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)

We retrospectively reviewed the charts and radiographs of 38 patients with systemic-onset juvenile rheumatoid arthritis, attempting to identify early in the disease course the clinical and laboratory observations most predictive of the later development of destructive arthritis. In 12 of the patients, destructive arthritis developed within 2 years of disease onset. When first examined, these patients could not readily be differentiated from those in whom joint destruction did not develop, but they more commonly had hepatosplenomegaly (p less than 0.04), serositis (p less than 0.01), and a lower mean serum albumin concentration (26.7 vs 31.3 gm/L; p less than 0.02). However, by 6 months after onset, patients with destructive arthritis more frequently had persistent systemic symptoms (92% vs 12%; p less than 0.0001), polyarthritis (67% vs 19%; p less than 0.0005), a lower mean hemoglobin level (95 vs 114 gm/L; p less than 0.001), a higher mean leukocyte count (21.2 vs 10 x 10(9)/L; p less than 0.0003), a higher mean platelet count (794 vs 400 x 10(9)/L; p less than 0.0001), and a higher mean erythrocyte sedimentation rate (43 vs 24 mm/hr; p less than 0.05). Multivariate analysis of the results at 6 months revealed that persistent systemic symptoms and a platelet count greater than or equal to 600 x 10(9)/L were the variables most highly predictive of the later development of joint destruction. We conclude that patients at high risk for the development of destructive arthritis may be identified within 6 months of disease onset, thereby indicating the need for more aggressive early therapy.
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PMID:Prognostic indicators of joint destruction in systemic-onset juvenile rheumatoid arthritis. 173 15

We report a patient with the syndrome of large granular lymphocytes in whom the initial clinical features were polyarthritis, hepatosplenomegaly and neutropenia. Relative lymphocytosis was also demonstrated at the expense of a subpopulation with morphology and surface markers characteristic of large granular lymphocytes (CD2+, CD8+, CD16+ and HNK-1+). After 6 months of asymptomatic course, without changes in clinical or laboratory data, the patient died from an acute abdomen with mesenteric ischemia of different likely causes as suggested by necropsy data (multivisceral diffuse infiltrate by large granular lymphocytes, systemic vasculitis and Clostridium sepsis). The association between this syndrome and systemic vasculitis is discussed.
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PMID:[Vasculitis associated with proliferation of large granular lymphocytes]. 225 May 16

We analysed cases of hematologically proved cases of Plasmodium falciparum malaria encountered from July 1984 to Dec 1985 in a military hospital. The age range was 3 days to 10 years. Majority presented with classical symptom of fever (92.7%). Others presented with gastrointestinal (7.3%), hepatic (4.5%), central nervous system (7.3%) and respiratory (3.6%) symptoms. Anemia was found in 36.4%. Hepatosplenomegaly was present in majority of the cases. The rare findings were polyarthritis, rash, urticaria and cerebellar features. All cases responded to chloroquine or quinine. No resistance to drugs was found and there was no mortality.
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PMID:Falciparum malaria. 268 Sep 41

A sister and brother with congenital leucocyte adhesion deficiency developed systemic-onset juvenile rheumatoid arthritis (JRA). They showed polyarthritis, spiking fever, reddish eruptions, anaemia, hepatosplenomegaly, increased erythrocyte sedimentation rate, and positive rheumatoid factor. Occurrence of JRA in our patients was thought to be mainly due to a combination of recurrent bacterial infections and abnormal lymphocyte function as a consequence of membrane adhesion-protein deficiency. In view of the familial occurrence, hereditary factors may have played a role in the development of JRA in our patients.
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PMID:Juvenile rheumatoid arthritis in two siblings with congenital leucocyte adhesion deficiency. 323 29

A case is presented with early-onset polyarthritis involving both large and small joints, prolonged fever, skin rash, hepatosplenomegaly, persistent cerebro-spinal fluid pleocytosis, brain atrophy, macrocephaly with ventricular dilatation, a persistently open fontanelle, lymphadenopathy, subcutaneous nodules, developmental delay, failure to thrive, persistent hypochromic microcytic anemia, leukocytosis with shift to the left, early thrombocytopenia followed by thrombocytosis, high erythrocyte sedimentation rate, elevated immunoglobulin level, and vasculitis involving several organs. Thirteen cases have been previously reported under different names. A unified name is needed; we suggest "infantile-onset arthritis and multisystem inflammatory disease."
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PMID:Infantile-onset arthritis and multisystem inflammatory disease: "a new syndrome". 355 12

The clinical picture of twelve patients with sarcoidosis commencing under 4 years of age is presented. Follow-up ranged from 5 to 30 years with a mean of 11.5 years. Two patients were mother and daughter, the other cases were sporadic. All children developed the typical symptom triad of rash, polyarthritis and uveitis with onset before their 4th birthday. Further symptoms comprised fever, hepatosplenomegaly, parotid swelling and hypertension. Eight patients suffered from cardiac and four from cerebral involvement; one girl died from cerebral involvement at the age of 12. Two developed severe lung changes. The prognosis was poor with sequelae in all patients. Several children seem to have improved on immunosuppressive therapy. Early onset sarcoidosis is a multisystemic granulomatous disease; since polyarthritis is a dominant manifestation, many patients are diagnosed as having juvenile chronic arthritis. The early onset form must be distinguished from later onset sarcoidosis, which mainly affects children over 8 years of age or young adults. The disease types differ in their presentation, symptoms, course and prognosis; some of the clinical features and the histology, however, are identical.
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PMID:Sarcoidosis of early onset. A challenge for the pediatric rheumatologist. 829 66

A previously healthy 18-year-old boy presented with daily spiking fever, polyarthritis, and evanescent skin rashes, as well as hepatomegaly and Raynaud's phenomena for 2 months. He was initially diagnosed with adult-onset Still's disease (AOSD). During the period of follow-up, intermittent fever and migratory polyarthritis persisted and an insidiously growing mass over the right axillary region was noted 1 year after the diagnosis of AOSD. Excisional biopsy of the mass revealed a group of lymph nodes with histological features of the hyaline vascular type of Castleman's disease. The patient's symptoms disappeared soon after excision of the lymph nodes. evanescent rash, lymphadenopathy, hepatosplenomegaly and serositis [5]. A clinical picture compatible with the diagnosis of AOSD has not been described in the localised hyaline vascular type of Castleman's disease. We report such a case in an 18-year-old male patient who presented prolonged fever and polyarthritis with an initial diagnosis of AOSD. The diagnosis of hyaline vascular type of Castleman's disease was made 1 year later, when the patient developed an insidiously growing mass over the right axilla.
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PMID:Localised hyaline vascular type of Castleman's disease mimicking adult-onset Still's disease. 1063 75

Two children with systemic-onset juvenile chronic arthritis (S-JCA) suffering from severe, symptomatic thrombocytopenia are reported. Case 1 is a 10-year-old girl who had fever, chronic polyarthritis and hepatosplenomegaly for 6 months and severe gum bleeding and generalised petechiae for 1 week. Immune thrombocytopenic purpura (ITP) was indicated by bone marrow findings of megakaryocytic hyperplasia, and her thrombocytopenia responded well to steroid therapy without recurrence, but progressive, deforming polyarthritis with anaemia and thrombocytosis persisted. Case 2 is a 7-year-old girl who presented with spiking fever, arthritis and skin rashes and was suspected of having S-JCA. She developed a severe nosebleed with impending shock while receiving full-dose aspirin. Pancytopenia and elevated liver enzymes were also noted. Bone marrow aspiration revealed erythroid hyperplasia, maturation arrest of myeloid series with increased blasts, and adequate megakaryocytes. Her thrombocytopenia resolved in 1 week without specific treatment. Her subsequent clinical course confirmed the diagnosis of S-JCA, while thrombocytopenia did not recur during follow-up.
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PMID:Thrombocytopenia in systemic-onset juvenile chronic arthritis: report of two cases with unusual bone marrow features. 1120 51

We report the case of a woman with a characteristic transient skin rash, fever, severe polyarthritis, hepatosplenomegaly, lymphadenopathy and myalgia. The clinical and laboratory data led to a diagnosis of adult-onset Still's disease. The elevated levels of serum ferritin and caeruloplasmin could be important as diagnostic indicators.
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PMID:Elevated serum caeruloplasmin level in a patient with adult Still's disease. 1195 55

A 31-year-old man with Crohn's disease developed arthritis, spiking fever, and skin rash indistinguishable from that of adult-onset Still's disease. He was admitted to our hospital because of a periumbilical intestinal skin fistula. Crohn's disease had been diagnosed in 1991, and had required intestinal resection twice, and schizophrenia had been diagnosed in 1993. He developed polyarthritis and spiking fever, accompanied by a macular skin rash on both forearms. Marked hepatosplenomegaly and bilateral pleural effusion were detected on computed tomography examination. These findings are indistinguishable from those of adult-onset Still's disease. Because his mental status had deteriorated following high-dose prednisolone on a previous admission, he was treated with an immunosuppressive agent on this occasion, with the treatment being successful. This is the first report of adult-onset Still's disease complicating Crohn's disease. In patients with Crohn's disease, polyarthritis and skin rash can easily be misdiagnosed as enteropathic arthritis with erythema nodosum associated with the Crohn's disease. Although adult-onset Still's disease may not be fatal, early diagnosis is important because it can, in rare cases, result in life-threatening complications.
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PMID:Crohn's disease complicated by adult-onset Still's disease. 1456 35


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