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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of B cell lymphoma with clinical and histological features of malignant histiocytosis was described. A 57-year-old male was admitted to Shinshu University Hospital because of transverse myelopathy. Five months before admission, he noticed urinary disturbance, which progressed to urinary obstruction. The following month, bilateral muscular weakness appeared in his legs. A few days later he could not stand up, and was admitted to a local hospital. Neurological examination revealed sensory disturbances below the level of Th12 in all modalities, and marked weakness and hyperreflexia in the lower limbs. A spinal tumor was suspected. However, myelography showed no abnormality. The patient's condition worsened and he became bed-ridden in February 1990. He was transferred to Shinshu University Hospital for further evaluation. On admission he was poorly nourished with fever, anemia, hepatomegaly, and bilateral pretibial pitting edema. No lymphadenopathy was observed. Neurological examination showed total sensory loss below the level of Th12, spastic paraplegia, hyperreflexia in the legs, and urinary obstruction. Laboratory findings revealed an elevated erythrocyte sedimentation rate, increased CRP, pancytopenia, and hypoalbuminemia. Serum level of IgG, IgA, IgM, LDH, ALP, GPT and total bilirubin were increased. CSF and MRI imaging of the spinal cord were normal. Proliferation of atypical histiocytes with marked erythrophagocytosis, which is a characteristic pathological feature of malignant histiocytosis, was observed in peripheral blood and aspirated bone marrow. Immunoenzyme staining of bone marrow using monoclonal antibody L-26, which is a B-cell marker, revealed B-cell lymphoma.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of B-cell lymphoma with clinical and histological features of malignant histiocytosis]. 130 24

A rare form of plasma cell dyscrasia characterized by associated polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes has been termed the POEMS syndrome. The pathophysiology is unknown; plasma cell dyscrasia is essential; secondary manifestations are unexplained. We report a 67-year-old man with a 7-month history of progressive weakness and numbness of the legs. Clinical examination revealed sensorimotor polyneuropathy, predominantly affecting the lower extremities, hepatomegaly, and skin haemangiomas. Additional investigations disclosed IgG-lambda monoclonal serum protein, endocrine abnormalities, elevated cerebrospinal fluid protein level and an osteoblastic lesion of the lumbar vertebra. Biopsy of the osteosclerotic vertebra showed a marked lymphoplasmocytic infiltrate. MRI of the liver disclosed two haemangiomas; this association has not been reported previously.
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PMID:Polyneuropathy with osteosclerotic myeloma--POEMS syndrome. A case report. 154 70

A 69-year-old male, who had been found (during a routine roentgenogram of the chest at an annual check-up) to have cardiomegaly, had been asymptomatic until 63 years of age, when DOE and swelling of the legs appeared. He visited our hospital because these symptoms has become more severe. Physical examination revealed JVD, third heart sound and a grade 3 holosystolic murmur, hepatomegaly and edema in the lower legs. His ECG showed atrial fibrillation. His chest X-P showed marked cardiomegaly and rt pleural effusion. His echocardiography and MRI revealed a marked enlargement of the right atrium and a slight enlargement of the right ventricle. The latter also showed persistent left superior vena cava. The cardiac catheterization, angiocardiography and intracavitary electrocardiography revealed no organic cardiac disease which induced enlargement of the right atrium. The idiopathic enlargement of the right atrium is a rare disease. Patients suffering from this disease are asymptomatic in most cases. We reported the idiopathic enlargement of the right atrium with persistent left superior vena cava in this paper.
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PMID:[Idiopathic enlargement of the right atrium with persistent superior vena cava]. 214 15

Ascites, hepatomegaly, and abdominal pain constitute the classic triad of the Budd-Chiari syndrome of hepatic-vein or inferior-vena-cava obstruction. This condition was first mentioned by Budd in the mid 1800s and additional information was provided by Chiari in the 1890s. In nearly two-thirds of patients the exact etiology cannot be determined. The syndrome has, however, been associated with hypercoagulable states, neoplasms, trauma, medications, and congenital abnormalities. The diagnosis is difficult to make clinically; therefore, radiology plays a critical role in the workup of these patients. Nuclear medicine, sonography, CT, angiography, and MRI all provide valuable diagnostic information. These data combined with hepatic biopsy determine which patients should be treated by percutaneous angioplasty or surgery, and also determine the type of shunt to be performed (such as the mesoatrial shunt when the inferior vena cava is occluded or severely compressed). Noninvasive imaging is also useful in the follow-up of patients after both percutaneous angioplasty and surgery.
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PMID:The Budd-Chiari syndrome: a review. 294 Aug 46

The clinicopathological features of a previously unrecognised type of acute encephalopathy prevalent among Japanese children is described by reviewing the records of 13 consecutive patients treated and 28 previously reported cases. The hallmark of this encephalopathy, proposed to be a novel entity termed acute necrotising encephalopathy of childhood, is multiple, necrotic brain lesions showing a symmetric distribution. The encephalopathy was noted in previously healthy children after respiratory tract infections, with presenting symptoms of coma, convulsions, vomiting, hyperpyrexia, and hepatomegaly. Laboratory examinations disclosed liver dysfunction, uraemia, and hypoproteinaemia. The histological appearance of the liver was variable and non-specific. Cerebrospinal fluid contained an increased amount of protein. Computed tomography and MRI showed the presence of symmetrically distributed brain lesions of the thalamus, cerebral white matter, brainstem, and cerebellum. Necropsy examination confirmed extensive fresh necrosis of these regions with evidence of local breakdown of the blood-brain barrier. Based on the characteristic combination of clinical and pathological findings, acute necrotising encephalopathy of childhood can be distinguished from previously known encephalopathies, including Reye's syndrome.
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PMID:Acute necrotising encephalopathy of childhood: a new syndrome presenting with multifocal, symmetric brain lesions. 2996 23

In 24 patients with Budd-Chiari syndrome age 15-66, 19 were men, and 5 women. The patients experienced abdominal pain, hepatomegaly, splenomegaly and ascites. Imaging examinations were carried out in all the patients (16 ultrasonography, 5 Doppler, 10 CT, 18 venography, and 4 MRI). Direct features of Budd-Chiari syndrome were striking reduction in caliber, complete absence or obstruction of hepatic veins, intrahepatic inferior vena cava, "comma-shaped" or curled-tubular intrahepatic collateral veins; indirect signs included hepatomegaly, splenomegaly and ascites, extrahepatic collateral veins. Ultrasonography,CT, MRI and venography were all useful in the diagnosis of patients with Budd-Chiari syndrome. It is suggested that ultrasonography is a non-invasive procedure and MRI is the best imaging procedure of choice in the evaluation of Budd-Chiari syndrome.
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PMID:[Imaging diagnosis of Budd-Chiari syndrome: report of 24 cases]. 812 14

A 4-week-old male infant was admitted to the hospital with acute gastrointestinal bleeding and marked coagulopathy secondary to vitamin K malabsorption in the presence of cholestasis. Physical examination revealed hepatomegaly and cutaneous haemangiomas. Ultrasonography, CT, and MRI demonstrated a multifocal vascular process and allowed the diagnosis of infantile hepatic haemangioendothelioma to be made without the use of more invasive diagnostic procedures. To avoid high-output congestive heart failure, the patient was treated with oral corticosteroids. After 5 months, rapid involution of the vascular malformations ensued. At age 2 years, a magnetic resonance scan confirmed complete resolution of the hepatic haemangioendothelioma.
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PMID:Imaging diagnosis and follow-up of infantile hepatic haemangioendothelioma: a case report. 815 13

This report describes the sonographic and MR appearances of acute Budd-Chiari syndrome (BCS) in a 30-year-old woman. Current imaging techniques including duplex Doppler sonography and dynamic gadolinium-enhanced MRI were employed, and direct histopathologic correlation was made. Duplex Doppler sonography revealed a heterogeneous enlarged liver, no flow in the left and middle hepatic veins, and only trace abnormal flow in the distal-most right hepatic vein. Dynamic serial gadolinium-enhanced, spoiled gradient echo imaging demonstrated early homogeneous enhancement of an enlarged caudate lobe, as well as heterogeneously decreased enhancement of the remainder of the hepatic parenchyma. This differential enhancement pattern persisted on delayed imaging. Patent hepatic veins were not demonstrated on any sequence. Associated findings included hepatomegaly, ascites, and patent portal vasculature. These imaging findings were interpreted as consistent with acute BCS. Within 10 days, the patient underwent orthotopic liver transplantation. Histopathology demonstrated hepatic necrosis and hepatic venous thrombosis consistent with acute BCS.
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PMID:Ultrasound and MR findings in acute Budd-Chiari syndrome with histopathologic correlation. 879 23

Infantile hemangioendothelioma is the most common vascular tumor in infancy. A three-month-old infant was admitted to the hospital for hepatomegaly. Abdominal ultrasonography, a CT-scan, and MRI studies demonstrated bilateral diffuse hepatic nodules, which were characteristic of hepatic hemangioendothelioma. A highly elevated alpha-fetoprotein (AFP) level misled us to an impression of hepatoblastoma, but the pathology report through an open biopsy disclosed a liver hemangioendothelioma. The patient responded to methylprednisolone therapy. A follow-up sonogram revealed regression of the hepatic masses. This case emphasizes that an elevated AFP level of up to 400 ng/ml is normally found in some neonates until two months of age. Careful interpretation of this value is very important, especially when it is associated with a hepatic tumor. Herein, we present a case of infantile hemangioendothelioma in a three-month-old boy with a highly elevated serum AFP.
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PMID:Infantile hemangioendothelioma with a highly elevated serum alpha-fetoprotein level. 963 27

The clinical, biochemical, pathological and neuroradiological findings of a 2-year-old Saudi boy with infantile G(M1) gangliosidosis are reported. The patient had a progressive neurologic deterioration, manifesting with developmental regression, sensorimotor and psychointellectual dysfunction and generalized spasticity that started at 4 months of age. Cherry-red macula, facial dysmorphia, hepatomegaly, exaggerated startle response to sounds, skeletal dysplasia, and vacuolated foamy lymphocytes that contain finely fibrillar material in addition to lamellar membranes and electron-dense rounded bodies were seen. MRI of the brain demonstrated mild diffuse brain atrophy and features of delayed dysmyelination and demyelination. Brain FDG PET scan revealed a mild decrease in the basal ganglia uptake, and moderate to severe decrease in thalamic and visual cortex uptake, and an area of increased glucose uptake in the left frontal lobe, probably representing an active seizure focus. The functional changes indicated by FDG PET scan and the structural abnormalities shown on MRI were found to be complementary in the imaging evaluation of infantile G(M1) gangliosidosis.
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PMID:Cerebral fluorine-18 labeled 2-fluoro-2-deoxyglucose positron emission tomography (FDG PET), MRI, and clinical observations in a patient with infantile G(M1) gangliosidosis. 1059 59


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