Efficacy and safety of prenatal dexamethasone treatment in offspring at risk for congenital adrenal hyperplasia due to 21‐hydroxylase deficiency: A systematic review and meta‐analysis

2019 ◽  
Vol 92 (2) ◽  
pp. 109-123 ◽  
Author(s):  
Lizhen Xu ◽  
Wei Lin ◽  
Liangchun Cai ◽  
Huibin Huang ◽  
Jixing Liang ◽  
...  
Keyword(s):  
Systematic Review ◽  
At Risk ◽  
Congenital Adrenal Hyperplasia ◽  
Meta Analysis ◽  
Efficacy And Safety ◽  
Adrenal Hyperplasia ◽  
Dexamethasone Treatment ◽  
Hydroxylase Deficiency ◽  
21 Hydroxylase Deficiency

scholarly journals MON-156 Efficacy and Safety of Prenatal Dexamethasone Treatment in Offspring at Risk for Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Lizhen Xu ◽  
Wei Lin ◽  
Nengying Wang ◽  
Junping Wen ◽  
Gang Chen
Keyword(s):  
At Risk ◽  
Congenital Adrenal Hyperplasia ◽  
Behavioral Problems ◽  
Cognitive Functions ◽  
Cochrane Library ◽  
Efficacy And Safety ◽  
Adrenal Hyperplasia ◽  
Dexamethasone Treatment ◽  
21 Hydroxylase Deficiency ◽  
Physical Outcomes

Abstract Objective: To assess the efficacy and safety of prenatal dexamethasone treatment in offspring at risk for congenital adrenal hyperplasia. Methods: MEDLINE, EMBASE, the Cochrane Library, the clinicaltrials.gov website databases was systematically searched from inception through March 2019. WMD and SMD with 95%CIs were calculated using random or fixed effects models. Results: There was a significant reduction of virilizationin the DEX-treated group (WMD: -2.39, 95%CI: -3.31,-1.47). No significant differences were found in newborn physical outcomes for birth weight (WMD: 0.09, 95%CI: -0.09, 0.27) and birth length (WMD= 0.27, 95%CI: -0.68, 1.21). Concerning cognitive functions, no significant differences in the domains of psychometric intelligence (SMD: 0.05, 95%CI: -0.74, 0.83), verbal memory (SMD: -0.17, 95%CI: -0.58, 0.23), visual memory (SMD: 0.10, 95%CI: -0.14, 0.34), learning (SMD: -0.02, 95%CI: -0.27, 0.22), verbal processing (SMD: -0.38, 95%CI: -0.93, 0.17). Regarding behavioral problems, no significant differences in the domains of internalizing problems (SMD: 0.16, 95%CI: -0.49, 0.81), externalizing problems (SMD: 0.07, 95%CI: -0.30, 0.43), total problems (SMD: 0.14, 95%CI: -0.23, 0.51). With respect to temperament, no significant differences in the domains of emotionality (SMD: 0.13, 95%CI: -0.79, 1.05), activity (SMD: 0.04, 95%CI: -0.32, 0.39), shyness (SMD: 0.25, 95%CI: -0.70, 1.20), sociability (SMD: -0.23, 95%CI: -0.90, 0.44). Conclusions: Prenatal DEX treatment reduced virilization with no significant differences in newborn physical outcomes, cognitive functions, behavioral problems, temperament. The results need to be interpreted cautiously due to the existence of limitations.

Height outcome in congenital adrenal hyperplasia caused by 21-hydroxylase deficiency: A meta-analysis

2001 ◽  
Vol 138 (1) ◽  
pp. 26-32 ◽  
Author(s):  
Erica A. Eugster ◽  
Linda A. DiMeglio ◽  
James C. Wright ◽  
Gary R. Freidenberg ◽  
Roopa Seshadri ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Meta Analysis ◽  
Adrenal Hyperplasia ◽  
Hydroxylase Deficiency ◽  
21 Hydroxylase Deficiency

scholarly journals PREVALENCE AND CHARACTERISTICS OF ADRENAL TUMORS AND MYELOLIPOMAS IN CONGENITAL ADRENAL HYPERPLASIA: A SYSTEMATIC REVIEW AND META-ANALYSIS

2020 ◽  
Vol 26 (11) ◽  
pp. 1351-1361
Author(s):  
Ingrid Nermoen ◽  
Henrik Falhammar
Keyword(s):  
Cytochrome P450 ◽  
Congenital Adrenal Hyperplasia ◽  
Case Reports ◽  
Meta Analysis ◽  
Adrenal Tumors ◽  
Adrenal Hyperplasia ◽  
Hydroxylase Deficiency ◽  
Adrenal Imaging ◽  
Cytochrome P450 Family ◽  
21 Hydroxylase Deficiency

Objective: The prevalence of adrenal tumors in congenital adrenal hyperplasia (CAH) is uncertain. Our objective was to estimate the prevalence and characteristics of adrenal tumors and myelolipoma in CAH, and investigate clinical features of this population. Methods: We carried out systematic searches in Medline Ovid and Embase for articles published until January, 2020. Studies with confirmed CAH, biochemically and/or genetically, were included. The two authors independently extracted data from each study. Results: Six cohort studies were included in the prevalence calculation. In addition, 32 case reports on adrenal myelolipomas and CAH were included. The prevalence of adrenal tumors in CAH was 29.3%. When only studies with genetically verified cytochrome P450, Family 21, subfamily A, polypeptide 2 gene ( CYP21A2) mutations were included the prevalence was 23.6%. The prevalence of myelolipoma in CAH was 7.4% (verified CYP21A2 mutations 8.6%). The proportion of myelolipoma in the adrenal tumors was 25.4% (genetically verified 36.6%). The median (range) age at tumor diagnosis was 36.0 (12 to 60) years and there were more tumors in males than in females (37.9% versus 22.1%; P<.05). In patients with myelolipomas, 93.5% had an undiagnosed or poorly managed CAH. Conclusion: Patients with CAH had a high prevalence of adrenal tumors, particularly myelolipomas. Those with myelolipomas had a high frequency of late-diagnosed or poorly controlled CAH. Adrenal imaging may be considered in patients with CAH, especially if abdominal pain is present. Abbreviations: ACTH = adrenocorticotropic hormone; CAH = congenital adrenal hyperplasia; CT = computed tomography; CYP21A2 = cytochrome P450, Family 21, subfamily A, polypeptide 2 gene; HU = Hounsfield units; MRI = magnetic resonance imaging; 21-OHD = 21-hydroxylase deficiency; 17-OHP = 17-hydroxyprogesterone; SV = simple virilizing

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