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Biomedical subjects

J D Kivlin

Publications and source records attributed to J D Kivlin.

31 records · Page 2Linked to original sources
PubMed1985

The cherry-red spot in Tay-Sachs and other storage diseases.

The disappearance of the cherry-red spot in an otherwise typical patient with Tay-Sachs disease is described and illustrated. This clinical observation has rarely been made, but it is consistent with the pathological finding of loss of retinal ganglion cells. Disappearance of the cherry-red spot has also been observed in GM1 generalized gangliosidosis, the sialidoses known as the cherry-red spot-myoclonus syndrome and the Goldberg syndrome, and Niemann-Pick, type C disease. Thus the absence of a cherry-red spot in the neurologically impaired child or adult does not exclude these diagnoses.

Humans↗
PubMed1984

Optic neuritis secondary to sinus disease.

Sinus disease is an infrequent, but treatable, cause of optic neuritis. We treated three patients in whom optic neuritis was caused by sinus disease. Treatment of the underlying sinus condition resulted in the improvement of vision.

Child, Preschool↗
PubMed1984

Linkage analysis in lattice corneal dystrophy.

Two kindreds of lattice corneal dystrophy (LCD) [McKusick, 1983, catalog No. 12220] were studied for linkage. Fifty-one relatives were examined clinically, and 27 affected and 24 normal persons were ascertained. Tight linkage could be excluded for 15 informative markers with LOD scores of less than -2.0. The largest positive LOD score was 0.56 at 0 = 0.17 for linkage between haptoglobin and LCD. Combined with a previous study, the combined LOD score is 0.96.

Corneal Dystrophies, Hereditary↗
PubMed1984

Auditory brain-stem responses in Marcus Gunn ptosis.

The auditory brain-stem response (ABR) can detect anomalies both in the auditory pathways and in structures adjacent to these pathways. Patients with Duane's retraction syndrome, associated with hypoplasia of the abducens nerve in the brain-stem, and patients with hemifacial spasm, due to compression of the facial nerve in the brain-stem, have been found to have abnormal ABRs. Marcus Gunn ptosis with 'jaw winking' is considered to be due to misconnection of oculomotor, trigeminal and other cranial nerves. Suspecting that perhaps some 'jaw-winking' phenomena may be due to detectable brain-stem anomalies we tested 7 patients with Marcus Gunn ptosis. Three of the patients demonstrated abnormal ABRs indicative of pontine pathology.

Adolescent↗
PubMed1983

Linkage analysis in dominant optic atrophy.

A kindred of German descent was studied for dominant optic atrophy, type Kjer (McKusick catalog no. 16540). One hundred twenty-three family members were examined clinically, and 36 affected, 81 normal, and six uncertain members were ascertained. Twenty-seven markers were analyzed for 121 members. The maximum lod score obtained was 2.0 at theta = .18 for linkage between the Kidd locus and dominant optic atrophy. Twenty-eight offspring were informative with 2-generation data. There was insufficient information for the acid phosphatase locus to aid gene localization. These data suggest that the locus for dominant optic atrophy is on chromosome 2.

Chromosome Mapping↗
PubMedDate not supplied

Therapy of anisometropic amblyopia.

In 67 patients with strictly anisometropic amblyopia, we found statistically significant correlations among three measurable parameters: the vision before treatment, the amount of anisometropia and the vision after treatment. The predetermined factors associated with success of treatment were 1) lower amounts of anisometropia, particularly less than six diopters for myopes, 2) better initial vision, and 3) a younger patient for hyperopic differences. Patients with less than three diopters of anisometropia were more likely to succeed with the use of glasses alone and a trial of glasses did not prolong therapy time. Despite a substantial number of older patients with extreme amounts of anisometropia or very poor vision, the group as a whole did well with 70% achieving 20/40 or better. Thus therapy should be approached enthusiastically, even for highly myopic patients since other authors have reported success with them. The factors under the control of the ophthalmologist which will promote success in these patients are correct glasses prescriptions, particularly with full astigmatic correction, continued use of glasses as maintenance therapy, and patience, since these children required a median of eight months for therapy to be successful.

Adolescent↗
PubMedDate not supplied

Iris hypoplasia in incontinentia pigmenti.

This report describes a young girl with ocular and dermatologic manifestations of incontinentia pigmenti. Fortunately, she has thus far been spared the more severe systemic problems, as well as any vision-threatening complications. However, a unique finding of sector stromal hypoplasia of the iris in her left eye was noted. It is interesting to speculate that this iris pigmentary disturbance may in some way relate to the underlying pigmentary disturbance seen in the skin and in the retinal pigment epithelium.

Child, Preschool↗
PubMedDate not supplied

Acquired retraction syndrome associated with orbital metastasis.

A patient with metastatic disease of the lateral orbit developed an acquired motility defect similar to Duane's retraction syndrome. An acquired retraction syndrome, unlike the congenital form, is due either to mechanical defects of the orbit and eye or to neurologic disease. The direction of gaze in which the retraction occurs is the clinical clue to the location of the restricting element in the mechanically caused cases.

Carcinoma↗
PubMedDate not supplied

Compliance with a standard of care for retinopathy of prematurity in one neonatal intensive care unit.

Standards of care are an integral part of providing safe and proper health care to the public. One such standard is the screening of premature infants for retinopathy of prematurity. In a retrospective chart review of 67 infants in an intensive care nursery, a high incidence of non-compliance with the standard was found in infants with over 1251-g birth weight, but that infants under 1251-g birth weight and eligible for participation in an investigation of retinopathy of prematurity had a high degree of compliance with the standard. These findings resulted in the adoption of recommendations to improve compliance with the standard.

Female↗
PubMedDate not supplied

Follow up retrospective study of compliance with a standard of care for retinopathy of prematurity in one neonatal intensive care unit.

A retrospective chart review was conducted to determine the impact of recently instituted guidelines on compliance with the standard of care for retinopathy of prematurity (ROP) in the Newborn Intensive Care Unit at the University of Utah Medical Center. In a previous study, it was found that infants eligible for participation in a National Institutes of Health-funded study of ROP were routinely receiving screening eye examinations, whereas infants who qualified for ROP screening according to the standard of care, but not eligible for the study, were seen infrequently. Consequently, a plan to improve the compliance with the standard of care for all infants who fit the standard criteria was implemented. The comparison revealed significant improvement in compliance rates. In one subgrouping of infants, compliance rose from three of 16 (18.75%) infants screened for ROP according to the standard of care, to nine of 11 (82%) infants screened for ROP according to the revised standard. This increase in compliance appears to be due to adherence to guidelines recommended in an earlier study, which included increasing staff awareness of the standard of care, designating a person to schedule and track infants who fit the screening criteria, and including the need for ROP screening on the admission and discharge summary.

Female↗
PubMedDate not supplied

The visually inattentive preterm infant.

A retrospective study was made of 16 premature infants who were visually inattentive despite normal eye findings and a lack of factors predisposing them to cerebral blindness. A comparison of this study group with other premature infants who were visually attentive revealed a much greater incidence of upper motor neuron disease and mental retardation in the study infants.

Female↗
PubMedDate not supplied

Periocular infection after strabismus surgery. The Periocular Infection Study Group.

Fewer than 15 cases of cellulitis and subconjunctival abscess after extraocular muscle surgery have been reported in detail. In an effort to provide greater information about this serious complication, the members of the American Association for Pediatric Ophthalmology and Strabismus (AAPO&S) were surveyed and contributed cases were analyzed. Among the 25 cases of cellulitis, there was a predominance of preschool age patients and Staphylococcus aureus cultures. Many cases presented after a normal initial postoperative visit. The most common symptoms were marked swelling and pain. Possible predisposing factors included unsuspected sinusitis, eye rubbing, and poor hygiene. Two patients had documented bacteremia, but no patient developed endophthalmitis. All cases resolved on antibiotics, but only five patients were successfully treated with oral antibiotics alone. Periocular infections after extraocular muscle surgery can present after a normal initial postoperative visit. Symptoms of marked swelling and pain are cause for closer inspection. S. aureus is the most common organism and oral antibiotics alone can fail to halt the infection.

Abscess↗