• 제목/요약/키워드: Hematologic disease

검색결과 154건 처리시간 0.031초

Wilson 병의 진단시 임상 발현 양상에 대한 고찰 (The Study of the Initial Presentations of Wilson Disease at Diagonosis)

  • 양태진;지근하;송민섭;황태규
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제4권2호
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    • pp.199-206
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    • 2001
  • 목적: 윌슨병으로 진단 받은 환자들의 진단 당시의 임상 양상의 분포, 발병 연령과 검사 소견들을 파악하고, 또한 초기 발견의 중요성을 고찰하고자 본 연구를 시행하였다. 방법: 1990년 1월부터 10년간 부산 백병원에 입원하여 윌슨병으로 진단 받은 환자 20례를 대상으로 진단 당시의 증상과 증상 발현시기, 진단까지의 기간, 검사 소견 등을 후향적으로 조사하였다. 결과: 20명의 환자들 중에 간 증상 단독 발현은 12명(60%), 그 중 2명은 우연히 AST/ALT가 증가된 것을 발견한 경우였으며, 신경 증상 단독 발현은 2명(10%), 신경 정신증상과 간 증상이 같이 나타난 경우는 4명(20%), 혈액 증상이 주된 경우 1명(5%), 그리고 증상이 없으며 AST/ALT도 정상이나 가족 검진에서 혈중 ceruloplasmin이 감소된 것을 발견한 경우가 1명(5%) 있었다. 모든 환자들에게서 혈중 ceruloplasmin의 농도는 유의하게 낮았다. 가족력은 5례에서 있었다. 간 증상은 진단시 이미 간경화로 진행한 것을 초음파로 진단한 경우가 12명(60%)이었으며, 이 중 2명이 진단 후 1년 내에 사망하였다. 결론: 윌슨병은 빠른 진단과 치료가 중요하다. 그러나 다른 유전성 대사질환들과는 달리 전체 신생아의 선별검사는 의미가 없으며, 환자가 있는 가족에서 혈중 ceruloplasmin과 소변 내 구리, 간 효소 수치와 각막을 검사하는 것이 좋다. 소아기에 간염의 증상이나 지속적으로 증가된 간 효소 수치를 보일 때 또는 가족 중에 원인이 밝혀지지 않은 간질환의 가족력이 있을 때에는 윌슨병을 의심해 보아야 하겠다. 또한 청소년기 이후에 추체외로 증상이나 소뇌 증상 등 운동 신경계의 증상을 호소하든지 혈액 이상, 소변 검사상 이상이 복합적으로 나타나는 경우 등에서도 혈중 ceruloplasmin을 꼭 확인해 보아야 한다. 그 외에 신경 증상 및 정신 증상을 주소로 내원한 경우에도 간기능 검사를 꼭 시행해 보아야 하겠다.

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Ki-67 Can Predict the Response to the Gemcitabine, Oxaliplatin And L-asparaginase Regimen (GELOX) and Prognosis in Patients with Nasal Natural Killer/T-cell Lymphoma

  • Zhang, Jing;Jiang, Wei;Wang, Wei-Da;Liu, Cheng-Cheng;Hu, Yan-Ping;Xia, Zhong-Jun
    • Asian Pacific Journal of Cancer Prevention
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    • 제16권11호
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    • pp.4515-4520
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    • 2015
  • GELOX (gemcitabine, oxaliplatin and L-asparaginase) regimen showed an impressive result in our previous study, but the effect of this new regimen is still dissatisfying for some patients, so it is necessary to identify which patients will benefit from this regimen. A total of fifty-one cases with nasal natural killer/T-cell lymphoma receiving initial GELOX chemotherapy were enrolled in this study. The ki-67 expression detected by immunohistochemistry (IHC) in the specimens ranged from 10% to 90%, with a median value of 70%, so cases higher than the median value (${\geq}70%$) were defined as high ki-67 expression, and the others were designated as low ki-67 expression. The response rate had no statistical difference between low ki-67 expression group and high ki-67 expression group (P=0.291) though the value in the former group was relatively high. After a median follow-up of 18.03 months, the 3-year progression-free survival (PFS) for patients with low ki-67 expression was significantly higher than those with high ki-67 expression (83.8% vs. 47.9%, P=0.038). In the stage I/II subgroup, 3-year PFS and overall survival (OS) were statistically higher in the patients with low ki-67 expression than those with high ki-67 expression. Multivariate analysis revealed high ki-67 expression was an independent prognostic factor for PFS. These results suggest that low ki-67 expression can predict a good response of GELOX in these patients, and the combination of ki-67 expression and early stage is helpful to identify an excellent prognosis subgroup from patients receiving GELOX in this disease.

A prospective study on changes in body composition and fat percentage during the first year of cancer treatment in children

  • Yang, Hye Ran;Choi, Hyoung Soo
    • Nutrition Research and Practice
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    • 제13권3호
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    • pp.214-221
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    • 2019
  • BACKGROUNDS/OBJECTIVES: Cancer treatment may lead to significant body composition changes and affect growth and disease outcomes in pediatric cancer patients. This prospective study aimed to evaluate short- and long-term body compositions changes focused on body fat during the first year of cancer treatment in children. SUBJECTS/METHODS: A prospective study was conducted in 30 pediatric cancer patients (19 hematologic malignancies and 11 solid tumors) and 30 age- and sex-matched healthy controls. Anthropometric measurements and body composition analysis using whole body dual energy X-ray absorptiometry were performed at baseline and 1, 6, and 12 month(s) of cancer treatment. Kruskal-Wallis tests, Wilcoxon paired t tests, and generalized estimation equation (GEE) were applied for statistical analysis. RESULTS: At baseline, no differences in weight, height, body mass index, abdominal circumferences, body fat, and fat-free mass were observed between 30 controls and 30 pediatric cancer patients. Total fat mass (P < 0.001) and body fat percentage (P = 0.002) increased significantly during the first month, but no changes were observed from 1 to 12 months; however, no changes in the total mass were observed during the first year of cancer treatment. Meanwhile, the total fat-free mass decreased during the first month (P = 0.008) and recovered between 6 and 12 months of follow-up (P < 0.001). According to GEE analysis, there was a significant upward trend in body fat percentage during the first year, especially the first month, of cancer treatment in children with hematologic malignancies, but not in those with solid tumors. CONCLUSIONS: Our results indicate that cancer treatment is related to significant body composition changes and rapid body fat gain, particularly during the first month after initiating cancer treatment, in children with hematologic malignancies. Therefore, individualized dietary strategies to prevent excessive fat gain are needed in pediatric cancer patients for better outcomes.

위암에서 수술 전 혈액학적 검사의 예후인자로서의 가치 (Prognostic Values of Preoperative Hematologic Parameters in Gastric Cancer)

  • 김형주;권성준
    • Journal of Gastric Cancer
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    • 제5권1호
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    • pp.29-33
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    • 2005
  • 목적: 위암 환자에 있어 치료시작 전 환자의 전반적 상태를 평가하기 위해 시행하는 혈액학적 검사 소견이 예후를 예측하는데 유용한가를 알아보고자 한다. 대상 및 방법: 2000년 12월부터 2003년 12월까지의 기간 동안 위암으로 한양대학교병원 외과에서 위절제술을 받은 환자 중 수술 전 혈액학적 검사결과 및 생존여부를 확인할 수 있었던 357명의 환자를 대상으로 하였다. 침윤성 처치가 혈액학적 검사 결과에 미칠 수 있는 영향을 배제하기 위해 외래 및 입원직후 어떤 처치도 시행하기 전에 채취한 혈액만을 이용하였다. 대상환자들의 기록을 통해 혈청 알부민, 혈색소, 혈소판 수, 림프구 수, 병기, 술 후 생존여부 및 생존기간 등을 조사하였다. 혈청 알부민은 3.5 g/dl 이상, 혈색소는 12 g/dl 이상을 정상으로 정의하였고, 혈소판 증가증은 $400\times10^{3}{\mu}l$ 이상이라 정의하였다. 비정상적인 검사 결과가 한 가지라도 있는 경우(제1군), 세 검사 결과 모두 정상인 경우(제2군)등의 두 군으로 나누어 나이, 성별, 종양의 크기, 위치, 병기분포의 차이 및 생존율 차이의 통계적 유의성을 비교, 검토하였다. 결과: 각 군 간에 연령, 종양의 크기 등에서는 통계적으로 유의한 차이가 없었다. TNM 병기가 진행할수록 혈청 알부민과 혈색소가 감소하고 혈소판 수가 증가하였으며 이는 모두 통계적 유의성을 보였다(P=0.000). 병기 분포에 있어서 제 1군에서 제 3군에 비해 진행된 경우가 유의하게 많았다. 종양의 위벽 침윤도 및 림프절 전이에 있어서 제 1군과 제 2군 사이에 유의한 차이가 있었으나(P=0.001, P=0.000) 원격전이의 경우 각 군 사이에 통계적인 차이는 없었다(P=0.125). 3년 생존률에 있어서 제 1군과 제 2군 사이에는 통계적으로 유의한 차이를 보였다(P=0.0375). 다변량 생존분석결과, 혈액학적 검사는 독립적인 예후 관련인자는 아니었다. 결론: 위암 환자에 있어서 술 전 시행한 혈액학적 검사는 비교적 저렴하고 간편한 검사로 환자의 일반적인 상태를 반영하며, 특히 혈소판 증가증, 혈청 알부민 감소, 빈혈 등이 나타난 경우 병의 예후가 나쁠 것임을 예측하는데 유용한 것으로 판단된다.

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종합 건강검진을 원하는 피검진자의 건강특성에 관한 연구 (Characteristics of Individuals Seeking Comprehensive Health Check-ups)

  • 성미혜
    • 대한간호학회지
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    • 제27권3호
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    • pp.563-576
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    • 1997
  • This study was carried out to identify basic data for more efficient operation of comprehensive health check-up centers in the national health care system. The researcher reviewed and analyzed the main symptoms of the subjects according to sex, age, occupation and positive rate in the screening tests of a comprehensive health check-up, performed at one comprehensive health check-up center in located in a hospital. The subjects were 512 persons who had a check-up at the comprehensive health check-up, center in K university hospital in Seoul from October 2, 1996 to March 30, 1997. A questionaire developed by the researcher to obtain subject's general characteristics, main symptoms, medical examination, diagnosed disease was used as the tool for the study. The researcher analyzed the data with SPSS PC/sup +/ program. : the distribution of the subject's general characteristics and main symptoms was described with percentages and the difference between main symptoms and screening tests analyzed with X²-test, t-test, ANOVA. The result of this study are as follows : 1. For the distribution of diagnosis in males. liver disease was most frequent and hypertension was second : in females, hypertention and liver disease were not common in that order. 2. The most common complaints were indigestion in the digestive system, sputum in the respiratory system, irregular heart beat in the cardiovascular system, frequent urine in the urinary system vertigo in the hematologic system. 3. The main symptoms according to sex were for females, a statistically siguificant higher rate of complaints in the digestive system, cardiovascular system, urinary system, hematologic system, musculoskeletal, mental & nervous system as compared to males. 4. The main symptoms according to age were that in the the second decade there wara higher rate of complaints in the digestive system, and cardiovascular system than in the older age groups : and in the fifth decade more in the musculoskeletal system. 5. The main symptoms according to occupation were that formers and fishers had a higher rate of complaints in the mental and nervous system as compared to other occupation groups : and office workers, in gynecology. 6. The main symptoms according to diagnosis, were symptoms of the digestive system, frequent in liver disease : symptoms of the cardiovascular system in allergic disease. 7. The screening tests which showed the highest rates were UGI /FGS, PFT : In males a higher rate of abnormality was seen in abdominal ultrasono- graphy and in, females, in mammography. 8. In screening tests according to sex, males showed a higher positive rate for chest X-rays, and LFT, and females, in mammography, pap smear, CBC. 9. In screening tests according to age, higher positive rates of obesity, high blood pressure, chest X-ray, mammography, pap smear, FBS, CBC, urine test were seen in the fifth decade : PFT and abdominal ultrasonography in the third decade. The subjects who wanted comprehensive health check-up were not asymptomatic but had an illness with several symptoms. It is found that the most frequent diagnosed diseases were hypertention and liver disease. So proper observations must be done for them. Comprehensive health check-ups should be changed to a comprehensive examination including treatment beyond examination for those subjects with positive results.

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Surgical Roles for Spinal Involvement of Hematological Malignancies

  • Kim, Sang-Il;Kim, Young-Hoon;Ha, Kee-Yong;Lee, Jae-Won;Lee, Jin-Woo
    • Journal of Korean Neurosurgical Society
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    • 제60권5호
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    • pp.534-539
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    • 2017
  • Objective : Patients with hematological malignancies frequently encounter spine-related symptoms, which are caused by disease itself or process of treatment. However, there is still lack of knowledge on their epidemiology and clinical courses. The purpose of this article is to review clinical presentations and surgical results for spinal involvement of hematologic malignancies. Methods : From January 2011 to September 2014, 195 patients (98 males and 97 females) suffering from hematological malignancies combined with spinal problems were retrospectively analyzed for clinical and radiological characteristics and their clinical results. Results : The most common diagnosis of hematological malignancy was multiple myeloma (96 patients, 49.7%), followed by chronic myeloid leukemia (30, 15.2%), acute myeloid leukemia (22, 11.2%), and lymphoma (15, 7.56%). The major presenting symptoms were mechanical axial pain (132, 67.7%) resulting from pathologic fractures, and followed by radiating pain (49, 25.1%). Progressive neurologic deficits were noted in 15 patients (7.7%), which revealed as cord compression by epidural mass or compressive myelopathy combined with pathologic fractures. Reconstructive surgery for neurologic compromise was done in 16 patients. Even though surgical intervention was useful for early paralysis (Frankel grade D or E), neurologic recovery was not satisfactory for the progressed paralysis (Frankel grade A or B). Conclusion : Hematological malignancies may cause various spinal problems related to disease progression or consequences of treatments. Conservative and palliative treatments are mainstay for these lesions. However, timely surgical interventions should be considered for the cases of pathologic fractures with progressive neurologic compromise.

Gelatinous Transformation of Bone Marrow Mimicking Malignant Marrow-Replacing Lesion on Magnetic Resonance Imaging in a Patient without Underlying Devastating Disease

  • Lee, Joohee;Yoo, Yeon Hwa;Lee, Sarah;Kim, Hak Sun;Kim, Sungjun
    • Investigative Magnetic Resonance Imaging
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    • 제22권1호
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    • pp.50-55
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    • 2018
  • Gelatinous transformation of bone marrow is characterized by hypoplasia of fat cells with focal loss of hematopoietic cells and deposition of extracellular gelatinous substances. It is known to be associated with devastating underlying diseases that starve bone marrow. Here, we present a case of a patient whose magnetic resonance (MR) imaging findings of vertebral column were interpreted as metastasis or hematologic malignancy, however, the final diagnosis revealed a gelatinous transformation of bone marrow. This is the first report of gelatinous transformation of bone marrow without evidence of underlying devastating disease.

쉐그렌증후군의 임상 증례 (A Case Report of Sjogren Syndrome)

  • JK Choi;SC Yoon;HK Park
    • Journal of Oral Medicine and Pain
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    • 제20권1호
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    • pp.29-37
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    • 1995
  • We diagnosed a patient with chronic dry mouth for 3 years as definite Sjogren's syndrome by the criteria for diagnosis of Sjogren's syndorne proposed by the 1st international seminar on Sjogren's syndrome. The clinical immunologic , hematologic, scintigraphic, and histologic examinations were performed. The patient showed severe xeorstomia, keratoconjunctiva sicca, severe infiltration of lymphocytes in minor salivary glands, and evidences of autoantibodies. The patient's history and clinical findings excluded preexisting lymphoma, graft-versus-host disease, acquired immunodeficiency disease, and sarcoidosis.

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Retrospective study of the medical status of 34 Formosan sika deer (Cervus nippon taiouanu) at the Taipei Zoo from 2003 to 2014

  • Kang, Chu-Lin;Yu, Jane-Fang;Lai, Hsueh;Guo, Jun-Cheng;Wang, Lih-Chiann
    • 대한수의학회지
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    • 제55권3호
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    • pp.169-173
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    • 2015
  • The Formosan sika deer (Cervus nippon taiouanus) is an endemic subspecies in Taiwan. The original wild deer has been extinct since the late 1960s. The largest captive population is located at the Taipei Zoo. Except for infectious disease outbreaks, no systemic medical research has been reported for this subspecies. This study was conducted to analyze the medical status of the captive Formosan sika deer population, including the hematological and serum chemistry characteristics. To accomplish this, medical records for 34 Formosan sika deer from January 2003 to January 2014 were acquired and analyzed. The most common illness and cause of death was trauma, followed by gastrointestinal and respiratory disease, respectively. The hematologic and serum chemical values of healthy adults were quite different from those of sika deer (Cervus nippon yesoensis). This study provides a closer medical understanding of this subspecies and the results will facilitate its management.

Conventional Radiotherapy with Concurrent Weekly Cisplatin in Locally Advanced Head and Neck Cancers of Squamous Cell Origin - a Single Institution Experience

  • Dimri, Kislay;Pandey, Awadhesh Kumar;Trehan, Romeeta;Rai, Bhavana;Kumar, Anup
    • Asian Pacific Journal of Cancer Prevention
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    • 제14권11호
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    • pp.6883-6888
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    • 2013
  • Background: Platinum based concurrent chemo-radiation is the de-facto standard of care in the non-surgical management of locally-advanced head and neck cancer of squamous origin. Three-weekly single agent cisplatin at 100 $mg/m^2$ concurrent with radical radiotherapy has demonstrated consistent improvement in loco-regional control and survival. This improvement is however at the cost of considerable hematologic toxicity and poor overall compliance. The routine use of this regime is improbable in developing countries with limited resources. We therefore aimed to determine the safety and efficacy of an alternative regime of weekly cisplatin and concurrent radiotherapy in such patients. Materials and Methods: January-05 and April-12, 188 patients of locally-advanced head and neck cancer of squamous origin were treated with concurrent weekly-cisplatin at $35mg/m^2$ and conventional radiotherapy 60-66Gy/30-33 fractions/5days per week. Results: Overall, 95% patients received planned doses of RT while 74% completed within the stipulated overall treatment time of <50 days. Eighty-two percent received at-least 5 weekly cycles. Grade-III/IV mucositis was seen in 58%/9% respectively, which resulted in mean weight loss of 9.2% from a pre-treatment mean of 54.5 kg. Grade-III hematologic toxicity-0.5%; grade II nephrotoxicity-2.5% and grade III emesis-3% were also seen. Grade-III/IV subcutaneous toxicity-10%/1% and grade-III/IV xerostomia-10%/0% were observed. Complete responses at the primary site, regional nodes and overall disease were seen in 86%, 89% and 83% patients respectively. The median and 5-years disease-free survival were 26 months and 39.4% respectively, while the median and overall survival were 27 months and 41.8% respectively. Conclusions: Weekly-cisplatin at 35 $mg/m^2$ when delivered concurrently with conventional radical RT (at-least 66y/33 fractions) in locally-advanced head and neck cancer is well tolerated with minimal hematologic and neprologic toxicity and can be routinely delivered on an out-patient basis. It is an effective alternative to the standard 3-weekly cisplatin especially in the context of developing countries.