Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A series of 29 cases of amyloidosis of the alimentary tract is reported. Five cases (17%) were primary amyloidosis; 14 cases (48%) were amyloidosis secondary to other diseases (such as chronic inflammatory and neoplastic diseases); 10 cases (35%) were amyloidosis of the heredo-familial type connected with Familial Mediterranean Fever. In 23 patients (79%) the diagnosis was established by biopsies, and in 6 more cases on autopsy. Gastrointestinal involvement was found in all age groups. Gastro-enterologic complications observed in the present series include: diarrhea, malabsorption, ileus and gastrointestinal bleeding. In addition other conditions such as jaundice (3 cases), esophagitis and acute hemorrhagic pancreatitis were observed. In 22 patients proteinuria was observed and in 13 patients the nephrotic syndrome. Among 17 patients, in 11 the clinical picture before death was that of terminal renal failure. The survival after diagnosis among 14 patients reached 4 years in 9 cases, and 19 years in one case. The diagnostic value of the rectal biopsy is emphasized.
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PMID:[Gastrointestinal amyloidosis]. 18 89

Necropsy of a 34-year-old women four years after she had undergone pancreaticoduodenal resection showed amyloidosis, fatty liver, and cachexia. In life a malabsorption syndrome had been diagnosed two years after the resection, but at this stage tests for amyloidosis were still negative. Besides the fatty liver, positive histochemical tests for lipids (lipoproteins) in amyloid deposits were also found. Taken together with other findings these features reinforce the suggestion that lipprotein accumulation plays an essential part in the formation of amyloid.
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PMID:Secondary amyloidosis developing after pancreaticoduodenal resection. 86 80

The natural clinical course of four patients with systemic amyloidosis was favourably altered by continuous colchicine therapy. One patient had primary amyloidosis, and the other three suffered from amyloidosis of familial Mediterranean fever. All had a nephrotic syndrome, and one showed features of intestinal malabsorption. The institution of colchicine therapy was followed by a gradual remission of the nephrotic syndrome, a rise of serum albumin to normal values, a slight improvement of renal function, and regression of the intestinal malabsorption. This pattern has remained steady during an observation period of 30 months.
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PMID:Prolonged colchicine treatment in four patients with amyloidosis. 92 Oct 85

The records of a series of 700 patients with inflammatory bowel disease, 498 with Crohn's disease and 202 with ulcerative colitis, have been analyzed to determine the relative incidence and characteristic features of their extra-intestinal manifestations. The group with Crohn's disease included 62 with colitis, 223 with ileocolitis, and 213 with regional enteritis. A consideration of the clinical patterns and an understanding of their pathophysiology suggested a subdivision into two main groups: one "colitis related" and one related to the pathophysiology of the small nonspecific third group. Group A, colitis related, comprises joint, skin, mouth, and eye disease. The complications might be immunologically determined, were closely associated with active inflammation, and often responded to medical or surgical treatment of the underlying bowel disease. They occurred in 36% of the entire series of patients: joints were involved in 23%, skin in 15%, and mouth and eye each in 4%. Pyoderma gangrenosum was observed most often in ulcerative colitis and erythema nodosum most often in granulomatous colitis. The incidence of Group A complications was higher in disease involving the colon (42%) than in disease restricted exclusively to the small bowel (23%). There were interrelationships among the various members of Group A, with multiple manifestations occurring in a third of affected patients. Group B, related to small bowel pathophysiology, includes malabsorption, gallstones, kidney stones, and non-calculous hydronephrosis and hydroureter. Disorders in this group were generally related to the severity of the disease in the small bowel and tended to persist even in the absence of active inflammation. In contrast to Group A, this group occurred most frequently in small bowel disease, and least in colonic disease. Malabsorption was virtually confined to the patients with small bowel disease (10% incidence), while gallstones and renal stones were also both more frequent in Crohn's disease (11% and 9% respectively), the latter usually in association with small bowel resection or ileostomy. Group C, found in a small percentage of patients, consists of nonspecific complications, including osteoporosis (3%), liver disease (5%), peptic ulcer (10%), and amyloidosis (1%).
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PMID:The extra-intestinal complications of Crohn's disease and ulcerative colitis: a study of 700 patients. 95 99

The neurological and muscular complications seen in coeliac disease in adults are usually attributed to deficiency secondary to malabsorption. Amongst them, however, there exists a very rare cateogory, described by Cooke et al. (1966) taking the form of a chronic myeloneuropathy which cannot be explained in terms of the malabsorption syndrome. Our two cases of gluten intolerance enteropathy, confirmed by biopsy before and after diet, fell into this group of polyneuropathies. The patients, both women, suffered from an essentially sensory ataxic polyneuropathy with accessory motor component with pyramidal and posterior column signs. CSF findings showed a meningeal inflammatory reaction in one of the two cases. These neurological signs, appearing paradoxically during a digestive disease cured by diet, evolve chronically but become stabilised with corticosteroid therapy. Any vitamin deficiency may be excluded in the aetiology of these problems. Neuropathological study of neuromuscular biopsies in very fine serial sections confirmed the mild peripheral nervous involvement but revealed identical inflammatory lesions in the nerve and muscle which were remarkable by virtue of their very highly segmentally selective micro-vasculitis appearance. In these two cases, general, clinical and biological arguments, as well as the type of histological lesion, make it possible to exclude monoclonal gammapathies, malignant haemopathies, amyloidosis and the major collagen diseases. This micro-vasculitis, having transient forms with P.A.N. is no less distinctive, and may be integrated into the provisional group of "allergic angeitis", related to physiopathology of circulating immune complexes and very fashionable in theories as to the mechanism of gluten-sensitive enteropathies. The exact nature of the link between the latter and these types of polyneuropathy remains unknown.
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PMID:[Nondeficiency chronic polyneuropathies in celiac disease in adults (2 cases with inflammatory neuromuscular vascularitis)]. 100 65

The pathology of primary familia amyloidosis with polyneuropathy is described on the basis of post-mortem examination of six cases from Northern Sweden. Clinically the disease is characterized by progressive sensory and motor disturbances with loss of sensation, muscular wasting and flaccid paralysis. Impotence, urinary bladder dysfunction, motility disturbances of the gastro-intestinal tract and postural hypotension indicate affection of the autonomic nervous system as well. Malabsorption, cardiac insufficiency and vitreous opacites also occur. As regards the distribution of amyloid, the following findings seemed to be characteristic. Usually there were no gross lesions indicating the amyloid disease. Histopathologically, amyloid deposits were observed in great extent in the peripheral nervous system and in various parts of the peripheral autonomic nervous system as well. It occurred extensively in the walls of blood vessels of various calibres, in the perivascular collagenous connective tissue and adjacent to the smooth musculature. Amyloid deposition was also found more or less abundantly in various other organs and tissues. No deposits, however, or only insignificant amounts, were found in the central nervous system, either in the parenchyma of the liver, in the islets of Langerhans, or in the bone marrow. Clinical manifestations seemed to be related to the local deposition of the amyloid substance. Our clinical and pathological findings in this particular type of familial amyloidosis conformed mostly to those previously described.
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PMID:Postmortem findings in primary familial amyloidosis with polyneuropathy. 115 99

Gastrointestinal dysfunction due to autonomous neuropathy is a complication described in various diseases such as diabetes mellitus, multiple sclerosis, and familial amyloidosis with polyneuropathy. We present the results of a prospective investigation of bile acid malabsorption in 17 patients with familial amyloidosis by means of 75Se-labelled homocholic-tauro acid (SeHCAT). The diagnosis was in all cases verified by the DNA test for mutation of transthyretin in position 30. Small-intestinal biopsy specimens were examined for deposits of amyloid, and the presence of gastric retention was evaluated by gastroscopy. In addition, the patients were investigated for bacterial overgrowth by means of the bile acid breath test (BABT). A high frequency of abnormal BABT results (44%) was encountered. However, 65% also had abnormal low SeHCAT values, indicating bile acid malabsorption. Only two patients had abnormal BABT and normal SeHCAT results, indicating bacterial contamination of the small intestine. Bile acid losses increased with the duration of gastrointestinal symptoms. Significantly lower SeHCAT values were encountered in patients with gastric retention, whereas the occurrence of amyloid deposits in small-intestinal biopsy specimens was without effect on SeHCAT retention. Bile acid malabsorption is frequently encountered in familial amyloidosis with polyneuropathy and seems to be more closely associated with gastrointestinal motility dysfunction than with amyloid deposits in the intestinal mucosa.
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PMID:Bile acid malabsorption caused by gastrointestinal motility dysfunction? An investigation of gastrointestinal disturbances in familial amyloidosis with polyneuropathy. 150 82

The clinical outcome of 61 patients with renal amyloidosis treated with chronic dialysis was reviewed. Eighteen patients, 4 with primary or AL amyloidosis and 14 with reactive or AA amyloidosis, died within one month from starting treatment. The other 43 patients were treated with dialysis for 3 to 199 months and are the object of this study. Sixteen patients had AL amyloidosis and 27 had AA amyloidosis. Thirty-five patients were treated with hemodialysis (HD) for a mean period of 40 +/- 47 months and 8 were treated with continuous ambulatory peritoneal dialysis (CAPD) for 20 +/- 15 months. Patient survival rate at 1 and 5 years was 68% and 30% respectively. There was no difference in survival rate between patients treated with HD and those treated with CAPD, while patients younger than 45 had a better 5-year survival rate. Twenty four (60%) patients achieved a satisfactory rehabilitation with dialysis. At the last follow-up, 15 patients (14 on HD, 1 on CAPD) were alive 61 +/- 58 months after starting dialysis. Twenty-eight patients died after 30 +/- 20 months. The main causes of death were: cardiovascular accident (11), stroke (3), sepsis (5) and cachexia (5). The most important extra-renal complications of amyloidosis were related to cardiovascular involvement (heart failures, arrhythmias, hypotension) and gastrointestinal involvement (malabsorption). Intra-dialytic hypotension in patients on HD and peritonitis in patients on CAPD were the main problems related to dialytic procedure. his study confirms that life expectancy and the quality of life of dialysis patients with systemic amyloidosis are poorer than those of general dialysis population.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Chronic dialysis in patients with systemic amyloidosis: the experience in northern Italy. 151 84

To investigate small bowel motility in gastrointestinal amyloidosis, lactulose breath hydrogen tests were performed on 16 patients with histologically proven amyloidosis and 12 age- and sex-matched controls. Fasting breath hydrogen concentration (FBHC) was not significantly different between the two groups, but there was a tendency for FBHC in symptomatic amyloidosis patients (median 31.5, range 3-78 ppm) to be higher than in asymptomatic amyloidosis patients (4, 0-34 ppm, 0.05 less than P less than 0.1) and controls (6, 1-19 ppm, 0.05 less than P less than 0.1). Orocecal transit time (OCTT) was significantly delayed in the amyloidosis group (median 150, range 40-220 min) when compared to the controls (60, 20-110 min, P less than 0.01), but OCTT was not statistically different between symptomatic and asymptomatic amyloidosis patients. These data suggest an impaired motility of the stomach and small intestine in gastrointestinal amyloidosis and the possible role of small intestinal dysfunction such as bacterial overgrowth and malabsorption in the occurrence of symptoms in this disorder.
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PMID:Breath hydrogen test using water-diluted lactulose in patients with gastrointestinal amyloidosis. 174 46

The authors describe a rare case of amyloidosis in a female patient suffering from periodic disease (PD) for 18 years without any clinico-laboratory signs of renal impairment but with marked clinical, (malabsorption, cachexia), endoscopic, x-ray and other manifestations of gastrointestinal amyloidosis. This case is of interest since patients suffering from amyloidosis due to PB develop malabsorption very rarely, namely in 2-3% of cases. As a rule, it develops in patients with pronounced chronic renal failure on hemodialysis or with a history of kidney transplantation. In this particular case, the patient demonstrated selective marked damage to the gastrointestinal tract, with the kidneys remaining practically intact. A possibility of the indicated variety of amyloidosis should be considered in specification of the genesis of persistent diarrhea in PB patients.
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PMID:[Selective involvement of the gastrointestinal tract in amyloidosis in a female patient with periodic disease and intact kidneys]. 179


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