Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Alpha-1-antitrypsin deficiency and nephropathy.

A Montanelli1, E Mainardi, L Pini

  • 1Department of Clinical Pathology, Ospedale Maggiore of Crema, Italy. laboratorio@hcrema.lombardia.it

Nephron
|December 18, 2001
PubMed
Summary

Alpha-1 antitrypsin deficiency is rarely linked to glomerulonephritis. This study found no direct correlation between antiprotease deficiency severity and nephropathy, suggesting complex disease mechanisms.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Antioxidant capacity and athletic condition of endurance horses undergoing nutraceutical supplementation.

Journal of equine veterinary science·2025
Same author

Self-administration of augmentation therapy for alpha 1-antitrypsin deficiency.

IJTLD open·2025
Same author

Alpha-1 deficiency in severe asthma patients.

The international journal of tuberculosis and lung disease : the official journal of the International Union against Tuberculosis and Lung Disease·2024
Same author

Evaluation of a large set of patients with Autoimmune Polyglandular Syndrome from a single reference centre in context of different classifications.

Journal of endocrinological investigation·2023
Same author

Diagnostic role of internal mammary lymph node involvement in tuberculous pleurisy: a multicenter study.

Pulmonology·2022
Same author

Phenotyping OSAH patients during wakefulness.

Sleep & breathing = Schlaf & Atmung·2022

Area of Science:

  • Nephrology
  • Genetics
  • Internal Medicine

Background:

  • Alpha-1 antitrypsin deficiency (AATD) is a genetic disorder.
  • Glomerulonephritis associated with AATD is considered rare and understudied.
  • The full spectrum of glomerular damage in AATD remains unclear.

Purpose of the Study:

  • To investigate the relationship between the degree of antiprotease deficiency and the presence of nephropathy.
  • To evaluate kidney involvement in patients with different alpha-1 antitrypsin deficiency phenotypes.
  • To determine if nephropathy is a direct consequence of protein deficiency.

Main Methods:

  • Studied two groups: 47 heterozygotic (PiMZ) and 12 homozygotic (PiZZ) subjects with AATD.
  • Utilized noninvasive methods, including urinalysis and proteinuria measurement.

Related Experiment Videos

  • Assessed the correlation between antiprotease deficiency extent and nephropathy expression.

    • Main Results:

    • No statistically significant differences in proteinuria were found between the PiMZ and PiZZ groups.
    • The severity of antiprotease deficiency did not directly correlate with the level of proteinuria.
    • These findings suggest nephropathy in AATD is not solely determined by the degree of protein deficiency.

    Conclusions:

    • Nephropathy in alpha-1 antitrypsin deficiency is not a direct or singular manifestation of the protein deficiency.
    • Further research is needed to elucidate the complex pathophysiology of glomerular lesions in AATD.
    • Noninvasive methods can be used to assess kidney involvement in AATD patients.