Pediatrics Flashcards

1
Q

Perinatal Mortality

A

Fetal Death between 20 weeks EGA to the 28th day post-delivery

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2
Q

Prematurity (Pre-term Infant)

A

< 38 weeks

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3
Q

Low Birth Weight (LBW)

A

< 2500gm ( Prematurity # 1 cause)

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4
Q

Very Low birth weight (VLBW)

A

< 1500gm

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5
Q

Risks for Low Birth Weight (LBW)

A

Maternal Age < 16 or >35 y/o

Low economic status

TOB/ETOH/Drugs/Rx

Unmarried, African American, Low Education

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6
Q

APGAR test predicts long term outcomes and it is performed at what times

A

1 (Birth Toleration) and 5 minutes (Adapting to envir.)

Every 5 min. if score < 8

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7
Q

APGAR stands for

A
  • Appearance *Pulse
  • Grimace *Activity
  • Respiration
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8
Q

Neonates respond to hypoxia w _____ rather than tachypnea

A

Apnea

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9
Q

IV fluid bolus indicated for hypotension in Neonatal Resuscitation

A

10ml/kg NS or LR

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10
Q

When is Narcan not given

A

If mother is narcotic or drug abuser or addict–> neonatal seizures

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11
Q

When is fetal hemoglobin replaced by adult HGB

A

3-6 months

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12
Q

Ductus arteriosus closes typically within ___ days

A

1 Day

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13
Q

Ductus Arteriosus shunts blood from

A

Pulmonary Artery trunk to the aorta

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14
Q

Foramen Ovale shunts blood from

A

R Atrium to Left Atrium Closes up 1st fetal breath

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15
Q

Initial neonatal Nursing care

A

Erythromycin, Vitamin K, Hearing screen, and HEP-B

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16
Q

Vitamin K in the neonate prevents

A

Hemorrhagic disease of the newborn MC breast fed pts

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17
Q

Iron supplementation for breast fed infants begins at

A

4 Months of age ( 1mg/ kg/day)

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18
Q

start vitamin D 400 IU/day to prevent

A

Rickets

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19
Q

Changes seen in Vitamin D deficiencies infants

A

Craniotabes- Thinning of outer table of skull (feels like a ping pong ball to touch)

Thickening of wrists and ankles, Rachitic Rosary (Costochondral Junction Enlargement), Enlarged Ant. Fontanelle–> delayed closure

Bowlegs or knock knees

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20
Q

<10% weight for age

A

Small Gestational Age (SGA)

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21
Q

> 90% weight for age Associated w maternal Diabetes

BG< 45 mg/dL, jitteriness, seizures, lethargy, poor feed

A

Large Gestational Age (LGA)

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22
Q

Large Gestational Age (LGA) Tx:

A

Oral feeding or IV D10W

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23
Q

Score to ID abnormal growth patterns and predict neonatal complications

A

Ballard Score

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24
Q

Enlarged anterior fontanelle/ sutures > 5 cm suggests

A

hypothyroidism

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25
Q

Term for closed fontanelles/sutures

A

Craniocynostosis

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26
Q

Leukokoria or white reflex can include which DO

A

Cataract, ocular tumor, chorioretinitis, retinopathy

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27
Q

Cyst or bulge at ant. Sternocleidomastoid muscle

A

Branchial cleft cyst

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28
Q

Cyst or bulge at Post. Sternocleidomastoid muscle

A

Cystic Hygroma

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29
Q

Cyst or Bulge anterior midline of neck

A

Thyroid d/o

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30
Q

Neonatal Exam

A
  • Head fontanelle/suture *Eyes * Neck
  • Clavicle Fx *Heart/Lungs * Pulses *Abdomen
  • Umbilical cord * Hips * Penis/Vagina * UE/LE
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31
Q

Abnormalities of the Fontanelles/ sutures

A
  • Closed sutures *Overriding sutures
  • Caput Succedaneum * Cephalohematoma

Subgaleal hemorrhage

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32
Q

Less common newborn head deformity that does not cross the suture lines and resolves in weeks-months

Increases in size after birth 12-24 hrs. Can lead to jaundice from Inc. RBC breakdown

A

Cephalohematoma

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33
Q

Common newborn head deformity that crosses suture lines. Boggy edematous swelling of fetal scalp

Resolves in days. Disappears without treatment

A

Caput succedaneum

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34
Q

Rare newborn head deformity that crosses suture lines, beneath epicranial aponeurosis, extends to orbits

Pushes ears anteriorly. May have crepitus or fluid waves. Progressive may be massive

A

Subgaleal Hemorrhage

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35
Q

Subgaleal Hemorrhage Tx

A

Compression and resuscitate as needed

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36
Q

Neonatal Strabismus should resolve by what age

A

X 4 months

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37
Q

improper alignment appearance due to Epicanthal folds

A

Pseudostrabismus

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38
Q

A scaphoid Abdomen (sunken) in a newborn =______ until proven otherwise

A

Diaphragmatic Hernia

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39
Q

Umbilical cord inspection includes ____ artery____ vein

A

2 arteries (Deoxygenated) 1 vein (oxygenated)

Falls off 3-4 weeks

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40
Q

Examiner adducts while applying post. pressure on knee. Test will dislocate hip.

A

Barlow

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41
Q

Examiner abducts the hip while applying ant. force on the femur, reduces the hip

A

Ortolani

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42
Q

Ligamentous laxity that can –> spontaneous dislocation and reduction of femoral head.

Left hip is affected 3x more than the right.
Joint capsule tightening, muscle contractures dec. motion, and flattening acetabulum. F:M 9:1

A

Congenital Hip dysplasia

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43
Q

Congenital Hip dysplasia Evaluation initial study

A

Ultrasound obtained after 6 weeks of age w laxity at birth

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44
Q

Congenital Hip dysplasia Tx

A

Refer to Peds/Ortho

  • Pavlik Harness (Effective up to 6 mth age)
  • Abduction Orthosis or Closed redux with spica cast
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45
Q

Newborn exam of the back for

A

Spine symmetry, Lumbosacral hair tuft (spina Bifida), Gluteal fold for dimples (malformation of spine)

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46
Q

Nodules resemble emerging teeth. on gum or palate. Harmless and resolve w/I few weeks of life

A

Epstein Pearls/ Bohn Pearls

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47
Q

occurs 24-48 hrs where infant skin sloths off (Normal)

A

Infant Desquamation

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48
Q

Chalky-white to gray mixture of shed epithelial cells, sebum, Keratin, and sometimes hair. common in preterms

Funx Unknown thought to protect and lubricate in womb

A

Vernix Caseosa

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49
Q

White, smooth papules (up to 2mm) on face and scalp. caused by epidermal pore occlusion w trapped keratin

Self limited within few weeks of life

A

Milia

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50
Q

Common in overheated/ febrile infants head, neck trunk, scalp. “Heat rash” correct overheating

A

Milia Rubra

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51
Q

Transient blue black pigmented macules. Lower back and buttocks. African American/Asians/ Indian

Fades over 1st several years of life

A

Mongolian Spots

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52
Q

Light-Dark brown sharply defined oval macules on any skin surface. > 6 macules > 5mm

A

Café-Au-Lait Macules

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53
Q

Café-Au-Lait Macules should lead you to evaluate further for what d/o?

A

Neurofibromatosis

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54
Q

Stork Bite (Nape of neck) or Angel Kiss (Forehead eyelids) Transient and benign.

A

Nevus Simples (Salmon Patch)

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55
Q

Caused by malformation of capillary bed. Persists throughout the patient’s life

AKA “Port Wine Stain”

A

Nevus Flameus

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56
Q

Nevus Flameus present should lead provider to evaluate further for

A

Sturge Weber Sydnrome ( Trigeminal Distribution)

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57
Q

Pustules w erythematous base appearing 24-48 hrs post birth. Resolves in 14 days. On trunk or back.

Microscopic evaluation reveals esoinophils

A

Erythema Toxycum Neonaturum

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58
Q

Due to exposure of maternal estrogen. Appears 1st weeks of life or birth. Cheeks and scalp.

Self-limited

A

Neonatal Acne

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59
Q

Very common physiologic response to cold. Resolves w warmth. decreases w aging

A

Cutis Marmorata (Mottling)

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60
Q

Persistent Cutis Marmorata (Mottling) may indicate____

A

Hypothyroidism or vascular malformations

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61
Q

Seborrheic dermatitis or may be the 1st sign of Atopic Dermatitis

A

Cradle Cap

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62
Q

Medial deviations of the mid and forefoot

A

Metatarsus Adductus

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63
Q

Mid heel bisector line should go between toes ____ and ____

Should not gap at _______ MT with V finger test

A

Toes 2 and 3 Not Gap at base of 5th MT

Tx- Serial Castings

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64
Q

Inversion, adduction and plantar flexion of foot. Bilat 50% of cases.

Extrinsic-able to reduce on exam (Supple)

Intrinsic- Unable to reduce on exam (rigid)

A

Clubfoot (Talipes Equinovarus)

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65
Q

Occurs from incomplete development of the brain spinal cord or meninges

diagnosed during pregnancy 2nd trimester US or Maternal alpha fetal protein

A

Spina Bifida

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66
Q

Minor defect where hair tuft is present no neuro S/S. Check for connecting sinus or Inc. risk for meningitis

A

Spina Bifida Oculta

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67
Q

meninges herniates through neural arch.

A

Meningocele

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68
Q

Meninges and cord herniate through neural arch

A

Meningomyelocele

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69
Q

Open skin and spinal cord exposed

A

Myeloschisis

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70
Q

Spina Bifida is defined as

A

Meningocele, Meningomyelocele, Myeloschisis.

Any exposure besides hair tuft

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71
Q

Spina Bifida S/S and Tx.

A

Learning/ Mental disability, Paralysis, sphincter laxity, hydrocephalus.

Tx : Neurosurgery (Prevention = Folate)

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72
Q

A result of forceps used in delivery

A

Facial Nerve paralysis (Transient Drooping)

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73
Q

Associated w phrenic nerve lesion. Lesion C5-C6
Grasp present, Bicep flex absent. (Waiter tip Palsy)

Shoulder Dystocia typically resolves w PT and Observe

A

Erb-Duchenne’s Palsy (Brachial Plexus Lesion)

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74
Q

Assoc. w Ipsilateral Horner’s Syndrome (Symp. nerve injured). C8-T1 Lesion Grasp Absent, Bicep flex Pres.

“claw hand” (Rare Lesion)

A

Klumpke’s (Brachial Plexus Lesion)

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75
Q

Increased volume in the CSF due to a communicating or Non-comm. w subarachnoid

Macrocephaly, vomiting, anorexia, irritable, papilledema bulging fontanel, Setting sun Gaze

A

Hydrocephalus

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76
Q

Hydrocephalus Tx

A

Ventriculoperitoneal shunt (Enlarged ventricle to Stomach)

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77
Q

Risk factors for neonatal sepsis

A

Prematurity

> 24hrs Ruptured membranes (Prolonged)

Group B Strep Colonization * Maternal fever

Amnionitis

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78
Q

Most common bacterias in Early onset neonatal sepsis

(0-7 days old) Fast onset progresses quickly

A

GBS #1 E. Coli #2

Klebsiella Listeria

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79
Q

Most common bacterias in Late onset neonatal sepsis

(8-28 days old) Insidious onset Assoc w Meningitis

A

H. Influenzae Staphylococcus

HSV CMV

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80
Q

Neonatal sepsis treatment

A

Ampicillin + Gentamycin (Or Cefotaxime)
Sepsis confirmed = 14 days tx Meningitis=21 days

(Negative culture = 48-72 hrs)

Add vancomycin if late onset or meningitis

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81
Q

caused by insufficient surfactant production by type II pneumatocytes. common < 34 EGA –> end respiration atelectasis.

Bilateral Ground-glass appearance

A

Respiratory Distress Syndrome

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82
Q

Respiratory Distress Syndrome preventive tx prior to delivery 32-34 wks

A

Maternal steroids

Post birth Tx intubationand Artificial surfactant via ET

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83
Q

caused by acute and chronic effects of O2 toxicity on developing retinal blood vessels

–> retinal detachment, neovascularization, fibrous prolif behind lens

A

Retinopathy of prematurity

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84
Q

Medulla and pons do not stimulate phrenic nerve

Tx:

A

Apnea of prematurity

Tx O2, Stimulant (Caffeine/theophylline),Tx anemia

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85
Q

High pitch or rattling/ noisy breath sounds. worsens w crying, coughing or feeding

If mild, Tx?

A

Tracheomalacia (Floppiness of cartilage wall)

Tx monitor

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86
Q

retained amniotic fluid causing mild hypoxia post-birth that resolves in 24 hrs. CXR shows fluid in fissures

MC LGA infants and C-section (No Pelvic squeeze)

A

Transient tachypnea of newborne

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87
Q

MC cause of hemolytic dz/ Neonatal anemia in newborns.

A

ABO Incompatibility #1 Rh Incompatibility #2

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88
Q

hyperbilirubinemia is defined as Bilirubin..? and MC cause

A

Bilirubin > 5mg/dL MC cause Hemolytic Dz

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89
Q

Jaundice from supply issue due to insufficient milk production or intake during 1st week of life

A

Breastfeeding Jaundice

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90
Q

Unknown beast milk factor inhibits Bilirubin conjugation or breast mil enzyme enhances bili absorption.

After 1st week of life. Infant must be healthy. Dx of exclusion

A

Breast milk jaundice

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91
Q

Physical evidence of jaundice begins at _____mg/dL

A

5-10 mg/dL

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92
Q

Term infant Bili Generally

A

< or = 12 on day 3

< or = 15 on day 5

< or = 13mg/dL in term infants Rise (.5/hr or >5mg/day)

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93
Q

Always pathological jaundice

A

Jaundice on 1st day of life (Hemolysis ABO/Rh) unconjugated

Conjugated= Hypothyroidism, Gilbert’s or Crigler-Najjar, cholestasis, hepatitis, Cystic Fibrosis

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94
Q

Neonate physical exam for juandice

A

Prior siblings, Under tongue 1st to show then sclera

Progresses from head-toe

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95
Q

Lipid-soluble, unconjugated indirect Bilirubin is toxic to developing CNS, deposited in brain cells. disrupts neuronal metabolism and fx

Not common in term infants in Bili of <20-25 mg/dL

A

Kernicterus (Bilirubin Encephalopathy)

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96
Q

Kernicterus (Bilirubin Encephalopathy) S/S and Tx

A

Lethargy, hypotonia, poor feeding, poor Moro, emesis Bulging fontanel, fever, paralysis, upward gaze, seizures

noticed after day 4 of life.

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97
Q

Kernicterus (Bilirubin Encephalopathy) Tx

A

Mild- Improve/ increase feeding. Exposure to sunlight

Mod- Phototherapy (Unconjugated only)

Severe- Exchange transfusion

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98
Q

HOV immunization indications

A

2-3 shot series given prior to exposure target age 11 as early as 9 (for 90% of Genital Warts)

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99
Q

Car seat rules for pediatrics

A

rear facing until the age of 2

Front facing booster car seat starting at age 2 until 4ft 9in ot 8-12 years of age.

< 13 y/o kids should be restrained in backseats.

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100
Q

unexpected death of an infant under 1 year of age that remains unexplained.

MC 2-4 mos Rare before weeks or after 6 mos

A

Sudden Infant Syndrome (SIDS)

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101
Q

Cause due to limited front leaning. First born and prematurity. Improves in 2-3 mos

No improvement by 4-6 months refer to craniofacial specialist.

A

Plagiocephaly

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102
Q

Premature closure of 1 or more cranial sutures.

Type of cranio deformity- Elective Sx @ 6 mos

A

Craniosynostosis

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103
Q

Feeding recommendations

A

0-6 mos solely breast or formula.

6 mos- start solids; Milk no earlier than 1 year o/a 2%

18-30 oz/day- No Honey or canned goods (Botulism)

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104
Q

Patterns of breast milk supply (mom)

A

Day 1 Colostrum

Day 2-4 Lactogenesis

Day 5 mIlk Present and fullness, leaking felt

Day 6 Brest should feel empty after feeding.

105
Q

Diapers for well hydrated baby in general

A

6-8 wet diapers at least 4 loose stools a day.

Normal to not stool for 1 week in otherwise notmal appearing baby.

106
Q

Vitamin D and Iron supplementation

A

Vitamin D if solely breastfeeding until 4 and begin Iron supplement at 4 mos

107
Q

Important mom screen.

A

Postnatal depression scale 1st 6mths of life

108
Q

toilet training begins at

A

2-3 Y/O

109
Q

Delayed eruption of teeth

A

Check TSH and Ca2+/ Hypothyroid, hypopituitarism, rickets. (1st Central Incisor L=6-+2 U=7+- 2

No fluoride tooth paste before 2 y.o begin dentist @ 1 for q 6 mos

110
Q

temporary GH deficiency and poor growth 2T psych abuse. In different psycho social environment –> thrive

A

Psychosocial Dwarfism

111
Q

GH Rec. mutations, elevated GH serum, and decreased IGF-1, that is Autosomal Recessive

Prominent forehead, hypoplastic nasal bridge, delayed dentition, sparse hair, blue sclera, osteoporosis

A

Laron Dwarfism

112
Q

Effected by GH Binding Protein (GHBP) –>tissues to produce ______ that attaches to cell membranes Rec.

—> productions of cellular changes and growth effects

A

Insulin Growth Factor-1

113
Q

Once chronic disease and familial short stature is R/O then offer GH testing for pts who are

A

short < 5th percentile growth rate for age

Height projection based on bone age is below target

114
Q

Normal routine labs for tests/ studies for failure to thrive

A

Karyotype (Down, Turner, Kleinfelter, IGF-1, IGF-BP3, Free thyroxine, Prolactine, Bone age, and cranial imaging

115
Q

Failure to thrive definition

A

Weight is < 3rd percentile or decreases crossing two major percentile lines.

Weight < 80% of median weight for height.

116
Q

Which FTT includes long standing malnutrition, chromosomal abn., congenital infx, teratogenic exp.

Has proportional weight , height/length, and head circumference.

A

Symmetric Type III

117
Q

Which FTT includes deficiency of linear growth where head circumference is spared

caused by mos of malnutrtition.

A

Stunting Type II

118
Q

Which FTT includes Deficiency in weight gain caused by malnutrition

A

Wasting Type I

119
Q

Causes of FTT

A

2/3 Nonorganic causes (Abuse, neglect, Ignorance)

1/3 Organic (75% GI fistula, atresia Dz process)

120
Q

COmplications of FTT

A

Malnutrition infection cycle

Refeeding syndrome- Phosphorus, K+, Ca+2, Mg+–> cardiac, pulmonary, and neuro life threatening problems

121
Q

Single copy of genes is sufficient to express condition

(Only one parent required for gene to be expressed) Heterozygous or Homozygous

A

Autosomal Dominant D/O

122
Q

Two copies of gene required to express a condition HomozygousX2 expression= 100% HeteroX2= 25%

A

Autosomal Recessive D/O

123
Q

Chromo that carries 500 genes males more commonly affected 50% chance of transmission.

No- father-son transmission. All daughters of affected man will be carriers

A

X-linked D/O

124
Q

Inc. RF of D/O w maternal age and inheritance. Decreased Moro Reflex, small head,

Up-slanting palpebral fissures, epicanthal folds, flat nasal bridge, small irregular ears, macroglossia, single palmar crease (simian), short mid 5th phalanx, short stubby fingers

A

Down-syndrome (Trisomy Chrmosome 21)

125
Q

Types of Trisomy 21

A

Translocation - Part of 21 get stuck on some other chromo before replication

Mosaicism Only some cells in body have 3 copies. Individuals often phenotypically normal

126
Q

Down syndrome Medical complications

A

Mental retardation- 100%

Hearing loss (SNHL)

cardiac anomalies (50%)- ASD or VSD –>Eisenmengers

Polycythemia (70%) Esophageal Atresia

127
Q

The MC drug induced congenital defect. 3rd MC cause of MR. *ADHD, Smooth philtrum, thin upper lip, Ptosis

Railroad tracks ears (underdeveloped), growth retardation. microcephaly.

A

Fetal Alcohol Syndrome (X-Linked D/O)

128
Q

Moderate to severe MR w above average memory for events and directions, *Autism, hyperarousal, handflap (sterotypy)

Oblong face, large testes,

A

Fragile X Syndrome

129
Q

Short Stature, webbed next, shield chest (wide spaced nipple), Aortic Coarctation HTN, Triangular face,

Normal except visual-spatial skills and superior verbal skills. Hypothyroidism, DM I and osteoporosis

A

Turner Syndrome (45 XO)

130
Q

Results from test deficiency. Hypogonadism, infertile, Gynecomastia 50%, diminished male pattern hair,

Tall feminine build (wide hips), low or normal IQ, Breast cancer 16-30 times, violent tendencies and MR. Prior to puberty, PT normal

A

Klinefelter Syndrome (XXY)

131
Q

AD mutations in Fibrillin 1 Gene on chromosome 15. Aortic Dissection, Reduced Upper to lower body ratio

Arm span exceeds height, arachnodactyly, Lens dislocation,

A

Marfan Syndrome (Autosomal Dominant)

132
Q

2nd MC autosomal trisomy. 95% abort in 1st trimester.

<10% survive to 1 yoa 5% live past 1 YOA

A

Trisomy 18

133
Q

3rd MC trisomy. usually fatal in 1st YOA 8% survive past 1. Aplasia cutis punched out scalp lesion

A

Trisomy 13

134
Q

Trisomy 21 Tx and Dx

A

Chromosomal analysis parents/child

Tx: Hearing screen q 6mos until 3 YOA
Echo always, Ophtho q year by 6 mos OA
Annual Celiac screen and TSH/CBC

135
Q

Fetal Alcohol Syndrome Dx and Tx

A

Early referral and prevention.

Multidisciplinary effort ADHD, Anxiety, and speech therapy. special ed

136
Q

Turner Syndrome Dx and Tx

A

Dx- Direct Karyotyping if features seen

Tx- Echo, Cardiac MRI, and ECG
Endocrinology GH estrogen start at 14 YOA

137
Q

Klinefelter Dx and Tx

A

Direct Karyotyping (Barr Body)

Tx- Testosterone replacement for hypogonadism

138
Q

Marfan Syndrome Dx or Tx

A

Dx confirmed w FISH studies

Tx- Beta Blockers, restrict vigorous exercise, yearly aortic sonography, aortic root replacement.

139
Q

newborn w hypothermia, acrocyanosis, resp. distress, large fontanelles, abd. distention, lethargy,

poor feeding, prolonged jaundice, constipation, dry skin, hoarse cry.

A

Congenital Hypothyroidism

Tx Levothryoxine (1st year started= good prognosis intellectual development is excellent

140
Q

Inborne errors of metabolism

A

Phenylketonuria, Tyrosinemia, homocystinuria, Maple Syrup Urine Dz,

141
Q

Consequence of severe liver Dz or response to ascorbic acid (Vitamin C). Accumulates metabolites

ID’d During neonatal screening

A

Tyrosinemia

142
Q

Severe liver dz (hypoalbuminemia hypoglycemia bleeding d/o), Renal tubular defect, Liver carcinoma

A

Type I- Tyrosinemia

Tx- Nitisinone (Phenylalanine and Tyrosine restrict diet)

143
Q

Hyperkeratosis of palms/soles and keratitis

A

Type II/III Tyrosinemia

Tx- (Phenylalanine and Tyrosine restrict diet)

144
Q

Autosomal recessive involves connective tissue, brain and vascular system. Cystathionine B-Synthase Deficiency–> inc. AA

Lens Dislocation, lon-slender extremeties, malar flushing, arachnodactyly, scoliosis, MR, thromboses

A

Homocystinuria

145
Q

Homocystinuria has increased____ and _____ AA in Connective tissue, brain and vascular system

A

Homocysteine and Methionine (Via Homocysteine reconversion)

146
Q

Homocystinuria Tx

A

1st Pyridoxine and Folic Acid

2nd Methionine Restricted diet, Cystine and Folic Aid

147
Q

Autosomal recessive profound CNS depression- poor feeding, vomiting, tachypnea, opisthonos, seizures

Rare except in Mennonites, manifests @ 1-4 weeks, Maple syrup odor urine

A

Maple syrup Urine Disease

148
Q

Maple syrup Urine Disease Dx and Tx

A

Dx: Elevated Leucine, isoleucine, valine, alloisoleucine

Tx: Leucine restricted diet, Dialysis, tx cerebral edema
Liver transplantation.

149
Q

Autosomal recessive uridyltransferase deficiency. when a neonate is fed milk–> Liver failure (hyperbili), coagulation and hypoglycemia.

Renal tubular dysfx (acidosis, glycosuria), cataracts, affected infants may die 1st week from E. Coli sepsis

A

Galactosemia

150
Q

Galactosemia Dx and Tx

A

Dx: Quick neonatal screening time

Tx: Eliminate dietary Galactose

151
Q

Galactosemia is caused by a deficiency of the enzyme

A

Galactose-1-phosphate uridyltransferase

152
Q

Increased fructose 1 phosphate leading to emesis, hypoglycemia, severe kidney and liver disease.

A

Hereditary Fructose intolerance

153
Q

Hereditary Fructose intolerance enzyme deficiency of?

Tx:

A

Fructose-1-Phosphate aldolase deficiency

Tx: Eliminate Fructose/sucrose from diet

154
Q

Neonatal red flags post delivery

A

Maternal Polyhydramnios

Delayed Meconium Passage

Abdominal distention (obstruction)

Perinatal vomiting

155
Q

cannot swallow amniotic fluid–> fluid cannot pass–> placenta cannot dispose–> polyhydramnios

Inc. Salivation, cough, choking, resp. distress, feeding worsens s/s, single umbilical artery.

A

Tracheoesophageal Fistula

156
Q

Tracheoesophageal Fistula closely associated with other presentations such as

A
Vertebral anomalies (70%)
Limb anomalies (70%)
Anal Atresia (50%)
Renal Anomalies (50%)
157
Q

Tracheoesophageal Fistula Dx-Tx

A

Failed OG tube pass

CXR OGT tube curled in esophageal pouch

Swallow study (Gastrografin contrast)

Tx=Sx (tube feeding until Sx)

158
Q

Post prandial progressive, progressive non-bilious. Projectile vomiting*, ravenously hungry-> lethargy
Malnourishment and dehydration–> failure to thrive

hypochloremic/hypokalemic metabolic alkalosis, hypertrophy pylorus (Olive), thickened pyloris (sausage)
Barium= String sign (constricted pyloris channel)

A

Pyloric stenosis

159
Q

Pyloric stenosis management

A

IV fluid resuscitation (D5W K+ Cl)

OG tube feeding until sx (Polyoromyotomy)

160
Q

Usually unilateral (L side). progressive severe resp. distress post delivery. Scaphoid abdomen Hollow abd.

BS in left chest. Bowel develops before lung. Bochdalek Foramen MC. Posterolateral diaphragm

A

Congenital Diaphragmatic Hernia

161
Q

Congenital Diaphragmatic Hernia Dx- Tx

A

Dx- X-ray (Bowels in L diaphragm

Tx- ET and respiratory support, OGT decompression
Sx- Definitive

162
Q

Typically easily reducible, appear at 6 mos and disappear at 1 yoa.

Sx if persists 4-5 yrs or progressively larger after age 1-2 yrs

A

Umbilical Hernia

163
Q

Intestines twist on themselves–> intestinal obstruction and mesenteric artery occlusion–> necrosis

Bilious vomiting 1st mth of life. 60%.. 40% infancy/child

A

Mid-gut volvulus

164
Q

Failure of the intestines to rotate normally during development. predisposed to midgut volvulus

A

Intestinal Malrotation

165
Q

Mid-gut volvulus/Malrotation Dx-TX

A

DX-Plain x-rays-Corkscrew effect volvulus

   barium enema (cecum in RUQ=malrotation)

TX- Fluid resuscitation, OG decompression, Laparotomy

166
Q

Polyhydromnios, failure at attempts to feed. Bilious vomiting, abdominal distention, jaundice

Double-bubble sign, long segments of bowel dilated

A

Intestinal Atresia

Tx- IV resuscitation, OGNG tube decompress, BSABX,Sx

167
Q

Split/open stomach DT abd. wall defect. lat. median plane. Does not involve umbilicus. DT ischemia of wall

Intestines are exposed and bathed in amniotic fluid. –> exudative covering peel. Atresia common. prolonged parenteral nutrition

A

Gastrochisis-

Tx: Sx correction

168
Q

DT growth of meso/Ecto-dermal of abd. wall. Intestines fail to return to the abd. during develop. –>

Bowel remains in the umbilical cord and covered by peritoneum and amniotic membranes. umbilical herniation

A

Omphalocele

169
Q

Omphalocele is associated with what anomalies

A

Bekwith-Wiedemann syndrome (Cardiac)

170
Q

This disorder is assoc. w Trisomy 21 (30%)
Malrotation (25%)
Anular Pancreas (20%)

A

Intestinal Atresia

171
Q

Omphalocele and Gastrochisis management

A

OG/NG decompress, IV fluids, Parenteral nutrition

cover w sterile dressing dec, heat/water loss

<2cm =immediate sx repair >2cm = staged repair

172
Q

May contain small patches of pancreatic/Gastric tissues. Ectopic gastric mucosa secretes acid–> ulcers

remnant omphalomesenteric duct and outpouching of the distal ileum. Mesenteric border of ileum.

A

Meckel’s Diverticulum

173
Q

Meckel’s Diverticulum rule of 2s

A
2% of population
2 ectopic mucosae Gastric/Pancreatic
Presents by 2 years
within 2 feet of cecum
2 inches long
174
Q

Massive painless GI bleeding, Ectopic gastric tissue–> mucosal ulceration of adjacent ileum. intuss/volvulus

Dx- Tx

A

Dx- Acid producing gastric mucosa in diverticulum
(technetium Scan)

Tx- Sx excision

175
Q

Congenital aganglionic megacolon. failure of ganglion cells to migrate to distal bowel–> absent motility/Obstr.

75%=Rectosigmoid. proximal dilatation to segment. 95% no passage of stool 1st 24hrs w distention/Bilious vomit

A

Hirschsprung Disease

176
Q

Hirschsprung Disease Dx and Tx

A

X-ray= Dialated proximal to affected segment

Barium enema= colonic impaction/Megacolon

DRE-Empty rectum/ Expulsion of retained stool

Tx- Colostomy of segment

177
Q

Ischemia 2DT immature GI system. Assoc. w premies <34weeks. Breast milk reduces incidence.

Abd. distention, feeding intolerance, rectal bleeding, emesis, DIC/schock, bluish abd. discoloration

A

Necrotizing Enterocolitis (NEC)

178
Q

Necrotizing Enterocolitis (NEC) Dx and Tx

A

Dx- Bowel loop thickening w Ileus and air-fluid levels.
Pneumatosis Intest.- Bacterial gas in SB lining

   Dilated bowel intrahepatic venous gas/pneumoperi

Tx: Total parenteral nutrition. BSABX, Sx, IV fluid resusc

179
Q

Telescoping of proximal bowel into downstream bowel
Peyer’s patches- Meckel’s Divert assoc., Rotavirus Ass.

Currant Jelly Stool*, RUQ pain sausage shaped mass, paroxysmal crampy pain, refuses feeding, draws legs

A

Intussusception

180
Q

Intussusception dx and Tx

A

Dx- Pneumatic or contrast enema under fluoroscopy.
(No Barium if perforation)

Tx- IV Fluid Resusc., NGT, Pneumatic or contrast
enema under fluoroscopy: Resect Bowel

181
Q

MC sx Emergency in children. Peak age 10-12 DT obst.
Less common <5yrs. Ruptures w/I 24 hrs.

RLQ pain (McBurney’s), voluntary guarding–>Rebound tenderness w rupture/peritonitis)

A

Appendicitis

182
Q

Alvarado/mantrels Rule.

A

Fever Nausea/Vomiting (x2)
Anorexia WBC shift.75% neutro
Rebound pain
Migration pain RLQ (x2)

183
Q

Appendicitis Dx-Tx

A

Dx: Ct w Contrast, US, CBC, CMP

Tx: Appendectomy and IV Abx

184
Q

Pt laying on his side and hip is extended is what test?

A

Psoas Sign

185
Q

Pt laying supine and hip is flexed and is rotated is what test?

A

Obturator sign

186
Q

Neonates may not have febrile response or may present with ___________

A

hypothermia

187
Q

older infants and children <5 may have fevers up to

A

105 degrees (exaggerated febrile response)

> 5 YOA and Temp 105 = serious illness

188
Q

fever over 14 days w/o ID etiology despite hx, PE and lab test or after 1wk of hospitalization.

MC cause=__________ Tx=Abx?

A

Fever of unknown origin

MC Cause= UTI

Tx <1mth=(Ampicillin/Gentamycin)
Tx 1-36 mos = Ceftriaxone or Cefataxime

189
Q

presence of bacteria in bloodstream may be primary or secondary to a focal infx

A

Bacteremia

190
Q

systemic response to infx w hyperthermia, tachycardia, tachypnea, shock (HYTN)

A

Sepsis

191
Q

higher risk for serious bacterial infx

A

Fever in infant < 3 mths

192
Q

All febrile infants < 4wks of age testing

A

hospitalization admission for empirical abx, pending culture Consider LP 3-36 mos w neuro signs. CXR if resp. , Stool WBC/Culture w NVD

193
Q

Fever 3 mos- 3 years

A

Most viral infx

194
Q

Antipyretics in children

A

Acetaminophen 10-15 mg/kg q 4-6 hrs

Ibuprofen 10mg/kg q 6 hrs (not before 6mos old)

NO ASA Reye’s Syndrome (Mitochondria destoyed in liver)

195
Q

Risk factors age <36 mos, Temp 102.2 , WBC > 15000, elevated ESR CRP

MC bacteria neonates=______
MC bacteria Infants/childrens= ______
MC Autoimmune =___________

A

Occult Bacteremia

Neonates= GBS
Infants/children= Strep pneumo, H influenza
Autoimmune= JRA (Juvenile RA)
196
Q

fever w impaired splenic fx and preperdin-dependent opsonization. Incr. risk for bacteremia in 1st 5 yo life

Osteomyelitis (salmonella) Temp>= 104 WBC> 30 Abx empirically, blood Cx, close outpatient F/U

A

Sickle cell anemia

197
Q

Systemic Inflammatory Response syndrome= two of the following

A

Temp < 98.6 or > 100.4
Tachypnea
HR > 90bpm or > 2 SD for age
WBC < 4K or >12K

198
Q

Definition of sepsis

A

SIRS due to infx

199
Q

Definition of severe sepsis

A

One or more end organ compromise

200
Q

Definition of septic shock

A

severe infx w hypotension and hypoperfusion

201
Q

Common to precede a URI s/s fever, headache, diplopia, vomiting. infants=poor feeding, restless,

bulging fontanelle, nuchal rigidity, seizures, sepsis, coma. fecal excretion persist for weeks w virus

A

Meningitis

202
Q

MC virus for meningitis

MC Bacteria for meningitis

A

Enterovirus/ Parechovirus- Herpes, Epstein, CMV

S. Pneumonieae or N. Meningitides (*Think Purpura)

203
Q

Meningitis Tx-

A

Infants <2 mos= Ceftriaxone or cefotaxime + Ampicillin to cover listeria

1 month-13 yrs= Ceftriaxone or cefotaxime + Vancomyci

N. Meningitidis = 7 days
N influenza= 10 days (Dexamethasone)
S pneumoniae = 14 days

204
Q

kernig and Brudzynski

A

Kernig’s= Flat neck and cannot extend knee

Brudzinski’s= Flex neck–> flexion of knees

205
Q

Complications of Meningitis

A

SIADH (ADH–> Water retention and Hearing loss

206
Q

Sore throat, fever, HA then lethargy behavior changes, neuro deficits, seizures common, macule/papule rash

usually resolves days-2-3 wks. MC virus entero/arbo/herpes (east/West Equine west nile)

A

Encephalitis (inflammation of Brain)

207
Q

Lumbar puncture

A

Bacterial - Glucose < 40, Incr. pressure, incr. proteins and PMNs

Virus- Normal Glucose, Normal or slight pressure, Mononuclear cells

Fungal- Glucose <50, Incr. pressure, Mononuclear cells

208
Q

Encephalitis Varicella zoster tx
CMV Tx

                  Bacterial
A

Varicella- IV Acyclovir
CMV- Ganciclovir

Doxy, Azithromycin, clarithromycin, erythromycin

209
Q

Highly contagious. Paramyxovirus infx of URI. Infectious 1-2 days before onset. Secretion, Blood, UA

S/S 5 days prior, 4 days after rash. High fever: *Cough, Coryza, Conjunctivitis, Koplik spots. Macul,niar Rash head-caudal over 24 hrs. C-LAD, splenomegaly, AOM (MC), liver involvement adults

A

Measles (Rubeola) (Hard Measles)

210
Q

Measles Dx-Tx

A

Leukopenia, PCR IgM Ab

Tx-Supportive hydration and Antipyretics w/I 72 hrs
vaccinate MMR and Immunoglobulins w/I 6 days
Vitamin A for 2 days

211
Q

Invades resp. epithelium. spread through nasopharyngeal secretions. Togavirus in springtime

Most Contagious 2 days prior until 5-7 days post rash.
LAD, pharyngitis, conjunctivitis, anorexia, HA, Erythematous macule papule face to body lasts 3 days

Rose-colored spots on palate, polyarthritis (hands adults) Paresthesias, tendinitis

A

Rubella (German Measles)

212
Q

Rose-colored spots on palate AKA

Rubella Tx

A

Forschheimer Spots

Hydration and antipyretics

213
Q

Rubella German Measles complications

A

In Utero= Blueberry muffin baby (No vaccine in Prego)

Deafness, Cataracts, congenital heart dz (infant sheds dz >12 mos)

214
Q

Parvo Virus B19 high affinity to RBC–> anemias SC, Thalasemias, spherocytosis) resp. secret and transfuse.

3 stages 1. Slapped cheek 2. maculopapular rash 3. Stage 2 rash fades central clearing occurs “lacy rash” desquamation w bathing exercise, rubbing stress

A

Fifth’s Dz (Erythema Infectiosum)

215
Q

Fifth’s Dz (Erythema Infectiosum) Dx-Tx

A

Dx-Hx-N-PE , IgM, PCR

Tx- Transfusions, Hydration/Antipyretics
IV Immunoglobulin w anemia and immunocomp

216
Q

Infects mononuclear cells. Herpes HHV6-7. abrupt fever lasts 3-5 days. GI S/S, URI, red TMs, cough

High fever 3-5 days w/o findings. Defervescence of fever following rash. complications=hemophagocytosis

A

Roseola (exanthem Subitum)

217
Q

Infects via conjunctivae or resp. replicates in nasopharynx and URT. contagious until lesions cursted

Peak age 10-14 YOA. late winter all lesion in same stage. pruritic rash teardrop vesicles–> crust heal.
Mortality incr. >20 YOA

A

Varicella

218
Q

Varicella complications

A

Skin infx- Strep/staph
Pneumonia’
Reye’s Syndrome (ASA)

219
Q

Varicella Tx-

A

Symptomatic-Hydrate antipyretics

Valacyclovir-acyclovir (Not healthy) only severe risk

VZIG- immunoglobulin Neonatal ASAP

220
Q

Acute neuritis w malaise and fever. 75% Occur>45 YOA recurrence of latent Dz

post herpetic neuralgia >1 month. CNV involvement branch or CN VII =facial paralysis Ramsay hunt w ear canal involved.

A

Zoster

221
Q

Mouth pain, sore throat, refusal to eat/drink, febrile, NVD, abd. pain, 2-3 day fever Rt to school

oral, palm and sole ulcers. Dz lasts 10-14 days. virus shed for wks to months

A

Hand, foot, mouth Dz

222
Q

Phase in adolescence where abstract thinking, experimentation, fantasy takes place. Identity concern

Strive for independence, high risk behavior.

A

Middle Adolescence Psych Dev

223
Q

Phase in adolescence where things are concrete and gives little insight to questions.

Concern w normalcy and body changes. focused on present. Ambivalent to independence

A

Early Adolescence Psych Dev.

224
Q

Phase in adolescence where there is formal concern for future: marriage occupation. commit to sex partners

separation anxiety from formal development stages.

A

Late adolescence Psych Dev

225
Q

Interview pneumonic for adolescents

A

Home/ friends Diet
Education Sex
Alcohol Suicide/depression
Drugs

226
Q

first sign of secondary sexual characteristics

A

Females: Thelarche (Breast development)

Males: Testicular enlargement

227
Q

Onset of menarche is

A

12.2 -12.9 YOA African American 1st

Obesity =Early thelarche peripheral Estrogen

228
Q

Growth spurt

A

females = 1 year after thelarche

Males= 10-16

229
Q

Tanner stage 1

A

<10 yo pre-pubertal no glandular tissue

<9 yoa penis < 3 cm

230
Q

Tanner stage 2

A

10-11.5 yoa female= Breast buds under areola
9- 11.5male/Female= Pubic hair straight and darkens
Testes enlarge

231
Q

Tanner stages 3

A

11.5-13 yoa Female= Breast enlarge outside of areola

11-13 Male= Penis lengthens, pubic hair dark and curls

232
Q

Tanner stage 4

A

female 13-15 yoa Areola protrudes, hair completely

Male 12.5-15 yoa Penis widens and hair fills completely

233
Q

Tanner stage 5

A

female 14+ = Areola rejoins breast
hair fills in completely w thigh

Male= Developed complete w hair to medial thigh

234
Q

secondary sex characteristics < 8yoa FEM <9 MALE
Hypothalamic activation prematurely. Pituitary-Gonadal

GnRH MC, or familial. tumors or Brain or pituitary controlled any condition affecting CNS** if thelarche/menarche or testicular growth** dominant

A

Precocious Puberty Central

235
Q

secondary sex characteristics < 8yoa FEM <9 MALE
Gonad or adrenal tumors HCG secreting tumors pineal gland or hepatoblastoma. MRI adrenals

Congenital adrenal Hyperplasia males–> early penile growth. * Pubic/axilla hair, body odor, acne dominant

A

Precocious Puberty peripheral

236
Q

MC cause of GnRH independent precocious puberty MC (girls > boys) ovarian hyperfunction–> gonadarche–> episodic estrogen secretion

hamartoma (B>G) non malignant that produces GnRH. bony DO w Café au-lait spots, Polyostotic fibrous dysplasia

A

McCune Albright Syndrome

237
Q

Precocious Dx and Tx

A

Dx- Ct/MRI for central LH/FSH (Incr. in central low in Peripheral)

Tx- GnRH Analogues Suppress pituitary activity
(cease menses and growth velocity dec.) Testolactone
Peripheral= No GnRH response
Antiandrogens- blocks estrogen synth.
Antiestrogens - Tamoxifen

238
Q

Untreated precocious puberty leads to

A

Short stature, closure of epiphyses

Recurrent ovarian cysts and hypopituitarism

239
Q

No pubertal development by age 13= Fem 14=Mal
MC lack response to hypothalamic pituitary-gonad axis

Hypogonadotropic/hypergonadotropic?

A

Delayed onset puberty

240
Q

Low FSH/LH secretion delayed onset puberty
Malnutrition, stress, or hypothyroidism

idiopathic or tumors

A

Hypogonadotropic (It is the Hypothalamus-Pituitary)

241
Q

High FSH/LH secretion delayed onset puberty

Turner syndrome, Klinefelter, Androgen Insensitivity, PCOS

A

Hypergonadotropic (It is the Gonads)

242
Q

Female Athlete Triad

A

Eating disorder, irregular periods, low bone density

243
Q

Dx Hypogonadotropic

A

Low FSH/LH

TSH, Prolactin, & head MRI

244
Q

DX Hypergonadotropic

A

Karyotype (turner’s or Klinefelter’s)

245
Q

Breast mass in young

A

US better for dense breast

Fibroedema or cyst MC

246
Q

Leukorrhea peripubertal girls Tanner stage III clear w no odor or pruritis

A

Normal variant from ovarian estrogen stimulation of uterus and vagina (culture w/o speculum)

247
Q

Menarche onset (MC complaint in adolescence )

A

2-3 years after thelarche irregular in 2-5 yrs persist= normal

after 1 tear of regular cycles, irregular bleeding= abnormality

248
Q

Gynecomastia in Males

A

45-75% of boys. < 3cm= benign

If large, fixed or nipple DC= evaluate
W liver dz= Klinefelter’s

Refer >17 yoa (Azoles and marijuana)

249
Q

Primary Amenorrhea

A
  • 16 yoa with thelarche and no period yet= bad

- 14 yoa with no thelarche or period = bad

250
Q

Secondary Amenorrhea

A

cessation of menses > 3 consecutive mos post menarche

251
Q

MC causes of Secondary Amenorrhea

A

pregnancy, anorexia, stress, PCOS, Low FSH/LH estradiol.

R/O thyroid, prolactinoma

252
Q

Hirsutism, moderate-severe acne, menstrual irregularities, obesity and insulin resistance, polycystic ovaries (US)

2 of the following- Infrequent menses, hyperandrogenism, Polycystic morphology ovary US

A

Polycystic Ovary Syndrome

253
Q

Amenorrhea Tx

A

Combo hormonal contraceptive

Hypothalamic low estrogen (Estrogen/progesterone combo)

PCOS- weight loss, exercise, progesterone withdrawal, combo estrogen progesterone.

254
Q

Treatment for hirsutism

A

spironolactone

metformin may restore ovulatory cycles w insulin resistant pts

255
Q

pelvic pain in absence of pelvic pathology. 1-3 years post menarche, MC complaint in young women

inc. incidence to age 24. incr degeneration sof endometrium (Progest. declines)–> Inc uterine contract.–> ischmenmia and uterine pressure

A

Primary Dysmenorrhea

256
Q

Menstrual pain assoc. w pelvic pathology MC caused by endometriosis or PID.

If outflow tract obstructed–> severe endometriosis soon after menarche

A

Secondary Dysmenorrhea

257
Q

DX Tx of Dysmenorrhea

A

Outlet obstruction w US, PCOS w US, lLaparoscopy to dx endometriosis and PID Dx if Tx fails (Failed Therapy)

Tx= NSAIDS 2-3 days
NSAID Fail - Combo Estrogen/Progesterone (Depo or Implant) > 4 mos persist re-evaluate

258
Q

Definition of obesity

A

BMI weight kg/height in meters=BMI - >95th%

Child < 7 yoa = maintain weight not loss
Child >7 yoa = 1 lb/mth until <85%