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Considerazioni su un caso di due Gemelle Monozigotiche affette da Anemia Drepanocitica

Published online by Cambridge University Press:  01 August 2014

L. Perosa
Affiliation:
Istituto di Clinica Medica dell'Università di Bari, (Direttore Prof. V. Chini)
M. Ramunni
Affiliation:
Istituto di Clinica Medica dell'Università di Bari, (Direttore Prof. V. Chini)
G. Manganelli
Affiliation:
Istituto di Clinica Medica dell'Università di Bari, (Direttore Prof. V. Chini)
L. Bini
Affiliation:
Istituto di Clinica Medica dell'Università di Bari, (Direttore Prof. V. Chini)

Summary

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The Authors describe the case of homozygous twins affected by sickle-cell anemia. The patients were physically similar and showed identical haematological findings. One of them had suffered often of severe sicklemic crisis during one of these appeared also a noticeable neurological complication.

Therefore the Authors discuss the pathogenesys of sickle-cell anemia according to the modern views.

Riassunto

Riassunto

Gli A.A. descrivono il caso di due gemelle monozigotiche affette da anemia drepanocitica. Le due gemelle presentavano non solo una perfetta somiglianza fisica ma anche un identico reperto ematologico. Una sola delle due gemelle però aveva più volte sofferto di gravi « crisi » falcemiche; durante una di queste era comparsa inoltre una grave complicazione neurologica. Gli A.A. pertanto prendono lo spunto per discutere, alla luce delle moderne acquisizioni, la patogenesi delle « crisi » falcemiche.

Résumé

RÉSUMÉ

Les AA. décrivent un cas de deux jumelles monozygotiques atteintes d'anémie drépanocytique. Les deux jumelles se ressemblaient tout-à-fait entr'elles et davantage montraient le même cadre hématologique. Une d'elles avait souffert beaucoup de fois par des poussées falcémiques aiguës; pendant une de ces poussées il est arrivé une grave complication neurologique.

Les AA. prennent l'occasion pour discuter, à la lumière des modernes connaissances, la pathogénie des « poussées » falcémiques.

Zusammenfassung

ZUSAMMENFASSUNG

Die Autoren beschreiben den fall der homozigotischen Zwillinge, die an Drepanocytaemie leiden. Die zwei Zwillinge sind nicht nur Koerperlich aehnlich sondern zeigen auch dasselbe ematologische Bild auf. Nur eines der beiden Zwillinge hatte mehrmals unter schweren Krisen der Drepanocytaemie zu leiden gehabt: waehrend einer dieser Krisen ist eine schwere neurologische Komplikation aufgetreten.

Die Autoren legen dies zugrunde um, beim Lichte moderner Kenntnisse, ueber die Patogenesis der drepanocytischen Krisen zu diskutieren.

Type
Research Article
Copyright
Copyright © The International Society for Twin Studies 1959

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