Keratitis, Ichthyosis, and Deafness (Kid) Syndrome: Management With Chronic Oral Ketoconazole Therapy

Hazen, Paul G.; Walker, Allen E.; Stewart, John J.; Carney, James F.; Engstrom, Conley W.; Turgeon, Karen Larson

doi:10.1111/j.1365-4362.1992.tb03524.x

Cited by 29 publications

(12 citation statements)

References 11 publications

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“…Increased susceptibility to bacterial (3,7,11,12,14,(17)(18)(19)(20)(21)(22), fungal (3,(7)(8)(9)(10)16,17,19,20,22), and viral (22) infections, as well as scabies infestations (7) has been reported in association with KID syndrome. The most commonly reported pathogens include Staphylococcus aureus, E. coli, Pseudomonas aeruginosa, Trychophyton rubrum, and Candida albicans.…”

Section: Discussionmentioning

confidence: 99%

“…It is postulated to be an autosomal dominant disorder, in which most cases represent new mutations (4,5). Other frequently reported findings include recurrent skin infections, malformed or absent nails, alopecia or sparse hair, teeth anomalies, hypohidrosis, short heel cords, growth delay, and development of squamous cell carcinoma (6)(7)(8)(9)(10)(11)(12)(13)(14)(15). Increased susceptibility to viral, bacterial, and mycotic infections has also been observed, and a number of patients have undergone extensive investigations for immunologic dysfunction (3,(11)(12)(13)(14)(15)(16)(17)(18)(19).…”

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confidence: 99%

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Fatal Septicemia in an Infant with Keratitis, Ichthyosis, and Deafness (KID) Syndrome

Gilliam

Williams

2002

Pediatric Dermatology

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Keratitis, ichthyosis, and deafness (KID) syndrome is a rare congenital disorder of unknown etiology in which increased susceptibility to viral, bacterial, and mycotic infections has been observed. We report an infant with KID syndrome who died from overwhelming systemic infection. To date, investigations into the immune function of patients with this syndrome have not revealed a common underlying systemic immune deficit. However, the severity of infections and multiplicity of organisms observed in this syndrome suggest that a primary immunodeficiency is present in addition to an impaired cutaneous barrier to microorganisms.

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Fatal Septicemia in an Infant with Keratitis, Ichthyosis, and Deafness (KID) Syndrome

Gilliam

Williams

2002

Pediatric Dermatology

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“…and in therapy of mycotic blepharitis and other ophthalmic mycoses is discussed below. In addition, long-term oral ketoconazole therapy has been credited with improvement in a woman suffering from the keratitis-ichthyosis-deafness syndrome (141) and, in association with cyclosporin, has been shown to be effective in controlling and preventing reactivation of endogenous uveitis as well as in treating chronic uveitis affecting the posterior pole of the eye (312).…”

Section: Azolesmentioning

confidence: 99%

Current Perspectives on Ophthalmic Mycoses

2003

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Fungi may infect the cornea, orbit and other ocular structures. Species of Fusarium, Aspergillus, Candida, dematiaceous fungi, and Scedosporium predominate. Diagnosis is aided by recognition of typical clinical features and by direct microscopic detection of fungi in scrapes, biopsy specimens, and other samples. Culture confirms the diagnosis. Histopathological, immunohistochemical, or DNA-based tests may also be needed. Pathogenesis involves agent (invasiveness, toxigenicity) and host factors. Specific antifungal therapy is instituted as soon as the diagnosis is made. Amphotericin B by various routes is the mainstay of treatment for life-threatening and severe ophthalmic mycoses. Topical natamycin is usually the first choice for filamentous fungal keratitis, and topical amphotericin B is the first choice for yeast keratitis. Increasingly, the triazoles itraconazole and fluconazole are being evaluated as therapeutic options in ophthalmic mycoses. Medical therapy alone does not usually suffice for invasive fungal orbital infections, scleritis, and keratitis due to Fusarium spp., Lasiodiplodia theobromae, and Pythium insidiosum. Surgical debridement is essential in orbital infections, while various surgical procedures may be required for other infections not responding to medical therapy. Corticosteroids are contraindicated in most ophthalmic mycoses; therefore, other methods are being sought to control inflammatory tissue damage. Fungal infections following ophthalmic surgical procedures, in patients with AIDS, and due to use of various ocular biomaterials are unique subsets of ophthalmic mycoses. Future research needs to focus on the development of rapid, species-specific diagnostic aids, broad-spectrum fungicidal compounds that are active by various routes, and therapeutic modalities which curtail the harmful effects of fungus- and host tissue-derived factors

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“…4 Sufferers from KID syndrome are more susceptible to bacterial and fungal infections of the skin and mucous membranes, mainly candida, which may be due to an intrinsic immunodeficiency, with death sometimes occurring in the first years of life. [5][6][7] A less frequent complication, but which can considerably decrease life expectancy of patients is squamous cell carcinoma, which occurs in 29% of cases. [4][5][6][7][8][9] A high probablility of extracutaneous involvement exists in KID syndrome.…”

Section: Discussionmentioning

confidence: 99%

“…[5][6][7] A less frequent complication, but which can considerably decrease life expectancy of patients is squamous cell carcinoma, which occurs in 29% of cases. [4][5][6][7][8][9] A high probablility of extracutaneous involvement exists in KID syndrome. One manifestation is sensorineural deafness, generally with severe, bilateral and progressive evolution due to cochlear saccular dysplasia.…”

Section: Discussionmentioning

confidence: 99%

Você conhece esta síndrome?

Enei

Cassettari²,

Córdova³

et al. 2011

An. Bras. Dermatol.

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A síndrome de KID é uma displasia ectodérmica congênita rara que afeta a pele, o epitélio da córnea e o ouvido interno. Clinicamente, observam-se placas de eritroqueratodermia na face e pregas, geralmente presentes desde o nascimento, a surdez neurossensorial severa e bilateral, e a vascularização córnea associado à queratite de evolução progressiva à qual surge após as alterações cutâneas e auditivas na puberdade. Face ao quadro surdez, às infecções cutâneas, ao risco de cegueira e à degeneração maligna, o diagnóstico precoce da síndrome é fundamental, bem como o seguimento clínico periódico e o aconselhamento genético

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Keratitis, Ichthyosis, and Deafness (Kid) Syndrome: Management With Chronic Oral Ketoconazole Therapy

Cited by 29 publications

References 11 publications

Fatal Septicemia in an Infant with Keratitis, Ichthyosis, and Deafness (KID) Syndrome

Fatal Septicemia in an Infant with Keratitis, Ichthyosis, and Deafness (KID) Syndrome

Current Perspectives on Ophthalmic Mycoses

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