Imerslund-Grasbeck syndrome associated with recurrent aphthous stomatitis and defective neutrophil function.

Imerslund-Grasbeck syndrome associated with recurrent aphthous stomatitis and defective neutrophil function. Research Abstract Details 

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Imerslund-Grasbeck syndrome associated with recurrent aphthous stomatitis and defective neutrophil function. Abstract Text:

Arnon Broides,Baruch Yerushalmi,Rachel Levy,Nurit Hadad,Norma Kaplun,Stephan M Tanner,Allbert de la Chapelle,Jacov Levy,

Vitamin B(12) deficiency is a well-known cause of recurrent aphthous stomatitis (RAS). However, the mechanism by which this deficiency causes the stomatitis is not well understood. Imerslund-Grasbeck syndrome (IGS) causes vitamin B(12) deficiency and proteinuria due to a defect in the vitamin B(12) receptor. We sought to determine whether the RAS observed in IGS patients is associated with neutrophil dysfunction. We report 3 infants with vitamin B(12) deficiency due to IGS, who presented with borderline or normal hemoglobin concentrations, RAS, and a neutrophil function defect. All 3 patients were homozygous for a splice site mutation affecting exon 4 of the AMN gene. A direct correlation was observed between low serum vitamin B12 levels and defective neutrophil function (low chemotaxis and elevated superoxide production) in the patients. Vitamin B(12) therapy led to an immediate resolution of aphthous stomatitis and full correction of neutrophil function. We demonstrated that serum vitamin B(12) deficiency is associated with a neutrophil chemotactic defect and RAS in IGS patients. We suggest that the RAS observed in these patients is due to this defect.

Imerslund-Grasbeck syndrome associated with recurrent aphthous stomatitis and defective neutrophil function. Publishing Authors By Initials

A Broides,B Yerushalmi,R Levy,N Hadad,N Kaplun,SM Tanner,Ade Chapelle,J Levy,

For similar nutritional and metabolic diseases: nutrition disorders: malnutrition: deficiency diseases: avitaminosis: vitamin b deficiency research abstracts see: nutritional and metabolic diseases: nutrition disorders: malnutrition: deficiency diseases: avitaminosis: vitamin b deficiency research

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Imerslund-Grasbeck syndrome associated with recurrent aphthous stomatitis and defective neutrophil function. Journal Published:

PUBLICATION TYPE: Research Support, N.I.H., Extr

Journal: Journal of pediatric hematology/oncology : officia

VOLUME: 28

Page Numbers: 715-9

Journal Abbreviation: J. Pediatr. Hematol. Oncol.

ISSN: 1077-4114

DAY: 3

MONTH: Nov

YEAR: 2006

Imerslund-Grasbeck syndrome associated with recurrent aphthous stomatitis and defective neutrophil function. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 9505928

Imerslund-Grasbeck syndrome associated with recurrent aphthous stomatitis and defective neutrophil function. Keywords Mesh Terms:

KEYWORDS: Vitamin B Deficiency

MESH TERMS: genetics

Chemical & Substance for Abstract: Imerslund-Grasbeck syndrome associated with recurrent aphthous stomatitis and defective neutrophil function. Information

Substance Name: Vitamin B 12

Registry Number: 68-19-9

Grant and Affiliation Information for Imerslund-Grasbeck syndrome associated with recurrent aphthous stomatitis and defective neutrophil function.

AFFILIATION: Pediatric Immunology Clinic, Soroka Medical Center and the Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel. broides@bgu.ac.il

Country: United States

AGENCY: United States NCI

GRANT: CA16058

ACRONYM: CA

MEDLINETA: J Pediatr Hematol Oncol

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