Table 1.

Signs and symptoms that should raise clinical suspicion of MPS.

Sign/Symptom of MPS		MPS Types
		I	II	III	IV	VI	VII	IX	Plus
Head and neck	Coarse facial features	+	+	+	+	+	+		+
	Hypertrichosis/thick scalp hair	+	+	+		+	+		+
	Hearing loss	+	+	+	+	+	+		+
	Macrocephaly/scaphocephaly	+	+	+		+	+
	Corneal clouding	+			+	+	+
	Abnormal dentition	+	+	+	+	+	+
	J-shaped sella turcica	+	+			+	+		+
Osteoarticular	Short stature	+	+		+	+	+		+
	Joint stiffness	+	+	+		+	+	+	+
	Hip dysplasia	+	+	+	+	+	+	+
	Thoracolumbar kyphosis	+	+		+	+	+		+
	Genu valgum	+	+		+	+	+
	Odontoid dysplasia	+	+		+	+
	Claw hands	+	+			+	+		+
	Bullet-shaped phalanges	+	+			+	+		+
	Carpal tunnel syndrome	+	+			+
	Joint laxity				+
Cardiovascular	Valve thickening / dysfunction	+	+	+	+	+	+
	Left ventricular hypertrophy	+	+	+	+	+	+		+
Neurological	Developmental delay/intellectual disability	+	+	+			+		+
	Ventriculomegaly	+	+	+		+	+
	Dilated perivascular spaces	+	+	+		+
	Hyperactive / aggressive behavior		+	+
Airways	Recurrent respiratory infections	+	+	+	+	+	+		+
	Obstructive airway disease	+	+		+	+	+		+
Abdomen	Hepatomegaly/splenomegaly	+	+	+		+	+
	Umbilical/inguinal hernia	+	+	+			+
Others	Abnormal granulation in leukocytes	+	+	+	+	+	+		+
	Fetal hydrops	+			+		+
	Proteinuria								+
	Cytopenias								+

A plus sign (+) indicates that the manifestation is associated to that specific mucopolysaccharidosis (MPS) disorder. MPSPS: mucopolysaccharidosis-plus syndrome.