Outcome of childhood ALK-positive anaplastic large cell lymphoma relapses: Real-life experience of the French Society of Pediatric Oncology (SFCE) cohort of 75 French children.

Objective: To describe treatments and outcomes of French children treated for relapsed/refractory anaplastic lymphoma kinase-positive anaplastic large cell lymphoma (ALK+ ALCL).

Methods: We conducted the analysis of a series of 75 French children treated for a first relapsed/refractory ALK+ ALCL between 1999 and 2017.

Results: The median time to first relapse was 8.

Genetics of adnexal tumors: An update.

Cutaneous adnexal tumors form a vast heterogeneous group that include frequent entities that are mostly benign, as well as rare tumors that are occasionally malignant. In contrast to cutaneous tumors arising from the interfollicular epidermis that develop as a result of accumulation of UV-induced DNA damage (basal cell carcinoma, squamous cell carcinoma), the oncogenesis of adnexal tumors is related to a broad spectrum of genetic mechanisms (e.g.

The Treat-to-Target Project in Atopic Dermatitis: One Year On.

Atopic dermatitis is a chronic skin condition for which a range of systemic treatments have recently been approved. A treat-to-target strategy has been developed previously alongside an algorithm to guide the management of patients with atopic dermatitis. Here, we review the strategy and algorithm in the context of the evolving therapeutic landscape, and identify areas for further refinement and development.

Kidney biopsy in very elderly patients: indications, therapeutic impact and complications.

Background: Few data is available on the risk/benefit balance of native kidney biopsy (KB) in very elderly patients.

Methods: Multicenter retrospective cohort study in the Aix-Marseille area: the results of KB and medical charts of all patients over 85 years biopsied between January 2010 and December 2018 were reviewed.

Results: 104 patients were included.

Pediatric hospital care organization: Cross-sectional enquiry in four regions in France.

Objective: The aim of this study was to characterize conventional pediatric care capacities in French public hospitals and identify the main difficulties in guiding upcoming health policies. The secondary objective was to assess the quality of care by the implementation of the European Charter of the Rights of Children in Hospital.

Method: Multicenter cross-sectional study using a questionnaire survey sent by e-mail to the heads of conventional pediatric departments in four French regions identified on the French Hospital Federation's website.

An integrated approach to inherited platelet disorders: results from a research collaborative, the Sydney Platelet Group.

Inherited disorders of platelet function (IPFD) and/or number (IPND) are heterogeneous conditions that result in variable mucocutaneous bleeding symptoms as a result of deranged primary haemostasis caused by platelet dysfunction or thrombocytopenia. Diagnosis is important to guide post-operative bleeding prophylactic strategies, to avoid treatment with inappropriate medications, and inform prognosis. Achieving an accurate diagnosis has traditionally been hampered by the requirement of multiple, often complex, laboratory tests that are not always available at single centres.

The influence of word frequency on word reading speed when individuals with macular diseases read text.

People with central field loss (CFL) use peripheral vision to identify words. Eccentric vision provides ambiguous visual inputs to the processes leading to lexical access. Our purpose was to explore the hypothesis that this ambiguity leads to strong influences of inferential processes, our prediction being that increasing word frequency would decrease word reading time.

Severe postoperative complications decrease overall and disease free survival in pancreatic ductal adenocarcinoma after pancreaticoduodenectomy.

Background: Postoperative complications influence overall and disease free survival after pancreaticoduodenectomy for pancreatic ductal adenocarcinoma is still a matter of debate and controversy.

Methods: The outcome of 942 consecutive patients, from the multicentric study of the French Association of Surgery, between January 2004 and December 2009 was analyzed. Perioperative data, including severe complications (grade III and above), were used in univariate and multivariate analysis to assess their influence on overall and disease free survival.

Anti-α β immunization in Glanzmann thrombasthenia: review of literature and treatment recommendations.

Glanzmann thrombasthenia (GT) is caused by inherited defects of the α β platelet glycoprotein. This bleeding disorder can be treated with platelet transfusion therapy, but some patients will be immunized and begin to form anti-human leucocyte antigen (HLA) and/or anti-α β antibodies. These antibodies can bind and interfere with the function of the transfused platelets, rendering treatment ineffective.

Early Halt of a Randomized Controlled Study with 3% Hypertonic Saline in Acute Bronchiolitis.

Background: Albeit not recommended because of contradictory results, nebulized 3% hypertonic saline is widely used for treating acute viral bronchiolitis. Whether clinical differences may be attributed to the type of nebulizer used has never been studied.

Objectives: By modifying the amount of salt deposited into the airways, the nebulizer characteristics might influence clinical response.

NUP98 is rearranged in 3.8% of pediatric AML forming a clinical and molecular homogenous group with a poor prognosis.

Pediatric acute myeloid leukemia (AML) is a rare disease whose prognosis is highly variable according to factors such as chromosomal abnormalities. Recurrent genomic rearrangements are detected in half of pediatric AML by karyotype. NUcleoPorin 98 (NUP98) gene is rearranged with 31 different fusion partner genes.

Clustering of Eye Fixations: A New Oculomotor Determinant of Reading Speed in Maculopathy.

Purpose: To describe and quantify a largely unnoticed oculomotor pattern that often occurs when patients with central field loss (CFL) read continuous text: Horizontal distribution of eye fixations dramatically varies across sentences and often reveals clusters. Also to statistically analyze the effect of this new factor on reading speed while controlling for the effect of saccadic amplitude (measured in letters per forward saccade, L/FS), an established oculomotor effect.

Methods: Quantification of nonuniformity of eye fixations (NUF factor) was based on statistical analysis of the curvature of fixation distributions.

FFCD-1004 Clinical Trial: Impact of Cytidine Deaminase Activity on Clinical Outcome in Gemcitabine-Monotherapy Treated Patients.

Purpose: Because cytidine deaminase (CDA) is the key enzyme in gemcitabine metabolism, numerous studies have attempted to investigate impact of CDA status (i.e. genotype or phenotype) on clinical outcome.

Eye movements and reading speed in macular disease: the shrinking perceptual span hypothesis requires and is supported by a mediation analysis.

Purpose: Reading speed of patients with central field loss (CFL) correlates with the size of saccades (measured in letters per forward saccade [L/FS]). We assessed whether this effect is mediated by the total number of fixations, by the average fixation duration, or by a mixture of both.

Methods: We measured eye movements (with a video eye tracker) of 35 AMD and 4 Stargardt patients (better eye decimal acuity from 0.

Patient education after amputation: systematic review and experts' opinions.

Objective: To perform a systematic review of the literature regarding amputee self-care, and analyze current experts' opinions.

Method: The research in Medline and Cochrane Library databases was performed using the keywords "amputee self-care", "amputee health care", "amputee education", and "amputee health management". The methodological quality of the articles was assessed using four levels of evidence and three guideline grades (A: strong; B: moderate; C: poor).