Published in Gut on September 01, 1991
The natural history of Niemann-Pick disease type C in the UK. J Inherit Metab Dis (2006) 1.87
Significance of low or normal serum gamma glutamyl transferase level in infants with idiopathic neonatal hepatitis. Eur J Pediatr (2006) 0.88
Observational cohort study of the natural history of Niemann-Pick disease type C in the UK: a 5-year update from the UK clinical database. BMC Neurol (2015) 0.84
Diversity of disorders causing neonatal cholestasis - the experience of a tertiary pediatric center in Germany. Front Pediatr (2014) 0.78
The usefulness of bone marrow aspiration in the diagnosis of Niemann-Pick disease type C in infantile liver disease. Arch Dis Child (2006) 0.77
Diagnostic value of procalcitonin and apo-e in extrahepatic biliary atresia. Iran J Pediatr (2014) 0.77
Analysis of the histologic features in the differential diagnosis of intrahepatic neonatal cholestasis. World J Gastroenterol (2009) 0.75
Niemann-Pick disease type C in the newborn period: a single-center experience. Eur J Pediatr (2017) 0.75
Liver transplantation (1). N Engl J Med (1989) 4.53
Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): review of 80 cases. J Pediatr (1987) 3.50
Late referral for biliary atresia--missed opportunities for effective surgery. Lancet (1989) 3.27
Extrahepatic biliary atresia versus neonatal hepatitis. Review of 137 prospectively investigated infants. Arch Dis Child (1976) 2.67
Niemann-Pick disease group C: clinical variability and diagnosis based on defective cholesterol esterification. A collaborative study on 70 patients. Clin Genet (1988) 2.50
Alpha 1-antitrypsin deficiency, emphysema, and liver disease. Genetic basis and strategies for therapy. J Clin Invest (1990) 2.35
Results of surgical treatment for extrahepatic biliary atresia in United Kingdom 1980-2. Survey conducted on behalf of the British Paediatric Association Gastroenterology Group and the British Association of Paediatric Surgeons. Br Med J (Clin Res Ed) (1985) 2.29
Fetal ascites: an unusual presentation of Niemann-Pick disease type C. Arch Dis Child (1989) 1.77
Long-term follow-up study of patients with cholangitis after successful Kasai operation in biliary atresia: selection of recipients for liver transplantation. J Pediatr Gastroenterol Nutr (1989) 1.69
alpha 1-Antitrypsin: molecular pathology, leukocytes, and tissue damage. J Clin Invest (1986) 1.61
Biliary atresia and other structural anomalies in the congenital polysplenia syndrome. J Pediatr (1974) 1.45
Early assessment of evolution of liver disease associated with alpha 1-antitrypsin deficiency in childhood. J Pediatr (1983) 1.20
Outcome of liver disease associated with alpha 1 antitrypsin deficiency (PiZ). Implications for genetic counselling and antenatal diagnosis. Arch Dis Child (1983) 1.17
Repair of the secretion defect in the Z form of alpha 1-antitrypsin by addition of a second mutation. Science (1988) 1.14
Syndromic paucity of the intrahepatic bile ducts: diagnostic difficulty; severe morbidity throughout early childhood. J Pediatr Gastroenterol Nutr (1988) 1.11
Bacterial cholangitis after surgery for biliary atresia. J Pediatr (1987) 1.10
Endoscopic sclerotherapy in the management of esophageal varices in 61 children with biliary atresia. J Pediatr Surg (1989) 1.04
Endoscopic retrograde cholangiopancreatography in infantile cholestasis. Arch Dis Child (1991) 1.02
Primary sclerosing cholangitis in childhood. Gastroenterology (1987) 1.02
Different immune mechanisms leading to autoimmunity in primary sclerosing cholangitis and autoimmune chronic active hepatitis of childhood. Hepatology (1989) 0.99
Prenatal diagnosis of alpha-1-antitrypsin deficiency by fetal blood sampling. Prenat Diagn (1987) 0.93
HLA phenotypes and gene polymorphisms in juvenile liver disease associated with alpha 1-antitrypsin deficiency. Hepatology (1990) 0.86
Is there still a place for the Kasai procedure in the treatment of extrahepatic biliary atresia? J Pediatr Gastroenterol Nutr (1989) 0.85
Genetics of alpha 1-antitrypsin deficiency in relation to neonatal liver disease. Mol Biol Med (1990) 0.81
Cholestatic disorders of infancy. Pediatr Clin North Am (1988) 0.78
Liver transplantation for metabolic disorders. Baillieres Clin Gastroenterol (1989) 0.78
Pathogenesis of alpha 1-antitrypsin deficiency-associated liver disease, 1990. J Pediatr Gastroenterol Nutr (1990) 0.77
Severe hypertension after liver transplantation in alpha 1 antitrypsin deficiency. Arch Dis Child (1990) 0.77
Late referral for biliary atresia--missed opportunities for effective surgery. Lancet (1989) 3.27
Extrahepatic biliary atresia versus neonatal hepatitis. Review of 137 prospectively investigated infants. Arch Dis Child (1976) 2.67
1 -Antitrypsin deficiency and neonatal hepatitis. Br Med J (1972) 2.40
Niemann-Pick disease type C: diagnosis and outcome in children, with particular reference to liver disease. J Pediatr (1993) 2.32
Results of surgical treatment for extrahepatic biliary atresia in United Kingdom 1980-2. Survey conducted on behalf of the British Paediatric Association Gastroenterology Group and the British Association of Paediatric Surgeons. Br Med J (Clin Res Ed) (1985) 2.29
Congenital hepatoportal arteriovenous fistula. Surgery (1995) 2.14
Monitoring of veno-occlusive disease after bone marrow transplantation by serum aminopropeptide of type III procollagen. Lancet (1993) 2.09
Rapid equilibrium radioimmunoassay for the amino-terminal propeptide of human type III procollagen. Clin Chem (1988) 2.01
Autoimmune hepatitis in childhood: a 20-year experience. Hepatology (1997) 1.97
Association between HLA and extrahepatic biliary atresia. J Pediatr Gastroenterol Nutr (1993) 1.96
Liver transplantation in 100 children: Cambridge and King's College Hospital series. BMJ (1992) 1.90
Internal anal sphincter. Observations on development and mechanism of inhibitory responses in premature infants and children with Hirschprung's disease. Arch Dis Child (1968) 1.90
Hepatitis syndrome in infancy--an epidemiological survey with 10 year follow up. Arch Dis Child (1985) 1.89
The changing clinical pattern of Reye's syndrome 1982-1990. Arch Dis Child (1996) 1.84
Autonomic nerves in rectum and colon in Hirschsprung's disease. A cholinesterase and catecholamine histochemical study. Arch Dis Child (1969) 1.78
Fetal ascites: an unusual presentation of Niemann-Pick disease type C. Arch Dis Child (1989) 1.77
Biliary atresia: the King's College Hospital experience (1974-1995) J Pediatr Surg (1997) 1.75
The internal anal sphincter in the cat: a study of nervous mechanisms affecting tone and reflex activity. J Physiol (1974) 1.68
Adrenergic myenteric nerves in Hirschsprung's disease. Br Med J (1968) 1.67
Jaundice at 14 days of age: exclude biliary atresia. Arch Dis Child (1991) 1.61
Immunohistochemistry of the liver and biliary tree in extrahepatic biliary atresia. J Pediatr Surg (2001) 1.55
Increased hepatic copper concentration in Indian childhood cirrhosis. Lancet (1979) 1.54
Fulminant hepatic failure in childhood: an analysis of 31 cases. Arch Dis Child (1980) 1.53
Benign non-traumatic inflammatory strictures of the extrahepatic biliary system. Br J Surg (1989) 1.52
Reye's syndrome: 20 years on. Br Med J (Clin Res Ed) (1983) 1.50
Incarceration of umbilical hernias in children: a rare but important complication. Pediatr Surg Int (1998) 1.49
Choledochal cysts: lessons from a 20 year experience. Arch Dis Child (1995) 1.48
Neonatal hepatitis syndrome and alpha-1-antitrypsin deficiency: an epidemiological study in south-east England. Postgrad Med J (1974) 1.48
Orcein-positive liver deposits in Indian childhood cirrhosis. Lancet (1978) 1.47
Lymphocyte cytotoxicity to autologous hepatocytes in HBsAg-negative chronic active hepatitis. Clin Exp Immunol (1979) 1.47
Results of surgery in 88 consecutive cases of extrahepatic biliary atresia. J R Soc Med (1982) 1.45
Molecular basis, clinical consequences and diagnosis of alpha-1 antitrypsin deficiency. Ann Clin Biochem (1997) 1.44
Hemangioendothelioma of the liver in infants. J Pediatr Surg (1995) 1.42
Biliary atresia splenic malformation syndrome: an etiologic and prognostic subgroup. Surgery (1993) 1.41
Pancreatitis in childhood associated with villous adenoma of the ampulla of Vater. Br J Surg (1996) 1.39
Antenatal diagnosis of congenital anomalies of the biliary tract. J Pediatr Surg (1998) 1.37
The prelaparotomy diagnosis of extrahepatic biliary atresia. Arch Dis Child (1983) 1.36
Extrahepatic biliary atresia. Recent developments in management. Arch Dis Child (1977) 1.35
Congenital microcephaly with hiatus hernia and nephrotic syndrome in two sibs. J Med Genet (1968) 1.33
Biliary amylase and congenital choledochal dilatation. J Pediatr Surg (1995) 1.31
Choledochal cyst: a 14-year surgical experience with 36 patients. Br J Surg (1988) 1.29
Assessment of injection sclerotherapy in the management of 152 children with oesophageal varices. Br J Surg (1988) 1.28
Gastric volvulus in childhood. J Pediatr Surg (1987) 1.25
Preferential activation of helper/inducer T lymphocytes in autoimmune chronic active hepatitis. Clin Exp Immunol (1987) 1.23
Developmental effects on liver D-glucuronolactone dehydrogenase levels and on D-glucaric acid excretion in urine; hormonal effects on D-glucaric acid excretion in urine. J Endocrinol (1968) 1.21
Experience of orthotopic liver transplantation and hepatic resection for hepatocellular carcinoma of less than 8 cm in patients with cirrhosis. Br J Surg (1995) 1.20
Allelic sequence variation in the HLA class II genes and proteins in patients with autoimmune hepatitis. Hepatology (1994) 1.19
Crigler-Najjar syndrome types I and II. Clinical experience--King's College Hospital 1972-1978. Phenobarbitone, phototherapy and liver transplantation. Mol Aspects Med (1987) 1.19
Novel surgical treatment and gastric pathology in diabetic gastroparesis. Diabet Med (1999) 1.17
Outcome of liver disease associated with alpha 1 antitrypsin deficiency (PiZ). Implications for genetic counselling and antenatal diagnosis. Arch Dis Child (1983) 1.17
Extrahepatic biliary atresia: a first-trimester event? Clues from light microscopy and immunohistochemistry. J Pediatr Surg (1994) 1.16
Wilson's disease: clinical presentation and use of prognostic index. Gut (1986) 1.16
The intrinsic myenteric innervation of the hind-gut and accessory muscles of defaecation in the cat. Z Zellforsch Mikrosk Anat (1973) 1.16
Value of urinary copper excretion after penicillamine challenge in the diagnosis of Wilson's disease. Hepatology (1992) 1.16
Abdominal tuberculosis: still a problem today. J R Soc Med (1986) 1.15
Extrahepatic biliary atresia: preoperative assessment and surgical results in 47 consecutive cases. Arch Dis Child (1980) 1.12
Focal nodular hyperplasia of the liver: results of treatment and options in management. Gut (1991) 1.12
Syndromic paucity of the intrahepatic bile ducts: diagnostic difficulty; severe morbidity throughout early childhood. J Pediatr Gastroenterol Nutr (1988) 1.11
Deterioration in lung function after general anaesthesia in patients with cystic fibrosis. Acta Paediatr Scand (1984) 1.10
Injection sclerotherapy for oesophageal varices in children. Br J Surg (1982) 1.08
Exocrine pancreatic insufficiency in syndromic paucity of interlobular bile ducts. J Pediatr Gastroenterol Nutr (1989) 1.05
Complications and limitations of injection sclerotherapy in portal hypertension. Gut (1993) 1.04
Spontaneous perforation of the bile duct in infants. Br J Surg (1991) 1.04
Endoscopic sclerotherapy in the management of esophageal varices in 61 children with biliary atresia. J Pediatr Surg (1989) 1.04
Longterm outcome after injection sclerotherapy for oesophageal varices in children with extrahepatic portal hypertension. Gut (1994) 1.04
Effects of rectal distension on the internal anal sphincter of cats. J Physiol (1972) 1.03
Current therapy for Crigler-Najjar syndrome type 1: report of a world registry. Hepatology (1996) 1.03
HLA DQA, DQB, and DRB genotyping by oligonucleotide analysis: distribution of alleles and haplotypes in British caucasoids. Hum Immunol (1992) 1.03
Partial purification of hepatic UDP-glucuronyltransferase. Studies of some of its properties. Biochim Biophys Acta (1970) 1.02
Endoscopic retrograde cholangiopancreatography in infantile cholestasis. Arch Dis Child (1991) 1.02
Alagille syndrome: family studies. J Med Genet (1995) 1.02
Primary sclerosing cholangitis in childhood. Gastroenterology (1987) 1.02
Does the morphology of the extrahepatic biliary remnants in biliary atresia influence survival? A review of 205 cases. J Pediatr Surg (1994) 1.01
Cytotoxicity to isolated rabbit hepatocytes by lymphocytes from children with liver disease. J Pediatr (1977) 1.00
Immunohistochemical features of the portal tract mononuclear cell infiltrate in chronic aggressive hepatitis. Arch Dis Child (1992) 1.00
Hepatic complications of cystic fibrosis. Lancet (1981) 0.99
Focal nodular hyperplasia of the liver: a rational approach to treatment. Gut (1976) 0.99
Different immune mechanisms leading to autoimmunity in primary sclerosing cholangitis and autoimmune chronic active hepatitis of childhood. Hepatology (1989) 0.99
Developmental intrahepatic shunts of childhood: radiological features and management. Eur Radiol (1997) 0.97
Surgery in children with homozygous sickle cell anaemia. Ann R Coll Surg Engl (1979) 0.97
Graft-versus-host disease after orthotopic liver transplantation in a child. Transplant Proc (1990) 0.97
Congenital structural abnormalities in biliary atresia: evidence for etiopathogenic heterogeneity and therapeutic implications. Acta Paediatr Scand (1991) 0.97
Cellular expression of tumour necrosis factor-alpha and interferon-gamma in the liver biopsies of children with chronic liver disease. J Hepatol (1994) 0.97