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)

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

Female LSH hamsters infected with Treponema pallidum subsp, endemicum before pregnancy or during early pregnancy transmit a form of syphilis to the fetus that is similar to human congenital syphilis. The offspring develops rhinitis, skin rash, failure to thrive, and hepatosplenomegaly. T. pallidum is detectable in their livers, spleens, and nasal secretions. Immunoglobulin M antibodies are detected in the serum.
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PMID:Experimental model of congenital syphilis. 833 90

An outbreak of louse-borne relapsing fever, due to the return of soldiers to their original recruitment areas, after the end of thirty years of fighting in northern Ethiopia, was reported in Arsi region, southern Ethiopia. The epidemic spread to different members of the community and eventually the schools. We studied 389 patients affected by the epidemic and who were admitted to Asella Hospital between June 1991 and May 1992. Twenty-seven per cent of the patients were ex-soldiers; 28% were students, who were admitted to the hospital since the schools were opened after the summer vacations. The common clinical features of the disease were fever (99%), headache (92%), hepatosplenomegaly (66%), myalgia (55%), arthralgia (51%), petechial rash (43%), epistaxis (24%) and jaundice (23%). Observed complications were pneumonia (10%), pulmonary edema (6%), myocarditis (3%) and 6 abortions in 15 pregnancies. Patients were treated with low dose penicillin and i.v. fluids. The in-hospital case fatality rate was 3.6%. Jarisch-Herxheimer reaction occurred in 43% of the patients. 1.8% of the patients had relapses after treatment.
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PMID:Louse-borne relapsing fever. A clinical and an epidemiological study of 389 patients in Asella Hospital, Ethiopia. 794 Oct 15

A case of a healthy 23-year-old woman is reported with cytomegalovirus mononucleosis as a result of infection of cytomegalovirus probably primary. The patient presented with symptoms of generalized adenopathy, migratory arthralgias and arthritis, hepatosplenomegaly, long lasting rash as well as complications of pneumonia and myocarditis. Because on histopathological examination of lymph node the Hodgkin-Reed-Sternberg-like cells were found a misdiagnosis of Hodgkin's disease was initially made. After about 8 weeks period there was a complete recovery. The current problems related to cytomegalovirus infection are presented.
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PMID:[Difficulties in diagnosis of cytomegalovirus mononucleosis syndrome]. 852 1

A 9-year-old girl was admitted to Mie University Hospital June 25, 1992, complaining of fever, skin rash and pallor. Physical examination revealed anemia and hepatosplenomegaly. Laboratory findings showed normocytic normochromic anemia, increased reticulocyte counts and remarkably decreased haptoglobin level. Red blood cells had no morphological abnormality. Bone marrow examination showed erythroid hyperplasia without abnormal cells. Hemoglobin electrophoresis showed an abnormal band. The amino acid structure and sequence of the abnormal hemoglobin was determined to be an unstable hemoglobin, Hb Buenos Aires [beta 85 (F1) Phe-->Ser]. Sequence of genomic DNA and cDNA was compatible to Hb Buenos Aires. Parvovirus B19 infection was thought to have caused severe anemia due to hemolytic crisis in this patient because its IgM antibody was positive on admission. She recovered soon without any treatment and has been followed in the outpatient clinic. Her parents and brother showed no hemoglobin abnormality.
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PMID:[The first case in Japan of unstable hemoglobin, Hb Buenos Aires [beta 85 (F1) Phe-->Ser], with parvovirus B19 infection (first case in Japan)]. 858 69

An 8-month-old male infant with severe anemia and thrombocytopenia was brought to our hospital due to fever and pallor. The physical examination on admission showed pale conjunctivae, skin rash, lymphadenopathy and hepatosplenomegaly. Subsequent laboratory studies showed pancytopenia, impaired liver function, elevated lactate dehydrogenase level but without evidence of disseminated intravascular coagulation. Bone marrow aspiration demonstrated increased macrophages and prominent hemophagocytosis. The serological studies revealed a recent infection of human herpesvirus-6 which was confirmed by blood mononuclear cell culture and polymerase chain reaction. This infant was also found to have beta-thalassemia which was confirmed by hemoglobin electrophoresis performed before first transfusion. After treatment with intravenous immunoglobulin and other supportive therapies, the bone marrow abnormalities has completely recovered after 5 days, and the hemogram improved. This report demonstrates the human herpesvirus-6 as the etiology of hemophagocytic syndrome.
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PMID:Human herpesvirus-6 associated hemophagocytic syndrome in beta-thalassemia: report of one case. 860 65

Adult onset Still's disease is a rare condition presenting with high spiking fever, transient maculopapular rash, myalgias, polyarthralgias or arthritis, lymphadenopathy, hepatosplenomegaly and a sore throat, associated with leucocytosis and neutrophilia. Early diagnosis is difficult because clinical features are non-specific. We report a 33-year-old Chinese female with this condition who presented with recurrent high spiking fever and rash over a 4-month period. We highlight the sequence of events leading to this diagnosis with emphasis on the cutaneous changes.
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PMID:A case of adult onset Still's disease presenting with fever and a rash. 879 28

A 28-year-old man was admitted to our hospital, because of double vision, memory disturbance and dysarthria. These symptoms developed in November, 1994. His mental activity was gradually decreased and he became apathetic. A physical examination on admission was unremarkable. There were no lymphadenopathy, hepatosplenomegaly or skin rash. Neurological examination revealed disorientation, decreased mental activity and left gaze-evoked nystagmus. Neuropsychological tests demonstrated impairment of recent memory and performance but frontal lobe functions were maintained. Laboratory data showed hyponatremia, elevation of antinuclear antibodies and IgG-antibodies to Epstein-Barr virus capsid antigen and Epstein-Barr virus nuclear antigen. A brain MRI revealed multiple lesions in the cerebral subcortex, cerebellum and brainstem. These lesions showed high signal intensity on T2-weighted images and enhanced high signal intensity on T1-weighted images using Gd-DTPA injection. A cystic mass was seen between bilateral lateral ventricles and contacted with the fornix and thalamus. Lymphomatoid granulomatosis was diagnosed by an open brain biopsy, which showed diffuse infiltration of small lymphocytes, histiocytes and plasma cells, especially around the small vessels. Epithelioid granuloma or necrosis was not seen. An immunohistochemical study showed that nearly all lymphocytes were MT-1 positive T lymphocytes. The patient was treated by whole brain irradiation (30Gy) and pulse therapy of methylprednisolone. Multiple lesions in the brain responded to these modalities with complete resolution of the lesions and clinical improvement. In this case, impressive multiple and cystic lesions seen on brain MRI disappeared by whole brain irradiation and steroid. Since etiologies of multiple lesions on MRI includes many possibilities, histological diagnostic methods should be performed to obtain a definitive diagnosis. This is the first case of lymphomatoid granulomatosis that showed multiple and cystic lesions on brain MRI in Japan.
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PMID:[Lymphomatoid granulomatosis of the brain with multiple lesions on MRI]. 893 97

Two children with acute lymphoblastic leukemia presenting with cellulitis and sepsis are described. Both presented as having hemophagocytic syndrome with the manifestations of prolonged fever, jaundice, pancytopenia, coagulopathy and histiocytic proliferation with hemophagocytosis in their bone marrows. It is similar to the virus-associated hemophagocytic syndrome (VAHS), except for the absences of lymphadenopathy, skin rash and hepatosplenomegaly. Concomitant virus infections were excluded in these two cases. Both patients' conditions improved after appropriate antibiotics and intravenous immunoglobulin therapy. The prognosis seemed better in bacteria-associated hemophagocytic syndrome (BAHS) than in VAHS even in immunocompromised patients.
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PMID:Bacteria-associated hemophagocytic syndrome in childhood acute lymphoblastic leukemia: report of two cases. 907 88

We describe four unrelated children with neonatal maculopapular rash, fever, arthritis, hepatosplenomegaly, lymphadenopathy, eye involvement, and neurologic symptoms. Radiographs of the joints were surprisingly similar, showing an abnormal epiphyseal and metaphyseal appearance. These clinical and radiologic findings allowed us to include these children in a very peculiar syndrome described as infantile-onset multisystemic inflammatory disease. A chondrosarcoma developed in one of our patients.
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PMID:Infantile-onset multisystem inflammatory disease: a differential diagnosis of systemic juvenile rheumatoid arthritis. 910 44


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