Puberty Today, Gone Tomorrow: Transient Refractory Central Precocious Puberty in a Toddler with End-Stage Kidney Disease.

IF 2.6 3区 医学 Q3 ENDOCRINOLOGY & METABOLISM
Priyanka Bakhtiani, Rachana Srivastava, Mitchell Geffner
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Abstract

Novel Insights: - There are only three reported cases of precocious puberty in boys with CKD - Hypothalamic dysfunction due to uremia, and disordered renal clearance of LH, may lead to central precocious puberty, which resolves after uremia is corrected, e.g., after kidney transplant. - Increased drug clearance via peritoneal dialysis and/or an effect of uremia may lead to a sub-optimal response to leuprolide therapy. - Further studies are needed to characterize the relationship of CKD and peritoneal dialysis on puberty.

Introduction: The onset of puberty is typically delayed in children with chronic kidney disease (CKD), with only three reported cases of precocious puberty in boys with CKD.

Case presentation: We report the case of a boy with end-stage kidney disease secondary to posterior urethral valves who, while undergoing peritoneal dialysis, presented at 17 months with central precocious puberty characterized by clinical signs of testicular and penile enlargement, pubic hair, and acne; rapid linear growth with advanced bone age; and pubertal luteinizing hormone (LH) and testosterone levels. Monthly leuprolide injections were commenced at 24 months with no pubertal or biochemical suppression thereafter, along with continued rapid bone-age advancement through 32 months. He then received a deceased-donor kidney transplant at 33 months of age, with good graft function. Within 2 months, he was noted to have prepubertal GnRH-stimulated LH and testosterone levels. Leuprolide injections were discontinued at that time with no further progression of puberty. The patient is now 48 months with minimal further bone-age advancement, and consistently prepubertal LH and testosterone levels.

Conclusion: Our case demonstrates the development of precocious puberty due to premature activation of the hypothalamic-pituitary-testicular axis, presumably secondary to uremia and/or disordered renal clearance of gonadotropins, which was refractory to standard management with a gonadotropin-releasing hormone agonist, perhaps due to excessively rapid removal by peritoneal dialysis and/or the uremic state itself. Kidney transplantation led to a correction of uremia and a return to the prepubertal state.

今天青春期,明天就消失:患有终末期肾病的幼儿出现的短暂难治性中枢性性早熟。
新见解:- 尿毒症导致的下丘脑功能障碍和肾脏对 LH 的清除障碍可能会导致中枢性性早熟,尿毒症纠正后(如肾移植后),性早熟会消失。- 通过腹膜透析增加的药物清除率和/或尿毒症的影响可能会导致对利库瑞德治疗的次优反应。- 需要进一步研究来确定慢性肾脏病和腹膜透析与青春期的关系:导言:慢性肾脏病(CKD)患儿的青春期发育通常会延迟,目前仅有三例关于CKD男孩性早熟的报道:我们报告了一例继发于后尿道瓣膜的终末期肾病男孩,他在接受腹膜透析治疗期间,于 17 个月时出现中枢性性早熟,临床表现为睾丸和阴茎增大、阴毛和痤疮;快速线性生长,骨龄提前;青春期黄体生成素(LH)和睾酮水平升高。他在 24 个月时开始每月注射亮丙瑞林,此后没有出现青春期或生化抑制现象,骨龄持续快速增长,直至 32 个月。随后,他在 33 个月大时接受了死体肾移植,移植功能良好。在两个月内,他被发现具有青春期前 GnRH 刺激的 LH 和睾酮水平。当时他停止了亮丙瑞林注射,青春期没有进一步发展。现在患者已经 48 个月了,骨龄进展极小,LH 和睾酮水平一直保持在青春期前水平:我们的病例表明,下丘脑-垂体-睾丸轴的过早激活导致了性早熟的发生,这可能是继发于尿毒症和/或肾脏对促性腺激素的清除功能紊乱。肾移植后,尿毒症得到纠正,并恢复到青春期前的状态。
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来源期刊
Hormone Research in Paediatrics
Hormone Research in Paediatrics ENDOCRINOLOGY & METABOLISM-PEDIATRICS
CiteScore
4.90
自引率
6.20%
发文量
88
审稿时长
4-8 weeks
期刊介绍: The mission of ''Hormone Research in Paediatrics'' is to improve the care of children with endocrine disorders by promoting basic and clinical knowledge. The journal facilitates the dissemination of information through original papers, mini reviews, clinical guidelines and papers on novel insights from clinical practice. Periodic editorials from outstanding paediatric endocrinologists address the main published novelties by critically reviewing the major strengths and weaknesses of the studies.
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