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)

Kasabach-Merritt phenomenon (KMP) is life-threatening, charactered by the profound thrombocytopenia and consumptive coagulopathy associated with vascular tumors. The therapy of KMP still remains challenging. In this study, we retrospectively analyzed the clinical data of KMP treated in Nanjing Children's Hospital and Jinling Hospital, China, and brief reviewed the literature on KMP. From Jan. 2005 to Dec. 2014, a total of 19 cases of KMP were enrolled into this study. Laboratory results showed that seven patients had typical disseminated intravascular coagulation (DIC), and others were atypical DIC. CT scanning showed the low-density tumor with obvious intensification in enhanced scanning, and the large distorted arteries in association with the tumor. After the admission, the patients received the infusion of platelets and the applying of dipyridamole, steroids, and other necessary drugs. Eight patients underwent complete surgical removal of the tumor, or partial removal with subsequent chemotherapy of vincristine. Three patients underwent only the chemotherapy of vincristine. Eight patients underwent the intralesional injection of absolute ethanol. Pathological examination showed eighteen samples were kaposiform hemangioendothelioma, and one tufted agioma. In our cases, six patients died from extensive hemorrhage and subsequent multiple organ failure. The others survived. In conclusion, KMP in Chinese children has typical symptoms. Kaposiform hemangioendothelioma is the most frequent vascular tumor associated with KMP. The individual treatments with surgical management, chemotherapy with vincristine, and intralesional injection of absolute ethanol can achieve good results in most of the patients with KMP.
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PMID:Kasabach-Merritt phenomenon in Chinese children: Report of 19 cases and brief review of literature. 2630 43

We discussed the diagnostic and treatment value and clinical significance of computer assisted surgery system (Higemi) in precision surgeries for pediatric complex liver tumors. A total of 21 pediatric cases receiving hepatectomy for tumors in the portal vein and giant liver tumors from June 2012 to January 2015 were analyzed. Higemi was used for 3-dimensional (3D) reconstruction of thin-slice CT images and surgical planning. Tumors were precisely located and blood vessel neighborhood was determined so as to evaluate surgical feasibility. In addition, pathological classification, surgical time, intraoperative blood loss, transfusion rate and complications were predicted. After 3D reconstruction using Higemi, the neighboring relationship of tumors with blood vessels and the running direction of the blood vessels were clearly visualized. Of 21 cases, 10 cases had tumors located in the left lobe, 5 cases in the right lobe, 3 cases showing involvement of right trilobes, and 3 cases in the middle lobe. Lobes exceeding one third of the total liver volume were resected in 18 cases. Postoperative pathological examination indicated 10 cases of hepatoblastoma, 3 cases of hepatocellular carcinoma, 3 cases of hamartoma, 3 cases of infantile hemangioendothelioma, 1 case of teratoma and 1 case of undifferentiated malignant mesenchymoma. The surgical time was 90-240 min with an average of 130 min; the medium intraoperative blood loss was 60 ml and the minimum blood loss was 3 ml; the transfusion rate was 42.9% (9/21). Surgeries were successful in 20 cases, who were discharged after recovery. However, one case had giant liver tumor combined with severe obstructive jaundice and hepatic insufficiency and died of postoperative liver failure and DIC. 3D reconstruction of CT data using Higemi can clearly visualize the running direction of blood vessels and the neighboring relationship with tumors. Higemi can improve the precision and safety of complex hepatectomy.
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PMID:Application value of computer assisted surgery system in precision surgeries for pediatric complex liver tumors. 2677 Apr 45

Vascular tumors in neonates are mostly benign; however, locally aggressive voluminous forms may destabilize the hemodynamics of a neonate. Herein, we present an unusual case of a neonatal giant vascular tumor in the right upper extremity, causing a consumption coagulopathy and acute deterioration of vital signs. The patient required mechanical ventilation, inotropic support, and administration of blood products by the seventh day. Vascular embolization attempts failed to improve the general condition of the patient. Due to the deteriorating and life-threatening general condition of the patient, amputation around the upper arm level occurred under emergency conditions on the twelfth day. The patient's hemodynamic parameters were regained immediately, with neither inotropic agents nor blood products required after the second postoperative day. Clinical and pathological diagnosis revealed kaposiform hemangioendothelioma. Patient monitoring proceeded until the age of 15 months, with no local recurrence around the stump or soft tissue coverage complications. Therefore, since other treatment options failed, the early amputation decision was life-saving.
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PMID:Upper arm life-saving amputation of a 12 day-old neonate due to extensive vascular tumor of the upper extremity. 3217 5


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