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)

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

A preterm infant presented with high-output cardiac failure. Color Doppler imaging of the liver revealed a large hepatic hemangioma. Pulsed-wave Doppler of the proximal aorta was used to monitor noninvasively the reduction in flow through the arteriovenous malformation in response to steroid therapy. The hemangioma resolved completely by 7 weeks of age.
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PMID:Noninvasive investigation of infantile hepatic hemangioma: a case study. 770 Jul 64

A 48-year-old woman with giant haemangioma of the liver underwent percutaneous transjugular placement of a stent in the left hepatic vein for relief of an obstruction due to the compression of the benign liver tumour. Following the procedure, paroxysmal atrial fibrillation occurred and right-sided heart failure gradually appeared. Echocardiography and cardiac catheterization demonstrated a fistula between the aortic root and the right atrium, that was confirmed on surgery.
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PMID:Aorto-atrial fistula following internal jugular vein catheterization. 774 2

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

Hypergalactosaemia was discovered in a newborn girl during routine metabolic screening. Hereditary enzyme deficiency was ruled out. She had multiple hepatic haemangiomas with portal-hepatic venous and hepatic arterio-venous shunts. Since she showed signs of high-output heart failure due to the arterio-venous shunt, hepatic artery embolization was performed at age 3 months. A galactose tolerance test was performed before and after embolization and when the haemangioma no longer appeared on ultrasonography. Even after embolization, the level of blood galactose was abnormally elevated in the galactose tolerance test, but the blood galactose was eliminated more rapidly than before embolization. When the hepatic haemangioma was no longer detected by ultrasonography, the peak galactose level decreased. We surmise that the hypergalactosaemia was due to these shunts. In cases of hypergalactosaemia of unknown cause; liver haemangioma with portal-hepatic venous shunting should be considered as a possible cause. If a hepatic arterio-venous shunt also exists, this may contribute to the effect of the portosystemic shunting.
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PMID:Hypergalactosaemia in a patient with portal-hepatic venous and hepatic arterio-venous shunts detected by neonatal screening. 813 18

Hepatic hemangiomas are benign vascular tumors that represent 5-15% of all liver tumors. They occur more often in females than in males (relation 2:1). They may produce cardiac failure, because of high output; less frequently hemolytic anemia, thrombocytopenia or bleeding are observed. We present a case of a newborn premature child with a gestation age of 34 weeks and weight of 1359 g who presents a palpable abdominal tumor in the right hypochondrium without additional symptoms. The diagnosis was confirmed by ultrasound with Doppler system any by means of the magnetic resonance imaging (MRI) both methods were able localize the hemangioma in the posterior segment of the right hepatic lobulus. MRI is a diagnostic tool which can provide finer anatomic details than ultrasound or axial computed tomography in hepatic hemangiomas. MRI has a sensitivity of 90% and specificity of 92%; the treatment depends of the complications which the patient presents.
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PMID:[Hepatic hemangioma in a premature newborn. The magnetic resonance images]. 844 69

A rare case of an ulcerated hemangioma of the upper arm in a neonate is described herein. Resection of the huge hemangioma, which occupied the axilla and extended down the entire length of the upper arm, was necessary due to massive hemorrhage from the ulcer and progressive heart failure caused by the arteriovenous fistula in the hemangioma. After complete resection of the hemangioma, extensive defects of the skin and subcutaneous tissue were replaced by a skin flap closure technique which involved considerable difficulty. However, the arm gradually recovered its size and function with good wound healing. The present case illustrates that rapid surgical intervention may be required to prevent clinical deterioration in such cases.
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PMID:Hemangioma of the upper arm associated with massive hemorrhage in a neonate. 846 82

From 1958 through 1992, 30 patients with hepatic hemangiomas were seen and treated at Children's Hospital Los Angeles. The majority of the patients were less 1 month of age (90 % younger than the first year of life) and there was no difference in sex distribution. Patients presented with coagulopathy, heart failure, abdominal mass, and respiratory distress. Eleven patients (33%) had hemangiomas in other sites. Fourteen patients were treated with steroid therapy. Of these, eight patients did not respond and received radiotherapy. Eleven patients who had the hemangioma confined to an anatomical lobe had resection of the hemangioma by liver lobectomy. Five of the most recent patients were successfully treated with hepatic artery embolization. Two other patients who were seen many years ago underwent diagnostic laparotomy and biopsy of the lesion before treatment with steroids. In one patient who presented with ruptured hepatic hemangioma, hepatic arterial ligation was performed. In another patient, seen recently, treatment with interferon alpha-2 was initiated, but the patient died. There were six deaths in the series. Four patients died of intractable congestive heart failure, steroids are given first. Course of steroid therapy may be repeated if necessary. Whereas formerly radiation therapy was added to the treatment of a patient resistant to steroids, therapeutic hepatic arterial embolization is a very good alternative for these patients. Surgical excision of the lesion can be performed by liver lobectomy if there is a solitary hemangioma within the boundaries of the surgical excision. Recently, in massive hemangioma with intractable thrombocytopenia, interferon alpha-2 therapy has been used, but so far our experience of this mode of therapy is limited.
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PMID:Hepatic hemangiomas in infants and children: a review of 30 cases. 861 61

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

Of 16 infants with infantile hepatic hemangioendothelioma, 14 (88%) presented before age 3 months. For seven cases (44%), the diagnosis was suspected from antenatal ultrasonographic findings. Two (13%) presented with asymptomatic hepatomegaly. The most common presenting features were high-output cardiac failure in 11 (69%), consumptive coagulopathy in 12 (75%), and anemia in 12(75%). Sixty-three percent of the children had associated cutaneous hemangiomata, and disseminated hemangiomatosis was noted in two (13%). Medical measures were effective in stabilizing seven (44%) cases with high-output congestive cardiac failure and/or consumptive coagulopathy. Partial right hepatectomy was successful for four patients; the only death occurred in a newborn, after intraperitoneal rupture of the hepatic hemangioma. Embolization was used in two children to induce involution. Spontaneous involution occurred in two patients. Initially, hepatic hemangiomas should be treated conservatively, with surgery reserved for intractable cardiac failure and/or refractory consumptive coagulopathy.
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PMID:Infantile hepatic hemangioendothelioma: the role of surgery. 878 79


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