Alterations of the Digestive Function in Children

1. Alterations of the Digestive Function in Children

2. Congenital Impairment of Motility • Cleft lip • Cleft palate • Esophageal Malformation • Pyloric Stenosis • Intestinal Malrotation • Meconium Ileus • Distal Intestinal Obstruction • Obstruction of the Duodenum, Jejunum, Ileum • Congenital Aganglionic Megacolon • Anal-rectal Malformations

3. Cleft lip and Cleft Palate • Cleft lip is the incomplete fusion of the nasomedial or intermaxillary process. It is a deformity usually beneath the center of one or both nostrils. • Cleft palate is often associated with cleft lip but may occur alone.

4. A and B show variations of cleft lip. C and D show variations of cleft palate.

5. Risk factors/causes: Cleft lip and palate Multiple gene-environment interactions including maternal alcohol and tobacco use, diabetes, and genetic variations. The cleft can be part of a single mutant gene or part of chromosomal defect. Clinical Manifestations: Feeding may be difficult depending on the severity of defect and if infant is able to show a productive suck. Specialized nipples and orthodontic prosthesis for the roof of the mouth are an option.

6. Esophageal Malformations • Esophageal atresia is a condition where the esophagus ends in a blind pouch. This is often seen with a fistula between the esophagus and trachea.

7. Risk factors/causes: Esophageal Malformations Thought to arrive from impaired differentiation as the trachea separates from the esophagus during the 4th-6th week of embryonic development, concurrent anorectal malformation. Clinical manifestations: Drooling and occasional aspiration as blind end of pouch fills and overflows into pharynx. If there is a fistula feeing and breathing may be problematic.

8. Pyloric Stenosis • One of the most common disorders of early infancy. • It is an obstruction of the pyloric sphincter caused by hypertrophy of the sphincter muscle.

9. Pyloric stenosis continued… • An infant who is well fed and gaining weight begins to vomit for no apparent reason. Some evidence shows higher risk in infants with familial history of pyloric stenosis.

10. Risk factor/causes: Pyloric Stenosis Maternal stress related factors (increased gastric secretion in last trimester), white male, exogenous administration of prostoglandin E, Downs syndrome, and genetic relative with pyloric stenosis. Clinical manifestations: Projectile vomiting (increasingly forceful), prolonged retention of food in the stomach, constipation, occasionally fluid and electrolyte issues, weight loss, and death.

11. Intestinal Malrotation • A condition that occurs in embryonic development where the normal rotation of the ileum and cecum is altered so that the colon remains in the URQ. The small intestine lacks the normal attachment and can twist (causing volvulus) leading to infarction and necrosis of part of the intestine.

12. Clinical manifestations: Intestinal Malrotation Volvulus and infarction, bile stained nausea/vomiting after feedings, abdominal distention, dehydration and electrolyte imbalance, fever pain, and bloody stools.

13. MeconiumIleus • Meconium is a thick sticky substance that fills a newborns entire intestine and is normally passed during the first few days after birth. Ileus occurs when abnormally sticky meconium causes obstruction in the small intestine.

14. Risk factor/causes: Meconium Ileus Cystic fibrosis, premature birth, maternal hydramnios Clinical manifestations: Abdominal distention, vomiting, possibly pulmonary involvement (especially with CF, hyperactive perisalsis, firm movable masses.

15. DIOS (Distal Intestinal Obstructive Syndrome) • Intestinal contents become abnormally thick and impact intestinal lumen. • Affects 15% of people with cystic fibrosis Consider: 31 y/o patient with CF dx with DIOS who in 24h had 5 doses of GoLYTELY, 400mg docolax PO, 200mg senna PO, and 34g polyethylene glycol 3350 PO with no change in bowel patterns.

16. Risk factor/causes: DIOS Cystic fibrosis Clinical manifestations: Abdominal distention, pain, nausea, vomiting.

17. Obstruction of the Duodenum, Jejunum, and Ileum • Caused by internal malformation or external pressure

18. Risk factor/causes: Obstruction small intestine Failure of duodenum to function, peritoneal band contrition of duodenum, ileus, pancreatic defect, hHirschsprung, Meckel diverticulum, hernia. Clinical manifestations: Pain, nausea, vomiting, distention

19. Congenital Aganglionic Megacolon • Functional obstruction of the colon caused by inadequate motility. The most distal part of rectum is always involved. Increased incidence in males, Hirschsprung disease and Down syndrome.

20. Risk factor/causes: Megacolon Hirschsprung disease, inadequate motility, complex inheritance factors, male, Downs syndrome. Clinical manifestations: Constipation, diarrhea, impaction, edema, ischemia, infarction of mucosa, sepsis, fever, vomiting, death.

21. Anorectal Malformation • General category for several conditions that may obstruct the passage of stool including: -anal stenosis -anal or rectal agenesis -atresia -fistula • Complete obstruction are known as imperforate anus

22. Risk factor/causes: Anorectal Malformation Other developmental anomalies, Down syndrome, congenital heart disease, renal issues, esophageal atresia, cryptorchidism, malformation of the spine. Clinical manifestations: Anal stenosis, bowel incontinence.

23. Aquired Impairment of Motility • Intussusception • Gastroesophageal Reflux (GER)

24. Intussusception • Intussusception is the invagination of one portion of intestine into another. Sudden adominal pain, vomiting, & possibly bloody stools. If untreated almost always fatal.

25. Risk factors/causes: Intussusception Male under 2 years, all ages abdominal surgery, cystic fibrosis, bowel obstruction Clinical manifestations: Sudden pain, irritable (draws up the knees), vomiting, dark/gelatinous stool, tender sausage shaped abdominal mass.

26. Gatroesophageal Reflux • Involves dilation of esophagus with intrusion of acid contents. Increased incidence in children with neurological impairment, cerebral palsy, and cystic fibrosis. Eighty-five percent of affected infants vomit excessively.

27. Risk factors/causes: GER Premature infants, cerebral palsy, reactive airway disease, cystic fibrosis. Clinical manifestations: Excessive vomiting (sometimes forceful), aspiration pneumonia, chronic cough, failure to thrive, pain, bleeding, iron deficiency.

28. Impairment of Digestion, Absorption, and Nutrition • Cystic Fibrosis • Gluten-Sensitivity Enteropathy • Protein Energy Malnutrition • Failure to Thrive • Necrotizing Enterocolitis

29. Cystic Fibrosis (CF) • Hallmark triad: 1) Pancreatic enzyme deficiency 2) overproduction of mucus 3) abnormally elevated sodium and chloride concentrations • Once only a childhood disease many people are surviving into adulthood • Secondary problems include maldigestion, diabetes, frequent infections, chronic obstructive pulmonary disease, cirrhosis, bowel issues

30. Risk factors/causes: CF Genetically inherieted mutation of the long arm of chromosome 7 Clinical manifestations: Overproduction/retention of mucus, hyponatremia and hypocloremia, nutritional deficiencies, diabetes, pulmonary complications, intestinal complications, frequent infections.

31. Gluten-Sensitive Enteropathy(formerly called Celiac disease) • Loss of mature villious epithelium caused from eating gluten (protein in wheat, rye, barley, and oats). • Cellular and humoral immunity are thought be play a role.

32. Risk factors/causes: Gluten-Sensitive Enteropathy Concurrent autoimmune diseases, white. Clinical manifestations: Diarrhea, pale, bulky, greasy, foul smelling stool, constipation, vomiting, pain, failure to thrive, malnutrition.

33. Failure to Thrive (FTT) • Organic FTT is caused from a known gastrointestinal source that causes nutritional deficiencies and inadequate physical development of an infant or child. • Nonorganic FTT occurs in the absence of any known gastrointestinal, endocrine or other chronic disease and often has behavioral and psychological roots.

34. Risk factors/causes: FTT Economic and psycho-social deprivation, GER, pyloric stenosis, intestinal parasite, chronic disease. Clinical manifestations: Slowed growth, reduced energy/interaction, stress, malnutrition.

35. Necrotizing Enterocolitis(NEC) Exact etiology unclear • If not treated bowel necrosis, perforation, and death can result. • Contributing factors: infection, immature immunity, maternal age<35, and perinatal stress. • Terminal ileum and proximal colon most frequently involved.

36. Risk factors/causes: NEC Prematurity, newborn full term infants (risk decreases as GI tract matures) Clinical manifestations: Abdominal distention, bowel perforation, sepsis, pain, temperature, bradycardia, apnea, bloody stool, elevated WBC, falling platelet counts, death.

37. Diarrhea • Acute • Chronic

38. Acute Diarrhea • Most commonly from viral or bacterial gastroenteritis. • Rotovirus is the primary cause of severe diarrhea in children.

39. Risk factors/causes: Acute diarrhea Bacterial gastroenteritis, antibiotic therapy, appendicitis, chemotherapy, inflammatory bowel disease, parasitic invasion, rotavirus, ingestion of toxin. Clinical manifestations: Dehydration, electrolyte issues, fatigue, skin breakdown from stool, abdominal pain, bloating, flatulence.

40. Chronic Diarrhea • Occurs with symptoms after 4 weeks • Most often caused by impaired motility, lactose intolerance, encopresis, parasitic infestation, antibiotic use, disease states that impair absorption

41. Risk factors/causes: Chronic diarrhea Acute diarrhea that has not been treated, increased prostaglandin synthesis, family history, irritable bowel syndrome, lactose intolerance. Clinical manifestations: Dehydration, electrolyte issues, fatigue, skin breakdown from stool, abdominal pain, bloating, flatulence.

42. Disorders of the Liver • Neonatal Jaundice • Biliary Atresia • Hepatitis • Cirrhosis • Portal Hypertension • Metabolic Disorders

43. Disorders of the Biliary Metabolism and Transport • Neonatal Jaundice • Biliary Atresia

44. Neonatal Jaundice • Neonatal jaundice is typically a temporary benign icterus from a high level unconjugated hyperbilirubinemia. • Pathologic jaundice can cause brain damage. Premature infants are at risk.

45. Risk factors/causes: Neonatal jaundice Premature infants as well as full term (initially transient and benign). Clinical Manifestations: Yellowish discoloration to tissue, increased bilirubin values, if not treated-respiratory distress, acidosis, sepsis, encephalopathy, athetoid cerebral palsy, speech and hearing impairment.

46. Biliary Atresia • Rare congenital malformation where there is an absence or obstruction of intrahepatic or extrahepatic ducts. • Jaundice, hepatomegaly, and alcoholic (clay colored) stool are primary signs. • Cirrhosis and liver failure lead to death if untreated.

47. Risk factor/causes: Biliary Atresia Congenital chromosomal abnormality Clinical manifestations: Jaundice, bile duct destruction, biliary cirrhosis, portal hypertension, liver failure, hepatomegaly, acholic stool, death.

48. Inflammatory Disorders • Hepatitis • Cirrhosis

49. Hepatitis • Hepatitis A in children account for all reported cases. Normally self limiting with full recovery. • Hepatitis B occur in infants from mothers who carry the hepatitis B surface antigen, hemophiliacs, IV drug users, institutionalized children. • Hepatitis C is caused mostly from blood transfusions. • Chronic Hepatitis unknown etiology; possibly autoimmune deficiency. • Interferon has been effective in treating Hepatitis B and C (and managing some chronic hepatitis.

50. Risk factor Clinical Manifestation Hepatitis A Preschool age, poor hygiene nausea, vomiting, diarrhea Hepatitis B drug users, hemophilia n/v/d, cirrhosis, institutionalized children liver cancer Hepatitis C blood transfusions n/v/d, chronic liver disease Chronic Hepatitisautoimmune related malaise, anorexia, fever, GI bleeding, hepatomegaly, edema, pain, liver impairment impairment