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Results for “COLLAGEN DISEASES”

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At least 73 records · Page 4Linked to original sources
PubMed1994

[Three cases of Castleman's disease mimicking the features of collagen disease].

We report three cases of Castleman's disease mimicking the features of collagen disease. Case 1: A 39-year-old woman presented with intermittent arthralgia and fever. Laboratory findings were positive results for antinuclear antibody (80x speckled type), the LE test, anti-SSA antibody, anti-RNP antibody, and Coombs test. The patient was suspected to have systemic lupus erythematosus (SLE) or Sjögren syndrome, but a lymph node biopsy revealed the plasma cell type of Castleman's disease. Steroid treatment led to resolution of her symptoms. Case 2: A 60-year-old man with mixed type Castleman's disease had proteinuria with renal dysfunction, autoimmune thrombocytopenia, antinuclear antibody, anti-RNP antibody, anti-DNA antibody and anti-cardiolipin antibody. The patient was suspected to have SLE but cervical lymph node biopsy revealed the mixed type of Castleman's disease. Symptoms were not controlled with steroid therapy. He developed renal failure that required for hemodialysis and died of gastrointestinal bleeding due to severe thrombocytopenia. Case 3: A 46-year-old woman had Raynaud's phenomenon, sclerodactylia, and nail fold bleeding. Laboratory tests were revealed positive for antinuclear antibody, anti-ENA antibody, and LE cell preparation. Radiographic study showed multiple masses in the retroperitoneal spaces, which necessitated laparotomy. Firstly, the patient was suspected to have systemic sclerosis or mixed connective tissue disease (MCTD). A biopsy revealed the hyaline-vascular type of Castleman's disease. The serum level of IL-6 by ELISA was high in all of three cases. In case 1, symptoms improved and the IL-6 level normalized after steroid treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMedDate not supplied

The feasibility of epidural anesthesia without endotracheal intubation for abdominal surgery in patients with collagen diseases.

BACKGROUND/AIMS: Patients with collagen diseases are generally regarded as high-risk surgical candidates. MATERIAL AND METHODS: To evaluate the feasibility of epidural anesthesia and to determine the risk factors in abdominal surgery for patients with collagen diseases, 20 patients with collagen diseases who underwent elective abdominal surgery were examined for their surgical outcomes and clinical characteristics. Among the 20 cases, 12 received epidural anesthesia alone without endotracheal intubation, 3 received general anesthesia only, 4 received general anesthesia with epidural anesthesia and one received lumbar anesthesia. RESULTS: Only one patient receiving epidural anesthesia died after operation. The mortality in patients receiving epidural anesthesia was 8.3% (1/12) while the overall mortality was 5.0% (1/20). No significant difference was observed either in the mortality or incidence of postoperative complications among the 4 groups according to the method of anesthesia. Patients with a dysfunction of the vital organs more often had postoperative complications than those without a dysfunction of the vital organs (p = 0.043). CONCLUSIONS: Although only a small number of patients were included in this study, these results suggested that 1) elective abdominal surgery can be as safely performed under epidural anesthesia alone as with general anesthesia even for patients with collagen diseases, and 2) the patients with collagen diseases, who preoperatively showed a dysfunction of the vital organs, might be at a higher risk for abdominal surgery.

Adult↗
PubMed1967

Viral plasmacytosis (Aleutian disease) of mink resembling human collagen disease.

A disease in mink has been discovered that has many of the features of collagen diseases in man. Affected animals suffer from wasting with leukopenia and thrombocytopenia as well as plasma cell infiltration, hypergammaglobulinemia, glomerulonephritis, arteritis and amyloidosis. Cell-free filtrates and ultracentrifugates from diseased animals induced the disease in normal mink, and aleutian genotypes were unusually susceptible to infection. This genotype was characterized by abnormal lysosomal structures in all the granule-forming cells, resembling the Chediak-Higashi syndrome of man. Anti-gamma-globulin factors similar to human rheumatoid factors were reported, although tests for antibodies such as ANF and LE factors have been negative. Arteritis and glomerulonephritis lesions stained positively for gamma-globulin, and Coombs-type sensitized red cells have been detected in the majority of affected mink. Some mink develop a monodispersion of hypergammaglobulinemia resembling the serum protein changes in human myeloma. These studies highlight genetic, immunological and microbiological causative factors in a mink disorder resembling human collagen disease.

Alaska↗
PubMed2006

[Type IX collagen diseases].

Type IX collagen is a structural protein which consists of the cartilage collagen II/IX/XI heteropolymer. Transgenic mice overexpressing a mutant alpha 1 (IX) collagen chain in cartilage develop a chondrodysplasia-like phenotype associated with early-onset osteoarthritis and spondylosis. Subsequent identifications of the human type IX collagen diseases proved the correctness of the work on mice. Now we know that type IX collagen gene mutations cause multiple epiphyseal dysplasia, an osteochondrodysplasia which frequently features early onset osteoarthritis. Polymorphic variants of the type IX collagen genes are associated with lumbar disc diseases. It is getting more important to understand the biological roles of collagen IX in the musculoskeletal system.

Animals↗
PubMed1977

Collagen diseases and the biosynthesis of collagen.

Increasing knowledge concerning the molecular structure of collagen and the steps involved in its biosynthesis provides the basis for a new look at the collagen diseases. Some, like the Ehlers-Danlos syndrome, are now known to be the expression of primary molecular defects; others, such as scurvy and scleroderma, appear to be secondary to some process that disrupts normal controls over collagen synthesis or deposition.

Amino Acid Sequence↗