A Case of Merkel Cell Polyomavirus-Negative Merkel Cell Carcinoma Concurrent with Squamous Cell Carcinoma / 대한피부과학회지

Merkel cell carcinoma (MCC), a rare cutaneous neuroendocrine carcinoma, is most common in sun-exposed areas of aged individuals. Merkel cell polyomavirus (MCPyV) is one causative agent of MCC. Cases of MCC concurrent with other skin tumors, especially squamous cell carcinoma, are rarely reported. Immunohistochemical staining is performed using antibodies to the MCPyV large-T antigen (CM2B4) only in select cases. To date, no cases of MCPyV have been reported in Korea. Here we report a case of MCC concurrent with squamous cell carcinoma in an aged man and discuss the pathogenesis of the case through CM2B4 staining.

A Case of Perineal Hemangioma, External Genitalia Malformations, Lipomyelomeningocele, Vesicorenal Abnormalities, Imperforate Anus, and Skin Tag (PELVIS) Syndrome with Extensive Perineal Infantile Hemangioma

PELVIS syndrome describes the constellation of perineal hemangioma, external genitalia malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, and skin tag. A 2-month-old girl presented with infantile hemangioma on her perineum and genitalia with imperforate anus, rectovaginal fistula and perineal skin tag at birth. Under the impression of PELVIS syndrome, consequential spinal sonography was conducted and revealed an intrasacral meningocele without clinical neurologic deficit. The anorectal malformation was surgically corrected, she was taking oral propranolol for the cutaneous lesion, and she showed improvement and no complications.

Gastric Mixed Adenoneuroendocrine Carcinoma with Revised Diagnosis after Retrospective Pathologic Review

Gastric mixed adenoneuroendocrine carcinoma (MANEC) is an entity defined by World Health Organization in 2010, which is composed of adenocarcinoma and neuroendocrine neoplasm. This is a very rare type of tumor in the stomach, which is estimated to be one to two per one million people per year on gastrointestinal track. In general, as MANEC often shows a poor prognosis, early diagnosis of the disease is important for achieving a favorable outcome. However, due to its rarity and histological heterogeneity, it is hard to suspect this disease initially, and sometimes this tumor is misdiagnosed as adenocarcinoma. The present case was a 76-year-old man who was initially diagnosed with gastric tubular adenocarcinoma without distant metastasis. Only 1 month after a curative surgery, multiple liver metastases were newly detected. Retrospective pathologic review resulted in a revised diagnosis as a gastric MANEC, and the patient underwent palliative systemic chemotherapy to achieve partial response.

A Fibroma of Tendon Sheath Causing Carpal Tunnel Syndrome:A Case Report of an Atypical Clinical Presentation

Fibroma of the tendon sheath is a benign slow-growing fibrous tumor. Although rare, cases occurring in the upper extremities usually involve the fingers. It appears as a well-defined, roundor oval-shaped mass originating from the flexor tendon. Abundant fibrous stroma makes fibromas appear as a low intensity mass in all MRI sequences. Most of the fibromas manifest as painless soft tissue masses. Herein, we report a case of fibroma of the tendon sheath with an unusual clinical presentation, triggering carpal tunnel syndrome during wrist movement.

A Case of Vulvar Melanosis Associated with Lichen Sclerosus et Atrophicus / 대한피부과학회지

Vulvar melanosis (VM) is clinically characterized by asymmetric macules or patches of varying size with a tan to black color and irregular borders. VM is more commonly found among perimenopausal women with a median age of 42 years. The exact pathogenesis of VM is not well understood. The proposed etiological factors for VM include oral contraceptive use, infection with human papillomavirus, and chronic inflammatory skin diseases such as lichen sclerosus et atrophicus. Histologic examination can easily differentiate mucosal lentiginosis from melanoma, but VM superimposed on a background of anogenital lichen sclerosus, both clinically and histologically, may mimic malignant melanoma. There have been two cases of VM associated with Dowling-Degos disease in the Korean dermatologic literature. To the best of our knowledge, this is the first case of VM associated with lichen sclerosus et atrophicus in the Korean dermatologic literature.

The Efficacy and Safety of the Topical Application of Poly-Gamma Glutamic Acid Hydrogel Nanoparticle-Based Fermented Extract of a Medicinal Plant Solution for Androgenetic Alopecia: A Randomized Double-Blinded Placebo-Controlled Clinical Trial / 대한피부과학회지

Background@#Androgenetic alopecia (AGA) is the most common cause of hair loss in adults. Topical application of the fermented extract of medicinal plants promoted hair growth through stimulation of the telogen to anagen transition in a mouse model. However, controlled studies are necessary to evaluate the efficacy of the topical application of the fermented extract solution in humans. @*Objective@#We aimed to assess the efficacy and safety of topical poly-gamma glutamic acid hydrogel nanoparticlebased fermented extract of a medicinal plant solution (PGA solution) in terms of the clinical improvements in patients with AGA. @*Methods@#This 24-week, randomized, double-blinded, placebo-controlled, single-center clinical trial enrolled 50 patients with AGA (36 men and 14 women); the participants were treated with either PGA solution or placebo twice daily for 24 weeks. Efficacy was evaluated by a phototrichogram (total hair count per 1 cm 2 ), patient’s subjective assessments, and investigator’s subjective assessments of clinical improvements in overall hair regrowth. Any adverse reactions were reported during the study. @*Results@#At week 24, compared with baseline, the PGA solution group showed a significantly increased hair density compared to the placebo group (7.24±2.52/cm 2 vs. 1.53±1.39/cm 2 , p＜0.0001). The average hair density after 24 weeks was 129.90±23.04/cm 2 and 115.32±20.35/cm 2 in the PGA solution group and the placebo group, respectively.There was no adverse reaction over the 24-week period. @*Conclusion@#PGA solution may provide a safe and effective adjunctive treatment for patients with AGA.

A Case of Syringoid Eccrine Carcinoma on the Shoulder / 대한피부과학회지

Syringoid eccrine carcinoma (SEC) is a rare cutaneous malignant tumor thought to be derived from eccrine sweat glands. It is usually present in the scalp and face and often occurs in the fourth to seventh decades of life. A 76-year-old female patient visited our department with a 3-year history of a lesion showing a 5×4 cm-sized erythematous firm plaque with ulceration on her right shoulder. Histological findings revealed a tumor consisting of numerous proliferating tubular structures with two layers of basaloid cells with cellular atypia. Some ductal structures showed a tadpole appearance. Based on these findings, the final diagnosis was SEC. The patient was treated with slow Mohs micrographic surgery and a full-thickness skin graft and did not show any recurrence during the follow-up period of 6 months. Herein, we report a very rare case of a 76-year-old woman diagnosed with SEC that developed on the right shoulder.

A Case of Vulvar Melanosis Associated with Lichen Sclerosus et Atrophicus / 대한피부과학회지

Vulvar melanosis (VM) is clinically characterized by asymmetric macules or patches of varying size with a tan to black color and irregular borders. VM is more commonly found among perimenopausal women with a median age of 42 years. The exact pathogenesis of VM is not well understood. The proposed etiological factors for VM include oral contraceptive use, infection with human papillomavirus, and chronic inflammatory skin diseases such as lichen sclerosus et atrophicus. Histologic examination can easily differentiate mucosal lentiginosis from melanoma, but VM superimposed on a background of anogenital lichen sclerosus, both clinically and histologically, may mimic malignant melanoma. There have been two cases of VM associated with Dowling-Degos disease in the Korean dermatologic literature. To the best of our knowledge, this is the first case of VM associated with lichen sclerosus et atrophicus in the Korean dermatologic literature.

Spontaneous Intracranial Hypotension Followed by Subdural Hemorrhage

No abstract available.

Numbers of Stroke Patients and Stroke Subtypes According to Highest and Lowest Daily Temperatures in Seoul

BACKGROUND AND PURPOSE: There is conflicting evidence for whether or not the incidence of stroke is influenced by the daily temperature. The association between daily temperature and incidence of stroke is largely unknown in Korea. This study attempted to evaluate whether the maximum or minimum daily temperature is associated with increased numbers of strokes and stroke subtypes among Seoul residents. METHODS: We obtained the maximum and minimum daily temperatures in Seoul from the Korean Meteorological Administration between January 2005 and December 2014. Consecutive patients with acute stroke were registered who visited the emergency room or outpatient clinic in Soonchunhyang University Hospital, Seoul. The residential addresses of cases were restricted to within a 2-kilometer radius of this hospital. The stroke events were prospectively recorded with onset time, and were classified by subtypes. The categories of daily temperature were divided by 10℃ from the mean temperature. The mean daily number of strokes was calculated during the study period. One-way analysis of variance and Duncan's post-hoc test were applied to compare the number of strokes among the temperature groups. RESULTS: In total, 2,313 acute strokes were identified during the period: 1,643 ischemic strokes and 670 hemorrhagic strokes. The number of cases was significantly higher when the maximum daily temperature was >32℃ or ≤3℃ (p=0.048) or the minimum daily temperature was ≤-11.0℃ (p=0.020). The lowest maximum daily temperature was associated with increased instances of intracerebral hemorrhage (p=0.029) and small-vessel occlusion (p=0.013), while the highest maximum daily temperature was associated with an increased instance of large-artery atherosclerosis (p=0.045). CONCLUSIONS: The daily temperature had measurable and different associations with the number of strokes and strokes subtypes in Seoul, Korea.

ATAD2 as a Poor Prognostic Marker for Hepatocellular Carcinoma after Curative Resection / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Cancer cells frequently express genes that are specifically or preferentially expressed in male germ cells under normal conditions. The ATPase family AAA domain-containing 2 (ATAD2) is one such and works as an important cofactor for MYC-dependent transcription. In hepatocellular carcinoma (HCC), ATAD2 has been identified as a candidate driver gene located within the amplified 8q24 locus. However, the prognostic significance of ATAD2 protein expression in HCC remains uncertain. MATERIALS AND METHODS: We investigated ATAD2 protein expression by immunohistochemistry in tumor tissue from 182 HCC patients who underwent curative resection. Associations of ATAD2 expression with clinicopathologic variables or prognosis of HCC patients were analyzed. RESULTS: ATAD2 expression was observed in 119 (65.4%) of the 182 HCCs and tended to be independent predictor of early recurrence (p=0.059). ATAD2 expression showed an unfavorable influence on recurrence-free survival (RFS) (p < 0.001). Subgroup analysis among patients with tumor size < or = 5.0 cm (n=109), patients at Barcelona Clinic Liver Cancer stage 0 or A (n=92), and patients with alpha-fetoprotein < or = 20 ng/mL (n=61), the ATAD2-positive groups unfavorably influenced RFS (p=0.008, p=0.009, and p=0.013, respectively). In addition, ATAD2 expression was an independent predictor of shorter RFS (p=0.002). ATAD2 expression showed an unfavorable influence on disease-specific survival (p=0.001), but was not an independent predictor of shorter disease-specific survival (p=0.109). CONCLUSION: ATAD2 protein expression may be a potential predictor of RFS in HCC patients after curative resection and ATAD2 may have prognostic value in patients with early stage HCC or normal serum alpha-fetoprotein level.

Relationship between Tetrahydrobiopterin and Portal Hypertension in Patients with Chronic Liver Disease

Tetrahydrobiopterin (BH4) is an essential cofactor in NO synthesis by endothelial nitric oxide synthase (eNOS) enzymes. It has been previously suggested that reduced intrahepatic BH4 results in a decrease in intrahepatic NO and contributes to increased hepatic vascular resistance and portal pressure in animal models of cirrhosis. The main aim of the present study was to evaluate the relationship between BH4 and portal hypertension (PHT). One hundred ninety-three consecutive patients with chronic liver disease were included in the study. Liver biopsy, measurement of BH4 and hepatic venous pressure gradient (HVPG) were performed. Hepatic fibrosis was classified using the Laennec fibrosis scoring system. BH4 levels were determined in homogenized liver tissues of patients using a high performance liquid chromatography (HPLC) system. Statistical analysis was performed to evaluate the relationship between BH4 and HVPG, grade of hepatic fibrosis, clinical stage of cirrhosis, Child-Pugh class. A positive relationship between HVPG and hepatic fibrosis grade, clinical stage of cirrhosis and Child-Pugh class was observed. However, the BH4 level showed no significant correlation with HVPG or clinical features of cirrhosis. BH4 concentration in liver tissue has little relation to the severity of portal hypertension in patients with chronic liver disease.

The usefulness of non-invasive liver stiffness measurements in predicting clinically significant portal hypertension in cirrhotic patients: Korean data

BACKGROUND/AIMS: Liver stiffness measurement (LSM) has been proposed as a non-invasive method for estimating the severity of fibrosis and the complications of cirrhosis. Measurement of the hepatic venous pressure gradient (HVPG) is the gold standard for assessing the presence of portal hypertension, but its invasiveness limits its clinical application. In this study we evaluated the relationship between LSM and HVPG, and the predictive value of LSM for clinically significant portal hypertension (CSPH) and severe portal hypertension in cirrhosis. METHODS: LSM was performed with transient elastography in 59 consecutive cirrhotic patients who underwent hemodynamic HVPG investigations. CSPH and severe portal hypertension were defined as HVPG > or =10 and > or =12 mmHg, respectively. Linear regression analysis was performed to evaluate the relationship between LSM and HVPG. Diagnostic values were analyzed based on receiver operating characteristic (ROC) curves. RESULTS: A strong positive correlation between LSM and HVPG was observed in the overall population (r2=0.496, P or =10 mmHg) was 0.851, and the sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) for an LSM cutoff value of 21.95 kPa were 82.5%, 73.7%, 86.8%, and 66.7%, respectively. The AUROC at prediction of severe portal hypertension (HVPG > or =12 mmHg) was 0.877, and the sensitivity, specificity, PPV, and NPV at LSM cutoff value of 24.25 kPa were 82.9%, 70.8%, 80.6%, and 73.9%, respectively. CONCLUSIONS: LSM exhibited a significant correlation with HVPG in patients with cirrhosis. LSM could be a non-invasive method for predicting CSPH and severe portal hypertension in Korean patients with liver cirrhosis.

A patient with acquired hemophilia A induced by clopidogrel / 대한혈액학회지

Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against factor VIII (FVIII). Treatment with clopidogrel is a cause of AHA, but its clinical course is unknown. Recently, we treated a 65-year-old man who was hospitalized for cerebellar infarction and had a prolonged activated partial thromboplastin time (aPTT) with soft tissue oozing after 3 weeks of clopidogrel use. We terminated clopidogrel administration and transfused the patient with fresh frozen plasma. However, the aPTT increased up to 98.8 seconds, and the FVIII and FVIII inhibitor levels were <1% and 5.4 Bethesda units/mL, respectively. Clopidogrel-associated AHA was considered, and we began steroid treatment. Two months later, FVIII, FVIII inhibitor, and aPTT values were normalized. No further bleeding or aPTT prolongation has been reported during the 2-year follow-up period. AHA should be considered in patients taking clopidogrel and experiencing bleeding, unless the platelet count and coagulation screen are normal.

A Rare Case of Pericardial Actinomycosis

Actinomycosis is a common chronic suppurative and granulomatous infection caused by anaerobic or microphilic bacteria primarily from the genus Actinomyces. However, Actinomyces is a rare cause of pericarditis. We experienced a rare case of pericardial actinomycosis. A previously healthy 44-year-old man presented with 3 days of fever, chest pain, and clinical signs of congestive heart failure. Chest computed tomography showed pericardial effusion, pericardial thickening, and bilateral pleural effusion. A subxiphoidpericardiotomy was performed, and a histological specimen was taken from the pericardium. A histological section of the pericardium showed an actinomycotic granule (sulfur granule). His symptoms and signs improved after administration of piperacillin/tazobactam and steroids. He was uneventfully discharged on oral amoxicillin/clavulanate. He recovered fully with no recurrence after six months of follow-up.

A Case of Renal Salt Wasting Syndrome Induced by Cisplatin for Chemotherapy / 대한신장학회지

Cisplatin is widely used for chemotherapy, but known to cause renal, auditory, hematologic, gastrointestinal, and neurologic toxicities. Hyponatremia after administration of cisplatin is related to renal tubular sodium excretion. A 71-year-old female was referred to our hospital for chemotherapy of laryngeal cancer. On admission, the patient's laboratory data were normal. The patient received for 3 days chemotherapy without complication, but presented mental confusion on the 4th hospital day. The laboratory findings were as follows; serum sodium was 118 mmol/L, urine sodium 163 mmol/L, serum osmolality 248 mmol/kg, and urine osmolality 594 mmol/kg. On physical exam, volume status was hypovolemic, so we supplied hypertonic and isotonic salines. On the 9th hospital day, she showed normal sodium concentration and clear consciousness. After chemotherapy, we should make differential diagnosis between SIADH (syndrome of inappropriate antidiuretic hormone) and renal salt wasting syndrome according to the physical examination. We report a case of renal salt wasting syndrome with severe mental change after chemotherapy using cisplatin.

Gender Differences in the Functional Recovery after Acute Stroke

BACKGROUND AND PURPOSE: PurposezzInterest in gender differences in the effects of acute stroke is growing worldwide. However, gender differences in functional recovery after acute stroke in the Korean population have yet to be evaluated. The aim of this investigation was to compare long-term functional outcomes between male and female after acute stroke. METHODS: Patients with acute stroke were enrolled prospectively between January 2005 and January 2009. Baseline characteristics, risk factors, stroke subtypes, time delay from onset to arrival at a hospital, active treatment, and stroke severity were compared between male and female. Differences in mortality and disability at discharge, and at 3 months and 1 year after stroke onset were also investigated. Functional disabilities were categorized into two groups: good outcome (score on modified Rankin scale 2). RESULTS: Among 1,055 patients with acute stroke, 575 were male (aged 64.83+/-11.98 years, mean+/-SD) and 480 were female (aged 70.09+/-13.02 years). There were no gender differences in mortality at 3 months and 1 year after stroke. The frequency of poor outcomes was higher in female patients than in male patients at discharge (39.8% versus 30.9%, respectively; p=0.003), the 3 months follow-up (32.3% versus 20.8%, respectively; p<0.001), and the 1 year follow-up (31.1% versus 18.7%, respectively; p=0.001). After adjusting for multiple confounding factors including age and stroke severity, the female gender persisted as a predictor of poor functional outcome at 3 months and 1 year after stroke. CONCLUSIONS: Female patients have greater difficulty than male patients in recovering from a disabled state after acute stroke. Future studies should investigate the causes of this gender difference.

Gender Differences in the Functional Recovery after Acute Stroke

BACKGROUND AND PURPOSE: PurposezzInterest in gender differences in the effects of acute stroke is growing worldwide. However, gender differences in functional recovery after acute stroke in the Korean population have yet to be evaluated. The aim of this investigation was to compare long-term functional outcomes between male and female after acute stroke. METHODS: Patients with acute stroke were enrolled prospectively between January 2005 and January 2009. Baseline characteristics, risk factors, stroke subtypes, time delay from onset to arrival at a hospital, active treatment, and stroke severity were compared between male and female. Differences in mortality and disability at discharge, and at 3 months and 1 year after stroke onset were also investigated. Functional disabilities were categorized into two groups: good outcome (score on modified Rankin scale 2). RESULTS: Among 1,055 patients with acute stroke, 575 were male (aged 64.83+/-11.98 years, mean+/-SD) and 480 were female (aged 70.09+/-13.02 years). There were no gender differences in mortality at 3 months and 1 year after stroke. The frequency of poor outcomes was higher in female patients than in male patients at discharge (39.8% versus 30.9%, respectively; p=0.003), the 3 months follow-up (32.3% versus 20.8%, respectively; p<0.001), and the 1 year follow-up (31.1% versus 18.7%, respectively; p=0.001). After adjusting for multiple confounding factors including age and stroke severity, the female gender persisted as a predictor of poor functional outcome at 3 months and 1 year after stroke. CONCLUSIONS: Female patients have greater difficulty than male patients in recovering from a disabled state after acute stroke. Future studies should investigate the causes of this gender difference.

The Outcome and Propriety of Surgical Treatment of Colorectal Cancer in the Elderly

PURPOSE: As life expectancy increases, elderly colorectal cancer patients are also increasing. Compared to younger patients, the elderly manifest higher co-morbidity with more advanced and emergent disease. However, recent studies have reported similar surgical approaches irrespective of age distribution. We evaluated the outcome and propriety of surgical treatment of colorectal cancer in the elderly. METHODS: The medial records of 464 colorectal patients, who underwent surgery during 2003 to 2007 in Ewha Womans University Mokdong Hospital were reviewed retrospectively. The patients were divided into three groups according to age: I (younger than 70), II (71~80), III (older than 81). Clinical and histological characteristics, surgical outcomes and survival rates were analyzed. RESULTS: Three hundred and thirty-eight patients belonged to group I, and 104 patients to group II, and group III included 22 patients. Although, male patients were more prevalent in all three groups, female distribution was slightly higher in group III. Clinical characteristics among the three groups did not reveal specific differences except TNM stage distribution. In group I and II, patients with stage II were more common compared to group III, whereas the latter showed most frequently stage III. Histological characteristics and postoperative morbidity rates did not show any difference among the three groups. The survival rate was lowest in group III. However, emergency operation was more frequent in group III, in accordance with increased postoperative complications. CONCLUSION: The elderly demonstrated comparable operative morbidity and mortality to the younger patients. Emergency operation was the only significantly influencing factor in the surgical outcome. Therefore, in colorectal cancer patients, surgical treatment in the elderly should be no longer contraindicated.