Eosinophilic Allergic proctocolitis Parasitic infection Ischemic Necrotizing enterocolitis Henoch-Schonlein purpura Hemolytic-uremic syndrome Hirschsprung-associated enterocolitis (late) Neutropenic colitis Acute Infections Hirschsprung-associated enterocolitis (early) Pseudomembranous Antibiotic associated (C. difficile) Other infections Hirschsprung-associated…
Series Time period Age range Total number of tumors Gastrointestinal tumors Finnish Cancer Registry (Teppo et al. 1975) 1953–1970 0–15 2,605 33 intestinal cancers, site not specified, 15 of these…
Neonate Infant Child I Congenital transport and enzymatic deficiencies (A) Carbohydrates Glucose–galactose malabsorption Disaccharidase deficiency Trehalase deficiency Congenital lactase deficiency Sucrase–isomaltase deficiency Fructose malabsorption (B) Lipids Abetalipoproteinemia/hypobetalipoproteinemia Chylomicron retention…
Usual presentationa Disorder Additional clinical features Inheritance Prenatal/neonatal Megacystis-microcolon hypoperistalsis syndrome Markedly enlarged bladder, intestinal malrotation, short dysmotile colon; females more common than males Probably autosomal recessive Hirschsprung disease 20…
1. Cholestatic disease (a) EHBA 33.6 % (b) Alagille and nonsyndromic paucity 4.6 % (c) Sclerosing cholangitis 1.3 % (d) Familial cholestasis 2.5 % (e) Choledochal cyst 0.3 % (f)…
Neonatal hepatitis Cholestatic syndromes Peroxisomal disorders Idiopathic NH PFIC Zellweger Viral NH Type 1 Byler P-type ATPase Infantile Refsum CMV Type 2 canalicular Bile Acid Tx Other enzymopathies Herpes Type…
Histologic features Neonatal hepatitis Obstruction Giant-cell transformation Usually diffuse Usually focal Lobular disarray Usually marked Usually mild Bile duct proliferation Usually absent, focal ductular reaction can be present Prominent and…
Fig. 9.1 Liver development. (a) 30-day embryo. The caudal portion of the hepatic diverticulum (HD) forms the extrahepatic biliary tree (green), while the cranial portion penetrates the septum transversum and…
Fig. 3.1 Cardia in a normal child (alcian blue PAS at pH 2.5). Notice that the surface columnar cells have a mixed staining pattern of magenta and blue indicating presence…
Primary clinical features Gene(s) Protein(s) Disorder(s) Cholangiopathy CIRH1A CIRH1A, cirhin; cirrhosis, autosomal recessive 1A North American Indian childhood cirrhosis CLDN1 CLDN1; claudin 1 Neonatal sclerosing cholangitis with ichthyosis and alopecia…
