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Renal amyloidosis was confirmed in 6 related male and female Beagles, ranging in age from 5 to 11 years. The most commonly reported signs of illness included lethargy, anorexia, vomiting, and weight loss. Common clinicopathologic abnormalities were normocytic, normochromic anemia; hypoalbuminemia; azotemia; hypercholesterolemia; proteinuria; and urine specific gravity values below the normal range. Histologic examination of renal tissue from the 6 Beagles revealed moderate to severe glomerular amyloidosis with inconsistently observed mild medullary interstitial amyloidosis. Congo red-stained kidney sections from 4 of 4 affected dogs were potassium permanganate-sensitive, suggestive of reactive amyloidosis. Hereditary predisposition for renal amyloidosis was suspected in these Beagles.
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PMID:Renal amyloidosis in a family of beagles. 151 31

Forty-five patients with proved gastrointestinal amyloidosis were examined to study the clinical and pathological features and to determine the correlation with amyloid fibril proteins. The examinations included physical examination, laboratory study, plain X-ray film of the abdomen, gastrointestinal radiography, gastrointestinal endoscopy, endoscopic biopsy, surgery, and autopsy. The results were as follows: 1) Amyloid fibril proteins consisted of amyloid light chain protein (AL) in 7 patients, amyloid A protein (AA) in 36, beta 2-microglobulin (AH) in one, and prealbumin (AF) in one. 2) At the time of examination, gastrointestinal symptoms were present in 43 (96%) of the 45 patients. The incidence of diarrhea and gastrointestinal bleeding was higher in patients with the AA type than in those with other types, whereas abdominal distension, nausea, and vomiting were more frequently evident in patients with the AL type. 3) The incidence of hypoalbuminemia, hypokalemia, and positive occult blood was higher in patients with the AA type, whereas abnormal electrocardiogram and cardiac failure were more frequently observed in patients with the AL type. The thyroid gland was enlarged in 7 patients with the AA type, and the macroglossia was evident in only 2 patients with the AL type. 4) The frequency of radiographic abnormalities in the gastrointestinal tract was as follows: 79% of the patients in the jejunum and ileum, 61% in the duodenum, 53% in the stomach, 32% in the colorectum, and 14% in the esophagus. Fine granular shadows were most frequently seen in patients with the AA type, whereas multiple polypoid protrusions and thickening of the folds were characteristic in patients with the AL type. 5) Endoscopic abnormalities were found as follows: 82% in the duodenum, 81% in the jejunum, 73% in the stomach, 42% in the colorectum, and 20% in the esophagus. Endoscopy revealed fine granular appearance in 31 (86%) of the 36 patients with the AA type, whereas multiple polypoid protrusions and thickening of the folds were evident in 5 (71%) of the 7 patients with the AL type. 6) On the histological examination of the biopsy specimens, amyloid deposition was found in the duodenum in 98% of the 45 patients studied, in the jejunum in 96% of the 26 patients, in the antrum in 93% of the 41 patients, and in the rectum in 86% of the 43 patients. 7) Histological findings of the biopsy and autopsy specimens revealed that the the degree of amyloid deposition was the highest in the duodenum and jejunum of the gastrointestinal tract.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Diagnosis of gastrointestinal amyloidosis with special reference to the relationship with amyloid fibril protein]. 178 55

Clinical and computed tomographic findings in 24 patients with hereditary cerebral hemorrhage with amyloidosis-Dutch type were reviewed. The common initial symptoms were headache and vomiting. Computed tomographic scans showed 50 hypodense and 49 hyperdense cortical lesions and in 20 patients the scans also showed generalized white matter hypodensity. Impairment of consciousness was related to the size of the hemorrhagic lesion. Dementia, seen in 11 patients, was related to the duration of the disease and the number of focal lesions on the computed tomographic scans, but not to the presence of white matter hypodensity. It is concluded that in hereditary cerebral hemorrhage with amyloidosis-Dutch type, lobar hemorrhages account predominantly for the acute clinical syndromes. The hemorrhages often have an irregular shape and are responsible for progression of the symptoms after an acute onset. Furthermore, cerebral amyloid angiopathy leads to a generalized abnormality of the white matter, probably due to chronic hypoperfusion.
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PMID:Hereditary cerebral hemorrhage with amyloidosis-Dutch type. Clinical and computed tomographic analysis of 24 cases. 234 93

The medical records of 59 dogs with renal amyloidosis were reviewed. Most dogs with amyloidosis were greater than 6 years old, and females were affected more often than males. Beagles, Collies, and Walker Hounds were at increased risk, whereas German Shepherd Dogs and mixed-breed dogs were at decreased risk. Common historical findings were anorexia, polyuria, polydipsia, lethargy, vomiting, and weight loss. Common laboratory findings were leukocytosis, lymphopenia, nonregenerative anemia, hypercholesterolemia, azotemia, hyperphosphatemia, metabolic acidosis, isosthenuria, cylindruria, and proteinuria. Proteinuria was moderate to severe in most dogs, as assessed by qualitative determination of urine protein concentration, urine protein/urine creatinine ratio, and 24-hour urine protein excretion. Conservative medical management was of little value, and survival ranged from 3 to 20 months in 12 dogs for which this information was available. Moderate to severe diffuse global glomerular amyloidosis was detected in all dogs. Medullary amyloid deposition was multifocal and less severe, but was evident in most dogs. Secondary tubulointerstitial and glomerular lesions were mild or absent in most dogs. Thromboembolism was identified in approximately 14% of affected dogs, underlying inflammatory disease in 37%, and neoplasia in 20%. Laboratory indicators of renal function correlated poorly with histologic lesions, with the exception of glomerular amyloid deposition and "chronic renal disease" index with endogenous creatinine clearance.
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PMID:Clinicopathologic findings in dogs with renal amyloidosis: 59 cases (1976-1986). 276 63

Alzheimer's disease is a degenerative brain disorder with a progressive dementia which develops in middle or late life. The pathological findings of this disease are characterized by neurofibrillary tangles, senile plaques and cerebrovascular amyloidosis. However, cerebral hemorrhage caused by amyloid angiopathy rarely occurs. A 71-year-old woman, who had been suffering from an impairment of her cognitive ability for the past several months, suddenly developed a severe headache with vomiting and gait disturbance. Brain CT disclosed a hemorrhagic lesion in the right parieto-occipital region. In the following two years she had experienced two episodes of the similar subcortical hemorrhage which occurred in the right parietal lobe and bilateral parieto-occipital regions. She died at the age of 73. Histopathological examinations of the brain revealed a decreased number of neurons with diffuse distribution of senile plaques and neurofibrillary tangles in the neocortex and hippocampus. Severe cerebrovascular amyloid deposits were also seen. Immunostaining for amyloid was carried out using a monoclonal antibody to amyloid beta protein. The senile plaque and cerebrovascular amyloid was strongly immunoreactive to anti-beta protein antibody. Cerebral amyloid angiopathy is commonly seen in the brains with Alzheimer's disease and severe cerebrovascular degeneration secondary to heavy amyloid deposits may cause recurrent subcortical hemorrhages in the patients with this disorder.
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PMID:[An Alzheimer's disease case showing recurrent subcortical hemorrhage: an autopsy findings with immunohistochemical studies of cerebral amyloid deposits]. 280 20

Eleven patients with rheumatoid arthritis that had been refractory to conventional drug therapy were treated with total lymphoid irradiation (TLI). Followup continued for 6 months in 9 patients, 12 months in 6 patients, and 24 months in 3 patients. At 6 and 12 months post-TLI, a significant improvement in clinical disease activity was demonstrated. Side effects noted during TLI included fatigue, nausea, diarrhea, and vomiting. One patient died of cardiorespiratory arrest, 2 patients died of kidney failure secondary to generalized amyloidosis, and 1 patient died of septic shock secondary to a multilocular septic arthritis. One patient experienced 2 episodes of septic arthritis; 2 patients manifested delayed wound healing. Immunologic assessments showed consistent lymphopenia in all patients. T lymphocyte subsets decreased after TLI, and showed a transient increase at 6 months post-TLI. The suppressed mitogen responsiveness, which was noted 2 months after irradiation, was found to increase almost to the pre-TLI levels at 12 months. The observed increase in morbidity and mortality after TLI is evidence that discourages the use of this therapeutic technique, at least in its present form.
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PMID:Total lymphoid irradiation in patients with refractory rheumatoid arthritis. 293 45

Intestinal pseudo-obstruction (IP) is an uncommon disorder of gut motility which must be differentiated from mechanical intestinal obstruction. We have seen 11 such patients over the last 5 years. Characteristic symptoms, shared by mechanical obstruction, include abdominal distention and pain, nausea, and vomiting. Radiologic studies reveal dilated loops of bowel with air fluid levels. In most patients a major differentiating feature from obstruction may be the presence of diarrhea rather than obstipation. Steatorrhea is secondary to an overgrowth of anaerobic bacteria in the motionless dilated loops of bowel. IP has been associated with various disorders: in our series two patients had scleroderma, one multiple small bowel diverticula, one systemic amyloidosis, one celiac disease, and one spinal cord injury; in only two patients was the disorder considered "idiopathic." Three patients had previously undergone a jejuno--ileal bypass for morbid obesity. During the acute episode, the patients were treated symptomatically with decompression by nasogastric or rectal tube with fluid and electrolyte replacement. Malabsorption treated with broad spectrum antibiotics reversing the steatorrhea but not episodes of pseudo-obstruction. Magnesium deficiency was present in seven patients and its correction resulted in amelioration of the symptom complex. In two patients episodes of pseudo-obstruction were markedly reduced by metoclopramide which was not effective in two others.
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PMID:Chronic intestinal pseudo-obstruction. 679 59

A 59-year-old man had been known for two years to have a clearly elevated blood sedimentation reaction and intermittent arthralgia in the hand. His general condition had progressively deteriorated over the last six months, with weight loss, recurrent vomiting and constipation. At first malignant lymphoma had been suspected because numerous lymph nodes were enlarged. But a lymph-node biopsy revealed massive macrophages with PAS-positive inclusion material, diagnosed as Whipple's disease. The patient died suddenly of a heart attack on the day of diagnosis. At necropsy generalized amyloidosis with marked cardiac involvement was found. Immunohistochemically the amyloid deposits gave a strongly positive reaction to anti-AA, while other amyloid syndromes were excluded using appropriate anti-sera. This was therefore a case of reactive (AA) amyloidosis in the presence of Whipple's disease.
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PMID:[Whipple's disease with reactive (AA) amyloidosis]. 768 37

Amyloidosis confined to the duodenum is uncommon. In the present report the history of an 83-year old patient, admitted for vomiting and heavy epigastric pain, is described. Radiographic and endoscopic investigation revealed two polypoid lesions in the duodenum (D2). Biopsies showed diffuse amyloid deposition in the lamina propria, muscularis mucosae and submucosa of the duodenum as well as vascular deposits. No other localisations were documented. Amyloid tumours of the gastrointestinal tract are rare but may lead to serious symptoms.
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PMID:Intermittent subobstruction and cholestasis as complications of duodenal amyloid tumours. 775 Jul 13

We studied the results of renal transplantation in 16 patients with renal amyloidosis and in 46 controls with primary glomerulonephritis. Amyloidosis was primary in five and secondary to familial Mediterranean fever (FMF) in 11. All patients received live related donor kidneys and the majority had one-haplotype HLA match. One- and 5-year graft and patient survival rates were comparable in both groups. Moreover, the frequency of acute rejection episodes and the mean serum creatinine values were not significantly different between members of the two groups. Significant gastrointestinal symptoms in the form of nausea, vomiting, abdominal pains, and diarrhoea occurred in seven of the patients with amyloidosis (43.7%) and in only one of the controls (2%) (P = 0.001). All seven recipients with amyloidosis who developed the gastrointestinal manifestations were receiving cyclosporin and six had FMF. Maintenance colchicine treatment prevented recurrence of FMF symptoms. In one patient discontinuation of colchicine was followed by recurrence of FMF symptoms. Recurrence of renal amyloidosis was not observed in five patients subjected to Trucut graft biopsies 1, 2, 3, 18 and 72 months post-transplantation. It is concluded that live-related donor kidney transplantation is a safe procedure in patients with amyloidosis and follows a course similar to glomerulonephritis patients.
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PMID:Study of live donor kidney transplantation outcome in recipients with renal amyloidosis. 797 Jan


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