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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present the pulmonary findings in 36 autopsies of children affected by the acquired immunodeficiency syndrome (AIDS). Twenty-three patients were male and 13 were female, ranging in age between 3 days and 13 years. Twenty children had human immunodeficiency virus (HIV)-positive parents or parents who were at high risk of exposure (intravenous drug abusers and prostitutes), five had a history of transfusion, and one had a history of renal transplantation and blood transfusion. Clinically, the patients presented with recurrent infections, failure to thrive, hepatosplenomegaly, fever, cough, and/or hemoptysis. Histologically, specific infectious processes were the most common finding (75% of cases), with Pneumocystis carinii pneumonia being the most prevalent type of infection, followed by bacterial pneumonia. Neoplastic conditions and lymphoid interstitial pneumonia were less frequent (approximately 10% of cases). In addition, in approximately 10% of the cases the pulmonary findings were non-specific (ie, pulmonary edema and atelectasis) and probably unrelated to HIV infection. Our findings suggest that specific infectious conditions constitute the most common type of pulmonary pathology in children with AIDS. However, because there is a small percentage of children with nonspecific findings, a transbronchial biopsy is important for proper evaluation before institution of therapy.
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PMID:The spectrum of pathological changes in the lung in children with the acquired immunodeficiency syndrome: an autopsy study of 36 cases. 808 62

We present a case of a patient with common variable immune deficiency presenting as the Letterer-Siwe syndrome (disseminated Langerhans cell histiocytosis). To our knowledge, this is the only known patient with this association. The clinical presentation was chronic diarrhea, weight loss, recurrent infections, hepatosplenomegaly, and interstitial pneumonitis. Laboratory evaluation revealed evidence of immunodeficiency, with agammaglobulinemia and diminished number and function of T cells. The diagnosis of Langerhans cell histiocytosis was confirmed by electron microscopic examination of the lung biopsy specimen demonstrating Birbeck granules in the cells of the infiltrate. It is known that patients with the combined immunodeficiency syndrome may present as disseminated Langerhans cell histiocytosis, and the case presented demonstrates that patients with common variable immune deficiency may similarly present. It is advisable that patients newly diagnosed with Langerhans cell histiocytosis be evaluated to screen for immunodeficiency. Conversely, patients presenting with combined immunodeficiency or common variable immune deficiency may display features of disseminated Langerhans cell histiocytosis. These associations must be considered in newly diagnosed immunodeficient patients.
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PMID:A 13-month-old child with chronic diarrhea, weight loss, and tachypnea. 821 98

We describe an infant with the unusual combination of Down syndrome, congenital toxoplasmosis, and central diabetes insipidus. Hydrocephalus was documented by fetal ultrasonography at 36 weeks' gestation. He developed central diabetes insipidus as a neonate, followed by interstitial pneumonia, anemia, and hepatosplenomegaly. The patient's serum titer for Toxoplasma-specific IgM (ELISA) at 37 days after delivery was negative, but the Toxoplasma SAG1 gene was detected from the cells of the cerebrospinal fluid on the same day using the polymerase chain reaction (PCR) method. Congenital toxoplasmosis can contribute to the development of central diabetes insipidus in infants. PCR was useful in diagnosing congenital toxoplasmosis rapidly and accurately.
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PMID:Congenital toxoplasmosis complicated by central diabetes insipidus in an infant with Down syndrome. 890 49

A 65-year-old man who had an 8-year history of chronic lymphocytic leukemia was admitted to our hospital on February 19, 1998 because of high fever, dry cough, and weight loss. Laboratory data on admission included serum lactate dehydrogenase at 980 IU/l, CRP at 21.8 mg/dl, and soluble interleukin-2 receptor at 7,280 U/ml. The results of serological tests for Epstein-Barr virus (EBV) antibodies were as follows: EBV capsid antigen IgG 1:2560, EBV early antigen IgG 1:640, and EBV nuclear antigens 1:20. Computed tomography revealed diffuse interstitial pneumonia in both lungs, hepatosplenomegaly with multiple nodules, and enlarged intra-abdominal lymph nodes. In addition, Gallium-67 scintigraphy demonstrated abnormal accumulations. Although the patient initially responded well to combination chemotherapy, he eventually deteriorated and died on November 2, 1988, despite salvage chemotherapy. Postmortem needle biopsy specimens from the liver and spleen revealed diffuse proliferation of polymorphic large lymphoma cells. The lymphoma cells were positive for L-26, latent membrane protein 1, and EBV nuclear antigen, but negative for UCHL-1 and CD3, 5, 10, and 30. In situ hybridization procedures disclosed the presence of EBV-encoded small RNA in lymphoma cells. These findings suggested the possibility of association with EBV infection in some cases of Richter's syndrome.
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PMID:[Epstein-Barr virus associated Richter's syndrome accompanied by interstitial pneumonia]. 1039 Aug 89

We studied a cohort of children with the human immunodeficiency virus (HIV) infection in Barbados in order to determine the prevalence of HIV infection, the clinical course including morbidity and mortality and the magnitude of the health care and social problems. Forty-seven children were diagnosed with HIV infection during the study period. The number of HIV infected children increased from 5 during 1981-85, to 14 during 1986-90, and to 21 during the 1991-95 period. The majority (91.5%) of infections resulted from perinatal transmission. Six (12.8%) cases remained asymptomatic and 41 (87.2%) were symptomatic with 19(46.3%) presenting in infancy, while 22 (53.5%) presented post-infancy. The median age at diagnosis (class P-2) was 13 months. Generalized lymphadenopathy (47.5%), hepatosplenomegaly (40.0%), failure to thrive (27.5%), persistent recurrent diarrhoea (15.0%), oral candidiasis (37.5%), Pneumocystis carinii pneumonia (37.5%), lymphoid interstitial pneumonia (12.5%) and progressive neurological disease (10.0%) were common HIV related conditions. Two children developed non-hodgkin's lymphoma. The median age at death for 23 children was 12 months, whereas the median survival after diagnosis was 4 months. Mortality was higher among those diagnosed in infancy (73.7%) as compared to those diagnosed post-infancy (42.8%). Pneumocystis carinii pneumonia was the most common (65.2%) cause of death. Paediatric HIV infection is rising and contributes considerably to infant mortality. In this study, children took longer to be symptomatic when compared to other reports. However, once symptomatic, they died early.
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PMID:HIV infection among children in Barbados. 1078 51

We searched for trisomy 11 in acute myelogenous leukemia (AML) patients using the Japan Adult Leukemia Study Group (JALSG) AML-92 and -95 databases to clarify the clinical and hematologic features of a rare numerical chromosome abnormality. Among the sequentially registered patients of JALSG AML-92 (655 patients) and JALSG AML-95 (531 patients), chromosome findings were obtained for 1074 patients (90.6%); we found 5 patients with trisomy 11 as the sole abnormality. The patients were 4 women and 1 man with trisomy 11 AML, all aged more than 45 years (median, 52 years), with 4 M1 morphologies and 1 M2. No patients manifested hepatosplenomegaly or lymph node enlargement, and no central nervous system leukemia or extramedullary lesions were detectable. All showed positivity for CD13 (5/5), CD33 (5/5), CD34 (3/3), CD38 (2/2), and HLA-DR (5/5). Except for 1 patient, all achieved complete remission after 1 course of induction chemotherapy, but 2 relapsed after discontinuation of chemotherapy. A third case of relapse occurred during intensification of chemotherapy, and the patient underwent allogenic bone marrow transplantation but died from interstitial pneumonia.
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PMID:Trisomy 11 acute myeloid leukemia: 5 additional cases from the Japan Adult Leukemia Study Group AML-92 and AML-95 databases. 1119 13

Cytomegalovirus causes pneumonia, hepatitis, thrombocytopenia, and hemolytic anemia. Cytomegalovirus adrenalitis in premature infants, however, is rare. This report described a premature newborn who had progressively worsening hyperbilirubinemia, pancytopenia, and hepatosplenomegaly at the age of 4 days. The baby's mother had prolonged rupture of amniotic membrane for about 8 weeks. The infant received exchange blood transfusion, empiric antibiotics treatment, and mechanical ventilation. Pneumonia and sepsis developed at the age of 18 days. Serum anticytomegalovirus immunoglobulin M and urine virus culture were positive for cytomegalovirus. The baby died at the age of 22 days. Autopsy showed cytomegalovirus infection complicated with interstitial pneumonitis and pulmonary edema, subacute bronchopulmonary dysplasia with interstitial fibrosis, and adrenalitis. We concluded that the functional status of the adrenal glands in cytomegalovirus-infected premature newborns who have unexplained electrolytes imbalance, fever, diarrhea, weight loss, or hypotension should be closely followed because of the possible involvement of adrenal glands.
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PMID:Perinatal cytomegalovirus infection complicated with pneumonitis and adrenalitis in a premature infant. 1182 12

A 66-year-old man was admitted to our hospital for fever on January 19, 1998. He began showing periodic high fever in June 1997 and an increased serum LDH in August 1997. His history included surgery for esophageal cancer in 1993. On admission, the patient's body temperature was 38.5 degrees C. Physical examination was negative for lymphadenopathy, hepatosplenomegaly, and skin rash. Peripheral blood revealed a hemoglobin level of 8.6 g/dl and a platelet count of 7.9 x 10(4)/microliter. Bone marrow examination showed hypocellularity with marked histiocytic hemophagocytosis. The various bacterial cultures were negative. Serum LDH was elevated to 1,606 IU/l, and ferritin was greater than 3,000 ng/ml. Antinuclear antibodies were negative. No significant elevation of viral antibody titers including that to Epstein-Barr virus was found. Hemophagocytic syndrome (HPS) was diagnosed, but no underlying diseases was identified. The patient's condition was complicated by interstitial pneumonia and pleural effusion. gamma-globulin and pulse methylprednisolone both proved ineffective for the HPS; however, complete remission was achieved with cyclic intravenous administration of etoposide (VP-16, 150 mg/day). Interestingly, the interstitial pneumonia resolved promptly with etoposide therapy. The patient relapsed, in July 2001, exhibiting high fever, cytopenia, and marrow hemophagocytosis. His condition was ameliorated by administration of etoposide. This was a rare case of chronic and recurrent HPS of unknown etiology accompanied by interstitial pneumonia. Etoposide should be considered as a primary therapy for HPS and its complications in cases such as our patients.
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PMID:[Successful use of etoposide in an elderly patient with chronic recurrent hemophagocytic syndrome]. 1270 51

The global epidemic of HIV infection remains appalling. By 2001, there were an estimated 1.4 million HIV-infected children, with 4.5 million deaths. In the UK, paediatric cases are clustered around population centres where there are high concentrations of infected immigrant adults, and to a lesser extent, areas where IV drug abuse is common. The highest incidence remains in London and the southeast. With the national redistribution of immigrant and refugee families, any doctor in any specialty may expect to be involved with children who are HIV positive, or have clinical AIDS. The majority of children are infected vertically, i.e. infection of the infant from an infected mother in the pre-, peri-, or post-natal periods. Rates of transmission vary from 15-20% in the developed countries. Children with HIV infection may have their primary presentation to ENT doctors, who should have appropriate thresholds for suspecting the diagnosis. The most common presenting features include persistent generalised lymphadenopathy, hepatosplenomegaly, chronic/recurrent diarrhoea, poor growth, and fever. Fifteen to twenty percent of untreated children will present with an AIDS-defining illness by 12 months, typically with Pneumocystis pneumonia at approximately 3-4 months of age. Seventy percent of perinatally infected children will exhibit some signs or symptoms by 12 months Without treatment, the median age to progression to AIDS is approximately 6 years, and 25-30% will have died by this age. The median age of death is approximately 9 years. Children may also present with repeated/unusual ear infections, sinus disease (inc. mastoiditis), tonsillitis, orbital/peri-orbital cellulitis, oral candidiasis, and dental infections. Infections with streptococcus pneumoniae and group A streptococcus are common, and often progress to severe systemic infection with an appreciable mortality. Infections may be due to unusual pathogens such as Pseudomonas, 'typical' and atypical Mycobacteria, Candida, Aspergillus, etc. Fungal infections of the sinuses (inc. Aspergillus and Rhizopus spp.) may be particularly devastating, with rapid spread to involve bone and the central nervous system. Another classical presentation, which may present to ENT doctors, is that of bilateral parotid enlargement, especially in children who are 'slow progressors', many of whom also have Lymphoid Interstitial Pneumonitis (LIP). A major attitudinal change has occurred due to advances in 3 main areas: (i) the multidisciplinary management of the infected mother (inc. counselling, antenatal screening, elective caesarean section, advising against breast feeding, etc.), (ii) the prevention of vertical transmission, using anti-retroviral therapy to the infected mother during pregnancy, and to the potentially infected infant in the first weeks of life, and (iii) major advances due to the advent of highly active anti-retroviral treatment. With effective use of these measures, transmission rates may be reduced to <2%. None of the measures though, affect a cure, and it will still be many years before the development of effective vaccines. ENT doctors may be referred children already known to be HIV-positive. Knowing how to talk to infected children (and their parents) is full of potential pitfalls, and requires careful forethought. Many infection-control policies have required considerable rethinking due to the AIDS epidemic. This has especially been the case with respect to needle-stick injuries, post-exposure prophylaxis, sterilization and re-use of equipment, and safe approaches to surgery.
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PMID:HIV infection in children--impact upon ENT doctors. 1466 74

The aim of the present study was to report clinical, radiological and bronchoalveolar lavage (BAL) findings in patients with pulmonary manifestations of HIV-associated multicentric Castleman's disease (MCD). This was a retrospective study of 12 patients with histologically proven MCD. Clinical manifestations were as follows: dyspnoea (nine out of 12 cases), cough (n = 10), bilateral crackles (n = 10), together with high fever, malaise, peripheral lymphadenopathy (n = 12), and hepatosplenomegaly (n = 10). Two patients developed acute respiratory distress syndrome. Chest radiographs and computed tomography scans showed reticular (n = 7) and/or nodular (n = 7) interstitial patterns, with mediastinal lymphadenopathy (n = 9), and bilateral pleural effusion (n = 3). Fibreoptic endoscopy was normal in all cases. BAL analysis showed hypercellularity (n = 6) and/or lymphocytosis (n = 6), and human herpesvirus-8 DNA was detected in two out of two cases. Specific stains and cultures for pathogens were negative. All patients received etoposide and/or vinblastine, and improved after 2-4 days. Relapses were frequent (50 attacks in 12 patients). Six patients developed a non-Hodgkin's lymphoma, and five died. In conclusion, the pulmonary manifestation of HIV-related multicentric Castleman's disease is an acute reticulo-nodular interstitial pneumonitis, associated with severe systemic symptoms and peripheral lymphadenopathy. In bronchoalveolar lavage fluid, cellularity is not specific and human herpesvirus-8 DNA is detected. The clinical course is specific due to a rapid onset and regression, frequent relapses and a high occurrence of non-Hodgkin's lymphoma.
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PMID:Pulmonary manifestations of multicentric Castleman's disease in HIV infection: a clinical, biological and radiological study. 1599 98


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