Paeds 1 🫁 🫀🚽 Flashcards

Renal, Resp, Cardo, GI

1
Q

causes of UTI?

A
E.coli
enterobacter 
klebsiella 
Proteus (phosphate stones)
pseudomonas (indicates structural defect)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

symptoms of UTI in infants

A

poor feeding, vomiting, irritability, failure to thrive, diarrhoea

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

symptoms of UTI in children (4)

A

abdominal pain, fever, dysuria, increased frequency, haematuria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

lower UTI symptoms (5)

A
urgency 
frequency 
nocturia 
dysuria
suprapubic pain 
fever
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

upper UTI symptoms (3)

A

abdominal pain
nausea and vomiting
rigors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

UTI investigations (3)

A

urine collection
bloods - cultures, FBC, U&E, ESR, CRP
urine dip MC and S
USS - check for abnormalities

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

management of UTI in children?

A

General: fluid, analgesics, antiemetics
<3 months –> refer to specialist
>3 months
upper UTI - cephalosporin or co-amoxiclav 7-10 days
lower UTI - trimethoprim, nitrofurantoin, cephalosporin or co-amoxiclav for 3 days

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what are the clinical features of pyelonephritis?

A

fever
loin pain
vomiting
white cells in urine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what is enuresis?

A

involuntary discharge of urine by day or night or both in children aged 5 years or older in the absence of congenital or acquired defects of the nervous system or urinary tract.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what is primary nocturnal enuresis?

A

the child had never achieved continence

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what is nocturnal secondary enuresis?

A

the child has been dry for at least 6 months before

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what is the management of nocturnal enuresis?

A

look for possible underlying causes/triggers (constipation, DM, UTI)
advise of fluid intake, diet and toilet behaviour
rewards systems

first line treatment for children under the age of 7 is an enuresis alarm

first line treatment for children over the age of 7 - desmopressin.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what can cause daytime enuresis? (4)

A
lack of attention to the bladder sensation 
detrusor instability
bladder neck weakness 
UTI, constipation
neuropathic bladder 

secondary - may be due to emotional upset, UTI, polyuria from DM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what are the classifications of acute kidney injury?

A

pre-renal - most common in children
renal - there is salt water retention
post renal - urinary obstruction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

management of acute kidney injury? (3)

A

metabolic acidosis –> sodium bicarbonate
hyerkalaemia –> calcium gluconate if ECG changes, salbutamol, calcium change resin, glucose and insulin, dietary restriction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

how would you treat pre-renal AKI?

A

suggested by hypovolaemia - needs to be corrected with fluid replacement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

how would you treat renal AKI?

A

if fluid overload - restrict fluids, emergency management of metabolic abnormalities

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

how would your treat post-renal AKI?

A

relief of obstruction with nephrostomy or catheterisation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

what is acute kidney injury?

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

what are pre-renal causes of AKI

A
  • most common cause in children - hypovolaemia (burns, sepsis, gastroenteritis, haemorrhage, nephrotic syndrome), circulatory failure
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

what are renal causes of AKI

A

vascular (haemolytic uraemia syndrome (HUS), vasculitis, embolus, renal vein thrombosis)
Tubular (acute tubular necrosis, ischaemic, toil, obstructive)
glomerular - glomerulonephritis
interstitial - interstitial nephritis, pyelonephritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

what are post renal causes of AKI?

A

obstruction

congenital or acquired

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

what is the presentation of chronic kidney disease? (5)

A
anorexia 
lethary 
polydipsia 
polyuria 
failure to thrive 
body deformities 
hypertension 
acute on chronic renal failure 
unexplained normochromic normocytic anaemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

management of chronic kidney disease (5)

A

Reduce CV risk - Control BP, statin
Potassium control - Dietary restriction
Prevention osteodystrophy - Calcium carbonate, vit D
Treat anaemia - Subcut EPO
Treat acidosis - Sodium bicarbonate
Prophylaxis - Influenza and pneumococcal vaccinations

Calorie supplements
NG feeding
Fluid correction
Hormonal abnormalities

Dialysis and transplant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

what causes chronic kidney disease? (4)

A
hypertension 
diabetes
glomerular sclerosis 
lupus 
RA
HIV
NSAIDS 
toxins - tobacco
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

what can uraemia cause? (3)

A
nausea 
loss of appetite
encephalopathy (tremor)
pericarditis 
increase bleeding 
uremic frost - crystals in skin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

what happens to potassium levels in CKD?

A

less potassium is excreted which leads to build up in blood

hyperkalemia - can cause arrhythmias

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

what happens to calcium levels in CKD?

A

low - hypocalcemia

kidneys activate vitamin D which aids the absorption of calcium

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What happens when calcium levels are low due to kidney dysfunction?

A

parathyroid hormone is released which causes the bones to lose calcium
this leads to bone being brittle which is called renal osteodystrophy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

how is CKD diagnosed (3)

A

changes in GFR
less than 60ml/kg/1.73m2
Proteinuria (ACR >3mg/ml)
kidney biopsy to look for changes such as glomerular sclerosis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

what are the three manifestations of nephrotic syndrome?

A

proteinuria (>3+ on dipstick)
hypoalbuminaemia
oedema

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

what is the most common cause of nephrotic syndrome in children ?

A

minimal change disease

can also be caused by focal glomerulosclerosis and post stept nephritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

what happens in nephrotic syndrome?

A

there is damage and inflammation to the podocytes which normally help prevent protein loss from the glomerulus. As a result of this, increased protein is lost through the nephron tubules.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

How is nephrotic syndrome treated?

A

90% of proteinuria resolves with corticosteorid therapy (prednisolone for 4 weeks then a smaller dose every other day for 4 weeks)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

what are the complications of nephrotic syndrome? (3)

A

hypovolaemia
thrombosis
infection
hypercholesteroleamia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

how does minimal change disease present?

A

nearly always presents as nephrotic syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

What are the causes of minimal change disease? (3)

A

the majority are idiopathic
drugs - NSAIDS, rifampicin
Hodgkin’s lypmhoma, thymoma
infectious mononucleosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

what are the features of nephrotic syndrome?

A

Oedema
Proteinuria
Hypoalbuminaemia
Hyperlipidemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

how is minimal change disease managed?

A

80% of cases are steroid responsive

cyclophoshphamide in the next step for steroid-resistant cases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

what is congenital nephrotic syndrome?

A

when nephrotic syndrome presents within the first 3 months of life.
it is rare
commonest kind is recessively inherited
associated with high mortality usually due to complications of hypoalbuminaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

what are the causes of acute nephritis? (3)

A

post infectious (including streptococcus)
Vasculitis (HSP, SLE, wegener granulomatosis)
IgA nephropathy and mesangiocapillary glomerulonephritis
anti-glomerular basement membrane disease (good pasture syndrome)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

what are the clinical features of nephritic syndrome?

A

decreased urine output and volume overload
hypertension which may cause seizures
oedema, characteristically around the eyes
haematuria and proteinuria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

how is nephritic syndrome managed?

A

water and electrolyte balance and the use of diuretics when necessary

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

what are the features of henoch-schonlein purpura? (4)

A
characteristic palpable purpuric rash on buttocks and extensor surfaces of arms and legs 
arthralgia 
periarticular oedema 
abdominal pain 
glomerulonephritis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

what are the common factors for HSP to occur? (3)

A
between 3 and 10 years 
more common in boys
peaks during winter months
often preceded by a response infection 
cause is unknown
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

what is Alport syndrome

A

it is a familial nephritis
usually an X-linked recessive disorder that progresses to end-stage renal failure by early adult life in males and is associated with sensorineural deafness and ocular defects.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

what is hypospadias?

A

congenital abnormality of the penis which occurs in approx 3/1000 male infants
an abnormal opening below the urethra
can also happen in girls but way way more common in boys

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

what is hypospadias characterised by? (3)

A

a ventral urethral meatus
a hooded prepuce
chord (ventral curvature of the penis) in more severe form
the urethral meatus may open proximally in the more severe variants

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

How is hypospadias treated?

A

corrective surgery is performed before 2 years of age

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

what are the investigations for nephrotic syndrome (5)

A
LFT
lipids 
infection 
calcium (low)
renal injury 
urine 
bloods 
CXR
renal USS
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

what are the investigations for HSP?

A

bloods (raised IgA, raised ESR, ASO titre (check for PSG)

urine dip

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

what is the management for HSP?

A

Supportive care- rest, rehydration, analgesia for MSK pain

prednisolone if severe

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

what is the presentation of post streptococcal glomerulonephritis?

A

URTI or pharyngitis

then 1-3 weeks later
haematuria
oliguria
proteinuria

odema
hypertension
signs of CV overload

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

what are the investigations for post streptococcal glomerulonephritis? (5)

A
bloods 
- FBC - anaemia 
- U&amp;Es - increased urea and creatinine, increased potassium, acidosis
- antistreptolysin O increased 
anti DNAase B titre 
low C3
urinalysis - haematuria, proteinuria
renal biopsy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

what is the management of post streptococcal glomerulonephritis? (3)

A

penicillin - 10 days
treat hypertension and fluid overload
correct metabolic imbalances

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

what is vesicoureteric reflux?

A

there is abnormal back flow of urine from the bladder to the ureter and kidneys

there is abnormal vesicoureteric junction where the ureters enter the bladder - the ureters are displaced laterally, entering the bladder in a more perpendicular fashion than at an angle therefore shortened ureter, and the junction cannot function adequately

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

what is the grading of vesicoureteric reflux?

A

I - Reflux into the ureter only, no dilatation
II -Reflux into the renal pelvis on micturition, no dilatation
III-Mild/moderate dilatation of the ureter, renal pelvis and calyces
IV - Dilation of the renal pelvis and calyces with moderate ureteral tortuosity
V - Gross dilatation of the ureter, pelvis and calyces with ureteral tortuosity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

what are the investigations (2) and management (3) for vesicoureteric reflux?

A

micturating cystourethrogram for diagnosis (MCUG)

Management:
Avoid constipation
Avoid an excessively full bladder
Prophylactic Abx
Surgical input from paediatric urology

DMSA (dimercapto succinic acid) scan should also be performed to check for scarring (4-6 months after illness)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

what are the triad of features of haemolytic uraemia syndrome (HUS)?

A

acute renal failure
microangiopathic haemolytic syndrome
thrombocytopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

what is typical HUS

A

secondary HUS

classically caused by shiga toxin producing E.coli (STEC)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

what is atypical HUS?

A

primary HUS due to complement dysregulation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

What investigations would you perform for HUS? (3)

A

FBC (anaemia, thrombocytopenia, fragmented blood film)
U&E (acute kidney injury)
stool culture (Shiga toxin-producing Escherichia coli)
LDH (non specific sign for haemolytic anaemia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

What is the management of HUS?

A

supportive treatment - fluids, blood transfusion and dialysis if required.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

what are the most common viral causes of respiratory infections? (4)

A
respiratory syncytial virus (RSV) 
rhinoviruses 
parainfluenza 
influenza 
metapneumovirus 
adenovirus
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

what are the most common bacterial causes of respiratory infections? (3)

A
streptococcus pneumoniae 
streptococci 
haemophilus influenzae 
bordetella pertussis
mycoplasma pneumoniae
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

what increases the risk of respiratory infection? (3)

A
parental smoking 
overcrowded housing, damp housing 
poor nutrition 
underlying lung disease 
male gender 
haemodynamically significant congenital heart disease 
immunodeficiency
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

whar are the classifications of respiratory infections?

A

upper respiratory tract infection (common cold, sore throat (pharyngitis including tonsilitis), acute otitis media, sinusitis)
Laryngeal tracheal infection (croup, bacterial tracheitis, acute epiglottitis)
bronchiolitis
pneumonia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

what are the symptoms of a common cold? (5)

A
acute onset 
rhinitis
sore throat 
sneezing 
post-nasal drainage/drip 
cough 
fever 
non-specfic red pharynx 
nasal mucosal oedema
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

what are the common causes of the common cold (coryza)?

A

rhinoviruses, coronaviruses, RSV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

what is pharyngitis?

A

pharynx and soft-palate are inflamed and local lymph nodes are enlarged and tender

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

what are the different types of sore throat?

A

pharyngitis
tonsilitis
laryngitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

what is tonsilitis?

A

intense inflammation of the tonsils often with a purulent exudate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

what are common causes of tonsilitis? What is the most common cause?

A

Usually viral - most commonly caused by rhinovirus, coronavirus and adenovirus
group A beta-haemolytic streptococci, and EBV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

what are the symptoms and signs of tonsilitis?

A

pain on swallowing
fever >38
tonsillar exudate
cervical lymphadenopathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

how do you treat bacterial tonsilitis?

A

1st line - Phenoxymethylpenicillin

2nd line - clarithromycin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

what is the criteria used for antibiotic prescription in tonsilitis?

A

the centor criteria

  • presence of tonsilar exudate
  • tender anterior cervical lymphadenopathy or lymphadenitis
  • history of fever
  • absence of cough

3 must be present

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

what test can be used to detect group A streptococcus pharyngitis?

A

rapid antigen detection test

should be used routinely in patients with sore throat to allow immediate point-of-care assessment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

What is bronchiolitis? What is the most common case?

A

it is a condition characterised by acute bronchiolar inflammation and a build up of mucus in the small airways of the lungs.
usually caused by RSV virus - 75-80% of cases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

what age group does bronchiolitis usually affect, when is the common time for it to occur?

A

it is almost exclusively an infantile disease - commonly affecting children under 2
most common cause of serious RTU in children under one
maternal IgG provides protection to neonates

higher incidence in winter months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

what can cause bronchiloitis other than RSV?

A

rhinovirus
influenza virus
may be secondary to a bacterial infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

what are the symptoms of bronchiolitis? (4)

A

coryzal symptoms, including virus precede:
Dry cough
increasing breathlessness
wheezing, fine inspiratory crackles
feeding difficulties
tachypnoea
increased work of breathing may be present - retractions, grunting, nasal flaring

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

what are the features of bronchiolitis that NICE recommend immediate referral for? (3)

A
apnoea 
child looks seriously unwell to health care professional 
severe resp distress
central cyanosis 
persistant oxygen sats less than 92%
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
83
Q

what are the features of bronchiolitis that NICE recommend consider referral for?

A

a resp rate over 60BPM
difficulty breast feeding or inadequate oral fluid intake
clinical dehydration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
84
Q

how would you diagnose bronchiolitis?

A

largely clinical
nasopharyngeal aspirate or throat swab - RSV rapid testing and viral cultures
blood and urine cultures if child is pyrexic
FBC
ABG if severely unwell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
85
Q

what is the treatment for bronchiolitis?

A

supportive care, supplemental oxygen and mechanical ventiliation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
86
Q

what is the prophylaxis for bronchiolitis and who is it given to?

A

monthly IM injection of palivizumab

given to preterm babies born before 29 weeks, or babies born with chronic lung disease of prematurity before 32 weeks
also can be given to those who are severely immunocompromised

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
87
Q

what is croup?

A

Viral URTI

croup is also known as laryngotracheobronchitis - common respiratory disease of childhood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
88
Q

what are the causes of croup?

A

usually parainfluenza

other causes include RSV adenovirus, rhinovirus, enterovirus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
89
Q

what age group does croup commonly affect?

what time of year is it common?

A

commonly children who are between 6 months and 3 years

Autum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
90
Q

what are the features of croup?

A

stridor (high pitched, wheezing sound caused by disrupted airflow)
barking seal like cough, which is typically worse at night
fever
coryzal symptoms
if severe there may be signs of resp distress

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
91
Q

what are the red flag signs of rep failure? (3)

A

cyanosis
lethargic/decreased level of consciousness
labored breathing
tachycardia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
92
Q

what are the differentials of croup? (3)

A
epiglottitis 
inhaled foreign body 
acute anaphylaxis 
bacterial tracheitis 
diptheria
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
93
Q

what is pseudomembranous croup? What bacteria is it caused by?

A

aka bacterial tracheitis
rare but very dangerous
similar to viral croup but child has high fever, appears toxic and has rapidly progressive aiway obstruction with copious amounts of thick airway secretions
it is caused by staphylococcus aureus
treatment is with IV Abx, intubation and ventilation if needed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
94
Q

what is mild, moderate and severe croup defined as?

A

mild: Occasional barking cough
No audible stridor at rest
No or mild suprasternal and/or intercostal recession
The child is happy and is prepared to eat, drink, and play

moderate: Frequent barking cough
Easily audible stridor at rest
Suprasternal and sternal wall retraction at rest
No or little distress or agitation
The child can be placated and is interested in its surroundings

severe: Frequent barking cough
Prominent inspiratory (and occasionally, expiratory) stridor at rest
Marked sternal wall retractions
Significant distress and agitation, or lethargy or restlessness (a sign of hypoxaemia)
Tachycardia occurs with more severe obstructive symptoms and hypoxaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
95
Q

when should you consider admission for croup? (3)

A

moderate or severe croup
< 6 months of age
known upper airway abnormalities (e.g. Laryngomalacia, Down’s syndrome)
uncertainty about diagnosis (important differentials include acute epiglottitis, bacterial tracheitis, peritonsillar abscess and foreign body inhalation)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
96
Q

how should you diagnose and manage croup?

A

clinical diagnosis

mild croup - single dose of oral dexamethasone
moderate - single dose of oral dexamethasone plus nebulised adrenaline
severe - single dose oral dexamethasone plus nebulized adrenaline plus oxygen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
97
Q

what are causes of stridor in children? (4)

A

croup
acute epiglotittis
inhaled foreign body
laryngomalacia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
98
Q

what is asthma?

A

a chronic respiratory condition characterised by reversible and paroxysmal constriction of the airways
secondary to type 1 hypersensitivity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
99
Q

what are the risk factors for the development of asthma? (5)

A
personal or family history of atopy 
antenatal factors: maternal smoking, viral infection during pregnancy
low birth weight 
not being breastfed 
maternal smoking around the child 
exposure to high concentrations of allergens (e.g. house dust mite)
air pollution 
premature birth
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
100
Q

what other atopic conditions are associated with asthma?

A

IgE-mediated atopic conditions such as
eczema
alleric rhinitis (hay fever)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
101
Q

what are the precipitating factors for asthma? (4)

A

cold air
atmospheric pollution
NSAIDs and beta-blockers
exposure to allergens
Exercise

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
102
Q

what are the clinical features of asthma? (5)

A

symptoms worse at night and early morning (diurnal variation)
symptoms that have a non-viral trigger
cough
dyspnoea
wheeze, chest tightness- episodic triggers
increased work of breathing

there may be expiratory wheeze on auscultation
reduced peak expiratory flow rate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
103
Q

what investigations would you perform for asthma? (5)

A

spirometry - FEV1 and FVC (FEV1 will be reduced, FVC normal )
peak expiratory flow
response to bronchodilator on spirometry
CXR

also consider 
skin prick testing 
fractional expired nitic oxide - elevated 
sputum culture 
exercise testing
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
104
Q

what are the differentials for asthma? (3)

A
bronchiolitis 
episodic viral wheeze 
inhaled foreign body 
recurrent aspiration 
cardiac failure 
CF
primary ciliary dyskinesia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
105
Q

how is asthma managed in children?

A

SABA when required

add a regular preventer - very low dose ICS or LTRA if less than 5.

Initial add on therapy - very low dose ICS plus inhaled LABA or LTRA

next consider increasing ICS dose or add LTRA or LABA
if no response to LABA consider stopping

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
106
Q

what are the two clinical patterns of preschool wheeze?

A

viral episodic wheeze - wheezing only in response to viral infection and no interval symptoms. Usually resolves by 5 years. Triggered by viruses that can cause the common cold.

Multiple trigger wheeze - wheeze in response to viral infection but also to another trigger such as exposure to aeroallergens and exercise. A significant proportion go on to have asthma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
107
Q

what would finger clubbing suggest?

A

CF or bronchiectasis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
108
Q

how do you manage viral episodic wheeze?

A

treatment is symptomatic only
first-line - SABA
second line - LTRA - montelukast

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
109
Q

how do you manage multiple trigger wheeze?

A

trial of either ICS or LTRA - typically for 4-8 weeks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
110
Q

when should you consider hospital admission for children with asthma?

A

if after a high dose of bronchodilator therapy they:

  • have not responded adequately clinically
  • if they are becoming exhausted
  • have decreased oxygen sats less than 92%
  • if CXR is indicated
  • if there are unusual features or signs of severe infection
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
111
Q

what are the classifications of a mild, moderate, severe and life threatening acute asthma attack?

A

MILD: : SaO2 >92% in air, vocalizing without difficulty, mild chest wall recession and moderate tachypnoea

MODERATE: SpO2 >92%, PEF>50% best or predicted, no clinical features of severe asthma

SEVERE: SaO2 <92% PEFR 33-50%, cannot complete sentences in one breath or too breathless to feed or talk. Heart rate >125 (if over 5 years) or >140 (2-5 years) RR > 30 (over 5 years) or >40 (2-5 years)

LIFE THREATENING: SaO2 <92% ,PEFR <33%, silent chest, poor resp effort, cyanosis, hypotension, exhaustion, confusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
112
Q

how do you manage a mild to moderate acute asthma attack?

A
  • give beta-2 agonist via a spacer - one puff every 30-60 seconds up to a max of 10 puffs
    if symptoms not controlled repeat and refer to hospital
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
113
Q

how should severe asthma attacks be managed?

A

if low oxygen sats give high flow oxygen
SABA via nebuliser
ipratropium bromide can be added
if no response to SABA
steroid therapy should be given for 3 days

2nd line IV salbutamol - essential to monitor for salbutamol toxicity
magnesium sulphate can be considered

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
114
Q

what are the common causes of pneumonia in different age groups?

A

Newborn - organisms from the mother’s genital tract particularly group B streptococcus, E.coli, klebsiella, staph aureus

infants and young children - RSV most common, streptococcus pneumoniae, chlamydia trachomatis

Children over 5 - mycoplasma pneumoniae, streptococcus pneumoniae and chlamydia pneumoniae ar the main causes

at all ages mycobacterium tuberculosis should be considered

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
115
Q

what are the clinical features of pneumonia in children? (3) and what would examination show? (5)

A

fever and difficulty breathing = the most common presenting symptoms
usually preceded by an upper RTI
cough
lethary
poor feeding
unwell child
localised chest, abdo or neck pain is a feature or pleural irritation and suggests bacterial infection

examination will show signs of resp distress, desaturation and cyanosis
tachypnoea, nasal flaring and chest indrawing
end inspiratory coarse crackles over the affected area
classical signs of consolidation - dull percussion, decreased breath sounds and bronchial breathing
oxygen sats may be decreased

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
116
Q

what are the investigations you would perform for pneumonia?

A

CXR - may confirm diagnosis but cannot differentiate between viral and bacterial
Nasopharageal aspirate - viral immunoflourenence identify viral causes
blood tests - FBC and acute phase reactants
blood cultures

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
117
Q

how should you treat pneumonia in children?

A

first line treatment is amoxicillin for all children with pneumonia

for children under 5 alternatives include co-amoxiclav for tyical pneumonia (steptococcus oneumoniae) or clarithromycin for atypial pneumonia (mycoplasma pneumoniae and chlamydia trachomatis)

if over 5 years - consider macrolide (clarithromycin) if mycoplasma or chlamydia is suspected, if staoh aureus suspected the consider macrolide or flucloxacillin with amoxicillin

severe pneumonia: co-amoxiclav , cefotaxime or cefuroxime IV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
118
Q

when would you consider admission for pneumonia? (4)

A

most cases can be managed at home, admission is indicated if:

  • oxygen sats less than 92%
  • severe tachypnoea
  • difficulty breathing
  • grunting
  • apnoea
  • not feeding
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
119
Q

what is cystic fibrosis?

A

an autosomal recesive disorder which leads to a defect in the CF transmembrane receptor protein which causes defective ion transport in exocrine glands.
causes thickening of respiratory mucus - the lungs therfore prone to inadequate mucociliary clearance, chronic bacterial infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
120
Q

what is the screening for CF?

A

all newborn babies are screened for CF

blood spot analysis on the Gurthrie card

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
121
Q

what are the presentations of CF at different age groups (6)

A

infancy - meconium ileus, prolonged neonatal jaundice

Childhood - recurrent lower respiratory chest infection, bronchiectasis, poor appetite, rectal prolapse, nasal polyps, sinusitis

Adolescence: bronchiectasis, DM, cirrhosis and portal hypertension, distal intestinal obstruction, pneumothorax, haemoptyysis, male infetiltiy

short stature
delayed puberty
female subfertility
weight loss or poor weight gain

90% of children with CF have pancreatic exocrine insuficiency (lipase, amylase and proteases) resulting in maldigestion and malabsorption

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
122
Q

how do you diagnose CF? (4)

A

sweat test - abnormally high sweat chloride
immunoreactive trypsinogen test (new born screening)
CXR
glucose tolerance test
LFT
faecal elastase to assess pancreatic function
genetic analysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
123
Q

what could cause a false positive sweat test for CF ? (3)

A
malnutrition 
adrenal insufficiency 
glycogen storage disease 
nephrogenic diabetes insupidus 
hypothyroidism 
G6PD
ectodermal dysplasia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
124
Q

How do you manage CF? (6)

A

MDT approach
annual review in specialist center

For ongoing respiratory disease:
twice daily physiotherapy to clear airway secretions and postural drainage, inhaled bronchodilator (salbutamol), inhaled mucolytic (dornase alpha), if they have chronic pseudomonas infection give inhaled tobramycin

GI disease:
monitoring and optimising nutrion
pacreatic insuficiency - pancreatic enzyme replacement (pancreatin), H2 antagonist or PPI (ranitidine or omeprazole), fat soluble vitamin supplementation (A, D, K and E)
liver disease - ursodeoxycholic acid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
125
Q

What is epiglottitis? What causes it? What age group is it most common in?

A

A cellulitis of the supraglottis
it is a life threatening emergency due to high risk of respiratory obstruction caused by haemophilus influenzae type B (HiB) - Hib immunisation has led to a 99% reduction in the incidence
most common in the age group 1-6 years but can affect all age groups

** important to distinguish between epiglottitis and croup

126
Q

what are the symptoms of epiglottitis? (5)

A
the onset is very acute/rapid 
high fever and generally unwell - toxic looking child 
stridor 
drooling of saliva
increasing resp difficulty over hours
sore throat 
tripod position
127
Q

what investigations would you perform for suspected epiglottitis? (3)

A

lateral neck radiograph (thumb print sign)
FBC
blood cultures
Direct visualisation by senior (diagnosis)

128
Q

how do you treat epiglottitis? (3)

A

secure airway and supplemental O2
IV Abx (cefotaxime or ceftriaxone or ampicillin or clindamycin)
dexamethasone can be added to reduce inflammation
inhaled adrenaline - if upper airways are compromised
intubation

once stable - oral Abx - amoxicillin or cefaclor

129
Q

what are the differences in presentation between croup and epiglottitis? (5)

A

Epiglottitis is rapid onset (hours), croups is days
croups has coryza prior to onset
croup has barking cough, epiglottitis usually has no cough
croup they can drink/feed
epiglottitis will have drooling saliva
epiglottitis will have a high fever
croup has rasping stridor epiglottitis is a soft stridor
in epiglottitis their voice will be weak or silent

130
Q

what can cause stridor in children? (5)

A

Croup – harsh loud stridor (mostly viral, 6 months to 6 years of age, harsh loud stridor, coryza, mild fever and hoarse voice)
Epiglottitis – (caused by H. influenzae type b, rare since Hub immunisation
Bacterial tracheitis – harsh loud stridor, higher fever, toxic
Inhaled foreign body – chocking on peanut, sudden onset of cough or respiratory distress
Laryngomalacia – recurrent or continuous since birth.

131
Q

what is surfactant deficient lung disease?

A

AKA respiratory distress sydrome
caused by inadequate production of surfactant type 2 from the pneumocytes in the lungs
low surfactant leads to alveoli collapsing on expiration and this increases the energy needed for breathing

132
Q

what are the risk factors for surfactant deficient lung disease? (4)

A
Premature
maternal DM at term
multiple pregnancy 
fam history of RDS
csection 
hypothermia 
male>female
133
Q

what are the causes of secondary surfactant deficiency? (3)

A
  • Intrapartum asphyxia
  • RTI: GB-BHS pneumonia
  • Meconium aspiration pneumonia
  • Pulmonary haemorrhage
  • Pulmonary hypoplasia
  • Congenital diaphragmatic hernia
134
Q

what is the presentation of surfactant deficient lung disease? (5)

A
Early signs 
•	Tachypnoea >60
•	laboured breathing 
•	grunting 
•	recession: subcostal and intercostal 
•	Nasal flaring 
•	Cyanosis 
•	Diminished breathing sounds 
Late signs 
•	Fatigue 
•	Apnoea 
•	Hypoxia
135
Q

how do you manage surfactant deficient lung disease?

A
  1. surfactant replacement therapy: endotracheal tube
  2. antibiotics
  3. O2 : SaO2 85-93%
    • Mild: via a hood
    • Moderate: CPAP
    • Severe : endotracheal tube
  4. Nutrition
    • If the infant is stable: IV nutrition: amino acids and lipids
    • If resp status is stable: small volume of gastic feed via a tube
136
Q

what causes whooping cough?

A

bordetella pertussis

137
Q

what are the symptoms of whooping cough? What are the three phases of whooping cough?

A

One week of coryza (Catarrhal phase), then the child develops a characteristic paroxysmal or spasmodic cough followed by a characteristic inspiratory whoop (paroxysmal phase).
Often worse at night
Often vomiting can occur
In infants the whoop may be absent, but apnoea may occur at this age
Epistaxis and subconjunctival haemorrhage may occur vigorous coughing
Paroxysmal phase may last 3-6 weeks and symptoms gradually decrease but may persist for many months (convalescent phase)

138
Q

how is whooping cough diagnosed?

A

nasal swab

PCR and serology

139
Q

how is whooping cough managed? (3)

A

under 6 month - admit

  • notifiable disease
  • oral macrolide - clarithromycin
  • household contacts - prophylaxis
  • school exclusion for 48 hours following commencing antibiotics
140
Q

what are the complications of whooping cough? (3)

A

subconjunctival haemorrhage
pneumonia
bronchiectasis
seizures

141
Q

how should inhaled drugs be administered in children under 3?

A

face masks

142
Q

what are the risks associated with long term use of inhaled steroids? (3)

A

adrenal suppression
growth suppression
osteoporosis
high dose steroids may cause adrenal crisis

143
Q

what are some upper RTI? (3)

A
rhinitis 
otitis media 
pharyngitis 
tonsilitis 
larygitis
Epiglottitis
144
Q

what are some lower RTI? (3)

A
bronchitis 
croup 
tracheitis 
bronchiolitis
pneumonia
145
Q

what can pneumococcus cause/ what is it course of infection?

A

consolidation of the nasopharynx

  • -> upper airways mucosa infection - otitis media, sinusitis
  • ->lower airway mucosal infection - bacterial bronchitis, pneumonia
  • -> occult septicaemia, pneumonia with septicaemia, meningitis
146
Q

what is pneumonia?

A

resp disease characterised by inflammation of the lung parenchyma (excluding the bronchi) with congestion caused by viruses, bacteria or irritants

147
Q

what is the bronchiectasis vicious circle hypothesis?

A

infection –> inflammation –> impaired muco-ciliary clearance –> infection

148
Q

who would be in the MDT team for CF? (5)

A
paediatric pulmonologist
physio
dietician 
nurse liason 
primary care team 
teacher 
psychologist
149
Q

what are some causes of wheeze in children? (5)

A

pneumonia, pulmonary oedema, bronchogenic cyst
enlarged left atrium compressing left mainstem bronchus
chest deformity
asthma
brochiolitis
bronchitis
CF
polyps
airway obstruction - foreign body, mucus, pus, blood

150
Q

what can cause a congenital left to right shunt?

how would they present?

A

ventricular septal defect
patent ductus arteriosus
atrial septal defect

they present as breathless

151
Q

what can cause right to lefts shunts?

how would they present?

A

tetralogy of Fallot
transposition of the great arteries

they would present as blue

152
Q

what are the circulatory changes at birth?

A

first breath = a fall in resistance to pulmonary blood flow

  • -> blood flow through the lungs increases six fold
  • ->this results in a rise in left atrial pressure.
  • -> mean while the blood returning to right atrium falls as the placenta is excluded from the circulation
  • -> the changes in the pressure difference causes the foramen ovale to close
  • the DA will normally close within the first few hours or days of life
153
Q

what are the symptoms (3) and signs (3) of heart failure?

A

symptoms - breathlessness (particularly on feeding or exertion), sweating, poor feeding, recurrent chest infection

signs: poor weight gain or faltering growth, tachypnoea, tachycardia, heart murmur, gallop rhythm, enlarged heart, hepatomegaly, cold peripheries.

154
Q

what usually causes heart failure in the first week of life?

A

left heart obstruction e.g. coarctation of the aorta

*if obstruction is very severe then arterial perfusion may be predominantly by right to left flow of the blood via the ductus arteriosus (duct dependant circulation) - closure of the duct = severe acidosis, collapse and death unless duct patency is restored

155
Q

after the first week of life what is the most common cause of progressive heart failure?

A

left to right shunt

this eventually causes pulmonary oedema and breathlessness due to decreased pulmonary vascular resistance

156
Q

what happens if a left to right shunt is left untreated?

A

children will develop Eisenmenger syndrome which is irreversibly raised pulmonary vascular resistance resulting from chronically raised pulmonary arterial pressure and flow. this lead the shunt reversing to a right to left shunt and results in a blue child. If this develops the only surgical option is a heart-lung transplant if available.

157
Q

what might cyanosis in the new-born infant in the new born be due to? (3)

A
  • cardiac disorders - cyanotic congenital heart disease
  • resp disorders - surfactant deficiency, meconium aspiration, pulmonary hypoplasia
  • persistent pulmonary hypertension of the new born - failure of the pulmonary vascular resistance to fall after birth
  • infection - septicaemia from group B streptococcus and other organisms
  • metabolic disease - metabolic acidosis and shock
158
Q

what are the different types of ASD?

A

secundum ASD

partial/primum ASD

159
Q

what are the symptoms of ASD? (2)

A

often asymptomatic
may have recurrent chest infections
from the fourth decade they may develop arrhythmias

160
Q

what are the signs of ASD? (2)

A

an ejection systolic murmur best heard at the upper left sternal edge - due to increased flow across the pulmonary valve because of the left-to-right shunt
a fixed and widely split second heart sound

161
Q

what investigations would you perform for ASD and what would they show? (3)

A

CXR - cardiomegaly, enlarged pulmonary arteries and increased pulmonary vascular markings
ECG - right bundle branch block
Echo

162
Q

how do you manage ASD? (2)

A

Management options include:

Active monitoring (small defects may close on their own)
Percutaneous transvenous catheter closure (via the femoral vein)
Open-heart surgery

163
Q

what is a small VSD?

A

smaller than the aortic valve in diameter - up to 3mm

164
Q

how would a small VSD present ?
what would you hear when auscultating a small VSD?
what investigations would you perform and how would you manage one?

A

often asymptomatic
a loud pan-systolic murmur at lower left sternal edge (loud murmur = smaller defect)
quiet pulmonary second sound
CXR and ECG will be normal
echo will demonstrate precise anatomy of defect

management - often will close spontaneously

165
Q

what is a large VSD?
how would a large VSD present ?
what would you hear when auscultating a large VSD?
what investigations would you perform and how would you manage one?

A
  • bigger than the size of the aortic valve
  • symptoms of heart failure with breathlessness and FTT after 1 week old, recurrent chest infections, tachypnoea, tachycardia and enlarged liver from heart failure, active precordium.
  • there may be a soft or no murmur = large defect
  • there will be a loud pulmonary second sound from raised pulmonary pressure
  • CXR - cardiomegaly, enlarged pulmonary arteries, increased pulmonary vascular markings, pulmonary oedema
  • ECG - biventricular hypertrophy by 2 months of age
  • ECHO - demonstrated anatomy of defect
  • Management: diuretic for heart failure (furosemide) often combined with captopril.
    Surgery should be performed at age 3-6 month
166
Q

what is patent ductus arteriosus?

A

a form of congenital heart defect
generally classed as ‘acyanotic’. However, uncorrected can eventually result in late cyanosis in the lower extremities, termed differential cynaosis.
connection between the pulmonary trunk and descending aorta
usually the ductus arteriosus closes within the first 24 hrs of life due to increased pulmonary flow which enhances prostaglandins clearance
more common in premature babies, born at high altitude or maternal rubella infection in the first trimester

167
Q

what are the signs of patent ductus arteriosus? (4)

A
left subclavicular thrill
continuous 'machinery' murmur
large volume, bounding, collapsing pulse
wide pulse pressure
heaving apex beat
168
Q

how do you manage a PDA?

A

1st line - IV indomethacin (inhibits prostaglandin synthesis ) - closes the duct in the majority of the cases

*if it is associated with another congenital heart defect amenable to surgery then prostaglandin E1 is usefull to keep the duct open until after surgical repair

use a percutaneous catheter device closure
if baby too small or large duct then use surgical ligation

169
Q

what test can be used in cyanosed infants to test for heart disease?

A

```
the hypoxia (nitrogen washout) test
place them in 100% oxygen for 10 mins and then if right radial artery PaO2 from blood gas remains low then it Is cyanotic congenital heart disease
~~~
It works on the assumption that if there is right to left shunting in cyanotic heart disease, no amount of oxygenation in the pulmonary circulation will alter the desaturating effect of the shunt. However, if there is a pulmonary defect causing cyanosis this may be corrected by increasing the inspired oxygen.

170
Q

what are the 4 cardinal anatomical features of tetralogy of Fallot?

A
  • ventricular septal defect (VSD)
  • right ventricular hypertrophy
  • pulmonary stenosis (right ventricular outflow tract obstruction)
  • overriding aorta
171
Q

what are the signs and symptoms of TOF? (3)

A

cyanosis - hypercyanotic episodes ‘tet’ spells
a loud harsh ejection systolic murmur
in older children there may be clubbing
may have tachypnoea

172
Q

what investigations would you perform for TOF and what would they show? (3)

A

CXR - boot shaped heart
ECG - will show right ventricular hypertrophy when older
ECHO - will show the cardinal features.

173
Q

how do you manage TOF?

A

complete surgical repair to close VSD and relieve RV outflow obstruction

174
Q

what is transposition of the great arteries?

A

the aorta is connected to the right ventricle and the pulmonary arteries are connected to the left ventricle - this means there is two parallel circulation
*incompatible with life unless there is a mixing of blood - often there is naturally occurring VSD, ASD, PDA which can achieve mixing short term

leads to right to left shunt

175
Q

what are the symptoms of transposition of the great arteries?

A

neonatal cyanosis
usually appears on day 2 when ductal closure leads to reduced mixing of desaturated and saturated blood
often a loud, single heart sound

176
Q

how do you manage transposition of the great arteries? (3)

A

maintain ductus arteriosus with prostaglandin infusion
a balloon atrial septostomy
all patients will require surgery, arterial switch procedure in the neonatal period

177
Q

what is an AVSD?
how does it present
how is it managed?
What condition is it common in?

A

atrioventricular septal defect
common in down-syndrome
may be complete or partial
it will present on antenatal USS screening
cyanosis at birth or heart failure at 2-3 weeks of life
manage heart failure and fix surgically at 3-6 months

178
Q

what is aortic stenosis?

A

Aortic stenosis is the most common valvular heart disease and the most common indication for valve replacement surgery. It refers to narrowing of the aortic valve, restricting blood flow from the left ventricle to the aorta.

179
Q

what are the symptoms (3) and signs (3) of aortic stenosis?

A

Symptoms: chest pain, syncope/presyncope, dyspnoea

Signs: slow rising pulse, narrow pulse pressure, ejection systolic murmur that radiates to the carotids, thrill

180
Q

How do you manage aortic stenosis and pulmonary stenosis ?

A

balloon dilatation

181
Q

what are the clinical features of pulmonary stenosis? (3)

what would investigations show?

A

mainly asymptomatic
small number of neonates have critical pulmonary stenosis and have duct- dependant circulation

ejection systolic murmur best heard at the upper left sternal edge
ejection click
there may be a ventricular heave when stenosis is severe

CXR - normal or post stenotic dilation of the pulmonary artey
ECG - evidence of right ventricular hypertrophy

182
Q

what is coarctation of the aorta?

A

Coarctation of the aorta is a congenital heart defect in which your baby’s aorta (the largest artery in their body) is pinched in or narrowed in one spot. This restricts normal blood flow to their lower body

183
Q

what are the clinical features of coarctation of the aorta? (3)

A
often asymptomatic 
sometimes when the DA closes the aorta also constricts, causing severe obstruction of the LV outflow
systemic hypertension 
ejection systolic murmur 
radiofemoral delay
184
Q

what would you see on CXR and ECG in coarctation of the aorta? (3)

A

CXR - rib notching due to the development of large collateral intercostal arteries running under the ribs posteriorly to bypass the obstruction, 3 sign - visible notch in the descending aorta at the site of the coarctation

ECG- LV hypertrophy

185
Q

how do you manage coarctation of the aorta?

A

insertion of a stent

sometimes surgical repair is required

186
Q

what is interruption of the aortic arch?

A

Interrupted aortic arch is a very rare heart defect in which the aorta is not completely developed. There is a gap between the ascending and descending thoracic aorta. In a sense it is the complete form of a coarctation of the aorta.

usually VSD is present
presentation is with shock in the neonatal period - circulatory collapse with absent femoral pulses and absent left brachial pulse

187
Q

how do you manage an interruption of the aortic arch?

A

maintain ABC
prostaglandin infusion
complete correction with closure of VSD and repair of the aortic arch is usually performed within the first few days of life

188
Q

what is hypoplastic left heart syndrome?

A

underdevelopment of the left side of the heart
small or atretic mitral valve
left ventricle is diminutive and there is usually aortic valve atresia
small ascending aorta
circulatory collapse - all peripheral pulses absent
sometimes detected antenatally
no flow through the left side of the heart, so ductal constriction leads to profound acidosis and rapid CV collapse

189
Q

what is a sinus arrhythmia?

A

it is normal in children and it detectable as a cyclical change in heart rate with respiration (acceleration during inspiration)

190
Q

what is the most common childhood arrhythmia?

A

supraventricular tachycardia

191
Q

what are the clinical features of supraventricular tachycardia? (2)

A

rapid HR between 250-300BPM
can cause poor CO and pulmonary oedema
it will typically present as heart failure

192
Q

what will an ECG show in supraventricular tachycardia?

A

narrow complex tachycardia (QRS less than 100ms)

193
Q

management of supraventricular tachycardia (3)

A
circulatory and respiratory support 
vagal stimulation manoeuvres e.g. carotid sinus massage 
IV adenosine 
electrical cardioversion 
flecainide
194
Q

What is congenital complete heart block? What will all children with symptoms require?

A

rare condition
usually related to the presence of anti-RO or anit-LA antibodies in maternal serum - this mothers will either manifest or latent connective tissue disorders
the antiboides appear to prevent the normal development of the conduction system in the developing heart with atrophy and fibrosis of the AV node
all children with symptoms will require the insertion of an endocardial pacemaker

195
Q

How does long QT syndrome present in children? What is the inheritance of long QT syndrome?

A

associated with sudden loss of consciousness during exercise, stress or emotion usually in late childhood
may be mistakenly diagnosed as epilepsy
inheritance is AD

196
Q

what is rheumatic fever?

A

there is an abnormal immune response to a streptococcal infection
usually develops 2-4 weeks following the infection
it is usually a pharyngeal infection - then polyarthritis, mild fever and malaise develop

197
Q

what is the evidence for a recent streptococcal infection?

A

raised ASO titre or other streptococcal antibodies
positive throat swabs
positive rapid group A strep antigen test

198
Q

what are the major (4) and minor criteria (4) for rheumatic fever?

A

Two major or one major and two minor

MAJOR: erythema marginatum - uncommon early manifestation rash on trunk and limbs, Sydenham’s chorea - a late feature - involuntary movements and emotional liability, polyarthritis, pancarditis (endocarditis, myocarditis, pericarditis), subcutaneous nodules

MINOR: fever, raised ESR or CRP, arthralgia, prolonged PR interval

199
Q

what are the symptoms of chronic rheumatic fever?

A

the most common long-term damage is mitral stenosis from scarring and fibrosis.

200
Q

How should you manage rheumatic fever? How is recurrence prevented? (3)

A

Outline of management

antibiotics: oral penicillin V
anti-inflammatories: NSAIDs are first-line
treatment of any complications that develop e.g. heart failure

recurrence should be prevented with monthly injections of benzathine penicillin

201
Q

what are risk factors for infective endocarditis? (3)

A

congenital heart disease
rheumatic fever valve disease
prosthetic valves
IVDU

202
Q

when should you suspect infective endocarditis?

A

it should be suspected in any child or adult with fever, malaise, raised ESR, unexplained anaemia or haematuria

203
Q

what are the most common causes of infective bacterial endocarditis?

A
streptococcus viridans - linked to poor dental hygiene 
staphylococcus aureus (common in IVDU)
204
Q

what are the clinical signs of infective endocarditis? (7)

A
205
Q

how should you diagnose infective bacterial endocarditis ?

A

blood cultures should be taken before starting Abx
detailed cross-section echo may confirm diagnosis but will not exclude diagnosis
acute phase reactants are raised

206
Q

how should you treat bacterial endocarditis?

A

high dose IV Abx -Amoxicillin plus gentamicin or vancomycin for a min of 4-6 weeks

207
Q

what are some causes of heart failure in children? (4)

A
large left to right shunt 
left sided obstructive lesion 
cardiomyopathy 
myocarditis
endocarditis
Kawasaki disease 
tachyarrhythmias
208
Q

what are the clinical features of heart failure in children? (5)

A
sweating
breathless, tachypnoea, coughing, lung creps 
poor feeding, poor weight gain and FTT
hepatomegaly cardiomegaly
tachycardia, gallop heart rhythm
209
Q

what are some causes of acyanotic heart disease? (5)

A
VSD
ASD
PDA
pulmonary valve disease 
coarctation of the aorta 
aortic stenosis 
hypoplastic left heart syndrome 
hypertrophic obstructive cardiomyopathy 
dextrocardia
210
Q

what are some causes of cyanotic heart disease? (2)

A

tetralogy of fallot
transposition of the great arteries
tricuspid atresia

211
Q

what are the principle causes of pericardial inflammation? (3)

A
  • Infections (coxsackie B , EBV, strept, TB, toxoplasmosis
  • connective tissue (RA, rheumatic fever, SLE, sarcoidosis
  • metabolic - hyperuricaemia, hypothyroidism
  • malignancy
  • radiotherapy
212
Q

what would an ECG show when there is pericarditis?

A
213
Q

what are the clinical features of pericarditis? (2)

A

typically sharp pain which is exacerbated and exaggerated by lying down and relieved by sitting or leaning forward. Pain is often referred to left shoulder

214
Q

how is pericarditis managed?

A

analgesia
anti-inflammatory drugs (colchicine, aspirin, ibuprofen)
pericardiocentesis for pericardial effusion

215
Q

what might myocarditis be due to? (3)

A

infections
Kawasaki
drugs - Adriamycin
connective tissue disease: SLE, RA, rheumatic fever, sarcoidosis

216
Q

what are the classifications of cardiomyopathy?

A

hypertrophic
dilated
restrictive

217
Q

what are the causes of dilated cardiomyopathy?

A

inherited
secondary to metabolic disease
direct result from a viral infection

218
Q

what is GORD?

A

gastro-oesophageal reflux disease - when there is the inappropriate effortless passage of gastric contents into the oesophagus

219
Q

what are the causes of reflux in infancy? (3)

A

it is associated with slow gastric emptying, liquid diet, horizontal position, and low resting lower oesophageal sphincter pressure.

lower oesophageal sphincter dysfunction (hiatus hernia) may cause reflux
increased gastric pressure 
external gastric pressure
gastric hypersecretion of acid 
food allergy 
cerebral palsy
220
Q

what age group is GORD common in?

A

common in the first 5 years of life but usually all symptoms clear by 12 months.

221
Q

how does GORD present? (3)

A

typically develops before 8 weeks
vomiting/regurgitation
milky vomits after feeds
may occur after being laid flat
excessive crying, especially while feeding

222
Q

what are the complications of GORD? (3)

A
oesophageal stricture 
barrett's oesophagus (squamous to columnar epithelium)
faltering growth 
anaemia 
lower resp disease
223
Q

how is GORD managed in children?

A

usually diagnosed clinically
nurse infants on a head up slope of 30 degrees
thicken milk feeds in infants, small frequent meals, avoid food before sleep, avoid fatty foods, cirtus juices, caffeine and fizzy drinks
add drugs if severe - ranitidine or omeprazole

224
Q

what causes pyloric stenosis in children?

A

idiopathic hypertrophic pyloric stenosis

hypertrophy of the pylorus muscle causing outlet obstruction

225
Q

when does pyloric stenosis usually present?

A

usually in the third or fourth week of life

** it is more common in boys

226
Q

what is the presentation of pyloric stenosis in infants?

A

projectile vomiting, non-bilious, may contain altered blood (coffee ground) or fresh blood from oesophagitis. Vomiting will occur within an hour of feeding and the baby is immediately hungry. If they present early (2nd/3rd week) then vomiting may not be projectile.
weight loss
constipation
dehydration, malnutrition and jaundice are late sings
palpable mass in the upper abdomen

227
Q

what tests would you perform to diagnose pyloric stenosis? What would biochemistry show?

A

test feed - peristalsis seen during feed, the pyloric tumour is usually easiest felt early in the feed or after the baby has vomited
US - if tumour cannot be felt, USS will confirm or exclude the diagnosis
biochemistry - hypochloraemic, hypokalaemic metabolic alkalosis

228
Q

how is pyloric stenosis managed in children?

A

rehydrate and correct the alkalosis before surgery
IV fluids
withold feeds - the stomach should be emptied with an NGT
Ramstedt’s pyloromyotomy

229
Q

what is Crohn’s disease?

A

it is a type of inflammatory bowel disease that may affect any part of the GI tract but terminal ilium and proximal colon are commonest sites of involvement
bowel involvement is non-continuous (skip lesions)

230
Q

how does crohns disease present? (5)

A
abdominal pain 
diarrhoea +/- blood/mucus 
weight loss
lethary 
fever
oral lesions 
perianal skin tag
uveitis 
erythema nodosum
231
Q

what investigations would you perform for crohns disease? (5)

A

FBC - anaemia may be present, leukocytosis is associated with acute or chronic inflammation, thrombocytosis is a useful marker for active inflammation

iron studies, B12, and folate

there may be hypoalbuminaemia, hypocholesterolaemia, and hypocalcaemia

raised ESR and CRP

stool sample - to rule out and infectious cause (faecal calprotectin- inflam)

endoscopy

CT or MRI - helps to localise disease

232
Q

How do you induce and maintain remission in crohns disease?

A

Glucocorticoids (prednisolone) are generally used to induce remission. Budesonide is an alternative.
5-ASA (mesalazine) are used second line but are not as effective.
Azathioprine and mercaptopurine may be used as add on therapy.
Methotrexate is an alternative azatioprine.
infliximab useful in refractory disease or fistulating disease.

Azathioprine or mercaptopurine are used first line to maintain remission.

surgical management: local surgical resection for severe localised disease e.g. strictures, fistula, may be indicated

233
Q

what is ulcerative colitis?

A

a form of inflammatory bowel disease, inflammation always starts at the rectum and never spreads beyond the ileocaecal valve and is continuous.

234
Q

What is the presentation of UC? (4)

A
bloody diarrhoea 
urgency 
tenesmus 
abdominal pain, particularly in the lower left quadrant 
weight loss
growth failure 

in children it is usually pancolitis where as in adults it is usually confined to the distal colon

235
Q

how is UC managed?

A

amino salicylates are used to induce remession (mesalazine)
oral corticosteroid can also be used
more agressive disease may need immunomodulator therapy (azathioprine) to maintain remission

if severe - manage in hospital

*methotrexate is not recommended in UC

236
Q

how is UC diagnosed? (5)

A
colonoscopy 
biopsies 
stool studies 
FBC
ESR
CRP
barium enema - will show loss of haustrations, superficial ulceration and in long-standing disease - the colon is narrow and short
237
Q

what are the viral causes of gastroenteritis? (3)

A

rotavirus (most common)
caliciviruses (norovirus and sapovirus)
adenovirus
astrovirus

238
Q

what is the presentation of gastroenteritis?

A
vomiting 
non-bloody diarrhoea 
cramping abdominal pain 
low grade fever 
dehydration
239
Q

what are the risk factors for dehydration in gastroenteritis?

A
  • infants under the age of 6 months or those born with a low birth weight
  • if they have passed more than 6 diarrhoeal stools in the previous 24 hours
  • if they have vomited three times of more in the past 24 hours
  • if they have been unable to tolerate/not been offered extra fluids
  • if they have malnutrition
240
Q

what are the different classifications of dehydration?

A

mild: (less than 5% loss of body weight): skin turgor may be dcreased, dry mucous membranes, low urine output, HR increased, BP normal, perfusion normal, pale, may be irritable
moderate: (5-10% loss of body weight): skin turgor decreased, very dry mucous membranes, oliguric, HR increased, BP may be normal, prolonged cap refil, grey skin colour, lethargic
severe: (10-15% loss of body weight): poor skin turgor with tenting, parched mucous membranes, anuric, increased HR, BP decreased, prolonged cap refil, mottled skin, comatose

241
Q

what are the red flag signs of hypovolaemic shock? (3)

A

appears unwell or deteriorating, altered responsiveness, tachycardia, tachypnoea, skin turgor reduced

242
Q

what is isonatraemic and hyponatraemic dehydration?

A

Isonatraemic - losses of sodium and water are proportional and the plasma sodium levels remains within a normal range
hyponatraemic - if the child with diarrohea drinks large quantities of water, it leads to a fall in plasma sodium - this leads to a shift of water from extra cellular to intracellular compartments which increases cellular volume - may result in convulsions

243
Q

what is hypernatraemic dehydration?

A

water losses exceeds the relative sodium loss and the plasma sodium concentration increases
the extracellular fluid becomes hypertonic with respect to intracellular fluid and this leads to a shift of water into the extracellular space from the intracellular compartments
this form of dehydration is hard to see as there is not signs of extracellular fluid delpetion.
This type of dehydration occurs when there is high insensible water loss (when water is lost through the skin - high fever or hot dry enviroment) or from perfuse, low sodium diarrhoea.
water is drawn out of the brain and cerebral shrinkage within rigid skull may lead to jittery movement, increased muscle tone with hyperreflexia, altered consciousness, seizures and multiple small cerebral haemorrhages

244
Q

how do you treat viral gastroenteritis?

A

fluids - oral rehydration solution

mild dehydration - less than 5% loss of body weight give 50mls/kg plus maintenance fluids

moderate (less than 10%) - 100ml/kg plus maintenece

if shock add fluid bolus

245
Q

what are some common causes of bacterial gastroenteritis? (4) What age group is it most common in?

A

it is most common in children under 2

salmonella 
campylobacter jejuni
shigella 
E. coli
clostridium difficile 
Bacillus cereus 

sources of infection include contaminated water, poor food hygiene, faecal-oral route

246
Q

what is the presentation of bacterial gastroenteritis? (5)

A
watery diarrhoea
vomiting
cramping abdo pain 
fever 
dehydration 
electrolyte disturbance 
malaise 
blood and mucus in diarrhoea 
abdo pain may mimic appendicitis or IBD
tenesmus
247
Q

what are the complications of bacterial gastroenteritis? (3)

A
bacteraemia 
secondary infections - pneumonia, osteomyelitis, meningitis 
Reiter's syndrome 
GBS
haemolytic-uraemic syndrome 
reactive arthropathy 
haemorrhagic colitis
248
Q

what investigations would you perform for bacterial gastroenteritis?

A

stool +/- culture
stool clostridium difficile toxin
sigmoidoscopy

249
Q

what are the classical symptoms of appendicitis? (3)

A

anorexia
abdominal pain - initially central but localises to the RIF
low-grade fever

nausea
diminished bowel signs

*younger children or children with a retrocaecal/pelvic appendix may present atypically

it is uncommon in children under the age of 4 - however if these children do get it, it often presents when perforated

250
Q

what investigations would you order for appendicitis? (3)

A

FBC - mild neutrophil predominant leukocytosis (increase in WCC)
pelvic or abdominal CT
USS
urinalysis - to rule out other causes

251
Q

how do you treat appendicitis?

A

appendectomy plus supportive care

IV antibiotics - cefoxitin

252
Q

What is a congenital diaphragmatic hernia? What can this result in?

A

it is characterised by the herniation of abdominal viscera into the chest cavity due to incomplete formation of the diaphragm - this can result in pulmonary hypoplasia and hypertension which can cause respiratory distress shortly after birth

253
Q

what is the most common type of congenital diaphragmatic hernia?

A

left-sided posterolateral Bochdalek hernia (85%)

- there is left sided herniation of abdominal contents through the posterolateral foramen of the diaphragm

254
Q

on physical examination what signs would you find suggesting a congenital diaphragmatic hernia? (2)

A

the apex beat and the heart sounds will be displaced to the right side of the chest wall, with poor air entry to the left side

255
Q

how are congenital diaphragmatic hernias diagnosed?

A

usually diagnosed on antenatal USS, but the prognosis for the foetus is poor
if the diagnosis is not made antenataly and the baby present at birth, clinical finding may include resp distress, scaphoid abdomen and apparent dextrocardia.
diagnosis can be confirmed with x-ray

256
Q

how is a congenital diaphragmatic hernia managed? What is the mortality rate and what is the primary reason for this?

A

initial management: sedation, paralysis, endotracheal intubation and mechanical ventilation with 100% oxygen.
NGT placement
avoid bag mask valve ventilation
after stabilisation the hernia is repaired surgically - however often the more pressing problem is lung hypoplasia - where the pressing of the herniated viscera has prevented the lungs developing - lung hypoplasia =high mortality

*only around 50% of newborn survive

257
Q

what is a hiatus hernia and what are the two types of hiatus hernia?

A

herniation of the stomach into the chest wall through the oesophageal hiatus in the diaphragm
sliding
rolling

258
Q

how is a hiatus hernia diagnosed?

A

radiologically by barium swallow test/barium meal

259
Q

what are the features of an inguinal hernia?

A

a reducible swelling in the groin, often extending to the scrotum
more common in boys and preterm babies
most commonly on the right side due to later descent of the right testis
15% are bilateral
symptoms are rare

260
Q

what is the treatment of an inguinal hernia?

A

reduce following opioid analgesia and sustained compression. Surgery is delayed for 24-48 hours to allow resolution of oedema.
If reduction is impossible, emergency surgery is required because of the risk of strangulation of bowel and damage to the testis

261
Q

what is the definition of constipation?

A

infrequent passage of stool associated with pain and difficulty or delay in defaecation

262
Q

what are the nice diagnosis criteria for constipation in children under the age of 1 and children older than 1?

A

children less than 1:

  • fewer than 3 complete stools per week (type 3 or 4 on bristol stool chart - rabbit dropping or hard large stools) - this does not apply for children who are exclusively breastfed as these infants may not pass stools for several days
  • distress on passing stools, bleeding associated with stool straining

children older than 1:

  • fewer than 3 complete stools per week, overflowing soiling, rabbit dropping, large infrequent hard stools that can block the toilet
  • poor appetite that improves with the passage of a large stool, waxing and wining of abdominal pain with passage of stool, straining, anal pain
263
Q

what are some causes of constipation? (6)

A
often idiopathic 
other causes:
low fibre diet
lack of mobility and exercise
poor colonic motility 
medications - opiates and anticholinergics 
anal fissure
Hirschsprung's disease 
hypothyroidism
hypercalcaemia 
sexual abuse
UTI
264
Q

what is the presentations of constipation? (5)

A
difficult or painful defecation 
long interval between passing stools 
faecal incontinence
overflow faecal incontinence 
palpable faecal mass in abdomen 
otherwise healthy child 
abdominal distension 
abdominal pain
265
Q

what are the reg flag symptoms with constipation? (3)

A
  • failure to pass meconium within 24 hours of life - indicates hirschsprung disease
  • failure to thrive/grow - indicates hypothyroidism or coeliac disease
  • gross abdominal distension -> indicated hirschprung disease or other gastrointestinal dysmotility
  • abnormal lower limb neurology deformity -> lumbosacral pathology
  • sacral dimple over the natal cleft, over the spider naevus, hairy patch, central pit or discoloured skin -> spina bifida
  • abdnormality of appearance or postion of anus –> abnormal anatomy
  • bruising -> sexual abuse
  • fistulae, abscesses or fissures -> perianal crohn disease
266
Q

how do you treat constipation? (3)

A

dietary modification (increase fluids and fibre)
start with a osmotic laxative (lactulose/ movicol)
add a stimulant laxative (senna)

if they are over one year of age a stool softener can be added - macrogol laxitive - polyethylene glycol lus electrolytes
* if they are under 4 years old had have faecal impactation a stimulant laxative should not be used

occasionally retention is so severe that evacuation is only possible using enemas or by manual evacuate under an anaesthetic

267
Q

what is Hirschsprung disease?

A

the absence of ganglionic cells from the myenteric plexus (Auerbach’s plexus) and submucosal plexus of part of the large bowel results in a narrow, contracted segment.
The abnormal bowel extends from the rectum for a variable distance proximally, ending in a normally innervated dilated colon.
in most cases it is confined to the rectum or sigmoid - short segment disease
however sometimes it involves the entire colon - long segment disease

268
Q

how do hirschsprung’s disease present?

A

usually withing the first few days of life with intestinal obstruciton - failure to pass meconium in the first 24 hours
later abdominal distension and later bile stained vomiting develop

  • sometimes children with short segment disease will represent later on in childhood with chronic constipation.
269
Q

how is hirschsprung’s disease diagnosed?

A

abdominal x-ray will show intestinal obstrucion
anorectal manometry or barium studies may be useful in giving the surgeon an idea of the length of the aganglionic segment but are unreliable for diagnostic purposes
rectal biopsy will show no ganglionic cells in the submucosa

270
Q

who is hirschsprung’s disease common in?

A

male and down syndrome

271
Q

How is hirschsprung’s disease managed?

A

Initially, bowel irrigation/rectal washouts
management is surgically, initially colostomy followed by anastomosing normally innervated bowel to the anus (pull-through procedure)

272
Q

what is the most important complication of hirschsprung’s disease? What toxin is it associated with and what is the mortality rate?

A

hirschsprung’s enterocolitis - a dramatic gastroenteric illness characterised my abdominal distension, bloody watery diarrhoea, circulatory collapse and septicaemia. usually associated with clostridium difficile toxin in the stools - mortality is 10%

273
Q

what is coeliac disease?

A

autoimmune disease triggered by dietary gluten peptides

is an enteropathy due to lifelong intolerance to gluten

274
Q

what part of gluten causes coeliac disease?

A

the gliadin fraction of gluten provokes a damaging response in the proximal small intestine mucosa

275
Q

what are the risk factors for coeliac disease? (3)

A
a positive family history 
type 1 diabetes 
down syndrome 
IgA deficiency 
autoimmune thyroid disease
276
Q

how does coeliac disease present?

A

the classical initial features include:

  • pallor
  • diarrhoea
  • pale, bulky floating stools
  • anorexia
  • failure to thrive
  • irritibilty

later there is
apathy, gross motor developmental delay, ascites, peripheral oedema, anaemia, delayed puberty, arthralgia, hypotonia, muscle waisting, specific nutrtional disorders

277
Q

how is coeliac disease diagnosed?

A

often is it diagnosed on screening of children at increased risk
diagnosis suggested by positive serology, conformation depends upon the demonstration of mucosal changes - jejunal biopsy showing subtotal villous atrophy, increased intraepithelial lymphocytes, crypt hypertrophy
-anti-endomysial IgA antibodies
- tissue transglutaminase IgA antibody
- anti-gliadin antibodies

278
Q

how do you manage coeliac disease?

A

gluten free diet

calcium and vitamin D deficiency

279
Q

how do you manage a coeliac crisis?

A

rehydration
correction of electrolyte abnormalities
corticosteroids

280
Q

what is failure to thrive?

A

suboptimal weight gain in infants and toddlers
growth falls away from standardized wight or height centile
weight is the most sensitive indicator in infants and young children, height is the best indicator in older children

*under stress - head circumfrance growth is more preserved than linear growth

281
Q

what are the classifications of failure to thrive?

A

mild - falls across two centile lines

severe - falls across three centile lines

282
Q

sometimes after birth the infants birth may drop, what does this mean?

A

birth weight reflects intrauterine enviroment - it is a poor guid to the childs correct genetic potential and weight may naturally fall until the correct level is attained.

283
Q

what are some causes of failure to thrive?

A

usually classified as organic or non-organic

organic include: impaired suck/swallow - oro-motor dysfunction, neurological disorder e.g. cerebral palsy, chronic ilness, malabsorption, inadequate retention - vomiting or severe GORD, failure to utilise nutrients , increased requirements - thyrotoxicosis

non-organic - broad spectrum of psychosocial and enviromental deprivation - neglect, socioeconomic deprivation, mothers poor mental health

284
Q

What is marasmus?

A

severe macronutrient malnutrition

285
Q

what is Kwashiorkor?

A

severe protein malnutrition where there is generalised oedma as well as wasting

286
Q

what measurements do WHO reccomend for nutritional status?

A

weight for age (a measurement of wasting and an index of acute malnutrition)
mid upper arm circumference
height for age - a measurment of stunting and an index of chronic malnutrition

287
Q

What are some signs of kwashiorkor? (5)

A

bilateral pitting oedema
a flacky-paint skin rash with hyperkeratosis (thickened skin) and desquamation
distended abdomen and enlarged liver
angular somatitis
hair which is sparse and depigmented
diarrhoea, hypothermia, bradycardia and hypotension
low plasma albumin, potassium, glucose and magnesium

288
Q

how would you treat kwashiorkor?

A

uncomplicated:
community based therapy with ready to use therapeutic food and also a broad spectrum antibiotic - amoxicillin

complicated:
empirical antibiotic therapy (gentamicin and ampicillin)
vitiman A supplementation
shock - oxygen and fluids
dehydration - rehydration solution for malnutrition (ReSoMal)
hypoglycaemia - supplemental glucose or sucrose
correct electrolyte imbalance
hypothermia - gentle waming - especially at night

289
Q

what is intussusception?

A

common cause of intestinal obstruction in young children and is defined as the prolapse of one part of the intestine into the lumen of the adjoining distal part.

290
Q

what is the most common region for intussusception to occur and at what age does it commonly occur?

A

the ileocaecal region

most commonly occuring in infants aged between 3 and 12 months

291
Q

What is the presentation of intussusception? (4)

A

paroxysmal severe colicky pain and pallor, during episodes the child becomes pale
they initially recover between painful periods but subsequently become increasingly lethargic
Inconsolable crying
During paroxysm infant will characteristically draw knees up
vomiting
red current jelly stool (late sign)
Sausage-shaped mass in RUQ

292
Q

what investigations would you perform for intussusception?

A

x ray
USS - will show target sign/doughnut sign
diagnostic enema

293
Q

how do you manage intussusception?

A

fluid resuscitation
unless there are signs of peritonitis, contrast enema reduction should be tried first
antibiotics should be given - clindamycin and gentamicin

if fails or contraindicated surgical reduction will be required

294
Q

What is Meckel’s diverticulum?

A

Meckel’s diverticulum is the most common congenital anomaly of the gastrointestinal tract. It results from incomplete obliteration of the vitelline duct leading to the formation of a true diverticulum of the small intestine contains ectopic ileal, gastric or pancreatic mucosa

occurs in 2% of the population
is 2 feet from the ileocaecal valve
is 2 inches long

295
Q

how does Meckel’s diverticulum present? (3)

A

usually asymptomatic
abdominal pain which may mimic appendicitis
rectal bleeding
intestinal obstruction

296
Q

what investigations would you consider for Meckel’s diverticulum? Which is most useful for diagnosis?

A

FBC
technetium scan - most useful to diagnose meckel’s diverticulum
USS
abdominal Xray

297
Q

how do you manage meckel’s diverticulum?

A

only treat if symptomatic

excision of the diverticulum

298
Q

what is toddlers diarrhoea?

A

chronic non-specific diarrhoea that occurs from 6 months to five years
stools vary in consistency and often contain undigested food
affected children are often well and thriving and there are no precipitating dietary factors

299
Q

how do you treat toddlers diarrhoea?

A

reassurance, dietary (increase fat intake, normalise fibre intake, reduce milk, fruit juice and sugary drink intake), loperamide (Imodium) may sometimes be needed.

300
Q

What is infant colic?

A

a common set of benign symptoms seen in young children
typically occurs in infants less than three months
characterised by excessive cry and pulling up of the legs which is often worse in the evening
often accompanied by excessive flatus
occurs in upto 40% of infants and usually the cause is unknown
if it is persistant then it may be due to cow’s milk protein allergy or gasto-oesophageal reflux

301
Q

what is biliary atresia?

A

is a progressive idiopathic, necroinflammatory process that may involve a segment or the entire biliary tree.
there is destruction or absence of the extrahepatic biliary tree and intrahepatic biliary ducts

302
Q

what are the features of biliary atresia? (3)

A

neonatal jaundice
babies have a normal birth weight but fail to thrive as disease progresses
pale stools and dark urine
bruising - due to vitamin k deficiency related to chronic cholestasis
hepatomegaly - not until disease has progressed, splenomegaly will often develop secondary to portal hypertension
there is obstruciton of the bile ducts which causes bile to go to the liver and cause increase of conjugated bilirubin in the blood

303
Q

how is biliary atresia diagnosed? (3)

A

serum total and conjugated bilirubin (high)
prothrombin time, INR
FBC - in advanced disease - low platelets and low WBC
LFTs
abdominal USS - will demonstrate contracted or absent gallbladder
liver biopsy will show extrahepatic biliary obstruction - although features may overlap with neonatal hepatitis

304
Q

How is biliary atresia treated?

A

kasai procedure AKA hepatoportoenterostomy - although even when successful can progress to cirrhosis and portal hypertension

305
Q

What is wilson’s disease? What gene is mutated and what chromosome is this located on?

what is it caused by?

A

an autosomal recessive disorder
caused by a defect in the ATP7B gene located on chromosome 14
reduced synthesis of ceruloplasmin (the copper binding protein) and defective excretion of copper in the bile, which leads to an accumulation of copper in the liver, brain, kidney and cornea.

306
Q

how does wilson’s disease present? (3)

A
  • Kayser Fleischer rings
  • blue nails
  • Hepatitis/liver cirrhosis
  • Neuro and psych (basal ganglia degeneration)
307
Q

how do you diagnose wilsons disease?

A

low serum ceruloplasmin
LFTs
24 hour urine copper
liver biopsy - elevated liver copper
Genetic testing

308
Q

how do you treat wilson’s disease?

A

with penicillamine or trientine - both promote urinary copper excretion
zinc - to reduce copper absorption

can be fatal from hepatic complications if left untreated

309
Q

What are choleductal cysts?

A

cystic dilations of the extrahepatic biliary system

310
Q

how do choleductal cysts present? (4)

A

25% present in infancy with cholestasis, in the older age group they present with abdominal pain, a palpable mass and jaundice or cholangitis

311
Q

how do you diagnose and treat choleductal cyst?

A

diagnosis with USS or radionuclide scanning

treatment is with surgical excision of the cyst