• 제목/요약/키워드: family disease history

검색결과 355건 처리시간 0.02초

Alport 증후군 환아 12명의 임상적 고찰 (Clinical Observations on 12 Children with Alport Syndrome)

  • 배영민;김성도;강현호;조병수
    • Childhood Kidney Diseases
    • /
    • 제4권1호
    • /
    • pp.48-56
    • /
    • 2000
  • 목 적 : Alport증후군은 유전성 신질환 중 가장 많은 형태로서 흔히 빠른 속도로 진행하는 신질환과 감각신경성 난청, 눈의 이상, 전자현미경상 특징적 소견, 그리고 대부분의 경우 가족력 동반을 특징으로 한다. 저자들은 Alport증후군의 임상적 특징을 관찰하여 진단에 도움이 되고자 후향적 조사를 실시하였다. 대상 및 방법 : 1991년 4월부터 1999년 6월까지 경희의대 부속병원 소아과에서 Alport증후군으로 진단된 환아 12명을 대상으로 하였으며 진단 기준으로 신질환, 가족력, 감각신경성 난청이나 눈의 이상 및 전자현미경상 특징 등 4가지를 설정하여 의무기록을 통하여 비교분석하였다. 결 과 : 12예중 설정된 4가지의 진단 기준에 모두 부합되는 경우는 2예였으며 가족력이 확실치 않았던 예가 3예, 감각신경성 난청이나 눈의 이상 소견이 없었던 예가 6예였다. 또한 1예에서는 만성신부전에 빠져 신생검을 시행치 못하였으나 나머지 진단 기준을 충족시켰다. 12예 중 남녀의 비는 1:2였으며 신질환 발견시의 연령은 평균 5.6세 였다. 처음 나타난 증상으로 신질환이 먼저 나타난 예는 10예 였으며 나머지 2예에서는 청각장애와 백내장이 각각 처음 증상으로 나타났다. 12예 중 신질환의 가족력을 동반한 경우는 9예였으며 11예에서 청력검사와 안과적검사를 시행하여 6예의 감각신경성난청과 2예의 눈의 이상을 발견하였다. 전자현미경 소견은 11예중 9예에서 사구체기저막의 두께가 불규칙하게 두꺼워지고 고밀도층의 박층화와 족돌기 융합의 소견을 보였고 나머지 2예에서는 기저막이 얇아져있고 박층화와 족돌기 융합 소견을 보였다. 평균추적기간은 3년 6개월로 현재 만성신부전에 이른 경우는 1예 있어 신이식을 시행하였다. 결 론 : Alport증후군의 진단에 신질환의 가족력, 감각신경성 난청, 눈의 이상 및 전자현미경상 특징적 소견은 중요하며 이러한 특성을 분석함으로써 앞으로 많은 환자의 발견에 도움이 되리라 생각된다. 알 수 있었다 (P<0.05). 결 론 : 저자의 경우 전체 환아 중 65$\%$만이 완전 회복을 보였고 35$\%$에서 사망이나 말기 신부전으로 이행하여, 병의 경과를 좋게 하고 사망률을 줄이는데 조기 진단과 조기 투석을 시행하는 것이 중요한 역할을 하는 것으로 알 수 있었다. 내원하여 시행한 검사 소견상 혈색소치가 불량한 예후를 보인 환자군에서 유의하게 감소하여, 낮은 혈색소치가 불량한 예후인자로서의 역할을 할 것으로 사료된다. 용혈성 요독 증후군은 아직 국내에서 드문 질환중의 하나이지만, 소아 영역에 있어서 급성 신부전증의 가장 흔한 원인중의 하나로 지속적인 연구가 필요할 것으로 사료된다.므로 임상적 위험인자로 자반의 지속기간과 함께 HS자반증 환아의 신침범에 대한 예측인자로 활용할 수 있을 것이며 따라서 HS신염의 예방과 치료에 있어서 TNF-$\alpha$에 대한 생성억제제나 특이항체 등의 이용가능성에 대한 연구가 필요할 것이다.야간 수분 섭취 제한의 중요성이 강조되어야 한다고 생각된다.임상상으로는 추정할 수 없었으며 그러므로 환아에 대한 장기적인 관리 계획을 수립하기 위해서는 신생검을 시행하여 정확한 조직학적 병변을 확인함이 필요하다고 생각된다.89$개월, FSGS군 $5.27{\pm}12.48$개월로 각 군간의 의미있는 차이는 없었다. 결 론 : 미세변화형 신증후군에서 치료 경과중 국소성 분절성 사구체 경화증으로 이행된 예는 전체 MCNS의 249명중 8명으로 $3.2\%$였다. 이들의 임상양상의 특징을 비교 고찰한 결과 이행여부를 예측할 수 있는 위험요인은 없었다. 미세변화형 신증후군으로 진단된 환아 중에서 스테로이드 치료에 반응이 없거나 자주 재발하는 경우, 또는 강력한 면역치료에도 관해가 오지 않는 경우 등에는 신장 조직 생검을 재시행하여 국소성 분절성 사구체 경화증으로의 이행 여부를

  • PDF

가족발생(家族發生) 양측성(兩側性) 성인형(成人型) 다낭종신(多囊腫腎) 3례(例)의 초음파진단(超音波診斷) - 보험가입자(保險加入者)를 대상(對象)으로 - (A Ultrasonic Diagnosis of Family Incidence Bilateral Adult Type Polycystic Kidney: Three Cases)

  • 문수형;한혜진;김강석
    • 보험의학회지
    • /
    • 제2권1호
    • /
    • pp.218-232
    • /
    • 1985
  • Congenital hereditary disease is in devided into Infantile type and Adult type, Adult type is hidden for many years and keeps normal renal function till middle age. Cyst is stimultaneously made in both sides and becomes lowered in renal function in 30's to 40's. Infantile type is generally born with the big kidneys, renal failure, undergrowth of intrahepatic bile duct. Both infantile and childhood type have ureteral dilatation and portal hypertension In infantile type, it is mostly developed into renal failure, but generally faces death as a result of hepatic disease. The reason of death is that an abnormal condition of recessive autosome affects the liver and kidneys. While the incidence of infantile type is rare as $0.017{\sim}0.07%$ and it is autosomal recessive heredity, Adult type can rarely exist in infantile period. Though it exists in middle period, 50% of patients can live for 2-4 years after the first symptom incidence and 25% can less than 2 years. It is hard to cure completely in medicine and surgery. Three difficulties in familial incidence are comparative decrease of the donor who have no affection on renal transplantation. For another consideration it is to show the family history for several generations. We, the Med. Dept. of Dae Han Kyouk Life Insurance Co. Ltd., used the ultrasonic apparatus in diagnosing the one case of adult type bilateral polycystic kidney and then doubted the family history. As a result of inspecting the family we experienced bilateral polycystic kidney from 3 persons out of 4 who can be inspected. The results are as follows: 1) We could confirm the polycystic kidney from 3 persons out of 4(75%). 2) Then when they came for check up, chief complaint was the pain in all 3 cases(100%). 3) Accompanying disease was hypertension in 2 cases(67%). 4) In early disease incidence, we couldn't observe the specific change in pathological opinion. 5) All 3 cases are not accompanied with cystic lesion in liver, spleen, pancreas.

  • PDF

Long-term Prognosis of thin Glomerular Basement Membrane Nephropathy in Children: A Retrospective Single Center Study

  • Lim, Myung Hee;Bae, Hee Jung;Jang, Kyung Mi;Park, Yong Hoon
    • Childhood Kidney Diseases
    • /
    • 제21권2호
    • /
    • pp.41-46
    • /
    • 2017
  • Purpose: Thin glomerular basement membrane nephropathy (TBMN) is, along with the IgA nephropathy, the most common cause of asymptomatic hematuria in Korean children. TBMN is usually a benign renal disease not requiring treatment and is associated with a good prognosis, but some cases hematuria is indicative of a state of progressive renal insufficiency. We aimed to retrospectively evaluate clinical manifestations and renal prognosis of patients with TBMN. Methods: Among the 428 renal biopsies performed on children at Yeungnam University Hospital between January 2000 and February 2017, 167 patients were diagnosed as having TBMN. We retrospectively investigated 167 pediatric patients and identified 59 children with follow-up duration >3 years. Results: Among 59 patients, there were 33 boys and 26 girls. Mean age of onset of hematuria was $7.18{\pm}2.64$ years, and mean time from onset of disease until a renal biopsy was performed was $2.48{\pm}2.10$ years. There were no clinical features or laboratory findings among studied children to indicate decreased renal function during follow-up; however, one case progressed to chronic kidney disease (CKD) due to an unknown cause. There were seven patients among these related a positive family history of hematuria or renal insufficiency. Concluson: Although almost all patients had normal renal functions during follow-up, there were one patient who progressed to CKD and seven patients with family history of hematuria or renal insufficiency. Moreover, four among the 428 patients over 17 years underwent repeat renal biopsies, which showed results different from their earlier biopsies.Thus, large-scales studies may be required to determine long-term prognosis of TBMN in children, and further evaluation for Alport syndrome in TBMN cases is essential.

지루성 피부염 환자 140례의 임상 특성 연구 (A Clinical Analysis of 140 cases of Seborrheic Dermatitis Patients)

  • 신윤진;이종우
    • 한방안이비인후피부과학회지
    • /
    • 제31권1호
    • /
    • pp.81-90
    • /
    • 2018
  • Objectives : This study was performed to investigate the characteristic of Seborrheic Dermatitis(SD) patients who visited Korean medicine clinics. Methods : The study was carried out with 140 SD outpatients who visited fourteen Gowoongyul Korean traditional medical clinics from January to December 2016 and was completed by reviewing patients' survey. Results : 1. Among 140 patients, there were 61 male patients(43.6%), 79 female patients(56.4%). The average age of the patients was $32.6{\pm}10.9years$ old, with many in their 20s and 30s in the distribution. 2. The average duration of disease was $4.2{\pm}4.11years$. 3. 80% of the patients had experience of treatment, 58.6% only took Western medicine, 17.1% had experience of both Western and Korean medicine treatment, and 4.3% received only Korean medicine treatment. 4. Analysis of seasonal effects of SD showed that 48.6% of the respondents reported that skin disease always appeared regardless of the season, with 33.6% in winter, with 29.3% in summer and 25.7% in seasonal change. 5. The rate of family history of seborrheic dermatitis was low(6.4%), and 75.7% of the patients answered that they had no family history of skin disease. 6. The major lesion of SD appeared on face(87.9%) and scalp(36.4%). 7. 70.7% answered they do some kind of self-managements. 70.0% used moisturizers. Bathing therapy was performed in 17.1%, and 55% were taking the health supplement food, and 35.7% restricted the avoidance diet such as instant and fatty foods. Conclusions : This study demonstrated the various clinical characteristics of Korean patients with seborrheic dermatitis.

Distinctive Features of Hepatic Steatosis in Children: Is It Primary or Secondary to Inborn Errors of Metabolism?

  • Karhan, Asuman Nur;Hizarcioglu-Gulsen, Hayriye;Gumus, Ersin;Akcoren, Zuhal;Demir, Hulya;Saltik-Temizel, Inci Nur;Orhan, Diclehan;Ozen, Hasan
    • Pediatric Gastroenterology, Hepatology & Nutrition
    • /
    • 제24권6호
    • /
    • pp.518-527
    • /
    • 2021
  • Purpose: The incidence of hepatic steatosis among children has been increasing; however, data distinguishing simple steatosis from a more complex disorder are lacking. Methods: This study identified the etiologies resulting in hepatic steatosis through a retrospective review of pediatric liver biopsies performed in the last 10 years. A total of 158 patients with hepatic steatosis proven by histopathological evaluation were enrolled in the study, and baseline demographic features, anthropometric measurements, physical examination findings, laboratory data, ultrasonographic findings, and liver histopathologies were noted. Results: The two most common diagnoses were inborn errors of metabolism (IEM) (52.5%) and nonalcoholic fatty liver disease/steatohepatitis (NAFLD/NASH) (29.7%). The three most common diseases in the IEM group were glycogen storage disorders, Wilson's disease, and mitochondrial disease. The rates of consanguineous marriage (75.6%; odds ratio [OR], 26.040) and positive family history (26.5%; OR, 8.115) were significantly higher (p=0.002, p<0.001, respectively) in the IEM group than those in the NAFLD/NASH group. Younger age (p=0.001), normal anthropometric measurements (p=0.03), increased aspartate aminotransferase levels (p<0.001), triglyceride levels (p=0.001), and cholestatic biochemical parameters with disrupted liver function tests, as well as severe liver destruction of hepatic architecture, cholestasis, fibrosis, and nodule formation, were also common in the IEM group. Conclusion: Parents with consanguinity and positive family history, together with clinical and biochemical findings, may provide a high index of suspicion for IEM to distinguish primary steatosis from the consequence of a more complex disorder.

영유아 아토피피부염 환자 69명의 한의학적 치료효과에 대한 후향적 연구 (Retrospective Study about the Effectiveness of a Korean Medicine Treatment on 69 Infant and Young Child Atopic Dermatitis Patients)

  • 민들레;한명화;박건;서산;한수련
    • 대한한방소아과학회지
    • /
    • 제28권3호
    • /
    • pp.17-30
    • /
    • 2014
  • Objectives The purpose of this study is to report the effectiveness of Korean medicine treatment on 69 infant and young child atopic dermatitis patients. Methods 69 infants and young child atopic dermatitis patients who had visited oriental medical clinics from 2011.1.9 to 2012.1.31 were studied. All of them were continually treated for 3 to 9 months, and were analyzed by Objective SCORAD Index (OSI). A detailed analysis of OSI was done according to gender, age, using of topical steroid ointment, family history of atopic disease, treatment period, and intensity. Results 1. Male and female percentage of the study group was 56.5%: 43.5%. The average period of treatment was 6.09 months. 71.0% used topical steroid ointment, and 65.2% had family history of atopic disease. 2. The average period of treatment was significantly longer in patients who used topical steroid ointment, or who had family history of atopic disease. 3. 95.7% of total patients reported decrease in OSI score at the final visit. OSI was significantly lowered after 3, 6, and 9 months of treatment. The longer period of treatment, the lower average post-treatment OSI. 4. 85.5% of the study group had severe dermatitis, and 14.5% was moderate case based on the OSI intensity assessment at initial visit. These percentages got changed at the final visit as 34.8% of severe, 58.0% of moderate, and 7.2% of mild cases. The average treatment period of patients who were diagnosed as severe at the initial visit was 6.18 months, and their final OSI improvement rate was 39.58%. 58% of them were improved to be mild or moderate at the final visit. Conclusion The Korean medicine treatment is effective in treating infant and young child atopic dermatitis patients. There was significant decrease in OSI score after 3, 6, and 9 months of treatment. The difference increase with the treatment period.

사회경제적 요인에 따른 지역사회 저소득층 중년여성의 심혈관질환 위험요인 차이 (Cardiovascular Disease Risk according to Socioeconomic Factors among Low-income Midlife Women)

  • 함옥경;김봉정;이영아
    • 한국보건간호학회지
    • /
    • 제22권1호
    • /
    • pp.27-38
    • /
    • 2008
  • Purpose: To determine the degree of cardiovascular disease risk according to socioeconomic factors among midlife women in the community and thereby provide baseline data for the development of health promotion programs. Method: A total of 200 women participated in health screenings and a health survey. The survey was performed in November and December, 2006. The survey instruments included socioeconomic factors, health behavior (smoking and exercise), and family history. Biophysical measurement included BMI and blood pressure. Blood samples were drawn for glucose and total cholesterol tests. Results: The mean age was 52.5 years, 34.0% had received education less than 6 years, 70.0% earned a monthly income of less than \1,500,000, and 61.5% were homemakers. Cardiovascular disease risk was significantly different by age (BMI, systolic BP, and exercise), education (systolic BP), monthly income (T. cholesterol), marital status (smoking), and occupation (exercise). Most women had 2 or 3 cardiovascular disease risks. Older age, lower education, and lower income were significantly associated with increased cardiovascular disease risk. Conclusion: Efforts should be made to decrease the number and severity of cardiovascular disease risk factors for midlife women in the community by developing health promotion programs targeting to modify their cardiovascular disease risk factors.

  • PDF

Clinicopathological Features and Survival of Patients with Gastric Cancer with a Family History: a Large Analysis of 2,736 Patients with Gastric Cancer

  • Jeong, Oh;Jung, Mi Ran;Park, Young Kyu;Ryu, Seong Yeob
    • Journal of Gastric Cancer
    • /
    • 제17권2호
    • /
    • pp.162-172
    • /
    • 2017
  • Purpose: Previous studies indicated conflicting results regarding the prognosis of gastric cancer with a family history (FHX). This study aimed to determine the clinicopathological features and survival of patients with gastric cancer with a FHX. Materials and Methods: We reviewed 2,736 patients with gastric cancer who underwent surgery between 2003 and 2009. The prognostic value of a FHX was determined in the multivariate model after adjusting for variables in the Asian and internationally validated prognostic models. Results: Of the patients, 413 (15.1%) had a FHX of gastric cancer. The patients with a FHX were younger (58.1 vs. 60.4 years; P<0.001) than the patients without a FHX. There were no significant differences in the histopathological characteristics between the 2 groups. A FHX was associated with a better overall survival (OS) rate only in the stage I group (5-year survival rate, 95% vs. 92%; P=0.006). However, the disease-specific survival (DSS) rate was not significantly different between the 2 groups in all stages. The multivariate model adjusted for the variables in the Asian and internationally validated prognostic models revealed that FHX has no significant prognostic value for OS and DSS. Conclusions: The clinicopathological features and survival of the patients with gastric cancer with a FHX did not significantly differ from those of the patients without a FHX.

19세기 조선 의약 풍경과 '약로(藥露)' - 신대우 가계 기록물과 서유구, 이규경의 저술을 중심으로 (An Aspect of Medical Life and Yakro in Joseon in the 19th Century Based on the Records of Sin Dae-woo's Family, Seo Yugu, and Lee Kyugyung)

  • 전종욱
    • 한국의사학회지
    • /
    • 제35권1호
    • /
    • pp.135-147
    • /
    • 2022
  • Sin Dae-woo was a scholar representing the Ganghwa School in the late Joseon Dynasty. He was Jeong Je-du's grandson-in-law and also in charge of organizing Jeong Je-du's collection of writings. His three sons had a particularly close relationship with their father, so even when they published a collection of writings after his father's death, the names of the three were combined and marked as Jin-jak-shin. The records they left include Seokcheon Il-seung, who described the history of the family along with the collection of writings, Seongdo Il-rok, a diary of the time of Seongcheon Busa, and many letters exchanged over time. These records draw attention as they contain records of medical life, such as eye diseases that the family suffered, infectious diseases that caused many casualties, and Yakro (distilled herbal essence) that showed records of being used for treatment at the time. In this paper: 1) We examine the medicines, prescriptions, acupuncture, and medication used to treat eye diseases. 2) We carefully examine the epidemic, the responses of the parties to it, and the attitudes of those who stood at the boundary between death and life. 3) We look for clues to the use of Western-origin drugs called Yakro. Intellectuals of the time looked deeper into Lee Kyugyung's book and Seo Yu-gu's book on how they were reflected in Joseon's medical life. In conclusion, in the 18th and 9th centuries, we see that the influential families of the Ganghwa school freely brought in famous acupuncturists, used prescriptions that were included in medical books or not, and used Yakro from western origin that were not traditional methods of Joseon. Thus, we reveal that doctors of the Joseon had the capacity to pursue their medical life more actively and had open-minded exchanges than our existing perceptions.

포스트구조주의적 분석을 이용한 간호와 보건의료의 역사에 대한 재해석의 한 시도 (An Attempt of Reinterprtation on History of Nursing and Health Care Using Post-structural Method)

  • 김남선
    • 대한간호학회지
    • /
    • 제27권3호
    • /
    • pp.531-540
    • /
    • 1997
  • The purpose of this study is to reinterpretate the history of nursing and health care from the view-point of post-structuralism. It has been emphasized that the development of modern health care has been due to the progressive efforts of medicine and to medical discoveries. Medicine has dominated the history of health care rather than nursing or other health professions. The present study adopts the post-structural method by Foucault, which tries to unite language and knowledge. Foucault examines "the institutionalization of knowledge and the power exerted thereby, with special reference to the devices of social regulation and their function over the madness, the disease, the crime, and the sexuality. " The concept of power in Foucault's writing is that it is exerted spontaneously in verbal behaviors of individuals through knowledge of everyday life such as definition of body or mind, sexuality and relationship of family. Therefore as to the problem of power, this study tries to understand the meaning of the health care history through an analysis of the formation of medical discourse. In order to have authority in a power relation, the medical professional asserts that medical discourse is the most scientific knowledge. The authority of medical professionals can be reinforced by the fact that male medical professionals outnumber female. Devaluation of nursing care is reinforced by the medicine which has the legitimate authority through use of the political skills.

  • PDF