Vallant Natalie, Sinha Manish D, Cheung Moira, Ware Nick, Jones Helen, Buck Jackie, Boffa Catherine, Irving Melita, Carroll Paul V, Hubbard Johnathan, Gökmen Refik, Rees Lesley, Gogalniceanu Petrut, Kessaris Nicos
Department of Nephrology and Transplantation, Guy's Hospital, London, United Kingdom.
Department of Paediatric Nephrology, Evelina London Children's Hospital, London, United Kingdom.
Transplant Direct. 2022 Mar 16;8(4):e1284. doi: 10.1097/TXD.0000000000001284. eCollection 2022 Apr.
Genetically determined hypoparathyroidism can lead to life-threatening episodes of hypocalcemia and, more rarely, to end-stage kidney disease at a young age. Parathyroid allotransplantation is the only curative treatment, and in patients already receiving immunosuppression for kidney transplantation, there may be little additional risk involved. We report the first such case in a child.
An 11-y-old girl, known to have hypoparathyroidism secondary to an activating pathogenic variant in the calcium-sensing receptor, developed end-stage kidney disease and was started on intermittent hemodialysis. Since the age of 2.5 y, she had been receiving treatment with exogenous synthetic parathyroid hormone (PTH). In June 2019, at the age of 11.8 y, she received a living-donor kidney and simultaneous parathyroid gland transplant from her father. The kidney was implanted into the right iliac fossa, followed by implantation of the parathyroid gland into the exposed rectus muscle.
The kidney graft showed immediate function while the intrinsic serum PTH level remained low at 3 ng/L. Exogenous PTH infusion was reduced on day 6 posttransplantation to stimulate PTH production by the new gland, which resulted in improving intrinsic PTH concentrations of 28 ng/L by day 9. Twelve months after transplantation, PTH levels remain in normal range and the kidney graft function is stable with a serum creatinine of 110 μmol/L.
Simultaneous living donation and transplantation of a kidney and a parathyroid gland into a child is safe and feasible and has the potential to cure primary hypoparathyroidism as well as kidney failure.
基因决定的甲状旁腺功能减退可导致危及生命的低钙血症发作,更罕见的是在年轻时发展为终末期肾病。甲状旁腺同种异体移植是唯一的治愈性治疗方法,对于已经接受肾移植免疫抑制治疗的患者,额外风险可能很小。我们报告了首例儿童甲状旁腺移植病例。
一名11岁女孩,因钙敏感受体激活致病变异继发甲状旁腺功能减退,发展为终末期肾病并开始间歇性血液透析。自2.5岁起,她一直接受外源性合成甲状旁腺激素(PTH)治疗。2019年6月,11.8岁时,她接受了来自父亲的活体供肾及同时进行的甲状旁腺移植。肾脏植入右髂窝,随后将甲状旁腺植入暴露的腹直肌。
肾移植立即发挥功能,而内源性血清PTH水平仍低,为3 ng/L。移植后第6天减少外源性PTH输注,以刺激新腺体产生PTH,到第9天内源性PTH浓度提高到28 ng/L。移植12个月后,PTH水平保持在正常范围内,肾移植功能稳定,血清肌酐为110 μmol/L。
同时进行活体供肾及甲状旁腺移植到儿童体内是安全可行的,有治愈原发性甲状旁腺功能减退及肾衰竭的潜力。