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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Monatsschrift Kinderheilkunde 146 (1998), S. 603-607 
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Hypernatriämie ; Hypernatriämie-Hypodypsie-Syndrom ; Arachnoidalzyste ; Stereotaxie ; Kinder ; Key words Hypernatremia ; Hypodipsic-hypernatremia-syndrome ; Arachnoid cyst ; Stereotactic surgery ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Background: In rare cases a hypernatremia is caused by cerebral dysregulation. Case: We describe a 3,5 year old girl who had a hypodipsic-hypernatremia-syndrome (serum sodium concentration 194 mmol/l) caused by an arachnoid cyst in front of the pons. Therapy: In a stereotactic puncture a catheter was implanted. Aspiration of the cyst liquid did reduce the size of the cyst. For two years now no other treatment is given. Hypernatremia did not occur again. Discussion: To our knowledge this is the first case of a hypodipsic-hypernatremia-syndrome caused by an arachnoid cyst, which was cured by a single operation.
    Notes: Zusammenfassung Hintergrund: In seltenen Fällen werden Hypernatriämien durch zerebrale Regulationsstörungen verursacht. Fall: Wir berichten von einem 3,5 Jahre alten Mädchen, bei dem ein Hypernatriämie-Hypodypsie-Syndrom (Serumnatrium 194 mmol/l) durch eine präpontine Arachnoidalzyste verursacht wurde. Therapie: Durch eine stereotaktische Operation wurde ein Katheter implantiert. Der Zysteninhalt wurde aspiriert und die Zyste dadurch verkleinert. Seit 2 Jahren trat ohne medikamentöse Therapie keine Hypernatriämie mehr auf. Diskussion: In diesem Fall konnte nach unserem Wissen erstmals ein Hypernatriämie-Hypodypsie- Syndrom, das durch eine Arachnoidalzyste verursacht wurde, durch eine Operation geheilt werden.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0584
    Keywords: Acute leukemia ; (4; 11) chromosome translocation ; Early B-precursor cell origin ; Mixed lineage leukemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Clinical and laboratory features of seven patients with acute leukemia associated with the (4; 11) chromosome translocation are presented. Leukemic blasts of these patients showed lymphoid morphology in 6 (although 1 was treated for monoblastic leukemia 3 years earlier) and monocytoid morphology in 1, were positive for TdT and HD 37 (CD 19) in 6 patients, whereas weak expression of CALLA was seen in only 1 patient and T-lineage-associated antigens in none. Leukemic blasts from four patients showed the simultaneous expression of B-lymphoid and myeloid antigens, suggesting leukemogenesis in a very early multipotent progenitor cell. In 2 patients an isochromosome of the long arm of No. 7 chromosome was found in the leukemic karyotypes in addition to t (4; 11) (q21; q23); in one instance present at diagnosis, in the other one occurring at relapse. In one other patient leukemia karyotype also demonstrated trisomy 8. Leukemic cells of three patients were investigated by molecular genetics and demonstrated immunoglobulin gene rearrangements for the Ig heavy chain sequences but not for the light chain constant regions and T cell receptor sequences. All patients were treated by intensive chemotherapy. Four of the 7 patients are in continuous complete remission. The longest event-free survival time (over 2 1/2 years) was seen in one patient who had also DOWN-syndrome. Including these 7 patients a clinical analysis of 71 patients with t (4; 11) acute leukemia was made, emphasizing the following characteristics at diagnosis: female sex (62%), age under 2 years (49%), leukocyte count over 100×109/1 (61%), splenomegaly (80%), CNS-disease (11%). Survival of over 2 years was reported in less than 15% of the patients. It remains to be seen if risk-adapted treatment can alter the course of this early B-precursor acute leukemia with hitherto very bad prognosis.
    Type of Medium: Electronic Resource
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