Table 3.
Recommended pre-closure endocrine assessment in patients with CPSS.
| System | Laboratory and clinical assessment |
|---|---|
| Thyroid1 | TSH, Free T4, total T4, TBG, free T3, total T3, reverse T3, thyroglobulin |
| Glucose homeostasis1 |
In pre-school patients or patients with
clinical suspicion of hypoglycaemia: fasting glucose and
glucose measurements 1.5 – 2 – 2.5 h after a carbohydrate rich meal
(when available preferably continuous glucose monitoring for several
days). In case of hypoglycaemia (<2.5-3.0 mmol/L): insulin, ketones and free fatty acids at time of hypoglycaemia. |
| Adrenal1 |
In case of clinical suspicion of
hyperandrogenism: bone age, IGF-1,
IGF-BP3, LH, FSH, testosterone, estradiol, DHEA, DHEA-S,
androstenedione. In case of clinical suspicion of adrenal insufficiency: morning cortisol followed by synacten stimulation. Diagnosis according to standard criteria (morning cortisol and/or synacten test). |
| Growth2 | Measure and plot weight and height + parental
height. Tanner staging |
CPSS, congenital portosystemic shunts; DHEA, dehydroepiandrosterone; DHEA-S, dehydroepiandrosterone sulphate; FSH, follicle stimulating hormone; IGF, insulin growth factor; LH, luteinizing hormone; TBG, thyroid binding globulin; TSH, thyroid stimulating hormone.
1
Repeat post-closure in case of abnormal findings.
2
Repeat post-closure irrespective of findings post-closure.