• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제17권4호
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• pp.266-269
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• 2014

Gilbert syndrome is the most common inherited disorder of bilirubin glucuronidation. It is characterized by intermittent episodes of jaundice in the absence of hepatocellular disease or hemolysis. Hereditary spherocytosis is the most common inherited hemolytic anemia and is characterized by spherical, osmotically fragile erythrocytes that are selectively trapped by the spleen. The patients have variable degrees of anemia, jaundice, and splenomegaly. Hereditary spherocytosis usually leads to mild-to-moderate elevation of serum bilirubin levels. Severe hyperbilirubinemia compared with the degree of hemolysis should be lead to suspicion of additional clinical conditions such as Gilbert syndrome or thalassemia. We present the case of a 12-year-old boy with extreme jaundice and nausea. The diagnosis of hereditary spherocytosis was confirmed by osmotic fragility test results and that of Gilbert syndrome by genetic analysis findings.

• Neonatal Medicine
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• 제17권2호
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• pp.266-269
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• 2010

Gilbert 증후군(Gilbert's syndrome)은 빌리루빈의 체외배설을 위해 포합시키는 기능을 가진 효소인 uridine diphosphate glucuronosyltransferase (UGT)의 활성도 감소에 의해 야기되며 만성, 비용혈성, 비포합 고빌리루빈혈증을 유발한다. 대부분 경증의 증상을 보이며 인구의 3-10%에서 나타나는 것으로 알려져 있다. 치료로 페노바비탈(phenobarbital)을 투여할 수 있으며 이 페노바비탈은 UGT 효소활성도를 증가시켜 혈중 빌리루빈 농도를 떨어뜨린다. 본 증례에서는 일반적인 경우와 달리 심한 신생아 황달이 동반된 Gilbert 증후군을 기술하였다. 환아는 생후 2-3일경부터 황달 소견을 보였으며 생후 5일경 혈중 총빌리루빈 수치가 34 mg/dL로 높게 상승되어 있어 집중적인 광선치료의 시행과 함께 경구 페노바비탈을 투여 받았다. 검사실 소견에서 정상 혈색소, 망상적혈구 수치 보였으며 direct Coombs' test 에서도 정상 소견 보여 용혈성 고빌리루빈혈증은 제외하였으며 이후 시행한 유전자 검사에서 UGT1A1 유전자의 -3279T>G, 211G>A 변이가 발견되어 Gilbert 증후군으로 진단되었다. 광선치료와 경구 페노바비탈 투여로 혈중 총 빌리루빈 농도의 지속적 감소를 보여 퇴원하였으며 이후 외래검사상 총 빌리루빈 수치는 안정적이었다. 저자들은 심한 신생아 황달을 보인 Gilbert 증후군의 예를 보고하는 바이다.

• 동의생리병리학회지
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• 제28권6호
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• pp.657-661
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• 2014

Gilbert's syndrome is one that shows a benign course with intermittent unconjugate hyperbilirubinemia without any evidence of hepatobiliary tract disease or hemolysis. It is often found in a health examination or blood laboratory test by chance. In particular, patients who are taking drugs, including herbal medicine should be careful for their medication due to the possibility of associations with changes in liver function because of drug metabolism, sometimes they have to quit the use of the medication for a certain period and often they should get an additional test. Two male patients increased serum total bilirubin level without other systemic symptoms in screening test for clinical herb medicine pharmacokinetics study. Therefor they was diagnosed with suspected Gilbert's syndrome. They had been calory deprivation test with 24 hours fasting state. They also performed liver function test and ultrasonogram for evaluation of hepatobiliary tract disease. Total serum bilirubin was markedly increased, especially unconjugate bilirubin level higher over the two times than base line after they had been calory deprivation for 24 hours, They was not found another abnormality all laboratory results and physical examination. This study is a report on two cases of hyperbilirubinemia, diagnosed as Gilbert's syndrome, which were found in the process of a clinical pharmacokinetic study of a decoction of medicinal herbs.

• 한방비만학회지
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• 제11권2호
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• pp.69-74
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• 2011

절식요법은 일정기간 섭취 칼로리를 제한하여 질병을 치료하는 방법이다. 600kcal이하로 칼로리를 제한하는 절식기에는 공복감, 속쓰림, 두통, 탈모 등의 여러 가지 증상들이 발생할 수 있으며, 길버트 증후군 환자에서는 특별한 간기능의 이상이나 용혈 없이 비포합 빌리루빈의 증가로 인한 황달이 나타날 수 있다. 이 증례보고서에서는 절식요법의 절식기에 황달이 발생한 길버트 증후군 환자 1례를 경험하여 문헌고찰과 함께 보고하였다.

• 대한한방내과학회지
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• 제26권3호
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• pp.634-640
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• 2005

Objective : This study was designed to investigate the effects of herbal medicine on jaundice caused by hepatic resection. Methods : This study was carried out on one patient with jaundice ensued by hepatic resection. The patient was admitted on October 25th, and treated until November 13th, 2004, using acupuncture and herbal medicine(Hepacure-I and Ex.Sosiho-tang.) Result : After 18 days, clinical symptoms had improved. Results of total bilirubin, ALP, GGT tests and urinalysis also showed improvement. Conclusions : These result support a role for this combination of acupunture and herbal medicine in treatment of chronic liver disease.

• Journal of Chest Surgery
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• 제56권5호
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• pp.346-352
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• 2023

Background: A significant proportion of cardiac surgery intensive care unit (CSICU) patients require long-term ventilation, necessitating tracheostomy placement. The goal of this study was to evaluate the long-term postoperative outcomes and complications associated with percutaneous dilatational tracheostomy (PDT) in CSICU patients. Methods: All patients undergoing PDT after cardiac, thoracic, or vascular operations in the CSICU between January 1, 2013 and January 1, 2021 were identified. They were evaluated for mortality, decannulation time, and complications including bleeding, infection, and need for surgical intervention. Multivariable regression models were used to identify predictors of early decannulation and the complication rate. Results: Ninety-three patients were identified for this study (70 [75.3%] male and 23 [24.7%] female). Furthermore, 18.3% of patients had chronic obstructive pulmonary disease (COPD), 21.5% had history of stroke, 7.5% had end-stage renal disease, 33.3% had diabetes, and 59.1% were current smokers. The mean time from PDT to decannulation was 39 days. Roughly one-fifth (20.4%) of patients were on dual antiplatelet therapy and 81.7% had anticoagulation restarted 8 hours post-tracheostomy. Eight complications were noted, including 5 instances of bleeding requiring packing and 1 case of mediastinitis. There were no significant predictors of decannulation prior to discharge. Only COPD was identified as a negative predictor of decannulation at any point in time (hazard ratio, 0.28; 95% confidence interval, 0.08-0.95; p=0.04). Conclusion: Percutaneous tracheostomy is a safe and viable alternative to surgical tracheostomy in cardiac surgery ICU patients. Patients who undergo PDT have a relatively short duration of tracheostomy and do not have major post-procedural complications.