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  • 1
    ISSN: 1432-1440
    Keywords: Recurrent infections ; Compromised host ; Granulocyte dysfunction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Several granulocyte functions were analyzed in vitro in 154 patients with chronic or recurrent infections, as well as in a variety of disorders known or suspected to affect host resistance. Only a few specific abnormalities were diagnostic and occurred in congenital, hereditary disorders. Opposed to these permanent changes are those which were probably acquired or transient and are often multifactorial in origin. In the majority of these patients, an inconstant and nonspecific pattern emerged which is not helpful in the diagnosis of underlying disease. In selected patients, however, and as research procedures, these and related tests should be helpful in elucidating the basic functions of granulocytes and may implicate therapeutical approaches.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1440
    Keywords: Bone marrow transplantation ; Aplastic anaemia ; Acute leukaemia ; Chronic granulocytic leukaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary From 1972–1983 53 patients underwent bone marrow transplantation. The median age was 18 years (3–41). 27 patients suffered from severe aplastic anaemia, 22 patients had acute leukaemia and 4 patients had chronic granulocytic leukaemia in chronic phase. Out of 22 patients with acute leukaemia, 2 had florid leukaemia, 2 had an early relapse and 18 patients were in first or second remission of their disease. 2/53 patients received a syngeneic transplant, 51/53 patients an allogeneic transplant. 47/51 patients had a HLA-A, B, C-identical, MLC-negative sibling donor, 1/51 had a HLA-A, B-C-identical, MLC-positive sibling donor, 2/51 a HLA-phaenotypical identical parental donor and 1/51 a HLA-identical, MLC-negative unrelated donor. The comparison of the results obtained in patients with severe aplastic anaemia transplanted from 1972–1979 with those transplanted from 1980–1983 shows that the bone marrow transplantation has to be performed in an early stage of the disease before the patients become multiple transfused, sensitized and severely infected and that the conditioning regimen for polytransfused patients has to be more intensive than in untransfused patients. From the patient group transplanted 1972–1979, only 1/14 patients is a long-term survivor in contrast to 8/13 patients transplanted from 1980–1983. 11/22 patients with acute leukaemia are alive between more than 5 years and 14 days after bone marrow transplantation. Only 1/4 patients, who were transplanted not in remission, is alive. For patients with acute leukaemia the bone marrow transplantation should be performed in an early stage of their disease when the tumor burden is small and when the patients are in good clinical condition. 2/4 patients with CGL are alive between 12 months and 3 months after bone marrow transplantation. In our patient group graft versus host disease was the most important problem with a high mortality due to GvHD associated infections.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 135 (1981), S. 261-266 
    ISSN: 1432-1076
    Keywords: Neutropenic children ; Granulocyte transfusions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Fifteen children with haematological diseases received an average of 10 granulocyte transfusions each, with 4.7×1010 granulocytes per m2 body surface area (BSA) per transfusion. All patients except one had less than 100 granulocytes per μl blood combined with aplasia or hypoplasia of granulopoiesis. Thirteen patients were transfused therapeutically because of fever and/or severe local lesions unresponsive to antibiotic therapy. Two patients, who were transfused prophylactically after bone-marrow transplantation (BMT), were in excellent clinical condition during the entire period of granulocyte transfusions in spite of severe granulocytopenia. All patients except one showed definite clinical benefit from granulocyte transfusions. The non-responding patient had leukocyte antibodies.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1076
    Keywords: Diabetes mellitus type I ; Initial metabolic decompensation ; Duration of remission ; HbA1c
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In 21 newly diagnosed children with type I diabetes mellitus initial hemoglobin A1c-concentrations, mean insulin requirements during the first 10 days of treatment to recompensate carbohydrate metabolism, duration of glucosuria after diagnosis and duration of remission were determined. Initial hemoglobin A1c-concentration and both mean insulin requirement during the first 10 days of treatment and duration of initial glucosuria showed a highly significant positive correlation. A highly significant, negative correlation was found between the duration of remission and both the mean insulin requirement during the first 10 days of treatment and the duration of initial glucosuria. Thus the present results together with previous findings suggest that the severity of initial metabolic decompensation in diabetes mellitus type I seems to determine at least in part the duration of remission.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 133 (1980), S. 5-10 
    ISSN: 1432-1076
    Keywords: Preleukemia ; Hematopoietic dysplasia ; Refractory anemia ; Myeloproliferative disease ; Acute nonlymphocytic leukemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The preleukemic syndrome or hematopoietic dysplasia is a marrow stem-cell disorder with clinically recognizable hematologic abnormalities which precede the development of acute nonlyphocytic leukemia. Its occurrence in childhood is extremely rare; seven “true” cases who fulfill all the criteria for the disorder have been reported until now. The preleukemic syndrome is generally characterized by peripheral cytopenia with fairly specific morphologic abnormalities in cell differentiation. The hematological and clinical features permit recognition of preleukemia even before the development of overt leukemia. Experimental data indicate that preleukemia is an “early” leukemic syndrome in which hematopoietic cell differentiation becomes progressively impaired with termination in the nearly complete maturation block which is characteristic of acute myelogenous leukemia.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Chromosomal DNA from three individuals with familial hemoglobin M (Hb M) Milwaukee was studied by restriction endonuclease analysis. The segregation of the mutant β-globin gene could be followed through three generations by direct Sst I analysis at the gene level. Various restriction endonucleases were used to confirm the positions of Sst I sites in the δ-βA and δ-βMi-globin gene regions.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Annals of hematology 40 (1980), S. 161-164 
    ISSN: 1432-0584
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Annals of hematology 44 (1982), S. 379-380 
    ISSN: 1432-0584
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Annals of hematology 46 (1983), S. 57-59 
    ISSN: 1432-0584
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Restriction endonuclease mapping of cellular DNA has been used to identify chromosomes that carry the mutant Hb Presbyterian β-globin genes in a family with individuals heterozygous for this disease. The presence of the polymorphic Hind III restriction site in the Gγ-globolin gene and its absence in the Aγ-globolin gene were shown to be in phase with the Hb Presbyterian mutation yielding a haplotype constellation that is diagnostic for any further affected offspring.
    Type of Medium: Electronic Resource
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