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Biomedical subjects

A L Young

Publications and source records attributed to A L Young.

At least 19 recordsLinked to original sources
PubMed2006

CYLD mutations underlie Brooke-Spiegler, familial cylindromatosis, and multiple familial trichoepithelioma syndromes.

Brooke-Spiegler syndrome (BSS), familial cylindromatosis (FC), and multiple familial trichoepithelioma (MFT), originally described as distinct inherited disorders, are characterized by a variety of skin appendage neoplasms. Mutations in the CYLD gene are found in individuals with these syndromes. We describe a single family with affected members exhibiting either the FC or the MFT phenotypes associated with a mutation in the CYLD gene. These findings support the notion that BSS, FC, and MFT represent phenotypic variation of a single defect. Of interest, one of the affected individuals described in this report exhibits a severe phenotype illustrating the morbidity of the disorder.

Aged↗
PubMed2005

A modified technique of scleral fixated intraocular lenses for aphakic correction.

PURPOSE: To examine the safety and efficacy of a modified scleral fixated intraocular lens (SFIOL) technique combining a scleral tunnel for intraocular lens (IOL) insertion, horizontal passage of sutures, and performing anterior vitrectomy through self-sealing pars plana sclerostomies. METHODS: Retrospective review of all patients who underwent SFIOL for aphakic correction at the Department of Ophthalmology and Visual Sciences, The Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong (a tertiary referral centre) between October 2000 and December 2001 with either the modified or conventional techniques. RESULTS: A total of 20 eyes were identified. Eight modified (Group 1) and 12 conventional (Group 2) with a mean follow-up of 18.3 months. Postoperatively, all the eyes improved on unaided preoperative acuity and they all had a stable and well-positioned IOL. In all, 85% (7/8) of eyes in Group 1 maintained or improved best-corrected visual acuity. CONCLUSIONS: The modified SFIOL technique was found to be as safe and effective as conventional techniques. The main advantages of this technique include easy intraoperative suture management and superior globe maintenance.

Aged↗
PubMed2005

Assessing automobile head restraint positioning in Portland, Oregon.

OBJECTIVE: Automobile head restraints, when used properly, have been shown to decrease the incidence and severity of whiplash injuries to the neck. Before the development of a public campaign on proper head restraint positioning, the authors assessed head restraint positioning and public understanding. DESIGN: Over a one month period, the position of the vehicle head restraint of drivers was observed in moving cars in the city of Portland, Oregon (population 530,000). Optimal position was defined as having the head restraint above the ears with the back of the head touching the head restraint. A questionnaire on head restraint understanding was administered to people during jury service. RESULTS: Of the 4287 drivers observed, 1% (n = 30) had no head restraint on their seat, 4% (n = 158) had a fixed head restraint, and 95% (n = 4099) had an adjustable head restraint. Among the fixed head restraints, 21% (33/158) were positioned optimally with no horizontal gap. Among the adjustable head restraints, only 7% (280/4099) had optimal head restraint positioning. Overall, 93% (3974/4287) of all head restraints observed were suboptimally positioned. Seventy five percent (38/51) of polled Portland residents identified safety as the primary head restraint function. CONCLUSION: Ninety three percent of all head restraints observed were suboptimally positioned. Fixed head restraints were three times more likely to be in optimal position than adjustable head restraints (21% v 7%). Most polled Portland residents understood the proper function and positioning of head restraints. This discrepancy between actual practice and understanding should be addressed with public education and manufacturer design changes.

Automobiles↗
PubMed2004

The use of autologous serum tears in persistent corneal epithelial defects.

PURPOSE: Persistent corneal epithelial defects (PED) present a very challenging problem to anterior segment surgeons. Autologous serum tears had been demonstrated to be beneficial in the treatment of PED. The current study was conducted to review the local spectrum of indications and to examine the outcome of autologous serum tear usage. METHODS: All cases of PED treated with autologous serum tears at a tertiary referral centre for the period August 1999 - July 2001 were identified and reviewed. RESULTS: A total of 10 eyes from 10 patients were identified (5OD : 5OS). The gender ratio was 7M : 3F and the mean age was 36.8 (range 17-73) years old. The mean duration of PED before the usage of autologous serum tears was 22.4+/-69.6 days. Six eyes healed within 2 weeks, but two eyes failed to heal after 1 month of treatment and two patients defaulted follow-up. No adverse effects were observed with the addition of autoserum tears. CONCLUSIONS: The results of the current study correlated well with previous reported studies. Autologous serum tears may be considered as a valuable adjunct in the management of recalcitrant cases of PED.

Adolescent↗
PubMed2004

The prevalence of pseudoexfoliation syndrome in Chinese people.

BACKGROUND: Pseudoexfoliation syndrome (PXS) is regarded as rare in people of Chinese ethnicity but the prevalence of this condition is not known. This epidemiology study was conducted to assess the prevalence of PXS in cataract patients and to report the clinical features present. METHODS: Prospective descriptive study conducted in the period from March 1999 to May 2001 in ophthalmology departments in cluster hospitals serving a population of about 1.2 million. 500 consecutive patients aged 60 or above attending the general ophthalmic clinics with a presumed diagnosis of cataract were recruited. A detailed examination including biomicroscopy, intraocular measurement, and gonioscopy were performed on all patients. All positive PXS cases were documented photographically. RESULTS: 500 patients were examined. They ranged from the ages of 60 to 91 years old, with a male to female ratio of 1:2. 40% suffered from hypertension while 24% were known diabetics. Only two positive cases (0.4%) of PXS were identified in the study population. 18% of all eyes were found to have narrow angles (defined as grade 0 to 2 by Shaffer grading). Nuclear sclerosis was the single most common type of lens opacity. CONCLUSION: PXS is a rare condition in Chinese people. A prevalence rate of 0.4% in patients aged 60 or above was identified in this hospital based epidemiology study. To the best of our knowledge, this was the first study conducted in a Chinese population to examine the prevalence of PXS.

Age Distribution↗
PubMed2004

A randomised trial comparing 0.02% mitomycin C and limbal conjunctival autograft after excision of primary pterygium.

BACKGROUND: Mitomycin C (MMC) and limbal conjunctival autograft (LCAU) are two known useful adjuvants in the prevention of pterygial recurrence. This study was conducted to compare the outcome of these two treatments. METHODS: Prospective study on consecutive cases of primary pterygium (February 2001 to March 2002) randomised into two adjuvant groups: (1) intraoperative 0.02% MMC for 5 minutes or (2) LCAU. Patients were followed for recurrence (defined as fibrovascular tissue invading the cornea >1.5mm) and complications for a period of one year. RESULTS: 115 eyes in 114 patients who completed the study were randomised to receive MMC (n = 63) and LCAU (n = 52). There were 10 recurrences (15.9%) in the MMC group and only one recurrence (1.9%) in the LCAU group. There was a statistically significant difference in the recurrence rate between the two groups (p = 0.04). There were a total of three conjunctival cysts, three symblephara, one granuloma, and one dellen. No other visually significant complications were encountered in either group. CONCLUSION: Although LCAU resulted in better one year success rates, it is technically more difficult and inapplicable in cases with previous limbal disturbance. Simple excision followed by MMC or LCAU are both safe and acceptable adjuvants for pterygium excision. Choice of adjuvant should be carefully made based on assessment of recurrence risk, local practices, and surgeon's expertise.

Adult↗
PubMed2002

Combined intravenous pulse methylprednisolone and oral cyclosporine A in the treatment of corneal graft rejection: 5-year experience.

PURPOSE: To report the mid-term results of a treatment strategy using topical steroids, intravenous pulse methyl prednisolone and oral cyclosporine A (CSA) for the treatment of acute corneal graft rejection. METHODS: Noncomparative, interventional case series. Treatment of corneal graft rejection included 1% prednisolone eye drops, intravenous infusion of 500 mg methyl prednisolone, and oral CSA in two regimens--standard dose was 15 mg/kg/day for 2 days, 7.5 mg/kg/day for 2 days, then adjusted to maintain trough blood levels of 100-200 microg/l; low dose was 2 mg/kg/day with no loading dose. RESULTS: Outcome in 34 eyes of 34 patients (21 M;13 F) aged 60 +/- 17.7 years (range 9-83 years), who presented after an average duration of 6.6 +/- 6.3 days (range 0-30 days) following acute corneal graft rejection, are reported. Twenty-five patients received standard dose CSA while nine patients received the low dose regimen. Mean duration of treatment before reversal of graft rejection was 13.6 +/- 12.1 days (range 3-54 days). Treatment was successful in reversing the graft rejection in 32/34 (94%) eyes. Irreversible graft failure occurred in one eye in each group. During a mean follow-up period of 19.2 +/- 16.7 months (range 1-55 months), further episodes of graft rejection were seen in 1/32 (3%) eyes. Complications due to treatment included: duodenal ulcer in one patient that responded to medical treatment, and transient elevation in serum creatinine levels in three patients, which returned to normal after decrease in dosage or cessation of oral CSA. CONCLUSION: Our 5-year experience with the use of oral CSA in the treatment of acute corneal graft rejection has shown this treatment approach to be safe and effective in reversing the rejection process. This approach may also protect the graft from subsequent episodes of allograft rejection. A randomised controlled trial to further delineate the role of CSA in reversing acute graft rejection seems warranted.

Administration, Oral↗
PubMed2001

Structural integrity of histone H2B in vivo requires the activity of protein L-isoaspartate O-methyltransferase, a putative protein repair enzyme.

Protein L-isoaspartate O-methyltransferase (PIMT) is postulated to repair beta-aspartyl linkages (isoaspartyl (isoAsp)) that accumulate at certain Asp-Xaa and Asn-Xaa sites in association with protein aging and deamidation. To identify major targets of PIMT action we cultured rat PC12 cells with adenosine dialdehyde (AdOx), a methyltransferase inhibitor that promotes accumulation of isoAsp in vivo. Subcellular fractionation of AdOx-treated cells revealed marked accumulation of isoAsp in a 14-kDa nuclear protein. Gel electrophoresis and chromatography of nuclei (3)H-methylated in vitro by PIMT revealed this protein to be histone H2B. The isoAsp content of H2B in AdOx-treated cells was approximately 18 times that in control cells, although no isoAsp was seen in other core histones, regardless of treatment. To confirm the relevance and specificity of this effect, we measured isoAsp levels in histones from brains of PIMT knockout mice. IsoAsp was found at near stoichiometric levels in H2B extracted from knockout brains and was at least 80 times greater than that in H2B from normal mice. Little or no isoAsp was detected in H2A, H3, or H4 from mice of either genotype. Accumulation of isoAsp in histone H2B may disrupt normal gene regulation and contribute to the reduced life span that characterizes PIMT knockouts. In addition to disrupting protein function, isoAsp has been shown to trigger immunity against self-proteins. The propensity of H2B to generate isoAsp in vivo may help explain why this histone in particular is found as a major antigen in autoimmune diseases such as lupus erythematosus.

Adenosine↗
PubMed2001

Efficacy of lignocaine gel for outpatient laser treatment in inflamed eyes.

PURPOSE: To evaluate the efficacy and safety of topical 2% lignocaine gel in providing analgesia during outpatient transpupillary or trans-scleral laser treatment of inflamed eyes. METHODS: A prospective study was carried out of consecutive eligible eyes undergoing laser treatment using 2% lignocaine gel as a topical anaesthetic and a coupling medium. At the conclusion of each procedure, patients were asked to grade a pain score (0 = no discomfort, 1 = mild discomfort, 2 = mild pain, 3 = moderate pain, 4 = severe pain). RESULTS: Twenty eyes in 19 patients received laser treatment. No pain was reported in 95% of cases treated (no discomfort in 75%, mild discomfort in 20%) and only mild pain in 5% (one patient). No adverse reactions were encountered in any of the patients. There were no complications associated with the procedures. CONCLUSIONS: Lignocaine 2% gel is safe and effective for outpatient transpupillary and trans-scleral laser treatment in inflamed eyes, providing adequate analgesia and serving as a coupling medium at the same time.

Administration, Topical↗
PubMed2001

Mitral valve regurgitation causing right upper lobe pulmonary edema.

When radiography is performed in patients with mitral regurgitation, cardiogenic pulmonary edema is a typical finding; however, asymmetric pulmonary edema has also been reported. We describe the case of a patient in whom mitral valve regurgitation caused isolated pulmonary edema in the right upper lung. We include a discussion of pulmonary edema in conjunction with mitral regurgitation.

Diagnosis, Differential↗
PubMed2000

Ocular hypertensive response to topical dexamethasone in children: a dose-dependent phenomenon.

OBJECTIVE: To investigate the ocular-hypertensive response to different dosages of topical dexamethasone eye drops in Chinese children. DESIGN: Prospective, randomized clinical trial. PARTICIPANTS: Thirty-one consecutive children undergoing bilateral strabismus surgery. INTERVENTION: Topical dexamethasone (0.1%) was administered to children undergoing bilateral strabismus surgery. They were all less than 10 years of age. One eye was randomized to receive a regimen of four times daily, and another received a twice daily regimen. Intraocular pressure (IOP) was serially measured in the postoperative period for 4 weeks or more. Topical steroids were discontinued if IOP was 30 mmHg or more. MAIN OUTCOME MEASURES: Intraocular pressure was measured on the day before the surgery, on postoperative days 1, 3, 5, 8, 12, 15, 22, 29, and 2 weeks thereafter until the IOP reached preoperative levels. Peak IOP, IOP net increase, and time to reach an IOP more than 20 mmHg in the two study groups were analyzed. RESULTS: A total of 31 patients (20 male, 11 female) were examined. The mean age was 5.8 +/- 2.0 years (range, 2-10 years). Preoperative IOP in groups treated twice daily and four times daily were similar. After topical dexamethasone treatment, both groups showed a significant rise in peak IOP compared with preoperative values (twice daily, 25.2 +/- 6.8 mmHg vs. 14.3 +/- 2.4 mmHg, P < 0.001; four times daily, 28.7 +/- 6.9 mmHg vs. 14.3 +/- 2.9 mmHg, P < 0.001). The peak IOP was significantly higher in the four times daily group (P < 0.001), as was the net increase in IOP (twice daily, 10.9 +/- 5.8 mmHg vs. four times daily, 14.5 +/- 6.4 mmHg; P < 0.001). There was no difference in time for both groups to achieve the peak IOP, but the time to exceed its upper normal value (20 mmHg) was shorter in the four times daily group (twice daily, 12.3 +/- 9.1 days vs. four times daily, 10.0 +/- 7.4 days; P < 0.05). CONCLUSIONS: In children treated with topical dexamethasone, ocular hypertension occurs in a dose-dependent manner. Children in the four times daily group had a quicker onset and more severe ocular hypertensive response than the twice daily group. Nevertheless, even the twice daily regimen produced significant IOP rise, suggesting that dexamethasone use in children should be avoided if possible, and it would be desirable to monitor the IOP twice weekly when it is administered to children.

Administration, Topical↗
PubMed2000

Corneal laceration with total but isolated aniridia caused by a pecking injury.

A 38-year-old man sustained a left eye injury after being attacked by a mynah bird. Ocular examination revealed a beak-shaped, full-thickness corneal laceration with total aniridia in the left eye. No other ocular injury was observed, and the lens and posterior segment remained normal over the subsequent 3 months. A penetrating keratoplasty was planned for visual restoration.

Adult↗
PubMed2000

Scleral fixation of a capsular tension ring for severe ectopia lentis.

A 4-year-old boy with bilateral idiopathic and progressive ectopia lentis had phacoaspiration of the lens and capsular tension ring (CTR) insertion in his left eye. Postoperatively, lens capsule centration remained poor. Single-point scleral fixation of the CTR was performed and centration of the lens capsule achieved. In-the-capsule intraocular lens (IOL) implantation was tried, but excessive IOL manipulation sliced open the capsule at the equator. Anterior vitrectomy and scleral IOL fixation were required. A similar approach was adopted in the right eye with meticulous attention given to gentle maneuvers. The operation was uneventful, with good IOL centration. Three and 11 months after surgery in the right and left eye, respectively, best corrected visual acuity was 20/50 in both eyes. Scleral fixation of the CTR, accompanied by gentle IOL manipulation, is an option to improve IOL centration in patients with severe zonular deficiency.

Child, Preschool↗