| Respiratory | Upper airway obstruction, sleep apnea, recurrent infections |
| Cardiovascular | Valve disease (mitral/aortic), cardiomyopathy, coronary artery disease |
| Skeletal | Dysostosis multiplex, short stature, joint stiffness, claw-hand deformities |
| Abdominal | Hepatomegaly, splenomegaly, inguinal/umbilical hernias |
| ENT | Chronic otitis media, hearing loss (conductive and sensorineural) |
| Ophthalmologic | Retinal degeneration, corneal clouding (less severe than MPS I) |
| Dermatologic | Characteristic "pebbly" papules on scapular region |
| Databases | OMIMOrphanetClinicalTrialsPubMed |