Short and long-term acceptability and efficacy of extended-release cornstarch in the hepatic glycogen storage diseases: results from the Glyde study.

BACKGROUND: Hypoglycaemia is the primary manifestation of all the hepatic types of glycogen storage disease (GSD). In 2008, Glycosade®, an extended-release waxy maize cornstarch, was reported as an alternative to uncooked cornstarch (UCCS) which could prolong the duration of fasting in the GSD population. To date, there has been minimal published experience in (a) young children, (b) the ketotic forms of GSD, and (c) with daytime dosing. The Glyde study was created as a prospective, global initiative to test the efficacy and tolerance of Glycosade use across a broader and more diverse population. METHODS: A randomised double-blind cross-over fasting study assessing the tolerance and efficacy of Glycosade compared with cornstarch was performed across disease types and ages. Participants and clinicians chose the product deemed superior, whilst still blinded. Participants were followed for 2 years to assess long-term metabolic control, growth, and quality of life. RESULTS: Sixty-one participants (age 2-62 years; 59% female) were enrolled, and 58 participants completed the fasting studies (28 GSD I; 30 GSD III, VI, IX). Glycosade improved duration of fasting in GSD I and duration of fasting without ketosis in the ketotic forms. Chronic Glycosade use was chosen by 69% of participants. Those treated with Glycosade for the 2-year chronic phase used fewer doses of therapy while markers of metabolic control remained stable. CONCLUSION: The Glyde study is the first multi-centre international trial demonstrating the efficacy and tolerance of Glycosade in a large cohort of hepatic GSD patients across a diverse international population. The ability to use fewer doses of therapy per day and avoidance of overnight therapy may improve compliance, safety, and quality of life without sacrificing metabolic control.

Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach.

BACKGROUND: There appears little consensus concerning protein requirements in phenylketonuria (PKU). METHODS: A questionnaire completed by 63 European and Turkish IMD centres from 18 countries collected data on prescribed total protein intake (natural/intact protein and phenylalanine-free protein substitute [PS]) by age, administration frequency and method, monitoring, and type of protein substitute. Data were analysed by European region using descriptive statistics. RESULTS: The amount of total protein (from PS and natural/intact protein) varied according to the European region. Higher median amounts of total protein were prescribed in infants and children in Northern Europe (n=24 centres) (infants <1 year, >2-3g/kg/day; 1-3 years of age, >2-3 g/kg/day; 4-10 years of age, >1.5-2.5 g/kg/day) and Southern Europe (n=10 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, 2 g/kg/day; 4-10 years of age, 1.5-2 g/kg/day), than by Eastern Europe (n=4 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, >2-2.5 g/kg/day; 4-10 years of age, >1.5-2 g/kg/day) and with Western Europe (n=25 centres) giving the least (infants <1 year, >2-2.5 g/kg/day, 1-3 years of age, 1.5-2 g/kg/day; 4-10 years of age, 1-1.5 g/kg/day). Total protein prescription was similar in patients aged >10 years (1-1.5 g/kg/day) and maternal patients (1-1.5 g/kg/day). CONCLUSIONS: The amounts of total protein prescribed varied between European countries and appeared to be influenced by geographical region. In PKU, all gave higher than the recommended 2007 WHO/FAO/UNU safe levels of protein intake for the general population.

Postoperative complications of excimer laser photorefractive keratectomy for myopia.

Excimer laser photorefractive keratectomy has become an increasingly popular surgical treatment for the correction of myopia. Unfortunately, no surgical technique can be entirely without complications. This article discusses the problems encountered following PRK for myopia in two main sections: those primarily refractive or optical in nature and those causing medical problems.

Secondary implantation of open-loop, flexible, anterior chamber intraocular lenses.

PURPOSE: To assess the results of implantation of secondary, open-loop, flexible, anterior chamber intraocular lenses (IOLs) and compare the findings with those of other published studies. SETTING: A combined ophthalmology and ear, nose, and throat hospital in Middlesbrough, Cleveland, England. METHODS: This retrospective study comprised 81 patients who had secondary implantation of a flexible, open-loop, anterior chamber IOL by the one surgeon. The incidence of postoperative complications was ascertained, and best corrected preoperative and postoperative visual acuities were compared. RESULTS: Two serious complications occurred: one severe loss of vision that is still under investigation and one retinal detachment that was repaired with good residual visual function. Of all 81 patients, 92.5% were within one Snellen line of their preoperative best corrected visual acuity. These results compare favorably with those of other published studies. CONCLUSION: Using an open-loop, flexible, anterior chamber lens for secondary implantation is still an acceptable way to treat aphakia. The poor reputation of these lenses is undeserved.

Flexible open-loop anterior chamber intraocular lens implantation after posterior capsule complications in extracapsular cataract extraction.

OBJECTIVES: To analyze 50 cases of flexible open-loop anterior chamber intraocular lens (IOL) implantation after posterior capsule rupture and vitreous loss and compare the results with those of other published series. SETTING: North Riding Infirmary, Cleveland, England. METHODS: This retrospective study, with a follow-up between 3 and 81 months, comprised all patients who had a flexible open-loop anterior chamber IOL implanted because of significant posterior capsule problems during extracapsular cataract extraction. The incidence of postoperative complications and best corrected visual acuity results were determined. RESULTS: Three patients with cystoid macular edema achieved final best corrected visual acuity of 6/9 (20/30). There was one retinal detachment and one traumatic incision rupture with IOL loss 6 weeks postoperatively and subsequent retinal detachment and phthisis. Seventy-two percent achieved a best corrected visual acuity of 6/9 (20/30). CONCLUSIONS: Our results agree with those of other published series. Implantation of a flexible open-loop anterior chamber IOL as a primary lens is still an acceptable way of treating posterior capsule complications in cataract surgery.

Some mechanical properties of 10/0 monofilament nylon sutures.

A short pilot study is described which investigates some of the mechanical properties of ophthalmic sutures. The Alcon 10/0 monofilament nylon suture was found to behave in a mechanically consistent manner. Like all nylon it possesses the property of 'creep', which helps to compensate for overtightening of the section during surgery.