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)

A 26-year-old male patient with pachydermoperiostosis is reported. He had severe anemia with myelofibrosis. Treatment with iron, prednisolone, oxymethorone and 1 alpha (OH)D3 were not satisfactory. But steroid pulse therapy with parenteral iron improved his anemia and pancytopenia, but was not sufficient to relieve the bone marrow fibrosis or splenomegaly. The mechanism of anemia which was considered to be multifactorial including gastro-intestinal bleeding associated with peptic ulcer or erosion and bone marrow failure due to myelofibrosis, is discussed.
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PMID:Pachydermoperiostosis with myelofibrosis and anemia: report of a case of anemia of multifactorial causes and its improvement with steroid pulse and iron therapy. 186 80

From 1960 to 1981, 31 children, 18 boys and 13 girls, have been treated at Ste-Justine Hospital for extra-hepatic portal hypertension. Age at the onset of gastro-intestinal bleeding 3 1/2 months and 13 years. Splenomegaly with hypersplenism, hematemesis and melena have been the most frequent clinical manifestations. Percutaneous splenoportography be coelio-mesenteric arteriography confirmed the diagnosis of cavernomatous transformation of the portal vein. Among the 31 children, 28 bled from their varices, the 3 others did not to date. Among the 28 patients with active bleeding complication, 7 have been treated conservatively, 11 had sclerotherapy (sclerosing injections of varices). On the surgical point of view, 7 had ligation of varices with intra-thoracic transposition of the spleen in 5 of them. Portosystemic shunts were performed in 10 patients, 3 central spleno-renal and 7 cavo-mesenteric shunt. Each of these 28 patients had an average of 9,5 episodes of gastro-intestinal bleeding, 8.7 hospitalizations, and received 7.5 liter of blood. Medical treatment, sclerotherapy, ligature of varices and intra-thoracic transposition of the spleen are palliative measures. Nevertheless the procedures are time and life savers, allowing improvement and development of natural porto-systemic shunts. Follow up of unshunted patients on a period of 8 to 17 years revealed a decreasing frequency and intensity of the hemorrhagic manifestations. Operative risks and morbidity of recurrent bleeding should be the guidelines for surgery. Natural course of illness and possible neuro-psychiatric consequences of a porto-caval shunt should also be considered.
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PMID:[Portal hypertension of extra-hepatic origin in children (author's transl)]. 697 8

Campylobacter fetus subspecies jejuni was isolated fom the feces of 63 (3.2%) of the 1,953 patients who had stools cultured at the Mayo Clinic in 1979. In contrast, Salmonella and Shigella combined were isolated from 31 (1.6%) patients. Two patients had double infections with Salmonella species and C. fetus subsp jejuni. Three patients had no diarrhea at the time of stool culture. One patient, who had chronic lymphocytic leukemia, had both blood and stool cultures positive for C. fetus subsp jejuni. There was a seasonal incidence that peaked in July when 7.8% of all patients who had stools cultured had C. fetus subsp jejuni isolated. Thirteen cases occurred in children 5 years of age and younger and 29 cases occurred between the ages of 15 and 30 years. Clinical features often included a prodrome of malaise, which preceded the onset of abdominal cramps, diarrhea, anorexia, fever, nausea, and vomiting. Grossly bloody diarrhea occurred in 33 patients, and massive intestinal bleeding occurred in 1 patient as a late complication after diarrhea had resolved. Transient splenomegaly was attributed to C. fetus subsp jejuni on one occasion. Proctoscopic findings may be similar to those seen in inflammatory bowel disease or pseudomembranous colitis. Three patients were referred to this institution with newly diagnosed chronic ulcerative colitis, and one patient was referred with newly diagnosed Crohn's disease. C. fetus subsp jejuni was isolated from their stools, and the diagnosis of inflammatory bowel disease was subsequently dropped. A selected review of cases illustrates the variety of gastrointestinal manifestations seen with this organism.
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PMID:Diarrhea due to Campylobacter fetus subspecies jejuni. A clinical review of 63 cases. 725 3

Interleukin-3 treatment of juvenile rhesus monkeys elicits a dose- and time-dependent syndrome that includes urticaria, palpable lymph nodes, splenomegaly, thrombocytopenia, anemia, vomiting, diarrhea, intestinal bleeding, edema, and arthritis, apart from a strong stimulation of hemopoiesis. Arthritis was found to occur significantly more often in animals expressing the major histocompatibility complex alleles B9 and Dr5. Histological analysis revealed an abundance of mast cells in urticaria and, to a lesser extent, in lungs and synovia of arthritic joints. Active osteoclasts were abundant in ribs and arthritic joints. Extramedullary hemopoiesis was encountered in liver, spleen, and kidneys. The spleen showed deposits of hemosiderin, and in the liver, Kupffer cells were loaded with iron, indicating enhanced turnover of hemoglobin. Lymph nodes and bone marrow showed macrophages involved in hemophagocytosis, which probably contributed to the development of anemia and thrombopenia. Biochemical parameters in sera were indicative of parenchymal liver damage, with cholestasis and increased erythrocyte destruction. The side effects were strongly reduced in monkeys subjected to total body irradiation just before interleukin-3 treatment. Histamine antagonists were not significantly effective in preventing side effects, which is explained by the perpetual stimulation of basophilic granulocytes by exogenous interleukin-3. The nature of the side effects indicates that interleukin-3 may be involved in the pathogenesis of acute type hypersensitivity reactions and arthritis.
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PMID:Acute side effects of homologous interleukin-3 in rhesus monkeys. 825 52

A diagnosis of blood culture-positive typhoid (TF; n = 39) or paratyphoid (PTF; n = 17) fever was made in 56 patients admitted to two Dutch university hospitals in the period 1984-1990. The group of TF patients constituted 9% of the reported national total during those years. A retrospective analysis of available clinical, laboratory and epidemiological data was carried out. Without exception, infections were contracted during travel abroad, especially to India and Indonesia. The clinical features and the response to antimicrobial treatment of TF and PTF proved essentially the same. Fever, headache and anorexia were important symptoms; rose spots and splenomegaly were found in 18/38 and 10/39 with S. typhi respectively. Most patients had a normal white blood cell count; less than half of the patients had thrombocytopenia. A positive Widal-test was found in 15/24 patients with S. typhi. 18/39 patients with S. typhi had been vaccinated; 10 did not know. Amoxycillin was the preferred antimicrobial agent in 69% of cases. Median defervescence time was 5 days in TF and 4 days in PTF. Relapse occurred in 3 TF cases. The recurrence rate after amoxycillin treatment was 7.6%. Profuse intestinal bleeding (1x), septic shock (1x) and cholangitis plus ARDS (1x) were major complications, seen in TF patients only. All patients recovered fully. None of the isolated strains of S. typhi or S. paratyphi proved multiresistant. Surveillance data from the Dutch National Institute of Public Health and Environmental Protection suggest that multidrug-resistance of S. typhi is increasing, especially in strains imported from countries such as India and Pakistan.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Abdominal typhus and paratyphoid fever in 2 academic hospitals: 1984-1990]. 843 74

Patients with typhoid fever presenting to the Tokyo Metropolitan Komagome Hospital during the period 1975-1998 were retrospectively investigated. All cases were diagnosed by a positive culture for Salmonella typhi in either of their clinical specimens. Of the total number of 130 patients, 57% contracted the disease abroad; this population increased in later years as the total numbers of cases decreased. The period from disease onset to diagnosis averaged 14 days with 20% of the cases requiring over three weeks to establish a diagnosis. As for symptomatology relative bradycardia was seen in less than half of the cases, and rose spots or splenomegaly in less than one third. A positive blood culture was the most frequent test establishing the diagnosis followed by a positive stool culture. Intestinal bleeding was recognized in as many as 35 cases (27%) and even intestinal perforation occurred in two cases (1.5%). Chloramphenicol was most commonly employed during the early study period, however, during the late period it was replaced by fluoroquinolones. The clinical cure rate was 98% with regimens that include fluoroquinolones/quinolone; however it was 87% with the other antimicrobial regimens. Bacteriological relapse occurred in 25% of the non-fluoroquinolone group while only in 2.0% in the fluoroquinolone/quinolone group. Four strains of Salmonella typhi that were multi-resistant to chloramphenicol, ampicillin and cotrimoxazole were isolated in travelers from Asia. Early diagnosis by appropriate bacteriological examination regardless of classical symptomatology should be stressed and the use of fluoroquinolones is warranted in the treatment of typhoid fever.
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PMID:Clinical and bacteriological profiles of patients with typhoid fever treated during 1975-1998 in the Tokyo Metropolitan Komagome Hospital. 1098 30

From 1995-2002, 14 patients with predominantly prehepatic, noncirrhotic portal hypertension were evaluated. At presentation, the eight females and six males had a mean age of 9 years (range 2-18). Seven were admitted with gastrointestinal, mostly esophageal bleeding, three with splenomegaly, three with hepato-pulmonary syndrome, and one with hyperammonemia. Imaging studies showed portal vein obstruction in six patients and non-obstructed but frequently anomalous vascular patterns, including hypoplasia of the portal vein, in the remaining eight patients. At the onset, liver function was marginally abnormal in all patients, but thrombocytopenia of approximately 100 x 10(9)/L was consistently observed, probably reflecting chronic mild consumption coagulopathy and hypersplenism. The most striking and frequent histopathologic finding in 25 liver samples, was the presence of hypoplastic portal triads with collapsed portal vein radicles. In contrast, other triads showed markedly distended and misshapen portal vein radicles and likely lymphatics. These two patterns of collapse and distention presumably reflect areas of impaired versus overloaded intrahepatic portal venous flow. Some of the biopsies showed variable portal/sinusoidal fibrosis. Four patients (two with intestinal bleeding, two with hepatopulmonary syndrome) required liver transplants and are doing well. Eight patients are doing well clinically after surgical or spontaneous vascular shunting. Two patients with intestinal bleeding and hepato-pulmonary syndrome, respectively) who had congenital dyskeratosis, underwent bone marrow transplantation and died of nonhepatic-related complications. It is possible to suggest prehepatic causes of portal hypertension even in needle biopsies when collapsed portal vein radicles are present in portal triads, but more than one biopsy sample with larger bore bioptomes may be required to see the changes. Conversely, identifying these changes may suggest to the clinicians the need to work-up a patient for portal hypertension.
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PMID:Pathology of noncirrhotic portal hypertension: clinicopathologic study in pediatric patients. 1470 35

Small intestinal bleeding in Crohn's disease patients with noncirrhotic portal hypertension and partial portal and superior mesenteric vein thrombosis is a life-threatening event. Here, a case is reported in which treatment with azathioprine may have resulted in nodular regenerative hyperplasia, portal hypertension and portal vein thrombosis. The 56-year-old patient with Crohn's disease developed nodular regenerative hyperplasia under treatment with azathioprine. He was admitted with severe bleeding. Gastroscopy showed small esophageal varices without bleeding stigmata. Blood was detected in the terminal ileum. CT scan revealed a partial portal vein thrombosis with extension to the superior mesenteric vein, thickening of the jejunal wall and splenomegaly. Because intestinal bleeding could not be controlled by conservative treatment, the thrombus was aspirated and a transjugular intrahepatic portosystemic shunt (TIPS) was placed. Switching the immunosuppressive medication to infliximab controlled Crohn's disease activity. Bleeding was stopped, hemoglobin normalized, and thrombocytopenia and bowel movements improved. In summary, small intestinal bleeding in a Crohn's patient with nodular regenerative hyperplasia, portal hypertension and portal vein thrombosis can be efficiently treated by TIPS. TIPS placement together with infliximab treatment led to the improvement of the blood panel and remission in this patient.
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PMID:Successful Treatment of Small Intestinal Bleeding in a Crohn's Patient with Noncirrhotic Portal Hypertension by Transjugular Portosystemic Shunt Placement and Infliximab Treatment. 2792 Jun 46