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)

An unusual patient with hairy cell leukemia (HCL) who developed marked hepatomegaly due to a large vascular tumor in the liver is reported. The relation of this vascular tumor to the microscopic splenic pseudosinuses and hepatic angiomatous lesions encountered in HCL is discussed. To the best of our knowledge this represents the first case report of the association of HCL with large macroscopic hemangioma of the liver causing hepatomegaly. The patient also developed a large paratracheal mediastinal mass with a recurrent pleural effusion which was shown to contain many typical hairy cells. This rare finding is discussed in relation to the isolated cases of lymphocytic lymphoma who present with clinical and morphological features mimicking HCL. This patient had HCL according to all established criteria with characteristic morphological, cytochemical and ultrastructural features and the pleural effusion and mediastinal mass were most probably part of the HCL neoplasia, despite the fact that biopsy was not performed.
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PMID:Hairy cell leukemia: report of an unusual case with hepatomegaly due to a large vascular tumor of the liver, mediastinal mass and pleural effusion containing hairy cells. 643 21

The authors describe a case of hepatic hemangiomathosis in a three months old infant with a clinical picture of multiple cutaneous hemangioma, epatomegaly and severe cardiac failure. The cardiac catheterization with selective angiography showed extensive communication between the mammary artery and the hepatic circulation with the presence of large bloody areas into the markedly enlarged liver. The medical treatment with digitalis, diuretics, and corticosteroids obtained rapid resolution of the cardiac failure and of the A-V fistula signs. A clinical picture of severe liver cholostatic disease developed few months later which was confirmed by histological examination. The subsequent course has been favorable with complete clinical resolution of the liver disease.
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PMID:[Hepatic hemangiomatosis with congestive cardiac failure and development into a cholostatic hepatopathy]. 653 23

The authors analyze 5 cases of perinatal hemangiomas in internal localizations respresenting relative or immediate emergencies. Three cases were subglottic proliferating hemangiomas with threatening suffocation, the other two were large hemangiomas of the liver causing conspicuous hepatomegaly. In 3 children there were also hemangiomas of the skin and/or in the oral cavity seen as important signs in the diagnostic reflections on the nature of the urgent clinical pictures. Moreover one child with subglottic hemangioma suffered from hematological disorders characterizing the syndrome Kasabach--Merritt. In all the children transcutaneous radiotherapy was performed (ranging from 12 Gy in two weeks up to an exceptional dose of 25 Gy over 3 months). This was followed by recession of subjective complaints and, eventually, by complete regression of the irradiated angiomatous lesions, both subglottic and hepatic. The disorders of hemocoagulation disappeared also quickly and completely. At present, i. e. after 3 to 21 years, there are no undersirable post-irradiation changes in any of the patients. Nevertheless, in view of possible post-irradiation effects, particularly on the thyroid gland, the patients continue to be regularly followed up at the respective clinical departments.
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PMID:Perinatal subglottic and hepatic hemangiomas as potential emergencies: effect of radiotherapy. 745 54

Five cases of hepatic haemangioma are described, and a sixth (previously reported) is reviewed. Clinical features, investigation, and management are described to show the great variability of the complications and prognosis. Five children presented in the first 10 weeks of life with hepatomegaly; 4 developed congestive cardiac failure; 3 had cutaneous haemangiomata. One child presented at age 4 years with hepatomegaly and anaemia, and on investigation had features of chronic disseminated intravascular coagulation. Focal decrease or patchiness in hepatic uptake of technetium-99m colloid, and abnormal intrahepatic circulation was shown in all cases. In 3 children liver biopsy was performed to exclude malignant disease. In one patient there was spontaneous regression of the tumour by age 3 years. In 3 cases hepatic artery ligation was necessary to control congestive cardiac failure which had persisted despite treatment with digoxin, diuretics, and oral corticosteroids, a procedure which was without complications after up to 8 years. One infant with intractable portal hypertension, hepatic vein obstruction, and severe cholestasis died with persisting alimentary haemorrhage and intra-abdominal sepsis. One child aged 4 years showed no immediate response to hepatic artery ligation but the size of her tumour got smaller and the clinical features diminished after irradiation. These tumours cause considerable morbidity and have a high reported mortality. If congestive cardiac failure is not rapidly controlled, hepatic artery ligation should be performed.
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PMID:Hepatic haemangiomata: diagnosis and management. 746 56

New, non-invasive neuroradiological techniques [computed tomography (CT) and magnetic resonance (MR)] have led to reassessment of the incidence of cavernous angioma of the brain (CCA), which is sometimes multiple and associated with cavernomas in other organs. CCA is known to be familial, with dominant autosomal transmission. This paper concerns a family with multiple CCA, sometimes in association with liver angiomas, in ten members belonging to four different generations. These malformations can vary in clinical expression: no neurological symptoms have been detected in subjects from the first or second generations, but they were found in adult age in subjects from the third generation; two fourth-generation patients came under our observation at 2.5 years of age. Symptoms include partial epileptic fits, which sometimes become generalized later and which are generally controlled adequately by therapy. Patients also present paresthesia and occasional motor deficiencies corresponding to CCA bleeding episodes; these symptoms have always abated with medical treatment alone. None of the patients are mentally retarded or restricted in their daily lives. Neuroradiological investigations (CT, MR, angiography) reveal typical multiple brain lesions in all patients. Given the first-generation patient's clinical history of symptomatic hepatomegaly and the postmortem finding of multiple liver and brain cavernomas, liver ultrasonography was performed on all members of the family. Liver angioma was detected in two subjects from the second and third generations. Retinal angioma was detected in one patient with quadrantanopsia.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Familial cerebral, hepatic, and retinal cavernous angiomas: a new syndrome. 775 14

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

We describe a case of giant cavernous haemangioma of the liver with disseminated intravascular coagulopathy (Kasabach-Merritt syndrome) which was cured by orthotopic liver transplant. A 47 year old man presented with bleeding and tender massive hepatomegaly after tooth extraction. Investigations showed disseminated intravascular coagulopathy and a giant hepatic haemangioma involving both lobes of the liver. Initial treatment failed to resolve the coagulopathy and liver resection was attempted. At laparotomy the tumour was unresectable and the only option for cure was to offer a liver transplantation. The orthotopic liver transplant was performed 20 days after initial laparotomy. Subsequently, all coagulation parameters returned to normal and the patient remains well after 12 months. Orthotopic liver transplant can be considered for giant hepatic haemangioma with Kasabach-Merritt syndrome when resection is necessary and a partial hepatectomy is not technically feasible.
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PMID:Treatment of a giant haemangioma of the liver with Kasabach-Merritt syndrome by orthotopic liver transplant a case report. 917 60

We report the case of a six-week-old baby who underwent irradiation for a giant hepatic hemangioma. After medical treatment including corticosteroids and interferon, no response was observed. She progressed to respiratory failure, requiring the use of mechanical ventilation. An emergency radiation therapy of the liver was decided. We observed a rapid improvement of the child, with the recovery of autonomous breathing without mechanical ventilation and a normalization of cardiac functions. Two months later, a partial left hepatic embolization was needed due to a progression of a localized blood flow. Six months later, she finally came back home. Cardiac output was normal and hepatomegaly began to regress. One year later, ponderal status is satisfactory, and it remains a localized hepatic right lobe hypertrophy without functional consequence.
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PMID:[Radiotherapy for a massive hepatic hemangioma in a six-week-old infant]. 1063 Jan 64

Hepatic hemangioendothelioma (HE) is a tumor that presents in infancy and toddler. It manifests hepatomegaly, abdominal mass, jaundice, abdominal distention, or high output cardiac failure. We reviewed patients with HE in our hospital in the past 15 years (from July 1986 to June 2001). The diagnosis was made by the histology specimen or various imaging studies. There were thirteen patients (9 males, 4 females) enrolled in our study. Their ages ranged from neonate to 2 years old. The common clinical manifestations included abdominal distention (53%), congestive heart failure (38.5%), abdominal mass (30.8%), jaundice (30.8%), and skin hemangioma (23.1%). Nine patients had serum alanine aminotransferase examination and were abnormal in 2. Anemia was noted in 7 of 13 (53.8%) patients, thrombocytopenia and hyperconsumptive coagulopathy were found in 4 and 5 patients, respectively. Serum alpha-fetoprotein was elevated in 4 of 7 patients. Abdominal ultrasonography (n = 13) showed heterogeneous and hypoechoic lesions in the liver. Computed tomography (n = 11) revealed central hypointensity with peripheral enhancement after contrast of the liver masses. Magnetic resonance imaging studies of the hepatic masses (n = 3) showed decreased signal intensity on T1 images and high signal intensity on T2. Most patients were treated with steroid. Other management included interferon, chemotherapy, embolization and/or surgery. Four patients were managed conservatively. Among the other nine patients, four patients died of sepsis, hepatic failure, disseminated intravascular coagulopathy or tumor rupture with hemorrhagic shock. HE appears to be a histologically benign tumor but may have a poor outcome because of complications. For its management, steroid is a first-line medication. Other methods of treatment were interferon, hepatic artery embolization, chemotherapy and surgery. Long term follow up is needed for the evaluation of treatment response.
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PMID:Hepatic hemangioendothelioma in children: analysis of thirteen cases. 1280 Mar 77

A 47-year-old man presented with fever of unknown origin, generalized weakness, edema, and renal failure. He had left-sided pleural effusion, generalized lymphadenopathy, multiple nontender cutaneous nodules, hepatomegaly, renal failure, and hypergammaglobulinemia. Axillary lymph node biopsy showed findings consistent with Castleman disease of the hyaline vascular type associated with interfollicular plasmacytosis. A renal biopsy performed in view of proteinuria and acute renal failure showed hypercellular glomeruli with capillary loop thickening and double contours consistent with membranoproliferative glomerulonephritis. Skin nodule biopsy showed a glomeruloid hemangioma characterized by dermal proliferation of capillary loops in a nodular manner resembling a glomerulus. He experienced clinical and biochemical remission with steroids. Discontinuation of steroid therapy was associated with recurrence of renal failure, reappearance of nodules, lymphadenopathy, and appearance of bony lytic lesions. Biopsy of bony lytic lesions showed plasmacytoma. The patient achieved complete remission on treatment with steroids and cyclophosphamide and is free of symptoms at the end of 40 months of follow-up. To our knowledge, this is the first case report of the occurrence of membranoproliferative glomerulonephritis, glomeruloid hemangioma of the skin, and plasmacytoma in a patient with multicentric Castleman disease without human immunodeficiency virus infection.
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PMID:Renal involvement in multicentric Castleman disease with glomeruloid hemangioma of skin and plasmacytoma. 1686 Jan 82


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