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Potential of three-dimensional ultrasound in neonatal and paediatric neurosonography.

The aim of this study was to describe the potential of three-dimensional ultrasound (3D US) in paediatric and neonatal neurosonography. The potential applications are illustrated based on our experience in 150 patients using three different 3D US techniques at two different sites. Various disease entities throughout the paediatric age have been evaluated. The potential of 3D US, including 3D US of the cerebral vessels based on colour Doppler data, is discussed based on comparison with conventional 2D US or other imaging (as available), and with regard to the literature. In our experience, 3D US is feasible in neonatal and paediatric neurosonography. It reduces imaging time, improves demonstration of complex anatomy and vasculature, and allows for evaluation of anatomy/pathology in any plane. The 3D US furthermore improves volume assessment (e.g. in hydrocephalus), and comparison with CT, MRI and during follow-up, with a potentially improved standardisation and documentation. The 3D US additionally offers an ideal modality for training and education, as the brain and the neonatal spine can be virtually rescanned at the workstation. Yet, limitations such as areas inaccessible to 2D US, limited resolution and motion artefacts have to be acknowledged. Three-dimensional US has the potential to become a valuable additional imaging tool in paediatric neurosonography.

Brain↗

Revascularisation surgery for paediatric moyamoya: a review of the literature.

INTRODUCTION AND AIMS: The role of and optimal surgical revascularisation technique for paediatric moyamoya syndrome (MMS) are controversial. In this literature review our primary aim was to evaluate the evidence base for the efficacy of surgical revascularisation for the treatment of paediatric MMS. Secondary aims were to estimate the rate of peri-operative complications and to ascertain whether direct or indirect revascularisation techniques resulted in differences in clinical or radiological outcomes. METHODS: Papers describing surgical revascularisation and its outcome in the treatment of children with MMS were identified from the OVID Medline database (1966-2004). Only papers in English were reviewed. Data were abstracted using a standardised form. RESULTS: Fifty-seven studies, including data on 1,448 patients, were reviewed. Most were Japanese; 10% were from Western institutions. Indications for revascularisation were described in <15% of studies and varied between centres. Indirect procedures were most commonly performed (alone in 73% of cases, combined with direct procedures in 23%). The rates of peri-operative stroke and reversible ischaemic events were 4.4 and 6.1% respectively. Out of 1,156 (87%) patients, 1,003 derived symptomatic benefit from surgical revascularisation (complete disappearance or reduction in symptomatic cerebral ischaemia), with no significant difference between the indirect and direct/combined groups. Data on developmental and functional outcomes were limited and of uncertain significance as they were not related to pre-operative status. Good collateral formation was significantly more frequent in the direct/combined group than in the indirect group (chi(2), p<0.001). CONCLUSIONS: Data from the medical literature suggest that surgical revascularisation is a safe intervention for paediatric MMS and most treated patients derive some symptomatic benefit. However, paucity of data on selection criteria and more global outcome measures means that the impact of surgical revascularisation on natural history remains uncertain. Direct and/or combined procedures provide better revascularisation, but this is not associated with differences in symptomatic outcome. International standardisation of the clinical approach to the treatment of paediatric MMS is urgently needed to critically evaluate the optimal indications for and timing of surgical revascularisation.

Cerebral Revascularization↗

Tele-echocardiography in paediatrics.

UNLABELLED: Echocardiographic images can be transmitted over increasing distances with less cost and better quality thanks to advances in the field of telecommunications. This technological support can be used to detect heart defects in newborns and children in remote situations. The intent of this study was to confirm the feasibility and usefulness of telemedical communication for echocardiographic evaluation of paediatric cardiovascular disease. A total of 214 echocardiographs were performed in 194 children at a remote hospital by an experienced sonographer in paediatric echocardiography. These echocardiograms were transmitted to a distant tertiary care paediatric cardiology centre using a telemedicine link across three ISDN lines. There an experienced paediatric cardiologist interpreted the tele-echocardiograms. Tele-distant diagnoses were prospectively documented and compared with the diagnoses made subsequently on direct consultation and echocardiography. The quality of transmitted echocardiographic images was sufficient for evaluation except for one case. In 191 children (98%), the remote echocardiographic diagnosis was correct as confirmed by follow-up face to face consultations. Three cases were diagnosed incorrectly. CONCLUSION: our results confirm that accurate and rapid diagnosis can be provided by tele-echocardiography in neonates and children. This facilitates the appropriate care of these patients as expensive and potentially dangerous long-distance transfers can be avoided.

Adolescent↗

What the paediatricians need--the launch of paediatric research in Europe.

UNLABELLED: Most parents and many paediatricians are not aware that medicines for children are often not tested, labelled, or approved for their prescribed use. In 1997, the Food and Drug Administration developed incentives for pharmaceutical companies to perform paediatric research in medications. The European Commission brought out its proposal for paediatric incentives after in-depth internal reflection. Now it should be adapted quickly to boost pharmaceutical paediatric research in the EU as soon as possible. CONCLUSION: The proposal must be balanced and aware that both incentives and requirements are mandatory in order to maintain and stimulate paediatric research on medicines in Europe.

Child↗

An immunohistochemical study of extracellular matrix proteins laminin, fibronectin and type IV collagen in paediatric glioblastoma multiforme.

AIMS: In the recent decades many studies have been addressed in the literature to assess specific factors related to glioblastoma multiforme (GBM) invasion. However, few studies have evaluated tumour cell's interaction with specific extracellular matrix (ECM) components, and, moreover, there is a lack of information regarding the occurrence of these phenomena in paediatric GBM. METHODS AND RESULTS: ECM proteins were evaluated in six cases of paediatric GBM assessing the immunohistochemical expression of laminin, fibronectin, and type IV collagen. We used a semiquantitative scale, ranging from not detected (zero) to marked (3). Laminin expression was minimal in three cases, moderate in one case, marked and generalised in one patient and marked and focal in the last case. Fibronectin expression was minimal in three patients; moderate immunoreactivity was documented in one case. Conversely, one case was classified as marked with generalised distribution and the remaining case as marked with focal immunostaining. Type IV collagen expression was minimal in three cases, moderate in one, marked with focal reaction in one and marked with generalised reaction in the remaining case. CONCLUSIONS: This study provides additional insights into tumour invasion features of paediatric GBM, as ECM plays a pivotal role in numerous cellular functions during normal and pathological processes. Although based on a limited number of patients, this investigation may serve as a challenge for the management of paediatric GBM, stimulating trials with larger patient numbers aimed at documenting specific factors influencing GBM prognosis.

Biomarkers, Tumor↗

Serodiagnosis of typhoid fever in paediatric patients by anti-LPS ELISA.

Enzyme-linked immunosorbent assay (ELISA) and immunoblot analysis showed that up to 50% of the anti-typhoid antibody in sera from blood culture positive paediatric typhoid fever patients is directed against lipopolysaccharide (LPS) antigen. Anti-Salmonella typhi LPS ELISA was therefore compared to Widal agglutination for serodiagnosis of typhoid fever in paediatric patients. Sera from 38 paediatric control individuals were ELISA negative for anti-S. typhi LPS IgG; all but 2 of these specimens were negative for anti-S. typhi LPS IgM. Paediatric patients hospitalized with signs and symptoms of typhoid fever were separated into 4 groups and tested by ELISA with the following results: 46 patients negative by both culture and Widal agglutination tests, 48% positive for anti-S. typhi LPS IgG and 35% for anti-S. typhi LPS IgM; 22 negative by culture but with positive Widal titres, 82% and 68% positive respectively; 28 culture positive for S. typhi, 93% and 82% respectively; and 12 culture positive for Salmonella other than S. typhi, 92% and 92% respectively. These data suggest that anti-S. typhi LPS ELISA is a suitable assay for diagnosis of typhoid fever in children.

Adolescent↗

Humanizing paediatric care.

This paper deals with the development of a paediatric team approach to the care of children in the 70 bed paediatric unit in the Royal Hobart Hospital in Tasmania, Australia, and describes the measures used to help children and their parents adapt to life in hospital with a limited budget. The staff includes paediatricians and pediatric surgeon, resident staff, nurses, teachers, social worker, physiotherapist and pharmacist. There are no visiting hours but the wards are open to parents and relatives at any time. Parents are encouraged to stay in hospital and their accommodation is either in flats at the end of wards or in single or double bed wards using foldup beds. Each young child has a personal details form completed and placed on the end of the cot allowing a nurse to discover likes, dislikes, methods of feeding and sleeping, etc. Parents are asked to complete questionnaires at the time of the child's discharge, asking for their assessment of the standards of care their child received. Playleaders are rostered for evenings and weekends and they work with, and direct Red Cross volunteer ladies. Two full-time teachers cooperate with the child's school in maintaining the child's educational progress. Volunteer visitors entertain children whose relatives are unable to visit or stay in hospital. The methods used to raise the standards of paediatric nursing are also described. Community groups, particularly kindergarten and primary school children, are encouraged to visit the paediatric unit and each group is usually given a brief health education talk by a member of staff.

Adolescent↗

[Evaluation of varicella complications through a retrospective hospital survey in a paediatric center over 16 years in France].

OBJECTIVE: Evaluation of the varicella severity through a prevalence study of hospital admissions justified by a complication directly related to the onset of an acute episode of varicella. METHODS: Retrospective study in one paediatric center in France with a follow-up of a paediatric cohort from April 1987 to December 2002. This general paediatric hospital recruits children from a 400,000 inhabitants area. Inclusion criterion: diagnosis main or associated of varicella. EXCLUSION CRITERIA: congenital or acquired immunodepression, including long-term oral high dosage steroid therapy. RESULTS: Three hundred and forty-three (343) complications of varicella were reported in 309 children hospitalised for a symptom in relationship with varicella. Most of children (75%) were <2 years of age. The annual number of hospitalisations varied with a maximum of 44. An increase of the number of hospitalisations was reported since 1997 but particularly since 2000. Main complications were gastro-intestinal (75), neurological (68), bronchopulmonary (52), and skin and soft tissue infections (52). Superinfections of soft tissues such as Streptococcus pyogenes cellulitis and life-threatening complications, which occurred in children treated by a short-course of corticosteroïds for an acute episode such as an asthma crisis, were mostly noticed since 1995. Two deaths occurred. CONCLUSION: These data collected over 16 years as part of a retrospective survey of a paediatric cohort show that varicella, often considered as a mild disease, can be responsible for severe complications in young immunocompetent children. The digestive complications (30%) are the main complication in our study with existence of gingivitis-stomatitis but also lower digestive manifestations (erosive gastritis aspect through endoscopy). These data have all the more to be taken into account because a vaccine, developed for healthy children, is now available in France. A national survey of hospitalised varicella has been set up since March 2003.

Adolescent↗

Paediatric idiopathic inflammatory muscle disease.

The paediatric idiopathic inflammatory myopathies (IIMs) are a group of rare but serious systemic autoimmune conditions of childhood. The most common of the paediatric IIMs is juvenile dermatomyositis (JDM), while polymyositis and inclusion body myositis are rare in children. JDM has a significantly different spectrum of disease from adult dermatomyositis. Juvenile myositis can also occur as part of other systemic autoimmune diseases such as scleroderma and systemic lupus erythematosus. There has recently been significant progress towards the development and validation of tools to measure disease activity and damage in the paediatric IIMs. In addition, several new therapeutic avenues have been used to treat JDM. This review will discuss developments in the diagnostic criteria for JDM, the clinical types and course of these conditions, recent progress in disease assessment, treatment options and new developments in research into the pathogenesis of paediatric IIM.

Child↗

Paediatric rheumatology--a global perspective.

This chapter aims to give a global perspective to paediatric rheumatology. The main points covered are the incidence, recognition of paediatric autoimmune diseases, and ethnic/geographic distribution. The most prevalent disease is juvenile idiopathic arthritis; robust data are still required for childhood-onset systemic lupus erythematosus, dermatomyositis, and scleroderma. Mimicking or overlapping infections are a major challenge in developing countries, and immunization policies in our patients in these areas need specific attention. The delivery of paediatric rheumatology care is also overviewed. Discrepancies in health-care resources and priorities are found in developing countries. Although most anti-rheumatic treatments are available worldwide, they are prohibitively expensive in many countries. For more traditional anti-rheumatic drugs there is still an ongoing need for good core outcome data across the world to ensure valid comparisons. Parent/patient education has been implemented worldwide in paediatric rheumatology through the power of the Internet. Physician and undergraduate training goals must be met to facilitate competent musculoskeletal assessment, a proper understanding of age-dependent variations, diagnosis, referral to specialists, and improved standards of care.

Age Factors↗

A global study of hospitalized paediatric burn patients.

The global incidence of hospitalized paediatric burn patients is unknown. In order to determine a working estimate of the size of the problem, one approach is to extrapolate from relevant published studies. A literature search of the Medline database was performed to identify epidemiological papers published since 1990, which addressed paediatric burn admissions. Extrapolation from population-based studies allowed global figures to be estimated. The highest incidence of hospitalized paediatric burns patients is in Africa, the lowest in the Americas. Europe, the Middle East and Asia share similar figures, but the considerably larger population of Asia indicates that it bears over half of the world's paediatric burn population. It is unfortunate that despite many published studies describing burn admission, few meet criteria that allow for comparative epidemiological data. More attention needs to be focused on uniformity in data collection and presentation to enhance the value of the data available.

Burns↗

Taxanes in paediatric oncology: and now?

Taxanes are a group of anticancer agents that target microtubules, promote their assembly, and stabilize them. The resulting effects are mitotic blockage and induction of apoptosis, whose mechanism of initiation remains to be fully determined. Among adults, taxanes are one of the most powerful and most commonly used anticancer drugs. They show a wide range of activity in malignancies such as breast, ovarian, and lung cancers. In paediatric oncology, pre-clinical and clinical data are quite limited. Six phase I studies, two phase II, and rare case reports have been published. This review will focus on the paediatric pre-clinical and clinical findings with taxanes. Based on data from the literature, we will try to explain the results and foresee the possible use of taxanes in paediatric oncology. Indeed, three main advances have been made regarding taxanes: (1) Increased efficacy when used in multidrug-based chemotherapy regimen. (2) New administration schedules based on anti-angiogenic properties when given at daily very low doses. (3) Development of new taxanes with decreased recognition by Pgp, the product of the MDR1 gene. Thus, although the initial experience with taxanes in paediatric oncology has been disappointing, it may be too early to leave out taxanes and we should further investigate their use among children with cancer.

Antineoplastic Agents, Phytogenic↗

Continuous EEG monitoring in a paediatric intensive care unit.

Monitoring with continuous EEG (cEEG) has become a valuable tool in the adult neurointensive care unit. The benefits of cEEG or amplitude-integrated EEG in neonatal intensive care have also been described. The aim of the present study was to describe and evaluate the use of cEEG in a paediatric intensive care unit. The study is a description of children and adolescents with acute neurological disorders monitored by cEEG in a paediatric intensive care unit for more than 12h. The indication for cEEG and the outcome are reported for 54 patients during a 4-year period. Twelve patients were monitored for high intracranial pressure, eight of whom died. Fourteen were monitored due to suspected, but not detected, epilepsy, their underlying diagnoses being variable. Refractory status epilepticus was the reason for cEEG in 24 cases. All of these patients survived the acute phase of status epilepticus. Four patients had seizure activity on cEEG due to global anoxia; these were not classified as status epilepticus. In conclusion, in the paediatric intensive care unit the most important indication for cEEG monitoring is in patients with suspected refractory status epilepticus where it adds to the diagnosis and choice of treatment. Continuous EEG should therefore be part of the paediatric intensive care unit technical support to select and monitor, among children with critical neurological disorders, those with refractory status epilepticus.

Adolescent↗

Transition from paediatric to adult endocrinology: hypopituitarism.

For patients at the transition stage between childhood and adulthood, cooperation between paediatric and adult endocrinologists is essential for optimum care. Evaluations of patients in transition consist of retrospective assessments of what happened in childhood combined with prospective planning for treatment and follow up into adult life. Successful transfer of patients in transition from paediatric to adult care should follow a general sequence tailored to local circumstances. First, all patients with GH deficiency and their families should be informed by their paediatric endocrinologist about the long term consequences of GH deficiency in adulthood and the potential need for life-time GH replacement therapy. Second, retesting will be needed for evaluating hypothalamic pituitary function, re-evaluation of the need for replacement therapy for other pituitary hormone deficits, measurement of fasting lipid concentration and assessment of skeletal integrity. Third, the patient and physicians should consider the option of attending an out-patient clinic having both a paediatric and an adult endocrinologist. Regardless, the wishes of the patient should be respected as much as possible. It is essential that those patients who had childhood-onset GH deficiency and who were treated with GH be followed throughout adult life.

Adolescent↗

Development and implementation of a multi-centre information system for paediatric and infant critical care.

BACKGROUND: With no UK collective information system, a need existed to establish an integrated information system for public and private sector hospitals providing paediatric and infant critical care services. A lack of information in the past made it difficult for those procuring, providing and monitoring services to make informed, evidence-based decisions using reliable integrated data. OBJECTIVES: To develop and implement a collective multi-purpose information system for paediatric and infant critical care that was easily adaptable to any UK infant or paediatric critical care setting. Information outputs had to fulfil policy requirements and meet the needs of stakeholders. METHOD: Two minimum datasets, corresponding data definitions, survey forms and a user database were developed through a process of consultation by utilising an information partnership. Design, content, development and implementation issues were identified, discussed and resolved through a co-ordinated collaborative process. RESULTS: Data collection was implemented in all London and Brighton National Health Service (NHS) general and cardio-thoracic paediatric intensive care (PIC) units, several private PIC units and one NHS tertiary referral neonatal unit (NNU) 24 months from project start. CONCLUSIONS: The development of universal integrated information systems for defined settings of care is achievable within reasonable timeframes; however, successful development and implementation requires working within an information partnership to maximise co-ordination, co-operation and collaboration. Those collecting and using data must be identified and involved in all aspects of development from project start. Financial and manpower resources must be well planned. Datasets should be as small as possible in order to make the collection of complete and valid data realistically achievable. When considering service-based information needs, considerable thought should be given to a multi-purpose; multi-use approach based on the most refined minimum dataset possible.

Child↗

Ethnicity and mutations in GJB2 (connexin 26) and GJB6 (connexin 30) in a multi-cultural Canadian paediatric Cochlear Implant Program.

OBJECTIVE: To determine the relationship between ethnicity and mutations in the GJB2 and GJB6 genes in multi-cultural patients enrolled in a Canadian paediatric Cochlear Implant Program. METHODS: Blood was analyzed from 65 paediatric cochlear implant users by direct sequencing of the coding region and intron/exon boundaries of the GBJ2 gene. Individuals heterozygous for one mutation in GJB2 or in whom mutations in GJB2 were not detected were analyzed for the common 342 kb deletion mutation D13S1830 in the GJB6 gene. Information regarding ethnicity of patients' families was obtained from patient records and/or interview. RESULTS: GJB2 mutations were found in 36.9% of paediatric cochlear implant users tested. Nine different GJB2 mutations were identified among individuals from 14 different countries of origin. Seventy-eight percent of all identified pathogenic GJB2 mutations were 35delG. Biallelic GJB2 mutations were found in 16 cochlear implant users (66.7% of GJB2 mutations). Three novel GJB2 sequence changes were identified: (1) a missense mutation T107C (L36P) in an individual of African decent; (2) a missense mutation G475T (D159Y) in an individual of Caribbean decent; (3) a regulatory region change 1-34C to T in an individual of African decent. GJB6-D13S1830 mutations were not found in any of the patients tested. Individuals of African, Caribbean and East Indian decent had different GJB2 mutations than the remainder of individuals tested. Patients of Asian, Italian, Spanish, Polish and Armenian decent were not found to carry mutations in GJB2 or the common GJB6-D13S1830 mutation. CONCLUSIONS: This study represents the largest number of biallelic GJB2 mutations isolated in a group of paediatric cochlear implant users to date. Numerous and diverse GJB2 mutations were found in this multi-cultural group of children. Even though GJB2 mutations have been widely reported in the literature, this discussion represents the first report of GJB2 mutations in a multi-ethnic population (Canadian), as compared with previous studies that investigated fairly homogeneous populations. The diversity of GJB2 mutations identified reinforces the importance of testing for changes in GJB2 by direct sequencing of the entire coding region rather than testing only for common mutations.

Canada↗

Pain management in paediatric trauma patients with long bone fracture.

OBJECTIVES: This study was done to review and describe the care of paediatric trauma patients with respect to pain assessment and medication administration. METHODS: A retrospective review of paediatric trauma patients, age <16 with a long bone fracture and GCS=15, cared for by our paediatric trauma response team (January 1998-August 2002). A single trained abstractor reviewed all records. Data were descriptively analysed. RESULTS: Fifty-six children were included. All but three received pain medication during resuscitation. The median time to first dose of pain medication after arrival was 20 min (95% CI: 14-29 min). The median pre- and post-treatment pain scores, on a 5-point scale, were 4 and 2, respectively. Vital signs were unaffected. CONCLUSIONS: As a group, our paediatric trauma resuscitation team did a much better job managing pain, in this segment of the population, than the preponderance of existing literature would predict.

Acute Disease↗

Can CT determine the site of traumatic osteochondral defects in the paediatric knee?

Currently the ability of pre-operative CT imaging to determine the origin of traumatic osteochondral lesions (OCL) in the knee in children is yet to be established. The surgical approach to the knee will to some extent be determined by the origin of the lesion. It is important to directly determine the site of the lesion from pre-operative scanning both to facilitate surgery, to have a better cosmetic result for the patient and have a quicker rehabilitation period. In a tertiary referral centre, from May 2004 to April 2005, eight patients were diagnosed as having an OCL. The initial reporting was done by either a senior registrar or consultant paediatric radiologist. Those children that had an OCL underwent an arthroscopy or definitive open surgery. The exact site of the lesion was then determined and recorded in the operative notes. All the original pre-operative CT scans were given to a senior paediatric radiologist. The consultant on this occasion had no access to operative findings, or original CT reports. CTs reported by the paediatric radiology department are only able to correctly identify the site of origin of the OCL 50% of the time. Recent MR scanning techniques have improved the visualization of OCL. We authors therefore feel that in the future MRI should be used to assess the paediatric knee when an acute OCL is suspected.

Acute Disease↗