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.

We presented a patient with cerebral decompression sickness, who showed predominant vasogenic edema on a 3.0 Tesla (3T) magnetic resonance imaging (MRI) findings, including diffusion-weighted image (DWI) and apparent diffusion coefficient (ADC) mapping. Within minutes of surfacing, he developed paresis of the right lower limb. During transport, he began shivering, followed by severe spasm that eventually progressed to a tonic-clonic seizure. Emergent hyperbaric oxygen therapy (HBOT) was performed with U.S. Navy treatment table 6A after a treatment of seizure activity. Brain MRI was performed after hyperbaric oxygen therapy to detect any cerebral lesions, which showed subcortical hyperintensity signal changes in the left fronto-parietal region on the ADC map. Overlying cortical hyperintensity on DWI sequences and cortical hypointensity on the ADC map were simultaneously observed. Moreover, these findings disappeared in a followup MRI with complete resolution of symptoms. These findings indicate that vasogenic edema can cause cerebral decompression sickness (DCS) and that 3T MRI with DWI and ADC mapping may be useful for diagnosing cerebral DCS. In addition, these findings suggest that DW-MRI may also be useful in predicting the prognosis of cerebral DCS.
Brain ; Brain Edema ; Decompression Sickness* ; Decompression* ; Diffusion ; Diffusion Magnetic Resonance Imaging ; Edema ; Follow-Up Studies ; Humans ; Hyperbaric Oxygenation ; Lower Extremity ; Magnetic Resonance Imaging* ; Paresis ; Prognosis ; Seizures ; Shivering ; Spasm

This retrospective observational case series on eyes from three patients was done to elucidate the developmental mechanism of spontaneous reattachment of rhegmatogenous retinal detachment (SRRRD). The study eyes of each patients showed evidence of retinal break and diffuse retinal pigmentary change. Ultrasound biomicroscopic examination revealed vitreous fibers attached to the area around the retinal break. Posterior vitreous attachment was confirmed in each eye. A thin fibrovascular membrane incompletely sealing the retinal break was noted in one case. We suggest that the vitreous attachment around the retinal break and the sealing of the break with adjacent vitreous fibers seem to be involved in the developmental mechanism of SRRRD.
Adult ; Atrophy ; Disease Progression ; Female ; Humans ; Male ; Middle Aged ; Remission, Spontaneous ; Retina/*abnormalities/pathology/*physiopathology ; Retinal Detachment/*etiology/pathology/*physiopathology ; Retinal Pigment Epithelium/abnormalities/pathology/physiopathology ; Retrospective Studies ; Vitreous Body/abnormalities/pathology/physiopathology ; Young Adult

PURPOSE: Return of spontaneous circulation (ROSC) is closely related to a favorable treatment outcome in out-of-hospital cardiac arrest and is also a reliable treatment target in clinical trials. The aim of this study is the identification of major factors affecting field ROSC by analyzing the updated data encompassing the pre-hospital treatment processes. METHODS: This is a retrospective review of cardiopulmonary resuscitation (CPR) cases performed by 119 rescuers before hospital arrival from January 2012 to December 2014 in one province. Cases with traumatic cardiac arrest, unnecessary CPR, arrest occurred during transport, under age 14 years, and incomplete medical records were excluded. RESULTS: Of 1,832 patients enrolled in the study, ROSC was achieved in 99 cases (5.4%). Among them, 50 (50.5%) had ROSC at the event field and others achieved ROSC during the transport process, respectively. Total cases were divided into two groups based on ROSC before arrival at the hospital. Age, underlying cardiovascular illnesses, the event place, presence of a witness, bystander CPR, application of an automated external defibrillator (AED), the initial shockable rhythm, defibrillation, advanced airway, hydration, the arrival time of rescuer CPR, application time of AED, and defibrillation showed significant statistical differences. In multivariate analysis, age, defibrilOf 1,832 patients enrolled in the study, ROSC was achieved in 99 cases (5.4%). Among them, 50 (50.5%) had ROSC at the event field and others achieved ROSC during the transport process, respectively. Total cases were divided into two groups based on ROSC before arrival at the hospital. Age, underlying cardiovascular illnesses, the event place, presence of a witness, bystander CPR, application of an automated external defibrillator (AED), the initial shockable rhythm, defibrillation, advanced airway, hydration, the arrival time of rescuer CPR, application time of AED, and defibrillation showed significant statistical differences. In multivariate analysis, age, defibrillation, hydration, bystander CPR, and the call-to-rescuer CPR interval appeared to be correlated with field ROSC. CONCLUSION: To improve the survival rate through field ROSC, a public campaign to improve bystander CPR, prompt recognition of cardiac arrest, and rapid application of pre-hospital treatment and political support of the public institution are mandatory.
Cardiopulmonary Resuscitation ; Defibrillators ; Emergency Medical Services ; Heart Arrest ; Humans ; Medical Records ; Multivariate Analysis ; Out-of-Hospital Cardiac Arrest* ; Retrospective Studies ; Survival Rate ; Treatment Outcome

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

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