ESPE2024 Poster Category 3 Adrenals and HPA Axis (22 abstracts)
Hamad General Hospital, Doha, Qatar
Introduction: Understanding the growth outcomes and management strategies in Salt-Wasting Congenital Adrenal Hyperplasia (SW-CAH) is crucial for optimizing patient care.
Objective: We review and synthesize the evidence on growth patterns and final adult height outcomes in pediatric patients with SW-CAH, focusing on the effects of different management strategies including glucocorticoid treatment, growth hormone (GH) therapy, and early hydrocortisone dose adjustments.
Methods: A systematic review of literature from 2000 to 2022 (16 studies) were studied and analyzed.
Results: (Table)
Author(s): | Year: | Key Findings: |
Cabrera, Vogiatzi, New: | 2001 | Adult males with CAH due to 21-hydroxylase deficiency exhibit short stature. Fertility issues noted in the salt-wasting form. |
Dixon et al: | 2017 | Adult height in CAH patients in Northern Ireland was -1.5 SDS below mean for both genders. |
Lemos-Marini et al: | 2005 | Final height in CAH patients was below target height, with no significant difference by CAH form or treatment onset age. |
Kawano et al: | 2014 | Early hydrocortisone dose adjustments based on growth velocity could lead to satisfactory height outcomes. |
Mohammad et al: | 2019 | Significant stunting in CAH cases, with salt-losing patients having lower height and higher BMI. |
Bomberg et al: | 2015 | Reduced final adult height SDS in CAH patients, especially in salt-wasting forms. |
Han et al: | 2014 | Adult CAH patients remain short; late-diagnosed, moderate severity CAH at risk of adult hypertension. |
Bonfig et al: | 2007 | Reduced final height in CAH patients treated with prednisone. |
Al Shaikh et al: | 2019 | Final height outcome in CAH patients treated with glucocorticoids is lower than the population norm. |
Bonfig: | 2016 | Treated SV and SW CAH children exhibit reduced final height. |
Lin-Su et al: | 2005 | GH and LHRHa treatment improve final adult height in CAH patients. |
Badawi et al: | 2021 | Bayley-Pinneau method overestimates final adult height in poorly controlled CAH patients. |
Mendes-dos-Santos et al: | 2011 | Growth recovery in CAH patients with mean height similar to general population but with higher body fat. |
Costa et al: | 2022 | Improvement in final height in CAH patients treated with recombinant GH. |
Abulgassem, BenRajab: | 2022 | Showed GH treatment effects on growth. |
Lin-Su et al: | 2011 | GH treatment improves final adult height in CAH patients. |
Conclusion: Pediatric patients with SW-CAH face significant challenges in achieving normal growth and final adult height. Management strategies including early and adjusted hydrocortisone dosing, along with adjunct therapies such as GH and LHRHa, have shown promise in improving growth outcomes.