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Query: UMLS:C0022568 (
keratitis
)
5,133
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Different manifestations on some ocular and adnexal structures may accompany
ichthyosis
. Some ophthalmological manifestations in the clinical course of
ichthyosis
may endanger the eye and even lead to the sight loss. The paper reports a case of a patient with congenital ichthyosiform erythrodermia and ophthalmological manifestations on the lids and cornea. In addition to
ichthyosis
of the lid skin, squamous blepharitis and cicatricial ectropium of the lower lids were also present, while signs of exposure
keratitis
on the right eye and corneal leucostaphyloma on the left eye were also noted. Presence of lagophthalmos and permanent exposure of both eyeballs led to deterioration of the condition which may result in the sight loss on the right eye, as well. Therefore, contracture release of both lower lids was performed followed by full-thickness skin graft. Surgical procedure and the postoperative course were uneventful. The operation successfully corrected position of the lower lids and enabled formation and reestablishment of the precorneal tears film which led to improvement of the condition of the cornea. The effect has been maintained for six postoperative years now.
...
PMID:[Ophthalmologic manifestations in patients with ichthyosis]. 130 16
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.
...
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.
...
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.
...
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.
...
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.
...
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.
...
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.
...
PMID:Ichthyosis, deafness, and Hirschsprung's disease. 270 59
KID (
keratitis
,
ichthyosis
, deafness) syndrome is a congenital ectodermal defect of unknown etiology. Although achilles tendon retraction has been seen on several occasions, no other evidence of skeletal, articular or enthesopathic processes has been described. We report a patient with KID syndrome and acroosteolytic changes on radiographs, along with clinical and radiographic evidence of Jaccoud arthropathy.
...
PMID:Jaccoud arthropathy and acroosteolysis in KID syndrome. 281 Feb 88
We describe a rare syndrome in a Finnish family, in which the father and his two sons are congenitally deaf and also exhibit corneal vascularization, and hyperkeratosis of the skin of the palms, soles, elbows and knees. In addition, all 3 suffer from fungal infections of the skin and nails. We also describe a fourth, sporadic case of a 9-year-old girl with the same disease. The KID (k =
keratitis
, i =
ichthyosis
, d = deafness) syndrome is an ectodermal dysplasia that probably arises through mutation, and is transmitted as an autosomal dominant in the family described by us.
...
PMID:The KID-syndrome in Finland. A report of four cases. 323 12
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