Unit 3 - Chapter 43 alterations of digestive fx in children

Question 1

cleft lip and cleft palate

Answer 1

• may occur separate or together
• both defects associated with multiple gene-environmental interactions and deficiency of b vitamins
• fissue may affect uvula, soft palate (back of top palate), hard palate (front top palate), nostril, maxillary alveolar ridge (gums above teeth)

Question 2

nonsyndromic (isolated) clp (cleft lip and/or cleft palate)

Answer 2

• most common malformation with incomplete separation between nasal and oral cavities without any associated anomalies

Question 3

esophageal atresia

Answer 3

• condition which esophagus ends in blind pouch
• may occur with or w/o trachoesophageal fistula (a connection between the esophagus and the trachea in bottom half) while esophageal atresia stops in blind pouch
• when infant swallows oral secretions or ingests milk, pouch fills l/t drooling or aspiration into lungs

Question 4

infantile hypertrophic pyloric stenosis

Answer 4

• obstruction of pyloric outlet d/t hypertrophy (increased growth of muscles) and hyperplasia (increased growth d/t reproducing cells) of circulat muscles in pyloric sphincter
• more commin in male infatns
• may require surgical corretion

Question 5

intestinasl malrotation

Answer 5

• occurs w/ colon failing to roate during fetal development
• an obstructing band or volvulus (twisting of bowel on itself) l/t partial or complete occlusion of GI tract and its blood vessels
• almost always require surgery

Question 6

meckel diverticulum

Answer 6

• outpouching of all layers of small intestine caused by failure of fibrous band that connects the small intestine to the umbilicus
• essentially leftover of the umbilical cord
• be repaired with surgery

Question 7

Hirschsprung disease

Answer 7

• aganglionic megacolonic
• A condition in which certain nerve cells are missing from the muscle layers of part of the large intestine. This causes severe constipation or blockage of the large intestine.
• d/t absence of enteric ganglia and malformatio nof parasympathetic nervous system in segment of colon
• pull-through surgery – bypass or remove part of colon lack nerve cells (pull healthy part to anus!)

Question 8

idiophatic intestinal pseudo-obstruction

Answer 8

• repetitive ep’s or continous sx of intestinal obstruction w/o an obstructing lesion and maybe d/t atrophy of enteric nerves and/or muscles
• colonoscopy to remove air
• fluids
• ng tube to remove air
• sometimes surgery

Question 9

DIOS (formerly known as meconium ilieus equivalent) or distal intestinal obstruction syndrome

Answer 9

• happens when intestinal content become abnormally thick and obstruct the intestinal
• Cystic fibrosis
• pancreatic enzymes deficiency
• dehydration
• above 3 are common causes

Question 10

cystic fibrosis

Answer 10

Cystic fibrosis is caused by carrying 2 variants of the gene for a protein called the cystic fibrosis transmembrane conductance regulator (CFTR), which regulates chloride, sodium, and bicarbonate transport across epithelial membranes. (abnormally elevated sodium and cl- concentrations in sweat)

The main complications involve the lungs, with damage to the small and large airways and chronic and recurrent bacterial infections, particularly by Pseudomonas aeruginosa. (mucus overproduction)

Other major consequences include pancreatic malfunction, leading to malabsorption of nutrients and vitamins with consequent impaired growth and development, and, in older patients, diabetes.

Airway clearance measures (eg, postural drainage, percussion, vibration, assisted coughing) are begun at diagnosis and done on a regular basis; regular aerobic exercise is recommended.

Drugs that correct or potentiate CFTR can help patients who have certain CFTR variants.
* Trikafta® (elexacaftor/tezacaftor/ivacaftor)
* Symdeko® (tezacaftor/ivacaftor)
* Orkambi® (lumacaftor/ivacaftor)
* Kalydeco® (ivacaftor)

Antibiotics are given early in any pulmonary exacerbation; drug selection may be based on culture and sensitivity testing.

Diet should be supplemented with pancreatic enzymes, high-dose vitamins, and 30 to 50% more calories derived primarily from fat.

Question 11

duodenal, jejunal, and ileal obstructions

Answer 11

• can be d/t
• meconium ileus (Meconium ileus (pronounced meh-COE-nee-um ILL-ee-us) means that a baby’s first stool (feces), called meconium, is blocking the last part of the baby’s small intestine (ileum). This can happen when the meconium is thicker and stickier than normal.)
• atresia (a birth defect that affects a part of the small intestine, the tube that connects the stomach to the large intestine and helps digest food. Depending on the extent of the blockage, the defect is classified as either atresia or stenosis)
• A peritoneal fibrous band – also known as Ladd’s band – can compress duodenum causing duodenal obstruction. Intestinal malrotation is a disease of newborn as it frequently manifests in the first month of life
• or acquried obstructive disorders

Question 12

imperforate anus

Answer 12

• malformations of anus and rectum from mild congenital stenosis of anus to complex deformities
• Imperforate anus or anal atresia is a congenital anorectal malformation (ARM) where a normal anal opening is absent at birth. ARMs comprise of a broad spectrum of defects ranging from minor (e.g., membranous covering) to complex cloacal malformations involving the urinary and genital tracts as well.

Question 13

meconium aspiration syndrome

Answer 13

• newborn respiratory distress caused by fetal inspiration of meconium-contaminated amniotic fluid associated with fetal hypoxic distress and in utero gasping respirations

1. Suctioning the baby’s upper airways, including the nose, mouth and throat.
2. Giving the baby supplemental oxygen by hood or mechanical ventilator.
3. Tapping on the baby’s chest to loosen secretions, a technique known as chest physiotherapy.
4. Antibiotics to treat infection.

• Meconium aspiration happens when a baby is stressed and gasps while still in the womb, or soon after delivery when taking those first breaths of air. When gasping, a baby may inhale amniotic fluid and any meconium in it. Babies are more likely to pass meconium when: They’ve had a long or hard delivery.

Question 14

meconium ileus

Answer 14

• newborn condition when intestinal secretions and amniotic waste products produce a thick tarry plug that obstructs the intestine, usually d/t lack of fetal digestive enzymes
• 10-15% off neonates with CF have this condition
• Enema
  May include n-acetylcysteine (drug that breaks down and softens thick meconium to allow it to pass)
• Sometimes surgery

Question 15

GERD

Answer 15

• d/t relaxation or incompetence of lower esophageal sphincter
• infants susceptible to reflux d/t non mature sphincter, liquid diet, and being only seldom in an upright position
• Acid-blocking medications include cimetidine (Tagamet HB), famotidine (Pepcid AC) and omeprazole magnesium (Prilosec

Question 16

eosinophilic esophagitis

Answer 16

• eosinophilic (higher than normal level of eosinophils) inflammation with dysphagia and vomiting that can be associated with asthma and eczema
• PPI (omeprazole), protonix + steroids (fluticasone or budesonide)

Question 17

celiac disease

Answer 17

• immune mediated lifelong disease with genetic predisposition with loss of mature villous epithelium in presence of gluten-containing diet
• # l/t malabsorption and growth failure

Celiac disease involves an inflammatory response to gluten that causes villous atrophy and malabsorption.

People of northern European heritage are most often affected.

Suspect the diagnosis if the serologic markers anti-tissue transglutaminase antibody and anti-endomysial antibody are present and confirm the diagnosis with a small-bowel biopsy.

Instruct the patient to follow a gluten-free diet and replace any vitamin or mineral deficiencies.

Grains that contain gluten include:

• wheat
• barley
• rye
• triticale
• farina
• spelt
• kamut
• wheat berries
• farro
• # couscous
• most dairy products, such as cheese, butter and milk.
• fruits and vegetables.
• meat and fish (although not breaded or battered)
• potatoes.
• rice and rice noodles.
• gluten-free flours, including rice, corn, soy and potato flour.

Question 18

nonceliac gluten sensitivity

Answer 18

• wheat allergy w/o autoantibodies associated with celiac disease
• avoid gluten as much as poss

Question 19

severe acute malnutrition

Answer 19

• group of d/o d/t severe dietary deficiency of proteins (kwashiorkor), carbohydrates, or both (marasmus)
• starvation – shunted mental and physical development
• kwashiorkor occurs most ofen in toddlers who have stopped breast feeding and rely on high-carbohydrate diet
• All children with SAM should receive nutritional treatment. Nutritional treatment is based on the use of specialised nutritious foods enriched with vitamins and minerals: F-75 and F-100 therapeutic milks, and ready-to-use therapeutic food (RUTF).

Question 20

Failure to thrive

Answer 20

• inadequate growth of child
• d/t biologic, psychosoical, and environmental contributions l/t delayed growth + malnutrition

Question 21

necrotizing enterocolitis

Answer 21

• d/o in neonates (lower birth weight babies or premature infants)
• d/t sress and anoxia (no o2) of an immature bowel wall and immature intestinal innate immunity
• # bacteria invade mucosa and cause colitis, necrosis, intestinal perforation, sepsis, and death

bowel rest, gastric decompression, systemic antibiotics and parenteral (IV) nutrition. Infants with perforation are generally operated upon; however, there has been recent interest in primary peritoneal drainage as an alternative.

Question 22

acute diarrhea in infants and children

Answer 22

• d/t infection from contaminated food or water (and), can cause dehydration and electrolyte imbalances b/c fluid reserves are relativel small
• most common is bacterial or viral enterocolitis
• breastmilk, formula, then solid food once hydration restored
• AB and antidiarrheal not recommended

Question 23

chronic diarrhea

Answer 23

• longer than 4 weeks
• grwoth failure and delayed development possible
• avoid triggering foods and fluid replacement (bland foods for now!)

Question 24

lactose malabsorption and intolerance

Answer 24

• diarrhea
• d/t failure to produce lactase l/t osmotic diarrhea with ingestion of lacose containing dairy products

Question 25

physiologic jaundice

Answer 25

• newborn
• mild hyperbillrubinemia that subsides in 1-2 weeks

Question 26

pathologic jaundice

Answer 26

severe hyperbillirubinemia and can cause brain damage (kernicterus)
* levels cross 25 mg/dl unconjugated billirubin
* light therapy, blood tranfusions exchange, and ivig or intravenous (immunoglobulins) for Rh disease
* yellowing of skin and eyes
* In addition to jaundice, signs of kernicterus may include:

• Lack of energy (lethargy).
• Fever.
• Vomiting.
• Involuntary muscle movements.

There are three stages of kernicterus, each with different symptoms.

• Early stage: Difficulty feeding and sucking, very sleepy, low muscle tone and no response to loud sounds (startle reflex).
• Middle stage: Irritability, a loud or high-pitched cry and tense muscles (a high muscle tone).
• Late stage: Not feeding, stiff muscles (arched back with your neck bent backward) and seizures.

Question 27

biliary atresia

Answer 27

• congenital malformation of ible ducts taht obstruct bile flow
• atresia causes jaundice, billary cirrhosis (When the bile ducts become swollen or inflamed, this blocks the flow of bile. These changes can lead to scarring of the liver called cirrhosis. This is called biliary cirrhosis. Advanced cirrhosis can lead to liver failure.), portal HTN, liver failure
• liver transplantation common

Question 28

acute hepatitis

Answer 28

• children have milder cases
• hep a most common amongst children and spread via stool and poor handwashing

Question 29

chronic hep b or hep c

Answer 29

young children more readily develop them d/t immature immune system, can be acquired by maternal vertical transmission in newborns or by parenteral (shared needles) transmission in older hildren

• First-line, antiviral therapy with tenofovir (TDF/viread) is recommended starting from week 28 of pregnancy until delivery but may continue 3 months postpartum. Please talk to your doctor about your own test results.
• hep c no tx

Question 30

cirrhosis

Answer 30

• fibrotic scarring of liver
• rare in children
• can develop from most forms of chronic liver disease

Question 31

portal HTN

Answer 31

• extrahepatic obstruction (usually) – lack of bile transit into the duodenum due to blockage along the common bile duct (CBD) or at its junction with the duodenum.
• thrombosis of portal vein is most common cause in children
• splenomegaly most common sign

Question 32

3 most common metabolic d/o that cause liver damage in children

Answer 32

1) Galactosemia is a rare, hereditary disorder of carbohydrate metabolism that affects the body’s ability to convert galactose to glucose. Galactose is a sugar contained in milk, including human mother’s milk as well as other dairy products. (enlarged liver, kidney failure, cataracts in the eyes or brain damage. If untreated, as many as 75% of infants with galactosemia will die.)
2) Hereditary fructosemia, also termed hereditary fructose intolerance, is an autosomal recessive deficiency of aldo-lase B (fructose biphosphate aldolase) in the liver, kidney, and intestines. Mutations occur in the aldolase B gene, located on chromosome 9q22. (the liver uses excess fructose to create fat, a process called lipogenesis. Eventually, people who consume too much fructose can develop nonalcoholic fatty liver disease, a condition in which too much fat is stored in the liver cells.)
3) Wilson disease is a genetic disorder that prevents the body from removing extra copper, causing copper to build up in the liver, brain, eyes, and other organs. Without treatment, high copper levels can cause life-threatening organ damage.

• all are rare, are inherited as genetic traits, and permit the accumulation of toxins in the liver.

Question 33

wilson disease

Answer 33

causes defective copper uptake and metabolism. Copper that is not excreted accumulates in the liver, brain, kidney, and corneal cells. Damage from accumulated copper is gradual; the disease is usually not diagnosed before age 4 or 5 years.