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Biomedical subjects

I Slavc

Publications and source records attributed to I Slavc.

At least 37 records · Page 2Linked to original sources
PubMed1994

Ocular sequelae of multimodal therapy of hematologic malignancies in children.

We retrospectively analyzed the ocular findings after polychemotherapy including intrathecal methotrexate, systemic corticosteroids, and prophylactic cranial irradiation in children with acute lymphoblastic leukemia (n = 16) and non-Hodgkin's lymphoma (n = 2). After a median surveillance time of 4.1 years, asymptomatic ocular abnormalities were observed in 83% of the patients: 7/18 had a decreased tear formation, 5/17 had an opacity of the vitreous body, and 13/18 had an opacity of the lens. It was not possible to determine retrospectively which therapy caused a particular effect. A comparison of the 2 irradiation techniques (with and without blocking of the lacrimal glands) showed that in 5/7 children who developed a reduced eye secretion, the lacrimal glands are within the treatment volume. Therefore, the reduced eye secretion is most likely radiation-induced. Whereas the opacities of the vitreous body were caused by thrombopenia and bleeding during the course of disease, corticoid therapy might have contributed to the lens opacities.

Adolescent↗
PubMed1994

Follow-up and quality of survival of 67 consecutive children with CNS tumors.

We report the findings at follow-up in 67 consecutive children with central nervous system tumors treated over a 5-year-period at a single institution. The diagnoses were supratentorial astrocytoma (n = 12), cerebellar astrocytoma (n = 10), ependymoma (n = 9), medulloblastoma (n = 9), brain stem glioma (n = 6), optic pathway glioma (n = 5), and others (n = 16). The survival rates were 83% for supratentorial astrocytomas at a median of 46.5 months, 90% for cerebellar astrocytomas and 55% for ependymomas at 40 months, respectively, 55% for medulloblastomas at 22 months, 33% for brain stem gliomas at 23 months, and 80% for optic pathway gliomas at 49 months. With regard to neurological sequelae, 13 patients were treated for epilepsy, 13 patients had mild to moderate neurological deficits, and 4 patients were severely disabled. Seventeen of 37 tested patients performed below average on formal neuropsychometric testing, one-fourth attended special education courses, and at least one-fourth suffered from behavioral and adjustment problems.

Adolescent↗
PubMed1994

Familial hemophagocytic lymphohistiocytosis associated with disseminated T-cell lymphoma: a report of two siblings.

Two siblings with evidence of disseminated T-cell lymphoma at the time of diagnosis of familial hemophagocytic lymphohistiocytosis (FHL) are reported, an association which has not been described previously. The first child with typical clinical and laboratory features of FHL died shortly after admission, before diagnosis could be established. Retrospective analysis of autoptic tissue revealed marked hemophagocytosis as well as morphological and immunohistochemical features suggestive of disseminated T-cell lymphoma. In the second child, FHL was diagnosed in time. Subsequent histologic investigation of bone marrow biopsies displayed a focal infiltration by T-cell lymphoma. DNA hybridization studies provided evidence of a monoclonal T-cell receptor beta chain gene rearrangement. Following conventional chemotherapeutic induction for FHL, the patient received an allogeneic bone marrow transplant (BMT) from a related healthy donor. Currently, 17 months after BMT, the boy is in unmaintained remission from FHL and T-cell lymphoma. The current pathogenetic concepts for FHL and a possible relationship between T-cell lymphoma and FHL are discussed.

Blotting, Southern↗
PubMed1992

Hyperfractionated radiotherapy and polychemotherapy in brain stem tumors in children.

Between October 1989 and January 1991 five children with brain stem tumors were treated with sequential chemo- and radiotherapy. The polychemotherapy consisted of procarbazine, ifosfamide, etoposide, methotrexate, cisplatin and cytosine arabinoside. Locally, hyperfractionated radiotherapy was delivered at a total dose of 63.8 Gy (1.1 Gy twice daily, 10 fractions per week). After a median observation time of 11.8 (range 4-23) months from diagnosis three children are alive and without evidence of tumor progression. Two patients died from tumor progression 11 and 16 months respectively after initiation of therapy.

Adolescent↗
PubMed1992

Paraparesis secondary to a spinal mass as the presenting feature of erythroleukaemia in a 10-month-old child.

Severe neurological impairment as the first symptom of acute leukaemia is a rather uncommon finding. We report the case of a 10-month-old infant who presented with acute paralysis of the lower extremities due to cord compression by an epidural tumour composed of malignant erythrocyte precursor cells. Diagnosis of erythroleukaemia (EL) was made by needle biopsy of the spinal epidural mass and confirmed by bone marrow aspiration. Antileukaemic treatment in combination with radiotherapy to the epidural tumour led to haematological remission and neurological recovery with disappearance of the mass lesion as demonstrated by MRI. However, haematological relapse occurred with death of the patient 7 months after diagnosis. This is the first reported case of EL presenting with paraparesis due to an epidural tumour. The clinical symptoms, results of cytogenetic and immunological studies and the clinical course are described.

Biopsy, Needle↗
PubMed1992

[Indications, problems and future perspectives of bone marrow transplantation in pediatrics].

Bone marrow transplantation (BMT) is an established therapy in pediatric oncology and is increasingly used as curative approach in the treatment of congenital, non-oncologic diseases of the lymphohematopoietic system. There is increasing evidence, however, that BMT can be followed by severe long term effects including neuroendocrine, ophthalmologic, dental and central nervous system abnormalities, particularly in children. Therefore the indication to BMT depends on the results obtained by conventional therapy. Due to the high cure rates of leukemia with conventional therapy BMT is only warranted following relapse except for certain forms with poor prognosis factors. For patients with chronic myelogeneous leukemia, however, BMT is the only chance of cure. In solid tumors the role of BMT is more difficult, because there is no clear evidence that BMT is superior to conventional therapy with regard to long term survival. In severe aplastic anemia, however, the long term results of BMT are clearly better than those obtained by conventional therapy. Other undisputed indications for BMT are severe combined immuno deficiencies and other congenital diseases for which BMT is currently the only curative therapy. Progress with matched unrelated donor transplantations by better histocompatibility testing and more specific immunosuppressive therapy to reduce graft-versus-host disease, still a major problem of allogeneic BMT, as well as the perspectives of gen therapy in the future will offer a chance of cure to many patients without a matched sibling donor.

Anemia, Aplastic↗
PubMed1992

ABO-incompatible bone marrow transplantation: prevention of hemolysis by alkaline hydration with mannitol diuresis in conjunction with red cell reduced buffy coat bone marrow.

Bone marrow transplantation (BMT) in the presence of major ABO incompatibility presents the risk of a potentially fatal hemolytic transfusion reaction at the time of marrow infusion. We describe the use of a forced alkaline hydration/mannitol diuresis regimen in combination with red blood cell (RBC) reduced bone marrow given as buffy coat in 5 patients undergoing allogeneic BMT from ABO incompatible donors. Three patients had ABO antibody titers of 1:32 and were not subjected to antibody removal procedures. Two patients with respective antibody titers of 1:512 and 1:128 underwent plasmapheresis to reduce the antibody titers to below 1:64. The forced diuresis/mannitol regimen was well tolerated. Although the RBC content was still high in the buffy coat preparation a significant hemolytic transfusion reaction was successfully prevented. No patient had back pain, hyperbilirubinemia or renal impairment despite clinical and laboratory evidence of hemoglobinuria. These data indicate that patients with antibody titers below 1:64 might be spared the risks and cost associated with plasmapheresis or complete RBC depletion of the bone marrow transplant.

ABO Blood-Group System↗
PubMed1991

Acute megakaryocytic leukemia in children. Clinical, immunologic, and cytogenetic findings in two patients.

An unusual presentation of acute megakaryocytic leukemia (AMKL) is reported in two young children. The first child had a 10-day history of ptosis of the right eyelid as the initial manifestation of AMKL, a clinical picture not previously described in this variant of leukemia. Computed tomographic scanning showed multiple intracranial mass lesions, and the diagnosis of AMKL was confirmed by immunophenotyping of bone marrow blasts. The second child had Down syndrome and received alkylating agents and radiation therapy for treatment of metastatic rhabdomyosarcoma of the orbit. She had AMKL as second malignancy. Both patients had acquired chromosome 21 anomalies in their leukemic blasts. The first patient, constitutionally normal, had an i(21q) in his leukemic blasts; the patient with constitutional trisomy 21 had tetrasomy 21 and additional chromosomal changes. The clinical symptoms and the results of morphologic, immunologic, and cytogenetic studies are discussed.

Blepharoptosis↗
PubMed1991

[Infections in the neutropenic phase following bone marrow transplantation: comparison of laminar airflow isolation with conventional isolation].

From May 1978 to September 1989 45 patients underwent 25 allogeneic and 21 autologous bone marrow transplantations (BMT) and 1 peripheral stem cell transplantation for the following indications: severe aplastic anemia (n = 4), hematological malignancies (n = 28), malignant solid tumours (n = 12) and sideroblastic anemia (n = 1). The first group of 20 patients was isolated in a conventional hospital room, while management of the aplastic phase in the second group of 25 patients was performed in a laminar air flow (LAF) unit. All patients received total decontamination. In a retrospective analysis the number of positive blood cultures during the neutropenic period was 85% in the first group, as compared with 40% in the second group, and the number of febrile episodes was 85% versus 64%, respectively. Despite the fact that the septic morbidity was lower in the LAF group, mortality during the neutropenic period (15% in group I versus 16% in group II) was unaffected and survival rate (45% in group I versus 36% in group II) did not improve. We conclude that LAF protection will only have a positive impact on survival rate if the incidence of non-infectious complications of BMT, such as organ toxicity or graft-versus-host disease, is likewise reduced.

Adolescent↗
PubMed1990

myc gene amplification and expression in primary human neuroblastoma.

Although N-myc amplification in neuroblastomas correlates with poor prognosis, not all neuroblastomas which fail to respond to therapy have N-myc amplification. To determine whether other modes of myc gene activation underlie progression of some neuroblastomas, 45 were analyzed for amplification of N-myc, c-myc and L-myc and 26 were studied for transcription of these oncogenes. N-myc amplification was found in 6 of 45 tumors; no tumor had amplification of c-myc or L-myc. Transcription of both N-myc and c-myc occurred in 21 of 26 neuroblastomas. No tumor without N-myc amplification had a level of N-myc expression near that of a tumor or cell line with amplification. One tumor with N-myc amplification was the only specimen with N-myc but not c-myc expression. Five samples had c-myc but not N-myc expression; all had histological features of ganglioneuroma. DNA index did not correlate with myc gene amplification or expression. It is concluded that N-myc and c-myc are commonly expressed in primary untreated neuroblastomas, but in the absence of N-myc amplification, expression of these genes does not appear to correlate with disease progression.

Adolescent↗
PubMed1990

Imipenem-antibiotic monotherapy in juvenile cancer patients with neutropenia.

From 1984 to 1987 two consecutive groups of juvenile cancer patients (n = 45) with fever and neutropenia corresponding in all criteria were examined. In half of the total of 90 febrile episodes and septicemias, a conventional antibiotic combination therapy (Pseudomonas-active penicillin/cephalosporin of the third generation/aminoglycoside) was instituted. In the remaining half imipenem was used as an antibiotic monoagent. In 66% and 60% of the febrile episodes treated with antibiotic combination therapy and with imipenem, respectively, septicemia was confirmed by positive blood cultures. Nineteen febrile episodes occurred in the myeloaplastic phase after bone marrow transplantation. In a comparative study of imipenem as monotherapy versus an antibiotic combination therapy the results obtained with imipenem were superior in many regards. No resistance developed necessitating change of antibiotic therapy. Coagulase-negative Staphylococci, primarily responsible for catheter-associated septicemia, were susceptible. Duration of fever and thus duration of treatment were shorter. The incidence of side effects and costs were lower. Therefore, imipenem as an antibiotic monotherapy in febrile cancer patients with neutropenia appears to be more efficacious than the conventional combination therapy, even during myeloaplasia following bone marrow transplantation. The results and rationale of this retrospective analysis are discussed.

Adolescent↗
PubMed1990

[Primary and secondary inoperable teratoid hepatoblastoma--multidisciplinary management].

Hepatoblastomas are the most frequent malignant liver tumors of childhood. Prognosis mostly depends on their resectability. Unresectable tumors are almost invariably fatal, unless their size can be decreased by chemotherapy and/or irradiation to permit surgical removal. In the present case report we discuss the clinical course of a 15 months old boy, who was admitted with primary inoperable teratoid hepatoblastoma. After alternating intravenous and intraarterial chemotherapy as well as radiation therapy, the tumor could be successfully resected. Specific problems of histological diagnosis and of the therapeutic management are discussed, and an overview of the recent literature in behalf of the histological basis, therapeutic possibilities and prognostic factors of hepatoblastomas is given.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1990

Anesthetic management of marrow harvesting from a 7-week-old premature baby.

Bone marrow was harvested from a 3.95 kg premature 7-week-old female baby for donation to a 13 kg HLA-identical sister with severe aplastic anemia. Two hundred ml of donor bone marrow were aspirated, containing a calculated dose of 3 x 10(8)/kg nucleated bone marrow cells for the recipient. This was equivalent to two-thirds of the donor's calculated blood volume (320 ml). Peri-operative care included invasive monitoring of intravascular pressures, arterial blood gas analysis, careful temperature control and the infusion of 150 ml of packed red cells, 150 ml of colloid and 50 ml of crystalloid. Rapid engraftment occurred. There were no complications and both donor and recipient are healthy 12 months later.

Anemia, Aplastic↗
PubMed1990

Busulfan/cyclophosphamide plus bone marrow transplantation is not sufficient to eradicate the malignant clone in juvenile chronic myelogenous leukemia.

Bone marrow transplantation plays an essential role in the successful treatment of both juvenile and adult chronic myelogenous leukemia. Recently, it has been reported that conditioning with high doses of busulfan can successfully replace total body irradiation (TBI), in patients with acute myelogenous leukemia as well as adult chronic myelogenous leukemia. We report here the case of a 29-month-old boy with juvenile chronic myelogenous leukemia (JCML) transplanted with HLA-identical bone marrow after conditioning with busulfan, etoposide and cyclophosphamide. Successful engraftment was followed by early relapse on day 67. A second HLA-identical transplant was performed following myeloablative treatment with TBI. Engraftment was once again successful and the patient remains free of disease more than 24 months after transplantation. We conclude that busulfan is insufficient in eradicating JCML and that TBI is required prior to transplantation.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1989

MR findings in methotrexate-induced CNS abnormalities.

MR of the brain was performed in eight patients (mean age, 14.9 years) with osteogenic sarcoma during or after IV treatment with high-dose methotrexate. MR detected brain abnormalities in four patients, three of whom had concomitant neurologic dysfunction. Pathologic findings demonstrated on MR were (1) chronic brain edema, demonstrable over a period of 3-14 months (proved by autopsy in one patient); (2) multifocal white matter necrosis; and (3) deep brain atrophy. MR appears to be valuable in the detection of abnormalities induced by treatment with high-dose methotrexate.

Adolescent↗
PubMed1989

Successful treatment of CMV retinitis with ganciclovir after allogeneic marrow transplantation.

Cytomegalovirus (CMV) infection of the retina is a well recognized complication in patients with the acquired immune deficiency syndrome but is rarely seen after bone marrow transplantation (BMT). Among a variety of drugs ganciclovir so far appears to be the most effective therapy for CMV retinitis, but in previous studies relapses occurred in all patients in whom ganciclovir was interrupted. We report the clinical findings in a 22-year-old BMT recipient who developed bilateral exudative CMV retinitis 64 days after BMT despite prophylactic treatment with high-titer CMV-immunoglobulins and transfusions of CMV-negative blood products and donor bone marrow. During a 12 day course of treatment with 7.5 mg/kg/day of ganciclovir the CMV retinitis improved and viruria ceased on day 4 of therapy. In contrast to the previous reports, CMV retinitis in this patient continued to improve even after ganciclovir was stopped and eventually complete healing of all intraretinal lesions as well as total reconstitution of the visual acuity was achieved. He is now free of disease and without relapse of CMV retinitis more than 1 year after transplantation.

Adult↗
PubMed1989

[Cyclosporin A in severe aplastic anemia in children].

Severe aplastic anemia should be treated with bone marrow transplantation if possible. Various clinical and experimental data support the view that the major pathogenetic defect in SAA is a dysregulated cellular immune response which in turn has a negative effect upon hematopoiesis. Therefore a large percentage of patients react favorably to treatment with immunosuppressive agents as antithymocyte globulin and high-dose methylprednisolone. Data concerning the efficiency of Cyclosporine A treatment are limited until now. We present in this report our own experience with Cyclosporine A treatment in three children with severe aplastic anemia who lack a bone marrow donor. Included is also a review of the present therapeutic possibilities.

Adolescent↗