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

The clinical characteristics of hepatic tuberculosis in 52 cases diagnosed pathologically were analyzed. Fever, abdominal pain and hepatomegaly were the major clinical manifestations; they were present in 96.2%, 42.3% and 88.5% of the patients respectively. The fever had no consistent pattern and the abdominal pain was usually localized to the right hypochondrium and not related to overwork. Hypergrammaglobulinaemia, elevated alkaline phosphatase level and increased ESR were noted in most of the patients (76.9%, 75.0%, 76.5% respectively). 62.5% of the 52 patients was diagnosed by percutaneous liver biopsy. Since there is no consistent clinical pattern in patients with hepatic tuberculosis, the diagnosis should be considered in patients with unexplained fever associated especially with hepatomegaly or hepatosplenomegaly, elevated alkaline phosphatase level, hypergrammaglobulinaemia and increased ESR, Liver biopsy is the most valuable method to confirm the diagnosis of hepatic tuberculosis.
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PMID:[Clinical characteristics of hepatic tuberculosis]. 760 Aug 75

A total of 592 children with clinical diagnosis of typhoid fever admitted to the Dr B. C. Roy Memorial Hospital for Children, Calcutta, India during the period between February 1990 and January 1992, were screened for Salmonella typhi by blood culture. S. typhi was isolated from 221 (37.3%) cases. The majority of the strains (92.3%) showed multi-drug resistant (MDR). They were resistant to chloramphenicol, ampicillin, tetracycline and trimethoprim-sulphamethoxazole. However, all the strains were uniformly (100%) susceptible to gentamicin, amikacin, furazolidone, norfloxacin and ciprofloxacin. Minimum inhibitory concentration of the antimicrobial agents against the resistant strains of S. typhi ranged between 200 and > 1600 micrograms/ml. Phage type 0 was most frequently encountered. The rate of isolation of S. typhi was more or less the same in all the pediatric age groups. The majority of the cases came from lower socio-economic classes with poor personal hygiene. Fever was the main presenting feature in all the cases. Other associated features of the MDR typhoid fever cases, who were uncomplicated during admission, were headache (36.0%), chill and rigor (23.2%), diarrhea (37.2%), anorexia (26.2%), vomiting (23.8%), cough (18.0%) and abdominal pain (19.8%). Hepatosplenomegaly was present in 42.4% cases. However, complications were less frequently encountered among the MDR typhoid fever cases who were uncomplicated during admission and treated as in-patients. Fourteen bacteriologically-confirmed MDR typhoid fever cases had jaundice and another 18 cases had an abnormal state of consciousness during admission. Four (2.0%) bacteriologically-confirmed MDR typhoid fever patients died during the period of observation.
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PMID:Multi-drug resistant typhoid fever in hospitalised children. Clinical, bacteriological and epidemiological profiles. 795 89

We report on the clinical presentation and diagnostic findings in 45 patients with Budd-Chiari syndrome. The diagnosis was confirmed by histology at the time of liver transplantation (n = 37) or shunt surgery (n = 8). An underlying disorder could be established only in half of the patients, oral contraceptives as predisposing factor were known in 18 cases. Clinically, abdominal pain and distension as well as hepatomegaly and ascites were most frequent findings, whereas changes in laboratory data were more or less unspecific. By use of repeated ultrasound, a definite diagnosis of a Budd-Chiari syndrome could be confirmed in all cases by obligatory demonstration of obstruction or thrombosis of at least one major liver vein. Hepatic venography revealed only one false-negative result. Celio-mesenteric arteriography plus portography, cavography and preoperative liver biopsy did not present additional diagnostic information. These techniques may contribute to treatment planning of portosystemic shunt surgery or hepatic transplantation. In conclusion, the presence of hepatosplenomegaly, ascites, abdominal pain and distension, especially in combination with a known hypercoagulable state, should alert to the possibility of a Budd-Chiari syndrome. Ultrasound is the diagnostic tool of choice. Hepatic venography should only be performed if even repeated ultrasound is not conclusive.
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PMID:The Budd-Chiari syndrome: clinical presentation and diagnostic findings in 45 patients treated by surgery. 798 58

A 21-year-old man was admitted with fever, jaundice, abdominal pain and general fatigue. Other clinical manifestations revealed liver dysfunction, hepatosplenomegaly, pancytopenia and disseminated intravascular coagulation. Anti-EB virus (EA-DR-IgG) was initially elevated on admission and was decreased after that, furthermore anti EBNA was elevated in the late period of his clinical course, which indicated primary infection or secondary alteration of EBV immunity. Bone marrow examination revealed hemophagocytosis by mature histiocytes. Therefore, he was diagnosed as Virus-associated hemophagocytic syndrome (VAHS). An arterial blood gas analysis on admission showed hypoxemia and findings of interstitial pneumonia (IP) were distinctly observed on chest CT scan. Steroid therapy was then initiated and the patient responded very well. The clinical findings and laboratory data including IP, were improved. The 20 adult cases of VAHS in Japan were clinically studied and a review of the complication of VAHS with IP was also made.
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PMID:[Virus-associated hemophagocytic syndrome with interstitial pneumonia in adults and a review of literature in Japan]. 806 21

A 34-year-old Turkish woman presented with septic fever, sweats, arthralgia, and abdominal pain. Further examination revealed generalized lymphadenopathy, hepatosplenomegaly, pancytopenia, and multiple caseous granulomas. Microbiological diagnosis revealed Brucella melitensis type 3 as causative agent. This case report demonstrates that abdominal pain can be a symptom in brucellosis, and caseous granulomas may occur. In our mobile society Brucella infection should be considered as possible differential diagnosis in patients with fever, lymphadenopathy, hepatosplenomegaly, and pancytopenia, although human brucellosis is rare in Germany and other Western countries.
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PMID:Brucellosis: differential diagnosis of acute abdominal pain. 829 Dec 80

We describe a case of peripheral T cell lymphoma that is remarkable for its fulminate course and selective targeting of both kidneys. The patient was a 6-year-old girl who was in her usual state of good health until the onset of abdominal pain and fever. She was treated for acute oliguric renal failure and visual disturbances. A renal biopsy was performed. Biopsy findings were interpreted as suggestive of a vasculitic process, and treatment was initiated for a presumptive diagnosis of Wegener's granulomatosis. The patient died 3 days following admission, and autopsy revealed extensive bilateral kidney infiltration by a peripheral T cell lymphoma. The remainder of the body was spared with the exception of mild infiltration of the pulmonary parenchyma and choroid plexus by neoplastic lymphocytes. The neoplastic nature of the disease was confirmed utilizing immunoperoxidase stains and T cell receptor gene rearrangement. Primary renal lymphoma and renal failure attributable to involvement by lymphoma are rare findings that should be considered when other more common causes of renal insufficiency have been excluded. The presenting clinical complaints are generally of short duration, nonspecific, and atypical. Most patients exhibit oliguria. Physical examination may reveal hepatosplenomegaly, lymphadenopathy, and flank and/or abdominal mass(es). Laboratory findings frequently include an elevated serum creatinine, blood urea nitrogen, lactate dehydrogenase, and a mild proteinuria. Electrolyte abnormalities are variably present. Possible radiographic findings include hypodense or hypoechoic renal lesions and diffuse bilateral renal enlargement. Although the prognosis is dismal, survival may be prolonged utilizing current treatment modalities, and rare patients may be "cured" of disease. The clinical presentation, radiological findings, and prognosis of patients with clinically evident renal involvement by non-Hodgkin's lymphoma are discussed.
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PMID:Rapidly progressive T cell lymphoma presenting as acute renal failure: case report and review of the literature. 918 23

A 42-year-old white man had headache, fever, chills, abdominal pain, nausea and vomiting, night sweats, and dark urine for 3 days before admission; he had history of a tick bite 6 weeks earlier. Progressive systemic deterioration, heralded by progressive hepatosplenomegaly and pancytopenia, occurred despite doxycycline therapy. Subsequent recovery was preceded by progressive resolution of hepatosplenomegaly. Progressive hepatosplenomegaly has not been previously reported in association with systemic monocytic ehrlichiosis.
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PMID:Systemic ehrlichiosis presenting as progressive hepatosplenomegaly. 919 48

Budd Chiari syndrome is a rare disorder resulting from occlusion of hepatic venous drainage by hepatic vein thrombosis or by a membranous web in the inferior vena cava. In western countries the commonest causes are myeloproliferative disorders and hypercoagulable states. Presentation may be acute with rapid accumulation of ascites and hepatic failure, or subacute with symptoms developing over a few months. A chronic progressive form has also been described. On presentation there is usually abdominal pain, ascites, and hepatosplenomegaly; hepatic encephalopathy is found in about a third. Noninvasive, ultrasound-Doppler is recommended in diagnosis, and has a high correlation with hepatic venography. Liver biopsy is required for therapeutic decisions. Those with advanced hepatic failure or severe fibrosis on liver biopsy are referred for hepatic transplantation. When biopsy shows only hepatic congestion and inflammatory infiltrates, portosystemic shunting is recommended. We present a 61-year-old woman with ascites and hepatosplenomegaly that had developed over the courses of a few months. Budd-Chiari syndrome with chronic myelofibrosis and congenital protein C deficiency were diagnosed. Portosystemic shunt was performed but death from sepsis followed shortly.
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PMID:[Budd-Chiari syndrome]. 933 72

Porphyrias, a group of inborn errors of heme synthesis, are classified as hepatic or erythropoietic according to clinical data and the main site of expression of the specific enzymatic defect. Hereditary coproporphyria (HC) is an acute hepatic porphyria with autosomal dominant inheritance caused by deficient activity of coproporphyrinogen III oxidase (COX). Typical clinical manifestations of the disease are acute attacks of neurological dysfunction; skin photosensitivity may also be present. We report a variant form of HC characterized by a unifying syndrome in which hematologic disorders predominate: harderoporphyria. Harderoporphyric patients exhibit jaundice, severe chronic hemolytic anemia of early onset associated with hepatosplenomegaly, and skin photosensitivity. Neither abdominal pain nor neuropsychiatric symptoms are observed. COX activity is markedly decreased. In a first harderoporphyric family, with three affected siblings, a homozygous K404E mutation has been previously characterized. In the present study, molecular investigations in a second family with neonatal hemolytic anemia and harderoporphyria revealed two heterozygous point mutations in the COX gene. One allele bore the missense mutation K404E previously described. The second allele bore an A-->G transition at the third position of the donor splice site in intron 6. This new COX gene mutation resulted in exon 6 skipping and the absence of functional protein production. In contrast with other COX gene defects that produce the classical hepatic porphyria presentation, our data suggest that the K404E substitution (either in the homozygous or compound heterozygous state associated with a mutation leading to the absence of functional mRNA or protein) is responsible for the specific hematologic clinical manifestations of harderoporphyria.
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PMID:Neonatal hemolytic anemia due to inherited harderoporphyria: clinical characteristics and molecular basis. 945 77

We report a case of a 37-year-old female who suffered from upper abdominal pain, progressive abdominal distention, shortness of breath, palpitation and pitting edema of lower legs for more than one month. Abdominal sonography showed small caliber of hepatic veins, mild hepatosplenomegaly and moderate ascites. Computed tomography of abdomen disclosed extensive thrombi in bilateral femoral veins, ovarian veins and inferior vena cava. Ascites was transudate with normal cell count. Laboratory data showed hypoalbuminemia, mild elevation of total bilirubin and iron deficiency anemia. Anti-cardiolipin antibody was positive and antinuclear antibody was negative. The histopathological features, including sinusoidal dilatation with atrophic change of adjacent hepatocytes, slight congestion and hemosiderin-like material within the cytoplasm of Kupffer cells, were compatible with the criteria of Budd-Chiari syndrome. Heparin was intravenously administered immediately to prevent further progression of thrombosis. The ascites was successfully controlled with diuretics (spironolactone and furosemide). After a two-week course of treatment, she was discharged in good condition and on warfarin anti-coagulant medication.
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PMID:Anticardiolipin antibody-related Budd-Chiari syndrome: report of a case. 951 90


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