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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Brucellosis has always been an unusual disease in children and, concomitant with the control of the disease in domestic animals, reports have become sparse. The pediatrician, therefore, may not be aware of the protean clinical manifestations of childhood brucellosis. In 1973, nine cases occurred during a three-month period in El Paso, Texas. All cases were marked by spiking fevers and lethargy of four days to four weeks in duration. Tender hepatomegaly or splenomegaly was striking in seven patients. Other characteristics included epistaxis, arthralgia, myalgia, and weight loss. Leukopenia and leukemoid reaction were found in five patients. All of the patients tested had elevated liver enzymes. Febrile agglutinins were invaluable in screening for an early clue to diagnosis. When Brucella abortus antigen agglutinated serum from patients with a positive screen in dilutions greater than 1:320, a presumptive diagnosis of brucellosis was made. Brucella was isolated from the blood or bone marrow in seven patients and the time of incubation proved crucial for successful recovery. Bacterial blood cultures are usually discarded at ten days of age, as were cultures from the only two patients from whom the organism was not recovered. All of the cultures incubated for 12 to 15 days grew B. melitensis, an unusual causative species in the United States. However, several patients admitted eating cheese from the State of Chihuahua, Mexico, made from unpasteurized goat's milk, the presumed source of the infection. Within one to three days, all patients responded dramatically to antibiotics; tetracycline was given orally for 21 days and streptomycin intramuscularly for 14 days. Pediatricians caring for patients in areas where consumption of unpasteurized milk products is likely would do well to consider brucellosis in a child with obscure fever or toxic hepatosplenomegaly.
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PMID:Brucellosis in childhood. 80 83

We report the findings in 21 Belgian patients (12 males and 9 females, median age 61 years) with LGLPD. Symptoms at presentation included infection (n = 9), weight loss (n = 5), asthenia (n = 9), pruritus (n = 2) and arthralgia (n = 7). Four patients were asymptomatic. The main clinical findings were hepatomegaly (n = 5), splenomegaly (n = 8), lymph node enlargement (n = 3) and arthritis (n = 5). All patients had an increased LGL count associated with anemia (n = 12), neutropenia (n = 17), often less than 0.5.10(9)/L (n = 10) and thrombocytopenia (n = 6). Three patterns of lymphocyte surface markers were observed: CD3+CD4-8+ (14 patients), CD3+CD4-8+ (5 patients) and CD3+CD4+8- (1 patient). An abnormal karyotype was found in 2 patients. T-cell receptor gene was rearranged in all cases tested (9/9).
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PMID:Large granular lymphocyte proliferative disease: 21 Belgian cases and review of the literature. 131 80

One-hundred-and-fifty-seven children admitted with brucellosis at Abha, Saudi Arabia, were studied prospectively. Ninety-two per cent gave a history of animal contact, usually with sheep or goats, or ingesting raw milk, milk products, or raw liver. Three-quarters of the patients had an acute or subacute presentation with diverse symptomatology: fever (100 per cent), malaise (91 per cent), anorexia (68 per cent), cough (20 per cent), abdominal symptoms (20 per cent), arthralgia (25 per cent). Hepatomegaly (31 per cent), splenomegaly (55 per cent), and lymphadenopathy (18 per cent) were common findings. Organ complications were rare except for arthritis (36 per cent) which usually presented as a peripheral oligoarthritis involving the hips and knees. All patients had significant agglutination titres; B. melitensis was grown from the blood in 7 of 16 (44 per cent) patients. Haematological variations were common, but non-specific: anaemia (64 per cent), thrombocytopenia (28 per cent), leucopenia (38 per cent), leucocytosis (12 per cent), and elevated erythrocyte sedimentation rate (81 per cent). Varying combinations of rifampicin, co-trimoxazole, tetracycline, and streptomycin resulted in a prompt pyrexial response (mean: 3.8 days), and a slower response in the arthropathy and hepatosplenomegaly. Relapses were related to poor compliance, use of a single drug or a shorter duration of chemotherapy. Brucellosis is a common childhood problem in southwestern Saudi Arabia as in other parts of the country and the Middle East. It should be considered in every child from an endemic area presenting with a febrile illness and a history of animal contact.
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PMID:Childhood brucellosis in southwestern Saudi Arabia: a 5-year experience. 152 11

Sarcoidosis is being increasingly recognised in Kuwait. Twenty patients were studied over three years and the clinical, biochemical and radiological data were analysed. The clinical profile revealed thoracic involvement in all the patients as well as constitutional symptoms (50%), arthralgia (55%), arthritis (15%), chest infection (35%), tuberculosis (10%), hypercalcaemia (5%), angina (15%) and hypertension (20%). None had central nervous system manifestations. Other clinical signs were erythema nodosum (25%), hepatomegaly (30%) splenomegaly (15%) and chest signs (25%), together with salivary gland (15%), skin (15%), eye (15%), and cardiac involvement (5%). The tuberculin test was negative in all those tested. The patients were classified radiologically into stage I (55%), stage II (40%) and stage III (5%) of the disease. The clinical profile was similar to the Western pattern of the disease, but there were several differences including an older age group, more frequent constitutional symptoms, the rarity of ocular and central nervous system involvement, and initial presentation as a chest infection. Therapy with steroids alone or steroids and azathioprine was used when appropriate and the response to therapy monitored.
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PMID:Sarcoidosis in Arabs: the clinical profile of 20 patients and review of the literature. 166 42

The present paper describes the clinical and laboratory follow-up of 11 patients with the diagnosis of common variable immunodeficiency. Their age varied from 8 to 45 years. The mean disease time was 12.6 years and mean diagnosis time 4.3 years. Infectious manifestations, mainly of the respiratory and digestive tracts, occurred in all patients. Polyadenomegaly was noted in seven, hepatomegaly in six, splenomegaly in five and arthralgia in four patients. All of them presented serum IgG less than 250 mg/dl. IgA less than 33 mg/dl and IgM less than 31 mg/dl, except one with IgM = 176 mg/dl. The isohaemagglutinin titers were less than 1/20 in all but one patient. The determination of the number of B lymphocytes in the peripheral blood revealed normal counts in three, elevated in one and decreased in five patients. The CD-4/CD-8 ratio was less than 1 in 8 and greater than 1 in three of them. Five patients had positive cutaneous late reactions to at least one of the following antigens: PPD, SK-SD (Varidase), Trichophytin and Levedurin (Candidin). A decrease of the proliferative activity of peripheral blood mononuclear cells stimulated by lectins (PHA, Con-A, PWM) was also noted. Natural killer function was decreased. The association a possible role of regulatory lymphocytes in the immunopathogenesis of this disease. The data presented here emphasize the diversity of clinical and immunological manifestations of this disease, which could be noted between diverse patients and in the follow-up of a single one. In our cases the disease had an evolutive character, with a primarily humoral dysfunction followed by cellular immunity disturbances that determined poorer prognosis and progressive difficulties in the therapeutics. We suggest a conceptual reevaluation of this condition and a new denomination, for instance "Late-Onset Combined Immunodeficiency". The long delay between the initial clinical manifestations of the disease and its diagnosis was a handicap for an adequate treatment. Early intervention could certainly decrease the morbidity and mortality of the disease.
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PMID:[Common variable immunodeficiency (hypogammaglobulinemia of late onset or acquired hypogammaglobulinemia): initial follow-up of 11 cases]. 172 73

In 1980, a 38-year-old man had remittent fever, swelling and arthralgia of the knee, ankle and wrist joints, as well as visual disturbance due to bilateral iritis. On his admission to our hospital, his laboratory data showed neutrophilia, normocytic normochromic anemia, hepatomegaly, hepatocellular damage, and a strongly positive RA test. All the microbiological examinations were negative. Thirty mg of prednisolone improved his symptoms and abnormal laboratory findings. Due to persistent mild arthralgia, he had continued to take 5-10 mg of prednisolone and analgesics until 1985 when he was readmitted to our hospital. In 1983, he began to complain of a steady pain around his left eye, and he sometimes had double vision. In 1985, he began to complain of decreasing left visual acuity and sensory disturbance in his left face in addition to pain in and around the eye. On his 2nd admission to our hospital, the neurological examination revealed involvement of the 2nd and 3rd cranial nerves and the 1st branch of the 5th cranial nerves of the left side. Laboratory data showed a positive RA test with RAHA titer at 1:320 and IgM at 216 mg/dl, but he had no joint deformities. The computed tomography (CT) of the brain demonstrated a high density mass of his left cavernous sinus extending to the left orbital apex. The prescription of the high dose of prednisolone (100 mg/day) relieved ophthalmic pain and improved visual acuity and neurological involvement within a week. Prednisolone was then gradually decreased to 10 mg. In 1986, he had partial and complex partial seizures.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Intracranial multiple granuloma preceded by rheumatic disease--a case report]. 224 32

A patient is described who presented with a 1-month history of daily fever to 38.8 degrees C. There was no sign of joint pain or swelling and no skin rash. The patient had impressive hepatomegaly without splenomegaly. The only abnormal laboratory test was a sedimentation rate of 120 mm/hr. Ultrasound examination showed hypoechoic foci throughout the liver. These foci were confirmed by CT scan, which showed multiple well-marginated lesions of decreased attenuation and variable size throughout the right and left lobes of the liver. A liver biopsy specimen showed large nodules that were yellow and gritty in texture. Microscopic examination of biopsy specimens of these nodules showed extensive areas of necrotizing granulomatous inflammation with palisading histiocytes and occasional giant cells surrounded by necrotic foci. There was an associated fibroinflammatory infiltrate. The patient was treated with a nonsteroidal anti-inflammatory agent with prompt cessation of fever. A repeat CT examination of the liver after 14 months of treatment showed only mild hepatomegaly and a normal liver parenchyma. The focal lesions had disappeared. This is a case of hepatic granulomata in a child showing features of necrotizing inflammation.
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PMID:Hepatic granulomata. Presenting with prolonged fever. Resolution with anti-inflammatory treatment. 236 43

A 20-year-old man was admitted to our hospital because of fever and knee joint pain on March 20, 1986. Physical examination revealed generalized lymphadenopathy and hepatomegaly. White blood cell count was 32,800 microliters with 74.4% blast cells. Bone marrow was hypercellular with 93.6% blast cells. Blast cells were weakly positive for acid phosphatase and PAS stainings but were negative for peroxidase, sudan black B and esterase stainings. Cell surface marker analysis of blast cells disclosed that they were positive for anti-HLA-DR, CD19, CD24, CD33 and CD38, but were negative for CD10 and CD20. Cytoplasmic immunoglobulin of blast cells was negative and TdT activity by immunofluorescent method was positive. Chromosomal analysis of bone marrow samples revealed normal karyotype. Therefore, this case was diagnosed as having acute lymphoblastic leukemia (L2) and achieved complete remission with LVP therapy consisting of 1-asparaginase, vincristine and prednisolone. Gene analysis of blast cells disclosed germ-line configuration of both the immunoglobulin heavy chain gene and T cell receptor beta chain gene. We speculated that the phenotype of leukemic cells might precede the genotype in some cases of acute leukemia.
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PMID:[Germ-line configuration of the immunoglobulin heavy chain gene in a case of B cell precursor acute lymphoblastic leukemia]. 255 12

Mediterranean Spotted Fever is a disease caused by Rickettsia conorii. It is endemic to the Mediterranean area, where, for the last few years, the number of cases has increased, possibly due, in part, to climatic factors. The main clinical aspects of a prospective series of 246 cases diagnosed from 1983 to 1988 are presented. The most characteristic manifestations were fever, exanthema and tache noire. Other frequent manifestations were headache, myalgia and arthralgia, and with lesser frequency, hepatomegaly, splenomegaly, gastrointestinal symptoms and conjunctivitis. Notable analytical changes are the rise of hepatic and muscular enzymes in a large number of patients. In some cases we have found signs of myositis in muscular biopsy. The evolution of our patients was usually favorable though serious and even deadly forms of the disease have been described. One of the factors that seems to greatly influence the appearance of these forms is delay in the initiation of effective treatment.
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PMID:New trends in Mediterranean spotted fever. 269 Dec 73

Spontaneous improvement of active juvenile rheumatoid arthritis (JRA) occurred after T lymphocytosis in an 8-year-old boy. He had prominent lymphocytosis, the count reaching 59,000/mm3, followed by spontaneous disappearance of fever, arthralgia, lymphadenopathy, hepatomegaly, and C-reactive protein. The serum immunoglobulin levels were gradually decreased. The surface marker analysis, using two color flow cytometry, showed that the lymphocytes were activated suppressor T lymphocytes, expressing CD3, CD8, HLA-DR, and CD8 plus CD11. When studied in vitro with pokeweed mitogen stimulation, the T lymphocytes significantly suppressed the immunoglobulin production by autologous B lymphocytes as compared with the T lymphocytes at remission (p less than 0.01). Based on the widely believed notion that depression of suppressor T lymphocyte functions is one of the important mechanisms underlying systemic JRA, the activated T lymphocytosis with the suppressor phenotype and suppressive function on the immunoglobulin production may have been related to the improvement of active JRA in the patient.
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PMID:Spontaneous improvement of juvenile rheumatoid arthritis after T lymphocytosis with suppressor phenotype and function. 297 87


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