Diagnosis Of Cronkhite-Canada Syndrome Research Articles

Cronkhite-Canada Syndrome Presenting with life-threatening protein-losing enteropathy: a case report

Background: Cronkhite Canada Syndrome (CCS) is a rare gastrointestinal polyposis syndrome. The diagnosis of CCS is made by clinical, endoscopic and histopathological findings. Chronic diarrhea, malnutrition, alopecia, skin hyperpigmentation, onychodystrophy, hypogeusia, protein loss due to chronic inflammatory changes in the intestinal mucosa are common findings.Nutritional support, high protein diet, antibiotics, correction of electrolyte imbalance, and corticosteroids are frequently used in CCS treatment. Aim of the study: Here we present a newly diagnosed Cronkhite Canada Syndrome patient who has suffered life-threatening protein loss. Material and methods: Medical documentation was used. Case report: A 62-year-old male patient presented with bloodless diarrhea, happening 8-10 times a day for 4 months, and general condition disorder. On admission, arterial blood pressure was 80/50 mm/hg, pulse was 110 per minute and body temperature was 38.8°C. In laboratory tests, total protein: 38 mg/dl and albumin: 20 g/L.Upper and lower gastrointestinal system (GIS) endoscopy revealed 2-20 mm polyps in the stomach, duodenum, colon, and a small amount in the distal esophagus. Pathological examination of polypectomy materials revealed edematous and inflamed lamina propria consisting of plasma cells, neutrophils and eosinophils.The patient benefited from total parenteral nutrition, high protein dietary supplementation, antibiotic therapy and was followed with upper and lower GIS endoscopy. Conclusions: CCS is a rare disease that can cause life-threatening hypoalbuminemia and requires close follow-up.

Cronkhite-Canada syndrome with hypothyroidism: Clinical, endoscopic and pathologic pitfalls

Cronkhite-Canada Syndrome (CCS) is a rare non-hereditary protein-losing enteropathy, and characterized by ectodermal and gastrointestinal abnormalities. The etiology remains unclear, however, some studies suggest the likelihood of immune dysregulation. The diagnosis of CCS is challenging because the clinical symptoms are non-specific and the endoscopic and pathologic findings can overlap with other processes. This can potentially lead to delay in diagnosis, resulting in delayed patient management. We reported a 74-year-old male presented with initial symptoms of abdominal pain and nausea, and later with diarrhea, weight loss, hypothyroidism and multiple strokes. Gastric Antral Vascular Ectasia (GAVE) was suspected on the initial endoscopy. The diagnosis of CCS was rendered on the third biopsy. After steroid treatment, the patient’s clinical symptoms were relieved. In this paper, we discussed the clinical, endoscopic and pathologic pitfalls of CCS.

Case report of patient with a\xa0Cronkhite-Canada syndrome: sustained remission after treatment with corticosteroids and mesalazine

BackgroundCronkhite-Canada syndrome is a rare disease of unknown etiology and the optimal treatment for this syndrome is unknown.Case presentationWe present the case of a man who at the age of 66.0 years was diagnosed with Cronkhite-Canada syndrome (CCS). In addition to watery diarrhea, alopecia, and a complete loss of toenails and fingernails, the patient had been suffering from dysgeusia and rapid weight loss of more than 10.0 kg within a few months. The patient had recently incurred a distal radius fracture. During the initial endoscopy an extensive polyposis of the stomach and jejunum was found. The diagnosis of CCS was made and after initiation of a steroid therapy his diarrhea improved immediately. A discontinuation of the steroid therapy was not possible and mesalazine (1000 mg t.i.d.) was added to prednisolone (10.0 mg/d). This therapy led to a remission within 6.0 months with weight gain and normalization of serum albumin levels. The prednisolone dose was reduced to 7.5 mg/d. During the following year, the steroids could be further reduced and nails had regrown again. Within three years, all polyps had disappeared and the steroid therapy was finished while the dosage of mesalazine was reduced in a stepwise fashion. Four years later, the mesalazine was stopped and more than 14.0 years after the initial diagnosis the patient is still in complete remission without any treatment.ConclusionThe optimal treatment for CCS is unknown. In our case, the initial combination therapy of corticosteroids plus mesalazine followed by a mesalazine monotherapy has led to a remarkable long-lasting remission with complete resolution of all intestinal polyps.

Cronkhite-Canada Syndrome: A Rare Cause of Chronic Diarrhoea in a Young Man.

A young Indian man presented with nine-month history of chronic diarrhea, occasionally mixed with blood and intermittent colicky abdominal pain. He also complained of generalized body swelling for the last three months. On examination, he had diffuse hyperpigmentation of the skin and dystrophic nail changes. Upper and lower gastrointestinal endoscopy revealed multiple sessile polyps in the stomach, small bowel, and colon and rectum. Biopsy of polyps showed adenomatous changes with stromal edema and dilated glands. Cronkhite-Canada syndrome (CCS) was diagnosed and treated with glucocorticoids and enteral nutritional supplementation. There was an associated small intestinal bacterial overgrowth (SIBO) and stool was positive for clostridium difficile toxin. After 12 weeks of treatment, the patient achieved remission. Close correlation with clinical findings, including pertinent ectodermal abnormalities, endoscopic studies, and careful examination of biopsies will ensure a timely and correct diagnosis of CCS.

Cronkhite-Canada Syndrome: A Case Report and Literature Review of Gastrointestinal Polyposis Syndrome

Cronkhite-Canada syndrome (CCS) is a rare, non-inherited polyposis syndrome, characterized by diffuse gastrointestinal (GI) hamartomatous polyposis with unique dermatologic changes including alopecia, skin hyperpigmentation, and nail dystrophy. Patients can typically present with diarrhea, weight loss, protein-losing enteropathy, and nutritional deficiency. However, it can demonstrate diverse other clinical features, usually with poor prognosis. Currently, there are no specific diagnostic criteria established yet. The etiology of CCS is still obscure, but an autoimmune process has been suggested. Here we present an 81-year-old Caucasian female who had clinical presentations, physical exam, imaging, endoscopy and pathology findings that were all consistent with the diagnosis of CCS. We also include a detailed literature review of the other gastrointestinal polyposis syndromes (hamartomatous, adenomatous, hyperplastic and inflammatory polyposis). A high index of suspicion and recognition of the characteristic clinical, endoscopic as well as histopathological findings of CCS, as well as different gastrointestinal polyposis conditions, can help clinicians with more timely and correct diagnosis.

Cronkhite–Canada Syndrome Diagnosis in the Absence of Gastrointestinal Polyps

A 66-year-old male patient presented with nausea, abdominal pain, occasional rectal bleeding, progressive dysgeusia, onicodystrophy, and alopecia. Endoscopic exam and biopsies revealed severe atrophy and diffuse marked edema of mucosa of stomach and duodenum. No evidence of polyps was found in any portion of the gastrointestinal tract. The diagnosis of Cronkhite-Canada syndrome (CCS) was rendered. The patient symptoms resolved completely after initiation of steroid treatment. This additional case of CCS illustrates how the diagnosis of CCS does not require the presence of polyps but is defined by the appreciation of the diffuse marked edema and atrophy of the gastrointestinal mucosa.

Hyperpigmentation, nail dystrophy and alopecia with generalised intestinal polyposis: Cronkhite–Canada syndrome

A 62-year-old Malaysian woman presented with a constellation of skin signs including alopecia, hyperpigmentation and nail dystrophy. On questioning, a history of diarrhoea, taste disturbance and weight loss was found. The onset of these changes coincided with the administration of thyroxine prescribed for a benign multinodular goitre. Hormonal investigations showed no abnormality and no underlying malignancy was found. Investigation of the diarrhoea showed a protein-losing enteropathy with generalized intestinal polyposis and non-specific histology. A diagnosis of Cronkhite-Canada syndrome was made. Treatment with prednisone and nutritional support has been partially effective.