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)

Splenomegaly associated with myelodysplastic disorders in children may be massive and can result in pancytopenia, abdominal discomfort, and respiratory distress. When these symptoms cannot be relieved by nonsurgical means, splenectomy may be indicated. Under such conditions, surgical splenectomy carries increased risks, as the thrombocytopenia is difficult to correct secondary to splenic sequestration. Additionally, the surgical anatomy is often distorted secondary to the massive spleen and dissection can be difficult. These factors can lead to uncontrollable hemorrhage. In an attempt to decrease intraoperative blood loss, the authors successfully performed preoperative splenic artery embolization in 11 of 12 children (age range, 1-11 years) with pancytopenia due to hypersplenism. Hypersplenism requiring surgical splenectomy was due to leukemia (n = 9), myelodysplastic syndrome (n = 1), immune thrombocytopenia (n = 1), and osteopetrosis (n = 1). Embolization was performed under general anesthesia, prior to surgery, with gelatin sponge particles alone, Gianturco coils alone, or a combination of polyvinyl alcohol sponge particles and Gianturco coils. Embolization allowed for safe surgical splenectomy.
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PMID:Preoperative embolization of the spleen in children with hypersplenism. 144 26

A red-tailed hawk (Buteo jamaicensis) with signs of respiratory distress and diarrhea was captured in the Manchac Wildlife Management Area, Louisiana (USA) and died the following day. At necropsy, the carcass was emaciated and there were splenomegaly, and fibrinous pericarditis, airsacculitis, and perihepatitis. Microscopically, there were fibrinous pericarditis and airsacculitis, myocardial necrosis, necrotizing hepatitis, splenic necrosis with reticuloendothelial cell hyperplasia, interstitial pneumonia and focal pancreatic necrosis. Intracytoplasmic chlamydial inclusion bodies were noticed in macrophages in the fibrinous exudate covering air sac and pericardium, and in spleen, liver, heart, lung, and pancreas. Schizonts compatible with a Sarcocystis sp.-like protozoon were present in the walls of air capillaries in the lung. A Chlamydia sp.-like organism was isolated in embryonating chicken eggs and cell culture and identified as C. psittaci with immunofluorescent staining.
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PMID:Chlamydiosis in a red-tailed hawk (Buteo jamaicensis). 160 82

Listeria monocytogenes can cause sepsis and meningitis during the neonatal period. Six cases of early onset neonatal sepsis caused by Listeria monocytogenes are reported here. These cases were diagnosed in a private hospital at Santiago, Chile from December 1984 throughout November 1986. The incidence rate was 1.4 x 1,000 liveborns. Clinical findings included prematurity (6), meconium stained amniotic fluid (6), hepatomegaly (6), splenomegaly (6), maculopapular exanthem (4), anal prolapse (3) and meningitis (1). Additionally 5 patients developed respiratory distress and 4 required ventilatory support. Overall mortality was 50% (3/6). All deaths were related to respiratory failure and occurred during the first week of disease. All patients received ampicillin and amikacin early in the course of their infection. Listeriosis of the newborn infant might be preventable by prompt recognition and treatment of maternal infections. Since Listeria infection in pregnancy is usually mild and symptoms and signs are nonspecific, prevention may be difficult. Pregnant women with fever of no clear origin or with an influenza like syndrome should be screened for listeriosis with cultures from blood, vagina and cervix samples.
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PMID:[Early onset neonatal septicemia caused by Listeria monocytogenes]. 215 19

The authors describe a young girl presenting with fever and respiratory distress and a chest x-ray showing a left lower lobe infiltrate and an effusion. She also had splenomegaly. Salmonella enteritidis serotype Heidelberg was isolated by thoracentesis. Further evaluation disclosed an occult but large left subphrenic abscess, explaining the misleading presentation and radiograph. A review of salmonella infections associated with subphrenic abscess is discussed.
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PMID:Left pleural effusion: masking subphrenic abscess--caused by Salmonella enteritidis serotype Heidelberg. 265 53

Excessive unexplained mortality was observed in flocks of double-crested cormorants located at Snake Island in Green Bay, Michigan, in June 1992. Clinical signs included weakness, lethargy, diarrhea, respiratory distress, paralysis of the wings and legs, torticollis, and incoordination. The most significant and consistent gross lesions included edema of the eyelids and periocular tissues, pulmonary edema and congestion, marked splenomegaly, hepatic necrosis, and scattered hemorrhages in visceral organs. Histologically, the principal alterations were severe lymphocytic meningoencephalitis and myelitis, as well as splenic lymphoid necrosis with hemorrhage. A type 1 paramyxovirus was isolated from the affected birds and characterized as a velogenic neurotropic strain of Newcastle disease virus. Since the infection occurred in free-living migratory birds, there exists the potential for spread of the virus over a large area, thus posing a hazard to domestic poultry.
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PMID:Neurotropic velogenic Newcastle disease in cormorants in Michigan: pathology and virus characterization. 770 23

We report a rare case of congenital mumps infection in a newborn girl. Her mother developed bilateral parotitis beginning the day of the delivery. The child was subsequently severely ill and suffered from fever, splenomegaly and thrombocytopenia, however, without parotitis nor pancreatic involvement. Both mother and child recovered well with symptomatic treatment. A review of the literature shows that clinical mumps is rare and usually benign in neonates. However, severe respiratory distress may occur. The recent appearance of mumps outbreaks in adolescents and young adults calls for a reinforcement of mumps vaccination and should prompt an immunological assessment of pregnant women after exposure.
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PMID:Thrombocytopenia in a case of neonatal mumps infection: evidence for further clinical presentations. 822 5

Underlying diseases, complications, clinical findings, and laboratory findings were evaluated in 158 cases of septicaemia admitted to Jikei University Hospital from 1975 to 1994, in order to conjectured factors that prescribe for the prognosis. 50% of the patients had underlying diseases. Malignancy including leukaemia (31 cases, 39.2%) was the most common underlying disease, followed by low birth weight infant (17 cases, 21.5%), aplastic anemia (9 case, 11.4%), and congenital heart disease (7 cases, 8.9%). The death rate for patients with underlying disease (27.8%) was significantly greater than the mortality for normal patients with septicaemia (8.9%) (p < 0.05). Meningitis (24.7%) was the most common complication, followed by DIC (19.6%), shock (15.2%), and pneumonia (10.8%). The mortality rate of septicaemia complicated by shock was 66.7% (p < 0.01), and that complicated by DIC was 45.2% (p < 0.01). The mortality rate for patients with the clinical findings of respiratory distress, cough, abdominal distention, cyanosis, splenomegaly, or peripheral coldness was more than 40% and significantly greater (p < 0.01). Mortality rate in patients with granulocyte counts of < 4.000/mm3, platelet counts of < 5 x 10(4)/ mm3, total protein of < 5.0 g/dl, or ESR of < 20 mm/hr were significantly greater (p < 0.01) than those in patients with normal laboratory findings. Coincidence rate of blood and stool cultures was 57.9% for E. coli, and 28.6% for Klebsiella sp., and that of blood and throat cultures was more than 30% for Pseudomonas sp., Haemophilus influenzae, and Staphylococcus aureus. In the study of antimicrobial susceptibility for microorganisms isolated, the number of drug resistant S. aureus had increased in the last 10 years.
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PMID:[Study on septicaemia in infants and children in the past 20 years. Part 2. An analysis of factors that prescribe for the prognosis]. 889 May 45

A 6-mo-old hand-raised male western lowland gorilla (Gorilla gorilla gorilla) was diagnosed with acute lymphocytic leukemia based on complete blood count and bone marrow cytology. Clinical signs of the disease were pyrexia, abdominal distention, splenomegaly, and lethargy. Acute lymphocytic leukemia has rarely been reported in this species, and therapy was based on human oncologic protocols. Remission induction chemotherapy resulted in complete clearing of leukemia cells from the bone marrow. Consolidation and maintenance chemotherapy followed. Therapy was facilitated by the use of an infusion port for i.v. treatments and an indwelling lumbar catheter for intrathecal therapy. Side effects associated with chemotherapy were inappetence, moderate alopecia, pancytopenia resulting in sepsis, and bleeding tendency. In spite of initial success, the leukemia reappeared 120 days into treatment. The gorilla was euthanized 7 days later when respiratory distress developed. Intensive care by the animal staff was a key factor in the treatment of this gorilla.
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PMID:Acute lymphocytic leukemia in a six-month-old western lowland gorilla (Gorilla gorilla gorilla). 1048 44

Congenital dyserythropoietic anemias (CDAs) are a group of hereditary refractory anemias characterized by ineffective erythropoiesis, typical morphological abnormalities of erythroblasts, a low or no reticulocyte response, hyperbilirubinemia, and splenomegaly. A massive hydropic newborn born with a very severe anemia (Hb 4.8 g/dL), diffuse edema, hepatosplenomegaly, ascites, pulmonary edema and respiratory distress, and shortness and hallux varus deformity of the great toe of the right foot was diagnosed to have congenital dyserythropoietic anemia on the basis of the hematological (macrocytosis, anisopoikilocytosis, fragmented red cells and erythroblastosis in the peripheral blood, and erythroid hyperplasia with erythroblastosis and erythroblasts with double nuclei and thin chromatin bridges connecting these nuclei in the bone marrow) and serological (negative acidified serum lysis test and no agglutination with anti-i antibodies) findings. In this article the seventh case of neonatal congenital dyserythropoietic anemia presenting with a very severe (lethal) form of hydrops fetalis and a new (hallux varus) deformity of the great toe of the right foot is presented. Congenital dyserythropoietic anemia should be considered in the differential diagnosis of hydrops fetalis presenting with a very severe anemia and a skeletal abnormality of the great toe.
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PMID:Lethal hydrops fetalis due to congenital dyserythropoietic anemia in a newborn: association of a new skeletal abnormality. 1176 4

Adult onset Still's disease (AOSD), the adult variant of the systemic form of the juvenile rheumatoid arthritis, is an uncommon disorder of unknown origin. Although the pathogenesis has not yet been clarified, an immunologically mediated inflammation occurs in active AOSD. High spiking fever, evanescent maculo-papular skin rash, arthralgias/arthritis, neutrophilic leukocytosis, negative rheumatoid factor and antinuclear antibodies, as well as a marked hyperferritinemia are the major features of AOSD. Sore throat, lymphadenopathies, hepato-splenomegaly, abdominal pain, polyserositis, respiratory distress syndrome, multiple organ dysfunction and disseminated intravascular coagulation may also occur. The clinical course of AOSD is extremely variable and unpredictable and can be divided into three main patterns: a self-limited or monocyclic pattern, a polycyclic or intermittent course, with one or more flares of the disease and complete remission among the episodes, and a chronic course, characterized by persistently active disease, usually due to a chronic, destructive arthritis. Since there are not pathognomonic laboratory parameters or histological findings, the diagnosis of AOSD requires the exclusion of infectious, malignant and autoimmune disorders. Some sets of criteria for classification have been proposed, but so far not validated. The prognosis of AOSD is usually considered relatively benign, although a destructive arthritis may cause severe disability and the multisystemic life-threatening complications of the disease may determine a fatal outcome. Treatment usually consists in nonsteroidal anti-inflammatory drugs and corticosteroids, but a more aggressive approach with disease modifying antirheumatic or immunosuppressive drugs may be required.
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PMID:[Adult onset Still's disease]. 1185 Jun 12


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