Fatal neonatal liver failure and mitochondrial cytopathy: An observation with antenatal ascites

Fayon, Michaël; Lamireau, T.; Bioulac‐Sage, Paulette; Letellier, Thierry; Moretto, Brigitte; Parrot-Roulaud, F.; Coquet, M; Malgat, Monique; Sarlangue, J.; Balabaud, Charles; Demarquez, Jean-Louis; Mazat, Jean Pierre

doi:10.1016/0016-5085(92)91525-9

Cited by 36 publications

(16 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Immunostaining in hepatocytes for C5b-9, the terminal complement cascade neoantigen occurring specifically after complement classical pathway activation, is a highly suggestive biomarker of the disease (13,14) even if recent observations in a galactosemic family have raised the question of its specificity (38). By increasing cellular iron stocks, a secondary oxidant injury would worsen foetal hepatocyte damage (33). A recent hypothesis suggested that hepatocyte alloimmune injury results in dysregulation of foetal iron homeostasis by removing foetal liver hepcidin feedback on maternofoetal iron flux mediated by transporters including ferroportin (12).…”

Section: Discussionmentioning

confidence: 99%

Transient Neonatal Liver Disease After Maternal Antenatal Intravenous Ig Infusions in Gestational Alloimmune Liver Disease Associated With Neonatal Haemochromatosis

Baruteau¹,

Heissat²,

Broué³

et al. 2014

J. pediatr. gastroenterol. nutr.

Self Cite

View full text Add to dashboard Cite

show abstract

Section: Discussionmentioning

confidence: 99%

Transient Neonatal Liver Disease After Maternal Antenatal Intravenous Ig Infusions in Gestational Alloimmune Liver Disease Associated With Neonatal Haemochromatosis

Baruteau¹,

Heissat²,

Broué³

et al. 2014

J. pediatr. gastroenterol. nutr.

Self Cite

View full text Add to dashboard Cite

show abstract

“…We report a case of neonatal fatal mitochondrial cytopathy with COX deficiency with hypertrophic cardiomyopathy and disseminated fatty nodules in the liver. Indeed mitochondrial cytopathy have been recognized as possible causes of hepatic failure in neonate, [5][6][7] but to the best of our knowledge, reports of the fatty nodule formation in the liver have not been found.…”

Section: Discussionmentioning

confidence: 89%

Section: Discussionmentioning

confidence: 99%

“…In fatal neonatal liver failure, microvesicular steatosis, micronodular cirrhosis, major canalicular cholestasis, and bile duct thrombi of the liver are common histological features. 6,7 Bioulac-Sage et al . hypothesized that severe microvesicular steatosis and chronic cholestasis led to cell death, fibrosis and eventually cirrhosis of the liver.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Fatal neonatal mitochondrial cytopathy with disseminated fatty nodules in the liver

Teraoka¹,

Yokoyama²,

Ichimura³

et al. 2003

Pediatrics International

View full text Add to dashboard Cite

“…3.131). 1878 Changes at the ultrastructural level include pleomorphic mitochondria with few or no cristae and a fluffy, granular matrix 1819,1875 (Fig. 3.132).…”

Section: Histological Diagnosis Of Mitochondriopathiesmentioning

confidence: 99%

Developmental and Inherited Liver Disease

Quaglia¹,

Roberts²,

Torbenson³

2018

Macsween's Pathology of the Liver

View full text Add to dashboard Cite

Diagnostic approach to histology 113 Neonatal and early infantile liver disease 113 Postneonatal, childhood and adulthood presentation 118 Neonatal hepatitis 119 Histopathological features 120 Biliary atresia 121 Classification and aetiopathogenesis 121 Pathological features at surgical intervention 124 Pathology of intrahepatic changes 126 Paucity of intrahepatic bile ducts 129 Alagille syndrome (arteriohepatic dysplasia) 129 Nonsyndromic duct paucity disorders 131 Bile duct anomalies, congenital dilations and ductal plate malformation disorders 131 Choledochal cyst 132 Hereditary fibropolycystic disease (ductal plate malformation) 133 Cystic fibrosis 141 Hereditary disorders of bile acid synthesis and bilirubin metabolism 143 Primary disorders of bile acid synthesis 143 Disorders of bile canalicular transporters 146 Other diseases causing intrahepatic cholestasis 150 Hereditary defects of bilirubin metabolism 151 Disorders of porphyrin metabolism 153 Porphyria cutanea tarda 154 Erythropoietic protoporphyria 155 Disorders of carbohydrate metabolism and related conditions 156 Glycogen storage diseases (glycogenoses) 156 Myoclonus epilepsy, Lafora type (Lafora disease) 161 Galactosaemia Hereditary fructose intolerance Disorders of glycoprotein and glycolipid metabolism Mucopolysaccharidoses Aspartylglucosaminuria α-Mannosidosis Fucosidosis Mucolipidoses Congenital disorders of glycosylation (carbohydrate-deficient glycoprotein syndrome) Endoplasmic reticulum storage diseases α1-Antitrypsin deficiency α1-Antichymotrypsin deficiency Afibrinogenaemia and hypofibrinogenaemia Antithrombin III deficiency Disorders of amino acid metabolism Hereditary tyrosinaemia type 1 Congenital hyperammonaemia syndromes and urea cycle disorders Cystinosis Homocystinuria (cystathionine β-synthase deficiency) Disorders of lipoprotein and lipid metabolism Abetalipoproteinaemia Familial hypobetalipoproteinaemia Familial high-density lipoprotein deficiency (Tangier disease) Familial hypercholesterolaemia Wolman disease and cholesteryl ester storage disease Gangliosidoses α-Galactosidase A deficiency (Fabry disease) Sulphatide lipidosis (metachromatic leucodystrophy) Chapter 3 Developmental and Inherited Liver Disease

show abstract

Fatal neonatal liver failure and mitochondrial cytopathy: An observation with antenatal ascites

Cited by 36 publications

References 10 publications

Transient Neonatal Liver Disease After Maternal Antenatal Intravenous Ig Infusions in Gestational Alloimmune Liver Disease Associated With Neonatal Haemochromatosis

Transient Neonatal Liver Disease After Maternal Antenatal Intravenous Ig Infusions in Gestational Alloimmune Liver Disease Associated With Neonatal Haemochromatosis

Fatal neonatal mitochondrial cytopathy with disseminated fatty nodules in the liver

Developmental and Inherited Liver Disease

Contact Info

Product

Resources

About