Paediatrics Flashcards
What is the commonest Congenital heart disease?
VSD
Which congenital heart disease is associated with Turner?
CoA
Which congenital heart disease is associated with Down Syndrome?
ASD
What is the presentation of VSD?
Holosystolic murmur
LLSB
What is the presentation of ASD?
Systolic ejection murmur
USB
What is the treatment of ASD/VSD?
Small: spontaneous closure Surgical management if - failed medical management - pulmonary HTN - large defects
What is the presentation of a PDA?
Continuous machinery murmur
Pounding peripheral pulse
Wide pulse pressure
Where does the blood flow in a PDA?
Aorta to pulmonary artery
What is the treatment of a PDA?
Indomethacin (NSAID)
If indomethacin fails/child > 6-8mo: surgery
Come IN and CLOSE the door
Give INdomethacin to CLOSEE the PDA
What is the presentation of CoA?
High BP In upper extremities
Low BP in lower extremities
Weak femoral pulses
What are signs of CoA on CXR?
3 sign
Rib notching
What is the commonest cyanotic congenital heart lesion in the newborn?
Transposition of Great Vessels
When does Transposition of great vessels present?
Within first few hours after birth
What is the anatomy of transposition of great vessels?
Aorta on R ventricle
Pulmonary vessels on L ventricle
Need ASD/VSD and PDA to be compatible with life
What are maternal RF for Transposition of great vessels?
Diabetic mother
Mnemonic for DiGeorge Syndrome
Cardiac abnormalities (transposition) Abnormal facies (retrognathia, micrognatia, long face) Thymic aplasia Cleft palate Hypocalcemia 22q11 deletion
What is seen on CXR of transposition of great vessels/
Egg shaped silhouette
What is the treatment of transposition of great vessel?
IV PGE1
Surgical correction
Balloon atrial septostomy if cannot do surgery in first few days of life
What is the commonest cyanotic congenital heart lesion in childhood?
Tetralogy of Fallot
What is the anatomy of a tetralogy of fallot?
RV outflow tract obstruction
Overriding aorta
VSD
RVH
What is the presentation of a tetralogy of fallot?
Cyanosis in first 2 years of life but not immediately
SOB
Fatiguability
Get spell relieved by squat
What is seen on CXR of tetralogy of Fallot?
Boot shaped heart
What is the treatment of tetralogy of Fallot?
Immediate PGE1 to keep PDA open
Surgical
consultation
Tet spells” oxygen, morphine,
What are features of Edward Syndrome?
Rocker-bottom feet
Micrognathia
Clenched hands
Prominent occiput
What is the genetic mutation in cystic fibrosis?
Autosomal recessive
CFTR gene mutation on Kr 7
What is the newborn presentation of cystic fibrosis?
Obstruction of distal ileum
What is the > 1yo presentation of cystic fibrosis?
FTT
Chronic sinopulmonary disease/sputum production
Recurrent pulmonary infections
Digital clubbing
Chronic cough
Nasap polyps
Pancreatic insufficiency: greasy stools, rectal prolapse
Whar are CF patients at risk of?
Fat soluble vitamin deficiency secondary to malabsorption
What is the treatment of CF?
Antibiotics to cover Pseudomonas
Pancreatic enzymes and fat soluble vitamins
High calorie high protein diet
What is the age of onset of intussusseption?
6 mo - 3 yo
What is the presentation of intussusseption?
non-bilious vomit
red currant jelly stool (late sign)
colicky pain
sausage shape RUQ mass
What is the sign on U/S for intussusseption?
target sign
What is the diagnostic test if high clinical suspicion of intussusseption
Air insufflation enema
What are the associations of pyloric stenosis?
First born infant
Boy
Formula fed
Maternal erythromycin ingestion
What is the presention of pyloric stenosis?
Non-bilious vomit
After a feed
Projectile vomit
Palpable olive mass
What is the age of onset of pyloric stenosis?
3 weeks - 6 weeks
What laboratory finding for pyloric stenosis?
Hypochloremic hypokalaemic metabolic alkalosis
Treatment of pyloric stenosis?
Initially:
- NPO
- IV access
- Correct dehydration and acid-base abnormalities
- Pyloromyotomy
What is the cause of Meckel’s Diverticulum?
Omphalomesenteric duct fails to obliterate
What is the presentation of Meckel’s diverticulum and age of onset?
< 2 yo
Painless rectal bleeding
Usually incidental finding
Rule of 2 for Meckel
2% of population Symptomatic by age 2 2 x more common in boys 2 types of tissues" gastric, pancreatic 2 inches long 2 feet from iliocecal valve
Investigation of choice for Meckel?
Scintigraphy scan
Whar ia Hurschsprung disease?
Lack of ganglion cells in distal colon
Decreased motility due to unopposed smooth muscle tone
What diseases is Hurschsprung disease associated with?
Male
Down Syndrome
Waardenbur syndrome
MEN 2
Presentation of Hurschsprung disease?
Failure to pass meconium in first 48 hours of birth
On Physical exam: explosive stool after rectal exam
Lack of stool in rectum
Abnormal sphincter tone
Investigation of choice for Hurshsprung?
Barium enema
Rectal biopsy confirms diagnosis
Presentation of malrotation?
Bilius vomit
First month of life
What is the characteristic appearance of malrotation on imaging?
Bird beak appearance
Treatment of malrotation?
NG tube insertion to decompress intestine
IV fluid hydration
Emergent surgical repair
What are risk factors for necrotizing enterocolitis?
Low birth weight
Premature infant
Hypotension
Enteral feed: formula
What portion of the bowel undergoes necrosis in NEC?
Terminal ileum/proximal colon
Presentation of necrotizing enterocolitis?
First few days of life Feeding intolerance Delayed gastric emptying Abdominal distension Bloody stools
Can complicate to shock, intestinal perforation, abdominal erythema