Table 8 “Red flag” signs and symptoms of MPS II that occur early in the disease course

Coarse facial features (may be subtle in the attenuated phenotype)

Recurrent respiratory infections

Chronic rhinorrhoea

Upper airway restriction/noisy breathing/snoring

Recurrent otitis media

Hearing loss

Heart murmur

Hepatomegaly

Umbilical and inguinal hernia

Recurrent watery diarrhoea

Joint stiffness

Developmental delay and/or speech delay (in severe phenotype only)