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

An inappropriate, sustained and absolute lymphocytosis in a young man was investigated using technics which attempted to define the more detailed features of these cells and their T and B subpopulations. The results of the tests for lymphocyte RFC (E) and complement (EAC) rosette formation, immunofluorescence, in vitro phytohemagglutin (PHA) stimulation and 14C-cyclo-phosphamide binding assays, when evaluated in a combined and interrelated fashion, indicated that the lymphocytosis was mainly but not solely, due to an absolute elevation in circulating T lymphocytes. Moreover, an aneuploid cell line with 47 chromosomes, presumably T cells, also was detected in PHA-stimulated cultures. These data, associated with the patient's anemia, lymphocyte-infiltrated bone marrow, hepatosplenomegaly, pharyngeal lymphoid tumor and the clonal proliferation of lymphocytes bearing an abnormal karyotype, furnish evidence that the lymphocytosis is more than an unremitting leukemoid reaction and is potentially neoplastic. The detection of the chromosomal abnormality at this patient's age and point in disease may make him especially unique in that he could be a clinical prototype for other patients with lymphoreticular tumors which remain undetected at their onset.
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PMID:Persistent lymphocytosis with chromosomal evidence of malignancy. 109 66

Two cases of infection (miliary tuberculosis and a presumed viral infections) are described in which phagocytosis of erythrocytes, leukocytes, and platelets by reticuloendothelial cells was a prominent feature in bone marrow aspirates, associated with a clinical picture of fever, anemia, malaise, and hepatosplenomegaly. All these findings were "transient," and disappeared on treatment of the underlying infection.
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PMID:Transient histiocytosis with striking phagocytosis of platelets, leukocytes, and erythrocytes. 115 59

Zinc takes part in the catalytic function of many metalloenzymes. In others it plays a role in conformational stability. In zinc deficient animals protein synthesis is disturbed. Conversely zinc metabolism is influenced by protein deficiency. Zinc takes part in drug metabolism, in mobilizing vitamin A from the liver, and in a system defending the organism against free radical damage. Zinc distribution in the organism is influenced by steroid hormones and leucocytic endogenous mediators. Of the intracellular zinc only a small part is bound to metalloenzymes, most being coordinated to binding sites of nonspecific proteins. Thus the organism defends itself against conformational changes of irritable enzymes which may bind excess zinc to side chains. Zinc can protect the organism against cadmium toxicity. In the serum the smaller part of zinc is firmly bound to several specific proteins, the majority being loosely bound to albumin. Some aspects of human zinc metabolism in health and disease are reviewed. Zinc deficiency in man is rare. In Iran and Egypt a syndrome of iron and zinc deficiency associated with anaemia, hepatosplenomegaly, dwarfism, and hypogonadism is known. In wound healing and tissue repair substitution of zinc is beneficial only if a zinc deficiency exists. For purposes of long term parenteral nutrition zinc should be added to the different infusion solutions.
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PMID:[The biological significance of zinc (author's transl)]. 120 Mar 35

Two cases of disseminated histoplasmosis caused by H. capsulatum in Nigerian children are reported. This is a rare infection in this part of the world. The main clinical features were fever, weight loss, lassitude, lymphadenopathy, hepatosplenomegaly and severe anaemia, features indistinguishable from those of tuberculosis, Hodgkins and other reticuloses. Recognition of this infection in this environment is possible if it is considered in the differential diagnosis of pyrexia of undetermined origin and appropriate laboratory tests carried out on suitable specimens such as bone marrow, splenic aspirate or biopsy material. Treatment of choice is amphotericin B given intravenously, starting with 0-25 mg/kg. and increasing slowly to 1 mg/kg. Other useful drugs are Septrin and rifampicin which can be given concurrently. Subcutaneous abscesses and multiple bone lesions occurred in both our cases presumably as a result of blood stream infection, or embolisation from endocarditis.
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PMID:Disseminated histoplasmosis due to histoplasma capsulatum in two Nigerian children. 122 26

1) Geophagia characterized by, severe, anaemia, dwarfism, hypogonadism and hepatosplenomegaly is sometimes seen in young patients (and children) in Iran. 2) Haematological aspects of the syndrome are those of, severe, iron deficiency anaemia. 3) Gastric biopsies and histological findings revealed superficial or atrophic gastritis showing some resemblance to those seen in pernicious anaemia. 4) Haematological features, anaemia and many of the clinical signs of the syndrome were improved after appropriate iron therapy. 5) Histological changes of gastric mucosa improved, in 5 patients, 6 months after correction of the anaemia.
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PMID:Studies on clinical, haematological aspects and pathological changes of gastric mucosa in geophagia. 123 30

A fatal case of an apparently congenital form of hemophagocytic reticulosis is reported. The onset was manifested by hyperbilirubinemia and hepatosplenomegaly which were present at birth and persisted throughout life. Fever, anemia and pancytopenia developed at 1 month of age and became progressively worse. A splenectomy was performed at the age of 3 months, but the child died one day later with disseminated intravascular coagulation and pulmonary hemorrhage. The literature is reviewed with regard to the relationship of this case to (familial) hemophagocytic reticulosis and malignant histiocytosis (histiocytic medullary reticulosis). It is suggested that congenital hemophagocytic reticulosis, as described here, (familial) hemophagocytic reticulosis in infants, and malignant histiocytosis in adults all represent the same basic disorder with different ages of onset and clinicopathologic manifestations.
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PMID:Congenital hemophagocytic reticulosis. 126 10

A 21-year-old woman was admitted to our hospital because of high fever, cough, and headache. Other clinical manifestations included hepatosplenomegaly, anemia, leukopenia, and mild liver dysfunction. As she had been diagnosed to have systemic lupus erythematosus (SLE) previously, we first suspected that this disease had become exacerbated. However, the titers of anti-EB virus (VCA-IgG) and some other viruses were high on admission, and hemophagocytosis by macrophages was observed on bone marrow examination. Therefore, she was diagnosed to have virus-associated hemophagocytic syndrome (VAHS). Prednisolone therapy was then initiated, but the patient responded poorly. We next tried gamma globulin, and the clinical findings and laboratory data were improved. Our patient showed a very favourable clinical course following gamma globulin therapy, suggesting that steroid and gamma globulin should be considered as a treatment even in the early stages of this syndrome in adults.
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PMID:[Steroid and gamma globulin therapy against virus-associated hemophagocytic syndrome]. 127 36

A 14-year-old boy presented with fever, anemia, hepatosplenomegaly, generalized lymphadenopathy and nephrotic syndrome. Lymph node biopsy showed angiofollicular lymph node hyperplasia (generalized Castleman's disease) of the plasma cell type. Kidney biopsy showed membranoproliferative glomerulonephritis type 1. Complete remission was achieved with corticosteroid treatment and repeat kidney biopsy 22 months later showed complete resolution of the renal pathology. The association between membranoproliferative glomerulonephritis and multicentric angiofollicular lymph node hyperplasia, plasma cell type, has not previously been reported.
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PMID:Membranoproliferative glomerulonephritis associated with multicentric angiofollicular lymph node hyperplasia. Case report and review of the literature. 129 48

One hundred and ten children with hemoglobin H (Hb H) disease who attended the hematology unit of the Department of Pediatrics at Songklanagarind Hospital from 1982 to 1988 were studied. Hb Constant spring (Hb CS) was found in 55 patients. Four patients, two with Hb CS, were diagnosed during the newborn period. Anemia and jaundice were the main symptoms in three neonates, while the fourth one was found to have anemia with hepatosplenomegaly. Nine infants were diagnosed in the first year of life with the chief symptoms of anemia with or without fever. Two of them needed blood transfusions. Hb H was found in only three infants, while Hb Bart's was the constant finding in every infant. The Hb H children with Hb CS had a more severe clinical course than the group without Hb CS. The levels of Hb at steady state were found to be lower and the reticulocyte counts, red cells with inclusion bodies and Hb H were higher in patients with Hb CS. The clinical picture of acute hemolysis in Hb H children can be found in the neonatal period and the difference in clinical severity between the two genotypes of Hb H disease seems to develop from the first year of life.
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PMID:Hemoglobin H disease in children. 130 83

The primary complex like Ghon was observed in a child's clinical roentgenographic study. C.S., white, male, 6 years old, was born in Curitiba (PR), Brazil and living in Guaratingueta (SP), Brazil, developed "common cold", bimodal diary fever, chills, shake and sweats. Dyspnea, cough with general lymphadenopathy. Foot and right shoulder arthralgias. Six months ago visited a cave, equitation practice, dog and cat contacts and no transfusion, frontal sweats, fever (38.4 degrees C). T.A. was 8/6, tachycardia in generalized lymphadenopathy. Cardiopulmonary system was normal, mesogastric tumoral mass, hepatosplenomegaly and no ascites. Bone marrow with eosinophilia; nodule demonstrated presence of P. brasiliensis, hypoalbuminemia; hyperglobulinemia; anemia; leukocytosis with eosinophilia. Immunodiffusion with exoantigen 43 kd of P. brasiliensis was 1/32. Primary complex like Ghon was observed in interstitial pneumonia followed by mediastinal and mesogastric mass (35 to 40 days). Clavicular osteolytic lesions (45 to 60 days) appeared during paracoccidioidomycosis therapy. Recovery was observed 2 months after treatment of acute infantile paracoccidioidomycosis.
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PMID:[Pulmonary lymph node in acute juvenile paracoccidioidomycosis (a case report)]. 130 53


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