Hepatic Tumors in Childhood
Texas Children’s Hospital Consultations HB 85 185 HCC 33 57 Fibrolamellar carcinoma 5 6 Cholangiocarcinoma 2 1 Undifferentiated sarcoma 18 31 Rhabdomyosarcoma 2 3 Mesenchymal hamartoma 14 26 Hemangioma…
Gastrointestinal and Hepatic Involvement in Immunodeficiencies and Systemic Disease of Childhood
Disease Proposed cause Selective IgA deficiency Impaired IgA synthesis; molecular defect unknown in most cases; mutation in TNFRSF13B in ~5 % Common variable immunodeficiency Impaired B cell maturation; molecular defect…
The Liver in Metabolic Disease
1. Nonimmune fetal hydrops 2. Antecedent history in a newborn infant of pregnancy complications such as HELLP 3. syndrome or fatty liver of pregnancy 3. Unexpected deterioration and death of…
Colitis in Infancy and Childhood
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…
Polyps and Tumors of the Gastrointestinal Tract in Childhood
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…
Enteropathies Associated with Chronic Diarrhea and Malabsorption in Childhood
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…
Intestinal Motor Disorders
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…
GI and Liver Transplantation Pathology in Childhood
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)…
Diseases of the Biliary Tree
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…
Hepatitis and Liver Failure in Infancy and Childhood
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…
