Paediatrics Flashcards
(131 cards)
1
Q
what is physiological jaundice?
A
- high concentration of RBC in the foetus and neonate -> these are more fragile than normal RBCs + they have a less developed liver -> fetal RBCs break down more rapidly, leading to lots of bilirubin being released
- this bilirubin is usually excreted via the placenta however, there’s no longer a placenta so there’s a normal rise in bilirubin shortly after birth -> yellow skin and sclera form 2-7 days of birth
2
Q
causes of jaundice from an increased production and decreased clearance of bilirubin?
A
Increased production: - haemolytic disease of the newborn
- ABO incompatibility
- Haemorrhage
- Intraventricular haemorrhage
- Cephalo-haematoma
- Polycythaemia
- Sepsis and DIC
- G6PD deficiency
Decreased clearance
- prematurity
- Breast milk jaundice
- Neonatal cholestasis
- Extrahepatic biliary atresia
- Endocrine disorders (hypothyroid and hypopituitary)
- Gilbert syndrome
3
Q
why are breastfed babies more likely to have neonatal jaundice?
A
- components of breast milk inhibit the ability of the liver to process bilirubin
- Breastfed babies are more likely to become dehydrated if not feeding adequately
- Inadequate breastfeeding may lead to slow passage of stools, increasing the absorption of bilirubin in the intestines
4
Q
when is jaundice considered prolonged?
A
When it lasts longer than would be expected in physiological jaundice:
> 14 days in full term babies
> 21 days in preterm babies
5
Q
management of jaundice?
A
- treatment threshold chart to monitor total bilirubin levels
- These charts are specific for the gestational age of the baby at birth
- Age of Baby on x-axis and total bilirubin level on y
- If the total bilirubin level reaches the threshold, they need to be started on treatment to lower their bilirubin levels
6
Q
what is phototherapy and what’s measured after it?
A
- it converts unconjugated bilirubin into isomers that can be excreted in the bile and urine without needed conjugation in the liver
- Blue light shines on the baby’s skin and little to no UV light is used
- Double phototherapy involves 2 light boxes
- Bilirubin is closely monitored during treatment
- rebound bilirubin is measured 12-18 hours after stopping to ensure the levels don’t rise above threshold
7
Q
what is kernicterus?
A
brain damage caused by excessive bilirubin levels
- unconjugated bilirubin can cross the BBB
- Excessive bilirubin causes direct damage to the CNS - basal ganglia and brainstem
- Less responsive, floppy, drowsy baby with poor feeding
- The damage to the CNS is permanent ⇒ cerebral palsy, learning disability and deafness
8
Q
what are some conditions that can cause prolonged jaundice?
A
- biliary atresia
- hypothyroidism
- G6PD deficiency
9
Q
what are some issues in neonatal resuscitation?
A
- babies have a large SA:weight - get cold easily/risk of hypothermia
- babies are born wet, so loose heat rapidly
- meconium can get stuck in their mouth/airway
10
Q
neonatal life support summary
A
delayed cord clamping if possible -> assess tone, colour, breathing, heart rate -> if baby is gasping/not breathing, give 5 inflation breaths -> reassess and look for hear rate, chest movements, technique -> if chest not moving, repeat 5 inflation breaths -> if heart rate not improving and <60bp, start compressions and ventilation breaths at a rate of 3:1
11
Q
what is delayed cord clamping + benefits, risks?
A
delaying clamping the cord for 1 minute (in uncompromised neonates), to allow time for fetal blood in the placenta (significant volume of it), so enter the circulation of the baby = placental transfusion
- allows improved Hb, iron stores and BP + reduction in intraventricular haemorrhage and necrotising enterocolitis
- increased risk of neonatal jaundice
12
Q
difference between inflation and ventilation breaths?
A
inflation breaths last 2-3 seconds, ventilation breaths are shorter and last 1-2
13
Q
a timer is started at birth, what is the baby continuously assessed for?
A
- tone
- breathing effort
- heart rate
- colour
14
Q
when performing inflation breaths, what should be used?
A
- air should be used in term babies or near term babies
- a mix of air and oxygen should be used in pre term babies (upto 30% FiO2)
- if there’s concerns about the breathing, o2 sats can be monitored throughout the resuscitation
- aim for a gradual rise in o2 sats, not exceeding 95%
15
Q
how do you calculate the apgar score?
A
- appearance (skin colour), pulse, grimace (reflex irritability), activity (tone), respiration
- done at 1,5 and 10 minutes whilst resuscitation continues (if severely unwell, do an assessment at 20 minutes too)
- used as an indicator of the progress over the first minutes after birth
- helps stimulate neonatal resuscitation efforts
- lowest score = 0, highest = 10
16
Q
why are babies given vitamin K after birth?
A
- babies are born with a deficiency in vitamin K (important in blood clotting)
- IM injection of vitamin K is given in the thigh shortly after brith
- it reduces the risk of vitamin K deficiency bleeding, which can involve bruising, umbilical stump bruising and haemorrhage
17
Q
what is blood spot screening and how long does it take for the results to come back?
A
- carried out on day 5
- involves a heel prick to provide 4 separate drops of blood to test for 9 congenital conditions;
- sickle cell, CF, congenital hypothyroidism, phenylketonuria, MCADD, maple syrup urine disease, isovaleria acideaemia (IVA), glutamic aciduria type 1 (GA1), homocystein
- takes 6-8 weeks for the results to come bakc
18
Q
what is caput succedaneum?
A
- oedema collecting on the scalp outside the periosteum
- caused by pressure to a specific area of the scale during traumatic, prolonged or instrumental delivery
- as the fluid is outside the periosteum, it can cross the suture lines (sagittal)
19
Q
what is cephalohaematoma and what is it also known as?
A
- collection of blood between the skull and periosteum
- caused by damaged to the blood vessels during traumatic, prolonged or instrument delivery
- also known as traumatic subperiosteal haematoma
20
Q
how can you differentiate between cephalohaematoma and caput succedaneum?
A
- the blood is below the periosteum, therefore the lump does not cross the sagittal suture lines of the skull
- the blood can cause discolouration of the skin in the affected area
21
Q
what is frey’s syndrome and how does it present?
A
- damage to the auriculotemporal nerve during forceps delivery
- after trauma the parasympathetic fibres may be rewired to send signals to the sympathetic fibres of the sweat and blood vessels
- the consequence is that when the child eats, the signals that would normally stimulate the salivary glands to release saliva, instead trigger sweating
- -presents during weaning
- after eating certain foods, unilateral facial erythema occurs across the child’s cheek
22
Q
what are the common causative organisms of neonatal sepsis?
A
- group b streptococcus (found in mothers vagina)
- e.coli
- listeria
- klebsiella
- s.aureus
23
Q
red flags for neonatal sepsis?
A
- confirmed/suspected sepsis in mum
- signs of shock
- seizures
- term baby needing ventilation
- respiratory distress starting >4 hours after birth
24
Q
what antibiotics are used in neonatal sepsis?
A
benzylpenicillin and gentamicin
25
what is neonatal sepsis?
when a serious bacterial or viral infection in the blood affects babies within the first 28 days of life (early onset = within 72 hours)
26
what is HIE + causes?
result of prolonged hypoxia during birth
- maternal shock
- intrapartum haemorrhage
- prolapsed cord (compressing the cord during birth)
- nuchal cord (cord wrapped around the neck of the baby)
27
when should HIE be suspected?
- where there are events that could lead to hypoxia during the perinatal or intrapartum period
- **acidosis** (pH <7) on the **umbilical ABG**
- poor **APGAR scores**
- features of **mild, moderate or severe HIE**
- evidence of **multi organ failure**
28
what staging is used for HIE?
29
what is therapeutic hypothermia?
- **actively cooling the core temperature** of the baby according to strict protocol
- the baby is transferred to neonatal ICU and **actively cooled using cooling blankets and a cooling hat, passively cooling (taking off blankets), cooling jacket**
- the temperature is monitored with a target of between **33-34 degrees**, measured with a **rectal probe**
- continued for 72 hours, after which the baby is gradually warmed to a normal temp over 6-12 hours
30
what is the intention of therapeutic hypothermia?
- helps to **protect the brain from hypoxic injury**
- **reduce** the **inflammation and neurone loss after the acute hypoxic injury**
- it **reduces the risk of cerebral palsy**, developmental delay, learning disability, blindness and death
31
WHO classification of prematurity?
birth before 37 weeks
- <28 weeks = extremely premature
- 28-32 weeks = very premature
- 32-37 weeks = moderate to late premature
32
How do you try to delay birth if they seem small on USS before 24 weeks?
- **prophylactic vaginal progesterone** - putting progesterone suppository in the vagina to discourage labour
- **prophylactic cervical cerclage** - putting a suture in the cervix to hold it closed
33
cause of apnoeas?
(periods where breathing stops for > 20 seconds ++ bradycardia)
- due to immaturity of the ANS which controls respiration and heart rate
34
what are apnoeas a sign of?
Apnoea are often a sign of developing illness eg:
1. infection
2. anaemia
3. airway obstruction
4. CNS pathology eg. seizures or haemorrhage
5. gastro-oesophageal reflux
6. neonatal abstinence syndrome (withdrawal from drugs like opioids that the baby was exposed to in the womb)
35
management of apnoeas?
- apnoea monitors
- tactile stimulation to restart breathing
- IV caffeine to prevent apnoea and bradycardia in babies with recurrent eps
- eps settle as baby grows and develops
36
what is retinopathy of prematurity + who does it occur in?
abnormal detachment of the blood vessels in the retina, which can lead to scarring, retinal detachment and blindness
- premature and low birth weight babies
37
which babies are screened for ROP and what's examined in screening?
- <32 weeks or <1.5kg
- all retinal areas and vessels looked at and plus disease
38
treatment of ROP?
- systematically targeting areas of the retina to stop new blood vessels forming
- 1st line = **transpupillary laser photocoagulation** to halt and **reverse neovascularisation** (laser used to burn abnormal areas of the retina in which there is inadequate vascularisation, this burning process prevents abnormal blood vessel proliferation).
- other options = **cryotherapy** and **injections of intraviteral VEGF inhibitors**
- **surgery** may be needed in **retinal detachment** occurs
39
management for suspected/confirmed preterm labour mothers?
- antenatal steroids (dexmethasone), increases the production of surfactant
40
management for premature neonates with RDS?
- **intubation and ventilation** to fully assist breathing is RDS is severe
- **endotracheal surfactant** - artificial surfactant delivered to the lungs via an endotracheal tube
- **CPAP** - via nasal mask to help keep the lungs inflated whilst breathing
- **supplementary oxygen** to maintain o2 sats between **91-95%** in preterm neonates
41
long term complications of RDS?
- **chronic lung disease of prematurity/bronchopulmonary dysplasia**
- **retinopathy of prematurity** occurs more often and more severely in neonates with RDS
- **neurological, hearing and visual** impairment
42
a 34 week old baby presents with green bile vomit, not feeding, his abdomen is distended and in their stool there is blood. He's also formula fed and was in resp distress. What does this baby have?
necrotising enterocolitis
43
the investigation of choice for NecEnt is abdominal x-ray, what is seen in this?
- **dilated loops** of bowel
- **bowel wall oedema** (thickened bowel walls)
- **pneumatosis intestinalis** is the **gas in the bowel wall** and is a sign of NEC
- **pneumoperitoneum** is **free gas in the peritoneal cavity** and indicates **perforation**
- gas in the portal veins
44
how do you manage nec ent?
- **NBM**
- **IV fluids**
- **total** parenteral nutrition (TPN)
- **antibiotics**
- **NGT** can be inserted to **drain fluid and gas from the stomach and intestines**
- **surgery** may be needed to **remove the dead bowel tissue** and babies may be left with a **temporary stoma** if significant bowel is removed
45
what are some complications of necrotising enterocolitis?
Short term
- **perforation and peritonitis**
- **sepsis**
- **death**
Long term complications
- **strictures**
- **abscess** formation
- **recurrence**
- **long term stoma**
- **short bowel syndrome** after surgery
46
what substances can cause NAS?
opiates, methadone, benzodiazepines, alcohol, cocaine, amphetamines, SSRIs
47
how soon after birth do NAS symptoms occur?
- opiates, diazepam, SSRIs and alcohol: 3-72 hours
- methadone, other benzodiazepines: 24 hours - 21 days
48
medical management for moderate to severe symptoms of NAS?
- oral morphine sulphate for opiate withdrawal
- oral phenobarbital for non opiate withdrawal
- gradually wean off oral treatment
- SSRI withdrawal doesn't need medical treatment
49
how are babies with NAS monitored after birth?
- **kept in hospital with monitoring on a NAS chart** for at least **3 days (48 hours for SSRIs)** to **monitor for withdrawal symptoms**
- **urine sample** can be **collected** from the neonate to **test for substances**
- support them in a **quiet** and **dim environment** with **gentle handling and comforting**
50
how can the risk of SIDS be minimised?
- put the **baby on their back when not directly supervised**
- keep their head **uncovered**
- place their feet at the foot of the bed to prevent them sliding down and under the blanket
- keep the cot **clear of toys and blankets**
- maintain a **comfortable room temp (16-20 degrees)**
- **avoid smoking** and **avoid handling the baby after smoking as smoke stays on clothes**
- **avoid co-sleeping esp on a sofa or chair**
- if **co-sleeping, avoid alcohol, drugs, smoking, sleeping tablets or deep sleepers**
51
what support is out there for families affected by SIDS?
- **Lullaby trust**
- **Care of next infant team (CONI)** - supports parents with their next infant after a sudden infant death. Provides extra support and home visit, resuscitation training and access to equipment eg. movement monitors that alarm is the baby stops breathing for a prolonged period
52
a 37 week old baby, who was born prematurely, is presenting with low oxygen sats, poor feeding and crackles on auscultation. He has been on supplementary oxygen. what does he have and what are the major risk factors of this?
Bronchopulmonary dysplasia
- maternal smoking and IUGR
53
what can reduce the risk of BPD?
1. using CPAP rather that intubation and ventilation
2. using caffeine
3. not over oxygenating with supplemental oxygen
54
management of BPD?
1. **Overnight oximetry study** to **record O2 sats** during **sleep & guide management**
2. Discharged on **home oxygen which is slowly weaned**
3. **Monthly palivizumab** **injections** to **protect against RSV and bronchiolitis**
55
what happens in the first breath after birth?
- The first breaths expand the **alveoli**, **decreasing** pulmonary vascular resistance
- This causes a **fall in pressure in the RA and RV** so **left atrial pressure is greater** than right atrial pressure
- This squashes the atrial septum, **closing the foramen ovale** which then becomes the fossa ovalis
56
what do each of the 3 fetal shunts become after birth?
- foramen ovalis = fossa ovalis
- ductus arteriosus = ligamentum arteriosum
- ductus venosus = ligamentus venosum
57
what keeps the ductus arteriosus open?
Prostaglandins so increased blood oxygenation causes a drop in circulating prostaglandins, causing the ductus arteriosus to close → ligamentum arteriosum
58
a chest x-ray shows ground glass appearance, what does this baby have?
respiratory distress syndrome (premature babies with inadequate surfactant)
59
how do you test if a mother is immune against VZV, and if not what can they be treated with?
- if previously had chicken pox, now immune and safe
- to test, check IgG levels for VZV
- if not immune, treat with IV varicella immunoglobulins as prophylaxis against developing chicken pox
60
if chickenpox rash starts in pregnancy, what should be given?
treat with oral aciclovir if present within 24 hours and more than 20 weeks
61
when does cyanotic heart disease occur and what are some heart defects that can cause it?
- where the blood **bypasses the lungs and pulmonary circulation**, across a **R to L shunt**.
- this occurs when a **defect** allows blood to flow from the right to left side of the heart, without travelling to the lungs to be oxygenated
- VSD, ASD, PDA, transposition of the great arteries
62
what is Eisenmenger syndrome?
where the **pulmonary pressure increases beyond the systemic pressure** and so blood will flow from right to left across the defect, causing **cyanosis**
63
how does a baby with TOGA present?
- if not detected during pregnancy, it’ll present with **cyanosis** **at** or **within a few days of birth**
- a **PDA or VSD** can **initially compensate** by **allowing blood to mix** between the **systemic circulation and lungs**
- but within a **few weeks they will develop respiratory distress**, **tachycardia**, **poor feeding, poor weight gain and sweating**
64
clinical signs of TOGA?
- cyanosis
- tachypnoea
- loud single S2
- prominent RV impulse palpable on examination
- 'egg on side' appearance on CXR
65
management of TOGA?
- VSD will allow some mixing between 2 system and provide time for definitive management
- prostaglandin infusion
- balloon septosomy
- 'arterial switch' = definitive management
66
a baby presenting with a continuous crescendo-decrescendo 'machinery' murmur, heard loudest below the clavicle, is indicative of what heart defect?
patent ductus arteriosus
67
signs and symptoms of PDA?
Symptoms:
- SOB, difficulty feeding, poor weight gain, LRTIs
Signs:
- a **small PDA** may **not have any abnormal heart sounds**
- larger PDAs cause a **continuous crescendo-decrescendo ‘machinery’ murmur** which is heart loudest below the clavicle (there is a normal S1, but S2 may be difficult to hear over the murmur
- **large volume, bounding, collapsing pulse**
- the murmur may be detected during the new born exam
68
what is the medical and surgical management of PDA?
- indomethacin, ibuprofen and paracetamol attempted in **preterm** infants
- transcatheter PDA closure for **term**patients - catheter used inserted into peripheral vessel and fed to the heart
- open surgery
69
what are some complications of ASD?
- stroke (VTE)
- AF, atrial flutter
- pulmonary hypertension and RSH failure
- Eisenmenger syndrome
70
a baby presents with a mid systolic, crescendo decrescendo murmur heard loudest at the upper left sternal border. There's also a fixed split second heart sound. What does this baby have?
ASD
71
what is splitting of the second heart sound and 'fixed split'
- splitting is when you hear the closure of the pulmonary and aortic valves at slightly different times
Fixed:
- the split **doesn’t change with inspiration or expiration**
- in an ASD, blood flows from the **left to right atrium across the ASD**, **increasing the volume of blood that the RV has to empty** **before** the **pulmonary valve can close**
- the **pulmonary valve closes later than the aortic valve**, causing a **split second heart sound** that **doesn’t vary with respiration**
72
management of ASD?
- **active monitoring** (small defects may close on their own)
- **percutaneous transvenous catheter closure** (via the femoral vein)
- **open heart surgery**
73
infective endocarditis is associated with which congenital heart defects and what prophylaxis should be give?
- ventricular septal defect
- prophylaxis antibiotics during surgical procedures to reduce the risk of developing it
74
A baby presents with a pan systolic murmur heard most prominently at the left LSE in the 3rd and 4th intercostal spaces. On palpation, you can feel a systolic thrill. What does this baby have?
ventricular septal defect
75
what happens to the body when cyanotic?
- cyanosis related to a **low level of oxygen saturation in the blood**
- the bone marrow responds to low oxygen sats by **producing more RBCs and Hb** to **increase the oxygen carrying capacity** of the blood
- this leads to **polycythaemia** which is a **high concentration of Hb in the blood**
- polycythaemia gives the **patients a plethoric complexion**
- a **high concentration of RBCs and Hb** makes the **blood more viscous**, making the patient **more prone to to developing blood clots**
76
examination findings associated with pulmonary HTN?
- **RV heave**: RV contracts forcefully against increased pressure in the lungs
- **loud P2:** loud second heart sound due to forceful shutting of the pulmonary valve
- **raised JVP**
- **peripheral oedema**
77
- findings related to the right to left shunt and chronic hypoxia?
- cyanosis
- clubbing
- dyspnoea
- plethoric complexion
78
medical management of eisemenger syndrome and definitive management?
- **oxygen** to help symptoms
- treatment of **pulmonary hypertension** eg. **sildenafil**
- treatment of **arrhythmias**
- treatment of **polycythaemia** with **venesection**
- prevention and treatment of **thrombosis with anticoagulation**
- prevention of **infective endocarditis using prophylactic antibiotics**
- once the pulmonary pressure is high enough to cause the syndrome, it’s not possible to medically reverse the condition → the only definitive treatment is a heart lung transplant
79
turner's syndrome is associated with which heart defect?
coarctation of the aorta
80
examination findings of CoA, including the murmur?
- often the only indication of coarctation in a neonate may be **weak femoral pulses**
- performing a **four limb BP** will reveal high BP in the limbs supplied from the arteries that come **before the narrowing** and **low BP in the limbs distal to the narrowing**
- **systolic murmur** heard below the left clavicle (**left infraclavicular area**) and **below the left scapula**
Over time:
- **left ventricular heave** due to **left ventricular hypertrophy**
- **underdeveloped left arm** where there is **reduced flow to the left subclavian artery**
- **underdevelopment of legs**
81
management of CoA?
- in mild cases, patients can live symptoms free until adulthood without requiring surgical input
- severe cases - emergency surgery shortly after birth
- in critical cases where there’s risk of heart failure and death shortly after birth, **prostaglandin** **E** is used to **keep the ductus arteriosus open** while **waiting for sugery**
- this allows **some blood flow through the ductus arteriosus into the systemic circulation distal to the coarctation**
- **surgery** is then **performed** to **correct the coarctation and ligate the ductus arteriosus**
82
a baby presents with an ejection systolic murmur which is heard loudest at the aortic area. It has a crescendo-decrescendo character, and can be heard at the carotids. what does this baby have an what else can be found on examination?
Aortic valve stenosis
- ejection click just before the murmur
- palpable thrill during systole
- slow rising pulse and narrow pulse pressure
83
management of aortic stenosis?
- patients with more significant AS may need to **restrict physical activities**
- **percutaneous balloon aortic vavloplasty (inflating a balloon to stretch the valve via a catheter)**
- **surgical aortic valvotomy** (open surgery via a midline sternotomy incision to widen the aortic valve)
- **valve replacement (open surgery via midline sternotomy to replace the aortic valve)**
84
signs of pulmonary valve stenosis?
- **ejection systolic murmur heard loudest at the pulmonary area**
- **palpable thrill** in the **pulmonary area**
- **right ventricular heave** due to **RV hypertrophy**
- **raised JVP with giant A waves**
85
what can pulmonary stenosis be associated with?
- often **without any associations**
- **TOF**
- **william syndrome**
- **noonan syndrome**
- **congenital rubella syndrome**
86
what are tet spells?
- **intermittent symptomatic periods** where the **right to left shunt** becomes **temporarily worsened, precipitating a cyanotic episode** → this happens when the **pulmonary vascular resistance increases** or the **systemic resistance decreases**
- eg. if the child is **physically exerting** themselves, they’re generating a lot of **co2** → this is a **vasodilator** that **causes systemic vasodilation** and **therefore reduces the systemic vascular resistance** → blood flows at the path of least resistance, so **blood will be pumped from the RV to the aorta instead of the pulmonary vessels** (less resistance in the aorta than pulmonary vessels), **bypassing the lungs**
- these episodes can be **precipitated by walking, physical exertion or crying**
- the child will become **irritable, cyanotic and SOB**
- severe spells can lead to **reduced consciousness, seizures and potentially death**
87
immediate management of TET spell?
- **older children** may **squat** when a tet spell occurs
- **younger children** can be positioned with their **knees to their chest**
- squatting **increases the systemic vascular resistance**
- this **encourages blood to enter the pulmonary vessels**
88
what causes ebstein's anomaly and what is it?
- Lithium use during pregnancy eg. in bipolar disorder
- when the **tricuspid valve** is set lower in the right side of the heart, causing a **bigger RA and smaller RV**
- this leads to poor flow from the RA to the RV and therefore poor flow to the pulmonary vessesl
- it's often associated with a R->L shunt across a **ASD** and when this happens, you get cyanosis
(symptoms start after a few days as the ductus arteriosus minimises the cyanosis before it closes)
89
which condition can be associated with Wolff Parkinsons white syndrome?
ebstein's anomaly
90
a 4 day old baby presents with blue skin, poor feeding and short of breath, he also look oedematous. On examination, a gallop rhythm is heard. What does this baby have and how is it managed?
Ebstein's anomaly
- medical management is treated arrhythmias and heart failure, prophylactic abx to prevent infective endocarditis. Definitive management is by surgical correction
91
what is HOCM?
Hypertrophy (thickening) of the left ventricle muscle, particularly affecting the ventricular septum which reduces space inside the ventricle and blocks blood flow up to the aorta which is called left ventricular outflow tract obstruction
- autosomal dominant genetic condition (defect in genes for sarcomere proteins)
92
heart failure, MI, arrhythmia and sudden cardiac death is associated with what?
HOCM
93
examination findings of HOCM and how you can differentiate it from aortic stenosis?
- **Ejection systolic murmur** at the **lower left sternal border** (**louder with Valsalva manoeuvre and decreases with squatting** - these have no effects on AS)
- **Fourth heart sound**
- **Thrill at lower L sternal border**
94
what medications should be avoided in HCOM and why?
ace inhibitors and nitrates as they can worsen LVOT obstruction
95
apart from BBs and heart transplant, how else can HCOM be managed?
- **Surgical myectomy** (**removing part** of the **heart septum** to **relieve the obstruction**)
- **Implantable cardioverter defibrillator** (for those at risk of sudden cardiac death or ventricular arrhythmias)
96
a 1 year old baby who recently had a viral illness, presents with colicky abdominal pain, redcurrant jelly stool, pallor and they're drawing up their legs. On examination there is a sausage shaped mass in the RUQ. What does this baby have and what is the USS showing?
Intussusception
- donuts/target sign
97
how is intussusception managed?
- **NBM and NG tube** (drip and suck)
- **IV fluids**
- **therapeutic enema**: used to try and **reduce the intussusception**, **contrast, water or air** are pumped into the colon to force the folded bowel out of the bowel and into the normal position
- **surgical reduction** if **enema doesn’t work**
98
what is hirschsprung's disease & presentation?
a congenital condition where nerve cells of the myenteric plexus are absent in the distal bowel and rectum
- can present with **acute intestinal obstruction shortly after birth**
- **delay in passing meconium (>24 hours)**
- **chronic constipation** since birth
- **abdominal pain and distention**
- **vomiting**
- **poor weight gain** and **failure to thrive**
99
what is hirschsprung associated enterocolitis and management?
- **inflammation and obstruction of the intestine** occurring in around 20% of neonates with **hirschsprung’s disease**
- it typically presents **within 2-4 weeks of birth**, with **fever, abdominal distension, diarrhoea** (often with blood) and **features of sepsis**
- life threatening & can lead to **TMC** and **perforation** of the bowel
- **urgent antibiotics, fluid resuscitation and decompression of the obstructed bowel**
100
what presents with absolute constipation and bilious vomiting, with high pitched tinkling sounds on auscultation?
intestinal obstruction
- absent sounds later on in obstruction
101
a term baby has been jaundiced for 16 days, on examination his liver and spleen are enlarged. What does he have?
Biliary atresia = congenital condition where a section of the bile duct is narrowed or absent leading to cholestasis and consequent jaundice due to build up of conjugated bilirubin
102
how do you managed biliary atresia?
Kasai portoenterostomy
- this involves removing the damaged bile ducts outside of the liver and **attaching a section of the small intestine** to the **opening of the liver**, where the bile duct normally attaches
- this can clear the jaundice and prolong survival
- often patients need a full liver transplant
102
what autoantibodies are related to coeliac disease?
- tissue transglutaminase antibodies **(anti-TTG)**
- endomysial antibodies **(EMAs)**
- deaminated gliadin peptides antibodies **(anti-DGPs)**
103
how do coeliac patients present?
- often **asymptomatic** (important to have a low threshold when testing for coeliac)
- **failure to thrive** in young children
- **diarrhoea**
- **weight loss**
- **mouth ulcers**
- **anaemia** secondary to **iron, B12 or folate deficiency**
- **dermatitis herpetiformis** (an itchy blistering skin rash that typically appears on the abdomen)
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a 2 week old baby presents in the first 2 weeks of life, failing to thrive, thin and projectile vomiting. On palpation, you feel a small, olive shaped mass in the upper abdomen and you can see visible peristalsis. What does this baby have and what would investigations show?
pyloric stenosis
- blood gas: hypochloric metabolic alkalosis
- abdominal USS: thickened pylorus
105
how do you manage pyloric stenosis?
- **laparoscopic pyloromyotomy (Ramstedt’s operation**)
- the incision is made in the smooth muscle of the pylorus to **widen the canal**, allowing food to pass from the stomach to duodenum as normal
106
Crohn's vs UC?
107
management to induce remission in crohns and UC (mild/moderate and severe)?
Crohn's:
- first line = **steroids** (eg. oral prednisolone or IV hydrocortisone)
- **enteral nutrition** if concerns about steroids affecting growth in children (specially formulated liquid diet oral or NG, replacing the patients diet
UC:
Mild to moderate disease
- First line: aminosalicylate (eg. **mesalazine** oral or rectal)
- second line: corticosteroids eg. prednisolone
Severe disease
- first line: **IV corticosteroids** (eg. hydrocortisone)
- second line: IV ciclosporin
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management to maintain remission in crohns and UC?
Crohn's:
- **first line: azathioprine, mercaptopurine**
- alternatives: methotrexate, infliximab, adalimumab
UC:
- aminosalicylate eg. mesalazine oral or rectal
- azathioprine
- mercaptopurine
- surgical
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surgical management of crohns and UC?
Crohns:
- when the disease **only affects the distal ileum, it’s possible to resect this area to prevent further flares** (crohn’s typically involves the entire GI tract)
- surgery can be used to treat strictures and fistulas secondary to crohn’s
UC:
- UC **only affects the colon and rectum**, so removing the colon and rectum (**panproctocolectomy**) will remove the disease
- the patient is then left with either a **permanent ileostomy** or an **ileo-anal anastomosis (J-pouch)**
110
what is Sandifer syndrome?
- rare congenital condition causing **brief episodes of abnormal movements** **associated with GORD in infants**
- the infants are usually **neurologically normal**
- rare congenital condition causing **brief episodes of abnormal movements** **associated with GORD in infants**
- the infants are usually **neurologically normal**
111
a 6 year old presents with episodes of central abdominal pain which lasts for more than an hour. Examination is normal. She also complains of feeling sick and a headache. When turning the light on, she complains of it hurting her eyes. What does she have and how would you treat it acutely?
Abdominal migraine
- low stimulus environment
- paracetamol
- ibuprofen
- sumatriptan
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Preventative medication for abdominal migraine and what are one of these meds associated with?
- **Pizotifen** = serotonin antagonist → main preventative medication
- **Propanolol** = beta blocker
- Pizotifen: ++ with - **withdrawal symptoms such as depression, anxiety, poor sleep and tremor**
- therefore needs to be **withdrawn slowly when stopping it**
113
difference between viral induced wheeze and asthma?
VIW presents < 3, no history of atop and only occurs during viral infections. Whereas asthma is triggered by infections as well as other triggers eg. exercise, cold, dust, emotions
114
how is VIW managed?
same as acute asthma in children
- **Salbutamol inhalers** via a **spacer device**: starting with **10 puffs every 2 hours**
- **Nebulisers** with **salbutamol** / **ipratropium bromide**
- Oral **prednisone** (e.g. 1mg per kg of body weight once a day for **3 days)**
- supplementary oxygen
115
a 6 month old baby presents with chronic inspiratory stridor, worsened when upset or feeding. when looking down the throat, you seen an 'omega' shape. what does the patient have and describe the condition?
Laryngomalacia = laryngeal abnormality characterised by flaccidity of the supraglottic structures. The larynx is soft and floppy as a result and collapses during breathing. This causes chronic stridor on inhalation, where the larynx flops across the airway as the infant breathes in.
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how is Laryngomalacia managed?
Resolves itself as the larynx matures, grows and is able to better support itself, preventing it from flopping over.
Rarely a tracheostomy might be needed and surgery.
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what is primary ciliary dyskinesia and how does it present?
- autosomal condition affecting the cilia on the surface of cells
- Neonatal respiratory distress
- Chronic wet cough
- Recurrent lower resp tract infections
- Chronic sinusitis
- Recurrent otitis media
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what is kartagener's triad?
involves 3 features associated with PCD
- **Paranasal sinusisitis**
- **Bronchiectasis**
- **Situs inversus**
119
A previously healthy 4-year-old boy is brought to the emergency department by his parents. Over the past few hours, he has developed a sudden onset of **sore throat**, is **drooling**, and appears increasingly **distressed**. He is noted to be **febrile** and is sitting **upright, leaning forward with his hands on his knees**. His voice sounds **muffled**, and there is **audible stridor on inspiration**. He becomes more agitated when approached for examination and resists lying down. what is the most likely diagnosis and how would you manage this patient ?
Epiglottitis
- prep incase intubation is needed, as there's risk of sudden upper airway closure
- IV antibiotics eg. ceftriaxone
- IV steroids eg. dexamethasone
120
a 6 year old presents to the GP complaining of hip pain. His mum mentions that he recently had a viral respiratory infection. On examination he has a limp and his temperature is 36.9. He is otherwise well.
What does this patient have and how is it managed?
Transient synovitis
- rule out sepsis (would have a fever)
- simple analgesia
- follow up at 48 hours and 1 week to ensure symptoms are improving and then fully resolve
121
a 4 year old boy presents complaining of pain in his hips that's been worsening over the last couple of weeks. on examination he has a limp and has reduced range of movement. What does he have and how would you manage this patient? What is he at risk of?
Perthes disease (disruption of blood flow to the femoral head leading to avascular necrosis, affecting the epiphysis of the femur)
- as he's under 6, only observation needed
- if older and not healthy, may need surgery
- at risk of early hip OA as you get a soft and deformed femoral head
122
A 12-year old obese boy presents with a painful limp and vague knee pain that has worsened over the last few days. His mother reports he recently had a growth spurt and has become more overweight. He recalls a minor fall during football last week, but the pain seems worse than expected for the injury. On examination, there's reduced range of motion in the hip, especially on internal rotation. The pain appears to radiate from the hip to the knee, and he's struggling to bear weight. What does the patient have and management?
**Slipped upper femoral epiphysis**
- surgery needed to return the femoral head to the correct position and fix it in place to prevent it slipping further - internal rotation
123
prevention against RSV and how it works?
- **palivizumab**
- a **MCA** that targets the **RSV**
- a **monthly injection** is given as **prevention against bronchiolitis** caused by RSV
- it’s given to **high risk babies** eg. ex-premature babies and those with congenital heart disease- not a true vaccine as it doesn’t stimulating the infants immune system
- it provides **passive protection** by circulating the body until the virus is encountered → then it works as an antibody against the virus → activating the immune system to fight the virus
- the **levels of circulating antibodies decreased over time**, which is why a monthly injection is needed
124
ventilatory support in bronchiolitis?
- low flow oxygen
- **high flow humidified oxygen via a tight nasal cannula** (Airvo or optiflow)→ (air and oxygen) to oxygenate the lungs and prevent airways from collapsing
- **continuous positive airway pressure (CPAP)** → involves using a sealed nasal cannula to deliver **much higher and more controlled pressure/PEEP**
- **intubation and ventilation** → insert an **endotracheal tube** into the trachea to fully control breathing using mechanical ventilation
125
A 3-month-old infant is brought to the emergency department with a 3-day history of a runny nose, cough, and difficulty breathing. The symptoms have worsened over the past 1–2 days, with the infant becoming increasingly tachypnoeic and feeding poorly. The mother also reports a mild fever. On examination, the infant is tachypnoeic, with intercostal retractions and nasal flaring. Auscultation reveals widespread wheezing and crackles. The child’s oxygen saturation is 92% on room air. what does he have?
Bronchiolitis
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when would you admit a baby with bronchiolitis?
- presence of **grunting**
- if parents are not confident in their ability to manage at home or difficult accessing medical help from home
- **<3 months old or any pre existing conditions** eg. premature, downs syndrome, CF
- 50-75% or less of their normal intake of milk
- clinical dehydration
- RR>70
- O2 <92%
- cyanosis
- moderate to severe resp distress - eg. deep recessions or head bobbing
- apnoeas
127
A 1 year old boy presents to a&e with increased work of breathing, a temperature of 37.8 degrees. He has a barking cough and on inspiration you hear a high pitched sound. There's also tracheal tugging. What does he have, describe it and what are some common causes?
Croup = viral infection of the upper airways in children, causing oedema in the larynx
- parainfluenza virus, influenza A and B, adenovirus, RSV -> spread via droplets
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how is croup managed?
- supportive fluids and rest
- oral dexamethason single dose 150mcg/kg which can be repeated after 12 hours
129
stepwise approach in severe croup?
- oral dexamethasone
- oxygen
- nebulised budesonide
- nebulised adrenaline
- intubation and ventilation
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when should a child with croup be taken to the doctor/admitted?
- if stridor is continually heard/at rest
- intercostal recessions with every breath
- the child is restless or agitated
