Isolated myeloid sarcoma is a rare extramedullary manifestation, characterized by the presence of myeloid blasts in the body and occurring without bone marrow involvement. While the isolated myeloid sarcoma involvement in the nasal cavity of adults has been reported sparsely, there have been no documented cases involving children in South Korea. Given its rarity, diagnosing isolated myeloid sarcoma is challenging without resorting to a biopsy accompanied by a comprehensive panel of immunohistochemistry. In this context, we present a case of a 5-yearold child diagnosed with isolated myeloid sarcoma situated within the right nasal cavity. Notably, this case is unique as there is no evidence of bone marrow invasion or underlying hematological disorders.

Objectives: . This study evaluated the surgical outcomes of patients with Beckwith-Wiedemann syndrome who underwent tongue-reduction surgery and analyzed whether the malocclusion and mandibular prognathism caused by macroglossia could be improved. Methods: . A retrospective medical record review was performed for 11 patients with Beckwith-Wiedemann syndrome whose macroglossia was surgically treated. Demographic data, symptoms and signs, and intraoperative and postoperative surgical outcomes were evaluated. Surgery was performed by a single surgeon using the “keyhole” technique, involving midline elliptical excision and anterior wedge resection. Preoperative and postoperative plain skull lateral X-rays were evaluated to assess prognathism improvement. Results: . The median age at the time of surgery was 35.09 months, and the ratio of males to females was 4:7. The median surgical time was 98±31.45 minutes, and the median duration of the postoperative intensive care unit stay was 3.81±2.4 days. There were no airway complications. Two patients (18.2%) had postoperative wound dehiscence; however, there was no nerve damage, recurrence, or other complications. Among the five patients who underwent postoperative speech evaluation, all showed normal speech development, except one patient who had brain dysfunction and developmental delay. Measurements of the A point-nasion-B point (ANB) angles and sella-nasion-B point (SNB) angles (point A is the most concave point of the anterior maxilla; point B is the most concave point on the mandibular symphysis) on plain X-rays showed a significant decrease in the postoperative SNB angle (P <0.001) and a significant increase in the ANB angle (P <0.011). Conclusion . Tongue-reduction surgery is an effective and safe technique for severe forms of macroglossia associated with Beckwith-Wiedemann syndrome. In addition, it improves mandibular prognathism in young Beckwith-Wiedemann syndrome patients with macroglossia.

Background: Hematopoietic stem cell (HSC) transplantation is an important therapeutic option for many malignant and non-malignant diseases. The whole transplant process involves multiple areas and complex steps. The laboratory procedures include the collection, processing, and storage of HSC. The HSC registry aims to identify the current situation and draw improvement points by voluntarily registering the information of an HSC graft collected by each institute sharing the analyzed data. This study analyzed and shared the data for 2018. Methods: Data for 2018 registered at the HSC registry website (www.ksfa-registry.org) was downloaded and analyzed. The data were to enter the information of each collection and include the demography of the donors, transplant type, instrument, vascular access, mobilization modality, and the number of CD34＋ cells. Results: Two thousand eight hundred eighty-eight collection datasets from 1,373 donors were registered from 19 institutes, which was slightly higher than that reported in 2017. The number of collections in one patient was in the range of 1∼17 times, and the average was two times. In allogeneic HSCT, the number of related donors was higher than that of unrelated donors. The frequency of collecting more than four times per donor was 25.2% for autologous donors, compared to 95.4% for allogeneic donors less than twice. Conclusion The HSC registry is not limited to identifying the current situation and sharing the analyzed data, but is expected to contribute to the development of guidelines, education of human resources, and the standardization of laboratory procedures involved in hematopoietic stem cell transplantation.

Nocardiosis is a rare but potentially life-threatening infectious disease caused by several species of the genus Nocardia (N.), which are aerobic, filamentous, gram-positive bacilli. A definitive diagnosis depends on the isolation and identification of Nocardia species. But identification from clinical specimens may involve performing invasive techniques on the patient and lengthy process (take up to 1 or 3 weeks) owing to slow growth, and require a professional microbiologist. Currently the genus Nocardia is best identified using 16S rRNA and 18S rRNA gene sequence analysis. Recently matrix-assisted laser desorption ionization-time of flight mass spectrometry (MALDI-TOF MS) has launched a new era in the routine microbiology laboratory. This method has proved its efficacy for the identification and diagnosis of microorganism. MALDI-TOF MS has potential for use as a rapid (within 1 hour) and dependable method for the identification of Nocardia species with reproducibility and cost effectiveness. We report a 76-year-old woman who suffered from ulcer with papules on her right wrist and forearm. A biopsy of the skin showed granulomatous inflammation with central suppuration. A bacterial isolate from the skin was identified to be N. brasiliensis on comparative 16S rRNA gene sequencing and MALDI-TOF MS. To the best of our knowledge, this is the first case of nocardiosis in Korea caused by N. brasiliensis identified on MALDI-TOF MS.
Aged ; Biopsy ; Communicable Diseases ; Cost-Benefit Analysis ; Diagnosis* ; Female ; Forearm ; Genes, rRNA ; Humans ; Inflammation ; Korea ; Mass Spectrometry* ; Methods ; Nocardia ; Nocardia Infections* ; Sequence Analysis ; Skin ; Suppuration ; Ulcer ; Wrist

Amelanotic melanoma comprises only 1.8~8.1% of malignant melanomas, and is difficult to diagnose clinically due to the lack of the diagnostic evidence of clinical pigmentation. To our knowledge, it is rarely reported, and only 10 cases have been reported in the Korean dermatological literature. It presents rather conflicting features such as a pink or red macule, papule, plaque, or nodule mimicking various benign and malignant conditions; therefore, it is difficult to diagnose. We performed a review of six patients with amelanotic melanoma focusing on differential diagnosis, particularly at the time of the initial visit. Clinical impressions included pyogenic granuloma, dermatofibrosarcoma protuberans, eccrine poroma, epidermal cyst, keloid, pilomatricoma, and squamous cell carcinoma in addition to malignant melanoma. The biopsy specimens were consistent with malignant melanoma with little or no melanin pigment on hematoxylin and eosin and Fontana-Masson stains. Four of the six patients were positive for S-100 and HMB-45, but two patients were positive for S-100 only. We report these cases to remind clinicians of the necessity of including malignant melanoma in the differential diagnosis process when patients show poor and unpredictable responses to treatment after a clinical diagnosis of other benign and malignant conditions.
Biopsy ; Carcinoma, Squamous Cell ; Coloring Agents ; Dermatofibrosarcoma ; Diagnosis ; Diagnosis, Differential* ; Eosine Yellowish-(YS) ; Epidermal Cyst ; Granuloma, Pyogenic ; Hematoxylin ; Humans ; Keloid ; Melanins ; Melanoma ; Melanoma, Amelanotic* ; Pigmentation ; Pilomatrixoma ; Poroma

BACKGROUND: Although transfusion in neonates needs to be strictly regulated due to the vulnerability of neonates, there is lack of systematic studies and the working process is not well-established. This study was aimed to point out the problems of current status and to improve the efficiency of systems used in blood aliquots for neonatal transfusions. METHODS: Total red blood cell (RBC) aliquots were analyzed between May 2009 and January 2016 in the neonate intensive care unit. We investigated the aliquot number, issued day interval from the first issued aliquot among the post-aliquots, patients' blood type, and discarded RBC units among the requested RBC units. RESULTS: Of the 472 RBC aliquots, 95.4% (450/472) were divided into two units. The distribution of patients' blood type was similar to that of the Korean population, in decreasing order: A blood group (34.3%), B group (28.2%), and O group (27.5%). The second, third, and forth units of post-aliquots were taken after an average of 49.9 (0∼617.9) hours. Among the post-aliquots, the number of units discarded without use was 22.5%. CONCLUSION: According to the evaluation of current status for neonatal transfusions, we should use aliquot RBC properly and reduce unnecessary requests for aliquot RBC. In addition, in order to reduce the number of near misses, we propose a new label to be attached on the aliquotted blood bags and suggest a development of electronic blood issuing system.
Erythrocytes* ; Humans ; Infant, Newborn ; Intensive Care Units

No abstract available.
Arthrodermataceae* ; Epidemiology* ; Korea*

BACKGROUND: Various systemic agents have been assessed for the treatment of alopecia areata (AA); however, there is a paucity of comparative studies. OBJECTIVE: To assess and compare cyclosporine and betamethasone minipulse therapy as treatments for AA with regard to effectiveness and safety. METHODS: Data were collected from 88 patients who received at least 3 months of oral cyclosporine (n=51) or betamethasone minipulse therapy (n=37) for AA. Patients with ≥50% of terminal hair regrowth in the alopecic area were considered responders. RESULTS: The responder of the cyclosporine group was 54.9% and that of the betamethasone minipulse group was 37.8%. In the cyclosporine group, patients with mild AA were found to respond better to the treatment. Based on the patient self-assessments, 70.6% of patients in the cyclosporine group and 43.2% of patients in the betamethasone minipulse group rated their hair regrowth as excellent or good. Side effects were less frequent in the cyclosporine group. CONCLUSION: Oral cyclosporine appeared to be superior to betamethasone minipulse therapy in terms of treatment effectiveness and safety.
Alopecia Areata* ; Alopecia* ; Betamethasone* ; Cyclosporine* ; Hair ; Humans ; Pulse Therapy, Drug ; Self-Assessment ; Treatment Outcome

No abstract available.
Neurofibromatoses*

No abstract available.
Exophiala* ; Phaeohyphomycosis*