1/10. Overview of chronic diarrhea caused by functional neuroendocrine neoplasms.Eight different neoplastic disorders can cause chronic diarrhea attributable to humoral-mediated diarrhea. These include pancreatic endocrine tumor (PET) syndromes (gastrinomas, VIPomas, glucagonomas, somatostatinomas, PET's releasing calcitonin), carcinoid syndrome, medullary thyroid cancer, and systemic mastocytosis. Because these disorders are an uncommon cause of all chronic diarrheas (<1%), they are not often considered in the differential diagnosis, leading to a delay in diagnosis. This is problematic not only because all are treatable, but also because the neoplasm is frequently malignant. In this article, the characteristics and pathogenesis of the diarrhea, important clinical and diagnostic laboratory features, and treatment of each disorder are briefly reviewed, with an emphasis on recent insights.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
2/10. Diffuse cutaneous mastocytosis with bone marrow infiltration in a child: a case report.mastocytosis encompasses a range of disorders characterized by overproliferation and accumulation of tissue mast cells. Mast cell disease is most commonly seen in the skin, but the skeleton, gastrointestinal tract, bone marrow, and central nervous system may also be involved. We present a 10-year-old boy with diffuse cutaneous mastocytosis characterized by disseminated papular, nodular, and infiltrated leathery lesions. The patient presented with chronic diarrhea and malnutrition. Laboratory studies were normal except for an elevated urinary 1-methylhistamine level. The bone marrow aspirate showed a dense mast cell infiltrate confirming systemic involvement.- - - - - - - - - - ranking = 5keywords = mastocytosis (Clic here for more details about this article) |
3/10. Systemic mastocytosis treated with histamine H1 and H2 receptor antagonists.A case of systemic mastocytosis with skin characteristics of telangiectasia macularis eruptiva perstans is reported. Systemic involvement was confirmed as a combination of highly increased urinary excretion of methyl imidazole acetic acid (Melm AA) and increased amounts of mast cells in skin, liver and colon transversum. Treatment with cimetidine (H2 receptor antagonist) resulted in diarrhea, however, the addition of cyproheptadine (H1 receptor antagonist) was beneficial in amelioration of the cutaneous symptoms of mastocytosis. The excretion of Melm AA was unchanged during treatment. After 7 months of continuous cimetidine and cyproheptadine treatment no side effects were observed.- - - - - - - - - - ranking = 6keywords = mastocytosis (Clic here for more details about this article) |
4/10. Aggressive systemic mastocytosis mimicking sclerosing cholangitis.A 43 year-old woman presented with fever, abdominal pain, epato-splenomegaly, ascites, cholestasis, anemia, thrombocytopenia and previous diagnosis of sclerosing cholangitis based on liver biopsy and endoscopic retrograde cholangiopancreatography(ERCP). The bone marrow biopsy and the revision of liver biopsy using antitryptase stain diagnosed systemic mastocytosis. Because of the aggressive course of the disease the patient was treated with an acute myeloid leukaemia chemotherapy regimen without success.- - - - - - - - - - ranking = 5keywords = mastocytosis (Clic here for more details about this article) |
5/10. Idiopathic gastric acid hypersecretion presenting as a diarrheal disorder and mimicking both zollinger-ellison syndrome and Crohn's disease.Many gastric acid hypersecretory states (basal acid output of greater than 15.0 mEq/h) exist for which the etiology is known, such as zollinger-ellison syndrome, systemic mastocytosis, antral exclusion, antral predominant helicobacter pylori gastritis (antral G cell hyperplasia), chronic gastric outlet obstruction, short gut syndrome and basophilic leukemias. However, many hypersecretory patients have no identified etiology for their acid hypersecretion and are designated as idiopathic gastric acid hypersecretors with a basal acid output of greater than 10 mEq/h and a normal serum gastrin level. Because of the gastric acid hypersecretion these patients also commonly have an increased frequency of stools. Idiopathic gastric acid hypersecretion represents a known cause of gastric acid hypersecretion that is far more common than zollinger-ellison syndrome and it has a markedly different treatment regimen and natural history. We report a case of a patient with idiopathic gastric acid hypersecretion previously misdiagnosed as having Crohn's disease because of a presenting complaint of diarrhea and mimicking zollinger-ellison syndrome because her fasting serum gastrin level was elevated when incorrectly measured in the presence of antisecretory treatment.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
6/10. Systemic mastocytosis mimicking inflammatory bowel disease: A case report and discussion of gastrointestinal pathology in systemic mastocytosis.Gastrointestinal (GI) symptoms are present in up to 80% of patients with systemic mastocytosis (SM). GI symptoms include mainly abdominal pain, diarrhea, nausea, and vomiting. It is believed that most of the GI symptoms are due to the secondary effect of mast cell mediators on the GI tract. Direct involvement of the GI tract by neoplastic mast cell infiltration has not been well documented. We report a case of SM that initially mimicked inflammatory bowel disease based on clinical, radiographic, endoscopic, and histopathologic findings. On routine histologic sections of small bowel and colonic mucosal biopsies, there was expansion of the lamina propria by mononuclear inflammatory cells, foci of erosions with associated acute inflammation, and evidence of chronic mucosal injury with architectural distortion and gland foreshortening. Only on repeat biopsies and with ancillary tests for mast cells was a diagnosis of SM made, with extensive involvement of the GI tract. This is the first reported case of SM presenting as and mimicking inflammatory bowel disease. It is critical that clinicians and pathologists are aware that neoplastic mast cells in patients with SM can infiltrate the mucosa throughout the GI tract and that this infiltration can lead to symptoms and findings that can mimic inflammatory bowel disease.- - - - - - - - - - ranking = 9keywords = mastocytosis (Clic here for more details about this article) |
7/10. Effect of cimetidine on gastric hypersecretion and diarrhea in systemic mastocytosis.Two patients had systemic mastocytosis and histamine excess, marked gastric acid and pepsin hypersecretion, and diarrhea. cimetidine inhibited gastric acid secretion in both, but with only partial initial control of diarrhea. The other manifestations of mastocytosis were only partly controlled, even by combined H-1 and H-2 antagonist therapy. Basal and pentagastrin-stimulated acid and pepsin secretion before and after 5 and 14 months, respectively, of continuous and effective cimetidine therapy were essentially the same. Thus there was no evidence for an increase in the number of H-2 receptors during the long period of blockade or of involution of the parietal or peptic cell mass.- - - - - - - - - - ranking = 6keywords = mastocytosis (Clic here for more details about this article) |
8/10. Forty years of diarrhoea in a patient with urticaria pigmentosa.A patient with urticaria pigmentosa who gave a 40-year history of diarrhoea was found to have systemic mastocytosis with gut involvement. The radiological appearance of the gut in this disease, although not widely recognized, are specific and should be looked for carefully in patients with urticaria pigmentosa who complain of gastro-intestinal symptoms. Gastro-intestinal symptoms, due mainly to alterations in bowel motility or peptic ulceration, are said to occur in some 25-50% of cases of systemic mastocytosis (3, 6). These symptoms have usually been ascribed to generalized histamine release acting on the gut, although cases where mast cell infiltration of the bowel has occurred have also been reported (4, 5). In a review of the radiological features (2), increased gastric rugosity with or without evidence of peptic ulceration and nodular space-filling defects of the bowel mucosa were the most commonly found. Occasionally, diffuse thickening of the bowel wall was seen. It was concluded that these appearance were probably due to local release of vasoactive substances causing submucosal oedema following mast cell accumulation in the gut. Another result of such infiltration may be malabsorption (1).- - - - - - - - - - ranking = 2keywords = mastocytosis (Clic here for more details about this article) |
9/10. The effect of cimetidine and propantheline on the symptoms of a patient with systemic mastocytosis.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.- - - - - - - - - - ranking = 5keywords = mastocytosis (Clic here for more details about this article) |
10/10. Systemic mastocytosis with review of gastrointestinal manifestations.A 63-year-old man presented with fever, splenomegaly, steatorrhea, diarrhea, and weight loss. A tissue diagnosis of systemic mastocytosis was made. This case is unusual in that diarrhea and steatorrhea were present in the absence of skin lesions and because fever was a prominent symptom. Thus, systemic mastocytosis should be included in the differential diagnosis of intestinal malabsorption even when the skin shows no abnormalities. The gastrointestinal manifestations of systemic mastocytosis are reviewed.- - - - - - - - - - ranking = 7keywords = mastocytosis (Clic here for more details about this article) |
| | Next -> |