Paeds Flashcards
1
Q
Developmental milestones - 6 weeks
A
Valentine’s day
Newborn bb check @ GP
GM: good head control
FM: tracking objects/face
S/L: startles to loud noice
S: social smile
<span><em>(In GP examining bb - check for head control, using steth and watching bb track is w eyes. Someone accidentally bangs room door and bb cries, then parents comforts them and they smile)</em></span>
2
Q
Developmental milestones - 6 months
A
Summer
GM: sits unsupported with rounded back, rolls tummy (prone) to back (supine)
FM: palmar grasp and transfer hand to hand
S/L: turns head to loud sounds, understands bye bye, babbles
S: puts objects in mouth, shakes rattle, reaches for bottle
<span><em>(Imagine bb on beach in middle of summer. Can sit w rounded back bc 6 is curved - rolling from front to back catching sunrays. Palmar grasp under the palm trees and passing stuff between hands - turn to a loud squawking bird and babbling away, mum says “say bye bye birdie”)</em></span>
3
Q
Developmental milestones - 9 months
A
End of September - autumn & falling leaves
GM: stands holding on, sits w straight back
FM/V: inferior pincer grip, object permanence
S/L: responds to own name, imitates adult sounds
S: stranger fear, hold and bites food
<em>(Autumn park able to sit up in autumn leaves or stand holding hand. Can pick up leaves w pincer grip. Dad calls name in park and responds. Stranger approaches and cries. Can play peekaboo bc of object permanence and hold and bite food at picnic)</em>
4
Q
Developmental milestones - 12 months
A
Christmas time
GM: walks alone
FM: Casting bricks/toys (dropping them on the floor), can build a tower of 2 bricks
S/L: shows understanding of nouns “where’s Santa?”, speaks 3 words
S: waves bye bye, hand clapping, plays alone if someone nearby, drinks from beaker w lid
<em>(Walking to Xmas tree, putting small present on top of another. Could understand “Where’s Santa?” and will wave bye bye. 3word vocab “happy new year” - lol realisticlly mama, papa, santa. Claps to Xmas song and drinks festive drink w lid)</em>
5
Q
Developmental milestones - 18 months
A
2nd summer
GM: runs (16), jumps (18)
FM: scribble to and fro, stack 4 bricks
S/L: shows understanding of nouns “show me the pool”, 1-6 words
S: imitates everyday activities
<em>(Posh resort - running and jumping by pool. Parents give crayon and paper to keep them occ whilst sipping beers - scribble back and forth. Can stack double no. suitcases (LOL) from Xmas. Copies parents.)</em>
6
Q
Developmental milestones - 2years
A
2nd birthday - “2 themed party”
GM: runs on (2) tiptoes, walks upstairs (2) feet 1 step at a time, throws ball (2) someone
FM/V: draws vertical line, stacks 8 bricks (2 circles in 8), turns (2) pages of book
S/L: shows understanding of verbs, (2)-3 words sentences, 50 word vocab
S: eats skillfully with spoon (2os in spoon)
7
Q
Developmental milestones - 3 years
A
GM: hops on one foot for (3) steps, walks upstairs 1 foot per step but downstairs 2 feet per step (1+2=3)
FM/V: draw a circle, build a bridge
S/L: understand negatives, understand adjectives
S: Begin share toys, eat alone w/o parents, use fork and spoon, bowel control
8
Q
Developmental milestones - 4 years
A
Starting reception
GM: Walks up and down stairs in adult manner
FM/V: Draws a cross, build 12 blocks, cut paper in half
S/L: Understands complex instructions
S: Concern/sympathy for others, best friend, bladder control. eats w/o help. dress/undress
9
Q
What can hand preference under the age of 2 suggest?
A
Cerebral palsy
10
Q
Traffic light system
A
11
Q
Vaccination schedule
A
Key
Hib = Haemophilus influenzae B vaccine
PCV = Pneumococcal Conjugate Vaccine
Men B = Meningococcal B vaccine
Men C = Meningococcal C vaccine
Men ACWY = Meningococcal vaccine covering A, C, W and Y serotypes
MMR = Measles, Mumps, Rubella vaccine
HPV = Human Papilloma Vaccine
12
Q
Mx upper airway in children
A
13
Q
Signs of resp distress
3Es
A
14
Q
Asthma
A
Wheezing inbetween viral infections and evidence of allergy
15
Q
Asthma long term Mx
A
- Escalate asthma if:
- Using SABA>3x/week
- Syx > 3x/week
- Waking from syx
- Hospital admission –> steroid use
SABA = short acting beta agonist, i.e., albutamol inhaler (PRN)
ICS = inhaled cortico-steroid, i.e., beclometasone (regular)
LTRA = leukotriene receptor antagonist, i.e., montelukast
LABA = long acting beta agonist
MART = maintenance and reliever therapy (regularly + PRN in 1 inhaler)
*not often diagnosed until 5y/o unless wheezy despite lack of viral infx + strong family hx
*<5y/o if given ICS again - lower dose and long term
16
Q
Asthma life threatening presentation
A
- PEFR <33% (peak expiratory flow rate)
- SpO2 <92%
- Altered consciousness
- Arrhythmia
- Hypotension
- Cyanosis
- Poor respiratory effort
- Silent chest
17
Q
Acute asthma presentation
A
18
Q
Asthma exacerbation levels
A
19
Q
Acute asthma mx
A
- Salbutamol nebuliser
- Helps to open up airway and improve breathing
- Ipratroprium (Atrovent) nebuliser
- Short acting muscarinic antagonist helps relax smooth muscle around bronchi
- IV Magnesium sulphate + aminophylline + salbutamol
If life threatening: CALL FOR SENIOR HELP - consider intubation and ventilation
On D/C: Oral prednisolone (3-5 days)
(dampens down inflammation)
20
Q
Acute epiglottitis presentation
A
- Stridor
- Drooling
- Rapid onset fever
21
Q
Acute epiglottits cause
A
Haemophilus influenzae B
22
Q
Bronchiolitis
A
Inflammation of bronchioles (small airways) due to respiratory syncytial virus (RSV) - lower airway problem -> inflamm and mucus narrows airway ->less airflow
23
Q
Bronchiolitis presentation
A
Under 1 y/o!!
- Dry cough
- SOB
- Wheeze
- Crackles/crepitation
24
Q
Bronchiolitis RF
A
- Formula milk
- Being younger than 3 months
- Premature
*Congenital heart disease can cause bronchiolitis to be more severe
25
Bronchiolitis Ix
Clinical diagnosis
* Resp exam
26
Bronchiolitis Mx
Conservative
* Self limting 5-7 days
* Simple analgesia
* Oxygen as required
* Intubate and ventilate if really worried
* Encourage reg feeds
* NG tube if normal feeds not tolerated
! Can be very severe in vulnerable children, e.g., pre term infant
* Palivizumab
* To prevent severe bronchiolitis infx
27
When to admit Bronchiolitis pt?
* oxygen saturation persistently \<92%
* Feeding affected
28
Croup
Inflammation of the larynx and trachea (URTI) due to **parainfluenza** *(upper airway problem)*
29
Croup presentation
6 months - 2 years
* **Barking cough**
* **Stridor**
* SOB
* Fever
* Coryzal prodrome
OFTEN IN AUTUMN
30
Croup Ix
Clinical diagnosis
* Resp exam
31
Croup Mx
* Oral dexamethasone 0.15mg/kg
* Repeat if still mild stridor following day
If severe (significant airway compromise)
* Oxygen
* Nebulised adrenaline
* To try settle inflammation and open up airways
32
When to admit child with Bronchiolitis?
Consider if:
* RR \>60
* Difficulty breastfeeding/ inadequate oral fluid intake (50–75% of usual volume)
* Clinical dehydration
33
When to admit child with croup?
If moderate- severe
34
Why are children so prone to wheezing?
Small airways so only takes small amount of inflammation to narrow airways a bit more to cause whistling sound as air passes through
35
Multiple trigger wheeze
Wheezing due to multiple stimuli (e.g., infection, dust, pollen, smoking, cold air)
36
Viral induced wheeze
**Wheezing only** during viral infx caused by bronchoconstiction
Usually \<3yrs
Acute mx - asthma stairs exactly the same
37
Wheeze v stridor
Wheeze
Caused by small airways narrowing - causes rattling high pitched sound usually during (prolonged) expiratory phase
Stridor
Caused by upper airway obstruction/narrowing of some form - causes harsh sound primarily during inspiration
38
Laryngomalacia
Structural abnormality of the larynx which cause partial obstruction resulting in stridor/noisy breathing
39
Laryngomalacia mx
Self resolving
40
Pneumonia presentation
* Dyspnoea
* Fever
* Tachypnoea
41
Pneumonia Ix
* Resp exam
* Reduced air entry to specific area
* Creptitations ^
* O2 sats
* Lowered
* sputum culture
* Capillary blood gas
* CXR
* Consolidation
42
Cystic fibrosis
Autosomal recessive condition caused by mutation in CFTR gene on Cr7(cystic fibrosis transmembrance conductance regulator - Delta-F508 mutation most common)) leading to failure of chloride secretion.
Cl- important to get out of cells into extracellular space to places where you have secretions as Cl- draws water with it, making secretions less viscous and easier to move around/get out.
43
Main organ systems affected by CF
* Respiratory
* Thick airway secretions -\> encourage bacterial colonisation -\> more infx
* GI
* Thick pancreatic & biliary secretions -\> blockage -\> lack of digestive enzymes
* Endocrine
44
CF manifestations and mx
Respiratory
* Bronchiectasis
* Recurrent infx
Mx: Antibiotics (prophylactic flucox) + physiotherapy
GI
* Pancreatic exocrine insufficiency
* Diarrhoea
* Malabsorption
Mx: Vitamin supplementatin + nutritional support (high cal diet)
Endocrine
* Diabetes mellitus (due to pancreatic endocrine dysfunction)
* Random blood glucose \>11
* Osteoporosis (due to lack of absorption of ca and other minerals)
Mx: Diabetes mx / Vit D + Ca supplementation
+ MDT input!
\*CF itself - Ivacaftor/Lumacaftor
*Tries to improve construction of CFTR genes and insertion into membrane*
45
CF Ix
Sweat test
46
CF fertility
Male fertility affected as normal sperm but absence of vas deferens -\> cant be released
47
Tracheitis and epiglottitis (the other upper airway disease)
48
Meconium aspiration syndrome presentation
* Born through thick meconium
* Breathing problems
49
Meconium aspiration CXR
hyper inflated lungs with areas of consolidation
50
Respiratory distress syndrome Ix
CXR: Ground glass
51
Transient tachypnoea of the newborn presentation
* Tachypnoea within 1st 2h of life
* Grunting
* Use of accessory muscles
* Goes within 24h
52
Transient Tachypnoea of the newborn Mx
Supportive care ± O2
53
Scarlet Fever cause
Group A Streptococcus
54
Scarlet fever presentation
* Fever
* Sore throat
* Strawberry tongue
* Petechiae (small red spots) visible on the hard and soft palate are called 'Forchheimer spots'
* Blanching rash with rough, sandpaper-like texture
55
Scarlet fever Mx
10 day course of phenoxymethylpenicillin (penicillin V)
* Return to school 24h after commencing abx
* Notifiable disease
56
Whooping cough
Whooping cough is caused by bacteria bordella pertussis - attach to cillia of upper airway.
57
Whooping cause presentation
* Short period of coryzal symptoms followed by
* Onset of paroxysmal bouts of coughing
* Coughing intense, apnoeic, and may cause vomiting
58
Whooping cough mx
Presenting within 3 weeks start of cough
* Oral azithromycin or clarithromcyin
* Eradicate org and try reduce spread
(Admit and IV abx if pt clinically unstable)
Presenting \>3 weeks start of cough
* Advice
59
Vomiting differentials
* Posset - small volumes, painless, common
* No cause for concern
* Cow's milk protein allergy
* GORD - larger volume, relatively forceful
* No cause for concern if growth unaffected
* Intussusception
* Pyloric stenosis - forceful, projectile vomiting
* Red flag requiring further ix
60
Cow's milk protein allergy
Abnormal immune reaction to a protein found in cow's milk
61
Cow's milk protein allergy presentation
* Vomiting
* Diarrhoea or constip
* ±PR bleed - red tinged nappy blood
* Rash!
* Common in eczema!
* ±Wheezing
* ±Faltering growth
62
Cow's milk protein allergy mx
If breast fed
* Mother to avoid dairy
If formula
* Extensively hydrolysed formula
\*If growth still faltering -\> lack of compliance or different diagnosis
\*Most cases resolve by 5y/o
63
Duodenal atresia
The duodenum, which is the first part of the small intestine just beyond the stomach, is closed off/narrowed rather than being a tube
64
Duodenal atresia presentation
Low birth weight, premature, billious/non bilious vom, upper abdominal swelling
usually occurring within the first 24 to 38 hours of life after the first feeding
65
Duodenal atresia XR sign
double bubble
66
Duodenal atresia genetic syndrome association
Down's
67
Duodenal atresia mx
NG tube + IV fluids --\> surgery
68
GORD
Regurgitation of feed due to immaturity of lower oesophageal sphincter
Common in 1st year of life
69
GORD Mx
Breast fed
1. Breast feeding assessed by midwife/health visitor
* Position upright after feeds
2. Alginate therapy, e.g., Gaviscon infant
* small droplets given to baby - reacts w contents of stomach to form thickened, foam-like layer at top of stomach contents + generally
3. PPI or H2 antagonist - omeprazole
4. Paeds referral
Formula fed
1. Review feeding hx - smaller and more frequent feeds
2. Thickened formula or feed-thickeners
3. Alginate therapy added to formula
4. PPI or H2 antagonist (4 weeks)- omeprazole
5. Paeds referral
Usually resolves spontaneously - early weaning may be recc
70
Intussusception
Telescoping of proximal bowel into distal bowel causing an obstruction
71
Intussusception presentation
3 months - 2years
* Paroxysmal, colicky pain
* Bouts of abdo pain causing significant distress -\> screaming, drawing legs up
* Pallor
* Vomiting
* Non bilious at first, then once intestinal obstruction - vilious
* Sausage-shaped mass in abdo
* Red currant jelly stools (blood in stools - late stage feature)
72
Intussusception Ix
* Abdominal USS
* Target sign
* Abdominal XR
* If obstructed
* Contrast enema XR (gold standard)
73
Intussusception Mx
* Rectal air insufflation - pumping air into rectum and stretching out bowel allowing it to pop into place
Consider pathological lead point - an abnormality in tube where intussusception develops
74
Pyloric stenosis
Hypertrophy (narrowing) of pyloric muscle causing gastric outlet (opening from stomach to small intestine) obstruction
75
Pyloric stenosis presentation
2-8 weeks old, M\>F
* Projectile vomiting (non-bile stained)
* **Hungry** after feeds
* Visible persistalsis LUQ
* stomach tries to push its contents past the obstruction
* Olive shaped mass o/e
76
Pyloric stenosis Ix
* VBG
* Hypochloraemic, hypokalaemic alkalosis due to persistent vomiting
* USS
* assess pyloric sphincter and see whether enlarged - donut sign
* Gold standard
77
Pyloric stenosis mx
* Fluid resus - NBM and NG tube
* Ramstedt pyloromyotomy
* Surgical intervention where pyloric sphincter is opened up and relaxed
78
Constipation differentials
* Foecal impaction
* Hirschsprung disease
* Hypothyroidism
* Hypercalcaemia
79
Impaction - where can mass most often be felt?
left iliac fossa
80
Constipation mx
If impaction -
Disimpaction!
1. Movicol
* If unresolved 2 weeks/not tolerated*
2. Add stimulant laxative
If disimpacting not required/complete,
Maintenance dose movicol
81
Laxative classes (4)
* Softeners (more an oily material which infiltrates faecal masses and loosens them up)
* Lactulose
* Bulking agent (lend indigestible fibre to stool)
* Fybogel
* Osmotic laxatives (draw water into intestinal lumens and loosen up stools)
* Movicol
* Stimulants (augments intestinal contraction to help move things along)
* Senokot
82
Hirschsprung disease
Condition in which there is **absence of ganglion cells of the myenteric and submucosal plexus** -\> leads to area of bowel that is unable to contract appropriately to expel contents
Hirschsprung disease is a birth defect in which some nerve cells are missing in the large intestine, so a child’s intestine can’t move stool and becomes blocked.
(nerve fibres and ganglia help coordination contraction of bowels to keep things moving in one direction)
83
Hirschsprung disease presentation
Early in life
* Delayed/unable to pass meconium
* Abdominal distension
* Billious vomiting
Later
* Chronic hx constipation often later in childhood
84
Hirschsprung disease Ix
* Abdo XR
* dilated loops of bowel with fluid levels
* **Full thickness rectal biopsy**
* Observe absence of ganglion cells
85
Hirschsprung disease Mx
Supprotive
* Bowel irrigation
* Allow baby to pass meconium
Definitive
* Anorectal pull-through
* Removing affected chunk of bowel and re-anastomosing the ends to maintain continence
86
Hirschsprung associated genetic condition
Down's syndrome
87
Malrotation
Birth defect that occurs when the intestines do not correctly or completely rotate into their normal final position during development
88
Malrotation presentation
* Bile-stained vomiting
* Failure to thrive
* Painful/tender abdomen
* May also be paroxysmal w pulling up legs
* Bloating
* PR bleed/melaena
Usually \<1 y/o at presentation
89
Malrotation Ix
upper GI contrast study (gold standard) and USS
90
Malrotation Mx
Laparoscopic Ladd procedure to reposition intestines.
91
Malrotation complication
Volvulus - A volvulus happens when the intestine becomes twisted. This causes an intestinal blockage.
92
Meckel's diverticulum
Congenital diverticulum of the small intestine
Meckel's diverticulum is an outpouching or bulge in the lower part of the small intestine. The bulge is congenital (present at birth) and is a leftover of the umbilical cord.
93
Meckel's diverticulum presentation
Usually asyx
* Abdo pain mimicking appendicitis
* Rectal bleed
94
Meckel's diverticulum Ix
Technetium scan
95
Meconium ileus
Meconium is blocking the last part of the baby’s small intestine (ileum) --\> bowel obstruction.
Happens as meconium is more viscous and stickier than usual
96
What condition is meconium ileus often caused by?
Cystic fibrosis
97
Meconium ileus Ix
* Sweat test for CF
* Abdo XR
* distended coils of small bowel, but no fluid levels.
* PR contrast studies
* can be therapeutic
98
Meconium ileus Mx
* PR contrast studies
* May dislodge meconium plugs
* NG acetyl cysteine
* Liquifies meconium
If not, surgery
99
Mesenteric adenitis
Inflamed abdominal lymph glands
100
Mesenteric adenitis presentation
URTI and generalised abdominal pain
101
Necrotising enterocolitis
Intestinal tissue is inflamed and starts to die. This can lead to perforation, allowing intestinal contents to leak into abdomen.
102
Necrotising enterocolitis presentation
First 2 weeks life
* Feeding intolerance
* Abdominal distension
* Bilious vomiting
* Melaena
* ±signs of sepsis
* Common in premature infants
103
Necrotising enterocolitis Ix
* Abdo exam
* Reduced bowel sounds
* Abdo XR
* Dilated asymmetrical bowel loops
* Bowel wall oedema
* Air inside & outside bowel wall (Rigler sign)
* Air outlining falciform ligament (football sign)
104
Congenital diaphragmatic hernia
Herniation of abdominal organs into the chest cavity due to incomplete formation of the diaphragm
105
Congenital diaphragmatic hernia presentation
Respiratory distress
106
Congenital diaphragmatic hernia Ix
* Cardio + resp exam
* Heart sounds absent on LHS
* Tinkling sounds heard (bowel)
* Abdo exam
* Scaphoid abdomen (raised level of chest where abdo is)
* CXR
* Bowel loops can be seen in thoracic cavity
107
Congential diaphragmatic hernia Mx
Initial
* Intubation and ventilation
* NG tube with aim of keeping air out of gut
Definitive:
Surgical repair of diaphragm
108
Neonatal jaundice causes
**\<24 hours -\> Pathological**
_Haemolytic_
* Rhesus incompatibility
* ABO incompatibility
* G6PD deficiency
* Pyruvate kinase deficiency
* Hereditary spherocytosis
_Congenital_
* **T**oxoplasmosis
* **O**thers (e.g., syphilis)
* **R**ubella
* **C**MV
* **H**SV
**24 hours - 2 weeks**
* Physiological
* Common
* Increased RBC breakdown following birth - trauma
* Decreased bilirubin metab due to immature liver
* Breast milk jaundice
* Pathological (causes as above and below)
**\>2 weeks**
* Biliary atresia
* abnormal development of biliary tree
* pale sools, bruising
* Congenital hypothyroidism
* Neonatal hepatitis
109
ABO incompatibility jaundice
mother has the blood type O (and therefore has antibodies against A and B cells) and her newborn is of blood type A or B
110
Neonatal jaundice Ix
\<24 hours
* Serum bilirubin
* Group and save
* DAT test (Coombs'/direct antibody test)
* See whether antibody binding to neonate blood cells
* Autoimmune haemolytic anaemia
* FBC + CRP
* Blood culture
\>2 weeks
* TSH
* LFTs
* Bilirubin
* Transcutaneous (TC) bilirubin
* Non invasive
* Less accurate - more for screening
* Used if jaundice \>24h or born \>35weeks (lower risk of pathological cause of jaundice)
* Serum bilirubin
* Used if jaundice at \<24h or born \<35 weeks gestation
* Hepatobiliary scintigraphy/nadionucleotide scan
* If considering biliary atresia
111
Neonatal jaundic Mx
1. Phototherapy
* Light therapy (eyes protected and temp monitored) where unconjugated bilirubin converted to water-soluble pigment which can be excreted
* Bilirubin checked every 6-12h via heel prick test blood gas
2. Intensified phototherapy
* +/- IVIG
* Primarily used in ABO or rhesus haemolytic disease
\*carry on until 5 boxes below line on trx threshold graph:)
or Exchange transfusion - removal of infant blood and replaced w donor blood
112
Biliary atresia presentation
jaundice, pale stools and dark urine
113
Biliary atresia Ix
* High levels conjugated bilirubin
* Liver is doing its thang but not able to excrete through biliary duct into bowel
* USS abdo - contracted/absent GB
* **Liver biopsy**
114
How is biliary atresia managed?
Surgical intervention - Kasai procedure
removing the blocked bile ducts and gallbladder and replacing them with a segment of child's small intestine
115
Neonatal jaundice complx
Kernicterus
Unconj bilirubin can cross BBB and deposit in basal ganglia -\> can result in cerebral palsy
116
Antenatal resp-cardio adaptation
* Oxygenated blood from umbilical vein
* Foramen ovale
* Right to left shunt (due to lower Pa on RHS)
* Ductus arteriosus
* Less resistance to go to aorta rather than pulm arteries
* Lungs are non functional (high pulmonary vascular resistance)
117
Innocent murmurs
* Systolic
* Soft / low amplitude
* Change position when breathing
* Single
* Short duration
118
2 types of congenital cyanotic heart diseases
Cyanotic heart failure = right to left shunt (deoxygenated blood in systemic circ)
* Transposition of the great arteries
* Tetralogy of fallot
119
Main features of cyanotic heart disease
Cyanosis onset
* Within days -\> TGA
* Within months -\> TOF (+murmur)
| (When exerting themselves, e.g., feeding)
120
Transposition of the great arteries
Aorta and pulmonary trunk are switched around
Two completely separate circuits
* RHS (aorta) is systemic - goes to body and back
* LHS (pulm trunk) goes to lungs and back
\*Ductus arteriosus maintains life by allowing blood from LHS (oxygenated) to mix with RHS and aorta -\> rest of body
121
Abnormalities in tetralogy of fallot
1. Pulmonary stenosis
* Narrowing of pulmonary valve -\> lots of resistance going back towards RHS of heart
2. Ventricular septal defect (VSD)
* Allows blood to mix between 2 sides of heart
3. Overriding aorta
* Large proportion of outflow from heart going through aorta over pulmonary artery
4. Right ventricular hypertrophy
* RHS having to pump against higher pressure than used to due 1) and 3)
122
TofF murmur
systolic ejection murmur at the left upper sternal border
123
Cyanotic heart disease ix
* Pulse oximetry
* TGA - suddenly profoundly hypoxic as DA is closing
* Hyperoxia test
* Sats probe placed on infant then + high flow O2 -\> check if improvement
* TGA - no improvement as circuits are separate and sats probe only showing peripheral sat
* CXR
* TGA: egg on a string
* TOF: boot shaped heart
124
Cyanotic heart disease mx
* IV prostaglandin infusion
* Maintain patency of DA
* Surgery
125
Acyanotic heart disease (4)
Defects in embryological development of heart or its major vessels (left to right shunt w/ exception of CoA)
* Ventricular septal defect (VSD)
* Hole in septum betwen ventricles
* Most common
* Atrial septal defect (ASD)
* Hole in spetum between atria
* Patent ductus arteriosus (PDA)
* Ductus arteriosis remains open beyond 1 month (usually shuts before 1 month)
* Coarctation of the aorta
* Narrowing of aorta
126
Acyanotic heart disease presentation
Asymptomatic, or HF signs:
* Fatigue
* SOB
* Exercise intolerance
* Poor feeding/failure to thrive
* (Systolic) murmur
127
Murmurs associated w/ ASD, VSD and PDA
* VSD: systolic murmur at left sternal border
* ASD: soft midsystolic murmur at the upper left sternal border with splitting of the 2nd heart sound (S2)
* PDA: continuous 'machinery' murmur over left clavicle
128
AVSD complx and association
May lead to pulmonary hypertension + associated with Down syndrome
129
PDA mx
* NSAIDs
* e.g., indomethacin
130
Coarctation of aorta specific sign
Brachio-femoral delay
Can feel strong brachial pulse, but delayed and v weak or even absent femoral pulse
131
Acyanotic heart disease complx
* Heart failure (SOB)
* Faltering growth
* Recurrent chest infx
* Infective endocarditis
132
Anaphylaxis
Severe allergic reaction
133
Anaphylaxis presentation
* Stridor
* Angioedema
* Urticaria
134
Anaphylaxis Mx
1. Call for help +
2. A to E
* 2 large bore cannulae
3. Lie patient flat
4. **Definitive** (to carry on if anaphylaxis persists / refractory anaphylaxis)
1. IM Adrenaline 1:1000
* age adjusted doses
2. Repeat after 5 mins if needed
5. IV fluid challenge - crystalloid
6. IM or slow IV chlorphenamine + hydrocortisone
**Upon d/c**
* Safety net
* Avoiding allergen (if known)
* EpiPen
* Allergy clinic
135
Diabetic ketoacidosis
Pancreatic beta cells are not producing insulin -\> serum glucose concentration increases considerably and increased ketone generation.
136
DKA presentation
* Polyuria -\> dehydration
* N+V
* Thirsty
* Abdo pain
* Unexplained weight loss
* Coma
137
Ketones
* How are they produced?
* How are they useful?
* How are they harmful?
* Ketone bodies are produced during B-oxidation of fatty acids when body is low on glucose supply.
* Ketones allow brain to continue functioning in a time when glucose is scarce.
* They are acidic -\> enzyme dysfunction -\> coma & death:(
138
Actions of insulin (2)
* Reduces serum glucose concentration via
* push glucose into hepatic glucose store
* push glucose into tissues
* Block ketone generation (within liver)
139
DKA Mx
* IV fluid resuscitation
* Shock
* Bolus **20ml/kg 0.9%NaCl** over 15 mins
* Not in shock
* Bolus **10ml/kg 0.9% NaCl** over 1h
* + Deficit \* weight (in kg) \* 10 --\> total deficit vol(ml) /48h
* pH\>7.1 = 5%
* pH\<7.1 = 10%
* + Maintenance fluids
Fluid choice: **0.9% NaCl** + **40mmol KCl**, then once glucose \<14mmol/L add **5-10%** **dextrose**
* Insulin
Long term
* Education
* Family support
* Insulin rotating sites
* MDT care: diabetic nurse, dietician, paeds
* Annual r/v
140
IV Fluid resuscitation
Bolus (if in shock) + replacement fluid + maintenance fluid
141
How much bolus fluid for fluid resus?
20ml/kg/10mins
142
How much replacement fluid for fluid resus?
100ml/kg/24h
143
How much maintenance fluid for fluid resus?
100ml/kg for first 10kg +
50ml/kg for 2nd 10kg +
20ml/kg thereafter
144
CIs for LP
* Raised ICP
* Profoundly impaired consciousness
* Focal signs
* Focal or prolonged fits
* Cardio-pulmonary compromise (ie in shock or needing artificial ventilation)
* Local skin infection
145
Neonatal sepsis signs
* Respiratory distress (85%)
* Grunting
* Nasal flaring
* Use of accessory respiratory muscles
* Tachypnoea
* Tachycardia
* Apnoea
* Change in mental status/lethargy
* Jaundice
* Seizures
* if cause of sepsis is meningitis
* Poor/reduced feeding
* Abdominal distention
* Vomiting
* Temperature
\*Frequently, the symptoms will be related to the source of infection (e.g. pneumonia + respiratory symptoms, meningitis + neurological symptoms)
146
Status Epilpeticus
Single continuous seizure or repetitive seizures ± recovery period of consciousness lasting 5 or less mins.
147
Status epilepticus Ix
* Glucose
* U&E
* Calcium
* Anticonvulsant levels
* Toxicology screen
148
Status epilepticus Mx
1. IV Lorazepam
* If no IV access --\> rectal diazepam or buccal midazolam*
* *after 10 mins**
2. IV Lorazepam (2nd dose)
3. Phenytoin infusion
4. General anaesthesia
149
Testicular torsion
Rotation of testicle leading to lack of blood supply as the spermatic cord which brings blood to scrotum is twisted
150
Testicular torsion presentation
* Severe testicular pain
* Nausea and vomiting
* Swelling of the testis with overlying erythema
* Absent cremaster reflex
151
Achondroplasia presentation
* Short stature
* Macrocephaly
* Bowing of legs
* Trident config of hands
(Dwarfism vibes)
152
Developmental dysplasia of the hip
Condition in neonates where acetabulum is shallow -\> head of demur does not actually sit within acetabulum and can slip out quite easily
153
DDH presentation in non-neonates
Painles intermittent limp
154
DDH RF
* female sex: 6 times greater risk
* breech presentation
* family history
* firstborn children
* oligohydramnios
* birth weight \> 5 kg
155
DDH Ix
* Barlow and Ortolani manoeuvres during newborn infant screening check
* USS at 6 weeks (if suspcion from B&O test)
* If present with possible DDH beyond 6/12, hip XR
156
DDH Mx
* Spontaneous resolution
* Pavlik harness
* Holds infant's hips in a flexed position -\> helps burrow head of femur into acetabulum
* Surgery
157
Duchenne muscular dystrophy
Duchenne muscular dystrophy (DMD) is a genetic disorder characterized by progressive muscle deterioration and weakness
158
DMD presentation
* Onset in early childhood
* Walking on tip toes
* Enlarged calves
* Calf pseudohypertrophy
159
DMD cause
Alterations of a protein called dystrophin which helps keep muscle cells intact
160
DMD Ix
* Gower's sign
* +ve
* child uses arms to stand up from a squatted position
* CK
* Raised
* Genetic testing
161
What is the systemic onset of Juvenile Idiopathic Arthritis called?
Still's disease
162
Osgood schlatter disease
inflammation of the area just below the knee where the tendon from the kneecap (patellar tendon) attaches to the shinbone (tibia)
163
Osgood schlatter disease presentation
* Active child/teen
* Pain on one KNEE
* Systemically well
164
Osgood schlatter Mx
Rest, ice, analgesia, stretching + strengthening exercises
165
Osteogenesis imperfecta presentation
* Bowing of legs
* Prone to fractures
* Discolouration of sclera
166
Osteomyelitis
bacterial infection from another part of the body spreads to the bone
167
Osteomyelitis presentation
* Fever
* Fussiness or irritability
* Tiredness
* Area of infected bone:
* Tenderness or pain
* Not using the affected arm, leg or other part of the body
* Swelling/redness
168
Osteomyelitis v transient synovitis
Transient synovitis is response to a previous infx - does not present until days-weeks after infx
169
Perthes disease
Avascular necrosis of femoral head
too little blood is supplied to the ball portion of the hip joint (femoral head). Without enough blood, this bone becomes weak and fractures easily.
170
Perthes disease presentation
* 5-10 year olds
* Gradual onset of limp
* Gradual onset of HIP pain
* Pain on internal rotation and abduction
171
Perthes disease RFs
* Short stature
* Hyperactivity
172
Perthes disease trx
* Analgesia + mobilisation
* Surgery
173
Rickets presentation
* Pain
* Bones affected can be sore and painful
* Reluctant to walk/ waddling/ tire easily
* Skeletal deformities
* Thickening of the ankles, wrists and knees
* Bowed legs
* Soft skull bones
* Dental problems
* Delay in teeth coming through and increased risk of cavities
* Poor growth and development
* Fragile bones
* Weaker and more prone to fractures
174
Septic arthritis presentation
* Severe joint pain
* sually in just 1 joint, that started suddenly
* Swelling around a joint
* Skin around a joint has changed colour
* High temperature
175
Juvenile idiopathic arthritis
Arthritis occurring in someone who is less than 16 years old that lasts for **more than 6 weeks**
176
Still's presentation
* pyrexia
* salmon-pink rash
* lymphadenopathy
* arthritis
* uveitis
* anorexia and weight loss
177
Still's/JIA Ix
* ANA
* May be positive
* Rheumatoid factor
* Negative
178
Slipped upper femoral epiphysis (SUFE)
Fracture through growth plate resulting in slippage of femur
head of the femur slips off the neck of the bone at the growth plate
179
SUFE presentation
* 10-15 year old (often obese)
* Gradual onset of limp
* Gradual onset of hip or knee pain
* Pain on internal rotation and abduction
180
SUFE RFs
* Obesity
* Endocrine abnormalities
* hypothyroidism
* hypogonadism
181
SUFE Mx
Internal fixation to realign two components of femur
182
Transient synovitis
inflammation of the synovial lining of the hip joint
(a type of reactive arthritis)
183
Transient synovitis presentation
* Acute onset limp
* Fever
* Generally preceeded by viral URTI 1-2 weeks prior
184
Limp/ hip pain + fever Mx
Referral for same-day assessment -MRI
| (in case of septic joint)
185
Likely cause of spiral fracture and mx?
NAI
Admit and alert safeguarding team
186
What is the main aim of primary haemostasis?
Platelet plug formation
## Footnote
Endothelial injury -\> vWF (kind of like adaptor) sticks onto exposed subendothelial structures -\> platelets (brown) bind to vWF or even directly onto subendothelial structures -\> platelets become activated & bind to e/o -\> fibrinogen (inactivated) slots itself between platelets
+ vWF exists as multimers before being cleaved -\> v sticky and prone to stick onto endothelium even in absence of injury; cleaved by ADAMTS13
187
What is the aim of secondary haemostasis?
Formation of fibrin (from fibrinogen) by coagulation cascade
## Footnote
Tissue factor is a protein found on cells that make up your tissues that's exposed only when there is endothelial damage -\> tissue factor exposure activates factor VII-\> VIIa (extrinsic pathway) -\> no. enzyme reactions -\> conversion of fibrinogen to fibrin -\> fibrin mesh keeps platelets together
188
Microangiopathic haemolytic anaemia (MAHA)
Red cell breakdown in small vessels resulting in anaemia.
## Footnote
Obstruction in small vessel, usually platelet plug, causing RBCs to be damaged (shearing) when passing through vessel.
\*Mechanism not disease in itself
189
Haemolytic uraemic syndrome triad
Haemolysis (due to MAHA) + thrombocytopenia (low platelets) + renal failure
190
Cause of haemolytic uraemic syndrome
E. coli O157:H7
## Footnote
E. coli produces shiga-like toxin --\> causes glomerular endothelial damage --\> platelet plug formation (c) --\> RBC gets fragmented/non-intact (a) in trying to pass --\> poor end organ, kidney, perfusion (b) -\> renal failure
3 features: (a) haemolytic, (b) uraemic, (c) thrombocytopaenia
191
HUS presentation
* Bloody diarrhoea
* Pale
* Poor UO
* Nosebleed
192
HUS Ix
* FBC
* Low Hb
* Low Plt
* Creatinine
* High due to poor renal function
193
Henoch-Schönlein purpura
capillaries become inflamed and damaged
194
HSP presentation
* raised red or purple spots (purpura) non-blanching
* confined to buttocks, extensor surfaces of legs and arms
* joint pain
* abdo pain
(some overlap with ALL!)
195
HSP Ix
* BP
* May be raised
* Urinarlysis
* +Protein
* RBC
* NORMAL platelet level
* Kidney function
196
HSP Mx
Self-resolving
+paracetamol/NSAIDs if pain
197
Immune/Idiopathic thrombocytopaenia
Immune-mediated reduction in platelet count
198
ITP presentation
* Bruising
* Petechial or purpuric rash
* widespread
* Splenomegaly
* Atypical feature
* (Recent cold)
199
ITP Ix
* FBC
* **Isolated low platelets**
* Blood film
* Bone marrow biopsy
* IF ATYPICAL FEATURES/ failure to respond to trx
200
ITP Mx
* Usually no trx
* Resolves in around 80% children within 6/12
* Avoid activities that may result in trauma
* E.g., contact sports
If platelet VERY low (\<10), or significant bleeding
* Oral/IV corticosteroid
* IV Ig
* Platelet transfusion
* in emergencies, but only temp as they are desroyed by circulating antibodies
201
Thrombotic thrombocytopaenic purpura pentad
MAHA + thrombocytopaenia + renal failure + fever + altered mental state
202
TTP presentation
* Confusion
203
Cause of TTP
**ADAMTS13 deficiency** --\> increased sticky, uncleaved, vWF multimer -\> stick onto endothelial wall --\> platelet plug formation --\> insufficient end organ perfusion
204
Disseminated Intravascular Coagulation
Clotting within blood vessels throughout body
205
Cause of DIC
**Excessive tissue factor exposure** most commonly due to sepsis, tumour and pancreatits.
## Footnote
Excessive exposure tissue factor --\> excessive activation factor of extrinsic pathway --\> lots of tiny clots formed --\> very depleted platelets (anticoag) and clotting factors (procoag) due to increased consumption
\*We exist in a procoag state (bc historically we are more at risk of bleeding to death), and the anticoag state adjusts itself in response to procoag levels. When all factors are gone -\> tips towards not clotting at all
206
DIC presentation
* Widespread bleeding from orfices
* Fever
* Shock
207
DIC Ix
* aPTT (increased)
* PT (increased)
* Platelet (low)
208
Haemophilia presentation
* Painful joint with limited range of movement
* Swelling on bumping limb
* Bluish discolouration/purpuric rash to the overlying skin
209
Haemophilia Ix
* FBC
* ESR
* CRP
* May be lil raised
* PT and bleeding time is normal
* APTT
* **elevated**
210
Lymphoma
Cancer of the lymph nodes
211
Lymphoma presentation
* Swollen glands - painless lymphadenopathy
* Pruritus
* B symptoms in poorer prognosis
* Fever
* Nightsweats
* Weight loss
212
Non Hodgkin's Lymphoma
* 90% of lymphomas
* B or T cell
* Affects multiple groups of nodes
213
Hodgkin's lymphoma
* Only affects B cells
* Usually only affects single group of lymph nodes in 1 specific area
* Reed-sternberg cells
* owl
214
SCD Presentation
* Intermittent abdominal pain
* Generalised aches
* Pale, febrile and icteric
* Splenomegaly
* **Swelling** of wrists and **fingers**
215
Nephritic syndrome
Inflammation of glomerulus and nephrons
## Footnote
\*Leaky walls --\> blood and protein leaking out into tubule
216
Nephritic syndrome causes & presentation
* IgA Nephropathy\* --\> abdo pain, rash, arthritis
* Henoch-Schonlein purpura --\> abdo pain, purpuric rash, arthritis
* related to IgA however it is widespread whereas IgA is renal
* Post-streptococcal glomerulonephritis\*\* --\> HTN
* Haemolytic uraemic syndrome
* SLE
* Goodpasture's syndrome
\*occurs 5-7 days post resp/GI infx
\*\* occurs 4-6weeks post group A streptococcal infx (pharyngitis, skin)
217
Nephritic syndrome Ix
* Urine dipstick
* Blood
* Protein
* Creatinine
* increased
218
Nephritic syndrome mx
* IgA Nephropathy
* BP control ± steroids
* Henoch-Schonlein purpura
* Self-resolving ± steroids
* Post-streptococcal glomerulonephritis
* BP control
219
Nephrotic syndrome
Heavy proteinuria + hypoalbuminaemia + oedema
\*Increased VTE risk due to proteinuria --\> clotting factor lost in abundance is antithrombin3 (main anticoag in body)
220
Nephrotic syndrome presentation
* Puffy eyes
* URTI
* Abdo bloating
* Oedema of ankles, sacrum
221
Nephrotic syndrome causes
**Podocyte effacement**
\*Podocytes integral in maintaining barrier to proteins at interface between glomerulus and bowmans capsule
* **Minimal change disease**
* Focal segmental glomerulosclerosis (more adults)
* Membranous glomerulonephritis (more adults)
222
UTI Presentation
* Vomiting
* Fever
* Poor feeding
* ±stomach pain
* ±dysuria
223
Chicken pox complx in immunocompromised child
Pneumonitis
224
Cytomegalovirus presentation
* Fever
* Aching muscles
* Tiredness
* Rash
* Sore throat
* Swollen glands
225
What pathogen is Glandular Fever caused by?
Epstein Barr Virus (EBC)
\*Glandular Fever a.k.a infectious mononucleosis
226
Glandular fever presentation
* Pyrexia
* Tender, swollen lymph glands, mainly in the neck (and often in the armpits and groin)
* Red, spotty rash
227
Glandular fever Ix
* FBC
* High WCC
* CRP
* High
* Monospot test
* Atypical/reactive lymphocytes
228
Glandular Fever Mx
Arrange admission if:
* Stridor
* Dehydration or difficulty swallowing fluids.
Otherwise:
* Paracetamol/ibuprofen for pain and fever syx
* Advise
* Syx 2-4weeks
* Tiredness is common and often last syx to resolve
* Exclusion from school not necessary
* Limit spread by avoiding kissing/ sharing eating or drinking utensils, and to thoroughly clean all items that may have been contaminated by saliva.
229
Hand foot and mouth disease cause
Coxsackie A16 virus, although other group A and B Coxsackie viruses may be causative
230
Hand, foot and mouth disease presentation
vesicular eruptions (ulcers) in the mouth and papulovesicular lesions of the distal limbs
+ fever
231
HSV complication
Encephalitis
232
Kawasaki disease
An illness that causes inflammation in blood vessels throughout the body
233
Kawasaki disease presentation
CRASH
* Conjunctivitis (bilaterally)
* Rash
* Adenopathy
* Cervical lymphadenopathy
* Strawberry tongue/cracked lips
* Hands and feet sweeling
& burn
Child under age of 5 + fever \>5 days
234
Kawasaki disease Ix
* Echo
* Check for complx of coronary artery aneurysm
235
Kawasaki disease Mx
IV Ig (Gama globulin)
/
High dose aspirin
236
Lymphadenitis presentation
* Fever
* Warm, tender enlarged lymph node on one side (usually in the neck, but can also be in the arm pit or groin)
* Skin redness over the lymph nodes
237
Measles presentation
* Fever + cold like syx
* Lymphadenopathy
* Koplik spots in mouth
* Flat red spots (maculopapular) that appear on the face at the hairline
* Spread downward to the neck, trunk, arms, legs, and feet
238
Complication of measles
Otitis media
239
Parvovirus B19 presentation
* "Slapped cheek" rash appearance
* Spreading to trunk and extremities
* Fever
240
What can Parvovirus B19 result in
Aplastic crisis in SCD pts
(rapid fall in hemoglobin levels associated with few or no reticulocytes - immature RBC)
241
What is Roseola caused by?
Human Herpes Virus-6
242
Roseola presentation
* High fever (greater than 39.5°C) for 3 to 7 days.
* Rash on torso and spread to arms, legs, back, face
* Atfer the fever disappears
243
Rubella presentation
* Low-grade fever
* Pink-ish rash
* Mild conjunctivitis
* Swollen and enlarged lymph nodes
* Cough
* Runny nose
244
Threadworms cause
Enterobius vermicularis
245
Threadworms presentation
* Asymptomatic (90%cases)
* perianal itching, particularly at night
* girls may have vulval symptoms
246
Threadworms Ix
applying Sellotape to the perianal area and sending it to the laboratory for microscopy to see the eggs
(however mostly treated empirically)
247
Threadworms Mx
* Hygiene measures for all household
* Mebendazole
* Single dose for whole household
248
Sudden infant death syndrome RFs
* prone sleeping
* parental smoking
* bed sharing
* hyperthermia and head covering
* prematurity
249
Risk of maternal alcohol use
Foetal alcohol syndrome
* Microcephaly (small head)
* Absent philtrum
* Cardiac abnormalities
* Reduced IQ
| (depending on severity)
250
Foetal Alcohol syndrome presentation
* Jittery baby
* Sensitive to external stimuli
* Poorly developed philtrum
251
Risk of maternal smoking
Increased risk of
* Miscarriage
* Stillbirth
* Pre-term labour
252
Risk of Rubella infx during preg
* Cataract
* Deafness
* Cardiac abnormalities
± jaundice, hepatosplenomegaly, microencephaly, reduced IQ
\*Most at risk first 16 weeks.
253
Attention Deficit Hyperactivity Disorder features/criteria
* Hyperactivity
* Impulsivity
* Inattention
* Psychological, social or educational impairment that is pervasive
* 2 or more settings
254
ADHD Mx
\<5 y/o
* ADHD-focused group parent-training programme
\>5y/o
* Training programme
* Medication
1. Methylphenidate
2. Lisdexamphetamine
3. Atomoxetine or Guanfacine
* Monitor height, weight & ECG
255
Autism spectrum disorder features
* Impaired social interaction
* Not seeking close friendships/socially inappropriate behaviour/not understanding other's feelings
* Speech & language disorder
* Literal interpretation of speech/monotonous speech
* Routines and ritualistic behaviours
* Fixed ideas/interests/routines
256
ASD comorbidities
* Seizures
* MH disorders
257
ASD Mx
Behavioural modification
258
Cerebral palsy
Permanent disorder of movement and/or posture and of motor function due to a non-progressive\* abnormality in the developing brain
## Footnote
(\*brain damage does not change/evolve- same throughout life but manifestations will vary as child grows)
259
Cerebral palsy classification (3)
* Spastic (90%)
* Dyskinetic
* Ataxic
260
Cerebral palsy causes
* Antenatal (80%)
* Intracranial haemorrhage/ischaemia
* Genetic syndromes
* Congenital infx
* Perinatal
* Hypoxic-ischaemic injury
* Postnatal
* Intracranial infx
* Trauma
* Hydrocephalus
261
Cerebral palsy presentation
* Abnormal tone
* Delayed motor milestones
* Feeding abnormalities
* Asymmetrical hand function at \<12 months
* Hand preference
* Persistence of primitive reflexes
262
(spastic) Cerebral Palsy (described hemiplegic weakness with brisk reflexes), what area of the brain is affected?
Motor cortex
263
Difficult balance, cannot build block of towers, hyperreflexia (description of ataxic CP). Where is lesion?
Cerebellum
264
What would warrant paeds referral re febrile convulsions?
Still drowsy 2h after seizure
265
Focal seizures presentation
* Period of being difficult to communicate with
* Sleepy/falls asleep after
* No memory of abn event
266
Epilepsy Mx
* Tonic clonic, tonic, atonic, myoclonic - **Sodium valproate**
* ****but not in F of child bearing age
* Absence - **Ethosuximide**
* Focal - **Lamotrigine**
267
Congenital infections (main 5)
Toxoplasmosis
Others
Rubella
CMV
Herpes
268
Congenital infections keypoints
Toxoplasmosis
* Undercooked meat, raw eggs, handling cat litter
* Higher risk later in preg
* Baby: Usually no immediate syx then jaundice, lymphadenopathy, retinitis, developmental delay
Others
* Syphilis, VZV, Parvovirus B19
Rubella
* Highest risk in 1st trimester
* Vaccinate mother pre conception
* Baby: deafness, cataracts, congenital heart disease (PDA)
CMV
* Most common intrauterine infx
* Baby: low birth weight, microcephaly, seizures, hepatosplenomegaly, learning disability, deafness
Herpes
* Occurs when mother has active genital herpes during childbirth
* Baby: skin, eyes, mouth disease
269
What is the genetic abnormality for Turner's syndrome?
45X
270
What are the features of Turner's syndrome?
* Wide or weblike neck
* Low-set ears
* Broad chest with widely spaced nipples
* Bicuspid aortic valve and coarctation of aorta
* High, narrow roof of the mouth (palate)
271
What is the genetic abnormality for Klinefelter's syndrome?
47XXY
272
What are features of Klinefelter's syndrome?
* Taller than average stature
* Longer legs, shorter torso and broader hips for M
* Absent, delayed or incomplete puberty
* Post puberty, less muscle + facial and body hair for M teen
273
What are the featues of Fragile X syndrome?
* Balance problems
* Shaky hands
* Numbness in hands and feet
* Unstable mood
* Memory loss & cognitive problems
* Primary ovarian insufficiency in F
* Enlarged testes post pubert in M
274
What is the genetic abnormality for Patau's syndrome?
Trisomy 13
275
What is the genetic abnormality for Patau's syndrome?
Trisomy 13
276
What are the features of Patau's syndrome?
* Abnormally small eye or eyes (micropthalmia)
* Absence of 1 or both eyes (anophthalmia)
* Reduced distance between the eyes
* Problems with development of nasal passages
277
What is the genetic abnormality for Edward's syndrome?
Trisomy 18
278
What are the features of Edward's syndrome?
* Microcephaly (small head)
* Malformed and low set ears
* Microagnathia (small mouth &/or jaw)
* Cleft palate/cleft lip
* Clenched fists
* Club feet
* Rocker bottom feet
279
What is the genetic abnormality for Down's syndrome?
Trisomy 21
280
Down's syndrome features
* Face
* upslanting palpebral fissures (eyes)
* epicanthic folds (eyes)
* Brushfield spots in iris
* protruding tongue
* small low-set ears
* round/flat face
* Head
* Flat occiput (back of head)
* Hands/feet
* Single palmar crease
* Pronounced 'sandal gap' between big and first toe
* Hypotonia
281
Prader willi presentation
* Voraciously hungry kid
* Hypotonia
* Almond eyes
282
Acne
Acne is a skin condition that occurs when your hair follicles become plugged with oil and dead skin cells.
283
Acne Mx
Mild-moderate
* Benzoyl peroxide
* Benzoyl peroxide + clindamycin
* Adapalene
* Azelaic acid
If not responding
* Oral antibiotics
* Lymecycline or doxycycline for max 3 months
* Consider COCP (F)
Specialist referral - roccutane is popular trx option (extensive side effect profile)
* Severe
* Scarring
* Marked
* Psych distress
* Multipl treatment failure
284
Eczema presentation
Dry, scaly rash - typically occurring on flexor regions
285
Eczema Mx
* Emollients
* Liberal use regularly
* Skin has lost its ability to act as a barrier - trying to sustain barrier
* Steroids
* Mild: hydrocortisone 1%
* Moderate: Betamethasone valerate 0.025%, Clobetasone butyrate 0.05%
* High: Betamethasone valerate 0.1%, Mometasone
* Calcineurin inhibitors
286
What is this presentation?
Impetigo
Staphylococcal or streptococcal infection that presents with golden-crust like rash (often on face)
287
Impetigo Mx
Localised
* Topical fusidic acid
Extensive
* Oral flucloxacillin
* Clarithromycin if pen allergic
No school until lesions dry and scab over
288
Scalded skin syndrome presentation
* Skin feels off
* Fever
* Irritability
* Rash
289
Salmon patch presentation
Flat, pink lesion. May change colour, e.g., darker when bb cries
290
Retinoblastoma
Rare eye cancer in young children
291
UTI Treatment
Not requiring admission
* PO co-amox (Augmentin duo) or PO cephalosporin 5-7/7
Inpt
* IV cefotaxime + gentamicin 10/7
292
Acute lymphoblastic leukaemia presentation
* Recurrent sore throat
* Pallor
* Joint pain
* Massive hepatomegaly
* Petechial rashes
293
Acute leukaemia Ix
* FBC
* Hb low
* WCC high
* Plt low
* Neutrophils low
* Bone marrow biopsy
* Definitive
294
Suspected ALL
Send to A&E
295
NAI Mx
to involve more senior help
An on-call team for safeguarding children in every acute Trust.
During the day, the named nurse for child protection is likely to be the right person to contact. On-call, there will be a named consultant.
296
Caput succedaneum
lumps caused by scalp swelling due to pressure
crosses suture lines
297
Cephalohaematoma
blood that collects between a newborn's scalp and skull
does not cross suture lines
298
What can foreign body present with?
Unilateral nose bleed
299
Urinary continence at night Mx
* Positive reward system
* Enuresis alarm
* Desmopressin melts administered e/ night
300
At what age does enuresis become a problem?
From 5!
301
Meningococcal speticaemia presentation
* Temperature
* Reduced cap refill
* Bulging fontanelles
* Generally unwell
302
What is this presentation?
Slate grey nevi (Mongolian blue spots)
303
What is this presentation?
Malar rash
304
What can cause malar rash?
Rosacea
Lupus
305
What is non-blanching rash indicative of?
Neisseria meningitidis
306
What is this presentation and what to prescribe?
Umbilicated papules in molluscum contagiosum
Do nothing!
307
What is this presentation?
Blanching rash on trunk due to post-viral rash/reactive rash/ exanthem
308
What is this presentation and how to manage?
Eczema herpeticum
Eczema hx with exposure to HSV
Send to A&E!
309
What is this presentation and what to prescribe?
Mild erythema nappy rash
Barrier preparation to protect skin - Zinc, castor oil
(+Regular nappy changes, nappy-free time)
310
What is this presentation and what to prescribe?
Satellite lesions - nappy rash due to candida
Prescribe clotrimazole cream
311
What is this presentation and what to prescribe?
Chicken pox
Do nothing
312
What is this presentation and what to prescribe?
Scabies
Treat with permethrin
313
Phimosis
Tight foreskin
314
Phimosis presentation
* Foreskin ballooning on urination
* Non-retractile
315
Infantile spasms presentation
Infant with episodes of throwing arms forward with fists clenched
316
Reflex anoxic seizures presentation
* Child falls
* Parents pick them up
* Tonic-clonic seizure
* Completely well afterwards.
317
Breath-holding attacks presentation
* Cry and then be silent while holding their breath
* Turn blue or grey
* Be floppy or stiff, or their body may jerk
* Faint for 1 or 2 minutes
* Sleepy or confused for a while afterwards
318
Absence seizure presentation
* Sudden loss of awareness
* Suddenly stop talking/what they are doing and stare blankly into space
* Not response to people talking to them
319
CKD presentation
* Polydipsia (with no dysuria)
* Hypertensive
* Poor growth
* Anaemic/pale
320
Tonsilitis complx
Quinsy's
(Abscess between one of your tonsils and wall of throat)
321
Temper tantrum presentation
* Screaming
* Kicking
* Biting
* Crying
322
Chicken pox presentation
Itchy maculovesicular rash w fever started on chest and spread to arms
323
Chicken pox developed cool peripheries -\> Dx?
Viral meningitis
324
Neonate exposure to chickenpox mx
IV VZIG
325
Fussy eater blood film results
hypochromic microcytic anaemia and low ferritin
326
Constipation + 1 ep blood streaked poop Mx
reassure
327
What is West syndrome and what does it show on EEG?
Infantile spasms (+developmental delay) shows hypsarrhythmia on EEG.
328
Which TB drug can cause discolouration of tears/urine? (reddish-brown)
Rifampicin
329
Bacterial tonsillitis Mx
Phenoxymethylpenicillin
## Footnote
*Clarithryomycin in pen allergic*
330
Benign intracranial hypertension headache presentation
* Throbbing
* Worse in morning
* N+V
331
Migraine Mx
* Headache diary
* NSAIDs
* Nasal triptan, e.g., nasal sumatriptan
* if NSAIDs don't work
332
Hypoglycaemia newborn Mx
Asymptomatic
* Breast feed
Symptomatic
* Oral/IV glucose
333
Wilm's tumour (nephroblastoma)
Kidney ca affecting children which develops from nephroblast cells
334
Wilm's tumour presentation
* Painless abdominal distension
* Haematuria
335
Neuroblastoma presentation
* Painful abdominal bloating
* Abdo mass on palpation
* Haematuria
336
Traveller's diarrhoea + derranged LFTs
Hepatitis A infx
337
Screen for latent TB - when is it considered +?
Mantoux Tuberculin skin reaction test
Induration/firm swelling:
1) ≥5mm if recent contact someone w infectious TB
2) ≥10mm children under 5
3) ≥15mm no RFs
^Start anti-TB Trx
\*IGRA (Interferon gamma release assay) also offered in immunocomproised
338
Central precocious puberty Ix
gonadotropin stimulation test
339
On newborn check, inverted ankles and plantar flexed
Clubfoot - talipes equinus
340
ittery baby - what should your first test be?
Capillary blood glucose
341
Kid with diarrhoea for the last few months, with pieces of vegetables undigested, otherwise healthy
Toddler’s diarrhoea
342
Girl goes to pakistan. Develops macular rash. High fever for 5 days. HR was 70bpm. Cause?
Typhoid (relative bradycardia!)
343
Cannot examine ear directly, lump behind ear
mastoiditis
344
Sickle cell anaemia patient with hb of 40 and low reticulocyte count, Howell-Jolly bodies cause
Parvovirus B19 - causing aplastic crisis
345
Kid take medication for UTI, becomes anaemic and jaundiced, irregularly contracted cells and polychromasia
G6PD deficiency
346
Maternal T1DM increases risk of what condition in newborn?
Neural tube defects
347
kid fighting with brother, sustains injury where elbow is flexed and pronated, pain on supination. What is the injury?
Subluxation of the radial head
348
Child with a single enlarged lymph node in posterior cervical neck, had a viral infections one week age. How would you manage it?
reassurance and watchful waiting (Most cases of cervical lymphadenopathy are self-limited and require no treatment other than observation w f/u in 2 weeks)
If lymphadenopathy has not resolved after 2–4 weeks, arrange urgent referral to an ear, nose, and throat surgeon for further investigation, depending on clinical judgement.
349
Kid from Pakistan whose parents are first cousins has acute respiratory failure and pneumocystis pneumonia, what dx?
SCID
Most primary immunodeficiencies are inherited in an autosomal recessive pattern; therefore, they are more common in areas with high rates of consanguineous marriage
350
neonate with fever, sunken fontanelle and reduced right leg movement, most likely dx?
Osteomyelitis
351
Bilateral undescended testes in a phenotypically male newborn examination, most likely dx?
Klinefelter's
(if phenotypically F, androgen insensitivity syndrome)
352
Kid with acute otitis media, high temperature. What to do next?
Admit if: \<3/12 w temp \>38.5
Consider admit if: \<3/12, or 3-6/12 and temp \>39
Systemically unwell: immediate abx prescription
Systemically well: reassure (paracetamol) and r/v
353
15 year old girl who had generalised abdominal pain and then now right iliac fossa pain, she had a cough and coryza a few days previously. What is the most likely diagnosis?
mesenteric adenitis
354
Hearing difficulties in a 7 year old boy, which test would you do?
Pure tone audiometry
355
3 year old girl well-kempt, who has reached developmental milestones, nursery comments that she does not speak much, her mother says at home she is well-behaved and plays alone quietly. Most likely diagnosis?
selective mutism
356
10 month old child with UTI infection by non-E coli organism. USS was normal. What ix needed?
micturating cystourethrogram (MCUG)
357
Child migrated to the UK from Pakistan, with a history of recurrent chest infections and dropped some centiles on the growth chart. Most def ix?
Sweat test
358
Parents have noticed beer-coloured urine in a girl following URTI, what diagnosis?
Ig A Nephropathy (recent), Post-strep glomerulonephritis (weeks)
359
Neonate with red sticky eyes and purulent discharge - most likely cause?
2-5 days post birth: N. Gonorrhoea
5-12 days (?up to 28): C. Trachomatis
360
Young boy with swelling around eye. Which medication do you want to give?
Chloramphenicol
361
Child with JIA has weight gain, abdominal striae and plethoric face. What drug is he likely on?
Steroids
362
Child had sore throat, was given abx, a few days later a rash appeared. What is the likely cause of sore throat?
EBV
363
Child with non-blanching rash and fever at GP. What immediate tx to give?
IM benzylpenicillin
364
Trisomy 21 most common cardiac defects (in descending order) x3
1. AVSD
2. VSD
3. ASD
Pulmonary atresia
Mild coarctation of the aorta???
365
A 15 year old boy has 3 weeks of cough, weight loss, night sweats and fever. He moved to the UK from India 3 years ago. His chest x-ray is show in the image (can’t find exact image but showed some left upper zone shadowing I think). Which investigation is regarded as the **gold standard** for diagnosis for his underlying condition?
Sputum culture (gold-standard for active pulmonary TB)
366
A 3 year old girl in the paediatric Emergency Department has conjunctival pallor, lethargy and dark urine. Last week she finished a course of nitrofurantoin for a urinary tract infection. Her blood results are as follows: Hb 70 g/L (110-140), WBC 3.2 x 10^9/L (5.0 - 12.0), platelets 520 x 10^9/L (150 - 400), albumin 37 g/L (30 - 50), bilirubin 75 umol/L (\<21), ALT 18 IU/L (0 - 29), ALP 130 IU/L (60 - 425). Which is the most likely underlying diagnosis?
G6PD deficiency
367
A 7 year old boy has a history of recurrent chest infections, persistent sinusitis and has been prescribed multiple courses of antibiotics. He has bibasal crepitations and a right-sided apex beat. Which is the most likely underlying diagnosis?
Primary ciliary dyskinesia
## Footnote
**Kartagener syndrome**: **Primary ciliary dyskinesia** (no cilia movement-\> mucus plugs -\> bacterial colonisation -\> chronic bronchiectasis) + **persistent sinusitis** (impaired mucociliary clearance -\> recurrent sinus infx) + **dextrocardia** (no cilia movement -\> heart stays on RHS during embryogenesis)
368
A 3 day old baby girl is not feeding well. Her temperature is 36.7C, heart rate 180 bpm, respiratory rate 66 breaths per minute. There is a systolic murmur and femoral pulses are not palpable. Which is the most likely diagnosis?
Coarctation of the aorta
369
A 5 year old boy in the paediatric Emergency department recently had a sore throat and now has a rash across her lower legs. She is afebrile but has abdominal pain and joint pains in both legs. Her full blood count result is as follows: Hb 110 g/L (115 - 140), WCC 10.3 x 10^9/L (3.8 - 10), neutrophils 6 x 10^9/L (150 - 400). Which is the most likely diagnosis?
Acute lymphoblastic leukaemia
370
A 7 year old girl has breast buds but no pubic hair. Her height is on the 98th centile and her weight is on the 75th centile. She is otherwise fit and well. There is no significant family history. Investigations show: bone age of 10 years, FSH 3.2 IU/L (\<1), LH 3.4 IU/L (\<1). Which is the most likely diagnosis?
Central precocious puberty
371
A 3 year old boy attends the Emergency Department after an episode of sudden onset jerky movements of both arms and legs for 5 minutes, which stopped before the ambulance arrived. His current observations are: temperature 38.5C, pulse 130 bpm, blood pressure 100/90 mmHg, respiratory rate 25 breaths per minute, oxygen saturation 98% breathing air. He has had a coryzal illness for 24 hours and is otherwise fit and well. He is alert, with a blood glucose of 4.5 mmol/L (3.0 - 6.0) and neurological examination is normal. Which is the most appropriate next step in management?
Prescribe oral antipyretics and observe
(Observe as first time, benzodiazepine rescue med given if seizure \>5 mins)
372
Coeliac disease
Coeliac disease is a condition where your immune system attacks your own tissues when you eat gluten.
373
Coeliac signs/syx
* Fatigue/weakness
* N+V
* Failure growth
* Abdo distension
* Atrophic buttock muscle
374
Coeliax Ix
* Gold standard: duodenal biopsy
* Bloods
* IgA Anti TTG
* Anti-endomysial
* Serum IgA deficiency
375
Coeliax Mx
* Lifelong gluten free diet
* Pneumococcal polysaccharide vaccine/pneumovax as hyposplenic
376
What other conditions are coeliac associated with?
* Enteropathy associated T cell lymphoma
* Erethyma Herpetiformis
377
Volvulus Mx
* Admit to hospital
* Prepare for surgery
* NG tube to empty GI contents
* Keeps fluid and gas from building up
* IV fluids
* IV abx
378
Wilson's disease
Autorecess mutation in Cr13 -\> reduced synthesis of caeruloplasmin (copper binding protein) and defective excr copper in bile leading to accum of copper in liver, brain, kidney and cornea
379
Wilson's disease presentation
fatigue, worsining school performance + change in behaviour + jaundice + kayser-fleischer rings in cornea
380
Wilson's disease Ix
* Serum caeruloplasmin (low)
* Liver biopsy - high levels of copper in liver
381
Wilson's disease mx
penicillamine - promotes copper excr +ophthal + pt + school support
382
Meningitis 2ndry care mx
* Admit pt
* Senior input
* Start IV abx\*
* Send urine, blood and CSF for M, C&S
* Start maintenance IV fluids
\* \<3/12: IV cefotaxime and amoxicillin
\>3/12: IV ceftriaxone
383
Acute epiglottitis mx
Urgent referral to ENT, paeds, anaesthetics (visualisation under GA + intubation)
IV ceftriaxone
384
Query septic arthritis Mx
* FBC
* CRP
* Blood cultures
* Joint aspirate
* Refer for an orthopaedic opinion
## Footnote
*hip septic arthritis, aspiration needs to be done emergently by ortho*
385
T1DM dx criteria
* Symptoms +
* Biochemical:
* Fasting ≥7.0mmol/L
* Random ≥11.1mmol/L
* 2 hours post load \> 11.1
* HbA1c \> 48
\*If no symptoms 2 tests
386
Nephrotic syndrome Mx
reducing dose of steroids (pred).
If steroid resistant \> specialist
387
DKA dx criteria
All 3:
* Ketonaemia ≥3, or 2+ ketonuria
* Glucose ≥11mmol, or known DM
* Bicarb \< 15mmol &/or venous pH \< 7.3
388
389
FB inhalation presentation
Short hx of dyspnoea and cough
390
FB inhalation XR
Increased volume of translucency + unilateral hyperinflation
391
Acute appendicitis Ix
USS
If inconclusive, CT (gold standard)
392
Newborn testing
1) When?
2) Which tests?
1) 4-5 weeks
2) **Otoacoustic emission test**
then auditory brain response test (if former strange result)
393
Clubfoot mx
Ponseti method casting
394
Neuroblastoma Ix
Urine catecholamines: VMA and HVA high
395
Hirschsprung disease chromosmal defect
Cr 10
396
Crohns v UC endoscopy
Crohns: skip lesions and strictures from mouth to anus (esp terminal ileum)
UC: contunous distribution w/ no structures from rectus to colon
397
Crohns v UC biopsy
Crohns: transmural deep colon ulceration, cobble stone mucosa, granulomas
UC: mucosal lesions, crypt abscesses, pseudopolyps
398
Most common childhood arrhythmia
1) PC
2) Mx
SVT
PC: younger children - HF symptoms, older - syncope
Mx: 1) Vagal manouvre 2) IV adenosine
399
SCD - painful vaso-occlusive crisis mx
1. Analgesia (avoid morphine)
2. PO + IV fluids
3. If evid bact infx -\> Abx
4. If low O2 -\> O2
400
Minimal change disease complx
DVT
401
Neonatal life support
APGAR scores at 1, 5 and 10 mins
Assessing: colour, tone RR, HR
1. Dry/stimulate
* If gasping/not breathing*
2. 5 inflation breaths
3. Consider suction if visible obstruction
4. Repeat inflation breaths
5. 3 chest compressions : 1 ventilation breath
402
Chondromalacia patellae presentation
* Knee pain, no trauma
* Creptius palpable when knee extended
* Grating sensation
403
Chondromalacia patellae mx
Rest and analgesia
