FINAL Flashcards

(84 cards)

1
Q

growth trajectory

A

-@ 1mo 1.5 lbs heavier
-@ 6mo double birth wt
-@ 12mo triple birth wt

-ex: born at 6lbs:
-1mo- 7.5lbs
-6mo- 12lbs
-12mo- 18lbs

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2
Q

complementary feeding

A

-first 6mo -> breast feed exclusively -> breast feeding for 2yrs
-solid foods @6mo -> cereal, fruit, veggie, meat (iron & zinc)
-single ingredient foods every 3-4 days
-peanut protein puree @ 4-6mo
-whole cow milk and honey @ 1yr

-2yr+ -> 3 meals, high fiber, poultry, fish

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3
Q

vitamin A deficiency

A

-ocular signs -> xerophthalmia
-Night blindness, followed by xerosis (dry) of conjunctiva and cornea
-Immunodeficiency (measles)

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4
Q

niacin (B3) deficiency

A

-Pellagra (niacin deficiency): Weakness, lassitude, photosensitivity, inflammation of mucous membranes, V/D

-4 D’s:
-dermatitis
-diarrhea
-dysphagia
-dementia

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5
Q

vitamin C deficiency

A

-Scurvy:
-Irritability
-bone tenderness/swelling
-pseudoparalysis of legs

-Progression:
-Subperiosteal hemorrhage
-bleeding gums/petechiae
-hyperkeratosis of hair follicles
-mental changes
-anemia
-decreased iron absorption
-abnormal folate metabolism

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6
Q

vitamin D deficiency

A

-rickets- children
-osteomalacia- postpubertal adolescents

-RICKETS:
-Failure of mineralization -> soft zones of bone -> compression/lateral bulging or flaring of ends of bones
-Sx- MC < 2yo
-Craniotabes: Thinning of outer table of skull (when compressed -> feels like ping-pong ball)
-Enlargement of costochondral junction (rachitic rosary) and thickening of wrists and ankles
-Enlarged anterior fontanelle
-Scoliosis, exaggerated lordosis, bow-legs/knock knees, greenstick fractures

-DX: clinical
-Serum Ca low-normal, phosphorus reduced, alk phos increased
-Best measure -> 25(OH)D

-Imaging:
-Distal ulna/radius: Widening, concave cupping, frayed/poorly demarcated ends

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7
Q

GERD

A

-reflux + 2ndary sx
-common in young infants
-RF- small stomach, frequent large feedings, short esophagus, supine, slow swallow response to reflux
-MC sx- frequent, postprandial regurgitation
-usually resolves by 12-18mo
-FTT, food refusal, pain, GI bleed, upper/lower airway sx -> indicate GERD

-OLDER KIDS:
-Regurgitation into mouth, heartburn, and dysphagia
-2ndary complications (GERD): Esophagitis
-RF: Asthma, CF, developmental delay/spasticity, hiatal hernia (HH), repaired esophageal atresia-tracheoesophageal fistulas

-Dx:
-differentiate btwn benign, recurrent vomiting (GER) from red flags for GERD
-Red flags -> Bilious emesis, GI bleed, vomiting > 6mo, FTT, diarrhea, fever, hepatosplenomegaly, abdominal tenderness/distension, or neuro changes
-Upper GI series if recurrent vomit suspicious of anatomic etiologies
-Older kids: Trial of acid-suppressant therapy may be dx and tx -> Referral to specialist if no improvement
-Esophagoscopy and mucosal bx for eval of mucosal injury 2ndary to GERD (Barrett esophagus, stricture, erosive esophagitis) or other ds like EoE
-Intraluminal esophageal pH monitoring (probe) and combined multiple impedance and pH monitoring (impedance probe) to quantify reflux

-Tx:
-self limited in 85% by 12mo (erect posture and solid feedings)
-small feedings at frequent intervals
-thickening feedings with rice cereal
-Older infants/kids: Acid suppression for suspected esophageal/extraesophageal complications of reflux
-Histamine-receptor antagonists or PPI (x 8-12 weeks)
-Older kids: Intermittent use of acid blockers versus chronic acid suppression

-Antireflux surgery (Nissen fundoplication) for pts who:
-Fail medical therapy
-Depend on persistent, aggressive medical therapy
-Have sx and are nonadherent to medical therapy
-Have persistent, severe respiratory/life-threatening complications of GERD

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8
Q

pyloric stenosis

A

-hypertrophy
-gastric outlet obstruction
-projectile postprandial vomit (not bilious but may be blood streaked)
-2-4wks yrs old
-babys will be hungry
-distended abdomen after eating
-peristaltic waves from L to R
-oval mass- 5-15mm in RUQ

-Dx- hypochloremic alkalosis with low K
-dehydration- high Hmg/Hct

-Imaging:
-US- hypoechoic muscle ring >4mm and pyloric channel length >15mm
-Barium upper GI- retention of contrast in stomach and long narrow pyloric channel with double track of braium

-Tx:
-pyloromyotomy
-tx dehydration before

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9
Q

duodenal obstruction/atresia

A

-obstruction is intrinsic (atresia, stenosis, mucosal webs) or extrinsic (malrotation, annular pancreas, duodenal duplication)

-Imaging:
-Double bubble- distention of stomach and proximal duodenum
-Atresia -> absence of distal intestinal gas

-Duodenal atresia:
-maternal polyhydramnios
-bilious emesis and epigastric distention first few hrs of birth
-assoc with preterm and down

-Tx:
-duodenoduodenostomy to bypass

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10
Q

intestinal malrotation

A

-occludes SMA
-volvulus
-1st 3 wks of life- bilious emesis or SBO
-Later signs- intermittent obstruction, malabsorption, protein losing enteropathy, or diarrhea
-older kids- chronic GI sx of N/V/D, abd pain, dyspepsia, bloating, early satiety

-Imaging:
-upper GI series- Gold standard- corkscrew sign
-barium enema- mobile cecum
-US/CT- whirlpool sign- midgut vulvulus

-Tx:
-Ladd procedure
-Midgut volvulus -> surgical emergency

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11
Q

intussusception

A

usually proximal to ileocecal valve
-MCC Of obstruction in first 2yrs of life (3x in males)
-causes: polyp, meckel diverticulum, omphalomesenteric remnant, duplication, lymphoma (MC >6yo), lipoma, parasites, FB, viral enteritis w/ hypertrophy of peyer patches (MC)

-paroxysms of abd pain with screaming and drawing up of knees
-V/D
-blood stool (current jelly)
-febrile
-sausage shaped mass palpated

-Dx:
-US- target sign
-Barium and air enema = dx and tx
-if ischemia or perf -> surgery

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12
Q

acute appendcitis

A

-15-30yrs MC

-WBCs seldom >15
-pyuria, fecal leukocytes, guaiac +
-high CRP and leukocytosis
-radio-opaque fecalith
-US- thickened appendix
-CT- with rectal contrast

-Tx:
-laparotomy or laparoscopy

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13
Q

congenital aganglionic megacolon/hirschsprung ds

A

-MC chromosomal abnormality assoc is downs
-colon cant relax in certain areas
-contracted parts are narrow -> proximal parts are dilated/thin

-newborn wont pass meconium within 24hrs -> vomiting, distention
-enterocolitis, fever, dehydration, explosive diarrhea
-ischemia, perf, sepsis
-Later infant- alternating obstipation and diarrhea
-Older kid- constipation
-foul smell, ribbon like, distended abd, hypoproteinemia, FTT

-no stool in anal canal/rectum even though obvious retained stool on imaging

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14
Q

meckel diverticulum

A

-bleeding due to ileal ulcers adjacent to diverticulum
-cased by acid secreted by heterotopic gastric tissue
-can cause obstruction / intussusception

-imaging:
-Meckel scan
-Technetium-99m-pertechnetate take up by heterotopic gastric mucosa in the diverticulum and outlines diverticulum on a nuclear scan

-Tx: Surgical with good prognosis

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15
Q

umbilical hernia

A

-MC hernia
-MC- full-term, African American infants
-Most regress spontaneously if fascial defect has diameter < 1cm
-Asymptomatic -> no intervention until 4-5yrs -> surgery

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16
Q

inguinal hernia

A

-any age
-MC- indirect, M>F

-Painless inguinal swelling
-Partial obstruction -> severe pain
-Rarely, bowel becomes trapped in hernia -> complete intestinal obstruction occurs -> gangrene or testis may occur
-Girls -> may prolapse into hernia sac -> presents as mass below inguinal ligament
-Hx of inguinal fullness assoc with coughing or long periods of standing, or presence of firm, globular, and tender swelling -> assoc with vomiting and abdominal distention

-Tx:
-Incarceration of inguinal hernia: Manual reduction
-CI if present > 12hrs or if bloody stool noted
-Surgery if hernia has incarcerated in past

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17
Q

functional constipation/encopresis

A

-Infrequent BMs, hard stools, large-diameter stool, and soiling
-MC @ introduction of solids (>6mo), toilet training (2-3yrs), start of school (3-5yrs)

-occurrence after infancy
-presence of stool-withholding behavior
-absence of red flags
-episodic passage of large-diameter stools
-Red flags: Poor growth, wt loss, FTT, emesis, abdominal distention and bloating, perianal ds, blood in stool, abnormal urinary stream, hx of delayed meconium
-!Encopresis: Intentional or involuntary feces into clothing > 4yo
-Leakage of stool due to underlying constipation or fecal impaction

-Dx:
-Rome Criteria/Bristol stool charts
-stool impaction on exam
-eval anal placement and neuro exam (spinal cord abn)

-Tx:
-1. educate not to force potty training
-well balanced diet with veggies/fruits and age-appropriate fiber (increasing wont help)
-sorbitol juices- prune, pear, apple
-2a. behavioral training- timed toilet sitting session at scheduled frequencies, praise/+ reinforcement
-2b. stool softening therapy +/- laxative (sorbitol, miralax!!!!/polyethylene glycol)
-3. successful cleanout/disimpaction -> maintenance phase: promotes regular stool production and prevent re-impaction
-enema

-2-3days no BM -> no distention, dietary mod (miralax maybe)
-5-7 days no BM w/ mass & distention -> suppository, enema, disimpaction

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18
Q

dehydration

A

-higher SA to volume ratio

-vitals (orthostatic BP too)
-urine- high SG, ketonuria, BMP, serum bicarb (metabolic acidosis), BUN

-Classified by % of total body water lost (mild, moderate, severe)
-Tx:
-Mild-moderate- oral rehydration therpay (ORT) (pedialyte/gatorage), BRAT diet (diarrhea)
-1 mL/kg q 5-10 mins or 0.5 ounces q 5-10 mins
-Ondansetron if needed to tolerate ORT (for vomiting)

-Severe: IV fluids
-Initial bolus of 20 mL/kg normal saline over 20-30 mins

-Ongoing tx: fluid deficit (FD) = % dehydration x weight (kg)
-½ fluid deficit over first 8 hours, second ½ over next 16 hours

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19
Q

inborn errors of metabolism: galactosemia

A

-Autosomal recessive
-Caused by near total deficiency of galactose-1-phosphate uridyltransferase > accumulation of galactose-1-phosphate > hepatic parenchymal ds and renal Fanconi syndrome

-Neonates: Vomiting, jaundice (direct and indirect), hepatomegaly, liver insufficiency (progressive cirrhosis) after initiation of milk feeding
-Increased risk of language deficits, intellectual disability, tremor, ataxia, and ovarian failure
-Cataracts if untreated
-Death within 1mo w/o tx (often from E. coli sepsis)

-Dx:
-Infants receiving foods containing galactose: Liver dysfunction (PT prolongation), proteinuria, and aminoaciduria
-Elevated galactose-1-phosphate in RBCs
-When suspected, galactose-1-phosphate uridyltransferase should be assayed in RBCs or GALT sequencing pursued
-Newborn screening demonstrating enzyme deficiency in RBCs or increased serum galactose for institution of early tx

-Tx:
-Galactose-free diet as soon as possible (lifelong)
-Calcium and vitamin D
-DEXA scans
-Monitoring of speech and language development
-Screening for hypergonadotropic hypogonadism during adolescence

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20
Q

inborn erros of metabolism: phenylketonuria

A

-Autosomal recessive
-Disorder of amino acid metabolism caused by decreased activity of phenylalanine hydroxylase (converts phenylalanine to tyrosine)
-Normal diet: Elevated phenylalanine levels -> severe intellectual disability!, hyperactivity, seizures, light complexion, eczema

-Dx:
-elevated plasma phenylalanine
-elevated phenylalanine/tyrosine ratio in child with normal diet
-neurotoxic

-Tx: Restriction of phenylalanine as early as possible

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21
Q

inborn errors of metabolism: maple sugar urine ds

A

-Autosomal recessive
-Deficiency of enzyme complex that catalyzes oxidative decarboxylation of branched-chain ketoacid derivatives of leucine, isoleucine, and valine -> accumulated ketoacids -> cause sweet odor, leucine and corresponding ketoacid cause CNS dysfunction

-Birth- Normal
-2-3 days old -> irritability/feeding issues
-1 week -> seizures and coma (most die within 1st mo of life w/o tx)

-Dx:
-Marked elevation of branched-chain amino acids
-genetic testing for confirmation

-Tx:
-Leucine! restriction and avoidance of catabolism
-Infant formulas deficient in branched-chain amino acids must be supplemented with normal foods for growth
-Very high leucine levels require hemodialysis
-Liver transplant corrects the disorder

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22
Q

normal short stature: constitutional growth delay

A

-late bloomers
-normal birth wt and ht
-lower growth based on parents, delayed skeletal maturation compared to age, late puberty
-growth continues beyond average child stops
-final ht is normal
-growth spirt at 17-18yo

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23
Q

growth hormone deficiency

A

-Decreased growth velocity and delayed skeletal maturation in absence of other explanations
-May be congenital, genetic, or acquired
-Idiopathic is MC

-Infantile GHD: Normal birthweight and slightly reduced length, hypoglycemia (with adrenal insufficiency), micropenis (with gonadotropin deficiency), and conjugated hyperbilirubinemia

-Dx- clinical and lab evidence
-Labs: Serum IGF-1 gives reasonable estimations of GH secretion and action
-MRI of hypothalamus/pituitary gland to evaluate for tumor

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24
Q

disproportionate short stature: achondroplasia

A

-dwarfism
-Autosomal dominant transmission
-Upper arms and thighs are proportionately shorter than forearms/legs
-Skeletal dysplasia
-Height measurements for screening

-Bowing of extremities, waddling gait, limited ROM, relaxation of ligaments, short stubby fingers, frontal bossing, midface hypoplasia, otolaryngeal dysfunction, moderate hydrocephalus, depressed nasal bridge, lumbar lordosis

-Imaging:
-Short, thick, tubular bones and irregular epiphyseal plates
-Ends of bones are thick, with broadening and cupping
-Delayed epiphyseal ossification
-Narrowed spinal canal (diminished growth of pedicles)

-Tx: Growth hormone

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25
DMT1
-Polyuria, polydipsia, and wt loss -Heavy diaper in a dehydrated child w/o diarrhea -> alarm -Labs: -HbA1c does not r/o dx (less sensitive than blood glucose) -Tx: -Aim for lowest HbA1c w/o severe hypoglycemia or frequent, moderate hypoglycemia -Diet/exercise: At least 60 mins of daily aerobic exercise with bone/muscle strength training at least 3 days/week -Monitor glucose at least 4x/day -> 7-10 for optimal management -CGM- Subq glucose measured q 1-5 mins -Subq may lag behind blood glucose if rapid change, so finger sticks still recommended for tx and monitoring of recovery from hypoglycemia -New onset- long acting insulin for basal level // rapid acting analog for mealtime dosing -peak dose @ 1 wk and then decrease it slightly -Pre-pubertal: Higher rate early in night -Post-pubertal: Higher rates early in morning – “dawn phenomenon”
26
DKA
-Venous blood pH < 7.30, OR -bicarbonate < 15 and blood B-hydroxybutyrate > 3 mmol/L, OR -moderate to large ketonuria -Mild: 7.2-7.29/10-14 -Moderate: 7.10-7.19/5-9 -Severe: < 7.10/<5 -> ICU monitoring -Abd pain, N/V (flu-like, gastroenteritis, acute abdomen); mild-moderate dehydration (5-10%) -Kussmaul respirations -progressively somnolent and obtunded -Tx: -Restoration of fluid volume -Inhibition of lipolysis and return to glucose utilization (insulin) -Replacement of electrolytes (Na and K) -Correction of acidosis (spontaneous correction)
27
DMT1: HHS
-hyperglycemia > 600 and hyperosmolarity > 320 -no ketosis and no acidosis -profound AMS, seizure, rhabdo -similar tx to DKA with close ICU monitoring -longer term complications: HTN, lipid abn, nephropathy, retinopathy, neuropathy
28
hypothyroidism
-mostly sporadic from hypoplasia or aplasia of thryoid gland/failure to migrate to normal anatomic position -acquired = hashimoto thyroiditis -> thyroid enlargement -congenital: normal @ birth, +/- jaundice -acquired: poor linear growth, delayed bone age/dental eruption, skin changes (dry, coarse, brittle), hair loss, lateral thinning of brows, neuro (hypotonia, slow relaxation of DTR), sluggish, nonpitting myxedema, constipation, cold temp, brady, delayed puberty -Dx: -Primary: TSH elevated, total T4/FT4 normal/decreased -> Autoantibodies to thyroid peroxidase and/or thyroglobulin possible -Central: TSH normal, total T4/FT4 decreased -Imaging: Thyroid imaging unnecessary, bone age delayed, cardiomegaly common -Screening: All newborns @ birth, tx started asap (otherwise -> intellectual impairment!!) -Tx: -Synthetic T4/levothyroxine (75-100 mcg/m^2/day) with monitoring of TFTs for response
29
hyperthyroidism
-MCC - Graves disease (antibodies directed at TSH receptor -> stimulates TH production) -Other causes: Thyroiditis, thyroid nodules, TSH-producing tumors, McCune-Albright syndrome, exogenous TH excess, acute iodine exposure -F > M, occurs during adolescence, course may be cyclic -Poor concentration, hyperactivity, fatigue, emotional lability, personality disturbance, insomnia, wt loss, palpitations, heat intolerance, increased perspiration, increased stool frequency, polyuria, and irregular menses -Tachycardia, HTN, increased pulse pressure, tremor, proximal muscle weakness, moist/warm skin, accelerated growth/development, diffuse/firm goiter, thyroid bruit/thrill, exophthalmos -Thyroid storm: Fever, cardiac failure, emesis, and delirium -> coma/death -Dx: -TSH suppressed; T4, FT4, T3, and FT3 are elevated -TSH receptor-binding antibodies- elevated -Presence of TSI or thyroid eye ds confirms Graves -Radioactive iodine uptake increased in Graves -> decreased in subacute/chronic thyroiditis; autonomous hyperfunctioning nodules take up iodine (hot nodules) -Advanced bone age, !!premature fusion of cranial sutures!!, osteoporosis (long-standing) -bad for brain growth!!! -Tx: -Avoid strenuous activity bc cardiovascular instability -BB agents: Atenolol (B1-specific/cardioselective), propranolol (decreases conversion of T4 to active T3; used in severe cases/thyrotoxicosis) -Antithyroid agents (methimazole) -used in initial management of childhood hyperthyroidism -Interfere with TH synthesis -Take several weeks for clinical response -If medical therapy unsuccessful -> thyroidectomy or radioiodine ablation considered
30
precocious puberty in girls
-onset of 2ndary sexual characteristics before 8yo in Caucasian girls -7yo for African American and Hispanic girls -Central PP: Idiopathic or 2ndary to a CNS abnormality that disrupts prepubertal restraint on the GnRH pulse generator -Abnormalities include hypothalamic hamartomas, CNS tumors, cranial irradiation, hydrocephalus, and trauma -Peripheral PP (GnRH-independent): Ovarian/adrenal tumors, ovarian cysts, late-onset congenital adrenal hyperplasia, McCune-Albright syndrome, or exposure to exogenous estrogen -starts with breast development -> pubic hair -> menarche -PPP: -!Ovarian cysts/tumors usually with signs of estrogen excess: Breast development, vaginal discharge, vaginal bleeding -!Adrenal tumors and CAH with signs of androgen excess: Pubic hair, axillary hair, acne, and increased body odor -Accelerated growth/maturation; skeletal maturation quicker than linear growth = compromised adult stature
31
precious puberty in girls dx and tx
-Labs: -CPP: Random FSH and LH may confirm dx -PPP: LH response to GnRH is suppressed by autonomously secreted gonadal steroids -Estradiol levels, androgen levels (testosterone, androstenedione, dehydroepiandosterone sulfate), and 17-hydroxyprogesterone should be measured -Imaging: -Bone age (L hand and wrist) -CPP: MRI of brain for CNS lesions -PPP: Imaging of ovaries and/or adrenal gland -Tx: -CPP: GnRH analogues that downregulate pituitary GnRH receptors -Leuprolide (IM injections), histrelin subdermal implant (replaced annually) -After stopping therapy, pubertal progression resumes, and ovulation and pregnancy have been documented -PPP: Dependent on underlying cause -Regardless of cause, attention to the psychological needs of the patient and family is essential
32
delayed puberty in girls
-Primary hypogonadism: Primary abnormality of ovaries -!!!MCC is Turner syndrome -Central hypogonadism: Hypothalamic or pituitary deficiency of GnRH or FSH/LH -Functional (reversible): Stress, undernutrition, prolactinemia, excessive exercise, or chronic illness -Permanent: Congenital hypopituitarism, CNS tumors, or cranial irradiation -H&P, BONE AGE: -Low bone age (< 12 years): -abn growth rate! -Bone age > 12 years: -FSH/LH distinguishes between primary ovarian failure (elevated FSH/LH) and central hypogonadism (low FSH/LH) -Cranial MRI for central -GIRLS W/ ADEQUATE BREAST DEVELOPMENT AND AMENORRHEA: -Progesterone challenge to determine if sufficient estrogen is being produced and to evaluate for anatomical defects -Producing estrogen: Withdrawal bleeding 5-10 days of PO progesterone -> !!MCC of amenorrhea in this case is PCOS -Estrogen-deficient/anatomical defect: No bleeding -Tx: -Replacement therapy in hypogonadal: Estrogen alone @ lowest available dosage -increased slowly then 18-24 months later -> add progesterone -Unopposed estrogen = endometrial hyperplasia
33
precocious puberty in boys
-2ndary sexual characteristics before 9yo -Pubic hair -> penile enlargement -> scrotal maturation, axillary hair, voice deepening, increased growth velocity -CPP: Testes enlarge -PPP: Testes remain much smaller than expected for degree of virilization -Labs: -Elevated testosterone levels -CPP: High LH/FSH -PPP: Low LH/FSH -> CAH: Adrenal androgens and 17-hydroxyprogesterone will be elevated -Imaging: -Bone age -CPP: Brain MRI -PPP: Rule out hepatic, adrenal, and testicular tumors -Tx: -CPP: GnRH analogues/tx of underlying cause -PPP: Steroid synthesis blockers (ketoconazole) or combination of antiandrogens (spironolactone) and aromatase inhibitors (anastrozole or letrozole) that block conversion of testosterone to estrogen
34
delayed puberty in boys
-No 2ndary sexual characteristics by 14yo or if >5 years since first signs of puberty w/o completion of genital growth -!!!!MCC is constitutional growth delay -Hypogonadism may be primary or central -Primary: Testicular insufficiency/anorchia, Klinefelter syndrome/sex chromosome anomalies, enzymatic defects in testosterone synthesis, inflammation/destruction of tests following infection, autoimmune disorders, radiation, trauma -Central: Deficiencies in pituitary/hypothalamic function (same as girls) -H&P, bone age: -Low bone age relative to chronological age + normal growth velocity (prepubertal) -> constitutional growth delay -Bone age > 12 years -Elevated LH/FSH: Primary hypogonadism or testicular failure -Low LH/FSH: Central hypogonadism -Tx: -4-6-month course of low-dose depot testosterone to promote virilization and possibly “jump-start” endogenous development
35
UTI
-<1yo -> VUR -> self limited -Newborns/infants: Fever, hypothermia, jaundice, poor feeding, irritability, vomiting, FTT, sepsis; strong, foul-smelling urine -Preschool children: Abdominal/flank pain, vomiting, fever, urinary frequency, dysuria, urgency, enuresis -School-aged children: -Signs of cystitis: Frequency, dysuria, urgency -Signs of pyelonephritis: Fever, vomiting, flank pain -CVAT is unusual -Physical: BP, abdominal, GU exam -DX: -!!!Most have negative nitrites (70%) -Urine cx is gold standard (properly collected) -Toilet-trained: Midstream, clean-catch specimen -Infants/younger children: Bladder catheterization or suprapubic collection -Bagged specimens only for screening (if negative) -!Positive results: -SPT: Any growth -Catheterization: > 50K cfu/mL -Clean-catch: > 100K cfu/mL -Renal US in all infants after first febrile UTI -f/u with VCUG if abn -Tx: -< 3mo + dehydration, toxicity, or sepsis -> hospital for parenteral antibiotics -Older infants/children: Outpt -Uncomplicated UTIs: First gen cephalosporin (cephalexin first-line), amoxicillin, trimethoprim-sulfamethoxazole x 7-10 days -Sexually mature teenagers: Fluoroquinolones x 3 days -Complicated UTIs: Parenteral, third-generation cephalosporin or aminoglycoside -adjusted after C&S results -Prophylactic antibiotics: -children with frequently occurring UTIs may benefit -high-grade VUR (V), dysfunctional voiding -Trimethoprim-sulfamethoxazole and nitrofurantoin approved for prophylaxis
36
glomerulonephritis
-hematuria- micro or gross -HTN- glomerular inflammation & renin -edema- periorbital, ascites, extremities -protein excretion ranges from normal (Pr/Cr <.2) to nephrotic (Pr/Cr >2) -Dx: -BP -renal function- ablumin, urine protein -C3 levels- differentiate cause- low in acute post-infectious glomerulonephritis -ACUTE POST-INFECTIOUS GLOMERULONEPHRITIS: -infection within 7-14 days (MC GABHS) -dx- + culture or elevated ASO titer -Tx: -antibiotic if underlying active infection -limit salt, diuretics, CCB -hemodialysis or peritoneal dialysis -recovery in 6-8wks
37
hemolytic uremic syndrome (HUS)
-MC glomerular cause of acute renal failure in childhood -Diarrhea-assoc form -> Shiga toxin-producing strains of shigella or E. coli – MC pathogen is E. coli O157:H7 -Ingestion of undercooked ground beef/unpasteurized food -presenting complaint- Bloody diarrhea -followed by hemolysis, thrombocytopenia, and renal failure -Toxin -> endothelial damage -> platelet consumption -> microvascular occlusion -> hemolysis -Prodrome- abdominal pain, diarrhea, vomiting -> then, oliguria, pallor, and bleeding (GI) -Anemia -Shistocytes on blood smears -High reticulocyte count, increased LDH, low haptoglobin (consistent with hemolysis) -Severe thrombocytopenia -Hematuria and proteinuria often present -Complications: -AKI -Seizures, PRES from hyponatremia, HTN, or CNS vascular disease -Thrombosis from endothelial damage (despite thrombocytopenia) -Tx: -Attention to fluid and electrolyte status is critical -No antimotility or antibiotic agents! -May worsen release of Shiga toxin -Timely dialysis improves prognosis -RBC transfusions often necessary (EPO may reduce this need) -Prognosis: -Most recover within 2-3 wks -Residual renal ds (HTN, proteinuria, CRI) in 30% -End-stage renal failure in 15% -Overall mortality (with CNS or cardiac complications) 3-5%
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idiopathic nephrotic syndrome of childhood
-MCC- minimal change ds (MCD) -MCD: Minimal change on light microscopy -> effacement of podocytes on electron microscopy -Other causes: Focal segmental glomerulosclerosis (FSGS) and membranous nephropathy -<10yo -periorbital swelling and oliguria -> within days -> increasing edema (possibly anasarca) -> dyspnea/massive ascites -Urine sediment normal -Gross hematuria more common with FSGS than MCD -Plasma albumin low -> lipid levels increased (increased hepatic lipogenesis) -Complications: Infections, hypercoagulability, HTN, renal insufficiency -Tx: -Corticosteroids, QD x 6wks (tapered) -May add calcineurin inhibitor (tacrolimus) or mycophenolate mofetil to achieve steroid discontinuance while maintaining remission -Goal is disappearance of proteinuria -No response -> renal bx for further evaluation
39
ambiguous genitalia
-genitalia formed 6-13wks gestation -SRY (on Y chromosome) -> differentiates into testis -> testosterone -> DHT (via 5a-reductase) -> scrotum & penis -Mullerian-inhibitory substance -> Regression of Mullerian ducts + fallopian tubes/uterus -In presence of testosterone -> Wolffian ducts develop into -> vas deferens, seminiferous tubules, and prostate -Female phenotype develops unless specific male influences alter develop -Dx: -1. Determine if androgen excess of genetic female or androgen deficiency of genetic male -Virilized females: -MC- Congenital adrenal hyperplasia; 90% with 21-hydroxylase deficiency -low 21 (low cortisol and aldosterone) causes higher 17 (androgens) -Dx- plasma concentration of 17-hydroxyprogesterone and androstenedione (typically 100 x normal) -Underdeveloped males: -Adrenal hyperplasia- defects in androgen production of testes, excessive ACTH secretion -> elevated adrenal steroid precursors (with defective enzymes to convert them) -Dx- Measure testosterone and precursors in basal state + after stimulation by HCG (normally inc test) -if testosterone is normal: Androgen resistance or interruption of normal morphogenesis of genitalia -if increase with HCG -> CAH, enzyme issue -if test remains the same -> defect in testosterone production -Tx: -Hormone replacement: Cortisol (adrenal hyperplasia), testosterone -Surgical restoration -Psychologic support of family -Approach- wait until child can be part of dialogue -tx is individualized
40
46, XX disorder of sexual development
-Male external genitalia of genotypic females -caused by excessive androgens -Mild clitoral enlargement to development of a male phallus with penile urethra and fused scrotum with raphe -MCC- congenital virilizing adrenal hyperplasia (ACTH hypersecretion -> hyperplasia of adrenal cortex -> excessive adrenal production of androgens)
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46, XY disorder of sexual development
-Underdevelopment of male external genitalia -deficiency of testosterone production/resistance -Small penis -varying hypospadias -B/L cryptorchidism may be present -Androgen Resistance/Androgen Insensitivity Syndrome (MC): -46, XY karyotype -Normal testes (in inguinal canal/labia majora) -Female external genitalia -no internal Mullerian structures -At puberty: Breast development w/o pubic, facial, or axillary hair or menstruation (estrogen unopposed by androgen) -5a-reductase deficiency: -DHT problem -> external male genitalia -@ birth- female phenotype or ambiguous genitalia -At puberty: 2ndary male sexual development occurs -> testosterone takes into affect
42
phimosis/paraphimosis
-90% of uncircumcised -> foreskin retractable by adolescence -Prior to adolescence -> prepuce may be tight and doesnt require tx -After adolescence: -Inability to retract -> phimosis -May be congenital or inflammation -Rarely sx -Tx: -Reassurance (loosening of prepuce by puberty) -Topical steroid if needed -If narrowing is severe -> gentle stretching -Circumcision -> most severe cases -Paraphimosis: Prepuce retracted behind coronal sulcus -> cant resume normal position -> swelling of glans/pain (venous stasis) -Tx: -When discovered early: Retraction of foreskin with lubrication -In some cases -> circumcision may be needed
43
hydrocele
-collection of serous fluid between the 2 layers of tunica vaginalis (congenital or acquired) -congenital: Connection of peritoneal cavity through patent processes vaginalis -MCC in children -Excessive production of fluid (2ndary) -Defective absorption of fluid -Interference with lymphatic drainage of scrotal structures -Painless -transillumination -fluctuance -Congenital- intermittent, disappear when supine (drainage of fluid back to peritoneum) -Imaging: -US: Anechoic or echolucent area surrounding testes -Duplex US: r/o torsion versus epididymitis -AXR: r/o inguinal hernia -Tx: -spontaneously resolve within 1st yr (congenital) -Surgery- tx of choice (hydrocelectomy) -Aspiration (fluid usually always accumulates again in short term) -NOTE: if it were varicocele -> irregular, worse with standing -> testicular atrophy is the MC commplication
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ovarian torsion
-Twisting of ovary over supportive ligaments of adnexa -> venous congestion, edema, compression of arteries -> loss of blood flow to ovaries -> necrosis, loss of ovary, infertility -RF- ovarian mass > 5 cm -Lower abd/pelvic pain, +/- N/V -Infants- feeding intolerance/irritability -PE: +/- abd TTP, possible palpable mass -> Guarding, rigidity, rebound -> possible necrosis -Imaging: Doppler US (transvaginal, pelvic) -Ovarian edema, abnormal ovarian blood flow, relative enlargement of ovary -Def dx during surgery with direct visualization -Tx: Surgical detorsion -90% are functional after
45
balanoposthitis
-Candida, Gardnerella, or Streptococcus pyogenes) -sole presenting sx of DM -PE- retract foreskin -> red, warm, discharge -Dx- glucose test, cultures -Tx: -cleanse with soap/water -topical antifungal cream (nystatin, clotrimazole)
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cryptorchidism
-MC- premature -infertility, malignancy -after 6mo -> descent rare -Dx: -feel if in scrotum or inguinal canal -imaging- not usually indicated -1-4mo -> LH, FSH, testosterone; insulin B and/or HCG stimulation to confirm presence or absence of functional testes -Tx: -surgical orchidopexy if no descent by 6-12mo -HCG 2x weekly x 5wks for inductino of descent -> low success rate -NOTE- labial adhesions onset @ 2yo and resolve within 1yr -> if not, estrogen cream or surgery -sx- post-void dripping, hematuria, dysuria, UTI -educate recurrence
47
-Seizures (general, focal, abscence, febrile)
-FOCAL: -awareness OR unaware -sensory, autonomic, emotional, and/or motor -1. simple partial- aura, jacksonian march (unilateral but travels) -> AWARE w/ motor sx -2. complex partial (focal w/ impaired awareness)- automatisms (blinking, lip smack, swallowing), zoning out! -GENERALIZED: -tonic-clonic- LOC -absence- nonmotor, behavioral arrest, NO POSTICTAL -myoclonic- twitching/jerking, NO POSTICTAL -atonic- loss of tone, NO POSTICTAL -myotonic atonic- jerk followed by loss of tone -FEBRILE: -6mo-6yrs, >38.8C, non-CNS infection -MC- generalized tonic clonic, <5mins, early in illness -dx- find source -> consider meningitis/encephalitis (CNS) -imaging/labs/EEG- not helpful -recurrence- 30-50%, as well as other seizures -control of fever doesnt help recurrence -ASM not recommended
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seizure dx and tx
-<3yo with new onset of unprovoked seizures -> EEG and MRI -Routine EEG useful for interictal activity and/or recording provoked seizure -EEG showing epileptiform activity may confirm and clarify dx -> not always dx -Other dx used selectively: -Routine labs -> unnecessary in well child w/ seizures -workup for suspicion of acute systemic etiology (renal failure, sepsis, substance abuse) -Emergent imaging -> unnecessary if no trauma or acute abnormalities on exam -Tx: -turn to side, do NOT restrain ->5mins or >6hr clusters -> acute home tx with benzos (rectal diazepam gel) or intranasal midazolam -meds until no seizures for 1-2yrs -> taper for 6-8wks -restart for another 1-2yrs if relapse -Poor prognosis: Identified etiology, older onset, continued epileptiform EEG, difficult seizure control, polytherapy, generalized tonic-clonic or myoclonic seizures, abnormal neuro exam -Education: -supervision with swimming -no CI to sports -avoid sleep deprivation/alcohol -seizure free for 6-12mo -> drive -schools must work with parents -> plan
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sleep disorders
-assoc w/ maladaptive daytime behaviors, poorer development, parental stress, obesity, insulin resistance, alterations in sympathetic tone, and immune dysfunction -newborns- 10-19hrs/day in 2-5hr blocks -1st year- 9-12hr blocks -3-5yr- no naps -BEARS screening tool: -Bedtime resistance -Excessive daytime sleepiness -Awakening during the night -Regularity and duration of sleep -Sleep-disordered breathing -Medical psychosocial hx, PE -Assess allergies, lateral neck films, polysomnography -GER, dental pain, eczema, meds -Tx: ABCs of SLEEPING -Age-appropriate -Bedtimes and wake times w/ -Consistency -Schedules and routines -Location -Exercise and diet -no Electronic in bedroom or before bed -Positivity -Independence when falling asleep -Needs of child met throughout the day, equals -Great sleep -Little evidence to support use of melatonin/clonidine for sleep disorders
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parasomnias
-NIGHT TERRORS: -3-8yo -w/in 2 hrs -> deepest stage of NREM -sit up screaming, thrashing, tachypnea, tachycardia, sweat -cant comfort -30 mins episodes -tx- reassurance -> avoid stress, irregular sleep schedule, sleep deprivation -SOMNAMBULISM: -sleep walking -4-8yo -create safe environment (lock doors, bells on door) -avoid stress, sleep deprivation -NIGHTMARES: -3-5yo -during REM (later in night) -will have memory of it -responds to comfort -wakes them -tx: self-limited, avoid stress, anxiety, sleep deprivation
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insomnia
-cant fall asleep and nighttime awakenings -daytime fatigue for parents and child -parental discord about tx, and family disruption -Quantity and timing of feeds in 1st yr ->6mo can go throughout night w/o feedings (waking for feeds is probably a learned behavior) -Bedtime habits -Child’s temperament -Low sensory thresholds and less rhythmicity (regulatory disorder) -Separation anxiety (9mo) -Psychosocial stressors -Complex medical conditions: Neuro, developmental, psychiatric disorders
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sleep disordered breathing/obstructive sleep apnea
-loud snoring -chest retractions -morning HA -dry mouth -daytime sleepiness -2-6yo peaks -assoc w/ ADD/ADHD
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restless leg syndrome (RLS) and periodic limb movement disorder (PLMD)
-creepy crawly or itchy bones -PLMD: -repetitive limb movements assoc with partial arousal or awakening -both assoc with iron deficiency and drugs (caffeine, nicotine, antidepressants) -no tx
54
narcolepsy
-sleep in daytime that occurs regardless of activity or surroundings -not relieved by increased sleep -1/2 have 1st sx in childhood -> around/after puberty -dx- short latency between sleep onset and transition into REM Tx: -sleep hygiene/behavior modification -CNS stimulations/antidepressants (off-label)
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CNS infections
-fever, malaise, chills, organ dysfunction -CNS sx- HA, stiff neck, fever/hypothermia, AMS, seizures, focal sensory/motor deficit -kernig and brudzinski -young infants: -signs of meningeal irritation -> can be absent -temp instability/hypothermia MC than fever -buldging fontanelles -> inc head circumference -older kids- papilledema -Dx: -CBC, chem panel, blood cx, CSF!!! -start antibiotics even if LP is delayed -CSF- RBCs, WBCs, protein, glucose, bacteria, microorganisms, culture -> serologic, immunologic, PCR -CT/MRI w/ and w/o contrast -> brain abscess, meningeal inflammation, vascular/effusions -EEGs -ASEPTIC: -neg CSF bacterial cx -viral, lyme, syphilis, TB, parameningeal infection, chemical exposure, autoimmune, kawasaki ds
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cerebral palsy
-static impairment of motor development -> tone, strength, coordination, movement -causes: preterm, hypoxic brain injury, infection, genetics -nonprogressive but impairment may not be apparent until 2-4yo when motor expectations are there -Subtypes: spastic (80%), ataxic, athetoid/dyskinetic, hypotonia w/o spasticity -SPASTIC: -Described based on limb(s) affected: Monoplegia, hemiplegia, diplegia, and quadriplegia -Affected limb may be smaller/shorter than others -increase muscle tone and DTR -scissor gait -muscle weakness, atrophy -MC comorbidities: Epilepsy (25%) and intellectual disability (50%) -Others: Vision impairment, language delay, AS, learning disabilities -Dx- -MRI- extent, etiology, stroke/brain malformation -genetic testing, metabolic testing -Tx: -PT, OT, speech therapy -task-specific approach -intensive constraint-induced movement therapy -mild CP - improve with age -> can resolve motor deficits -severe- affects lifespan -PNA or sepsis- MCC of death -many have normal lifespan and productive life
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tics tx
-Pharm tx if tics are physically painful, disrupt social relationships, or prevent pt from focusing on school -1st-line: Clonidine or guanfacine -SE: Drowsiness, lightheadedness -2nd-line: Topiramate -FDA-approved: Haloperidol, pimozide, and aripiprazole – typically reserved for refractory tics
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spina bifida
-defective closure of caudal neural tube (end of 4wk gestation) -Myelomeningocele: -Flaccid paralysis -loss of sensation in legs -incontinence of bowel/bladder -Extent depends on location -assoc Chiari type II malformation -> hydrocephalus and weakness of face/swallowing -Meningocele: -Spinal canal and cystic meninges exposed on back -underlying spinal cord is anatomically and functionally intact -Spina bifida occulta: -Skin of back is intact -defects of underlying bone or spinal cord present -Meningoceles/spina bifida occulta may: -Be assoc w/ lipoma, dermoid cyst, or dimple/tuft of hair -Have dermoid sinus: Epithelial tract extending from skin to meninges -> increased risk of meningitis -Have weakness/numbness in feet (ulcerations) or difficulties controlling bowel/bladder function (UTIs, reflux nephropathy, renal insufficiency) -Dx: -Myelomeningocele - high (AFP) -> confirmed by US and AChE + AFP in amniotic fluid -After birth: Screening US w/ MRI to confirm less dramatic spinal abnormalities -Tx/Prevention: -Myelomeningocele- Operative closure of open spinal defects -Tx of hydrocephalus -> VP shunt -Toddlers/children w/ lower spinal cord dysfunction -> PT, bracing of LE, and intermittent bladder catheterization -Most survivors have normal intelligence -Learning problems and epilepsy -Prevention -> folate
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bacterial meningitis
-acute, subacute, or chronic -Brain parenchyma inflamed and blood vessel walls infiltrated by inflammatory cells -> endothelial cell injury -> vessel stenosis -> 2ndary ischemia/infarction -Tx: -Empiric tx w/ broad-spectrum antibiotic coverage (varies w/ age) -VP shunt: MC coagulase-negative staphylococci -> MRSA -Severely ill pts: Vancomycin + 3rd-generation cephalosporin (S. aureus and gram-negative rods MC) -N. meningitidis: 5-7 days of tx -H. influenzae: 7-10 days of tx -> !Adjuvant dexamethasone -> significantly decrease hearing loss!!!! -S. pneumoniae: 10-14 days of tx -> subdural effusion is MC complication -Gram-negative bacilli: 21 days of tx
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CNS infection: poliovirus
-ASYMPTOMATIC- 90-95% -Initial sx: Fever, myalgia, sore throat, HA for 2-6 days -<5% of infected kids: asymptomatic -> then recurrent fever + sx of aseptic meningitis (HA, nuchal rigidity, and nausea) -1-2% have high fever, severe myalgia, and anxiety -> herald progression to loss of reflexes and subsequent acute flaccid asymmetrical paralysis -paralysis- proximal limb muscles -> distal limbs + LE -> UE -Bladder distention and marked constipation w/ LE paralysis -Paralysis complete by time temp normalizes -Atrophy by 4-8 weeks -Improvement of muscle paralysis w/in 6mo -Sensation remains intact -> hyperesthesia of skin overlying paralyzed muscles is pathognomonic! -brainstem involvement affects swallowing, speech, and cardiorespiratory function, accounting for most deaths -Dx: -Meningeal sx: CSF w/ lymphocytic pleocytosis, normal glucose, mildly elevated protein -Poliovirus on cell cx, readily differentiated from other enteroviruses -Rarely isolated from spinal fluid -> often throat and stool for several weeks following infection -PCR is preferred -Complications: -permanent destruction of anterior horn cells and paralysis -Respiratory, pharyngeal, bladder, and bowel malfunction are most critical -Death usually from respiratory dysfunction -Limbs injured near time of infection -> most severe and have worst prognosis for recovery -Tx/Prognosis: -Supportive -No IM injections during acute phase -> may enhance paralysis -Intubation or tracheostomy, enteral feeding, catheter drainage of bladder -Post-polio muscular atrophy in 30-40% of paralyzed limbs 20-30 years later -Increasing weakness, fasciculations in previously affected, partially recovered limbs
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CNS infection: tetanus
-Clostridium tetani (anaerobic, gram-positive bacillus) -clostridial spores from animal manure soil in wound -potent neurotoxin -> to CNS -> increases reflex excitability (muscle spasms) -MC- minor puncture wounds of hand or feet -RF: IV drug use, DM, no immunization, contamination of umbilical cord (underdeveloped countries) -Incubation- 3-21 days (neonates, 4-14 days) -1st sx- mild pain at wound site -> hypertonicity and spasm of regional muscles -Trismus (difficulty opening mouth) w/in 48hrs -Newborns: 1st sign -> irritability and inability to nurse -> stiffness of jaw/neck, increasing dysphagia, and generalized hyperreflexia/spams of abdomen and back (opisthotonos) -Facial distortion/grimacing (risus sardonicus) -Convulsions triggered by minimal stimuli (sound, light, or movement) -Individual spasms last secs or mins -Temp- normal -conscious and lucid -Profound circulatory disturbance assoc w/ sympathetic overactivity days 2-4 > contributes to mortality rate -Tx: -Surgical debridement of wound -Human TIG x 1 dose (3000-6000U), IM with infiltration of part of dose around wound -If unavailable -> equine tetanus antitoxin -Age-appropriate tetanus toxoid containing vaccine (different limb from TIG administration site) -Antibiotics -> 1st-line: IV/PO metronidazole x 7-10 days -IV PCN G alternate -Complications: Sepsis, malnutrition, PNA, atelectasis, asphyxia spasms, decubitus ulcers, fx in spine (from contractions) -Prognosis: -high fatality in newborns- injection drug users and pts w/ DM -US mortality- 6% -> PNA or respiratory failure -Complete recovery -> months -Prevention: -Active immunization w/ tetanus toxoid -Tetanus immunoglobulin (TIG) to prevent tetanus in those w/ < 3 doses who have tetanus-prone wounds (or those who are immunocompromised w/ tetanus-prone wounds regardless of immunization hx) -Tetanus toxoid booster at time of injury if: -None given w/in 10 years -None given w/in 5 years for heavily contaminated wounds
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depresssion
-children/younger -> irritable mood -adolescents- sad -“I have no friends,” “life is boring,” “there is nothing I can do to make things better,” “I wish I were dead” -social isolation, poor schoolwork, anhedonia, anger, sleep/appetite changes, HA, MSK pain, abd pain -Dx: -Major depressive disorder- persist > 2wks, and impairment -Dysthymic disorder- lesser severity, persist for >1yr -Adjustment disorder w/ depressed mood- milder sx in response to stressful life event -screen annually in 12yo+ -> PHQ-9A -Tx: -mild- close monitor over several wks and psychoeducation that includes caregivers -moderate-severe- comprehensive plan, support within school and parents -CBT- coping skills and change negative thought patterns -interpersonal therapy (IPT) -moderate + persistent or severe -> SSRI -> BEST- CBT + fluoxetine!!! -toddlers and young kids -> parent-child relational therapies -ONLY fluoxetine and escitalopram are FDA approved -comprehensive tx -> complete remission over 1-2mo -> continue meds 6-12mo after remission of sx
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ADHD
-hyperactive/impulsive and/or inattentive -in at least 2 settings -Tx: -behavioral therapy 1st line -Stimulants 1st line med -> try diff classes if one doesnt work (amphetamine vs methylphenidate) -ADRs: Anorexia, wt loss, abd distress, HA, insomnia, dysphoria/tearfulness, irritability, lethargy, mild tachycardia, mild elevation in BP -Less common SE: Interdose rebound of ADHD sx, anxiety, tachycardia, HTN, depression, mania, and psychotic sx -Caution taken w/ cardiovascular hx, personal/family hx of drug abuse/addictive disorders (transdermal patches may be better) -Use w/ caution in those w/ psychotic disorders and those w/ bipolar affective disorder (worsen sx) -MONITOR: Pulse, BP, ht/wt q 3-4 mo and at times of dosage increases; also record abnormal movements (tics) -Prognosis: -60-85% w/ ADHD in childhood continue into adolescence -1/3rd of adults w/ ADHD in childhood require ongoing med management
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opposition defiant disorder (OOD) vs conduct disorder
-OOD: -RF- family/environmental dysfunction -> substance abuse, stress etc., multiple changes of primary caregiver, neglect, abuse. violence exposure -Dx: -<8yo -presents at home then school -negativistic, hostile, defiant behavior for at least 6mo -argues, defies rules -blames others for own mistakes -does not meet criteria for conduct disorder -Tx: parenting skills, assess for learning disability, depression, ADHD -CONDUCT DISORDER: -overlap with ADHD, substance abuse, learning disability, neuropsychiatric disorders, mood disorder, family dysfunction -RF- domestic violence, abuse, drugs, shifting parental figures, poverty -inconsistent and severe parental discipline, parental alcoholism, parental antisocial behavior -Dx: -at least 12mo -hyperactive, AGGRESSIVE, uncooperative -DEFIANCE, fighting, tantrum, running away, school failure, DESTRUCTION of property -older- fire setting, THEFT, vandalism, sexual promiscuity -Tx: -multisystemic therapy (MST)- home based model of care -> stabilize and improve home -identify learning disability -> adapt -programs- big brothers, team sports, scouts -> adult mentors -juvenile system if criminal -Prognosis: -worse if disorder <10yo, antisocial behaviors across multiple settings, pts raised in environments w/ antisocial behaviors of parent -50% develop antisocial personality disorder as adults
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autism
-deficits in social communication/interaction in multiple contexts -Restricted, repetitive patterns of behavior, interests, or activities -ASD includes pervasive developmental disorder and Asperger syndrome -M>F -Dx Criteria: -sx < 3yo (usually) -> dx at 3/4yo but can see signs 12-18mo -impairment in function -Severity = level I, II, III based on how much support is needed -MC sx (12-18mo): cant orient to names, regard people directly, gesture, develop speech -Evidence dx can be made at 14-18mo -> early intervention is important -> multiple screening tools (for parents and clinicians) -When ASD behaviors noted: -Refer to team of specialists -early intervention program -speech / language pathologist -Testing: -1st-tier: Audiology eval, chromosomal microarray/DNA for fragile X syndrome -2nd-tier: Whole exome sequencing, whole genome sequencing, autism gene panels -Metabolic screening, lead level, TFTs -Tx: -Early, intensive (25 hrs/week) behavior intervention -Training models < 3yo -> 90% functional use of language (20% after 5yo) -parent training/involvement in tx, ongoing assessment, program eval, and programmatic adjustment as needed -communication, social interaction, play skills -> generalized in a naturalistic setting -PCP for medical concerns -> sleep problems, feeding problems w/ limited diet, constipation w/ withholding, and seizures -Psychiatric comorbidities (anxiety/ADHD) also common -Moderate efficacy of CBT for anxiety and/or SSRIs -Irritability/aggression: Risperidone and aripiprazole -Complementary tx: Special diets/supplements (limited evidence)
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anxiety
-can be identified as early as infancy (especially if living w/ anxious parents -children have physical complaints -> HA, abd pain -screen for depression, meds/substances that can cause anxiety/present similar (caffeine, weed, amphetamines, cocaine, alcohol withdrawal), thyroid -Tx: -young- educate parents and teach skills to manage -incorporate child into therapy as soon as they can understand anxiety and coping -1st line- CBT w/ exposure -> desensitize -2nd line- SSRI (>=6yo) -DONT use benzos while waiting for SSRIs to kick in -anithistamines (hydroxyzine), BB, and alpha agonists are alternatives -tx the parents too if they have anxiety -high risk for depression, substance abuse
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child abuse
-MC- neglect -> physical -> sexual -PHYSICAL: -alopecia (hair pulling), dental, ligature marks -bruise patterns- belt marks, looped cord marks, grab/pinch marks over soft tissue -burn patterns: stocking/glove, immersion of butt (doughnut hole), branding (cigarette, hot objects), NO splash marks -LE burn that spare flexor surface -unwitnessed injury, delay in care -TEN - torso, ears, neck -FACES - frenulum, angle of jaw, cheeks, eyelids, subconjunctivae -INFANTS- 4mo or less -IMAGING: -Metaphyseal “corner” or “bucket handle” fx of long bones in infants, spiral fx in non-ambulatory infants, rib fx, spinous process fx, fx in multiple stages of healing -CT/MRI w/ subdural hemorrhage in infants (in absence of clear accidental injury) -Abdominal CT for suspected abdominal trauma -Coagulation studies, CBC w/ platelets, LFTs -EMOTIONAL/NEGLECT: -MC- verbal abuse or degradation -MC sx- abnormal parent-child attachment and inability to respond to childs needs -FTT -> dietary needs not met, clothing, shelter -> social services -> rapid wt gain after replacement -medical care neglect- not getting care for life threatening/chronic illness -medical child abuse- unecessary medical care -> MC- recurrent apnea, dehydration, sepsis, change in mental status, fever, GI bleed, seizures
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suicide
-firearms > suffocation > poisoning -MC- american indian youth, fastest increase in black youth -RF: other mental ds, drugs, sleep ds, intox at time of event, hx of attempt, hx of self harm, social media exposure -other external RF: trauma, adoption, loss, familial suicide, parental mental ds -most young pts who attempt show signs of distress or plans -on screen 60% say “I wish I were dead” or “I just can’t deal with this any longer” -70% had crisis event (loss, public shame, failure, arrest) -protective factors- peers, communities (school, sports, work, religion), family, problem solving, emotional regulation -ASSESSMENT: ->12yo annual screening -if suicidal thinking -> ask ab plan, intent to carry out plan, access to lethal menas, hx of prior attempts -suicidal ideation + plan = psychiatric crisis assessment (ED) -Tx: -dont leave pt alone -meet with the pt and fam (alone and together) to start finding assistance of professionals -referral or ED -> always be extra cautious -prevention- awareness and resources, restrict access to firearms/sharps, lock meds
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transgender
-GnRH at tanner stage G2/B2 -eval for confirmed dx and capacity for informed consent
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osteogenesis imperfecta
-multiple recurrent fx -Several forms -Reduced cortical thickness in shafts of long bones -accessory skull bones -blue sclerae -thin skin -hyperextensibility of ligaments -otosclerosis with hearing loss -hypoplastic/deformed teeth -Tx: -Surgery: Correction of long bone deformities -Intramedullary rods to decrease incidence of fractures and prevent deformity from fracture malunion
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sexual abuse
-teach kids ab private parts at 3yo (toilet training) -prepubertal- N. gonorrhoeae or syphilis is dx -consider w/ c. trachomatis and HIV -NAATs (Female) and culture (males) for screening -rape kit -prior to speculum consider child maturation -colposcopy to determine extent of trauma -Ceftriaxone + doxycycline now preferred; metronidazole (bacterial vaginosis) -preg test -contraception if rape within 120hrs -hep B w/ or w/o Ig - perpetrator known to be infected)
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scoliolosis
-4 categories: idiopathic, congenital, neuromuscular, syndromic -Idiopathic (80%): F>M, 10-12yo or sooner, genetic/multifactorial etiology -Congenital (5-7%): failure of formation/segmentation of vertebrae -no back pain -curvature of >= 30° -> deformity of rib cage and asymmetry of waistline -Adams Forward Bend test -> for lesser curves -Rotation of spine measured with scoliometer -marked rib hump and lateral curvature increases severity -Imaging: -xray of entire spine- standing (PA/lateral): Primary curve evident with compensatory curvature to balance body -Tx: -dependent on Cobb angle – measured on standing PA x-ray -< 20°: No tx -20-40°: Bracing if skeletally immature -> 40°: Resistant to bracing -40-60°: Surgical -> Spinal instrumentation (rods, screws, hooks) and fusion (bone graft) -Thoracic curvatures > 70°: Poor pulmonary function
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nursemaids elbow
-presentation- elbow fully pronated and painful (point tenderness over radial head) -Reduction: Palpable/audible click -stabilize radial head with one hand (thumb) -> Place elbow in full supination and slowly move arm into full flexion -Holding elbow at 90° angle of flexion, then slowly hyperpronating the wrist -immediate relief of pain -May immobilize in sling for comfort x 24hr (or longer, as needed) -refractory- posterior splint and f/u with ortho
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growth plate fx
-MC- Distal radius, distal tibia, and distal fibula -Growth plates are most risk to torsional and angular forces -Salter-Harris classification!!!!!! -Type I: Transverse fx through growth plate (physis) -> no growth disturbance -Type II: Fx through portion of physis and metaphysis; MC type (75%) -Type III: Fx through portion of physis and epiphysis -> complication bc of intraarticular component/disruption of growing/hypertrophic zone of physis -Type IV: Fx through metaphysis, physis, and epiphysis -> high risk of complication -Type V: Crush injury to physis with poor functional prognosis -Tx: -Types I and II: Closed reduction -Types III and IV: Anatomic alignment for successful tx -Type V: Rare, often result in premature closing of the physis
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other fractures: torus, supracondyle, toddlers
-TORUS (BUCKLE): -wrist and ankle -buckling of cortex due to compression -MC- distal radius or ulna -> FOOSH -Dx- important to get both views -Tx- immbolize for 3wks -SUPRACONDYLAR: -fall from ht -3-6yo -MC elbow fx in kids -close to brachial artery -> check pulses -Dx- anterior and posterior fat pad (sale sign) -Tx- closed reduction with percutaneous pinning -complication- volkmann ischemic contracture of forearm (vascular compromise) -> cubitus varus (gunstock deformity) -TODDLERS (pic): -trampoline, learning to walk -Oblique fx of distal tibia w/o fibular fx -no significant trauma -1-3yo, but can be as old as 6 -limping and pain with wt-bearing vs minimal pain/swelling -Initial x-ray may not show fx -> repeat in 7-10 days (with oblique view) -Tx: Immobilization, usually heal within 3-4wks w/o disruption in growth
75
developmental dysplasia of hip
-Dislocated hip: Femoral head not in contact with acetabulum -Dislocatable hip: Femoral head in acetabulum, but can be dislocated -Subluxatable hip: Femoral head comes partially out of joint -Acetabular dysplasia: Insufficient acetabular development (radiographic dx) -4 major RF: 1st born child, female, breech, and family hx -Clinical dx -Barlow sign: Adduction of hip (hip slips posteriorly) -> palpable “clunk” as head dislocates -Ortolani sign: Hips flexed to 90 degrees, abducted, lift greater trochanter forward -> feeling of “slipping” as head relocates -Limited abduction (60 degrees) with hip at 90 degrees flexion is most sensitive sign for detecting a dysplastic hip -instability less evident after 1mo -Galeazzi sign: Knees at unequal heights when hips and knees flexed -> dislocated hip on side with lower knee -Dx after walking begins: -Painless limp/lurch to affected side -Trendelenburg sign: Standing on affected leg -> dip of pelvis evident on opposite side (weakness of gluteus medius muscle) = swaying gait -B/L dislocation = waddling gait -Imaging: -US- most useful in newborns -Xray after first 6wks of life: Lateral displacement of femoral head -Tx: -Dysplasia progressive w/o correction –> early dx and tx is essential -Pavlik harness: Maintains reduction by placing hip in flexed and abducted position -If hip cannot reduce easily on exam, Pavlik harness is CI -Closed reduction with arthrogram, followed by hip spica cast -Still unstable with closed reduction -> open reduction
76
avascular necrosis of proximal femur (Legg-Calve-Perthes)
-no Vascular supply -> necrosis -> replacement of necrotic bone (creeping substitution) -Rapid growth of 2ndary ossification centers in epiphyses -> outgrow blood supply -> avascular necrosis (also with trauma/infection) -4-8yo -Persistent pain with possible limp and/or limited motion -Imaging: -look for density changes -shape of femoral head determines extent -Tx: -Protection of joint by minimizing impact - less wt bearing -Nonop (casting) and surgical approaches to promote containment of femoral head within acetabulum and abduction of hip -Prognosis: -Better outcomes for < 6yo -Poorer prognosis for more completed involvement of epiphyseal center, metaphyseal defects, more complete involvement of femoral head
77
slipped capital femoral epiphysis (SCFE)
-MC- adolescent, obese males -Displacement of femoral epiphysis due to disruption of growth plate -Head of femur is displaced medially and posteriorly -increased stress across growth plate or resistance to shear is reduced -RF: Endocrine/renal ds, obesity, coxa profunda (deep acetabular socket), femoral/acetabular retroversion -classified as STABLE vs UNSTABLE: -Stable: Child can bear wt -Unstable: Child cant bear wt with increased rate of AVN -Temporally classified as ACUTE vs CHRONIC -Acute: after fall/direct trauma to hip; sx < 3wks -Chronic: Vague sx over period in an otherwise healthy pt who has pain and limp -Pain referred to thigh or medial side of knee (always examine hip in obese pt presenting with knee pain!) -PE: Limitation of internal rotation of hip -Imaging: AP/lateral pelvis xray -Steel sign, widening of growth plate on affected side, epiphysis below Klein line -kline should go through epiphysis -Tx: -NWB on crutches and immediate referral to an ortho surgeon -ORIF -> due to high risk of AVN of femoral head assoc with closed reduction -Prognosis is guarded: -Most pts continue to be overwt and overstress hip joints -High rate of premature degenerative arthritis (even w/o AVN) -30% have B/L involvement which may occur as late as 1-2 years after primary episode
78
transient synovitis vs pyogenic (septic) arthritis
-TRANSIENT SYNOVITIS: -MCC of limping and hip pain in US kids (3-10yo) -Self-limiting, acute inflammatory reaction -after URI or GI infection -Hip joint -> limited ROM, particularly internal rotation -xray- nonspecific; some swelling around joint -Must r/o septic arthritis! -No elevation in ESR, WBC count, or fever > 38.3C; clear aspirate from joint (if performed) -definitively r/o with dynamic contrast enhanced MRI -Tx: -Rest and NSAIDs -xray f/u important -> precursor to AVN of femoral head (6wks or earlier if limp persists) -PYOGENIC (SEPTIC) ARTHRITIS: -Infant: osteomyelitis -Older kid: Isolated infection w/o bony involvement -Teens: Underlying systemic ds or organism that has affinity for joints (Neisseria gonorrhoeae) -< 4mo: GBS, S. aureus -4mo–4yrs: H. influenza B (if unimmunized), S. aureus -Older kids/adolescents: S. pyogenes, S. aureus, S. pneumoniae, N. meningitidis, N. gonorrhoeae -Kingella kingae is a gram-neg increasingly recognized in < 5yo -Initial effusion rapidly becomes purulent -> may affect adjacent bone -Infants: Decreased abduction of hip, irritable, or feeding poorly -Older kids: Fever, malaise, vomiting, restriction of motion, joint swelling, warmth, erythema, and/or tenderness -Imaging: -xray: -Dislocation of joint (within days) -Destruction of joint space, resorption of epiphyseal cartilage, erosion of adjacent bone of metaphysis (later) -Bone scan: Increased flow and uptake about joint -MRI/US: Joint effusion detection -Dx: -Aspiration of joint for dx -Need for aspiration evaluated with Kocher criteria -NWB, ESR > 40 mm/h, temp > 38.5C, WBC > 12K cells/mm^3, CRP > 2.0 mg/dL -Tx: -WBCs > 30-50K cells/mm^3 -> surgical drainage + antibiotics -Empiric: Nafcillin, oxacillin + 3rd gen cephalosporin -Antistaphylococcal agent alone adequate > 5 yo (unless gonococcal/meningococcal infection suspected) -Prognosis: Excellent prognosis if joint drained before damage to articular cartilage (> 24hrs) and/or growth plates
79
juvenille idiopathic arthritis
-Oligoarticular (MC, 40-60%): 4 or less joints, medium-large joints, asymmetrical (leg-length discrepancy) -20% - !!asymptomatic uveitis (may cause blindness) -Polyarticular (20-35%): 5+ joints, large and small joints, symmetrical -Low grade fever, fatigue, rheumatoid nodules, and anemia -Further divided into !RF-positive vs RF-negative -Positive is chronic, destructive arthritis -Systemic (10-15%): Any # of joints, large and small joints -High fever (39-40C), 1-2 times per day -~80-90%- evanescent, salmon-pink macular rash! -> most prominent on pressure areas, especially when fever is present -Hepatosplenomegaly, lymphadenopathy, leukocytosis, and serositis -Enthesitis-associated (5-10%): MC in males >10yo, -assoc with LE large joints -hallmark is inflammation of tendinous insertions (enthesitis) -Low back pain and sacroiliitis also common -Psoriatic: -psoriasis or more subtle changes, like nail pitting -May have dactylitis (“sausage digit!”) – painful swelling of entire finger or toe -Undifferentiated (10%): Chronic arthritis w/o meeting criteria for any or meet more than one criterion that classify them into multiple subgroups -tx: NSAIDs, methotrexate (CBC+LFTs q 2-3mo), biologics, steroids, steroid drops for eyes
80
head injury
-CONCUSSION: -resolves in 1-4wks -remove from play -CT if dereriorating/AMS, prolonged LOC, repeated vomiting, severe HA, signs of skull fx, neuro deficit, severe MOI -NO CT if >24hrs -Return to play- 6-step process (each 24 hrs): -1. Asymptomatic at rest -2. light aerobic exercise -3. sport-specific exercise -4. noncontact drills -5. contact practice drills -6. release to game play -Worsening sx in 1st 24hrs -> more severe TBI -vital signs, LOC by AVPU system or GCS -PE: neuro exam, basilar skull fx -IMAGING: -Close observation vs CT scan (PECARN score) -infants- normal neuro exam does not r/o significant ICH -Consider imaging if large scalp hematoma or concerns of nonaccidental trauma -COMPLICATIONS: -CNS infection -increase ICP- AMS, HA, gait change, vision, vomit, pupils -> PAPILLEDEMA -cushing triad- brady, HTN, irregular respiration -CT before LP if high ICP -PREVENTION: -helmet -furniture falling
81
animal bites
-UE- MC -Imaging- fx and dislodged teeth -debride -irrigate with normal saline under high pressure (>1L) -tetanus prophylaxis -rabies prophylaxis -bites involving tendon, joint, periosteum, fx -> ortho surgery consult -surgery consult for hands and feet (especially knuckles / extensor tendon)
82
swim and heat safety, sunscreen/block
-SWIM SAFETY: -toilets, buckets, washing machine -protective fencing, life vests, never swim alone, supervision -swimming lessons- even 1-4yo -warm them -HEAT: -avoid extreme temp for long times -go early morning or late afternoon -acclimation, water, shade, rest -remove clothing -incoherent/combative/CNS- hallmark sign of heat stroke (>40.6) -SUNSCREEN/BLOCK: -infant approved sunscreen >6mo -sunscreen- absorbs UV -minimum SPF is 15-30 -reapply q 2-3hrs if getting wet -sunblock- deflects the suns ray
83
poison
-<6yo- usually accidental -large BSA -> vulnerable to topical exposure -ACETAMINOPHEN: -liver necrosis -N/V, abdominal pain -> asymptomatic -> LFTs peak, coagulopathy, renal failure, fulminant hepatic failure, encephalopathy, sepsis, coma, death -Tx- NAC PO or IV -IBUPROFEN: -asymptomatic -MC- abd pain, vomit, drowsy, lethargic -Tx: -if <100mg/kg -> supportive for GI upset -if >400mg/kg -> seizure/coma -> no specific antidotes -SALICYLATES: -ASA -increase heat production, excessive sweating, dehydration -hypo or hyperglycemia -respiratory center stimulation -mild-moderate- vomiting, tinnitus/hearing loss, AMS, sweat, tachypnea, kussmauls -severe- fever, sweat, pulmonary edema, seizures, death -Tx: -charcoal if acute and vomiting (normal mentation) -mild- PO fluids, salicylate level monitoring -moderate- IV fluids with K supplementation -severe- hemodialysis -CARBON MONOXIDE: -toxicity correlates to carboxyhemoglobin concentration -HA, flu-like -confusion, unsteadiness, coma -cardiac, liver, renal, CNS damage -Tx- 100% oxygen (lowers half life from 200-300mins to 60-90mins)