Anterior capsule adherence to iris leading to pseudophakic pupillary block

We present a rare case of anterior capsule adherence to the iris following extracapsular cataract extraction with posterior chamber intraocular lens implantation and leading to pseudophakic pupillary block. There were no synechiae at the pupillary margins associated with the capsule/iris adherence, but aqueous was entrapped behind the iris and intraocular pressure rose. Laser iridotomy was temporarily beneficial, but it had to be repeated several times.     

Missense mutations in the human glutathione synthetase gene result in severe metabolic acidosis, 5-oxoprolinuria, hemolytic anemia and neurological dysfunction

Severe glutathione synthetase (GS) deficiency is a rare genetic disorder with neonatal onset. The enzymatic block of the gamma-glutamyl cycle leads to a generalized glutathione deficiency. Clinically affected patients present with severe metabolic acidosis, 5- oxoprolinuria, increased rate of hemolysis and defective function of the central nervous system. The disorder is inherited in an autosomal recessive mode and, until recently, the molecular basis has remained unknown. We have sequenced 18 GS alleles associated with enzyme deficiency and we detected missense mutations by direct sequencing of cDNAs and genomic DNA. In total, 13 different mutations were identified. Four patients were found to be compound heterozygotes and two individuals were apparently homozygous. Reduced enzymatic activities were demonstrated in recombinant protein expressed from cDNAs in four cases with different missense mutations. The results from biochemical analysis of patient specimens, supported by the properties of the expressed mutant proteins, indicate that a residual activity is present in affected individuals. Our results suggest that complete loss of function of both GS alleles is probably lethal. It is postulated that missense mutations will account for the phenotype in the majority of patients with severe GS deficiency.      

Mobile Application for Promoting Gluten-Free Diet Self-Management in Adolescents with Celiac Disease: Proof-of-Concept Study

Celiac disease (CD) is a chronic disease treated by maintaining and managing a lifelong restrictive gluten-free diet. The purpose of this study was to develop a mobile application, Plan My C-Day, to promote self-management skills among youth with CD during adolescence—a time when decreased adherence often occurs—and examine its usability among adolescents with CD. Plan My C-Day contains three simulations of activities involving eating out and actions to take when preparing for these events. It was developed and pilot tested by 13 adolescents with CD. Application use and user perception data were collected and analyzed. Participants chose 160 actions within the simulations. For over 75% of participants, the time to complete the simulation decreased from the first to the third (last) simulation by an average of 50%. The average reported usability perception was 3.71 on a scale of 1 to 5, with system ease of use and ease of learning obtaining the highest scores. This study demonstrated that the Plan My C-Day mobile application’s self-management content, features, and functions operated well and that the simulations were easy to understand and complete. Further development will include the option to add self-created activities and adaptation to different languages and cultures.     

The long-term effect on intraocular pressure of a procedure combining trabeculectomy and cataract surgery, as compared with trabeculectomy alone

The long-term reduction of intraocular pressure (IOP) resulting from a procedure combining extracapsular cataract extraction and posterior chamber lens implantation with trabeculectomy was compared retrospectively with the IOP-lowering effect of trabeculectomy alone. Forty patients who underwent the combined procedure and 38 who underwent trabeculectomy alone had been followed for an average of 22 +/- 7 months. Both these surgical procedures significantly reduced IOP, but after a year or more, pressure levels were significantly lower in the trabeculectomy group than they were in the combined group: 12.8 +/- 4.2 mm Hg, and 16.5 +/- 5.6 mm Hg, respectively, at 18 months. Also, the mean postoperative fall in IOP was greater in the "filtered" eyes than it was in the combined group (9.8 +/- 4.6 mm Hg and 12.1 +/- 5.2 mm Hg, respectively, at 18 months). Finally, the number of medications required to maintain controlled IOP in the combined group was greater (and resumed preoperative values at 2 years) than it was in the trabeculectomy group (62.5% of the filtered eyes remained controlled unaided).     

A methodology model for quality management in a general hospital

A reappraisal is made of the relevance of industrial modes of quality management to the issues of medical care. Analysis of the nature of medical care, which differentiates it from the supplier-client relationships of industry, presents the main intrinsic characteristics, which create problems in application of the industrial quality management approaches to medical care. Several examples are the complexity of the relationship between the medical action and the result obtained, the client's nonacceptance of economic profitability as a value in his medical care, and customer satisfaction biased by variable standards of knowledge. The real problems unique to hospitals are addressed, and a methodology model for their quality management is offered. Included is a sample of indicator vectors, measurements of quality care, cost of medical care, quality of service, and human resources. These are based on the trilogy of planning quality, quality control, and improving quality. The conclusions confirm the inadequacy of industrial quality management approaches for medical institutions and recommend investment in formulation of appropriate concepts.     

Comparison of the effects of argon and neodymium:YAG laser iridotomy on prostaglandin E2 and blood-aqueous barrier disruption

We compared in rabbits the effects of laser iridotomy by either argon or neodymium (Nd):YAG laser on prostaglandin E2 (PGE2) production by the iris/ciliary body, its accumulation in the aqueous humor and the aqueous protein levels. PGE2 production by the iris/ciliary body, following exposure to argon or Nd:YAG laser, was similarly enhanced, with peak levels of 22.8 +/- 4.7 and 24.1 +/- 8.2 ng/mg, respectively. An increase in aqueous PGE2 levels was more pronounced in the argon as compared with the Nd:YAG group, with peak levels of 56.7 +/- 17.3 and 20.0 +/- 7.7 ng/ml, respectively. PGE2 production correlated well with aqueous PGE2 levels in the argon group, but not in the Nd:YAG group. Protein accumulated in the aqueous humor following irradiation by both types of laser light, with higher levels occurring in the argon group. These results are indicative of possible differences in the extent of the inflammatory reaction induced by the application of argon and Nd:YAG laser light to the iris.