Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Eighty previously untreated patients with B-cell chronic lymphocytic leukemia (B-CLL) were analyzed to study the proliferation rate of their peripheral blood (PB) leukocytes to determine its relationship with the extension of the disease and its value in discriminating among patients with similar tumor cell mass. The 80 B-CLL patients were distributed into two different groups according to the absolute count of PB S-phase leukocytes: a low proliferative group (less than 1 x 10(9)/I) of 48 patients and a high proliferative group (greater than or equal to 1 x 10(9)/I) of 32 patients. The high proliferative group displayed a higher incidence of splenomegaly (p less than 0.005), hepatomegaly (p less than 0.08), anemia (p less than 0.02) and thrombocytopenia (p less than 0.03) as well as a higher lymphocytic infiltration both in PB (p less than 0.0004) and in bone marrow (BM) (p less than 0.003). These patients also showed a higher incidence of a diffuse pattern of BM involvement (p less than 0.04), advanced clinical stages [stage III/IV (p less than 0.03) and group C (p less than 0.04)] and infections (p less than 0.0008) together with significantly lower IgG (p less than 0.03) and IgM (p less than 0.03) serum levels. Regarding the immunophenotype, there was a greater percentage of either CD19+ (p less than 0.06) and CD19+ CD5+ (p less than 0.05) B-cells, together with a greater reactivity for both the CD25 (p less than 0.04) and CD9 (p less than 0.08) antigens in the high proliferative group. According to the prognostic value of the PB S-phase leukocyte count it was seen that patients with low S-phase white blood cell (WBC) numbers displayed a significantly higher survival (p less than 0.03). In addition, multivariate analysis revealed that the S-phase WBC count, although partially related to other clinical and biological prognostic factors, displayed an important independent value in predicting early deaths in patients with B-CLL.
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PMID:Prognostic value of S-phase white blood cell count in B-cell chronic lymphocytic leukemia. 173 13

A review was carried out of 187 previously untreated cases of chronic lymphocytic leukaemia diagnosed between 1969 and 1988. The median age of the patients was 65 years (range, 36-87). There were 118 males (M/F ratio, 1.7). In accordance with Rai's staging system the patients were distributed as follows: 0.29%; I, 20%; II, 25%; III, 13%; IV, 13%, and according to Binet's staging the distribution was: A, 55%; B, 21%; C, 24%. The most frequently found physical findings were lymph node enlargement (55%), splenomegaly (32%) and hepatomegaly (28%). Anaemia was present in 20% of the cases and thrombocytopenia in 13%. The mean lymphocyte count was 62 x 10(9)/L (range, 6-475 x 10(9)/L). Bone marrow infiltration of over 80% was seen in 46% of the patients. Bone marrow biopsy was performed on 97 patients, the diffuse pattern of involvement being most commonly found (44%). Increased BUN (55%), alkaline phosphatase (42%) and LDH (38%) were the most frequent biochemical alterations. Hypogammaglobulinaemia was present in 55% of the patients, IgM being the most commonly affected immunoglobulin (66%). Monoclonal gammopathy was seen in 4% of the cases. LDT, measured in 75 patients, was less than a year in 32%. No antileukaemic drugs were needed in 34% of the patients. When concluding this study, 100 patients had died. The median survival was 57 months and death was related to chronic lymphocytic leukaemia in 53% of such patients.
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PMID:[Chronic lymphatic leukemia. I. Clinico-biological features and survival analysis. Study of 187 patients]. 177 6

The Wiskott-Aldrich Syndrome (WAS) is a rare X-linked immunohematological disorder characterized by eczema, profound thrombocytopenia, and progressive immunodeficiency. Severe hemorrhage, overwhelming sepsis, or lymphoreticular malignancy usually cause death in childhood. Recently, bone marrow transplantation (BMT) has been curative in some well-established cases, but there is no general agreement about the place of BMT in infants with WAS before the development of significant immunological abnormalities. We describe the successful use of early histocompatible BMT in a 10-month-old infant in whom WAS was diagnosed on the basis of eczema, thrombocytopenia, small platelets, and raised serum immunoglobulin A (Ig) and IgE, but before the development of immunodeficiency as evidenced clinically by recurrent infections, or immunologically by low serum IgM or consistently abnormal lymphocyte responses to mitogens. After an unstable period for several weeks posttransplantation when he developed marked hepatomegaly and severe interstitial pneumonitis, he made a good recovery. His eczema and thrombocytopenia resolved and he has shown no clinical or laboratory evidence of immunodeficiency. It is now over 2 years since his BMT. Because of the poor prognosis of WAS, where a histocompatible donor is available, BMT at the earliest opportunity, despite the inherent risks of such a procedure, may be the best option for an infant with WAS.
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PMID:Early bone marrow transplantation in an infant with Wiskott-Aldrich syndrome. 179 57

During the dengue 3 epidemic which occurred in French Polynesia between September 1989 and March 1990, number of cases of Dengue Haemorrhagic Fever (DHF) were reported among children and infants. Of interest, five cases of neonatal infections were recorded and involved the transmission of the virus from mother to child as the end of the pregnancy. Detailed observations on these cases are presented in this paper. Fever in mother, when delivering or just a few days before, represents the circumstances of the diagnosis. Analysis of the clinical features shows that fever and vasomotor troubles (blotches) were the dominating traits of the disease in the newborns. Also, hepatomegaly was constantly observed. Concerning the biological results, thrombopenia (platelets count: 100,000/mm3) was reported for all cases. According to these data, all cases were classified into DHF cases. Laboratory confirmation was obtained by serological investigations (antidengue IgM immunocapture ELISA and Haemagglutination Inhibition Tests) and/or virus isolation in both mother and child. Thus, virus was recovered in two newborns while IgM were detected in all infants and in four mothers. Severity of the disease was variable. However, out of five cases one fulfills the WHO definition of Dengue Shock Syndrome (DSC). Moreover, the authors discuss about the difficulties encountered to classify the cases according to WHO criteria for the grade classification of DHF.
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PMID:[Materno-fetal dengue. Apropos of 5 cases observed during the epidemic in Tahiti (1989)]. 181 1

The clinicopathological features of five fatal cases of diffuse haemangiomatosis presenting in neonatal life or early infancy are presented. The infants all had multiple skin haemangiomas as well as deep-seated lesions in many different tissues that caused protean clinical manifestations and management problems. Because the outlook may be improved by early diagnosis and application of new modes of treatment, any infant with multiple cutaneous haemangiomas should be closely assessed for possible visceral involvement. Development of hepatomegaly, high-output cardiac failure, unexplained anaemia or thrombocytopenia in these infants should immediately suggest disseminated disease. Early recognition with implementation of steroid and/or antiangiogenic therapy, embolization and/or surgery is essential to improve the chances of survival.
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PMID:Diffuse infantile haemangiomatosis: clinicopathological features and management problems in five fatal cases. 139

Budd-Chiari syndrome is an unusual form of portal hypertension, resulting from partial or total obstruction of the major hepatic veins or of inferior vena cava. This is illustrated by the clinical history of a two year old girl admitted by anemia, hepatomegaly, thrombocytopenia, pleural effusion, ascites and ultrasonographic, scintigraphic and angiographic evidence os suprahepatic veins and inferior vena cava obstruction. Liver biopsy showed signs of chronic congestion and fibrosis. Initial manifestation of illness may be acute or, as in this patient, with an insidious onset of symptoms. Medical therapy gives usually poor results. Several surgical approaches have been tried to alleviate liver congestion, which in this case was intended by a 6 mm wide Goretex ringed tube prosthesis extending from superior mesenteric vein throughout right atrium. Patency of the artificial shunt was proven after several days by Doppler ultrasound but the patient died of pneumonia two months after operation.
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PMID:[Budd-Chiari syndrome]. 184 28

We have observed hepatopathy, associated with thrombocytopenia, in children receiving chemotherapy for Wilms' tumor. We have studied this hepatopathy-thrombocytopenia syndrome (HTS) in patients enrolled in the United Kingdom Childrens' Cancer Study Group (UKCCSG) Wilms' tumor trials (UKW1 and UKW2). At the time of this study, 501 patients had completed therapy. Treatment flow sheets were examined for evidence of hepatopathy (hepatomegaly with abnormal liver function tests) and severe thrombocytopenia (platelet count less than 25 x 10(9)/L). No child who developed the syndrome had received irradiation. HTS was seen in five of 355 (1.4%) of patients treated with combination chemotherapy but in none of the 146 patients treated with vincristine alone. In each instance, the onset was less than 10 weeks after diagnosis. In two children, hepatopathy was severe with jaundice, ascites, transaminases greater than 1,000 IU/L, and prolongation of prothrombin time. On average, HTS lasted 12 days, and resolved with supportive treatment. After recovery, the children tolerated chemotherapy, mostly at reduced dosage, without recurrence. There was no evident long-term morbidity. Dactinomycin is the probable cause of this syndrome. We conclude that the HTS is a rare but important complication of dactinomycin-containing combination chemotherapy for Wilms' tumor. Children developing "isolated" thrombocytopenia following dactinomycin are "at risk" of developing the full-blown syndrome and should have their treatment modified accordingly.
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PMID:Hepatopathy-thrombocytopenia syndrome--a complication of dactinomycin therapy for Wilms' tumor: a report from the United Kingdom Childrens Cancer Study Group. 184 5

Seventy children from 7 months to 15 years old have been treated for malaria at Hospital Trousseau (Paris) during years 1987 and 1988. Thirty nine of them were living in France usually. The infection was one chiefly in Africa (68 cases), and by P. falciparum in 78% of children. The digestive symptoms were frequent (40/70); splenomegaly was observed in 40 children and hepatomegaly in 31. Anemia was present in 59 cases and mild thrombopenia for 31 cases. The C. reactive protein raised in 92% of cases. The diagnosis was late in 31 patients. Only one cerebral malaria case was observed. The chemoprophylaxis was unfitted or absent in 74% of children living in Paris. The chloroquino-resistance was clinically present in 17 cases and the mefloquine was more often used during 1988 year.
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PMID:[Malaria of importation in the child: epidemiological, clinical and therapeutic analysis. Apropos of 70 cases observed in a pediatric hospital in Paris]. 191 46

Four newborns with adenovirus infection are described, and the profile of neonatal adenovirus disease is outlined based on the cases of these newborns and nine previously described. Characteristic historical features included prolonged rupture of membranes, maternal illness, vaginal delivery, and onset of illness within the first 10 days of life. Clinical findings included lethargy, fever or hypothermia, anorexia, apnea, hepatomegaly, bleeding, and progressive pneumonia. Thrombocytopenia, coagulopathy, and hepatitis were typical laboratory manifestations. Illness was severe and generally unremitting; only two survivors have been reported. Pathologic changes were prominent in lung, liver, and brain. Virus isolates, predominantly serotypes 3, 7, 21, and 30 were obtained from multiple sites and organs. Epidemiologic evidence suggests that viral acquisition from the mother, perhaps via the birth canal, is a major mode of transmission. Neonatal adenovirus infection, which is frequently disseminated and generally fatal, should be considered in the differential diagnosis of neonatal sepsis and pneumonia.
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PMID:Neonatal adenovirus infection: four patients and review of the literature. 203 95

A series of 27 patients with myelodysplastic syndrome (MDS) was analysed to determine the clinical and pathologic features, the natural history and the prognostic factors. The clinical features of these patients included the following: mean age 53.77 years; symptoms due to cytopenias 100 per cent; hepatomegaly 44.5 per cent; splenomegaly 11 per cent. Almost all patients with MDS presented anemia; additional cytopenias were present in many patients. The bone marrow was hypocellular in 1/3 of cases. The patients have been classified according to the French-American-British (FAB) Group criteria: there were 11 patients with refractory anemia, 11 patients with refractory anemia with sideroblasts and 5 patients with refractory anemia with excess of blasts. The mean survival was 19 months, 9 patients died: death was due to infection or hemorrhage in 7 cases and to evolution to acute myelogenous leukemia (AML) in 2 cases (10.5%). Thrombocytopenia, the Bournemouth scoring system and FAB classification were independent prognostic factors.
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PMID:[Myelodysplastic syndromes. A clinico-biological analysis of 27 cases]. 207 37


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