Daily Ards Research Analysis

OBJECTIVE: To determine the prevalence, clinical characteristics, outcomes, and mortality risk factors of pediatric acute respiratory distress syndrome (PARDS) among children with sepsis, and to compare pulmonary and extrapulmonary PARDS phenotypes. METHODS: This retrospective cohort study analyzed children aged 0-14 years with Phoenix-defined sepsis admitted to a tertiary pediatric intensive care unit between 2015 and 2023. PARDS was defined according to PALICC-2 criteria. Demographics, illness severity, microbiology, organ support requirements, and clinical outcomes were compared between children with and without PARDS and between pulmonary and extrapulmonary phenotypes. Multivariable logistic regression models were used to identify independent predictors of mortality. RESULTS: Among 279 children with Phoenix-defined sepsis, 161 (57.7%) developed PARDS. Children with PARDS were younger, had higher PELOD-2 and Phoenix severity scores, and required significantly more mechanical ventilation, vasoactive support, and renal replacement therapy compared with those without PARDS. Mortality was substantially higher in the PARDS cohort (36.6% vs. 7.6%). Model-estimated mortality probability increased stepwise with worsening PARDS severity and was highest among children with both septic shock and severe PARDS. Pulmonary PARDS accounted for two-thirds of cases, whereas extrapulmonary PARDS demonstrated a higher inflammatory burden and more bacterial infections. In adjusted analyses, the presence of PARDS, higher PELOD-2 score, and greater cumulative fluid balance were independently associated with mortality. CONCLUSION: PARDS is a common and common complication associated with high risk of pediatric sepsis, associated with severe organ dysfunction, increased support requirements, and markedly elevated mortality. These findings underscore the need for multicenter validation to confirm the epidemiology and risk factors of sepsis-associated PARDS and to guide earlier recognition and management approaches for this high-risk population.

BACKGROUND: ATP-binding cassette transporter A3 (ABCA3) deficiency is a rare form of interstitial lung disease caused by biallelic pathogenic variants in ABCA3, it is the most common cause of genetic surfactant deficiency among European and US infants and children. This study aimed to elucidate the genetic features of ABCA3 deficiency in Japanese children. MATERIALS AND METHODS: From April 2011 to March 2024, 291 candidates with children's interstitial lung disease (chILD) were enrolled. Sanger sequencing or next-generation sequencing for ABCA3 was performed for all candidates. RESULTS: Eleven cases of ABCA3 deficiency were identified, including one pair of siblings. Among eight cases with onset at birth, five-including the siblings-died. All three cases whose disease onset occurred after 1 yr of age survived, and no cases presented between 1 month and 1 yr of age. Of the 11 cases, nine carried compound heterozygous ABCA3 variants. In the remaining two cases, diagnosed as ABCA3 deficiency based on specific pathological findings, only one pathogenic variant was detected in each. No homozygous variants were found, and aside from the siblings, no cases shared the same pathogenic variants. Eighteen distinct pathogenic variants were identified, including nine missense, five nonsense, three splicing, and one frameshift variant. All were considered disease-causing. DISCUSSION: The incidence of ABCA3 deficiency among chILD candidates (11/291, 3.8%) was significantly lower (p < 0.0001) than those reported in the United States (185/632, 29.3%), Europe (79/398, 19.8%) and Argentina (14/50, 28%). The frequency of ABCA3 deficiency among Japanese children is low.

BACKGROUND: Epidermal necrolysis (EN) is a rare yet severe drug-induced disease that results in the detachment of the epidermis leading to infection and acute respiratory failure requiring mechanical ventilation. This study aims to describe the prevalence of ventilator-associated pneumonia (VAP) and its microbiological characteristics in patients with EN, as well as identifying the associated risk factors and outcomes. METHODS: This is an observational, retrospective, single-centre cohort study of adult patients with EN who were admitted to the intensive care unit (ICU) and required mechanical ventilation between 2000 and 2022. A Cox proportional hazards model was used to identify risk factors associated with the first episode of VAP. EN patients were matched with non-EN patients according to age, sex, SAPS II score, acute respiratory distress syndrome status, period of admission, and duration of mechanical ventilation. RESULTS: A total of 77 patients were included in the study. Of these, 40 (51%) experienced at least one VAP episode. Patients who developed VAP exhibited more frequent bronchial lesions (n = 26/40 [65%] vs n = 15/37 [41%]; p = 0.046), larger detached-detachable body surface area (BSA) involvement (40% [25-60] vs 25% [10-41]; p = 0.011), and had been treated more frequently with cyclosporine (n = 19/40 [48%] vs n = 9/37 [24%]; p = 0.041), as compared to those who did not develop VAP. In-hospital and ICU mortality was 36% (n = 28/77), with no significant difference between groups. By multivariable Cox regression analysis, deep (digestive and/or bronchial) mucosal involvement (adjusted standardized hazard ratio [aSHR] 2.22 IC95% [1.01;4.88]; p = 015), maximal BSA in ICU (aSHR 1.01 IC95 [1.00;1.02] for each percent; p = 0.011), and the use of cyclosporine (aSHR 2.46 [1.22;4.96]; p = 0.012) were identified as risk factors for VAP development. After matching EN and non-EN patients, it was found that EN patients were more likely to experience VAP during their stay in the ICU than non-EN patients (standardized mean difference 0.29). CONCLUSIONS: One out of two mechanically ventilated patients with EN will develop VAP, especially if they present with deep mucosal involvement, an extensive BSA in ICU, and have previously been treated by cyclosporine.