ABSTRACT
Objective:To explore the efficacy of andrographolide sulfonate(ADS) nebulized inhalation on pediatric moderate to severe acute respiratory distress syndrome(ARDS) requiring invasive mechanical ventilation.Methods:We conducted a prospective randomized controlled single-blind study.Children with moderate-to-severe ARDS admitted to the PICU at Beijing Children′s Hospital of Capital Medical University from November 1, 2018 to December 31, 2019, aging from 29 days to 18 years, and requiring invasive mechanical ventilation therapy were collected.The experimental group received ADS, while the control group received normal saline.Bronchoalveolar lavage fluid was collected to detect cytokines before and after the experiment.The differences of demography, cytokines and management between two groups were analyzed.Results:Twenty children with a median age of 2.15(1.48, 8.01)years were included and 15(75.00%)cases were boys.Median score of pediatric index of mortality-2 was 12.25(4.53, 16.30). There was no significant differences in demography, basic clinical data and prognosis between two groups( P>0.05). Monocyte chemoattractant protein-1 decreased in the experimental group while increased in the control group with statistic difference[967.50(119.25, 5 206.00)pg/mL vs.-945.00(-3 935.50, 495.09)pg/mL, P=0.041]. Interleukin(IL)-8 decreased in the experimental group but increased in the control group[303.22(-452.00, 1 172.38)pg/mL vs.-490.14(-780.25, 240.52)pg/mL, P=0.151]; and the IL-6 increase of the experimental group was lower than that of the control group[-24.53(-501.76, 135.27)pg/mL vs.-325.85(-633.22, 133.75)pg/mL, P=0.364]; all with no statistic differences( P>0.05). The oxygenation index[11.35(6.00, 15.83) vs.20.65(6.23, 38.35), P=0.374] and the improvement rate of ARDS(80%vs.60%, P=0.628) of the experimental group was better than that of the control group, but with no statistic difference( P>0.05). There was no statistic difference of mortality and mechanical ventilation time between two groups( P>0.05). Conclusion:Inhalation of ADS might reduce the increase of IL-6 and the concentration of monocyte chemoattractant protein-1 and IL-8 in bronchoalveolar lavage fluid of children with ARDS, and might improve pulmonary oxygenation function.Further research is needed to verify the above conclusion.
ABSTRACT
Objective To explore the diagnosis and treatment of atypical severe combined immunodeficiency disease (SCID). Methods The clinical data of atypical SCID in 7 children with IL2RG,JAK3,and RAG1 mutations were reviewed and analyzed from September 2012 to June 2017. Results In 7 cases (6 males and 1 female), there were 5 infants, 1 toddler and 1 school-age child. Cases 2, 4, and 6 were classic SCID clinical phenotypes. Cases 1, 3, 5, 7 were atypical SCID clinical phenotypes. Case 6 were diagnosed with Omenn syndrome. Cases 2, 5 were classic SCID immune phenotypes, cases 1, 3, 4, 6, 7 were atypical SCID immune phenotypes, and case 1 had maternal chimera. The next generation sequencing indicated that case 1 had a compound heterozygous JAK3 mutation with c.3097-1G>A/c.946-950GCGGA>ACinsGGT.Cases 2,3,and 4 had IL2RG mutations,with c.865C>T/p.R289X,c.664C>T/R222C,52delG,respectively.Case 5 had JAK3 mutations with c.2150A>G/p.E717G and c.1915-2A>G.Sanger sequencing indicated that case 6 had a RAG1 mutation of complex heterozygosity with c.994C>T/p.R332X and c.1439G>A/p.S480N. Case 7 had homozygous RAG1 mutation with c.2095C>T/p.R699W.Conclusion Under certain conditions,gene mutation can lead to atypical clinical and/or immune phenotypic SCID.