Volume : VIII, Issue : IV, April - 2018

Challenge in the management of a case of Mucopolysaccharidosis type-1

Dr. Krittibus Samui

Abstract :

3years old baby girl had fever since 1year of age. Based on clinical evaluation, serum enzyme assay and excess glycosaminoglycan level in urine, patient was diagnosed to have mucopolysaccharidosis type I.Glycosaminoglycan progressively accumulates within the cellsand compromises organ function. After a few weeks course of enzyme replacement therapy, haematopoietic stem cell transplantation was done. But fever persisted. Clinical evaluation and investigation did not find out any infective focus. Initially fever was thought to be due to engraftment syndrome. But in spite of neutrophil recovery in Day+14 after transplant, fever spike continued to be same. Immunosuppressant therapy was increased and fever spike improved because of settled immunological reactions in the body. So, in case of fever after bone marrow transplant immune mechanism is to be kept in mind.

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Article: Download PDF   DOI : 10.36106/ijar  

Cite This Article:

Dr. Krittibus Samui, Challenge in the management of a case of Mucopolysaccharidosis type-1, INDIAN JOURNAL OF APPLIED RESEARCH : Volume-8 | Issue-4 | April-2018

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