Fanconi anemia and primary cataracts: first case

Fanconi anemia or pancytopenia is an autosomal recessive condition presenting with a combination of pancytopenia with a mean age of onset of about eight years, a tendency to leukemia, and congenital anomalies. Although ocular abnormalities have been described, cataracts have not been previously reported. We present a patient with proven Fanconi anemia and cataracts.     

Juvenile fibromyalgia syndrome: The role for occupational therapists

Juvenile fibromyalgia syndrome, a form of nonarticular rheumatism, is a clinical condition affecting children and adolescents. To date, it has received little or no attention in the professional literature. Occupational therapists have an important role to play in the assessment and management of this paediatric client group. A literature review, the clinical symptoms, prognosis and possible aetiology of this clinical syndrome are presented. The potential role for occupational therapists with paediatric clients diagnosed with juvenile fibromyalgia syndrome is then briefly described.     

Community Outreach: An expanding role for hospitals

Abstract Pressures for paediatric institutions to expand their traditional roles have come from changes in morbidity patterns, training requirements and community needs. Planning for new models of health care delivery has led to the development of the Community Outreach Program (COP), a hospital-based model which utilizes existing community resources to provide early intervention services, training in community paediatrics and integtration of hospital and community based health care.
A process evaluation of the COP was performed after 18 months operation. Eighty-eight children were seen in a 7 week period, 20% with complex problems requiring further assessment and community consultation. Staff at the community agencies serviced considered the COP to be a major contributor to health care in their areas. Medical trainees developed new assessment techniques and management strategies for children with problems not previously encountered. It appears that the COP is a useful model for the delivery of health care, in particular to disadvantaged communities.     

Malnutrition in Belgian children with congenital heart disease on admission to hospital

? In paediatrics, body weight and height are two important growth parameters. Moreover, weight for height and height for age are useful tools for evaluation of acute and chronic malnutrition during childhood. The fact that children with congenital heart disease soon deviate from their birth percentiles for weight or height is common knowledge. However, the extent of this deviation in terms of malnutrition is less well documented. ? The aim of this study was to explore the presence and the extent of malnutrition among Belgian children with congenital heart disease on admission to hospital. A retrospective chart review of 300 children (aged 0–18 years) with congenital heart disease was conducted at a large Belgian university hospital. ? Results of this study show an increase in the frequency and severity of acute and chronic malnutrition after birth; more malnutrition in children with multiple heart defects than in those with single heart defects; more chronic malnutrition in children with heart failure, cyanosis or a combination of both and in children with additional non‐cardiac anomalies; and less malnutrition in children who had never had surgery. The prevalence of malnutrition was not influenced by sex, nationality, nor reason for admission.     

Accidents following extradural analgesia in children. The results of a retrospective study

A retrospective multicentre study of the complications observed after regional anaesthesia in children was undertaken in 1991 at the request of the association of Anesthésistes-Réanimateurs Pédiatriques d'Expression Française (ADARPEF). The incidence of accidents seen in the study was comparable to that found in the literature. Five cases which were exceptional due to the severity of the sequelae have been analysed separately. Different pathophysiological mechanisms are proposed.     

What adolescents living with long-term conditions say about being involved in decision-making about their healthcare: A systematic review and narrative synthesis of preferences and experiences

Objective

To understand the preferences and experiences of adolescents (age 10–19) with long-term conditions (LTCs) towards involvement in discussions and decisions regarding management of their condition.

Long‐term follow up of paediatric liver transplant recipients: outcomes following transfer to adult healthcare in New Zealand

Poor outcomes are reported in young people with chronic health conditions. We performed a retrospective notes review of New Zealand paediatric liver transplant recipients transferred to adult services. Two patients were lost to follow up. Out of 20, 12 were non‐adherent, and out of 12, 7 developed rejection. Other risk behaviours were common in the non‐adherent group. We conclude that dedicated services for these young people may be needed to optimise outcomes.