Open AccessAnesthetic management of a child with Hunter′s syndrome
Author(s) -
Jasmeet Kaur,
Aditi Swami,
Amit Kumar,
Sneh Lata
Publication year - 2012
Publication title -
journal of anaesthesiology-clinical pharmacology/journal of anaesthesiology clinical pharmacology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.466
H-Index - 32
eISSN - 2231-2730
pISSN - 0970-9185
DOI - 10.4103/0970-9185.94914
Subject(s) - medicine , glycosaminoglycan , hunter syndrome , inguinal hernia , anesthetic , airway management , enzyme replacement therapy , hernia , disease , bioinformatics , airway , surgery , pathology , anatomy , biology
Hunter's syndrome is a member of a group of recessively inherited metabolic disorders termed mucopolysaccharidoses, caused by deficiency of lysosomal enzymes required for degradation of mucopolysaccharides or glycosaminoglycans, leading to accumulation of partially degraded glycosaminoglycans in various tissues. This leads to various anatomical abnormalities and systemic involvement, posing a challenge to an anesthetist. We present the anesthetic management of a 4-year old child with Hunter's disease with anticipated difficult airway, who presented for adenotonsillectomy and repair of umbilical and inguinal hernia.