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This is the first comprehensive report of coeliac disease from South Africa. Twenty patients with adult coeliac disease diagnosed in Johannesburg between 1966 and 1978 are reviewed. It is noted that a significant number did not present with the classic features of the disease. Secondary nutritional manifestations were frequently absent, and may have been masked in some patients by self-medication or by the prescription of vitamins and haematinics before the diagnosis was made. The majority of patients were from the middle or upper socio-economic class, and few complained of anorexia, nausea, vomiting or abdominal pain. It is therefore possible that many maintained an adequate intake of nutrients throughout the course of their illness. The severity of diarrhoea and steatorrhoea did not correlate with the state of nutrition of the patients as assessed by a variety of blood studies. Megacolon, present in 2 patients, responded to a gluten-free diet. An interesting observation was that patients diagnosed after the age of 40 years had been symptomatic for a much shorter period of time than younger patients.
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PMID:Adult coeliac disease in South Africa. An analysis of 20 cases emphasizing atypical presentations. 722 82

Fifty-five patients with celiac disease and coexistent malignant disease (27 lymphoma, 28 other malignancies) are described. The important clinical features at presentation of lymphoma were weight loss, abdominal pain, diarrhea, profound weakness and fever, associated with anemia, raised ESR, hypoalbuminemia and steatorrhea. There were no specific features to enable earlier diagnosis. Radiology was unhelpful and in no case were malignant cells seen in the jejunal biopsy. Four of the lymphomas were Hodgkin's disease, none of which involved the bowel; the remainder were reticulum cell sarcoma, 17 of which involved the bowel. Definitive diagnosis prior to death was made in only 18 patients, of whom 16 survived from 2 to 226 days (mean, 76 days). Of the remaining two patients, one is still alive, while the other died 26 years after the original diagnosis of Hodgkin's disease. The possibility of lymphoma should be considered in those who present with celiac disease in middle life and in those who deteriorate for no apparent reason after a period of stability on a gluten-free diet. The index of suspicion for lymphoma in celiac disease should be high and early laparotomy be considered in patients with unexplained deterioration. Twenty-eight patients with 29 carcinomas and 3 other tumors are also described. The presentations of these malignancies were no different from their presentations in non-celiac patients, and their development did not provoke a relapse of celiac disease. Considering the whole series of 55 patients, there was little evidence for the view that malignancy itself was the cause of the flat jejunal mucosal appearances seen in these patients.
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PMID:Celiac disease and malignancy. 739 45

Eight adult female patients suffering from abdominal pain and chronic diarrhea which was often incapacitating and frequently nocturnal, had dramatic relief on a gluten-free diet and return of symptoms after gluten challenge. Previous nonspecific measures and a milk-free diet were ineffective. Multiple jejunal biopsies showed minor, but significant changes in cellularity which returned to normal on the gluten-free diet. Apart from a slight increase in jejunal cellularity, no immunological abnormalities were found after gluten challenge. Steatorrhea or other biochemical defects, common in celiac disease, were not found. It was concluded that these patients had a gluten-sensitive diarrhea, but had no evidence of celiac disease.
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PMID:Gluten-sensitive diarrhea without evidence of celiac disease. 741 3

Peroral jejunal biopsies were performed in 63 adult patients from a population of 40,000. Twenty-two patients had definite pathologic biopsy findings; 20 of these had gluten-induced enteropathy. With the 15 previously known patients in the region, this makes a minimum prevalence of 1 per 1,143. Subtotal villous atrophy was found in 8 patients, whereas partial villous atrophy was found in 12 patients. The clinical picture varied considerably, borborygmia, diarrhoea, abdominal pain, and psychiatric symptoms being the most commonly encountered symptoms. Steatorrhoea was found in only two patients with subtotal villous atrophy. In all the remaining patients, including six with subtotal villous atrophy, the faecal fat excretion was normal. A low whole blood folic acid concentration was the most consistent laboratory finding, although some patients had values slightly above the lower normal limit. Anaemia was not pronounced. Iron deficiency, as measured by the erythrocyte protoporphyrin IX concentration, was not common. A high degree of clinical awareness and broad indications for jejunal biopsy-taking is recommended.
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PMID:Prevalence and clinical picture of adult gluten-induced enteropathy in a Norwegian population. 743 1

Focal and multilobular biliary cirrhosis are considered pathognomonic of cystic fibrosis (CF) and almost invariably have been reported in patients with steatorrhea. In contrast, patients with pancreatic sufficiency and normal absorption are considered less likely to develop liver or biliary tract problems. The authors report three patients with CF and pancreatic sufficiency, presenting with recurrent abdominal pain (unrelated to pancreatitis). All had common bile duct disease, one with multilobular cirrhosis and portal hypertension. Pancreatic sufficiency was proven by quantitative pancreatic stimulation tests, 3-day fecal fat analyses, and serum pancreatic isoamylases. All three patients had mild lung disease. Two were homozygous for the common delta F508 mutation, and the other, a delta F508 compound heterozygote. Hepatobiliary structure and function were determined by serial hepatobiliary scintigraphy, percutaneous transhepatic cholecystography, and biochemical liver function tests. Patients 1 and 3 had mild hepatomegaly, normal liver biochemistry, and distal common bile duct strictures. Patient 2 had a firm nodular liver with splenomegaly, abnormal liver biochemistry, and a cholangiographic appearance of sclerosing cholangitis. All have undergone operative treatment for persistent abdominal pain. These cases confirm the occurrence of common bile duct pathology and liver disease in patients with CF and pancreatic sufficiency. They demonstrate that liver and biliary tract disease can occur independently of the underlying disease severity and the presence of steatorrhea. Further, they suggest that obstruction of the biliary tract may be an additional factor in the evolution of liver disease in CF.
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PMID:Hepatobiliary disease in cystic fibrosis patients with pancreatic sufficiency. 753 38

Hereditary pancreatitis (HP) is a rare cause of chronic pancreatitis. Recurrent abdominal pain is the most common clinical manifestation, with onset in childhood or adolescence. PURPOSE--Report of a case of HP with atypical presentation and a review of the literature. METHODS--A non-alcoholic patient, without history of abdominal pain, with steatorrhea and malnutrition was investigated. The diagnostic evaluation revealed severe chronic pancreatitis. Two close relatives with early onset calcifying pancreatitis were detected. Epidemiologic and clinical features of HP were reviewed. CONCLUSION--Although uncommon, HP should be regarded in the differential diagnosis of chronic pancreatitis. Familial screening of suspected cases should be routinely performed.
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PMID:[Hereditary pancreatitis]. 763 5

Chronic pancreatitis should be considered in all patients with unexplained abdominal pain. The importance of small duct disease without obvious radiographic abnormalities is an important new concept. Diagnostic evaluation should begin with simple, non-invasive, inexpensive tests (serum trypsinogen) to be followed by more complicated testing (hormone stimulation test) if needed. Enteric-coated pancreatic enzymes are the drugs of choice for treating steatorrhea, while conventional non-enteric coated enzymes are preferred for managing pain. The somatostatin analogue octreotide may become an important therapy for treating abdominal pain unresponsive to pancreatic enzyme therapy. Endoscopic approaches to the treatment of chronic pancreatitis are experimental and may cause damage to the pancreas. Surgical ductal decompression is appropriate in selected patients.
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PMID:Medical management of chronic pancreatitis. 777 9

In the period 1968-1989 50 patients, 38 women and 12 men, aged 30-76 years, median 58 years, were referred to the Department of Gastroenterology, Rigshospitalet for severe chronic radiation enteropathy. Most women had received radiation for gynaecological cancer, and most men for urogenital cancer. The initial symptoms of the enteropathy were diarrhoea in 74%, abdominal pain in 62% and weight loss in 52%. Twelve per cent had visible blood in the stools. Ten per cent had fistulas. The symptoms occurred 0-37 years, median ten months after the radiation. The radiation enteropathy had necessitated one or more laparotomies in 35 patients, most often because of subileus/ileus, including resection of the small bowel or the colon in 25 patients, and establishment of an ileostomy or a colostomy in 11. Seven patients developed new fistulas postoperatively. In 32 patients one or more tests for malabsorption were performed as a guidance for therapy: stool mass (26 patients), faecal fat excretion (26 patients), Schilling test (22 patients), lactose absorption (11 patients) and bile acid breath test (seven patients). Half of the patients had diarrhoea, including one third of the patients without intestinal resection. Two thirds had steatorrhoea, including half of the patients without small bowel resection. Three fourths showed decreased absorption of vitamin B12, including half of the patients without ileal resection. All patients studied had abnormal deconjugation of bile acids and more than half of them had bile acid malabsorption. Malabsorption of lactose was found in only one patient. There was no correlation between the radiological and functional abnormalities of the small intestine.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Chronic radiation enteropathy. A retrospective study]. 827 44

Chronic pancreatitis is caused most often by chronic excessive alcohol consumption. The disease can be diagnosed by investigations that measure function or assess morphology of the pancreas, thereby permitting a tailored management approach. In many patients, abdominal pain and steatorrhea can be managed effectively by enzyme supplementation, provided such supplements are administered in appropriate formulations and doses. Definition and correction of mechanical problems are possible by modern endoscopic approaches, and surgery plays an important role in the management of local and regional complications of the disease.
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PMID:Chronic pancreatitis. 843 93

We conducted a retrospective study of patients younger than 20 years of age who had a diagnosis of chronic pancreatitis and underwent assessment at the Mayo Clinic between 1960 and 1990. Those with a known etiologic factor for the pancreatitis (such as a virus, trauma, alcohol, or hyperlipidemia) were excluded from the study. We compared the clinical course of the 42 patients who had hereditary pancreatitis (HP)--defined as at least two family members affected by the condition--with that of the 28 patients who had idiopathic pancreatitis (IP). The mean age at initial assessment was 7 years for those with HP and 12 years for those with IP. All patients in both groups had abdominal pain. Vomiting was more frequent in patients with HP than in those with IP; otherwise the initial symptoms were similar in both groups. Patients with HP, however, had more complications, including pseudocysts (seven patients), steatorrhea (four), ascites (three), portal hypertension (two), and diabetes (one), than did patients with IP (one each had diabetes, steatorrhea, and a pseudocyst). Complications or pain necessitated surgical intervention in 23 of 42 patients with HP versus 4 of 28 patients with IP. Overall in comparison with IP, HP seems to be a more severe variant of chronic pancreatitis, inasmuch as it is associated with more frequent complications and need for surgical intervention.
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PMID:Long-term follow-up of young patients with chronic hereditary or idiopathic pancreatitis. 847 8


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