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)

Eight cases of acute human herpesvirus type 6 (HHV6) infection in infants were diagnosed serologically by the demonstration of IgM anti-HHV6 (8/8) and a significant change in total anti-HHV6 antibody titre (6/8). Four infants were sufficiently ill to require admission to hospital and further investigations: one with encephalitis and three with gross hepatosplenomegaly, two of whom had evidence of simultaneous infection with another herpes-virus. The remaining four infants had an illness compatible with roseola infantum, although this diagnosis had not been made clinically. Sera from two of those infants with rash had been sent for analysis to exclude rubella because the infants' mothers were pregnant. The other two had received antibiotics when febrile, and the subsequent appearance of the roseola rash had raised the possibility of antibiotic allergy. The data suggest that there are clinical syndromes in addition to roseola infantum associated with the presence of IgM anti-HHV6, in which serological screening for evidence of acute HHV6 infection may be useful.
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PMID:Roseola infantum and other syndromes associated with acute HHV6 infection. 217 78

Severe hepatotoxicity from phenobarbital occurred in an infant boy who had a complicated illness with chronic bilateral subdural hematomas and sepsis. Skin rash began after 2 weeks of treatment, and signs of hepatocellular failure developed 3 weeks after phenobarbital had been started. Signs of severe liver disease included elevated aminotransferases, conjugated hyperbilirubinemia, significant coagulopathy, hepatosplenomegaly and ascites. Other features of this adverse drug reaction were unremitting fever, leukocytosis with eosinophilia and atypical lymphocytosis, and proteinuria. Sepsis, viral hepatitis, and metabolic liver disease were excluded. The child was on no other medication and had been previously well. In-vitro rechallenge of the patient's lymphocytes with cytochrome P-450 generated metabolites of phenobarbital showed extensive cytotoxicity compared to control. These data support the hypothesis that a defect in drug detoxification was responsible for the child's susceptibility to this drug hepatotoxicity.
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PMID:Phenobarbital hepatotoxicity in an 8-month-old infant. 233 96

Rickettsial infections of a spotted fever group have recently been experience in Japan. Although there have been 50 cases reported by this time, they were exclusively distributed in the areas along the Pacific coast. We report in this paper the first case of spotted fever group rickettsiosis in Shimane prefecture, an area faced to the Japan Sea. A 57 year-old man with high fever, general fatigue and rash was admitted to the hospital of Shimane Medical University at the end of September 1987, who had been treated with cefaclor for a few days without effect before his visit to the University Hospital. Physical examination revealed erythematous eruption, eschar on the left side of the abdomen, and generalized lymphadenopathy. Hepatosplenomegaly was not detected. Laboratory studies showed normal leukocyte counts with relative lymphopenia, high ESR and increased CRP. Transaminase levels were slightly elevated. Paul-Bunnel and Weil-Felix tests resulted in negative. Specific immunofluorescence tests demonstrated that IgG and IgM antibodies of acute-phase serum to Rickettsia japonica, a spotted fever group rickettsia isolated from patients in Japan, were not detected, while both IgG and IgM antibody titers of convalescent-phase serum increased to 1:320. Convalescent-phase serum reacted at significantly lower titers with R.typhi as well as other pathogenic spotted fever group rickettsiae. The patient was treated with ofloxacin for six days and then with combination of ofloxacin and minocycline. It should be noted that clinical symptoms such as high fever and general fatigue disappeared during a single therapy with ofloxacin.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case report of spotted fever group rickettsiosis first encountered in Shimane Prefecture, Japan]. 233 50

The patient was a 29-year-old woman. She was well until autumn 1983, when she presented with polyarthralgia, fever above 39 degrees C, hepatosplenomegaly, swelling of lymphnode and salmon pink rash. Laboratory tests revealed marked leucocytosis with shift to the left, elevated ESR, strong positivity of CRP and abnormal liver function tests. However, anti-nuclear antibody and RA factor were negative. She was diagnosed as adult onset Still's disease (AOSD) by characteristic clinical course and laboratory data. During her disease course these abnormal findings could be well controlled neither by nonsteroidal anti-inflammatory drugs, immunosuppressive agents nor corticosteroids. Two and half years after the first admission, she began to complain of dry cough, dyspnea on efforts. Auscultation revealed an increased pulmonic sound and systolic murmur of cardiac apex. Chest X-Rays showed enlarged main pulmonary arteries. The lung fields were normal. Pulmonary function tests gave no evidence of a significant obstructive or restrictive defect but showed the low DLco and hypoxemia. Ventilation-perfusion lung scanning failed to reveal pulmonary embolism. Finally, right heart catheterization confirmed the pulmonary precapillary hypertension. Her pulmonary hypertension has progressed rapidly, strongly suggesting poor prognosis. Her pulmonary hypertension associated with no apparent parenchymal involvement was thought to be caused by a pulmonary vascular change probably related to AOSD. This case is a first case of AOSD with pulmonary hypertension.
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PMID:[A case of adult Still's disease with pulmonary hypertension]. 237 40

A trial is presented of evaluation of the frequency of clinical symptoms and signs, activity of disease process, and course of the disease in a group of children with juvenile chronic arthritis with generalized manifestations at onset. The analysed group comprised 36 children aged from 2 to 15 years. The duration of the disease was from 6 months to 13 years. The evaluation of the most frequent symptoms and signs in the so called early period of the disease covered the first year after the beginning of the first clinical signs. During the disease involvement of many internal organs develops and establishing of correct diagnosis at the onset is difficult, particularly, since in this initial period the signs of articular involvement may be absent or slight. Arthritis in this early period was observed in only 7 of 36 children. The most frequent symptoms and signs in decreasing frequency were: fever, rash, arthralgias, lymphadenopathy, hepatosplenomegaly. Heart involvement was noted in 16 of 36 cases, and pneumonia in 7 of 36. Anaemia and leucocytosis were present in all cases. Amyloidosis was recognized in 6 of 36 cases. In view of the severe course of the disease all children were treated with glycocorticoids, and cytostatic agents were given to those with evidence of amyloidosis.
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PMID:Considerations on juvenile chronic arthritis with generalized (polysystemic) onset. 248 68

We reported a case of adult Still's disease accompanied by pericarditis, pleuritis and extensive pneumonitis with respiratory failure. A 59-year-old woman was admitted to our hospital because of high grade fever and sore throat. She had a spiking fever between 38 degrees C and 40 degrees C. Surface lymph nodes were palpable in the neck and inguinal lesions and hepatosplenomegaly was recognized. Laboratory data showed a marked increase in peripheral leukocytes, erythrocyte sedimentation rate, liver dysfunction and anemia. Serologic tests were negative for various autoantibodies and rheumatoid factor. She received various antibiotics, but there was no improvement. Later, a rheumatoid rash which suggested adult Still's disease developed. The diagnosis of adult Still's disease was made by skin biopsy and clinical course. Although pericarditis, pleuritis and extensive pneumonitis were accompanied with severe respiratory failure, her symptoms improved with steroid pulse-therapy. A transbronchial lung biopsy revealed moderate fibrosis and cell infiltration in alveoli.
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PMID:[A case of adult Still's disease with severe pulmonary complications]. 258 6

A 6-year-old girl with cerebral palsy developed conscious disturbance and generalized convulsion after one-hour hot herb drug bath. Physical examination on admission revealed rectal temperature 41 degrees C, hot skin, respiration 46/min, regular heart beat 98/min, BP 130/60 mmHg, Glascow coma scale 4 (E2M1V1), soft and flat abdomen, no hepatosplenomegaly, no skin rash, no focal neurological sign, increased generalized muscle ton. Laboratory data showed CBC: WBC 20400 cumm (Neutrophils 31%, Lymphocytes 69%), Hb 11.6gm%, ESR 11 mm/hr, arterial blood gas: PH 7.077, PO2 43mmHg, PCO2 57.1mmHg, HCO3- 16 mEq/L, BE-11.5mEq/L, serum sodium 143 mEq./L, potassium 5.2 mEq/L, chloride 101 mEq/L, free calcium ion 3.8mg%, GOT 63IU/L, GPT 263 IU/L, amylase 193 IU/L, alkaline phosphatase 388 IU/L, LDH 1245 IU/L, CPK 677 IU/L, total bilirubin 0.8 mg/dl, direct type 0.1 mg/dl, BUN 18 mg/dl, Glucose 35 mg/dl. Urinalysis revealed proteinuria( ) trace hematuria and pyuria, but no cast. Lumbar puncture is within normal limits. Bacteriology including blood and CSF are normal. Multiple organ failure was noted at that time. Intensive cooling methods were performed including central and peripheral cooling. We used luminal and valium to control the seizure. Condition didn't improve. Afterwards cardiopulmonary arrest developed. Patient expired 8 hours after admission despite of resuscitation. Heat stroke in infancy and childhood is different from that in adulthood. The predisposing factors are high ambient temperature, dehydration, very young baby, sweat gland dysfunction, or ectodermal dysplasia. Definition of heat stroke includes 1) rectal temperature above 41 degrees C, 2) behavioral change, 3) warm skin, wet or dry.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Status epilepticus induced by prolonged immersion in hot herb bath: report of one case]. 263 19

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

Neonatal onset multisystem inflammatory disease is a rare disorder first described by Lorber in 1973. An additional 29 cases have been recorded. Two patients are described here, one with a 17 year follow-up. The typical features are a rash, fever, adenopathy, hepatosplenomegaly, and a severe, deforming arthropathy predominantly affecting large joints. The most striking feature is the onset in the neonatal period. Other associated features include inflammation, chronic meningitis, anemia, and persistent leukocytosis. Most, if not all, patients develop bizarre epiphyseal radiographic findings that are virtually pathognomonic. This disease is distinct from Still disease.
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PMID:NOMID--a neonatal syndrome of multisystem inflammation. 278 38

Cytomegalovirus (CMV) infection is relatively frequent and severe in immunosuppressed patients giving rise to diagnostic and therapeutic problems. We describe a series of 7 patients, six with acute lymphoblastic leukemia and one with aplastic anemia. All patients had CMV infection at the moment of maximum immunodepression. Two patients had undergone recent bone-marrow transplant. Six had been transfused in the two months prior to the onset of infection. Diagnosis was established through isolation of CMV from blood or serological methods. Symptoms ranged from prolonged fever to multi-organic involvement. Two cases had pulmonary involvement as well as fever, hepatitis and petechial rash. Two other cases presented with fever and hepatosplenomegaly and in the remaining, 3, fever was the only sign. Clinical course was favourable in all cases including the two with pneumonitis; of these two the first received acyclovir and anti-CMV Ig and the other received no specific therapy. One of the remaining cases was also given acyclovir and specific anti CMV Ig was administered to the 3 patients with isolated fever. In conclusion, CMV infection should be suspected in immunosuppressed patients with prolonged fever.
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PMID:[Cytomegalovirus disease in immunosuppressed patients]. 283 17


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