: Intravenous treprostinil could be an adjuvant therapy option for term and preterm infants with PH, especially for those who cannot receive oral medication. The efficacy and safety of treprostinil in this population with PH should be investigated further. [ABSTRACT FROM AUTHOR]