• Title/Summary/Keyword: early onset

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Epidemioclinical Feature of Early-Onset Colorectal Cancer at-Risk for Lynch Syndrome in Central Iran

  • Zeinalian, Mehrdad;Hashemzadeh-Chaleshtori, Morteza;Akbarpour, Mohammad Javad;Emami, Mohammad Hassan
    • Asian Pacific Journal of Cancer Prevention
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    • 제16권11호
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    • pp.4647-4652
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    • 2015
  • Background: Colorectal cancer (CRC) is becoming one of the most complicated challenges of human health, particularly in developing countries like Iran. In this paper, we try to characterize CRC cases diagnosed < age 50 at-risk for Lynch syndrome within central Iran. Materials and Methods: We designed a descriptive retrospective study to screen all registered CRC patients within 2000-2013 in Poursina Hakim Research Center (PHRC), a referral gastroenterology clinic in central Iran, based on being early-onset (age at diagnosis ${\leq}50years$) and Amsterdam II criteria. We calculated frequencies and percentages by SPSS 19 software to describe clinical and family history characteristics of patients with early-onset CRC. Results: Overall 1,659 CRC patients were included in our study of which 413 (24.9%) were ${\leq}50years$ at diagnosis. Of 219/413 successful calls 67 persons (30.6%) were reported deceased. Family history was positive for 72/219 probands (32.9%) and 53 families (24.2%) were identified as familial colorectal cancer (FCC), with a history of at-least three affected members with any type of cancer in the family, of which 85% fulfilled the Amsterdam II Criteria as hereditary non-polyposis colorectal cancer (HNPCC) families (45/219 or 20.5%). Finally, 14 families were excluded due to proband tumor tissues being unavailable or unwillingness for incorporation. The most common HNPCC-associated extracolonic-cancer among both males and females of the families was stomach, at respectively 31.8 and 32.7 percent. The most common tumor locations among the 31 probands were rectum (32.3%), sigmoid (29.0%), and ascending colon (12.9%). Conclusions: Given the high prevalence of FCC (~1/4 of early-onset Iranian CRC patients), it is necessary to establish a comprehensive cancer genetic counseling and systematic screening program for early detection and to improve cancer prognosis among high risk families.

Very Early-Onset Inflammatory Bowel Disease: A Challenging Field for Pediatric Gastroenterologists

  • Arai, Katsuhiro
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제23권5호
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    • pp.411-422
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    • 2020
  • With the increasing number of children with inflammatory bowel disease (IBD), very early-onset IBD (VEO-IBD), defined as IBD that is diagnosed or that develops before 6 years of age, has become a field of innovation among pediatric gastroenterologists. Advances in genetic testing have enabled the diagnosis of IBD caused by gene mutations, also known as monogenic or Mendelian disorder-associated IBD (MD-IBD), with approximately 60 causative genes reported to date. The diagnosis of VEO-IBD requires endoscopic and histological evaluations. However, satisfactory small bowel imaging studies may not be feasible in this small population. Both genetic and immunological approaches are necessary for the diagnosis of MD-IBD, which can differ among countries according to the available resources. As a result of the use of targeted gene panels covered by the national health insurance and the nationwide research project investigating inborn errors of immunity, an efficient approach for the diagnosis of MD-IBD has been developed in Japan. Proper management of VEO-IBD by pediatric gastroenterologists constitutes a challenge. Some MD-IBDs can be curable by allogenic hematopoietic stem cell transplantation. With an understanding of the affected gene functions, targeted therapies are being developed. Social and psychological support systems for both children and their families should also be provided to improve their quality of life. Multidisciplinary team care would contribute to early diagnosis, proper therapeutic interventions, and improved quality of life in patients and their families.

Early-onset epileptic encephalopathies and the diagnostic approach to underlying causes

  • Hwang, Su-Kyeong;Kwon, Soonhak
    • Clinical and Experimental Pediatrics
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    • 제58권11호
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    • pp.407-414
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    • 2015
  • Early-onset epileptic encephalopathies are one of the most severe early onset epilepsies that can lead to progressive psychomotor impairment. These syndromes result from identifiable primary causes, such as structural, neurodegenerative, metabolic, or genetic defects, and an increasing number of novel genetic causes continue to be uncovered. A typical diagnostic approach includes documentation of anamnesis, determination of seizure semiology, electroencephalography, and neuroimaging. If primary biochemical investigations exclude precipitating conditions, a trial with the administration of a vitaminic compound (pyridoxine, pyridoxal-5-phosphate, or folinic acid) can then be initiated regardless of presumptive seizure causes. Patients with unclear etiologies should be considered for a further workup, which should include an evaluation for inherited metabolic defects and genetic analyses. Targeted next-generation sequencing panels showed a high diagnostic yield in patients with epileptic encephalopathy. Mutations associated with the emergence of epileptic encephalopathies can be identified in a targeted fashion by sequencing the most likely candidate genes. Next-generation sequencing technologies offer hope to a large number of patients with cryptogenic encephalopathies and will eventually lead to new therapeutic strategies and more favorable long-term outcomes.

만발성 정신분열증 4례 (Four Cases of Late-Onset Schizophrenia)

  • 박종득;윤도준
    • 생물정신의학
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    • 제2권2호
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    • pp.295-300
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    • 1995
  • Late-onset schizophrenia(LOS) is a controversial entity. It has been thought that onset of schizophrenia is limited to early adulthood, but many European psychiatrists have reported on the occurrence of schizophrenia in late life. DSM-III restricted the diagnosis of schizophrenia to patients with onset of illness before age 45 years. But, DSM-III-R, DSM-IV, and ICD-10 recognize no upper limit to the age at onset of schizophrenia. Patients with LOS have more visual, tactile, and olfactory hallucinations. Patients with LOS have more persecutory delusions, premorbid schizoid personality traits, and less affective blunting. The course of illness was favorable in LOS. We present four cases of LOS. Their detailed clinical features are reported hear with brief review.

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Comparison between Early and Late Onset Breast Cancer in Pakistani Women Undergoing Breast Conservative Therapy: is There any Difference?

  • Bhatti, Abu Bakar Hafeez;Jamshed, Aarif;Khan, Amina;Siddiqui, Neelam;Muzaffar, Nargis;Shah, Mazhar Ali
    • Asian Pacific Journal of Cancer Prevention
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    • 제15권13호
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    • pp.5331-5336
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    • 2014
  • Background: Early onset breast cancer is associated with poor outcomes but variable results have been reported. It is a significant problem in Pakistani women but remains under reported. Breast conservation plays an important role in surgical management of this younger patient group. The objective of this study was to determine the outcome of breast conservative therapy in patients with early onset breast cancer in our population and compare it with their older counterparts. Materials and Methods: A review of patients with invasive breast cancer who underwent breast conservation surgery at Shaukat Khanum Cancer Hospital from 1997 to 2009 was performed. Patients were divided into two groups i.e. Group I age ${\leq}40$ and Group II >40 years. A total of 401 patients with breast cancer were identified in Group I and 405 patients in Group II. Demographics, histopathological findings and receptor status of the two groups were compared. The Chi square test was used for categorical variables. Outcome was assessed on basis of 10 year locoregional recurrence free survival (LRRFS), disease free survival (DFS) and overall survival (OS). For survival analysis Kaplan Meier curves were used and significance was determined using the Log rank test. Cox regression was applied for multivariate analysis. Results: Median follow up was 4.31 (0.1-15.5) years. Median age at presentation was 34.6 years (17-40) and 51.9 years (41-82) for the two groups. Groups were significantly different from each other with respect to grade, receptor status, tumor stage and use of neoadjuvant therapy. No significant difference was present between the two groups for estimated 10 year LRRFS (86% vs 95%) (p=0.1), DFS (70% vs 70%) (p=0.5) and OS (75% vs 63%) (p=0.1). On multivariate analysis, tumor stage was an independent predictor of LRRFS, DFS and OS. Conclusions: Early onset breast cancer is associated with a distinct biology but does not lead to poorer outcomes in our population.

벨마비 입원환자 35례에 대한 임상보고 조기 한방치료가 벨마비에서 House-Brackmann grade에 미치는 영향을 중심으로 (A Clinical Report on 35 Inpatients with Facial Paralysis Effects of Early Oriental Medicine Treatment on House-Brackmann Grade in Bell's Palsy)

  • 양현주;주현아;백상철;박진수;홍석훈
    • 한방안이비인후피부과학회지
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    • 제24권3호
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    • pp.108-118
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    • 2011
  • Objective : This study was designed to evaluate the influence of time from onset of Bell's palsy until commenced Oriental medicine therapy on Bell's palsy. Methods : We investigated 35 cases of patients with peripheral facial palsy who visited Wonkwang University Oriental Hospital from 10th, December 2010 to 31st August, 2011 and classified them as time from onset of Bell's palsy until commenced Oriental medicine therapy. Time from onset of Bell's palsy until commenced Oriental medicine therapy of Group A was within 3 days. Group B was from 4 days to 8 days. We evaluate the treatment effect of each group by House-Brackmann grade before treatment and 1,3 week treatment. Results : 1. There was a statistical difference in period from onset of Bell's palsy until commenced Oriental medicine therapy between Group A and Group B. 2. There was a statistical difference after 1 week treatment in Group B. 3. There was a statistical difference after 3 weeks treatment in both Group A and B.3. After 3 week treatment, there was statistical significant difference in decreased value of House-Brackmann grade between Group A and Group B. Group A showed more recovery compared with Group B. Conclusions : 1. These results suggest that early oriental medicine treatment could be more effective in recovery of Bell's palsy. 2. Further studies will be required to identify the beneficial effect of early oriental medicine treatment in bell's palsy.

조기발병형 치주염의 임상적 및 면역유전학적 연구 (CLINICAL AND IMMUNOGENETIC STUDY ON THE EARLY-ONSET PERIODONTITIS)

  • 김준홍;김성조;최점일
    • Journal of Periodontal and Implant Science
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    • 제25권3호
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    • pp.568-586
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    • 1995
  • 542 periodontal patients having early-onset periodontitis(EOP) have been reclassified into a more homogeneous phenotypic subsets by newly revised radiographic criteria. Representative patients of each EOP subform have been examined of serum IgG subclass antibodies against periodontopathic bacteria, Porphyromonas gingivalis(Pg) 381 and of genetic markers for IgG allotypes to clarify the relationship between these parameters and phenotype expression of each subform. The early onset periodontitis could be reclassified by the radiographic parameters combining the mean interproximal alveolar bone loss(BL) and the radiographic ratio(between 1st molars and the adjacent teeth: Ratio) with statistical significance(p<0.001 by MANOVA). Moreover these EOP subforms could clearly be delineated from adult periodontitis. Of subform I and II(localized type EOP) patients with minimal mean bone loss(BL<5.0), patients demonstrating disease activities in localized areas(Ratio.>1.5) showed the elevated responses in all the IgG subclasses against Pg compared with those of patients without disease activity(Ratio <1.5). There were gradual increase in the IgG2 and IgG4 titers against Pg as the disease developed into the generalized forms suggesting the possible role of these antibodies in modulating the phenotype expression. The genetic marker study for IgG allotype revealed that mean IgG2 and IgG4 subclass titers were significantly higher(p<0.01, p<0.05, respectively) in patients who were positive for G2m(n). This indicated that IgG subclass responsiveness against the bacterial antigens are under the immnuogenetic control. The observed frequencies of G2m(n) were significantly higher (p<0.05) in subfrom IV patients who had the characteristic features of classical rapidly progressing periodontitis indicating the possible genetic predisposition in these patients.

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BK virus-associated hemorrhagic cystitis after pediatric stem cell transplantation

  • Han, Seung Beom;Cho, Bin;Kang, Jin Han
    • Clinical and Experimental Pediatrics
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    • 제57권12호
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    • pp.514-519
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    • 2014
  • Hemorrhagic cystitis is a common stem cell transplantation-related complication. The incidence of early-onset hemorrhagic cystitis, which is related to the pretransplant conditioning regimen, has decreased with the concomitant use of mesna and hyperhydration. However, late-onset hemorrhagic cystitis, which is usually caused by the BK virus, continues to develop. Although the BK virus is the most common pathogenic microorganism of poststem cell transplantation late-onset hemorrhagic cystitis, pediatricians outside the hemato-oncology and nephrology specialties tend to be unfamiliar with hemorrhagic cystitis and the BK virus. Moreover, no standard guidelines for the early diagnosis and treatment of BK virus-associated hemorrhagic cystitis after stem cell transplantation have been established. Here, we briefly introduce poststem cell transplantation BK virus-associated hemorrhagic cystitis.

비연속시간 생존분석을 적용한 청소년의 최초 가출 발생시점에 대한 영향요인 연구 (Determinants of the Onset of Adolescent Runaway Behavior : An Application of Discrete-Time Survival Analysis)

  • 홍세희;김동기
    • 아동학회지
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    • 제28권2호
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    • pp.217-233
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    • 2007
  • The present study investigated the effects of individual, family, school, and community factors on the onset of adolescent runaway behavior. Using the Korean Youth Panel data(n=3,118), discrete-time survival analysis was applied for research purposes. Results showed that the likelihood of onset of adolescent runaway behavior increased sharply during the early middle school years. The variables of self-control, aggression, parents' divorce, paternal abuse, attachment with parents, and number of delinquent peers were associated with the likelihood of runaway behavior. These results suggest that special attention should be paid to adolescents in the early middle school years and that various preventive programs, e.g., aggression and stress management, and peer relations programs, should be developed and implemented.

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조기 발병 조현병과 후기 발병 조현병의 임상 양상에 대한 비교 연구 (Comparative Study of Clinical Features between Early- and Late-Onset Schizophrenia in South Korea)

  • 고미애;이선구;이정석
    • 대한조현병학회지
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    • 제22권2호
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    • pp.51-55
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    • 2019
  • Objectives: Late-onset schizophrenia (LOS, age at first onset ≥40 years) is characterized by including predominance of women, better premorbid social adjustment and lower severity of positive/negative symptoms. However, few studies have been conducted on LOS, especially in Asian countries. This study aimed to examine the clinical features of LOS in comparison with early-onset schizophrenia (EOS). Methods: By retrospectively reviewing medical records, we assessed demographic data and clinical features of 76 LOS (20 males) and 357 EOS (144 males) who admitted to the psychiatric ward of a general hospital. Results: The mean ages of onset were 47.3±5.1 (LOS) and 25.7±6.5 (EOS) years. There were significantly more women in LOS (73.7%) than EOS (59.6%). Significantly more LOS patients had a marital (88.2% vs. 25.8%) and employment history (28.9% vs. 13.1%) than EOS. Patients with LOS had fewer negative (14.3±9.0 vs. 19.9±9.3), general psychopathology score (36.9±11.1 vs. 42.3±13.9) than EOS patients. Conclusion: In line with previous studies, this study demonstrated that LOS patients have better premorbid social adjustment. Our finding also replicates previous findings that LOS patients differ from EOS in predominance of women and relative lack of negative symptoms. These results suggest that LOS may be a distinct subtype of schizophrenia.