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)

A newborn infant is described with a highly vascular hepatic mesenchymal hamartoma causing hydrops fetalis, congestive heart failure, and consumption coagulopathy. There was a remarkable response of this life-threatening condition to cyclophosphamide therapy. A review of the various alternative therapeutic modalities is presented.
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PMID:Mesenchymal hamartoma of the liver responsive to cyclophosphamide therapy: therapeutic approach. 400 57

A 9-year-old black girl underwent right hepatectomy because of a large primary tumor in the right lobe of the liver. Histopathologic examination revealed the diagnosis of mesenchymal hamartoma, a rare benign tumor occurring mostly in children below the age of 2 years. Preoperatively, the patient had laboratory evidence of mild disseminated intravascular coagulation (DIC) but had no bleeding manifestations. In preparation for the surgery, the patient received whole blood exchange transfusion and platelet transfusion which re-resulted in marked improvement in hemostatic parameters. Despite what appeared to be adequate replacement of blood loss during the surgical procedure, the patient developed sudden cardiac arrest near the end of the procedure and died. The probable cause of death was hypovolemia. This case of mesenchymal hepatic hamartoma illustrates two unusual features: age of the patient and the association with DIC. The latter, to our knowledge, has not been reported before.
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PMID:Mesenchymal hamartoma of the liver in an older child: association with disseminated intravascular coagulation. 670 May 45

Hepatic resection is the main treatment modality for hepatic tumors in childhood. Advances in diagnostic technique, preoperative preparation, surgical technique, and postoperative management increased the success rate. The aim of this study is to report our experience in hepatic lobectomy, which is relatively rare procedure in childhood. Medical records of 25 patients who underwent hepatic lobectomy between January 1977 and June 2002 were reviewed retrospectively. Age, gender, diagnosis, physical examination findings, results of preoperative laboratory investigations, radiological examination, resectability criteria, preoperative biopsies, chemotherapies, radiotherapies, postoperative pathological results, incisions, operation technique, intraoperative transfusions, drains used, antibiotic prophylaxes, and intraoperative and postoperative complications were evaluated for all patients. Out of 25 patients with hepatic tumor seven patients with hepatoblastoma and four patients with hepatocellular carcinoma were given 5.7 +/- 0.3 cycles of chemotherapy before the operation. Right lobectomy (n = 12), left lobectomy (n = 5), extended left lobectomy (n = 4), and extended right lobectomy (n = 3) and right lobectomy with enucleation of two masses from left lobe (n = 1) were performed. Intraoperative blood transfusion of 30.7+/-6.0 ml/kg body weight was necessary. Pathological examination of resected tumors revealed hepatoblastoma (n=11), mesenchymal hamartoma (n = 5), hepatocellular carcinoma (n = 4), hemangioendothelioma (n=1), malignant mesenchymal tumor (n = 1), hemangioma (n = 1), cyst adenoma (n = 1), and metastasis of cellular mesoblastic nephroma (n = 1). Patients were observed in the intensive care unit for 3.4 +/- 0.3 days. Postoperative complications were sepsis (n = 1), disseminated intravascular coagulation (n = 2), fever (n = 3), jaundice (n = 3), intraabdominal abscess (n = 3), ileus (n = 2), and subdiaphragmatic abscess with pleural effusion (n = 1). Hepatic lobectomy is a major operation, which is feasible yielding curative results in children. Safe hepatic resections with acceptable blood loss can be performed by a technique relying on good anatomic dissection and surgical control.
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PMID:Hepatic lobectomies in children: experience of a center in the light of changing management of malignant liver tumors. 1639 9

Placental mesenchymal dysplasia (PMD) is a rare disease that may be difficult to distinguish from molar pregnancy. The disease is associated with major fetal complications, including Beckwith-Wiedemann syndrome, fetal growth restriction and intrauterine fetal death. Rarely, fetal hematological disorders and liver tumors also may occur. Two patients were referred to our hospital during their second trimesters because of suspected molar pregnancies. Fetal karyotyping and maternal serum human chorionic gonadotropin level determinations led to the PMD diagnoses. In one case, the maternal clinical course was normal, but the neonate suffered from disseminated intravascular coagulation and needed a platelet transfusion. In the second case, the PMD decreased during pregnancy, but a gradually increasing fetal liver tumor appeared. The tumor was diagnosed as mesenchymal hamartoma, based on ultrasound and magnetic resonance imaging studies. The neonate was delivered without cardiovascular compromise. Due to the difficulty of immediate surgical treatment, expectant management, with close follow-up, was chosen.
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PMID:Rare fetal complications associated with placental mesenchymal dysplasia: a report of two cases. 2522 97

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