Hunter syndrome is an X-linked lysosomal storage disease caused by mutations of the gene encoding the lysosomal enzyme iduronate-2-sulfatase, resulting in a deficiency of that enzyme and accumulation of large sugar molecules called glycosaminoglycans in body tissues. Symptoms are diagnosed through urinary glycosaminoglycan analysis, enzyme activity testing in plasma, and genetic testing. Treatments include enzyme replacement therapy and symptom-based therapies, while Ayurveda views it as a disease caused by weak digestion from accumulation of undigested matter (aam) in the body, recommending treatments to improve digestion and remove impurities.

1. Hunter syndrome and its
Ayurveda View
BY : Dr. V. K. Pathak

2. Introduction
• Hunter syndrome, or mucopolysaccharidosis type II (MPS II)
• X-linked lysosomal storage disease caused by mutations of the gene encoding the lysosomal enzyme
iduronate-2-sulfatase
• Deficiency of the lysosomal enzyme iduronate-2-sulfatase (I2S) causes accumulation of Large sugar
molecules called glycosaminoglycans (or GAGs or mucopolysaccharides) in body tissues.
GAG 1 GAG 2
GAG 1
GAG 2
Accumulation
of GAG
O linked sulfates
Iduronate-2-sulfatase

5. Symptoms

8. diagnosis
• Urinary GAG’s analysis
• Enzyme activity testing in plasma
• Genetic testing
• Prenatal screening

9. Treatment
• Enzyme replacement therapy
• Symptom based therapy

10. Jatharagni, Bhootagni, Dhatwagni
Mandya
Formation of Aam
Accumulation of Aam
Diasease symptoms
Agni Vardhan Sodhan
Basti Kayavirechan

11. THANK YOU