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fourth year > paeds > Flashcards

paeds Flashcards

1
Q

what are the three fetal shunts

A

ductus venosus, foramen ovale, and ductus arteriosus

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2
Q

ductus venousus shunts

A

from the umbilical vein to the IVC to bypass the liver

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3
Q

foramen ovale shunts

A

right atrium to left atrium to bypass right ventricle and pulmonary circulation

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4
Q

ductus arteriosus shunts

A

the pulmonary artery with the aorta to bypass pulmonary circulation

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5
Q

why does the foramen ovale close?

A

first breath expands alveoli decreasing pulmonary vascular resistance causes pressure to fall in the right atrium which squashes the atrial septum.

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6
Q

foramen ovale after shutting gradually becomes the

A

fossa ovalis

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7
Q

what is required to keep the ductus arteriosus open?

A

prostaglandins

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8
Q

why does the ductus arteriosus close after first breath

A

increased blood oxygen decreases circulating prostaglandins

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9
Q

after closing the ductus arteriosus forms

A

ligamentum arteriosum

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10
Q

ductus venosus closes because

A

of umbilical cord clamping

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11
Q

ductus venosus becomes

A

ligamentum venosum

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12
Q

innocent murmurs are

A

systolic flow murmurs

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13
Q

features of an innocent murmur are

A

soft, short, systolic, asymptomatic, and situation dependent.

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14
Q

red flags for a murmur are

A

loud, diastolic, louder on standing or systemic features.

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15
Q

investigations for murmurs are

A

ECG, CXR and echo

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16
Q

pan systolic murmur examples are

A

mitral regurgitation, tricuspid regurgitation, and ventricular septal defects

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17
Q

ventricular septal defects are heard at the

A

lower left sternal border

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18
Q

ejection systolic murmurs examples

A

aortic stenosis, pulmonary stenosis and hypertrophic obstructive cardiomyopathy

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19
Q

hypertrophic obstructive cardiomyopathy is heard at

A

fourth intercostal space on the left sternal border

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20
Q

negative thoracic breathing during inspiration causes

A

physiological splitting of the heart sound

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21
Q

atrial septal defect murmur

A

mid systolic crescendo decrecscendo murmur with a fixed split second heart sound

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22
Q

patent ductus arteriosus murmur

A

continous crescendo-decrescendo machinery murmur

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23
Q

tetralogy of fallot murmur

A

ejection systolic murmur due to pulmonary stenosis

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24
Q

cyanotic heart disease pathology

A

deoxygenated blood in systemic circulation. occurs with a right to left shunt.

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25
Q

heart defects that cause right to left shunt (cyanotic heart disease)

A

VSD, ASD, PDA, and transposition of the great arteries

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26
Q

risk factors for patent ductus arteriosus (PDA)

A

rubella or prematurity

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27
Q

left to right shunt increases pressure in the

A

pulmonary hypertension and causes right heart strain due to increases resistance leading to hypertrophy.

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28
Q

presentation of PDA

A

SOB, difficulty feeding, poor weight gain, lower respiratory tract infection

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29
Q

management of PDA

A

monitor with ECHO until 1 as may close conservatively.

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30
Q

surgical management of PDA

A

trans-catheter or surgical closure.

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31
Q

the separation of the upper chamber of the heart (the atria) during development is by the

A

two walls that form with the endocardial cushion

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32
Q

the name of the two walls that separate the atria are

A

septum primum and septum secondum

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33
Q

pulmonary hypertension may lead to what syndrome

A

Eisenmenger syndrome

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34
Q

most common atrial septal defect is

A

ostium secondum

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35
Q

types of atrial septal defect are

A

ostium secondum, patent foramen ovale, ostium primum

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36
Q

27 year old male develops a DVT after post operation that causes a massive stroke but as to the cause, no one is sure. what may you suspect?

A

lifelong asymptomatic ASD

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37
Q

complications of ASD?

A

stroke, atrial fibrillation, pulmonary hypertension with right sided heart failure, and eisenmenger syndrome.

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38
Q

presention of ASD in adulthood are

A

dyspnoea, heart failure or stroke

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39
Q

ASD presentation in childhood

A

asymptomatic, Shortness of breath
Difficulty feeding
Poor weight gain
Lower respiratory tract infections

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40
Q

management of ASD is

A

conservative, transverse catheter closeure via femoral vein, open heart surgery or anticoagulants

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41
Q

ventricular septal defects genetic associations

A

down’s syndrome and turner’s syndrome

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42
Q

symptoms of VSD are

A 
initially asymptomatic but Poor feeding
Dyspnoea
Tachypnoea
Poor feeding
Failure to thrive
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43
Q

VSD may be corrected by

A

transvenous catheter closure via femoral vein or open heart surgery

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44
Q

risk of what infection is likely with a VSD?

A

infective endocarditis,

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45
Q

bone marrow response to cyanosis in eisenmenger’s syndrome

A

production of more RBS leading to polycythaemia causing a plethoric complexion.

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46
Q

complication of polycythaemia?

A

increases likelihood of clots.

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47
Q

pulmonary hypertension examination signs

A

raised JVP, right ventricular heave, loud P2, peripheral oedema

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48
Q

right to left shunt and chronic hypoxia findings

A

Cyanosis
Clubbing
Dyspnoea
Plethoric complexion

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49
Q

mortality prognosis in eisenmenger syndrome

A

reduced life expectancy of 20 years

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50
Q

can you medically reverse eisenmenger syndrome?

A

no

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51
Q

treatment of eisenmenger syndrome could be through a

A

heart and lung transplant

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52
Q

eisenmenger syndrome pulmonary hypertension mx

A

sildenafil

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53
Q

eisenmenger syndrome mx of polycythaemia

A

venesection

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54
Q

eisenmenger syndrome mx of infective endocarditis

A

prophylactic antibiotics

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55
Q

aortic coarctation is related to

A

turner’s syndrome

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56
Q

only indication of aortic coarctation may be

A

weak femoral pulses

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57
Q

investigation of coarctation may be through

A

four limb blood pressure, high blood pressure will arise from the limbs supplied before the narrowing

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58
Q

signs of coarctation

A

systolic murmur below left clavicle, tachypnoea, poor feeding and grey/floppy baby

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59
Q

later in life signs of coarctation

A

left ventricular heave, underdeveloped limbs

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60
Q

management of coarctation

A

critical care require prostaglandin with surgery to ligate the ductus arteriosus and correct the coarctation.

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61
Q

the aortic valve is called

A

the three leaflets are called the aortic sinuses of valsalva

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62
Q

significant aortic stenosis is causes

A

fatigue, shortness of breath, dizziness and fainting. worse on exertion and likely to present with heart failure.

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63
Q

aortic stenosis ejection systolic murmur may radiate to the

A

carotids

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64
Q

examination signs of aortic stenosis also includes

A

ejection click before the murmur, palpable thrill and slow rising pulse with narrow pulse pressure.

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65
Q

gold standard investigation for aortic stenosis is

A

ECHO

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66
Q

additional Ix for aortic stenosis is

A

ECGs and exercise testing

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67
Q

treatment for aortic stenosis includes

A

Percutaneous balloon aortic valvoplasty
Surgical aortic valvotomy
Valve replacement

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68
Q

complication of aortic stenosis is

A 
Left ventricular outflow tract obstruction
Heart failure
Ventricular arrhythmia
Bacterial endocarditis
Sudden death
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69
Q

pulmonary valve consists of how many leaflets?

A

three

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70
Q

pulmonary valve stenosis is associated with

A

Tetralogy of Fallot
William syndrome
Noonan syndrome
Congenital rubella syndrome

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71
Q

presentation of pulmonary valve stenosis

A

symptoms of fatigue on exertion, shortness of breath, dizziness and fainting.

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72
Q

signs of pulmonary stenosis includes

A

ejection systolic murmur, palpable thrill, right ventricular heave and raised JVP with giant a waves.

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73
Q

symptomatic pulmonary stenosis treatment is through

A

balloon valvuloplasty via venous catheter via femoral vein or open heart surgery.

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74
Q

tetralogy of fallot consists of

A

Ventricular septal defect (VSD)
Overriding aorta
Pulmonary valve stenosis
Right ventricular hypertrophy

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75
Q

risk factors for tetralogy of fallot

A

Rubella infection
Increased age of the mother (over 40 years)
Alcohol consumption in pregnancy
Diabetic mother

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76
Q

CXR may show tetralogy of fallot as

A

a boot shaped heart

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77
Q

severe cases of tetralogy of fallot will cause what before one year?

A

heart failure

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78
Q

signs and symptoms of tetralogy of fallot

A 
Cyanosis 
Clubbing
Poor feeding
Poor weight gain
Ejection systolic murmur 
“Tet spells”
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79
Q

tet spells refer too

A

temporary worsening of right to left shunt causing a cyanotic episodes

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80
Q

tet spells pathology are

A

pulmonary vascular resistance increasing or systemic resistance decreasing due to presence of carbon dioxide

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81
Q

why does carbon dioxide causes a decrease in systemic vascular resistance?

A

vasodilator.

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82
Q

tet episodes may be triggered by?

A

waking, physical exertion or crying

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83
Q

tet spells initial management

A

squatting to increase systemic pressure

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84
Q

medical management of tet spell

A

oxygen, beta blockers, IV fluids, morphine, sodium carbonate and phenylephrine infusion.

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85
Q

management of tetralogy of fallot

A

prostaglandin infusion to maintain ductus arteriosus, total surgical repair or open heart surgery.

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86
Q

ebsteins anomaly refers too

A

lower tricuspid valve in right side of heart.

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87
Q

anatomically ebstein’s anomaly causes

A

large right atrium but small right ventricle

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88
Q

ebsteins anomaly is related to what syndrome

A

wolff-parkinson-white syndrome

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89
Q

presentation of ebstein’s anomaly is

A 
Evidence of heart failure (e.g. oedema)
Gallop rhythm
Cyanosis
Shortness of breath and tachypnoea
Poor feeding
Collapse or cardiac arrest
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90
Q

ECG findings for ebsteins anomaly includes

A

Arrhythmias
Right atrial enlargement
Right bundle branch block
Left axis deviation

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91
Q

medical management of ebsteins anomaly is for

A

treating arrhythmias and heart failure

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92
Q

transposition of the great vessels anatomically means?

A

RV pumps into aorta and LV pumps into pulmonary vessels

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93
Q

immediate survival of the baby with transposition of great vessels is through

A

patent ductus arteriosus, atrial septal defect or ventricular septal defect.

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94
Q

transposition of the great vessels is usually identified through

A

antenatal ultrasound scans

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95
Q

how may a balloon septostomy assist in transposition of great vessels.

A

via umbilicus a catheter can open a atrial septal defect

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96
Q

definitive management of transposition of great vessels is

A

open heart surgery

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97
Q

type 1 diabetes may be triggered by what viruses

A

Coxsackie B virus and enterovirus.

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98
Q

functions of insulin are

A

enables glucose entry to the cell, and enable muscle, livers cells to produce glycogen.

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99
Q

insulin is an example of what sort of hormone

A

anabolic

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100
Q

insulin is produced by

A

pancreas, beta cells in the islets of langerhan

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101
Q

glucagon is an example of what sort of hormone

A

catabolic

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102
Q

glucagon is produced by

A

alpha cells in the islets of langerhan in the pancreas.

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103
Q

glucagon function is

A

liver to breakdown glycogen via glycogenolysis, and for the liver to convert fats into glucose through gluconeogenesis

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104
Q

ketogenesis occurs through

A

liver using fatty acids to create water soluble fatty acids

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105
Q

people in ketosis have what smell in their breath?

A

acetone.

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106
Q

triad of hyperglycaemia is

A

polyuria, polydispia and weight loss.

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107
Q

other symptoms of hyperglycaemia are

A

secondary enuresis and recurrent infections.

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108
Q

diagnosis of type 1 diabetes should involve

A

FBC, renal profile and formal lab glucose, and HbA1c.

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109
Q

additional tests to consider with a type 1 diabetic

A

blood cultures for infection, thyroid function tests (autoimmune thyroid disease), coeliac disease and insulin antibodies.

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110
Q

insulin injections in the same spot may cause

A

lipodystrophy

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111
Q

patients with type 1 diabetes usually started on

A

basal bolus regime

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112
Q

example of basal is

A

long acting insulin lantus usually given in the evening

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113
Q

example of bolus is

A

short acting insulin actrapid, usually three times a day according to carb intake.

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114
Q

two types of insulin pump are

A

tethered or patch

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115
Q

hypoglycaemia symptoms include

A

unger, tremor, sweating, irritability, dizziness and pallor. More severe hypoglycaemia will lead to reduced consciousness, coma and death

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116
Q

first line for hypoglycaemia is

A

lucozade

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117
Q

severe hypoglycaemia consider

A

IV dextrose and intramuscular glucagon.

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118
Q

differentials for a hypoglycaemia episode

A

hypothyroidism, glycogen storage disorders, growth hormone deficiency, liver cirrhosis, alcohol and fatty acid oxidation defects

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119
Q

common complication of type 1 diabetes in kids are

A

nocturnal hypoglycaemia

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120
Q

triad of long term complications of chronic hyperglycaemia

A

macrovascular, microvascular and infection

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121
Q

macrovascular complications of hyperglycaemia

A

coronary artery disease, diabetic foot, stroke, hypertension.

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122
Q

microvascular complications of hyperglycaemia

A

peripheral neuropathy, retinopathy, kidney disease (glomerulosclerosis)

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123
Q

monitoring of blood sugars can be through

A

Hb1Ac every 3-6 months, capillary glucose or FreeStyle libre.

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124
Q

triad of outcome of diabetic ketoacidosis

A

ketoacidosis, dehydration and potassium imbalance

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125
Q

pathology of acidosis in DKA

A

ketones increase acid levels, bicarbonate before but eventually run out

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126
Q

pathology of dehydration in DKA

A

glucose leaks into urine, osmotic diuresis draws water into the urine causing polyuria and results in dehydration

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127
Q

potassium imbalance pathology in DKA

A

insulin normally drives potassium into cells. leads to arrhythmias.

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128
Q

priority of treatment in DKA is

A

fluid resus followed by insulin infusion.

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129
Q

DKA complication of treatment is

A

cerebral oedema

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130
Q

pathology of cerebral oedema via DKA tx

A

fluid moves to extracellular space in DKA. fluids and insulin cause rapid shift of fluid into intracellular space causing cells to swell.

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131
Q

signs of cerebral oedema

A

headaches, altered behaviour, bradycardia or changes to consciousness.

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132
Q

management of cerebral oedema in DKA tx

A

slowing IV fluids, IV mannitol and IV hypertonic saline.

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133
Q

symptoms of DKA

A 
Polyuria
Polydipsia
Nausea and vomiting
Weight loss
Acetone smell to their breath
Dehydration and subsequent hypotension
Altered consciousness
sepsis
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134
Q

diagnosis of DKA is

A

Hyperglycaemia (i.e. blood glucose > 11 mmol/l)
Ketosis (i.e. blood ketones > 3 mmol/l)
Acidosis (i.e. pH < 7.3)

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135
Q

rehydration of DKA should occur over

A

evenly over 48 hours

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136
Q

principles of treatment for DKA

A

avoid bolus, treat underlying triggers, avoid hypoglycaemia, add potassium to fluids, monitor for cerebral oedema.

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137
Q

adrenal insufficiency refers too when

A

adrenal glands do not produce enough steroids particularly cortisol and aldosterone.

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138
Q

addison’s disease refers too

A

primary adrenal insufficiency of cortisol and aldosterone

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139
Q

common cause of addison’s disease is

A

autoimmune

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140
Q

secondary adrenal insufficiency refers too

A

inadequate ACTH

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141
Q

causes of secondary adrenal insufficiency includes

A

damage to pituitary, congenital, infection, surgery, RT or loss of blood.

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142
Q

tertiary adrenal insufficiency refers too

A

inadequate CRH release by the hypothalamus

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143
Q

cause of tertiary adrenal insufficiency is

A

sudden withdrawal or exogenous long term steroids (>3 weeks)

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144
Q

features of adrenal insufficiency in babies

A 
Lethargy
Vomiting
Poor feeding
Hypoglycaemia
Jaundice
Failure to thrive
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145
Q

features of adrenal insufficiency in children

A 
Nausea and vomiting
Poor weight gain or weight loss
Reduced appetite (anorexia)
Abdominal pain
Muscle weakness or cramps
Developmental delay or poor academic performance
Bronze hyperpigmentation
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146
Q

investigations for adrenal insufficiency

A

U&Es (hyponatraemia and hyperkalaemia) and blood glucose (hypoglycaemia) as well as cortisol, ACTH, aldosterone and renin levels

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147
Q

addison’s disease specific hormone levels

A

Low cortisol
High ACTH
Low aldosterone
High renin

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148
Q

secondary adrenal insufficiency hormone levels.

A

Low cortisol
Low ACTH
Normal aldosterone
Normal renin

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149
Q

what specific test can be performed to confirm adrenal insufficiency

A

short synacthen test

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150
Q

short synacthen testing involves

A

baseline cortisol measure, injection then measure 30 and 60 minutes post.

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151
Q

short synacthen test is a failure when

A

cortisol fails to rise less than double the baseline confirming addison’s disease.

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152
Q

glucocorticoid replacement for adrenal insufficiency is

A

hydrocortisone (cortisol)

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153
Q

mineralcorticoid hormone replacement for adrenal insufficiency is

A

fludrocortisone (aldosterone)

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154
Q

daily monitoring post adrenal insufficiency involves

A 
Growth and development
Blood pressure
U&amp;Es
Glucose
Bone profile
Vitamin D
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155
Q

sick day rules for adrenal insufficiency involves

A

dose of steroid increased and more regularly, blood glucose monitoring, vomiting and diarrhoea involves IM injection of steroid likely.

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156
Q

Addisonian crisis presentation

A

Reduced consciousness
Hypotension
Hypoglycaemia, hyponatraemia and hyperkalaemia

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157
Q

management of addisonian crisis involves

A

I.V. hydrocortisone, intense monitoring of electrolytes, fluid and blood sugars with I.V. resus.

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158
Q

congenital adrenal hyperplasia is the deficiency in the

A

21-hydroxylase enzyme

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159
Q

congenital adrenal hyperplasia effects hormones how?

A

nderproduction of cortisol and aldosterone and overproduction of androgens

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160
Q

congenital adrenal hyperplasia genetic pattern is

A

autosomal recessive

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161
Q

mineralcorticoid hormone is released in response to what and for what function?

A

renin, to control the balance of salt and water in the blood

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162
Q

aldosterone acts on the kidney to

A

increase sodium reabsorption into the blood and increase potassium secretion into the urine. Therefore, aldosterone acts to increase sodium and decrease potassium in the blood.

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163
Q

21-hydroxylase is the enzyme responsible for

A

converting progesterone into aldosterone and cortisol.

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164
Q

growth hormone stimulates the release of

A

insulin-like growth factor 1 (IGF-1) by the liver,

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165
Q

empty sella syndrome refers too

A

under developed pituitary gland.

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166
Q

neonate presentation of growth hormone deficiency

A

Micropenis (in males)
Hypoglycaemia
Severe jaundice

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167
Q

older infants present with what? for GH deficiency

A

Poor growth, usually stopping or severely slowing from age 2-3
Short stature
Slow development of movement and strength
Delayed puberty

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168
Q

growth hormone stimulation test involves measuring the response to

A

glucagon as well as insulin, arginine and clonidine.

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169
Q

other investigations for growth hormone deficiency

A

thyroid and adrenal insufficiency, MRI brain, genetic testing, x-ray or DEXa

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170
Q

associated genetic conditions for GH deficiency include

A

Turner syndrome and Prader–Willi syndrome

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171
Q

Tx for GH deficiency is

A

Daily subcutaneous injections of growth hormone (somatropin)

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172
Q

two causes of congenital hypothyroidism are

A

dysgensis (underdeveloped gland thyroid gland) or dyshormonogenesis.

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173
Q

screening for congenital hypothyroidism is through

A

newborn blood spot screening test.

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174
Q

symptoms of congenital hypothyroidism include

A 
Prolonged neonatal jaundice
Poor feeding
Constipation
Increased sleeping
Reduced activity
Slow growth and development
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175
Q

commonest cause of acquired hypothyroidism is

A

autoimmune thyroiditis known as hashimoto’s thyroiditis

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176
Q

autoimmune thyroiditis/ hashimoto’s thyroiditis pathology

A

antithyroid peroxidase (anti-TPO) and antithyroglobulin antibodies .

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177
Q

hashimoto’s thyroiditis is associated with

A

type 1 diabetes and coeliac disease.

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178
Q

symptoms of hashimoto’s thyroiditis are

A 
Fatigue and low energy
Poor growth
Weight gain
Poor school performance
Constipation
Dry skin and hair loss
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179
Q

follow up investigations of hashimoto’s thyroiditis include

A

thyroid function blood tests (TSH, T3 and T4), thyroid ultrasound and thyroid antibodies.

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180
Q

treatment for hashimoto’s thyroiditis

A

levothyroxine.

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181
Q

commonest cause of bronchiolitis

A

respiratory syncytial virus RSV

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182
Q

bronchiolitis commonly occurs in children aged

A

under 1 year

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183
Q

why does RSV not affect adults in the same way as young kids?

A

proportional size of the airways

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184
Q

presentation of bronchiolitis

A

coryzal symptoms, dyspnoea, tachypnoea, poor feeding, mild fever, apnoeas, wheeze and crackles.

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185
Q

signs of bronchiolitis

A

raised respiratory rate, accessory muscles (SCM, abdominal), intercostal and subcostal recessions, nasal flaring, head bobbing, tracheal tugging, cyanosis and abnormal airway nosies.

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186
Q

wheezing is causes by

A

narrowed airways usually on expiration

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187
Q

grunting is caused by

A

exhaling with a partially closed glottis to increase end expiratory pressure.

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188
Q

stridor is caused by

A

obstruction of upper airway

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189
Q

RSV typical course

A

chesty symptoms day 1-2.
onset of coryzal symptoms with peak being day 3/4.
recovery over week 2 - 3.

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190
Q

reasons to admit for bronchiolitis

A

under 3 months old, prexisitng condition, down’s syndrome, prematurity, respiratory rate >70, oxygen sats <92%, severe respiratory distress.

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191
Q

management of bronchiolitis

A

adequate intake via NG tube or IV fluids, saline nasal drops, supplementary oxygen.

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192
Q

ventilatory support for bronchiolitis includes

A

high flow humidified oxygen, continuous positive airway pressure, intubation and ventilation

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193
Q

why is high flow humidified oxygen helpful outside oxygenation?

A

provides end expiratory pressure.

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194
Q

how can you assess ventilation?

A

rising pCO2, falling pH and respiratory acidosis all signs of poor ventilation.

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195
Q

prevention of bronchiolitis may be managed through?

A

Palivizumab a monoclonal antibody given with monthly injections. passive protection.

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196
Q

who is in the high risk group for RSV?

A

congenital heart disease or premature babies.

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197
Q

viruses that commonly cause a viral induced wheeze?

A

RSV or rhinovirus

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198
Q

Poiseuille’s law dictates

A

hat flow rate is proportional to the radius of the tube to the power of four.

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199
Q

features that differentiate a viral induced wheeze to asthma are

A

before 3 years old, not history of atopy and only occurs during infection.

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200
Q

presentation of viral wheeze is

A

Shortness of breath
Signs of respiratory distress
Expiratory wheeze throughout the chest

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201
Q

focal wheeze indicates

A

focal airway obstruction

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202
Q

acute asthma presentation

A

Progressively worsening shortness of breath
Signs of respiratory distress
Fast respiratory rate (tachypnoea)
Expiratory wheeze on auscultation heard throughout the chest
The chest can sound “tight” on auscultation, with reduced air entry

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203
Q

moderate acute asthma signs

A

peak flow >50% and normal speech

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204
Q

severe acute asthma signs

A

peak flow <50%, sats <92%, unable to complete sentences in one breath, respiratory distress, hear rate (>140 in 1-5yrs, >125>5yrs), RR (>40 1-5yrs, >30 in >5yrs)

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205
Q

life threatening asthma signs

A

peak flow <33%, sats <92%, exhaustion and poor respiratory effort, hypotension, silent chest, cyanosis, altered consciousness.

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206
Q

staples of management of virally induced wheeze or acute asthma are

A

supplementary oxygen, bronchodilators, steroids and antibiotics.

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207
Q

bronchodilators for acute asthma are

A

salbutamol (Beta 2 agonist), ipratropium bromide (anti-muscarinic), IV magnesium sulphate, IV aminophylline

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208
Q

moderate to severe asthma stepwise progression

A
  1. salbutamol
  2. nebuliser salbutamol/ipratropium bromide
  3. oral prednisolone
  4. IV hydrocortisone
  5. IV magnesium sulphate
  6. IV salbutamol
  7. IV aminophylline
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209
Q

salbutomal dosage for moderate to severe asthma

A

10 puffs every 2 hours

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210
Q

oral prednisone loading dose

A

1mg per kg of body weight once a day for 3 days.

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211
Q

high doses of salbutamol should make you consider the levels of

A

potassium as they absorbed into cells

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212
Q

post acute asthma steps to consider

A

Finish the course of steroids if these were started (typically 3 days total)
Provide safety-net information about when to return to hospital or seek help
Provide an individualised written asthma action plan

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213
Q

asthma is a

A

chronic inflammatory airway disease leading to variable airway obstruction.

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214
Q

asthma is one atopic condition, what are other examples, perhaps in the family history?

A

allergies, eczema, asthma, hay fever

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215
Q

presentation of asthma

A

Episodic symptoms intermittent exacerbations
Diurnal variability, t
Dry cough with wheeze and shortness of breath
Typical triggers
Bilateral widespread “polyphonic” wheeze
Symptoms improve with bronchodilator

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216
Q

investigations for asthma may include

A

spirometry, direct bronchial challenge with histamine or methacholine, fractional exhaled nitric oxide, peak flow variability

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217
Q

chronic asthma therapy <5yrs

A
  1. SABA
  2. Add a low dose corticosteroid inhaler or a leukotriene antagonist (i.e. oral montelukast)
  3. Add the other option from step 2.
  4. Refer to a specialist.
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218
Q

medical therapy for chronic asthma 5-12 years

A
  1. Start a SABA as req.
  2. low dose corticosteroid inhaler
  3. LABA
  4. corticosteroid inhaler to a medium dose.
  5. Consider adding:
    Oral leukotriene receptor antagonist (e.g. montelukast)
    Oral theophylline
  6. corticosteroid to a high dose.
  7. Referral to a specialist.
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219
Q

therapy for chronic asthma >12 years

A
  1. SABA
  2. low dose corticosteroid
  3. LABA
  4. increase corticosteroid dose, trial LAMA, monteleukast, theophylline etc.
  5. increase corticosteroid dose.
  6. oral steroids.
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220
Q

does inhaled corticosteroids effect growth?

A

effect dose dependent hence the need for good asthma control and regular reviews

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221
Q

MDI spacer cleaning tips

A

once a month, avoid scrubbing and air dry.

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222
Q

pneumonia on a cxr presents with

A

consolidation

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223
Q

presentation of pneumonia is

A 
Cough (typically wet and productive)
High fever (> 38.5ºC)
Tachypnoea
Tachycardia
Increased work of breathing
Lethargy
Delirium
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224
Q

general signs of pneumonia include

A 
Tachypnoea (raised respiratory rate)
Tachycardia (raised heart rate)
Hypoxia (low oxygen)
Hypotension (shock)
Fever
Confusion
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225
Q

chest signs of pneumonia include

A

bronchial breathing, focal coarse crackles, dullness to percussion

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226
Q

most common cause of bacterial pneumonia

A

streptococcus pneumonia

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227
Q

staphylococcus aureus signs on CXr with pneumonia

A

pneumatocoeles (round air filled cavities) and consolidations in multiple lobes.

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228
Q

what bacteria may effect unvaccinated or pre vaccinated children with pneumonia

A

group B strep and haemophilus influenza

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229
Q

mycoplasma pneumonia presentation of pneumonia

A

extra-pulmonary manifestations (e.g. erythema multiforme).

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230
Q

commonest viral cause of pneumonia include

A

RSV the most common

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231
Q

other viruses that may cause pneumonia

A

parainfluenza, and influenza

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232
Q

ix for pneumonia

A

CXR, sputum cultures, throat swab,viral PCR

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233
Q

Tx for pneumonia first line antibiotic is

A

amoxicillin

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234
Q

what may be used to treat atypical pneumonia or as alternative to penicillin?

A

macrolides

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235
Q

examples of macrolides include

A

erythromycin, clarithromycin or azithromycin

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236
Q

additional tests in recurrent respiratory tract infection

A

sweat test, serum immunoglobulins, IgG, sweat test and HIV, FBC and CXR

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237
Q

croup commonly effects what age group?

A

6 months to 2 years

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238
Q

croup causes

A

barking cough due to oedema in the larynx

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239
Q

classic cause of croup is

A

parainfluenza virus

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240
Q

croup used to be caused by

A

diptheria

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241
Q

diptheria croup can lead to

A

epiglottitis

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242
Q

presentation of croup

A 
Increased work of breathing
“Barking” cough, occurring in clusters of coughing episodes
Hoarse voice
Stridor
Low grade fever
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243
Q

what treatment is very effective for croup

A

single dose 150 mcg/kg of oral dexamethasone.

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244
Q

severe croup may require.

A

Oxygen
Nebulised budesonide
Nebulised adrenalin
Intubation and ventilation

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245
Q

epiglottitis is typically caused by

A

haemophilus influenza type B.

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246
Q

presentation of epiglottis

A 
Patient presenting with a sore throat and stridor
Drooling
Tripod position, sat forward with a hand on each knee
High fever
Difficulty or painful swallowing
Muffled voice
Scared and quiet child
Septic and unwell appearance
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247
Q

lateral X-ray of epiglottis reveals

A

thumbprint sign

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248
Q

treatment of epiglottitis is

A

securing the airway (intubation, tracheostomy, ITU) with IV antibiotics (ceftriaxone) and steroids (dexamethasone)

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249
Q

common complication of epiglottitis is

A

abscess

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250
Q

laryngomalacia is a condition that refers too

A

the supraglottis larynx flops

causing partial airway obstruction

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251
Q

in laryngomalcia the aryepiglottic folds are

A

shortened causing an omega shape

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252
Q

laryngomalacia presentation is with

A

harsh whistling sound; inspiratory stridor

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253
Q

management of laryngomalacia is through

A

child development (grow out of it)

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254
Q

whooping cough is caused by

A

bordetella pertussis

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255
Q

bordella pertussis is what type of bacteria?

A

gram negative

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256
Q

is there a vaccination for pertussis?

A

yes

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257
Q

presentation of pertussis ?

A

mild coryzal symptoms, low grade fever, severe coughing fits post 1 week (paroxysmal cough) with inspiratory whoop post fit.

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258
Q

complications of coughing fits from whooping cough?

A

pneumothorax, faint or vomit. long term risk of bronchiectasis

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259
Q

infants may present differently with pertussis with instead

A

apnoeas

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260
Q

diagnosis of whooping cough is with

A

nasopharyngeal swab, PCR or bacterial culture.

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261
Q

management of whooping cough requires

A

supportive care and notifying public health. macrolide antibiotics and prophylactic antibiotics for contacts.

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262
Q

chronic lung disease of prematurity is also known as

A

bronchopulmonary dysplasia

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263
Q

chronic lung disease of prematurity occurs usually with those born before

A

28 weeks gestation

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264
Q

those born with chronic lung disease of prematurity usually require

A

oxygen therapy or intubation and ventilation at birth

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265
Q

Dx of chronic lung disease of prematurity

A

CXR

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266
Q

chronic lung disease of prematurity features

A 
Low oxygen saturations
Increased work of breathing
Poor feeding and weight gain
Crackles and wheezes on chest auscultation
Increased susceptibility to infection
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267
Q

chronic lung disease of prematurity prevention is through

A

corticosteroids prior to birth, post caffeine, not over oxygenating and CPAP

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268
Q

CF is what sort of genetic condition?

A

autosomal recessive.

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269
Q

CF is a mutation of the

A

cystic fibrosis transmembrane conductance regulatory gene on chromosome 7

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270
Q

CF common mutation is the

A

delta-F508

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271
Q

CFTR gene in Cf codes for

A

cellular channels, particularly a type of chloride channel

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272
Q

fraction for carriers are

A

1/25

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273
Q

fraction of kids born with CF is

A

1/2500

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274
Q

consequences of CF includes

A

Thick pancreatic and biliary secretions, Low volume thick airway secretions, Congenital bilateral absence of the vas deferens

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275
Q

what pancreatic enzyme is missing in CF

A

pancreatic lipase

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276
Q

both parents are healthy, one sibling has cystic fibrosis and a second child does not have the disease, what is the likelihood of the second child being a carrier?

A

two in three.

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277
Q

CF is screened for at birth using

A

newborn bloodspot test.

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278
Q

first sign of CF is

A

Meconium ileus (thick and sticky bowel obstruction)

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279
Q

symptoms of CF are

A 
Chronic cough
Thick sputum production
Recurrent respiratory tract infections
 (steatorrhoea)  
Abdominal pain and bloating
 child tastes particularly salty  sweat
(failure to thrive)
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280
Q

signs of CF

A 
Low weight or height on growth charts
Nasal polyps
Finger clubbing
Crackles and wheezes on auscultation
Abdominal distention
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281
Q

during pregnancy CF may be tested for through

A

amniocentesis or chorionic villous sampling

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282
Q

sweat test requires

A

pilocarpine application and electrodes, then measure chloride concentration.

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283
Q

sweat test diagnostic point

A

> 60 mmol/l

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284
Q

key colonisers in CF is

A

staph aureus and pseudomonas

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285
Q

Staph aureus in CF is prevented with

A

prophylactic flucloxacillin

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286
Q

Pseudomonas in Cf can be attempted to treat with

A

long term nebulised antibiotics such as tobramycin or Oral ciprofloxacin

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287
Q

management of Cf includes

A

Chest PT, exercise, high calorie diet, CREON tablets (enzyme replacement), flucloxacillin, bronchodilators, nebulised DNase and hypertonic saline and vaccinations

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288
Q

other treatments for Cf include

A

organ transplant, fertility treatment and genetic counselling.

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289
Q

patients with CF may require monitoring for

A

diabetes, osteoporosis, vitamin D deficiency and liver failure.

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290
Q

life expectancy median in Cf is

A

47 years

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291
Q

kartagner’s syndrome refers too

A

primary ciliary dyskinesia

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292
Q

primary ciliary dyskinesia genetic spread is

A

autosomal recessive

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293
Q

key risk factor for primary ciliary dyskinesia is

A

consanguinity

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294
Q

kartagner’s triad is

A

Paranasal sinusitis
Bronchiectasis
Situs Inversus

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295
Q

situs inversus refers too

A

internal (visceral) organs are mirrored inside the body

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296
Q

when only the heart is reversed it is called

A

dextrocardia

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297
Q

Dx of primary ciliary dyskinesia is

A

sample of the ciliated epithelium via nasal brushing or bronchoscopy.

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298
Q

Mx of primary ciliary dyskinesia is through

A

daily physiotherapy, a high calorie diet and antibiotics.

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299
Q

medical causes of abdominal pain

A 
Constipation is also very common
Urinary tract infection
Coeliac disease
Inflammatory bowel disease
Irritable bowel syndrome
Mesenteric adenitis
Abdominal migraine
Pyelonephritis
Henoch-Schonlein purpura
Tonsilitis
Diabetic ketoacidosis
Infantile colic
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300
Q

common causes of abdominal pain in girls

A 
Dysmenorrhea (period pain)
Mittelschmerz (ovulation pain)
Ectopic pregnancy
Pelvic inflammatory disease
Ovarian torsion
Pregnancy
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301
Q

surgical causes of pain in kids

A

appendicitis
intussuception
bowel obstruction
testicular torsion

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302
Q

red flags of abdominal pain

A 
Persistent or bilious vomiting
Severe chronic diarrhoea
Fever
Rectal bleeding
Weight loss or faltering growth
Dysphagia (difficulty swallowing)
Nighttime pain
Abdominal tenderness
303
Q

abdominal migraine refers too

A

episodes of central abdominal pain lasting more than 1 hour but with normal examination

304
Q

abdominal migraine presentation

A 
Nausea and vomiting
Anorexia
Pallor
Headache
Photophobia
Aura
305
Q

treatment of acute abdominal migraine

A

Low stimulus environment (quiet, dark room)
Paracetamol
Ibuprofen
Sumatriptan

306
Q

main preventative drug for abdominal migraine is

A

Pizotifen

307
Q

Pizotifen is

A

serotonin agonist

308
Q

secondary causes of constipation include

A

Hirschsprung’s disease, cystic fibrosis or hypothyroidism.

309
Q

encopresis refers too

A

faecal incontinence

310
Q

signs of constipation

A

abdominal pain, overflow soiling, straining painful stool and retentive posturing.

311
Q

faecal incontinence is no pathological until over the age of

A

4 years

312
Q

faecal impaction (rectally retained faeces) leads too

A

desensitisation of the rectum.

313
Q

red flags for constipation

A

not passing meconium, neuro signs, vomiting, ribbon stools, abnormal anus or buttocks, failure to thrive or pain and bloating.

314
Q

nice recommends for constipation

A

laxatives, high fibre diet, hydration, disimpaction regime and bowel diary.

315
Q

first line laxative in constipation is

A

movicol

316
Q

signs of a problematic reflux include

A 
Chronic cough
Hoarse cry
Distress, crying or unsettled after feeding
Reluctance to feed
Pneumonia
Poor weight gain
317
Q

red flags for vomiting include

A

not keeping down any feed, projectile vomiting, bile stained, haematemesis, abdominal distension, reduced consciousness, respiratory symptoms, blood in stools, rash, angiodema, signs of allergy, apnoeas and infection.

318
Q

practical advice for reflux is

A

Small, frequent meals
Burping regularly to help milk settle
Not over-feeding
Keep the baby upright after feeding

319
Q

problematic cases of reflux can be treated with

A

Gaviscon mixed with feeds
Thickened milk or formula (specific anti-reflux formulas are available)
Ranitidine
Omeprazole

320
Q

severe causes of reflux may require

A

barium meal and endoscopy and rarely Surgical fundoplication

321
Q

Sandifer’s Syndrome refers too

A

brief episodes of abnormal movements associated with gastro-oesophageal reflux

322
Q

key features of sandifer’s syndrome is

A

torticollis and dystonia

323
Q

torticollis refers too

A

forceful contraction of the neck muscles causing twisting of the neck

324
Q

dystonia refers too

A

abnormal muscle contractions causing twisting movements, arching of the back or unusual postures

325
Q

West syndrome refers too

A

infantile spasms

326
Q

features of pyloric stenosis are

A

failure to thrive, projectile vomiting, firm mass “large olive”, visible peristalsis

327
Q

blood gas analysis of pyloric stenosis will reveal

A

hypochloric (low chloride) metabolic alkalosis

328
Q

Dx of pyloric stenosis is through

A

abdominal US

329
Q

treatment of pyloric stenosis is

A

laparoscopic pyloromyotomy (known as “Ramstedt’s operation“)

330
Q

viral causes of diarrhoea include

A

rotavirus and norovirus

331
Q

what virus causes subacute diarrhoea?

A

adenovirus

332
Q

what bacteria produces shiga toxin

A

E. coli 0157 and shigella

333
Q

shiga toxin from E. coli 0157 may cause

A

haemolytic uraemic syndrome

334
Q

commonest cause of traveller’s diarhroea is

A

campylobacter jejuni

335
Q

campylobacter is what sort of bacteria?

A

curved or spiral gram negative

336
Q

spread of campylobacter is through

A

Raw or improperly cooked poultry
Untreated water
Unpasteurised milk

337
Q

symptoms of campylobacter is

A

Abdominal cramps
Diarrhoea often with blood
Vomiting
Fever

338
Q

incubation of campylobacter is

A

2-5 days

339
Q

antibiotic choices for campylobacter are

A

azithromycin or ciprofloxacin.

340
Q

antibiotic treatment for shigella is

A

azithromycin or ciprofloxacin.

341
Q

shigella causes

A

bloody diarrhoea, abdominal cramps and fever for usually 1 week

342
Q

salmonella incubation is for

A

12 hours to 3 days

343
Q

symptoms of salmonella is

A

diarrhoea that can be associated with mucus or blood, abdominal pain and vomiting.

344
Q

severe cases of salmonella treatment should be guided by

A

stool culture

345
Q

patient develops symptoms of gastroenteritis soon after eating leftover fried rice that has been left at room temperature. It has a short incubation period after eating the rice before symptoms occur, and they recover within 24 hours. what was the causitive bacteria?

A

bacillus cereus

346
Q

bacillus cereus type of bacteria?

A

gram positive rod

347
Q

bacillus cereus toxin is called

A

cereulide

348
Q

symptoms of bacillus cereus are

A

abdominal cramping and vomiting within 5 hours of ingestion and watery diarrhoea

349
Q

Yersinia is an example of what sort of bacteria?

A

gram negative bacillus

350
Q

what animal is a key carrier of yersinia

A

pigs

351
Q

yersinia symptoms in children are

A

watery or bloody diarrhoea, abdominal pain, fever and lymphadenopathy.

352
Q

older children with yersinia entercolitica may present with

A

ight sided abdominal pain due mesenteric lymphadenitis (inflammation in the intestinal lymph nodes) and fever.

353
Q

Staph aureus can produce what type of toxin?

A

enterotoxin.

354
Q

giardia lamblia is a

A

microscopic parasite

355
Q

giardia lamblia lives in the

A

small intestine of mammals

356
Q

giardia lamblia spreads via the

A

faecal oral transmission

357
Q

diagnosis and treatment of giardia lamblia is via

A

stool microscopy and metronidazole.

358
Q

principles of gastroenteritis management

A

barrier nursing, infection control, fluid challenge or rehydration solutions

359
Q

post gastroenteritis complications include

A

Lactose intolerance
Irritable bowel syndrome
Reactive arthritis
Guillain–Barré syndrome

360
Q

coeliac disease refers to

A

an autoimmune condition triggered by exposure to gluten

361
Q

coeliac auto antibodies target

A

epithelial cells of the intestine

362
Q

key coeliac antibodies to remember are

A

anti-tissue transglutaminase (anti-TTG) and anti-endomysial (anti-EMA).

363
Q

coeliac disease particularly causes atrophy and inflammation of the

A

intestinal villi of the jejunum

364
Q

symptoms of coeliac disease may include

A 
Failure to thrive in young children
Diarrhoea
Fatigue
Weight loss
Mouth ulcers
Anaemia secondary to iron, B12 or folate deficiency
Dermatitis herpetiformis
365
Q

genetic association with coeliac disease is

A

HLA-DQ2 gene

366
Q

what type of antibody is anti-TTG and anti-EMA

A

IgA

367
Q

diagnostic gold standard of coeliac disease is through

A

biopsy of the jejenum revealing crypt hypertrophy and villous atrophy

368
Q

features of Crohn’s disease

A

N – No blood or mucus (these are less common in Crohns.)
E – Entire GI tract
S – “Skip lesions” on endoscopy
T – Terminal ileum most affected and Transmural (full thickness) inflammation
S – Smoking is a risk factor (don’t set the nest on fire)

369
Q

features of ulcerative colitis

A 
C – Continuous inflammation
L – Limited to colon and rectum
O – Only superficial mucosa affected
S – Smoking is protective
E – Excrete blood and mucus
U – Use aminosalicylates
P – Primary sclerosing cholangitis
370
Q

presentation of IBD is

A

perfuse diarrhoea, abdominal pain, bleeding, weight loss or anaemia. They may be systemically unwell during flares, with fevers, malaise and dehydration.

371
Q

extra intestinal manifestations of IBD include

A 
Finger clubbing
Erythema nodosum
Pyoderma gangrenosum
Episcleritis and iritis
Inflammatory arthritis
Primary sclerosing cholangitis (ulcerative colitis)
372
Q

testing for IBD includes

A

faecal calprotectin, endoscopy, CT, MRI, biopsy (gold standard) and CRP with FBC, U+E, TSH and LFT

373
Q

first line for inducing remission for crohn’s is through

A

steroids

374
Q

Crohn’s specialist treatment for inducing remission is through

A 
Azathioprine
Mercaptopurine
Methotrexate
Infliximab
Adalimumab
375
Q

maintaining remission for crohn’s disease is through first line

A

Azathioprine

Mercaptopurine

376
Q

surgical options for crohn’s disease

A

resect if limited to distal ileum, or treat strictures and fistulae’s.

377
Q

inducing remission for ulcerative colitis is through first line

A

aminosalicylate (e.g. mesalazine oral or rectal)

378
Q

second line for mild UC remission treatment

A

corticosteroids (e.g. prednisolone)

379
Q

maintaining remission in UC is through

A

Aminosalicylate (e.g. mesalazine oral or rectal)
Azathioprine
Mercaptopurine

380
Q

surgical options for UC include

A

panproctocolectomy with permanent ileostomy or ileo-anal anastomosis (J-pouch)

381
Q

biliary atresia refers too

A

narrow or absent bile duct

382
Q

biliary atresia results in

A

cholestasis

383
Q

biliary atresia prevents the excretion of

A

conjugated bilirubin.

384
Q

biliary atresia presents with

A

significant jaundice due to high conjugated bilirubin levels

385
Q

suspect biliary atresia in children with

A

babies with a persistent jaundice, lasting more than 14 days in term babies and 21 days in premature babies

386
Q

initial investigation for biliary atresia is with

A

is conjugated and unconjugated bilirubin.

387
Q

majority of cases with neonatal jaundice are due to

A

benign breast milk jaundice

388
Q

management of biliary atresia is through

A

“Kasai portoenterostomy

389
Q

Kasai portoenterostomy refers too

A

attaching a section of the small intestine to the opening of the liver, where the bile duct normally attaches

390
Q

long term biliary atresia commonly requires

A

full liver transplant

391
Q

presentation of intestinal obstruction

A

Persistent vomiting. This may be bilious, containing bright green bile.
Abdominal pain and distention
Failure to pass stools or wind
Abnormal bowel sounds. These can be high pitched and “tinkling” early in the obstruction and absent later.

392
Q

diagnosis of intestinal obstruction

A

AXR

393
Q

abdominal AXR of obstruction reveals

A

dilated loops of bowel, collapsed loops of bowel distal to the obstruction and absence of air in the rectum.

394
Q

management of intestinal obstruction requires

A

nil by mouth, nasogastric tube to drain the stomach and IV fluids.

395
Q

congenital condition with the absence of bowel and rectal nerves refer too

A

Hirschsprung’s disease

396
Q

myenteric plexus is also known as

A

Auerbach’s plexus

397
Q

myenteric plexus is responsible for

A

peristalsis

398
Q

the key pathophysiology of hirschsprung’s disease is

A

absence of parasympathetic ganglion cells from the myenteric plexus due to failure of migration from proximal to distal gut

399
Q

what occurs to the aganglionic bowel in hirschsprung’s disease

A

constriction.

400
Q

syndromes related to hirschsprung’s disease

A

Downs syndrome
Neurofibromatosis
Waardenburg syndrome
Multiple endocrine neoplasia type II

401
Q

Waardenburg syndrome refers too

A

(a genetic condition causing pale blue eyes, hearing loss and patches of white skin and hair)

402
Q

presentation of hirschprung’s disease is

A 
Delay in passing meconium (more than 24 hours)
Chronic constipation since birth
Abdominal pain and distention
Vomiting
Poor weight gain and failure to thrive
403
Q

hat life threatening complication is there of hirschsprung’s disease?

A

Hirschsprung-Associated Enterocolitis which can lead too toxic megacolon and perforation of the bowel.

404
Q

Hirschsprung-Associated Enterocolitis presents

A

within 2-4 weeks of birth with distension and diarrhoea with blood and septic signs

405
Q

treatment of Hirschsprung-Associated Enterocolitis is

A

antibiotics, fluid resuscitation and decompression

406
Q

diagnosis of Hirschsprung’s disease is through

A

rectal biopsy

407
Q

definitive treatment of Hirschsprung’s is

A

surgical removal of aganglionic section of bowel.

408
Q

intussusception commonly occurs between what ages

A

It typically occurs in infants 6 months to 2 years and is more common in boys.

409
Q

associated conditions with intussusception

A 
Concurrent viral illness
Henoch-Schonlein purpura
Cystic fibrosis
Intestinal polyps
Meckel diverticulum
410
Q

presentation of intussusception

A 
Severe, colicky abdominal pain
Pale, lethargic and unwell child
“Redcurrant jelly stool”
Right upper quadrant mass on palpation. This is described as “sausage-shaped”
Vomiting
Intestinal obstruction
411
Q

diagnosis of intussusception is through

A

US or contrast enema

412
Q

treatment of intussusception is with

A

surgical reduction, or therapeutic enema

413
Q

peak incidence of appendicitis is between ages of

A

10 to 20 years old.

414
Q

key presentation of appendicitis is

A

his typically starts as central abdominal pain, that moves down to the right iliac fossa (RIF)

415
Q

McBurney’s point refers too

A

localised area one third the distance from the anterior superior iliac spine (ASIS) to the umbilicus.

416
Q

appendicitis Rovsing’s sign is

A

palpation of the left iliac fossa causes pain in the RIF)

417
Q

Rebound tenderness and percussion tenderness suggest

A

peritonitis, caused by a ruptured appendix.

418
Q

other symptoms of appendicitis include

A

Loss of appetite (anorexia)

Nausea and vomiting and guarding.

419
Q

diagnosis of appendicitis is through

A

inflammatory markers, CT, US then a diagnostic laparoscopy.

420
Q

differentials of appendicitis

A

ovarian cysts, meckel’s diverticulum, mesenteric adenitis, appendix mass

421
Q

type 1 diabetes may be caused by what viruses?

A

Coxsackie B virus and enterovirus

422
Q

ideal blood glucose is between?

A

between 4.4 and 6.1 mmol/l.

423
Q

Baby UTI presentation?

A 
Fever
Lethargy
Irritability
Vomiting
Poor feeding
Urinary frequency
424
Q

signs and symptoms of UTI in children?

A 
Fever
Abdominal pain, particularly suprapubic pain
Vomiting
Dysuria (painful urination)
Urinary frequency
Incontinence
425
Q

acute pyelonephritis presentation is through

A

A temperature greater than 38°C

Loin pain or tenderness

426
Q

Ix of UTI is through

A

clean catch urine with evidence of leukocytes and nitrites and a Midstream urine sample with culture and sensitivity testing.

427
Q

children under 3 months with a fever should receive

A

IV antibiotics (e.g. ceftriaxone) and have a full septic screen, including blood cultures, bloods and lactate. A lumbar puncture should also be considered.

428
Q

typical antibiotics for a UTI are

A

Trimethoprim
Nitrofurantoin
Cefalexin
Amoxicillin

429
Q

all children under 6 months with a UTI should also receive a

A

US

430
Q

all children with recurrent UTI’s should receive a US within

A

6 weeks

431
Q

damage from recurrent UTI’s should receive what post 4-6 months to asses the damage?

A

DMSA scan

432
Q

DMSA scan requires

A

gamma camera to assess how well the material is taken up by the kidneys. to indicate scarring

433
Q

what condition predisposes to upper UTI’s

A

Vesico-ureteric reflux

434
Q

Vesico-ureteric reflux is diagnosed through

A

micturating cystourethrogram (MCUG).

435
Q

micturating cystourethrogram (MCUG). should be used to investigate recurrent UTI’s in those under the age of

A

<6 months or if FH present of VUR

436
Q

micturating cystourethrogram (MCUG). involves

A

It involves catheterising the child, injecting contrast into the bladder and taking a series of xray films

437
Q

vulvovaginitis presents as

A 
Soreness
Itching
Erythema around the labia
Vaginal discharge
Dysuria (burning or stinging on urination)
Constipation
438
Q

urine dipstick of a vulvovaginitis will show

A

leukocytes but no nitrates

439
Q

nephrotic syndrome occurs when

A

the basement membrane in the glomerulus becomes highly permeable to protein,

440
Q

nephrotic syndrome is common between the ages of

A

of 2 and 5 years.

441
Q

the classic triad of nephrotic syndrome is

A

Low serum albumin
High urine protein content (>3+ protein on urine dipstick)
Oedema

442
Q

other features that appear alongside nephrotic syndrome

A

Deranged lipid profile, with high levels of cholesterol, triglycerides and low density lipoproteins
High blood pressure
Hyper-coagulability

443
Q

most common cause of nephrotic syndrome in children under the age of ten is

A

minimal change disease

444
Q

secondary causes of nephrotic syndrome secondary to intrinsic kidney disease includes

A

Focal segmental glomerulosclerosis

Membranoproliferative glomerulonephritis

445
Q

systemic illnesses that may also cause nephrotic syndrome include

A

Henoch schonlein purpura (HSP)
Diabetes
Infection

446
Q

renal biopsy and microscopy of minimal change disease will show

A

usually no abnormality

447
Q

urinalysis of minimal change disease will usually show

A

small molecular weight proteins and hyaline casts.

448
Q

management of minimal change disease is with

A

corticosteroids

449
Q

2 – 5 year old child with oedema, proteinuria and low albumin, what is the underlying cause?

A

nephrotic syndrome.

450
Q

nephrotic syndrome treatment is with

A

High dose steroids (i.e. prednisolone)
Low salt diet
Diuretics may be used to treat oedema
Albumin infusions may be required in severe hypoalbuminaemia
Antibiotic prophylaxis may be given in severe cases

451
Q

alternative treatment for steroid resistant nephrotic syndrome is

A

ACE inhibitors and immunosuppressants

452
Q

nephritis causes

A

Reduction in kidney function
Haematuria: invisible or visible amounts of blood in the urine
Proteinuria: although less than in nephrotic syndrome

453
Q

commonest causes of nephritis is

A

post-streptococcal glomerulonephritis and IgA nephropathy (Berger’s disease).

454
Q

Post-Streptococcal Glomerulonephritis occurs

A

1 – 3 weeks after a β-haemolytic streptococcus infection, such as tonsillitis caused by Streptococcus pyogenes

455
Q

pathophysiology of post streptococcal glomerulonephritis

A

Immune complexes made up of streptococcal antigens, antibodies and complement proteins get stuck in the glomeruli of the kidney and cause inflammation. This inflammation leads to an acute deterioration in renal function, causing an acute kidney injury.

456
Q

Ix of Post-Streptococcal Glomerulonephritis is with

A

, positive throat swab results and anti-streptolysin antibody titres found on a blood test.

457
Q

majority of patients with post streptococcal glomerulonephritis will recover but some will require

A

They may need treatment with antihypertensive medications and diuretics if they develop complications such as hypertension and oedema.

458
Q

IgA Nephropathy is related too

A

Henoch-Schonlein Purpura, which is an IgA vasculitis

459
Q

IgA nephropathy pathology

A

IgA deposits in the nephrons of the kidney causes inflammation

460
Q

renal biopsy of IgA nephropathy will show

A

“IgA deposits and glomerular mesangial proliferation”.

461
Q

IgA nephropathy usually presents in

A

teenagers or young adults.

462
Q

Tx of IgA nephropathy is with

A

supportive treatment of the renal failure and immunosuppressant medications such as steroids and cyclophosphamide

463
Q

Haemolytic Uraemic Syndrome is triad of

A

Haemolytic anaemia, AKI and thrombocytopenia

464
Q

management of HUS requires

A

Urgent referral to the paediatric renal unit for renal dialysis if required
Antihypertensives if required
Careful maintenance of fluid balance
Blood transfusions if required

465
Q

enuresis refers too as

A

involuntary urination

466
Q

most children develop control of daytime urination by

A

2 years

467
Q

most children develop control of night time urination by

A

3-4 years

468
Q

Primary nocturnal enuresis refers too

A

the child has never managed to be consistently dry at night.

469
Q

causes of primary nocturnal enuresis

A

overactive bladder, fluid intake, failure to wake, psychological distress or chronic conditions

470
Q

Secondary Nocturnal Enuresis refers too

A

wetting the bed when they have previously been dry for at least 6 months

471
Q

causes of secondary nocturnal enuresis include

A 
Urinary tract infection
Constipation
Type 1 diabetes
New psychosocial problems (e.g. stress in family or school life)
Maltreatment
472
Q

nocturnal enuresis may be treated with

A

Desmopressin is an analogue of vasopressin (also known as anti-diuretic hormone).

473
Q

over active bladder may be treated with

A

Oxybutinin

474
Q

what form of polycystic kidney disease presents earlier in life

A

autosomal recessive polycystic kidney disease

475
Q

Autosomal recessive polycystic kidney disease (ARPKD) is caused by a mutation of

A

polycystic kidney and hepatic disease 1 (PKHD1) gene on chromosome 6.

476
Q

polycystic kidney and hepatic disease 1 (PKHD1) gene on chromosome 6 codes for

A

fibrocystin/polyductin protein complex (FPC)

477
Q

fibrocystin/polyductin protein complex (FPC) is responsible for

A

the creation of tubules and the maintenance of healthy epithelial tissue in the kidneys, liver and pancreas.

478
Q

underlying pathology of Autosomal recessive polycystic kidney disease (ARPKD) is

A

Cystic enlargement of the renal collecting ducts
Oligohydramnios, pulmonary hypoplasia and Potter syndrome
Congenital liver fibrosis

479
Q

ARPKD usually presents in the antenatal period with

A

oligohydramnios and polycystic kidneys seen on antenatal scans

480
Q

lack of amniotic fluid causes

A

Potter syndrome and pulmonary hypoplasia

481
Q

Potter syndrome refers too

A

underdeveloped ear cartilage, low set ears, a flat nasal bridge and abnormalities of the skeleton

482
Q

outcomes of polycystic kidney disease are

A 
Liver failure due to liver fibrosis
Portal hypertension leading to oesophageal varices
Progressive renal failure
Hypertension due to renal failure
Chronic lung disease
483
Q

survival rate to adulthood for polycystic kidney disease

A

1/3rd

484
Q

Multicystic dysplastic kidney (MCDK) is diagnosed

A

on antenatal US

485
Q

a child under the age of 5 years presenting with a mass in the abdomen. consider

A

Wilm’s tumour

486
Q

Wilm’s tumour presentation

A 
Abdominal pain
Haematuria
Lethargy
Fever
Hypertension
Weight loss
487
Q

diagnosis of wilm’s tumour is through

A

US then CT or MRI to stage and definitively biopsy with histology

488
Q

management of wilm’s tumour is with

A

nephrectomy with adjuvant chemo or RT

489
Q

posterior urethral valve is a cause of

A

hydronephrosis

490
Q

posterior urethral valve presents with

A 
Difficulty urinating
Weak urinary stream
Chronic urinary retention
Palpable bladder
Recurrent urinary tract infections
Impaired kidney function
491
Q

posterior urethral valve ix

A

antenatal scans or abdo US, MCUG, cystoscopy which can also ablate the tissue.

492
Q

Undescended testes increase risk of

A

testicular torsion, infertility and testicular cancer

493
Q

undescended testes post 6 months will require

A

Orchidopexy

494
Q

Hypospadias refers too

A

posterior urethral meatus

495
Q

hydrocele is a collection of fluid within the

A

tunica vaginalis

496
Q

tunica vaginalis can communicate with the peritoneal cavity via

A

the processus vaginalis

497
Q

hydrocele presentation is

A

soft, smooth, non-tender swelling around one of the testes.

498
Q

key feature of a hydrocele is that it

A

transilluminates with light

499
Q

differentials for a hydrocele include

A 
Hydrocele
Partially descended testes
Inguinal hernia
Testicular torsion
Haematoma
Tumours (rare)
500
Q

surfactant is produced by

A

type 2 alveolar cells

501
Q

type 2 alveolar cells mature around what weeks of gestation?

A

24 and 34 weeks

502
Q

extended hypoxia during child birth may lead too

A

hypoxic-ischaemic encephalopathy (HIE),

503
Q

hypoxic-ischaemic encephalopathy (HIE), may lead too

A

cerebral palsy

504
Q

issues with neonatal resus

A

babies have a large surface area, they loos heat rapidly and risk of meconium inhalation.

505
Q

principles of neonatal resus

A 
warm the baby
calculate APGAR score
stimulate breathing
inflation breaths  
and chest compressions
506
Q

how best to stimulate breathing in a neonate in resus

A

neutral position to keep airway open with a towel under shoulders

507
Q

cycle of breaths in neonate resus

A

Two cycles of five inflation breaths. No response the 30 seconds of ventilation breaths.

508
Q

chest compression to breath ratio

A

3:1

509
Q

APGAR score consists of

A

appearance, pulse, grimmace, activity, and respiration

510
Q

score 0 on APGAR would be

A

blue baby, absent pulse, no response to stimulation, floppy and absent respirations.

511
Q

APGAR score 2 for pulse would be

A

> 100

512
Q

max APGAR score is

A

10

513
Q

delayed cord clamping is beneficial for

A

improved haemoglobin, iron stores and blood pressure and a reduction in intraventricular haemorrhage and necrotising enterocolitis

514
Q

immediately afterbirth you should seek to

A 
Skin to skin
Clamp the umbilical cord
Dry the baby
Keep the baby warm with a hat and blankets
Vitamin K
Label the baby
Measure the weight and length
515
Q

Vit K to the baby is via a

A

IM injection

516
Q

on day 5 what neonate screening test is under taken?

A

day 5

517
Q

blood spot screening test screens for

A 
Sickle cell disease
Cystic fibrosis
Congenital hypothyroidism
Phenylketonuria
Medium-chain acyl-CoA dehydrogenase deficiency (MCADD)
Maple syrup urine disease (MSUD)
Isovaleric acidaemia (IVA)
Glutaric aciduria type 1 (GA1)
Homocystin
518
Q

results for a blood spot test take how many weeks to come back?

A

6-8 weeks

519
Q

newborn examination is undertaken how many hours after birth?

A

72 hours

520
Q

neonate sats should be measured as

A

pre ductal and post ductal

521
Q

what would be considered pathological difference in ductal sats

A

> 2%

522
Q

pre ductal sats are measured in the

A

right hand

523
Q

the right hand receives blood from the

A

right subclavian artery, a branch of the brachiocephalic artery, which branches from the aorta before the ductus arteriosus.

524
Q

post ductal sats are measured from the

A

feet

525
Q

red reflex is absent in

A

congenital cataracts and retinoblastoma.

526
Q

single palmar crease is associated with

A

down’s syndrome

527
Q

moro reflex is the

A

when rapidly tipped backwards the arms and legs will extend

528
Q

rooting reflex is the

A

tickling the cheek will cause them to turn towards the stimulus

529
Q

stepping reflex is the

A

when held upright and the feet touch a surface they will make a stepping motion

530
Q

talipes refer too

A

the ankles are in a supinated position, rolled inwards

531
Q

Port wine stains refer too

A

pink patches of skin, often on the face, caused by abnormalities affecting the capillaries.

532
Q

sturge weber syndrome refers too

A

visual impairment,port wine stains and learning difficulties, headaches, epilepsy and glaucoma.

533
Q

examples of birth injures?

A

Caput Succedaneum, Cephalohaematoma, Facial Paralysis, Erbs Palsy and Fractured Clavicle

534
Q

refers too

A

oedema collecting on the scalp outside the periosteum.

535
Q

does caput succedeneum (oedema) cross the suture lines?

A

yes

536
Q

the presence of a cephalohaematoma requires the monitoring for

A

anaemia, jaundice and resolution

537
Q

Erb’s palsy is damage to the

A

C5/C6 nerves in the brachial plexus during birth.

538
Q

Erb’s palsy presentation

A

Internally rotated shoulder
Extended elbow
Flexed wrist facing backwards (pronated)
Lack of movement in the affected arm

539
Q

common organism for neonatal sepsis is

A

Group B streptococcus (GBS)

540
Q

features of neonatal sepsis

A 
Fever
Reduced tone and activity
Poor feeding
Respiratory distress or apnoea
Vomiting
Tachycardia or bradycardia
Hypoxia
Jaundice within 24 hours
Seizures
Hypoglycaemia
541
Q

red flags for neonatal sepsis

A

Confirmed or suspected sepsis in the mother
Signs of shock
Seizures
Term baby needing mechanical ventilation
Respiratory distress starting more than 4 hours after birth
Presumed sepsis in another baby in a multiple pregnancy

542
Q

for one risk factor of neonatal sepsis you should

A

monitor the observations and clinical condition for at least 12 hours

543
Q

for two risk factors of neonatal sepsis you should

A

start antibiotics

544
Q

first line antibiotics for neonatal sepsis are

A

benzylpenicillin and gentamycin

545
Q

causes of Hypoxic-Ischaemic Encephalopathy

A

Maternal shock
Intrapartum haemorrhage
Prolapsed cord, causing compression of the cord during birth
Nuchal cord, where the cord is wrapped around the neck of the baby

546
Q

Hypoxic-Ischaemic Encephalopathy grading is called

A

sarnat staging

547
Q

Hypoxic-Ischaemic Encephalopathy mild features

A

Poor feeding, generally irritability and hyper-alert
Resolves within 24 hours
Normal prognosis

548
Q

Hypoxic-Ischaemic Encephalopathy moderate features

A

Poor feeding, lethargic, hypotonic and seizures
Can take weeks to resolve
Up to 40% develop cerebral palsy

549
Q

Hypoxic-Ischaemic Encephalopathy severe features

A

Reduced consciousness, apnoeas, flaccid and reduced or absent reflexes
Up to 50% mortality
Up to 90% develop cerebral palsy

550
Q

managements of Hypoxic-Ischaemic Encephalopathy

A

MDT, supportive and therapeutic hypothermia

551
Q

intention of therapeutic hypothermia is to

A

reduce the inflammation and neurone loss

552
Q

physiological jaundice mechanism

A

fragile RBC’s, less developed liver function

553
Q

in preamture neonates the immature liver puts the neonates at risk of

A

kernicterus.

554
Q

kernicterus is

A

brain damage due to high bilirubin levels

555
Q

prolonged jaundice is classified as how long with full term babies?

A

14 days

556
Q

prolonged jaundice is classified as how long in premature babies?

A

21 days

557
Q

causes of prolonged jaundice in the neonate

A

biliary atresia, hypothyroidism and G6PD deficiency.

558
Q

direct coombs test is for

A

haemolysis

559
Q

neonatal jaundice may be corrected through

A

phototherapy, or sometimes exchange transfusion.

560
Q

phototherapy mechanism

A

converts unconjugated bilirubin into isomers for urine and bile excretion without conjugation.

561
Q

phototherapy rebound bilirubin should be measured

A

12-18 hours after stopping

562
Q

kernicterus presents with

A

less responsive, floppy, drowsy baby with poor feeding.

563
Q

apnoeas are defined as periods of

A

breathing stops spontaneously for more than 20 seconds, or shorter periods with oxygen desaturation or bradycardia.

564
Q

apnoeas occurs due to immaturity of the

A

autonomic nervous system

565
Q

apnoeas may be a sign of

A 
Infection
Anaemia
Airway obstruction (may be positional)
CNS pathology, such as seizures or haemorrhage
Gastro-oesophageal reflux
Neonatal abstinence syndrome
566
Q

apnoeas may be managed through

A

tactile stimulation, I.V caffeine and apnoea monitors

567
Q

retinal blood vessel development starts around what weeks and is completed by

A

16 weeks and completed by 37 - 40 weeks

568
Q

retinal blood vessel development is stimulated by

A

hypoxia

569
Q

post delivery for a premature neonate after support and hypoxia returns what occurs with the retinal vessels?

A

excessive and scarred blood vessels that may regress or cause retinal detachment

570
Q

zone 1 of the retina includes

A

optic nerve and the macula

571
Q

zone 2 of the retina includes

A

ora serrata, the pigmented boarder between the retina and ciliary body

572
Q

zone 3 of the retina includes

A

ora serrata

573
Q

premature retinopathy plus disease refers too

A

tortuous vessels and hazy vitreous humour.

574
Q

screening for premature retinopathy should occurs

A

for every baby <32W or <1.5kg and for every 2 weeks and can cease once the retinal vessels enter zone 3, usually at around 36 weeks gestation.

575
Q

first line treatment for premature retinopathy

A

transpupillary laser photocoagulation

576
Q

treatment for premature retinopathy alternatives

A

cryotherapy and injections of intravitreal VEGF inhibitors, Surgery

577
Q

CXR or respiratory distress syndrome demonstrates

A

ground glass

578
Q

presentation of Necrotising Enterocolitis

A 
Intolerance to feeds
Vomiting, particularly with green bile
Generally unwell
Distended, tender abdomen
Absent bowel sounds
Blood in stools
579
Q

Necrotising Enterocolitis Ix

A

FBC, CRP, capillary blood gas for metabolic acidosis, blood culture, AXR

580
Q

AXR in necrotising enterocolitis demonstrates

A

dilated bowel, oedema, pneumatosis intestinalis (gas in bowel wall), pneumoperitoneum (free gas in peritoneal cavity), gas in portal veins.

581
Q

necrotising enterocolitis Tx

A

nil by mouth with IV fluids, total parenteral nutrition (TPN) and antibiotics. surgical emergency.

582
Q

necrotising enterocolitis carries what risk post op

A

Short bowel syndrome

583
Q

CNS Signs of neonatal abstinence

A 
Irritability
Increased tone
High pitched cry
Not settling
Tremors
Seizures
584
Q

Vasomotor and respiratory Signs of neonatal abstinence:

A

Yawning
Sweating
Unstable temperature and pyrexia
Tachypnoea (fast breathing)

585
Q

Metabolic and gastrointestinal: signs of neonatal abstinence

A

Poor feeding
Regurgitation or vomiting
Hypoglycaemia
Loose stools with a sore nappy area

586
Q

management of neonatal abstinence syndrome

A

NAS chart for at least 3 days with urine samples

587
Q

opiate withdrawal in neonates require

A

Oral morphine sulphate

588
Q

non-opiate withdrawal in neonates require

A

Oral phenobarbitone

589
Q

fetal alcohol syndrome presents as

A 
Microcephaly (small head)
Thin upper lip
Smooth flat philtrum (the groove between the nose and upper lip)
Short palpebral fissure (short horizontal distance from one side of the eye and the other)
Learning disability
Behavioural difficulties
Hearing and vision problems
Cerebral palsy
590
Q

congenital rubella syndrome presentation

A

Congenital cataracts
Congenital heart disease (PDA and pulmonary stenosis)
Learning disability
Hearing los

591
Q

Congenital Toxoplasmosis presentation

A

Intracranial calcification
Hydrocephalus
Chorioretinitis

592
Q

congenital cytomegalovirus presentation

A 
Fetal growth restriction
Microcephaly
Hearing loss
Vision loss
Learning disability
Seizures
593
Q

zika virus is spread by the

A

Aedes mosquitos

594
Q

zika congenital syndrome

A

Microcephaly
Fetal growth restriction
Other intracranial abnormalities, such as ventriculomegaly and cerebellar atrophy

595
Q

acceptable weight loss in babies are

A

breast fed babies to loose up to 10% and formula fed babies to loose up to 5% of their body weight by day 5

596
Q

if a child is on the 91st centile for height this means?

A

she is taller than 91% of children her age.

597
Q

three phases of growth are

A

First 2 years: rapid growth driven by nutritional factors
From 2 years to puberty: steady slow growth
During puberty: rapid growth spurt driven by sex hormones

598
Q

overweight is defined as a BMI above what centile?

A

85th percentile

599
Q

obese is defined as being above what centile?

A

above the 95th percentile

600
Q

mid parental height =

A

(height of mum + height of dad) / 2.

601
Q

initial investigations for faltering growth

A 
Urine dipstick, for urinary tract infection
Coeliac screen (anti-TTG or anti-EMA antibodies)
602
Q

boys predicated height formula

A

(mother height + fathers height + 14cm) / 2

603
Q

girls predicated height formula

A

(mothers height + father height – 14cm) /

604
Q

Constitutional Delay In Growth and Puberty refers too

A

short stature in childhood when compared with peers but normal height in adulthood

605
Q

Constitutional Delay In Growth and Puberty key feature

A

delayed bone age

606
Q

4 domains of child development

A

Gross motor
Fine motor
Language
Personal and social

607
Q

gross motor 4 months

A

This starts with being able to support their head and keep it in line with the body

608
Q

gross motor 6 months

A

They can keep their trunk supported on their pelvis (i.e. maintain a sitting position) by 6 months, however they often don’t have the balance to sit unsupported at this stage.

609
Q

gross motor 9 months

A

They should sit unsupported by 9 months. They can start crawling at this stage. They can also keep their trunk and pelvis supported on their legs (i.e. maintain a standing position) and bounce on their legs when supported.

610
Q

gross motor 12 months

A

They should stand and begin cruising (walking whilst holding onto furniture).

611
Q

gross motor 15 months

A

Walk unaided.

612
Q

gross motor 18 months

A

Squat and pick things up from the floor.

613
Q

gross motor 2 years

A

Run. Kick a ball.

614
Q

fine motor early milestones 6 months:

A

Palmar grasp of objects (wraps thumb and fingers around the object).

615
Q

fine motor early milestones 9 months:

A

Scissor grasp of objects (squashes it between thumb and forefinger).

616
Q

fine motor early milestones

A

Pincer grasp (with the tip of the thumb and forefinger).

617
Q

fine motor early milestones 14-18 months

A

They can clumsily use a spoon to bring food from a bowl to their mouth

618
Q

fine motor drawing skills 12 months

A

Holds crayon and scribbles randomly

619
Q

fine motor skills tower of bricks 14 months

A

Tower of 2 bricks

620
Q

fine motor skills tower of bricks 18 months

A

Tower of 4 bricks

621
Q

fine motor skills pencil grasp under 2 years

A

Palmar supinate grasp (fist grip)

622
Q

expressive language milestones month 3

A

Cooing noises

623
Q

expressive language milestones month 9

A

Babbles, sounding more like talking but not saying any recognisable words

624
Q

expressive language milestones month 12

A

Says single words in context, e.g. “Dad-da” or “Hi”

625
Q

expressive language milestones month 18

A

Has around 5 – 10 words

626
Q

expressive language milestones 2 years

A

Combines 2 words. Around 50+ words total.

627
Q

receptive language milestones month 3

A

Recognises parents and familiar voices and gets comfort from these

628
Q

receptive language milestones month 5

A

Listens to speech

629
Q

receptive language milestones month 12

A

Follows very simple instructions

630
Q

receptive language milestones 2 years

A

Understands verbs, for example “show me what you eat with”

631
Q

personal and social milestones week 6

A

smiles

632
Q

personal and social milestones month 3

A

Communicates pleasure

633
Q

personal and social milestones month 12

A

Engages with others by pointing and handing objects. Waves bye bye. Claps hands.

634
Q

personal and social milestones month 18

A

Imitates activities such as using a phone

635
Q

not sitting unsupported is a red flag at what month?

A

12

636
Q

not holding an object is a red flag at what month?

A

5

637
Q

not standing independently is a red flag at what month?

A

18

638
Q

not walking independently at how many years is a red flag?

A

2 years

639
Q

global developmental delay differentials

A 
Down’s syndrome
Fragile X syndrome
Fetal alcohol syndrome
Rett syndrome
Metabolic disorders
640
Q

gross motor delay differentials

A 
Cerebral palsy
Ataxia
Myopathy
Spina bifida
Visual impairment
641
Q

fine motor delays differentials

A 
Dyspraxia
Cerebral palsy
Muscular dystrophy
Visual impairment
Congenital ataxia
642
Q

language delays differentials

A 
Hearing impairment
Learning disability
Neglect
Autism
Cerebral palsy
643
Q

personal and social delay differentials

A

Parenting issues

Autism

644
Q

dysgraphia refers too

A

refers to a specific difficulty in writing.

645
Q

dyspraxia refers too

A

developmental co-ordination disorder

646
Q

capacity requires you to demonstrate

A

understanding, retaining info, weighing up options and communication

647
Q

klinefleter’s syndrome chromosomes

A

XXY

648
Q

turner’s syndrome chromosomes

A

XO

649
Q

legal framework for children safeguarding is

A

Children Act 1989

650
Q

both parents are carriers of an autosomal recessive condition, what is the chance of the children having the condition?

A

have a 1 in 4 (or 25%) of having the disease,

651
Q

one parent has an autosomal recessive disease, and the other parent is a carrier. what is the chance of the children being inflicted?

A

Therefore the children of these parents have a 50% chance of having the disease and a 50% chance of being a carrier.

652
Q

both parents are healthy, one sibling has a single gene disease (e.g. cystic fibrosis) disease, a second child does not have the disease, what is the likelihood of the second child being a carrier.

A

the risk of the second child being a carrier is 2 in 3. this is because we know that they do not have the phenotype of the disease.

653
Q

example of deletion disorder?

A

cri du chat, which is caused by a missing portion of chromosome 5

654
Q

example of duplication disorder

A

Charcot-Marie-Tooth, which can be caused by a duplication of the short arm of chromosome 17.

655
Q

translocation disorder example

A

“Philadelphia chromosome” translocation in acute myeloid leukaemia, which is a reciprocal translocation between chromosome 9 and chromosome 22.

656
Q

acrocentric chromosome examples

A

13, 14, 15, 21 and 22. They have one long arm and one short arm.

657
Q

Robertsonian translocation refers too

A

acrocentric chromosome loosing its short arm

658
Q

patau syndrome is a

A

trisomy 13

659
Q

patau syndrome causes

A

ysmorphic features, structural abnormalities affecting almost all areas of their body and learning disability. They have characteristic “rocker bottom feet”,

660
Q

edward syndrome is

A

trisomy 18

661
Q

edward syndrome causes

A

resulting in dysmorphic features and learning disability. They also have “rocker bottom feet”.

662
Q

in sex cells male mitochondria is located in the

A

the tail

663
Q

mitochondrial DNA inheritance comes from the

A

mother

664
Q

down syndrome features

A 
Hypotonia (reduced muscle tone)
Brachycephaly (small head with a flat back)
Short neck
Short stature
Flattened face and nose
Prominent epicanthic folds
Upward sloping palpable fissures
Single palmar crease
665
Q

complications for down syndrome

A

Learning disability
Recurrent otitis media
Deafness. Eustachian tube, Visual problems, Hypothyroidism, Cardiac defects, Atlantoaxial instability, Leukaemia, Dementia

666
Q

combined test screening for down’s biochemical results that indicate high risk

A

BHCG high, PAPPA low

667
Q

klinefelter syndrome features

A 
Taller height
Wider hips
Gynaecomastia
Weaker muscles
Small testicles
Reduced libido
Shyness
Infertility
Subtle learning difficulties
668
Q

klinefelter syndrome treatment

A

Testosterone injections
Advanced IVF techniques
Breast reduction surgery +
MDT

669
Q

features of Turner’s syndrome

A 
Short stature
Webbed neck
High arching palate
Downward sloping eyes with ptosis
Broad chest with widely spaced nipples
Cubitus valgus
Underdeveloped ovaries with reduced function
Late or incomplete puberty
Most women are infertile
670
Q

cubitus valgus refers too

A

arm is extended downwards with the palms facing forward, the angle of the forearm at the elbow is exaggerated, angled away from the body.

671
Q

management of turner’s syndrome involves

A

GH therapy, oestrogen, progesterone and fertility treatment

672
Q

noonan syndrome transmission

A

autosomal dominant

673
Q

features of noonan syndrome

A 
Short stature
Broad forehead
Downward sloping eyes with ptosis
Hypertelorism (wide space between the eyes)
Prominent nasolabial folds
Low set ears
Webbed neck
Widely spaced nipples
674
Q

noonan syndrome associated conditions

A

Congenital heart disease, Cryptorchidism, Learning disability
Bleeding disorders
Lymphoedema, increased risk of leukaemia and neuroblastoma

675
Q

marfan syndrome genetic transmission

A

autosomal dominant

676
Q

pathology of marfan

A

affecting the gene responsible for creating fibrillin

677
Q

features of marfan syndrome

A 
Tall stature
Long neck
Long limbs
Long fingers (arachnodactyly)
High arch palate
Hypermobility
Pectus carinatum or pectus excavatum
Downward sloping palpable fissures
678
Q

associated conditions with marfan

A

Lens dislocation in the eye
Joint dislocations and pain due to hypermobility
Scoliosis of the spine
Pneumothorax
Gastro-oesophageal reflux
Mitral valve prolapse (with regurgitation)
Aortic valve prolapse (with regurgitation)
Aortic aneurysms

679
Q

meet someone with tall, has hypermobility or a murmur suggestive of mitral or aortic regurgitation what do you think of?

A

marfan syndrome

680
Q

management of marfan’s involves

A

surgical correction of aortic aneurysms, avoiding stimulants and intense exercise, betablockers and ARB’s, PT, genetic counselling and yearly ECHO

681
Q

features of fragile x syndrome

A 
Intellectual disability
Long, narrow face
Large ears
Large testicles after puberty
Hypermobile joints (particularly in the hands)
Attention deficit hyperactivity disorder (ADHD)
Autism
Seizures
682
Q

prada willi syndrome arises from

A

deletion mutation of the proximal arm of chromosome 15 from the father

683
Q

features of prada willi syndrome

A

Constant insatiable hunger that leads to obesity
Poor muscle tone as an infant (hypotonia)
Mild-moderate learning disability
Hypogonadism
Fairer, soft skin that is prone to bruising
Mental health problems, particularly anxiety
Dysmorphic features
Narrow forehead
Almond shaped eyes
Strabismus
Thin upper lip
Downturned mouth

684
Q

NICE indicates for prada willi syndrome

A

Growth hormone to imrpove muscle and body composition

685
Q

angelman syndrome is caused by the loss of which gene

A

UBE3A gene

686
Q

angelman syndrome is due to a deletion on which chromosome

A

chromosome 15

687
Q

features of angelman syndrome

A 
Delayed development and learning disability
Severe delay or absence of speech development
Coordination and balance problems (ataxia)
Fascination with water
Happy demeanour
Inappropriate laughter
Hand flapping
Abnormal sleep patterns
Epilepsy
Attention-deficit hyperactivity disorder
Dysmorphic features
Microcephaly
Fair skin, light hair and blue eyes
Wide mouth with widely spaced teeth
688
Q

william syndrome is caused by deletion on what chromosome?

A

7

689
Q

features of william syndrome ?

A 
Broad forehead
Starburst eyes (a star-like pattern on the iris)
Flattened nasal bridge
Long philtrum
Wide mouth with widely spaced teeth
Small chin
Very sociable trusting personality
Mild learning disability
690
Q

associated conditions with william syndrome

A

Supravalvular aortic stenosis (narrowing just above the aortic valve)
Attention-deficit hyperactivity disorder
Hypertension
Hypercalcaemia

691
Q

features of syncope that differentiate it from a seizure

A

lightheaded, prolonged upright, sweating, blurring of vision, reduced tone, return to consciousness shortly after falling, no prolonged post-ictal period

692
Q

generalised tonic clonic seizures associated with

A

tongue biting, incontinence, groaning and irregular breathing.

693
Q

first line for generalised tonic-clonic seizure

A

sodium valproate

694
Q

second line for generalised tonic clonic seizure

A

lamotrigine or carbamazepine

695
Q

focal sezirues start in the

A

temporal lobes

696
Q

focal seizures present with

A

Hallucinations
Memory flashbacks
Déjà vu
Doing strange things on autopilot

697
Q

first line focal seizure treatment is

A

carbamazepine or lamotrigine

698
Q

second line for focal seizures are

A

sodium valproate or levetiracetam

699
Q

first line absence seizures treatment

A

sodium valproate or ethosuximide

700
Q

atonic seizures a characterised by

A

They are characterised by brief lapses in muscle tone.

701
Q

atonic seizure first line

A

sodium valproate

702
Q

myoclonic seizure refers too

A

sudden brief muscle contractions, like a sudden “jump”.

703
Q

myoclonic seizure first line is

A

sodium valproate

704
Q

infantile spasms “west syndrome” prognosis

A

1/ die by 25, 1/3rd seizure free

705
Q

infantile spasms treatment

A

Prednisolone

Vigabatrin

706
Q

EEG as an Ix for seizure occurs usually

A

after second presentation unless atypical

707
Q

MRI for epilepsy should be considered when

A

The first seizure is in children under 2 years
Focal seizures
There is no response to first line anti-epileptic medications

708
Q

additional investigations to consider for epilepsy

A

ECG, blood electrolytes, blood glucose, blood cultures, urine cultures and lumbar punctures

709
Q

sodium valproate mechanism

A

by increasing the activity of GABA,

710
Q

SE of sodium valproate

A

Teratogenic, so patients need careful advice about contraception
Liver damage and hepatitis
Hair loss
Tremor

711
Q

SE of carbamazepine

A

Agranulocytosis
Aplastic anaemia
Induces the P450 system so there are many drug interactions

712
Q

SE of lamotrigine

A

Stevens-Johnson syndrome or DRESS syndrome. These are life threatening skin rashes.
Leukopenia

713
Q

status epilepticus is defined as

A

lasting more than 5 minutes or more than 3 seizures in one hour.

714
Q

status epileptics management

A

Secure the airway
Give high-concentration oxygen
Assess cardiac and respiratory function
Check blood glucose levels
Gain intravenous access (insert a cannula)
IV lorazepam, repeated after 10 minutes if the seizure continues

715
Q

Cyanotic breath holding spells occur

A

After letting out a long cry they stop breathing, become cyanotic and lose consciousness. Within a minute they regain consciousness and start breathing.

716
Q

reflexic anoxic seizure is when

A

the child is startled. The vagus nerve sends strong signals to the heart that causes it to stop beating. The child will suddenly go pale, lose consciousness and may start to have some seizure-like muscle twitching. Within 30 seconds the heart restarts and the child becomes conscious again.

717
Q

features of a migraine that are different to a tension type headache

A

Unilateral
More severe
Throbbing in nature
Take longer to resolve

718
Q

migraines are associated with

A

Visual aura
Photophobia and phonophobia
Nausea and vomiting
Abdominal pain

719
Q

managment of migraines in children require

A 
Rest, fluids and low stimulus environment
Paracetamol
Ibuprofen
Sumatriptan
Antiemetics, such as domperidone
720
Q

migraine prophylaxis includes

A

propranolol, pizotifen, topiramate

721
Q

when an adult patient presents with a migraine ask about what?

A

recurrent central abdominal pain as a child. They may have a history of abdominal migraine that started before the headaches.

722
Q

spastic hypertonia cerebral palsy refers too

A

(increased tone) and reduced function resulting from damage to upper motor neurones

723
Q

dyskinetic cerebral palsy refers too

A

problems controlling muscle tone, with hypertonia and hypotonia, causing athetoid movements and oro-motor problems. This is the result of damage to the basal ganglia.

724
Q

ataxic cerebral palsy refers too

A

problems with coordinated movement resulting from damage to the cerebellum

725
Q

hemiplegic gait indicates

A

indicates an upper motor neurone lesion

726
Q

ataxic gait indicates

A

cerebellar lesion

727
Q

high stepping gait indicates

A

indicates foot drop or a lower motor neurone lesion

728
Q

waddling gait indicates

A

indicates pelvic muscle weakness due to myopathy

729
Q

upper motor neurone signs

A

muscle bulk preserved, hypertonia, slightly reduced poer and brisk reflexes

730
Q

lower motor neurone signs

A

reduced muscle bulk, fasiculations, hypotonia, reduced power and reduced reflexes

731
Q

people with cerebral palsy often have what signs

A

upper motor neurone signs

732
Q

paediatrician my use what drugs for those with cerebral palsy

A

muscle relaxants, anti-epileptics and glycopyrronium bromide for excessive drooling

733
Q

misalignment of the eyes is referred to as

A

strabismus

734
Q

strabismus causes

A

double vision

735
Q

concomitant squints are due to

A

differences in the control of the extra ocular muscles.

736
Q

Amblyopia refers too

A

the affected eye becomes passive and has reduced function compared to the other dominant eye

737
Q

Hirschberg’s test demonstrates

A

Deviation from the centre of a pen torch reflecting off the cornea will indicate a squint.

738
Q

Tx for squints in kids are

A

occlusive patch or atropine drops in the good eye

739
Q

CSF is created in each ventricle by

A

choroid plexuses

740
Q

CSF is absorbed by what to get into the venous sytem

A

arachnoid granulations.

741
Q

Arnold-Chiari malformation is where

A

cerebellum herniates downwards through the foramen magnum, blocking the outflow of CSF

742
Q

signs of hydrocephalus in kids are

A

increasing occipito-frontal circumference, Bulging anterior fontanelle
Poor feeding and vomiting
Poor tone
Sleepiness

743
Q

mainstay treatment for hydrocephalus is

A

Ventriculoperitoneal Shunt

744
Q

craniosynostosis is the process of

A

the skull sutures close prematurely leading to increasing intracranial pressure

745
Q

plagiocephaly means

A

flattening of one area of the baby’s head

746
Q

brachycephaly refers too

A

refers to flattening at the back of the head

747
Q

what should you look for and what should you exclude when managing Plagiocephaly

A

exclude craniosynotosis, and assess for congenital muscular torticollis

748
Q

If there is a 5 year old boy presenting with vague symptoms of muscle weakness and the description is that you notice them using their hands on their legs to help them stand up. what is the condition and underlying mechanism?

A

Duchenne’s muscular dystrophy and x linked recessive

749
Q

Gower’s sign is

A

To stand up, they get onto their hands and knees, then push their hips up and backwards like the “downward dog” yoga pose. They then shift their weight backwards and transfer their hands to their knees. Whilst keeping their legs mostly straight they walk their hands up their legs to get their upper body erect.

750
Q

duchennes muscular dystrophy is caused by a defect in the what gene?

A

dystrophin

751
Q

what has been used to slow progression of duchennes muscular dystrophy

A

oral steroids and creatine supplementals

752
Q

features of myotonic dystrophy

A

Progressive muscle weakness
Prolonged muscle contractions
Cataracts
Cardiac arrhythmias

753
Q

Emery-Dreifuss muscular dystrophy usually presents in childhood with

A

contractures, most commonly in the elbows and ankles.

754
Q

classic initial symptoms of Facioscapulohumeral Muscular Dystrophy

A

A classic initial symptom is sleeping with their eyes slightly open and weakness in pursing their lips

fourth year (12 decks)

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