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Biomedical subjects

J W Simon

Publications and source records attributed to J W Simon.

At least 73 records · Page 4Linked to original sources

A simple grating visual acuity test for impaired children.

Twenty-five developmentally delayed or neurologically impaired nonverbal children, aged 2 to 15 years, referred for visual acuity assessment, were beyond the age at which standard preferential-looking techniques are considered to be effective and none could be tested with Snellen letters, illiterate Es, or Allen pictures. Our method, in which the children learn to point to grating stimuli, enabled us to obtain monocular visual acuity estimates in 19 of the 25 patients. When indicated, patching therapy was begun and was monitored with this method. Similar testing of 31 unimpaired children showed good prediction of recognition visual acuities except in patients with visual acuities worse than 20/160. The grating method accurately identified or excluded amblyopia in 26 of 31 children (84%). We consider this a clinically useful test of visual acuity in nonverbal impaired children.

Adolescent↗

Albinotic characteristics in congenital nystagmus.

We studied 26 white patients (19 males and seven females), ranging in age from 1 to 57 years, with congenital nystagmus for the characteristics of albinism. None of the patients was known to be an albino and none had readily apparent signs of albinism. The study also included 26 controls matched by age, sex, race, and complexion. Twelve of the 26 patients with congenital nystagmus had one or more of the following characteristics: detectable iris transillumination (six of 25 patients), choroidal depigmentation (four of 22 patients), and abnormal tanning history (eight of 26 patients). Iris transillumination and abnormal tanning were not found in any of the controls but two controls showed choroidal depigmentation and one showed blunting of the macular reflex (this was determined to be age-related). These differences were significant for iris transillumination (P = .01), blunting of the macular reflex (P = .01), and abnormal tanning (P = .002) but not significant for choroidal depigmentation (P = .38). Within the group of patients with congenital nystagmus, iris transillumination was significantly associated with blunting of the macular reflex (P = .03), choroidal depigmentation (P = .03), and abnormal tanning (P = .004) but not with eye color (P = .16) or visual acuity (P = .33). These findings suggested that many patients with apparently isolated congenital nystagmus may have subclinical albinism or a form of albinoidism.

Adolescent↗

Reflux and voiding abnormalities in children.

Twenty-four patients with vesicoureteral reflux were studied urodynamically because of a history of dysfunctional voiding. Nine patients eventually had antireflux surgery. The remaining 15 patients were followed from two to six years. These patients were managed conservatively with an emphasis on controlling infection and improving voiding habits. Reflux resolved spontaneously in 12 patients and improved in 1 patient. Dysfunctional voiding is a definite cause of childhood reflux. Treatment should be directed at improving voiding patterns. Coordinated voiding may allow a child to "grow out" of his reflux.

Counseling↗

Congenital HIPO syndrome.

A newborn girl had a combination of hemihypertrophy, intestinal web, preauricular skin tag, and congenital corneal opacity. This association is contrasted to Goldenhar's syndrome.

Adult↗

Study of acute trichinosis in Ghurkas: specificity and sensitivity of enzyme-linked immunosorbent assays for IgM and IgE antibodies to Trichinella larval antigens in diagnosis.

The enzyme-linked immunosorbent assay (ELISA) was successfully applied to detect IgE antibodies against parasitic antigens by using an additional antibody layer to attain an amplification effect. The sera of 18 Gurkha patients with clinical manifestations of acute trichinosis and 35 Chinese with other parasitic infections were tested for antibodies to Trichinella spiralis by IgE-, IgM- and IgG-ELISA, IgG-radioimmunoassay (RIA) and indirect haemagglutination test (IHA). ELISAs for detection of IgE and IgM antibodies provided a 100% specific and sensitive diagnosis. Although IHA, IgG-RIA and IgG-ELISA detected antibodies in 94% of patients, non-specific reactions were also observed in the two last named methods. Muscle biopsies were positive in only 56% of patients.

Biopsy↗

Ophthalmic manifestations of the Hermansky-Pudlak syndrome (oculocutaneous albinism and hemorrhagic diathesis).

We report the association of oculocutaneous albinism and defective platelet function, the Hermansky-Pudlak syndrome, in two young Puerto Rican patients, a 17-year-old boy and a 9-year-old girl. Wide variation in pigmentation may obscure the diagnosis of albinism. Puerto Rican albinos are at increased risk of inheriting this autosomal recessive syndrome. Although usually mild, bleeding in affected patients can be life-threatening, especially after aspirin administration. Albino patients should be questioned about a history of bleeding and referred for hematologic consultation.

Adolescent↗

Ocular reticulum cell sarcoma.

Although rare, ocular reticulum cell sarcoma presents a recognisable clinical pattern, as confirmed by 3 new cases. Typical patients, in their sixth and seventh decades, initially complain of gradual visual loss. Examination reveals 'uveitis' with prominent vitreous debris and/or chorioretinal infiltrative lesions. Topical steroid and mydriatic therapy is ineffective. Reticulum cell sarcoma in the central nervous or other systems may precede or accompany the ophthalmic presentation. In an increasing number of cases tissue diagnosis and effective therapy have followed vitreous aspiration.

Aged↗

Congenital esotropia.

Congenital esotropia represents the most common type of strabismus. Its pathogenesis, however, remains uncertain. It is typically characterized as a large angle, constant esotropia with onset during the first six months of life. Associated clinical findings include normal refractive errors for age, amblyopia, dissociated vertical deviation, inferior oblique muscle overaction and nystagmus. It must be distinguished from Duane's retraction syndrome, Moebius syndrome, nystagmus blockage syndrome, and early onset accommodative esotropia, as well as other causes of esotropia in infancy. The surgical management may involve recession of both medial recti muscles, unilateral recession of a medial rectus muscle and a resection of a lateral rectus muscle or three or four muscle surgery.

Botulinum Toxins↗

Ocular colobomata.

Ocular colobomata present diagnostic and therapeutic challenges in patients of all ages, but especially in young children. The "typical" coloboma, caused by defective closure of the fetal fissure, is located in the inferonasal quadrant, and it may affect any part of the globe traversed by the fissure from the iris to the optic nerve. Ocular colobomata are often associated with microphthalmia, and they may be idiopathic or associated with various syndromes. Types and severity of complications vary depending on the location and size of the colobomata. This article reviews the pathogeneses, categorization, genetic bases, differential diagnoses and management of ocular coloboma.

Abnormalities, Multiple↗

Anterior segment ischemia following strabismus surgery.

Anterior segment ischemia (ASI) is a potentially blinding complication of extensive eye muscle surgery. Eyes have been described with this complication following surgery on as few as two rectus muscles, especially in patients with thyroid dysfunction or other medical illnesses. In an attempt to assess the risk of ASI, we have reviewed the records of 34 eyes in 26 patients who underwent surgery on three or four rectus muscles. Seven eyes of six patients with thyroid ophthalmopathy which underwent two-or three-muscle surgery were also studied. Evidence of clinically significant ASI was apparent in only one patient during a follow-up period of seven months to eleven years. This case was mild and no visual loss resulted. Our findings suggest that surgery on three or four rectus muscles in healthy patients is probably safe when performed in a staged fashion.

Adolescent↗

Familial simple ectopia lentis: a case study.

Hereditary simple ectopia lentis affected nine patients in three generations of a family. Inheritance appeared to be autosomal dominant. Examination of 12 family members, employing body proportion measurements, chest x-ray, echocardiogram, and urinary cystine or blood methionine levels, revealed no evidence of any systemic disease. In all cases except two, lenses were bilaterally and superiorly dislocated. The degree of dislocation varied considerably among those affected, causing no visual disturbance in some and severely limiting visual acuity in others. Visual deficits were greatest in patients with intermediate degrees of dislocation. To date, the only known complications related to the dislocations have been two cases of bilateral cataracts. The indications for lensectomy in patients with ectopia lentis are reviewed.

Ectopia Lentis↗

Prosthetic treatment of severe microphthalmos in infancy.

Four infants with blind, severely microphthalmic eyes have been treated with cosmetic scleral shells. Through periodic refitting, this method has permitted the development of symmetrical ocular appearance without the need for surgery. We recommend that this treatment be initiated in early infancy in appropriate patients.

Child↗

Preferential looking as a guide for amblyopia therapy in monocular infantile cataracts.

Despite recent advances in surgical technique and refractive correction, amblyopia remains a significant problem in the treatment of monocular infantile cataracts. Amblyopia therapy recommended by different authors during the preverbal period has varied from no patching to nearly full-time occlusion of the phakic eye. We measured preferential looking (PL) acuities sequentially in four infants following monocular cataract surgery and contact lens correction. This information was used to adjust occlusion of the phakic eye, which was prescribed 25%-100% of waking hours. Patching schedules were altered, on average, every three to four months in response to changes in PL acuities. Four increases in patching intensity retarded acuity development in the phakic and benefited development in the aphakic eyes. Nine decreases enhanced progress or yielded no change in the phakic and, with two exceptions, impeded progress in the aphakic eyes. Recognition acuities now available for two of the children show continuity with previous PL acuities and confirm useful vision in the aphakic eyes. We describe guidelines for adjusting occlusion based on our experience. We believe that PL modulation of patching therapy may result in more favorable visual outcomes for both eyes of infants with monocular cataracts.

Amblyopia↗