Developmental Anatomy and Anomalies of the Gastrointestinal Tract with Involvement in Major Malformative Syndromes

Jul 4, 2016 by in GASTROENTEROLOGY Comments Off on Developmental Anatomy and Anomalies of the Gastrointestinal Tract with Involvement in Major Malformative Syndromes

Feature Embryo Fetus Blastogenesis Organogenesis  Week 1 2 3 4 5 6 7 8 9 10 Bilaminar disc. Endoderm X           Yolk sac. Connecting stalk  X         Trilaminar disc   X         Early: midline…

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Hepatic Tumors in Childhood

Jul 4, 2016 by in GASTROENTEROLOGY Comments Off on 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…

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The Liver in Metabolic Disease

Jul 4, 2016 by in GASTROENTEROLOGY Comments Off on 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…

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Colitis in Infancy and Childhood

Jul 4, 2016 by in GASTROENTEROLOGY Comments Off on 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…

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Enteropathies Associated with Chronic Diarrhea and Malabsorption in Childhood

Jul 4, 2016 by in GASTROENTEROLOGY Comments Off on 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…

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Intestinal Motor Disorders

Jul 4, 2016 by in GASTROENTEROLOGY Comments Off on 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…

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Diseases of the Biliary Tree

Jul 4, 2016 by in GASTROENTEROLOGY Comments Off on 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…

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