Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 51-year-old male patient admitted to the hospital because of colic-like abdominal pain, paralytic ileus, anal bleeding and microhaematuria with proteinuria, developed an intestinal ischemia with a serum lactate level of 6.3 mmol/l. An occlusion of the large vessels was excluded angiographically. Perfusion disorders were detected both endoscopically and histologically in the upper gastrointestinal tract and in the terminal ileum. When after two days a palpable purpura appeared on the anterior of both feet, a vasculitis type Schoenlein-Henoch was suspected and treated with high doses of steroids, resulting in decreasing symptoms. From the point of admittance, a nephritic urinary sediment had been apparent, and the renal affliction developed into a nephrotic syndrome without notable reduction in the glomerular filtration rate. On the 13th day of treatment the patient-being on a reduced dose of steroids-suffered from a severe relapse; however, this responded favorably to an increase of the dosage. The kidneys required approximately one year for complete recovery. Based on this case, the Schoenlein-Henoch purpura syndrome and its differential diagnosis are presented, particularly with respect to gastrointestinal symptoms and in view of the pertinent literature.
 ...
PMID:[Schoenlein-Henoch purpura with intestinal involvement]. 949 May 51

Two girls with mixed connective tissue disease (MCTD) were treated in our hospital in the past 5 years. Patient 1, a 10-year-old girl presenting with migratory arthralgia, had an initial diagnosis of juvenile rheumatoid arthritis. Muscle weakness with elevated levels of creatine kinase and liver enzymes, sclerodactyly, Raynaud's phenomenon and heliotrope sign developed subsequently in the following 3 years. Patient 2, a 13-year-old girl, had been treated for suspected systemic lupus erythematosus since 9 years of age. She presented with lymphadenopathy, arthralgia, pericardial effusion, and paralytic ileus. The symptoms waxed and waned. Sclerodactyly, Raynaud's phenomenon, proteinuria, and hypertension were also noted. Both patients had high serum titers of antinuclear antibody (speckled pattern, 1:5120) and were seropositive for antiribonuclear protein antibody. Intravenous immunoglobulin, prednisolone, cyclosporine A, and nonsteroidal anti-inflammatory drugs (NSAIDs) were given to patient 1. Patient 2 received cyclosporine A, prednisolone, and methylprednisolone pulse therapy. The disease has been well controlled for 2 years by low-dose immunosuppressants and NSAIDs. MCTD is a rare juvenile rheumatic disease: early identification and appropriate treatment can improve the disease outcome.
 ...
PMID:Childhood mixed connective tissue disease. 1077 31

We present the case of a patient with lupus enteritis accompanied by both lupus cystitis and lupus nephritis without a history of systemic lupus erythematosus. The patient had a 2-month history of diarrhea and pollakiuria and was admitted to our hospital. Physical examination showed abdominal tenderness. Laboratory data revealed reductions in both total protein and albumin, elevated serum creatinine levels, and elevated antinuclear and anti-Smith antibodies. Urinalysis revealed proteinuria. Stool and urine cultures were negative. Abdominal computed tomography revealed diffuse edematous wall thickening, dilatation of the small intestine suggesting paralytic ileus, and irregular wall thickening of the urinary bladder. Histological results of the cystic and renal biopsies revealed interstitial cystitis and membranous nephropathy which were suggestive of lupus cystitis and lupus nephritis, respectively. The patient's gastrointestinal symptoms were finally diagnosed as resulting from lupus enteritis. After initiating 30 mg oral prednisolone daily, the diarrhea and pollakiuria subsided, and renal function became normal. Lupus enteritis should always be considered in the differential diagnosis when patients complaining of chronic diarrhea of unknown etiology are encountered.
 ...
PMID:Involvement of lupus enteritis in a patient with lupus cystitis and nephritis. 1977 78

Objective To clarify the underlying diseases, clinical manifestations, and treatment strategies for Amyloid A (AA) amyloidosis (AAA) in Japanese patients. Methods We conducted a survey on Japanese patients with AAA treated between January 1, 2012, and December 31, 2014. Results A total of 199 patients with AAA were included in the present study. The underlying diseases of AAA were rheumatoid arthritis (60.3%), uncharacterized inflammatory disorders (11.1%), neoplasms (7.0%), other rheumatic diseases (6.5%), inflammatory bowel diseases (4.5%), chronic infection (4.5%), Castleman's disease (4.0%), and autoinflammatory diseases (2.0%). The clinical manifestations at the diagnosis of AAA were moderate to severe renal dysfunction (46.2%), moderate to severe proteinuria (30.7%), intractable diarrhea (32.2%), melena (4.5%), paralytic ileus (3.5%), heart failure (11.6%), cardiac conduction disturbances (10.1%), arrhythmia (5.5%), and hypothyroidism (11.6%). Diagnostic biopsies were performed most frequently in the gastrointestinal tract (66.3%), followed by the kidneys (22.1%), heart (5.5%), abdominal fat (4.0%), and others (3.0%). Biologics were used to treat 97 patients with AAA (48.7%). Tocilizumab (TCZ) was administered to 66 patients, with 95.5% showing good responses. Anti-TNF agents were administered to 27 patients, with 74.1% showing good responses. The treatment effects of TCZ were significantly superior to those of anti-TNF agents (p<0.007). Conclusion The most common underlying diseases of AAA were rheumatic diseases. Uncharacterized inflammatory disorders and neoplasms were also frequently observed in patients with AAA. Renal and gastrointestinal manifestations were common and important for the diagnosis of AAA, with cardiac manifestations also being of significance. Biologics, particularly TCZ, were effective therapeutic modalities.
 ...
PMID:First Nationwide Survey of 199 Patients with Amyloid A Amyloidosis in Japan. 3010 21