Autoimmune polyglandular failure associated with malabsorption and gastric carcinoid tumour. 1993

G Leibowitz, and G Amir, and I S Losses, and R Eliakim
Department of Medicine, Hadassah University Hospital, Mount Scopus, Jerusalem, Israel.

A 21-year-old female with autoimmune polyglandular failure (APG) manifested by insulin-dependent diabetes mellitus (IDDM), hypothyroidism and pernicious anaemia developed severe malabsorption due to exocrine pancreatic insufficiency. Supplemental pancreatic enzymes resulted in marked improvement of steatorrhea. There was also an incidental finding of gastric carcinoid tumour. We identified only 13 other patients in our institution with either type 2 or 3 APG, one of which had significant steatorrhoea. Another patient with IDDM, hypothyroidism and pernicious anaemia had an asymptomatic gastric carcinoid tumour. The possible mechanisms for malabsorption in APG are discussed.

UI MeSH Term Description Entries
D008286 Malabsorption Syndromes General term for a group of MALNUTRITION syndromes caused by failure of normal INTESTINAL ABSORPTION of nutrients. Malabsorption Syndrome,Syndrome, Malabsorption,Syndromes, Malabsorption
D002276 Carcinoid Tumor A usually small, slow-growing neoplasm composed of islands of rounded, oxyphilic, or spindle-shaped cells of medium size, with moderately small vesicular nuclei, and covered by intact mucosa with a yellow cut surface. The tumor can occur anywhere in the gastrointestinal tract (and in the lungs and other sites); approximately 90% arise in the appendix. It is now established that these tumors are of neuroendocrine origin and derive from a primitive stem cell. (From Stedman, 25th ed & Holland et al., Cancer Medicine, 3d ed, p1182) Argentaffinoma,Carcinoid,Carcinoid, Goblet Cell,Argentaffinomas,Carcinoid Tumors,Carcinoids,Carcinoids, Goblet Cell,Goblet Cell Carcinoid,Goblet Cell Carcinoids,Tumor, Carcinoid,Tumors, Carcinoid
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D013274 Stomach Neoplasms Tumors or cancer of the STOMACH. Cancer of Stomach,Gastric Cancer,Gastric Neoplasms,Stomach Cancer,Cancer of the Stomach,Gastric Cancer, Familial Diffuse,Neoplasms, Gastric,Neoplasms, Stomach,Cancer, Gastric,Cancer, Stomach,Cancers, Gastric,Cancers, Stomach,Gastric Cancers,Gastric Neoplasm,Neoplasm, Gastric,Neoplasm, Stomach,Stomach Cancers,Stomach Neoplasm
D016884 Polyendocrinopathies, Autoimmune Autoimmune diseases affecting multiple endocrine organs. Type I is characterized by childhood onset and chronic mucocutaneous candidiasis (CANDIDIASIS, CHRONIC MUCOCUTANEOUS), while type II exhibits any combination of adrenal insufficiency (ADDISON'S DISEASE), lymphocytic thyroiditis (THYROIDITIS, AUTOIMMUNE;), HYPOPARATHYROIDISM; and gonadal failure. In both types organ-specific ANTIBODIES against a variety of ENDOCRINE GLANDS have been detected. The type II syndrome differs from type I in that it is associated with HLA-A1 and B8 haplotypes, onset is usually in adulthood, and candidiasis is not present. Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal-Dystrophy,Autoimmune Syndrome Type I, Polyglandular,Autoimmune Syndrome Type II, Polyglandular,Polyglandular Type I Autoimmune Syndrome,Polyglandular Type II Autoimmune Syndrome,Schmidt's Syndrome,AIRE Deficiency,APECED,APS Type 1,Autoimmune Polyendocrine Syndrome, Type 2,Autoimmune Polyendocrine Syndrome, Type II,Autoimmune Polyendocrinopathy Syndrome Type 1,Autoimmune Polyendocrinopathy with Candidiasis and Ectodermal Dystrophy,Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy,Autoimmune Polyglandular Syndrome Type I,Autoimmune Polyglandular Syndrome Type II,Autoimmune Polyglandular Syndrome Type III,Autoimmune Polyglandular Syndrome, Type 1,Autoimmune Polyglandular Syndrome, Type 3,Autoimmune Polyglandular Syndrome, Type I,Autoimmune Syndrome Type III, Polyglandular,Diabetes Mellitus, Addison Disease, Myxedema,Diabetes Mellitus, Addison's Disease, Myxedema,Multiple Endocrine Deficiency Syndrome, Type 2,Polyendocrine Autoimmune Syndrome, Type II,Polyendocrinopathy-Candidiasis-Ectodermal-Dystrophy, Autoimmune,Polyglandular Autoimmune Syndrome, Type 1,Polyglandular Autoimmune Syndrome, Type 2,Polyglandular Autoimmune Syndrome, Type 3,Polyglandular Autoimmune Syndrome, Type I,Polyglandular Deficiency Syndrome, Type 2,Polyglandular Type III Autoimmune Syndrome,Schmidt Syndrome,AIRE Deficiencies,Autoimmune Polyendocrinopathy,Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy,Deficiency, AIRE,Polyendocrinopathy Candidiasis Ectodermal Dystrophy, Autoimmune,Polyendocrinopathy, Autoimmune,Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy, Autoimmune,Syndrome, Schmidt,Syndrome, Schmidt's

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