This pandemic has profoundly changed our lives for many months and its long-term consequences remain largely hypothetical. The containment measures, the threats to the health of relatives, the ...constraints limiting social openings have left no one indifferent, but may have particularly impeded "adolescent separation work". Most of adolescents have been able to deploy their adaptation capacities, while for others this exceptional situation has triggered stressful reactions for those around them. Some were immediately overwhelmed by the direct or indirect manifestations of their anxiety or by their intolerance of governmental instructions, others revealed their difficulties only when the schools reopened, or even in the distant "aftermath", as shown by some studies carried out at a distance revealing a clear increase in suicidal ideation. We will not be surprised by the problems of adaptation of the most fragile, of those suffering from psychopathological disorders, but it is necessary to note an increase in the needs for psychological care. Teams dealing with the suffering of adolescents are puzzled by the increase in self-vulnerable acts, anxious school refusals, eating disorders or various forms of addiction to screens. However, everyone agrees on the key role of parents and the impact of their own suffering on that of their children, even if they are young adults. Of course, it is important that caregivers do not forget the parents in the support they aim to provide to their young patients.
Factor H and MCP are regulatory proteins of the alternative pathway. 8 Since the description of a genetic association of the disease with the cluster of complement related genes on chromosome 1, ...several mutations have been reported on the exons coding for the C-terminal domains of FH, which are important for C3b binding. 5, 9- 12 Recently, two groups presented evidence that CD46 mutations may also predispose to HUS. 7, 13 Considering the major role of FH in complement alternative pathway regulation, we speculated that other complement regulators might be involved in the disease process. Briefly, Nunc MaxiSorp ELISA plates (Nunc, Roskilde, Denmark) were coated with goat polyclonal IgG anti-human factor I (Calbiochem, Meudon, France).\n FH deficiencies have been reported in both homozygous and heterozygous forms. 15 Genetic studies have also found several different heterozygous missense mutations between SCR16 and SCR20 suggesting a particular role of the C-terminal domains of the protein relevant to the pathophysiology of HUS. 9, 10, 26, 27 Neumann et al found FH mutations in 13% of 111 patients with atypical HUS. 12 The RCA genomic region contains several candidate genes for HUS including the five human regulators of complement activation proteins: FH, complement receptor type 1 (CR1, CD35), membrane cofactor protein (MCP, CD46), decay accelerating factor (DAF), and C4b-binding protein. 28 Recently, MCP mutations were detected by Richards et al in affected individuals of three families and by Noris et al in one family with low C3 levels. 7, 13 On the other hand, we can speculate that a link between the candidate proteins may be ascribed to the alternative pathway regulation.
The perception of adolescent medicine (AM) among future pediatricians in France has scarcely been explored although adolescent health care is an integral part of the pediatrician's job. Moreover, ...pediatric residents seem to have a lack of enthusiasm for this field. The aim of this study was to assess the beliefs and perceptions of fourth-year French pediatric residents regarding adolescents and AM.
We conducted a national electronic survey among French pediatric residents in their last year of residency. An original 51-item, open-ended declarative questionnaire was sent out including questions on demographics, career expectations, AM block rotation experiences, and perceptions and beliefs regarding adolescents and AM. Difficulties and barriers experienced in this field were investigated. Queries about residents' specific AM courses and about their educational needs were also explored.
The survey was conducted online between January and March 2015. There were 121 respondents, achieving a 43% response rate. The majority of respondents perceived teens as interesting (75%), vulnerable (83%), and time-consuming (84%). Up to four fifths of respondents (81%) considered AM to be a skill that all pediatricians should acquire. A subgroup analysis showed that the residents who had not experienced an AM block rotation did not seem to be convinced of the need for training in this field (P=0.09). The following issues were reported as major difficulties by residents: providing care for teens reluctant to seek health care (65%), managing violent behavior among adolescents in the emergency ward (98%), and managing adolescents who attempt suicide (80%). Less than half (45%) of the residents reported their residency program had a specific AM course. Among them, 73% attended the lectures and 57% found them useful. One third (32%) of the respondents had experienced a 2-month block rotation in the field of AM during their residency.
This is the first nationwide study in France in this field. Although the response rate is below 50%, it exceeded our expectations. Our results are in line with previous studies showing that pediatric residents report a high interest in working with adolescents and yet their educational needs in AM are unmet. The limitations of this study include the self-selection bias and implicit limitations carried by the phrasing of the survey questions.
There is a need to devise a better educational approach combining a broader proposal of AM block rotations together with a specific competency framework in adolescent health, since we know it improves the quality of health care delivered to adolescents. Moreover, training pediatricians in AM is a public health priority so as to comply with medical schools' principles of social accountability and address the priority health needs for an efficient, equitable, and sustainable health system. At a time when postgraduate reform is being made, the inclusion of an AM curriculum in the general pediatrician course is necessary.
Chronic idiopathic intestinal pseudo-obstruction is a rare and serious chronic disease starting in childhood, which can affect the entire digestive tract. It is caused by a peristalsis intestinal ...disorder that leads to occlusions without any obvious obstruction. Few studies have been carried out regarding the prognosis of this illness. This disease is often diagnosed by a process of elimination, but some histological anomalies have been present in the digestive wall of certain patients. This clinical case concerns a 17-year-old girl affected by CIPO and eating disorders. It seems difficult to discriminate between digestive disorders and eating disorders. What psychological effects can this severe pathology have? Are eating disorders induced by CIPO? These questions are raised in this article through the example of this patient's somatopsychic complexity and the ensuing difficulties in her overall care.
Introduction 50 % des diabètes de type I sont révélés avant l’âge de 20 ans. Ainsi au moment de la période délicate qu’est l’adolescence, le passage de la pédiatrie vers les structures de soins ...d’adultes est une étape cruciale pour ces jeunes patients. Cette transition, mettant en jeu de nombreux acteurs (patients, parents, équipes pluridisciplinaires pédiatriques et adultes) soulève de nombreuses questions. Le but de ce travail était, à partir d’une évaluation par les patients des modalités actuelles de passage, de proposer des moyens afin d’optimiser ce transfert. Patients et Méthodes Notre étude prospective était basée sur un questionnaire envoyé à 220 patients nés entre janvier 1973 et décembre 1992, suivis dans le service de diabétologie pédiatrique pour un diabète de type I et actuellement passés vers les services d’adultes. Le questionnaire explorait les conditions et modalités de passage et la situation et le suivi actuels des patients. Les données discontinues étaient analysées par un test du X2, les données continues par un test anova. Résultats Sur 137 questionnaires reçus par les patients, nous avons obtenons 83 réponses (60 %). Les patients répondeurs, âgés de 23,9 ± 5,3 ans, étaient diabétiques en moyenne depuis 15,8 ± 5,7 ans. Au moment du passage, les patients étaient âgés de 16,3 ± 1,8 ans. Seuls 52 % des patients souhaitaient ce transfert, 76 % s’estimaient assez bien ou bien préparés. La proportion de patients satisfaits (46 %) et peu ou insatisfaits (44 %) de leur passage était similaire. Dans les deux ans suivants le passage, 35 % ont présenté une rupture de suivi, définie comme ne consultant pas durant au moins 6 mois, principalement en raison d’un mauvais contact avec le diabétologue d’adultes. Discussion Les difficultés lors du passage sont de l’ordre du travail de deuil avec l’équipe de pédiatrie, et sont essentiellement liées aux difficultés à créer une alliance thérapeutique entre cet adolescent-jeune adulte et l’équipe soignante. Conclusion Le passage des diabétiques de type I de la pédiatrie vers les services d’adultes est un moment crucial qu’il faut anticiper et préparer. Nous proposons 3 axes de travail afin d’optimiser cette transition : renforcer la préparation, consolider les liens entre les services et organiser un temps de rencontre entre jeunes et membres de l’équipe d’adultes.
Insulin pumps are booming in pediatric diabetology. The objective of this study was to assess changes for children and adolescents with type 1 diabetes using a pump in terms of quality of life (QOL), ...satisfaction, and glycosylated hemoglobin. A retrospective self-evaluation questionnaire was distributed to 41 patients. It focused on general QOL, diabetes-specific QOL supplemented by specific questions on the pump, and satisfaction. Clinical and biological parameters (glycated hemoglobin: HbA1c) were compared before and after pump use. The score for QOL with the pump was positive, more so if started early after diagnosis of diabetes (P=0.03) and with children under the age of 8 years (P<0.02). These positive results are mainly related to the characteristics of the pump, "insulin management" and "injections," as well as "diabetes management," "behavior," "school," "family life," "daily life," and "physical activities." On the other hand, the improvement was not significant for the item "life in society, friends and family." A decrease in the number of injections and the flexibility of meals were the most positive points. HbA1c improved as soon as the pump was indicated before its use was begun (P=0.005) and remained constant for 4 years (P≤0.05). Forgotten injections, comments on diabetes, and technical problems appeared to be exceptional. The pump changed the patient's body image because of ambivalent feelings between being normal (greater freedom) and different (visibility and a reminder of the disease). The benefits in terms of QOL and glycemic control with the pump cannot be dissociated and can only be considered accompanied by paramedical and medical assistance. Improving QOL over the short and long term by reducing the risk of further complications is the daily challenge of families and diabetologists.
Nous avons tous, enfants, adolescents, adultes subi cette pandémie qui a profondément bouleversé nos vies pendant de nombreux mois et dont les conséquences sur le long terme restent largement ...hypothétiques. Les mesures de confinement, les menaces pesant sur la santé des proches, les contraintes restreignant les ouvertures sociales n’ont laissé personne indifférent, mais ont particulièrement pu faire obstacle au travail de séparation des adolescents. La plupart ont su déployer leurs capacités d’adaptation, alors que pour d’autres cette situation d’exception a déclenché des réactions propres à mettre à l’épreuve, voire à inquiéter leur entourage. Certains se sont d’emblée montrés débordés par les manifestations directes ou indirectes de leur angoisse ou par leur intolérance aux consignes gouvernementales, d’autres n’ont révélé leurs difficultés qu’au moment de la réouverture des établissements scolaires, voire dans un lointain « après-coup » comme en témoignent quelques études réalisées à distance révélant une nette augmentation des idéations suicidaires. On ne s’étonnera pas des problèmes d’adaptation des plus fragiles, de ceux souffrant de troubles psychopathologiques, mais force est de constater une augmentation de la demande de soins psychiques. Les dispositifs propres à accueillir et traiter la souffrance des adolescents font état de leur perplexité face à l’accroissement des gestes auto-vulnérants, des refus scolaires anxieux, des troubles des conduites alimentaires ou des diverses formes d’addiction aux écrans… Chacun s’accorde cependant à reconnaître le rôle crucial des parents et de l’impact de leurs propres souffrances sur celle de leurs enfants, fussent-ils jeunes adultes. Il importe naturellement que les soignants ne les oublient pas dans le soutien qu’ils cherchent à offrir à leurs jeunes patients.
This pandemic has profoundly changed our lives for many months and its long-term consequences remain largely hypothetical. The containment measures, the threats to the health of relatives, the constraints limiting social openings have left no one indifferent, but may have particularly impeded “adolescent separation work”. Most of adolescents have been able to deploy their adaptation capacities, while for others this exceptional situation has triggered stressful reactions for those around them. Some were immediately overwhelmed by the direct or indirect manifestations of their anxiety or by their intolerance of governmental instructions, others revealed their difficulties only when the schools reopened, or even in the distant “aftermath”, as shown by some studies carried out at a distance revealing a clear increase in suicidal ideation. We will not be surprised by the problems of adaptation of the most fragile, of those suffering from psychopathological disorders, but it is necessary to note an increase in the needs for psychological care. Teams dealing with the suffering of adolescents are puzzled by the increase in self-vulnerable acts, anxious school refusals, eating disorders or various forms of addiction to screens. However, everyone agrees on the key role of parents and the impact of their own suffering on that of their children, even if they are young adults. Of course, it is important that caregivers do not forget the parents in the support they aim to provide to their young patients.
Les pompes à insuline sont en plein essor en diabétologie pédiatrique. L’objectif de cette étude était d’évaluer la qualité de vie (QV) et la satisfaction des enfants et adolescents diabétiques de ...type 1 sous pompe à insuline. Un auto-questionnaire rétrospectif a été distribué à 41 enfants. Il portait sur la QV générale et la QV spécifique au diabète et était complété par des éléments spécifiques à la pompe et sur la satisfaction. Des paramètres cliniques et biologiques dont l’hémoglobine glyquée (HbA1c) ont été comparés avant et après la mise en place de la pompe. Le score de QV sous pompe était positif, d’autant plus qu’elle était mise en place précocement après le diagnostic de diabète (p=0,03) et chez l’enfant de moins de 8ans (p<0,02). Cet effet positif concernait surtout les spécificités de la pompe : « gestion de l’insuline » et « injections » mais aussi la gestion du diabète, les émotions et comportement, la scolarité, la vie en famille, la vie quotidienne et les activités physiques. En revanche, le score pour l’item « vie en société, entourage » n’était pas significatif. La baisse du nombre de piqûres et la souplesse des repas étaient les points les plus positifs. L’HbA1c s’améliorait dès l’indication de la pompe avant sa mise en route (p=0,005) et était stable pendant 4ans (p≤0,05). Les oublis des injections, les remarques sur le diabète et les problèmes techniques semblaient faire exception. La pompe modifiait l’image du corps par un sentiment ambivalent de normalité (libertés engendrées) et de différence (visibilité, rappel de la maladie). Les bénéfices de QV et d’équilibre glycémique sous pompe sont indissociables et ne peuvent s’envisager qu’entourés d’un accompagnement médical et paramédical. Améliorer la QV à court et à long terme en diminuant le risque de complications ultérieures constitue le défi quotidien des familles et des diabétologues.
Insulin pumps are booming in pediatric diabetology. The objective of this study was to assess changes for children and adolescents with type 1 diabetes using a pump in terms of quality of life (QOL), satisfaction, and glycosylated hemoglobin. A retrospective self-evaluation questionnaire was distributed to 41 patients. It focused on general QOL, diabetes-specific QOL supplemented by specific questions on the pump, and satisfaction. Clinical and biological parameters (glycated hemoglobin: HbA1c) were compared before and after pump use. The score for QOL with the pump was positive, more so if started early after diagnosis of diabetes (P=0.03) and with children under the age of 8 years (P<0.02). These positive results are mainly related to the characteristics of the pump, “insulin management” and “injections,” as well as “diabetes management,” “behavior,” “school,” “family life,” “daily life,” and “physical activities.” On the other hand, the improvement was not significant for the item “life in society, friends and family.” A decrease in the number of injections and the flexibility of meals were the most positive points. HbA1c improved as soon as the pump was indicated before its use was begun (P=0.005) and remained constant for 4 years (P≤0.05). Forgotten injections, comments on diabetes, and technical problems appeared to be exceptional. The pump changed the patient's body image because of ambivalent feelings between being normal (greater freedom) and different (visibility and a reminder of the disease). The benefits in terms of QOL and glycemic control with the pump cannot be dissociated and can only be considered accompanied by paramedical and medical assistance. Improving QOL over the short and long term by reducing the risk of further complications is the daily challenge of families and diabetologists.
Summary Background Achalasia or idiopathic megaesophagus is rare in children, and an association with growth hormone (GH) deficiency has rarely been described. Case report This report is of a girl ...treated with recombinant GH (rGH) for isolated GH deficiency who simultaneously presented with idiopathic megaesophagus. Conclusion This case report highlights the fact that practitioners need to be aware of this possible morbid association.
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