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Sample records for korea clinical features

  1. Clinical Features of Ulcerative Colitis in Korea

    PubMed Central

    Park, Seon Mee; Han, Dong Soo; Yang, Suk-Kyun; Hong, Weon-Seon; Min, Young Il

    1996-01-01

    Objectives : This study was conducted to investigate the clinical features of ulcerative colitis in Korea and to evaluate the clinical course after medical therapy. Methods : Symptoms, signs and results of the treatment were retrospectively analyzed in 66 patients (male 32, female 34) diagnosed to have ulcerative colitis at the Asan Medical Center. Results : The median age of the beginning of symptoms was 36 years (range, 14–72). Diarrhea and rectal bleeding were observed in 95.1 and 91.4%, respectively, at the time of diagnosis, while extra-colonic manifestations were observed in 24.1%. In 41 patients (62.1%), colitis developed in the rectum and sigmoid colon, while left colitis and extensive colitis developed in 11 (16.7%) and 14 patients (21.2%), respectively. The severity of disease was determined according to the clinical criteria, resulting in 22 (33.3%) mild, 21 (31.8%) moderate and 23 (34.8%) severe diseases. The seventy was also classified as 1, 2 and 3 by sigmoido-colonoscopic findings: 1;17 patients(25.8%), 2;27(40.9%) and 22(33.3%). Among 23 patients with severe disease, 5 patients (7.6%) received total colectomy due to toxic megacolon, intractability to medical therapy, ileocolic fistula and intestinal stenosis. The severity determined by colonoscopic findings was well correlated with that determined clinically and was closely related to the severity of symptoms, levels of albumin, hemoglobin and the count of leukocyte. The median duration of symptoms before treatment was 4 weeks (range. 11–300). All patients were treated with sulfasalazine and prednisolone. All patients with medical therapy, except 2 patients (96. 7%), obtained clinical remission. The median days required for remission was 14 (range, 3–70). Relapse rates at 6 months, 1 year and 2 years after the initiation of treatment were 19.7, 34.1 and 49.3%, respectively. The median disease-free interval from the time of remission was 10 months (range, 2–60). After remission, the

  2. Clinical Features and Awareness of Hand Eczema in Korea

    PubMed Central

    Park, Jae Beom; Lee, Seung Ho; Kim, Kea Jeung; Lee, Ga-Young; Yang, Jun-Mo; Kim, Do Won; Lee, Seok Jong; Lee, Cheol Heon; Park, Eun Joo; Kim, Kyu Han; Eun, Hee Chul; Chang, Sung Eun; Moon, Kee Chan; Kim, Seong Hyun; Kim, Seong Jin; Kim, Byung-Soo; Lee, Jun Young; Kim, Hyung-Ok; Kang, Hoon; Lee, Min Geol; Kim, Soo-Chan; Ro, Young Suck; Ko, Joo Yeon; Park, Mi Youn; Kim, Myung Hwa; Shin, Jeong Hyun; Choi, Hae Young; Hong, Chang Kwun; Lee, Sung Yul; Bak, Hana

    2016-01-01

    Background Hand eczema is one of the most common skin disorders and negatively affects quality of life. However, a large-scale multicenter study investigating the clinical features of patients with hand eczema has not yet been conducted in Korea. Objective To identify the prevalence of various hand diseases, which is defined as all cutaneous disease occurring in hands, and to investigate the clinical features of patients with hand eczema and the awareness about hand eczema in the general population and to compare the prevalence of hand eczema between health care providers and non-health care providers. Methods To estimate the prevalence of hand diseases, we analyzed the medical records of patients from 24 medical centers. Patients were assessed by online and offline questionnaires. A 1,000 from general population and 913 hand eczema patients answered the questionnaire, for a total of 1,913 subjects. Results The most common hand disease was irritant contact dermatitis. In an online survey, the lifetime prevalence of hand eczema was 31.2%. Hand eczema was more likely to occur in females (66.0%) and younger (20~39 years, 53.9%). Health care providers and housewives were the occupations most frequently associated with hand eczema. Winter (33.6%) was the most common season which people experienced aggravation. The 63.0% and 67.0% answered that hand eczema hinders their personal relationship and negatively affects daily living activities, respectively. Conclusion Hand eczema is a very common disease and hinders the quality of life. The appropriate identification of hand eczema is necessary to implement effective and efficient treatment. PMID:27274632

  3. Clinical Features of Cluster Headache Patients in Korea

    PubMed Central

    2017-01-01

    Cluster headache (CH) is a rare underdiagnosed primary headache disorder with very severe unilateral pain and autonomic symptoms. Clinical characteristics of Korean patients with CH have not yet been reported. We analyzed the clinical features of CH patients from 11 university hospitals in Korea. Among a total of 200 patients with CH, only 1 patient had chronic CH. The average age of CH patients was 38.1 ± 8.9 years (range 19–60 years) and the average age of onset was 30.7 ± 10.3 years (range 10–57 years). The male-to-female ratio was 7:1 (2.9:1 among teen-onset and 11.7:1 among twenties-onset). Pain was very severe at 9.3 ± 1.0 on the visual analogue scale. The average duration of each attack was 100.6 ± 55.6 minutes and a bout of CH lasted 6.5 ± 4.5 weeks. Autonomic symptoms were present in 93.5% and restlessness or agitation was present in 43.5% of patients. Patients suffered 3.0 ± 3.5 (range 1–25) bouts over 7.3 ± 6.7 (range 1–30) years. Diurnal periodicity and season propensity were present in 68.5% and 44.0% of patients, respectively. There were no sex differences in associated symptoms or diurnal and seasonal periodicity. Korean CH patients had a high male-to-female ratio, relatively short bout duration, and low proportion of chronic CH, unlike CH patients in Western countries. PMID:28145655

  4. Clinical Features and Causes of Endogenous Hyperinsulinemic Hypoglycemia in Korea

    PubMed Central

    Woo, Chang-Yun; Jeong, Ji Yun; Jang, Jung Eun; Leem, Jaechan; Jung, Chang Hee; Koh, Eun Hee; Lee, Woo Je; Kim, Min-Seon; Park, Joong-Yeol; Lee, Jung Bok

    2015-01-01

    Background Endogenous hyperinsulinemic hypoglycemia (EHH) is characterized by an inappropriately high plasma insulin level, despite a low plasma glucose level. Most of the EHH cases are caused by insulinoma, whereas nesidioblastosis and insulin autoimmune syndrome (IAS) are relatively rare. Methods To evaluate the relative frequencies of various causes of EHH in Korea, we retrospectively analyzed 84 patients who were diagnosed with EHH from 1998 to 2012 in a university hospital. Results Among the 84 EHH patients, 74 patients (88%), five (6%), and five (6%) were diagnosed with insulinoma, nesidioblastosis or IAS, respectively. The most common clinical manifestation of EHH was neuroglycopenic symptoms. Symptom duration before diagnosis was 14.5 months (range, 1 to 120 months) for insulinoma, 1.0 months (range, 6 days to 7 months) for nesidioblastosis, and 2.0 months (range, 1 to 12 months) for IAS. One patient, who was diagnosed with nesidioblastosis in 2006, underwent distal pancreatectomy but was later determined to be positive for insulin autoantibodies. Except for one patient who was diagnosed in 2007, the remaining three patients with nesidioblastosis demonstrated severe hyperinsulinemia (157 to 2,719 µIU/mL), which suggests that these patients might have had IAS, rather than nesidioblastosis. Conclusion The results of this study suggest that the prevalence of IAS may be higher in Korea than previously thought. Therefore, measurement of insulin autoantibody levels is warranted for EHH patients, especially in patients with very high plasma insulin levels. PMID:25922806

  5. Clinical features and course of generalized pustular psoriasis in Korea.

    PubMed

    Jin, Hyunju; Cho, Hyun-Ho; Kim, Won-Jeong; Mun, Je-Ho; Song, Margaret; Kim, Hoon-Soo; Ko, Hyun-Chang; Kim, Moon-Bum; Kim, Hyojin; Kim, Byung-Soo

    2015-07-01

    The clinical course of generalized pustular psoriasis (GPP) is variable and unpredictable. Sufficient data on the clinical course of the disease has not been reported due to its rarity. To investigate the clinical features and course of GPP according to its subtypes, medical records of patients diagnosed with GPP from 2002 to 2012 at two tertiary hospitals were reviewed. The data included patient demographics, associated symptoms, aggravating factors, patterns of relapse and prognosis. Thirty-three patients with GPP were included in our study, with a mean age of 45.6 years and a male : female ratio of 1:1.2. Patients were categorized based on the following subtypes: acute GPP, 21 (63.6%); GPP of pregnancy, two (6.1%); juvenile GPP, three (9.1%); and annular GPP, seven (21.2%). In the acute GPP population, skin lesions cleared within 2 months in 11 (73.3%) patients, and six (40.0%) of these had no relapse. Severe complications, abortion or death, were observed in two patients (100.0%) with GPP of pregnancy. Nineteen (76.0%) of the GPP patients experienced persistence or relapse of skin lesions. The patterns of skin lesions upon relapse included plaques in six patients (31.6%), pustules in eight patients (42.1%), and plaques and pustules in five patients (26.3%). Among acute GPP patients, 16.7% of patients with no relapse had a history of plaque psoriasis. However, 77.8% of patients with persistence and relapse in their clinical course had a history of plaque psoriasis. In conclusion, our study presents the detailed clinical course of GPP by subtype in Korean patients. © 2015 Japanese Dermatological Association.

  6. Epidemiological features and clinical manifestations of Lyme borreliosis in Korea during the period 2005-2012.

    PubMed

    Moon, Shinje; Hong, Yeongseon; Hwang, Kyu-Jam; Kim, Suyeon; Eom, Jihye; Kwon, Donghyok; Park, Ji-Hyuk; Youn, Seung-Ki; Sohn, Aeree

    2015-01-01

    Lyme borreliosis is one of the most common tick-borne infections in the northern hemisphere. However, the epidemiological features and clinical manifestations of this disease in Korea are unknown. The present study is the first to investigate the characteristics of Lyme borreliosis in Korea. We traced suspected cases of Lyme borreliosis during the period 2005-2012. Of the 16 identified patients with the disease, 11 had acquired autochthonous infection within Korea, while 5 patients were infected outside Korea. The history of past exposure was investigated in 8 of the 11 patients with autochthonous infection; 5 of these 8 patients (62.5%) were suspected to have acquired the infection in the northeastern alpine region. Clinically, of 11 patients with autochthonous infection, 6 (54.5%) showed early skin manifestations, 4 (36.4%) showed neurological manifestations, and 1 (9.1%) showed acrodermatitis chronica atrophicans. In conclusion, Lyme borreliosis could be endemic in the northeastern alpine region of Korea, and neurological and early skin manifestations are likely to be the major clinical characteristics of autochthonous Lyme borreliosis in Korea.

  7. Changes in clinical and laboratory features of Kawasaki disease noted over time in Daejeon, Korea.

    PubMed

    Kil, Hong-Ryang; Yu, Jae-Won; Lee, Sung-Churl; Rhim, Jung-Woo; Lee, Kyung-Yil

    2017-08-07

    Kawasaki disease (KD) becomes one of the common diseases in Korea. Changes in clinical features and laboratory findings of KD were evaluated over a period of 10 years. We reviewed the medical records of KD patients and compared the clinical and laboratory features of two KD patient groups: those admitted from 2000 to 2004 (group A, 284 cases) and those admitted from 2010 to 2014 (group B, 331 cases). There were a total of 615 KD patients (mean age: 29.7 months; male-to-female ratio = 1.6:1), including 228 incomplete KD patients. Incomplete KD patients had milder values in some laboratory indices. The preadmission and total fever durations were longer in group A than in group B. The proportion of incomplete KD was higher in group B, but incidence of coronary artery lesions (CALs) was lower. For laboratory indices, the C-reactive protein and follow-up platelet values were lower, and the hemoglobin and albumin values were higher in group B. The same clinical and laboratory findings were confirmed in the KD subgroups; those with the same fever duration of 5 or 6 days and same ages, those with complete KD, and those with incomplete KD in the two different time periods. Our findings suggest that clinical features of KD tend to be milder over time and manifest in a higher incidence of incomplete KD, lower incidence of CALs, and less severe laboratory findings in recent KD patients in Korea compared with their historic counterparts.

  8. Clinical Features and Outcomes of Patients With Genotype 3 Hepatitis C Virus Infection in Korea

    PubMed Central

    Cha, Ra Ri; Lee, Sang Soo; Lee, Chang Min; Ji, Sung Bok; Jung, Hee Cheul; Cho, Hyun Chin; Kim, Jin Joo; Lee, Jae Min; Kim, Hong Jun; Ha, Chang Yoon; Kim, Hyun Jin; Kim, Tae-Hyo; Jung, Woon Tae; Lee, Ok-Jae

    2016-01-01

    Abstract Hepatitis C virus (HCV) genotype 3 infection is very rare in high-income Asia Pacific. The aim of our retrospective observational study was to evaluate the incidence, clinical features, and treatment outcomes of patients with a genotype 3 HCV infection in the Gyeongnam Province of Korea. Ninety-eight consecutive patients diagnosed with a genotype 3 HCV infection at Gyeongsang National University Hospital, between January 2005 and December 2014, were enrolled into the study. Relevant characteristics of the study group included: 80.6% men, mean age of 41.8 years, and including 69 patients with chronic hepatitis, 25 with liver cirrhosis, and 4 with hepatocellular carcinoma (HCC). Risk factors for HCV infection, sustained virologic response rate, development of HCC, and mortality in patients with genotype 3 were retrospectively analyzed. Among all patients diagnosed with a HCV infection during the study period, the prevalence of genotype 3 was 7.3%. The incidence of genotype 3 was higher in young patients with a risk factor of IVDU (54.0%) and tattooing (62.3%). Among 45 treatment-naive genotype 3 patients, sustained virologic response was achieved with a combination of pegylated-interferon alpha and ribavirin in 75.6%. The cumulative 5-year incidence of HCC was 13.6%, and 8.9% for overall mortality. Liver cirrhosis at enrollment was an independent risk factor for HCC development. This is the first study to elucidate the clinical features and outcomes among the patients with HCV genotype 3 infection in Korea. Further prospective studies are needed to investigate transmission routes and outcomes for HCV genotype 3 infections. PMID:26871824

  9. Clinical Features of Post-Vaccination Guillain-Barré Syndrome (GBS) in Korea

    PubMed Central

    2017-01-01

    Guillain-Barré syndrome (GBS) is the most common immune-mediated polyradiculoneuropathy and it is also the most commonly reported severe adverse event following immunization in adults. To evaluate the results of clinical and laboratory features of GBS after vaccination in Korea, we analyzed the claims-based data from 2002 to 2014 using materials collected for the Advisory Committee Vaccination Injury Compensation (ACVIC) meeting including, clinical features, nerve conduction studies (NCSs), cerebrospinal fluid (CSF) profiles, treatment, and outcomes. Forty-eight compensated GBS cases (median age, 15 years; interquartile range [IQR], 13–51; male:female ratio, 1:1) of 68 suspected GBS were found following immunization and all of them with influenza immunizations with either monovalent (n = 35) or trivalent (n = 13). Among them, 30 cases fulfilled the Brighton criteria level 1–3 (62.5%). The median duration between the onset of symptoms to nadir, duration of the nadir, and total admission period were 3 (IQR, 2–7 days), 2 (IQR, 1–5 days), and 14 (IQR, 6–33 days) days, respectively. The most frequently reported symptom was quadriparesis which was present in 36 cases (75%) at nadir. CSF examination revealed albuminocytologic dissociation in 25.0% and NCS was abnormal in 61.8%. After treatment, most of them showed improvement. Clinical features were similar to typical post-infectious GBS and there were both demyelinating and axonal forms suggesting heterogeneous pathogenic mechanism. In order to improve the diagnostic certainty of post-vaccination GBS, careful documentation of clinical features and timely diagnostic work-up with follow-up studies are needed. PMID:28581273

  10. Clinical Features of Fixed Drug Eruption at a Tertiary Hospital in Korea

    PubMed Central

    Jung, Jae-Woo; Cho, Sang-Heon; Kim, Kyu-Han; Min, Kyung-Up

    2014-01-01

    Purpose Fixed drug eruption (FDE) is characterized by a well-defined erythematous patch, plaque, or bullous eruption that recurs at the same site as the result of systemic exposure to a causative drug, and resolves with or without hyperpigmentation. This study was carried out to identify the common causative drugs and clinical features of FDE in Korea. Methods We reviewed electronic medical records of all patients diagnosed with FDE from January 2000 to December 2010 at a tertiary hospital in Korea. Results A total of 134 cases were diagnosed as FDE. The mean age was 35.9 years (range, 0-82 years) and 69 (51.5%) of the patients were male. The mean duration from the first event to attending hospital was 1.9 years (range, 1-20 years). The mean number of recurrences was 2.6 (1-10), and 72.6% of patients sought medical care after experiencing symptoms twice or more. Four patients (3.1%) needed hospitalization. The most common sites were the upper extremities (47.7%), followed by the lower extremities, face, abdomen, chest, buttocks and perineum. Clear documentation on the causative drugs was available for 38 patients (28.4%), and among these, non-steroidal anti-inflammatory drugs (NSAIDs) and acetaminophen accounted for 71.1% of cases, and antibiotics accounted for 15.8%. Eighty patients (59.7%) underwent active treatment for FDE, and topical steroids were most frequently prescribed (43.3%), with systemic steroids used in 11.2% of patients. Conclusions NSAIDs and acetaminophen were the main causative agents of FDE, however, the causative agents were not assessed in 25% of patients. PMID:25228998

  11. Clinical features of fixed drug eruption at a tertiary hospital in Korea.

    PubMed

    Jung, Jae-Woo; Cho, Sang-Heon; Kim, Kyu-Han; Min, Kyung-Up; Kang, Hye-Ryun

    2014-09-01

    Fixed drug eruption (FDE) is characterized by a well-defined erythematous patch, plaque, or bullous eruption that recurs at the same site as the result of systemic exposure to a causative drug, and resolves with or without hyperpigmentation. This study was carried out to identify the common causative drugs and clinical features of FDE in Korea. We reviewed electronic medical records of all patients diagnosed with FDE from January 2000 to December 2010 at a tertiary hospital in Korea. A total of 134 cases were diagnosed as FDE. The mean age was 35.9 years (range, 0-82 years) and 69 (51.5%) of the patients were male. The mean duration from the first event to attending hospital was 1.9 years (range, 1-20 years). The mean number of recurrences was 2.6 (1-10), and 72.6% of patients sought medical care after experiencing symptoms twice or more. Four patients (3.1%) needed hospitalization. The most common sites were the upper extremities (47.7%), followed by the lower extremities, face, abdomen, chest, buttocks and perineum. Clear documentation on the causative drugs was available for 38 patients (28.4%), and among these, non-steroidal anti-inflammatory drugs (NSAIDs) and acetaminophen accounted for 71.1% of cases, and antibiotics accounted for 15.8%. Eighty patients (59.7%) underwent active treatment for FDE, and topical steroids were most frequently prescribed (43.3%), with systemic steroids used in 11.2% of patients. NSAIDs and acetaminophen were the main causative agents of FDE, however, the causative agents were not assessed in 25% of patients.

  12. Epidemiology and clinical features of HIV infection/AIDS in Korea.

    PubMed

    Kim, June Myung; Cho, Goon Jae; Hong, Sung Kwan; Chang, Kyung Hee; Chung, Joo Sup; Choi, Young Hwa; Song, Young Goo; Huh, Aejung; Yeom, Joon Sup; Lee, Kkot Sil; Choi, Jun Yong

    2003-06-30

    HIV infection/AIDS shows characteristic epidemiological and clinical patterns according to the region, country, and race. The epidemiological and clinical patterns of HIV infection/ AIDS in Korea was investigated by retrospectively analyzing the medical records of 176 HIV-infected persons who visited two major referral hospitals of AIDS in Korea from 1985 to April 2000. The most common transmission route was heterosexual contact (52.3%), followed by homosexual contact (23.9%). Among the opportunistic diseases, candidiasis was the most prevalent (21.6%), followed by Pneumocystis carinii pneumonia (15.9%), tuberculosis (12.5%), and CMV infection (9.1%). The most common initial AIDS-defining opportunistic disease was tuberculosis (33.3%). The most common causes of death were tuberculosis (25.7%) and Pneumocystis carinii pneumonia (25.7%). This study describes the epidemiological and clinical patterns of HIV infection/AIDS in Korea, which not only enables us to accurately understand HIV infection/ AIDS in this country, but eventually to aid in establishing effective preventive measures and treatment guidelines in Korea.

  13. Epidemiological and Clinical Features of Severe Fever with Thrombocytopenia Syndrome during an Outbreak in South Korea, 2013–2015

    PubMed Central

    Park, Sun-Whan; Ryou, Jungsang; Choi, Woo-Young; Han, Myung-Guk; Lee, Won-Ja

    2016-01-01

    Since the first reported case of severe fever with thrombocytopenia syndrome (SFTS) in South Korea in 2013, between 2013 and 2015, we collected 1,697 serum samples from suspected patients who experienced symptoms of SFTS. We performed reverse transcriptase polymerase chain reaction using total RNA extracted from the patients' sera. When viral RNA was detected in the sera, SFTS was diagnosed. Among the 1,697 samples, 170 were positive for SFTS virus. We then analyzed the epidemiologic features of these 170 cases. As a result, we found that the annual number of cases increased steadily. However, the annual case fatality rate exhibited a downward trend. The majority of patients were aged ≥ 60 years, and most cases occurred during May–October in the eastern and southern parts of the country. These results may be useful for effective SFTS control by describing the clinical and epidemiologic features of the disease in South Korea. PMID:27928084

  14. Epidemiology and Clinical Features of Kawasaki Disease in South Korea, 2012-2014.

    PubMed

    Kim, Gi Beom; Park, Sohee; Eun, Lucy Youngmin; Han, Ji Whan; Lee, Soo Young; Yoon, Kyung Lim; Yu, Jeong Jin; Choi, Jong-Woon; Lee, Kyung-Yil

    2017-05-01

    To reveal the recent epidemiologic features of Kawasaki disease (KD) in South Korea based on data from a nationwide survey. We collected data between 2012 and 2014 regarding the incidence, symptoms and signs, treatment trends and coronary complications associated with acute KD by sending questionnaires to 97 hospitals with pediatric residency programs as well as 19 community hospitals without residency training. We received full and partial data from 97 and 13 hospitals, respectively (response rate: 94.8%). A total of 14,916 cases of KD were reported by these 110 hospitals (4588 in 2012, 5183 in 2013 and 5145 in 2014). The male-to-female ratio was 1.4:1, and the median age at diagnosis was 29 months. The incidence of KD per 100,000 children younger than 5 years of age were 170.9, 194.9 and 194.7 in 2012, 2013 and 2014, respectively. The recurrence rate was 4.7%. KD occurred more frequently during summer (especially June and July) and winter (December and January) seasons. Intravenous immunoglobulin was administered to 95.4% of the patients, and the nonresponder rate for the first intravenous immunoglobulin was 11.8%. Coronary aneurysm occurred in 1.7% of the patients, and giant aneurysm developed in 19 patients (0.16%) during the 3 years. One patient had myocardial infarction and 1 patient died of suspected coronary aneurysm rupture. The incidence of KD in South Korea increased to 194.7 per 100,000 children younger than 5 years in 2014; meanwhile, the coronary aneurysm rate decreased to 1.7%.

  15. Clinical and molecular epidemiological features of hemorrhagic fever with renal syndrome in Korea over a 10-year period.

    PubMed

    Noh, Ji Yun; Cheong, Hee Jin; Song, Joon Young; Kim, Woo Joo; Song, Ki-Joon; Klein, Terry A; Lee, Sam H; Yanagihara, Richard; Song, Jin-Won

    2013-09-01

    Laboratory diagnosis of hemorrhagic fever with renal syndrome (HFRS), an infectious disease caused by rodent-borne hantaviruses in Asia and Europe, depends primarily on serological methods. Since the advent of such serodiagnostic tests, few reports are available about the clinical and molecular epidemiological features of HFRS. To investigate the epidemioclinical features of HFRS patients treated at a tertiary-care teaching hospital in Seoul over a 10-year period. Medical records of HFRS patients, admitted to a tertiary-care teaching hospital during February 2002 to February 2012, were reviewed. Sera from patients were tested for Hantaan virus (HTNV) and Seoul virus (SEOV) RNA using RT-PCR. Among 35 HFRS patients (mean age was 44.2±14.7 years), 29 were male (82.9%). Acute renal failure developed in 27 patients (77.1%), and 12 patients (34.3%) were admitted to the intensive care unit (ICU). Conjunctival injection (OR 10.32, 95% CI 1.09-97.77, P=.04) and initial serum albumin less than 3g/dL (OR 22.83, 95% CI 1.45-359.93, P=.03) were risk factors for ICU admission. Of 35 acute-phase sera, 11 (31.4%) were positive for HTNV RNA. None were positive for SEOV RNA. HFRS was characterized by the clinical triad of fever, renal insufficiency and gastrointestinal symptoms. Conjunctival injection and serum albumin level were related to severity. A large-scale multi-center study is needed to enhance insights into epidemioclinical characteristics of HFRS in Korea. Copyright © 2013 Elsevier B.V. All rights reserved.

  16. Clinical features and genetic analysis of children with hyperekplexia in Korea.

    PubMed

    Lee, Cha Gon; Kwon, Min-Jung; Yu, Hee Joon; Nam, Sook Hyun; Lee, Jeehun; Ki, Chang-Seok; Lee, Munhyang

    2013-01-01

    Hyperekplexia is a rare inherited neurologic disorder that is characterized by hypertonia and an exaggerated startle response to sudden external stimuli. Until now, 5 genes are known to be associated with hyperekplexia: GLRA1, SLC6A5, GLRB, GPHN, and ARHGEF9. In this report, we performed a clinical and genetic analysis of 4 Korean children with hyperekplexia. Two patients had typical clinical manifestations of hyperekplexia that initially were misdiagnosed as epilepsy. Direct sequencing of the GLRB and GLRA1 genes revealed 2 novel mutations, GLRB c.298-1G>A and c.1028C>T (p.S343F), in patient 1 and 1 novel mutation, GLRA1 c.895C>T (p.R299X), in patient 2. The other 2 familial cases, patients 3 and 4, exhibited startle responses, which appeared at the age of 1 year, and had global developmental delay. Those patients showed negative results for the 5 genes.

  17. The clinical and virological features of the first imported case causing MERS-CoV outbreak in South Korea, 2015.

    PubMed

    Lee, Ji Yeon; Kim, You-Jin; Chung, Eun Hee; Kim, Dae-Won; Jeong, Ina; Kim, Yeonjae; Yun, Mi-Ran; Kim, Sung Soon; Kim, Gayeon; Joh, Joon-Sung

    2017-07-14

    In 2015, the largest outbreak of Middle East respiratory syndrome coronavirus (MERS-CoV) infection outside the Middle East occurred in South Korea. We summarized the epidemiological, clinical, and laboratory findings of the first Korean case of MERS-CoV and analyzed whole-genome sequences of MERS-CoV derived from the patient. A 68-year-old man developed fever and myalgia 7 days after returning to Korea, following a 10-day trip to the Middle East. Before diagnosis, he visited 4 hospitals, potentially resulting in secondary transmission to 28 patients. On admission to the National Medical Center (day 9, post-onset of clinical illness), he presented with drowsiness, hypoxia, and multiple patchy infiltrations on the chest radiograph. He was intubated (day 12) because of progressive acute respiratory distress syndrome (ARDS) and INF-α2a and ribavirin treatment was commenced. The treatment course was prolonged by superimposed ventilator associated pneumonia. MERS-CoV PCR results converted to negative from day 47 and the patient was discharged (day 137), following rehabilitation therapy. The complete genome sequence obtained from a sputum sample (taken on day 11) showed the highest sequence similarity (99.59%) with the virus from an outbreak in Riyadh, Saudi Arabia, in February 2015. The first case of MERS-CoV infection had high transmissibility and was associated with a severe clinical course. The patient made a successful recovery after early treatment with antiviral agents and adequate supportive care. This first case in South Korea became a super-spreader because of improper infection control measures, rather than variations of the virus.

  18. Clinical neuropsychology in South Korea.

    PubMed

    Kim, Myung-Sun; Chey, Jeanyung

    2016-11-01

    Clinical neuropsychology in South Korea, albeit its relatively short history, has advanced dramatically. We review a brief history and current status of clinical neuropsychology in South Korea. The history, the educational pathway, the training pathway, the certification process, and careers in clinical neuropsychology in South Korea are reviewed. We have reviewed the neuropsychological services, including assessment and treatment, research on neurological and psychiatric populations, and neuropsychology education and the requirements related to education, training, and board examinations of those providing neuropsychological services in South Korea. We also describe how the Korean Society for Neuropsychology Research, the first and only meeting for neuropsychologists in the country established in 1999, has played a major role in how clinical neuropsychology is practiced and developed as a professional field in South Korea. Clinical neuropsychology in South Korea has achieved major progress over just a quarter of a century, and its future is promising in light of the increasing demand for neuropsychological services and advances in neuroscience in the country. Challenges that the community of clinical neuropsychologists are currently facing in South Korea, including formalizing neuropsychological curriculum and training programs and developing advanced credentialing procedures, are discussed.

  19. Clinical Features of Re-Emerging Hepatitis A: An Analysis of Patients Hospitalized during an Urban Epidemic in Korea

    PubMed Central

    Choi, Hee Kyoung; Song, Young Goo; Kim, Chang Oh; Shin, So Youn; Chin, Bum Sik; Han, Sang Hoon; Jin, Sung Joon; Chae, Yun Tae; Baek, Ji-Hyeon; Kim, Sun Bean; Kim, Do Young; Park, Jun Yong; Kim, June Myung

    2011-01-01

    From April 2008 to November 2008, many cases of hepatitis A were reported in Seoul and Gyeonggi Province in Korea. Furthermore, the rate of severe or fulminant hepatitis have significantly increased during the latest epidemic (13.4% vs. 5.2%, p=0.044). Therefore, widespread use of vaccine is warranted to reduce the burden of hepatitis A in Korea. PMID:21623615

  20. Clinical features of HBsAg seroclearance in hepatitis B virus carriers in South Korea: A retrospective longitudinal study

    PubMed Central

    Park, Young Min; Lee, Seong Gyu

    2016-01-01

    AIM To investigate the characteristic features of hepatitis B surface antigen (HBsAg) seroclearance among Korean hepatitis B virus (HBV) carriers. METHODS Carriers with HBsAg seroclearance were selected by analyzing longitudinal data collected from 2003 to 2015. The period of time from enrollment to the negative conversion of HBsAg (HBsAg-NC) was compared by stratifying various factors, including age, sex, hepatitis B e antigen (HBeAg), HBV DNA, sequential changes in the signal-to-cutoff ratio of HBsAg (HBsAg-SCR), as measured by qualitative HBsAg assay, and chronic liver disease on ultrasonography (US-CLD). Quantification of HBV DNA and HBsAg (HBsAg-QNT) in the serum was performed by commercial assay. RESULTS Among the 1919 carriers, 90 (4.7%) exhibited HBsAg-NC at 6.2 ± 3.6 years after registration, with no differences observed among the different age groups. Among these carriers, the percentages of those with asymptomatic liver cirrhosis (LC) and hepatocellular carcinoma (HCC) at registration were 31% and 7.8%, respectively. The frequency of HBsAg-NC significantly differed according to the HBV DNA titer and US-CLD. HBeAg influenced HBsAg-NC in the 40-50 and 50-60 year age groups. HBsAg-SCR < 1000 was correlated with an HBsAg-QNT < 200 IU/mL. A gradual decrease in the HBsAg-SCR to < 1000 predicted HBsAg-NC. Six patients developed HCC after registration, including two before and four after HBsAg-NC. The rate at which the patients developed new HCC after HBsAg seroclearance was 4.8%. LC with excessive drinking and vertical infection were found to be risk factors for HCC in the HBsAg-NC group. CONCLUSION HCC surveillance should be continued after HBsAg seroclearance. An HBsAg-SCR < 1000 and its decrease in sequential testing are worth noting as predictive markers of HBsAg loss. PMID:27956808

  1. Clinical features and risk factors for severe complications among patients with acute hepatitis A virus infection in the Jeonbuk Province of Korea.

    PubMed

    Yoo, Sun Ho; Kim, In Hee; Jang, Ji Won; Choi, Chung Hwan; Moon, Jin Chang; Park, Jin Kyoung; Lee, Sang Youn; Kim, Seong Hun; Kim, Sang Wook; Lee, Seung Ok; Lee, Soo Teik; Kim, Dae Ghon; Cho, Eun Young; Jung, Gum Mo

    2014-01-25

    The frequency of symptomatic acute HAV infections in adulthood are increasing in Korea. This study analyzes the clinical severity in patients with acute HAV infection and investigates risk factors associated with three severe complications: prolonged cholestasis, acute kidney injury, and acute liver failure. We performed a retrospective analysis of 726 patients diagnosed from January 2006 to December 2010 at three tertiary hospitals in Jeonbuk Province, Republic of Korea with acute HAV infection. In the group of 726 patients, the mean age was 30.3 years, 426 (58.6%) were male, and 34 (4.7%) were HBsAg positive. Severe complications from acute HAV infection occurred as follows: prolonged cholestasis in 33 (4.6%), acute kidney injury in 17 (2.3%), and acute liver failure in 16 (2.2%). Through multivariate analysis, age ≥40 years (OR 2.63, p=0.024) and peak PT (INR) ≥1.5 (OR 5.81, p=0.035) were found to be significant risk factors for prolonged cholestasis. Age ≥40 years (OR 5.24, p=0.002) and female gender (OR 3.11, p=0.036) were significant risk factors for acute kidney injury. Age ≥40 years (OR 6.91, p=0.002), HBsAg positivity (OR 5.02, p=0.049), and peak total bilirubin (OR 1.11, p=0.001) were significant risk factors for acute liver failure. Age ≥40 years, female gender, HBsAg positivity, peak PT (INR) ≥1.5, and peak total bilirubin were significant risk factors for severe complications in acute HAV infections.

  2. Clinical Characteristics of Cholinergic Urticaria in Korea

    PubMed Central

    Kim, Jung Eun; Eun, Young Sun; Park, Young Min; Park, Hyun Jeong; Yu, Dong Su; Kang, Hoon; Cho, Sang Hyun; Park, Chul Jong; Kim, Si Yong

    2014-01-01

    Background Cholinergic urticaria is a type of physical urticaria characterized by heat-associated wheals. Several reports are available about cholinergic urticaria; however, the clinical manifestations and pathogenesis are incompletely understood. Objective The purpose of this study was to investigate the clinical characteristics of cholinergic urticaria in Korea. Methods We performed a retrospective study of 92 patients with cholinergic urticaria who were contacted by phone and whose diagnoses were confirmed by the exercise provocation test among those who had visited The Catholic University of Korea, Catholic Medical Center from January 2001 to November 2010. Results All 92 patients were male, and their average age was 27.8 years (range, 17~51 years). Most of the patients had onset of the disease in their 20s and 30s. Non-follicular wheals were located on the trunk and upper extremities of many patients, and the symptoms were aggravated by exercise. Eight patients showed general urticaria symptoms and 15 had accompanying atopic disease. Forty-three patients complained of seasonal aggravation. Most patients were treated with first and second-generation antihistamines. Conclusion Dermatologists should consider these characteristics in patients with cholinergic urticaria. Further investigation and follow-up studies are necessary to better understand the epidemiological and clinical findings of cholinergic urticaria. PMID:24882973

  3. The Prevalence and Clinical Features of Non-responsive Gastroesophageal Reflux Disease to Practical Proton Pump Inhibitor Dose in Korea: A Multicenter Study.

    PubMed

    Park, Hong Jun; Park, Soo Heon; Shim, Ki Nam; Kim, Yong Sung; Kim, Hyun Jin; Han, Jae Pil; Kim, Yong Sik; Bang, Byoung Wook; Kim, Gwang Ha; Baik, Gwang Ho; Kim, Hyung Hun; Park, Seon Young; Kim, Sung Soo

    2016-07-25

    In Korea, there are no available multicenter data concerning the prevalence of or diagnostic approaches for non-responsive gastroesophageal reflux disease (GERD) which does not respond to practical dose of proton pump inhibitor (PPI) in Korea. The purpose of this study is to evaluate the prevalence and the symptom pattern of non-responsive GERD. A total of 12 hospitals who were members of a Korean GERD research group joined this study. We used the composite score (CS) as a reflux symptom scale which is a standardized questionnaire based on the frequency and severity of typical symptoms of GERD. We defined "non-responsive GERD" as follows: a subject with the erosive reflux disease (ERD) whose CS was not decreased by at least 50% after standard-dose PPIs for 8 weeks or a subject with non-erosive reflux disease (NERD) whose CS was not decreased by at least 50% after half-dose PPIs for 4 weeks. A total of 234 subjects were analyzed. Among them, 87 and 147 were confirmed to have ERD and NERD, respectively. The prevalence of non-responsive GERD was 26.9% (63/234). The rates of non-responsive GERD were not different between the ERD and NERD groups (25.3% vs. 27.9%, respectively, p=0.664). There were no differences between the non-responsive GERD and responsive GERD groups for sex (p=0.659), age (p=0.134), or BMI (p=0.209). However, the initial CS for epigastric pain and fullness were higher in the non-responsive GERD group (p=0.044, p=0.014, respectively). In conclusion, this multicenter Korean study showed that the rate of non-responsive GERD was substantially high up to 26%. In addition, the patients with the non-responsive GERD frequently showed dyspeptic symptoms such as epigastric pain and fullness.

  4. Chest CT Features of Cystic Fibrosis in Korea: Comparison with Non-Cystic Fibrosis Diseases

    PubMed Central

    Yang, So Yeon; Cha, Min Jae; Kim, Tae Jung; Kim, Tae Sung; Yoon, Hyun Jung

    2017-01-01

    Objective Cystic fibrosis (CF) is a rare congenital disease in Korea, and its clinical and imaging findings are unclear. The objective of our study was to describe the clinical and CT features of CF in Korea and compare its features with those of other diseases mimicking CF. Materials and Methods From November 1994 to December 2014, a presumptive diagnosis of CF was made in 23 patients based on clinical or radiological examination. After the exclusion of 10 patients without diagnostic confirmation, 13 patients were included in the study. A diagnosis of CF was made with the CF gene study. CT findings were evaluated for the presence and distribution of parenchymal abnormalities including bronchiectasis, tree-in-bud (TIB) pattern, mucus plugging, consolidation, and mosaic attenuation. Results Of the 13 patients, 7 (median age, 15 years) were confirmed as CF, 4 (median age, 19 years) had primary ciliary dyskinesia, 1 had bronchiectasis of unknown cause, and 1 had chronic asthma. CT of patients with CF showed bilateral bronchiectasis, TIB pattern, mosaic attenuation, and mucus plugging in all patients, with upper lung predominance (57%). In CT of the non-CF patients, bilateral bronchiectasis, TIB pattern, mosaic attenuation, and mucus plugging were also predominant features, with lower lung predominance (50%). Conclusion Korean patients with CF showed bilateral bronchiectasis, cellular bronchiolitis, mucus plugging, and mosaic attenuation, which overlapped with those of non-CF patients. CF gene study is recommended for the definitive diagnosis of CF in patients with these clinical and imaging features. PMID:28096734

  5. Episodic Ataxias: Clinical and Genetic Features

    PubMed Central

    Choi, Kwang-Dong; Choi, Jae-Hwan

    2016-01-01

    Episodic ataxia (EA) is a clinically heterogeneous group of disorders that are characterized by recurrent spells of truncal ataxia and incoordination lasting minutes to hours. Most have an autosomal dominant inheritance pattern. To date, 8 subtypes have been defined according to clinical and genetic characteristics, and five genes are known to be linked to EAs. Both EA1 and EA2, which are caused by mutations in KCNA1 and CACNA1A, account for the majority of EA, but many patients with no identified mutations still exhibit EA-like clinical features. Furthermore, genetically confirmed EAs have mostly been identified in Caucasian families. In this article, we review the current knowledge on the clinical and genetic characteristics of EAs. Additionally, we summarize the phenotypic features of the genetically confirmed EA2 families in Korea. PMID:27667184

  6. Clinical features of actinomycosis

    PubMed Central

    Bonnefond, Simon; Catroux, Mélanie; Melenotte, Cléa; Karkowski, Ludovic; Rolland, Ludivine; Trouillier, Sébastien; Raffray, Loic

    2016-01-01

    Abstract Actinomycosis is a rare heterogeneous anaerobic infection with misleading clinical presentations that delay diagnosis. A significant number of misdiagnosed cases have been reported in specific localizations, but studies including various forms of actinomycosis have rarely been published. We performed a multicenter retrospective chart review of laboratory-confirmed actinomycosis cases from January 2000 until January 2014. We described clinical characteristics, diagnostic procedures, differential diagnosis, and management of actinomycosis of clinical significance. Twenty-eight patients were included from 6 hospitals in France. Disease was diagnosed predominately in the abdomen/pelvis (n = 9), orocervicofacial (n = 5), cardiothoracic (n = 5), skeletal (n = 3), hematogenous (n = 3), soft tissue (n = 2), and intracranially (n = 1). Four patients (14%) were immunocompromised. In most cases (92 %), the diagnosis of actinomycosis was not suspected on admission, as clinical features were not specific. Diagnosis was obtained from either microbiology (50%, n = 14) or histopathology (42%, n = 12), or from both methods (7%, n = 2). Surgical biopsy was needed for definite diagnosis in 71% of cases (n = 20). Coinfection was found in 13 patients (46%), among which 3 patients were diagnosed from histologic criteria only. Two-thirds of patients were treated with amoxicillin. Median duration of antibiotics was 120 days (interquartile range 60–180), whereas the median follow-up time was 12 months (interquartile range 5.25–18). Two patients died. This study highlights the distinct and miscellaneous patterns of actinomycosis to prompt accurate diagnosis and earlier treatments, thus improving the outcome. Surgical biopsy should be performed when possible while raising histologist's and microbiologist's awareness of possible actinomycosis to enhance the chance of diagnosis and use specific molecular methods. PMID:27311002

  7. Clinical Characteristics of Marfan Syndrome in Korea.

    PubMed

    Lim, A Young; Song, Ju Sun; Kim, Eun Kyoung; Jang, Shin Yi; Chung, Tae-Young; Choi, Seung-Hyuk; Sung, Kiick; Huh, June; Kang, I-Seok; Choe, Yeon Hyeon; Ki, Chang-Seok; Kim, Duk-Kyung

    2016-11-01

    Marfan syndrome (MFS) is a connective tissue disorder with autosomal dominant inheritance and a highly variable clinical spectrum. However, there are limited data available on the clinical features of Korean patients with MFS. The aim of the present study was to describe the clinical characteristics and outcomes of Korean patients with MFS. We included all patients who were diagnosed with MFS between January 1995 and May 2015 at a single tertiary medical center. Patients with an MFS-related disorder including MASS phenotype (myopia, mitral valve prolapse, borderline and non-progressive aortic root dilatation, skeletal findings, and striae), mitral valve prolapse syndrome, and ectopia lentis syndrome were excluded. A total of 343 Korean patients aged ≥15 years who satisfied the revised Ghent nosology were included. The mean patient age at diagnosis was 35.9±12.6 years and 172 (50.1%) patients were male. Median follow-up duration was 52.8 months. A total of 303 patients (88.6%) had aortic root dilatation with Z score ≥2 or aortic root dissection. Ectopia lentis was relatively less common (163 patients, 55.1%) and systemic score ≥7 was found in 217 patients (73.8%). Among 219 probands, a family history of MFS was present in 97 patients (44.5%) and sporadic cases in 121 patients (55.5%). Among the 157 probands who underwent genetic analysis, 141 (89.8%) had an FBN1 mutation associated with aortic root aneurysm/dissection. Aortic dissection (AD) or intramural hematoma (IMH) was identified in 110 patients (32.1%). Among the 221 patients without AD or IMH, descending aortic aneurysms were identified in 19 patients (8.6%). Two hundred thirteen patients (62%) underwent cardiovascular surgery of any type. Eight patients died during follow-up. We described the clinical characteristics and outcomes of Korean MFS patients. Cardiovascular manifestations were commonly detected and FBN1 mutation was present in approximately 90% of patients. In contrast, ectopia lentis was

  8. Clinical Characteristics of Marfan Syndrome in Korea

    PubMed Central

    Lim, A Young; Song, Ju Sun; Kim, Eun Kyoung; Jang, Shin Yi; Chung, Tae-Young; Choi, Seung-Hyuk; Sung, Kiick; Huh, June; Kang, I-Seok; Choe, Yeon Hyeon; Ki, Chang-Seok

    2016-01-01

    Background and Objectives Marfan syndrome (MFS) is a connective tissue disorder with autosomal dominant inheritance and a highly variable clinical spectrum. However, there are limited data available on the clinical features of Korean patients with MFS. The aim of the present study was to describe the clinical characteristics and outcomes of Korean patients with MFS. Subjects and Methods We included all patients who were diagnosed with MFS between January 1995 and May 2015 at a single tertiary medical center. Patients with an MFS-related disorder including MASS phenotype (myopia, mitral valve prolapse, borderline and non-progressive aortic root dilatation, skeletal findings, and striae), mitral valve prolapse syndrome, and ectopia lentis syndrome were excluded. A total of 343 Korean patients aged ≥15 years who satisfied the revised Ghent nosology were included. Results The mean patient age at diagnosis was 35.9±12.6 years and 172 (50.1%) patients were male. Median follow-up duration was 52.8 months. A total of 303 patients (88.6%) had aortic root dilatation with Z score ≥2 or aortic root dissection. Ectopia lentis was relatively less common (163 patients, 55.1%) and systemic score ≥7 was found in 217 patients (73.8%). Among 219 probands, a family history of MFS was present in 97 patients (44.5%) and sporadic cases in 121 patients (55.5%). Among the 157 probands who underwent genetic analysis, 141 (89.8%) had an FBN1 mutation associated with aortic root aneurysm/dissection. Aortic dissection (AD) or intramural hematoma (IMH) was identified in 110 patients (32.1%). Among the 221 patients without AD or IMH, descending aortic aneurysms were identified in 19 patients (8.6%). Two hundred thirteen patients (62%) underwent cardiovascular surgery of any type. Eight patients died during follow-up. Conclusion We described the clinical characteristics and outcomes of Korean MFS patients. Cardiovascular manifestations were commonly detected and FBN1 mutation was present

  9. The Clinical Characteristics of Anisakis Allergy in Korea

    PubMed Central

    Choi, Sung-Jin; Lee, Jae-Chun; Kim, Moo-Jung; Hur, Gyu-Young; Shin, Seung-Youp

    2009-01-01

    Anisakidae larvae can cause anisakiasis when ingested by humans. Although several groups have reported a gastrointestinal Anisakis allergy among people in Spain and Japan, our report is the first to summarize the clinical features of 10 Anisakis allergy cases in Korea. We enrolled 10 Korean patients (6 men and 4 women) who complained of aggravated allergic symptoms after ingesting raw fish or seafood. Sensitization to Anisakis was confirmed by detecting serum specific IgE to Anisakis simplex. The most common manifestation of anisakiasis was urticaria (100%), followed by abdominal pain (30%) and anaphylaxis (30%). All patients presenting with these symptoms also exhibited high serum specific IgE (0.45 to 100 kU/L) to A. simplex. Nine patients (90%) exhibited atopy and increased total serum IgE levels. The fish species suspected of carrying the Anisakis parasite were flatfish (40%), congers (40%), squid (30%), whelk (10%), and tuna (10%). Anisakis simplex should be considered as a possible causative food allergen in adult patients presenting with urticaria, angioedema, and anaphylaxis following the consumption of raw fish or seafood. PMID:19543498

  10. Clinical features of elderly chronic urticaria

    PubMed Central

    Ban, Ga-Young; Kim, Mi-Yea; Yoo, Hye-Soo; Nahm, Dong-Ho; Ye, Young-Min; Shin, Yoo-Seob

    2014-01-01

    Background/Aims Chronic urticaria (CU) is defined as itchy wheals lasting 6 weeks or more. As the aged population increases worldwide, it is essential to identify the specific features of this disease in the elderly population. Methods We investigated the prevalence and clinical features of CU in elderly patients. Medical records of 837 CU patients from the outpatient Allergy Clinic of Ajou University Hospital, Korea were analyzed retrospectively. Patients with chronic spontaneous urticaria according to the EAACI/GA2LEN/EDF/WAO guidelines were included. Patients older than 60 years were defined as elderly. Results Of the 837 patients, 37 (4.5%) were elderly. In elderly versus nonelderly CU patients, the prevalence of atopic dermatitis (AD) was significantly higher (37.8% vs. 21.7%, respectively; p = 0.022), while that of aspirin intolerance was lower (18.9% vs. 43.6%, respectively; p = 0.003) in terms of comorbid conditions. The prevalences of serum specific immunoglobulin E antibodies to staphylococcal enterotoxin A and staphylococcal enterotoxin B were considerably higher in elderly CU patients with AD than in those without AD (37.5% vs. 0%, respectively). Conclusions Elderly patients with CU had a higher prevalence of AD. Therefore, there is a need to recognize the existence of AD in elderly CU patients. PMID:25378979

  11. Pemphigus in Korea: clinical manifestations and treatment protocol.

    PubMed

    Seo, Pan Gyo; Choi, Won Woo; Chung, Jin Ho

    2003-11-01

    Pemphigus, a rare, chronic blistering disease of the skin and mucous membranes with severe morbidity and occasional mortality, is the most common autoimmune bullous disease in Korea. The purpose of this study was to evaluate the clinical features and propose a treatment strategy for patients with pemphigus. A retrospective analysis was conducted of 51 pemphigus patients seen between 1993 and 2001. Pemphigus vulgaris (PV) was the most common type with 32 cases, followed by 19 cases of pemphigus foliaceus (PF). The male to female ratio was 1:1.3, with females predominating, particularly among PV patients (PV, 1:1.5; PF, 1:1.1). The average ages at onset of PV and PF were 44.3 and 51.0 years old, respectively. Mucosal involvement was noted in 27 cases (84.4%) of PV but in only 3 cases (15.8%) of PF. Most patients initially received relatively low to intermediate doses (0.3-1.0 mg/kg/day) of prednisolone, and 23 (71.9%) PV patients and 10 (52.6%) PF patients also received immunosuppressive agents. Oral prednisolone and azathioprine (100 mg/day) formed the mainstay of treatment for our patients (47.1%). At the time of writing, 25.5% (13/51) of patients are in complete remission, and 72.5% (37/51) are undergoing maintenance therapy. One patient died due to sepsis during the treatment. For the treatment of pemphigus, a course of the lowest possible corticosteroid dosage in combination with immunosuppressive agents appears to be effective and less toxic than a high corticosteroid dosage.

  12. Integrating clinical information in National Biobank of Korea.

    PubMed

    Kim, Hangchan; Yi, Byoung-Kee; Kim, Il Kon; Kwak, Yun Sik

    2011-08-01

    The National Biobank of Korea (NBK) is a government supported project that aims at consolidating various human-originated biomedical resources collected by individual hospitals nation-wide and integrating them with their donors' clinical information which researchers can take advantage of. In this paper, we present our experiences in developing the Clinical Information Integration System (CIIS) for NBK. The system automatically extracts clinical data from hospital information systems as much as possible to avoid errors from manual entry by human errors. It maintains the independence of individual hospitals by employing a two-layer approach, one of which takes care of all hospital-specific aspects. Interoperability is achieved by adopting HL7 v2.x messaging between the biobank and hospitals. We report the current status of the biobank and system deployments. We finally identify limitations and discuss how to improve them.

  13. [Multinational clinical trial in Japan and Korea on detrusitol].

    PubMed

    Yoshinaga, Yukio

    2009-02-01

    A randomized, double-blind, placebo- and active comparator-controlled study was conducted in 69 centers to compare detrusitol and oxybutynin with placebo in Japanese and Korean patients with an overactive bladder (OAB). Detrusitol had similar efficacy but was better tolerated than oxybutynin in Japanese and Korean patients with OAB. The study result was acknowledged as pivotal data in the clinical data package when NDA was filed and successfully approved both in Japan and Korea. Some differences were found in the efficacy and safety of the drug between the Japanese and Korean data, though. We therefore investigated the differences through stratified analysis; however exact causes could not be identified. This study is positioned as a first multinational clinical trial conducted in East Asia. From the aspects of utilization of interoperable data obtained from such multinational clinical trials for NDA filing and earliest possible registration of drugs in the participating countries, we believe it is important to accumulate more experiences in conducting multinational clinical trials. At this time, it is our prime task to minimize the "drug lag" in Japan; I think improving the speed of clinical trials is one of the factors to solve the issue. Global clinical trials involving Western and Asian countries make it possible to use the study data effectively and commonly in many countries. Moreover, from the viewpoint of revitalization of clinical trials, conducting global clinical trials is critically important; so we intend to continue accumulation of our experiences in global clinical trials.

  14. Acromegaly: clinical features at diagnosis.

    PubMed

    Vilar, Lucio; Vilar, Clarice Freitas; Lyra, Ruy; Lyra, Raissa; Naves, Luciana A

    2017-02-01

    Acromegaly is a rare and underdiagnosed disorder caused, in more than 95% of cases, by a growth hormone (GH)-secreting pituitary adenoma. The GH hypersecretion leads to overproduction of insulin-like growth factor 1 (IGF-1) which results in a multisystem disease characterized by somatic overgrowth, multiple comorbidities, physical disfigurement, and increased mortality. This article aims to review the clinical features of acromegaly at diagnosis. Acromegaly affects both males and females equally and the average age at diagnosis ranges from 40 to 50 years (up to 5% of cases < the age 20). Due to insidious onset and slow progression, acromegaly is often diagnosed five to more than ten years after its onset. The typical coarsening of facial features include furrowing of fronthead, pronounced brow protrusion, enlargement of the nose and the ears, thickening of the lips, skin wrinkles and nasolabial folds, as well as mandibular prognathism that leads to dental malocclusion and increased interdental spacing. Excessive growth of hands and feet (predominantly due to soft tissue swelling) is present in the vast majority of acromegalic patients. Gigantism accounts for up to 5% of cases and occurs when the excess of GH becomes manifest in the young, before the epiphyseal fusion. The disease also has rheumatologic, cardiovascular, respiratory, neoplastic, neurological, and metabolic manifestations which negatively impact its prognosis and patients quality of life. Less than 15% of acromegalic patients actively seek medical attention for change in appearance or enlargement of the extremities. The presentation of acromegaly is more often related to its systemic comorbidities or to local tumor effects.

  15. Clinical Features of Influenza and Acute Respiratory Illness in Older Adults at Least 50 Years of Age in an Outpatient Setting in the Republic of Korea: a Prospective, Observational, Cohort Study

    PubMed Central

    2017-01-01

    Two prospective, multi-centre, observational studies (GlaxoSmithKline [GSK] identifier No. 110938 and 112519) were performed over 2 influenza seasons (2007–2008 and 2008–2009) in the Republic of Korea (ROK) with the aim to evaluate the burden of laboratory-confirmed influenza (LCI) in patients ≥ 50 years of age seeking medical attention for acute respiratory illness (ARI). The median participant age was 58 years in the 2007–2008 season and 60 years in the 2008–2009 season. LCI was observed in 101/346 (29.2%) of ARI patients in the 2007–2008 season and in 166/443 (37.5%) of ARI patients in the 2008–2009 season. Compared to patients with non-influenza ARI, those with LCI had higher rates of decreased daily activities (60.4% vs. 32.9% in 2007–2008 and 46.4% vs. 25.8% in 2008–2009), work absenteeism (51.1% vs. 25.6% and 14.4% vs. 7.7%), and longer duration of illness. These results indicated that influenza is an important cause of ARI in adults aged 50 and older causing more severe illness than non-influenza related ARI. PMID:28145642

  16. Clinical Features of Influenza and Acute Respiratory Illness in Older Adults at Least 50 Years of Age in an Outpatient Setting in the Republic of Korea: a Prospective, Observational, Cohort Study.

    PubMed

    Kim, Woo Joo; Lee, Jin Soo; Lee, Chang Kyu; Cheong, Hee Jin; Kim, Mijeong; Monegal, Javier Sawchik; Carneiro, Rute; Kyaw, Moe H; Haguinet, François; Ray, Riju; Matias, Gonçalo

    2017-03-01

    Two prospective, multi-centre, observational studies (GlaxoSmithKline [GSK] identifier No. 110938 and 112519) were performed over 2 influenza seasons (2007-2008 and 2008-2009) in the Republic of Korea (ROK) with the aim to evaluate the burden of laboratory-confirmed influenza (LCI) in patients ≥ 50 years of age seeking medical attention for acute respiratory illness (ARI). The median participant age was 58 years in the 2007-2008 season and 60 years in the 2008-2009 season. LCI was observed in 101/346 (29.2%) of ARI patients in the 2007-2008 season and in 166/443 (37.5%) of ARI patients in the 2008-2009 season. Compared to patients with non-influenza ARI, those with LCI had higher rates of decreased daily activities (60.4% vs. 32.9% in 2007-2008 and 46.4% vs. 25.8% in 2008-2009), work absenteeism (51.1% vs. 25.6% and 14.4% vs. 7.7%), and longer duration of illness. These results indicated that influenza is an important cause of ARI in adults aged 50 and older causing more severe illness than non-influenza related ARI.

  17. Clinical Features of Eosinophilic Bronchitis

    PubMed Central

    Joo, Jae Hak; Park, Sang Joon; Park, Sung Woo; Lee, June Hyuk; Kim, Do Jin; Uh, Soo Taek; Kim, Yong Hoon; Park, Choon Sik

    2002-01-01

    Background Eosinophilic inflammation of the airway is usually associated with airway hyper-responsiveness in bronchial asthma. However, there is a small group of patients which has the eosinophilic inflammation in the bronchial tree with normal spirometry and no evidence of airway hyper-responsiveness, which was named eosinophilic bronchitis. The objectives of this study are 1) to investigate the incidence of eosinophilic bronchitis in the chronic cough syndrome and 2) to evaluate the clinical features and course of eosinophilic bronchitis. Methods We evaluated 92 patients who had persistent cough for 3 weeks or longer. In addition to routine diagnostic protocol, we performed differential cell count of sputum. Eosinophilic bronchitis was diagnosed when the patient had normal spirometric values, normal peak expiratory flow variability, no airway hyper-responsiveness and sputum eosinophilia (>3%). Results The causes of chronic cough were post-nasal drip in 33%, cough variant asthma in 16%, chronic bronchitis in 15% and eosinophilic bronchitis in 12% of the study subjects. Initial eosinophil percentage in the sputum of patients with eosinophilic bronchitis was 26.8±6.1% (3.8–63.7%). Treatment with inhaled steroid is related with a subjective improvement of cough severity and a significant decrease of sputum eosinophil percentage (from 29.1±8.3% to 7.4±3.3%). During the follow-up period, increase in sputum eosinophil percentage with aggravation of symptoms were found. Conclusion Eosinophilic bronchitis is one of the important cause of chronics cough. Assessment of airway inflammation by sputum examination is important in investigating the cause of chronic cough. Cough in eosinophilic bronchitis is effectively controlled by inhaled corticosteroid, but may follow a chronic course. PMID:12014210

  18. Scrub typhus in Korea: importance of early clinical diagnosis in this newly recognized endemic area.

    PubMed

    Yi, K S; Chong, Y; Covington, S C; Donahue, B J; Rothen, R L; Rodriguez, J; Arthur, J D

    1993-04-01

    Scrub typhus became a well recognized infectious disease threat to military operations in the Pacific Theater during World War II. Early diagnosis and treatment with tetracycline or chloramphenicol dramatically reduces the mortality and morbidity of this disease. Korea is a newly recognized scrub typhus endemic country. We report our experience with 189 scrub typhus patients seen at a civilian outpatient clinic in Chinhae, Republic of Korea, from 1985 through 1990, and verify the accuracy of clinical diagnosis by serologic tests.

  19. Clinical Review of 20 Cases of Addison’s Disease in Korea

    PubMed Central

    Sung, Sang Kyu; Kwon, Yong Joon; Lee, Bum Woo; Kim, Doo Man; Yoo, Hyung Joon

    1988-01-01

    Addison’s disease is a rare disorder resulting from a chronic deficiency of adrenol cortical hormone. The clinical manifestations and data of 20 patients with Addison’s disease were reviewed. They include 14 previously reported cases, total cases in korea upto 1987, and 6 new cases at National Medical Center. 13 patients were male and 7 female. The mean age was 39 years and ranged from 16 to 61 years.Common symptoms included mucocutaneous pigmentation (95 %), general weakness (90%), nasuea and/or vomiting (80%), and weight loss (60%).Initial mean blood pressure was 96±21/63±14 mmHg and adrenal crises were found in 5 cases (25%).Laboratory data indicated hyponatremia and hyperkalemia, resulting in a decreased Na/K ratio. Serum basal cortisol and ACTH levels were 2.86±1.93 ug/dl and 482.5/269.3 pg/ml, respectively.Extra-adrenal tuberculosis was present in 14 cases (70%) as a very high incidence.These 14 cases (70%) were presumed to be due to adrenal tuberculosis, although only two cases were confirmed as such by histopathology. The other cases were likely to be non-tuberculous. Thus, tuberculosis may be-considered as a predominant cause of Addision’s disease in Korea. Other features were not unusual. But further detailed and extensive studies are necessary including more pathologically confirmed cases. PMID:3153796

  20. Clinical Characteristics of Spinal Muscular Atrophy in Korea Confirmed by Genetic Analysis.

    PubMed

    Hwang, Heewon; Lee, Jung Hwan; Choi, Young Chul

    2017-09-01

    The objective of this study was to review the clinical characteristics of patients with spinal muscular atrophy and to emphasize the importance of performing genetic mutational analysis at initial patient assessment. This is a single center oriented, retrospective, and descriptive study conducted in Seoul, South Korea. Genetic mutational analysis to detect the deletion of exon 7 of the SMN1 gene on chromosome 5q13 was performed by multiplex ligation-dependent probe amplification. Clinical features, electrodiagnostic study results, muscle biopsy results, and laboratory test results were reviewed from patient medical records. Of all 28 patients (15 males and 13 females), all showed bilateral symmetric proximal dominant weakness. Among them, 3 patients were classified as type I, 14 patients as type II, and 11 patients as type III. Twenty-five patients had scoliosis and eight of these patients received surgical treatment for scoliosis with improvement in clinical outcomes. Ventilator support was used in 15 patients. In terms of the diagnostic process, 15 patients had completed an electrodiagnostic study and muscle biopsy before genetic testing, and six of these patients were initially misdiagnosed with myopathy. Owing to the similar clinical features of SMA and congenital myopathy, an electrodiagnostic study and muscle biopsy could create confusion in the correct diagnosis in some cases. Therefore, it is recommended that genetic mutation analysis should be conducted along with an electrodiagnostic study or muscle biopsy in the diagnostic process for spinal muscular atrophy. © Copyright: Yonsei University College of Medicine 2017.

  1. Rosacea: clinical features and treatment.

    PubMed

    Lavers, Isabel

    2016-03-30

    Rosacea is a chronic inflammatory skin condition that predominantly affects the central face. It is characterised by a variable range of symptoms, including erythema, telangiectasia, papules, pustules and changes in skin texture. Rosacea may be transient, recurrent or persistent. Because it affects the most visible part of the body, the psychosocial effects of this condition can be significant. This article describes the features and management of the condition.

  2. Clinical features of legionnaires' disease.

    PubMed

    Cunha, B A

    1998-06-01

    Legionnaires' disease is a systemic infectious disease primarily involving the lungs, with multisystemic extrapulmonary manifestations. Any species of Legionella may cause legionnaires' disease in normal and compromised hosts. The clinical diagnosis of legionnaires' disease may be made on the basis of associated extrapulmonary clinical and laboratory findings. Although no single finding in legionnaires' disease is pathognomonic, the association of key extrapulmonary constitutes a typical pattern that is diagnostically characteristic. The syndromic approach based on a weighted point evaluation system described in the article gives physicians a system to arrive at a rapid presumptive clinical diagnosis of legionnaires' disease. Definitive diagnosis of legionnaires' disease is by direct fluorescent antibody testing of respiratory specimens, serological methods, Legionella urinary antigenuria, or culture.

  3. Comparative epidemiological features of Japanese encephalitis in the Republic of Korea, China (Taiwan) and Japan

    PubMed Central

    Kono, Reisaku; Kim, Kyong Ho

    1969-01-01

    The epidemiology of Japanese encephalitis in the Republic of Korea from 1949 to 1966 is described and comparisons are made with the situations in Japan and China (Taiwan). Some similarities and some differences are noted. Recent epidemics in Korea coincided with those in southern Japan but the annual fluctuations were more distinct in Korea. The disease mainly affected children in Korea and, in contrast to the situation in Japan, persons in the older age-groups were rarely affected. The authors also discuss the geographical pathology of Japanese encephalitis in Korea. PMID:4308334

  4. Clinical features of gastroenteropancreatic tumours

    PubMed Central

    Czarnywojtek, Agata; Bączyk, Maciej; Ziemnicka, Katarzyna; Fischbach, Jakub; Wrotkowska, Elżbieta; Ruchała, Marek

    2015-01-01

    Gastroenteropancreatic (GEP) endocrine tumours (carcinoids and pancreatic islet cell tumours) are composed of multipotent neuroendocrine cells that exhibit a unique ability to produce, store, and secrete biologically active substances and cause distinct clinical syndromes. The classification of GEP tumours as functioning or non-functioning is based on the presence of symptoms that accompany these syndromes secondary to the secretion of hormones, neuropeptides and/or neurotransmitters (functioning tumours). Non-functioning tumours are considered to be neoplasms of neuroendocrine differentiation that are not associated with obvious symptoms attributed to the hypersecretion of metabolically active substances. However, a number of these tumours are either capable of producing low levels of such substances, which can be detected by immunohistochemistry but are insufficient to cause symptoms related to a clinical syndrome, or alternatively, they may secrete substances that are either metabolically inactive or inappropriately processed. In some cases, GEP tumours are not associated with the production of any hormone or neurotransmitter. Both functioning and non-functioning tumours can also produce symptoms due to mass effects compressing vital surrounding structures. Gastroenteropancreatic tumours are usually classified further according to the anatomic site of origin: foregut (including respiratory tract, thymus, stomach, duodenum, and pancreas), midgut (including small intestine, appendix, and right colon), and hindgut (including transverse colon, sigmoid, and rectum). Within these subgroups the biological and clinical characteristics of the tumours vary considerably, but this classification is still in use because a significant number of previous studies, mainly observational, have used it extensively. PMID:26516377

  5. 2014 Clinical Practice Guidelines for Overweight and Obesity in Korea

    PubMed Central

    Kim, Mee Kyoung; Lee, Won-Young; Kang, Jae-Heon; Kang, Jee-Hyun; Kim, Bom Taeck; Kim, Seon Mee; Kim, Eun Mi; Suh, Sang-Hoon; Shin, Hye Jung; Lee, Kyu Rae; Lee, Ki Young; Lee, Sang Yeoup; Lee, Seon Yeong; Lee, Seong-Kyu; Lee, Chang Beom; Chung, Sochung; Jeong, In Kyung; Hur, Kyung Yul

    2014-01-01

    The dramatic increase in the prevalence of obesity and its accompanying comorbidities are major health concerns in Korea. Obesity is defined as a body mass index ≥25 kg/m2 in Korea. Current estimates are that 32.8% of adults are obese: 36.1% of men and 29.7% of women. The prevalence of being overweight and obese in national surveys is increasing steadily. Early detection and the proper management of obesity are urgently needed. Weight loss of 5% to 10% is the standard goal. In obese patients, control of cardiovascular risk factors deserves the same emphasis as weight-loss therapy. Since obesity is multifactorial, proper care of obesity requires a coordinated multidisciplinary treatment team, as a single intervention is unlikely to modify the incidence or natural history of obesity. PMID:25559568

  6. Clinical Presentation and Outcomes of Middle East Respiratory Syndrome in the Republic of Korea.

    PubMed

    Choi, Won Suk; Kang, Cheol-In; Kim, Yonjae; Choi, Jae-Phil; Joh, Joon Sung; Shin, Hyoung-Shik; Kim, Gayeon; Peck, Kyong Ran; Chung, Doo Ryeon; Kim, Hye Ok; Song, Sook Hee; Kim, Yang Ree; Sohn, Kyung Mok; Jung, Younghee; Bang, Ji Hwan; Kim, Nam Joong; Lee, Kkot Sil; Jeong, Hye Won; Rhee, Ji-Young; Kim, Eu Suk; Woo, Heungjeong; Oh, Won Sup; Huh, Kyungmin; Lee, Young Hyun; Song, Joon Young; Lee, Jacob; Lee, Chang-Seop; Kim, Baek-Nam; Choi, Young Hwa; Jeong, Su Jin; Lee, Jin-Soo; Yoon, Ji Hyun; Wi, Yu Mi; Joung, Mi Kyong; Park, Seong Yeon; Lee, Sun Hee; Jung, Sook-In; Kim, Shin-Woo; Lee, Jae Hoon; Lee, Hyuck; Ki, Hyun Kyun; Kim, Yeon-Sook

    2016-06-01

    From May to July 2015, the Republic of Korea experienced the largest outbreak of Middle East respiratory syndrome (MERS) outside the Arabian Peninsula. A total of 186 patients, including 36 deaths, had been diagnosed with MERS-coronavirus (MERS-CoV) infection as of September 30th, 2015. We obtained information of patients who were confirmed to have MERS-CoV infection. MERS-CoV infection was diagnosed using real-time reverse-transcriptase polymerase chain reaction assay. The median age of the patients was 55 years (range, 16 to 86). A total of 55.4% of the patients had one or more coexisting medical conditions. The most common symptom was fever (95.2%). At admission, leukopenia (42.6%), thrombocytopenia (46.6%), and elevation of aspartate aminotransferase (42.7%) were observed. Pneumonia was detected in 68.3% of patients at admission and developed in 80.8% during the disease course. Antiviral agents were used for 74.7% of patients. Mechanical ventilation, extracorporeal membrane oxygenation, and convalescent serum were employed for 24.5%, 7.1%, and 3.8% of patients, respectively. Older age, presence of coexisting medical conditions including diabetes or chronic lung disease, presence of dyspnea, hypotension, and leukocytosis at admission, and the use of mechanical ventilation were revealed to be independent predictors of death. The clinical features of MERS-CoV infection in the Republic of Korea were similar to those of previous outbreaks in the Middle East. However, the overall mortality rate (20.4%) was lower than that in previous reports. Enhanced surveillance and active management of patients during the outbreak may have resulted in improved outcomes.

  7. Moyamoya Disease: Epidemiology, Clinical Features, and Diagnosis

    PubMed Central

    Kim, Jong S.

    2016-01-01

    Moyamoya disease (MMD) is a chronic, occlusive cerebrovascular disease characterized by progressive stenosis at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. Although its etiology remains unknown, recent genetic studies identified RNF213 in the 17q25-ter region as an important susceptibility gene of MMD among East Asian populations. Possibly because of genetic differences, MMD is relatively common in people living in East Asian countries such as Korea and Japan, compared to those in the Western Hemisphere. The prevalence of MMD appears to be slightly lower among Chinese, compared to Koreans or Japanese. There are two peaks of incidence with different clinical presentations, at around 10 years and 30-40 years. The peak appears to occur later in women than men. In children, ischemic symptoms, especially transient ischemic attacks, are predominant. Intellectual decline, seizures, and involuntary movements are also more common in this age group. In contrast, adult patients present with intracranial hemorrhage more often than pediatric patients. In patients with MMD, intracerebral hemorrhage is more often accompanied by intraventricular hemorrhage than in patients with hypertensive intracerebral hemorrhage. These different age peaks and different clinical presentations in each age group are also observed in MMD patients in the USA. Catheter angiography is the diagnostic method of choice. Magnetic resonance (MR) angiography and computed tomographic angiography are noninvasive diagnostic methods. High-resolution vessel wall MR imaging also helps diagnose MMD by revealing concentric vessel wall narrowing with basal collaterals. PMID:26846755

  8. Analysis of seasonal water pollution based on rainfall feature at Anyang river basin in Korea

    NASA Astrophysics Data System (ADS)

    Han, J. G.; Lee, Y. K.; Kim, T. H.; Hwang, E. J.

    2005-08-01

    To determine selected water pollution parameters of the Anyang River (one of the biggest contributory branches of the Han River in Korea) and its main tributaries, the geological and topographical and rainfall features in its basin were investigated, and the resulting data were tabulated. Samples were collected at the upper, mid and down parts of the Anyang River and its branches and were analyzed based on biochemical and chemical methods, Korean biotic index (KBI) and Saprobien systems. Selected parameters of concern include BOD, heavy metals, nonpoint pollution and sewage discharge. The Anyang River basin has a torrential heavy rainfall; however, the rate of rainfall significantly varies from season to season. Water pollution levels in the dry season increase dramatically. The mainstream of the Anyang River is classified as fifth grade polysaprobic water according to Saprobien system. In addition, the biotic index is over 2.5 in overall. General pollution at the junction of the Anyang River and each branch stream varies. Possible countermeasures to improve the water quality of the river include intercept the non-treated waste water and sewage at the Anyang River junction and each branch stream, enforcement of water management during the rainy season, and continuous investment on environmental restoration.

  9. Clinical characteristics of late-onset neuromyelitis optica spectrum disorder: A multicenter retrospective study in Korea.

    PubMed

    Seok, Jin Myoung; Cho, Hye-Jin; Ahn, Suk-Won; Cho, Eun Bin; Park, Min Su; Joo, In-Soo; Shin, Ha Young; Kim, Sun-Young; Kim, Byung-Jo; Kim, Jong Kuk; Cho, Joong-Yang; Huh, So-Young; Kwon, Ohyun; Lee, Kwang-Ho; Kim, Byoung Joon; Min, Ju-Hong

    2017-01-01

    There are currently few studies regarding late-onset neuromyelitis optica spectrum disorder (LO-NMOSD). We aimed to describe the characteristic features of patients with LO-NMOSD in Korea. Anti-aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder (NMOSD) from nine tertiary hospitals were reviewed retrospectively. The patients were divided into two groups based on age of onset: LO-NMOSD (⩾50 years of age at onset) versus early-onset neuromyelitis optica spectrum disorder (EO-NMOSD) (<50 years of age at onset). Clinical, laboratory, and magnetic resonance imaging (MRI) parameters were investigated. Among a total of 147 patients (125 female; age of onset, 39.4 ± 15.2 years), 45 patients (30.6%) had an age of onset of more than 50 years. Compared to patients with EO-NMOSD, patients with LO-NMOSD had more frequent isolated spinal cord involvement at onset (64.4% vs 37.2%, p = 0.002), less frequent involvement of the optic nerve (40.0% vs 67.7%, p = 0.002), and less frequent brain MRI lesions (31.1% vs 50.0%, p = 0.034). Furthermore, there was a significant positive correlation between age of onset and Expanded Disability Status Scale (EDSS) score at last follow-up ( r = 0.246, p = 0.003). Age of onset could be an important predictor of lesion location and clinical course of patients with NMOSD.

  10. Korea University Joins the International Efforts in Clinical Proteogenomics Cancer Research | Office of Cancer Clinical Proteomics Research

    Cancer.gov

    The National Cancer Institute's (NCI) Office of Cancer Clinical Proteomics Research, part of the National Institutes of Health, and Korea University (KU) located in The Republic of Korea have signed a Memorandum of Understanding (MOU) in clinical proteogenomics cancer research. The MOU between NCI and KU represents the twenty-ninth institution and eleventh country to join the continued effort of the International Cancer Proteogenome Consortium (ICPC), an effort catalyzed through the vision of the 47th Vice President of the United States Joseph R. Biden, Jr. and the Cancer Moonshot.

  11. Mitochondrial disorders: clinical and genetic features.

    PubMed

    Simon, D K; Johns, D R

    1999-01-01

    Virtually all cells in humans depend on mitochondrial oxidative phosphorylation to generate energy, accounting for the remarkable diversity of clinical disorders associated with mitochondrial DNA mutations. However, certain tissues are particularly susceptible to mitochondrial dysfunction, resulting in recognizable clinical syndromes. Mitochondrial DNA mutations have been linked to seizures, strokes, optic atrophy, neuropathy, myopathy, cardiomyopathy, sensorineural hearing loss, diabetes mellitus, and other clinical features. Mitochondrial DNA mutations also may play an important role in aging, as well as in common age-related neurodegenerative disorders such as Parkinson's disease. Therefore, it is becoming increasingly important for clinicians to recognize the clinical syndromes suggestive of a mitochondrial disorder, and to understand the unique features of mitochondrial genetics that complicate diagnosis and genetic counseling.

  12. [Clinical features of hypokalemic periodic paralysis].

    PubMed

    Wang, Wei; Zhao, Chun-yun; Gao, Yan-ming

    2009-12-18

    To explore the clinical features of hypokalemic periodic paralysis, and compare clinical features of primary group with those of thyrotoxicosis secondary group. Clinical data of 44 patients with hypokalemic periodic paralysis in Peking University First Hospital from 1996 December to 2008 December were retrospectively analyzed. There were 22 patients in primary group, and 22 in thyrotoxicosis group. Identical clinical features of both the groups: (1)It had a predilection in young men. (2)Main symptoms were limb movement disorder and fatigue, and paralysis recurrent attacked in most patients. (3) 40.9% to 68.2% patients had obvious incentives, and the common ones were a heavy meal, sweet drinks, or strenuous exercise. (4) Serum potassium levels of the two groups were obviously lower than the normal range. (5)In 20% patients of primary group and 25% patients of thyrotoxicosis secondary group, CK levels were higher than normal, while LDH and HBDH levels were normal. (6)The doses of potassium replishment were not correlated to serum potassium levels at the onset. Different clinical features of the two groups: (1) Patients of thyrotoxicosis group had hypermetabolism symptoms and thyroid dysfunction. Patients of primary group had no hypermetabolism symptoms, and all of them were euthyroid. (2)Serum potassium levels of thyrotoxicosis secondary group were lower than those of primary group significantly [(2.25 +/- 0.67) vs (2.78 +/- 0.49) mmol/L, P=0.007]. (3) Hyperkalemia is easier than primary group to rebound in thyrotoxicosis secondary group, after replenishment of potassium. Hypokalemic periodic paralysis has its clinical features, and patients with early diagnosis and replenishment of potassium in time have good prognosis. The doses of potassium replenishment are not determined by serum potassium levels at the onset. Hyperkalemia is easier to rebound in thyrotoxicosis secondary group after replenishment of potassium, serum potassium levels should be monitored closely

  13. Clinical and imaging features of fludarabine neurotoxicity.

    PubMed

    Lee, Michael S; McKinney, Alexander M; Brace, Jeffrey R; Santacruz, Karen

    2010-03-01

    Neurotoxicity from intravenous fludarabine is a rare but recognized clinical entity. Its brain imaging features have not been extensively described. Three patients received 38.5 mg or 40 mg/m per day fludarabine in a 5-day intravenous infusion before bone marrow transplantation in treatment of hematopoietic malignancies. Several weeks later, each patient developed progressive neurologic decline, including retrogeniculate blindness, leading to coma and death. Brain MRI showed progressively enlarging but mild T2/FLAIR hyperintensities in the periventricular white matter. The lesions demonstrated restricted diffusion but did not enhance. Because the neurotoxicity of fludarabine appears long after exposure, neurologic decline in this setting is likely to be attributed to opportunistic disease. However, the imaging features are distinctive in their latency and in being mild relative to the profound clinical features. The safe dose of fludarabine in this context remains controversial.

  14. Molecular mechanisms of carbapenem resistance in Enterobacter cloacae clinical isolates from Korea and clinical outcome.

    PubMed

    Lee, Yangsoon; Choi, Heekyeong; Yum, Jong Hwa; Kang, Girung; Bae, Il Kwon; Jeong, Seok Hoon; Lee, Kyungwon

    2012-01-01

    We investigated the molecular mechanisms of carbapenem resistance in clinical isolates of Enterobacter cloacae and their clinical characteristics. Nonreplicable E. cloacae isolates were recovered from six cancer patients and one patient with liver cirrhosis at a tertiary-care hospital in Korea between 2002 and 2009. Two patients who were considered to have a true infection caused by these microorganisms have died. All isolates produced AmpC β-lactamases, including ACT-1, ACT-2, MIR-3 and DHA-1, and CTX-M- or SHV-type extended-spectrum β-lactamase. Two isolates produced plasmid-borne VIM-2 carbapenemase. All probes specific for bla(AmpC) genes hybridized with I-CeuI chromosomal fragments were also recognized by a probe specific for 16S rDNA, suggesting a chromosomal location. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis showed that a major outer membrane protein, OmpF, was absent in all isolates. PFGE of XbaI-digested DNA were considered to be unrelated. The results of our study suggest that the chromosomal AmpC β-lactamase with impermeability in E. cloacae clinical isolates implicated in reduced carbapenem susceptibility. Although carbapenem-resistant E. cloacae isolates were isolated in a few patients in our study, the clinical outcomes were grave. Therefore, the patients colonized or infected by carbapenem-resistant E. cloacae isolates should gain attention of antibiotic therapy.

  15. Clinical Significance of Component Allergens in Fagales Pollen-Sensitized Peanut Allergy in Korea

    PubMed Central

    Park, Kyung Hee; Son, Young Woong; Lee, Sang Chul; Jeong, Kyunguk; Sim, Da Woon; Park, Hye Jung; Lee, Sooyoung; Lee, Jae-Hyun

    2016-01-01

    Purpose Clinical features of peanut allergy can range from localized to systemic reactions. Because peanut and birch pollen have cross-reactivity, peanut can lead to localized allergic reaction in Fagales pollen-sensitized oral allergy syndrome (OAS) patients without peanut sensitization per se. The purpose of this study was to discriminate true peanut food allergy from cross-reactive hypersensitivity in birch-sensitized peanut allergy. Methods Birch-sensitized (n=81) and peanut anaphylaxis patients (n=12) were enrolled. Peanut-related allergic reactions and sensitization profiles were examined. Specific IgE to Fagales tree pollens (birch, oak), peanut, and their component allergens (Bet v 1, Bet v 2, Ara h 1, Ara h 2, Ara h 3, Ara h 8, and Ara h 9) were evaluated. Based on these specific IgEs and clinical features, the patients were classified into 4 groups: group 1 (Fagales pollen allergy without OAS), group 2 (Fagales pollen allergy with OAS), group 3 (OAS with peanut anaphylaxis), and group 4 (peanut anaphylaxis). Results After peanut consumption, one-third of OAS patients experienced oral symptoms not associated with peanut sensitization. Ara h 1 or Ara h 2 was positive in peanut anaphylaxis patients, whereas Ara h 8 was positive in OAS patients. There were 4 patients with both peanut anaphylaxis and OAS (group 3). Both Ara h 2 and Ara h 8 were positive in these patients. Foods associated with OAS in Korea showed unique patterns compared to Westernized countries. Conclusions Ara h 2 and Ara h 8 may be important component allergens for discriminating peanut allergy. PMID:27582401

  16. Clinical Significance of Component Allergens in Fagales Pollen-Sensitized Peanut Allergy in Korea.

    PubMed

    Park, Kyung Hee; Son, Young Woong; Lee, Sang Chul; Jeong, Kyunguk; Sim, Da Woon; Park, Hye Jung; Lee, Sooyoung; Lee, Jae Hyun; Park, Jung Won

    2016-11-01

    Clinical features of peanut allergy can range from localized to systemic reactions. Because peanut and birch pollen have cross-reactivity, peanut can lead to localized allergic reaction in Fagales pollen-sensitized oral allergy syndrome (OAS) patients without peanut sensitization per se. The purpose of this study was to discriminate true peanut food allergy from cross-reactive hypersensitivity in birch-sensitized peanut allergy. Birch-sensitized (n=81) and peanut anaphylaxis patients (n=12) were enrolled. Peanut-related allergic reactions and sensitization profiles were examined. Specific IgE to Fagales tree pollens (birch, oak), peanut, and their component allergens (Bet v 1, Bet v 2, Ara h 1, Ara h 2, Ara h 3, Ara h 8, and Ara h 9) were evaluated. Based on these specific IgEs and clinical features, the patients were classified into 4 groups: group 1 (Fagales pollen allergy without OAS), group 2 (Fagales pollen allergy with OAS), group 3 (OAS with peanut anaphylaxis), and group 4 (peanut anaphylaxis). After peanut consumption, one-third of OAS patients experienced oral symptoms not associated with peanut sensitization. Ara h 1 or Ara h 2 was positive in peanut anaphylaxis patients, whereas Ara h 8 was positive in OAS patients. There were 4 patients with both peanut anaphylaxis and OAS (group 3). Both Ara h 2 and Ara h 8 were positive in these patients. Foods associated with OAS in Korea showed unique patterns compared to Westernized countries. Ara h 2 and Ara h 8 may be important component allergens for discriminating peanut allergy.

  17. Genotypes of Clinical and Environmental Isolates of Cryptococcus neoformans and Cryptococcus gattii in Korea

    PubMed Central

    Park, So Hae; Choi, Seok Cheol; Lee, Kyung Won; Kim, Mi-Na

    2015-01-01

    Multilocus sequence typing analysis was applied to determine the genotypes of 147 (137 clinical and 10 environmental) Cryptococcus neoformans and three clinical Cryptococcus gattii isolates from 1993 to 2014 in Korea. Among the 137 clinical isolates of C. neoformans, the most prevalent genotype was ST5 (n = 131), followed by ST31 (n = 5) and ST127 (n = 1). Three C. gattii strains were identified as ST57, ST7, and ST113. All environmental isolates were identified as C. neoformans with two genotypes, ST5 (n = 7) and ST31 (n = 3). Our results show that C. neoformans isolates in Korea are genetically homogeneous, and represent a close genetic relationship between clinical and environmental isolates. PMID:26539057

  18. Clinical and radiographic maxillofacial features of pycnodysostosis

    PubMed Central

    Alves, Nilton; Cantín, Mario

    2014-01-01

    The aim of this study was to review of the literature to determine the radiographic and clinical maxillofacial features of pycnodysostosis emphasizing the main aspects of interest to the dentist in order to make them fit for the proper treatment of this population. It is important to make the diagnosis as early as possible in order to plan the treatment more suitable to provide a better life’s quality to the patients. The most frequent clinical maxillofacial features were: grooved palate, midfacial hypoplasia, mandibular hypoplasia and enamel hypoplasia. The most common radiographic maxillofacial features were: obtuse mandibular angle, frontal/parietal/occiptal bossing, open fontanels and sutures, multiple impacted teeth. The earlier diagnostic of pycnodysostosis has a fundamental role in general health of the patients. We consider that is very important that the dentist know recognize the radiographic and clinical maxillofacial features of pycnodysostosis, which allows correct treatment planning avoiding risks and ensuring better life’s quality to the patients. PMID:24753741

  19. Clinical constructions by nurses in Korea, Norway, and the United States.

    PubMed

    Kim, Hesook Suzie; Ellefsen, Bodil; Kyung Ja Han; Alves, Steve L

    2008-02-01

    Nursing practice involves engagement of nurses in clinical fields through deliberation and enactment. In the phase of deliberation, nurses observe, recognize, form ideas about and decide on clinical situations, and construct clinical pictures. Clinical pictures are critically connected to nursing enactments, thus it is important to discover how nurses arrive at clinical pictures. The purpose of this article is to describe how nurses construct meanings of clinical situations and arrive at specific clinical pictures. The results are from a clinical fieldwork study replicated in Korea, Norway, and the United States, with samples of nurses working in acute-care hospitals. Data were collected through participant observations and in-depth interviews. A general model of clinical construction was derived from the results, which specifies four dimensions (i.e., problem, progress, status, and particularism) as the bases for clinical picture evocations. Clinical pictures are constituted by nurses' elicitations of meanings of clinical situations on these dimensions.

  20. Clinical features and prognosis for intraventricular liponeurocytoma

    PubMed Central

    Xu, Nini; Cai, Jinxiu; Du, Jiang; Yang, Rong; Zhu, Huachen; Gao, Peiyi; Zhou, Jian; Li, Xiaofeng

    2017-01-01

    Cerebellar liponeurocytoma is a rare central nervous system tumor, we investigate its biological behaviors and clinical prognosis to improve the understanding of this tumor. We retrospectively analyzed the clinical, radiological and histopathological findings as well as follow-up data of two patients with intraventricular liponeurocytomas in Beijing Tiantan Hospital between July 2000 and July 2016. The main clinical manifestations of the two patients were headache. The supratentorial intraventricular liponeurocytoma appeared as isodense to slight hyperdense on CT scan and heterogeneous intensity on T1-weighted imaging (T1WI) and T2-weighted imaging (T2WI). The plaque-like hypodense on CT images and hyperintensity on T1WI resembling fat could be seen inside the tumor. The liponeurocytoma located in the fourth ventricle showed isointensity on T1 and T2WI as well as slight enhancement on contrast. Two patients accepted gross total resection of tumors. Two intraventricular tumors demonstrated similarly histopathological features, such as isomorphic small tumor cells with clear cytoplasm, sheets of monomorphic round cells and focal lipomatous differentiation. In addition, expression of synaptophysin, neuron specific enolase, microtubule-associated protein 2 and S-100 were found. No radiological or clinical evidence of recurrence of the tumors was observed in their follow-up surveys. In conclusion, intraventricular liponeurocytoma has a favorable clinical course, radiological features may be useful in the diagnosis of this rare tumor before surgery. PMID:28977976

  1. Clinical features and prognosis for intraventricular liponeurocytoma.

    PubMed

    Xu, Nini; Cai, Jinxiu; Du, Jiang; Yang, Rong; Zhu, Huachen; Gao, Peiyi; Zhou, Jian; Li, Xiaofeng

    2017-09-22

    Cerebellar liponeurocytoma is a rare central nervous system tumor, we investigate its biological behaviors and clinical prognosis to improve the understanding of this tumor. We retrospectively analyzed the clinical, radiological and histopathological findings as well as follow-up data of two patients with intraventricular liponeurocytomas in Beijing Tiantan Hospital between July 2000 and July 2016. The main clinical manifestations of the two patients were headache. The supratentorial intraventricular liponeurocytoma appeared as isodense to slight hyperdense on CT scan and heterogeneous intensity on T1-weighted imaging (T1WI) and T2-weighted imaging (T2WI). The plaque-like hypodense on CT images and hyperintensity on T1WI resembling fat could be seen inside the tumor. The liponeurocytoma located in the fourth ventricle showed isointensity on T1 and T2WI as well as slight enhancement on contrast. Two patients accepted gross total resection of tumors. Two intraventricular tumors demonstrated similarly histopathological features, such as isomorphic small tumor cells with clear cytoplasm, sheets of monomorphic round cells and focal lipomatous differentiation. In addition, expression of synaptophysin, neuron specific enolase, microtubule-associated protein 2 and S-100 were found. No radiological or clinical evidence of recurrence of the tumors was observed in their follow-up surveys. In conclusion, intraventricular liponeurocytoma has a favorable clinical course, radiological features may be useful in the diagnosis of this rare tumor before surgery.

  2. Spinal infections: clinical and imaging features.

    PubMed

    Arbelaez, Andres; Restrepo, Feliza; Castillo, Mauricio

    2014-10-01

    Spinal infections represent a group of rare conditions affecting vertebral bodies, intervertebral discs, paraspinal soft tissues, epidural space, meninges, and spinal cord. The causal factors, clinical presentations, and imaging features are a challenge because the difficulty to differentiate them from other conditions, such as degenerative and inflammatory disorders and spinal neoplasm. They require early recognition because delay diagnosis, imaging, and intervention may have devastating consequences especially in children and the elderly. This article reviews the most common spinal infections, their pathophysiologic, clinical manifestation, and their imaging findings.

  3. Cellulitis: definition, etiology, and clinical features.

    PubMed

    Gunderson, Craig G

    2011-12-01

    Cellulitis is a common condition seen by physicians. Over the past decade, skin and soft tissue infections from community-associated methicillin-resistant Staphylococcus aureus have become increasingly common. In this article, the definition, etiology, and clinical features of cellulitis are reviewed, and the importance of differentiating cellulitis from necrotizing soft tissue infections is emphasized. Empiric antimicrobial recommendations are suggested, including the most recent recommendations from the Infectious Disease Society of America.

  4. Clinical Characteristics of Microscopic Colitis in Korea: Prospective Multicenter Study by KASID

    PubMed Central

    Baek, Dae Hyun; Kim, Won Ho; Kim, Joo Sung; Yang, Suk-Kyun; Jung, Sung-Ae; Jang, Byung Ik; Choi, Chnag Hwan; Han, Dong Soo; Kim, Young-Ho; Chung, Yong Woo; Kim, Sang Woo; Kim, You Sun

    2011-01-01

    Background/Aims Microscopic colitis (MC) encompasses collagenous and lymphocytic colitis and is characterized by chronic diarrhea. In cases of MC, colonic mucosae are macroscopically normal, and diagnostic histopathological features are observed only upon microscopic examination. We designed a prospective multicenter study to determine the clinical features, pathological distribution in the colon and prevalence of MC in Korea. Methods We prospectively enrolled patients having watery diarrhea no more than 3 times a day between March 2008 and February 2009. We obtained patient histories and performed colonoscopies with random biopsies at each colon segment. Results A total of 100 patients with chronic diarrhea were enrolled for a normal colonoscopy and stool exam. MC was observed in 22 patients (22%) (M:F 1.2:1; mean age, 47.5 years). Of those 22 patients, 18 had lymphocytic colitis and 4 had collagenous colitis. The entire colon was affected in only 3 cases (13.6%), the ascending colon in 6 cases (27.2%), the transverse colon in 3 cases (13.6%), and the left colon in 3 cases (13.6%). More than 2 segments were affected in 7 cases (31.8%). Nonsteroidal anti-inflammatory drug-associated MCs were observed in 4 cases (18.2%), 3 of which showed improved diarrhea symptoms following discontinuation of the medication. Frequently associated symptoms were abdominal pain and weight loss. Autoimmune diseases were observed in 4 cases (18.2%). Half of the 22 patients with MC improved with conservative care by loperamide or probiotics. Conclusions In a prospective multicenter study of Korean patients with chronic diarrhea, the frequency of MC was found to be approximately 20%, similar to the percentage observed in Western countries. Therefore, the identification of MC is important for the adequate management of Korean patients with chronic diarrhea. PMID:21814598

  5. Epidemiological features and surveillance performance of measles in the Republic of Korea, 2002-2011.

    PubMed

    Choe, Young June; Park, Young-Joon; Eom, Hye Suk; Kim, Eun Seong; Bae, Geun-Ryang; Lee, Jong-Koo

    2013-01-01

    Measles was declared eliminated from the Republic of Korea in 2006; however, recently the number of reported cases has been gradually increasing. To address this issue, we summarized the measles surveillance data collected during 2002-2011, and aimed to evaluate the performance of the current surveillance system in Korea. We analyzed data from the national surveillance system to describe the occurrence of measles. Surveillance indicators proposed by the World Health Organization were used to evaluate the performance of the current measles surveillance system. Between 2002 and 2005, a gradual decrease in confirmed cases of measles was noted, whereas cyclical increases were noted from 2006 to 2011. Since 2006, confirmed cases of measles were more likely to be identified by laboratory methods. In general, the incidence of confirmed case was less than one per million in Korea; however, this figure increased in 2002 (1.3/million), 2007 (4.0), and 2010 (2.3). Most cases were occurred in the age groups 0-23 months and 12-17 years. Laboratory testing was performed in most suspected cases; however, the proportion of discarded cases was low. Overall, more than half of the reported cases experienced an onset of symptoms from April to June. The incidence of measles is relatively low in Korea, and the laboratory surveillance may have helped in identifying under-diagnosed cases within the country. It remains important to continuously assess the surveillance data to improve the surveillance performance.

  6. Epidemiologic Features of Animal Bite Cases Occurring in Rabies-Endemic Areas of Korea, 2005 to 2009

    PubMed Central

    Han, Myung Guk; Jung Sang, Ryou; Jeong, Young Eui; Ju, Young Ran; Cho, Jung Eun; Park, Jun-Sun

    2012-01-01

    Objectives Human rabies is a reemerging infectious disease in Korea. There was no human rabies case for 14 years until the disease had reoccurred in 1999. To prevent occurrence of human rabies, surveillance for animal bite patients in rabies endemic areas in Korea was conducted since 2005 as a part of a human rabies control program. The animal bite cases were analyzed to determine whether patients were treated according to the post-exposure prophylaxis (PEP) guideline of the Korea Centers for Disease Control and Prevention. Methods Information of animal bite cases that occurred from 2005 to 2009 in rabies high-risk regions were collected by cooperation with Regional Public Health Centers in 18 cities/districts of rabies endemic areas. Results A total of 2458 animal bite cases were reported. Dogs accounted for 86% of animal bites and 67% of the animals were not vaccinated against rabies virus. For PEP, among rabies-vaccinated animals, 92.7% were observed for clinical signs and 1.4% underwent necropsy. Among unvaccinated animals, 72.7% were observed for clinical signs and 4.1% underwent necropsy. The remaining animals were not available for examination. Of the animal bite patients, 32.5% received PEP and 51.6% were treated by first aid or by washing the wound. Conclusions Given that no human rabies cases were reported since 2005 and animal rabies was continuously reported in endemic areas of Korea, the human rabies control program implemented in 2005 appears to have a significant role in the prevention and control of human rabies. PMID:24159481

  7. Clinical features of neuromyelitis optica in children

    PubMed Central

    Ness, Jayne; Krupp, Lauren; Waubant, Emmanuelle; Hunt, Tyler; Olsen, Cody S.; Rodriguez, Moses; Lotze, Tim; Gorman, Mark; Benson, Leslie; Belman, Anita; Weinstock-Guttman, Bianca; Aaen, Greg; Graves, Jennifer; Patterson, Marc; Rose, John W.; Casper, T. Charles

    2016-01-01

    Objective: To compare clinical features of pediatric neuromyelitis optica (NMO) to other pediatric demyelinating diseases. Methods: Review of a prospective multicenter database on children with demyelinating diseases. Case summaries documenting clinical and laboratory features were reviewed by an adjudication panel. Diagnoses were assigned in the following categories: multiple sclerosis (MS), acute disseminated encephalomyelitis, NMO, and recurrent demyelinating disease not otherwise specified. Results: Thirty-eight cases of NMO were identified by review panel, 97% of which met the revised International Panel on NMO Diagnosis NMO-SD 2014 criteria, but only 49% met 2006 Wingerchuk criteria. Serum or CSF NMO immunoglobulin G (IgG) was positive in 65% of NMO cases that were tested; however, some patients became seropositive more than 3 years after onset despite serial testing. No patient had positive CSF NMO IgG and negative serum NMO IgG in contemporaneous samples. Other than race (p = 0.02) and borderline findings for sex (p = 0.07), NMO IgG seropositive patients did not differ in demographic, clinical, or laboratory features from seronegatives. Visual, motor, and constitutional symptoms (including vomiting, fever, and seizures) were the most common presenting features of NMO. Initiation of disease-modifying treatment was delayed in NMO vs MS. Two years after onset, patients with NMO had higher attack rates, greater disability accrual measured by overall Expanded Disability Status Scale score, and visual scores than did patients with MS. Conclusion: The new criteria for NMO spectrum disorders apply well to the pediatric setting, and given significant delay in treatment of NMO compared to pediatric MS and worse short-term outcomes, it is imperative to apply these to improve access to treatment. PMID:26683648

  8. Prevalence and Clinical Relevance of Exon 2 Deletion of COMMD1 in Bedlington Terriers in Korea.

    PubMed

    Kim, Y G; Kim, S Y; Kim, J H; Lee, K K; Yun, Y M

    2016-11-01

    Deletion of exon 2 of copper metabolism domain containing 1 (COMMD1) results in copper toxicosis in Bedlington terriers (CT-BT). This study was conducted to identify the prevalence and clinical relevance of the COMMD1 mutation in Bedlington terriers in Korea. A total of 105 purebred Bedlington terriers (50 males, 55 females) from the kennels and pet dog clubs in Korea were examined during the period 2008-2013. A multiplex PCR was carried out to detect exon 2 deletion of COMMD1. Clinical analysis was performed on each genetic group, and clinical status of the dogs was followed up to estimate survival probability. Of the 105 samples, 52 (49%) were wild-type homozygote, 47 (45%) were heterozygote, and 6 (6%) were mutant-type homozygote. Plasma alanine aminotransferase (ALT) activity was increased in the mutant-type homozygous group >2 years of age (P < .0001). The survival probability of 6 mutant-type homozygotes surviving 2.5 years was 0.67, and 4 years was 0.5. Results show the prevalence and clinical relevance of exon 2 deletion of COMMD1 and could help establish a structured selective breeding program to prevent CT-BT in Korea. Copyright © 2016 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.

  9. Primary Sjogren syndrome: clinical and immunopathologic features.

    PubMed

    Fox, R I; Howell, F V; Bone, R C; Michelson, P

    1984-11-01

    Primary Sjogren syndrome is an autoimmune condition in which dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia) result from lymphocytic infiltration of lacrimal and salivary glands. Clinical and laboratory features of 60 primary Sjogren syndrome patients seen at our clinic during the past three years are presented. These patients illustrate the wide spectrum of extraglandular features that may occur as a result of lymphoid infiltration of lung, kidney, skin, stomach, liver, and muscle. They further emphasize the difficulty in classifying a patient as primary or secondary Sjogren syndrome (ie, sicca symptoms associated with systemic lupus erythematosus, rheumatoid arthritis, or scleroderma), particularly early in the disease course. As an initial step in understanding the pathogenesis, the lymphocytes that infiltrate the salivary glands and lymph nodes were characterized by using monoclonal antibodies that recognize distinct lymphocyte subsets and by using in vitro functional assays. These studies have demonstrated that affected tissues have infiltrates of T cells with helper/inducer activity and with a high frequency of "activation antigens." The immunohistologic techniques are useful in differentiating "benign" and "pseudolymphoma" lesions (both due predominantly to T cells) from non-Hodgkin lymphoma (usually due to B-cell infiltrates). Although there is no "cure" for primary Sjogren syndrome patient's symptoms may be significantly improved by measures aimed at prevention of ocular and dental complications and by the recognition of extraglandular features that may be amenable to specific treatment.

  10. Epidemiological and clinical features of Minamata disease.

    PubMed

    Igata, A

    1993-10-01

    Minamata disease is methyl mercury intoxication from fish contaminated by a chemical factory in Minamata city. Based on the results of our regional survey, cardinal clinical features of the disease were clarified by a multivariant analysis of all symptoms in inhabitants in the polluted area. The clinical features were found to be essentially the same as those of Hunter Russell syndrome; however, some additional symptoms were also found. Those symptoms are influenced by many factors, such as degree of exposure and duration of pollution. The disposition of each inhabitant also plays a role in clinical manifestation. This analysis contributes to a correct individual diagnosis and to the correct estimation of patients in polluted areas. Long-term studies also uncovered a few inhabitants who claimed to have begun to experience some neurological symptoms after pollution ceased. These symptoms were attributed mainly to aging. As many inhabitants with mild neurological complaints were not easily diagnosed, a questionable borderline group should be postulated for social settlement of Minamata disease. The characteristics of Minamata disease are discussed and compared to cases of methyl mercury poisoning in other parts of the world.

  11. Basal cell carcinoma: clinical and pathological features.

    PubMed

    Di Stefani, A; Chimenti, S

    2015-08-01

    Basal cell carcinoma (BCC) is a slow-growing, locally invasive malignant epidermal skin neoplasm that represents the most common malignancy in Caucasians. The clinical presentation of BCC can be extremely variable: nodular, ulcerative, superficial, morpheiform, pigmented, and fibroepithelioma of Pinkus are the main clinical variants described. Clinical factors influencing negatively prognosis of BCC are: anatomic location, recurrence and/or persistance at site after treatment, and tumor size. A precise correlations between clinical and histopathological variants is not always possible, especially in biopsy samples. From a histopathological point of view various subtypes has been described: nodular, superficial, infiltrating, morpheiform, micronodular, fibroepithelial BCC and basosquamous carcinoma. A classification system based by growth pattern allows the identification of high-risk subtypes with potential tumor recurrence and aggressive biologic behavior such as infiltrating, morpheiform, micronodular and basosquamous subtypes. Further histopathological aspects determining high risk clinical morbidity are the level of invasion, perineural and lymphovascular invasion, involved surgical margins. The awareness of these clinicopathological features is helpful to better select the appropriate treatment management.

  12. Neuromyelitis optica: clinical features, immunopathogenesis and treatment

    PubMed Central

    Jarius, S; Wildemann, B; Paul, F

    2014-01-01

    The term ‘neuromyelitis optica’ (‘Devic's syndrome’, NMO) refers to a syndrome characterized by optic neuritis and myelitis. In recent years, the condition has raised enormous interest among scientists and clinical neurologists, fuelled by the detection of a specific serum immunoglobulin (Ig)G reactivity (NMO-IgG) in up to 80% of patients with NMO. These autoantibodies were later shown to target aquaporin-4 (AQP4), the most abundant water channel in the central nervous system (CNS). Here we give an up-to-date overview of the clinical and paraclinical features, immunopathogenesis and treatment of NMO. We discuss the widening clinical spectrum of AQP4-related autoimmunity, the role of magnetic resonance imaging (MRI) and new diagnostic means such as optical coherence tomography in the diagnosis of NMO, the role of NMO-IgG, T cells and granulocytes in the pathophysiology of NMO, and outline prospects for new and emerging therapies for this rare, but often devastating condition. Other Articles published in this series Paraneoplastic neurological syndromes. Clinical and Experimental Immunology 2014, 175: 336–48. Disease-modifying therapy in multiple sclerosis and chronic inflammatory demyelinating polyradiculoneuropathy: common and divergent current and future strategies. Clinical and Experimental Immunology 2014, 175: 359–72. Monoclonal antibodies in treatment of multiple sclerosis. Clinical and Experimental Immunology 2014, 175: 373–84. CLIPPERS: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Review of an increasingly recognized entity within the spectrum of inflammatory central nervous system disorders. Clinical and Experimental Immunology 2014, 175: 385–96. Requirement for safety monitoring for approved multiple sclerosis therapies: an overview. Clinical and Experimental Immunology 2014, 175: 397–407. Myasthenia gravis: an update for the clinician. Clinical and Experimental Immunology 2014, 175: 408

  13. Current Status of Electronic Medical Record Systems in Hospitals and Clinics in Korea.

    PubMed

    Park, Young-Taek; Han, Dongwoon

    2017-07-01

    Many healthcare organizations and professionals have had interests in healthcare information and communication technology (ICT). The objective of this study was to investigate the current status of overall healthcare ICT, especially focusing on Electronic Medical Record (EMR) systems in Korea. This study used a part of the nationwide survey collected for the OECD benchmarking ICT study. The Health Insurance Review and Assessment Service conducted the survey from November 19, 2013 to January 10, 2014. This study followed the methodological guidelines of the OECD. A total of 2,093 hospitals and clinics, including long-term care hospitals, participated in the survey. Among them, 554 hospitals and 906 clinics were included in this study for the generalization of the results. The adoption rates of EMR systems were 96.3% in hospitals and 95.7% in clinics. Most of the hospitals and clinics had high rates of healthcare information exchange (HIE) within the organization; however, there were extremely low HIE rates among external organizations. Most of the hospitals and clinics had EMR systems with clinical-decision-supporting functionalities. Ninety-six percent of the EMR systems of the hospitals and 89.2% of the clinic systems had checking functions, such as alerts or reminders, on contraindications of drug-drug and drug-age interaction. Korea has maintained a high healthcare ICT status compared to countries in the European Union. The EMR systems of hospitals and clinics in Korea had sophisticated functionalities; however, their HIE status was extremely low, which indicates the need for healthcare ICT standardization.

  14. Clinical and microbiological characterization of serotype 6D pneumococcal infections in South Korea.

    PubMed

    Cheong, Hee Jin; Song, Joon Young; Choi, Min Joo; Jeon, Ji Ho; Kang, Seong Hee; Jeong, Eun Joo; Noh, Ji Yun; Kim, Woo Joo

    2016-08-01

    The prevalence of Serotype 6D Streptococcus pneumoniae was reported relatively high in South Korea. Since the introduction of 7-valent pneumococcal conjugate vaccine (PCV7), serotype replacement was observed. This study was designed to better clarify genetic diversity of pneumococcal serotype 6D and its clinical characteristics after introduction of PCV7 in 2000. We performed serotyping analysis with 1298 pneumococcal isolates from clinical specimens in South Korea from 2004 to 2011. Multilocus sequence typing was performed, and minimal inhibitory concentration was determined for the available serotype 6D and nontypeable (NT) pneumococcal isolates during the 2006-2007 period. The proportion of serotype 6D pneumococci increased from 0.8% (2004-2007) to 2.9% (2008-2011) of all clinical pneumococcal isolates, accounting for 14.9% of serogroup 6 pneumococci in South Korea. NT pneumococci markedly increased to 13.3% during 2006-2007 in advance of the increase in serotype 6D. Among the 26 available serotype 6D pneumococcal isolates, ST282 was predominant (23 isolates, 88.5%). The STs of NT pneumococci (26 isolates) were diverse, but clonal complex 271 was the dominant clone. The oral penicillin non-susceptibility rate was 92.3% (24 among 26 isolates) for both serotype 6D and NT pneumococci. The ceftriaxone non-susceptibility rates of serotype 6D and NT pneumococci were 7.7% and 3.8%, respectively. ST228(6D) strain expanded, particularly among old adults with comorbidities in South Korea. Both antibiotic and PCV7 pressure might have contributed to the selective increase of NT and serotype 6D pneumococci. Copyright © 2016 Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

  15. [Clinical and hematologic features of pediatric leukemias].

    PubMed

    Hasanbegović, Edo

    2006-01-01

    to present main clinical and hematologic features of pediatric leukemias treated at Hematooncologic department of Pediatric Clinic in Sarajevo during last 7 years. In retrospective study we followed up children with leukemia aged 0-15 who were treated during period of 01.01.1997-31.12.2003. at Hematooncologic department on Pediatric Clinic in Sarajevo. A total number of patient with leukemia was 130 of them 112 (83.2%) had acute lymphoblastic leukemia (ALL), 16 (12.3%) of them had acute myeloid leukemia (AML) and 2 (1.5%) patients had chronic myeloid leukemia (CML). There were 84 (64.6%) boys and 46 (35.4%) girls. Median age of newly diagnosed patients was 6 years and 4 months. Dominant clinical signs were: high temperature-72.9%, fatigue and paleness-74.8% and bone pain-87.9%. Most of the children had leucocitosis (51.5%), anemia (56.1%) and trombocitopenia (57.5%). Most frequent signs at the beginning of the illness are general symptoms like fatigue, unclear febrile state and accented bone pains. Those united signs with complete blood picture finding should be enough reason for suspicion under possible leukemia.

  16. Biochemical and clinical features of hereditary hyperprolinemia.

    PubMed

    Mitsubuchi, Hiroshi; Nakamura, Kimitoshi; Matsumoto, Shirou; Endo, Fumio

    2014-08-01

    There are two classifications of hereditary hyperprolinemia: type I (HPI) and type II (HPII). Each type is caused by an autosomal recessive inborn error of the proline metabolic pathway. HPI is caused by an abnormality in the proline-oxidizing enzyme (POX). HPII is caused by a deficiency of Δ-1-pyrroline-5-carboxylate (P5C) dehydrogenase (P5CDh). The clinical features of HPI are unclear. Nephropathy, uncontrolled seizures, mental retardation or schizophrenia have been reported in HPI, but a benign phenotype without neurological problems has also been reported. The clinical features of HPII are also unclear. In addition, the precise incidences of HPI and HPII are unknown. Only two cases of HPI and one case of HPII have been identified in Japan through a questionnaire survey and by a study of previous reports. This suggests that hyperprolinemia is a very rare disease in Japan, consistent with earlier reports in Western countries. The one case of HPII found in Japan was diagnosed in an individual with influenza-associated encephalopathy. This suggests that HPII might reduce the threshold for convulsions, thereby increasing the sensitivity of individuals with influenza-associated encephalopathy. The current study presents diagnostic criteria for HPI and HPII, based on plasma proline level, with or without measurements of urinary P5C. In the future, screening for HPI and HPII in healthy individuals, or patients with relatively common diseases such as developmental disabilities, epilepsy, schizophrenia or behavioral problems will be important.

  17. Ossified spinal meningiomas: Clinical and surgical features.

    PubMed

    Alafaci, Concetta; Grasso, Giovanni; Granata, Francesca; Salpietro, Francesco M; Tomasello, Francesco

    2016-03-01

    Meningiomas constitute 25% of primary spinal tumors and predominantly involve the thoracic spinal cord. Although calcifications are commonly seen in intracranial meningiomas, gross calcifications are observed in only 1-5% of all spinal meningiomas. We report the clinical findings, surgical strategy and histological features of 9 patients with ossified spinal meningiomas (OSMs). Clinical and surgical features of 9 patients with ossified spinal meningiomas were retrospectively reviewed. There were 8 women and 1 man with a mean age of 59 years. In 7 patients, the lesions were localized in the thoracic segment of the spine while in 2 patients in the lower cervical segment. All patients presented with weakness of the lower limbs and hypoesthesia below the site level of the lesion. Only 2 patients presented with urinary incontinence. Gross-total resection of the tumor was achieved in 6 patients while in 3 a subtotal removal of the meningioma was obtained. In all patients the postoperative course was uneventful. Six patients presented with a significant neurological improvement while in 3 patients a mild improvement was observed. Microscopically, all tumors showed typical histological pattern of ossified meningioma. OSMs are amenable to surgery if the complete removal can be achieved. Because of their hard-rock consistency complete resection can be challenging. In difficult cases, subtotal removal can be advised and follow-up imaging is mandatory. Overall, the risk of long-term recurrence of the lesions is low, and a good clinical outcome after total or subtotal removal can be expected. Copyright © 2016 Elsevier B.V. All rights reserved.

  18. Clinical features of congenital retinal folds.

    PubMed

    Nishina, Sachiko; Suzuki, Yumi; Yokoi, Tadashi; Kobayashi, Yuri; Noda, Eiichiro; Azuma, Noriyuki

    2012-01-01

    To investigate the clinical features and prognosis of congenital retinal folds without systemic associations. Retrospective observational case series. The characteristics, clinical course, ocular complications, and best-corrected visual acuity (BCVA) of eyes with congenital retinal folds were studied during the follow-up periods. The affected and fellow eyes were examined by slit-lamp biomicroscopy, binocular indirect ophthalmoscopy, and fundus fluorescein angiography. The parents and siblings of each patient also underwent ophthalmoscopic examinations. The BCVA was measured using a Landolt ring VA chart. One hundred forty-seven eyes of 121 patients with congenital retinal folds were examined. Fifty-five patients (45.5%) were female. The fold was unilateral in 95 patients (78.5%), and 69 of those patients (72.6%) had retinal abnormalities in the fellow eye. The meridional distribution of folds was temporal in 136 eyes (92.5%). The family history was positive in 32 patients (26.4%). Secondary fundus complications, including fibrovascular proliferation and tractional, rhegmatogenous, and exudative retinal detachments, developed in 44 eyes (29.9%). The BCVAs could be measured in 119 eyes and ranged from 20/100 to 20/20 in 5 eyes (4.2%), 2/100 to 20/200 in 45 eyes (37.8%), and 2/200 or worse in 69 eyes (58.0%). The follow-up periods ranged from 4 to 243 months (mean, 79.7 ± 58.9 months). These clinical features suggested that most congenital retinal folds may result from insufficient retinal vascular development, as in familial exudative vitreoretinopathy, rather than persistent fetal vasculature. Adequate management of active retinopathy and late-onset complications, especially retinal detachment, is required. Copyright © 2012 Elsevier Inc. All rights reserved.

  19. Autoimmune uveitis: clinical, pathogenetic, and therapeutic features.

    PubMed

    Prete, Marcella; Dammacco, Rosanna; Fatone, Maria Celeste; Racanelli, Vito

    2016-05-01

    Autoimmune uveitis (AU), an inflammatory non-infectious process of the vascular layer of the eye, can lead to visual impairment and, in the absence of a timely diagnosis and suitable therapy, can even result in total blindness. The majority of AU cases are idiopathic, whereas fewer than 20 % are associated with systemic diseases. The clinical severity of AU depends on whether the anterior, intermediate, or posterior part of the uvea is involved and may range from almost asymptomatic to rapidly sight-threatening forms. Race, genetic background, and environmental factors can also influence the clinical picture. The pathogenetic mechanism of AU is still poorly defined, given its remarkable heterogeneity and the many discrepancies between experimental and human uveitis. Even so, the onset of AU is thought to be related to an aberrant T cell-mediated immune response, triggered by inflammation and directed against retinal or cross-reactive antigens. B cells may also play a role in uveal antigen presentation and in the subsequent activation of T cells. The management of AU remains a challenge for clinicians, especially because of the paucity of randomized clinical trials that have systematically evaluated the effectiveness of different drugs. In addition to topical treatment, several different therapeutic options are available, although a standardized regimen is thus far lacking. Current guidelines recommend corticosteroids as the first-line therapy for patients with active AU. Immunosuppressive drugs may be subsequently required to treat steroid-resistant AU and for steroid-sparing purposes. The recent introduction of biological agents, such as those targeting tumor necrosis factor-α, is expected to remarkably increase the percentages of responders and to prevent irreversible sight impairment. This paper reviews the clinical features of AU and its crucial pathogenetic targets in relation to the current therapeutic perspectives. Also, the largest clinical trials

  20. Electroencephalographic and clinical features of cerebral malaria

    PubMed Central

    Crawley, J; Smith, S; Muthinji, P; Marsh, K; Kirkham, F

    2001-01-01

    BACKGROUND—Seizures are a prominent feature of childhood cerebral malaria, and are associated with an increased risk of death and neurological sequelae. We present the electroencephalographic (EEG) findings from a detailed clinical and electrophysiological study.
METHODS—Children with cerebral malaria had EEGs recorded within six hours of admission, and at 12 hourly intervals until recovery of consciousness. Ten deeply comatose children underwent intracranial pressure monitoring. Children were not mechanically ventilated, which made it possible to directly correlate the clinical and EEG findings.
RESULTS—Of 65 children aged 9 months and above, 40 had one or more seizures, and 18 had an episode of status epilepticus. Most seizures were partial motor, and spike wave activity consistently arose from the posterior temporo-parietal region, a border zone area lying between territories supplied by the carotid and vertebrobasilar circulations. Fifteen children had seizures that were clinically subtle or electrographic. Clinical seizures were associated with an abrupt rise in intracranial pressure. Fifty children recovered fully, seven died, and eight had persistent neurological sequelae. Initial EEG recordings of very slow frequency, or with background asymmetry, burst suppression, or interictal discharges, were associated with an adverse outcome.
CONCLUSIONS—Serial EEG recording has uncovered a range of clinical, subtle, and electrographic seizures complicating childhood cerebral malaria, and has emphasised their importance in the pathogenesis of coma. Further work is required to determine the most appropriate regimen for the prophylaxis and treatment of seizures in cerebral malaria, in order to improve outcome.

 PMID:11207176

  1. [Clinical features of acute hepatitis A in recent two years].

    PubMed

    Lee, Eung Jun; Kwon, So Young; Seo, Tae Ho; Yun, Hyun Sung; Cho, Han Su; Kim, Byung Kook; Choe, Won Hyeok; Lee, Chang Hong; Kim, Jin Nam; Yim, Hyung Joon

    2008-11-01

    The purpose of this study was to characterize the clinical features of acute hepatitis A in Seoul and Gyeonggi province during the recent 2 years. We reviewed the medical records of 222 patients who were diagnosed as acute hepatitis A between August 2005 and March 2007 at the Konkuk University Hospital and Korea University, Ansan Hospital. The clinical manifestation, serological tests, and image findings were analyzed. Median age of the patients was 28.1 years and the age groups of highest incidence were the second and third decade. The frequent symptoms were anorexia (66.4%), fatigue (49.2%), fever (47.7%), and abdominal discomfort (42.5%). Fourteen cases (6.3%) showed renal insufficiency, and hemodialysis was performed in one. Cholestatic hepatitis in 2 cases, relapsing hepatitis in 4 cases and prolonged hepatitis in 13 cases were observed. However, there was no case of fulminant hepatitis or death. The underlying diseases including chronic hepatitis B, diabetes mellitus and thyroid disorder did not affect the disease severity of hepatitis A. IgM anti-HAV was not detected initially in 6.7% of the patients. Anti-HEV (IgM) was detected simultaneously in 3 of 150 patients. The age of patients with acute hepatitis A has been increased in the recent years. Most patients recovered uneventfully. However, unusual patterns of severe hepatitis and renal insufficiency occurred in considerable number of cases. Follow-up serologic test for IgM anti-HAV is needed in seronegative cases with hepatitis A.

  2. Clinical and pathological features of pachyonychia congenita.

    PubMed

    Leachman, Sancy A; Kaspar, Roger L; Fleckman, Philip; Florell, Scott R; Smith, Frances J D; McLean, W H Irwin; Lunny, Declan P; Milstone, Leonard M; van Steensel, Maurice A M; Munro, Colin S; O'Toole, Edel A; Celebi, Julide T; Kansky, Aleksej; Lane, E Birgitte

    2005-10-01

    Pachyonychia congenita (PC) is a rare genodermatosis affecting the nails, skin, oral mucosae, larynx, hair, and teeth. Pathogenic mutations in keratins K6a or K16 are associated with the PC-1 phenotype whereas K6b and K17 mutations are associated with the PC-2 phenotype. Analysis of clinical, pathological, and genetic data from the literature and two research registries reveal that >97% of PC cases exhibit fingernail and toenail thickening, and painful plantar keratoderma. Prospective evaluation of 57 PC patients from 41 families revealed variable clinical findings: hyperhidrosis (79%), oral leukokeratosis (75%), follicular keratosis (65%), palmar keratoderma (60%), cutaneous cysts (35%), hoarseness or laryngeal involvement (16%), coarse or twisted hair (26%), early primary tooth loss (14%), and presence of natal or prenatal teeth (2%). Stratification of these data by keratin mutation confirmed the increased incidence of cyst formation and natal teeth among PC-2 patients, although cysts were more commonly seen in PC-1 than previously reported (25%-33%). Previously unreported clinical features of PC include development of painful oral and nipple lesions during breastfeeding, copious production of waxy material in ears, and inability to walk without an ambulatory aid (50%). Possible pathogenic mechanisms are discussed with respect to the clinicopathologic and genetic correlations observed.

  3. The clinical outcomes of chronic hepatitis C in South Korea

    PubMed Central

    Ok, Kyeong Sam; Jeong, Sook-Hyang; Jang, Eun Sun; Kim, Young Seok; Lee, Youn Jae; Kim, In Hee; Cho, Sung Bum; Bae, Si Hyun; Lee, Han Chu

    2016-01-01

    Abstract This prospective cohort study aimed to elucidate the clinical outcome and its related factors of chronic hepatitis C in a hepatitis B-dominant Asian region. From January 2007 to October 2012, 382 patients with chronic hepatitis C without liver cirrhosis were prospectively enrolled at 6 university hospitals, and regularly followed until Apr 2014 to identify the development of liver cirrhosis, decompensated cirrhosis, hepatocellular carcinoma (HCC), and overall survival. During the median follow-up of 39.0 months (range 18.0–81.0 months), liver cirrhosis, hepatic decompensation, and HCC developed in 42 patients (11.0%), 4 patients (1.0%), and 12 patients (3.1%), respectively. The cumulative probability of development of cirrhosis at 3 years and at 5 years was 9.6% and 16.7%, respectively. That of HCC at 3 and 5 years was 1.6% and 4.5%, respectively. The 3-year and 5-year overall survival rate was 99.7% and 96.0%, respectively. Pegylated interferon-based antiviral therapy was undertaken in 237 patients (62.0%) with a sustained virologic response (SVR) rate of 74.3%. The factors related to the overall clinical outcomes were age ≥55 years (HR 2.924, P = 0.016), platelet counts <150  × 109/L (HR 3.195, P = 0.007), and the achievement of SVR (HR 0.254, P = 0.002). The clinical outcomes of this Korean chronic hepatitis C cohort were modest with minimal mortality, but significant disease progression occurred in the patients with old age, low platelet, and non-SVR after interferon-based antiviral treatment or no treatment, suggesting priority for direct acting antiviral therapy. PMID:27583874

  4. Clinical Features and Treatment of Ocular Toxoplasmosis

    PubMed Central

    Park, Young-Hoon

    2013-01-01

    Ocular toxoplasmosis is a disease caused by the infection with Toxoplasma gondii through congenital or acquired routes. Once the parasite reaches the retina, it proliferates within host cells followed by rupture of the host cells and invasion into neighboring cells to make primary lesions. Sometimes the restricted parasite by the host immunity in the first scar is activated to infect another lesion nearby the scar. Blurred vision is the main complaint of ocular toxoplasmic patients and can be diagnosed by detection of antibodies or parasite DNA. Ocular toxoplasmosis needs therapy with several combinations of drugs to eliminate the parasite and accompanying inflammation; if not treated it sometimes leads to loss of vision. We describe here clinical features and currently available chemotherapy of ocular toxoplasmosis. PMID:24039281

  5. Sporotrichosis arthritis: clinical features in seven patients.

    PubMed

    Crout, J E; Brewer, N S; Tompkins, R B

    1977-03-01

    A review of the clinical features of seven patients with sporotrichosis arthritis showed that six had joint infection without previous skin or lung involvement and that one with myelofibrosis had joint and skin infection. The average time from onset of joint symptoms to diagnosis was 25 months, resulting in joint damage that required arthrodesis in four patients. Tissue from open synovial biopsy was superior to synovial fluid for obtaining a positive culture; concomitant synovial fluid and synovial tissue cultures were superior to either one alone. Granulomatous inflammation was seen in synovial tissue in six patients biopsied. Amphotericin B with surgical debridement of the affected joint was successful treatment in four patients. Although an uncommon cause of joint disease, sporotrichosis arthritis may go unrecognized and mimic other forms of arthritis, resulting in irreparable damage in an otherwise curable form of arthritis.

  6. Unilateral cerebellar hypoplasia with different clinical features.

    PubMed

    Benbir, Gulcin; Kara, Simay; Yalcinkaya, Beyza Citci; Karhkaya, Geysu; Tuysuz, Beyhan; Kocer, Naci; Yalcinkaya, Cengiz

    2011-03-01

    Unilateral cerebellar hypoplasia (UCH) is a rare pathological condition characterized by the loss of volume in cerebellar hemispheres ranging from mild asymptomatic to severe symptomatic cases. As the designation of UCH remains problematic, the underlying etiopathogenesis also lacks explanation. We investigated the patients admitted to Departments of Child Neurology, Neurology, and Genetics between the years 1992 and 2010 and detected 12 patients with unilateral cerebellar volume loss, with the exclusion of all other cerebellar pathologies. The ages of patients ranged between 6 months to 55 years. Five patients had a delay in developmental milestones, and one of these was diagnosed with neurofibromatosis type 1. Two patients had epileptic seizures, one patient had peripheral facial paralysis as a component of Moebius syndrome, and four patients were incidentally diagnosed during etiological work-up for headache. The clinical outcomes of patients varied from healthy subjects to marked developmental impairment. Radiologically, five patients had severe disproportionate UCH, six had moderate disproportionate, and one had mild proportionate UCH. Cerebellar peduncles were affected in all, and vermis was partly hypoplastic in eight patients. Brainstem was involved in four patients, and seven patients showed involvement of white matter and/or corpus callosum. Imaging features supported that patients with severe disproportionate UCH also displayed additional cerebral and commissural changes, which were related to ischemic or vascular injuries, implying a prenatally acquired disruption. In the presence of such a wide spectrum of clinical and radiological features, a prenatally acquired lesion and, thus, a disruption seem to be more explanatory rather than a primary developmental process or malformation in the etiopathogenesis of unilateral cerebellar hypoplasia.

  7. Costeff syndrome: clinical features and natural history.

    PubMed

    Yahalom, Gilad; Anikster, Yair; Huna-Baron, Ruth; Hoffmann, Chen; Blumkin, Lubov; Lev, Dorit; Tsabari, Rakefet; Nitsan, Zeev; Lerman, Sheera F; Ben-Zeev, Bruria; Pode-Shakked, Ben; Sofer, Shira; Schweiger, Avraham; Lerman-Sagie, Tally; Hassin-Baer, Sharon

    2014-12-01

    Costeff syndrome (CS) is a rare autosomal-recessive neurological disorder, which is known almost exclusively in patients of Iraqi Jewish descent, manifesting in childhood with optic atrophy, ataxia, chorea and spastic paraparesis. Our aim was to study the clinical spectrum of CS and natural history using a cross-sectional study design. Consecutive patients with CS were recruited to the study. Patients were diagnosed based on clinical features, along with elevated urinary levels of methylglutaconic and methylglutaric acid, and by identification of the disease-causing mutation in the OPA3 gene in most. All patients were examined by a neurologist and signs and symptoms were rated. 28 patients with CS (16 males, 21 families, age at last observation 28.6 ± 16.1 years, range 0.5-68 years) were included. First signs of neurological deficit appeared in infancy or early childhood, with delayed motor milestones, choreiform movements, ataxia and visual disturbances. Ataxia and chorea were the dominant motor features in childhood, but varied in severity among patients and did not seem to worsen with age. Pyramidal dysfunction appeared later and progressed with age (r = 0.71, p < 0.001) leading to spastic paraparesis and marked gait impairment. The course of neurological deterioration was slow and the majority of patients could still walk beyond the fifth decade. While visual acuity seemed to deteriorate, it did not correlate with age. CS is a rare neurogenetic disorder that causes serious disability and worsens with age. Spasticity significantly increases over the years and is the most crucial determinant of neurological dysfunction.

  8. [Clinical features of strabismus in psychomotor retardation].

    PubMed

    Arias-Cabello, Belina; Arroyo-Yllanes, María Estela; Pérez-Pérez, José Fernando; Fonte-Vázquez, Anselmo

    2016-01-01

    In psychomotor retardation there is an abnormal development of mental, sensory and motor skills associated with ocular manifestations. There are biological and psychosocial risk factors that predispose an individual to neurological damage. From 50% to 80% of patients with strabismus retardation have special features that differentiate it from the rest of strabismus in healthy patients. To determine the most common type of strabismus in patients with psychomotor retardation and their clinical features. Patients with psychomotor retardation and strabismus were included. An ophthalmological examination was performed, as well as an evaluation of the characteristics of strabismus, including perinatal and post-natal history. Esotropia was the most frequent squint with 65.3%, followed by exotropia with 32.7%. The variability in the squint magnitude was 60% in both types, and 6 patients had dissociated vertical deviation. Most of the patients started to present strabismus since they were born. The most frequent perinatal risk factors were threatened miscarriage, pre-eclampsia, foetal distress, and hypoxia. Esotropia is the most common type of strabismus in psychomotor retardation. The variability of squint magnitude is a characteristic in these patients. The moderate variability is the most frequent in both esotropia and exotropia. The most common refractive error is hyperopic astigmatism in esotropia and the myopic kind in exotropia. Copyright © 2015 Academia Mexicana de Cirugía A.C. Published by Masson Doyma México S.A. All rights reserved.

  9. Discretization of continuous features in clinical datasets

    PubMed Central

    Maslove, David M; Podchiyska, Tanya; Lowe, Henry J

    2013-01-01

    Background The increasing availability of clinical data from electronic medical records (EMRs) has created opportunities for secondary uses of health information. When used in machine learning classification, many data features must first be transformed by discretization. Objective To evaluate six discretization strategies, both supervised and unsupervised, using EMR data. Materials and methods We classified laboratory data (arterial blood gas (ABG) measurements) and physiologic data (cardiac output (CO) measurements) derived from adult patients in the intensive care unit using decision trees and naïve Bayes classifiers. Continuous features were partitioned using two supervised, and four unsupervised discretization strategies. The resulting classification accuracy was compared with that obtained with the original, continuous data. Results Supervised methods were more accurate and consistent than unsupervised, but tended to produce larger decision trees. Among the unsupervised methods, equal frequency and k-means performed well overall, while equal width was significantly less accurate. Discussion This is, we believe, the first dedicated evaluation of discretization strategies using EMR data. It is unlikely that any one discretization method applies universally to EMR data. Performance was influenced by the choice of class labels and, in the case of unsupervised methods, the number of intervals. In selecting the number of intervals there is generally a trade-off between greater accuracy and greater consistency. Conclusions In general, supervised methods yield higher accuracy, but are constrained to a single specific application. Unsupervised methods do not require class labels and can produce discretized data that can be used for multiple purposes. PMID:23059731

  10. Gestational Trophoblastic Disease: Clinical and Imaging Features.

    PubMed

    Shaaban, Akram M; Rezvani, Maryam; Haroun, Reham R; Kennedy, Anne M; Elsayes, Khaled M; Olpin, Jeffrey D; Salama, Mohamed E; Foster, Bryan R; Menias, Christine O

    2017-01-01

    Gestational trophoblastic disease (GTD) is a spectrum of both benign and malignant gestational tumors, including hydatidiform mole (complete and partial), invasive mole, choriocarcinoma, placental site trophoblastic tumor, and epithelioid trophoblastic tumor. The latter four entities are referred to as gestational trophoblastic neoplasia (GTN). These conditions are aggressive with a propensity to widely metastasize. GTN can result in significant morbidity and mortality if left untreated. Early diagnosis of GTD is essential for prompt and successful management while preserving fertility. Initial diagnosis of GTD is based on a multifactorial approach consisting of clinical features, serial quantitative human chorionic gonadotropin (β-hCG) titers, and imaging findings. Ultrasonography (US) is the modality of choice for initial diagnosis of complete hydatidiform mole and can provide an invaluable means of local surveillance after treatment. The performance of US in diagnosing all molar pregnancies is surprisingly poor, predominantly due to the difficulty in differentiating partial hydatidiform mole from nonmolar abortion and retained products of conception. While GTN after a molar pregnancy is usually diagnosed with serial β-hCG titers, imaging plays an important role in evaluation of local extent of disease and systemic surveillance. Imaging also plays a crucial role in detection and management of complications, such as uterine and pulmonary arteriovenous fistulas. Familiarity with the pathogenesis, classification, imaging features, and treatment of these tumors can aid in radiologic diagnosis and guide appropriate management. (©)RSNA, 2017.

  11. Clinical features and molecular bases of neuroacanthocytosis.

    PubMed

    Rampoldi, Luca; Danek, Adrian; Monaco, Anthony P

    2002-08-01

    The term acanthocytosis is derived from the Greek for "thorn" and is used to describe a peculiar spiky appearance of erythrocytes. Acanthocytosis is found to be associated with at least three hereditary neurological disorders that are generally referred to as neuroacanthocytosis. Abetalipoproteinaemia is an autosomal recessive condition, characterised by absence of serum apolipoprotein B containing lipoproteins leading to fat intolerance and fat-soluble vitamin deficiency. This results in a progressive spinocerebellar ataxia with peripheral neuropathy and retinitis pigmentosa. Chorea-acanthocytosis is also an autosomal recessive condition and is characterised by chorea, orofaciolingual dyskinesia, dysphagia, dysarthria, areflexia, seizures and dementia. Some of its features, including choreic movements, peripheral neuropathy with areflexia, elevated serum creatine kinase levels and myopathy are shared by another form of neuroacanthocytosis, McLeod syndrome. Patients affected by this X-linked disorder also show abnormal expression of Kell blood group antigens and a permanent haemolytic state. In addition to these cases, acanthocytosis is occasionally associated with other neurological disorders, such as Hallervorden-Spatz disease. For each of the neuroacanthocytosis syndromes we review the main clinical features and their molecular bases. The recent molecular genetics findings are the first step towards the understanding of the pathogenetic mechanisms and eventually the search for effective treatments.

  12. Features and ethical considerations associated with living kidney and liver transplantations in South Korea.

    PubMed

    Kim, J H

    2014-12-01

    When the Organ Transplantation Act came into effect in 2000 in South Korea, living organ donations were legalized and the Korean Network for Organ Sharing (KONOS) had a duty to approve the application of donation. The number of living organ donors has increased and the waiting time of recipients has been steady or decreased. The Organ Transplantation Act mainly focuses on the informed consent process of donations, so unrelated directed donations are permitted unless there is a suspicion of organ trafficking. But the annual reports show that directed donations of unrelated and related donors may have an ethical concern about organ sales. The donations of family members show another ethical concern. The numbers of ABO-incompatible transplantations have steadily increased since 2008, and lineal descendants, including minors, comprised 61% of living liver donors in 2012. Addressing the unethical practices without inhibiting living organ donations is the current task in South Korea. Private agencies have actively operated the living organ donations programs. The web-based computerized organ exchange program has been cooperatively run by hospital-based organizations. The strict legal regulations that could decrease living organ donations are hard to adopt. In the current situation, the functions of the official system need to be more developed. A national organ exchange program run by KONOS could be an option which could reduce ABO-incompatible transplantations and relieve the ethical concern of organ sales in unrelated directed donations. Copyright © 2014 Elsevier Inc. All rights reserved.

  13. Clinical characteristics and outcome of multiple sclerosis in Korea: does multiple sclerosis in Korea really differ from that in the Caucasian populations?

    PubMed

    Kim, Su-Hyun; Huh, So-Young; Kim, Woojun; Park, Min Su; Ahn, Suk-Won; Cho, Joong-Yang; Kim, Byung Jo; Kim, Ho Jin

    2013-10-01

    Multiple sclerosis (MS) in Asia is thought to have different clinical characteristics from MS in Western countries; however, previous studies in Asia were performed without properly differentiating neuromyelitis optica (NMO) from MS. To evaluate the clinical characteristics of MS in Korea after careful exclusion of potential explanations other than MS, particularly NMO spectrum disorder (NMOSD). This study is a retrospective review of consecutive MS patients attending five referral hospitals in Korea. All patients' MS diagnoses were re-evaluated. Of the 105 patients, 70 were female and 35 were male. The mean age of onset was 30.4 years and the mean disease duration was 5.4 years. On initial magnetic resonance imaging (MRI), 58% and 64% fulfilled the criteria for dissemination in space for the 2005 and 2010 McDonald criteria, respectively. Spinal cord lesions were observed in 78% of patients, primarily present as multiple small lesions with a mean length of 0.9 vertebral segments. The median time from disease onset to an Expanded Disability Status Scale 6 was 20 years. After careful exclusion of NMOSD, we found that the clinical pattern of MS in Korea does not fundamentally differ from that seen in Western countries.

  14. Participation determinants in the DRG payment system of obstetrics and gynecology clinics in South Korea.

    PubMed

    Song, Jung-Kook; Kim, Chang-yup

    2010-03-01

    The Diagnosis Related Group (DRG) payment system, which has been implemented in Korea since 1997, is based on voluntary participation. Hence, the positive impact of this system depends on the participation of physicians. This study examined the factors determining participation of Korean obstetrics & gynecology (OBGYN) clinics in the DRG-based payment system. The demographic information, practice-related variables of OBGYN clinics and participation information in the DRG-based payment system were acquired from the nationwide data from 2002 to 2007 produced by the National Health Insurance Corporation and the Health Insurance Review & Assessment Service. The subjects were 336 OBGYN clinics consisting of 43 DRG clinics that had maintained their participation in 2003-2007 and 293 no-DRG (fee-for-service) clinics that had never been a DRG clinic during the same period. Logistic regression analysis was carried out to determine the factors associated with the participation of OBGYN clinics in the DRG-based payment system. The factors affecting participation of OBGYN clinics in the DRG-based payment system were as follows (p<0.05): (1) a larger number of caesarian section (c/sec) claims, (2) higher cost of a c/sec, (3) less variation in the price of a c/sec, (4) fewer days of admission for a c/sec, and (5) younger pregnant women undergoing a c/sec. These results suggest that OBGYN clinics with an economic practice pattern under a fee-for-service system are more likely to participate in the DRG-based payment system. Therefore, to ensure adequate participation of physicians, a payment system with a stronger financial incentive might be more suitable in Korea.

  15. Clinical pathologist in Korea--training program and its roles in laboratories.

    PubMed

    Cho, Han-Ik; Lee, Kap No; Park, Jong-Woo; Park, Hyosoon; Kwak, Yun Sik

    2002-01-01

    A rapid development of practice of laboratory medicine in Korea owes its success to the clinical pathologists (CP), who have played a role of a pathfinder for laboratories. The Korean CP postgraduate education (residency) program is unique in that it is exclusively for laboratory medicine. The training program for clinical pathologists includes diagnostic hematology, diagnostic immunology, clinical microbiology, clinical chemistry, blood bank, diagnostic genetics, informatics and laboratory management. The program has produced a strong group of about 600 laboratory physicians, officially clinical pathologists since 1963. Most of Korean clinical pathologists work as laboratory directors, directors of university hospital laboratories or teaching faculty members in medical schools. The roles of clinical pathologists are laboratory management, interpretation of laboratory test results, clinical consulting services to clinicians and patients, ordering secondary tests after reviews of requested test results and utilization management. The clinical pathologists have developed clinical laboratories to be a main contributor for improved medical practice. During the last 40 years under the turbulent healthcare system, clinical pathologists have significantly contributed to safeguard the laboratory interests. The education program and the role of clinical pathologists are described.

  16. Clinical features of pediatric hereditary angioedema.

    PubMed

    Nanda, Maya K; Elenburg, Shelby; Bernstein, Jonathan A; Assa'ad, Amal H

    2015-01-01

    There is a paucity of data that describe the clinical course of hereditary angioedema (HAE) in children. The purpose of this study was to examine the clinical features of children with HAE. Electronic medical records from the past 10 years at Cincinnati Children's Hospital Medical Center and an outpatient allergy community practice were searched for ICD-9 code 277.6 (Other deficiencies of circulating enzyme). Exclusion criteria included laboratory data not supportive of type I or II HAE diagnosis or age at diagnosis greater than 18 years. Chart review was performed and missing data were collected by telephone interviews with patient families. Descriptive statistics were performed using SAS version 9.4. Twenty-one children were identified. The median age was 13.2 years (interquartile range [IQR], 9.1-18.8), 71% were male, 86% had an HAE family history, and 95% were Caucasian. The median age of symptom onset and diagnosis was 5.7 (IQR, 5-9 years) and 5.0 (IQR, 4-8 years), respectively. Five children diagnosed were asymptomatic. Three children without a family history had a 6.0-year delay in diagnosis. The most common angioedema attack sites were abdominal, peripheral, and laryngeal, which occurred at least once in 93%, 73%, and 27%, respectively. Of the 15 children with onset of symptoms, only 6 children received on-demand therapy for an acute attack, whereas 13 children were administered either short-term or long-term prophylaxis therapy. In this pediatric HAE population, symptom onset and diagnosis occurred at a median age of 5 years with a delay in diagnosis in those without a family history. Abdominal attacks were more common than peripheral attacks in this population. Copyright © 2015 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  17. Current Clinical Status of Telehealth in Korea: Categories, Scientific Basis, and Obstacles

    PubMed Central

    Kim, Hun-Sung; Kim, Hyunah; Lee, Suehyun; Lee, Kye Hwa

    2015-01-01

    Objectives Through telehealth, medical services have expanded beyond spatial boundaries and are now available in living spaces outside of hospitals. It can also contribute to patient medical knowledge improvement because patients can access their hospital records and data from home. However, concepts of telehealth are rather vague in Korea. Methods We refer to several clinical reports to determine the current clinical status of and obstacles to telehealth in Korea. Results Patients' health conditions are now reported regularly to doctors remotely, and patients can receive varied assistance. Self-improvement based on minute details that are beyond medical staff's reach is another possible benefit that may be realized with the help of a variety of medical equipment (sensors). The feasibility, clinical effect, and cost-benefit of telehealth have been verified by scientific evidence. Conclusions Patients will be able to improve their treatment adherence by receiving help from various professionals, such as doctors, nurses, nutritionists, and sports therapists. This means that the actual treatment time per patient will increase as well. Ultimately, this will increase the quality of patients' self-administration of care to impede disease progression and prevent complications. PMID:26618030

  18. Clinical characteristics and outcomes of acute hepatitis a in Korea: a nationwide multicenter study.

    PubMed

    Kwon, So Young; Park, Sang Hoon; Yeon, Jong Eun; Jeong, Sook Hyang; Kwon, Oh Sang; Lee, Jin Woo; Kim, Hong Soo; Seo, Yeon Seok; Kim, Young Seok; Sohn, Joo Hyun; Yim, Hyung Joon; Choi, Jong Young; Lee, Myung Seok; Kweon, Young Oh; Cheong, Jae Youn; Kim, Haak Cheoul; Lee, Heon Ju; Baik, Soon Koo; An, Hyonggin; Byun, Kwan Soo

    2014-02-01

    The aim of this study was to investigate the clinical characteristics of acute hepatitis A during a recent outbreak in Korea. Data of patients diagnosed with acute hepatitis A from 2007 to 2009 were collected from 21 tertiary hospitals retrospectively. Their demographic, clinical, and serological characteristics and their clinical outcomes were analyzed. A total of 4,218 patients (mean age 33.3 yr) were included. The median duration of admission was 9 days. The mean of the highest ALT level was 2,963 IU/L, total bilirubin was 7.3 mg/dL, prothrombin time INR was 1.3. HBsAg was positive in 3.7%, and anti-HCV positive in 0.7%. Renal insufficiency occurred in 2.7%, hepatic failure in 0.9%, relapsing hepatitis in 0.7%, and cholestatic hepatitis in 1.9% of the patients. Nineteen patients (0.45%) died or were transplanted. Complications of renal failure or prolonged cholestasis were more frequent in patients older than 30 yr. In conclusion, most patients with acute hepatitis A recover uneventfully, however, complication rates are higher in patients older than 30 yr than younger patients. Preventive strategies including universal vaccination in infants and active immunization of hepatitis A to adult population should be considered for prevention of community-wide outbreaks of hepatitis A in Korea.

  19. Differences in clinical features according to Boryoung and Karp genotypes of Orientia tsutsugamushi.

    PubMed

    Kim, Dong-Min; Yun, Na Ra; Neupane, Ganesh Prasad; Shin, Sung Heui; Ryu, So Yeon; Yoon, Hee Jung; Wie, Seong Heon; Kim, Woo Jin; Lee, Chang Youl; Choi, Jong Soo; Yang, Tae Young

    2011-01-01

    Scrub typhus is an infectious disease caused by Orientia tsutsugamushi. The differences in virulence of O. tsutsugamushi prototypes in humans are still unknown. We investigated whether there are any differences in the clinical features of the Boryoung and Karp genotypes. Patients infected with O. tsutsugamushi, as Boryoung and Karp clusters, who had visited 6 different hospitals in southwestern Korea were prospectively compared for clinical features, complications, laboratory parameters, and treatment responses. Infected patients in the Boryoung cluster had significantly more generalized weakness, eschars, skin rashes, conjunctival injection, high albumin levels, and greater ESR and fibrinogen levels compared to the Karp cluster. The treatment response to current antibiotics was significantly slower in the Karp cluster as compared to the Boryoung cluster. The frequency of occurrence of eschars and rashes may depend on the genotype of O. tsutsugamushi.

  20. [Clinical features of 233 cases of keratoconus].

    PubMed

    Li, Shao-wei; Li, Zhao-xia; Shi, Wei-yun; Zeng, Qing-yan; Jin, Xiu-ming

    2005-07-01

    To analyze clinical features of keratoconus. The case history of keratoconus patients who underwent penetrating keratoplasty, epikeratophakia or deep lamellar keratoplasty in our hospital during January 1993 to March 2002 was reviewed, and inquiry form was sent to those who without complete data. Patients were followed during January 2000 to March 2002. Two hundred and thirty three cases of keratoconus were enrolled in this study with 216 males and 17 females. Patient's family history, systemic complications, refractive status, progress of disease, eye axial length and anterior chamber depth were recorded. The clinical natural history of these patients followed the model of "myopia-astigmatism-poor spectacle correction-acute corneal hydrops or stromal scar". The presenting age of myopia ranged from 6 to 29 years, with a mean age of 13.8 years, 10.1% (22/219) of the patients were younger than 10 years, 67.1% (147/219) were between 11 and 15 years. The interval time between emerging myopia and poor spectacle correction (best corrected visual acuity less than 20/200) was ranged from 1 to 20 years, of which 85.2% (161/189) were shorter than 6 years. Twenty percent (47/233) of the patients had acute corneal hydrops in one eye at the age of 12 to 31 years, and 4.3% of them (2/47) resulted in corneal perforation. Thirty-four patients had corneal stromal scars 1 to 13 years after keratoconus developed, and 94.1% of them (32/34) were within 8 years. Topography examination demonstrated a tendency of the cone base position progress: first, paracentral-enlargement following dominant astigmatism axis, then, enlargement along smaller astigmatism axis, and finally, the cone base moving centrally. The eye axial length averaged at (25.9 +/- 1.4) mm, the depth of anterior chamber (AC) were (4.3 +/- 0.3) mm, 89.7% (279/311) eyes had an axial length longer than 24 mm. All cases had an asymmetry clinical progress course. Two cases had Down syndrome, 2 with intelligence defect. No

  1. Anaphylaxis in children: clinical and allergologic features.

    PubMed

    Novembre, E; Cianferoni, A; Bernardini, R; Mugnaini, L; Caffarelli, C; Cavagni, G; Giovane, A; Vierucci, A

    1998-04-01

    Despite the importance of anaphylaxis, little information is available on its clinical features. To evaluate the clinical and allergologic features of anaphylaxis in children referred to the allergology and immunology unit of A. Meyer Children's Hospital (Florence, Italy) from 1994 to 1996. Ninety-five episodes of anaphylaxis occurred in 76 children (50 boys and 26 girls). Sixty-six children (87%) had only one episode of anaphylaxis, while 10 (13%) had two or more episodes. Sixty-two (82%) of the 76 patients had a personal history of atopic symptoms, although 14 (18%) did not. Sixty (79%) of the 76 children studied had at least one positive skin prick test to one or more of the common inhalant and/or food allergens. Children with venom-induced anaphylaxis usually had negative skin tests to the allergens tested. A younger age and eczema were more frequent among children with food-dependent anaphylaxis, whereas an older age together with urticaria-angioedema were common among those with exercise-induced anaphylaxis. The mean latent period (+/-SD) of the anaphylaxis episodes was 15.4 +/- 27.5 minutes. Skin and respiratory manifestations had an earlier onset and were more common than the gastrointestinal and cardiovascular ones. The most frequent clinical manifestation in children with food anaphylaxis was gastrointestinal symptoms, whereas cardiovascular symptoms were rare. The most probable causative agents in the 95 episodes described were foods (57%), drugs (11%), hymenoptera venom (12%), exercise (9%), additives (1%), specific immunotherapy (1%), latex (1%), and vaccines (2%), but in 6 cases (6%) the agent was never determined. Among the foods, seafood and milk were the most frequently involved. As for location, 57% of the anaphylactic events occurred in the home (54/95), 12% outdoors (11/95); 5% in restaurants (5/95); 3% in the doctor's office (3/95); 3% in hospitals (3/95); 3% on football fields (3/95); 2% on the beach (2/95); 1% in the gym (1/95); 1% at school

  2. Social and Clinical Characteristics of Immigrants with Tuberculosis in South Korea.

    PubMed

    Min, Gee Ho; Kim, Young; Lee, Jong Seok; Oh, Jee Youn; Hur, Gyu Young; Lee, Young Seok; Min, Kyung Hoon; Lee, Sung Yong; Kim, Je Hyeong; Shin, Chol; Lee, Seung Heon

    2017-05-01

    To determine the social and clinical characteristics of immigrants with tuberculosis (TB) in South Korea. The registered adult TB patients who were diagnosed and treated in Korea Medical Centers from January 2013 to December 2015 were analyzed retrospectively. A total of 105 immigrants with TB were compared to 932 native Korean TB patients. Among these 105 immigrants with TB, 86 (82%) were Korean-Chinese. The rate of drug-susceptible TB were lower in the immigrants group than in the native Korean group [odds ratio (OR): 0.46; 95% confidence interval (CI): 0.22-0.96, p=0.035]. Cure rate was higher in the immigrant group than in the native Korean group (OR: 2.03; 95% CI: 1.26-3.28, p=0.003). Treatment completion rate was lower in the immigrant group than in the native Korean group (OR: 0.50; 95% CI: 0.33-0.74, p=0.001). However, treatment success rate showed no significant difference between two groups (p=0.141). Lost to follow up (default) rate was higher in the immigrant group than in the native Korean group after adjusting for age and drug resistance (OR: 3.61; 95% CI: 1.36-9.61, p=0.010). There was no difference between defaulter and non-defaulter in clinical characteristics or types of visa among these immigrants (null p value). However, 43 TB patients with recent immigration were diagnosed as TB even though they had been screened as normal at the time of immigration. Endeavor to reduce the default rate of immigrants with TB and reinforce TB screening during the immigration process must be performed for TB infection control in South Korea.

  3. Clinical trials for vaccine development in registry of Korea Food and Drug Administration

    PubMed Central

    2013-01-01

    Based on the action plan "Ensuring a stable supply of National Immunization Program vaccines and sovereignty of biopharmaceutical products," Korea Food and Drug Administration (KFDA) has made efforts to develop vaccines in the context of self reliance and to protect public health. Along with the recognized infrastructures for clinical trials, clinical trials for vaccines have also gradually been conducted at multinational sites as well as at local sites. KFDA will support to expand six to eleven kinds of vaccines by 2017. In accordance with integrated regulatory system, KFDA has promoted clinical trials, established national lot release procedure, and strengthened good manufacturing practices inspection and post marketing surveillance. Against this backdrop, KFDA will support the vaccine development and promote excellent public health protection. PMID:23596594

  4. Yoga for Health Care in Korea: A Protocol for Systematic Review of Clinical Trials.

    PubMed

    Choi, Jiae; Jun, Ji Hee; Lee, Ju Ah; Lee, Myeong Soo

    2016-08-01

    This systematic review aims to evaluate the therapeutic effects of yoga therapy using an evidence-based approach and investigates the relationship between yoga and the meridian energies based on all available clinical studies in Korea. Sixteen electronic databases will be searched from the inception of the study until January 2016. All clinical evidences that evaluate any type of yoga and any type of control in individuals with any type of condition will be eligible. The methodological quality will be assessed using the Cochrane risk of bias tool for randomized clinical trials and the Newcastle-Ottawa scale for nonrandomized studies. Two authors will independently assess each study for eligibility and the risk of bias, and then they will extract the data. With its extensive, unbiased search of the Korean literature from various databases without any language restrictions, this systematic review will be useful for both practitioners in the field of yoga research as well as for patients.

  5. Clinical trials for vaccine development in registry of Korea Food and Drug Administration.

    PubMed

    Kang, Seog-Youn

    2013-01-01

    Based on the action plan "Ensuring a stable supply of National Immunization Program vaccines and sovereignty of biopharmaceutical products," Korea Food and Drug Administration (KFDA) has made efforts to develop vaccines in the context of self reliance and to protect public health. Along with the recognized infrastructures for clinical trials, clinical trials for vaccines have also gradually been conducted at multinational sites as well as at local sites. KFDA will support to expand six to eleven kinds of vaccines by 2017. In accordance with integrated regulatory system, KFDA has promoted clinical trials, established national lot release procedure, and strengthened good manufacturing practices inspection and post marketing surveillance. Against this backdrop, KFDA will support the vaccine development and promote excellent public health protection.

  6. Clinical Features and Treatments of Odontogenic Sinusitis

    PubMed Central

    Lee, Sung Jin

    2010-01-01

    Purpose The aim of this study was to investigate how clinical features such as sex, age, etiologic factors, and presenting symptoms of odontogenic sinusitis are differentiated from other types of sinusitis. Also, this study was designed to find methods for reducing the incidence of odontogenic sinusitis. Materials and Methods A retrospective chart analysis was completed on twenty-seven patients with odontogenic sinusitis. They were all treated at Kangbuk Samsung Hospital between February 2006 and August 2008. The study protocol and informed consent forms were approved by the institutional review boards for human beings at Kangbuk Samsung Hospital. Results Ten patients (37.0%) had dental implant related complications and 8 (29.6%) had dental extraction related complications. Unilateral purulent nasal discharge was the most common symptom (66.7%). The therapeutic modality included transnasal endoscopic sinus surgery in 19 (70.4%) patients, and a Caldwell-Luc operation in two (7.4%) patients. Conclusion In our study, there was no significant difference in the incidence between genders. The average age of the patients was 42.9 years. The incidence was highest in the fourth decade. There were no significant differences between the symptoms of odontogenic sinusitis and that of other types of sinusitis. However, almost all of the patients with odontogenic sinusitis had unilateral symptoms. Iatrogenic causes, which include dental implants and dental extractions, were the most common etiologic factors related to the development of odontogenic sinusitis. Therefore, a preoperative consultation between a rhinologist and a dentist prior to the dental procedure should be able to reduce the incidence of odontogenic sinusitis. PMID:20879062

  7. Vertebral osteomyelitis: clinical features and diagnosis.

    PubMed

    Eren Gök, S; Kaptanoğlu, E; Celikbaş, A; Ergönül, O; Baykam, N; Eroğlu, M; Dokuzoğuz, B

    2014-10-01

    We aimed to describe clinical and diagnostic features of vertebral osteomyelitis for differential diagnosis and treatment. This is a prospective observational study performed between 2002 and 2012 in Ankara Numune Education and Research Hospital in Ankara, Turkey. All the patients with vertebral osteomyelitis were followed for from 6 months to 3 years. In total, 214 patients were included in the study, 113 out of 214 (53%) were female. Out of 214 patients, 96 (45%) had brucellar vertebral osteomyelitis (BVO), 63 (29%) had tuberculous vertebral osteomyelitis (TVO), and 55 (26%) had pyogenic vertebral osteomyelitis (PVO). Mean number of days between onset of symptoms and establishment of diagnosis was greater with the patients with TVO (266 days) than BVO (115 days) or PVO (151 days, p <0.001). In blood cultures, Brucella spp. were isolated from 35 of 96 BVO patients (35%). Among 55 PVO patients, the aetiological agent was isolated in 11 (20%) patients. For tuberculin skin test >15 mm, sensitivity was 0.66, specificity was 0.97, positive predictive value was 0.89, negative predictive value was 0.88, and receiver operating characteristics area was 0.8. Tuberculous and brucellar vertebral osteomyelitis remained the leading causes of vertebral osteomyelitis with delayed diagnosis. In differential diagnosis of vertebral osteomyelitis, consumption of unpasteurized cheese, dealing with husbandry, sweating, arthralgia, hepatomegaly, elevated alanine transaminase, and lumbar involvement in magnetic resonance imaging were found to be predictors of BVO, thoracic involvement in magnetic resonance imaging and tuberculin skin test > 15 mm were found to be predictors of TVO, and history of spinal surgery and leucocytosis were found to be predictors of PVO.

  8. Clinical features and treatments of odontogenic sinusitis.

    PubMed

    Lee, Kyung Chul; Lee, Sung Jin

    2010-11-01

    The aim of this study was to investigate how clinical features such as sex, age, etiologic factors, and presenting symptoms of odontogenic sinusitis are differentiated from other types of sinusitis. Also, this study was designed to find methods for reducing the incidence of odontogenic sinusitis. A retrospective chart analysis was completed on twenty-seven patients with odontogenic sinusitis. They were all treated at Kangbuk Samsung Hospital between February 2006 and August 2008. The study protocol and informed consent forms were approved by the institutional review boards for human beings at Kangbuk Samsung Hospital. Ten patients (37.0%) had dental implant related complications and 8 (29.6%) had dental extraction related complications. Unilateral purulent nasal discharge was the most common symptom (66.7%). The therapeutic modality included transnasal endoscopic sinus surgery in 19 (70.4%) patients, and a Caldwell-Luc operation in two (7.4%) patients. In our study, there was no significant difference in the incidence between genders. The average age of the patients was 42.9 years. The incidence was highest in the fourth decade. There were no significant differences between the symptoms of odontogenic sinusitis and that of other types of sinusitis. However, almost all of the patients with odontogenic sinusitis had unilateral symptoms. Iatrogenic causes, which include dental implants and dental extractions, were the most common etiologic factors related to the development of odontogenic sinusitis. Therefore, a preoperative consultation between a rhinologist and a dentist prior to the dental procedure should be able to reduce the incidence of odontogenic sinusitis.

  9. Epidemiology and Clinical Characteristics of Zika Virus Infections Imported into Korea from March to October 2016.

    PubMed

    Yoon, Doran; Shin, Seung Hwan; Jang, Hee Chang; Kim, Eu Suk; Song, Eun Hee; Moon, Song Mi; Shin, So Youn; Choe, Pyeong Gyun; Sung, Jung Joon; Choi, Eun Hwa; Oh, Myoung Don; Jee, Youngmee; Kim, Nam Joong

    2017-09-01

    Zika is a re-emerging, mosquito-borne viral infection, which has been recently shown to cause microcephaly and Guillain-Barré syndrome. Since 2015 the number of infected patients has increased significantly in South America. The purpose of this study was to identify the epidemiologic and clinical characteristics of patients with Zika virus (ZIKV) infections in Korea. Patients who had visited areas of risk and tested positive in the ZIKV reverse transcriptase polymerase chain reaction (RT-PCR) in blood, urine, or saliva specimens were included. The first Korean case of ZIKV infection was reported in March 2016, and 14 cases had been reported by October 2016. The median age of the patients was 34 years (19-64 years). Ten patients had been exposed in Southeast Asia and 4 in Latin America. Rash was the most common symptom (92.9%; 13/14), followed by myalgia (50.0%; 7/14), and arthralgia (28.6%, 4/14). There were no neurologic abnormalities and none of the patients was pregnant. Results of biochemical tests were normal. Positivity rates of RT-PCR for ZIKV in serum, urine, and saliva were 53.8%, 100.0%, and 83.3%, respectively in the first week of symptoms. In conclusion, 14 patients with ZIKV infections were reported in Korea by October 2016 and all of them had mild clinical symptoms. © 2017 The Korean Academy of Medical Sciences.

  10. Dynamic genetic features of eukaryotic plankton diversity in the Nakdong River estuary of Korea

    NASA Astrophysics Data System (ADS)

    Lee, Jee Eun; Chung, Ik Kyo; Lee, Sang-Rae

    2016-08-01

    Estuaries are environments where freshwater and seawater mix and they display various salinity profiles. The construction of river barrages and dams has rapidly changed these environments and has had a wide range of impacts on plankton communities. To understand the dynamics of such communities, researchers need accurate and rapid techniques for detecting plankton species. We evaluated the diversity of eukaryotic plankton over a salinity gradient by applying a metagenomics tool at the Nakdong River estuary in Korea. Environmental samples were collected on three dates during summer and autumn of 2011 at the Eulsukdo Bridge at the mouth of that river. Amplifying the 18S rDNA allowed us to analyze 456 clones and 122 phylotypes. Metagenomic sequences revealed various taxonomic groups and cryptic genetic variations at the intra- and inter-specific levels. By analyzing the same station at each sampling date, we observed that the phylotypes presented a salinity-related pattern of diversity in assemblages. The variety of species within freshwater samples reflected the rapid environmental changes caused by freshwater inputs. Dinophyceae phylotypes accounted for the highest proportion of overall diversity in the seawater samples. Euryhaline diatoms and dinoflagellates were observed in the freshwater, brackish and seawater samples. The biological data for species composition demonstrate the transitional state between freshwater and seawater. Therefore, this metagenomics information can serve as a biological indicator for tracking changes in aquatic environments.

  11. Dynamic genetic features of eukaryotic plankton diversity in the Nakdong River estuary of Korea

    NASA Astrophysics Data System (ADS)

    Lee, Jee Eun; Chung, Ik Kyo; Lee, Sang-Rae

    2017-07-01

    Estuaries are environments where freshwater and seawater mix and they display various salinity profiles. The construction of river barrages and dams has rapidly changed these environments and has had a wide range of impacts on plankton communities. To understand the dynamics of such communities, researchers need accurate and rapid techniques for detecting plankton species. We evaluated the diversity of eukaryotic plankton over a salinity gradient by applying a metagenomics tool at the Nakdong River estuary in Korea. Environmental samples were collected on three dates during summer and autumn of 2011 at the Eulsukdo Bridge at the mouth of that river. Amplifying the 18S rDNA allowed us to analyze 456 clones and 122 phylotypes. Metagenomic sequences revealed various taxonomic groups and cryptic genetic variations at the intra- and inter-specific levels. By analyzing the same station at each sampling date, we observed that the phylotypes presented a salinity-related pattern of diversity in assemblages. The variety of species within freshwater samples reflected the rapid environmental changes caused by freshwater inputs. Dinophyceae phylotypes accounted for the highest proportion of overall diversity in the seawater samples. Euryhaline diatoms and dinoflagellates were observed in the freshwater, brackish and seawater samples. The biological data for species composition demonstrate the transitional state between freshwater and seawater. Therefore, this metagenomics information can serve as a biological indicator for tracking changes in aquatic environments.

  12. Accumulation features of arsenic species in various fishes collected from coastal cities in Korea

    NASA Astrophysics Data System (ADS)

    Choi, Sung-Deuk; Son, Hee-Sik; Choi, Minkyu; Park, Min-Kyu

    2015-12-01

    In this study, 36 fish species were collected from three coastal cities in Korea to investigate levels and patterns of six arsenicals (arsenite: As (III), arsenate: As (V), arsenocholine: AsC, arsenobetaine: AsB, monomethylarsonic acid: MMA, and dimethylarsinic acid: DMA). The levels of ∑6 As in the different fish species varied substantially, ranging from 0.02 μg As/g ww (Islaeli carp) to 9.65 μg As/g ww (Skate ray) with a median of 0.40 μg As/g ww. All the arsenicals in marine fishes showed higher levels than those in freshwater fishes due to fish feed living in saline water. Overall, marine carnivorous fishes seem to be more contaminated with arsenic. For all the fish samples, AsB (mean fraction: 90.6%) was dominant among the six arsenicals, indicating biomethylation of inorganic arsenic and accumulation of AsB. Fish species with high water contents showed elevated levels of As (III), but there was no further significant correlations between arsenicals and water/lipid contents. Concentrations of As (V) were significantly lower than those of As (III), which implies that As (V) is reduced during biomethylation of inorganic arsenic. Consequently, we hypothesize that the toxicity of arsenic (mainly derived from As (III)) can be increased by the reduction of As (V), especially for the fish species with higher water contents.

  13. Clinical Validation of Anyplex II HPV HR Detection Test for Cervical Cancer Screening in Korea.

    PubMed

    Jung, Sunkyung; Lee, Byungdoo; Lee, Kap No; Kim, Yonggoo; Oh, Eun-Jee

    2016-03-01

    The Anyplex II HPV HR detection kit (Seegene Inc, Seoul, Korea) is a new, multiplex, real-time polymerase chain reaction assay to detect individual 14 high-risk (HR) human papillomavirus (HPV) types in a single tube. To evaluate the clinical performance of the HPV HR kit in predicting high-grade squamous intraepithelial lesions and cervical intraepithelial lesions grade 2 or worse in cervical cancer screening. We analyzed 1137 cervical samples in Huro Path medium (CelltraZone, Seoul, Korea) from Korean women. The clinical performance of the HPV HR kit was compared with Hybrid Capture 2 (Qiagen, Valencia, California) using the noninferiority score test in a routine cervical cancer screening setting. The intralaboratory and interlaboratory agreements of HPV HR were also evaluated. Overall agreement between the 2 assays was 92.4% (1051 of 1137) with a κ value of 0.787. Clinical sensitivity of HPV HR for high-grade squamous intraepithelial lesions and cervical intraepithelial lesions grade 2 or worse was 94.4% (95% confidence interval [CI], 89.2-99.7) and 92.5% (95% CI, 84.3-100.0), respectively. The respective values for Hybrid Capture 2 were 93.1% (95% CI, 87.2-98.9) and 87.5% (95% CI, 77.3-99.7). Clinical sensitivity and specificity of HPV HR were not inferior to those of Hybrid Capture 2 (P = .005 and P = .04, respectively). The HPV HR showed good intralaboratory and interlaboratory reproducibility at 98.0% (κ = 0.953) and 97.4% (κ = 0.940), respectively. The HPV HR demonstrates comparable performance to the Hybrid Capture 2 test and can be useful for HPV-based cervical cancer screening testing.

  14. Localized scleroderma: a clinical study at a single center in Korea.

    PubMed

    Noh, Jung Won; Kim, Jinseok; Kim, Jae-Wang

    2013-08-01

    Localized scleroderma (morphea) is a rare autoimmune disease limited to the skin, characterized by cutaneous fibrosing and obstructive vasculopathy. Localized scleroderma may invade into the subcutaneous fat layer and cause permanent functional disability. Because of its rarity, there have been few clinical surveys of patients with localized scleroderma in Korea. The aim of this study was to elucidate the clinical presentation, serological data, and clinical outcomes of localized scleroderma. This was a retrospective survey conducted by reviewing available medical records during a 7 year-period from 2004 to 2010 in a single medical center in Jeju Island, South Korea. In total 43 patients with localized scleroderma were included. Localized scleroderma occurred primarily in females (female to male ratio 2.6 : 1.0). Most patients were between 10 and 29 years of age and the mean age at diagnosis was 26.2 years. Plaque (51.2%) and linear morphea (37.2%) were most common. No case was associated with systemic scleroderma (systemic sclerosis). The most common site of plaque morphea was the trunk (47.8%). In the linear type, the most common site was head-neck (52.9%). Fluorescent antinuclear antibody was positive in 23.3% of all cases. Treatment included systemic corticosteroids, colchicine, anti-malarial agents, D-penicillamine or intralesional triamcinolone injection. Clinical improvement, including significant and partial response, was seen in only 62.8% of treated patients. Localized scleroderma is a chronic inflammatory condition confined to the skin. In order to exclude other conditions, thorough history taking, physical examination, serologic studies and histopathologic examinations should be conducted. © 2013 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  15. Clinical and Microbiologic Characteristics of Clostridium difficile Infection Caused by Binary Toxin Producing Strain in Korea

    PubMed Central

    Kim, Jieun; Seo, Mi-ran; Kang, Jung Oak; Choi, Tae Yeal

    2013-01-01

    Background Binary toxin-producing Clostridium difficile infections (CDI) are known to be more severe and to cause higher case fatality rates than those by binary toxin-negative isolates. There has been few data of binary toxin-producing CDI in Korea. Objective of the study is to characterize clinical and microbiological trait of CDI cause by binary-toxin producing isolates in Korea. Materials and Methods From September 2008 through January 2010, clinical characteristics, medication history and treatment outcome of all the CDI patients were collected prospectively. Toxin characterization, PCR ribotyping and antibiotic susceptibility were performed with the stool isolates of C. difficile. Results During the period, CDI caused by 11binary toxin-producing isolates and 105 toxin A & toxin B-positive binary toxin-negative isolates were identified. Comparing the disease severity and clinical findings between two groups, leukocytosis and mucoid stool were more frequently observed in patients with binary toxin-positive isolates (OR: 5.2, 95% CI: 1.1 to 25.4, P = 0.043; OR: 7.6, 95% CI: 1.6 to 35.6, P = 0.010, respectively), but clinical outcome of 2 groups did not show any difference. For the risk factors for acquisition of binary toxin-positive isolates, previous use of glycopeptides was the significant risk factor (OR: 6.2, 95% CI: 1.4 to 28.6, P = 0.019), but use of probiotics worked as an inhibitory factor (OR: 0.1, 95% CI: 0.0 to 0.8; P = 0.026). PCR ribotypes of binary toxinproducing C. difficile showed variable patterns: ribotype 130, 4 isolates; 027, 3 isolates; 267 and 122, 1 each isolate and unidentified C1, 2 isolates. All 11 binary toxin-positive isolates were highly susceptible to clindamycin, moxifloxacin, metronidazole, vancomycin and piperacillin-tazobactam, however, 1 of 11 of the isolates was resistant to rifaximin. Conclusions Binary toxin-producing C. difficile infection was not common in Korea and those isolates showed diverse PCR ribotypes with high

  16. Clinical and Microbiologic Characteristics of Clostridium difficile Infection Caused by Binary Toxin Producing Strain in Korea.

    PubMed

    Kim, Jieun; Seo, Mi-Ran; Kang, Jung Oak; Choi, Tae Yeal; Pai, Hyunjoo

    2013-06-01

    Binary toxin-producing Clostridium difficile infections (CDI) are known to be more severe and to cause higher case fatality rates than those by binary toxin-negative isolates. There has been few data of binary toxin-producing CDI in Korea. Objective of the study is to characterize clinical and microbiological trait of CDI cause by binary-toxin producing isolates in Korea. From September 2008 through January 2010, clinical characteristics, medication history and treatment outcome of all the CDI patients were collected prospectively. Toxin characterization, PCR ribotyping and antibiotic susceptibility were performed with the stool isolates of C. difficile. During the period, CDI caused by 11binary toxin-producing isolates and 105 toxin A & toxin B-positive binary toxin-negative isolates were identified. Comparing the disease severity and clinical findings between two groups, leukocytosis and mucoid stool were more frequently observed in patients with binary toxin-positive isolates (OR: 5.2, 95% CI: 1.1 to 25.4, P = 0.043; OR: 7.6, 95% CI: 1.6 to 35.6, P = 0.010, respectively), but clinical outcome of 2 groups did not show any difference. For the risk factors for acquisition of binary toxin-positive isolates, previous use of glycopeptides was the significant risk factor (OR: 6.2, 95% CI: 1.4 to 28.6, P = 0.019), but use of probiotics worked as an inhibitory factor (OR: 0.1, 95% CI: 0.0 to 0.8; P = 0.026). PCR ribotypes of binary toxinproducing C. difficile showed variable patterns: ribotype 130, 4 isolates; 027, 3 isolates; 267 and 122, 1 each isolate and unidentified C1, 2 isolates. All 11 binary toxin-positive isolates were highly susceptible to clindamycin, moxifloxacin, metronidazole, vancomycin and piperacillin-tazobactam, however, 1 of 11 of the isolates was resistant to rifaximin. Binary toxin-producing C. difficile infection was not common in Korea and those isolates showed diverse PCR ribotypes with high susceptibility to antimicrobial agents. Glycopeptide

  17. Prevalence of Trichomonas vaginalis in Women Visiting 2 Obstetrics and Gynecology Clinics in Daegu, South Korea.

    PubMed

    Goo, Youn-Kyoung; Shin, Won-Sik; Yang, Hye-Won; Joo, So-Young; Song, Su-Min; Ryu, Jae-Sook; Lee, Won-Myung; Kong, Hyun-Hee; Lee, Won-Ki; Lee, Sang-Eun; Lee, Won-Ja; Chung, Dong-Il; Hong, Yeonchul

    2016-02-01

    This study explored epidemiological trends in trichomoniasis in Daegu, South Korea. Wet mount microscopy, PCR, and multiplex PCR were used to test for Trichomonas vaginalis in vaginal swab samples obtained from 621 women visiting 2 clinics in Daegu. Of the 621 women tested, microscopy detected T. vaginalis in 4 (0.6%) patients, PCR detected T. vaginalis in 19 (3.0%) patients, and multiplex PCR detected T. vaginalis in 12 (1.9%) patients. Testing via PCR demonstrated high sensitivity and high negative predictive value for T. vaginalis. Among the 19 women who tested positive for T. vaginalis according to PCR, 94.7% (18/19) reported vaginal signs and symptoms. Notably, more than 50% of T. vaginalis infections occurred in females younger than 30 years old, and 58% were unmarried. Multiplex PCR, which simultaneously detects pathogens from various sexually transmitted infections, revealed that 91.7% (11/12) of patients were infected with 2 or more pathogens. Mycoplasma hominis was the most prevalent co-infection pathogen with T. vaginalis, followed by Ureaplasma urealyticum and Chlamydia trachomatis. Our results indicate that PCR and multiplex PCR are the most sensitive tools for T. vaginalis diagnosis, rather than microscopy which has been routinely used to detect T. vaginalis infections in South Korea. Therefore, clinicians should take note of the high prevalence of T. vaginalis infections among adolescent and young women in order to prevent persistent infection and transmission of this disease.

  18. Clinical characteristics of pediatric thalassemia in Korea: a single institute experience.

    PubMed

    Hong, Che Ry; Kang, Hyoung Jin; Lee, Ji Won; Kim, Hyery; Kim, Nam Hee; Park, Kyung Duk; Park, June Dong; Seong, Moon-Woo; Park, Sung Sup; Shin, Hee Young; Ahn, Hyo Seop

    2013-11-01

    Few literatures have elaborated on the clinical characteristics of children with thalassemia from low-prevalence areas. A retrospective analysis was conducted on children genetically confirmed with thalassemia at Seoul National University Children's Hospital in Korea. Nine children (1α thalassemia trait, 6β thalassemia minor, 2β thalassemia intermedia) were diagnosed with thalassemia at median age of 4.3 yr old with median hemoglobin of 9.7 g/dL. Seven (78%) children were incidentally found to be anemic and only 2 with β thalassemia intermedia had presenting symptoms. Five children (56%) were initially misdiagnosed with iron deficiency anemia. Despite the comorbidities due to α thalassemia mental retardation syndrome, the child with α thalassemia trait had mild hematologic profile. Children with β thalassemia intermedia had the worst phenotypes due to dominantly inherited mutations. None of the children was transfusion dependent and most of them had no complications associated with thalassemia. Only 1 child (11%) with codon 60 (T→A) mutation of the HBB gene needed red blood cell transfusions. He also had splenomegaly, cholelithiasis, and calvarial vault thickening. Pediatricians in Korea must acknowledge thalassemia as a possible diagnosis in children with microcytic hypochromic hemolytic anemia. High level of suspicion will allow timely diagnosis and managements.

  19. In vitro activity of gemifloxacin against recent clinical isolates of bacteria in Korea.

    PubMed

    Yong, Dong Eun; Cheong, Hee-Jin; Kim, Yang Soo; Park, Yeon Joon; Kim, Woo-Joo; Woo, Jun Hee; Lee, Kyung Won; Kang, Moon Won; Choo, Youn-Sung

    2002-12-01

    Gemifloxacin is an enhanced-affinity fluoroquinolone with broad-spectrum antibacterial activity. In Korea, resistant bacteria are relatively more prevalent than in other industrialized countries. In this study, we studied the in vitro activities of gemifloxacin, gatifloxacin, moxifloxacin, levofloxacin, ciprofloxacin, and other commonly used antimicrobial agents against 1,689 bacterial strains isolated at four Korean university hospitals during 1999-2000. Minimum inhibitory concentrations (MICs) were determined using the agar dilution method of National Committee for Clinical Laboratory Standards. Gemifloxacin had the lowest MICs for the respiratory pathogens: 90% of Streptococcus pneumoniae, Moraxella catarrhalis, and Haemophilus influenzae were inhibited by 0.06, 0.03, and 0.03 mg/L, respectively. Gemifloxacin was more active than the other fluoroquinolones against methicillin-susceptible Staphylococcus aureus, coagulase-negative staphylococci, streptococci, and Enterococcus faecalis. The MIC90s of gemifloxacin for Klebsiella oxytoca, Proteus vulgaris, and non-typhoidal Salmonella spp. were 0.25, 1.0, and 0.12 mg/L, respectively, while those for other Gram-negative bacilli were 4-64 mg/L. In conclusion, gemifloxacin was the most active among the comparative agents against Gram-positive species, including respiratory pathogens isolated in Korea.

  20. In vitro activity of gemifloxacin against recent clinical isolates of bacteria in Korea.

    PubMed Central

    Yong, Dong Eun; Cheong, Hee-Jin; Kim, Yang Soo; Park, Yeon Joon; Kim, Woo-Joo; Woo, Jun Hee; Lee, Kyung Won; Kang, Moon Won; Choo, Youn-Sung

    2002-01-01

    Gemifloxacin is an enhanced-affinity fluoroquinolone with broad-spectrum antibacterial activity. In Korea, resistant bacteria are relatively more prevalent than in other industrialized countries. In this study, we studied the in vitro activities of gemifloxacin, gatifloxacin, moxifloxacin, levofloxacin, ciprofloxacin, and other commonly used antimicrobial agents against 1,689 bacterial strains isolated at four Korean university hospitals during 1999-2000. Minimum inhibitory concentrations (MICs) were determined using the agar dilution method of National Committee for Clinical Laboratory Standards. Gemifloxacin had the lowest MICs for the respiratory pathogens: 90% of Streptococcus pneumoniae, Moraxella catarrhalis, and Haemophilus influenzae were inhibited by 0.06, 0.03, and 0.03 mg/L, respectively. Gemifloxacin was more active than the other fluoroquinolones against methicillin-susceptible Staphylococcus aureus, coagulase-negative staphylococci, streptococci, and Enterococcus faecalis. The MIC90s of gemifloxacin for Klebsiella oxytoca, Proteus vulgaris, and non-typhoidal Salmonella spp. were 0.25, 1.0, and 0.12 mg/L, respectively, while those for other Gram-negative bacilli were 4-64 mg/L. In conclusion, gemifloxacin was the most active among the comparative agents against Gram-positive species, including respiratory pathogens isolated in Korea. PMID:12482994

  1. Prevalence of Trichomonas vaginalis in Women Visiting 2 Obstetrics and Gynecology Clinics in Daegu, South Korea

    PubMed Central

    Goo, Youn-Kyoung; Shin, Won-Sik; Yang, Hye-Won; Joo, So-Young; Song, Su-Min; Ryu, Jae-Sook; Lee, Won-Myung; Kong, Hyun-Hee; Lee, Won-Ki; Lee, Sang-Eun; Lee, Won-Ja; Chung, Dong-Il; Hong, Yeonchul

    2016-01-01

    This study explored epidemiological trends in trichomoniasis in Daegu, South Korea. Wet mount microscopy, PCR, and multiplex PCR were used to test for Trichomonas vaginalis in vaginal swab samples obtained from 621 women visiting 2 clinics in Daegu. Of the 621 women tested, microscopy detected T. vaginalis in 4 (0.6%) patients, PCR detected T. vaginalis in 19 (3.0%) patients, and multiplex PCR detected T. vaginalis in 12 (1.9%) patients. Testing via PCR demonstrated high sensitivity and high negative predictive value for T. vaginalis. Among the 19 women who tested positive for T. vaginalis according to PCR, 94.7% (18/19) reported vaginal signs and symptoms. Notably, more than 50% of T. vaginalis infections occurred in females younger than 30 years old, and 58% were unmarried. Multiplex PCR, which simultaneously detects pathogens from various sexually transmitted infections, revealed that 91.7% (11/12) of patients were infected with 2 or more pathogens. Mycoplasma hominis was the most prevalent co-infection pathogen with T. vaginalis, followed by Ureaplasma urealyticum and Chlamydia trachomatis. Our results indicate that PCR and multiplex PCR are the most sensitive tools for T. vaginalis diagnosis, rather than microscopy which has been routinely used to detect T. vaginalis infections in South Korea. Therefore, clinicians should take note of the high prevalence of T. vaginalis infections among adolescent and young women in order to prevent persistent infection and transmission of this disease. PMID:26951983

  2. Seroprevalence of Leptospira spp. in clinically healthy racing horses in Korea.

    PubMed

    Jung, Byeong Yeal; Lee, Kyung Woo; Ha, Tae Young

    2010-02-01

    Leptospirosis is a zoonotic disease of global importance, and has a worldwide distribution. The present study aimed to determine leptospiral seroprevalence in clinically healthy racing horses from all three racecourses in Korea. Serum samples from 1,226 racing horses were examined using a microscopic agglutination test to detect the presence of antibodies against 18 Leptospira serovars. Of the tested samples, 307 (25.0%) were found to be positive. The distribution of seroprevalence differed significantly by racecourse (P=0.004); the Jeju course had the highest incidence (31.1%), followed by the Seoul (25.2%) and Busan (19.5%) racecourses. Seasonal variation in seropositivity was also apparent (P=0.000), being lower in spring (13.0%) and winter (12.5%), and higher in summer (36.7%) and autumn (34.7%). No significant age- or gender-related difference in seroprevalence was noted in this study (P>0.05). Seroprevalence was higher (P=0.006) among ponies than among thoroughbreds. Sejroe was the most frequently detected serovar (n=236), followed by Bratislava (n=35), Ballum (n=16), Autumnalis (n=10), and Canicola (n=10). The majority of serum titers were relatively low; most values ranged from 1:100 (n=217) to 1:200 (n=69). These results suggest that the Sejroe serovar may be maintained in the racing horse population in Korea.

  3. Clinical image evaluation of film mammograms in Korea: comparison with the ACR standard.

    PubMed

    Gwak, Yeon Joo; Kim, Hye Jung; Kwak, Jin Young; Son, Eun Ju; Ko, Kyung Hee; Lee, Jin Hwa; Lim, Hyo Soon; Lee, You Jin; Park, Ji Won; Shin, Kyung Min; Jang, Yun-Jin

    2013-01-01

    The goal of this study is to compare the overall quality of film mammograms taken according to the Korean standards with the American College of Radiology (ACR) standard for clinical image evaluation and to identify means of improving mammography quality in Korea. Four hundred and sixty eight sets of film mammograms were evaluated with respect to the Korean and ACR standards for clinical image evaluation. The pass and failure rates of mammograms were compared by medical facility types. Average scores in each category of the two standards were evaluated. Receiver operating characteristic curve analysis was used to identify an optimal Korean standard pass mark by taking the ACR standard as the reference standard. 93.6% (438/468) of mammograms passed the Korean standard, whereas only 80.1% (375/468) passed the ACR standard (p < 0.001). Non-radiologic private clinics had the lowest pass rate (88.1%: Korean standard, 71.8%: ACR standard) and the lowest total score (76.0) by the Korean standard. Average scores of positioning were lowest (19.3/29 by the Korean standard and 3.7/5 by the ACR standard). A cutoff score of 77.0 for the Korean standard was found to correspond to a pass level when the ACR standard was applied. We suggest that tighter regulations, such as, raising the Korean pass mark, subtracting more for severe deficiencies, or considering a very low scores in even a single category as failure, are needed to improve the quality of mammography in Korea.

  4. Factor V Deficiency in Korean Patients: Clinical and Laboratory Features, Treatment, and Outcome.

    PubMed

    Park, Young Hoon; Lim, Joo Han; Yi, Hyeon Gyu; Lee, Moon Hee; Kim, Chul Soo

    2016-02-01

    Due to rarity of factor V (FV) deficiency, there have been only a few case reports in Korea. We retrospectively analysed the clinical-laboratory features of FV deficiency in 10 Korean patients. Between January 1987 and December 2013, 10 case reports published in a Korean journal or proceedings of Korea Society on Thrombosis and Hemostasis were reviewed. Severity is defined as mild (> 5% of factor activity), moderate (1%-5%), and severe (< 1%). The median age at diagnosis, six males and four females, was 26 years (range, 1 month-73 years). Six of 10 patients were classified as moderate, three as mild, and one as severe disease. Eight patients were diagnosed as inherited FV deficiency. The most frequent symptoms were mucosal tract bleedings (40%) such as epistaxis, and menorrhagia in female. Hemarthroses and postoperative bleeding occurred in one and four patients, respectively. Life-threatening bleeding episodes occurred in the peritoneal cavity (n = 2), central nerve system (n = 1), and retroperitoneal space (n = 1). No lethal haemorrhages happened to patients with mild disease. The majority of bleeding episodes were controlled with local measures and fresh-frozen plasma replacement. Two acquired FV deficient-patients showing life-threatening haemorrhages received the immunosuppressive therapy, but one of them died from postoperative bleeding complications. Despite the small sample size of this study due to rarity of the disease, we found that Korean patients with FV deficiency had similar clinical manifestations and treatment outcomes shown in previous studies.

  5. Factor V Deficiency in Korean Patients: Clinical and Laboratory Features, Treatment, and Outcome

    PubMed Central

    2016-01-01

    Due to rarity of factor V (FV) deficiency, there have been only a few case reports in Korea. We retrospectively analysed the clinical-laboratory features of FV deficiency in 10 Korean patients. Between January 1987 and December 2013, 10 case reports published in a Korean journal or proceedings of Korea Society on Thrombosis and Hemostasis were reviewed. Severity is defined as mild (> 5% of factor activity), moderate (1%–5%), and severe (< 1%). The median age at diagnosis, six males and four females, was 26 years (range, 1 month-73 years). Six of 10 patients were classified as moderate, three as mild, and one as severe disease. Eight patients were diagnosed as inherited FV deficiency. The most frequent symptoms were mucosal tract bleedings (40%) such as epistaxis, and menorrhagia in female. Hemarthroses and postoperative bleeding occurred in one and four patients, respectively. Life-threatening bleeding episodes occurred in the peritoneal cavity (n = 2), central nerve system (n = 1), and retroperitoneal space (n = 1). No lethal haemorrhages happened to patients with mild disease. The majority of bleeding episodes were controlled with local measures and fresh-frozen plasma replacement. Two acquired FV deficient-patients showing life-threatening haemorrhages received the immunosuppressive therapy, but one of them died from postoperative bleeding complications. Despite the small sample size of this study due to rarity of the disease, we found that Korean patients with FV deficiency had similar clinical manifestations and treatment outcomes shown in previous studies. PMID:26839474

  6. Prevalence of Trichomonas vaginalis by PCR in men attending a primary care urology clinic in South Korea.

    PubMed

    Seo, Jun-Hyeok; Yang, Hye-Won; Joo, So-Young; Song, Su-Min; Lee, Yu-Ran; Ryu, Jae-Sook; Yoo, Eun Sang; Lee, Won Kee; Kong, Hyun-Hee; Lee, Sang-Eun; Lee, Won-Ja; Goo, Youn-Kyoung; Chung, Dong-Il; Hong, Yeonchul

    2014-10-01

    Trichomonas vaginalis, a causative agent of trichomoniasis, may trigger symptomatic or asymptomatic nongonococcal urethritis and chronic prostatitis in men. Despite the availability of highly sensitive diagnostic tests, such as nucleic acid amplification tests, including PCR, few prospective studies present data on male T. vaginalis infection in South Korea. In the present study, the prevalence of T. vaginalis and associated clinical conditions were evaluated in 201 male patients from a primary care urology clinic in South Korea. The prevalence of T. vaginalis infection in our cohort was 4% (8/201) by PCR. T. vaginalis infection was common in men older than 40 years (median age, 52 years). Among the 8 Trichomonas-positive patients, 87.5% (7/8) had prostatic diseases, such as prostatitis and benign prostatic hyperplasia, and 25.0% (2/8) and 12.5% (1/8) were coinfected with Chlamydia trachomatis and Mycoplasma genitalium, respectively. Our results suggest that T. vaginalis infection is not rare in men attending primary care urology clinics in South Korea, especially in those older than 40 years, in whom it may explain the presence of prostatic disease. The possibility of T. vaginalis infection should be routinely considered in older male patients with prostatic diseases in South Korea.

  7. Cachexia: clinical features when inflammation drives malnutrition.

    PubMed

    Laviano, Alessandro; Koverech, Angela; Mari, Alessia

    2015-11-01

    Cachexia is a clinically relevant syndrome which impacts on quality of life, morbidity and mortality of patients suffering from acute and chronic diseases. The hallmark of cachexia is muscle loss, which is triggered by disease-associated inflammatory response. Cachexia is a continuum and therefore a staging system is needed. Initially, a three-stage system (i.e. pre-cachexia, cachexia and refractory cachexia) was proposed. More recent evidence supports the use of a five-stage classification system, based on patient's BMI and severity of weight loss, to better predict clinical outcome. Also, large clinical trials in cancer patients demonstrated that cachexia emerging during chemotherapy has greater influence on survival than weight loss at baseline. Therefore, becoming widely accepted is the importance of routinely monitoring patients' nutritional status to detect early changes and diagnose cachexia in its early phases. Although cachexia is associated with the presence of anabolic resistance, it has been shown that sustained yet physiological hyperaminoacidaemia, as well as the use of specific nutrients, is able to overcome impaired protein synthesis and revert catabolism. More importantly, clinical evidence demonstrates that preservation of nutritional status during chemotherapy or improvement of body weight after weight loss is associated with longer survival in cancer patients.

  8. Metabolic myopathies: clinical features and diagnostic approach.

    PubMed

    Smith, Edward C; El-Gharbawy, Areeg; Koeberl, Dwight D

    2011-05-01

    The rheumatologist is frequently called on to evaluate patients with complaints of myalgia, muscle cramps, and fatigue. The evaluation of these patients presents a diagnostic challenge given the nonspecific and intermittent nature of their complaints, often leading to inappropriate diagnostic testing. When these symptoms are associated with physical exertion, a metabolic myopathy should be suspected Although inflammatory myopathies may present with similar features, such a pattern should prompt a thorough evaluation for an underlying metabolic myopathy. This review discusses the most common causes of metabolic myopathies and reviews the current diagnostic options available to the clinician.

  9. Clinical characteristics of patients with treated epilepsy in Korea: a nationwide epidemiologic study.

    PubMed

    Kim, Dong Wook; Lee, Seo-Young; Chung, Soo-Eun; Cheong, Hae-Kwan; Jung, Ki-Young

    2014-01-01

    Although a number of epidemiologic studies have been conducted on the prevalence and incidence of epilepsy around the world, only a few studies have investigated the clinical characteristics of patients with epilepsy in a population-based sample. The purpose of the present study was to describe the clinical characteristics of treated patients with epilepsy in Korea via a nationwide medical records survey. The study population was obtained through a nationwide database registered to the Health Insurance Review and Assessment service. Patients were recruited from clinics and hospitals in each cluster according to region and referral level by random selection from a preallocated sample of patients. All patients were being treated with antiepileptic drug medication with or without a diagnosis code for epilepsy or seizure between January 2009 and December 2009. Among the 6,436 selected patients, 2,150 met the diagnostic criteria for epilepsy and were included in our survey on the clinical characteristics of patients who were with treated epilepsy. The proportion of male patients with epilepsy in this study was higher (1,226; 57.0%) than that of female patients. In addition, 10.6% of patients were first diagnosed with epilepsy in 2009, and 53.6% of patients experienced at least one seizure over the course of 2009; 78.1% were classified as having localization-related epilepsy, whereas 7.3% were considered to have generalized epilepsy. Thirty-five percent of patients were thus classified as idiopathic or cryptogenic cases. The most common cause of symptomatic epilepsy was trauma (10.0%), followed by stroke (9.6%), central nervous system (CNS) infection (5.7%), and hippocampal sclerosis (4.9%). This is the first nationwide study of the clinical characteristics of treated epilepsy in Korea using a national database validated by medical records survey. The etiologies of epilepsy and epilepsy syndrome classifications were comparable to those previously reported in other

  10. Parasomnias: clinical features and forensic implications.

    PubMed

    Bornemann, Michel A Cramer; Mahowald, Mark W; Schenck, Carlos H

    2006-08-01

    Parasomnias are undesirable behavioral or experiential phenomena arising from the sleep period. Once felt to be a unitary phenomenon, it is now clear that a wide variety of sleep disorders are capable of resulting in complex behaviors arising during sleep. The most common are disorders of arousal and rapid eye movement sleep disorder. Less common conditions include nocturnal seizures and psychogenic dissociative states. Malingering and Munchausen syndrome by proxy, while they are not actually parasomnias, may masquerade as parasomnias. Careful clinical and sleep laboratory evaluation can usually provide an accurate diagnosis with effective therapeutic implications. Due to the potential forensic implications, sleep medicine specialists may be asked to participate in legal proceedings resulting from sleep-related violence. An awareness of the spectrum of such behaviors, and their clinical and legal evaluation, is becoming more important in the practice of sleep medicine.

  11. Clinical Characteristics and Treatment Outcomes of Primary Pulmonary Artery Sarcoma in Korea

    PubMed Central

    2016-01-01

    Pulmonary artery sarcomas (PAS) are rare malignant neoplasms. Right heart failure due to tumour location is the main cause of death in PAS patients. The hemodynamic influence of PAS may effect prognosis, but this has not been proven. We aimed to identify the clinical characteristics and prognostic factors of PAS in Korea, their association with pulmonary hypertension (PH). PAS patients treated at the Asan Medical Center between 2000 and 2014 were reviewed. We examined demographic characteristics, diagnostic and treatment modalities. Potential prognostic factors were evaluated by univariate and multivariate analysis. Twenty patients were diagnosed with PAS. Ten patients were male, the median age was 54 years (range, 33–75 years). The most common symptom observed was dyspnea (65%). The most common histologic type was spindle cell sarcoma (30%). Ten patients had a presumptive diagnosis of pulmonary embolism (PE) and received anticoagulation therapy. Seventeen patients underwent surgery, but only 5 patients had complete resection. Eleven patients received post-operative treatment (chemotherapy = 3, radiotherapy = 5, chemoradiotherapy = 3). PH was observed in 12 patients before treatment and in 6 patients after treatment. Overall median survival was 24 months. Post-treatment PH was associated with poor prognosis (HR 9.501, 95% CI 1.79–50.32; P = 0.008) while chemotherapy was negatively associated with mortality (HR 0.102, 95% CI 0.013–0.826; P = 0.032) in univariate analysis. Post-treatment PH was also associated with poor prognosis in multivariate analysis (HR 5.7, 95% CI 1.08–30.91; P = 0.041). PAS patients are frequently misdiagnosed with PE in Korea. Post-treatment PH is associated with a poor prognosis. PMID:27709853

  12. Cortical auditory disorders: clinical and psychoacoustic features.

    PubMed Central

    Mendez, M F; Geehan, G R

    1988-01-01

    The symptoms of two patients with bilateral cortical auditory lesions evolved from cortical deafness to other auditory syndromes: generalised auditory agnosia, amusia and/or pure word deafness, and a residual impairment of temporal sequencing. On investigation, both had dysacusis, absent middle latency evoked responses, acoustic errors in sound recognition and matching, inconsistent auditory behaviours, and similarly disturbed psychoacoustic discrimination tasks. These findings indicate that the different clinical syndromes caused by cortical auditory lesions form a spectrum of related auditory processing disorders. Differences between syndromes may depend on the degree of involvement of a primary cortical processing system, the more diffuse accessory system, and possibly the efferent auditory system. Images PMID:2450968

  13. Distribution features of biological hazardous pollutants in residential environments in Korea.

    PubMed

    Lee, Cheol Min; Hong, Soo Jong; Kim, Yoon Shin; Park, Gee Yong; Nam Goung, Sun Ju; Kim, Kyung Hwan

    2014-01-01

    This research has been conducted continuously since 2009 as part of a cohort of studies examining relationships between asthma and genetic factors, dietary habits, and environmental factors. Based on data from environmental research on house dust mites and endotoxins, which are widely known as pollutants in bedding that cause asthma in pregnant women and children, this work was conducted to obtain basic data that can be used in future cohort studies that analyze links between distribution of biological hazards and physical features of residential environments. The detection rates of house dust mite allergens, Der p1 and Der f1, were 52.7 and 86.5%, respectively, indicating that Der f1 is a dominant species in domestic indoor environments. According to comparisons between concentrations of house dust mites and endotoxins in bedding of pregnant women and children, Der p1 and endotoxins showed significantly lower concentrations in bedding of pregnant women compared with those in bedding of children, whereas Der f1 showed no significant difference in concentration according to bedding.

  14. Clinical and laboratory features of Nocardia nova.

    PubMed Central

    Wallace, R J; Brown, B A; Tsukamura, M; Brown, J M; Onyi, G O

    1991-01-01

    Recent studies have shown that Nocardia asteroides isolates have five major antibiotic resistance patterns; one of these patterns identifies isolates of Nocardia farcinica. In the current study, we investigated a second pattern characterized by susceptibility to ampicillin and erythromycin. This pattern was seen in 17% of 223 clinical isolates identified by standard techniques as N. asteroides and associated with diseases typical for nocardiae. Biochemically, isolates with this drug pattern were relatively homogeneous and identical to the type strain and previous descriptions of Nocardia nova. The strains studied were unique among nocardiae in having both alpha- and beta-esterase activity (85 and 95%, respectively). However, the arylsulfatase activity at 14 days (75%) and antimicrobial susceptibility patterns, including susceptibility to erythromycin (100%), were the only routinely available methods that would separate N. nova strains from other members of N. asteroides. N. asteroides should be considered a complex because current clinical identification schemes include isolates of N. farcinica and N. nova and may well include additional species. This is the first detailed description of N. nova as a pathogen in humans. PMID:1774244

  15. Clinical Features and Outcome of Mucormycosis

    PubMed Central

    Camara-Lemarroy, Carlos Rodrigo; González-Moreno, Emmanuel Irineo; Rodríguez-Gutiérrez, René; Rendón-Ramírez, Erick Joel; Ayala-Cortés, Ana Sofía; Fraga-Hernández, Martha Lizeth; García-Labastida, Laura; Galarza-Delgado, Dionicio Ángel

    2014-01-01

    Mucormycosis (MCM) is a life-threatening infection that carries high mortality rates despite recent advances in its diagnosis and treatment. The objective was to report 14 cases of mucormycosis infection and review the relevant literature. We retrospectively analyzed the demographic and clinical data of 14 consecutive patients that presented with MCM in a tertiary-care teaching hospital in northern Mexico. The mean age of the patients was 39.9 (range 5–65). Nine of the patients were male. Ten patients had diabetes mellitus as the underlying disease, and 6 patients had a hematological malignancy (acute leukemia). Of the diabetic patients, 3 had chronic renal failure and 4 presented with diabetic ketoacidosis. All patients had rhinocerebral involvement. In-hospital mortality was 50%. All patients received medical therapy with polyene antifungals and 11 patients underwent surgical therapy. Survivors were significantly younger and less likely to have diabetes than nonsurvivors, and had higher levels of serum albumin on admission. The clinical outcome of patients with MCM is poor. Uncontrolled diabetes and age are negative prognostic factors. PMID:25210515

  16. Partial biotinidase deficiency: clinical and biochemical features.

    PubMed

    McVoy, J R; Levy, H L; Lawler, M; Schmidt, M A; Ebers, D D; Hart, P S; Pettit, D D; Blitzer, M G; Wolf, B

    1990-01-01

    Neonatal screening for profound biotinidase deficiency (less than 10% of the mean normal activity level) has identified a group of children with partial biotinidase deficiency (10% to 30% of mean normal activity). Because partial biotinidase deficiency may result in clinical consequences that may be prevented by treatment with biotin, we evaluated such individuals and their family members (1) to determine whether partial biotinidase deficiency is associated with symptoms and (2) to determine the inheritance pattern. We quantified serum biotinidase activity levels and obtained medical histories of probands, their parents and siblings, and additional family members. All children with partial deficiency were healthy at the time of diagnosis. One child, who was not initially treated with biotin, later developed hypotonia, hair loss, and skin rash, which resolved with biotin therapy. Four adults and three children with partial biotinidase deficiency were identified among family members of infants identified by neonatal screening. All these individuals were healthy, although one sibling had elevated urinary lactate excretion. A fifth adult with partial deficiency, found among clinically normal adult volunteers, later showed minor symptoms that resolved after biotin therapy. Like children with profound biotinidase deficiency, children with partial biotinidase deficiency are symptoms free at birth. However, the subsequent occurrence of symptoms of profound biotinidase deficiency in some persons with partial deficiency suggests that biotin therapy for this condition may be warranted.

  17. Punctate follicular porokeratosis: clinical and pathologic features.

    PubMed

    Trikha, Ritika; Wile, Anna; King, Joy; Ward, Kimberley H M; Brodell, Robert T

    2015-11-01

    Porokeratosis is a disorder of keratinization characterized by an abnormal cornoid lamella surrounding an annular, scaly plaque with an atrophic center. A histologic variant of this condition has been proposed, termed follicular porokeratosis, in cases where follicular involvement was contiguous with an annular cornoid lamella. There has been only 1 report of punctate follicular porokeratosis, in which cornoid lamellae originated exclusively from hair follicles with no associated annular plaque. The authors present the second case of punctate follicular porokeratosis, further supporting the contention that this entity is a unique form of porokeratosis rather than a histologic variant. A 56-year-old African American female presented to the dermatology clinic with a 3-month history of keratotic lesions localized on the right posterior shoulder. Examination revealed an area of perifollicular keratotic papules, each surrounded by an erythematous rim. Histopathology revealed a cornoid lamella originating within a hair follicle, with the parakeratotic column protruding through the follicular orifice. The static nature of the condition along with exclusive involvement of hair follicles supports the notion of punctate follicular porokeratosis as a distinct clinical entity. The diagnosis of this condition relies heavily on proper histopathologic sampling revealing punctate follicular cornoid lamellae.

  18. [Clinical features of malignant hyperthermia crisis].

    PubMed

    Cornet, C; Moeller, R; Laxenaire, M C

    1989-01-01

    Malignant hyperthermia (MH) is a pharmacogenetic disorder. It is classically described as a hypermetabolic state triggered by halogenated anaesthetics and/or depolarizing muscle relaxants. In fact, since Denborough and Lovel's case, it has been shown that MH has a great number of clinical forms. The overwhelming picture of muscular hypercatabolism with fulminating hyperthermia and generalized rigidity is becoming rare. A better knowledge of the first symptoms explains in part the better prognosis: masseter spasm after suxamethonium, an increase in expired CO2 concentration, unexplained tachycardia, ventricular arrhythmias. The use of dantrolene reduced the mortality of MH. The different types of clinical manifestations are due to genetic differences, the concentration of the anaesthetic agent, and the length of time of exposure to the drug. The severity of the episode is linked to environmental factors such as stress, physical exercise, ambient temperature, concomitant use of other drugs. Masseter spasm after suxamethonium is specific for MH, but not pathognomonic. It occurs in 1% of cases in children when using halothane with suxamethonium. However, in those patients who displayed such a spasm, more than 50% had a positive contracture test. Masseter spasm is often associated with severe rhabdomyolysis in patients with muscle dystrophy, especially Duchenne's dystrophy. In the latter case, major cardiac problems may occur at the time of anaesthetic induction. Even if there are no other signs of MH, all patients who have had a masseter spasm must be considered as open to doubt, and should be further explored. MH is often difficult to diagnose in medium severity types.(ABSTRACT TRUNCATED AT 250 WORDS)

  19. Benign Occipital Epilepsies of Childhood: Clinical Features and Genetics

    ERIC Educational Resources Information Center

    Taylor, Isabella; Berkovic, Samuel F.; Kivity, Sara; Scheffer, Ingrid E.

    2008-01-01

    The early and late benign occipital epilepsies of childhood (BOEC) are described as two discrete electro-clinical syndromes, eponymously known as Panayiotopoulos and Gastaut syndromes. Our aim was to explore the clinical features, classification and clinical genetics of these syndromes using twin and multiplex family studies to determine whether…

  20. Benign Occipital Epilepsies of Childhood: Clinical Features and Genetics

    ERIC Educational Resources Information Center

    Taylor, Isabella; Berkovic, Samuel F.; Kivity, Sara; Scheffer, Ingrid E.

    2008-01-01

    The early and late benign occipital epilepsies of childhood (BOEC) are described as two discrete electro-clinical syndromes, eponymously known as Panayiotopoulos and Gastaut syndromes. Our aim was to explore the clinical features, classification and clinical genetics of these syndromes using twin and multiplex family studies to determine whether…

  1. Which ante mortem clinical features predict progressive supranuclear palsy pathology?

    PubMed

    Respondek, Gesine; Kurz, Carolin; Arzberger, Thomas; Compta, Yaroslau; Englund, Elisabet; Ferguson, Leslie W; Gelpi, Ellen; Giese, Armin; Irwin, David J; Meissner, Wassilios G; Nilsson, Christer; Pantelyat, Alexander; Rajput, Alex; van Swieten, John C; Troakes, Claire; Josephs, Keith A; Lang, Anthony E; Mollenhauer, Brit; Müller, Ulrich; Whitwell, Jennifer L; Antonini, Angelo; Bhatia, Kailash P; Bordelon, Yvette; Corvol, Jean-Christophe; Colosimo, Carlo; Dodel, Richard; Grossman, Murray; Kassubek, Jan; Krismer, Florian; Levin, Johannes; Lorenzl, Stefan; Morris, Huw; Nestor, Peter; Oertel, Wolfgang H; Rabinovici, Gil D; Rowe, James B; van Eimeren, Thilo; Wenning, Gregor K; Boxer, Adam; Golbe, Lawrence I; Litvan, Irene; Stamelou, Maria; Höglinger, Günter U

    2017-07-01

    Progressive supranuclear palsy (PSP) is a neuropathologically defined disease presenting with a broad spectrum of clinical phenotypes. To identify clinical features and investigations that predict or exclude PSP pathology during life, aiming at an optimization of the clinical diagnostic criteria for PSP. We performed a systematic review of the literature published since 1996 to identify clinical features and investigations that may predict or exclude PSP pathology. We then extracted standardized data from clinical charts of patients with pathologically diagnosed PSP and relevant disease controls and calculated the sensitivity, specificity, and positive predictive value of key clinical features for PSP in this cohort. Of 4166 articles identified by the database inquiry, 269 met predefined standards. The literature review identified clinical features predictive of PSP, including features of the following 4 functional domains: ocular motor dysfunction, postural instability, akinesia, and cognitive dysfunction. No biomarker or genetic feature was found reliably validated to predict definite PSP. High-quality original natural history data were available from 206 patients with pathologically diagnosed PSP and from 231 pathologically diagnosed disease controls (54 corticobasal degeneration, 51 multiple system atrophy with predominant parkinsonism, 53 Parkinson's disease, 73 behavioral variant frontotemporal dementia). We identified clinical features that predicted PSP pathology, including phenotypes other than Richardson's syndrome, with varying sensitivity and specificity. Our results highlight the clinical variability of PSP and the high prevalence of phenotypes other than Richardson's syndrome. The features of variant phenotypes with high specificity and sensitivity should serve to optimize clinical diagnosis of PSP. © 2017 International Parkinson and Movement Disorder Society. © 2017 International Parkinson and Movement Disorder Society.

  2. [Clinical Features and Treatment of Hashimoto Encephalopathy].

    PubMed

    Maki, Yoshimitsu; Takashima, Hiroshi

    2016-09-01

    Hashimoto encephalopathy (HE) is characterized by heterogeneous neurological symptoms. HE is diagnosed based on three criteria-the presence of antithyroid antibodies, neurological symptoms from the cerebrum and/or cerebellum, and a positive response to immunotherapy. We clinically analyzed 18 patients (3 men, 15 women; age range, 38-81years) diagnosed with HE in our hospital from May 2013 to January 2016. Eleven patients showed sensory abnormalities such as strong pain, deep muscle pain, dysesthesia, paresthesia, or neuralgia. Surprisingly, the majority of the pain was distributed in a manner that was not explainable anatomically. Seventeen patients showed motor disturbances, such as weakness, paresis of extremities, or dexterity movement disorder, and eight patients showed give-way weakness, which is disruption of continuous muscle contraction. Other symptoms indicative of brain-related anomalies such as tremor, dystonia, involuntary movements, cerebellar ataxia, parkinsonism, memory loss, and chronic fatigue were also seen. In most patients, such motor, sensory, or higher brain functions were markedly improved with immunosuppressive therapies such as prednisolone, azathioprine, or immunoadsorption therapy. Although give-way weakness and anatomically unexplainable pain are typically considered as being psychogenic in origin, the presence of these symptoms is indicative of HE. HE exhibits diffuse involvement of the entire brain and thus, these symptoms are explainable. We propose that physicians should not diagnose somatoform disorders without first excluding autoimmune encephalopathy.

  3. Pediatric multiple sclerosis: Clinical features and outcome.

    PubMed

    Waldman, Amy; Ness, Jayne; Pohl, Daniela; Simone, Isabella Laura; Anlar, Banu; Amato, Maria Pia; Ghezzi, Angelo

    2016-08-30

    Multiple sclerosis (MS) in children manifests with a relapsing-remitting MS (RRMS) disease course. Acute relapses consist of new neurologic deficits persisting greater than 24 hours, in the absence of intercurrent illness, and occur with a higher frequency early in the disease as compared to adult-onset RRMS. Most pediatric patients with MS recover well from these early relapses, and cumulative physical disability is rare in the first 10 years of disease. Brainstem attacks, poor recovery from a single attack, and a higher frequency of attacks portend a greater likelihood of future disability. Although prospective pediatric-onset MS cohorts have been established in recent years, there remains very limited prospective data detailing the longer-term clinical outcome of pediatric-onset MS into adulthood. Whether the advent of MS therapies, and the largely off-label access to such therapies in pediatric MS, has improved prognosis is unknown. MS onset during the key formative academic years, concurrent with active cognitive maturation, is an important determinant of long-term outcome, and is discussed in detail in another article in this supplement. Finally, increasing recognition of pediatric MS worldwide, recent launch of phase III trials for new agents in the pediatric MS population, and the clear imperative to more fully appreciate health-related quality of life in pediatric MS through adulthood highlight the need for standardized, validated, and robust outcome measures.

  4. [Oral candidiasis: clinical features and control].

    PubMed

    Yamamoto, Tetsuya

    2010-10-01

    Candidiasis is the most commonly encountered fungal infection, and oral candidiasis is often observed as a local opportunistic infection. Oral candidiasis is clinically divided into three types: acute forms, chronic forms, and Candida-associated lesions. Candida adhesion and multiplication are largely regulated by the local and systemic factors of the host. The local factors include impairment of the oral mucosal integrity, which is usually impaired by hyposalivation, anticancer drugs/radiation for head and neck cancers, denture wearing, a decrease in the oral bacterial population, and poor oral hygiene. Among Candida species, oral candidiasis is mostly caused by Candida albicans (C. albicans), C. glabrata, or C. tropicalis. Oral Candida induces a variety of symptoms, such as oral mucosal inflammation manifesting as an uncomfortable feeling, pain, erythema, erosion, taste abnormalities, and hyperplasia of the oral mucosa. Candida overgrowth in the oral cavity may disseminate to distant organs. Therefore, in order to avoid the sequelae of systemic candidiasis, oral candidiasis should be rapidly controlled. Oral candidiasis is usually treated by the local application of antifungal drugs. However, oral candidiasis occasionally escapes the control of such local treatment due to the development of multi-drug resistant Candida strains and species or due to the suppression of salivation or cellular immune activity. When drug-resistant strains are suspected as the pathogens and when the host is generally compromised, the oral administration of combinations of antifungal drugs, enhancement of cellular immune activity, and improvement of the nutritional condition are recommended.

  5. Porphyria cutanea tarda: clinical and laboratory features.

    PubMed Central

    Sweeney, G. D.; Jones, K. G.

    1979-01-01

    Eleven patients with porphyria cutanea tarda were studied. Biochemical confirmation of the clinical diagnosis required only determination of the total urine porphyrin concentration in a sample of urine voided on rising in the morning. The patients were divided for convenience of discussion into four groups differing in age, sex and etiologic factors. Of the six patients in whom a liver biopsy was done one was shown to have micronodular cirrhosis. Except for a modest elevation in the serum glutamic oxaloacetic transaminase values when the patients were first seen, no evidence was found for liver disease apart from the presence of porphyria cutanea tarda. One patient recovered solely by abstaining from alcohol consumption. Five patients underwent phlebotomy; their iron stores had been found to be between 2 and 3 g. Decreasing urine porphyrin values correlated well with decreasing serum ferritin values during the course of phlebotomy. Porphyria cutanea tarda, which is due to a deficiency of uroporphyrinogen decarboxylase, is manifested in association with alcohol abuse, estrogen therapy, exposure to chlorinated hydrocarbons or increased tissue iron stores, or a combination of these factors. Although relatively uncommon, this condition raises important and unresolved issues regarding the hepatotoxicity of alcohol, estrogens, chlorinated hydrocarbons and iron. PMID:427687

  6. Clinical features of Lassa fever in Liberia.

    PubMed

    Frame, J D

    1989-01-01

    Two hundred thirteen cases of Lassa fever (LF) were diagnosed by virus isolation and seroconversion at Curran Lutheran Hospital in Zorzor, Liberia, between July 1980 and April 1986. An additional 40 cases of probable and presumptive LF were diagnosed on the basis of single serum samples. Of the 246 assessable patients, 23 (9%) died; no data were available for seven patients. Five (16%) of 32 pregnant women and three (43%) of seven immediately postpartum women died. Four (26%) of 15 children less than 12 years died. Case-fatality rates among 125 nonpregnant women and 67 men were approximately 6%. Among 150 patients studied in detail, the case-fatality rate was also 9%. Seventeen (11%) of these patients had abnormal bleeding; of these, six (35%) died. Most platelet counts were at low normal to mildly depressed levels. However, serial counts in seven patients suggested a decrease on about days 10-12 of illness. The symptoms of LF in Liberia are those of a viral syndrome. Edema, sometimes marked, is noted in seriously ill patients. A great variation in mortality and incidence of abnormal bleeding is recorded in reported series of LF; it appears that hemorrhage is a marker for cases with a high mortality. The incidence and severity of hearing defects in LF outbreaks vary. Elucidation of a number of clinical problems in LF requires more information on how strain differences affect the pattern of illness.

  7. [Pathogenesis and clinical features of polypous rhinosinusitis].

    PubMed

    Trofimenko, S L

    2010-01-01

    The paper presents recommendations of the current European and Russian documents concerning pathogenesis of polypous rhinosinusitis (International Consensus Conference on Nasal Polyposis (2006), European documents EAACI - EP3OS (2007), and Summit of the Russian Society of Rhinologists "Nasal polyposis and inflammation" (2009)). The bilateral polypous process in the nasal cavity is considered to be a "special form of rhinosinusitis" in which bacterial superantigens or fungal infection induce chronic eosinophilic inflammation. Researchers of the ENT Department, Rostov State Medical University, undertook analysis of the results of long-term comprehensive examination of patients with polypous rhinosinusitis that included clinical, bacteriological, histomorphological, and allergological studies as well as evaluation of local and systemic immunity. The data obtained allowed to describe one of the forms of polypous rhinosinusitis as chronic infection-dependent allergic rhinosinusitis with the manifestation of all four types of allergic reactions, formation of the autoimmune component, and development of persistent immune inflammation leading to remodeling of endonasal mucosa. In all these cases, the process progressed parallel to the development of combined secondary immune deficiency (SID). A hypothetical scheme of pathogenesis of chronic polypous allergic rhinosinusitis is proposed.

  8. Clinical features and pathophysiology of belching disorders.

    PubMed

    Sun, Xiaomin; Ke, Meiyun; Wang, Zhifeng

    2015-01-01

    The symptomatic characteristics and psychosocial factors of belching were analyzed through questionnaire, and the pathophysiology of belch was studied with the combined methods of high-resolution manometry and impedance (HRM-IMP). 21 consecutively-enrolled patients with repeated belching, were conducted a questionnaire survey, and the data were analyzed with Pearson correlation analysis and exact propability method. 10 patients were performed HRM-IMP. 20 patients met the diagnostic criteria of belch, among who 17 cases had overlapped symptoms, and the functional dyspepsia was the most common, followed by gastroesophageal reflux disease; 16 patients experienced mental stimulation or negative events; 12 cases existed anxiety and (or) depression; 8 cases had neurotic personality. The number of the overlapped symptoms was associated with the anxiety status, while the belch severity had nothing to do with the anxiety/depression status. Among the 10 patients who accepted HRM-IMP, 9 existed the esophageal motility dysfunction, and 9 had supra-gastric belching. Belch had a variety of clinical manifestations, related to the mood changes and the environmental stress, and normally was combined with the abnormalities of psychology and personality traits. The belch patients normally experienced the esophageal motility disorders, among which the typical pattern was supra-gastric belching.

  9. Fetal alcohol spectrum disorders: Clinical phenotype among a high-risk group of children and adolescents in Korea.

    PubMed

    Lee, Hyun-Seung; Jones, Kenneth Lyons; Lee, Hae Kook; Chambers, Christina D

    2016-01-01

    Little is known about the prevalence and phenotype of fetal alcohol syndrome (FAS) or spectrum disorders (FASD) in Korea. This study was performed to describe the distribution of alcohol-related physical features in a genetically homogeneous sample of children and adolescents in institutional settings in Korea. Children and adolescents receiving services in one of seven institutions in Seoul, Korea were screened for growth deficiency. Those who screened positive were assessed using a structured protocol for the key cardinal features of FAS, and for 11 additional alcohol-related dysmorphologic features. Based on these findings, children and adolescents were categorized as FAS, Deferred (some characteristic features of FAS), and No FAS. Groups were compared on the prevalence of specific additional features and number of additional features, stratified by gender and age. Of 307 children and adolescents screened, 87 received the dysmorphology evaluation. Thirteen were classified as FAS, 44 Deferred, and 30 No FAS. The frequency of 10 of the 11 additional alcohol-related features did not differ significantly by FAS category. Palmar crease abnormalities were more common in FAS (53.8%) than in the Deferred category (25.0%) or the No FAS category (6.7%) (P = 0.003). A high prevalence across all groups was found for midfacial hypoplasia and epicanthal folds, whereas only one child exhibited ptosis. This study suggests that an FASD phenotype variant related to ethnic differences in the range of defects specific to prenatal alcohol exposure may be present in the Korean population. © 2015 Wiley Periodicals, Inc.

  10. Clinical Features of Lysosomal Acid Lipase Deficiency

    PubMed Central

    Burton, Barbara K.; Deegan, Patrick B.; Enns, Gregory M.; Guardamagna, Ornella; Horslen, Simon; Hovingh, Gerard K.; Lobritto, Steve J.; Malinova, Vera; McLin, Valerie A.; Raiman, Julian; Di Rocco, Maja; Santra, Saikat; Sharma, Reena; Sykut-Cegielska, Jolanta; Whitley, Chester B.; Eckert, Stephen; Valayannopoulos, Vassili; Quinn, Anthony G.

    2015-01-01

    Abstract Objective: The aim of this study was to characterize key clinical manifestations of lysosomal acid lipase deficiency (LAL D) in children and adults. Methods: Investigators reviewed medical records of LAL D patients ages ≥5 years, extracted historical data, and obtained prospective laboratory and imaging data on living patients to develop a longitudinal dataset. Results: A total of 49 patients were enrolled; 48 had confirmed LAL D. Mean age at first disease-related abnormality was 9.0 years (range 0–42); mean age at diagnosis was 15.2 years (range 1–46). Twenty-nine (60%) were male patients, and 27 (56%) were <20 years of age at the time of consent/assent. Serum transaminases were elevated in most patients with 458 of 499 (92%) of alanine aminotransferase values and 265 of 448 (59%) of aspartate aminotransferase values above the upper limit of normal. Most patients had elevated low-density lipoprotein (64% patients) and total cholesterol (63%) at baseline despite most being on lipid-lowering therapies, and 44% had high-density lipoprotein levels below the lower limit of normal. More than half of the patients with liver biopsies (n = 31, mean age 13 years) had documented evidence of steatosis (87%) and/or fibrosis (52%). Imaging assessments revealed that the median liver volume was ∼1.15 multiples of normal (MN) and median spleen volume was ∼2.2 MN. Six (13%) patients had undergone a liver transplant (ages 9–43.5 years). Conclusion: This study provides the largest longitudinal case review of patients with LAL D and confirms that LAL D is predominantly a pediatric disease causing early and progressive hepatic dysfunction associated with dyslipidemia that often leads to liver failure and transplantation. PMID:26252914

  11. [Clinical features of accessory parotid gland tumors].

    PubMed

    Iguchi, Hiroyoshi; Wada, Tadashi; Yamamoto, Hidefumi; Yamada, Kei; Matsushita, Naoki; Okamoto, Sachimi; Teranishi, Yuichi; Koda, Yuki; Kosugi, Yuki; Yamane, Hideo

    2013-12-01

    Accessory parotid gland tumors are relatively rare; hence, adequately detailed clinical analyses of these tumors are difficult to perform at a single institution. In this report, we describe the findings for 65 patients [29 men, 36 women; median age, 51 (9-81) years] with accessory parotid gland tumors, consisting of 4 cases documented by us and 61 cases previously reported by other Japanese authors. Approximately 50% of the patients were treated in an otolaryngology department, while the remaining patients were treated in plastic surgery, oral surgery, or dermatology departments. In 4 patients, the results of preoperative fine-needle aspiration cytology indicated that the tumor was benign; however, the postoperative histopathology results revealed malignant tumors. The frequencies of malignant and benign tumors were 44.6% (n = 29) and 55.4% (n = 36), respectively. Mucoepidermoid carcinoma and pleomorphic adenoma were the most frequent types of malignant and benign accessory parotid gland tumors, respectively. Among the various surgical methods that were used, such as direct cheek and intraoral incisions, a standard parotidectomy incision was the most preferred treatment approach for these tumors. Recently, an endoscopic approach has also been found to yield satisfactory results. An optimal approach should be selected after evaluating the advantages and disadvantages of these methods. No definite guidelines are available regarding the choice of elective neck dissection and postoperative radiation therapy for malignant accessory parotid gland tumors. Although tumor resection (plus elective neck dissection) and postoperative radiation therapy have been frequently performed for various kinds of malignant accessory parotid gland tumors to date, additional studies are needed regarding the criteria for selecting elective neck dissection and postoperative radiation therapy. Since the malignancy rate for accessory parotid gland tumors is higher than that for parotid gland

  12. Clinical Features of Lysosomal Acid Lipase Deficiency.

    PubMed

    Burton, Barbara K; Deegan, Patrick B; Enns, Gregory M; Guardamagna, Ornella; Horslen, Simon; Hovingh, Gerard K; Lobritto, Steve J; Malinova, Vera; McLin, Valerie A; Raiman, Julian; Di Rocco, Maja; Santra, Saikat; Sharma, Reena; Sykut-Cegielska, Jolanta; Whitley, Chester B; Eckert, Stephen; Valayannopoulos, Vassili; Quinn, Anthony G

    2015-12-01

    The aim of this study was to characterize key clinical manifestations of lysosomal acid lipase deficiency (LAL D) in children and adults. Investigators reviewed medical records of LAL D patients ages ≥5 years, extracted historical data, and obtained prospective laboratory and imaging data on living patients to develop a longitudinal dataset. A total of 49 patients were enrolled; 48 had confirmed LAL D. Mean age at first disease-related abnormality was 9.0 years (range 0-42); mean age at diagnosis was 15.2 years (range 1-46). Twenty-nine (60%) were male patients, and 27 (56%) were <20 years of age at the time of consent/assent. Serum transaminases were elevated in most patients with 458 of 499 (92%) of alanine aminotransferase values and 265 of 448 (59%) of aspartate aminotransferase values above the upper limit of normal. Most patients had elevated low-density lipoprotein (64% patients) and total cholesterol (63%) at baseline despite most being on lipid-lowering therapies, and 44% had high-density lipoprotein levels below the lower limit of normal. More than half of the patients with liver biopsies (n = 31, mean age 13 years) had documented evidence of steatosis (87%) and/or fibrosis (52%). Imaging assessments revealed that the median liver volume was ∼1.15 multiples of normal (MN) and median spleen volume was ∼2.2 MN. Six (13%) patients had undergone a liver transplant (ages 9-43.5 years). This study provides the largest longitudinal case review of patients with LAL D and confirms that LAL D is predominantly a pediatric disease causing early and progressive hepatic dysfunction associated with dyslipidemia that often leads to liver failure and transplantation.

  13. Polymicrobial Infective Endocarditis: Clinical Features and Prognosis

    PubMed Central

    García-Granja, Pablo Elpidio; López, Javier; Vilacosta, Isidre; Ortiz-Bautista, Carlos; Sevilla, Teresa; Olmos, Carmen; Sarriá, Cristina; Ferrera, Carlos; Gómez, Itziar; Román, José Alberto San

    2015-01-01

    Abstract To describe the profile of left-sided polymicrobial endocarditis (PE) and to compare it with monomicrobial endocarditis (ME). Among 1011 episodes of left-sided endocarditis consecutively diagnosed in 3 tertiary centers, between January 1, 1996 and December 31, 2014, 60 were polymicrobial (5.9%), 821 monomicrobial (81.7%), and in 123 no microorganism was detected (12.2%). Seven patients (0.7%) were excluded from the analysis because contamination of biologic tissue could not be discarded. The authors described the clinical, microbiologic, echocardiographic, and outcome of patients with PE and compared it with ME. Mean age was 64 years SD 16 years, 67% were men and 30% nosocomial. Diabetes mellitus (35%) were the most frequent comorbidities, fever (67%) and heart failure (43%) the most common symptoms at admission. Prosthetic valves (50%) were the most frequent infection location and coagulase-negative Staphylococci (48%) and enterococci (37%) the leading etiologies. The most repeated combination was coagulase-negative Staphylococci with enterococci (n = 9). Polymicrobial endocarditis appeared more frequently in patients with underlying disease (70% versus 56%, P = 0.036), mostly diabetics (35% versus 24%, P = 0.044) with previous cardiac surgery (15% versus 8% P = 0.049) and prosthetic valves (50% versus 37%, P = 0.038). Coagulase-negative Staphylococci, enterococci, Gram-negative bacilli, anaerobes, and fungi were more frequent in PE. No differences on age, sex, symptoms, need of surgery, and in-hospital mortality were detected. Polymicrobial endocarditis represents 5.9% of episodes of left-sided endocarditis in our series. Despite relevant demographic and microbiologic differences between PE and ME, short-term outcome is similar. PMID:26656328

  14. A hybrid feature selection algorithm integrating an extreme learning machine for landslide susceptibility modeling of Mt. Woomyeon, South Korea

    NASA Astrophysics Data System (ADS)

    Vasu, Nikhil N.; Lee, Seung-Rae

    2016-06-01

    An ever-increasing trend of extreme rainfall events in South Korea owing to climate change is causing shallow landslides and debris flows in mountains that cover 70% of the total land area of the nation. These catastrophic, gravity-driven processes cost the government several billion KRW (South Korean Won) in losses in addition to fatalities every year. The most common type of landslide observed is the shallow landslide, which occurs at 1-3 m depth, and may mobilize into more catastrophic flow-type landslides. Hence, to predict potential landslide areas, susceptibility maps are developed in a geographical information system (GIS) environment utilizing available morphological, hydrological, geotechnical, and geological data. Landslide susceptibility models were developed using 163 landslide points and an equal number of nonlandslide points in Mt. Woomyeon, Seoul, and 23 landslide conditioning factors. However, because not all of the factors contribute to the determination of the spatial probability for landslide initiation, and a simple filter or wrapper-based approach is not efficient in identifying all of the relevant features, a feedback-loop-based hybrid algorithm was implemented in conjunction with a learning scheme called an extreme learning machine, which is based on a single-layer, feed-forward network. Validation of the constructed susceptibility model was conducted using a testing set of landslide inventory data through a prediction rate curve. The model selected 13 relevant conditioning factors out of the initial 23; and the resulting susceptibility map shows a success rate of 85% and a prediction rate of 89.45%, indicating a good performance, in contrast to the low success and prediction rate of 69.19% and 56.19%, respectively, as obtained using a wrapper technique.

  15. Antimicrobial activity of commonly used antibiotics and DNA fingerprint analysis of Pseudomonas aeruginosa obtained from clinical isolates and unchlorinated drinking water in Korea, 2010.

    PubMed

    Kim, Jung Rae; Lee, Do Kyung; An, Hyang Mi; Kim, Mi Jin; Lee, Si Won; Cha, Min Kyeong; Lee, Kang Oh; Ha, Nam Joo

    2011-08-01

    Pseudomonas aeruginosa exists in various environments, and can cause mild or serious infections resulting in a wide range of symptoms. In this study, we collected bacterial isolates from hospitalized patients and unchlorinated drinking water, in Korea, 2010. The water-borne and clinical isolates were compared using colony morphology, antimicrobial susceptibility testing, and random amplification of polymorphic DNA analysis. We first compared morphological features of the water-borne and clinical isolates. The clearest difference in colony morphology was colony shape; five water-borne isolate colonies (83%) had a smooth, circular morphology, while nine (75%) clinical isolate colonies had a rough, irregular morphology. Minimum inhibitory concentrations analyses were performed to determine antimicrobial resistant patterns; using ceftazidime, gentamicin, tigecycline, chloramphenicol, meropenem, and tobramycin according to Clinical and Laboratory Standard Institute (CLSI, 2009) methodology. All waterborne isolates were not resistant to gentamicin, tobramycin, and meropenem. The clinical isolates were resistant to every antibiotic except chloramphenicol. Genotyping was performed using the repetitive extragenic palindromic polymerase-chain-reaction. The DNA fingerprinting patterns did not reveal genetic similarity between the water-borne and clinical P. aeruginosa isolates. On the contrary, they showed that genetically distinct populations have been established in each of these environments. We have revealed significant morphological, clinical and genetic differences between water-borne and clinical isolates of the same bacterial species.

  16. Clinical features and diagnosis of hairy cell leukemia.

    PubMed

    Quest, Graeme R; Johnston, James B

    2015-12-01

    Significant advances in the diagnosis and treatment of hairy cell leukemia (HCL) have recently been made. Improved distinction of HCL from its mimics though clinical presentations, morphologic and immunophenotypic features, and more recently molecular biology, has highlighted marked differences in treatment response and overall prognosis between these disorders. As our understanding of the unique pathobiology of HCL has grown, exciting new avenues of treatment as well as insight into immune function have been obtained. This review provides an overview of the clinical features and diagnostic attributes of HCL, with contrast to other mature B cell lymphoproliferative disorders with overlapping features. Copyright © 2015 Elsevier Ltd. All rights reserved.

  17. Identifying clinical correlates for suicide among epilepsy patients in South Korea: A case-control study.

    PubMed

    Park, Sung-Jin; Lee, Hochang Benjamin; Ahn, Myung Hee; Park, Subin; Choi, Eun Ju; Lee, Hoon-Jin; Ryu, Han Uk; Kang, Joong-Koo; Hong, Jin Pyo

    2015-12-01

    Suicide is a major cause of premature mortality in patients with epilepsy. We aimed to identify the clinical correlates of suicide in these patients. We conducted a matched, case-control study based on a clinical case registry of epilepsy patients (n = 35,638) treated between January 1994 and December 2011 at an academic tertiary medical center in Seoul, Korea. Each epilepsy patient in the suicide group (n = 74) was matched with three epilepsy patients in the nonsuicide group (n = 222) by age, gender, and approximate time at first treatment. The clinical characteristics of the patients in both groups were then compared. In a univariate analysis, seizure frequency during the year before suicide, use of antiepileptic drug polytherapy, lack of aura before seizure, diagnosis of temporal lobe epilepsy, use of levetiracetam, psychiatric comorbidity, and use of antidepressants were all significantly higher in the suicide group than in the nonsuicide group. Multivariate analysis revealed that a high seizure frequency (odds ratio [OR] 3.3, 95% confidence interval [CI] 1.04-10.2), a lack of aura before seizure (OR 4.0, 95% CI 1.7-9.3), temporal lobe epilepsy (OR 3.7, 95% CI 1.6-8.6), and use of levetiracetam (OR 7.6, 95% CI 1.1-53.7) and antidepressants (OR 7.2, 95% CI 1.5-34.1) were all associated with a higher probability of suicide. Patients with temporal lobe epilepsy who experience seizures weekly or more frequently, experience a lack of aura, use levetiracetam, or take antidepressants are all at a higher risk of suicide and should be monitored closely. Wiley Periodicals, Inc. © 2015 International League Against Epilepsy.

  18. Seroepidemiology of Toxocariasis and Its Clinical Implications in Gwangju and Jeonnam-province, Korea.

    PubMed

    Won, Eun Jeong; Kim, Jin; Shin, Myung-Geun; Shin, Jong Hee; Suh, Soon Pal; Ryang, Dong Wook

    2015-07-01

    We investigated the seroepidemiological, clinical, and laboratory characteristics of patients suspected to have toxocariasis in Gwangju and Jeonnam-province, Korea. In total, 228 specimens were analyzed for anti-Toxocara canis IgG at two university hospitals from 2010 to 2012. The overall seropositive rate was 67.1%, and the seropositive rates among the eosinophilic and non-eosinophilic groups were 76.1% (105/138) and 53.3% (48/90), respectively. Risk factors for eosinophilia and toxocariasis were male sex (odds ratios [OR]=2.632 and 3.477, respectively) and a history of ingesting raw meat (OR=2.884 and 3.274, respectively), especially raw cow liver (OR=2.089 and 10.038, respectively). T. canis seropositivity (OR=5.807, P=0.004) and a history of consuming raw cow liver (OR=2.766, P=0.052) were risk factors for organ involvement. The anti-T. canis IgG level showed weakly positive correlations with eosinophil counts (r=0.234, P<0.001) and the duration of eosinophilia (r=0.155, P=0.019). Although limited to the regions of Gwangju and Jeonnam-province, this study supports the opinion that toxocariasis is a reasonable focus as a cause of eosinophilia and that it is also associated with organ involvement.

  19. Clinical characteristics of constrictive pericarditis diagnosed by echo-Doppler technique in Korea.

    PubMed Central

    Yang, H. S.; Song, J. K.; Song, J. M.; Kang, D. H.; Lee, C. W.; Nam, G. B.; Choi, K. J.; Kim, Y. H.; Hong, M. K.; Kim, J. J.; Park, S. W.; Park, S. J.; Song, H.; Lee, J. W.; Song, M. G.

    2001-01-01

    A retrospective analysis of clinical data of 71 patients with constrictive pericarditis (CP) diagnosed by echo-Doppler technique (mean age, 49+/-17) was done. In 27 patients (38%), the etiology was unknown, and the three most frequent identifiable causes were tuberculosis (23/71, 32%), cardiac surgery (8/71, 11%), and mediastinal irradiation (6/71, 9%). Pericardiectomy was performed in 35 patients (49%) with a surgical mortality of 6% (2/35), and 11 patients (15%, 11/ 71) showed complete resolution of constrictive physiology with medical treatment. Patients with transient CP were characterized by absence of pericardial calcification, shorter symptom duration, and higher incidence of fever, weight loss, and tuberculosis. The 5-yr survival rates of patients with transient CP and those undergoing pericardiectomy were 100% and 85+/-6%, respectively, which were significantly higher than that of patients without undergoing pericardiectomy (33+/-17%, p=0.0083). Mediastinal irradiation, higher functional class, low voltage in ECG, low serum albumin, and old age were the independent variables associated with a higher mortality. Tuberculosis is still the most important etiology of CP in Korea, and not infrequently, it may cause transient CP. Early diagnosis and decision-making using follow-up echocardiography are crucial to improve the prognosis of patients with CP. PMID:11641523

  20. Clinical Features of Community-Acquired Helicobacter cinaedi Bacteremia.

    PubMed

    Uwamino, Yoshifumi; Muranaka, Kiyoharu; Hase, Ryota; Otsuka, Yoshihito; Hosokawa, Naoto

    2016-02-01

    There are growing numbers of reports concerning the clinical and pathological features of Helicobacter cinaedi (H. cinaedi) bacteremia; however, few reports have discussed the features of this condition in healthy individuals. A retrospective observational study was conducted at a Japanese tertiary care hospital to assess the clinical features of community-acquired H. cinaedi. All patients in whom H. cinaedi was isolated between January 2009 and March 2014 were identified from the hospital database. Of the 28 patients included in the study, 12 had community-acquired H. cinaedi bacteremia. The most common clinical feature was cellulitis (n = 17). However, nearly half of the patients with healthcare-associated or nosocomial-associated bacteremia displayed no symptoms with the exception of fever. Most patients were successfully treated with a 14-day regime of third-generation cephalosporins or tetracycline. Our results show that H. cinaedi infections are quite common in immunocompetent community-dwelling individuals. © 2015 John Wiley & Sons Ltd.

  1. Clustering clinical trials with similar eligibility criteria features.

    PubMed

    Hao, Tianyong; Rusanov, Alexander; Boland, Mary Regina; Weng, Chunhua

    2014-12-01

    To automatically identify and cluster clinical trials with similar eligibility features. Using the public repository ClinicalTrials.gov as the data source, we extracted semantic features from the eligibility criteria text of all clinical trials and constructed a trial-feature matrix. We calculated the pairwise similarities for all clinical trials based on their eligibility features. For all trials, by selecting one trial as the center each time, we identified trials whose similarities to the central trial were greater than or equal to a predefined threshold and constructed center-based clusters. Then we identified unique trial sets with distinctive trial membership compositions from center-based clusters by disregarding their structural information. From the 145,745 clinical trials on ClinicalTrials.gov, we extracted 5,508,491 semantic features. Of these, 459,936 were unique and 160,951 were shared by at least one pair of trials. Crowdsourcing the cluster evaluation using Amazon Mechanical Turk (MTurk), we identified the optimal similarity threshold, 0.9. Using this threshold, we generated 8806 center-based clusters. Evaluation of a sample of the clusters by MTurk resulted in a mean score 4.331±0.796 on a scale of 1-5 (5 indicating "strongly agree that the trials in the cluster are similar"). We contribute an automated approach to clustering clinical trials with similar eligibility features. This approach can be potentially useful for investigating knowledge reuse patterns in clinical trial eligibility criteria designs and for improving clinical trial recruitment. We also contribute an effective crowdsourcing method for evaluating informatics interventions. Copyright © 2014 Elsevier Inc. All rights reserved.

  2. Pediatric Clinical Trials Conducted in South Korea from 2006 to 2015: An Analysis of the South Korean Clinical Research Information Service, US ClinicalTrials.gov and European Clinical Trials Registries.

    PubMed

    Choi, Sheung-Nyoung; Lee, Ji-Hyun; Song, In-Kyung; Kim, Eun-Hee; Kim, Jin-Tae; Kim, Hee-Soo

    2017-08-02

    The status of pediatric clinical trials performed in South Korea in the last decade, including clinical trials of drugs with unapproved indications for children, has not been previously examined. The aim was to provide information regarding the current state of pediatric clinical trials and create a basis for future trials performed in South Korea by reviewing three databases of clinical trials registrations. We searched for pediatric clinical studies (participants <18 years old) conducted in South Korea between 2006 and 2015 registered on the Clinical Research Information Service (CRIS), ClinicalTrials.gov, and the European Clinical Trials Registry (EuCTR). Additionally, we reviewed whether unapproved indications were involved in each trial by comparing the trials with a list of authorized trials provided by the Ministry of Food and Drug Safety (MFDS). The primary and secondary outcomes were to determine the change in number of pediatric clinical trials with unapproved indications over time and to assess the status of unauthorized pediatric clinical trials from the MFDS and the publication of articles after these clinical trials, respectively. We identified 342 clinical studies registered in the CRIS (n = 81), ClinicalTrials.gov (n = 225), and EuCTR (n = 36), of which 306 were reviewed after excluding duplicate registrations. Among them, 181 studies were interventional trials dealing with drugs and biological agents, of which 129 (71.3%) involved unapproved drugs. Of these 129 trials, 107 (82.9%) were authorized by the MFDS. Pediatric clinical trials in South Korea aiming to establish the safety and efficacy of drugs in children are increasing; however, non-MFDS-authorized studies remain an issue.

  3. Visual Aggregate Analysis of Eligibility Features of Clinical Trials

    PubMed Central

    He, Zhe; Carini, Simona; Sim, Ida; Weng, Chunhua

    2015-01-01

    Objective To develop a method for profiling the collective populations targeted for recruitment by multiple clinical studies addressing the same medical condition using one eligibility feature each time. Methods Using a previously published database COMPACT as the backend, we designed a scalable method for visual aggregate analysis of clinical trial eligibility features. This method consists of four modules for eligibility feature frequency analysis, query builder, distribution analysis, and visualization, respectively. This method is capable of analyzing (1) frequently used qualitative and quantitative features for recruiting subjects for a selected medical condition, (2) distribution of study enrollment on consecutive value points or value intervals of each quantitative feature, and (3) distribution of studies on the boundary values, permissible value ranges, and value range widths of each feature. All analysis results were visualized using Google Charts API. Five recruited potential users assessed the usefulness of this method for identifying common patterns in any selected eligibility feature for clinical trial participant selection. Results We implemented this method as a Web-based analytical system called VITTA (Visual Analysis Tool of Clinical Study Target Populations). We illustrated the functionality of VITTA using two sample queries involving quantitative features BMI and HbA1c for conditions “hypertension” and “Type 2 diabetes”, respectively. The recruited potential users rated the user-perceived usefulness of VITTA with an average score of 86.4/100. Conclusions We contributed a novel aggregate analysis method to enable the interrogation of common patterns in quantitative eligibility criteria and the collective target populations of multiple related clinical studies. A larger-scale study is warranted to formally assess the usefulness of VITTA among clinical investigators and sponsors in various therapeutic areas. PMID:25615940

  4. Psoriasis: Epidemiology, clinical features, co-morbidities, and clinical scoring

    PubMed Central

    Dogra, Sunil; Mahajan, Rahul

    2016-01-01

    On the basis of current evidence derived from hospital-based studies, mostly from North India, the prevalence of psoriasis in adults varies from 0.44 to 2.8%, with a much lower prevalence in children. The peak age at onset in adults is in the third and fourth decade of life, with a slight male preponderance. It is recommended that population-based large epidemiologic studies should be undertaken in different parts of the country for estimating the correct prevalence of psoriasis in general population. Chronic plaque-type psoriasis is the most common morphologic presentation of psoriasis, accounting for more than 90% of all cases. Other morphologic variants that deserve special mention include palmoplantar psoriasis, pustular psoriasis, and recalcitrant psoriasis. For epidemiologic purposes, psoriasis can be classified into early and late onset psoriasis. Psoriasis can be classified on the basis of morphology and extent of involvement into localized and widespread disease. For the purpose of clinical trials, psoriasis may be classified as mild psoriasis, moderate psoriasis, and severe psoriasis. The literature shows that there is a significant risk of psoriatic arthritis (7–48%) in patients with plaque-type psoriasis. Hence, it is recommended to evaluate for its presence by detailed history taking and clinical examination, and if necessary, by appropriate radiological investigations. Evidence on the association between plaque-type psoriasis and cardiovascular disease risk factors and ischemic heart disease isinconsistent. On the basis ofavailable evidence, it is prudent to proactively look for metabolic syndrome, dyslipidemia, and obesity, especially in patientswith severe psoriasis (Level 1+ evidence based on systematic reviews and meta-analysis). Based on the current evidence, the psoriasis area severity index appears to be the most valid and reproducible clinical severity score in the management of adult patients with plaque-type psoriasis. PMID:27990381

  5. Clinical characteristics of patients with serrated polyposis syndrome in Korea: comparison with Western patients.

    PubMed

    Kim, Eun Ran; Jeon, Jaryong; Lee, Jin Hee; Lee, Yoon Jung; Hong, Sung Noh; Chang, Dong Kyung; Kim, Young-Ho

    2017-07-01

    Serrated polyposis syndrome (SPS) has been shown to increase the risk of colorectal cancer (CRC). However, little is known about the characteristics of Asian patients with SPS. This study aimed to identify the clinicopathological features and risk of CRC in Korean patients with SPS as well as the differences between Korean and Western patients based on a literature review. This retrospective study included 30 patients with SPS as defined by World Health Organization classification treated at Samsung Medical Center, Korea, between March 1999 and May 2011. Twenty patients (67%) were male. The median patient age at diagnosis was 56 years (range, 39-76 years). A total of 702 polyps were identified during a median follow-up of 43 months (range, 0-149 months). Serrated polyps were noted more frequently in the distal colon (298/702, 55%). However, large serrated polyps and serrated adenomas were mainly distributed throughout the proximal colon (75% vs. 25% and 81% vs. 19%, respectively); 73.3% had synchronous adenomatous polyps. The incidence of CRC was 10% (3/30 patients), but no interval CRC was detected. A total of 87% of the patients underwent esophagogastroduodenoscopy and 19.2% had significant lesions. The phenotype of SPS in Korean patients is different from that of Western patients. In Korean patients, SPS is more common in men, there were fewer total numbers of serrated adenoma/polyps, and the incidence of CRC was lower than that in Western patients. Korean patients tend to more frequently have abnormal gastric lesions. However, the prevalence of synchronous adenomatous polyps is high in both Western and Korean patients.

  6. Variability of clinical features in attacks of migraine with aura.

    PubMed

    Hansen, Jakob M; Goadsby, Peter J; Charles, Andrew C

    2016-03-01

    There is significant variability in the clinical presentation of migraine, both among patients, and between attacks in an individual patient. We examined clinical features of migraine with aura in a large group of patients enrolled in a clinical trial, and compared retrospective migraine attack characteristics reported upon enrollment in the trial with those recorded prospectively in the trial. Patients with migraine (n = 267) with typical visual aura in more than 30% of their attacks were enrolled from 16 centers for a clinical trial. Upon enrollment, patients provided a detailed retrospective description of the clinical features of their attacks of migraine. During the trial, clinical symptoms in migraine attacks starting with aura were recorded prospectively in 861 attacks. Retrospectively reported visual aura symptoms were variable and often overlapping; the most common symptoms were dots or flashing lights, wavy or jagged lines, blind spots, and tunnel vision. Multiple patients reported more than one visual phenomenon. Approximately half of the patients reported nonvisual aura symptoms, the most common were numbness and tingling, followed by difficulty in recalling or speaking words. A significant percentage of patients also reported a change in olfaction. There were several inconsistencies between the features of prospectively recorded and retrospectively reported attacks. Headache, nausea, photophobia, and phonophobia were all less common in prospectively recorded attacks as compared with retrospective reporting. Nausea was prospectively recorded in only 51% of attacks and mostly with mild intensity. The occurrence and severity of nausea was reduced with advancing patient age. Phonophobia was not consistently recorded in conjunction with photophobia. These findings are consistent with variable involvement of different brain regions during a migraine attack. The variable occurrence of nausea, and phonophobia in conjunction with photophobia, both defining

  7. Oral candidosis. Clinical, historical, and therapeutic features of 100 cases.

    PubMed

    Fotos, P G; Vincent, S D; Hellstein, J W

    1992-07-01

    Oral candidosis is an increasingly important disease that affects a significant percentage of the population. Traditionally known as an opportunistic pathogen, the broader clinical scope of oropharyngeal candidal infections is now being recognized. The clinical and historical features of 100 patients referred for diagnosis and management of candidosis have been reviewed. The age, gender, chief complaint, medical history, medications, and clinical findings have been noted. A wide range of clinical signs and symptoms, and the rationale behind the topical and systemic antifungal therapies provided to this patient population, are discussed.

  8. Characterization and Antimicrobial Resistance of Salmonella Typhimurium Isolates from Clinically Diseased Pigs in Korea.

    PubMed

    Oh, Sang-Ik; Kim, Jong Wan; Chae, Myeongju; Jung, Ji-A; So, Byungjae; Kim, Bumseok; Kim, Ha-Young

    2016-11-01

    This study investigated the prevalence of Salmonella enterica serovar and antimicrobial resistance in Salmonella Typhimurium isolates from clinically diseased pigs collected from 2008 to 2014 in Korea. Isolates were also characterized according to the presence of antimicrobial resistance genes and pulsed-field gel electrophoresis patterns. Among 94 Salmonella isolates, 81 (86.2%) were identified as being of the Salmonella Typhimurium serotype, followed by Salmonella Derby (6 of 94, 6.4%), Salmonella 4,[5],12:i:- (4 of 94, 4.3%), Salmonella Enteritidis (2 of 94, 2.1%), and Salmonella Brandenburg (1 of 94, 1.1%). The majority of Salmonella Typhimurium isolates were resistant to tetracycline (92.6%), followed by streptomycin (88.9%) and ampicillin (80.2%). Overall, 96.3% of Salmonella Typhimurium isolates showed multidrug-resistant phenotypes and commonly harbored the resistance genes blaTEM (64.9%), flo (32.8%), aadA (55.3%), strA (58.5%), strB (58.5%), sulII (53.2%), and tetA (61.7%). The pulsed-field gel electrophoresis analysis of 45 Salmonella Typhimurium isolates from individual farms revealed 27 distinct patterns that formed one major and two minor clusters in the dendrogram analysis, suggesting that most of the isolates (91.1%) from diseased pigs were genetically related. These findings can assist veterinarians in the selection of appropriate antimicrobial agents to combat Salmonella Typhimurium infections in pigs. Furthermore, they highlight the importance of continuous surveillance of antimicrobial resistance and genetic status in Salmonella Typhimurium for the detection of emerging resistance trends.

  9. Clinical Characteristics of Severe Japanese Encephalitis: A Case Series from South Korea.

    PubMed

    Sunwoo, Jun-Sang; Lee, Soon-Tae; Jung, Keun-Hwa; Park, Kyung-Il; Moon, Jangsup; Jung, Ki-Young; Kim, Manho; Lee, Sang Kun; Chu, Kon

    2017-08-01

    Japanese encephalitis (JE) virus is a major cause of devastating viral encephalitis, especially in Asia. Although a successful vaccination program led to its near-elimination over three decades in South Korea, the incidence of JE has increased since 2010. The present study investigated the clinical manifestations, laboratory findings, and factors affecting neurological outcomes of reemerging JE. We retrospectively reviewed medical records of laboratory-confirmed JE patients who presented with acute encephalitis syndrome at three tertiary hospitals between 2010 and 2015. A total of 17 patients with JE were identified. Their median age was 51 years, and 10 (58.5%) were men. The most common symptoms and signs were fever (94.1%), altered consciousness (94.1%), and headache (80.2%). Hyporeflexia (47.1%), seizures (35.2%), abnormal brainstem reflex (23.5%), and flaccid weakness (17.6%) were also noted. Brain imaging revealed thalamic lesions in all patients, with the hippocampus, midbrain, basal ganglia, and cerebral cortex affected to varying degrees. Sixteen patients (94.1%) required management in the intensive care unit with mechanical ventilation due to neurological deterioration. At the time of discharge, 11 (64.7%) had poor recovery, defined as Glasgow coma scale scores of less than 8, and remained ventilator dependent. Comparison between the two outcome groups indicated that midbrain involvement (P = 0.028) and rapid deterioration (P = 0.005) were associated with severe neurological sequelae. Given that JE is a vaccine-preventable disease, vaccination for adults should be considered in response to the reemergence of JE.

  10. The clinical and genetic features of Huntington disease.

    PubMed

    Sturrock, Aaron; Leavitt, Blair R

    2010-12-01

    Huntington disease (HD) is a dominantly inherited neurodegenerative disorder that usually presents in adulthood with characteristic motor and cognitive features and with variable and diverse psychiatric disturbances. Following the discovery of the causative defect in the HTT gene in 1993, great advances in understanding the pathogenesis of HD have been made, yet no effective disease-modifying therapy has been identified. In this new era of HD research, we have seen the emergence of a number of large clinical trials, the systematic search for novel biomarkers and the recent initiation of the first pre-manifest HD clinical studies. In this review, we seek to provide an overview of the clinical and genetic features of HD together with a summary of clinical research at this time.

  11. Basic clinical and laboratory features of filoviral hemorrhagic fever.

    PubMed

    Kortepeter, Mark G; Bausch, Daniel G; Bray, Mike

    2011-11-01

    The filoviruses Marburg and Ebola cause severe hemorrhagic fever (HF) in humans. Beginning with the 1967 Marburg outbreak, 30 epidemics, isolated cases, and accidental laboratory infections have been described in the medical literature. We reviewed those reports to determine the basic clinical and laboratory features of filoviral HF. The most detailed information was found in descriptions of patients treated in industrialized countries; except for the 2000 outbreak of Ebola Sudan HF in Uganda, reports of epidemics in central Africa provided little controlled or objective clinical data. Other than the case fatality rate, there were no clear differences in the features of the various filovirus infections. This compilation will be of value to medical workers responding to epidemics and to investigators attempting to develop animal models of filoviral HF. By identifying key unanswered questions and gaps in clinical data, it will help guide clinical research in future outbreaks.

  12. Update on Clinical Features and Brain Abnormalities in Neurogenetics Syndromes

    ERIC Educational Resources Information Center

    Jackowski, Andrea Parolin; Laureano, Maura Regina; Del'Aquilla, Marco Antonio; de Moura, Luciana Monteiro; Assuncao, Idaiane; Silva, Ivaldo; Schwartzman, Jose Salomao

    2011-01-01

    Neuroimaging methods represent a critical tool in efforts to join the study of the neurobiology of genes with the neurobiology of behaviour, and to understand the neurodevelopmental pathways that give rise to cognitive and behavioural impairments. This article reviews the clinical features and highlights studies with a focus on the relevant…

  13. Update on Clinical Features and Brain Abnormalities in Neurogenetics Syndromes

    ERIC Educational Resources Information Center

    Jackowski, Andrea Parolin; Laureano, Maura Regina; Del'Aquilla, Marco Antonio; de Moura, Luciana Monteiro; Assuncao, Idaiane; Silva, Ivaldo; Schwartzman, Jose Salomao

    2011-01-01

    Neuroimaging methods represent a critical tool in efforts to join the study of the neurobiology of genes with the neurobiology of behaviour, and to understand the neurodevelopmental pathways that give rise to cognitive and behavioural impairments. This article reviews the clinical features and highlights studies with a focus on the relevant…

  14. Multiple sclerosis with clinical and radiological features of cerebral tumour

    PubMed Central

    Sagar, HJ; Warlow, CP; Sheldon, PWE; Esiri, MM

    1982-01-01

    Three cases of multiple sclerosis, all confirmed pathologically, are described in whom both the unusual clinical features and the CT scan appearances suggested cerebral tumours. The failure of mass effect reliably to differentiate plaques and tumours on a CT scan is stressed and the literature relating to CT scanning in multiple sclerosis is reviewed. Images PMID:7131013

  15. Repetitive Behaviors in Autism: Relationships with Associated Clinical Features

    ERIC Educational Resources Information Center

    Gabriels, Robin L.; Cuccaro, Michael L.; Hill, Dina E.; Ivers, Bonnie J.; Goldson, Edward

    2005-01-01

    Relationships between repetitive behaviors (RBs) and associated clinical features (i.e., cognitive and adaptive functioning levels, sleep problems, medication use, and other behavioral problems) were examined in two groups (High nonverbal IQ greater than or equal to 97 versus Low nonverbal IQ less than or equal to 56) of children with autism…

  16. The clinical features of ankle syndesmosis injuries: a general review.

    PubMed

    Kellett, John J

    2011-11-01

    To review the research conclusions relating to clinical aspects of syndesmosis, the incidence and prognosis of syndesmosis injuries, and the effectiveness of the history and clinical examination to reliably diagnose ankle syndesmosis injury. Google Scholar search: Syndesmosis paired with incidence, prognosis, history, and examination in turn. There was no time limit for the search. Articles were selected by reading titles, abstracts, and the full article, if indicated, seeking original articles determining these clinical aspects of syndesmosis injuries. Further articles were derived from the references of the primary articles. The prognosis for isolated syndesmosis injuries, including the time to functional recovery, is unknown. The incidence of acute syndesmosis injury in moderate to severe ankle injuries requiring imaging is of the order of 5%. Historical features and special clinical tests of syndesmosis injury have not been proven reliable by clinical studies using evidence-based diagnostic criteria. Acute local tenderness of the anterior inferior tibiofibular ligament will indicate significant syndesmosis injury in only approximately half of nonspecific ankle injuries. There is limited, evidence-based, standard, published literature from which to draw conclusions regarding the validity or reliability of various clinical special tests for syndesmosis injury. Literature assessing the incidence, prognosis, and clinical features is generally not based on definitively confirmed syndesmosis injuries, which is a critical aspect of evidence-based medicine before valid conclusions can be drawn.

  17. Adverse Events Associated with Metal Contamination of Traditional Chinese Medicines in Korea: A Clinical Review

    PubMed Central

    Kim, Hyunah; Hawes, Emily M.

    2014-01-01

    This study was performed to review studies carried out in Korea reporting toxic reactions to traditional Chinese medicines (TCMs) as a result of heavy metal contamination. PubMed (1966-August 2013) and International Pharmaceutical Abstracts (1965-August 2013) were searched using the medical subject heading terms of "Medicine, Chinese Traditional," "Medicine, Korean Traditional," "Medicine, Traditional," "Metals, Heavy," and "Drug Contamination". For Korean literature, Korea Med (http://www.koreamed.org), the Korean Medical Database (http://kmbase.medric.or.kr), National Discovery for Science Leaders (www.ndsl.kr), Research Information Sharing Service (http://www.riss.kr), and Google Scholar were searched using the terms "Chinese medicine," "Korean medicine," "herbal medicine," and "metallic contamination" in Korean. Bibliographies of case reports and case series, identified using secondary resources, were also utilized. Only literature describing cases or studies performed in Korea were included. Case reports identified clear issues with heavy metal, particularly lead, contamination of TCMs utilized in Korea. No international standardization guidelines for processing, manufacturing and marketing of herbal products exist. Unacceptably high levels of toxic metals can be present in TCM preparations. Health care providers and patients should be educated on the potential risks associated with TCMs. International advocacy for stricter standardization procedures for production of TCMs is warranted. PMID:25048473

  18. Adverse events associated with metal contamination of traditional chinese medicines in Korea: a clinical review.

    PubMed

    Kim, Hyunah; Hughes, Peter J; Hawes, Emily M

    2014-09-01

    This study was performed to review studies carried out in Korea reporting toxic reactions to traditional Chinese medicines (TCMs) as a result of heavy metal contamination. PubMed (1966-August 2013) and International Pharmaceutical Abstracts (1965-August 2013) were searched using the medical subject heading terms of "Medicine, Chinese Traditional," "Medicine, Korean Traditional," "Medicine, Traditional," "Metals, Heavy," and "Drug Contamination". For Korean literature, Korea Med (http://www.koreamed.org), the Korean Medical Database (http://kmbase.medric.or.kr), National Discovery for Science Leaders (www.ndsl.kr), Research Information Sharing Service (http://www.riss.kr), and Google Scholar were searched using the terms "Chinese medicine," "Korean medicine," "herbal medicine," and "metallic contamination" in Korean. Bibliographies of case reports and case series, identified using secondary resources, were also utilized. Only literature describing cases or studies performed in Korea were included. Case reports identified clear issues with heavy metal, particularly lead, contamination of TCMs utilized in Korea. No international standardization guidelines for processing, manufacturing and marketing of herbal products exist. Unacceptably high levels of toxic metals can be present in TCM preparations. Health care providers and patients should be educated on the potential risks associated with TCMs. International advocacy for stricter standardization procedures for production of TCMs is warranted.

  19. [Clinical features of pediatric multiple sclerosis: epidemiology and treatment].

    PubMed

    Torisu, Hiroyuki; Hara, Toshiro

    2014-11-01

    Multiple sclerosis (MS) in children is essentially not different from MS in adults; however, in children, it is sometimes difficult to distinguish MS from other demyelinating diseases. The main reason for this is that acute encephalitis/encephalopathy associated with infectious diseases, especially acute disseminated encephalomyelitis (ADEM), often causes demyelinating events in the central nervous system in childhood, and that the demyelinating episodes of MS in younger children clinically resemble ADEM events. Therefore, a number of studies on pediatric demyelinating diseases have been conducted to elucidate the clinical features of pediatric MS. In this article, the clinical features of pediatric MS in Japan were reviewed on the basis of the results of a nationwide survey as well as those in other countries.

  20. Clinical features distinguishing grief from depressive episodes: A qualitative analysis.

    PubMed

    Parker, Gordon; McCraw, Stacey; Paterson, Amelia

    2015-05-01

    The independence or interdependence of grief and major depression has been keenly argued in relation to recent DSM definitions and encouraged the current study. We report a phenomenological study seeking to identify the experiential and phenomenological differences between depression and grief as judged qualitatively by those who had experienced clinical (n=125) or non-clinical depressive states (n=28). Analyses involving the whole sample indicated that, in contrast to grief, depression involved feelings of hopelessness and helplessness, being endless and was associated with a lack of control, having an internal self-focus impacting on self-esteem, being more severe and stressful, being marked by physical symptoms and often lacking a justifiable cause. Grief was distinguished from depression by the individual viewing their experience as natural and to be expected, a consequence of a loss, and with an external focus (i.e. the loss of the other). Some identified differences may have reflected the impact of depressive "type" (e.g. melancholia) rather than depression per se, and argue for a two-tiered model differentiating normative depressive and grief states at their base level and then "clinical" depressive and 'pathological' grief states by their associated clinical features. Comparative analyses between the clinical and non-clinical groups were limited by the latter sub-set being few in number. The provision of definitions may have shaped subjects׳ nominated differentiating features. The study identified a distinct number of phenomenological and clinical differences between grief and depression and few shared features, but more importantly, argued for the development of a two-tiered model defining both base states and clinical expressions. Copyright © 2015 Elsevier B.V. All rights reserved.

  1. A wide variation of the quality of colonoscopy reporting system in the real clinical practice in southeastern area of Korea

    PubMed Central

    Lee, Jung Min; Kang, Yu Jin; Lee, Yoo Jin; Park, Kyung Sik; Cho, Kwang Bum; Jeon, Seong Woo; Jung, Min Kyu; Lee, Hyun Seok; Kim, Eun Young; Jung, Jin Tae; Jang, Byung Ik; Kim, Kyeong Ok; Chung, Yun Jin; Yang, Chang Hun

    2016-01-01

    Background/Aims Establishment of a colonoscopy reporting system is a prerequisite to determining and improving quality. This study aimed to investigate colonoscopists' opinions and the actual situation of a colonoscopy reporting system in a clinical practice in southeastern area of Korea and to assess the factors predictive of an inadequate reporting system. Methods Physicians who performed colonoscopies in the Daegu-Gyeongbuk province of Korea and were registered with the Korean Society of Gastrointestinal Endoscopy (KSGE) were interviewed via mail about colonoscopy reporting systems using a standardized questionnaire. Results Of 181 endoscopists invited to participate, 125 responded to the questionnaires (response rate, 69%). Most responders were internists (105/125, 84%) and worked in primary clinics (88/125, 70.4%). Seventy-one specialists (56.8%) held board certifications for endoscopy from the KSGE. A median of 20 colonoscopies (interquartile range, 10–47) was performed per month. Although 88.8% of responders agreed that a colonoscopy reporting system is necessary, only 18.4% (23/125) had achieved the optimal reporting system level recommended by the Quality Assurance Task Group of the National Colorectal Cancer Roundtable. One-third of endoscopists replied that they did not use a reporting document for the main reasons of "too busy" and "inconvenience." Non-endoscopy specialists and primary care centers were independent predictive factors for failure to use a colonoscopy reporting system. Conclusions The quality of colonoscopy reporting systems varies widely and is considerably suboptimal in actual clinical practice settings in southeastern Korea, indicating considerable room for quality improvements in this field. PMID:27799886

  2. Chronological Trends in Clinical and Urinary Metabolic Features over 20 Years in Korean Urolithiasis Patients.

    PubMed

    Kang, Ho Won; Seo, Sung Pil; Ha, Yun Sok; Kim, Won Tae; Kim, Yong June; Yun, Seok Joong; Kim, Wun Jae; Lee, Sang Cheol

    2017-09-01

    Urolithiasis is common and is becoming more prevalent worldwide. This study assessed the chronological trends in clinical and urinary metabolic features over 20 years in Korean urolithiasis patients. We performed a retrospective analysis of 4,076 patients treated at our clinic from 1996 to 2015. Urinary metabolic data and stone analysis data were available for 1,421 and 723 patients (34.9% and 17.7%), respectively. Patients were categorized into 4 groups according to the date of initial diagnosis: group 1 (1996-2000, n = 897), group 2 (2001-2005, n = 1,018), group 3 (2006-2010, n = 1,043), and group 4 (2011-2015, n = 1,118). Incidental detection of uric acid renal stones has become more prevalent in the past 10 years, accompanied by an increase in body mass index and age at diagnosis. Similarly, the prevalence of diabetes mellitus and of hypertension increased from one group to the next throughout the study period. Levels of 24-hour urinary excretion of sodium, calcium, uric acid, and oxalate have decreased significantly over the study period. The incidence of urinary metabolic abnormalities also showed an identical tendency. The proportion of stones composed of uric acid increased over the study period. In conclusion, incidental detection of uric acid renal stones has become more prevalent in Korea in the past 20 years. Urinary excretion of lithogenic constituents and the incidence of urinary metabolic abnormalities have decreased significantly over this period. © 2017 The Korean Academy of Medical Sciences.

  3. Clinical and diagnostic features and treatment of giardiasis.

    PubMed

    Begaydorova, R Kh; Nasakaeva, G E; Tabagari, S I; Iukhnevich, E A; Alshinbekova, G K

    2014-11-01

    Giardia is the most common causes of protozoan diarrhea that lead to significant morbidity and mortality worldwide. Giardiasis can be cause of disturbance of host immune response. The treatment of Giardiasis is unsuccessful in some cases. The purpose of this study was to determine the clinical features and the content of secretory immunoglobulin A (sIgA) among adults and to evaluate efficiency of new plant preparation "Sausalin". The clinical studies were conducted in Karaganda Regional Infection Hospital (Kazakhstan). 250 patients with giardiasis were randomly assigned to receive sausalin at a dose 720 mg/day or ornidazole at 1500 mg/day. Clinical symptoms of giardisis and efficiency of treatment were evaluated. Protozoal clearance rate and clinical symptoms were assessed. Stool samples were collected from 40 patients and examined the content of sIgA. Our study found the prevalence of abdominal pain, dyspeptic syndrome and the symptoms of intoxication in patients with giardiasis. The increase the level of sIgA was detected, especially in females (88 mg/l). Sausalin was more effectiveness than ornidazole. After the treatment, the clearance rate of giardia (85.71% vs. 42.19%; P<0.05) and the clinical efficacy were significantly higher in the sausalin-treated group than in the ornidazole-treated group. The features of clinic manifestations of giardiasis were identified in population of Kazakhstan. Our data suggest the higher level of sIgA was significantly associated with features of clinic manifestations that the participant had. Treatment with sausalin was more effective than treatment with ornidazole. Further research is needed to explain the existence relationship between Giardia infection and host immune response.

  4. Introduction and Administration of the Clinical Skill Test of the Medical Licensing Examination, Republic of Korea (2009)

    PubMed Central

    2010-01-01

    The first trial of the clinical skill test as part of the Korean Medical Licensing Examination was done from September 23 to December 1, 2009, in the clinical skill test center located in the National Health Personnel Licensing Examination Board (NHPLEB) building, Seoul. Korea is the first country to introduce the clinical skill test as part of the medical licensing examination in Asia. It is a report on the introduction and administration of the test. The NHPLEB launched researches on the validity of introducing the clinical skill test and on the best implementation methods in 2000. Since 2006, lists of subjects of test items for the clinical skill test has been developed. The test consisted of two types of evaluation, i.e., a clinical performance examination (CPX) with a standardized patient (SP) and objective structured clinical examination (OSCE). The proctor (medical faculty member) and SP rate the examinees' proficiency for the OSCE and CPX respectively. Out of 3,456 applicants, 3,289 examinees (95.2%) passed the test. Out of 167 examinees who failed the clinical skill test, 142 passed the written test. This means that the clinical skill test showed characteristics independent from the written test. This successful implementation of the clinical skill test is going to improve the medical graduates' performance of clinical skills. PMID:21179230

  5. Dementia pugilistica with clinical features of Alzheimer's disease.

    PubMed

    Areza-Fegyveres, Renata; Rosemberg, Sergio; Castro, Rosa Maria R P S; Porto, Claudia Sellitto; Bahia, Valéria Santoro; Caramelli, Paulo; Nitrini, Ricardo

    2007-09-01

    A 61-year-old ex-boxer presented with a three-year history of progressive memory decline. During a seven-year follow-up period, there was a continuous cognitive decline, very similar to that usually observed in Alzheimer's disease. Parkinsonian, pyramidal or cerebellar signs were conspicuously absent. Neuropathological examination revealed the typical features of dementia pugilistica: cavum septi pellucidi with multiple fenestrations, numerous neurofibrillary tangles in the cerebral isocortex and hippocampus (and rare senile plaques). Immunohistochemistry disclosed a high number of tau protein deposits and scarce beta-amyloid staining. This case shows that dementia pugilistica may present with clinical features practically undistinguishable from Alzheimer's disease.

  6. Clinical and dermatoscopic features of porokeratosis palmaris et plantaris

    PubMed Central

    Udare, Satish; Hemmady, Karishma

    2016-01-01

    A dermatoscope is an important tool in a dermatologist's armamentarium as it can eliminate the need for a biopsy in a wide array of conditions. Porokeratosis was described by Mibelli and Respighi in 1893, as a disorder of keratinization which on the basis of distribution patterns was described as five clinical variants that portrayed a coronoid lamella on histopathology. We describe a case of asymptomatic, long-standing palmar and plantar pits, which on dermatoscopy showed features suggestive of porokeratosis, which was later reconfirmed by histopathologic sections. This report depicts diagnostic features of porokeratosis and obviates the need for invasive procedures for its diagnosis. PMID:27559506

  7. An introduction to Chuna manual medicine in Korea: History, insurance coverage, education, and clinical research in Korean literature.

    PubMed

    Park, Tae-Yong; Moon, Tae-Woong; Cho, Dong-Chan; Lee, Jung-Han; Ko, Youn-Seok; Hwang, Eui-Hyung; Heo, Kwang-Ho; Choi, Tae-Young; Shin, Byung-Cheul

    2014-06-01

    The objectives of this study were to summarize the curriculum, history, and clinical researches of Chuna in Korea and to ultimately introduce Chuna to Western medicine. Information about the history and insurance coverage of Chuna was collected from Chuna-related institutions and papers. Data on Chuna education in all 12 Korean medicine (KM) colleges in Korea were reconstructed based on previously published papers. All available randomized controlled trials (RCTs) of Chuna in clinical research were searched using seven Korean databases and six KM journals. As a result, during the modern Chuna era, one of the three periods of Chuna, which also include the traditional Chuna era and the suppressed Chuna era, Chuna developed considerably because of a solid Korean academic system, partial insurance coverage, and the establishment of a Chuna association in Korea. All of the KM colleges offered courses on Chuna-related subjects (CRSs); however, the total number of hours dedicated to lectures on CRSs was insufficient to master Chuna completely. Overall, 17 RCTs were reviewed. Of the 14 RCTs of Chuna in musculoskeletal diseases, six reported Chuna was more effective than a control condition, and another six RCTs proposed Chuna had the same effect as a control condition. One of these 14 RCTs made the comparison impossible because of unreported statistical difference; the last RCT reported Chuna was less effective than a control condition. In addition, three RCTs of Chuna in neurological diseases reported Chuna was superior to a control condition. In conclusion, Chuna was not included in the regular curriculum in KM colleges until the modern Chuna era; Chuna became more popular as the result of it being covered by Korean insurance carriers and after the establishment of a Chuna association. Meanwhile, the currently available evidence is insufficient to characterize the effectiveness of Chuna in musculoskeletal and neurological diseases.

  8. Cryptogenic NORSE: Its distinctive clinical features and response to immunotherapy.

    PubMed

    Iizuka, Takahiro; Kanazawa, Naomi; Kaneko, Juntaro; Tominaga, Naomi; Nonoda, Yutaka; Hara, Atsuko; Onozawa, Yuya; Asari, Hiroki; Hata, Takashi; Kaneko, Junya; Yoshida, Kenji; Sugiura, Yoshihiro; Ugawa, Yoshikazu; Watanabe, Masashi; Tomita, Hitomi; Kosakai, Arifumi; Kaneko, Atsushi; Ishima, Daisuke; Kitamura, Eiji; Nishiyama, Kazutoshi

    2017-11-01

    To report the distinctive clinical features of cryptogenic new-onset refractory status epilepticus (C-NORSE) and the C-NORSE score based on initial clinical assessments. A retrospective study was conducted for 136 patients with clinically suspected autoimmune encephalitis who underwent testing for autoantibodies to neuronal surface antigens between January 1, 2007, and August 31, 2016. Eleven patients with C-NORSE were identified. Their clinical features were compared with those of 32 patients with anti-NMDA receptor encephalitis (NMDARE). The clinical outcome of 11 patients (median age, 27 years; 7 [64%] women) with C-NORSE was evaluated after a median follow-up of 11 months (range, 6-111 months). Status epilepticus was frequently preceded by fever (10/11 [91%]). Brain MRIs showed symmetric T2/fluid-attenuated inversion recovery hyperintensities (8/11 [73%]) and brain atrophy (9/11 [82%]). Only 2 of the 10 treated patients responded to the first-line immunotherapy, and 4 of the 5 patients treated with IV cyclophosphamide responded to the therapy. The long-term outcome was poor in 8 patients (73%). Compared with 32 patients with NMDARE (median age, 27 years; 24 [75%] women), those with C-NORSE had more frequent prodromal fever, status epilepticus, ventilatory support, and symmetric brain MRI abnormalities, had less frequent involuntary movements, absent psychobehavioral symptoms, CSF oligoclonal bands, or tumor association, and had a worse outcome. The C-NORSE score was higher in patients with C-NORSE than those with NMDARE. Patients with C-NORSE have a spectrum of clinical-immunological features different from those with NMDARE. The C-NORSE score may be useful for discrimination between them. Some patients could respond to immunotherapy.

  9. Clinical and Virological Features of Dengue in Vietnamese Infants

    PubMed Central

    Bich Chau, Tran Nguyen; Anders, Katherine L.; Lien, Le Bich; Hung, Nguyen Thanh; Minh Hieu, Lu Thi; Tuan, Nguyen Minh; Thuy, Tran Thi; Phuong, Le Thi; Hong Tham, Nguyen Thi; Lanh, Mai Ngoc; Farrar, Jeremy J.; Whitehead, Stephen S.; Simmons, Cameron P.

    2010-01-01

    Background Infants account for a small proportion of the overall dengue case burden in endemic countries but can be clinically more difficult to manage. The clinical and laboratory features in infants with dengue have not been extensively characterised. Methodology/Principal Findings This prospective, cross-sectional descriptive study of infants hospitalized with dengue was conducted in Vietnam from November 2004 to December 2007. More than two-thirds of 303 infants enrolled on clinical suspicion of dengue had a serologically confirmed dengue virus (DENV) infection. Almost all were primary dengue infections and 80% of the infants developed DHF/DSS. At the time of presentation and during hospitalization, the clinical signs and symptoms in infants with dengue were difficult to distinguish from those with other febrile illnesses, suggesting that in infants early laboratory confirmation could assist appropriate management. Detection of plasma NS1 antigen was found to be a sensitive marker of acute dengue in infants with primary infection, especially in the first few days of illness. Conclusions/Significance Collectively, these results provide a systematic description of the clinical features of dengue in infants and highlight the value of NS1 detection for diagnosis. PMID:20405057

  10. [Clinical features and comorbidities of Asperger syndrome in children].

    PubMed

    Fu, Xiao-Yan; Xie, Xiao-Tian; Mei, Zhu; Cheng, Wen-Hong

    2013-09-01

    To investigate and summarize the clinical features and comorbidities of Asperger syndrome (AS) in children and to provide a theoretical basis for improving the understanding and diagnosis of AS. Inquiry of medical history, physical examination, behavioral observation, psychiatric examination, questionnaire survey, and the Wechsler Intelligence Scale were used to summarize and analyse the clinical data of 95 children with AS, including chief complaint, symptoms, perinatal and familial conditions, family genetic history, and common comorbidities. AS was more common in male children, with hyperactivity, inattention, and social withdrawal as frequent chief complaints. The main clinical manifestations included poor communication skills (95%), restricted interest (82%), repetitive and stereotyped patterns of behavior (77%), semantic comprehension deficit (74%), and indiscipline (68%). Verbal IQ was higher than performance IQ in most patients. The comorbidities of AS included attention deficit hyperactivity disorder (ADHD) (39%), emotional disorder (18%), and schizophrenia (2%); emotional disorder was more common in patients aged 13-16 years, while ADHD was more common in patients aged 7-16 years. Among these patients, 61% had fathers with introverted personality, 43% had mothers with introverted personality, and 19% had a family history of mental illness. AS has specific clinical manifestations. It is essential to know more about the clinical features and comorbidities of AS, which is helpful for early identification and diagnosis of AS.

  11. Confetti-like Sparing: A Diagnostic Clinical Feature of Melasma.

    PubMed

    Wu, Douglas C; Fitzpatrick, Richard E; Goldman, Mitchel P

    2016-02-01

    Diagnostic uncertainty when a patient presents with melasma-like Undings can lead to suboptimal treatment and inaccurate prognostic expectations. In this study, the authors present a unique clinical feature of melasma that they term the "Fitzpatrick macule" and test its Utility in establishing diagnostic certainty. The "Fitzpatrick macule" is a confetti-like macule of regularly pigmented skin located within a larger patch of melasma hyperpigmentation. To test its diagnostic Utility, the authors compared clinical photography of known cases of melasma with common mimickers, such as poikiloderma of Civatte and solar lentiginosis, and determined the positivity rate of the Fitzpatrick macule in each scenario. Their results show that 89.1 percent of clinical photographs of melasma were positive for the presence of Fitzpatrick macules compared to 1.1 percent that were negative. In contrast, 37.5 and 56.3 percent of clinical photographs of poikiloderma of Civatte were positive and negative for Fitzpatrick macules, respectively. Solar lentiginosis showed a 5.6 percent positivity and a 77.8 percent negativity for Fitzpatrick macules. The sensitivity and specificity of Fitzpatrick macules for melasma was 99 and 83 percent, respectively. In summary, the authors report a highly sensitive and specific clinical feature of melasma. In cases of diagnostic uncertainty, the presence of Fitzpatrick macules may aid in establishing a diagnosis of melasma.

  12. Clinical features of trisomy 12 mosaicism-Report and review.

    PubMed

    Hong, Bo; Zunich, Janice; Openshaw, Amanda; M Toydemir, Reha

    2017-03-27

    Trisomy 12 mosaicism is a rare condition. Herein, we report a patient with mosaic trisomy 12 who was conceived by in vitro fertilization. She presented with mild dysmorphic features at birth, including down-slanting palpebral fissures, a depressed and creased nasal bridge, and mild rhizomelic shortening of the limbs. She had age-appropriate development at 6 months of age, but displayed slightly more dysmorphic features than at birth. Chromosome analysis on peripheral blood revealed a normal female karyotype in 50 metaphases. A concurrent genomic microarray analysis showed trisomy 12 in about 25% of the specimen, which was also confirmed by fluorescence in situ hybridization analysis with the CEP12 probe. Our findings further delineate the clinical features in trisomy 12 mosaicism in liveborns and demonstrate the utility of genomic microarray analysis in identification of mosaic aneuploidies.

  13. Acute hepatitis E presenting with clinical feature of autoimmune hepatitis

    PubMed Central

    Patel, Ishan; Companioni, Rafael Ching; Bansal, Raghav; Vyas, Neil; Catalano, Carmine; Aron, Joshua; Walfish, Aaron

    2016-01-01

    A 32-year-old immigrant man presented with new onset jaundice. His past medical history was significant for type 2 diabetes mellitus, hypertension, and hyperlipidemia. His initial laboratory finding and liver biopsy were suggestive of autoimmune hepatitis (AIH). The plan was to start steroids pending negative results for viral serology, but it came back positive for hepatitis E virus. The patient's liver function test and clinical condition improved significantly on conservative management over a period of 1 month. Therefore, we suggest testing for hepatitis E especially in immigrants or recent travelers to endemic areas who presents with clinical features suggestive of AIH. PMID:27987286

  14. Psoriasis: epidemiology, clinical features, and quality of life

    PubMed Central

    Langley, R; Krueger, G; Griffiths, C

    2005-01-01

    Psoriasis is a common chronic, recurrent, immune mediated disease of the skin and joints. It can have a significant negative impact on the physical, emotional, and, psychosocial wellbeing of affected patients. Psoriasis is found worldwide but the prevalence varies among different ethnic groups. It has a strong genetic component but environmental factors such as infections can play an important role in the presentation of disease. There are several clinical cutaneous manifestations of psoriasis but most commonly the disease presents as chronic, symmetrical, erythematous, scaling papules and plaques. The epidemiology, clinical features, and impact on quality of life of psoriasis are reviewed. PMID:15708928

  15. [Genotypic and clinical features of spinal muscular atrophy type 3].

    PubMed

    Wang, Yan-yun; Feng, Shan-wei; Cao, Ji-qing; Yang, Juan; Li, Ya-qin; Li, Jin; Zhang, Cheng

    2012-04-01

    To explore the genotypic and clinical features and laboratory examinations of spinal muscular atrophy type 3 (SMA III). Results of genetic testing and laboratory exams of 18 SMA III patients were collected and analyzed. The average age of onset of patients was 6.1 years, with the course of disease lasting from 13 months to 28 years. All patients became symptomatic with lower extremity muscle weakness. The symptoms gradually aggregated, with proximal lower limb muscle becoming atrophic and proximal upper limb muscle becoming weak. Genetic testing indicated that all subjects possessed homozygous deletions of SMN1 gene. Electromyography (EMG) of 15 subjects indicated neurogenic damage. Whilst younger patients had normal level of creatine kinase (CK), elder patients had higher level of CK, though no linear correlation was found. Full understanding of Clinical, especially the growth features of SMA III, in combination with genetic testing, can facilitate diagnosis and early intervention of the disease.

  16. [Clinical, hormonal and histological features in ovarian stromal hyperthecosis].

    PubMed

    Abuladze, M V; Sharabidze, N G

    2006-10-01

    The aim of our study was to investigate clinical, hormonal and histological features of ovarian stromal hyperthecosis We have studied 104 patients with a histological diagnosis of ovarian stromal hyperthecosis and hyperplasia. Analyzing the results we can conclude that clinical features of ovarian stromal hyperthecosis and hyperplasia with polycystic ovarian disease and without it were identical and characterised by virilization syndrome, disorders of menstrual cycle and reproductive function; metabolic and vegeto-vascular disorders; breast and endometrial hyperplasias, high blood level of testosterone . The histological study of ovarian stromal hyperthecosis has showed that superficial part of ovarian cortex is hypocellular and fibrotic, contains scattered follicles with few granulosa cells, luteinized internal theca cells, hyperplasia of deep cortex and medulla, corpus luteus is absent.

  17. Clinical and Laboratory Features Distinguishing Juvenile Polymyositis and Muscular Dystrophy

    PubMed Central

    MAMYROVA, GULNARA; KATZ, JAMES D.; JONES, ROBERT V.; TARGOFF, IRA N.; LACHENBRUCH, PETER A.; JONES, OLCAY Y.; MILLER, FREDERICK W.; RIDER, LISA G.

    2016-01-01

    Objective To differentiate juvenile polymyositis (PM) and muscular dystrophy, both of which may present with chronic muscle weakness and inflammation. Methods We studied 39 patients with probable or definite juvenile PM and 9 patients with muscular dystrophies who were initially misdiagnosed as having juvenile PM. Differences in demographic, clinical, and laboratory results; outcomes; and treatment responses were evaluated by Fisher’s exact and rank sum tests. Random forests classification analysis and logistic regression were performed to examine significant differences in multivariable models. Results Clinical features and serum muscle enzyme levels were similar between juvenile PM and dystrophy patients, except 89% of dystrophy patients had muscle atrophy compared with 46% of juvenile PM patients. Dystrophy patients had a longer delay to diagnosis (median 12 versus 4 months) and were less frequently hospitalized than juvenile PM patients (22% versus 74%). No dystrophy patients, but 54% of juvenile PM patients, had a myositis autoantibody. Dystrophy patients more frequently had myopathic features on muscle biopsy, including diffuse variation of myofiber size, fiber hypertrophy, and myofiber fibrosis (44–100% versus 8–53%). Juvenile PM patients more frequently had complex repetitive discharges on electromyography and a complete response to treatment with prednisone or other immunosuppressive agents than dystrophy patients (44% versus 0%). Random forests analysis revealed that the most important features in distinguishing juvenile PM from dystrophies were myositis autoantibodies, clinical muscle atrophy, and myofiber size variation on biopsy. Logistic regression confirmed muscle atrophy, myofiber fibrosis, and hospitalization as significant predictors. Conclusion Muscular dystrophy can present similarly to juvenile PM. Selected clinical and laboratory features are helpful in combination in distinguishing these conditions. PMID:23925923

  18. Pineal cyst: a review of clinical and radiological features.

    PubMed

    Choy, Winward; Kim, Won; Spasic, Marko; Voth, Brittany; Yew, Andrew; Yang, Isaac

    2011-07-01

    Pineal cysts (PCs) are benign and often asymptomatic lesions of the pineal region that are typically small and do not change in size over time. PCs appear as small, well circumscribed, unilocular masses that either reside within or completely replace the pineal gland. This article reviews and discusses the characteristic features of PCs-clinical, histological, and identifiable by various imaging modalities-which assist clinicians in narrowing the differential diagnosis for pineal lesions.

  19. [Clinical features of NMO according to brain MRI findings].

    PubMed

    Shimizu, Yuko

    2010-09-01

    Neuromyelitis optica (NMO) is a severe inflammatory, demyelinating disease, and its clinical characteristics include recurrent optic neuritis and longitudinally extensive transverse myelitis. The NMO-immunoglobulin (Ig) G auto-antibody (Ab), which binds to the aquaporin-4 (AQP4) water channel protein, is a marker for NMO. These clinical and immunological features have been used to distinguish NMO from multiple sclerosis (MS). In 1999, Wingerchuk et al. broadened the clinical criteria for diagnosing NMO to include "negative brain magnetic resonance imaging (MRI) at onset." However, after NMO-IgG/AQP4-Ab became a supportive criterion for diagnosing NMO, patients with NMO were frequently found to have symptomatic or asymptomatic brain lesions. In 2006, Pittock et al. reported that asymptomatic brain lesions were common in NMO, and that NMO brain lesions characteristically occurred in the hypothalamus and periventricular areas, which correspond to brain regions with high levels of AQP4 expression. Furthermore, Nakashima et al. detected abnormalities on brain MRI in 71% of NMO-IgG-positive Japanese patients. Patients with NMO have unique brain lesions that are clearly different from the lesions of patients with MS. In patients with NMO, involvement of the dorsal portion of the medulla oblongata causes intractable hiccups and nausea. Some studies described a hypothalamic lesion, and hypothalamic dysfunction could cause symptomatic hypersomnia, narcolepsy, and endocrinopathies. In some patients with NMO and NMO spectrum disorder who experience blood pressure fluctuations, vasogenic edema, manifesting as posterior reversible encephalopathy syndrome, may occur. In a recent report highlighting brain MRI with contrast enhancement, the most prominent feature that appeared to be a specific finding in NMO was "cloud-like enhancement" with multiple patchy enhancing lesions with a blurred margin. Another report showed that acute, large, edematous callosal lesions with

  20. Systemic lupus erythematosus in Asturias, Spain: clinical and serologic features.

    PubMed

    Gómez, Jesús; Suárez, Ana; López, Patricia; Mozo, Lourdes; Díaz, José Bernardino; Gutiérrez, Carmen

    2006-05-01

    Asturias is an autonomous region in the north of Spain with historical and anthropologic peculiarities. In the current report, we examine the main clinical and immunologic features of 363 patients with systemic lupus erythematosus (SLE), virtually the entire population of SLE patients in Asturias. We constructed a database with the clinical and immunologic features of all patients fulfilling the American College of Rheumatology criteria, based on the review of hospital records corresponding to blood samples received for antinuclear antibodies testing since 1992. Arthritis was the most frequently observed main clinical feature and neuropathy was the rarest. Male patients had a disease more frequently characterized by serositis (p<0.05) and neurologic disorder (p<0.01) than females, while children presented malar rash (p<0.05), fever (p<0.05), and kidney involvement (p<0.01) more often than adults. Late-onset patients were characterized by lower frequencies of malar rash (p<0.01), neurologic disorder (p<0.05), alopecia (p<0.01), and lymphadenopathy (p<0.05) than young adults. Numerous direct and inverse significant associations were found among clinical and immunologic features. The most relevant significant associations were neurologic disorder with lupus anticoagulant (p<0.01); kidney involvement with serositis (p<0.01) and DNA antibodies (p<0.05); and thrombosis with DNA antibodies (p<0.05), cardiolipin antibodies (p<0.01), and lupus anticoagulant (p<0.01). A low mortality was found in our series, although kidney involvement (p<0.05) and cardiolipin antibodies (p<0.05) are factors associated with poor survival.

  1. Clinical Practice Guidelines for Gastric Cancer in Korea: An Evidence-Based Approach

    PubMed Central

    Lee, Jun Haeng; Jung, Hye-Kyung; Kim, Jung Hoon; Jeong, Woo Kyoung; Jeon, Tae Joo; Kim, Joon Mee; Kim, Young Il; Ryu, Keun Won; Kong, Seong-Ho; Kim, Hyoung-Il; Jung, Hwoon-Yong; Kim, Yong Sik; Zang, Dae Young; Cho, Jae Yong; Park, Joon Oh; Lim, Do Hoon; Jung, Eun Sun; Ahn, Hyeong Sik; Kim, Hyun Jung

    2014-01-01

    Although gastric cancer is quite common in Korea, the treatment outcome is relatively favorable compared to those in western countries. However, there are currently no Korean multidisciplinary guidelines for gastric cancer. Experts from related societies developed guidelines de novo to meet Korean circumstances and requirements, including 23 recommendation statements for diagnosis (n=9) and treatment (n=14) based on relevant key questions. The quality of the evidence was rated according to the GRADE evidence evaluation framework: the evidence levels were based on a systematic review of the literature, and the recommendation grades were classified as either strong or weak. The applicability of the guidelines was considered to meet patients' view and preferences in the context of Korea. The topics of the guidelines cover diagnostic modalities (endoscopy, endoscopic ultrasound, and radiologic diagnosis), treatment modalities (surgery, therapeutic endoscopy, chemotherapy, and radiotherapy), and pathologic evaluation. An external review of the guidelines was conducted during the finalization phase. PMID:25061536

  2. Clinical features of 405 Japanese patients with systemic sclerosis.

    PubMed

    Hashimoto, Atsushi; Endo, Hirahito; Kondo, Hirobumi; Hirohata, Shunsei

    2012-04-01

    We aimed to clarify the clinical features of Japanese patients with systemic sclerosis (SSc), especially with reference to organ involvement and autoantibodies. A cohort of 405 patients with SSc who attended our institution from 1973 to 2008 was identified retrospectively. Data on clinical features, including autoantibodies, organ involvement, and overlap of other connective tissue diseases, were obtained by following the medical records until 2009. The percentage of male patients during or after 1990 was greater than that before 1990 (3.9 vs. 10.6%, respectively). Limited cutaneous SSc (lSSc) was twice as frequent as diffuse cutaneous SSc (dSSc). About half of the patients had lung involvement (50.4%), while only 3.2% had scleroderma renal crisis. Male gender was associated with lung involvement, and dSSc was associated with most organ involvements except for pulmonary arterial hypertension (PAH). Anti-Scl-70 antibody was associated with lung or heart involvement, while anti-U1-RNP antibody was only associated with PAH. Conversely, patients with anti-centromere antibody had less organ involvement. SSc-Sjögren overlap syndrome was related to lSSc, further overlapping systemic lupus erythematosus (SLE), and less lung or heart involvement. In conclusion, these results not only confirmed previous reports but revealed several other findings, such as the increased proportion of male patients in recent years and the relationships between clinical features.

  3. Clinical and Echographic Features of Retinochoroidal and Optic Nerve Colobomas

    PubMed Central

    Venincasa, Vincent D.; Modi, Yasha S.; Aziz, Hassan A.; Ayres, Bernadette; Zehetner, Claus; Shi, Wei; Murray, Timothy G.; Flynn, Harry W.; Berrocal, Audina M.

    2015-01-01

    Purpose. We reported the clinical and echographic features of colobomas, prevalence of retinal detachment, and associated visual acuity in these patients Methods. The study is a nonrandomized consecutive case series of 140 colobomatous eyes in 98 patients (age range, 0–83 years). Coloboma depth, width, volume, and relative coloboma excavation (coloboma depth/axial length) were measured using standardized echographic images. The presence of structural and other ocular abnormalities was noted. The clinical and echographic findings present were correlated with visual acuity of the patient. In addition, these features were correlated with the presence or absence of retinal detachment. Results. Increased relative coloboma excavation was significantly associated with an increased risk of retinal detachment. A relative coloboma excavation (ratio of coloboma depth to axial length) greater than 0.15 was associated with an increased risk of retinal detachment (52%), compared to those with a relative coloboma excavation less than 0.15 (23%, P = 0.014). The presence of any structural abnormality and the presence of a retrobulbar cyst were associated with increased risk of retinal detachment and severe visual impairment (worse than 20/200). Increased coloboma depth, width, volume, and relative coloboma excavation were not associated with increased risk of severe visual impairment. Conclusions. Clinical and echographic features of colobomas may be used in predicting the risk of retinal detachment. Measuring relative coloboma excavation upon presentation may alter follow-up and assist in the diagnosis of retinal detachment. PMID:26047048

  4. Clinical features associated with ictal osmophobia in migraine.

    PubMed

    Baldacci, Filippo; Lucchesi, Cinzia; Ulivi, Martina; Cafalli, Martina; Vedovello, Marcella; Vergallo, Andrea; Prete, Eleonora Del; Nuti, Angelo; Bonuccelli, Ubaldo; Gori, Sara

    2015-01-01

    Olfactory hypersensitivity may occur during migraine attacks and has been found to be very specific for this form of headache. Aim of this study was to investigate if migraineurs with ictal osmophobia have particular clinical features comparing to patients without ictal osmophobia. We recruited 200 consecutive migraineurs. Other primary headaches comorbidity and migraine prophylaxis were exclusion criteria. Each patient was interviewed following a structured questionnaire including general features about migraine, depression and anxiety symptoms. Migraine triggers both spontaneously and selecting from a specific list. Allodynia during the migraine attack was measured using the Allodynia symptoms check-list 12 (ASC-12). Eighty four (42 %) patients are non-osmophobic vs. 116 patients (58 %) who are osmophobic. After a logistic regression analysis, pain intensity (OR 1.391; p = 0.008) and anxiety (OR 1.099; p = 0.047) were significantly higher while aura (OR 0.421; p = 0.028) is less frequent in osmophobic migraineurs. We found significant differences in clinical features of osmophobic patients in respect to non-osmophobic ones. Ictal osmophobia seems being related to a broader sensorial hypersensitivity that could lead to a more florid clinical presentation.

  5. Clinical features of drug abuse that reflect genetic risk

    PubMed Central

    Kendler, K. S.; Ohlsson, H.; Sundquist, K.; Sundquist, J.

    2014-01-01

    Background Drug abuse (DA) is a clinically heterogeneous syndrome. Can we, in a large epidemiological sample, identify clinical features of DA cases that index genetic risk? Method Using registration in medical, legal or pharmacy records, we identified four kinds of relative pairs (n =935854) starting with a proband with DA: monozygotic co-twins; full siblings; half-siblings; and cousins. Using linear hazard regression, we examined the interaction between three clinical features of DA in the proband and risk for DA in these four relative pairs, ordered by degree of genetic relationship. Results Increased risk for DA in relatives was robustly predicted by early age at first registration, total number of registrations, and ascertainment in the criminal versus the medical or pharmacy registry. In multivariate models, all three of these variables remained significant and in aggregate strongly predicted DA risk in relatives. The risk for DA in siblings of DA probands in the highest decile of genetic risk predicted by our three indices was more than twice as great as that predicted in siblings of probands in the lowest decile of risk. Conclusions In an epidemiological sample, genetic risk for DA can be substantially indexed by simple clinical and historical variables. PMID:24461082

  6. Prevalence and Clinical Features of Atopic Dermatitis in China

    PubMed Central

    Wang, Xin; Zhao, Da-yu; Shen, Yi-wei

    2016-01-01

    Background. The epidemiology of atopic dermatitis (AD) in Chinese outpatients is yet to be clarified. Objectives. To investigate population-based prevalence and clinical features of AD in Chinese outpatients. Methods. A multicenter cross-sectional study was conducted in outpatients with eczema or dermatitis from 39 tertiary hospitals in 15 provinces. Results. This study included 682 patients diagnosed with AD, with the mean age of 28.8 ± 20.1 years and the median course of 5.3 ± 6.9 years. AD patients had more severe itching (30.4% versus 13.8%, p < 0.001) and clinically suspected bacterial infection (21.7% versus 16.1%, p < 0.001) than those of other types of dermatitis. Older patients were more susceptible to have a history of flexion dermatitis (p < 0.001), bacterial infection (p = 0.005), and severe itching (p < 0.001). Outpatients with clinically suspected bacterial infection had 3.53-fold increased risk of AD than those without it (p < 0.001). The morbidity rate of AD in the (20–25°N) region is 2.86 times higher than that in the (40–45°N) region [OR (95% CI): 0.352 (0.241–0.514), p < 0.001]. Conclusions. AD is characterized by unique clinical/demographic features. Bacterial infection and latitude region may have an impact on the incidence of AD in China. PMID:27957490

  7. Protocol of a Thyroid Cancer Longitudinal Study (T-CALOS): a prospective, clinical and epidemiological study in Korea

    PubMed Central

    Lee, Kyu Eun; Park, Young Joo; Cho, Belong; Hwang, Yunji; Choi, June Young; Kim, Su-jin; Choi, Hoonsung; Choi, Ho-Chun; An, Ah Reum; Park, Do Joon; Park, Sue K; Youn, Yeo-Kyu

    2015-01-01

    Introduction Thyroid cancer incidence in Korea is the highest in the world and has recently increased steeply. However, factors contributing to this sudden increase have not been fully elucidated, and few studies have explored the postoperative prognosis. The Thyroid Cancer Longitudinal Study (T-CALOS) was initiated with three aims: (1) to identify factors predicting quality of life, recurrence, and incidence of other diseases after thyroid cancer treatments; (2) to investigate environmental exposure to radiation, toxicants and molecular factors in relation to tumour aggressiveness; and (3) to evaluate gene–environment interactions that increase thyroid cancer in comparison with healthy participants from a pool of nationwide population-based healthy examinees. Methods and analysis T-CALOS enrols patients with incident thyroid cancer from three general hospitals, Seoul National University Hospital, Seoul National University Bundang Hospital and National Medical Center, Korea. The study is an ongoing project expecting to investigate 5000 patients with thyroid cancer up until 2017. Healthy examinees with a normal thyroid confirmed by sonography have been enrolled at the Healthy Examination Center at Seoul National University Hospital. We are also performing individual matching using two nationwide databases that are open to the public. Follow-up information is obtained at patients’ clinical visits and by linkage to the national database. For statistical analysis, we will use conditional logistic regression models and a Cox proportional hazard regression model. A number of stratifications and sensitivity analyses will be performed to confirm the results. Ethics and dissemination Based on a large sample size, a prospective study design, comprehensive data collection and biobank, T-CALOS has been independently peer-reviewed and approved by the three hospitals and two funding sources (National Research Foundation of Korea and Korean Foundation for Cancer Research

  8. Clinical and pathological features of patients with nemaline myopathy.

    PubMed

    Yin, Xi; Pu, Chuan Qiang; Wang, Qian; Liu, Jie Xiao; Mao, Yan Ling

    2014-07-01

    Nemaline myopathy (NM) is a rare congenital myopathy of great heterogeneity, characterized by the presence of rods in the cytoplasm of muscle fibers. This study aimed to summarize and analyze retrospectively the clinicopathological features of 28 patients with NM. Among the 28 patients, 15 were classified as of the typical congenital type, manifested as lower- or four-limb weakness as the first symptom and slowly progressive course. Six patients were classified as of childhood onset type, with lower-limb weakness and progressive course. Seven patients were classified as of the adult onset type, with rapidly progressive course and obvious muscle atrophy. Patient's 1, 16 and 23 had rapid clinical progression. On follow up, the three patients showed respiratory failure. Limb weakness in all patients was proximal‑dominant. Hypotonia was observed in most patients. High arched feet were also observed as dysmorfic features. In all patients, the creatine kinase (CK) level was normal or mildly elevated, and electromyography revealed myogenic changes. Nemaline bodies were observed under a light microscope in more than half of the patients' muscle fibers, and especially in type I fibers. All patients showed fiber type I predominance and atrophy. Modified Gömöri trichrome staining showed characteristic purple‑colored rods. Muscle electron microscopy revealed the presence of high electron‑dense nemaline bodies around the nucleus, and of a disorganized myofibrillar apparatus, with broken myofilaments and irregular myofibrils and Z lines. The 28 patients with NM shared a number of clinical features, such as proximal limb weakness, reduced deep tendon reflex and dysmorfic features. Differences were also observed between the three types of patients, with regards to course progression, disease severity and respiratory failure. In conclusion, patients with NM showed great clinical heterogeneity. The diagnosis of NM was mainly based on the muscle biopsy.

  9. Clinicopathologic Features and Clinical Outcomes of Esophageal Gastrointestinal Stromal Tumor

    PubMed Central

    Feng, Fan; Tian, Yangzi; Liu, Zhen; Xu, Guanghui; Liu, Shushang; Guo, Man; Lian, Xiao; Fan, Daiming; Zhang, Hongwei

    2016-01-01

    Abstract Clinicopathologic features and clinical outcomes of gastrointestinal stromal tumors (GISTs) in esophagus are limited, because of the relatively rare incidence of esophageal GISTs. Therefore, the aim of the current study was to investigate the clinicopathologic features and clinical outcomes of esophageal GISTs, and to investigate the potential factors that may predict prognosis. Esophageal GIST cases were obtained from our center and from case reports and clinical studies extracted from MEDLINE. Clinicopathologic features and survivals were analyzed and compared with gastric GISTs from our center. The most common location was lower esophagus (86.84%), followed by middle and upper esophagus (11.40% and 1.76%). The majority of esophageal GISTs were classified as high-risk category (70.83%). Mitotic index was correlated with histologic type, mutational status, and tumor size. The 5-year disease-free survival and disease-specific survival were 65.1% and 65.9%, respectively. Tumor size, mitotic index, and National Institutes of Health risk classification were associated with prognosis of esophageal GISTs. Only tumor size, however, was the independent risk factor for the prognosis of esophageal GISTs. In comparison to gastric GISTs, the distribution of tumor size, histologic type, and National Institutes of Health risk classification were significantly different between esophageal GISTs and gastric GISTs. The disease-free survival and disease-specific survival of esophageal GISTs were significantly lower than that of gastric GISTs. The most common location for esophageal GISTs was lower esophagus, and most of the esophageal GISTs are high-risk category. Tumor size was the independent risk factor for the prognosis of esophageal GISTs. Esophageal GISTs differ significantly from gastric GISTs in respect to clinicopathologic features. The prognosis of esophageal GISTs was worse than that of gastric GISTs. PMID:26765432

  10. Clinical features of depression in Asia: results of a large prospective, cross-sectional study.

    PubMed

    Srisurapanont, Manit; Hong, Jin Pyo; Tian-Mei, Si; Hatim, Ahmad; Liu, Chia-Yih; Udomratn, Pichet; Bae, Jae Nam; Fang, Yiru; Chua, Hong Choon; Liu, Shen-Ing; George, Tom; Bautista, Dianne; Chan, Edwin; Rush, A John

    2013-12-01

    The objective of this study was to investigate the clinical features of depression in Asian patients. It was a cross-sectional, observational study of depression in China, Korea, Malaysia, Singapore, Taiwan, and Thailand. Participants were drug-free outpatients with depressed mood and/or anhedonia. Symptoms and clinical features were assessed using the Montgomery-Asberg Depression Rating Scale, Symptoms Checklist 90-Revised (SCL-90-R), and the Fatigue Severity Scale. Other measures included the Medical Outcome Survey 36-Item Short-Form Health Survey (SF-36), the Sheehan Disability Scale, and the Multidimensional Scale of Perceived Social Support (MSPSS). A total of 547 outpatients with major depressive disorder were included in the analyses. Among the Montgomery-Asberg Depression Rating Scale symptoms, "reported sadness" and "reduced sleep" had the highest severity, with means (SDs) of 3.4 (1.2) and 3.4 (1.6), respectively. Apart from the SCL-90-R depression and anxiety domains, the SCL-90-R obsession-compulsion syndrome had the highest domain score, with a mean (SD) of 1.9 (0.9). Among eight domains, the mean (SD) SF-36 pain subscale score of 58.4 (27.7) was only second to that for the SF-36 physical function. In comparison to other disability domains, the Sheehan Disability Scale work/school had the highest subscale score, with a mean (SD) of 6.5 (2.9). The mean (SD) MSPSS "family" subscale score of 4.7 (1.7) was higher than the MSPSS "friends" and "significant others" subscale scores. This study suggests that pain has a minimal impact on the quality of life in Asian patients with depression. Noteworthy issues in this population may include insomnia, obsessive-compulsive symptoms, working/school disability, and family support. Copyright © 2013 Wiley Publishing Asia Pty Ltd.

  11. Clinical features and course of ocular toxocariasis in adults.

    PubMed

    Ahn, Seong Joon; Woo, Se Joon; Jin, Yan; Chang, Yoon-Seok; Kim, Tae Wan; Ahn, Jeeyun; Heo, Jang Won; Yu, Hyeong Gon; Chung, Hum; Park, Kyu Hyung; Hong, Sung Tae

    2014-06-01

    To investigate the clinical features, clinical course of granuloma, serologic findings, treatment outcome, and probable infection sources in adult patients with ocular toxocariasis (OT). In this retrospective cohort study, we examined 101 adult patients diagnosed clinically and serologically with OT. Serial fundus photographs and spectral domain optical coherence tomography images of all the patients were reviewed. A clinic-based case-control study on pet ownership, occupation, and raw meat ingestion history was performed to investigate the possible infection sources. Among the patients diagnosed clinically and serologically with OT, 69.6% showed elevated immunoglobulin E (IgE) levels. Granuloma in OT involved all retinal layers and several vitreoretinal comorbidities were noted depending on the location of granuloma: posterior pole granuloma was associated with epiretinal membrane and retinal nerve fiber layer defects, whereas peripheral granuloma was associated with vitreous opacity. Intraocular migration of granuloma was observed in 15 of 93 patients (16.1%). Treatment with albendazole (400 mg twice a day for 2 weeks) and corticosteroids (oral prednisolone; 0.5-1 mg/kg/day) resulted in comparable outcomes to patients on corticosteroid monotherapy; however, the 6-month recurrence rate in patients treated with combined therapy (17.4%) was significantly lower than that in patients treated with corticosteroid monotherapy (54.5%, P=0.045). Ingestion of raw cow liver (80.8%) or meat (71.2%) was significantly more common in OT patients than healthy controls. Our study discusses the diagnosis, treatment, and prevention strategies for OT. Evaluation of total IgE, in addition to anti-toxocara antibody, can assist in the serologic diagnosis of OT. Combined albendazole and corticosteroid therapy may reduce intraocular inflammation and recurrence. Migrating feature of granuloma is clinically important and may further suggest the diagnosis of OT. Clinicians need to carefully

  12. Clinical features of avian vacuolar myelinopathy in American coots

    USGS Publications Warehouse

    Larsen, R.S.; Nutter, F.B.; Augspurger, T.; Rocke, T.E.; Tomlinson, L.; Thomas, N.J.; Stoskopf, M.K.

    2002-01-01

    Objectivea??To characterize clinical features of avian vacuolar myelinopathy (AVM) in American coots. Designa??Case-control study. Animalsa??26 AVM-affected American coots and 12 unaffected coots. Proceduresa??Complete physical, neurologic, hematologic, and plasma biochemical evaluations were performed. Affected coots received supportive care. All coots died or were euthanatized, and AVM status was confirmed via histopathologic findings. Resultsa??3 severely affected coots were euthanatized immediately after examination. Seventeen affected coots were found dead within 7 days of admission, but 5 affected coots survived > 21 days and had signs of clinical recovery. Abnormal physical examination findings appeared to be related to general debilitation. Ataxia (88%), decreased withdrawal reflexes (88%), proprioceptive deficits (81%), decreased vent responses (69%), beak or tongue weakness (42%), and head tremors (31%), as well as absent pupillary light responses (46%), anisocoria (15%), apparent blindness (4%), nystagmus (4%), and strabismus (4%) were detected. Few gross abnormalities were detected at necropsy, but histologically, all AVM-affected coots had severe vacuolation of white matter of the brain. None of the control coots had vacuolation. Conclusions and Clinical Relevancea??Although there was considerable variability in form and severity of clinical neurologic abnormalities, clinical signs common in AVM-affected birds were identified. Clinical recovery of some AVM-affected coots can occur when supportive care is administered. Until the etiology is identified, caution should be exercised when rehabilitating and releasing coots thought to be affected by AVM.

  13. [The clinical-radiologic-pathologic features of imported pulmonary histoplasmosis].

    PubMed

    Gong, Pihua; Cao, Zhaolong; Mu, Xinlin; Dong, Xiaosong; Wang, Keqiang; Feng, Rui'e; Sun, Kunkun; Wang, Hui; Gao, Zhancheng

    2015-01-01

    To describe the clinical features and treatment of imported pulmonary histoplasmosis and therefore to improve the recognition and differential diagnosis of this disease. The clinical data of 3 patients with imported pulmonary histoplasmosis in our hospital were collected and analyzed. Literatures published since 1989 were retrieved with 'pulmonary histoplasmosis' from PubMed, China National Knowledge Infrastructure (CNKI), Wanfang Data and VIP data, of which all the literatures about imported pulmonary histoplasmosis were reviewed. The clinical manifestations, diagnostic methods and treatment were summarized. All the 3 cases of imported pulmonary histoplasmosis were immunocompetent hosts, all were males, age were from 44-67 years, and had a history of exploring the cave or tunnel inhabited by bats in the epidemic areas. All of them developed influenza-like symptoms varying in severity after the onset of the disease. Pulmonary multiple nodules and mediastinal lymphadenopathy were found on chest images. One patient underwent percutaneous lung biopsy and the other two received video-assisted thoracoscopic lung biopsy. All the 3 patients showed consistent histopathological findings, such as granulomatous inflammation with necrosis. Pathogen culture with lung biopsy in the first case was identified as histoplasma. All the 3 cases were treated with itraconazole, and recovered with good prognosis. Thirteen literatures in English were obtained, which reported 60 cases with imported pulmonary histoplasmosis. Forty-two of them were males, 16 were females and 2 undefined. The range of their age was from 17-64 years. No imported pulmonary histoplasmosis was reported so far in Chinese literature. Common features of imported pulmonary histoplasmosis were consistent with our patients, including epidemiology, influenza-like symptoms and bilateral pulmonary nodules, recovery with or without antifungal therapy. The epidemiologic history, influenza-like symptoms and bilateral

  14. Intramedullary gangliogliomas: clinical features, surgical outcomes, and neuropathic scoliosis.

    PubMed

    Yang, Chenlong; Li, Guang; Fang, Jingyi; Wu, Liang; Yang, Tao; Deng, Xiaofeng; Xu, Yulun

    2014-01-01

    Intramedullary spinal cord gangliogliomas are rare tumors composed of glial components and ganglion cells. These gangliogliomas are generally considered as slow-growing tumors, corresponding histologically to WHO grade I or II. There are few reports of large case series of intramedullary spinal cord gangliogliomas from a single center. We retrospectively reviewed a consecutive series of 18 patients with pathologically diagnosed ganglioglioma. Clinical manifestations, radiological features, treatment and follow-up data, and concomitant scoliosis were investigated. The mean age at diagnosis was 27.5 years, with a slight female predominance. The primary clinical symptoms were sensorimotor deficits. Magnetic resonance (MR) imaging manifestations varied considerably. Some associated, but not necessary, features were found, such as young age at onset, large tumor dimension, and bony changes. Scoliosis was observed in seven patients. Remnant tumor progression was observed in five patients during the follow-up period, and no deaths occurred. The last neurological evaluation showed functional improvement from preoperative status in five patients. Differential diagnosis of ganglioglioma based on MR images alone is challenging, but the combination of some characteristic features can be helpful. An accurate diagnosis of ganglioglioma depends on pathological criteria. Despite the benign course of ganglioglioma, considerable growth may affect its resectability and prognosis. The extent of resection should be meticulously planned, and the potential risk of recurrence and neurological deterioration should be evaluated. The concomitant scoliosis is noteworthy.

  15. Pituitary apoplexy: an update on clinical and imaging features.

    PubMed

    Boellis, Alessandro; di Napoli, Alberto; Romano, Andrea; Bozzao, Alessandro

    2014-12-01

    Pituitary apoplexy (PA) is a rare and potentially fatal clinical condition presenting acute headache, vomiting, visual impairment, ophthalmoplegia, altered mental state and possible panhypopituitarism. It mostly occurs in patients with haemorrhagic infarction of the pituitary gland due to a pre-existing macroadenoma. Although there are pathological and physiological conditions that may share similar imaging characteristics, both clinical and imaging features can guide the radiologist towards the correct diagnosis, especially using magnetic resonance imaging (MRI). In this review, we will describe the main clinical and epidemiological features of PA, illustrating CT and MRI findings and discussing the role of imaging in the differential diagnosis, prognosis and follow-up. Teaching points • Headache, ophtalmoplegia and visual impairment are frequent symptoms of pituitary apoplexy. • CT is often the first imaging tool in PA, showing areas of hyperdensity within the sellar region. • MRI could confirm haemorrhage within the pituitary gland and compression on the optic chiasm. • Frequent simulating conditions are aneurysms, Rathke cleft cysts, craniopharingioma and mucocele. • The role of imaging is still debated and needs more studies.

  16. Clinical and polysomnographic features of patients with Restless Legs Syndrome.

    PubMed

    Mungan, Semra; Ozcan, Murat; Orhan, Gurdal; Titiz, Ayse; Arli, Berna; Koseoglu, Sabri; Ak, Fikri; Dere, Haci Huseyin

    2016-05-01

    To reveal clinical and polysomnographic features in patients treated for restless leg syndrome, and to examine the compatibility of sleep data and clinical features. The study was conducted at the Department of Neurology, Ankara Numune Training and Education Hospital, Ankara, Turkey, and comprised patients who presented to the outpatient clinic between January and July 2014 who were diagnosed with restless leg syndrome based on the International RestIess Leg Syndrome Study Group criteria. Patients underwent polysomnography test in spontaneous sleep in a single room. SPSS 18 was used for statistical analyses. Of the 18 patients, 13(72%)were females and 5(28%)were males. Overall mean age was 51.56±11.57years (range: 23-66 years). Fourteen (77.8%) patients reported insomnia; 10(55.5%) patients had excessive daytime sleepiness; 13(72.2%) reported snoring; and 3(17%) had apnoea. Mean International Restless Legs Syndrome Study Group Rating Scale score was 26.11±7.9 (range: 16-40).Mean Epworth Sleepiness Scale score was 9.17±5.1 (range: 0-20). Restless leg syndrome was more common in women and the most common complaint was insomnia.

  17. [Corneal macular dystrophy: clinical, histopathologic and ultrastructural features].

    PubMed

    Gulias-Cañizo, R; Castañeda-Díez, R; Gómez-Leal, A; Klintworth, G K; Rodríguez-Reyes, A A

    2006-06-01

    To assess the main clinical, genetic, histopathological and ultrastructural features of Mexican patients with macular corneal dystrophy, and to compare the results with those previously reported. We analyzed six cases where a histopathologic diagnosis of macular corneal dystrophy had been made between 1957 and 2004. Clinically, all corneas showed focal grayish-white stromal opacities with diffuse edges. Histopathologically, intrastromal granules stained strongly positive with Alcian blue and colloidal iron. Transmission electron microscopy showed enlargement of smooth endoplasmic reticulum and the presence of intracytoplasmic vacuoles that corresponded to glycosaminoglycans. Genetic analysis showed novel mutations in the CHST6 gene in 2 of the patients. Females were more affected than males and the mean age at the time of diagnosis was older than that reported previously, however the clinical, histopathological and ultrastructural features were similar to those of previous reports. As described in other cases in the literature, in some instances a disorder is found in CHST6 gene as a basis for this condition.

  18. Clinical features and teratogenic mechanisms of congenital absence of digits.

    PubMed

    Ogino, Toshihiko

    2007-08-01

    To have a better understanding of classification of congenital hand anomalies, clinical features and teratogenic mechanisms of congenital absence of digits including ulnar and radial deficiencies, cleft hand, symbrachydactyly and constriction band were reviewed. There seemed to be four different teratogenic mechanisms of congenital absence of digits. Ulnar and radial deficiencies have the same clinical features and the cause of these deficiencies is closely related to a deficit of mesenchymal cells in the limb-bud due to impairment before the formation of the limb-bud. Cleft hand, central polydactyly and osseous syndactyly were induced by the same treatment at the same developmental stage in rats. Roentgenograms of the clinical cases and skeletal changes of the anomalies in rats appear to demonstrate that cleft hand formation proceeds from osseous syndactylies and central polydactylies. The teratogenic mechanism of a cleft hand seemed to be failure of induction of digital rays in the hand plate. The sequence of anomalies from brachysyndactyly, or the atypical cleft hand, to the congenital amputation, can be regarded as equivalent to the category of transverse deficiency that is bony dysplasia of the hand. Congenital constriction ring syndrome appears after the formation of the digital rays.

  19. Clinical features and prognosis of intracranial artery dissection.

    PubMed

    Sikkema, Tineke; Uyttenboogaart, Maarten; van Dijk, J Marc C; Groen, Rob J M; Metzemaekers, Jan D M; Eshghi, Omid; Mazuri, Aryan; Bakker, Nicolaas A; Luijckx, Gert-Jan

    2015-06-01

    Intracranial artery dissections (IADs) are an important cause of stroke or subarachnoid hemorrhage (SAH). Outcome of IAD in the anterior circulation or presentation without SAH is rarely investigated and might be different. To evaluate the clinical features and prognosis of patients with IAD, with special emphasis on the location (anterior vs posterior circulation) and clinical presentation (SAH or cerebral ischemia). Between January 1998 and May 2012, 60 patients with IAD were included in this single-center cohort study. Clinical features, functional outcome, mortality, and prognostic factors were evaluated. Unfavorable functional outcome was defined as a modified Rankin scale score of 3 to 6. In 18 patients (30%), IAD was located in the anterior circulation. At a median follow-up of 6.4 months, 35.3% of patients with IAD in the anterior circulation had an unfavorable functional outcome vs 39.0% in patients with IAD in the posterior circulation (P = .79). Forty-two patients (70%) presented with SAH. Clinical presentation with SAH was not significantly associated with poor functional outcome (41.5% vs 29.4%, P = .39). Low Glasgow Coma Scale score on admission (odds ratio, 0.72, P = .003) and older age (odds ratio, 1.04, P = .04) were independent predictors of unfavorable functional outcome. Mortality rate was 13% and did not significantly differ with location or clinical presentation. Low Glasgow Coma Scale score on admission and older age were independent predictors of unfavorable functional outcome. IAD presenting with SAH was not significantly associated with poor functional outcome.

  20. The Clinical Features of Myositis-Associated Autoantibodies: a Review.

    PubMed

    Gunawardena, Harsha

    2017-02-01

    The idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases traditionally defined by clinical manifestations including skeletal muscle weakness, skin rashes, elevated skeletal muscle enzymes, and neurophysiological and/or histological evidence of muscle inflammation. Patients with myositis overlap can develop other features including parenchymal lung disease, inflammatory arthritis, gastrointestinal manifestations and marked constitutional symptoms. Although patients may be diagnosed as having polymyositis (PM) or dermatomyositis (DM) under the IIM spectrum, it is quite clear that disease course between subgroups of patients is different. For example, interstitial lung disease may predominate in some, whereas cutaneous complications, cancer risk, or severe refractory myopathy may be a significant feature in others. Therefore, tools that facilitate diagnosis and indicate which patients require more detailed investigation for disease complications are invaluable in clinical practice. The expanding field of autoantibodies (autoAbs) associated with connective tissue disease (CTD)-myositis overlap has generated considerable interest over the last few years. Using an immunological diagnostic approach, this group of heterogeneous conditions can be separated into a number of distinct clinical phenotypes. Rather than diagnose a patient as simply having PM, DM or overlap CTD, we can define syndromes to differentiate disease subsets that emphasise clinical outcomes and guide management. There are now over 15 CTD-myositis overlap autoAbs found in patients with a range of clinical manifestations including interstitial pneumonia, cutaneous disease, cancer-associated myositis and autoimmune-mediated necrotising myopathy. This review describes their diagnostic utility, potential role in disease monitoring and response to treatment. In the future, routine use of these autoAb will allow a stratified approach to managing this complex set of conditions.

  1. Cardiovascular Magnetic Resonance Myocardial Feature Tracking: Concepts and Clinical Applications.

    PubMed

    Schuster, Andreas; Hor, Kan N; Kowallick, Johannes T; Beerbaum, Philipp; Kutty, Shelby

    2016-04-01

    Heart failure-induced cardiovascular morbidity and mortality constitute a major health problem worldwide and result from diverse pathogeneses, including coronary artery disease, nonischemic cardiomyopathies, and arrhythmias. Assessment of cardiovascular performance is important for early diagnosis and accurate management of patients at risk of heart failure. During the past decade, cardiovascular magnetic resonance myocardial feature tracking has emerged as a useful tool for the quantitative evaluation of cardiovascular function. The method allows quantification of biatrial and biventricular mechanics from measures of deformation: strain, torsion, and dyssynchrony. The purpose of this article is to review the basic principles, clinical applications, accuracy, and reproducibility of cardiovascular magnetic resonance myocardial feature tracking, highlighting the prognostic implications. It will also provide an outlook on how this field might evolve in the future.

  2. Obesity, age, ethnicity, and clinical features of prostate cancer patients

    PubMed Central

    Wu, Victor J; Pang, Darren; Tang, Wendell W; Zhang, Xin; Li, Li; You, Zongbing

    2017-01-01

    Approximately 36.5% of the U.S. adults (≥ 20 years old) are obese. Obesity has been associated with type 2 diabetes mellitus, cardiovascular disease, stroke, and several types of cancer. The present study included 1788 prostate cancer patients who were treated with radical prostatectomy at the Ochsner Health System, New Orleans, Louisiana, from January, 2001 to March, 2016. The patient’s medical records were retrospectively reviewed. Body mass index (BMI), age, ethnicity (Caucasians versus African Americans), clinical stage, Gleason score, and prostate-specific antigen (PSA) levels were retrieved. The relative risk of the patients was stratified into low risk and high risk groups. Associative analyses found that BMI was associated with age, clinical stage, Gleason score, but not ethnicity, PSA levels, or the relative risk in this cohort. Age was associated with ethnicity, clinical stage, Gleason score, and PSA levels, as well as the relative risk. Ethnicity was associated with Gleason score and PSA levels as well as the relative risk, but not clinical stage. These findings suggest that obesity is associated with advanced prostate cancer with stage T3 or Gleason score ≥ 7 diseases, and age and ethnicity are important factors that are associated with the clinical features of prostate cancer patients. PMID:28337464

  3. First molecular detection of Borrelia afzelii in clinical samples in Korea.

    PubMed

    Choi, Yeon-Joo; Han, Seung-Hoon; Park, Jin-Mi; Lee, Kyung-Min; Lee, Eun-Mi; Lee, Seung-Hyun; Song, Hyeon-Je; Koh, Young-Sang; Lee, Keun-Wha; Jang, Won-Jong; Park, Kyung-Hee

    2007-01-01

    Borrelia afzelii nucleic acids were detected in the sera of febrile disease patients by a nested PCR that targeted the rrf (5S)-rrl (23S) spacer of B. burgdorferi sensu lato. The B. afzelii-specific DNA fragment was detected in 8 out of 283 sera which were proven to have immunoglobulin G or M antibodies against B. burgdorferi antigens through IFA. The results were further confirmed through restriction fragment length polymorphism and sequencing analysis of the DNA fragments. The results indicated for the first time that Lyme borreliosis is prevalent in Korea.

  4. Clinical and Ultrasonographic Features of Secondary Intussusception in Children.

    PubMed

    Zhang, Yao; Dong, Qian; Li, Shi-Xing; Ren, Wei-Dong; Shi, Bo; Bai, Yu-Zuo; Zhang, Shu-Cheng; Zheng, Li-Qiang

    2016-12-01

    The aim of this study was to review the ultrasonographic features of secondary intussusception (SI) in children and assess the value of ultrasound in the diagnosis of pediatric SI. The authors performed a retrospective analysis on the ultrasound findings of 1977 cases of primary intussusception (PI) and 37 cases of SI in children. The SI cases were diagnosed by ultrasonography and confirmed by laparotomy or histopathologic diagnosis. The clinical and ultrasonographic features were analyzed and compared between these two groups. The age, no flatus or defecation, position, diameter and length of intussusception, the presence of free intraperitoneal liquid, and intestinal dialation at the proximal end present, all contributed to the differentiation between PI and SI (all P < 0.05). Ultrasound was able to demonstrate the pathological lead point (PLP) shadows in all of the 37 SI cases, either in the cervical part or intussusceptum of the intussusception. Among the 37 SI patients, 21 cases (56.8 %) were accurately categorized with lesions, including intestinal polyps, cystic intestinal duplication, intestinal wall lymphoma, and a small part of Meckel's diverticulum. Ultrasound can be used as a feasible and effective method to discriminate PI from SI. Once the PLP is detected, a definite diagnosis can be made. • The clinical and ultrasonographic features were compared between SI and PI. • The age, location, diameter and length of intussusception, and intestinal dilation were distinguishing features. • The causes of SI were found to be polyps, intestinal duplication, lymphoma, and Meckel's diverticulum. • Ultrasound can be used as an important method to diagnose SI. • Demonstration and confirmation of PLP are vital to diagnosing SI.

  5. Ranitidine-induced anaphylaxis: clinical features, cross-reactivity, and skin testing.

    PubMed

    Park, K H; Pai, J; Song, D-G; Sim, D W; Park, H J; Lee, J-H; Jeong, K Y; Pan, C-H; Shin, I; Park, J-W

    2016-04-01

    Histamine H2 receptor antagonists are commonly prescribed medications and are known to be well tolerated. However, 99 cases of ranitidine-induced anaphylaxis occurred in Korea from 2007 to 2014. The purpose of this study was to determine the incidence, clinical features, and diagnostic methods for ranitidine-induced anaphylaxis. Ranitidine-related pharmacovigilance data from 2007 to 2014 were reviewed. Adverse drug reactions with causal relationships were selected, and clinical manifestations, outcomes, and drug-related information were assessed. For further investigation, 8 years of pharmacovigilance data were collected at a single centre. Twenty-three patients participated in in vivo and in vitro studies. Skin tests, oral provocation tests, and laboratory tests were performed, including tests using other kinds of histamine H2 receptor antagonists. Over 7 years, 584 patients suffered adverse reactions to ranitidine. The most common manifestation was cutaneous symptoms. Among them, 99 patients (17.0%) experienced anaphylaxis. In a single-centre study, skin prick tests were positive in 91.7% of ranitidine-induced anaphylaxis patients (11/12); the optimal concentration was 20 mg/mL. Detection of ranitidine-specific immunoglobulin E failed. Cimetidine and proton pump inhibitors showed no cross-reactivity with ranitidine based on the skin prick test, oral provocation test, or clinical determination. Surprisingly, 82.6% of patients reintroduced ranitidine and re-experienced the same adverse reactions because ranitidine was not considered the culprit drug. Although ranitidine is known as a safe drug, it can also cause diverse adverse reactions, including anaphylaxis. This study demonstrates the need to pay attention to adverse reactions to ranitidine and consider ranitidine as a cause of anaphylaxis. © 2016 John Wiley & Sons Ltd.

  6. Clinical Features of Male Breast Cancer: Experiences from Seven Institutions Over 20 Years.

    PubMed

    Hong, Ji Hyung; Ha, Kyung Sun; Jung, Yun Hwa; Won, Hye Sung; An, Ho Jung; Lee, Guk Jin; Kang, Donghoon; Park, Ji Chan; Park, Sarah; Byun, Jae Ho; Suh, Young Jin; Kim, Jeong Soo; Park, Woo Chan; Jung, Sang Seol; Park, Il Young; Chung, Su-Mi; Woo, In Sook

    2016-10-01

    Breast cancer treatment has progressed significantly over the past 20 years. However, knowledge regarding male breast cancer (MBC) is sparse because of its rarity. This study is an investigation of the clinicopathologic features, treatments, and clinical outcomes of MBC. Clinical records of 59 MBC patients diagnosed during 1995-2014 from seven institutions in Korea were reviewed retrospectively. Over a 20-year period, MBC patients accounted for 0.98% among total breast cancer patients, and increased every 5 years. The median age of MBC patientswas 66 years (range, 24 to 87 years). Forty-three patients (73%) complained of a palpable breast mass initially. The median symptom duration was 5 months (range, 1 to 36 months). Mastectomy was performed in 96% of the patients. The most frequent histology was infiltrating ductal carcinoma (75%). Ninety-one percent of tumors (38/43) were estrogen receptor-positive, and 28% (11/40) showed epidermal growth factor receptor 2 (HER-2) overexpression. After curative surgery, 42% of patients (19/45) received adjuvant chemotherapy; 77% (27/35) received hormone therapy. Five out of ten patients with HER-2 overexpressing tumors did not receive adjuvant anti-HER-2 therapy, while two out of four patients with HER-2 overexpressing tumors received palliative trastuzumab for recurrent and metastatic disease. Letrozole was used for one patient in the palliative setting. The median overall survival durations were 7.2 years (range, 0.6 to 17.0 years) in patients with localized disease and 2.9 years (range, 0.6 to 4.3 years) in those with recurrent or metastatic disease. Anti-HER-2 and hormonal therapy, except tamoxifen, have been underutilized in Korean MBC patients compared to female breast cancer patients. With the development of precision medicine, active treatment with targeted agents should be applied. Further investigation of the unique pathobiology of MBC is clinically warranted.

  7. Clinical and microbiologic features of dacryocystitis-related orbital cellulitis.

    PubMed

    Wladis, Edward J; Shinder, Roman; LeFebvre, Daniel R; Sokol, Jason A; Boyce, Michelle

    2016-10-01

    Dacryocystitis-related orbital cellulitis is a relatively rare condition, and large case series of this clinical entity have been reported. This study was undertaken to identify a larger cohort of patients with this ailment, with the intent of defining its clinical and microbiologic features. Case logs from four institutions were reviewed to identify patients that suffered from dacryocystitis-related orbital cellulitis. A retrospective chart review was then performed to identify clinical features, management strategies, microbiologic features, and outcomes. A dedicated statistical software package was utilized to identify correlations between these variables. 13 patients (7 females, 6 males; mean age = 57.2 years, range = 7-89 years) were identified. One patient carried a diagnosis of immunosuppressive disease. All patients underwent emergent surgical drainage and received intravenous antibiotics. Primary acquired nasolacrimal duct obstruction was found to be the underlying etiology in nine cases (69.2%), whereas four patients suffered from specific causes of their obstructions. An average of 1.07 organisms/patient (standard deviation = 0.49 organisms/patient) were recovered from microbiologic cultures, and Gram-positive bacteria represented the majority of cultured organisms. All patients experienced either stable or improved vision upon discharge. The relationships between a specific etiology and the possibility of vision loss or the number of organisms cultured, between the number of organisms cultured and vision loss, and immunosuppression and vision loss or the number of organisms cultured were all not statistically significant (p > 0.05). Dacryocystitis-related orbital cellulitis most commonly occurs in adult patients who do not carry immunosuppressive diagnoses and suffer from primary obstructions. Multiple microbiologic species may cause this problem, although Gram-positive organisms are most common. With appropriate management, stable or improved vision

  8. Uncovering Clinical Features of De Novo Philadelphia Positive Myelodysplasia

    PubMed Central

    Armas, Aristides; Chen, Chen; Mims, Martha

    2017-01-01

    Myelodysplastic syndrome (MDS) is cytogenetically heterogeneous and retains variable risk for acute myeloid leukemia transformation. Though not yet fully understood, there is an association between genetic abnormalities and defects in gene expression. The functional role for infrequent cytogenetic alteration remains unclear. An uncommon chromosomic abnormality is the presence of the Philadelphia (Ph) chromosome. Here, we report a patient with Ph+ MDS treated with low dose Dasatinib who achieved hematologic response for 7 months. In addition, we also examined the English literature on all de novo Ph + MDS cases between 1996 and 2015 to gain insight into clinical features and outcome. PMID:28321349

  9. Uncovering Clinical Features of De Novo Philadelphia Positive Myelodysplasia.

    PubMed

    Armas, Aristides; Chen, Chen; Mims, Martha; Rivero, Gustavo

    2017-01-01

    Myelodysplastic syndrome (MDS) is cytogenetically heterogeneous and retains variable risk for acute myeloid leukemia transformation. Though not yet fully understood, there is an association between genetic abnormalities and defects in gene expression. The functional role for infrequent cytogenetic alteration remains unclear. An uncommon chromosomic abnormality is the presence of the Philadelphia (Ph) chromosome. Here, we report a patient with Ph+ MDS treated with low dose Dasatinib who achieved hematologic response for 7 months. In addition, we also examined the English literature on all de novo Ph + MDS cases between 1996 and 2015 to gain insight into clinical features and outcome.

  10. Clinical and Biological Features of Interval Colorectal Cancer

    PubMed Central

    Lee, Yu Mi; Huh, Kyu Chan

    2017-01-01

    Interval colorectal cancer (I-CRC) is defined as a CRC diagnosed within 60 months after a negative colonoscopy, taking into account that 5 years is the “mean sojourn time.” It is important to prevent the development of interval cancer. The development of interval colon cancer is associated with female sex, old age, family history of CRC, comorbidities, diverticulosis, and the skill of the endoscopist. During carcinogenesis, sessile serrated adenomas/polyps (SSA/Ps) share many genomic and colonic site characteristics with I-CRCs. The clinical and biological features of I-CRC should be elucidated to prevent the development of interval colon cancer. PMID:28320200

  11. [Clinical radiographic features of basilar impression (author's transl)].

    PubMed

    Scotti, G; Redaelli, M R

    1978-09-01

    After a review of the literature and an analysis of the clinical and radiographic features of basilar impression (b.i.) the authors describe 27 personal cases. In 6 b.i. was present in its pure form and in 21 it was associated with other anomalies at the cranio-vertebral junction. Because of the frequent misdiagnosis with multiple sclerosis or other chronic neurologic diseases and because of the possible improvement of symptoms following decompressive occipital craniectomy and cervical laminectomy, the importance of a correct and complete radiographic study of the cranio-vertebral junction in these cases is stressed.

  12. Skeletal Muscle Laminopathies: A Review of Clinical and Molecular Features

    PubMed Central

    Maggi, Lorenzo; Carboni, Nicola; Bernasconi, Pia

    2016-01-01

    LMNA-related disorders are caused by mutations in the LMNA gene, which encodes for the nuclear envelope proteins, lamin A and C, via alternative splicing. Laminopathies are associated with a wide range of disease phenotypes, including neuromuscular, cardiac, metabolic disorders and premature aging syndromes. The most frequent diseases associated with mutations in the LMNA gene are characterized by skeletal and cardiac muscle involvement. This review will focus on genetics and clinical features of laminopathies affecting primarily skeletal muscle. Although only symptomatic treatment is available for these patients, many achievements have been made in clarifying the pathogenesis and improving the management of these diseases. PMID:27529282

  13. Epidemiologic features of the first MERS outbreak in Korea: focus on Pyeongtaek St. Mary’s Hospital

    PubMed Central

    Kim, Kyung Min; Ki, Moran; Cho, Sung-il; Sung, Minki; Hong, Jin Kwan; Cheong, Hae-Kwan; Kim, Jong-Hun; Lee, Sang-Eun; Lee, Changhwan; Lee, Keon-Joo; Park, Yong-Shik; Kim, Seung Woo; Choi, Bo Youl

    2015-01-01

    OBJECTIVES: This study investigated the epidemiologic features of the confirmed cases of Middle East Respiratory Syndrome (MERS) in Pyeongtaek St. Mary’s Hospital, where the outbreak first began, in order to identify lessons relevant for the prevention and control of future outbreaks. METHODS: The patients’ clinical symptoms and test results were collected from their medical records. The caregivers of patients were identified by phone calls. RESULTS: After patient zero (case #1) was admitted to Pyeongtaek St. Mary’s Hospital (May 15-May 17), an outbreak occurred, with 36 cases between May 18 and June 4, 2015. Six patients died (fatality rate, 16.7%). Twenty-six cases occurred in the first-generation, and 10 in the second-generation. The median incubation period was five days, while the median period from symptom onset to death was 12.5 days. While the total attack rate was 3.9%, the attack rate among inpatients was 7.6%, and the inpatients on the eighth floor, where patient zero was hospitalized, had an 18.6% attack rate. In contrast, caregivers and medical staff showed attack rates of 3.3% and 1.1%, respectively. CONCLUSIONS: The attack rates were higher than those of the previous outbreaks in other countries. The outbreak spread beyond Pyeongtaek St. Mary’s Hospital when four of the patients were moved to other hospitals without appropriate quarantine. The best method of preventing future outbreaks is to overcome the vulnerabilities observed in this outbreak, such as ward crowding, patient migration without appropriate data sharing, and the lack of an initial broad quarantine. PMID:26725225

  14. Satisfaction Survey on Information Technology-Based Glucose Monitoring System Targeting Diabetes Mellitus in Private Local Clinics in Korea.

    PubMed

    Kim, Hun Sung; Yang, So Jung; Jeong, Yoo Jin; Kim, Young Eun; Hong, Seok Won; Cho, Jae Hyoung

    2017-06-01

    Private local clinics in Korea have little experience with information technology (IT)-based glucose monitoring (ITGM). Our aim is to examine user satisfaction and the possibility of using ITGM service practically. Patients sent their blood glucose levels to physicians in local clinics. The physicians reviewed the blood glucose values online and provided personal consultations through text messaging or phone calls. Thereafter, a satisfaction survey on the ITGM service, the modified Morisky scale, and patient assessment of chronic illness care were administered. One hundred and seventy patients from seven private local clinics used the ITGM. Overall satisfaction, including that about the ITGM service, the device, and its usefulness, was rated higher than "mostly satisfied" (score 4.2±0.8 out of 5.0) and even higher among the elderly. Satisfaction was positively associated with age, especially in those older than 60 years. The main reason for intent for future use of the service was the time/place flexibility. Highly motivated patients tended to answer positively regarding information satisfaction (P=0.0377). Our study is the first to investigate ITGM satisfaction in private local clinics. The feasibility of users utilizing ITGM should be clarified, and future clinical research on the service's clinical effects and cost-benefit analysis is needed.

  15. Clinical features of paroxysmal kinesigenic dyskinesia: report of 24 cases.

    PubMed

    Sun, Wei; Li, Jianing; Zhu, Yulan; Yan, Xiaobo; Wang, Weizhi

    2012-12-01

    Paroxysmal kinesigenic dyskinesia (PKD) is the most common type of paroxysmal dyskinesia and is characterized by involuntary, intermittent movements induced by sudden movements. Here, we describe 24 patients with PKD, whose clinical data were analyzed. The attacks of involuntary movements were all short lasting, and could involve extremities, trunk, neck, or face without alteration of consciousness. The motor function was normal between attacks, and in some cases, attacks could be evoked during examination. Most patients had normal electroencephalogram (EEG) and neuroimaging results, but 2 cases had abnormal EEGs, and another 2 cases had bilateral calcification of basal ganglion on brain computed tomography (CT) scans. Previous history of misdiagnosis was a predominant feature, while treatments based on misdiagnosis sometimes did lead to improvement. Here, we discuss the clinical characteristics, especially the abnormalities of investigations and misdiagnosis, and recent insights into the pathophysiology of PKD.

  16. Clinical features of infections and colonization by Acinetobacter genospecies 3.

    PubMed

    Molina, José; Cisneros, José Miguel; Fernández-Cuenca, Felipe; Rodríguez-Baño, Jesús; Ribera, Anna; Beceiro, Alejandro; Martínez-Martínez, Luis; Pascual, Álvaro; Bou, Germán; Vila, Jordi; Pachón, Jerónimo

    2010-12-01

    Two hundred twenty-one isolates of Acinetobacter baumannii and 15 of Acinetobacter genospecies 3 (AG3) were consecutively collected in a 30-day period during the nationwide project GEIH-Ab2000. Nosocomial acquisition (P=0.01), intensive care unit admission (P=0.02), and antibiotic pressure (P = 0.03) were observed to be lower in the AG3 group. AG3 isolates were more frequently implied in wound infections (P=0.05), while A. baumannii tended to be recovered from respiratory samples (P=0.08). To our knowledge, this is the first report analyzing the clinical differences among Acinetobacter genospecies, with our findings suggesting that clinical features of AG3 may not be equivalent to those traditionally described for A. baumannii.

  17. Radiological and clinical features of vein of Galen malformations.

    PubMed

    Chow, Maggie L; Cooke, Daniel L; Fullerton, Heather J; Amans, Matthew R; Narvid, Jared; Dowd, Christopher F; Higashida, Randall T; Halbach, Van V; Hetts, Steven W

    2015-06-01

    Vein of Galen malformations (VOGMs) are rare and complex congenital arteriovenous fistulas. The clinical and radiological features of VOGMs and their relation to clinical outcomes are not fully characterized. To examine the clinical and radiological features of VOGMs and the predictors of outcome in patients. We retrospectively reviewed the available imaging and medical records of all patients with VOGMs treated at the University of California, San Francisco between 1986 and 2013. Radiological and clinical features were identified. We applied the modified Rankin Scale to determine functional outcome by chart review. Predictors of outcome were assessed by χ(2) analyses. Forty-one cases were confirmed as VOGM. Most patients (78%) had been diagnosed with VOGM in the first year of life. Age at treatment was bimodally distributed, with predominantly urgent embolization at <10 days of age and elective embolization after 1 year of age. Patients commonly presented with hydrocephalus (65.9%) and congestive heart failure (61.0%). Mixed-type (31.7%) VOGM was more common in our cohort than purely mural (29.3%) or choroidal (26.8%) types. The most common feeding arteries were the choroidal and posterior cerebral arteries. Transarterial embolization with coils was the most common technique used to treat VOGMs at our institution. Functional outcome was normal or only mildly disabled in 50% of the cases at last follow-up (median=3 years, range=0-23 years). Younger age at first diagnosis, congestive heart failure, and seizures were predictive of adverse clinical outcome. The survival rate in our sample was 78.0% and complete thrombosis of the VOGM was achieved in 62.5% of patients. VOGMs continue to be challenging to treat and manage. Nonetheless, endovascular approaches to treatment are continuing to be refined and improved, with increasing success. The neurodevelopmental outcomes of affected children whose VOGMs are treated may be good in many cases. Published by the BMJ

  18. Clinical Features and Extraintestinal Manifestations of Crohn Disease in Children

    PubMed Central

    Lee, Young Ah; Chun, Peter; Hwang, Eun Ha; Mun, Sang Wook; Lee, Yeoun Joo

    2016-01-01

    Purpose The aim of this study was to investigate the clinical features and extraintestinal manifestations (EIMs) of Crohn disease (CD) in Korean pediatric patients. Methods The medical records of 73 children diagnosed with CD were retrospectively reviewed. Data regarding baseline demographic and clinical characteristics, including CD phenotype at diagnosis based on the Montreal classification, and clinical features and course of EIMs were investigated. Results Fifty-two (71.2%) of the patients were males. The mean age of the patients was 12.5 years. The mean follow-up period was 3.4 years. The disease location was ileal in 3 (4.1%) of the patients, colonic in 13 (17.8%), ileocolonic in 56 (76.7%). The clinical behavior was inflammatory in 62 (84.9%) of the patients, stricturing in 8 (11.0%), and penetrating in 3 (4.1%). Perianal abscesses or fistulas were found in 37 (50.7%) of the patients. EIMs observed during the study period were anal skin tag in 25 patients (34.2%), hypertransaminasemia in 20 (27.4%), peripheral arthritis in 2 (2.7%), erythema nodosum in 2 (2.7%), vulvitis in 1 (1.4%), uveitis in 1 (1.4%), and pulmonary thromboembolism in 1 (1.4%). Conclusion Perianal diseases and manifestations were present in more than half of Korean pediatric CD patients at diagnosis. Inspection of the anus should be mandatory in Korean children with suspicious CD, as perianal fistulas, abscesses, and anal skin tags may be the first clue to the diagnosis of CD. PMID:28090468

  19. Hemorrhage in posterior reversible encephalopathy syndrome: imaging and clinical features.

    PubMed

    Hefzy, H M; Bartynski, W S; Boardman, J F; Lacomis, D

    2009-08-01

    Hemorrhage is known to occur in posterior reversible encephalopathy syndrome (PRES), but the characteristics have not been analyzed in detail. The purpose of this study was to evaluate the imaging and clinical features of hemorrhage in PRES. Retrospective assessment of 151 patients with PRES was performed, and 23 patients were identified who had intracranial hemorrhage at toxicity. Hemorrhage types were identified and tabulated, including minute focal hemorrhages (<5 mm), sulcal subarachnoid hemorrhage, and focal hematoma. Clinical features of hemorrhage and nonhemorrhage PRES groups were evaluated, including toxicity blood pressure, coagulation profile/platelet counts, coagulation-altering medication, and clinical conditions associated with PRES. Toxicity mean arterial pressure (MAP) groups were defined as normal (<106 mm Hg), mildly hypertensive (106-116 mm Hg), or severely hypertensive (>116 mm Hg). The overall incidence of hemorrhage was 15.2%, with borderline statistical significance noted between the observed clinical associations (P = .07). Hemorrhage was significantly more common (P = .02) after allogeneic bone marrow transplantation (allo-BMT) than after solid-organ transplantation. The 3 hemorrhage types were noted with equal frequency. A single hemorrhage type was found in 16 patients, with multiple types noted in 7. Patients undergoing therapeutic anticoagulation were statistically more likely to develop hemorrhage (P = .04). No difference in hemorrhage incidence was found among the 3 blood pressure subgroups (range, 14.9%-15.9%). Three distinct types of hemorrhage (minute hemorrhage, sulcal subarachnoid hemorrhage, hematoma) were identified in PRES with equal frequency. The greatest hemorrhage frequency was seen after allo-BMT and in patients undergoing therapeutic anticoagulation. Hemorrhage rate was independent of the toxicity blood pressure.

  20. [The clinical features of migraine-associated vertigo].

    PubMed

    Goto, Fumiyuki; Tsutsumi, Tomoko; Ogawa, Kaoru

    2013-08-01

    Migraine-associated vertigo (MAV) is proposed as a new clinical entity on the basis of the assumption that the typical migraine and vertigo or dizziness have a common pathophysiology. Some of the patients with recurrent vertigo syndromes with unknown pathology may have MAV. We performed a retrospective study to clarify the clinical characteristics of MAV in the Japanese population. The following were considered as diagnostic criteria: (1) recurrent vestibular symptoms, (2) migraine headache as defined by the International Headache Society (IHS) criteria, (3) at least one instance of synchronization of a vertiginous attack with a migraine headache, (4) no associated unilateral hearing loss, and (5) absence of other diseases that may have caused vertiginous attacks. Of 552 patients with dizziness or vertigo, 46 (8.3%) were diagnosed as having MAV. A typical feature of this clinical entity is that migraine occurs before the onset of vertigo in women aged 30-40 years. Usually the attacks occur once in a year for 1 to 10 years. An attack lasts for 1-24 h and presents as vertigo and unsteadiness with simultaneous headache. The presence of hearing loss presents an important clinical dilemma. Whether the condition in patients experiencing hearing loss should be defined as MAV or not is still a matter for discussion.

  1. Clinical features and neuropsychological profile in vascular parkinsonism.

    PubMed

    Benítez-Rivero, Sonia; Lama, María J; Huertas-Fernández, Ismael; Alvarez de Toledo, Paloma; Cáceres-Redondo, María T; Martín-Rodríguez, Juan F; Carrillo, Fátima; Carballo, Manuel; Palomar, Francisco J; Mir, Pablo

    2014-10-15

    The clinical profile in vascular parkinsonism (VP) patients is well described in the literature, but little is known about the neuropsychological features of this disease. The aim of our study was to evaluate the clinical characteristics and the profile of cognitive impairment in patients with VP. We prospectively evaluated 12 patients with VP, 15 with Parkinson's disease (PD) and 13 healthy controls (HC) with similar age and sex distribution. Different clinical and demographic details were collected. All subjects underwent detailed neurological and neuropsychological examinations. The neuropsychological tests included analysis of global efficiency, executive function, verbal memory, language and visuospatial function. VP patients exhibited lower disease duration, older age at onset and higher frequency of cardiovascular risk factors. Non-motor symptoms were found to be more frequent in PD. We found that VP patients developed cognitive impairment with a significantly higher frequency than HC of a similar age. Additionally, we found that these patients had a global pattern of cognitive impairment, including executive function, verbal memory and language. Only visuospatial function was more impaired in PD than in HC. Our data contribute to clarify the pattern of neuropsychological impairment in VP. Therefore, in the clinical evaluation, besides assessing the motor status of the patient, given that these symptoms are frequently found not to be self-reported complaints, the neurologist should evaluate them routinely as a comprehensive assessment of this disease. Copyright © 2014 Elsevier B.V. All rights reserved.

  2. Nonautistic motor stereotypies: clinical features and longitudinal follow-up.

    PubMed

    Harris, Kendra M; Mahone, E Mark; Singer, Harvey S

    2008-04-01

    To characterize further the clinical features and long-term outcomes among children with motor stereotypies who do not manifest mental retardation or pervasive developmental disorders, a review of clinical records and semistructured telephone interviews were undertaken. The identified clinical cohort consisted of 100 typically developing children with motor stereotypies. The mean length of follow-up was 6.8 +/- 4.6 years. At most recent follow-up, movements had continued in 94% of the sample (62% for >5 years). Only six children reported complete cessation of movements, with four (3 of 4 with head nodding) doing so >1 year after their initial diagnosis. Thus the course of motor stereotypies, especially in children with arm/hand movements, appears chronic. Nearly half the children in this cohort exhibit other comorbidities, including attention-deficit-hyperactivity disorder (30%), tics (18%), and obsessive-compulsive behaviors/obsessive-compulsive disorder (10%). Twenty-five percent of children with motor stereotypies reported positive family histories of motor stereotypies, suggesting an underlying genetic abnormality. Finally, evidence is emerging that the clinical course of children who exhibit head nodding may differ from those whose motor stereotypy predominantly involves the hands and arms.

  3. [Risk factors and clinical features of ectopic pregnancy].

    PubMed

    Escobar-Padilla, Beatriz; Perez-López, Carlos A; Martínez-Puon, Horacio

    2017-01-01

    Ectopic pregnancy (EP) is the most frequent cause of maternal death in the first trimester of pregnancy. The objective was to establish the clinical features and risk factors associated with EP. Observational, retrospective, transversal and analytic case-control study. Two groups were included: the cases group (28 patients) and the control group (56 postpartum patients). Univariate and bivariate descriptive statistical analysis were carried out using the Pearson chi-square test, p < 0.05, with odds ratios (OR) and 95% confidence intervals (95% CI). Statistically significant values (OR) were: smoking: 18.33, history of EP: 11.96, IUD use: 11.9, abdominal surgery: 5.87, being multigravid: 3.80, and having two or more sexual partners: 2. The most common clinical symptom was pelvic pain with 57.1% (16), right ruptured EP 53.6%, tubal pregnancy 82.1% (23), unruptured EP 60.7% (17), hemoperitoneum 60.7% (17). Gestational age for EP was of 4-8 weeks (75%) and surgical treatment 96.4%. The frequency of EP found in our population was 1 in every 122 live births. Risk factors associated with ectopic pregnancy with statistically higher values were: smoking, being multigravid, having a clinical record of EP, IUD use before conception, abdominal surgery. The more frequent clinical characteristics were pelvic pain, right EP, tubal pregnancy, EP with no ruptures, hemoperitoneum < 750 ml, a gestational age between four and eight weeks.

  4. Hypertrophic cardiomyopathy in infants: clinical features and natural history

    SciTech Connect

    Maron, B.J.; Tajik, A.J.; Ruttenberg, H.D.; Graham, T.P.; Atwood, G.F.; Victorica, B.E.; Lie, J.T.; Roberts, W.C.

    1982-01-01

    The clinical and morphologic features of hypertrophic cardiomyopathy in 20 patients recognized as having cardiac disease in the first year of life are described. Fourteen of these 20 infants were initially suspected of having heart disease solely because a heart murmur was identified. However, the infants showed a variety of clinical findings, including signs of marked congestive heart failure (in the presence of nondilated ventricular cavities and normal or increased left ventricular contractility) and substantial cardiac enlargement on chest radiograph. Other findings were markedly different from those usually present in older children and adults with hypertrophic cardiomyopathy (e.g., right ventricular hypertrophy on the ECG and cyanosis). Consequently, in 14 infants, the initial clinical diagnosis was congenital cardiac malformation other than hypertrophic cardiomyopathy. The clinical course was variable in these patients, but the onset of marked congestive heart failure in the first year of life appeared to be an unfavorable prognostic sign; nine of the 11 infants with congestive heart failure died within the first year of life. In infants with hypertrophic cardiomyopathy, unlike older children and adults with this condition, sudden death was less common (two patients) than death due to progressive congestive heart failure.

  5. The clinical features of the piriformis syndrome: a systematic review

    PubMed Central

    Song, Fujian; Riera, Ricardo; Sambandan, Sidha

    2010-01-01

    Piriformis syndrome, sciatica caused by compression of the sciatic nerve by the piriformis muscle, has been described for over 70 years; yet, it remains controversial. The literature consists mainly of case series and narrative reviews. The objectives of the study were: first, to make the best use of existing evidence to estimate the frequencies of clinical features in patients reported to have PS; second, to identify future research questions. A systematic review was conducted of any study type that reported extractable data relevant to diagnosis. The search included all studies up to 1 March 2008 in four databases: AMED, CINAHL, Embase and Medline. Screening, data extraction and analysis were all performed independently by two reviewers. A total of 55 studies were included: 51 individual and 3 aggregated data studies, and 1 combined study. The most common features found were: buttock pain, external tenderness over the greater sciatic notch, aggravation of the pain through sitting and augmentation of the pain with manoeuvres that increase piriformis muscle tension. Future research could start with comparing the frequencies of these features in sciatica patients with and without disc herniation or spinal stenosis. PMID:20596735

  6. Clinical features and treatment of obstructive sleep apnea.

    PubMed Central

    Kimoff, R J; Cosio, M G; McGregor, M

    1991-01-01

    OBJECTIVE: To review the clinical features and treatment of obstructive sleep apnea (OSA). DATA SOURCE AND SELECTION: All articles on OSA published in French and English between 1970 and 1990 and indexed in Index Medicus were reviewed. Studies addressing the epidemiologic features and clinical aspects of OSA were selected, and special emphasis was given to articles reporting the effects of treatment on morbidity and mortality rates. MAIN RESULTS: OSA is characterized by episodes of upper airway obstruction during sleep that result in repetitive hypoxemia and sleep disruption. OSA leads to various neuropsychologic and cardiovascular complications, including daytime hypersomnolence, cognitive impairment, systemic and pulmonary hypertension and cardiac arrhythmias. There is suggestive evidence that the death rate among affected people is increased. The true incidence of OSA is unknown, but estimates have varied from 1% upwards among men. The current treatment with the greatest overall effectiveness and acceptability is nasal continuous positive airway pressure. CONCLUSION: This common, readily treatable disorder is associated with serious complications and therefore must be widely recognized by health professionals. PMID:1998928

  7. Hereditary and Clinical Features of Retinitis Pigmentosa in Koreans

    PubMed Central

    Lee, Sun Ho; Seo, Jong Mo; Moon, Sang Woong; Moon, Jun Woong; Kim, Sang Jin; Chung, Hum

    2010-01-01

    There has been no report about hereditary and clinical features of retinitis pigmentosa (RP) in Koreans. To evaluate these, data were collected from 365 RP patients including age, gender, visual acuity (VA), spherical equivalent (SE) of refractive errors, funduscopic findings, color vision test, visual field score (VFS) obtained from Goldmann perimetry, and the inheritance patterns from pedigrees. Simplex RP was the most common inheritance pattern (61.9%); followed by autosomal recessive RP (17.3%), autosomal dominant RP (12.1%) and X-linked recessive RP (8.8%). Myopia was the most common refractive errors (77.5%) including 16.1% of high myopia. The most common cataract type was posterior subcapsular cataract (25.8%). Observed retinal findings included changes of retinal pigment epithelium (88.8%), bony spicule-like pigmentation (79.7%), attenuation of retinal vessel (76.2%), waxy disc pallor (12.6%), golden ring around optic disc (2.2%), epiretinal membrane (0.8%) and cystoid macular edema (0.5%). Corrected VA and refractive errors did not show any significant difference between the inheritance patterns. VFS was significantly worse in autosomal recessive RP than in autosomal dominant RP. Color vision defect was noted in 66.1% on Hardy-Rand-Rittlers color vision test. In conclusion, Korean RP patients have the indigenous hereditary and clinical features as well as the ordinary ones. PMID:20514315

  8. Hereditary and clinical features of retinitis pigmentosa in Koreans.

    PubMed

    Lee, Sun Ho; Yu, Hyeong Gon; Seo, Jong Mo; Moon, Sang Woong; Moon, Jun Woong; Kim, Sang Jin; Chung, Hum

    2010-06-01

    There has been no report about hereditary and clinical features of retinitis pigmentosa (RP) in Koreans. To evaluate these, data were collected from 365 RP patients including age, gender, visual acuity (VA), spherical equivalent (SE) of refractive errors, funduscopic findings, color vision test, visual field score (VFS) obtained from Goldmann perimetry, and the inheritance patterns from pedigrees. Simplex RP was the most common inheritance pattern (61.9%); followed by autosomal recessive RP (17.3%), autosomal dominant RP (12.1%) and X-linked recessive RP (8.8%). Myopia was the most common refractive errors (77.5%) including 16.1% of high myopia. The most common cataract type was posterior subcapsular cataract (25.8%). Observed retinal findings included changes of retinal pigment epithelium (88.8%), bony spicule-like pigmentation (79.7%), attenuation of retinal vessel (76.2%), waxy disc pallor (12.6%), golden ring around optic disc (2.2%), epiretinal membrane (0.8%) and cystoid macular edema (0.5%). Corrected VA and refractive errors did not show any significant difference between the inheritance patterns. VFS was significantly worse in autosomal recessive RP than in autosomal dominant RP. Color vision defect was noted in 66.1% on Hardy-Rand-Rittlers color vision test. In conclusion, Korean RP patients have the indigenous hereditary and clinical features as well as the ordinary ones.

  9. Prevalence and Clinical Features of Focal Takotsubo Cardiomyopathy.

    PubMed

    Kato, Ken; Kitahara, Hideki; Fujimoto, Yoshihide; Sakai, Yoshiaki; Ishibashi, Iwao; Himi, Toshiharu; Kobayashi, Yoshio

    2016-07-25

    Because it is difficult to distinguish between focal takotsubo cardiomyopathy and aborted myocardial infarction, there is little information about the prevalence and clinical features of focal takotsubo cardiomyopathy. Our cardiac catheterization databases were queried to identify patients with focal takotsubo cardiomyopathy and other types of takotsubo cardiomyopathy. We defined focal takotsubo cardiomyopathy as hypo-, a- or dyskinesis in both anterolateral and septal segments without obstructive coronary artery disease explaining the wall motion abnormality. A total of 10 patients were diagnosed with focal takotsubo cardiomyopathy. The control group comprised patients with takotsubo cardiomyopathy with apical, mid-ventricular, or basal ballooning. Clinical features and in-hospital outcomes were compared between patients with focal takotsubo cardiomyopathy and those with other types of takotsubo cardiomyopathy. Among the 144 patients with takotsubo cardiomyopathy, the apical, mid-ventricular, basal, and focal types occurred in 85 (59.0%), 49 (34.0%), 0 (0%), and 10 patients (6.9%), respectively. The left ventricular ejection fraction was significantly higher in the focal group compared with the apical and mid-ventricular group (56±13 vs. 45±13 vs. 46±12%, P=0.03). In-hospital outcome was not significantly different among the 3 groups. Focal takotsubo cardiomyopathy is not rare. Biplane left ventriculography is useful for its diagnosis. (Circ J 2016; 80: 1824-1829).

  10. Cockayne syndrome: Clinical features, model systems and pathways.

    PubMed

    Karikkineth, Ajoy C; Scheibye-Knudsen, Morten; Fivenson, Elayne; Croteau, Deborah L; Bohr, Vilhelm A

    2017-01-01

    Cockayne syndrome (CS) is a disorder characterized by a variety of clinical features including cachectic dwarfism, severe neurological manifestations including microcephaly and cognitive deficits, pigmentary retinopathy, cataracts, sensorineural deafness, and ambulatory and feeding difficulties, leading to death by 12 years of age on average. It is an autosomal recessive disorder, with a prevalence of approximately 2.5 per million. There are several phenotypes (1-3) and two complementation groups (CSA and CSB), and CS overlaps with xeroderma pigmentosum (XP). It has been considered a progeria, and many of the clinical features resemble accelerated aging. As such, the study of CS affords an opportunity to better understand the underlying mechanisms of aging. The molecular basis of CS has traditionally been ascribed to defects in transcription and transcription-coupled nucleotide excision repair (TC-NER). However, recent work suggests that defects in base excision DNA repair and mitochondrial functions may also play key roles. This opens up the possibility for molecular interventions in CS, and by extrapolation, possibly in aging. Published by Elsevier B.V.

  11. Clinical Features and Course of Ocular Toxocariasis in Adults

    PubMed Central

    Ahn, Seong Joon; Woo, Se Joon; Jin, Yan; Chang, Yoon-Seok; Kim, Tae Wan; Ahn, Jeeyun; Heo, Jang Won; Yu, Hyeong Gon; Chung, Hum; Park, Kyu Hyung; Hong, Sung Tae

    2014-01-01

    Purpose To investigate the clinical features, clinical course of granuloma, serologic findings, treatment outcome, and probable infection sources in adult patients with ocular toxocariasis (OT). Methods In this retrospective cohort study, we examined 101 adult patients diagnosed clinically and serologically with OT. Serial fundus photographs and spectral domain optical coherence tomography images of all the patients were reviewed. A clinic-based case-control study on pet ownership, occupation, and raw meat ingestion history was performed to investigate the possible infection sources. Results Among the patients diagnosed clinically and serologically with OT, 69.6% showed elevated immunoglobulin E (IgE) levels. Granuloma in OT involved all retinal layers and several vitreoretinal comorbidities were noted depending on the location of granuloma: posterior pole granuloma was associated with epiretinal membrane and retinal nerve fiber layer defects, whereas peripheral granuloma was associated with vitreous opacity. Intraocular migration of granuloma was observed in 15 of 93 patients (16.1%). Treatment with albendazole (400 mg twice a day for 2 weeks) and corticosteroids (oral prednisolone; 0.5–1 mg/kg/day) resulted in comparable outcomes to patients on corticosteroid monotherapy; however, the 6-month recurrence rate in patients treated with combined therapy (17.4%) was significantly lower than that in patients treated with corticosteroid monotherapy (54.5%, P = 0.045). Ingestion of raw cow liver (80.8%) or meat (71.2%) was significantly more common in OT patients than healthy controls. Conclusions Our study discusses the diagnosis, treatment, and prevention strategies for OT. Evaluation of total IgE, in addition to anti-toxocara antibody, can assist in the serologic diagnosis of OT. Combined albendazole and corticosteroid therapy may reduce intraocular inflammation and recurrence. Migrating feature of granuloma is clinically important and may further suggest the

  12. Effectiveness of applying flipped learning to clinical nursing practicums for nursing students in Korea: A randomized controlled trial.

    PubMed

    Kim, Hyun Sook; Kim, Mi Young; Cho, Mi-Kyoung; Jang, Sun Joo

    2017-07-25

    The purpose of this study was to develop flipped learning models for clinical practicums and compare their effectiveness regarding learner motivation toward learning, satisfaction, and confidence in performing core nursing skills among undergraduate nursing students in Korea. This study was a randomized clinical trial designed to compare the effectiveness of 2 flipped learning models. Data were collected for 3 days from October 21 to 23, 2015 before the clinical practicum was implemented and for 2 weeks from October 26 to December 18, 2015 during the practicum period. The confidence of the students in performing core nursing skills was likely to increase after they engaged in the clinical practicum in both study groups. However, while learner confidence and motivation were not affected by the type of flipped learning, learner satisfaction did differ between the 2 groups. The findings indicate that applying flipped learning allows students to conduct individualized learning with a diversity of clinical cases at their own level of understanding and at their own pace before they participate in real-world practicums. © 2017 John Wiley & Sons Australia, Ltd.

  13. Headache associated with sexual activity: demography, clinical features, and comorbidity.

    PubMed

    Frese, A; Eikermann, A; Frese, K; Schwaag, S; Husstedt, I-W; Evers, S

    2003-09-23

    S: To provide data on the demography, clinical features, and comorbidity of headache associated with sexual activity (HSA). Between 1996 and 2001, 51 patients with the diagnosis of HSA were questioned using a structured interview. The mean age at onset was 39.2 (+/-11.1) years. There was a clear male preponderance (2.9:1). The age at onset had two peaks, with a first peak between the 20th and 24th (n = 13) years of life and a second peak between the 35th and 44th (n = 20) years of life. Eleven patients had HSA type 1 (dull subtype), which gradually increased with increasing sexual excitement. The remaining (n = 40) had HSA type 2 (explosive subtype). The pain was predominantly bilateral (67%), and diffuse or occipital (76%). The quality was nearly equally distributed among dull, throbbing, and stabbing. HSA was not dependent on specific sexual habits and most often occurred during sexual activity with the usual partner (94%) and during masturbation (35%). There was a high comorbidity with migraine (25%), benign exertional headache (29%), and tension-type headache (45%). HSA types 1 and 2 did not significantly differ in demography, clinical features, or comorbidity, except for a higher probability of stopping the attack by breaking off sexual activity in HSA type 1. There were no cases with HSA type 3 (postural subtype). Mean age at onset, a male preponderance, a predominantly bilateral and occipital pain, and a high comorbidity with other primary headaches are in concordance with case reports in the literature. The authors found two peaks for the age at onset, however. There was no clinical evidence proving subtypes 1 and 2 to be distinct disorders. HSA types 1 and 2 may be different manifestations of the same disease rather than distinct entities.

  14. Clinical and metabolic features of urolithiasis and microlithiasis in children.

    PubMed

    Alpay, Harika; Ozen, Ahmet; Gokce, Ibrahim; Biyikli, Nese

    2009-11-01

    We evaluated the clinical, radiological and metabolic features of 162 children with urolithiasis or microlithiasis who had been referred to our pediatric nephrology clinics between 1998 and 2008 with suspected urolithiasis. The medical histories of these children (78 girls, 84 boys), who ranged in age from 2 months to 16 years (mean age 5.59 +/- 0.35 years), were reviewed retrospectively for clinical and metabolic features of urinary tract calculi. Urinary tract infections (UTI) were present in 45.9% of the cases. The most common presenting symptoms were flank pain or restlessness (25.3%) and hematuria (21.6%), followed by UTI (16%), whereas 23.5% of the cases were detected incidentally during evaluation for other medical conditions. Other symptoms at presentation included dysuria, passing stones, penile edema, enuresis, vomiting and anorexia. Urine analysis revealed metabolic abnormalities in 87% of the cases, including hypercalciuria (33.8%), hypocitraturia (33.1%), hyperoxaluria (26.5%), hyperuricosuria (25.4%), hypocitraturia + hypercalciuria (21.1%), hyperphosphaturia (20.8%) and cystinuria (5.7%). Almost 50% of the patients had a positive family history for urolithiasis. The most frequently involved site was in the kidneys (86%). Ureters and bladder were involved in 12 and 2% of the cases, respectively. A family history of urolithiasis, presenting symptoms and underlying metabolic abnormalities were similar for microlithiasis and the patients with larger stones. However, in our study population, microlithiasis was mainly a disease of young infants, with a greater chance for remission and often not associated with structural changes. The presenting symptoms of urolithiasis show a wide spectrum, so that a high index of suspicion is important for early detection. A metabolic abnormality can be identified in 87% of cases of urolithiasis. Detection of microlithiasis may explain a number of symptoms, thus reducing invasive diagnostic procedures and allowing early

  15. Cardioembolic Stroke: Clinical Features, Specific Cardiac Disorders and Prognosis

    PubMed Central

    Arboix, Adrià; Alió, Josefina

    2010-01-01

    This article provides the reader with an overview and up-date of clinical features, specific cardiac disorders and prognosis of cardioembolic stroke. Cardioembolic stroke accounts for 14-30% of ischemic strokes and, in general, is a severe condition; patients with cardioembolic infarction are prone to early and long-term stroke recurrence, although recurrences may be preventable by appropriate treatment during the acute phase and strict control at follow-up. Certain clinical features are suggestive of cardioembolic infarction, including sudden onset to maximal deficit, decreased level of consciousness at onset, Wernicke’s aphasia or global aphasia without hemiparesis, a Valsalva manoeuvre at the time of stroke onset, and co-occurrence of cerebral and systemic emboli. Lacunar clinical presentations, a lacunar infarct and especially multiple lacunar infarcts, make cardioembolic origin unlikely. The more common high risk cardioembolic conditions are atrial fibrillation, recent myocardial infarction, mechanical prosthetic valve, dilated myocardiopathy, and mitral rheumatic stenosis. Transthoracic and transesophageal echocardiogram can disclose structural heart diseases. Paroxysmal atrial dysrhyhtmia can be detected by Holter monitoring. In-hospital mortality in cardioembolic stroke (27.3%, in our series) is the highest as compared with other subtypes of cerebral infarction. In our experience, in-hospital mortality in patients with early embolic recurrence (within the first 7 days) was 77%. Patients with alcohol abuse, hypertension, valvular heart disease, nausea and vomiting, and previous cerebral infarction are at increased risk of early recurrent systemic embolization. Secondary prevention with anticoagulants should be started immediately if possible in patients at high risk for recurrent cardioembolic stroke in which contraindications, such as falls, poor compliance, uncontrolled epilepsy or gastrointestinal bleeding are absent. PMID:21804774

  16. Clinical features of headache at altitude: a prospective study.

    PubMed

    Silber, E; Sonnenberg, P; Collier, D J; Pollard, A J; Murdoch, D R; Goadsby, P J

    2003-04-08

    Headache is the most common nervous system complication at altitude; however, there have been few attempts to characterize clinical features of high-altitude headaches (HAH). To measure prospectively the incidence of HAH and to determine its risk factors and characteristics. Members of an expedition to Kanchenjunga base camp in Nepal (5,100 m) were prospectively studied. Subjects were interviewed prior to the trip and while trekking recorded headaches experienced at >3,000 m using a structured questionnaire incorporating International Headache Society (IHS) and acute mountain sickness (AMS) criteria. In addition, clinical features of headaches in 19 trekkers in other groups above 3,000 m were recorded using the same questionnaire. Eighty-three percent (50/60) reported at least 1 HAH (median 2, range 0 to 10). Those who developed HAH were younger (p = 0.04); women and persons with headaches in daily life were more likely to report severe headaches (p = 0.03 and p = 0.07). One hundred thirty-eight HAH, experienced by 69 persons, are described. The mean altitude at which headaches occurred was 4,723 m. Twenty-six percent of headaches woke subjects at night or occurred upon awakening. HAH reported by migraineurs were accompanied by more phonophobia (p = 0.008). There were no IHS accompanying symptoms in 44% of headaches or symptoms of AMS in 52% of headaches. Headaches are a frequent complication of ascent to altitude. Older age appears to offer some protection, whereas headaches were more severe in women and persons with headaches in daily life. There is a wide clinical spectrum, with some suggesting intracranial hypertension. There is a need for evidence-based diagnostic criteria for headaches at altitude.

  17. Pediatric surface osteosarcoma: clinical, pathologic, and radiologic features.

    PubMed

    Kaste, Sue C; Fuller, Christine E; Saharia, Ashish; Neel, Michael D; Rao, Bhaskar N; Daw, Najat C

    2006-08-01

    Osteosarcoma (OS) arising from the surface of bone is far less common than its intramedullary counterpart. Although surface OSs share some radiographic and clinical features, they can be divided into three distinct histologic subtypes. We reviewed the clinical, radiographic, and pathologic features of 14 cases of pediatric surface OS treated at St. Jude Children's Research Hospital between 1970 and 2003. Seven patients had parosteal, five had periosteal, and two had high-grade surface OS. The median age at diagnosis was 16.2 years (range, 13.6-18.5 years). Nine patients were male; 11 were Caucasian. None had metastatic disease at diagnosis. Primary tumor sites included distal femur (n = 6), mid to proximal femur (n = 4), and mid to proximal tibia (n = 4). All 14 patients were treated with surgery, and 7 (1 with parosteal, 4 with periosteal, 2 with high-grade tumors) received chemotherapy. One patient experienced pulmonary metastasis of periosteal OS 16 months and 43 months after diagnosis; long-term disease-free survival followed resection of the metastatic tumors. Twelve patients remained alive and disease-free a median of 10 years (range, 1.5-25.4 years) after diagnosis. One patient died of high-grade surface OS 1.8 years after diagnosis, and one patient with periosteal OS died of gastric cancer 18.2 years after diagnosis of OS. The histologic grade predicts the clinical behavior of pediatric surface OS. Complete resection is the treatment of choice regardless of tumor subtype. Whereas chemotherapy is not indicated for parosteal OS, its role in periosteal OS remains controversial.

  18. Novel clinical features of recurrent human respiratory syncytial virus infections.

    PubMed

    Yui, Ikuko; Fujino, Motoko; Sawada, Akihito; Nakayama, Tetsuo

    2014-09-01

    Children and elderly individuals are often infected easily and repeatedly with human respiratory syncytial virus (HRSV); however, the features of recurrent infection in the same individual are defined poorly. To clarify the clinical significance of repeated HRSV infections in relation to subgroup epidemiology, this study performed prospective and longitudinal analyses in children with lower respiratory tract infections over 20 consecutive epidemics between 1985 and 2005 at a pediatric outpatient clinic in Kawasaki, Japan. HRSV infections were confirmed by 2 types of reverse-transcription PCR. Samples obtained from patients with repeated infections were subjected to sequence analysis and cloning analysis. A total of 1,312 lower respiratory tract infections observed in 1,010 patients were diagnosed as HRSV infections. Repeated HRSV infections occurred in 208 of the 1,010 patients. Analysis of the patients with repeated infections revealed that children were often infected multiple times even within a single short epidemic. Some patients were re-infected with strains having the same or virtually identical N gene sequences. In patients infected more than 4 times, cloning analysis revealed more frequent dual infections with both subgroups (23.8%). The HRSV-A subgroup caused subsequent homologous infections more frequently than did HRSV-B; furthermore, HRSV-A infections provided no protection from a second homologous infection. In contrast, HRSV-B infections offered significant protection against a second homologous infection. Statistical analysis revealed alleviation of symptoms with a reduced rate of dyspnoeic attacks only in the group re-infected with homologous HRSV-A strains. Thus, this study elucidates new clinical features of recurrent HRSV infection.

  19. Clinical features of idiopathic inflammatory polymyopathy in the Hungarian Vizsla.

    PubMed

    Tauro, Anna; Addicott, Diane; Foale, Rob D; Bowman, Chloe; Hahn, Caroline; Long, Sam; Massey, Jonathan; Haley, Allison C; Knowler, Susan P; Day, Michael J; Kennedy, Lorna J; Rusbridge, Clare

    2015-04-21

    A retrospective study of the clinicopathological features of presumed and confirmed cases of idiopathic inflammatory polymyopathy in the Hungarian Vizsla dog and guidelines for breeding. 369 medical records were reviewed (1992-2013) and 77 Hungarian Vizslas were identified with a case history consistent with idiopathic inflammatory polymyopathy. Inclusion criteria were: group 1 (confirmed diagnosis); histopathology and clinical findings compatible with an inflammatory polymyopathy and group 2 (probable diagnosis); clinical findings compatible with a polymyopathy including dysphagia, sialorrhea, temporal muscle atrophy, elevated serum creatine kinase (CK) activity, and sufficient clinical history to suggest that other neuromuscular disorders could be ruled out. Some group 2 dogs had muscle biopsy, which suggested muscle disease but did not reveal an inflammatory process. The mean age of onset was 2.4 years; male dogs were slightly overrepresented. Common presenting signs were dysphagia, sialorrhea, masticatory muscle atrophy, and regurgitation. Common muscle histopathological findings included degenerative and regenerative changes, with multifocal mononuclear cell infiltration with lymphoplasmacytic myositis of variable severity. A positive response to immunosuppressive treatment supported an immune-mediated aetiology. The mean age at death and survival time were 6.4 and 3.9 years, respectively. Recurrence of clinical signs and aspiration pneumonia were common reasons for euthanasia. Diagnosis of Vizsla idiopathic inflammatory polymyopathy can be challenging due to lack of specific tests, however the presence of dysphagia, regurgitation and masticatory muscle atrophy in this breed with negative serological tests for masticatory muscle myositis and myasthenia gravis, along with muscle biopsies suggesting an inflammatory process, support the diagnosis. However, there is an urgent need for a more specific diagnostic test. The average of inbreeding coefficient (Co

  20. [Synopsis on clinical practice guideline of gastric cancer in Korea: an evidence-based approach].

    PubMed

    Lee, Jun Haeng; Kim, Jae G; Jung, Hye Kyung; Kim, Jung Hoon; Jeong, Woo Kyoung; Jeon, Tae Joo; Kim, Joon Mee; Kim, Young Il; Ryu, Keun Won; Kong, Seong-Ho; Kim, Hyoung Il; Jung, Hwoon-Yong; Kim, Yong Sik; Zang, Dae Young; Cho, Jae Yong; Park, Joon Oh; Lim, Do Hoon; Jung, Eun Sun; Ahn, Hyeong Sik; Kim, Hyun Jung

    2014-02-01

    Although, gastric cancer is quite common in Korea, the treatment outcome is relatively favorable compared to that of Western countries. However, there is no Korean multidisciplinary guideline for gastric cancer and thus, a guideline adequate for domestic circumstances is required. Experts from related societies developed 22 recommendation statements for the diagnosis (n=9) and treatment (n=13) based on relevant key questions. Evidence levels based on systematic review of literatures were classified as five levels from A to E, and recommendation grades were classified as either strong or weak. The topics of this guideline cover diagnostic modalities (endoscopy, endoscopic ultrasound, radiologic diagnosis), treatment modalities (surgery, therapeutic endoscopy, chemotherapy, radiotherapy) and pathologic evaluation. External review of the guideline was conducted at the finalization phase.

  1. Clinical Practice Guidelines for the Management of Atypical Hemolytic Uremic Syndrome in Korea

    PubMed Central

    2016-01-01

    Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome characterized by micro-angiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. The major pathogenesis of aHUS involves dysregulation of the complement system. Eculizumab, which blocks complement C5 activation, has recently been proven as an effective agent. Delayed diagnosis and treatment of aHUS can cause death or end-stage renal disease. Therefore, a diagnosis that differentiates aHUS from other forms of thrombotic microangiopathy is very important for appropriate management. These guidelines aim to offer recommendations for the diagnosis and treatment of patients with aHUS in Korea. The guidelines have largely been adopted from the current guidelines due to the lack of evidence concerning the Korean population. PMID:27550478

  2. Primary breast sarcoma: prevalence, clinical signs, and radiological features.

    PubMed

    Surov, Alexey; Holzhausen, Hans-Jürgen; Ruschke, Kathrin; Spielmann, Rolf Peter

    2011-07-01

    Primary breast sarcoma is very rare. Most reports regarding sarcoma of the breast are clinical observations or pathological series and provide either no or inconstant radiological information. Radiological publications consist predominantly of isolated case reports or small series. To determine the prevalence, clinical signs, and radiological features of primary breast sarcoma. This is a retrospective review of 21 patients with breast sarcoma. All patients were female and their median age was 66 years (range 27-86). In all patients the diagnosis was confirmed histopathologically. The prevalence of breast sarcoma was 0.1% of all identified cases with breast malignancies. Clinically, all patients presented with solitary painless breast lumps. There was no uni- or bilateral axillary lymphadenopathy. On mammography (n = 19), two mammographic patterns could be identified: breast masses (68%), and architectural distortion (32%). On ultrasound (n = 8), most lesions were homogeneously hypoechoic, lobular or oval in shape with microlobulated or indistinct margins. On magnetic resonance imaging (n = 3), marked inhomogeneous contrast enhancement was seen in all investigated cases. The imaging findings of primary breast sarcoma are not pathognomonic. However, they should be taken into consideration in the differential diagnosis of breast lesions.

  3. Prevalence, clinical, endoscopic and pathological features of duodenitis in children

    PubMed Central

    Alper, Arik; Hardee, Steven; Rojas-velasquez, Danilo; Escalera, Sandra; Morotti, Raffaella A; Pashankar, Dinesh S.

    2015-01-01

    Objectives While gastritis and esophagitis are well studied in children, there is very limited literature on duodenitis in children. We aimed to assess the prevalence, etiology, clinical, endoscopic, and pathological features in a large cohort of unselected children with duodenitis. Methods We reviewed the pathology reports of all upper endoscopies performed at our institution over 5 years to identify children with duodenitis. Biopsy sections were reviewed to confirm the diagnosis of duodenitis. Demographic, clinical, endoscopic data and presence of associated gastritis and esophagitis were noted in all children with duodenitis. Etiology of duodenitis was correlated with the patient's clinical diagnosis. Results Out of 2772 children who had endoscopy, 352 had duodenitis with the prevalence rate of 12.7%. Gastritis was seen in 64% of children with duodenitis compared with 46% of children without duodenitis (P < 0.001). Common indications for endoscopy in children with duodenitis were abdominal pain, positive celiac serology and diarrhea. The most common etiology was celiac disease (32%), followed by Crohn's disease (13%), ulcerative colitis (3%), and Helicobacter pylori infection (6%). In 63% of cases, endoscopic appearance of duodenum was normal. Cryptitis, villous changes and cellular infiltration were noted on histology. Conclusions Prevalence of duodenitis is 12.7% in children undergoing endoscopy. Celiac disease and inflammatory bowel disease are common causes of duodenitis. Associated gastritis is common in children with duodenitis and correlation of endoscopic appearance with histology is poor. PMID:26252915

  4. Clinical Features of Severe or Fatal Mycoplasma pneumoniae Pneumonia

    PubMed Central

    Izumikawa, Koichi

    2016-01-01

    Mycoplasma pneumoniae is one of the most common causes of community-acquired pneumonia in children and young adults. The incidence of fulminant M. pneumoniae pneumonia (MPP) is relatively rare despite the high prevalence of M. pneumoniae infection. This literature review highlights the clinical features of fulminant MPP by examining the most recent data in epidemiology, clinical presentation, pathogenesis, and treatment. Fulminant MPP accounts for 0.5–2% of all MPP cases and primarily affects young adults with no underlying disease. Key clinical findings include a cough, fever, and dyspnea along with diffuse abnormal findings in radiological examinations. Levels of inflammatory markers such as white blood cells and C-reactive protein are elevated, as well as levels of lactate dehydrogenase, IL-18, aspartate transaminase, and alanine transaminase. The exact pathogenesis of fulminant MPP remains unclear, but theories include a delayed hypersensitivity reaction to M. pneumoniae and the contribution of delayed antibiotic administration to disease progression. Treatment options involve pairing the appropriate anti-mycoplasma agent with a corticosteroid that will downregulate the hypersensitivity response, and mortality rates are quite low in this treatment group. Further research is necessary to determine the exact pathogenesis of severe and fulminant types of MPP. PMID:27313568

  5. [Clinical features of reversed halo sign in cryptogenic organizing pneumonia].

    PubMed

    Inoue, Yukihisa; Yoshimura, Nobuyuki; Shimada, Hiroyuki; Saito, Hiroaki; Shimizu, Kyouko; Kurata, Kiyoko; Kobayashi, Akiko; Yamazaki, Keiichi; Jin, Yasuto; Inase, Naohiko

    2011-02-01

    Reversed halo sign (RHS) is often seen in computed tomography (CT) scans of cryptogenic organizing pneumonia (COP). To investigate its clinical features, we retrospectively reviewed 30 cases of COP in 13 men and 17 women, whose age range 28 to 73, with a mean of 58.4 years. All diagnoses were made with transbronchial lung biopsy (TBLB), but it took an average of 24.8 days from the first visit until the diagnosis of COP. RHS was seen in 7 cases (23%) and multiple RHSs were seen in 3 cases. We treated 5 cases (71%) with steroids. Their CT images showed parenchymal abnormalities which started as nodular lesions, then enlarged, and then the central lesion changed into ground-glass opacities, until finally, RHS was formed. The presence of RHS does not necessarily indicate a marked difference in the clinical course of COP. In conclusion, in the present series RHS was a phase of the clinical course of COP, and was useful to diagnose COP.

  6. The clinical features of the overlap between COPD and asthma

    PubMed Central

    2011-01-01

    Background The coexistence of COPD and asthma is widely recognized but has not been well described. This study characterizes clinical features, spirometry, and chest CT scans of smoking subjects with both COPD and asthma. Methods We performed a cross-sectional study comparing subjects with COPD and asthma to subjects with COPD alone in the COPDGene Study. Results 119 (13%) of 915 subjects with COPD reported a history of physician-diagnosed asthma. These subjects were younger (61.3 vs 64.7 years old, p = 0.0001) with lower lifetime smoking intensity (43.7 vs 55.1 pack years, p = 0.0001). More African-Americans reported a history of asthma (33.6% vs 15.6%, p < 0.0001). Subjects with COPD and asthma demonstrated worse disease-related quality of life, were more likely to have had a severe COPD exacerbation in the past year, and were more likely to experience frequent exacerbations (OR 3.55 [2.19, 5.75], p < 0.0001). Subjects with COPD and asthma demonstrated greater gas-trapping on chest CT. There were no differences in spirometry or CT measurements of emphysema or airway wall thickness. Conclusion Subjects with COPD and asthma represent a relevant clinical population, with worse health-related quality of life. They experience more frequent and severe respiratory exacerbations despite younger age and reduced lifetime smoking history. Trial registration ClinicalTrials.gov: NCT00608764 PMID:21951550

  7. Genetic Epidemiology, Hematological and Clinical Features of Hemoglobinopathies in Iran

    PubMed Central

    Rahimi, Zohreh

    2013-01-01

    There is large variation in the molecular genetics and clinical features of hemoglobinopathies in Iran. Studying structural variants of hemoglobin demonstrated that the β-chain variants of hemoglobin S and D-Punjab are more prevalent in the Fars (southwestern Iran) and Kermanshah (western Iran) provinces, respectively. Also, α-chain variants of Hb Q-Iran and Hb Setif are prevalent in western Iran. The molecular basis and clinical severity of thalassemias are extremely heterogenous among Iranians due to the presence of multiethnic groups in the country. β-Thalassemia is more prevalent in northern and southern Iran. Among 52 different β-thalassemia mutations that have been identified among Iranian populations, IVSII-1 G:A is the most frequent mutation in most parts of the country. The presence of IVS I-5 G:C mutation with high frequency in southeastern Iran might reflect gene flow from neighboring countries. A wide spectrum of α-thalassemia alleles has been detected among Iranians with −α 3.7 kb as the most prevalent α-thalassemia mutation. The prevention program of thalassemia birth in Iran has reduced the birth rate of homozygous β-thalassemia since the implementation of the program in 1997. In this review genetic epidemiology, clinical and hematological aspects of hemoglobinopathies, and the prevention programs of β-thalassemia in Iran will be discussed. PMID:23853772

  8. Genetic epidemiology, hematological and clinical features of hemoglobinopathies in Iran.

    PubMed

    Rahimi, Zohreh

    2013-01-01

    There is large variation in the molecular genetics and clinical features of hemoglobinopathies in Iran. Studying structural variants of hemoglobin demonstrated that the β-chain variants of hemoglobin S and D-Punjab are more prevalent in the Fars (southwestern Iran) and Kermanshah (western Iran) provinces, respectively. Also, α-chain variants of Hb Q-Iran and Hb Setif are prevalent in western Iran. The molecular basis and clinical severity of thalassemias are extremely heterogenous among Iranians due to the presence of multiethnic groups in the country. β-Thalassemia is more prevalent in northern and southern Iran. Among 52 different β-thalassemia mutations that have been identified among Iranian populations, IVSII-1 G:A is the most frequent mutation in most parts of the country. The presence of IVS I-5 G:C mutation with high frequency in southeastern Iran might reflect gene flow from neighboring countries. A wide spectrum of α-thalassemia alleles has been detected among Iranians with -α(3.7 kb) as the most prevalent α-thalassemia mutation. The prevention program of thalassemia birth in Iran has reduced the birth rate of homozygous β-thalassemia since the implementation of the program in 1997. In this review genetic epidemiology, clinical and hematological aspects of hemoglobinopathies, and the prevention programs of β-thalassemia in Iran will be discussed.

  9. Extraocular muscle cysticercosis: clinical features and management outcome.

    PubMed

    Murthy, Ramesh; Samant, Monica

    2008-01-01

    Extraocular muscle cysticercosis is rare and can be classified based on the stage of evolution as viable, degenerating, and inactive. A tailored approach to therapy using a combination of albendazole and steroids has been proposed. In this study, we have evaluated the clinical features and response of "viable" extraocular muscle cysticercosis to a tailored therapy with oral albendazole and steroids. A retrospective review of 32 patients with viable extraocular muscle cysticercosis was performed over a 3-year period. Ultrasound examination was performed at 3 weeks and 6 weeks for resolution of the cyst and clinical signs following treatment with oral albendazole and prednisolone. The clinical presentation, ocular alignment, motility restriction, and ultrasound examination details were recorded on serial visits. The mean age was 19.5 years. The most common presenting signs included limitation of ocular motility in 75%, conjunctival mass in 37.5%, diplopia in 28.1%, and proptosis in 28.1% of the patients, respectively. Ocular alignment evaluation revealed esotropia in 3.1%, exotropia in 12.5%, and hypertropia in 6.25%. Restriction of abduction and abduction in elevation was present in two patients each. The average time for complete resolution was 65.9 days. All the patients had resolution, except four, who had residual motility limitation but were asymptomatic. Combination therapy with oral albendazole and steroids is effective in the management of viable extraocular muscle cysticercosis. However, residual motility restriction may persist despite treatment.

  10. Clinical features and course of bacterial meningitis in children.

    PubMed

    Juganariu, Gabriela; Miftode, Egidia; Teodor, Danuţ; Leca, Daniela; Dorobăţ, Carmen-Mihaela

    2012-01-01

    To analyze the clinical features and course of and to define the risk factors for bacterial meningitis in children. Retrospective study of 100 cases of bacterial meningitis in patients aged 0-18 years admitted to the Iasi Infectious Diseases Hospital between 2005 and 2010. We found a clear prevalence in male children (58%) from rural area (67%), with the highest incidence in the age group 2-5 years. A significant percentage of patients (43%) had previous hospitalization, condition which is known as predisposing factor for bacterial meningitis, the most common being ear infections (20%) and height and weight deficit (9%). 71% of patients were admitted within the first 48 h. The most common onset clinical manifestations were fever (84%), vomiting (70%), signs of meningeal irritation (59%), somnolence (23%), loss of appetite (19%), and coma in 5% of patients. In 36% of cases CSF was opalescent with moderate pleocytosis (35%); in 29% of patients CSF albumin level ranged between 0.7-1.0 g, the majority presenting normal glycorahia (71%). In only 21% of cases the microbial agent was identified (pneumococcal and meningococcal etiology, 8% and 6%, respectively). The course was generally favorable, and mortality rate was low (5%). Complications occurred in 3% of patients consisting in hydrocephalus and brain abscess. Bacterial meningitis remains a disease with potentially severe course. Clinical onset, most commonly atypical in children, requires differential diagnosis at the time of admission in order to initiate the most appropriate antibiotic therapy.

  11. The clinical features of the overlap between COPD and asthma.

    PubMed

    Hardin, Megan; Silverman, Edwin K; Barr, R Graham; Hansel, Nadia N; Schroeder, Joyce D; Make, Barry J; Crapo, James D; Hersh, Craig P

    2011-09-27

    The coexistence of COPD and asthma is widely recognized but has not been well described. This study characterizes clinical features, spirometry, and chest CT scans of smoking subjects with both COPD and asthma. We performed a cross-sectional study comparing subjects with COPD and asthma to subjects with COPD alone in the COPDGene Study. 119 (13%) of 915 subjects with COPD reported a history of physician-diagnosed asthma. These subjects were younger (61.3 vs 64.7 years old, p=0.0001) with lower lifetime smoking intensity (43.7 vs 55.1 pack years, p=0.0001). More African-Americans reported a history of asthma (33.6% vs 15.6%, p<0.0001). Subjects with COPD and asthma demonstrated worse disease-related quality of life, were more likely to have had a severe COPD exacerbation in the past year, and were more likely to experience frequent exacerbations (OR 3.55 [2.19, 5.75], p<0.0001). Subjects with COPD and asthma demonstrated greater gas-trapping on chest CT. There were no differences in spirometry or CT measurements of emphysema or airway wall thickness. Subjects with COPD and asthma represent a relevant clinical population, with worse health-related quality of life. They experience more frequent and severe respiratory exacerbations despite younger age and reduced lifetime smoking history. ClinicalTrials.gov: NCT00608764.

  12. Meningioangiomatosis. A comprehensive analysis of clinical and laboratory features.

    PubMed

    Wiebe, S; Munoz, D G; Smith, S; Lee, D H

    1999-04-01

    Meningioangiomatosis (MA) is a rare, benign, focal lesion of the leptomeninges and underlying cerebral cortex characterized by leptomeningeal and meningovascular proliferation. It may occur sporadically or in association with neurofibromatosis type 2. Previous reports have emphasized histological and imaging features. Data on the management of these patients are sparse, and electrophysiological features of MA lesions have not been published. We assessed the clinical, electrophysiological, histopathological and imaging features as well as the surgical outcome in MA, and compared MA with and without neurofibromatosis. Seven patients with MA at our centre were investigated and their outcome was assessed. A review of the literature is included. MA exhibits a wide range of clinical, imaging, histopathological and electrophysiological features, making the diagnosis difficult. Sporadic MA cases are not associated with neurofibromatosis and the two disorders are genetically distinct. Medically refractory, localization-related epilepsy is the commonest presentation in sporadic cases, but atypical presentations also occur. Unlike sporadic cases, MA with neurofibromatosis is often found incidentally, does not produce seizures, occurs less frequently (ratio of 1:4), and is multifocal. MRI findings in MA correspond to the histological picture. However, the appearance on imaging is non-specific and may suggest cystic atrophy, angioma and tumours. Several abnormalities have been found in close proximity to MA lesions, i.e. meningioma, oligodendroglioma, arteriovenous malformation, encephalocoel and orbital erosion. In spite of histopathological diversity, MA lesions are either predominantly cellular or vascular. Immunohistochemical results are inconsistent among cases, add little to the diagnosis, and do not support a meningeal origin. Electrocorticographic recordings from the surface and within MA lesions revealed a spectrum of electrophysiological expressions. Intrinsic

  13. Survey of Clinical Laboratory Practices for 2015 Middle East Respiratory Syndrome Coronavirus Outbreak in the Republic of Korea

    PubMed Central

    Lee, Mi-Kyung; Kim, Sinyoung; Kim, Mi-Na; Kweon, Oh Joo; Lim, Yong Kwan; Ki, Chang-Seok; Kim, Jae-Seok; Seong, Moon-Woo; Sung, Heungsup; Yong, Dongeun; Lee, Hyukmin; Choi, Jong-Rak

    2016-01-01

    Background It is crucial to understand the current status of clinical laboratory practices for the largest outbreak of Middle East respiratory syndrome coronavirus (MERS-CoV) infections in the Republic of Korea to be well prepared for future emerging infectious diseases. Methods We conducted a survey of 49 clinical laboratories in medical institutions and referral medical laboratories. A short questionnaire to survey clinical laboratory practices relating to MERS-CoV diagnostic testing was sent by email to the directors and clinical pathologists in charge of the clinical laboratories performing MERS-CoV testing. The survey focused on testing volume, reporting of results, resources, and laboratory safety. Results A total of 40 clinical laboratories responded to the survey. A total of 27,009 MERS-CoV real-time reverse transcription PCR (rRT-PCR) tests were performed. Most of the specimens were sputum (73.5%). The median turnaround time (TAT) was 5.29 hr (first and third quartile, 4.11 and 7.48 hr) in 26 medical institutions. The median TAT of more than a half of the laboratories (57.7%) was less than 6 hr. Many laboratories were able to perform tests throughout the whole week. Laboratory biosafety preparedness included class II biosafety cabinets (100%); separated pre-PCR, PCR, and post-PCR rooms (88.6%); negative pressure pretreatment rooms (48.6%); and negative pressure sputum collection rooms (20.0%). Conclusions Clinical laboratories were able to quickly expand their diagnostic capacity in response to the 2015 MERS-CoV outbreak. Our results show that clinical laboratories play an important role in the maintenance and enhancement of laboratory response in preparation for future emerging infections. PMID:26709263

  14. Clinical features of catheter-related candidemia at disease onset

    PubMed Central

    Yoshino, Yusuke; Wakabayashi, Yoshitaka; Suzuki, Satoshi; Seo, Kazunori; Koga, Ichiro; Kitazawa, Takatoshi; Okugawa, Shu; Ota, Yasuo

    2014-01-01

    INTRODUCTION Early detection of catheter-related candidemia is necessary to ensure that patients receive prompt and appropriate treatment. The aim of the present case-control study is to investigate the clinical features of catheter-related candidemia at disease onset, so as to determine the clinical indications for empiric antifungal therapy. METHODS All 41 cases of catheter-related candidemia from September 2009 to August 2011 at a teaching hospital were included in the present study. To determine the characteristics that were risk factors for developing catheter-related candidemia, we compared all cases of catheter-related candidemia with all 107 cases of catheter-related blood stream infection (CRBSI) caused by non-Candida spp. RESULTS In comparison with CRBSI due to non-Candida spp., the duration of catheter use was significantly longer in cases of catheter-related candidemia (13.9 ± 9.0 days vs. 23.2 ± 25.2 days). There was also a significant difference in the frequency of pre-antibiotic treatment between catheter-related candidemia and CRBSI due to non-Candida spp. (97.6% [40/41 cases] vs. 44.9% [48/107 cases]). Patients with catheter-related candidemia also had significantly more severe clinical statuses (measured using the Sepsis-related Organ Failure Assessment score) than patients with CRBSI due to non-Candida spp. (7.63 ± 3.65 vs. 5.92 ± 2.81). CONCLUSION When compared to patients with CRBSI caused by non-Candida spp., patients with catheter-related candidemia had significantly more severe clinical backgrounds, longer duration of catheter use and more frequent prior administration of antibiotic agents. PMID:25631968

  15. Clinical and microbiological features of maternal sepsis: a retrospective study.

    PubMed

    Abir, G; Akdagli, S; Butwick, A; Carvalho, B

    2017-02-01

    Identifying pregnant women with sepsis is challenging because diagnostic clinical and laboratory criteria overlap with normal pregnant physiologic indices. Our primary study aim was to describe clinical and laboratory characteristics of women diagnosed with sepsis, severe sepsis and septic shock. Our secondary aim was to determine positive predictive values for International Classification of Disease (ICD)-9 billing codes for sepsis, severe sepsis, and septic shock. After gaining Institutional Review Board approval, we identified women with ICD-9 codes for sepsis, severe sepsis and septic shock who were admitted to a single tertiary obstetric center from 2007-2013. Diagnoses were confirmed using criteria from the International Sepsis Definitions Conference report. Demographic, obstetric, vital signs and laboratory data were abstracted by medical chart review. We identified 190 women with sepsis-related ICD-9 codes: of these, 35 (18%) women met the criteria for a clinical diagnosis of sepsis, severe sepsis or septic shock. Twenty (57%) women had a sepsis-related diagnosis after cesarean delivery. Twenty-one (60%) women had one or more pre-existing medical conditions and 19 (54%) women had one or more obstetric-related conditions. The genital tract was the most common site of infection. We observed considerable heterogeneity in maternal vital signs and laboratory indices for women with ICD-9 codes for sepsis, severe sepsis, and septic shock. The positive predictive value for each sepsis-related ICD-9 code was low: 16% (95% CI 10 to 24%) for sepsis, 10% (95% CI 3 to 25%) for severe sepsis and 24% (95% CI 10 to 46%) for septic shock. We identified marked heterogeneity in patient characteristics, clinical features, laboratory indices and microbiological findings among cohorts of women diagnosed with maternal sepsis, severe sepsis or septic shock. Based on our findings, the incidence of maternal sepsis using ICD-9 codes may be significantly overestimated. Copyright

  16. Clinical features and management of Hadronyche envenomation in man.

    PubMed

    Miller, M K; Whyte, I M; White, J; Keir, P M

    2000-03-01

    Using case reports and a review of the literature, the clinical features of envenomation by the genus of Australian funnel web spiders known as Hadronyche, are characterised. Five cases are reported here, including the first life-threatening envenomation by Hadronyche species 14 (the Port Macquarie funnel web). Two severe envenomations by Hadronyche cerberea (the Southern Tree funnel web) and one each by Hadronyche formidabilis (the Northern Tree funnel web) and Hadronyche infensa (the Darling Downs funnel web) are also described. The clinical experience of the authors' provided the five cases described in detail one of which has previously been reported in brief. Eight cases of Hadronyche envenomation from the literature (Medline 1966-1998 and Embase 1980-1998) were analysed in order to draw comparisons between this syndrome and the well described envenomation syndrome of Atrax robustus (the Sydney funnel web). Reports of funnel web spider antivenom use to Commonwealth Serum Laboratories (CSL) between 1995 and June 1998 were also examined. The biology of these dangerous spiders, their geographic distribution, venom characteristics and management issues are addressed. It is concluded that bites from at least six Hadronyche species have produced a life-threatening envenomation syndrome clinically indistinguishable from that of Atrax robustus. Atrax robustus derived antivenom is effective although antivenom requirements may be greater than for Atrax envenomation. Antivenom supplies are limited and sufficient stocks to treat a severe envenomation are unlikely to be found in any one institution. Pressure-immobilisation first aid is effective in delaying onset of envenomation, may enhance local inactivation of venom and early removal can result in rapid clinical deterioration.

  17. Clinical features of acute corpus callosum infarction patients

    PubMed Central

    Yang, Li-Li; Huang, Yi-Ning; Cui, Zhi-Tang

    2014-01-01

    The clinical manifestation of acute corpus callosum (CC) infarction is lack of specificity and complex, so it is easily missed diagnosis and misdiagnosis in the early stage. The present study aims to describe the clinical features of the acute CC infarction. In this study, 25 patients with corpus callosum infarction confirmed by the brain MRI/DWI and the risk factors were summarized. Patients were classified into genu infarction (3 cases), body infarction (4cases), body and genu infarction (4 cases), body and splenium infarction (1 case), splenium infarction (13 cases) according to lesion location. Clinical manifestation and prognosis were analyzed among groups. The results indicated that CC infarction in patients with high-risk group accounted for 72%, moderate-risk group accounted for 20%, low-risk group (8%). The main risk factors are carotid intimal thickening or plaque formation, hypertension, hyperlipidemia, cerebral artery stenosis, and so on. The CC infarction often merged with other parts infarction, and splenium infarction had the highest incidence, the clinical symptoms in the body infarction which can appear typical signs and symptoms, but in other parts infarction which always merged many nerve defect symptoms. The body infarction prognosis is poor; the rest parts of infarction are more favorable prognosis. In conclusion, CC infarction has the highest incidence in the stroke of high-risk group; neck color Doppler and TCD examination can be found as early as possible to explore the pathogenic factors. Prognosis is usually much better by treatment according to the location and risk factors. PMID:25197390

  18. Clinical features of soft bipolarity in major depressive inpatients.

    PubMed

    Utsumi, Takeshi; Sasaki, Tsukasa; Shimada, Iwao; Mabuchi, Mayuko; Motonaga, Takuro; Ohtani, Toshiyuki; Tochigi, Mamoru; Kato, Nobumasa; Nanko, Shinichiro

    2006-10-01

    Because of the difficulties of ascertaining episode of hypomania by past history of the patients, it is of clinical value to find variables which predict the development of bipolar II disorder in depressive patients. Taking advantage of relatively long hospitalization, the authors tried to elucidate fine clinical features of the soft bipolarity. The subjects were 39 patients with Major Depressive Episode, diagnosed according to the 4th edition of the Diagnostic and Statistical Manual criteria. Among them, 15 patients were diagnosed as bipolar II disorder (BPII), whereas 24 patients were with unipolar depression (UP), using a structured clinical interview to assess the mood spectrum (SCI-MOODS). In addition to ordinary clinical and demographic variables, the authors studied fine symptomatology of depression, premorbid personality, and interpersonal relationship. Continuous variables were analyzed by t-test. Categorical variables were tested by chi2 analysis. In terms of premorbid personality, manic type (Zerssen) was found more frequently in BPII (UP 2/24, BPII 9/15, P < 0.05). Patients with BPII tended to show apparently quick disappearance of depressive symptoms (UP 2/24, BPII 9/15, P = 0.01). The most prominent result was a high prevalence of comorbidity of borderline personality disorder (BPD) among BPII (UP 0/24, BPII 6/15, P = 0.02). As Akiskal indicated that mood lability represents the most powerful predictor of hypomanias, patients with BPII showed quick response in mood to admission. The current subjects with BPII had high frequency of manic type of premorbid personality, indicating the usefulness of this variable for the prediction of hypomanias. Finally, the authors could observe development of BPD during hospitalization exclusively among BPII, to support the possibility of BPD as a state effect of BPII.

  19. Radiological and clinical features of adult non-puerperal mastitis

    PubMed Central

    Tan, H; Li, R; Liu, H; Gu, Y; Shen, X

    2013-01-01

    Objective: To describe the radiological and clinical features of adult non-puerperal mastitis and to determine the most accurate method of preventing unnecessary surgical procedures. Methods: Clinical and imaging findings were retrospectively reviewed in 51 females with non-puerperal mastitis, which was confirmed by biopsy/surgical pathology. All 51 patients had pre-operative MRI; 45 patients also had sonograms and 25 also had mammograms, pre-operatively. Results: Of the 51 cases with non-puerperal mastitis, 94.1% (48/51) were confirmed as having acute or chronic inflammation, and the other 3 had plasma cell mastitis; areola papillaris inflammation was found in 39.2% (20/51) of the cases. Overall, 6 of the 25 cases that were examined with mammography and 2 of the 45 cases that were examined with sonography appeared normal, but all 51 lesions were positively identified on MRI. Asymmetrical density (12/25) on mammograms and solitary or separated/contiguous, clustered, hypoechoic mass-like lesions (31/45) on ultrasound were the most common signs of non-puerperal mastitis. On enhanced MRI, 90.2% (46/51) of patients showed non-mass-like enhanced lesions. Multiple regional enhancements in the pattern of distribution (32/46) and separated or contiguous, clustered, rim-like enhancements in the pattern of internal enhancement (29/46) were the most common manifestations in non-mass-like enhanced lesions. Of the 51 patients, mastitis Type 1 and Type 2 in the time–signal intensity curve were detected in 47.1% and 51.0% of the patients, respectively. The breast imaging reporting and data system categories with the highest number of patients were Category 0 (9/25) on mammography, Category 4a on sonography (18/45) and Category 4a on MRI (29/51). Conclusion: The findings from mammography and ultrasound are non-specific; therefore, using MR can be helpful in the diagnosis, especially in the presence of non-mass-like enhancements that are multiple, regional, separated, or

  20. Classification, clinical features, and genetics of neural tube defects

    PubMed Central

    Salih, Mustafa A.; Murshid, Waleed R.; Seidahmed, Mohammed Z.

    2014-01-01

    Neural tube defects (NTDs) constitute a major health burden (0.5-2/1000 pregnancies worldwide), and remain a preventable cause of still birth, neonatal, and infant death, or significant lifelong handicaps. The malformations result from failure of the neural folds to fuse in the midline, and form the neural tube between the third and the fourth week of embryonic development. This review article discusses their classification, clinical features, and genetics. Most NTDs are sporadic and both genetic, and non-genetic environmental factors are involved in its etiology. Consanguinity was suggested to contribute to the high incidence of NTDs in several countries, including Saudi Arabia. Syndromes, often associated with chromosomal anomalies, account for <10% of all NTDs; but a higher proportion (20%) has been documented in Saudi Arabia. Genetic predisposition constitutes the major underlying risk factor, with a strong implication of genes that regulate folate one-carbon metabolism and planar cell polarity. PMID:25551113

  1. [Analysis of six cases concerning clinical features of rhinolith].

    PubMed

    Ran, Xiangqun; Zhao, Xingyu; Tong, Hongxia

    2014-11-01

    To analyze six cases concerning clinical features, the treatment process and curative effect of Rhinolith. The six patients had unilateral nasal obstruction, three of them were accompanied with purulent nasal discharge, two had blood with them, one patient had repeated hemorrhage of nasal cavity and two patients had developed headache. By using endoscopic, four irregular stones which are yellow, gray and brown were found in the middle and back end. Because of the severe nasal septum deviation and inferior turbinate polypoid, the stone could not be seen clearly but could be touched by aspirator. In sinus CT examination of 6 cases, it showed incomplete irregular shapes and had high density unilateral nasal cavity in the posterior segment. 4 patients had nasal deviation, 3 patients had ipsilateral sinusitis. 4 patients removed their stone by using endoscopy, 2 patients took the stone away after correction of nasal septum. Pathologic diagnosis: rhinolith. Two patients also had exogenous foreign matters which were considered as pseudo nasal stones.

  2. Myhre syndrome: Clinical features and restrictive cardiopulmonary complications.

    PubMed

    Starr, Lois J; Grange, Dorothy K; Delaney, Jeffrey W; Yetman, Anji T; Hammel, James M; Sanmann, Jennifer N; Perry, Deborah A; Schaefer, G Bradley; Olney, Ann Haskins

    2015-12-01

    Myhre syndrome, a connective tissue disorder characterized by deafness, restricted joint movement, compact body habitus, and distinctive craniofacial and skeletal features, is caused by heterozygous mutations in SMAD4. Cardiac manifestations reported to date have included patent ductus arteriosus, septal defects, aortic coarctation and pericarditis. We present five previously unreported patients with Myhre syndrome. Despite varied clinical phenotypes all had significant cardiac and/or pulmonary pathology and abnormal wound healing. Included herein is the first report of cardiac transplantation in patients with Myhre syndrome. A progressive and markedly abnormal fibroproliferative response to surgical intervention is a newly delineated complication that occurred in all patients and contributes to our understanding of the natural history of this disorder. We recommend routine cardiopulmonary surveillance for patients with Myhre syndrome. Surgical intervention should be approached with extreme caution and with as little invasion as possible as the propensity to develop fibrosis/scar tissue is dramatic and can cause significant morbidity and mortality.

  3. Diffuse Lewy body disease: clinical features in 15 cases.

    PubMed Central

    Byrne, E J; Lennox, G; Lowe, J; Godwin-Austen, R B

    1989-01-01

    Fifteen cases of diffuse Lewy body disease were diagnosed on pathological grounds during a single year in one health district. The range and frequency of clinical features contrast strikingly with previous reports. The majority of cases presented with classical levodopa-responsive Parkinson's disease either alone (6 cases) or with mild cognitive impairment (3 cases); the remaining 6 cases presented with cognitive impairment alone. In time almost all patients developed both dementia and Parkinsonism. The dementia was cortical in type, but unusual in that most (12 cases) showed day-to-day fluctuation in severity at some point in their illness. These findings suggest that diffuse Lewy body disease is not rare, and that it presents in a range of ways from dementia with subsequent Parkinsonism to Parkinson's disease with subsequent dementia. The latter mode of presentation suggests that it should be considered as a significant pathological substrate of dementia in Parkinson's disease. Images PMID:2545827

  4. Classification, clinical features, and genetics of neural tube defects.

    PubMed

    Salih, Mustafa A; Murshid, Waleed R; Seidahmed, Mohammed Z

    2014-12-01

    Neural tube defects (NTDs) constitute a major health burden (0.5-2/1000 pregnancies worldwide), and remain a preventable cause of still birth, neonatal, and infant death, or significant lifelong handicaps. The malformations result from failure of the neural folds to fuse in the midline, and form the neural tube between the third and the fourth week of embryonic development. This review article discusses their classification, clinical features, and genetics. Most NTDs are sporadic and both genetic, and non-genetic environmental factors are involved in its etiology. Consanguinity was suggested to contribute to the high incidence of NTDs in several countries, including Saudi Arabia. Syndromes, often associated with chromosomal anomalies, account for <10% of all NTDs; but a higher proportion (20%) has been documented in Saudi Arabia. Genetic predisposition constitutes the major underlying risk factor, with a strong implication of genes that regulate folate one-carbon metabolism and planar cell polarity.

  5. Clinical features of cystic fibrosis patients with rare genotypes.

    PubMed Central

    Castaldo, G; Rippa, E; Raia, V; Salvatore, D; Massa, C; de Ritis, G; Salvatore, F

    1996-01-01

    We describe the clinical features of seven cystic fibrosis patients from southern Italy who bear rare genotypes: (1) a patient homozygous for the 2183 AA-->G mutation who was affected by a very early pulmonary form of cystic fibrosis, and five patients who were compound heterozygotes either for the 2183 AA-->G mutation or for the I148T mutation, in both instances with the delta F508 mutation; and (2) a patient homozygous for the early nonsense R553X mutation who showed only a moderately severe form of cystic fibrosis. Our results confirm that environmental or genetic factors unrelated to the CF disease contribute significantly to the development of the phenotype. Images PMID:8825054

  6. [Clinical features of sleep disorders in older adults].

    PubMed

    Chiba, Shigeru; Tamura, Yoshiyuki

    2015-06-01

    There are three major neurophysiological mechanisms underlying the sleep-waking cycle: the sleep system, the waking system, and the system that determines sleep-waking timing. Sleep dlisorders of older adults seem to be caused by functional or organic changes in one or more of the three systems, and are roughly classified into two categories: (i) normal age-related, and (ii) pathological. The former includes decreased amplitude and advanced phase of circadian rhythms (body temperature, melatonin secretion, and sleep-waking), as well as reduced sleep duration, sleep fragmentation, and a decrease of slow-wave sleep in sleep architecture. Pathological sleep disorders include medical and psychiatric diseases (e.g., lifestyle-related diseases, dementia, delirium, and depression) and primary age-related sleep disorders (e.g., REM sleep behavior disorder and periodic limb move- ment disorders). This mini-review delineates the clinical features of sleep disorders in older adults.

  7. Idiopathic photosensitive occipital epilepsy: clinical and electroencephalographic (EEG) features.

    PubMed

    Politi-Elishkevich, Keren; Kivity, Sara; Shuper, Avinoam; Levine, Hagit; Goldberg-Stern, Hadassa

    2014-03-01

    Idiopathic photosensitive occipital lobe epilepsy is a reflex, age- and localization-related syndrome. We describe the clinical and electroencephalographic features, therapy, and outcome of 16 children/adolescents with this syndrome. The cohort included 2 sets of siblings and 7 patients with other first- or second-degree relatives with a seizure history. All patients had occipital onset seizures and 15 had secondarily generalized tonic-clonic seizures. Seizure frequency was relatively low in all patients but one. Myoclonic seizures later developed in 2 patients with juvenile myoclonic epilepsy. Eight patients achieved full seizure control with monotherapy, and 5 required a second drug; 3 patients had rare seizures and were not treated with antiepileptics. Seven patients required special education or developmental assistance. This interesting syndrome sheds light on the pathophysiology and genetic etiology of common phenomena such as photosensitivity and headache. Further large prospective studies are required to better define this unique syndrome and its implications.

  8. Pachydermoperiostosis, a unique entity with distinctive clinical features.

    PubMed

    Tinoco-Fragoso, Fátima; Méndez-Flores, Silvia; Domínguez-Cherit, Judith

    2015-05-18

    Pachydermoperiostosis, which occurs more frequently in men, is a rare entity with distinctive clinical features and an insidious onset.. We report the case of a 30-year-old man with a one-year history of acropachy, arthralgias, hiperhidrosis, and progressive skin thickening of the face and scalp. The radiological findings were consistent with periostosis and the histopathological analysis from a facial skin biopsy showed a pandermal increase in the thickness and number of collagen bundles. The pathogenesis of PDP is currently unknown, although an increased secretion of prostaglandin E2 (PGE2), which stimulates the overexpression of vascular endothelial growth factor (VEGF), has been suggested as a major factor. No specific treatment exists; however, in most cases, the disease tends to stabilize over time.

  9. [Clinical and microbiological features of Salmonella gastroenteritis in children].

    PubMed

    Saporito, Laura; Colomba, Claudia; Scarlata, Francesco; Li Vecchi, Vatentina; Mammina, Caterina; Titone, Lucina

    2007-03-01

    The aim of our study was to evaluate the role of Salmonella spp in children hospitalised for acute gastroenteritis, and to study clinical and microbiological features of paediatric salmonellosis in our geographical area. In all, 540 patients admitted from March to September 2003 with symptoms of acute enteritis to the Infectious Diseases department of the "G. Di Cristina" hospital in Palermo were enrolled. Stool samples were collected within 48 hours of admission and tested for intestinal pathogens (bacterial, viral, parasites). Salmonella spp was detected in 18.5% of samples. The median age of infected children was 4.5 years. Salmonella enteritidis (49%) and Salmonella typhimurium (37%) were the most commonly identified genotypes. S. enteritidis infection was more frequently characterized by vomiting (65.3%) and dehydration (61.2%). Bloody diarrhoea was more common in S. typhimurium infection (40.5%). All strains were susceptible to ceftriaxone, while 40% of strains were resistant to tetracyclines and 37% to ampicillin.

  10. Clinical and Histopathologic Features of Fluoroquinolone-Induced Liver Injury

    PubMed Central

    Orman, Eric S.; Conjeevaram, Hari S.; Vuppalanchi, Raj; Freston, James W.; Rochon, James; Kleiner, David E.; Hayashi, Paul H.

    2011-01-01

    Background & Aims Fluoroquinolone-induced liver injury is rare; no prospective studies of well-characterized case series have been published. We studied patients with fluoroquinolone-induced hepatoxicity, using data from the Drug-Induced Liver Injury Network (DILIN) to characterize injury patterns, outcomes, and associated features. Methods We identified subjects with fluoroquinolone hepatotoxicity who enrolled in the DILIN from September 2004 to January 2010. Demographic, clinical, and laboratory data were analyzed by descriptive statistical methods. Results Of the 679 registrants in the DILIN prospective study, 12 had hepatoxicity from fluoroquinolones (6 ciprofloxacin, 4 moxifloxacin, 1 levofloxacin, and 1 gatifloxacin). Seven were women; the median age was 57 years (range 23–80 years), and the median time from the start of fluoroquinolone therapy to symptoms was only 4 days (range 1–39 days). Nine cases developed symptoms on medication (2, 8, and 32 days after they stopped the medication, 3 patients each). Cases were equally distributed among hepatocellular injury (predominantly increased levels of alanine aminotransferase), cholestatic injury (predominantly increased levels of alkaline phosphatase [AP]), and both. Seven cases had immunoallergic features. Patients with mixed hepatocellular and cholestatic injury had mild disease without jaundice—all recovered. In contrast, 2 of 4 patients with hepatocellular injury and jaundice died, 1 of acute liver failure. One patient with cholestatic injury developed vanishing bile duct syndrome and required liver transplantation; another had a persistently increased serum level of AP. Conclusions Fluoroquinolone liver injury is rapid in onset and often has immunoallergic features, indicating a hypersensitivity reaction. The pattern of injury is can be hepatocellular, cholestatic, or mixed—mixed cases are the least severe. Acute and chronic liver failure can occur. PMID:21356330

  11. Extraocular retinoblastoma in Indian children: clinical, imaging and histopathological features

    PubMed Central

    Sethi, Sumita; Pushker, Neelam; Kashyap, Seema; Sharma, Sanjay; Mehta, Mridula; Bakhshi, Sameer; Khurana, Saurbhi; Ghose, Supriyo

    2013-01-01

    AIM To study eyes with extraocular dissemination (EORB), with the following aims: first to establish the mean lag period and to understand various reasons for delayed presentation, second to study their imaging profiles and third to analyze histopathological features of eyes enucleated after neoadjuvant chemotherapy. METHODS Prospective study of clinical and imaging features of EORBs (stage III and IV International Retinoblastoma Staging System) presenting to a tertiary eye care centre. Histopathological features of eyes enucleated after receiving neoadjuvant chemotherapy were analyzed. A pictorial illustration of the varied imaging profile of EORB was also presented. RESULTS Over a period of one year, 97 eyes were diagnosed with retinoblastoma; 32 children (36 eyes) (37.1%) had EORB. Mean age 3.6±1.9 years, 71.9% males, 71.9% unilateral, 3.1% with positive family history and 40.6% with metastasis. On imaging, there was extrascleral involvement in 22.2%, involvement of orbital part of optic nerve in 33.3%, involvement of central nervous system in 27.8% and orbital wall involvement in 2.9% eyes. On histopathological analysis of eyes enucleated after neoadjuvant chemotherapy, 25.0% had no residual viable tumour tissue and rest all tumours were poorly differentiated. CONCLUSION There are very few human malignancies where definitive treatment is started without any confirmed histopathological diagnosis and imaging plays an important role in diagnosis and appropriate staging of the disease. Chemotherapy has a variable effect on EORB, 75.0% of eyes with EORB had residual viable tumour tissue when enucleated after receiving neoadjuvant chemotherapy. PMID:23991383

  12. Extraocular retinoblastoma in Indian children: clinical, imaging and histopathological features.

    PubMed

    Sethi, Sumita; Pushker, Neelam; Kashyap, Seema; Sharma, Sanjay; Mehta, Mridula; Bakhshi, Sameer; Khurana, Saurbhi; Ghose, Supriyo

    2013-01-01

    TO STUDY EYES WITH EXTRAOCULAR DISSEMINATION (EORB), WITH THE FOLLOWING AIMS: first to establish the mean lag period and to understand various reasons for delayed presentation, second to study their imaging profiles and third to analyze histopathological features of eyes enucleated after neoadjuvant chemotherapy. Prospective study of clinical and imaging features of EORBs (stage III and IV International Retinoblastoma Staging System) presenting to a tertiary eye care centre. Histopathological features of eyes enucleated after receiving neoadjuvant chemotherapy were analyzed. A pictorial illustration of the varied imaging profile of EORB was also presented. Over a period of one year, 97 eyes were diagnosed with retinoblastoma; 32 children (36 eyes) (37.1%) had EORB. Mean age 3.6±1.9 years, 71.9% males, 71.9% unilateral, 3.1% with positive family history and 40.6% with metastasis. On imaging, there was extrascleral involvement in 22.2%, involvement of orbital part of optic nerve in 33.3%, involvement of central nervous system in 27.8% and orbital wall involvement in 2.9% eyes. On histopathological analysis of eyes enucleated after neoadjuvant chemotherapy, 25.0% had no residual viable tumour tissue and rest all tumours were poorly differentiated. There are very few human malignancies where definitive treatment is started without any confirmed histopathological diagnosis and imaging plays an important role in diagnosis and appropriate staging of the disease. Chemotherapy has a variable effect on EORB, 75.0% of eyes with EORB had residual viable tumour tissue when enucleated after receiving neoadjuvant chemotherapy.

  13. Factors Associated with Clinical and Topographical Features of Laryngeal Tuberculosis

    PubMed Central

    Reis, João Gustavo Corrêa; Reis, Clarissa Souza Mota; da Costa, Daniel César Silva; Lucena, Márcia Mendonça; Schubach, Armando de Oliveira; Oliveira, Raquel de Vasconcellos Carvalhaes; Rolla, Valéria Cavalcanti; Conceição-Silva, Fátima; Valete-Rosalino, Cláudia Maria

    2016-01-01

    Introduction Laryngeal tuberculosis (LTB) is the most frequent granulomatous disease of the larynx and represents less than 2% of extrapulmonary TB cases. There are no pathognomonic clinical and endoscopic features of this disease and studies on LTB that can assist in its diagnostic characterization are lacking. Objective To identify factors associated with clinical and topographical features of LTB. Method a retrospective cross-sectional study was conducted from the medical records of 36 patients with confirmed LTB diagnosis. Results Dysphonia and cough were the main symptoms presented by patients and the true vocal folds the most frequently affected site. The average of the duration of the disease evolution was significantly higher in patients with dysphonia than in patients without this symptom. We observed association between dysphonia and true vocal fold lesions and between odynophagia and lesions in the epiglottis, arytenoids and aryepiglottic folds. Odynophagia was more frequent in individuals with lesions in four or more laryngeal sites. Weight loss equal or above 10% of the body weight was more frequent in patients with odynophagia as first symptom and in patients with ulcerated lesion. Dyspnea on exertion was more frequent in individuals with more extensive laryngeal lesions. The percentage of smokers with lesions in four or more laryngeal sites was greater than that found in non-smokers. Laryngeal tissue fragment bacilloscopy and culture examinations were less positive than sputum ones. Conclusions Smoking appears to be associated with the development of more extensive LTB lesions, and LTB with dyspnea on exertion and odynophagia with consequent impairment of nutritional status. We emphasize the need for histopathologic confirmation, once positive sputum bacteriological examinations seem not to necessarily reflect laryngeal involvement. PMID:27077734

  14. Idiopathic Hypersomnia: Clinical Features and Response to Treatment

    PubMed Central

    Ali, Mohsin; Auger, R. Robert; Slocumb, Nancy L.; Morgenthaler, Timothy I.

    2009-01-01

    Objective: A recent American Academy of Sleep Medicine publication identified a need for research regarding idiopathic hypersomnia. We describe various clinical and polysomnographic features of patients with idiopathic hypersomnia, with an emphasis on response to pharmacotherapy. Methods: A retrospective review of our database initially identified 997 patients, utilizing “idiopathic hypersomnia,” “hypersomnia NOS,” and “primary hypersomnia” as keywords. The charts of eligible patients were examined in detail, and data were abstracted and analyzed. Response to treatment was graded utilizing an internally developed scale. Results: Eighty-five patients were ultimately identified (65% female). Median (interquartile range) ages of onset and diagnosis were 19.6 (15.5) and 33.7 (15.5), respectively. During a median follow-up duration of 2.4 (4.7) years, 65% of patients demonstrated a “complete response” to pharmacotherapy as assessed by the authors' grading schema. Methylphenidate was most commonly used as a first-line agent prior to December 1998, but subsequently, modafinil became the most common first drug. At the last recorded follow-up visit, 92% of patients were on monotherapy, with greater representation of methylphenidate versus modafinil (51% vs. 32%). Among these patients, methylphenidate produced a higher percentage of “complete” or “partial” responses than modafinil, although statistical significance was not reached (38/40 [ 95%] vs 22/25 [88%], respectively, p = 0.291). Conclusions: The majority of patients with idiopathic hypersomnia respond well to treatment. Methylphenidate is chosen more often than modafinil as final monotherapy in the treatment of idiopathic hypersomnia, despite the fact that it is less commonly used initially. Further prospective comparisons of medications should be explored. Citation: Ali M; Auger RR; Slocumb NL; Morgenthaler TI. Idiopathic hypersomnia: clinical features and response to treatment. J Clin Sleep

  15. Mercury-Induced Membranous Nephropathy: Clinical and Pathological Features

    PubMed Central

    Li, Shi-Jun; Zhang, Su-Hua; Chen, Hui-Ping; Zeng, Cai-Hong; Zheng, Chun-Xia; Li, Lei-Shi

    2010-01-01

    Background and objectives: Long-term contact with mercury may induce membranous nephropathy (MN); however, the clinical pathologic features and pathogenesis of mercury-induced MN have not been investigated. Design, setting, participants, & measurements: The present study retrospectively evaluated 11 cases of mercury-induced MN to analyze its causes and its clinical and pathologic features. Results: A total of 10 women and 1 man ages 15 to 45 years were enrolled in the present study. Mercury exposure was caused by mercury-containing pills (five patients), skin lightening cream (four patients), hair-dyeing agents (one patient), and mercury vapor (one patient). The duration of contact with mercury ranged from 2 to 60 months, and the urinary mercury concentrations were 1.5 to 50 times higher than reference values. All patients presented with proteinuria and normal renal function; three had nephrotic syndrome. Light microscopy revealed thickened glomerular basement membrane and mildly proliferative mesangial cells. Acute tubulointerstitial injury occurred in three patients. The immunofluorescence findings showed granular deposits of IgG and C3 along the glomerular capillary wall, mostly accompanied by deposits of C4 and C1q. IgG1 and IgG4 (predominantly IgG1) deposits were observed along the glomerular capillary loops. Nine patients reached complete remission in follow-up after withdrawal from mercury exposure. Conclusions: Deposits of IgG1 subclasses in renal tissues indicated that the pathogenesis of mercury-induced MN differs from that of idiopathic MN. It is important that clinicians are aware that mercury exposure should be considered a possible cause of membranous nephropathy. PMID:20089494

  16. Pancreatic neuroendocrine tumors: clinical features, diagnosis and medical treatment: advances

    PubMed Central

    Ito, Tetsuhide; Igarashi, Hisato; Jensen, Robert T.

    2013-01-01

    Pancreatic neuroendocrine tumors (pNETs) comprise with gastrointestinal carcinoids, the main groups of gastrointestinal neuroendocrine tumors (GI-NETs). Although these two groups of GI-NETs share many features including histological aspects; over-/ectopic expression of somatostatin receptors; the ability to ectopically secrete hormones/peptides/amines which can result in distinct functional syndromes; similar approaches used for tumor localization and some aspects of treatment, it is now generally agreed they should be considered separate. They differ in their pathogenesis, hormonal syndromes produced, many aspects of biological behavior and most important, in their response to certain anti-tumor treatment (chemotherapy, molecular targeted therapies). In this chapter the clinical features of the different types of pNETs will be considered as well as aspects of their diagnosis and medical treatment of the hormone-excess state. Emphasis will be on controversial areas or recent advances. The other aspects of the management of these tumors (surgery, treatment of advanced disease, tumor localization) are not dealt with here, because they are covered in other chapters in this volume. PMID:23582916

  17. Biotinidase deficiency: initial clinical features and rapid diagnosis.

    PubMed

    Wolf, B; Heard, G S; Weissbecker, K A; McVoy, J R; Grier, R E; Leshner, R T

    1985-11-01

    Biotinidase deficiency is the primary defect in most individuals with late-onset multiple carboxylase deficiency. We have reviewed the presenting clinical features of 31 children with the disorder. Seizures, either alone or with other neurological or cutaneous findings, are the most frequent initial symptom observed. Other neurological symptoms, such as hypotonia, ataxia, hearing loss, optic atrophy, and developmental delay, are seen, in addition to skin rash and alopecia. The disorder is also characterized by ketolactic acidosis and organic aciduria. Biotinidase activity may be diagnosed using a simple, rapid, semiquantitative colorimetric procedure. Samples of whole blood spotted on the same filter paper used by most states to screen for phenylketonuria and other inborn errors of metabolism may be sent to an appropriate reference laboratory. None of the common anticonvulsants or sedatives used to treat newborns and children interfere with the test. Because biotinidase deficiency can be treated readily with biotin, this disorder should be considered in children with infantile seizures, especially in the presence of other characteristic neurological or cutaneous features.

  18. Atypical depression among psychiatric inpatients: clinical features and personality traits.

    PubMed

    Derecho, C N; Wetzler, S; McGinn, L K; Sanderson, W C; Asnis, G M

    1996-06-20

    This study investigates the frequency and characteristics of Atypical Depression (AD) among depressed inpatients. Twenty-one depressed inpatients received DSM-IV diagnoses, were rated on the Hamilton Depression Rating Scale (HAMD), and assessed for AD using the Atypical Depressive Disorder Scale. AD was defined as the presence of mood reactivity and two of four associated features: hyperphagia, hypersomnia, leaden paralysis, rejection sensitivity. Mood reactivity was defined as the ability to reach 50% of a non-depressed mood. All subjects completed the SCL-90, MCMI-II, and a suicide survey. Seven patients (33%) met criteria for AD. AD and non-AD patients did not differ in terms of severity of depression, history of suicide attempts, levels of clinical symptomatology, age of onset of depression, prior hospitalizations, and most personality characteristics. However, AD patients scored significantly higher than non-AD patients on the SCL-90 Interpersonal Sensitivity and MCMI-II Avoidant scales, and were more likely to be single. AD is fairly prevalent on an inpatient service, comparable to the frequency found in outpatient settings. AD is not a milder form of depression. The only differences between AD and non-AD patients reflect the personality trait of rejection sensitivity which is a defining feature of AD.

  19. The neuromuscular features of acromegaly: a clinical and pathological study.

    PubMed Central

    Khaleeli, A A; Levy, R D; Edwards, R H; McPhail, G; Mills, K R; Round, J M; Betteridge, D J

    1984-01-01

    A study of the neuromuscular features of acromegaly was performed in six patients. Clinical assessment was supplemented by quadriceps force measurements, plasma creatine kinase (CK) activities, electromyography (EMG) and nerve conduction studies. Muscle mass was measured by urinary creatinine/height indices (CHI) and cross sectional area (CSA) of thighs and calves on computed tomography. Quadriceps force/unit cross sectional area was derived. Needle biopsies of vastus lateralis were studied by histochemical and ultrastructural methods. Mean fibre area (MFA) and fibre type proportions were measured. Most of the subjects studied had muscle pain and proximal muscle weakness confirmed by quadriceps force measurements. This occurred in the absence of muscle wasting, as shown by cross sectional area measurements and normal or raised creatinine/height indices. "Myopathic" features were demonstrated by needle biopsy in half the patients and occasionally by electromyography and raised plasma creatine kinase activity. Abnormalities on needle biopsy included variation in fibre size, type 2 fibre atrophy and large type 1 MFA relative to type 2 MFA. Electronmicroscopy showed the non-specific findings of increased glycogen accumulation, excess lipofuscin pigment and myofilament loss. Images PMID:6481369

  20. Clinical features of headache associated with mobile phone use: a cross-sectional study in university students

    PubMed Central

    2011-01-01

    Background Headache has been reported to be associated with mobile phone (MP) use in some individuals. The causal relationship between headache associated with MP use (HAMP) and MP use is currently undetermined. Identifying the clinical features of HAMP may help in clarifying the pathophysiology of HAMP and in managing symptoms of individuals with HAMP. The aim of the present study is to describe the clinical features of HAMP. Methods A 14-item questionnaire investigating MP use and headache was administered to 247 medical students at Hallym University, Korea. Individual telephone interviews were subsequently conducted with those participants who reported HAMP more than 10 times during the last 1 year on the clinical features of HAMP. We defined HAMP as a headache attack during MP use or within 1 hour after MP use. Results In total, 214 (86.6%) students completed and returned the questionnaire. Forty (18.9%) students experienced HAMP more than 10 times during the last 1 year in the questionnaire survey. In subsequent telephone interviews, 37 (97.4%) interviewed participants reported that HAMP was triggered by prolonged MP use. HAMP was usually dull or pressing in quality (30 of 38, 79.0%), localised ipsilateral to the side of MP use (32 of 38, 84.2%), and associated with a burning sensation (24 of 38, 63.2%). Conclusion We found that HAMP usually showed stereotyped clinical features including mild intensity, a dull or pressing quality, localisation ipsilateral to the side of MP use, provocation by prolonged MP use and often accompanied by a burning sensation. PMID:21943309

  1. [South] Korea.

    PubMed

    1987-04-01

    The Republic of Korea occupies approximately 38,000 square miles in the southern position of a mountaineous peninsula. It shares a land boundary with North Korea. With a population of more than 40 million people, South Korea has 1 of the highest population densities in the world. The language spoken is a Uralic language, closely akin to Japanese, Hungarian, Finnish, and Mongolian, and the traditional religions are Shamanism and Buddhism. Over the course of time, South Korea has been invaded and fought over by its neighbors. The US and the Soviet Union have never been able to reach a unification agreement for North and South Korea. The 3rd Republic era, begun in 1963, saw a time of rapid industrialization and a great deal of economic growth. The 5th Republic began with a new constitution and new elections brought about the election of a president to a 7-year term of office beginning in 1981. Economic growth has been remarkable over the last 25 years despite the fact that North Korea possesses most of the mineral and hydroelectric resources and the existing heavy industrial base built by the Japanese while South Korea has the limited agricultural resources and had, initially, a large unskilled labor pool. Serious industrial growth began in South Korea in the early 1960s and the GNP grew at an annual rate of 10% during the period 1963-78. Current GNP is now, at $2000, well beyond that of its neighbors to the north. The outlook for longterm growth is good; however, the military threat posed by North Korea and the absence of foreign economic assistance has resulted in Korea spending 1/3 of its budget on defense. South Korea is active in international affairs and in the UN. Economic realities have forced Korea to give economics priority in their foreign policy. There has been an on-again, off-again quality to dialogue between the 2 nations. However, the US is committed to maintaining peace on the Korean peninsula. In order to do so, they have supplied manpower and

  2. Toxoplasma gondii infection in schizophrenia and associated clinical features.

    PubMed

    Esshili, Awatef; Thabet, Sihem; Jemli, Achraf; Trifa, Fatma; Mechri, Anouar; Zaafrane, Ferid; Gaha, Lotfi; Juckel, Georg; Babba, Hamouda; Bel Hadj Jrad, Besma

    2016-11-30

    The belief that latent toxoplasmosis is asymptomatic has been questioned, in particular due to the repeated highlighted link between the Toxoplasma gondii infection and an increased incidence of schizophrenia. However, to understand this relationship, the effect of infection with Toxoplasma gondii on the severity of schizophrenia has been poorly studied. Our work focused on comparing the prevalence of Toxoplasma infection between schizophrenic patients and healthy controls, as well as comparing the clinical features and the demographic characteristics between Toxoplasma-seronegative and Toxoplasma-seropositive patients with schizophrenia. The rate of IgG antibody in the schizophrenia patients was 74.8% compared 53.8% in controls. Patients with schizophrenia had a significantly higher mean of serum IgG antibodies to T. gondii compared to controls. The seropositive male patients had a higher age of disease onset, a higher BPRS score, a greater negative PANSS score and a lower GAF score than the seronegative male patients. These results suggest a higher severity of clinical symptoms in the male patients with schizophrenia. This study provides further evidence to the hypothesis that exposure to Toxoplasma may be a risk factor for schizophrenia. Moreover, toxoplasmosis in men with schizophrenia may lead to more severe negative and cognitive symptoms and a less favorable course of schizophrenia. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  3. Ebola in children: epidemiology, clinical features, diagnosis and outcomes.

    PubMed

    Olupot-Olupot, Peter

    2015-03-01

    Ebola virus disease is caused by a highly contagious and pathogenic threadlike RNA virus of the Filoviridae family. The index human case is usually a zoonosis that launches human-to-human transmission interface with varying levels of sustainability of the epidemic depending on the level of public health preparedness of the affected country and the Ebola virus strain. The disease affects all age groups in the population. Clinical diagnosis is challenging in index cases especially in the early stages of the disease when the presenting features are usually nonspecific and only similar to a flu-like illness. However, in the agonal stages, hemorrhage frequently occurs in a high proportion of cases. The diagnostic gold standard is by detecting the antigen using reverse transcription-polymerase chain reaction. Mortality rates in the past 28 outbreaks since 1976 have ranged from 30% to 100% in different settings among adults, but lower mortality rates have been documented in children. This review aims to describe Ebola virus infection, clinical presentation, diagnosis and outcomes in children.

  4. A unified concept of idiopathic orofacial pain: clinical features.

    PubMed

    Woda, A; Pionchon, P

    1999-01-01

    The main features of atypical facial pain, stomatodynia, atypical odontalgia, and masticatory muscle and temporomandibular joint (TMJ) disorders are compared in this article, which included a search of articles indexed in MEDLINE. The fact that their terminology has been the subject of many debates can be considered a consequence of taxonomic difficulties and uncertainties. Epidemiologic studies indicate marked female predominance for all types of idiopathic orofacial pain. There is also a difference in the age of maximal prevalence between masticatory muscle and TMJ disorders and the other entities. The clinical presentations display several symptoms in common. Pain is oral, perioral, or facial and does not follow a nervous pathway. It has been present for the last 4 to 6 months or has returned periodically in the same form over a period of several months or years. The pain is continuous, has no major paroxysmal character, and is present throughout all or part of the day. It is generally absent during sleep. Clinical, radiographic, or laboratory examination does not reveal any obvious organic cause of pain. There is also a frequent presence of certain psychologic factors, personality traits, or life events. Based on these shared characteristics, a unified concept is proposed. Each of these entities belongs to a group of idiopathic orofacial pain and could be expressed in either the jaws, the buccal mucosa, the teeth, the masticatory muscles, or the TMJ.

  5. [Incidence and clinical features of anaphylaxis during general anesthesia].

    PubMed

    Nabatame, Maki; Mori, Masanobu; Ikeda, Yoshikazu; Matsushita, Mitsuji; Tsujimura, Shigehisa

    2010-02-01

    Anaphylaxis is an acute life-threatening systematic reaction, and it requires early diagnosis and correct management to save the patients. The true incidence of anaphylaxis during general anesthesia is unknown. Although anaphylaxis is a rare intraoperative complication, most drugs used in the perioperative period can lead to anaphylaxis. All patients who had received operations under general anesthesia in our hospital were covered in this study during the 5-year period. We searched for the incidence and clinical features of anaphylaxis retrospectively. Seven cases in 9,844 patients were recognized. Various drugs were suspected as causal agents, but they were all nonanesthesia related drugs. The diagnosis of anaphylaxis relied on the history and clinical course of each patient. There was no reliable diagnostic testing such as serum tryptase and skin test. So we could not determine whether the reaction was IgE-mediated or not. Immediate discontinuation of causal drugs and early administration of epinephrine are cornerstones of treatment. More specific diagnostic tests are required for correct identification of causal drugs. To reduce the risk of anaphylaxis during anesthesia, we hope practical data bases and guidelines for anaphylaxis related to anesthesia will be published in the future.

  6. Cystinosis distal myopathy, novel clinical, pathological and genetic features.

    PubMed

    Cabrera-Serrano, Macarena; Junckerstorff, Reimar C; Alisheri, Ali; Pestronk, Alan; Laing, Nigel G; Weihl, Conrad C; Lamont, Phillipa J

    2017-09-01

    Nephropathic cystinosis is an autosomal recessive lysosomal disease in which cystine cannot exit the lysosome to complete its degradation in the cytoplasm, thus accumulating in tissues. Some patients develop a distal myopathy involving mainly hand muscles. Myopathology descriptions from only 5 patients are available in the literature. We present a comprehensive clinical, pathological and genetic description of 3 patients from 2 families with nephropathic cystinosis. Intrafamiliar variability was detected in one family in which one sibling developed a severe distal myopathy while the other sibling did not show any signs of skeletal muscle involvement. One of the patients was on treatment with Cysteamine for over 12 years but still developed the usual complications of nephropathic cystinosis in his twenties. Novel pathological findings consisting in sarcoplasmic deposits reactive for slow myosin were identified. Three previously known and one novel mutation are reported. Nephropathic cystinosis should be included in the differential diagnosis of distal myopathies in those with early renal failure. Novel clinical and pathological features are reported here contributing to the characterization of the muscle involvement in nephropathic cystinosis. Copyright © 2017 Elsevier B.V. All rights reserved.

  7. Clinical presentation and metabolic features of overt and occult urolithiasis.

    PubMed

    Polito, Cesare; Apicella, Andrea; Marte, Antonio; Signoriello, Giuseppe; La Manna, Angela

    2012-01-01

    Although pediatricians are frequently confronted with patients presenting urolithiasis symptoms without obvious stones, the syndrome of occult urolithiasis may be still viewed with some skepticism. We have compared the clinical and metabolic features of 197 children with obvious calculi, 189 with microcalculi (diameter ≤ 3 mm based on renal sonography), and 114 with symptoms of urolithiasis and normal renal sonography findings. Only microcalculi and normal sonography subjects with a urinary abnormality potentially leading to urolithiasis were included in the study. Age at presentation increased significantly (p = 0.0001) in the groups in the order normal sonography to microcalculi to calculi groups. There was no significant difference among the three groups in terms of family history of urolithiasis, gender distribution, and degree of hypercalciuria, hyperuricosuria, hyperoxaluria, or hypocitraturia. The average frequency of pain attacks of patients with recurrent abdominal pain (RAP) ranged from 3.6 to 4.6 days of pain per month among the three groups, which is four to ninefold lower than that reported for children with functional or organic gastrointestinal RAP. The consistency of many clinical and urinary metabolic characteristics indicates a common underlying disorder in overt and occult urolithiasis. The increase of age at presentation from the normal sonography to microcalculi and calculi groups may reflect progressive crystal accretion leading ultimately to overt stone formation.

  8. Clinical features of schizophrenia with enhanced carbonyl stress.

    PubMed

    Miyashita, Mitsuhiro; Arai, Makoto; Kobori, Akiko; Ichikawa, Tomoe; Toriumi, Kazuya; Niizato, Kazuhiro; Oshima, Kenichi; Okazaki, Yuji; Yoshikawa, Takeo; Amano, Naoji; Miyata, Toshio; Itokawa, Masanari

    2014-09-01

    Accumulating evidence suggests that advanced glycation end products, generated as a consequence of facilitated carbonyl stress, are implicated in the development of a variety of diseases. These diseases include neurodegenerative illnesses, such as Alzheimer disease. Pyridoxamine is one of the 3 forms of vitamin B6, and it acts by combating carbonyl stress and inhibiting the formation of AGEs. Depletion of pyridoxamine due to enhanced carbonyl stress eventually leads to a decrease in the other forms of vitamin B6, namely pyridoxal and pyridoxine. We previously reported that higher levels of plasma pentosidine, a well-known biomarker for advanced glycation end products, and decreased serum pyridoxal levels were found in a subpopulation of schizophrenic patients. However, there is as yet no clinical characterization of this subset of schizophrenia. In this study, we found that these patients shared many clinical features with treatment-resistant schizophrenia. These include a higher proportion of inpatients, low educational status, longer durations of hospitalization, and higher doses of antipsychotic medication, compared with patients without carbonyl stress. Interestingly, psychopathological symptoms showed a tendency towards negative association with serum vitamin B6 levels. Our results support the idea that treatment regimes reducing carbonyl stress, such as supplementation of pyridoxamine, could provide novel therapeutic benefits for this subgroup of patients.

  9. Eruptive Facial Postinflammatory Lentigo: Clinical and Dermatoscopic Features.

    PubMed

    Cabrera, Raul; Puig, Susana; Larrondo, Jorge; Castro, Alex; Valenzuela, Karen; Sabatini, Natalia

    2016-11-01

    The face has not been considered a common site of fixed drug eruption, and the authors lack dermatoscopic studies of this condition on the subject. The authors sought to characterize clinical and dermatoscopic features of 8 cases of an eruptive facial postinflammatory lentigo. The authors conducted a retrospective review of 8 cases with similar clinical and dermatoscopic findings seen from 2 medical centers in 2 countries during 2010-2014. A total of 8 patients (2 males and 6 females) with ages that ranged from 34 to 62 years (mean: 48) presented an abrupt onset of a single facial brown-pink macule, generally asymmetrical, with an average size of 1.9 cm. after ingestion of a nonsteroidal antiinflammatory drugs that lasted for several months. Dermatoscopy mainly showed a pseudonetwork or uniform areas of brown pigmentation, brown or blue-gray dots, red dots and/or telangiectatic vessels. In the epidermis, histopathology showed a mild hydropic degeneration and focal melanin hyperpigmentation. Melanin can be found freely in the dermis or laden in macrophages along with a mild perivascular mononuclear infiltrate. The authors describe eruptive facial postinflammatory lentigo as a new variant of a fixed drug eruption on the face.

  10. Body dysmorphic disorder among dermatologic patients: Prevalence and clinical features.

    PubMed

    Conrado, Luciana Archetti; Hounie, Ana Gabriela; Diniz, Juliana Belo; Fossaluza, Victor; Torres, Albina Rodrigues; Miguel, Euripedes Constantino; Rivitti, Evandro Ararigboia

    2010-08-01

    An impairing preoccupation with a nonexistent or slight defect in appearance is the core symptom of body dysmorphic disorder (BDD), a psychiatric condition common in dermatology settings. We sought to determine the prevalence of BDD in dermatologic patients, comparing general and cosmetic settings, and describing some demographic and clinical characteristics. In all, 300 patients were consecutively assessed. Screening and diagnoses were performed with validated instruments plus a best estimate diagnosis procedure. The final sample comprised 150 patients in the cosmetic group, 150 patients in the general dermatology group, and 50 control subjects. Standard statistical analyses were performed (chi(2), nonparametric tests, logistic regression). The current prevalence was higher in the cosmetic group (14.0%) compared with general (6.7%) and control (2.0%) groups. No patient had a previous diagnosis. Frequently the reason for seeking dermatologic treatment was not the main BDD preoccupation. Patients with BDD from the cosmetic group were in general unsatisfied with the results of dermatologic treatments. Cross-sectional study conducted in a university hospital is a limitation. It is uncertain if the findings can be generalized. Retrospective data regarding previous treatments are not free from bias. BDD is relatively common in a dermatologic setting, especially among patients seeking cosmetic treatments. These patients have some different features compared with general dermatology patients. Dermatologists should be aware of the clinical characteristics of BDD to identify and refer these patients to mental health professionals. Copyright 2009 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

  11. Substantia nigra echogenicity correlated with clinical features of Parkinson's disease.

    PubMed

    Zhou, Hai-Yan; Sun, Qian; Tan, Yu-Yan; Hu, Yun-Yun; Zhan, Wei-Wei; Li, Dun-Hui; Wang, Ying; Xiao, Qin; Liu, Jun; Chen, Sheng-Di

    2016-03-01

    Transcranial sonography can display structural alterations in the substantia nigra (SN) of patients with Parkinson's disease (PD), and is considered to be a potential useful tool for the diagnosis of PD. The aim of this study was to assess the correlation between SN echogenicity and clinical features in Chinese patients with PD. A total of 420 subjects including 290 patients with PD and 130 controls were recruited from the neurological clinic or the community. Transcranial sonographic evaluations of the SN were performed in all subjects, and motor and non-motor symptoms were thoroughly assessed by a series of rating scales in PD patients. Two hundred and one patients were successfully assessed by transcranial sonography. SN hyperechogenicity was found to be associated with male sex (p = 0.004), higher scores on the Unified Parkinson's Disease Rating Scale (UPDRS) part II (p = 0.001) and autonomic symptoms scores (p = 0.003). Moreover, regression analysis revealed that UPDRS part II scores (odds ratio = 1.141, p < 0.001) and gender (odds ratio = 2.409, p = 0.007) could be the independent predictors for SN hyperechogenicity; in addition, among all items of UPDRS part II, speech, dressing, hygiene, and turning in bed and adjusting bed clothes significantly correlated with SN hyperechogenicity. This is the first report suggesting the correlation between SN echogenicity and UPDRS part II, and we conclude that increased SN echogenicity might reflect more severe disease disability or poorer medical response. Copyright © 2016 Elsevier Ltd. All rights reserved.

  12. Clinical features and prognostic factors of Churg-Strauss syndrome

    PubMed Central

    Kim, Mi-Yeong; Sohn, Kyoung-Hee; Song, Woo-Jung; Park, Heung-Woo; Cho, Sang-Heon; Min, Kyung-Up

    2014-01-01

    Background/Aims Churg-Strauss syndrome (CSS) is a rare systemic necrotizing small-vessel vasculitis, with accompanying bronchial asthma, eosinophilia, and eosinophilic infiltration of various tissues. The purposes of our study were to characterize the clinical features of CSS and to identify factors associated with CSS prognosis in Koreans. Methods Medical records were reviewed retrospectively for all physician-diagnosed CSS patients in the Seoul National University Hospital between January 1990 and March 2011. Results Data from 52 CSS patients were analyzed. The respiratory tract was the most commonly involved organ (90.4%). Renal involvement was less frequent in antineutrophilic cytoplasmic antibody (ANCA)(-) patients than in ANCA(+) patients (p = 0.048). Clinical remission occurred in 95.3% of patients, but 16.3% of them relapsed. Patients who maintained remission for more than 6 months were relatively older (median, 51 years) at diagnosis (p = 0.004), had been diagnosed in earlier stages (p = 0.027), showed more frequent respiratory involvement (p = 0.024) and generalized symptoms (p = 0.039), and showed less frequent cutaneous involvement (p = 0.030) than those who did not achieve persistent (> 6 months) remission. Patients who achieved persistent remission also showed higher C-reactive protein (CRP) levels (p = 0.031) than those who did not. Conclusions ANCA(-) CSS patients showed less frequent renal involvement. Characteristics of good responders were older age, diagnosis at earlier stages, less cutaneous involvement, more respiratory involvement, high CRP values, and more generalized symptoms. PMID:24574837

  13. Clinical features and treatment of endophthalmitis after cataract surgery.

    PubMed

    Zhu, J; Li, Z H

    2015-06-18

    The aim of this study was to investigate the clinical features and treatment results of endophthalmitis after cataract surgery. Five patients with endophthalmitis after phacoemulsification with intraocular lens implantation were enrolled in this study. The pathogenesis, clinical manifestation, and surgical outcomes of 5 patients were compared. Three patients were surgically treated with anterior chamber irrigation and vitrectomy with intravitreal injection. The remaining two patients were medically treated with an intravitreal injection of vancomycin and ceftazidime. Treatment results of the five patients were analyzed. Four patients had positive cultures for bacteria (two cases Staphylococcus epidermidis, one case Enterococcus faecalis, and one case head-like Staphylococcus). The culture of the fifth patient did not have bacterial growth. One year following treatment, four patients had restored visual acuity and a clear vitreous cavity. Retinal detachment and other complications were not observed. The remaining patient had a visual acuity of index at 30 cm one year following treatment. For patients with endophthalmitis after cataract surgery, a biochemical laboratory examination should be promptly performed and should include a bacterial culture and drug sensitivity test. When necessary, vitrectomy combined with an intravitreal injection of vancomycin should be performed to treat the infection early and to help retain useful vision.

  14. Clinical features of inpatients with methamphetamine-induced psychosis.

    PubMed

    Fasihpour, Bahareh; Molavi, Siamak; Shariat, Seyed Vahid

    2013-08-01

    An epidemic of methamphetamine use has begun in Iran in recent years and psychiatric emergency services are overloaded with patients with methamphetamine-induced psychosis (MIP). To define the clinical features of inpatients with MIP in a psychiatric hospital. The files of all MIP patients admitted to Iran Psychiatric Hospital located in Tehran from April 2008 to April 2010 were assessed. Data related to psychotic episode, substance use, previous psychiatric history and demographic data of 111 MIP patients were extracted and analyzed using descriptive statistical methods. The most prevalent psychotic symptoms were persecutory delusion (82%), auditory hallucination (70.3%), reference delusion (57.7%), visual hallucination (44.1%), grandiosity delusion (39.6%) and jealousy delusion (26.1%). The mean duration of admission and psychotic episode was 21.43 and 17.37 days, respectively. In seven cases (8.75%), symptoms continued for more than one month. Frequency of psychotic symptoms in this study is relatively similar to previous studies. However, some clinical determinants such as latency of psychosis from first use and the course of psychosis are more similar to the first epidemic of methamphetamine in Japan than to more recent epidemics; which could be due to the short history of methamphetamine use in Iran.

  15. Effect of Dialysis Initiation Timing on Clinical Outcomes: A Propensity-Matched Analysis of a Prospective Cohort Study in Korea

    PubMed Central

    Lee, Jeonghwan; An, Jung Nam; Hwang, Jin Ho; Kim, Yong-Lim; Kang, Shin-Wook; Yang, Chul Woo; Kim, Nam-Ho; Oh, Yun Kyu; Lim, Chun Soo; Kim, Yon Su; Lee, Jung Pyo

    2014-01-01

    Background Controversy persists regarding the appropriate initiation timing of renal replacement therapy for patients with end-stage renal disease. We evaluated the effect of dialysis initiation timing on clinical outcomes. Initiation times were classified according to glomerular filtration rate (GFR). Methods We enrolled a total of 1691 adult patients who started dialysis between August 2008 and March 2013 in a multi-center, prospective cohort study at the Clinical Research Center for End Stage Renal Disease in the Republic of Korea. The patients were classified into the early-start group or the late-start group according to the mean estimated GFR value, which was 7.37 ml/min/1.73 m2. The primary outcome was patient survival, and the secondary outcomes were hospitalization, cardiovascular events, vascular access complications, change of dialysis modality, and peritonitis. The two groups were compared before and after matching with propensity scores. Results Before propensity score matching, the early-start group had a poor survival rate (P<0.001). Hospitalization, cardiovascular events, vascular access complications, changes in dialysis modality, and peritonitis were not different between the groups. A total of 854 patients (427 in each group) were selected by propensity score matching. After matching, neither patient survival nor any of the other outcomes differed between groups. Conclusions There was no clinical benefit after adjustment by propensity scores comparing early versus late initiation of dialysis. PMID:25137235

  16. Clinical features of Crohn disease concomitant with ankylosing spondylitis

    PubMed Central

    Liu, Song; Ding, Jie; Wang, Meng; Zhou, Wanqing; Feng, Min; Guan, Wenxian

    2016-01-01

    Abstract Extraintestinal manifestations (EIMs) cause increased morbidity and decreased quality of life in Crohn disease (CD). Ankylosing spondylitis (AS) belongs to EIMs. Very little is known on the clinical features of CD concomitant with AS. This study is to investigate the clinical features of CD patients with AS. We retrospectively collected all CD patients with AS in our hospital, and established a comparison group (CD without AS) with age, sex, and duration of Crohn disease matched. Clinical information was retrieved for comparison. Eight CD + AS patients were identified from 195 CD patients. Sixteen CD patients were randomly selected into comparison group. All CD + AS patients were male, HLA-B27 (+), and rheumatoid factor (−) with an average age of 40.8 ± 4.52 years. Significant correlation between disease activity of CD and AS was revealed (r = 0.857, P = 0.011). Significant correlation between disease activity of CD and functional limitation associated with AS was identified (r = 0.881, P < 0.01). C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and globulin were positively correlated to Crohn disease activity index (CDAI), Bath AS disease activity index, and Bath AS functional index(BASFI) scores (r = 0.73–0.93, P < 0.05). Albumin was negatively associated with CDAI and BASFI (r = −0.73 to −0.91, P < 0.05). The ratio of albumin to globulin (Alb/Glo) was significantly related to all 3 scores (r = −0.81 to −0.91, P < 0.05). Male predominance with a 4.12% concomitant incidence of AS is observed in CD patients. Disease activity of CD correlates with disease activity of AS and functional limitation caused by AS. CRP, ESR, and Alb/Glo may serve as biomarkers for disease activity and functional limitation in CD patients concomitant with AS, although future studies are expected. PMID:27428240

  17. [Features of Clinical Register of Chinese Medicine and Pharmacy Based on ClinicalTrials.gov. (USA)].

    PubMed

    Lu, Peng-fei; Liao, Xing; Xie, Yan-ming; Wang, Zhi-guo

    2015-11-01

    In recent 10 years, clinical trials of Chinese medicine and pharmacy (cMP) at clinicalTrials.gov.(USA) are gradually increasing. In order to analyze features of CMP clinical register, ClinicalTrials.gov register database were comprehensively retrieved in this study. Included clinical trials were input one item after another using EXCEL. A final of 348 CMP clinical trials were included. Results showed that China occupied the first place in CMP clinical register, followed by USA. CMP clinical trials, sponsored mainly by colleges/universities and hospitals, mostly covered interventional studies on evaluating safety/effectiveness of CMP. The proportions of studies, sponsored by mainland China and companies, recruitment trials and multi-center clinical trials in interventional trials were increasing. The proportions of studies sponsored by Hong Kong and Taiwan, research completed trials, unclear research status, phase III clinical trials, and published research trials in interventional trials were decreasing. Published ratios of CMP clinical trials were quite low. There were more missing types and higher proportions in trial register information.

  18. [Clinical features and comprehensive treatment of skull base osteosarcoma].

    PubMed

    Hu, Ke; Wan, Jinghai; Ni, Song; Li, Xueji; Liu, Shaoyan; Meng, Xiaoli; Qian, Haipeng

    2015-05-01

    To analyze the clinical features and treatment of skull base osteosarcoma. The clinical data of 18 patients with skull base osteosarcoma, who were admitted to the CAMS Cancer Hospital from January 2005 to November 2013, were retrospectively analyzed. The patients were followed up by telephone, outpatient review and other means. Fifteen patients were followed up, 4 cases received surgery only, and 11 cases received surgery with adjuvant chemotherapy and/or radiotherapy. Kaplan-Meier survival curve analysis was used to analyze the clinical data and Log rank method was used for verification. Nine patients died among the 15 patients who were followed up for 3-103 months (mean 25.0 months): seven patients died of local recurrence, and two patients died of distant metastasis, and six patients were still alive. Four patients received surgery only, with a median survival time of 25.0 months, and 11 patients received comprehensive treatment, with a median survival time of 47.0 months (P = 0.02). Five patients received sub-total resection, with a mean survival time of 47.0 months, and 10 patients received total resection, with a mean survival time of 45.0 months (P = 0.37). The 1- and 2-year recurrence rates were 46.6% and 68.9%, respectively. The overall 1-, 2-, 3- and 5-year survival rates were 82.4%, 61.8%, 36.0% and 36.0%, respectively, with a median survival time of 30.0 months. To compare the long bone and head and neck osteosarcoma with skull base osteosarcoma, the skull base osteosarcoma has a lower total resection rate, a higher recurrence rate, and a poorer prognosis. Radical surgery and comprehensive treatment are appropriate for skull base osteosarcoma.

  19. [Clinical features of protracted bacterial bronchitis in children].

    PubMed

    Chi, Fanfan; Wang, Yuqing; Hao, Chuangli; Sun, Huiquan; Fan, Liping; Huang, Li; Yu, Xingmei; Yang, Xiaoyun; Lu, Yanhong; Zhou, Jing; Lu, Quan

    2015-10-01

    To analyze the clinical characteristics of protracted bacterial bronchitis (PBB) in children. The clinical data of patients seen from October, 2010 to March, 2014 in Department of Respiratory Diseases of our hospital were retrospectively analyzed. Inclusion criteria were over 4 weeks cough, receiving fiberoptic bronchoscopy, positive bacterial culture and (or) the increased percentage of neutral granulocytes in bronchoalveolar lavage fluid (BALF). Twenty eight patients were involved, 26 were male (93%) and two were female (7%). The median age of patients was 8.5 months. The median duration of cough was four weeks. The average length of hospital stay was (8.3 ± 3.9)days. The main clinical feature was wet cough in 28 cases, wet cough with wheezing was seen in 21 cases. The wet cough phase distribution was irregular in 21 cases. The crackles with wheeze (in 21 cases) was main signs of PBB. The percentage of CD3⁻ CD16⁺ 56⁺ cells increased in peripheral blood. The fiberoptic bronchoscopic manifestations of PBB were luminal mucosal edema. Eleven patients also had airway malacia. The neutrophil median in BALF was 0.2. The positive rate of bacterial culture of BALF was 36%. The main bacteria were Streptococcus pneumoniae (50%) and Haemophilus influenzae (30%). The main treatment for PBB patients included amoxycillin/clavulanate potassium and second-generation cephalosporins. The average duration of treatment was (17.3 ± 3.2)days, the prognosis was good. PBB is common in male infants. Persistent wet cough with wheezing was the main characteristic of PBB. PBB is commonly accompanied by immune dysfunction and airway malacia, and the pathogens were Streptococcus pneumoniae and Haemophilus influenzae.

  20. Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis).

    PubMed

    Bohm, Marek; Gonzalez Fernandez, Maria Isabel; Ozen, Seza; Pistorio, Angela; Dolezalova, Pavla; Brogan, Paul; Barbano, Giancarlo; Sengler, Claudia; Klein-Gitelman, Marisa; Quartier, Pierre; Fasth, Anders; Herlin, Troels; Terreri, Maria Teresa R A; Nielsen, Susan; van Rossum, Marion A J; Avcin, Tadej; Castell, Esteban Rodolfo; Foeldvari, Ivan; Foell, Dirk; Kondi, Anuela; Koné-Paut, Isabelle; Kuester, Rolf-Michael; Michels, Hartmut; Wulffraat, Nico; Amer, Halima Ben; Malattia, Clara; Martini, Alberto; Ruperto, Nicolino

    2014-01-01

    Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), belongs to the group of ANCA-associated necrotizing vasculitides. This study describes the clinical picture of the disease in a large cohort of GPA paediatric patients. Children with age at diagnosis ≤ 18 years, fulfilling the EULAR/PRINTO/PRES GPA/WG classification criteria were extracted from the PRINTO vasculitis database. The clinical signs/symptoms and laboratory features were analysed before or at the time of diagnosis and at least 3 months thereafter and compared with other paediatric and adult case series (>50 patients) derived from the literature. The 56 children with GPA/WG were predominantly females (68%) and Caucasians (82%) with a median age at disease onset of 11.7 years, and a median delay in diagnosis of 4.2 months. The most frequent organ systems involved before/at the time of diagnosis were ears, nose, throat (91%), constitutional (malaise, fever, weight loss) (89%), respiratory (79%), mucosa and skin (64%), musculoskeletal (59%), and eye (35%), 67% were ANCA-PR3 positive, while haematuria/proteinuria was present in > 50% of the children. In adult series, the frequency of female involvement ranged from 29% to 50% with lower frequencies of constitutional (fever, weight loss), ears, nose, throat (oral/nasal ulceration, otitis/aural discharge), respiratory (tracheal/endobronchial stenosis/obstruction), laboratory involvement and higher frequency of conductive hearing loss than in this paediatric series. Paediatric patients compared to adults with GPA/WG have similar pattern of clinical manifestations but different frequencies of organ involvement.

  1. Clinical Features and Patient Management of Lujo Hemorrhagic Fever

    PubMed Central

    Sewlall, Nivesh H.; Richards, Guy; Duse, Adriano; Swanepoel, Robert; Paweska, Janusz; Blumberg, Lucille; Dinh, Thu Ha; Bausch, Daniel

    2014-01-01

    Background In 2008 a nosocomial outbreak of five cases of viral hemorrhagic fever due to a novel arenavirus, Lujo virus, occurred in Johannesburg, South Africa. Lujo virus is only the second pathogenic arenavirus, after Lassa virus, to be recognized in Africa and the first in over 40 years. Because of the remote, resource-poor, and often politically unstable regions where Lassa fever and other viral hemorrhagic fevers typically occur, there have been few opportunities to undertake in-depth study of their clinical manifestations, transmission dynamics, pathogenesis, or response to treatment options typically available in industrialized countries. Methods and Findings We describe the clinical features of five cases of Lujo hemorrhagic fever and summarize their clinical management, as well as providing additional epidemiologic detail regarding the 2008 outbreak. Illness typically began with the abrupt onset of fever, malaise, headache, and myalgias followed successively by sore throat, chest pain, gastrointestinal symptoms, rash, minor hemorrhage, subconjunctival injection, and neck and facial swelling over the first week of illness. No major hemorrhage was noted. Neurological signs were sometimes seen in the late stages. Shock and multi-organ system failure, often with evidence of disseminated intravascular coagulopathy, ensued in the second week, with death in four of the five cases. Distinctive treatment components of the one surviving patient included rapid commencement of the antiviral drug ribavirin and administration of HMG-CoA reductase inhibitors (statins), N-acetylcysteine, and recombinant factor VIIa. Conclusions Lujo virus causes a clinical syndrome remarkably similar to Lassa fever. Considering the high case-fatality and significant logistical impediments to controlled treatment efficacy trials for viral hemorrhagic fever, it is both logical and ethical to explore the use of the various compounds used in the treatment of the surviving case reported here

  2. Clinical characteristics of metabolic syndrome in Korea, and its comparison with other Asian countries

    PubMed Central

    Hong, A Ram; Lim, Soo

    2015-01-01

    Metabolic syndrome is referred to as syndrome X or insulin resistance syndrome, and is primarily composed of abdominal obesity, diabetes, glucose intolerance, dyslipidemia and high blood pressure. Asians have a lower frequency of obesity than Caucasians, but have an increasing tendency toward metabolic syndrome. Thus, metabolic syndrome poses a major challenge for public health professionals, and is set to become a social and economic problem in Asian populations. Most data on metabolic syndrome are based on studies from Western countries with only limited information derived from Asian populations. Recently, several studies were carried out on a large scale that represents the general Korean population. The prevalence of metabolic syndrome in Korean adults has varied depending on the study designs and different criteria, but shows a distinct increasing trend of metabolic syndrome driven by an increase in abdominal obesity and dyslipidemia. Given the rapid economic progression of Korea over the past 30 years along with a rise of the aged population, it is expected that the prevalence of metabolic syndrome will further increase. Therefore, a proactive strategy at the governmental level for metabolic syndrome prevention should be implemented, reducing abdominal obesity and dyslipidemia. Healthy dietary habits and regular exercise should be emphasized as a part of such a strategy. PMID:26417407

  3. Clinical characteristics of metabolic syndrome in Korea, and its comparison with other Asian countries.

    PubMed

    Hong, A Ram; Lim, Soo

    2015-09-01

    Metabolic syndrome is referred to as syndrome X or insulin resistance syndrome, and is primarily composed of abdominal obesity, diabetes, glucose intolerance, dyslipidemia and high blood pressure. Asians have a lower frequency of obesity than Caucasians, but have an increasing tendency toward metabolic syndrome. Thus, metabolic syndrome poses a major challenge for public health professionals, and is set to become a social and economic problem in Asian populations. Most data on metabolic syndrome are based on studies from Western countries with only limited information derived from Asian populations. Recently, several studies were carried out on a large scale that represents the general Korean population. The prevalence of metabolic syndrome in Korean adults has varied depending on the study designs and different criteria, but shows a distinct increasing trend of metabolic syndrome driven by an increase in abdominal obesity and dyslipidemia. Given the rapid economic progression of Korea over the past 30 years along with a rise of the aged population, it is expected that the prevalence of metabolic syndrome will further increase. Therefore, a proactive strategy at the governmental level for metabolic syndrome prevention should be implemented, reducing abdominal obesity and dyslipidemia. Healthy dietary habits and regular exercise should be emphasized as a part of such a strategy.

  4. [Antipsychotic-drug-induced hyperprolactinemia: physiopathology, clinical features and guidance].

    PubMed

    Besnard, I; Auclair, V; Callery, G; Gabriel-Bordenave, C; Roberge, C

    2014-02-01

    prevalence. In the long-term, hypogonadism involves a premature bone loss in men and women. Klibanski and colleagues showed that this loss is significant only in women with hyperprolactinemia associated with amenorrhea. That suggests that prolactin is not directly responsible for this clinical feature. Nevertheless, prolactin seems to be involved in the development of breast cancer, but its role is unclear for prostate cancer. Our review promotes a check-up before beginning a treatment with antipsychotic agents. First, a baseline prolactin level should be measured. It should also include the research on previous treatment with antipsychotic-drugs and the assessment of adverse effects suggestive of hyperprolactinemia. Questioning should finally look for any contra-indication to antipsychotics. Monitoring during antipsychotic treatment has been studied by a group of international experts in psychiatry, medicine, toxicology and pharmacy who made a critical review of clinical guidance on hyperprolactinemia. Experts notify that it is important to check whether patients have any sexual dysfunction, such as loss of libido or menstrual irregularity, and galactorrhoea. Prolactin level should also be controlled after three months of stable dose treatment, or if any clinical feature of hyperprolactinemia appears. If a patient prescribed antipsychotic-drugs has a confirmed prolactin level above the normal range, it is necessary to exclude other causes of hyperprolactinemia. If antipsychotics are really involved, the management should be adapted with the prolactin level and the patient him/herself. To summarize, clinicians can decrease the dose of the antipsychotic or switch to a prolactin-sparing drug. Oral contraceptives can be added whether to prevent pregnancy or to prevent bone loss and osteoporosis. Finally, experts recommend reserving dopamine agonists to treat antipsychotic-induced hyperprolactinemia in very exceptional circumstances as it can worsen the mental illness. Copyright

  5. Developing Key-Feature Problems and Examinations to Assess Clinical Decision-Making Skills.

    ERIC Educational Resources Information Center

    Page, Gordon; And Others

    1995-01-01

    An approach to testing medical students' clinical decision-making skills identifies key features (critical steps in resolution of a clinical problem) and presents a clinical case scenario followed by questions focusing on those key features. Key-feature problems provide flexibility on issues of question format, multiple responses to questions, and…

  6. [Clinical features of traumatic optic neuropathy in 265 cases].

    PubMed

    Chen, M; Jiang, Y; Wen, J F; Li, N; Qiu, J; Zhang, N K; Ju, J B

    2017-09-07

    Objective: To analyze the clinical features and the pathogenetic law of traumatic optic neuropathy through epidemiologic study. Methods: 265 cases (275 eyes) with TON treated in Department of Otorhinolaryngology Head and Neck Surgery, the Affiliated Hospital of Qingdao University from April 1999 to August 2015 were retrospectively analyzed. Multiple Logistic regression analyses were used to evaluate potential prognostic factors on visual acuity. Results: TON occured mostly in young (194/265, 73.21%) man (235/265, 88.68%), the majority of patients came from villages and towns (209/265, 78.87%). Traffic accident (197/265, 74.34%) remained the main etiology, with strike (36/265, 13.58%) and fall (17/265, 6.42%) as the common etiology. Most patients had head injuries. The effective rate of vision improvement was 53.45%(147/275). Multiple logistic regression analyses identified that initial visual acuity with light perception or better vision, optic canal fracture and orbital wall fracture were visual acuity key factors of TON (χ(2) value was 24.674, 19.755, 9.175, respectively, all P<0.01), initial visual acuity with light perception or better vision was the protective factor on visual acuity recovery (OR=5.008, P<0.001), the presence of optic canal fracture and orbital wall fracture were the risk factors on visual acuity recovery (OR value was 0.110, 0.329, respectively, all P<0.01). Conclusions: Ton occurs mostly in young man because of traffic accident. Visual impairment of TON is severe. The suitable preventive measures should be carried out according to its epidemiological features.

  7. Clinical features of delusional jealousy in elderly patients with dementia.

    PubMed

    Hashimoto, Mamoru; Sakamoto, Shinichi; Ikeda, Manabu

    2015-06-01

    Delusional jealousy is a psychotic syndrome characterized by a belief in the infidelity of one's spouse that reaches delusional intensity. Although delusional jealousy has been described in relation to organic psychosis, little is known concerning the actual role of delusional jealousy in dementia. The aim of the present study was to investigate the clinical features of delusional jealousy and possible mechanisms whereby delusional jealousy arises in patients with dementia. We studied 208 consecutive outpatients with dementia (diagnosis based on DSM-III-R criteria; mean [SD] age of 77.0 [8.0] years; study period: September 2011-August 2012). Delusional jealousy was defined as a false belief derived from a pathological jealousy that makes the patient believe that his or her spouse is unfaithful. The prevalence of delusional jealousy was compared between Alzheimer's disease, dementia with Lewy bodies, and vascular dementia. Patients with and without delusional jealousy were compared in terms of general characteristics. In addition, each patient with delusional jealousy and their primary caregivers were interviewed about the clinical features of the syndrome. Of the 208 patients with dementia, 18 (8.7%) showed delusional jealousy. The prevalence of delusional jealousy in patients who had dementia with Lewy bodies (26.3%) was significantly higher than that in patients with Alzheimer's disease (5.5%) (P < .01). There were no significant differences between patients with and without delusional jealousy in regard to gender (P = 1.00), age (P = .81), educational attainment (P = .29), presence of other persons living with the couple (P = .22), and Mini-Mental State Examination score (P = .47). On the other hand, delusional jealousy was preceded by the onset of serious physical diseases in nearly half of the patients. Delusional jealousy resolved within 12 months after treatment in 15 of 18 patients (83%). Although delusional jealousy is a considerable problem in dementia

  8. Clinical Features of Newly Diagnosed Cytomegalovirus Retinitis in Northern Thailand

    PubMed Central

    Ausayakhun, Somsanguan; Keenan, Jeremy D; Ausayakhun, Sakarin; Jirawison, Choeng; Khouri, Claire M; Skalet, Alison H; Heiden, David; Holland, Gary N; Margolis, Todd P

    2011-01-01

    Purpose To characterize the clinical manifestations of cytomegalovirus (CMV) retinitis in northern Thailand. Design Prospective, observational cross-sectional study. Methods We recorded characteristics of 52 consecutive patients newly diagnosed with CMV retinitis at a tertiary university-based medical center in northern Thailand. Indirect ophthalmoscopy by experienced ophthalmologists was supplemented with fundus photography to determine the proportion of eyes with various clinical features of CMV retinitis. Results Of the 52 patients with CMV retinitis, 55.8% were female. All were HIV-positive. The vast majority (90.4%) had started antiretroviral therapy. CMV retinitis was bilateral in 46.2% of patients. Bilateral visual acuity worse than 20/60 was observed in 23.1% of patients. Of 76 eyes with CMV retinitis, 61.8% had zone I disease and 21.6% had lesions involving the fovea. Lesions larger than 25% of the retinal area were observed in 57.5% of affected eyes. CMV retinitis lesions commonly had marked or severe border opacity (47.4% of eyes). Vitreous haze was often present (46.1% of eyes). Visual impairment was more common in eyes with larger retinitis lesions. Retinitis lesion size, used as a proxy for duration of disease, was associated with fulminant appearance (OR 1.24 [1.01 – 1.51]), and marked or severe border opacity (OR 1.36 [1.11 – 1.67]). Based on lesion size, retinitis preceded antiretroviral treatment in each patient. Conclusions Patients presenting to a tertiary medical center in northern Thailand have advanced CMV retinitis, possibly due to delayed diagnosis. Earlier screening and treatment of CMV retinitis may limit progression of disease and prevent visual impairment in this population. PMID:22265148

  9. Age-related distance esotropia: Clinical features and therapeutic outcomes.

    PubMed

    Gómez de Liaño Sánchez, P; Olavarri González, G; Merino Sanz, P; Escribano Villafruela, J C

    2016-12-01

    To describe the clinical characteristics and surgical outcomes of a group of patients with age-related distance esotropia (ARDE). A retrospective study was conducted on a consecutive case series of 16 adult patients diagnosed with ARDE between 2008 and 2015. The clinical features evaluated included mean age and gender, primary position deviations at distance and near, measured in prism dioptres (pd), treatment offered in each case, and post-surgical deviations. Ductions and versions were full, with no evidence of lateral rectus paresis. None of these patients had any obvious underlying neurological disorder, such as, high myopia or thyroid disease. A good result is considered to be the disappearance of diplopia in all positions of gaze. A total of 16 patients (11 females [68.8%]) were identified. The mean age at diagnosis was 78.19±6.77 years. The mean initial esodeviation was 2.25±3.08 pd at near (-4 to +8 pd) and 9.5±4.18 pd at distance (2 to 18 pd). Treatment was not necessary in 5 cases because the symptoms were intermittent or well-tolerated. Of the 11 patients with symptoms, one was corrected with an external base therapeutic prism. Botulinum toxin was administered in another patient, without satisfactory results. Unilateral medial rectus muscle recession was performed on one patient, and unilateral lateral rectus plication on 7 patients, indicating prisms before surgery. One patient refused surgery despite continuous diplopia in far vision. After a mean follow-up of 16.5 months, all operated patients were asymptomatic. Not all patients with ARDE require treatment, as the tolerance to diplopia varies from one subject to another. Both medial rectus weakening and lateral rectus strengthening provides excellent results. Crown Copyright © 2016. Publicado por Elsevier España, S.L.U. All rights reserved.

  10. Clinical, serologic, and histologic features of gluten sensitivity in children.

    PubMed

    Francavilla, Ruggiero; Cristofori, Fernanda; Castellaneta, Stefania; Polloni, Carlo; Albano, Veronica; Dellatte, Stefania; Indrio, Flavia; Cavallo, Luciano; Catassi, Carlo

    2014-03-01

    To describe the clinical, serologic, and histologic characteristics of children with gluten sensitivity (GS). We studied 15 children (10 males and 5 females; mean age, 9.6 ± 3.9 years) with GS who were diagnosed based on a clear-cut relationship between wheat consumption and development of symptoms, after excluding celiac disease (CD) and wheat allergy, along with 15 children with active CD (5 males and 10 females; mean age, 9.1 ± 3.1 years) and 15 controls with a functional gastrointestinal disorder (6 males and 9 females; mean age, 8.6 ± 2.7 years). All children underwent CD panel testing (native antigliadin antibodies IgG and IgA, anti-tissue transglutaminase antibody IgA and IgG, and anti-endomysial antibody IgA), hematologic assessment (hemoglobin, iron, ferritin, aspartate aminotransferase, erythrocyte sedimentation rate), HLA typing, and small intestinal biopsy (on a voluntary basis in the children with GS). Abdominal pain was the most prevalent symptom in the children with GS (80%), followed by chronic diarrhea in (73%), tiredness (33%), bloating (26%), limb pain, vomiting, constipation, headache (20%), and failure to thrive (13%). Native antigliadin antibodies IgG was positive in 66% of the children with GS. No differences in nutritional, biochemical, or inflammatory markers were found between the children with GS and controls. HLA-DQ2 was found in 7 children with GS. Histology revealed normal to mildly inflamed mucosa (Marsh stage 0-1) in the children with GS. Our findings support the existence of GS in children across all ages with clinical, serologic, genetic, and histologic features similar to those of adults. Copyright © 2014 Mosby, Inc. All rights reserved.

  11. Genetic, hypothalamic and endocrine features of clinical and experimental obesity.

    PubMed

    Bray, G A

    1992-01-01

    Obesity occurs in both clinical and animal forms in a variety of specific models which allow study of its underlining endocrine and mechanistic features. Among the neuroendocrine varieties of obesity, polycystic ovaries are probably the most common. The importance of the gonadal feedback system for regulation of food intake and obesity is indicated by the effects of castration in experimental animals which is a widely used mechanism for producing experimental obesity. Cushing syndrome and hypothalamic obesity are rare clinical syndromes. The current evidence suggests that there are two types of hypothalamic obesity from a mechanistic point of view--one associated with hyperphagia as a necessary and sufficient cause and a disturbance of the autonomic nervous system without hyperphagia as a second mechanism. Although genetic factors underlie most types of human obesity, there are several dymorphic forms of obesity including the Prader-Willy syndrome, Cohen's syndrome, Carpenter's syndrome, Ahlstrom's syndrome and the Bardet-Biedel syndrome. The Prader-Willi syndrome is characterized by obesity hypotonia hypogonadism and mental retardation. In animals, a dominant form of inheritance of obesity is seen in the yellow mouse. Current evidence suggests that this syndrome can be explained by reduced acetylation of MSH in the pituitary and/or hypothalamus. Several recessively inherited forms of obesity exist including the obese mouse, the diabetes mouse, fatty rat, the fat mouse, tubby mouse and the corpulent rat. In addition, there are a number of polygenic types of experimental obesity. The final mechanistic classification of obesity are those due to dietary manipulation. For both human beings and animals, a highly fat diet appears to be particularly problematic for the development of obesity.(ABSTRACT TRUNCATED AT 250 WORDS)

  12. Clinical Features and Outcomes of Pasteurella multocida Infection

    PubMed Central

    Giordano, Antonio; Dincman, Toros; Clyburn, Benjamin E.; Steed, Lisa L.; Rockey, Don C.

    2015-01-01

    Abstract Pasteurella multocida, a zoonotic infectious organism, has most often been described in patients after an animal bite. Here, we characterize the clinical features and outcomes of P multocida infection in a large cohort of patients according to the presence or absence of an animal bite. We retrospectively searched MUSC's laboratory information system for all patients with positive P multocida cultures from 2000 to 2014. Extensive data were abstracted, including clinical and outcome data. The Charlson comorbidity index (CCI) was used to assess comorbidities among patients. We identified 44 patients with P multocida infections, including 25 with an animal bite. The average age was 64 years and the majority of patients were women (N = 30). There was no difference in age and sex distribution among those with and without a bite (P = 0.38 and 0.75, respectively). A CCI ≥1 was significantly associated with the absence of a bite (P = 0.006). Patients presenting without a bite were more frequently bacteremic (37% vs 4%, respectively, P = 0.001), and were hospitalized more often (84% vs 44%, respectively, P = 0.012). Of the 8 patients who required intensive care unit (ICU)-based care, 7 were non-bite-related. There were 4 deaths, all occurring in patients not bitten. P multocida infections not associated with an animal bite were often associated with bacteremia, severe comorbidity(ies), immune-incompetent states, the need for ICU management, and were associated with substantial mortality. PMID:26356688

  13. Cerebral proliferative angiopathy: Clinical, angiographic features and literature review

    PubMed Central

    Liu, Peng; Lv, Xianli; Lv, Ming

    2016-01-01

    Purpose Here we present our experience with five cerebral proliferative angiopathy (CPA) patients to better delineate the clinical and angiographic features as well as the treatment selection of this disease. Methods Between October 2008 and October 2012, five consecutive patients diagnosed with CPA were admitted to our department in our hospital. All the five patients received magnetic resonance imaging, digital subtraction angiography, and positron emission computed tomography (PET) to definitively confirm this disease. We also collected 15 previously published instances of CPA to analyze the characteristics of this rare entity. Results As to the five patients, three were female and two were male, between the ages of 4 and 52 years with a mean age of 24.8 ± 20.6 years. The PET results showed that perfusion was decreased over the affected hemispheres in all five patients. As to the treatment, only one patient received encephalo-duro-arterio-synangiosis (EDAS) revascularization surgery. The other four patients were conservatively observed. During the follow-up period (range 3–6 years, mean 4 ± 1.9 years), the patient who underwent EDAS surgery manifested relief of clinical symptoms. In the conservative series, the symptoms in two patients aggravated and suffered permanent neurologic deficits. Conclusion CPA is a rare entity. Natural history has showed this disease is not stable and may progress at a certain time point. The EDAS procedure may be a treatment for CPA-related oligemia since there is currently little data and follow-up available. PMID:26472638

  14. Clinical features of celiac disease: a prospective birth cohort.

    PubMed

    Agardh, Daniel; Lee, Hye-Seung; Kurppa, Kalle; Simell, Ville; Aronsson, Carin Andrén; Jörneus, Ola; Hummel, Michael; Liu, Edwin; Koletzko, Sibylle

    2015-04-01

    To investigate clinical features of celiac disease (CD) and their association with risk factors for CD in a genetic risk birth cohort. Children from 6 clinical centers in 4 countries positive for HLA-DR3-DQ2 or DR4-DQ8 were annually screened for tissue transglutaminase antibodies (tTGA) and assessed for symptoms by questionnaires. Associations of symptoms with anthropometrics, known risk factors for CD, tTGA levels, and mucosal lesions in those biopsied were examined. Of 6706 screened children, 914 developed persistent positive tTGA, 406 underwent biopsies, and 340 had CD. Compared with age-matched tTGA-negative children, those with persistent tTGA were more likely to have symptoms at 2 (34% vs 19%, P < .001) and 3 years of age (28% vs 19%, P = .009) but not at 4 years (27% vs 21%, NS). Z-scores for height, weight, and BMI did not differ between groups. In children with persistent tTGA, having ≥ 1 symptom was associated with family history of CD (odds ratio = 2.59, 95% confidence interval, 1.21-5.57) but not with age, gender, or HLA-DR3-DQ2 homozygosity. At seroconversion, tTGA levels were higher in symptomatic than asymptomatic children (P < .001), in those from CD families (P < .001), and in US participants (P < .001) but not associated with age, gender, or HLA genotype. tTGA levels correlated with severity of mucosal lesions both in symptomatic (r = 0.53, P < .001) and asymptomatic children (r = 0.22, P = .01). A majority of children detected with persistent tTGA in screenings are asymptomatic and have normal growth by age 4 years. tTGA levels correlate more strongly with severity of mucosal lesions in symptomatic as compared with asymptomatic children. Copyright © 2015 by the American Academy of Pediatrics.

  15. Varicella zoster virus vasculopathy: clinical features and pathogenesis.

    PubMed

    Nagel, Maria A

    2014-04-01

    Varicella zoster virus (VZV) vasculopathy is caused by productive virus infection of cerebral arteries, leading to inflammation, pathological vascular remodeling, and ischemic or hemorrhagic stroke. VZV vasculopathy occurs in immunocompetent and immunocompromised individuals and involves both large and small vessels. MRI abnormalities include more deep-seated than superficial lesions, particularly at gray-white matter junctions, and lesions may enhance. Diagnosis is challenging, since stroke can occur months after zoster rash and in the absence of rash or CSF pleocytosis. The best virological test for diagnosis is detection of anti-VZV IgG antibody in the CSF. Pathological studies of VZV-infected arteries from patients with VZV vasculopathy reveal that the arterial adventitia is the initial site of infection, after which virus spreads transmuraly towards the lumen. Histological and immunohistochemical studies of VZV-infected arteries show a thickened intima, disrupted internal elastic lamina, and loss of smooth muscle cells, that likely contribute to weakening of the vessel wall and occlusion. Early in disease, VZV-infected arteries contain CD4+ and CD8+ T cells, macrophages, and rare B cells, in addition to abundant neutrophils in early disease. Importantly, perivascular inflammatory cells underlie the areas of thickened intima, raising the possibility that soluble factors secreted by these cells contribute to arterial remodeling. This review discusses the clinical features of VZV vasculopathy and potential mechanisms of VZV-induced cerebrovascular remodeling and stroke.

  16. Varicella zoster virus vasculopathy: clinical features and pathogenesis

    PubMed Central

    Nagel, Maria A.

    2013-01-01

    Varicella zoster virus (VZV) vasculopathy is caused by productive virus infection of cerebral arteries, leading to inflammation, pathological vascular remodeling and ischemic or hemorrhagic stroke. VZV vasculopathy occurs in immunocompetent and immunocompromised individuals and involves both large and small vessels. MRI abnormalities include more deep-seated than superficial lesions, particularly at grey-white matter junctions and lesions may enhance. Diagnosis is challenging since stroke can occur months after zoster rash and in the absence of rash or CSF pleocytosis. The best virological test for diagnosis is detection of anti-VZV IgG antibody in the CSF. Pathological studies of VZV-infected arteries from patients with VZV vasculopathy reveal that the arterial adventitia is the initial site of infection, after which virus spreads transmuraly towards the lumen. Histological and immunohistochemical studies of VZV-infected arteries show a thickened intima, disrupted internal elastic lamina and loss of smooth muscle cells, that likely contribute to weakening of the vessel wall and occlusion. Early in disease, VZV-infected arteries contain CD4+ and CD8+ T cells, macrophages and rare B-cells, in addition to abundant neutrophils in early disease. Importantly, perivascular inflammatory cells underlie the areas of thickened intima, raising the possibility that soluble factors secreted by these cells contribute to arterial remodeling. This review discusses the clinical features of VZV vasculopathy and potential mechanisms of VZV-induced cerebrovascular remodeling and stroke. PMID:23918503

  17. Pigmented lesions of the nail unit: clinical and histopathologic features.

    PubMed

    Ruben, Beth S

    2010-09-01

    Probably the most common reason to perform biopsy of the nail unit is for the evaluation of irregular pigmentation, especially longitudinal melanonychia or pigmented bands. When narrow and solitary, these are usually the product of melanocytic activation/hypermelanosis, lentigines, or melanocytic nevi. Multiple pigmented bands are generally a benign finding, the result of melanocytic activation, as seen in racial pigmentation in darker-skinned patients, for example. In the context of an irregular, broad, heterogeneous or "streaky" band, the chief concern is the exclusion of subungual melanoma. Before assessing the histologic features of any such entities, it is important to understand the normal nail anatomy and melanocytic density of nail unit epithelium, as well as the type of specimen submitted, and whether it is adequate to undertake a proper histologic evaluation. The criteria for diagnosis and prognosis of melanoma of the nail unit are still evolving, and a variety of factors must be weighed in the balance to make a correct diagnosis. The importance of the clinical context cannot be overemphasized. There are also nonmelanocytic conditions to be considered that may produce worrisome nail discoloration, such as subungual hemorrhage, squamous cell carcinoma, and pigmented onychomycosis.

  18. Eosinophilic colitis: epidemiology, clinical features, and current management

    PubMed Central

    Alfadda, Abdulrahman A.; Storr, Martin A.; Shaffer, Eldon A.

    2011-01-01

    Primary eosinophilic gastrointestinal disorders (EGIDs) represent a spectrum of inflammatory gastrointestinal disorders in which eosinophils infiltrate the gut in the absence of known causes for such tissue eosinophilia. EGIDs can be subgrouped as eosinophilic esophagitis (EE), eosinophilic gastroenteritis (EG), and eosinophilic colitis (EC). The least frequent manifestation of EGIDs is EC. EC is a heterogeneous entity with a bimodal age distribution, presenting with either an acute self-limited bloody diarrhea in otherwise healthy infants or as a more chronic relapsing colitis in young adults. The pathophysiology of primary EC appears related to altered hypersensitivity, principally as a food allergy in infants and T lymphocyte-mediated (i.e. non-IgE associated) in young adults. In adults, symptoms include diarrhea, abdominal pain, and weight loss. Endoscopic changes are generally modest, featuring edema and patchy granularity. Although standardized criteria are not yet established, the diagnosis of EC depends on histopathology that identifies an excess of eosinophils. Therapeutic approaches are based on case reports and small case series, as prospective randomized controlled trials are lacking. Eosinophilic colitis in infants is a rather benign, frequently food-related entity and dietary elimination of the aggressor often resolves the disorder within days. Adolescent or older patients require more aggressive medical management including: glucocorticoids, anti-histamines, leukotriene receptors antagonists as well as novel approaches employing biologics that target interleukin-5 (IL-5) and IgE. This review article summarizes the current knowledge of EC, its epidemiology, clinical manifestations, diagnosis, and treatment. PMID:21922029

  19. Clinical features and outcome of severe malaria in Gambian children.

    PubMed

    Waller, D; Krishna, S; Crawley, J; Miller, K; Nosten, F; Chapman, D; ter Kuile, F O; Craddock, C; Berry, C; Holloway, P A

    1995-09-01

    The clinical and laboratory features of severe falciparum malaria in 180 Gambian children were studied between 1985 and 1989. Of the 180 children, 118 (66%) presented with seizures, 77 (43%) had cerebral malaria, 35 (20%) had witnessed seizures after admission, 29 (16%) were hypoglycemic, and 27 (15%) died. Respiratory distress was a common harbinger of a fatal outcome. The differences in admission parasite counts in the blood, hematocrit, and opening cerebrospinal pressures for patients who died and survivors were not significant. A multiple logistic regression model identified neurological status (coma, particularly if associated with extensor posturing), stage of parasite development on the peripheral blood film, pulse rate of > 150 or respiratory rate of > 50, hypoglycemia, and hyperlactatemia (plasma lactate level, > 5 mmol/L) as independent indicators of a fatal outcome. Biochemical evidence of hepatic and renal dysfunction was an additional marker of a poor prognosis, but, in contrast to severe malaria in adults, none of these children with severe malaria had acute renal failure.

  20. Clinical features of serum sickness after Australian snake antivenom.

    PubMed

    Ryan, Nicole M; Downes, Michael A; Isbister, Geoffrey K

    2015-12-15

    Serum sickness is a delayed immune reaction in which the immune system responds to a protein in antiserum as a potentially harmful substance and mounts an IgG-mediated antibody response. A 32 year-old female patient had systemic envenoming following a bite by a red-bellied black snake (Pseudechis porphyriacus). She was treated with Tiger snake antivenom and recovered over 24 h and did not develop myotoxicity. She then presented with local pain, itching and swelling, which was partially treated with antihistamines. Eleven days after the bite she presented again with symptoms of worsening serum sickness including rash on the upper legs, joint and muscle pain in arms, ankles and knees, and nausea. The patient was prescribed five days of prednisone 50 mg/day, antihistamine 10 mg/day and analgesia 1000 mg/day and improved over 2 days. She had no further problems on follow up at 4 months. This case highlights that serum sickness can cause significant effects after the treatment of snake envenoming. It develops 5-14 days after antivenom administration and has characteristic clinical and laboratory features. Severe cases of serum sickness can result in morbidity but it appears to respond well to corticosteroid treatment. Copyright © 2015 Elsevier Ltd. All rights reserved.

  1. Risk factors and clinical features of text message injuries.

    PubMed

    Sharan, Deepak; Ajeesh, P S

    2012-01-01

    Use of mobile phone and sending text message is a very common in today's life. While sending a text message the users need to use their thumb and other palm muscles extensively. The thumb most of the time adducted on the key pad of the mobile and use high force to type the letters. Studies in literature showed that text messaging has an adverse effect on musculoskeletal system of hand. But the extensive study on the type of disorders set in among the users who extensively use mobile phone for texting. This study aims at to evaluate risk factor and clinical feature of the MSD due to hand held devices. Twenty seven subjects participated in this study. Predefined protocols were used to evaluate type of MSD occurred among the subjects. The study revealed that development of tendinitis in extensor pollicis longus, myofascial pain syndrome (70.37%) of adductor pollicis, 1st interossei and extensor digitorum communis . Other associated problems diagnosed were thoracic outlet syndrome (51.85%), fibromyalgia syndrome (25.93%), hypothyroidism (7.41%), wrist tendinitis (14.81%) and De Quervain's syndrome (7.41%). It has been observed that the pathology were tendinitis of extensor pollicis longus, myofascial pain syndrome of thenar muscles and 1st interossei, extensor digitorum communis.

  2. Delta hepatitis: molecular biology and clinical and epidemiological features.

    PubMed Central

    Polish, L B; Gallagher, M; Fields, H A; Hadler, S C

    1993-01-01

    Hepatitis delta virus, discovered in 1977, requires the help of hepatitis B virus to replicate in hepatocytes and is an important cause of acute, fulminant, and chronic liver disease in many regions of the world. Because of the helper function of hepatitis delta virus, infection with it occurs either as a coinfection with hepatitis B or as a superinfection of a carrier of hepatitis B surface antigen. Although the mechanisms of transmission are similar to those of hepatitis B virus, the patterns of transmission of delta virus vary widely around the world. In regions of the world in which hepatitis delta virus infection is not endemic, the disease is confined to groups at high risk of acquiring hepatitis B infection and high-risk hepatitis B carriers. Because of the propensity of this viral infection to cause fulminant as well as chronic liver disease, continued incursion of hepatitis delta virus into areas of the world where persistent hepatitis B infection is endemic will have serious implications. Prevention depends on the widespread use of hepatitis B vaccine. This review focuses on the molecular biology and the clinical and epidemiologic features of this important viral infection. PMID:8358704

  3. Cytogenetics and clinical features of pediatric myelodysplastic syndrome in Japan.

    PubMed

    Moriwaki, Koichi; Manabe, Atsushi; Taketani, Takeshi; Kikuchi, Akira; Nakahata, Tatsutoshi; Hayashi, Yasuhide

    2014-11-01

    We analyzed the cytogenetics and clinical features of pediatric myelodysplastic syndrome (MDS) in Japan. Data on patients (<16 years) diagnosed with MDS from 1990 to 2000 were retrospectively collected from pediatric hematologists in 234 institutions. Chromosome analysis was successfully performed in 255 of 277 MDS patients. The numbers of patients with refractory anemia, refractory anemia with ringed sideroblasts, refractory anemia with excess of blasts (RAEB), refractory anemia with excess of blasts in transformation (RAEBt), chronic myelomonocytic leukemia, and juvenile myelomonocytic leukemia were 67 (24%), 51 (18%), 51 (18%), 20 (7%), and 65 (23%), respectively. The other 23 patients (8%) could not be classified specifically. The distribution of childhood MDS in Japan according to the French-American-British subclassification was similar to that in other countries. However, we identified a higher incidence of therapy-related cases. As for relationship between cytogenetics and prognoses, abnormal karyotypes were related to poorer prognoses than normal karyotype (P < 0.01). However, patients with trisomy 8 had prognoses comparable to those with normal karyotypes. Complex karyotypes were associated with poorer prognoses among RAEB and RAEBt patients. In conclusion, prognosis of pediatric MDS is related to cytogenetics. A more precise diagnosis and classification system is needed for childhood MDS.

  4. Congenital Aural Stenosis: Clinical Features and Long-term Outcomes

    PubMed Central

    Li, Chen-long; Chen, Ying; Chen, Yong-zheng; Fu, Yao-yao; Zhang, Tian-yu

    2016-01-01

    The aim of the present study was to comprehensively evaluate the clinical features and long-term outcomes of congenital aural stenosis (CAS). This study presents a retrospective review of patients who underwent meatoplasty for CAS at a tertiary referral hospital from 2008 to 2015. A total of 246 meatoplasty procedures were performed on 232 patients in the present study. We performed multivariate regression analysis. Except in the age < 6 years group, no significant difference was observed among different age groups for cholesteatoma formation, p > 0.05. Except for the stenosis of the external auditory canal (EAC) (>4 mm) group, the other stenosis of EAC groups were not associated with cholesteatoma formation, p > 0.05. Postoperative air-bone gaps (ABG) less than 30 dB occurred in 77.3% (99/128) of the patients, and the Jahrsdoerfer score was associated with postoperative ABG, p < 0.001. The complication rate of CAS was 13.8% (20/144), and males showed a higher risk for postoperative complications (OR, 6.563; 95% CI, 1.268–33.966, p = 0.025). These results indicate that meatoplasty was an effective surgical intervention for CAS, showing a stable hearing outcome with prolonged follow-up. There was no significant difference between the cholesteatoma and no cholesteatoma groups for hearing outcomes, p > 0.05. PMID:27257165

  5. [The clinical features of indium-related lung diseases].

    PubMed

    Guo, Kongrong; Liu, Jia; Zhang, Jingbo; Sun, Daoyuan

    2015-08-01

    To discuss the clinical features of Indium-related lung diseases. We searched database of Chinese and Pubmed, Embase, Web of Science to collect research data of indium-related lung diseases from Jan. 1998 to Aprl. 2014. Case reports, exposure histories and lab results were analysed and summarized. 1998 to Mar 2010, ten cases of indium-related lung diseases were published. Seven cases of interstitial pneumonia were reported in Japan, two cases of pulmonary alveolar proteinosis (PAP) were reported in the USA and one case of PAP reported in China. Chest computer tomography (CT) showed diffuse or local ground glass appearance (GGA) in 8 cases, 3 of which also showed centrilobular nodules; Pulmonary function test were normal only in one out of 8 cases. Cholesterol clefts were found in 4 cases of interstitial pneumonia. 3 cases died among 6 cases who were followed-up. Occupational exposure to indium compounds are contributory to different pulmonary diseases, which are composed of interstitial pneumonia and pulmonary alveolar proteinosis. The relationships between In-C, In-S and these pulmonary diseases are unclear.

  6. Cytokine profiles in localized scleroderma and relationship to clinical features.

    PubMed

    Kurzinski, Katherine; Torok, Kathryn S

    2011-08-01

    Localized scleroderma (LS) is a disfiguring autoimmune disease of the skin and underlying tissue that mainly affects the pediatric population. Inflammation of the tissue leads to fibrosis and atrophy, causing physical and psychological disability that can continue throughout childhood into adulthood. Available therapies for LS have had variable effects and are associated with morbidity themselves. A better understanding of the pathophysiology of LS, especially during the active inflammatory phase, would lead to more directed and efficacious therapies. As in systemic sclerosis (SSc), the other form of scleroderma, T-helper (Th) cells and their associated cytokines have been suggested to contribute significantly to the pathophysiology of LS supported by the presence of cytokines from these lineages in the sera and tissue of LS patients. It is postulated that the imbalance between Th1/Th2/Th17 cell subsets drives inflammation in the early stages of disease (Th1 and Th17 predominant) and fibrosis in the later stages of scleroderma (Th2 predominant). We review the available experimental data regarding cytokines in LS and compare them to available clinical disease severity and activity features. This provides the platform to launch further investigations into the role of select cytokines in the pathogenesis of LS and to provide directed therapeutic options in the future. Published by Elsevier Ltd.

  7. Chronic intestinal pseudo-obstruction: clinical features, diagnosis, and therapy.

    PubMed

    De Giorgio, Roberto; Cogliandro, Rosanna F; Barbara, Giovanni; Corinaldesi, Roberto; Stanghellini, Vincenzo

    2011-12-01

    CIPO is the very “tip of the iceberg” of functional gastrointestinal disorders, being a rare and frequently misdiagnosed condition characterized by an overall poor outcome. Diagnosis should be based on clinical features, natural history and radiologic findings. There is no cure for CIPO and management strategies include a wide array of nutritional, pharmacologic, and surgical options which are directed to minimize malnutrition, promote gut motility and reduce complications of stasis (ie, bacterial overgrowth). Pain may become so severe to necessitate major analgesic drugs. Underlying causes of secondary CIPO should be thoroughly investigated and, if detected, treated accordingly. Surgery should be indicated only in a highly selected, well characterized subset of patients, while isolated intestinal or multivisceral transplantation is a rescue therapy only in those patients with intestinal failure unsuitable for or unable to continue with TPN/HPN. Future perspectives in CIPO will be directed toward an accurate genomic/proteomic phenotying of these rare, challenging patients. Unveiling causative mechanisms of neuro-ICC-muscular abnormalities will pave the way for targeted therapeutic options for patients with CIPO.

  8. Clinical and Immunological Features of Common Variable Immunodeficiency in China

    PubMed Central

    Lin, Lian-Jun; Wang, Yu-Chuan; Liu, Xin-Min

    2015-01-01

    Background: Common variable immunodeficiency (CVID) is one of the most common symptomatic primary immunodeficiency syndromes. The purpose of this article was to broaden our knowledge about CVID for better diagnosis and treatment. Methods: Clinical and immunological features of 40 Chinese patients with CVID were analyzed retrospectively. Results: The median age at onset was 11-year-old (range 4–51 years). The median age at diagnosis was 14.5-year-old (range 5–66 years). The average time of delay in diagnosis was 5.3 years (range 1–41 years). The most common main complaint was fever due to infections (35 cases, 87.5%). Pneumonia (28 cases, 70%) was the most common type of infections. Bronchiectasis was present in 6 patients (15%). Autoimmune disease was detected in 6 cases of CVID, and malignancy in 2 cases. The median total serum levels of IgG, IgA, and IgM at diagnosis were 1.07 g/L, 0.07 g/L, and 0.28 g/L, respectively. The percentages of CD3−/CD10+ B-cells were 1%–3.14%. Conclusions: Infection is the most frequent presentation of CVID. Patients with unexplainable infections should receive further examination including serum immunoglobulin (Ig) and lymphocyte subset analysis. Regular and sufficient substitution with Ig is recommended. PMID:25635425

  9. [Intermittent priapism as a clinical feature of lumbar spinal stenosis].

    PubMed

    Rojas, J I; Zurrú-Ganen, M C; Romano, M; Patrucco, L; Cristiano, E

    Lumbar spinal stenosis is defined as a narrowing of the neural canal and foramina that result in compression of the lumbosacral nerve roots or cauda equina. Patients with lumbar spinal stenosis may present a variety of signs and symptoms. One such syndrome is neurogenic intermittent claudication, characterized by radicular symptoms exacerbated by walking or standing and relieved by rest. Infrequently, lumbar spinal stenosis produces a cauda equina compression, characterized by intermittent urinary or fecal incontinence, impotence and in rare cases priapism. A 50 year-aged male, presented with spontaneous intermittent priapism and few months later weakness, numbness and pain of his legs provoked by bipedestation or physical exertion that completely disappeared by sitting or lying down. A computed tomographic scan showed a lumbar canal narrowing of L4 through L5. A diagnosis of neurogenic intermittent claudication with dysfunction of the cauda equina roots secondary to the presence of lumbar spinal stenosis was carried out. The symptoms completely resolved after descompressive lumbar laminectomy of L4 and L5. Causal interpretation of neurogenic intermittent claudication still remains obscure. An inadequate blood supply provoked by increased intra-raquid pressure among the roots may act as a dynamic factor. This mechanism could cause stagnant anoxia during the exercise resulting in clinical symptoms. Lumbar spinal stenosis should be kept in mind when autonomic features appear.

  10. Clinical and histological features of nonalcoholic steatohepatitis in Iranian patients

    PubMed Central

    Bahrami, Hossein; Daryani, Nasser Ebrahimi; Mirmomen, Shahram; Kamangar, Farin; Haghpanah, Babak; Djalili, Mehdi

    2003-01-01

    Background Although several studies have been performed on risk factors and natural course of NASH, it seems that NASH tends to be more than a disease confined to strict boundaries. The objective of this study was to assess the clinical and paraclinical features and risk factors for non-alcoholic steatohepatitis (NASH) patients in an Iranian population Methods Patients with histologically confirmed NASH who had elevated liver aminotransaminases, negative serologic markers of viral or autoimmune hepatitis and no findings in favor of metabolic liver disease were enrolled. A careful history was taken regarding alcohol intake. Results 53 patients consisting of 32 male and 21 female entered the study. The mean age was 37.8 ± 11.3 years. Twenty-six patients (55.3%) were overweight, 15 (31.9%) obese, 40 (75.5%) dyslipidemic, and three patients (5.7%) were diabetic. Liver biopsy showed mild steatosis in 35.7%, moderate steatosis in 53.6%, and severe forms in 10.7%. In 80.2% of patients, portal inflammation was present, and 9.4% had cirrhosis. The amount of increase in liver enzymes bore no relationship with fibrosis, portal inflammation, and degree of steatosis. Conclusions The patients in our study showed a male predominancy and were somewhat younger than other studies. PMID:14561231

  11. The clinical features and treatment of pediatric intracranial aneurysm.

    PubMed

    Liang, Jiantao; Bao, Yuhai; Zhang, Hongqi; Wrede, Karsten Henning; Zhi, Xinglong; Li, Meng; Ling, Feng

    2009-03-01

    Intracranial aneurysms are relatively rare in the pediatric population. The objective of this study was to highlight the clinical and radiological features and the therapeutic outcome and clarify the choice of therapeutic strategies for pediatric intracranial aneurysms. Twenty-four consecutive children (age

  12. Epidemiology and clinical features of pediatric psoriasis in tertiary referral psoriasis clinic.

    PubMed

    Kwon, Hyuck Hoon; Na, Sun Jae; Jo, Seoung Jin; Youn, Jai Il

    2012-03-01

    Few epidemiological studies of pediatric patients with moderate to severe psoriasis have been available despite there being no approved systemic therapy for these patients. The aim of the present study was to elucidate clinical features of pediatric psoriasis in a tertiary referral psoriasis clinic. We analyzed the clinical data of 358 patients under 18 years of age referred to our clinic from other private clinics and medical centers. Our data showed a male :female ratio of 1.06:1 and a peak age of onset of 10-11 years. Of the patients, 32.4% had a positive family history. The most prevalent phenotype was plaque type (67.3%) and the mean Psoriasis Area and Severity Index score was 17.2 ± 12.7. The most frequently affected body part was the trunk (69.5%), followed by the legs (65.3%). Exposure to sunlight and summer season improved psoriatic lesions, while stress and winter season aggravated the clinical course. Only 26.0% of patients received systemic therapy or phototherapy during the therapeutic course. Oral acitretin (11.2%) was most frequently used followed by ultraviolet B phototherapy (7.3%). The childhood group (<13 years) showed higher prevalence of guttate and generalized pustular phenotypes and more severe clinical course compared with the adolescent group (13-18 years). In conclusion, our patients showed distinctive features in clinical phenotypes, disease severity and affected body parts compared with previous reports. We also found that clinical application of systemic therapies were limited considering the severe disease state of our patients, demanding a need for more research on treatment of pediatric psoriasis.

  13. Clinical and genetic features of Huntington disease in Sri Lanka.

    PubMed

    Sumathipala, Dulika S; Jayasekara, Rohan W; Dissanayake, Vajira H W

    2013-12-05

    Huntington disease was one of the first neurological hereditary diseases for which genetic testing was made possible as early as 1993. The study describes the clinical and genetic characteristics of patients with Huntington disease in Sri Lanka. Data of 35 consecutive patients tested from 2007 to 2012 at the Human Genetics Unit, Faculty of Medicine, University of Colombo was analyzed retrospectively. Clinical data and genetic diagnostic results were reviewed. Statistical analysis was performed using descriptive statistics. Thirty patients had fully penetrant (FP) CAG repeat mutations and 5 had reduced penetrant (RP) CAG repeat mutations. In the FP group mean ages of onset and diagnosis were 37.5 and 40.4 years, while in the RP group it was 63.0 and 64.8 years respectively. The age of diagnosis ranged from 15 to 72 years, with 2 patients with Juvenile onset (<20 years) and 3 with late onset (>60 years) Huntington disease. The symptoms at diagnosis were predominantly motor (32/35 -91%). Three patients had psychiatric and behavioral disorders. The age difference between onset and genetic diagnosis showed significant delay in females compared to males (p < 0.05). Twenty two (62.8%) had a positive family history, with 13/22 (59.1%) showing a paternal inheritance of the disease. In both groups, those with a family history had a significantly lower age of presentation (p < 0.05). The mean CAG repeat length in patients with FP alleles was 44.6 ± 5 and RP alleles was 37.2 ± 1.1. Age of onset and CAG repeat length of the HTT gene showed significant inverse correlation (p < 0.0005, R2 = 0.727). The clinical and genetic features seen in patients with Huntington disease in the Sri Lankan study population were similar to that previously reported in literature.

  14. Clinical, Epidemiologic, Histopathologic and Molecular Features of an Unexplained Dermopathy

    PubMed Central

    Pearson, Michele L.; Selby, Joseph V.; Katz, Kenneth A.; Cantrell, Virginia; Braden, Christopher R.; Parise, Monica E.; Paddock, Christopher D.; Lewin-Smith, Michael R.; Kalasinsky, Victor F.; Goldstein, Felicia C.; Hightower, Allen W.; Papier, Arthur; Lewis, Brian; Motipara, Sarita; Eberhard, Mark L.

    2012-01-01

    Background Morgellons is a poorly characterized constellation of symptoms, with the primary manifestations involving the skin. We conducted an investigation of this unexplained dermopathy to characterize the clinical and epidemiologic features and explore potential etiologies. Methods A descriptive study was conducted among persons at least 13 years of age and enrolled in Kaiser Permanente Northern California (KPNC) during 2006–2008. A case was defined as the self-reported emergence of fibers or materials from the skin accompanied by skin lesions and/or disturbing skin sensations. We collected detailed epidemiologic data, performed clinical evaluations and geospatial analyses and analyzed materials collected from participants' skin. Results We identified 115 case-patients. The prevalence was 3.65 (95% CI = 2.98, 4.40) cases per 100,000 enrollees. There was no clustering of cases within the 13-county KPNC catchment area (p = .113). Case-patients had a median age of 52 years (range: 17–93) and were primarily female (77%) and Caucasian (77%). Multi-system complaints were common; 70% reported chronic fatigue and 54% rated their overall health as fair or poor with mean Physical Component Scores and Mental Component Scores of 36.63 (SD = 12.9) and 35.45 (SD = 12.89), respectively. Cognitive deficits were detected in 59% of case-patients and 63% had evidence of clinically significant somatic complaints; 50% had drugs detected in hair samples and 78% reported exposure to solvents. Solar elastosis was the most common histopathologic abnormality (51% of biopsies); skin lesions were most consistent with arthropod bites or chronic excoriations. No parasites or mycobacteria were detected. Most materials collected from participants' skin were composed of cellulose, likely of cotton origin. Conclusions This unexplained dermopathy was rare among this population of Northern California residents, but associated with significantly reduced health-related quality of

  15. Clinical, epidemiologic, histopathologic and molecular features of an unexplained dermopathy.

    PubMed

    Pearson, Michele L; Selby, Joseph V; Katz, Kenneth A; Cantrell, Virginia; Braden, Christopher R; Parise, Monica E; Paddock, Christopher D; Lewin-Smith, Michael R; Kalasinsky, Victor F; Goldstein, Felicia C; Hightower, Allen W; Papier, Arthur; Lewis, Brian; Motipara, Sarita; Eberhard, Mark L

    2012-01-01

    Morgellons is a poorly characterized constellation of symptoms, with the primary manifestations involving the skin. We conducted an investigation of this unexplained dermopathy to characterize the clinical and epidemiologic features and explore potential etiologies. A descriptive study was conducted among persons at least 13 years of age and enrolled in Kaiser Permanente Northern California (KPNC) during 2006-2008. A case was defined as the self-reported emergence of fibers or materials from the skin accompanied by skin lesions and/or disturbing skin sensations. We collected detailed epidemiologic data, performed clinical evaluations and geospatial analyses and analyzed materials collected from participants' skin. We identified 115 case-patients. The prevalence was 3.65 (95% CI = 2.98, 4.40) cases per 100,000 enrollees. There was no clustering of cases within the 13-county KPNC catchment area (p = .113). Case-patients had a median age of 52 years (range: 17-93) and were primarily female (77%) and Caucasian (77%). Multi-system complaints were common; 70% reported chronic fatigue and 54% rated their overall health as fair or poor with mean Physical Component Scores and Mental Component Scores of 36.63 (SD = 12.9) and 35.45 (SD = 12.89), respectively. Cognitive deficits were detected in 59% of case-patients and 63% had evidence of clinically significant somatic complaints; 50% had drugs detected in hair samples and 78% reported exposure to solvents. Solar elastosis was the most common histopathologic abnormality (51% of biopsies); skin lesions were most consistent with arthropod bites or chronic excoriations. No parasites or mycobacteria were detected. Most materials collected from participants' skin were composed of cellulose, likely of cotton origin. This unexplained dermopathy was rare among this population of Northern California residents, but associated with significantly reduced health-related quality of life. No common underlying medical

  16. [Clinical and myopathological features of Jo-1 syndrome].

    PubMed

    Meng, L C; Li, Y; Zhang, W; Hao, H J; Gao, F; Yuan, Y

    2016-08-02

    To report the clinical and myopathological features of 16 patients with Jo-1 syndrome. Sixteen patients were recruited in this study, who were diagnosed as Jo-1 syndrome in Department of Neurology of Peking University First Hospital from January, 2011 to July, 2015. The clinical data and myopathological data were analyzed. The mean onset age was 41±14 (21-68) years old. 87.5% was female. The median duration was 9.5 months (1-192 months). The main clinical manifestations were weakness in 13 cases (81.2%), arthritis in 10 cases (62.5%), interstitial lung diseases in 8 cases (50%), dermatomyositis-like skin lesions in 5 cases (31.2%), fever in 3 cases (18.8%), Raynaud's phenomenon in 2 cases (12.5%) and mechanic's hands in 2 cases (12.5%). There were 3 cases with other connective tissue diseases and 1 case with non-Hodgkin's lymphoma. Mean serum CK was 3 054±2 058(470-5 222) U/L. All patients had anti-Jo-1antibody, combined with anti- Mi-2 antibody in 1 case, anti-Ro-52 antibody in 5 cases, and anti-nuclear antibody in 5 cases. 4/5 cases showed myopathic changes for electromyography (EMG) tests. Myopathological changes included edema, fragmentation and inflammatory infiltration in perimysium in 14 cases (87.5%), muscle atrophy in 13 cases including 7 cases(43.8%) predominantly in perifascicular field. Muscle fiber necrosis appeared in 8 cases with predominantly in perifascicular area in 4 cases (25%). Muscle fiber regeneration occurred in 11 cases with predominantly in perifascicular field in 5 cases (31.2%). CD8 positive T-lymphocytes, CD20 positive B-lymphocytes and CD68 positive macrophages infiltrated in various degrees, most of which were located in perimysium. MHC-Ⅰ were expressed on muscle fiber membranes in different degrees, including 7 cases (43.8%) predominantly in the cytoplasm of perifascicular muscle fibers. C5b-9 deposited in perifascicular muscle fiber membranes in 7 cases (43.8%) and perifascicular capillaries in 2 cases (12.5%). The main

  17. Molecular identification and amphotericin B susceptibility testing of clinical isolates of Aspergillus from 11 hospitals in Korea.

    PubMed

    Heo, Min Seok; Shin, Jong Hee; Choi, Min Ji; Park, Yeon Joon; Lee, Hye Soo; Koo, Sun Hoe; Lee, Won Gil; Kim, Soo Hyun; Shin, Myung Geun; Suh, Soon Pal; Ryang, Dong Wook

    2015-11-01

    We investigated the species distribution and amphotericin B (AMB) susceptibility of Korean clinical Aspergillus isolates by using two Etests and the CLSI broth microdilution method. A total of 136 Aspergillus isolates obtained from 11 university hospitals were identified by sequencing the internal transcribed spacer (ITS) and β-tubulin genomic regions. Minimal inhibitory concentrations (MICs) of AMB were determined in Etests using Mueller-Hinton agar (Etest-MH) and RPMI agar (Etest-RPG), and categorical agreement with the CLSI method was assessed by using epidemiological cutoff values. ITS sequencing identified the following six Aspergillus species complexes: Aspergillus fumigatus (42.6% of the isolates), A. niger (23.5%), A. flavus (17.6%), A. terreus (11.0%), A. versicolor (4.4%), and A. ustus (0.7%). Cryptic species identifiable by β-tubulin sequencing accounted for 25.7% (35/136) of the isolates. Of all 136 isolates, 36 (26.5%) had AMB MICs of ≥2 μg/mL by the CLSI method. The categorical agreement of Etest-RPG with the CLSI method was 98% for the A. fumigatus, A. niger, and A. versicolor complexes, 87% for the A. terreus complex, and 37.5% for the A. flavus complex. That of Etest-MH was ≤75% for the A. niger, A. flavus, A. terreus, and A. versicolor complexes but was higher for the A. fumigatus complex (98.3%). Aspergillus species other than A. fumigatus constitute about 60% of clinical Aspergillus isolates, and reduced AMB susceptibility is common among clinical isolates of Aspergillus in Korea. Molecular identification and AMB susceptibility testing by Etest-RPG may be useful for characterizing Aspergillus isolates of clinical relevance.

  18. Molecular Identification and Amphotericin B Susceptibility Testing of Clinical Isolates of Aspergillus From 11 Hospitals in Korea

    PubMed Central

    Heo, Min Seok; Choi, Min Ji; Park, Yeon-Joon; Lee, Hye Soo; Koo, Sun Hoe; Lee, Won Gil; Kim, Soo Hyun; Shin, Myung-Geun; Suh, Soon-Pal; Ryang, Dong-Wook

    2015-01-01

    Background We investigated the species distribution and amphotericin B (AMB) susceptibility of Korean clinical Aspergillus isolates by using two Etests and the CLSI broth microdilution method. Methods A total of 136 Aspergillus isolates obtained from 11 university hospitals were identified by sequencing the internal transcribed spacer (ITS) and β-tubulin genomic regions. Minimal inhibitory concentrations (MICs) of AMB were determined in Etests using Mueller-Hinton agar (Etest-MH) and RPMI agar (Etest-RPG), and categorical agreement with the CLSI method was assessed by using epidemiological cutoff values. Results ITS sequencing identified the following six Aspergillus species complexes: Aspergillus fumigatus (42.6% of the isolates), A. niger (23.5%), A. flavus (17.6%), A. terreus (11.0%), A. versicolor (4.4%), and A. ustus (0.7%). Cryptic species identifiable by β-tubulin sequencing accounted for 25.7% (35/136) of the isolates. Of all 136 isolates, 36 (26.5%) had AMB MICs of ≥2 µg/mL by the CLSI method. The categorical agreement of Etest-RPG with the CLSI method was 98% for the A. fumigatus, A. niger, and A. versicolor complexes, 87% for the A. terreus complex, and 37.5% for the A. flavus complex. That of Etest-MH was ≤75% for the A. niger, A. flavus, A. terreus, and A. versicolor complexes but was higher for the A. fumigatus complex (98.3%). Conclusions Aspergillus species other than A. fumigatus constitute about 60% of clinical Aspergillus isolates, and reduced AMB susceptibility is common among clinical isolates of Aspergillus in Korea. Molecular identification and AMB susceptibility testing by Etest-RPG may be useful for characterizing Aspergillus isolates of clinical relevance. PMID:26354348

  19. The clinical impact of thalidomide maintenance after autologous stem cell transplantation in patients with newly diagnosed multiple myeloma in real clinical practice of Korea.

    PubMed

    Lee, Ho Sup; Min, Chang-Ki; Lee, Je-Jung; Kim, Kihyun; Kim, Seok Jin; Yoon, Dok Hyun; Eom, Hyeon-Seok; Lee, Hyewon; Lee, Won Sik; Shin, Ho-Jin; Lee, Ji Hyun; Park, Yong; Jo, Jae-Cheol; Do, Young Rok; Mun, Yeung-Chul; Lee, Mark Hong

    2016-05-01

    In real clinical settings (not clinical trials), thalidomide has been accepted as maintenance therapy to patients with multiple myeloma (MM) because of the cost of drugs, the limitations of medical insurance, etc., in our country (South Korea). The purpose of this study was to evaluate the utility of thalidomide maintenance for improving survival in transplantation-eligible patients with MM in the real clinical field. Differences in survival rates were estimated in patients treated with or without thalidomide maintenance. The 3-year progression-free survival rates (PFS) of patients with and without maintenance, respectively, were 55.4 and 37.2 % (p = 0.005). The 3-year overall survival rates (OS) were 88.0 and 84.0 % (p = 0.105). No difference in 3-year OS after relapse or progression (OS2) was observed between the two groups (50.4 and 55.3 %, p = 0.661). The 3-year PFS of patients with and without maintenance therapy who had shown less than CR after ASCT were 68.4 and 23.3 % (p < 0.001). In conclusion, Thalidomide maintenance therapy showed longer PFS in real clinical practice, and long-term use of thalidomide did not interfere with the efficacy of salvage chemotherapy in patients who experienced progression or relapse after ASCT. In addition, thalidomide maintenance might be also useful for patients who have shown less than CR after ASCT.

  20. [Clinical and morphological features of different types of Castleman's disease].

    PubMed

    Melikyan, A L; Egorova, E K; Kovrigina, А М; Subortseva, I N; Gilyazitdinova, E A; Karagyulyan, S R; Silaev, M A; Gemdzhian, E G; Savchenko, V G

    2015-01-01

    To study the clinical features of Castleman's disease (CD) and to elaborate therapeutic approaches in its different morphological types. The clinical and laboratory data were studied in 59 prospectively examined patients and 17 retrospectively examined ones with CD who had been treated at the Outpatient Department, Hematology Research Centre, in 1996 to 2014. There were a total of 37 men (median age, 36 years) and 39 women (median age, 34 years). The diagnosis was established from the results of histological and immunohistochemical examinations of removed lymph nodes (LN) or tumors in all the cases. A hyaline vascular variant (HVV) with local LN involvement was diagnosed in 38 (50%) patients; a plasma cell variant (PCV) was in 38 (50%); among the latter, 17 (22%) patients were found to have local involvement and 21 (28%) had generalized (multicentriC) involvement (multicentric Castleman's diseases (MCD)). Five (24%) patients with MCD were established to be infected with human herpesvirus type 8 (HHV-8). HVV was more frequently diagnosed in women (4%) than in men (29%); PCV was equally common in both men (47%) and women (53%); MCD was statistically significantly more frequently encountered in men (86%) than in women (14%) (p=0.05). The basic involvement areas in local HVV and PCV were peripheral (38%), mediastinal (29), retroperitoneal (18%), abdominal (9%), and small pelvic (6%) LNs. HVV and local PCV were benign and these were cured by surgical removal of LNs involved in the pathological process. MCD took its aggressive course with obvious constitutional symptoms, generalized lymphadenopathy, hepatosplenomegaly, hypergammaglobulinemia, autoimmune hemolysis, thrombocytopenia, and involvement of extranodal foci in the pathological process. MCD transformation to plasmablastic lymphoma was observed in 4 of the 5 HHV8-positive patients and followed by a poor outcome. The prognosis of untreated MCD was unfavorable. In a number of cases prednisolone monotherapy worsened

  1. Clinical features of IgG4-related rhinosinusitis.

    PubMed

    Hanaoka, Machiko; Kammisawa, Terumi; Koizumi, Satomi; Kuruma, Sawako; Chiba, Kazuro; Kikuyama, Masataka; Shirakura, Satoshi; Sugimoto, Taro; Hishima, Tsunekazu

    2017-05-30

    IgG4-related disease is a systemic disease that affects various organs of the body. Aim of this study is to elucidate the clinical characteristics of IgG4-related rhinosinusitis. Clinical features, laboratory findings, radiological and endoscopic findings, associated disease, treatment and prognosis were retrospectively examined in 10 patients with IgG4-related rhinosinusitis. The age was 59.1±11.3 years old and male-to-female ratio was 1:1. The chief nasal complaints were hyposmia (n=4), nasal obstruction (n=3), and nothing (n=3). Serum IgG4 levels were elevated in all patients and the value was 740.4±472.4mg/dl. Other IgG4-related diseases were associated in all 10 patients, including IgG4-related sialadenitis (n=6), IgG4-related dacryoadenitis (n=5), and autoimmune pancreatitis (n=5). Imaging findings on CT/MRI were obstruction of the way of elimination (n=10), thickening of the sinus mucous membrane (n=10), and fluid in the sinus (n=6). All of the cases had bilateral findings. Nasal endoscopic findings were chiefly deviated nasal septum (n=5), polyps (n=4), edema of the mucous membrane (n=3). Histologically, abundant infiltration of IgG4 positive plasma cell and lymphocyte and an elevated IgG4+/IgG+ cell ration was detected in all 8 patients and 5 patients, respectively. Endoscopic sinus surgery was performed in 8 patients. Eight patients were treated with steroid therapy for other associated IgG4-related diseases. Symptoms improved in all 6 patients after an initial treatment (endoscopic surgery (n=5) and steroids (n=1)), but one patient suffered relapse. IgG4-related rhinosinusitis is a distinct entity of IgG4-related disease, and is associated in patients with multiple IgG4-related diseases. Copyright © 2017 Medical University of Bialystok. Published by Elsevier B.V. All rights reserved.

  2. Bullous X linked retinoschisis: clinical features and prognosis.

    PubMed

    Hinds, Anne-Marie; Fahim, Abigail; Moore, Anthony T; Wong, Sui Chien; Michaelides, Michel

    2017-08-28

    A subset of patients with X linked retinoschisis (XLRS) have bullous schisis cavities in the peripheral retina. This study describes the characteristics and prognosis of the bullous form of XLRS. A retrospective case series was performed of nine patients with molecularly proven bullous XLRS seen at a single tertiary centre. All cases of bullous peripheral schisis were bilateral, with one unilateral case at presentation which developed into bilateral bullous schisis over time. The mean age of onset was 1.9 years (range: 1 month-7 years, SD: 2.1 years) and at clinical diagnosis was 5.9 years (range: 1 month-27 years, SD: 9.0 years). Mean follow-up was 11 years (range: 6 months-36 years, SD: 10.8 years). Strabismus was the most common presentation (n=7). Other presenting complaints included decreased vision, floaters and an irregularly shaped pupil. The most frequently associated ocular features were strabismus (100%), vitreous haemorrhage (4/18 eyes, 22%), nystagmus (2/9, 22%) and persistent fetal vasculature (1/18, 6%). Localised tractional detachment was seen in 2/18 (11%) eyes, total detachment that underwent surgical repair in 1/18 (6%) and pigmented demarcation lines in a further 22% of the eyes. There was one eye with exudative retinal detachment. In XLRS, bullous schisis may be congenital or develop soon after birth and most commonly presents with strabismus. Cases may be complicated by some form of retinal detachment, which may be tractional or a Coats-like exudative detachment. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  3. Clinical features of polyhydramnios associated with fetal anomalies.

    PubMed

    Hara, Kikue; Kikuchi, Akihiko; Miyachi, Keiko; Sunagawa, Sorahiro; Takagi, Kimiyo

    2006-12-01

    The purpose of this study was to examine the clinical features of pregnancy complicated by polyhydramnios associated with fetal anomalies. Sixty-nine patients with a singleton pregnancy complicated by polyhydramnios were retrospectively analyzed. Based on prenatal ultrasonographic findings, 13 cases were considered to have idiopathic polyhydramnios and the remaining 56 cases were associated with fetal anomalies. Between these two groups, no significant difference was found in the gestational weeks when polyhydramnios developed. However, significant difference was noted in the maximum amniotic fluid index (AFI) values during the pregnancy period; 25.4 +/- 2.7 cm in the former, and 30.6 +/- 8.9 cm in the latter (P = 0.0004). In all of 13 cases with idiopathic polyhydramnios, AFI values remained less than 30 cm until delivery. Twenty-two patients (39%) with fetal anomalies required a prenatal treatment such as amnioreduction and tocolysis, whereas only one patient (7.7%) with idiopathic polyhydramnios needed tocolysis therapy (P = 0.03). There was a significant risk of premature delivery with fetal anomalies (35.6 +/- 3.9 weeks' gestation vs. 38.8 +/- 1.5 weeks' gestation, P = 0.004) because of refractory polyhydramnios, rupture of membranes, non-reassuring fetal status, and intrauterine fetal death, and although most infants with idiopathic polyhydramnios were appropriate-for-dates, many of the infants with congenital anomalies were small-for-dates. Significant risk of fetal anomalies should be considered in pregnant women with severe polyhydramnios (AFI > or = 30 cm), an increased trend of amniotic fluid during the pregnancy period, polyhydramnios requiring a prenatal treatment, or fetal growth restriction. On the other hand, based on our experience, a fetus without these conditions seems to have a low risk of congenital anomalies even if polyhydramnios is noted.

  4. Clinical features and prognostic factors in solitary plasmacytoma.

    PubMed

    Finsinger, Paola; Grammatico, Sara; Chisini, Marta; Piciocchi, Alfonso; Foà, Robin; Petrucci, Maria T

    2016-02-01

    This study aimed to review the clinical features and outcome of 53 patients with solitary plasmacytoma managed at our Institution between 1976 and 2012. Thirty-five patients had bone solitary plasmacytoma and 18 extramedullary solitary plasmacytoma. Tumour sizes were larger in patients with bone involvement (P = 0·003). Treatment consisted of local radiotherapy (n = 26), radiotherapy + chemotherapy (n = 15), surgery (n = 4) and chemotherapy (n = 8); the local control rate was 94·3%. Progression to multiple myeloma was recorded in 20/35 (57·1%) patients with bone involvement and in 1/18 (5·5%) patients with extramedullary disease (P = 0·0003). The 5-year overall survival (OS) rate was 78·4%; bone solitary plasmacytoma patients had a significantly worse OS (71·9% vs. 88·2%, respectively; P = 0·029) and 5-year progression-free survival (PFS; 53·0% vs. 88·5%; P = 0·0003) compared to extramedullary solitary plasmacytoma patients. On univariate analysis, bone disease and size (≥5 cm) impacted negatively on PFS (P = 0·0027 and P = 0·04, respectively). Bone disease also affected OS (P = 0·04). In multivariate analysis bone location was the only independent prognostic factor for PFS (P = 0·0041) and OS (P = 0·021). Patients with bone solitary plasmacytoma have a significantly worse prognosis than extramedullary solitary plasmacytoma cases. © 2015 John Wiley & Sons Ltd.

  5. Characteristic clinical features associated with aggressive posterior retinopathy of prematurity.

    PubMed

    Ahn, Y J; Hong, K E; Yum, H R; Lee, J H; Kim, K S; Youn, Y A; Park, S H

    2017-02-24

    PurposeTo identify the risk factors for, and clinical features and treatment outcomes of aggressive posterior retinopathy of prematurity (APROP) in Korean infants.MethodsAmong 770 premature infants who underwent screening, 105 infants (198 eyes, 13.63%) received treatment for ROP. A total of 24 infants (48 eyes, 3.12%) developed APROP while 81 infants (150 eyes, 10.52%) developed non-APROP treatment-requiring type. The medical records of ROP-treated infants were reviewed retrospectively. The associated systemic and maternal risk factors were analyzed and anatomical outcomes were compared according to the severity of ROP and treatment modalities.ResultsThe mean gestational age and birth weight at birth in the APROP group were significantly lower than those in the non-APROP group (P=0.019, P<0.001, respectively). Infants who were born small for their GA developed APROP more frequently than non-APROP patients (P<0.001). Chorioamnionitis-positive infants also showed higher incidence rate of APROP (APROP vs non-APROP; P<0.001 and zone I APROP vs posterior zone II APROP; P=0.036, respectively). Infants with APROP required heavier laser treatment with a higher retreatment rate compared to infants with non-APROP. Favorable anatomical outcomes were achieved in 95.3% from treatment-requiring non-APROP group, 85.7% from zone I APROP and 84.6% from posterior zone II APROP group.ConclusionIntrauterine growth restriction and chorioamnionitis were associated with development of APROP. These findings suggest that perinatal maternal environment inhibiting normal retinal vascular growth in utero may contribute to increasing the risk of APROP in premature infants.Eye advance online publication, 24 February 2017; doi:10.1038/eye.2017.18.

  6. Fecal incontinence in men: Causes and clinical and manometric features

    PubMed Central

    Muñoz-Yagüe, Teresa; Solís-Muñoz, Pablo; Ciriza de los Ríos, Constanza; Muñoz-Garrido, Francisco; Vara, Jesús; Solís-Herruzo, José Antonio

    2014-01-01

    AIM: To determine the causes and characteristics of fecal incontinence in men and to compare these features with those presented by a group of women with the same problem. METHODS: We analyzed the medical history, clinical and manometric data from 119 men with fecal incontinence studied in our unit and compared these data with those obtained from 645 women studied for the same problem. Response to treatment was evaluated after 6 mo of follow-up. RESULTS: Fifteen percent of patients studied in our unit for fecal incontinence were male. Men took longer than women before asking for medical help. Ano-rectal surgery was the most common risk factor for men related to fecal incontinence. Chronic diarrhea was present in more than 40% of patients in both groups. Decreased resting and external anal sphincter pressures were more frequent in women. No significant differences existed between the sexes regarding rectal sensitivity and recto-anal inhibitory reflex. In 17.8% of men, all presenting soiling, manometric findings did not justify fecal incontinence. Response to treatment was good in both groups, as 80.4% of patients improved and fecal incontinence disappeared in 13.2% of them. CONCLUSION: In our series, it was common that men waited longer in seeking medical help for fecal incontinence. Ano-rectal surgery was the major cause of this problem. Chronic diarrhea was a predisposing factor in both sexes. Manometric differences between groups were limited to an increased frequency of hypotony of the external anal sphincter in women. Fecal incontinence was controllable in most patients. PMID:24976729

  7. Pseudotumour cerebri in children: Aetiology, clinical features, and progression.

    PubMed

    Mosquera Gorostidi, A; Iridoy Zulet, M; Azcona Ganuza, G; Gembero Esarte, E; Yoldi Petri, M E; Aguilera Albesa, S

    2017-01-09

    The definition, associated aetiologies, diagnosis, and treatment of idiopathic intracranial hypertension, or pseudotumour cerebri (PTC), are constantly being revised in the paediatric population. Our study included children younger than 15 years old with PTC and attended at a reference hospital in the past 12 years. We analysed the clinical and epidemiological features of our sample and the diagnostic and treatment approaches. PTC was defined as presence of intracranial hypertension (CSF opening pressure>25cmH2O) and absence of space-occupying lesions in brain MR images. A total of 12 children with PTC were included; mean age was 10 years and 90% were girls. Weight was normal in all patients. Eighty-two percent of the patients had symptoms: headache (66%), diplopia (8%), and visual loss (8%). All of them displayed papilloedema (17% unilaterally). Lumbar puncture (LP) provided the diagnosis in all cases and 91% showed no relevant MRI findings. A potential cause of PTC was identified in 5 cases: pharmacological treatment in 2 and infection (Mycoplasma pneumoniae [M. pneumoniae]) in 3. Ninety-one per cent of the patients received treatment: 75% underwent several LPs and 42% received acetazolamide and/or prednisone. Outcomes were favourable in all cases. The incidence of PTC was estimated at approximately 1 case per 100 000 children/years, in line with data reported by previous studies. Overweight was not found to be a risk factor for PTC in this population. M. pneumoniae infection may trigger PTC and cause recurrences at later stages. The absence of symptoms seems to be independent from the degree of intracranial hypertension. Acetazolamide treatment is effective in most cases, and it represents a viable alternative to repeated LP. Copyright © 2016 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  8. Antimicrobial activity of mupirocin, daptomycin, linezolid, quinupristin/dalfopristin and tigecycline against vancomycin-resistant enterococci (VRE) from clinical isolates in Korea (1998 and 2005).

    PubMed

    Lee, Do Kyung; Kim, Yuna; Park, Kun Sup; Yang, Jae Wook; Kim, Kyungjae; Ha, Nam Joo

    2007-11-30

    It is a hot clinical issue whether newly approved antimicrobial agents such as daptomycin, linezolid, quinupristin/dalfopristin (synercid) and tigecycline are active enough to be used for infections caused by vancomycin resistant bacteria. We performed susceptibility tests for mupirocin, which is in widespread clinical use in Korea, and four new antimicrobials, daptomycin, linezolid, quinupristin/dalfopristin and tigecycline, against vancomycin-resistant Enterococcus faecalis and Enterococcus faecium isolated from Korean patients in 1998 and 2005 to evaluate and compare the in vitro activity of these antimicrobials. Among these agents, quinupristin/dalfopristin, which is rarely used in hospitals in Korea, showed relatively high resistance to several vancomycin-resistant enterococci (VRE) isolated in 2005. Likewise, daptomycin, linezolid and tigecycline have not yet been in clinical use in Korea. However, our results showed that most of the 2005 VRE isolates were already resistant to linezolid and daptomycin (highest minimum inhibitory concentration (MIC) value >100 microg/ml). Compared with the other four antimicrobial agents tested in this study, tigecycline generally showed the greatest activity against VRE. However, four strains of 2005 isolates exhibited resistance against tigecycline (MIC >12.5 microg/ml). Almost all VRE were resistant to mupirocin, whereas all E. faecium isolated in 1998 were inhibited at concentrations between 0.8 to approximately 1.6 microg/ml. In conclusion, resistances to these new antimicrobial agents were exhibited in most of VRE strains even though these new antibiotics have been rarely used in Korean hospitals.

  9. Clinical Characteristics and Precipitating Factors of Adolescent Suicide Attempters Admitted for Psychiatric Inpatient Care in South Korea

    PubMed Central

    Park, Subin; Kim, Jae-Won; Kim, Bung-Nyun; Bae, Jeong-Hoon; Shin, Min-Sup; Yoo, Hee-Jeong

    2015-01-01

    Objective We aimed to examine the rates, correlates, methods, and precipitating factors of suicide attempts among adolescent patients admitted for psychiatric inpatient care from 1999 to 2010 in a university hospital in Korea. Methods The subjects consisted of 728 patients who were admitted for psychiatric inpatient care in a university hospital over a 12-year period and who were aged 10-19 years at the time of admission. We retrospectively investigated the information on suicidal behaviors and other clinical information by reviewing the subjects' electronic medical records. Whether these patients had completed their suicide on 31 December 2010 was determined by a link to the database of the National Statistical Office. Results Among 728 subjects, 21.7% had suicidal ideation at admission, and 10.7% admitted for suicidal attempts. Female gender, divorced/widowed parents, and the presence of mood disorders were associated with a significantly increased likelihood of suicide attempts. Most common method of suicide attempts was cutting, and most common reason for suicide attempts was relationship problems within the primary support group. A diagnosis of schizophrenia was associated with increased risk of death by suicide after discharge. Conclusion These results highlight the role of specific psychosocial factor (e.g., relational problems) and psychiatric disorders (e.g., mood disorders) in the suicide attempts of Korean adolescents, and the need for effective prevention strategies for adolescents at risk for suicide. PMID:25670943

  10. Clinical Guidelines for the Antimicrobial Treatment of Bone and Joint Infections in Korea

    PubMed Central

    2014-01-01

    There are many various diseases in the bone and joint infections, and we tried to make antimicrobial treatment guidelines for common infectious diseases based on available data for microbiology and clinical trials. This guidelines focused on the treatment of osteomyelitis and septic arthritis, which can be experienced by physicians at diverse clinical settings. This guidelines is not applicable to diabetic foot infections, postoperative infections or post-traumatic infections which need special considerations. The guidelines for those conditions will be separately developed later. Surgical treatment of bone and joint infections, pediatric bone and joint infection, tuberculous bone and joint infection, and prophylactic antibiotic use were not included in this guideline. PMID:25024877

  11. Clinical features of a paediatric asthma population in Trinidad.

    PubMed

    Matthew, Jason; Bekele, Isaac; Pinto Pereira, Lexley Maureen

    2013-04-01

    Clinical features and management of paediatric asthma, highly prevalent in the Caribbean, was explored in Trinidadian children. Children (2-16 years) with acute [visiting Accident and Emergency (A&E) in the past 12 months (50.2%, 120)] or stable asthma [controlled during the previous 3 months (49.8%, 119)] were studied. There were more (P < 0.001) boys (71.5%) than girls. Asthma was associated with asthmatic mothers (48.5%), fathers (19.2%) and both parents (15.1%) (P < 0.001). Children's average age of first wheeze was 2.5 (standard deviation = 1.3) years; 30.1% were nebulized before 1 year. In the past 12 months, frequencies of A&E visits were once (20.6%), >1 (61.9%) and >3 times (26.4%) (P < 0.001). Sufferers of exacerbations showed negative logistic regression for age (-0.129, standard error = 0.039, P < 0.001) independent of gender. Acute asthmatics who suffered cough, fever and sore throat in the preceding week were respectively 15.2 [odds ratio (OR) = 15.2, 95% confidence interval (CI) = 6.8-34.0], 13.7 (OR = 13.7, 95% CI = 6.7-28.2) and 3.4 (OR = 3.4, 95% CI = 1.7-6.7) times more likely to suffer wheeze exacerbations than stable children. Most stable children (76.5%) inhaled corticosteroids with salbutamol reliever compared with 22.5% of acute asthmatics, whereas 40.8% of the latter group used salbutamol alone compared with 19.3% of stable children (P < 0.001). Childhood wheeze occurs before 3 years and is associated with maternal asthma. Cough, fever and sore throat in the previous week are strongly associated with exacerbations. Defining these associations could enhance preventive approaches to combat childhood asthma. © 2012 Blackwell Publishing Ltd.

  12. Psychopathy: clinical features, developmental basis and therapeutic challenges.

    PubMed

    Thompson, D F; Ramos, C L; Willett, J K

    2014-10-01

    Psychopathy is a personality disorder characterized by deficits in personality and behaviour. Personality deficits are marked by interpersonal and affective facets, including pathological lying, grandiose sense of self-worth, lack of remorse and callousness. Behavioural deficits are defined by lifestyle and antisocial deficits, including impulsivity, parasitic lifestyle and poor behavioural controls. The objective of this review is to provide clinicians with (i) an appreciation of the clinical features of psychopathy, (ii) an understanding of the structural and functional derangements and the genetic and environmental factors which serve as the basis for the development of psychopathy and (iii) a summary of published reports of pharmacological approaches to the management of this disorder. A literature search of MEDLINE/PubMed (1966-present) was conducted using the MeSH search terms psychopathy and antisocial personality disorder alone and in combination with the subheading drug therapy. Additional databases included Web of Science (1945-present) and International Pharmaceutical Abstracts (1970-present) using the text words psychopath and antisocial personality were searched. A search of Amazon books using the search terms psychopathy and sociopathy was also performed. Bibliographies of relevant articles were searched for additional citations. All data sources in English were considered for inclusion. For background information, broad subject headings were searched for review articles first. Human and animal drug therapy articles were evaluated giving preference to those papers using a controlled trial methodology. Psychopathy is a personality disorder characterized by a lack of conscience, pathologic lying, manipulative behaviour and often superficial charm. The incidence of psychopathy in the general population is generally considered to be 0·6-4% with a higher proportion of males to females. Brain imaging studies of psychopaths suggest a smaller and less active

  13. Characterization of mutations in multi- and extensive drug resistance among strains of Mycobacterium tuberculosis clinical isolates in Republic of Korea.

    PubMed

    Jnawali, Hum Nath; Hwang, Sung Chul; Park, Young Kil; Kim, Hyejin; Lee, Yeong Seon; Chung, Gyung Tae; Choe, Kang Hyeon; Ryoo, Sungweon

    2013-06-01

    In order to characterize molecular mechanisms of first- and second-line drug resistance in Mycobacterium tuberculosis and to evaluate the use of molecular markers of resistance, we analyzed 62 multidrug-resistant, 100 extensively drug-resistant, and 30 pan-susceptible isolates from Korean tuberculosis patients. Twelve genome regions associated with drug resistance, including katG, ahpC, and inhA promoter for isoniazid (INH); embB for ethambutol (EMB), rpoB for rifampin (RIF), pncA for pyrazinamide (PZA), gyrA for fluoroquinolones; rpsL, gidB, and rrs for streptomycin; rrs and eis for kanamycin (KM); rrs and tylA for capreomycin (CAP); and rrs for amikacin (AMK) were amplified simultaneously by polymerase chain reaction, and the DNA sequences were determined. We found mutations in 140 of 160 INH-resistant isolates (87.5%), 159 of 162 RIF-resistant isolates (98.15%), 127 of 143 EMB-resistant isolates (88.8%), 108 of 123 ofloxacin-resistant isolates (87.8%), and 107 of 122 PZA-resistant isolates (87.7%); 43 of 51 STM-resistant isolates (84.3%), 15 of 17 KM-resistant isolates (88.2%), and 14 of 15 (AMK and CAP)-resistant isolates (93.3%) had mutations related to specific drug resistance. In addition, the sequence analyses of the study revealed many novel mutations involving these loci. This result suggests that mutations in the rpoB531, katGSer315Thr, and C-15T in the inhA promoter region, and gyrA94, embB306, pncA159, rpsL43, and A1401G in the rrs gene could serve as useful markers for rapid detection of resistance profile in the clinical isolates of M. tuberculosis in Korea, with potentials for the new therapeutic benefits in actual clinical practice.

  14. Phenotypic, immunologic, and clinical characteristics of patients with nontuberculous mycobacterial lung disease in Korea

    PubMed Central

    2013-01-01

    Background This study aimed to elucidate the phenotypic, immunologic, and clinical characteristics of Korean patients with nontuberculous mycobacterial (NTM) lung disease and compare them with non-NTM bronchiectasis (BE) patients. Methods We prospectively recruited patients between 20 and 80 years of age who had nodular BE type NTM lung disease. Phenotypic, immunologic, and clinical characteristics were evaluated through physical examination, laboratory tests, pulmonary function tests, and radiographic examinations. Questionnaires were also answered. The results of the evaluations were compared with the results of non-NTM BE patients. Results A total of 84 patients with NTM lung disease and 47 non-NTM BE patients participated in the study. Mycobacterium avium complex lung disease and M. abscessus lung disease were most common. Patients with NTM lung disease had lower body mass index than non-NTM BE patients. Scoliosis was observed more frequently in patients with NTM lung disease than in non-NTM BE patients. Conclusions Significant similarities were seen between Korean patients with NTM lung disease and patients from other countries. Differences in phenotypic and clinical characteristics between NTM lung disease and non-NTM BE patients suggest differences in the immunopathogenesis of NTM lung disease and non-NTM BE. Trial registration information ClinicalTrials.gov Registration number; NCT01616745 PMID:24274658

  15. Coronary artery spasm--clinical features, diagnosis, pathogenesis, and treatment.

    PubMed

    Yasue, Hirofumi; Nakagawa, Hitoshi; Itoh, Teruhiko; Harada, Eisaku; Mizuno, Yuji

    2008-02-01

    Coronary (artery) spasm plays an important role in the pathogenesis of ischemic heart disease, including stable angina, unstable angina, myocardial infarction, and sudden death. The prevalence of coronary spasm differs among populations, is higher in Japan and Korea than in the Western countries probably due to genetic as well as environmental factors. Coronary spasm occurs most often from midnight to early morning and is usually not induced by exercise in the daytime. The attacks of coronary spasm are associated with either ST segment elevation or depression, or negative U wave on ECG. Patients with multi-vessel coronary spasm may suffer from lethal arrhythmia, including advanced AV block, ventricular tachycardia or fibrillation, or even sudden death, and they are often resistant to conventional medical therapy including Ca-channel blockers (CCBs). Endothelial nitric oxide (NO) activity is reduced and markers of oxidative stress are elevated in patients with coronary spasm. Thrombogenesis is enhanced and plasma levels of hsCRP and P-selection are elevated in patients with coronary spasm. Thus, patients with coronary spasm have endothelial dysfunction and are suffering from a low-grade chronic inflammation. Polymorphisms of endothelial NO synthase, smoking, and low-grade inflammation are the most important risk factors for coronary spasm. Coronary spasm is a hyper-contraction of coronary smooth muscle triggered by an increase of intracellular Ca2+ in the presence of an increased Ca2+ sensitivity. It has been shown that RhoA/ROCK pathway is involved in Ca2+ sensitivity and that the reduced endothelial NO activity results in increased Ca2+ sensitivity through enhanced RhoA/ROCK pathway. Accordingly, it is possible that in addition to CCBs, RhoA/ROCK pathway blockers may prove to be useful for the treatment of coronary spasm.

  16. Clinical Guidelines for the Diagnosis and Treatment of Cushing's Disease in Korea

    PubMed Central

    Hur, Kyu Yeon; Kim, Jung Hee; Kim, Byung Joon; Kim, Min-Seon; Lee, Eun Jig

    2015-01-01

    Cushing's disease (CD) is a rare disorder characterized by the overproduction of adrenocorticotropic hormone due to a pituitary adenoma that ultimately stimulates excessive cortisol secretion from the adrenal glands. Prior to the detection of pituitary adenomas, various clinical signs of CD such as central obesity, moon face, hirsutism, and facial plethora are usually already present. Uncontrolled hypercortisolism is associated with metabolic, cardiovascular, and psychological disorders that result in increased mortality. Hence, the early detection and treatment of CD are not only important but mandatory. Because its clinical manifestations vary from patient to patient and are common in other obesity-related conditions, the precise diagnosis of CD can be problematic. Thus, the present set of guidelines was compiled by Korean experts in this field to assist clinicians with the screening, diagnoses, and treatment of patients with CD using currently available tests and treatment modalities. PMID:25827452

  17. Travel Pattern and Prescription Analysis at a Single Travel Clinic Specialized for Yellow Fever Vaccination in South Korea.

    PubMed

    Chin, Bum Sik; Kim, Jae Yoon; Gianella, Sara; Lee, Myunghee

    2016-03-01

    Travel-related risks for infectious diseases vary depending on travel patterns such as purpose, destination, and duration. In this study, we describe the patterns of travel and prescription of vaccines as well as malaria prophylaxis medication (MPM) at a travel clinic in South Korea to identify the gaps to fill for the optimization of pre-travel consultation. A cohort of travel clinic visitors in 2011 was constructed and early one-third of the visitors of each month were reviewed. During the study period, 10,009 visited the travel clinic and a retrospective chart review was performed for 3,332 cases for analysis of travel patterns and prescriptions. People receiving yellow fever vaccine (YFV) (n = 2,933) were traveling more frequently for business and tourism and less frequently for providing non-medical service or research/education compared to the 399 people who did not receive the YFV. Overall, most people were traveling to Eastern Africa, South America, and Western Africa, while South-Eastern Asia was the most common destination for the non-YFV group. Besides YFV, the typhoid vaccine was the most commonly prescribed (54.2%), while hepatitis A presented the highest coverage (74.7%) considering the natural immunity, prior and current vaccination history. Additionally, 402 (82.5%) individuals received a prescription for MPM among the 487 individuals travelling to areas with high-risk of malaria infection. Age over 55 was independently associated with receiving MPM prescription, while purpose of providing service and travel duration over 10 days were associated with no MPM prescription, despite travelling to high-risk areas. Eastern Africa and South America were common travel destinations among the visitors to a travel clinic for YFV, and most of them were travelling for tourism and business. For the individuals who are traveling to areas with high-risk for malaria, more proactive approach might be required in case of younger age travelers, longer duration, and

  18. Travel Pattern and Prescription Analysis at a Single Travel Clinic Specialized for Yellow Fever Vaccination in South Korea

    PubMed Central

    Gianella, Sara

    2016-01-01

    Background Travel-related risks for infectious diseases vary depending on travel patterns such as purpose, destination, and duration. In this study, we describe the patterns of travel and prescription of vaccines as well as malaria prophylaxis medication (MPM) at a travel clinic in South Korea to identify the gaps to fill for the optimization of pre-travel consultation. Materials and Methods A cohort of travel clinic visitors in 2011 was constructed and early one-third of the visitors of each month were reviewed. During the study period, 10,009 visited the travel clinic and a retrospective chart review was performed for 3,332 cases for analysis of travel patterns and prescriptions. Results People receiving yellow fever vaccine (YFV) (n = 2,933) were traveling more frequently for business and tourism and less frequently for providing non-medical service or research/education compared to the 399 people who did not receive the YFV. Overall, most people were traveling to Eastern Africa, South America, and Western Africa, while South-Eastern Asia was the most common destination for the non-YFV group. Besides YFV, the typhoid vaccine was the most commonly prescribed (54.2%), while hepatitis A presented the highest coverage (74.7%) considering the natural immunity, prior and current vaccination history. Additionally, 402 (82.5%) individuals received a prescription for MPM among the 487 individuals travelling to areas with high-risk of malaria infection. Age over 55 was independently associated with receiving MPM prescription, while purpose of providing service and travel duration over 10 days were associated with no MPM prescription, despite travelling to high-risk areas. Conclusion Eastern Africa and South America were common travel destinations among the visitors to a travel clinic for YFV, and most of them were travelling for tourism and business. For the individuals who are traveling to areas with high-risk for malaria, more proactive approach might be required in

  19. Clinical and Therapeutic Implications of Aeromonas Bacteremia: 14 Years Nation-Wide Experiences in Korea

    PubMed Central

    Jung, Dong Sik

    2016-01-01

    Background To elucidate the clinical presentation, antimicrobial susceptibility, and prognostic factors of monomicrobial Aeromonas bacteremia in order to determine the most effective optimal therapy. Materials and Methods We reviewed the medical records of Aeromonas bacteremia patients for the period January 2000 to December 2013 in a retrospective multi-center study. Results A total of 336 patient records were reviewed, with 242 having community-acquired bacteremia. The major clinical infections were of the hepatobiliary tract (50.6%) and peritonitis (18.5%), followed by primary bacteremia (17.9%). The infections usually occurred in patients with malignancy (42.3%), hepatic cirrhosis (39.3%), or diabetes mellitus (25.6%). High antimicrobial-resistance rates (15.5% for ceftriaxone, 15.5% for piperacillin/tazobactam) were noted. However, resistance to carbapenem and amikacin was only 9.8% and 3.0%, respectively. Aeromonas hydrophila (58.9%) was the most common pathogen, followed by Aeromonas caviae (30.4%). The severity of A. caviae bacteremia cases were less than that of A. hydrophila or Aeromonas veronii bacteremia (P <0.05). A. hydrophila showed higher antimicrobial resistance than did other Aeromonas species (P <0.05). Patients with hospital-acquired bacteremia were more likely to have severely abnormal laboratory findings and relatively high antimicrobial-resistance rates. Mortality was associated with metastatic cancer, shock, delayed use of appropriate antimicrobial agents, increased prothrombin time, and increased creatinine level (P <0.05). Conclusions Aeromonas species should be considered one of the causative agents of bacteremia in patients with intra-abdominal infections or malignancies. Although ceftriaxone-resistant Aeromonas bacteremia was not statistically related to mortality in this study, it was associated with severe clinical manifestations and laboratory abnormalities. Appropriate antibiotics, including carbapenem, should be administered early

  20. Similar clinical characteristics of familial and sporadic inflammatory bowel disease in South Korea.

    PubMed

    Chung, Sook Hee; Park, Soo Jung; Lee, Hye Sun; Hong, Sung Pil; Cheon, Jae Hee; Kim, Tae Il; Kim, Won Ho

    2014-12-07

    To investigate differences of clinical characteristics and disease courses between familial and sporadic inflammatory bowel disease (IBD) patients. We obtained clinical data on Crohn's disease (CD) (n = 691) and ulcerative colitis (n = 1113) from a tertiary referral medical center between 2005 and 2012. Seventeen patients (2.5%) with CD and 27 patients (2.4%) with ulcerative colitis (UC) were identified as having a familial history of IBD, including the first and second degree relatives. For each control case, three times the number of age-, sex-, and diagnosis year-matched CD and UC patients, without a family history of IBD, were randomly selected in this case control study. There were no significant differences in age or main symptom at diagnosis, extraintestinal manifestation, location/extent, behavior of disease activity, number of hospitalizations, number of operations, operation type, number of relapses, or oral medical treatment between familial and sporadic CD and UC patients. Median (min-max) follow-up periods after diagnosis of familial CD and sporadic CD patients were 84 (24-312) and 36 (8-240) mo, respectively (P = 0.008). Familial CD patients more frequently used anti-tumor necrosis factor (TNF) antibodies compared to sporadic CD patients (17.6% vs 0%, P = 0.014). In conclusion, a family history of IBD does not seem to be an important predictive factor affecting clinical characteristics or disease course even if there is a more frequent use of anti-TNF antibodies in familial CD patients compared to sporadic CD patients.

  1. Parasitic Infections Based on 320 Clinical Samples Submitted to Hanyang University, Korea (2004-2011)

    PubMed Central

    Choi, Sung-Chul; Lee, Soo-Young; Song, Hyun-Ouk; Ryu, Jae-Sook

    2014-01-01

    We analyzed 320 clinical samples of parasitic infections submitted to the Department of Environmental Biology and Medical Parasitology, Hanyang University from January 2004 to June 2011. They consisted of 211 nematode infections, 64 trematode or cestode infections, 32 protozoan infections, and 13 infections with arthropods. The nematode infections included 67 cases of trichuriasis, 62 of anisakiasis (Anisakis sp. and Pseudoterranova decipiens), 40 of enterobiasis, and 24 of ascariasis, as well as other infections including strongyloidiasis, thelaziasis, loiasis, and hookworm infecions. Among the cestode or trematode infections, we observed 27 cases of diphyllobothriasis, 14 of sparganosis, 9 of clonorchiasis, and 5 of paragonimiasis together with a few cases of taeniasis saginata, cysticercosis cellulosae, hymenolepiasis, and echinostomiasis. The protozoan infections included 14 cases of malaria, 4 of cryptosporidiosis, and 3 of trichomoniasis, in addition to infections with Entamoeba histolytica, Entamoeba dispar, Entamoeba coli, Endolimax nana, Giardia lamblia, and Toxoplasma gondii. Among the arthropods, we detected 6 cases of Ixodes sp., 5 of Phthirus pubis, 1 of Sarcoptes scabiei, and 1 of fly larva. The results revealed that trichuriasis, anisakiasis, enterobiasis, and diphyllobothriasis were the most frequently found parasitosis among the clinical samples. PMID:24850969

  2. Clinical characteristics of children with mental retardation of unknown etiology in Korea.

    PubMed Central

    Yim, S. Y.; Lee, I. Y.

    1999-01-01

    The purpose of this study was to investigate the clinical characteristics of children with mental retardation (MR) of unknown etiology for early recognition and intervention. In this study, we defined children with MR of unknown etiology as those without clear etiologies for MR despite extensive evaluation and were not associated with pathological behavioral problems such as pervasive developmental disorders and attention-deficit/hyperactivity disorder. The clinical characteristics of children with MR of unknown etiology were as follows. 1) MR of unknown etiology was 48.8% of all MR. 2) MR of unknown etiology was more common in males. 3) Delayed language development was a leading factor that made the parents of children with MR of unknown etiology seek help from physicians. However, most of the children with MR of unknown etiology showed a relatively uniform delay in several areas of development. 4) Most children with MR of unknown etiology were delayed walkers. 5) Most children with MR of unknown etiology were mild cases. PMID:10331556

  3. Ten-year incidence and prevalence of clinically diagnosed blepharitis in South Korea: a nationwide population-based cohort study.

    PubMed

    Rim, Tyler Hyungtaek; Kang, Min Jae; Choi, Moonjung; Seo, Kyoung Yul; Kim, Sung Soo

    2017-07-01

    Blepharitis is one of the most common conditions. However, no study has yet evaluated the epidemiology by evaluating a large population-based sample. To evaluate the incidence and prevalence of clinically diagnosed blepharitis in South Korea. Nationwide population-based study. We investigated the Korean National Health Insurance Service-National Sample Cohort, a representative one million-sample of the Korean population, for patients diagnosed with blepharitis according to the Korean Classification of Diseases. Annual and overall incidence and prevalence of blepharitis during the study period (2004-2013) were estimated after excluding chronic blepharitis patients, diagnosed during 2002-2003. Sociodemographic factors and comorbidities associated with blepharitis were evaluated using Cox proportional hazard regression. The first occurrence of blepharitis. A total of 1 116 363 individuals over 9 698 118 person-years were evaluated (mean follow up: 8.7 years) from 2004 to 2013. The overall incidence was 1.1 (95% confidence interval, 1.1-1.1) per 100 person-years. The incidence increased with time (0.9 vs. 1.3 per 100 person-years, in 2004 and 2013, respectively) and was higher in female patients (1.3 vs. 0.9 per 100 person-years, respectively). The overall prevalence was 8.1% (95% confidence interval: 8.0-8.1) among subjects aged 40 years or older. Chalazion, gastritis, Sjögren's syndrome, pterygium, rosacea, prostatic hypertrophy, atopy, irritable bowel disease and peptic ulcer were associated with an increased incidence of blepharitis in the multivariable Cox model. We found that blepharitis was a relatively common disease and is associated with various ocular and systemic conditions. © 2017 Royal Australian and New Zealand College of Ophthalmologists.

  4. Clinical features, histology, and histogenesis of combined hepatocellular-cholangiocarcinoma

    PubMed Central

    Gera, Shweta; Ettel, Mark; Acosta-Gonzalez, Gabriel; Xu, Ruliang

    2017-01-01

    Combined hepatocellular-cholangiocarcinoma (CHC) is a rare tumor with poor prognosis, with incidence ranging from 1.0%-4.7% of all primary hepatic tumors. This entity will be soon renamed as hepato-cholangiocarcinoma. The known risk factors for hepatocellular carcinoma (HCC) have been implicated for CHC including viral hepatitis and cirrhosis. It is difficult to diagnose this tumor pre-operatively. The predominant histologic component within the tumor largely determines the predominant radiographic features making it a difficult distinction. Heterogeneous and overlapping imaging features of HCC and cholangiocarcinoma should raise the suspicion for CHC and multiple core biopsies (from different areas of tumor) are recommended before administering treatment. Serum tumor markers CA19-9 and alpha-fetoprotein can aid in the diagnosis, but it remains a challenging diagnosis prior to resection. There is sufficient data to support bipotent hepatic progenitor cells as the cell of origin for CHC. The current World Health Organization classification categorizes two main types of CHC based on histo-morphological features: Classical type and CHC with stem cell features. Liver transplant is one of the available treatment modalities with other management options including transarterial chemoembolization, radiofrequency ablation, and percutaneous ethanol injection. We present a review paper on CHC highlighting the risk factors, origin, histological classification and therapeutic modalities. PMID:28293379

  5. Current Status and Clinical Studies of Oriental Herbs in Sexual Medicine in Korea

    PubMed Central

    Shin, Yu Seob; Zhao, Chen; Zhang, Li Tao

    2015-01-01

    Erectile dysfunction (ED) is one of the most common diseases among aging men. Although previous studies have shown that type 5 phosphodiesterase inhibitors (PDE5-Is) are very effective for the treatment of ED, many researchers are currently attempting to identify therapeutic agents from natural sources with comparable or better effects than PDE5-Is. Herbal medicine is thought to be advantageous because it is natural; moreover, it not only treats isolated symptoms, but also maintains general well-being. Furthermore, since newly created chemical compound libraries have limited structural diversity with regard to pharmaceutical agents, more attention has recently been paid to the ability of oriental herbs to enhance physical health, including sexual function. Herein, we review the current status of Korean preclinical or clinical studies of the application of oriental herbs to sexual medicine. PMID:26331122

  6. A Case of Primary Biliary Cirrhosis Mimicking Acute Hepatitis B in the Clinic, Republic of Korea

    PubMed Central

    Kwon, Woo Hyuk; Park, Hong Min; Park, Jeong Jun; Lee, Sung Hoon

    2017-01-01

    Primary biliary cirrhosis (PBC) is a slowly progressive cholestatic autoimmune liver disease characterized by progressive bile duct injury. The most common symptoms of this disease include fatigue and pruritus. The diagnosis of PBC is based on cholestatic biochemical liver tests, presence of antimitochondrial antibodies, and characteristic histological biopsy findings. We report a case of a patient with PBS, who was initially suspected to be in the window period of hepatitis B by a private doctor in a local clinic based on the detection of isolated immunoglobulin M antibody against hepatitis B core antigen. The presence of this antibody is the most useful index in diagnosing acute hepatitis B (+) by immunoserological test. The final diagnosis of the patient in Good Gang-An Hospital was PBC through additional tests. The patient is receiving outpatient treatment. PMID:28197333

  7. Application of Single-nucleotide Polymorphism and Mycobacterial Interspersed Repetitive Units-Variable Number of Tandem Repeats Analyses to Clinical Mycobacterium tuberculosis Isolates from Korea

    PubMed Central

    Choi, Go Eun; Jang, Mi Hee; Cho, Hyun-Jung; Lee, Sun Min; Yi, Jongyoun; Lee, Eun Yup; Chang, Chulhun L.; Kim, Yeong Dae

    2011-01-01

    Background Single-nucleotide polymorphism (SNP) analysis is a powerful strategy for large-scale molecular population studies examining phylogenetic relationships among bacterial strains. Mycobacterial interspersed repetitive units-variable number of tandem repeats (MIRU-VNTR) can be easily digitized to share data among laboratories. This study applied SNP and MIRU-VNTR analyses for molecular strain typing of Mycobacterium tuberculosis isolates collected throughout Korea. Methods We studied 102 clinical M. tuberculosis isolates, including 6 paired strains, collected from 11 university hospitals in Korea in 2008 and 2009. SNPs were detected using hairpin primer assays, and then, MIRU-VNTR analysis was performed. Results Thirty-five SNPs contained polymorphisms that helped differentiate the 96 tested isolates. The isolates were classified into 15 clusters. The Beijing family strains were distributed within closely related clusters in the SNP dendrogram. For MIRU-VNTR analysis, the 96 isolates were divided into 12 groups. The discriminatory index in 8 of these groups (MIRU-10, -23, -26, and -31; ETR-A, -B, -C, and -F) was high (Hunter-Gaston diversity index > 0.6). Unlike the SNP method, MIRU-VNTR analysis did not identify any notable localizations of Beijing or non-Beijing family isolates in specific clusters. Conclusions SNP and MIRU-VNTR analyses are surrogate molecular strain-typing methods for M. tuberculosis in Korea where Beijing family isolates are predominant. PMID:21239869

  8. Clinical characteristics of nonradiographic axial spondyloarthritis in Korea: a comparison with ankylosing spondylitis.

    PubMed

    Jeong, Hyemin; Yoon, Ji Y; Park, Eun-Jung; Hwang, Jiwon; Kim, Hyungjin; Ahn, Joong K; Lee, Jaejoon; Koh, Eun-Mi; Cha, Hoon-Suk

    2015-07-01

    To evaluate the clinical characteristics and outcomes of nonradiographic axial spondyloarthritis (nr-axSpA) in Korean patients. A retrospective analysis evaluated 155 patients with nr-axSpA at a single tertiary hospital between January 2001 and January 2011. Baseline characteristics and clinical courses were reviewed and compared with those of patients with ankylosing spondylitis (AS). The mean age at disease onset was 29.5 ± 10.8 years and 52 (33.5%) patients were female. The mean age at symptom onset was older (29.5 ± 10.8 vs 25.9 ± 9.2, respectively, P < 0.001) and the male-to-female ratio was lower (2 : 1 vs 5 : 1, respectively, P = 0.001) in patients with nr-axSpA compared with patients with AS. The proportion of females was higher among patients with late onset SpA than early-onset nr-axSpA (55.0% vs 30.1%, respectively, P = 0.029). Among 74 patients with nr-axSpA, whose follow-up duration was more than 1.5 years, 29 (39.2%) patients progressed to AS during the follow-up period. The proportion of females was lower in progressors that that of non-progressors (13.8% vs 44.4%, respectively, P = 0.010). Presence of syndesmophyte and minimal X-ray changes at baseline were frequently observed in progressors compared with non-progressors (26.7% vs 0.0%, P = 0.006 and 69.0 vs 35.6%, P = 0.005, respectively). The predominance of male patients is more prominent among Korean patients with SpA compared with Caucasians. Female nr-axSpA patients had late symptom onset and less progression to AS. X-ray changes at baseline were associated with radiographic progression. © 2014 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  9. Factors affecting workplace bullying and lateral violence among clinical nurses in Korea: descriptive study.

    PubMed

    Oh, Hyunjin; Uhm, Dong Choon; Yoon, Young Joo

    2016-04-01

    Workplace bullying and lateral violence are serious issues affecting the work life of hospital nurses. The purpose of this study was to identify the selected individual and institutional characteristics for workplace bullying and lateral violence using a conceptual framework. A descriptive survey design was used. A convenience sample of 255 nurses in tertiary hospitals, who had a minimum of 6 months clinical experience, completed the survey. Regression analysis was used to determine factors significantly associated with workplace bullying and lateral violence. The Negative Acts Questionnaire-Revised and the Lateral Violence scale were used to measure workplace bullying and lateral violence. A negative affect, individualism and working in hospital specialty units predicted workplace bullying. Individualism, a negative affect, affiliated hospital and working hours predicted verbal abuse whereas the place of employment was significantly associated with lateral violence. The results of this study identified factors that are associated with bullying and violence but did not fully support the conceptual framework. The individual characteristic negative affect was significantly associated with most types of workplace bullying and lateral violence bully whereas the place of employment was an important factor in lateral violence. Nurse managers need to be aware that both individual and institutional factors may impact levels of workplace bullying and lateral violence in their hospitals and need to prepare specific strategies to address these multiple factors. © 2015 John Wiley & Sons Ltd.

  10. A systematic heuristic approach for feature selection for melanoma discrimination using clinical images

    PubMed Central

    Chang, Ying; Stanley, R. Joe; Moss, Randy H.; Van Stoecker, William

    2011-01-01

    Background Numerous features are derived from the asymmetry, border irregularity, color variegation, and diameter of the skin lesion of dermatology for diagnosing malignant melanoma. Feature selection for the development of automated skin lesion discrimination systems is an important consideration. Methods In this research, a systematic heuristic approach is investigated for feature selection and lesion classification. The approach integrates statistical-, correlation-, histogram-, and expert system-based components. Using statistical and correlation measures, interrelationships among features are determined. Expert system analysis is performed to identify redundant features. The feature selection process is applied to 19 shape and color features for a clinical image data set containing 355 malignant melanomas, 125 basal cell carcinomas, 177 dysplastic nevi, 199 nevocellular nevi, 139 seborrheic keratoses, and 45 vascular lesions. Results Experimental results show reduced lesion classification error rates based on condensing the shape and color feature set from 19 features to 13 features using the feature selection process. Specifically, average test lesion classification error rates for discriminating malignant melanoma from non-melanoma lesions were reduced from 26.6% for 19 features to 23.2% for 13 features over five randomly generated training and test sets. Conclusions The experimental results show that the systematic heuristic approach for feature reduction can be successfully applied to achieve improved lesion discrimination. The feature reduction technique facilitates the elimination of redundant information that may inhibit lesion classification performance. The clinical application of this result is that automated skin lesion classification algorithm development can be fostered with systematic feature selection techniques. PMID:15998327

  11. Clinical outcomes of robotic mitral valve repair: a single-center experience in Korea

    PubMed Central

    Kim, Ho Jin; Kim, Joon Bum; Jung, Sung-Ho

    2017-01-01

    Background Since the inception of robotic mitral valve repair (MV) in 2007 at our institution, it has become an acceptable surgical option with proven efficacy and safety. The objective of this study is to analyze the early and long-term clinical outcomes of patients undergoing robotic MV repair. Methods A total of 310 patients (aged 48.4±13.7 years, 201 males) undergoing robotic MV repair using the da Vinci system (Intuitive Surgical, Inc., Sunnyvale, CA) between August 2007 and December 2015 in our institution were evaluated. The preoperative demographics, operative profiles and postoperative outcomes including follow-up echocardiographic results were analyzed. Results Successful MV repair was achieved in 98.4% (n=305) of patients, with no significant residual mitral regurgitation (MR) postoperatively. There were no early postoperative deaths. Early postoperative complications included: stroke (n=3, 1.0%), new onset dialysis (n=1, 0.3%) and reoperation (n=3, 1.0%). During a median follow-up of 55.7 months (inter-quartile range 30.3 to 81.3 months), six (1.9%) patients died, while four patients underwent late reoperation for mitral regurgitation (n=2) or infective endocarditis (n=2). Major event-free survival at five years was 87.6%. Late echocardiographic profiles (>6 months) were obtained in 295 (95.2%) patients. During follow-up, 32 (10.8%) patients developed significant mitral regurgitation (MR > grade 2), while freedom from significant MR at five years was 86.5%. Conclusions Robotic MV repair is a safe procedure with acceptable postoperative results, including low early postoperative morbidity and mortality and acceptable long-term repair durability. PMID:28203536

  12. Improving clinical practice using clinical decision support systems: a systematic review of trials to identify features critical to success

    PubMed Central

    Kawamoto, Kensaku; Houlihan, Caitlin A; Balas, E Andrew; Lobach, David F

    2005-01-01

    Objective To identify features of clinical decision support systems critical for improving clinical practice. Design Systematic review of randomised controlled trials. Data sources Literature searches via Medline, CINAHL, and the Cochrane Controlled Trials Register up to 2003; and searches of reference lists of included studies and relevant reviews. Study selection Studies had to evaluate the ability of decision support systems to improve clinical practice. Data extraction Studies were assessed for statistically and clinically significant improvement in clinical practice and for the presence of 15 decision support system features whose importance had been repeatedly suggested in the literature. Results Seventy studies were included. Decision support systems significantly improved clinical practice in 68% of trials. Univariate analyses revealed that, for five of the system features, interventions possessing the feature were significantly more likely to improve clinical practice than interventions lacking the feature. Multiple logistic regression analysis identified four features as independent predictors of improved clinical practice: automatic provision of decision support as part of clinician workflow (P < 0.00001), provision of recommendations rather than just assessments (P = 0.0187), provision of decision support at the time and location of decision making (P = 0.0263), and computer based decision support (P = 0.0294). Of 32 systems possessing all four features, 30 (94%) significantly improved clinical practice. Furthermore, direct experimental justification was found for providing periodic performance feedback, sharing recommendations with patients, and requesting documentation of reasons for not following recommendations. Conclusions Several features were closely correlated with decision support systems' ability to improve patient care significantly. Clinicians and other stakeholders should implement clinical decision support systems that incorporate these

  13. Early-Onset Psychoses: Comparison of Clinical Features and Adult Outcome in 3 Diagnostic Groups

    ERIC Educational Resources Information Center

    Ledda, Maria Giuseppina; Fratta, Anna Lisa; Pintor, Manuela; Zuddas, Alessandro; Cianchetti, Carlo

    2009-01-01

    A comparison of clinical features and adult outcome in adolescents with three types of psychotic disorders: schizophrenic (SPh), schizoaffective (SA) and bipolar with psychotic features (BPP). Subjects (n = 41) were finally diagnosed (DSM-IV criteria) with SPh (n = 17), SA (n = 11) or BPP (n = 13). Clinical evaluation took place at onset and at a…

  14. Evaluating the effectiveness of four contextual features in classifying annotated clinical conditions in emergency department reports.

    PubMed

    Chu, David; Dowling, John N; Chapman, Wendy W

    2006-01-01

    Determine how four contextual features (Validity, Certainty, Directionality, and Temporality) contribute to classification of respiratory syndrome-related clinical conditions as acute, chronic, or absent from manual annotations in Emergency Department Reports. Based on the results, we will direct our research towards automatic identification of the contextual features found to be discriminating. A physician annotated all instances of 56 clinical conditions in 120 ED reports and encoded four contextual features for every annotation. We classified clinical conditions using the contextual features and measured agreement to reference standard classifications made by the physician using a weighted kappa (Kw). Kw was 0.518 when not using any of the features and 0.953 when using all of the features. Validity, Directionality, and Temporality all improved accuracy. Negation(Directionality) was the most important feature for improving accuracy. Using Certainty made the classification worse.

  15. Pine nut allergy: clinical features and major allergens characterization

    USDA-ARS?s Scientific Manuscript database

    Pine nuts, the seeds of pine trees, are widely used for human consumption in Europe, America, and Asia. The aims of this study were to evaluate IgE-mediated hypersensitivity to pine nut in a large number of patients with details of clinical reactions, and to characterize major pine nut allergens. Th...

  16. Key Clinical Features to Identify Girls with "CDKL5" Mutations

    ERIC Educational Resources Information Center

    Bahi-Buisson, Nadia; Nectoux, Juliette; Rosas-Vargas, Haydee; Milh, Mathieu; Boddaert, Nathalie; Girard, Benoit; Cances, Claude; Ville, Dorothee; Afenjar, Alexandra; Rio, Marlene; Heron, Delphine; Morel, Marie Ange N'Guyen; Arzimanoglou, Alexis; Philippe, Christophe; Jonveaux, Philippe; Chelly, Jamel; Bienvenu, Thierry

    2008-01-01

    Mutations in the human X-linked cyclin-dependent kinase-like 5 ("CDKL5") gene have been shown to cause infantile spasms as well as Rett syndrome (RTT)-like phenotype. To date, less than 25 different mutations have been reported. So far, there are still little data on the key clinical diagnosis criteria and on the natural history of…

  17. Key Clinical Features to Identify Girls with "CDKL5" Mutations

    ERIC Educational Resources Information Center

    Bahi-Buisson, Nadia; Nectoux, Juliette; Rosas-Vargas, Haydee; Milh, Mathieu; Boddaert, Nathalie; Girard, Benoit; Cances, Claude; Ville, Dorothee; Afenjar, Alexandra; Rio, Marlene; Heron, Delphine; Morel, Marie Ange N'Guyen; Arzimanoglou, Alexis; Philippe, Christophe; Jonveaux, Philippe; Chelly, Jamel; Bienvenu, Thierry

    2008-01-01

    Mutations in the human X-linked cyclin-dependent kinase-like 5 ("CDKL5") gene have been shown to cause infantile spasms as well as Rett syndrome (RTT)-like phenotype. To date, less than 25 different mutations have been reported. So far, there are still little data on the key clinical diagnosis criteria and on the natural history of…

  18. Clinical features of multiple primary carcinomas of the oral cavity

    PubMed Central

    Li, Ya-Dong; Ma, Xin; Han, Yao-Lun; Peng, Li-Wei

    2017-01-01

    The present study aimed to elucidate the clinical characteristics of multiple primary carcinomas of the oral cavity. The clinical records of 1,024 patients who were treated during follow-up for oral cancer at the Department of Stomatology, Henan Provincial People's Hospital, between March 2013 and December 2014 were retrospectively reviewed. The clinical characteristics of 961 patients who developed single primary oral squamous cell carcinoma (SCC) during follow-up and 54 patients who subsequently developed multiple primary carcinomas in the oral cavity were compared. Multiple primary carcinomas exhibited a female predilection, were most prevalent in the gingiva, and tended to show earlier tumor and nodal stages, as compared with single primary carcinomas. The local recurrence rate was higher for multiple primary carcinomas, as compared with single primary carcinomas, and was demonstrated to increase with the number of multiple primary occurrences. The cumulative incidence rates for metachronous second primary carcinomas following the onset of the first carcinoma at 10 years was 8.0%. Recurrence of multiple primary carcinomas did not decrease the survival rates of the patients assessed in the present study. Furthermore, differences were detected in the clinical characteristics between patients with single oral SCC and those with multiple primary oral carcinomas. The results of the present study indicated that early diagnosis and treatment and close long-term follow-up are required for patients with multiple primary oral carcinomas. PMID:28352343

  19. The ritual abuse of children: clinical features and diagnostic reasoning.

    PubMed

    Nurcombe, B; Unützer, J

    1991-03-01

    A case of alleged ritual sexual abuse is presented. Clinical recognition and diagnostic reasoning are discussed. After a brief account of modern satanism, it is concluded that, although the evidence for the occurrence of ritual abuse is sketchy, a high index of suspicion is appropriate.

  20. Acute hepatitis E in Montenegro: epidemiology, clinical and laboratory features.

    PubMed

    Terzic, Dragica; Dupanovic, Brankica; Mugosa, Boban; Draskovic, Nenad; Svirtlih, Neda

    2009-01-01

    To evaluate the incidence, demographic, clinical and laboratory characteristics of patients with acute viral hepatitis E in Montenegro. A total of 400 patients with acute viral hepatitis from January 1st, 2000 to December 31st, 2007 were enrolled in the study. Serological tests for hepatitis A, B, C, D, and E viruses, Epstein-Barr virus, cytomegalovirus, and herpes simplex viruses were performed. Standard laboratory tests for liver function were analyzed. The results are presented as absolute numbers, mean +/- SD, range of values, and percent. A P value < 0.05 was considered significant. Twenty-four (6%) patients had clinically and/or serologically confirmed acute hepatitis E. The mean age of the patients was 25 +/- 6 years; 62.5% were males. The majority of the patients (66%) belonged to the 20 to 40 yrs age group (P < 0.05). Seven patients were asymptomatic. Foremost symptoms were loss of appetite (100%), fatigue (94%) and vomiting (75%). The most frequent clinical sign was mild to moderate liver enlargement (94%). Jaundice had 12/17 symptomatic patients. Elevation of alanine aminotransferase was found in 19 patients including two patients without symptoms. The enzyme, gamma glutamyltranspeptidase was increased in all patients. Acute hepatitis E in Montenegro emerges as an autochthonous infection with a low incidence. Sub-clinical and anicteric infections may occur. Elevation of gamma glutamyltranspeptidase is an important parameter of the biochemical profile of the disease.

  1. Clinical Features and Management of Congenital Fibrinogen Deficiencies.

    PubMed

    Casini, Alessandro; de Moerloose, Philippe; Neerman-Arbez, Marguerite

    2016-06-01

    Congenital fibrinogen disorders are rare diseases affecting either the quantity (afibrinogenemia and hypofibrinogenemia) or the quality (dysfibrinogenemia) or both (hypodysfibrinogenemia) of plasmatic fibrinogen. Afibrinogenemia is often diagnosed at birth following prolonged umbilical cord bleeding and is characterized by spontaneous bleeding in all tissues, while hypofibrinogenemic patients are more often asymptomatic. Spontaneous spleen ruptures, painful bone cysts, cardiovascular events, and intrahepatic inclusions can complicate the clinical course of patients with quantitative fibrinogen disorders. Clinical manifestations of dysfibrinogenemia are very heterogeneous, from absence of symptoms to major bleeding or thrombosis, chronic thromboembolic pulmonary hypertension, and renal amyloidosis. Hypodysfibrinogenemic patients can suffer from both major bleeding and recurrent thrombosis. Pregnancy of women with congenital fibrinogen disorders is a high-risk situation. Owing to the absence of controlled randomized studies, clinical management is mainly based on expert consensus. For the treatment and/or the prevention of bleeding, plasma-derived fibrinogen concentrates are the optimal choice. Treatment of thrombosis may be challenging. More specifically, management strategies should be tailored to each patient, taking the personal and familial history of bleeding and thrombosis, the genotype, and the specific clinical situation into account. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  2. Clinical features and multidisciplinary approaches to dementia care

    PubMed Central

    Grand, Jacob HG; Caspar, Sienna; MacDonald, Stuart WS

    2011-01-01

    Dementia is a clinical syndrome of widespread progressive deterioration of cognitive abilities and normal daily functioning. These cognitive and behavioral impairments pose considerable challenges to individuals with dementia, along with their family members and caregivers. Four primary dementia classifications have been defined according to clinical and research criteria: 1) Alzheimer’s disease; 2) vascular dementias; 3) frontotemporal dementias; and 4) dementia with Lewy bodies/Parkinson’s disease dementia. The cumulative efforts of multidisciplinary healthcare teams have advanced our understanding of dementia beyond basic descriptions, towards a more complete elucidation of risk factors, clinical symptoms, and neuropathological correlates. The characterization of disease subtypes has facilitated targeted management strategies, advanced treatments, and symptomatic care for individuals affected by dementia. This review briefly summarizes the current state of knowledge and directions of dementia research and clinical practice. We provide a description of the risk factors, clinical presentation, and differential diagnosis of dementia. A summary of multidisciplinary team approaches to dementia care is outlined, including management strategies for the treatment of cognitive impairments, functional deficits, and behavioral and psychological symptoms of dementia. The needs of individuals with dementia are extensive, often requiring care beyond traditional bounds of medical practice, including pharmacologic and non-pharmacologic management interventions. Finally, advanced research on the early prodromal phase of dementia is reviewed, with a focus on change-point models, trajectories of cognitive change, and threshold models of pathological burden. Future research goals are outlined, with a call to action for social policy initiatives that promote preventive lifestyle behaviors, and healthcare programs that will support the growing number of individuals affected by

  3. Clinical features and surgical outcomes of primary canaliculitis with concretions.

    PubMed

    Xiang, Shengjin; Lin, Bin; Pan, Qintuo; Zheng, Meiqin; Qin, Xiaoyi; Wang, Youpei; Zhang, Zongduan

    2017-03-01

    The purpose of our study is to analyze the clinical, ultrasonic, microbiologic, and histopathologic characteristics, management, and outcomes in a series of primary canaliculitis with concretions patients who underwent canaliculotomy with curettage.Thirty-six patients were reviewed for age, sex, location and laterality, duration of symptoms, clinical symptoms, ultrasonic signs, result of microbiologic culture and histopathologic examination, treatment, and outcomes. Main outcomes were the clinical, ultrasonic, and microbiological characteristics of the canalicular concretions; the histopathologic profiles; and the treatment effect.Thirty-six patients were identified with concretions in all 37 cases of the patients with canaliculitis. There were 30 (83.3%) female patients with a mean age of 54.2 years. Twenty-eight (77.8%) patients were misdiagnosed or delayed diagnosed, and the mean duration was 17.1 months. The common most clinical presentations were discharge (100%), epiphora (66.7%), erythema (52.8%), and swelling (47.2%), and concretions were found in 31 of 37 patients by typical clinical manifestations and in 5 of 6 patients by ultrasonic. Actinomyces was found in 8 of 13 histopathologic specimens, and microbiological cultures were positive in 13 of 24 patients. All patients underwent canaliculotomy with curettage to completely remove all concretions and contents; 35 of 36 patients' symptoms improved and 1 recurred after treatment at a median of 21.7 months follow-up according to the telephonic questionnaires.Canalicular concretions play an important role in primary canaliculitis. Canaliculotomy with curettage is a standard therapy with canalicular concretions, and the surgical removal of all possible concretions is essential for cure.

  4. Clinical and laboratory features of viral hepatitis A in children.

    PubMed

    Blechová, Zuzana; Trojánek, Milan; Kynčl, Jan; Cástková, Jitka; John, Jerry; Malý, Marek; Herrmannová, Kristýna; Marešová, Vilma

    2013-02-01

    Recent outbreaks of viral hepatitis A in non-endemic European countries and the potential outbreak risk in susceptible populations has led us to evaluate the clinical characteristics of children hospitalised with hepatitis A. Retrospective study included 118 children (68 boys and 50 girls) with the mean age of 8.5 years hospitalised at Hospital Na Bulovce in Prague from June 2008 to June 2009. The clinical course was symptomatic icteric in 57 (48.3 %) children, symptomatic anicteric in 23 (19.5 %), subclinical in 22 (18.6 %) and asymptomatic inapparent in 16 (13.6 %). The relapse of the disease occurred in three patients. There were no cases of fulminant hepatitis. The most frequent symptoms included jaundice (57 cases), abdominal pain/discomfort (38), vomiting (38), dark urine (37), subfebrility (29) and fever (25). Hepatic injury markers were substantially elevated in icteric patients, but moderate elevations were identified in anicteric and subclinical cases as well. Lower white blood cell and lymphocyte counts were independently associated with symptomatic and more severe clinical course. Active immunisation was provided to 22 patients, and as a post-exposure prophylaxis to 19 of them. The clinical course and laboratory parameters in vaccinated children were not significantly different from non-vaccinated children. The clinical course of hepatitis A was largely self-limiting and benign. Asymptomatic infections are frequent, representing risk for disease spread; however, they are associated with elevations of hepatic injury markers. The inclusion of significant proportion of asymptomatic cases that were identified and investigated only because of active epidemiological surveillance in the outbreak focus represents the particular asset of this study.

  5. Clinical features and surgical outcomes of primary canaliculitis with concretions

    PubMed Central

    Xiang, Shengjin; Lin, Bin; Pan, Qintuo; Zheng, Meiqin; Qin, Xiaoyi; Wang, Youpei; Zhang, Zongduan

    2017-01-01

    Abstract The purpose of our study is to analyze the clinical, ultrasonic, microbiologic, and histopathologic characteristics, management, and outcomes in a series of primary canaliculitis with concretions patients who underwent canaliculotomy with curettage. Thirty-six patients were reviewed for age, sex, location and laterality, duration of symptoms, clinical symptoms, ultrasonic signs, result of microbiologic culture and histopathologic examination, treatment, and outcomes. Main outcomes were the clinical, ultrasonic, and microbiological characteristics of the canalicular concretions; the histopathologic profiles; and the treatment effect. Thirty-six patients were identified with concretions in all 37 cases of the patients with canaliculitis. There were 30 (83.3%) female patients with a mean age of 54.2 years. Twenty-eight (77.8%) patients were misdiagnosed or delayed diagnosed, and the mean duration was 17.1 months. The common most clinical presentations were discharge (100%), epiphora (66.7%), erythema (52.8%), and swelling (47.2%), and concretions were found in 31 of 37 patients by typical clinical manifestations and in 5 of 6 patients by ultrasonic. Actinomyces was found in 8 of 13 histopathologic specimens, and microbiological cultures were positive in 13 of 24 patients. All patients underwent canaliculotomy with curettage to completely remove all concretions and contents; 35 of 36 patients’ symptoms improved and 1 recurred after treatment at a median of 21.7 months follow-up according to the telephonic questionnaires. Canalicular concretions play an important role in primary canaliculitis. Canaliculotomy with curettage is a standard therapy with canalicular concretions, and the surgical removal of all possible concretions is essential for cure. PMID:28248874

  6. Clinical Features of Spontaneous Partial Healing During Mycobacterium ulcerans Infection

    PubMed Central

    Marion, Estelle; Chauty, Annick; Kempf, Marie; Le Corre, Yannick; Delneste, Yves; Croue, Anne; Marsollier, Laurent

    2016-01-01

    Background. Buruli ulcer, caused by Mycobacterium ulcerans, is a necrotizing skin disease leading to extensive cutaneous and subcutaneous destruction and functional limitations. Spontaneous healing in the absence of medical treatment occurs in rare cases, but this has not been well described in the literature. Methods. In a retrospective case study in an area of Benin where this disease is highly endemic, we selected 26 Buruli ulcer patients presenting features of spontaneous healing from a cohort of 545 Buruli ulcer patients treated between 2010 and 2013. Results. The 26 patients studied had a median age of 13.5 years and were predominantly male (1.4:1). Three groups of patients were defined on the basis of their spontaneous healing characteristics. The first group (12 patients) consisted of patients with an ulcer of more than 1 year′s duration showing signs of healing. The second (13 patients) group contained patients with an active Buruli ulcer lesion some distance away from a first lesion that had healed spontaneously. Finally, the third group contained a single patient displaying complete healing of lesions from a nodule, without treatment and with no relapse. Conclusions. We defined several features of spontaneous healing in Buruli ulcer patients and highlighted the difficulties associated with diagnosis and medical management. Delays in consultation contributed to the high proportion of patients with permanent sequelae and a risk of squamous cell carcinoma. Early detection and antibiotic treatment are the best ways to reduce impairments. PMID:26925431

  7. Clinical and molecular features of young-onset colorectal cancer

    PubMed Central

    Ballester, Veroushka; Rashtak, Shahrooz; Boardman, Lisa

    2016-01-01

    Colorectal cancer (CRC) is one of the leading causes of cancer related mortality worldwide. Although young-onset CRC raises the possibility of a hereditary component, hereditary CRC syndromes only explain a minority of young-onset CRC cases. There is evidence to suggest that young-onset CRC have a different molecular profile than late-onset CRC. While the pathogenesis of young-onset CRC is well characterized in individuals with an inherited CRC syndrome, knowledge regarding the molecular features of sporadic young-onset CRC is limited. Understanding the molecular mechanisms of young-onset CRC can help us tailor specific screening and management strategies. While the incidence of late-onset CRC has been decreasing, mainly attributed to an increase in CRC screening, the incidence of young-onset CRC is increasing. Differences in the molecular biology of these tumors and low suspicion of CRC in young symptomatic individuals, may be possible explanations. Currently there is no evidence that supports that screening of average risk individuals less than 50 years of age will translate into early detection or increased survival. However, increasing understanding of the underlying molecular mechanisms of young-onset CRC could help us tailor specific screening and management strategies. The purpose of this review is to evaluate the current knowledge about young-onset CRC, its clinicopathologic features, and the newly recognized molecular alterations involved in tumor progression. PMID:26855533

  8. Hypertrophic scar in a dog: histological and clinical features.

    PubMed

    Avallone, Giancarlo; Bonaldi, Martina; Caniatti, Mario; Lombardo, Rocco

    2011-08-01

    An adult male bullmastiff dog was treated for paraparesis and ataxia due to discospondylitis and disc herniation. At this time, the dog had a nonhealing ulcer between the pads of the left hindfoot. At re-evaluation, the dog had developed a large exophitic mass in the previously ulcerated area. Cytological examination revealed occasional spindle cells with mild atypia, and a soft tissue tumour was suspected. The mass was excised and submitted for histology. The lesion was characterized by superficial ulceration, an intermediate layer of granulation tissue and a deep portion containing vertically orientated capillaries and perpendicularly arranged fibroblasts and collagen. The histological features led to a diagnosis of hypertrophic scar. Eight weeks after surgery, the lesion recurred and was treated with an intralesional injection of methylprednisolone acetate. The lesion regressed in 10 days, but recurred after 3 months following severe self-trauma. Hypertrophic scars and keloids are two types of exuberant scarring reported in human beings, the pathogenesis of which is still unclear but seems to involve several cytokines, growth factors and inflammatory cells. The histological features identified in this case paralleled those reported in hypertrophic scars in humans. In this case, intralesional corticosteroid therapy was useful in the management of the lesion, but the severe self-trauma could have influenced the recurrence. Even if uncommon, hypertrophic scar should be included among the differential diagnoses of spindle cell tumours in dogs. © 2011 The Authors. Veterinary Dermatology. © 2011 ESVD and ACVD.

  9. LEPROSY NEPHROPATHY: A REVIEW OF CLINICAL AND HISTOPATHOLOGICAL FEATURES

    PubMed Central

    da Silva, Geraldo Bezerra; Daher, Elizabeth De Francesco; Pires, Roberto da Justa; Pereira, Eanes Delgado Barros; Meneses, Gdayllon Cavalcante; Araújo, Sônia Maria Holanda Almeida; Barros, Elvino José Guardão

    2015-01-01

    Leprosy is a chronic disease caused by Mycobacterium leprae, highly incapacitating, and with systemic involvement in some cases. Renal involvement has been reported in all forms of the disease, and it is more frequent in multibacillary forms. The clinical presentation is variable and is determined by the host immunologic system reaction to the bacilli. During the course of the disease there are the so called reactional states, in which the immune system reacts against the bacilli, exacerbating the clinical manifestations. Different renal lesions have been described in leprosy, including acute and chronic glomerulonephritis, interstitial nephritis, secondary amyloidosis and pyelonephritis. The exact mechanism that leads to glomerulonephritis in leprosy is not completely understood. Leprosy treatment includes rifampicin, dapsone and clofazimine. Prednisone and non-steroidal anti-inflammatory drugs may be used to control acute immunological episodes. PMID:25651321

  10. Clinical, biological, and imaging features of monogenic Alzheimer's Disease.

    PubMed

    Pilotto, Andrea; Padovani, Alessandro; Borroni, Barbara

    2013-01-01

    The discovery of monogenic forms of Alzheimer's Disease (AD) associated with mutations within PSEN1, PSEN2, and APP genes is giving a big contribution in the understanding of the underpinning mechanisms of this complex disorder. Compared with sporadic form, the phenotype associated with monogenic cases is somewhat broader including behavioural disturbances, epilepsy, myoclonus, and focal presentations. Structural and functional imaging show typical early changes also in presymptomatic monogenic carriers. Amyloid imaging and CSF tau/A β ratio may be useful in the differential diagnosis with other neurodegenerative dementias, especially, in early onset cases. However, to date any specific biomarkers of different monogenic cases have been identified. Thus, in clinical practice, the early identification is often difficult, but the copresence of different elements could help in recognition. This review will focus on the clinical and instrumental markers useful for the very early identification of AD monogenic cases, pivotal in the development, and evaluation of disease-modifying therapy.

  11. Gluteal Tendinopathy: Integrating Pathomechanics and Clinical Features in Its Management.

    PubMed

    Grimaldi, Alison; Fearon, Angela

    2015-11-01

    Synopsis Gluteal tendinopathy is now believed to be the primary local source of lateral hip pain, or greater trochanteric pain syndrome, previously referred to as trochanteric bursitis. This condition is prevalent, particularly among postmenopausal women, and has a considerable negative influence on quality of life. Improved prognosis and outcomes in the future for those with gluteal tendinopathy will be underpinned by advances in diagnostic testing, a clearer understanding of risk factors and comorbidities, and evidence-based management programs. High-quality studies that meet these requirements are still lacking. This clinical commentary provides direction to assist the clinician with assessment and management of the patient with gluteal tendinopathy, based on currently limited available evidence on this condition and the wider tendon literature and on the combined clinical experience of the authors. J Orthop Sports Phys Ther 2015;45(11):910-922. Epub 17 Sep 2015. doi:10.2519/jospt.2015.5829.

  12. Myelofibrosis: Review of clinical and pathological features in fourteen dogs

    PubMed Central

    Hoff, Brent; Lumsden, John H.; Valli, Victor E.O.; Kruth, Stephen A.

    1991-01-01

    A clinicopathological study was performed on 14 dogs with myelofibrosis (MF), in order to correlate clinical, laboratory, and histomorphological parameters and investigate factors of prognostic significance. The clinical signs included fatigue, weight loss, anorexia, and diarrhea. Physical findings included pale mucous membranes and wasting/emaciation. The major laboratory observations were moderate to severe, poorly-responsive anemia with various degrees of marrow cellularity and fibrosis. All dogs with severe, non-responsive anemia should have a bone marrow core biopsy, stained for connective tissue, in order to detect myelofibrosis. Myelofibrosis regressed in six dogs. ImagesFigure 1aFigure 1bFigure 1cFigure 1d PMID:17423803

  13. Genetic and clinical features of primary torsion dystonia

    PubMed Central

    Ozelius, Laurie J.; Bressman, Susan B.

    2011-01-01

    Primary torsion dystonia (PTD) is defined as a syndrome in which dystonia is the only clinical sign (except for tremor), and there is no evidence of neuronal degeneration or an acquired cause by history or routine laboratory assessment. Seven different loci have been recognized for PTD but only two of the genes have been identified. In this review we will described the phenotypes associated with these loci and discuss the responsible gene. PMID:21168499

  14. Epidemiological Features of Bronchiolitis in the Pediatric Clinic of Clinical center of Sarajevo University

    PubMed Central

    Bakalovic, Ganimeta; Dzinovic, Amra; Baljic, Rusmir; Dizdar, Selma; Selimovic, Amina

    2015-01-01

    Objective: To present the epidemiological features of bronchiolitis in a one-year period in patients of Pediatric Clinic, Clinical Centre of Sarajevo University. Introduction: Bronchiolitis is the most common respiratory infection of early infant age. The disease is one the most common reason for hospitalization of children under the age of six months. The disease is characterized by occurrences in the winter season November–March. For daily diagnosing of severe forms of bronchiolitis as a clinical syndrome, often sufficient are the knowledge of the epidemiological data, age of the patient, clinical examination and insight into the risk factors. Patients and methods: The history of the disease in 155 infant patients, who were clinically treated because of bronchiolitis in the period from February 2013 to February 2014 in the Department of Pediatric Pulmonary Clinic in Sarajevo was retrospectively analyzed. Results: The majority of patients were aged less than 6 months (87.7%). The monthly distribution of bronchiolitis had a peak in January and February. Almost 50% of patients had a risk factors for the development of severe forms of bronchiolitis out of which the most common were artificial diet (53.5%), low birth weight below 2500g (17%), prematurity (16.1%), congenital heart anomalies (14.2%), bronchopulmonary dysplasia (1.9%). 46,5% of patients were on natural nutrition. 46 patients (29.6%) were serologically or by respi-strip test in nasopharyngeal lavage positive on respiratory syncytial virus (RSV). There were no patients who required mechanical ventilation. Conclusion: During the one-year period, bronchiolitis was the most common diagnose in the early infantile period up to 6 months, with a peak incidence in January and February. Risk factors such as prematurity, low birth weight, congenital heart anomalies and bronchopulmonary dysplasia have been less present in the studied period compared to the period before the introduction of RSV chemoprophylaxis. The

  15. Lichenoid sarcoidosis: a case with clinical and histopathological lichenoid features.

    PubMed

    Garrido-Ruiz, Maria C; Enguita-Valls, Ana B; de Arriba, Marta González; Vanaclocha, Francisco; Peralto, Jose Luis Rodriguez

    2008-06-01

    Sarcoidosis is a chronic multisystemic granulomatous disease of unknown etiology, characterized by the formation of noncaseating granulomas in the involved organs. Cutaneous involvement is about 25% with different clinical expressions, the lichenoid pattern being one of the rarest types of cutaneous sarcoidosis. Lichenoid sarcoidosis clinically manifests with multiple scale papules involving extensive skin areas, especially the trunk, limbs, and face mimicking a lichen planus. Although diverse histologic patterns have been previously related, a lichenoid granulomatous infiltrate involving the dermo-epidermal junction has never been reported in lichenoid sarcoidosis. We report a case of a 43-year-old woman presenting with skin-colored pruritic papules, slightly scaling in trunk, extremities, and ears. These symptoms condition continued to expand and worsen for several years. The patient was otherwise in good health with no lymphadenopathies. Histopathologic examination of a skin biopsy showed an upper dermal granulomatous infiltrate of epithelioid cells, without necrosis, distributed in a lichenoid pattern with many cytoid bodies. We consider this may be the first case presenting a characteristic microscopic granulomatous lichen-like pattern in the setting of a clinically lichenoid type of sarcoidosis.

  16. Clinical features and diagnosis of epidermolysis bullosa acquisita.

    PubMed

    Vorobyev, Artem; Ludwig, Ralf J; Schmidt, Enno

    2017-02-01

    Epidermolysis bullosa acquisita (EBA) is a rare autoimmune blistering disease of skin and mucous membranes. EBA is caused by autoantibodies against type VII collagen, which is a major component of anchoring fibrils, attaching epidermis to dermis. Binding of autoantibodies to type VII collagen leads to skin fragility and, finally, blister formation. The clinical picture of EBA is polymorphic, with several distinct phenotypes being described. Despite recent progress in understanding the pathophysiology of EBA, its diagnosis is still challenging. Areas covered: This review provides an update on the clinical manifestations and diagnostic methods of EBA. We searched PubMed using the terms 'epidermolysis bullosa acquisita' covering articles in English between 1 January 2005 and 31 May 2016. Relevant older publications were retrieved form cited literature. Expert commentary: While the clinical picture is highly variable, diagnosis relies on direct immunofluorescence (IF) microscopy of a perilesional skin biopsy. Linear deposits of IgG, IgA and/or C3 along the dermal-epidermal junction with an u-serrated pattern are diagnostic for EBA alike the detection of serum autoantibodies against type VII collagen. Several test systems for the serological diagnosis of EBA have recently become widely available. In some patients, sophisticated diagnostic approaches only available in specialized centers are required.

  17. Spontaneous Primary Intraventricular Hemorrhage: Clinical Features and Early Outcome

    PubMed Central

    Arboix, Adrià; García-Eroles, Luis; Vicens, Adela; Oliveres, Montserrat; Massons, Joan

    2012-01-01

    Purpose. Primary hemorrhage in the ventricular system without a recognizable parenchymal component is very rare. This single-center retrospective study aimed to further characterize the clinical characteristics and early outcome of this stroke subtype. Methods. All patients with primary intraventricular hemorrhage included in a prospective hospital-based stroke registry over a 19-year period were assessed. A standardized protocol with 161 items, including demographics, risk factors, clinical data, neuroimaging findings, and outcome, was used for data collection. A comparison was made between the groups of primary intraventricular hemorrhage and subcortical intracerebral hemorrhage. Predictors of primary intraventricular hemorrhage were identified by logistic regression analysis. Results. There were 12 patients with primary intraventricular hemorrhage (0.31% of all cases of stroke included in the database) and 133 in the cohort of subcortical hemorrhage. Very old age (≥85 years) (odds ratio (OR) 9.89), atrial fibrillation (OR 8.92), headache (OR 6.89), and altered consciousness (OR 4.36) were independent predictors of intraventricular hemorrhage. The overall in-hospital mortality rate was 41.7% (5/12) but increased to 60% (3/5) in patients aged 85 years or older. Conclusion. Although primary intraventricular hemorrhage is uncommon, it is a severe clinical condition with a high early mortality. The prognosis is particularly poor in very old patients. PMID:22966468

  18. [Scabies: epidemiological, clinical and therapeutic features in Bangui].

    PubMed

    Kobangué, L; Guéréndo, P; Abéyé, J; Namdito, P; Mballa, M D; Gresenguet, G

    2014-02-01

    The scabies infects about 300 million people worldwide. Its spread is linked to living conditions especially in economically poor countries. In Central African Republic (CAR) we do not have data on this disease often causes morbidity and expenses, and the disease is often confused and poorly treated. The authors' goal was to describe the epidemiological, clinical and therapeutic characteristics of scabies in Bangui. This was a cross-sectional study by counting records of cases of scabies observed in the dermatology and venereology department of Bangui from 1 January 2006 to 31 December 2010. The diagnosis was based mainly on the combination of a concept of pruritus predominantly night with the notion of contagion and preferential localization of lesions. Three hundred and seventy six cases of scabies were identified from a total of 6391 patients (a hospital prevalence of 5.88%) with high frequency among the population aged 0 to 9 years (33%), an important achievement of the disadvantaged classes (preschool age and pupils / students respectively 25.5% and 26.3%), a prevalence of scabies nodules as type of clinical lesion, localization predominantly on buttocks and the most common complication of eczema-type (19.9 %). Benzyl benzoate solution at 25% applied for 2 consecutive days yielded very satisfactory results (96.7% on day 28) in all forms. The scabies is present in CAR with classical clinical and epidemiological aspects. We recommend first-line benzyl benzoate in two days of application.

  19. Suicide attempts and clinical features of bipolar patients.

    PubMed

    Berkol, Tonguç D; İslam, Serkan; Kırlı, Ebru; Pınarbaşı, Rasim; Özyıldırım, İlker

    2016-06-01

    To identify clinical predictors of suicide attempts in patients with bipolar disorder. This study included bipolar patients who were treated in the Psychiatry Department, Haseki Training and Research Hospital, Istanbul, Turkey, between 2013 and 2014; an informed consent was obtained from the participants. Two  hundred and eighteen bipolar patients were assessed by using the structured clinical interview for Diagnostic and Statistical Manual of Mental Disorders, 4th edition (DSM-IV) Axis-I (SCID-I) in order to detect all possible psychiatric comorbid diagnoses. Clinical predictors of suicide attempts were examined in attempters and non-attempters. The study design was retrospective. The lifetime suicide attempt rate for the entire sample was 19.2%. Suicide attempters with bipolar disorder had more lifetime comorbidity of eating disorder. Female gender and family history of mood disorder were significant predictors for suicide attempts. There was no difference between groups in terms of bipolar disorder subtype, onset age of bipolar disorder, total number of episodes, first and predominant episode type, suicide history in first degree relatives, severity of episodes, and hospitalization and being psychotic. Our study revealed that female gender, family history of mood disorder, and eating disorder are more frequent in bipolar patients with at least one suicide attempt.

  20. Geographic atrophy: clinical features and potential therapeutic approaches.

    PubMed

    Holz, Frank G; Strauss, Erich C; Schmitz-Valckenberg, Steffen; van Lookeren Campagne, Menno

    2014-05-01

    In contrast to wet age-related macular degeneration (AMD), where loss of vision is typically acute and treatment leads to a relatively rapid reduction in retinal fluid and subsequent improvements in visual acuity (VA), disease progression and vision loss in geographic atrophy (GA) owing to AMD are gradual processes. Although GA can result in significant visual function deficits in reading, night vision, and dark adaptation, and produce dense, irreversible scotomas in the visual field, the initial decline in VA may be relatively minor if the fovea is spared. Because best-corrected VA does not correlate well with GA lesions or progression, alternative clinical endpoints are being sought. These include reduction in drusen burden, slowing the enlargement rate of GA lesion area, and slowing or eliminating the progression of intermediate to advanced AMD. Among these considerations, slowing the expansion of the GA lesion area seems to be a clinically suitable primary efficacy endpoint. Because GA lesion growth is characterized by loss of photoreceptors, it is considered a surrogate endpoint for vision loss. Detection of GA can be achieved with a number of different imaging techniques, including color fundus photography, fluorescein angiography, fundus autofluorescence (FAF), near-infrared reflectance, and spectral-domain optical coherence tomography. Previous studies have identified predictive characteristics for progression rates including abnormal patterns of FAF in the perilesional retina. Although there is currently no approved or effective treatment to prevent the onset and progression of GA, potential therapies are being evaluated in clinical studies.

  1. Clinical features, comorbidity, and cognitive impairment in elderly bipolar patients

    PubMed Central

    Rise, Ida Vikan; Haro, Josep Maria; Gjervan, Bjørn

    2016-01-01

    Introduction Data specific to late-life bipolar disorder (BD) are limited. Current research is sparse and present guidelines are not adapted to this group of patients. Objectives We present a literature review on clinical characteristics, comorbidities, and cognitive impairment in patients with late-life BD. This review discusses common comorbidities that affect BD elders and how aging might affect cognition and treatment. Methods Eligible studies were identified in MedLine by the Medical Subject Headings terms “bipolar disorder” and “aged”. We only included original research reports published in English between 2012 and 2015. Results From 414 articles extracted, 16 studies were included in the review. Cardiovascular and respiratory conditions, type II diabetes, and endocrinological abnormalities were observed as highly prevalent. BD is associated with a high suicide risk. Bipolar elderly had an increased risk of dementia and performed worse on cognitive screening tests compared to age-matched controls across different levels of cognition. Despite high rates of medical comorbidity among bipolar elderly, a systematic under-recognition and undertreatment of cardiovascular disease have been suggested. Conclusion There was a high burden of physical comorbidities and cognitive impairment in late-life BD. Bipolar elderly might be under-recorded and undertreated in primary medical care, indicating that this group needs an adapted clinical assessment and specific clinical guidelines need to be established. PMID:27274256

  2. Clinical and pathological features of alcohol-related brain damage.

    PubMed

    Zahr, Natalie M; Kaufman, Kimberley L; Harper, Clive G

    2011-05-01

    One of the sequelae of chronic alcohol abuse is malnutrition. Importantly, a deficiency in thiamine (vitamin B(1)) can result in the acute, potentially reversible neurological disorder Wernicke encephalopathy (WE). When WE is recognized, thiamine treatment can elicit a rapid clinical recovery. If WE is left untreated, however, patients can develop Korsakoff syndrome (KS), a severe neurological disorder characterized by anterograde amnesia. Alcohol-related brain damage (ARBD) describes the effects of chronic alcohol consumption on human brain structure and function in the absence of more discrete and well-characterized neurological concomitants of alcoholism such as WE and KS. Through knowledge of both the well-described changes in brain structure and function that are evident in alcohol-related disorders such as WE and KS and the clinical outcomes associated with these changes, researchers have begun to gain a better understanding of ARBD. This Review examines ARBD from the perspective of WE and KS, exploring the clinical presentations, postmortem brain pathology, in vivo MRI findings and potential molecular mechanisms associated with these conditions. An awareness of the consequences of chronic alcohol consumption on human behavior and brain structure can enable clinicians to improve detection and treatment of ARBD.

  3. Tuberculous spondylodiscitis: epidemiology, clinical features, treatment, and outcome.

    PubMed

    Trecarichi, E M; Di Meco, E; Mazzotta, V; Fantoni, M

    2012-04-01

    Tuberculous spondylodiscitis (TS) is a rare but serious clinical condition which may lead to severe deformity and early or late neurological complications. To discuss certain aspects of the approach to TSs, focusing upon epidemiology, diagnosis, and treatment outcome. For the purpose of this review, a literature search was performed using the Pubmed database through to 19th October 2011 to identify studies published in the last 20 years, concerned in epidemiological, clinical, diagnostic, and therapeutical aspects of TS in adults. Only studies drafted in English language and reporting case series of more than 20 patients have been included. TS has been reported to accounts for 1-5% of all TB cases, and for about 50% of the cases of articulo-skeletal TB infections. Despite the actual availability of more effective diagnostic tools, early recognition of TS remains difficult and a high index of suspicion is needed due to the chronic nature of the disease and its insidious and variable clinical presentation. A prompt diagnosis is required to improve long term outcome, and a microbiological confirmation is recommended to enable appropriate choice of anti-mycobacterial agents. Surgery has an important role in alleviating pain, correcting deformities and neurological impairment, and restoring function. Further studies are required to assess the appropriate duration of anti-microbial treatment, also in regarding of a combined surgical approach.

  4. [Dengue with unusual clinical features in an infant. Case report].

    PubMed

    Méndez-Domínguez, Nina; Achach-Medina, Kikey; Morales-Gual, Yareni María; Gómez-Carro, Salvador

    2016-07-01

    The state of Yucatan, in Mexico, is an endemic area for dengue. During 2015, there was an unpredicted increase in the number of expected cases of dengue fever. To describe and analyse the clinical presentation, progress, and management of a case of dengue infection with non-specific clinical manifestations in an infant which resulted in a dengue shock syndrome. One month old infant admitted to hospital with a generalised rash and a history of being bitten by an insect. He was diagnosed with anaphylaxis based on clinical manifestations and anamnesis. While in hospital, he developed hypotension, tachycardia, anaemia, and respiratory distress. He was transferred to the intensive care unit, but died on the fifth day. He tested positive to dengue virus in the PCR test and for IgG antibodies using Elisa. The basic cause of death was dengue shock syndrome. Dengue fever in young infant infections may be afebrile, so it is important to suspect them appropriately in the presence of a generalised rash, tachycardia, and hypotension, in order to avoid the deadly consequences of dengue shock. Copyright © 2016 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  5. Central Neurocytoma: A Review of Clinical Management and Histopathologic Features.

    PubMed

    Lee, Seung J; Bui, Timothy T; Chen, Cheng Hao Jacky; Lagman, Carlito; Chung, Lawrance K; Sidhu, Sabrin; Seo, David J; Yong, William H; Siegal, Todd L; Kim, Minsu; Yang, Isaac

    2016-10-01

    Central neurocytoma (CN) is a rare, benign brain tumor often located in the lateral ventricles. CN may cause obstructive hydrocephalus and manifest as signs of increased intracranial pressure. The goal of treatment for CN is a gross total resection (GTR), which often yields excellent prognosis with a very high rate of tumor control and survival. Adjuvant radiosurgery and radiotherapy may be considered to improve tumor control when GTR cannot be achieved. Chemotherapy is also not considered a primary treatment, but has been used as a salvage therapy. The radiological features of CN are indistinguishable from those of other brain tumors; therefore, many histological markers, such as synaptophysin, can be very useful for diagnosing CNs. Furthermore, the MIB-1 Labeling Index seems to be correlated with the prognosis of CN. We also discuss oncogenes associated with these elusive tumors. Further studies may improve our ability to accurately diagnose CNs and to design the optimal treatment regimens for patients with CNs.

  6. Clinical feasibility of rapid confocal melanoma feature detection

    NASA Astrophysics Data System (ADS)

    Hennessy, Ricky; Jacques, Steve; Pellacani, Giovanni; Gareau, Daniel

    2010-02-01

    In vivo reflectance confocal microscopy shows promise for the early detection of malignant melanoma. One diagnostic trait of malignancy is the presence of pagetoid melanocytes in the epidermis. For automated detection of MM, this feature must be identified quantitatively through software. Beginning with in vivo, noninvasive confocal images from 10 unequivocal MMs and benign nevi, we developed a pattern recognition algorithm that automatically identified pagetoid melanocytes in all four MMs and identified none in five benign nevi. One data set was discarded due to artifacts caused by patient movement. With future work to bring the performance of this pattern recognition technique to the level of the clinicians on difficult lesions, melanoma diagnosis could be brought to primary care facilities and save many lives by improving early diagnosis.

  7. Cutaneous invasion from sarcomatoid urothelial carcinoma: clinical and dermatopathologic features*

    PubMed Central

    Bernardes Filho, Fred; de Melo, Alessandro Severo Alves; Pires, Andréa Rodriguez Cordovil; Lupi, Omar; Neves, Daniel Gama das; da Cruz, Margareth Fernandes; Kac, Bernard Kawa

    2016-01-01

    In Brazil, without considering the non-melanoma skin tumors, bladder cancer in men is the eighth most common, and the urothelial carcinoma or transitional cell carcinoma is the most common among these. Cutaneous metastases from urothelial neoplasms appear as single or multiple erythematous, infiltrated nodules or plaques, and like other cases of distant disease, it is indicative of poor prognosis. The invasive urothelial carcinoma is recognized for its ability to present divergent differentiation and morphological variants. The sarcomatoid urothelial carcinoma is a rare cancer that consists of two different components: one composed of epithelial tissue and the other with sarcomatoid features of mesenchymal origin. The authors describe a case of cutaneous metastasis of sarcomatoid urothelial carcinoma in a 63-year-old male patient. PMID:26982782

  8. Central Neurocytoma: A Review of Clinical Management and Histopathologic Features

    PubMed Central

    Lee, Seung J.; Bui, Timothy T.; Chen, Cheng Hao Jacky; Lagman, Carlito; Chung, Lawrance K.; Sidhu, Sabrin; Seo, David J.; Yong, William H.; Siegal, Todd L.; Kim, Minsu

    2016-01-01

    Central neurocytoma (CN) is a rare, benign brain tumor often located in the lateral ventricles. CN may cause obstructive hydrocephalus and manifest as signs of increased intracranial pressure. The goal of treatment for CN is a gross total resection (GTR), which often yields excellent prognosis with a very high rate of tumor control and survival. Adjuvant radiosurgery and radiotherapy may be considered to improve tumor control when GTR cannot be achieved. Chemotherapy is also not considered a primary treatment, but has been used as a salvage therapy. The radiological features of CN are indistinguishable from those of other brain tumors; therefore, many histological markers, such as synaptophysin, can be very useful for diagnosing CNs. Furthermore, the MIB-1 Labeling Index seems to be correlated with the prognosis of CN. We also discuss oncogenes associated with these elusive tumors. Further studies may improve our ability to accurately diagnose CNs and to design the optimal treatment regimens for patients with CNs. PMID:27867912

  9. The capybara eye: clinical tests, anatomic and biometric features.

    PubMed

    Montiani-Ferreira, Fabiano; Truppel, Jesse; Tramontin, Mariana H; Vilani, Ricardo G D'Octaviano; Lange, Rogério R

    2008-01-01

    To carry out a descriptive investigation of the capybara (Hydrochaeris hydrochaeris) eye and to perform selected ophthalmic diagnostic tests with the aim of establishing normal physiological reference values for this species. A total of 22 healthy, capybaras were used to test most of the parameters in this investigation. Ages varied from 2 to 4 years of age. Selected diagnostic ocular tests were performed including Schirmer tear test, tonometry using an applanation tonometer (Tonopen), central corneal thickness using an ultrasonic pachymeter (Sonomed, Micropach, Model 200P +), axial globe length and culture of the normal conjunctival bacterial flora. Capybara's normal ocular features include: dorsal and ventral puncta, vestigial third eyelid, true cilia only at the upper eyelid margins. The bulbar conjunctiva is noticeably densely pigmented with a brown to bronze color. The capybara's pupil is oval in shape and vertical in position No tapetum lucidum is present in this species and the retinal blood vessels are almost absent. Results for selected ocular diagnostic tests investigated were: Intraocular pressure: 18.4 +/- 3.8 mmHg; Schirmer tear test: 14.9 +/- 5.1 mm/min; Central corneal thickness: 0.46 +/- 0.03 mm; Axial globe length: 22.20 +/- 1.71 mm. No statistically significant differences between ages or genders were found for any of the results. Corynebacterium sp., Micrococcus sp., Bacillus sp. and Staphylococcus sp. were isolated from healthy conjunctiva, suggesting they are normal constituents of the conjunctival flora of the capybara eye. The corneal epithelium of the capybara possesses a thin and discrete Bowman's layer. Results and parameters obtained in this investigation exposed unique anatomic features of the capybara eye and will help veterinary ophthalmologists to more accurately diagnose discrete or unusual pathological changes of the capybara eye. Furthermore, corneal thickness and axial length of the capybara are similar to that of human beings

  10. Clinical Features of Alzheimer Disease With and Without Lewy Bodies

    PubMed Central

    Chung, Eun Joo; Babulal, Ganesh M.; Monsell, Sarah E.; Cairns, Nigel J.; Roe, Catherine M.; Morris, John C.

    2015-01-01

    IMPORTANCE Lewy bodies are a frequent coexisting pathology in late-onset Alzheimer disease (AD). Previous studies have examined the contribution of Lewy bodies to the clinical phenotype of late-onset AD with variable findings. OBJECTIVE To determine whether the presence of Lewy body pathology influences the clinical phenotype and progression of symptoms in longitudinally assessed participants with AD. DESIGN, SETTING, AND PARTICIPANTS Retrospective clinical and pathological cohort study of 531 deceased participants who met the neuropathologic criteria for intermediate or high likelihood of AD according to the National Institute on Aging–Ronald Reagan Institute guidelines for the neuropathologic diagnosis of AD. All participants had a clinical assessment within 2 years of death. The data were obtained from 34 AD centers maintained by the National Alzheimer Coordinating Center and spanned from September 12, 2005, to April 30, 2013. EXPOSURES Standardized neuropathologic assessment and then brain autopsy after death. MAIN OUTCOMES AND MEASURES Clinical and neuropsychiatric test scores. RESULTS The mean (SD) age at death was statistically significantly younger for participants who had AD with Lewy bodies (77.9 [9.5] years) than for participants who had AD without Lewy bodies (80.2 [11.1] years) (P = .01). The mean (SD) age at onset of dementia symptoms was also younger for participants who had AD with Lewy bodies (70.0 [9.9] years) than for participants who had AD without Lewy bodies (72.2 [12.3] years) (P = .03). More men than women had AD with Lewy bodies (P = .01). The frequency of having at least 1 APOE ε4 allele was higher for participants who had AD with Lewy bodies than for participants who had AD without Lewy bodies (P = .03). After adjusting for age, sex, education, frequency of plaques (neuritic and diffuse), and tangle stage, we found that participants who had AD with Lewy bodies had a statistically significantly higher mean (SD) Neuropsychiatric

  11. Clinical Features of Alzheimer Disease With and Without Lewy Bodies.

    PubMed

    Chung, Eun Joo; Babulal, Ganesh M; Monsell, Sarah E; Cairns, Nigel J; Roe, Catherine M; Morris, John C

    2015-07-01

    Lewy bodies are a frequent coexisting pathology in late-onset Alzheimer disease (AD). Previous studies have examined the contribution of Lewy bodies to the clinical phenotype of late-onset AD with variable findings. To determine whether the presence of Lewy body pathology influences the clinical phenotype and progression of symptoms in longitudinally assessed participants with AD. Retrospective clinical and pathological cohort study of 531 deceased participants who met the neuropathologic criteria for intermediate or high likelihood of AD according to the National Institute on Aging-Ronald Reagan Institute guidelines for the neuropathologic diagnosis of AD. All participants had a clinical assessment within 2 years of death. The data were obtained from 34 AD centers maintained by the National Alzheimer Coordinating Center and spanned from September 12, 2005, to April 30, 2013. Standardized neuropathologic assessment and then brain autopsy after death. Clinical and neuropsychiatric test scores. The mean (SD) age at death was statistically significantly younger for participants who had AD with Lewy bodies (77.9 [9.5] years) than for participants who had AD without Lewy bodies (80.2 [11.1] years) (P = .01). The mean (SD) age at onset of dementia symptoms was also younger for participants who had AD with Lewy bodies (70.0 [9.9] years) than for participants who had AD without Lewy bodies (72.2 [12.3] years) (P = .03). More men than women had AD with Lewy bodies (P = .01). The frequency of having at least 1 APOE ε4 allele was higher for participants who had AD with Lewy bodies than for participants who had AD without Lewy bodies (P = .03). After adjusting for age, sex, education, frequency of plaques (neuritic and diffuse), and tangle stage, we found that participants who had AD with Lewy bodies had a statistically significantly higher mean (SD) Neuropsychiatric Inventory Questionnaire score (6.59 [1.44] [95% CI, 3.75-9.42] vs 5.49 [1.39] [95% CI, 2

  12. Spinal meningioma in childhood: clinical features and treatment.

    PubMed

    Wang, Xiao-Qiang; Zeng, Xian-Wei; Zhang, Bi-Yun; Dou, Ya-Fang; Wu, Jin-Song; Jiang, Cheng-Chuan; Zhong, Ping; Chen, Hong

    2012-01-01

    The purpose of this retrospective study is to determine the clinical characteristics and the prognosis of the spinal meningioma in childhood (under 18 years of age) based on the treatment at our institution. Ten spinal meningioma cases in children were treated during the last 9 years. The clinical data was retrospectively analyzed and the results were compared with those in the literature. The series included eight males and two females and the mean age was 13.2 years. The most common initial symptoms were pain (6/10) and the common signs were limb weakness (4/10) and gait disturbance (2/10) and distal paresthesia (1/10) and bladder disturbance (1/10). Four patients had other clinical signs of neurofibromatosis type II (NF-2) such as tumors elsewhere. All the tumors were located in cervical and thoracic vertebrae. Resection according to Simpson Grade I (6/10), II (2/10), III (1/10), and IV (1/10) were performed. Grade II meningiomas accounted for 3/10 in this series. All patients were followed up with mean follow-up period of 43 months. Seven patients had recurrence of the tumor in that period and one had died. Spinal meningioma is an uncommon pediatric neoplasm and has a poor prognosis. It has a male predominance and is inclined to be associated with NF-2, and those that are associated with higher pathologic subtypes and NF-2 have more unfavorable outcome. Every effort should be made to achieve total removal which may decrease the incidence of recurrence.

  13. First Chikungunya Outbreak in Suriname; Clinical and Epidemiological Features

    PubMed Central

    van Genderen, Farah T.; Krishnadath, Ingrid; Sno, Rachel; Grunberg, Meritha G.; Zijlmans, Wilco; Adhin, Malti R.

    2016-01-01

    Background In June 2014, Suriname faced the first Chikungunya outbreak. Since international reports mostly focus on hospitalized patients, the least affected group, a study was conducted to describe clinical characteristics of mainly outpatients including children. In addition, the cumulative incidence of this first epidemic was investigated. Methodology During August and September 2014, clinically suspected Chikungunya cases were included in a prospective follow-up study. Blood specimens were collected and tested for viral RNA presence. Detailed clinical information was gathered through multiple telephone surveys until day 180. In addition, a three stage household-based cluster with a cross-sectional design was conducted in October, December 2014 and March 2015 to assess the cumulative incidence. Principal Findings Sixty-eight percent of symptomatic patients tested positive for Chikungunya virus (CHIKV). Arthralgia and pain in the fingers were distinctive for viremic CHIKV infected patients. Viremic CHIKV infected children (≤12 years) characteristically displayed headache and vomiting, while arthralgia was less common at onset. The disease was cleared within seven days by 20% of the patients, while 22% of the viremic CHIKV infected patients, mostly women and elderly reported persistent arthralgia at day 180. The extrapolated cumulative CHIKV incidence in Paramaribo was 249 cases per 1000 persons, based on CHIKV self-reported cases in 53.1% of the households and 90.4% IgG detected in a subset of self-reported CHIKV+ persons. CHIKV peaked in the dry season and a drastic decrease in CHIKV patients coincided with a governmental campaign to reduce mosquito breeding sites. Conclusions/Significance This study revealed that persistent arthralgia was a concern, but occurred less frequently in an outpatient setting. The data support a less severe pathological outcome for Caribbean CHIKV infections. This study augments incidence data available for first outbreaks in the

  14. Clinical and Immunologic Features of Ultra-Short Celiac Disease.

    PubMed

    Mooney, Peter D; Kurien, Matthew; Evans, Kate E; Rosario, Eleanor; Cross, Simon S; Vergani, Patricia; Hadjivassiliou, Marios; Murray, Joseph A; Sanders, David S

    2016-05-01

    The clinical effects of gluten-sensitive enteropathy with villous atrophy limited to the duodenal bulb (D1) have not been delineated in adults with celiac disease. We investigated the sensitivity of D1 biopsy analysis in the detection of celiac disease, the number and sites of biopsies required to detect ultra-short celiac disease (USCD, villous atrophy limited to D1), and the clinical phenotype of USCD. We performed a prospective study of 1378 patients (mean age, 50.3 y; 62% female) who underwent endoscopy at a tertiary medical center in the United Kingdom from 2008 through 2014; routine duodenal biopsy specimens were collected from D1 and the second part of the duodenum (D2). Quadrantic D1 biopsy specimens were collected from 171 consecutive patients with a high suspicion of celiac disease (mean age, 46.5 y; 64% female). Clinical data from patients diagnosed with USCD, based on biopsy analysis, were compared with those from patients with conventional celiac disease (CCD) (villous atrophy beyond D1) and individuals without celiac disease (controls). The number of intraepithelial lymphocytes (IELs) and immune phenotypes were compared between D1 vs D2 in patients with celiac disease. Of the 1378 patients assessed, 268 (19.4%) were diagnosed with celiac disease; 9.7% of these patients had villous atrophy confined to D1 (USCD; P < .0001). Collection of a single additional biopsy specimen from any D1 site increased the sensitivity of celiac disease detection by 9.3%-10.8% (P < .0001). Patients with USCD were younger (P = .03), had lower titers of tissue transglutaminase antibody (P = .001), and less frequently presented with diarrhea (P = .001) than patients with CCD. Higher proportions of patients with CCD had ferritin deficiency (P = .007) or folate deficiency (P = .003) than patients with USCD or controls. Patients with celiac disease had a median of 50 IELs/100 enterocytes in D1 and a median of 48 IELs/100 enterocytes (P = .7) in D2. The phenotype of IELs

  15. Clinical and pathologic features of thymoma in 15 dogs.

    PubMed

    Aronsohn, M G; Schunk, K L; Carpenter, J L; King, N W

    1984-06-01

    Thymoma was diagnosed in 15 dogs at Angell Memorial Animal Hospital between 1972 and 1983. All thymomas developed in the cranial portion of the mediastinum. An autoimmune paraneoplastic syndrome was observed in 10 (67%) of the dogs and included myasthenia gravis, nonthymic neoplasms, and polymyositis. Clinical signs were variable and inconsistent, depending on whether they were attributable to the cranial mediastinal mass or to the paraneoplastic syndrome. Eleven dogs were necropsied. Two thymomas had gross characteristics of malignancy. In 3 cases, a cell consistent with a subclass of mast cell was found and in 1 thymoma, melanocytes were observed.

  16. Vertebral Artery Dissection: Natural History, Clinical Features and Therapeutic Considerations

    PubMed Central

    Park, Kwan-Woong; Park, Jong-Sun; Hwang, Sun-Chul; Im, Soo-Bin; Shin, Won-Han

    2008-01-01

    When a tear occurs in one of the major cervicocerebral arteries and allows blood to enter the wall of the artery and split its layers, the result is either stenosis or aneurysmal dilatation of the vessel. Vertebral artery dissection (VAD) is an infrequent occurrence but is a leading cause of stroke in young and otherwise healthy patients. This article discusses recent developments in understanding of the epidemiology and pathogenesis of VAD and the various clinical manifestations, methods of diagnosis, and approaches to treatment. PMID:19096659

  17. Venous compression syndromes: clinical features, imaging findings and management

    PubMed Central

    Liu, R; Oliveira, G R; Ganguli, S; Kalva, S

    2013-01-01

    Extrinsic venous compression is caused by compression of the veins in tight anatomic spaces by adjacent structures, and is seen in a number of locations. Venous compression syndromes, including Paget–Schroetter syndrome, Nutcracker syndrome, May–Thurner syndrome and popliteal venous compression will be discussed. These syndromes are usually seen in young, otherwise healthy individuals, and can lead to significant overall morbidity. Aside from clinical findings and physical examination, diagnosis can be made with ultrasound, CT, or MR conventional venography. Symptoms and haemodynamic significance of the compression determine the ideal treatment method. PMID:23908347

  18. Auditory dysfunction in schizophrenia: integrating clinical and basic features

    PubMed Central

    Javitt, Daniel C.; Sweet, Robert A.

    2015-01-01

    Schizophrenia is a complex neuropsychiatric disorder that is associated with persistent psychosocial disability in affected individuals. Although studies of schizophrenia have traditionally focused on deficits in higher-order processes such as working memory and executive function, there is an increasing realization that, in this disorder, deficits can be found throughout the cortex and are manifest even at the level of early sensory processing. These deficits are highly amenable to translational investigation and represent potential novel targets for clinical intervention. Deficits, moreover, have been linked to specific structural abnormalities in post-mortem auditory cortex tissue from individuals with schizophrenia, providing unique insights into underlying pathophysiological mechanisms. PMID:26289573

  19. Glycogen storage diseases: a brief review and update on clinical features, genetic abnormalities, pathologic features, and treatment.

    PubMed

    Hicks, John; Wartchow, Eric; Mierau, Gary

    2011-10-01

    Glycogen storage diseases (GSD) affect primarily the liver, skeletal muscle, heart, and sometimes the central nervous system and the kidneys. These unique diseases are quite varied in age of onset of symptoms, morbidity, and mortality. Glycogen storage diseases are classified according to their individual enzyme deficiency. Each of these enzymes regulates synthesis or degradation of glycogen. Interestingly, there is great phenotypic variation and variable clinical courses even when a specific enzyme is altered by mutation. Depending on the specific mutation in an enzyme, a GSD patient may have a favorable or unfavorable prognosis. With neonatal or infantile forms, some GSDs lead to death within the first year of life, whereas other glycogen storage diseases are relatively asymptomatic or may cause only exercise intolerance. The paper provides a brief review and update of glycogen storage diseases, with respect to clinical features, genetic abnormalities, pathologic features, and treatment.

  20. Stress fractures: pathophysiology, clinical presentation, imaging features, and treatment options.

    PubMed

    Matcuk, George R; Mahanty, Scott R; Skalski, Matthew R; Pa