No abstract available.
Homocystine ; Homocystinuria*

No abstract available.

No abstract available.
Female* ; Humans ; Neck* ; Urinary Bladder* ; Urinary Incontinence*

BACKGROUND: Several mechanisms have been proposed to account for bone marrow failure in aplastic anemia (AA), including deficiency in hematopoietic stem cells, a secondary stem cell defect involving immune regulation and defective marrow stroma, or microenvironment. We investigated the pathophysiology of AA through long-term bone marrow cultures (LTBMCs) using bone marrrow of AA patients before treatment and of patients responded to immunosuppressive therapy with anti-thymocyte globulin (ATG) and/or cyclosporine. METHODS: We investigated the hematopoietic defect in severe aplastic anemia (SAA) patients by using long-term bone marrow cultures (LTBMCs). Twenty patients with SAA have been studied. In these patients, 10 had been treated with ATG plus cyclosporine and the remainders were studied before therapy was begun. Subsequent assays of the production of negative-acting hematopoietic cytokines (TNF-alpha, IFN-gamma, MIP-1alpha and TGF-beta) by AA stroma in LTBMCs were performed. RESULTS: Initial assessment of CD34+ cells, CFU-GM and CFU-MK from LTBMCs in AA demonstrated severely reduced or absent in patients with SAA, even following hematologic recovery with immunosuppressive therapy,when compared with normal controls. Significant difference in concentrations of TNF-alpha, INF-gamma, and MIP-1alpha between the AA and control groups were apparent. Interestingly, the levels of those negative-acting hematopoietic cytokines were decreased in SAA patients receiving immunosuppressive therapy, but not the levels of controls. However, the mean TGF-beta concentrations in the AA patients and normal controls were not significantly different. The percent of CD34+ cells and CFU-MK in bone marrow was lower in SAA patients before immunosuppressive therapy was begun than that in SAA patients receiving immunosuppressive therapy and that in normal controls (mean 0.54+/-0.32% vs 0.96+/-0.32% vs 1.94+/-0.61%). CONCLUSIONS: These results indicate the presence of a in vitro functional deficiency in the hematopoietic system of patients with AA, including those that achieved partial or complete remission after immunosuppressive treatment, and add to the available evidence for defect of microenvironment with hematopoeitic stem cell in some cases of AA.
Anemia, Aplastic* ; Antilymphocyte Serum ; Bone Marrow* ; Chemokine CCL3 ; Cyclosporine ; Cytokines ; Granulocyte-Macrophage Progenitor Cells ; Hematopoietic Stem Cells ; Hematopoietic System ; Humans ; Stem Cells ; Transforming Growth Factor beta ; Tumor Necrosis Factor-alpha

PURPOSE: Acute lower respiratory infection (ALRI), which is frequently encountered in pediatric patients, is the leading cause of hospitalization. We aimed to identify particular cytokines that correlated with ALRI clinical characteristics. We also aimed to identify any differences in cytokines between respiratory syncytial virus (RSV)-related ALRI and non-RSV-related ALRI. METHODS: Cytokine levels were measured in the sera sampled from 103 pediatric patients diagnosed with ALRI and admitted to Seoul St. Mary's Hospital between May 2012 and April 2013. The correlations between cytokine levels and the length of hospitalization, the number of days with fever, body temperature, pulse rate, respiration rate, oxygen saturation upon admission, and duration of oxygen supplementation were analyzed. RESULTS: In children with ALRI, the level of interleukin (IL)-6, granulocyte-colony stimulating factor (G-CSF), and IL-10 were correlated with a higher body temperature on admission. In addition, the IL-8 level was correlated with pulse rate and respiration rate, and IL-1β level was related with oxygen saturation on admission. In children with RSV-related ALRI, the IL-6 was correlated the with duration of fever, and the IL-1β, IL-2, and IL-8 levels were related to pulse rate and respiration rate. In addition, the increase in interferon-gamma-inducible protein-10 (IP-10) level was correlated with a higher body temperature on admission and a longer duration of hospitalization in children with RSV-related ALRI. CONCLUSION: In children with ALRI, the levels of IL-6, IL-8, IL-1β, G-CSF, and IP-10 were correlated with its clinical features. In children with RSV-related ALRI, the IL-1β, IL-2, IL-6, IL-8, and IP-10 level was correlated with the severity of the disease.
Body Temperature ; Child* ; Cytokines* ; Fever ; Granulocyte Colony-Stimulating Factor ; Heart Rate ; Hospitalization ; Humans ; Interleukin-10 ; Interleukin-2 ; Interleukin-6 ; Interleukin-8 ; Interleukins ; Oxygen ; Respiratory Rate ; Respiratory Syncytial Viruses ; Seoul

PURPOSE: To assess the usefulness of flexible bronchoscopy in patients with suspected pulmonary tuberculosis (PTB) who have difficulty in sputum expectoration. METHODS: The subjects of this study were patients who were suspected of PTB and visited the Division of Pediatric Pulmonology at a tertiary hospital from April 2006 to March 2016. PTB suspects were determined by clinical symptoms, radiologic findings, and immunologic studies. We aimed to examine the value and safety of bronchoscopy in diagnosis and differential diagnosis of PTB in PTB-suspected patients. The diagnostic criteria for PTB were defined when Mycobacterium tuberculosis was cultured in the sputum specimen or in the bronchial washing fluid. RESULTS: A total of 19 PTB suspects were included. One patient was diagnosed with PTB by using the sputum study. However, the remaining 18 patients could not expectorate sputum or showed no evidence of Mycobacterium tuberculosis infection from the sputum study. Of the 18 patients, 15 underwent bronchoscopy. After bronchoscopy, 6 patients were diagnosed with PTB and 9 patients were diagnosed with Mycoplasma, viral, or fungal pneumonia, and tumors. For antituberculous drug resistance, there were 1 case of isoniazid (INH) resistance and 1 case of concurrent resistance to INH and prothionamide. There was no multidrug-resistant tuberculosis. None of the patients had significant complications due to bronchoscopy. CONCLUSION: Flexible bronchoscopy appears to be a definitive and safe procedure for the differential diagnosis of patients suspecting PTB in children who have difficulty expectorating sputum.
Bronchoscopy* ; Child* ; Diagnosis ; Diagnosis, Differential ; Diagnostic Imaging ; Drug Resistance ; Humans ; Isoniazid ; Mycobacterium tuberculosis ; Mycoplasma ; Pneumonia ; Prothionamide ; Pulmonary Medicine ; Sputum* ; Tertiary Care Centers ; Tuberculosis, Multidrug-Resistant ; Tuberculosis, Pulmonary*

PURPOSE: To identify the correlation between nasal eosinophilia and aeroallergen sensitization in children and adolescents. METHODS: This is a retrospective study of patients below 18 years of age who had a history of rhinitis that lasted more than 2 weeks or had been repeated more than once a year, received nasal eosinophil examinations, and had serum specific IgE to aeroallergens measured at an Allergy Clinic in a single tertiary teaching hospital in Seoul, Korea. The percentage of nasal eosinophils was calculated by the number of eosinophils per total leukocytes in a high-power field of 1,000×. Data was analyzed to determine the association between nasal eosinophilia and 18 aeroallergens. RESULTS: Of the 245 patients included, 156 (63.7%) were male and the mean age (±standard deviation) was 7.9 years (±3.8). In total, 175 patients (71.4%) were sensitized to at least 1 of the 18 aeroallergens tested, and sensitization to house dust mite was most common. In addition, 118 (48.2%) and 69 patients (28.2%) had nasal eosinophilia of at least 1% and 5%, respectively. There were no significant correlations between serum total IgE or age and the percentage of nasal eosinophils. However, the percentage of nasal eosinophils in the group sensitized to any aeroallergens was significantly increased compared to the nonsensitized group (P=0.002). The percentage of nasal eosinophils was significantly higher in patients who were sensitized to Birch-Alder Mix, oak white, Bermuda grass, orchard grass, timothy grass, sweet vernal grass, rye, mugwort, short ragweed, Alternaria alternata, cats, dogs or Dermatophagoides farinae compared to those nonsensitized. CONCLUSION: Nasal eosinophilia was significantly associated with sensitization to aeroallergens.
Adolescent* ; Alternaria ; Ambrosia ; Animals ; Artemisia ; Cats ; Child* ; Cynodon ; Dactylis ; Dermatophagoides farinae ; Dogs ; Eosinophilia* ; Eosinophils ; Hospitals, Teaching ; Humans ; Hypersensitivity ; Immunoglobulin E ; Korea ; Leukocytes ; Lolium ; Male ; Phleum ; Pyroglyphidae ; Retrospective Studies ; Rhinitis* ; Seoul

We experienced a case of atypical hairy cell leukemia in a 42-year-old woman. She showed marked splenomegaly without palpable lymphadenopathy. Complete blood cell count revealed leukocytosis at 44,000/micro L with lymphocytes 74% and peripheral blood smear showed abnormal lymphoid cells with cytoplasmic projections. The bone marrow was easily aspirated and also revealed the abnormal lymphocytes in up to 95%. Tartrate-resistant acid phosphatase reactivity was negative in the hairy cells. Immunophenotyping results of lymphoid cells were CD5(-), CD7(-), CD10(-), CD19(+), and HLA-DR(+). She was treated with an adenosine analogue, fludarabine at a daily dose of 30mg/m2 for 5 consecutive days, every four weeks. Immediately after treatment, the size of the spleen was normalized. Correct diagnosis was difficult due to insufficient laboratory and pathologic data. The differential diagnosis of mature B-cell neoplasms with cytoplasmic projections in patients with splenomegaly includes hairy cell leukemia and splenic lymphoma with villous lymphocytes. We herein described the present case with a brief review of the literature.
Acid Phosphatase ; Adenosine ; Adult ; B-Lymphocytes ; Blood Cell Count ; Bone Marrow ; Cytoplasm ; Diagnosis ; Diagnosis, Differential ; Female ; Humans ; Immunophenotyping ; Leukemia, Hairy Cell* ; Leukocytosis ; Lymphatic Diseases ; Lymphocytes ; Lymphoma ; Spleen ; Splenomegaly

PURPOSE: The environmental factors human rhinoviruses (HRVs) and house dust mites (HDMs) are the most common causes of acute exacerbations of asthma. The aim of this study was to compare the chemokine production induced by HRVs in airway epithelial cells with that induced by other respiratory viruses, and to investigate synergistic interactions between HRVs and HDMs on the induction of inflammatory chemokines in vitro. METHODS: A549 human airway epithelial cells were infected with either rhinovirus serotype 7, respiratory syncytial virus (RSV)-A2 strain, or adenovirus serotype 3 and analyzed for interleukin (IL)-8 and regulated on activation, normal T-cell expressed and secreted (RANTES) release and mRNA expression. Additionally, activation of nuclear factor (NF)-kappaB and activator protein (AP)-1 were evaluated. The release of IL-8 and RANTES was also measured in cells stimulated simultaneously with a virus and the HDM allergen, Der f1. RESULTS: HRV caused greater IL-8 and RANTES release and mRNA expression compared with either RSV or adenovirus. NF-kappaB and AP-1 were activated in these processes. Cells incubated with a virus and Der f1 showed an increased IL-8 release. However, compared with cells incubated with virus alone as the stimulator, only HRV with Der f1 showed a statistically significant increase. CONCLUSIONS: IL-8 and RANTES were induced to a greater extent by HRV compared with other viruses, and only HRV with Der f1 acted synergistically to induce bronchial epithelial IL-8 release. These findings may correspond with the fact that rhinoviruses are identified more frequently than other viruses in cases of acute exacerbation of asthma.
Adenoviridae ; Antigens, Dermatophagoides ; Arthropod Proteins ; Asthma ; Chemokine CCL5 ; Chemokines ; Cysteine Endopeptidases ; Epithelial Cells ; Humans ; Interleukin-8 ; Interleukins ; NF-kappa B ; Pyroglyphidae ; Respiratory Syncytial Viruses ; Rhinovirus ; RNA, Messenger ; Sprains and Strains ; T-Lymphocytes ; Transcription Factor AP-1 ; Viruses

OBJECTIVES: To examine the effects of the oligohydramnios on perinatal outcome and latency period in patients with preterm premature rupture of membranes. METHODS: We performed a retrospective analysis of 98 singleton pregnancies complicated by preterm premature rupture of membranes, with delivery between 26 and 35 weeks' gestation. Amniotic fluid index was determined using transabdominal ultrasound at admission. All medical records of mothers and neonates were reviewed. Oligohydramnios was defined as amniotic fluid index less or equal to 5.0 cm and latency period was defined as time interval from membrane rupture to delivery. Chi-spuare test, Fisher's exact test, Student-t test, Mann-Whitney U test were used for statistical analysis. RESULTS: 1) Of the 98 patients, 59 patients(60%) were oligohydramnios group(AFI< or =5.0) and 39 patients(40%) were non-oligohydramnios group(AFI>5.0). Both groups were similar with respect to selected dermographics, gestational age at rupture of the membranes, chorioamnionitis, 1 min Apgar score and 5 min Apgar score. Patients with oligohydramnios demonstrated a lower gestatoinal age at birth and lower birth weight. There were no statistically significant correlations in neonatal morbidity and perinatal mortality between both groups. 2) For comparing latency period, we excluded deliveries of Cesarean section or induction. Comparing the remained group(25 patients), median of latency period in oligohydramnios group were 41.5 hours and median of latency period in non-oligohydramnios group were 44 hours. There were no statistically significant correlations in oligohydramnios and latency period. CONCLUSION: There were no significant effects of the oligohydramnios on poor perinatal outcome and latency period in patients with preterm premature rupture of membranes
Amniotic Fluid ; Apgar Score ; Birth Weight ; Cesarean Section ; Chorioamnionitis ; Female ; Gestational Age ; Humans ; Infant, Newborn ; Latency Period (Psychology)* ; Medical Records ; Membranes* ; Mothers ; Oligohydramnios* ; Parturition ; Perinatal Mortality ; Pregnancy ; Retrospective Studies ; Rupture* ; Ultrasonography