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Biliary atresia: Where do we stand now?

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World Journal of Hepatology 2016年第36期8卷 1593-1601页

作者： Krishna Kumar Govindarajan Department of Pediatric Surgery Jawaharlal Institute of Postgraduate Medical Education and Research

The pathway from clinical suspicion to establishing the diagnosis of biliary atresia in a child with jaundice is a daunting task. However, investigations available help to point towards the correct diagnosis in reasonable time frame. Imaging by Sonography has identified several parameters which can be of utility in the diagnostic work up. Comparison of Sonography with imaging by Nuclear medicine can bring out the significant differences and also help in appropriate imaging. The battery of Biochemical tests, available currently, enable better understanding of the line-up of investigations in a given child with neonatal cholestasis. Management protocols enable standardized care with optimal outcome. The place of surgical management in biliary atresia is undisputed, although Kasai procedure and primary liver transplantation have been pitted against each other. This article functions as a platform to bring forth the various dimensions of biliary atresia.

关键词： Biliary atresia Neonatal cholestasis Kasai procedure Neonatal jaundice hyperbilirubinemia

Effects of therapeutic plasma exchange on survival in patients with postoperative liver failure:a retrospective single-center study

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Emergency and Critical Care Medicine 2021年第2期1卷 70-74页

作者： Yoshiro Kobe Yoshihisa Tateishi Shigeto Oda Department of Surgery Chiba Emergency Medical Center3-32-1 IsobeMihama-kuChibaJapan Department of Emergency and Critical Care Medicine Japanese Red Cross Narita HospitalChibaJapan Department of Emergency Medicine Chiba Kaihin Municipal HospitalChibaJapan

Background:Recent decreases in the incidence of postoperative liver failure(POLF)have been attributed to advances in surgical techniques,critical care,and postoperative ***,POLF management remains a challenge,and worsening POLF is a significant cause of morbidity and *** plasma exchange(TPE)is used as a salvage strategy for POLF in some countries,and several studies conducted between 1980 and 1990 have reported the use of TPE for *** conducted this retrospective single-center study to investigate the outcomes of patients with POLF treated with ***:We retrospectively reviewed the charts of patients with POLF admitted to the intensive care unit who underwent TPE between November 2010 and March *** was defined as the presence of persistent *** on patient demographics,Glasgow Coma Scale score,platelet count,prothrombin time,and serum total bilirubin level were collected,and their Sequential Organ Failure Assessment scores were *** lengths of postoperative hospital stays,lengths of post-TPE hospital stays,and patient outcomes were also ***:TPE was performed in 20 patients with POLF during the study *** was initiated on the 49th postoperative day and was performed for a median of five *** improved hyperbilirubinemia and coagulopathy but had no effect on neurological and clinical *** 20 patients treated with TPE died after the 36th day(median)from the initial ***:Although TPE may improve laboratory values in patients with POLF,the current study suggests that it has no survival benefit.

关键词： Fresh-frozen plasma hyperbilirubinemia Plasmapheresis Postoperative liver failure Therapeutic plasma exchange

Glucose-6-phosphate-dehydrogenase deficiency and its correlation with other risk factors in jaundiced newborns in Southern Brazil

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Asian Pacific Journal of Tropical Biomedicine 2011年第2期1卷 110-113页

作者： Clarissa Gutirrez Carvalho Simone Martins Castro Ana Paula Santin Carina Zaleski Felipe Gutirrez Carvalho Roberto Giugliani Genetics Service Hospital de Clinicas de Porto AlegrePGP in PediatricsUFRGS Pharmacy School UFRGS Medical School UFRGS

Objective:To evaluate the correlation between glucose-6-phosphate-dehydrogenase(G6PD) deficiency and neonatal ***:Prospective,observational case-control study was conducted on 490 newborns admitted to Hospital de Clinicas de Porto Alegre for phototherapy, who all experienced 35 or more weeks of gestation,from March to December *** screening of G6PD activity was performed,followed by ***:There was prevalence of 4.6% and a boy-girl ratio of 3:1 in jaundiced *** jaundiced neonate with ABO incompatibility presented G6PD deficiency,and no Mediterranean mutation was found.A higher proportion of deficiency was observed in *** was no association with UGT1A1 variants. Conclusions:G6PD deficiency is not related to severe hyperbilirubinemia and considering the high miscegenation in this area of Brazil,other gene interactions should be investigated.

关键词： Glucosephosphate dehydrogenase Jaundice hyperbilirubinemia Neonatal Hemoglobin

Liver failure caused by prolonged state of malnutrition following bariatric surgery

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World Journal of Hepatology 2018年第3期10卷 396-399页

作者： Willem J Lammers Antonie JP van Tilburg Jan A Apers Janneke Wiebolt Department of Gastroenterology and Hepatology Erasmus Medical Center Department of Gastroenterology and Hepatology Franciscus Gasthuis and Vlietland Department of Surgery Franciscus Gasthuis and Vlietland Department of Internal Medicine Franciscus Gasthuis and Vlietland

Bariatric surgery is an effective tool in the treatment of patients with morbid obesity. In these case reports we describe 2 patients who developed liver failure after currently-practiced types of bariatric surgery, caused by a prolonged state of malnutrition provoked by psychiatric problems. Despite intensive guidance of a psychologist and dieticians after surgery, our patients deteriorated psychologically, resulting in a prolonged state of severe malnutrition and anorexia. Finally, a state of starvation was reached, passing a critical level of the liver capacity. Patients who present with signs of severe protein malnutrition after bariatric surgery should be closely monitored and checked for nutritional status. Specific attention should be given to patients who develop psychiatric problems post-bariatric surgery. If refeeding does not result in clinical improvement, reversal surgery should be considered in a timely manner.

关键词： Protein deficiency hyperbilirubinemia Hyperammonemia Liver failure Urea cycle

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Journal of Zhejiang University-Science B(Biomedicine & Biotechnology) 2014年第5期15卷 474-481页

作者： Yan LI Yu-jin QU Xue-mei ZHONG Yan-yan CAO Li-min JIN Jin-li BAI Xin MA Yu-wei JIN Hong WANG Yan-ling ZHANG Fang SONG Department of Medical Genetics Capital Institute of Pediatrics Department of Gastroenterology Affiliated Children’s HospitalCapital Institute of Pediatrics

Cdgler-Najjar syndrome type Ⅰ （CN-I） is the most severe type of hereditary unconjugated hyperbilirubinemia. It is caused by homozygous or compound heterozygous mutations of the UDP-glycuronosyltransferase gene （UGT1A1） on chromosome 2q37. Two patients clinically diagnosed with CN-I were examined in this paper. We sequenced five exons and their flanking sequences, specifically the promoter region of UGT1A 1, of the two patients and their parents. Quantitative real-time polymerase chain reaction （qRT-PCR） was used to determine the UGT1A1 gene copy number of one patient. In patient A, two mutations, c.239_245delCTGTGCC （***80HisfsX6; had not been reported previously） and c.1156G〉T （***1386Phe）, were identified. In patient B, we found that this patient had lost heterozygosity of the UGTIA1 gene by inheriting a deletion of one allele, and had a novel mutation c.1253delT （***418ArgfsX5） in the other allele. In summary, we detected three UGTIA 1 mutations in two CN-I patients： c.239_ 245delCTGTGCC （***80HisfsX6）, c.1253delT （***18ArgfsX5）, and c.1156G〉T （***1386Phe）. The former two mutations are pathogenic; however, the pathogenic mechanism of c.1156G〉T （***1386Phe） is unknown.

关键词： Crigler-Najjar syndrome type Ⅰ (CN-I) hyperbilirubinemia UDP-glycuronosyltransferase gene (UGT1A 1) Mutation Loss of heterozygosity

Changing pattern of neonatal jaundice and kernicterus in Chinese neonates

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Chinese Medical Journal 1997年第6期110卷 50-56页

作者： Chap-YungYeung 杨执庸 Department of Paediatrics University of Hong Kong Queen Mary Hospital Hong Kong

ＣｈａｎｇｉｎｇｐａｔｅｒｎｏｆｎｅｏｎａｔａｌｊａｕｎｄｉｃｅａｎｄｋｅｒｎｉｃｔｅｒｕｓｉｎＣｈｉｎｅｓｅｎｅｏｎａｔｅｓＣｈａｐＹｕｎｇＹｅｕｎｇ杨执庸Ｃｈｉｎｅｓｅｎｅｏｎａｔｅｓａｒｅｄｉｆｅｒｅｎｔｆｒ... 详细信息

ＣｈａｎｇｉｎｇｐａｔｅｒｎｏｆｎｅｏｎａｔａｌｊａｕｎｄｉｃｅａｎｄｋｅｒｎｉｃｔｅｒｕｓｉｎＣｈｉｎｅｓｅｎｅｏｎａｔｅｓＣｈａｐＹｕｎｇＹｅｕｎｇ杨执庸ＣｈｉｎｅｓｅｎｅｏｎａｔｅｓａｒｅｄｉｆｅｒｅｎｔｆｒｏｍＣａｕｃａｓｉａｎｓｉｎｍａｎ...

关键词： hyperbilirubinemia PREVENTION RISK

Neonatal Bilirubin Encephalopathy: Study of 30 Cases at Albert Royer National Children Hospital of Dakar

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Open Journal of Pediatrics 2020年第1期10卷 116-124页

作者： P. M. Faye Y. J. Dieng I. Deme D. F. Cissé M. Kane S. R. Tcheuffa Wekang A. Thiongane I. D. Ba A. Ba M. Gueye B. Niang P. S. Sow Amadou Sow Djibril Boiro N. R. Diagne-Gueye A. L. Fall O. Ndiaye Centre Hospitalier National d’Enfants Albert Royer Dakar Senegal Centre Hospitalier Abass Ndao Dakar Senegal Hopital pour Enfants de Diamniadio Dakar Senegal

Introduction: Unconjugated bilirubin jaundice is a common symptom in neonatal period. In some babies, excessive serum bilirubin concentrations can place them at risk of acute bilirubin encephalopathy (BE) when the unconjugated pigment crosses the blood-brain barrier. Our study aimed to describe epidemiology, diagnosis and prognosis of BE at the Neonatology Department of Albert Royer Children’s Hospital of Dakar. Materials and Methods: It was a retrospective, descriptive study of cases of BE from January 1, 2015 to June 30, 2019. Obstetric and perinatal data as well as postnatal jaundice data (onset time, associated signs, signs of encephalopathy, treatment and evolution) were collected and analyzed by SPSS software version 2.0. Almost all newborns (27 cases;90%) were exclusively breastfed. At admission, all children exhibited blunt jaundice and signs of encephalopathy dominated by the abolition of archaic reflexes (76.7%), low suction (22 cases;73.3%), central apnea (12 cases, 40%). The mean serum bilirubinemia was 322 mg/litre. Neonatal infection (10 cases;33.3%) and fetal-maternal incompatibility (16 cases;53.3%) were the main causes. All children received intensive phototherapy and exsanguino transfusion was performed for 7 newborns (23.3%). Nine children died (30% mortality rate). Conclusion: Only better organisation of perinatal care with enhanced postnatal follow-up can reduce the incidence of EB.

关键词： Neonatal hyperbilirubinemia Encephalopathy Perinatal Care

The Effect of Pomegranate Paste on Neonatal Jaundice Incidence: A Clinical Trial in Women during Pregnancy

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International Journal of Clinical Medicine 2017年第3期8卷 144-151页

作者： Mohsen Manouchehrian Mehrdad Shakiba Mamak Shariat Mohammad Hassan Lotfi Mohammad Kamalinejad Mohammad Babaeian Department of Traditional Medicine Shahid Sadoughi University of Medical Sciences Ardakan Iran Department of Pediatrics Shahid Sadoughi University of Medical Sciences Ardakan Iran Fetal & Neonatal Research Center Tehran University of Medical Sciences Tehran Iran Department of Biostatistics & Epidemiology Health Faculty Shahid Sadoughi University of Medical Sciences Ardakan Iran School of Pharmacy Shahid Beheshti University of Medical Sciences Tehran Iran

Purpose: Some topics such as women’s life style and nutrition and using some special medicines during pregnancy have been discussed and demonstrated as effective factors on infant’ health. Based on recommendations in Iranian traditional medicine, we aimed to evaluate the efficacy of pomegranate paste consumed by women during their pregnancy on the neonatal jaundice incidence. Methods: In this single-blinded controlled clinical trial, 80 healthy pregnant women were randomly divided into 2 groups, as treatment and control. The treatment group members added pomegranate paste to their daily diet from the 34th week of the pregnancy to birth, as administered. The levels of neonates’ bilirubin were checked in the 5th day of the delivery. Results: Both the need of phototherapy and the mean level of neonates’ bilirubin in the group fed with pomegranate paste were significantly lesser compared to control group. The number of neonates who were undergone phototherapy in the pomegranate paste receiving group was significantly lesser than that in the control group (P value = 0.029). By measuring the total bilirubin, statically significant difference between the treatment group and the control group was seen (P value = 0.021). Conclusion: The results of this study suggest the possible effect of adding pomegranate paste to pregnant women’s diet on the incidence of neonatal jaundice.

关键词： Pomegranate hyperbilirubinemia Neonatal Jaundice Pregnancy Iranian Traditional Medicine

Efficacy of Whole Blood Reconstituted (WBR) in Exchange Transfusion (ET) in Hemolytic Disease of New Born (HDN) —A Study of 110 Cases

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Open Journal of Blood Diseases 2013年第1期3卷 15-20页

作者： Dharmesh Chandra Sharma Sunita Rai Sudha Iyengar Bharat Jain Satya Sao Ajay Gaur Rahul Sapra Department of Pathology Gajra Raja Medical College Gwalior India Emergency Blood Bank Gwalior India Kilkari Nursing Home Gwalior India

Aim: This study was aimed to review and establish the practice of exchange transfusion (ET) with whole blood reconstituted (WBR) in hemolytic disease of newborn (HDN). Objectives: To observe fall in indirect serum bilirubin, correction of anemia and comparison with related studies. Background: Hemolytic disease of the Newborn is characterized by presence of IgG antibodies in maternal circulation, which causes hemolysis in the fetus by crossing the placenta and sensitizing red cells for destruction by macrophages in the fetal spleen with consequent hyperbilirubinemia. Exchange transfusion with or without phototherapy is the method of choice for treating the newborn with on going hemolysis Methods/Materials: Sample size consisted of 110 neonates in whom 119 exchange transfusions were carried out with WBR. WBR was prepared by suspending O Rhesus-D (RhD) positive/negative cells (compatible with neonate’s/ mother’s serum) in AB plasma. Double volume exchange transfusion(s) were carried out through umbilical vein by push-pull technique. Results: Out of 110 cases, 61 (55.5%) were of RhD HDN whereas ABO and other group HDN cases were 30 (27.3%) and 19 (17.3%) respectively. An average post-ET fall in indirect serum bilirubin by 54.6% and correction of anemia by3.7 gm/dl were reported in the study. Conclusion: An average post-ET fall in indirect serum bilirubin and correction of anemia was found to be more significant when compared to other studies. Hence we recommend exchange transfusion in HDN with WBR to obtain reasonable fall in indirect serum bilirubin and high average rate of correction of anemia.

关键词： Whole Blood Reconstituted (WBR) Hemolytic Disease of Newborn (HDN) Exchange Transfusion (ET) hyperbilirubinemia Anemia Correction