Koen Joosten

Dutch national survey to test the STRONGkids nutritional risk screening tool in hospitalized children

BACKGROUND & AIMSnChildren admitted to the hospital are at risk of developing malnutrition. The aim of the present study was to investigate the feasibility and value of a new nutritional risk screening tool, called STRONG(kids), in a nationwide study.nnnMETHODSnA Prospective observational multi-centre study was performed in 44 Dutch hospitals (7 academic and 37 general), over three consecutive days during the month of November 2007. The STRONG(kids) screening tool consisted of 4 items: (1) subjective clinical assessment, (2) high risk disease, (3) nutritional intake, (4) weight loss. Measurements of weight and length were performed. SD-scores <-2 for weight-for-height and height-for-age were considered to indicate acute and chronic malnutrition respectively.nnnRESULTSnA total of 424 children were included. Median age was 3.5 years and median hospital stay was 2 days. Sixty-two percent of the children were classified at risk of developing malnutrition by the STRONG(kids) tool. Children at risk had significantly lower SD-scores for weight-for-height, a higher prevalence of acute malnutrition and a longer hospital stay compared to children with no nutritional risk.nnnCONCLUSIONSnThe nutritional risk screening tool STRONG(kids) was successfully applied to 98% of the children. Using this tool, a significant relationship was found between having a high risk score, a negative SD-score in weight-for-height and a prolonged hospital stay.

National malnutrition screening days in hospitalised children in The Netherlands

Objective Nationwide prevalence studies on malnutrition in hospitalised children have not been done. This study aimed to investigate the prevalence of malnutrition of all newly admitted children in The Netherlands during 3 consecutive days. Design Prospective observational study. Setting Paediatric wards of 44 hospitals (7 academic and 37 general). Participants A total of 424 children aged>30 days and hospitalised for > 1 day were included, 63% male, 86% non-white. Median age was 3.5 years and median hospital stay was 2 days. Main outcome measures SD scores ,22 for weight for height and height for age were considered to indicate acute and chronic malnutrition, respectively. Results Overall 19% of the children had acute and/or chronic malnutrition at admission (academic 22% and general 17%). The proportion of children with chronic malnutrition was significantly higher in academic hospitals (14% vs 6%). Logistic regression analysis allowing for age, underlying disease, ethnicity, surgery and type of centre showed a significant relation between the presence of malnutrition at admission and underlying disease (odds ratio (OR) 2.2). For chronic malnutrition both underlying disease and non-white ethnicity were significantly related to a higher prevalence (OR 3.7 and OR 2.8, respectively). Multiple regression analysis showed that children with acute malnutrition stayed on average 45% longer (95% CI 7% to 95%) in the hospital than children without such malnutrition. Conclusions This unique nationwide study shows that 19% of children admitted to Dutch hospitals are malnourished at admission. This high prevalence underlines the need for routine screening and treatment of malnutrition in hospitalised children.

Undiagnosed obstructive sleep apnea syndrome in children with syndromal craniofacial synostosis.

Children with syndromal craniofacial synostosis have a high risk for obstructive sleep apnea syndrome. Early diagnosis and treatment can relieve symptoms and morbidity. Little is known about the development and natural history of obstructive sleep apnea syndrome through life. The aim of this study was to investigate our experience of clinical history and treatment modalities concerning obstructive sleep apnea syndrome from birth until the current age in children with syndromal craniofacial synostosis. Children with one of the three syndromal craniofacial synostoses (Apert, Crouzon, or Pfeiffer) born between 1984 and 2001 were evaluated. The medical history and symptoms of obstructive sleep apnea syndrome were assessed by retrospective analysis of the medical records. The present and past complaints were explored by means of a questionnaire. Retrospective analysis of the medical records showed a suspicion for obstructive sleep apnea syndrome in 26% of the children compared with 53% in the questionnaire. The severity and presentation of obstructive sleep apnea syndrome were not related to the age of the child. Obstructive sleep apnea syndrome symptoms occurred in almost half of the children during colds. Several symptoms were significantly more common in children with a high suspicion for obstructive sleep apnea syndrome. Treatment modalities consisted of adenotonsillectomies, continuous positive airway pressure, and Le Fort III surgery. Use of a standard questionnaire showed that the suspicion for obstructive sleep apnea syndrome in children with syndromal craniofacial synostosis is much higher than reported in the medical records. Regular screening for obstructive sleep apnea syndrome with a standard questionnaire could be of additional value for the detection of obstructive sleep apnea syndrome in children with syndromal craniofacial synostosis.

Obstructive sleep apnea in children with syndromic craniosynostosis: long-term respiratory outcome of midface advancement

Almost 50% of patients with Apert, Crouzon or Pfeiffer syndrome develop obstructive sleep apnea (OSA), mainly due to midface hypoplasia. Midface advancement is often the treatment of choice, but the few papers on long-term outcome report mixed results. This paper aimed to assess the long-term respiratory outcome of midface advancement in syndromic craniosynostosis with OSA and to determine factors contributing to its efficacy. A retrospective study was performed on 11 patients with moderate or severe OSA, requiring oxygen, continuous positive airway pressure (CPAP), or tracheostomy. Clinical symptoms, results of polysomnography, endoscopy and digital volume measurement of the upper airways on CT scan before and after midface advancement were reviewed. Midface advancement had a good respiratory outcome in the short term in 6 patients and was ineffective in 5. In all patients without respiratory effect or with relapse, endoscopy showed obstruction of the rhino- or hypopharynx. The volume measurements supported the clinical and endoscopic outcome. Despite midface advancement, long-term dependence on, or indication for, CPAP or tracheostomy was maintained in 5 of 11 patients. Pharyngeal collapse appeared to play a role in OSA. Endoscopy before midface advancement is recommended to identify airway obstruction that may interfere with respiratory improvement after midface advancement.

Status epilepticus : Clinical analysis of a treatment protocol based on midazolam and phenytoin

The efficacy of a combination of midazolam and phenytoin in treating generalized convulsive status epilepticus in children was studied retrospectively. The patient group comprised all patients admitted for generalized convulsive status epilepticus to the pediatric intensive care unit over 7 years. Patients treated according to the protocol were included (N = 122). These patients were treated with the following regimen; each subsequent step was taken if clinical evidence of epileptic activity persisted: midazolam 0.5 mg/kg rectally or 0.1 mg/kg intravenously. After 10 minutes: midazolam 0.1 mg/kg intravenously. After 10 minutes: phenytoin 20 mg/kg intravenously in 20 minutes. After phenytoin load: midazolam 0.2 mg/kg intravenously followed by midazolam 0.1 mg/kg/hour continuously, increased by 0.1 mg/kg/hour every 10 minutes to maximum 1 mg/kg/hour. Phenobarbital 20 mg/kg intravenously or pentobarbital 2 to 5 mg/kg intravenous load, 1 to 2 mg/kg/hour continuously intravenously. Patients who received initial rectal diazepam were included. Patients were categorized according to the cause of generalized convulsive status epilepticus. These categories were then related to the level of antiepileptic therapy needed. Patients ages ranged from 0.5 to 197.4 months. The cause of generalized convulsive status epilepticus was idiopathic or febrile convulsions in two thirds of cases. Most (89%) patients were managed on midazolam and phenytoin. Generalized convulsive status epilepticus was terminated with midazolam alone in 58 patients, with the addition of phenytoin in 19 patients and with continuous midazolam in 32 patients. Thirteen patients needed additional barbiturates. The relationship between the level of antiepileptic therapy and etiology was not significant. Fifty-two patients needed artificial ventilation. Seven patients died; no deaths were directly attributable to generalized convulsive status epilepticus itself. With the use of the proposed protocol, combining midazolam and phenytoin, 89% of the cases of generalized convulsive status epilepticus could be successfully managed. (J Child Neurol 2005;20:476-481).

How does obstructive sleep apnoea evolve in syndromic craniosynostosis? A prospective cohort study

Objective To describe the course of obstructive sleep apnoea syndrome (OSAS) in children with syndromic craniosynostosis. Design Prospective cohort study. Setting Dutch Craniofacial Centre from January 2007 to January 2012. Patients A total of 97 children with syndromic craniosynostosis underwent level III sleep study. Patients generally undergo cranial vault remodelling during their first year of life, but OSAS treatment only on indication. Main outcome measures Obstructive apnoea-hypopnoea index, the central apnoea index and haemoglobin oxygenation-desaturation index derived from consecutive sleep studies. Results The overall prevalence of OSAS in syndromic craniosynostosis was 68% as defined by level III sleep study. Twenty-three patients were treated for OSAS. Longitudinal profiles were computed for 80 untreated patients using 241 sleep studies. A mixed effects model showed higher values for the patients with midface hypoplasia as compared to those without midface hypoplasia (Omnibus likelihood ratio test=7.9). In paired measurements, the obstructive apnoea-hypopnoea index (Z=−3.4) significantly decreased over time, especially in the first years of life (Z=−3.3), but not in patients with midface hypoplasia (Z=−1.5). No patient developed severe OSAS during follow-up if it was not yet diagnosed during the first sleep study. Conclusions OSAS is highly prevalent in syndromic craniosynostosis. There is some natural improvement, mainly during the first 3u2005years of life and least in children with Apert or Crouzon/Pfeiffer syndrome. In the absence of other co-morbid risk factors, it is highly unlikely that if severe OSAS is not present early in life it will develop during childhood. Ongoing clinical surveillance is of great importance and continuous monitoring for the development of other co-morbid risk factors for OSAS should be warranted.

Glucocorticoid receptor mRNA levels are selectively decreased in neutrophils of children with sepsis

ObjectiveCorticosteroids are used in sepsis treatment to benefit outcome. However, discussion remains on which patients will benefit from treatment. Inter-individual variations in cortisol sensitivity, mediated through the glucocorticoid receptor, might play a role in the observed differences. Our aim was to study changes in mRNA levels of three glucocorticoid receptor splice variants in neutrophils of children with sepsis.Patients and designTwenty-three children admitted to the pediatric intensive care unit with sepsis or septic shock were included. Neutrophils were isolated at days 0, 3 and 7, and after recovery (>3xa0months). mRNA levels of the glucocorticoid receptor splice variants GR-α (determining most of the cortisol effect), GR-P (increasing GR-α effect) and GR-β (inhibitor of GR-α) were measured quantitatively.Main resultsNeutrophils from sepsis patients showed decreased levels of glucocorticoid receptor mRNA of the GR-α and GR-P splice variants on day 0 compared to after recovery. GR-α and GR-P mRNA levels showed a gradual recovery on days 3 and 7 and normalized after recovery. GR-β mRNA levels did not change significantly during sepsis. GR expression was negatively correlated to interleukin-6 (a measure of disease severity, rxa0=xa0−0.60, Pxa0=xa00.009).ConclusionsChildren with sepsis or septic shock showed a transient depression of glucocorticoid receptor mRNA in their neutrophils. This feature may represent a tissue-specific adaptation during sepsis leading to increased cortisol resistance of neutrophils. Our study adds to understanding the mechanism of cortisol sensitivity in immune cells. Future treatment strategies, aiming at timing and tissue specific regulation of glucocorticoids, might benefit patients with sepsis or septic shock.

Critically ill infants benefit from early administration of protein and energy-enriched formula: A randomized controlled trial

BACKGROUND & AIMSnNutritional support improves outcome in critically ill infants but is impeded by fluid restriction, gastric intolerance and feeding interruptions. Protein and energy-enriched infant formulas may help to achieve nutritional targets earlier during admission and promote anabolism.nnnMETHODSnRandomized controlled design. Infants with respiratory failure due to RSV-bronchiolitis received a protein and energy-enriched formula (PE-formula, n=8) or a standard formula (S-formula, n=10) during 5 days after admission.nnnPRIMARY OUTCOMEnnutrient delivery, energy and nitrogen balance and plasma amino acid concentrations. Secondary outcome: tolerance and safety.nnnRESULTSnNutrient intakes were higher in PE fed infants and met population reference intake (PRI) on day 3-5 whilst in S-fed infants PRI was met on day 5 only. Cumulative nitrogen balance (cNB) and energy balance (cEB) were higher in PE-infants compared to S-infants (cNB: 866+/-113 vs. 296+/-71 mg/kg; cEB: 151+/-31 and 26+/-17 kcal/kg, both P<0.01). Essential amino acid levels were higher in PE-infants but within reference limits whereas below these limits in S-infants. Both formulas were well tolerated.nnnCONCLUSIONSnEarly administration of a protein and energy-enriched formula in critically ill infants is well tolerated, promotes a more adequate nutrient intake and improves energy and nitrogen balance without adverse effects.

Surveillance study of apparent life-threatening events (ALTE) in the Netherlands

SIDS and ALTE are different entities that somehow show some similarities. Both constitute heterogeneous conditions. The Netherlands is a low-incidence country for SIDS. To study whether the same would hold for ALTE, we studied the incidence, etiology, and current treatment of ALTE in The Netherlands. Using the Dutch Pediatric Surveillance Unit, pediatricians working in second- and third-level hospitals in the Netherlands were asked to report any case of ALTE presented in their hospital from January 2002 to January 2003. A questionnaire was subsequently sent to collect personal data, data on pregnancy and birth, condition preceding the incident, the incident itself, condition after the incident, investigations performed, monitoring or treatment initiated during admission, any diagnosis made at discharge, and treatment or parental support offered after discharge. A total of 115 cases of ALTE were reported, of which 110 questionnaires were filled in and returned (response rate 97%). Based on the national birth rate of 200,000, the incidence of ALTE amounted 0.58/1,000 live born infants. No deaths occurred. Clinical diagnoses could be assessed in 58.2%. Most frequent diagnoses were (percentages of the total of 110 cases) gastro-esophageal reflux and respiratory tract infection (37.3% and 8.2%, respectively); main symptoms were change of color and muscle tone, choking, and gagging. The differences in diagnoses are heterogeneous. In 34%, parents shook their infants, which is alarmingly high. Pre- and postmature infants were overrepresented in this survey (29.5% and 8.2%, respectively). Ten percent had recurrent ALTE. In total, 15.5% of the infants were discharged with a home monitor. In conclusion, ALTE has a low incidence in second- and third-level hospitals in the Netherlands. Parents should be systematically informed about the possible devastating effects of shaking an infant. Careful history taking and targeted additional investigations are of utmost importance.

Current recommended parenteral protein intakes do not support protein synthesis in critically ill septic, insulin-resistant adolescents with tight glucose control

Objective:To investigate the effects of insulin infusion and increased parenteral amino acid intakes on whole body protein balance, glucose kinetics, and lipolysis in critically ill, insulin-resistant, septic adolescents. Design:A single-center, randomized, crossover study. Setting:A medicosurgical intensive care unit in a tertiary university hospital. Patients:Nine critically ill, septic adolescents (age 15.0 ± 1.2 yrs, body mass index 20 ± 4 kg m−2) receiving total parenteral nutrition. Interventions:Patients received total parenteral nutrition with standard (1.5 g·kg−1·day−1) and high (3.0 g·kg−1·day−1) amino acid intakes in a 2-day crossover setting, randomized to the order in which they received it. On both study days, we conducted a primed, constant, 7-hr stable isotope tracer infusion with [1-13C]leucine, [6,6-2H2]glucose, and [1,1,2,3,3-2H5]glycerol, in combination with a hyperinsulinemic euglycemic clamp during the last 3 hrs. Measurements and Main Results:Insulin decreased protein synthesis at standard amino acid and high amino acid intakes (p < .01), while protein breakdown decreased with insulin at standard amino acid intake (p < .05) but not with the high amino acid intake. High amino acid intake improved protein balance (p < .05), but insulin did not have an additive effect. There was significant insulin resistance with an M value of ∼3 (mg·kg−1·min−1)/(mU·mL−1) which was 30% of reported normal values. At high amino acid intake, endogenous glucose production was not suppressed by insulin and lipolysis rates increased. Conclusion:The current recommended parenteral amino acid intakes are insufficient to maintain protein balance in insulin-resistant patients during tight glucose control. During sepsis, insulin decreases protein synthesis and breakdown, and while high amino acid intake improves protein balance, its beneficial effects may be offset by enhanced endogenous glucose production and lipolysis, raising concerns that insulin resistance may have been exacerbated and that gluconeogenesis may have been favored by high amino acid intakes. Dose-response studies on the effect of the level of amino acid intakes (protein) on energy metabolism are needed.