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Wolfram syndrome, also called DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness), is a rare autosomal-recessive genetic disorder that causes childhood-onset diabetes mellitus, optic atrophy, and deafness as well as various other possible disorders including neurodegeneration.
Central diabetes insipidus, recently renamed arginine vasopressin deficiency (AVP-D), [1] is a form of diabetes insipidus that is due to a lack of vasopressin (ADH) production in the brain. Vasopressin acts to increase the volume of blood (intravascularly), and decrease the volume of urine produced.
Persons with nephrogenic diabetes insipidus must consume enough fluids to equal the amount of urine produced. Any underlying cause such as high blood calcium must be corrected to treat nephrogenic diabetes insipidus. The first line of treatment is hydrochlorothiazide and amiloride. [10] Patients may also consider a low-salt and low-protein diet.
Despite the name, diabetes insipidus is unrelated to diabetes mellitus and the conditions have a distinct mechanism, though both can result in the production of large amounts of urine. [1] Treatment involves drinking sufficient fluids to prevent dehydration. [1] Other treatments depend on the type. [1]
A fasting blood sugar level of ≥ 7.0 mmol / L (126 mg/dL) is used in the general diagnosis of diabetes. [17] There are no clear guidelines for the diagnosis of LADA, but the criteria often used are that the patient should develop the disease in adulthood, not need insulin treatment for the first 6 months after diagnosis and have autoantibodies in the blood.
As of 2011 more than 36 genes have been found that contribute to the risk of type 2 diabetes. [2] All of these genes together still only account for 10% of the total genetic component of the disease. [2] There are a number of rare cases of diabetes that arise due to an abnormality in a single gene (known as monogenic forms of diabetes). [1]
ICD-10 is the 10th revision of the International Classification of Diseases (ICD), a medical classification list by the World Health Organization (WHO). It contains codes for diseases, signs and symptoms, abnormal findings, complaints, social circumstances, and external causes of injury or diseases. [1]
In the absence of diabetes mellitus, the most common causes are the decreased secretion of aldosterone due to adrenal cortical tumor, primary polydipsia (excessive fluid drinking), central diabetes insipidus, and nephrogenic diabetes insipidus. [6] Polyuria may also be due to various chemical substances, such as diuretics, caffeine, and ethanol.