Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Disseminated visceral coccidiosis (DVC) caused by Eimeria spp. was recognized as a disease entity in captive sandhill cranes (Grus canadensis) and whooping cranes (Grus americana) in the late 1970s. While most avian species of Eimeria inhabit the intestinal tract of its host, the crane eimerians, Eimeria reichenowi and Eimeria gruis, invade and multiply systemically and complete their development in both digestive and respiratory tracts. In DVC, cranes, especially chicks, may succumb to acute infections resulting in hepatitis, bronchopneumonia, myocarditis, splenitis, and enteritis. Cranes may also develop chronic, subclinical infections characterized by granulomatous nodules in various organs and tissues. This paper reviews the pathology and pathogenicity of natural and experimental DVC in sandhill and whooping cranes. Naturally infected birds appeared clinically normal, but progressive weakness, emaciation, greenish diarrhea, and recumbency before death were observed in birds administered doses > or = 10 x 10(3) sporulated oocysts per os. At necropsy, naturally infected birds had nodules in the mucosa of the oral cavity and the esophagus, and in thoracic and abdominal viscera. Experimentally infected birds necropsied less than 7 days after infection (a.i.) had no gross lesions. Birds examined later had hepatosplenomegaly, liver mottling, lung congestion and consolidation with frothy fluid in airways, and turgid intestinal tracts with hyperemic mucosa. From 28 days a.i., grossly visible granulomatous nodules were seen in the esophagus, heart, liver, cloaca, and eyelids. By light microscopy, the basic host response was a granulomatous inflammation with non-suppurative vasculitis affecting many organs and tissues. With time, multifocal aggregates of mononuclear cells, many laden with asexual coccidial stages, increased in size and number. Widespread merogony resulted in morbidity and death, particularly in birds administered 20 x 10(3) sporulated oocysts. Ultrastructural examination revealed developing asexual coccidian stages in the cytoplasm of large lymphocytes or monocytes within a parasitophorous vacuole, often indenting the nucleus. Oocysts and gametocytes were found in the intestines by 12 days a.i., and in the esophagus, trachea, bronchi, and lung by 14 days a.i., indicating that crane eimerians can complete their life cycle at these sites. Thus, DVC in cranes could be a useful animal model for the study of eimerian extra-intestinal stages and the evaluation of potential systemic anticoccidial drugs.
 ...
PMID:Pathology and pathogenesis of disseminated visceral coccidiosis in cranes. 1522 53

Two cases of CD56+CD33+ leukemia/lymphoma are reported. The patient in case 1 presented with skin rash, diffuse lymphadenopathy, and hepatosplenomegaly. Blasts with monocytoid and lymphoid features were present in the peripheral blood. The tumor cells expressed HLA-DR, CD4, CD33, CD38, and CD56. Cytogenetic analysis revealed del(2)(p13),del(9)(q22),add(6)(q25),add(12)(p12),-13,-18, and -20. The clinicopathologic features were similar to those of blastic natural killer cell leukemia/lymphoma or type 2 dentritic cell leukemia. The patient in case 2 presented with generalized weakness and skin erythema not responding to antibiotics. Circulating blasts with monocytoid features were seen in the peripheral blood. The tumor cells expressed CD7, CD13, CD33, CD38, and CD56, and cytogenetic analysis revealed -5,add(7)(p22),-8, del(10)(p11.2),-12,der(13; 14)(p10;p10),+14,-16,-18,-19, and del(20)(q13.1). The clinicopathologic features were consistent with a myeloid/ natural killer cell precursor acute leukemia. Both disorders are aggressive hematopoietic malignancies that have similar clinical presentation and morphology but differ in immunophenotype and cytogenetic features.
 ...
PMID:Challenge in diagnosis of CD56+ lymphoproliferative disorders: two cases of CD56+CD33+ lymphoma/leukemia. 1527 May 96

The aim of this article is to present a case report of Gaucher disease which was diagnosed in patient with hepatosplenomegaly. A 43 years old man has complained of weakness, stomach pain and yellowish skin color for several years. The severity of symptoms has increased during the last 2 years. Laboratory data revealed thrombocytopenia (platelet count 108 G/l) and slightly elevated bilirubin level (1.68 mg/dl). In ultrasound examination hepatosplenomegaly was observed. Computer tomography evaluated the size of the spleen to 20 x 12 cm. Gaucher cells were found in bone marrow. The some type of cells was recognized in liver biopsy. To confirm diagnosis of Gaucher disease enzyme test was performed. Chitotriosidase level in serum was pronouncedly increased (11,540 nmol/mg protein/hr) while normal level is under 150 nmol/mg protein/hr. Glucocerebrosidase activity in leucocytes was within the limit. Thus the enzyme activity was 21.8 nmol/mg protein/hr and was below the normal range which is between 111-455 nmol/mg protein/hr. The last assay, measuring glucolukocerebrosidase activity in cultured fibroblast confirmed the diagnosis of Gaucher disease.
 ...
PMID:[Gaucher disease--one of the possible causes of splenomegaly--case report]. 1572 93

Myeloid/natural killer (NK) cell precursor acute leukemia is characterized by coexpression of myeloid and natural killer cell antigens and an aggressive clinical course. Here we report a case of myeloid/NK precursor acute leukemia in a 37-year-old woman. Clinical presentation was correlated with leukemic blast morphology, immunophenotype, and cytogenetic analysis. The patient had noted fever, weakness, purpura, peripheral lymphadenopathy, and moderate hepatosplenomegaly. Peripheral blood smears and bone marrow aspirate smears at presentation revealed blastic cells, which were generally L2 shaped, with variation in cell size, round to moderately irregular nuclei and prominent nucleoli, pale cytoplasm, and a lack of azurophilic granules. Immunophenotypic analysis of the blasts displayed coexpression of myeloid and natural killer cell antigens with relatively immature phenotype: CD7+, CD33+, CD34+, CD56+, CD57+, CD16-, MPO-. Cytogenetic analysis of marrow cells showed 62% of cells with a normal female karyotype; in the remaining 38%, tetraploid changes were detected, where the chromosome number was 92, with no preferential losses or gains of chromosomes. Fluorescence in situ hybridization analysis revealed the same abnormality. The patient did not respond to chemotherapy (cytosine arabinoside and idarubicin) and died of a septic complication on the 34th day after admission. To our knowledge, this is the first description of tetraploidy in myeloid/NK cell precursor acute leukemia.
 ...
PMID:Myeloid/natural killer cell precursor acute leukemia with tetraploidy. 1633 59

A 64-year-old man was diagnosed as having urticaria pigmentosa in 1998, and treated with PUVA therapy. In January 2002, X-ray imaging revealed osteosclerosis was detected in the systemic bone and bone scintigraphy. A bone marrow aspiration sample was not obtained due to a dry tap. CT scans showed hepatosplenomegaly and mesenteric lymphadenopathy. Myelofibrosis and diffuse mast cell infiltration were revealed by a bone marrow biopsy, and a diagnosis of systemic mastocytosis with severe osteosclerosis and myelofibrosis was made. In October 2003, he was admitted to our hospital because of mid back pain. A neurological examination showed muscle weakness in the upper and lower limbs, sensory disturbance below the level of Th4 and urinary obstruction. T1 and T2 weighted images of MRI demonstrated a high intensity epidural mass lesion extending from the vertebral level of C5 to Th2 and severely compressing the spinal cord. We considered the possibility of the invasion of the spinal canal by the mastocytosis. The patient was treated with interferon alpha-2b (IFN-alpha2b) and prednisolone. Subsequently, the motor and sensory disturbances were gradually alleviated, and spinal MRI confirmed a marked reduction in the size of the epidural tumor. However, the patient became resistant to interferon, and died of multiple organ failure in spite of steroid pulse and cladribine therapies. Multiple organ infiltration by mast cells was revealed at autopsy.
 ...
PMID:[Case of intraspinal epidural tumor developing after systemic mastocytosis with marked osteosclerosis and myelofibrosis]. 1644 Jul 79

Total 14 cases of myeloma in young age group (<40 years) have been reported out of 178 cases of myeloma in a time period of 7 years (1993-1999). Males predominated overfe males. Like adult myeloma, patients presented mostly with the backache, pain in pelvis, lower spine and weakness in about 60% of cases followed by swelling of bone in 40% of cases. One case presented with bleeding gum, malena and hepatosplenomegaly and was diagnosed as plasma cell leukemia. Radiological examination revealed lytic lesion in almost all the cases with fracture femur and rib in 28.57% of cases. Anaemia and raised ESR was noted in all the cases. Myeloma typing revealed IgG myeloma in 10 cases, light chain myeloma in 3 cases and IgA myeloma in one case. None of the patient was traceable after 2 years. Thus our study concludes that myeloma in the young age in India occurs in increased frequency and clinically presents just like adult and elderly myeloma, but serologically are predominantly of IgG type. There is also an increased frequency of solitary plasmacytoma as compared to adult myeloma.
 ...
PMID:Myeloma in young age. 1676 40

Eosinophilic fasciitis is a rare connective tissue disease in children characterized by hardening and thickening of the skin and soft tissues, peripheral eosinophilia, elevated erythrocyte sedimentation rate, and hypergammaglobulinemia. In this study, we report three pediatric patients with eosinophilic fasciitis whose clinical presentation and response to therapy differed from those reported in the literature. All three patients were female. Two of them had Raynaud's phenomenon as an initial feature, and the third had hepatosplenomegaly. Muscle involvement with weakness was present in two patients, as documented not only by increased aldolase levels at the time of presentation but also by inflammatory changes in the muscle biopsy. Serum immunoglobulin G levels were increased only in the most severely involved patient. Peripheral eosinophilia was present in all three cases. All three patients responded well to prednisone therapy. Two patients had a uni-phasic course, and one required further therapy to control a postinfection relapse. In summary, we found that Raynaud's phenomenon and hepatosplenomegaly can be part of the spectrum of clinical manifestations of childhood eosinophilic fasciitis. Identification as eosinophilic fasciitis and not scleroderma, despite hand involvement and Raynaud's phenomenon, can suggest that the illness may be steroid responsive.
 ...
PMID:Faces of eosinophilic fasciitis in childhood. 1704 31

A neuronal storage disease affecting 5 captive Humboldt penguins is described. One bird died after 3 days of lethargy and anorexia. The 4 remaining birds died after a slowly progressing course of disease with signs that included lethargy, weakness, and neurologic dysfunction. Neurologic signs included dysphagia and ataxia. Gross lesions in the first animal to die consisted of hepatosplenomegaly indicative of avian malaria, which was confirmed histologically. The 4 remaining animals were mildly to moderately emaciated. Moderate to marked vacuolation of the neuronal perikarya was observed in Purkinje cells, neurons of the brainstem nuclei, and motorneurons of the spinal cord in all birds. By electron microscopy the vacuoles represented multilayered concentric lamellar structures. These findings were indicative of sphingolipidosis. All animals had been prophylactically treated for avian malaria, aspergillosis, and possible bacterial infections with chloroquine, itraconazole, and enrofloxacin. Circumstantial evidence implicates chloroquine therapy as the possible cause of the storage disease.
 ...
PMID:Neuronal storage disease in a group of captive Humboldt penguins (Spheniscus humboldti). 1709 67

A 28-year-old normotensive euthyroid man presented with recurrent lower motor neuron type of weakness without sensory or autonomic involvement, with preserved reflexes. Systemic examination was significant for mild hepatosplenomegaly. Investigations revealed persistent hypokalemic, hyperchloremic, normal-anion-gap metabolic acidosis with deranged liver functions. Urine pH was 6.0 even after oral ammonium-chloride loading test. Type I renal tubular acidosis was diagnosed. A search for the etiology revealed bilateral Kayser-Fleischer ring, with low serum ceruloplasmin levels and high urinary copper, confirming it to be Wilson's disease.
 ...
PMID:Recurrent limb weakness as initial presentation of Wilson's disease. 1740 Dec 35

Histoplasmosis is a very rare disease in Korea. Clinical manifestations are very similar to those of tuberculosis. This is the first case report of combined disseminated histoplasmosis and tuberculosis in a patient with HIV infection in Korea. A 42-year-old Korean with Acquired Immunodeficiency Syndrome (AIDS) was diagnosed with tuberculosis. He had lived in Guatemala for the past five years. Upon diagnosis of disseminated tuberculosis with HIV infection, he was treated with anti-tuberculosis medications and anti-retroviral agents. Fever, weakness, hepatosplenomegaly and pancytopenia were persistent despite treatment. The patient's history of living in Guatemala caused us to seek opportunistic infectious organisms other than tuberculosis. Bone marrow aspiration and biopsy were performed and the result revealed numerous intracellular organisms consistent with Histoplasma capsulatum; therefore, the diagnosis of disseminated histoplasmosis was made.
 ...
PMID:Disseminated histoplasmosis and tuberculosis in a patient with HIV infection. 1759 64


<< Previous 1 2 3 4 5 6 7 8 9 Next >>