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

The experience of the University of Colorado Medical Center affiliated hospitals with leukemic reticuloendotheliosis, hairy cell leukemia, during the past two years has been reviewed. Eight instances were found. The majority of patients in this study presented with fatigue, pancytopenia and splenomegaly. Diagnosis was based upon finding characteristic hairy cells in the blood, bone marrow or spleen. Treatment, unlike other hematopoietic malignant conditions, was primarily surgical, with splenectomy being the treatment of choice, which may lead to prolonged remission in the majority of instances.
Surg Gynecol Obstet 1978 Sep
PMID:Surgical leukemia. 68 93

We describe nine Spanish-American children from five families with an unusual hereditary lipid storage disease. The family origins were in two small southern Colorado towns. The clinical course varied, but all of the children were found to bruise easily and to have splenomegaly, while most had hepatomegaly. Post-natal jaundice and hepatitis occurred in four. Impairment of vertical gaze and intellectual and neurologic deterioration occurred in most of the patients, with the onset of the disease, usually in childhood. The bone marrow in all patients examined contained both foamy and sea-blue histiocytes. Sphingomyelinase levels in skin fibroblast cultures were greatly decreased in seven of the eight cases evaluated. It is believed that these patients have a sphingomyelin lipidosis and represent a variant of the Niemann-Pick disease. Clinical and enzymatic findings are compared with those of other cases in the literature.
Am J Dis Child 1977 Sep
PMID:Nine cases of sphingomyelin lipidosis, a new variant in Spanish-American Children. Juvenile variant of Niemann-Pick Disease with foamy and sea-blue histiocytes. 90 82

A study of liver abnormalities in 36 patients with mixed cryoglobulinemia in the absence of underlying infectious, connective tissue, or lymphoproliferative disorders revealed clinical or biochemical evidence of liver dysfunction in 84%. Hepatomegaly was detected in 77%, splenomegaly in 54%, and abnormalities in bilirubin, alkaline phosphatase, or serum glutamic oxalacetic transaminase in 77%. Only four of the patients had overt liver disease. Of 15 biopsies from 12 patients, there was normal tissue structure in two, minimal nonspecific changes in one, portal fibrosis in three, chronic persistent hepatitis in one, chronic active hepatitis in two, chronic active hepatitis with cirrhosis in four, and postnecrotic cirrhosis in two. These findings, together with the previously reported high incidence of serologic evidence of hepatitis B virus (HBV) infection, support the view that the syndrome of purpura, arthritis, and nephritis is often a consequence of immune-complex vasculitis secondary to HBV infection.
Ann Intern Med 1977 Sep
PMID:Liver involvement in the syndrome of mixed cryoglobulinemia. 90 Jun 72

Thrombocytopenia is often one of the first signs of vinylchloride disease. Morphological investigations showed that it is caused by splenic alterations. Besides splenomegaly, there are significant structural changes in the red pulp cords affecting the microcirculation. These quantitative and qualitative alterations cause increased platelet pooling and and increased destruction of platelets, which result in a decrease in thrombocytes in the peripheral blood.
Blut 1977 Sep 18
PMID:[Aetiology of the thrombocytopenia in vinylchloride disease (author's transl)]. 91 12

1. Equine infectious anemia (EIA) is an immunologically-medicated disease. Immune complexes formed in blood and tissues are responsible for most symptoms and lesions (anemia, splenomegaly, lymphadenopathy, glomerulonephritis, etc.). In addition, a state of cellular hypersensitivity of the delayed type is involved in the pathogenesis. 2. Periodical attacks of pyrexia and clinical illness in the presence of immunity are caused by antigenically-modified variants of virus. By means of immunosuppressive treatments similar relapses of fever associated with the appearance of new virus variants can be also provoked during longlasting asymptomatic periods. 3. The mechanisms responsible for the lifelong persistence of virus are not fully elucidated. Obviously of prime importance is the viral antigenic drift allowing the virus to escape from humoral and cellular immune reaction. Finally, however, a state of cell-mediated immunity ensuring protection against homologous and heterologous virus strains may be reached. 4. Pathogenetic analogies and differences existing between EIA and other chronic viral infections of animals are recorded.
Bull Schweiz Akad Med Wiss 1977 Sep
PMID:[Pathogenesis of equine infectious anemia (with reference to similar chronic viral infection)]. 91 50

Besides lymphodenopathy and splenomegaly, hepatomegaly may also be detected in 25-50% of children with juvenile rheumatoid arthritis. This is particularly evident in patients with complete Still's syndrome. The hepatomegaly increases during relapse situations and disappears during remissions. Transient icterus, elevation of aminotransferases and delayed bromsulfalein excretion have been reported, particularly in patients with complete Still's syndrome, and indicate impairment of liver function. Liver biopsies have been performed only rarely and show nonspecific infiltrations of portal fields with lymphocytes and, in a few cases, "autoimmune" hepatitis and even cirrhosis with portal hypertension. Plasma cell hepatitis with affection of joints can be readily differentiated from juvenile rheumatoid arthritis: the synovitis is merely transiet and disappears with institution of steroid therapy. As in the adult, severe liver dysfunction leads to remission of arthritis. Amyloidosis should be considered in every case of long-lasting hepatomegaly.
Schweiz Med Wochenschr 1977 Sep 03
PMID:[Liver pathology in juvenile chronic polyarthritis]. 91 83

Patients who run a fulminating course in association with histologically proven myelofibrosis are distinctly unusual, since the natural history of this entity is characteristically one of slow progression. Because of its rarity and proteam manifestations, acute myelofibrosis may easily go unrecognized. We report 2 such patients. In one, rapid clinical deterioration was dominated by spreading skin lesions, and in the other by refractory intravascular haemolysis. There was no splenomegaly in the first patient, and it was minimal in the second. Although it is seen in frequently, it should be emphasized that myelofibrosis may arise de novo as an acute illness in which the usual degree of splenomegaly is absent.
S Afr Med J 1977 Sep 17
PMID:Acute of fulminating myelofibrosis. 91 96

After an episode of abacterial meningitis in a 5-year-old boy there developed splenomegaly, uveitis, and a granulomatous arteritis of the lower limbs requiring amputation of one leg. Immune complexes persist in the blood, but the precise antigenic stimulus remains conjectural.
Arch Dis Child 1977 Sep
PMID:Giant cell arteritis with gangrene in a child. 92 24

The use of ultrasound in obstetrics is well established; however, its use in nonobstetric disease during pregnancy has not been emphasized. A diagnostic work-up during pregnancy is complicated by the fact that many of the usual tests require radiation to the fetus. This paper presents 3 cases in which ultrasonic scanning contributed to the diagnosis of nonobstetric disease during pregnancy. Postitive findings included enlarged edematous pancreas, gallstones, and splenomegaly. In 1 case, the finding of a normal gallbladder was helpful. When usual diagnostic procedures such as oral cholecystogram, retrograde endoscopic pancreatography, and nuclear medicine scans are perhaps contraindicated during pregnancy, the ultrasound scan is the diagnostic test of choice.
Obstet Gynecol 1976 Sep
PMID:Ultrasound diagnosis of nonobstetric disease during pregnancy. 94 83

For over 15 years, upper respiratory tract obstruction due to adenotonsillar hypertrophy has been known to cause hypoxia, hypercapnia, increased pulmonary vascular resistance and thereby cor pulmonale and congestive heart failure. This is now an uncommon but not rare entity and three recent cases prompted this report. The typical patient is dyspneic with retractions, cyanosis, occasional periods of apnea and somnolence. Edema and hepatomegaly and at times splenomegaly are common. X-rays show cardiomegaly, which on electrocardiogram is found to involved mainly the right ventricle. The strict definition of cor pulmonale is right ventricular hypertrophy secondary to lung disease or abnormal pulmonary function, a definition that may logically be stretched to include abnormal respiratory function secondary to upper airway pathology. The mechanisms by which this occurs are generally agreed upon. Hypoxia has been demonstrated to cause pulmonary vasoconstriction. Acidosis and hypercapnia are thought by some to have the same effect. Pressure across the pulmonary vascular bed is also increased, as predicted by Poiseuille's law, by the high rate of blood flow required to maintain tissue oxygenation with poorly oxygenated blood. Conditions producing hypoxia of hypercapnia or both lead to hypertrophy and eventually to dilatation of the right ventricle. Three cases of children who underwent cardiac catheterization while suffering from cor pulmonale due to adenotonsillar hypertrophy are reported. Right ventricular pressure averaged 44/5, PAO2 72, pH 7.32, and PACO2 52. All were clinically improved following adenotonsillectomy. Cardiac catheterization was repeated in one case, with right ventricular pressure dropping from 44/5 to 21/2, pulmonary vascular resistance from eight units to three, and PACO2 from 62 to 44.
Laryngoscope 1976 Sep
PMID:Cardiac and pulmonary failure secondary to adenotonsillar hypertrophy. 95 48


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