Peds 7 Flashcards
What is bronchiectasis and a common cause in children
♣ Chronic infection of the bronchi causes permanent dilation of airways
Common cause = cystic fibrosis
The presence of what bacteria as a cause of pneumonia is an indicator of potential cystic fibrosis?
Psuedomonas
Will patients with CF be hypo- or hyper- natremic and chloremic
Hyponatremic and hypochloremic due to excess loss of NaCl
Diagnose: child with anorexia, irritability, bleeding, petechiae, leg/jiont pain, hepatosplenomegaly, fever, lymphadenopathy
Acute lymphoblastic leukemia (most common childhood cancer)
Presents with very non-specific findings - often called the great imitator
How do you diagnose ALL
Examination of bone marrow, most commonly aspirated from the posterior iliac crest
Will show minimum of 25% blasts (normal marrow has <5%)
Tx of ALL
Combination chemotherapy
Describe immune thrombocytopenic purpura (ITP)
o IgG autoantibodies to GP2b3a
o Antibodies produced by plasma cells of spleen and antibody-bound platelets consumed by macrophages of spleen
Describe thrombotic thrombocytopenic purpura (TTP)
o Platelets used up in pathologic formation of microthrombi in small vessels
o Due to decreased ADAMTS13, enzyme that normally cleaves vWF for degradation
♣ No vWF degradation = abnormal platelet adhesion = microthrombi
What is pulsus paradoxus
• Decrease in amplitude of systolic BP by > 10 mm Hg during inspiration
o Usually when you inspire this decreases intrathoracic pressure and causes the ventricular septum to deviate to the L, causing a smaller LV and a normal but slight decrease in BP
o In pulsus paradoxus, there will be an exaggerated decrease in BP
What are some things that cause pulsus paradoxus
Seen in cardiac tamponade, asthma, obstructive sleep apnea, pericarditis
Treatment of acute asthma exacerbation
O2, SABA, systemic prednisone
Risk factors for SIDS
♣ Prone or side sleep position, sleeping on a soft surface, bed sharing, pre- and postnatal exposure to tobacco smoke, maternal prenatal use of opiates, overheating, late or no prenatal care, young maternal age, prematurity and/or low birth weight, male gender
What are the heart defects that cause early cyanosis
R to L shunt
The 5 T’s o Truncus arteriosus (1 vessel) o Transposition (2 switched vessels) o Tricuspid atresia (3 = tri) o Tetralogy of Fallot (4 = tetra) o TAPVR (5 letters in the name)
What are the heart defects that cause late cyanosis
L to R shunts (late cyanosis caused by Eisenmenger syndrome)
- Atrial septal defect
- Ventricular septal defect
- Patent ductus arterosus
Obstructive defections
- Pulmonic stenosis
- Aortic stenosis
- Coarctation of the aorta
What two things does the ductus arteriosus connect?
Aorta and pulmonary artery
What heart condition typically causes bounding pulses
Aortic regurg
Describe fixed wide splitting of S2 and what defect it is seen in
- Occurs during right heart overload (e.g. atrial septal defect)
- ASD -> L-to-R shunt -> increased RA and RV volumes -> increased flow through pulmonic valve such that, regardless of breath, pulmonic closure is delayed
Describe how coarcation of the aorta can be “ductus dependent”
• If coarctation is proximal to ductus arteriosus it is usually associated with PDA
• Presentation:
o Lower extremity cyanosis in infants – blood going to LE is deoxygenated (from RV to pulmonary artery to PDA)
Symptoms often don’t appear until the ductus begins to close (can give prostaglandins to keep the ductus open until you can surgically repair)
Describe X-ray findings of Tetralogy of fallot
Boot-shaped heart due to RV hypertrophy
What is the immediate intervention for transposition of the great arteries
- Medications to reduce CHF (digoxin, diuretics, etc.)
- Atrial septostomy via catherization to allow for blood mixing until surgery is able to be performed
Why might a child with inflammatory bowel disease have anemia
- Microcytic = Iron deficiency anemia due to blood loss
OR - Macrocytic = B12 deficiency due to chronic inflammation of the terminal ilium (where B12 is absorbed)
What part of the gut is B12 absorbed in
Ileum
Describe pernicious anemia
B12 deficiency due to autoimmune destruction of gastric mucosa (parietal cells in the stomach), which leads to intrinsic factor deficiency
What are the 2 main concerns in feeding newborns goat milk
- Vitamin B12 deficiency
- Brucellosis if milk in unpasteurized
What heart defect presents as narrow mediastinum on CXR ““egg on a string”
Transposition of the great vessels
Presentation of lead poisoning
• GI (abd pain, constipation, anorexia)
• Neuro (cognitive defects, peripheral neuropathy, encephalopathy, memory loss, delirium)
o *Wrist/foot drop
• Hematologic (microcytic anemia with basophilic stippling, ringed sideroblasts in marrow)
• Burton lines – lead lines in gingiva and gums
• Hyper dense lines on metaphysis of long bones
• Renal failure
What does the ductus venosus connect
Allows blood to bypass the liver and flow from the umbilical vein directly into the IVC
Descrieb fetal circulation
o Umbilical vein carries oxygenated blood from placenta to liver
♣ In the liver there is mixing of oxygenated blood from umbilical vein with deoxygenated blood from lower extremities
♣ Some blood from umbilical vein goes to liver into hepatic circulation while some blood is shunted directly into the IVC via ductus venosus, bypassing hepatic circulation
o Blood goes from IVC to RA
♣ In the RA, blood can either go into the RV or straight into the LA via foramen ovale
o Blood that went to the RV then enters the pulmonary artery
♣ In the pulmonary artery, blood can either go to the lungs, or go through the ductus arteriosus which will shunt the blood directly into the descending aorta
• This shunt is due to the high fetal pulmonary artery resistance
Describe transition from fetal circulation to adult circulation
♣ When the infant takes a breath, this decreases intrathoracic pressure, thus decreasing resistance in pulmonary vasculature
♣ Decreased resistance leads to more blood entering the pulmonary artery (less leaving through ductus arteriosus), and thus more blood entering the LA
♣ Increased LA pressure causes closure of the foramen ovale
♣ Highly oxygenated blood in the aorta causes closure of ductus arteriosus
What is murmur you hear in ASD
Pulmonary flow murmur - due to increase volume crossing the pulmonary valve
Flow across ASD does not create a murmur because there is only a very small difference in pressure between RA and LA
What other heart sound will you hear in ASD
Fixed, widened split S2
Tx of truncus arteriosus
- Requires surgical repair in neonatal period
* Don’t need prostaglandin before repair
Tx of transposition of great vessels
- Medications to reduce CHF (digoxin, diuretics, etc.)
- Atrial septostomy via catherization to allow for blood mixing until surgery is able to be performed
- Surgical repair necessary
Describe tet spells and what defect they occur in
Occur in tetralogy of fallot
• Cyanosis exacerbated by increased R-to-L shunt due to crying, fever, exercise
• Symptoms relieved by squatting
o Increased SVR = R-to-L shunt becomes L-to-R shunt = improved cyanosis
What is total anomalous pulmonary venous return
- Pulmonary veins drain into R heart circulation (e.g. SVC, coronary sinus, RA)
- Associated with ASD and sometimes PDA to allow for R-to-L shunting
Coarctation of the aorta is associated with what syndrome
Turner Syndrome
Truncus arteriosus is associated with what syndrome
Down Syndrome
What is the major cause of mortality in DKA
Cerebral edema
Can occur when DKA treated with too much insulin, bicarb, and insulin
Are younger kids more likely to get T1DM or T2DM
T1DM
What may cause central precocious puberty?
Hypothalamic hamartoma
Treatment of central precocious puberty
Continuous leuprolipde
Consequences of untreated precocious puberty
Premature height maturation - 2 year old that finishes puberty will stop growing at that height
Diagnose: Thelarche (early breast bud development), bone age not advanced, no adrenarche
- Idiopathic isolated premature thelarche
- Ovarian cyst
Diagnose: + Thelarche (early breast bud development), + bone age advanced, no adrenarche
McCune Albright Syndrome (café au lait spots, polyostotic fibrous dysplasia, precocious puberty)
Diagnose: Bone age advanced, +adrenarche, no thelarche
- Adrenal tumor – get adrenal US
- Late-onset congenital adrenal hyperplasia – get 17-hydroxyprogesterone and androgens to begin
Describe pathogenesis of PCOS
o Increases LH -> stimulation of theca cells -> increased androgen production by theca cells -> increased peripheral conversion of androgens into estrone in adipose tissue -> decreased FSH (negative feedback) -> decreased stimulation of granulosa cells -> degeneration of follicles -> cystic follicles
What is metabolic sydrome
o Must have any 3 of the following:
♣ Abdominal obesity: Waist circumference > 40 in in men or > 35 in in women
♣ Triglyerides > 150 mg/dl
♣ HDL < 40 mg/dl in men or < 50 mg/dl in women
♣ BP > 130/85
♣ Fasting serum glucose > 100 mg/dl (or 2 hour post oral glucose > 140 mg/dl)
