Gastrointestinal & Nutrition Flashcards
What is the work-up for a newborn with conjugated hyperbilirubinemia and hepatomegaly?
Evaluate for biliary atresia!
- Abdominal ultrasound–> look for absent or abnormal gallbladder
Treatment:
Biliary Atresia
Kasai procedure (hepatoportoenterostomy)
Clinical Manifestation:
Pellagra
- diarrhea
- dermatitis
- dementia
- death (possibly)
Pathophysiology:
Pellagra
Niacin (vitamin B3) deficiency
Clinical manifestation:
- impaired adaptation to darkness
- photophobia
- dry scaly skin
- xerosis conjunctiva
- xerosis cornea
- keratomalacia
- bitot spots
- follicular hyperkeratosis of the shoulders, buttocks and extensor surfaces
Vitamin A deficiency
xerosis=dry; bitot spots=build up of keratin in conjunctiva
Clinical Manifestation:
- malnourished patient
- angular chelitis
- stomatitis
- glossitis
- normocytic-normochromic anemia
- seborrheic dermatitis
Vitamin B2 (riboflavin) deficiency
Meat, eggs, dairy products, yeast, green vegetables and other enriched foods are good sources of riboflavin.
Pathophysiology:
What causes the indirect hyperbilirubinemia seen in physiologic jaundice?
- increased bilirubin production
- decreased bilirubin clearance
- increased enterohepatic recycling
Treatment:
rapidly rising hyperbilirubinemia (as seen in physiologic jaundice)
Phototherapy is the gold standard of treatment for rapidly rising hyperbilirubinemia to prevent kernicterus.
What hereditary disease is responsible for almost all cases of meconium ileus?
Cystic fibrosis
What is the ONLY contraindication to breastfeeding?
If an infant is found to have galactosemia on newborn screening, it should not be breastfed.
Which conditions are breastfed babies at a lower risk of developing?
- otitis media
- respiratory, gastrointestinal and urinary tract infections
- necrotizing entercolitis
- type 1 diabetes mellitus
- childhood cancer
Clinical Manifestation:
- abdominal pain
- jaundice
- palpable mass
biliary cysts
What are biliary cysts?
Biliary cysts are congenital or acquired dilatations of the biliary tree. They may be single or multiple and extrahepatic or intrahepatic.
Treatment:
Biliary cysts
Surgical excision
Clinical manifestation:
- recurrent, self-limiting episodes of vomiting and nausea without an apparent cause
- Family history of migraine (often)
cyclical vomiting
Treatment:
cyclical vomiting
- anti-emetic agent
2. anti-migraine agent
Clinical Manifestation:
- poor feeding
- abdominal distension
- absent air in the rectum
Complete bowel obstruction
If these symptoms are present in a patient with Down syndrome think of Hirschsprung disease.
What should be at the top of your differential for a newborn who fails to pass meconium within 48 hours after birth?
Hirschsprung disease (symptoms= poor feeding, abdominal distension and absent air in the rectum)
This is especially at the top of your differential if the patient has Down syndrome.
Clinical manifestation:
- bilious vomiting within the first two days of life
- “double bubble” sign on abdominal xray
…in a patient with Down Syndrome
Duodenal atresia
Those signs alone are highly suggestive for duodenal obstruction!
Pathophysiology:
Why do cystic fibrosis patients commonly present with growth failure?
pancreatic enzyme deficiency –> steatorrhea and fat-soluble vitamin malabsorption–> poor weight gain
A pediatric patient with growth failure and recurrent respiratory infections should always raise your suspicion for…
Cystic fibrosis
Clinical Manifestation:
- nonbilious projectile vomiting
- olive-shaped abdominal mass
Infantile hypertrophic pyloric stenosis
What electrolyte imbalance is commonly seen in pyloric stenosis?
hypochloremic, hypokalemic metabolic alkalosis
Treatment:
Infantile hypertrophic pyloric stenosis
- IV rehydration
- normalization of electrolyte imbalance
- pyloromyotomy
Perform in this order. Correcting the electrolyte imbalance is important to reduce the risk of postoperative apnea.
Clinical Manifestation:
- generally well appearing infant
- intermittent cyanosis and distress when feeding, but relieved by crying
Choanal atresia
Diagnosis:
Choanal atresia
- failure to pass a catheter through the nose into the oropharynx
- CT scan showing narrowing in the posterior nasal cavity at the level of the pterygoid plate
Clinical Manifestation:
abdominal defect lateral to the umbilical cord with uncovered bowel herniating through the abdominal wall
Gastrocschisis
What serum protein is elevated in pregnant women bearing a fetus with gastrocschisis?
Maternal serum alpha-fetoprotein
What is the immediate workup for a stable patient with neonatal bilious emesis?
- X-ray
- Contrast studies (if and only if patient is stable)
X-ray will help you evaluate the need for emergency surgery or further diagnostic studies. Contrast studies help you determine the level of obstruction.
Diagnosis:
Intussusception
- positive “target sign” on ultrasound (first-line)
Treatment:
positive “target sign” noted on ultrasound
- reduction with air or water-soluble contrast enema
This is pathognomonic for intussusception!
Clinical Manifestation:
- well-appearing neonate
- painless bloody stools
Suspect (1) milk-protein proctocolitis or (2) soy-protein proctocolitis.
Treatment:
Milk-protein proctocolitis
- Eliminate dairy products from diet. The rectal bleeding should stop within 2 weeks.
Clinical Manifestation:
preterm neonate with…
- increased abdominal residual volume
- vomiting (feeding intolerance)
- abdominal distension
- bloody stools
Necrotizing entercolitis
Diagnosis:
X-ray findings in necrotizing entercolitis
- pneumatosis intestinalis (air visible in the bowel wall giving a “double line” or “tram track” appearance)
- portal venous air (linear areas of lucency seen over the liver)
Pneumatosis intestinalis is a hallmark x-ray finding for necrotizing entercolitis.
Whey vs. Casein
Which protein source is found in human milk and which is in formula?
Whey= human milk
Casein=formula
“Remember, human milk is the whey to go!”Also, whey is more easily digested and helps to improve gastric emptying.
What are the main risk factors associated with the development of necrotizing entercolitis?
- prematurity
- hypotension
- congenital heart disease
Clinical Manifestation:
neonate with
- bilious vomiting
- abdominal distension
suspect malrotation with midgut volvulus
Volvulus=bowel twisted around the mesentary attached to the gastrointestinal tract
What are the complications of untreated volvulus?
- frank bowel ischemia
- bloody stools
- perforation
Diagnosis:
Malrotation with volvulus
Upper gastrointestinal contrast study is the gold standard for diagnosis malrotation.
What subset of patients most commonly present with infantile hypertrophic pyloric stenosis?
first-born boys 3-5 weeks old
What OTC medication is contraindicated in young children with viral infections?
Aspirin (eg salicylates)
Aspirin in young children can precipitate Reye syndrome (vomiting, agitation and irrational behavior that progresses to lethargy, stupor and restlessness).
Clinical manifestation:
Newborn in the first week of life with
- unconjugated hyperbilirubinemia
- dehydration
Breastfeeding failure jaundice
Note, this can be differentiated from breastmilk jaundice which would NOT present with dehydration.
Pathophysiology:
Breastfeeding failure jaundice
inadequate milk ingestion–>inadequate stooling–> suboptimal bilirubin elimination –>increase enterohepatic circulation of bilirubin
Treatment:
Breastfeeding failure jaundice
- Lactation consultation
- Increase frequency of breast feeding
- supplement cow’s-milk base formula (if mother’s milk supply is inadequate)
DO NOT discontinue breastfeeding. Breastfeeding is the BEST form of nutrition for an infant.
Clinical Manifestation:
- macrosomia
- macroglossia
- umbilical hernia/omphalocele
- hemihyperplasia
- hypoglycemia
Beckwith-Wiedemann syndrome
Name two solid tumors associated with Beckwith-Wiedemann syndrome.
- Wilms tumor
2. Hepatoblastoma
List three common reasons children develop constipation.
- transition to cow’s-milk and solid foods
- toilet training
- school entry
What complications are associated with chronic constipation in children?
- anal fissures
- encopresis
- enuresis
- UTI
encopresis=involuntary defecation
Treatment:
Constipation
Laxatives
Initiate laxative therapy promptly to soften stools and prevent complications (anal fissures, encopresis, enuresis, UTI).
Treatment:
esophageal coins
- observe if patient is asymptomatic or ingestion occurred within the last 24 hours
- Immediate flexible endoscopy if patient is symptomatic or if time of ingestion is unknown
