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Phase 3a > Paeds 4 - Endocrine, Malignancy, and MSK > Flashcards

Paeds 4 - Endocrine, Malignancy, and MSK Flashcards

1
Q

What is a preventable cause of learning difficulty?

A

Congenital hypothyroidism

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2
Q

What are the causes of congenital hypothyroidism?

A

Maldescent of thyroid
Dyshormonogenesis
Iodine deficiency

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3
Q

What are the common symptoms of congenital hypothyroidism?

A 
Failure to thrive
Constipation
PNJ
Pale, cold, mottled skin
Coarse facies and large tongue
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4
Q

What is the Guthrie test?

A

Neonatal blood spot screening between days 5-9 of life

Tests for CF, sickle cell, congenital hypothyroidism, etc

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5
Q

What is the treatment of hypothyroidism?

A

Thyroxine lifelong

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6
Q

What are the causes of hyperthyroidism in children?

A

Graves’ disease (autoimmune thyroiditis)

Neonatal - transplacental transfer of TSIs

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7
Q

What is the acronym for hyperthyroidism?

A 
Sweating
Weight loss
Emotionally labile
Appetite increase
Tremor and tachycardia
Intolerance to heat/irregular periods
Nervousness
Goitre and GI - diarrhoea

Eye signs (exophthalmos and lid lag) not common in children

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8
Q

How is hyperthyroidism diagnosed?

A

Increased T3 and T4

Very decreased TSH

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9
Q

How is hyperthyroidism treated?

A

Carbimzole/propythiouracil
Radioactive iodine
Thyroidectomy

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10
Q

Which hormone promotes bone formation via osteoblasts?

A

Parathyroid hormone

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11
Q

What occurs when calcium levels are low?

A

PTH promotes bone resorption via osteoclasts, increases renal uptake of calcium, activates metabolism of Vit D to increase gut Ca absorption

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12
Q

What are the symptoms of hypoparathyroidism?

A

Hypocalcaemia = muscle spasm, diarrhoea, stridor, fits

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13
Q

What are the symptoms of hyperparathyroidism?

A

Hypercalcaemia = constipation, polyuria, renal and gallstones, polydipsia, abdo pain, anorexia

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14
Q

What is the treatment of acute hypocalcaemia?

A

IV calcium gluconate

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15
Q

What is the treatment of acute hypercalcaemia?

A

Rehydration and bisphosphonates

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16
Q

What is the name of the condition where primary adrenal cortical insufficiency occurs?

A

Addison’s disease

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17
Q

What are the main causes of Addison’s disease?

A

Autoimmune

Haemorrhage

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18
Q

What is the acute presentation of Addison’s disease in infants?

A

Low sodium and glucose, high potassium
Dehydration and hypotension
Circulatory collapse

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19
Q

Older children with Addison’s disease present more subtly, with…

A

Vomiting
Brown hyperpigmentation
Growth failure
Fatigue

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20
Q

What hormone production is disrupted in Addison’s disease?

A

Decreased cortisol

Increased ACTH

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21
Q

What is the treatment of an adrenal crisis?

A

IV saline, glucose, hydrocortisone

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22
Q

What is the main treatment of Addison’s disease?

A

Long term mineralo-corticoid and glucocorticoid replacement

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23
Q

Glucocorticoid excess is known as?

A

Cushing syndrome

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24
Q

What conditions require long term steroids that can result in Cushing syndrome? (ACTH independent)

A

Nephrotic syndrome
Asthma
Severe bronchopulmonary dysplasia

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25
Q

What are the symptoms of Cushing syndrome?

A 
Growth failure
Face and truncal obesity
Red cheeks
Hirsuitism and striae
Bruising
Hypertension
Muscle wasting and weakness
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26
Q

How is Cushing syndrome diagnosed?

A

Loss of diurnal variation of cortisol (high in morning)

Dexamethasone has no effect on plasma cortisol levels

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27
Q

Give three causes of limp in a child

A 
Osteomyelitis
Fracture
Perthes disease
Septic arthritis
Sickle cell crisis
Slipped epiphysis
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28
Q

What are the likely organisms for MSK infections in infants, <4 years, and >4 years?

A

Infants - Group B strep, staph, coliforms
<4 years - Staph, pneumococcus, haemophilus
>4 years - Staph, gonococcus

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29
Q

What is a pathological cause of genu varum?

A

Bowed legs - ricketts

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30
Q

What is the name for knock knees?

A

Genu valgum

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31
Q

What is pes planus?

A

Flat feet

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32
Q

What is important to exclude in a child who toe-walks?

A

Duchenne muscular dystrophy
JIA
Spastic diplegia

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33
Q

Talipes equinovarus can be caused by what?

A

Oligohydramnios, IUGR

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34
Q

What is pes cavus?

A

High arched foot

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35
Q

Describe the spectrum of disorders in developmental dysplasia of the hip

A

Dysplasia to subluxation to complete dislocation

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36
Q

What are the risk factors for DDH?

A

Family history
Breech
Macrosomia/first born child
Female sex x6

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37
Q

How is DDH diagnosed?

A

Routine neonatal screening
Later - limp or abnormal gait
Ultrasound

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38
Q

What is a complication of DDH?

A

Necrosis of femoral head

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39
Q

What is scoliosis?

A

Lateral curvature in the frontal plane of the spine

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40
Q

What is the most common cause of scoliosis?

A

Idiopathic

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41
Q

What is infection of the metaphysis of the long bones?

A

Osteomyelitis

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42
Q

What are the most common sites for osteomyelitis?

A

Distal femur and proximal tibia

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43
Q

What are the symptoms of osteomyelitis?

A

Pseudoparesis and pain in limb
Acute febrile illness
Swelling, erythema, tender
Sterile effusion of adjacent joint

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44
Q

What is seen on X-Ray in osteomyelitis?

A

Soft tissue swelling and subperiosteal new bone formation after 7 days

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45
Q

How is osteomyelitis managed?

A

IV antibiotics

Aspiration or surgical decompression if atypical or immunocompromised

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46
Q

What are the complications of osteomyelitis?

A

Bone necrosis

Limb deformities

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47
Q

What is Osgood-Schlatter disease?

A

Osteochondritis of the patellar tendon insertion at the knee

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48
Q

What is important in examining a painful knee?

A

Examine the hip as hip pain is often referred to the knee

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49
Q

Give three red flags for back pain

A 
Young age
High fever
Night waking
Focal neurological signs
Weight loss
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50
Q

Give three causes of back pain

A

Muscle spasm or injury
Benign or malignant tumours
Spinal cord compression
Vertebral osteomyelitis or discitis

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51
Q

Avascular necrosis of the capital femoral epiphysis of the femoral head is known as…

A

Perthes’ disease

52
Q

What is seen on X-Ray in Perthes’ disease?

A

Increased density in femoral head

Fragmented and irregular bone

53
Q

Who typically gets Perthes’ disease?

A

Boys aged 5-10 years

54
Q

What are the symptoms of Perthes’ disease?

A

Insidious hip or knee pain, limp

Pain increases with movement, especially internal hip rotation

55
Q

How is Perthes’ disease treated?

A

Bed rest and analgesia if under 6

Calipes

56
Q

What is Perthes’ disease a risk factor for?

A

Degenerative arthritis in adult life

57
Q

Transient synovitis is usually accompanied by…

A

A viral infection (can be recent)

58
Q

What is inflamed in transient synovitis?

A

Synovium of the capsule of the hip joint usually

59
Q

How does transient synovitis present?

A

Sudden onset of hip pain or limp
No pain at rest
Decreased range of movement, especially internal rotation
Afebrile

60
Q

What causes a slipped capital femoral epiphysis?

A

Fracture through the growth plate (physis) which results in slippage of the epiphysis
Epiphysis is displaced posteroinferiorly

61
Q

Who typically gets a SCFE?

A

Obese boys 10-15 years

62
Q

How does SCFE present?

A

Waddling gait
Limp or hip pain
Decreased range of movement

63
Q

How is SCFE managed?

A

X-Ray

Fixation surgery to prevent avascular necrosis

64
Q

What does a septic joint look like?

A

Erythematous, swollen, warm, acutely tender joint, with decreased range of movement and pseudoparalysis in an unwell child

65
Q

How is septic arthritis managed?

A

US guided aspiration of joint fluid to culture and identify organisms
IV antibiotics

66
Q

What is the name for brittle bone disease?

A

Osteogenesis imperfecta

67
Q

Define juvenile idiopathic arthritis

A

Persistent joint swelling (>6 weeks) before 16 years, in the absence of infection or any other cause.

68
Q

What are the main subtypes of JIA?

A

Oligoarthritis
Polyarthritis (rheumatoid factor positive or negative)
Systemic arthritis
Psoriatic arthritis

69
Q

What is gelling in JIA?

A

Stiffness after periods of rest e.g. in the morning

70
Q

What are the other symptoms of JIA?

A

Pain
Intermittent limp
Swelling
Deformities such as leg lengthening, valgus deformity, fever

71
Q

What are the complications of JIA?

A 
Anterior uveitis (can lead to visual impairment)
Flexion contractures
Growth failure
Delayed puberty
Osteoporosis
72
Q

What is the use of NSAIDs for in JIA?

A

Relieve symptoms

73
Q

What is the first line treatment for JIA?

A

Ultrasound guided steroid periarticular injections

74
Q

What are the systemic symptoms in systemic JIA?

A

Evanescent salmon pink rash
Lymph node enlargement
Hepatomegaly/splenomegaly
Serositis e.g. pleuritis, pericarditis

75
Q

What is first line treatment if multiple joints are affected in JIA?

A

Methotrexate SC

76
Q

Which other drugs may be used in JIA?

A

Etanercept (anti-TNF)
Sulfasalazine
Tocilizumab

77
Q

Name an opportunistic infection in immunocompromised children

A

Pneumocystis jiroveci pneumonia
Aspergillus or candidiasis
VZV

78
Q

Which is the most common childhood leukaemia?

A

Acute Lymphoblastic Leukaemia (80%)

79
Q

Which syndrome has an increased risk of ALL?

A

Down syndrome

80
Q

What are the main symptoms of ALL?

A 
Fever
Anaemia
Infection
Bruising
Petechiae
Hepatosplenomegaly
Lymphadenopathy
81
Q

What is seen on blood film in ALL?

A

Blast cells

82
Q

Which chromosome is translocated in leukaemia?

A

Philadelphia (short arm of C22)

83
Q

What is seen on FBC in leukaemia?

A

Low haemoglobin

Thrombocytopenia

84
Q

What is the treatment of leukaemia?

A

Chemo - methotrexate, vincristine, thiopurine

Bone marrow transplant

85
Q

What brain tumour is the most malignant in children?

A

Glioblastoma multiforme

86
Q

What are the symptoms of increased ICP in children?

A

Headache worse in the morning and vomiting
Papilledema
Ataxia
Visual changes

87
Q

What are the symptoms of increased ICP in neonates?

A

Vomiting
Tense fontanelle
Torticollis
Skull sutures separate

88
Q

Which type of lymphoma is more common in childhood?

A

Non-Hodgkins’ lymphoma

89
Q

50% of NHL is due to…

A

EBV

90
Q

How does lymphoma present?

A

Painless cervical lymphadenopathy

91
Q

How is lymphoma investigated?

A

LN biopsy and then BM biopsy

92
Q

What is neuroblastoma?

A

Tumour of the neural crest tissue found in the adrenal medulla or sympathetic chain

93
Q

How does a neuroblastoma typically present?

A

Abdominal mass, weight loss, pallor, hepatomegaly

94
Q

How is neuroblastoma diagnosed?

A

Increased urinary catecholamines

US and MRI

95
Q

What is another name for nephroblastoma?

A

Wilms’ tumour

96
Q

What syndrome is associated with Wilm’s tumour?

A

Denys-Drash

97
Q

What are the symptoms of Wilm’s tumour?

A

Large abdominal mass, anorexia, haematuria, hypertension

98
Q

A soft tissue sarcoma in the head or neck will cause what symptoms?

A

Proptosis
Nasal obstruction
Blood stained nasal discharge

99
Q

A soft tissue sarcoma in the GU tract will cause what symptoms?

A

Dysuria
Urinary obstruction
Blood stained vaginal discharge

100
Q

What are the risk factors for osteosarcoma?

A

13q14 deletion
Paget’s disease
Li-Fraumeni syndrome

101
Q

What type of tumour is Ewing’s sarcoma?

A

Malignant small, round, blue cell tumour

102
Q

What is the main symptom of bone tumours?

A

Persistent bone pain

103
Q

What are the risk factors for retinoblastoma?

A

80% family history - chromosome 13

104
Q

How does retinoblastoma present?

A

Leukocoria - white pupillary reflex

StrabismusRed eye

105
Q

How is retinoblastoma treated?

A

Enucleation of eye
Chemotherapy
Laser therapy
Radiotherapy

106
Q

What laboratory finding indicates germ cell tumour?

A

Increased alpha-FP and bHCG

107
Q

What are the symptoms of type 1 diabetes?

A 
Hyperglycaemia
Ketonuria
Polydipsia and polyuria
Weight loss
Metabolic acidosis with respiratory compensation
108
Q

What is the management of DKA?

A

High flow oxygen
Correct hypovolaemic shock with normal saline
Commence IV insulin infusion
Close monitoring of Serum glucose, Na, K, and cerebral oedema

109
Q

What are the symptoms of DKA?

A 
Vomiting and diarrhoea
Abdominal pain
Pear drop odour to breath
Anorexia and fatigue
Kussmaul respiration (deep sighing breathing)
110
Q

What are the risk factors for DKA?

A

Infection

Poorly controlled diabetes

111
Q

What laboratory findings would you see with metabolic acidosis?

A

pH<7.3, HCO3 <15

112
Q

What would you find on urine dipstick in newly presenting diabetes?

A

Glucose and ketones

113
Q

How does the breathing assessment differ in basic life support between adults and children?

A

In children you do 5 rescue breaths if the child is not breathing (not adults)

114
Q

What is the required rate of chest compressions in paediatric life support?

A

100-120 a minute at 1/3 the depth of the chest

115
Q

Give three possible causes of left hip pain in a 5 year old boy.

A

Transient synovitis
Septic arthritis
Perthes’ disease
Malignancy

116
Q

What investigations are needed in a child with a limp?

A

X-Ray
Bloods - FBC, CRP
Joint aspirate if febrile

117
Q

What is the treatment of bone marrow failure in ALL?

A

Granulocyte colony stimulating factor - stimulates bone marrow to produce RBC

118
Q

What is the treatment of febrile neutropenia in ALL?

A

Tazocin/teicoplanin

119
Q

What is tumour lysis syndrome?

A

Renail failure secondary to increased levels of urate, phosphate, and potassium at initiation of chemotherapy

120
Q

What is the treatment of tumour lysis syndrome?

A

Hyperhydration
Rasburicase
Dialysis

121
Q

Define neonate

A

A baby up to 28 days of age

122
Q

What is the average age of diagnosis of retinoblastoma?

A

18 months

123
Q

What is the further management of breech babies if their newborn examination is normal?

A

Ultrasound at 6 weeks to check for DDH (regardless of mode of delivery)

124
Q

What are the characteristics of pain in Osgood Schlatter disease?

A 
Located to the tibial tuberosity
Usually unilateral
Gradual and intermittent in onset, but can progress to be severe and continuous
Relieved by rest
Made worse by kneeling and jumping
125
Q

What is the inheritance pattern in achondroplasia?

A

Autosomal dominant

But 70% are sporadic

126
Q

What are the signs of achondroplasia?

A

Macrocephaly with frontal bossing
Trident hand
Lumbar lordosis
Short limbs and fingers

Phase 3a (18 decks)

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