presentation after taking certain protein or carb, infection Although IEM may be included in newborn screening tests, infants can present before the results are available.
Hypocal - often seen first few days of life, high phosphate level in cow’s milk formula, maternal or congenital hypoparathyroidism, maternal vit D deficiency, hypomagnesemia
Lip smacking, bicycling movements of the legs, tongue thrusting, apnea, and staring spells Electrolytes: hypoglycemia, hypo/hypernatremia, hypocalcemia, hypomagnesemia Drug withdrawal: methadone, barbiturates CNS infection: including STORCH
Arrest in embryogenic gut rotation resulting in narrow mesenteric base Risk of bowel ischemia Ladd’s band procedure
* The DJJ is low and to the right of the normal location In the image on the left, the duodenal bulb is to left of the spine. In the image on the right, with malrotation, the duodenal bulb is overlying spine. * The proximal small bowel (jejunum) is in the right upper quadrant. * The cecum is in the upper and/or left abdomen. * The large bowel is in the left abdomen.
anoxic or stress event at birth
cyanosis due to R to L shunting Mottle or gray due to outflow obstruction CHF due to L to R intracardiac shunting
-cyanotic lesions & obstructive lesions = ductal dependent -The ductal dependent lesions are usually of 2 types: left-sided and right-sided obstructive lesions. -Left-sided obstructive lesions (aortic stenosis, coarctation of the aorta, interrupted aortic arch, hypoplastic left heart syndrome) present with signs of severe systemic hypoperfusion with pallor, mottling, decreased or absent pulses, severe metabolic acidosis, and cardiomegaly with pulmonary congestion on chest radiograph (CXR). -R-sided obstructive lesions (pulmonary atresia, severe pulmonary stenosis, tetralogy of Fallot, tricuspid atresia) present with severe cyanosis, metabolic acidosis, and decreased perfusion of the lung fields on CXR.
-Lesions with pulmonary overcirculation (atrial or ventricular septal defects, atrioventricular canal, truncus arteriosus, and partial anomalous pulmonary venous return) can present with evidence of congestive heart failure and respiratory distress; however, the deterioration in these infants is not as dramatic as with ductal dependent lesions. Congestive heart failure may also be due to supraventricular tachycardia
Symptoms similar to septic shock!!!!! In septic appearing infants, if there is no clinical improvement after resuscitation and treatment of septic shock … .. think of congenital heart disease !!!!!!!!!!!!!!!!! Diagnosis will be delayed: transferring to another facility for echo
home remedies: baking soda for colic, herbal tea for constipation or colic
-The most common is 21-hydroxylase deficiency resulting in the inability to convert progesterone to aldosterone or cortisol and causing an accumulation of testosterone. -Adrenal hyperplasia develops as a result of overstimulation by ACTH (which has no negative feedback from cortisol) -Deficiency of aldosterone with urinary salt wasting: classic electrolyte findings and cardiovascular collapse -Female infants classically present with ambiguous genitalia with varying degrees of virilization. Typically recognized in nursery -However, a male infant may only have a hyperpigmented scrotum and no other physical abnormalities and can only be recognized by appropriate laboratory evaluation and results of the newborn screen if available.
Earl y onset: 85% presents within 24 hours. Early-onset sepsis syndrome is associated with acquisition of microorganisms from the mother Maternal risk factors: PROM, maternal fever Meningitis: GBS 50%, E. Coli 25%, Rest < 10% each Late-onset sepsis syndrome occurs at 7-90 days of life and is acquired from the caregiving environment Pneumonia is more common in early-onset sepsis, whereas meningitis and bacteremia are more common in late-onset sepsis. Absence of fever or hypothermia doesn ’ t rule out Bulging fontanelle and nuchal rigidity are present in small number of patients
enterovirus (coxsackievirus, ECHO virus)
(cover gram neg bacilli or late onset)
-weight: lost 10% - level off by day 5-7, back to BW day 10-14, gain 20-30 g/day -Poor feeding, improper formula mixing, sweating during the feed (equivalent to stress test - if sweaty, poor suck, stops feeding after few minutes - consider CHF) -fever - although overwhelming infection can present with high, normal or low temperature
-most murmurs in neonates are pathologic: VSD, PDA, PS, Aortic stenosis -Abdominal distention nonspecific, tenderness may be difficult to evaluate, rigidity is worrisome, hepatomegaly - CHF
Give O2
-poor feeding, small glycogen storage, high metabolic demands in critically ill infants
Ampicillin treats gram positive, listeria and enterococcus. Gentamin treats gram negative and synergistic effect. Ceftriaxone in younger infants can cause biliary sludging. S. pneumo meningitis - add vanco to cover resistant strain
stress dose hydrocortisone
infants may not demonstrate pyuria when UTI is present, always obtain urine culture