[The transport of severely ill children in the Amsterdam region; 1995-2001]. / Het transport van ernstig zieke kinderen in de regio Amsterdam; 1995-2001.

OBJECTIVE: To inventory experiences of the transport of critically ill children in the Amsterdam region. DESIGN: Retrospective, observational. METHOD: Data were collected from the 1299 children who were transported to our paediatric intensive-care unit from 1 January 1995 until 31 December 2001. Severity of illness was scored and mortality risk calculated. Data on 535 children who were retrieved by our intensive-care team were compared to those from the 764 who were attended by the referring team. The impact on the outcome of distance and duration of transports from both inside and outside the Amsterdam region was analyzed. RESULTS: Two thirds of the transports took place during the evening and night. The median age of the children was 7.5 months. Main indication for admission was respiratory or circulatory insufficiency. During the stabilizing procedure before retrieval, one or more interventions were conducted by our team in 368 (69%) of the 535 retrieved children. 940 children were transported within our region. There were no significant differences between retrieval and non-retrieval groups with respect to length of stay, length of ventilation and mortality. In patients from outside our region the mortality in the retrieval group was higher than in the non-retrieval group. CONCLUSION: Retrieval by a specialized team did not always contribute to a favourable outcome. However, from both a logistical and a medical point of view, a retrieval system seems warranted in order to guarantee a higher level of care. There is a need for more clarity regarding the indications for retrieval by an intensive-care team.

Meningococcal disease: a comparison of eight severity scores in 125 children.

OBJECTIVE: To investigate the accuracy of eight different prognostic scores (Stiehm, Niklasson, Leclerc, Garlund, the MOC score, Tesero, the Glasgow Meningococcal Septicaemia Prognostic Score (GMSPS) and Tüyzüs) in the prediction of fatal outcome in meningococcal disease. DESIGN: Combined prospective and retrospective study. SETTING: A 175-bed pediatric department of a university hospital providing secondary care to +/- 180,000 inhabitants and serving as a referral center. The Pediatric Intensive Care (14 beds) is one of the six PICUs in the Netherlands and provides tertiary care for children under 18 years. PATIENTS: During an 8-year period (1986-1994) 125 children (mean age 4 years, 10 months) with culture-proven meningococcal disease were studied: 34 patients presenting with meningitis, 33 patients with septic shock and 58 patients with meningitis and septic shock. MAIN RESULTS: All eight scores discriminated above average between survivors and non-survivors, as expressed by the corresponding Receiver Operator Characteristic (ROC) curves. The area under the ROC curve (AUC) ranged from 0.74 for the Garlund score to 0.93 for the GMSPS. The GMSPS performed significantly better than its competitors, even after exclusion of the base deficit as one of the score components (AUC = 0.92). It showed above average calibration when logistically transformed into a probability of mortality, and accurately identified a subgroup of patients with no mortality. None of the scores correctly identified non-survivors. CONCLUSION: The GMSPS is a simple score that can be reliably used for risk classification and the identification of low-risk patients.

[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.

[Obstructive sleep apnea syndrome]. / Het obstructieve slaap-apneu syndroom.

Four children were found to have clinically significant obstructive sleepapnea. Hypertrophy of the nasopharyngeal lymphoid tissue, specifically the adenoid and tonsils causing obstructive sleepapnea in children, is a well defined clinical entity with nocturnal CO2 retention, retarded growth and impaired physical and psychological status. A small number of these children may develop pulmonary hypertension, cor pulmonale and ultimately death. Children with only moderately enlarged tonsils in association with neuromuscular hypotonia and anatomical defects may also develop this syndrome. Polysomnographic monitoring during natural sleep proved useful in confirming the diagnosis. All patients had improved after surgical relief of airway obstruction. The subjective impressions were documented by objective improvement: normalization of growth curves, ECG improvement of cor pulmonale and improvement of bloodgasses. Increased awareness of obstructive sleepapnea and examination of the sleeping patient and polysomnographic monitoring should result in earlier treatment and less morbidity for children with obstructive sleepapnea.

Cor pulmonale and acute liver necrosis, due to upper airway obstruction as part of pycnodysostosis.

A male patient with pycnodysostosis suffered from chronic respiratory insufficiency and pulmonary hypertension. This was caused by concomitant upper airway obstruction, resulting from a low implanted uvula and a long soft palate, in combination with glossoptosis and retrognathia due to the flattened mandibular angles. An inter-current respiratory infection gave rise to an acute deterioration, with right-sided heart failure, severe liver damage and coma. Surgical shortening of the soft palate was performed, after which the blood gas values returned to normal.

Septicemia due to coagulase-negative staphylococci in a neonatal intensive care unit: clinical and bacteriological features and contaminated parenteral fluids as a source of sepsis.

During the years 1979 to 1981 we experienced an increasing incidence of septicemia due to coagulase-negative staphylococci in the neonatal intensive care unit (NICU). A detailed analysis was performed for the 1981 NICU population. More than 90% of cases occurred in premature infants of low birth weight (less than 2500 g). All septicemic infants were receiving intravenous therapy and total parenteral nutrition (TPN) solutions had been administered to nearly 80% just before or during the septic episode. A case-control study performed for the 1981 NICU population, which included 26 proved cases of coagulase-negative staphylococcal septicemia and 26 matched controls, did not uncover any differences in underlying diseases or modes of treatment between cases and controls. However, the infusion of contaminated TPN fluids was identified as a significant risk factor. Random bacteriological checks of TPN fluids revealed that nearly 20% of these solutions were contaminated, mainly with coagulase-negative staphylococci. The incidence of staphylococcal septicemia in infants who had received contaminated TPN fluids was 10-fold higher than in infants who had received sterile solutions (P less than 0.0005). The majority of coagulase-negative staphylococci isolated from the blood cultures from the NICU were multiply resistant to antibiotics although all isolates were susceptible to cephalothin. Treatment, consisting of removal or replacement of the intravenous devices and the administration of cephalothin and fresh plasma, was universally successful.