Mucopolysaccharidoses (MPSs) are a group of inherited heterogenous metabolic disorders, caused by deficiency of an enzyme involved in the degradation of mucopolysaccharides, and thus deposition of mucopolysaccharides in all connective tissue. MPS type III is heparan sulphate storage disorder, especially marked central nervous system involvement. As with all the MPS disorders, this patient with MPS III presents coarse outlook features, such as macrocephaly, macroglossia, prominent mandible, short neck, and unstable atlantoaxial joints, as well as tracheal abnormality. These clinical findings give significant challenges to the anesthesiologist for adequate airway management and tracheal intubation. Recently, a number of new devices have been developed to facilitate intubation. We report an experience of facilitated tracheal intubation using video styletscope (OptiScope PM201(R), Pacific Medical, Seoul, Korea) in a 16 year old male patient with MPS III, who was undergoing inguinal hernia repair.
Airway Management ; Atlanto-Axial Joint ; Central Nervous System ; Connective Tissue ; Glycosaminoglycans ; Hernia, Inguinal ; Humans ; Intubation ; Intubation, Intratracheal ; Macrocephaly ; Macroglossia ; Male ; Mandible ; Mucopolysaccharidoses ; Neck

BACKGROUND: The hemodynamic reactions to psychological stress were different between adult genders. Our aim was to investigate the hypothesis that preoperative cardiovascular responses in children undergoing ear reconstruction surgeries will be different according to gender. METHODS: We obtained data from children, aged 7-16 years, undergoing reconstruction surgeries for microtia during 2005-2009. Number of boys receiving primarily rib cartilage graft (RCG) surgery and secondarily ear elevation procedure was 121 and 83, respectively, and girls 48 and 28, respectively. Preoperative systolic (S), diastolic blood pressure (DBP) and heart rate (HR) on each surgery were compared between genders, and within genders. RESULTS: Boys were younger than girls by one year at first and second admission. Boys showed significantly lower preoperative DBP than girls (mean difference [MD]: -3.7 mmHg, 95%CI: -6.1 to -1.3, P = 0.032) before RCG, whereas significantly higher SBP (MD: 21.5 mmHg, 95%CI: 16.6 to 26.4, P < 0.001), and DBP (MD: 10.2 mmHg, 95%CI: 6.0 to 14.4, P < 0.001) before ear elevation. Comparison between the two surgeries within the same gender indicated that boys demonstrated significantly higher SBP (P < 0.001) and DBP (P < 0.001) before ear elevation than before RCG; however SBP was comparable and DBP was significantly lower (P = 0.035) before ear elevation than before RCG in girls. CONCLUSIONS: Gender impact on the preoperative cardiovascular reactions was evident in children undergoing microtia surgery.
Adult ; Aged ; Blood Pressure ; Cartilage ; Child ; Congenital Abnormalities ; Ear ; Heart Rate ; Hemodynamics ; Humans ; Ribs ; Stress, Psychological ; Transplants

Essential thrombocythemia (ET) is characterized by most cases in which platelet counts exceed 1 million/µL. ET is usually no symptoms during non-pregnancy, but arterial and venous thrombosis and hemorrhage may develop in pregnancy. Pregnancy in these patients is associated with many complications in both pregnant women and fetuses such as recurrent abortion, intrauterine fetal growth restriction, preterm delivery, preeclampsia, and stillbirth. In these patients, aspirin, low-molecular-weight heparin (LMWH), and interferon alpha (INF-α) are recommended during pregnancy. We report a case of four consecutive abortions despite being treated with INF-α, low dose aspirin, and LMWH in patient with ET.
Abortion, Habitual* ; Aspirin ; Female ; Fetal Development ; Fetus ; Hemorrhage ; Heparin, Low-Molecular-Weight ; Humans ; Interferon-alpha ; Platelet Count ; Pre-Eclampsia ; Pregnancy ; Pregnant Women ; Stillbirth ; Thrombocythemia, Essential* ; Venous Thrombosis

BACKGROUND: Doppler myocardial performance index (DMPI), defined as the sum of isovolumetric contraction time (IVCT) and isovolumetric relaxation time (IVRT) divided by ejection time (ET), is an easily measurable index which has been shown to reflect the severity of the disease. It has been known each component of DMPI, as IVCT, IVRT and ET, was affected by the change of preload. Therefore, the objective of this study was to estimate the changes of DMPI during intravascular volume reduction in patients with end-stage renal disease and to determine which components of DMPI contribute to DMPI alteration during intravacular volume reduction. METHODS: We measured blood pressure, heart rate, M-mode echocardiographic and Doppler parameters within 10 minutes before and after hemodialysis and ultrafitration with amount of average 2 L. RESULTS: We studied 40 end-stage renal disease patients (22 men and 18 women, mean age of 52 years) who had left ventricular hypertrophy 39 (97.5%) and normal left ventricular systolic function (diastolic interventricular septal thickness, 13.8+/-2 mm; diastolic left ventricular posterior wall thickness, 12.6+/-2 mm; Ejection fraction, 63.1+/-0.1%). Peak E-wave velocity was significantly decreased after hemodialysis and ultrafiltration (84.85+/-25 cm/s vs 72.89+/-23 cm/s, p<0.05), but other Doppler parameters such as peak A-wave velocity and E deceleration time were not changed. E/A ratio showed decreased tendency which was not significant statistically (p<0.097). DMPI was significantly increased after hemodialysis and ultrafiltration (0.41+/-0.14 vs 0.45+/-0.15, p<0.001). The increase of DMPI was mainly affected by prolongation of IVRT/ET which was due to prolonged IVRT, but IVCT/ET was not changed. The changes of DMPI was little with hemodialysis and ultrafiltration of about 2 L. CONCLUSION: We could prove that DMPI was preload dependent parameter of myocardial function. We suggest the change of preload should be considered as an important factor which may alter the DMPI.
Blood Pressure ; Deceleration ; Echocardiography ; Female ; Heart Rate ; Humans ; Hypertrophy, Left Ventricular ; Kidney Failure, Chronic* ; Male ; Relaxation ; Renal Dialysis* ; Ultrafiltration* ; Ventricular Function, Left*

Purpose: Preoperative tumor size is associated with clinical stage, treatment plan and even survival rate of patient. We investigated the accuracy of tumor size estimation between magnetic resonance imaging (MRI) and ultrasonography (US) findings, comparing these with pathologic tumor size in the diagnosis of early breast carcinoma. Methods: Between 2011 and 2016, 136 patients with early breast cancer were analyzed and their tumor size on US and MRI findings were compared with their pathologic tumor size retrospectively. The background parenchymal enhancement of MRI was categorized as minimal, mild, moderate, and extreme. The patients who underwent neoadjuvant chemotherapy, had positive resection margins, underwent excisional biopsy for cancer diagnosis, and had non-mass lesions on MRI scans, were excluded. Results: In all, 83.1% of the cases showed concordance between MRI findings and pathologic tumor size within 0.5cm. MRI overestimated the findings by 10.3% and underestimated them by 6.6%; 78.7% showed concordance between US findings and pathologic tumor size within 0.5cm. US overestimated the findings by 5.9% and underestimated them by 15.4%. The tumor size on MRI (r=0.87) showed a stronger correlation to the pathologic tumor size than that on US (r=0.64) in early breast cancer patients. US had a tendency to underestimate the tumor size. The degree of breast parenchyma did not affect the accuracy of the measurement of preoperative tumor size. Conclusion MRI is relatively more accurate than US for assessing preoperative tumor size in breast cancer patients. US tends to underestimate tumor size.

PURPOSE: To investigate the effects of mitomycin C on the scleral collagen surfaces using atomic force microscopy (AFM). METHODS: Two non-contact mode AFM machines were used to observe changes in the morphological characteristics of human scleral surfaces before and after one, three, and five minutes of 0.02% mitomycin C application. Based on AFM topography and deflection images of the collagen fibril, the morphological characteristics of scleral fibrils including the fibril diameter and D-period were measured using the line profile. RESULTS: The sclera collagen fibril treated with 0.02% mitomycin C for one minute did not show any significant increases in mean fibril diameter (155.04 +/- 17.46 nm) or mean D-periodicity (70.02 +/- 3.33 nm), compared to those of the control group. However, the scleral collagen fibrils treated with 0.02% mitomycin C for three and five minutes showed significant increases in mean fibril diameter (182.33 +/- 16.33 nm, 199.20 +/- 12.40 nm, respectively) and mean D-periodicity (70.27 +/- 13.66 nm, 72.75 +/- 19.32 nm, respectively), compared to those of the control group. CONCLUSIONS: The present study examined the structural changes in the scleral collagen fibrils before and after mitomycin C application according to atomic force microscopy. The results indirectly suggest that three or more minutes of 0.02% mitomycin C application affects the morphology of scleral collagen.
Collagen ; Humans ; Microscopy, Atomic Force ; Mitomycin ; Sclera

We report a case of 51-year-old woman with pure unroofed coronary sinus without persistent left superior vena cava and other cardiac anomaly. She presented with dyspnea on exertion during several years. Her chest film showed prominent cardiomegaly and dilated hilar vessels. Cardiac rhythm was atrial fibrillation. Transthoracic echocardiography demonstrated the enlarged coronary sinus with defect toward left atrium on parasternal long axis view and significant flow from coronary sinus into right atrium on subxyphoid view, and its other findings were dilated right ventricle and right atrium, paradoxical septal motion, moderate tricuspid regurgitation and mild mitral regurgitation, which were mimicking of large secundum atrial septal defect. Radionuclide cardioangiography and cardiac catheterization showed the existence of significant shunt. There was no evidence of persistent left superior vena cava on chest CT. Closure of Coronary sinus opening was done. Thereafter her symptoms of congestive heart failure were much improved. We think that careful examination of 2-D echocardiography can be valuable tool for diagnosis of unroofed coronary sinus in adult patient.
Adult ; Atrial Fibrillation ; Axis, Cervical Vertebra ; Cardiac Catheterization ; Cardiac Catheters ; Cardiomegaly ; Coronary Sinus* ; Diagnosis ; Dyspnea ; Echocardiography ; Female ; Heart Atria ; Heart Failure ; Heart Septal Defects, Atrial ; Heart Ventricles ; Humans ; Middle Aged ; Mitral Valve Insufficiency ; Thorax ; Tomography, X-Ray Computed ; Tricuspid Valve Insufficiency ; Vena Cava, Superior*

OBJECTIVES: This study investigated the effects of chronic medical diseases on depressive symptoms in individuals at high risk for depression living in rural areas, over a 1-year period.METHODS: A community-based longitudinal study was conducted; 67 participants aged 18–79 years residing in rural areas were included. In the first survey, all participants completed a self-report questionnaire battery. An interview was also conducted to obtain data on demographic variables and current or past chronic medical diseases. In the first survey, participants with the Center for Epidemiologic Studies Depression scale(CES-D) scores of 16 or higher were categorized as being at high risk for depression; the same assessments were carried out 1 year later in a follow-up survey. Multiple regression analysis was performed to determine the association of chronic medical diseases with 1-year follow-up depressive symptoms in the high-risk group.RESULTS: In model 1, which controlled for sociodemographic variables, the number of chronic medical diseases (p =0.026), baseline severity of depressive symptoms(p =0.002), and presence of diabetes(p =0.039) were significantly associated with the follow-up CES-D scores. In model 2, which further adjusted for Alcohol Use Disorder Identification Test and Beck Anxiety Inventory scores, the number of chronic medical diseases(p =0.036), baseline severity of depressive symptoms(p =0.017), and prevalence of diabetes(p =0.037) were also significantly associated with the follow-up CES-D scores.CONCLUSION: This study suggests that the number of chronic medical diseases, prevalence of diabetes, and severity of depressive symptoms are significantly associated with 1-year follow-up depressive symptoms in individuals at high risk for depression.
Anxiety ; Depression ; Diabetes Mellitus ; Epidemiologic Studies ; Follow-Up Studies ; Longitudinal Studies ; Prevalence ; Rural Population

The first edition of the ‘Standardized Pathology Report for Colorectal Cancer,’ which was developed by the Gastrointestinal Pathology Study Group (GIP) of the Korean Society of Pathologists, was published 13 years ago. Meanwhile, there have been many changes in the pathologic diagnosis of colorectal cancer (CRC), pathologic findings included in the pathology report, and immunohistochemical and molecular pathology required for the diagnosis and treatment of colorectal cancer. In order to reflect these changes, we (GIP) decided to make the second edition of the report. The purpose of this standardized pathology report is to provide a practical protocol for Korean pathologists, which could help diagnose and treat CRC patients. This report consists of “standard data elements” and “conditional data elements.” Basic pathologic findings and parts necessary for prognostication of CRC patients are classified as “standard data elements,” while other prognostic factors and factors related to adjuvant therapy are classified as “conditional data elements” so that each institution could select the contents according to the characteristics of the institution. The Korean version is also provided separately so that Korean pathologists can easily understand and use this report. We hope that this report will be helpful in the daily practice of CRC diagnosis.