Reye Syndrome with Severe Hyperammonemia and a Good Neurological Outcome.

BACKGROUND Reye syndrome (RS) is a rare life-threatening condition combining acute noninflammatory encephalopathy and acute liver failure with an absence of defined etiology. We present a case of fulminant RS that had a good neurological outcome. CASE REPORT A 4-year-old previously healthy boy had no history of acetylsalicylic acid (ASA) use, nor had he been diagnosed with any inborn errors of metabolism. RS was preceded by a mild viral infection, possibly caused by human bocavirus, which has not been previously implicated in RS. He presented with a combination of a very high concentration of ammonia but only mildly elevated aminotransferases and mild hypoglycemia. Computed tomography (CT) of the head additionally showed diffuse cerebral edema with tentorial herniation. The extensive metabolic evaluation did not confirm any inborn errors of metabolism to explain the etiology. We provided optimal treatment of severe hyperammonemia (>500 µmol/L) and cerebral edema, including high doses of arginine chloride, sodium benzoate, hemodialysis, mild hypothermia, and supportive care. He has been followed up for over 4 years. The patient recovered completely, with no long-term psycho-cognitive or neurological sequelae. CONCLUSIONS Although extremely rare, hyperammonemia and RS should be considered in cases of an acute encephalopathy to be treated as soon and as decisively as possible to enable a good outcome.

Rezidivierende fulminante Myokarditis mit wiederholtem Einsatz der ECMO bei einem Kind.

Recurrent myocarditis is rare with only few reports having been published for paediatric cases. Repeated use of extracorporeal membrane oxygenation is also uncommon. In this paper we will present a very rare case of a 7-year old girl with recurrent fulminant myocarditis with heart failure requiring cardiopulmonary resuscitation and mechanical circulatory support with extracorporeal membrane oxygenation. Both episodes were precipitated by a viral upper respiratory tract infection, and in both cases the cardiac function eventually completely recovered. The second episode of fulminant myocarditis was particularly complex with markedly elevated markers of myocardiocytolysis, multiorgan dysfunction and the need for prolonged mechanical circulatory support. Nevertheless, the patient made a remarkable recovery. A comprehensive diagnostic workup pointed towards an aberrant immune response as the likely cause of the girl's susceptibility for fulminant myocarditis.

Passive therapeutic hypothermia during ambulance and helicopter secondary neonatal transport in neonates with hypoxic brain injury: a 10-year retrospective survey.

BACKGROUND: Therapeutic hypothermia is a method of treatment in newborns with hypoxic ischemic encephalopathy. Hypothermia should be initiated no later than 6 h after birth. The purpose of this study was to evaluate the quality of the passive therapeutic cooling during neonatal transport. PURPOSE: The study aims to evaluate the efficiency of our transport in maintenance of target body temperature during transport. METHODS: We conducted a 10-year retrospective study in neonates, transported by helicopter or ambulance, who received therapeutic passive-induced hypothermia during transport to the Department of Pediatric Surgery and Intensive Therapy at the University Medical Centre Ljubljana between September 1, 2006, and December 31, 2016. RESULTS: Out of 68 transported newborns, 57 met the criteria for therapeutic induced hypothermia. Eight out of 51 (15.7%) were within therapeutic temperature zone before start of transport while 30 out of 57 (52.6%) were within therapeutic temperature zone at the end of transport. There was a negative correlation between the duration of transport and temperature at the admission (ρ = - 0.306; p = 0.026). A positive correlation was found between the body temperature before and at the end of transport (ρ = 0.410; p = 0,003). A positive correlation between axillary and rectal temperature on admission was found (ρ = 0,832; p < 0,0005). The type of transport, meteorological season, or gender differences did not affect any of measured parameters. Newborns who received chest compression had lower temperature. CONCLUSION: Therapeutic temperature zone during transport was achieved in 52.6% of transported neonates. Axillary temperature positively correlated with rectal temperature on admission.

Two episodes of systemic capillary leak syndrome in an 8-year-old boy, following influenza A virus infection.

Systemic capillary leak syndrome is a rare condition, characterized by hypotension, edema, hemoconcentration and hypoalbuminemia. We describe 2 episodes of systemic capillary leak syndrome, following influenza A virus infection, occurring during 2 subsequent influenza seasons, in an 8-year-old boy.

Heart rate variability of transported critically ill neonates.

UNLABELLED: Determining heart rate variability (HRV) in infants is a useful measure of physiological stability. Transport of ill neonates imposes a measurable degree of stress. A prospective observational study on 58 critically ill neonates, transported to an intensive care unit (ICU) was performed. HRV during the 24-h period before, during and after transport, heart rate (HR), mean arterial pressure and transport risk index of physiologic stability (TRIPS) score were observed. The median HRV total power value of 40.80 ms(2) was set as the cutoff value, and neonates with values below this were designated as the low-HRV group (l-HRV; n=29), and those above this as the high-HRV group (h-HRV; n=29). The h-HRV group had a significantly lower HR at retrieval and 1 h after admission and a significant 2- and 4-day shorter duration of mechanical ventilation and ICU treatment compared to the l-HRV group. Spearman's correlations between total power and duration of mechanical ventilation (ρ=-0.346; P<0.01) and ICU treatment (ρ=-0.346; P<0.01) were significant. Transported neonates were also tested for differences in HRV and other physiological and demographic parameters between the transport mode and time. No differences were found, except that the nighttime ambulance group had a statistically higher HRV compared to the daytime ambulance group. CONCLUSION: Higher HRV of group of neonates, who did not differ in illness severity TRIPS score from the lower HRV group, is associated with a faster and significant decrease in HR after transport and a 2- and 4-day shorter duration of mechanical ventilation and ICU treatment.

Prolonged prostaglandin E1 therapy in a neonate with pulmonary atresia and ventricular septal defect and the development of antral foveolar hyperplasia and hypertrophic pyloric stenosis.

Prostaglandin E1 (alprostadil) is widely used for maintaining the patency of ductus arteriosus in ductus-dependent congenital heart defects in neonates to improve oxygenation. Among more common side effects are fever, rash, apnoea, diarrhoea, jitteriness, and flushing. More severe side effects are brown fat necrosis, cortical hyperostosis, and gastric outlet obstruction, most commonly the result of antral foveolar hyperplasia or hypertrophic pyloric stenosis. We report on an infant with a ductus-dependent congenital heart defect who developed symptoms and sonographic evidence of focal foveolar hyperplasia and hypertrophic pyloric stenosis after prolonged treatment with prostaglandin E1. Gastrointestinal symptoms persisted after corrective cardiac surgery, and pyloromyotomy was required. Study of the case and of available literature showed an association between the total dose of prostaglandin E1 administered and duration of treatment and the development of gastric outlet obstruction. We conclude that if patients are treated with a prostaglandin E1 infusion, careful monitoring for symptoms and signs of gastric outlet obstruction is required.

Continous monitoring of central venous oxygen saturation in children and infants.

Maintenance of adequate tissue oxygenation is an important task in intensive care units. There are many variables which are measured for this purpose. Central venous oxygen saturation (ScvO2) monitoring has some advantages over the mixed venous oxygen saturation (SvO2) monitoring in children and infants as there is no need to insert a pulmonary catheter. The clinical usefulness seems promising.

Heart rate and leukocytes after air and ground transportation in artificially ventilated neonates: a prospective observational study.

OBJECTIVE: To evaluate the effect of interhospital air and ground transportation of artificially ventilated neonates on heart rate and peripheral blood leukocyte counts. DESIGN: Prospective, observational study. SETTING: Level III multidisciplinary Neonatal and Pediatric Intensive Care Unit. PATIENTS: Fifty-eight near-term artificially ventilated transported neonates between May 2006 and April 2007. INTERVENTIONS: Day-helicopter, day- and night-ground transportation. MEASUREMENTS AND RESULTS: Heart rate at retrieval, on admission to the ICU and 1 h later, and peripheral blood leukocyte counts on admission and 1 d later were compared. Fifteen neonates were transported by helicopter during the daytime (D-HEL), 20 by daytime ground and 23 by nighttime ground transportation (D-GROUND, N-GROUND). No differences in delivery mode, birth weight, gestational age, gender, primary diagnoses for transportation, response time and duration of transportation were found between the groups. Similarly, no differences in pH, pCO(2), blood pressure and skin temperature at retrieval and on admission to the ICU were found between the three groups. The mean heart rate at retrieval did not differ significantly, while on arrival in the ICU and 1 h later the D-GROUND group of patients showed a significantly higher mean heart rate compared to the D-HEL and N-GROUND groups. Moreover, leukocyte counts on arrival in the ICU showed significantly higher leukocyte counts in the D-GROUND group of patients compared to the D-HEL group of patients. CONCLUSIONS: These results demonstrate that there is an association between daytime ground transportation and higher heart rate and peripheral blood leukocytes.