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)

The clinical features of 13 patients with angioimmunoblastic lymphadenopathy were analyzed to determine prognostic factors and response to therapy. Eleven patients presented with sudden onset of fever, weight loss, generalized lymphadenopathy, and hepatosplenomegaly. Laboratory features included autoimmune hemolytic anemia and polyclonal hypergammaglobulinemia. Pulmonary involvement was seen in six cases and skin rash in four. Two patients had localized lymphadenopathy without systemic symptoms. Both are alive at 5.5 and 2.5 years, respectively, after diagnosis, although the latter patient has required intermittent prednisone for recurrent lymphadenopathy. An additional patient is alive on treatment for months following diagnosis. The remaining ten have died, nine of sepsis and one of cerebral hemorrhage. The immunosuppression and myelosuppression of combination chemotherapy may have hastened their deaths. An individualized, conservative treatment approach is recommended.
Cancer 1981 Dec 01
PMID:Angioimmunoblastic lymphadenopathy: clinical spectrum of disease. 729 97

Urinary neopterine levels were studied in 79 normal subjects and in 112 patients with haematological neoplasias. The mean values in 79 patients with active disease were significantly raised compared to the control group. Results obtained in 79 patients with active disease indicate that 91% had neopterine levels higher than the mean value of 79 normal individuals +3 SD. There is only a little overlap between the range of neopterine levels in cancer patients and the range in healthy subjects. No significant difference was found between the mean urinary neopterine levels of 33 patients with non-Hodgkin's or with Hodgkin's lymphoma in remission and the healthy group. Only 15% of these patients had elevated neopterine levels. The mean urinary neopterine levels correlated well with the tumor stage in patients with chronic lymphocytic leukaemia and with non-Hodgkin's disease. In patients with chronic leukaemia those without hepatosplenomegaly excreted significantly more neopterine than controls, and patients with hepatosplenomegaly significantly more than those without hepatosplenomegaly. It is concluded that urinary neopterine levels are of value for following the progression of haematological neoplasias.
Clin Chim Acta 1981 Dec 24
PMID:Urinary neopterine as marker for haematological neoplasias. 731 83

Six hundred and ninety-two cases of kala-azar were studied, all confirmed cases. Vaishali, Muzaffarpur, Samsatipur and Sitamarhi were the most affected districts of Bihar. Fever, weight loss, hepatosplenomegaly, anaemia, leucopenia and monocytes were the most commonly found features, whereas pigmentation of skin was found in only 4.48% of cases. Lymphadenopathy and voracious appetite were conspicious by their absence. Study of the incubation period of the disease showed that it may be as short as 24 h. Bone marrow study revealed hypoplastic marrow with increase in the cells of the monocytic series. Serum electrophoresis revealed reduction in albumin and rise in globulin, leading to decrease in A : G ratio. Tuberculin test was negative in the majority of the patients.
J Trop Med Hyg 1981 Dec
PMID:Kala-azar hits again. 732 Oct 74

Clinical analysis of 293 cases of cirrhosis from two moderate sized hospitals in the city of Dacca has been presented. Maximum number of cases were in the age group over 40 with 150 (51.2%) males and 19 (5.8%) females. Significant past history included viral hepatitis (21.5%), kala-azar (11.6%) and malaria (10.24%). History of alcoholism was present only in 16 (5.5%) cases. Weakness (84.3%), weight loss (72%) and anorexia (39.3%) constituted the most common symptoms. Ascites (45%), haematemesis (11.6%) and melaena (28.7%) were the next common symptoms. Hepatosplenomegaly was found in about one-third of the cases. Testicular atrophy was recorded in 41.63% cases whereas gynaecomastia was relatively less common (5.5%). Scanty body hair and white nails were present in almost equal number of cases (14.7% and 18%). The cases presented here are those with overt manifestation. Nevertheless, the clinical features are not materially different from those reported by other authors. In the absence of alcoholism, viral hepatitis is presumably the most important aetiological factor in our cases and the clinical features compare favourably with non-alcoholic cirrhosis of the western writers. Cryptogenic cirrhosis has been considered to be most common type constituting 43.7% of our cases.
Bangladesh Med Res Counc Bull 1981 Dec
PMID:Cirrhosis of liver. 734 4

A case of chronic myelomonocytic leukemia (CMML) associated with primary amyloidosis (AL) is presented. Hepatosplenomegaly, macroglossia, and xanthelasma were the major physical findings. Laboratory tests showed macrocytic anemia, thrombocytopenia, monocytosis and a bi-clonal gammopathy. Early monocytes and monoblasts were noted in the bone marrow aspiration biopsy. Cytogenetic evaluation showed a clonal deletion of chromosome 21 long arm (21q-). Amyloid was present in the liver, tongue and xanthelasma. In addition, the patient was noted to have osteosclerosis of the lower extremities. Treatment with prednisone and colchicine resulted in a subjective response. The unusual association of CMML, and primary amyloidosis is discussed.
Leuk Lymphoma 1994 Dec
PMID:Chronic myelomonocytic leukemia associated with primary amyloidosis. 769 26

A 74-year-old woman with erythropoietic protoporphyria (EPP) with hepatic dysfunction is reported. She had been photosensitive for two years prior to admission. Physical examination revealed hepatosplenomegaly and erosions on her face. Moderate increases in serum bilirubin and biliary tract enzymes were noted. Histology of the biopsied liver revealed moderate fibrosis and dilatation of bile canaliculi containing orange pigment. Marked increases in protoporphyrin in erythrocytes and feces were noted. The patient died of hepatic failure a year and a half after admission with maximum serum bilirubin of 34 mg/dl. This patient seems to be the oldest among reported EPP cases with liver dysfunction.
Intern Med 1994 Dec
PMID:Erythropoietic protoporphyria with severe cholestasis. 771 66

S. mansoni and S. japonicum complex schistosomes cause hepatosplenic and hepatointestinal schistosomiasis. The prevalence and incidence of this disease is increasing in all the endemic areas. Hepatosplenic schistosomiasis is seen in a small subset of clinically infected patients and represents a good model of intrahepatic portal hypertension characterised by a presinusoidal portal block and a well preserved liver parenchyma. Symmers' fibrosis is seen in a significant proportion of patients with high worm load. While the pathogenesis of Symmers' pipe stem fibrosis has not been well established, experimental and clinical data point to egg induced granulomata. The main consequences are presinusoidal portal hypertension, oesophageal varices and hepatosplenomegaly. The most striking symptoms are haematemesis or melena secondary to variceal and gastrointestinal bleeding. Cofactors associated with the pathogenesis include aflatoxins, malnutrition, alcoholism, hepatitis B and C virus. While stool examination is the best technique for diagnosis, a number of immunological tests though sensitive are not specific. Ultrasonography is sensitive for detection of Symmer's fibrosis. Praziquantel and oxaminiquine are drugs found to be effective in the treatment of hepatosplenic schistosomiasis. Recently beta-blockers have been found to be effective in the treatment of gastrointestinal rebleeding. Endoscopic sclerotherapy has been found to be effective for treatment of bleeding oesophageal varices. The treatment of choice for portal hypertension is oesophagogastric devascularization with splenectomy (EGDS).
Singapore Med J 1994 Dec
PMID:Tropical gastrointestinal disease: hepatosplenic schistosomiasis--pathological, clinical and treatment review. 776 89

The pharmacokinetics and therapeutic efficacy of praziquantel (Distocide; Epico, El-Asher-Men-Ramadan City, Egypt) were studied in 40 patients with schistosomiasis mansoni and various degrees of hepatic dysfunction. The patients were allocated into four groups: the first included 10 patients with simple active schistosomiasis while the other three were made up of patients with schistosomiasis associated with liver cirrhosis and splenomegaly according to Child's classification of hepatocellular function. Every patient was treated with 40 mg/kg of praziquantel as a single oral dose. The efficacy of the drug was evaluated after two months by rectal snip examination. The pharmacokinetic parameters did not differ significantly between patients with simple active schistosomiasis (group 1) and those with hepatosplenomegaly with liver involvement but without ascites and jaundice (group 2). However, as liver cell dysfunction became more evident (groups 3 and 4), pharmacokinetic parameters of praziquantel such as the half-life of elimination, the half-life of absorption, the maximum concentration, the time to maximum concentration, and the area under the concentration-time curve increased proportional to the degree of hepatic insufficiency. Linear correlations were found between each of the these parameters on the one hand and hepatic function test results (total bilirubin, direct bilirubin, and serum albumin) on the other. In spite of these pharmacokinetic differences, the cure rates were 70%, 80%, 90%, and 90% in the four groups, respectively. Although the incidence of side effects was high (53%), such effects were transient and mild.
Am J Trop Med Hyg 1994 Dec
PMID:Clinical and pharmacokinetic study of praziquantel in Egyptian schistosomiasis patients with and without liver cell failure. 781 Aug 16

A 60-year-old man presented with hepatosplenomegaly and signs of hepatic failure. The patient underwent splenectomy and liver biopsy. Histologic sections of the liver and the spleen showed nodular infiltrates composed of scattered large atypical cells that resembled Sternberg-Reed (SR) cells intermingled with abundant small T-lymphocytes. Large atypical cells showed B phenotype (CD20 positivity) and did not express CD15 and CD30 antigens typical of SR cells.
Cesk Patol 1994 Dec
PMID:[T-lymphocyte-rich large B-cell lymphoma--case report]. 785 15

Myelofibrosis with myeloid metaplasia, or agnogenic myeloid metaplasia (AMM) is a chronic myeloproliferative disorder characterized by fibrosis of the bone marrow accompanied by aniso- and poikilocytosis, leukoerythroblastosis and hepatosplenomegaly with extramedullary hematopoiesis. Agnogenic myeloid metaplasia is very rare in children. In this report, two cases of AMM in whom the onset of the illness were at 3 and 12 months of age, are presented. Both had severe anemia, hepatosplenomegaly and bone marrow fibrosis. Lymph node biopsy of the first patient and liver biopsy of the second revealed extramedullary hematopoiesis. They were treated with an intravenous high dose of methylprednisolone (daily 30 mg/kg for 3 days, 20 mg/kg for 4 days, 10 mg/kg for 1 week, 5 mg/kg for 1 week). A complete improvement of hematological and clinical findings was observed.
Acta Paediatr Jpn 1994 Dec
PMID:Agnogenic myeloid metaplasia in childhood: a report of two cases and efficiency of intravenous high dose methylprednisolone treatment. 787 86


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