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R Kerbl

Publications and source records attributed to R Kerbl.

At least 19 recordsLinked to original sources
PubMed2001

Persistent hypercapnia in children after treatment of obstructive sleep apnea syndrome by adenotonsillectomy.

Obstructive sleep apnea syndrome (OSAS) in childhood is frequently in part a consequence of enlarged adenoids and/or tonsils and may lead to hypoxemia and hypercapnia during sleep. Whereas long-term blood gas alterations are well documented in adults, only few polygraphic data are available for children. It was the aim of this study to document blood gas alterations before and after treatment in this population. 9 children with OSAS (6 male, 3 female, median age 5.9 years, range 1.1-13.5 years) were investigated by polysomnography before and after adenotonsillectomy. Prior to intervention most children presented with moderate hypercapnia (ETCO2 mean 44.3 +/- 3.8 mm Hg, ETCO2 maximum 53.2 +/- 5.2) and hypoxemic episodes (oxygen saturation mean 93.2 +/- 3.2%, minimum 74.4 +/- 16.5%). Following adenotonsillectomy subsequent polygraphic investigations displayed normalisation of oxygen saturation (saturation mean 96.1 +/- 0.8%, minimum 90.1 +/- 3.1%). In contrast, moderate hypercapnia in several patients persisted up to five months after treatment (ETCO2 mean 44.9 +/- 2.8 mm Hg, ETCO2 maximum 51.2 +/- 3.6). Persistent hypercapnia most likely reflects an adaptation process of chemosensitivity and respiratory control due to preceding long-term hypercapnia.

Adaptation, Physiological↗
PubMed2001

Intratumoural heterogeneity of 1p deletions and MYCN amplification in neuroblastomas.

BACKGROUND: At least three genetic hallmarks identify aggressive tumour behaviour in neuroblastomas; amplification of the oncogene MYCN; deletion (loss of heterozygosity [LOH]) at the short arm of chromosome 1 (del1p36), seen in approximately 28% of the cases; and di-tetraploidy. The MYCN oncogene is amplified in approximately 23% of all neuroblastomas and becomes important for the stratification of therapy in localised and 4s tumours. Up to now, it has been believed that the genetic constellation of neuroblastic tumours is stable and does not alter during tumour evolution or during tumour progression. PROCEDURE: Using fluorescence in situ hybridisation techniques (FISH) to investigate different tumour areas on touch preparations and histological sections, we show that genetic heterogeneity can be detected in neuroblastomas, especially in tumours detected by urinary mass screening. CONCLUSION: The identification of such cell clones is important, because the MYCN amplification and/or the deletion at 1p36 appear to be responsible for aggressive local growth and development of metastases.

Chromosomes, Human, Pair 1↗
PubMed2001

Outcome and long-term side effects after synchronous radiochemotherapy for childhood brain stem gliomas.

Between 1993 and 1999, 11 children with histologically confirmed diffuse and exophytic brain stem glioma (BSG) were treated with intensive induction chemotherapy and simultaneous external beam irradiation. Chemotherapy was performed according to the German/Austrian Pediatric Brain Tumor Study HIT '91 and included two cycles of ifosfamide (days 1-3), etoposide (days 4-6), methotrexate (days 15 and 22), cisplatin (days 29-31) and cytarabine (days 29-31), separated by a 3-week interval. Maintenance chemotherapy with carmustine, carboplatin and vincristine (8 cycles over a 1-year period) was given in those patients who responded clinically or radiographically to induction chemotherapy. Six of 11 patients showed an objective reduction in tumor size on magnetic resonance imaging and 4 of 11 are alive in good general condition >22, >22, >90 and >92 months, respectively, after diagnosis without radiographic evidence of tumor progression (1 complete remission, 2 partial remissions, 1 stable disease), but suffer from moderate to severe long-term side effects. Three patients died due to disease progression after having achieved a partial remission which lasted 5, 6 and 18 months, respectively, whereas only short-term stabilization was observed in 4 patients who died within 1 year after diagnosis. Acute hematologic toxicity was severe but manageable. This intensive combined modality treatment was toxic but yielded objective responses in more than 50% and long-term survivors in one third of childhood BSG patients.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2001

Tunneled femoral central venous catheters in children with cancer.

OBJECTIVE: We discuss the feasibility of long-term femoral venous access by means of a cuffed subcutaneously tunneled central venous catheter (Broviac catheter) in selected pediatric cancer and stem cell transplant patients in whom access via the veins of the upper part of the torso is difficult or contraindicated and in whom alternative routes must be used. PATIENTS AND METHODS: We report on our experience with 9 patients (3 of whom underwent stem cell transplantation) who received femoral Broviac catheters between December 1990 and November 1999. Results. Time in place ranged from 4 to 155 days with a median of 58 days (mean: 71.2 days). Three catheters had to be removed: 1 because of infection of the subcutaneous tunnel and 2 because of catheter obstruction. The remaining 6 catheters functioned well without problems as long as they were needed; 1 of them got accidentally dislodged while the patient was off treatment. No episodes of catheter-related septicemia, thrombosis, kinking, or drug extravasation were noted; there were no catheter-related infectious complications in the transplant patients. CONCLUSIONS: Our experience indicates that in those instances in which customary access to the superior vena cava is precluded, long-term venous access by way of the femoral vein is a feasible and safe alternative in children, even in the setting of stem cell transplantation.

Adolescent↗
PubMed2000

[4th Austrian SIDS Consensus-Consultation and the Viennese SIDS prevention campaign "Secure Sleep"].

Despite numerous investigations the pathophysiologic mechanisms of SIDS have not been fully elucidated. In large epidemiologic studies highly variable SIDS mortality rates were noted between different countries and cultures. This presumably is due not only to differences in diagnostics and classification of SIDS but also in lifestyle and newborn care. The common denominator is the identification and prevention of the main risk factors: smoking, sleeping in the prone position, over-heating, wrong "bedding". SIDS prevention campaigns that have focussed upon these risk factors have led to a dramatic reduction in the incidence of SIDS. In preparation for the SIDS prevention campaign of Vienna ("Safe Sleep") the content, strategy and procedure of the Austrian prevention campaigns were analysed. The current focus is to convey a clear and uniform message in personal conversations before and after birth of the child. These conversations with parents are the most important tool to detect SIDS related anxiety and a possibly increased risk of SIDS. In the last 30 years various polysomnographic parameters were published that were associated with an increased risk of SIDS. Today there is international consent that polysomnography is not an efficient screening method to demonstrate increased risk of SIDS. Therefore the use of polysomnography, besides research purposes, has been limited to investigating clinical symptoms of infants and children. Concerning monitoring it is important to note that--in contrast to the undisputed importance of monitoring breathing disorders--the effectiveness in SIDS prevention is unproven. State of the art are instruments that monitor heart and breathing rate and have adequate storage functions. The duration of monitoring should encompass the symptomatic period as well as a safety period of three months. The monitor should not be routinely prescribed for a year. The guiding principle is "As short as possible with stringent indication". Prerequisite for the monitoring is good instruction of the parents and a continuous consultation by competent outpatient clinics.

Austria↗
PubMed2000

[SIDS and polygraphy].

The description of an association between central apneas and the sudden infant death syndrome (SIDS) by Steinschneider et al. in 1972 was followed by a sharp rise of polygraphic studies. The hope to use these as a screening method for an increased risk of SIDS could subsequently not be confirmed by prospective studies. Pathological alterations, such as obstructive apneas and hypoxemias, however, seem to have prognostical relevance. They thus represent a focus of current research even though being unable as well to reliably predict a predisposition to SIDS for an individual subject. Instead of being used as a diagnostic tool for SIDS, polygraphic studies have nowadays acquired an accepted role in the investigation of certain physical symptoms and signs such as unexplained 'apparent life threatening events' (ALTE) or cyanotic attacks. Basic technical requirements for a polygraphic laboratory include facilities to record thoracic/abdominal respiration, nasal air flow, ECG/heart rate, oxygen saturation, and to classify different stages of sleep. Registration of additional parameters (EEG, breath sounds, video etc.) is optional, but may prove helpful to arrive at an appropriate diagnosis. Recently, Austrian and German centres have been trying in cooperation to standardize performance conditions and evaluation criteria for polygraphic measurements in pediatric patients under the guidance of the German Association for Sleep Medicine, Pediatric Workgroup. Their aim is to establish common guidelines for the diagnosis and treatment of sleep and arousal disorders in infancy and childhood.

Apnea↗
PubMed2000

Prospective evaluation of late effects after childhood cancer therapy with a follow-up over 9 years.

UNLABELLED: Intensive multimodality treatment has led to a remarkable improvement of prognosis in paediatric cancer patients, however, a great number of long-term survivors suffer from considerable tumour- or treatment-related late effects. Between January 1990 and December 1998, 223 consecutive survivors of childhood malignancies entered a prospective follow-up study designed to evaluate the frequency and severity of tumour- and/or therapy-related long-term sequelae. After cessation of therapy and subsequently once a year, all patients underwent a detailed examination programme including physical examination, laboratory tests, abdominal sonography, echocardiography, electrocardiography, electroencephalography, spirometry, audiometry, ophthalmological examination and endocrine stimulation tests. Median follow-up was 5 years (range 0.4 to 9.6 years). A total of 167 patients (75%) had at least one chronic medical problem of whom 80 needed permanent medical support. The organ systems most frequently affected were the nervous system in 39%, the endocrine system in 32%, the ears/eyes in 22%, the kidneys in 17%, and the liver in 12% of the patients. Some late effects (endocrine deficits, hearing loss, tubulopathy) were primarily diagnosed only several years after the end of oncological therapy. CONCLUSION: The results of this study indicate that a considerable number of former paediatric cancer patients suffer from remarkable long-term side-effects. Since life quality is an important parameter of cancer survival, careful follow-up of long-term survivors is mandatory with the aim to reduce or even abrogate possible side-effects at the earliest time.

Adolescent↗
PubMed2000

Unrelated peripheral blood stem cell transplantation with 'megadoses' of purified CD34+ cells in three children with refractory severe aplastic anemia.

Three children with refractory severe aplastic anemia were transfused with high numbers of unrelated matched (n = 2) or C-locus haploidentical mismatched (n = 1) CD34-selected peripheral blood stem cells in the absence of an HLA-identical family donor. Two leukaphereses of the donors yielded a median number of 10.1 x 10(10) nucleated cells (range 9.7-15.4) with a median number of 9.89 x 10(8) CD34+ cells (range 7.46-26.1) and a median percentage of CD34+cells of 0.98% (range 0.77-1.7). After positive selection by magnetic cell sorting the patients received a median of 14.3 x 10(6) CD34+ cells/kg (range 11.7-24.3) and of 1.3 x 10(4) CD3+ cells/kg (range 0.57-5.8). Median time to ANC >/=0.5 x 10(9)/l was 7 days (range 7-12) and to platelets >/=20 x 10(9)/l 13 days (range 13-27). Chimerism analysis of peripheral blood after transplantation revealed permanent 100% donor hematopoiesis in all patients. The patient with the C-locus haploidentical mismatch presented with acute GVHD (grade III-IV) of the skin, liver and lower gastrointestinal tract (onset day +40) and died despite intensive immunosuppressive treatment on day +238. The two survivors developed lymphopoietic recovery of B and T lymphocytes within 3 months after transplantation. To our knowledge this experience represents the first report of transplantation with unrelated CD34+ enriched peripheral blood stem cell in children with refractory severe aplastic anemia. Bone Marrow Transplantation (2000) 25, 513-517.

Adolescent↗
PubMed2000

Unrelated partially matched peripheral blood stem cell transplantation with highly purified CD34+ cells in a child with Wiskott-Aldrich syndrome.

Stem cell transplantation is the only curative approach to the treatment of Wiskott-Aldrich syndrome. However, using grafts from partially matched unrelated donors is associated with increased risk of graft rejection and graft-versus-host disease. In an attempt to prevent these problems, a 6-year-old boy with Wiskott-Aldrich syndrome lacking a suitable family donor, was transplanted with large numbers of unrelated highly purified CD34+ peripheral blood stem cells mismatched at one C locus. Conditioning consisted of busulfan 16 mg/kg body weight, cyclophosphamide 200 mg/kg body weight and antithymocyte globulin 20 mg/kg body weight x 3 days. The boy had a rapid hematopoietic engraftment and showed immunologic reconstitution by day +92. Although he did not receive prophylactic immunosuppression he did not develop any graft-versus-host disease and is well and alive up to now, 25 months after transplantation.

Antigens, CD34↗
PubMed2000

Interferon-alpha and ribavirin in treating children and young adults with chronic hepatitis C after malignancy.

OBJECTIVE: Chronic hepatitis C is a major long-term problem for children who survive cancer. Interferon (IFN)-alpha has been shown to be effective in treating patients with chronic hepatitis C; however, the rate of sustained response is low. Combining IFN-alpha and ribavirin (RBV) has been shown to significantly improve the response in adult patients with chronic hepatitis C. The aim of this pilot study was to evaluate the efficacy and safety of a combined virostatic treatment with IFN-alpha and RBV in a small cohort of children and adolescents with chronic hepatitis C and previous malignancy. METHODS: Twelve patients with a history of a hematooncologic disease (median follow-up: 13.5 years; range: 7-14.7 years) and chronic hepatitis C were treated with recombinant IFN-alpha-2a (6 megaunits/m(2) body surface area, 3 times a week, subcutaneously) combined with RBV (15 mg/kg body weight/day, orally) for 12 months. They were tested monthly for blood counts and liver function, and for serum virus concentrations (hepatitis C virus RNA by polymerase chain reaction) every 3 months. RESULTS: At the end of the treatment, hepatitis C virus RNA could not be detected in the serum of 8 of the 12 patients; 2 of these patients relapsed soon after therapy withdrawal, whereas 6 patients maintained in sustained virologic and biochemical remission (follow-up: 12 months). Treatment-induced toxicity was moderate and reversible with influenza-like symptoms and a decrease in blood counts in all 12 patients, alopecia in 5 of the 12, hemolysis in 4 of the 12, and weight loss of >10% in 2 of the 12. CONCLUSIONS: As demonstrated in adults with chronic hepatitis C, treatment with IFN-alpha and RBV also seems to be an effective and safe therapeutic option for children and adolescents with chronic hepatitis C after malignancy.

Adolescent↗
PubMed1999

Transplantation of related and unrelated umbilical cord blood stem cells in Austria. Austrian Working Party for Stem Cell Transplantation. Austrian Society of Hematology and Oncology.

Allogeneic bone marrow transplantation is limited by the availability of suitable HLA-matched donors and the risk of graft versus host disease (GvHD). In an attempt to overcome these limitations umbilical cord blood (UCB), has become a further alternative. UCB transplantations in Austria were started in 1991. As of September 31, 1998, six patients have been transplanted. Diagnoses were severe aplastic anaemia (SAA) (n = 2), acute lymphoblastic leukaemia (ALL) (n = 1), familial hemophagocytic syndrome (FHL) (n = 2) and chronic myelomonocytic leukaemia (CMML) (n = 1). Three patients received UCB grafts from HLA-identical siblings and three patients from unrelated donors, of whom two were disparate at two HLA loci (A/B) and one mismatched at one locus (C). Five patients were engrafted with complete donor hematopoiesis, with a median time of 26.5 days (range 14 to 39 days) to an ANC count of > or = 0.5 x 10(9)/L and a median time of 42.5 days (range 24 to 67 days) to a platelet count of > or = 20 x 10(9)/L. One patient with FHL had partial engraftment and died due to reactivation of cytomegalovirus (CMV) infection and CMV pneumonia on day +25. Of the five patients surviving the post-transplant period, one with CMML had a relapse on day +128 and died after a HLA-matched bone marrow transplantation from the same sibling donor in the second relapse. Another patient with ALL relapsed on day +200 but is still alive under palliative treatment; one patient with SAA showed graft rejection and autologous hematopoietic reconstitution and later had a successful CD34(+)-selected allogeneic peripheral stem cell transplant from a C-locus mismatched unrelated donor. Two patients (one with SAA and one with FHL) are alive with complete remission of the underlying disease. This report reflects the experience and results of UCB transplantation in Austria and discusses the position of UCB transplantation in the context of the other stem cell alternatives available today.

Anemia, Aplastic↗
PubMed1999

Comparison of different methods for separation and ex vivo expansion of cord blood progenitor cells.

Umbilical cord blood is capable of hematopoietic stem cell reconstitution in children. However, the major limitation of cord blood is a relatively low content of pluripotent progenitor cells. Thus, safe engraftment for adolescents and for adults is still not predictable and a technology for ex vivo expansion of umbilical cord blood cells is desirable. In a first step, four different methods of red cell depletion followed by magnetic cell sorting of CD34+ cells were evaluated in this study in order to assess the efficacy and safety of optimal stem cell recovery. A modified two-step Ficoll gradient separation and a hydroxyethyl starch separation tended to produce a better WBC/MNC recovery (median 94.2+/-2.44% vs. 90.2+/-5. 8%) as compared with standard Ficoll gradient separation and a gelatin-based procedure (median 78.35+/-7.1% vs. 67.2+/-5.5%). However, the recovery of CD34+ cells after magnetic cell sorting did not reach a statistically significant difference after the four different methods of red cell depletion, indicating that the recovery of WBC/MNC is not predictably correlated with the recovery of stem cells within these fractions. In a second step, we established three different cytokine combinations by adding the megakaryocyte growth and development factor +/- erythropoietin and granulocyte colony-stimulating factor to a fetal calf serum containing medium with Flt 3, stem cell factor, and interleukin-3. Net expansion of total colony-forming cells 20- to 50-fold and expansion of colony-forming cells after 5 weeks of culture 1.5- to 3-fold were obtained over a period of 7-14 days. These results demonstrate that cord blood stem cells can be expanded substantially in this short-term culture system.

Antigens, CD34↗
PubMed1999

ABL amplification in a patient with lymphoid blast crisis of chronic myelogenous leukaemia.

Although chronic phase myelogenous leukaemia (CML) is characterised by the Philadelphia (Ph) chromosome leading to a fusion of the BCR and ABL genes, additional genetic alterations involved in blast crisis are poorly understood. We report an at least 15-fold amplification of the ABL oncogene in a 29-year-old male patient with a variant Ph-positive t(19;22)(p13;q11.2) CML who presented in lymphoid blast crisis. Our finding suggests that an amplification of the ABL oncogene might play a part in the appearance of an aggressive phenotype in some cases of CML.

Adult↗