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.
This is a preview of subscription content, access via your institution.
Buy single article
Instant access to the full article PDF.
Tax calculation will be finalised during checkout.
Herman PE, Huizenga KA, Hoffman HN, Brown AL Jr, et al: Dysgammaglobulinemia associated with nodular lymphoid hyperplasia of the small intestine.Amer J Med 40:78, 1966
Gryboski JD, Self TW, Clemett A, et al: Selective immunoglobulin A deficiency and intestinal nodular lymphoid hyperplasia: Correction of diarrhea with antibiotics and plasma.Pediatrics 42:833, 1968
Kirkpatrick CH, Waxman D, Smith OD, et al: Hypogammaglobulinemia with nodular lymphoid hyperplasia of the small bowel.Arch Intern Med 121:273, 1968
Grise JW: Dysgammaglobulinemia with nodular lymphoid hyperplasia of the small intestine.Radiology 90:579, 1968
Scheidegger JJ: Une micro-methode de l'immunoelectrophorese.Int Arch Allerg 7:103, 1955
West CD, Hong R, Holland, NH: Immunoglobulin levels from the new born period to adulthood and in immunoglobulin deficiency states.J Clin Invest 41:2054, 1966
Avioli LV, McDonald JE, Singer RA, et al: A new oral isotopic test of calcium absorption.J Clin Invest 44:128, 1965
Jowsey J, Kelly PJ, Riggs BL, et al: Quantitative microradiographic studies of normal and osteoporotic bone.J Bone Joint Surg 47A:785, 1965
Cohen N, Paley D, Janowitz HD: Acquired hypogammaglobulinemia with malabsorption and marked alterations in the gastrointestinal mucosa.Acta Med Scand 173:549, 1963
Williams E, Wood C: The syndrome of hypoparathyroidism and steatorrhea.Arch Dis Child 34:302, 1959
Jackson WPU: Steatorrhea and hypoparathyroidism.Lancet 272:1086, 1957
Clarkson B, Kowlessar OD, Horwith M, et al: Clinical and metabolic study of a patient with malabsorption and hypoparathyroidism.Metabolism 9:1093, 1960
Jackson WPU, Hoffenberg R, Linder GC, et al: Syndrome of steatorrhea, pseudohypoparathyroidism, and amenorrhea.J Clin Endocr 16:1043, 1956
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.
About this article
Cite this article
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
- Public Health
- Small Intestine
- Serum Calcium