We herein presented a case of left ectopic ureteral orifice to the vagina in a 14 year old girl containing stones in the distal ectopic ureteral portion of associated complete duplicated ureter. She has been suffered from urinary leakage while normal voiding since birth without other urinary symptoms. She was treated with left ureteroneocystostomy.
Adolescent ; Female ; Humans ; Parturition ; Ureter* ; Vagina

PURPOSE: There are compelling evidences that house dust mites are important in the pathogenesis of atopic dermatitis. Recently, detection of house dust mite allergens from clothing, skin, and dust from human hair has been reported. To evaluate the importance of house dust mite exposure in the clinical severity of atopic dermatitis. METHODS: We measured house dust mite allergens (Der f1 from Dermatophagoides farinae and Der p1 from D. pteronyssinus) in scalp dander from 27 children with atopic dermatitis and 41 children with asthma only by enzyme-linked immunosorbent assay and examined correlation between house dust mite allergen concentrations in scalp dander and clinical severity of atopic dermatitis in children. RESULTS: Der f1 was detectable in scalp dander samples of 32 patients (47%) from overall 68 patient and Der p1 was detectable in 33 patients (49%). House dust mite allergens (Der f1 or Der p1) were detectable in scalp dander samples of 46 patients (70%) from overall 68 patient. There was no significant differences in house dust mite allergen levels between atopic dermatitis patients and patients with asthma only. There was a tendency of inverse correlation between frequency of scalp washing and concentration of Der f1 in scalp dander extract (r=-0.24, P=0.052). There was a significant correlation between severity grade of atopic dermatitis and concentration of Der f1 in scalp dander (r=0.39, P<0.05) in 27 children with atopic dermatitis. However, there was no significant correlation between severity grade of atopic dermatitis and concentration of Der p1 in scalp dander (r=-0.05, P>0.05). CONCLUSION: There was a significant correlation between house dust mite allergen concentrations in scalp dander and clinical severity in children with atopic dermatitis. And these results suggest that exposure to house dust mite allergen is important in the pathogenesis of atopic dermatitis.
Allergens ; Asthma ; Child* ; Clothing ; Dander* ; Dermatitis, Atopic* ; Dermatophagoides farinae ; Dust* ; Enzyme-Linked Immunosorbent Assay ; Hair ; Humans ; Pyroglyphidae* ; Scalp* ; Skin

Background: Liver transplant (LT) recipients were considered a vulnerable population during the coronavirus disease 2019 (COVID-19) pandemic. The clinical efficacy of the COVID-19 vaccine is unknown in immunocompromised patients. The purpose of this study was to provide evidence of antibody responses after COVID-19 vaccination in LT recipients. Methods: This study enrolled 46 patients who underwent LT at Samsung Medical Center (Seoul, Korea) before implementation of the one-dose vaccine in Korea. Those who completed the two-dose COVID-19 vaccine between August 2021 and September 2021 were included and followed through December 2021. Semiquantitative anti-spike serologic testing was performed using the Roche Elecsys anti-SARS-CoV-2 S enzyme immunoassay (Roche Diagnostics, Rotkereuz, Switzerland) with a positive cutoff of at least 0.8 U/mL. Results: Among all 46 participants, 40 (87%) demonstrated an antibody response after the second dose of a COVID-19 vaccine, while six (13%) had no antibody response after the second dose. Upon univariate analysis, patients with higher antibody titer had longer years since LT (2.3 ± 2.8 vs. 9.4 ± 5.0, P < 0.001). A lower median tacrolimus (TAC) level before vaccination and after the second dose of COVID-19 vaccine indicated a significantly higher antibody response (2.3 [1.6–3.2] vs. 7.0 [3.7–7.8], P = 0.006, 2.5 [1.6–3.3] vs. 5.7 [4.2–7.2], P = 0.003). Period between 2nd vaccination and serologic testing was significantly higher in the antibody-response group compared to the no-antibody-response group (30.2 ± 24.0 vs. 65.9 ± 35.0, P = 0.012). A multivariate analysis of antibody responses revealed TAC level before vaccination as a statistically significant factor. Conclusion A higher TAC level before vaccination resulted in less effective vaccination in LT patients. Booster vaccinations are required, especially for patients in the early stage after LT who have compromised immune function.

PURPOSE: X-linked agammaglobulinemia(XLA) is an immunodeficiency caused by abnormalities in Bruton's tyrosine kinase(Btk), and is characterized by a deficiency of peripheral blood B cells. We studied the cytoplasmic expression of Btk protein and analyzed the Btk gene in peripheral blood mononuclear cells from two siblings and one cousin with XLA, as well as additional family members. METHODS: Btk protein expression was analyzed by flow cytometry. Isolation of the coding sequence of the Btk gene was performed by amplification using the reverse transcription-polymerase chain reaction(RT-PCR) technique. Sequence alterations were screened by the single-stranded conformation polymorphism(SSCP) method and characterized by standard sequencing protocols. RESULTS: Cytoplasmic expression of Btk protein in monocytes was not detected in three patients with XLA. In addition, Btk protein analysis clearly showed cellular mosaicism in monocytes from four obligate carriers, findings further supported by SSCP. A single base pair mutation(T to C) in Btk-exon three, which encodes the PH domain, was identified in four XLA patients. A diagnostic sequencing analysis was established to detect heterozygotic pattern in 4 carrier females. Furthermore, we found significant clinical heterogeneity in individuals with the same gene mutation. CONCLUSION: The implicating genetic alteration provided valuable clues to the pathogenesis of XLA in Korea and the flow cytometric analysis was suggested as a useful tool for rapid detection of XLA patients and carriers. The present study has identified a genetic mutation in the Btk coding region and demonstrated heterogeneity in clinical manifestations among patients with the same mutation. A flow cytometric analysis was found to be informative in establishing a deficiency of Btk protein in both patients and carriers and is recommended as a frontline procedure in the molecular diagnosis and work-up of XLA.
Agammaglobulinemia* ; B-Lymphocytes ; Base Pairing ; Clinical Coding ; Cytoplasm ; Diagnosis ; Female ; Flow Cytometry ; Humans ; Hydrogen-Ion Concentration ; Korea ; Monocytes ; Mosaicism ; Polymorphism, Single-Stranded Conformational ; Population Characteristics ; Protein-Tyrosine Kinases* ; Siblings ; Tyrosine*

PURPOSE: This study compared the patency of the splenic vessels between laparoscopic and open spleen and splenic vessel-preserving distal pancreatectomy. METHODS: We retrospectively reviewed a database of 137 patients who underwent laparoscopic (n = 91) or open (n = 46) spleen and splenic vessel-preserving distal pancreatectomy at a single institute from 2001 through 2015. Splenic vessel patency was assessed by abdominal computed tomography and classified into three grades according to the degree of stenosis. RESULTS: The splenic artery patency rate was similar in both groups (97.8 vs. 95.7%, P = 0.779). Also, the splenic vein patency rate was not significantly different between the 2 groups (74.7% vs. 82.6%, P = 0.521). Postoperative wound complication was significantly lower in the laparoscopic group (19.8% vs. 28.3%, P = 0.006), and hospital stay was significantly shorter in the laparoscopic group (7 days vs. 9 days, P = 0.001) than in the open group. Median follow-up periods were 22 months (3.7–96.2 months) and 31.7 months (4–104 months) in the laparoscopic and open groups, respectively. CONCLUSION: Laparoscopic distal pancreatectomy showed good splenic vessel patency as well as open distal pancreatectomy. For this reason, splenic vessel patency is not an obstacle in performing laparoscopic splenic vessel-preserving distal pancreatectomy.
Constriction, Pathologic ; Follow-Up Studies ; Humans ; Laparoscopy ; Length of Stay ; Pancreatectomy ; Retrospective Studies ; Spleen ; Splenic Artery ; Splenic Vein ; Vascular Patency ; Wounds and Injuries

PURPOSE: The needs of stem cell transplantation are increasing in Korea under the circumstance of medical insurance coverage since 1996. But allogeneic bone marrow transplants have the limitation in acquisition of matched donor because of small number of siblings in modern society. Although unrelated stem cell transplantations were made possible in Korea with the establishment of Korean Marrow Donor Program, less than one third of patients who need stem cell transplantation are seeking the matched donors through this program and it still takes too much time and efforts. In this clinical setting, cord blood offers an exciting alternative to traditional sources of stem cells. To implement the widespread clinical use of cord blood stem cells, it is necessary to establish the cord blood bank from which stem cell specimens are obtained. METHODS: To establish the cord blood bank in Korea, we started to develop the proper methods to obtain the cord bloods. Various ways of collecting the cord bloods, separation of red blood cells and storage methods were tried and the standard recommendations were made. RESULTS: Mean volume of collected umbilical cord blood was above 70mL by open or closed method in vaginal delivery and cesarean section. Among the RBC depletion methods by using 3% gelatin, Ficoll-Hypaque, or modified Ficoll-Hypaque method, 3% gelatin sedimentation showed the best result in viability and number of mononuclear cells(MNC), CD34+ cell, and day 14 CFU-GM. The recovery rate after freezing-thawing MNC was higher in the group of using 10% DMSO and programmed freezer starting from 4degrees C than in the group of using mixed with DMSO and hydroxyethyl starch and without programmed freezer. Nine patients underwent cord blood transplantation until December 1998. Among them, 6 patients showed the long-term successful engraftment. CONCLUSION: We have developed simple, efficient, and reproducible methods for collection, processing, and storage of cord blood to establish the cord blood bank. There are some administrative issues including the legal and financial problems that possibly will be resolved by the support of the government and the medical team.
Bone Marrow ; Cesarean Section ; Dimethyl Sulfoxide ; Erythrocytes ; Female ; Fetal Blood* ; Gelatin ; Granulocyte-Macrophage Progenitor Cells ; Humans ; Insurance Coverage ; Korea* ; Pregnancy ; Siblings ; Starch ; Stem Cell Transplantation ; Stem Cells* ; Tissue Donors