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)

By presenting this case report describing Parinaud's oculoglandular syndrome, we review the medical literature on its most frequent etiology: catscratch disease, a self-limited, systemic illness caused by a Gram-negative bacillus, Bartonella henselae, principally affecting children under 15 years of age. Typical symptoms include regional lymphadenopathy, fever, malaise, and fatigue, possibly with more severe complications such as splenomegaly, granulomatous hepatitis, and encephalopathy. Ocular manifestations may include follicular conjunctivitis, Parinaud's oculoglandular syndrome, neuroretinitis, optic neuritis, and chorioretinitis. Diagnosis is based on serologic tests, and when necessary, antimicrobial treatment can be considered.
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PMID:[Cat's cratch disease and Parinaud's oculoglandular syndrome]. 1502 49

The characteristic rash of systemic juvenile idiopathic arthritis is a transient erythematous eruption associated with a quotidian spiking fever. Usually asymptomatic, it can be pruritic, with dermatographism at sites of scratching or pressure. An illness similar to this entity in adults is designated adult-onset Still disease. The relationship between the pediatric and adult disease is uncertain and differences in case definition have evolved. Specifically, a sustained arthritis for at least 6 weeks is required for a diagnosis of systemic juvenile idiopathic arthritis, whereas transient arthritis and arthralgia are accepted criteria in adult-onset Still disease. We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption. Intense pruritus and linear erythematous lesions flared with a spiking fever, usually in the late afternoon and evening. Periorbital edema/erythema and nonlinear urticarial lesions were also seen. Two children had splinter hemorrhages of the nail beds and one girl developed a fixed, scaling, pigmented, linear eruption. Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient. Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis. The course of the disease was variable. One patient, diagnosed with macrophage activation syndrome, recovered on oral naproxen. Two patients responded to systemic corticosteroid therapy. One girl developed status epilepticus and died from aspiration and asphyxia. A boy with severe hepatitis developed renal failure and thrombotic thrombocytopenic purpura and was treated with plasmapheresis, dialysis, and systemic corticosteroids; he had recurrent episodes of rash and fever into adult life. These children did not fulfill the case definition of systemic juvenile idiopathic arthritis because they lacked a persistent arthritis. Adolescent and adult patients with the same clinical and laboratory findings are described under the rubric of adult-onset Still disease. Recognition of the distinctive urticarial skin eruption and spiking fever is important in the diagnosis of a disease with severe morbidity and potentially life-threatening complications.
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PMID:A pruritic linear urticarial rash, fever, and systemic inflammatory disease in five adolescents: adult-onset still disease or systemic juvenile idiopathic arthritis sine arthritis? 1546 68

Thrombocytopenic purpura associated with brucellosis has been rarely reported in the world literature. Thrombocytopenic purpura is generally part of the array of manifestations of brucellosis such as fever, arthritis, malaise and hepatosplenomegaly. We describe a nine-year-old girl who presented with thrombocytopenic purpura as the sole manifestation of brucellosis, which resolved with anti-Brucella chemotherapy. Her physical examination was remarkable for hepatomegaly of 3 cm and splenomegaly of 2 cm palpable below the costal margin. Initial laboratory investigations revealed isolated thrombocytopenia with platelet count of 11,300/mm3 and positive serology for Brucella. Thrombocytopenia resolved promptly with proper antibiotics on 7th day of treatment. Brucellosis should be included in the differential diagnosis of thrombocytopenic purpura in Brucella-endemic areas.
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PMID:Thrombocytopenic purpura as only manifestation of brucellosis in a child. 1550 83

A 31-year-old man was admitted to the hospital because of a low-grade fever, general malaise, nausea, vomiting, and a poor appetite. On admission his renal function was severely deteriorated (serum creatinine 16.12 mg/dl, BUN 163 mg/dl), and he had severe anemia (Hb 7.5 g/dl) and thrombocytopenia (67,000/microl). A radiological examination revealed the presence of multiple cysts in his kidneys bilaterally. The patient was diagnosed as having end-stage renal disease due to polycystic kidney disease, and hemodialysis was started on the day of admission. After the initiation of hemodialysis, his symptoms and laboratory tests improved, except for anemia and thrombocytopenia. He was noted to have marked splenomegaly and dilation of the portal vein, raising the suspicion of portal hypertension as the cause of the splenomegaly and pancytopenia. To treat his pancytopenia (anemia and thrombocytopenia) and to determine the reason for his portal hypertension, a splenectomy and open-wedge biopsy of the liver were performed. Histological findings in the liver included extensive fibrosis of the portal areas with an excess of moderately dilated bile ducts, compatible with a diagnosis of congenital hepatic fibrosis. After splenectomy, his red blood cell and platelet counts returned to normal, and he was discharged on maintenance dialysis. Congenital hepatic fibrosis is often associated with autosomal recessive polycystic kidney disease (ARPKD), but not with autosomal dominant polycystic kidney disease (ADPKD). However, both his mother and older brother had multiple renal cysts, indicating that this was an unusual case of ADPKD complicated by congenital hepatic fibrosis.
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PMID:[Case of autosomal dominant polycystic kidney disease associated with congenital hepatic fibrosis]. 1597 90

Virus-associated hemophagocytic syndrome (VAHS) is a rare complication in acute hepatitis A virus (HAV) infection. There is no standard therapy for VAHS and the clinical course is variable. Data on the use of intravenous immunoglobulin (IVIG) in the treatment of HAV-associated VAHS is limited. We report a previously healthy, 32-year-old man who presented with general malaise, fever, chills and splenomegaly. Laboratory examination showed marked elevation of aminotransferase, leukopenia and thrombocytopenia. Acute hepatitis A was documented by the presence of immunoglobulin M anti-HAV and compatible liver biopsy findings. Bone marrow examination revealed extensive hemophagocytosis. IVIG was administered after the diagnosis of HAV-associated VAHS. His symptoms and laboratory abnormalities improved, and following a smooth recovery he was discharged 1 month after admission.
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PMID:Successful treatment of acute hepatitis A-associated hemophagocytic syndrome by intravenous immunoglobulin. 1609 28

A 36-year-old man was admitted because of numbness and muscle weakness in the lower extremities. He had gait disturbance, malaise, and body weight loss. Based on the existence of monoclonal gammopathy, the proliferation of abnormal plasma cells in the bone marrow, the presence of sclerotic bone lesion, polycythemia, mild splenomegaly, and an elevated level of serum vascular endothelial growth factor (VEGF) (14,900 pg/mL; normal, 62-707), he was diagnosed as having peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. Following 2 courses of conventional chemotherapy with doxorubicin and dexamethasone, peripheral blood stem cells were mobilized by high-dose etoposide (500 mg/m(2) x 3 days) and granulocyte colony-stimulating factor. After purging by CD34+ selection using the CliniMACS device, the selected cells (12.4 x 10(6)/kg) were cryopreserved. He was then treated with tandem high-dose chemotherapy (HDC) (melphalan 100 mg/m(2) x 2 days) with autologous stem cell rescue. After the first course of HDC, the serum level of VEGF normalized and the minimal residual disease in the bone marrow was reduced below the detection limit of CDR3 analysis by polymerase chain reaction. The patient has been in remission for more than 20 months. He has gradually recovered from the neurological symptoms and now has no impairments of daily living. Our experience suggests that autologous purged stem cell transplantation should be considered as the treatment of choice for POEMS syndrome.
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PMID:Successful treatment of a patient with POEMS syndrome by tandem high-dose chemotherapy with autologous CD34+ purged stem cell rescue. 1692 43

Virus-associated hemophagocytic syndrome (VAHS) is a rare complication in early cytomegalovirus (CMV) infection. There is no standard therapy for VAHS and the clinical course is variable. Data on the use of intravenous immunoglobulin (IVIG) in the treatment of CMV-associated VAHS are limited. We report a previously healthy, 32-year-old woman who presented with general malaise, fever, chills, and splenomegaly. Laboratory examination showed marked elevation of aminotransferase, leucopoenia, and thrombocytopenia. Acute CMV-infection was documented by the presence of immunoglobulin M anti-CMV and positive viremia in blood sample. Bone marrow examination revealed extensive hemophagocytosis. IVIG was administered after the diagnosis of CMV-associated VAHS. Her symptoms and laboratory abnormalities improved dramatically after the onset of the treatment and she did not require antiviral agent.
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PMID:Successful treatment of cytomegalovirus-associated hemophagocytic syndrome by intravenous immunoglobulins. 1784 65

Pulse therapy is one of the most well-known methods used in the treatment of juvenile rheumatoid arthritis. This study assessed the outcome of methylprednisolone pulse therapy, its rate of efficacy, and its associated complications in patients with juvenile rheumatoid arthritis (JRA). This cross-sectional study was performed on 120 children with JRA who attended the Pediatric Ward of Imam Khomeini Hospital from 1994 to 2004 and who had undergone around 500 cycles of methylprednisolone pulse therapies. Clinical signs, including signs of improvement, complications, or recurrence of disease, were noted. SPSS version 11.5 and paired t test were used to compare the variables prior to and after treatment. Clinical signs observed included: feeling of weakness (100%), malaise (98.3%), loss of appetite (93.3%), fever (88.3%), skin rash (28%), lymphadenopathy (18.3%), serositis (4.2%), splenomegaly (3.3%), and hepatomegaly (1.7%); however, none of these findings were present after pulse therapy. The number of swollen and tender joints, duration of morning stiffness, erythrocyte sedimentation rate, C-reactive protein, and hemoglobin levels showed significant improvement after pulse therapy. Complications of pulse therapy included tachycardia (n = 16, 13.3%), hypertension (n = 10, 8.3%), headache (n = 2, 1.7%), and flashing (n = 2, 1.7%). The mean duration of remission was 3.3 +/- 0.7 months.
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PMID:Efficacy of methylprednisolone pulse therapy in children with rheumatoid arthritis. 1860 80

Hepatitis C is caused by the hepatitis C virus (HCV) infection. According to World Health Organization data, 3% of the world population (approximately 170 million people) is infected with HCV; in Poland there are over 700,000. Over 70% of those infected manifest no symptoms in the acute phase of the disease, and in about 70-80% the acute phase progresses into a chronic form. Patients with symptoms in the acute phase of HCV infection most commonly present with unspecific signs and symptoms that may develop in other viral liver infections, e.g. malaise, fatigue, abdominal pain, mild hepato- and splenomegaly and arthralgia. These symptoms usually persist for 2 to 12 weeks. In the chronic phase a subset of patients complain of malaise, nausea, abdominal pain and itching. With time, chronic hepatitis C may develop into liver cirrhosis. The basic diagnostic methods in HCV infection involve determination of anti-HCV antibodies using the ELISA immunoassay and examination of HCV-RNA with the RT-PCR method. The current treatment of HCV infection involves administration of pegylated interferon a and ribavirin over a period of 48 weeks in HCV-1 genotype infection, and 24 weeks for HCV-2 and 3 genotypes. Effectiveness of therapy depends on the HCV genotype. HCV elimination can be achieved in 78% of patients with HCV-2 and 3 genotypes, and in 55% of patients with HCV-1 genotype.
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PMID:Viral hepatitis C. 1920 52

A 57-year-old-man with a history of malaise, fever, night sweats and shortness of breath presented a diagnostic challenge to his medical team. He was pancytopaenic and had splenomegaly on admission but other investigations, including bone marrow aspiration, proved inconclusive. After the patient deteriorated clinically, the general surgical team was requested to perform a diagnostic splenectomy. The histology of this showed infection with visceral leishmaniasis. He recovered completely with Amphotericin treatment. Although this is a rare condition, particularly for the general surgeon, this case highlights the difficult position surgeons are often put in when performing major surgery diagnostically.
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PMID:Diagnostic splenectomy for visceral leishmaniasis. 1933 64


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