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Intrinsic factor within parietal cells of patients with juvenile pernicious anemia. A retrospective
Insights
Juvenile pernicious anemia involves inadequate intrinsic factor (IF) production. This study found immunogenic IF in parietal cells of affected children, suggesting diverse underlying causes for this condition.
Area of Science:
- Pediatric Gastroenterology
- Hematology
- Immunology
Background:
- Juvenile pernicious anemia is characterized by inadequate intrinsic factor (IF) production, leading to cobalamin deficiency.
- Previous assessments inferred inadequate IF production from absent stimulated gastric secretions.
Purpose of the Study:
- To investigate the presence of immunogenic intrinsic factor (IF) within parietal cells of children diagnosed with juvenile pernicious anemia.
- To explore the heterogeneity of juvenile pernicious anemia by examining IF expression in gastric biopsy specimens.
Main Methods:
- Utilized an indirect immunoperoxidase method on paraffin-embedded gastric biopsy specimens from children with juvenile pernicious anemia.
- Conducted a blinded evaluation of biopsy material, including specimens from siblings and patients with Imerslund's syndrome.
Main Results:
- Immunogenic IF was detected in six out of nine fundic biopsy specimens from children with juvenile pernicious anemia.
- Sibling pairs showed concordant results for intracellular IF presence or absence.
- All six gastric biopsy specimens from patients with Imerslund's syndrome tested positive for IF.
Conclusions:
- Juvenile pernicious anemia represents a heterogeneous group of disorders.
- The clinical presentation may stem from inadequate IF synthesis, secretion defects, or the production of functionally abnormal IF variants.
Abstract:
One of the diverse group of disorders that cause pernicious anemia in childhood, juvenile pernicious anemia, has been characterized by normal acid secretion, normal gastric mucosal histology, adequate intestinal absorption of cobalamin in the presence of exogenous gastric intrinsic factor (IF), but the inadequate "production" of IF from birth. Inadequate production has been inferred from the absence of measured IF in stimulated gastric secretions. To assess whether immunogenic IF was commonly present within the parietal cells of subjects with juvenile pernicious anemia, we studied paraffin-embedded biopsy material from the largest reported series of childhood pernicious anemia, using a well-characterized indirect immunoperoxidase method. Preliminary studies were able to identify IF in fundic mucosal biopsy specimens that had been stored for as long as 27 yr. In a blinded evaluation, six of the nine fundic biopsy specimens from children with juvenile pernicious anemia demonstrated immunogenic IF. Two sets of siblings were concordant for the presence or absence of intracellular IF, and six gastric biopsy specimens from patients with Imerslund's syndrome were all positive for IF. These findings indicate that juvenile pernicious anemia is a heterogeneous group of disorders whose similar clinical expression might be caused by (a) inadequate synthesis of IF, (b) a block in IF secretion, (c) the secretion of an abnormal IF that does not bind to cobalamin, or (d) the secretion of other abnormal IFs that could contain a number of other functional defects.
