Indian Journal of Radiology and Imaging Indian Journal of Radiology and Imaging

LETTER
Year
: 2008  |  Volume : 18  |  Issue : 2  |  Page : 175-

Hurler syndrome or Morquio syndrome: Intelligence required for diagnosing the case


Amar Taksande, Krishna Vilhekar 
 Department of Pediatrics, Mahatma Gandhi Institute of Medical Sciences, Sevagram - 442 102, Wardha, Maharashtra, India

Correspondence Address:
Amar Taksande
Department of Pediatrics, Mahatma Gandhi Institute of Medical Sciences, Sevagram - 442 102, Wardha, Maharashtra
India




How to cite this article:
Taksande A, Vilhekar K. Hurler syndrome or Morquio syndrome: Intelligence required for diagnosing the case.Indian J Radiol Imaging 2008;18:175-175


How to cite this URL:
Taksande A, Vilhekar K. Hurler syndrome or Morquio syndrome: Intelligence required for diagnosing the case. Indian J Radiol Imaging [serial online] 2008 [cited 2019 Sep 20 ];18:175-175
Available from: http://www.ijri.org/text.asp?2008/18/2/175/40306


Full Text

Dear Sir,

I have read with interest the article by Das et al ., [1] in which they have described a child with Hurler syndrome (MPS I), diagnosed on the basis of the clinical findings and radiological examination. In Hurler syndrome, the diagnosis is made between 6-24 months in the presence of hepatosplenomegaly, skeletal deformity, coarse facial features, corneal clouding, joint stiffness, and short stature. Hearing loss is common and developmental delay, with moderate mental retardation, is present. [2] Morquio syndrome, on the other hand, is characterized by short-trunk dwarfism, corneal deposits, skeletal dysplasia, and preservation of intelligence. Extraskeletal manifestations may include hepatomegaly, cardiac valvular lesions, and small teeth with caries formation. [2],[3]

The author has not mentioned the status of mental development or the results of echocardiography.

In my opinion, this may be a patient with Morquio syndrome in view of the presence of hepatomegaly, cardiac valvular lesion, swollen alveolar ridges and gums with misaligned teeth, cloudy corneas, skeletal dysplasia, normal auditory functions, and normal intelligence.

References

1Das A, Ray B, Das D, Das S. Images: Hurler's disease. Indian J Radiol Imaging 2007;17:277-9.
2Kliegman RM, Muenzer JL. Mucopolysaccharidoses. In : Behrman RE, Kliegman RM, Jenson HB, editors. Nelson's textbook of pediatrics. 17 th ed. Saunders: Philadelphia; 2004. p. 482-6.
3Rekhi GS. Morquio syndrome (MPV IV): A case report. Indian J Ophthalmol 1991;39:78-81.