Biomedical subjects
Publications and source records attributed to W Muntean.
A previously healthy 9-year-old boy died from acute liver failure during an acute Epstein-Barr virus infection. Epstein-Barr virus DNA could be demonstrated in the liver by Southern blot--and by in situ hybridization techniques. The identification of the virus in the liver suggests a causal relation between the Epstein-Barr virus and the acute massive liver cell necrosis.
Thirteen children with ALL and L-asp-induced alterations of the coagulation system were treated with fresh-frozen plasma and antithrombin III (AT III) concentrate. Fresh-frozen plasma was given three times daily to maintain fibrinogen levels greater than 100 mg/dl. AT III concentrate was administered in a continuous infusion over 24 h as long as replacement with fresh-frozen plasma was given. When fibrinogen was greater than 100 mg/dl and AT III less than 80% of normal, only AT III concentrate was administered in a continuous infusion. In all patients treated with the replacement regimen described, fibrinogen levels were maintained greater than 100 mg/dl and AT III levels greater than 80%. No bleeding or thrombosis and no signs of disseminated intravascular coagulation were observed. Our study shows that correction of the alterations of the coagulation system induced by asparaginase can be achieved with a replacement regimen substituting both procoagulant material and the most important inhibitor of the coagulation system.
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Grey scale abdominal ultrasound was used in a total of 146 children for primary diagnostic evaluation of abdominal masses and for follow-up of patients with malignant diseases. Of 93 patients examined for a suspected abdominal mass, 6 showed to have an intraabdominal tumour. In each case the site of tumour origin could be ascertained by ultrasound. The remaining 87 patients showed no pathological findings and have been tumour-free at routine follow-up studies. Sonography proved to be also useful in the follow-up of patients with malignant diseases. Local recurrence or metastases could be ruled out on the basis of sonographic findings alone in 32 out of 35 surgically treated patients, in 3 children with non-resectable tumours, changes in tumour size during radio- and chemotherapy could be studied. Diagnostic ultrasound was also of great importance in the assessment of complications after surgery, radio- and chemotherapy. So the early detection of hydronephrosis in 2 patients with treated nephroblastoma prevented a damage of the remaining kidney. Due to the high accuracy of sonography, invasive diagnostic methods could be restricted to a small number of patients in whom ultrasound failed to provide adequate information.
A complex of factor VIII coagulant moiety and phospholipid was purified by means of immunoadsorbent chromatography of factor VIII concentrate and consecutive dissociation of the immobilized factor VIII complex by human placental phospholipid (Fibraccel). The complex of factor VIII coagulant moiety and phospholipid displayed factor VIII coagulant activity (VIII C) and factor VIII coagulant antigen (VIII C:Ag), but no factor VIII related antigen (VIII R:Ag). When incubated with factor VIII inhibitor plasma, the complex of factor VIII coagulant moiety and phospholipid exerted higher factor VIII coagulant activity than native factor VIII complex, and than purified factor VIII coagulant moiety alone. Our results prove that in activated prothrombin complex concentrates a complex of factor VIII coagulant moiety and phospholipid is the active mechanism exerting procoagulant activity in factor VIII inhibitor plasma.
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Factor VIII activity (VIII C), factor VIII coagulant antigen (VIII C:Ag), factor VIII-related antigen (VIII R:Ag), Ristocetin cofactor (R:Cof), and growth hormone were studied in eight children with growth hormone deficiency prior to, and 60, 120, and 180 minutes after administration of human growth hormone (Crescormone, Kabi), 4 U/m2. Growth hormone has been administered for 1.0 to 6.8 years but was stopped one week prior to this investigation. Basal levels of VIII C, VIII C:Ag, VIII R:Ag, and R:Cof were within the normal limits. After administration of human growth hormone, VIII C, VIII C:Ag, VIII R:Ag, and R:Cof showed a significant rise. Our study shows that growth hormone is not necessary to maintain the basal levels of the factor VIII complex within the normal limits. However, out data suggest that growth hormone has some influences on the levels of the factor VIII complex. The mechanisms for the rise of the factor VIII complex--whether this is a direct effect of growth hormone or not--are not clear at this point.
To study the dissociation of the two moieties of the factor-VIII complex during clotting, plasma, concentrate and serum were chromatographed on 4% agarose. In plasma and concentrate factor-VIII coagulant activity (VIII C), factor-VIII coagulant antigen (VIII C:Ag), and factor-VIII-related antigen (VIII R:Ag) eluted together in the void volume, but some VIII C:Ag eluted after the void volume. The amount of VIII C:Ag eluting after the void volume was made smaller by adding proteinase inhibitors. In serum nearly all VIII C:Ag eluted after the void volume. From factor-VIII complex immunoadsorbed by means of an antibody against VIII R:Ag no VIII C:Ag was dissociated by thrombin or by thrombin and physiologic CaCl2 concentrations. Radiolabelled human thrombin did not bind to the VIII C:Ag of immunoadsorbed factor-VIII complex. VIII C:Ag displaying VIII C was dissociated from immunoadsorbed factor-VIII complex by human brain thromboplastin or by phosphatidyl-serine. Our results suggest that VIII C:Ag and VIII R:Ag dissociate during clotting. This dissociation seems not to be mediated by thrombin, but may be mediated by phospholipids.
A 6 year old previously healthy girl developed an acute thrombocytopenia of 13000 platelets per cm3 that lead to the diagnosis of selective IgA deficiency and antinuclear antibodies in this patient. Platelet numbers became normal within 4 weeks, but the antinuclear antibodies persisted. The reported case represents another example of an immunoregulatory disorder in children with "idiopathic" thrombocytopenia.
Newborns have low levels of the vitamin K dependent clotting factors. Early studies were suggestive of vitamin K deficiency. Recently these findings were questioned by studies that failed to detect signs of vitamin K deficiency in the clotting system of newborns using more specific methods, while other studies did find signs of vitamin K deficiency using the same methods. The question was finally solved by direct measurement of vitamin K showing very low levels of the vitamin in the serum of newborns immediately after birth. Whether vitamin K supplementation to the mother reduces the incidence of vitamin K induced changes in the clotting system of newborns remains to be elucidated. In the meantime it seems prudent to administer parenteral vitamin K prophylactically to all newborns immediately after birth.
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A two year old boy was seen in the orthopedic clinics because of typical symptoms of Legg-Perthes disease, a scintigraphy with Technetium99m showed a distinct deficiency of nuclear activity in the femoral head which is characteristic of the early stage of Legg-Perthes disease. A routine blood count lead to the diagnosis of acute lymphoblastic leukemia. The boy was treated according to the Austrian cooperative leukemia protocol and complete remission was achieved. No orthopedic treatment of the femur head necrosis was done, after eight weeks of treatment with multiagent chemotherapy the boy started to walk again and subsequently became free of all symptoms of Legg-Perthes disease. A scintigraphy done eight weeks after the initial scintigraphy showed that the deficiency of radionuclear activity of the femoral head was nearly vanished. This case illustrates the variability of bone involvement in acute lymphoblastic leukemia, which often is the most prominent symptom at an early stage of the disease.
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This paper presents the case report of a 2 year-old boy in whom a grade II fetal alcohol syndrome was diagnosed at 17 months. At 21 months, an embryonal rhabdomyosarcoma of the urinary bladder trigone was found, and the tumour excised. Polychemotherapy was initiated, but bone-marrow depression and death in septicaemic shock followed. The main autopsy findings included the demonstration of tumour remnants in the urinary bladder and prostate, and a severe acute purulent pyelonephritis. This is the fifth case of fetal alcohol syndrome in conjunction with a malignant tumour to be reported in the international literature. These cases do not show any uniformity as to tumour type. No conclusion can as yet be drawn as to whether there is a pathogenetic connection between the fetal alcohol syndrome and the occurrence of malignant tumours (as is the case with the fetal hydantoin syndrome and neuroblastoma), or whether these findings are purely coincidental. Clinicians and pathologists should, however, be advised to look more closely for even minimally pronounced forms of the fetal alcohol syndrome in children with embryonic tumours.
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Reports on variants of von Willebrand's disease are numerous, but many of these are based on tests that will show marked fluctuations with time and tests that might not be similar in affected family members. This report describes 8 patients with a new variant of von Willebrand';s disease in which there is a normal APTT, slightly reduced one-stage factor VIII:C assay (VIII:C-1), and a drastically reduced two-stage factor VIII:C assay (VIII:C-2). The VIII:C in this variant is more readily adsorbed to AI(OH)3. This variability in VIII:C assays and excessive adsorption to AI(OH)3 are corrected by the addition of either hemophilic plasma or hemophilic factor-VIII-related antigen. This variant is stable with restudy on multiple occasions and is inherited in a stable fashion in three generations of one family. The multimeric structure of the VIIIR:Ag appears normal, although the concentration is moderately reduced. The differences in functional activity, the adsorption to AI(OH)3, and the differences between functional and antigenic (VIII:C Ag) assays of VIII:C support that this is a functional abnormality of type I von Willebrand's disease.
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