Background: Sterility and safety assurance of hematopoietic stem cell (HSC) products is critical in transplantation. Microbial contamination can lead to product disposal and increases the risk of unsuccessful clinical outcomes. Therefore, it is important to implement and maintain good practice guidelines and regulations for the HSC collection and processing unit in each hospital. We aimed to share our experiences and suggest strategies to improve the quality assurance of HSC processing. Methods: We retrospectively analyzed microbial culture results of 11,743 HSC products processed over a 25-year period (January 1996 to May 2021). Because of reorganization of the HSC management system in 2008, the 25-year period was divided into periods 1 (January 1996 to December 2007) and 2 (January 2008 to May 2021). We reviewed all culture results of the HSC products and stored aliquot samples and collected culture results for peripheral blood and catheter samples. Results: Of the 11,743 products in total, 35 (0.3%) were contaminated by microorganisms, including 19 (0.5%) of 3,861 products during period 1 and 16 (0.2%) of 7,882 products during period 2. Penicillium was the most commonly identified microorganism (15.8%) during period 1 and coagulase-negative Staphylococcus was the most commonly identified (31.3%) during period 2. HSC product contamination occurred most often during HSC collection and processing. Conclusions The contamination rate decreased significantly during period 2, when the HSC management system was reorganized. Our results imply that handling HSC products by trained personnel and adopting established protocols, including quality assurance programs, aid in decreasing the contamination risk.

PURPOSE: To discuss the clinical course and diagnosis of corneal dysplasia in a xeroderma pigmentosum patient based on a genetic evaluation. CASE SUMMARY: A 42-year-old female visited our clinic for decreased left visual acuity and corneal opacity. She had undergone several surgeries previously due to the presence of basosquamous carcinoma in the left lower eyelid, neurofibroma, and malignant melanoma of the facial skin. The patient showed repeated corneal surface problems, with a suspicious dendritic lesion; however, antiviral therapy was ineffective, and herpes simplex virus polymerase chain reaction results were negative. Despite regular follow-ups, the patient showed neovascularization around the corneal limbus and an irregular corneal surface. We performed corneal debridement with autologous serum eye drops for treatment. The patient's visual acuity and corneal surface improved after the procedure. The impression cytology result was corneal dysplasia. In whole exome sequencing, two pathogenic variants and one likely pathogenic variant of the POLH gene were detected. CONCLUSIONS: This is the first genetically identified xeroderma pigmentosum case with ophthalmological lesions of the eyelid and cornea in Korea. Debridement of the irregular corneal surface and autologous serum eye drop administration in xeroderma pigmentosum could be helpful for improving visual acuity.
Adult ; Carcinoma, Basosquamous ; Cornea ; Corneal Opacity ; Debridement ; Diagnosis ; Exome ; Eyelids ; Female ; Follow-Up Studies ; Humans ; Ichthyosis ; Korea ; Limbus Corneae ; Melanoma ; Neurofibroma ; Ophthalmic Solutions ; Polymerase Chain Reaction ; Simplexvirus ; Skin ; Visual Acuity ; Xeroderma Pigmentosum

Adrenal hypoplasia congenita (AHC) is a rare cause of adrenal insufficiency during neonatal period. Mutations in the gene coding for DAX1 cause X-linked adrenal hypoplasia. Most affected patients are shown to have salt wasting and hyperpigmentation on the skin during the neonatal period and require intensive medical care. In addition, it is usually associated with hypogonadotropic hypogonadism in adolescence. The DAX1 gene is expressed in the adrenal cortex, pituitary gland, hypothalamus, testis, and ovary. We report on a patient with genetically confirmed AHC whose initial clinical presentations were consistent with congenital adrenal hyperplasia. A point mutation in the DAX1 gene identified in this report resulted in a truncated DAX1 protein. Our patient was diagnosed with AHC.
Adolescent ; Adrenal Cortex ; Adrenal Hyperplasia, Congenital ; Adrenal Insufficiency ; Clinical Coding ; Female ; Humans ; Hyperpigmentation ; Hypogonadism ; Hypothalamus ; Infant, Newborn* ; Korea* ; Ovary ; Pituitary Gland ; Point Mutation ; Skin ; Testis

BACKGROUND/AIMS: Recurrence after hepatic resection is one of the most important factors impacting the prognosis and survival of patients with hepatocellular carcinoma (HCC). We identified prognostic factors affecting overall survival (OS) and disease-free survival (DFS) in patients with HCC after hepatic resection. METHODS: This study was of a retrospective cohort design, and 126 patients who underwent hepatic resection for HCC at Gachon University Gil Medical Center between January 2005 and December 2010 were enrolled. Various clinical, laboratory, and pathological data were evaluated to determine the prognostic factors affecting OS and DFS. RESULTS: Two- and 4-year OS and 2- and 4-year DFS were 78.1% and 65% and 51.1% and 26.6%, respectively. In a multivariate analysis, preoperative alpha-fetoprotein (> 400 ng/mL), tumor size (> or = 5 cm), multiple tumors (two or more nodules), presence of portal vein invasion, modified Union for International Cancer Control (UICC) stage III/IV, and Barcelona Clinic Liver Cancer (BCLC) stage B/C were independent prognostic factors affecting a shorter OS. In the multivariate analysis, presence of microvascular invasion, modified UICC stage III/IV, and BCLC stage B/C were independent prognostic factors for a shorter DFS. CONCLUSIONS: The presence of vascular invasion was an independent poor prognostic factor for OS and DFS in patients with HCC after hepatic resection. Thus, close postoperative surveillance for early detection of recurrence and additional treatments are urgently needed in patients with vascular invasion after hepatic resection.
Carcinoma, Hepatocellular/blood/mortality/secondary/*surgery ; Disease-Free Survival ; Female ; *Hepatectomy/adverse effects/mortality ; Humans ; Kaplan-Meier Estimate ; Liver Neoplasms/blood/mortality/pathology/*surgery ; Male ; Middle Aged ; Multivariate Analysis ; Neoplasm Invasiveness ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Proportional Hazards Models ; Republic of Korea ; Retrospective Studies ; Risk Factors ; Time Factors ; Treatment Outcome ; Tumor Burden ; alpha-Fetoproteins/analysis

BACKGROUND/AIMS: Reactivation of hepatitis B virus (HBV) has been reported in HBV surface antigen (HBsAg)-positive patients undergoing chemotherapy, as well as HBsAg-negative patients with antibodies against HBV core antigen (HBcAg) and/or HBsAg (HBsAb). Chemotherapy-including rituximab-has recently been identified as a predictive factor for HBV reactivation in HBsAg-negative patients with malignant lymphoma. The aim of our study was to identify the factors predictive of HBV reactivation after chemotherapy in patients with malignant lymphoma. METHODS: We conducted a retrospective analysis of medical records from patients diagnosed with malignant lymphoma at Gachon University Gil Medical Center in City, County from January 2005 to December 2010. We subsequently determined HBsAg, HBsAb and anti-HBc status in the 196 patients treated with chemotherapy. RESULTS: The mean age of the patients was 57.3 +/- 14.5 years; 56.3% were male. A total of 172 of 196 (88%) patients in the study population were HBsAg (+) prior to chemotherapy. Three patients (3/11, 27.3%) in the HBsAg (+) group had confirmed HBV reactivation after chemotherapy. In addition, 26 of 196 (13%) patients in the study population tested HBcAg (+) positive prior to chemotherapy. One patient (1/15, 6.7%) in the HBsAg (-)/HBcAb (+) group had confirmed HBV reactivation. In the four patients with HBV reactivation, infection was resolved after treatment with 0.5 mg entecavir or 100 mg lamivudine. CONCLUSIONS: Reactivation of HBV after systemic chemotherapy can occur in HBsAg (-) patients. We recommend that malignant lymphoma patients undergoing chemotherapy be screened for HBV infection status, including HBcAg, and followed closely to prevent HBV reactivation.
Antibodies ; Antigens, Surface ; Drug Therapy* ; Hepatitis B Core Antigens ; Hepatitis B Surface Antigens ; Hepatitis B virus* ; Hepatitis B* ; Hepatitis* ; Humans ; Lamivudine ; Lymphoma* ; Male ; Medical Records ; Retrospective Studies

Revascularization of immature necrotic teeth is a reliable treatment alternative to conventional apexogenesis or apexification. In case 1, a 12-year-old boy had his necrotic, immature mandibular left second premolar treated with a revascularization technique. At a24-month follow-up, periapical radiolucency had disappeared and thickening of the root wall was observed. In cases 2 and 3, a10-year-old boy had his necrotic, immature, bilateral mandibular second premolars treated with the same modality. At 48-month(in case 2) and 42-month (in case 3) follow-ups, loss of periapical radiolucencies and increases in the root wall thickness were also observed.
Apexification ; Bicuspid ; blood supply ; diagnostic imaging ; pathology ; surgery ; Child ; Dental Papilla ; blood supply ; drug effects ; pathology ; Dental Pulp ; blood supply ; drug effects ; pathology ; Dental Pulp Necrosis ; pathology ; therapy ; Follow-Up Studies ; Humans ; Male ; Mandible ; Neovascularization, Physiologic ; Radiography ; Regeneration ; Root Canal Irrigants ; therapeutic use ; Root Canal Therapy ; methods ; Tooth Apex ; blood supply ; diagnostic imaging ; Tooth, Deciduous ; blood supply ; pathology ; Treatment Outcome

Revascularization of immature necrotic teeth is a reliable treatment alternative to conventional apexogenesis or apexification. In case 1, a 12-year-old boy had his necrotic, immature mandibular left second premolar treated with a revascularization technique. At a 24-month follow-up, periapical radiolucency had disappeared and thickening of the root wall was observed. In cases 2 and 3, a 10-year-old boy had his necrotic, immature, bilateral mandibular second premolars treated with the same modality. At 48-month (in case 2) and 42-month (in case 3) follow-ups, loss of periapical radiolucencies and increases in the root wall thickness were also observed.

PURPOSE: Intraductal papilloma of the breast (IDP) is the most common causes of nipple discharge and it is often a solitary, centrally located tumor that most commonly occurs in the fifth and sixth decades of life. There have been many conflicting reports on the malignant potential of IDP. METHODS: From February 2003 to November 2008, we operated 161 patients who were diagnosed with IDP at Konyang University Hospital. A retrospective review of all the pathologic reports and the corresponding radiological reports was undertaken. RESULTS: The mean age of the patients was 43.23 years (SD, +/-10.1). Eighty-three (51.6%) had nipple discharge, 37 (44.6%) had bloody discharge and 46 (55.4%) had yellowish serous discharge. Twenty-four had a mass and 2 had both bloody discharge and a mass. Radiologic significant findings were shown on 20 mammography exams, 71 ductography exams and 157 breast ultrasound exams. Onehundred forty-four (89.4%) patients with IDP or papillomatosis had surrounding pathologic lesions in the operated specimen according to the final pathology, 107 (66.5%) had fibrocystic change, 26 (16.1%) had fibroadenoma, 30 (18.6%) had atypical ductal hyperplasia (ADH), 11 (6.8%) had carcinoma in situ and 4 (2.5%) had invasive ductal carcinoma. During the follow up, 7 patients (4.3%) developed recurrent IDP, 3 patients developed ADH and 5 patients developed carcinoma in situ (4 patients had ductal carcinoma in situ and 1 patient had lobular carcinoma in situ). CONCLUSION: Intraductal papilloma should be closely followed up due to its malignant potential and the surrounding breast tissue with IDP should be carefully evaluated due to the high rate of developing other precancerous lesions.
Breast ; Carcinoma in Situ ; Carcinoma, Ductal ; Carcinoma, Intraductal, Noninfiltrating ; Carcinoma, Lobular ; Fibroadenoma ; Follow-Up Studies ; Humans ; Hyperplasia ; Mammography ; Nipples ; Papilloma ; Papilloma, Intraductal ; Precancerous Conditions ; Retrospective Studies

PURPOSE: Intraductal papilloma of the breast (IDP) is the most common causes of nipple discharge and it is often a solitary, centrally located tumor that most commonly occurs in the fifth and sixth decades of life. There have been many conflicting reports on the malignant potential of IDP. METHODS: From February 2003 to November 2008, we operated 161 patients who were diagnosed with IDP at Konyang University Hospital. A retrospective review of all the pathologic reports and the corresponding radiological reports was undertaken. RESULTS: The mean age of the patients was 43.23 years (SD, +/-10.1). Eighty-three (51.6%) had nipple discharge, 37 (44.6%) had bloody discharge and 46 (55.4%) had yellowish serous discharge. Twenty-four had a mass and 2 had both bloody discharge and a mass. Radiologic significant findings were shown on 20 mammography exams, 71 ductography exams and 157 breast ultrasound exams. Onehundred forty-four (89.4%) patients with IDP or papillomatosis had surrounding pathologic lesions in the operated specimen according to the final pathology, 107 (66.5%) had fibrocystic change, 26 (16.1%) had fibroadenoma, 30 (18.6%) had atypical ductal hyperplasia (ADH), 11 (6.8%) had carcinoma in situ and 4 (2.5%) had invasive ductal carcinoma. During the follow up, 7 patients (4.3%) developed recurrent IDP, 3 patients developed ADH and 5 patients developed carcinoma in situ (4 patients had ductal carcinoma in situ and 1 patient had lobular carcinoma in situ). CONCLUSION: Intraductal papilloma should be closely followed up due to its malignant potential and the surrounding breast tissue with IDP should be carefully evaluated due to the high rate of developing other precancerous lesions.
Breast ; Carcinoma in Situ ; Carcinoma, Ductal ; Carcinoma, Intraductal, Noninfiltrating ; Carcinoma, Lobular ; Fibroadenoma ; Follow-Up Studies ; Humans ; Hyperplasia ; Mammography ; Nipples ; Papilloma ; Papilloma, Intraductal ; Precancerous Conditions ; Retrospective Studies

PURPOSE: In Korea, colorectal cancer (CRC) is one of the most sharply-increasing malignancies, and the National Colorectal Cancer Screening Program for persons over 50 years of age began in 2004. To determine the effectiveness of the program, comparative data regarding CRCs treated prior to 2004 must be analyzed. The present study assessed CRC status at diagnosis and treatment patterns in 2003. METHODS: In 2003, 503 patients were newly diagnosed with CRC and were treated at the Center for Colorectal Cancer, National Cancer Center (NCC). Clinical data were retrospectively reviewed. RESULTS: The 503 patients included 256 colon and 247 rectal cancer patients. Of the 256 colon cancer patients, 5 (2.0%) were diagnosed during screening colonoscopies and were successfully treated using an endoscopic mucosal resection (EMR), and 17 (6.6%) received only palliative chemotherapy because of distant metastases. Forty patients (15.6%) were treated with palliative surgery and chemotherapy, and 194 (75.8%) with curative surgery with or without adjuvant chemotherapy. Of the 247 rectal cancer patients, 9 (3.6%) were treated with an EMR, 20 (8.1%) with palliative chemotherapy with or without radiotherapy, 19 (7.7%) with palliative surgery and chemoradiotherapy, and 199 (80.6%) with curative surgery with or without chemoradiotherapy. Treatment with curative intent was possible in 199 of 256 (77.7%) colon cancer patients and in 208 of 247 (84.2%) rectal cancer patients. CONCLUSIONS: Only 12.1% of colon and 8.5% of rectal cancer patients were diagnosed early and treated without adjuvant therapies at the NCC in Korea in 2003.
Chemoradiotherapy ; Chemotherapy, Adjuvant ; Colon ; Colonic Neoplasms ; Colonoscopy ; Colorectal Neoplasms* ; Diagnosis ; Drug Therapy ; Humans ; Korea* ; Mass Screening ; Neoplasm Metastasis ; Palliative Care ; Radiotherapy ; Rectal Neoplasms ; Retrospective Studies