• Title/Summary/Keyword: Hematologic diseases

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Klinefelter Syndrome: Review of the Literature

  • Jun, Kyung Ran
    • Journal of Interdisciplinary Genomics
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    • v.4 no.2
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    • pp.24-30
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    • 2022
  • Klinefelter's syndrome (KS) is a syndrome with extra X chromosome(s), in XY individuals, characterized by gynecomastia, small testes, and infertility. Additional X chromosomes can be present as variable karyotypic forms, including mosaicism (47,XXY/46,XY). The reported prevalence of KS ranges from one in 500 to one in 1,000 live males, but is probably underestimated. The classic phenotype is small, firm testes and infertility resulting from seminiferous tubule dysgenesis and androgen deficiency. The spectrum of KS includes tall stature with relatively long legs and arm span, decreased body hair, learning disabilities, behavioral problems, poor motor skills, and other important medical issues, such as metabolic syndrome, diabetes, autoimmune diseases, cardiovascular disease, certain neoplasia. The increased risk of certain medical problems in KS can be attributed to a direct effect of the extra X chromosome, the combined action of multiple genomic and epigenetic factors, or the hormonal imbalances. Typically, chromosome analysis is not ordered for adult patients with general medical conditions, except for suspected cases of hematologic and lymphoid disorders. Even though it was found during work-up for certain disorders in adult patient, most physicians do not suspect KS or consider its impact. Therefore, understanding the pathophysiology and variable manifestation in KS is necessary, and discussions with multidisciplinary teams will help to diagnose and treat males with KS.

A Case of Typhlitis Developed after Chemotherapy with Irinotecan and Cisplatin in a Patient with Small Cell Lung Carcinoma

  • Ji, Eun Hye;Kim, Young Min;Kim, Soo Jeong;Yeom, Soo Jeong;Ha, Sung Eun;Kang, Hyeon Hui;Kang, Ji Young;Lee, Sang Haak;Moon, Hwa Sik
    • Tuberculosis and Respiratory Diseases
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    • v.73 no.5
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    • pp.288-291
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    • 2012
  • Typhlitis is a necrotizing colitis that usually occurs in neutropenic patients and develops most often in patients with hematologic malignancies such as leukemia and lymphoma. Typhlitis may proceed to bowel perforation, peritonitis and sepsis, which requires immediate treatment. Irinotecan is a semisynthetic analogue of the natural alkaloid camptothecin which prevents DNA from unwinding by inhibition of topoisomerase I. It is mainly used in colon cancer and small cell lung carcinoma (SCLC), of which the most common adverse effects are gastrointestinal toxicities. To the best of our knowledge, no case of typhlitis after chemotherapy with a standard dose of irinotecan in a solid tumor has been reported in the literature. We, herein, report the first case of typhlitis developed after chemotherapy combining irinotecan and cisplatin in a patient with SCLC.

Efficacy and Safety of Pemetrexed in Advanced Non-Small Cell Lung Carcinoma (진행성 비소세포폐암 환자에서 Pemetrexed의 효과와 안전성)

  • Lee, Gyu Jin;Jung, Mann Hong;Jang, Tae Won;Ok, Chul Ho;Jung, Hyun Joo
    • Tuberculosis and Respiratory Diseases
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    • v.67 no.2
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    • pp.121-126
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    • 2009
  • Background: Pemetrexed has been prescribed newly as a second line chemotherapy in advanced non-small cell lung carcinoma (NSCLC). The aim of study was to determine the efficacy and toxicity of pemetrexed in advanced NSCLC. Methods: Patients with histologically or cytologically confirmed NSCLC were evaluated from June 2006 to December 2008. The patients had relapsed or progressed after prior chemotherapy treatment. They were treated with intravenous pemetrexed $500mg/m^2$ for 10 min on Day 1 of each 21-day cycle. Results: A total of 89 patients were eligible for analysis. The response rate and disease control rate were 11% and 66%. Non-squamous cell carcinoma histology was significantly associated with a superior response rate (p=0.035) and disease control rate (p=0.009) than squamous cell carcinoma histology. The median survival time was 13 months and the median progression free survival time was 2.3 months. The median survival time of patients with ECOG PS 0~1 was 13.2 months, whereas median survival time was 11.6 months for patients with PS 2 (p=0.002). The median progression free survival time of patients with PS 0~1 were 3.8 months, but 2.1 months for patients with PS 2 (p=0.016). The median progression free survival time of smokers with non-squamous cell carcinoma was 3.4 months, which was significant (p=0.014). Grade 3~4 neutropenia were seen in 7.9% patients. Conclusion: Pemetrexed has efficacy in patients who had prior chemotherapy with advanced NSCLC and less hematologic toxicity.

Pulmonary Mucormycosis with an Appearance of Consolidation (경화 양상으로 나타난 폐의 Mucormycosis)

  • Kim, Shin-Chae;Lee, Heung-Bum;Lee, Yong-Chul;Rhee, Yang-Keun
    • Tuberculosis and Respiratory Diseases
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    • v.49 no.1
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    • pp.117-121
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    • 2000
  • The mucormycosis is a group of serious opportunistic infections caused by fungi of the class Zygomycetes and order Mucorales. Pulmonary mucormycosis is a relatively rare disease but typically manifested by a rapidly progressive, often fatal pneumonia in patients with diabetes mellitus, hematologic malignant neoplasms, or organ transplants. The radiologic manifestations of pulmonary mucormycosis are nonspecific and include progressive lobar or multilobar consolidations, pulmonary masses and pulmonary nodules. Recently, we experienced a pulmonary mucormycosis in 32-year-old man with uncontrolled diabetes. He complained of cough, left pleuritic chest pain and generalized weakness. Initial chest X-ray finding was the consolidation on the lower lobe of the left lung. On the sixth hospital day, bronchoscopic examination with lung biopsy revealed broad, non-septate hyphae with right-angle branching, diagnostic of mucormycosis, and consequently amphotericin B therapy was started. We performed a lobectomy of the left lower lobe of the lung on 29th hospital day.

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A Case of Gold Induced Hypersensitivity Pneumonitis Diagnosed by Lymphocyte Stimulation Test with Gold (림프구 자극 시험으로 확진된 금제에 의한 과민성폐렴 1예)

  • Yum, Ho-Kee;Han, Seong-Hoon;Kim, Hyung-Gon;Lee, Hye-Kyung;Jeon, Woo-Ki;Lee, Yun-Woo;Kim, Dong-Soon
    • Tuberculosis and Respiratory Diseases
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    • v.41 no.5
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    • pp.546-551
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    • 1994
  • Gold therapy of rheumatoid arthritis is effective, but adverse reactions are also common. There are some cutaneous, hematologic, renal, and pulmonary side effects which have been associated with immunologic abnormalities. Also, hypersensitivity pneumonitis induced by gold therapy was rarely reported in literature. But, the mechanism for the induction of the pulmonary disease is not known. We report a case of seropositive rheumatoid arthritis presented as gold induced hypersensitivity pneumonitis which was confirmed by the peripheral blood lymphocyte stimulation test with gold.

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The Changes of Serologic Markers in Pneumoconiosis of Coal Workers (진폐증 환자에서의 혈청학적 표지자의 변화)

  • Yoo, Kwang-Ha;Yun, Ho-Sang;Lee, Sang-Yeup;Jin, Choon-Jo;Ahn, Cheol-Min;Kim, Hyung-Joong
    • Tuberculosis and Respiratory Diseases
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    • v.50 no.5
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    • pp.615-623
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    • 2001
  • Background : Pneumoconiosis is a parenchymal lung disease that results from the accumulation of coal dust in the lungs and the consequent tissue reaction. To evaluate the role of various personal factors in pneumoconiosis and the significance of some serologic markers for assessing the disease activity related to pneumoconiosis, the Rheumatoid Factor(RF), ${\alpha}_1$-AT, C-Reactive Protein(CRP), ceruloplasmin and fibrinogen levels were measured. Method : All the patients were males, 45-76 years old, and the mean duration of coal dust exposure was 23.2 years. 51 patients were classified as having Simple Pneumoconiosis (SP), 59 had Progressive Massive Fibrosis (PMF). Fifty eight men with ages ranging from 26-70 years were used as normal controls. The serum RF and CRP were titrated using an Autochemistry analyzer (HITACHI 7150 : Japan) and the ${\alpha}_1$-AT and ceruloplasmin levels were measured using a Nephelometer (Behring Nephelometer : Germany) and the fibrinogen levels were estimated by using an Autoanalyzer for hematologic coagulation. Result : There was a higher RF level in the SP, and PMF groups than in the control groups but there was no statistical difference. The CRP, ${\alpha}_1$-AT, and ceruloplasmin levels were also higher in the SP, and PMF groups. However, the fibrinogen concentration was within the normal ranges in both the SP and PMF groups. Conclusion : The CWP (Ed note : Define CWP) patients had significantly higher CRP, ${\alpha}_1$-AT, and ceruloplasmin levels compared to the control group. It is believed that these serologic changes could be used as a marker of the disease activity.

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A Case of Isoniazid Induced Acute Pancreatitis (Isoniazid에 의해 유발된 급성 췌장염 1예)

  • Chung, Byung Ha;Nam, Hae Seong;Kwon, Jung Hyun;Im, So Hi;Park, Sun Hee;Kim, Jin Woo;Kim, Seung Joon;Lee, Sook Young;Kim, Young Kyoon;Park, Sung Hak
    • Tuberculosis and Respiratory Diseases
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    • v.56 no.4
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    • pp.411-414
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    • 2004
  • Isoniazid is a first-line drug in the treatment of tuberculosis. A variety of adverse reactions of isoniazid have been reported. These include hepatitis, peripheral neuropathy, skin rashes, neurologic disturbances and hematologic alterations. Among these, acute pancreatitis due to isoniazid is very rare. We report a case of acute pancreatitis due to isoniazid confirmed by rechallenge test with review of some literatures.

A Case Report on Oro-Facial Manifestations in Leukemia (Oral Chronic GVHD) (백혈병 환자의 구강악안면 증상 발현에 관한 증례보고 (Oral Chronic GVHD))

  • Ahn, Hyoung-Joon;Kwon, Byung-Ki;Shin, Kyoung-Jin;Choi, Jong-Hoon;Kim, Chong-Youl
    • Journal of Oral Medicine and Pain
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    • v.25 no.2
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    • pp.159-165
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    • 2000
  • Subsequent to an allogenic stem cell transplantation(ASCT) on patients with hematologic malignancy(AML, ALL, CML, multiple myeloma, lymphoma etc.), chronic GVHD(graft versus host disease), which is an immunological reaction, occurs. With treatment results from patients who were diagnosed with ALL(acute lymphocytic leukemia), undergone BMT(bone marrow transplantation) and showed oral and skin lesions due to GVHD, treatment of oral manifestations of leukemia and its general management were studied. 90% of patients with chronic GVHD show change in the oral mucosa causing oral manifestations such as leukoplakia, lichenoid change of the oral mucosa, mucosal atrophy, erythema, ulceration and xerostomia. In treating GVHD, extensive systemic immunosuppression cause bacterial, viral, fungal infection that are fatal, and even if the treatment is successful, the patient is already in a severe immunosuppressed state. Therefore, localized target therapy is preferred. In another words, topical application(rinse, cream, ointment etc.) of cyclosporin and steroid in treating oral chronic GVHD is highly recommended, and the use of PUVA(Psoralen Ultraviolet A) and thalidomide is reported to be effective. In treating such diseases, dental treatment to control pain and prevent secondary infection of oral manifestations is very important. To those patients with systemic diseases who show limited effect by general dental treatment, non-invasive treatment such as the dental laser, in addition to the use of drugs, may be necessary to actively treat pain and help the healing process. For greater results, new effective methods are to be developed for treatment.

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The Correlation of Sonographic Finding of Fatty Liver with Hematologic Examination and Body Fat Percentage (초음파상 지방간과 혈액학적 검사 및 체지방률과의 상관관계)

  • Cheon, Hae-Kyung;Lee, Tae-Yong;Kim, Young-Ran
    • Journal of radiological science and technology
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    • v.32 no.4
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    • pp.437-444
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    • 2009
  • Ultrasonography has been used as a basic examination of a medical check up for prevention and diagnostics of diseases. Even the person who has no particular subjective symptoms can have a variety of diseases. Especially fatty liver is found in many cases. In this study, we tested 3582 persons who are in between the ages of 15 to 81 and observed that 1390 persons had fatty liver while 2192 persons are normal. We classified the grade of fatty liver and compared their life styles with the results of liver function test and BMI. The results are as follows. Ratio of the subjects who had a fatty liver is 38.8%. Male and female ratio was 46.2% and 24.2%. On the correlation among the fatty liver, the body mass index and the body fat%, the average value of body mass index and body fat% were significantly higher in the group of the fatty liver than in those of the normal liver. The influence of the related factor and the correlation on the fatty liver was shown that it was more related with the order of age, body mass index, triglyceride, ALT, body fat%, sex, HDL-Cholesterol, LDL-Cholesterol, and GGT. The result of the ultrasonography carried out for the purpose of regular health check up indicates that even the 38.8% of those who was diagnosed as normal condition could have the fatty liver and have possibility of other diseases. Therefore, if there are any troubles related to liver function and lipid through hematologic examination or when practicing follow-up study with ultrasonography concerning the correlation relation between the body fat% and dietary preference, alcohol consumption and exercise, the ultrasonography is definitely useful for prevention and treatment of diseases.

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

  • 성미혜
    • Journal of Korean Academy of Nursing
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    • v.27 no.3
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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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