Body positioning and medical therapy for infantile gastroesophageal reflux symptoms.

Objective: Proton-pump inhibitors (PPIs) reduce acid gastroesophageal reflux (GER) and esophageal acid exposure in infants; however, they do not reduce total GER or symptoms attributed to GER. Reflux is reduced in the left lateral position (LLP). We hypothesize that the effect of LLP in combination with acid suppression is most effective in reducing GER symptoms in infants.

Respiratory disturbances in rett syndrome: don't forget to evaluate upper airway obstruction.

Rett syndrome is characterized by loss of motor and social functions, development of stereotypic hand movements, seizures, and breathing disturbances. This study evaluates the presence of overnight respiratory disturbances. Polysomnography in combination with a questionnaire (the Sleep Disturbance Scale for Children) was performed in 12 Dutch patients with Rett.

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.

Role of health technology assessment in shaping the benefits package in The Netherlands.

In many countries of the Western world, the role of health technology assessment (HTA) in funding decisions of medical technologies is increasing. HTAs are expected to support decision-makers in delineating the collectively funded benefits package. To maximize their potential, it is essential that assessments are valid, reliable and timely, and that it is transparent how information provided in assessments is used in decision-making.

Neonatal paroxysmal trismus and camptodactyly: the Crisponi syndrome.

The Crisponi syndrome is an infrequently described syndrome characterized by extensive muscular contractions in the face after even minimal stimuli, hypertonia, camptodactyly, and typical facial features (chubby cheeks, broad nose with anteverted nares, and long philtrum). Most patients have died in the first months of life due to hyperthermia. The syndrome has been described in Italians only; the inheritance pattern is most probably autosomal recessive.

[The transport of severely ill children in the Amsterdam region; 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.

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.

High levels of interleukin-10 during the initial phase of fulminant meningococcal septic shock.

Interleukin (IL)-10 has an important antiinflammatory effect by inhibiting endotoxin-induced production of proinflammatory cytokines, such as tumor necrosis factor-alpha and IL-1. Since both cytokines are produced in massive amounts during fulminant meningococcal septic shock and are associated with severity of disease, IL-10 was measured in plasma samples of 25 consecutive children with fulminant meningococcal septic shock shortly after admittance to a pediatric intensive care unit. High levels of IL-10 (median, 6021 pg/mL; range, 137-24,600) were found in surviving patients (median, 1268 pg/mL; range, 137-24,600) and in those who died (median, 9915 pg/mL; range, 3996-14,100).

Coagulation activation and tissue necrosis in meningococcal septic shock: severely reduced protein C levels predict a high mortality.

In 35 consecutively admitted children (mean age: 4.3 years) with a clinical diagnosis of meningococcal septic shock (MSS), activation of the coagulation and fibrinolytic pathways was evaluated directly at admittance to the paediatric intensive care unit (ICU). The association of clinical signs and haemostatic abnormalities was assessed.

[Not meningitis but septic shock as the killer in acute meningococcal disease].

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.

[Obstructive sleep apnea syndrome].

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.

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.

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).