Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038379 (strabismus)
 9,317 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We examined the latex surgical gloves used by 56 primary surgeons in 454 ophthalmic surgical procedures performed over a 7-month period. Of five techniques used to detect pinholes, air inflation with water submersion and compression was found to be the most sensitive, yielding a 6.80% prevalence in control glove pairs and a 21.8% prevalence in postoperative study glove pairs, for a 15.0% incidence of surgically induced perforations (P = 0.000459). The lowest postoperative perforation rate was 11.4% for cataract and intraocular lens surgery, and the highest was 41.7% for oculoplastic procedures. Factors that correlated significantly with the presence of glove perforations as determined by multiple logistic regression analysis were oculoplastic and pediatric ophthalmology and strabismus surgical procedures, surgeon's status as a fellow in training, operating time, and glove size. The thumb and index finger of the nondominant hand contained the largest numbers of pinholes. These data suggest strategies for reducing the risk of cross-infection during ophthalmic surgery.
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PMID:Occult glove perforation during ophthalmic surgery. 149 36

Soft contact lenses (SCL) were used for the correction of aphakia in young children. Eleven surgeries were carried out in 7 children with congenital cataracts (aspiration in 2 cases and transciliary lensectomy in the rest). SCLs were made individually of gel with 70% water content by mechanical treatment, their parameters differing from the commercial 'aphakic' lenses. These SCL parameters were chosen with consideration for the data of measurements of the corneal curvature and diameter, carried out during surgery and then specified by trials. To determine the optic force of SCL, a special formula and skiascopy were used. Only in one case the parents refused from SCL application, being satisfied with the cosmetic effect of surgery; in the rest cases SCLs were tolerated satisfactorily as evidenced by a follow-up of 3 to 8 months. SCL wearing improved the visual function, that manifested in a more stable fixation, reduction and stabilization of strabismus angle, development of an ability to recognise closely situated small articles. These results are in fact tentative; further studies will be aimed at a more accurate assessment of the visual function and of the corneal status (including the status of the endothelium).
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PMID:[The first results of the contact lens correction of aphakia in young infants]. 259 95

A 100% silicone polymer contact lens can be used successfully for extended wear in pediatric aphakia. Compared to a hydrogel extended wear contact lens, the silicone lens has several advantages. These include easier handling, a less traumatic fitting procedure for the child, fluorescein use for evaluation of the lens-cornea relationship and a lower loss rate. Bacterial and preservative contamination are minimized due to the lower water content. The silicone lens appears to be safe, having no permanent adverse effects on the cornea. It would be more advantageous if the lens could be obtained in higher plus powers; however, the available power (up to +32.00D) is appropriate in almost all cases.
J Pediatr Ophthalmol Strabismus
PMID:Extended wear contact lenses in pediatric aphakia. 385 96

A healthy child developed V. parahaemolyticus endophthalmitis after receiving a perforating corneal injury while swimming in the Gulf of Mexico. Peculiar greenish anterior chamber and vitreous exudates were a striking feature of the infection. A history of ocular injury in a salt water environment should alert the clinician to the possibility of infection due to a marine Vibrio, and appropriate means should be employed for the isolation and identification of such organisms.
J Pediatr Ophthalmol Strabismus
PMID:Vibrio parahaemolyticus endophthalmitis. 696 12

A 4-year-old boy with suprasellar arachnoid cyst was reported. At the age of 30-month-old his aunt was aware of his squint. During the observation by ophthalmologists from the age of 1y. to 3y., enlargement of the head and impairment of the visual acuity were manifested. Cranial CT scan revealed the enlargement of the ventricular system and a round low density area located superior to the sella. Absorption coefficient of the lesion was similar to that of the cerebrospinal fluid. No abnormal contrast enhancement was seen. Examination revealed the head circumference of 53.3 cm larger than doubled standard deviation, the right external strabismus, impaired vision (R:0.03, L:0.3) and optic atrophy but no other neurological signs. Cerebral angiography showed suprasellar mass lesion. After the ventriculography with water-soluble contrast medium, V-P shunt operation was performed and then the patient was transferred to the CT room. CSF enhanced CT scan showed no communication between the ventricles and the cyst. By frontotemporal approach, microsurgical removal of the cystwall was performed and the histological diagnosis was arachnoid membrane. Several days after the operation, bilateral subdural effusion was seen on CT scan and was treated with bilateral S-P shunt and the removal of V-P shunt. Follow up CT scan disclosed the disappearance of the subdural effusion and the suprasellar cyst. The visual acuity was improved well and the endocrinological study was normal. Analysis of the 45 reported cases of suprasellar arachnoid cyst suggested that direct removal of the cyst wall is better than the V-P shunt operation and the cyst shunting is advisable for repeat recurrence of the cyst. Removal of the ventricular shunting system may be effective for the prevention of the subdural effusion as a complication after direct operation.
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PMID:[Suprasellar arachnoid cyst--report of a case (author's transl)]. 709 83

We used an animal model of restrictive strabismus analogous to the fat adherence syndrome in humans to test the efficacy of topical intraoperative mitomycin-C (MMC) in preventing the development of restrictive scar tissue. A cicatricial adhesion was created between the inferior rectus muscle and the inferior orbital rim of each eye in eight rabbits, and passive forced ductions were quantitatively measured with a spring scale. Eight eyes were treated intraoperatively with topical MMC 0.5 mg/mL, the other eight with sterile water. Passive forced ductions were again measured 4 weeks postoperatively and representative orbits were exenterated for histopathologic examination. Significant restriction of motility was produced in six of the eight control eyes. Though prophylactic treatment with MMC may have been beneficial in some cases, on average, the restriction developing in these eyes did not significantly differ from that in the control eyes. In addition, longer exposure times to MMC led to marked orbital inflammation and severe restriction of ocular motility. Finally, histopathologic evaluation of the orbits of the MMC-treated eyes revealed marked fibrosis of perimuscular connective tissues. Although MMC may have a role in the management of fat adherence syndrome, further study is needed to establish safe and efficacious methods of delivery.
J Pediatr Ophthalmol Strabismus
PMID:Fat adherence syndrome treated with intraoperative mitomycin-C: a rabbit model. 896 20

To prevent postoperative croup in children, many anaesthesiologists use a tracheal tube that allows a leak when tested with 20 to 25 cm of water pressure. We studied the correlation of postoperative croup with leak, duration of anaesthesia, and a recent cold in 159 healthy outpatient children who had strabismus correction by the same surgeon and the same anaesthesiologist. We found no correlation between the presence or absence of a leak and the incidence or severity of postoperative croup. There was a strong trend toward significance when postoperative croup and duration of anaesthesia were compared (P = 0.056) and a significant, positive correlation between severe croup (requiring racemic epinephrine) and duration of anaesthesia (P = 0.005). Patients having a recent cold did not have an increased incidence of postoperative croup. A leak around the tracheal tube at 20 to 25 cm of water pressure may not be required for a healthy child who undergoes surgery lasting less than 2 h if the child has no history of croup.
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PMID:Absence or presence of a leak around tracheal tube may not affect postoperative croup in children. 974 33

Mucolipidosis type IV (MLIV) is an autosomal recessive, neurodegenerative, lysosomal storage disorder characterized by psychomotor retardation and ophthalmological abnormalities including corneal opacities, retinal degeneration and strabismus. Most patients reach a maximal developmental level of 12?15 months. The disease was classified as a mucolipidosis following observations by electron microscopy indicating the lysosomal storage of lipids together with water-soluble, granulated substances. Over 80% of the MLIV patients diagnosed are Ashkenazi Jews, including severely affected and mildly affected patients. The gene causing MLIV was previously mapped to human chromosome 19p13.2-13.3 in a region of approximately 1 cM (ref. 7). Haplotype analysis in the MLIV gene region of over 70 MLIV Ashkenazi chromosomes indicated the existence of two founder chromosomes among 95% of the Ashkenazi MLIV families: a major haplotype in 72% and a minor haplotype in 23% of the MLIV chromosomes (ref. 7, and G.B., unpublished data). The remaining 5% are distinct haplotypes found only in single patients. The basic metabolic defect causing the lysosomal storage in MLIV has not yet been identified. Thus, positional cloning was an alternative to identify the MLIV gene. We report here the identification of a new gene in this human chromosomal region in which MLIV-specific mutations were identified.
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PMID:Identification of the gene causing mucolipidosis type IV. 1097 63

Mucolipidosis type IV (MLIV) is a neurodegenerative lysosomal storage disorder characterized by psychomotor retardation and ophthalmological abnormalities, including corneal opacities, retinal degeneration, and strabismus. Severely affected as well as milder patients have been described. Over 80% of the MLIV patients are Ashkenazi Jews; the estimated heterozygote frequency in this population is 1/100. The disease is classified as a mucolipidosis due to the simultaneous lysosomal storage of lipids together with water-soluble substances. A broad spectrum of lipids and acid mucopolysaccharides were identified as the storage substances. Kinetic studies demonstrated that this heterogeneous storage stems from an abnormal endocytosis process in cells from MLIV patients of membrane components from late endosomes to the lysosomes and/or delayed efflux to the Golgi apparatus. The MLIV gene was mapped to chromosome 19p13.2--13.3 where a novel gene, MCOLN1, with MLIV-causing mutations, was identified. Two mutations were found among 95% of the Ashkenazi MLIV alleles, including an intronic acceptor splice-site mutation in 72% of the alleles and a partial gene deletion in 23%. Each of these mutations was associated with a defined haplotype in this chromosomal region. Other mutations were mostly identified in single, Ashkenazi and non-Ashkanazi patients, including missense, nonsense nucleotide deletions, and insertions. All mutations but one were identified in patients exhibiting the severe phenotype, an in-frame amino acid deletion was identified in a mild patient. MCOLN1 encodes a 580 aa protein, mucolipin 1, which is a member of a new protein family of unknown function at present, the mucolipins. Mucolipin 1 is a membrane protein with 6 transmembrane domains, a serine lipase, and nuclear localization signal motives. The protein shows homology to a group of calcium channels of the TRP/TRPL family. The involvement of this protein in the endocytosis process of membrane components is currently studied. A population screening operation among the Ashkenazi population for the detection of heterozygotes has been started in Israel as a prevention program.
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PMID:Mucolipidosis type IV. 1146 Nov 86

Strabismus is the lack of binocular vision due to an inability to control one of the eye muscles. Corrective surgery is the most common recourse and consists of adjusting and reattaching the extraocular muscle to the sclera. In approximately 10% of cases involving re-insertment of the extraocular muscle via suture techniques, the needle is inserted too deeply into the eye resulting in perforation of the retina. Fibrin glues and cyanoacrylates have been substituted with unsatisfactory mechanical results. The goal of this study was to maximize the tensile strength of rabbit extraocular muscles repaired using a laser-solder technique developed by McNally et al., Biodegradable polymer membranes of controlled porosity were fabricated with poly(L-lactic-co-glycolic acid) (PLGA) and salt particles using a solvent-casting and particulate-leaching technique. The porous membranes were doped with protein solder composed of 25% and 50% (w/v) serum albumin and 0.5 mg/ml indocyanine green (ICG) dye mixed in deionized water. In vitro tissue specimens were repaired using the solder-doped polymer membranes in conjunction with an 805 nm diode laser. The tensile strength was tested on an MTS machine and results were analyzed with the Student's T-test.
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PMID:Optimization of laser-solder repair technique for possible application in strabismus surgeries. 1208 31


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