Which problem is common in a child with Hurler's syndrome?

Which problem is common in a child with Hurler's syndrome?

• A. Developmental delay from birth
• B. Sleep obstructive apnea
• C. Ectopic lentis
• D. Congenital heart disease

Answer: (B)

Hurler syndrome causes storage of glycosaminoglycans in tissues, leading to airway and craniofacial changes that narrow the upper airway. This makes obstructive sleep apnea a very common problem in affected children, as the tongue, tonsils, and airway structures become bulky and the airway easily collapses during sleep. Cardiac involvement and developmental delays can occur in Hurler’s, but the frequent, defining issue related to daily life for these kids is sleep-disordered breathing from airway obstruction. Ectopic lentis points to other disorders like homocystinuria, not Hurler’s, and while congenital heart disease can be seen, obstructive sleep apnea is the most characteristic and common problem in this context.

Hurler syndrome causes storage of glycosaminoglycans in tissues, leading to airway and craniofacial changes that narrow the upper airway. This makes obstructive sleep apnea a very common problem in affected children, as the tongue, tonsils, and airway structures become bulky and the airway easily collapses during sleep. Cardiac involvement and developmental delays can occur in Hurler’s, but the frequent, defining issue related to daily life for these kids is sleep-disordered breathing from airway obstruction. Ectopic lentis points to other disorders like homocystinuria, not Hurler’s, and while congenital heart disease can be seen, obstructive sleep apnea is the most characteristic and common problem in this context.