Related Experiment Video
Updated: Aug 16, 2026
07:50
A Metadata Extraction Approach for Clinical Case Reports to Enable Advanced Understanding of Biomedical Concepts
Published on: September 20, 2018
Bilateral consecutive central corneal perforations associated with hypogammaglobulinemia
E K Akpek1, R S Haddad, J A Winkelstein
1Wilmer Eye Institute, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Ophthalmology
|January 27, 2000
Summary
Patients with common variable immunodeficiency may experience severe sterile corneal thinning and perforation. Prompt management is crucial to prevent vision loss in these rare cases.
Area of Science:
- Ophthalmology
- Immunology
Background:
- Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by low levels of immunoglobulins.
- Ocular manifestations in CVID are uncommon but can be severe.
Observation:
- A case report detailing a patient with CVID who developed consecutive central sterile corneal perforations.
- Initial investigations for infectious or autoimmune causes were negative.
Findings:
- The patient experienced progressive sterile stromal thinning and perforation in one eye, despite treatment, leading to secondary infection and vision loss.
- The fellow eye also developed sterile thinning and required a lamellar patch graft, preserving vision for two years.
Implications:
- This case highlights a potentially devastating, yet treatable, ocular complication in patients with CVID.
- Early recognition and intervention for sterile corneal thinning are vital in immunocompromised individuals.
Related Concept Videos
01:15Transcytosis of IgG
Transcytosis is the process in which molecules are internalized by endocytosis, transported across the cell, and released through exocytosis from the opposite end of the cell. Molecules such as insulin, immunoglobulins, and certain nutrients are transferred through the recycling endosomes by recycling and transcytosis.
IgG molecules from a mother undergo transcytosis starting around 13 weeks of gestation. The amount of IgG transferred and entering the fetal blood circulation increases with...
IgG molecules from a mother undergo transcytosis starting around 13 weeks of gestation. The amount of IgG transferred and entering the fetal blood circulation increases with...
01:25Glaucoma: Overview
Glaucoma is an eye condition characterized by increased intraocular pressure that damages the retina and optic nerve, leading to irreversible blindness if left untreated. The human eye has various components, including the cornea, iris, pupil, lens, and optic nerve. Aqueous humor is secreted by the epithelium of the ciliary body in the posterior chamber and flows through the trabecular meshwork and canal of Schlemm, maintaining normal intraocular pressure. The trabecular meshwork and the canal...
01:25Immunodeficiency Diseases
Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
There are three main causes of immunodeficiency disorders...
There are three main causes of immunodeficiency disorders...
01:27Cytomegalovirus Disease
Cytomegalovirus (CMV) disease is caused by human cytomegalovirus, a double-stranded DNA virus of the Herpesviridae family. While primary CMV infection is often asymptomatic in immunocompetent individuals, the virus can cause severe disease in neonates and immunocompromised patients. CMV is the most common cause of congenital viral infection in the United States, and a major pathogen in solid organ and hematopoietic stem cell transplant recipients.CMV is transmitted via bodily fluids, sexual...
01:27Cryptococcal Meningitis
Cryptococcal meningitis is a life-threatening opportunistic infection predominantly associated with HIV/AIDS, accounting for over 100,000 deaths annually worldwide. However, it also affects individuals with other forms of immunosuppression, including those undergoing immunosuppressive therapy, organ transplant recipients, patients with innate immunodeficiencies, and individuals with hematological disorders. The infection is caused mainly by Cryptococcus neoformans and Cryptococcus gattii,...
01:19Hypersensitivity Reactions: Immune-Complex Reactions
Type III hypersensitivity reactions occur when antigen–antibody complexes form and activate the complement system. Normally, these complexes help the clearance of antigens by phagocytes and red blood cells. However, when large numbers of immune complexes are present, they can deposit in tissues—particularly in the walls of blood vessels—leading to inflammation and tissue injury. These deposits trigger complement activation and neutrophil recruitment, resulting in serum sickness, a systemic...