Stuart S Kaufman , Gabriel E Gondolesi , Thomas M Fishbein
{"title":"Parenteral nutrition associated liver disease","authors":"Stuart S Kaufman , Gabriel E Gondolesi , Thomas M Fishbein","doi":"10.1016/S1084-2756(03)00094-0","DOIUrl":"10.1016/S1084-2756(03)00094-0","url":null,"abstract":"<div><p>Liver disease is relatively common during parenteral nutrition (PN). Cholestasis predominates in infants, and ranges in severity from mild increases in plasma conjugated bilirubin to progressive liver failure that results in death of the patient. Severity of liver disease depends primarily on the magnitude of the underlying intestinal problem that indicated PN. Transient ileus resulting from a non-intestinal disorder usually results in trivial, self-limited liver injury. Removal of a large segment of the intestinal tract because of necrotizing enterocolitis or a congenital malformation predicts a more prolonged course with a guarded prognosis, particularly when initially complicated by sepsis. Pathogenesis of PN-associated liver disease is not completely understood. There is no proven treatment short of ending PN through adaptation of remnant intestine or intestinal transplantation, with or without a concurrent liver graft. Effective interventions that are less radical than transplantation are needed. Research that includes prospective trials of novel therapies in PN-associated liver disease is the key to improving outcome.</p></div>","PeriodicalId":74783,"journal":{"name":"Seminars in neonatology : SN","volume":"8 5","pages":"Pages 375-381"},"PeriodicalIF":0.0,"publicationDate":"2003-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S1084-2756(03)00094-0","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24426401","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Neonatal liver tumours","authors":"Dietrich von Schweinitz","doi":"10.1016/S1084-2756(03)00092-7","DOIUrl":"10.1016/S1084-2756(03)00092-7","url":null,"abstract":"<div><p>Primary liver tumours are very rare during the neonatal period, but increasing numbers of them are now diagnosed prenatally by routine ultrasound scan. A precise diagnosis is sometimes problematic because of non-specific clinical symptoms, misleading imaging and difficulties with histological interpretation. Benign infantile haemangioendothelioma usually undergoes spontaneous regression, but may be life-threatening due to congestive heart failure and/or consumptive coagulopathy when treatment with resection, embolization or arterial ligation is necessary. Malignant hepatoblastoma may occur in the newborn, and often has to be treated with chemotherapy to achieve resectability. Symptoms are less specific and the prognosis is worse than in older children. Mesenchymal hamartoma is a benign cystic lesion that should be resected whenever possible. Rarely, germ cell tumours occur in the neonatal liver. Benign teratomas have to be resected, while malignant choriocarcinomas may respond to chemotherapy and can be cured in some cases.</p></div>","PeriodicalId":74783,"journal":{"name":"Seminars in neonatology : SN","volume":"8 5","pages":"Pages 403-410"},"PeriodicalIF":0.0,"publicationDate":"2003-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S1084-2756(03)00092-7","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24426922","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Hepatic function and physiology in the newborn","authors":"S.V Beath","doi":"10.1016/S1084-2756(03)00066-6","DOIUrl":"10.1016/S1084-2756(03)00066-6","url":null,"abstract":"<div><p>The liver develops from progenitor cells into a well-differentiated organ in which bile secretion can be observed by 12 weeks' gestation. Full maturity takes up to two years after birth to be achieved, and involves the normal expression of signalling pathways such as that responsible for the JAG1 genes (aberrations occur in Alagille's syndrome), amino acid transport and insulin growth factors. At birth, hepatocytes are already specialized and have two surfaces: the sinusoidal side receives and absorbs a mixture of oxygenated blood and nutrients from the portal vein; the other surface delivers bile and other products of conjugation and metabolism (including drugs) to the canalicular network which joins up to the bile ductules. There is a rapid induction of functions such as transamination, glutamyl transferase, synthesis of coagulation factors, bile production and transport as soon as the umbilical supply is interrupted.</p><p>Anatomical specialization can be observed across the hepatic acinus which has three distinct zones. Zone 1 borders the portal tracts (also known as periportal hepatocytes) and is noted for hepatocyte regeneration, bile duct proliferation and gluconeogenesis. Zone 3 borders the central vein and is associated with detoxification (e.g. paracetamol), aerobic metabolism, glycolysis and hydrolysis and zone 2 is an area of mixed function between the two zones.</p><p>Preterm infants are at special risk of hepatic decompensation because their immaturity results in a delay in achieving normal detoxifying and synthetic function. Hypoxia and sepsis are also frequent and serious causes of liver dysfunction in neonates.</p><p>Stem cell research has produced many answers to the questions about liver development and regeneration, and genetic studies including studies of susceptibility genes may yield further insights. The possibility that fatty liver (increasingly recognized as non-alcoholic steatohepatitis or NASH) may have roots in the neonatal period is a concept which may have important long-term implications.</p></div>","PeriodicalId":74783,"journal":{"name":"Seminars in neonatology : SN","volume":"8 5","pages":"Pages 337-346"},"PeriodicalIF":0.0,"publicationDate":"2003-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S1084-2756(03)00066-6","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24426398","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Neonatal hepatitis syndrome","authors":"Eve A Roberts","doi":"10.1016/S1084-2756(03)00093-9","DOIUrl":"10.1016/S1084-2756(03)00093-9","url":null,"abstract":"<div><p>Conjugated hyperbilirubinaemia in an infant indicates neonatal liver disease. This neonatal hepatitis syndrome has numerous possible causes, classified as infective, anatomic/structural, metabolic, genetic, neoplastic, vascular, toxic, immune and idiopathic. Any infant who is jaundiced at 2–4 weeks old needs to have the serum conjugated bilirubin measured, even if he/she looks otherwise well. If conjugated hyperbilirubinaemia is present, a methodical and comprehensive diagnostic investigation should be performed. Early diagnosis is critical for the best outcome. In particular, palliative surgery for extrahepatic biliary atresia has the best chance of success if performed before the infant is 8 weeks old. Definitive treatments available for many causes of neonatal hepatitis syndrome should be started as soon as possible. Alternatively, liver transplantation may be life saving. Supportive care, especially with attention to nutritional needs, is important for all infants with neonatal hepatitis syndrome.</p></div>","PeriodicalId":74783,"journal":{"name":"Seminars in neonatology : SN","volume":"8 5","pages":"Pages 357-374"},"PeriodicalIF":0.0,"publicationDate":"2003-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S1084-2756(03)00093-9","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24426400","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Prenatal diagnosis of liver and biliary tract disease","authors":"Mark Davenport , Nedim Hadzic","doi":"10.1016/S1084-2756(03)00096-4","DOIUrl":"10.1016/S1084-2756(03)00096-4","url":null,"abstract":"<div><p>The diagnosis and detection of some forms of liver and bile duct disease have become possible since the adoption of routine fetal ultrasound scanning in the UK, and the remarkable advances in molecular biological techniques. Nevertheless, although antenatal detection may well have improved, it remains difficult to offer a specific diagnosis, much less a prognosis, in most cases.</p><p>Cystic dilatation of the biliary tree is probably the most common finding. This may be due to cystic choledochal malformations or, occasionally but crucially, biliary atresia. Postnatal investigations (such as ultrasonography, magnetic resonance cholangiography and liver histology) must take the latter possibility into account, as early surgery is the key to a successful outcome following reconstructive biliary surgery.</p><p>Antenatally diagnosed liver tumours are usually of vascular origin and have an unpredictable natural history. Some may cause fetal cardiac failure and are candidates for in-utero intervention, but in all likelihood, a large proportion will resolve without specific therapy.</p></div>","PeriodicalId":74783,"journal":{"name":"Seminars in neonatology : SN","volume":"8 5","pages":"Pages 347-355"},"PeriodicalIF":0.0,"publicationDate":"2003-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S1084-2756(03)00096-4","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24426399","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Biliary atresia","authors":"Hiroyuki Kobayashi , Mark D Stringer","doi":"10.1016/S1084-2756(03)00065-4","DOIUrl":"10.1016/S1084-2756(03)00065-4","url":null,"abstract":"<div><p>Biliary atresia (BA) is a congenital obliterative cholangiopathy of unknown aetiology, affecting both the intra- and extrahepatic bile ducts. Although relatively rare, BA must be excluded in any infant with conjugated hyperbilirubinaemia since the prognosis is improved by early diagnosis and prompt surgery. At least two phenotypes of BA are currently recognized; the syndromic variety is associated with other congenital anomalies and a poorer outcome. The results of treatment have steadily improved and, with a combination of timely expert surgery (Kasai portoenterostomy) and liver transplantation in specialist centres, good quality long-term survival is now possible in more than 90% of affected patients. A better understanding of the aetiology of BA and the pathogenesis of hepatic fibrosis is needed in order to develop new therapeutic strategies.</p></div>","PeriodicalId":74783,"journal":{"name":"Seminars in neonatology : SN","volume":"8 5","pages":"Pages 383-391"},"PeriodicalIF":0.0,"publicationDate":"2003-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S1084-2756(03)00065-4","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24426402","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Disorders of the Neonatal Liver and Bile Ducts","authors":"Mark D. Stringer","doi":"10.1016/S1084-2756(03)00091-5","DOIUrl":"10.1016/S1084-2756(03)00091-5","url":null,"abstract":"","PeriodicalId":74783,"journal":{"name":"Seminars in neonatology : SN","volume":"8 5","pages":"Page 335"},"PeriodicalIF":0.0,"publicationDate":"2003-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S1084-2756(03)00091-5","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"56439813","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Neonatal liver failure","authors":"Patricia McClean, Suzanne M Davison","doi":"10.1016/S1084-2756(03)00095-2","DOIUrl":"10.1016/S1084-2756(03)00095-2","url":null,"abstract":"<div><p>Liver failure in the neonatal period is challenging to diagnose and manage, and still carries a high mortality. With ongoing developments in the field of metabolic disorders and antiviral therapy, and the ability to offer liver transplantation to small babies, an overall survival of 40% has been achieved. Early recognition of liver failure, good supportive care and prompt referral to a paediatric liver transplant centre are essential elements in improving the outcome for these babies. Decisions about contra-indications to and timing of transplantation are complex as many of the disease processes are still evolving in the neonatal period, and extrahepatic disease, which cannot be corrected by a transplant, may appear later.</p></div>","PeriodicalId":74783,"journal":{"name":"Seminars in neonatology : SN","volume":"8 5","pages":"Pages 393-401"},"PeriodicalIF":0.0,"publicationDate":"2003-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S1084-2756(03)00095-2","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24426921","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Management of acute and chronic renal failure in the newborn","authors":"George B Haycock","doi":"10.1016/S1084-2756(03)00044-7","DOIUrl":"10.1016/S1084-2756(03)00044-7","url":null,"abstract":"<div><p>Acute renal failure may be caused by a failure of renal perfusion (pre-renal failure), damage to the renal parenchyma (intrinsic renal failure) or obstruction of the urinary tract (post-renal failure). Most cases of intrinsic renal failure in the newborn are due to asphyxia, often in combination with sepsis and nephrotoxic drugs. Persistent elevation of the plasma creatinine concentration above 132.5<!--> <!-->μmol/l (1.5<!--> <!-->mg/dl) is widely accepted as a diagnostic criterion. Oliguria or anuria may occur but is not always present. Post-renal failure is diagnosed by renal ultrasonography and is treated by relief of the obstruction. Pre-renal and post-renal failure can be distinguished by an analysis of urinary indices, especially the fractional sodium excretion, and by the response to fluid replacement. The conservative management of intrinsic renal failure includes careful attention to fluid balance, maintenance of adequate nutrition and prevention or correction of hyperkalemia, acidosis and hyperphosphatemia. Severe cases may require dialysis: peritoneal dialysis is used in most cases, but extracorporeal methods, including intermittent hemodialysis, hemofiltration and hemodiafiltration, are possible. Congenital chronic renal failure, usually caused by renal dysplasia with or without obstruction, presents in a manner similar to that of acute renal failure, with a progressive deterioration of plasma biochemical values. Dialysis is rarely necessary in the newborn period. The conservative management of chronic renal failure is similar to that of acute renal failure, with particular emphasis on nutrition, control of acidosis and the prevention of renal osteodystrophy by the use of dietary phosphate binders and vitamin D analogs.</p></div>","PeriodicalId":74783,"journal":{"name":"Seminars in neonatology : SN","volume":"8 4","pages":"Pages 325-334"},"PeriodicalIF":0.0,"publicationDate":"2003-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S1084-2756(03)00044-7","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24426930","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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