Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0016382 (flushing)
 6,387 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 24 year old white woman with a lifelong history of systemic mastocytosis and symptoms of diarrhea and flushing was demonstrated to have a normal gastric analysis and inconsistent steatorrhea. She responded well to oral cimetidine therapy for 11 months. A symptomatic recurrence was controlled with the addition of propantheline. Gastric secretory studies demonstrated cimetidine suppression of both basal acid and basal pepsin secretion, as well as maximal pentagastrin-stimulated acid secretion; suppression of stimulated pepsin secretion was minimal. The combination of cimetidine and propantheline markedly suppressed both peak acid and peak pepsin secretion in response to pentagastrin stimulation. These data support a dominant role of cholinergic mechanisms in the control of gastric pepsin secretion; additional data obtained with maintenance of constant intragastric pH are required for further clarification.
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PMID:The effect of cimetidine and propantheline on the symptoms of a patient with systemic mastocytosis. 677 32

Patients with mastocytosis at times experience spontaneous episodes of severe flushing and vasodilatory shock. Antihistamine therapy has not been found to prevent uniformly the recurrence of such attacks or reverse the hypotension during the acute episode when given intravenously. We previously found marked overproduction of prostaglandin D2 in two patients with mastocytosis. This suggested the possibility that prostaglandin D2 may be an important mediator in addition to histamine in mastocytosis. We have now generalized our initial findings and have found an increased production of prostaglandin D2 ranging from 1.5-to 32.6-fold in eight additional patients with severe episodes of flushing associated with mastocytosis. In one of those patients, the release of prostaglandin D2 was found to increase approximately 81-fold during an attack of flushing. Without exception, chronic combined therapy with antihistamines and the prostaglandin biosynthesis inhibitor, aspirin, has successfully prevented the recurrence of such severe attacks in these patients, further implicating prostaglandin D2 as an important mediator. The immediate treatment of an acute hypotensive episode, however, represents a different therapeutic situation. We recently observed a severe hypotensive episode in a hospitalized patient with mastocytosis being treated with an H1 receptor antagonist alone. The hypotension was found to be refractory to intravenous fluids, dopamine, and antihistamines, but was immediately reversed by intravenous epinephrine. The self-administration of subcutaneous epinephrine during episodes of flushing in other patients has also been found to ameliorate the flushing rapidly. The apparent unique effectiveness of epinephrine in this situation may derive from an ability of epinephrine to inhibit mast-cell mediator release as a result of beta-adrenergic receptor activation.
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PMID:Shock syndrome associated with mastocytosis: pharmacologic reversal of the acute episode and therapeutic prevention of recurrent attacks. 713 41

Urticaria pigmentosa is a usually a benign and asymptomatic cutaneous variant of mastocytosis, which is a relatively rare disorder characterized by abnormal aggregates of mast cells in the dermis. These aggregations, if present, can abruptly release vasoactive mediators such as histamine, heparin, and prostaglandins under some physical stresses and may produce flushing, hypotension, syncope, shock, etc. One of the interesting aspects to an anesthesiologist is the effect of drugs administered perioperatively on mast cell degranulation. We report an infant case of urticaria pigmentosa and discuss the anesthetic significance especially of mastocytosis during anesthesia and surgery.
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PMID:[Anesthetic management of a patient with urticaria pigmentosa]. 773 6

Flushing disorders are associated with sweating (wet flushes) or without sweating (dry flushes). When associated with systemic symptoms and histaminuria, flushing reactions may easily be confused with mastocytosis or pheochromocytoma. We report a case of a woman with significant transient histaminuria that could be dissociated from episodes of thermal wet flushing. Treatment with clonidine led to a decrease in both frequency and intensity of the flushing reactions.
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PMID:Parmesan cheese and vegetable-induced histaminuria in a thermal flusher. 781 39

A 41-year-old man was admitted in circulatory shock of unknown aetiology (systolic pressure 50 mm Hg) and marked reddening of the upper part of the body as well as maculopapular rash over the whole body. After 1500 ml of colloidal solution had been infused the blood pressure rose to a level at which the patient's condition was no longer at risk. He reported having had a similar attack of flushing and circulatory collapse during the last few years, each time after drinking 3-41 of beer. Laboratory tests showed thromboplastin time 56%, partial thromboplastin time 130 s and thrombin time > 180 s. Three hours after admission the coagulation times had further deteriorated, but had become normal within 20 hours. After rest and after a provocation (hot bath) the serum concentrations were: heparin 0.21 U/ml and 0.85 U/ml; histamine 1.9 micrograms/ml and 2.3 micrograms/ml; serotonin 23 micrograms/ml and 38 micrograms/ml. Histological examination of an iliac crest bone marrow biopsy revealed dense collections of mast cells, as seen in systemic mastocytosis. A skin biopsy was diagnostic of urticaria pigmentosa.
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PMID:[Shock and coagulation disorders in systemic mastocytosis]. 792 7

Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of mastocytosis. It appears most frequently in adults and only occasionally will affect young children or infants. In this disease, multiple brownish-red confluent macules and telangiectasias develop, primarily on the trunk. Pruritus frequently occurs, and may be mild to severe. Most patients have only skin involvement; however, involvement may be systemic as well. Clinical signs and symptoms of systemic mastocytosis are varied and depend on which internal organs are affected. Classic symptoms--such as episodic flushing, gastrointestinal complaints, heart palpitations, and syncope--may be confused with those of other diseases, most notably the carcinoid syndrome. A simple workup can help to differentiate between these two conditions. The authors describe a 48-year old woman who was seen with cutaneous features of TMEP and with multiple symptoms suggesting systemic mastocytosis. They discuss the clinical features, diagnostic workup, and therapeutic options in the management of this relatively rare condition.
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PMID:Telangiectasia macularis eruptiva perstans. 820 Aug 29

We describe the clinicopathologic features of 10 patients with recurrent unexplained flushing. These patients were referred to the National Institutes of Health with a diagnosis of mastocytosis or idiopathic anaphylaxis. Both diagnoses were eliminated after evaluation. Patients reported attacks of flushing lasting 15 minutes to 2 days and associated with such symptoms as anxiety, chest tightness, paresthesia, slurred speech, weakness, and pruritus. Abdominal pain was a constant feature, often associated with cramping and an increase in stool frequency. Attacks witnessed by physicians consisted of an exaggerated blush response of the face and upper part of the chest, and were sometimes associated with tachycardia, mild hypertension, and tachypnea. Hives, angioedema, wheezing, and hypotension were not observed. Routine laboratory studies and 5-hydroxyindoleacetic acid, vanillylmandelic acid, and plasma histamine levels were normal. Plasma histamine levels did not elevate during attacks. When performed, results of bone marrow examinations, skin biopsies, and bone scans were normal. Psychiatric examinations frequently revealed somatization disorders. Patients had often been prescribed a wide variety of medications including antihistamines, nonsteroidal anti-inflammatory drugs, and steroids, with little or no benefit. Despite the benign nature of the clinical and laboratory findings, patients had undergone repeated, often invasive, examinations for several years. Whether such patients have a prominent flush response exaggerated through a somatization disorder or a relatively benign flushing disorder associated with putative mediator release remains to be determined. Recognition of this category of patients with unexplained flushing will avoid subjecting such patients to unwarranted repeated examinations, procedures, and inappropriate therapy.
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PMID:A clinicopathologic study of ten patients with recurrent unexplained flushing. 830 82

We report a case of diffuse erythrodermic cutaneous mastocytosis with bone marrow infiltration. An 11-month-old female patient was referred to our hospital for intermittent flushing, fever, intense itching, erythematous rash and bullous lesions. Cutaneous biopsy demonstrated diffuse cutaneous mastocytosis. The bone marrow aspirate revealed mast cell infiltration. Ketotifen treatment was very effective.
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PMID:Diffuse erythrodermic cutaneous mastocytosis with bone marrow infiltration. 835 1

A 24-year-old man had a mediastinal embryonal carcinoma containing yolk sac foci. Combination chemotherapy with cisplatin, bleomycin, etoposide, and vinblastine was given, and the residual mass was then resected. Histology showed only necrotic cells. No other treatment was given. Two years later the patient presented with episodes of flushing and syncopes related to a systemicmastocytosis. Bone marrow examination showed a diffuse infiltration with large, atypical mast cells often with multilobulated nuclei. The patient suffered several episodes of cardiovascular collapse and died during one of these episodes, 8 months after the diagnosis of systemic mastocytosis and 40 months after the diagnosis of mediastinal tumor. Autopsy findings included the absence of mediastinal tumor and a diffuse liver and spleen mast cell infiltration. This was the second case with the similar clinicopathologic picture of two rare diseases being associated. This fact supports the hypothesis of a distinct entity, part of the mediastinal germ cell tumor/hematologic malignancy syndrome. The hypothesis of a cytokine secretion induced by mediastinal germ cell tumor supporting mast cell proliferation may be considered.
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PMID:Systemic mastocytosis following mediastinal germ cell tumor: an association confirmed. 838 Feb 74

Mastocytosis is a rare disease of mast-cell proliferation with involvement of the reticuloendothelial systems including skin, bone, gastrointestinal tract, liver, lungs, spleen, and lymph nodes. Systemic mastocytosis is characterized by a combination of symptoms that relate to the mast cells' release of vasoactive substances, such as histamine. These symptoms include urticaria pigmentosa, flushing, syncope with hypotension, headaches, nausea, vomiting, diarrhea, and occasional bronchospasm. The diagnosis of mastocytosis is typically based on the presence of the characteristic extraosseus manifestations. A well recognized roentgenographic feature seen in 70-75% of patients with mastocytosis is diffuse osteolysis and osteosclerosis, affecting primarily the axial skeleton and the ends of the long bones. Rarely, the bony involvement consists of generalized osteoporosis, which may lead to pathologic fracture, or solitary lesions (mastocytomas) which may cause symptoms of localized pain. Four patients with previously diagnosed systemic mastocytosis had unusual skeletal lesions. Clinical and laboratory evaluation of these patients eventually led to the correct diagnosis of systemic mastocytosis. We report these four cases to emphasize the need for thorough evaluation of unusual musculoskeletal findings in association with extraosseus symptoms that are characteristic of mastocytosis. Knowledge of a wide differential diagnosis of unusual skeletal lesions should include systemic mastosytosis.
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PMID:Mastocytosis presenting as a skeletal disorder. 912 84


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