Nouvelles stratégies thérapeutiques en oncologie ophtalmo-pédiatrique [New strategies in pediatric ophthalmic oncology]

Le rétinoblastome représente 11% de tous les cancers apparaissant pendant la première année de vie. Pour éviter les effets secondaires de la chimiothérapie systémique ou de la radiothérapie externe, de nouveaux médicaments et de nouvelles techniques de traitement focalisé ont été développés. Des voies d'administration telles la voie périoculaire (topotécan) ou la voie artérielle ophtalmique directe (carboplatine) sont utilisées aujourd'hui dans certains cas résistants. La radiothérapie actuelle, appliquée exclusivement en deuxième intention, fait appel également à des techniques ciblées, permettant d'éviter les tissus sains et de réduire le risque de cancers radio-induits non oculaires. La radiothérapie stéréotaxique conformationnelle ou conformale et la proton-thérapie font ainsi partie du nouvel arsenal thérapeutique du rétinoblastome. Retinoblastoma represents 11% of all cancers during the first year of life. New drugs and focal treatments have been developed in order to avoid the side effects of systemic chemotherapy and external radiotherapy. New targeted and local administration strategies such as periocular chemotherapy (topotecan) or direct ophthalmic artery delivery (carboplatin), are already used today in selected resistant cases. Radiotherapy, presently indicated only as a second-line treatment, is also subject to new techniques, targeting tumors more closely to avoid involving healthy tissue and reduce the risk of radio-induced nonocular tumors. Stereotactic conformal radiotherapy and proton therapy may thus be included in the new range of treatment methods in retinoblastoma

Prise en charge des jeunes patients avec endocrinopathies pediatriques chroniques: les défis d'une transition souvent difficile [Caring for patients with pediatric endocrinopathies and diabetes into adulthood: challenges of an often difficult transition].

The success of therapies for a number of pediatric disorders has posed new challenges for the long-term follow-up of adolescents with chronic endocrinopathies. Unfortunately, too many patients are lost during the transfer from pediatric to adult clinics. The transition process should be well-organized and include the young person and family. Recognizing the special needs of these adolescents is an important step in developing patient-centered approaches to care that enable patients to develop autonomy and self care skills. Key elements in this process include structured policies and guidelines, communication and close collaboration between pediatric and adult clinics, and integrating nurse clinicians in the transition process to help close the gaps in care.

Deep sclerectomy combined with trabeculectomy in pediatric glaucoma

Résumé : Ce travail porte sur l'étude rétrospective d'une série de jeunes patients opérés de glaucomes pédiatriques. Le but est d'évaluer le résultat au long cours d'une intervention chirurgicale combinant une sclérectomie profonde et une trabéculectomie (sclérectomie profonde pénétrante). Durant la période de mars 1997 à octobre 2006, 28 patients on été suivis pour évaluer le résultat de cette chirurgie effectuées sur 35 yeux. Un examen ophtalmologique complet a été pratiqué avant la chirurgie, 1 et 7 jours, puis 1, 2, 3, 4, 6, 9, 12 mois, enfin tous les 6 mois après l'opération. Les critères d'évaluation du résultat postopératoire sont : les changements de pression intraoculaire, le traitement antiglaucomateux adjuvant, le taux de complication, le nombre de reprises chirurgicales,- l'erreur de réfraction, la meilleure acuité visuelle corrigée, l'état et le diamètre de la cornée. L'âge moyen est de 3.6 ± 4.5 ans et le suivi moyen de 3.6 ± 2.9 ans. La pression intraoculaire préopératoire de 31.9 ± 11.5 mmHg baisse de 58.3% (p<0.005) à la fin du suivi. Sur les 14 patients dont l'acuité visuelle a pu être mesurée, 8 (57.1 %) ont une acuité égale ou supérieure à 5/10e, 3 (21.4%) une acuité de 2/10e après intervention. Le taux de succès cumulatif complet à 9 ans est de 52.3%, le succès relatif 70.6%. Les complications menaçant la vision (8.6%) ont été plus fréquentes dans les cas de glaucome réfractaire. Pour conclure la sclérectomie profonde combinée à une trabéculectomie est une technique chirurgicale développée afin de contrôler la pression intraoculaire dans les cas de glaucomes congénitaux, juvéniles et secondaires. Les résultats intermédiaires sont encourageants et prometteurs. Les cas préalablement opérés avant cette nouvelle technique ont cependant un pronostic moins favorable. Le nombre de complications menaçant la vision est essentiellement lié à la sévérité du glaucome et au nombre d'interventions préalables. Abstract : Purpose : To evaluate the outcomes of combined deep sclerectomy and trabeculectomy (penetrating deep sclerectomy) in pediatric glaucoma. Design : Retrospective, non-consecutive, non-comparative, interventional case series. Participants : Children suffering from pediatric glaucoma who underwent surgery between March 1997 and October 2006 were included in this study. Methods : A primary combined deep sclerectomy and trabeculectomy was performed in 35 eyes of 28 patients. Complete examinations were performed before surgery, postoperatively at 1 and 7 days, at 1, 2, 3, 4, 6, 9, 12 months and then every 6 months after surgery. Main Outcome Measures : Surgical outcome was assessed in terms of intraocular pressure (IOP) change, additional glaucoma medication, complication rate, need for surgical revision, as well as refractive errors, best corrected visual acuity (BCVA), and corneal clarity and diameters. Results : The mean age before surgery was 3.6 ± 4.5 years, and the mean follow-up was 3.5 ± 2.9 years. The mean preoperative IOP was 31.9 ± 11.5 mmHg. At the end of follow-up, the mean IOP decreased by 58.3% (p<0.005), and from 14 patients with available BCVA 8 patients (57.1 %) achieved. 0.5 (20/40) or better, 3 (21.4%) 0.2 (20/100), and 2 (14.3%) 0.1 (20/200) in their better eye. The mean refractive error (spherical equivalent) at final follow-up visits was +0.83 ± 5.4. Six patients (43%) were affected by myopia. The complete and qualified success rates, based on a cumulative survival curve, after- 9 years were 52.3% and 70.6%, respectively (p<0.05). Sight threatening complications were more common (8.6%) in refractory glaucomas. Conclusions : Combined deep sclerectomy and trabeculectomy is a surgical technique developed to control IOP in congenital, secondary and juvenile glaucomas. The intermediate results are satisfactory and promising. Previous classic glaucoma surgeries performed before this new technique had less favourable results. The number of sight threatening complications is related to the severity of glaucoma and number of previous surgeries.

Optimisation des doses en tomographie computérisée pédiatrique [Patient dose optimization in pediatric computerized tomography]

The development of CT applications might become a public health problem if no effort is made on the justification and the optimisation of the examinations. This paper presents some hints to assure that the risk-benefit compromise remains in favour of the patient, especially when one deals with the examinations of young patients. In this context a particular attention has to be made on the justification of the examination. When performing the acquisition one needs to optimise the extension of the volume investigated together with the number of acquisition sequences used. Finally, the use of automatic exposure systems, now available on all the units, and the use of the Diagnostic Reference Levels (DRL) should allow help radiologists to control the exposure of their patients.

The AO comprehensive classification of pediatric long-bone fractures: a web-based multicenter agreement study.

The first AO comprehensive pediatric long-bone fracture classification system has been proposed following a structured path of development and validation with experienced pediatric surgeons. A Web-based multicenter agreement study involving 70 surgeons in 15 clinics and 5 countries was conducted to assess the reliability and accuracy of this classification when used by a wide range of surgeons with various levels of experience. Training was provided at each clinic before the session. Using the Internet, participants could log in at any time and classify 275 supracondylar, radius, and tibia fractures at their own pace. The fracture diagnosis was made following the hierarchy of the classification system using both clinical terminology and codes. kappa coefficients for the single-surgeon diagnosis of epiphyseal, metaphyseal, or diaphyseal fracture type were 0.66, 0.80, and 0.91, respectively. Median accuracy estimates for each bone and type were all greater than 80%. Depending on their experience and specialization, surgeons greatly varied in their ability to classify fractures. Pediatric training and at least 2 years of experience were associated with significant improvement in reliability and accuracy. Kappa coefficients for diagnosis of specific child patterns were 0.51, 0.63, and 0.48 for epiphyseal, metaphyseal, and diaphyseal fractures, respectively. Identified reasons for coding discrepancies were related to different understandings of terminology and definitions, as well as poor quality radiographic images. Results supported some minor adjustments in the coding of fracture type and child patterns. This classification system received wide acceptance and support among the surgeons involved. As long as appropriate training could be performed, the system classification was reliable, especially among surgeons with a minimum of 2 years of clinical experience. We encourage broad-based consultation between surgeons' international societies and the use of this classification system in the context of clinical practice as well as prospectively for clinical studies.

Creation and validation of an instrument to identify nursing activities in pediatric wards: information for determining workload

The aim of this study was to develop and validate an instrument for identifying nursing activities performed in a pediatric ward and to provide a basis for defining the workload of these units. The instrument was developed by selecting the activities relevant to pediatric nursing from the Nursing Intervention Classification and then submitting them to a panel of judges for validation. The panel considered the selected activities relevant and representative of pediatric nursing practice. Now that representative activities for the nursing workload have been identified, we envision new studies to verify their usefulness in practice. Determining the amount of time each activity takes to perform will help to develop a system for measuring the workloads of nursing teams in pediatric wards.

Role of surgery in pediatric epilepsy.

Twenty five percent of patients with intractable epilepsy have surgically remediable epilepsy syndromes. This article reviews the treatment paradigm for pediatric epilepsy and also the indications, methods, and surgical options for the subgroup of patients with surgically remediable epileptic disorders based on our experience in the management of these children. The article also discusses the rationale for offering surgery and the timing of surgery in these patients. The study of surgically remediable epilepsy can best be divided into focal, sub hemispheric, hemispheric and multifocal epileptic syndromes. These syndromes have both acquired and congenital etiologies and can be treated by resective or disconnective surgery. The surgical management of these conditions (with the exception of multifocal epilepsy) provides Engel's Class 1 outcome(complete seizure freedom) in approximately 80% of children. The consequences of seizure freedom leads to a marked improvement in the quality of life of these children. The benefits to society, of allowing a child to grow to adulthood with normal cognition to earn a livelihood and contribute actively to society, cannot be understated.

Health facility environment as humanization strategy care in the pediatric unit: systematic review

Objective: To identify and analyze the production of knowledge about the strategies that health care institutions have implemented to humanize care of hospitalized children. Method: This is a systematic review conducted in the Virtual Health Library - Nursing and SciELO, using the seven steps proposed by the Cochrane Handbook. Results: 15 studies were selected, and strategies that involved relationship exchanges were used between the health professional, the hospitalized child and their families, which may be mediated by leisure activities, music and by reading fairy tales. We also include the use of the architecture itself as a way of providing welfare to the child and his/her family, as well as facilitating the development of the work process of health professionals. Conclusion: Investments in research and publications about the topic are necessary, so that, the National Humanization Policy does not disappear and that the identified strategies in this study do not configure as isolated and disjointed actions of health policy.


Pediatric patient classification system: improvement of an instrument

Objective Improve the content validity of the instrument for classification of pediatric patients and evaluate its construct validity. Method A descriptive exploratory study in the measurement of the content validity index, and correlational design for construct validation through exploratory factor analysis. Results The content validity index for indicators was 0.99 and it was 0.97 for graded situations. Three domains were extracted in the construct validation, namely: patient, family and therapeutic procedures, with 74.97% of explained variance. The instrument showed evidences of content and construct validity. Conclusion The validation of the instrument occurred under the approach of family-centered care, and allowed incorporating some essential needs of childhood such as playing, interaction and affection in the content of the instrument.


Nurses’ attitudes regarding the importance of families in pediatric nursing care

Affective, cognitive and behavioral components affect nurses´ attitudes to include families in the care processes. The purpose of this study was to investigate the attitudes of nurses about the importance of including families in nursing care. Data collection was performed in pediatric and maternal-child unit of a Brazilian university hospital. A sample of 50 nurses completed the Portuguese version of the instrument Families’Importance in Nursing Care-Nurses’ Attitudes (FINC-NA). The results indicated that nurses have supportive attitudes regarding families participation in nursing care. Attitudes of lower support for involving families in nursing care were found among nurses with older age, more time in the profession and who had no previous contact with contents related to Family Nursing. The application of the instrument in other contexts of assistance may help to illuminate important aspects of the challenges to implementing a family-centered approach in clinical practice.





The practice of nurses caring for families of pediatric inpatients in light of Jean Watson

Objective To know the facilities and the difficulties of nurses in caring practice of hospitalized children’s families in the light of Jean Watson’s Theory of Human Caring. Method It was used the descriptive qualitative approach. The data collection was conducted in three stages: presentation of theoretical content; engagement with families in the light of Watson’s theory; and semi-structured interview with 12 pediatric nurses. The interviews were analysed using inductive thematic analysis, being possible to form three themes: Recognizing a framework for care; Considering the institutional context; and Challenges in family’s relationship. Results The theory favored reflections about self, about the institutions and about nurses’ relationship with the family of the child, normalized by a consciousness toward caring attitudes. Conclusion In this process, it is imperative that nurses recognize the philosophical-theoretical foundations of care to attend the child’s family in hospital.



Prevalence of Burnout syndrome in health professionals of an onco-hematological pediatric hospital

OBJECTIVE To identify the prevalence of Burnout Syndrome in medical professionals, nurses and nursing technicians working in an Onco-Hematological Pediatric Hospital in São Paulo. METHOD An exploratory, descriptive study with cross-sectional design and quantitative approach, with a sample of 188 health professionals. Data were collected using two self-report instruments: the Maslach Burnout Inventory (MBI-HSS) which is a biosocial data form, and a non-participant observation guide. RESULTS High depersonalization for nurses (29.8%), low job performance for physicians (27.8%), and of nursing technicians (25.5%). High scores were identified in at least two domains of Burnout in 19.2% of nurses, 16.8% of nursing technicians, and 16.6% of doctors. CONCLUSION Health professionals are highly vulnerable to each of the dimensions of Burnout syndrome - namely emotional exhaustion, alienation, and low job performance/satisfaction- in the hospital work.

Transition from pediatric to adult health care: expectations of adolescents with chronic disorders and their parents.

The aim of this study was to assess the expectations of adolescents with chronic disorders with regard to transition from pediatric to adult health care and to compare them with the expectations of their parents. A cross-sectional study was carried out including 283 adolescents with chronic disorders, aged 14-25 years (median age, 16.0 years), and not yet transferred to adult health care, and their 318 parents from two university children's hospitals. The majority of adolescents and parents (64%/70%) perceived the ages of 18-19 years and older as the best time to transfer to adult health care. Chronological age and feeling too old to see a pediatrician were reported as the most important decision factors for the transfer while the severity of the disease was not considered important. The most relevant barriers were feeling at ease with the pediatrician (45%/38%), anxiety (20%/24%), and lack of information about the adult specialist and health care (18%/27%). Of the 51% of adolescents with whom the pediatric specialist had spoken about the transfer, 53% of adolescents and 69% of parents preferred a joint transfer meeting with the pediatric and adult specialist, and 24% of these adolescents declared that their health professional had offered this option. In summary, the age preference for adolescents with chronic disorders and their parents to transfer to adult health care was higher than the upper age limits for admission to pediatric health care in many European countries. Anxiety and a lack of information of both adolescents and their parents were among the most important barriers for a smooth and timely transfer according to adolescents and parents.

Clinical Proton MR Spectroscopy in Central Nervous System Disorders.

A large body of published work shows that proton (hydrogen 1 [(1)H]) magnetic resonance (MR) spectroscopy has evolved from a research tool into a clinical neuroimaging modality. Herein, the authors present a summary of brain disorders in which MR spectroscopy has an impact on patient management, together with a critical consideration of common data acquisition and processing procedures. The article documents the impact of (1)H MR spectroscopy in the clinical evaluation of disorders of the central nervous system. The clinical usefulness of (1)H MR spectroscopy has been established for brain neoplasms, neonatal and pediatric disorders (hypoxia-ischemia, inherited metabolic diseases, and traumatic brain injury), demyelinating disorders, and infectious brain lesions. The growing list of disorders for which (1)H MR spectroscopy may contribute to patient management extends to neurodegenerative diseases, epilepsy, and stroke. To facilitate expanded clinical acceptance and standardization of MR spectroscopy methodology, guidelines are provided for data acquisition and analysis, quality assessment, and interpretation. Finally, the authors offer recommendations to expedite the use of robust MR spectroscopy methodology in the clinical setting, including incorporation of technical advances on clinical units. © RSNA, 2014 Online supplemental material is available for this article.