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)

Clinical course and histopathologic features of a typical case of familial hemophagocytic lymphohistiocytosis (FHLH) are presented. FHLH, initially known as familial hemophagocytic reticulosis (FHR), is rare and without proper treatment is invariably rapidly fatal, usually accompanied by fever, anorexia, vomiting, irritability and pallor. Sporadic examples with prolonged survival have been reported. Other significant findings include hepatosplenomegaly, progressive anemia, leukopenia, thrombocytopenia, hyperlipidemia and hypofibrinogenemia. Varying degrees of hemophagocytosis by widely disseminated histiocytes in different organs and structures is one hallmark of the disease. Hemophagocytosis may also occur in viral and bacterial infections and in certain malignant processes. Very high parental consanguinity in FHLH was mentioned in two relatively recent reports. A probable immunologic defect has been the focus of recent investigations. The genetic defect is believed to be transmitted as an autosomal recessive trait.
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PMID:Familial hemophagocytic lymphohistiocytosis (FHLH). 909 88

A case is reported of a 43-year-old man who presented prostatitis and hepatitis due to Brucella melitensis. His symptoms were icterus, weakness, anorexia, fever, and urinary discomfort. Physical examination revealed icterus and hepatosplenomegaly. Lymphomonocytosis, elevated erythrocyte sedimentation rate and abnormal liver functions had been detected in laboratory tests. Brucella melitensis was isolated from prostatic fluid and blood cultures.
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PMID:Prostatitis and hepatitis due to Brucella melitensis: a case report. 951 79

A 29-year-old Caucasian woman presented to hospital with a 2-day history of diarrhoea, anorexia and rigors. Investigations showed abnormal liver function tests, hyponatremia, hypoalbuminaemia and lymphopenia. The initial chest radiograph was normal. A bone marrow trephine biopsy showed non-caseating granulomata and she subsequently developed miliary shadowing on the chest radiograph. A transjugular liver biopsy confirmed the presence of acid-alcohol fast bacilli. Despite starting triple therapy for miliary tuberculosis she remained febrile and developed massive hepatosplenomegaly, jaundice and pancytopenia. Standard triple therapy was substituted with ethambutol, streptomycin and oral prednisolone and the patient made a dramatic recovery. The clinical symptoms of miliary tuberculosis are frequently non-specific and the onset of the illness is often insidious. The liver is involved in almost all patients with miliary tuberculosis, but massive hepatosplenomegaly and jaundice are rare. Standard triple-therapy should be discontinued when there is significant liver dysfunction, and corticosteroids should be considered for patients with miliary tuberculosis who fail to respond to conventional therapy.
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PMID:Massive hepatosplenomegaly, jaundice and pancytopenia in miliary tuberculosis. 957 Jun 66

We present a case of hepatosplenic sarcoidosis. A 51-year-old Japanese male, who was diagnosed to have sarcoidosis 4 years previously, was presented to our hospital because of dry cough and anorexia with weight loss. He had tender hepatosplenomegaly. A dynamic abdominal computed tomography (CT) revealed multiple small low-density areas in both liver and spleen, as well as in magnetic resonance imaging (MRI). The laparoscopic photographs showed many small whitish nodules surfacing on the liver and several tumorous nodules on the spleen. Multiple imaging modalities including dynamic CT and MRI are valuable for detecting focal hepatic and splenic lesions of sarcoidosis.
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PMID:Hepatic and splenic sarcoidosis evaluated by multiple imaging modalities. 965 99

The characteristics of two cases of histoplasmosis in AIDS patients in our institution are presented together with a review of the 11 cases published in Spain since 1988 in addition to the current knowledge on histoplasmosis in patients with human immunodeficiency virus infection (HIV). In all except 2 of the 13 patients there was epidemiologic history of a stay in a country in which histoplasmosis is endemic. The 12 cases described in which this information is available had CD4 counts under 100/microL. The clinical manifestations of presentation were fever (92.3%) associated or not with other unspecific symptoms (asthenia, anorexia, cough, diarrhea) with a subacute course of two or three months. Physical examination demonstrated hepatosplenomegaly in 76.9% of the cases and 61.5% of the patients presented cutaneous lesions. Thoracic radiography was abnormal in 55% (61.5% had respiratory symptoms). Diagnosis was achieved by isolation of the fungus in the cutaneous biopsies in all the patients with dermatologic involvement and in 7 cases identification was performed in the bone marrow. In all the cases induction treatment was with anphotericin B and in those who reached the maintenance phase itraconazol was used in 7 cases and ketoconazol in one case. None of the patients treated with itraconazol, including the two in our center, presented recurrence at the time of completion of follow up. In conclusion, histoplasmosis is frequently presented as a prolonged febrile syndrome with unspecific characteristics, thus emphasizing the importance of including travel history to other countries in the anamnesis. The increase in journeys to endemic countries and immigration from these areas had led to an increase in the number of cases of histoplasmosis in patients with HIV infection in Spain.
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PMID:[Disseminated histoplasmosis in AIDS patients. A study of 2 cases and review of the Spanish literature]. 980 81

Miliary tuberculosis infection in childhood remains a significant health problem in developing countries. To characterize the clinical features of the disease, symptoms, signs and laboratory findings, associated infections and outcomes of the 23 children (14M, 9F) with miliary tuberculosis admitted to the Dicle University Hospital, Diyarbakir, Turkey from 1990 to 1997 were analysed. Mean age of the patients was 3.7 y ranging between 6 months and 11 y. All but 3 patients were below 6 y of age, and 5 of the patients were under 1 y of age. All of the children had not received BCG vaccination except for 1 who was immunized after the age of 5 y. Only 6 children had a positive reaction to 5 TU tuberculin test at admission to hospital. Eight of the 23 subjects were infected in the family, and all were nonHIV infected cases. All of the children were diagnosed clinically and by a miliary infiltrate visible on chest roentgenogram. Five cases had positive culture results for Mycobacterium tuberculosis. Duration of onset of symptoms prior to admission ranged between 15 d and 8 months with a mean of 58 d. The most frequently seen symptoms and clinical findings were fever in 17 cases, rales in 14, loss of appetite and weight in 10 and hepatosplenomegaly in 9 cases. Two cases had prior measles infection and 1 of the patients presented facial paralysis. Seven cases had also tuberculous meningitis. The outcome in general was good, with mortality in 2 cases.
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PMID:Miliary tuberculosis in children: a clinical review. 981 15

We describe four cases of disseminated infection caused by endemic Penicillium marneffei in human immunodeficiency virus (HIV)-infected patients from the Manipur state of India. The most common clinical features observed were fever, anorexia, weight loss, hepatosplenomegaly, and, more importantly, skin lesions resembling molluscum contagiosum. The diagnosis in each of the four cases was achieved by direct examination of smears, observance of intracellular yeast-like cells multiplying by fission in biopsied tissue from skin lesions, and isolation of the dimorphic P. marneffei in pure culture in each case. In one case, fluorescent antibody studies allowed specific diagnosis. This report documents a new area in which P. marneffei is endemic, located in eastern India, and describes the first occurrence in India of P. marneffei in HIV-infected patients as well as the extension of the areas of P. marneffei endemicity westward to the northeastern state of Manipur.
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PMID:Indigenous disseminated Penicillium marneffei infection in the state of Manipur, India: report of four autochthonous cases. 1040 25

This is a case report of a 29 year old male with pneumocystis pneumonia and tuberculosis, and who was initially suspected of having HIV infection, based on risk factor analyses, but was subsequently shown to be HIV negative. The patient arrived at the hospital with fever, cough, weight loss, loss of appetite, pallor, and arthralgia. In addition, he was jaundiced and had cervical lymphadenopathy and mild heptosplenomegaly. He had interstitial infiltrates of the lung, sputum smears positive for Mycobacterium tuberculosis and Pneumocystis carinii, and stool tests were positive for Strongyloides stercoralis and Schistosoma mansoni. He was diagnosed as having AIDS, and was treated for tuberculosis, pneumocystosis, and strongyloidiasis with a good response. The patient did not receive anti-retroviral therapy, pending outcome of the HIV tests. A month later, he was re-examined and found to have worsening hepatosplenomegaly, pancytopenia, fever, and continued weight loss. At this time, it was determined that his HIV ELISA antibody tests were negative. A bone marrow aspirate was done and revealed amastigotes of leishmania, and a bone marrow culture was positive for Leishmania species. He was treated with pentavalent antimony, 20 mg daily for 20 days, with complete remission of symptoms and weight gain. This case demonstrates that immunosuppression from leishmaniasis and tuberculosis may lead to pneumocystosis, and be misdiagnosed as HIV infection. The occurrence of opportunistic infections in severely ill patients without HIV must always be considered and alternate causes of immunosuppression sought.
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PMID:Pneumocystis carinii pneumonia, pulmonary tuberculosis and visceral leishmaniasis in an adult HIV negative patient. 1150 79

Diagnosis of tuberculosis (TB) in children is usually based on presumptions from several elements: clinical picture and course, x-rays, tuberculin test, and culture of pathology later on. TB is usually found in a child because of symptoms of primary disease, or through case-finding of a contact. TB is children is often a primary infection and may be gradual or acute in onset. Some of the symptoms of primary TB are low-grade fever, pallor, fatigue, and anorexia. The child may have erythema nodosum, a yellow module on the conjunctiva, hilar or mediastinal lymphadenopathy, a primary TB complex on the lung (3-10 mm), segmental density, or a positive PPD test. Children with pulmonary disease do not have adult-type cavity lesions, but may have a primary cavity that drains into the bronchi, mechanical complications, fistulas, or atelectasis. Acute TB often appears as meningitis. The pathognomonic signs are cerebrospinal fluid high in lymphocytes with very high albumin (0.6-2 g) and low glucose (0.4-0.2 g/l). TB organisms are rarely seen, but may be cultured. TB meningitis is also notable for choroidal tubercles, which are yellow nodules visible in the fundus. These presumptive signs, as well as increasing neurological findings, prompt immediate treatment. Children also may have acute miliary TB, marked by high fever, gastrointestinal symptoms, hepatosplenomegaly, dyspnea, cyanosis, and respiratory distress, with characteristic diffuse grainy spots on the chest x-ray. A child may have both conditions and may also have localized TB infection elsewhere. Thus, clinical findings may point to possible cultures of urine, gastric lavage, pleural fluid or biopsy, pericardial fluid, bone marrow, or ascitic fluid, any of which should be cultured to rule out other causes. The most common sites for extra-pulmonary TB are cervical nodes, spine, knee. shoulder, hip and peritoneum. Pelvic and urinary tract infections are rare in children.
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PMID:Diagnosis of tuberculosis in children. 1234 39

Cutaneous sarcoidosis is rare in children. We report a case of a 5-year-old Bangladeshi girl who presented with fever, a papular eruption on the lower limbs and trunk, malaise, anorexia and weight loss. There was multisystem involvement with marked hepatosplenomegaly, generalized lymphadenopathy, parotid fullness and chronic uveitis. Pulmonary infiltrates were seen on the chest X-ray. Histology of a skin biopsy showed naked noncaseating granulomata and PCR for Mycobacterium tuberculosis was negative. A clinical diagnosis of sarcoidosis was made. The patient was treated with oral prednisolone (2 mg/kg per day). An excellent clinical response with resolution of the rash and improvement of extracutaneous signs was noted within 3 months and she remains well on low-dose prednisolone on alternate days. We discuss the presentation and management of sarcoidosis in children, and highlight the potential difficulty in differentiating this from disseminated tuberculosis.
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PMID:A case of childhood sarcoidosis. 1237 81


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