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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.
The number of new cases of diabetes insipidus each year is 3 in 100,000. [4] Central DI usually starts between the ages of 10 and 20 and occurs in males and females equally. [2] Nephrogenic DI can begin at any age. [3] The term "diabetes" is derived from the Greek word meaning siphon. [6]
A fluid or water deprivation test is a medical test [1] which can be used to determine whether the patient has diabetes insipidus as opposed to other causes of polydipsia (a condition of excessive thirst that causes an excessive intake of water). The patient is required, for a prolonged period, to forgo intake of water completely, to determine ...
Polymyositis and the associated inflammatory myopathies have an associated increased risk of cancer. [3] The features they found associated with an increased risk of cancer were older age, age greater than 45, male sex, difficulty swallowing, death of skin cells, cutaneous vasculitis, rapid onset of myositis (<4 weeks), elevated creatine kinase, higher erythrocyte sedimentation rate and higher ...
Pyomyositis is most common in tropical areas but can also occur in temperate zones. Pyomyositis can be classified as primary or secondary. Primary pyomyositis is a skeletal muscle infection arising from hematogenous infection, whereas secondary pyomyositis arises from localized penetrating trauma or contiguous spread to the muscle. [1]
Craniopharyngioma, germinoma, pinealoma, glioma, and meningioma can all cause central diabetes insipidus. [10] Trauma - Central diabetes insipidus can be caused by head trauma that affects the posterior pituitary and hypothalamus. [16] Vascular disorders - Vascular disorders such as subarachnoid hemorrhage, intracranial hemorrhage, and Sheehan ...
Dr Searle added: “These drugs are a huge breakthrough in this type of cancer, allowing patients without standard treatment options to achieve remission, in many cases for months or years.
Local steroid cream is applied to skin lesions. Endocrine deficiency often require lifelong supplement e.g. desmopressin for diabetes insipidus which can be applied as nasal drop. Chemotherapeutic agents such as alkylating agents, antimetabolites, vinca alkaloids either singly or in combination can lead to complete remission in diffuse disease.