• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제21권4호
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• pp.297-305
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• 2018

Purpose: There is a lack of scholarly reports on pediatric emergency department (PED) exposure to hyperbilirubinemia. We aimed to describe the epidemiology of hyperbilirubinemia in patients presenting to a PED over a three-year period. Methods: This was a retrospective cohort study, completed at an urban quaternary academic PED. Patients were included if they presented to the PED from 2010 to 2012, were 0 to 18 years in age, and had an elevated serum bilirubin for age. A chart review was completed to determine the incidence of hyperbilirubinemia, etiology, diagnostic work up and prognosis. The data set was stratified into four age ranges. Results: We identified 1,534 visits where a patient was found to have hyperbilirubinemia (0.8% of all visits). In 47.7% of patients hyperbilirubinemia was determined to have arisen from an identifiable pathologic etiology (0.38% of all visits). First-time diagnosis of pathologic hyperbilirubinemia occurred in 14% of hyperbilirubinemia visits (0.11% of all visits). There were varying etiologies of hyperbilirubinemia across age groups but a male predominance in all (55.0%). 15 patients went on to have a liver transplant and 20 patients died. First-time pathologic hyperbilirubinemia patients had a mortality rate of 0.95% for their initial hospitalization. Conclusion: Hyperbilirubinemia was not a common presentation to the PED and a minority of cases were pathologic in etiology. The etiologies of hyperbilirubinemia varied across each of our study age groups. A new discovery of pathologic hyperbilirubinemia and progression to liver transplant or death during the initial presentation was extremely rare.

• Clinical and Experimental Pediatrics
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• 제50권1호
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• pp.52-55
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• 2007

목 적 : 고빌리루빈혈증과 황달, 담낭수종 등은 가와사끼병의 일부에서 발생한다. 이 경우에 복통과 식욕부진, 복부팽만 등이 나타나 입원 기간이 길어질 수도 있다. 가와사끼병 급성기의 임상과정에서 고빌리루빈혈증의 관련인자가 있는가를 알아보고자 본 연구를 시행하였다. 방 법 : 1997년 1월부터 2004년 12월까지 만 8년간 원광대학병원 소아과에 입원하여 가와사끼병으로 치료받았던 282명의 환자를 대상으로 후향적으로 조사하였다. 나이와 성별, 발열-입원 기간, 치료-해열 기간, 입원 당시의 백혈구수, 혈소판수, ESR, CRP, AST, ALT치와 무균성 농뇨 등을 정상빌리루빈군(A)(n=269)과 고빌리루빈혈증군(B)(n=13)으로 나누어 비교 분석하였다. 통계처리는 Mann-Whitney test와 Fisher's exact test, logistic 회귀분석으로 하였다. 결 과 : 13 례(4.6%)에서 고빌리루빈혈증을 보였고 B군에서 나이와 ALT치가 높았다(P값 각각 0.003, 0.018). 백혈구 증가증이 있었던 경우와 AST/ALT치가 동시에 상승한 경우에는 고빌리루빈혈증의 발현 빈도가 더 높았다(P값 각각 0.007, 0.042). 짧은 발열-입원 기간과 고연령층이 고빌리루빈혈증에 영향을 주는 요인으로 분석되었다(P값 각각 0.007, 0.003). 반면에 ESR, CRP, 혈소판수, AST, ALT, 무균성 농뇨, 치료-해열 기간 등은 영향을 주지 않았다. 결 론 : 소아기의 가와사끼병에서 짧은 발열-입원 기간과 고연령층 환자군이 고빌리루빈혈증의 발현에 영향을 주는 인자로 분석되었다.

• Journal of Chest Surgery
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• 제26권3호
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• pp.170-179
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• 1993

Three hundred consecutive adult patients having cardioaortic surgery under the cardiopulmonary bypass for a variety of cardioaortic lesions were evaluated retrospectively for postoperative hyperbilirubinemia[above 5mg/100ml].We found twenty eight patients with postoperative hyperbilirubinemia and divided them into two groups according to the postoperative day of peak s-bilirubin .Group A was the patients with a peak s-bilirubin level within three days ,and group B above three days postoperatively.Group B was divided into group S[survive] and group D[death] . We had compaired the survival & death group and observed the correlation between the decreasing tendency of postoperative s-bilirubin & the nutrition per os in group B. The incidence of postoperative hyperbilirubinemia,as defined by a s-bilirubin concentration of 5.0mg/100ml or greater,was 9.3%.The mortality rates of group A & B were 0.0% and 35.7% respectively.Important contributing factors between group A & B were the age,duration of ICU,Max.DOAB[maximum dose of catecholamine used],amount of blood transfused during or shortly after surgery and preoperative pulmonary hypertension[main pulmonary artery pressure > 30mmHg] and backward heart failure. The risking factors of group D compared to group S were as follows the age,preoperative & postoperative SGOT[serum glutamic-oxaloacetic trasaminase],postoperative total & indirect bilirubin,cardiopulmonary bypass time,duration of ICU & mechanical ventilation ,Max.DOAB,preoperative pulmonary hypertension and backward heart failure.The six patients in group B showed good correlation between the decreasing point of s-bilirubin and the starting day of oral or tube feeding.

• Clinical and Experimental Pediatrics
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• 제49권1호
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• pp.6-13
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• 2006

Jaundice is one of the most common gastrointestinal conditions found in neonatal period, and most jaundice is benign. But because of the possibility of bilirubin toxicity, every newborn infants must be examined to identify the development of severe hyperbilirubinemia. To prevent the development of severe hyperbilirubinemia, promote and support successful breast-feeding, perform a systemic assessment before discharge for the risk of severe hyperbilirubinemia, provide early and close follow-up program, and treat with phototherapy or exchange transfusion or other therapeutic modalities, if indicated, are recommended.

• 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.

• Journal of Yeungnam Medical Science
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• 제31권2호
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• pp.75-81
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• 2014

Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease. Despite of proper treatment and improving treatment regimens, HLH patients still show a fatal prognosis. Therefore the evaluation of prognostic factor is important and there are many studies about hyperbilirubinemia as a prognostic factor in HLH. So we studied the prognostic value of hyperbilirubinemia in HLH children. Methods: A retrospective analysis was performed about 33 patients who were diagnosed with HLH at Pusan National University Hospital and Yangsan Pusan University Hospital between January 2000 to December 2012. We reviewed the clinical characteristics, laboratory findings, and results of treatment to identify hyper-bilirubinemia as a prognostic factor in HLH patients. Results: The median age of patients at diagnosis was 32 months. Most of patients presented with fever, pale appearance, abdominal pain and jaundice. Forty-eight point five percentage of patients showed normal serum bilirubiln level (<2.0 mg/dL) and 51.5% showed hyperbilirubinemia (${\geq}2.0mg/dL$). In normal serum bilirubin group, 1 patient (6.3%) was relapsed and 1 patient (5.9%) was relapsed in hyperbilirubinemia group. In the hyperbilirubinemia group, the mortality was higher than the normal bilirubin group but, there was no statistical significance. Conclusion: As a prognostic factor serum bilirubin at diagnosis in HLH patients, there was no significant correlation between hyperbilirubinemia and poor outcome. But, our study has a limitation that the number of patients is too small and almost showed good prognosis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제7권2호
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• pp.243-247
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• 2004

저자 등은 정계정맥류 수술을 위해 본원 비뇨기과에 입원한 14세 남아가 공막의 황달과 직접형 빌리루빈의 증가 소견을 보였고 간생검 등으로부터 로터 증후군으로 증명되어 참고문헌과 아울러 보고하는 바이다.

• 대한한방소아과학회지
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• 제30권4호
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• pp.99-110
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• 2016

Introduction Jaundice is one of the most common conditions found in neonatal period. Phototherapy is one of the main treatments for neonatal jaundice. However, several adverse effects of the phototherapy have been reported, including DNA damage recently. Therefore, a variety of treatments have been conducted to shorten the duration of phototherapy. Meanwhile, it has been hardly tried to prevent neonatal jaundice, but diverse approaches have been tried in traditional Chinese medicine (TCM) for many years. Therefore, this study aims to analyze the studies for the treatments to prevent neonatal jaundice based on TCM. Materials and Methods Various literatures have been searched via CNKI, and PubMed using the terms "neonatal jaundice" (新生儿黄疸, 胎黃, 胎疸) in category of 'Traditional Chinese Medicine', 'Traditional Chinese Medicinal Herbs' and 'Combination of Traditional Chinese Medicine With Western Medicine'. The search range included randomized controlled trials (RCTs), controlled clinical trials (CCTs), case reports, reviews and animal experiments published from 2013 to 2015. Results A total of 104 studies were found. 93 articles were excluded by reviewing the titles and abstracts. Out of the remaining 11 studies, 9 articles were RCTs, 2 articles were CCTs. The treatment used in neonatal babies in the studies were, using herbal remedies (meditation 5, bath 3 and retention enema 1) and the acupressure. All treatments were initiated within the first 24-48 hours after their birth. Then, there was a study that the subjects taken the herbal medicine were women during pregnancy. The outcome assessments used were the serum total bilirubin (TB), transcutaneous bilirubin measurement (TCB), the incidence of the hyperbilirubinemia, the duration time of the jaundice. Especially, TB, TCB and the incidence of the hyperbilirubinemia have decreased significantly more than that of the control group in the most of the results. No severe adverse events were reported in all articles reviewed. Conclusions Conventional treatment such as, herbal medicine in TCM, seems to be the effective way to prevent neonatal jaundice or hyperbilirubinemia. Therefore, the conventional treatment may be favorable choice for preventive treatment for neonatal jaundice.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제11권2호
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• pp.219-222
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• 2008

저자들은 4개월간 지속된 황달을 주소로 내원한 13년 6개월 남아에서 유전자 검사로 UGT1A1 유전자의 5번째 exon에서 1456번째 염기 치환(1456T>G)으로 인한 486번째 아미노산인 tyrosine이 aspartate로 치환된 변이(Y486D)를 확인하고 제2형 Crigler-Najjar 증후군으로 진단한 증례를 경험하였기에 보고한다.

• Clinical and Experimental Pediatrics
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• 제60권4호
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• pp.106-111
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• 2017

Purpose: This study aimed to determine the prevalence of glucose-6-phosphate dehydrogenase (G6PD) deficiency among infants with neonatal indirect hyperbilirubinemia (NIH); compare G6PD-deficient and G6PD-normal patients regarding hyperbilirubinemia and need for exchange transfusions (ET); and assess risk factors for ET and kernicterus. Methods: This is a case-control retrospective study. Medical records of NIH patients admitted to the Pediatric Department, Salmaniya Medical Complex, Bahrain, between January 2007 and June 2010 were reviewed. Data on sex, age at presentation, hospitalization duration, need for ET, hemoglobin (Hb) level, reticulocyte count, direct Coombs test, serum total and indirect bilirubin levels, thyroid function, blood and urine cultures, G6PD status, and blood groups were collected and compared between the G6PD-deficent and G6PD-normal patients. Results: Of 1,159 NIH patients admitted, 1,129 were included, of whom 646 (57%) were male. Among 1,046 patients tested, 442 (42%) were G6PD deficient, 49 (4%) needed ET, and 11 (1%) had suspected Kernicterus. The G6PD-deficient patients were mainly male (P<0.0001), and had lower Hb levels (P<0.0001) and higher maximum bilirubin levels (P=0.001). More G6PD-deficient patients needed ET (P<0.0001). G6PD deficiency (P=0.006), lower Hb level (P=0.002), lower hematocrit count (P=0.02), higher bilirubin level (P<0.0001), higher maximal bilirubin level (P<0.0001), and positive blood culture result (P<0.0001) were significant risk factors for ET. Maximal bilirubin level was a significant risk factor for kernicterus (P=0.021) and independently related to ET (P=0.03). Conclusion: G6PD deficiency is an important risk factor for severe NIH. In G6PD-deficent neonates, management of NIH should be hastened to avoid irreversible neurological complications.