Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Angioimmunoblastic lymphadenopathy with disproteinemia (AILD) is a systemic lymphoproliferative disorder characterized by constitutional symptoms such as generalized lymphadenopathy, hepatosplenomegaly and skin rash. In this article, we report on a patient with seronegative Rheumatoid Arthritis of 18 years duration who recently developed AILD.
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PMID:Angioimmunoblastic lymphadenopathy with disproteinemia arising in a patient with rheumatoid arthritis. 786 84

Childhood sarcoidosis is a rare disorder with protean manifestations. The case of a child with prolonged fever, hepatosplenomegaly, pancytopenia, and systemic necrotizing vasculitis manifesting as fever, rash and skin infarctions, digital pregangrene, and foot drop is reported. This is the first case of systemic necrotizing vasculitis reported in sarcoidosis. The fulminant course of the disease required treatment with intravenous pulsed cyclophosphamide and high doses of corticosteroids. The spectrum of vasculitis in childhood and adult sarcoidosis is reviewed.
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PMID:Systemic necrotizing vasculitis associated with childhood sarcoidosis. 793 24

Out of 330 adult Systemic Lupus Erythematosus (SLE) cases who attended the Rheumatic Care Centre, Government General Hospital, 59 children were analysed. There was no case with onset before the age of 5 years. There were 49 females and 110 males (M:F = 1:4.9). The initial manifestations were fever (67%), arthritis (61%), skin rash (59%) and lymphadenopathy (27.1%). There was no case of Raynaud's phenomenon. Only 10.1% of patients presented with thrombocytopenic purpura. In the cumulative clinical features, arthritis in 86.6%, fever in 79.8%, skin rash in 69.4%, lymphadenopathy in 61% and hepatosplenomegaly in 39.9% were observed. Renal involvement was seen in 49.1%, neuropsychiatric manifestations in 27.1%, pleuropulmonary in 22% and cardiac manifestations in 10.2%. Anaemia was seen in 50.8%, leukopenia in 18.4%, thrombocytopenia in 11.8%, ANA in 100%, anti-dsDNA in 92.3%, anti-Sm in 34.7%, anti-SSA in 38.5%, anti-SSB in 15.4%, ACL in 30.8%, low C3 in 50% and false positive VDRL in 3.3%. Death occurred in 8 children, 3 due to infection, 2 due to renal causes, 1 due to cardiac and 2 due to central nervous system involvement.
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PMID:Childhood systemic lupus erythematosus in south India. 795 96

Daspone syndrome was noted within six weeks of starting treatment in 1.3% of about 700 leprosy patients on MDT reporting to the skin department of Goa Medical College. Skin rash, photosensitivity, fever, lymphadenopathy, sore throat, hepatosplenomegaly, abnormal liver function tests and raised reticulocyte count were consistent features in all the patients. Other drugs, infectious mononucleosis and viral exanthemata were considered in differential diagnosis. Withdrawal of dapsone and administration of prednisolone controlled the condition within three to four weeks in majority of the patients. One patient died of ischemic heart disease unrelated to dapsone syndrome.
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PMID:Dapsone syndrome in Goa. 798 93

During a four-week trip to Nigeria a 54-year-old German developed a fever of 39 degrees C. Later on he had lymphadenopathy, pretibial oedema, dyspnoea and weight loss. After 16 weeks a wreath-like pale pink skin rash, increased pulse rate with pulse deficit and hepatosplenomegaly were noted. Abnormal laboratory findings were an increased blood sedimentation rate (95 mm), raised immunoglobulin M (483 mg/dl), haemoglobin of 12.0 g/dl, mean corpuscular volume of 76 fl and Borrelia IgM antibody titre of 1:512. The electrocardiogram was suggestive of myocarditis: the cardiac symptoms were controlled with digoxin and verapamil. The patient's general condition deteriorated while he was receiving antibiotic treatment with tetracycline and penicillin. Cerebrospinal fluid (CSF) showed an increased cell count (39/microliters) and albumin (0.98 g/dl). There was a mild, predominantly proximal, tetraplegia which--on the basis of electromyographic and biopsy findings--was thought to be due to polyneuritis and myositis. At this stage blood smear and CSF examination revealed Trypanosoma. He thereupon received suramin (1.0 g) and prednisolone (120 mg down to 40 mg) daily, to which melarsoprol was added after 6 days (0.5 ml up to 5.0 ml daily for 36 days). Almost all symptoms then regressed within 6 weeks.
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PMID:[Polyneuritis and myositis in Trypanosoma gambiense infection]. 798 74

Ten children (five boys and five girls) with juvenile chronic myelocytic leukemia were seen over a period of 12 years (1980-1991) at the All India Institute of Medical Sciences, New Delhi. With the exception of one who was aged 4.5 years, all children were below 4 years of age (mean age 20.4 months). The presenting features included fever, bleeding secondary to thrombocytopenia, marked hepatosplenomegaly, and skin rash. The striking hematological features were anemia, thrombocytopenia, peripheral blood monocytosis, and normoblastemia. There was no significant myeloid proliferation in the bone marrow aspirate (mean M:E = 5:1), while erythroid proliferation was prominent along with monocytosis (mean 11.2%). Fetal hemoglobin was raised in 8 of the 10 patients (mean 14.1%). Long-term survival was poor, with maximum survival being 18 months in one case. New modalities of management of this rare entity are discussed.
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PMID:Juvenile chronic myelocytic leukemia--report of 10 cases. 799 Jul 56

A female newborn was delivered prematurely at gestational age of 26 weeks with birth weight of 1,000 grams. Her initial white blood cell count was 68,900/mm3, which increased to 207,000/mm3 on the fifth day of life with a remarkable shift-to-left pattern. The most immature myeloid series in the peripheral blood smear was promyelocyte; and the promyelocyte, myelocyte and meta-myelocyte accounted for 38% of the peripheral white blood cells. Neither hepatosplenomegaly nor skin rash was noted, but the platelet count dropped to 86,000/mm3 transiently on Day 8. Cytogenetic study for the bone marrow aspiration revealed normal results. The leukemoid reaction recovered within three weeks without specific treatment. Antenatal usage of steroid to promote lung maturation was considered to be one possible cause of the transient leukemoid reaction.
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PMID:Transient leukemoid reaction in an extremely premature neonate. 804 7

The clinical manifestations of pediatric systemic lupus erythematosus (SLE) are similar to those seen in adults with SLE with increased frequency of the following features: hepatosplenomegaly, chorea, nephritis, and avascular necrosis. Similarly, pediatric SLE patients are now showing the same improvement in survival as adult SLE patients, and it is no longer felt that the course of childhood-onset SLE is more severe than that seen in adult-onset SLE. Children of mothers with SLE can develop both transient and persistent features of SLE in the neonatal period. Transient features include photosensitive discoid rash, cytopenia, hepatosplenomegaly, myocarditis, and pericarditis; the permanent features include congenital complete heart block, endomyocardial fibroelastosis, and other structural cardiac defects.
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PMID:Pediatric systemic lupus erythematosus and neonatal lupus. 815 96

We describe a case of disseminated Penicillium marneffei infection in a patient infected with human immunodeficiency virus. The diagnosis was made by examination of a peripheral blood smear. The patient presented with fever, jaundice, generalized lymphadenopathy, hepatosplenomegaly, and an erythematous, papular rash. Microscopic examination of a Wright's-stained peripheral blood smear revealed many yeast cells in neutrophils. Some yeast cells had clear central septation. Presumptive diagnosis of disseminated P. marneffei infection was made, and treatment was started several days before the culture results were available.
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PMID:Disseminated Penicillium marneffei infection diagnosed on examination of a peripheral blood smear of a patient with human immunodeficiency virus infection. 816 35

Multicentric Castleman's disease (MCD), or multicentric angiofollicular lymph node hyperplasia, is an uncommon lymphoproliferative disorder which typically present with constitutional symptoms, multicentric lymphadenopathy, hepatosplenomegaly, effusions, and ascites. We describe a patient with several novel manifestations of MCD: sicca syndrome, lacrimal and salivary gland enlargement, cardiomyopathy, and palmar and plantar rash. Treatment of MCD with chlorambucil and prednisone was effective in the short term followup of this patient. MCD merits consideration in patients with lymphadenopathy and multisystem disease, including sicca syndrome, heart failure or rash.
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PMID:Presentation of multicentric Castleman's disease with sicca syndrome, cardiomyopathy, palmar and plantar rash. 816 21


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