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Multiple organ dysfunction syndrome (MODS) is altered organ function in an acutely ill patient requiring immediate medical intervention. [1]There are different stages of organ dysfunction for certain different organs, both in acute and in chronic onset, whether or not there are one or more organs affected.
The word porphyria originated from the Greek word porphura. Porphura actually means "purple pigment", which, in suggestion, the color that the body fluid changes when a person has Gunther's disease. [3] It is a rare, autosomal recessive [4] metabolic disorder affecting heme, caused by deficiency of the enzyme uroporphyrinogen cosynthetase. [5]
Anti-MOG antibodies have been described in some patients with NMOSD [15] [16] who were negative for the aquaporin 4 (AQP-4) antibody. However, most NMOSD is an astrocytopathy, specifically an AQP4 antibody-associated disease, whereas MOG antibody-associated disease is an oligodendrocytopathy, suggesting that these are two separate pathologic entities. [2]
Historically, acute MS was a fatal disease, with death occurring within a year of onset, often secondary to extensive brainstem demyelination. Treatments include plasma exchange and/or high-dose glucocorticoids (e.g., 1 g/day of methylprednisolone for 3–5 days).
With AMML being difficult to fully treat, the five-year survival rate is about 38-72% which typically decrease to 35-60% if there's no bone marrow transplantation performed. [11] Generally older patients over 60 have a poor outlook due to prior health status before the diagnosis and the aggressive chemotherapy regimen used. [ 13 ]
The prevalence of LAM is estimated using data from registries and patient groups and is between 3.4 and 7.8/million women. The number of new cases each year is between 0.23 and 0.31/million women/year in the US, UK and Switzerland.
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The syndrome was first recognised by Saap and colleagues who recognised the spectrum of symptoms from a set of seven patients. [4] In this initial description the syndrome is named CLOVE syndrome. It is believed that the first description of a case of CLOVES syndrome was written by Hermann Friedberg , a German physician, in 1867.