Hyper-IgE syndrome (HIES) is a very rare primary immune deficiency characterized by elevated serum IgE levels, recurrent bacterial infections, chronic dermatitis, and connective tissue abnormalities. Autosomal dominant (AD) HIES involves a mutation in signal transducer and activator of transcription 3 (STAT3) that leads to an impaired T(H)17 response. STAT3 signaling is also involved in the function of RORγt⁺ type 3 innate lymphoid cells (ILC3s) and RORγt⁺T(H)17 cells. The aim of this study was to investigate the role of innate immune cells such as innate lymphoid cells (ILCs), granulocytes, and monocytes in a patient with HIES. Peripheral blood mononuclear cells (PBMCs) from a patient with HIES and three age-matched healthy controls were obtained for the analysis of the innate and adaptive immune cells. The frequencies of ILCs in PBMCs were lower in the patient with HIES than in the controls. Moreover, granulocyte-macrophage colony-stimulating factor (GM-CSF) and IL-17A produced by ILC3s in PBMCs were lower in the patient with HIES than the controls. Compared with the controls, classical monocytes (CD14⁺CD16(low)), which have a high antimicrobial capability, were also lower in the patient with HIES, while non-classical monocytes (CD14(low)CD16⁺) as well as intermediate monocytes (CD14⁺CD16(intermediate)) were higher. Taken together, these results indicate that the impaired immune defense against pathogenic microbes in the patient with HIES might be partially explained by functional defects in ILC3s and inflammatory monocytes.
Bacterial Infections ; Connective Tissue ; Cytokines ; Dermatitis ; Granulocyte-Macrophage Colony-Stimulating Factor ; Granulocytes ; Humans ; Immunity, Innate ; Immunoglobulin E ; Interleukin-17 ; Job Syndrome* ; Lymphocytes* ; Monocytes* ; STAT3 Transcription Factor

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe cutaneous adverse reaction involving various internal organs. Flare-ups after recovery from the initial presentation of DRESS are caused by relapse of drug-induced T-cell-mediated reactions. However, the specific underlying mechanism is unclear. Here, we report a case of a 60-year-old man with allopurinol-induced DRESS who suffered recurrent episodes of generalized rash with eosinophilia, which mimicked immune reconstitution inflammatory syndrome. Analysis of immunological profiles revealed that the percentages of T lymphocytes and regulatory T cells in the patient with DRESS were higher than those in healthy controls. In addition, there was a notable change in the subtype of monocytes in the patient with DRESS; the percentage of nonclassical monocytes increased, whereas that of classical monocytes decreased. Upon viral infection, nonclassical monocytes exhibited strong pro-inflammatory properties that skewed the immune response toward a Th2 profile, which was associated with persistent flare-ups of DRESS. Taken together, the results increase our understanding of the pathogenesis of DRESS as they suggest that expansion of nonclassical monocytes and Th2 cells drives disease pathogenesis.
Allopurinol ; Drug Hypersensitivity Syndrome ; Eosinophilia ; Exanthema ; Herpesviridae ; Humans ; Immune Reconstitution Inflammatory Syndrome ; Middle Aged ; Monocytes ; Recurrence ; T-Lymphocytes ; T-Lymphocytes, Regulatory ; Th2 Cells

Breastfeeding has numerous benefits both for infants and mothers. WHO, UNICEF, and OECD report the breastfeeding rate (BR) and exclusive breastfeeding rate (EBR) at 3, 4, and 6 months of age for the international comparison. This article investigates the nationwide changes in BR and EBR in Korea from 1994 to 2012. EBR declined from 1994 to 2000, however progressively increased untill 2012. The latest data in 2012 revealed EBRs at 3, 4, and 6 months were 50.0%, 40.5%, and 11.4% respectively. The exclusive formula feeding rate (EFR) was highest in 2000 and gradually declined thereafter. In 2012, the EFRs at 3, 4 and 6 months were 21.7%, 26.5%, and 10.1%. In 2009, the EBRs at 3 and 6 months in the United States were 36.0% and 16.3% compared to 50.0% and 11.4% in Korea. In England, the EBRs were 17% and 12% in 2010. Amongst OECD countries, Hungary ranked highest EBRwith 95%, and Iceland, Norway, Slovak Republic, Australia, New Zealand followed. In conclusion, BRs were lowest in 2000, and there have been remarkable increases in BRs over the past 10 yr in Korea. Although BRs have been increasing, further efforts to increase BRs should be made continuously.
Breast Feeding/*statistics & numerical data/*trends ; Data Collection ; Female ; Humans ; Infant ; Infant, Newborn ; Mothers ; Republic of Korea

PURPOSE: To compare high-order aberrations in the pseudophakia with different types of intraocular lenses (IOLs), as well as with normal phakic eyes. METHODS: This single-center prospective study comprised 80 cataract patients who underwent cataract surgery and 20 phakic eyes. Twenty eyes received an AF-1(R) VA-60BB; 20 eyes, a Corneal(R) ACR6D SE; 20 eyes, a Sensar(R) AR40e; and 20 eyes, a ClariFlex(R). One month after cataract surgery, the eye aberration was measured using a Hartmann-Shack type aberrometer. The same test was performed for 20 normal phakic eyes. The individual Zernike polynomials, the root mean square (RMS) values of the third and fourth order aberration, and the total RMS among the groups were compared. RESULTS: The individual Zernike coefficient (Z3(-1), Z3(3), Z4(-4), Z4(0), Z4(4)) showed a significant difference in the IOL-implanted groups compared with the normal phakic eyes. There were statistically significant differences in the RMS values of the third and fourth order aberration, and total among each group. CONCLUSIONS: There was a statistically significant increase in aberration in patients with high refractive index IOL and a statistically significant decrease in patients with silicone IOL, compared to the normal phakic eyes.
Cataract ; Humans ; Lenses, Intraocular* ; Prospective Studies ; Pseudophakia* ; Refractometry ; Silicones

BACKGROUND AND PURPOSE: Exercise is recommended for every patient with Parkinson's disease (PD). The effectiveness of two different forms of exercise for PD, Tai Chi and combined stretching-strengthening exercise, was compared. METHODS: Patients with mild-to-moderate PD were recruited to join either the combined stretching-strengthening exercise group (n=7), the Tai Chi group (n=9), or the control (nonintervention) group (n=7). Exercise was performed three times a week over a period of 8 weeks. The Tai Chi exercise was led by certified instructors based on a Tai-Chi-for-arthritis program. The combined stretching-strengthening exercise comprised folk dancing, stepping, and elastic-band exercises. The subjects' functional fitness, parkinsonian symptoms, quality of life (QoL), and depression were evaluated. RESULTS: Both exercise groups yielded better results in their overall functional fitness after the intervention. However, no improvement with exercise was found for parkinsonian symptoms, as evaluated using the Unified Parkinson's Disease Rating Scale. With respect to the domains of QoL, the combined stretching-strengthening exercise group fared better in the social domain of QoL, and the Tai Chi group fared better in the emotional domain, while QoL and depression worsened in the control group. The postintervention QoL was improved relative to the control condition only for the Tai Chi group. Although the exercise interventions did not have any effect on depression, the control group was associated with a significant deterioration. CONCLUSIONS: Exercise improved the functional fitness and QoL of PD patients, with Tai Chi yielding better results in QoL and favorable results in functional fitness. These findings suggest that Tai Chi could be a good exercise strategy for patients with PD.
Dancing ; Depression ; Exercise ; Humans ; Parkinson Disease* ; Quality of Life ; Tai Ji*

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare but potentially fatal drug-induced systemic hypersensitivity response characterized by erythematous eruption, fever, leukocytosis with eosinophilia, and internal organ involvement. Antitubercular agents are potential causative agents for DRESS syndrome but difficult to verify as a culprit drug, since antitubercular agents are coadministered as a combination regimen. A 42-year-old female with endobronchial tuberculosis was diagnosed with DRESS syndrome after 4-week treatment of isoniazid, rifampicin, ethambutol, and pyrazinamide with prednisolone 50 mg. All the antitubercular agents were stopped and replaced with levofloxacin, cycloserine, p-aminosalicylic acid, and kanamycin. However, severe exacerbation of DRESS syndrome compelled the patient to discontinue the administration of the second-line antitubercular agents. Two months later, the patient underwent a patch test for all the antitubercular agents which had been used, and the results showed positivity to isoniazid and cycloserine. We report a rare case of DRESS syndrome that reacted to cycloserine as well as isoniazid. Development of coreactivity to other drugs should be differentiated with a flare-up reaction in the management of DRESS syndrome.
Adult ; Aminosalicylic Acid ; Antitubercular Agents ; Cycloserine ; Drug Hypersensitivity Syndrome ; Eosinophilia* ; Ethambutol ; Female ; Fever ; Humans ; Hypersensitivity ; Isoniazid ; Kanamycin ; Leukocytosis ; Levofloxacin ; Patch Tests ; Prednisolone ; Pyrazinamide ; Rifampin ; Tuberculosis

PURPOSE: To compare the results of laser epithelial keratomileusis (LASEK) and epi-laser in situ keratomileusis (Epi-LASIK) for the treatment of moderate to high myopia. METHODS: In this study, 50 patients with a manifest refraction of moderate to high myopia were assigned to two groups: 50 eyes of 25 patients treated with LASEK and 50 eyes of 25 patients treated with epi-LASIK. Uncorrected visual acuity (UCVA), manifest refraction, corneal haze, and postoperative pain were reviewed in LASEK and epi-LASIK treated eyes at 3 months after the operation. RESULTS: At one week after surgery, UCVA was recovered more rapidly after epi-LASIK than LASEK (LASEK: 0.61+/-0.23, Epi-LASIK: 0.79+/-0.11, p<0.05), but UCVA at both 1 and 3 months showed no significant difference between the LASEK and epi-LASIK groups. However, epi-LASIK-treated eyes had lower postoperative pain scores and lower corneal haze scores (1 month; p=0.01) than those of LASEK-treated eyes. CONCLUSIONS: Epi-LASIK showed a more rapid visual recovery than LASEK at 1 week after surgery for the correction of myopia. Epi-LASIK-treated eyes also had less postoperative pain and an earlier reduction of postoperative corneal haze. These results suggest epi-LASIK could be a viable alternative procedure for the correction of moderate to high myopia.
Humans ; Keratectomy, Subepithelial, Laser-Assisted ; Myopia* ; Pain, Postoperative ; Visual Acuity

PURPOSE: To investigate the risk factors for dry eye in thyroid-associated ophthalmopathy patients. METHODS: The present retrospective study was conducted on 78 eyes in 39 patients diagnosed with thyroid-associated ophthalmopathy. The risk factors for dry eye in thyroid-associated ophthalmopathy patients were examined by analyzing data including tear break-up time, Schirmer's test, age, gender, thyroid function status, palpebral fissure width, lagophthalmos, eyelid retraction, and exophthalmos through multiple logistic regression analysis. RESULTS: The risk factors for dry eye disease were palpebral fissure width (OR = 3.422, p = 0.022) and lagophthalmos (OR = 13.068, p = 0.049). Age, gender, thyroid hormone status, eyelid retraction and exophthalmos did not show significant association with dry eye in multiple logistic regression analysis. The only risk factor for tear film break-up time less than 10 seconds was palpebral fissure width (OR = 2.787, p = 0.045), and none of the risk factors for Schirmer's test less than 10 mm at 5 minutes were statistically significant. CONCLUSIONS: Risk factors for dry eye in thyroid ophthalmopathy patients were palpebral fissure width and lagophthalmos.
Dry Eye Syndromes ; Exophthalmos ; Eye ; Eye Diseases ; Eyelids ; Graves Ophthalmopathy ; Humans ; Logistic Models ; Retrospective Studies ; Risk Factors ; Tears ; Thyroid Gland

PURPOSE: This study is aimed to evaluate the effectiveness and tolerability of rufinamide as add-on therapy in patients with intractable epilepsies. METHODS: We retrospectively reviewed the medical records of 70 patients treated with rufinamide in Asan Medical Center, children's hospital. Two cases with incomplete medical records were excluded and total sixty-eight cases were enrolled. Rufinamide was added on the existing antiepileptic drugs and the total seizure frequency at pre-medication, 3 months and 12 months were examined. RESULTS: The mean age of 68 patients (43 male) was 10.5 yrs (range, 1-24 yrs). At 3 months after rufinamide initiation, 5 patients achieved freedom from seizures and 28 (41.2%) achieved a ≥50% seizure reduction. At 12 months, 7 patients achieved seizure freedom and 29 (42.6%) achieved ≥50% seizure reduction. The retention rate was hold up to 75.0% at 3 months and 66.2% at 12 months of study. Total 29 patients reported adverse events in order of seizure aggravation, somnolence, insomnia, common cold, nausea and vomiting. CONCLUSION: In this study, rufinamide is effective and tolerable in patients with other intractable epilepsy of childhood onset as well as the patients with LGS. Further research is required to define the efficacy of rufinamide in intractable epilepsy other than LGS.
Anticonvulsants ; Chungcheongnam-do ; Common Cold ; Drug Resistant Epilepsy ; Encephalitis, Viral* ; Freedom ; Humans ; Medical Records ; Nausea ; Retrospective Studies ; Seizures ; Sleep Initiation and Maintenance Disorders ; Vomiting

Hypoxic insult increases the level of extracellular glutamate, which leads to the influx of toxic Ca++ through the activation of N-methyl-D-aspartate(NMDA) receptors. The neuroprotective action of NMDA antagonist against hypoxic insult has been demonstrated in vitro. It has been demonstrated that the concentration of 50hydrox-ytryptamine(5-HT) also increased after ischemia in rat hippocampus. However, there is paucity of studies concerning the funtional relationships between the spontaneous release of 5-HT and NMDA receptor activity during hypoxia in vitro. Therefore, the present study was aimed to investigate whether hypoxia and/or NMDA was able to stimulate the release of 5-HT from the hypoxia-sensitive rat hippocampl slices.The hippocampus was obtained from the rat brain and sliced 400um thickness with manual chorpper. After 30 min's preincubation in the normal buffer, the slices were incubated for 20 min in a buffer containing 3H-5HT(0.1uM, 74uCi) for uptake, and washed. To measure the release of 3H-5-HT into the buffer, the incubation medium was drained off and refilled every ten minutes through a sequence of 14 tubes. Administration of NMDA or induction of hypoxia (gassing it with 95% N2/5% CO2) was done in the 6th and 7th tube, and APV was added 10 minutes prior to these manipulations. The radioactivities in each buffer and the tissue were counted using liquid scintillation counter and the results were expressed as a percentage of the total radioactivity. When slices were exposed to hypoxia for 20min, 3H-5-HT release was markedly decreased and a rebound release of 3H-5-HT was observed on the post-hypoxic period. NMDA(1mM) incereased 3H-5-HT release in the control group. NMDA also incereased rebound release of 3H-5-Htrelease. When 2-amino-5-hposphonovaleric acid (APV, 30uM or 60 uM) were added to the incubation media, NMDA-induced increase of 3H-5-HT release were blocked does-dependently. The rebound release of 3H-5-HT during post-hypoxic period was also blocked by APV. These results suggest that the spontaneous release of 3H-5HT decreases during hypoxic period, but 20min hypoxic exposure causes rebound increase of 3H-5-HT release during post-hypoxic period which is mediated by the increased activity of the NMDA receptor.
Animals ; Anoxia* ; Brain ; Glutamic Acid ; Hippocampus ; Ischemia ; N-Methylaspartate* ; Radioactivity ; Rats* ; Scintillation Counting ; Serotonin*