Genetic syndromes predisposing to paediatric brain tumours: the chicken or the egg?

Cancer in childhood is a disorder of growth and development. Up to 10% of patients diagnosed with cancer during childhood have a known underlying genetic predisposition syndrome. Affected individuals usually have multisystem involvement from the underlying syndrome and certain syndromes are associated with development of characteristic tumours with sites of predilection within the neuraxis. For the healthcare professionals involved with paediatric patients it is important to have basic knowledge of the cancer susceptibility syndromes. A holistic multidisciplinary approach is required for the overall management of the syndrome itself with specific recommendations for imaging surveillance and genetic counselling based on the pattern of inheritance and the relative risk of developing a tumour. Appropriate knowledge of these syndromes will help paediatricians manage and refer patients at risk to specialist neuro-oncology centres. A typical brain tumour diagnosis can also indicate certain underlying genetic disorders and examples of such tumours include optic pathway glioma, choroid plexus carcinoma and subependymal giant cell astrocytoma. A detailed family history can be helpful in identifying at risk patients and families as the typical clinical signs associated with the genetic condition are often not fully apparent in young children. This article focuses on well-known genetic diagnoses associated with or predisposing to childhood brain tumours. In some instances, the brain tumour diagnosis subsequently leads to the diagnosis of an underlying genetic syndrome.     

What is the role of the prevention of allergy in the first period of life?

The widespread increase in the prevalence of allergic disease, which has occurred over the last 20 y, has created general concern in Europe and in the rest of the world. The reason for this increase is still partially unknown. In this period, despite a greater understanding of the pathogenesis and risk factors of allergies plus the greater efficacy of drugs to control the symptoms, the cost of intervention and the socio-economic impact are still very high. For these reasons, prevention in the first period of life represents a goal for both developed and developing countries in order to reduce this upward trend.     

Does the length of the history influence the outcome of pneumatic reduction of intussusception in children?

Purpose  

Intussusception is the most common cause of acute abdomen in infants and preschool children. Nonoperative reduction using air enema is an established treatment in children with intussusception. The aim of this study was to determine whether length of the history influences the outcome of pneumatic reduction of intussusception in children?     

In the footsteps of Frances Tustin: Further reflections on the construction of the body‐ego

The author cites material from infant observation and from work with children with autism which illustrates primitive processes of identification and their relation to the development of the body image. The sense of a skin which reliably contains the child's body is seen as being built up through the combination of eye contact with the mother, as a mediator of emotional containment, and tactile reinforcement of the child's sense of having a backbone. The author proposes the term ‘lateral object of primary identification’ to describe the baby's experience of his mother and himself each occupying half of his body. She describes the process by means of which the dual link with the mother — tactile and visual — is progressively inscribed in the major joints of the body, from the neck downwards, in the course of normal development. In this way, separation can be experienced as a loss of part of the body. The experience of losing part of the mouth, as described by Tustin, is discussed with reference to theories of containment (Bion) and projection (Green).