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)

We retrospectively studied the incidence of familial hemophagocytic lymphohistiocytosis (FHL) in children during the 16-year period 1971-86. First, all departments of pediatrics, pathology, and infectious diseases were enquired for children with FHL or disorders resembling FHL. Secondly, the causes of death of all children who died during the study period in Sweden (n = 19,542) were also investigated. Files and histological specimens were further studied in selected children. By using a set of inclusion/exclusion criteria, we found 32 children with FHL. The incidence was 1.2/1,000,000 children per year. One child per 50,000 live borns developed FHL during this period. The sex ratio was close to 1:1. Prominent early clinical signs were fever (91%), splenomegaly (84%), hepatomegaly (90%), rash (43%), and lymph node enlargement (42%). Neurological symptoms, which developed in 47%, could totally dominate the clinical picture and develop prior to other symptoms and signs. Common laboratory findings were pancytopenia, hypertriglyceridemia, hypofibrinogenemia, elevated serum transaminases, hyperbilirubinemia, hyponatremia, hypoalbuminemia, and a moderate spinal fluid pleocytosis. Chest X-ray often revealed mostly discrete pulmonary infiltrates. FHL is an underdiagnosed disease and in only 11/32 children was diagnosis made during their lifetime. It is important to be aware of the disorder as potential therapy now exists.
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PMID:Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis. 205 92

Haemorrhagic septicaemia (HS) is an infectious disease of cattle and buffalo caused by particular serotypes of Pasteurella multocida and is one of the most economically important livestock diseases in South-East Asia. While HS has been recognized for many years, very little is understood about the disease, primarily because of the expense of cattle and a lack of suitable animal models. The suitability of using mice to study HS was assessed using parameters such as the critical pathogenic dose, kinetics of infection, pathology of disease and resistance to reinfection. Pasteurella multocida M1404, the type strain for Carter group B, the serotype responsible for Asian HS, was injected intraperitoneally into BALB/c mice. As few as 20 colony forming units produced an overwhelming septicaemia in mice in less than 30 h. The kinetics of infection demonstrated a very rapid in vivo multiplication rate. There was no evidence of inhibition of bacterial cell growth by natural host defence mechanisms, even with the very small inocula used. The gross pathology of the disease in mice was characterized by splenomegaly, lymphadenopathy and petechial haemorrhages similar to that observed in cattle and buffalo with HS. Mice were found to develop a short-lived resistance to reinfection following a primary infection which had been successfully treated with antibiotics. The mouse would seem to provide an ideal tool by which to study HS, but warrant further studies in order to be able to critically assess it as a model for this economically important disease.
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PMID:Pasteurella multocida infections in mice with reference to haemorrhagic septicaemia in cattle and buffalo. 210 81

We present a report of a pregnancy complicated by chronic schistosomiasis. A.H. is a 22 year old G1 Filipina, referred at 34 weeks gestational age for evaluation of hypersplenism and progressive thrombocytopenia. Physical exam was remarkable for hepatosplenomegaly. Laboratory evaluation of hepatic, renal, hematologic, autoimmune, or infectious disease etiology or abnormality was negative. Ultrasound revealed a normal fetus, an enlarged spleen, dilated splenic and hepatic vasculature and an enlarged liver with periportal fibrosis. Abdominal computed tomography revealed subcapsular calcifications, dilated ducts, and periportal fibrosis. Esophagogastrojejeunoscopy showed no varices. The presumptive diagnosis of hypersplenism secondary to chronic hepatosplenic schistosomiasis was made. Based on pathophysiology and literature review conservative management was elected. Liver functions, clotting functions, and platelet counts were monitored closely. Intense antepartum fetal monitoring was performed. Platelet counts remained between 30 and 40K. The patient went into labor at 38 3/7 weeks and delivered a 3148 g female Apgars 8/9 by spontaneous vaginal delivery. Evaluation of the placenta was negative for ova. By 2 weeks postpartum platelet counts had increased to the 60-70 k range, and a rectal biopsy, positive for S. japonicum ova was performed. Treatment with three doses of Praziquantel 20 mg/kg q8h was completed prior to her return to the Philippines.
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PMID:Hepatosplenic schistosomiasis in pregnancy: report of a case and review of the literature. 212 Nov 52

Infection with the Friend virus complex (FV) in (B10.A x A/WySn)F1 mice containing the Rfv-3r/s genotype results in several disease manifestations analogous to those seen in patients with acquired immune deficiency syndrome, predominantly high levels of specific antibody and low levels of infectious virus with eventual retroviral disease-induced death of the host. Other immunologic manifestations of FV infection in this murine host included inhibition of percent total T, T-helper, and T-suppressor/cytotoxic cells of total splenic lymphocytes and phytohemagglutinin-induced response of spleen cells. Interleukin-1 production was not affected but the numbers of splenic B cells were increased by the infection. 3'-Azido-3'-deoxythymidine (zidovudine, AZT) administered (a) intraperitoneally three times daily for 24 days beginning 4 h after virus inoculation in doses of 60 to 480 mg/kg/day, (b) in drinking water for 22 days beginning 4 h after virus inoculation in doses of 22 to 216 mg/kg/day, or (c) in drinking water for 29 days beginning 6 days after virus inoculation in doses of 22 to 216 mg/kg/day markedly inhibited FV-induced disease. In the mice receiving early-initiated AZT therapy, FV-induced splenomegaly and hematocrit values were inhibited and infectious centers in the spleen and FV titers in the plasma were reduced to below detectable levels at the higher AZT dosage levels. The percent of total T cells in splenic lymphocytes was increased in the infected, AZT-treated mice. In the intraperitoneal experiment, FV disease-induced death was prevented by treatment with all doses of AZT. Neutralizing antibody to FV was significantly reduced in all AZT-treated groups. Toxicologic manifestations of these AZT treatments included splenic enlargement and reduced hematocrit, although all treated, uninfected mice survived the treatments, gained weight, and displayed no significant effects on enumeration of T and B cells.
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PMID:Effects of zidovudine on Friend virus complex infection in Rfv-3r/s genotype-containing mice used as a model for HIV infection. 215 36

There is an increased frequency of disseminated tuberculosis in patients with acquired immunodeficiency syndrome (AIDS). The authors reviewed 6 thoracic and 10 abdominal computed tomography scans from 11 patients who had AIDS and disseminated tuberculosis. All scans demonstrated multiple, large, mediastinal or retroperitoneal lymph nodes, or both; low-density centers within enlarged nodes were identified in seven patients (63%). The scans also showed a diffuse miliary pattern (three patients), pericardial effusion (three patients), mild hepatomegaly (six patients), moderate splenomegaly (seven patients), hypodense splenic lesions (one patient), peritoneal fluid (four patients), bowel involvement (two patients) and dilatation of the biliary tract (two patients). Infection with Mycobacterium tuberculosis was demonstrated in all cases. The findings of this study show that lymph-node enlargement and nodes with low-density centers in patients who have AIDS are suggestive of disseminated tuberculous infection.
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PMID:Disseminated lymphatic tuberculosis in acquired immunodeficiency syndrome: computed tomography findings. 225 9

We prospectively studied 339 patients diagnosed of brucellosis over a six year period in order to evaluate the clinical and serological characteristics of brucellosis in the elderly. 319 patients (94.1%) were under 65 years of age (group A), and 20 patients (5.9%) were older than 65 (group B). No patient in group B developed splenomegaly as opposed to 69 (21.6%) in group A (p less than 0.05). The percentage of positive blood cultures (Brucella melitensis isolated in all cases) was 72.1% in group A and 60% in group B. The mean titer of IgM antibodies measured by indirect immunofluorescence test was significantly lower in the elderly patients, with no other differences in serologic response between the two groups. 103 patients (32.2%) in group A and seven patients (35%) in group B developed some complications; spondylitis was more common and severe among group B patients. There was no therapeutic failure or relapse among patients over 65. We conclude that clinical, bacteriological and serological characteristics and the outcome of brucellosis in the elderly are similar to those seen in younger patients.
Infection
PMID:Serology, clinical manifestations and treatment of brucellosis in different age groups. 236 66

The clinical, haematological and biochemical features of 50 Sudanese patients with sickle cell disease (SCD) were determined. Of 23 patients with complete family data, 21 had sickle cell anaemia (homozygous HbSS), 2 had sickle-cell/beta+thalassaemia but none had sickle cell/beta Othalassaemia. The remaining 27 patients had HbSS phenotype. 84% of patients were from the Baggara tribe in western Sudan, where HbS is a natural extension of the west African HbS belt. 21 patients were children under 2 years old; 19 were 3-10 years old; and the remaining 10 were over 10 years old. Young patients presented mainly with painful vaso-occlusive crisis, severe anaemia, hand and foot syndrome, fever, underweight, malnutrition and various infectious diseases. All patients had mild to moderate cardiac enlargment; 42% had a moderately enlarged spleen but only 10% had an enlarged liver; 20% had infarctive lesions of long bones and another 8% had Salmonella osteomyelitis. Leg ulcers, priapism, enuresis and cholelithiasis were not observed. Patients had a mean haemoglobin concentration of 7.3 g/dl; reticulocyte count of 15.1%; serum bilirubin of 2.1 mg/dl; HbA2 level of 2.8% and HbF of 7%. Thus, the observed pattern of SCD in Sudan is comparable to the severe type described for Africans and not comparable to the benign form found in Shiite Moslem Arabs of Saudi Arabia. 6 adults with mild SCD had HbF levels below 5%. Amelioration of the disease, therefore, does not seem to be related to HbF levels; nor was it possible to relate it to high levels of erythrocyte 2,3-diphosphoglycerate.
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PMID:Sickle cell disease in Sudan. 245 19

Marble spleen disease (MSD) is a contagious disease of captive-reared ring-necked pheasants (Phasianus colchicus) characterized by variable mortality and high morbidity. The etiologic agent is a type II avian adenovirus closely related to hemorrhagic enteritis virus of turkeys and splenomegaly virus of chickens. Gross lesions of MSD consist of enlargement and mottling of the spleen and pulmonary edema. Microscopic alterations are most pronounced in the spleen and are characterized by reticuloendothelial (RE) cell hyperplasia and lymphoid necrosis. Intranuclear viral inclusions occur within hyperplastic RE cells. Serologic detection of MSD is routinely accomplished using an agar gel precipitin test. Oral vaccination with hemorrhagic enteritis virus or avirulent strains of MSD virus is effective in preventing MSD. There is no specific treatment available for MSD but supportive care, strict sanitation and good management practices are helpful in reducing mortalities and limiting the spread of an epizootic.
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PMID:A review of marble spleen disease of ring-necked pheasants. 255 99

We have analysed 139 consecutive cases (71 males and 68 females) of nutritional megaloblastic anaemia over a period of four and a half years. The majority of these patients belonged to the low socio-economic class and many of them were strict vegetarians. Sixty one percent were in the second and third decades of life. At the time of presentation, 46% had mild hepatomegaly, 42% fever, 34% mild splenomegaly and 20% bleeding manifestations. Of 102 cases in whom the biochemical parameters were available, vitamin B12 deficiency was detected in 76%, folate deficiency in 6.8%, combined B12 and folate deficiency in 8.8%; the remaining 7.8% had normal vitamin levels at presentation. All 139 patients had severe anaemia, 80.5% had thrombocytopenia and 43.8% had neutropenia as well as thrombocytopenia. It appears that during progression (in terms of duration) of megaloblastosis, anaemia is followed by thrombocytopenia and then neutropenia. Infection and bleeding in these patients may be aggravated by impaired functions of neutrophils and platelets, respectively.
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PMID:Pancytopenia in nutritional megaloblastic anaemia. A study from north-west India. 263 48

Since 1985 organ donors are routinely tested for the presence of HIV-antibodies, but prior to that time several patients acquired HIV-infection from grafts. In May 1984 a 65-year-old woman on hemodialysis received a cadaver kidney graft from a young iv drug addict. The transplant functioned perfectly with cyclosporin A immunosuppression. Retrospectively, 22 days after surgery HIV antigen was detected. At this time only a faint band of anti-p24 antibodies was found in the Western blot. Two years after surgery splenomegaly was found in the apparently healthy patient. During the third year thrombocytes fell and she developed lymphadenopathy and constitutional symptoms. Up to this time the immunological parameters were in the range of 10 healthy renal transplant patients with cyclosporin A treatment. In the 4th year T-lymphocytes dropped to values below 200 and the patient developed Pneumocystis carinii pneumonia. A few months later a pulmonary node, which later proved to be a B-cell lymphoma, appeared. Slightly less than 5 years after transplantation the patient died from clinically diagnosed pulmonary embolism. The progression of the HIV-Infection in this patient and in one of 18 patients in published reports show that the incubation period is several years shorter in renal transplant patients than in those who acquire HIV from blood products.
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PMID:[HIV infection caused by kidney transplant: case report and review of 18 published cases]. 267 39


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