Term
| What percent of FTT is due to nonorganic causes? |
|
Definition
| nearly all cases in primary care are nonorganic and up to 1/3 to 1/2 of cases in tertiary care are nonorganic |
|
|
Term
| If you suspect failure to thrive due nonorgnaic causes, what can you tell a parent to do in order to keep track of the infants dietary habits? |
|
Definition
| keep a food diary where parent notes all foods offered and taken by the child and any associated symptoms with feeding |
|
|
Term
| In infants with FTT what should you ask about as far as GI ROS goes? |
|
Definition
| type of milk, freqeuncy and quality of feeding, voiding, vomiting, or stooling; milk used and mixing process should be reviewed |
|
|
Term
| Nonorganic FTT is commonly associated with... |
|
Definition
|
|
Term
| What is the significance of an occipital bald spot in infants? |
|
Definition
| can be secondary to neglect where the child is on their back and left alone in the crib all day long |
|
|
Term
| Can pts with FTT "catch-up"? |
|
Definition
| if they have nonorganic FTT they will catch up; some types of organic FTT will show catch up |
|
|
Term
| What is a good way to evaluate FTT on physical exam? |
|
Definition
| observe the mother feeding the infant |
|
|
Term
| How do you work up FTT if the history and physical exam do not suggest an organic cause for the FTT? |
|
Definition
| CBC, lead level, UA and urine culture, CMP; maybe HIV and TB skin testing |
|
|
Term
| How do you manage pts with nonorganic FTT? |
|
Definition
| improved dietary intake, close follow-up, and attention to psychosocial issues |
|
|
Term
| How many calories do infants need in the first year of life? after the first year of life? |
|
Definition
| they need about 120 kcal/kg/day in the first year; about 100 kcal/kg/day after the first year |
|
|
Term
| What mealtime routine is recommended for families? |
|
Definition
| eating together in a nondistracting environment with meals lasting between 20 and 30 minutes |
|
|
Term
| In pts with failure to thrive, how should they be fed in order to ensure maximum caloric intake? |
|
Definition
| solid foods offered before liquids; children not forcefed; low calorie drinks, juices, and water are limited; age appropriate high calorie foods are encouraged; can use high calorie formulas or PediaSure or Ensure for older children |
|
|
Term
| What is the standard calorie density of formula? |
|
Definition
|
|
Term
| What can cause lack of appetite in an infant? |
|
Definition
| depression and chronic disease |
|
|
Term
| What is a common renal cause of FTT? |
|
Definition
|
|
Term
| What causes of FTT are due to increased metablism and therefore increased caloric requirements? |
|
Definition
| congenital heart disease, chronic respiratory disease, neoplasms, chronic infection, hyperthyroidism |
|
|
Term
| What causes of inadequate weight gain in children can be due to malabsorption? |
|
Definition
| biliary atresia, celiacs, inflammatory bowel disease, infections, toxins |
|
|
Term
| How much more calories does an infant with FTT need in order to "catch up" compared to a normal requirement? |
|
Definition
| needs an additional 50-100% |
|
|
Term
| When do pts need a different growth chart from the standard one? |
|
Definition
| if they have syndrome like Down's or Turner's or if they are premmature |
|
|
Term
| What symptoms are common to both CMV and toxo congenital infections? |
|
Definition
| developmental delay, IUGR, cateracts, seizures, hepatosplenomegaly, prolonged neonatal jaundice, and purpura at birth |
|
|
Term
| When do children become picky eaters? |
|
Definition
| between 18 and 30 months of age |
|
|
Term
| What should you recommend for parents of children who are picky eaters and whose growth rate is slowing? |
|
Definition
| calm counseling of parents to provide nutrition, avoid "force-feeding", and avoid providing snacks; close followup required |
|
|
Term
|
Definition
| oral bicarb supplementation (to correct the elevated chloride level), and potentially potassium supplements |
|
|
Term
|
Definition
| proximal tubule defect caused by impaired tubular bicarb reabsorption |
|
|
Term
|
Definition
| distal tubule defect caused by impaired hydrogen ion secretion |
|
|
Term
|
Definition
| distal tubule problem associated with impaired ammoniagenesis |
|
|
Term
| If you suspect a adolescent pt is abusing drugs, what other health concerns should you address? |
|
Definition
|
|
Term
| What is the threefold approach for adolescent drug abuse? |
|
Definition
| 1) detoxification program 2) f/u with developmentally appropriate psychosocial support systems, 3) possible long-term assistance with a professional trained in substance abuse management |
|
|
Term
| What is the most common cause of new onset truant behavior, depression, or declining grades in an adolescent? |
|
Definition
|
|
Term
| What is the term for alcohol or other drug use leading to impairement or distress, causing fialure of school or work obligations, physical harm, substance related legal problems or continued use despite social or interpersonal consequences resulting from the drug's effect? |
|
Definition
|
|
Term
| What is the term for alcohol and other drug use, causing loss of control with continued use (tolerance requiring higher doses or withdrawal when terminated), compulsion to obatin and use the drug, and continued use despite persistent or recurernt negative consequences? |
|
Definition
|
|
Term
| Which children are at risk for drug abuse? |
|
Definition
| those with significant behavior problems, learning difficulties, and impaired family functioning |
|
|
Term
| What are the most commonly used drugs by adolescents? |
|
Definition
|
|
Term
| What is the most commonly used illicit drug by children? |
|
Definition
|
|
Term
| Methyphenidate is often abused by combining it with... |
|
Definition
|
|
Term
| T/F Needle marks and nasal mucosal injuries are rare in patients with drug abuse histories. |
|
Definition
|
|
Term
| When is it appropriate to use a urine drug screen to evaluate a child? |
|
Definition
| if they present with psychiatric symptoms, have signs and symptoms commonly attributed to drugs or alcohol, are in a a serious accident or are part of a recovery monitoring program |
|
|
Term
| Before getting a UDS on an adolescent it is very important to... |
|
Definition
| obtain the adolescent's permissiona nd maintain confidentiality |
|
|
Term
| What are the signs and symptoms of alcohol intox? |
|
Definition
| euphoria, grogginess, impaired short-term memory, talkativeness, vasodilation, and at high serum levels, respiratory depression |
|
|
Term
| What is the retention time of alcohol? |
|
Definition
| 7-10 hrs in blood; 10-13 hrs in urine |
|
|
Term
| What are the symptoms of marijuana intox? |
|
Definition
| elation and euphoria, impaired short term memory, distortion of time perception, poor performance of tasks requiring concentration (such as driving), and loss of judgment |
|
|
Term
| What is the retention time for marijuana use? |
|
Definition
| 3-10 days for occasional users or up to 2 months for chronic users |
|
|
Term
| What are the signs and symptoms of cocaine use? |
|
Definition
| euphria, increased motor activity, decreased fatigability, dilated pupils, tachycardia, hypertension, and hyperthermia; sometimes associated with paranoid ideation |
|
|
Term
| What is the retention time for cocaine? |
|
Definition
|
|
Term
| What are the signs and symptoms of methamphetamine tox? |
|
Definition
| euprhoria, increased sensual awareness, increased psychic and emotional energy, nausea, teeth grinding, blurred vision, jaw clenching, anxiety, panic attacks and psychosis |
|
|
Term
| What is the retention time for methamphetamines/ |
|
Definition
|
|
Term
| What is the scientific name for ecstasy? |
|
Definition
| methamphetamine or methylenedioxymethamphetamine |
|
|
Term
| What are the symptoms of opitate intox? |
|
Definition
| euphroia, decreased pain perception, pinpoint pupils, hypothermia, vasodilation and possible respiratory depression |
|
|
Term
| What is the retention time for opiates? |
|
Definition
|
|
Term
| What are the symptoms of PCP intox? |
|
Definition
| euphoria, nystagmus, ataxia, and emotional lability; hallucinations affecting body image that can result in panic reactions, disorientation, hypersalivation, and abusive language |
|
|
Term
| What is the retention time of PCP? |
|
Definition
|
|
Term
| What are the signs of barbiturate intox? |
|
Definition
| sedation, pinpoint pupils, hypotension, bradycardia, hypothermia, hyporeflexia, as well as central nervous system and respiratory depression |
|
|
Term
| What is the retention time of barbiturates? |
|
Definition
| 1 day for short acting; 2-3 weeks for long acting |
|
|
Term
| What is the symptoms timeline of LSD intox? |
|
Definition
| symptoms start 30-60 min after ingestion; peak 2-4 hrs later; resolve by 10-12 hours |
|
|
Term
| What are thesymtposm of LSD intox? |
|
Definition
| delusional ideation, body distortion, and paranoia; also can "see" sound and "hear" color, dizziness, pupillary dilatation, nausea, fever, tachycardia, and facial flushing |
|
|
Term
| What life threatening complications should you evaluate for if you suspect a newborn has Down's? |
|
Definition
| cardiac and GI abnormalities (duodenal atresia) |
|
|
Term
| When is the maternal serum triple screen performed? |
|
Definition
| between 15th and 20th weeks of pregnancy |
|
|
Term
| What is advanced maternal age? |
|
Definition
|
|
Term
| What do you call excessive shortening of hand and foot tubular bones resulting in a box like appearance? |
|
Definition
|
|
Term
| What is incurving of on of the digits called? |
|
Definition
|
|
Term
| What is a dysmorphic child? |
|
Definition
| a child with problems of generalized growth or body structure formation that can be due to either a syndrome, an association, or a sequence |
|
|
Term
|
Definition
| constellation of features from a common cause |
|
|
Term
|
Definition
| two or more features of unkown cuase occuring together more commonly than expected |
|
|
Term
|
Definition
| vertebral problems, anal abnormalities, trachea/esophageal abnormalities, and radius or renal anomalies are associated more commonly than would be expected |
|
|
Term
|
Definition
| a single defect that leads to subsequent abnomralities |
|
|
Term
| Describe the characteristic facies of potter's syndrome? |
|
Definition
| wide-set eyes, flattened palpaebral fissures, prominent epicanthus, flattened nasal bridge, mandibular micrognathia, and large, low set cartilage deficient ears |
|
|
Term
| What is tested for in the serum trisomy screening? |
|
Definition
| estriol, AFP, and betahCG |
|
|
Term
| What percent of down syndrome babies are pickedup by serum trisomy screening? |
|
Definition
|
|
Term
| What percent of babies with NTDs are picked up by serum trisomy screening? |
|
Definition
|
|
Term
| What are the characteristic features of down syndrome patients? |
|
Definition
| upslanting palpebral fissures, epicanthal folds, Brushfield spots, flat facial profile, brachycephaly, small and rounded ears, excess nuchal skin, widespread nipples, pelvic dysplasia, joint hyperflexibility, hypotonia, short stature, poor moro reflex, fifth finger clinodactyly, single transverse palmar crease, wide spacing between first and second toes |
|
|
Term
| What percent of down syndrome infants have cardiac defects? |
|
Definition
|
|
Term
| What are the most common cardiac defects in down syndrome patients? |
|
Definition
| 60% endocardial cushion defect; 32% have VSD; 6% have tetralogy of fallot |
|
|
Term
| What should you do for a Down syndrome newborn to evaluate for cardiac abnormalities? |
|
Definition
| get a card consult and an echo |
|
|
Term
| What percent of Down syndrome pts have intestinal atresia? |
|
Definition
|
|
Term
| When should you start to worry about intestinal/duodenal atresia in Down syndrome patients? |
|
Definition
| although all DS infants have hypotonia and sometimes slower feedings; you should worry if the infant develops persistant vomiting after feeds, esp if bilious or with history of polyhydramnios; upper GI study likely will reveal characteristic "double bubble" pattern of duodenal atresia |
|
|
Term
| What causes Downs syndrome? |
|
Definition
| 95% nondisjunction; 2% translocations; 3% mosaics |
|
|
Term
| Why is it important to educate families of patients who have Down syndrome due to translocation? |
|
Definition
| because recurrence risk of downs can approach 100% in some cases |
|
|
Term
| How is caring for a Down's syndrome patient different than a normal well baby? |
|
Definition
| periodic thyroid, hearing and vision screenings are important; also psychosocial intervention; thyroid is part of newborn screen; hearing is evaluated by 3 months; opthalmo eval by age 6 months |
|
|
Term
| DS patient shave higher risk of... |
|
Definition
| hearing loss, strabismus, cateracts, nystagmus, congenital hypothyroidism, leukemia, acquired hypothyroidism, atlandoaxial instability, and premature aging with increased risk of alzhiemers |
|
|
Term
| T/F All downs sydnrome children are mentally retarded. |
|
Definition
| true; but severity varies and can approach normal almost in mosaic |
|
|
Term
| What are thesymptoms of trisomy 18? |
|
Definition
| clenched hands with overlapping digits, small palpebral fissures, prominent occiput, short sternum, and cardiac defects (VSD, ASD, PDA or coarc), low set and malformed ears, microcephaly, rocker-bottom feet, inguinal hernia, cleft lip or palate, an dmicrognathia, weak cry, single umbilicalartery, micrognathia with small mouth and higharched palate |
|
|
Term
| What are white or grey spots in the periphery of the iris called? |
|
Definition
|
|
Term
| What are the features oftrisomy 13? |
|
Definition
| microcephaly and sloping forehead, deafness, scalp cutis aplasia microphthalmia, coloboma cardiac defect (esp VSD), omphalocele, singleumbilical artery, and hypersensitivity to agents containing atropine and pilocarpine |
|
|
Term
| What does the intial HIV test actually tsting for? How soon after infection does it tern positive? |
|
Definition
| ELISA screening for HIV-1 IgG; 2 weeks to 6 months after initial exposure |
|
|
Term
| What might cause false negative ELISA HIV screenings? |
|
Definition
| immunization or hepaticdisease, autoimmune disease, or advanced AIDS |
|
|
Term
| What is the confirmatory test after a positive ELISA? |
|
Definition
| western blot: directvisulaization of antibodies to virion proteins |
|
|
Term
| What is the function of CD4 cells? |
|
Definition
| essential for humoral and cellular immunity; binds to antigens presented by B cells, prompting antibody productionand to antigens presented by phagocytes, prompting lymhpokine release; rendereddysfunctional in HIV infection |
|
|
Term
| What is primary or syndromic immunodeficiency? |
|
Definition
| immunodeficiency due to a genetic defect either inherited or related to gene mutation |
|
|
Term
| Most primary immunodeficienciesare... |
|
Definition
| humoral or characterized by humoral and cellular dysfunction |
|
|
Term
| Name an immunodeficiency characterized by defective macrophages. |
|
Definition
|
|
Term
| What is a secondary immunodeficiency? |
|
Definition
| pts with normal immune function at birth but subsequently developan illness or metabolic abnormality that disrupts immune cell production or function |
|
|
Term
| What conditions adversely affect a pts immune status? |
|
Definition
| HIV infection, diabetes, malnutrition, hepatic disease,autoimmune disease (scleroderma), aging and stress |
|
|
Term
| How many people in the world have HIV? |
|
Definition
|
|
Term
| When did they start screening the blood supply for HIV? |
|
Definition
|
|
Term
| How is HIV acquired in the pediatric population? |
|
Definition
| approx 80% via intrapartum transfer, but HIV can also be acquired from infected secretions at delivery and from breast milk |
|
|
Term
| What is the risk of transfering HIV to a fetus vertically with and without antiretrovirals? |
|
Definition
|
|
Term
| How long do you use antiretrovirals to prevent vertical transmission of HIV? |
|
Definition
| started by the mother during the second trimester and given to the baby through age 6 weeks, reduces the risk of HIV transmission to less than 10% |
|
|
Term
| What isthe prognosis of kids with congenital HIV infection? |
|
Definition
| 75% follow a course similar to adults with an extended period of disease inactivity; the remaining 25% progress rapidly during the first several months of life |
|
|
Term
| How do you diagnose young infants with HIV? |
|
Definition
| if they are less than 18 months you use HIV DNA PCR, if over18 months you perform an HIV antibody ELISA and subsequent western blot forconfirmation |
|
|
Term
| How do you determine the degree of immunosuppressionin HIV pos pts? |
|
Definition
|
|
Term
| How do you manage pts with congenital HIV infection? |
|
Definition
| neonates should recieve 6 weeks AZT, PCP prophylaxis starts at 6 weeks for HIV+, CD4 levels are followed in quarterly intervals if HIV+, RNA activity is measured because it correlates with disease progression= more than 100,000 copies/mL have been associated with advanced progression and early death |
|
|
Term
| What are the three major classes of antiretrovirals? |
|
Definition
| nucleoside reverse transcriptase inhibitors (didanosine, stavudine, zidovudine), nonnucleoside reverse transcriptase inhibitors (efavirenza, nevirapine), and protease inhibitors (indinavir, nelfinavir) |
|
|
Term
| What are some common adverse effects of antiretrovirals seen in children? |
|
Definition
| headahce, emesis, abdominal pain, and diarrhea; osteopenia and drug rash; anemia, entruopenia, elevated transaminases, hyperglycemia and hyperlipidemia |
|
|
Term
| What is the current pediatric antiretroviral therapy recommendation? |
|
Definition
| three drugs= two nucleoside reverse transcriptase inhibitors and one protease inhibitor |
|
|
Term
| How do you change the vaccine schedule for pts with congenital HIV infection? |
|
Definition
| immunizations should be kept current, except for live vaccines such as MMR and varicella |
|
|
Term
| How does diabetes cause immune deficiency? |
|
Definition
| hyperglycemia promotes neutrophil dysfunction and circulatory insufficiency contributes to ineffective neutrophil chemotaxis during infection |
|
|
Term
| What is Leukocyte adhesion deficiency? |
|
Definition
| LAD is an inheritable disorder of leukocyte chemotaxisand adherence characterized by recurring sinopulmonary, oropharyngeal and cutaneous infections with delayed wound healing. Neutrophilia is common with WBC counts of typically more than 50,000 cells/mm3 |
|
|
Term
| What severe life threatening infections can pts with LAD get? |
|
Definition
| staph, enterobacter, and candida |
|
|
Term
| What is the treatment for LAD? |
|
Definition
| good skin and oral hygiene are important, broad-spectrum antimicrobials and surgical debridement are early considerations with infection |
|
|
Term
|
Definition
| autosomal recessive or x-linked |
|
|
Term
| What lab abnormalities are seen in SCID? |
|
Definition
| serum Ig and T cells are often markedly diminihsed or absent |
|
|
Term
| What kind of infections are seen with SCID? |
|
Definition
| recurrig cutaneous gastrointestinal or pulmonary infections occur with opportunistic organisms such as CMV and PCP |
|
|
Term
| What is the prognosis of SCID? |
|
Definition
| death usually occurs in the first 12 to 24 months of life unless bone marrow transplantation is performed |
|
|
Term
| What mutation causes diGeorge? |
|
Definition
|
|
Term
| What characterizes the immunodeficiency of DiGeorge? |
|
Definition
| decreased T cell production and recurring infection |
|
|
Term
| What heart defects are seen in DiGeorge? |
|
Definition
| VSD adn tetralogy of Fallot |
|
|
Term
| DiGeorge can be accompanied by dysgenesis of... |
|
Definition
| thymus and parathyroid; can cause hypocalcemia and seizures |
|
|
Term
| DiGeorge can be characterized by developmental delay. |
|
Definition
|
|
Term
| What is the incidence of Klinefleter syndrome? |
|
Definition
|
|
Term
| What is mental retardation? |
|
Definition
| a clinically and socially important impairment of measured intelligence and adaptive behavior that is diagnosed before 18 yoa |
|
|
Term
|
Definition
| preconceptual and early embryonic disruptions (teratogens, chromosomal abnormalities, placental dysfunction, congenital CNS malformations); fetal brain insults ( prematurity, metabolic disorders, infections); postnatal brain injuries (infections, trauma, metabolic disorders, toxins, poor nutrition), and miscellaneous postnatal family difficulties (poverty, poor caregiver-child interaction, parental mental illness) |
|
|
Term
| Pts with klinefelter syndrome have delay in what areas of learning? |
|
Definition
| verbal cognitive areas (underachieve in reading, speling and mathematics) |
|
|
Term
| What variations of klinefelters have increased MR? |
|
Definition
|
|
Term
| What diagnosis should be considrered for all boys who have been identified as having mental retardation or psychosocial, school or adjustment problems? |
|
Definition
|
|
Term
| What physical characteristics should be noted in pts with MR? |
|
Definition
| size of the occiput, unusual hair color or distriution, distinctive eyes, malformed ears or nose, and abnormalities in jaw size, mouth shape, or palate height; hands= short metacarpals or metatarsals, overlapping or supernumerary digits, abnormal creases or nails, skin= cafe au lait, depigmented nevi, abnormal genitalia |
|
|
Term
| Pts with klinefelters have an increased risk of what cancers? |
|
Definition
|
|
Term
| What laboratory/radiologic testing is reasonable in a MR workup? |
|
Definition
| karyotype, urine and serum amino and organic acids, serum levels of ammonia, lead, zinc, copper and serum for congenital infections; radiologic= CT, MRI and EEG |
|
|
Term
| What facial features are characteristic of XYY? |
|
Definition
| long and asymmetrical ears, increased length versus breadth for the hands, feet and cranium and mild pectus excavatum; by age 5 to 6 they start to be taller than their peers, nodulocystic acne, mild pectus excavatum, large teeth, prominent glabella, relatively long face andfingers, poor fine motor skills, low-normal IQ |
|
|
Term
| What can you do for an adolescent boy with klinefelters? |
|
Definition
| perscribe him testosterone supplements |
|
|
Term
| What are characteristics associated with Turner's syndrome? |
|
Definition
| widely spaced nipples, broad chest, cubitus valgus, edema of the hands andfeet in the newborn period, congenital heart disease (coarctation of the aorta or bicuspid aortic valve), horseshoe kidney, short fourht metacarpal and metatarsal; hypothyroidism and decreased hearing. hypertension, lowposterior hairline, prominent low set ears, excess nuchal skin |
|
|
Term
| How does prematurity lead to MR? |
|
Definition
|
|
Term
| What causes the hypertension sometimes seen with turner syndrome? |
|
Definition
| possibly due to renal abnormalities like horseshoe kidney |
|
|
Term
| What is the most common form of inherited mental retardation? |
|
Definition
|
|
Term
| What characteristics are seen in fragile x? |
|
Definition
| macrocephaly, long face, high archedpalate, largeears and macroorchidism |
|
|
