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)

Acute Francisella tularensis infection in 3 domestic cats was presumptively diagnosed on the basis of clinical signs and lesions and confirmed by culturing or immunofluorescent demonstration of the organism. Clinical findings include marked signs of depression, oral/lingual ulceration, regional or generalized lymphadenomegaly, hepatosplenomegaly, panleukopenia with severe toxic change of neutrophils, and hyperbilirubinemia with bilirubinuria. Lesions found at necropsy included icterus, oropharyngeal and lingual ulceration, multiple foci of necrosis in lymph nodes, spleen, liver, and lung, and severe segmental or diffuse enterocolitis. Results of serologic testing for F tularensis was positive in only 1 of the 3 cats. The organism was cultured aerobically from several tissues, including aspirated bone marrow obtained before death in 1 cat. Results of an indirect fluorescent antibody test, performed on fresh and formalin-fixed tissues of all cats, were positive. Because of the severe clinical course, opportunity to evaluate therapeutic regimens was not possible. Until now, confirmed diagnosis of feline tularemia only has been made retrospectively, in instances when cats were suspected to have transmitted infection to human beings in whom the primary diagnosis was made. The findings in this report provide a basis for presumptive diagnosis that will help to minimize public health risk associated with this potentially fatal zoonotic disease.
J Am Vet Med Assoc 1991 Dec 01
PMID:Acute tularemia in three domestic cats. 177 44

Constrictive chronic pericarditis in a 13-year-old male patient was previously treated as chronic hepatitis for seven years, with the use of furosemide and spironolactone. Investigation for diagnosis included chest radiography, echo-doppler-cardiography, thoracocentesis with pleural biopsy and computerized tomography of chest, and showed ventricular diastolic restriction due to constrictive chronic pericarditis. After eight weeks of tuberculostatic treatment, the patient was submitted to hemodynamic study that confirmed the diagnosis and a pericardiectomy was performed. Long-term follow-up showed regression of diastolic restriction and decrease of hepatosplenomegaly and of jugular stasis. Tuberculostatic drugs were given for 12 months postoperatively, associated to corticosteroids.
Arq Bras Cardiol 1991 Dec
PMID:[Constrictive pericarditis as differential diagnosis of hepatic disease]. 182 20

Familial hemophagocytic lymphohistiocytosis (FHL) is a frequently missed and almost uniformly fatal childhood disorder. It is characterized by fever, hepatosplenomegaly, cytopenia, coagulopathy, and hypertriglyceridemia. The pathogenesis of FHL is not known but the above clinical and laboratory findings are compatible with reported in vitro and in vivo effects of several inflammatory cytokines. We measured circulating interferon-gamma (IFN-gamma), tumor necrosis factor/cachectin (TNF), and interleukin-6 (IL-6) in nine children with FHL. During active disease, elevated IFN-gamma was detected in seven of seven children, TNF in six of six, and IL-6 in two of six children studied. Thus, important inflammatory cytokines are augmented in active FHL and may contribute to the pathogenesis of the disease. Soluble CD8 was also increased in seven of seven children, which suggests a pathophysiologic importance of cytotoxic T lymphocytes. Because FHL appears to be associated with a systemic hypercytokinemia, our results also indicate that studies of FHL may contribute to the understanding of cytokine effects in vivo. Moreover, FHL is a hereditary disorder, suggesting that the hypercytokinemia is caused by a genetic defect in cytokine regulation.
Blood 1991 Dec 01
PMID:Hypercytokinemia in familial hemophagocytic lymphohistiocytosis. 195 80

A prospective study was conducted in the Bronx, New York, of 70 infants of human immunodeficiency virus (HIV)-infected (n = 33) and uninfected (n = 37) mothers who had a history of intravenous drug use or of intravenous drug-using sex partners. Infants were observed from birth to a median age of 23 months (range 3 to 54 months). HIV infection was confirmed in seven infants (21%) of seropositive mothers; six developed HIV disease, with symptoms observed in the first year. Of these, three died (3, 9, and 36 months) of HIV-related causes; 3 of 4 survivors were greater than 25 months of age. HIV symptoms preceded or were concurrent with abnormalities in T-lymphocyte subsets; postneonatal polymerase chain reaction confirmed HIV infection in five infants with symptoms and one without symptoms. Among infants of seropositive mothers, seven without laboratory evidence of HIV (including polymerase chain reaction) had findings suggestive of HIV infection, including persistent generalized lymphadenopathy, hepatosplenomegaly, oral candidiasis, parotitis, and inverted T-lymphocyte ratios. These findings were not observed in infants of seronegative mothers. Although the presence of HIV proviral sequences was associated with HIV disease, the observation of indeterminate symptoms in at-risk infants indicates the importance of long-term clinical follow-up to exclude HIV infection. Disease manifestations in comparable infants of seronegative mothers are important for assessment of the impact of maternal drug use, development of specific clinical criteria for early diagnosis of HIV and eligibility for antiretroviral therapy.
Pediatrics 1991 Dec
PMID:A prospective study of infants of human immunodeficiency virus seropositive and seronegative women with a history of intravenous drug use or of intravenous drug-using sex partners, in the Bronx, New York City. 195 45

A 31-year-old man was admitted for investigation of proteinuria and hematuria. Physical examination on admission revealed systemic lymphoadenopathy, no hepatosplenomegaly, and ankle edema. Hemoglobin was 14.3 g/dl, platelet 21.4 x 10(4)/microliters and WBC 40,800/microliters which contained 86% mature lymphoid cells. Immunological phenotyping of peripheral lymphoid cells gave positive reactions for CD19, and CD20, and negative reaction for smlg. Urinary protein excretion was 8.3 g/dl in 24h. Serum total protein was 4.1 g/dl with albumin of 2.5 g/dl. Serum IgG was 302 mg/dl, IgA 43 mg/dl, and IgM 56 mg/dl. Renal biopsy showed characteristic features of membranoproliferative glomerulonephritis (MPGN). He was diagnosed as having nephrotic syndrome associated with B-cell chronic lymphocytic leukemia (B-CLL), and was treated with prednisolone and cyclophosphamide without effect. Therefore, he was treated with 18 MU of recombinant-alpha-2a-interferon (IFN-alpha)/day. This treatment resulted in almost normal WBC and differential counts, and urinary protein excretion of 3g in 24h 2 months later. After IFN-alpha treatment was discontinued, WBC count and the amount of urinary protein again increased. He was again treated with IFN-alpha at the dose of 9.0 MU/day three times a week, and is now well without any complaints. This is the first case report in which IFN-alpha was effective in a patient with nephrotic syndrome associated with B-CLL. We think that IFN-alpha therapy is worth trying in similar cases.
Rinsho Ketsueki 1990 Dec
PMID:[Nephrotic syndrome associated with B-cell chronic lymphocytic leukemia successfully treated with interferon-alpha]. 207 29

The frequency and clinical significance of the pseudo-Chediak-Higashi (PCH) anomaly were studied in 20 children with acute myeloid leukemia (AML) M2 in the FAB nomenclature. PCH granules were recognized as giant eosinophilic granules, measuring up to 5 microns, in the cytoplasm of leukemic cells on smears. At the electron microscope level, most PCH granules were round to oval and outlined by a limiting membrane, and contained homogeneous, granular, crystalloid, rod-like or myelin-like materials. The PCH anomaly was demonstrable in five (25.0%) of the 20 patients, which indicates that the anomaly is not rare in childhood AML M2. There were no differences between PCH anomaly-positive and PCH anomaly-negative groups with regard to hepatosplenomegaly, hemoglobin levels, white blood cell counts, bone marrow cellularity, t(8q-, 21q+) chromosome abnormalities or prognoses. Circulating leukemic cells were observed less frequently in the PCH anomaly-positive group than in the PCH anomaly-negative group (p less than 0.05); the leukemic cells were not demonstrable in three of the five patients in the former group, although they were detected in all 15 patients in the latter group. The existence of PCH granules and/or a defect of the cytoskeleton responsible for the PCH anomaly in leukemic cells may impede their movement from the bone marrow to the peripheral blood.
Acta Paediatr Jpn 1990 Dec
PMID:Pseudo-Chediak-Higashi anomaly in acute myeloid leukemia (M2) of childhood. 208 66

Eight cases of spinal brucellosis are included in this study. Diagnosis was established by positive serology. Back pain was the most common complaint. Functional disturbance in walking was observed in three cases; in two others this was because of impairment of cord function. Clinical hepatosplenomegaly was found in one case. Subclinical organomegaly was diagnosed in two other patients. Psoas abscess was identified by computed tomographic scan in two separate cases. Response to drug therapy and surgical decompression, when indicated, resulted in complete recovery in all patients.
J R Coll Surg Edinb 1990 Dec
PMID:Brucellosis of the spine. 208 5

For studying the side effects of praziquantel in children with active intestinal bilharziasis 6 groups of children were followed: group P-1 (active intestinal bilharziasis +/- hepatosplenomegaly). They were treated with praziquantel (40 mg/Kg b.w. orally every 6 months). group P-2 (children with active mansoniasis +/- hepatosplenomegaly. They were treated with an initial full dose of praziquantel (40 mg/kg) to be followed by suppressive dose (20 mg/kg) at 3-months intervals, group P-3 (school children with active mansoniasis +/- hepatosplenomegaly). Initial loading praziquantel dose was followed by suppressive dose at monthly intervals, group N-1 (non-bilharzial children given an oral monthly praziquantel prophylactic dose of 20 mg/kg, group N-2 (non-bilharzial children given an oral 3-monthly praziquantel prophylactic dose of 20 mg/kg), group N-3 (non-bilharzial school children given an oral placebo in the form of vitamin B complex tablets at 3-monthly intervals. Surveillance for praziquantel adverse reactions for all these groups was done. It revealed that the adverse reactions were nausea, vomiting, abdominal colic, diarrhea, dizziness, headache and pyrexia. These were noticed more after full therapeutic praziquantel dose than half doses (subcurative or prophylactic) & among bilharzial children than non-bilharzial cases. As regards school children with active urinary hematobiasis 3 groups were followed: Group 1 (school children with active urinary hematobiasis treated with praziquantel orally 40 mg/kg b.w. every 6 months). Group 2 (non-bilharzial school children given oral monthly prophylactic dose of 20 mg/kg b.w. praziquantel). Group 3 (non-bilharzial school children given oral placebo in the form of two vitamin B-complex tablets monthly).(ABSTRACT TRUNCATED AT 250 WORDS)
J Egypt Soc Parasitol 1990 Dec
PMID:Side effects of praziquantel in bilharzial children on a field level. 212 46

We describe the clinical findings over the first 18 years of a patient with a novel phenotype for galactosialidosis, the storage disease produced by the combined deficiency of beta-galactosidase and neuraminidase. Clinical findings in the first few months included somewhat unusual appearance and hepatosplenomegaly. Dysostosis multiplex was evident by age 2 1/2 years. Mitral and aortic valvular disease appeared over the next few years and cardiac disease has become the most important clinical problem. Foam cells were present in the bone marrow, and vacuolated lymphocytes were present in the peripheral blood smear. The patient had no neurological symptoms, cherry red spots, or intellectual deterioration during the first 18 years. Evidence presented elsewhere indicates that the basic defect in this late infantile form of galactosialidosis (as is thought to be true for the other forms of galactosialidosis) is a reduced amount of the 32 kDa phosphoglycoprotein which associates with beta-galactosidase and alpha-neuraminidase in lysosomes.
Am J Med Genet 1990 Dec
PMID:Combined deficiency of beta-galactosidase and neuraminidase: natural history of the disease in the first 18 years of an American patient with late infantile onset form. 214 53

By the mid 1990s the number of newly diagnosed children with HIV infections in the US may rival the 6,500 children diagnosed with cancer each year. But recent developments in therapy for the child with AIDS offer some hope. A pediatric trial at the NCI used a continuous infusion of azidothymidine in order to achieve the desired blood and cerebrospinal fluid levels. Objective and subjective evidence of response to therapy was noted in all patients who presented with neurodevelopmental deficit. Increases in appetite and weight gain and reductions in lymphadenopathy and hepatosplenomegaly, and increases in CD4 count similar to those observed in adults were apparent. Promising preliminary results have also been seen in trials of dideoxycytidine, dideoxyinosine, and recombinant CD4.
Oncology (Williston Park) 1990 Dec
PMID:Pediatric AIDS: a perspective for the oncologist. 214 35


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