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)

Clinical and laboratory features have been reviewed in 66 episodes of disseminated histoplasmosis that occurred during two large urban outbreaks in Indianapolis. Immunosuppression, age greater than 54 years, and presence of other serious underlying illnesses predisposed to the disseminated form of the disease; only 21% of patients lacked one of these risk factors. Central nervous system findings, splenomegaly, hepatomegaly, and lymphopenia suggested disseminated disease but were present in only about one-third of patients. Miliary or diffuse pulmonary infiltrates also suggested dissemination and were noted in about one-third of patients, while mediastinal lymphadenopathy was present in only 17%. Histoplasmal serologic tests, positive in 90% of patients, provided useful diagnostic clues. The diagnosis could be confirmed by culture in 88% of patients, and special stains were positive in about two-thirds. Although 10% of patients recovered without treatment, 11 patients (17%) died because of failure to suspect the diagnosis and initiate therapy promptly. Amphotericin B was effective in all patients receiving at least 500 mg, but relapse occurred if the total dose was less than 30 mg/kg. Ketoconazole appeared effective in non-immunosuppressed patients but not in those with underlying immunosuppression; however, a controlled trial comparing ketoconazole and amphotericin B is required to establish the role of this new fungistatic oral agent.
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PMID:Clinical and laboratory features of disseminated histoplasmosis during two large urban outbreaks. 631 46

The efficiency of stored platelet transfusion was evaluated in terms of clinical status in 136 thrombocytopenic patients. In a paired prospective study in which fresh platelets were used as controls, clinical efficiency was assessed on the basis of the ability to increase platelet count (recovery) and the interval to the next transfusion (D). In 48 clinically stable patients, recovery of fresh and stored platelets was similar (47% and 41% respectively) and the interval to the next transfusion was D4 and D3. In contrast, 27 patients who had bacterial infections showed significantly different recoveries (24%/5%) and the interval to the next transfusion was D3/D1 for fresh and stored platelets respectively. Similarly, in 16 patients who were treated concurrently with Amphotericin B, 18 other patients with graft-versus-host disease, 5 with splenomegaly and 3 with veno-occlusive disease (VOD), fresh platelets performed better than stored platelets, showing recoveries of 27%/18%, 29%/15%, 15%/1%, 22%/3%. Furthermore, the need for retransfusion within 24 hours was significantly increased with stored platelets. In 19 patients with anti-HLA allo-immunization who were transfused with HLA-matched fresh and stored APC, efficiency was similar (38%/36% and D4/D3). This study indicates that the storage has a major detrimental effect on platelet recovery and survival in patients with certain clinical conditions.
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PMID:[Influence of clinical status on the efficacy of stored platelet transfusion]. 825 53

Two imported cases of Penicillium marneffei infection in Belgium are reported. Both patients are Thai women co-infected with HIV. P. marneffei infection should be suspected in immunocompromised patients originating or travelling from South-East Asia with unexplained fever (> 38 degrees C), weight loss, a generalised lymphadenopathy, hepatomegaly, splenomegaly, skin lesions, cough and anaemia. Diagnosis is made by culture and/or histopathological examination. Mild to moderate infections are treated with itraconazole 400 mg/day as first choice. Amphotericin B parenteral therapy may be required for seriously ill patients. Maintenance therapy with itraconazole 200 mg/day is necessary to prevent relapses.
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PMID:Two imported cases of Penicillium marneffei infection in Belgium. 979 45

A 7-year-old previously healthy Czech boy was admitted due to fever, hepatosplenomegaly and pancytopenia. Aspiration of bone marrow revealed no signs of hemoblastosis (nor hemophagocytosis). He was treated with antibiotics and virostatics without effect. Progression of hepatosplenomegaly and pancytopenia induced suspicion of hemophagocytic lymphohistiocytosis (HLH). Five weeks later, bone marrow hemophagocytosis of erythrocytes, nuclear elements and platelets was detected. He was given corticoids and intravenous immunoglobulins and transferred to our haematology department. Laboratory findings of mild pancytopenia, hypofibrinogenaemia, hyperlipidaemia and elevated levels of ferritin, LDH and immunoglobulins were compatible to the diagnosis of HLH. Immunologic evaluation revealed T-lymphocyte activation. Appropriate immunosuppressive treatment with Dexamethasone, etoposide and Cyclosporine A was launched, followed by transient subside of fever and improvement of peripheral blood count, but not regression of hepatosplenomegaly. Four weeks later, relapse of fever and deterioration of blood count led to intensification of immunosuppression. However, no effect was evident. Moreover, hypertrophic cardiomyopathy with ventricular arrhythmia occurred. Treatment with antilymphocytic globulin for resistant course of HLH was planned. Before that, a fifth bone marrow aspiration was performed. Surprisingly, many Leishmania amastigotes were observed within marrow macrophages. Leishmania infection was confirmed by positive serology. Immunosuppressive treatment was withdrawn and changed for causal treatment with liposomal Amphotericin B. Positive clinical effect with subside of fever was evident in ten days, splenomegaly gradually resolved during three weeks, restoration of normal blood count lasted six weeks. No relapses of HLH nor leishmaniasis occurred. In control bone marrow aspirate performed three months later, the parasites were not detected. Ten months after the event, the patient is in complete remission of HLH with normal immunologic parameters. Most probably, he contracted visceral leishmaniasis during a visit of a Neapol area in Italy 3 months before the onset of the disease.
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PMID:[Hemophagocytic lymphohistiocytosis as a manifestation of visceral leishmaniasis]. 1242 69

Visceral leishmaniasis (VL) is an acute or subacute disease that is almost invariably fatal if untreated. It is a rare disease in renal transplant recipients and frequently reported together with other infectious agents. A 39-year-old renal transplant patient was admitted to hospital for elective coronary surgery. In the post-operative period, he developed spiking fever and non-productive cough and his general condition deteriorated. While he was taking medication for non-specific pneumonia, a cavitary lesion occurred in his lung, and he had the diagnosis of pulmonary tuberculosis and antituberculous treatment was started. Despite treatment, his fever continued. As the patient developed pancytopenia and splenomegaly, a bone marrow aspiration was done. Evaluation of bone marrow aspirate indicated Leishmania parasites. He was successfully treated with a more intensive liposomal amphotericin (L-AmB). Complete cure was achieved during follow-up period of 10 months without clinical relapse. In the existence of fever and long-standing pancytopenia, VL should be suspected although the patient had another proved infection and did not live or visit an endemic area. L-AmB usage can be safely preferred for treatment of selected renal transplant recipients with VL as first-line therapy.
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PMID:A renal transplant recipient with pulmonary tuberculosis and visceral leishmaniasis: review of superimposed infections and therapy approaches. 1457 46

Visceral leishmaniasis (VL) is endemic in areas bordering the Mediterranean Sea (Spain, Italy, France, Greece, Morocco, Tunisia) where it is caused by Leishmania infantum and is transmitted by the bite of a hematophagous sandfly belonging to Phlebotomus spp.; the dog constitutes the main reservoir of infection. Two cases of VL in immunocompetent children are described. Both patients lived in endemic areas for leishmaniasis (Sicily) and at admission were febrile, pale and had splenomegaly. In both patients anti-leishmania antibodies were present and a definitive diagnosis was confirmed by demonstration of leishmania parasites by microscopy or polymerase chain reaction (PCR) in the bone marrow aspirates. The use of PCR performed on peripheral blood has been reported to be highly sensitive for the diagnosis and follow-up of children with VL. One patient was treated with N-dimethylglucamine, Glucantim, the other one with liposomal Amphotericin B (AmBisome). Both had symptomatic relapses 3 months later, and recovered following re-treatment with AmBisome administered intravenously at a dosage of 3 mg/Kg for ten consecutive days. The patients were monitored for one year after treatment was completed.
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PMID:[Mediterranean visceral leishmaniasis in immunocompetent children. Report of two cases relapsed after specific therapy]. 1531 2

Kala-azar is a chronic infection of reticuloendothelial system caused by flagellated protozoan, leishmania donovani injected into human host by the bite of the sand fly (phlebotomous) previously infected by biting and sucking the blood of a patient of leishmaniasis. It is characterized by irregular fever of long duration, large spleen and liver, anaemia, leucopenia and progressive emaciation. This article reports a case of a 10 year old girl from Khotang, a nonendemic zone for Kala-azar, who presented with long history of abdominal distension for 11 months, fever for 9 months, cough for a week and weight loss. Clinical examination revealed pallor, enlarged liver and huge splenomegaly. Investigations confirmed the diagnosis of kala-azar by the presence of L.D bodies in bone marrow smear. The patient is being treated with i.v Amphotericin B in Infectious Disease Hospital, Teku.
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PMID:Kala-azar (visceral Leishmaniasis) from Khotang. 1860 4

One hundred and one children with visceral leishmaniasis (VL) who admitted to Akdeniz University Hospital during a 20-year period were analyzed. Median age of the patients was 3 years (range: 5.5 months-13 years). The most common symptoms at presentation were fever, pallor and abdominal distension. Splenomegaly was found in all of the patients while hepatomegaly was present in 98%. Anemia (96%), leukopenia (74%) and thrombocytopenia (56%) were the main laboratory abnormalities. Thirty-three (33%) of the patients were pancytopenic on admission. Bone marrow smear was positive for leishmania in 91% of the patients. Seventy-four patients were treated with antimony +/- pentamidine and 27 with amphotericin B. Three of our patients died because of secondary infections and hemorrhage. Relapse was observed in two patients. No patient showed post kala-azar dermal leishmaniasis findings. We conclude that VL should be considered in patients with prolonged fever, hepatosplenomegaly and cytopenia who live in an endemic region. Amphotericin B is a therapeutic agent as effective as pentavalent antimony compounds and could be preferred.
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PMID:Visceral childhood leishmaniasis in southern Turkey: experience of twenty years. 1937 83

Visceral leishmaniasis (VL) caused by the parasite Leishmania donovani, is a potentially fatal disease. It is characterized by prolonged fever, enlarged spleen and liver, substantial weight loss and progressive anemia. Available drugs are toxic, costly and require prolonged treatment duration viz; 28 days of oral treatment with miltefosine, 30 days infusion with Amphotericin B and 21 days intramascular with paromomycin sulfate. Drug combination for VL clinically proved to shorten the duration of treatment. The efficacy of drugs is also compromised due to suppression of immune function during the course of infection. To combat this situation leishmanicidal efficacy of already marketed standard antifungal drug, ketoconazole under the approach of 'therapeutic switching' in combination with standard antileishmanial drug, miltefosine and a potent immunomodulator agent, picroliv were evaluated in L. donovani/hamsters model. Animals treated with combination of ketoconazole (50 mg/kg, 5 days, po)+miltefosine (5 mg/kg, 5 days, po) showed augmentation in efficacy against leishmania parasite (72%) in comparison to those treated with ketoconazole (54.67%) and miltefosine (54.77%) separately. Co-administration of picroliv (10 mg/kg, 12 days, po) has further enhanced antileishmanial efficacy from 72% to 82%. Significant generation of ROS, RNS and H(2)O(2) and increased phagocytosis was observed in animals treated with ketoconazole+miltefosine; however, addition of picroliv to this combination did not alter the level of metabolites and phagocytosis due to its antioxidative and nonleishmanicidal characteristics, respectively. Significant rise in cell mediated immunity witnessed in this group reveals the role played by the immunomodulator, picroliv and justifies the significance of enhanced cell mediated immunity in the therapy. These findings suggest a new strategy for leishmanial chemotherapy at reduced cost and toxicity.
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PMID:Improved treatment of visceral leishmaniasis (kala-azar) by using combination of ketoconazole, miltefosine with an immunomodulator-Picroliv. 2167 79

Leishmaniasis affects both the visceral and cutaneous tissues in body. Oral Mucosal involvement in leishmaniasis is rare and is often overlooked. We present a case 17 year old boy from the north east region of Bihar who has a history of visceral leishmaniasis one year back, came to the department of oral surgery for treatment of persistent oral ulcers. Oral examination did not give any diagnostic information while systemic examination revealed enlarged spleen and low grade fever. Patient was screened for leishmaniasis by rK39 based immunochromatographic strip test which came to be positive. Biopsy of the ulcer as well as splenic and bone marrow aspirate confirmed the presence of leishmaniasis. Patient was administered Amphotericin B for 20 days following which significant clinical and haematological improvement followed.
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PMID:Oral mucosal involvement in visceral leishmaniasis. 2337 44


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