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)

A 60-year-old woman was admitted because of fatigue. Physical examination revealed prominent peripheral lymphadenopathy, marked tonsillar swelling and hepatosplenomegaly. The leukocyte count was 68,900/microliters with 75% lymphoid blasts and 5% basophils. The karyotype of the blood cells was 46, XX, Ph1/47, XX, Ph1, +Ph1. The diagnosis of CML in blast crisis was made. After chemotherapy using adriamycin, cyclophosphamide, vincristine, and prednisolone (CHOP), lymphadenopathy and splenomegaly reduced and lymphoid blasts disappeared from the blood and bone marrow. At that time only single Ph1 (46, XX, Ph1) clone was detected in her bone marrow. Four months later, hematological relapse accompanied by lymphadenopathy occurred and DNA analysis of the blasts showed the rearrangement of bcr gene. The simultaneous chromosomal analyses of the blood, bone marrow and lymph node revealed that almost all cells examined had the karyotype "47, XX, Ph1, + Ph1". In spite of repeated chemotherapy the patient did not improve and died. This case suggests a relationship between lymphadenopathy and double Ph1 chromosomes in CML.
Rinsho Ketsueki 1990 Sep
PMID:[Prominent lymphadenopathy and double Ph1 chromosomes as initial and recurrent manifestations of chronic myelogenous leukemia in blast crisis: report of a case and review of the literature]. 224 24

The authors describe the examination, diagnostic possibilities and difficulties associated with the diagnosis of the spleen by means of computed tomography. The finding obtained by computed tomography is evaluated as splenomegaly when two of the three main dimension are greater than the maximal values reported, or when the lienal index is higher than 440. From the practical aspect the decision on splenomegaly is facilitated by the position of the anterior margin of the spleen in relation to the medioaxillary line. Splenomegaly is the most frequent pathological finding on the spleen. The authors give an account of causes of splenomegaly and their own experience with the diagnosis of anomalies, cystic processes, tumours, injuries and obstructions of the lienal vessels.
Cesk Radiol 1990 Sep
PMID:[Evaluation of the size of the spleen using computer tomography]. 224 9

Splenomegaly is often a manifestation of sarcoidosis, but giant splenomegaly is rare. Only 25 cases of sarcoidosis presenting as massive splenomegaly have been reported. Splenectomy was performed before the diagnosis of sarcoidosis was made in more than half of these patients. Serious complications of giant splenomegaly including rupture, severe thrombocytopenia and hemolytic anemia were not consistently present preoperatively. A case of disseminated sarcoidosis presenting with giant splenomegaly and normal chest roentgenograms is described. Prednisone therapy improved the patient's pulmonary function testing while constitutional symptoms, splenomegaly and elevated angiotensin-converting enzyme levels completely resolved. Sarcoidosis should be considered in the differential diagnosis of massive splenomegaly. A trial of steroids is warranted as initial treatment, and in selected cases may prevent unnecessary splenectomy, thereby precluding a compromise in host defenses against encapsulated bacteria.
Sarcoidosis 1990 Sep
PMID:Sarcoidosis and giant splenomegaly. 225 87

The clinical courses of 214 patients with infective endocarditis treated between 1958 and 1987 at the First Medical Hospital of the University of Kiel (FRG) were analyzed retrospectively. A decrease in the incidence of endocarditis occurred during the 30-year observation period. The mean age of patients was 48 years, and men were more frequently affected than women. In the course of the investigation, a rise in isolated aortic valve disease was noted, whereas the number of patients with isolated involvement of the mitral valve and combined mitral-aortic valvular defects declined. Streptococci (57%) were the most frequent pathogens isolated; as opposed to their increase, the percentages of Staphylococcus aureus and enterococci decreased. Otolaryngological, dentogenic and urogenital diseases were most frequently held to be responsible for the development of infective endocarditis. Prior cardiosurgical interventions became increasingly significant as a cause of the disease. In this connection, a rising percentage of endocarditis cases was linked with prosthetically replaced heart valves. Complications and concomitant symptoms of endocarditis included the development of heart failure, cerebral embolism and encephalitis, splenomegaly, and renal inflammation. Finally, the marked decrease in mortality contrasted with a simultaneous rise in the number of endocarditis cases achieving full recovery.
Klin Wochenschr 1990 Sep 14
PMID:Infective endocarditis at a hospital of the University of Kiel, 1958-1987. 225 87

We previously reported that streptococcal preparation (OK-432), which is a TNF inducer, inhibits insulitis and development of autoimmune diabetes in nonobese diabetic (NOD) mice and Bio-Breeding (BB) rats, as animal models of insulin-dependent diabetes mellitus. We have recently shown that recombinant human (h)TNF-alpha also suppresses development of diabetes in NOD mice. In this study we have extended our observation on TNF to BB rats in order to see whether TNF generally inhibits autoimmune diabetes. A total of 5 x 10(4) U of rhTNF-alpha was administered i.p., twice a week to male and female BB rats from 4 to 27 wk of age. The cumulative incidence of diabetes by 27 wk of age in nontreated rats was 36.4% (8/22), whereas that in hTNF-alpha-treated rats was 0% (0/21) (p less than 0.001). The hTNF-alpha-treated rats did not lose body weight and maintained normal blood glucose concentrations. Immunologic and histologic examinations were performed at the end of the experiment. Spleen cell cytotoxicities for NK-sensitive YAC-1 and rat insulinoma (RINm5F) cells in hTNF-alpha-treated rats significantly decreased in comparison with nontreated and nondiabetic BB rats. Intensity of insulitis was also inhibited in hTNF-alpha-treated rats. Interestingly, a huge hepatomegaly and splenomegaly was found in two of the 21 hTNF-alpha-treated rats. The latter consisted of W3/13dull+ and W3/25dull+ cells, which did not exhibit cytotoxicity for either YAC-1 or RINm5F cells. These results indicate that the chronic and systemic administration of TNF has a regulatory role in autoimmune diabetes in BB rats as well as in NOD mice, and that these animals may have a defect in TNF-mediated immunoregulation.
J Immunol 1990 Sep 01
PMID:Inhibition of type 1 diabetes in BB rats with recombinant human tumor necrosis factor-alpha. 238 63

The patient was a 65 year old woman with a massive splenomegaly and no lymphoadenopathy. An electron microscopic study of the peripheral blood cells revealed that these cells were prolymphocytes with a single large nucleolus. Treatment with native and recombinant alpha-interferon has produced an impressive reduction in spleen size and in the count of prolymphocytes.
Am J Hematol 1990 Sep
PMID:Prolymphocytic leukemia treated with natural and recombinant alpha-interferon. 238 69

Because growth failure is a frequent complication of chronic liver disease in childhood, we examined the growth hormone/insulin-like growth factor type I axis and its relationship to growth disturbances, nutritional status, and carbohydrate metabolism in nine children (2.1 to 18.6 years of age) with chronic cholestatic liver disease. Seven had cholestasis associated with splenomegaly and histologic findings of cirrhosis; two patients had Alagille syndrome. Stature was less than or equal to 15th percentile in all except the youngest subject and less than 5th percentile in five subjects. Ten-hour, nocturnal, integrated serum concentrations of growth hormone were considerably higher in patients with cholestasis than in control subjects (mean +/- SD) 9.7 +/- 3.8 vs 4.7 +/- 1.9 ng/ml; p less than 0.02). Serum concentrations of insulin-like growth hormone type I were less than 95th percentile confidence intervals for age- and sex-matched norms in five patients and at the lower limits of normal in the remaining four patients. Insulin sensitivity, determined with the minimal model intravenous glucose tolerance test, was not decreased in five patients despite elevated levels of circulating growth hormone. The estimated mean caloric and protein intake exceeded the recommended dietary allowance and the weight-for-height index was greater than 90% for six of nine patients. Triceps and subscapular skin-fold thicknesses, indicators of body fat stores, were greater than 25th percentile for five of nine and eight of nine patients, respectively, suggesting deficient lipolytic action of GH. We conclude that children with cholestatic liver disease have a resistance to the growth-promoting, diabetogenic, and lipolytic properties of growth hormone.
J Pediatr 1990 Sep
PMID:Resistance to the growth-promoting and metabolic effects of growth hormone in children with chronic liver disease. 239 94

Black children with homozygous sickle-cell disease usually have anatomic asplenia by 6-8 years of age. In the Eastern Province of Saudi Arabia, sickle-cell disease runs a benign course. Here we report six sicklers with hypersplenism as manifested by 1: splenomegaly (6/6); 2: persistent thrombocytopenia (6/6); 3: increasing severity of anaemia with the need for repeated blood transfusion (5/6); 4: leukopenia (1/6); 5: high reticulocyte count (6/6); 6: circulating nucleated red blood cells (5/6). All improved after splenectomy, with resolution of the thrombocytopenia and leukopenia, improvement in the degree of anaemia, decrease in the reticulocytes and disappearance of circulating nucleated red blood cells.
Ann Trop Paediatr 1985 Sep
PMID:Hypersplenism in homozygous sickle-cell disease in Saudi Arabia. 241 52

Seventeen patients with either chronic myelogenous leukemia (CML) or myelofibrosis with myeloid metaplasia (MMM) received 24 courses of splenic irradiation at this institution from 1973 to 1982. Eleven of the 17 patients had received prior chemotherapy. Patients were treated with 60Co gamma rays or 6 MV photons. The fraction size ranged from 15 to 100 rad and the total dose per treatment course from 15 to 650 rad, with the exception of one patient who received 1650 rad. Fourteen of 19 courses (71%) given for splenic pain yielded significant subjective relief while 17 of 26 courses given for splenomegaly obtained at least 50% regression of splenic size. Blood counts were carefully monitored before each treatment to limit hematologic toxicity. From this experience, the authors conclude that splenic irradiation effectively palliates splenic pain and reverses splenomegaly in the majority of patients with CML and MMM. Intermittent fractionation (twice or thrice weekly) is more convenient for the patient, appears to be as effective as daily treatment, and may be associated with less hematologic toxicity. Preliminary results of concurrent treatment with splenic irradiation and oral hydroxyurea show promise and warrant further study.
Cancer 1986 Sep 15
PMID:Splenic irradiation in the treatment of patients with chronic myelogenous leukemia or myelofibrosis with myeloid metaplasia. Results of daily and intermittent fractionation with and without concomitant hydroxyurea. 242 84

Using rabbit erythrocyte-derived neutral glycosphingolipids enriched for a ceramide pentasaccharide as the antigen, we detected elevated anti-galactosyl-alpha(1-3)galactose (anti-G alpha G) antibody levels in 76% of children with active visceral leishmaniasis (kala-azar [KA]) and in 42% of clinically cured patients with KA who had been treated about 5 years previously with meglumine antimonate (30 mg/kg in a series of 15 daily injections). The long-term persistence of elevated G alpha G antibodies was also found in 56% of children living in the same geographic zone who, at the time of the initial clinical examination, had fever and evident splenomegaly with hyperglobulinemia but a negative bone marrow aspirate for leishmanial bodies. Five years after antimonate treatment, these clinically cured children with presumptive KA were studied serologically. Their mean G alpha G antibody values were slightly lower than those in patients with active KA but were still abnormal. Using different biochemical and immunological approaches, we found that elevated G alpha G antibodies present in patients with KA bound specifically to glycoconjugates with an alpha(1-3)-terminal galactose residue. G alpha G antibodies were mainly distributed between immunoglobulin classes G and M in patients with active KA and in antimonate-treated patients with clinically cured KA. The possibility of the existence of remnant living parasites or the persistence of inserted G alpha G epitopes in parasitized macrophages was proposed as a mechanism to explain the long-term persistence of abnormal G alpha G antibodies in patients apparently cured of KA.
J Clin Microbiol 1988 Sep
PMID:Persistence of elevated levels of galactosyl-alpha(1-3)galactose antibodies in sera from patients cured of visceral leishmaniasis. 246 Apr 98


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