Cerebrocostomandibular syndrome is characterized by micrognathia, cleft palate, multiple thorax deformity and frequently, mental deficiency. Respiratory compromise is a common cause of death. We experienced a case of cerebrocostomandibular syndrome with congenital heart disease in a 2 day old female baby with the chief complaint of cyanosis and respiratory difficulty since birth. She was delivered by cesarian section due to delayed labor at IUP 42 weeks. The diagnosis was made on the basis of clinical features, radiologic findings and echocardiogram, which showed micrognathia, deformity of 3rd finger Lt, hemivertebrae of T1-T6 Lt., absence of 1-6th rib Lt., dextrocardia, tetralogy of fallot, atrial septal defect secondum. We reported this case and reviewed related litertures briefly.
Cause of Death ; Cleft Palate ; Congenital Abnormalities ; Cyanosis ; Dextrocardia ; Diagnosis ; Female ; Fingers ; Heart Defects, Congenital* ; Heart Septal Defects, Atrial ; Humans ; Intellectual Disability ; Parturition ; Ribs ; Tetralogy of Fallot ; Thorax

OBJECTIVES: The Korean version of Bowel disease questionnaire (BDQ-K) was developed to evaluate the symptom items required to meet the definition of functional gastrointestinal disorders (FGIDs). We evaluated the test-retest reliability and validity of the self-reported BDQ-K and prevalence of functional dyspepsia (FD) and irritable bowel syndrome (IBS) according to the Rome-III criteria. METHODS: Sixty-nine patients participated in the test-retest reliability study, with a two week interval, and another 74 patients were enrolled to assess the self-reported questionnaire versus a doctor's interview (concurrent validity). A total of 3,325 subjects (mean age, 44+/-9 yrs; 58.3% male) presenting for an upper endoscopy responded to the BDQ-K at a health promotion center, but 797 subjects with organic diseases were excluded. RESULTS: In the validity study of the BDQ-K, the median kappa value was 0.74 (0.36~1.0). The median kappa value for the test-retest was 0.56 (range 0.22~1.0), including abdominal pain (kappa=0.51, P<0.001), pain onset before 6 months (kappa=0.51, P<0.001), epigastric pain (kappa=0.69, P<0.001), early satiety (kappa=0.40, P<0.001), and postprandial fullness (kappa=0.34, P<0.001). The prevalence of FD was 8.3% (209/2,528); epigastric pain more than once a week 4.4%, early satiety 2.5%, and postprandial fullness 6.1%. FD was more prevalent in women (P=0.001). The prevalence of IBS was 6.1% and IBS also predominated in women (7.1% vs 5.1% in men, P=0.032). CONCLUSION: The BDQ-K is a reliable and valid instrument for identifying FGIDs. The prevalence of FD according to the Rome III criteria was 8.3% and that of IBS was 6.1%.
Abdominal Pain ; Dyspepsia ; Endoscopy ; Female ; Gastrointestinal Diseases ; Health Promotion ; Humans ; Irritable Bowel Syndrome ; Male ; Prevalence ; Reproducibility of Results ; Rome ; Surveys and Questionnaires

OBJECTIVE: This study was designed to investigate the efficacy in the combination of haloperidol and clonidine with chronic schizophrenia. Moreover, we evaluated the effects of clonidine to the plasma haolperidol and reduced haloperidol concentration 36 patiens were entered on 4 weeks by the double blind trial. METHOD: The patients (male=11, female=25) were randomly divided into two groups of eighteen patients each. Their antipsychotic medication was fixed for 2 weeks before double blind trial with haloperidol and clonidine or placebo. Clonidine was started at 0.15 mg/day and then raised 0.6mg/day during 4 days, and the placebo was applied by same method as clonidine. The clinical assessment of the patients was assessed with BPRS and NOSIE. The plasma concentrations of haloperidol and reduced haloperidol were measured by HPLC (UV detector). RESULTS: 1) There were no statistical differences in sex distribution, age, duration of illness, dosages of haloperidol, plasma concentration of haloperidol and reduced haloperidol of each groups before clonidine or placebo trial. 2) On total and subcluster scores of BPRS and total score of NOSIE, there were decreased tendencies over the time, but there was no statistical significance in both groups. 3) Plasma concentrations of haloperidol and reduced haloperidol after 4 weeks of clonidine trial were revealed statistically significant increasement as compared to baseline. CONCLUSION: The combination of haloperidol and clonidine for 4 weeks did not reveal the improvement. In our opinions the reasons of those results were that our samples were not made of paranoid type of schizophrenia, the duration of clonidine trial was not good enough. So we should suggest that more specifying design will be needed.
Chromatography, High Pressure Liquid ; Clonidine* ; Haloperidol* ; Humans ; Norepinephrine ; Plasma ; Schizophrenia* ; Sex Distribution

No abstract available.
Pregnancy, Heterotopic*

The lymphatic vasculature has been regarded as a passive conduit for interstitial fluid and responsible for the absorption of macromolecules such as proteins or lipids and transport of nutrients from food. However, emerging data show that the lymphatic vasculature system plays an important role in immune modulation. One of its major roles is to coordinate antigen transport and immune-cell trafficking from peripheral tissues to secondary lymphoid organs, lymph nodes. This perspective was recently updated with the notion that the interaction between lymphatic endothelial cells and leukocytes controls the immune-cell migration and immune responses by regulating lymphatic flow and various secreted molecules such as chemokines and cytokines. In this review, we introduce the lymphatic vasculature networks and genetic transgenic models for research on the lymphatic vasculature system. Next, we discuss the contribution of lymphatic endothelial cells to the control of immune-cell trafficking and to maintenance of peripheral tolerance. Finally, the physiological roles and features of the lymphatic vasculature system are further discussed regarding inflammation-induced lymphangiogenesis in a pathological condition, especially in mucosal tissues such as the gastrointestinal tract and respiratory tract.
Absorption ; Chemokines ; Cytokines ; Endothelial Cells ; Endothelium ; Extracellular Fluid ; Gastrointestinal Tract ; Leukocytes ; Lymph Nodes ; Lymphangiogenesis ; Mucous Membrane ; Peripheral Tolerance ; Respiratory System

BACKGROUND: The aim of the study is to evaluate the difference of immunophenotypes of stromal cells between the pure phyllodes tumor (PT) type and the co-existent type of phyllodes tumor and fibroadenoma, and between benign and malignant PT. METHODS: Immunohistochemical staining for actin, CD34, CD10, c-kit, bcl-2, p53 and MIB-1 was performed using tissue microarray blocks that contained 25 cases of pure PT (16 benign tumors, 4, borderline malignant tumors, and 5 malignant tumors) and 6 cases of co-existent type. RESULTS: The expression rates of CD34 and MIB-1 in the pure PT type were significantly higher and the expression rate of actin in the pure PT was significantly lower than that of the co-existent type. However, there were no significant differences in the bcl-2, CD10, and p53 expressions between the pure PT type and the co-existent type, and no c-kit expression was observed in both types. No significant differences in the CD34, actin, bcl-2, CD10, and p53 expressions between the benign and borderline/malignant PT cases were found. However, a significant difference of the MIB-1 expression rate was noted. CONCLUSIONS: The stromal cells of the pure PT type are regarded as less mature myofibroblasts, and the CD10 reactivity in some phyllodes tumors suggests a myoepithelial origin. The MIB-1 labeling index would be useful for the grading of phyllodes tumor.

The incidence and mortality of colorectal cancer (CRC) have decreased through regular screening colonoscopy, surveillance, and endoscopic treatment. However, CRC can still be diagnosed after negative colonoscopy. Such CRC is called interval CRC and accounts for 1.8–9.0% of all CRC cases. Most cases of interval CRC originate from missed lesions and incompletely resected lesions. Interval CRC can be minimized by improving the quality of colonoscopy. This has led to a growing interest in and demand for high-quality colonoscopy. It is important to reduce the risk of CRC and its associated mortality by improving the quality of colonoscopy. In this review article, we provide an overview of colonoscopy quality indicators, including bowel preparation adequacy, the cecal intubation rate, the adenoma detection rate, the colonoscopy withdrawal time, appropriate polypectomy, and complication of the procedure. Because colonoscopy is a highly endoscopist-dependent procedure, colonoscopists should be well-acquainted with quality indicators and strive to apply them in daily clinical practice for the prevention of CRC.

In the Republic of Korea, relevant documents are submitted to forensic doctors or agencies when courts grant confiscation warrants for autopsy. If the essential data on unusual death are not submitted at the time of the autopsy, it may be difficult to properly understand the situation relating to an unusual death prior to the autopsy, thus reducing the accuracy of the autopsy. As many as 6,133 out of 6,610 autopsy data (92.8%) in the Republic of Korea in 2015 were analyzed. Most autopsy appraisal requests (99.8%) were submitted. Unusual death occurrence reports (86.0%) and command recommendations of unusual death (70.3%) were submitted in many cases. However, prosecutor commands on unusual death were submitted only in 27.8% cases, and confiscation warrants were not submitted in 7.4% cases. As for postmortem inspection and death scene investigation reports, 29.3% and 34.1% cases were submitted, respectively. In addition to the above two documents, death certificates and records of statement of a relative had significant regional variations (0.3%–80.1%, 3.1%–64.7%, 27.8%–81.3%, and 40.8%–96.8%, respectively). For postmortem inspection and death scene photos, 2.7% and 3.2% were submitted in black-and-white photographs, respectively. The authors propose a list of forensic autopsy requests including autopsy appraisal requests, unusual death occurrence reports, command recommendations of unusual deaths, prosecutor commands on unusual death, and confiscation warrants unconditionally, as an essential document reflecting the progress of investigations. We suggest that postmortem inspection reports and photos, death scene investigation reports and photos, and death certificates should be included as part of postmortem investigation data.
Autopsy ; Data Interpretation, Statistical ; Death Certificates ; Financing, Organized ; Korea ; Republic of Korea

In the Republic of Korea, relevant documents are submitted to forensic doctors or agencies when courts grant confiscation warrants for autopsy. If the essential data on unusual death are not submitted at the time of the autopsy, it may be difficult to properly understand the situation relating to an unusual death prior to the autopsy, thus reducing the accuracy of the autopsy. As many as 6,133 out of 6,610 autopsy data (92.8%) in the Republic of Korea in 2015 were analyzed. Most autopsy appraisal requests (99.8%) were submitted. Unusual death occurrence reports (86.0%) and command recommendations of unusual death (70.3%) were submitted in many cases. However, prosecutor commands on unusual death were submitted only in 27.8% cases, and confiscation warrants were not submitted in 7.4% cases. As for postmortem inspection and death scene investigation reports, 29.3% and 34.1% cases were submitted, respectively. In addition to the above two documents, death certificates and records of statement of a relative had significant regional variations (0.3%–80.1%, 3.1%–64.7%, 27.8%–81.3%, and 40.8%–96.8%, respectively). For postmortem inspection and death scene photos, 2.7% and 3.2% were submitted in black-and-white photographs, respectively. The authors propose a list of forensic autopsy requests including autopsy appraisal requests, unusual death occurrence reports, command recommendations of unusual deaths, prosecutor commands on unusual death, and confiscation warrants unconditionally, as an essential document reflecting the progress of investigations. We suggest that postmortem inspection reports and photos, death scene investigation reports and photos, and death certificates should be included as part of postmortem investigation data.

OBJECTIVE: Patients with bipolar disorder have attention deficit during even euthymic status. Bipolar disorder patients showed more childhood attention deficit and other ADHD like feature. Alpha 7 nicotinic receptor (CHRNA7) gene has been known to play roles in attention and sensory gating, and association between CHRNA7 gene and bipolar disorder has been reported. Therefore, we investigated a possible association between childhood attention deficit of bipolar disorder and CHRNA7 gene polymorphisms. METHODS: We included 122 patients with bipolar disorder (89 subjects of bipolar disorder type I, 33 subjects of bipolar disorder type II). Childhood attention deficit was measured by Wender Utah Rating Scale (WURS). Factor analysis was done for WURS to extract inattention factor from childhood ADHD like feature. Three factors were extracted: Impulsivity, Inattention, and Mood instability. All subjects were ethnically Korean. Genotyping was done for three intronic Single Nucleotide Polymorphism (SNPs) of CHRNA7 gene: rs2337506 (A/G), rs6494223 (C/T), and rs12916879 (A/G). Analysis of association was done by UNPHASED version 3.1.4, a free software for genetic statistics. RESULTS: Genetic association tests found no association between factor score of inattention and any SNP or combination of SNPs of CHRNA7. Positive association between WURS total score and SNP rs6494223 (p=0.043), factor score of impulsivity and SNP rs2337506 (p=0.038) as well as SNP rs6494223 (p=0.043) was revealed. These positive associations were survived after 1,000 permutation tests. Combination of SNPs association tests performed for total WURS and factor scores could not find any significant association. CONCLUSION: We could not find association between CHRNA7 gene and childhood attention deficit in bipolar disorder. However, we found CHRNA7 gene involved in childhood impulsivity of bipolar disorder, another ADHD like feature. Further studies with larger sample and denser polymorphisms are necessary to clarify genetic role of CHRNA7 in attention and impulsivity of bipolar disorder.
Bipolar Disorder ; Humans ; Introns ; Polymorphism, Single Nucleotide ; Receptors, Nicotinic ; Sensory Gating ; Utah