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