Quality of life in children with severe forms of idiopathic nephrotic syndrome in stable remission-A cross-sectional study.

AIM: Severe forms of idiopathic nephrotic syndrome (INS) require immunosuppressive therapy: oral treatment or intravenous therapy (rituximab, RTX). The main objective was to describe quality of life (QOL) in these specific patients. METHODS: Cross-sectional, multicentre, observational study analysed QOL using a standardised questionnaire in children from 7 to 17 years, with a steroid-dependent or steroid-resistant INS in stable remission. The questionnaire consisted of 30 questions concerning physical and emotional well-being, self-esteem, family, friends, school and disease resulting in a global score of 0-100. RESULTS: A total of 110 patients with a mean age of 11.6 years from three French paediatric nephrology centres were included. A total of 71 patients had oral immunosuppressive treatment, 27 had RTX, and 12 had both. 13.6% of patients had a steroid-resistant INS. The mean number of relapses was 5.8. Seventy-eight patients answered the questionnaire. The global score in the whole study population was 74.7; 72.6 in the RTX group, 76.2 in the oral drugs group, (P = 0.49). The results of sub-dimension 'school' were statistically lower in RTX group (61.6 ± 19.5) compared with oral drugs group (71.4 ± 16; P = 0.02). CONCLUSION: Global QOL score was high in 'difficult-to-treat' patients with INS in stable remission on oral immunosuppressive or RTX treatment.

A comparison of individual and ecological indicators of social deprivation and their association with hospital efficiency in the context of infectious diseases in two French general paediatric departments.

BACKGROUND: To identify deprivation indicators usable in everyday practice and included in medico-administrative databases, particularly with infectious diseases, which represent the greatest proportion of hospitalizations. Our objective was to compare ecological indicators to individual questionnaires and apply both types to the study of the impact of deprivation on hospital efficiency. METHODS: We conducted an epidemiological observational prospective multicentre study in two French public hospitals between 20 October 2016 and 20 March 2017. Children hospitalized for one of the four most common infectious diseases were included and their parents were asked to answer the Evaluation of Precarity and Inequalities in Health Examination Centers (EPICES) questionnaire. The ecological indicator French DEPrivation index (FDep) was derived from patients' address, both at the zip code and at a smaller geographical area (IRIS [ilôts de regroupement pour l'information statistique]) level. Correlation and concordance between the three indicators were assessed. The endpoint used to assess the impact on hospital efficiency was the ratio between patients' length of stay (LOS) and the national LOS of their disease-related group. RESULTS: Data were available for 540 patients with a mean age of 9 mo. A total of 56.1% of patients were considered deprived with EPICES, 50.4% with zip code FDep and 45.7% with IRIS FDep. Concordance between EPICES and either type of FDep was <0.1. There was no increase in LOS compared with national LOS with any of the indicators. CONCLUSIONS: Individual and ecological indicators do not measure the same aspects of deprivation. The decision to use one or the other must be carefully weighed when studying the impact of deprivation on the healthcare system.

Acute Renal Failure after Abdominal Trauma: Renal Artery Spasm Hypothesis in Ischemic Infarction in a 12-Year-Old Girl.

Posttraumatic renal failure is often due to postischemic renal infarction, caused by identified vascular lesions. In our patient, a 12-year-old girl with acute anuric renal failure requiring hemodialysis after severe abdominal trauma, no vascular lesion or thrombosis was identified. Nevertheless, CT-scan and renal biopsy showed typical lesions of diffuse bilateral renal ischemic necrosis. The main hypothesis is a severe bilateral arterial vasospasm after a blunt abdominal trauma. The patient recovered only partially with persisting chronic renal failure.

[Diagnostic strategy in pediatrics soft tissue sarcomas]. / Stratégie diagnostique des sarcomes des tissus mous de l'enfant et adolescent.

Soft tissue sarcomas in children are rare tumor, representing around 6 to 7% of children cancer. They spread mostly sporadically (90%) and therefore are rarely associated to an underlying constitutional genetic disease (10%). About half of those sarcomas are rhabdomyosarcomas and the others are a very heterogenous histologic group with various bio-pathologies and prognosis. Clinical presentation is mainly a soft tissue lump often difficult to distinguish from more frequent benign causes (malformative, infectious, benign, or pseudotumor). Inappropriate initial diagnosis work-up has a strong impact on soft tissue sarcomas' prognosis. Adapted complementary investigations (first ultrasound and MRI) are important to compile arguments for a malign origin and to indicate a biopsy. However, predictive value of imaging exams still remains imperfect, and histological analysis by percutaneous image-guided biopsy and sometimes by surgical biopsy is often necessary. Authors realize an update on optimal diagnostic pathway including molecular tests in presence of a soft tissue mass in a child.

[Evolution of pharmacological treatments in paediatric oncology]. / Évolution des traitements médicamenteux en oncologie pédiatrique.

With around 1 800 new cases each year in children under the age of 15 and 800 new cases a year in teenagers, childhood cancers are a major public health issue. Chemotherapy remains a key component in the treatment of childhood cancers but the advances made in genetics has opened the way to targeted therapies which enable the treatment to be adapted to each child significantly improving survival and reducing sequelae.