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Objective: To compare the ongoing pregnancy rate per initiated cycle between patients with functional hypothalamic amenorrhea (FHA) and patients with congenital hypogonadotropic hypogonadism (CHH) treated with pulsatile gonadotropin-releasing hormone (GnRH) administration., Design: Retrospective monocentric cohort study conducted at the University Hospital of Lille from 2004 to 2022., Subjects: A total of 141 patients diagnosed with central suprapituitary amenorrhea during infertility evaluation and subsequently treated with pulsatile GnRH therapy. 111 and 30 patients were diagnosed with FHA or CHH, respectively., Exposure: Pulsatile GnRH administration., Main Outcome Measure(s): Ongoing pregnancy rate per initiated cycle., Result(s): Ongoing pregnancy rates per initiated cycle were comparable between groups: 21.5% in the FHA group vs. 22% in the CHH group. Comparison of baseline characteristics showed a more pronounced follicle-stimulating hormone (FSH) deficiency in patients with CHH than in those with FHA: 2.55 (0.6-4.92) vs. 4.80 (3.90-5.70) UI/L. Within the CHH group, basal FSH level was positively associated with the occurrence of ongoing pregnancies (odds ratio, 1.57; 95% confidence interval, 1.11-2.22). In the CHH group, the duration of treatment was higher than in the FHA group: 23.59 (± 8.02) vs. 18.16 (± 7.66) days., Conclusion(s): The baseline FSH level is lower in patients with CHH than in patients with FHA. The lower the FSH, the lower the chance of pregnancy in patients with CHH. These patients also require more days of GnRH administration. However, the rate of ongoing pregnancies is comparable between the two groups., Competing Interests: Declaration of Interests H.E. has nothing to disclose. V.S. has nothing to disclose. A.B. has nothing to disclose. M.L. has nothing to disclose. C.D. has nothing to disclose. D.D. has nothing to disclose. S.C.-J. has nothing to disclose. G.R. reports lectures and presentations for Ferring., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Introduction: The TransEAsome project, funded by the Agence Nationale de la Recherche, aims to evaluate the long-term outcomes of patients with oesophageal atresia (OA) between 13 and 14 years old and establish multiomics profiles using data from the world's biggest OA registry., Methods and Analysis: TransEAsome is a national multicentre population-based cohort study recruiting participants from all qualified French centres for OA surgery at birth. The primary objective is to assess the prevalence of gastro-oesophageal reflux disease in adolescence among patients with OA, with several secondary objectives including the identification of risk factors and multiomic profiles from oesophageal biopsies and blood samples collected between 13 and 14 years old, compared with a control group. This comprehensive characterisation of phenotype and omic profiles aims to enhance the understanding of disease evolution in patients with OA and inform tailored care management strategies., Ethics and Dissemination: The study, coconstructed with input from patients, parents and research-expert adolescents, has obtained approval from the ethics research committee: Comité de protection des personnes Est II. Findings will be disseminated to various target audiences, including the scientific community, research participants, the patient community, the general public, regulatory authorities and policymakers. Data will be made available in a Findable, Accessible, Interoperable, Reusable format on the France Cohortes platform on study completion., Trial Registration Number: NCT05995171:Clinical trial., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2025. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group.)

Background: A subgroup of CHDs can only be treated palliatively through a Fontan circulation. In case of a failing Fontan situation, serum proteins are lost unspecifically and can also lead to a loss of vaccine antibodies. In a failing Fontan situation, heart transplantation may be the only feasible option., Patient: We describe a 17-year-old patient born with a hypoplastic left heart complex, who underwent Fontan completion at the age of 4 years and developed a failing Fontan physiology. Therefore, a Fontan takedown with creation of a reverse 1½-circulation was performed. Multiple exacerbations of protein losing enteropathy occurred with a hypoproteinaemia, hypalbuminaemia, and hypogammaglobulinaemia. The patient was hospitalised several times and treated with intravenous application of immunoglobulins and albumin for symptom control. Before one of this substitutions, the immunoglobulin G against measles, mumps, and rubella was determined: the patient's serum demonstrated a positive measles and rubella, but mumps was negative. After administration of the iv-therapy, the lacking antibodies were replenished, and there was a positive test for mumps., Method: Serum samples were analysed by neutralisation test and enzyme-linked immunosorbent assay (ELISA)., Conclusion: Although the patient had been vaccinated according to national guidelines, we saw an intermittent immune deficiency for mumps, but not for rubella and measles. For patient with a failing Fontan circulation, we recommend to test to vaccine antibodies for mumps, measles, and rubella with an ELISA an if its negative with a neutralisation test, especially in view of a possible heart transplantation to find a possible immune deficiency.