Term
Nutrition in the Elderly
5-10-20 rule
plus 75 |
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Definition
weight loss triggers for evaluation:
>5% weight loss over 30 days
>10% weight loss over 180 days
BMI < 20
OR intake <75% of all meals for >7 days |
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Term
Nutrition in the Elderly
1-30-30 rule
nutritional maintenance requirements |
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Definition
protein: 1.0 g/kg/day
calories: 30 kcal/kg ideal body weight/day
water: 30 ml/kg/day |
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Term
Nutrition in the Elderly
what are some supplements you might recommend? |
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Definition
vitamin D with calcium (osteoporosis, fractures, m weakness, falls, fatigue)
B12 (atrophic gastritis, gastric/ileal surgery, pernicious anemia, H. pylori, gastric antacids, PPI, H2-receptors)
folate (alcohol abuse, methyltrexate, malnutrition) |
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Term
Nutrition in the Elderly
indications for tube feedings
in what condition is survival NOT enhanced by TF?
TF & mortality |
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Definition
neuromuscular disease, i/c BMR w/ inability to meet nutritional demands, post op patients who can't eat
Alzheimer's dementia
in all pts on TF, there is a high initial mortality of 28% at 30 days |
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Term
Nutrition in the Elderly - reasons for anorexia
changes in taste
GI factors
gut hormones |
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Definition
d/c taste receptor turnover; medications
delayed gastric emptying
higher levels of glucagon, CCK, leptin
d/c levels of ghrelin |
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Term
Diseases of childhood & infancy
malformations |
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Definition
errors of morphogenesis, in which there is an intrinsically abnormal developmental process; usually multifactorial
congenital heart defects, anencephaly |
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Term
Diseases of childhood & infancy
deformations
-due to?
most common underlying factor
maternal factors
fetal/placental factors |
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Definition
extrinsic disturbance of development
affect ~2% of newborns
compression of the growing fetus by abnormal mechanical forces
most common: uterine constraint
maternal factors: first pregnancy, small uterus, malformed uterus, leiomyomas
fetal/placental factors: oligohydramnios, multiple fetuses, abnormal fetal presentation
clubfeet (Potter sequence) |
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Term
Diseases of childhood & infancy
disruptions
examples? |
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Definition
secondary destruction of something that was normal in development; extrinsic disturbance in morphogenesis
amniotic bands
not heritable |
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Term
Diseases of childhood & infancy
what's a sequence?
example? |
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Definition
anomalies triggered by one initiating aberration
oligohydramnios (Potter) sequence
-d/c amniotic fluid |
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Term
causes of Oligohydramnios
resultant PT? |
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Definition
chronic leakage of amniotic fluid b/c of amniotic rupture
uteroplacental insufficiency d/t maternal HTN or severe toxemia
renal agenesis in the fetus
flattened facies
positional abnormalities of the hands and feet - clubfeet
dislocated hips
hypoplastic lungs
amnion nodosum (nodules in the amnion) |
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Term
Diseases of childhood & infancy
what's a syndrome? |
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Definition
can't be explained on the basis of a single initiating defect
most often caused by a single etiologic agent |
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Term
Diseases of childhood & infancy
most common *genetic* causes of congenital anomalies |
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Definition
chromosomal (karyotypic) abnormalities, aneuploidy
-trisomy 21
-most arise as defects in gametogenesis, so not familial
-vast majority die in utero
Mendelian inheritance (single-gene mutations)
-most are AD or AR
-holoprosencephaly, achondroplasia |
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Term
Diseases of childhood & infancy
most common *environmental* causes of congenital anomalies |
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Definition
Maternal disease states
-diabetes, phenylketonuria, endocrinopathies
-maternal hyperglycemia-induced fetal hyperinsulinemia --> fetal macrosomia, cardian anomalies, neural tube defects (diabetic embryopathy)
Maternal/placental infections
-rubella, toxoplasmosis, syphilis, CMV, HIV
-the at-risk period for rubella infxn: just before conception to gw16 - cataracts, heart defects, deafness, mental retardation
-highest at-risk period for CMV: second trimester - mental retardation, microcephaly, deafness, hepatosplenomegaly
Drugs & chemicals
-alcohol, folic acid antagonists, androgens, phenytoin, thalidomide, warfarin, 13-cis-retinoic acid
Irradiations
-microcephaly, blindness, skull defects, spina bifida |
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Term
Diseases of childhood & infancy
when is the embryo most susceptible to teratogenesis?
when is the peak sensitivity? |
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Definition
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Term
Diseases of childhood & infancy
causes of prematurity (< 37 wks) |
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Definition
preterm premature rupture of placental membranes (PPROM)
-inflammation of placental membranes
-enhanced collagen degradation by matrix metalloproteinases
Intrauterine infxn
-chorioamnionitis (inflammation of the placental membranes)
-funisitis (inflammation of the fetal umbilical cord
-microorganisms
-TLR-4
Uterine, cervical, & placental structural abnormalities
-uterine fibroids
-cervical incompetence
-placenta previa
-abruptio placentae
Multiple gestation |
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Term
Diseases of childhood & infancy
Causes of fetal growth restriction (FGR) |
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Definition
Fetal
-chromosomal disorders
-congenital anomalies
-congenital infxns (TORCH)
-proportionate (symmetric) FGR
Placental
-uteroplacental insuff
-asymmetric, w/ relative sparing of the brain
-down-reg of growth in the latter half of gestation b/c of limited O2 or nutrients
-confined placental mosaicism (viable genetic mutation that occurs after zygote formation)
Maternal - most common
-preeclampsia (toxemia of pregnancy)
-chronic HTN
-inherited thrombophilias
-drugs, alcohol, smoking
-maternal malnutrition |
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Term
Diseases of childhood & infancy
Hazards of prematurity |
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Definition
Hyaline membrane disease (neonatal resp distress syndrome, NRDS)
Necrotizing enterocolitis
Sepsis
Intraventricular hemorrhage
Long-term comp, inc developmental delay |
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Term
Diseases of childhood & infancy
Neonatal Respiratory Distress Syndrome
-etiology
-pathogenesis
-gross, microscopic findings
-clinical course, complications (risks of tx) |
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Definition
a.k.a. hyaline membrane disease
Deposition of a layer of hyaline proteinaceous material in the peripheral airspaces
*immaturity of the lungs*
-incidence is inversely prop to gestational age
*deficiency of pulmonary surfactant* - type II alveolar cells
-stiff atelectatic lungs, soft thoracic wall
-fibrin-hyaline membranes are barriers to gas exchnage --> CO2 retention and hypoxemia
-glucocorticoid release i/c surfactant
-insulin (from diabetic moms) d/c it
preterm, AGA
male, maternal diabetes, cesarean section
within 30 min, breathing difficult, cyanosis w/in hours
fine rales over both lung fields
x-ray: "ground glass"
alveoli are poorly developed, collapsed
necrotic cellular debris
alternating atelectasis & alveoli dilation
eosinophilic thick hyaline membranes line the dilated alveoli
Give surfactant tx, O2 to preterm babies (24-34 wks)
Risks of tx:
-O2 toxicity
-retrolental fibroplasia in the eyes (d/c VEGF, followed by i/c after tx)
-bronchopulmonary dysplasia (hyperoxemia, hyperventilation, prematurity, inflammatory cytokines, vascular maldevelopment)
Infants who recover from RDS are at i/c risk for:
-patent ductus arteriosus, intraventricular hemorrhage, necrotizing enterocolitis |
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Term
Diseases of childhood & infancy
-etiology
-when do symptoms appear? what do they suggest?
-"vicious cycle"
-clinical course
-gross & microscopic changes
-tx & outcome |
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Definition
prematurity, enteral feeding, infectious agents, inflammatory mediators (PAF)
bloody stools, ab distention, circulatory collapse
pneumatosis intestinalis
involved part of the SI or LI is distended, friable, congested, or gangrenous
mucosal or transmural coag necrosis, ulceration, bacterial colonization, submucosal gas bubbles
granulation tissue & fibrosis develop after an acute episode
20-60% of cases require resection of necrotic bowel segments
high perinatal mortality
those who survive often develop post-NEC strictures from fibrosis caused by the healing process |
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Term
Diseases of childhood & infancy
Perinatal infxns (3 kinds) |
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Definition
Transcervical (ascending) infxns
-most bacterial, a few viral (HSV II)
-fetus inhales infected amniotic fluid shortly before birth or by passing thru birth canal
-pneumonia, sepsis, meningitis
Transplacental (hematologic) infxns
-most parasitic and viral, few bacterial
-anytime during gestation or at time of delivery (HBV, HIV)
-parvovirus B19 (intranuclear inclusions)
-TORCH - fever, encephalitis, chorioretinitis, hepatosplenomegaly, pneumonitis, myocarditis, hemolytic anemia, vesicular or hemorrhagic skin lesions
Sepsis *know early, late, organisms*
-early onset (first 7 days of life) (group B strep)
-late onset (7 days-3 mo) (Listeria, Candida) |
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Term
Diseases of childhood & infancy
Fetal hydrops
-immune causes
-morphology
-clinical features |
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Definition
accumulation of edema fluid in fetus during intrauterine growth
hemolytic anemia caused by Rh incompatibility (D antigen)
can also be caused by ABO incompatibility (less often leads to hemolytic disease that requires tx)
anemia -> extramedullary hematopoiesis, cardiac decompensation -> hydrops fetalis
Hb degradation -> unconj bilirubin -> jaundice, kernicterus (CNS damage) |
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Term
Diseases of childhood & infancy
Fetal hydrops
-non-immune causes
-three major causes
-what is cystic hygroma?
-parvovirus B19
clinical presentation |
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Definition
Cardiovascular defects
-malformations, tachyarrhythmia, high-output failure
chromosomal anomalies
-Turner, Trisomy 21, Trisomy 18
Fetal anemia
-homozygous a-thal, parvovirus B19, immune hydrops (Rh and ABO)
CH: postnuchal fluid accumulation, resulting from abnormalities of lymph drainage from the neck (in Turner)
Transplacental infxn by parvovirus B19, causes erythema infectiosum in older kids
-tropism for erythroid cells, enters normoblasts, replicates, leads to apoptosis of red cell prog & red cell aplasia
-*diagnostic viral inclusions in early erythroid prog cells*
pallor, hepatosplenomegaly, jaundice, generalized edema, neurologic involvement |
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Term
Diseases of childhood & infancy
Inborn Errors of Metabolism
*Phenylketonuria*
-what is it?
-considerations with maternal PKU
-sweat |
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Definition
inability to convert phenylalanine to tyrosine
severe phenylalanine hydroxylase def -> i/c phenylalanine, severe mental retardation by 6 mo (in homozygous AR children)
with maternal PKU, maternal dietary restriction of phenylalanine is crucial before conception and throughout pregnancy
musty, mousy odor to sweat |
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Term
Diseases of childhood & infancy
Inborn errors of metabolism
Galactosemia
-know the enzyme
-clinical features |
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Definition
Galactose-1-phosphate uridyl transferase
-def leads to buildup of galactose-1-P, leading to producing of galactitol and galactonate
hepatomegaly
opacification of the lens (cataract)
nonspecific CNS alternations
failure to thrive
V&D
jaundice
mental retardation |
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Term
Diseases of childhood & infancy
Inborn errors of metabolism
*Cystic fibrosis (mucoviscidosis)*
-what is it?
-clinical features
-the gene?
-GT-PT correlation |
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Definition
the most important peds disorder, the most common lethal disease in whites
widespread disorder in epithelial transport affecting *fluid secretion in exocrine glands* and the *epithelial lining of the respiratory, GI, and reproductive tracts*
chronic lung disease, pancreatic insufficiency, steatorrhea, malnutrition, hepatic cirrhosis, intestinal obstruction, male infertility
the main defect in CF results from abnormal fn of an epithelial chloride channel protein encoded by the CFTR gene (7 q31-32)
more severe forms -> total lack of CFTR protein or function (class I-III)
milder PT (IV-VI)
-2 "severe mutations -> classic CF (pancreatic insuff, sinopulmonary infxn, GI symptoms)
-"mild" mutation on one or both alleles -> less severe PT
-hypertonic sweat
-lowered H2O content of the surface fluid layer coating mucosal cells
-abnormal bicarbonate transport |
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Term
Diseases of childhood & infancy
Clinical manifestations of CF |
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Definition
Chronic sinopulmonary disease
-persistent infxn: staph aureaus, H. influenzae, Pseudomonas aeruginosa
-chronic cough & sputum
-bronchiectasis, atelectasis, infiltrates, hyperinflation
-airway obstruction
-nasal polyps
-digital clubbing
GI & nutritional abnormalities
-meconium ileus, distal intestinal obstruction syndrome, rectal prolapse
-exocrine pancreatic insuff (most are "severe" CFTR mutations on both alleles), pancreatitis
--pancreas-suff PT: one or both CFTR mutations are mild
-liver disease, asymptomatic hepatomegaly, diffuse biliary cirrhosis
-malnutrition, hypoproteinemia, edema, fat-sol vit def
infertile males (azoospermia)
elevated sweat electrolyate conc |
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Term
Diseases of childhood & infancy
*SIDS*
-incidence
-some risk factors
-pathogenesis
-What is the enzyme involved in the inborn error of metabolism associated with fatty acid oxidation (used to be classified as SIDS) |
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Definition
57/100,000
young mom, smoker, drugs, low SES
infant brain stem abnormalities, SGA, male, multiple births
prone/side sleeper, hyperthermia
most occur w/in first 6 mo
heterogenous, multifactorial disorder
-vulnterable infant
-critical developmental period in homeostatic control
-exogenous stressor
delayed development of arousal & cardiorespiratory control
Acyl coenzyme a dehydrogenase (SCID) |
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Term
Diseases of childhood & infancy
hemangioma |
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Definition
the most common tumor of infancy
most are on the skin (esp face/scalp), "port-wine stain"
assoc neurocutaneous syndromes |
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Term
Diseases of childhood & infancy
lymphatic tumors |
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Definition
lymphangioma: hamartomatous or neoplastic
-cystic/cavernous spaces
-benign, but may encroach on vital structures
lymphangiectasis: abnormal dilations of preexisting lymph channels
-diffuse swelling of an extremity
-distortion, deformation may occur |
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Term
Diseases of childhood & infancy
fibrous tumors |
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Definition
fibromatosis: sparse, spindle-shaped cells
cellular fibromatoses/infantile fibrosarcomas: richly cellular, look like fibrosarcomas in adults |
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Term
Diseases of childhood & infancy
teratomas
-histologic maturity & biologic behavior
-two "peaks" of incidence
-most are __?
-sacrococcygeal teratomas |
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Definition
benign, well-diff cystic - mature
indeterminate potential - immature
admixed with another germ cell tumor component - malignant
2 yo
late adolescence/early adulthood
sacrococcygeal, gonadal, midline
more common in girls
dx: prenatal imaging, may require cesarean
10%-congenital abnormalities
75%-histologically mature/benign
12%-lethal/malignant
rest are immature |
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Term
Diseases of childhood & infancy
most common malignant tumors in children (0 to 4 yrs)? |
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Definition
leukemia
Rb
Nb
Wilms tumor
Hepatoblastoma
rhabdomyosarcoma
teratomas
CNS tumors |
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Term
Diseases of childhood & infancy
cancers: kids vs adults |
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Definition
adults - skin, lung, breast, prostate, coloan
kids - hematopoietic, CNS/neuroendocrine, soft tissue, bone, kidney |
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Term
Diseases of childhood & infancy
small round "blue cell" tumors |
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Definition
so called b/c of their primitive histologic appearance - childhood tumors
Nb
Ewing sarcoma/PNET
rhabdomyosarcoma
Burkitt lymph
lymphoblastic lymphoma/ALL
Wilms tumor
Rb
medulloblastoma |
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Term
Diseases of childhood & infancy
Neuroblastoma
-location?
-histologic features?
-classic presentation |
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Definition
one of the most common childhood tumors
most common tumor diagnosed in < 1 yo
adrenal medulla
anywhere along ht esympathetic chain (paravertebral ab, post mediastinum)
small, primitive-looking cells with dark nuclei, scant cytoplasm, poorly defined cell borders
increased BP, abdominal mass, fever |
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Term
Diseases of childhood & infancy
Neuroblastoma
-clinical course
-prognostic factors
-what do 90% of Nb make?
-low, int, high risk |
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Definition
kids < 18 mo have better prognosis
favorable:
stage-1, 2A, 2B, 4S
<18 mo
hyperdiploid or near-triploid
N-MYC not amplified
present TRKA
catecholamines (VMA, HVA)
low: stage 1 or 2, hyperploidy, < 18 mo, non-amp N-myc, TRKA present
int: >18 mo
*high: N-myc amplification* (even if everything else is ok) |
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Term
Diseases of childhood & infancy
Wilms tumor
-WAGR syndrome
-Denys-Drash
-Beckwith-Wiedemann syndrome
-precursor lesion?
-clinical features
-what does the presence of anaplastic histology correlate with?
-prognosis?
-WT-3 |
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Definition
the most common primary renal tumor of childhood, 4th most common peds malignancy
WAGR: aniridia, genital anomalies, mental retardation, 33% of getting Wilms
-deletions in WT1, PAX6
D-D: gonadal dysgenesis, early-onset nephropathy, diffuse mesangial sclerosis
-WT1 abnormalities (missense mutation, both alleles)
-i/c risk for developing gonadoblastomas
B-W: organomegaly, macroglossia, hemihypertophy, omphalocele, adrenal cytomegaly
-genomic imprinting
-more heterogenous, involves WT2
Nephrogenic rests (tend to develop Wilms tumors in contralateral kidney)
abdominal mass, hematuria, abdominal pain, HTN, pulmonary metastases
anaplasia = adverse prognosis; hyperchromatic, pleomorphic nuclei w/ abnormal mitoses, presence of p53 mutation, resistant to chemo
prognosis is good for most pts, but for those who survive there is i/c risk for getting second primary tumors (sometimes b/c of therapy) |
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Term
| What happens to baby if mother takes thalidomide? |
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Definition
used for maternal nausea and sedation
causes limb defects |
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Term
which organism group is most likely responsible for the development of acute chorioamnionitis in the fetus? (wk 27)
how does this infxn spread?
what other symptoms might a fetus with this infxn have? |
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Definition
Group B strep
ascending transcervical
sepsis, pneumonia, meningitis |
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