Right aortic arch with isolation of the left subclavian artery is a rare anomaly. The incidence of bilateral ductus arteriosus is sporadic, and a right aortic arch with isolation of the left subclavian artery in association with bilateral ductus arteriosus is therefore extremely rare. Since the symptoms and signs of isolation of the left subclavian artery can include the absence or underdevelopment of the left arm, subclavian steal syndrome, or pulmonary artery steal syndrome, the proper therapeutic approach is controversial. We report a case in which surgical reconstruction was used to treat isolation of the left subclavian artery with right aortic arch in association with bilateral ductus arteriosus and a ventricular septal defect.
Aorta, Thoracic* ; Arm ; Ductus Arteriosus* ; Embryology ; Heart Defects, Congenital ; Heart Septal Defects, Ventricular* ; Incidence ; Pulmonary Artery ; Subclavian Artery* ; Subclavian Steal Syndrome

BACKGROUND: Beta-adrenergic receptor Kinase 1(betaARK1) is a serine/threonine kinase attached, which inhibits the coupling of beta-adrenergic receptor with G-protein. Myocardial betaARK1 level is usually elevated in heart failure and hypertrophy, but it is not known whether the circulating betaARK1 level is related with the degree of cardiac hypertrophy. This study was performed to evaluate the association of the betaARK1 level in circulating mononuclear leukocytes(MNL) in untreated hypertension with left ventricular mass in hypertensive patients. Method: Nineteen non-treated hypertensive patients were included for this study. High blood pressure was confirmed when systolic BP is over 150 mmHg or diastoli BP is over 95 mmHg. Echocardiography was performed to evaluate the degree of hypertrophy by measuring the left ventricular mass index(LVMI) and relative wall thickness(RWT), and test the LV function by measuring the ejection fraction(EF) according to ASE guideline. At the same time, blood was collected from each patient and MNL were isolated by gradient centrifuge with Ficoll-400. Total RNA was purified from MNL and semi-quantitative RT-PCR was performed. After reverse transcription, PCR was done with primers for human betaARK1 and GAPDH as external control. betaARK1 levels were expressed by ratio to GAPDH level and estimated the relations with clinical and Echocardiographic parameters. Result: We studied confirmed 19 hypertensive patients(10 men and 9 women, mean age of 50.6 years). Echocardiographically measured indices(mean+/-SD) were as follows; LVMI(137.3+/-30.6g/m2), PWT(0.53+/-0.09) and EF(54.6+/-8.5%). Ratio of betaARK1 levels to GAPDH was from 0.10 to 0.96 (0.62+/-0.25). betaARK1 levels were correlated with LVMI(correlation coefficient: r=.502, p=.029) and RWT(r=.627, p=.004). But Systolic BP(r=0.009, p=.93), diastolic BP(r=.07, p=.85) or EF(r=.045, p=.84) were not related to level of betaARK1. CONCLUSIONS: The betaARK1 level of circulating MNL was correlated well with the degree of the cardiac hypertrophy estimated by LVMI and RWT. This data suggests that activation of sympatho-adrenal system would exert a major role in developing cardiac hypertrophy and we can expect the decreased responsiveness to catecholamine in the heart of hypertensive patients. betaARK1 in circulating MNL might be used as a predictor or marker for LV hypertrophy in hypertensive patients.
beta-Adrenergic Receptor Kinases ; Cardiomegaly ; Echocardiography ; Female ; GTP-Binding Proteins ; Heart ; Heart Failure ; Humans ; Hypertension ; Hypertrophy ; Leukocytes, Mononuclear* ; Male ; Phosphotransferases ; Polymerase Chain Reaction ; Reverse Transcription ; RNA

PURPOSE: The prevalence of developmental delay is 5-10% of the total pediatric population and early diagnosis and proper intervention are essential but a challenge for clinicians. We performed this study for several purposes. First is to know the distribution & characteristics of developmental disorders in Korea. Second is to identify all possible causes of these disorders through a multidisciplinary diagnostic approach, and thus to find out the clinical variables that are helpful in finding the etiology. And finally to develop a useful protocol that eliminates the cost of unnecessary tests and raises the diagnostic rate of the cause. METHODS: 518 patients(M 349, F 169) were studied who visited Ilsan Hospital Developmental Disorder Clinic(DDC) for the evaluation of developmental delay from April 2001 to Jan 2005. RESULTS: The mean age was 51.5+/-32.9 months, ranging from 2 months to 16.0 years of age with a majority of the preschool children(<6 yr)(79.3%). Phenomenological diagnosis consisted of 133 cases of mental retardation, 122 cases of autistic disorders, 101 cases of delayed language disorders, 27 cases of cerebral palsies, and 91 cases labeled as simple developmental delay requiring follow up due to age less than 2 years of age. Etiologic diagnosis was obtained in 119 cases(22.9%) out the 518 cases of developmental delays. 37 cases of chromosomal anomalies, 23 cases of Periventricular leukomalacia and hypoxic ischemic encephalopathy, 21 cases of syndromes, 7 cases of malformation of cortical development, 4 cases of myopathies, 4 cases of neuropathies, and 4 cases of cerebral infarctions were found. Among the clinical variables, low birth weight, facial dysmorphism, hypotonia, focal neurologic signs, and abnormalities in MRI, chromosome, EEG and EMG studies contributed to the yields of etiologic diagnosis significantly. CONCLUSION: Possible etiology was determined in about 23% of the subjects. The most important part of the assessment for the identification of etiology is thorough history taking, physical and neurologic examination. Neuroimaging study is useful in case of micro or macrocephaly, focal neurologic signs. Genetic studies increasingly produce a yield, when there is family history of inherited disorder and there are dysmorphic features. Routine metabolic screening test has limited utility. Development of a useful screening protocol adequate for Korean situation is required.
Cerebral Infarction ; Diagnosis ; Early Diagnosis ; Electroencephalography ; Follow-Up Studies ; Humans ; Hypoxia-Ischemia, Brain ; Infant, Low Birth Weight ; Infant, Newborn ; Intellectual Disability ; Korea ; Language Disorders ; Leukomalacia, Periventricular ; Macrocephaly ; Magnetic Resonance Imaging ; Mass Screening ; Muscle Hypotonia ; Muscular Diseases ; Neuroimaging ; Neurologic Examination ; Neurologic Manifestations ; Paralysis ; Prevalence

Isolated fractures of lower sacrum are commonly treated conservatively in most cases because of low energy damage and stable fracture. However, surgical treatment is required in displaced unstable fracture. But the surgical technique is not established and even case reports are not common. We reported a case of displaced transverse fracture of the lower sacrum that was treated with an open reduction and dual plate fixation.

BACKGROUND AND OBJECTIVES: The incidence of retropharyngeal abscess has been decreased with use of antibiotics, but it still causes critical complications such as airway obstruction, aspiration pneumonia, mediastinitis, or sepsis. For this reason, early diagnosis and proper management of retropharyngeal abscess should be undertaken as soon as possibile. The treatment includes maintaining airway, performing surgical drainage, and administering antibiotics; but there are no definite guidelines for treating patients with retropharyngeal abscess, nor a golden rule for the correct surgical approach. SUBJECTS AND METHOD: A retrospective analysis of patients with retropharyngeal abscess who were treated at the Chungbuk University Hospital from 1993 to 2003 was performed. We analized general symptoms, signs, causing factors, and causing bacteria. We also analized the treatment outcome and selected approaches from the surgically treated patients. RESULTS: The general symptoms of retropharyngeal abscess were sore throat, fever, dyspnea, with the most common cause being the upper airway infection. Bacteriologically, hemolytic streptococcus was the most common bacteria among aerobes and bacteroides was the most common bacteria among anaerobes. However, in most cases, both aerobe and anaerobes were cultured at the same time. Among 18 cases, 6 cases were treated conservatively with antibiotics and surgical interventions were performed in 12 cases, which included 11 cases of transoral and 1 case of transcervical approach. CONCLUSION: Retropharyngeal abscess can be treated with either conservative or surgical treatment according to initial abscess size. The abscess size which is greater than 3 cm is prefered to be treated surgically, and transoral approach is a useful way of dealing with less invasive procedure.
Abscess ; Airway Obstruction ; Anti-Bacterial Agents ; Bacteria ; Bacteroides ; Chungcheongbuk-do ; Drainage ; Dyspnea ; Early Diagnosis ; Fever ; Humans ; Incidence ; Mediastinitis ; Pharyngitis ; Pneumonia, Aspiration ; Retropharyngeal Abscess* ; Retrospective Studies ; Sepsis ; Streptococcus ; Treatment Outcome

Purpose: We investigated the serial culture of multiuse eye drops applied by medical personnel by intentionally touching the tip of the bottle. Methods: Ten different unpreserved and preserved eye drops including eye drops for allergies, artificial tears, antibiotics, and corticosteroids were included. One bottle was stored at room temperature and the other bottle stored under refrigeration. The experimenter intentionally touched the tip of the bottle with a finger twice daily. The first culture test was performed 2 weeks after contamination and the culture were performed six times; 2 weeks apart. Results: Contamination was noted in three types of eye drops (artificial tears without preservatives, corticosteroids, and anti-allergic eye drops) under refrigerated conditions and four types of eye drops (artificial tears without preservatives, artificial tears with preservatives, corticosteroids, and anti-allergic eye drops) under room temperature conditions. The contamination in 13 eye drops was attributable to five microbial species: Staphylococcus epidermidis (m/c), Staphylococcus hominis, Rhodotorula sp., Corynebacterium sp., and Bacillus sp. Under refrigerated conditions, three eyedrop bottles were contaminated, with five positive cultures in 12 weeks. However, at room temperature, four eye drop bottles were contaminated with eight positive cultures. The culture results changed according to culture time. In some cases, the same result was obtained 2 weeks after the first positive culture but in other cases the result was negative or one of two strains disappeared. Conclusions Eye drops can be contaminated under both room temperature and refrigerated conditions. We should consider serial culture for eye drops because the culture results could be variable according to time.

No abstract available.
Immunoglobulin A* ; Immunoglobulins*

This study evaluated the methodological quality of CPGs using the Korean AGREE II scoring guide and a web-based appraisal system and was conducted by qualified appraisers. A total of 27 Korean CPGs were assessed under 6 domains and 23 items on the AGREE II instrument using the Korean scoring guide. The domain scores of the 27 guidelines were as following: the mean domain score was 82.7% (median 84.7%, ranging from 55.6% to 97.2%) for domain 1 (scope and purpose); 53.4% (median 56.9%, ranging from 11.1% to 95.8%) for domain 2 (stakeholder involvement); 63.0% (median 71.4%, ranging from 13.5% to 90.6%) for domain 3 (rigor of development); 88.9% (median 91.7%, ranging from 58.3% to 100.0%) for domain 4 (clarity of presentation); 30.1% (median 27.1%, ranging from 3.1% to 67.7%) for domain 5 (applicability); and 50.2% (median 58.3%, ranging from 0.0% to 93.8%) for domain 6 (editorial independence). Three domains including scope and purpose, rigor of development, and clarity of presentation were rated at more than 60% of the scaled domain score. Three domains including stakeholder involvement, applicability, and editorial independence were rated at less than 60% of the scaled domain score. Finally, of the 27 guidelines, 18 (66.7%) were rated at more than 60% of the scaled domain score for rigor of development and were categorized as high-quality guidelines.
Humans ; Internet ; *Practice Guidelines as Topic ; *Program Evaluation ; Quality Assurance, Health Care ; Republic of Korea

PURPOSE: Neonatal jaundice is one of the most common problems in our country leading to hospitalization. Agar is low cost, low risk, and easily fed orally; it can bind bilirubin in the intestine, decreasing its enterohepatic circulation, thereby decreasing serum bilirubin levels. At present, however, the effectiveness of agar in the prevention and treament of neonatal jaundice is quite conflicting and controversy. Recently we have read Caglayan's 'Superiority of Oral Agar and Phototherapy Combination in the Treatment of Neonatal Hyperbilirubinemia'. The result was very hopeful and attractive enough, and which gave us a motivation to study if it was really of value. METHODS: From May 1995 to April 1996, a total 50 term neonates admitted in nursery of Dong-Eui Medical Center with the capillary serum bilirubin levels greater than 10mg/ dl were enrolled in the study. Those with pathologic causes and breast fed infants were all excluded. The neonates were randomly devided into two groups; 25 of conventional phototherapy alone (P group) and 25 of oral agar plus conventional phototherapy combination (A+P group). The study was terminated when the capillary serum bilirubins were decreased to 8mg/dl. Pastagar B (Pasteur Institute 64946) 500mg in 10ml distilled water were fed four times a day using 10ml syringes prior to bottle feeding. Capillary serum bilirubin levels were measured daily at 10:00 a.m. with heel pad samples. Daily stool frequency and adverse effects of treatment were observed closely. RESULTS: 1) The decrement of the serum bilirubin levels at first 24 hours of therapy was significantly different between P and A+P groups showing as 1.7+/-1.2 and 2.4+/-1.0mg/dl respectively (p<0.05). 2) Mean time for bilirubin to decrease to 8mg/dl was shorter in A+P group than in P group showing as 45.7+/-20.8 and 57.5+/-32.3 hours each other, but those differences were statistically insignificant (p>0.05). 3) No adverse effects such as rashes or abdominal pains were observed during treatment. Differences of mean stool frequency were significant between P and A+P groups showing as 3.7+/-1.2 and 4.7+/-2.0 times per day respectively (p<0.05). CONCLUSION: The agar plus conventional phototherapy combination was superior to conventional phototherapy alone at first 24 hours of therapy in neonatal hyperbilirubinemia, but further more careful researches would be necessary for using it routinely in the treatment of neonatal hyperbilirubinemia in future.
Abdominal Pain ; Agar* ; Bilirubin ; Bottle Feeding ; Breast ; Capillaries ; Enterohepatic Circulation ; Exanthema ; Heel ; Hope ; Hospitalization ; Humans ; Hyperbilirubinemia, Neonatal* ; Infant ; Infant, Newborn ; Intestines ; Jaundice, Neonatal ; Motivation ; Nurseries ; Phototherapy* ; Syringes ; Water

Seesaw nystagmus refers to torsional nystagmus with dissociated vertical components. In this disorder, one eye goes up and intorts while the other one descends and extorts. We describe a 40-year-old man who presented with decreased vision due to pendular seesaw nystagmus. Brain MRI showed the empty sella and enlarged pituitary fossa. The delayed and decreased responses of the visual evoked potentials in the ipsilateral occipital lobe with monocular stimulation suggested unequal crossing of the optic pathways as an underlying mechanism.
Adult ; Brain ; Evoked Potentials, Visual ; Humans ; Magnetic Resonance Imaging ; Occipital Lobe