Dr Kola Tytler MBBS CertHE MBA MRCGPClinical Lead • iatroX
Long-term monitoring requirements for patients diagnosed with congenital adrenal hyperplasia (CAH) include:
- Regular ongoing reviews with an appropriate specialist team to assess clinical status and medication management NICE NG243.
- For children and young people under 16 years, appointments should be offered at least every 6 months, with at least one face-to-face annual review to measure height and weight and adjust glucocorticoid and mineralocorticoid doses accordingly NICE NG243.
- Adjust the frequency of reviews based on clinical needs, growth phases (including puberty), changes in personal or family circumstances, transition to adult services, medication adherence concerns, or vulnerability NICE NG243.
- During reviews, assess psychological wellbeing, medication adherence, understanding of sick-day rules, frequency of adrenal crises, and signs of glucocorticoid under- or over-replacement, aiming for physiological glucocorticoid dosing NICE NG243.
- Monitor for signs of mineralocorticoid under- or over-replacement, including symptoms like light-headedness, salt craving, swollen ankles, or hypertension, and consider measuring renin to adjust fludrocortisone dose if needed NICE NG243.
- Offer measurements including blood pressure (lying and standing), electrolytes, HbA1c, bone density (at least once within 5 years after diagnosis in adults), and lipid profile to aid clinical decision-making NICE NG243.
- For children and young people, monitor growth parameters, progression through puberty, menstrual frequency if relevant, bone age (via X-ray), and bone density after growth completion or if fractures occur NICE NG243.
- Provide education and management plans to patients and carers, including emergency hydrocortisone injection kits and advice on dose adjustments during illness, stress, or lifestyle changes NICE NG243,NICE CKS.