Term
| Regurgitation of stomach contents, no harm/discomfort to infant, "spitting up", occurs in a large amount of infants, peaks at 1-4 months; resolves by 18 m.o. |
|
Definition
Gastroesophageal Reflux
Lifestyle modifications in Infants: Thick formula w/ rice cereal,
Positioning = sleep prone if severe, avoid sitting positing post prandial
Try Casein hydrolysate formula--> no soy formula-->cross reactivity with cow's milk
Lifesyle modification- Older children
Same guidline as adults- late eating then sleeping/ avoid fat food/chocolate/acidic liquid/spicy food/pepermint
Elevate HOB |
|
|
Term
Action of peppermint
prone position action for infants
Sitting action in infants |
|
Definition
relaxes LES
increases pressure on LES making it tighter
intra abdominal pressure fights against LES |
|
|
Term
| pathologic, wt loss (FTT I), pain while eating, esophagitis (pain/irritability/Fe deficiency anemia, aspiration respiratory Sx( apnea/wheezing/cough, Sandifer's syndrome, persist PAST/begin after 1 y/o |
|
Definition
GERD; Lifestyle modifications in Infants: Thick formula w/ rice cereal,
Positioning = sleep prone if severe, avoid sitting positing post prandial
Try Casein hydrolysate formula--> no soy formula-->cross reactivity with cow's milk
Lifesyle modification- Older children
Same guidline as adults- late eating then sleeping/ avoid fat food/chocolate/acidic liquid/spicy food/pepermint
Elevate HOB, Medications: H2 blockers-Cimetidine= less popular, gynecomastia, HA/ Rantidine= first line choice; Prokinetic agents- Metoclopromide=EPS/dystonia / Cisapride=agent of choice, QTc; PPI=H2 blockers |
|
|
Term
| Spasmodic, torsional dystonia w/ arching of back. Common in GERD, esophagitis, hiatal hernia. Mistaken for siezures-->neurology consult, chronic in children w/ neurtologic d/o, resolves with GERD tx |
|
Definition
|
|
Term
|
Definition
|
|
Term
| classic GERD Sx predominate- dyspepsia, esophageal stricture, asthma, Barret's esophagus |
|
Definition
>=child age GERD;Thick formula w/ rice cereal,
Positioning = sleep prone if severe, avoid sitting positing post prandial
Try Casein hydrolysate formula--> no soy formula-->cross reactivity with cow's milk
Lifesyle modification- Older children
Same guidline as adults- late eating then sleeping/ avoid fat food/chocolate/acidic liquid/spicy food/pepermint
Elevate HOB, Medications: H2 blockers-Cimetidine= less popular, gynecomastia, HA/ Rantidine= first line choice; Prokinetic agents- Metoclopromide=EPS/dystonia / Cisapride=agent of choice, QTc; PPI (Nexium) =H2 blockers |
|
|
Term
| Delineates anatomy well- Dx strictures/webs/rings/pyloric stenosis/hiatal hernia, NOT diagnostic for GERD- Reflux is often seen |
|
Definition
| Upper GI barium fluoroscopy |
|
|
Term
|
Definition
|
|
Term
| # episodes (>5min), total duration of espisode(reflux index with pH <4, overnight hospital stay, GOLD STANDARD for GERD |
|
Definition
|
|
Term
| proportionate and health child require no testing, FTT/>18 m.o. no longer simple condition |
|
Definition
|
|
Term
| Definitive difficult, made with GI consultation, Choice of testing based on Hx and PE |
|
Definition
|
|
Term
| Taking pills w/o water, drinking caustic chemicals (suicidal/accidental), Candida cause associated w/ immunocompromised |
|
Definition
| Esophagitis; NPO/ IV fluids, viscous lidocaine, PPI (Used MC), sucralfate (barrier fluid), Metoclopramide (antiemetic)--no Tx has been proven |
|
|
Term
| 3+ episodes abd pain causing functional limitations >3 mo, peaks at 5-7 y/o Male=female & 13-18y/o MC in female, have functional cause of pain, ENS "gut-brain" dysfunction |
|
Definition
| Functional Abdominal Pain; Primary= return child to functional life, secondary= eliminate Sx, r/o organic causes: order specific tests if suspected, ordering tests may worsen anxiety, encompass GU (STD/Homecoming Queen Syndrome) if teens, education essential, Sx diary, return to school, reassurance, relaxation Tx, Diet- water, fiber, exercise, decrease milk, Rx only for specific Dx, Listen to Hx, point out lack of impact on child (no: fevers/FTT/wt loss) |
|
|
Term
| Functional GI etiologies of childhood |
|
Definition
| Functional dyspepsia, IBS, functional abdominal pain, abdominal migraine, aerophagia |
|
|
Term
| if present, must look for another explanation than Functional GI d/o |
|
Definition
|
|
Term
| Upper abdominal pain >3mo, no relation/change in bowel movements |
|
Definition
|
|
Term
| Recurrent abdominal pain >3 days/mo for 3 mo with 2-3 Rome Criteria |
|
Definition
|
|
Term
| Pain: relieved with BM, change in BM fz, BM consistency |
|
Definition
|
|
Term
| near daily abd pain not c/w other functional Dx for >3 mo |
|
Definition
| Functional abdominal pain |
|
|
Term
| Abdominal pain, teenager, girl |
|
Definition
|
|
Term
| 3 episodes acute mid line pain in <12wks, (2/5)= HA during, photophobia during, aura prior to, Migraine Hx, Migrain FHx |
|
Definition
|
|
Term
| Air swallowing >12 wks, abd distention, repetative belching |
|
Definition
|
|
Term
| Recurrent vomiting (bilious), fever, awakening night pain, far from umbilicus, wt loss, hemechezia/melena/hematoemesis, FTT, delayed puberty |
|
Definition
| Red Flags c/w organic pain |
|
|
Term
| In children the farther from the umbilicus the pain is located |
|
Definition
| more likely it is to be pathologic |
|
|
Term
|
Definition
| Sprue, non-tropical spru, gluten sensitive enteropathy |
|
|
Term
| What's all the crave about a gluten free diet... |
|
Definition
| Nothing, there is no additional benefit from not eating gluten if you are not allergic |
|
|
Term
Pt with: Injury of the intestines due to allergic response to gluten 2. caused by Wheat/Barley possibly oats but unlikely, consider in ANY child with chronic abd complaints
3. Malabsorption - FTT class
Abdominal bloating, pain and diarrhea, Ascites due to low protien
What is it? Cause by?
Presentation?
Labs to order- background info on these tests?
Tx of Celiac Dz
Safe Foods |
|
Definition
Celiac Dz
4. MUST NOT ALREADY BE ON A GLUTEN FREE DIET- ALTER TEST RESULTS AND DX
Serum IgA antigliadin AB- older/wrong
Tissue Transgluatminase and Endomysial AB- newer/ more accurate
Duodenal biopsy - Dx and r/o IBS
5. No gluten
6. Rice, soy, tapioca, buckwheat, potatoes, oats |
|
|
Term
allergy to protien in formula/milk-breast milk is least common
Pt with: Abd distention, gas/fussiness after feeding, blood streaked stool, (-)N/V/abd pain
what is the allergy to?
Clinical presentation
How to r/o fissure/intussusception
What age group?
LABS to order
How to Dx
Tx |
|
Definition
Allergic Colitis
3. PE- abd pain v. none; red currant jelly stools
4. 1-2w.o. up to 1y/o
5. CBC/Fe panel to r/o anemia
6. Clinical--> can do colon bx (eosinophilia) or Anoscope (mucosal friability) both w/ lympoid hyperplasia (mosquito bite appearance)
7. Maternal diet change- no cows/soy milk
Casein hydrolysate |
|
|
Term
1.rapid onset diarrheal illness, 3+ loose, watery stools/day; leading cause of morbidity, second most common dz in children
2.What is key with this d/o
3. Hx/PE
4. Assess Severity
daycare think...
Bloody think...
Hx of ABX use think...
Labs Needed |
|
Definition
1.AGE
2.Further investigation/Tx is needed
3. r/o other underlying dz
4. PO intake, number/duration of episodes, activity level, vital signs (only use pulse- BP does NOT help), Skin: turgor/mucus membranes
Shigella/cryptosporidium/rotavirus
Bacterial/intussusception
C. diff
5. None needed unless special circumstances which is on another card |
|
|
Term
| Pt with: Decreased absorpion, increased secretion, watery stools, persists (+)cholera/HUS/C. diff |
|
Definition
|
|
Term
| Pt with: Maldigestion, transportation defect, unabsorbed solute; watery stool, low pH, (+) reducing substances in stools, stops with fasting, no WBC in stool, Lactase deficiency/ laxitive causing |
|
Definition
|
|
Term
| Inflammation, decrease GI surface area; (+) blood, WBCs in stool, Dysentery-blood, mucus, WBC in stools; Celiac, Shigella, Salmonella, Campylobacter, Rotavirus, Amebiasis |
|
Definition
| Mucosal Inflammation (absorption) |
|
|
Term
Etiology of AGE:
Causes
MCC |
|
Definition
Viruses-MCC
Bacterial-2nd MCC
Parasites
Other |
|
|
Term
Etiology of AGE
Viral-MC
Bacterial-MC
Parasites-MC
Other |
|
Definition
1. Rotavirs MCC (norovirus/adenovirus)
2. Campylobacter (MC bacterial cause)
Shigella (2nd)
3. Parasite- Giaria lamblia (MC)-fecal contaminated water source- 3rd world
4. Meds:
other: Metformin/ colchicine/ steroids
ABX- Clindamycin=C diff/Augmentin, excess carb intake |
|
|
Term
Kid with AGE and:
Severe dehydration or prolonged diarrhea(10d)--action
Blood, mucus in stool or high fevers--action
Sx>10d--action
Sx are recurrent -- action
Hx of ABX use(esp. clindamycin/cephalosporins)--action |
|
Definition
1. Specific gravity-->elevated (hydration status)
EKG-->dysrhythmias, weakness(Hypokalemia)
Hypertonia/ hyper-reflexia, lethargy--> Hyponatremia (hypochloremic metabolic alkalosis with vomiting, nongap metabolic acidosis common with diarrhea
2. obtain stool leukocytes (shigella), stool culture
3. Stool O&P to r/o Giargia
4.Stool pH(<6.0 c/w carbohydrate malabsorption)
5.C. diff toxin screen |
|
|
Term
DM
D/O of puberty
Thyroid d/o
d/o of sexual differentiation
adrenal d/o |
|
Definition
I and II
Pecocious puberty, delayed puberty
hypothyroidism/ hyperthyroidism
female virilization / male feminization
adrenal insufficiency/ 21-hydroxylase deficiency (CAH) / steroid excess |
|
|
Term
MC pediatric endocrine dz
2nd MC chronic dz to....
There has been a shift in this dz to its counterpart due to...
(+) HLA DR3 / DR4 alleles on what chromosome? |
|
Definition
DM
Asthma
Obesity
chromosome 6 |
|
|
Term
Definition of:
DM
Prediabetes
(substance typically used for a this lab measurment) |
|
Definition
>= 2/4: fasting glucose >=126, 2 h post-prandial >=200mg/dL, Ha1C >=6.5
FBG >=101, 2h PPGL >= 141, Ha1C >=5.7
Glucola- 2h PPBG |
|
|
Term
| Pt with: Polyuria, polydipsia, polyuria; wt loss; enuresis, fatigue, weakness, blurred vision, yeast vaginitis |
|
Definition
|
|
Term
HUGE RED FLAG of DM
Lab values for DM
How does polydipsia develop?
How does polyphagia develop? |
|
Definition
Wt loss
Blood sugar - fasting/random/post glucola
UA (indicates blood glucose >180)
Ha1C(nml <5.7; >=6.5)
AB Screens (Insulin AB/ Islet cell AB/ Glutamic acid decarboxylase AB)
LOts of glucose getting filtered, with it goes water (osmotic difference) taking Na/K--> dehydration-->thirsty
No insulin-->no glucose entrance-->fat/protien broken down-->insufficient sustainable leading to a constant hunger and wt loss |
|
|
Term
|
Definition
SQ insulin- (NPH + regular BID insulin) / Lantus (am) + regular (prn)
Diet- balanced w/ snacks; avoid carb heavy meals
Exercise- increases insulin sensitivity and self esteem; decreases obesity
Education -improves compliance and monitoring |
|
|
Term
Insulin activity: onset/peak/duration
1. Short Acting
2.Regular Acting
3.NPH
4.Long acting |
|
Definition
1.Humalog/Lispro(15min/30min-1h/3-4h)
2.Humalin R/Novalog R (30min/2-4h/4-8h)
3.Humalin NPH/Novalog NPH (2-4h/6-8h/12-15h)
4a. Ultralente(4-6h/8-15h/15-18h)
4b. Glargine/Lantus(2-4h/8-10h/ >24h) |
|
|
Term
Monitoring of DM
1. How often?
2. Nml Glucometer readings:
3. Transient improvement in function of endogenous insulin with onset of Tx. Unfortunately, this decreases over the course of wks-mo |
|
Definition
3 x's/ day
2. <6 = 100-200
6-12 = 80-180
>12 = 70-150
3. Honeymoon phase |
|
|
Term
| Typical Insulin regimen and advantages |
|
Definition
1. BID NPH + reg: eliminates need for dosing at school
2. Lantus + reg: flexibility of regimin |
|
|
Term
Name the regimen associated with the drawback:
strictly control meals/snacks times. Difficult to control with a changing schedule
requires 4-5 (many) daily injections, unsuitable for pre-adolescents |
|
Definition
BID NPH + reg
Lantus + reg |
|
|
Term
Acute Complications of DM
MC
3 types and Tx |
|
Definition
Hypoglycemia(MC)-: glucose (p.o. gel, tabs, are faster than most food or sucrose), glucagon; adjust medication regimen.
Hyperglycemia
(MC is dawn phenomenon)-insulin increase
Somogyi phenomenon-decrease evening insulin
DKA- discussed later |
|
|
Term
1. <60-70mg/dL reading, Tx?
2. Increased reading at 8am, early 2am increase and persists throughout the night- due to physiologic GH release
Tx? |
|
Definition
<60-70mg/dL
2. Usually around 8am
Dawn Phenomenon- Increase pm insulin dose- "sun rises you must increase insulin" |
|
|
Term
rise in level around 8 am, early 2am hypoglycemia from too large nighttime insulininsulin dose-->rebound hyperglycemia on waking
Tx? |
|
Definition
| Somogyi phenomenon- decrease bed time insulin |
|
|
Term
Pt with:Kussmaul respirations, acetone oder breath(sweet breath), N/V, AMS
Tx? |
|
Definition
DKA
Fluids- 20cc/kg NS,repeat bolus,1.5 X Maintainence(100cc/kg for first 10kg, 50cc/kg for next 10kg (up to 20kg), 20cc/kg >20kg)
Insulin- 0.1U/kg/h- can lead to cerebral edema if Tx too fast
Electrolyte replacement- Ensure they are not anuric before starting K replacement; Anticipate hypokalemia (1st is not correct), replace with 20-40 mg/L(NS given) of KCl+KPO4 racemic (give phosphate w/o causing hypocalcemia) |
|
|
Term
Chronic Complications of DM
Name-how to avoid |
|
Definition
Liposystrophies at injection site-rotate injection site
Retinopathy- Annual exam >3yr of DM
Nephropathy-No dip stick->special needed->microalbuminuria(>20microg/dL) check annually >3yr of DM
Neuropathy- annual foot checks(10g/filament)
CAD Risk-Annual lipid profile
Thyroid dz-annual TSH |
|
|
Term
Pt with: skin d/o which can affect the lower extremities of insulin dependent diabetics, although it may occur in non-diabetic pt. Lesion is yellowish brown, shiny, pale, thin, telangiectasia, ulcerate if perforated, granulomatous inflammatory reaction-->collagen destroyed?
Tx? |
|
Definition
Necrobiosis Lipoidica (NLD)
2. topical/intralesional steroids |
|
|
Term
Pt with: Insulin resistance
Risk factors of this d/o
Tx?- S/E |
|
Definition
DMT2
Obesity, sedentary lifestyle, African American, Hispanic, Pima Indian
Same as DMT1, Metformin or TZD:
(Actos-Bladder cancer
Avandia - CVD) |
|
|
Term
| Short Stature: definition, Causes |
|
Definition
1. >= 3.5 SD below age normal
2. Idiopathic-Hereditary, growth delay
Time - Constitutional delay
Endocrine- GH deficency, decreased insulin, Hypothyroidism, Adrenal d/o- adrenal insufficiency (addison's dz), precocious puberty
Neonate Development- IUGR, achondroplasia, inborn errors of metabolism (Turner's), fibroblast growth factor receptor 3 |
|
|
Term
MC Cause of Short stature
Constitutional growth delay v. hereditary growth delay and what does that mean |
|
Definition
1. Idiopathic
2. constitutional - delayed bone age (bone age < chronologic age)
Hereditary - normal bone age (bone = chronologic age) |
|
|
Term
| evident at birth w/ a gap b/t index and long fingers, head autosomal dominant. disporportionatly large for the body, short limbs, bowed legs, frontal bossing, decreased muscle tone. |
|
Definition
|
|
Term
| PE findings associated with Short stature |
|
Definition
V/S: elevated BP may point to metabolic dz
Ht/ Wt pattern: acute= organic/ chronic= non-organic |
|
|
Term
| If bone age is lagging behind chronological age, it’s... |
|
Definition
| a "constitutional growth delay" with good prognosis |
|
|
Term
| Labs for Short Stature and hoping to find with each test |
|
Definition
CBC - infection, cancer
ESR - infection, RA
Chem 7 - renal, metabolic dz
CMP - metabolic dz
UA - Renal dz
TSH, LFT
Chromosomal analysis - Turner's, Down's
GH- GH stimulation test (Arginine)
X-ray - Bone age |
|
|
Term
1.Tx of Short Stature
2.Hormone replacement considered when, and S/E? |
|
Definition
Correct underlying cause
Low hormone/idiopathic; Benign Intracranial HTN (Pseudoturmor cerebri)
SCFE |
|
|
Term
When does hypothalamus begin production of GnRH?
At what point are sex steroids produces |
|
Definition
8 y/o
GnRH reaches a threshold-->release of FSH and LH-->release of sex steroids-->breasts/uterus/testicle/penile development |
|
|
Term
What is the definition of Precosious puberty? MC found in....
Central Causes: MC?
Peripheral Causes: |
|
Definition
>2.5 SD before the mean age of development; (<8 y/o-female(MC); <9 y/o-male)
Central:
Idiopathic GnRH secretion
Constitutional
CNS tumors (hamartomas/gliomas)
ANY condition affecting the CNS
Peripheral:
Goanad/adrenal tumors
CAH- females: virilized, Males: nml at birth, small penis deveopment |
|
|
Term
| MCC of GnRH independant precocious puberty; polystotic fibrous dysplasia(deformity, fragility), cafe au lait spots, MC in females |
|
Definition
|
|
Term
Adult odor, pubic and axillary hair, facial oiliness, acne
Breasts/uterine development then menarche
Testicular enlargment=
Labs needed and expected results
What imaging is needed? |
|
Definition
Male/Female androgen excess
Female estrogen excess
>2.5cm, gonadarche- central
<2.5cm, peripheral (adrenal/exogenous source)
LH & FSH (central = elevated, peripheral= decreased)
CT/MRI evaluate for central causes= tummor |
|
|
Term
| Tx for Precocious puberty (Central) |
|
Definition
Tx any underlying d/o
Most are idiopathic(central)--> endocrinology referral, GnRH analogues (leuprolide)-->decrease pituitary-gonadal activity/menses cease/growth velocity decreases/ slow sexual maturity |
|
|
Term
| Tx of peripheral causes (ie. McCune-Albright Syndrome) |
|
Definition
| Do not respond to GnRH analogues-->Antiandrogens (testolactone) or Antiestrogens (tamoxifen) |
|
|
Term
GnRH analogues=
Antiandrogens=
Antiestrogens=
Thyroid=
Anti-Thyroid=
CAH= |
|
Definition
Leuprolide
Testolactone
Tamoxifen
Synthroid
Methemoxizole/ PTU
Hydrocortisone/Fludrocortisone |
|
|
Term
| Complication of UnTx precocity? |
|
Definition
| Short stature-->early closure of epiphyses--> <5th%-ile |
|
|
Term
Testicular/ Thelarche then Menarche=
Excessive Pubic/ axillary hair, acne, odor, facial hair= |
|
Definition
Central, High LH/FSH, ID Hormone levels, MRI of the head
Peripheral (adrenals/exogenous), DHEAS level, MRI of adrenals) |
|
|
Term
Hypogonadism definition
MCC
Other cause |
|
Definition
1. puberty delayed >2.5 SD after normal age; no breast by 13y/o, no testicular enlargment by 14 y/o
2. Central-Hypogonadotropism- Low LH/FSH- MC
Gonads - Hypergonadotropism-high LH/FSH |
|
|
Term
Hypogonadotropic Hypogonadism= define, causes
Hypergonadotropic, hypogonadism=" " " |
|
Definition
Low LH/FSH; GnRH inhibition secretion, Hypothalamus/pituitary dz
High LH/FSH; Gonadal failure |
|
|
Term
What type of delayed onset:
1. Malnutrition(female athelete triad), Stress, Chronic dz(hypothyroidism)
2. Idiopathic/ tumors
3. Turner syndrome, Klinfelter syndrome, androgen insensitivity, PCOS |
|
Definition
1.Hypo/Hypo; GnRH secretion inhibition
2. Hypo/hypo; Hypothalamus/pituitary dz
3. Hyper/Hypo; Gonadal failure |
|
|
Term
Pt with:
1. Genetically male due to XY chromosomal analysis. Under-responsive androgens-->feminization w/ incomplete virilization. Both sexes are present, and can be raised as either sex.
2. genetically male chromosomal analysis. External feminization and raised as such (female). U/S reveals no ovaries/fallopian tubes, uterus, but intra-abdominal testes are ID |
|
Definition
1. Androgen Insenstivity
2. Complete androgen insensitivity |
|
|
Term
How is Hypogonadism Dx and Tx?
Labs for:
Hypergonadotropism
Hypergonadotropism |
|
Definition
Order LH/FSH for both
1. Karyotype (ID Turner/Kleinfelter), PE and U/S to detect the presence of gonadal organs
2. TSH, prolactin and head MRI-->CNS and metabolic pathology
3. PUNT TO ENDO |
|
|
Term
Causes of:
Hypothyroidism
Hyperthryroidism |
|
Definition
1. Congenital hypothyroidism (aplasia/hypoplasia)/ Aquired hypothyroidism
2. Graves Dz/ Multinodular Goiter |
|
|
Term
| Classic organ system for example of releasing hormone/ end hormone system? (Gay, but fitting for the instructor to ask) |
|
Definition
|
|
Term
Pt with: jaundice, respiratory distress, macroglossia, umbilical hernia, >5cm anterior fontonel / >1cm posterior fontonel, Type II FTT
Labs to order?
Tx- When to start? Check levels how often? |
|
Definition
1.Congenital Hypothyroidism
2. Detected by state mandated blood tests once they are born, TSH (high), T4/T3 (Low)
3. Synthroid- <1mo start Tx; re-check in 6 wk |
|
|
Term
Peak incidenc 8-15y/o, MC in females, growth retardation, poor school performance, firm, symmetric, non-tender mass on the anterior cervical region.
Name
MCC
Dx-Labs and confirmatory test
Tx- Rx and timing |
|
Definition
1. Aquired Hypothyroidism
2. Hashimoto's (CLT)
3. Lab:elevated TSH, low T4/T3, Antithyroglobin and antithryoidperoxidase AB, FNA confirms
4. Synthroid, q 6 wk f/u until stable then annually |
|
|
Term
Pt: 12-14y/o, usually female, wt loss, tremor, heart rate, proptosis, flushing, fine straight hair, finger clubbing, diarrhea, menstrual changes(amennorrhea), muscle wasting, heat intolerance
2. Causes
3. Dx
4. Tx algorithm- S/E, indications |
|
Definition
exopthalomos
1. Hyperthyroidism
2. Graves dz, multinodular goiter, acute thyroidits, exogenous ingestion of thyroxine
3. Low TSH, elevated T4/T3
4. Propranolol-tachycardia/palpitations/anxiety
PTU(granulocytopenis and lupus)
methimoxazole
RIU 131- PTU/methoxazole failure after 2 yr
Subtotal thyroidectomy-if Radioablation does not work |
|
|
Term
Adrenal dysfunction:
1. Low K, High Na, fluid retention, HTN
2. Adiposity, weakness, purple stiae, HTN, Hyperglycemia
3. Hirsutism, acne, virilization |
|
Definition
1. Aldosterone excess
2. Cortisol excess
3. Androgen excess |
|
|
Term
Pt with: Hyperpigmentation, salt craving, apneic spells, hypoglycemic, hyponatremia, hyperkalemia, acidosis, shock. Rapid progressing. Death may occur in HOURS, spontaneous recovery is possible
Name
Tx |
|
Definition
1.Adrenal Crisis (Acute Adrenal 2.Insufficiency)- CAH caused
Initially- IV hydocortisone
Fine tune later- Fluorinef |
|
|
Term
Cause of:
Adrenal Crisis?
Addison's Dz?
Iatrogenic Adrenal Insufficiency? |
|
Definition
1.stems from poor secretion of cortisol (primary/secondary)
2.Hereditary- dysfunctional enzyme
Aquired- autoimmune destruction
3. chronic oral corticosteroid d/c |
|
|
Term
Pt with:
salt craving, vomiting->dehydration, FTT, hyperpigmentation
Dx?
Tx? |
|
Definition
1. Addison Dz
2. ACTH stimulation test (dosyntropic stimulation test)
3. Hydrocortisone, dexamethasone |
|
|
Term
Definition of chronic use in Iatrogenic Adrenal Insufficiency?
MC conditions this is found in?
Tx? |
|
Definition
1.>2wk of prednisone or >10days of high dose corticosteroid use
2.Asthma/Nephrotic syndrome/ITP
3.Taper the dose to prevent |
|
|
Term
MCC of intersex (virilized genitalia)
Etiology
Intersex defined |
|
Definition
1. CAH- androgen excess
2. AR 21-hydoxylase deficiency
Low cortisol
Excess ACTH->increase DHEAS (caused from both Primary(adrenal)/Secondary(pituitary)
Androgen excess
3. pseudohermaphrodism and hermaphroditism |
|
|
Term
Pt: born female, develops pubic hair, deep voice, increased linear growth (height/muscle) by 2y/o leading to hyponatremia, hyperkalemia
Name
Dx
Tx |
|
Definition
CAH leads to adrenal crisis found on different NC
Lab: High 17 hydroxy progesterone->lack of 21-hydroxylase
3. Hydrocortison- suppress androgen production
Fludrocortisone- supplement aldosterone
Cosmetic plastic SX |
|
|
Term
Excess pituitary ACTH->adrenal over stimulation of Cortisol
Cause
Tx |
|
Definition
1. Cushing's Dz (brain)
2. Pituitary adenoma
3. SX |
|
|
Term
Excess ACTH NOT due to a mass in the brain
Causes
Tx |
|
Definition
1.Cushing's Syndrome
2. Iatrogenic Glucocorticoid excess (ACTH is low)
Adrenal carcinoma/ adenoma
ACTH secreting pulomonary tumor |
|
|
Term
| Pt with: Central obesity, purple stiae, moon facies, HTN, Glucose intolerance, muscle atrophy, glucose intolerance, muscle atrophy, fluid retention, plethora (flushed, full face) |
|
Definition
|
|
Term
1. Time frame of gonadal differentiation
2.What causes testicular development?
3.What does testosterone do in utero?
4.Actions of DHT? |
|
Definition
1. 9-13 wks
2. SRY (Sex determing region Y) gene
3. develop Wolffian ducts->vas degerens/ prostate
Mullerian duct obliteration(fallopian tubes)
Testosterone -> 5 alpha reductase-> DHT
4. Labia fusion->scrote
Clitoris fusion->penis |
|
|
Term
1.MCC of Female Pseudohermaphrodism? Think disfucntion here first?
MCC result of Male 2. Pseudohermaphrodism? Think first deficiency here first?
3. Dx
4. Tx |
|
Definition
1. CAH; adrenal source
2. Hypspadius; abd testes; deficient produciton/ insensitive end organ receptor
3. U/S or PE finding
4. Initially - Tx adrenal crisis
Refer out (multi-team approach)
SX (~2 y/o) and hormonal correction |
|
|
Term
|
Definition
Antidiarrheal agents
Antiemetic agents |
|
|
Term
Antidiarrheal agents: cause, recommendations
Anitemetic agents: Risks associated with older agents |
|
Definition
1. Worse infectious/ inflammatory diarrhea; may cause toxic megacolon(Hirschprung's); cautious about recommending
2. Risk of EPS, acute dystonic rxn and sedation outweight benefits-Zofran
Vomiting usually self limiting and improves w/ rehydration |
|
|
Term
ABX typiclly not needed for AGE but may be used if:
1. Shigella:
2. Salmonella:
3. E. Coli:
4. C. diff:
5. Giardia: |
|
Definition
1. IV 3rd gen cephalosporin/Azithromycin/Cipro(if old enough)not Tx can be life threatening
2. Ampicillin- must Tx,severe infection/<3mo only;
3. Trimethroprim - NOT for O157:H7, severe infection only
4. Metronidazole(mild); Vanc(severe); Difcid; Interfecal Transfer (parent/sibling/spouse)
5. Metronidazole |
|
|
Term
Consequences of not treating Shigella?
Draw back of Tx salmonella? |
|
Definition
1.high fever-->Siezures, intestinal abcess
2. prolongation of organism excretion |
|
|
Term
Summary of Acute Diarrhea:
non-bloody,afebrile=
nonbloody, febrile =
febrile, bloody=
afebrile, bloody= |
|
Definition
1. viral; rehydration
2. viral; fecal leucocyte/stool culture
3. bacterial; ABX if severe (macrolide/FQ)
4. consider HUS/IBS/Intusception |
|
|
Term
How to asses the complications of dehydration?
Prognosis of uncontrolled dehydration?
MC months to happen and why? |
|
Definition
1. v/s(BP not helpful only use Pulse)
General impression
Mucous membrane appearance
Tear production/absent
Cap refill (<2s)
Tugor/ Tenting
2. electrolyte imbalance-->fluid shift/cardiac arrhythmias-->vascular instability-->shock-->death
3. Oct-Feb; Rotavirus |
|
|
Term
Pt with: <5y/o, irritable, slight skin tugor, nml orthostatic BP, Dry mucus membranes, oliguria
Tx |
|
Definition
Moderate Dehydration
2. PO fluids- if can keep it down-->IV if not |
|
|
Term
Pt with: <5y/o, ill appearing, hypotension, sunken fontennels, parched mucus membranes, anuria
Tx |
|
Definition
Severe Dehydration
2. IV hydration; hospitalization |
|
|
Term
Pt with: >6y/o, ALOC, apprehensive, muscle cramps, hypotension, sunken fontennels, parched mucus membranes, anuria
Tx |
|
Definition
Severe Dehydration
2. IV hydration; hospitalization |
|
|
Term
What lab value should be given before replacement?
Deficit Equations:
Water deficit
Potassium deficit
Sodium deficit-->complication |
|
Definition
CMP value
1. % dehydration X (current)Kg
2. Water deficit X 30mEq/L-->round to nearest even number!! (30.9=30)(31.1=32)
3. Water deficit X 80mEq/L; if HYPERnatremic may lead to cerebral edema |
|
|
Term
Tx of:
Hyponatremia
Hypernatremia |
|
Definition
|
|
Term
Oral rehydration Tx for AGE
Mild-
Moderate-
Why not severe?
The higher the osmolality=
How to bypass an upset stomach? |
|
Definition
1.Use commercial product (-lyte)
2. 50mL/Kg q4h
3. 100mL/Kg (gtt/24h)
Tx until diarrhea resolves
4. requires IV Tx
5. more likely to cause diarrhea
6. feed tsp/3min |
|
|
Term
Tx of severe AGE:
type of Tx
For who
How much
How often |
|
Definition
1. IV Tx
2. moderate-severe/ unable to take PO
3. 20mL/Kg IV bolus (NS/LR) over 20min
(10mL/Kg for neonates)
4. Repeat prn dictated by v/s and LOC |
|
|
Term
| Maintainance Tx of IV fluids: |
|
Definition
100mL/Kg/d or 4mL/Kg/h for first 10Kg
50mL/Kg/d or 2mL/Kg/h for second 10Kg
20mL/Kg/d or 1mL/Kg/h for >20Kg |
|
|
Term
Steps for replacement Tx:
Type of maintainance fluids:
Time frame: |
|
Definition
1. Give fluids for bolus for first 20min
2. Start Continuous fluids= (Maintainence+remaining water deficit+potassium deficit equation)/24h
3. D5NS; D5, NS25 W25 (<20kg)
3. 1/2 given over first 8h; 1/2 over next 16h |
|
|
Term
Chronic diarrhea
definition
Causes |
|
Definition
1. >2wk
2. Same as AGE but longer
(decreased absorption, increased intestinal permeability(secretory), increased motility |
|
|
Term
Examples of:
Decreased abosorption-
Increased secretion- |
|
Definition
1. decreased cellular transport-Celiac sprue/lactase deficiency
Loss of surface area- IBS/ resection
Unabsorbable osmotic particles- Sortibol/sylitol/polyethylene glycol/lactulose
2. typical cause of diarrhea |
|
|
Term
Etiology of Chronic Diarrhea:
General |
|
Definition
Enzyme deficiencies
Allergies
Chronic GI infections
Inflammatory bowel dz |
|
|
Term
Etiology of chronic diarrhea:
Enzyme deficiencies-
Allergies-
Chronic GI infections-
Inflammatory bowel dz- |
|
Definition
1. Disaccharidas deficiencies-lactase
CF-Lipase
2. Celiac dz (gluten enteropathy)
Milk Protein allergy (children <12m.o.
3. Parasites (ie Giardia)
4. IBS (UC/ Chron's dz) |
|
|
Term
Pt with: diarrhea >2wk, not acutely ill
General Dx?
Management? |
|
Definition
1. Chronic Diarrhea
2. CBC, chem 7, stool O&P, C&S, stool pH and reducing substance, referral for definitive Dx
3. general screening tests to r/o common malabsorptive and infectious etiology |
|
|
Term
Chronic Diarrhea:
What does stool pH measure?
How is stool reductase tested? |
|
Definition
1. colonic bacteria take up unabsorbed sugars releasing acid (low pH)
2. Once substances are reduced a color change is seen (Cupric-->cuprous ions) |
|
|
Term
Chronic Diarrhea-->Hx-->d/o--> Dx specific tests done-->Tx:
Delayed stooling since birth
Respiratory infections, oily stools
FTT, thrush
Bloody diarrhea, afebrile, cramps
ABD pain
Sx start with intak eof wheat/ rye
Too much cold Juice |
|
Definition
1. Hirschprung-Endoscopy w/ bx
2. CF-sweat chloride test/ FISH
3. IBD-Endoscopy w/ bx
4. Bacterial/parasitic
5. Celiac dz-endomysial, antigliadin, and transglutaminase AB's
6. IBS-Endoscopy w/ bx-STOP the juice
(Most of these are referral for Definitive Dx) |
|
|
Term
1.Cause by overiding the Gastrocolic reflex
2.standing or sitting w/ legs extended and stiffened or cross legged |
|
Definition
Constipation
2. Retentative posturing |
|
|
Term
<2 stools/wk or passage of hard, pellet-like stools for >2wk
Prolonged straining and crying followed by passage of soft stool, self limiting, resolves by 3m.o.
Voluntary witholding of stool leading to infrequent, large-caliber, often painful stooling. ofetn leads to encopresis |
|
Definition
1. Constipation
2. Infantile dyschezia
3. Functional fecal retention |
|
|
Term
2 kinds of constipation
1. MCC, fromno neonatal constipation, active fecal retention, infrequent large stools
2. Anatomy, nutrition/dz causing abnormal function, no distinct pattern. Present from birth-->causes |
|
Definition
1. Functional
2. Organic--> Hirshprung's, Anorectoal malformation, spinal cord defects, multisystem dz, medications(psychotropics, narcotics) |
|
|
Term
Constipation:
Occurance MC when (lifesyle change; age)?
MCC related to :
We don't know
Decreased fiber/fluids
avoident behavior
Causes not to miss?
-no honey in <1y/o
-meconium ileus
-spina bifida
-pain chips
-asynaptic colitis
-fat/myedema coma/ dry skin/ increased Diastolic BP |
|
Definition
1.introduction of solid food; 6m.o.
Toilet training; 2-3 y/o
Start of school; 4-5 y/o
2. Chronic, idiopathic
Dietary
Painful
3. Intantile botulism
CF
Spinal dysraphism
Lead poisoning
Hirshsprung's dz
Hypothyroidism |
|
|
Term
Constipation:
Labs to order with presentation of:
Hirschrung's
Malformation
Spinda bifida
DM/Thyroid/Celiac |
|
Definition
1. Colonic/rectal bx
2. BE
3. Radiographs
4. FBG,2HPP, Ha1C/ TSH, AB/ AB associated |
|
|
Term
Constipation->Acute Tx for:
Infants
Children/adolescents |
|
Definition
1. Diet change, rectal stimulation, glycerine suppositories
2. Diet change, medications |
|
|
Term
Acute Tx of constipation->diet change for:
Infants
Children/adolescents |
|
Definition
1. add sorbitol/PEG/corn syrup- 1/2-1 tsp/ cup of formula hs
2. increase fiber and water intake and exercise |
|
|
Term
Acute Tx of constipation->children/adolescents
Medications used:
Best choice overall
For children >3m.o. |
|
Definition
1.Osmotic laxitives(sorbitol/lactulose/polyethylene glycol/magnesium citrate
2. Stimulant laxitives (senna, ducolax, caster oil)
3. Fleets Enema |
|
|
Term
Chronic Tx of Constipation
Education-
Stool softeners- when to give them, kinds |
|
Definition
1. maximize gastro-colic reflex->5min toilet time q a.m. and p.c.
2. Analgesia BM must be present
Polyethylene glycol
Milk of magnesia
Mineral Oil |
|
|
Term
Anemia:
1.Definition
2.Normal Hgb values
3.Definition of Physiologic "anemia" of infancy?
4. Recommendations by AAP? and UPSTF high risk pt, who are these groups? |
|
Definition
1.Hgb/Hct more than 2SD below the mean for age
2.Newborns: 17
2m.o.: 11
1 y/o: 12.7
3. Notice a drop during first 6mo of life-->often misDx as anemia (Hgb F-->Hgb A)
4. AAP-->6-12m.o.
USPTF-->6-12mo of life; Black/ Native American/ Alaskan/ Poverty/Immigrant/Premature/SGA infant |
|
|
Term
Pt with: Pallor, jaundice, petechiae, purpura, hemorrhage, impaired cognition, FTT.
Concurrence with what? |
|
Definition
1. Abnormality of platelets/coagulaopathy
2. Thrombocytopenia/pancytopenia |
|
|
Term
Pt with: pallor, jaundice, fatigue, HF, Tachycardia, Hepatosplenomagaly, Koilonychia.
Dx: |
|
Definition
Anemia
CBC/Retic count/ Peripheral smear |
|
|
Term
Pt with anemaia what is seen on:
1.CBC
2. >2% - healthy bone marrow response to anemia; first parameter to improve in Tx
3. ID structural abnormalities- spherocytes/shistocytes/sickle cells |
|
Definition
1. Hgb/Hct-makes Dx
MCV(RBC size)-macro/micro/normocytic
RDW
WBC/Platelet count
2. Reticulocyte count
3. Peripheral Smear |
|
|
Term
Abnormalities in labs mean:
RDW=
Elevated:
Normal:
WBC/Platelet count
low= |
|
Definition
1. RBC destruction
2. Low production
3. Pancytopenia- bone marrow problem |
|
|
Term
1.Size of RBC's and directs your evelation?
2.d/o indicated by:
Typically indicaticative of Hgb problem--> Fe deficiency/structural?
Chronic disease, infection, hemorrhage
B12, folate, thyroid, liver |
|
Definition
1. MCV
2. Low MCV
Nml MCV
High MCV |
|
|
Term
| Common DDX of Microcytic Anemia: |
|
Definition
FLATS:
Fe deficiency
Lead poisoning
Anemia of chronic dz
Thalassemia
Sideroblastic anemia (rare) |
|
|
Term
| On Microscope you see >1/3 hypochromia of RBC and small RBC. What is possibly going on, with no chronic dz or Pb? What should you do? And when should recovery be seen? |
|
Definition
1.Microcytic Anemia--> MC Fe defiency
Start 4-6 mg/kg/d of Fe
Reticulocytosis should be seen in 3-5d
Increase Hgb in 1mo
Tx:3-4 mo
Further evaluation if no response |
|
|
Term
1.Pt: microcytic anemia, pallor, tachycardia, jaundice. Fe deficiency is ruled out?
2. How is sideroblastic anemia Dx? what is seen? |
|
Definition
1.Thalassemia
2.Prussian blue staining Bone Marrow bx; Sideroblasts; found on peripheral smears--nucleated RBC's |
|
|
Term
What are the usual causes of Fe deficiceny anemia?
What d/o has basophilic stippling on peripheral smear? what is done next? |
|
Definition
1. Insufficient Fe intake (non-fortified foods/ Cow's milk consumption <12y/o
2. Pb poisoning, screening questionnaire at 12 and 24 m.o.--> house built before 1950--> if positive test for Pb level |
|
|
Term
| What does cow's milk due to pt <12y/o? |
|
Definition
| inhibits Fe absorption/lows in Fe content/GI allergy (protien)-->GI bleeding/ lactase deficiency |
|
|
Term
Normocytic anemia:
categorized 2 ways-->name-->RDW measurement-->types in each category
w/u-tests conducted
Tx |
|
Definition
1. Decreased RBC production (RDW nml)--> Transient erythroblastopenia of childhood/anemia of chronic dz/ Bone marrow failure
2. Increased RBC destruction (RDW high); G6PD/Sickle cell/Spherocytosis
3. Erythroblasopenia-->Hct/Hgb
Anemia-->MCV
Bone marrow failure-->complete CBC
G6PD--> simple blood test
Sickle cell--> Hgb electrophoresis
Spherocytosis--> fragility test
Marrow bx
Peripheral smear |
|
|
Term
Lab test that measures the difference in RBC size?
Volume of the RBC derived from the Hct value? |
|
Definition
|
|
Term
|
Definition
| Shistocytes (broken RBC)-->increased serum/free hgb(hemoglobinuria)-->decreased haptoglobin-->increased bilirubin |
|
|
Term
Low haptoglobin + High bilirubin =
Causes of hemolysis:
Intrinsic
Extrinsic |
|
Definition
1. Hemolysis
2. genetic-SSA/Thalasemia
3. Infection
Rx induced- PCN/sulphas/ APAP(acetomenophen)
Autoimmune- JRA/SLE
Leukemia/Lymphoma
Trauma (running/marching) |
|
|
Term
Common normocytic anemias:
Name->Cause |
|
Definition
1. Transien erythroblastopenia of childhood->RBC hypoproduction due to BM suppression from infection (erythroid hypoplasia)
2. Infectious agents causing pancytopenia->severe aplastic crisis that lasts 1-2 wks |
|
|
Term
Common normocytic anemia:
similarities and differeces
TEC v. Infectious agents causing pancytopenia |
|
Definition
Similarities:
slow onset over 1-2 mo in 1-4 y/o; ASX
Differences:
TEC:caused by HHV6/parvovirus B19;longer lasting; transient and resolves spontaneously
Infectious agents: lasts 2wks; immunocompromised/sickle cell pt; supportive Tx through blood transfusions/IV |
|
|
Term
| Congenital pure RBC aplasia, Triphalangeal, short stature, wide eyes, snub nose, hypogonadism, retardation |
|
Definition
| Diamond-Blackfan Syndrome |
|
|
Term
Macrocytic Anemias DDx:
Name
w/u
Tx |
|
Definition
FLAHB
Folate deficiency->folate level->replace folate->CBC improved <1wk
Liver dz->LFT's->Tx any underlying liver dz
Alcohol->LFT/folate->Tx hepatic d/o/ replace folate
Hypothyroidism->TSH->Tx underlying thyroid d/o->CBC improved <1wk
B12 deficiency->B12 value->replace B12->impovement CBC <1wk |
|
|
Term
Failure to produce enough of the proper Hgb despite adequate precursor supply
Types |
|
Definition
Hemoglobinopathies
Thalassemia
Sickle cell dz
Pyruvate kinase deficiency
G6PD deficiency |
|
|
Term
Insufficient production of normal Hgb due to decreased production of either alpha or beta glob components of hemoglobin
How many genes code for protiens?
Mutations typically seen in which race? |
|
Definition
1. Thalassemia
2. 4 protein chains; 2 genes for 2 protiens(alpha/beta)
3. Alpha mutations- Asians->chromosome 16
Beta mutations - Blacks->chromosome 11 |
|
|
Term
| Beta thalassemia major aka |
|
Definition
|
|
Term
Alpha thalassemia mutations:
Type
Sx
Dx
Only one that shows Tx |
|
Definition
1. 1 gene mutation - "silent"->ASX->Dx through genetic study
2. 2 gene mutation- "Alpha thalassemia trait" ->ASX-> Dx through mildly decreased Hgb/Hct:microcytosi, increased RBC number with nml retic, r/o IDA, Hgb electrophoresis nml
3. 3 gene mutations "Hgb H disease"-> severe anemia-> Dx through Hgb electrophoresis = high Hgb H, Folic acid supplementation-> transfusions during crisis-> genetc counseling(dominant)
4. 4 gene mutations "Hydrops Fetalis"-> fetal death |
|
|
Term
| Causes of Hydrops fetalis: |
|
Definition
| ABO/Rh incompatibility, 4 alpha gene mutations, 5th dz |
|
|
Term
Beta thalasemia mutations:
Name
Sx
Dx
Only one that shows a Tx |
|
Definition
Beta thalassemia minor (heterozygous)-> 1 beta gene mutation->ASX->Dx through Hgb electrophoresis(~7) high in A2, Hgb F (infants body to compensate for lack of HgbA)->2 alpha and 2 delta chains
2. Beta Thalessemia Major "Cooley's Anemia"-> homozygous->severe anemia w/ frequent cisis needing regular transfusion by 2m.o.->Hgb electrophoresis high Hgb F->Iron overload common->Marrow transplantation curative if HLA donor matches |
|
|
Term
| Best way to asses Fe overload in Cooley's Anemia and how to correct? |
|
Definition
Liver bx
Fe chelation-> deferoxamine SQ-> binds and prevents absorption-> retino/oto toxic |
|
|
Term
Sickle Cell- MC found in which race?
Dz-mutation in which component?
Trait-mutation in which component? |
|
Definition
Blacks
Homozygous->Hgb S
Heterozygous->Hgb A |
|
|
Term
Pathophysiology of Sickle cell:
What worsens this d/o? |
|
Definition
Hgb S polymerizes at low pO2/low pH->RBC distortion->gets stuck in arterioles/capillaries->Thrombosis/sequesterateion/infarction->usually spleen
2. Hypoxia, acidosis, pyrexia, dehydration |
|
|
Term
| Pt with: onset after 6m.o. with dactylitis, vaso-occlusive espisodes in extremities(young)/chest and back(older) leading to pain in that area |
|
Definition
|
|
Term
| How to differentiate Sickle cell in a neonate from an older child? |
|
Definition
|
|
Term
Pt with: splenomegaly, dactylitis, priapism
Acute Tx
Chronic Tx |
|
Definition
Sickle cell
>4 splenomegaly <13 due to scarring down
Priapism >13-->impotence
2. Commonly hospitalized for
pain crisis-->narcotics, fever, thrombotic complications-sequestration crisis, stroke, seizure
Tx with oxygen, IV hydration, analgesics, blood transfusion, ABX
3. Hydroxyurea->increases HgF
Bone Marrow Transplant-> <16 w/ match
Prevention->PCN 2m.o.-5y/o; immunizations, folate supplementation
Referral |
|
|
Term
| Complications of Sickele cell |
|
Definition
Asplenia
Stroke
Aplastic crisis
Splenic sequesteration crisis |
|
|
Term
Usually pt:
are aplenic by what age? #1 cause of death? common cause of osteomylitis?
Usual cause of aplastic crisis? |
|
Definition
1. 5y/o
2. Strep pneumo
3. Salmonella
4. Parvovirus B19 |
|
|
Term
| Pathophysiology, presentation, Dx, and Tx of Pyruvate kinase |
|
Definition
1. PK creates ATP through glycolysis->lack of ATP->Na/K pump failure->rigid RBC structurally unstable/degrades
2. hemolysis->anemia (jaundice, pallor)
3. Special testing
4. Neonates->exchange transfusion
Splenectomy may be helpful |
|
|
Term
|
Definition
1. G6PD reduces glutathion->absorbs free radicals, too much glutathion->increase free radials->damage Hgb->Heinz bodies->RBC membrane fragility->hemolysis
2. Hemoglobinuria, anemia (pallor, jaundice) w |
|
|
Term
Hemoglobinuria, anemia (pallor, jaundice) after 24-48h with ingestion of Fava beans/sulfa drugs/ ASA/ Primaquine/chloraquine
Dx-
Tx |
|
Definition
G6PD deficiency
2. Measure G6PD enzyme
3. Avoid oxidative drugs/foods |
|
|
Term
Cluster of denatured Hgb in a RBC?
Found in what d/o? |
|
Definition
Heinz body
G6PD deficiency,Chronic liver dz, thalassemia |
|
|
Term
MC bleeding d/o of childhood.
common age range
Pathophysiology |
|
Definition
1. ITP
2. 1-4wks post viral infection-->EBV/HIV
3. Autoimmune-> anti-platelet IgG/IgM |
|
|
Term
Pt with: petechia on lips/buccal mucosa, multiple bruises on their skin, parent complains of epistaxis/bleeding gums/hematuria
1. name
2. labs to run
3. Acute Mild Tx
4. Acute Severe Tx
5. Chronic Tx |
|
Definition
1. ITP
2. Platelets <50K, WBC/RBC nml, peripheral smear is normal, PT/PTT normal, Bone bx shows increased number of megakaryocytes (body's compensation for decreased platelets)
3. None; resolve in 6mo; sepsis/severe bleed
4. Plt <20K= IVIG(1-2d), Prednisone(2-3wk),IV anti-D in Rh(+) pt, splenectomy
5. >6mo: r/o secondary causes(SLE/HIV), splenectomy |
|
|
Term
|
Definition
A: factor VIII deficiency, MC
B: factor IX, rare
Both are X-linked->commonly found in men |
|
|
Term
Hemophilia:
Dx-lab values, definitive test
Tx |
|
Definition
1. Coagulation studies: PTT prolonged, PT and BT(bleed time) usually normal, PTT corrects with mixing study
Factor assays: measure amount and function provide, definitive Dx
2. Avoid trauma, replace factor->refer to hematology, Desomopressin(DDAVP)->low level hemophilia A by increasing VIII and vWF |
|
|
Term
Bleeding into joints makes you think of...
Bleeding into skin(petichea/bruising) makes you think of... |
|
Definition
1. hemophilia
2. platelet d/o |
|
|
Term
vWF dz
purpose
subtypes and definition |
|
Definition
deficiency in quantity/function of vWF
1. binds platelets to endothelium/ prevents factor VIII degradation
2. I- decreased production; AD; MC
II- nml production; defective vWF
III- no production of vWF |
|
|
Term
Pt with: mild bleeding associated with trauma which the parent/pt also complains of bleeding gums/heavier menses/prolonged bleeding/epistaxis/mucocutaneous bleeding
Dx
Tx |
|
Definition
1. vWF dz
2. vWF level/function measured-> Ristocetin(induces vWF to bind to platelets)
3. DDAVP-> I/II
vWF concentrate |
|
|
Term
Henoch-Schonlein Purpura aka...
Pathophysiology
Common organs found in
Age range
Caused by |
|
Definition
Vasculitis of the small b/v especially skin/GI/renal vessals
3-15y/o, MC in boys, IgA reaction->by viral/bacterial URI's/medications |
|
|
Term
Pt with: abdominal pain, polyarthralgias palpable purpura on LE/buttocks, arthritis, renal, GI effects, LE edema
Dx
Tx |
|
Definition
1. Henoch-Schonlein Purpura(HSP)
2. Labs: CBC->platelet count is nml; WBC/ESR/CRP normally elevated; UA and Chem 7->renal dz; stool for occult blood
Made >=2/4: palpable purpura, bowel angina(pain/documented bowel ischemia), Dx bx( granulocytes in arteriole/venule walls), <20yrs at onset
3. Supportive:
CrCl/BUN->Renal function
NSAID-arthritis
Steroids->renal dysfunction/GI ischemia
Prognosis is good except if pt goes into renal failure->elevated BUN, >=2+ protienuria |
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Term
|
Definition
ITP->plt count will be low on CBC
Abuse->pattern of skin findings
Meningococcemia->febrile/ill looking |
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Term
HSP v. Kawasaki
Similarities
Differences |
|
Definition
1. Autoimmune vasculitis
2. HSP- Small b/v; 6m.o.-5y/o
Kawasaki- >small b/v; 3-15y/o (rare >7) |
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Term
Pt with: 1-2wks of High fever >5d (>103), marked conjunctival, oral mucosa, tongue, lip erythema (strawberry tongue), cervical LAD, scarlatina-like rash- <24hr post-pyrexia
Dx
Tx |
|
Definition
| 1. Kawasaki dz- Acute phase |
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Term
|
Definition
| Acute, sub-acute, convelescent |
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Term
| Pt with: 1-2 wk of skin desquamation, coronary artery aneurysm, MI is possible |
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Definition
| Kawasaki dz-Subacute phase |
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Term
| Pt with: 2-4 wks skin desquamation resolution, cardiac lab values normalize |
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Definition
| Kawasaki dz-convelescent phase |
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Term
Dx of Kawasaki dz:
Clinical criteria
Tests |
|
Definition
Fever >5d w:
a. Bilateral conjunctival injection
b. 1+ of: pharyngeal injection/ dry fissured lips/ injected lips/ strawberry tongue
c. 1+ of extremity changes:
Peripheral erythema/ peripheral edema/periunguinal, generalized desquamaton
d. Cervical LAD
e. Truncal Rash
Labs: BC/UC (r/o infection); WBC, ESR, CRP |
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Term
DDx for Kawasaki:
How to differentiate |
|
Definition
| Scarlet fever->ARF->TC/lacks conjunctival and lip findings |
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Term
| Tx and complications of Kawasaki: |
|
Definition
1.Imaging for coronary artery aneurysms-> echo q 2-3wk to asses aneurysms
2. Mainstay of Tx->IVIG(prevent Coronary aneurysm) + Predisolone(better than ASA)/ASA(adm <48h after onset; Reye's syndrome: Benefit>Risk) |
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Term
|
Definition
|
|
Term
| #1 and #2 leading childhood mortality |
|
Definition
|
|
Term
|
Definition
Prolonged LAD (>4wks)
Prolonged limp-->esp if atraumatic
Persistant HA-->esp in the morning-->vomiting/awakens
Morning vomiting
F/C/NS/wt loss |
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Term
Kids with Cancer PE should focus on?
Size
General Impression
Palpation
Immune system
Skin exam
continuity if HA or N/V |
|
Definition
Growth and V/S
Gen Impression: Color, cachexia, pain, ill appearance, distress
palpate masses
Immune--> ID/quantify organomegaly/LAD
Skin Exam-->rashes/petechiae/ bruises
Neuro/endo/ophthalmic exams |
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Term
| Best Screen for many pediatric malignancies? |
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Definition
| CBC with peripheral smear |
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|
Term
LABS/RADS--->what to order when?
Any suspicion of Cancer and first test ordered?
Elevated in fast growing tumors?
Electrlyes/renal/hepatic function assesment?
Fever/respiratory Sx?
Abd tumor palpated/suspected?
Bone Tumor? |
|
Definition
1. CBC w/ peripheral smear
2. Serum LDH/uric acids
3. Chem 7/ CMP (CrCl/BUN/LFT/ Ion levels)
4. PA/Lat CXR
5. Abd CT/ U/S
6. Extremity MRI |
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Term
|
Definition
1. SX resection
2. Chemo
3. Radiation |
|
|
Term
General Tx of Cancer-->
1. Helpful in solid tumors, but not leukemia; BUT is helpful in lymphoma
2. Highly effective in most pediatric cancers
3. less effective in pediatric tumors
4. S/E of Cancer Tx |
|
Definition
1. SX resection-->aided by chemo/radiation
2. Chemotherapy
3. Radiation
4. Chemo/radiation Tx-->cause new malignancy/Infertility/Hepatotoxicity/Sepsis/Gland defects |
|
|
Term
Definition:
Cancer in place
Post excision of cancer |
|
Definition
1. Neoadjuvant
2. Adjuvant |
|
|
Term
How is sepsis cause by chemo?
Specific glands affected by radiation? |
|
Definition
1. Neutropenia->susceptible to encapsulated bacteria post op splenectomy
2. Pituitary/thyroid |
|
|
Term
Oncologic Emergencies:
Name-->caused by |
|
Definition
1. Sepsis-->Neutropenia(chemo)
2. Tumor lysis Syncrome--> hyperkalemia/hyperphosphatemia secondary to lysis of cells
3. Anemia/Thrombocytopenia (chemo)
4. Increased ICP-->brain tumor
5. Tracheal compression-->mediastinal mass |
|
|
Term
MC pathogens due to cancer:
Bacteria
Fungal
Protozoa
Viruses |
|
Definition
1. S. Aureus/ E. coli, Psueomonas, Kliebasella
2. Candida/Aspergillus
3. Toxoplasmosis
4. CMV/HSV |
|
|
Term
| Unusual infections of cancer? |
|
Definition
Central catheter/port infections
Atypical pneumonia (P. Jirovecii)
Aspergillus
HSV
CMV |
|
|
Term
| Cancer pt with infection. Next step? |
|
Definition
| Tx aggresively and early w/ broad spectrum ABX (Carbapenams/Vanc/Daptomycin) |
|
|
Term
| Pt with: cancer, fever, neutropenia. Next steps? |
|
Definition
BC (bacteria),
Fungi (venipuncture + any indwellings)
UC
CXR
CT/MRI (sinuses/abdomen)
Radionuclide scans |
|
|
Term
Leukemia:
Definition
Subtypes-->MC's (populations affected)
General Tx |
|
Definition
1. Proliferation of leukocytes
2. Acute Lymphocytic(Lymphoblastic) Leukemia-->MC in males; 2-5y/o
Acute Myelogenous Leukemia-->MC in neonates/late adolescence
Chronic Myelogenous Leukemia-->least
3. Chemotherapy-->prognosis good |
|
|
Term
Pt with: constitutional Sx of cancer, bone pain, anorexia, pallor, hepatosplenomegaly, petechiae/purpura, adenopathy
Tests to order-->expecting to see |
|
Definition
1. Leukemia
2. CBC-->WBCC extremely high-->lymphoblast (premature) cells on peripheral smear; anemia; thrombocytopenia
Bone marrow bx (ASAP)-->Diagnostic-->leukemic blast cells are taking over
LP-->r/o CNS involvement |
|
|
Term
| Cause of Pallor/hepatosplenomegaly in Leukemia? |
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Definition
| WBC over-production inhibits Myeloid cell line making RBC/platelets/etc. Hepatosplenomegaly-->destroying abnormals cells(increased with increased production of cells) and takes over RBC production causing hypertrophy |
|
|
Term
|
Definition
| EBV/CMV/JRA/ Transient Erythroblastopenia of childhood/ Transient myeloproliferative d/o |
|
|
Term
Definitive Dx of Leukemia?
Which ratio has a poorer prognosis |
|
Definition
1. Bone marrow bx
2. t(9:22)-->poorer prognosis |
|
|
Term
Tx of ALL is based on the phase:
Name the phase-->Tx |
|
Definition
1. Induction phase-inducing remission- (~1mo)-->chemo(prednisone/vincristine/asparaginase/danorubicin)-->CNS-->intrathecal
2. Consolidation(6-9mo)-->Systemic chemotherapy-->CNS-->Cranial radiation
3. Continuation(~2yrs)--> Daily/weekly/monthly pulse doses
4. Bone marrow transplant-->post chemo |
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|
Term
| Tx of AML: not helpful/recommended |
|
Definition
1. Chemo is not helpful
2. Induction Ts-->continuation Tx is not helpful-->Bone marrow transplants-->recommended-->sibling |
|
|
Term
Prognosis: Leukemia
What does it depend on?
Poor prognostic indicators?
AML v. ALL prognosis |
|
Definition
1. WBCC and age at onset-->outside age range is worse
2. <1y/o, >=10y/o; >50K; CNS involvement; Genetic factor; no Tx response >4wks
3. AML worse than ALL (ALL survive) |
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|
Term
| MC Lymphoma; age affected |
|
Definition
| Hodgkin's Lymphoma-->affects adolescents (and >50y/o) |
|
|
Term
| Non-Hodgkins Lymphoma--> distribution-->age affected-->subtypes |
|
Definition
1. Always diffuse-->highly malignant
2. Children >5y/o--> increasing w/ age
3. Burkitt's/small cell-->B cells
Lymphoblastic-->T cells
Large cell-->B or T cells |
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|
Term
Causitive agent in Lymphoma?
Associated with which subtype of non-hodgkin's lymphoma? |
|
Definition
|
|
Term
Pt with: Fatigue, anorexia, pruritis, "B symptoms"--> F/NS, wt loss, mediastinal LAD-->pleural effusion-->SOB, cervical/supraclavicular LAD
Dx-->Tests-->what to find
Tx-->what to do |
|
Definition
Lymphoma
2. CBC(normal); Tissue bx-->Reed-Sternberg cells= Hodgkin's Lymphoma
Mediastinal mass on CXR/CT
CT used for staging of the dz
3. Hodgkins--> Low dose chemo/field radiation-->5 yr survival(Stage I/ II)
Non-Hodgkin's-->More aggressive/persistent chemo--> NO SX--> Prognosis-->depends on age/stage |
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|
Term
| Reed-Sternberg cells (aka)= |
|
Definition
| Hodgkin's lymphoma (owl cells) |
|
|
Term
Ann Arbor Staging:
I
II
III
IV
A
B
E
S |
|
Definition
Need numeral and letter:
I= single LN region
II= One side of diaphragm
III= Both sides of diaphragm
IV= Disseminated
A= no constitutional Sx
"B-Symptoms"= F/NS/ wt loss
E= Extralymphatic Site
S= Splenic Dz |
|
|
Term
| bi-lobed nucleus-->B cells--> |
|
Definition
| Reed Sternberg Cells-->Owl Cells--> Hodgkin's Lymphoma |
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|
Term
CNS tumors: MC location for:
<2 y/o
>2y/o |
|
Definition
1. Infratentorial-->cerebellum/brainstem
2. Spinal/supratentorial--> Cerebrum |
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|
Term
| <2y/o Pt with: acute onset of vomiting, lethargy, irritability, ataxia |
|
Definition
| Acute CNS tumors-->rapidly growing-->BAD |
|
|
Term
| <2y/o Pt with: insidious onset of macrocephaly, hyperreflexia, CN palsies, wt loss |
|
Definition
| Chronic CNS tumors-->slow growing |
|
|
Term
| >2y/o Pt with: visual field deficits, generalized/partial siezures, focal weakness/sensory changes, personality changes |
|
Definition
|
|
Term
| >2y/o Pt with: Hydodephalus and increassed ICP-->papilledema, CN palsies, Ataxia |
|
Definition
|
|
Term
Types of Brain tumors:
1. MC, posterior fossa, low fever, good prognosis
2. 2nd MC, cerebellar vermis, high fever |
|
Definition
1. Astrocytomas
2. Meduloblastomas |
|
|
Term
| Pt with CNS tumor-->Next step? |
|
Definition
w/u:
(+) Neurologic PE
CT/MRI-->diagnostic
(NO LP/Xray/EEG)
Tx:
Pediatric oncology referral-->SX |
|
|
Term
| Complications of CNS tumor Tx and explanation: |
|
Definition
Cerebellar mutism: irritability/ataxia/mustism post op-->self limited--> (Astrocytoma)Posterior fossa tumor ressection
Posterior fossa syndrome-->HA-->aseptic meningitis-->1-2wk post op
Somnolence Syndrome-->self limited--> post irratiation-->lasts months |
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|
Term
MC solid childhood neoplasm outside the CNS?
MC age?
Derived from cells found where (MC)? |
|
Definition
1. Neuroblastoma
2. 0-3y/o; (~20m.o.)
3. adrenal medulla/sympathetic nervous system-->occur anywhere SNS-->MC abdomen/adrenal/paraspinal area |
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|
Term
Pt with: (Abd mass/pain; intra/extra-adrenal masses) ; fever; irratibility; bone pain; Horner's syndrome; wt loss; (HTN, diarrhea, dancing eyes-feet)
Name
Dx-->tests and expected findings
Tx |
|
Definition
(Abd mass-->intra-adrenal-->poorly defined/extends beyond midline/pain OR Extra-adrenal--> mediastinal/neck/pelvic/paraspinal mass)
1. Neuroblastoma
2. Labs-->CBC (anemia due to mets)
Urine catecholamines
Imaging-->CXR/KUB (stippled calcifications) / CT-->Inferolateral kidney displacement common
3. SX, chemo, radiation; better prognosis with young age and faster Tx; depends on stage/age/ cell type |
|
|
Term
2nd MC abdominal tumor
origination?
Associated with what other anomolies?
Age MC found in? |
|
Definition
1. Nephroblastoma
2. Kidney
3. WAGR= wilms, aniridia, GU malformation, retardation
4. 2-5 y/o |
|
|
Term
Pt with: rapidly enlarging abdominal mass, pain, fevers, painless hematuria, well defined abd mass that does not cross midline
Dx-->test-->expected findings
Tx-->preferred-->prognosis |
|
Definition
1. Wilms
2. Abd CT/ U/S--> mass margins
Chest CT-->r/o pulmonary metastasis
3. TOC-->SX-->chemotherapy(pre/post op)+ radiation for stage III/IV--> Surgical staging
Prognosis-->age/stage-->anaplastic variant with poor prognosis-->most survive |
|
|
Term
| MC presentation is abdominal mass discovered by parent? |
|
Definition
|
|
Term
Tumors from bone/soft tissues of the MSK system?
2 types-->MC's for each type?
Increased risk with concomittent... |
|
Definition
1. Sarcomas
2. Soft tissues-->Rhabdomyosarcome
Bone-->Osteosarcoma (MC); Ewing(2ndMC)
3. Li-FraumeniSarcoma
Neurofibromatosis
Retinoblastoma |
|
|
Term
Syndroms and associated increase of Sarcomas:
Li-Fraumeni-->
Neurofibromatosis-->
Retinoblastoma--> |
|
Definition
-->increased risk of both
-->soft tissue
-->Osteosarcoma |
|
|
Term
Pt with: Pain and mass at epiphyses site--> distal femur, proximal tibia, proximal humerus, recent trauma, Starburst pattern on radiograph
Dx
Tx |
|
Definition
Osteosarcoma
Light microscopy-->osteoid findings
3. Neonatal adjuvant-->SX (adjuvant not helpful) |
|
|
Term
Pt with: Sx consistent with osteomyelitis in the femur and pelvis; pain and fever, onion skin/moth eaten on radiograph
Dx-->how to differentiate Ewings from Rhabdomyosarcoma
Tx |
|
Definition
Ewing Sarcoma
2. light microscopy-->small, round, blue cells tumors; Immunohistochemical/ cytogenic analysis
3. Neoadjuvant chemo/radiation-->SX excision |
|
|
Term
Rhabdomyosarcoma
Dx-->differentiation of subtypes
Tx |
|
Definition
1. Light microscopy--> small, round, blue cell tumors
2. Embryonal-->young patients-->GU/head/neck tumor
Alveolar-->older pt--> extremity tumor
3. SX-->post op chemo and radiation-->depends on staging and site |
|
|
Term
|
Definition
Staging-->metastasis-->bad
Degree of tumor necrosis post neoadjuvant chemo |
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