INTRODUCTION

Endocrine cells may be localized in a defined glandular organ such as the pancreas (islet cells) or in the adrenal gland. Others may be distributed throughout a nonendocrine gland such as the stomach. Functional disorders of the endocrine gland may result from overactivity of the glands or atrophy. The former results in overproduction of a given hormone, while the latter is a failure to produce the hormone.

Although a number of endocrine glands are involved in this process and other organs such as the thyroid and adrenal gland will be discussed in other chapters (see “Autoimmunity,” Chapter 6), we will concentrate our discussion on a single organ, the pancreas, and the disease insulin-dependent diabetes mellitus (IDDM).

INSULIN-DEPENDENT DIABETES MELLITUS

IDDM is a T-cell-mediated autoimmune disease. Its etiology is multifactorial, involving several predisposing genes and complex environmental factors. The analysis of disease pathogenesis and the search for new treatments have benefited enormously from the availability of two spontaneous animal models of the disease: (1) the nonobese diabetic (NOD) mouse and (2) the bio-breeding (BB) rat.

CLINICAL PRESENTATION

Most commonly, IDDM starts suddenly in a previously healthy individual, usually a child. The initial clinical symptoms include polyuria and polydipsia as a consequence of osmotic diuresis induced by glycosuria. If the disease is not diagnosed early, weight loss is observed, and in some cases ketoacidosis, leading to coma. The biological hallmarks of the disease are hyperglycemia and glycosuria. The treatment is based on the regular administration of exogenous insulin in doses and with a timing adapted to each individual patient.