Objective: To determine whether we can safely and successfully transplant an ovary tissue allograft from a nonidentical donor to her Turner syndrome sister.
Design: Transplantation of cryopreserved ovary tissue, as well as fresh transplantation of ovarian tissue between identical twins, is now well established with numerous reported successful cases. However, there have not yet been any ovary transplants between nonidentical women requiring immunosuppression (ovary allotransplant). This could be a much more common indication for ovary tissue transplantation if safe and reliable immunosuppression were available.
Patient(s): A 20-year-old amenorrheic woman with nonmosaic 45-XO Turner syndrome requested ovary tissue transplantation from her fertile 22-year-old 46-XX sister. They were an human leukocyte antigens match but were ABO incompatible, a well-known contra-indication to solid tissue or organ transplantation. The Turner syndrome sister strongly preferred to be able to become pregnant naturally without donor egg in vitro fertilization and to avoid hormone replacement therapy. In her religious group, that would also be important for finding a marital match. Despite the poor prognosis associated with ABO incompatibility, an ovary from her 22-year-old nonidentical fertile sister was transplanted to her employing the immunosuppression protocol now used for kidney transplant patients in our centers at Washington University and Johns Hopkins.
Intervention(s): Not applicable.
Main Outcome Measure(s): Post operatively at 5 months she developed normal monthly menstrual ovarian function, and she became spontaneously pregnant with a normal infant girl. The relation between her postoperation follicle stimulating hormone and antimüllerian hormone levels continue to support the theory that tissue pressure controls primordial follicle recruitment. The fact that ABO incompatibility did not prevent success suggests that diffusion and not revascularization may be all that is required for successful long-term ovarian cortex transplant survival with spontaneous pregnancy.
Result(s): Ovary allotransplantation with safe immunosuppression allows natural conception, and also normal hormone function obviates the need for hormone replacement therapy. Orthotopic placement of the graft and surgical technique is critical for natural conception and a higher pregnancy rate.
Conclusion(s): Allotransplantation requiring safe immunosuppression, if successful, maybe a much more commonly used indication for ovary transplantation in the future than frozen ovary grafts or grafts between identical twins.
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http://dx.doi.org/10.1016/j.fertnstert.2024.08.324 | DOI Listing |
Kardiol Pol
January 2025
2nd Department of Radiology, Medical University of Lublin, Lublin, Poland.
ERJ Open Res
January 2025
Centre for Human and Applied Physiological Sciences, School of Basic and Medical Biosciences, Faculty of Life Sciences and Medicine, King's College London, London, UK.
Introduction: Refractory chronic cough (RCC), persisting despite addressing contributory diagnoses, is likely underpinned by neurally mediated cough hypersensitivity. disorders are genetic neurodegenerative conditions caused by biallelic repeat expansion sequences, commonly presenting with cough, followed by neurological features including cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS). The prevalence and identifying clinical characteristics of repeat-expansion disorders in patients with RCC are unknown.
View Article and Find Full Text PDFAnn Pediatr Endocrinol Metab
January 2025
Department of Medicine, Surgery and Health Science, University of Trieste, Trieste, Italy.
Long-acting growth hormones (LAGHs) represent a significant advancement in the treatment of pediatric growth hormone deficiency (GHD), offering an alternative to daily recombinant human growth hormone (rhGH) therapy. Traditional rhGH treatments, while effective, require daily injections, often leading to poor adherence due to the frequency of dosing, injection pain, and difficulties with storage and travel. In contrast, LAGHs, such as somatrogon, somapacitan, and lonapegsomatropin, are designed for once-weekly administration, improving patient compliance and quality of life.
View Article and Find Full Text PDFClin Endocrinol (Oxf)
January 2025
Oxford Heart Centre, Oxford University Hospitals NHS Foundation Trust, Oxford, UK.
Objective: The risk of aortic dissection is increased in Turner Syndrome (TS). Aortic dilation is thought to contribute to this risk and may be managed with elective aortic surgery. New TS guidance has lowered the aortic size thresholds for consideration of aortic surgery.
View Article and Find Full Text PDFAm J Med Genet C Semin Med Genet
January 2025
GeneDx LLC, Gaithersburg, Maryland, USA.
Given the overwhelming volume of medical information, medical guidelines play a key role in informing clinicians and payors and guidelines directly affect how patients, and their families will be treated. In this review, we describe the production cycle of international guidelines for Turner syndrome (TS) and promote the timely and proactive dissemination of these guidelines. We encourage deliberate adoption of an updated standard of care by providers, payors, patients and their families, and professional organizations.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!