Ishaq Abu-Arafeh

Myocarditis and haemolytic uraemic syndrome.

A 13 year old girl is reported who presented with haemolytic uraemic syndrome (HUS) due to Escherichia coli O157:H7 infection. She died during the acute phase of the illness after an episode of unexplained sudden circulatory collapse. Postmortem examination confirmed the diagnosis of HUS and showed histological evidence of myocarditis manifested by the presence of inflammatory cell infiltration in the myocardium and around the conducting system.

Primary Headaches in Children Under the Age of 7 Years

Recurrent headache is increasingly recognised in young children. Migraine and tension-type headache feature commonly amongst the primary headache disorders seen at this age. Headaches at this age are more likely than in older patients to be ‘unclassifiable’, possibly a reflection of the difficulties in obtaining a detailed headache history from a young child. Together with recent epidemiological data this review highlights the more prevalent primary headache types with advice on making a focussed headache assessment and guidance on management in this age group.

Chronic post-traumatic headache in pediatrics

Both primary headaches and minor head injuries are common in children. If headache presents for the first time or becomes exacerbated soon after head injury it is described as post-traumatic headache (PTH). Acute PTH resolves within 3 months from injury, but chronic PTH continues beyond 3 months. The pathogenesis of PTH is not well understood. Several mechanisms were proposed such as axonal injuries and disturbances of cerebral metabolic processes. The clinical features of PTH are those of primary headache disorders such as migraine and tension-type headache and usually follow a favorable prognosis. Investigations and management should therefore be relevant to the type of headache and focused on clinical needs of the child.

3D movies and risk of seizures in patients with photosensitive epilepsy

3D television (TV) and cinema have experienced a recent surge in popularity aided in part by the success of films such as Toy Story 3 and Avatar. In parallel with this trend there have been increasing concerns about the safety of 3D TV and cinema for patients with photosensitive epilepsy. General practitioners, paediatricians and neurologists are being consulted about their opinions on the risk of triggering a seizure. Photosensitive epilepsy occurs in 1 in 4000 of the population but the incidence is higher in people aged 7-19 years. We found little evidence in the literature and confusing advice on various websites. We discuss this evidence in an attempt to clarify the advice that health professionals should be giving on this issue. We conclude that 3D cinema and television are only unlikely to trigger seizures in patients with non-photosensitive epilepsy. In young people with photosensitive epilepsy the risk of triggering a seizure is not greater with 3D TV or cinema than with conventional television, and we suggest means by which this risk can be minimised. We suggest removing 3D glasses when watching conventional TV to prevent the eyes from picking up flicker. Unfortunately there is currently insufficient evidence to draw more formal conclusions and clinical trials would be of great benefit.

Complex regional pain syndrome in children: a systematic review of clinical features and movement disorders

AIMnTo ascertain clinical features of complex regional pain syndrome (CRPS) in children with a focus on movement disorders.nnnMETHODSnall publications with original data on children with CRPS were assessed. Data were tabulated and descriptive statistics were applied.nnnRESULTSnOne population-based study and nine clinic-based studies provided data on demographic and clinical characteristics of childhood CRPS. Mean age of onset was 12.5 years and 85% of patients were females (risk ratio: 1.70; 95% CI: 1.54-1.88). History of trauma in 71% and the lower limbs were affected in 75% of patients. A secondary site involvement was present in 15%. Movement disorders and dystonia were reported in 30% of children.nnnCONCLUSIONnMajority of cases of CRPS in children are females with mean age of 12.5 years. Movement disorders (mainly dystonia) affect at least one in three children with CRPS.

Status dystonicus in children: Early recognition and treatment prevent serious complications

This is a retrospective study of all patients presenting to our paediatric unit with status dystonicus (SD) over a period of five years. Anonymous information was collected and a descriptive analysis is made. There were four episodes of SD in three children between 11 and 15 years of age. All children are known to have severe dyskinetic cerebral palsy and presented with an acute or sub-acute deterioration in their symptoms. Symptoms were triggered by infections in three of the four episodes. Early features included frequent and repetitive generalized muscle spasms, poor swallowing, poor sleep, distress and pain. Patients responded to supportive treatment, rehydration, benzodiazepines, baclofen and l-dopa. Intensive care was not necessary in any of the patients and patients made full recovery within 5-14 days. This report shows the value of early recognition and treatment of SD can be successful in preventing serious complications.

Complex regional pain syndrome in children: incidence and clinical characteristics

Objective To study the clinical and epidemiological characteristics of complex regional pain syndrome (CRPS) in children. Patients and methods All children and adolescents under 16u2005years of age with a new diagnosis of CRPS who were reported to the Scottish Paediatric Surveillance Unit were included. Patients’ recruitment ran between 1 November 2011 and 31 October 2015. Information was collected on patients’ demography, clinical features, investigations, management and impact of disease on child and family. The diagnosis of CRPS was made on fulfilling the clinical criteria of the International Association for the Study of Pain. Results 26 cases of CRPS were reported over 4u2005years, giving a minimum estimated incidence of 1.16/100u2005000 (95% CI 0.87 to 1.44/100u2005000) children 5–15u2005years of age. Nineteen patients were female (73%) and mean age at diagnosis was 11.9 (range 5.5–15.4u2005years). The median interval between onset of symptoms and diagnosis was 2u2005months (range 1–12). The majority of children have single site involvement, with legs been more often affected than arms and the right side is more often affected than the left. There was a clear trauma at onset of the illness in 19 children and possible nerve injury in one. All investigations were normal and several treatment modalities were used with variable success. The disease had significant impacts on the patients’ education and family lives. Conclusions The estimated incidence of CRPS is 1.2/100u2005000 children 5–15u2005years old. The diagnosis of CRPS is often delayed. CRPS has a significant impact on children and their families.

Chronic post-traumatic headache in children and adolescents: systematic review of prevalence and headache features

The aim of this systematic review is to determine the prevalence and clinical features of chronic post-traumatic headache (CPTH) in children and adolescents. Literature search of PubMed, Embase, Cochrane databases and Google Scholar was carried out for all studies reporting on CPTH in children and young people under the age of 18xa0years between January 1980 and November 2016. Search command included post-traumatic headache, postconcussion syndrome, child and adolescent. Demographic data, diagnostic criteria of headache disorders, occurrence of headache after head injury and headache phenotypes were collected. The prevalence of nonspecific chronic headache after head injury in children was 39% and prevalence of CPTH, as defined by the International Classification of Headache Disorders (2004), was 7.6% (95% CI: 5.9-9.7). Migraine-like headache was the most common presentation followed by tension-type headache. Other children had either mixed or unclassified headache disorders.

Migraine with atypical aura: is there a role for cerebral perfusion studies?

lived experience in the natural environment and clarifies the meaning and perceptions of people. Professionals who wish to use a specific PROM need to evaluate whether the content of the measure originates from a comparable population of interest, and be suspicious about the content validity if this information is absent or poorly described. Self-reported health and functioning (including mental health), QoL, and subjective well-being are examples of patient-reported outcome constructs. Each is a distinct entity that overlaps and interacts with the others. In populations with chronic conditions, health can be conceptualized positively – not simply as freedom from all disease but also whether the person is functioning well, can do what they want to do, and can become what they want to become. The World Health Organization’s (WHO) International Classification of Functioning, Disability and Health (ICF) encourages healthcare professionals to recognize the biopsychosocial dimensions of health beyond the level of impairment, and to address the experiences, relationships, and views of patients. The ICF concept of participation has gained traction, as evidenced by the development of a number of participation PROMs. Subjective well-being is a broad concept that includes autonomy, environmental mastery, personal growth, positive relationships with others, purpose in life, and self-acceptance. On the other hand, QoL refers to the individual’s perceptions of their position in life in the environment in which they live and in relation to their goals, expectations, and concerns, as suggested by the WHO. In children, QoL also varies with the stage of development and the illness trajectory. Many QoL instruments include the domains of well-being. We now recognize that it is difficult to attribute better or poorer health, QoL, or well-being solely to the biological aspect of a disease and its medical treatment – an observation that has been coined ‘the disability paradox’. How can we choose an appropriate measure for specific patients and be able to interpret the results accurately? Reviews have consistently detailed the actual content of many generic and condition-specific PROMs, some using the WHO definitions of QoL and biopsychosocial functioning. Several papers also identify the specific ICF components present in each measure. However, because most of the studies reporting PROs are cross-sectional, we don’t yet know which PROMs are able to detect meaningful change in health and QoL over time in relation to our interventions. Assessing change is a challenge still to be addressed effectively.

Fractures And Bone Health Monitoring In Boys With Duchenne Muscular Dystrophy Managed Within The Scottish Muscle Network

There are limited reports of radiologically confirmed fractures and bone health monitoring in with Duchenne muscular dystrophy. We performed a retrospective study of 91 boys, with a median age of 11.0 years, who are currently managed in Scotland with the aim to assess the frequency of radiologically confirmed fractures and report on bone health monitoring in relation to International Care Consensus Guidance. Of these boys, 59 (65%) were receiving glucocorticoid (GC) therapy and 23 (25%) had received previous treatment. Of those currently on GC, 37 (63%) had an assessment of bone mineral density and none had routine imaging for vertebral fractures during the study period. Of the 91 boys, 44 (48%) had sustained at least one symptomatic radiographically confirmed fracture. The probability of sustaining a first symptomatic fracture was 50% by 12.8 years old (95%CI: 12.1, 13.6). The most common sites for non-vertebral fracture were the femur and tibia. In this review of boys with DMD, almost half had sustained at least one radiologically confirmed symptomatic fracture. There is a need for standardized bone health monitoring in DMD that includes routine imaging of the spine to identify vertebral fractures, given the persistence of insult to the skeleton in these boys.