Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The effect of polyinosinic-polycytidylic acid (poly I:C) treatment on the rat graft-versus-host reaction (GVHR) initiated in parental to F1 hybrid strain combinations differing at either major or minor histocompatibility determinants were studied in three different protocols. 1 A GVHR initiated in juvenile (LBN)F1 recipients and treated concurrently with poly I:C alone produced neither splenomegaly nor dermatitis in these juvenile rats. (2) Pretreatment of L donors with a single injection of poly I:C 3 days before initiation of the GVHR enhanced resultant splenomegaly in the newborn (LBN)F1 recipients. A high poly I:C dose was inhibitory. (3) Newborn recipients which received lymphocytes from L donors and which were concurrently treated with poly I:C developed dermatitis at an accelerated rate. However, poly I:C alone given to newborns mimicked the GVHR by induction of a syndrome characterized by splenomegaly, dermatitis, thymic involution, and body growth retardation. The parental L and (LF)F1 hybrid strain combination differing only at a minor histocompatibility determinant or in an isogenic hybrid combination (LBN)F1 leads to (LBN)F(1) developed no GVHR when recipients were treated with poly I:C. We conclude that poly I:C can stimulate a rat GVHR initiated with unsensitized donor cells differing at a major histocompatibility locus.
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PMID:Stimulation of rat graft-versus-host reactions by polyinosinic-polycytidylic acid. 23 51

Herein we report a new familial form of hepatic disease. Each of the four patients had splenomegaly, hypersplenism, a small liver, biochemical evidence of hepatic excretory dysfunction and hepatocellular damage, kidneys without demonstrable cysts, and normal blood pressue. An evaluation of serum immunoproteins, autoantibodies, histocompatibility antigens, and mixed lymphocyte reactivity further defined the immunologic features of this syndrome. Extrahepatic manifestations included a papulosquamous dermatitis with deposition of immunoglobulins and complement in both normal and abnormal skin, a membranoproliferative glomerulonephritis with subendothelial deposits, arthritis, and pericardial, pleural, and synovial effusions.
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PMID:The association of familial liver disease, subepidermal immunoproteins, and membranoproliferative glomerulonephritis. 87 Jun 58

The second documented case of prolidase deficiency is presented. Clinical manifestations include chronic otitis media and sinusitis, dermatitis, and splenomegaly. Prolidase is undetectable in the white blood cells of the patient and near or less than the lower range of normal in each parent. The peptide chromatographic pattern of the urine is similar to that of the previously reported patient with prolidase deficiency. The quantity of amino acids excreted in urine per 24 hours is at least three times that of the upper range of normal (of these same amino acids) for the patients age group. More than 80% of the total amino acids excreted are in peptide form. The proline-to-hydroxyproline ratio suggests that the dipeptides are the catabolic products of other proteins in addition to collagen.
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PMID:Prolidase deficiency: report of a second case with quantitation of the excessively excreted amino acids. 87 81

A graft-vs.-host (GvH) reaction can be initiated by injection of immunocompetent lymphocytes into a histoincompatible host that is unable to reject these cells. The reaction is characterized by splenomegaly, hepatomegaly, lymph node atrophy, body weight loss, dermatitis, and diarrhea, often leading to mortality. The onset and severity of the GvH reaction are determined by differences in histocompatibility antigens between the donor and the acceptor, and by the number and nature of the transplanted allogeneic cells. Many different in vivo and in vitro systems have been devised for experimental studies of the GvH reaction. In several of these models, however, different parameters are measured. Furthermore, the conclusions drawn from these investigations sometimes contradict each other. This paper reviews the experimental data, and discusses the mechanisms underlying the GvH reaction.
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PMID:Graft-vs.-host reactions: mechanisms and contemporary theories. 315 48

Toxoplasmosis was diagnosed in a free-ranging wild turkey (Meleagris gallopavo) from West Virginia (USA) in June 1993. Gross findings included emaciation, splenomegaly, multifocal necrotizing hepatitis and splenitis, and crusting dermatitis on the head and neck. Histologically, multifocal necrosis with mononuclear inflammation was present in kidney, liver, spleen, heart, lungs, and pancreas. Toxoplasma gondii was confirmed in sections of liver by avidin-biotin immunohistochemical analysis. Subsequently, a retrospective serosurvey of wild turkeys for T. gondii antibodies was conducted using turkey sera collected between 1984 and 1989. An antibody prevalence of 10% was detected in 130 birds from 21 locations in the southeastern United States. While wild turkeys in the Southeast have T. gondii antibodies, this is only the second natural case of fatal toxoplasmosis reported; it appears that wild turkeys infrequently develop clinical disease when infected with T. gondii.
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PMID:Toxoplasmosis in wild turkeys: a case report and serologic survey. 858 48

A 69-year-old woman was admitted with generalized lymph node swelling and huge splenomegaly. CD5(+), Sm-IgM (+) and SmIgD (+) lymphocytes were increased in lymph nodes, spleen and bone marrow, and she was diagnosed as having mantle cell lymphoma. A diagnosis of hyper-IgE syndrome was also made, because IgE was markedly increased (174,780 u/ml) and chronic dermatitis, which was often complicated with infection, occurred repeatedly on her extremities. In this case, interleukin-4 was considered to be one of the factors involved in the hyper-IgE syndrome, because increased IgG1 and reduced IgG2 were observed. Immunological abnormality associated with the hyper-IgE syndrome seemed to contribute to the development malignant lymphoma in this case.
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PMID:[Mantle cell lymphoma associated with hyper-IgE syndrome]. 899 29

Cockayne syndrome (CS) is a rare autosomal recessive disorder characterized by growth retardation, senile-like appearance, loss of subcutaneous adipose tissue, photosensitive dermatitis, microcephaly, deafness, pigmentary degeneration of retina, disproportionately long limbs, skeletal malformations with knee contractures and neurologic abnormalities. This is a description of a three-year-old boy with typical features of Cockayne syndrome complicated with tetralogy of Fallot, pneumonia and hepato-splenomegaly. He had been suffering from frequent attacks of pneumonia and had been hospitalized for several times since birth. Tetralogy of Fallot was diagnosed under echocardiography study and he died suddenly in hospital during a mydriatic procedure in the Ophthalmologic Clinic.
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PMID:Cockayne syndrome with tetralogy of Fallot: a case report. 919 97

A null mutation was prepared in the mouse for CD18, the beta2 subunit of leukocyte integrins. Homozygous CD18 null mice develop chronic dermatitis with extensive facial and submandibular erosions. The phenotype includes elevated neutrophil counts, increased immunoglobulin levels, lymphadenopathy, splenomegaly, and abundant plasma cells in skin, lymph nodes, gut, and kidney. Very few neutrophils were found in spontaneously occurring skin lesions or with an induced toxic dermatitis. Intravital microscopy in CD18 null mice revealed a lack of firm neutrophil attachment to venules in the cremaster muscle in response to N-formyl- methionyl-leucyl-phenylalanine. A severe defect in T cell proliferation was found in the CD18 null mice when T cell receptors were stimulated either by staphylococcal enterotoxin A or by major histocompatibility complex alloantigens demonstrating a greater role of CD11/CD18 integrins in T cell responses than previously documented. The null mice are useful for delineating the functions of CD18 in vivo.
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PMID:Spontaneous skin ulceration and defective T cell function in CD18 null mice. 965 89

A 50-year-old patient, suffering from familial polyposis (Gardner's syndrome), initially presented with several nodules on his left arm. Histological examination revealed primary cutaneous T-cell-rich B-cell lymphoma (TCRBCL). Staging procedures failed to detect any systemic involvement. Three years after total excision of the tumours, the patient presented with a non-specific dermatitis, enlarged axillary lymph nodes and splenomegaly. Histological and immunohistochemical examination of lymph node and spleen biopsy specimens resulted in the diagnosis of Hodgkin's disease (HD) of the nodular sclerosis type. Sequence analysis of single cells micromanipulated from skin and from lymph node lesions indicated that both lymphoma infiltrates were derived from the same precursor germinal centre B-cell clone. This is a case showing a clonal relationship between TCRBCL and HD, providing support to the B-cell origin of Hodgkin and Reed-Sternberg cells.
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PMID:Primary cutaneous T-cell-rich B-cell lymphoma and Hodgkin's disease in a patient with Gardner's syndrome. 1114 53

Prolidase deficiency is a rare, inherited disorder characterized by ulceration of the skin, mental retardation, and massive urinary excretion of imidodipeptides. Most patients also have recurrent infections, an unusual facial appearance, and splenomegaly. We describe a girl presenting with chronic dermatitis, recurrent respiratory tract infections since her first months of life, and facial features characteristic of prolidase deficiency. The diagnosis of prolidase deficiency was made at 4.5 months of age. The immunologic study in this patient showed an extreme and progressive increase of total immunoglobulin E (IgE) in serum (reaching the value of 77,600 IU/l) and defective chemotactic function of the neutrophils. Treatment with a hyper-proteic diet supplemented with ascorbic acid, manganese chlorite, and topical proline resulted in reduction of the frequency and severity of the infections and significant improvement of the skin lesions. The authors discuss the immunologic alterations and the favorable evolution with treatment in this patient.
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PMID:Prolidase deficiency with hyperimmunoglobulin E: a case report. 1200 Apr 88


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