Importance of professional oral hygiene for prevention of aspiration pneumonia caused by progressive central nervous system disorders in Type 2 Gaucher disease

Gaucher disease (GD) is rare that is caused by an enzyme deficiency of lysosomal glucocerebrosidase and is one of the most frequent lysosomal storage diseases. We present here the case of an infant male with Type 2 GD (GD2). GD2 is clinically characterized by precocious and rapid neurological degene...

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Published inJournal of oral and maxillofacial surgery, medicine, and pathology Vol. 31; no. 5; pp. 346 - 349
Main Authors Noriko, Komatsu, Eri, Watanabe, Rie, Yoshimura, Yoshiko, Suzuki, Kazuto, Hoshi, Tsuyoshi, Takato, Keiichi, Yamamoto, Jinichi, Fukuta
Format Journal Article
LanguageEnglish
Published Elsevier Ltd 01.09.2019
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Summary:Gaucher disease (GD) is rare that is caused by an enzyme deficiency of lysosomal glucocerebrosidase and is one of the most frequent lysosomal storage diseases. We present here the case of an infant male with Type 2 GD (GD2). GD2 is clinically characterized by precocious and rapid neurological degeneration comprising brainstem involvement associated with hepatosplenomegaly, pulmonary and hematological involvements, and death will be in infancy or early childhood. We performed professional oral hygiene on a patient with GD2, and we contributed to the communication between the patient and his family. Here we report on the oral cavity features in GD2 and how our experience can contribute to the prevention of aspiration pneumonia.
ISSN:2212-5558
2212-5566
DOI:10.1016/j.ajoms.2019.03.007