No abstract available.
Anemia, Hemolytic, Autoimmune* ; Child* ; Hepatitis, Chronic* ; Humans

Nowadays, tumor necrosis factor-alpha (TNF-alpha) blockers are used for treatment of rheumatoid arthritis, inflammatory bowel diseases, ankylosing spondylitis, psoriatic arthritis, and psoriasis. Paradoxically, there are some reports on the appearance of psoriasis after administration of TNF-alpha blockers. Here, we report on a patient with monoarthritis in a knee joint who experienced psoriasis after TNF-alpha blocker therapy (adalimumab and etanercept). Oral medication was not a treatment option due to patient intolerance; thus, we tried ustekinumab, an anti-interleukin (IL)-12/23 monoclonal antibody used for treatment of psoriasis. Following ustekinumab injection, psoriatic skin lesions and joint symptoms were much improved. However, in the following period, joint pain and swelling became aggravated and synovial fluid cytokine levels including IL-6 and IL-17 were elevated. The treatment was changed to tocilizumab, a humanized monoclonal antibody against IL-6 receptor. After injection, knee joint swelling rapidly subsided without worsening of psoriatic skin lesions.
Arthralgia ; Arthritis, Psoriatic ; Arthritis, Rheumatoid ; Humans ; Inflammatory Bowel Diseases ; Interleukin-17 ; Interleukin-6 ; Joints ; Knee Joint ; Psoriasis* ; Receptors, Interleukin-6 ; Skin ; Spondylitis, Ankylosing ; Synovial Fluid ; Tumor Necrosis Factor-alpha* ; Ustekinumab

Dendritic cells (DCs) play a key role in activating the immune response against invading pathogens as well as dying cells or tumors. Although the immune response can be initiated by the phagocytic activity by DCs, the molecular mechanism involved in this process has not been fully investigated. Trp-Lys-Tyr-Met-Val-Met-NH2 (WKYMVM) stimulates the activation of phospholipase D (PLD) via Ca2+ increase and protein kinase C activation in mouse DC cell line, DC2.4. WKYMVM stimulates the phagocytic activity, which is inhibited in the presence of N-butanol but not t-butanol in DC2.4 cells. Furthermore, the addition of phosphatidic acid, an enzymatic product of PLD activity, enhanced the phagocytic activity in DC2.4 cells. Since at least two of formyl peptide receptor (FPR) family (FPR1 and FPR2) are expressed in DC2.4 as well as in mouse bone marrow-derived dendritic cells, this study suggests that the activation of FPR family by WKYMVM stimulates the PLD activity resulting in phagocytic activity in DC2.4 cells.
1-Butanol/pharmacology ; Animals ; Bone Marrow Cells/cytology/metabolism ; Calcium Signaling/*drug effects ; Cell Death/immunology ; Cell Line ; Communicable Diseases/immunology ; Dendritic Cells/immunology/*metabolism ; Mice ; Neoplasms/immunology ; Oligopeptides/*pharmacology ; Phagocytosis/*drug effects ; Phosphatidic Acids/pharmacology ; Phospholipase D/*metabolism ; Receptors, Formyl Peptide/*metabolism ; Research Support, Non-U.S. Gov't ; tert-Butyl Alcohol/pharmacology

PURPOSE: Immunotherapy is accepted as the only treatment of allergic disease that can modify the natural course of the disease and ameliorate symptoms. This study aimed to evaluate the safety and efficacy of ultra-rush therapy using Dermatophagoides extracts in children. METHODS: Of children older than four years who had visited Bundang CHA Pediatric Allergy Clinic, those showing positive reactions only to Dermatophagoides in the skin prick test and to the nasal provocation test were included. In all, 11 and 12 patients respectively preferred conventional and ultra-rush immunotherapy. We elevated allergen concentrations diluted to 1,000:1 of the end strength by 2-3 times with 30-minute intervals and checked oxygen saturation, pulse rate, blood pressure, and systemic reactions every 15 minutes. Immunotherapy effectiveness was valued by changes in nasal provocation test scores before and after immunotherapy. RESULTS: The average ages of patients in the conventional and ultra-rush immunotherapy groups were 8.3+/-2.3 and 9.2+/-2.8 years, respectively. Systemic reactions were observed in six in the ultra-rush group (50%) without anaphylaxis and one (9%) in the conventional group. The average scores in the nasal provocation test before and after treatment in the conventional group were 8.2+/-1.5 and 4.6+/-2.1, respectively (P=0.043). In the ultra-rush immunotherapy group, the scores changed from 6.2+/-2.2 to 3.7+/2.5 (P=0.017). CONCLUSION: Ultra-rush immunotherapy using Dermatophagoides in children is effective for treating allergic disease but can induce systemic effects rather than conventional immunotherapy.
Anaphylaxis ; Blood Pressure ; Child ; Dermatophagoides pteronyssinus ; Heart Rate ; Humans ; Hypersensitivity ; Immunotherapy ; Nasal Provocation Tests ; Oxygen ; Pyroglyphidae ; Skin

PURPOSE: There have been few reports regarding same-day discharge following uncomplicated procedures such as cerebral angiography and neurointervention. We present same-day experience with cerebral angiography and neurointervention during the past three years. MATERIALS AND METHODS: Four hundred and fifty-three patients underwent cerebral angiography or neurointervention at Asan Medical Center between January 2009 and December 2011. Of these patients, 249 (55%) underwent diagnostic catheter cerebral angiography and 204 patients (45%) underwent neurointerventional procedures as same-day procedures. We analyzed any complications, the modified patient-care process, the yearly trend in patient increases, disease categories, and the additional duration of admission for these procedures. RESULTS: The number of overall patients increased by an average of 51% annually. The disease categories included aneurysm (51%), atherosclerosis (11%) and arteriovenous malformation (10%), etc. for which the patient underwent angiography, and aneurysm (42%), venous malformation (28%), and arteriovenous malformation (17%), etc. for which patients underwent neurointervention. Same-day care patients were admitted to the intermediary care unit in the angiosuite. Neurointervention patients were sent to the neurology intensive unit after the procedure. The same-day care patients stayed in angiosuite for six hours following the transfemoral procedure. The mean admission duration for neurointervention was 2.4 days. There were no reported complications for the same-day care procedures. CONCLUSION: Our study revealed an increasing tendency toward same-day care for patients who require angiography and neurointervention. Further studies will be required to better define the cost-minimization effects of outpatient practice as well as the patient perception of this fast-tracking method. We propose that outpatient angiography and neurointervention will undoubtedly continue to increase over the next decade.
Ambulatory Care ; Aneurysm ; Angiography ; Arteriovenous Malformations ; Atherosclerosis ; Catheters ; Cerebral Angiography ; Humans ; Neurology ; Outpatients

Purpose: To investigate growth response in children with either idiopathic short stature (ISS) or growth hormone (GH) deficiency (GHD). Methods: The data of prepubertal GHD or ISS children treated using recombinant human GH were obtained from the LG Growth Study database. GHD children were further divided into partial and complete GHD groups. Growth response and factors predicting growth response after 1 and 2 years of GH treatment were investigated. Results: This study included 692 children (98 with ISS, 443 partial GHD, and 151 complete GHD). After 1 year, changes in height standard deviation score (ΔHt-SDS) were 0.78, 0.83, and 0.96 in ISS, partial GHD, and complete GHD, respectively. Height velocity (HV) was 8.72, 9.04, and 9.52 cm/yr in ISS, partial GHD, and complete GHD, respectively. ΔHt-SDS and HV did not differ among the 3 groups. Higher initial body mass index standard deviation score (BMI-SDS) and midparental height standard deviation score (MPH-SDS) were predictors for better growth response after 1 year in ISS and the partial GHD group, respectively. In the complete GHD group, higher Ht-SDS and BMI-SDS predicted better growth response after 1 year. After 2 years of GH treatment, higher BMI-SDS and MPH-SDS predicted a better growth outcome in the partial GHD group, and higher MPH-SDS was a predictor of good growth response in complete GHD. Conclusion Clinical characteristics and growth response did not differ among groups. Predictors of growth response differed among the 3 groups, and even in the same group, a higher GH dose would be required when poor response is predicted.

Morning glory syndrome (MGS) is a rare congenital optic disc anomaly with a characteristic fundal finding with severe visual impairment. It may occur in association with various systemic manifestations, even though most of the reported cases were isolated. A 6-year-old male visited the nephrology clinic with a history of microscopic hematuria and at the age of 12 years, he was diagnosed thin glomerular basement membrane nephropathy by kidney biopsy. After the following years, the patient had progressive deterioration of visual acuity, and diagnosed as MGS. Whole Exome Sequencing of this patient and his mother revealed heterozygous COL4A4 mutations [c.81_86del (p.Ile29_Leu30del)]. It is more reasonable to consider MGS seen in this patient as a coincidental finding of autosomal dominant Alport syndrome. To our knowledge, this case represents the first case report of autosomal dominant Alport syndrome associated with MGS.

Exposure to storage mite (SM) and house dust mite (HDM) allergens is a risk factor for sensitization and asthma development; however, the related immune responses and their pathology have not been fully investigated. The HDMs Dermatophagoides farinae and Dermatophagoides pteronyssinus and SM Tyrophagus putrescentiae are potent allergens that induce asthma. Most SM-related studies have focused on the allergic reactions of individuals by measuring their immunoglobulin (Ig)E expression. Considering the limited research on this topic, the present study aims to investigate the differences in the immune responses induced by HDMs and SMs and histologically analyze lung tissues in a mouse asthma model to understand the differential effects of HDM and SM. The results revealed that all mite species induced airway inflammation. Mice challenged with T. putrescentiae had the highest airway resistance and total cell, eosinophil, and neutrophil counts in the bronchoalveolar lavage fluid (BALF). The SM-sensitized groups showed more severe lesions and mucus hypersecretions than the HDM-sensitized groups. Although the degree of HDM and SM exposure was the same, the damage to the respiratory lung tissue was more severe in SM-exposed mice, which resulted in excessive mucin secretion and increased fibrosis. Furthermore, these findings suggest that SM sensitization induces a more significant hypersensitivity response in mucosal immunity than HDM sensitization in asthma models.

Exposure to storage mite (SM) and house dust mite (HDM) allergens is a risk factor for sensitization and asthma development; however, the related immune responses and their pathology have not been fully investigated. The HDMs Dermatophagoides farinae and Dermatophagoides pteronyssinus and SM Tyrophagus putrescentiae are potent allergens that induce asthma. Most SM-related studies have focused on the allergic reactions of individuals by measuring their immunoglobulin (Ig)E expression. Considering the limited research on this topic, the present study aims to investigate the differences in the immune responses induced by HDMs and SMs and histologically analyze lung tissues in a mouse asthma model to understand the differential effects of HDM and SM. The results revealed that all mite species induced airway inflammation. Mice challenged with T. putrescentiae had the highest airway resistance and total cell, eosinophil, and neutrophil counts in the bronchoalveolar lavage fluid (BALF). The SM-sensitized groups showed more severe lesions and mucus hypersecretions than the HDM-sensitized groups. Although the degree of HDM and SM exposure was the same, the damage to the respiratory lung tissue was more severe in SM-exposed mice, which resulted in excessive mucin secretion and increased fibrosis. Furthermore, these findings suggest that SM sensitization induces a more significant hypersensitivity response in mucosal immunity than HDM sensitization in asthma models.

Exposure to storage mite (SM) and house dust mite (HDM) allergens is a risk factor for sensitization and asthma development; however, the related immune responses and their pathology have not been fully investigated. The HDMs Dermatophagoides farinae and Dermatophagoides pteronyssinus and SM Tyrophagus putrescentiae are potent allergens that induce asthma. Most SM-related studies have focused on the allergic reactions of individuals by measuring their immunoglobulin (Ig)E expression. Considering the limited research on this topic, the present study aims to investigate the differences in the immune responses induced by HDMs and SMs and histologically analyze lung tissues in a mouse asthma model to understand the differential effects of HDM and SM. The results revealed that all mite species induced airway inflammation. Mice challenged with T. putrescentiae had the highest airway resistance and total cell, eosinophil, and neutrophil counts in the bronchoalveolar lavage fluid (BALF). The SM-sensitized groups showed more severe lesions and mucus hypersecretions than the HDM-sensitized groups. Although the degree of HDM and SM exposure was the same, the damage to the respiratory lung tissue was more severe in SM-exposed mice, which resulted in excessive mucin secretion and increased fibrosis. Furthermore, these findings suggest that SM sensitization induces a more significant hypersensitivity response in mucosal immunity than HDM sensitization in asthma models.