PubMed HealthSearch

Biomedical subjects

C Urban

Publications and source records attributed to C Urban.

At least 19 recordsLinked to original sources

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

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

Reduction of the dose to the lens in prophylactic cranial irradiation: a comparison of three different treatment techniques and two different beam qualities.

Three treatment techniques using two beam qualities have been compared on the basis of dose to the lens in prophylactic cranial irradiation. The dose to the lens and the globe was measured with thermoluminescent crystals in an anthropomorphic phantom and calculated by a computer-assisted planning system. A comparison was made of large field and small field techniques using 60Co and 8 MV photons. Modifications to the basic techniques studied included angulation of the gantry, angulation of the couch, and placement of an additional eye block close to the surface. The dose to the lens could be reduced to four percent of the midplane dose by applying the small-field technique combined with the use of 8 MV energy photons, by placing an additional block close to the surface, and by five degree occipitally angling the gantry, as well as rotating the treatment couch to account for the divergence of the beam. The use of 60Co produced an underdosage of the posterior segment of the globe in angled treatment techniques.

Cataract

Congenital sideroblastic anemia successfully treated by allogeneic bone marrow transplantation.

Allogeneic bone marrow transplantation (BMT) was carried out on a 34-month-old boy with congenital sideroblastic anemia. The patient had been red blood cell transfusion dependent since the age of 7 weeks. He did not respond to therapy with pyridoxine and developed secondary progressive hemosiderosis. The preparatory regimen consisted of busulfan (3.5 mg/kg for 4 days) and cyclophosphamide (50 mg/kg for 4 days). Full engraftment of donor bone marrow was achieved and effective hemopoiesis is still maintained 3 years after BMT.

Anemia, Sideroblastic

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

Treatment of infant leukemia with busulfan, cyclophosphamide +/- etoposide and bone marrow transplantation.

Infants with acute leukemia have a poor chance of being cured by conventional chemotherapy. We therefore treated cases of infant leukemia with high dose chemotherapy followed by bone marrow transplantation (BMT). Six suffered from acute leukemia and one from refractory anemia with excess of blasts (RAEB-t). The conditioning regimen consisted of busulfan (BU) and cyclophosphamide (CY), and was intensified by adding etoposide (VP) in four cases. At the time of BMT the children were 4, 5, 12, 13, 13, 14, and 20 months old. Three children were autografted, three received HLA-identical marrow from a sibling donor, and one child received matched unrelated donor marrow. All five children who were grafted in complete (CR) or partial remission (PR) are alive and well in CR 7, 13, 24, 37, and 46 months after allogeneic (two patients) or autologous (three patients) BMT, and 13, 17, 29, 42, and 53 months after initial diagnosis. The child with RAEB-t and the one transplanted in second chemotherapy-resistant relapse of acute non-lymphoblastic leukemia relapsed at 7 and 17 months respectively. The chemotherapy regimen was well tolerated. BU-CY-VP is a promising alternative treatment to regimens including total body irradiation for very young children suffering from acute leukemia.

Bone Marrow Transplantation

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

Effect of a beta-lactamase inhibitor, tazobactam, on growth and penicillin-binding proteins of Borrelia burgdorferi.

The effects of tazobactam, a relatively new beta-lactamase inhibitor, were investigated on growth and penicillin-binding proteins (PBPs) of Borrellia burgdorferi. A previous communication from our group demonstrated several proteins capable of binding labelled penicillin in this organism. Of these proteins, 94-kDa and 57-kDa PBPs possessed the highest affinity for penicillin and were assumed to be essential proteins involved in cell-wall synthesis. In these experiments, tazobactam was used in competition binding experiments as well as on whole spirochetes. Only the 94-kDa and 57-kDa PBPs were affected by increasing amounts of tazobactam during competition-binding experiments and growth of B. burgdorferi was also inhibited. These results may explain the in vitro activity of beta-lactamase inhibitors in general and suggest a utility for these compounds when examining PBPs with hydrolysing activity and/or organisms with beta-lactamases.

Bacterial Proteins

Role of candida in pathogenesis of antibiotic-associated diarrhoea in elderly inpatients.

The part that candida plays in antibiotic-associated diarrhoea was investigated in 24 elderly inpatients (mean age 74 years) who tested negative for Clostridium difficile toxin and other intestinal pathogens. 7 had intestinal overgrowth of Candida species (greater than or equal to 10(5) cfu/ml). None of the 24 matched, antibiotic-treated controls without diarrhoea had candida overgrowth. All 5 patients with diarrhoea and candida overgrowth treated with oral nystatin responded with resolution of diarrhoea and lowering of faecal counts to less than 10(4) cfu/ml within 7 days of start of antifungal therapy despite continuation of antibacterial therapy. In the other 2 patients with candida overgrowth, the diarrhoea subsided spontaneously and faecal candida counts returned to normal (less than 10(4) cfu/ml) after antibacterial agents were withdrawn. In patients without candida overgrowth, diarrhoea persisted until antibiotics were withdrawn, at a mean of 16 days after study entry.

Aged

[Oligosymptomatic Bourneville-Pringle tuberous sclerosis of the brain with giant cell astrocytoma].

We report on a 9-year-old boy who developed subependymal giant-cell astrocytoma associated with tuberous sclerosis. Apart from a few hypopigmented skin patches, the boy's clinical picture was completely unremarkable until the onset of diplopia and vertigo reflecting increased intracranial pressure. Magnetic resonance imaging of the brain revealed subcortical hamartomas in addition to the intraventricular tumour. Seizures and mental retardation were absent. The boy's mother had suffered from seizures during childhood and presented with typical skin lesions. The importance of thorough examination and regular follow-up even in patients with oligosymptomatic variants of tuberous sclerosis is emphasized.

Adult

[Endocrinologic function following cranial irradiation in acute lymphoblastic leukemia in childhood].

Endocrinological function was evaluated in 31 children after successful treatment of acute lymphoblastic leukaemia. All patients had received combination chemotherapy and 12-24Gy of cranial irradiation according to the German therapy protocols BFM-81, BFM-83 and BFM-86. Height, weight, bone age and pubertal development, as well as hypothalamic-pituitary function were measured. Long-term linear growth was unaffected in all patients. However, 9 patients showed subnormal serum growth hormone levels in response to pharmacological stimulation of the pituitary. All patients had normal levels of T3 and T4, but one patient showed an increased response of thyrotropin to thyrotropin releasing hormone. All prepubertal and postpubertal children demonstrated appropriate secretion of follicle-stimulation hormone (FSH) and luteinizing hormone (LH) after stimulation with LH-releasing hormone (LH-RH). 3 pubertal girls showed adequate oestradiol levels, but abnormally high levels of gonadotropins in response to LH-RH. Sexual development was normal in two of them, but the third had a late menarche and irregular menses. The significance of these findings is discussed in the context of recommendations possibly to further reduce or completely delete prophylactic cranial irradiation.

Adolescent

[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

[(Management of acute toxicity after high dose methotrexate therapy)].

A 13-year-old girl with osteosarcoma and pulmonary metastases developed life-threatening renal toxicity, encephalopathy, and bone marrow failure following high dose methotrexate therapy. After successful treatment, high dose methotrexate therapy was continued without further problems. Recommendations for the prevention and the current management of methotrexate toxicity are discussed.

Adolescent

[Immunosuppressive treatment of endogenous uveitis in childhood (author's transl)].

This report is about a seven year old boy suffering from endogenous uveitis of unknown etiology in both eyes. A local and systemical steroid therapy did not stop the progressive course. Therefore an immunosuppressive treatment with methotrexate was begun. After the second intravenous application of this antifolate an amelioration could be noted and after 6 week a normalisation of the visual acuity on the left eye and an improvement from 0.4 to 0.8 on the right eye was obtained. Immunosuppressive treatment was discontinued after a duration of 14 months; now 2 years later, the inital success has been maintained. The advantages of methotrexate in comparison to other immunosuppressive agents are discussed.

Child

[Thrombasthenia in a newborn (author's transl)].

Thrombasthenia is an extemely rare hereditary disorder of platelet function characterized by impairment of adhesiveness, ADP-induced aggregation, and clot retraction. Petechiae and ecchymoses in a newborn infant followed by prolonged jaundice facilitated early diagnosis.

Blood Coagulation Factors