Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Seven patients with hemangiomatosis of the liver are presented together with 100 cases reported in the literature. Six of seven patients survived; four with supportive treatment only. Follow-up is from 2 to 11 yr. Hepatomegaly, heart failure, and cutaneous angiomas constitute the classical clinical triad. Arteriography is the best method for diagnosis; biopsy should be avoided. Clinical features and histologic examination of these benign vascular tumors seem to confirm the theory that hemangioendothelioma and hemangioma represent only different developmental stages of one disease. These lesions can regress spontaneously if left alone. Despite contrary prevailing opinion, the authors recommend nonoperative treatment as the first choice of the clinician.
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PMID:Hemangiomatosis of the liver in infants. 111 83

The authors present 7 patients with hemangiomatosis of the liver together with about 100 cases reported in the literature. 6 of 7 patients survived; 4 with supportive treatment only. Follow-up is from 2 to 12 years. Hepatomegaly, heart failure and cutaneous angiomas constitute the classical clinical triad. Arteriography is the best method to diagnose this lesion, and biopsy can thus be avoided. Clinical features and histological examination of these benign vascular tumors seem to confirm the theory that hemangioendothelioma and hemangioma represent only two different developmental stages of one disease. These lesions can regress spontaneously if left alone. Despite contrary prevailing opinion the conservative treatment is recommended as the first choice of the clinician.
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PMID:[Hemangiomatosis of the liver in infants: Diagnosis and treatment]. 115 Apr 80

Studies are presented on a 62-yr-old woman with extreme hepatomegaly due to a giant hemangioma with alterations in the clotting system indicating a consumption coagulopathy. There was a fall of hemoglobin, fibrinogen, antithrombin III, and platelet number after arteriography of the truncus celiacus. Furthermore, there was sustained bleeding in the patient's right thigh caused by puncture of the arteria femoralis. Continuous administration of iv heparin corrected the clotting disorder including a rise in platelets from 95,000/microliters to 148,000/microliters, permitting surgical removement of the hematoma. Celiacography is a useful tool for the diagnosis of hepatic hemangiomata, as well as ultrasound, computed tomography, and magnetic resonance imaging. Being an invasive technique, it requires testing for possible consumption coagulopathy if used in patients with hemangiomatosis. Its application should be restricted to cases in which exact diagnosis cannot be established by other means.
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PMID:The Kasabach-Merritt syndrome: severe bleeding disorder caused by celiac arteriography--reversal by heparin treatment. 266 39

Infantile hepatic hemangioendothelioma is a rare tumor of infancy, sometimes associated with cutaneous hemangiomatosis. It is clinically evident within the first six months after birth and can be life threatening because of heart failure, intraperitoneal hemorrhage or thrombocytopenia. In less severe forms spontaneous regression has been described. Current treatment may be surgical ligation of the hepatic artery, or pharmacological therapy with corticosteroids or radiotherapy. A 4-month infant is described, admitted with acute heart failure and huge hepatomegaly. Since a surgical approach was not possible and corticosteroid therapy failed to achieve the expected effect, radiotherapy was given with excellent results.
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PMID:Infantile hepatic hemangioendothelioma treated by radiotherapy. 662 93

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

A female neonate with diffuse hemangiomatosis and hepatic hemangiomas had cardiac insufficiency develop and had failure to thrive. Her disease was unresponsive to prednisone. She received two courses of cyclophosphamide 10 days apart and a final course 2.5 weeks later. Twelve days after the second course of cyclophosphamide, her liver was significantly smaller. She is now 6-years-old, well-developed, and has no signs of hepatomegaly, malnourishment, or heart failure. Cyclophosphamide appears to be a safe, effective, and rapid treatment of life-threatening hemangiomas of infancy.
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PMID:Successful treatment with cyclophosphamide of life-threatening diffuse hemangiomatosis involving the liver. 1113 22

Hemangioma is the most common vascular tumor of infancy; presentation is often as cutaneous infantile hemangioma (IH). Cutaneous hemangioma is a clinical diagnosis. Most IHs follow a benign course, with complete involution without treatment in the majority of cases. Visceral hemangioma often involves the liver and manifests as a life-threatening disorder. Hepatic hemangiomas may be associated with high output cardiac failure, coagulopathy, and hepatomegaly which generally develop between 1 and 16 weeks of age. Mortality has been reportedly high without treatment. We report a rare case of a male infant with neonatal hemangiomatosis with diffuse peritoneal involvement, which mimicked a malignant-looking tumor on imaging, and discuss therapeutic options and efficacy. Propranolol is efficacious for IH but generally not useful for other forms of vascular hemangiomas, tumors, and malformations. In our case of neonatal peritoneal hemangiomatosis, propranolol appears to have halted the growth and possibly expedite the involution of the hemangiomatosis without other treatments.
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PMID:Neonatal Abdominal Hemangiomatosis: Propranolol beyond Infantile Hemangioma. 2711 Apr 21