Testosterone Replacement Therapy in Adolescents With Sickle Cell Disease Reverses Hypogonadism Without Promoting Priapism: A Case Report

Abstract

Delayed puberty secondary to hypogonadism is commonly seen in sickle cell disease (SCD), affecting normal growth and development. The condition is rarely treated in SCD for fear of inducing priapism episodes. We present a case report of an Afro-Jamaican adolescent male at 16 years of age who presented with symptoms of delayed puberty as well as frequent stuttering priapism episodes. Endocrinological assessment revealed low serum total testosterone levels. Treatment was commenced monthly with testosterone enanthate which resulted in improved symptoms of delayed puberty, improvement in anthropometric parameters while apparently ameliorating priapism episodes.

Nitric Oxide Resistance in Priapism Associated with Sickle Cell Disease: Mechanisms, Therapeutic Challenges, and Future Directions

“…Currently, it is believed that the administration of testosterone at physiologic levels does not induce priapism (Morrison et al, 2013). On the contrary, this treatment seems to reduce priapism due to normalizing molecular mechanisms that promote normal erectile responses (Morrison et al, 2015).…”

Medical treatment of recurrent ischaemic priapism: a review of current molecular therapeutics and a new clinical management paradigm

“…Morrison et al [46] evaluated testosterone replacement in seven hypogonadal men with SCD, finding no significant increase in priapism events, notably, with enhanced normal sexual function. This group further reported on an adolescent patient with SCDassociated hypogonadism and delayed puberty with stuttering priapism who, after testosterone replacement, recovered pubertal growth and experienced less severe and frequent priapism episodes [47]. The combination of preclinical studies and early clinical research suggests that normalization of the androgen molecular pathway may be a preferred approach to treat RIP.…”

“…For first-line prevention, treatment of underlying conditions and restoration of the normal HPG axis should be the primary interventions. Based on previous evaluation, patients who have evidence of hypogonadism should be treated with testosterone replacement therapy to restore a eugonadal state [43,47]. It is important to closely monitor testosterone levels during testosterone replacement therapy in patients with RIP to ensure there is no overcorrection, which may exacerbate this condition.…”

Perfil Hormonal E Hipogonadismo De Pessoas Com Doença Falciforme Acompanhadas Em Um Centro Especializado Na Cidade De Feira De Santana-Ba

“…O hipogonadismo, síndrome clínica associada à produção diminuída de testosterona e infertilidade, pode ser primário ou secundário a depender da etiologia, se central ou periférica, sendo caracterizado de acordo com os achados laboratoriais das dosagens hormonais (GRINSPON;FREIRE;REY, 2019;MORRISON et al, 2015). O "hipogonadismo compensado" é um conceito novo, ainda não relatado nas pessoas com DF e sugere hipoandrogenismo periférico, provocado por um provável defeito na ação da testosterona sobre seu receptor (MARTINS et al, 2019).…”