Endoscopic adenoidectomy in a case of Scheie syndrome (MPS I S)

Research output: Contribution to journalArticle

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Abstract

The mucopolysaccharidoses (MPS) are a heterogenous group of relatively rare, progressive, inherited lysosomal storage disorders, characterized by a deficiency of lysosomal enzymes which are responsible for the stepwise degradation of glycosaminoglycans. Their deficiency leads to the accumulation of glycosaminoglycans in various organs causing progressive disruption of cellular functions and multiple systemic effects including otolaryngological problems, upper airway obstructive disease being the most common. Scheie syndrome (MPS I S) is due to the deficient activity of α-L-iduronidase leading to the intralysosomal accumulation of dermatan sulfate and heparan sulfate. We present our experience in one such case occuring in a 6-year-old girl with ENT manifestations and who underwent a successful endoscopic adenoidectomy for symptomatic adenoid hypertrophy. This procedure was preferred over a conventional adenoidectomy in order to avoid complications associated with abnormal cervical vertebrae.

Original languageEnglish
Pages (from-to)177-181
Number of pages5
JournalInternational Journal of Pediatric Otorhinolaryngology
Volume44
Issue number2
DOIs
Publication statusPublished - 10-07-1998

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Mucopolysaccharidosis I
Adenoidectomy
Glycosaminoglycans
Iduronidase
Mucopolysaccharidoses
Cervical Vertebrae
Adenoids
Dermatan Sulfate
Heparitin Sulfate
Hypertrophy
Enzymes

All Science Journal Classification (ASJC) codes

  • Otorhinolaryngology
  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine
  • Surgery

Cite this

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abstract = "The mucopolysaccharidoses (MPS) are a heterogenous group of relatively rare, progressive, inherited lysosomal storage disorders, characterized by a deficiency of lysosomal enzymes which are responsible for the stepwise degradation of glycosaminoglycans. Their deficiency leads to the accumulation of glycosaminoglycans in various organs causing progressive disruption of cellular functions and multiple systemic effects including otolaryngological problems, upper airway obstructive disease being the most common. Scheie syndrome (MPS I S) is due to the deficient activity of α-L-iduronidase leading to the intralysosomal accumulation of dermatan sulfate and heparan sulfate. We present our experience in one such case occuring in a 6-year-old girl with ENT manifestations and who underwent a successful endoscopic adenoidectomy for symptomatic adenoid hypertrophy. This procedure was preferred over a conventional adenoidectomy in order to avoid complications associated with abnormal cervical vertebrae.",
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Endoscopic adenoidectomy in a case of Scheie syndrome (MPS I S). / Nayak, Dipak Ranjan; Balakrishnan, Ramaswamy; Adolph, Shekher.

In: International Journal of Pediatric Otorhinolaryngology, Vol. 44, No. 2, 10.07.1998, p. 177-181.

Research output: Contribution to journalArticle

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