Clinical characteristics and management of adipsic arginine vasopressin deficiency in children and adolescents with sellar germ cell tumors.

Autor: Tong T; Department of Endocrinology, Beijing Tiantan Hospital, Capital Medical University, No. 119, South 4 Ring Road West, Beijing, 100071, China., Xu J; Department of Endocrinology, Beijing Tiantan Hospital, Capital Medical University, No. 119, South 4 Ring Road West, Beijing, 100071, China., Chen H; Department of Geriatrics, Beijing Jishuitan Hospital, Capital Medical University, Beijing, 100035, China., Guo Y; Department of Endocrinology, Beijing Tiantan Hospital, Capital Medical University, No. 119, South 4 Ring Road West, Beijing, 100071, China., Mo C; Department of Endocrinology, Beijing Tiantan Hospital, Capital Medical University, No. 119, South 4 Ring Road West, Beijing, 100071, China., Wang Y; Department of Endocrinology, Beijing Tiantan Hospital, Capital Medical University, No. 119, South 4 Ring Road West, Beijing, 100071, China., Zhong L; Department of Endocrinology, Beijing Tiantan Hospital, Capital Medical University, No. 119, South 4 Ring Road West, Beijing, 100071, China. zhongliyong@bjtth.org.
Jazyk: angličtina
Zdroj: European journal of pediatrics [Eur J Pediatr] 2024 Dec; Vol. 183 (12), pp. 5279-5289. Date of Electronic Publication: 2024 Oct 09.
DOI: 10.1007/s00431-024-05803-0
Abstrakt: Adipsic arginine vasopressin deficiency(aAVP-D) is a rare, high-risk syndrome, particularly difficult to recognize and manage in children and adolescents. This investigation examined the clinical features and management of aAVP-D in children and adolescents with sellar germ cell tumors (GCTs). A retrospective survey was performed on 260 patients with sellar GCTs, categorized into aAVP-D and non-aAVP-D groups based on thirst presence. General characteristics, hypothalamic syndrome, pituitary function, metabolic indicators, and complications were compared. Biochemical indicator changes in the aAVP-D group were analyzed after systematic management, and receiver operating characteristic (ROC) curve analysis established the optimum serum sodium cut-off for predicting the aAVP-D. 25 patients (9.6%) developed aAVP-D. The aAVP-D group had larger tumors with hypothalamic involvement and more surgical resections. They also demonstrated more hypothalamic syndrome, central adrenal insufficiency, central hypogonadism, and insulin-like growth factor-1 levels below norms. Furthermore, aAVP-D patients exhibited significantly higher rates of hypernatremia (100% vs 20.9%, p < 0.001), hyperuricemia (60.0% vs 23.4%, p < 0.001), renal impairment (32.0% vs 1.7%, p < 0.001), and venous thrombosis (4.0% vs 0%, p = 0.002). Following systematic management, aAVP-D patients experienced significant reductions in serum sodium, uric acid, and creatinine levels, although these remained higher than in the non-aAVP-D group. ROC analysis indicated that a serum sodium level above 149.5 mmol/L predicted aAVP-D. Conclusion Patients with aAVP-D had more tumor involvement in the hypothalamic region, surgical resections, hypothalamic syndrome, hypopituitarism, and complications. Serum sodium levels above 149.5 mmol/L necessitated heightened vigilance for aAVP-D. Early identification and systematic management reduced complications, though clinical management remained challenging. What is Known • Adipsic arginine vasopressin deficiency (aAVP-D) is a rare and high-risk syndrome that is difficult to recognize and manage. • There are few reports on aAVP-D, most of which focus on adult patients. • The characteristics and management of aAVP-D in children and adolescents remain unclear. What is New • Children and adolescents with aAVP-D experienced higher rates of hypothalamic region tumor involvement, surgical resections, hypothalamic syndrome, hypopituitarism, and associated complications. • Serum sodium levels above 149.5 mmol/L necessitated heightened vigilance for aAVP-D. • Early recognition and structured management of ADI lowered the risk of complications.
(© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Databáze: MEDLINE
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