Vitulia questionnaire: a new instrument to evaluate quality of life in children aged 4-7 years with food allergy.

Summary: Background. Food allergy (FA) negatively affects health-related quality of life (HR-QoL) of children and caregivers. To date, no questionnaire self-compiling assessing the HR-QoL in pre-school children with FA is available. The aim of this study is to develop and validate a self-administered, rapid and easy questionnaire to evaluate the HR-QoL in children ≥ 7 years with IgE-mediated FA. Methods. A two-center prospective study was conducted including children aged 4-7 years with IgE-mediated FA. The Vitulia questionnaire was administered to study participants at the baseline (T0) and after one month (T1). To assess the feasibility and reliability, the Vitulia questionnaire was compared with other two pre-existing questionnaires: FAQLQ-PF and the KiddyKindl, which were also tested at both T0 and T1. The validation phase aimed to assess the following psychometric properties: convergent validity, internal consistency, discriminant validity and sensitivity to change. Results. One hundred patients (62% male, mean age 5.4 ± 1.2 years) were enrolled. The Vitulia questionnaire showed a good internal consistency along with an excellent reliability and repeatability of the measure. Another noteworthy feature of the questionnaire was its discriminant validity as demonstrated by the ability to provide different scores in subgroups, which have differences in terms of quality of life. On the other hand, the questionnaire seems not be sensitive to changes in health status over time. Conclusions. The Vitulia questionnaire represents a valid tool, quick and easy to interpret, which can be used to assess the quality of life in preschool children with IgE-mediated FA.

Asthma incidence can be influenced by climate change in Italy: findings from the GEIRD study-a climatological and epidemiological assessment.

An association between climatic conditions and asthma incidence has been widely assumed. However, it is unclear whether climatic variations have a fingerprint on asthma dynamics over long time intervals. The aim of this study is to detect a possible correlation of the Summer North Atlantic Oscillation (S-NAO) index and the self-calibrated palmer drought severity index (scPDSI) with asthma incidence over the period from 1957 to 2006 in Italy. To this aim, an analysis of non-stationary and non-linear signals was performed on the time series of the Italian databases on respiratory health (ISAYA and GEIRD) including 36,255 individuals overall, S-NAO, and scPDSI indices to search for characteristic periodicities. The ISAYA (Italian Study on Asthma in Young Adults) and GEIRD (Gene Environment Interactions in Respiratory Diseases) studies collected information on respiratory health in general population samples, born between 1925 and 1989 and aged 20-84 years at the time of the interview, from 13 Italian centres. We found that annual asthma total incidence shared the same periodicity throughout the 1957-2006 time interval. Asthma incidence turned out to be correlated with the dynamics of the scPDSI, modulated by the S-NAO, sharing the same averaged 6 year-periodicity. Since climate patterns appear to influence asthma incidence, future studies aimed at elucidating the complex relationships between climate and asthma incidence are warranted.

Serious Games: A new Approach to Foster Information and Practices About Covid-19?

The current Covid-19 pandemic poses an unprecedented global challenge in the field of education and training. As we have seen, the lack of proper information about the virus and its transmission has forced the general population and healthcare workers to rapidly acquire knowledge and learn new practices. Clearly, a well-informed population is more likely to adopt the correct precautionary measures, thus reducing the transmission of the infection; likewise, properly educated healthcare workers are better equipped to manage the emergency. However, the need to maintain physical distancing has made it impossible to provide in-presence information and training. In this regard, new technologies have proved to be an invaluable resource by facilitating distance learning. Indeed, e-learning offers significant advantages because it does not require the physical presence of learners and teachers. This innovative method applied to serious games has been considered potentially effective in enabling rapid and large-scale dissemination of information and learning through content interactivity. We will review studies that have observed the development and use of serious games to foster information and practices about Covid-19 aimed at promoting behavioral changes in the population and the healthcare personnel involved on the front line.

Issue 1 - "Update on adverse respiratory effects of outdoor air pollution". Part 1): Outdoor air pollution and respiratory diseases: A general update and an Italian perspective.

OBJECTIVE: to summarize the main updated evidence about the health effects of air pollution and to focus on Italian epidemiological experiences on the respiratory effects. RESULTS: the recent literature indicates that there is strong evidence for causal relationships between PM2.5 air pollution exposure and all-cause mortality as well as mortality from acute lower respiratory infections, ischaemic heart disease, stroke, chronic obstructive pulmonary disease, and lung cancer. A growing body of evidence also suggests causal relationships with type II diabetes and impacts on neonatal mortality from low birth weight and short gestation as well as neurologic effects in both children and adults. Italy, a Southern European country, faces a more threatening air pollution challenge because of the effects of both anthropogenic pollutants and natural dust (particulate matter, PM). The 2020 Report of the European Environment Agency highlighted the number of premature deaths in Italy attributable to main pollutants: 52,300 for PM2.5, 10,400 for NO2 and 3,000 for O3 in 2018. In Italy, original time series and analytical epidemiological studies showed increased cardio-respiratory hospital admissions and mortality and increased risk of respiratory diseases in people living in urban areas. CONCLUSIONS: adverse health effects of air pollutants, even at low levels, have been confirmed by recent epidemiological studies. Further studies should focus on the potential link between air pollution exposure and respiratory infections. This topic has become particularly important in the current SARS-COV-2 pandemic. Based on strong scientific evidence, the Italian government, which hosts the Global Alliance against Chronic Respiratory Diseases (GARD)-Italy at the Ministry of Health, the scientific respiratory societies and the patients' associations, as well as others in the health sector and civil society, must increase their engagement in advocacy for clean air policies, especially in light of the new Air Quality Guidelines of the World Health Organization.

Atopic Dermatitis Phenotypes in Preschool and School-Age Children: A Latent Class Analysis.

BACKGROUND: Atopic dermatitis (AD) is the most common chronic inflammatory skin disease in childhood. Few data are available about AD phenotypes and their nationwide distribution. METHODS: We performed a cross-sectional multicenter study involving some of the main Italian pediatric allergy centers from 9 Italian cities. A structured questionnaire was administered to 371 children with AD. Patients were divided in 2 groups: preschool children (aged ≤5 years) and schoolchildren (aged 6-14 years). A latent class analysis was used to detect AD phenotypes and to investigate their association with risk factors and other atopic diseases. RESULTS: Latent class analysis identified 5 AD phenotypes in preschoolers ("moderate-severe AD, high comorbidity", 8%; "moderatesevere AD, low comorbidity", 35%; "mild AD, low comorbidity", 20%; "mild AD, respiratory comorbidity", 32%; "mild AD, food-induced comorbidity", 5%) and 4 AD phenotypes in schoolchildren ("moderate-severe AD, high comorbidity", 24%; "moderate-severe AD, low comorbidity", 10%; "mild AD, low comorbidity", 16%; "mild AD, respiratory comorbidity", 49%). Parental history of asthma and eczema, early day-care attendance, and exposure to molds were significantly associated with the "moderate-severe AD, high comorbidity" phenotype in preschool children (P<.05). The "moderate-severe AD" phenotypes were also associated with the highest burden in terms of medication use and limitations in daily activities. CONCLUSIONS: The detection of different AD phenotypes highlights the need for a stratified approach to the management of this complex disease and for further studies to predict the course of AD and to develop more efficient therapeutic strategies.

Atopic dermatitis phenotypes in preschool and school-age children: a latent class analysis

BACKGROUND: Atopic dermatitis (AD) is the most common chronic inflammatory skin disease in childhood. Few data are available about AD phenotypes and their nationwide distribution. METHODS: We performed a cross-sectional multicenter study involving some of the main Italian pediatric allergy centers from 9 Italian cities. A structured questionnaire was administered to 371 children with AD. Patients were divided in 2 groups: preschool children (aged ≤5 years) and schoolchildren (aged 6-14 years). A latent class analysis was used to detect AD phenotypes and to investigate their association with risk factors and other atopic diseases. RESULTS: Latent class analysis identified 5 AD phenotypes in preschoolers ("moderate-severe AD, high comorbidity", 8%; "moderatesevere AD, low comorbidity", 35%; "mild AD, low comorbidity", 20%; "mild AD, respiratory comorbidity", 32%; "mild AD, food-induced comorbidity", 5%) and 4 AD phenotypes in schoolchildren ("moderate-severe AD, high comorbidity", 24%; "moderate-severe AD, low comorbidity", 10%; "mild AD, low comorbidity", 16%; "mild AD, respiratory comorbidity", 49%). Parental history of asthma and eczema, early day-care attendance, and exposure to molds were significantly associated with the "moderate-severe AD, high comorbidity" phenotype in preschool children (P<.05). The "moderate-severe AD" phenotypes were also associated with the highest burden in terms of medication use and limitations in daily activities. CONCLUSIONS: The detection of different AD phenotypes highlights the need for a stratified approach to the management of this complex disease and for further studies to predict the course of AD and to develop more efficient therapeutic strategies

ANTECEDENTES: La dermatitis atópica (DA) es la enfermedad crónica cutánea más frecuente en la infancia. Hay pocos datos disponibles sobre los diferentes fenotipos de DA y su distribución geográfica. MÉTODOS: Se realizó un estudio transversal multicéntrico en nueve de los principales centros italianos de alergia infantil. Se realizó un cuestionario a 371 con DA. Los pacientes fueron divididos en dos grupos: preescolares (<5 años) y escolares (6-14 años). Se empleó un análisis de clases latentes (ACL) para establecer los fenotipos de la DA y su asociación con factores de riesgo y otras enfermedades atópicas. RESULTADOS: El ACL identificó cinco fenotipos de DA en el grupo preescolar (8% DA moderada-severa con alta comorbilidad, 35% DA moderada-severa con baja comorbilidad, 20% DA leve con baja comorbilidad, 32% DA leve con patología respiratoria asociada, 5% DA leve con alergia alimentaria asociada) y cuatro fenotipos en escolares (24% DA moderada-severa con alta comorbilidad, 10% DA moderada-severa con baja comorbilidad, 16% DA leve con baja comorbilidad, 49% DA leve con patología respiratoria asociada). Los antecedentes familiares de asma y eccema, la asistencia temprana a guardería y la exposición a hongos se asociaron al fenotipo DA moderada-severa con alta comorbilidad en niños preescolares (p < 0,05). Los fenotipos moderados-severos requerían mayor uso de medicación y tenían mayores limitaciones para su actividad diaria. CONCLUSIONES: La clasificación de la DA en diferentes fenotipos implica la importancia de un tratamiento estratificado para esta compleja enfermedad así como la necesidad de estudios capaces de predecir el curso de la DA y con ello desarrollar estrategias de tratamiento más eficientes

Is alpha-fetoprotein decline a prognostic factor of childhood non-seminomatous germ cell tumours? Results of the French TGM95 study.

PURPOSE: In adults' non-seminomatous germ cell tumours (NS-GCT), alpha-fetoprotein (AFP) decline was identified as an important prognostic factor. We investigated its prognostic value in the French TGM95 study for childhood NS-GCT. PATIENTS AND METHODS: Three risk groups were defined: low risk (LR: localised and completely resected pS1, AFP<15000 ng/ml), with a 'wait-and-see' strategy; intermediate-risk (IR: localised incompletely resected, AFP<15000 ng/ml) with 3-5 vinblastine-bleomycine-cisplatin courses; high risk (HiR: AFP≥15000 ng/ml and/or metastatic) with 4-6 etoposide-ifosfamide-cisplatin courses. The multivariable prognostic analysis for progression-free survival (PFS) included age (±10 years), primary tumour site (1-testis, 2-ovary, 3-extragonadal), extent of disease (1-pS1, 2-loco-regional dissemination, 3-metastasis) and AFP (±10,000 ng/ml). AFP decline prognostic value was investigated in IR + HiR groups using predicted time to normalisation (TTN), AFP change, and difference between observed and expected (based on AFP half-life) area under the curve (O-E AUC). RESULTS: From January 1995 to December 2005, 239 patients (median age = 3years, 60 LR, 65 IR, 114 HiR) were included. Main sites were testis (n = 66), ovary (n = 77) and sacrococcygeal (n = 57). Five-year PFS and OS were 85% (95% confidence interval [CI] = 80-89%) and 93% (89-95%), respectively. Age ≥ 10 years (hazard ratio [HR] = 4.6, 95% CI = 2.1-10.1, p = 0.0001) and extragonadal primary (HR = 6.3, 95% CI = 2.0-19.9, p = 0.005) were significant prognostic factors. In AFP decline analysis (n = 151, 17 events), TTN (p = 0.61) and AFP change (p = 0.10) were not prognostic, whereas we showed a significant effect of O-E AUC (HR = 2.1, 95% CI = 1.0-4.2, p = 0.05). CONCLUSION: Age ≥ 10 years and extragonadal tumours remain as poor prognostic factors. Contrary to adults, TTN is not reliable in paediatric NS-GCT. The prognostic value of O-E AUC should be investigated in larger studies.

Comparative Effect of Beclomethasone Dipropionate and Cetirizine on Acoustic Rhinometry Parameters in Children With Perennial Allergic Rhinitis: A Randomized Controlled Trial.

BACKGROUND AND OBJECTIVES: The effect of intranasal corticosteroids and oral antihistamines on acoustic rhinometry parameters has not been directly compared. The primary objective was to compare the effect of a 21-day course of treatment with nasal beclomethasone dipropionate (nBDP) with that of cetirizine (CTZ) on nasal patency measured using acoustic rhinometry in children with perennial allergic rhinitis (PAR). The secondary objective was to compare the effect of both drugs on nasal cytology, symptom severity, sleep quality, and quality of life. METHODS: In this 21-day, open-label, randomized controlled study, 34 children with PAR (age 6-14 years) with a Total 5-Symptom Score (T5SS) ≥5 received nBDP 100 µg per nostril twice daily or CTZ 10 mg tablets once daily. The measures of effect were the least square mean change (LSmc) in nasal volume, minimal cross-sectional area (MCA), and nasal cytology, as well as the scores on the T5SS, Pittsburgh Sleep Quality Index (PSQI), and Paediatric Rhinoconjunctivitis Quality of Life Questionnaire (PRQLQ). RESULTS: After 21 days, nBDP improved nasal volume and MCA more than CTZ (LSmc, 2.21 cm3 vs 0.20 cm3 [P=.013]; and LSmc 0.63 cm2 vs 0.13 cm2 [P=.002], respectively). Compared with the CTZ group, a more marked improvement was found in the nBDP group with respect to eosinophil classes (LSmc, -1.10 vs -0.40; P=.031) and neutrophil classes (LSmc, -0.97 vs -0.17; P=.010), T5SS (LSmc, -5.63 vs -3.54; P=.008), PSQI (LSmc, -1.30 vs -0.19; P=.025), and PRQLQ total scores (LSmc, -1.15 vs -0.69; P=.031). CONCLUSIONS: In children with PAR, nBDP is more effective than CTZ in improving nasal patency measured by acoustic rhinometry, with associated beneficial effects on nasal cytology, symptoms, sleep quality, and quality of life.

Comparative effect of beclomethasone dipropionate and cetirizine on acoustic rhinometry parameters in children with perennial allergic rhinitis: a randomized controlled trial

Background: The effect of intranasal corticosteroids and oral antihistamines on acoustic rhinometry parameters has not been directly compared. Objectives: The primary objective was to compare the effect of a 21-day course of treatment with nasal beclomethasone dipropionate (nBDP) with that of cetirizine (CTZ) on nasal patency measured using acoustic rhinometry in children with perennial allergic rhinitis (PAR). The secondary objective was to compare the effect of both drugs on nasal cytology, symptom severity, sleep quality, and quality of life. Methods: In this 21-day, open-label, randomized controlled study, 34 children with PAR (age 6-14 years) with a Total 5-Symptom Score (T5SS) ≥5 received nBDP 100 μg per nostril twice daily or CTZ 10 mg tablets once daily. The measures of effect were the least square mean change (LSmc) in nasal volume, minimal cross-sectional area (MCA), and nasal cytology, as well as the scores on the T5SS, Pittsburgh Sleep Quality Index (PSQI), and Paediatric Rhinoconjunctivitis Quality of Life Questionnaire (PRQLQ). Results: After 21 days, nBDP improved nasal volume and MCA more than CTZ (LSmc, 2.21 cm3 vs 0.20 cm3 [P=.013]; and LSmc 0.63 cm2 vs 0.13 cm2 [P=.002], respectively). Compared with the CTZ group, a more marked improvement was found in the nBDP group with respect to eosinophil classes (LSmc, -1.10 vs -0.40; P=.031) and neutrophil classes (LSmc, -0.97 vs -0.17; P=.010), T5SS (LSmc, -5.63 vs -3.54; P=.008), PSQI (LSmc, -1.30 vs -0.19; P=.025), and PRQLQ total scores (LSmc, -1.15 vs -0.69; P=.031). Conclusions: In children with PAR, nBDP is more effective than CTZ in improving nasal patency measured by acoustic rhinometry, with associated beneficial effects on nasal cytology, symptoms, sleep quality, and quality of life

Antecedentes: No hay estudios previos en los que se comparan los efectos sobre la rinometría acústica de los corticoides intranasales y los antihistamínicos orales. Objetivos: El objetivo principal fue comparar los efectos de un tratamiento de 21 días con dipropionato de beclometasona (nBDP) frente a ceterizina (CTZ) sobre la obstrucción nasal medida con rinometría acústica en niños con rinitis alérgica perenne (PAR). Los objetivos secundarios incluyen los efectos sobre la citología nasal, la gravedad de los síntomas, la calidad del sueño y la calidad de vida. Métodos: Estudio abierto, aleatorizado y controlado, de 21 días de duración. Se incluyeron 34 niños con PAR (6-14 años) con una puntuación de síntomas ≥5 (T5SS) que recibieron 100 μg de nBDP por fosa nasal dos veces al día o CTZ 10 mg una vez al día. Se realizaron las siguientes mediciones: cambios en los mínimos cuadrados (LSmc) del volumen nasal, del área transversa mínima (MCA), de la citología nasal, el T5SS, índice de calidad del sueño (PSQI) y el cuestionario de calidad de vida pediátrico (PRQLQ). Resultados: después de 21 días, los tratados con nBDP mejoraron el volumen nasal y el MCA más que los tratados con CTZ (LSmc 2,21 cm3,vs 0.20 cm3, p=0,013 and LSmc 0,63 cm2 vs 0,13 cm2, p=0,002, respectivamente). En el grupo tratado con nBDP, con respecto a los tratados con CTZ tuvieron una mayor mejoría en la disminución de clases de eosinófilos (LSmc -1,10 vs -0,40, p=0,031) y neutrófilos (LSmc -0,97 vs -0,17, p=0,010), en el T5SS (LSmc -5,63 vs -3,54, p=0,008), PSQI (LSmc -1,30 vs -0,19, p=0,025) y en la puntuación total de PRQLQ (LSmc -1,15 vs -0,69, p=0,031). Conclusiones: en niños con PAR, la nBDP es más efectiva que la CTZ en mejorar la obstrucción nasal medida por rinometría acústica, con los beneficios asociados sobre citología nasal, síntomas, calidad de sueño y calidad de vida

Unusual outer ear swelling: Childhood auricular rhabdomyosarcoma.

INTRODUCTION: Rhabdomyosarcoma (RMS) is the most frequent soft-tissue sarcoma in children and makes up 5% of all pediatric malignant tumors. The main head and neck locations are the base of the skull, nasopharynx, nasal cavity and orbit. An outer ear location is considered extremely rare. We present 3 cases of children, aged 6 to 14 years, presenting with auricular RMS. CASE REPORTS: The first child, aged 6, was managed by 4 chemotherapy cycles followed by surgical resection of the tumor bed, completed by 5 further cycles of chemotherapy. The second, aged 14, was managed by 4 chemotherapy cycles followed by external radiation therapy of the tumor bed and lymph node areas, completed by 5 further cycles of chemotherapy. The third, aged 13, was managed by 4 chemotherapy cycles followed by surgery, completed by 5 further cycles of chemotherapy. DISCUSSION: In these 3 patients, the treatment program achieved complete disease control. Prognosis was good, thanks to good surgical access. Diagnosis should be considered in case of unusual progressive swelling in the outer ear.

Does aggressive local treatment have an impact on survival in children with metastatic rhabdomyosarcoma?

PURPOSE: Due to the extensive initial distant tumour spread in metastatic rhabdomyosarcoma, the importance of local treatment is sometimes underestimated. A retrospective study was conducted to identify the prognostic value of aggressive local treatment in paediatric metastatic rhabdomyosarcoma. PATIENTS: Patients with metastatic rhabdomyosarcoma aged 1-21 years treated in France from 1998 to 2011 according to European protocols MMT-4-89, 4-91, 98 and recent national guidelines were selected. Survival comparison were performed between patients with 'aggressive local treatment' (surgery and radiotherapy) and exclusive surgery or radiotherapy, after exclusion of patients with early progression. End-points were event-free and overall survival (OS). RESULTS: A total of 101 children, median age 9 years, with majority of primaries in unfavourable sites (73 patients, pts), T2 tumours (66 pts), alveolar subtypes (65 pts) and large tumours (>5 cm, 83 pts) received various chemotherapy regimens. On univariate and multivariate analyses, OS was better after 'aggressive local treatment' (49 pts; 44.3 ± 8%), than after exclusive surgery (10 pts; 18.8% ± 15.5%) or exclusive radiotherapy (29 pts; 16.1 ± 7.2%, P < 0.006). Moreover, OS was better in the case of surgery with complete resection (41.1 ± 10.2%) or microscopic residue (56.4 ± 14.9%) than macroscopic residue (20.0 ± 12.6%; P < 0.03). CONCLUSIONS: In this large retrospective analysis, OS appeared to be better for patients receiving 'aggressive local treatment' even after adjustment for the initial patient and tumour characteristics. Isolated debulking surgery is associated with a very poor outcome and should be avoided. Aggressive local treatment in patients with rhabdomyosarcoma, even with metastasis, should be seriously considered.

SPRED1, a RAS MAPK pathway inhibitor that causes Legius syndrome, is a tumour suppressor downregulated in paediatric acute myeloblastic leukaemia.

Constitutional dominant loss-of-function mutations in the SPRED1 gene cause a rare phenotype referred as neurofibromatosis type 1 (NF1)-like syndrome or Legius syndrome, consisted of multiple café-au-lait macules, axillary freckling, learning disabilities and macrocephaly. SPRED1 is a negative regulator of the RAS MAPK pathway and can interact with neurofibromin, the NF1 gene product. Individuals with NF1 have a higher risk of haematological malignancies. SPRED1 is highly expressed in haematopoietic cells and negatively regulates haematopoiesis. SPRED1 seemed to be a good candidate for leukaemia predisposition or transformation. We performed SPRED1 mutation screening and expression status in 230 paediatric lymphoblastic and acute myeloblastic leukaemias (AMLs). We found a loss-of-function frameshift SPRED1 mutation in a patient with Legius syndrome. In this patient, the leukaemia blasts karyotype showed a SPRED1 loss of heterozygosity, confirming SPRED1 as a tumour suppressor. Our observation confirmed that acute leukaemias are rare complications of the Legius syndrome. Moreover, SPRED1 was significantly decreased at RNA and protein levels in the majority of AMLs at diagnosis compared with normal or paired complete remission bone marrows. SPRED1 decreased expression correlated with genetic features of AML. Our study reveals a new mechanism which contributes to deregulate RAS MAPK pathway in the vast majority of paediatric AMLs.

[Recurrent small-bowel intussusceptions revealing a disseminated BCG infection related to severe combined immunodeficiency]. / Invagination intestinale récidivante révélatrice d'une bécégite disséminée liée à un déficit immunitaire combiné sévère.

Infant small-bowel intussusceptions, most of the time idiopathic, may exceptionally reveal a severe digestive disease. We report the case of a 4-month-old infant who presented multiple and simultaneous ileal intussusceptions associated with severe acute gastroenteritis. Initially, the infant showed a protein-losing enteropathy with a clear alteration of the general state of health and undocumented fever, resistant to broad-spectrum antibiotic therapy. Skin and splenic nodules associated with hepatosplenomegaly and pancytopenia set in progressively. The etiologic evaluation led to the diagnosis of a Mycobacterium bovis BCG infection related to severe combined immune deficiency. The treatment consisted in anti-tuberculosis quadruple therapy in association with immunoglobin supplementation. Secondarily, the patient underwent gene therapy in a clinical trial. An early BCG vaccine in the first weeks of life, before the outbreak of infection revealing the immune deficiency, is a risk factor in triggering a disseminated BCG infection in immunodepressed infants. This clinical case is the first reported of severe combined immune deficiency revealed by small-bowel intussusceptions related to a disseminated BCG infection.

Ovarian-sparing surgery for ovarian teratoma in children.

BACKGROUND: Ovarian teratoma (OT) is the most common ovarian neoplasm in children. Oophorectomy has been the standard treatment but may impair fertility. The aim of this study was to investigate the feasibility and outcome of ovarian-sparing surgery (OSS) for OT. PROCEDURE: We retrospectively studied all children treated for OT at a pediatric teaching hospital in Paris, France, between March 1992 and July 2006. OSS was performed when deemed technically feasible in patients who had no lymphadenopathy by preoperative imaging or surgical exploration, normal tumor marker levels, and calcifications on radiographs. RESULTS: We identified 30 patients, including 29 with unilateral OT and 1 with synchronous bilateral OT. Emergent surgery was performed in five patients, among whom four had ovarian torsion requiring oophorectomy and one underwent OSS. Of the 26 OTs in the 25 remaining patients, 10 were managed with OSS and 16 with oophorectomy. Subsequently, ultrasound monitoring detected OT development in the contralateral ovary in 4 (14%) patients, after a median of 3 years (range, 1-14 years); OSS was performed in all four cases. The patient with bilateral synchronous OT, managed by OSS initially, underwent unilateral oophorectomy 3 years later for a recurrence. Overall OSS was performed for 15 (42%) OTs. CONCLUSIONS: Our results suggest recommendations for preserving fertility whenever possible without compromising the oncological prognosis. In particular, OSS should be reserved for patients who meet all criteria for localized mature teratoma. Long-term follow-up is crucial.

[Is there a risk of steroid-induced adrenal deficit after induction treatment of acute lymphoblastic leukemia?]. / Existe-t-il un risque de déficit corticotrope au décours du traitement d'induction d'une leucémie aiguë lymphoblastique ?

The occurrence of eight cases of adrenal deficit in children hospitalized for acute lymphoblastic leukemia (ALL) led us to conduct a prospective study from May 2006 to May 2007 to better characterize this corticoid-induced adrenal deficit. Forty of the 48 patients hospitalized for ALL were given a low-dose Synacthen test (1 µg), a mean 7 days after the induction phase. An adrenal deficit was diagnosed in 27 patients (67.5%). No significant clinical or hematological difference was identified between the "with deficit" (n = 27) and "without deficit" (n = 13) groups. The diagnosis of adrenal deficit was not more common for children who had received dexamethasone (13/19) or prednisone (14/21), or for those who had (19/29) or had not (8/11) experienced corticoid toxicity during induction. The clinical signs suggesting adrenal deficit were identical in the two groups and none of the children presented an acute episode. In biological terms, only hypoprotidemia was significantly more common in patients with adrenal deficit (p = 0.0004). Of 13 patients with a deficit at the end of the induction who had received a 2nd low-dose Synacthène(®) test before intensification no. 1, 3 weeks on average after the end of corticotherapy, only two still had a deficit. Thus, corticoid-induced adrenal deficit is a common complication in children treated for ALL, although it is not highly symptomatic. Most of these children recover normal adrenal function before intensification no. 1, but it does not eliminate the risk of a secondary deficit after other courses of corticotherapy. Systematic repeated Synacthène(®) tests in common practice among children treated for ALL does not seem justified. However, the results of this study encouraged us to propose a hydrocortisone substitution to children treated for ALL in the event of stress.

[Inherited thrombocytopenias]. / Les thrombopénies génétiques.

Secondary causes of thrombocytopenia as immunologic thrombopenia purpura, or ITP, are far more common than inherited causes, which even as a group, are rare. Nevertheless, diagnosis is important and progress made in uncovering the molecular basis of these disorders has contributed greatly to our knowledge of these diseases. Inherited thrombocytopenias are a heterogeneous group of disorders. Different criteria have been suggested to classify the forms, such as the inheritance mechanism and the platelet volume as well as the associated platelet dysfunctions or clinical abnormality. This paper describes the clinical and biological data, and current knowledge of the molecular findings of inherited thrombocytopenia, allowing a diagnostic approach to these diseases.

[How to discuss death with a dying child: a story can help?]. / Comment parler de la mort à un enfant en fin de vie: un conte peut-il être une aide?

Is it necessary - and possible - to discuss death with a child in palliative phase? How should one approach the subject? A recent Swedish study demonstrated the benefits for parents who discussed with their child his or her imminent death, and raised the question of the role nursing can play to help parents. The mother of one child treated in our unit recently wrote a story 48 hours before the child's death. The story served to broach a number of recurrent questions often raised by dying children and their families: fear of the unknown, of being replaced, the inevitability of death, grief, and fear of being forgotten... The story was given to 13 families with dying children. In order to evaluate the story's impact on families and to determine whether a document which stimulates dialogue should continue to be given to parents, we asked that they fill out a questionnaire. Fifty-five percent of parents answered, and confirmed that the story was experienced as a positive thing and helped parents to talk with their children. This study raises many questions and should be part of a global accompaniment strategy. A review of medical, general and children's literature, as well the results of our study, lead us to conclude that the medical body should lend its full support to families who wish to engage in this dialogue with their children.

[Role of Abelcet in children with febrile neutropenia]. / Intérêt d'Abelcet dans la prise en charge des enfants neutropéniques fébriles.

AIM OF STUDY: In order to optimise the use of new forms of Amphotericine B (Ampho B), a decisional tree was created at the end of 2001 in the paediatric hemato-oncology unit for the empirical antifungal treatment in febrile neutropenic children: the standard remained conventional Ampho B and Abelcet was proposed in case of antecedent or occurrence of a deterioration of the renal function (DRF). In order to validate the place of Abelcet we initiated a retrospective study over year 2002. RESULTS: 21 treatments were begun in 14 children for a median duration of 8 days (1-48 days). Three kind of indications were found: DRF antecedent (10 episodes: A group), DRF occurrence during a treatment with conventional Ampho B (7 episodes: B group), age lower than 1 year (3 episodes). 81% of the children were thus treated according to the decisional tree. The clinical tolerance was good in 90% of the cases, with a premedication in half of the cases. The study of the renal function showed a good renal tolerance for 6 episodes out of 9 evaluable in A group, 3 resolutions and 2 stabilisation of the renal failure for the 5 evaluable episodes of the B group. Seven to ten days of treatment by Abelcet were necessary to obtain the renal failure resolved. CONCLUSION: This study confirms the interest of Abelcet in the empirical antifungal treatment in febrile neutropenic children and specially in children having antecedents of DRF related or not to a treatment with conventional Ampho B.

[Severe candidosis infections in neutropenic patient in onco-hematologic department]. / Infections candidosiques sévères chez le patient neutropénique en onco-hématologie pédiatrique.

Incidence of severe candidal infections is rapidly increasing since 15 years and is becoming a major concern in onco-hematology practice, especially due to its poor prognosis in neutropenic patients. Diagnosis of candidemia is suspected in case of persistent fever resistant to a large antibiotherapy and requires to search for secondary locations as cutaneous and hepatosplenic candidal infection. Improvement of yeasts detection in blood culture bottles with specific medium is now helpful but use of specific immunoserodiagnosis or PCR methods is at this point unuseful. Fluconazole and Amphotericine B remain the recommended treatments for candidemia. Indications for "new antifongal drugs" are still limited regarding their high cost and the limited clinical studies.