Comparison of visual field progression between temporally tilted disc and nontilted disc, in patients with normal tension glaucoma.

PURPOSE: To investigate the long-term visual field (VF) progression of temporally tilted disc and nontilted disc in normal tension glaucoma (NTG). METHODS: Retrospective, observational case series. Forty-seven patients with temporally tilted disc (47 eyes), 44 patients with nontilted disc in NTG (44 eyes) patients, who were examined by at least 5 VF tests, and were followed-up over a 5-year period, at the Department of Ophthalmology of the Samsung Medical Center, from May 1998 to 2013. VF progression was defined by modified Anderson-Hodapp criteria, and Glaucoma Progression Analysis (GPA). Multivariate analysis was used to identify the risk factors for VF progression in the temporally tilted disc. RESULTS: According to the Anderson-Hodapp criteria, progression rates of the temporally tilted disc and nontilted disc at 60 months were 19% and 72%, respectively (P<0.0001). According to GPA, they were 25% and 53%, respectively (P<0.0001). Twenty of 47 patients in the temporally tilted disc did not show progression. Among them, the more tilted disc showed the more VF defects. The hazard ratio of retinal nerve fiber layer (RNFL) defect type was 3.08 (95% CI, 1.17-8.14; P=0.02). The simultaneous superior and inferior RNFL defect type was the most common in progressors in the temporally tilted disc (P=0.04). CONCLUSION: Through long-term follow-up, the cumulative survival rate of temporally tilted disc was higher than that of nontilted disc. Caution is required in the treatment of the temporally tilted disc. New treatment policy for the temporally tilted disc may follow.

Clinical characterization and molecular classification of 12 Korean patients with pseudohypoparathyroidism and pseudopseudohypoparathyroidism.

CONTEXT: Pseudohypoparathyroidism (PHP) is defined as resistance toward parathyroid hormones. PHP and pseudopseudohypoparathyroidism (PPHP) are rare disorders resulting from genetic and epigenetic aberrations within or upstream of the GNAS locus. This study investigated the clinical characteristics and performed a molecular analysis of PHP and PPHP. METHODS: A total of 12 patients with (P)PHP from 11 unrelated families (4 with PHP-Ia, 6 with PHP-Ib, and 2 with PPHP) were characterized using both clinical and molecular methods. Clinical features included the presenting symptoms, Albright hereditary osteodystrophy features, and resistance to hormones. Comprehensive analysis of the GNAS and STX16 loci was undertaken to investigate the molecular defects underlying (P)PHP. RESULTS: All PHP-Ib patients displayed hypocalcemic symptoms. All PHP-Ia patients showed resistance toward TSH, in addition to PTH. In most patients with PHP, when the diagnosis of PHP was first established, hypocalcemia and hyperphosphatemia were associated with a significant increase in serum PTH levels. One patient with PHP-Ia was diagnosed with growth hormone deficiency and showed a good response to human recombinant growth hormone therapy. 6 patients with PHP-Ia and PPHP showed 5 different mutations in the GNAS gene. 5 patients with PHP-Ib displayed a loss of differentially methylated region (DMR) imprints of the maternal GNAS. One PHP-Ib patient showed a de novo microdeletion in STX16 and a loss of methylation of exon A/B on the maternal allele. No patients revealed paternal disomy among 4 patients with PHP-Ib. CONCLUSIONS: Identification of the molecular causes of PHP and PPHP explains their distinctive clinical features and enables confirmation of the diagnosis and exact genetic counseling.

Long-term use of preservatives on rat nasal respiratory mucosa: effects of benzalkonium chloride and potassium sorbate.

OBJECTIVES: The preservatives benzalkonium chloride (BZC) and potassium sorbate (PS) are widely used, not only for nasal drops, but also for eyedrops and cosmetics. However, there have been many case reports that consider lesions such as dermatitis or conjunctivitis to be the results of irritation induced by BZC or PS. METHODS: We evaluated the histological changes after the long-term administration of BZC or PS on rat nasal respiratory mucosa. Forty rats were used for the BZC group and 40 rats for PS group. Animals in each group were divided into four subgroups The first subgroup received a low-concentration preservative solution that was commonly used for nasal sprays. The second subgroup received a high-concentration preservative solution that was reported to induce dermatitis in humans. The third and fourth subgroups received a steroid mixed preservative solution of low and high concentrations, respectively. The control group was administrated normal saline. After each group received 1, 2, and 4 weeks of topical administration, the symptomatic and histological changes on H&E stain were observed. RESULTS: Sneezing and nasal rubbing with forelegs were observed in almost all subgroups by the seventh day of treatment. The preservatives induced nasal lesions, including intraepithelial glandular formation, inflammatory cell infiltration, vascular hyperplasia, and edematous change. The symptomatic and histological changes were pronounced with the prolonged duration of administration. Similar results were observed in the steroid mixed-solution groups. In the PS steroid mixed-solution group, however, symptoms and nasal lesions were reduced with the prolonged duration of administration. CONCLUSION: It is our finding that even a low-concentration solution of preservative can lead to nasal lesion. Hence there is a strong need to develop both a preservative that can be safely and widely used and a nasal spray without preservatives.

Hypothalamic-pituitary-adrenal (HPA) function in adrenocortical steroid treated minimal change nephrotic syndrome.

Seventeen children aged 1.5 to 15.6 years with minimal change nephrotic syndrome (MCNS) had hypothalamic-pituitary-adrenal (HPA) tests performed. The study consisted of baseline cortisol levels (8 a.m. and 4 p.m.), 6-hour ACTH stimulation tests and metyrapone tests. Ten patients were frequent relapsers (4 or more relapses per year) and 7 patients were infrequent relapsers (less than 4 relapses per year). All relapses were treated with daily prednisone 60 mg/m2 until the urine was protein-free for 3 days followed by prednisone 40 mg/m2 every other day for 28 days. All were tested no sooner than 1 week after alternate-day prednisone therapy was discontinued. The morning and afternoon cortisol levels of all frequent relapsers were abnormally low. The average a.m. and p.m. cortisol levels in the frequent relapsers were 2.6 +/- 0.08 and 1.4 +/- 0.4 microgram/dl respectively (normal a.m. value 10-20 microgram/dl, p.m. 50% a.m. value). In the frequent relapsers 9 out of 10 had a normal 6-hour ACTH test and 8 out of 9 had a normal response to metyrapone. In all infrequent relapsers the morning and afternoon cortisols were normal (mean a.m. 12.9 +/- 1.4 and p.m. 6.8 +/- 0.8 microgram/dl). Also, all infrequent relapsers had normal responses to ACTH and metyrapone. It is concluded that low baseline plasma cortisol levels are predictably present in frequent relapsers treated with prednisone.