Scheie and Hurler–Scheie diseases/mucopolysaccharidosis IS and IHS/α-iduronidase deficiency
William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop in Atlas of Inherited Metabolic Diseases, 2020
The supportive management and enzyme replacement set out in Chapter 76 is particularly appropriate for Scheie and Hurler–Scheie patients. Hematologic stem cell transplantation, especially if performed before two years of age has resulted in rescue of neurocognition [45]. In 45 patients with attenuated disease recombinant laronidase had decreased excretion of glycosaminoglycan and decreased hepatic volume, improved shoulder flexion and decrease in sleep apnea. Corneal transplantation has been successful [46, 47]. Aggressive surgical treatment of glaucoma and carpal tunnel syndrome is also indicated. Cardiac valve replacement has also been successful in both IS and IH/IS patients [18, 19]. Mitral valve replacement and resection of a large left atrial appendage was successful in a patient with Hurler–Scheie syndrome [48]. Hydrocephalus requires shunting, and cervical cord decompression may be required.
Diagnosis and Treatment of Fungal Keratitis
Mahendra Rai, Marcelo Luís Occhiutto in Mycotic Keratitis, 2019
For all fungal corneal ulcers, if there is no perforation or no tendency for perforation, the preferred treatment method is a combination of various antifungal drugs. Then, depending on the outcome of the treatment, the size, location, depth of the lesion, and the visual acuity, the ophthalmologist, together with the patient, decides whether corneal transplantation is needed. The main indications for lamellar keratoplasty include: Drug treatment for one week or more is ineffective or the patient has suffered from the disease for a long time but has not been cured.It is superficial or medium layer ulcers, infiltration only affecting the anterior 2/3 corneal stroma.The visual acuity drops seriously to 20/200 or below.
Corneal Disorders
Ching-Yu Cheng, Tien Yin Wong in Ophthalmic Epidemiology, 2022
Since the first successful penetrating keratoplasty performed more than 100 years ago, corneal transplantation remains the main method for restoring corneal clarity and vision in patients with visually debilitating corneal diseases.85 It is the most common transplantation performed worldwide, with >40,000 cases/year and ~3,500 cases/year performed in the USA and the UK, respectively.85 However, shortage of donor corneas remains a persistent global issue, with around one cornea available for 70 needed.69 This has led to a range of innovative measures being considered and implemented to improve the eye donation rate and utilization of donor corneas.86–89
Awareness on Eye Donation in the North-eastern State of Tripura, India – The Tripura Eye Survey
Published in Ophthalmic Epidemiology, 2022
Srinivas Marmamula, Ruchi Priya, Rajashekar Varada, Jill E Keeffe
Globally, 5% of the vision loss is attributed to corneal opacities in2010.1 Corneal transplantation is a standard procedure that offers the potential for sight restoration to those with vision loss from corneal diseases. Over 12.7 million people worldwide are estimated to benefit from corneal transplantation.2 Unfortunately, half of the global population does not have access to corneal transplantation due to limited eye banking services.3 In India, there are approximately 6.8 million people with visual acuity worse than 6/60 in at least one eye due to corneal blindness and about a million with bilateral corneal involvement.4 The number of individuals with unilateral corneal blindness in India was estimated as 10.6 million in 2020.5 The major causes of corneal-related vision loss include trachoma, corneal ulcerations, xerophthalmia, ophthalmia neonatorum, harmful traditional eye medicines, onchocerciasis, leprosy, and ocular trauma.6
Toxicity of Amphotericin B in Rabbit Corneal Epithelial Cells Stored in Optisol™-GS: Corneal Epithelial Cell Morphology and Migration
Published in Current Eye Research, 2022
Kohei Harada, Hideki Fukuoka, Yuriko Ban, Yulia Aziza, Go Horiguchi, Hidetoshi Tanioka, Shigeru Kinoshita, Masafumi Uematsu, Takashi Kitaoka, Chie Sotozono
It should be noted that this current study did have some limitations. First, the experiments performed in this study involved the use of rabbit corneas, as Japanese law prohibits the use of domestic human donor corneas in academic research. Second, the number of vacuoles and the ZO-1 expression in the histological experiments were not quantitatively evaluated. The findings in this study reveal that an AmB concentration of ≥5 μg/ml in Optisol™-GS may affect CEC morphology and migration ability. It should be noted that there are numerous corneal transplantation techniques, such as penetrating keratoplasty, epithelial transplantation, endothelial transplantation, etc. However, the method used for corneal preservation has yet to be standardized globally. Thus, the toxicity of AmB needs to be further evaluated, not only in regard to corneal endothelial cells but also CECs. Our findings showed that AmB at the concentration of 2.5 μg/ml can be considered safe and effective for the preservation of donor corneal tissue used for corneal epithelial transplantation surgery. However, for a more detailed and precise evaluation of AmB toxicity, further studies evaluating morphological, functional, and clinical changes in human corneal grafts may be necessary.
Attitudes and Perception Towards Eye Donation in Patients with Corneal Disease: A Case-controlled Population-based Study
Published in Current Eye Research, 2018
Gupta Noopur, Vashist Praveen, Tandon Radhika, Gupta Sanjeev K, Kalaivani Mani, Kumar Deepak
The questionnaire was administered to all participants with corneal opacity in the 25 study clusters, detected on ophthalmic examination during the house-to-house visits by the ophthalmologist. Hence, the study questionnaire on awareness about eye donation was administered after the clinical examination. The advice about corneal transplantation was given to eligible participants during the clinical examination by the ophthalmologist. In children with corneal opacities, the interview was taken from either parent or any available adult in the respective household. Participants without any corneal disease served as controls for the present study. The controls were matched for age and gender and for this purpose, more stress was laid on age than on the gender and was done as best feasible as both cases and controls were part of the same randomly selected study clusters with fixed population size. A difference of ±2 years was taken as acceptable for selection of participants without corneal opacity (controls) as compared to patients with corneal opacity (cases).
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