No abstract available.
Adult ; Hidradenitis ; Humans

No abstract available.
Acupuncture ; Sarcoidosis

BACKGROUND/AIMS: We investigated the risk factors for short-term recurrence and analyzed the correlation between subjective clinical symtoms and objective radiological findings in patients with achalasia undergoing pneumatic balloon dilatation. METHODS: Twenty patients who were treated by pneumatic balloon dilatation were enrolled. We compared prospectively various indices before and after the treatment as follows: 1) Eckardt symptom score and dysphagia grade, 2) The ratio of the maximal width in mid-esophageal lumen to the minimal width in distal esophagus around lower esophageal sphincter, and 3) the percentage of maximum activity retained in the esophagus at 30 seconds and T in esophageal scan two days after the treatment. RESULTS: 1) Clinical indices and radiologic indices significantly improved after pneumatic dilatation. 2) There was no significant correlation between the clinical indices and the radiologic indices before and after the treatment. 3) The difference percentage of clinical indices did not show significant correlation with the difference percentage of the radiologic indices. 4) Compared to the group above 20% in the difference percentage of 30 second residual fraction, the one below 20% had a four-fold risk in short-term recurrence. CONCLUSIONS: Clinical symptoms and radiologic indices significantly improve after pneumatic dilatation but have no significant correlation to each other. The group below 20% in the difference percentage of 30 second residual fraction has a high risk of recurrence and may need careful examination and early repeated pneumatic dilation.
Adolescent ; Adult ; *Balloon Dilatation ; Data Interpretation, Statistical ; Esophageal Achalasia/diagnosis/*radiography/*therapy ; Esophageal Sphincter, Lower/physiopathology ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Risk Factors ; Severity of Illness Index ; Time Factors

A 32-yr-old male diagnosed with myelodysplastic syndrome underwent an unmanipulated, unrelated, HLA matched, peripheral blood stem cell transplantation. The patient and donor were both blood type O, CcDEe. Twelve weeks post-transplantation, he developed acute autoimmune hemolytic anemia (AIHA). He was transfused multiple times with washed O red cells. High-dose steroid therapy was initiated and he underwent splenectomy; however, AIHA was refractory to therapy. The patient was further treated with combined treatment modalities including immunosuppressive therapy with mycophenolate mofetil and cyclosporine and three cycles of plasma exchange, and AIHA responded to treatment. This is the third case of AIHA complicating hematopoietic stem cell transplantation reported in Korea. Since AIHA is relatively common after hematopoietic stem cell transplantation, accurate and timely diagnosis of the disease and treatment strategies with multiple modalities are necessary.
Adult ; Anemia, Hemolytic, Autoimmune/*diagnosis/drug therapy/etiology ; Combined Modality Therapy ; Cyclosporine/therapeutic use ; Hematopoietic Stem Cell Transplantation/*adverse effects ; Humans ; Male ; Mycophenolic Acid/analogs & derivatives/therapeutic use ; Myelodysplastic Syndromes/complications/diagnosis/therapy ; Plasma Exchange

BACKGROUND/AIMS: Esophageal eosinophilia occurs in many conditions, including eosinophilic esophagitis (EoE) and proton pump inhibitor-responsive esophageal eosinophilia (PPI-REE), which have been increasingly recognized in Western countries. There have been only a few reports in Korea. Here, we evaluated the clinical and endoscopic characteristics of patients with esophageal eosinophilia from our experience. METHODS: Nineteen patients were diagnosed with esophageal eosinophilia based on typical symptoms, endoscopic features, esophageal eosinophilia with ≥15 eosinophils/high power field, and response to medication by PPI. Symptoms, endoscopic and pathological findings were evaluated. RESULTS: Of the 19 patients, 2 patients were diagnosed with EoE, 7 patients were diagnosed with PPI-REE, and 10 patients were undetermined due to loss to follow-up. Among these 19 patients, dysphagia was present in 11, and heartburn, dyspepsia and reflux in 8. Sixteen patients had common endoscopic features, such as longitudinal furrows, concentric rings, strictures, and white plaques; however, 3 patients had normal findings. Nine patients underwent endoscopy at the time of follow-up. Two patients had complete resolution, and 3 had partial resolution. However, 4 patients showed no endoscopic changes. All patients showed symptom improvements. CONCLUSIONS: The clinical and endoscopic characteristics of both groups in Korea were undistinguishable. However, after treatment, endoscopic findings were different between the two groups. Large-scale studies are warranted to confirm our findings.
Constriction, Pathologic ; Deglutition Disorders ; Dyspepsia ; Endoscopy ; Eosinophilia* ; Eosinophilic Esophagitis ; Esophagus ; Follow-Up Studies ; Heartburn ; Humans ; Korea ; Proton Pump Inhibitors ; Proton Pumps

Most cases of congenital hyperthyroidism are autoimmune forms caused by maternal thyroid stimulating antibodies. Nonautoimmune forms of congenital hyperthyroidism caused by activating mutations of the thyrotropin receptor (TSHR) gene are rare. A woman gave birth to a boy during an emergency cesarean section at 33 weeks of gestation due to fetal tachycardia. On the 24th day of life, thyroid function tests were performed due to persistent tachycardia, and hyperthyroidism was confirmed. Auto-antibodies to TSHR, thyroid peroxidase, and thyroglobulin were not found. The patient was treated with propylthiouracil and propranolol, but hyperthyroidism was not well controlled. At 3 months of age, the patient had craniosynostosis and hydrocephalus, and underwent a ventriculoperitoneal shunt operation. Direct sequencing of the TSHR gene showed a heterozygous mutation of c.1899C>A (p.Asp633Glu) in exon 10. No mutations were discovered in any of the parents in a familial genetic study. We have reported a case of sporadic nonautoimmune congenital hyperthyroidism, by a missense mutation of the TSHR gene, for the first time in South Korea.
Cesarean Section ; Craniosynostoses ; Emergencies ; Exons ; Female ; Germ-Line Mutation ; Humans ; Hydrocephalus ; Hyperthyroidism* ; Immunoglobulins, Thyroid-Stimulating ; Iodide Peroxidase ; Korea ; Male ; Mutation, Missense ; Parents ; Parturition ; Pregnancy ; Propranolol ; Propylthiouracil ; Receptors, Thyrotropin ; Tachycardia ; Thyroglobulin ; Thyroid Function Tests ; Ventriculoperitoneal Shunt

Background: Vulvar dermatoses are common, but numerous obstacles impede their adequate clinical investigation. Many outpatient patients with vulvar dermatoses had inappropriate behavioral habits to manage their diseases. Objective: This study was performed to investigate the clinical aspects of vulvar dermatoses and to evaluate the proper management for vulvar eczema and pruritus vulvae. Methods: We retrospectively reviewed medical records from female patients presenting with vulvar dermatoses in a tertiary hospital and analyzed the age, clinical manifestation, diagnosis, and further evaluated treatment and clinical course of vulvar eczema and pruritus vulvae. Results: A total of 163 patients were reviewed in this study. The most frequent type of skin manifestation was macule/patch, with itching as the most common symptom. Lichen simplex chronicus (35, 21.5%) was the most common dermatosis, followed by lichen sclerosus et atrophicus (21, 12.9%) and Behcet’s disease (18, 11.0%). In addition, 57 patients (35.0%) suffered from acute/chronic vulvar eczema or pruritus vulvae and over half of them had been misdiagnosed as fungal infection or had mistreated themselves with multiple self-products. These patients mostly showed good response to hygiene management plus low potency topical corticosteroids. Conclusion In this study, we identified clinically common vulvar dermatoses in a cohort of women visiting our dermatology clinic. We found that patients with vulvar eczema or pruritus vulvae suffered from misdiagnosis and inappropriate self-management sustained patients’ problems. They could be effectively controlled by education of hygiene management with/without steroids. Further prospective investigation with a larger group of patients would provide better understanding of the characteristics of vulvar dermatoses.

An 80-year-old woman presented with a yellowish to erythematous plaque that persisted on her left temple for several years. Histopathologic examination revealed a large area of atypical squamous cell infiltration into the epidermis and dermis and a small area of neoplastic basaloid and clear cell proliferation with nuclear atypia in the central portion of the lesion. Immunohistochemical staining was positive and negative for epithelial membrane antigen and cytokeratin 7, respectively, in both the squamous cell and central clear cell infiltration areas. The patient was diagnosed with sebaceous carcinoma associated with squamous cell carcinoma in situ and underwent surgical excision. There have been several reports of sebaceous carcinoma with coinciding tumors in the same lesion, including nevus sebaceous and actinic keratosis. Here, we report a case of sebaceous carcinoma with coexisting squamous cell carcinoma in situ in a patient with a previous history of actinic keratosis in the temple area.

Invasive fungal infections (IFIs) are common causes of mortality and morbidity in patients with hematologic diseases. Delayed initiation of antifungal treatment is related to mortality. Aspergillus sp. is the leading cause of IFI followed by Candida sp. Diagnosis is often challenging owing to variable conditions related to underlying diseases. Clinical suspect and prompt management is important. Imaging, biopsy, and non-culture-based tests must be considered together. New diagnostic procedures have been improved, including antigen-based assays and molecular detection of fungal DNA. Among hematologic diseases, patients with acute myeloid leukemia, myelodysplastic syndrome, recipients of hematopoietic stem cell transplantation are at high risk for IFIs. Antifungal prophylaxis is recommended for these high-risk patients. There are continuous attempts to achieve ideal management of IFIs. Scoring system for quality control has been developed with important recommendations of current guidelines. Higher adherence to guidelines is related to decreased mortality in IFIs.

Background: The skin barrier is made of the outermost layers of skin and is responsible for preventing cutaneous water loss. Skin barrier function is easily affected by external environmental factors, such as temperature and relative humidity, especially in areas with four distinct seasons, such as Busan, Korea. Objective: This study was conducted to analyze the seasonal variation in skin barrier function on the face and to determine whether it is affected by thermo-hygrostat conditioning. Methods: Ten healthy female volunteers aged 28 to 34 years were enrolled in this study. Each subject was put into a thermo-hygrostat–controlled room maintained at a temperature of 22.4°C to 23.8°C with a relative humidity of 50.2% to 52.0%. Transepidermal water loss (TEWL) and stratum corneum hydration (SCH) were measured before and after a 20-minute session in the room. Measurements were taken once in the middle of each season. Results: Regardless of thermo-hygrostat conditioning, TEWL and SCH values changed significantly between all four seasons (both p＜0.001). With thermo-hygrostat conditioning, 1 of 6 ΔTEWL and 4 of 6 ΔSCH values showed significant seasonal differences in post-hoc analysis. Difference of TEWL and SCH obtained before and after thermo-hygrostat conditioning was significant only in summer (TEWL, p=0.009; SCH, p=0.002). Conclusion Skin barrier function differed significantly between seasons, regardless of thermo-hygrostat conditioning. Thermo-hygrostat conditioning significantly affected TEWL and SCH only during summer. However, with Busan’s excessively humid summer, even a short period of thermo-hygrostat conditioning could increase the precision of skin barrier function measurement.