Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020639 (hypoproteinemia)
 1,134 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of Cronkhite-Canada syndrome is presented. The patient had alopecia, onychodystrophy and gastrointestinal polyposis, mainly in the stomach and duodenum, with transient diarrhea and hypoproteinemia. Marked atrophy and weakness of the shoulder girdle muscles due to myopathy were also present. In addition she had primary hypothyroidism. The outcome of the disease is usually fatal within months, but so far our patient is alive four years after the onset of symptoms. The pathological changes, pathophysiology, symptoms, course and treatment of this rare disorder of unknown etiology are discussed.
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PMID:A patient with Cronkhite-Canada syndrome, myxedema and muscle atrophy. 43 75

Two autopsy cases of Cronkhite-Canada syndrome were reported. The caused of hypoproteinemia, electrolyte imbalance and ectodermal changes were discussed with reference to previously reported cases. The mechanism of protein loss was probably due to outflow into the intestinal lumen of the mucous substance in the cystically dilated glands, directly and/or indirectly followed by loss of mucosal surface. Electrolyte imbalance probably developed from gastrointestinal loss as well as poor substitution. The ectodermal changes were probably not a subsequent part of the emaciation or hypoproteinemis, but an inherent part of this disease. Therapy, whether substitution or surgical procedure, should be selected in order to control the general condition of the patient.
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PMID:Two autopsy cases of diffuse gastrointestinal polyposis with ectodermal changes. Cronkhite-Canada syndrome. 121 85

An elderly man with non-familial gastrointestinal polyposis, malabsorption and progressive hypoproteinemia is reported. Associated alopecia, cutaneous hyperpigmentation and nail dystrophy with loss of nails were consistent with the diagnosis of Cronkhite-Canada syndrome. Hypothyroidism was present in this patient and the rare association of these two conditions is discussed.
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PMID:Cronkhite-Canada syndrome with hypothyroidism. 237 35

We report about a 76 years old patient with Cronkhite-Canada syndrome. The diagnosis has been found with the following clinical symptoms: diarrhea, anorexia, alopecia, and onychotrophia. Laboratory values: severe hypoproteinemia (total serum protein 4.3 g/dl, albumin 2.4 g/dl); endoscopical and radiological findings: a generalized polyposis which involved the whole intestine except the oesophagus. As far as we saw in our literature-overview of 55 patients with Cronkhite-Canada syndrome, this patient had for the first time a carcinoma of the urinary bladder and a Bricker operation 17 years before the onset of his disease. Further we remarked a lack in the resorption of the enterally administered thyroidal hormones. The progress was fatal despite a parenteral hyperalimentation and a treatment with antibiotics and glucocorticoids.
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PMID:[New observations in a case of Cronkhite-Canada syndrome]. 396 97

A 41-year-old man with Cronkhite-Canada syndrome presented with multiple juvenile polyps with hyperplastic and adenomatous changes throughout his stomach and entire colorectum. Dysgeusia was recognized and the degree of hypoproteinemia was remarkable. A barium enema study and colonofiberscopy also revealed an advanced cancer in the rectum. Chronic hepatitis B and membranous glomerulonephritis were also present. It was difficult to design a conservative protocol using steroids for the treatment of protein-loosing enteropathy because the patient was a hepatitis B virus carrier. As a result, a subtotal colectomy while preserving the cecum with cecorectal anastomosis was performed. Pathologically, the ulcerated rectal tumor was a moderately differentiated adenocarcinoma with invasion into the muscularis propria. Most polyps showed cystically dilated glands without dysplasia or edematous stroma with inflammatory cell infiltration. A few polyps were juvenile-type polyps with adenoma components. Although no remarkable improvement was observed in the hypoproteinemia postoperatively, an alpha1-antitrypsin clearance test showed a significant decrease in protein loss from the gastrointestinal tract, which was only about one third of the loss seen preoperatively. These findings lead us to conclude that when improvement using conservative treatment can be neither obtained nor is expected, then the use of surgery should be considered when treating patients with Cronkhite-Canada syndrome.
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PMID:Cronkhite-Canada syndrome associated with advanced rectal cancer treated by a subtotal colectomy: report of a case. 1142 6

We describe a case of Cronkhite-Canada syndrome associated with sigmoid colon cancer, and provide a literature review. A 77-year-old man was diagnosed with sigmoid colon cancer after presenting with hypoproteinemia, nail atrophy, loss of scalp hair, hyperpigmentation, and gastrointestinal polyposis. The findings were consistent with Cronkhite-Canada syndrome. The colon polyps were histologically serrated adenomas, whose crypts showed a saw-toothed growth pattern with dysplasia, or tubular adenoma. Cronkhite-Canada syndrome associated with colon cancer has been reported in 31 cases. The availability of histologic material permitted reexamination of 25 of these cases. Serrated adenoma of the polypoid lesions was retrospectively found in 10 (40%) of the 25 cases. By comparison, the incidence of serrated adenomas has been estimated to occur in about 1% of all general polyps. Taken together, it is suggested that Cronkhite-Canada syndrome associated with colorectal cancer frequently has polyps containing serrated adenoma lesions. In the case described here, microsatellite instability and overexpression of the p53 protein were found in the cancer lesion and serrated adenoma lesions, and none of the lesions showed a loss of heterozygosity of various genes or K-RAS mutations. Thus, genetic alterations between the serrated adenoma and the colorectal cancer was correlated in this case. These findings suggested the possibility of a serrated adenoma-carcinoma sequence in this case of Cronkhite-Canada syndrome.
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PMID:Cronkhite-Canada syndrome containing colon cancer and serrated adenoma lesions. 1594 6

A 67-year-old man with nausea, appetite loss, frequent diarrhea and severe weight loss presented with alopecia, skin hyperpigmentation and onychodystrophy. Laboratory investigations showed mild anemia, hypoproteinemia and hypoalbuminemia. Colonoscopy identified the numerous, hyperemic and sessile polyps with mucous exudation of various sizes throughout the colorectum. The ileocecal valve was substantially swollen. Magnified chromoendoscopy revealed sparsely distributed crypt openings with widening of the preicryptal space without destruction in the affected lesions. Upper gastrointestinal endoscopy revealed multiple small, reddish, and sessile polyps in the duodenum and Helicobacter pylori-associated gastritis. Histopathological examination of the colonic polyps revealed cystic dilatation and elongation of scattered glands with epithelial hyperplasia and stromal edema and inflammatory cell infiltrates. Thus, a diagnosis of Cronkhite-Canada syndrome was made. The patient was given clarithromycin, amoxicillin and lansoprazole, resulting in negative (13)C-urea breath tests. Three months later, his clinical symptoms and edema of the legs resolved with normalization of serum total protein and albumin levels and return to his previous body. The ectodermal abnormalities were resolved 8 months later. On repeat colonoscopic examinations, there was progressive remission of the duodenal and colorectal polyposis, leaving scattered pedunculated polyps in the transverse and ascending colon and on the almost normal-appearing ileocecal valve. At the follow-up magnifying endoscopic examination 8 months later, small round or round-oval pits were densely and regularly distributed.
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PMID:A case of Cronkhite-Canada syndrome: remission after treatment with anti-Helicobacter pylori regimen. 1894 92

There have been no reports of Cronkhite-Canada syndrome (CCS) associated gastric cancer resected with endoscopy because it is very difficult to identify small cancers that are candidates for endoscopic resection. We report a case of CCS with gastric cancer treated with endoscopic submucosal dissection, and we evaluate the molecular pathological analysis of malignant transformation in patients with CCS. A 74-year-old man had an advanced rectal cancer and gastrointestinal polyposis after presenting with hypoproteinemia, partial hair loss and atrophic nails as well as hyperpigmentation on the hands. He was diagnosed as having CCS. On upper endoscopy, a 7 mm discolored polyp with an irregular microvascular pattern revealed by magnified narrow-band imaging (NBI) was identified in gastric diffuse CCS polyposis. This lesion was treated with endoscopic submucosal dissection and diagnosed as a flat, elevated-type, mucosal well-differentiated tubular adenocarcinoma without lymphatic or venous infiltration, and with tumor-free margins. Microsatellite instability was detected in both the cancer and the surrounding CCS polyps. Mucin-histochemical analysis of the cancer area showed the complete intestinal type, and thus may have differentiated the CCS polyps from that of the common gastric hyperplastic polyps. This case illustrates that a clue to detecting small cancers may be to look for the discolored lesion among reddish CCS polyposis and thereafter to observe the irregular vascular pattern with NBI endoscopy. From the viewpoint of genetic alterations, patients with CCS polyps are considered to be at high risk for developing gastric cancer, and therefore careful follow-up examinations are necessary for the early detection of malignancies.
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PMID:Endoscopically treated Cronkhite-Canada syndrome associated with minute intramucosal gastric cancer: an analysis of molecular pathology. 2195 Oct 93