[Show abstract][Hide abstract] ABSTRACT: This study evaluated the efficacy of a stepwise regimen of estradiol valerate for height control in girls with Marfan syndrome. Eight girls with Marfan syndrome who had completed estrogen treatment for height control were included. Estradiol valerate was started at a dose of 2 mg/day, and then was increased. The projected final height was estimated using the initial height percentile (on a disease-specific growth curve for Korean Marfan syndrome [gcPFHt]), and the initial bone age (baPFHt). After the estrogen treatment, the projected final height was compared to the actual final height (FHt). The median baseline chronological and bone age were 10.0 and 10.5 years, respectively. After a median of 36.5 months of treatment, the median FHt (172.6 cm) was shorter than the median gcPFHt (181.0 cm) and baPFHt (175.9 cm). In the six patients who started treatment before the age of 11 years, the median FHt (171.8 cm) was shorter than the median gcPFHt (181.5 cm) and baPFHt (177.4 cm) after treatment. The median differences between the FHt and gcPFHt and baPFHt were 9.2 and 8.3 cm, respectively. In two patients started treatment after the age of 11, the differences between FHt and gcPFHt, and baPFHt after treatment were -4 and 1.4 cm, and -1.2 and 0 cm for each case, respectively. A stepwise increasing regimen of estradiol valerate may be an effective treatment for height control in girls with Marfan syndrome, especially when started under 11 years old.
Preview · Article · Jan 2016 · Journal of Korean Medical Science
[Show abstract][Hide abstract] ABSTRACT: Background:
Although current guidelines for pulmonary embolism (PE) treatment recommend surgical embolectomy when thrombolysis is contraindicated or has failed, their clinical outcomes rarely have been compared directly.
After excluding patients aged under 18years and those with submassive or non-massive PE, 45 consecutive patients (median age, 68years; 62% female; 31% experienced cardiac arrest before PE treatment onset; 33% had cancer diagnosis history; and 29% received extracorporeal membrane oxygenation [ECMO]) who underwent only thrombolysis (TL group; n=19) or surgical embolectomy (SE group; n=26, including 4 who had failed thrombolysis) for acute massive PE from 2000 to 2013 at Samsung Medical Center were enrolled to assess cardiac mortality as primary outcome.
Median follow-up duration was 17.2months. In the SE group, significantly higher proportions of patients had recent surgery and ECMO. Overall 30-day all-cause mortality rate was 24% (n=11), without significant difference between the SE (15%) and TL (37%) groups (P=0.098); however, cardiac mortality rate was significantly higher in the TL than SE group (Log rank P=0.023). TL was an independent multivariate predictor of cardiac death (P=0.03).
In this small retrospective single center experience, surgical embolectomy is associated with lower cardiac mortality risk than thrombolysis, which might render it first-line treatment option for acute massive PE for patients without life-limiting comorbidities.
Full-text · Article · Nov 2015 · International journal of cardiology
[Show abstract][Hide abstract] ABSTRACT: Hypertensive disorders of pregnancy (HDP) is one of the most important lethal complications in pregnant mothers. It is also associated with the subsequent development of chronic hypertension. The objective of this study was to identify the clinical risk factors of postpartum chronic hypertension in women diagnosed with HDP.
Six hundred patients as HDP, who diagnosed and followed-up at least 6 month after delivery, were included in the study. We divided the included subjects in 2 groups based on the development of postpartum chronic hypertension: presenting with the chronic hypertension, “case group” (n = 41) and without chronic hypertension, “control group” (n = 559).
Clinical and demographic factors were evaluated. By multiple regression analysis, early onset hypertension with end-organ dysfunction, smoking, higher prepregnancy body mass index (BMI), and comorbidities, systemic lupus erythematosus (SLE) or antiphospholipid syndrome (APLS), were associated with progression to chronic hypertension in the postpartum period. The value of area under the curves (AUC) for the 5 models, that generated to combine the significant factors, increased from 0.645 to 0.831, which indicated improved prediction of progression to the chronic hypertension. Additional multivariate analysis revealed significant specific risk factors.
This retrospective single hospital-based study demonstrated that the clinical risk factors, that is early onset hypertension with end-organ dysfunction, smoking, and higher prepregnancy BMI, were significant independent predictors of chronic hypertension in women after delivery. Identification of risk factors allowed us to narrow the subject field for monitoring and managing high blood pressure in the postpartum period.
[Show abstract][Hide abstract] ABSTRACT: A new CT-based diagnostic method of protrusio acetabuli (PA) was introduced. However, prevalence of PA by this method and correlation between PA and other manifestations of Marfan syndrome (MFS) is unknown in Korean MFS patients. This study aimed to investigate the prevalence of PA diagnosed by a CT-based method in Korean patients with MFS, the association of PA with other manifestations of MFS, and the contribution of PA to MFS diagnosis. We retrospectively reviewed the records of 146 MFS patients with the presence of a causative FBN1 mutation and 146 age- and sex-matched controls from a single tertiary care center. All MFS patients underwent a complete assessment of criteria based on the revised Ghent nosology. PA was assessed quantitatively using a CT-based circle-wall distance (CWD) method. PA was diagnosed in 77.4% of patients in the MFS group and in 11.0% of the control group. CWD was significantly different between the two groups (1.50 mm vs. -0.64 mm, P<0.001). The presence of PA did not correlate with the presence of ectopia lentis, aortic root diameter, or history of aortic dissection. The presence of PA did not have a significant impact on the final diagnosis of MFS. Even though the presence of PA does not related to the cardinal clinical features of MFS or influence MFS diagnosis, its presence may be helpful for the suspicion of MFS when aortic dissection or aneurysm is found on CT angiography of the aorta because of the high frequency of PA in MFS patients.
Preview · Article · Sep 2015 · Journal of Korean medical science
[Show abstract][Hide abstract] ABSTRACT: Because of frequent tuberculosis in patients with Takayasu arteritis (TA), a possible relationship between TA and tuberculosis has been proposed. However, there are no studies to date that have examined clinical manifestations in patients diagnosed with TA with or without tuberculosis. Two hundred sixty-seven patients were diagnosed with TA according to the 1990 American College of Rheumatology criteria between September 1994 and April 2014. Patients with TA were classified into groups with or without tuberculosis. Among the 267 patients with TA studied, 47 patients (17.7 %) who had a history of previous treatment of tuberculosis (34 patients), concurrent diagnosis of tuberculosis with TA (10 patients), or diagnosis of tuberculosis during the follow-up period for TA (3 patients) were included in the group with tuberculosis. The group with tuberculosis comprised of 33 patients (70.2 %) with pulmonary tuberculosis, 12 patients (25.5 %) with tuberculous lymphadenitis, and 2 patients (4.3 %) with tuberculosis of the skin and colon, respectively. Comorbid disease and patients' signs and symptoms were not significantly different between TA patients with and without tuberculosis. Additionally, the site of disease involvement in angiographic findings and distribution of angiographic type were similar between the two groups. In conclusion, tuberculosis including tuberculous lymphadenitis was frequently observed in patients with TA. Clinical features and angiographic findings in TA were not different according to the presence or absence of concomitant tuberculosis.
No preview · Article · Aug 2015 · Heart and Vessels
[Show abstract][Hide abstract] ABSTRACT: Because vagally mediated reflex bradycardia occurs frequently during gastrectomy and is potentially harmful, we compared the incidence of clinically significant reflex bradycardia between patients undergoing laparoscopic gastrectomy (LG) and open gastrectomy (OG) and examined whether the type of surgery (OG vs. LG) was an independent risk factor for clinically significant reflex bradycardia.
This prospective observational study evaluated 358 adult patients (age 18-70 years) who were undergoing elective OG or LG for gastric cancer resection. Symptomatic reflex bradycardia was defined as a sudden decrease in heart rate to <50 beats per minute (bpm), or to 50-59 bpm with a systolic blood pressure <70 mmHg, associated with a specific surgical maneuver. If bradycardia or hypotension developed, atropine or ephedrine was administered, in accordance with a predefined treatment protocol.
The overall incidence of symptomatic reflex bradycardia was 24.6 % (88/358). Univariate analysis revealed the incidence of symptomatic reflex bradycardia in the LG group was significantly lower than that in the OG group [13.0 % (13/100) vs. 29.1 % (75/258), p = 0.002]. Multivariate logistic regression analysis revealed that the type of surgery (OG vs. LG), advanced age, preoperative bradycardia, type of muscle relaxant (vecuronium vs. rocuronium), no use of intravenous remifentanil, and low core temperature, were independent risk factors for symptomatic reflex bradycardia (odds ratio 3.184; 95 % confidence interval 1.490-6.800; p = 0.003).
The LG approach was associated with a reduced risk of clinically significant reflex bradycardia compared with the OG approach.
No preview · Article · Aug 2015 · Journal of Anesthesia
[Show abstract][Hide abstract] ABSTRACT: Patients with Marfan syndrome (MFS) presents with primary skeletal manifestations such as tall stature, chest wall abnormality, and scoliosis. These primary skeletal manifestations affect the growth pattern in MFS. Therefore, it is not appropriate to use normal growth charts to evaluate the growth status of MFS. We aimed to develop disease-specific growth charts for Korean MFS patients and to use these growth charts for understanding the growth patterns in MFS and managing of patients with MFS. Anthropometric data were available from 187 males and 152 females with MFS through a retrospective review of medical records. Disease-specific growth charts were generated and 3, 25, 50, 75, and 97 percentiles were calculated using the LMS (refers to λ, μ, and σ, respectively) smoothing procedure for height and weight. Comparisons between MFS patients and the general population were performed using a one-sample t-test. With regard to the height, the 50th percentile of MFS is above the normative 97th percentile in both genders. With regard to the weight, the 50 percentile of MFS is above the normative 75th percentile in male and between the normative 50th percentile and the 75th percentile in female. The disease-specific growth charts for Korean patients with MFS can be useful for monitoring growth patterns, planning the timing of growth-reductive therapy, predicting adult height and recording responses to growth-reductive therapy.
Full-text · Article · Jul 2015 · Journal of Korean Medical Science
[Show abstract][Hide abstract] ABSTRACT: Because Takayasu arteritis (TA) predominantly affects females, few data regarding gender differences have been reported. The aim of the present study is to describe clinical features and angiographic findings of patients with TA according to gender.
According to the 1990 American College of Rheumatology criteria, 294 patients were diagnosed with TA between September 1994 and April 2014 at a single tertiary hospital. We reviewed clinical, laboratory, and radiologic data at the time of diagnosis.
Among the 294 patients studied, 257 (87.4%) were female (male:female ratio=1:6.9). Female patients had a higher tendency to exhibit blood pressure differences between arms (p=0.595) and a weak pulse at the brachial artery (p=0.063). In male patients, we observed higher serum creatinine levels (p=0.038) and hypertension more frequently (p=0.061) than in females. Females exhibited more common lesions in the thoracic aorta and its branches, while males had more frequent lesions in the abdominal aorta and its branches. An analysis of angiographic classification according to the International TA Conference in Tokyo 1994 classification revealed that male patients had a higher incidence of type IV and females showed a higher incidence of types I, IIa, and IIb.
Female patients with TA have more frequent involvement of the thoracic aorta and its branches, whereas involvement of the abdominal aorta and its branches is more common in males. Considering these gender-specific differences, adjustment of diagnostic criteria for TA according to gender may be necessary.
No preview · Article · May 2015 · Clinical and experimental rheumatology
[Show abstract][Hide abstract] ABSTRACT: The influence of body-mass index (BMI) on spinal anesthesia is still controversial, with discrepant results reported in previous studies. To compare spinal anesthesia in obese and non-obese subjects, the anesthesia profiles in patients who underwent spinal anesthesia using intrathecal hyperbaric bupivacaine were compared. A total of 209 patients undergoing elective total knee replacement arthroplasty (TKRA) surgery under spinal anesthesia were divided into an NO (non-obese) group (BMI < 30 kg/m2, n = 141) and an O (obese) group (BMI ≥ 30 kg/m2, n = 68). Anesthesia was deemed successful if a bilateral T12 sensory block occurred within 15 minutes of intrathecal drug administration, and if the level of sensory block was higher than T12 when the surgery ended. Logistic regression analysis with multiple variables known to influence spinal anesthesia was performed to identify which parameters independently determined the spinal anesthesia outcome. Similar doses of bupivacaine were administered to the NO and O groups. The incidence of anesthesia failure was significantly lower in the O group [n = 43 (30.5%) in the NO group vs. n = 10 (18.9%) in the O group, p = 0.014]. The independent predictors for successful anesthesia in all patients were dose of hyperbaric bupivacaine [odds ratio (OR) 2.12, 95% CI: 1.64-2.73] and obese status (BMI ≥ 30 kg/m2, OR 2.86, 95% CI: 1.25-6.52). Time to first report of postoperative pain and time to first self-void were significantly longer in the O group. These results suggest that the duration of block with hyperbaric bupivacaine is prolonged in obese patients and obesity is independently associated with spinal anesthesia outcomes, as is bupivacaine dosage. A further study enrolling patients with morbid obesity and using a fixed bupivacaine dosage is required to confirm the effect of obesity on spinal anesthesia.
[Show abstract][Hide abstract] ABSTRACT: To identify the incidence and the risk factors of emergence agitation in adults undergoing general anesthesia for nasal surgery.
We retrospectively examined 792 patients aged ≥18 years who underwent general anesthesia for elective nasal surgery between July 2012 and August 2013. Patients in the postanesthesia care unit with a Richmond Agitation Sedation Scale≥+1 at any time were considered to have emergence agitation.
The overall incidence of emergence agitation is 22.2%. From multivariate regression analysis, the following six variables were found to be significantly associated with emergence agitation (P<0.05): younger age, recent smoking, sevoflurane anesthesia, postoperative pain on the numerical rating scale (NRS)≥5, presence of a tracheal tube, and presence of a urinary catheter. Presence of a tracheal tube was the greatest risk factor, increasing the risk of developing emergence agitation by approximately fivefold (odds ratio, 5.448; 95% confidence interval, 2.973 to 9.982). Younger age was also a strong risk factor (odds ratio, 0.975 for each 1-year increase; 95% confidence interval, 0.964 to 0.987). Current smoking, sevoflurane anesthesia, postoperative pain of NRS≥5, and the presence of a urinary catheter nearly doubled the risk of emergence agitation.
Emergence agitation following general anesthesia is a common complication in adult nasal surgery patients. To reduce the occurrence and consequences of agitation episodes, elimination of the associated risk factors is necessary, especially in at-risk patients.
Preview · Article · Mar 2015 · Clinical and Experimental Otorhinolaryngology
[Show abstract][Hide abstract] ABSTRACT: Using the Korean Society of Anesthesiologists database of anesthesia-related medical disputes (July 2009-June 2014), causative mechanisms and injury patterns were analyzed. In total, 105 cases were analyzed. Most patients were aged < 60 yr (82.9%) and were classified as American Society of Anesthesiologists physical status ≤ II (90.5%). In 42.9% of all cases, the injuries were determined to be 'avoidable' if the appropriate standard of care had been applied. Sedation was the sec most common type of anesthesia (37.1% of all cases), following by general anesthesia. Most sedation cases (27/39, 69.2%) showed a common lack of vigilance: no pre-procedural testing (82.1%), absence of anesthesia record (89.7%), and non-use of intra-procedural monitoring (15.4%). Most sedation (92.3%) was provided simultaneously by the non-anesthesiologists who performed the procedures. After the resulting injuries were grouped into four categories (temporary, permanent/minor, permanent/major, and death), their causative mechanisms were analyzed in cases with permanent injuries (n=20) and death (n=82). A 'respiratory events' was the leading causative mechanism (56/102, 54.9%). Of these, the most common specific mechanism was hypoxia secondary to airway obstruction or respiratory depression (n=31). The sec most common damaging event was a 'cardiovascular events' (26/102, 25.5%), in which myocardial infarction was the most common specific mechanism (n=12). Our database analysis demonstrated several typical injury profiles (a lack of vigilance in seemingly safe procedures or sedation, non-compliance with the airway management guidelines, and the prevalence of myocardial infarction) and can be helpful to improve patient safety.
Full-text · Article · Feb 2015 · Journal of Korean Medical Science
[Show abstract][Hide abstract] ABSTRACT: Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN) are complications of portal hypertension and cirrhosis. Their pathophysiological mechanisms clearly differ. HPS is characterized by a defect in arterial oxygenation induced by pulmonary vascular dilatation. In contrast, PPHTN is predominantly due to excessive pulmonary vasoconstriction and vascular remodeling, but is rarely associated with hypoxia. We report a case of a patient who had both HPS and PPHTN at the time of presentation. HPS was aggravated after sildenafil administration for the treatment of PPHTN. We demonstrated increased amount of intrapulmonay shunt after sildenafil challenge by using agitated saline contrast transthoracic echocardiography.
Preview · Article · Jan 2015 · Korean Circulation Journal
[Show abstract][Hide abstract] ABSTRACT: Purpose The aim of this study is to investigate the association between short sleep hour and body mass index (BMI) obesity after adjustment for socioeconomic position and health behavior factors in the general representative population in Korea in 2007-2009.
Methods The fourth Korea National Health and Nutrition Examination Survey was conducted by the Korea Institute for Health and Social Affairs for the Korean Ministry of Health and Welfare in 2007-2009. The sample included 6,424 Korean Young adults aged 20-44 years (male 3,400 and female 3,017). Sleep duration was grouped by hours slept but was also classified into two categories: short sleepers (<6 h/night) and normal sleepers (6–9 h/night).
Results The distribution of short sleepers and normal sleepers was 9.5% and 90.5%, respectively. The distribution of obesity was 27.8%. The adjusted odds ratio (OR) for obesity was 1.39 (95% Confidence Interval (CI) 1.09-1.77) after adjustment for confounding factors. In male, the adjusted OR for obesity was not significant difference, however, in female, the adjusted OR for obesity was 1.66 (95% CI 1.14-2.40)
Conclusions This community-based random sample drawn from the entire Korean young adults population showed that short sleep hour was significantly associated with obesity, especially, female.
[Show abstract][Hide abstract] ABSTRACT: Purpose The aim of this study is to examine the prevalence of chronic kidney disease (CKD) in metabolic syndrome (MetS) and to investigate the association between metabolic syndrome (MetS)and chronic kidney disease (CKD) after adjustment for socioeconomic position and health behavior factors in the general representative population in Korea in 1998 through 2009.
Methods The first, second, third and fourth Korea National Health and Nutrition Examination Survey (KNHANES I, II, III, and IV) was conducted by the Korea Institute for Health and Social Affairs for the Korean Ministry of Health and Welfare in 1998, 2001, 2005 and 2007-2009. The sample included 11,289 Korean men and women aged 45-64 years. Subjects were classified into two groups based on their baseline glomerular filtration rate (GFR) using the Chronic Kidney Disease Epidemiology Collaboration equation: 1) greater than or equal to 60 mL/min/1.73 m2 (non-CKD group; normal and mild renal dysfunction), and 2) less than 60 mL/min/1.73 m2 (CKD group; moderate, severe renal dysfunction, and renal failure). The MS was determined using National Cholesterol Education Program Adult Treatment Panel III criteria and the Asia-Pacific criteria for obesity based on waist circumference.
Results The distribution of CKD was 1.8%, 6.8%, 3.6% and 2.9% in 1998, 2001, 2005, and 2007-2009, respectively. The distribution of MetS was 41.4%, 41.9%, 39.7% and 34.3%, respectively. The adjusted odds ratio for MS was 6.26 (95% Confidence Interval (CI) 1.78-21.9), 2.96 (95% CI 1.02-8.54), 2.55 (95% CI 0.50-13.0), and 1.89 (95% CI 1.19-3.02) after adjustment for confounding factors, respectively.
Conclusions This community-based random sample drawn from the entire Korean middle aged population showed that MS was significantly associated with CKD.
[Show abstract][Hide abstract] ABSTRACT: Hereditary protein S deficiency is one of the natural anticoagulant deficiency causing thrombophilia. Protein S deficiency is caused by a mutation in the PROS1 gene on 3q11.2 and is typically inherited in an autosomal dominant manner. We herein describe a Korean man with protein S deficiency from a novel nonsense mutation of PROS1. The patient was a 47-year-old man with deep-vein thrombosis. No relevant family history was documented. Coagulation test results included a significantly decreased protein S activity at 30%. Molecular genetic analysis targeting PROS1 on suspicion of hereditary protein S deficiency revealed that he was heterozygous for a novel transversion mutation, c.903C>G, in the exon 9 of PROS1. The mutation was predicted to result in premature termination at the codon 301 in the laminin G-type domain (p.Tyr301) of the protein (nonsense mutation). According to a review of the literature and database, the mutation described herein is the first substitution mutation affecting the codon 301 of PROS1.
No preview · Article · Sep 2014 · Blood coagulation & fibrinolysis: an international journal in haemostasis and thrombosis
[Show abstract][Hide abstract] ABSTRACT: Purpose
Compared to open gastrectomy (OG), laparoscopic gastrectomy (LG) has improved short-term outcomes and equivalent oncological outcomes. In this study, a potential short-term advantage of LG over OG, reduced risk of emergence agitation, was evaluated.
This retrospective study compared LG versus OG with respect to emergence agitation in 400 adult patients who underwent sevoflurane anesthesia. In all cases, a serial Richmond Agitation–Sedation Scale (RASS) assessment was performed in the postanesthesia care unit (PACU). Patients with a RASS score ≥+1 at any time were considered to have emergence agitation. Severe agitation was defined as a RASS score of +3 or +4.
This study included 214 OG group subjects and 186 LG group subjects in the analysis. The overall incidence of emergence agitation was significantly lower in the LG group than the OG group (23.7 vs. 43.5 %, p
No preview · Article · Aug 2014 · Journal of Anesthesia
[Show abstract][Hide abstract] ABSTRACT: Dilated cardiomyopathy (DCM) is a progressive heart disease characterized by left ventricular dilation and contractile dysfunction. Although many candidate genes have been identified with mouse models, few of them have been shown to be associated with DCM in humans. Germline depletion of Ncoa6, a nuclear hormone receptor coactivator, leads to embryonic lethality and heart defects. However, it is unclear whether Ncoa6 mutations cause heart diseases in adults. Here, we report that two independent mouse models of NCOA6 dysfunction develop severe DCM with impaired mitochondrial function and reduced activity of peroxisome proliferator-activated receptor δ (PPARδ), an NCOA6 target critical for normal heart function. Sequencing of NCOA6-coding regions revealed three independent nonsynonymous mutations present in 5 of 50 (10%) patients with idiopathic DCM (iDCM). These data suggest that malfunction of NCOA6 can cause DCM in humans.