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Background: Worldwide, disease in children due to exposure to rats is increasing, also in the Netherlands. Not only the generally known pathogen Leptospira should be considered, also S. moniliformis , Yersinia pestis , Lymphocytic choriomeningitis virus, Hantavirus, Francisella tularensis and Pasteurella multocida are also known rat-associated zoonosis., Case Description: An 12-year-old boy visited the pediatrician with fever, headache and nausea, followed by generalized erythema and arthritis. The boy had a pet rat. The patient's blood culture was positive for S. moniliformis. The patient was treated with antibiotics and made a full recovery., Conclusion: Just like many rat-associated diseases have 'rat-bite fever' caused by S. moniliformis an nonspecific clinical presentation. It is not necessary to have had a rat bite, to develop rat-bite fever. Better awareness and knowledge about rat related diseases should contribute to earlier diagnosis and treatment. Which is of great importance because of increased morbidity and mortality associated to rat related diseases.

In this article, the new Dutch pediatric guideline Brief Resolved Unexplained Event is discussed, which replaces the old guideline Apparent Life Threatening Event. The main goal of the new guideline is identification of a group of low-risk infants who need not be admitted to the hospital and in which only limited diagnostic workup is indicated. Three fictional cases are presented to highlight the major changes in management of infants who present with an unexplained event. Application of the new guideline will likely result in less clinical admissions and diagnostic testing in these patients.

We describe a 13-year-old girl with abdominal complaints and looking pregnant. MRI investigation showed an adnexal mass (28 x 19 x 12 cm), which was surgically removed. Histolpathological investigation revealed a mature cystic teratoma with no signs of malignant transformation.

Skin-to-skin contact after birth is propagated to facilitate breast-feeding and mother-child bonding. We describe two term infants with sudden unexpected postnatal collapse (SUPC) during skin-to-skin contact. The infants were found with abnormal colour, hypotonia and apnoea, in a prone position on the chest of their mothers, both of whom were primipara with a high BMI. After stimulation, both infants recovered completely. No specific potential cause, other than the position, could be found. These cases illustrate that skin-to-skin contact after birth is not without risk. In available literature, risk factors for SUPC include primiparity and infant orientation such as prone and lateral positions. These positions are also risk factors for sudden infant death syndrome (SIDS). To improve safety, a primipara should be supervised during skin-to-skin contact and not be left alone in the first hours after delivery; the infant should be guaranteed a free airway, especially when the mother has a high BMI.

Background: Pyomyositis is a rare bacterial infection of striated muscle, usually caused by Staphylococcus aureus., Case Description: A healthy 9-year-old boy was referred to us because of pain in the hip and fever. We noticed he could point to the pain with one finger; this is referred to as 'pinpoint pain'. An MRI indicated a diagnosis of pyomyositis of the iliacus muscle and piriformis. The infection was caused by S. aureus. The boy recovered completely and swiftly with antibiotic therapy., Conclusion: This specific type of pyomyositis in the hip is rare, especially in children. Being able to localise the pain with one finger is a typical characteristic of this condition. MRI is the investigation of first choice to confirm this diagnosis.

Great progress has been made in reducing the incidence of SIDS in the Netherlands, but the number of SIDS cases has remained constant in recent years. The American Academy of Pediatrics has new guidelines for safe sleep. Existing recommendations remain valid such as (a) discouraging prone- and side-sleeping; (b) encouraging parent and child to sleep in one room but not in one bed; and (c) avoiding second-hand smoke. The new recommendations include: (a) pre-term neonates born after 32 weeks should be placed in a supine position; (b) twins should not sleep in the same bed ('co-bedding'); (c) use of a pacifier is recommended once breastfeeding is well underway; and (d) use of stabilization pillows is not recommended. There should also be an emphasis on educating parents before discharging their newborn from the hospital.

Two infants with congenital toxoplasmosis are presented. A girl born prematurely was treated postnatally after the mother had received antimicrobial treatment during pregnancy for acute toxoplasmosis. Apart from being small for gestational age, she remained without symptoms and treatment was ceased after 13 months. A 2-month-old boy presented with hydrocephalus and chorioretinitis, consistent with congenital toxoplasmosis. Despite antimicrobial treatment, at 12 months of age he suffered from epilepsy, cerebral palsy and vision impairment. Most infants with congenital toxoplasmosis (2 per 1000 live births in the Netherlands) are asymptomatic at birth. The education of pregnant women is crucial for the prevention of congenital toxoplasmosis. Awareness of antenatal and postnatal presenting signs and symptoms is important for clinicians, because early diagnosis and treatment may minimize sequelae. Untreated, the majority of affected infants will develop chorioretinitis, deafness and/or neurological symptoms.

In order to reach a consensus concerning diagnosis, support and follow-up in children with an 'apparent life threatening event' (ALTE) multidisciplinary guidelines have been developed by the Dutch Paediatric Association and the Dutch Institute for Healthcare Improvement (CBO). All children presenting as an emergency with ALTE should undergo inpatient clinical observation for 24-72 h, with at least 24 h of cardio-respiratory monitoring. Observation does not need to be continued once an explanation for the incident has been established. The most common causes of ALTE are: gastro-oesophageal reflux (31%), neurological insult (11%) and lower respiratory tract infection (8%). Results from a targeted case history and from physical and neurological examination serve as a guideline for further investigations and support, in accordance with the formulated flowchart. A complete blood-count, CRP- and glucose concentration determination, blood-gas analysis and exploratory urine analysis should be carried out in all infants admitted with an ALTE, and an acute phase urine sample should be collected and frozen for possible metabolic investigation at a later date. Routine monitoring at home is not recommended following an ALTE.

Objective: To determine whether the incidence of sudden infant death syndrome (SIDS) in child care settings (child care centres or child minders) is different from that in the home setting, and to search for any differences in the prevalence of SIDS risk factors in both settings., Design: Descriptive and comparative., Method: All SIDS cases (< 2 years), that occurred between September 1996-August 2006 and known to the 'Landelijke Werkgroep Wiegendood' (the National Cot Death Study Group) of the Dutch Paediatric Association were analysed. The percentage of children involved in child care and the mean duration of their participation in child care, was calculated from national surveys carried out in well-baby clinics., Results: In the 10 years of the study, 216 cases of SIDS became known to the Cot Death Study Group. In the first year of life, the number was 75% of the number registered by Statistics Netherlands. 28 of these infants died from SIDS between the ages of 3-6 months and on Monday-Friday between 8:00 am-5:00 pm: the usual hours of opening of child care facilities. Based on the uptake of child care during this period, 15% of this mortality was expected to have occurred in a child care setting and 85% at home. In reality, 61% (17/28) of the deaths occurred at a child care facility and 39% (11/28) at home. The relative risk was 8.8 (95% CI: 4.1-19.0). This high incidence of SIDS in a child care setting did not appear to be due to a higher prevalence of known risk factors for SIDS at child care facilities i.e. sleeping position (prone or side), passive smoking, heat congestion, or use of a quilt or pillow., Conclusion: For infants aged 3-6 months, the relative risk ofSIDS during child care appeared to be increased 8.8 times (95% CI: 4.1-19.0) when compared with home settings in The Netherlands in September 1996-August 2006.

Purpose: Evaluating the guideline 'Diagnosis and treatment of respiratory syncytial (RS) virus bronchiolitis' on the number of chest X-rays, C-reactive proteïn (CRP) counts, leukocyte counts, and antibiotic prescriptions in infants admitted to hospital with RS bronchiolitis., Design: Retrospective 'before-after' cohort study., Location: Canisius-Wilhelmina Hospital, Nijmegen, The Netherlands., Patients: Infants admitted with proven RS virus infection., Methods: Guidelines, including sound restriction of performance of X-rays, CRP and leukocytes, were introduced in February 2003. Data from infants admitted with RS virus infection during 1997- 1999 (cohort A) were compared with those admitted from 2003- April 2006 (cohort B)Results: There were 155 infants in cohort A and 170 in cohort B. Implementation of guidelines led to significant reductions of CRP and leukocyte determinations: 49.0% and 48.2%, respectively (both p<0.001) and X-rays: 30.3% (p=0.020). Numbers of antibiotic prescriptions decreased with 55% (p<0.001). The chance of antibiotic prescription increased significantly when X-rays (OR=5.2), CRP (OR=5.4), or leukocytes (OR=4.2) were done. After implementation of the guidelines, the median stay in hospital decreased significantly from 8.0 to 6.0 days (p<0.001; ranges 1-13 days and 2-23 days, respectively). Performing X-ray, CRP or leukocytes, or antibiotic prescription did not significantly alter the total duration of hospital stay., Conclusion: Implementation of the guidelines led to significant decreases in numbers of X-rays, CRP and leukocytes determinations, and antibiotic prescriptions. Our data support the restrictive use of chest X-rays, CRP and leukocyte determinations in infants, admitted to hospital with RS virus bronchiolitis., (© Bohn Stafleu van Loghum 2008.)

Objective: To explain the increase in the number of deaths due to sudden infant death syndrome (SIDS) that occurred while the infant was in a playpen or on a playpen mat placed outside of the pen. 13 deaths were reported in the period 1 September 1996-31 August 2004, in contrast to 0 deaths in the years 1984/'96., Design: Descriptive., Method: Data on the 13 infants that were reported to the National Study Group on SIDS were analysed and the presence of risk factors for SIDS was assessed., Results: Of the 13 infants, 9 were male and 4 were female. 12 were aged less than 12 months and 1 was aged 13 months. Known risk factors for SIDS were often present and frequently occurred in combinations: male sex (n = 9), higher birth order (n = 9), age 1-8 months (n = 12), parental smoking (n = 6), primary prone sleeping position (n = 4), secondary prone (n = 6, often the first time), face down (n = 9), no adult present (n = 13)., Conclusion: There was no clear explanation for the observed increase in SIDS that occurred in a playpen or on a playpen mat placed outside of the pen.

In a newborn infant, frequent bowel movements diminish the enterohepatic circulation of bilirubin, thereby increasing bilirubin excretion. In breastfed newborn infants, the frequency of latching on and administration of supplementary feeds are associated with serum bilirubin concentrations. Frequent breast feeding (at least 8 times a day) and fewer supplementary feeds will result in increased breast milk intake, less weight loss, and lower bilirubin concentrations. In the case of a breastfed infant presenting with neonatal hyperbilirubinaemia, the advice should be to breastfeed more frequently and to withhold supplementary feedings. An icteric newborn infant should be seen and weighed daily. If the infant has lost more than 10% of its birth weight, drinks poorly, or fails to gain weight despite latching onto the breast frequently, it should be referred to the paediatrician for further diagnosis and treatment. To ensure optimal production of breast milk during the first days after birth, early latching on is recommended, preferably within one hour after birth.

A premature neonate was born with a generalized eruption of vesicles, within a day developing into an erythrodermia, with bullae and widespread desquamation, due to congenital cutaneous candidiasis.

Objective: Investigation of the incidence of neonatal herpes in the Netherlands between 1992 and 1998., Design: Inventory questionnaire survey., Methods: All virological laboratories in the Netherlands were sent a questionnaire on the number of culture proven cases of neonatal herpes recorded between 1992 and 1998 and on the type of herpes simplex virus (HSV-1 or HSV-2). The gynaecological and paediatric departments of all university hospitals and of half of the general hospitals were sent questionnaires as well. Gynaecologists were asked how often caesarean section was performed in order to prevent neonatal herpes and how frequently pregnant women were seen with genital herpes. Paediatricians were asked how often they observed neonatal herpes, the type of HSV and the possible transmission route. Based on these data the figures for the whole of the Netherlands were estimated., Results: The incidence of neonatal herpes in the Netherlands in the period 1992 to 1998 was 2.4 per 100,000 neonates. HSV-1 was the cause of neonatal herpes in 73%, HSV-2 in 9%, and in 18% of the cases the type of infection was not recorded. The number of pregnant women with genital herpes had increased, but, in agreement with a consensus statement, the gynaecologists hardly performed caesarean sections any more to prevent neonatal herpes (2 per year)., Conclusions: The incidence of neonatal herpes in the Netherlands had not increased. There was no predominant role of HSV type 2 causing neonatal herpes.

Congenital nasolacrimal duct obstruction (in the literature also called congenital nasolacrimal mucocele or dacryocystocele) presents either as a simple nasolacrimal obstruction with epiphora or as a complicated obstruction on both sides of the lacrimal sac with midfacial deformation and respiratory insufficiency. Five neonates were treated for such complicated congenital nasolacrimal duct obstruction. Symptoms varied from an inner canthal purple-red swelling in the medial corner of the eye, with epiphora, to a serious midfacial deformation with respiratory insufficiency. The presence of a bilateral nasolacrimal mucocele leads to a broadened nasal bridge, which may be falsely interpreted as a dysmorphic feature of a congenital syndrome. Recognition of the condition allows a quick and efficient diagnosis and prevents unnecessary diagnostic procedures and anxiety. Differential diagnosis mainly includes frontobasal encephalocele and haemangioma. Treatment consists of massage, and catheterization if necessary.

During pregnancy irregular blood group antibodies, originating either from earlier pregnancies or from blood transfusions, may severely jeopardize both mother and child. Three patients are described with pregnancy-associated blood group incompatibility. In one case of Kell antagonism a previous child had reportedly died of cot death, but in retrospect it had most probably suffered from erythroblastosis fetalis as a result of anti Kell antibodies. In the second case, a twin pregnancy, the diagnosis of neonatal haemolytic anaemia on the basis of blood group incompatibility with a very rare antibody (anti-Kpb) had been established in the previous child. No precautions had been taken during this pregnancy, putting both mother and children at risk. All three children recovered, the twins after repeated transfusion of Kpb-free erythrocytes. The described cases emphasize the importance of being informed about the presence of antibodies during pregnancy. Such information can only be obtained by assessing the antibody status during pregnancy. In the Netherlands, the screening of all pregnant women for the presence of irregular antibodies was introduced last year.

A fullterm newborn boy developed severe respiratory insufficiency, multiple air leaks and severe pulmonary hypertension, leading to his death on the third day of life. Family history revealed that a sister of the patient had died earlier after a similar course with respiratory problems. The most common causes of respiratory insufficiency could be subsequently excluded. After extensive postmortem investigation alveolar proteinosis was found in the lung tissue. DNA investigation was then performed in the parents, and both appeared to be heterozygotic for the 121ins2 mutation. This finding suggests both children in this family to have been homozygotic for the 121ins2 mutation resulting in a lack of synthesis of surfactant protein B (SP-B). Homozygotic SP-B deficiency in the newborn is a fatal disease with no curative perspectives, except for lung transplantation and gene therapy.

In three women, aged 28, 35, and 38 years, with multiple pregnancies and discordant foetal growth, the question arose what to do in case of (threatening) intrauterine death of one twin. In one monochorionic pregnancy with single foetal death the survivor suffered irreversible neurological damage and died at the age of five months, in one monochorionic pregnancy the survivor was born healthy and in one dichorionic pregnancy both twins were born healthy although one twin showed severe intrauterine growth retardation. The problem concerning single foetal death in a monochorionic pregnancy is whether to terminate the pregnancy and accept the risk of premature birth to the surviving twin, or to continue the pregnancy and accept the risk of damage to the survivor. In a dichorionic pregnancy foetal death of one twin does not entail any great risk of damage to the survivor; in such a pregnancy single foetal death in a premature phase may be accepted and the pregnancy may be continued. Sonographic determination of the chorionicity in multiple pregnancy at an early stage is essential because it also determines the policy if foetal problems occur.

Three infants aged 1, 3 and 9 months with severe theophylline intoxication are reported. Maximum serum theophylline concentrations were 65, 44 and 156 mg/l, respectively. Vomiting, agitation and tachycardia are the classical features. Seizures and cardiac arrhythmias suggest severe intoxication. The third patient underwent immediate peritoneal dialysis. All patients survived and recovered without sequelae. Haemoperfusion is considered to be the definite treatment for severe forms of theophylline intoxication.

Serum IgG, IgA and IgM concentrations of premature infants, 21 boys and 17 girls, gestational age 29.8 +/- 2.5 weeks, birth weight 1243 +/- 378 gram, were measured during the first weeks post partum. At birth, IgG concentrations of newborn infants correlate with gestational age. Subsequently a gradual decline of IgG concentrations occurs. Infants of 25-29 weeks gestational age appeared to have significantly lower IgG concentrations if compared to infants of 29-32 weeks gestational age. IgA concentrations remained below the detection limit during the period covered by this study, whereas serum IgM concentrations showed a gradual increase. Significantly more infants from the group with IgG < 400 mg/dl, measured at the first day of life, had infections if compared to the group with IgG > 400 mg/dl. Exchange transfusions and administration of fresh frozen plasma (FFP) resulted in a rise of immunoglobulin concentrations, whereas administration of packed cells (PC's) and pasteurised plasma protein solution (GPO) did not.

A newborn child of a diabetic mother is described, who developed neonatal hypertension after birth. Clinical signs were not specific and the diagnosis would have been missed if blood pressure had not been measured. The cause of the neonatal hypertension appeared to be a thrombus in the left renal artery, probably originating from the ductus arteriosus Botalli. Control of the neonatal hypertension according to a stepwise treatment regime was very difficult. Based on our experience and on study of the literature it is advised to start treatment in the acute stage with nifedipine orally or with sodium nitroprusside intravenously.

Stimulated by reports in literature concerning high aluminium content of human milk formulas, ten Dutch formulas were investigated. The aluminium content appeared to be rather low. Conventional home preparation induced a pronounced rise of aluminium content (3 to 100 fold). Even in this way prepared, milk formula is safe.

Neonatal Adrenal Haemorrhage (NAH) is a condition, which often presents with a palpable abdominal mass and prolonged jaundice. Supra-renal calcifications, seen on routine X-ray films, often result from adrenal haemorrhage in early life. Disturbed renal function at the ipsilateral side, as can be seen from excretory urography and renal scintigraphy, is a much less known condition in association with NAH. The case history of three patients is described. The presentation, diagnostical approach and course of NAH is discussed. Echography is the most important diagnostic tool. Echographic follow up discriminates NAH from other conditions presenting with a palpable abdominal mass and allows for a waiting attitude.