Summary
A patient is reported with a malabsorption syndrome associated with nodular lymphoid hyperplasia of the small intestine and dysgammaglobulinemia characterized by virtual absence of IgA and IgM, with a reduced concentration of IgG. Prominent clinical features were severe steatorrhea and tetany. The low serum calcium and transient mild hyperphosphatemia suggested parathyroid hypofunction, but studies of calcium metabolism did not substantiate this diagnosis. The degree of hypocalcemia seemed dependent upon the severity of the steatorrhea. Evidence of early osteomalacia probably reflected an associated Vitamin-D deficiency. Investigation failed to reveal the cause for steatorrhea, and the pathogenesis of malabsorption seen in patients with dysgammaglobulinemia remains undefined.
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Supported in part by NIH Research Grant FR-158, Division of Research Facilities and Resources, Bethesda, Md.
The authors are indebted to Nancy H. Holland, MD, and Miss Carol Bishop, Department of Pediatrics, University of Kentucky College of Medicine, who kindly performed and interpreted the immunoelectrophoretic studies. We also wish to thank Lawrence G. Raisz, MD, School of Medicine and Dentistry, University of Rochester, who performed the serum thyrocalcitonin assay.
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Anderson, F.L., Pellegrino, E.D. & Schaefer, J.W. Dysgammaglobulinemia associated with malabsorption and tetany. Digest Dis Sci 15, 279–286 (1970). https://doi.org/10.1007/BF02233462
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DOI: https://doi.org/10.1007/BF02233462