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Globular and Fibrous Proteins02:21

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Many proteins can be classified into two distinct subtypes - globular or fibrous. These two types differ in their shapes and solubilities.
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Fibrous proteins are either long and narrow proteins or assemble to form long and thin structures. They contain repetitive units and usually consist of either alpha helices or beta sheets and, in rare cases, a mix of both. The amino acids in the primary structure often consist of repeating amino acid sequences. The role of fibrous proteins is primarily structural. Many are located in the extracellular matrix and are present in connective tissues to impart strength and joint mobility. They are...
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Fibrous joints are a type of joint where the bones are connected by fibrous connective tissue. These joints provide stability and minimal to no movement between the articulating bones. There are three types of fibrous joints.
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Fibrous dysplasia imitating malignancy.

Markus Martini1, Anne Klausing1, Nils Heim1

  • 1Department of Maxillofacial and Plastic Surgery, (Head: Prof. Dr. Dr. R. H. Reich), University of Bonn, Sigmund-Freud-Str. 25, 53127, Bonn, Germany.

Journal of Cranio-Maxillo-Facial Surgery : Official Publication of the European Association for Cranio-Maxillo-Facial Surgery
|June 4, 2018
PubMed
Summary

Aggressive fibrous dysplasia (FD) can exhibit rapid, malignant-like growth, particularly in cranio-facial cases. Quantitative analysis helps define these rare aggressive forms, estimated to occur in about 5% of FD cases.

Keywords:
Aggressive growthFibrous dysplasiaMalignancyMcCune-AlbrightPolyostoticTumor

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Area of Science:

  • Orthopedics
  • Oncology
  • Pathology

Background:

  • Fibrous dysplasia (FD) is a benign bone disorder with unpredictable growth patterns.
  • It can manifest as single or multiple lesions, or as part of syndromes like McCune-Albright.
  • Aggressive, rapid growth is an extremely rare presentation of FD, with unclear diagnostic criteria.

Purpose of the Study:

  • To define and characterize aggressive forms of fibrous dysplasia.
  • To implement quantitative growth measures for objective assessment.
  • To differentiate aggressive non-malignant from malignant bone lesions.

Main Methods:

  • Analysis of 39 cranio-facial fibrous dysplasia cases.
  • Volumetric-time analysis to determine tumor doubling rates and growth curves.
  • Literature review to compare findings with existing data on aggressive FD.

Main Results:

  • Four out of 39 cases exhibited fast growth tendencies.
  • Two McCune-Albright syndrome patients showed malignant-like rapid growth.
  • Quantitative analysis revealed a 74-day tumor doubling rate in one aggressive case, with an exponential growth curve.

Conclusions:

  • Aggressive fibrous dysplasia is rare, with an estimated prevalence of approximately 5%.
  • Cranio-facial aggressive FD may present in younger patients compared to extra-cranial forms.
  • Quantitative growth analysis provides an objective method for defining aggressive FD, irrespective of tumor type or location.