Term
| T/F Digital clubbing commonly occurs with severe asthma. |
|
Definition
|
|
Term
| What is the definition of clubbing? |
|
Definition
| increase in angle between the nail and nail base of 180 degrees or greater and softening of the nail base to palpation |
|
|
Term
| What causes clubbing in children? |
|
Definition
| chronic pulmonary, cardiac, hepatic, or GI disease; rarely can be a familial condition |
|
|
Term
| What is the origin of the name cystic fibrosis? |
|
Definition
| describes characteristic pancreatitis findings |
|
|
Term
| What is the incidence of CF in whts, blks, and asians? |
|
Definition
| 1/3300 whts; 1/16,300 AA; 1/32,100 asians |
|
|
Term
| Most pts with CF develop bronchiectasis by age... |
|
Definition
|
|
Term
| Children with CF are commonly misdiagnosed as having... |
|
Definition
| asthma; 50% have airway reactivity but response to bronchodilator is unpredictable and varies |
|
|
Term
| What causes bacterial pneumonia in CF patients? |
|
Definition
| initally S aureus; then Pseudomonas aeruginosa |
|
|
Term
| Most pts with advanced CF harbor what pathogen? |
|
Definition
| heavy, slime-producing mucoid variants of P aeuroginosa rarely found in other conditions |
|
|
Term
| Rapid clinical deterioration in a pt with CF may be associated with recent colonization with... |
|
Definition
|
|
Term
| What are some pulmonary complications of advanced CF? |
|
Definition
| pneumothorax, hemoptysis, and cor pulmonale |
|
|
Term
| T/F CF causes both acute and chronic cases of sinusitis. |
|
Definition
| false; acute sinusitis is rare in CF |
|
|
Term
| What are the upper airway symptoms of CF? |
|
Definition
| nasal congestion, sinus opacification, nasal polyps with resultant nasal obstruction, headaches and mouth breathing |
|
|
Term
| What are the GI complications of CF? |
|
Definition
| maldigestion, abdominal distention, rectal prolapse, frequent passage of oily malodorous stools that predispose to obstruction, voluvulus, or intussusception, pancreatitis, deficiency of fat soluble vitamins (AEK esp) |
|
|
Term
| What are the liver complications of CF? |
|
Definition
| fatty liver infiltration or focal biliary cirrhosis occurs in many CF pts; hepatomegally, esophagealvarices, and hypersplenism caused by portal HTN develop in small number of teens; neonates may have prolonged jaundice |
|
|
Term
| What other gland besides the pancreas can cause clinical problems in CF pts? |
|
Definition
| they can have enlarged submaxillary glands |
|
|
Term
| What extremity problems occur in CF? |
|
Definition
|
|
Term
| What are some GU problems associated with CF? |
|
Definition
|
|
Term
|
Definition
| positive sweat test in conjunction with one of the following: typical chronic obstructive pulmonary disease, documented exocrine pancreatic insufficiency and/or a positive family history |
|
|
Term
| What conditions are associated with false positive sweat chloride test? |
|
Definition
| anorexia nervosa, hypothyroidism, and nephrogenic diabetes insipidus |
|
|
Term
| What can cause a false negative sweat chloride test? |
|
Definition
| if they have edema and hypoproteinemia |
|
|
Term
| When is genetic testing for CFindicated? |
|
Definition
| when CF is suspected but the results of sweat testing are negative or equivocal |
|
|
Term
| CF is caused by a mutation where? |
|
Definition
|
|
Term
| What is the most common CF mutation? |
|
Definition
| single phenylalanine deletion at aa delta 508 |
|
|
Term
| Available genetic tests can detect what percent of CF mutations? |
|
Definition
|
|
Term
| How does the NBS for CF work? |
|
Definition
| detects the pancreatic enzyme immunoreactive trypsinogen (IRT) |
|
|
Term
| What is the next step if a NBS detects high levels of IRT? |
|
Definition
| second teir testing relies on a second IRT test or DNA testing; infants with positive results on NBS undergo sweat chloride testing for definitive ocnfirmation |
|
|
Term
| What is the mean CF survival? |
|
Definition
|
|
Term
| What factors indicate a better CF prognosis? |
|
Definition
| males live slightly longer than females; children from socioeconomically disadvantaged homes have a poorer prognosis |
|
|
Term
| What electrolyte abnormality is c/w CF? |
|
Definition
| hyponatremic, hypochloremic alkalosis |
|
|
Term
| What's another term for Hirschsprungs? |
|
Definition
| congenital aganglionic megacolon |
|
|
Term
| Describe radiograph findings c/w meconium ileus? |
|
Definition
| distended bowel loops and a bubbly pattern in a portion of intestine; the colon is narrow |
|
|
Term
| Is meconium ileus treater urgently or emergently? |
|
Definition
| it is a surgical emergency |
|
|
Term
| ciliary dyskinesia + visceral situs inversus= |
|
Definition
|
|
Term
|
Definition
| severe pulmonary infections, chronic diarrhea, and wasting |
|
|
Term
| Meconium ileus is nearly pathognomonic for... |
|
Definition
|
|
Term
| What do youcall a reduction in total circulating leukocytes? |
|
Definition
|
|
Term
|
Definition
| a reduction in circulating erythrocytes, leukocytes and platelets |
|
|
Term
| What is the most common childhood malignancy? by what percent? |
|
Definition
| leukemia (approx 40% of all pediatric cancers) |
|
|
Term
| What are the two most common types of leukemia in children? |
|
Definition
| 75% of leukemia is ALL; and AML 20% |
|
|
Term
| What cell lines are affected by AML? |
|
Definition
| myeloid cell line (granulocytes, monocytes and can affect erythrocytes or megakaryocytes) and comprises approximately 20% of childhood leukemia |
|
|
Term
| What is the difference in clinical manifestation between ALL and AML? |
|
Definition
|
|
Term
| What is the peak age of ALL? |
|
Definition
|
|
Term
| ALL is more common in which gender? |
|
Definition
|
|
Term
| What are the symptoms of ALL? |
|
Definition
| anorexia, irritability, lethargy, pallor, bleeding petechiae, leg and joint pain and fever |
|
|
Term
| What are the PE findings of ALL? |
|
Definition
| bleeding, bruising, petechiae, pallor, pain upon palpating bones or joints and HSM |
|
|
Term
| What is included in a ddx if you suspect leukemia? |
|
Definition
| ITP, aplastic anemia, mononucleosis, JIA, and leukemoid reaction |
|
|
Term
| What's the difference btween ITP and leukemia? |
|
Definition
| anemia, leukocyte disturbances and HSM are absent in ITP |
|
|
Term
| What's the difference between ALL and aplastic anemia? |
|
Definition
| aplastic anemia will not usually have LAD, arthralgias, bone pain, and HSM |
|
|
Term
| How do you differentiate between ALL and infectious mono? |
|
Definition
| mono is characterized by atypical lymphocytes |
|
|
Term
| What test will definitively differentiate ALL from all other diagnoses? |
|
Definition
|
|
Term
| Besides leukemia, kids' bone marrow can be infiltrated by other malignant cells like... |
|
Definition
| neuroblastoma, rhabdomyosarcoma, Ewing sarcoma, retinoblastoma |
|
|
Term
| T/F ALL is characterized by leukocytosis in nearly all pts. |
|
Definition
| false; almost half of the children with newly diagnosed leukemia have total leukocyte counts less than 10,000 /mm3 |
|
|
Term
| A normal bone marrow contains what percent of blasts? |
|
Definition
|
|
Term
| What finding on bone marrow bopsy confirms the diagnosis of ALL? |
|
Definition
|
|
Term
| What percent of pts with ALL have karyotype or structural gene abnormalities of their leukemic cells? |
|
Definition
|
|
Term
| What gender, race and age is associated with better or worse prognosis? |
|
Definition
| girls have a better prognosis, AA and hispanic populations have lower remission and higher recurrence rates; children with ALL younger than 1 yoa and those older than 10 yoa have a worse prognosis |
|
|
Term
| What leukocyte count is associated with a poor ALL outcome? |
|
Definition
|
|
Term
| What type of cancer cells are associated with a worse or better prognosis in ALL? |
|
Definition
| mature B cell or T cell immunophenotypes atpically have a worse outcome compared to those with B precursor ALL |
|
|
Term
| Why it is it important to get karyotypes of leukemic cells of ALL? |
|
Definition
| the aryotype has diagnostic prognostic and therapeutic significance |
|
|
Term
| What karyotype abnormalities are associated with better or worse prognosis in ALL? |
|
Definition
| hyperdiploidy is more favorable; hypodiploidy and pseudodiploidy do less well; translocations with a poor outcome include t(9;22) and in pts with pre-b all |
|
|
Term
| What genetic abnormality is associated with a poor prognosis in pts with AML? |
|
Definition
|
|
Term
| Besides bone marrow biopsy, what else is involved in the workup for leukemia? |
|
Definition
| LP toexamine the CNS for early leukemic involvement, chest radiograph to detect a mediastinal mass; bone radiographs may show altered medullary trabeculation, cortical defects or transverse radiolucent lines |
|
|
Term
| What is the significance of altered appearance of bone on radiographs of pts with ALL? |
|
Definition
| there is no prognostic or diagnostic significance |
|
|
Term
| What are the steps of ALL chemo? |
|
Definition
| induction, consolidation, prophylactic CNS therapy, an dmaintenence |
|
|
Term
| What is induction therapy for ALL? |
|
Definition
| prednisone, vincristine and asparaginase |
|
|
Term
| How long does induction tehrapy for ALL take and how well does it work? |
|
Definition
| 4 weeks; 98% go into remission |
|
|
Term
| What is consolidation treatment for ALL? |
|
Definition
| mutliple chemotherapies in a relatively short period of time |
|
|
Term
| What does prophylactic CNS tx for ALL involve? |
|
Definition
| intrathecal cytarabine and/or methotrexate; +/- craniospinal irradiation |
|
|
Term
| Why is it important to prophylactically treat CNS for ALL? |
|
Definition
| reduces recurrence rate in CNS from 50% to 3-6% |
|
|
Term
| What drugs are mainentance therapy for ALL? |
|
Definition
| methotrexate and 6-MP, vincristine and prednisone X2-3 years |
|
|
Term
| WHen can you dc ALL maintenance treatment? |
|
Definition
| when children are in remission for 2 to 3 years |
|
|
Term
| What is the 5 yr survival rate of ALL? |
|
Definition
|
|
Term
| What are hte late effects of tx for ALL? |
|
Definition
| neuropsychological deficits, seizures, and endocrine disturbances (i.e. GH def), spermatogenesis dysfunction related to cyclophosphamide; delayed sexual maturation in boys who recieved irradiation of the gonadal tissue due to leukemic invasion of the testes; leukoencephalopathy and neurodevelopmental problems (esp in post CNS radiaiton pts) and secondary malignancies |
|
|
Term
| What inheritable disease are associated with a higher risk of leukemia? |
|
Definition
| klinefelter, bloom syndrome, fanconi syndrome, ataxia telangiectasia, and neurofibromatosis; Down's syndrome |
|
|
Term
| How much higher is the risk of developing ALL if you have Down's? |
|
Definition
|
|
Term
| What is the increased risk in siblings of pts with ALL? |
|
Definition
|
|
Term
| What should you do for vaccine schedule in pts with ALL? |
|
Definition
| live viruses arecontraindicated for pts with ALL and all members of the household during chemo and for at least 6 months after treatment; immuniziation with inactivated ivrus is relatively contraindicated because immunosuppression of chemo often inhibits antibiody response |
|
|
Term
| What is the best inital screening test to differentiate between JRA and leukemia? |
|
Definition
| CBC with diff and platelets (leukocyte and plateles are normal to increased in JIA) |
|
|
Term
| Thrombocytopenia + recent viral illness= |
|
Definition
|
|
Term
|
Definition
| observation; IVIG, anti-RhD, immunosuppresives/steroids |
|
|
Term
| In a pts presenting with low platelets its important to ask about... |
|
Definition
| recent MMR vaccination, drug ingestion, and HIV status |
|
|
Term
| What neonatal blood glucose level requires immediate PO glucose? IV glucose? |
|
Definition
| PO if level is 25 to 40; IV if it is less than 25 |
|
|
Term
| What findings on CMP/CBC are c/w IDM? |
|
Definition
| polycythemia, hypocalcemia, and hyperbilirubinemia |
|
|
Term
| What is the definition of gestational diabetes? |
|
Definition
| persistent hyperglycemia during pregnancy with unterated serum glucose levels greater than 100 mg/dL in the fasting state or greater than 130 mg/dL otherwise |
|
|
Term
| What is the definition of hypoglycemia? |
|
Definition
| traditionally a blood glucose less than 40 but other definitions exist |
|
|
Term
| What is the definition of macrosomnia? |
|
Definition
| exceeding the 90th percentile for gestational age |
|
|
Term
| What level of polycythemia is significant in a newborn and treated with partial exchange transfusion? |
|
Definition
|
|
Term
| What is caudal regression syndrome? |
|
Definition
| rare malformations found almost exclusively in IDMs, characterized by hypoplasia of the sacrum and lower extremities |
|
|
Term
| What percent of pregnancies are complicated by diabetes? |
|
Definition
|
|
Term
| When are women screened for gestational diabetes? |
|
Definition
|
|
Term
| How is gestational diabetes classified? |
|
Definition
| the White classification which stages it according to what stage of the pregnancy it was diagnosed, the duration of symptoms, and the presence of vasculopathy |
|
|
Term
| T/F Women who require insulin therapy are at higher risk for a poor perinatal outcome than those whose carbohydrate intolerance can be managed by diet alone. |
|
Definition
|
|
Term
| When does the fetal pancreas begin producing insulin? |
|
Definition
| during the fourth months of gestation |
|
|
Term
| When does the insulin producing ability of the pancreas become functionally significant? |
|
Definition
|
|
Term
| When can macrosomnia first be noted in IDM? |
|
Definition
| after week 26 when the secretion of fetal insulin becomes significant |
|
|
Term
| What causes increased weight and length of IDM? |
|
Definition
| hyperinsulinemia causes increased adipose tissue deposition and increased grwoth is due to the growth hormone effects of insulin |
|
|
Term
| Where is the excess glycogen stored in IDM? |
|
Definition
| liver, kidney, skeletal muscle and heart |
|
|
Term
| Does IDM cause a larger head circumference? |
|
Definition
| head circumference is less significantly affected because insulin does not affect brain grwoth; thus the weight of a IDMis typically in its shoulders and abdomen |
|
|
Term
| What predisposes IDMs to perinatal asphyxia? |
|
Definition
| macrosomnia,increased oxygen requirements, an dplacental insufficiency |
|
|
Term
| IDM leads to polycythemia which can lead to what complications? |
|
Definition
| elevated bilirubin levelsand renal vein thrombosis |
|
|
Term
| Hypocalcemia associated with IDM can lead to what complications? |
|
Definition
| irritibility or decreased myocardial contractility |
|
|
Term
| IDMs are at increased risk for congenital malformations such as... |
|
Definition
| congenital heart disease, neural tube defects and caudal regression syndrome |
|
|
Term
| T/F IDM are always larger infants. |
|
Definition
| false; they can be smaller if there is a significant amount of placental insufficiency |
|
|
Term
| What is class D pregestational diabetes? |
|
Definition
| insulin-dependent, with vascular disease |
|
|
Term
| What is class A pregestational diabetes? |
|
Definition
|
|
Term
| What is class B pregestational diabetes? |
|
Definition
| insulin-requiring but without vascular disease |
|
|
Term
| What is class Cpregestational diabetes? |
|
Definition
| insulin dependent but without vascular diseae |
|
|
Term
| What kind of heart abnormalities are found in IDM? |
|
Definition
| cardiomyopathy, septal hypertrophy, and subaortic stenosis |
|
|
Term
|
Definition
| renal vein thrombosis with hydronephrosis 2ndary to polycythemia; if meconium hasn't passed may be small left colon syndrome |
|
|
Term
| What are the symptoms of renal vein thrombosis in a newborn? |
|
Definition
| abdomenal mass, gross hematuria, microscopic hematuria, hypertension |
|
|
Term
| What are some risk factors for neonatal physiologic jaundice? |
|
Definition
| male gender, cephalohematoma, asian origin, and breast feeding are the main ones; also= maternal diabetes, prematurity, polycythemia, trisomy 21, cutaneous bruising, delayed bowel movements, upper gastrointestinal obstruction, hypothyroidism, swallowed maternal blood and a sibling with physiologic jaundice |
|
|
Term
| T/F Conjugated hyperbilirubinemia is not neurotoxic. |
|
Definition
|
|
Term
| Name some hereditary defects in bilirubin conjugation. |
|
Definition
| Crigler-Najjar syndrome; gilbert disease |
|
|
Term
| T/F Postasphyxia can cause jaundice? |
|
Definition
|
|
Term
| Conjugated bilirubin is bilirubin attached to... |
|
Definition
|
|
Term
| Kernicterus isdue to unconjugated bilirubin depositing in the... |
|
Definition
| basal ganglia, globus pallidus, putamen and caudatenuclei |
|
|
Term
| What are the symptoms of kernicterus? |
|
Definition
| lethargy, poor feeding, and loss of a Moro reflex |
|
|
Term
| What percent of babies get physiologic jaundice? |
|
Definition
| 60% of full term and 80% of preterm |
|
|
Term
| Full term newborns usually have peak bilirubin concentrations of ___ mg/dL and age ___> |
|
Definition
| 5 to 6; 2nd to 4th day of life |
|
|
Term
| What findings are suggestive of nonphysiologic jaundice? |
|
Definition
| appearance in the first 24 to 36 hours of life; bilirubin rate of rise greater than 5 mg/dL/24 hrs, bilirubin greater than 12 mg/dL in a ful lterm infant without other physiologic jaundice risk factors listed; and jaundice that persists after 10 to 14days of life |
|
|
Term
| What are some causes of nonphysiologic neonatal jaundice? |
|
Definition
| septicemia,biliary atresia, hepatitis, galactosemia, hypothyroidism, cystic fibrosis, congenital hemolytic anemia, drug induced hemolytic anemia |
|
|
Term
| What are causes of jaundice presenting within the first 24 horusof life? |
|
Definition
| erythroblastosis fetalis, hemorrhage, sepsis, cytomegalic inclusion disease, rubella, and congenital toxoplasmosis |
|
|
Term
| ddx of a infant in the first few days of life with lethargy and poor feeding followed by a gravely ill appearance with respiratory distress and diminished tendon reflexes= |
|
Definition
| kernicterus, sepsis, asphyxia, hypoglycemia and ICH |
|
|
Term
| What percent of breast fed full term infants develop significant unconjugated bilirubin elevations (breast milk jaundice)? |
|
Definition
|
|
Term
| When does breast milk jaundice peak and at what bili level? |
|
Definition
| peaks after the seventh day of life and concentrationsare up to 30 mg/dL |
|
|
Term
| What can be done to decrease bili levels in breast milk jaundice? |
|
Definition
| formula substitution for breast milk for 12 to 24 hours results in a rapid bilirubin level decrease; breast-feeding can be resumed wihtout return of hyperbilirubinemia |
|
|
Term
| What is the workup for significant hyperbilirubinemia? |
|
Definition
| indirect and direct bilirubin concentrations, hemoglobin level, reticulocyte count, bloodtype, Coombs test |
|
|
Term
| What does Coomb's test measure? |
|
Definition
| indirect measuresantibiodies to RBCs in the blood; direct coombs identifies antibodies o the surface of the infants RBCs |
|
|
Term
| When giving an infant phototherapy, what else should you automatically do? |
|
Definition
| make sure the eyes are sheilded and hydration is maintained |
|
|
Term
| At what bilirubin level do you start phototherapy in full term infants? |
|
Definition
| if 24 to 72 hours=16-18 mg/dL; if over 72 hours=20 mg/dL |
|
|
Term
| What are teh risks of exchange transfusion? |
|
Definition
| air embolus, volume imbalance, arrhythmias, acidosis, resipratory distress, electrolyte imbalance, anemia or polycythemia, blood pressure flucuation, infection and necrotizing colitis |
|
|
Term
| What conditions increase the risk of neurologic damage from jaundice by increasing the blood-brain barrier permeability to bilirubin? |
|
Definition
|
|
Term
| What condition reduces the infants ability to transport unconjugated bilirubin to the liver? |
|
Definition
|
|
Term
| What is the deficiency in Crigler-Najjar type 1 and 2? |
|
Definition
| deficiency in uridine diphosphate glucuronosyltransferase; type 1 is a severe deficiency; type 2 isa milderdeficiency in which bilirubin levels rarely exceed 20 mg/dL |
|
|
Term
| What is initial management of an asthma attack? |
|
Definition
| O2, inhaled beta agonist, systemic dose of prednisone; if in a lot of distress you can give IVfluids and meds; a stat blood gass determination and monitoring O2 sats will aid further management |
|
|
Term
| What are some signs of asthma attack with impending respiratory failure? |
|
Definition
| paucity of wheezes and drowsiness |
|
|
Term
| What arethe criteria for asthma? |
|
Definition
| episodic symptoms of airflow obstruction are present; airflow obstruction is at least partially reversible; alternativ ediagnoses are excluded |
|
|
Term
| Asthma exacerbations are characterized by a triad of... |
|
Definition
| bronchoconstriction, airway inflammation, and mucus plugging |
|
|
Term
| What is pulses paradoxus? |
|
Definition
| a blood pressure that varies greater than 10mmHg between inspiration and expiration which suggests obstructive airway diseaes, pericardial tamponade or constrictive pericarditis |
|
|
Term
| What is the median age ofonset for asthma? |
|
Definition
|
|
Term
| What percent of children with asthma develop symptoms in the first year of life? |
|
Definition
|
|
Term
| What are risk factors for development of asthma? |
|
Definition
| atopy and a family history of asthma are strong risk factors for its development as is respiraotry infections early in life |
|
|
Term
| What percent of children with RSV develop asthma later in life? |
|
Definition
|
|
Term
| What percent of patients with asthma have symptom resolution by young adulthood? |
|
Definition
|
|
Term
| What makes resolution of asthma by young adulthood less likely? |
|
Definition
| heavy exposure to pollution, allergens or cigarrette smoke |
|
|
Term
| How fast does the immediate response of asthma take? |
|
Definition
|
|
Term
| What are common triggersfor asthma? |
|
Definition
| dust mites, animal dander, cigarrette smoke, pollution, weather changes, upper respiratory infections, certain drugs (beta blockers, NSAIDS) and excercise |
|
|
Term
| How long doesit take for the late phase reaction to begin? |
|
Definition
| 2 to 4hours after the acute response |
|
|
Term
| Late phase reaction (LPR) is characterized by... |
|
Definition
| infiltration of inflammatory cells into the airway parenchyma |
|
|
Term
| T/F Airway responsiveness can persis forweeks after the LPR. |
|
Definition
|
|
Term
| What is pharmacotherapy for asthma? |
|
Definition
| beta adrenergic agonists, anticholinergics, antiinflammatory agents, leukotriene modifiers |
|
|
Term
| T/F Bronchodilators can be used prior to exercise or exposure to allergens to minimize the acute asthmatic resonse. |
|
Definition
|
|
Term
| What is the toxicity of beta agonists? |
|
Definition
| tachycardia and muscle tremor |
|
|
Term
| T/F Over-reliance on short acting inhalers can be associated with death in severe asthma attacks. |
|
Definition
|
|
Term
| Do anticholinergics work well for treating asthma? |
|
Definition
| they are useful for acute management of asthma exacerbation but are of little value in chronic therapy; they work by inhibiting the vagal reflex at smooth muscles |
|
|
Term
| How do cromolyn and nedocromil work? |
|
Definition
| anti-inflammatory drugs that act by reducing the immune response to allergen exposure |
|
|
Term
| How long does it take for cromolyn and nedocromil to becom effective? |
|
Definition
|
|
Term
| What percent of patients are nedocromil and cromolyn effective in? |
|
Definition
|
|
Term
| T/F THe prevalence of asthma has been increasing steadily in western countries. |
|
Definition
|
|
