Internal and emergency medicine
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Review Historical Article
From sadness to stiffness: the spleen's progress.
The spleen is a lymphoid organ that has been poorly studied compared to other solid organs, probably because it has been considered a useless and unnecessary part of the body. For many centuries it has been considered a mysterious organ with uncertain functions. The first descriptions of the spleen date back to ancient ages. ⋯ The study of hyposplenic states, which occur much more commonly than originally thought in many immune-mediated disorders, has rapidly increased after the validation of a simple method for assessing spleen function, namely pitted red cell count. In recent years, spleen morphology, in particular spleen stiffness, has been proposed as a marker of portal hypertension. In this paper, we retrace the fundamental steps of the discovery of the functions of the spleen.
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Behçet syndrome (BS) is a multi-systemic complex disorder with unknown etiology and a unique geographic distribution. It could not be possible to include it into specific classification schemes and it is certainly not a uniform disease. Several cluster and association studies revealed that it has been composed of multiple phenotypes ascribing the principal problem such as skin-mucosa, joint, eye, vascular, neurological and gastrointestinal involvement. ⋯ In this setting, BS resembles rather a construction made of several dynamic and interactive LEGO pieces of different shapes and colors. These pieces presenting phenotypes with their own disease mechanism have presumably different genetic determinants. The analysis of phenotyping could help us to identify this disorder and hence could contribute to find better ways of treatment.
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Idiopathic systemic capillary leak syndrome (ISCLS) presents with recurrent potentially life-threatening episodes of hypovolemic shock associated with severe hemoconcentration and hypoproteinemia. Timely recognition is of paramount importance because ISCLS, despite resembling other kinds of hypovolemic shock, requires a peculiar approach, to prevent life-threatening iatrogenic damage. Due to the rarity of this condition with only scattered cases described worldwide, evidence-based recommendations are still lacking. ⋯ The post-acute (recovery) phase may last from 48 h to 1 week. Monitor for cardiac overload to prevent cardiac failure; in case of persistent renal failure, hemodialysis may be necessary; consider albumin infusion. Complications listed by frequency in our patients were acute renal failure, compartment syndrome and neuropathy, rhabdomyolysis, myocardial edema, pericardial-pleural-abdominal effusion, cerebral involvement, acute pulmonary edema and deep vein thrombosis.