Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Kasabach-Merritt syndrome is characterized by thrombocytopenia and localized coagulopathy associated with a hemangioma. Most techniques applied to eradicate the tumor or accelerate its involution (surgery, radiation therapy, embolization) are invasive and require transfusion of large amounts of blood products. In some cases, medical treatment is the only alternative. Efficacy of steroids and antifibronolytic agents has already been described, but even this approach is associated with the administration of blood products. We report two cases of infants with Kasabach-Merritt syndrome associated with cardiac and hepatic hemangiomas. At admission, both had signs of cardiac failure. They were successfully treated with prednisone and epsilon-aminocaproic acid (EACA). Blood products were not required once the diagnosis was made. These observations have important implications for the management of patients with Kasabach-Merritt syndrome because they show that even in severe cases blood transfusions can be avoided by the use of prednisone and EACA.
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PMID:Successful treatment of Kasabach-Merritt syndrome with prednisone and epsilon-aminocaproic acid. 831 77

Clinical features and management of 47 children with Klippel-Trenaunay syndrome treated since 1970 were reviewed. Haemangiomas and soft tissue and/or skeletal hypertrophy were present in all 47 patients; venous varicosities developed in 37 (79 per cent). There was no clinical evidence of macrofistulous arteriovenous communications in any patient. Thromboembolic episodes occurred in five children (11 per cent) and 25 (53 per cent) experienced thrombophlebitis. The Kasabach-Merritt syndrome was observed in 21 (45 per cent) and six (13 per cent) presented with high-output cardiac failure. Other manifestations included haematuria in five (11 per cent), rectal or colonic haemorrhage in six (13 per cent), and vaginal, vulval or penile bleeding in six (13 per cent) children with visceral and pelvic haemangiomas. In 26 patients (55 per cent) symptomatic treatment only was required. Surgery was undertaken in selected cases for complications of the haemangioma, for cosmetic reasons and for chronic venous insufficiency. Only one of four children who underwent resection of varicose veins improved. There was no death, but significant morbidity was associated with the treatment of Kasabach-Merritt syndrome and high-output cardiac failure.
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PMID:Klippel-Trenaunay syndrome: clinical features, complications and management in children. 754 89

Hemangiomas are the most common benign neoplasm in the neonatal period. While most small hemangiomas involute spontaneously, up to 20% may grow to a massive size and never completely involute. Massive hemangiomas are also frequently associated with life-threatening complications, such as airway obstruction, platelet trapping (Kasabach-Merritt syndrome), and high-output heart failure. The use of interferon alfa-2a for the treatment of massive hemangiomas not responsive to traditional therapy has recently been reported. We present the successful use of interferon alfa-2a in a series of five patients with massive hemangiomas complicated by airway compromise, congestive heart failure, need for tracheotomy, Kasabach-Merritt syndrome, and failure to thrive, despite traditional therapy.
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PMID:The use of interferon alfa-2a for life-threatening hemangiomas. 777 25

Vascular tumours of the liver present variously in the paediatric age group. Their clinical course depends on tumour size, its growth characteristics, localization and complications. The most feared complications are cardiac failure and the Kasabach-Merritt syndrome which may occur in up to 50% of affected children. In haemangioendotheliomata and capillary haemangiomata spontaneous regression can be expected. Regression may be stimulated by x-ray radiation therapy, corticosteroids, Endoxane or alpha Interferon. Emergency percutaneous catheter embolization or operation must be carried out in the event of such complications. The Nd:YAG laser is particularly well suited for these cases. In all events, careful interdisciplinary cooperation among paediatricians, radiologists, cardiologists, oncologists and paediatric surgeons is required owing to threatening complications which mostly occur in neonates and infants.
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PMID:The use of the laser in the treatment of arterio-venous malformations and vascular tumours of the liver. 821 73

A small number of patients with haemangioma need treatment for serious complications such as Kasabach-Merritt syndrome, cardiac failure and obstruction of the airway. We report on the management of an infant with Kasabach-Merritt syndrome.
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PMID:Cavernous haemangioma with Kasabach-Merritt syndrome: treatment with alpha-interferon. 870 89

A case of a large vascular lesion of the skull is reported. The lesion was discovered at 22 weeks' gestation and it rapidly increased in size, reaching 8 cm a month later. Tumor echogenicity was the same as that for soft tissues and colour Doppler examination revealed intense vascularization. Cordocentesis showed features of the Kasabach-Merritt syndrome and very high plasma levels of alpha-fetoprotein. A Caesarean section was performed to avoid dystocia and led to the birth of a baby with cardiac failure in the immediate neonatal period. Biopsies of the mass led to the diagnosis of a non-malignant haemangio-endothelioma. The mass was removed and plastic surgery performed. The baby is alive and well 9 months after the operation.
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PMID:Prenatal aspects of giant fetal cranial haemangio-endothelioma. 873 13

Kasabach-Merritt syndrome is characterized by thrombocytopenia and bleeding tendency leading to disseminated intravascular coagulation with giant hemangiomas. We present a very low birth weight infant with this syndrome who underwent four operations. A male baby (1179 g, 37 cm) was born at a gestational age of 28 weeks and 6 days by caesarean section. A large hemangioma, 7 x 8 cm in size, was recognized on the left thigh. As associated consumption coagulopathy (Kasabach-Merritt syndrome) was diagnosed with platelet count 5.1 +/- 10(4) mm-3 and fibrinogen 49 mg.dl-1. Despite treatment with liniac X-ray radiation, systemic steroid and component transfusion, coagulopathy became worse with extremely low platelet count of 1.1 x 10(4) mm-3. Infusion of dopamine and dobutamine was necessary for high output cardiac failure. On day 9, PDA ligation was performed. Cerebro-ventricular drainage, ventricuro-peritoneal shunt and shunt revision were required on day 15, 49 and 88, respectively, for hydrocephalus due to intraventricular hemorrhage. Main anesthetics used were fentanyl and sevoflurane. Major problems encountered by anesthetists were: bleeding tendency, water and electrolyte management, body temperature control, and immaturity and fragility of premature infant. Coagulopathy in Kasabach-Merritt syndrome must be a risk factor for intraventricular hemorrhage, which is a characteristic complication of a very low birth weight infant.
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PMID:[Anesthetic problems in a very low birth weight infant with Kasabach-Merritt syndrome]. 881 2

Extensive haemangioma with platelet consumption (Kasabach-Merritt syndrome) is rare. The lesion is usually a single cutaneous cavernous haemangioma similar to that found in our patient. Multiple superficial strawberry naevi were also seen all over the rest of the body. This condition was diagnosed antenatally in this patient with the use of colour Doppler. There is one recent report where the thrombocytopaenia was diagnosed by cordocentesis. Unfortunately the baby developed heart failure with intractable coagulopathy and died 2 days later.
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PMID:Prenatal diagnosis of an extensive haemangioma of the fetal leg: a case report. 887 Apr 22

This report describes a female baby having a hemangioma over the right thigh that had appeared as an irregular bruise since two days old. Severe thrombocytopenia, consumptive coagulopathy, anemia, and heart failure developed at three months old. With a diagnosis of Kasabach-Merritt syndrome, systemic corticosteroid, vincristine, subcutaneous alpha-interferon, and massive plate transfusion were given. However, the platelet count remained low and the skin lesion enlarged gradually. Trans-feeding-arterial embolotherapy with a 5 ml pure ethanol (1 ml/kg) injection was performed at four months of age. Thirty days later, her platelet count recovered and the hemangioma shrunk in size. This case illustrates that absolute ethanol embolotherapy is an effective treatment for hemangioma with Kasabach-Merritt syndrome and may be life-saving for those with lesions refractory to medical therapy.
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PMID:Absolute ethanol embolotherapy for hemangioma with Kasabach-Merritt syndrome. 955 94

We describe the case of a patient with a neonatal giant cutaneous hemangioma with high-output cardiac failure and Kasabach-Merritt syndrome and successfully treated with transcutaneous arterial embolization aimed at controlling severe congestive heart failure and consumption coagulopathy. A patient was admitted to the neonatal care unit on the first day of age because of a large hemangioma on his right lateral chest wall and respiratory distress, associated with cardiac failure resulting from arteriovenous shunting. On the second day of age the platelet count decreased to 5.7 x 10(4)/microliter and fibrinogen level was 85 mg/dl. The values of prothrombin time and activated partial thromboplastin time were prolonged. Intravenous predonisone therapy was started immediately, but bleeding tendency was getting worse and the evidence of congestive heart failure persisted. On the third day the patient then underwent embolization of feeding arteries with microcoils. The cardiac failure and thrombocytopenic coagulopathy had improved significantly without complications. We conclude that transcutaneous arterial embolization is an effective and safe treatment in this neonate and should be considered for the treatment of control high-output cardiac failure and coagulopathy in infants with hemangioma and Kasabach-Merritt syndrome.
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PMID:Successful transcutaneous arterial embolization of a giant hemangioma associated with high-output cardiac failure and Kasabach-Merritt syndrome in a neonate: a case report. 1064 62


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