Dysgammaglobulinemia associated with nodular lymphoid hyperplasia of the small intestine |
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| Authors: | P E Hermans K A Huizenga H N Hoffman A L Brown H Markowitz |
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| Affiliation: | 1. Department of Radiology, Erasmus Medical Center, Rotterdam, The Netherlands;2. Industrial Engineering & Innovation Sciences (IE&IS), Eindhoven University of Technology, Eindhoven, The Netherlands;3. Department of Medical Informatics, Erasmus Medical Center, Rotterdam, The Netherlands;4. Department of Internal Medicine, Division of Endocrinology, Erasmus Medical Center, Rotterdam, The Netherlands;5. Department of Epidemiology, Erasmus Medical Center, Rotterdam, The Netherlands;6. Department of Radiology, Erasmus Medical Center, Rotterdam, The Netherlands;7. Department of Health Policy and Management, Harvard T.H. Chan School of Public Health, Boston, USA;1. Unit of Reproductive Endocrinology, First Department of Obstetrics and Gynecology, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece;2. Department of Endocrinology and Diabetes, Hellenic Red Cross Hospital, Athens, Greece;1. University of South Carolina School of Medicine, Greenville, Greenville Health System Gastroenterology and Liver Center, Greenville, South Carolina;3. Department of Internal Medicine, Division of Digestive Diseases and Nutrition, Joy McCann Culverhouse Center for Swallowing Disorders, University of South Florida, Tampa, Florida;1. University of Kansas Medical Center, General Surgery, Kansas City, Kansas;2. University of Kansas School of Medicine - Kansas City, Kansas City, Kansas;3. University of Kansas School of Medicine, Family and Community Medicine, Wichita, Kansas |
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| Abstract: | A syndrome, noted in eight patients, is described. It is characterized by (1) dysgammaglobulinemia, consisting of virtual absence of the IgA and IgM immunoglobulins and a moderately decreased level of IgG immunoglobulin in the serum; (2) an unusual susceptibility to infections; (3) diarrhea, sometimes with steatorrheal features; (4) the presence of Giardia lamblia in the stools; and (5) nodular lymphoid hyperplasia of the small intestine. This combination of features allowed differentiation from gluten-sensitive idiopathic sprue and from acquired idiopathic hypogammaglobulinemia. The screening of patients with sprue-like syndromes also resulted in the detection of two patients with isolated IgA immunoglobulin deficiency. The nodular lymphocytic hyperplasia which may occur in association with dysgammaglobulinemia may be related to functional abnormalities of the so-called central lymphoid tissues. Of practical clinical importance are (1) the possibility of a presumptive diagnosis by roentgenographic studies which may show the nodules in the small intestine in most cases and (2) the suggestion that, in at least some of the patients, remission of the diarrhea may be achieved by the use of tetracycline. Also of clinical significance and of theoretical interest is an increased incidence of carcinoma of the gastrointestinal tract in patients with dysgammaglobulinemia and nodular lymphoid hyperplasia of the small intestine. |
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