Neonatal Extracorporeal Membrane Oxygenation Due to Respiratory Failure: A Single Center Experience Over 28 Years.

Background: ECMO therapy is worldwide declining in the neonatal population; hence, its therapeutic value is sometimes questioned. Objectives: To report our experience with neonatal ECMO due to respiratory failure over a 28 year time period. Methods: Retrospective single center observational study including all neonates admitted to ECMO due to respiratory failure between 1989 and 2016 at Graz, Austria. Data were collected regarding survival rate, duration of ECMO, complications, length of hospital stay, changes over time, and follow-up. Results: Sixty-seven neonates were admitted and 43 (64%) needed ECMO-median birth weight 3390 grams (range 1810-4150) and gestational age 39 weeks (32-43). Survival rate was 65% (28/43); with higher rates in meconium aspiration syndrome (MAS) 89% vs. congenital diaphragmatic hernia (CDH) 46% and septic shock 44% (p = 0.005 and p = 0.006, respectively). ECMO duration was median 5 days (1-30) and veno-arterial ECMO (52%) dominated. Need for ECMO therapy decreased over time (p < 0.001). Complications occurred in 31 (72%) neonates. Five neonates had cerebral hemorrhages (11.4%) and four had cerebral infarction (9.1%). Of 26 survivors 17 (65%) showed normal neurodevelopmental outcome at median follow-up of 73 months. Motor deficits were present in one case, cognitive deficits in 9 (35%). Median length of hospital stay was 78 days in those with deficits and 29 in those with normal neurodevelopmental outcome (p < 0.001). Conclusions: Survival rate did not change over the study time but indications for ECMO did. Cognitive impairment was the major long-term deficit following neonatal ECMO being associated with longer hospital stay.

Effect of body position on ventilation distribution during PEEP titration in a porcine model of acute lung injury using advanced respiratory monitoring and electrical impedance tomography.

BACKGROUND: Lung failure after acute lung injury remains a challenge in different clinical settings. Various interventions for restoration of gas exchange have been investigated. Recruitment of collapsed alveoli by positive end expiratory pressure (PEEP) titration and optimization of ventilation-perfusion ratio by prone positioning have been extensively described in animal and clinical trials. This animal study was conducted to investigate the effects of PEEP and positioning by means of advanced respiratory monitoring including gas exchange, respiratory mechanics, volumetric capnography and electrical impedance tomography. METHODS: After induction of acute lung injury by oleic acid and lung lavage, 12 domestic pigs were studied in randomly assigned supine or prone position during a PEEP titration trial with maximal PEEP of 30 mbar. RESULTS: Induction of lung injury resulted in significant deterioration of oxygenation [partial pressure of arterial oxygen/inspiratory fraction of oxygen (PaO2/FiO2): p = 0.002] and ventilation [partial pressure of arterial carbon dioxide (PaCO2): p = 0.002] and elevated alveolar dead-space ratios (Valv/Vte: p = 0.003) in both groups. Differences in the prone and the supine group were significant for PaCO2 at incremental PEEP 10 and 20 and at decremental PEEP 20 (20d) and 10 (10d), for PaO2/FiO2 at PEEP 10 and 10d and for alveolar dead space at PEEP 10d. Electrical impedance tomography revealed homogenous ventilation distribution in prone position during PEEP 20, 30 and 20d. CONCLUSIONS: Prone position leads to improved oxygenation and ventilation parameters in a lung injury model. Respiratory monitoring with measurement of alveolar dead space and electrical impedance tomography may visualize optimized ventilation in a PEEP titration trial.

Hemodiafiltration in infants with complications during peritoneal dialysis.

End-stage renal disease (ESRD) in neonates still has a high mortality, particularly in the first year of life. We present the combination of peritoneal dialysis (PD) with intermittent hemodiafiltration (iHDF) in neonates with ESRD. Four infants younger than 28 days were treated with PD and iHDF. Renal diagnoses leading to ESRD were cortical necrosis, prune belly syndrome, neonatal hemolytic uremic syndrome, and autosomal recessive polycystic kidney disease. Initially, three patients were on iHDF until PD was started. At the time when complications occurred during PD, patients were switched back to iHDF. iHDF was used five times as a bridge to PD in case of abdominal surgery. Two of the four patients were switched to iHDF because of peritoneal ultrafiltration failure due to recurrent peritoneal leaks. Once, iHDF became necessary due to refractory peritonitis. All four patients survived the first year of life. Two patients were transplanted successfully at an age of 35 and 22 months, respectively. The others are on renal replacement therapy, one on PD at the age of 28 months and one on iHDF at the age of 25 months, respectively. In case of PD complications, iHDF may be an appropriate bridge to achieve long-term survival until kidney transplantation.

Continuous renal replacement therapy with Prismaflex HF20 disposable set in children from 4 to 15 kg.

Continuous venovenous hemodiafiltration (CVVHDF) in infants is challenging due to a lack of specific and widely available technology. There is need for high precision of fluid balance and hemodynamic stability. The aim of this study is to report the first experience with the newly developed Prismaflex HF20 disposable set (HF20 set) in infants with CVVHDF. We provided safe treatment in four infants (4-14 kg) suffering from acute renal failure with the HF20 set. Treatment monitoring included patients' blood pressure, patients' weight change, and fluid balance. The anticoagulation was performed with unfractionated heparin. We used commercially available bicarbonate-buffered solutions as dialysate fluid and substitution fluid. At start and during the treatment, the patients showed hemodynamic stability. Desired fluid balance was achieved in all patients during the treatment periods of 49-102 hours. No complications occurred. This report presents CVVHDF experience on the feasibility and safety of the HF20 set in infants. It can be used in CVVHDF or hemodialysis mode. Treatments were well tolerated in all patients, and flow rate adaptability to infants' needs was very acceptable, and the usage of this device was easy and safe.

Differentiating inherited human herpesvirus type 6 genome from primary human herpesvirus type 6 infection by means of dried blood spot from the newborn screening card.

To differentiate active human herpesvirus type 6 (HHV-6) infection from inherited HHV-6 (iHHV-6), we analyzed dried blood spots from archived newborn screening cards in 3 patients with high HHV-6 DNA copy numbers. Two patients were positive for HHV-6 DNA as neonates suggesting iHHV-6. In 1 patient, the absence of HHV-6 DNA excluded iHHV-6.

First experience with the Prismaflex HF 20 set in four infants.

PURPOSE: Renal replacement therapy (RRT) in infants is challenging due to a lack of widely available technology that is specific to this patient population. We present our initial experience with the newly developed Prismaflex HF20 disposable set used on the Prismaflex device in infants with renal failure. PATIENTS: Four infants, age 5 to 24 months, were enrolled. Overall 120 treatment sessions were performed over 300 patient-days. Treatment monitoring included patient weight change and fluid balance, treatment efficacy, number of interventions, and alarms. RESULTS: Desired fluid balance according to the prescribed weight loss was achieved in all patients (R²=0.86, p<0.0001). Treatment efficacy was monitored by blood urea nitrogen (BUN) and serum creatinine values at the start of RRT (59 ± 17 mg/dL and 5.1 ± 1.1 mg/dL) and their decrease after 4 hours of RRT (23 ± 7 mg/dL and 2.2 ± 0.6 mg/dL). Measured urea and creatinine clearances for the HF20 filter were 23 ± 7 ml/min and 19 ± 4 ml/min, respectively. No complications occurred. CONCLUSION: This is the first report on the use of the Prismaflex HF20 set in infants. No adverse events were observed, treatments were well tolerated in all patients, and flow rate adaptability to infants' needs was good.

Severe epstein-barr virus encephalitis with hemophagocytic syndrome: rapid clearance of virus following allogeneic hematopoietic stem cell transplantation from a seropositive donor.

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with encephalitis is a life-threatening condition that requires treatment with immunochemotherapy; refractory patients are eligible for allogeneic hematopoietic stem cell transplantation. We report on an adolescent female who failed to respond to induction immunochemotherapy and was salvaged by allogeneic hematopoietic stem cell transplantation from her human leukocyte antigen-identical, Epstein-Barr virus-seropositive brother leading to rapid clearance of Epstein-Barr virus from blood and cerebrospinal fluid.

Transcatheter closure of an atrial septal defect in a newborn with aortic stenosis.

AIM: A newborn with valvular aortic stenosis and a 5 mm atrial septal defect (ASD) underwent emergency aortic valvotomy. The small left ventricle of the newborn with signs of fibroelastosis showed good function but a reduced compliance and caused a large left to right shunt at atrial level. METHODS AND RESULTS: As the patient became respirator-dependant the ASD was interventionally closed with an 18 mm Amplatzer PFO occluder. The patient could be weaned from the ventilator within two days and had an uneventful recovery. CONCLUSION: Interventional closure of an ASD is possible even in the newborn period and should be considered as an alternative to surgery.

Use of Suprathel dressing in a young infant with TEN.

Toxic epidermal necrolysis and Stevens-Johnson syndrome are potentially life-threatening skin disorders. We report that a 3-month-old infant, a patient with toxic epidermal necrolysis, who in addition to a standard resuscitation protocol for burns received treatment with Suprathel (PolyMedics Innovations GmbH, Filderstadt, Germany) and fatty gauze as topical wound dressings in the form of a whole body cover with complete recovery. This is the first case report of Suprathel being used successfully in a baby with toxic epidermal necrolysis.

Aortic coarctation associated with alveolar capillary dysplasia and misalignment of the pulmonary veins.

After surgical repair of an aortic coarctation a term infant presented with severe pulmonary hypertension and cyanosis unresponsive to treatment including extracorporeal membrane oxygenation. The atypical clinical course became apparent once the accompanying diagnosis of congenital alveolar capillary dysplasia with misalignment of the pulmonary veins had been established at autopsy. In infants with congenital heart defects and with refractory pulmonary hypertension unexplainable on anatomic findings, a lung biopsy at the time of cardiac repair should be considered to avoid further therapies that would not alter the uniformly fatal course of this rare lung disorder.

Two decades' experience of renal replacement therapy in paediatric patients with acute renal failure.

During the past 20 years, childhood renal replacement therapy (RRT) and the treatment of underlying diseases experienced extensive advances. We reviewed the data of our critically ill patients with acute renal failure (ARF) and RRT, comparing two decades from 1985 to 1994 and from 1995 to 2004. There were 87 patients with a mortality rate of 45% in the first decade, decreasing to 28 patients with a mortality rate of 39% in the second decade. The mortality rate decreased from 51% to 20% in patients older than one year, while the mortality rate in patients younger than one year increased from 38% to 88%. Yet, the absolute number of these non-survivors younger than one year decreased from 16 to seven patients. The decrease of RRT was mainly caused by a decrease of ARF secondary to heart surgery, oncologic disorders and sepsis. Whereas the majority of patients (75%) were treated with continuous haemofiltration in the first decade, 75% of patients were treated with continuous haemodiafiltration in the second decade. In conclusion, advances in the diagnosis and treatment of underlying disorders have reduced the need for RRT in critically ill paediatric patients during the past 20 years. In addition, there was a tendency for a decrease in the overall mortality, which might be caused by changing treatment policies and advances in RRT technology. Nevertheless, the high mortality rate in small infants is challenging.

Partial liquid ventilation combined with two different gas ventilatory strategies in acute lung injury in piglets: Effects on gas exchange, respiratory mechanics, and hemodynamics.

BACKGROUND/PURPOSE: Partial liquid ventilation (PLV) has been shown to improve oxygenation and lung mechanics in different models of acute lung injury. This study was designed to investigate the effects of 2 gas ventilatory strategies during PLV on gas exchange, respiratory mechanics, and hemodynamics in acute lung injury in piglets. METHODS: After induction of acute lung injury, the animals were assigned randomly to 2 groups with different positive end-expiratory pressure (PEEP) levels and tidal volumes (Vt) (group A, Vt > 12.5 mL/kg; PEEP = 6 cm H2O, n = 7; group B, Vt < 9 mL/kg, PEEP = 12 cm H2O, n = 7). Thereafter, the perfluorocarbon (PFC) liquid (30 mL/kg) was instilled into the endotracheal tube. Cardiorespiratory parameters were measured at baseline, after induction of acute lung injury, and every 30 minutes up to 120 minutes. RESULTS: During PLV, oxygenation significantly improved with no difference between both gas ventilatory strategies. The high PEEP-moderate Vt gas ventilatory strategy reduced the inspiratory airway resistance and was associated with moderate hypercapnia. There were no significant differences in hemodynamics and respiratory compliance between both gas ventilatory strategies. CONCLUSIONS: The results of this pilot study suggest that oxygenation was equally improved during PLV. This effect was independent of the mode of gas ventilation. However, the high PEEP-moderate Vt gas ventilatory technique resulted in moderate hypercapnia.

Partial liquid ventilation versus conventional mechanical ventilation with high PEEP and moderate tidal volume in acute respiratory failure in piglets.

This prospective randomized pilot study aimed to test the hypotheses that partial liquid ventilation combined with a high positive end-expiratory pressure (PEEP) and a moderate tidal volume results in improved gas exchange and lung mechanics without negative hemodynamic influences compared with conventional mechanical ventilation in acute lung injury in piglets. Acute lung injury was induced in 12 piglets weighing 9.0 +/- 2.4 kg by repeated i.v. injections of oleic acid and repeated lung lavages. Thereafter, the animals were randomly assigned either to partial liquid ventilation (n = 6) or conventional mechanical ventilation (n = 6) at a fractional concentration of inspired O(2) of 1.0, a PEEP of 1.2 kPa, a tidal volume < 10 mL/kg body weight (bw), a respiratory rate of 24 breaths/min, and an inspiratory/expiratory ratio of 1:2. Perfluorocarbon liquid 30 mL/kg bw was instilled into the endotracheal tube over 10 min followed by 5 mL/kg bw/h. Continuous monitoring included ECG, mean right atrial, pulmonary artery, pulmonary capillary, and arterial pressures, arterial blood gas, and partial pressure of end-tidal CO(2) measurements. When compared with control animals, partial liquid ventilation resulted in significantly better oxygenation with improved cardiac output and oxygen delivery. Dead space ventilation appeared to be lower during partial liquid ventilation compared with conventional mechanical ventilation. No significant differences were observed in airway pressures, pulmonary compliance, and airway resistance between both groups. The results of this pilot study suggest that partial liquid ventilation combined with high PEEP and moderate tidal volume improves oxygenation, dead space ventilation, cardiac output, and oxygen delivery compared with conventional mechanical ventilation in acute lung injury in piglets but has no significant influence on lung mechanics.