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)

Perinatal tuberculosis is a rare disease with a high mortality rate and is difficult to diagnose. We report a case of perinatal tuberculosis diagnosed by postmortem study at the age of 3 months. An 83-day-old male infant presented with cough for 3 weeks and intermittent fever for 1 week. A focal tonic convulsion occurred on the day of admission. Physical examination revealed failure to thrive, tachypnea, and marked hepatosplenomegaly. Chest roentgenogram showed bilateral nodular alveolar-interstitial infiltrates. Abdominal computed tomography showed multiple nodules in the liver and spleen as well as lymphadenopathy in the hepatic portal hilum. Antituberculous therapies were prescribed on the second hospital day. The patient died from respiratory failure on the sixth hospital day. Mycobacterium tuberculosis was cultured from gastric aspirates and cerebrospinal fluid 4 weeks after inoculation. Postmortem examination revealed disseminated necrotizing granulomas in several organs and tissues, including the porta hepatis lymph nodes, a primary hepatic complex. M. tuberculosis infection was diagnosed in his mother based on positive findings of Mautoux test and chest roentgenogram. This case illustrates that tuberculosis, though rare, still should be considered in poor-weight-gain neonates with cough, fever, and/or hepatosplenomegaly. Careful maternal and other family contact history is essential to establishing the diagnosis.
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PMID:Perinatal tuberculosis in a three-month-old infant. 1508 46

A 5-week-old female infant with vertical HIV-1 exposure, progressive cough, and failure to thrive was given a diagnosis of bilateral diffuse nodular lung lesions. The child was without fever, leukocytosis, anemia, peripheral adenopathy, or hepatosplenomegaly, and the results of repeated blood tests for HIV-1 DNA were negative. A needle biopsy of the lungs revealed granulomatous inflammation and giant cells, with fungal organisms suggestive of Aspergillus species. A nitroblue tetrazolium dye test performed on the patient's blood specimen demonstrated absence of dye reduction, suggesting a diagnosis of chronic granulomatous disease. Further analysis revealed that the child had a deficiency of the p47(phox) component of the nicotinamide adenine dinucleotide phosphate oxidase system. Thus this child with vertical HIV-1 exposure and diffuse pulmonary nodules actually had an autosomal recessive form of chronic granulomatous disease. This case study clearly demonstrates that children with suspected HIV-1 infection might also need evaluation for primary immunodeficiency and that the clinical immunology laboratory is a powerful adjunct in coming to a correct diagnosis.
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PMID:A 5-week-old HIV-1-exposed girl with failure to thrive and diffuse nodular pulmonary infiltrates. 1510 Jun 65

A 44-year-old male presented with dizziness, blurring of vision, unproductive cough, dyspnoea, heaviness of head, fever and hepatosplenomegaly along with reticulonodular infiltration in chest x-ray. His serum showed monoclonal IgM gammopathy. Peripheral blood smear, bone marrow examination and serum protein electrophoresis suggested it was a case of Waldenstrom's macroglobulinaemia. Chest x-ray and CT scan of thorax were suggestive of pulmonary involvement in the form of interstitial lung disease.
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PMID:Hyperviscosity syndrome with pulmonary involvement. 1516 94

Disseminated cryptococcosis is a rare and often fatal disease in children. The majority of cases usually occur in individuals with defective cell-mediated immunity, most commonly due to HIV infection. The authors here in report an 8-year-old girl from Nepal who presented with fever, cough, headache, lymphadenopathy, hepatosplenomegaly and cutaneous lesions. Lymph node biopsy revealed multiple granulomas composed of histiocytes and epitheliold cells along with numerous yeast forms of cryptococcus. Cultures of CSF, sputum and urine yielded cryptococcus neoformans. Surprisingly,the immune function in terms of T-cell number, CD4 : CD8 ratio, serum immunoglobulins and HIV serology was normal. After the diagnosis of disseminated cryptococcosis was established, the patient was treated with 5-fluorocytosine (100 mg/kg/day) for initial two weeks and amphotericin B (1 mg/kg/day) for 13 weeks. Patient responded well to the treatment with disappearance of presenting symptoms, cutaneous lesions, and lymphadenopathy, though she still had hepatosplenomegaly, which also decreased. Unfortunately, she developed loss of vision in 10th week of therapy. The patient was discharged on oral fluconazole (6 mg/kg/day) and no recurrence was found during the follow-up period of more than 9 months. This is the first case of disseminated cryptococcosis with no detectable immune deficit, from India.
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PMID:Disseminated infection with Cryptococcus neoformans var neoformans in an 8 years immunocompetent girl. 1568 60

Fasciolopsiasis is a disease caused by the largest intestinal fluke, Fasciolopsis buski. The disease is endemic in the Far East and Southeast Asia. Human acquires the infection after eating raw freshwater plants contaminated with the infective metacercariae. There has been no report of fasciolopsiasis either in man or in animal in Malaysia. We are reporting the first case of fasciolopsiasis in Malaysia in a 39-year-old female farmer, a native of Sabah (East Malaysia). This patient complained of cough and fever for a duration of two weeks, associated with loss of appetite and loss of weight. She had no history of traveling overseas. Physical examination showed pallor, multiple cervical and inguinal lymph nodes and hepatosplenomegaly. Laboratory investigations showed that she had iron deficiency anemia. There was leukocytosis and a raised ESR. Lymph node biopsy revealed a caseating granuloma. Stool examination was positive for the eggs of Fasciolopsis buski. The eggs measure 140 x 72.5 microm and are operculated. In this case, the patient did not present with symptoms suggestive of any intestinal parasitic infections. Detection of Fasciolopsis buski eggs in the stool was an incidental finding. She was diagnosed as a case of disseminated tuberculosis with fasciolopsiasis and was treated with antituberculosis drugs and praziquantel, respectively.
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PMID:Fasciolopsiasis: a first case report from Malaysia. 1591 55

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

The characteristics of Epstein-Barr virus (EBV)-associated infectious mononucleosis (IM) in Chinese children are rarely reported. To evaluate the clinical presentations and risk factors for complications of EBV-associated IM in previously healthy children in Taiwan, hospitalized children with the diagnosis of IM due to EBV infection from January 1998 to December 2002 were enrolled. Patients had to fulfill the serologic criteria for the diagnosis of primary EBV infection [viral capsid antigen immunoglobulin M (IgM)-(+), viral capsid antigen IgG-(+), and anti-Epstein-Barr nuclear antigen (EBNA) antibody-(-) with exclusion of other concurrent infections or underlying diseases]. Ninety eight children were eligible, with 79% younger than 5 years old (mean, 4.0 +/- 2.3 years). The male-to-female ratio was 2:1. Nearly all patients suffered from fever (mean duration 10.3 +/- 6.0 days). Cough/rhinorrhea, tonsillopharyngitis, cervical lymphadenopathy and hepatosplenomegaly were found over half of the patients. Atypical lymphocytosis (mean, 12 +/- 13%) and elevated serum aspartate aminotransferase (AST; mean, 167 +/- 183 IU/L) and alanine aminotransferase (mean, 221 +/- 222 IU/L) were the most striking laboratory findings. Various complications, including hematologic, hepatobiliary, central nervous system, and obstructive airway problems occurred in about 20% of patients with significantly prolonged course of hospitalization. All patients recovered uneventfully under supportive and immunomodulating management. Female gender, no signs of tonsillopharyngitis, white blood cell count < or =10,000/mm3 and AST > or =150 IU/L were significant risk factors for the occurrence of complications. Clinicians should monitor such patients closely and give proper treatment to decrease possible morbidity or even mortality should complications occur.
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PMID:Epstein-Barr virus-associated infectious mononucleosis and risk factor analysis for complications in hospitalized children. 1611 72

In this study which was carried over a period of 2 years, from 2003 to 2004, 270 paediatric patients with active Tuberculosis (TB) disease attending the OPD of S.N. Medical College, Agra were screened for Human Immunodeficiency Virus (HIV)-1/2 antibodies. Of these, 23 were found to be HIV-positive. Seroprevalence of HIV infection among paediatric TB patients in Agra is 8.51% (23/270). The HIV infection was found to be significantly higher, i.e. 82.61% in male children than in female children, i.e. 17.39%. Among the age groups, which were divided into < or =1, 2-5, 6-10 and 11-15 years, maximum cases of HIV-positivity, i.e. 65.22% was observed in the age group, 2-5 years of age. Among the HIV-positive children with TB, 86.75% were of pulmonary and 13.04% were of extra-pulmonary type. Among the vaccinated children, 65.22% were found to be HIV-positive, while 34.78% of the HIV-positive children were not BCG vaccinated. HIV-positive children are more likely to suffer from prolonged fever, weight loss, failure to thrive, developmental delay, stunted growth, cough, anorexia, lethargy, lower respiratory tract infections (LRTI) and hepatosplenomegaly while HIV negative are more likely to suffer from fever, diarrhoea, lymphadenitis, pallor and LRTI. 82.60% (19/23) of these TB patients had a history of positive contact with HIV, i.e. one of the parents was HIV-infected. The mode of transmission of HIV infection among paediatric TB patients was perinatal as revealed during the counselling sessions (pre-test and post-test) of both the parents.
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PMID:Seroprevalence of HIV infection among paediatric tuberculosis patients in Agra, India: a hospital-based study. 1690 56

Visceral Leishmaniasis is a multi-organic parasitic disease caused by an intracellular protozoon named Leishmania Donovani; the mean signs are: weight loss, cough, fever, hepatosplenomegaly, adenopathy and cutaneous lesions; death without treatment is the rule. The main treatment is a conservative one. Surgical treatment is necessary for complications, especially for those intra-abdominally. We wish to present a young female patient who underwent two subsequent interventions due to an unclear diagnosis. We emphasize the difficulties in achieving a certain diagnostic, because of the rarity of disease in Romania; there are also revealed surgical aspects, which are important because of very few available data in the literature.
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PMID:[Visceral leishmaniasis--surgical aspects]. 1692 25

Brucellosis remains a major zoonotic disease worldwide. It has never been reported at King Chulalongkorn Memorial Hospital (KCMH). The authors describe the first case of brucellosis in KCMH, and also review all previous reports in Thailand. The presented case was a 52-year-old Thai man, living in Phetchabun Province, who was diagnosed with idiopathic pulmonary fibrosis two years prior to admission. He presented with prolonged fever, dry cough, weight loss of eight kg over three months, hepatosplenomegaly, and pancytopenia. Blood and bone marrow cultures grew Brucella melitensis at 72 hours of incubation. A slide agglutination (Rose Bengal) test was also positive for Brucella antibody. He had been exposed to contaminated placenta of his goats that had spontaneous abortion in the past few months before his illness. The patient was successfully treated with gentamicin, doxycycline, and rifampicin. Clinicians should have a high index of suspicion when evaluating patients presenting with prolonged fever and having an exposure risk of brucellosis.
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PMID:Brucellosis: the first case of King Chulalongkorn Memorial Hospital and review of the literature. 1704 47


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