Cases reported "Hepatomegaly"

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1/20. Hepatic amyloidosis with light chain deposition disease. A rare association.

    Monoclonal immunoglobulin deposition diseases are due to pathological protein deposition in various tissues and organs. Protein deposits may be found in a single tissue or systemically and the organs most frequently involved are kidney, heart, peripheral nerves and the liver. Depending on the pattern of the deposits and the type of immunoglobulin, these diseases are distinguished as primary amyloidosis, light chain deposition disease. Differential diagnosis is made in tissue specimens: microscopically by the identification of positive congo red staining of the deposits, by immunohistochemical demonstration of proteins reacting with light chain (lambda or kappa) antisera or by recognition of fibrillar structures on electron microscopy. We report an unusual case of light chain deposition disease associated with amyloidosis, where hepatomegaly was the presenting manifestation and liver failure the cause of death, without any kidney involvement.
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2/20. 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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3/20. Granulomatosis associated to porcelain wear debris.

    PURPOSE: To determine the origin of a cryptogenic granulomatosis using an innovative diagnosis technique. MATERIALS AND methods: A patient affected by fever of unknown origin for 9 years was diagnosed with colestasis and acute renal failure with pathological evidence, in parenchimal samples, of granulomatosis of unknown origin. New scanning electron microscopic observations on the biopsy samples from the liver and the kidney and x-ray elemental microanalyses showed the presence of debris made of silicone, aluminum, sodium and potassium, and aluminum-silicate similar to dental porcelain. The same SEM and x-ray analyses were carried out on the patient's worn porcelain dental bridges. RESULTS: A correlation was demonstrated between wear debris of porcelain and the cryptogenic granulomatosis, which lead to a different therapeutic approach and the removal of the origin of the debris; this stabilized the situation and caused an improvement of the disease. The results indicated that a material can be biocompatible when used in a solid bulk, but this property can be lost when it is degraded into small particles.
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4/20. Unusual prenatal presentation of beckwith-wiedemann syndrome.

    When beckwith-wiedemann syndrome (BWS) is detected prenatally, it is usually on the basis of macroglossia, exomphalos or enlarged kidneys. We describe a case that presented as gross hepatomegaly and a suspected enlarged pancreas at 20 weeks' gestation, with none of the usual features.
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5/20. Congenital hepatic fibrosis: a very uncommon cause of pancytopenia in children.

    The disease presentation of autosomal recessive polycystic kidney disease (OMIM #263200, ARPKD) is highly variable and includes polycystic kidneys, pulmonary hypoplasia, and congenital hepatic fibrosis. The authors report an unusual case of ARPKD presenting with hepatosplenomegaly and cytopenia mimicking acute leukemia.
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keywords = kidney disease, kidney
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6/20. Pelvic insufficiency fracture related to autosomal dominant polycystic kidney disease.

    We report the case of a patient with autosomal dominant polycystic kidney disease (ADPKD) and an insufficiency-type fracture of the pelvis. A 60-year-old Japanese woman was admitted because of pain in the right ischium and pubis that began suddenly with no precipitating cause. Computed tomography showed the bony pelvis to be compressed by enlarged dependent kidneys and an enlarged liver. We relieved compression on the pelvic bones by means of transarterial embolization (TAE) to the kidneys and liver after initiation of hemodialysis therapy. The fracture healed gradually after TAE, and the patient could walk 4 months later. In an iliac bone specimen obtained before TAE, cancellous bone was intact, but periosteal and endosteal surfaces of cortical bone showed marked resorption and were irregular. Normally, many ligaments are connected tightly to the periosteal surface, supporting the cortical bone. However, because of extensive surface resorption associated with pressure from enlarged kidneys, connections between ligaments and the periosteal surface presumably became fragile, promoting an insufficiency fracture from unapparent external forces. Thus, ADPKD is a potential cause of insufficiency fracture owing to abnormalities of cortical bone.
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ranking = 121.96270496837
keywords = kidney disease, kidney
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7/20. liver transplantation for liver rupture due to light chain deposition disease: a case report.

    light chain deposition disease (LCDD) is a rare pathologic condition distinct from amyloidosis. amyloidosis is most often characterized by overproduction of lambda light chains, while kappa chains are overproduced in LCDD. In contrast to amyloid deposits, those of LCDD do not stain with congo red and have a granular ultrastructure. LCDD primarily affects the kidney; clinically significant liver dysfunction is less common and less severe than renal disease. We describe a case of kappa chain deposition disease in a patient with plasma cell dyscrasia and platelet pool storage defect, which produced massive hepatomegaly and rupture of the liver leading to orthotopic liver transplantation. The liver weighed 6800 g and showed severe atrophy due to massive deposition of light chains. In this case, the deposits were composed of unbranched fibrils, which measured 12 to 20 nm in width, did not possess a hollow core, and were arranged randomly rather than in structured arrays.
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8/20. Massive cystic hepatomegaly in a female patient with polycystic kidney disease treated by combined hepatic and renal transplantation.

    A patient with cystic kidney disease of adult onset and severe cystic hepatomegaly is presented. The patient was severely disabled solely by her abdominal bulk. Simultaneous liver and renal transplantation was undertaken successfully.
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ranking = 120.46270496837
keywords = kidney disease, kidney
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9/20. Chronic Niemann-Pick disease with sphingomyelinase deficiency in two brothers with mental retardation.

    Clinical, biochemical, and electron microscopic studies are presented in two brothers with Niemann-Pick disease. The clinical features include hepatosplenomegaly and mental retardation without any other neurological signs. Roentgenograms of the chest showed bilateral diffuse reticular infiltration. The amounts of sphingomyelin and cholesterol in liver were increased, and sphingomyelinase activities in both liver and skin fibroblasts were markedly reduced in Case 1. Numerous foam cells and myelin figures were observed in the liver, kidneys, bone marrow, and lymph nodes on electron microscopical examination. These cases were regarded as a variant of Niemann-Pick disease from our investigations as they have mental retardation as an exceptional symptom when they are diagnosed as type B.
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10/20. Massive hepatomegaly in adult polycystic liver disease.

    In adult polycystic liver disease, the liver gradually enlarges as it is replaced by cysts. The disease rarely produces symptoms or complications. Liver cysts are thought to arise from aberrant embryonic intrahepatic bile ducts (Von Meyenburg's complexes). We present a case of adult polycystic liver disease with Von Meyenburg's complexes and unusually severe hepatomegaly (7.7 kg, 22,080 cm3). The autopsy prevalence of adult polycystic liver disease at UCLA Medical Center is 0.13%; 93% of these cases had polycystic kidney disease. adult polycystic kidney disease had associated liver cysts in 45% of cases.
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ranking = 24.592540993673
keywords = kidney disease, kidney
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