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Peds > BRS Ch. 1-5 > Flashcards

BRS Ch. 1-5 Flashcards

1
Q

What are four red flags in motor development?

A

Persistent fisting beyond 3 months of age
Early rolling over/early pulling to a stand
Spontaneous postures
Early hand dominance <18 months

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2
Q

What is the best indicator of intellectual potential?

A

Language

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3
Q

What are four red flags in cognitive development?

A

Delayed language and problem solving: mental retardation
Delayed language only: hearing/communication disorder
Delayed problem-solving only: visual/fine motor disorder
Discrepancy between language and problem solving: learning disability

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4
Q

What are two main motor deficits?

A 
Cerebral palsy (spastic = increased tone)
Extrapyramidal cerebral palsy (writhing, oral involvement)
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5
Q

How is mental retardation defined and diagnosed?

A
  • signficantly subaverage general intellectual functioning with deficits in adaptive behavior
  • use the Wechsler Intelligence Scale or IQ to classify (mild, moderate, severe, profound)
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6
Q

How is ADHD managed?

A

Demystification, classroom modifications, educational assistance, counseling
Medications: stimulants (increased NE/DA transmission) include methylphenidate, amphetamines

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7
Q

How is colic defined and managed?

A
  • crying that lasts >3 hours and occurs >3 days per week, occurs in healthy infants, 2-4wk –> 3-4mo
  • treat underlying condition, reassure parents, recommend comfornt measures
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8
Q

What are four characteristics of adolescent growth?

A
  1. ) average duration = 2-3 years
  2. ) controlled by growth hormone
  3. ) 50% of body weight, 25% of body height
  4. ) occurs 18-24 months earlier in females than males
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9
Q

Male Tanner staging

A

Stage 1: pre-adolescent, no pubic hair, prepubertal testes
Stage 2: larger testes, sparse/long/downy hair
Stage 3: larger testes, penis length enlarged, darker/coarser/curlier hair
Stage 4: darkening of scrotal skin, penis width/length increases, glans develops, coarse & curly pubic hair
Stage 5: adult penis and testes, adult-type pubic hair that spreads to medial surface of thighs

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10
Q

Female breast Tanner staging

A

Stage 1: preadolescent
Stage 2: elevation of breast and nipple as small projections
Stage 3: enlargement of breast, no separation of areola and breast, no separation of areola and breast
Stage 4: areola and nipple project to form secondary mound
Stage 5: only nipple projects, areola usually recedes to contour of breast, adult breast size

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11
Q

Female pubic hair Tanner staging

A

Stage 1: no pubic hair
Stage 2: sparse/long/downy hair along labia
Stage 3: darker/coarser/curlier hair
Stage 4: coarse/curly adult type hair
Stage 5: adult-type hair spreading to medial surface of thighs

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12
Q

What are three vaccines that must be administered or confirmed during adolescence?

A
  1. ) Tetanus/diphtheria booster: between 11-12 and every 10 years after
  2. ) MMR/HepB
  3. ) Varicella: if the child hasn’t already received or hasn’t had chicken pox
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13
Q

What are four specific criteria for anorexia nervosa?

A
  1. ) refusal to maintain body weight at normal leves. Body weight = 15% below ideal
  2. ) intense fear of weight gain
  3. ) disturbed body image
  4. ) absence of three consecutive menstrual cycles
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14
Q

What are five specific criteria for bulimia nervosa?

A
  1. ) recurrent episodes of binge eaing at least twice a week for 3 months
  2. ) lack of control over eating plus anxiety, guilt or sadness after each binge
  3. ) purging
  4. ) fasting, rigorous exercise, diet pills
  5. ) disturbed body image
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15
Q

What are three causes of adolescent vagiinits?

A
  1. ) Trichomonas vaginalis - yellow-green discharge, friable/strawberry cervix, dx w/ positive culture, tx: metronidazole
  2. ) bacterial vaginosis (most common) - gray/white discharge, reduction of lactobacilli, dx: increased odor with 10% KOH, clue cells on microscopy, tx: metronidazole, topical therapy
  3. ) candidal vulvovaginitis - severe itching, white curd-like discharge, dx: fungal hypahe on wet mount, positive yeast culture, tx: fluconazole or anti-yeast topicals
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16
Q

What are two causes of adolescent cervicitis?

A
  1. ) Chlamydia

2. ) Gonorrhea (often found with chlamydia)

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17
Q

How is PID diagnosed?

A
  1. ) All of the following: lower ab pain, cervical motion tenderness, adnexal tenderness
  2. ) One of the following: fever, WBC count, inflammatory pelvic mass, elevated ESR, lab evidence of infection
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18
Q

How is dysmenorrhea diagnosed and treated?

A

Dx: pain associated with menstrual flow, primary is caused by increased prostaglandins leading to excessive uterine contractions, tx: PG inhibs (NSAIDs)

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19
Q

How is amenorrhea diagnosed and treated?

A

Dx: absence of menstrual flow, either late onset or stoppage, rule out pregnancy and thyroid disorders, high FSH/LH = ovarian failure, low FSH/LH = hypothalamic/pituitary failure

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20
Q

What are 6 types of abnormal vaginal bleeding?

A

dysfunctional uterine bleeding - frequent, irregular periods with painless bleeding
polymenorrhea - regular but frequent (35 days

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21
Q

What are three painful scrotal masses?

A
  1. ) Torsion of the spermatic cord
  2. ) Torsion of testicular appendage
  3. ) Epididymitis
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22
Q

What are four painless scrotal masses?

A
  1. ) testicular neoplasms
  2. ) indirect inguinal hernia
  3. ) hydroceles
  4. ) varicoceles
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23
Q

What does an Apgar score measure?

A

Low score indicates need for resuscitation until score of 7 is acheived
Five criteria: HR, RR, muscle tone, reflex irritability, color

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24
Q

What are the 5 T’s of cyanotic congenital heart disease?

A
  1. ) Tetralogy of Fallot
  2. ) Transposition of the great vessels
  3. ) Truncus arteriosus
  4. ) Tricuspid atresia
  5. ) Total anomalous pulmonary venous connection
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25
Q

What is omphalocele?

A

A true hernial sac where the abdominal contents are covered with the peritoneal sac

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26
Q

What is gastroschisis?

A

Congenital fissure of the anterior abdominal in the right periumbilical area, allowing for colonic herniation

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27
Q

What are common causes of intestinal obstruction in newborns?

A

Intestinal atresia
Meconium ileus
Volvulus
Hirschsprung’s disease

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28
Q

What are causes of hypoglycemia in newborns?

A

Hyperinsulinemia, diminished glucose production or substrate deficits, inborn errors of metabolism

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29
Q

What are causes of polycythemia in newborns?

A

Increased EPO secretion due to placental insufficiency, hypoxemia, delayed cord clamping

30
Q

What are two maternal serum markers used in prenatal diagnosis?

A 
alpha-fetoprotein: low AFP --> overestimated gestational age, tisomies 21 and 18, intrauterine growth retardation
triple marker (AFP, unconjugated estriol, B-hCG): low, low, high = Down syndrome
31
Q

A genetic defect in chromosome 15 (codes for fibrillin) leads to what syndrome?

A

Marfan’s –> ocular, cardiovascular, skeletal systems. DDx: homocystinuria.

32
Q

There are two main imprinting disorders. What are they?

A

Absence of paternal 15 = Prader-Willi (paternal=Prader), craniofacial, growth, neurologic, hypogonadism
Absence of maternal 15 = Angelman (mom is an angel), happy puppet syndrome

33
Q

A deletion at chromosome 22q11 leads to what syndrome?

A

DiGeorge. CATCH-22 = cardiac anomaly, abnormal facies, thymic hypoplasia, cleft palate, hypocalcemia, chr. 22

34
Q

What syndrome is characterized by the three symptoms of hyperextensible joints, fragile vessels and loose skin?

A

Ehlers-Danlos syndrome, defective Type V collagen. Also look for loose, fragile skin.

35
Q

What disease arises from a defect in Type I collagen?

A

Osteogenesis imperfecta. Look for blue sclera, fragile bones, yellow/blue-gray teeth, easy bruising

36
Q

What syndrome is associated with a cocktail party personality?

A

Williams syndrome, deletion on Chr 7. Features: Elfin facies, MR, aortic stenosis, hyperCa,

37
Q

Why does Trisomy 21 (Down syndrome) increase with maternal age?

A

The increased occurence fo nondisjunction.

38
Q

What are the epidemiologies of Trisomy 21 and Trisomy 18?

A

Trisomy 21 is more common in males, trisomy 18 is much more common in females.

39
Q

Why is Trisomy 13 not commonly seen in patients?

A

It is extremely fatal, with death usually occuring in the first month of life due holoprosencephaly, mental retardation, micropthalmia and cleft lip and palate.

40
Q

What should be considered in any patient with delayed puberty?

A

Turner syndrome (only one X chromosome): short stature, webbed neck, shield chest, DELAYED PUBERTY

41
Q

What is the most common cause of inherited mental retardation?

A

Fragile X syndrome (CGG repeats on X chromosome). More severe and common in males. Large ears and testes

42
Q

What is the chromosomal finding in Klinefelter’s syndrome?

A

XXY

43
Q

How are skeletal dysplasias classified?

A

By location. Rhizomelia = proximal long bones, meso- = medial long bone, acro = distal

44
Q

What disorder is caused by a mutation in the FGR-R3 gene?

A

Achondroplasia.
Autosomal dominant, but mostly sporadic. Increases with paternal age.
Look for megalencephaly, foramen magnum stenosis, lumbar kyphosis –> lordosis, rhizomelic shortening

45
Q

What cardiac defect is most common in Fetal Alcohol Syndrome?

A

Ventricular septal defects. Also, think about SGA, MR, FTT

46
Q

What are three features that suggest an etiology of inborn error of metabolism?

A

Acute illness w/o response to therapy, unexplained neurological symptoms, lab values inconsistent with presentation
NOTE: IEMs are easily confused with sepsis, which is more common

47
Q

What defect in amino acid metabolism is similar to Marfan’s syndrome?

A

Homocystinuria. Look for Marfanoid body, hypercoagulability, CV abnormalities, scoliosis, DD. Dx: increased methionine in urine and plasma

48
Q

A newborn with hepatomegaly and hypoglycemia should be considered for what defect of carbohydrate metabolism?

A

Galactosemia. Look for sx after cow’s or breast milk, vomiting, diarrhea, FTT. Cataracts. RTA.

49
Q

What two signs should suggest a mitochondrial disorder?

A

A common disease has an abnormal presentation.

A disease involves three or more organ systems.

50
Q

Ashekenazi Jews are at a higher risk of what lysosomal storage disorder?

A

Tay-Sachs disease. AR. Hyperacusis, macrocephaly, cherry-red macula.

51
Q

What enzyme is missing in Niemann-Pick disease?

A

Sphingomyelinase deficiency. Progressive neurodegeneration, ataxia, seizures, etc.

52
Q

What is the main difference between the two mucopolysaccharidoses, Hurler and Hunter syndrome?

A

Corneal involvement. Hurler syndrome has corneal involvement. Hunters need sharp vision and an intact cornea, so Hunter syndrome has no corneal involvement.

53
Q

What is the most obvious clinical feature of the most common disorder of metal, specifically copper, metabolism?

A

Kayser-Fleischer rings in ther peripheral cornea. Also look for neurologic findings and hepatic dysfunction

54
Q

Under what category of anemia does folic acid deficiency fall?

A

Macrocytic anemia. Vitamin B12 deficiency is also a macrocytic anemia.

55
Q

What are possible etiologies of normocytic and normochromic anemia?

A

Hemolytic (hereditary spherocytosis, hereditary elliptocytosis, PK deficiency, G6PD deficiency)
Defects extrinsic to RBC (autoimmune hemolytic [Coombs test!], ABO/Rh incompatibilities, microangiopathic anemia)
Sickle cell hemoglobinopathies (tx with hydroxyurea)

56
Q

Why are infections common in patients with sickle cell anemia?

A

Decreased splenic function –> increased of risk of infection w/ encapsulated bacteria
Osteomyelitis by staphylococcus or Salmonella

57
Q

Faconi anemia present with pancytopenia and what other clinical features?

A

Fanconi syndrome = pancytopenia

  • skeletal abnormalities (NO THUMB!)
  • skin hyperpigmentation
  • renal abnormalities
58
Q

What is the most common cause of elevated Hgb or Hct?

A

Dehydration

Also consider primary and secondary polycythemias

59
Q

What is the classic presentation of Hemophilia A (X-linked bleeding disorder)?

A

Hemarthroses and deep soft-tissue bleeding

Look for prolonged aPTT, normal PT/bleeding time/plt count/plt function, low VIII activity

60
Q

What is the most common hereditary bleeding disorder?

A

vWF disease, Type 1 (quantitative deficiences in vWF)
Look for epistaxis, menorrhagia, bruising, bleeding (NO HEMARTHROSES)
Prolonged aPTT, test for vWF

61
Q

What can be causes of Vitamin K deficiency (and thus decreased II, VII, IX, X, Prot C and S)?

A

Pancreatic insufficiency, biliary obstruction, prolonged diarrhea
Broad-spectrum antibiotics, other medications

62
Q

What mediates the vasculitiis in HSP (Henoch-Schonlein purpura)?

A

Ig-A, presents with palpable purpura on lower extremities/buttocks, renal insufficiency, arthritis, ab pain.
Look for increased serum IgA, normal plt counts

63
Q

What is the most common acquired platelet abnormality?

A

ITP (idiopathic thrombocytopenic purpura), viral/drug-induced/idiopathic (cross-reactive antibodies)
Tx: IVIG

64
Q

Deficiencies in what proteins/factors will cause hypercoagulability?

A

Proteins C and S, anti-thrombin III, factor V

Lok for purpura fulminans (fever, shock, rapidly spreading skin bleeding, intravascular thrombosis)

65
Q

What is the most common childhood cancer?

A

Acute lymphocytic leukemia. Look for fever, bone/joint pain, HSM, lympho, pallor/bruising
Tests reveal anemia and thrombocytopenia, WBCs variable
Tx: induction (kill cancer cells), consolidation (protect CNS), maintenance (during remission)

66
Q

Down syndrome patients are at an increased risk for what cancer?

A

Acute Myeloblastic Leukemia.

More common CNS involvement. Fever, HSM, bruising. Auer rods on biopsy.

67
Q

The Reed-Sternberg cell is the hallmark histologic feature for what cancer?

A

Hodgkin’s lymphoma.

68
Q

Intussusception can be caused by what cancer?

A

Non-Hodgkin’s Lymphoma

69
Q

What embryonic germ layer does a rhabdomyosarcoma arise from?

A

Mesenchymal layer (same as skeletal muscle)

70
Q

What tumors are involved with Beckwith-Wiedemann syndrome?

A

Hepatoblastoma, Wilm’s tumor, pancreatoblastoma

Peds (6 decks)

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