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      Multiple organ failure and coma as initial presentation of pheochromocytoma in a patient with multiple endocrine neoplasia (MEN) type II A

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          Abstract

          The unusual case of a 65-year-old woman with intermittent hypotension, fever, pulmonary edema and coma as initial presentation of pheochromocytoma is reported. The patient developed respiratory, cardiac and renal failure, disseminated intravascular coagulation and liver dysfunction. She, had to be defibrillated on multiple occasions, occurring in periods of severe hypertension. After successful surgical removal of a pheochromocytoma a thyroid medullary carcinoma was detected. Several members of the patients family had presented with multiple endocrine neoplasia (MEN II).

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          Focal myocarditis associated with pheochromocytoma.

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            Neurogenic pulmonary edema.

            A variety of central nervous system (CNS) insults may be complicated by the acute development of pulmonary edema. This occurrence has been termed neurogenic pulmonary edema (NPE), and experimental models have clearly shown that CNS insults may cause pulmonary edema. Unfortunately, the pathophysiologic aspects of this response are not clearly understood. Basing an approach to the development of pulmonary edema on Starling's equation leads to the conclusion that NPE is caused by changes in pulmonary endothelial permeability and/or microvascular pressures. It was previously suggested (the "blast theory") that CNS insults caused acute systemic arterial and pulmonary venous spasm and increased venous return, which would result in a severe pulmonary vascular hydrostatic insult and a secondary permeability defect. Although such hydrostatic derangements may explain certain cases of NPE, recent clinical and experimental studies have indicated that CNS disorders may cause a permeability defect without a vascular insult. The mediating factor for a permeability defect is not clear. The implications of these findings are that NPE may be caused by either permeability abnormalities or hydrostatic insults, may present clinically in a variety of ways, and may require different approaches to treatment. Our understanding of the CNS sites that might mediate NPE is not sophisticated enough, at present, to define the neural mechanisms involved in the pathogenesis of NPE.
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              Diagnosis and Management of Pheochromocytoma

              Pheochromocytomas arise from chromaffin tissue, usually in the adrenal medulla, and are a cause for curable hypertension. Nearly all patients with this tumor are symptomatic, the most common symptoms being headache, palpitations and inappropriate perspiration. Diagnosis is confirmed by finding high levels of plasma catecholamines or increased excretion of catecholamine metabolites (metanephrines, vanillymandelic acid) in the urine. Localization of tumors(s) is important for the surgeon and is accomplished by CT scan, 131 I-metaiodobenzylguanidine scintiscans or abdominal aortography. Treatment is surgical extirpation by an experienced team after depleted plasma volume has been replenished. Ten percent of tumors are malignant, 10% are bilateral in the adrenal medullae and 10% are extra-adrenal.
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                Author and article information

                Journal
                Intensive Care Med
                Intensive Care Med
                Intensive Care Medicine
                Springer-Verlag (Berlin/Heidelberg )
                0342-4642
                1432-1238
                1993
                : 19
                : 4
                : 235-238
                Affiliations
                GRID grid.6612.3, ISNI 0000000419370642, Medical Department, Kantonsspital Liestal, , University of Basel, ; CH-4410 Liestal, Switzerland
                Article
                BF01694777
                10.1007/BF01694777
                7095150
                8103532
                c3c2e013-5bf5-4a5f-b811-e9156c7bb06c
                © Springer-Verlag 1993

                This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.

                History
                : 25 March 1992
                : 1 September 1992
                Categories
                Brief Reports
                Custom metadata
                © Springer-Verlag 1993

                Emergency medicine & Trauma
                pheochromocytoma crisis,multiple endocrine neoplasia type ii a,multiple organ failure,fever,pulmonary edema,coma

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