A Case of Human Intramuscular Adrenal Gland Transplantation as a Cure for Chronic Adrenal Insufficiency
A Case of Human Intramuscular Adrenal Gland Transplantation as a Cure for Chronic Adrenal Insufficiency
复制标题
DOI：
10.1111/j.1600-6143.2009.02929.x
复制
发表时间：
2010-02-01
影响因子：
8.8
通讯作者：
Goldstein, M. J.
中科院分区：
文献类型：
--
作者：
Grodstein, E.;Hardy, M. A.;Goldstein, M. J.
肌内内分泌腺移植与甲状旁腺自体移植有关，已被很好地描述；然而，肾上腺移植的特征较少。虽然在库欣病的背景下肾上腺自体移植已经被描述过，但据我们所知，肌内肾上腺异体移植作为治疗肾上腺功能不全的方法以前还没有进行过。目前对肾上腺功能不全的治疗使患者的皮质醇释放没有昼夜变化，容易受到慢性高皮质醇症的有害影响。我们在这里描述了一个5岁的肾衰竭女孩，她在暴发性脑膜炎球菌血症后出现肾上腺功能不全，导致需要同时使用应激剂量类固醇和矿化皮质激素替代。发病10个月后，她接受了母亲的肾脏和肾上腺移植手术。同种异体肾上腺移植物被切成1毫米(3)段，植入在她的腹直肌上形成的3个2厘米的口袋中。手术后三年，她的同种异体移植物功能完全正常，对促肾上腺皮质激素刺激反应良好，患者不需要任何类固醇或矿物皮质激素补充。我们相信这个病例代表了第一个成功的功能性肌内肾上腺异体移植与长期随访治疗肾上腺功能不全的描述。
Intramuscular endocrine gland transplantation has been well described as it pertains to parathyroid autotransplantation; however, transplantation of the adrenal gland is less well characterized. While adrenal autotransplantation in the setting of Cushing's disease has been described, intramuscular adrenal allotransplantation as a cure for adrenal insufficiency to our knowledge has not been previously carried out. Current treatment for adrenal insufficiency leaves patients without diurnal variation in cortisol release and susceptible to the detrimental effects of chronic hypercortisolism. We describe here the case of a 5-year-old girl with renal failure who had adrenal insufficiency following fulminant meningococcemia that led to requirements for both stress-dose steroid and mineralocorticoid replacement. Ten months after the onset of her disease, she received a simultaneous renal and adrenal gland transplant from her mother. The adrenal gland allograft was morselized into 1 mm(3) segments and implanted into three 2 cm pockets created in her rectus abdominis muscle. Three years after surgery, her allograft remains fully functional, responding well to adrenocorticotropin hormone stimulation and the patient does not require any steroid or mineralcorticoid supplementation. We believe this case represents the first description of successful functional intramuscular adrenal allograft transplantation with long-term follow up as a cure for adrenal insufficiency.