Random Peds Stuff 2 Flashcards

1
Q

numerous fractures in various stages of healing, presence of blue sclerae, short stature

A

osteogenesis imperfecta

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2
Q

fine, pink, sandpaper-like rash following episode of pharyngitis. Rash is in flexural areas and desquamates (shedding of top layer) but does not cause blistering or a positive nikolsky sign.

A

scarlet fever - group A strep

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3
Q

What is nikolsky sign?

A

The detachment of a superficial layer of skin when gentle pressure is applied

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4
Q

What are the most common bleeding disorders in children?

A

hemophilias and von willebrand disease

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5
Q

What are the findings in the CSF for viral meningitis? gram stain?

A
  • WBCs elevated (pleocytosis) w/ lymphocytic predominance
  • protein level normal to slightly elevated
  • glucose normal
  • gram stain shows no organisms
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6
Q

18 month old girl with rash and blisters. Fever that responded well to ibuprofen. Erythema and peeling skin on mouth, axillae, inguinal areas. Superficial flaccid bullae and an erythematous rash. Slight focal pressure results in sloughing of the top layer of skin.

A

staphylococcal scalded skin syndrome

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7
Q

What is viral meningitis ? What are the most likely viruses causing it?

A

Self-limited inflammation of the leptomeninges caused by viral infection. 90% of cases are caused by echovirus and coxsackievirus

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8
Q

well demarcated, warm, tender area of erythema. No diffuse rash or shedding.

A

erysipelas - Group A strep

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9
Q

Injury pattern of non accidental trauma

A

1) subdural & epidural hematomas
2) retinal hemorrhage
3) frenulum treas and gingival lesions
4) linear type immersion burns
5) long bone fractures in the humerus or femur

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10
Q

patient appearing well, blister confined to primary area of infection. Ruptured blister leaves a honey-colored crust

A

bullous impetigo - S. aureus

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11
Q

What test is used to diagnosis osteogenesis imperfecta?

A

type 1 collagen assay

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12
Q

What is the treatment for viral meningitis?

A

supportive as symptoms will resolve within 7-10 days

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13
Q

pathogenesis of SSSS

A

infection of staph aureus that produces exfoliative toxins, which disrupt keratinocyte adhesion in the superficial epidermis.

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14
Q

Describe the clinical picture of viral meningitis

A

viral prodrome, consitutional and upper respiratory symptoms with low grade fever. Over the next 36-48 hours patient develops high fevers, headache, irritability and nuchal rigidity. Focal neurological findings are usually not seen

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15
Q

targetoid lesions with central bullae. Nikolsky sign negative.

A

erythema multiforme

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16
Q

If a patient had bacterial meningitis, what would the CSF results say? gram stain?

A
  • increased WBC with predominant neutrophils
  • increased protein
  • decreased glucose
  • gram stain will show bacteria
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17
Q

What are patients with sickle cell disease most at risk for?

A

Severe anemia

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18
Q

Aplastic crisis

A

A sudden drop in RBC production (erythropoiesis)

  • acute drop in hemoglobin (severe, often <6g/dL)
  • low reticulocyte count (<1%)
  • NO splenomegly
  • normal WBC and platelet count
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19
Q

What is the most common cause of aplastic crisis?

A

parvovirus B19

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20
Q

What is the treatment of aplastic crisis?

A

blood transfusions

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21
Q

What are the acute severe anemia crisis that can in occur in a patient with sickle cell disease?

A

1) aplastic crisis

2) splenic sequestration crisis

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22
Q

What causes splenic sequestration?

A

vasoocclusion and pooling of red blood cells in the spleen.

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23
Q

What are the features in splenic sequestration crisis?

A
  • increased reticulocytes
  • splenic vaso-occlusion –> rapidly enlarging spleen
  • occurs in children prior to autosplenectomy
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24
Q

What are some triggers for generalized seizures

A
  • fever
  • hypoglycemia
  • sleep deprivation
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25
Q

A previously healthy 6 year old boy with sudden appearance of petechiae over trunk and extremities. 3 weeks ago had an URTI that resolves. Vital signs normal. Cooperative well-appearing. Platelet count is 40 000/microL

A

immune thrombocytopenia (ITP)

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26
Q

What are the laboratory findings in immune thrombocytopenia

A
  • isolated thrombocytopenia < 100 000

- peripheral smear with megakaryocytes

27
Q

What age group is ITP most common

A

2-5 years

28
Q

what is the pathogenesis of ITP

A

antibodies that bind to platelets and subsequent destruction of antibody-platelet complexes in spleen.

29
Q

clinical presentation of ITP

A
  • asymptomatic purpura and petechiae
  • if severe, mucosal bleeding,
  • antecedent viral infection
  • low platelets <100 000
30
Q

What is the treatment for ITP patients?

A

1) observation

2) IVIG or glucocorticoids if bleeding

31
Q

How long would a patient wait before spontaneous recovery from ITP occur?

A

~6 months

32
Q

What is the most common congenital cause of aplastic anemia in children?

A

Fanconi anemia

33
Q

What is the inheritance pattern for faconi anemia

A

autosomal recessive or x-linked

34
Q

What are the clinical manifestations of Faconi Anemia?

A

1) bone marrow –> aplastic anemia, and progressive bone marrow failure
2) appearance –> short stature, microcephaly, abnormal thumbs, hypogonadism
3) skin –> hypopigmented/hyperpigmented areas, cafe au last spots and large freckles
4) eyes/ears –> strabismus, low-set ears, middle ear abnormalities

35
Q

How is the diagnosis of faconi anemia made?

A

chromosomal breaks on genetic analysis

36
Q

What is the definitive treatment for aplastic anemia?

A

hematopoietic stem cell transplantation

37
Q

What is aplastic anemia ?

A

when the bone marrow and the hematopoietic stem cells are damaged. Usually causes pancytopenia

38
Q

What does TORCH stand for?

A
toxoplasmosis
other infections 
rubella
cytomegalovirus 
herpes simplex
39
Q

Why are the TORCH infections significant?

A

They are known to cause significant neonatal and perinatal morbidity and mortality. Normally lead to growth restriction.

40
Q

What are the risk factors for respiratory distress syndrome?

A

1) prematurity (most important)
2) maternal diabetes

Others

  • male
  • perinatal asphyxia
  • C-section w/o labor
41
Q

What is seen on a chest X-ray of a patient with RDS?

A

ground glass opacities and air bronchograms

42
Q

How does RDS present?

A

tachypnea, retractions, grunting, nasal flaring, cyanosis at birth

43
Q

How is RDS treated?

A

1) antenatal prevention with corticosteroids
2) postnatal treatment with exogenous surfactant
3) respiratory support

44
Q

How is tourette disorder treated?

A
  • antipsychotics (risperidone)
  • alpha-2 adrenergic receptor agonists (clonidine, guanfacine)
  • behavioural therapy (habit reversal therapy)
45
Q

What is the inheritance pattern for klaxon’s syndrome?

A

x-linked recessive

46
Q

what is the pathophysiology of kallmann syndrome?

A

disorder of fetal gonadotropin-releasing hormone (GnRH) and olfactory neurons resulting in hypogonadotropic hypogonadism and rhinencephalon hypoplasia

47
Q

What is the karyotype of a female pt with kallman syndrome likely to have?

A

46 XX

48
Q

What are the clinical features of a pt with kallmann syndrome?

A
  • short stature
  • delayed or absent puberty
  • decreased sense of smell (anosmia)
49
Q

what is the management for kallman syndrome?

A

early diagnosis and hormonal treatment to facilitate development of secondary sex characteristics, bone development, muscle mass and improve fertility.

50
Q

Typical presentation of laryngotracheitis (croup)

A
  • age 6-3 years

- “barky” coughing, stridor, hoarse voice

51
Q

Typical presentation of epiglottis

A
  • unvaccinated

- Sore troat, dysphagia, drooling, “tripod” positioning

52
Q

Typical presentation of bronchiolitis

A
  • Age <2 years

- Wheezing, coughing

53
Q

What is the classic pathogen in croup?

A

parainfluenza

54
Q

what is the classic pathogen in epiglottitis ?

A

Haemophilus influenzae

55
Q

what is the classing pathogen in bronchiolitis?

A

RSV

56
Q

What is seen on chest x-ray for a patient with croup?

A

steeple sign

57
Q

What is the treatment for croup?

A
  • corticosteroids (dexamethasone)

- nebulized racemic epinephrine for patients with stridor at rest

58
Q

What is the most common complication of sickle cell trait?

A

painless hematuria due to sickling in the renal medulla. Isothernuria (impairment in concentrating ability) is also common

59
Q

3 year old boy from refugee camp, rash, angular cheilitis, glossitis, stomatitis, normocytic-normochromic anemia, seborrheic dermatitis

A

riboflavin deficiency (B2)

60
Q

What vitamin deficiency leads to: mucocutaneous symptoms, ecchymoses, petechiae, bleeding gums, hyperkeratosis, coiled hair

A

vitamin C deficiency

61
Q

pruritic papular or vesicular rash associated with celiac disease located on the knees, elbows, forearms, and buttocks

A

dermatitis herpetiformis

62
Q

How can celiac disease be diagnosed.

A

anti-tissue transglutaminase antibody IgA level followed by endoscopic duodenal biopsy for confirmation

63
Q

What is the pathophysiology of Guillian Barre syndrome?

A

demyelination of the peripheral motor nerves

64
Q

What is the classic presentation of Guillian Barre syndrome?

A
  • ascending weakness

- feet tingling (pins and needles) and neuropathic pain