Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0022568 (keratitis)
 5,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients had an unusual ichthyosiform dermatosis, neurosensory deafness, and vascularizing keratitis. There was no family history of the disorder and no consanguinity. A squamous cell carcinoma of the tongue developed in one child. The association of such an eruption and deafness in previously reported cases is reviewed.
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PMID:Ichthyosiform dermatosis, keratitis, and deafness. 59 1

A case history of a 15-year-old schoolgirl with fluctuating bilateral uveitis, bilateral stromal keratitis with vascularisation, and bilateral deafness associated with tinnitus and balance disturbance is described. Three years from the onset of her clinical signs she died of a sudden cardiac arrest caused by endocarditis associated with valvular and arterial lesions. Chlamydia psittaci was isolated from her conjunctiva. In her blood type-specific antichlamydial antibody at a level of 1/64 against her own isolate was detected. The clinical findings in this patient were suggestive of a Cogan's syndrome. It is highly probable that the chlamydia isolated from the eyes was responsible for her various lesions.
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PMID:Isolation of Chlamydia psittaci from a patient with interstitial keratitis and uveitis associated with otological and cardiovascular lesions. 70 73

Two patients with ichthyosiform erythroderma of the same unusual but characteristic distribution are described. Both patients were born with perceptive deafness and developed severe vascularizing keratitis in early childhood. There is no family history of the disorder in either case. This syndrome is discussed in relation to previous reports of atypical ichthyosiform erythroderma associated with deafness.
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PMID:Atypical ichthyosiform erythrodernam deafness and keratitis. A report of two cases. 125 51

In 1981, the term KID syndrome was suggested for patients with congenital ichthyosis associated with deafness and keratitis. We had a chance to examine the temporal bone of an infant with this syndrome. This patient showed no auditory brain stem response in either ear. Temporal bone studies revealed cochleosaccular abnormality. These findings are offered as a possible explanation for the patient's deafness. The pathologic inner ear findings of congenital deafness syndromes associated with ichthyosis have been heretofore reported in Refsum's syndrome and in a case with universal alopecia. In these cases, the temporal bone pathologic findings were a result of cochleosaccular abnormality. From our case and previous reports, it is suggested that the deafness associated with congenital ichthyosis might be the result of cochleosaccular abnormality.
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PMID:Temporal bone findings in keratitis, ichthyosis, and deafness syndrome. Case report. 157 Sep 35

Extensive cutaneous mycoses have been described in patients with the keratitis, ichthyosis, and deafness (KID) syndrome. We present a case occurring in a 48-year-old woman where improvement in the ichthyosiform dermatosis, stabilization of her ocular disease, and apparent partial prevention of further cutaneous malignancies occurred in association with ketoconazole therapy.
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PMID:Keratitis, ichthyosis, and deafness (KID) syndrome: management with chronic oral ketoconazole therapy. 173 93

We describe a patient with the keratitis, ichthyosis, and deafness (KID) syndrome who also had a generalized cytomegalovirus infection. Patients with the KID syndrome are susceptible to not only bacterial and fungal infections, but also to viral infections.
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PMID:Systemic cytomegalovirus in a patient with the keratitis, ichthyosis, and deafness (KID) syndrome. 216 Jun 57

We report three patients with keratitis, ichthyosis and deafness (KID)-syndrome. All had characteristic hyperkeratotic skin lesions and neurosensory hearing defects. Two had ophthalmologic symptoms. The third patient did not have eye involvement at the age of 3 years, but exhibited the other typical signs of the syndrome. In none of the three cases were any of the patients' relatives affected, and a spontaneous new mutation is the most likely explanation for the occurrence of this rare syndrome. Histopathological and electron microscopic studies revealed orthohyperkeratosis but no other pathology and no abnormal deposits of glycogen were found. Treatment with the aromatic retinoid etretinate proved to be of little value in any of the patients. The necessity for early audiologic and ophthalmologic evaluation and the need for life-long medical care for patients with KID-syndrome is emphasized.
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PMID:Keratitis, ichthyosis and deafness (KID)-syndrome: report of three cases and a review of the literature. 219 10

Keratitis, ichthyosis, and deafness (KID) syndrome in a mother and her daughter is reported. Both patients showed the typical cutaneous abnormality of papillomatous hyperkeratosis, which gave the skin a grainy, rugose, spiculated appearance, together with keratitis with corneal neovascularization and a neurosensory hearing defect. Treatment with etretinate did not benefit the daughter. The mother had marked keratitis with progressive corneal opacification that required repeated bilateral corneal grafts, which were unsuccessful. This is the second report of hereditary transmission of this disorder. An autosomal dominant mode of inheritance is probable.
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PMID:Familial occurrence of KID (keratitis, ichthyosis, deafness) syndrome. Case reports of a mother and daughter. 239 58

Electron microscopy performed in a young girl suffering from keratitis, ichthyosis, and deafness syndrome showed pathologic storage of glycogen in various types of tissues. This finding may be interpreted as evidence of an underlying inborn error of metabolism.
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PMID:Keratitis, ichthyosis, and deafness syndrome with glycogen storage. 240 86

An infant with congenital ichthyosis and deafness developed Hirschsprung's disease. No evidence of keratitis was present. No previous cases of ichthyosis have been associated with aganglionic megacolon. Although no corneal changes were observed, we believe that the clinical features of ichthyosis and deafness suggest the diagnosis of KID (keratitis, ichthyosis, deafness) syndrome. Whether corneal changes would have occurred is unknown, since the infant died of malnutrition and infectious complications.
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PMID:Ichthyosis, deafness, and Hirschsprung's disease. 270 59


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