Objective To explore the relationship between serum silent information regulator 2 related en-zyme(SIRT)3,SIRT6,and sepsis complicated with acute respiratory distress syndrome(ARDS)and progno-sis.Methods Sixty-eight patients with sepsis complicated with ARDS admitted to the Baoding First Central Hospital from March 2020 to February 2022 were selected as the study objects,and were divided into mild group,moderate group and severe group according to the criteria of mild,moderate and severe ARDS.Accord-ing to the prognosis within 28 days,the patients were divided into survival group and death group.The levels of serum SIRT3 and SIRT6 were detected by enzyme-linked immunosorbent assay.The sequential organ fail-ure assessment(SOFA)scores,acute physiology and chronic health evaluation Ⅱ(APACHE Ⅱ)scores,the levels of serum SIRT3 and SIRT6 in each group were compared.The clinical data of patients were collected,the influencing factors of mortality in patients with sepsis complicated with ARDS was analyzed by univariate and multivariate Logistic regression.The predictive value of serum SIRT3 and SIRT6 of mortality in patients with sepsis complicated with ARDS was analyzed by receiver operating characteristic(ROC)curve.Results The levels of serum SIRT3 and SIRT6 in moderate group and severe group were lower than those in mild group,the scores of SOFA and APACHE Ⅱ were higher than those in mild group,the levels of serum SIRT3 and SIRT6 in severe group were lower than those in moderate group,and the scores of SOFA and APACHE Ⅱwere higher than those in moderate group,with statistical significance(P＜0.05).There were statistically significant differences in age,mechanical ventilation time,lactate,arterial oxygenation index[arterial partial pressure of oxygen(PaO2)/inhaled oxygen concentration(FiO2)],C reactive protein(CRP),interleukin(IL)-6,SIRT3,SIRT6,SOFA score and APACHE Ⅱ score between the two groups(P＜0.05).Longer mechanical ventilation time,higher lactic acid,higher CRP,higher IL-6,higher SOFA score and higher APACHE Ⅱ score were all risk factors for death in sepsis complicated with ARDS within 28 days,while larger PaO2/FiO2,high-er SIRT3 and higher SIRT6 were protective factors(P＜0.05).ROC curve results showed that the area under the curve and 95％CI of serum SIRT3,SIRT6 levels,SOFA score and APACHE Ⅱ score were 0.706(0.493-0.922),0.722(0.497-0.954),0.753(0.570-0.922),0.710(0.442-0.952)and 0.872(0.761-0.976),respectively when applied alone and in combination.Conclusion Serum SIRT3 and SIRT6 levels are decreased in patients with sepsis complicated with ARDS,and the lower the serum SIRT3 and SIRT6 levels are as the disease worsens,the two can help predict the prognosis of patients with sepsis complicated with ARDS.

The clinical data, laboratory testing, genetic testing results, diagnosis and treatment process of a child with PERCHING syndrome diagnosed and treated in the Department of Neonatology, the Third Affiliated Hospital of Zhengzhou University in June 2022 were retrospectively analyzed, and the relevant literatures were reviewed.The proband mainly presented with dyspnea and feeding difficulties after delivery, facial nevus flammeus, protrusion of eyes, small fissure of eyes, wide nasal root, limited opening of mouth, slightly high palatal arch, special posture, cryptorchid, hypospadias, and high muscle tone of limbs.Magnetic resonance imaging of the brain suggested possible agenesis of corpus callosum.Genetic testing showed complex heterozygous variations in the KLHL7 gene, and the two mutation sites have not been previously reported.A case of PERCHING syndrome caused by the KLHL7 gene mutation in China was reported for the first time, which provided new ideas for the diagnosis and treatment of children with PERCHING syndrome and reliable genetic evidence for family reproduction.