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  • Essay / Cronkhite-Canada syndrome (CCS) - a rare case report

    Table of ContentsIntroductionCase reportLaboratoryEndoscopyDiscussionIntroductionCronkhite-Canada syndrome (CCS) is a sporadic condition that involves tissue from the ectodermal germ cell layer. It is characterized by gastrointestinal hamartomatous polyposis, diarrhea, hypoproteinemia and skin manifestations including alopecia, onychodystrophy, hyperpigmentation. Other symptoms such as dysguitis, hypoguitis and xerostomia due to zinc and cobalt deficiency have been reported in some patients. Its incidence is low but mortality is high. Only 500 cases of CTS have been reported worldwide, with an overall mortality rate of approximately 60%. The main age of presentation is 59 years with a slight male predominance in the ratio 3:2, however the majority of cases identified at an age above 50 years. Say no to plagiarism. Get a tailor-made essay on "Why violent video games should not be banned"? Get the original essay The very first case of Cronkhite syndrome-Canada was reported in 1955 by internist Leonard Wolsey Cronkhite and radiologist Wilma Jeanne Canada in New England. Journal of Medicine, yet the etiopathogenesis of this disease is not fully understood, but there are two schools of thought: one that supports the immunological mechanism, which is more believed in, and the other that supports the infectious mechanism. The latter was also considered due to histopathological issues. Gastrointestinal polyp biopsy results showed infiltration of inflammatory cells by mononuclear cells and eosinophils. While CCS polyps were infiltrated with IgG4 plasma cells; elevated antinuclear antibody; a decrease in disease manifestations during treatment with corticosteroids and an association with other autoimmune diseases such as hypothyroidism, systemic lupus erythematosus, rheumatoid arthritis and scleroderma strongly support the autoimmune pathophysiology of this disease. The only postulated risk factors for CTS include mental and physical stress. Case ReportA 44-year-old Indian man residing in Canada for 16 years and having a history of gout for 7 years complained of chronic diarrhea, intermittent bloody stools and nausea. , abdominal pain, weight loss for 2 months, he had occasionally taken alcohol for 10 years. He was taking proton pump inhibitors for chronic gastritis, allopurinol for gout, ibuprofen for symptomatic relief of gouty pain and after a week of ibuprofen treatment he spontaneously developed diarrhea that did not resolve with medication with increasing frequency up to 10 times a day, with her hair. initially graying from the pubis advancing to the axillary hair, progressing to the mustache and beard, with the scalp being the last site affected. He initially had intermittent abdominal cramps which developed into several bouts of abdominal pain. He started losing all his hair, including his nose, causing a continuous runny nose and dried nasal mucosa. and developed brittle nails that also began to fall off. During this period, he suffered from severe malnutrition and lost almost 4 kg of weight in 3 weeks. During his first hospital visit, intestinal pathology was suspected, so pantoprazole and antibiotics were prescribed after an x-ray and blood tests. After further examination, he underwent an esophagogastroduodenoscopy with biopsy which revealed small sessile polyps throughout the digestive tract exceptin the esophagus, he was quickly put on corticosteroids, with a possible immune response leading to polyp formation, kept in mind. Over the next 5 weeks, he lost another 6 kg of weight with loss of taste sensation. He then underwent video colonoscopy during which a biopsy of the colonic and gastric polyps was performed for immunohistological results. On physical examination, he was fairly built, poorly nourished, weighing 68 kg, presenting without eyebrows, without baldness, without onychodystrophy with loss of a finger and a nail. The gouty swelling was present on the right toe. There were hyperpigmented oval spots measuring 5 to 15 mm in diameter on all palms and soles of feet. His palms were sweaty with patchy hyperpigmentation noted on different parts of the body. Physical examination also showed symmetrical bilateral lower extremity edema. No other significant results were observed. Symptomatic treatment initially included corticosteroids, analgesics, and broad-spectrum antibiotics. Nutritional support was provided with multivitamin tablets, calcium and iron. The PPIs were stopped. A high protein diet was recommended. This reduced the symptoms, but not effectively. Then in India he stopped all medicines and started Ayurvedic powder along with diet treatment. The Ayurvedic powder was a mixture of dried fruits of Aegle marmelos (Indian bael), Zingiber officinale (dried ginger) and Gymnema sylvestre leaf extract in the ratio of 1:1:2 respectively. The daily diet consisted of only turmeric milk, cracked wheat and puffed rice. Calcium and iron folic acid tablets were supplemented. This diet was followed for six weeks. By the end of the sixth week, there were only 2 episodes of bloody diarrhea, the frequency of stools decreased from 10 times a day to 5-6 times. After six weeks, khichri, an Indian dish made from cracked wheat and lentils, was added to his diet. Ayurvedic powder was replaced by Ayurvedic tablets consisting of Holarrhena antidysenterica bark extracts, Plumbago zeylanica root extracts, Piper longum root extracts, Bryophyllum pinnatum leaf extracts and Cinnamomum camphora bark extracts. Six weeks later, he started to have white hairs on his eyebrows, mustache and pubic area. Stool frequency decreased to 3 to 4 times per day. The hair began to turn black, the weight increased by 2 kg and finally new nails began to grow. A CT scan was also performed at the end of 12 weeks after the start of diet-based treatment, which confirmed the improvement of the pathology. Currently, he no longer takes Ayurvedic medicines and takes 1,200 mg of mesalazine, four times a day. Laboratory His eosinophil count was 7.4% (normal range: 0.0 – 6) with an absolute count of 0.58 x 10^3 / μl (normal range: 0- 0.5), his hemoglobin was of 12.5 g/dl (normal range 13 – 17), he had a mean corpuscular hemoglobin of 26 pq (normal range 27-32), a mean corpuscular hemoconcentration of 26.5 g/dl (normal range 31.5 – 34.5). Red Blood Cell Distribution Width – Sd of 53.6 fL (Normal Range 39-46) Red Blood Cell Distribution Width – CV 14.6% (Normal Range 11.6-14), Total Vitamin D 25-OH of 6.5 ng/ml, calcium levels of 8.09 mg/dl (normal 8.8 – 10.6, uric acid level of 9.4 (normal range 3.7 – 9.2), estimated glomerular filtration rate of 81 (between 60 – 89 indicates a slight decrease). EndoscopyEsophagogastroduodenoscopy revealed polyps with atrophy of gastric villi and a normal esophagus..