Related Experiment Video
Updated: Jul 21, 2026
07:36
Isolation of Human Lymphatic Endothelial Cells by Multi-parameter Fluorescence-activated Cell Sorting
Published on: May 1, 2015
Cogan's syndrome: a rare systemic vasculitis
1Inverclyde Royal Hospital, Greenock.
Archives of Disease in Childhood
|August 1, 1994
Summary
Cogan's syndrome, a rare autoimmune disorder, can cause severe hearing loss and arthritis in children. Early diagnosis and treatment are crucial to prevent irreversible damage and preserve hearing.
Area of Science:
- Ophthalmology
- Rheumatology
- Pediatrics
- Immunology
Background:
- Cogan's syndrome is a rare autoimmune condition characterized by non-syphilitic interstitial keratitis and vestibuloauditory dysfunction.
- It primarily affects young adults but can manifest in children, presenting diagnostic challenges.
Observation:
- A 4-year-old girl presented with keratitis and ataxia, initial symptoms suggestive of neurological or ocular involvement.
- Over two months, she developed profound hearing loss, arthritis, and polychondritis, indicating a systemic inflammatory process.
Findings:
- The constellation of symptoms led to a diagnosis of Cogan's syndrome in this pediatric patient.
- Review of literature confirms the varied presentation and potential for rapid progression of Cogan's syndrome.
Implications:
- This case underscores the importance of recognizing Cogan's syndrome in pediatric patients with overlapping ocular, auditory, and rheumatologic symptoms.
- Prompt diagnosis and initiation of immunosuppressive therapy are critical to prevent irreversible hearing loss and manage systemic inflammation.
Related Concept Videos
01:16Vascular Spasm
The vascular phase, also known as vasospasm, is the initial stage of hemostasis, crucial for preventing excessive bleeding when a blood vessel is injured. After a vessel is cut, nerves in the damaged area trigger pain and other sensory impulses. Simultaneously, the smooth muscles in the vessel wall contract, resulting in a vascular spasm. This contraction reduces the vessel's diameter at the injury site, slowing or stopping blood loss through the vessel wall. Vascular spasms typically last for...
01:01Hypersensitivity Reactions: Cytolytic Reactions
Type II hypersensitivity involves IgG and IgM antibodies targeting cell surface antigens, leading to cell destruction. This can occur through complement activation, antibody-dependent cell-mediated cytotoxicity (ADCC), or acting as opsonins for phagocytosis. When excessive, these reactions cause significant tissue damage.Drug-induced hemolytic anemia is a common example, where drugs like penicillin or cephalosporins bind to red blood cells, forming drug-protein complexes. These complexes...
01:26Cushing Syndrome I: Introduction
Cushing syndrome refers to the collection of clinical manifestations that arise when tissues are exposed to excessive amounts of cortisol or cortisol-like medications over an extended period. Cortisol, a glucocorticoid produced by the adrenal cortex, regulates metabolism, immune responses, and the body’s adaptation to stress. When its concentration remains chronically elevated, these physiological pathways become dysregulated, resulting in the characteristic features of the syndrome.Exogenous...
01:19Cushing Syndrome II: Pathophysiology
Cortisol production is normally governed by the hypothalamic–pituitary–adrenal (HPA) axis, which maintains hormonal balance through tightly regulated feedback mechanisms. Disruption of this regulatory system is central to the development of Cushing syndrome, whether the excess cortisol originates from external medications or internal pathology. Persistent cortisol elevation alters metabolism, immune function, and endocrine signaling, producing the characteristic clinical features of the...
01:21Cytotoxic Edema: Pathophysiology
Cytotoxic edema is a form of cerebral edema characterized by intracellular swelling of neurons, astrocytes, and other glial cells. It develops when the mechanisms responsible for maintaining ionic gradients across the cell membrane become impaired. Under normal physiological conditions, the sodium–potassium ATPase actively transports sodium ions out of the cell and potassium ions into the cell, preserving osmotic balance and enabling electrical signaling. This pump requires a continuous supply...
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...