• Title/Summary/Keyword: jaundice

Search Result 399, Processing Time 0.029 seconds

Alagille syndrome and a JAG1 mutation: 41 cases of experience at a single center

  • Ahn, Kyung Jin;Yoon, Ja Kyoung;Kim, Gi Beom;Kwon, Bo Sang;Go, Jung Min;Moon, Jin Su;Bae, Eun Jung;Noh, Chung Il
    • Clinical and Experimental Pediatrics
    • /
    • v.58 no.10
    • /
    • pp.392-397
    • /
    • 2015
  • Purpose: Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis. Methods: We conducted a retrospective study of 41 patients with Alagille syndrome or a JAG1 mutation between 1983 and 2013. Results: The first presentations were jaundice, murmur, cyanosis, and small bowel obstruction at a median age of 1.0 months (range, 0-24 months). The JAG1 mutation was found in 27 of the 28 genetically-tested patients. Cardiovascular anomalies were identified in 36 patients, chronic cholestasis was identified in 34, and liver transplantation was performed in 9. There was no significant correlation between the severity of the liver and cardiac diseases. The most common cardiovascular anomaly was peripheral pulmonary stenosis (83.3%), with 13 patients having significant hemodynamic derangement and 12 undergoing surgical repair. A total bilirubin level of >15 mg/dL with a complex surgical procedure increased the surgical mortality (P=0.022). Eight patients died after a median period of 2.67 years (range, 0.33-15 years). The groups with fetal presentation and with combined severe liver and heart disease had the poorest survival (P<0.001). Conclusion: The group with combined severe liver and heart disease had the poorest survival, and a multidisciplinary approach is necessary to improve the outcome.

Intelligent Diagnosing Method Based on the Conditional Probability for the Pancreatic Cancer Early Detection (췌장암 조기진단을 위한 조건부 확률 기반 지능형 진단 방식)

  • JANG, IK GYU;JUNG, JOONHO;KO, JAE HO;MOON, HYUN SEOK;JO, YUNG HO
    • Journal of Biomedical Engineering Research
    • /
    • v.38 no.5
    • /
    • pp.227-231
    • /
    • 2017
  • Early diagnosis of pancreatic cancer had been considered one of the important barrier for successful therapy since the five year survival rate after treatment of pancreatic cancer was critically low. Nonetheless, patients often miss the golden time of treatment because they rarely visit the hospital until their symptoms are severe. To overcome these problems, a lot of information about the patient's symptoms should be applied as biomarkers for early diagnosis. For this reason, a biomarker for early detection of pancreatic cancer (CA19-9) has been developed as a diagnostic kit. However, since the diagnosis is not accurate enough, pancreatic symptoms (abdominal pain, jaundice, anorexia, diabetes, etc.) and biomarkers (CA19-9) should be considered together. We develop an intelligent diagnostic system that considers CA19-9 and the incidence of pancreatic cancer for pancreatic symptoms that was determined by studying a large number of patient information. It shows a higher accuracy than one using CA19-9 alone. It may increase the survival rate of pancreatic cancer because it can diagnose pancreatic cancer early.

A comparative study of Curcuma longa L. and Curcuma aromatica S. in medical texts (강황(薑黃)과 울김(鬱金)의 역대문헌(歷代文獻)에 대(對)한 비교(比較) 연구(硏究))

  • Kim, Yong-Ryul;Lee, Hyun-Jeong;Jeong, Hyun-Jong;Keum, Kyung-Soo
    • Journal of the Korean Institute of Oriental Medical Informatics
    • /
    • v.17 no.1
    • /
    • pp.203-255
    • /
    • 2011
  • This study covers the historical aspects of the turmeric and curcuma only in detail on the medicinal uses, supported by references to the medical texts. And the result is as follows: 1. Turmeric and curcuma are rhizomatous herbaceous perennial plants of the ginger family, but both produced on the same herb. The rhizome is considered turmeric while the tuber is considered curcuma. 2. Turmeric is the round, oval, or ovate, and scutiform rhizome. 3. Curcuma is yellowish externally, internally more or less orange-yellow passing into reddish-brown. The tuber has a round and cuspidate appearance. The smell is aromatic, somewhat analogous to ginger. 4. Turmeric is somewhat analogous to curcuma in shape, but turmeric is pungent and bitter in taste, warm and intoxious in property, and yellow in color, acting on the spleen and liver channels and governing the gi of the blood while curcuma is pungent and bitter in taste, cold and intoxious in property, red in color, acting on the heart and pericardium channels and governing the blood. 5. Turmeric is referred to zedoary, sliced turmeric, old jaundice, precious aromatic, and ovate rhizoma, and curcuma is referred to radix curcuma, curcuma aromatica, and cicada-belly curcuma

  • PDF

Comparison of Two Intramuscular Injection Techniques on the Severity of Discomfort and leasions at the Injection Site (근육주사법에 따른 주사부위의 불편감과 조직손상의 차이에 관한 연구)

  • 김경선
    • Journal of Korean Academy of Nursing
    • /
    • v.18 no.3
    • /
    • pp.257-268
    • /
    • 1988
  • The purpose of this study was to compare the effect of the Z-track intramuscular injection technique with the effect of the stand and intramuscular injection technique on the severity of discomfort and leasions at the injection site. The subjects of the study were 20 patients with only early tuber culosis excluding another abnormalities (a akin rash, allergy to topical use of alcohol, jaundice, edema, neurosensory abnormality, coagulation defects, obesity and thin). Data collection was done from Feb. 1 to March 15, 1988 by means of Korean Pain Measurement Tool, Visual Analogue Scale, and Objective measures of injection site lesions. The results of this study were as follows ; 1) Hypothesis 1 ; “The severity of subject discomfort is less following administration of the Z-track intramuscular injection technique than following administra tion injection technique.” was not sopported 2) Hypothesis 2 ; “The degress of severity subject discomfort is less following administration of the Z-track intramuscular injection technique than following administra tion of the standard intramuscular injection technique.” was not supported. 3) Hypothesis 3 ; “The severity of injection site lesions is less following administra tion of the Z-track intramuscular injection technique than following administration of the standard intramuscular injection techniques.” was not supported. 4) The terms that were selected included factor II (mild-moderate pain) of Ratio Scale Measuring Pain using Korean Pain Terms. In conclusion ; it was found that there was not a difference from the severity of subject discomfort between two groups, but the degress of severity of subject discomfort about following administration of the Z-t-rack intramuscular injection was tended to be declined. Therefore further studies suggest that the Z-track intramuscular injection technique can decrease the severity of discomfort in persons receiving frequently intramuscular injections. First of all, it is necessery to be developed an effective tool of discomfort measurement for the intramuscular injection in Korean.

  • PDF

ORAL CHARACTERISTIC OF ALAGILLE SYNDROME - A CASE REPORT (Alagille 증후군을 가진 환자의 구강내 특징에 대한 증례보고)

  • Kim, Tae-Wan;Kim, Young-Jin
    • The Journal of Korea Assosiation for Disability and Oral Health
    • /
    • v.3 no.1
    • /
    • pp.17-21
    • /
    • 2007
  • Alagille syndrome is an autosomal dominant genetic disorder and occurs in approximately 1 in 100,000 live births. Diagnostic criteria was established by Alagille. It is mainly caused by a mutation in the Jagged1 gene. Major clinical features of this syndrome are paucity of intrahepatic bile duct with cholestasis, characteristic facies, cardiac murmur, defects of vertebrae, and embryotoxon. And minor clinical features are mental retardation, renal involvement, growth retardation, other skeletal abnormalities, a high-pitched voice. The surviving prognosis of Alagille syndrome patients depends on the severity of cardiovescular malformation in the early ages of infant. However, with the increasing years, it depends on the severity of the liver disease. Cholestasis causes congenital jaundice, malnutrition and growth retardation. Also, the increase of serum cholesterol level cause xanthoma and pruritus. Even though the severity of these problems are reduce with age, there is cases where there is no way but liver transplantation. For oral features of Alagille syndrome patients, green discoloration of entire dentition, induced by bilirubin infiltration into dentinal tubules, is especially. Also, xanthoma on gingiva and partial hypodontia have been reported. This report is on the oral features of an Alagille syndrome patient who visited to Kyung-Pook University Hospital.

  • PDF

Health Literacy and Its Associated Factors in Korean-Chinese and Other Asian Immigrant Women in Korea (조선족과 다른 출신국 여성결혼이민자의 건강문해력과 영향 요인 비교)

  • Yang, Sook Ja;Chee, Yeon Kyung;An, Jisook;Park, Min Hee;Jung, Sunok
    • Journal of Korean Public Health Nursing
    • /
    • v.28 no.2
    • /
    • pp.211-227
    • /
    • 2014
  • Purpose: The purpose of this study was to compare health literacy levels of Asian immigrant women and factors associated with health literacy focusing on Korean-Chinese and other Asian immigrants in Korea. Methods: Data were drawn from 258 immigrant women, comprising 56 Korean-Chinese and 202 other immigrants from the Philippines, Vietnam, Japan, Cambodia, Thailand, Uzbekistan, Taiwan, and Han-Chinese. The Rapid Estimate of Adult Literacy in Medicine - Short Form (REALM-SF) was used to assess health literacy, which consisted of seven medical words in Korean (behavior, exercise, menopause, rectal, antibiotics, anemia, and jaundice). Data analysis was performed using descriptive statistics, t-test, ANOVA with $Sch{\acute{e}}ffe$ post hoc test, and hierarchical regression. Results: Korean-Chinese women showed significantly higher levels of health literacy ($M=6.41{\pm}1.07$), compared to their other immigrant counterparts ($M=4.55{\pm}2.15$). While Korean-Chinese women with longer time since immigration (p=.048) reported higher health literacy, for other immigrants, those living with children (p<.001), longer time since immigration (p=.011), and greater Korean language proficiency (p=.004) showed an association with higher health literacy. Conclusion: When providing health care services and health education, health care professionals should consider differences in levels of health literacy and factors associated with health literacy among Korean Chinese and other immigrants.

A study on the diagnosis of observing face color by Dongeuibogam (동의보감(東醫寶鑑)중 망면색(望面色)에 대한 연구(硏究))

  • Kim, Min-Jung;Park, Won-Hwan
    • The Journal of the Society of Korean Medicine Diagnostics
    • /
    • v.17 no.2
    • /
    • pp.131-155
    • /
    • 2013
  • Objectives A study on the importance of observing face color in the Dongeuibogam and the other things to know when we observe face color. Methods We used Deyuk Dongeuibogam of Dongeuibogam publishing company from original photographic edition. Results (1) In all section(ex. Naekyungpyeon, Oehyeongpyeon and Japbyoungpyeon), the chapters related with observing face color were fifty-one. In Naekyungpyeon, the chapters related with observing face color were nineteen(37%). In Oehyeongpyeon, the chapters related with observing face color were ten(20%). In Japbyoungpyeon, the chapters related with observing face color were twenty-two(43%). In all section(ex. Naekyungpyeon, Oehyeongpyeon and Japbyoungpyeon), the statements related with observing face color were three-hundred and nine. In Naekyungpyeon, the statements related with observing face color were sixty-four(21%). In Oehyeongpyeon, the statements related with observing face color were fifty-two(17%). In Japbyoungpyeon, the statements related with observing face color were one hundred ninety-three(62%). (2) In each chapter, the number of statement related with observing face color was studied. Pediatric Chapter (75) were the most common, followed by The way of finding the cause of a disease (27) Face (26) Cold (24) Weak&Fatigue (12) Phlegm, Jaundice (8) Nose, Worm, Chronic indigestion (7) Mouth&Tongue, Woman, Stool (6) Bodyshape, Liver, Heart, Kidney, Cough (5) Eye, Carbunculosis (4) Blood, Voice, Spleen, Lung, Chest, Fire, Edema, Detoxification (3) in order. (3) In each section(ex. Naekyungpyeon, Oehyeongpyeon and Japbyoungpyeon), the rate of chapter related with observing face color was studied. Naekyungpyeon(73%) was the highest. Japbyoungpyeon(61%). Oehyeongpyeon(38%) was the lowest. Conclusions The importance of each chapter for observing face color in the Dongeuibogam was studied. In addition, the entire ocular inspection, the part ocular inspection, moisture and the difference of skin according to the personal constitution should be considered. There are not only the important parts of the past that does not mean but also the unimportant parts of the past that are meaningful now. Additional studies will be needed for the latter.

CASE REPORT OF THE INTRINSIC STAINED TEETH OF PATIENTS WITH BILIARY ATRESIA (담도폐쇄증 환아의 내인성 착색치아에 대한 증례보고)

  • Lee, Chang-Hui;Lee, Sang-Dae;Kim, Young-Jae;Kim, Jung-Wook;Hahn, Se-Hyun;Lee, Sang-Hoon
    • Journal of the korean academy of Pediatric Dentistry
    • /
    • v.31 no.3
    • /
    • pp.400-405
    • /
    • 2004
  • Biliary atresia is defined as a complete obstruction of bile flow owing to destruction or absence of all or part of the extrahepatic bile ducts. This disease is occurring in approximately 1:10,000 live births and moderate predominance of female is noted. The etiology of biliary atresia remained unsolved. The signs and symptoms are hyperbilirubinemla, jaundice, clay-colored stools, steatorrhea, dark yellow urine and hepatomegaly. Currently biliary atresia is best managed by hepatic portoenterostomy with or without liver transplantation. Biliary atresia patients with these cases showed staining of the teeth. The stains ranged in color from yellowish-brown to deep green. Enamel hypoplasia was all erupted teeth present. Patients had poor oral hygiene and rampant caries.

  • PDF

A Case of Vivax Malaria Complicated by Adult Respiratory Distress Syndrome and Successful Management with Extracorporeal Membrane Oxygenation

  • Lee, Hyun-Jung;Baek, Ji-Hyeon;Chae, Myoung-Hun;Joo, Hoyeon;Lee, Jin-Soo;Chung, Moon-Hyun;Park, Yun-Kyu;Kim, Joung-Teak
    • Parasites, Hosts and Diseases
    • /
    • v.51 no.5
    • /
    • pp.551-555
    • /
    • 2013
  • Complicated malaria is mainly caused by Plasmodium falciparum, but, increasingly, Plasmodium vivax is also being reported as a cause. Since the reemergence of indigenous vivax malaria in 1993, cases of severe malaria have been steadily reported in Korea. Herein, we report a case of vivax malaria complicated by adult respiratory distress syndrome (ARDS) that was successfully managed with extracorporeal membrane oxygenation (ECMO). A 59-year-old man presented at our hospital with fever and abdominal pain, which had persisted for 10 days. On admission, the patient had impaired consciousness, shock, hypoxia and haziness in both lungs, jaundice, thrombocytopenia and disseminated intravascular coagulation, metabolic acidosis, and acute kidney injury. A peripheral blood smear and a rapid diagnostic test verified P. vivax mono-infection. Ten hours after admission, hypoxia became more severe, despite providing maximal ventilatory support. The administration of antimalarial agents, ECMO, and continuous venovenous hemofiltration resulted in an improvement of his vital signs and laboratory findings. He was discharged from the hospital 7 weeks later, without any sequelae.

Early Diagnosis of ABCB11 Spectrum Liver Disorders by Next Generation Sequencing

  • Lee, Su Jeong;Kim, Jung Eun;Choe, Byung-Ho;Seo, An Na;Bae, Han-Ik;Hwang, Su-Kyeong
    • Pediatric Gastroenterology, Hepatology & Nutrition
    • /
    • v.20 no.2
    • /
    • pp.114-123
    • /
    • 2017
  • Purpose: The goal of this study was the early diagnosis of ABCB11 spectrum liver disorders, especially those focused on benign recurrent intrahepatic cholestasis and progressive familial intrahepatic cholestasis. Methods: Fifty patients presenting neonatal cholestasis were evaluated to identify underlying etiologies. Genetic analysis was performed on patients suspected to have syndromic diseases or ABCB11 spectrum liver disorders. Two families with proven ABCB11 spectrum liver disorders were subjected to genetic analyses to confirm the diagnosis and were provided genetic counseling. Whole exome sequencing and Sanger sequencing were performed on the patients and the family members. Results: Idiopathic or viral hepatitis was diagnosed in 34%, metabolic disease in 20%, total parenteral nutrition induced cholestasis in 16%, extrahepatic biliary atresia in 14%, genetic disease in 10%, neonatal lupus in 2%, congenital syphilis in 2%, and choledochal cyst in 2% of the patients. The patient with progressive familial intrahepatic cholestasis had novel heterozygous mutations of ABCB11 c.11C>G (p.Ser4*) and c.1543A>G (p.Asn515Asp). The patient with benign recurrent intrahepatic cholestasis had homozygous mutations of ABCB11 c.1331T>C (p.Val444Ala) and heterozygous, c.3084A>G (p.Ala1028Ala). Genetic confirmation of ABCB11 spectrum liver disorder led to early liver transplantation in the progressive familial intrahepatic cholestasis patient. In addition, the atypically severe benign recurrent intrahepatic cholestasis patient was able to avoid unnecessary liver transplantation after genetic analysis. Conclusion: ABCB11 spectrum liver disorders can be clinically indistinguishable as they share similar characteristics related to acute episodes. A comprehensive genetic analysis will facilitate optimal diagnosis and treatment.