Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0012739 (disseminated intravascular coagulation)
 8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The present status of regular dialysis and renal transplantation in patients with end-stage renal disease secondary to primary hyperoxaluria is reported. Clinical studies include one personal case with an 18-month period of follow-up and data concerning thirteen patients treated in 10 centres in Europe which have been collected through a cooperative survey carried out with the assistance of Registry of the EDTA. On January 1 st, 1974, mean survival of patients with oxalosis treated by RDT was 30.4 months (range 6 to 102 months). Five cadaveric renal transplants have been performed in four patients; two patients are surviving with grafts functioning for 18 and 45 months. Dialysis and/or transplantation should be performed in patients with oxalosis early enough to prevent ischaemic, cardiac and neuromusclar complications which occur at the end-stage of the disease. Evidence for blood coagulation disorders, particularly chronic consumption coagulopathy, should be investigated for with adequate laboratory methods and long-term heparin therapy instituted if necessary. No convincing reports concerning the efficiency of the various drugs which have been tried out to reduce the biosynthesis of oxalic acid in patients with oxalosis have been issued to this date.
 ...
PMID:Terminal renal failure due to oxalosis in 14 patients. 110 57

A case of malignant rhabdoid tumor (MRT) occurring as a primary hepatic neoplasm in a 12-month-old Japanese female infant is presented. The patient had a slight fever for 2 weeks and presented with a palpable mass in her left hypochondrial region. After admission, the hepatic artery was embolized due to intra-abdominal hemorrhage arising from the tumor. The patient received chemotherapy with cisplatin, cyclophosphamide and adriacin. Despite treatment, the patient developed dyspnea, pancytopenia and disseminated intravascular coagulation. Rupture of the tumor resulted in death within 3 weeks. A limited abdominal autopsy revealed that the liver weighed 1240 g and was occupied by multiple hemorrhagic and/or necrotic tumor nodules. Histologically, neoplastic cells had an abundant eosinophilic cytoplasm containing paranuclear inclusions, and vesicular nuclei with a centrally located prominent nucleolus. Ultrastructurally, the cytoplasmic inclusions were composed of whorled filaments measuring 10 nm. Immunohistochemically, almost all of the neoplastic cells were positive for vimentin and cytokeratins (CK) 8 and 18, some were positive for CK 7 and 19, while none were positive for CK 1, 10, 13-17 and 20. The tumor cells did not express desmin, myoglobin, and alpha-fetoprotein. We found 18 cases of MRT of the liver published in English language literature and then, adding the present case, we summarized the 19 cases. Hepatic MRT is an uncommon neoplasm. However, it should be considered in the differential diagnosis of an aggressive liver neoplasm in childhood.
 ...
PMID:Malignant rhabdoid tumor of the liver: case report and literature review. 1526 Aug 54