Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020639 (hypoproteinemia)
 1,134 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two personal cases are compared with CLARKSON's case, enabling one to describe a syndrome arising in adults without a family history, and characterised by episodes of repeated shock (cyclical shock) with hemoconcentration and hypoproteinemia. The physiopathological mechanism is an acute hyperpermeability. Two cases out of three took a fatal course, with a negative autopsy. In the three cases, the presence of light kappa chain IgG type monoclonal dysglobulinemia was proven. The diagnosis of multiple myeloma can be excluded. Comparison of this syndrome with periodic disease and primary amyloidosis can be envisaged.
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PMID:Cyclical shock with hyperglobulinemia. 123 76

A simian acquired immunodeficiency syndrome (SAIDS) associated with retroperitoneal fibromatosis (RF) has been observed in several species of macaque at the Washington Regional Primate Research Center. Clinical signs were recurrent diarrhea, weight loss, mesenteric lymphadenopathy, and opportunistic infections. Most affected macaques in the later stages of illness showed marked immunodeficiency. Response of peripheral blood mononuclear cells to mitogens was impaired significantly. There was sharply depressed primary and secondary antibody response to the T-cell dependent antigen, bacteriophage phi X174. Affected monkeys did not switch from IgM to IgG antibody following a secondary immunization, as did normal macaques. Twenty-four (67%) of 36 affected animals with progressive RF or deteriorated stages of illness had hypoproteinemia and hypoalbuminemia. Quantitative serum immunoglobulins of 23 cases showed that eight (35%) had hypogammaglobulinemia, six (26%) had hypergammaglobulinemia, and the remainder (39%) were within the normal range. Opportunistic infections were predominantly bacterial pathogens. Type D retrovirus appeared to be closely associated with RF-affected macaques (12/12 or 100%). The case fatality rate (including animals sacrificed after prolonged illness) was 98%. The leading cause of death was due directly to RF lesions in 43%, to enterocolitis in 36%, septicemia in 12%, amyloidosis in 5%, and malignant lymphoma (2%). Clinical, immunologic and pathologic changes reveal an acquired immunodeficiency syndrome that has many similarities to human AIDS. SAIDS and RF may be a useful model for studying human AIDS.
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PMID:Retroperitoneal fibromatosis and acquired immunodeficiency syndrome in macaques: clinical and immunologic studies. 348 18

Amyloidosis was diagnosed in 6 Holstein cows that were examined because of chronic intractable diarrhea. Besides diarrhea, the chief finding was a nephrotic-like syndrome, in that there was edema, hypoproteinemia, and proteinuria. Other consistent clinicopathologic abnormalities were hyperfibrinogenemia, low-normal serum calcium content or hypocalcemia, hypomagnesemia, prolonged bromosulphalein half time, high serum urea nitrogen concentration, high serum creatinine concentration, and low urine specific gravity. Foci of inflammation including traumatic reticuloperitonitis, traumatic pericarditis, salpingitis, mastitis, and metritis were found. There was histologic evidence of amyloid in the kidneys, liver, adrenal glands, and spleen. The iodine-sulfuric acid test for amyloid was positive in 2 cows. The Congo red dye test for amyloid was positive in 2 other cows. In spite of supportive care, all the cows either died naturally or were euthanatized. Because foci of inflammation were found in each cow, it was concluded that the most likely classification of amyloidosis in these cases would be reactive systemic amyloidosis and that the major amyloid fibril protein would be type AA.
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PMID:Amyloidosis in six dairy cows. 651 26

A 50-year-old-man with peripheral edema and muscle cramps without proteinuria as presenting symptoms was found to have hypoproteinemia due to amyloidosis of the stomach. After failure to control the symptoms with diuretics, oral calcium supplement and colchicine, large doses of intact gamma-globulin (250 mg/kg bodyweight, total, 3 separate infusions every other day) infused i.v., helped to control the symptoms for more than 8 months.
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PMID:GI amyloidosis treated with megadoses of i.v. gammaglobulin. 805 16

A large basal cell carcinoma, 39 x 26 cm in size, is presented as second in size only to the largest basal cell carcinoma documented (40 x 30 cm), reported by Beck and co-workers (1). A 61-year-old Japanese male visited our clinic with a huge ulcerating tumor on the back. He had hidden the tumor for the previous 30 years. The tumor was histologically confirmed as basal cell carcinoma. The condition was associated with anemia, hypoproteinemia, and dyspnea, and with systemic amyloidosis in the skin, in the lymph nodes, and in the intestinal canal. On admission, the tumor had metastasized to the regional lymph nodes, and, about two years after the first operation, there were metastases to bone and lung, leading to death due to respiratory failure.
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PMID:Giant basal cell carcinoma associated with systemic amyloidosis. 867 23

In order to examine the clinical characteristics and genetic background of secondary amyloidosis associated with rheumatoid arthritis, we analyzed clinical features and HLA typing of 85 patients in a multicenter study. Eighty-five patients with secondary amyloidosis associated RA were studied. The diagnosis of secondary amyloidosis were made on histological findings by biopsy or autopsy. The most common biopsy site was gastrointestinal tract (79.5%). Clinical symptom and the frequency at the time of diagnosis were; diarrhea (35 cases), abdominal pain (22 cases) and vomiting and nausea (16 cases). Abnormalities and the frequency in a laboratory test included proteinuria (49 cases), increased serum creatinine (32 cases), anemia (30 cases) and hematuria (15 cases). Twenty-eight patients were dead and 57 patients were alive at the time of the study. The average duration between diagnosis of amyloidosis and death was 19.4 +/- 18.5 (SD) months among the dead patients. The average duration after diagnosis of amyloidosis was 24.2 +/- 19.5 (SD) months in surviving patients. The causes of death were renal failure complicated with heart failure (6 patients), heart failure alone (3 patients) and renal failure alone (2 patients). Fifty-nine patients in the control group who were negative to amyloid deposition on biopsies at more than one site in the gastrointestinal tract, were clinically compared with patients in the amyloidosis group. No difference were noted in the age of RA occurrence and the stage between the two groups. As to the class, however, the number of patients with severe functional disorder (class 3 or severe) was larger in the amyloidosis group. There were no significant difference between the two groups in Lansbury's activity index. On hematology, biochemistry and urinalysis, the incidences of increased white blood cell count, anemia, increased platelet count, increased serum creatinine, hypoproteinemia, hypoalbuminemia, increased IgA, and increased urine and blood BMG were statistically significantly higher in the amyloidosis group than in the control group. HLA-A, -B, -C, and DR-locus antigens were compared in the 53 patients in the amyloidosis group and in the 59 subjects in the control group. There were no significant differences in frequency of HLA-A, and -B antigens between two groups. Frequency of CW7 antigen was significantly decreased in the amyloidosis group (13.2%) than in the control group (39.0%). Frequency of DR1 antigen was decreased in the amyloidosis group (3.8%) than in the control group (22.0%), although the difference was not significant. These findings suggest the possible involvement of genetic factors in the occurrence of amyloidosis. It is suggested that the occurrence of amyloidosis is suppressed by some genes which are linked with CW7 antigen.
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PMID:[Clinical characteristics and genetic background of secondary amyloidosis associated with rheumatoid arthritis in Japanese]. 871 35

A 77-year-old woman was hospitalized repeatly due to frequent hemoptysis and production of bloodly sputum for several years. Bronchography in 1989 revealed bronchiectasis. She had complained of abdominal pain and diarrhea since 1991, and her urine was first positive for protein in 1992. She was admitted to our hospital in October 1992 because of edema, anemia, and hypoproteinemia. Despite treatment, renal dysfunction and the gastrointestinal disorder progressed and she died in January 1993. An autopsy revealed diffuse depositions of amyloid in many organs, especially in the kidney and the gastrointestinal tract. This amyloid protein was identified as AA protein, which was suggestive of secondary amyloidosis. Bronchiectasis appears to have been the disease underlying this patient's amyloidosis.
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PMID:[Amyloidosis secondary to bronchiectasis]. 875 22

We report a 51-year-old man diagnosed as having immunotactoid glomerulopathy (IT) who achieved partial remission after approximately 1 year of a low-dose prednisolone regimen. On admission, he was noted to show proteinuria, hypoproteinemia, and hypocomplementemia. On electron microscopy of the renal biopsy specimen, the mesangial and subendothelial areas were expanded because of the electron-dense deposits, which were represented by mostly straight and nonbranching hollow microtubule structures. The microtubular width was on average 22.0 nm. Clinical and histological findings did not support the diagnosis of amyloidosis, cryoglobulinemic glomerulonephritis, systemic lupus erythematosus, or paraproteinemias. Under treatment with oral prednisolone, 4 months later, the patient's serum albumin level increased from its lowest level of 2.3 to 3.6 g/dL, and CH50 from the lowest level of less than 6.3 to 32.4 U/mL. A 24-hour collection of urine showed that the protein had decreased from its highest level of 3.9 g to 2.0 g. This case suggests the effectiveness of long-term, low-dose prednisolone therapy for IT.
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PMID:Immunotactoid glomerulopathy: report of a case. 921 17

A 63-year-old woman who started to have polyarthralgia in December 1993 has been diagnosed as rheumatoid arthritis (RA) and treated with muscular injection of gold sodium thiomalate. She began to have nausea, vomiting, anorexia and watery diarrhea in October 1995. A year later, she had to receive intravenous infusion on admission since more frequent watery diarrhea occurred more than ten times within a day. On admission in our hospital in December 1996, she had proteinuria in addition to gastrointestinal symptoms. The biopsy specimen from stomach, duodenum and kidney proved systemic amyloidosis associated with RA. In spite of steroid-pulse, dimethyl sulfoxide (DMSO) and colchicine therapy, profound proteinuria in nephrotic syndrome was continued in association with hypoproteinemia, anasarca and renal failure. She was treated on hemodialysis and intravenous hyperalimentation (IVH) until November 1997 when A-V shunt operation on left forearm was performed. However, the shunt was not available for HD and she suffered from septicemia and died on December 1997. This patient was a rare case of secondary systemic amyloidosis associated with RA in early clinical course.
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PMID:[A case of secondary systemic amyloidosis associated with rheumatoid arthritis after 3-year disease duration]. 1033 14

On August 14, 2001, a 76-year-old woman with a history of rheumatoid arthritis was admitted to our hospital with fever, cough, dyspnea and diarrhea. On admission, her chest radiography showed pleural effusion on the right side, and thoracocentesis was used to diagnose empyema. The patient underwent pleural drainage and received antibiotics. Alpha-Streptococcus was detected in both aerobic and anaerobic cultures of the pleural effusion. After 2 weeks of therapy, her empyema had improved; but her diarrhea, which had started 1 week before admission, had worsened, and her hypoproteinemia had progressed. Examination of the fecal clearance of alpha-1-antitrypsin and biopsied rectal material revealed that the diarrhea was caused by protein-losing enteropathy due to gastrointestinal amyloidosis secondary to rheumatoid arthritis. The patient was treated with steroids, but developed an additional infectious disease and died on September 29, 2001. In this case, she suffered from various infectious diseases including empyema and fungus infections. It has been reported that protein-losing enteropathy accompanies abnormalities in the immune system, by the loss of immunoglobulins and lymphocytes from the gut. We therefore suspect that protein-losing enteropathy due to gastrointestinal amyloidosis caused this patient's empyema.
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PMID:[An autopsy case of protein-losing enteropathy due to gastrointestinal amyloidosis, occurring in empyema]. 1260 5


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