A pair of 19-year-old female identical twins was referred to our hospital with progressive visual loss. They exhibited bilateral chorioretinal atrophy involving the midperiphery on fundoscopy and fluorescein angiography. Bilateral visual field constriction was noted on dynamic Goldmann perimetry, and a markedly impaired response was observed on both photopic and scotopic electroretinograms. Cystoid macular edema was identified in both eyes on optical coherence tomography. Plasma levels of ornithine were elevated. Based on these observations, the patients were diagnosed with gyrate atrophy of the choroid and retina. The clinical diagnosis was confirmed by mutation analysis of the ornithine-delta-aminotransferase (OAT) gene. Patients were treated with a pyridoxine supplement (300 mg/day) and an arginine-restricted diet to lower plasma levels of ornithine, which were successfully reduced without progression of chorioretinal atrophy for 15 months. Our report describes the first case of gyrate atrophy in the Korean population diagnosed by OAT gene analysis and treated with vitamin B6 dietary supplementation.
DNA/*analysis ; DNA Mutational Analysis ; Diagnosis, Differential ; Electroretinography ; Female ; Fluorescein Angiography ; Fundus Oculi ; Gyrate Atrophy/*diagnosis/enzymology/genetics ; Humans ; *Mutation ; Ornithine-Oxo-Acid Transaminase/*genetics/metabolism ; Tomography, Optical Coherence ; Visual Acuity ; Young Adult

Background: There is increasing evidence that probiotics are effective in treating allergic rhinitis (AR), while some controversies remain. This study was performed to evaluate the therapeutic effect and safety of a mixture of Bifidobacterium longum and Lactobacillus plantarum (NVP-1703) in children with AR. Methods: In a randomized, double-blind, placebo-controlled study, children aged 6 to 19 years with perennial AR were treated with NVP-1703 at a dose of 1 × 1010 CFU/day or placebo once a day for 4 weeks. Total nasal symptom score (TNSS), nasal symptom duration score (NSDS), quality of life (QoL), allergic inflammatory markers, and safety parameters were evaluated. Results: After 4 weeks of treatment, the TNSS in the NVP-1703 group significantly decreased compared to that in the placebo group (P = 0.011), both in the morning and the evening (P = 0.031 and P = 0.004, respectively). The NSDS also significantly decreased in the NVP-1703 group compared to that in the placebo group (P = 0.018). QoL scores, particularly those related to mouth breathing and itchy nose, showed a significant improvement in the NVP-1703 group compared to the placebo group. The ratios of interleukin (IL)-4/IL-22 and IL-5/IL-22 were significantly reduced in the NVP-1703 group after the treatment compared to the baseline values. No notable adverse events were reported in the NVP-1703 group. Conclusion Oral administration of a mixture of B. longum and L. plantarum (NVP-1703) improved both AR symptoms and QoL in children with perennial AR, accompanied by decreases in the ratios of T helper 2 cytokines to IL-22.

Background: There is increasing evidence that probiotics are effective in treating allergic rhinitis (AR), while some controversies remain. This study was performed to evaluate the therapeutic effect and safety of a mixture of Bifidobacterium longum and Lactobacillus plantarum (NVP-1703) in children with AR. Methods: In a randomized, double-blind, placebo-controlled study, children aged 6 to 19 years with perennial AR were treated with NVP-1703 at a dose of 1 × 1010 CFU/day or placebo once a day for 4 weeks. Total nasal symptom score (TNSS), nasal symptom duration score (NSDS), quality of life (QoL), allergic inflammatory markers, and safety parameters were evaluated. Results: After 4 weeks of treatment, the TNSS in the NVP-1703 group significantly decreased compared to that in the placebo group (P = 0.011), both in the morning and the evening (P = 0.031 and P = 0.004, respectively). The NSDS also significantly decreased in the NVP-1703 group compared to that in the placebo group (P = 0.018). QoL scores, particularly those related to mouth breathing and itchy nose, showed a significant improvement in the NVP-1703 group compared to the placebo group. The ratios of interleukin (IL)-4/IL-22 and IL-5/IL-22 were significantly reduced in the NVP-1703 group after the treatment compared to the baseline values. No notable adverse events were reported in the NVP-1703 group. Conclusion Oral administration of a mixture of B. longum and L. plantarum (NVP-1703) improved both AR symptoms and QoL in children with perennial AR, accompanied by decreases in the ratios of T helper 2 cytokines to IL-22.

Background: There is increasing evidence that probiotics are effective in treating allergic rhinitis (AR), while some controversies remain. This study was performed to evaluate the therapeutic effect and safety of a mixture of Bifidobacterium longum and Lactobacillus plantarum (NVP-1703) in children with AR. Methods: In a randomized, double-blind, placebo-controlled study, children aged 6 to 19 years with perennial AR were treated with NVP-1703 at a dose of 1 × 1010 CFU/day or placebo once a day for 4 weeks. Total nasal symptom score (TNSS), nasal symptom duration score (NSDS), quality of life (QoL), allergic inflammatory markers, and safety parameters were evaluated. Results: After 4 weeks of treatment, the TNSS in the NVP-1703 group significantly decreased compared to that in the placebo group (P = 0.011), both in the morning and the evening (P = 0.031 and P = 0.004, respectively). The NSDS also significantly decreased in the NVP-1703 group compared to that in the placebo group (P = 0.018). QoL scores, particularly those related to mouth breathing and itchy nose, showed a significant improvement in the NVP-1703 group compared to the placebo group. The ratios of interleukin (IL)-4/IL-22 and IL-5/IL-22 were significantly reduced in the NVP-1703 group after the treatment compared to the baseline values. No notable adverse events were reported in the NVP-1703 group. Conclusion Oral administration of a mixture of B. longum and L. plantarum (NVP-1703) improved both AR symptoms and QoL in children with perennial AR, accompanied by decreases in the ratios of T helper 2 cytokines to IL-22.

Background: There is increasing evidence that probiotics are effective in treating allergic rhinitis (AR), while some controversies remain. This study was performed to evaluate the therapeutic effect and safety of a mixture of Bifidobacterium longum and Lactobacillus plantarum (NVP-1703) in children with AR. Methods: In a randomized, double-blind, placebo-controlled study, children aged 6 to 19 years with perennial AR were treated with NVP-1703 at a dose of 1 × 1010 CFU/day or placebo once a day for 4 weeks. Total nasal symptom score (TNSS), nasal symptom duration score (NSDS), quality of life (QoL), allergic inflammatory markers, and safety parameters were evaluated. Results: After 4 weeks of treatment, the TNSS in the NVP-1703 group significantly decreased compared to that in the placebo group (P = 0.011), both in the morning and the evening (P = 0.031 and P = 0.004, respectively). The NSDS also significantly decreased in the NVP-1703 group compared to that in the placebo group (P = 0.018). QoL scores, particularly those related to mouth breathing and itchy nose, showed a significant improvement in the NVP-1703 group compared to the placebo group. The ratios of interleukin (IL)-4/IL-22 and IL-5/IL-22 were significantly reduced in the NVP-1703 group after the treatment compared to the baseline values. No notable adverse events were reported in the NVP-1703 group. Conclusion Oral administration of a mixture of B. longum and L. plantarum (NVP-1703) improved both AR symptoms and QoL in children with perennial AR, accompanied by decreases in the ratios of T helper 2 cytokines to IL-22.

Kounis syndrome refers to an acute coronary syndrome induced by an allergic reaction. Kounis syndrome is not a rare disease but is considered to be underdiagnosed. This syndrome is a potentially life threatening medical emergency. We described a case of Kounis syndrome caused by injection of intravenous gadolinium-based contrast agent. We also discussed emergency drugs used for treatment of Kounis syndrome.
Acute Coronary Syndrome* ; Anaphylaxis* ; Contrast Media ; Emergencies ; Hypersensitivity ; Rare Diseases

PURPOSE: To evaluate the efficacy of vitrectomy combined with intravitreal injection of triamcinolone acetonide (IVTA) and macular laser photocoagulation for the treatment of nontractional diabetic macular edema (DME) refractory to anti-vascular endothelial growth factor (VEGF) therapy. METHODS: Twenty-eight eyes from 28 subjects who were diagnosed with nontractional DME refractory to three or more sequential anti-VEGF injections underwent sequential vitrectomy, IVTA, and macular laser photocoagulation. Changes in best-corrected visual acuity (BCVA) and central subfield thickness (CST) during the six months following vitrectomy were evaluated. Additionally, the CST and BCVA outcomes were compared with those of 26 eyes treated with the same triple therapy for nontractional DME refractory to conventional treatment, such as IVTA or macular laser photocoagulation, or both. RESULTS: The mean logarithm of the minimum angle of resolution BCVAs before and one, three, and six months after vitrectomy were 0.44 +/- 0.15, 0.36 +/- 0.18, 0.31 +/- 0.14, and 0.34 +/- 0.22, respectively. The mean CSTs were 433.3 +/- 77.9, 329.9 +/- 59.4, 307.2 +/- 60.2, and 310.1 +/- 80.1 microns, respectively. The values of both BCVA and CST at one, three, and six months were significantly improved from baseline (p < 0.05). The extent of CST reduction during the first month after triple therapy was greater in eyes refractory to conventional treatment than in eyes refractory to anti-VEGF (p = 0.012). CONCLUSIONS: Vitrectomy combined with IVTA and macular laser photocoagulation had a beneficial effect on both anatomical and functional outcomes in eyes with nontractional DME refractory to anti-VEGF therapy.
Aged ; Combined Modality Therapy ; Diabetic Retinopathy/*drug therapy/*surgery ; Female ; Humans ; Immunosuppressive Agents/administration & dosage ; Intravitreal Injections ; Light Coagulation/*methods ; Macula Lutea/drug effects/surgery ; Macular Edema/*drug therapy/*surgery ; Male ; Middle Aged ; Retrospective Studies ; Triamcinolone Acetonide/*administration & dosage ; Vitrectomy/*methods

BACKGROUND: Isoflurane and propofol are known to have a low potential for hepatotoxicity. However, no study has compared the effects on hepatic function after of enflurane, isoflurane or propofol in tympanomatoidectomy. Anesthetic techniques should permit the maintenance of low blood pressure during operation for a bloodless surgical field. Because of a long operation time and low blood pressure, the liver may be damaged. The purpose of this study was to evaluate the effects of isoflurane and propofol on liver function by comparing them with enflurane in patients who had been received tympanomatoidectomy. METHODS: Eighty-seven patients with normal liver function were studied following tympanomastiodectomy. The patients were randomly assigned to Group E (n = 28), who received enflurane, Group I (n = 30), who received isoflurane or Group P (n = 29), who received propofol. The plasma concentrations of aspartate aminotransferase (AST), alanine aminotransferase (ALT) and alkaline phosphatase (ALP) were measured on the day before the operation, and 1, 2 and 3 days after the operation. RESULTS: one and two days after the operation, all groups showed a significant increase in AST (P < 0.05), but this recovered to the baseline level at 3 days postoperatively, and there was no difference among the three groups. ALT and ALP were not changed until 3 days postoperatively in all groups, and there were no difference between the three groups. CONCLUSIONS: We conclude that there are no significant difference in terms of postoperative hepatic function after tympanomatoidectomy between the three groups.
Alanine Transaminase ; Alkaline Phosphatase ; Aspartate Aminotransferases ; Enflurane* ; Humans ; Hypotension ; Isoflurane* ; Liver ; Plasma ; Propofol*

To report the surgical outcome of full-thickness sclerotomy in five cases of uveal effusion syndrome (UES). Full-thickness sclerotomy without sclerectomy was performed on five eyes of four patients with UES with or without nanophthalmos. In four of the eyes, exudative retinal detachment associated with UES resolved after the sclerotomy. The subretinal fluid in one eye, which had a normal axial length, was relieved after undergoing three sclerotomy procedures. Full-thickness sclerotomy without vortex vein decompression or sclerectomy is an effective surgical option for the management of significant UES.
Adult ; Aged ; Drainage/methods ; Exudates and Transudates/*metabolism ; Humans ; Male ; Middle Aged ; Ophthalmologic Surgical Procedures/*methods ; Retinal Detachment/metabolism/surgery ; Sclera/*surgery ; Uveal Diseases/*metabolism/*surgery

Immersion pulmonary edema (IPE) is a form of pulmonary edema that can occur in divers or swimmers. IPE is a rare condition, but can be life-threatening and recurrent condition. The pathophysiology of IPE is still incompletely understood. We present a case of scuba diving induced pulmonary edema in a 46-year-old woman diving in cold seawater. After 20 minutes of diving at 18 m, she felt difficulty of breathing. Symptoms on surfacing were dyspnea, cough, and frothy sputum. Chest X-ray showed pulmonary edema and she was treated with an intravenous diuretic and supplemental oxygen. The patient showed rapid resolution of symptoms and was discharged on the same day. Sport physician should be aware of this potentially life-threatening recurrent condition.
Cough ; Diving* ; Dyspnea ; Female ; Humans ; Immersion* ; Middle Aged ; Oxygen ; Pulmonary Edema* ; Respiration ; Seawater ; Sports ; Sputum ; Thorax