2022
DOI: 10.1016/j.dld.2021.09.011
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Diagnostic approach to neonatal and infantile cholestasis: A position paper by the SIGENP liver disease working group

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Cited by 18 publications
(13 citation statements)
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“…If our reconstruction of pathogenic events is correct, this newborn would be, to our knowledge, the first patient presenting HH with prenatal onset. The high incidence of the HFE genetic defect suggests that other infants with the same defect may have suffered from iron overload during intrauterine life but remained undiagnosed because HH is not usually included in panels for the differential diagnosis of neonatal cholestasis (2,4). In our patient, the neurologic outcome was characterized by the persistence of axial hypotonia, EEG, and MR abnormalities.…”
Section: Discussionmentioning
confidence: 84%
See 1 more Smart Citation
“…If our reconstruction of pathogenic events is correct, this newborn would be, to our knowledge, the first patient presenting HH with prenatal onset. The high incidence of the HFE genetic defect suggests that other infants with the same defect may have suffered from iron overload during intrauterine life but remained undiagnosed because HH is not usually included in panels for the differential diagnosis of neonatal cholestasis (2,4). In our patient, the neurologic outcome was characterized by the persistence of axial hypotonia, EEG, and MR abnormalities.…”
Section: Discussionmentioning
confidence: 84%
“…Other causes of conjugated bilirubin accumulation include anatomic abnormalities of the biliary system, in primis biliary atresia, genetic disorders, metabolic diseases, and α1-antitrypsin deficiency. Less frequently, cholestasis can occur because of infections, endocrine disorders, toxin and drug exposures, vascular malformations, or neoplasms (2).…”
Section: Introductionmentioning
confidence: 99%
“…To help this process, the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) published joint guidelines in 2017 [1]. In 2022, the Italian Society of pediatric gastroenterology, hepatology, and nutrition (SIGENP) liver disease group published guidelines on evaluation of neonatal cholestasis which was developed by pediatric hepatologists, surgeons, radiologists, neonatologists, a metabolic disease expert, pathologist, and geneticist [2 ▪ ]. We will briefly review both guideline papers and highlight their similarities and differences.…”
Section: Evaluation Of Neonatal Cholestasismentioning
confidence: 99%
“…A multicenter study concluded that duct/ductal bile plugs and portal stromal edema were the strongest independent histologic predictors of obstruction[ 17 ]. The sensitivity and specificity of histological findings are over 90%, although histopathological findings similar to those seen in BA may also be observed with other neonatal cholestasis diseases [such as alpha 1 antitrypsin deficiency, progressive familial intrahepatic cholestasis (PFIC), cystic fibrosis], though not necessarily in the early period[ 49 , 50 ]. The absence of an extrahepatic biliary tree in intraoperative cholangiography is, therefore, the leading indicator of BA[ 2 ].…”
Section: Biliary Atresiamentioning
confidence: 99%