Pleuroparenchymal fibroelastosis after hematopoietic stem cell transplantation in children: a propensity score-matched analysis.

OBJECTIVES: To investigate the incidence, risk factors, and clinical outcomes of pleuroparenchymal fibroelastosis (PPFE) in pediatric hematopoietic stem cell transplantation (HSCT) recipients. METHODS: This single-center, retrospective, case-control study included 738 consecutive patients who underwent chest CT more than 3 months after HSCT. We identified patients who fulfilled the diagnostic criteria for PPFE and assessed their clinical characteristics and radiologic findings. Propensity score-matched analysis was performed using four covariates (age, sex, HSCT type, and primary disease). The risk factors and clinical outcomes of PPFE were analyzed using the Fine and Gray regression model and stratified log-rank test in the matched groups. RESULTS: PPFE was identified in 4% (31/738, 8.3 ± 3.1 years, 15 males) of the pediatric HSCT recipients with a median time of 2.7 years after HSCT, and it occurred following allogeneic (5%, 15/317), autologous (4%, 15/379), or both (2%, 1/42). Matching yielded 30 and 130 cases in the PPFE and control groups, respectively. The PPFE group showed more frequent late-onset noninfectious pulmonary complications (LONIPCs) and pneumonia more than 3 months after HSCT (p < 0.05). Multivariable analysis showed a significantly higher risk of PPFE in HSCT recipients who had pneumonia more than 3 months after HSCT (hazard ratio = 10.78 [95% confidence interval: 4.29, 27.13], p < 0.001). The PPFE group showed higher mortality (73%, 22/30) and poorer median overall survival (6.8 years [95% confidence interval: 4.1, 9.5]) than the control group (p < 0.001). CONCLUSIONS: PPFE represents a severe type of LONIPC after HSCT. HSCT recipients with pneumonia after HSCT may have an increased risk of PPFE. KEY POINTS: • The incidence of pleuroparenchymal fibroelastosis is not negligible (4%), and it can occur after either allogeneic or autologous hematopoietic stem cell transplantation. • Pleuroparenchymal fibroelastosis after hematopoietic stem cell transplantation showed poor outcome with a high mortality rate of 73% and median overall survival of 6.8 years. • After hematopoietic stem cell transplantation, pneumonia may increase the risk of pleuroparenchymal fibroelastosis development in children. • Lung biopsy should not be indicated in patients with pleuroparenchymal fibroelastosis findings on chest CT as it can cause refractory pneumothorax without helping the diagnosis.

Outcome and risk factors associated with perirenal subcapsular fluid collections in extremely preterm infants with acute kidney injury.

OBJECTIVES: To investigate the incidence of, clinical outcome of, and risk factors for perirenal subcapsular fluid collections in extremely preterm infants with acute kidney injury (AKI). METHODS: Extremely preterm infants with AKI who underwent renal ultrasonography (US) during neonatal intensive care unit stay were classified into two groups according to the presence of a perirenal subcapsular fluid collection at US. Clinical outcome was compared, and relevant data were analysed, including demographics and comorbidities of the infants, as well as maternal demographics. The risk factor of perirenal subcapsular fluid in infants with AKI was tested with univariate and multivariate logistic regression analysis. RESULTS: A perirenal subcapsular fluid collection was detected in 7 of 56 (13%) extremely preterm infants with AKI (male to female ratio, 5:2; mean gestational age, 23.6 ± 1.4 weeks) and it appeared bilaterally in most cases (86%, 6/7). The mortality rate was higher in infants with perirenal subcapsular fluid collections and AKI (86%, 6/7) than with AKI alone (35%, 17/49) (p = 0.015). Infants with perirenal subcapsular fluid collections and AKI were of a lower gestational age, and more frequently showed episodes of intestinal perforation, use of medication having potential to impair renal function, and a history of maternal chorioamnionitis (p < 0.05). Multivariate analysis revealed a significantly higher risk for perirenal subcapsular fluid collections in extremely preterm infants who were treated with anti-fungal agents (OR, 13.2 (95% CI: 1.5, 119.4); p = 0.022). CONCLUSIONS: Although a perirenal subcapsular fluid collection occurred in a small proportion of extremely preterm infants with AKI, its presence was associated with high mortality. The use of anti-fungal agents was an independent risk factor for a perirenal subcapsular fluid collection. KEY POINTS: • A perirenal subcapsular fluid collection may occur in association with acute kidney injury. • A perirenal subcapsular fluid collection has a grave prognostic implication in extremely preterm infants. • The use of anti-fungal agent might be associated with perirenal subscapular fluid collections in critically ill extremely preterm infants with AKI.

Lymphomatoid Granulomatosis Involving the Lung and Brain in a Child: A Case Report.

Lymphomatoid granulomatosis (LG) is a rare B-cell type angiocentric lymphoproliferative disease that can progress to extranodal lymphoma with high mortality. It most commonly affects the lungs, although extrapulmonary systems, including the brain and skin, can also be involved. LG in pediatric patients has been very rarely reported in the literature with limited imaging features. Herein, we report a pediatric case of LG involving the lung and brain with characteristic imaging findings.