Lung Ultrasound to Monitor Extremely Preterm Infants and Predict Bronchopulmonary Dysplasia. A Multicenter Longitudinal Cohort Study.

Rationale: Lung ultrasound is useful in critically ill patients with acute respiratory failure. Given its characteristics, it could also be useful in extremely preterm infants with evolving chronic respiratory failure, as we lack accurate imaging tools to monitor them. Objectives: To verify if lung ultrasound can monitor lung aeration and function and has good reliability to predict bronchopulmonary dysplasia in extremely preterm neonates. Methods: A multicenter, international, prospective, longitudinal, cohort, diagnostic accuracy study consecutively enrolling inborn neonates with gestational age 30+6 weeks or younger. Lung ultrasound was performed on the 1, 7, 14, and 28 days of life, and lung ultrasound scores were calculated and correlated with simultaneous blood gases and work of breathing score. Gestational age-adjusted lung ultrasound scores were created, verified in multivariate models, and subjected to receiver operator characteristics (ROC) analyses to predict bronchopulmonary dysplasia at 36 weeks postmenstrual age. Measurements and Main Results: Mean lung ultrasound scores are different between infants developing (n = 72) or not developing (n = 75) bronchopulmonary dysplasia (P < 0.001 at any time point). Lung ultrasound scores significantly correlate with oxygenation metrics and work of breathing at any time point (P always < 0.0001). Gestational age-adjusted lung ultrasound scores significantly predict bronchopulmonary dysplasia at 7 (area under ROC curve, 0.826-0.833; P < 0.0001) and 14 (area under ROC curve, 0.834-0.858; P < 0.0001) days of life. Bronchopulmonary dysplasia severity and gestational age-adjusted lung ultrasound scores are significantly correlated at 7 and 14 days (P always < 0.0001). Conclusions: Lung ultrasound scores allow monitoring of lung aeration and function in extremely preterm infants. Gestational age-adjusted scores significantly predict the occurrence of bronchopulmonary dysplasia, starting from the seventh day of life.

Echography-Guided Surfactant Therapy to Improve Timeliness of Surfactant Replacement: A Quality Improvement Project.

OBJECTIVE: To improve time of surfactant administration with a surfactant replacement protocol based on semiquantitative lung ultrasound score (LUS) thresholds. STUDY DESIGN: Quality improvement (QI), prospective, before-after, pilot study. In a 6-month period surfactant replacement was based only on inspired oxygen fraction (FiO2) thresholds. In the second 6-month period, surfactant was given when either the FiO2 or LUS exceeded the limits. The main QI measures were the proportion of neonates receiving surfactant within the first 3 hours of life and maximal FiO2 reached before surfactant replacement. Secondary QI measures were the duration of respiratory support and ventilator-free days. Data were also collected for 1 year after the study to verify sustainability. RESULTS: Echography-guided Surfactant THERapy (ESTHER) increased the proportion of neonates receiving surfactant within the first 3 hours of life (71.4%-90%; P < .0001) and reduced the maximal FiO2 reached before surfactant replacement (0.33 [0.26-0.5]) vs 0.4 [0.4-0.55]; P = .005). The global need for surfactant did not significantly change. ESTHER also resulted in a significant decrease in duration of invasive ventilation and ventilator-free days. CONCLUSIONS: ESTHER improved the timeliness of surfactant administration and secondary QI indicators related to surfactant replacement.

Tonsillectomy efficacy in children with PFAPA syndrome is comparable to the standard medical treatment: a long-term observational study.

OBJECTIVES: Tonsillectomy has recently been suggested as an effective treatment for PFAPA syndrome but little is known about its long-term efficacy. We compared the clinical features and the long-term outcome of a large cohort of patients with PFAPA syndrome treated with tonsillectomy or with standard medical treatment. METHODS: We conducted a retrospective study on patients with PFAPA syndrome followed at a tertiary care centre from January 1993 to August 2010. Clinical characteristics and laboratory parameters were evaluated at onset and during the follow-up. Disease outcomes of patients who underwent tonsillectomy and of those treated with medical therapy (NSAIDs, prednisone) were compared. Clinical remission on medication (CRM) was considered the persistence of fever attacks which were well controlled by medical therapy, clinical remission (CR) was defined as the absence of fever attacks, without any treatment, for more than 12 months. RESULTS: 275 patients with PFAPA syndrome, 59.6% males, aged 27.9 months at onset and followed for mean 54.5 months, entered the study. CR was reported in 59.6% of the patients and was significantly less frequent in those with positive family history for PFAPA (46.4% vs. 66.1%, p=0.003). 27/41 patients (65.9%), responded to tonsillectomy and this result was comparable with that observed in those treated with medical therapy (59.1%, p=0.51). Disease duration, age at remission or presence of associated symptoms were not significantly different in both groups. No predictors of tonsillectomy failure were found. CONCLUSIONS: In a large cohort of patients with PFAPA syndrome, tonsillectomy efficacy was comparable to the standard medical treatment.

Semiquantitative Ultrasound Assessment of Lung Aeration Correlates With Lung Tissue Inflammation.

We studied the relationship between ultrasound-assessed lung aeration and inflammation in a particular population of ventilated preterm neonates with mild-to-moderate lung inflammation and no congenital heart defect. Lung aeration estimated by a semiquantitative lung ultrasound score significantly correlated with several inflammatory markers both at cellular (neutrophil count in bronchoalveolar lavage: ρ = 0.400, p = 0.018) and molecular level (total proteins: ρ = 0.524, p = 0.021; interleukine-8: ρ = 0.523, p = 0.021; granulocytes-macrophages colony stimulating factor: ρ = 0.493, p = 0.020; all measured in bronchoalveolar lavage and expressed as epithelial lining fluid concentrations). Lung ultrasound might detect changes in lung aeration attributable to mild-to-moderate local inflammation if cardiogenic lung edema is excluded. Thus, it is possible to describe some levels of lung inflammation with semiquantitative lung ultrasound.

Periodic fevers with aphthous stomatitis, pharyngitis, and adenitis (PFAPA).

PFAPA syndrome (acronym of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) is the most common cause of periodic fever in childhood. Nowadays, it is considered part of the wide family of the autoinflammatory diseases, but a genetic or molecular marker hasn't been identified yet, therefore, its etiology is still unknown. Diagnosis is essentially based on clinical criteria but, especially in younger children, it is sometimes difficult to differentiate it from other hereditary periodic fever syndromes. Fever attacks in PFAPA have a spontaneous resolution and in a high rate of patients the syndrome ends spontaneously over time. Treatment is still a matter of debate. Usually a single administration of oral corticosteroids aborts attacks. Tonsillectomy may be an alternative option but its role remains to be clarified.