[Acute care of critically ill children in general hospitals: organisation and training]. / Acute opvang van kinderen in algemene ziekenhuizen.

OBJECTIVE: To gain insight into how the acute care of critically ill children at general hospitals is organised, whether staff is sufficiently trained and whether the necessary materials and medications are present. DESIGN: Questionnaire combined with a site visit. METHOD: Questionnaires were sent to all primarily involved specialists (emergency room specialists and paediatricians), and to the auxiliary anaesthetists and intensivists involved, at the nine general hospitals in Southeast Netherlands. Two researchers performed standardised interviews with the lead paediatricians on site and checked for materials and medication present in the emergency and paediatric departments. RESULTS: Of the 195 questionnaires sent, 97 (49.7%) were deemed suitable for analysis. The response from the primary specialists involved (77.6%) was more than twice that of the auxiliary specialists (31.9%). At 7 hospitals, verbal agreements on the organisation of acute care were maintained, 1 hospital had a written protocol, and 2 hospitals had a task force addressing this topic. One out of 5 respondents was unaware of the verbal agreements and 1 out of 3 mistakenly assumed that a protocol existed. Two out of 3 primary specialists involved were certified for Advanced Paediatric Life Support (APLS); 1 out of 13 of the auxiliary specialists had such a certificate. Scenario training was being conducted at 8 hospitals. A paediatric resuscitation cart was available at both the emergency and paediatric departments of 8 hospitals, 3 of which were fully stocked at both departments. Laryngeal mask airways and PEEP-valves (Positive End Expiratory Pressure) were lacking at 6 of the 9 hospitals. The medication stock was complete at all the hospitals. CONCLUSION: The organisation of and training for the acute care of critically ill children and presence of materials - the aspects we investigated - need attention at all general hospitals evaluated. It appeared that many specialists are not APLS certified and written protocols concerning organisation and training were lacking. The establishment of a task force responsible for the acute care of children is one measure that could result in rapid improvement. Another measure could be the introduction of a standardised list of inventory needed for acute care.

[Two children with severe complications following incomplete removal of a percutaneous endoscopic gastrostomy (PEG) catheter]. / Twee kinderen met ernstige complicaties na onvolledige verwijdering van een percutane-endoscopischegastrostomie(PEG)-katheter.

A 6-year-old boy with multiple severe disabilities was admitted with acute and progressive dyspnoea. A new percutaneous endoscopic gastrostomy (PEG) catheter had been placed 2 weeks earlier, during which the old catheter was cut and left in the stomach. Radiological assessment revealed pneumonia and a traumatic fistula between the oesophagus and the left main bronchus. Respiratory support was required. The patient recovered after oesophagoscopic removal of the remaining portion of the PEG catheter. A 7-year-old boy with multiple severe disabilities presented with an acutely reduced level of consciousness, vomiting and progressive dyspnoea. Chest x-ray revealed signs of aspiration pneumonia and, after respiratory problems worsened, a foreign object in the oesophagus. The foreign object was likely the remaining portion of a PEG catheter that was removed 12 months earlier. The patient was discharged in good condition a few days after oesophagoscopic removal of the remaining catheter. PEG is a commonly used method for enteral feeding in children. The Dutch guideline on enteral feeding in children indicates that endoscopic removal of the PEG catheter is often necessary. In daily practice, however, endoscopic removal is not always performed. To avoid serious complications, authors recommend endoscopic removal ofthe silicon disk when replacing or removing a PEG catheter in children aged less than 6 years and all children with mental retardation, prior laparotomy or constipation. Endoscopic removal of the disk should be considered in all other children if the disk is not passed in stool within 2 weeks and an x-ray shows that the disk is in the oesophagus, stomach or proximal intestine.

Problems in interhospital pediatric intensive care transport in The Netherlands: results from a survey of general pediatricians.

OBJECTIVE: Interhospital pediatric intensive care transport accompanied by non-trained specialists usually occurs with inadequate equipment and has been associated with high incidence of complications. These facts have serious consequences for patients but also can be very disconcerting for specialists. This survey was undertaken to gain insight into the problems encountered in organizing pediatric intensive care transport in The Netherlands to measure the specialist's satisfaction or dissatisfaction with the current state of affairs in the organization of such transports, and additional workload and feelings of insecurity experienced during self-organized transports. DESIGN: Survey, retrospective. SETTING: A postal questionnaire sent to all pediatricians of community hospitals in The Netherlands. METHODS: Results of direct questioning are given as discrete frequencies. After factor and reliability analysis 5-point Likert scale items are summed up in scale constructions. Relationships between scales are examined in regression analysis. RESULTS: Pediatricians appear to be satisfied with current specialist retrieval teams if these teams are available in their region, and highly dissatisfied if not available. Many nontrained specialists consider these transports burdening tasks with a high workload, and they feel insecure during these transports, especially if they report lack of knowledge of the transport equipment. CONCLUSIONS: The need for pediatric specialist retrieval teams in The Netherlands is seen not only in the insufficient level of care delivered by accompanying nontrained specialists and the reported high incidence of complications as shown in the literature but also in the dissatisfaction and high stress of these specialists.

Effect of perfluorochemical liquid ventilation on cardiac output and blood pressure variability in neonatal piglets with respiratory insufficiency.

Respiration and mechanical ventilation induce cyclic variation in cardiac output and blood pressure. We examined these phasic hemodynamic influences of mechanical ventilation during gas ventilation and partial and tidal liquid ventilation in 7 anesthetized and paralyzed young piglets (body weight, 3.0-4.9 kg) made respiratory-insufficient by repeated saline lung lavage. Nonlinear regression analysis of cardiovascular parameters vs. time was done to quantify respiratory-induced fluctuations in hemodynamic variables. The amplitude of oscillations was expressed as a percentage of the mean hemodynamic variable during the study period, and was called the relative oscillation amplitude. The relative oscillation amplitude of left ventricular stroke volume, left ventricular output, systemic arterial pressure, and systemic perfusion pressure was significantly larger (at least twofold) during tidal liquid ventilation compared to partial liquid ventilation. No such differences were observed between gas and partial liquid ventilation at comparable gas ventilator settings. We conclude that in this animal model, within-breath modulation of left ventricular output, systemic blood pressure, and perfusion pressure was significantly increased during tidal liquid ventilation as compared to partial liquid ventilation.

High-frequency oscillatory ventilation, partial liquid ventilation, or conventional mechanical ventilation in newborn piglets with saline lavage-induced acute lung injury. A comparison of gas-exchange efficacy and lung histomorphology.

It has been reported that, in diseased lungs, either partial liquid ventilation (PLV) or high-frequency oscillatory ventilation (HFOV) can improve oxygenation better and with less lung injury than conventional mechanical ventilation (CMV). This study was intended as a preclinical comparison between the effects of HFOV, PLV and CMV on gas exchange, lung mechanics and histology. Fifteen anesthetized newborn piglets, with respiratory insufficiency due to repeated saline lung lavage, were allocated to either a PLV, HFOV or CMV (n = 5 each) strategy, and treated for 4 h. Within 30 min of commencing therapy, PLV, HFOV, and CMV improved arterial PO2 (Pa,O2), alveoloarterial oxygen gradient (P(A-a),O2), oxygenation index (OI), venous admixture (va), and arterial PCO2 (Pa,CO2). After 4 h, oxygenation parameters (Pa,O2, P(A-a),O2, OI and venous admixture) were significantly better in the HFOV group than in the PLV group; the CMV group showed a higher Pa,O2 and lower OI than the PLV group. Gas exchange at the end of the experiment was not different from baseline in the HFOV and CMV groups. Lung histology and morphometry were performed after perfusion-fixation at endotracheal deflation pressure corresponding to mean airway pressure at the end of the experiment. Lung injury score and mean linear intercept were not different between the three treatment groups. We conclude that in this model, gas exchange improved significantly in all three ventilation strategies. Indices of oxygenation improved less during PLV. The saline lavage-induced acute lung injury model used as in this study, is less stable than previously thought. The final lung injury is not influenced by the ventilation strategy. We speculate that the impaired gas exchange during PLV is an expression of diffusion limitation and ventilation-perfusion mismatch in a recovering lung.

Congenitally short trachea with compression of the left mainstem bronchus: MRI findings.

The MRI features of congenitally short trachea in a patient presenting with respiratory distress are presented. Compression of the left mainstem bronchus by the aortic arch, a recognised complication of this anomaly, could be demonstrated by MRI, obviating the need for other imaging modalities.

Treatment of respiratory failure due to respiratory syncytial virus pneumonia with natural surfactant.

We describe two infants suffering from severe pneumonia caused by respiratory syncytial virus (RSV) infection and needing mechanical ventilation with both high ventilator settings and a high fraction of inspired oxygen. The severity of the respiratory failure and the possibility of decreased and/or altered surfactant production led us to treat these infants with intratracheal instillation of natural surfactant. This resulted in an improvement of lung compliance and a decrease in the amount of oxygen required to maintain acceptable oxygen saturations. Intratracheal surfactant instillation might, therefore, be useful in the treatment of severe RSV pneumonia.

Abnormal glomerular basement membrane in idiopathic multicentric osteolysis.

The primary cause of nephropathy in idiopathic multicentric osteolysis is as yet unknown. We report a young girl with idiopathic multicentric osteolysis and nephropathy. An abnormal glomerular basement membrane was the only abnormality found in a renal biopsy taken 2 years before the development of end-stage renal failure. We believe that this biopsy finding represents or is related to the unknown primary lesion causing nephropathy in idiopathic multicentric osteolysis.

Perfluorochemical liquid ventilation: from the animal laboratory to the intensive care unit.

Perfluorochemical or perfluorocarbon liquids have an enormous gas-carrying capacity. During tidal liquid ventilation the respiratory medium of both functional residual capacity and tidal volume is replaced by neat perfluorocarbon liquid. Tidal liquid ventilation is characterized by convective and diffusive limitations, but offers the advantage of preserved functional residual capacity, high compliance and improved ventilation-perfusion matching. During partial liquid ventilation only the functional residual capacity is replaced by perfluorocarbon liquid. Both tidal and partial liquid ventilation improve gas exchange and lung mechanics in hyaline membrane disease, adult respiratory distress models and meconium aspiration. Compared to gas ventilation, there is less histologic evidence of barotrauma after liquid ventilation. Cardio-pulmonary interaction, inherent to the high density of liquid, and long term safety need further study. However, extrapolating from animal data, and taking into account promising human pilot studies, liquid ventilation has the desired properties to occupy an important place in the therapy of restrictive lung disease in man.

Tension pneumoperitoneum as an early complication after adenotonsillectomy.

Pneumoperitoneum as a result of pneumomediastinum has been described already. Amongst the causes pneumoperitoneum due to a complication of adenotonsillectomy, has not previously been described. We describe a five-year-old girl with tension pneumoperitoneum with respiratory and circulatory insufficiency half an hour after adenotonsillectomy. Removal of the intra-abdominal air with a 14 gauge needle restored normal respiration and circulation.

An unexpected cause of upper airway obstruction.

A three-year-old boy with a swelling on the right side of his neck was suspected of having a parapharyngeal abscess after clinical examination and CT scan (computed tomography scan) of this region. Later it became clear, that the swelling was caused by an aneurysm of the internal carotid artery. This case report describes the pitfalls and difficulties encountered in the diagnostic course and treatment planning.

Meningitis due to Fusobacterium necrophorum subspecies necrophorum. Case report and review of the literature.

This paper reports a case of fatal meningitis caused by Fusobacterium necrophorum subsp. necrophorum in a previously healthy five-year-old child. The organism was isolated in pure culture from the cerebrospinal fluid and from intracranial pus obtained at autopsy. The role of F. necrophorum in the development of meningitis is reaffirmed and its isolation and identification are discussed. The clinical presentation of the present case resembles the previously published reports and highlights the poor prognosis in spite of appropriate antibiotic treatment.

Clinical and biochemical observations in a patient with combined Pompe disease and cblC mutation.

Metabolic studies are described in a patient who presented at 3 weeks of age with severe anaemia, hyperbilirubinaemia and hypotonicity. Clinically, glycogen storage disease type II (Pompe disease) was suspected because of a massively enlarged heart and hepatosplenomegaly. This was confirmed biochemically by the demonstration of glycogen accumulation in skeletal muscle and undetectable acid alpha-1,4-glucosidase activity in fibroblasts. Further biochemical studies in this patient surprisingly revealed homocystinuria and methylmalonic aciduria, suggesting a defect in the uptake, transport or intracellular metabolism of vitamin B12. Studies in cultured fibroblasts from the patient revealed a low uptake of [57Co]cyanocobalamin and an impaired intracellular conversion to both 5'-deoxyadenosylcobalamin and methylcobalamin. Moreover, the incorporation of labelled propionate into proteins as well as the formation of labelled methionine from labelled 5-methyltetrahydrofolate was deficient in fibroblasts from the patient. Complementation studies revealed the presence of the cblC mutation in this patient. No treatment was initiated and the patient died at the age of 31 days. We conclude that the patient was affected by both glycogen storage disease type II and cblC disease. The remarkable combination of these two rare inborn errors can be the result of the consanguinity of the parents.

[Not meningitis but septic shock as the killer in acute meningococcal disease]. / Niet nekkramp, maar septische shock als schrikbeeld acute meningokokkenziekte.

The symptoms and clinical course of meningococcaemia in 14 cases are described; 10 patients died; in one of the four survivors amputations were inevitable for necrosis of hands and feet. The foremost symptoms at the first time that a doctor was contacted were fever, lethargy, petechiae and purpura. The fulminant course is shown by the high number of resuscitation at the time of admission or in the first hours after admission, and by the time between first symptoms and death. The mortality of meningococcaemia is mostly not due to meningitis. Most patients die of septic shock even before signs of meningitis can develop. The early signs of meningococcaemia are not those of meningitis, but those of sepsis. Meningism and headache are rare symptoms. The severest symptoms are fever and lethargy, in combination with petechiae and purpura. The fulminant course of the disease requires immediate admission. Treatment of infection and septic shock may be lifesaving.