Parry-Romberg syndrome is a rare acquired disease of unknown etiology that is classically characterized by progressive atrophy of the skin, subcutaneous tissues, and underlying bone structures. Eyelid myokymia is a disorder of involuntary, fine, continuous, undulating muscle fiber contractions, which is seen as a rippling of the overlying periorbital area. We report a patient with Parry-Romberg syndrome presenting eyelid myokymia.

Background: and Purpose We performed a population-based study to determine the prevalence and incidence of chronic inflammatory demyelinating polyneuropathy (CIDP) in South Korea using data from the Korean Health Insurance Review and Assessment Service (HIRA) database. Methods: Data recorded in the HIRA database between January 2016 and December 2020 were analyzed. The inclusion criteria in this study for patients with CIDP were a diagnostic code of G61.8 in the seventh and eighth revision of the Korean Standard Classification of Disease and a >3-month history of oral immunosuppressant use. The age-adjusted incidence rate and prevalence of CIDP in South Korea were also analyzed. Results: CIDP was newly diagnosed in 953 patients during the study period. The mean age at diagnosis was 58.36 years, and the male-to-female ratio was 1.74. The age-adjusted incidence rates were 0.22, 0.21, 0.23, 0.30, and 0.25 per 100,000 person-years in 2016, 2017, 2018, 2019, and 2020, respectively. The age-adjusted prevalence was estimated at 1.16 per 100,000 persons in 2020. Age and the Elixhauser Comorbidity Index were associated with the in-hospital mortality of patients with CIDP. Infection and cardiovascular disease (CVD) were also significantly associated with the in-hospital mortality of those patients. Acute-onset CIDP was initially diagnosed in an estimated 101 out of 953 patients with CIDP. Conclusions The prevalence and incidence rates of CIDP in South Korea were comparable between this nationwide cohort study and previous studies. Common comorbidities such as CVD and diabetes should be appropriately monitored in patients with CIDP to prevent a poor prognosis and socioeconomic burden.

PURPOSE: The use of endovascular aneurysm repair (EVAR) for ruptured abdominal aortic aneurysms (r-AAA) is steadily increasing. We report early experiences of EVAR for r-AAA performed in two tertiary referral centers in Korea. METHODS: We retrospectively reviewed r-AAA patients treated by EVAR from May 2013 to December 2017. An EVAR-first strategy for r-AAA was adopted whenever feasible. The demographic information, anatomic characteristics, operative details, postoperative complications with special attention to abdominal compartment syndrome (ACS), and 30-day mortality were collected and analyzed. RESULTS: We identified 13 patients who underwent EVAR for r-AAA. Mean age was 74.2 years and mean AAA size was 74.2 mm. Two patients underwent cardiopulmonary resuscitation at initial presentation. Bifurcated stent grafts were used in 12 out of 13 cases and physician-modified endografts with fenestrated/chimney techniques were performed in 2 cases with short neck. Successful stent graft deployment was achieved in all cases. Three patients were suspected of having ACS and 2 of them underwent laparotomy for decompression. The 30-day mortality was 7.7% (1 of 13), the only mortality being a patient that refused decompressive laparotomy for suspected ACS. CONCLUSION: Despite the small numbers, the outcomes of EVAR for treatment of r-AAA were very promising, even in selected cases with unfavorable anatomy. These outcomes were achieved by a dedicated and well-trained team approach, and by use of high-end angiographic technology. Finally, ACS after EVAR is not uncommon, and requires a high index of suspicion as well as liberal use of decompressive surgery.
Aneurysm ; Aortic Aneurysm, Abdominal ; Blood Vessel Prosthesis ; Cardiopulmonary Resuscitation ; Decompression ; Endovascular Procedures ; Humans ; Intra-Abdominal Hypertension ; Korea ; Laparotomy ; Mortality ; Neck ; Postoperative Complications ; Retrospective Studies ; Rupture ; Tertiary Care Centers

Neuromyelitis optica spectrum disorder (NMOSD) is generally known as selective involvement of central nervous system. However, in recent years, some evidences have been found that NMOSD invades other peripheral organs. Especially, skeletal muscle involvement of NMOSD has been documented scantily and further studies must be required. Here, we describe a patient who first had generalized fatigue, mild weakness, and myalgia with increased level of serum creatine kinase and was finally diagnosed with myopathy associated with NMOSD.
Central Nervous System ; Creatine Kinase ; Fatigue ; Humans ; Muscle, Skeletal ; Muscular Diseases ; Myalgia ; Myotonia ; Myotonic Disorders ; Neuromyelitis Optica

Purpose: Obesity has been determined to be associated with fat mass and obesity-associated (FTO) gene and thyroid cancer risk. However, the effect of combined interactions between obesity and the FTO gene on thyroid cancer needs further investigation. This study aimed to examine whether interactions between body mass index (BMI) and the FTO gene are associated with an increased risk of thyroid cancer. Materials and Methods: A total of 705 thyroid cancer cases and 705 sex- and age-matched normal controls were selected from the Cancer Screenee Cohort in National Cancer Center, Korea. A conditional logistic regression model was used to calculate the odds ratios (ORs) and 95% confidence intervals (CIs) for the measure of associations and the combined effect of BMI and FTO gene on thyroid cancer. Results: BMI was associated with an increased risk of thyroid cancer in subclasses of overweight (23-24.9 kg/m2; adjusted OR, 1.50; 95% CI, 1.12 to 2.00) and obese (≥ 25 kg/m2) (adjusted OR, 1.62; 95% CI, 1.23 to 2.14). There were positive associations between the FTO genetic variants rs8047395 and rs8044769 and an increased risk of thyroid cancer. Additionally, the combination of BMI subclasses and FTO gene variants was significantly associated with thyroid cancer risk in the codominant (rs17817288), dominant (rs9937053, rs12149832, rs1861867, and rs7195539), and recessive (rs17817288 and rs8044769) models. Conclusion Findings from this study identified the effects of BMI on thyroid cancer risk among individuals carrying rs17817288, rs9937053, rs12149832, rs1861867, rs7195539, and rs8044769, whereas the effects of BMI may be modified according to individual characteristics of other FTO variants.

Background: Prediabetes is a metabolic state between normoglycemia and diabetes and is known to carry a higher risk of developing overt diabetes and cardiovascular disease (CVD). The relative and absolute risks of all-cause mortality, CVD, coronary heart disease, and stroke in prediabetes patients, as well as in diabetic patients, is higher than that in patients with normoglycemia. Carotid intima-media thickness (cIMT) is a method used to stratify CVD risk. In this study, we aimed to determine whether the neutrophil-to-lymphocyte ratio (NLR) correlates with cIMT in prediabetes patients. Methods: From January 1, 2016, to February 20, 2021, 581 adults their 30s–70s who underwent carotid ultrasonography as part of a comprehensive medical examination at the Dongtan Sacred Heart Hospital were enrolled. Statistical analysis using SPSS presented t-test and chi-square test significance levels into a group with normal cIMT (nIMT; cIMT <1 mm) and a group with thick cIMT (tIMT; cIMT ≥1 mm). Binary logistic regression analysis was performed to confirm the correlation between NLR and cIMT. Results: In prediabetic adults, age, hemoglobin A1c (HbA1c), systolic blood pressure, and NLR were significantly higher in the tIMT group than in the nIMT group. In the regression analysis, NLR, age, and HbA1c were significantly correlated with cIMT. Conclusion NLR was significantly higher in the tIMT group than in the nIMT group; therefore, NLR may be used to assess CVD risk in prediabetes patients.

Background: Quantitative evaluation of renal cortical echogenicity (RCE) has been tried anddeveloped in human and veterinary medicine. Objectives: The objective of this study was to propose a method for evaluating RCEquantitatively and intuitively, and to determine associations between ultrasonographic renalstructural distinction and estimated glomerular filtration rate (eGFR) in canine chronickidney disease (CKD). Methods: Data were collected on 63 dogs, including 27 with normal kidney function and 36CKD patients. Symmetric dimethylarginine and creatinine concentrations were measuredfor calculating eGFR. RCE was evaluated as 3 grades on ultrasonography images accordingto the distinction between the renal cortex and outer medulla. The RCE grade of each kidneywas measured. Results: There was a significant difference in eGFR between the group normal and CKD (p< 0.001). As mean of RCE grades (the mean values of each right and left kidney's RCE grade)increases, the proportion of group CKD among the patients in each grade increases (p <0.001). Also, severity of RCE (classified as "high" if any right or left kidney evaluated as RCEgrade 3, "low" otherwise) and eGFR is good indicator for predicting group CKD (p < 0.001). Conclusions The degree of distinction between the renal cortex and the outer medulla isclosely related to renal function including eGFR and the RCE grade defined in this study canbe used as a method of objectively evaluating RCE.

Purpose: Cyst excision with hepaticojejunostomy has been the classic procedure for treating choledochal cysts, and the use of laparoscopic treatment has been favored recently. The purpose of this study was to compare the long-term biliary complication of laparoscopic operation with open surgery for choledochal cyst presenting in children. Methods: A retrospective study comparing the laparoscopic and open procedures was performed in 185 patients with choledochal cyst in a single children’s hospital. There were 109 patients who were operated with open surgery, and 76 patients operated with laparoscopic surgery. The primary outcome was long-term biliary complications and the secondary outcome included operative time, intraoperative transfusion, length of hospital stay, and other late postoperative complications. Results: In the patient’s demographics, there was no significant difference between the 2 groups. Notably, it was shown that the operative time was longer in the laparoscopic group. The number of patients requiring blood transfusion intraoperatively was lower in the laparoscopic group. It was noted that the hospital stay was not statistically different. The duration to resumption of diet and duration of drainage were longer in the laparoscopic group. Biliary complications were shown to be significantly higher in the open group. The risk factor for long-term biliary complications was noted with the intraoperative transfusion. Conclusion The use of a laparoscopic choledochal cyst excision with hepaticojejunostomy is a safe and feasible technique in a young patient. The long-term biliary complication was lower compared to open surgery, rendering this a good option for pediatric patients.