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Determination of 5-methyltetrahydrofolate in cerebrospinal fluid of paediatric patients: reference values for a paediatric population.

BACKGROUND: Cerebral folate deficiency (CFD) has been described as a neurological syndrome associated with low 5-methyltetrahydrofolate (5-MTHF) values in cerebrospinal fluid (CSF) with normal folate concentrations in plasma. Our aim was to analyse CSF 5-MTHF concentrations in a paediatric control population and in patients with various neurological disorders. METHODS: We studied plasma and CSF samples from 63 paediatric controls (age range: 2 days to 18 years, average: 3.8 years) and from 165 patients (age range: 1 day to 22 years, average: 5.0 years) with severe epileptic encephalopathies of unknown origin, movement disorders, Rett syndrome and mitochondrial diseases. CSF 5-methyltetrahydrofolate was analysed by reverse phase HPLC with fluorescence detection (excitation: 295 nm and emission: 355 nm). RESULT: A negative correlation between 5-MTHF values and age of controls was observed (r=-0.468; p<0.0001) and reference values were therefore stratified into 3 age groups. Regarding patients, 122 out of 165 showed normal CSF 5-MTHF values while 43 showed decreased values ranging from profound to mild deficiencies. Increased CSF total protein values were associated with the presence of low 5-MTHF concentrations (chi(2)=7.796; p=0.005). CONCLUSIONS: The application of this method has been useful for the establishment of reference values and for diagnosis of CFD in paediatric patients. Furthermore, increased CSF total protein concentrations should be considered as a marker of a possible CFD.

Adolescent↗

The European Resuscitation Council's paediatric life support course 'Advanced Paediatric Life Support'.

The poor outcome for resuscitation from cardiopulmonary arrest in childhood is widely recognised. The European Resuscitation Council has adopted the Advanced Paediatric Life Support course (originating in the UK and now available in a number of countries) as its course for providers caring for children. This paper outlines the course content and explains its remit, which is to reduce avoidable deaths in childhood by not only resuscitation from cardiac arrest but, more effectively, by recognising and treating in a timely and effective fashion life-threatening illness and injury in infants and children. Two related courses Paediatric Life Support, a less intense course for less advanced providers, and Pre-Hospital Paediatric Life Support for immediate care providers are also described.

Advanced Cardiac Life Support↗

Diagnostic accuracy of paediatric echocardiograms interpreted by individuals other than paediatric cardiologists.

OBJECTIVE: To assess the diagnostic error rate among echocardiograms undertaken by individuals other than paediatric cardiologists in our referral area. METHODOLOGY: External group: The charts and echocardiographic results of all patients who had undergone outside echocardiograms between January 1996 and December 1999 were reviewed (110). Age at echocardiography, diagnostic complexity, presence of any diagnostic errors and the severity of any diagnostic errors were identified. Internal group: To assess our own error rate, the initial echocardiographic diagnoses of 100 patients undergoing cardiac catheterisation or corrective surgery were compared with the post-catheterisation or postoperative diagnoses. Age and diagnostic complexity were also assessed in the control group. RESULTS: Diagnostic errors occurred in 47/110 patients (44%) of the externally studied group (of which 24% were either major or life threatening) as opposed to 3/100 of the internally studied group, despite the internally studied group being of increased diagnostic complexity. Errors were more common and of increased severity in infants less than 1 month of age but extended throughout all age groups. Major and life threatening errors increased with increasing diagnostic complexity. In the externally studied group, 8/47 errors were patients inappropriately designated as normal. Four of these patients required cardiac surgery or interventional cardiac catheterisation. CONCLUSIONS: This study suggests an unacceptably high error rate in paediatric echocardiographic diagnoses by non-paediatric cardiologists throughout all age groups. Such errors are more likely in younger infants and with increasing diagnostic complexity.

Age Distribution↗

Quality of care: a tracer diagnosis study of acute otitis media, comparing a district paediatric service with paediatric and otolaryngology emergency departments.

Acute otitis media was used as a tracer condition for comparing patient care as regards patient satisfaction, compliance and medical outcome in a district paediatric office, an otolaryngological and a paediatric hospital emergency department. The initial work-up of the 216 patients studied at the three units was in good agreement with predefined standards. However, while the district paediatrician arranged a follow-up for all patients, the hospitals did so only in 10--30% of the visits. Parent satisfaction was significantly higher at the district office when the time spent waiting and the time the patient was with the doctor were kept constant. Urinary penicillin was not detected in 1/17 of district paediatric and in 6/19 of otological patients. Compliance with follow-up was also much higher at the district paediatrician. Medical outcome did not differ between the units. The majority of the parents expressed preference for the type of care given in a neighbourhood service. It is suggested that the criteria for adequate treatment of acute otitis media adapted for emergency departments may be revised in case easy access is provided to a medical centre offering continuity of care and proper follow-up. Studies of the quality of care have so far not managed to demonstrate a definite and positive relationship between the process of care, patient satisfaction and final medical results.

Adult↗

Guidelines for paediatric life support. Paediatric Life Support Working Party of the European Resuscitation Council.

The paediatric life support working party of the European Resuscitation Council was set up in 1992 with the aim of producing guidelines for basic and advanced paediatric resuscitation that would be acceptable throughout Europe. The commonest cause of cardiac arrest in children is problems with the airway. The resulting difficulties in breathing and the associated hypoxia rapidly cause a severe bradycardia or asystole. In contrast, adults have primary cardiac events resulting in ventricular fibrillation. This important difference in the pathogenesis of paediatric and adult cardiac arrest is reflected in these European Resuscitation Council guidelines, which complement those already published for adults.

Airway Obstruction↗

Ethical principles and operational guidelines for good clinical practice in paediatric research. Recommendations of the Ethics Working Group of the Confederation of European Specialists in Paediatrics (CESP).

A child has the full right of protection of his/her life by provision of optional medical care. There is a need in paediatrics for better evidence based practice founded on quality research into efficacy and safety of children's medications. To protect the best interests of the child one must balance the ethical demand to do clinical studies with the necessity to avoid doing harm. To achieve this end good clinical practice in paediatric research demands that studies comply with the Declaration of Helsinki, ICH topic E11, EU Directives and other relevant international guidelines. Evident differences in physiology, pharmacology, pharmacokinetics and pharmacodynamics between children of differing ages and between children and adults demand properly constructed and conducted studies that respect the special somatic, emotional and mental needs of children. To justify any research project one must balance the benefit/risk ratio, provide experienced, competent personnel and infracture, obtain adequate informed consent/assent, and have the study evaluated and approved by an ethics committee containing expertise on the rights and needs of children.

Adolescent↗

A radiopharmaceuticals schedule for imaging in paediatrics. Paediatric Task Group European Association Nuclear Medicine.

There is no standard schedule for the amounts of radiopharmaceutical administered to children at present. Various alternative methods are currently in practice, all with some support from the literature. Optimization of the amount of radiopharmaceutical to be administered is discussed in relation to the radiation burden of such an examination. The Paediatric Task Group of the European Association of Nuclear Medicine has investigated the current situation and now suggests a method for standardization of administered amounts of radiopharmaceutical in paediatrics. The fraction of the adult amount of radiopharmaceutical to be administered should be calculated from the child's body weight according to the accompanying table. In small infants, the minimum amount of a radiopharmaceutical is discussed and suggested amounts to be administered are presented.

Body Weight↗

Azithromycin versus penicillin V in the treatment of paediatric patients with acute streptococcal pharyngitis/tonsillitis. Paediatric Azithromycin Study Group.

The efficacy and safety of azithromycin and penicillin V in the treatment of acute streptococcal pharyngitis/tonsillitis in paediatric patients were compared in a double-blind, double-dummy prospective study. A total of 489 children (age range, 2-13 years) were randomized to receive treatment with penicillin V (125-250 mg 4 x daily for 10 days) or azithromycin in an oral suspension (10 or 20 mg/kg 1 x daily for 3 days). Only patients with baseline cultures positive for Streptococcus pyogenes and complete clinical and microbiological assessments at the end of the therapy and follow-up one month later were included in the efficacy analysis. A satisfactory clinical response (cure or improvement) was recorded in 99% of the 10 mg/kg azithromycin group, 100% of the 20 mg/kg azithromycin group, and 97% of the penicillin V group at the end of therapy (day 12-14). At the follow-up evaluation (day 28-30), relapse rates in patients cured or improved at the end of therapy were 6%, 5%, and 2%, respectively. Bacteriological eradication rates at the end of therapy were 98% in both azithromycin groups and 92% in patients who received penicillin V (p = 0.011); pathogen recurrence was recorded at follow-up in 4% of the 20 mg/kg azithromycin group and in 6% of both the 10 mg/kg azithromycin and penicillin V groups. Treatment-related adverse events, the majority of mild to moderate severity, occurred in 13% of patients in the 20 mg/kg azithromycin group, 9% in the 10 mg/kg azithromycin group, and 5% in the penicillin V group. Azithromycin in a dosage of 10 or 20 mg/kg/day one daily for three days was as safe and effective as penicillin V administered four times daily in the treatment of paediatric patients with acute pharyngitis/tonsillitis.

Administration, Oral↗

Current evidence for the use of paediatric antiretroviral therapy--a PENTA analysis. Paediatric European Network for the Treatment of AIDS Steering Committee.

UNLABELLED: The introduction of combination antiretroviral therapy has been associated with a dramatic clinical improvement in children with human immunodeficiency virus infection. However, the uptake of antiretroviral therapy has been variable across Europe. The Paediatric European Network for the Treatment of AIDS Steering Committee has performed a systematic literature review of paediatric antiretroviral therapy trials. An analysis of the evidence base for the commencement and maintenance of antiretroviral therapy was produced. Suggestions for when to commence antiretroviral therapy, which drugs to start with and how to monitor and sequence drug regimens are given. CONCLUSION: The aim of these guidelines is to help in obtaining equity of access to a uniformly high standard of care for children with human immunodeficiency virus infection in all European countries.

Acquired Immunodeficiency Syndrome↗

[Epidemiological and clinical aspects of paediatric HIV infections in Albert-Royer Paediatric Hospital (Dakar, Senegal)].

UNLABELLED: Human Immunodeficiency Virus (HIV) infection prevalence rate is estimated at 1.4% in Senegal, and about 3,000 children could be infected. HIV positive children are followed up since 2000 in Albert Royer Hospital (Dakar, Senegal). OBJECTIVES: To describe clinical and epidemiological aspects of HIV paediatric infection, and to evaluate the implementation of high active antiretroviral therapy in HIV positive children in our country. POPULATION AND METHODS: Over a period of three years, the medical reports of 98 infected patients have been collected, 96% with HIV 1 infection. RESULTS: Most of the patients had a maternally transmitted HIV infection (99%). At their enrollment, the median age was 60 months; malnutrition (79%), persistent lymphadenopathy (65%) and skin lesions (64%) were the common clinical manifestations. Thirty-nine percent of the patients were in class C (CDC) and 81% had CD4 cell count< or =25%. Median viral load were 421,852 copies/ml at presentation. Seven infants had a rapid progressive disease with encephalopathy. Thirty-six patients received high active antiretroviral therapy with high observance and good tolerance. CONCLUSION: This study allowed to define clinical and biological profile of paediatric HIV infection in our country and to update the implementation of high active antiretroviral therapy.

Antiretroviral Therapy, Highly Active↗

Are paediatric burns more common in asylum seekers? An analysis of paediatric burn admissions.

The number of asylum seekers in Ireland has increased dramatically over the last 10 years. Based on our impression that the number of children admitted to our burn unit was disproportionately represented by children of asylum seekers we performed an audit to establish (1) what proportion of admissions are from this subgroup and (2) the characteristics of their burns. All paediatric burn admissions from May 2003 to April 2004 were reviewed. Data collected from a retrospective chart review included patient demographics and details of the burn injuries. The National Census of 2002 and the Office of the Refugee Applications Commissioner were consulted for population statistics. Total burn admissions for the period were 126: Irish nationals (n=107), non-national residents (n=2), asylum seekers (n=14) and patients of unknown asylum status (n=3, excluded from study). In the asylum seeker group, the median age was 18.6 months (range 10 months-5.3 years) with the majority less than 2 years (n=11). All burns occurred in the domestic setting. Scalds accounted for 13 cases, one contact burn occurred from a hot grill. The median total body surface area burned was 5.7% (range 1.5-26%). The National Census of 2002 recorded a population of 3,917,203. With less than 12,000 asylum seekers in the country, they comprise only approximately 0.3% of the population yet they account for 11.4% of the burn patients admitted to our unit, p<0.0001. Children of asylum seekers are over-represented in our series of paediatric admissions for burns and are more likely than Irish children to sustain a burn at a younger age and in the domestic setting. This may indicate an increased risk of injury and warrants further investigation.

Accidents, Home↗

European Resuscitation Council Guidelines 2000 for Basic Paediatric Life Support. A statement from the Paediatric Life Support Working Group and approved by the Executive Committee of the European Resuscitation Council.

The European Resuscitation Council (ERC) last issued guidelines for Paediatric Life Support (PLS) in 1998 [1]. These were based on the "Advisory Statements" of the International Liaison Committee on Resuscitation (ILCOR) published in 1997 [2]. Following this, the American Heart Association, together with representatives from ILCOR, undertook a series of evidence-based evaluations of the science of resuscitation which culminated in the publication of "Guidelines 2000 for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care" in August 2000 [3,4]. The Paediatric Life Support Working Party of the European Resuscitation Council has considered this document and the supporting scientific literature and has recommended changes to the ERC Basic PLS guidelines. These are presented in this paper. There have been few major changes to the ERC recommended guidelines as some of the changes agreed in "Guidelines 2000" had already been introduced into Europe subsequent to the 1998 ILCOR "Advisory Statements" (Fig. 1).

Adolescent↗

European Resuscitation Council Guidelines 2000 for Advanced Paediatric Life Support. A statement from Paediatric Life Support Working Group and approved by the Executive Committee of the European Resuscitation Council.

The European Resuscitation Council (ERC) last issued guidelines for Paediatric Life Support (PLS) in 1998 [1]. These were based on the "Advisory Statements" of the International Liaison Committee on Resuscitation (ILCOR) published in 1997 [2]. Following this, the American Heart Association, together with representatives from ILCOR, undertook a series of evidence-based evaluations of the science of resuscitation which culminated in the publication of "Guidelines 2000 for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care" in August 2000 [3,4]. The Paediatric Life Support Working Party of the European Resuscitation Council has considered this document and the supporting scientific literature and has recommended changes to the ERC Advanced PLS guidelines. These are presented in this paper. There have been few major changes to the ERC recommended guidelines as some of the changes agreed in "Guidelines 2000" had already been introduced into Europe subsequent to the 1998 ILCOR "Advisory Statements" (Fig. 1).

Advanced Cardiac Life Support↗

Future expectations--what paediatric nephrologists and urologists await from paediatric uroradiology.

Cooperation between paediatric nephrology/urology and paediatric radiology is essential for timely and correct diagnosis and therapy of kidney and urinary tract disorders. We need the direct contact between doctors before or after investigations, interdisciplinary discussions, and rapid access to the images. This should lead to optimal settings for investigations, a reduction of radiation burden and the number of investigations, and further improvement in the management of patients. Modern sonography including colour Doppler sonography, amplitude-coded Doppler sonography, and eventually 3D-ultrasound is and will be the method of choice as the basic, non-invasive investigation. These investigations should become the routine in all institutions. Refinement and standardisation of already established investigations are needed. Recently introduced investigations, like MRI, will have to show their impact on future diagnostic imaging. Future introduction of new non-invasive methods is welcome, e.g. to correctly diagnose vesicoureteral reflux without catheterisation/puncture of the bladder.

Child↗

Paediatric surgical oncology. 5--Nephroblastoma. International Society of Paediatric Oncology (SIOP) Nephroblastoma Trial & Study Committee.

The favourable results from the treatment of Wilms' tumour are an example of the success of multimodal therapy in paediatric oncology. The epidemiology, methods of diagnosis, benefits of pre-operative chemotherapy, basic principles of surgery and post-operative treatment modalities are presented. The approach to the management of Wilms' tumour considered in this paper is mainly that of the International Society of Paediatric Oncology.

Chemotherapy, Adjuvant↗

Transplantation activities and treatment strategies in paediatric stem cell transplantation centres: a report from the EBMT Working Party on Paediatric Diseases. European Group for Blood and Marrow Transplantation.

To determine the current approach to stem cell transplantation (SCT) in centres which treat predominantly paediatric patients, a questionnaire was sent to 67 centres known by the EBMT registry to perform SCT mainly in children. Fifty-five centres from 19 countries responded. Forty centres (75%) started their transplantation activities between 1980 and 1992. Median number of transplants/centre was 95 (range 8-400). Median number of transplants/centre/year was 18 (range 5-85). On average, there was one physician responsible for seven SCT/year while one nurse was involved for a median of 1.7 SCT/year. Median four rooms/centre (range 1-17) were available for paediatric SCT. The most common isolation facilities were rooms with high efficiency particulate air filtration (HEPA). Eighty-two percent (45/55) of the centres performed allogeneic as well as autologous SCT, while 5% (three centres) offered exclusively allogeneic SCT and 13% (seven centres) used only autologous stem cell rescue. Stem cell source for allogeneic SCT was bone marrow in 87%, peripheral blood (PB) in 10% and umbilical cord blood in 3%. Donors were HLA matched related in 57%, mismatched related in 13%, and matched unrelated in 30% of allogeneic SCT. PB was the most commonly used stem cell source for autologous SCT (48%), followed by BM (41%) and the two together (11%). Data analysis revealed substantial differences in protective care, stem cell processing and transplantation procedures within the centres, irrespective of the country, centre size and transplant type.

Adolescent↗

Diagnostic markers in paediatric medulloblastoma: a Paediatric Oncology Group Study.

AIMS: We have reviewed immunohistochemically 17 paediatric medulloblastomas in order to determine if correlations exist that might be useful in subclassifying these tumours. METHODS AND RESULTS: The patient group included 11 children who had died (mean survival 13 months) and six still alive (followed for up to 10 years). Ten tumours were diffuse and six were nodular (one biopsy had only perivascular tumour). Of the 10 diffuse tumours, three were desmoplastic: of the six nodular tumours, all six were desmoplastic. All 17 tumours were synaptophysin-reactive: three nodular tumours were glial fibrillary acidic protein (GFAP)-reactive in the nodules (two of three S 100-reactive tumours were also GFAP-reactive). MIB-1 labelling indices (LI) ranged from 5 to 80%. Six tumours exhibited at least 1% LI against Tp53 (Mab D07 and/or Mab 1801). Eight cases were 100% bcl2-reactive with nine cases having an LI <80% ('low labelling'). All nine 'low labelling' bcl2 cases were TP53 non-reactive; all six Tp53-reactive cases were bcl2 100% reactive. Six of 10 patients with diffuse medulloblastomas survived 18 months or less while four of 10 are alive up to 10 years. In contrast, five of six patients with nodular neoplasms died within 48 months of diagnosis with one patient followed up for less than 1 year. CONCLUSIONS: Immunohistochemistry is a useful adjunct in characterizing subsets of paediatric medulloblastomas and confirms that larger co-operative studies may be fruitful in identifying a prognostic utility of a combined histochemical/immunohistochemical analysis on these tumours.

Adolescent↗

The role of the paediatric nurse in promoting paediatric right to consent.

This article examines the processes involved in obtaining informed consent focusing on the abilities and legalities related to a child's right to consent. Most authors who have researched when a child may be considered competent to give a valid consent propose that the child must be 14 years old and thus able to think abstractly and consider the risks and benefits of the planned treatment. Qualitative research on this topic reveals that a child's previous life experiences can influence their ability to comprehend the intervention. This suggests children under 14 years of age may be regarded as competent. The confusion around the prevailing legal situation is examined. The role of the paediatric nurse is explored to distinguish areas in which the nurse could potentially make a contribution to the process of gaining consent from children. The paediatric nurse's role as an assessor, educator and evaluator are identified.

Child↗