PubMed Health⌕ Search

Biomedical subjects

R M Robb

Publications and source records attributed to R M Robb.

At least 37 records · Page 2Linked to original sources
PubMed1979

Internal ophthalmoplegia following inferior oblique myectomy: a report of three cases.

Three patients are reported in whom paralysis of accommodation and mydriasis (internal ophthalmoplegia) developed following inferior oblique myectomy. In all three patients, accommodation eventually returned to normal; in two, mild anisocoria persisted; in one patient, pupillotonia was noted after 2 1/2 years. The mechanism of this previously unreported surgical complication is thought to be excessive stretching of the nerve to the inferior oblique muscle with secondary trauma to the ciliary ganglion.

Accommodation, Ocular↗
PubMed1978

Pathology of the Lens in Down's syndrome.

A pathological study of the lenses of 21 patients with Down's syndrome disclosed no distinctive abnormalities of the lens capsule, as had been reported previously. Five patients, 15 years of age or older, had focal cortical changes consistent with the characteristic flake-like opacities seen in patients with Down's syndrome after the first decade of life. Three patients with more extensive, visually significant lens opacities showed nonspecific cataractous changes. No abnormalities other than pathological artifacts were seen in the lenses of patients younger than 15 years of age.

Adolescent↗
PubMed1978

Ocular manifestations of group A Niemann-Pick disease.

Four infants with Group A Niemann-Pick disease had similar ocular abnormalities secondary to this systemic disease. Each child demonstrated corneal opacification, brown discoloration of the anterior lens capsule, and retinal opacification with a macular cherry-red spot. These abnormalities were seen in each child during the first year of life and appeared stable. Recognition of this combination of ocular defects facilitates early identification of patients with Group A infantile Niemann-Pick disease.

Cornea↗
PubMed1978

Histochemical demonstration of cyclic guanosine 3',5'-monophosphate phosphodiesterase activity in retinal photoreceptor outer segments.

A technique for the histochemical demonstration of cyclic guanosine monophosphate phosphodiesterase in retina is described. Enzyme activity was identified on photoreceptor outer segment lamellae, a finding in agreement with previous biochemical data on isolated outer segment preparations. The distribution of phosphodiesterase activity for cyclic guanosine monophosphate was similar to that found previously in rod outer segments for cyclic adenosine monophosphate, suggesting that the same enzyme may hydrolyze both nucleotides.

3',5'-Cyclic-GMP Phosphodiesterases↗
PubMed1978

A pathological study of eye involvement in acute leukemia of childhood.

The eyes of 60 children dying of acute leukemia between 1968 and 1976 at the Children's Hospital Medical Center have been examined pathologically. An attempt has been made to relate eye findings to the state of the systemic disease at the time of death. Eight of the 60 patients had leukemia retinal infiltrates and all eight had fulminant disease with terminal leukocyte counts over 100,000 per cubic millimeter and a high percentage of "blast" cells. Twenty-six patients (43%) had leukemic infiltration of the choroid which was not apparent clinically, but which would require therapy in any effort to eradicate leukemic cells from the body. Five of six patients with optic nervic involvement had coexistant meningeal leukemia. Isolated retinal hemorrhages could not be correlated with other parameters of the leukemic process.

Acute Disease↗
PubMed1978

Ocular retardation (or) in the mouse.

The ocular retardation (or) mutation in mice has been studied morphologically in serial 1 mu sections. This recessively inherited, fully penetrant mutation is characterized by an early arrest of retinal development, aplasia of the optic nerve, cataractous degeneration of the lens, and microphthalmia. We describe early alterations of normally occurring morphogenetic cell death in the optic cup and aberrations of optic fissure formation which appear to precede the arrest of retinal and optic nerve development. The subsequent disappearance of central retinal vessels and cataract formation are interpreted as secondary phenomena.

Animals↗
PubMed1977

Sulfite oxidase deficiency. Biochemical and clinical investigations of a hereditary metabolic disorder in sulfur metabolism.

Study of a 4 1/2-year-old boy with the unusual combination of acute infantile hemiplegia, ectopia lentis and the absence of homocystinuria showed large amounts of abnormal sulfur-containing metabolites (sulfite, thiosulfate and S-sulfocysteine) in the urine. Sulfite and S-sulfocysteine were also present in the plasma. His inorganic sulfate excretion was only 50 per cent of total sulfur, as compared with 75 to 95 per cent by controls. Loading with L-cysteine hydrochloride and L-methionine further increased the excretion of sulfite and thiosulfate, but not inorganic sulfate excretion. Sulfite oxidase activity in skin fibroblasts average 1.07 nmol of cytochrome d reduced per milligram of protein per minute in control lines; it was not detectable (less than 5 per cent) in the patient. Activity was reduced in both parents (0.50 in the father and 0.32 in the mother)--compatible with autosomal recessive inheritance. Good biochemical responses to a low sulfur amino acid diet suggest that early treatment may benefit the patient.

Amino Acids, Sulfur↗
PubMed1977

Refractive errors associated with hemangiomas of the eyelids and orbit in infancy.

Asymmetrical refractive errors, both astigmatic and myopic, were associated with infantile hemangiomas of the eyelids and orbit in 46% of 37 patients who had large lesions and upper eyelid involvement predisposing to the ammetropia. The axis of the astigmatic error related to the location of the eyelid hemangioma and correlated closely with keratometric measurements of corneal astigmatism. The refractive errors tended to be stable despite eventual resolution of the hemangiomas. Efforts to combat strabismic and refractive amblyopia were rewarding in many patients. A history of complete eyelid occlusion during part of the first year of life was associated with dense amblyopia and eccentric fixation in some patients, but in other patients this history was compatible with the eventual development of useful vision. Absence of an asymmetrical refractive error in patients with eyelid and orbital hemangiomas rendered the prognosis for vision good in involved eyes.

Astigmatism↗
PubMed1976

Isolated foveal hypoplasia.

Although defective development of the fovea has long been known to occur in patients with aniridia and albinism, only rarely has isolated foveal hypoplasia unassociated with these conditions been reported. We have identified such foveal hypoplasia in nine patients with varying degrees of congenital nystagmus and poor visual acuity, but no evidence of albinism or aniridia, isolated foveal hypoplasia may be more common as a cause of congenital sensory nystagmus than has been appreciated in the past.

Adult↗