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)

Histiocytosis X describes a disease characterized by histiocytic infiltration of the reticuloendothelial system, skin, bones, and pituitary gland. The disseminated form frequently occurs in infants and children. Chemotherapy has significantly improved the prognosis in this disorder. Sixty-three per cent of survivors, however, have some residual disability related to fibrosis of tissues previously infiltrated by histiocytes. In instances of liver involvement, healing by fibrosis may result in cirrhosis with portal hypertension and bleeding esophageal varices. Clinical findings include hepatosplenomegaly, jaundice, ascites, hypoalbuminemia, prolonged prothrombin time, and Bromsulphalein retention. Histologic examination of the liver shows a characteristic dense "macronodular" periportal cirrhotic pattern. Three children with portal hypertension and bleeding varices due to healed histiocytosis X were sucessfully managed by portosystemic shunt procedures. Portacaval, mesocaval, and central splenorenal shunts were equally effective in relieving poral hypertension. These children had neither recurrence of bleeding nor evidence of encephalopathy. Two children remain well whereas in one patient a primary hepatoma developed fourteen years posthung and he died of pulmonary metastases. Portosystemic shunt procedures effectively relieve the threat of potentially fatal variceal hemorrhage and improve the opportunity for long-term survival in children with cirrhosis and portal hypertension due to healed histiocytosis X.
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PMID:Portal hypertension in infants and children with histiocytosis X. 108 50

We describe 3 children with a progressive encephalopathy that was characterised by irritability, convulsions, cranial nerve palsies, ataxia, nystagmus, walking difficulties, delayed psychomotor development, hemiplegia/tetraplegia, visual disturbance, vomiting, neck stiffness, and non-specific signs of raised intracranial pressure. A final diagnosis was made in all 3 patients from necropsy material. The clinical features were ascribed to multiple inflammatory, predominantly lymphocytic, reactions and raised intracranial pressure. This condition is an atypical form of haemophagocytic lymphohistiocytosis, which normally presents with fever, hepatosplenomegaly, and cytopenias. By contrast, the disease pattern in our 3 children was dominated by cerebromeningeal involvement, which can precede the typical systemic symptoms of haemophagocytic lymphohistiocytosis. An awareness of this condition is important because treatments are available.
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PMID:Cerebromeningeal haemophagocytic lymphohistiocytosis. 134 23

The objective for this work was to describe the transmission mechanisms and the clinical behavior of 60 HIV-infected pediatric patients. We studied children from newborn to 15 years old according to the CDC criteria. From January 1985 to February 1992, were evaluated 60 patients, 40 males and 20 females; 25 with perinatal transmission (23 transplacental and 2 breast-feeding), 22 hemophiliacs, 12 by blood transfusion and 1 by intramuscular injection with contaminated needle. The disease was symptomatic in 50 patients, asymptomatic in 5 and indeterminate in 5 cases. Up to date, 28 children are in phase P2, 10 in P0 and P1, and 22 patients have died. The clinical manifestations in 50 patients were: altered growth and development in 50, generalized lymphadenopathy in 30, severe infections in 23, fever in 15, hepatosplenomegaly in 15, chronic diarrhea in 10, and HIV-encephalopathy in one. It is concluded what at present time perinatal transmission is the main mechanism.
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PMID:[AIDS in children. 8 years experience at La Raza Medical Center Infectology Hospital, Mexican Social Security Institute]. 138 82

Cat scratch disease is a subacute regional lymphadenitis usually preceded by a history of being scratched by a cat or young kitten. The spectrum of illness ranges from mild self-limited adenopathy to severe systemic disease, including hepatosplenomegaly, encephalopathy, osteolytic lesions, splenic abscesses, mediastinal masses, and neuroretinitis. Vision loss is a rare complication of the disease. The authors report a patient with cat scratch disease associated with acute febrile illness, lymphocytic meningitis, and acute vision loss secondary to neuroretinitis. To their knowledge, this is the first ophthalmic case reported in which the diagnosis is supported by both a positive skin test and positive histopathology.
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PMID:Cat scratch disease associated with neuroretinitis in a 6-year-old girl. 155 16

This study concerns Budd-Chiari syndrome (BCS) caused by occlusion of the subdiaphragmatic inferior vena cava (IVC). It describes the experimental and clinical evaluation of the treatment of this disorder by one-stage combined portal and vena caval decompression with a direct side-to-side portacaval shunt (PCS) and a caval-atrial shunt (CAS) graft. BCS was produced in rats by gradual occlusion of the suprahepatic IVC with an ameroid constrictor. When ascites and portal hypertension were established, 12 control rats survived a sham thoracolaparotomy, 16 rats survived a mesoatrial shunt, and 16 rats survived combined PCS and CAS graft. All control rats re-formed ascites and died within 2 months. Nine of 16 rats with mesoatrial shunt developed graft thrombosis, re-formed ascites, and died within 2 months. In contrast, only 2 of 16 rats that underwent combined PCS and CAS developed graft thrombosis, re-formed ascites, and died. Liver biopsies showed reversal of severe pathologic changes in rats with patent grafts. Clinical evaluation of combined PCS and CAS using a 20-mm ring-reinforced Gore-Tex graft has been undertaken in five patients with BCS and ascites, hepatosplenomegaly, intense hepatic congestion on biopsy, and angiography showing occlusion of both the IVC and hepatic veins. All five patients are alive and well 6 months to 7.5 years postoperatively with patent grafts, no ascites or need for diuretics, no encephalopathy, normal liver function, and reversal of liver pathology. It is concluded that combined PCS and CAS create a high-flow shunt that decompresses both the portal system and IVC, has a low incidence of graft thrombosis, has been consistently effective in relieving BCS caused by IVC occlusion, and appears to be superior to mesoatrial shunt.
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PMID:Treatment of Budd-Chiari syndrome due to inferior vena cava occlusion by combined portal and vena caval decompression. 173 62

Mansonic schistosomiasis is an endemic disease in Brazil, with an estimated 10-12 million people infested. Among its clinical manifestations, the hepatosplenic form causes portal hypertension which, in turn, brings about severe digestive hemorrhage, the most serious complication of the disease. Normally, the patients are young, and have hepatosplenomegaly, hypersplenism without clinical manifestations, and slightly reduced hepatic function. The angiographic findings are characteristic, differing from those of hepatic cirrhosis. In Brazil, the definitive treatment for gastrointestinal hemorrhage is surgery, which should be done under elective conditions whenever possible. During a short period of time, known as the "risk period" (the time between the hemorrhagic episode and the surgery), propranolol has been used to prevent further bleeding. Surgical treatment is indicated only after the first episode, and never on a prophylactic basis. In 1977, a prospective, randomized trial was begun in order to assess the delayed results of the 3 surgical operations most widely used in this country. The study was interrupted after 94 patients had been operated on due to the high incidence of encephalopathy in the group who underwent classical splenorenal shunt. After a follow-up of at least 60 months and, at most, 130 months, the results showed that classical splenorenal shunt caused encephalopathy in 39.3% of the cases and distal splenorenal shunt in 14.8%. None of those submitted to esophagogastric devascularization with splenectomy developed encephalopathy. The 3 procedures showed similar rates of hemorrhagic recurrence.
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PMID:Portal hypertension in mansonic schistosomiasis. 190 30

Eleven children were identified as being seropositive for HIV-1 at the Ethio-Swedish Children's Hospital, Addis Abeba, Ethiopia between January 1988 and September 1989. The diagnosis was confirmed by both ELISA and Western blot methods performed at the National Research Institute of Health, Special Laboratory for AIDS. The mean age was 2 years and 5 months, with a range of 1 week to 10 years. There were 7 boys and 4 girls. The most common admitting diagnoses were pneumonia (5), gastroenteritis (5), marasmus (5), disseminated tuberculosis (4), and abandonment (3). One patient had extensive facial molluscum contagiosum. Symptoms at admission or during hospitalization included diarrhoea (9), failure to thrive (8), fever (7), and cough (7). Physical findings included hepatosplenomegaly (5), lymphadenopathy (3), and oral candidiasis (2). No patient with an opportunistic infection or radiographic evidence of lymphocytic interstitial pneumonitis (LIP) was identified. Five patients were classified as marasmic and 4 as underweight. Evidence suggestive of encephalopathy (developmental delay and/or microcephaly) was present in 5 patients. The VDRL was non-reactive in the 5 patients in whom it was tested. Nine children were presumed to have acquired the infection by perinatal transmission, though the passive transfer of maternal antibodies or postnatally acquired infection could not be excluded. One child was thought to have acquired the infection by blood transfusion. Three children died during their hospital stay. Paediatric HIV infection exists in Ethiopia; however, these children do not present with characteristic opportunistic infections but with signs and symptoms reflecting the most common paediatric problems seen in the country. Prevention of HIV infection in children entails the prevention of infection in women of childbearing age, counselling of infected women, and effective screening of blood products.
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PMID:Clinical and epidemiological features of HIV-1 seropositive hospitalized Ethiopian children. 206 May 7

The predominantly heterosexual transmission of human immunodeficiency virus (HIV) in Africa suggests that pediatric acquired immunodeficiency syndrome (AIDS) could develop into a significant child health problem in this region. To assist clinicians in recognizing HIV infection in African children, the clinical features of 185 children with symptomatic HIV-related disease diagnosed at the 2 central hospitals in Harare, Zimbabwe, from April 1986-July 1987 were enumerated. In this period, 185 such cases were diagnosed. 83 (47%) involved children 0-12 months of age and another 61 (35%) represented children 13-24 months old. The male/female ratio was 1.0:1.03. The most frequently recorded clinical feature (52% of cases) was generalized lymphadenopathy, with or without hepatosplenomegaly. 45% of HIV-infected children presented with respiratory symptoms and pulmonary infiltrates on chest x-ray. Failure to thrive was present in 38% of cases. Also relatively common were hepatomegaly and splenomegaly (35% and 26%, respectively). Chronic, recurrent diarrhea was present in 21%. Less frequently observed (under 10% of cases) clinical findings were maculopapular eczematoid rashes, parotid swelling, chronic suppurative otitis media, chronic mucopurulent rhinitis, meningitis, and encephalopathy. 3 main clinical modes of presentation were identified--children with failure to thrive or marasmus in association with chronic diarrhea and developmental delay, those with generalized lymphadenopathy and hepatosplenomegaly, and children who present with chronic cough with pulmonary infiltrates on chest x-ray.
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PMID:Clinical presentation of symptomatic human immuno-deficiency virus in children. 226 23

To produce concentrations of zidovudine (AZT) in plasma and cerebrospinal fluid that would provide constant inhibition of the replication of human immunodeficiency virus (HIV), we gave AZT by continuous intravenous infusion to 21 children ranging in age from 14 months to 12 years who had acquired HIV infection through transfusions or perinatally. All patients were symptomatic before AZT treatment (Class P2 of the Centers for Disease Control); 13 (62 percent) had evidence of neurodevelopmental abnormalities. The mean CD4/CD8 ratio was 0.18; 11 patients had CD4 counts below 0.2 x 10(9) per liter. We administered AZT at four dose levels: 0.5, 0.9, 1.4, and 1.8 mg per kilogram of body weight per hour. The plasma drug concentrations achieved at the respective dose levels were 1.9 +/- 0.3, 2.8 +/- 1.4, 3.1 +/- 1.1, and 4.5 +/- 1.0 microM. The steady-state cerebrospinal fluid:plasma ratio was 0.24 +/- 0.07. The only evidence of toxicity was bone marrow suppression. Transfusion was required in 14 patients because of low levels of hemoglobin (5 mmol per liter [less than 8 g per deciliter]). Dose-limiting neutropenia (less than 0.5 x 10(9) polymorphonuclear leukocytes per cubic millimeter) occurred in most patients who received doses of 1.4 mg per kilogram per hour or more. Improvement in neurodevelopmental abnormalities occurred in all 13 children who had presented with encephalopathy before treatment. Serial measurements of IQ before therapy and after three and six months of continuous therapy with AZT showed that IQ scores, including those for verbal and performance IQ, rose in these 13 patients and in 5 other children who had no detectable evidence of encephalopathy before treatment. Most patients also had increased appetite and weight, decreased lymphadenopathy and hepatosplenomegaly, decreased immunoglobulin levels, and increased numbers of CD4 cells. In some patients the improvement in the features of encephalopathy occurred despite the absence of immunologic improvement. We conclude that AZT is beneficial in children with symptomatic HIV infection, especially those with encephalopathy (which may be subclinical), and that the optimal continuous intravenous dose of AZT in children is between 0.9 and 1.4 mg per kilogram per hour.
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PMID:Effect of continuous intravenous infusion of zidovudine (AZT) in children with symptomatic HIV infection. 263 49

Eight of 13 children pre- or perinatally infected with the HIV virus subsequently developed neurological symptoms. Three children also had other nonspecific symptoms (fever, lymphadenopathy, diarrhoea, hepatosplenomegaly, failure to thrive and mucocutaneous thrush). Five children developed illnesses associated with AIDS (opportunistic infections, cachexia and lymphocytic interstitial pneumonia). The neurological abnormalities predominantly affected motor functions, only later also involving sensory ones. Motor, cognitive and language development was impaired in all eight children. A loss of developmental milestones occurred in three children with HIV encephalopathy: they have since died. In all the children the HIV infection caused symptoms within the first year, progressing more quickly in the three with encephalopathy. There were no discernible risk factors to account for the difference in the course of the disease.
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PMID:[Cerebral symptoms in pre- or perinatally HIV-infected children]. 768 71


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