Cervical Spinal Cord Compression and Sleep-Disordered Breathing in Syndromic Craniosynostosis.

BACKGROUND AND PURPOSE: Cerebellar tonsillar herniation arises frequently in syndromic craniosynostosis and causes central and obstructive apneas in other diseases through spinal cord compression. The purposes of this study were the following: 1) to determine the prevalence of cervical spinal cord compression in syndromic craniosynostosis, and 2) to evaluate its connection with sleep-disordered breathing. MATERIALS AND METHODS: This was a cross-sectional study including patients with syndromic craniosynostosis who underwent MR imaging and polysomnography. Measures encompassed the compression ratio at the level of the odontoid process and foramen magnum and the cervicomedullary angle. MR imaging studies of controls were included. Linear mixed models were developed to compare patients with syndromic craniosynostosis with controls and to evaluate the association between obstructive and central sleep apneas and MR imaging parameters. RESULTS: One hundred twenty-two MR imaging scans and polysomnographies in 89 patients were paired; 131 MR imaging scans in controls were included. The mean age at polysomnography was 5.7 years (range, 0.02-18.9 years). The compression ratio at the level of the odontoid process was comparable with that in controls; the compression ratio at the level of the foramen magnum was significantly higher in patients with Crouzon syndrome (+27.1, P < .001). The cervicomedullary angle was significantly smaller in Apert, Crouzon, and Saethre-Chotzen syndromes (-4.4°, P = .01; -10.2°, P < .001; -5.2°, P = .049). The compression ratios at the level of the odontoid process and the foramen magnum, the cervicomedullary angle, and age were not associated with obstructive apneas (P > .05). Only age was associated with central apneas (P = .02). CONCLUSIONS: The prevalence of cervical spinal cord compression in syndromic craniosynostosis is low and is not correlated to sleep disturbances. However, considering the high prevalence of obstructive sleep apnea in syndromic craniosynostosis and the low prevalence of compression and central sleep apnea in our study, we would, nevertheless, recommend a polysomnography in case of compression on MR imaging studies.

Sudden death due to pulmonary embolism as presenting symptom of renal tumors.

In 4-6% of patients with renal tumors in children intravascular infiltration is found. Tumor emboli are even rarer, and sudden death as presenting symptom has only been described at presentation in Wilms tumor (WT) in six cases so far. This report describes two recent cases of sudden death in patients with renal tumors in which a fatal pulmonary embolus was the first presentation.

[Fatal asthma in childhod preventable by recognizing risk factors and presenting features]. / Fataal astma op de kinderleeftijd te voorkomen door herkenning van risicofactoren en presentatiewijze.

A 6-year-old child known with asthma died from an asthma attack after having had severe dyspnoea which lasted for 1 day. She had been having an average of 40 salbutamol 'puffs' each day for 1 month. For the preceding 8 months she had been having just over half this number as well as fluticasone. A 13-year-old girl died of an asthma attack. Three weeks previously she had been dyspnoeic and had taken salbutamol and prednisone as well as amoxicillin at a later stage. Each year between 8 and 10 children die of an acute exacerbation of asthma in the Netherlands. There are 2 different types of acute fatal asthma: a slow type (I) and a rapidly progressing type (II). In type I there is progressive obstruction of the airways due to oedema, mucous and spasm. Type II predominantly consists of bronchoconstriction. The main risk factors are previous hospital admission with asthma and inadequate maintenance medication. Effective maintenance therapywith the correct dosage ofinhalational corticosteroids administered correctly can probably stop the potentially fatal asthma type II from developing.

[Campylobacter infections in pregnancy]. / Campylobacter-infecties in de zwangerschap.

In two pregnant women aged 39 and 35, who presented with fever and diarrhoea, Campylobacter was cultured from a blood sample. They were treated with antibiotics. One had a healthy neonate, in the other intrauterine foetal death had occurred. Campylobacter species have increasingly been recognized as possible causes of septic abortion, premature labour and neonatal sepsis. Early recognition and treatment of maternal Campylobacter infection may reduce the risk of serious foetal or neonatal complications.

Body weight in children with Turner syndrome treated with growth hormone.

OBJECTIVE: As overweight is a major concern in many children with Turner syndrome, we studied the effect of growth-promoting treatment with human growth hormone (hGH) on body weight indices. DESIGN: Longitudinal study of the effect of hGH on weight indices over time in a cohort of Turner girls of different ages. SUBJECTS: An index group of 199 hGH treated girls and a reference group of 569 untreated girls. METHODS: Turner-specific weight-for-age, weight-for-height and body mass index-for-age (BMI) values were computed. In order to take account of regression to the mean, we studied spontaneous changes of these variables in the reference group. References for spontaneous changes over 3, 6, 12 or 24 months were constructed. Observed changes in the index group were corrected by subtracting the expected spontaneous change. Corrected changes were compared between overweight, normal and underweight children. RESULTS: Treatment with hGH leads to a temporary decrease of weight indices during the first six months. This decreasing effect was not seen in overweight children. Treatment increases BMI in overweight children over 24 months, but not in normal or underweight children. BMI at start of hGH treatment did not modify long-term growth response. CONCLUSION: hGH treatment does not help to improve BMI in Turner syndrome children with a tendency to overweight.

Prevalence of penicillin-resistant viridans streptococci in healthy children and in patients with malignant haematological disorders.

The prevalence of penicillin-resistant viridans streptococci was studied in healthy children and in paediatric and adult patients with leukaemia to determine whether the frequent presence of penicillin-resistant streptococci in the oral cavity of children with leukaemia is the result of antibiotic therapy. Twenty of the oral swabs from 50 healthy children who had not received antibiotics in the three months prior to sampling yielded viridans streptococci that could be cultured on blood agar containing 2 micrograms/ml benzyl-penicillin. In 11 of the 20 cases the streptococci were resistant to penicillin (MIC > or = 4 micrograms/ml). This prevalence is significantly higher than that found in adult leukaemia patients (40% vs. < or = 5%) but is about the same as that found in paediatric patients with leukaemia. The high prevalence of penicillin-resistant streptococci in the paediatric age group should be considered when selecting therapy and prophylaxis, especially when the risk of infection with one of these cocci is enhanced.