Ped1 Flashcards
which vitamin is deficient in milk?
VitD and Vit A
X-ray in ricket?
Winding of metaphysis
Ricket treatment?
Vit D and Ca
Cause of Niemann-pick disease?
sphingomyelinase deficiency
AR
CM?
loss of motor milestone 2-6 month
Chery red macula
Hepatosplenomegaly
Hyporeflexia/areflexia
How to d/t from tay-sacks disease?
in Tay saks
b-hexosaminidase deficiency
No hepatosplenomegaly
Hyperreflexia
Biliary duct cyst?
cystic dilation of bile duct
single and extrahepatic form common
CM?
majority of patients <10 RUQ pain RUQ palpable mass Obstructive Jaundice with acholic stool Elevated Panc.Enzyme due to pancreatitis in old children or reflex of pan. enzyme
Diagnosis and Managment?
Diagnose with abdominal U/S Managment Tumor resection(relieve obstruction and reduce risk of cholangiocarcinoma transformation)
How to d/t from biliary atresia?
In biliary atresia, there will be Hepatomegaly Absent visible gallbladder Present with infancy (2-8) week No pancreatic enzyme elevation In U/S absent gall bladder/BD/CBD Intraoperative cholangiography is GS for diagnosis
Managment of BA?
Surgical hepato-portoenterpstomy (Kasai procedure)
Liver transplant
Breast milk jaundice?
A peak in the second week
Due to B-glucoronidase in milk-deconjugate intestinal bilirubin
Unconjugated hyperbilirubinemia
Neuroblastoma pathogenesis?
Neural crest origin
Involve adrenal medulla and sympathetic chain
CM?
Midian age < 2 Abdominal Mass Fever, weight loss Opsoclonus(rapid eye movt-Myoclonus(rapid extremity jerking movt) syndrome Periorbital echemosis Spinal metastasis
Diagnostic evaluation?
Increase catecholamine metabolite
Small, round blue cell tumor
N-myc gene amplification
Indications for non-contrast CT in pediatric TBI?
LOC
AMS
Foccal nurogic sighn
sever mechanism of head injury((fall(in-age <2,>0.9m in >2(fall >1.5m//MVC?high impact)
Non frontal scalp hematoma and palpable skul # in <2
Vomiting,S.Headache,basilar Skul # in age 2-18
Steeps of neonatal resuscitation?
Dry and stimulate
Clear suction
Provide warmth(skin to skin)
when to do first?
Term
Breath/cry
Appropriate tone
If above the APGAR will be 9
Ahy tracheal suctioning is not recommended?
Stimulate vagus–apnea/bradychardia
Indication for the cardiac monitor?
Bradycardia(<100)
Apnea
Gasping
The patient requires chest compression
Friedrick ataxia?
Autosomal resecive
Due to GAA repitision
Fraexine gene defect
CM?
Dysarthria LE weakness Loss of position and vibration(DCI) Loss of DTR(PRG injury) MSK(Kyphoscloliosis, High arched palate) High risk of Hypertrophic CM(Death by age 40)
Imaging?
Cervical spinal cord atrophy
Managment?
Supportive
Genetic testing for diagnosis and for family for feaucher risk
Not have disease-modifying drug