Centre Hospitalier Universitaire de Rouen

For children with Crohn's disease, there is a marked demand for long-term nutritional treatment strategies to avoid the side-effects related to drug treatment. We aimed to investigate whether paediatric patients with Crohn's disease responding to nutritional induction therapy can be maintained in remission on dietary therapy without the use of medication or surgery.

In this open-label, endpoint-blinded, randomised controlled trial (CD-HOPE) done in 21 hospitals of the GETAID pédiatrique network in France, eligible patients were aged 6 years to younger than 18 years with Crohn's disease (newly diagnosed or relapsing after drug treatment) who had reached clinical remission (weighted Paediatric Crohn's Disease Activity Index [wPCDAI] ≤12·5) after induction therapy with 6-12 weeks of exclusive enteral nutrition (EEN; 100% of calories). Stratified by age (≤10 years or >10 years) and relapse status, patients were randomly assigned (1:1) using block randomisation to receive either cyclic EEN (C-EEN; 100% of daily caloric requirements) for 2 weeks every 8 weeks for at least six cycles or daily partial enteral nutrition (PEN; 25% of daily caloric requirements) for 52 weeks. Oral MODULEN IBD was used for both. Except for the 2-week intervals of EEN in the C-EEN group, food access was not restricted. The primary endpoint was relapse rate at 12 months. A relapse was defined as: (1) a wPCDAI score greater than 12·5 at two successive visits, or (2) a wPCDAI greater than 12·5 once and the need for Crohn's disease-related surgery, medication, or study exit, or (3) a wPCDAI score continuously greater than 12·5 since the last study visit, or (4) incomplete wPCDAI and moderate or severe Physician Global Assessment at any timepoint. Primary and safety analyses were performed on an intention-to-treat basis. This trial was registered on ClinicalTrials.gov (NCT02201693) and is complete.

Between Dec 12, 2014, and Sept 4, 2018 (last patient visit Oct 2, 2019), 112 patients were screened and 100 patients (70 boys and 30 girls) were randomly assigned to C-EEN (n=49) or PEN (n=51). Median age of participants was 12 years (IQR 11-13) in the C-EEN group and 13 years (11-14) in the PEN group. At 12 months, 24 (49%) of 49 patients had relapsed on C-EEN compared with 39 (76%) of 51 patients on PEN (adjusted odds ratio 0·29 [95% CI 0·13-0·70], p=0·0051). 19 adverse events occurred in 17 patients: four serious adverse events in four patients on PEN, not related to treatment, and 15 non-serious adverse events in 13 patients (nine in seven patients on C-EEN and six in six patients on PEN). The serious adverse events were all related to Crohn's disease relapse requiring hospitalisation for rescue therapy. Six adverse events were possibly related to study treatment: one patient in the C-EEN group reported transient vomiting and diarrhoea at the start of the first EEN cycle, one patient in the C-EEN group developed anorexia and, in the PEN group, four patients reported either weight loss, nausea, Crohn's disease relapse, or skin infection. No malignancy or death was reported in this study.

C-EEN was superior to PEN in maintaining clinical remission over 1 year in paediatric patients with Crohn's disease responding to EEN induction therapy. These findings suggest a new way to use nutritional therapy for maintaining drug-free long-term remission in patients with Crohn's disease who are responding to EEN induction therapy.

Assistance Publique-Hôpitaux de Paris and Nestlé Health Science.

What are the outcomes for prepubertal and pubertal girls with Turner syndrome (TS) in terms of fertility counselling and preservation?

Fertility counselling is crucial for prepubertal and pubertal girls with TS, as it can facilitate their pursuit of fertility preservation (FP), primarily through oocyte cryopreservation (OC) and is particularly relevant for patients with blood karyotype abnormality with good prognosis for future fertility.

TS is a common genetic condition affecting ∼1 in 2500 live-born girls. One consequence of TS is premature ovarian insufficiency, significantly impacting the quality of life in adulthood. Therefore, appropriate counselling and effective FP or solutions are essential. When OC is proposed, the role of anti-Müllerian hormone (AMH) and FSH in predicting the outcomes of OC has been examined in the literature with controversial data.

This retrospective observational study was conducted at the Reproductive Biology Laboratory-CECOS of Rouen University Hospital, evaluating the follow-up of 40 prepubertal and pubertal girls with TS referred for fertility counselling. Clinical and biological data were collected from medical records between January 2008 and December 2022.

Prepubertal and pubertal patients with TS attended a fertility counselling consultation, accompanied by their parents or legal guardian if they were under the age of 18 years. The impact of TS on future fertility and potential FP procedures, including OC, were explained. An assessment of ovarian reserve was conducted. Based on the results and depending on the patient’s pubertal status, FP could be initiated immediately in TS patients with spontaneous menarche or follow-up could continue until spontaneous puberty and menarche occur.

In terms of FP, 25% (10/40) of the patients underwent OC. On average, 4.9 ± 3.8 oocytes per controlled ovarian hyperstimulation cycle were cryopreserved. No relationship was found between basal FSH or AMH serum level, karyotype abnormalities, and the number of mature oocytes retrieved. Conversely, a positive correlation was observed between the peak estradiol level at the time of triggering and the number of mature oocytes retrieved. In the multiple linear regression analysis with cross-validation, the peak estradiol level at triggering remained the only variable independently associated with mature oocyte yield. Most patients were aware of the impact of TS on future fertility but were uninformed about available parenthood alternatives.

The number of TS patients included in our study is a limitation, as well as the monocentric and retrospective nature of the study. Therefore, our data should be interpreted with caution.

Fertility counselling and FP are essential for prepubertal and pubertal TS patients. Regular and systematic follow-up of ovarian reserve and function should be implemented in their medical care. OC is a feasible option for some TS patients and should be considered after menarche but delayed until further pubertal maturation to allow robust patient engagement in the decision-making process. Further studies are needed to evaluate factors influencing the number of mature oocytes retrieved and the optimal number of oocytes necessary to ensure a good chance of pregnancy.

This work had institutional financial support from Rouen University Hospital. The authors have no conflicts of interest to declare.

N/A.

Current evidence on catheter ablation for patients with congenital heart disease (CHD) is derived from small, retrospective studies. This study aims to provide insights from a nationwide contemporary registry.

This prospective study included all CHD patients referred for catheter ablation from 2020 to July 2024 across 28 French centres. The primary outcome was the rate of per-procedural acute success. Secondary outcomes included complications as well as freedom from arrhythmia recurrence.

A total of 1135 consecutive catheter ablation procedures were performed in 998 patients (mean age 46.1 ± 16 years, 55.5% male). The main primary clinical arrhythmias targeted were atrial flutter/tachycardia in 677 (59.6%), atrial fibrillation in 195 (17.2%), ventricular arrhythmia in 188 (16.6%), and atrioventricular reentrant tachycardia in 38 (3.3%), with significant variations in patterns observed based on the underlying substrate. Clinical arrhythmia was successfully ablated in 1071 patients (94.4%). The mean number of arrhythmias targeted per procedure was 1.5 ± 0.7, with overall acute success rates exceeding 90% for all arrhythmias except for ventricular arrhythmias (86.7%). Acute complication occurred in 43 procedures (3.8%), including 1 (0.1%) death. The overall 1- and 2-year recurrence-free rates were 77.3% (95% confidence interval 74.2%–80.4%) and 68.4% (95% confidence interval 64.7%–72.3%), respectively. Significant variations in recurrence rates were noted based on the type of arrhythmia and the underlying CHD.

Catheter ablation in patients with CHD demonstrates highly favourable acute outcomes and a low complication rate. Recurrence rates during follow-up vary depending on the targeted arrhythmia and the underlying CHD. These findings should be considered in the benefit-risk assessment.