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Archives of Dermatological Research

, Volume 256, Issue 1, pp 53–65 | Cite as

Morphologische und biochemische untersuchungen am Haar bei gestörtem Aminosäurestoffwechsel

  • L. G. Wiest
  • P. Lutz
  • E. G. Jung
  • N. Paweletz
Article

Zusammenfassung

Der Einfluß angeborener Stoffwechselstörungen auf den Aminosäurengehalt, die Struktur und das Wachstum menschlicher Haare wurde bei Patienten mit Phenylketonurie, Cystinose, Homocystinurie und Tyrosinose untersucht. Rasterelektronenmikroskopische Untersuchungen zeigen bei Phenylketonurie und schweren Cystinosen Cuticuladefekte und -deformierungen in Form eines “Pliseemusters”. Der Aminosäuregehalt des hydrolisierten Haarkeratins ist bei allen Patienten im Normbereich und zeigt keine signifikanten Unterschiede von Phenylalanin, Cystin, Homocystin, Methionin oder Tyrosin. Störungen des Haarwachstums wurden an Hand von standardisierten Trichogrammen erfaßt. Die Ergebnisse zeigen, daß mit zunehmender Schwere der Stoffwechselstörung die Zahl der dystrophischen Haarwurzeln ansteigt.

Morphological and biochemical investigations of hairs in inborn errors of amino acid metabolism

Summary

The influence of inborn errors of metabolism on the amino acid content, the structure and growth of human hair has been studied in patients suffering from Phenylketonuria, Cystinosis, Homocystinuria and Tyrosinosis. Examination of hairs under the scanning electron microscope reveals defects and abnormalities such as a plicated pattern of the cuticula in patients with Phenylketonuria and Cystinosis.

The amino acid content of the hydrolized hair keratin of all patients was within normal range and did not reveal significant changes of phenylalanine, cystine, homocystine, methionine or tyrosine.

Disturbance in hair growth was determined by evaluation of standardized hair root samples. The results indicate an increase in hair root atrophy with increasing severity of the disorder of amino acid metabolism.

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Copyright information

© Springer-Verlag 1976

Authors and Affiliations

  • L. G. Wiest
    • 1
  • P. Lutz
    • 2
  • E. G. Jung
    • 1
  • N. Paweletz
    • 3
  1. 1.Univ.-Hautklinik HeidelbergHeidelbergGermany
  2. 2.Univ.-Kinderklinik HeidelbergGermany
  3. 3.Inst. für Zellforschung am Deut. Krebsforsch.-Zentrum HeidelbergGermany

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