To generate hypotheses about which subgroups of newborns with severe respiratory distress syndrome might benefit most from high-frequency oscillatory ventilation.
Retrospective analysis of a case ...series of newborns with severe respiratory distress syndrome who were treated in our department with high-frequency oscillatory ventilation.
Referral center for neonatal and pediatric intensive care medicine.
All newborns (n = 18), admitted between June 1991 and February 1993, of various gestational ages (26 to 41 wks), with severe respiratory distress syndrome caused by various underlying pulmonary diseases who did not respond to conventional therapy and who thus were treated with high-frequency oscillatory ventilation.
Survival until discharge from our unit and persistent improvement of gas exchange.
Eight (44%) of 18 patients survived; ten (55%) patients died. Four (22%) survivors showed marked clinical improvement with the initiation of high-frequency oscillatory ventilation. Four (22%) survivors did not respond to high-frequency oscillatory ventilation. The responder group consisted of term or near-term neonates (gestational age at least 35 wks) with pulmonary disease that was complicated by persistent pulmonary hypertension. The group of premature neonates with a gestational age of < 35 wks did not respond to high-frequency oscillatory ventilation.
As a result of our analysis, we hypothesize that term newborns with severe respiratory distress syndrome complicated by persistent pulmonary hypertension and hypercarbia can benefit from high-frequency oscillatory ventilation. Premature neonates with ventilation-induced lung injury are not likely to respond to high-frequency oscillatory ventilation.
The purpose of this report is to propose a staged therapeutic protocol for the treatment of persistent pulmonary hypertension of the newborn (PPHN) based on retrospective clinical experience. We ...analysed the clinical course of 19 term and near-term neonates with severe PPHN treated between 1991 and 1993, before the introduction of inhalational nitric oxide (NO) therapy. Basic therapy consisted of continuous sedation, and analgesia, circulatory support with dobutamine, dopamine and substitution of 5% albumin or packed red blood cells, mechanical hyperventilation, alkalisation with sodium bicarbonate and surfactant instillation. Consecutive therapy included the administration of norepinephrine, high frequency oscillatory ventilation (HFOV), Prostacyclin (PGI2) and extracorporeal membrane oxygenator (ECMO) therapy with entry criteria for each stage of treatment. From our observations we suggest that firstly, an early increase in systemic mean arterial pressure due to norepinephrine and secondly, HFOV trials are beneficial in patients with PPHN. The staged protocol shall be applicable in most neonatal intensive care units in which inhalational NO and ECMO therapies are not available and includes our present entry criteria for both therapies.
The ABCA3 gene encodes a lipid transporter in type II pneumocytes critical for survival and normal respiratory - function. The frequent ABCA3 variant R288K increases the risk for neonatal respiratory ...distress syndrome among term and late preterm neonates, but its role in children's interstitial lung disease has not been studied in detail. In a retrospective cohort study of 228 children with interstitial lung disease related to the alveolar surfactant - system, the frequency of R288K was assessed and the phenotype of patients carrying a single R288K variant further characterized by clinical course, lung histology, computed tomography and bronchoalveolar lavage phosphatidylcholine PC 32:0. Cell lines - stably transfected with ABCA3-R288K were analyzed for intracellular transcription, processing and targeting of the protein. ABCA3 - function was assessed by detoxification assay of doxorubicin, and the induction and volume of lamellar bodies. We found nine children with interstitial lung disease carrying a heterozygous R288K variant, a frequency significantly higher than in the general Caucasian population. All identified patients had neonatal respiratory insufficiency, recovered and developed chronic interstitial lung disease with intermittent exacerbations during early childhood. In vitro analysis showed normal transcription, processing, and targeting of ABCA3-R288K, but impaired detoxification function and smaller lamellar bodies. We propose that the R288K variant can underlie interstitial lung disease in childhood due to reduced function of ABCA3, demonstrated by decelerated detoxification of doxorubicin, reduced PC 32: 0 content and decreased lamellar body volume.
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