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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 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.

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 HF2O® 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, pConclusion 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.

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.

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.

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.

Toxic shock syndrome (TSS) is a rare complication of a Staphylococcus aureus infection and was first described in seven children by Todd [1]. Twenty per cent of the strains of S. aureus are capable of producing toxic shock syndrome toxin (TSST)-1, a superantigen (SAg), which causes the classic picture seen in TSS due to the over stimulation of T-cells [2]. Before the early 1980s, most cases occurred in young women with menstruation (MTSS) and tampon use. Non-menstrual (NMTSS) cases occur in cutaneous and subcutaneous infections, pharyngitis, vaginitis unrelated to menstruation and after surgical procedures (e.g. otolaryngologic). Frame et al. [3] first described TSS in children with burns in 1985. Children, who develop TSS during the first few days after the thermal injury usually only have small burns, which should be expected to heal uneventfully. The staphylococcal exotoxin-1 (TSST-1), which is implicated in the development of this disease, and the low levels or absence of anti TSST-1 antibodies may indicate susceptibility to this illness [4]. The majority of burn victims at our children’s burns unit, and elsewhere, are younger than four years. Most of them present with relatively small scalds.

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 volume10 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.

Background: Burning pain of red and warm hands and/or feet are the classical symptoms of erythromelalgia. Case report: We describe the symptoms of acute idiopathic erythromelagia and arterial hypertension in a five-year-old boy. Five days after a gastroenteritis the patient developed burning hands and feet in combination with arterial hypertension. Typically continuous cooling of all affected limbs was necessary to relieve the pain. Drug therapy with sodium nitroprusside only relieved the pain and dropped the blood pressure temporarily. Five weeks after onset of the disease all symptoms disappeared and the patient is still free of complaints (follow up period: 2 years).In the view of the presented case we discuss the differential diagnoses as well as therapeutical options. Hintergrund: Brennende Schmerzen geroteter und uberwarmter Handflachen und/oder Fussohlen stellen die charakteristischen Leitsymptome einer Erythromelalgie dar. Fallbericht: Wir beschreiben das Krankheitsbild der akuten idiopathischen Erythromelalgie und Hypertonie bei einem 5jahrigen Knaben. Der Patient entwickelte funf Tage nach einer Gastroenteritis brennende Schmerzen an Handflachen und Fussohlen kombiniert mit einer arteriellen Hypertonie. Typischerweise war eine permanente Kuhlung (Caldogelbeutel) aller vier Extremitaten als Schmerzlinderung erforderlich. In der medikamentosen Therapie fuhrte nur die Gabe von Nitroprussid-Natrium zu einer passageren Linderung der Schmerzen und Blutdrucksenkung. Funf Wochen nach Krankheitsbeginn kam es zur Ruckbildung aller Symptome. Der Patient ist seither beschwerdefrei (Nachbeobachtungszeit: 2 Jahre). Im Hinblick auf den beschriebenen Fall werden Differentialdiagnosen und Therapieoptionen diskutiert.

Objective: To investigate the effect of the combination of kinetic therapy (KT) with partial liquid ventilation (PLV) on gas exchange, lung mechanics and hemodynamics in acute lung injury (ALI). Design: Prospective, randomized, controlled pilot study. Setting: University research laboratory. Subjects: Eleven piglets weighing 8.3 ± 0.9 kg. Intervention: ALI was induced by the infusion of oleic acid (0.08 ml/kg) and repeated lung lavages with 0.9 % NaCl (20 ml kg−1). Thereafter the animals were randomly assigned either for PLV or a combination of PLV with KT (PLV/KT). The dose of perfluorocarbon administered was 30 ml/kg, evaporative losses were substituted with 5 ml/kg per h. Measurements and main results: Airway pressures, tidal volumes, dynamic compliance (Cdyn), expiratory airway resistance and arterial blood gases were measured. Hemodynamic monitoring included right atrial, mean pulmonary artery, pulmonary capillary wedge and mean systemic arterial pressures, and continuous flow recording of the pulmonary artery. In both groups the induction of ALI significantly reduced PaO2/FIO2 Cdyn and cardiac output, and significantly increased pulmonary artery pressure. After the initiation of PLV there was a significant increase of PaO2/FIO2, and Cdyn, and a significant decrease of pulmonary artery pressure in both groups. Except the PaCO2, which showed significantly lower values in the PLV/KT group, no variables showed any differences between the two groups. Conclusion: The additional use of KT did not show beneficial effects on oxygenation and lung mechanics during PLV. However, at constant minute ventilation PaCO2 levels were significantly lower during PLV/KT, indicating some positive influence on the ventilation/perfusion distribution within the lung. Extreme body positions during PLV/KT did not show any significant hemodynamic side effects.

Objective—To assess the use of detachable coils as an alternative method to occlude interatrial communications after Fontan operations. Design—Descriptive clinical study of selected patients after Fontan operation with interatrial communications inappropriate for transcatheter umbrella occlusion. Setting—Tertiary paediatric cardiac referral centre. Patients—Seven patients after Fontan operation with residual interatrial communications of various types producing a right to left shunt. Interventions—Transcatheter placement of detachable coils with a diameter of 3 or 5 mm within the interatrial communication. Results—A total of 14 coils were successfully placed within persistent patent fenestrations of the interatrial baffle, residual leaks at the suture line between the patch material and the right atrial wall, and unusual venous interatrial communications. The mean (SD) aortic oxygen saturation increased from 88 (1.1)% (range, 86-89%) to 92 (1.3)% (range, 89-93%; p

Objective: To look for the effects of inhaled nitric oxide on oxygenation and pulmonary hemodynamics during acute hypercapnia in acute respiratory failure. Design: Prospective, randomized, experimental study. Setting: University research laboratory. Subjects: Ten piglets, weighing 9 to 13 kg. Interventions: Acute respiratory failure was induced by oleic acid infusion and repeated lung lavages with 0.9% sodium chloride. The protocol consisted of three randomly assigned periods with different PaCO 2 levels. Tidal volume was reduced to induce hypercapnia. Inspiratory time was prolonged to achieve similar mean airway pressures. During permissive hypercapnia, pH was not corrected. At each PaCO 2 period, the animals were ventilated with inhaled nitric oxide of 10 parts per million and without nitric oxide inhalation. Measurements and Main Results: Continuous hemodynamic monitoring included right atrial, mean pulmonary arterial, and mean systemic arterial pressures, arterial and mixed venous oxygen saturations, and continuous flow recording at the pulmonary artery. In addition, airway pressures, tidal volumes, dynamic lung compliance and airway resistance, end-tidal CO 2 concentrations, and arterial and mixed venous blood gases were measured. Data were obtained at baseline and after lung injury, at normocapnia, at two levels of hypercapnia with and without nitric oxide inhalation. Acute hypercapnia resulted in a significant decrease in blood pH and a significant increase in mean pulmonary arterial pressure. There was no significant change in PaO 2 during normocapnia and hypercapnia. Inhaled nitric oxide significantly decreased the mean pulmonary arterial pressure during both hypercapnic periods. It significantly improved oxygenation during both normocapnia and hypercapnia. Conclusions: Acute hypercapnia resulted in a significant increase in pulmonary arterial pressure without influencing oxygenation and cardiac output. Inhaled nitric oxide significantly reduced the pulmonary hypertension induced by acute permissive hypercapnia but did not influence the flow through the pulmonary artery. Inhaled nitric oxide significantly improved oxygenation in this model of acute lung injury during normocapnia and acute hypercapnia.

The purpose of this experimental study was to compare heparin-coated versus non-coated systems for extracorporeal membrane oxygenation (ECMO), to investigate the dynamic course of clotting activation in both groups. Methods. Eight pigs weighing 19.7 (± 1.3) kg, each underwent ECMO for 24 hours. Two groups were formed: in group 1, heparin-coated circuits were used with low dose heparinization (10 IU/kg/hr), whereas in group 2 non-coated circuits with high dose heparinization (60 IU/kg/hr) were used. Coagulation was monitored by measuring prothrombin time, partial thromboplastin time, fibrinogen, antithrombin III (AT III) and specific markers of clotting activation (thrombin-antithrombin III complexes (TAT) and D-dimer). Furthermore, platelet count, hematocrit, activated clotting time (ACT), and plasma heparin concentration were determined regularly. Results. The dynamic course of the specific coagulation activation markers showed some differences: whereas TAT and D-dimer increased quickly in group 2, the increase in group 1 was delayed. Activation marker values tended to be lower in group 1 during the first six hours, after which no more differences between the groups were seen. After 24 hours of ECMO, TAT and D-dimer had nearly returned to baseline values. Platelets showed a continuous decrease throughout the experiment, which was very similar in both groups. Conclusions. The heparin coated system showed a distinct delay in clotting activation during the first six hours of ECMO. After six hours there were no more differences between the groups.

The paediatric patient we are describing suffered a scald injury covering 83 per cent of the total body surface area (TBSA). This injury was complicated by Klebsiella pneumoniae septicaemia resulting in multiorgan failure (MOF). Acute respiratory distress syndrome (ARDS), gastrointestinal insufficiency, hepathopathy and wound conversion to full thickness posed the main problems. The boy was ventilated with pressure-controlled mechanical ventilation. The concept of permissive hypercapnia (PHC) resulted in a complete resolution of ARDS within 4 weeks. From our experience, further lung injury among infants and children suffering from severe ARDS can be avoided by using controlled mechanical hypoventilation. It is a simple and safe technique that allows adequate oxygenation.

This study was a prospective, randomized, controlled design to evaluate gas exchange, lung mechanics, and pulmonary hemodynamics during partial liquid ventilation (PLV) combined with inhaled nitric oxide (NO) in acute respiratory failure (ARF) with pulmonary hypertension (PH). ARF with PH was induced in 12 piglets weighing 9.7-13.7 kg by repeated lung lavages and the continuous infusion of the stable endoperoxane analog of thromboxane. Thereafter the animals were randomly assigned either for PLV or conventional mechanical ventilation (CMV) at a fractional concentration of inspired O2 (Fio2) of 1.0. Perfluorocarbon (PFC) liquid (30 mL kg-1) was instilled into the endotracheal tube over 5 min followed by 5 mL kg-1h-1. All animals were treated with different concentrations of NO (1-10-20 ppm) inhaled in random order. Continuous monitoring included ECG, right atrial (Pra), mean pulmonary artery (Ppa), pulmonary capillary (Ppc'), and mean arterial (Pa) pressures, arteria oxygen saturation, and mixed venous oxygen saturation measurements. During PLV Pao2/Fio2 increased significantly from 8.2 +/- 0.4 kPa to 34.8 +/- 5.1 kPa (p0.01), whereas Pao2/FiO2 remained constant at 9.5 +/- 0.4 kPa during CMV. The infusion of the endoperoxane analog resulted in a sudden decrease of Pao2/Fio2 from 34.8 +/- 5.1 kPa to 14.1 +/- 0.4 kPa (p0.01) in the PLV group and from 9.5 +/- 0.4 kPa to 6.9 +/- 0.2 kPa (p0.05) in the control group. Inhaled NO significantly improved oxygenation in both groups (Pao2/Fio2: 45.7 +/- 5.3 kPa during PLV and 25.9 +/- 4.7 kPa during CMV). During inhalation of NO mean Ppa decreased significantly from 7.8 +/- 0.26 kPa to 4.2 +/- 0.26 kPa (p0.01) in the PLV group and from 7.4 +/- 0.26 kPa to 5.1 +/- 0.13 kPa (p0.01) in the control group. As documented in the literature PLV significantly improves oxygenation and lung mechanics in severe ARF. In addition, when ARF is associated with severe PH, the combined treatment of PLV and inhaled NO improves pulmonary hemodynamics resulting in better oxygenation.

1. Oxygen-resistant cyanosis in the absence of cardiorespiratory causes indicates methemoglobinemia. This patient developed methemoglobinemia as a consequence of rasburicase therapy in the setting of glucose-6phosphate dehydrogenase (G6PD) deficiency. 2. A blood gas analyzer can reliably measure the hemoglobin (Hb) subfractions in an arterial blood sample, including methemoglobin (metHb), with the spectrophotometric method. G6PD deficiency can be diagnosed biochemically or by molecular analysis of the G6PD gene. 3. Patients of African, Asian, Mediterranean and Middle Eastern descent, as well as patients with a history and/or family history of unexplained cyanosis, hemolytic anemia, or unexplained jaundice are particularly at risk for G6PD deficiency and should be screened for this enzyme deficiency prior to rasburicase administration. Commentary

OBJECTIVE: To determine the degree of clotting activation that occurs with the usual anticoagulation regimen with systemic heparinization. METHODS: To allow a standardized comparison of the patients, this study focused on the first 48 hours of extracorporeal membrane oxygenation (ECMO) in term newborn infants. The ECMO perfusion circuit consisted of a roller pump, silicone membrane lungs, and silicone rubber tubing. Coagulation was controlled routinely by measuring prothrombin time, fibrinogen, antithrombin III, and reptilase time. Platelet counts, activated clotting time, and heparin concentration were controlled regularly. The following specific activation markers of the clotting system were measured: prothrombin activation fragment 1 + 2 (F 1 + 2 ), thrombin-antithrombin III complexes, and D -dimer. Measurements were done before the start of ECMO, after 5 minutes, and at hours 1, 2, 3, 4, 6, 12, 24 and 48. RESULTS: All seven term infants had excessively high levels of clotting activation markers within the first 2 hours of ECMO: F l + 2 , 11.6 (±0.9) nmol/L (mean ± SEM); thrombin-antithrombin, 920 (±2.2) μg/L; D -dimer, 15.522 (±3.689) ng/L. During the next 46 hours of ECMO, F l + 2 and thrombin-antithrombin III complexes decreased from those high values, whereas D -dimer did not. The increase of activation markers was accompanied by low fibrinogen, low platelet counts. and prolongation of reptilase time. CONCLUSIONS: These findings fit the pattern of consumptive coagulopathy during neonatal ECMO, especially in the first 24 hours. (J P EDIATR 1996;129:264-8)

Fulminant meningococcemia defines a life-threatening disease with acute onset, severe septic shock, and progressive hemorrhagic necrosis of the skin. Despite advances in intensive care, the case fatality rate of this disease is still between 30% and 50%.1-5 Disseminated intravascular coagulation (DIC) with deposition of fibrin and histologically demonstrable widespread microvascular thromboses contributes significantly to the pathogenesis.6-9 Impairment of fibrinolysis caused by elevation of plasminogen activator inhibitor 1 (PAI-1), the physiologic inhibitor of tissue plasminogen activator, is part of these clotting abnormalities and has prognostic significance; the extent of elevation of PAI-1 is correlated to the development of shock, renal impairment, and mortality.10,11

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.

The objective of this investigation was to compare different scoring systems to assess the severity of illness in infants with bronchiolitis admitted to a tertiary paediatric intensive care unit (PICU). Over an 18-year period (1990–2007), infants with bronchiolitis aged up to 12 months and admitted to the PICU were prospectively scored using the Pediatric Risk of Mortality III (PRISM III) score, the Organ System Failure (OSF) score and the Acute Physiologic Score for Children (APSC) within 24 h. Infants were compared as to whether or not bronchiolitis was associated with respiratory syncytial virus (RSV). There was no difference between 113 RSV-positive and 80 RSV-negative infants regarding gestational age, birth weight, rate of premature delivery or bronchopulmonary dysplasia (BPD). The PRISM III score differed significantly between RSV-positive and RSV-negative cases (3.27 ± 0.39 vs. 1.96 ± 0.44, p = 0.006), as did the OSF score (0.56 ± 0.05 vs. 0.35 ± 0.06, p = 0.049) and the APSC (5.16 ± 0.46 vs. 4.1 ± 0.53, p = 0.048). All scores were significantly higher in the subgroup with mechanical ventilation (p

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.

Background The postoperative course of infants and children after open heart surgery is often complicated by cardiopulmonary insufficiency or low cardiac output. Methods From January 1989 to April 1992 441 infants and children with congenital heart disease underwent cardiac surgery. 128 of these patients (29%) required prolonged or extensive intensive care because of cardiopulmonary insufficiency or low cardiac output. Aortic cross clamp and cardiopulmonary bypass times were measured in all patients. In the postoperative period duration of mechanical ventilation, duration of intensive care, special monitoring and therapeutic strategies and clinical scores were documented. Results The overall mortality rate was 9.9%, the mortality rate in patients with postoperative cardiopulmonary insufficiency or low cardiac output was 34%. The mortality rate increased significantly up to 73% when the cardiopulmonary bypass time exceeded 200 min. Mean duration of intensive care of survivors (S) and nonsurvivors (NS) was 10.3±0.8 and 4.1±1.2 days, respectively (p

Introduction: Childhood stroke has an estimated incidence of about 10:100000 per year. Nevertheless stroke is one of the 10 most common causes for death in childhood. We were interested in the mean diagnostic delay and presenting symptoms of pediatric patients referred to our University Hospital. Method: We retrospectively analyzed the history of 16 patients (9 girls, 7 boys) with childhood stroke. Age at presentation varied from 2 months to 17.8 years. Initial symptoms were unspecific with vomiting in 4, collaps 2, headache1 and followed by specific signs of hemiparesis 12, aphasia 3, central facial palsy 2, seizure2, nystagmus 1, vertigo 1. Time delay from onset of symptoms to diagnosis by MRI ranged from 2h to 18 days (mean 58.7h±116). All patients were imaged by a 1.5 Tesla MRI, only 1 child had a CT due to orthodontic therapy. With respect to the vascular territory 9 had ischemic stroke of the medial cerebral artery, 4 of the posterior cerebral artery, 2 of the cerebellar artery. 1 had cerebral sinus venous thrombosis (CSVT). As underlying etiology we identified: heart disease 4, dissection 2, chemotherapy with lung cancer 1, chronic inflammatory bowel disease 1, otitis media 1 (CSVT), post varicella zoster vasculitis 1. Screening for thrombophilia revealed homozygosity for MTHFR(677T) in 2, heterozygosity in 3, positive lupus inhibitor in 2. Conclusions: As illustrated by this cohort the diagnosis of childhood stroke is still associated with marked diagnostic delay despite specific and acute neurologic deficits. Though in childhood stroke the option of lysis is only considered on an individual basis, the critical time window is often elapsed before a diagnosis was made. This illustrates that broader general awareness and logistic guidelines are needed to improve medical care of childhood stroke in pediatric centers.

SummarySevere stroke in children and adolescents with its devastating long term consequences remains a rare disorder with many open questions. Beside the well known risk factors such as infection or congenital and acquired heart disease, coagulation disorders have to be considered in the differential work up of the underlying aetiology. Here we report the case of an adolescent with a homozygote MTHFR 677CT mutation suffering a malignant stroke shortly after the start of oral contraceptives. Conclusion: Since prevention seems easily feasible, general screening for MTHFR mutations might be worthwhile in women before starting oral contraceptives.

To compare the effects of two different antibiotic regimes on the colonization and infection rates of critically ill pediatric patients.A prospective randomized trial.A pediatric ICU in a university hospital.Fifty critically ill pediatric patients who required intensive care for at least 4 days were randomly allocated to either the selective parenteral and enteral antisepsis regimen (treatment group, n = 25) or the control group (n = 25).The treatment group received oral nonabsorbable antimicrobial agents (polymyxin E, gentamicin, and amphotericin B) and parenteral cefotaxime, whereas the control group received either perioperative antibiotic prophylaxis or antibiotic therapy according to clinical or microbiological evidence of infection.Both groups were comparable for age, body weight, sex, and severity of illness. Colonization with Gram-negative microorganisms and yeasts in the oropharynx, and digestive and respiratory tracts increased rapidly up to 52% in the control group, whereas there was no colonization with these microorganisms in the treatment group. The occurrence rates of acquired secondary infections in the control and treatment groups were 36% and 8%, respectively (p less than .025). There were no differences between groups in the duration of intensive care or mortality rate.Selective oropharyngeal and gastrointestinal decontamination combined with systemic cefotaxime application allows for a significant reduction of the colonization rate with Gram-negative bacteria and yeasts in critically ill pediatric patients undergoing prolonged intensive care. In addition, it significantly reduces the Gram-negative infection rate of the respiratory system. However, this therapeutic approach does not alter ICU length of stay or mortality rate.

Eleven hypervolemic and oliguric children with low cardiac output after cardiac operations were treated by slow continuous ultrafiltration or continuous arteriovenous hemofiltration. A mean negative fluid balance of 1.63 +/- 0.37 ml/kg/hr (standard error of the mean [SEM]) significantly improved the hemodynamic status within 59 +/- 6.1 hours (SEM). Although the central venous pressure decreased significantly from 15.2 +/- 0.84 to 8.8 +/- 0.92 mm Hg (p less than 0.0001), the mean arterial pressure increased significantly from 41.5 +/- 2.54 to 53.5 +/- 2.21 mm Hg (p less than 0.001). In addition, pH increased significantly from 7.31 +/- 0.01 (SEM) to 7.43 +/- 0.001 (SEM) (p less than 0.001) and oxygenation index (arterial oxygen tension/inspired oxygen fraction) from 119 +/- 15.2 (SEM) to 214 +/- 27.0 (SEM) (p less than 0.001). Hemodynamic improvement during slow continuous extracorporeal fluid removal allowed a significant decrease of the catecholamine infusion rate. After normovolemia had been achieved, continuous arteriovenous hemofiltration had to be continued in four children because of persistent anuria. Eight patients could be weaned from artificial ventilation and vasopressor support. Two patients died without recovery of renal function and one with restored renal function. Slow continuous ultrafiltration and continuous arteriovenous hemofiltration improve the cardiovascular function in children with low cardiac output by optimizing the preload conditions of the failing heart. In addition, they improve acid-base balance and pulmonary gas exchange.

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.

Ten pigs with experimental respiratory distress syndrome were treated by extracorporeal CO2 removal (ECCO2-R) combined with low frequency positive pressure ventilation (LPPV). After lung damage had been induced by repeated lung lavages a PEEP trial was conducted in order to find the appropriate PEEP for the damaged lungs. This PEEP was then applied during the ECCO2-R/LPPV period. Blood gas values improved significantly on extracorporeal bypass within a short time (pre-bypass paO2: 54.2 ± 3.7 vs 168.5 ± 31.6 mmHg after 15 min on bypass, p < 0.001) and were kept constant during the next 4 hours. Minute ventilation (MV) was reduced from 4.01 ± 0.31 to 0.74 ± 0.07 l/min (p < 0.0001), FiO2 of the ventilator from 1.0 to 0.46 ± 0.08 (p < 0.0001) whereas FiO2 of the membrane lung (ML) was not changed significantly (FIO2ML 0.59 ± 0.07 vs 0.53 ± 0.06). During controlled mechanical ventilation (CMV), comparable adequate gas exchange was only achieved at a significantly higher mean airway pressure ([Formula: see text] vs 21.2 ± 0.47 cmH20, p < 0.0001). Hemodynamic variables did not change significantly during bypass time. ECCO2-R/LPPV driven by a simple renal perfusion system allows adequate gas exchange in experimental respiratory failure.

Acute renal failure (ARF) is defined as the cessation of renal function with or without changes in urinary output. The incidence of ARF in paediatric intensive care units is highly variable, ranging from 2% to 8%, and is often associated with severe medical or surgical illness [1]. Whereas patients with intrinsic renal abnormalities as the basis for their renal failure have mortality rates below 10%, patients with secondary ARF have mortality rates higher than 50% [2].

Einleitung: „Respiratory syncytial virus“ (RSV) ist eines der haufigsten Erreger von Viruserkrankungen der fruhen Kindheit. Es verursacht die meisten Bronchiolitisfalle, was mit dem vermehrten Auftreten von obstruktiven Bronchitiden und kindlichem Asthma in der spateren Kindheit verknupft ist. Wahrend der Primarinfektion tritt die Phase mit den ausgepragtesten klinischen Symptomen nicht wahrend des Gipfels der Virusreplikation, sondern wahrend der Entwicklung einer spezifischen T- und B-Zellantwort auf. Die Immunreaktion des Organismus ist verbunden mit der zellularen Infiltration des befallenen Gewebes und der Ausschuttung von Entzundungsmediatoren. Methoden: Die Proben wurden aus dem peripheren Blut von insgesamt 34 Sauglingen (davon 8 Kinder mit RSV-Bronchiolitis im Alter von 14 bis 115 Tagen und 6 infektfreie Kinder im Alter von 6 bis 291 Tagen sowie 10 Neugeborene mit „Early-onset“ Sepsis und 10 gesunde Neonaten am 1. Lebenstag) entnommen. Die Gewinnung der Blutproben erfolgte wahrend Routine-untersuchungen, nachdem das Einverstandnis mindestens eines Elternteils dazu erteilt worden war. Die Zellen wurden mittels Ammoniumchlorid-mediierter Lyse prapariert, mit den entsprechenden Antikorpern (Becton Dickinson, Franklin Lakes, NJ USA) inkubiert und mittels FACSCalibur (Becton Dickinson) entsprechend den Standardbedingungen analysiert. Ergebnisse: Wir bestimmten die CD40-Expression von CD19+ B-Lymphozyten. Bei Sauglingen mit Bronchiolitis fand sich ein Mittelwert von 876 CD40+ B-Zellen (Maximum 1351, Minimum 183), wahrend der Mittelwert bei infektfreien Kindern bei 201 CD40+ Lymphozyten lag (Maximum 382, Minimum 13). Bei Neonaten mit „Early-onset“ Sepsis fand sich ein Mittelwert von 184 CD40+ Zellen (Maximum 483, Minimum 66), bei gesunden Neugeborenen lag der Mittelwert bei 509 positiven Zellen (Maximum 1446, Minimum 158). Hinsichtlich der CD40-Expression zeigte ein Vergleich der vier Gruppen eine Korrelation einerseits zwischen Kindern mit Bronchiolitis versus gesunden Kindern (p 0,0064), andererseits zwischen Sauglingen mit Bronchiolitis und Neonaten mit „Early-onset“ Sepsis (p 0,0005). Eine Regressionsanalyse ergab eine Korrelation zwischen CD40-Expression von Kindern mit Bronchiolitis und gemessenem CRP-Wert. Schlussfolgerung: Bei Vorliegen einer Bronchiolitis kam es zu einer „Upregulation“ der CD40-Expression von B-Lymphozyten als Ausdruck der B-T-Zellinteraktion im Rahmen einer Infektion. Dies zeigte sich im Rahmen einer Korrelation zwischen CD40-Expression von erkrankten versus gesunden Kindern. Daruberhinaus korrelierte das Ausmas der CD40-Expression mit dem jeweiligen CRP-Wert. Hingegen blieb eine Upregulation der CD40-Expression bei „Early-Onset“ Sepsis wohl als Ausdruck der in der Fetalperiode fur den Erhalt einer Schwangerschaft erforderlichen reduzierten Immunantwort aus.