Cases reported "Hepatomegaly"

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1/9. Massive hepatic fibrosis in Gaucher's disease: clinico-pathological and radiological features.

    hepatomegaly is frequent in patients with type 1 Gaucher's disease and is associated with infiltration of the liver with pathological macrophages. Most patients suffer no significant clinical consequences, but a few develop portal hypertension which may progress to parenchymal liver failure. We describe four patients with Gaucher's disease who have developed portal hypertension. We have reviewed their clinical histories and all available histological and radiological material. All had severe Gaucher's disease with multi-organ involvement, and had undergone splenectomy in childhood. Histologically, this advanced liver disease was characterized by a picture of extreme and advanced confluent fibrosis occupying the central region of the liver. This massive fibrosis is associated with characteristic radiological appearances. The liver histology in these cases is highly unusual and virtually unknown in other conditions. Our studies indicate that without specific treatment the liver disease is progressive and rapidly fatal. However, institution of enzyme replacement therapy with imiglucerase may have beneficial effects even when the condition is far advanced.
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ranking = 1
keywords = enzyme replacement therapy, enzyme replacement, replacement therapy, replacement
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2/9. A new variant neuropathic type of Gaucher's disease characterized by hydrocephalus, corneal opacities, deformed toes, and fibrous thickening of spleen and liver capsules.

    We report a new variant type of Gaucher's disease characterized by hydrocephalus, corneal opacities, deformed toes, gastroesophageal reflux, and fibrous thickening of splenic and hepatic capsules. This patient had 1 D409H allele. He differed from other reported cases with a 1342G to C (D409H) homozygous mutation (onset at 4 months, no cardiac involvement until the age of 12 years, and massive hepatosplenomegaly with fibrous thickening of spleen and liver capsules). Enzyme replacement therapy was given for 4 years, resulting in an improvement of visceral and hematologic abnormalities but no neurologic improvement.
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ranking = 0.0024138309998776
keywords = replacement therapy, replacement
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3/9. pregnancy after avascular necrosis of the femur complicating Gaucher's disease.

    A patient with type I Gaucher's disease had avascular necrosis of the right femoral head that resulted in an altered bony pelvis and marked restriction of right hip abduction. enzyme replacement therapy with alglucerase prevented further deterioration and improved thrombocytopenia. Vaginal delivery was achieved with the patient in the left lateral position with exaggerated flexion at the contralateral hip.
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ranking = 0.0024138309998776
keywords = replacement therapy, replacement
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4/9. carcinoid heart disease: successful tricuspid valve replacement.

    A woman aged 46 was found to have severe tricuspid valve disease 14 years after the diagnosis of a malignant carcinoid tumour of the ileum and 33 years after the onset of symptoms attributable to the disease. Increasing ascites requiring repeated paracentesis had not responded to chemotherapy including infusion of 5-fluorouracil and vincristine into the coeliac artery. The tricuspid valve was replaced by a Bjork-Shiley prosthesis. After operation there was no recurrence of ascites or oedema. She remains well one year and 11 months later. It is suggested that valve replacement surgery should be considered more often in patients with carcinoid heart disease. hepatomegaly and ascites sould not be attributed too readily to advancing malignant disease without careful consideration of the role of right-sided valvar lesions in the production of these signs.
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ranking = 0.00061365627205017
keywords = replacement
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5/9. Mesenteric mass in a young girl--an unusual site for Gaucher's disease.

    We report the first case of a child with Gaucher's disease and a large mesenteric mass, confirmed histologically to be Gaucher's cell infiltrates. We describe the radiological findings and discuss further management. The advent of enzyme replacement therapy has prolonged survival and the emergence of previously undocumented manifestations of the disease is being observed. The radiologist and clinician should be alert to the possible development of these new problems and the fact that in Gaucher's disease a palpable right upper-quadrant mass need not necessarily represent hepatomegaly.
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ranking = 1
keywords = enzyme replacement therapy, enzyme replacement, replacement therapy, replacement
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6/9. Rebound hepatosplenomegaly in type 1 gaucher disease.

    A 19-year-old male patient with type 1 gaucher disease was put on regular biweekly infusions of alglucerase. After 1 yr of treatment, hepatic and splenic volumes decreased from 38 and 45 mL/kg to 31 and 34 mL/kg, respectively. In addition, hemoglobin concentration, platelet count and white cell count increased, acid phosphatase level decreased, and the patient gained weight and energy. Despite improvement, the patient refused enzyme replacement therapy (ERT) because of muscle rigidity, chest pain, trembling and anxiety, which he attributed to enzyme substitution. Two and 4.5 year after cessation of therapy, hepatic and splenic volumes increased to 36 and 53 mL/kg and to 53 and 110 mL/kg, respectively. The patient developed non-tractable hematuria because of compression and dislocation of the left kidney by the enlarged spleen, which necessitated splenectomy. This report suggests that cessation of ERT in gaucher disease may result in severe and complicated rebound visceromegaly.
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ranking = 1
keywords = enzyme replacement therapy, enzyme replacement, replacement therapy, replacement
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7/9. Acute hepatic failure and multi-system organ failure secondary to replacement of the liver with metastatic melanoma.

    BACKGROUND: Metastatic malignant melanoma to the liver resulting in fulminant hepatic failure is a rare occurrence. CASE PRESENTATION: A 46 year old man presented to hospital with massive hepatomegaly, elevated liver enzymes and increased lactate three weeks following resection of a malignant melanoma from his shoulder (Clark level 5). Initially stable, he decompensated 24 to 48 hours subsequent to presentation with respiratory failure requiring mechanical ventilation, distributive shock requiring high dose vasopressor infusion, coagulopathy refractory to plasma infusion, progressive rise in liver enzymes and severe metabolic abnormalities including hyperkalemia, acidosis, hyperphosphatemia, hyperuricemia and hypocalcemia. Refractory to aggressive physiologic support he received palliation. autopsy revealed >80% liver infiltration by metastatic malignant melanoma. CONCLUSION: We report a case of fulminant hepatic failure secondary to metastatic malignant melanoma infiltration of the liver.
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ranking = 0.00049092501764014
keywords = replacement
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8/9. Merkel cell tumor with liver metastases: presentation as fulminant hepatic failure.

    This report demonstrates that hepatic metastases can present with fulminant hepatic failure and that liver enzyme abnormalities may not become prominent until there has been massive replacement of the liver. The CT scan of liver may not demonstrate diffuse liver metastases as seen in this patient. Merkel cell tumor or trabecular neuroendocrine skin tumor is a previously unreported cause for the development of the syndrome of hepatic failure due to liver metastases.
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ranking = 0.00012273125441003
keywords = replacement
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9/9. Occupationally related angiosarcoma of the liver in the United Kingdom 1972-1994.

    BACKGROUND: Angiosarcoma of the liver (ASL) has been described in vinyl chloride workers worldwide. AIM: To describe the UK experience of occupationally related ASL. patients: Twenty patients who died from ASL after exposure to vinyl chloride. methods: The case records and pathological findings of these 20 patients were reviewed. RESULTS: Twenty men in the United Kingdom aged 37 to 71 years have developed ASL in association with occupational exposure to vinyl chloride monomer VCM in two factories. All had been exposed to VCM for three to 29 years, the tumour developing nine to 35 years after first exposure. Presenting clinical features included abdominal pain, malaise, jaundice, ascites, and massive hepatomegaly. In most cases the disease progressed rapidly, death occurring within a few weeks from hepatic coma. In 17 cases there was no spread outside the liver. In four cases there had been haemorrhage from oesophageal varices due to non-cirrhotic portal fibrosis diagnosed six to 18 years previously. At necropsy the livers of these men showed considerable, often massive, replacement by tumour, apparently multifocal, with necrosis and haemorrhage. CONCLUSIONS: In view of the long latency between exposure and development of the tumour the full extent of ASL occurrence may not be known until 35 years after the introduction of the Code of Practice in 1975.
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ranking = 0.00012273125441003
keywords = replacement
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