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Resume Divers dispositifs d’accompagnement des patient(e)s anorexiques et de leurs familles sont proposes par les equipes de soins. Les groupes de parents et les therapies familiales sont les plus courants, les groupes de patient(e)s sont moins repandus mais depuis quelques annees ont ete mis en place des groupes multifamiliaux associant plusieurs familles et plusieurs patient(e)s a des frequences variees. Nous tenterons de rendre compte d’un « montage » peu decrit dans la litterature que nous avons mis en place au CHU d’Amiens depuis quelques annees : un groupe de parents et un groupe d’adolescents anorexiques sont ainsi reunis successivement et alternativement un samedi matin par mois avec un meme therapeute qui assure un role de go-between, mais aussi de facilitateur de pensee du trouble. Les notes prises dans l’apres-coup des rencontres offrent un materiau permettant de mettre en valeur les axes de reflexion qui circulent dans chaque groupe et parfois d’un groupe a l’autre a la demande des participants. Les adolescent(e)s reprennent certes a leur compte les stereotypes largement utilises pour caracteriser leurs symptomes, mais parviennent secondairement a forger d’autres representations plus inedites ou moins « avouables » du piege anorexique. Ils/elles souhaitent que leurs parents prennent conscience de leur accrochage nostalgique a l’enfance et de leur propension excessive a repondre aux attentes de leurs proches venant faire obstacle a leurs revendications adolescentes. Par contre, l’evocation de la fierte tiree de leur volonte inflexible et de la poursuite d’un ideal de minceur auquel les autres ne parviennent pas a acceder est plus difficilement transmissible. Les parents se soutiennent mutuellement, font d’abord etat du bouleversement survenu dans la vie familiale depuis l’eclosion de cette maladie qui leur semble venue de nulle part, avant de pouvoir questionner timidement leurs principes educatifs, leurs propres adolescences sans vagues, voire meme le culte de l’esprit de famille et la position sacrificielle qu’il implique.

Resume Definie comme la non-concordance entre la prescription medicale et sa mise en application, l’observance est un enjeu majeur de sante pour le pediatre et l’equipe soignante prenant en charge l’enfant et l’adolescent porteurs d’une maladie chronique. Au temps de l’enfance, l’alliance avec les parents repose sur un accompagnement attentif et vigilant. L’adolescence est une periode sensible, souvent problematique ou le pediatre et l’equipe soignante doivent mobiliser leur capacite d’ecoute, d’empathie, pour apprehender toutes les dimensions de la non-observance : revolte, appropriation de la maladie, depression… non seulement en direction de l’adolescent, mais vers les parents. L’education therapeutique de l’enfant et de l’adolescent est ainsi essentielle pour mener l’adolescent a l’autonomie et preparer la transition vers la medecine adulte.

Resume Peu d’etudes explorent comment les futurs pediatres se representent la medecine de l’adolescent (MA) alors qu’ils y seront confrontes. Ce travail avait pour objectif principal de recueillir les representations de la MA aupres de futurs pediatres et pour objectif secondaire de preciser leurs besoins en termes de formation. Un questionnaire declaratif electronique a ete diffuse via l’Association des juniors de pediatrie (AJP) aux internes de pediatrie ayant valide 6 semestres. Ce questionnaire, compose de 51 questions, explorait leur caracteristiques demographiques et leur formation pratique en MA ; leur representations de la MA ; et les besoins ressentis en termes de formation. Cent vingt et un internes ont repondu a l’enquete (taux de reponse 43 %). Les adolescents etaient percus comme interessants (75 %) et chronophages (84 %). Quatre cinquieme des repondants consideraient qu’une formation specifique a la MA etait necessaire. Les internes jugeant cette formation inutile etaient ceux qui n’avaient pas pratique de stage hospitalier en MA. Un enseignement dedie a la MA avait ete propose a moins de la moitie des internes (45 %). Un tiers des repondants avait effectue un stage d’au moins 2 mois dans une unite de MA. Former les futurs pediatres a la MA est une priorite en matiere de sante publique. Le referentiel de competences dans ce champ de la pediatrie doit etre articule avec l’identification de terrains de stages hospitaliers et extra-hospitaliers. A l’heure de la reforme du troisieme cycle une mobilisation de la pediatrie s’impose.

Resume La pseudo-obstruction intestinale chronique (POIC) est une maladie rare et severe qui debute dans l’enfance et peut toucher toutes les structures du tube digestif. Son diagnostic est d’elimination mais certains patients presentent des anomalies histologiques. Cet article rapporte le cas d’une jeune fille atteinte d’une POIC et d’un trouble des conduites alimentaires (TCA) apparu au decours d’une intervention chirurgicale. Il semble difficile de dissocier les troubles digestifs et alimentaires. Quels effets psychologiques peut avoir cette maladie ? Les troubles alimentaires sont-ils secondaires ?

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.

Le but de cette etude est de determiner la prevalence des marqueurs biologiques de la maladie cœliaque (MC) dans une cohorte francaise de 84 enfants diabetiques de type 1. La detection des anticorps antitransglutaminase (AtTG), antiendomysium (AAE) et antigliadine (AAG) a ete realisee. Le groupe 1 inclut 81 patients (96,4 %) diabetiques avec des marqueurs serologiques negatifs pour la maladie cœliaque. Le groupe 2 inclut 3 patients (3,6 %) presentant des anticorps positifs : 1 AAG-AAE-AtTG et 1 AAE-AtTG avec une confirmation histologique du diagnostic de maladie cœliaque et 1 AAG avec une biopsie negative. Aucune difference statistique n’est observee entre les deux groupes concernant l’âge, la duree du diabete, la taille cible familiale, les ratios taille/âge et poids/taille. En ce qui concerne l’equilibre du diabete, un niveau moins eleve d’HbA1c (hemoglobine glyquee) est retrouve chez les patients presentant des marqueurs serologiques de la MC. Les anticorps anti-thyroperoxidase et anti-thyroglobuline ont ete recherches comme un marqueur d’auto-immunite. La prevalence des thyroidites est identique a celle retrouvee dans d’autres etudes semblables mais n’est pas associee a la MC. La prevalence des marqueurs serologiques de la MC est importante dans cette cohorte d’enfants diabetiques francais mais moindre que celle decrite dans d’autres pays.

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.

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.

In order to study physical adaptation of children with insulin dependent diabete mellitus (DID), two kinds of test were proposed: An isometric test as a force maintain (50% of volantary maximal force) in teg extension and an linear incremental test on ergocycle (from 20% to 100% of the maximal aerobic power during 8 min). For each test, electromyographic signal (EMG) of quadriceps muscle was characterised in the spectral domain by calculating total energy (ET) and mean power frequency (FM). During incremental test, cardio-respiratory variables (heart rate, O 2 consumption, CO 2 production) and metabolivariables (glycemia, lactatemie, and kaliemia) were measured. According to a healthy children group (SNS), DID showed in isometric condition a fatigability more important. In dynamic condition, changes m cardio-respiratoiry variables were similar while metabolic variables were differently modified. Changes in ET mid FM were illustrated by specific profiles for children with respect to adolescent children (quasilinear increase for ET, relative stability for FM). Using a discriminant analysis, SNS and DID children may be differencied. Thus, EMG signal reflects metabolic changes during incremental test and may be used to characterized DID physical aptitude.

Resume Une atteinte cardiaque peut survenir au cours du syndrome hemolytique et uremique (SHU) typique. Elle demeure cependant exceptionnelle, mais greve souvent le pronostic vital. Observations Cas 1. Charles, âge de 21 mois, avait un SHU typique; une insuffisance renale aigue necessitait une dialyse peritoneale durant 20 jours et ces antibiotiques a cause d'une fievre inexpliquee. Au 10 e jour de dialyse, on notait la survenue d'un infarctus du myocarde, vraisemblablement secondaire a une embolie coronaire liee a une endocardite. L'enfant etait traite par heparine. L'evolution etait favorable, avec cependant une necrose antero-apicale sequellaire et une insuffisance renale moderee. Cas 2 . Une fille, âgee de 24 mois, avait un SHU typique, necessitant une dialyse peritoneale durant 10 jours. Au 4 e jour de dialyse, apparaissait une myocardiopathie, decouverte lors d'une decompensation cardiaque qui ne pouvait etre imputee ni a un desordre hydroelectrolytique, ni a une hypertension arterielle, ni a une dialyse inefficace. Une atteinte neurologique apparaissait au 5 e jour de dialyse. L'evolution etait favorable sur les plans neurologique, cardiaque et nephrologique. Cas 3 . Une fille, âgee de 25 mois, avait un SHU typique, oligoanurique, necessitant une dialyse peritoneale pendant 25 jours. L'enfant n'avait pas de probleme cardiovasculaire pendant la phase aigue. Il persistait cependant une insuffisance renale chronique postdialytique (clairance de la creatinine: 15 mL/min/1,73 m 2 ). Trois mois plus tard, apparaissait une myocardiopathie dilatee hypokinetique sans cause precise. L'evolution etait favorable sous traitement digitalique. Conclusion Afin d'ameliorer le pronostic du SHU, une atteinte cardiaque doit etre recherchee au cours de la phase aigue de la maladie et plusieurs mois apres.

A method is presented for monitoring the relative variation of extracellular and intracellular fluid volumes using a multifrequency impedance meter and the Cole-Cole extrapolation technique. It is found that this extrapolation is necessary to obtain reliable data for the resistance of the intracellular fluid. The extracellular and intracellular resistances can be approached using frequencies of, respectively, 5 kHz and 1000 kHz, but the use of 100 kHz leads to unacceptable errors. In the conventional treatment the overall relative variation of intracellular resistance is found to be relatively small.

A technique for continuous measurements of haematocrit and plasma volume in the arterial line of dialysed patients has been tested in vitro and in vivo. This method uses impedance measurements at 5 kHz and requires a single haematocrit measurement. It relies on two assumptions: that plasma resistivity does not change during dialysis and that blood resistivity obeys Hanai's model. Both assumptions are verified during in vitro tests. Haematocrits measured in vivo by this method are found to be in good agreement with direct measurements from blood samples. The haematocrit variation is then used to monitor changes in plasma volume, assuming conservation of erythrocyte volume. In addition, it is possible to obtain the variation in interstitial volume by combining these data with body impedance measurements.

La prevalence de l'obesite de l'adolescent est en progression constante: face a ce probleme de sante publique, le pediatre doit elaborer des propositions de prise en charge. Les reperes de cette prise en charge sont: I) une definition - diagnostic etablie sur le calcul de l'index de masse corporelle (IMC): obesite de grade 1 superieure ou egale a 97 e centile, obesite de grade 2 superieure ou egale au seuil IOTF C 30; 2) une anamnese complete precisera la precocite du rebond et l'existence d'un surpoids familial; 3) les morbidites associees, presentes ou en risque; 4) evaluation des habitudes alimentaires de l'adolescent et de sa famille; 5) evaluation de l'activite physique; 6) appreciation de l'etat psychologique en precisant l'estime de soi, une souffrance physique et l'existence de troubles anxieux et/ou depressifs.

The kinetics of urea, creatinine and phosphate removal during dialysis were investigated in pediatric patients using a two-pool model taking into account fluid shifts and mass transfer between the two compartments. It is found that even urea must be described by a two-pool model since it presents a post dialysis rebound due to equilibration between the two compartments. Phosphate plasma concentration drops very sharply during the first hour of dialysis and rises rapidly during the rebound period. This pattern cannot be accounted for by the classical two-pool model with constant generation rate and mass transfer coefficients, but corresponds to a large time-dependent phosphate influx from the intracellular compartment in which phosphate is generated by biochemical reactions or liberated from the bones. This influx was calculated for four patients representing 8 dialysis sessions and was found to reach a plateau after 90 minutes of dialysis, dropping rapidly during the rebound period.

Cette etude vise a parvenir a une meilleure quantification des techniques d'hemodialyse chez l'enfant en s'appuyant sur la modelisation cinetique et sur des mesures d'impedance pour le suivi des transferts hydriques. Les cinetiques d'epuration de l'uree, de la creatinine et des phosphates ont ete etudiees chez quatre enfants hemodialyses a l'aide de modeles a deux compartiments. Les parametres necessaires aux modeles sont determines a partir des concentrations mesurees sur des prelevements sanguins et sur le dialysat recueilli. Les resultats montrent que le rebond de l'uree observe apres une seance correspond, comme pour la creatinine, a un reequilibrage entre les compartiments intra- et extracellulaires. Par contre, le phosphore suit une cinetique particuliere due a un influx dans le compartiment sanguin qui augmente rapidement au debut de la seance du fait de reactions biochimiques. Des mesures d'impedance, realisees a des frequences de 5, 50 et 100 KHz, permettent de calculer les resistances electriques des compartiments intra- et extracellulaires et d'en tirer l'evolution de leurs volumes respectifs. L'impedance varie lineairement avec le volume ultrafiltre en accord avec un modele electrique simple. Les bilans calciques ont ete etudies chez des patients adultes afin de verifier la validite d'un modele de transfert de masse du calcium a travers la membrane de l'hemodialyseur permettant de determiner la concentration en calcium du dialysat necessaire pour obtenir, chez un patient presentant une calcemie predialytique donnee, un bilan calcique nul au cours de la seance.

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 (P0.02). 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.

Resume Introduction – Une etude multiparametrique a ete menee chez des enfants diabetiques insulinodependants en vue de caracteriser leur aptitude a l'exercice. Methodes et resultas – Onze enfants DID et 24 enfants temoins ont effectue un test incremental lineaire de puissance sur ergocycle, normalise par rapport a la puissance maximale aerobie de chaque enfant. Les signaux electromyographiques de chaque chef musculaire du muscle quadriceps (energie totale et frequence moyenne du signal), et les variables cardiorespiratoires (consommation maximale d'oxygene, frequence cardiaque) et metaboliques (glycemie, lactatemie) ont ete quantifiees. Pour les enfants diabetiques, les evolutions des variables cardiorespiratoires ne sont pas significativement differentes de celles des temoins alors que les variables metaboliques evoluent differemment. Les variables electromyographiques montrent des profils d'evolution qui different selon les enfants mais pas selon les groupes. L'analyse discriminante realisee sur l'ensemble des profils de la frequence moyenne a permis de differencier les enfants diabetiques des enfants temoins. Conclusion – L'evolution du signal electromyographique permet de caracteriser l'aptitude a l'exercice des enfants diabetiques.

Hypothyroxinemia of prematurity (HTOP) is associated with neurodevelopmental impairment in pre-term newborns born at less than 32 weeks of gestation (WG). HTOP is not clearly defined in the literature and there is no consensus on whether or not treatment of HTOP is beneficial.To describe the epidemiologic characteristics of HTOP and to determine the population at risk of HTOP.Ninety-seven pre-term newborns under 32 WG were prospectively included in this study. FT4 and thyroid-stimulating hormone (TSH) serum levels were assessed between day of life 5 and 7. HTOP was defined as serum level of FT4 0.80 ng/dl or less and TSH less than 20 mUI/l.The HTOP incidence was 29% in pre-term newborns under 32 WG and 64% in pre-term newborns 28 WG or less. FT4 levels were correlated with gestational age (P0.001). The incidence of hypotension (61% vs 33%; P0.05), patent ductus arteriosus (50% vs 17%; P0.05), dopamine treatment (39% vs 16%; P0.05), and hydrocortisone treatment (25% vs 6%; p0.05) was higher in the HTOP group. Similarly, severe intracerebral hemorrhage (14% vs 0%; P0.01) and hypothermic events under 36 °C (1.8 ± 1.7 vs 0.0 ± 0.4; P0.05) were higher in the HTOP group.Incidence of HTOP is high in pre-term newborns born at less than 28 WG. Morbidity during the first week of life is higher in cases of HTOP. Whether or not treatment of all pre-term with l-thyroxin is beneficial is unknown. However, treatment of the subgroup of pre-term newborns under 28 WG with HTOP should be considered.

Cystinuria is an inherited autosomal-recessive disorder of renal reabsorption of the dibasic amino acids. It is the cause of about 6% of all kidney stones observed in children. Cystine is relatively insoluble at the physiological pH of urine. Cystine stones are characteristic and frequent recurrences are observed. We report on 4 cases and describe the initial presentation (obstructive renal failure, urinary sepsis, familial screening) and the medical and surgical management. Medical management is mainly based on hyperhydration and urine alkalinization. Long-term therapy with sulfhydryl agents to prevent formation of renal stones seems to be effective but adverse side effects are frequent, requiring the withdrawal of treatment. Urological management has evolved from surgical stone removal to minimally invasive procedures (extracorporeal shock wave lithotripsy, ureteroscopy).

Adynamic bone disease, characterized by a low bone formation rate with normal or reduced amount of unmineralized osteoid, is supposed to be the consequence of aluminum intoxication in uremic patients. However, the emergence of adynamic bone disease has been recently reported in hemodialyzed patients in the total absence of aluminum overload. This study was aimed to assess whether such a histological pattern of adynamic bone disease was already present in uremic patients not yet on dialysis. Twenty-seven asymptomatic uremic patients (mean age +/- SD 43 +/- 10 years, mean creatinine clearance 19 +/- 3 ml/mm) were studied and bone biopsies were repeated in 16 of them after 18 +/- 10 months of treatment with oral calcium carbonate (1-3 g of elemental calcium/day) and calcidiol (21 +/- 14 micrograms/day). None of the patients received aluminum hydroxide, and the search for bone aluminum deposits was negative in all patients both before and after treatment. Two patients fulfilled the criteria of adynamic bone disease on their post-treatment biopsies. They originated from patients classified as having normal bone histology before treatment. Comparison with the other patients showed that they had comparable plasma C-terminal PTH but higher plasma creatinine than patients with normal bone histology and lower plasma C-terminal PTH than patients with osteitis fibrosa but comparable plasma creatinine. The plasma levels of 1,25(OH)2D reached values above normal after treatment in these two patients. It is suggested that adynamic bone disease not related to aluminum intoxication can develop in uremic patients independently of dialysis, and is favored by a relative hypoparathyroidism for the degree of renal failure, possibly induced by elevated plasma concentrations of calcitriol.

Despite low end dialysis serum phosphate levels (Pe) the control of phosphate retention remains often unsatisfactory in dialyzed patients. In order to assess the value of Pe in dialyzed children as an indicator of dialytic phosphate removal, we studied serum phosphate kinetics over the period of dialysis and post dialysis and compared these with urea kinetics. A multicenter study was conducted in the 21 French pediatric hemodialysis units and included 144 children under 15 years of age. Blood urea and phosphate concentrations were measured at the beginning, at 45 min later, at the end of dialysis, and 30 min post dialysis. At 60 min and at 360 min post dialysis measurements were made only for a subgroup of 12 children. From the serum levels, reduction ratios for urea (URR) and phosphate (PRR) and post dialysis rebound for urea (PDUR) and phosphate (PDPR) were calculated. URR (over the dialysis session, 72%±9%) was higher than PRR (47%±12%). Moreover, urea removal continued throughout the dialysis period, while most of the reduction in phosphate occurred in the initial dialysis period. Post dialysis urea rebound was limited to the 60th min post dialysis, whereas post dialysis phosphate rebound occurred until the 360th min post dialysis; by this time the serum phosphate levels had almost reached the predialysis levels. In summary, serum phosphate kinetics over dialysis and post dialysis periods in children appear to be misleading for the quantification of phosphate removal, i. e., phosphate clearance is a poor indicator of dialytic phosphate removal.

The increasing prevalence of adolescent obesity justifies widespread effort and attention of the paediatrician. In order to manage, following points are to be documented: (I) weight status assessed on the basis of body mass index (BMI)split in two levels: obesity grade I:or=97 ème centile, obesity grade 2: level IOTFC 30; (2) complete anamnesis including age at adiposity rebound and existence of overweight in family; (3) research of associated morbidity and medical risks;(4) assessment of food intake and feeding practice; (5) assessment of physical activity; (6) talk with the adolescent to assess the psychological status, looking after depressive symptoms, anxiety, loss of self esteem. Once the items are documented, it allowed making a plan to manage the obesity in alliance with the adolescent and his family.

International audience; The aim of this study was to determine the prevalence of celiac disease (CD) markers in a French cohort of 84 children type 1 diabetics. Detection of antitransglutaminase (AtTG), antiendomysium (AEA) and antigliadin (AGA) antibodies was performed. Group 1 included 81 (96.4%) diabetic patients with negative antibodies. Group 2 included 3 patients (3.6%) with positive serological markers: 1 AGA-AEA-AtTG and 1 AEA-AtTG with proved histological diagnosis and 1 AGA positive with negative histology. No statistically significant difference was observed between the groups with regard to age, duration of diabetes, familial target stature, and ratios Height/Age and Weight/Height. Presence of CD serological markers was related to a lower level of HbA1c. Prevalence of CD serological markers is important in this French cohort but lower than other countries.

Neonatal renal venous thrombosis may result in severe morbidity. Predisposing conditions are well known. We report the case of an unusual and early neonatal renal venous thrombosis. The mother received an electrical shock at 34 weeks gestation. This case demonstrates that maternal electrical shock effect on the fetus should be early investigated.

The aim of this study was to evaluate the change in ureteropelvic junction management, from surgical correction to observation of the hydronephrosis. We checked wether or not it was deleterious for kidney.We retrospectively reviewed 96 charts between 1988 and 1998. Initial ultrasonography, and voiding cystourethrogram were available for all cases. Intravenous urography and diuretic renography were studied when available. Minimal follow-up of patients was one year. Patients were divided into three groups: surgery right away, surgery after observation, and observation only.Later was the diagnosis, more significant were the hydronephrosis and impairment of renal function (p0.01). In group operated on right away (69 cases), drainage improved, hydronephrosis decreased, but renal function did not improve significantly (p = 0.37). Sixteen patients were operated on after observation without deleterious effect for kidney. Eleven patients were only observed: hydronephrosis progressively decreased. Initial radiological results were not different between the last two groups (p0.05), and were not predictive of their course.Initial non operative management of hydronephrosis was not dangerous for renal function. It is advisable to detect at the earliest all signs of obstruction, because surgery improves renal drainage but not renal function.

Central venous catheter (CVC) are being increasingly used as hemodialysis vascular access. In order to evaluate the indications, practice and uses in the pediatric population, we conducted a study in children with renal failure (RF). A questionnaire was sent to the 21 french paediatric hemodialysis centers to inquire population deserved, number, type, site and indications of CVC, procedure of management and attitude towards infection and thrombosis. A prospective survey was added to compile information about CVC during the period 7/2000-31/12/2000. Results (exhaustivity response 80%) showed 1. large diversity in choice of material with predominance of pediatric permcath QUINTON; 2. exclusive surgical insertion for 8 out of 19 centers; 3. rare use of femoral route (never and exceptionally reported, respectively by 4 and 6 centers and predominantly in acute RF; 4. heterogene attitude towards fixing, dressing, heparine locking and procedure of urokinase administration in case of thrombosis, whilst procedure towards infection was homogeneous; 5. indications of insertion of the 31 prospective study CVC were distributed in 4 subgroups a) breakdown of vascular permanent access (6 cases) or of peritoneal dialysis (2 cases), b) unexpected terminal renal failure (6 cases), c) deliberate choice to wait transplantation (4 cases) or to face breakdown of transplantation (4 cases), d) dysfunction of a previous CVC (7 cases); 6. a rate of 20% of dysfunction (flow problem); 7. a rate of replacement of 11%. AVC are effective forms of vascular access. However CVC indications should be carefully analysed and balanced if possible with alternative route such as anticipated creation of arterioveinous fistula, peritoneal dialysis and preemptive transplantation.

Comparing Power Doppler imaging versus technetium-dimercapto-succinic-acid (Tc-DMSA) scintigraphy in acute pyelonephritis of childhood.First episode of urinary tract infection, clinical and biological findings suggesting an upper lesion, absence of urological malformation or obstruction, absence of reflux (or vesico-ureteral reflux inferior to grade 3). Number of patients: 49, length of the study: 26 months (from November 95 to January 98).Tc99m-DMSA scintigraphy (after five days), B mode and Power Doppler imaging (on the day of admission or the following day). Systematic cystography (day 5 to day 30).In terms of positive diagnosis, scintigraphy was superior to Power Doppler, and the latter was superior to B mode ultrasonography. Sensitivity (scintigraphy being the gold standard) was equal for both B mode and Power DopplerUS imaging, but combined Power Doppler and B mode US provided improved results.Currently, the results with Power Doppler imaging are insufficient to replace DMSA scintigraphy. However, Power Doppler is a good complement to B mode US.

A retrospective survey concerning all patients ages 12-19 who had registered themselves at the admitting service of the paediatric emergency unit in the university hospital centre of Amiens was carried out in 1995. 2812 adolescents were received (18% of total admissions). The male/female sex-ratio was 1 to 3. The average age was 13.2 years old. Hospitalisations were more frequent in the spring and were predominantly on Mondays (17%). More than 2/3 of the adolescents were accompanied by their parents and 20% were referred by their doctors or by the SAMU (mobile emergency medical service). Two percent arrive alone. Traumatic pathology is the main reason for admission (1960 patients). Organic pathologies represent 24% of admissions and are essentially digestive pathologies (40%). "Suffering" adolescents (defined as those requiring an intervention with a psychotherapist as a matter of priority) only represent 6% of visits to the emergency unit, of which 2/3 correspond to suicide attempts. 24% of adolescents are hospitalised at the university hospital centre of Amiens within the paediatric services or specialised services (ENT, stomatology, etc.).

Recent studies have shown a high prevalence of calcium and vitamin D deficiencies in adolescents. The aim of this present study was to follow the changes in calcium status and 25 hydroxyvitamin D (25[OH]D) and parathyroid hormone (iPTH) levels during winter in preadolescents and adolescents from four university hospitals in northern France.Two groups of teenagers and adolescents (range: 10-15 years) were followed from October 1996 to June 1997. They were given either 100,000 IU of vitamin D (treated group n = 33) or a placebo (control group n = 35) in October, January and April. Serum calcium, phosphate, 25(OH)D and iPTH levels were measured at inclusion and every three months thereafter.At inclusion, plasma or serum 25(OH)D levels wereor = 10 ng/mL in 16 subjects and6 ng/mL in six. In control children, no significant change in 25(OH)D occurred during the study, while plasma or serum iPTH levels increased to 34 +/- 11 pg/mL. In the treated groups, 25(OH)D levels remained20 ng/mL in every subject; no hypercalcemia was observed; and the mean plasma or serum iPTH level was 25 +/- 14 pg/mL at the end of the study.Teenagers presented with a high prevalence of biological vitamin D deficiency at the end of summer. The increase of iPTH during winter in the unsupplemented group suggests that this has secondary consequences on their calcium homeostasis unless they are supplemented with vitamin D. We advocate a sufficient calcium supply and a 100,000 IU vitamin D supplement given two or three times during winter to preadolescents and adolescents living in northern France.

Cardiac involvement rarely occurs in classic hemolytic uremic syndrome (HUS); it is often fatal.The first patient, a 21-month-old boy, developed classic HUS with acute renal failure. Peritoneal dialysis was performed for 20 days. On the 10th day of dialysis, myocardial infarction occurred, probably related to coronary thrombus. The patient was given heparin and antibiotics because of an unexplained fever. The outcome was favorable despite antero-apical cardiac necrosis, and moderated chronic renal failure. The second patient, a 24-month-old girl, also showed a classic HUS, which required peritoneal dialysis for 10 days. Dilated cardiomyopathy with cardiac failure appeared on the 4th day of dialysis, not related to the volume overload and metabolic consequences of the acute renal failure, such as systemic hypertension or ineffective dialysis. On the 5th day of dialysis neurological involvement appeared. Neurological, cardiac and renal outcome was favorable. The third patient, a 25-month-old girl, developed a classical HUS, requiring peritoneal dialysis for 25 days. No cardiac insult appeared during the acute phase of the disease. After dialysis, the child had chronic renal failure (creatinine clearance: 15 mL/min/1.73 m2). Dilated cardiomyopathy appeared 3 months later, without definite etiology. The outcome was favorable with digoxin treatment.A cardiac involvement should also be searched for in the acute phase of HUS and several months later.

Despite low end dialysis serum phosphate levels (Pe) the control of phosphate retention remains often unsatisfactory in dialyzed patients. In order to assess the value of Pe in dialyzed children as an indicator of dialytic phosphate removal, we studied serum phosphate kinetics over the period of dialysis and post dialysis and compared these with urea kinetics. A multicenter study was conducted in the 21 French pediatric hemodialysis units and included 144 children under 15 years of age. Blood urea and phosphate concentrations were measured at the beginning, at 45 min later, at the end of dialysis, and 30 min post dialysis. At 60 min and at 360 min post dialysis measurements were made only for a subgroup of 12 children. From the serum levels, reduction ratios for urea (URR) and phosphate (PRR) and post dialysis rebound for urea (PDUR) and phosphate (PDPR) were calculated. URR (over the dialysis session, 72% +/- 9%) was higher than PRR (47% +/- 12%). Moreover, urea removal continued throughout the dialysis period, while most of the reduction in phosphate occurred in the initial dialysis period. Post dialysis urea rebound was limited to the 60th min post dialysis, whereas post dialysis phosphate rebound occurred until the 360th min post dialysis; by this time the serum phosphate levels had almost reached the predialysis levels. In summary, serum phosphate kinetics over dialysis and post dialysis periods in children appear to be misleading for the quantification of phosphate removal, i.e., phosphate clearance is a poor indicator of dialytic phosphate removal.

1. 1 alpha (OH) vitamin D3 derivatives have an inconstant long term inhibitory effect on PTH secretion. As a matter of fact they act by three mechanisms, one of these being antagonistic: 1) a direct inhibitory action on the prepro-PTH gene; 2) an indirect inhibitory action by increasing plasma calcium; 3) an indirect stimulatory action by increasing plasma phosphate. These two latter phenomena are due to the stimulation of the intestinal absorption of these ions as well as to an intrinsic osteolytic action which may override the inhibition of the bone release of these ions in relation with the decrease of the PTH plasma levels. 2. The use of 1 alpha (OH)D3 derivatives in patients on chronic dialysis is justified in about 30% of the patients on dialysis when in spite of native vitamin D repletion and adequate predialysis control of plasma calcium (2.5 +/- 2 mmol/l) and of plasma phosphate (1.4 - 1.7 mmol/l), the PTH plasma levels are 3 or 5 fold the upper limit of normal whether the patient is on hemodialysis or on CAPD. When hyperphosphatemia is1.7 mmol/l it is first necessary to correct it by the use of higher doses of alkaline calcium salts given with the meals as phosphate binder together with a negative perdialytic calcium balance induced by a lower dialysate calcium in order to avoid hypercalcemia. Control of hyperphosphatemia is indeed a necessary prerequisite for the long term PTH suppressive efficacy of 1 alpha OH vitamin D derivatives. 3. The use of 1 alpha(OH)D3 derivatives in the treatment of the predialysis uremic patients is even more limited because there is no additional mean to decrease the risk of hypercalcemia when oral calcium is used as phosphate binder because of the danger of aluminum and magnesium phosphate binders. Fortunately in the adult, oral calcium used as phosphate binder in association with phosphate restriction and correction of possible vitamin D depletion and acidosis is usually efficace to control hyperparathyroïdism without 1 alpha OH vitamin D3. This is not the case in the child to whom protein and phosphate restriction should not be prescribed because of its incompatibility with the Recommended Diet Allowance. Fortunately the high remodeling rate of his growing bones, decreases the risk of hypercalcemia due to the combination of CaCO3 and 1 alpha OH vitamin D3.