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R J Gemke

Publications and source records attributed to R J Gemke.

At least 19 recordsLinked to original sources
PubMed2001

[Corticosteroid administration for critically ill patients].

In critically ill patients, the hypothalamic-pituitary-adrenal axis is usually activated, resulting in elevated plasma cortisol levels. This enables the human organism to cope with sepsis, trauma and other forms of stress. During critical illness, total adrenal insufficiency rarely occurs. On the other hand, septic shock can be accompanied by a relative deficit of cortisol. Causes of this relative adrenal insufficiency are a dysfunction of the hypothalamic-pituitary-adrenal axis and/or cortisol resistance. There are no strict biochemical criteria available to diagnose relative adrenal insufficiency; clinical observation is the decisive factor. In randomised trials with patients in septic shock, a more rapid haemodynamic recovery was obtained with physiological doses of hydrocortisone than with a placebo. The observed haemodynamic response following hydrocortisone administration supports the concept of relative adrenal insufficiency.

Adrenal Insufficiency↗
PubMed2001

[Intracranial hemorrhages in infants: child abuse or a congenital coagulation disorder?].

In children with head injuries the severity of the neurological symptoms should concord with the patient's history and signs of neurotrauma on examination. Discrepancies between the (hetero)anamnesis and physical examination on the one hand and neurological findings on the other may indicate child abuse. The presence of both old and new intracranial haemorrhages in the absence of proportional trauma is generally considered as evidence for child abuse. However, these symptoms may also be the first manifestation of a congenital coagulation disorder. Three children, two girls aged 8 and 5 months and a boy aged 6 months were presented with alarming neurological symptoms due to intracranial haemorrhages without external signs of head trauma. The first girl had 'shaken baby' syndrome while the other 2 had congenital coagulation disorders (haemophilia B and factor V deficiency, respectively). All three recovered, the last two with remaining one-sided neurological deficits. Child abuse and congenital coagulation disorders may present with similar neurological symptoms and radiological findings. In these patients coagulation tests are mandatory and--if abnormal--enable early substitution of deficits and prevent inappropriate suspicion or accusation of caretakers.

Blood Coagulation Disorders↗
PubMed2001

Carnitine-acylcarnitine translocase deficiency: phenotype, residual enzyme activity and outcome.

UNLABELLED: Carnitine-acylcarnitine translocase deficiency is a rare and life-threatening mitochondrial fatty acid beta-oxidation disorder. We describe a patient who, despite a severe clinical course and an extremely low carnitine-acylcarnitine translocase activity, is currently alive and in good health. We performed an extensive analysis of all previously published cases in order to evaluate the clinical features and prognostic factors. Reports on 21 patients with carnitine-acylcarnitine translocase deficiency were obtained. Only 5 out of the 21 patients survived early childhood. At least 20 siblings are reported to have died of sudden unexplained death in the neonatal period. Although phenotype and residual enzyme activity have been suggested to be related to outcome, we were not able to establish such a relationship. CONCLUSION: Phenotype and residual enzyme activity do not appear to be major prognostic factors. Vigorous work-up in order to reach an expedite diagnosis and prompt medical intervention during acute episodes, especially in the neonatal period, may prevent fatal complications.

Cardiomyopathy, Hypertrophic↗
PubMed2001

Measuring health status using the Health Utilities Index: agreement between raters and between modalities of administration.

The aim of this study was to evaluate interrater and intermodality agreement in assessing health status using the Health Utilities Index. A random sample from a Dutch cohort of 14-year-old Very Low Birth Weight children and their parents were invited to participate in a face-to-face (n = 150) or telephone (n = 150) interview. All 300 participants were also sent a questionnaire by mail. Response rate was 68%. Interrater and intermodality agreement were high for the physical HUI3 attributes and poor for the psychological attributes. Children and parents reported more dysfunction in the psychological attributes when interviewed than when completing the mailed questionnaire. High agreement on the physical attributes may have resulted from the fact that hardly any dysfunction was reported in these attributes, and poor agreement in the psychological attributes may have been a result of the fact that in these attributes much more dysfunction was reported. In measuring children's health status using the HUI3, the results and their interpretation vary with the source of information and the modality of administration. For maximum comparability between studies, written self-report questionnaires seem the preferred option.

Adolescent↗
PubMed2000

Pediatric risk of mortality (PRISM) score in meningococcal disease.

UNLABELLED: To assess the pediatric risk of mortality (PRISM) score as a prognostic scoring system in severe meningococcal disease, the files of 53 consecutive patients admitted to a tertiary pediatric intensive care with a clinical diagnosis of meningococcal disease and positive cultures from blood and/or cerebrospinal fluid were analysed. PRISM-score-based expected mortality was compared with observed mortality. Expected mortality in the whole study population was 29% while observed mortality was 19% (P<0.05). The highest expected and observed mortality was found in septicaemic patients without (documented) meningitis, while meningitis patients without septicaemia had the lowest mortality. All patients with a mortality risk below 18.3% (n = 29) survived whereas all those with a mortality risk of 65% or higher (n = 7) died. Of the 17 patients with a mortality risk between 18.3% and 63.9%, 14 survived and 3 died. The area under the receiver-operating characteristic (ROC) curve was 0.94, which is at least comparable with the best-performing meningococcal-disease-specific scoring systems. CONCLUSION: The PRISM score is a useful generic measure of severity of illness in meningococcal disease and can be used to determine the effectiveness of different treatment strategies.

Adolescent↗
PubMed2000

High-frequency oscillatory ventilation in pediatric patients.

BACKGROUND: High-frequency oscillatory ventilation (HFOV) is a ventilatory mode using small tidal volumes with low phasic pressures at supraphysiological frequencies. Beyond the neonatal period there are distinct lung diseases for which HFOV is used. Data of 35 children who deteriorated on conventional ventilation were retrospectively analysed in two tertiary pediatric intensive care units. METHODS: Depending on the underlying pulmonary pathophysiology, three strategies were employed. First, the 'open-lung' strategy designed to rapidly recruit and maintain optimal lung volume in DAD (n=27) and pulmonary hemorrhage (n=5). Second, the 'low-volume' strategy in persistent air leak (n=1) where, after an initial identical approach, mean airway pressure (MAP) is reduced until the air leak ceases. Third, the 'open-airway' strategy in obstructive airway disease (n=5) where MAP is used to recruit and stent the airways. RESULTS: Seven patients died, two due to respiratory failure. Three patients developed an air leak. Nine patients developed chronic lung disease. There was a significant decrease of the oxygenation index (OI) in the survivors. In the two patients who died of respiratory failure, the OI increased. CONCLUSION: If certain conditions are met, HFOV appears a safe and effective mode of ventilation in pediatric respiratory failure.

Adolescent↗
PubMed1999

Lethal encephalopathy complicating childhood shigellosis.

UNLABELLED: A 6-year-old girl is described who died following rapid neurological deterioration, ending in lethal cerebral oedema. Despite the absence of severe intestinal and metabolic derangement, Shigella was cultured from the stool. Toxic encephalopathy is responsible for death following this rare complication of childhood shigellosis in developed countries. The pathophysiology is unknown. CONCLUSION: Lethal toxic encephalopathy can be caused by Shigella despite the absence of severe intestinal and metabolic derangement. If shigelllosis is suspected, headache may be a first significant sign for the development of toxic encephalopathy. Early recognition and rapid measures to prevent brain oedema may improve outcome.

Bacteremia↗
PubMed1999

Circumstances of dying in hospitalized children.

UNLABELLED: Conditions of dying in a tertiary children's hospital were assessed in a retrospective cohort study. Non-survivors, excluding newborns and emergency room patients, were allocated to four groups: brain death (BD), failed cardiopulmonary resuscitation (failed CPR), death following a do-not-resuscitate (DNR) order and death following withholding or withdrawal of therapy (W/W). In a 4-year period 190 (1.3%) of 14,903 admitted patients died. Of these 134 (71%) died on the paediatric intensive care unit, 42 (22%) on the ward and 14 (7%) in the operating room. W/W was found in 75 (39%), failed CPR in 57 (30%), BD in 32 (17%), and death following a DNR order in 26 (14%). Justifications for restrictions of treatment (W/W or DNR) were imminent death in 41 (41%), lack of future relational potential in 13 (13%) and excessive burden of disease in 47 (47%). In non-survivors analgesics and sedatives were frequently used to relieve suffering in the terminal phase. General principles for the approach of terminally ill children in whom death may become an option instead of a fate are discussed. CONCLUSION: In the majority of children dying in hospital, death occurred following restrictions of life-sustaining treatment, comprising do-not-resuscitate or other forms of withholding or withdrawal of therapy.

Adolescent↗
PubMed1998

[Excess mortality in critically ill patients after treatment with human albumin].

According to the results of a systematic review of randomized clinical studies administration of human albumin to critically ill patients is associated with excess mortality, compared with withholding albumin or administration of crystalloid fluids. The study appears to be well done. Also, there are various explanatory pathophysiological mechanisms supporting the association. However, a favourable effect of albumin in certain patient groups cannot be excluded. Alternatives to albumin are available in most clinical situations, but unfortunately, they are not completely without drawbacks. The use of albumin has to be limited; it might only be abolished when a better effect of other fluids, such as synthetic solutions, is demonstrated.

Albumins↗
PubMed1998

The relation between TISS and real paediatric ICU costs: a case study with generalizable methodology.

OBJECTIVE: To determine the quantitative relation between the Therapeutic Intervention Scoring System (TISS) in combination with other relevant clinical variables and the real costs of (paediatric) intensive care. DESIGN: A prospective, observational study. SETTING: A Ten-bed paediatric intensive care unit in a university children's hospital. PATIENTS AND PARTICIPANTS: In a 17-months registration period we collected patient- and treatment-related data for all 611 consecutive admissions. A 21-day calibration period was used to collect detailed data to calculate the real costs of 33 consecutive admissions, in addition to the same data as in the registration period. MEASUREMENTS AND RESULTS: We used the Multi Moment Measurement method to measure time spent by nurses and physicians and medication used in the 21-day calibration period. The calibration period data set with explanatory variables including TISS was used to build a regression model to estimate nurse and physician time, which were converted to personnel costs, and to estimate medication costs. The regression models built from the calibration period were subsequently used to estimate the total costs per day and per admission in different patient groups in the registration period. CONCLUSION: It was feasible to calculate total direct medical costs based on a limited number of readily available clinical variables related to patient characteristics and treatment, of which TISS was the most important determinant. The proposed methods provide further tools for assessment of (paediatric) intensive care unit performance.

Direct Service Costs↗
PubMed1997

[The necessity for centralization of pediatric intensive care].

Substantial evidence indicates that outcome of critically ill children, treated in tertiary paediatric intensive care units (PICUs) is superior to that of those treated in other settings. However, a significant number of children who require this level of care are not admitted to such a unit e.g. due to capacity constraints, reluctance of physicians of general hospitals to refer children to a tertiary centre, and transportation problems. Centralization of care, as recently proposed in the UK, is necessary in the Netherlands, as well, to improve the quality of care. This will require a controlled number of PICU beds in a restricted number of centres, adequate transport facilities and step-down or high dependency units in large general hospitals.

Adolescent↗
PubMed1997

Cetrimide overdose.

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Anticestodal Agents↗