ESPE2024 Poster Category 1 Pituitary, Neuroendocrinology and Puberty 2 (9 abstracts)
International Practice in Management of Puberty for Patients with Hypogonadotropic Hypogonadism
Sebastian Castro 1,2 , Julia Rohayem 3 , Rachel Varughese 1,4 , Marco Bonomi 5,6 , Gary Butler 7,8 , Toby Candler 9 , Tim Cole 7 , Mehul Dattani 4,7,8 , Sabine Heger 10 , Channa Jayasena 11 , Nils Krone 12 , Anna Nordenstrom 13 , Senthil Senniappan 14 , Claire Wood 15 , Jillian Bryce 16 , Faisal Ahmed 16 & Sasha Howard 1,17
1Centre for Endocrinology, QMUL, London, United Kingdom. 2CEDIE, FEI - CONICET, Buenas Aires, Argentina. 3Department of Pediatric Endocrinology and Diabetology, St Gallen, Switzerland. 4Great Ormond Street Hospital, London, United Kingdom. 5Department of Medical Biotechnology and Translational Medicine, University of Milan, Milan, Italy. 6Department of Endocrine and Metabolic Diseases, IRCCS Istituto Auxologico Italiano, Milan, Italy. 7Institute for Child Health, ICH/UCL, London, United Kingdom. 8UCLH NHS Foundation Trust, London, United Kingdom. 9University Hospital Bristol NHS Foundation Trust, Bristol, United Kingdom. 10Children’s Hospital Hannover, Hannover, Germany. 11Imperial College London, London, United Kingdom. 12University of Sheffield, Sheffield, United Kingdom. 13Karolinska Institutet, Astrid Lindgren Children’s Hospital, Stockolm, Sweden. 14Alder Hey Children’s NHS Foundation Trust, Liverpool, United Kingdom. 15Newcastle Hospitals NHS Foundation Trust, Newcastle-upon-Tyne, United Kingdom. 16Developmental Endocrinology Research Group, Royal Hospital for Children, University of Glasgow, Glasgow, United Kingdom. 17Barts Health NHS Trust, London, United Kingdom
Background: Hypogonadotropic hypogonadism, a condition of central hypogonadism secondary to deficiency of gonadotropin releasing hormone and/or gonadotropins, is a rare disease affecting approximately 1 in 5000-15,000. A key step in improving understanding of best practice in this condition is the collection of geographically widespread data. Standardised and accessible international data collection can facilitate this, with implementation of established protocols and monitoring of outcomes. Clinical priorities for patients and carers include improving accuracy of diagnosis in childhood, tailored hormonal therapies at infancy and puberty and optimising potential for future fertility. Our group has led establishment of the first UK protocol for gonadotropin replacement of puberty in males with hypogonadotropic hypogonadism. Following this, we are developing a new electronic registry of hypogonadotropic hypogonadism (I-HH registry, 4th module in the sex development and maturation [SDM] registries series), due to go-live in summer 2024.
Methods and Anticipated Outputs: The new I-HH module has been developed with input from international stakeholders and clinical experts, to be incorporated within the existing SDM registries. Iterative testing of the developing module is ongoing and will be piloted before going live. Prior to active collection of data within the I-HH module, a project to review data from patients registered with hypogonadotropic hypogonadism within the existing I-DSD Registries has been approved. This pilot study, with data on 60 patients from 7 countries, can optimise the new registry and facilitate widespread implementation. Patients are evenly split between the diagnoses of Kallmann syndrome and normosmic hypogonadotropic hypogonadism, with 67% male and 33% female sex. Data are available on diagnostic testing, family history and follow up. Future analyses within I-HH will examine pubertal induction practices, including therapeutic regimens, and monitoring practices and response to therapy. Peak height velocity will be used as a surrogate marker of puberty, utilising SuperImposition by Translation and Rotation (SITAR) growth curve analysis, to facilitate easy collection of retrospective data. This database is also key to recording long-term outcomes for young patients, particularly pertaining to fertility in response to therapeutic interventions in childhood.
Summary: I-HH will be able to connect centres worldwide to assess clinical management and health status of patients with this condition, facilitating prospective longitudinal data collection. The analysis of these initial pilot data held in I-DSD will allow benchmarking of current practice, define the impact of differing management protocols, and identify specific unmet needs to support future research, including that using the I-HH registry.
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