lattice corneal dystrophy

格子状角膜変性症

WordNet

1. framework consisting of an ornamental design made of strips of wood or metal (同)latticework, fretwork
2. an arrangement of points or particles or objects in a regular periodic pattern in 2 or 3 dimensions
3. any degenerative disorder resulting from inadequate or faulty nutrition
4. the transparent dome-shaped anterior portion of the outer covering of the eye; it covers the iris and pupil and is continuous with the sclera
5. of or related to the cornea

PrepTutorEJDIC

1. 格子(こうし) / 格子窓,格子の戸(門) / …‘を'格子模様(造り)にする / …‘に'格子を取り付ける
2. 栄養障害 / 筋萎縮症,筋ジストロフィー(筋肉の退化・萎縮・運動障害などが起こる病気)
3. (眼球の)角膜
4. 角膜の
5. 格子つきの,格子作りの

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2019/01/23 06:37:13」(JST)

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Lattice corneal dystrophy type
A network of thick linear corneal opacities in patient with a variant of LCD1 (LCD type III) due to a homozygous p. Leu527Arg mutation in the TGFBI gene
Specialty	Ophthalmology

Lattice corneal dystrophy type, also known as Biber-Haab-Dimmer dystrophy, is a rare form of corneal dystrophy. It has no systemic manifestations, unlike the other type of the dystrophy, Lattice corneal dystrophy type II. Lattice corneal dystrophy was first described by Swiss ophthalmologist Hugo Biber in 1890.

Lattice dystrophy gets its name from an accumulation of amyloid deposits, or abnormal protein fibers, throughout the middle and anterior stroma.

Clinical presentation

Filamentous opacities appear in the cornea with intertwining delicate branching processes. During an eye examination, the doctor sees these deposits in the stroma as clear, comma-shaped overlapping dots and branching filaments, creating a lattice effect. Over time, the lattice lines will grow opaque and involve more of the stroma. They will also gradually converge, giving the cornea a cloudiness that may also reduce vision. The disease is bilateral, usually noted before the end of the first decade of life. Although lattice dystrophy can occur at any time in life, the condition usually arises in children between the ages of two and seven.

In some people, these abnormal protein fibers can accumulate under the cornea's outer layer—the epithelium. This can cause erosion of the epithelium. This condition is known as recurrent epithelial erosion. These erosions alter the cornea's normal curvature, resulting in temporary vision problems, and expose the nerves that line the cornea, causing severe pain. Even the involuntary act of blinking can be painful.

In systemic cases, kidney failure, heart failure and neuropathy such as facial nerve palsy, laxity of the skin may be noted.

Genetics

Lattice corneal dystrophy type II. Black and white light micrograph showing deposits of amyloid in cornea. Congo red stain.

Diagram depicting gelsolin and the amyloid protein derived from it because of mutations in codon

Lattice corneal dystrophy has two types:

• type I: with no systemic association. It is caused by mutations in TGFBI gene encoding keratoepithelin, which maps to chromosome 5q.
• type II or Finnish type amyloidosis: associated with manifestations of systemic amyloidosis due to accumulation of gelsolin. Associated conditions may include cutis laxa and ataxia.
• type III is also described which has an onset at age 70 to 90 years and is not associated with systemic amyloidosis.

Treatment

In case of corneal erosion, a doctor may prescribe eye drops and ointments to reduce the friction on the eroded cornea. In some cases, an eye patch may be used to immobilize the eyelids. With effective care, these erosions usually heal within three to seven days, although occasional sensations of pain may occur for the next six-to-eight weeks. As patients with LCD suffer with dry eyes as a result of erosion, a new technique involving the insertion of punctal plugs (both upper and lower) can reduce the amount of drops used a day, aiding ocular stability.

By about age 40, some people with lattice dystrophy will have scarring under the epithelium, resulting in a haze on the cornea that can greatly obscure vision. In this case, a corneal transplantation may be needed. There have been many cases in which teenage patients have had the procedure, which accounts for the change in severity of the condition from person to person.

Although people with lattice dystrophy have an excellent chance for a successful corneal transplantation, the disease may also arise in the donor cornea in as little as three years. In one study, about half of the transplant patients with lattice dystrophy had a recurrence of the disease between two and 26 years after the operation. Of these, 15 percent required a second corneal transplant. Early lattice and recurrent lattice arising in the donor cornea responds well to treatment with the excimer laser.

Phototherapeutic keratectomy (PTK) using [Excimer laser] can restore and preserve useful visual function for a significant period of time in patients with anterior corneal dystrophies.

External links

Classification	D ICD-10: H18.5 ICD-9-CM: 371.54 OMIM: 122200
External resources	eMedicine: article/1193793

UpToDate Contents

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• 1. 慢性腎疾患関連眼疾患 eye disorders associated with chronic kidney disease
• 2. アミロイド疾患の遺伝的要因 genetic factors in the amyloid diseases
• 3. 左室肥大が認められる患者におけるファブリー病の臨床症状およびスクリーニング cardiac manifestations of fabry disease and screening in patients with left ventricular hypertrophy

English Journal

• Features of recurrence after excimer laser phototherapeutic keratectomy for anterior corneal pathologies in north china.

• Chen M, Xie L.SourceQingdao Eye Hospital, Shandong Eye Institute, Shandong Academy of Medical Sciences, Qingdao, China.
• Ophthalmology.Ophthalmology.2013 Jun;120(6):1179-85. doi: 10.1016/j.ophtha.2012.12.001. Epub 2013 Feb 22.
• PURPOSE: To study long-term clinical patterns of recurrence of anterior corneal pathologies after excimer laser phototherapeutic keratectomy (PTK).DESIGN: Retrospective, noncomparative, interventional case series.PARTICIPANTS: Thirty patients (44 eyes) with anterior corneal pathologies who underwent
• PMID 23462272

• Classification of secondary corneal amyloidosis and involvement of lactoferrin.

• Araki-Sasaki K, Hirano K, Osakabe Y, Kuroda M, Kitagawa K, Mishima H, Obata H, Yamada M, Maeda N, Nishida K, Kinoshita S.SourceIdeta Eye Hospital, Kumamoto, Japan. Electronic address: sasakis@sa2.so-net.ne.jp.
• Ophthalmology.Ophthalmology.2013 Jun;120(6):1166-72. doi: 10.1016/j.ophtha.2012.11.047. Epub 2013 Feb 28.
• PURPOSE: To classify secondary corneal amyloidosis (SCA) by its clinical appearance, to analyze the demographics of the patients, and to determine the involvement of lactoferrin.DESIGN: Retrospective, observational, noncomparative, multicenter study.PARTICIPANTS: Twenty-nine eyes of 29 patients diag
• PMID 23453509

Japanese Journal

• New mode of treatment for lattice corneal dystrophy type I : corneal epithelial debridement and fibronectin eye drops

• Morita Yukiko,Chikama Tai-ichiro,Yamada Naoyuki [他],MORISHIGE Naoyuki,SONODA Koh-Hei,NISHIDA Teruo
• Japanese journal of ophthalmology 56(1), 26-30, 2012-01-01
• NAID 10030248289

• A Case of Lattice Corneal Dystrophy due to L527R Mutation in the TGFBI Gene with Asymmetric Corneal Opacity in Eye Laterality

• OHNISHI Takako,SAKIMOTO Tohru,SAWA Mitsuru
• Japanese journal of ophthalmology 54(6), 628-629, 2010-11-01
• NAID 10027666578

Related Links

• Lattice Corneal Dystrophy - Diseases & Conditions ...

Lattice corneal dystrophy (LCD), an IC3D category 1 dystrophy, is an autosomal-dominant condition and one of the most common stromal dystrophies. It is a slowly progressive disease that results in significant ...

• lattice corneal dystrophy type I - Genetics Home Reference

Lattice corneal dystrophy type I is an eye disorder that affects the clear, outer covering of the eye called the cornea. The cornea must remain clear for an individual to see properly; however, in lattice corneal dystrophy ...

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★リンクテーブル★

リンク元	「格子状角膜変性症」
関連記事	「dystrophy」「lattice」「corneal」「corneal dystrophy」

「格子状角膜変性症」

　　[★]

英

lattice corneal dystrophy

同

格子状角膜ジストロフィー

「dystrophy」

　　[★]

• n.

• (医)栄養失調、栄養失調症。(医)異栄養、異栄養症、ジフトロフィー

栄養障害。細胞や組織の物質代謝障害によって変性・萎縮などの起こること。

　 　

「lattice」

　　[★]

• 格子

関

grating、grid

　 　 　

「corneal」

　　[★]

• adj.

• 角膜の

関

cornea

　 　

「corneal dystrophy」

　　[★] 角膜ジストロフィー