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)

Primary intestinal lymphangiectasia (Waldmann's disease) is characterized by protein-losing enteropathy occurring more frequently in childhood. Chronic diarrhea and diffuse edema are the main clinical manifestations. Peripheral lymphedema may also be associated. Lymphedema is usually present at the time of diagnosis or appears later in the course of the disease. We report the observation of a 31-year-old man suffering from an upper, lower limb and genital lymphedema many years before diagnosis of primary intestinal lymphangiectasia was established. Lower limb lymphoscintigraphy confirmed lymphedema and duodenal biopsies lymphangiectasia. Hypoproteinemia, lymphopenia and hypogammaglobulinemia were also noted. Treatment of lymphedema included low stretch bandaging and elastic stocking. No dietary management with a low-fat diet was added. Search for primary intestinal lymphangiectasia with biological parameters would be useful when primary lymphedema is present. Especially since primary intestinal lymphangiectasia may be complicated by occurrence of B cell lymphoma.
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PMID:[Limb lymphedema as a first manifestation of primary intestinal lymphangiectasia (Waldmann's disease)]. 1522 6

Recently, plasma exchange (PE) has been added to the treatment regimen for patients with steroid-, cyclophosphamide-, and cyclosporine-resistant nephrotic syndrome. This is a case report of a female patient with severe acute renal failure (ARF) during the relapse of steroid-resistant nephrotic syndrome (SRNS) who recovered completely after PE and became steroid-sensitive in further follow-up of 48 months. An 8-year-old girl was referred to Nephrology Department of the University Children's Hospital due to relapse of SRNS complicated with ARF. Her nephrotic syndrome (mesangioproliferative glomerulonephritis) was diagnosed at the age of 17 months. During the following 6 years, she was given several therapeutic regimens including pulse prednisolone, cyclophosphamide, Cyclosporine (CyA), but she continued to have frequent relapses and during the last six months she was steroid- and cyclosporine-resistant. Three days before admission, she was febrile, had cellulites of the lower abdominal wall, diarrhea, vomiting, hypovolemic shock with generalized edema, severe hypoproteinemia and hypoalbuminemia. In a local hospital, she was treated with fresh frozen plasma, albumin, methylprednisolone, furosemide and antibiotics, but she became anuric and was referred to our hospital. There were no signs of hemolysis. Anuria lasted for 12 days. She was discharged after 42 days in remission with normal GFR. Principal treatment included: 13 sequential hemodialysis sessions (30% of body weight was removed as excess volume), 6 PE, corticosteroids, CyA, ACE inhibitor, antibiotics, antimycotics, and cimetidine. Six PE sessions were performed every other day. In further 48-month follow-up, while under the treatment of CyA the patient had a few steroid-sensitive relapses, the first being 6 months after PE. The second kidney biopsy showed focal segmental glomerulosclerosis with no signs of apparent CyA nephrotoxicity. "Malignant" course of disease in our patient was a good reason to introduce PE into the treatment. Since PE was the only additional mode of treatment, it is believed that its effect was crucial for milder activity of the disease.
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PMID:[The benefit of plasmapheresis in a patient with steroid-resistant nephrotic syndrome and anuria--long-term follow-up]. 1561 78

A 2.5-yr-old, intact male Japanese macaque (Macaca fuscata) was observed to have a thickened ileum during exploratory laparotomy. Lawsonia intracellularis-associated proliferative enteritis was diagnosed using histopathology (Warthin-Starry stain), immunohistochemistry, and polymerase chain reaction analysis of the ileal biopsy. The animal developed transient diarrhea and severe hypoproteinemia 16 days after surgery but recovered with intensive treatment using azithromycin. Given the fact that very specific tests are required for identifying this organism, L. intracellularis may be underdiagnosed in nonhuman primates.
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PMID:Proliferative enteritis associated with Lawsonia intracellularis in a Japanese macaque (Macaca fuscata). 1573

This report concerns two patients with severe protein losing enteropathy and refractory diarrhea due to AA amyloidosis who were successfully treated with corticosteroid and octreotide. In these patients, biopsied tissues from the gastrointestinal (GI) tract showed extensive deposition of AA amyloid, which was caused by rheumatoid arthritis in one case and was of unidentified etiology in the other. Both patients manifested severe diarrhea unresponsive to conventional treatment with hypoproteinemia, and protein leakage from the small intestine to the ascending colon was confirmed by 99mTc-diethylene triamine pentaacetic acid human serum albumin (HSA-D) scintigraphy. Soon after starting a long-acting somatostatin analogue, octreotide, with co-administration of oral prednisolone, their general status improved in parallel with a rapid decrease in the volume of watery diarrhea and an increase in serum levels of albumin and IgG. Also on 99mTc-HSA-D scintigraphy protein leakage from the GI tract was apparently decreased in both patients. Combination therapy with a somatostatin analogue and corticosteroid may be effective for protein losing enteropathy with intractable diarrhea ascribable to GI amyloidosis. Because of the lack of specific therapies in this serious clinical situation, the described therapy should actively be considered as a therapeutic option not only in AA amyloidosis, but also in other types of systemic amyloidosis.
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PMID:Severe protein losing enteropathy with intractable diarrhea due to systemic AA amyloidosis, successfully treated with corticosteroid and octreotide. 1607 11

A 9-year-old female Pug was presented to us with chronic diarrhea. Hematologic findings indicated severe hypoproteinemia and hypoalbuminemia, and endoscopy revealed severe edema of the duodenal mucosa. Based on these results and on additional histopathological findings, we made a diagnosis of protein-losing enteropathy caused by lymphocytic-plasmacytic enteritis with lymphangiectasia. The dog was initially treated with prednisolone and cyclosporine. This treatment regimen was not effective. However, when methotrexate was substituted for cyclosporine, progress was obtained and the diagnosis was confirmed.
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PMID:A case of protein-losing enteropathy treated with methotrexate in a dog. 1667 35

Coinfection with Ehrlichia canis, Babesia canis, Hepatozoon canis, Isospora spp., Giardia spp., and Dipylidium caninum were detected in a 6-week-old dog. The effect of multi-pathogen infection was a fatal combination of gastrointestinal and hematologic abnormalities, including diarrhea, vomiting, anorexia, distended painful abdomen, intussusception, severe thrombocytopenia, anemia, and hypoproteinemia.
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PMID:Coinfection with multiple tick-borne and intestinal parasites in a 6-week-old dog. 1761 60

A 63-year-old woman was referred and admitted to our department for further examination of protein-losing enteropathy (PLE), which was diagnosed by alpha-anti trypsin test. Her symptoms were frequent vomiting, watery diarrhea and hypoproteinemia. Although intensive examination for PLE was performed in her previous hospital, the origin of the disease was not detected. Abdominal computed tomography revealed diffuse enlargement and swelling of the intestine wall and a 5-cm diameter mass with unclear margin, which involved the mesenteric arteries and veins. Total colonoscopy showed a diffuse edematous lesion with hemorrhage at the terminal ileum. Enteropathy-type T-cell lymphoma (ETL) was diagnosed based on a biopsy of the lesion and CD45 gating analysis. Immediate start of chemotherapy (CHOP) led to a transient regression of the tumor even though her symptoms were not improved. During the second cycle of CHOP, the patient died of massive hemorrhage throughout the intestine. The pathological study revealed that intraepithelial CD3-positive clonal T-cells were present in the lesion, indicating that this case could be associated with celiac disease. In light of the histological findings, we concluded that this was a case of ETL associated with celiac disease, which is extremely rare in Japan.
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PMID:Enteropathy-type T-cell lymphoma that was pathologically diagnosed as celiac disease. 1767 73

Mycobacterium avium subsp. paratuberculosis (Map) is the causative agent of paratuberculosis or Johne's disease, a chronic enteric disease of domestic ruminants as well as some nondomestic ruminants. Paratuberculosis is characterized by a protracted subclinical phase followed by clinical signs such as diarrhea, weight loss, and hypoproteinemia. Fecal shedding of Map is characteristic of both the subclinical and clinical phases, and it is important in disease transmission. Lesions of paratuberculosis are characterized by chronic granulomatous enteritis and mesenteric lymphadenitis. Animal models of paratuberculosis that simulate all aspects of the disease are rare. Oral inoculation of 9-day-old white-tailed deer (Odocoileus virginianus) on 3 June 2002 with 1.87 x 10(10) colony-forming units of Map strain K10 resulted in clinical disease (soft to diarrheic feces) as early as 146 days after inoculation; lesions consistent with paratuberculosis were observed in animals at the termination of the study. Intermittent fecal shedding of Map was seen between 28 and 595 days (4 March 2004) after inoculation. These findings suggest that experimental oral inoculation of white-tailed deer fawns may mimic all aspects of subclinical and clinical paratuberculosis.
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PMID:Experimental infection of white-tailed deer (Odocoileus virginianus) with Mycobacterium avium subsp. paratuberculosis. 1798 54

A 76-year-old woman with a 5-mo history of recurrent diarrhea and generalized edema was admitted to our hospital. Colonoscopy revealed edematous mucosa, and histopathological examination was compatible with collagenous colitis. Protein leakage from the colon, particularly in the ascending portion, was identified on 99mTc-human serum albumin scintigraphy. Collagenous colitis associated with protein-losing enteropathy (PLE) without small bowel disease was diagnosed. Prednisolone treatment ameliorated diarrhea and hypoproteinemia. Collagenous colitis should be included in the differential diagnosis of chronic diarrhea with hypoproteinemia for appropriate management.
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PMID:A unique case of collagenous colitis presenting as protein-losing enteropathy successfully treated with prednisolone. 1893 90

A 67-year-old man with nausea, appetite loss, frequent diarrhea and severe weight loss presented with alopecia, skin hyperpigmentation and onychodystrophy. Laboratory investigations showed mild anemia, hypoproteinemia and hypoalbuminemia. Colonoscopy identified the numerous, hyperemic and sessile polyps with mucous exudation of various sizes throughout the colorectum. The ileocecal valve was substantially swollen. Magnified chromoendoscopy revealed sparsely distributed crypt openings with widening of the preicryptal space without destruction in the affected lesions. Upper gastrointestinal endoscopy revealed multiple small, reddish, and sessile polyps in the duodenum and Helicobacter pylori-associated gastritis. Histopathological examination of the colonic polyps revealed cystic dilatation and elongation of scattered glands with epithelial hyperplasia and stromal edema and inflammatory cell infiltrates. Thus, a diagnosis of Cronkhite-Canada syndrome was made. The patient was given clarithromycin, amoxicillin and lansoprazole, resulting in negative (13)C-urea breath tests. Three months later, his clinical symptoms and edema of the legs resolved with normalization of serum total protein and albumin levels and return to his previous body. The ectodermal abnormalities were resolved 8 months later. On repeat colonoscopic examinations, there was progressive remission of the duodenal and colorectal polyposis, leaving scattered pedunculated polyps in the transverse and ascending colon and on the almost normal-appearing ileocecal valve. At the follow-up magnifying endoscopic examination 8 months later, small round or round-oval pits were densely and regularly distributed.
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PMID:A case of Cronkhite-Canada syndrome: remission after treatment with anti-Helicobacter pylori regimen. 1894 92


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