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 11,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Recurrent corneal erosions (RCE) are common. They are characterised by repeated episodes of pain, difficulty in opening the eyes, watering, and photophobia resulting from poor epithelial adhesion. In the majority of patients with RCE, trauma is the initiating factor. Epithelial, stromal, and endothelial corneal dystrophies have all been described in association with RCE. Other causes that may lead to RCE include chemical and thermal injuries, previous herpetic keratitis, meibomian gland dysfunction, ocular rosacea, diabetes mellitus, Salzmann's nodular degeneration, band keratopathy, previous bacterial ulceration, kerato-conjunctivitis sicca, and epidermolysis bullosa. The conditions that are associated with RCE can be either primary or secondary depending on whether the basement membrane complex abnormality is intrinsic or acquired. Primary types tend to be bilateral, symmetrical and develop in multiple corneal locations. The pathogenetic mechanism of this disorder is related to poor adhesion of the corneal epithelium to the underlying stroma. Excessive matrix metalloproteinase (MMP) activity may play a role in the pathogenesis. Although the majority of patients will respond to simple measures such as padding and antibiotic ointment, RCE resistant to simple measures require approaches that are more elaborate. The common goal of these approaches is to encourage proper formation of adhesion complexes between the epithelium and the stroma. The use of long-term contact lenses, autologous serum eye drops, botulinum toxin, induced ptosis, oral MMP inhibitors, diamond burr polishing of Bowman's membrane have been reported with varying degree of success in treating RCE. Anterior stromal puncture with insulin needles or Neodymium : aluminium-yttrium-garnet may enhance the epithelial adhesion to the basement membrane by scar formation and success rates of up to 80% have been reported in the treatment of recalcitrant RCE. Excimer laser photo-therapeutic keratectomy (PTK) is now a well-established treatment modality for RCE and is being used both safely and effectively. Partial ablation of Bowman's layer with PTK gives a smooth surface for the newly generating epithelium to migrate and form adhesion complexes. The pathogenesis, clinical features, and management options of this common disorder are discussed in this review article.
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PMID:Pathogenesis, clinical features and management of recurrent corneal erosions. 1757 Oct 89

The association of epidermolysis bullosa simplex and muscular dystrophy (EBS-MD) has rarely been discussed in ophthalmology literature. This case report offers a brief summary of epidermolysis bullosa and describes what is known about EBS-MD. The case involves a patient with EBS-MD who presented with ptosis and ophthalmoplegia, suggesting that these may be complications of EBS-MD.
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PMID:Ptosis and ophthalmoplegia associated with epidermolysis bullosa simplex-muscular dystrophy. 2082 32

Epidermolysis bullosa associated with muscular dystrophy is a rare, autosomal recessive form of epidermolysis bullosa simplex caused by mutations in the plectin gene, PLEC1. We describe a phenotypically mild case due to compound heterozygous mutations in PLEC1 (2677_2685del and the novel mutation Q1644X). Clinical features included mild skin blistering since birth, slowly progressive and late-onset upper limb-predominant weakness, facial weakness, ptosis, incomplete ophthalmoplegia, and paroxysmal atrial fibrillation.
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PMID:Epidermolysis bullosa with late-onset muscular dystrophy and plectin deficiency. 2167 28

Plectin mutations have been reported in epidermolysis bullosa simplex with muscular dystrophy. We report the first case of left ventricular non-compaction in an 18-year-old male with epidermolysis bullosa simplex with muscular dystrophy. The patient was diagnosed with epidermolysis bullosa simplex after blistering was noted at birth. Motor function difficulties were first recognized at age 11, however the patient was lost to follow up. He was re-evaluated at age 17 and demonstrated significant ptosis, ophthalmoparesis, and pharyngeal muscle weakness. He had predominant proximal muscle weakness with the inability to raise arms against gravity. He was ambulatory for short distances but lost the ability to rise from the floor at 14 years. He was subsequently diagnosed with epidermyolysis bullosa simplex with muscular dystrophy and a PLEC1 mutation. Screening cardiovascular imaging revealed a diagnosis of isolated left ventricular non-compaction. This case highlights the potential for delayed onset muscular dystrophy in patients with epidermolysis bullosa simplex. Furthermore, this case also underscores the importance of long term, routine cardiac evaluation, including imaging and electrophysiologic evaluation, in patients with epidermolysis bullosa simplex with muscular dystrophy as the cardiac phenotype appears to parallel the variable severity and age of onset that characterize the neuromuscular phenotype.
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PMID:Left ventricular non-compaction cardiomyopathy associated with epidermolysis bullosa simplex with muscular dystrophy and PLEC1 mutation. 2580 Feb 22

Oculomotor dysfunction in epidermolysis bullosa simplex associated with muscular dystrophy has been reported rarely in the ophthalmic literature. In a series of 6 patients with epidermolysis bullosa simplex associated with muscular dystrophy, 3 demonstrated ptosis, ophthalmoplegia, or both. Ptosis and ophthalmoplegia may occur early in epidermolysis bullosa simplex associated with muscular dystrophy and aid in diagnosis. [J Pediatr Ophthalmol Strabismus. 2018;55:e26-e29.].
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PMID:Pediatric Ophthalmoplegia and Ptosis in Epidermolysis Bullosa Simplex Associated With Muscular Dystrophy. 3018 Feb 41

Therapeutic contact lenses (TCLs) are often used in the management of a wide variety of corneal and ocular surface diseases (OSDs). Indications of TCL include pain relief, enhancing corneal healing, corneal sealing, corneal protection, and drug delivery. For painful corneal diseases such as bullous keratopathy, epidermolysis bullosa, and epithelial abrasions/erosions, bandage contact lenses (BCLs) provide symptomatic relief. Postoperatively in photorefractive keratectomy or laser epithelial keratomileusis, BCLs also alleviate pain. In severe OSDs such as severe dry eye, Stevens-Johnson syndrome/toxic epidermal necrolysis, gas-permeable scleral contact lenses are often used to enhance corneal healing. BCLs are used post-keratoplasty, post-trabeculectomy, and post-amniotic membrane transplantation to enhance healing. BCLs, with or without glue adhesives, are used to seal small corneal perforations and sometimes also used as bridging treatment before penetrating keratoplasty in larger corneal perforations. In patients with eyelid conditions such as trichiasis, ptosis, and tarsal scarring, BCLs are also effective in forming a mechanical barrier to protect the cornea. A relatively new use for TCLs is in ocular drug delivery where TCLs are used to maintain therapeutic concentrations of medication on the ocular surface. Contraindications of the use of TCLs include infective keratitis, corneal anesthesia, and significant exposure keratopathy with inadequate eyelid position or movement. Complications of TCL include infective keratitis, corneal hypoxia and associated complications, corneal allergies and inflammation, and poor lens fit. Overall, TCLs are effective in the treatment of corneal and OSDs but contraindications and complications must be considered.
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PMID:Therapeutic Contact Lenses in the Treatment of Corneal and Ocular Surface Diseases-A Review. 3318 48