Skip to main content
SpringerLink
Log in

Akrodermatitis enteropathica — eine Zinkstoffwechselstörung mit Zinkmalabsorption

Acrodermatitis enteropathica — A disturbance of zinc metabolism with zinc malabsorption

  • Published:
Zeitschrift für Kinderheilkunde Aims and scope Submit manuscript

Abstract

The intestinal resorption of zinc using 65ZnCl2 was estimated in 3 patients with acrodermatitis enteropathica, 2 healthy controls, and 3 heterozygotes. After oral application of 65Zn the whole body activity was measured by a whole body counter for 34 days. The 65Zn resorption of the patients amounted to 16, 42 and 30% of the applied dose, whereas the resorption values of the heterozygotes and the controls were in the range of 58 and 77%. The elimination of 65Zn from the body amounted to about 0.7% of the applied dose with no difference between controls and patients with acrodermatitis enteropathica. Before therapy the serum-zinc levels of patients were markedly decreased. After oral application of high doses of zinc aspartate (2×400 mg/day) all clinical symptoms disappeared within a week.

The results point at a causal connection between zinc and the pathogenesis of acrodermatitis enteropathica. Ultrastructural alterations of the Paneth cells of the intestine are also shown in this disease [12] as have also been seen in Paneth cells of zinc deficient rats [Beitr. Path. 145, 336 (1972)].

Zusammenfassung

Bei 3 Patienten mit Akrodermatitis enteropathica wurde mit Hilfe der Ganzkörpermessung nach oraler Applikation von 65Zn eine verminderte intestinale Zinkresorption gedunden. Dagegen war die Zinkelimination aus dem Körper normal. Die Zinkkonzentration im Serum war bei den Patienten stark erniedrigt. Alle klinischen Symptome verschwanden nach sehr hohen oralen Zinkdosen. Diese Befunde sprechen um so mehr für eine ursächliche Rolle des Zinks in der Pathogenese der Akrodermatitis enteropathica, als bei dieser Krankheit ultrastrukturelle Veränderungen in den Panethschen Zellen nachzuweisen sind [12], die auch beim Zinkmangel der Ratten gefunden wurden [Beitr. Path. 145, 336 (1972)].

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

Literatur

  1. McCance, R. A., Widdowson, E. M.: The absorption and excretion of zinc. Biochem. J. 36, 692 (1942)

    Google Scholar 

  2. Cash, R., Berger, C. K.: Acrodermatitis enteropathica. Defective metabolism of unsaturated fatty acids. J. Pediat. 77, 717 (1969)

    Google Scholar 

  3. Danbolt, M., Closs, K.: Acrodermatitis enteropathica. Acta derm.-venereol. (Stockh.) 23, 127 (1942)

    Google Scholar 

  4. Evans, G. W., Grace, C. J., Votava, H. J.: A proposed mechanism for zinc absorption in the rat. Amer. J. Physiol. 228, 501 (1975)

    Google Scholar 

  5. Halsted, J. A., Ronaghy, H. A., Abadi, P., Haghshenass, M., Amirhakemi, G. H., Barakat, R. M., Reinold, J. G.: Zinc deficiency in man. The Shiraz experiment. Amer. J. Med. 53, 277 (1972)

    Google Scholar 

  6. Hambidge, K. M., Hambidge, C., Jacobs, M., Baum, J. D.: Low levels of zinc in hair, anorexia, poor growth, and hypogeusia in children. Pediat. Res. 6, 868 (1972)

    Google Scholar 

  7. Hansson, O.: Acrodermatitis enteropathica. Report of two cases with a hypothesis concerning the pathogenesis of the disease. Acta derm.-venereol. (Stockh.) 43, 456 (1963)

    Google Scholar 

  8. Hurley, L. S., Mutch, P. B.: Prenatal and postnatal development after transitory gestational zinc deficiency in rats. J. Nutr. 103, 649 (1973)

    Google Scholar 

  9. Kasperek, K., Schicha, H., Siller, V., Feinendegen, L. E.: Normalwerte von Spurenelementen im menschlichen Serum und Korrelation zum Lebensalter und zur Serum-Eiweiß-Konzentration. Strahlentherapie 143, 468 (1972)

    Google Scholar 

  10. Kasperek, K., Lombeck, I., Feinendegen, L. E., Bremer, H. J.: Serum zinc concentration of various ages. Clin. chim. Acta (in press)

  11. McKenzie, J. M.: Urinary excretion of zinc, cadmium, sodium, potassium, and creatinine in ninety-six studients. Proc. Univ. Otago med. Sch. 50, 16 (1972)

    Google Scholar 

  12. Lombeck, I., von Bassewitz, D. B., Becker, K., Tinschmann, P., Kästner, H.: Ultrastructural findings in acrodermatitis enteropathica. Pediat. Res. 8, 82 (1974)

    Google Scholar 

  13. Lombeck, I., Ritzl, F., Schnippering, H. G., Michael, H., Bremer, H. J.: Feinendegen, L. E., Kosenow, W.: Primary hypomagnesemia. I. Absorption studies. Z. Kinderheilk. 118, 249 (1975)

    Google Scholar 

  14. Michaelsson, G.: Zinc therapy in acrodermatitis enteropathica. Acta derm.-venereol. (Stockh.) 54, 377 (1974)

    Google Scholar 

  15. Moynahan, E. J., Barnes, P. M.: Zinc deficiency and a synthetic diet for lactose intolerance. Lancet 1973 I, 676

  16. Moynahan, E. J.: Le zinc et le poil dans l'acrodermatitis enteropathica. Bull. Soc. franç. Derm. Syph. 80, 541 (1973)

    Google Scholar 

  17. Moynahan, E. J.: Acrodermatitis enteropathica: A lethal inherited human zinc-deficiency disorder. Lancet 1974 II, 399

  18. Nordio, S., Donath, A., Macagno, F., Gatti, R.: Chronic hypomagnesemia with magnesiumdependent hypocalcemia. I. A new syndrome with intestinal magnesium malabsorption. Acta paediat. scand. 60, 441 (1971)

    Google Scholar 

  19. Prasad, A. S., Schulert, A. R., Sandstead, H. H., Miale, A., Farid, J.: Zinc, iron and nitrogen content of sweat in normal and deficient subject. J. Lab. clin. Med. 62, 84 (1963)

    Google Scholar 

  20. Prasad, A. S., Miale, A., Farid, Z., Sandstead, H. H., Schulert, A. R.: Zinc metabolism in patients with the syndrome of iron deficiency, anemia, hepatosplenomegaly, dwarfism and hypogonadism. J. Lab. clin. Med. 61, 537 (1963)

    Google Scholar 

  21. Price, N. O., Bunce, G. E., Engel, R. W.: Copper, manganese, and zinc balance in preadolescent girls. Amer. J. clin. Nutr. 23, 258 (1970)

    Google Scholar 

  22. Sandstead, H. H., Prasad, A. S., Schulert, A. R., Farid, Z., Miale, A., Bassily, S., Darby, W. J.: Human zinc deficiency, endocrine manifestations and response to treatment. Amer. J. clin. Nutr. 20, 422 (1967)

    Google Scholar 

  23. Schlage, C., Wortberg, B.: Die Versorgung gesunder Kleinkinder und Schulkinder mit dem Spurenelement Zink. Med. u. Ernähr. 12, 217 (1971)

    Google Scholar 

  24. Schlettwein-Gsell, C., Mommsen-Straub, S.: Spurenelemente in Lebensmitteln. Int. J. Vitam. Nutr. Res. Suppl. 13, 10 (1973)

    Google Scholar 

  25. Schnippering, H. G.: Ein Gesamtkörper-Radioaktivitätszähler zum vielseitigen Einsatz in klinischer Diagnostik und Forschung. Kerntechnik 13, 297 (1971)

    Google Scholar 

  26. Todd, W. R., Elvehjem, C. A., Hart, E. B.: Zinc in the nutrition of the rat. Amer. J. Physiol. 107, 146 (1934)

    Google Scholar 

  27. Tribble, H. M., Scoular, F. I.: Zinc metabolism of young college women on self-selected diets. J. Nutr. 52, 209 (1954)

    Google Scholar 

  28. Thyresson, N.: Acrodermatitis enteropathica. Report of a case healed with zinc therapy. Acta derm.-venereol. (Stockh.) 54, 383 (1974)

    Google Scholar 

  29. Tucker, H. F., Salmon, W. D.: Parakeratosis or zinc deficiency disease in the pig. Proc. Soc. exp. Biol. (N.Y.) 88, 613 (1955)

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Klinische Physiologie, Medizinische Einrichtungen der Universität Düsseldorf, Düsseldorf

    Ingrid Lombeck

  2. Institut für Medizin der Kernforschungsanlage Jülich GmbH, Jülich

    H. G. Schnippering, K. Kasperek, F. Ritzl & L. E. Feinendegen

  3. Universitäts-Hautklinik, Münster

    Hertha Kästner

  4. Kinderklinik III, Medizinische Einrichtungen der Universität Düsseldorf, Düsseldorf

    H. J. Bremer

Authors
  1. Ingrid Lombeck
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. H. G. Schnippering
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. K. Kasperek
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. F. Ritzl
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Hertha Kästner
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. L. E. Feinendegen
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. H. J. Bremer
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Lombeck, I., Schnippering, H.G., Kasperek, K. et al. Akrodermatitis enteropathica — eine Zinkstoffwechselstörung mit Zinkmalabsorption. Z. Kinder-Heilk. 120, 181–189 (1975). https://doi.org/10.1007/BF00439007

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00439007

Key words

Search

Navigation