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ObjectiveThis study aimed to assess the efficacy and safety of 2 Janus kinase (JAK) inhibitors (jakinibs) tofacitinib and ruxolitinib in the treatment of type I interferonopathies patients including STING-associated vasculopathy with onset in infancy (SAVI), Aicardi-Goutières syndrome (AGS), and spondyloenchondrodysplasia with immune dysregulation (SPENCD).MethodsA total of 6 patients were considered in this study: 2 patients with SAVI, 1 patient with AGS1, 1 patient with AGS7, and 2 patients with SPENCD. Clinical manifestations, laboratory investigations, radiology examinations, treatment, and outcomes were collected between November 2017 and November 2021 in Peking Union Medical College Hospital. The disease score for patients with SAVI and AGS scale for patients with AGS were documented. The expression of 6 interferon-stimulated genes (ISGs) was assessed by real-time PCR.ResultsThree patients (1 patient with SAVI, 2 patients with AGS) were treated with ruxolitinib and 3 patients (1 patient with SAVI, 2 patients with SPENCD) were treated with tofacitinib. The mean duration of the treatment was 2.5 years (1.25–4 years). Upon treatment, cutaneous lesions and febrile attacks subsided in all affected patients. Two patients discontinued the corticoid treatment. Two patients with SAVI showed an improvement in the disease scores (p p ConclusionThe JAK inhibitors baricitinib and tofacitinib are promising therapeutic agents for patients with SAVI, AGS, and SPENCD, especially for the improvement of cutaneous lesions and febrile attacks. However, further cohort studies are needed to assess the efficacy and safety.

ObjectivesTo explore the status of parental nutrition practice of hospitalized late preterm infants and the factors influencing the clinical prescription.MethodsA multi-center, prospective cohort study was conducted during October 2015 to October 2017. Infants born after 34 weeks and before 37 weeks were enrolled from twenty-five hospitals in the Beijing area of China. Data of enteral and parenteral nutrition were collected.ResultsA total of 1,463 late preterm infants were enrolled in this study, 53.9% of infants were supported by parenteral nutrition. Over 60% of 34 weeks’ infants were on parenteral nutrition during the 2nd to the 4th day. Logistic regression analysis showed that gestational age(GA) (OR = 0.69, 95%CI 0.58–0.81), birth weight (OR = 0.41, 95%CI 0.26–0.65), hypoglycemia (OR = 2.77, 95%CI 1.90–4.04), small for gestational age (SGA) (OR = 2.18, 95%CI 1.34–3.55), feeding intolerance (OR = 6.41, 95%CI 1.90–21.59), neonatal respiratory distress syndrome (NRDS) (OR = 2.16, 95%CI 1.12–4.18), neonatal infection(OR = 1.56 95%CI 1.16–2.10), and slow enteral nutrition advancement rate (OR = 0.92, 95%CI 0.90–0.95) were factors influencing the administration of parenteral nutrition.ConclusionOver half of hospitalized late preterm infants were prescribed with parenteral nutrition. Infants with lower GA, lower birth weight, diagnosed with hypoglycemia, SGA, feeding intolerance, NRDS, neonatal infection, or a slower rate of enteral nutrition advancement had a higher likelihood of receiving parenteral nutrition.