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

As long-term survival has become possible in patients with autosomal dominant polycystic kidney disease (ADPKD) with progress in hemodialysis (HD), complications by various extrarenal diseases has presented new problems. Recent experience of two rare cases of ADPKD ending fatally due to complications by polycystic liver is presented. Case 1: A 60-year-old female with a family history of ADPKD without a past history of liver disease, was diagnosed as ADPKD at the age of 45 years. Hemodialysis was started at the age of 58 years. From 6 months prior to her death, abdominal circumference increased (body height: 149 cm, abdominal circumference: 100 cm). Dyspnea, abdominal pain and anorexia appeared and she died of hepatic failure leading to cachexia. Case 2: A 76-year-old female with a family history of ADPKD without a past history of liver disease, was started on HD at the age of 73 years. Abdominal circumference was 84 cm (body height: 138 cm). She was repeatedly admitted to and discharged from the hospital due to febrile episodes. Infection of polycystic liver was complicated by DIC and she died of gastrointestinal hemorrhage. Autopsy revealed abscess in some of the cysts in the liver. Hepatic cysts most frequently complicating ADPKD so far have presented with scarcely any clinical problems. Recently, however, cases of infection of hepatic cysts, portal hypertension and hepatic insufficiency have been reported. The relationship between these hepatic diseases and the prognosis of ADPKD has received attention. Increase in the number of cases of complications similar to the present cases is anticipated.
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PMID:[Two cases of autosomal dominant polycystic kidney disease treated with hemodialysis associated with polycystic liver complications related to the cause of death]. 875 71

Peritoneovenous shunt placement has been reported as a treatment of refractory ascites by general surgeons, but without a clearly established role. The authors successfully inserted shunts under ultrasonographic and fluoroscopic guidance in 12 patients who had symptomatic refractory ascites (nine men, three women; mean maintenance duration, 88.5 d). Nine patients had advanced liver cirrhosis (five with superimposed hepatoma). Other patients had stomach cancer, colon cancer, and complicated polycystic kidney disease. The mortality rate was 83%. Causes of death included bleeding from preexisting varices, sepsis, hepatic failure, rupture of hepatoma, and disseminated intravascular coagulation. The authors describe the feasibility, technical details, and short-term results of percutaneous peritoneovenous shunt placement.
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PMID:Percutaneous peritoneovenous shunt creation for the treatment of benign and malignant refractory ascites. 1174 23

Autosomal-dominant polycystic kidney disease (ADPKD) has been known to be associated with a variety of vascular diseases. We present a hemodialysis patient with ADPKD who died of a massive intraperitoneal hemorrhage caused by the spontaneous rupture of a left gastroepiploic artery aneurysm. A 64-year-old male was admitted to our hospital with acute upper abdominal pain and hemorrhagic shock. An abdominal angiography showed three aneurysms and the source of hemorrhage was assumed to be the left gastroepiploic artery aneurysm. The patient died of severe metabolic acidosis and disseminated intravascular coagulation (DIC) on the second hospital day. At autopsy, there was massive bleeding into the abdominal cavity, and pathological examination of the left gastroepiploic artery aneurysm revealed a dissecting aneurysm. This is the first case describing a rupture of a gastroepiploic aneurysm in a patient with ADPKD.
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PMID:Spontaneous rupture of a left gastroepiploic artery aneurysm in a patient with autosomal-dominant polycystic kidney disease. 1573 59

Nilotinib, a second-generation tyrosine kinase inhibitor with 20- to 30-fold greater potency than imatinib, was developed to overcome imatinib intolerance or resistance. Recently, nilotinib has been approved as a first-line treatment for chronic myelogenous leukemia in the US and Japan. Tumor lysis syndrome (TLS) is an extremely rare adverse event that can occur during treatment with nilotinib, with only a few reported cases to date. Herein, we report two patients who developed TLS soon after the start of treatment with nilotinib. While in the first case, which co-presented with underlying mild-to-moderate renal insufficiency due to polycystic kidney disease, the TLS resolved on discontinuation of the drug, the second patient, who had an exceedingly high white blood cell count, presented with disseminated intravascular coagulation and severe liver injury triggered by TLS that developed after the start of nilotinib treatment, and died of multiple organ failure. Therefore, caution is necessary when this drug is used in the first-line setting in patients with renal insufficiency or a high tumor burden.
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PMID:Tumor lysis syndrome soon after treatment with hydroxyurea followed by nilotinib in two patients with chronic-phase chronic myelogenous leukemia. 2364 69

A 62-year-old man had a history of acute aortic dissection (Stanford type A) and had been diagnosed with polycystic kidney disease three years earlier, and then developed end-stage renal failure. He was referred with chief complaints of difficult hemostasis and consecutive hemorrhagic episodes at the puncture site of the shunt soon after dialysis introduction. We suspected chronic disseminated intravascular coagulation (DIC) due to mild thrombocytopenia and a fibrinolytic system abnormality. Plasma factor XIII activity was decreased, but no inhibitor was detected. In addition, contrast-enhanced computed tomography showed exacerbation of a dissecting aortic aneurysm. We finally diagnosed chronic DIC and secondary factor XIII deficiency associated with the aortic aneurysm. We selected treatment involving recombinant human soluble thrombomodulin (rTM) because he was on maintenance dialysis and required long-term follow-up bofore the operation. Hemostatic function improved with regular administration of rTM, and was well-controlled preoperatively.
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PMID:Successful treatment of chronic disseminated intravascular coagulation using recombinant human soluble thrombomodulin in a dialysis patient with dissecting aortic aneurysm. 2550 11