DIABETES INSIPIDUS dr. Juslan dr. Marwan dr. Suriyanti Listin Dr. dr. Himawan Sanusi, Sp. PD, K-EMD
Division of Endocrinology Diabetes and Metabolism
Departement of Internal Medicine University of Hasanuddin Makassar, 2020 Introduction Diabetes Insipidus (DI) is a very complex and rare disease. The word “Diabetes Insipidus” is a combination of two words “Diabetes” and “Insipidus”. Diabetes is a word of Greek origin which means “siphon” and Insipidus is a word of Latin origin which means “without taste”. 1 DI is actually inability of body to conserve water due to pathophysiology of production of antidiuretic hormone (ADH) and its action. ADH is produced by the neurons of supraoptic and paraventricular nuclei located in the hypothalamus. After the production ADH streamlines down along the hypothalmo-hypophyseal tract and is stored in posterior pituitary, which on proper stimulus from osmoreceptors, is released from its storage location. Polydipsia, polyuria, hypernatremia, dehydration and severe thirst are most common manifestations of DI. The incidence of DI in general population is about 3:100, 000 Epydemiology Diabetes insipidus is a rare disease with a nonunivocal reported prevalence of 1:25,000 . Less than 10% of diabetes insipidus can be attributed to hereditary forms In particular, X-linked NDI represents 90% of cases of congenital NDI and occurs with a frequency of 4–8 per 1 million male live births; autosomal accounts for approximately 10% of the remaining cases. No gender difference has been reported for the other forms Etiology Types of diabetes insipidus (DI) The Diabetes insipidus include following types10 1 Neurogenic diabetes insipidus 2 Nephrogenic diabetes insipidus 3 Gestational diabetes insipidus 4 Adipsic diabetes insipidus 5 Primary polydipsia 6 Dipsogenic diabetes insipidus 7 Psychogenic diabetes insipidus Pathophysiology Two very different mechanisms can cause diabetes insipidus (FIGURE 1): Inadequate release of antidiuretic hormone (ADH, also called vasopressin) from the hypothalamus (central diabetes insipidus) and Inadequate response of the kidney to ADH (nephrogenic diabetes insipidus) Amgad N Makaryus et al (2006) Diabetes insipidus: Diagnosis and treatment of a complex disease volume 73 : 65-71. Spada A., Mantovani G., et al (2005) Pathogenesis of Prolactinomas. Pituitaary 8: 7 – 15. Clinical Manifestations Symptoms can include polyuria, excessive thirst, and polydipsia, The patient wants to drinking everytime, especially cold water in large quantities. In the elderly with limited mobility to drink, other symptoms arise. Weakness, mental disorders, and seizures can occur in the elderly. 24 hours of urine output > 50 mL / kg / day and urine osmolality less than 300 mosmol / kg. Physical Examination Enlarged bladder, back pain, or pain radiating to the genital area Anemia is found if the cause is malignancy or chronic renal failure. Urinary incontinence due to bladder damage due to prolonged overdistention Diagnosis of Diabetes Insipidus Water deprivation Test Desmopressin (DDAVP) Test Radiological finding can be hydronephrosis on IVP examination or CT scan. MRI to examine the hypothalamus, pituitary gland, and surrounding tissue may be necessary to determine the cause. Treatment In mild cases, it can be treated with adequate water intake. Aggravate factors (such as glucocorticoids) are avoided. If water intake is insufficient and hypernatremia develops, immediately give hypoosmolar intravenous fluids. Avoid giving intravenous sterile fluids without dextrose as they cause hemolysis. To avoid hyperglycemia, fluid overload, and rapid correction of hypernatremia, fluid replacement is given at a maximum dose of 500-750 mL / hour. Treatment
DI pituitary can be treated with desmopressin
(DDAVP) subcutaneously (1-2 μg once or twice per day), through a nasal spray (10-20 μg two or three times daily), or orally (100–400 μg two or three. times a day Nephrogenic DI may be use thiazide and / or amiloride diuretics combined with a low-sodium diet, or with prostaglandin synthesis inhibitors (eg, indomethacin). Prognosis In general, diabetes insipidus rarely causes death. Central diabetes insipidus due to surgery will usually go into remission after a few days / weeks, but structural damage to the infundibulum can result in permanent diabetes insipidus. Drug-induced nephrogenic diabetes insipidus may remit after drug withdrawal, but in some cases chronic drug use can lead to permanent diabetes insipidus. THANK YOU