Klinische Pädiatrie
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Klinische Pädiatrie · Jan 2002
Case Reports[Hereditary deficiency of C1-esterase inhibitor presenting with recurrent abdominal pain].
Hereditary deficiency of C1-esterase inhibitor (C1-INH) which clinically manifests as hereditary angioedema is a rare disorder. In previously not diagnosed cases, a fatality rate of up to 30 % has been reported. The diagnosis of C1-esterase inhibitor deficiency should not be missed in patients presenting with angioedema in the face, stem or extremities. We report the case of a 17-year-old girl with recurrent abdominal pain and swelling of the hands.
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The telemedicine project of the Competence Net Pediatric Oncology of the German/Austrian Gesellschaft für Pädiatrische Onkologie und Hämatologie (GPOH) has as an initial step of its work-programme sent out a questionnaire to the 54 largest pediatric hematology/oncology units in Germany. Institutions were asked for their experience, motivation, existing infrastructure, and anticipated benefits and obstacles regarding the implementation of telemedicine in patient care and research. Of the 54 largest German Pediatric Oncology institutions asked, 46 completed the questionnaire (85 %). ⋯ 1. The need for further detailed information on implementation and for help in technical realization of telemedicine applications was expressed by all participants. 2. The majority expected practical advantages from telemedicine communication and anticipated that telemedicine will increase quality in treating children with cancer. 3. Expert consultation (study chairman, reference radiologists) is stated as to be most important. 4. Thirty-three of 46 physicians (72 %) believe that telemedicine will reduce costs in medical care within the next years. 5. It is anticipated that the introduction of telemedicine is time consuming. 6. The lack of available medical informatics competence and manpower was regarded as the most important obstacle. 7. Data security and standardization, transfer speed and transmission quality are considered most important. 8. Most of the institutions (91 %) use computers in the management of patients. Fourty-four (96 %) are connected to the Internet. 9. Thirty-seven of 46 institutions were prepared to invest in the implementation of telemedicine. This analysis demonstrates that the use of telemedicine is expected to become standard in pediatric oncology, while the existing infrastructure and status of information regarding this subject at present are insufficient. The most pressing practical need for telemedicine applications is seen in the field of electronic expert consultation. Hence, the Telemedicine-Project of the GPOH Competence Net will focus on this aspect first.
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Klinische Pädiatrie · Sep 2001
Review[Guidelines for Prevention of Pneumocystis carinii Pneumonitis in Children and Adolescents with Cancer].
Pneumocystis carinii pneumonitis (PCP) is one of the most important opportunistic infections in children and adolescents with cancer. Its high frequency and a considerable mortality have led to primary chemoprophylaxis in patients with hematological malignancies and following allogeneic hematopoietic stem cell transplantation. Although less well characterized, patients with autologous stem cell transplantation and patients with dose-intensive chemotherapy for pediatric solid tumors may have a similarly high risk for PCP based on their profound T-cell depletion. ⋯ Secondary alternatives to TMP/SMX include oral dapsone, oral atovaquone, and aerosolized pentamidine-isethionate. These modalities are less effective than TMP/SMX, and have been evaluated predominantly in HIV-infected patients. This article reviews epidemiology and current approaches to chemoprophylaxis for PCP in children and adolescents with cancer and/or hematopoietic stem cell transplantation, and provides evidence-based guidelines for indications and modalities of PCP prophylaxis in this population.
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Klinische Pädiatrie · Sep 2001
Review Comparative Study[Prevention of fungal infections in children and adolescents with cancer].
Opportunistic mycoses have emerged as important causes for morbidity and mortality in pediatric cancer patients, particularly in those with intensively treated hematological malignancies, allogeneic hematopoetic stem cell transplantation, and aplastic anemia. The incidence of invasive fungal infections in these settings may range from 10 to 25 % despite empirical antifungal therapy with an overall case fatality rate of up to 50 and 75 % depending on the organism. ⋯ However, its benefit in other high-risk populations is less well established, and a clearly effective approach to chemoprophylaxis for invasive Aspergillus infections has not been documented in appropriately designed clinical trials. This article reviews epidemiology and current approaches to chemoprophylaxis of opportunistic invasive fungal infections in children and adolescents with cancer and/or stem cell transplantation, and provides evidence-based guidelines for indications and modalities of antifungal prophylaxis and antifungal infection control measures in this population.
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Asplenia in childhood may be congenital (e.g. Ivemark-syndrome) or acquired (functional hyposplenism in sickle cell disease; after splenectomy or bone marrow transplantation). Hereditary spherocytosis is the most common indication for splenectomy in childhood. ⋯ Due to this fact the indication for splenectomy in childhood should be restricted to patients with hematologic disease (spherocytosis and other hemolytic anemias, chronic ITP etc.) and moderate to severe symptoms. It is unclear whether partial splenectomy for spherocytosis (and other hemolytic anemias) is an alternative regarding both longlasting reduction of hemolysis and prevention of severe infection. After trauma every effort should be undertaken to preserve a splenic remnant.