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Background: Factor (F)XI deficiency is a rare bleeding disorder with a poor correlation between bleeding tendency and FXI level. Management of pregnant women with FXI deficiency is not clearly established, especially regarding neuraxial analgesia (NA). Objectives: A retrospective multicenter observational study was conducted in French hemostasis centers on pregnant women with FXI of <60 IU/dL. Methods: Data to report were (i) FXI levels before pregnancy and at time of delivery, (ii) type of NA and delivery management modalities, and (iii) possible complications related to NA and bleeding complications. Results: Three hundred fourteen pregnancies in patients with FXI deficiency of <60 IU/dL were reported (from 20 centers); among them, 199 NA procedures have been completed (137 epidurals and 61 spinals, 1 had both). The period of childbirth was mostly from 2014 to 2020 (281/314; 89.5%). Congenital FXI deficiency was established with certainty by investigators in 32.8% patients (n = 103). Previous bleedings were described in 20.4% of the patients (64/314; 45.3% cutaneous, 31.3% gynecologic, and 15.6% postsurgical). Thirteen deliveries had an NA procedure with FXI of <30 IU/dL, 42 with FXI of 30-40 IU/dL, and 118 with FXI of 40-60 IU/dL. Median FXI levels at delivery in the epidural and spinal groups were not significantly different but were significantly lower in the group without NA by medical staff contraindications. There were no complications related to NA. A 17.5% postpartum hemorrhage or excessive postpartum bleeding incidence was reported, which is consistent with previous data. Conclusion: Our data support the use of a 30 IU/dL FXI threshold for NA, as suggested by the French proposals published in August 2023.

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Amyotrophic lateral sclerosis (ALS) is not a curable disease, but it is treatable. By definition, much of the care provided to ALS patients is palliative, even though active life-sustaining strategies are available to prolong survival. Healthcare professionals must develop communication skills that help patients cope with the inexorable progression of the disease and the inevitability of death. Symptomatic treatments as well as respiratory insufficiency and nutritional life-sustaining therapies must be regularly evaluated as the disease progresses, without losing sight of the burden placed on the patient's non-professional caregivers. The decision-making process regarding tracheostomy with invasive ventilation (TIV) is of greater complexity. Providing full information is crucial. Several long interviews are necessary to explain, discuss and allow assimilation of the information. Also, physicians should be careful not to focus exclusively on the biomedical aspects of disease, as ALS patients generally welcome the opportunity to discuss end-of-life issues with their physicians. Psychological factors, education level and cognitive status (especially the level of executive dysfunction) have a major influence on their decisions. However, as many patients do not complete advance directives with regard to TIV, advance care planning may instead be suggested in anticipation of emergency interventions. This should be discussed by healthcare professionals and the patient, and based on the wishes of the patient and caregiver(s), and communicated to all healthcare professionals. Many healthcare professionals are involved in the management of an ALS patient: they include not only those at ALS centers who provide diagnosis, follow-up and treatment initiation (particularly for respiratory and nutritional care), but also the medical and social care networks involved in disability support and home care. Specialist palliative care teams can work in partnership with ALS centers early in the course of the disease, with the center coordinating information-sharing and collaborative discussions.

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Since the Edmonton protocol, islet transplantation alone (ITA) offers the prospect of adequate glycemic control in type 1 diabetes without kidney failure. Patient motivation, evolution of diabetic complications, and hypoglycemia unawareness have to be balanced against the risks of portal puncture and long-term immunosuppressive therapy. The aim of this work was to assess the profile of 41 type 1 diabetic patients (21 men and 20 women of age 18 to 63 years) for whom islet transplantation was considered, between January 2000 and December 2002. Thirty-one of these patients lived in the area. The patients were divided into 3 groups according to their recruitment: 20, personal initiative (G1); 8, recruited from hospitalization (G2) for marked glycemic imbalance; and 13, (G3) referred by their diabetologist. Among this series of 41 patients, 14 (8 in G1, 4 in G2, and 2 in G3) did not fit the eligibility criteria, mainly because of a positive C-peptide, kidney failure, desire for pregnancy (G1, G3), liver disorders related to alcohol or iron overload related to HFE heterozygosity (G2), or good glycemic balance (G3). Sixteen did not wish to proceed after the first information step, 6 of these being more interested in a pump. Eleven, mainly recruited in G1 or G3, went through the clinical pretransplantation assessment. Among these, 2 have undergone transplantation, another 1 is enlisted. Therefore, it appears that patient motivation and information to the diabetologists are two important issues in the recruitment of patients eligible for islet transplantation. Equally important is the measurement of C-peptide, plasma creatinine, and microalbuminuria.

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