Analysis of the histologic features in the differential diagnosis of intrahepatic neonatal cholestasis

Table 1 Grading system used for histological parameters: cholestasis, eosinophilia in the inflammatory infiltrate, presence of giant cells, erythropoiesis, siderosis, and portal fibrosis

	Mild	Moderate	Severe
Cholestasis	Biliary pigment deposits in few hepatocytes of zone 3 acini	Hepatocytes with biliary pigment in two zone 3 acini associated with the presence of rare canalicular bilirubinostasis	The majority of the hepatocytes with biliary pigment biliary associated with several canalicular bilirubinostasis
Eosinophilia in the inflammatory infiltrate	Rare eosinophils in few space-porta	Some eosinophils in many space-porta and rare in the parenchyma	Many eosinophils in all the space-porta and several in the parenchyma
Presence of giant cells	Occurring in a maximum of 30% of the hepatocytes	Between 30% and 60% of the hepatocytes	> 60% of the hepatocytes
Erythropoiesis	Rare groupings of erythroblasts	Some groupings of erythroblasts	Groupings of erythroblasts and megakaryocytes
Siderosis	Deposits of ferric pigment in only a few Kupffer cells	Deposits of ferric pigment in Kupffer cells and a few hepatocytes	Deposits of ferric pigment in the majority of Kupffer cells and many hepatocytes
Portal fibrosis	Discrete widening of some space-porta	Widening of some space-porta	Widening of all space-porta

Table 2 Etiologies of intrahepatic neonatal cholestasis

Groups	Etiology	Number of cases
1	Neonatal sepsis	6
	Cytomegalovirus	6
	Urinary tract infection	3
	Syphilis	1
	Toxoplasmosis	2
2	Alpha1-antitripsyn deficiency	2
	Other metabolic diseases	6
	Galactosemia	2
	Alagille syndrome	2
	Byler’s disease	1
	Cystic fibrosis	1
	Secondary to use of parenteral nutrition	1
	Down’s sindrome	1
	Panhypopituitarism	2
3	Idiopathic	50
Total		86

Table 3 Clinical characteristics of the patients during the first evaluation, in accordance with groups 1 (infectious), 2 (genetic-endocrine-metabolic) and 3 (idiopathic)

	Group 1	Group 2	Group 3	P
Birth weight (g)	2160	2780	2750	0.014
	(SD = 650)	(SD = 594)	(SD = 767)
Weight during the first medical visit (g)	3040	3567	3970	0.105
	(SD = 1014)	(SD = 1170)	(SD = 1074)
Stature at birth (cm)	44.5	47	48	0.373
	(SD = 4.26)	(SD = 3.51)	(SD = 5.22)