Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0030552 (paresis)
 5,831 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Porphyrias are metabolic disorders related to heme biosynthesis pathway enzyme dysfunctions. The heme pathway is fundamental for the formation of a number of molecules, and such defects cause noxious precursors (porphyrins) to build up. Porphyrias are heterogeneously manifested by symptoms that can either be neurovisceral, cutaneous, or both, usually during outburst episodes called porphyric crises. This article presents a literature review and reports on a case of porphyric crisis initially diagnosed as acute abdomen and treated with an inconclusive exploratory laparotomy During the postoperative period, the patient progressed with tetraparesis, tetraplegia and respiratory distress, suggesting Guillain-Barre syndrome, which was precluded after cerebrospinal fluid analysis revealed no albumin-cytological dissociation. The patient was admitted to the intensive care unit due to her neurological disorders, which required ventilation support. After admission, she progressed with choluria and seizures. A porphyric crisis was suspected and confirmed upon a 24 hour urine porphyrins test. Supportive therapy was initiated, but due to unavailability in our hospital, heme derivatives were not given. The patient progressed with nosocomial infection, organ dysfunctions and eventually died. Porphyria should be considered as a differential diagnosis in acute abdomen cases of unknown origin and associated with neurological disorders such as paresis, hydroelectrolytic and psychiatric disorders, especially in patients with triggering factors, with a history of recurring crises and a family history of porphyria.
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PMID:Acute intermittent porphyria, an important and rare differential diagnosis of acute abdomen: case report and literature review. 2394 67

Nosocomial infections are a common occurrence in patients following traumatic brain injury (TBI) and are associated with an increased risk of mortality, longer length of hospital stay, and poor neurological outcome. Systemic immune suppression arising as a direct result of injury to the central nervous system (CNS) is considered to be primarily responsible for this increased incidence of infection, a view strengthened by recent studies that have reported novel changes in the composition and function of the innate and adaptive arms of the immune system post-TBI. However, our knowledge of the mechanisms that underlie TBI-induced immune suppression is equivocal at best. Here, after summarizing our current understanding of the impact of TBI on peripheral immunity and discussing CNS-mediated regulation of immune function, we propose roles for a series of novel mechanisms in driving the immune suppression that is observed post-TBI. These mechanisms, which have never been considered before in the context of TBI-induced immune paresis, include the CNS-driven emergence into the circulation of myeloid-derived suppressor cells and suppressive neutrophil subsets, and the release from injured tissue of nuclear and mitochondria-derived damage associated molecular patterns. Moreover, in an effort to further our understanding of the mechanisms that underlie TBI-induced changes in immunity, we pose throughout the review a series of questions, which if answered would address a number of key issues, such as establishing whether manipulating peripheral immune function has potential as a future therapeutic strategy by which to treat and/or prevent infections in the hospitalized TBI patient.
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PMID:Traumatic Brain Injury and Peripheral Immune Suppression: Primer and Prospectus. 2659 96