[End of life in pediatrics].

When medicine proves to be powerless to cure a child suffering from cancer, there remains the path of accompaniment by what we call palliative care. This is very different from the treatments that have been administered up to now - since they are not intended to cure - and its main objective is to create a comfortable living environment, a warm and reassuring climate around the young patient and his family.

Extent of Low-density Lipoprotein Cholesterol Reduction and All-cause and Cardiovascular Mortality Benefit: A Systematic Review and Meta-analysis.

Lipid-modifying agents steadily lower low-density lipoprotein cholesterol (LDL-C) levels with the aim of reducing mortality. A systematic review and meta-analysis were conducted to determine whether all-cause or cardiovascular (CV) mortality effect size for lipid-lowering therapy varied according to the magnitude of LDL-C reduction. Electronic databases were searched, including PubMed and ClinicalTrials.

[Place of internal medicine specialists in inpatient unprogrammed care of adult patients in France: A survey of in training and senior internal medicine specialists].

Introduction: French internal medicine specialists are trained in clinical immunology and rare diseases as well as frequent ones. The latest activity is rarely highlighted by practitioners themselves and their representative authorities. Frequent diseases care in French hospitals are also the tasks of physicians without internal medicine specialty, mostly trained in general medicine, who practice in departments carrying various names.

Variants and C1-INH Biological Function: A Close Relationship With C1-INH-HAE.

Hereditary angioedema with C1 Inhibitor deficiency (C1-INH-HAE) is caused by a constellation of variants of the gene ( = 809; 1,494 pedigrees), accounting for 86.8% of HAE families, showing a pronounced mutagenic liability of and pertaining to 5.6% variants.

Heterozygous variants in SIX3 and POU1F1 cause pituitary hormone deficiency in mouse and man.

Congenital hypopituitarism is a genetically heterogeneous condition that is part of a spectrum disorder that can include holoprosencephaly. Heterozygous mutations in SIX3 cause variable holoprosencephaly in humans and mice. We identified two children with neonatal hypopituitarism and thin pituitary stalk who were doubly heterozygous for rare, likely deleterious variants in the transcription factors SIX3 and POU1F1.

Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study.

Background: Riociguat and balloon pulmonary angioplasty (BPA) are treatment options for inoperable chronic thromboembolic pulmonary hypertension (CTEPH). However, randomised controlled trials comparing these treatments are lacking. We aimed to evaluate the efficacy and safety of BPA versus riociguat in patients with inoperable CTEPH.

Paclitaxel-bevacizumab combination in advanced non-squamous non-small-cell lung cancer (NSCLC): AVATAX, a retrospective multicentric study.

Introduction: Compared with docetaxel, the phase-III trial, ULTIMATE, showed a significant improvement of progression-free survival (PFS) with paclitaxel-bevacizumab combination (PB) as second- or third-line treatment in advanced non-small cell lung cancer (NSCLC). With the increase of immunotherapy treatment in first-line settings, the optimal treatment after first-line failure must be redefined.

Methods: This multicentric retrospective study identified all advanced NSCLC patients treated with PB as second-line therapy and beyond.

Non-invasive fetal monitoring: Fetal Heart Rate multimodal estimation from abdominal electrocardiography and phonocardiography.

Background: Fetal cardiac well-being is essential during labor as the delivery is at risk for fetal distress. Continuous monitoring by cardiotocography (CTG) is daily used to record the fetal heart rate (FHR) but this technique has important drawbacks in clinical use.

Objectives: We propose to monitor FHR with a non-invasive technique, using multimodal recordings of the fetus cardiac activity, associating electrocardiographic (ECG) and phonocardiographic (PCG) sensors.

AML with inv(16)/t(16;16) and high-risk cytogenetic abnormalities: atypical features and unfavorable outcome.

Objectives: Acute myeloid leukemia (AML) with inv(16)/t(16;16) is among the most frequent AML subtypes. It is recognized by the detection of the fusion which confers a favorable prognosis, irrespective of the presence of secondary cytogenetic abnormalities. However, the effect of additional genetic anomalies on the behavior of inv(16) AML is debatable.

[Child-family-caregiver collaboration, a factor in improving care].

Giving the parents of a child in need of care their rightful place is crucial. This creates a climate of trust that will guarantee the young patient a foundation of security and will promote optimal quality of care.

[The place of the family in pediatric oncology].

Pediatric oncology departments receive children with serious pathologies. Their family life is disrupted, hospitalizations are numerous and the prognosis is not always reassuring. The presence of the parents with the young patient is however essential to his recovery.

[From the hospital door to the heart of care].

From the 1970s to today, the care of children in hospitals has progressed enormously. The rigidity that was once the norm has gradually become more open and flexible, thereby encouraging parents to be present with their child.

[Helping parents to become parents].

The professional practices have largely evolved these last years within the services taking care of children. If this has been done in a heterogeneous way according to the places and the services, it is necessary to note that today parents are everywhere recognized as essential partners in the care.

[Caregiver-child-parent, an essential partnership in pediatrics].

When they turn to pediatrics, health care professionals accept to integrate into their practice the presence of parents as an essential element, preponderant in the healing of the child. If the resulting organization is to be taken into account in daily practice, it also reveals itself as a rich source of ethical reflection.

[Supporting the role of parents in child care].

Child care has a special dimension because it is aimed at a being in the making, whose parents are the foundation. Without them, it makes no sense to consider any kind of support. Several mechanisms are implemented when the caregiver is confronted with the hospitalization of a baby, a child or an adolescent.

Pituitary MRI Features in Acromegaly Resulting From Ectopic GHRH Secretion From a Neuroendocrine Tumor: Analysis of 30 Cases.

Context: Ectopic acromegaly is a consequence of rare neuroendocrine tumors (NETs) that secrete GHRH. This abnormal GHRH secretion drives GH and IGF-1 excess, with a clinical presentation similar to classical pituitary acromegaly. Identifying the underlying cause for the GH hypersecretion in the setting of ectopic GHRH excess is, however, essential for proper management both of acromegaly and the NET.

Hypersensitivity transfusion reactions to fresh frozen plasma: a retrospective analysis of the French hemovigilance network.

Few data are currently available on hypersensitivity transfusion reactions (HTRs) after exposure to fresh frozen plasma (FFP). Between 2000 and 2018, three different FFP production strategies have been used in France, leading to the concomitant use of different types of FFP. The objective of this study was to describe the rate of FFP-related HTRs and to assess the relative risk of each type of FFP.

Cumulative Incidence and Risk Factors for Severe Coronavirus Disease 2019 in French People With Cystic Fibrosis.

Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections are closely monitored in people with cystic fibrosis (pwCF), especially severe cases. Previous studies used hospitalization rates as proxy for severity.

Methods: We evaluated data from coronavirus disease 2019 (COVID-19) cases diagnosed in French pwCF over the first pandemic year.

A Glycemia Risk Index (GRI) of Hypoglycemia and Hyperglycemia for Continuous Glucose Monitoring Validated by Clinician Ratings.

Background: A composite metric for the quality of glycemia from continuous glucose monitor (CGM) tracings could be useful for assisting with basic clinical interpretation of CGM data.

Methods: We assembled a data set of 14-day CGM tracings from 225 insulin-treated adults with diabetes. Using a balanced incomplete block design, 330 clinicians who were highly experienced with CGM analysis and interpretation ranked the CGM tracings from best to worst quality of glycemia.

Ipsilateral hand impairment predicts long-term outcome in patients with subacute stroke.

Background: Ipsilateral hand (ILH) impairment is documented following motor stroke, but its impact on long-term outcome remains unknown. We assessed ILH impairment in subacute stroke and tested whether ILH impairment predicted long-term outcome.

Methods: We performed a longitudinal study in 209 consecutive patients with unilateral stroke and sensorimotor deficit at admission.

International consensus on lung function testing during the COVID-19 pandemic and beyond.

Coronavirus disease 2019 (COVID-19) has negatively affected the delivery of respiratory diagnostic services across the world due to the potential risk of disease transmission during lung function testing. Community prevalence, reoccurrence of COVID-19 surges and the emergence of different variants of SARS-CoV-2 have impeded attempts to restore services. Finding consensus on how to deliver safe lung function services for both patients attending and for staff performing the tests are of paramount importance.