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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.
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 ...
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]
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 ...
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.
Plotz and Oldstone presented evidence that autoimmunity can arise as a result of a cross-reaction between the idiotype on an antiviral antibody and a host cell receptor for the virus in question. In this case, the host-cell receptor is envisioned as an internal image of the virus, and the anti-idiotype antibodies can react with the host cells.
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.
Dermatopolymyositis is a family of myositis disorders that includes polymyositis and dermatomyositis. As such, it includes both a distinctive skin rash and progressive muscular weakness. [2] It is a rare disease.