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MCCQE1 > Paediatrics > Flashcards

Paediatrics Flashcards

1
Q

Normal neonatal heart rate range

A

100-160

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2
Q

Fragile X Syndrome- name: The mode of inheritance The name of the gene The type of mutation The type of diagnostic test

A

X-linked dominant FMR1 gene CGG triplet repeat Polymerase Chain Reaction PCR

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3
Q

Which grades of vesicoteric reflux require prophylactic antibiotics?

A

III to V

Grade I into the ureter only

Grade II into ureter + renal pelvis without distension

Grade III mild hydronephrosis

Grade IV moderate hydronephrosis

Grade V severe hydronephrosis + a twisted ureter

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4
Q

The only risk factor for 47XXY

A

This is Klinefelter’s and it’s maternal age

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5
Q

What is the mode of inheritance of Duchenne Muscular Dystrophy?

A

X-linked recessive

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6
Q

Complications of measles

A
  • Pneumonia
  • Diarrhoea
  • Encephalitis
  • Subacute sclerosing panencephalitis
  • Bacterial superinfection due to immune suppression
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7
Q

Name the disorder: low grade fever, arthralgia, + colicky abdominal pain. Then rash starting as pink maculopapules, progressing to petechiae or purpora, going from red to brown then fading. Arthritis involving the knees and ankles is present in 2/3.

A

Immunoglobulin A vasculitis (Henoch-Schonlein Purpura)

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8
Q

Presenting features of congenital hypothyroidism

A

Prolonged jaundice Feeding problems Lethargy Constipation Umbilical hernia Macroglossia Large fontanelles Puffy face

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9
Q

When to do a renal ultrasound for a UTI

A
  1. 1st febrile UTI and <2yo
  2. Any age with recurrent febrile UTIs
  3. Children with a UTI who have a strong family hx of renal/bladder issues or HTN
  4. Children who don’t respond to Abx treatment
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10
Q

Common chest pathogens in cystic fibrosis

A

Staph aureus

Haem influenza

Pseudomonas (esp in older pts)

Burkholderia cepacia is awful + assoc w multiple drug resistance

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11
Q

A serum test for Celiac disease with 90% sensitivity + specificity

A

Serum anti-tTG antibody Do IgA level as well to avoid a false positive in those with a specific deficiency

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12
Q

Phenylketonuria: mode of inheritance and affected gene

A

Autosomal recessive inherited disorder caused by the mutation of phenylalanine hydroxylase(PAH) gene

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13
Q

The gold standard diagnostic test for 22q11 microdeletion syndrome

A

DiGeorge Fluorescent insitu hybridization (FISH)

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14
Q

The drug of choice for epiglottitis

A

Ceftriaxone

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15
Q

Tetanus management

A

Rx: Supportive care, abx and tetanus IVIg

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16
Q

Most common cause of leukocoria in babies

A

Congenital cataracts

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17
Q

Prenatal exposure to which pathogen is associated with type 1 DM?

A

Rubella Note that this is not the case with rubella exposure after birth

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18
Q

The diagnostic test of choice for hypertrophic pyloric stenosis

A

Abdominal ultrasound, 90% sensitivity

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19
Q

CSF signs of meningeal inflammation- bacterial

A
  • Increased pleocytosis
  • High protein
  • Low glucose
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20
Q

Hand, foot and mouth disease is caused by what?

A

Coxsackie A16

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21
Q

The treatment of choice for absence seizures

A

Ethosuximide (or valproic acid)

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22
Q

Colic: the rule of 3s

A

>3 hours a day of crying (usually in evening), >3 days a week for > 3weeks

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23
Q

Name a pathology associated with breath holding attacks in infants

A

Iron deficiency anaemia Do a CBC + ferritin

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24
Q

The most common cause of meningitis in infants, children and adults + The name of the vaccine for it + The antibiotic regime of choice

A

Streptococcus pneumoniae 13-valent pneumococcal conjugate vaccine (PCV13) Ceftriaxone + vancomycin

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25
Q

Tourette’s syndrome co-morbidities

A

ADHD ODD OCD Conduct disorder

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26
Q

The drug of choice for accidental beta blocker poisoning

A

Glucagon

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27
Q

Presentation of intussusception

A
  • Sudden, intermittent abdo pain, emesis
  • Currant jelly stools (bloody)
  • Sausage shaped abdo mass
  • Lethargy or altered mental state
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28
Q

Risk factors for acute otitis media

A

Eustachian tube pathology

Cleft palate

Genetic syndromes eg Downs

Cilial dysfunction

Genetic predisposition

Indigenous population

Bottle fed

Immunocompromised

Passive smoking

Premature

Siblings in the household

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29
Q

Name a specific contraindication to Rota-1

A

Intussusception

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30
Q

Complications of cystic fibrosis

A

Respiratory failure

Pneumothorax (poor prognostic sign)

Cor pulmonale (late comp)

Pancreatic fibrosis w DM Gallstones

Bruising can result from pancreatic insufficiency →malabsorption of fat soluble vitamins reduced vit K → abnormal clotting

Rectal prolapse in 20% from bulky stools (pancreatic insufficiency)

Nasal polyps are common Infertility - all males

Malnutrition Death average 46 in Canada

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31
Q

ANA and anti-dsDNA are the main biomarkers for which condition?

A

SLE

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32
Q

The four malformations of the Tetralogy of Fallot

A

Pulmonary valve stenosis

Ventricular septal defect

Overriding aorta

RV hypertrophy

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33
Q

The rash associated with Lyme disease

A

Erythema migrans 1-2 weeks after transmission (spreading, annular rash with central clearing)

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34
Q

Name the syndrome: Learning disability Seizures Autism Funny ears Usually boys

A

Fragile X Note that physical traits get more prominent with age It’s the most common genetic cause of autism

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35
Q

The type of murmur associated with coarctation of the aorta

A

Systolic in the left interscapular area

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36
Q

75-85% of kids with coarctation of the aorta have what other cardiac condition?

A

A bicuspid aortic valve

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37
Q

Typical X-ray appearance in surfactant deficient lung disease

A

Ground glass appearance with indistinct heart borders

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38
Q

What’s the most appropriate investigation following a simple febrile seizure?

A

None- nothing required

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39
Q

Maternal contraindications to breastfeeding

A
  • Chemotherapy
  • Radioactive compounds
  • HIV/AIDS
  • Active TB or brucellosis
  • Herpes lesions on breasts
  • Consumption of more than 5g/kg/d alcohol
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40
Q

Diagnosis of epiglottitis

A

Diagnosis by indirect fiberoptic laryngoscopy There s a classic thumbprint sign on lateral c spine x ray but a normal X-ray does not exclude the diagnosis

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41
Q

Suspicious injuries in a child

A
  • Posterior rib #
  • Metaphyseal #
  • Scapular, vertebral #
  • Sternal #
  • Immersion injuries
  • Bruising on cushioned areas
  • Retinal haemorrhages (shaking)
  • Patchy hair loss
  • Torn frenulum
  • Burns w sharp margins
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42
Q

Pathological gynecomastia is suggested by what things? (5)

A
  • Rapid progression
  • Occurence outside neonatal or pubertal age range
  • Occurence in prepubertal boys
  • Breast enlargement over 4cm in diameter
  • Rapid or precocious virilization
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43
Q

Which treatment is contraindicated in the treatment of infant botulism & why?

A

Antibiotics for clostridium bcs of the risk of releasing more toxin through cell lysis

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44
Q

Name the first line therapy for primary eneuresis in a child under 7

A

Motivational therapy (reward system)

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45
Q

Congenital adrenal hyperplasia: The mode of inheritance and the most commonly affected enzyme

A

Autosomal recessive 21-hydroxylase deficiency

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46
Q

Diagnosis and treatment of phenylketonuria

A

Dx: Quantitative amino acid analysis (phenylalanine) Rx: dietary restriction of phenylalanine

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47
Q

Causes of transient congenital hypothyroidism

A

Iodine deficiency Maternal Antibody mediated Prenatal exposure to antithyroid Rx Congenital liver haemangiomas

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48
Q

The commonest cause of atypical pneumonia in school aged children

A

Mycoplasma pneumonia

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49
Q

Jaundice in the first 24 hours of life is caused by what? (2)

A

Rhesus incompatability Sepsis It’s always pathological

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50
Q

What are the criteria for diagnosing fetal alcohol syndrome?

A

Growth deficiency Facial anomalies CNS dysfunction

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51
Q

The gold standard diagnostic test for influenza

A

PCR Specifically viral reverse transcription PCR

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52
Q

Cystic Fibrosis- name (the most common): The gene The chromosome The mode of inheritance The incidence

A

CFTR gene Chromosome 7 Autosomal recessive 1 in 3000

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53
Q

Significance of ‘onion-skinning’ appearance of X-Ray of a long bone

A

Think of Ewing sarcoma

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54
Q

Complications of achondroplasia

A
  • Obesity
  • Otitis media
  • Apnea and bradypnea
  • Spinal stenosis
  • Obstetric problems
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55
Q

A child under 16 with a 6 week minimum history of this has what? Morning stiffness at least one hour min the morning Reduced ROM Joint pain Spiking fevers Evanescent rash on trunk Post exercise pain

A

Juvenile idiopathic arthritis (Do a slit lamp examination to rule out uveitis)

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56
Q

Features of congenital toxoplasmosis

A

• Macrocephaly (hydrocephalus), diffuse intracranial calcifications, jaundice, growth restriction, hepatosplenomegaly, blueberry muffin spots • Severe chorioretinitis

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57
Q

Galactosemia

A

Presents within first week of life, features: -hypoglycemia* -jaundice* -cyanosis, jitteriness, seizures -enlarged liver Urine will contain reducing substance (galactose) but has no ketone bodies

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58
Q

With which condition is the Steeple sign on X-Ray associated?

A

Croup

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59
Q

In the Canadian vaccine schedule, what is given at 12 months?

A

MMR

Pneumococcal conjugate

Meningococcal conjugate

Varicella

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60
Q

Meningococcus prophylaxis

A

Rifampin or CTX Ceftriaxone is the drug of choice for an exposed pregnant woman

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61
Q

Diagnosis of rubella

A

Rubella specific IgM antibodies, by virus culture or specific serology.

The virus can also be isolated from pharynx and blood.

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62
Q

When to treat acute otitis media with antibiotics

A
  1. Under 6m age
  2. Between 6m - 2y with symptoms persisting longer than 3days
  3. Over 2y but sick, with bilateral involvement, or who cannot be followed up within 48h
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63
Q

Treatment for Lyme disease

A

Amoxicillin until age 8

Doxycyline older than 8yo

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64
Q

Risk of recurrence of intussusception

A

Up to 10%

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65
Q

Live vaccines

A

Yellow fever Chicken pox Intranasal influenza Rotavirus Oral polio Herpes zoster MMR

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66
Q

Mineral deficiencies associated with febrile convulsions (2)

A

Iron + zinc

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67
Q

What reduces morbidity + mortality in children with severe measles?

A

Vitamin A

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68
Q

Three classical signs of croup

A
  • Stridor
  • Seal like barking cough
  • Subglottic swelling
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69
Q

Name the syndrome- failure of puberty + anosmia

A

Kallmann syndrome Isolated GnRH deficiency with anosmia

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70
Q

Risk factors for hip dysplasia

A
  • Female
  • Breech presentation
  • Oligohydramnios
  • Firstborn
  • Family history
  • Birthweight >5kg
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71
Q

A blood test to detect Duchenne Muscular Dystrophy in a boy who is slow to walk

A

Elevated creatine kinase

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72
Q

Which vaccine is contraindicated in kids with atopic dermatitis?

A

Smallpox

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73
Q

The presenting features of acute lymphoblastic leukaemia

A

Bone pain, arthritis, limping Pallor Tiredness Fevers common at presentation (but sepsis rare) Bleeding

Sometimes: hepatosplenomegaly + lymphadenopathy Plus signs of BM inflitration: Anaemia, neutropenia, thrombocytopenia

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74
Q

Prevention of iron deficiency anaemia in a neonate

A

Breastfed term infants supplement @ 4-6m

Non breastfed start from birth

Premature supplement from one month until 12m

No cow’s milk until 9-12m

Screening of Hb levels between 6 and 12 months of age

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75
Q

Drugs that enter breastmilk

A
  • Lithium
  • High dose diazepam
  • Cyclophosphamide
  • Tetracyclines
  • Metronidazole
  • Bromocriptine
  • Chloramphenicol
  • Gold
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76
Q

Diagnostic test for Meckel’s Diverticulum

A

Diagnostic test of choice is technetium-99m pertechnetate scan aka Meckel scan

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77
Q

A test with 90% negative predictive value for septic arthritis

A

ESR + CRP Note that 50% of synovial fluid aspirations are sterile Blood cultures are only positive in 40-50%

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78
Q

The investigation of choice to confirm coarctation of the aorta

A

Transthoracic echo

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79
Q

A Finnegan’s score >8 is an indication for what? And what would be the first line treatment in this circumstance?

A

Pharmacological therapy in neonatal abstinence syndrome.

First line therapy is morphine, in a NICU setting

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80
Q

There is an 80% chance a neonate with meconium ileus has what?

A

Cystic Fibrosis

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81
Q

Investigations for kids under 5y w recurrent UTIs

A

Renal ultrasound - rules out hydronephrosis

VCUG - rules out VUR

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82
Q

Melanocytic skin lesions

A

Freckles

Lentigo

Naevi

Melanoma

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83
Q

A syndrome characterized by frequent and prolonged febrile convulsions in the first year of life, followed by myoclonus and atypical absences in the second year, then developmental delay

A

Dravet syndrome

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84
Q

Features of Patau syndrome Trisomy 13

A
  • Midline defects: microcephaly/holoprosencephaly, microphthalmia, cleft lip/palate
  • Umbilical hernia / omphalocele
  • Rocker bottom feet
  • Renal/Cardiac defects
  • Polydactyly
  • Cutis aplasia
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85
Q

Treatment of choice for allergic rhinitis

A

Intranasal glucocorticoids

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86
Q

The most common presentation of Meckel’s Diverticulum- two things

A

1) Painless lower GI bleed 2) Intestinal obstruction

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87
Q

The dose of adrenaline in paediatric anaphylaxis

A

0.01mls/kg of 1 in 1000, max 0.5mls

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88
Q

Bartonella henselae causes what?

A

Cat scratch disease

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89
Q

Immunisation contraindications

A

MMR is contra in pregnancy + immunocomp even if asymptomatic

Rota-1 Contra w a Hx of intussusception and imnnunocompromised

DTaP contra first trimester

Influenza contra 1st 6m of life + immunocomp

Varicella is contra if preg or planning preg in next 3/12

Anaphylaxis to any vaccine is a contra

90
Q

Complications of acute otitis media

A

Hearing loss

Speech delay

TM perforation

Local spread eg mastoiditis

Cholesteatoma

Facial nerve palsy

Ossicular necrosis

Vestibular dysfunction

Meningitis

Epidural + brain abscess

Subdural empyema

Lateral + cavernous sinus thrombosis

Carotid artery thrombosis

91
Q

Name the syndrome:

Truncus arteriosus

Schizophrenia

Micrognathia

Bifid uvula

Positive Chvostek’s sign

Weak and fatigued

A

DiGeorge

92
Q

The gold standard test for identification of a contact allergen

A

Patch test aka thin layer rapid use epicutaneous test

93
Q

Trisomy 21 appearance

A
  • Hypotonia
  • Epicanthic folds
  • Upslanting palpebral fissures
  • Low set small ears
  • Flat facial profile
  • Short neck with excess skin
  • Sandal-toe deformity
  • Hypoplastic incurved 5th finger
  • Single transverse palmar crease
  • Brushfield spots
94
Q

Commonly observed signs in neonatal meningitis

A

Seizures Bulging anterior fontanelle Extensor posturing (opisthotonos) Focal cerebral signs eg gaze deviation cranial nerve palsies

95
Q

Things streptococcus pneumoniae causes

A

Meningitis Otitis media Pneumonia Sinusitis MOPS

96
Q

Area of brain typically affected by herpes simplex encephalitis in neonates

A

The temporal lobes

97
Q

SIDS risk factors

A
  • Bed sharing
  • Drugs + alcohol
  • Indigenous
  • Smoking in the household
  • No prenatal care
  • Low birthweight
  • Prone
  • Poverty
  • 3-5X increase after a sibling has died
98
Q

Most significant cause of blindness and deafness in neonates

A

Congenital rubella

99
Q

Complications of a Pavlik harness

A

Avascular necrosis of the femoral head Femoral nerve palsy

100
Q

What’s the cause of roseola infantum?

A

Human herpes virus 6

101
Q

Features of anaemia of prematurity

A

Impaired EPO production in prems Exacerbated by short red blood cell life span in prems Usually asymptomatic Can get tachycardia, apnea, poor weight gain Labs: • Low HgB and hematocrit • Low retics • Normocytic, normochromic

102
Q

Things that can cause elevated sweat chloride levels

A

Untreated adrenal insufficiency

Pseudohypoaldosteronism

Hypothyroidism

Hyoparathyroidism

Ectodermal dysplasia

Mucoploysaccharidoses

Fucosidosis Pancreatitis

103
Q

Name the disorder + the mode of inheritance: Jaundice Anaemia Gallstones Splenomegaly

A

Hereditary spherocytosis Autosomal dominant

104
Q

What is the best method for detecting drug use in pregnancy?

A

Meconium analysis Collect it before it is contaminated by milk or formula though

105
Q

A drug used usually on a temporary basis to treat primary enuresis

A

Desmopressin (DDAVP)

106
Q

Management of a pregnant woman exposed to varicella

A

Pregnant females and immunocompromised should receive IVIg the vaccine is live attenuated

107
Q

Think what in a neonate presenting with inconsolable crying, a mottled appearance and only brief sleeps after feeding?

A

Opioid withdrawal

108
Q

What is the mode of inheritance of neurofibromatosis type 1?

A

Autosomal dominant

109
Q

Physical causes of encopresis

A
  • Chronic constipation
  • Hirschsprung disease
  • Hypothyroid
  • Hypercalcaemia
  • Spinal cord lesions
110
Q

75-80% of cases of bronchiolitis are caused by what?

A

RSV

111
Q

X-linked agammaglobulinaemia is associated with very small tonsils, abnormal lymph nodes, and what immune cell abnormality?

A

Lack of B lymphocytes

112
Q

A severe sore throat in the presence of a normal appearing pharynx is worrying why?

A

This is suggestive of epiglottis caused by H influenzae and can be a life threatening emergency

113
Q

Treatment of croup

A
  1. Single dose of dexamethasone or two days of prednisolone- (sufficient alone for mild/moderate cases) 2. Adrenaline 1in1000 0.5mls/kg (severe)
114
Q

Features of phenylketonuria

A

Severe intellectual disability

Seizures

Musty body odor

Hypopigmentation involving skin, hair, eyes and brain nuclei

115
Q

The criteria for sepsis

A

Temp >38.5

Plus one:

Sats <92%

Inappropriate tachycardia

Tachypnoea

Altered consciousness

Cap refill >4s

Purpura/ non blanching rash

116
Q

Features of Prader-Willi syndrome

A
  • Hypotonia and FTT as neonate
  • Intellectual disability, delayed milestones
  • Extreme obesity, short stature
  • Hypogonadism/microphallus
  • Dysmorphic: narrow forehead, almond shaped eyes, downturned mouth
  • Hyperphagia
117
Q

Organisms typically involved in osteomyelitis

A

Staph aureus- most common Salmonella (especially in sickle cell anaemia patients) Pasteurella multocida from cat and dog bites

118
Q

Normal daily weight gain in a neonate

A

20-30g daily, or 150-200g weekly

119
Q

Pathologies that look like child abuse (2)

A

Infantile scurvy

Osteogenesis Imperfecta

120
Q

Maternal risk factors for VSD

A

Alcohol in pregnancy

Phenylketonuria

Diabetes

121
Q

Complications of obesity

A
  • Slipped capital femoral epiphysis
  • NIDDM
  • Asthma
  • Apnea
  • Gynecomastia
  • Early menarche
  • Irreg menses
  • Fatty liver disease
  • Steatohepatitis (can be reversed w Vitamin E)
122
Q

Complications of ulcerative colitis

A

Severe bleeding

Fulminant colitis

Toxic megacolon

Perforation

Primary sclerosis cholangitis esp in men

Colorectal cancer (risk increases w duration + extent of disease)

123
Q

Biggest risk factor for cerebral palsy

A

Prematurity

124
Q

The diagnostic test of choice for herpes simplex encephalitis

A

PCR of CSF for common viruses has 95% sensitivity + specificity

125
Q

Name the standard surgical technique for VSD repair

A

Apical left ventriculectomy

126
Q

Risk factors for ambiguous genitalia

A

Parental consanguinity FH Maternal meds in preg eg danazol, androgens, progesterone, phenytoin

127
Q

Complications of infectious mononucleosis

A

Splenic rupture

Guillain-Barre syndrome

128
Q

What type of hypersensitivity reaction is contact dermatitis?

A

Type IV

129
Q

What type of hypersensitivity reaction is skin prick testing for?

A

Type I (IgE mediated)

130
Q

The 5 Ds of epiglottitis

A

Drooling Dysphagia Distressed Dysphonia Dyspnoea

131
Q

Croup is caused by what?

A

Parainfluenza

132
Q

Beckwith-Wiedmann syndrome is associated with which pathology?

A

Wilm’s tumour

133
Q

Bacteria that cause acute otitis media

A
  • Strep pneumoniae
  • Haemophilus influenza
  • Moraxella catarrhalis
134
Q

How do you treat pregnant women who are contacts in a measles outbreak?

A

Give them immunoglobulin. MMR is contraindicated

135
Q

Name the disease from this pathognomic sign- a pseudomembrane on the tonsils, pharynx or nasal cavity

A

Diphtheria

136
Q

Treatment of rheumatic fever

A

Benzylpenicillin 0.6-1.2g IM then Pen V 250mg 6 hourly

Bed rest for 2/52 until the CRP is normal

Analgeisa for carditis/ arthritis w aspirin or naproxen

Immobilisation of joints in severe arthritis

Haloperidol or diazepam for the chorea

Monitor CRP + ESR

137
Q

Name the disorder and it’s causative agent: Prodrome: Very high spiking fever (> 40° C) Maculopapular rash appears as soon as high fever resolves Rash starts on the trunk and expands outward (opposite of measles)

A

Roseola, HHV6

138
Q

Cocaine exposure in utero is associated with what?

A
  • Increased risk of necrotizing enterocolitis
  • Small for gestational age
  • Preterm labour + spontaneous abortion
  • Abnormal neonatal sleep pattern
139
Q

Typical age for intussusception

A

75% in the first two years of life

140
Q

Investigations for intussusception

A

Abdo US (Target sign) Air or barium enema

141
Q

Presentation of intussusception

A

Colicky progressive abdominal pain, drawing of legs up to chest, lethargy, bloody “red currant jelly” stool (Triad) Progression to severe periumbilical pain Often preceded by a URTI

142
Q

Aetiology of intussusception

A

90% idiopathic, but children with CF or GJ tube at signicantly increased risk

143
Q

Management of intussusception

A

Rehydration + analgesia

Air enema can be therapeutic (reduces intussusception in 75% of cases)

144
Q

Rome III criteria for functional constipation

A

2 of the following for at least 1m:

2 defecations/wk

history of excessive stool retention

history of large-diameter stools

history of painful or hard bowel movements

in toilet trained children, the following additional criteria may be used:

at least one episode/wk of incontinence after the acquisition of toileting skills

history of large-diameter stools that may obstruct toilet

145
Q

Crucial time periods in the development of constipation in kids

A

When introducing cow’s milk after breast milk

During toilet training and then when starting school

146
Q

Complications of constipation

A

Pain retention cycle: anal fissures + pain from withholding passing stool

Chronic dilatation ± overflow incontinence

147
Q

The cardinal sign of malrotation in children

A

Bilious emesis is the cardinal sign, especially if abdomen nondistended

148
Q

Management of malrotation

A

Emergency laparotomy with Ladd’s procedure: counterclockwise reduction of the volvulus with division of Ladd’s bands

149
Q

Clinical features of insulinoma

A

• Whipple’s triad • palpitations, trembling, diaphoresis, confusion, seizure, and personality changes

150
Q

Features of malignant lymphadenopathy

A

Firm

Discrete

Non-tender

Enlarging

Immobile

Abnormal imaging findings or bloodwork

Constitutional symptoms

Worrisome location (i.e. supraclavicular or generalized)

151
Q

What is Legg-Calvé-Perthes Disease (Coxa Plana) and when does it present?

A

Idiopathic AVN of femoral head, presents at 4-8 yr of age

152
Q

Legg-Calvé-Perthes Disease presentation

A
  • child with antalgic or Trendelenburg gait ± pain
  • intermittent knee, hip, groin, or thigh pain
  • flexion contracture (sti hip): decreased internal rotation and abduction of hip
  • limb length discrepancy (late)
153
Q

Treatment of scoliosis

A

Based on Cobb angle <25°: observe for changes with serial radiographs >25° or progressive: bracing (many types) that halt/slow curve progression but do not reverse deformity >45°, cosmetically unacceptable, or respiratory problems: surgical correction (spinal fusion)

154
Q

Treatment of club foot

A

largely non-operative via Ponseti Technique (serial manipulation and casting)

correct deformities in CAVE order

change strapping/cast q1-2wk

surgical release in refractory case (rare) – delayed until 3-4 mo of age

155
Q

Typical timing of start and end of breast milk jaundice

A

Starts day 7 Peaks weeks 2+3 Usually resolved by week 6

156
Q

With what is cutis aplasia classically associated?

A

Trisomy 13

157
Q

When does pyloric stenosis typically present?

A

A month after birth

158
Q

Common causes of anaphylaxis

A

Wasp stings Bee stings Red fire ants Parenteral antibiotics Also Blood products Antivenom Radiological contrast materials Anaethetic agents

159
Q

Vaccines which increase the risk of febrile convulsions

A

DTP and MMR

160
Q

Developmental red flags (from QBank)

A
  • Not smiling at 3m
  • Rolling too early at <3m (possible hypertonia)
  • Persistence of primitive reflexes after 6m
  • Not babbling by 12m
  • Not pointing by 15-18m
  • <10words by 18m
  • Not walking at 18m
161
Q

Diagnostic test for varicocele

A

High resolution colour flow Doppler ultrasonography

162
Q

Diagnostic test for X-Linked Agammaglobulinemia (XLA)

A

Fluorocytometric studies (ie flow cytometry) of B + T-lymphocyte markers must be performed to confirm XLA diagnosis.

163
Q

Required annual screening investigation in XLA

A

Pulmonary function tests

164
Q

Bugs children with XLA are vulnerable to

A
  • Haemophilus
  • Pneumococcus
  • Mycoplasma
  • Ureaplasma
  • Campylobacter
  • Giardia
  • Enteroviruses
165
Q

Children’s respiratory rates

A

Age Category Age Range Normal RR Infant 0-12 months 30-60 per minute Toddler 1-3 years 24-40 per minute Preschooler 4-5 years 22-34 per minute School Age 6-12 years 18-30 per minute

166
Q

Treatment for idiopathic Bell’s Palsy

A

Prednisone

167
Q

Early onset neonatal sepsis typically occurs when and is associated with what?

A

In the first 72 hours, associated with early or prolonged rupture of membranes. Organisms are acquired from the mother

168
Q

What is the drug of choice for community acquired pneumonia in children aged 6 months to 5 years?

A

Amoxycillin

169
Q

What is tarsal coalition and when does it present? And how does it present?

A

Fusion of two or more of the tarsal bones, usually bilateral, presents in adolescence. Presents w tenderness over the subtalar jt, rigid flatfoot, limited subtalar motion, peroneal tightness & pain on foot inversion

170
Q

What is the HIV screening tool appropriate for infants born to HIV+ mothers?

A

HIV DNA PCR at 48h, 2 months and 6 months

171
Q

Jaundice, cataracts, vomiting, diarrhoea, and poor weight gain in a breastfed neonate are highly suggestive of what?

A

Galactosemia

172
Q

Red flags of asthma severity

A
  • Fatigue
  • Cyanosis
  • Silent chest
  • Diminished respiratory effort
  • Diminished level of consciousness
173
Q

Classification of severity of asthma

A

mild: occasional attacks of wheezing or coughing (<2/wk); symptoms respond quickly to inhaled bronchodilator; never needs systemic corticosteroids

moderate: more frequent episodes with symptoms persisting and chronic cough; decreased exercise tolerance; sometimes needs systemic corticosteroids

severe: daily and nocturnal symptoms; frequent ED visits and hospitalizations; usually needs systemic corticosteroids

174
Q

Asthma: indications for hospitalisation

A
  • ongoing need for supplemental Oxygen
  • persistently increased work of breathing
  • 2-agonists are needed more often than q4h after 4-8 h of conventional treatment
  • patient deteriorates while on systemic steroids
175
Q

Signs of poorly controlled asthma

A
  • Use of beta-2agonists more than four times a week
  • Asthma related school absence
  • Exercise induced asthma
  • Night-time symptoms occurring more than 2x/week
176
Q

What is the mode of inheritance of tuberous sclerosis?

A

Autosomal dominant

177
Q

The diagnostic test for hereditary spherocytosis

A

Osmotic fragility test

178
Q

Combination of arthritis and palpable purpuric rash

A

Immunoglobulin A vasculitis (Henoch-Schonlein Purpur)

179
Q

Management of Milk allergy (MA) and Cow’s Milk Protein allergy

A

Milk Allergy (IgE mediated)- avoid milk

Cow’s Milk Protein allergy- stop, reintroduce at 6-8m- most cases (>90%) outgrow intolerance by 1 year

(CanadaQBank says TOC is continue bf and mother removes whole milk from diet)

180
Q

Milk protein allergy- typical presentation

A

Usually in months 2-8 of infancy Evidence of malabsorption- poor weight gain, diarrhoea (may be bloody) May be associated with eczema (Note 25% pts are also intolerant to soy)

181
Q

G6PD deficiency presentation

A

• frequently presents as episodic hemolysis precipitated by: oxidative stress drugs (e.g. sulfonamide, antimalarials, nitrofurantoin) infection food (fava beans) • in neonates: can present as prolonged, pathologic neonatal jaundice

182
Q

G6PD deficiency inheritance

A

X-linked recessive

183
Q
A
184
Q

Risk factors for child abuse

A
  1. Prematurity
  2. Difficult child
  3. History of parental child abuse
  4. History of substance abuse
  5. Social isolation
  6. Developmental delay
  7. Poverty
  8. Parental character/personality disorder
185
Q

Initial management of constipation in children

A

in children with a hx of chronic constipation with stools that are difficult to pass, disimpaction is the first strategy and this is best achieved using orally administered stool softeners like polyethylene-glycol

***

186
Q

No meconium 48 hours after birth implies what?

A

Aganglionic megacolon (Hirschsprung disease). Most neonates pass meconium within 24h

***

187
Q

Neonate with meconium ileus

A

Cystic fibrosis

188
Q

Sudden onset altered mental state, emesis and liver dysfunction after aspirin

A

Reye syndrome

189
Q

Active teenager with anterior knee pain

A

Osgood-Schlatter disease

190
Q

Tanner stage 3 in a 6 year old girl

A

Precocious puberty

191
Q
A

Osteosarcoma with classical sunburst appearance

192
Q
A
193
Q
A

Ewing sarcoma with onion skin appearance

194
Q
A

Congenital hypothyroidism

195
Q
A

Gower’s sign

Duchenne muscular dystrophy

196
Q
A

Cutis aplasia

197
Q
A

Intussuseption

198
Q
A

Rubella

199
Q
A

Tarsal coalition

200
Q
A

Legg-Calve-Perthes disease

201
Q
A

Kawasaki disease

202
Q
A

Staphylococcal scalded skin syndrome

203
Q
A

Cholesteatoma is a destructive and expanding growth consisting of keratinizing squamous epithelium in the middle ear and/or mastoid process. Cholesteatomas are not malignant, but can cause signif problems because of their erosive and expansile properties. This can result in the destruction of the bones of the middle ear (ossicles), as well as growth through the base of the skull into the brain. They often become infected and can result in chronically draining ears. Treatment almost always consists of surgical removal. Usually caused by otitis media

204
Q
A

Roseola infantum

HHV-6

205
Q
A

Scabies

n web spaces and folds, v itchy, whole family affected

Sarcoptes scabeie

Rx permethrin

206
Q
A

Impetigo

V contagious. Honey coloured crusts or superficial bullae

Nose & mouth, hands & feet

Staph aureus or strep pyogenes

Rx topical abx if mild- fucidic acid, muciprocin ointment

Oral abx if severe or in numerous sites - cephalexin/ erythromycin

Consider MRSA

207
Q
A

Fetal alcohol syndrome

208
Q
A
209
Q
A

Angular chelitis

Iron deficiency anaemia

210
Q

Treatment for Von Willebrand Disease

A

Desmopressin

211
Q

Haemophilia A inheritance

A

X-linked recessive

212
Q

The best indicator of remission in ALL

A

Blast cell numbers in bone marrow, specifically remission is when there are <5% blast cells.

213
Q
A

Erythema marginatum

Rheumatic fever

214
Q
A

Meckel scan

Diagnostic test of choice is technetium-99m pertechnetate scan aka Meckel scan. It uses a radionuclide to bind plasma protein and accumulate in functional gastric mucosa.

215
Q
A

Ortolani test

The femoral head is reduced back into the acetabulum with this maneuver.

216
Q
A

Barlow test

The hip can be popped out of the acetabulum with this maneuver.

217
Q
A

Toxic megacolon

Complication of ulcerative colitis

218
Q
A
219
Q

A 29 year old man presented to primary care with anxiety and depression that had worsened since childhood. Further questioning revealed a history of poor school performance, poor body image, and poor self esteem. On physical exami- nation, the patient’s height was 189 cm and he had narrow shoulders, wide hips, sparse facial hair (which he shaved once every two months), and small, firm testicles. He was found to have elevated luteinising hormone and follicular stimulating hormone concentrations, low serum concentrations of testosterone and absent sperm on semen analysis

A

Klinefelters, usually 47,XXY

***

220
Q

A 7 year old girl presenting with growth of pubic hair and no other abnormalities, what action is required?

A

Nothing, this is premature puberty (before 8) and not of significance in the absence of any signs suggesting the progressive development of true precocious puberty

***

221
Q

Most likely cause of iron deficiency anaemia in a kid that has failed to respond to iron therapy

A

Thalassaemia

Do Hb electrophoresis

***

222
Q

What is shown?

A

Rickets in a child

In the growing skeleton, the deficiency of normal mineralisation is most evident at metaphyseal zones of provisional calcification where there is an excess of non-mineralised osteoid resulting in growth plate widening and abnormal configuration of the metaphysis:

fraying: indistinct margins of the metaphysis
splaying: widening of metaphyseal ends
cupping: concavity of metaphysis

***

MCCQE1 (20 decks)

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