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  • Articles: DFG German National Licenses  (6)
  • 1975-1979  (6)
  • 1
    ISSN: 1432-1440
    Keywords: Eisenabsorption ; Reserveeisen ; Eisenmangel ; zystische Fibrose ; Pankreatin ; Hämosiderose ; Iron absorption ; Storage iron ; Iron deficiency ; Cystic fibrosis ; Pancreatin ; Hemosiderosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The absorption of59Fe from a diagnostic 0.56 mg59Fe2+ dose was measured by 4π-geometry whole body counting and related to the amount of stainable diffuse cytoplasmatic non heme storage iron in the bone marrow macrophages of children with cystic fibrosis. When the storage iron was within the normal range (+/2+) children with cystic fibrosis absorbed 10–38% ( $$\bar X_a $$ a±S.D.=21±9.3) of the oral59Fe2+ dose which is identical with the59Fe-absorption in normal children with normal iron stores (9–45:23±8.7%). Depleted iron stores caused an increase of59Fe-absorption to 43–95% ( $$\bar X_a $$ a±S.D.=62±19) in children with cystic fibrosis and to 45–100% ( $$\bar X_a $$ a±S.D.=73±18) in control children. The interruption or continuation of pancreatin maintenance therapy and the simultaneous administration of 1–1.5 g pancreatin did not influence59Fe2+ absorption in cystic fibrosis. There is no evidence for a pancreatic factor required for or inhibiting inorganic and food iron absorption in human beings. Iron absorption is controlled also in cystic fibrosis chiefly by the amounts of available storage iron. It is therefore not justified to apprehend the development of hemosiderosis in children with cystic fibrosis who are not or not sufficiently treated with pancreatin.
    Notes: Zusammenfassung Bei Kindern mit zystischer Pankreasfibrose wurde die Eisenabsorption aus der diagnostischen 10 µMol (=0.56 mg)59Fe2+-Dosis durch Gesamtkörper-59Fe-Retentionsmessung in einem 4π-Meßgeometrie Gesamtkörper-Radioaktivitätsdetektor bestimmt und in Relation gesetzt zu dem mit der Berliner Blau-Reaktion in den Knochenmarks-Makrophagen anfärbbaren, diffus im Cytoplasma verteilten Nichthäm-Reserveeisen. Kinder mit zystischer Fibrose und normalen Eisenreserven (+/2+) absorbierten mit 10–38 ( $$\bar X_a $$ a±S.D.=21±9,3%) ebensoviel59Fe wie gesunde Kinder mit normalen Eisenreserven (9−45=23±8,7%). Die Erschöpfung der Eisenreserven im prälatenten/latenten Eisenmangel verursachte einen Anstieg der59Fe2+-Absorption auf 43–95 ( $$\bar X_a $$ a+S.D.=62±19%) bei den Kindern mit zystischer Fibrose bzw. auf 45–100 ( $$\bar X_a $$ a±S.D.=73±18%) bei den gesunden Kindern. Die Unterbrechung der Pankreatin-Substitutionstherapie sowie die gleichzeitige orale Verabfolgung von 1–1,5 g Pankreatin zeigten keinen Effekt auf die59Fe2+-Absorption bei den Kindern mit zystischer Fibrose. Es ergab sich kein Anhalt für die Existenz eines Pankreasfaktors, der entweder für die Eisenabsorption beim Menschen benötigt wird oder aber diese hemmen könnte. Auch bei exokriner Pankreasinsuffizienz infolge zystischer Fibrose wird das Ausmaß der intestinalen Eisenabsorption durch die Mengen an verfügbarem Reserveeisen bestimmt. Es besteht daher keine Veranlassung zu der geäußerten Befürchtung der Entwicklung einer Eisenabsorptions-Hämosiderose bei nicht oder nicht ausreichend mit Pankreatin substituierten Kindern mit zystischer Fibrose.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1440
    Keywords: Absorption ; Nahrungseisen ; Hämoglobin ; Fleisch ; Leber ; Vitamin B12 ; Selenomethionin ; Zystische Fibrose ; Pankreasinsuffizienz ; Maldigestion ; Eisenmangel ; Reserveeisen ; Pankreatin ; Intrinsic Factor ; Absorption ; Food iron ; Hemiglobin ; Meat ; Liver ; Vitamin B12 ; Selenomethionine ; Cystic fibrosis ; Pancreatic insufficiency ; Malabsorption ; Iron deficiency ; Iron stores ; Pancreatin ; Intrinsic factor
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The absorption of biosynthetically59Fe-labeled hemiglobin, pork and hog liver,60Co-vitamin B12 and75Se-selenomethionin labeled pork was measured in children with cystic fibrosis by whole body counting of the absorbed radionuclides within the 4π geometry of a large volume radioactivity detector with liquid organic scintillator. Children with normal or mildly reduced iron stores absorbed 6–13% of the 5 mg hemiglobin-iron, 7–14% of the 5 mg liver-iron and 17–34% of the pork-iron whereas children with depleted iron stores absorbed 19% of the hemiglobin-59Fe, 13 and 28% of the liver-59Fe and 23 and 30% of the pork-59Fe. These absorption values are all within the ranges of hemiglobin-, liver- and pork-59Fe absorption described for healthy adults with normal and depleted iron stores, respectively. Pancreatic proteolytic digestion is obviously not necessary for the intraluminal release preceeding the absorption of dietary heme- and non heme-iron from hemiglobin, meat and liver, respectively. An increased absorption of hemiglobin-, liver-and pork iron is caused by depleted iron stores also in children with cystic fibrosis. An intrinsic factor non-responsivevitamin B 12-malabsorption was observed in all investigated children with cystic fibrosis and confirms the requirement of a pancreatic proteolytic enzyme (trypsin) for the vitamin B12-intrinsic factor binding reaction or the fixation of the vitamin B12-intrinsic factor complex to the ileal receptors. Children with cystic fibrosis absorbed 60 and 71% of the75Se from an oral dose of 200 g75 Se-selenomethionine labeled pork, whereas normal adults absorbed 76–100% ( $$\bar X_1 $$ 1±S.D.=87±3.5) of the75Sc. Pancreatin did not improve the bioavailability of75Se-selenomethionine from labeled pork in cystic fibrosis which is just below the normal range. Intraluminal peptic digestion of dietary protein seems to be adequate in pancreatic insufficiency and pancreatin treatment did not improve protein utilization in cystic fibrosis
    Notes: Zusammenfassung Die Absorption des59Fe aus biosynthetisch markiertem Schweine-Hämoglobin-(59Fe), Leber-(59Fe) und Fleisch-(59Fe), des60Co aus Vitamin B12-(60Co) und des75Se aus Selenomethionin-(75Se)-markiertem Schweinefleisch wurde bei Kindern mit zystischer Fibrose durch Gesamtkörper-retentionsmessung der absorbierten Radionuklide in einem Großraum-Radioaktivitätsdetektor mit flüssigem organischem Szintillator und 4π-Meßgeometrie bestimmt. Kinder mit zystischer Fibrose und normalen oder nur leicht reduzierten Eisenreserven absorbierten 6–13% aus 5 mg Hämoglobin-Eisen (=1,44 g Hämoglobin), 7–14% aus 5 mg Leber-Eisen (=25 g Leber) und 17–34% aus 0,7–2,0 mg Fleisch-Eisen (=70–200 g Fleisch), während bei Erschöpfung der Eisenreserven eine59Fe-Absorption von 19% aus Hämoglobin, 13 und 28% aus Leber und 23 und 30% aus Fleisch gemessen wurde. Alle diese Eisenabsorptionen liegen innerhalb der Normalbereiche der Nahrungseisenabsorption, die bei gesunden Menschen mit normalen bzw. erschöpften Eisenreserven ermittelt worden waren. Offensichtlich ist die proteolytische Verdauung durch die Pankreasprotease nicht wesentlich für die der Absorption im oberen Dünndarm vorgeschaltete intraluminäre Freisetzung des Hämund Nichthäm-Eisen aus Hämoglobin, Fleisch und Leber. Eine erhöhte Nahrungseisenabsorption wird auch bei Kindern mit zystischer Fibrose durch die Erschöpfung der Eisenreserven verursacht. Eine Intrinsic Factor-nichtresponsive Vitamin B12-Malabsorption wurde bei allen untersuchten Kindern mit zystischer Fibrose festgestellt und ist möglicherweise auf die Notwendigkeit eines proteolytischen Pankreasenzymes (Trypsin?) für die Bindung des Vitamin B12-Intrinsic Factor-Komplexes an die Ileumreceptoren zurückzuführen. Die Absorption des75Se aus Selenomethionin-(75Se)-markiertem Schweinefleisch lag mit 60 und 71% bei Kindern mit zystischer Fibrose nur geringfügig unter dem bei gesunden Erwachsenen gemessenen Bereich der Selenomethionin-(75Se)-Absorption aus 200 g Fleisch (76–100%; $$\bar X_a $$ a±S.D.=87±3.5). Durch 3 g Pankreatin wurde die Bioverfügbarkeit des Selenomethionin-(75Se) aus Fleisch bei Kindern mit zystischer Fibrose nicht beeinflußt. Bei Kindern mit zystischer Pankreasfibrose reicht die peptische Verdauung der Nahrungsproteine für die Bioverfügbarkeit der Aminosäuren wohl noch aus, so daß die anschließende Spaltung durch Pankreasproteasen bzw. eine Substitution von Pankreasproteasen nicht mehr erforderlich ist.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Keywords: Hemoglobin iron absorption ; Iron supplementation ; Milk iron ; Iron stores ; Iron deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Small amounts of milk do inhibit ferrous iron absorption from a 5 mg 59Fe2+ dose in 1- to 18-month-old infants. Only 50 ml of 2/3 cow milk reduced the absorption from 18±3% (Xa ± S.E.) to 3.8 ± 1.2% in infants with normal iron stores (inhibition index 0.21) and from 26±3% to 8.5±1.4% in infants with depleted iron stores (inhibition index 0.33%). Milk does not inhibit the biovailability of hemoglobin iron. From a 5 mg dose of hemoglobin-59Fe added to 50 ml of 2/3 cow milk 4.8±1.0% were absorbed by infants with normal iron stores and 8.3±0.8% by infants with depleted iron stores. The low iron content of milk (50 μg Fe/100 ml) and its poor biovailability (∼5% in infants with normal iron stores) would require a daily consumption of 32 l of unfortified milk to cover infants daily iron requirement of 0.8 mg/day. The supplementation of 2–3 milk meals per day with 5 mg hemoglobin iron each meets the whole iron requirement of infants with depleted and normal iron stores respectively and can be used for iron prophylaxis in infancy during the first and second year. Prophylaxis with inorganic iron requires an empty stomach and duodenum for optimal bioavailability. A daily dose of only 5 mg ferrous sulfate iron is enough to cover the total iron requirement of infants.
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  • 4
    ISSN: 1432-1076
    Keywords: Hemolytia anemia ; Erythroblastic hyperplasia ; Iron absorption ; Storage iron ; Iron deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A high negative correlation (coefficient ∼ 0.9) between increased 59Fe absorption from a diagnostic 0.56 mg 59Fe2+ dose and the depletion of available storage iron was observed in menstruating and pregnant women, fullterm and premature infants, blood donors, patients with infections, inflammations, tumors, hepatic cirrhosis, gastric surgery, increased urogenital or gastrointestinal blood loss. The increased diagnostic 59Fe2+ absorption is a reliable and sensitive indicator of at least depleted iron stores or prelatent iron deficiency as caused by iron malnutrition or maldigestion, increased iron requirement in pregnancy, infancy, urogenital or gastrointestinal blood loss. Although the messenger system which signalyzes the depletion of iron stores to the iron absorbing enterocytes of the duodenal and jejunal mucosa is not yet known available storage iron seems to control intestinal iron absorption under normal and the great majority of pathological conditions in humans. Anemia per se or high erythropoietin levels in blood do not influence iron absorption since patients with even severe erythroblastic hypoplasia, aplastic anemia and megaloblastic anemia due to vitamin B12 deficiency absorb iron according to their iron stores. An only mild hyperplasia of the erythropoietic system in the bone marrow does also not effect iron absorption which was still under the control of available storage iron in patients with hereditary spherocytosis, nonspherocytic congenital hemolytic anemia due to glucose-6-phosphate dehydrogenase deficiency, acquired hemolytic anemia and vitamin B12 deficiency induced megaloblastic anemia. An exception from the general rule that depleted iron stores do cause increased iron absorption, which can be used therefore for the reliable diagnosis of depleted iron stores or prelatent iron deficiency, was observed only in anemic children with hereditary nonspherocytic enzymopenic hemolytic anemia due to pyruvate kinase deficiency. In these cases a strong normoblastic hyperplasia correlated well with the increased iron absorption in the presence of normal amounts of available storage iron. Blood transfusions which suppress the normoblastic hyperplasia do also reduce the increased iron absorption to normal levels. A similar iron absorption increasing effect of hyperplastic ineffective erythrocytopoiesis was also observed in adults with sideroblastic anemia and children with severe homozygous β-thalassemia although the iron stores of these patients were normal or increased. The messenger system which is used by the body for signalyzing severe normoblastic hyperplasia to the iron absorbing intestinal mucosa and induces a useless and even dangerous augmented iron absorption is not yet known. Since the diseases with severe normoblastic hyperplasia are extremely rare and easily to diagnose the increased intestinal iron absorption from the diagnostic 0.56 mg 59Fe2+ dose is a very useful, reliable and sensitive indicator of already the earliest stage of iron deficiency.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0584
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Ein Junge mit schwerer Knochenmarkaplasie und ein Mädchen mit rezidivierender akuter lymphatischer Leukämie wurden nach entsprechender immunsuppressiver und antileukämischer Vorbehandlung mit Knochenmark von HL-A-identischen, MLC-negativen Geschwistern transplatiert. Beide Kinder sind 7 bzw. 2 Monate nach Knochenmarkaplasie oder akuter lymphatischer Leukämie im Rezidiv in Betracht gezogen werden, wenn HL-A-identische, MLC-negative Geschwister zur Verfügung stehen.
    Notes: Summary A boy with severe Aplastic Anemia (AA) and a girl with Acute Lymphoblastic Leukemia (ALL) in relapse have been grafted with marrow from HL-A identical, mixed leukocyte culture (MLC) negative siblings after appropriate immunosuppressive and antileukemic therapy. Both of them are well 7 and 2 months after transplantation respectively. Bone marrow transplantation should be considered in children with AA and ALL in relapse, if HL-A identical, MLC negative sibling are available.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0584
    Keywords: Acute lymphatic leukaemia ; T cell antigen ; E-receptor ; C3-receptor ; Acute undifferentiated leukaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The lymphoblasts from 100 patients with acute lymphocytic leukaemia were investigated for the expression of receptors for sheep erythrocytes (E) and of a specific heterologous T cell antigen (T). In 17 cases, both T cell markers were expressed simultaneously on the leukaemic cells. In 13 cases only T antigens could be demonstrated on the lymphoblasts. A quantitative analysis of T antigens by immunoautoradiography revealed that the T expression of E−T+-lymphoblasts was in general like that of E+T+-lymphocytes in the blood of normal persons, in several cases even higher. Therefore, the failure of E-rosette formation cannot be correlated to a decrease of the other T cell differentiation marker. In 7 out of 9 tested cases, a strong acid phosphatase reaction product located paranuclearly could be demonstrated. Complement-receptors were expressed in 3 of 5 cases which were also demonstrated in some cases of the E+T+-ALL group. The latter group was characterized by a T antigen expression like that of thymocytes. 4 cases of the E−T+ALL group were adults. Since the leukaemia cells of 2 cases were negative for acid phosphatase, PAS and all surface markers including cALL antigen, the T antigen can classify undifferentiated and otherwise unclassificable leukaemias. The clinical significance of the E−T+-ALL seems to be important since 5 out of 9 children with this type of ALL died soon after diagnosis.
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