Fabry disease previously diagnosed as Henoch-Schonlein purpura

No abstract available.

Negative Pressure Pulmonary Edema Together with Pneumopericardium after General Anesthesia / 대한내과학회지

Negative pressure pulmonary edema is an uncommon complication related to general anesthesia. Its main pathophysiology is excessive negative intrathoracic pressure that is caused by an acute upper airway obstruction. Pneumopericardium, the presence of air within the pericardial sac, is another rare condition. The common pathophysiology of pneumopericardium, except for that caused by blunt or penetrating trauma, is barotrauma-induced alveolar rupture caused by positive intrathoracic pressure. Here, we report the case of a 61-year old female patient with negative pulmonary edema and pneumopericardium after general anesthesia. She recovered after conservative management.

Endoscopic Clip Closure of Duodenal Perforation Caused by Percutaneous Drainage Procedure

Acute cholecystitis is a disease commonly treated in health care institutions. Cholecystectomy is the standard treatment for acute cholecystitis, and emergent laparoscopic cholecystectomy is acceptable as an effective and safe treatment modality. One of the complications after laparoscopic cholecystectomy is intra-abdominal abscess. The standard treatment for postoperative intra-abdominal abscess is percutaneus transhepatic drainage and use of antibiotics. However, duodenal perforation can occur during insertion of the pigtail catheter for drainage. Operation is the treatment of choice for iatrogenic duodenal perforations. Recent reports describe nonsurgical treatments for small gastrointestinal perforation with localized peritonitis and suggest that endoclipping may be appropriate in the management of a well selected group of patients with iatrogenic perforation. We describe a case of duodenal perforation due to pigtail catheter insertion for percutaneous transhepatic drainge that was succesfully treated by using endoclips.

Primary Pure Squamous Cell Carcinoma Originating from the Stomach

Primary gastric squamous cell carcinoma represents a rare entity. Up to date, only seven cases have been reported in Korea. This paper reports a case of a 51-year-old male patient whose stomach cancer was diagnosed during general routine check-up. Upper gastrointestinal endoscopy showed a large ulcero-fungating mass on the body of the stomach. Histological examination of biopsy confirmed squamous cell carcinoma. An extended total gastrectomy including splenectomy and segmental resection of the large bowel was conducted. Postoperative adjuvant chemotherapy with capecitabine and oxaliplatin was followed. The patient has been following up the out patient's clinic after discharge.

Primary Pure Squamous Cell Carcinoma Originating from the Stomach

Primary gastric squamous cell carcinoma represents a rare entity. Up to date, only seven cases have been reported in Korea. This paper reports a case of a 51-year-old male patient whose stomach cancer was diagnosed during general routine check-up. Upper gastrointestinal endoscopy showed a large ulcero-fungating mass on the body of the stomach. Histological examination of biopsy confirmed squamous cell carcinoma. An extended total gastrectomy including splenectomy and segmental resection of the large bowel was conducted. Postoperative adjuvant chemotherapy with capecitabine and oxaliplatin was followed. The patient has been following up the out patient's clinic after discharge.

A Case of Midazolam Anaphylaxis

Midazolam is a type of anesthetic agent frequently used for conscious sedation during a variety of medical procedures. Anaphylactic reactions to midazolam are rarely reported. However, we observed a case of midazolam hypersensitivity in which emergency measures were required to ensure patient recovery after administration of midazolam as a sedative. The occurrence of the anaphylactic reaction to midazolam was confirmed by elevated serum tryptase levels. The current case report presents a discussion of our findings.

Relationship between serum total IgE, specific IgE, and peripheral blood eosinophil count according to specific allergic diseases

PURPOSE: Elevated IgE, and blood eosinophil count are considered the hallmarks of allergic diseases, while the levels of those parameters are shared with normal population. This study is aimed to investigate the relationship between those factor and clinical value according to specific allergic diseases. METHODS: We reviewed medical records of 946 patients with allergic diseases who had taken the allergy tests from 2001 to 2010. The effects of age, sex, specific diagnoses of diseases, such as atopic dermatitis (AD), allergic rhinitis (AR), bronchial asthma (BA), urticarial on allergy tests were assessed. RESULTS: In contrast to those of total eosinophil counts, there was the trend of increasing IgE value with the age in children with allergic diseases. The titer of total IgE and eosinophil count were higher than those of control group of patients with acute urticaria. There was the positive relation between total IgE and eosinophil count in patients with allergic diseases. The positive predictive value and negative predictive value of total IgE for specific IgE were higher than those of total eoshinophil count. CONCLUSION: There were the positive relation of total IgE and total eosinophil count in allergic diseases and the higher value of those compared with the control in this study. These values over a certain level have a positive predictive value of serum specific IgE and help us to determine whether we should test the serum specific IgE in patients with allergic diseases.

The Clinical Significance of Simplified Scoring Criteria as a Diagnostic Tool for Overlap Syndrome in Korea / 대한내과학회지

BACKGROUND/AIMS: The diagnosis of primary billiary cirrhosis (PBC)-autoimmune hepatitis (AIH) overlap syndrome remains challenging. In 2008, a simplified scoring system was proposed by the International Autoimmune Hepatitis Group, which aimed for wider applicability in routine clinical practice. We evaluated the performance of the new simplified AIH scoring criteria as a diagnostic tool for overlap syndrome. METHODS: We retrospectively reviewed the clinical histories, chemistry, autoimmune studies, and liver biopsy results of 25 patients diagnosed with PBC who visited Soonchunhyang University Hospital, Bucheon and Seoul, between November 2004 and December 2009. Parameters relevant to the revised and simplified scoring criteria were recorded, and outcomes were compared between those with and without features of overlap syndrome. RESULTS: Of 25 patients with a definite diagnosis of PBC, five (20%) were diagnosed with overlap syndrome using the revised criteria, and 18 patients (72%) were diagnosed with the simplified criteria. Those patients diagnosed according to the simplified scoring criteria revealed an increased frequency of anti-nuclear antibody (p = 0.030) and serum IgG levels (p = 0.092). Additionally, advanced fibrosis was significantly more frequent in patients with overlap syndrome (p = 0.017). CONCLUSIONS: The simplified scoring criteria seemed to be useful as a diagnostic tool to recognize overlap syndrome in Korea. However, a relatively small number of patients were included in our study, so further clinical trials based on larger populations should be performed.

The Comparison of Clinical and Radiologic Results Classified by Translation Type and Fusion Method in the Isthmic Spondylolisthesis

STUDY DESIGN: A retrospective study. OBJECTIVES: To examine the radiologic and clinical results of patients classified as excessive translation and excessive angulation, treated by posterolateral fusion only, or posterolateral fusion with posterior lumbar interbody fusion in isthmic spondylolisthesis. SUMMARY OF LITERATURE REVIEW: Isthmic spondylolisthesis is usually treated by PLF only or PLF with PLIF. But it is not reported the clinical and radiologic results classified by translation type. MATERIALS AND METHODS: Patients who had received surgery for spondylolisthesis between January 2005 to January 2010, there were 56 for whom follow-up observations were possible for 2 years. According to the fusion methods and preoperative flexion-extension simple radiograph, we classified as excessive translation and excessive angulation by segmental instability and as PLF and PLIF by surgical methods. We examine the clinical results(Visual Analogue Scale, Oswestry Distability Index, operation time, blood loss, complication rate) and the radiologic results(reduction rate of slippage, change of segmental angle, reduction ratio of disc height, bone union). RESULTS: In radiologic results, excessive angulation(group II) did not show significant difference from excessive translation(group I) in terms of reduction rate of slippage, change of segmental angle, reduction ratio of disc height, bone union. But we found excessive translation-PLIF(group I-B) was better than excessive translation-PLF(group I-A) and excessive angulation-PLIF(group II-B) was better than excessive angulation-PLF(II-A) in terms of reduction ratio of disc height(P<0.05). In clinical results, both sides group did not show significant difference in operation time, blood loss, complication rate. CONCLUSIONS: In spondylolisthesis patients, excessive translation group(I) and excessive angulation group(II) did not show significant difference in radiologic results and clinical results. But both sides group showed the PLF with PLIF was better than the PLF only in terms of reduction ratio of disc height.

Two Cases of Ascending Aortic Intramural Hematoma Combined with Cardiac Tamponade Treated with Different Strategies

Aortic intramural hematoma (IMH) is classically defined as a variation of aortic dissection where blood collects within the aortic media without the presence of an intimal flap. Ascending IMH is known to have worse clinical outcomes than IMH of the descending aorta or aortic arch. Therefore, some patients with higher risk of disease progression require surgical corrections. However, the indications and the benefits of surgical management of ascending IMH, compared with medical treatment only, have not yet been established. We present two cases of IMH in the ascending aorta that presented with cardiac tamponade; patients were treated differently according to risk factors.

A case of partial trisomy 3p syndrome with rare clinical manifestations / 소아과

Partial trisomy 3p results from either unbalanced translocation or de novo duplication. Common clinical features consist of dysmorphic facial features, congenital heart defects, psychomotor and mental retardation, abnormal muscle tone, and hypoplastic genitalia. In this paper, we report a case of partial trisomy 3p with rare clinical manifestations. A full-term, female newborn was transferred to our clinic. She had cleft lip-plate, dysgenesis of the corpus callosum, patent ductus arteriosus, pulmonary hypertension, and severe right-sided hydronephrosis, associated with ureteropelvic junction obstruction. Cytogenetic investigation revealed partial trisomy 3p; 46,XX,der(4)t(3;4) (p21.1;p16). The karyotype of her father showed a balanced translocation, t(3;4)(p21.1;p16). Therefore, the size of duplication can be an important factor.

A case of partial trisomy 3p syndrome with rare clinical manifestations / 소아과

Partial trisomy 3p results from either unbalanced translocation or de novo duplication. Common clinical features consist of dysmorphic facial features, congenital heart defects, psychomotor and mental retardation, abnormal muscle tone, and hypoplastic genitalia. In this paper, we report a case of partial trisomy 3p with rare clinical manifestations. A full-term, female newborn was transferred to our clinic. She had cleft lip-plate, dysgenesis of the corpus callosum, patent ductus arteriosus, pulmonary hypertension, and severe right-sided hydronephrosis, associated with ureteropelvic junction obstruction. Cytogenetic investigation revealed partial trisomy 3p; 46,XX,der(4)t(3;4) (p21.1;p16). The karyotype of her father showed a balanced translocation, t(3;4)(p21.1;p16). Therefore, the size of duplication can be an important factor.

Ozone Exposure Suppresses Proliferative Response in Mice Skin

No abstract available.

Proximal Femoral Nail Antirotation and Proximal Femoral Nail in Intertrochanteric Fractures / 대한정형외과학회잡지

PURPOSE: The purpose of this study was to compare the curative effect of Proximal Femoral Nail Antirotation (PFNA) with a Proximal Femoral Nail (PFN) for the treatment of intertrochanteric fracture of the femur. MATERIALS AND METHODS: This study compared 58 cases of 57 patients who were treated by PFNA from June 2007 to February 2009 with 60 cases of 58 patients who were treated by PFN from July 2005 to May 2007. The mean duration of follow-up was 17.2 months (range: 12-31 months). All the fractures were classified according to the AO/ASIF systems. The operative time, the average number of days of hospitalization, the amount of bleeding, the incidence of complications, the union time as assessed on radiologic examinations, the tip apex distance (TAD), the outcome according to the Cleveland index, the change of the neck shaft angle and the amount of sliding at the end of follow-up were compared between the two groups. The clinical outcomes were compared according to the mobility score of Parker, Palmer, Jensen. The results were analyzed using the Student T-test and chi-square tests. RESULTS: There was no significant difference in blood loss during surgery, the number of hospitalization days, radiographic bone union and TAD (p>0.05). Compared with PFN, the operation time, the postoperative sliding and the neck shaft angle change were significantly less in the PFNA group (p<0.05). For the postoperative complications, there was 1 case of cutting out and 1 case of superficial infection in the PFNA group, and there were 4 cases of cutting out, 2 cases of back out, 2 cases of varus collapse, 1 case of nonunion and 1 case of superficial infection in the PFN group. CONCLUSION: Using the PFNA had relatively satisfying clinical results for the treatment of intertrochanteric fracture, as compared with those of using PFN.

Decreasing Pattern in Perinatal Mortality Rates in Korea: In Comparison with OECD Nations / 대한주산의학회잡지

PURPOSE: In Korea before 1996, the perinatal mortality rates (PMRs) were reported by individual studies, but the Korea Ministry of Health and Welfare started official reports of PMRs from 1996, and Statistics Korea provides the annual official data since 2007. The present study observed the decreasing trends of PMR and compared the PMRs between OECD nations. Thereby, we surveyed the terminology of PMR and the international trends in the usage of the calculation methods and intended to provide uniformity in calculating PMR in Korea. Also, the authors speculated some perinatal problems to be improved in the future in order to decrease PMR further. METHODS: Data before 1996 were gathered from individual reports, and after 1996 were utilized from Ministry of Health and Welfare, and Statistics Korea. Data of OECD nations were analyzed from OECD Health Data 2010. The changes in annual PMR of Korea was reviewed, which is helpful to recognize the current state of Korea, and was compared to that of OECD nations. RESULTS: During the past 50 years, the PMRs of Korea were remarkably improved (PMRs: 60 in 1960s, 35 in 1970s, 30 in 1980s, approximately, respectively). The official PMR values of Korea are as following: 6.0 in 1996, 5.2 in 1999, 4.6 in 2002, 4.2 in 2005, 3.6 in 2006, 3.7 in 2007, 3.6 in 2008, and 3.4 in 2009. The decreasing pattern of the annual PMR value was observed and the most recent value reflected the excellent situation in PMR compared to other OECD nations. CONCLUSION: Accordingly, we could observe the encouraging levels of PMR in Korea. We speculate that following efforts should be accompanied to achieve further improvement in PMR, such as improvements in neonatal and maternity transfer system, establishment in perinatal care centers, and establishment in perinatal research network system in Korea.

Changes in Statistical Birth Data Related to Maternity and Newborn over the Past 15 Years in Korea / 대한주산의학회잡지

PURPOSE: The statistical birth data related to maternity and newborns over the past 15 years in Korea was analyzed in order to review the changing patterns of maternity and newborns in Korea. METHODS: Statistical data after 1995 in Korea were obtained from the Statistics Korea. Birth data related to maternity (distribution of birth rate, maternal age, advanced maternal age group, rank of birth, and time of pregnancy) and newborn (distribution of number of births, birth weight, ratio of sex, and multiple births) were analyzed. RESULTS: The average age of maternity is increasing while births in an advanced age has also increased from 4.7% in 1995 to 15.5% in 2009. There was a decrease in the total number of births, total birth rate and average birth weight. The rate of premature births in an advanced age occupied more than 6% of this study. The ratio of sex from the third born child and beyond has been relieved. The rate of multiple births has increased. The birth rate within two years of cohabitation before giving birth to a couple's first child has decreased. CONCLUSIONS: The rates of newborns requiring perinatal care such as births of prematurity, low birth weight infants, multiple pregnancies, etc are increasing. This is in relation to the increase in age of maternity and number of pregnancies in an advanced age. Therefore, there is an increase demand for strategies in order to decrease the age of maternity and the rate of pregnancies in an advanced age.

Treatment Outcome of Lower Lumbar Fracture with Neurological Deficit

STUDY DESIGN: A retrospective study. OBJECTIVES: To evaluate the clinical results of surgical treatment for a lower lumbar fracture with a neurological deficit. SUMMARY OF LITERATURE REVIEW: There are several methods for treating lower lumbar fractures with neurological deficits but no definitive guidelines have been established. MATERIALS AND METHODS: From 2000 to 2008, this study reviewed 26 patients who had undergone surgery to treat a lower lumbar fracture with a neurological deficit and could be followed up for more than 12 months. The changes in the kyphotic angle, changes in the vertebral body height, compromise ratio of the spinal canal, recovery of neurological deficit, and clinical results were evaluated. RESULTS: There were 15, 7 and 4 cases with a third, fourth and fifth lumbar fracture, respectively. There are 19, 2 and 5 cases of an unstable bursting fracture, chance fracture and translational injury, respectively. The compromise ratio of the spinal canal improved from 67.2+/-9.4% to 16.4+/-4.6%, and the changes in the kyphotic angle improved from 14.5+/-3.2degrees to 7.6+/-2.4degrees postoperatively and 9.7+/-4.3degrees at the last follow-up. The changes in the vertebral body height improved from 41.3+/-8.4% to 23.4+/-6.3% and the bone union rate was 92.3%. The neurological deficit recovered with 1.27degrees according to the Frankel classification and good functional results were obtained in 84.6% of cases. CONCLUSIONS: Recovery of the neurological deficit and good clinical results were obtained with the recovery of the kyphotic angle and bone union with posterior decompression and instrumented posterolateral fusion in lower lumbar fractures with a neurological deficit.

A Case of Combined Facial and Vestibulocochlear Nerve Paralysis in Brainstem Infarction / 대한이비인후과학회지

Isolated cranial nerve palsy without long tract signs such as contralateral hemiparesis, hemihypoesthesia and ataxia is often reported as brainstem infarction. But most of them are accompanied by oculomotor or abducens nerve palsy. Isolated cranial nerve palsy involving the facial nerve and vestibulocochlear nerve is very rare in brainstem infarction. We report, with a review of literature, a case of right caudal pontine infarct on the dorsolateral portion of the middle cerebellar peduncle with isolated 7th and 8th cranial nerve palsies.

The Effect of Contralateral Acoustic Stimulation on the Latency of Distortion Product Otoacoustic Emissions / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The auditory efferent system -Medial olivocochlear bundle (MOCB)-controls the sensitivity and frequency selectivity of the cochlea and maintains the cochlea for optimal acoustic signaling. Contralateral acoustic sound stimulates the MOCB and has inhibitory effects on the sound evoked amplitude response of the cochlea. There are only a few reports on the latency response of contralateral acoustic stimulation (CAS) on distortion product otoacoustic emission (DPOAE), and it has no consistent conclusion. The purpose of this study was to evaluate whether changing the latency of DPOAE by CAS could be a stable method for monitoring the function of MOCB. SUBJECTS AND METHOD: The change in the latencies of DPOAE after CAS were monitored in 24 normal hearing ears with f2 sweep paradigm. The CAS level was divided into two groups, one was under 65 dB SPL and other was over 65 dB SPL. RESULTS: As f2 frequency changed from 1 kHz to 2 kHz, the latency of DPOAE was shortened from 11.82 +/- 1.87 ms to 7.29 +/- 0.86 ms in low stimulation level (50 dB SPL) and from 10.70 +/- 2.65 ms to 6.16 +/- 1.59 ms in high stimulation level (75 dB SPL) There were no significant shortening on the latency of DPOAE after CAS in low stimulation level group. But in higher stimulation level group (75 dB SPL), DPOAE latency changed from 10.70 +/- 2.65 ms to 10.12 +/- 1.95 ms (CAS level: 35 dB SL) and to 9.76 +/- 2.97 ms (CAS level: 50 dB SL) in 1 kHz, from 6.16 +/- 1.59 ms to 5.96 +/- 1.49 ms (CAS level: 35 dB SL) and to 5.83 +/- 1.28 ms (CAS level: 50 dB SL) in 2 kHz. CONCLUSION: Changes in the latency of DPOAE after CAS is not a stable monitoring tool for the function of MOCB.

Auditory Effects of Microperfused Lidocaine on Guinea Pig Cochlea / 대한이비인후과학회지

OBJECTIVES: Lidocaine is one of the therapeutic trials to treat tinnitus. However, the exact mechanism of the effect of lidocaine remains unclear. The aim of this study was to elucidate the action site of lidocaine in the cochlea by measuring compound action potential (CAP) and transient evoked otoacoustic emission (TEOAE) amplitude in guinea pigs. METHOD: Artificial perilymph was perfused into the scala tympani of the guinea pig cochlea in the control group, and lidocaine diluted with artificial perilymph was perfused into the scala tympani of the experimental groups. Electrocochleogram (ECoG) and TEOAE were measured in each groups both before and after lidocaine perfusion. RESULTS: Artificial perilymph perfused to the scala tympani of the guinea pig cochleae did not affect either the CAP threshold or the TEOAE response. But, lidocaine perfused into the scala tympani of the guinea pig cochleae produced a dose-dependent increase in CAP threshold, but did not affect TEOAE response. CONCLUSION: This study revealed that lidocaine perfused into the scala tympani of the guinea pig cochlea affects the CAPthreshold but not the TEOAE amplitude and reproducibility. It means that the locally perfused lidocaine affects the cochlear nerve greater than the outer hair cells.