Blood-brain barrier breakdown in reversible cerebral vasoconstriction syndrome: Implications for pathophysiology and diagnosis.

OBJECTIVE: Diagnosis of reversible cerebral vasoconstriction syndrome (RCVS) is currently based on luminographic findings of vasoconstriction. In addition to vasoconstriction, the blood-brain barrier (BBB) breakdown has been postulated as a central mechanism of RCVS. Our aim was to document BBB breakdown in patients with RCVS and its role for the pathophysiology-based diagnosis of RCVS. METHODS: We prospectively recruited 72 consecutive patients with thunderclap headache who did not have aneurysmal subarachnoid hemorrhage from April 2015 to July 2016 at the Samsung Medical Center. Based on the International Classification of Headache Disorders-3 beta criteria and neuroimaging, patients were classified as having RCVS (n = 41; "definite" in 29 imaging-proven patients and "probable" in 12 imaging-negative patients), other secondary causes (n = 7), and thunderclap headache of undetermined cause (n = 24). BBB breakdown was evaluated using contrast-enhanced fluid-attenuated inversion recovery magnetic resonance imaging. RESULTS: BBB breakdown was documented in 20 (69.0%) patients with definite RCVS, 3 (25.0%) patients with probable RCVS, and none with other secondary causes. BBB breakdown was present in RCVS patients with (n = 4) and without (n = 19) concomitant posterior reversible encephalopathy syndrome. In patients with RCVS, the extent of BBB breakdown was independently associated with neurological complications (multivariate odds ratio = 1.48 per 1 territorial increase, 95% confidence interval = 1.04-2.12, adjusted p = 0.032). Three (12.5%) patients with thunderclap headache of undetermined cause were newly classified as having RCVS by the presence of BBB breakdown. INTERPRETATION: This is the first study to show BBB breakdown in patients with RCVS. This finding might broaden our understanding of the pathophysiology and clinical spectrum of RCVS. Ann Neurol 2017;81:454-466.

Chronic paroxysmal headache secondary to an orbital metastatic leiomyosarcoma: A case report.

Background Intraorbital lesions associated with symptomatic trigeminal autonomic cephalalgias (TACs) are rarely reported. We present a case of orbital metastatic leiomyosarcoma, presenting with chronic paroxysmal hemicrania-like headache. Case report A 43-year-old man presented with a severe paroxysmal headache in his left periocular and frontal area for a year. The attacks occurred 10-12 times per day, lasting 10-15 minutes with ipsilateral lacrimation and conjunctival injection. Neurological examination and brain MRI without contrast were unremarkable. Different medications were tried, without beneficial effects. A follow-up contrast-enhanced brain MRI performed one year after the baseline MRI revealed an enhancing mass in the left superior oblique muscle. Orbital metastatic leiomyosarcoma arising from the thigh was revealed. He received gamma knife surgery, which completely resolved the headache. Discussion Intraorbital lesion should be considered a possibility in patients with headache mimicking TACs. Baseline contrast-enhanced MRI is essential, and repeated MRI scans might be needed if clinically indicated.

Serial testing of the ICHD-3 beta diagnostic criteria for probable reversible cerebral vasoconstriction syndrome: A prospective validation study.

Objectives To serially test the International Classification of Headache Disorders (ICHD) 3rd edition beta version criteria for 6.7.3.1 probable reversible cerebral vasoconstriction syndrome (probable RCVS) in patients with thunderclap headache. Methods We prospectively screened consecutive patients with thunderclap headache who visited the Samsung Medical Center between October 2015 and March 2017. Patients were included in the analysis if they a) visited our hospital within 1 month after onset, b) completed a diagnostic work-up, and c) had no secondary causes other than RCVS. The ICHD-3 beta 6.7.3.1 criteria were evaluated serially during the first visit (visit 1), 2 weeks after the first visit (visit 2), and 1-3 months after onset (visit 3). Results A total of 99 patients completed this study. Based on the first clinical and radiological evaluation, 63 (63.6%) were diagnosed with angiogram-proven RCVS, whilst 36 (36.4%) showed normal neuroimaging. The ICHD-3 beta 6.7.3.1 criteria were fulfilled in 76.2% of patients with angiogram-proven RCVS. In patients with normal angiograms, the ICHD diagnosis of probable RCVS changed over time: 25.0%, 47.2%, and 38.9% at visits 1, 2, and 3, respectively. The sensitivity and specificity of the criteria at visit 1 were 72.4% and 95.5%, respectively, for the prediction of a final diagnosis of overall (angiogram-proven + probable) RCVS. Conclusions The ICHD-3 beta criteria for probable RCVS are applicable for patients with thunderclap headaches at the acute stage, with the exception of criterion C3. Criteria C1 and C2 are less reliable when applied at the first visit. Repeated evaluation is necessary to enhance diagnostic sensitivity.

Characteristics and demographics of reversible cerebral vasoconstriction syndrome: A large prospective series of Korean patients.

Objectives To report demographics and characteristics of reversible cerebral vasoconstriction syndrome (RCVS) in the Korean cohort. Methods We prospectively recruited patients with definite (imaging-proven) RCVS and probable (imaging-negative) RCVS who visited Samsung Medical Center between June 2012 and September 2016. Clinical manifestations, neuroimaging, treatment, and clinical outcomes were evaluated in all patients. Characteristics of RCVS without typical causes ("idiopathic RCVS") were compared with those of RCVS with identifiable causes ("secondary RCVS"). International Classification of Headache Disorders (ICHD)-3 beta criteria for 6.7.3 RCVS and 6.7.3.1 probable RCVS were tested. Results A total of 138 patients (104 definite and 34 probable RCVS) were included in this study. Patients with definite RCVS were predominantly female (85.6%) and middle-aged (mean, 50.7 [range, 23-82] years). Probable RCVS was associated with less female predominance (70.6%, p = 0.049), more typical manifestations ( p < 0.001), and none of neurological complications. One-hundred and one (97.1%) patients with definite RCVS had headache, but the typical "recurrent and/or triggered" thunderclap headache was reported in only 83 (82.2%). In most patients with definite RCVS (84.6%), RCVS was idiopathic, while only 16 (15.4%) had secondary causes. Compared to those with secondary RCVS, patients with idiopathic RCVS were older (52.8 ± 11.42 vs. 39.1 ± 9.55 years, p < 0.001). Patients with secondary RCVS had more complications than those with idiopathic RCVS (40.5% vs. 12.5%, p = 0.018). Among idiopathic RCVS patients, 33 (37.5%) reported a preceding event or a change in lifestyle, environment, health, or medication within one month before onset. Conclusion In our cohort, RCVS was benign and idiopathic in most patients, and occurred frequently in middle-aged women. Manifestations of RCVS were more diverse than previously recognized, and forms without any headache existed. Different genetic, social, and environmental factors should be taken into account to unveil the spectrum and pathophysiology of RCVS.

Intracranial structural alteration predicts treatment outcome in patients with spontaneous intracranial hypotension.

Background Intracranial structural dislocation in spontaneous intracranial hypotension (SIH) can be measured by various intracranial angles and distances. We aimed to identify the clinical significance of structural dislocation in relation to treatment outcome in patients with SIH. Methods In this retrospective analysis, we identified patients with SIH who received an epidural blood patch (EBP) at Samsung Medical Center from January 2005 to March 2015. Structural dislocation in pretreatment MRIs of SIH patients was assessed by measuring tonsillar herniation, mamillopontine distance, the angle between the vein of Galen and straight sinus (vG/SS angle), the pontomesencephalic angle, and the lateral ventricular angle. After the first EBP, poor response was defined as the persistence of symptoms that prompted a repeat EBP. Results Out of the 95 patients included, 31 (32.6%) showed poor response. Among the radiological markers of structural dislocation, the vG/SS angle was associated with poor response (49.82 ± 16.40° vs 66.58 ± 26.08°, p = 0.002). Among clinical variables, premorbid migraine ( p = 0.036) was related to poor response. In multivariate analysis, reduced vG/SS angle was independently associated with poor response (OR 1.04 [95% CI 1.01 - 1.07] per 1° decrease, p = 0.006). In 23 patients who underwent MRI after successful treatment, the vG/SS angle significantly increased after the EBP ( p < 0.001, by paired t-test), while two patients with aggravation or recurrence showed a further reduction of their vG/SS angles. Conclusions Intracranial structural dislocation, measured by the vG/SS angle, is associated with poor response to the first EBP in patients with SIH. Successful treatment can reverse the structural dislocation.

Natural course of untreated cluster headache: A retrospective cohort study.

Objective To determine the natural course of cluster headache. Methods We screened patients with cluster headache who were diagnosed at Samsung Medical Center and lost to follow-up for ≥5 years. Eligible patients were interviewed by phone about the longitudinal changes in headache characteristics and disease course. Remission was defined as symptom-free 1) for longer than twice the longest between-bout period and 2) for ≥5 years. Results Forty-two patients lost to follow-up for mean 7.5 (range, 5.0-15.7) years were included. The length of the last bout did not differ from the first one, while the last between-bout period was longer than the first one ( p = 0.012). Characteristics of cluster headache decreased over time: Side-locked unilaterality (from 92.9% to 78.9%), seasonal and circadian rhythmicity (from 63.9% to 60.9% and from 62.2 to 40.5%, respectively), and autonomic symptoms (from 95.2% to 75.0%). Remission occurred in 14 (33.3%) patients at a mean age of 42.3 (range, 27-65) years, which was not different from the age of last bouts in active patients ( p = 0.623). There was a trend for more seasonal and circadian predilection at baseline in the active group ( p = 0.056 and 0.063, respectively) and fewer lifetime bouts and shorter disease duration in patients in remission ( p = 0.063 and 0.090). Conclusions This study first shows the natural courses of cluster headache. Features of cluster headache become less prominent over time. Remission occurred regardless of age. Although no single predictor of remission was found, our data suggest that remission of cluster headache might not be a consequence of more advanced age, longer duration of disease, or accumulation of lifetime bouts.

Longitudinal changes in cerebral blood flow velocities in different clinical courses of migraine.

Objective To assess longitudinal changes in cerebral blood flow velocities (ΔCBFVs) according to the clinical course of migraine. Methods We retrospectively included migraine patients with two or more attacks per month at baseline who were followed up within 2 years with transcranial Doppler in a tertiary headache clinic. ΔCBFVs were analyzed in relation to clinical courses, defined as remission (0-1 headache days/month), persistence (2-14/month), or progression (≥15/month) in episodic migraine (EM), and conversion to EM (<15/month) and persistence (≥15/month) in chronic migraine (CM). Results A total of 166 patients (90 EM and 76 CM) were included. In EM, the remission group ( n = 30) showed a decrease in CBFV in the middle cerebral artery (MCA) and the basilar artery (BA). The progression group ( n = 10) showed increasing CBFVs in the bilateral MCAs. Patients with the persistence course ( n = 50) showed generally unchanged CBFVs. In CM, ΔCBFVs decreased in the BA and increased in the posterior cerebral artery (PCA) after conversion to EM ( n = 61), whereas they remained unchanged in the persistence group ( n = 15). In all patients, % change in headache days was positively correlated with the %ΔCBFVs of the bilateral MCAs and the BA. Conclusions CBFV changes are associated with the different clinical courses of migraine. The association is more prominent in EM than CM.

Cerebral endothelial dysfunction in reversible cerebral vasoconstriction syndrome: a case-control study.

BACKGROUND: The aim of this study is to investigate cerebral endothelial dysfunction in patients with reversible cerebral vasoconstriction syndrome (RCVS). METHODS: We prospectively recruited patients with RCVS, age-matched controls with episodic migraine, and age-matched healthy controls at Samsung Medical Center from Apr 2015 to Jul 2016. All participants underwent transcranial Doppler evaluation, with a breath-holding maneuver, for the evaluation of bilateral middle cerebral arteries (MCAs), posterior cerebral arteries (PCAs), and the basilar artery (BA). The breath-holding index (BHI) was used to measure cerebral endothelium-dependent vasodilation. Follow-up BHIs were recorded in selected patients with RCVS after 3 months. RESULTS: A total of 84 subjects were recruited for this study (n = 28 in each group of RCVS, episodic migraine, and healthy control; mean age, 49.8 years). The RCVS group showed lower BHIs in all basal arteries, in comparison to healthy controls (p < 0.001, 0.009 for bilateral MCAs, p < 0.001 and 0.028 for bilateral PCAs, and p = 0.060 for the BA). Compared to migraineurs, RCVS patients had lower BHIs only in the anterior circulation (p = 0.002 and 0.038 for bilateral MCAs; p = 0.069 and 0.247 for bilateral PCAs; p = 0.120 for the BA). Of the 10 patients who had follow-up BHIs at 3 months, 7 showed complete normalization, while three did not. CONCLUSIONS: Cerebral endothelial function is impaired in a widespread distribution in RCVS. Its role in the pathogenesis and clinical outcome of RCVS should be determined in further studies.

Clinical patterns of primary stabbing headache: a single clinic-based prospective study.

BACKGROUND: The clinical features and disease courses of primary stabbing headache (PSH) are diverse. We aimed to identify distinct clinical patterns of PSH. METHODS: We prospectively screened consecutive first-visit patients who presented with stabbing headache at the Samsung Medical Centre Headache Clinic from June 2015 to March 2016. Demographics, headache characteristics, and disease courses were prospectively evaluated. After discerning factors related to the chronicity at the time of presentation, clinical patterns were identified based on the frequency (daily vs. intermittent), clinical course (remitted or not), and total disease duration (<3 or >3 months). RESULTS: In the 65 patients with PSH included in this study, monophasic (n = 31), intermittent (n = 17), and chronic daily (n = 12) patterns were identified. The median disease durations were 9 days for monophasic PSH, 9 months for chronic daily PSH, and 2 years for intermittent PSH. The features of monophasic PSH were greater severity, single and side-locked locations, more attacks per day, daily occurrence, and good treatment response. Chronic daily PSH was associated with female predominance, longer-lasting stabs, and multiple or migrating locations on bilateral or alternating sides. The characteristics of intermittent PSH included female predominance and sporadic stabs with less intensity. CONCLUSIONS: Our study demonstrated distinct clinical patterns of PSH. In addition to help early recognition of disease, our findings suggest different pathophysiologic mechanisms. Future prospective studies are required to reveal the etiologies of these different PSH patterns and their optimal treatment strategies.

Caffeine discontinuation improves acute migraine treatment: a prospective clinic-based study.

BACKGROUND: Caffeine has both excitatory and vasoconstrictive effects on central nervous system. Caffeine use might be associated with development and chronification of migraine. We aimed to evaluate the effect of caffeine cessation on the acute treatment of migraine. METHODS: We prospectively recruited migraine patients who consumed caffeine drinks daily and instructed them to discontinue their caffeine intake. Triptans were prescribed for acute treatment. Patients were followed up after at least two weeks after screening and evaluated the efficacy of acute treatment with the migraine assessment of current therapy (Migraine-ACT) questionnaire. Excellent efficacy was defined as Migraine-ACT score of 4. Chronic migraine, body mass index, allodynia, depression, anxiety, antiemetic use, and use of prophylactic medication were included in the multivariate analysis if the univariate p < 0.2. FINDINGS: Among 108 patients included, 36 completely discontinued their caffeine intake (abstinence group). The efficacy of acute treatment was assessed at median 34.5 days (interquartile range, 28-89) after the screening. Twenty-six patients (72.2 %) in the abstinence group and 29 (40.3 %) in the non-abstinence group reported an excellent efficacy (p = 0.002). The abstinence group also showed a trend toward greater reduction of headache impact test-6 (HIT-6) scores (p = 0.085). Caffeine abstinence was independently associated with an excellent efficacy of acute treatment (multivariate odds ratio, 3.2; 95 % confidence interval, 1.2-8.4; p = 0.018) after controlling for covariates. CONCLUSIONS: Caffeine abstinence is associated with better efficacy of acute migraine treatment. Our uncontrolled study results encourage a further confirmatory study on this issue.

Avian species survey with citizen-science data in Janghang Wetland, Goyang, Republic of Korea.

Background: Monitoring of avian populations in Janghang Wetland, Goyang, Republic of Korea (ROK) is based on citizen science (also called community-based monitoring). This monitoring data can be used to track avian density, population status and waterbird census at local, national and regional levels. The Ministry of Environment (MoE) ROK has surveyed since 1999, including Odusan Unification Tower to Ilsan Bride, which connects the cities of Gimpo and Goyang along the Han River estuary. However, it has not covered Janghang Wetland, which is located in the Han River estuary at the transboundary between the two Koreas. The Janghang Wetland is a protected wetland in the Demilitarized Zone (DMZ) between the two Koreas. In 2019, Janghang Wetland was designated as a Flyway Network Site by Goyang City and the East Asian-Australasian Flyway Partnership. This Network site is a voluntary collaboration and includes many internationally significant wetlands for waterbirds that still lack formal national protection. In addition, it was designated as a Ramsar site in 2021. The wetland currently supports wintering population of White-naped Crane (Grusvipio), species listed as vulnerable and Tundra Bean Goose (Ansercygnoides), spring-autumn migration population of Swan Goose (Ansercygnoid), species listed as vulnerable and a breeding population of Black-faced Spoonbill (Plataleaminor), species listed as endangered in summer. New information: We provide data that the Janghang Wetland is a significant area for migration and breeding for waterbirds; and that Han River estuary is also internationally important for waterbirds during the migratory bird season. We observed 14 orders, 42 families and 132 species. The surveys also observed the critically-endangered Black-faced Spoonbill (Plataleaminor), Swan Goose (Ansercygnoides), White-naped Crane (Grusvipio), Whooper Swan (Cygnuscygnus) and Peregrine Falcon (Falcoperegrinus). We also observed the Black-faced Spoonbill, Great Egret, Little Egret, Great Cormorant, Eastern Spot-billed Duck, Pheasant and Brown-eared Bulbul at the sensor camera point and White-naped Crane, Hooded Crane, Bean Goose, White-fronted Goose, Snow Goose, Swan Goose, Great Cormorant and Eastern Spot-billed Duck at the closed-circuit television camera point from the camera-trap surveys. Based on the species recorded, the survey area is of clear importance for biodiversity conservation.

Sphingobacterium kyonggiense sp. nov., isolated from chloroethene-contaminated soil, and emended descriptions of Sphingobacterium daejeonense and Sphingobacterium mizutaii.

A Gram-reaction-negative, strictly aerobic, rod-shaped, non-motile strain, designated 2-1-2(T), was isolated from perchloroethylene/trichloroethene-contaminated soil in Suwon, South Korea. A polyphasic approach was used to study the taxonomic position of strain 2-1-2(T). Strain 2-1-2(T) showed highest 16S rRNA gene sequence similarities to Sphingobacterium daejeonense TR6-04(T) (97.9%) and Sphingobacterium mizutaii ATCC 33299(T) (97.1%); sequence similarities to other Sphingobacterium species were less than 93.0%. Phylogenetic analysis based on 16S rRNA gene sequences showed that strain 2-1-2(T) belonged to the clade formed by members of the genus Sphingobacterium in the family Sphingobacteriaceae. The G+C content of the genomic DNA was 36.6 mol%. Strain 2-1-2(T) showed the typical chemotaxonomic features of the genus Sphingobacterium, with the presence of a ceramide phosphorylethanolamine (CerPE-2) as the major ceramide, menaquinone 7 (MK-7) as the predominant respiratory quinone and iso-C(15:0), iso-C(17:0) 3-OH and summed feature 3 (comprising iso-C(15:0) 2-OH and/or C(16:1)ω7c) as the major fatty acids. On the basis of phylogenetic inference, fatty acid profile and other phenotypic properties, and DNA-DNA relatedness, strain 2-1-2(T) represents a novel species of the genus Sphingobacterium, for which the name Sphingobacterium kyonggiense sp. nov. is proposed; the type strain is 2-1-2(T) (=KEMC 2241-005(T)=JCM 16704(T)). Emended descriptions of Sphingobacterium daejeonense and Sphingobacterium mizutaii are also proposed.

Symptoms, resilience, coping, and psychosocial adjustment in women with gynaecological cancer in South Korea: A cross sectional study.

PURPOSE: This cross-sectional study aimed to evaluate the relationship between symptoms, resilience, coping, and psychosocial adjustment, and to identify the influence of these variables on the psychosocial adjustment in women with gynaecological cancer in South Korea. METHODS: A correlational research design was employed based on the stress-coping theory of Lazarus and Folkman. A total of 150 women with gynaecological cancer admitted to a Korean tertiary hospital were included via convenience sampling. Data were collected using structured questionnaires between January and April 2018. Data analyses included descriptive statistics, independent t-test, χ2 test, analysis of variance, Pearson's correlation coefficient, and hierarchical multiple regression using the SPSS WIN 25.0 program. RESULTS: The study found that symptoms had the strongest association with psychosocial adjustment in women with gynaecological cancer. Psychosocial adjustment showed a statistically significant relationship with symptoms (r = 0.34, p < .001), resilience (r = -.43, p < .001), and coping (r = -.32, p < .001). A hierarchical multiple regression analysis demonstrated that symptoms (ß = 0.33, p < .001), resilience (ß = -.30, p < .001), and coping (ß = -.17, p = .032) accounted for 28.1% of the variance in psychosocial adjustment. CONCLUSIONS: It is pertinent for healthcare providers to assess and develop symptom management to provide resilience and coping strategies for gynaecological cancer women and improve their psychosocial adjustment.

Comparing Perceptions, Determinants, and Needs of Patients, Family Members, Nurses, and Physicians When Making Life-Sustaining Treatment Decisions for Patients with Hematologic Malignancies.

Purpose: This descriptive study compared the perceptions, determinants, and needs of patients, family members, nurses, and physicians regarding life-sustaining treatment decisions for patients with hematologic malignancies in the hematology-oncology department of a tertiary hospital in Seoul, Korea. Methods: In total, 147 subjects were recruited, gave written consent, and provided data by completing a structured questionnaire. Data were analyzed using analysis of variance, the chi-square test, and the Fisher exact test. Results: Nurses (F=3.35) and physicians (F=3.57) showed significantly greater familiarity with the Act on Decisions on Life-Sustaining Treatment than patients (F=2.69) and family members (F=2.59); (F=19.58, P<0.001). Many respondents, including 19 (51.4%) family members, 16 (43.2%) physicians, and 11 (29.7%) nurses, agreed that the patient's opinion had the greatest effect when making life-sustaining treatment decisions. Twelve (33.3%) patients answered that mental, physical, and financial burdens were the most important factors in life-sustaining treatment decisions, and there was a significant difference among the four groups (P<0.001). Twenty-four patients (66.7%), 27 (73.0%) family members, and 21(56.8%) nurses answered that physicians were the most appropriate people to provide information regarding life-sustaining treatment decisions. Unexpectedly, 19 (51.4%) physicians answered that hospice nurse practitioners were the most appropriate people to talk to about life-sustaining treatment (P<0.001). Conclusion: It is of utmost importance that the patient and physician determine when life-sustaining treatment should be withdrawn, with the patient making the ultimate decision. Doctors and nurses have the responsibility to provide detailed information. The goal of end-of-life planning is to ensure patients' dignity and respect their values.

Avian Surveys in the Korean Inner Border Area, Gimpo, Republic of Korea.

BACKGROUND: Birds are useful environmental indicators as their presence reflects the health of the food web. Bird occurrence, rarity and abundance are reliable indicators of ecosystem health. Monitoring of avian populations in the Republic of Korea (ROK) is a primary requirement due to plummeting populations and the risks to threatened species. The Ministry of Environment of ROK started conducting winter bird censuses in 1999, including inland areas and coast areas, such as Cheorwon, Yeoncheon, Junam Reservoir and Han River. Cheolwon, Yeoncheon and some extent islands in the West Sea have been survey extensively due to iconic bird species, such as White-naped Crane (Grus vipio) or Red-Crowned Crane (Grus japonensis) wintering there. However, the winter bird census has not covered Yu Islet, Han River Estuary. Yu Islet is located within the Han River Estuary, a protected wetland in the Neutral Zone between the two Koreas and north of Gimpo in the ROK. The Islet currently supports a large, mixed breeding colony of waterbirds, such as one of the nation's largest concentration of breeding Great Cormorants (Phalacrocorax carbo) and smaller numbers of breeding Black-faced Spoonbill (Platalea minor), Grey Heron (Ardea cinereal), Great Egret (Ardea alba) and Intermediate Egret (Ardea intermedia). Access to the area has long been restricted for military reasons, but recently, regular survey activity is possible supported by Gimpo City and the military base in Gimpo from November 2018. NEW INFORMATION: Here, we provide data demonstrating that Yu Islet is important for breeding for waterbirds; and that the northern Gimpo part of Han River Estuary is also internationally important for waterbirds during the migratory bird season, as defined by the Ramsar Convention (Ramsar 1971, RRC-EA 2017). In particular, four waterbird species were found during the survey in the Main Survey Area: Swan Goose (Anser cygnoides), Taiga Bean Goose (Anser fabalis), Tundra Bean Goose (Anser serrirostris) and Greater White-fronted Goose (Anser frontalis). Once considered widespread in East Asia and abundant, the world population of Swan Goose is now estimated at only 60,000 - 78,000 individuals (Wetlands International 2020) and the species is assessed by BirdLife International as globally Vulnerable (BirdLife International 2020). The 1,010 Swan Goose (Anser cygnoides) counted on the vegetated mudflats at Jogang-Ri in the Main Survey Area on 27 November 2018 represents more than 1% of the total world population of this species. Notably, it is also the highest count of this species in the ROK for at least a decade. The count confirms the continuing international importance of the Han River Estuary for the survival of the Swan Goose. The number counted in November had fallen to 250 by 28 December 2018; and none was recorded in the Main Survey Area in January or February 2019. Although searched for in March, none was noted during the northward migration either. The surveys also found small numbers of nationally-scarce Grey-capped Pygmy Woodpecker (Yungipicus canicapillus) in several areas of woodland surrounded by the Han River Estuary. By selecting the most species-rich count within a given month in each of the two survey sectors, the number of species we recorded ranged from a minimum 29 in January to a maximum of 65 in April 2019. Based on the species recorded, the survey area is clearly important for avian conservation. Its importance derives from the combination of the extensive areas of high-quality wetland and its geographic location within one of the Korea Peninsula's largest and most important remaining wetland ecosystems, the Han River Estuary. Our surveys resulted in the detection of a substantial number of bird species, especially in March and April when forest-breeding birds are more obviously vocal. The survey result is provided in the supplementary material (Suppl. material 1).

Severe hemolytic disease of the newborn due to anti-Di b treated with phototherapy and intravenous immunoglobulin.

The Di(b) antigen usually occurs with high incidence, except in certain Asian and South American Indian populations. In general, hemolysis caused by anti-Di(b) is not severe and its clinical course is benign. We report a Korean neonate with severe hemolytic disease of the newborn caused by anti-Di(b). The phenotype and genotype of the Diego blood group system of the patient and his mother were Di(a+b+) and Di(a+b-), respectively. The mother's serum and eluate from the neonate's erythrocytes contained anti-Di(b). This case was successfully managed with phototherapy and high dose iv immunoglobulin. Since most commercial antibody detection panels do not contain Di(b-) red cells, it is important to consider anti-Di(b) in cases of hemolytic disease of the newborn caused by an antibody against a high frequency antigen.

Characteristics of hypertrophic pachymeningitis in patients with granulomatosis with polyangiitis.

Hypertrophic pachymeningitis (HP) is an important neurologic complication of granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis). The aim of this study is to investigate the clinical features, radiological findings, and diagnostic pitfalls of GPA-related HP. A retrospective chart review was performed to screen patients diagnosed with GPA at Samsung Medical Center between 1997 and 2016. Neurologic manifestation, laboratory findings, neuroimaging data, and clinical course were evaluated in all patients. Characteristics of patients with HP were compared to those of patients without HP. Sixty-five patients with GPA were identified. Twenty-five of these patients had central nervous system involvement. HP (N = 9, 36%) was the second most common radiologic finding. Other neurologic findings included stroke (N = 7, 28%) and granulomatous disease (N = 10, 40%). Patients with HP had lower incidences of systemic manifestations (N = 2, 22.2% vs. N = 38, 67.9%, p = 0.013 in the lung and N = 1, 11.1% vs. N = 28, 50.0%, p = 0.030 in the kidney) than those without HP. Six patients with GPA-related HP were MPO-ANCA positive (66.7%) and two had PR3-ANCA (22.2%). Most of the patients with HP presented with headache (N = 8, 88.9%) at a rate that is similar to those of primary headache disorders (migraine, tension-type, and stabbing) and other secondary headache disorders (postural type and meningitis). Patients with HP rarely had neurologic deficits (N = 3, 37.5%). Different clinical or radiologic features may be observed in GPA-related HP. Early recognition and accurate diagnosis of GPA-related HP are needed in addition to neuroimaging findings.

Neonatal clavicle fracture in cesarean delivery: incidence and risk factors.

BACKGROUND: Neonatal clavicle fracture in cesarean delivery is rare and has not been extensively studied. METHODS: We performed a retrospective review of cesarean deliveries with neonatal clavicle fracture during a 12-year period. Maternal and neonatal factors as well as surgical factors related to cesarean delivery for the fracture were determined and compared to the control group to analyze their significance. RESULTS: Among a total 89 367 deliveries during the study period, 36 286 babies were born via cesarean section. Nineteen cases of clavicle fractures in cesarean section were identified (0.05% of total live births via cesarean section). In the analysis of maternal and neonatal risk factors, birthweight, birthweight ≥ 4000 g and maternal age were significantly associated with clavicle fracture in cesarean section. However, clavicle fractures were not correlated with the selected surgical factors such as indication for cesarean section, skin incision to delivery time and incision type of skin and uterus. Logistic regression analysis showed that birthweight was the major risk factor for clavicle fracture. CONCLUSION: Clavicle fractures complicated 0.05% of cesarean deliveries. The main risk factor related to a clavicle fracture in cesarean section was the birthweight of an infant. As reported in previous studies associated with vaginal delivery, clavicle fracture is considered to be an unavoidable event and may not be eliminated, even in cesarean delivery.

Analysis of the distribution of bacteria within urinary catheter biofilms using four different molecular techniques.

BACKGROUND: Most nosocomial urinary tract infections are associated with the long-term use of urinary catheters. Such urinary catheter-associated infections are caused by bacteria that reside in biofilms. We determined the distribution of fastidious/nonculturable bacteria in biofilm of urinary catheters and evaluated the availability of concurrent applying various molecular techniques. METHODS: The biofilms were isolated from urinary catheters that had been installed in patients for 3 or 4 weeks and examined by the following 4 different 16S ribosomal RNA (rRNA) analysis techniques: capillary electrophoresis, terminal restriction fragment length polymorphism (T-RFLP), denaturing gradient gel electrophoresis (DGGE), and pyrosequencing. RESULTS: A total of 329 isolates was identified by capillary electrophoresis. The most common genera were Edwardsiella, Enterobacter, Escherichia, and Pseudomonas. A total of 32 bacterial strains was identified by T-RFLP. Escherichia, Pseudomonas, Enterobacter, Moraxella, Proteus, Serratia, and Yersinia were the most represented genera. Similarly, Escherichia, Pseudomonas, and Enterobacter were the most prevalent according to DGGE. Burkholderia, Corynebacterium, Achromobacter, Alcaligenes, Citrobacter, Stenotrophomonas, and Streptococcus were also detected. Escherichia and Pseudomonas were abundantly detected by pyrosequencing. Enterobacter, Bacteroides, Klebsiella, Corynebacterium were also seen. CONCLUSION: These 4 techniques detected different kinds of bacteria, suggesting that the simultaneous application of multiple techniques is necessary to accurately detect fastidious/nonculturable bacteria. Because bacterial growth within urinary catheter biofilms may be associated with urinary tract infections, further comprehensive studies are required.

The changes in circulating osteoprotegerin after hormone therapy in postmenopausal women and their relationship with oestrogen responsiveness on bone.

OBJECTIVE: Oestrogen replacement reduces the increased rate of bone remodelling after the menopause. Osteoprotegerin (OPG) is a negative regulator of osteoclast-mediated bone resorption. In vitro studies have shown that oestrogen stimulates OPG production. However, the role of OPG in physiological bone remodelling and its regulation by oestrogen in vivo remain controversial. In this study, we analysed the association between changes in serum OPG levels and bone turnover status before and after hormone therapy (HT) in healthy postmenopausal women. PATIENTS AND MEASUREMENTS: Ninety-nine healthy postmenopausal women of Korean ethnicity, aged 42-64 years (52.3 +/- 4.9 years, mean +/- SD) were enrolled in our study. Serum OPG levels were assessed by a highly sensitive sandwich-type enzyme immunoassay. Serum concentrations of osteocalcin (OC) and carboxyterminal telopeptides (CTx) were determined by electrochemiluminescence immunoassays. Bone mineral density (BMD) at the lumbar spine and femoral neck was measured by dual-energy X-ray absorptiometry (DEXA). RESULTS: Baseline levels of OPG correlated neither a the bone formation marker, serum OC, nor with a bone resorption marker, serum CTx. No significant association of baseline OPG was found with baseline BMD measured at the lumbar spine and femoral neck. Serum OPG levels measured after 3 months and 1 year of HT decreased significantly compared to baseline (P < 0.001 in both). The changes in circulating OPG at 3 months of HT correlated with the changes in both serum OC (r = 0.226, P = 0.029) and serum CTx (r = 0.214, P = 0.038) at 3 months after HT. However, there was no significant association between the changes in circulating OPG after 3 months of HT and BMD values of the lumbar spine or femoral neck after 1 year of HT. CONCLUSIONS: Our results suggest that baseline OPG levels do not reflect bone turnover status and that serial measurements of serum OPG after HT are not a useful predictor of the long-term effects of oestrogen on bone density. The decrease in serum concentrations of OPG after HT may occur to compensate for the action of oestrogen in suppressing bone resorption.