Treacher Collins syndrome is inherited as an autosomal dominant trait with variable penetrance. It shows a marked variability even in the same family. This syndrome is developmental defect affecting the branchial arches. It is not usually associated with acute respiratory distress, but has symptoms of microtia, hypoplastic zygomatic bones, hypoplastic mandibular rami, and bilateral coloboma. It usually requires an emergency operation immediately after the birth. We experienced an infant with Treacher Collins syndrome who showed retrognathia, glossoptosis, microtia, and cleft palate. Intermittent cyanosis, depression of the chest, respiratory difficulty associated with airway obstruction, and swallowing difficulty were also observed. To relieve severe upper airway obstruction caused by retrognathia and glossoptosis, we simultaneously performed tongue-lip adhesion and subperiosteal release of the floor of the mouth. The respiratory and swallowing difficulties were relieved and the tongue repositioned anteriorly. We report the present case with a review of the literature.
Airway Obstruction* ; Branchial Region ; Cleft Palate ; Coloboma ; Cyanosis ; Deglutition ; Depression ; Emergencies ; Humans ; Infant* ; Mandibulofacial Dysostosis* ; Mouth ; Parturition ; Penetrance ; Retrognathia ; Thorax ; Tongue

BACKGROUND: Pulmonary aspergilloma is a potential life-threatening disease resulting from massive hemoptysis. Pulmonary aspergilloma has been treated surgically for many years, however, it has also had higher risk of mortality and complication rate. The purpose of this study is to analyze the operative methods and the types of complications. MATERIAL AND METHOD: Sixty patients who underwent surgical resection for pulmonary their medical reconrds. RESULT: The mean age was 46.3+/-13.4 years(range 20 to 76 years). The most common clinical presentation was hemoptysis which occurred in 48 patients(80%). Pulmonary tuberculosis was the most common pre-existing disease, occurri9ng in 28 patients(46.7%). The other associated lung diseases were bronchiectasis(n=11), silicosis(n=2), and chronic pnumonia(n=1). Operative proceudres wer lobectomy in 35 patients, pneumonectomy in 6, segmentectomy in 5, lobectomy and thoracoplasty in 3, segmentectomy and thoracopasty in 1, and cavernostomy in 10. The operative mortality was 6%(n=3) in lung resection patients but 0% in cavernostomy patients. The most common complications were prolonged air leakage, wound infection and postoperative bleeding. CONCLUSIONS: In most cases of pulmonary aspergilloma surgical resectin remains the only effective therapy. However, cavernostomy may be more effective for pulmonary aspergilloma patients with decreased pulmonary functions and for patients with high risk for lung resection.
Hemoptysis ; Hemorrhage ; Humans ; Lung ; Lung Diseases ; Mastectomy, Segmental ; Mortality ; Pneumonectomy ; Preexisting Condition Coverage ; Pulmonary Aspergillosis ; Thoracoplasty ; Tuberculosis, Pulmonary ; Wound Infection

Traditionally, preeclamptic women who meet established criteria for severe disease aredelivered expeditiously. Although delivery is always appropriate thrapy for the mother, itmay not be for the fetus remote from term. The purpose of this investigation was to evaluatethe differences of pregnancy and neonatal outcome of severe preeclampsia and normalpregnancy, especially before 34 weeks gestational age, and to determine whether aggressiveor expectant management of severe preeclampsia before 34 weeks is more beneficial tomaternal and fetal outcome. A hundred and twenty consecutive pregnancies complicated bysevere preeclampsia and a hundred and twenty normal pregnancies were stuided.The results were as follows:1. The incidence of severe preeclampsia was 3.9%(130 of 3328). The 26~30 year oldage group was most common, 43%(52 patients).2. In case of study between severe preeclamptic patients and normal pregnant patient,there was no differences regarding gestational age at delivery, birth weight, cesarean sectionrate, incidence of RDS, perinatal death. The 1min/5 min Apgar scores of severe preeclampsiais significantly lower than control group(6/7 vs 7/8, p < 0.05). Neonates with SGA(small for gestational age) were found in 29% neonates of patients with severe preeclampsia,which is significantly higher than normal pregnancy group(6%, p < 0.05).3. There was no significant difference between expectant(n=10) and aggressive(n=21)management group less than 34 weeks gestation regarding the gestational age of admissionand delivery, blood pressure, proteinuria, serum creatinine, hemoglobin, hematocrit, pletelets,liver enzymes, and LDH. Only the prolongation of gestational age in the expectant managementgroup is significantly higher than in the aggressive management group(7 vs 2 days,p < 0.05).4. The neonatal and pregnancy outcome of expectant(n=10) and aggressive managementgroup(n=15) were as follow: The birth weight in the expectant management groupis lower than in the aggressive management group(1316.7+/-668.8g vs 1576.2+/-428.7 g, p < 0. 05). The incidence of SGA in the expectant management group is higher than in theaggressive management gorup(50% vs 27%, p=not significant). There was no significant differencebetween expectant and aggressive management group regarding the 1 min/ 5 minApgar score, the cesarean section rate, and the frequency of respiratory distress syndrome,cerebral hemorrhage, renal failure, sepsis, DIC of neonates.
Birth Weight ; Blood Pressure ; Cesarean Section ; Creatinine ; Dacarbazine ; Female ; Fetus ; Gestational Age ; Hematocrit ; Hemorrhage ; Humans ; Incidence ; Infant, Newborn ; Mothers ; Pre-Eclampsia ; Pregnancy ; Pregnancy Outcome ; Proteinuria ; Renal Insufficiency ; Sepsis

A total of 542 children under 10 years of age, admitted to the Seoul National University Children's Hospital, was examined for antibody titers of Toxoplasma gondii using indirect latex agglutination (ILA) test. Among them, 7.7% showed positive titers higher than 1:32, without significant difference between males (7.3%) and females (8.5%). The seropositive rate increased with age although the statistical significance was negligible (0.05 < P < 0.1). By residential areas, the prevalence appeared higher among children from southern provinces (Kyongsang-do and Cholla do) than those from other areas, but the statistical significance was also very low (0.05 < P < 0.1). When the seropositive cases were analyzed by coincidental diseases, the prevalence was significantly higher in patients with congenital diseases than in patients with non-congenital diseases (P < 0.05). The results showed that the seropositive rate of toxoplasmosis in children examined was not high compared with other endemic countries. Some correlations are suggested between toxoplasmosis and congenital anomalies in Korea.
Animal ; Antibodies, Protozoan/blood* ; Child ; Child, Preschool ; Female ; Human ; Infant ; Korea/epidemiology ; Latex Fixation Tests ; Male ; Prevalence ; Seroepidemiologic Studies ; Toxoplasma/immunology* ; Toxoplasmosis/epidemiology*

Angiodysplsia of the small bowel is uncommon, but provably remains frequently undiagnosed. In the small intestine, angiodysplasia presents a taxing surgical problem and is uauslly diagnosed for unexplained gastrointestinal bleeding. For the surgeon, the main technical problem is that even when identified by frequently impalpable, and invisible to the naked eye unless they are actively bleeding at the time of surgery. Ensocopy is often unfruitful because the majority of lesions are submucosal and rarely exceed a few milimeters in diameter. We described a simple intraoperative endoscopic transillumination technique used successfully to identify an angiodysplasia in the jejunum prior to the small bowel resection. This report summarized our experience and review of literature.
Angiodysplasia* ; Hemorrhage ; Intestine, Small ; Jejunum* ; Taxes ; Transillumination*

No abstract available.
Humans ; Survival Rate*

BACKGROUND/AIMS: Protein induced by vitamin K absence or antagonist-II (PIVKA-II), also known as des-carboxyprothrombin (DCP), can be used as an alternative tool to alpha-fetoprotein (AFP) for surveillance of hepatocellular carcinoma (HCC). The aims of the present study were to compare PIVKA-II levels between the patients with HCC and patients with non-HCC chronic liver disease, to evaluate the correlation of PIVKA-II and AFP in HCC patients, and finally to estimate the optimal cut-off value for PIVKA-II for the diagnosis of HCC with using the receiver operating characteristic (ROC) curve. METHODS: A total of 227 consecutive patients with HCC (n=42) or chronic liver disease (n=185) were enrolled in this study. HCC was diagnosed histologically or by imaging such as computed tomography, magnetic resonance imaging or angiography. The serum PIVKA-II and AFP levels were measured by electrochemiluminoimmunoassay with using the Haicatch PIVKA-II kit and by immunoradiometric assay, respectively. RESULTS: The PIVKA-II level in the HCC patients was significantly higher than the non-HCC chronic liver disease patients (903.0+/-1156.7 vs. 111.7+/-211.0 mAU/ mL, respectively, P<0.01). PIVKA-II and AFP showed a statistical correlation in HCC patients (r=0.46, P<0.01). The sensitivity and specificity of PIVKA-II for the diagnosis of HCC were 66.7% and 74.1%, respectively, and when tasted together with AFP, the sensitivity was increased by 85.7%. For the ROC curve of PIVKA-II in HCC patients, the specificity of a 250 mAU/mL level of PIVKA-II was 95%. CONCLUSIONS: PIVKA-II was as useful surveillance tool for differentiating HCC from chronic liver disease, and a PIVKA-II value of 250 mAU/ mL was proposed as a significant cut-off value for diagnosis of hepatocellular carcinoma.
Adult ; Aged ; Biological Markers/*blood ; Carcinoma, Hepatocellular/*diagnosis ; Female ; Humans ; Liver Neoplasms/*diagnosis ; Male ; Middle Aged ; Predictive Value of Tests ; Protein Precursors/*blood ; Prothrombin ; ROC Curve ; Tumor Markers, Biological/*blood ; alpha-Fetoproteins/analysis

PURPOSE: Exercise-induced bronchoconstriction (EIB) in patients with asthma occurs more frequently in winter than in summer. The concentration of house dust mite (HDM) allergens in beds also shows seasonal variation. This study examined the relationship between seasonal differences in the prevalence of EIB and sensitization to HDMs in patients with asthma. METHODS: The medical records of 74 young adult male patients with asthma-like symptoms who underwent bronchial challenge with methacholine, 4.5% saline and exercise, and allergen skin prick tests, were reviewed. The subjects were divided into summer (n=27), spring/fall (n=26) and winter (n=21) groups according to the season during which they underwent testing. RESULTS: The positive responses to exercise differed according to season (48.1% in summer, 73.1% in spring/fall, and 90.5% in winter; P<0.01). In addition, the prevalence of positive responses to HDM (70.4%, 88.5%, and 95.2%, respectively; P<0.05) and pollen skin tests (37.0%, 19.2%, and 0%, respectively; P<0.01) also showed significant seasonal differences. Severe responses to 4.5% saline showed a similar trend, although it was not statistically significant (44.4%, 50.0%, and 71.4%, respectively; P=0.07). Skin test reactivity to HDMs was significantly related to maximal fall in forced expiratory volume in one second (FEV1) following exercise (r=0.302, P<0.01) and the index of airway hyperresponsiveness (AHR) to 4.5% saline (r=-0.232, P<0.05), but not methacholine (r=-0.125, P>0.05). CONCLUSIONS: Positive skin test reactions to HDMs and EIB occurred in winter, spring/fall, and summer in decreasing order of frequency. Seasonal variation in the prevalence of EIB may be related to seasonal variation in sensitization to HDMs, accompanied by differences in indirect, but not direct, AHR.
Allergens ; Asthma ; Asthma, Exercise-Induced ; Bronchoconstriction ; Forced Expiratory Volume ; Humans ; Male ; Medical Records ; Methacholine Chloride ; Pollen ; Prevalence ; Pyroglyphidae ; Seasons ; Skin ; Skin Tests ; Young Adult

BACKGROUND :Prostatic adenocarcinoma makes up about 2% of the total cancer incidence and cancer death in Korean men, but the incidence of this malady is continuously increasing. So far, there have been only a few studies describing the pathologic characteristics of the prostatic adenocarcinoma in Korean patients. In this study, we analyzed 83 radical prostatectomy specimens by using mapping analysis to discover the clinico pathologic characteristics of Korean prostatic adenocarcinoma. METHODS: The resected prostates were serially sectioned and embedded for histologic mapping. The clinico pathologic findings, including the Gleason score, tumor size, prostate intraepithelial neoplasia (PIN) and tumor invasion to the surrounding tissues, were examined. RESULTS: The mean values were as follows: age, 64.1+/-6.6 years; serum prostate specific antigen (sPSA), 16.6+/-16.2 ng/mL; tumor volume, 22.3+/-22.4%; tumor size, 2.2+/-1.2 cm; and Gleason score, 6.9+/-0.9. The rate of high grade PIN was 79.7%. The Gleason score, tumor extent and T stage were statistically correlated (p<0.05). CONCLUSIONS: Some prognostic factors such as sPSA and the Gleason scores showed significantly lower levels compared with those of the previous studies on Korean prostate adenocarcinoma (16-36 ng/mL vs 16.6 ng/mL and 7.3-7.7 vs 6.9, respectively). Although these values are still higher than those of the western studies, this study implies that the early detection of prostate adenocarcinoma is increasing in Korea.
Adenocarcinoma* ; Asian Continental Ancestry Group ; Humans ; Incidence ; Korea ; Male ; Neoplasm Grading ; Prostate* ; Prostate-Specific Antigen ; Prostatectomy ; Tumor Burden

PURPOSE: Optical molecular luminescence imaging is widely used for detection and imaging of bio-photons emitted by luminescent luciferase activation. The measured photons in this method provide the degree of molecular alteration or cell numbers with the advantage of high signal-to-noise ratio. To extract useful information from the measured results, the analysis based on a proper quantification method is necessary. In this research, we propose a quantification method presenting linear response of measured light signal to measurement time. MATERIALS AND METHODS: We detected the luminescence signal by using lab-made optical imaging equipment of animal light imaging system (ALIS) and different two kinds of light sources. One is three bacterial light-emitting sources containing different number of bacteria. The other is three different non-bacterial light sources emitting very weak light. By using the concept of the candela and the flux, we could derive simplified linear quantification formula. After experimentally measuring light intensity, the data was processed with the proposed quantification function. RESULTS: We could obtain linear response of photon counts to measurement time by applying the pre-determined quantification function. The ratio of the re-calculated photon counts and measurement time present a constant value although different light source was applied. CONCLUSION: The quantification function for linear response could be applicable to the standard quantification process. The proposed method could be used for the exact quantitative analysis in various light imaging equipments with presenting linear response behavior of constant light emitting sources to measurement time.
Animals ; Bacteria ; Cell Count ; Enzyme Multiplied Immunoassay Technique ; Imidazoles ; Light ; Luciferases ; Luminescence ; Nitro Compounds ; Optical Imaging ; Photons ; Signal-To-Noise Ratio