MCQ – 87829

A 5-year-old child who has received no medical care since birth has had gradual onset of markedly decreased vision bilaterally. The child also has a history of increased respiratory tract infections caused by Haemophilus influenzae, Streptococcus pneumoniae, Klebsiella pneumoniae, and rubeola. The figure shows the representative microscopic appearance of the bronchial mucosa. The child also has passed several urinary tract calculi. On physical examination,generalized papular dermatosis is noted. The child has xerophthalmia, and there is marked keratomalacia with corneal clouding. Bilateral crackles are audible in the lungs on auscultation. What disease process would most likely lead to these findings?

Correct Answer � D Explanation

Answer : D Vitamin A deficiency

• Vitamin A deficiency leads to epithelial disorders affecting the cornea, skin, respiratory tract, and urinary tract.

• Squamous metaplasia (shown) in the respiratory tract increases the risk of infection; desquamation of keratin debris forms the nidus of urinary tract calculi. 

• Hyperkeratosis and follicular plugging affect the epidermis. Cystic fibrosis leads to an increased risk of respiratory tract infections, particularly infections caused by Pseudomonas, from widespread bronchiectasis

• The skin and eye are not affected. Congenital syphilis can produce bone deformities and gummas. HIV infection can be complicated by opportunistic infections, including infections of the respiratory tract, but keratomalacia is not a feature of HIV infection. 

• Kartagener syndrome can lead to bronchiectasis from an altered respiratory tract epithelium

• in which the ciliary dynein arms are absent, and there is situs inversus; eye and skin changes are not present.