Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0031511 (pheochromocytoma)
 14,622 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pheochromocytoma was the cause of arterial hypertension observed in 0.9% of children treated in 1982-1989. Out of clinical features the most characteristic was sustained hypertension often complicated by the accelerated phase of malignant hypertension and encephalopathy. Sustained tachycardia was also found in all patients. Increased urinary excretion of catecholamines and its metabolites confirmed the diagnosis in all cases. The most sensitive and specific methods for tumor diagnosis were ultrasonography and computer tomography of the adrenals while scintigraphy with meta-iodobenzylguanidine+ labelled with iodine-131 radioisotope gave a high percentage of false negative results.
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PMID:[Difficulties in the diagnosis of pheochromocytoma in children]. 148 33

Surgically confirmed pheochromocytoma was the cause of arterial hypertension in 6 out of 668 (0.8%) children with significant hypertension admitted to Child Health Centre in Warsaw. Among clinical features most characteristic was sustained hypertension observed in all patients, often complicated by the accelerated phase of malignant hypertension and encephalopathy. Sustained tachycardia was also found in all patients. Elevated sedimentation rate and electrocardiographic changes were observed in each child while other abnormal laboratory findings such as hyperglycemia, etc. occurred at similar rate as in adults. Increased urinary excretion of catecholamines and their metabolites confirmed the diagnosis. In our study the most sensitive methods for tumor localization were ultrasonography and computed tomography of the adrenals while scintigraphy with iodo-131-metabenzylguanidine gave a high percentage of false negative results. Clinical presentation of pheochromocytomas in children is different than in adults and all pediatric patients with severe hypertension should be screened for this disease.
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PMID:Pheochromocytoma in children: difficulties in diagnosis and localization. 219 28

Three of 27 patients treated for pheochromocytoma between 1974 and 1987 presented with pheochromocytoma multisystem crisis (PMC). This unusual presentation consists of multiple organ system failure, temperature often greater than 40 degrees C, encephalopathy, and hypertension and/or hypotension. Although urgent medical therapy achieved blood pressure control in all three patients, the other manifestations of PMC progressed rapidly in spite of alpha and even beta blockade. The first patient died during attempts to localize a septic focus. The other two patients underwent urgent adrenalectomy and had postoperative improvement in their multiple organ system failure. All three tumors were large and produced markedly elevated levels of epinephrine. In conclusion (1) PMC is an unusual presentation of pheochromocytoma; (2) its manifestations include multiple organ system failure, high fever, encephalopathy, and vascular lability; (3) it may result from increased epinephrine secretion; and (4) successful treatment of PMC demands prompt diagnosis, vigorous medical preparation, and emergency tumor removal if the patient's condition continues to deteriorate.
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PMID:Pheochromocytoma multisystem crisis. A surgical emergency. 289 26

Of 26 patients with pheochromocytoma treated between 1974 and 1986, two presented with pheochromocytoma crisis. This unusual presentation consists of hyper- and/or hypotension, high fevers (greater than 40 degrees C), encephalopathy, and multiple organ system failure. Both patients had large tumors associated with markedly elevated levels of epinephrine. Although hypertension was adequately controlled in both patients with phenoxybenzamine, phentolamine (1 patient) and nitroprusside, both patients deteriorated rapidly. The first patient expired during attempts to identify a source of sepsis. None was found at autopsy. The second patient underwent urgent adrenalectomy which reversed the multiple organ system failure and resulted in patient survival. We conclude from review of these patients and three others in the literature that (a) crisis is an unusual presentation of pheochromocytoma; (b) its manifestations include vascular lability, high fever, encephalopathy and multiple organ system failure; (c) it may be the result of increased epinephrine secretion; (d) successful treatment of pheochromocytoma crisis demands prompt diagnosis, vigorous medical therapy and emergent tumor removal if the patient continues to deteriorate.
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PMID:Pheochromocytoma crisis. 341 97

Two teenage boys were treated at separate pediatric institutions for four separate pheochromocytomas over the last 15 years. The first operation in each boy was performed between 9 and 11 years of age, after they presented with severe hypertensive encephalopathy. One boy had separate adrenal tumors excised in 1968, and 3 and 11 years later, two more separate new left adrenal pheochromocytomas were again resected. They were not recurrent left adrenal tumors from residual pheochromocytoma-secreting tissue, because no visible tumor tissue was left behind at the completion of each previous operation, and he was clinically well for years between each tumor resection. The pathology was benign pheochromocytoma. He remains well since the last operation in 1979 but has a residual hemiplegia from the first tumor. The family history is negative. The second boy was first operated on in 1976 at age 11 years when two benign pheochromocytomas were removed from around the right renal artery and the left para-aortic area. He remained well for 6 years when he became hypertensive again (this time without encephalopathy), and had a right chest paravertebral pheochromocytoma removed and several weeks later a left adrenal tumor was also removed. He remains well and followed closely. His family history is negative.
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PMID:Two boys with four pheochromocytomas each. 377 11

Hypertension in childhood is diagnosed according to age-adjusted values for each sex. It is more commonly diagnosed now than it was 2 decades ago because blood pressure is more frequently measured in children today. Surgically curable forms of hypertension are not common but usually present as moderate or severe hypertension (diastolic blood pressure greater than or equal to 110 mm Hg). Although many patients in this group are asymptomatic, some present with Bell's palsy, enlarged hearts, heart failure, encephalopathy, or stroke. Newer imaging techniques have proved particularly useful for localizing tumors, such as pheochromocytoma. Many antihypertensive drugs are now available, and therapy should be tailored to the patient's needs, with as few adverse or side effects as possible.
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PMID:Evaluation and management of childhood hypertension. 407 63

Pheochromocytoma usually presents with nonspecific symptoms such as headache, palpitation, chest pain and hypertension. We present a 48-year-old woman hospitalized due to a pheochromocytoma multi-organ crisis (PMC). It consists of a tetrad of symptoms: multi-organ failure, hyperthermia, encephalopathy and unstable blood pressure. Distinguishing PMC from septic shock may be difficult, and requires needs a high clinical index of suspicion.
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PMID:[Pheochromocytoma multi-organ crisis: nonseptic hyperthermia and shock]. 988 52

The pheochromocytomas are an important cause of secondary hypertension. Although pheochromocytoma susceptibility may be associated with germline mutations in the tumor-suppressor genes VHL and NF1 and in the proto-oncogene RET, the genetic basis for most cases of nonsyndromic familial pheochromocytoma is unknown. Recently, pheochromocytoma susceptibility has been associated with germline SDHD mutations. Germline SDHD mutations were originally described in hereditary paraganglioma, a dominantly inherited disorder characterized by vascular tumors in the head and the neck, most frequently at the carotid bifurcation. The gene products of two components of succinate dehydrogenase, SDHC and SDHD, anchor the gene products of two other components, SDHA and SDHB, which form the catalytic core, to the inner-mitochondrial membrane. Although mutations in SDHC and in SDHD may cause hereditary paraganglioma, germline SDHA mutations are associated with juvenile encephalopathy, and the phenotypic consequences of SDHB mutations have not been defined. To investigate the genetic causes of pheochromocytoma, we analyzed SDHB and SDHC, in familial and in sporadic cases. Inactivating SDHB mutations were detected in two of the five kindreds with familial pheochromocytoma, two of the three kindreds with pheochromocytoma and paraganglioma susceptibility, and 1 of the 24 cases of sporadic pheochromocytoma. These findings extend the link between mitochondrial dysfunction and tumorigenesis and suggest that germline SDHB mutations are an important cause of pheochromocytoma susceptibility.
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PMID:Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma. 1140 20

Neuroserpin is an axonally secreted serine protease inhibitor expressed in the nervous system that protects neurons from ischemia-induced apoptosis. Mutant neuroserpin forms have been found polymerized in inclusion bodies in a familial autosomal encephalopathy causing dementia, or associated with epilepsy. Regulation of neuroserpin expression is mostly unknown. Here we demonstrate that neuroserpin mRNA and the RNA-binding protein HuD are co-expressed in the rat central nervous system, and that HuD binds neuroserpin mRNA in vitro with high affinity. Gel-shift, supershift and T1 RNase assays revealed three HuD-binding sequences in the 3'-untranslated region (3'-UTR) of neuroserpin mRNA. They are AU-rich and 20, 51 and 19 nt in length. HuD binding to neuroserpin mRNA was also demonstrated in extracts of PC12 pheochromocytoma cells. Additionally, ectopic expression of increasing amounts of HuD in these cells results in the accumulation of neuroserpin 3'-UTR mRNA. Furthermore, stably transfected PC12 cells over-expressing HuD contain increased levels of both neuroserpin mRNAs (3.0 and 1.6 kb) and protein. Our results indicate that HuD stabilizes neuroserpin mRNA by binding to specific AU-rich sequences in its 3'-UTR, which prolongs the mRNA lifetime and increases protein level.
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PMID:HuD binds to three AU-rich sequences in the 3'-UTR of neuroserpin mRNA and promotes the accumulation of neuroserpin mRNA and protein. 1200 Aug 40

We describe neurological complications which manifested after a patient with unsuspected pheochromocytoma was administered dihydroergotamine. Following administration of dihydroergotamine, the patient developed Balint syndrome, with the appearance of symmetric, bilateral occipital signal change on magnetic resonance imaging suggestive of posterior reversible encephalopathy syndrome.
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PMID:PRES following administration of DHE in a patient with unsuspected pheochromocytoma. 1854 64


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