Zatsepina E A, Samoilov V S, Volynkina A P, Stepanenko A V, Novichikhina E E
Clinic City of Health (Family Medicine Center "Olympus of Health").
Clinic City of Health (Family Medicine Center "Olympus of Health"); State Scientific Center of the Russian Federation - Federal Medical Biophysical Center named after A.I. Burnazyan Federal Medical and Biological Agency of Russia.
Probl Endokrinol (Mosk). 2023 Apr 30;69(3):83-89. doi: 10.14341/probl13206.
The article presents a clinical observation of a patient with congenital adrenal dysfunction (CHD), a salt-losing form of 21-hydroxylase enzyme deficiency (homozygous mutation I 172N), and also with morbid obesity, due to long-term use of high doses of glucocorticosteroids, who underwent bariatric surgery - laparoscopic sleeve resection of the stomach. A feature of the presented case is the elimination of one of the causes of decompensation of the disease, namely, overweight, as well as insulin resistance, which requires the intake of large doses of glucocorticoids, which in turn leads to a worsening of the course of obesity, thereby causing a vicious circle. 7 months after surgical treatment, the goal was achieved - a reduction in the dose of Prednisolone by 25%, with a decrease in body weight by 72.1% of overweight.The presented case clearly demonstrates the possibility of performing bariatric surgery for the treatment of morbid obesity in patients with CAH with the participation and control of a specialized multidisciplinary team. If there are indications for bariatric intervention, VDKN should not be an absolute contraindication to such operations, and the ratio of the safety profile and the efficacy profile testifies in favor of the need for their implementation.
本文介绍了一名患有先天性肾上腺功能不全（CHD）的患者的临床观察情况，该患者为21-羟化酶缺乏的失盐型（纯合子突变I 172N），同时因长期使用高剂量糖皮质激素而患有病态肥胖症，该患者接受了减肥手术——腹腔镜胃袖状切除术。该病例的一个特点是消除了疾病失代偿的原因之一，即超重，以及胰岛素抵抗，胰岛素抵抗需要摄入大剂量糖皮质激素，而这反过来又会导致肥胖病程恶化，从而形成恶性循环。手术治疗7个月后，目标得以实现——泼尼松龙剂量降低25%（占超重体重的72.1%）。该病例清楚地表明，在专业多学科团队的参与和控制下，对患有先天性肾上腺皮质增生症（CAH）的患者进行减肥手术以治疗病态肥胖是可行的。如果有减肥干预的指征，先天性肾上腺皮质增生症不应成为此类手术的绝对禁忌症，安全性和有效性的比例证明了实施此类手术的必要性。
