The Complexity of Pain Management in Children Affected by Mucopolysaccharidoses

Table 1

Experiences about pain in children with MPS.

Source	Subjects	MPS type	Prevalence of joint pain
Brans et al. [10]	89 adult and pediatric MPS patients (55 of whom agreed to participate)	MPS I, MPS II, MPS III, MPS IV, MPS VI, MPS type unknown	69% of children reported joint pain, mainly hip (27,8%) and back pain (25,9%). The highest frequency of pain was observed in MPS III group (52.9%)
Hendriksz et al. [23]	Adult and pediatric MPS patients with	Morquio A Syndrome (MPS IVA)	64% of children reported joint pain (spinal area (63%), lower extremities (100%), upper extremities (69%), and head and neck area (56%))
Vijay and Wraith [11]	29 adult and pediatric MPS patients	Attenuated MPSI phenotype	Progressive arthropathy (86%), fixed flexion deformity of the fingers (24%), and kyphosis, scoliosis, and/or lordosis (24%)
White and Sousa [12]	18 pediatric MPS patients	MPSIII	Many patients requested orthopaedic evaluation of hip pain (hip dysplasia in 8 patients; bilateral osteonecrosis of the femoral heads in 4 patients)
de Ruijter et al. [13]	33 adult and pediatric MPS III patients	MPS-3A, MPS-3B, MPS-3C	For 15 of the 33 patients, pain was indicated in one or both hips