The frequency of invasive fungal infections has increased during the period of chemotherapy. Fungal infections are an increasing cause of morbidity and mortality in patients with hematological malignancies. The most common organism are Candida albicans. This report describes our experience in a 28-year-old woman who developed symptoms of hepatic candidiasis which were confirmed with liver biopsy after remission induction chemotherapy for acute myeloid leukemia. In our case, the patient did not improve clinically despite administration of 610 mg amphotericin B. Severe leukocytosis, increasing alkaline phosphatase and clinical deterioration developed rapidly, and then we switched to fluconazole, taking into consideration successful fluconazole treatment of patients with chronic disseminated candidiasis and acute hepatic candidiasis. She remains in clinically and radiological improving at the time of this report.
Adult ; Alkaline Phosphatase ; Amphotericin B ; Biopsy ; Candida albicans ; Candidiasis* ; Drug Therapy ; Female ; Fluconazole* ; Hematologic Neoplasms ; Humans ; Leukemia, Myeloid, Acute ; Leukocytosis ; Liver ; Mortality ; Remission Induction

Autoimmune hepatitis (AIH) is an undissolved inflammatory process of the liver characterized by the periportal hepatitis on histological examination and serum autoantibody. AIH seems to be partly responsible for chronic liver disease of unknown etiology in Korea. Perinuclear-Antineutrophil nuclear antibody (p-ANNA) are detected in up to 88% of patient with primary sclerosing cholangitis (PSC) and presence of p-ANNA in PSC makes them a reasonable diagnostic maker in conjuction with standard diagnostic test. But p-ANNA in AIH is rare and it's role remain unclear. We report the first case of 39 year-old-female patient with AIH type-1 with p-ANNA strong positivity.
Cholangitis, Sclerosing ; Diagnostic Tests, Routine ; Hepatitis ; Hepatitis, Autoimmune* ; Humans ; Korea ; Liver ; Liver Diseases

Although most of pseudocysts as one of complications of pancreatitis occur primarily within the pancreas, the extrapancreatic locations of pseudocysts, especially in the liver, are rare events. With advanced technology of imaging studies including abdominal computed tomography, ultrasonography, and magnetic resonance imaging, their frequency seems to be increasing. We report here a case of left intrahepatic pancreatic pseudocyst following acute pancreatitis. Percutaneous puncture revealed a high level of amylase and lipase in the collection, confirming the diagnosis of intrahepatic pseudocyst. Symptomatic intrahepatic pseudocysts can be managed surgically, transcutaneously or endoscopically, and asymptomatic intrahepatic pseudocysts can be treated conservatively. We report this case with a review of literature.
Acute Disease ; Aged ; Humans ; Liver Diseases/*diagnosis/etiology/ultrasonography ; Magnetic Resonance Imaging ; Male ; Pancreatic Pseudocyst/*diagnosis/etiology/ultrasonography ; Pancreatitis, Alcoholic/complications/*diagnosis/ultrasonography ; Tomography, X-Ray Computed

Purpose: A widely distributed cell cycle inhibitor, p27, regulates cyclin-dependent kinase-cyclin complexes. Although the prognostic value of p27 has been established for various types of carcinomas, its role in luminal breast cancer remains poorly understood. This study aimed to explore the functional enrichment of p27 and identify potential drug targets in patients with luminal-type breast cancer. Methods: Clinicopathological data were collected from 868 patients with luminal-type breast cancer. Additionally, publicly available data from the Molecular Taxonomy of Breast Cancer International Consortium (METABRIC) dataset (1,500 patients) and the Gene Expression Omnibus database (855 patients) were included in the analysis. Immunohistochemical staining for p27, differential gene expression analysis, disease ontology analysis, survival prediction modeling using machine learning (ML), and in vitro drug screening were also performed. Results: Low p27 expression correlated with younger age, advanced tumor stage, estrogen receptor/progesterone receptor negativity, decreased cluster of differentiation 8+ T cell count, and poorer survival outcomes in luminal-type breast cancer. The METABRIC data revealed that reduced cyclin-dependent kinase inhibitor 1B (CDKN1B) expression (encoding p27) was associated with cell proliferation-related pathways and epigenetic polycomb repressive complex 2. Using ML, p27 emerged as the second most significant survival factor after N stage, thereby enhancing survival model performance. Additionally, luminal-type breast cancer cell lines with low CDKN1B expression demonstrated increased sensitivity to specific anticancer drugs such as voxtalisib and serdemetan, implying a potential therapeutic synergy between CDKN1B-targeted approaches and these drugs. Conclusion The integration of ML and bioinformatic analyses of p27 has the potential to enhance risk stratification and facilitate personalized treatment strategies for patients with breast cancer.

Purpose: A widely distributed cell cycle inhibitor, p27, regulates cyclin-dependent kinase-cyclin complexes. Although the prognostic value of p27 has been established for various types of carcinomas, its role in luminal breast cancer remains poorly understood. This study aimed to explore the functional enrichment of p27 and identify potential drug targets in patients with luminal-type breast cancer. Methods: Clinicopathological data were collected from 868 patients with luminal-type breast cancer. Additionally, publicly available data from the Molecular Taxonomy of Breast Cancer International Consortium (METABRIC) dataset (1,500 patients) and the Gene Expression Omnibus database (855 patients) were included in the analysis. Immunohistochemical staining for p27, differential gene expression analysis, disease ontology analysis, survival prediction modeling using machine learning (ML), and in vitro drug screening were also performed. Results: Low p27 expression correlated with younger age, advanced tumor stage, estrogen receptor/progesterone receptor negativity, decreased cluster of differentiation 8+ T cell count, and poorer survival outcomes in luminal-type breast cancer. The METABRIC data revealed that reduced cyclin-dependent kinase inhibitor 1B (CDKN1B) expression (encoding p27) was associated with cell proliferation-related pathways and epigenetic polycomb repressive complex 2. Using ML, p27 emerged as the second most significant survival factor after N stage, thereby enhancing survival model performance. Additionally, luminal-type breast cancer cell lines with low CDKN1B expression demonstrated increased sensitivity to specific anticancer drugs such as voxtalisib and serdemetan, implying a potential therapeutic synergy between CDKN1B-targeted approaches and these drugs. Conclusion The integration of ML and bioinformatic analyses of p27 has the potential to enhance risk stratification and facilitate personalized treatment strategies for patients with breast cancer.

Purpose: A widely distributed cell cycle inhibitor, p27, regulates cyclin-dependent kinase-cyclin complexes. Although the prognostic value of p27 has been established for various types of carcinomas, its role in luminal breast cancer remains poorly understood. This study aimed to explore the functional enrichment of p27 and identify potential drug targets in patients with luminal-type breast cancer. Methods: Clinicopathological data were collected from 868 patients with luminal-type breast cancer. Additionally, publicly available data from the Molecular Taxonomy of Breast Cancer International Consortium (METABRIC) dataset (1,500 patients) and the Gene Expression Omnibus database (855 patients) were included in the analysis. Immunohistochemical staining for p27, differential gene expression analysis, disease ontology analysis, survival prediction modeling using machine learning (ML), and in vitro drug screening were also performed. Results: Low p27 expression correlated with younger age, advanced tumor stage, estrogen receptor/progesterone receptor negativity, decreased cluster of differentiation 8+ T cell count, and poorer survival outcomes in luminal-type breast cancer. The METABRIC data revealed that reduced cyclin-dependent kinase inhibitor 1B (CDKN1B) expression (encoding p27) was associated with cell proliferation-related pathways and epigenetic polycomb repressive complex 2. Using ML, p27 emerged as the second most significant survival factor after N stage, thereby enhancing survival model performance. Additionally, luminal-type breast cancer cell lines with low CDKN1B expression demonstrated increased sensitivity to specific anticancer drugs such as voxtalisib and serdemetan, implying a potential therapeutic synergy between CDKN1B-targeted approaches and these drugs. Conclusion The integration of ML and bioinformatic analyses of p27 has the potential to enhance risk stratification and facilitate personalized treatment strategies for patients with breast cancer.

BACKGROUND: Lymphangioma of the mediastinum is an uncommon benign tumor of lymphatic origin that is most often seen in children, is very rare in adults and is frequently discovered incidentally on chest x-ray exams. While radiology (CT and MRI) may suggest the diagnosis and allow an assessment of the operative difficulties, the histology of the surgical specimen is required for precise diagnosis. Complete resection is the only treatment; however, in some patients resection was incomplete because of the infiltrating character of these tumors, leading to recurrence. We report three cases of mediastinal lymphangioma with a review of the literature.
Adult* ; Child ; Diagnosis ; Humans ; Lymphangioma* ; Mediastinal Neoplasms ; Mediastinum ; Recurrence ; Thorax

Bronchiectasis is defined as an abnormal, irreversible dilatation of the bronchi, which may result from a number of possible causes, and the recognition of these causes may lead to a specific management strategy. Immunodeficiency is known as one of the conditions associated with bronchiectasis. X-linked agammaglobulinemia is a rare inheritable immunodeficiency disorder, caused by a differentiation block, leading almost to the complete absence of B lymphocytes and plasma cells. The affected protein is a cytoplasmic protein tyrosine kinase, Bruton's tyrosine kinase. The early detection and treatment with immunoglobulin replacement are most important for the management of recurrent infections and for reducing severe complications. We report a 20-year-old male patient, with X-linked agammaglobulinemia associated with bilateral bronchiectasis, carrying a missense mutation(R520P) in the BTK gene.
Agammaglobulinemia* ; B-Lymphocytes ; Bronchi ; Bronchiectasis* ; Cytoplasm ; Dilatation ; Humans ; Immunoglobulins ; Korea ; Male ; Plasma Cells ; Protein-Tyrosine Kinases ; Young Adult

BACKGROUND: Corticosteroids in combination with cytotoxic drugs are the mainstays of therapy for idiopathic pulmonary fibrosis (IPF). However, there has been no regimen showing any survival benefit. The aim of this study was to describe a short-term clinical experience on interferon gamma-1b (IFN-gamma1b) therapy for IPF, as an antifibrotic agent. METHODS: Medical records of 27 patients who were treated with IFN-gamma1b (2 million IU, 3 times a week, subcutaneous injection) were retrospectively reviewed. Treatment response was assessed using ATS/ERS criteria in 17 patients who received IFN-gamma1b for more than 6 months. In addition, we compared the efficacy of IFN-gamma1b therapy with that of cyclophosphamide+/-prednisolone therapy (n=26). RESULTS: The median age of IFN-gamma treated group (M:F=19:8) was 59 years (44-74 years). Compared to the patients who showed a stable response at 6 months (n=12), the deteriorated group (n=5) had worse baseline lung function (FVC, 55.4+/-11.3% vs. 70.7+/-10.9%, p=0.019; DLco, 50.3+/-7.3% vs. 76.9+/-19.6%, p=0.014). Lower baseline PaO2 on room air breathing was observed in the deteriorated group (68.6+/-7.8mmHg vs. 91.4+/-6.6mmHg p=0.001). Subcutaneous IFN-gamma1b did not show better efficacy than prednisolone. Five patients discontinued IFN-gamma because of severe side effects. ARDS developed in one patient, who eventually died. CONCLUSION: The administration of IFN-gamma1b is not desirable for patients diagnosed with IPF with poor lung function. Long-term and large-scaled clinical studies are needed for its efficacy in IPF.
Adrenal Cortex Hormones ; Humans ; Idiopathic Pulmonary Fibrosis* ; Interferon-gamma ; Interferons* ; Lung ; Medical Records ; Prednisolone ; Pulmonary Fibrosis ; Respiration ; Retrospective Studies

BACKGROUND: The tuberculin skin test has been used to diagnose latent tuberculosis infection, but is not widely used to diagnose or exclude pulmonary tuberculosis. The objective of this study was to evaluate the diagnostic utility of the tuberculin test in diagnosing and excluding pulmonary tuberculosis, and differentiating pulmonary tuberculosis from nontuberculous mycobacteria (NTM) pulmonary disease, when a sputum acid-fast bacilli (AFB) smear was positive. MATERIAL AND METHODS: From October 2002 to August 2003, among all the inpatients of the Division of Pulmonary and Critical Care Medicine at Samsung Medical Center, 258 patients with clinical suspicion of pulmonary tuberculosis were enrolled and underwent a tuberculin test. RESULTS: 156 males and 102 females were included, with a mean age of 57.5 years. The final diagnoses included lung cancer in 89 cases (34.5%), pulmonary tuberculosis in 59 cases (22.9%), bacterial pneumonia in 33 cases (12.8%) and NTM pulmonary disease in 24 cases (9.3%). The positive tuberculin test rate was higher in the tuberculosis than non-tuberculosis group; 81.4 (48/59) vs. 42.4% (81/199). (p<0.001). In 208 patients with a negative sputum AFB smear, the result of the tuberculin test was positive in 69.4% (25/36) of the tuberculosis group and in 44.8% (77/172) of the non-tuberculosis group (p=0.007), so a positive result of the tuberculin test could predict pulmonary tuberculosis with 69.4% sensitivity, 55.2% specificity, a 24.5% positive predictive value and a 89.6% negative predictive value. In 50 patients with a positive sputum AFB smear, the positive rates of the tuberculin test were 83.9% (26/31) in tuberculosis group and 21.1% (4/19) in NTM pulmonary disease group (p<0.001), so a positive result of the tuberculin skin test could predict pulmonary tuberculosis with 83.9% sensitivity, 78.9% specificity, a 86.7% positive predictive value and a 75.0% negative predictive value. CONCLUSION: The tuberculin test could be useful in excluding pulmonary tuberculosis when the sputum AFB smear is negative, and to differentiate pulmonary tuberculosis from NTM pulmonary disease when the sputum AFB smear is positive.
Adult* ; Critical Care ; Diagnosis ; Female ; Humans ; Inpatients ; Korea ; Latent Tuberculosis ; Lung Diseases ; Lung Neoplasms ; Male ; Nontuberculous Mycobacteria ; Pneumonia, Bacterial ; Skin Tests ; Sputum ; Tuberculin Test* ; Tuberculin* ; Tuberculosis ; Tuberculosis, Pulmonary*