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Phase 3A > Paediatrics 2 (Haem, Onc, MSK, Neon) > Flashcards

Paediatrics 2 (Haem, Onc, MSK, Neon) Flashcards

1
Q

What is the APGAR score and when is it performed in newborn resuscitation?

A

Done at 1, 5 and 10 minutes while resuscitation continues

a scoring system used to indicate the progress over the first few minutes after birth

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2
Q

What are the 5 categories in the APGAR scoring?

A

appearance (blue/pale centrally, blue extremeties or pink)

pulse (absent, <100, >100)

grimmace (no response, little response, good response to stimulation)

activity (floppy, flexed arms and legs, active)

respiration (absent, slow/irregular, strong/crying)

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3
Q

How can you stimulate breathing in the first minute of a newborns life?

A

stimulate the baby to prompt breathing, for example by drying vigorously with a towel

Place the baby’s head in a neutral position to keep airway open. A towel under the shoulders can help keep it neutral.

If gasping or unable to breath, check for airway obstruction (i.e. meconium) and consider aspiration under direct visualisation

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4
Q

If a neonate is not breathing/struggling or gaspin g for breath in their first few minutes of life, what should you do?

A

Give inflation breaths:

2 cycles of 5 inflation breaths (lasting 3 seconds each) can be given to stimulate breathing and heart rate

If there is no response and the heart rate is low, 30 seconds of ventilation breaths can be used

Essential to maintain a neutral head position and get a good seal around the mouth and nose

Look for a rise and fall in the chest

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5
Q

When should chest compressions be started in a newborn and at what rate?

A

start chest compressions if the heart rate remains below 60 bpm despite resuscitation and inflation breaths

chest compressions are performed at a 3:1 ratio with ventilation breaths

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6
Q

What is the purpose of delayed umbilical cord clamping? what are the current guidelines in how long the delay should be?

A

delayed clamping of the umbilical cord provides time for the fetal blood still contained in the placenta to enter the baby’s circulation

this is known as placental transfusion and has been shown to improve haemoglobin, iron stores and blood pressure as well as reducing intraventricular haemorrhage and necrotising enterocolitis

current guidelines state that uncompromised neonates should have a delay of at least 1 minute in the clamping of the umbilical cord following birth

in neonates requiring resuscitation this should be prioritised over delay

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7
Q

What is respiratory distress syndrome?

A

seen in premature neonates, born before the lungs start producing adequate surfactant (<32 weeks)

inadequate surfactant leads to high surface tension within the alveoli resulting in atelectasis –> inadequate gaseous exchange –> hypoxia, hypercapnia and respiratory distress

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8
Q

What is the management for respiratory distress syndrome

A

antenatal steroids (e.g. dexamethsone) given to mothers with suspected or confirmed preterm labour help to reduce the incidence and severity of RDS in the baby

premature neonates may need:
- intubation and ventilatiojn
- endotracheal surfactant
- CPAP
- supplementary oxygen

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9
Q

What are some short and long term complications of RDS?

A

Short term complications:

Pneumothorax
Infection
Apnoea
Intraventricular haemorrhage
Pulmonary haemorrhage
Necrotising enterocolitis

Long term complications:

Chronic lung disease of prematurity
Retinopathy of prematurity occurs more often and more severely in neonates with RDS
Neurological, hearing and visual impairment

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10
Q

What is hypoxic ischaemic encephalopathy?

A

malfunctioning of the brain due to a lack of oxygen and restriction of blood flow to the brain during birth

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11
Q

What foetal signs during the perinatal or intrapartum indicate increased risk of HIE? x4

A

acidosis
poor Apgar scores
features of mild, moderate or severe HIE
evidence of multi organ failure

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12
Q

give 4 examples of events which could cause HIE?

A

maternal shock
intrapartum haemorrhage
prolapsed cord
nuchal cord (cord wrapped around the baby’s neck)

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13
Q

What are signs of mild HIE according to sarnat staging?

A

poor feeding, generally irritable and hyper-alert

resolves within 24 hrs

normal prognosis

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14
Q

What are the signs and prognosis of moderate HIE according to sarnat staging?

A

poor feeding, lethargic, hypotonic and seizyres

can take weeks to resolve

up to 40% develop cerebral palsy

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15
Q

What are the signs and prognosis of severe HIE according to sarnat staging?

A

reduced consciousness, apnoeas, flaccid and reduced or absent reflexes

up to 50% mortality
up to 90% develop cerebral palsy

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16
Q

What is the management for HIE?

A

supportive care with neonatal resuscitation and ventilation, circulatory support, nutrition, acid-base balance and treatment of seizures

therapeutic hypothermia is an option in certain circumstances to help protect the brain from hypoxic injury

follow up from paediatrician to assess development and support any disability

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17
Q

How can therapeutic hypothermia help treat HIE?

A

involves actively cooling. the core temperature of the baby according to a strict protocol

the baby is transferred to neonatal ICU and actively cooled using cooling blankets and a cooling hat

the intention is to reduce the inflammation and neurone loss after the acute hypoxic injury reducing risk of CP, developmental delay, blindness and death

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18
Q

What is bronchopulmonary dysplasia (BPD)?

A

the most common chronic lung disease of the neonate, typically caused by prolonged ventilation and/or oxygen supplementation, which can disrupt normal lung development. It is characterised by inflammation, injury to the developing lung, and impaired growth of the alveoli and blood vessels.

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19
Q

What are the major risk factors for bronchopulmonary dysplasia? x4

A
  • prolonged mechanical ventilation
  • high concentrations of inspired oxygen
  • infection (e.g. chorioamnionitis, sepsis)
  • degree of prematurity
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20
Q

What are some key signs of BPD?

A

worsening hypoxemia, hypercapnia, and increasing oxygen requirements
inability to wean off oxygen therapy, mechanical ventilation or both

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21
Q

What is the management for BPD? x5

A

nutrition supplementation

fluid restriction

diuretics

oxygen supplementation as needed (wean from resp support asap)

respiratory syncytial virus prophylaxis

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22
Q

What are some measures taken in preterm infants to prevent BPD? x4

A

Use of antenatal corticosteroids as indicated

Prophylactic use of exogenous surfactant in selected high-risk infants (eg, weighing < 1000 g and requiring ventilator support)

Early therapeutic continuous positive airway pressure

Early use of surfactant for treatment of respiratory distress syndrome (RDS)

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23
Q

What is meconium aspiration syndrome?

A

When a neonate passes meconium during the pre or peri-natal period breathes it into their lungs causing respiratory distress and lung injury.

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24
Q

What causes meconium aspiration syndrome ?

A

physiologic stress at the time of labour and delivery e.g. hypoxia and/or acidosis caused by umbilical cord compression or placental insufficiency or infectious cause) may cause the foetus to pass meconium into the amniotic fluid before delivery

meconium passage may be normal before birth, particularly in term or post-term infants but is never normal before delivery of a preterm infant

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25
Q

What are some of the mechanisms by which aspiration causes meconium aspiration syndrome?

A
  • nonspecific cytokine release
  • airway obstruction
  • decreased surfactant production and surfactant inactivation
  • chemical pneumonitis
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26
Q

What are some signs of meconium aspiration syndrome?

A

tachypnoea
nasal flaring
retractions
cyanosis
desaturation
rales (rattling sounds)
rhonchi (sonorous wheezes)
visible meconium staining of the oropharynx

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27
Q

What is the gold standard investigation for meconium aspiration syndrome and what is indicated?

A

CXR which shows hyperinflation with variable areas of atelectasis and flattening of the diaphragm

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28
Q

What is the treatment for meconium aspiration syndrome? x6

A
  • endotracheal intubation and mechanical ventilation if needed
  • supplemental oxygen as needed to keep PaO2 high to relax pulmonary vasculature in cases with PPH
  • surfactant
  • IV antibiotics
  • Inhaled nitric oxide in severe cases of PPH
  • extracorporeal membrane oxygenation (ECMO) - if unresponsive to other therapies
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29
Q

What are the foetal infections described by the TORCH acronym?

A

Toxoplasmosis
Other - syphilis and HIV primarily (also gonorrhoea and varicella0
Rubella
Cytomegalovirus
Herpes and hepatitis

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30
Q

What are some clinical features common to TORCH infections? x9

A

low birthweight
preterm delivery
anaemia, thrombocytopaenia
hepatitis with jaundice and hepatosplenomegaly
seizures
microcephaly
mental handicap
encephalitis
failure to thrive

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31
Q

What are the 3 most common TORCH infections and their distinguishing features?

A

Toxoplasmosis –> neurological damage, cerebral calcification, hydrocephalus and chorioretinitis

Rubella –> congenital heart disease (PDA and pulmonary stenosis mainly), mental retardation, retinopathy, cataracts, glaucoma, purpura and microcephaly

Cytomegalovirus –> in 10-20%: hydrops (severe fetal oedema), chorioretinitis and microcephaly, in 80-90%: less specific mental handicap with visual and hearing problems later in life

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32
Q

What is the pathophysiology of physiological jaundice of the neonate?

A

There is a high concentration of RBCs in the foetus and neonate which are more fragile than normal RBCs.

Foetal RBCs break down more rapidly than normal RBCs which releases lots of bilirubin.

Normally this bilirubin is excreted via the placenta, however at birth the foetus no longer has access to a placenta to excrete bilirubin and the liver function is not fully developed so it is unable to process the excess bilirubin.

This leads to a normal rise in bilirubin following birth, causing a mild yellowing of skin and sclera between days 2-7, usually resolving completely by 10 days.

In most babies this doesn’t cause any issues.
However jaundice in the first 24hrs of life is pathological and requires urgent investigations and management.

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33
Q

What are the causes of neonatal jaundice linked to increased bilirubin production? x8

A

haemolytic disease of the newborn
ABO incompatibility
haemorrhage
intraventricular haemorrhage
cephalo-haematoma
polycythaemia
sepsis and disseminated intravascular coagulation
G6PD deficiency

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34
Q

What are the causes of neonatal jaundice linked to decreased bilirubin clearance? x6

A

prematurity
breast milk jaundice
neonatal cholestasis
extrahepatic biliary atresia
endocrine disorders (hypothyroid and hypopituitary)
Gilbert syndrome

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35
Q

What is a common cause of jaundice within the first 24hrs of life?

A

neonatal sepsis

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36
Q

What is kernicterus?

A

brain damage due to high bilirubin levels

presents with less responsive, floppy, drowsy baby with poor feeding.
the CNS damage is permanent causing cerebral palsy, learning disability and deafness.

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37
Q

Why are breastfed babies more likely to have neonatal jaundice?

A

certain components of breastmilk inhibit the ability of the liver to process bilirubin

breastfed babies are more likely to become dehydrated if not feeding adequately and this may lead to slow passage of stools, increasing absorption of bilirubin in the intestines

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38
Q

What is haemolytic disease of the newborn?

A

a disease characterised by haemolysis and jaundice in the neonate

it results from incompatibility between the rhesus antigens on the surface of the RBCs of the mother and foetus leading to the immune system of the foetus attacking their own RBCS.

this leads to haemolysis which causes anaemia and high bilirubin levels

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39
Q

Why do rhesus D negative mothers have a low risk of haemolytic disease of the newborn in their first pregnancy but high risk in subsequent pregnancies in the child is rhesus D positive?

A

In the first pregnancy with a rhesus positive child the mother’s immune system will be exposed to rhesus D antigens and produce antibodies so her immune system becomes sensitised but does not cause problems.

In subsequent pregnancies where the child is rhesus D positive the mother’s rhesus D antibodies can cross the placenta into the foetus and attach themselves to the RBCs of the foetus causing the immune system of the foetus to attack itself –> haemolytic disease of the newborn

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40
Q

What is the definition of prolonged jaundice in full term and preterm babies

A

lasting more than 14 days in full term babies
lasting more than 21 days in preterm babies

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41
Q

What investigations are needed for neonatal jaundice? x7 blood tests

A

FBC and blood film (for polycythaemia or anaemia)
Conjugated bilirubin (elevated levels indicate a hepatobiliary cause)
Blood type testing (of mother and baby for ABO or rhesus incompatibility)
Direct Coombs Test (direct antiglobulin test) for haemolysis
Thyroid function (particularly for hypothyroid)
Blood and urine cultures (if infection is suspected.)
Glucose-6-phosphate-dehydrogenase (G6PD) levels (for G6PD deficiency)

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42
Q

What is the management for neonatal jaundice?

A

monitoring of total bilirubin levels on treatment threshold charts
phototherapy is usually adequate but extremely
high levels may require an exchange transfusion

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43
Q

How does phototherapy work to correct neonatal jaundice?

A

a light box shines blue light on the baby’s skin which converts unconjugated bilirubin into isomers which can be excreted in the bile and urine without requiring conjugation in the liver.

a rebound bilirubin should be measured 12-18 hours after stopping

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44
Q

What is necrotising enterocolitis (NEC)

A

a life threatening emergency affecting premature neonates where part of the bowel becomes necrotic and can be at risk of perforation, peritonitis and shock

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45
Q

What are some of the risk factors for developing NEC?x5

A

very low birth weight or very premature
formula feeds
respiratory distress and assisted ventilation
sepsis
patent ductus arteriosus and other congenital heart diseases

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46
Q

What are the key symptoms/signs of NEC? x6

A

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

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47
Q

What are the key investigations for NEC?

A

Bloods (FBC, CRP, CBG, culture)
Abdo XR

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48
Q

What may be seen on abdo XR for NEC? x5

A

dilated bowel loops
bowel wall oedema
pneumatosis intestinalis (gas in bowel wall)
pneumoperitoneum (free gas in peritoneal cavity indicating perforation)
gas in the portal veins

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49
Q

What is the management for NEC?

A

nil by mouth with IV fluids, TPN and antibiotics to stabilise them
NG tube may be inserted to drain fluid and gas from the stomach and intestines

SURGICAL EMERGENCY as dead bowel tissue may need to be removed

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50
Q

What are some potential complications of NEC? x8

A

Perforation and peritonitis
Sepsis
Death
Strictures
Abscess formation
Recurrence
Long term stoma
Short bowel syndrome after surgery

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51
Q

What is gastroschisis?

A

protrusion of the abdominal viscera through a full-thickness abdo wall defect, usually to the right of the umbilical cord insertion

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52
Q

What is the difference between gastroschisis and omphalocele

A

in gastroschisis the abdominal viscera protrudes from an opening to the right of the umbilical cord insertion and there is no membranous covering of the intestine whereas with omphaloceles it protrudes from a midline defect at the base of the umbilicus and has no covering

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53
Q

What protein seen on prenatal blood tests may be elevated and indicate gastroschisis?

A

alpha-fetoprotein

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54
Q

What is oesophageal atresia? What frequently occurs along with this?

A

a congenital medical condition which affects the GI tract and causes the oesophagus to end in a pouch rather than connecting normally to the stomach

it frequently occurs along with tracheoesophageal fistula (TEF)

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55
Q

What are the 4 types of oesophageal atresia?

A

A - the upper and lower segments of the oesophagus end in pouches, like dead-end streets which don’t connect, TEF is not present

B - the lower segment ends in a blind pouch, TEF is present on the upper segment - very rare type

C - upper segment ends in a blind pouch, TEF is present on the lower segment (MC)

D - TEF is present on both upper and lower segments (rarest type)

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56
Q

What other birth defects are associated with oesophageal atresia? x6

A

Trisomy 13, 18 or 21

Other GI tract problems e.g. intestinal atresia or imperforate anus

Congenital heart defects e.g. ventricular septal defect, TOF, PDA

Kidney/UT problems e.g. horseshoe or polycystic kidney, absent kidney or hypospadias

Muscular or skeletal problems

Tethered spinal cord

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57
Q

What are some signs and symptoms of oesophageal atresia? x4

A

frothy white bubbles in mouth
coughing or choking when feeding
blue colour of skin especially when feeding
difficulty breathing

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58
Q

What is the treatment for oesophageal atresia?

A

Surgery to reconnect the two ends of the oesophagus
Foker process (stimulation of the upper and lower ends of the oesophagus to make them grow inside the baby allowing them to be joined together after days or weeks)

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59
Q

What is the difference between atresia and stenosis?

A

atresia = complete obstruction
stenosis = partial obstruction resulting in a narrowing or stricture

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60
Q

Where is the most common site for intestinal atresia to occur?

A

Jejunum or ileum - jejunoileal atresia

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61
Q

What is seen on XR to indicate proximal small bowel atresia?

A

“double bubble” where there is dilated bowel and fluid in the baby’s stomach and part of the duodenum but no fluid beyond that point

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62
Q

What is polyhydramnios and how is it linked to intestinal atresia?

A

Polyhydramnios = increase in amniotic fluid which usually develops in the third trimester

Normally the baby is continuously swallowing and excreting (as urine) the amniotic fluid but with intestinal atresia there is a blockage so the baby is unable to process the fluid.

As a result the baby’s stomach and intestine dilate before the site of obstruction and the amniotic fluid accumulates within the uterine cavity.

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63
Q

What are the definitions of macrosomia and microsomia?

A

Macrosomia = large for gestational age where the weight of the newborn is more than 4.5kg at birth

Microsomia = small for gestational age where the foetus measures below the 10th growth centile for their gestational age

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64
Q

What are some causes of macrosomia? x6

A

constitutional
maternal diabetes
previous macrosomia
maternal obesity or rapid weight gain
overdue
male baby

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65
Q

What are the risks to the baby in macrosomia x5

A

birth injury
shoulder dystocia
neonatal hypoglycaemia
obesity in childhood and later life
T2DM in adulthood

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66
Q

What are some signs of hyperthyroidism in neonates? x6

A

irritability
weight loss
tachycardia
heart failure
diarrhoea
exophthalmos in first 2 weeks of life

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67
Q

What is defined as hypoglycaemia in neonates?

A

has been controversial but usually plasma glucose concentration of 2.5mmol/l or less

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68
Q

What are some signs/symptoms of hypoglycaemia in neonates? x10

A

hypotonia
lethargy
poor feeding
hypothermia
apnoea
irritability
pallor
tachypnoea
tachycardia or bradycardia
seizures
abnormal feeding behaviour

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69
Q

What are some risk factors for neonatal hypoglycaemia? x5

A

intrauterine growth restriction in term infants
infants less than 37 weeks gestation
maternal diabetes
macrosomic babies
mother taking B-blockers

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70
Q

What are the management steps for neonatal hypoglycaemia in term babies?

A

asymptomatic may be treated by increasing breastfeeding frequency, supplementing with a breast milk substitute, or IV glucose therapy - blood-glucose should always be rechecked in 1 hr post treatment to check efficacy.

symptomatic neonates should be treated immediately with IV glucose infusion

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71
Q

Which organisms commonly cause neonatal sepsis? x5

A

Group B strep
E. coli
Listeria
Klebsiella
Staph. aureus

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72
Q

What are some risk factors for neonatal sepsis?

A

vaginal GBS colonisation (MC)
GBS in a previous baby
maternal sepsis, chorioamnionitis or fever >38
prematurity
early rupture of membrane
prolonged rupture of membranes

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73
Q

What are some of the clinical features of neonatal sepsis? x10

A

fever
reduced tone and activity
poor feeding
respiratory distress or apnoea
vomiting
tachycardia or bradycardia
hypoxia
jaundice within 24hrs
seizures
hypoglycaemia

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74
Q

What are some of the red flags for neonatal sepsis/ x6

A

confirmed or suspected sepsis in the mother
signs of shock
seizures
term baby needing mechanical ventilation
RDS starting more than 4 hrs post birth
presumed sepsis in another baby in a multiple pregnancy

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75
Q

What are the NICE guidelines for starting antibiotics with suspected neonatal sepsis? which abx?

A

2+ risk factors or clinical features
1x red flag

Take blood cultures before giving
Give within 1hr of making decision to start them
check FBC and CRP
perform a lumbar puncture if infection is strongly suspected or there are features of meningitis

the recommended abx are benzylpenicillin and gentamycin 1st line

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76
Q

What are the signs of listeria infection in a neonate? what are some potential consequences of maternal listeria infection ?

A

meconium staining of the amniotic fluid
widespread rash
septicaemia
pneumonia
meningitis

maternal infection may cause spontaneous abortion, preterm delivery or foetal/neonatal sepsis

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77
Q

What antibiotics are indicated for listeria infection?

A

IV amoxicillin and gentamycin

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78
Q

What are cleft lip and cleft palate?

A

cleft lip = a congenital condition where there is a split or open section of the upper lip (can occur at any point along the top lip and extend as high as the nose)

cleft palate = defect in the hard or soft palate at the roof of the mouth which leaves an opening between the mouth and nasal cavity

can occur together or separately

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79
Q

What are the potential complications of cleft lip/palate?

A

significant problems with feeding, swallowing and speech
psychosocial implications including affecting bonding between mother and child
can be more prone to hearing problems, ear infections and glue ear

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80
Q

What is the definitive treatment for cleft lip/palate and at what age is it usually administered

A

cleft lip surgery - 3 months
cleft palate surgery - 6-12 months

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81
Q

What is transient synovitis?

A

temporary irritation and inflammation in the synovial membrane of the hip joint

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82
Q

What is the most common presentation of transient synovitis>

A

well child aged 3-10 with acute/gradual onset limping, refusal to weight bear, groin/hip pain and mid/low grade temperature
recent viral upper respiratory track illness

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83
Q

What is the management and prognosis for transient synovitis?

A

Symptomatic treatment and exclusion of more concerning pathology (e.g. septic arthritis)

analgesia, anti-inflammatories

symptoms should significantly improve after 24-48hrs and fully resolve within 1-2 weeks without any lasting problems

can recur in around 20% of patients

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84
Q

What are the 4 parameter’s of Kocher’s criteria for septic arthritis?

A

non-weight bearing - 1 point
fever >38.5 - 1 point
WCC >12*10^9/L - 1 point
ESR >40mm/hr - 1 point

The probabilities are calculated :
0 points = very low risk
1 point = 3% probability of septic arthritis
2 points = 40% probability of septic arthritis
3 points = 93% probability of septic arthritis
4 points = 99% probability of septic arthritis

85
Q

What is septic arthritis?

A

infection inside a joint which can occur at any age but most commonly in children under 4 yrs

MEDICAL EMERGENCY as the infection can quickly begin to destroy the joint and cause serious systemic illness

86
Q

What is the presentation of septic arthritis?

A

hot, red, swollen and painful joint
refusing to weight bear
stiffness and reduced range of motion
systemic symptoms such as fever, lethargy and sepsis

87
Q

Which bacteria most commonly cause septici arthritis?

A

Staph, aureus (MC)
Neisseria gonorrhoea (sexually active teenagers)
Group A strep
haemophilus influenzae
E.coli

88
Q

What are some differentials for septic arthritis?

A

transient synovitis
perthes disease
slipped upper femoral epiphysis
juvenile idiopathic arthritis

89
Q

What is the management for septic arthritis?

A

empirical IV antibiotics until sensitivities known
once septic arthritis confirmed (joint aspiration) abx are usually continued for 3-6 weeks

flucloxacillin usually firstline

sometimes surgical drainage and washout of the joint is needed in severe cases

90
Q

What is a slipped upper femoral epiphysis?

A

where the head of the femur is displaced along the growth plate

also known as slipped capital femoral epiphysis

91
Q

What are some symptoms of SUFE?

A

hip, groin, thigh or knee pain
restricted range of hip movement
painful limp
restricted movement in the hip

92
Q

What investigations are used to diagnose SUFE? what is the management

A

Xray
Blood tests
Technetium bone scan
CT scan
MRI scan

surgery to return the femoral head to the correct position and fix it in place to prevent further slipping

93
Q

What is the typical presentation of slipped upper femoral epiphysis?

A

adolescent, obese male undergoing a growth spurt
minor trauma triggering onset of unilateral hip/knee pain
hip kept in external rotation with limited internal rotation

94
Q

What is Perthes disease? what age range is most common in?

A

Disruption of blood flow to the femoral head which causes avascular necrosis of the bone
This affects the epiphysis of the femur

revascularisation or neovascularisation occurs over time as the femoral head heals and there is remodelling of the bone.

occurs in children aged 4-12 yrs and is more common in boys

95
Q

What is the main complication of perthes disease and what is the resulting prognosis?

A

soft and deformed femoral head leading to early hip osteoarthritis

leads to an artificial total hip replacement in around 5% of patients

96
Q

What are the symptoms of Perthes disease?

A

pain in the hip or groin
limp
restricted hip movements
referred pain to the knee

97
Q

What is the management for Perthes disease?

A

aim is to maintain a healthy position and alignment in the joint and reduce the risk of damage or deformity to the femoral head
- bed rest
- traction
- crutches
- analgesia
- physiotherapy
- regular xrays to monitor healing
- surgery in severe cases to improve alignment

98
Q

What is osteomyelitis?

A

infection of the bone and bone marrow typically occurring in the metaphysis of the long bones

can be chronic - deep seated, slow growing infection with slow symptom development

or acute - acutely unwell child with fast onset of symptoms

99
Q

what are some risk factors for osteomyelitis? x9

A

open bone fracture
orthopaedic surgery
immunocompromised
sickle cell anaemia
HIV
Tuberculosis
male
<10yrs old

100
Q

What are the signs/symptoms of osteomyelitis?

A

Refusing to use the limb or weight bear
Pain
Swelling
Tenderness
Could be afebrile or have a low grade fever

101
Q

What is the treatment for osteomyelitis?

A

extensive and prolonged antibiotic therapy
surgery for drainage and debridement of the infected bone

flucloxacillin is usually first line

102
Q

What is rickets?

A

defective bone mineralisation causing soft and deformed bones

(same process in adults –> osteomalacia)

103
Q

what causes rickets?

A

deficiency in vitamin D or calcium
rare form is caused by genetic defects resulting in low blood phosphate, this is called hereditary hypophosphataemic rickets

(Vitamin D is either produced by the body in response to sunlight or obtained through foods such as eggs, oily fish or fortified cereals or nutritional supplements. Calcium is found in dairy products and some green vegetables.)

104
Q

What are some risk factors for rickets?

A

darker skin
low sunlight exposure
colder climates
laabsorption disorders e.g. IBD, coeliac
CKD (kidneys metabolise vit D)
poor nutrition

105
Q

What are some potential symptoms of rickets? x8

A

lethargy
bone pain
swollen wrists
bone deformity
poor growth
dental problems
muscle weakness
pathological or abnormal fractures

106
Q

What are the bone deformities which can occur in rickets ? x5

A

bowing of the legs
knock knees
rachitic rosary (ends of the ribs expand at the costochondral junctions causing lumps along the chest)
craniotabes (soft skull with delayed closure of the sutures and frontal bossing)
delayed teeth

107
Q

What are the preventative measures/treatments used for rickets??

A

formula feed fortified with vitamin D
vit D supplements for breastfeeding women and all children

can be treated with ergocalciferol

108
Q

What is scoliosis?

A

a lateral curvature of the frontal plane of the spine

in structural scoliosis there is rotation of the vertebral bodies which causes a prominence in the back from rib asymmetry

109
Q

What are some causes of scoliosis?

A

Idiopathic (MC)
Congenital e.g. hemivertebra, spina bifida, syndromes
Secondary - related to other disorders such as neuromuscular imbalance, bone disorders, leg length discrepancy

110
Q

What is the management for scoliosis?

A

in most cases of scoliosis the changes are mild, pain-free and primarily a cosmetic problem not requiring treatment
if more severe, the severity and progression of the curvature is determined by x-ray and non-medical treatments such as bracing or surgery will be considered

111
Q

What is developmental dysplasia of the hip ?

A

structural abnormality in the hips caused by abnormal development of the fetal bones during pregnancy - this leads to instability in the hips and increased risk of subluxation or dislocation

the changes can persist into adulthood resulting in weakness, recurrent subluxation or dislocation, an abnormal gait and early degenerative changes

112
Q

What are some risk factors for DDH?

A

1st degree FH
Breech presentation from 36+ weeks
Breech presentation at birth if 28 wks +
multiple pregnancy

113
Q

What are some findings in the NIPE which may suggest DDH? x5

A

different leg lengths
restricted hip abduction on one side
significant bilateral restriction in abduction
difference in the knee level when the hips are flexed
clunking of the hips on special tests (ortolani and barlow)

114
Q

How is the ortolani test performed?

A

baby on their back with the hips and knees flexed

palms are placed on the baby’s knees with thumbs on the inner thigh and four fingers on the outer thigh

gentle pressure is used to abduct the hips and apply pressure behind the legs with the fingers to see if the hips will dislocate anteriorly

115
Q

How is barlow test performed?

A

baby on their back with hips adducted and flexed at 90 degrees and knees bent at 90 degrees

gentle downward pressure is placed on knees through femur to see fi the femoral head will dislocate posteriorly

116
Q

What is the investigation of choice to confirm diagnosis of DDH?

A

hip USS

117
Q

What is the management for DDH?

A

If baby presents at <6mo a Pavlik harness is fitted and kept on permanently - this holds the femoral head in the correct position (hips flexed and abducted) to allow the acetabulum to develop normally, the harness is usually removed after 6-8 weeks

surgery is required when the harness fails or diagnosis is made after 6 mo

118
Q

What is Osgood-Schlatter disease?

A

inflammation at the tibial tuberosity where the patella ligament inserts, often affecting adolescent males who are physically active

usually unilateral, but can be bilateral

119
Q

Briefly describe the pathophysiology of Osgood-Schlatter disease

A

the patella tendon inserts into the tibial tuberosity at the epiphyseal plate

stress from running, jumping and other movements at the same time as growth in the epiphyseal plate result in inflammation on the tibial epiphyseal plate

there are multiple small avulsion fractures, where the patella ligament pulls away tiny pieces of the bone leading to growth of the tibial tuberosity and a visible lump below the knee

initially the bump is tender but as the bone heals and the inflammation settles it becomes hard and non-tender

120
Q

What is the presentation of Osgood-schlatter disease?

A

gradual onset symptoms
visible or palpable hard and tender lump at the tibial tuberosity
pain in the anterior aspect of the knee
pain exacerbated by physical activity, kneeling and on extension of the knee

121
Q

What is the management for Osgood-Schlatter disease?

A

aim is to reduce pain and inflammation
- reduction in physical activity
- ice
- NSAIDs

stretching and physio once symptoms settle

122
Q

What is Osteogenesis imperfecta?

A

a genetic condition affecting collagen formation resulting in brittle bones which are prone to fractures

also known as brittle bone syndrome

123
Q

What are the key features seen in OI?

A

HYPERMOBILITY
BLUE/GREY SCLERA
triangular face
short stature
deafness from early adulthood
dental problems, particularly with formation of teeth
bone deformities
joint and bone pain

124
Q

What is the management for OI?

A

bisphosphonates to increase bone density
vitamin D supplementation
surgical management of fractures

support from MDT

125
Q

What is juvenile idiopathic arthritis?

A

persistent joint swelling of >6 weeks duration presenting before 16 yrs of age in the absence of infection or any other defined cause

at least 7 subtypes

Most common chronic inflammatory joint disease in children and adolescents in the UK

126
Q

How is JIA classified?

A

based on the number of joints affected in the first 6 months:
polyarthritis (more than 4 joints)
oligoarthritis (up to and including 4 joints)
systemic (with fever and rash)

127
Q

What are the main features of JIA?

A

Gelling (stiffness after epriods of rest e.g. long car ride)
morning joint stiffness and pain
intermittent limp
deterioration in mood or behaviour
joint swelling
can be indolent in young children especially

128
Q

What are some potential complications of JIA?

A

Chronic anterior uveitis
Flexion contractures of the joints
Growth failure

129
Q

What is discoid meniscus and what are the 3 types?

A

a congenital defect of the meniscus causing it to be thicker than usual and often oval or disc-shaped - results in increased risk of knee injury

  • incomplete (slightly thicker and wider)
  • complete (meniscus completely covers the tibia)
  • hypermobile wrisberg (absence of the extracapsular and intracapsular ligaments)
130
Q

What are the symptoms of discoid meniscus?

A

Pain
Stiffness or swelling
Catching, popping, or locking of the knee
Feeling that the knee is “giving way”
Inability to fully extend (straighten) the knee

131
Q

What is the treatment for discoid meniscus?

A

if non-symptomatic no treatment is needed
if it is causing pain, popping or locking of the knee or other symptoms knee arthroscopy is indicated

132
Q

What is torticollis?

A

also known a wry neck

painful neck which can be associated with restriction of head turning and tilting of the head

133
Q

What is the most common cause of torticollis in infants?

A

sternomastoid tumour which usually occur within the first few weeks of life and present with a mobile, non-tender nodule, which can be felt within the body of the sternocleidomastoid muscle

134
Q

What is the prognosis/management of torticollis?

A

usually resolves in 2-6 months
passive stretching is advised
oral analgesics

135
Q
A
136
Q
A
137
Q

What is the difference between adult and foetal haemoglobin?

A

Adult haemoglobin= 2a, 2b chains
Foetal haemoglobin = 2a, 2y chains

138
Q

What is sickle cell anaemia?

A

An autosomal-recessive single gene defect in the beta chain of haemoglobin, which results in production of sickle cell haemoglobin (HbS).

139
Q

What is the pathophysiology of sickle cell anaemia?

A

On the 6th codon of B-globin glutamic->valine causing irreversible RBC sickling.
Sickled RBCs are more fragile and have a decreased SA so are less efficient and die quicker with increased risk of sequestration.

140
Q

What are the symptoms of SCA?

A

general anaemia + jaundice
+ complications:
- acute sickle crisis = vessel occluded, extremely painful and medical emergency (can be in pulmonary vessel = acute chest crisis –> resp. distress)
- splenic sequestration - can cause autosplenectomy (basically non-functional spleen)
- osteomyelitis (caused by salmonella)

141
Q

What are the investigations for SCA?

A
  • Newborns are screened in heel prick test
  • FBC + blood film = normocytic normochromic w/ increased reticulocytes, sickled RBCs+ Howell Joly bodies (nuclear remnants found in the RBCs of patients with reduced or absent splenic function)
  • Hb electrophoresis
142
Q

What is the management of SCA?

A

prevent complications whenever possible (stay warm + hydrated, vaccinations, monitoring, regular blood transfusion, hydroxycarbamide - ↑HbF
- only curative therapy = bone marrow transplant (signifciant risks)
- iron chelation (treats iron overload)

Acute attacks: IV fluid + analgesia + O2

143
Q

What is splenic sequestration in SCA?

A

Occurs when blood cells get stuck in the spleen and block the passage of platelets out of the spleen. This can result in 90% of circulating platelets becoming sequestered in the spleen and is a medical emergency.

144
Q

What is thalassaemia?

A

Overarching term for several conditions which result in lack of haemoglobin production.

145
Q

What is alpha thalassaemia?

A

Abnormality of a-globin gene
- less common than b thalassaemia
- 4 genes on chromosome 16
- deletions, associated with HbH (abnormal isoform where b-chain tetramers form)

146
Q

What is beta thalassaemia?

A

Abnormality of b-globin gene
- 2 genes on chromosome 11
- mutations, normal Hb isoforms
- just deletion of b-chains

147
Q

What are the clinical classifications of beta thalassaemia?

A
  • thalassaemia major - transfusion dependent
  • thalassaemia intermedia - less severe and can survive without regular blood transfusions
  • thalassaemia carrier - asymptomatic
148
Q

What are the risk factors for thalassaemia?

A
  • FH
  • More common in people of African, Mediterranean and Southeast Asian descent
149
Q

What are the key clinical manifestations of b-thalassaemia?

A

variable depending on number of mutations:
- general anaemia
- chipmunk facies (enlarged forehead and cheekbones)
- hepatosplenmegaly
- failure to thrive

150
Q

What are the investigations for thalassaemia?

A

FBC, peripheral smear, reticulocyte test
genetic testing to confirm diagnosis
XR skull, long bones, abdo US

151
Q

What is the treatment for thalassaemia?

A

Bone marrow stem cell transplant only curative option
Regular blood transfusions needed for survival (complications: iron overload, iron chelation)

152
Q

What are constitutional (B) symptoms?

A
  • night sweats
  • fever
  • unintentional weight loss (loss of >10% body weight over 6 months)
153
Q

What is the MCV test?

A

Mean cell (corpuscular) volume = cell size
macrocytic = cells larger than normal
microcytic = cells smaller than normal
normocytic = cell size within normal range (80-98fl)

154
Q

What is the MCH test?

A

Mean cell haemoglobin = amount of haemoglobin in each cell
Hypochromic = less haemoglobin in each cell than normal
Normochromic = normal amount of haemoglobin (27-33pg)

155
Q

What are the causes of microcytic anaemia?

A

T - Thalassaemia
A - Anaemia of chronic disease
I - Iron deficiency anaemia
L - Lead poisoning
S - Sideroblastic anaemia

156
Q

What is anaemia?

A

A condition in which the number of red blood cells or the haemoglobin concentration within them is lower than normal.
Defined as Hb <120g/l in females and <140 g/l in males

157
Q

What are the symptoms of anaemia?

A
  • Tiredness
  • SOB
  • headaches
  • dizziness
  • palpitations
  • worsening of other conditions like angina, heart failure or peripheral vascular disease
158
Q

What are the signs of anaemia?

A
  • pale skin
  • conjunctival pallor
  • tachycardia
  • raised respiratory rate
159
Q

What are the investigations for anaemia?

A

Haemoglobin
Mean cell volume (MCV)
B12
Folate
Ferritin
Blood film
Oesophago-gastruduodenoscopy (OGD) and colonoscopy to investigate for a gastrointestinal cause of unexplained iron deficiency anaemia (for suspected GI cancer)
Bone marrow biopsy if cause is unclear

160
Q

What is leukaemia?

A

cancer of a particular line of the stem cells in the bone marrow resulting in unregulated production of certain types of blood cells.

161
Q

What are the 4 main types of leukaemia?

A

Acute myeloid leukaemia
Chronic myeloid leukaemia
Acute lymphoblastic leukaemia
Chronic lymphocytic leukaemia

162
Q

What are the progressive ages of leukaemias?

A

Under 5 and over 45 – acute lymphoblastic leukaemia (ALL)
Over 55 – chronic lymphocytic leukaemia (CeLLmates)
Over 65 – chronic myeloid leukaemia (CoMmon)
Over 75 – acute myeloid leukaemia (AMbitions)

163
Q

What is ALL?

A

Malignant change of one of the lymphocyte precursor cells - causes acute proliferation of a single type of lymphocyte (usually B lymphocytes) which leads to replacement of other cell types being created in the bone marrow ⇒ pancytopenia.

164
Q

What are the risk factors for ALL?

A

Down’s syndrome (30x risk)
Radiation exposure
Smoking
Viral infections
History of malignancy
Poor maternal diet

165
Q

What are the key presentations for ALL?

A

General leukaemia symptoms (fatigue, failure to thrive, fever, pallor, petechiae, abnormal bleeding, lymphadenopathy, hepatosplenomegaly)

166
Q

What are the investigations for ALL?

A

FBC = pancytopenia
Blood film = ↑lymphoblasts
BM biopsy = ≥20% lymphoblasts
Immunofluorescence = TdT +ve lymphoblasts (terminal deoxynucleotidyl transferase)

167
Q

What is the treatment for ALL?

A

Chemotherapy (+consider allopurinol)

168
Q

What is thrombocytopenia?

A

Low platelet count of various causes.
Platelet count < 50 x10^9/L

169
Q

What is Immune thrombocytic purpura?(ITP)

A

Autoimmune platelet destruction (IgG)
Also known as autoimmune thrombocytopenic purpura, idiopathic thrombocytopenic purpura, primary thrombocytopenic purpura

170
Q

What is the pathophysiology of ITP?

A

Antibodies are created against platelets causing an immune response against platelets ⇒ destruction of platelets and low platelet count (thrombocytopenia)
T1: children 2-6yrs, post viral infection (acute + severe)
T2: adult women with malignancy, HIV, other autoimmune condition (chronic + mild)

171
Q

What are the key presentations of ITP?

A

Purpuric rash
Easy bleeding
Menorrhagia
Otherwise well systemically

172
Q

What are the investigations for ITP?

A

FBC + blood film, (thrombocytopenia + ↑BM megakaryoblasts)

173
Q

What is the management for ITP?

A

Prednisolone + IVIg (decrease splenic platelet destruction)
Rituximab (monoclonal antibody against B cells)
Splenectomy

174
Q

WHat are the causes of thrombocytopenia?

A

viral infection
drugs (NSAIDs, heparin, digoxin, PPIs)
alcohol
malignancies
liver + renal disease
aplastic anaemias
SLE

175
Q

What are the causes of thrombocytosis?

A

reactive (infection, inflammation, haemorrhages, post-surgery)
malignancies
splenectomy
iron deficiency

176
Q

What ages are the peak incidence of ALL and AML in children?

A

ALL - 2-3 yrs
AML <2 years

177
Q

What is Fanconi anaemia? what causes it?

A

a rare inherited bone marrow failure syndrome characterised by pancytopenia, predisposition to malignancy and characteristic physical abnormalities/congenital malformations

form of aplastic anaemia

caused by pathogenic variants in one of numerous genes involved with DNA repair

178
Q

What are some signs/symptoms of fanconi anaemia in children?

A

bone marrow related symptoms:
- tiredness/fatigue
- frequent infections
- bleeding problems

physical abnormalities:
- short stature
- abnormal skin pigmentation
- skeletal malformations of the limbs
- microcephaly
- ophthalmic and GU tract anomalies

179
Q

How is Fanconi anaemia treated in children?

A

blood transfusions
treatment for low blood count, infections
surgery to correct malformed bones

180
Q

What is haemophilia? difference between A and B

A

x-linked recessive inherited bleeding disorders causing excessive and/or spontaneous bleeding

haemophilia A is caused by a deficiency of factor VIII, haemophilia B (christmas disease) is caused by a deficiency in factor IX

181
Q

What are the presenting features of haemophilia?

A

excessive bleeding in response to minor trauma
spontaneous bleeding
intracranial haemorrhage
haematomas and cord bleeding in neonates

182
Q

How is haemophilia managed in children? what is a potential complication of this treatment?

A

clotting factor replacement by intravenous infusion - either regularly or in response to bleeding

complication of this treatment is the formation of antibodies against the treatment resulting in inefficacy

183
Q

What are the direct and indirect Coombs test?

A

direct Coombs test is used to detect if antibodies or complement system factors have bound to RBCs surface antigens - a positive test indicates that an immune mechanism is attacking the patients RBCs

indirect Coombs test is used to detect in-vitro antibody-antigen reactions and used to screen pregnant women for antibodies that may cause haemolytic disease of the newborn

184
Q

How is haemolytic disease of the newborn treated?

A

phototherapy
blood transfusion
IVIG

185
Q

What is von willebrand disease?

A

the most common inherited cause of abnormal and prolonged bleeding which results from deficiency/absense or malfunctioning of a glycoprotein called von Willebrand factor which is important for platelet adhesion and aggregation in damaged vessels

186
Q

What are the 3 types of von Willebrand disease?

A

Type 1 = partial deficiency of VWF, the most common and mildest variant

Type 2 = reduced function of VWF

Type 3 = complete deficiency of VWF, the rarest and most severe type

187
Q

What are the key symptoms of VW disease?

A

unusually easy, prolonged or heavy bleeding:

bleeding gums with brushing
nosebleeds
easy bruising
heavy menstrual bleeding
heavy bleeding during and after surgical procedures

188
Q

What is the management for VW disease?

A

doesnt usually require daily treatment, usually managed in response to bleeding or trauma

desmopressin (stimulates vWF from endothelial cells)
tranexamic acid (antifibrinolytic which prevents/reduces bleeding)
VWF infusion
factor VIII plus VWF infusion

189
Q

What is a wilms tumour?

A

a tumour affecting the kidneys in children, typically under 5 years old

190
Q

What are the symptoms/signs of wilms tumour?

A

mass in the abdomen
abdo pain
haematuria
lethargy
fever
hypertension
weight loss

191
Q

How is wilms tumour diagnosed?

A

USS abdo
CT or MRI for staging
Biopsy = gold standard

192
Q

What is the management for wilms tumour?

A

surgical excision along with nephrectomy
adjuvant chemo/radiotherapy depending on spread of tumour

193
Q

What is a neuroblastoma? what is unusual about it?

A

tumour arising from neural crest tissue in the adrenal medulla and sympathetic nervous system

unusual in that spontaneous regression sometimes occurs in very young infants

194
Q

What are the clinical features of neuroblastomas?

A

most commonly presents as an abdominal mass of adrenal origin which is often large and complex, involving major blood vessels and lymph nodes

  • pallor
  • weight loss
  • hepatomegaly
  • bone pain
  • limp

less commonly:
- paraplegia
- cervical lymphadenopathy
- proptosis
- periorbital bruising
- skin nodules

195
Q

What investigations are used to diagnose neuroblastoma?

A

biopsy (GS)
bone marrow sampling
MIBG scan
raised urine catecholamine levels

196
Q

What is the management for neuroblastoma?

A

localised primaries without metastatic disease can be surgically removed

metastatic disease in older children is treated with chemotherapy, autologous stem cell rescue and radiotherapy

immunotherapy

197
Q

What is a retinoblastoma?

A

malignant tumour of retinal cells which can affect 1 or both eyes and results in severe visual impairment in children

all bilateral tumours are hereditary, as are about 20% of unilateral ones

198
Q

When and how do retinoblastomas usually present?

A

most children present within the first 3 years of life

red reflex replaced by white pupillary reflex
squint

199
Q

What is the treatment and prognosis of retinoblastoma?

A

aim is to cure and preserve vision

treatment is based on the ophthalmological findings chemotherapy and laser treatment
radiotherapy

most patients are cured although many are visually impaired

significant risk of recurrence in patients with hereditary retinoblastoma

200
Q

What are the types of brain tumours seen in children? x6

A

Majority of childhood traumas are infratentorial

Astrocytomas (vary from benign to highly malignant)
Medulloblastoma (arising in the midline of the posterior fossa)
Ependydoma
Brainstem glioma (malignant tumours with poor prognosis)
Craniopharyngioma (developmental tumour arising from the squamous remnant of Rathke pouch)
Atypical teratoid/rhabdoid tumour

201
Q

What are the symptoms/signs of brain tumours?? give 2 symptoms specifically seen in adolescents and 2 seen in infants

A

persistent or recurrent vomiting
problems with balance, coordination or walking
behavioural change
abnormal eye movements
seizures (minus fever)
abnormal head position

child/adolescent:
- persistent or recurrent headache
- blurred or double vision
- lethargy
- deteriorating school performance
- delayed or arrested puberty, slow growth

infants:
- developmental delay/regression
- progressive increase in head circumference, separation of sutures, bulging fontanella
- lethargy

202
Q

What are the 2 main primary bone tumours seen in children?

A

osteosarcoma
Ewing’s sarcoma

203
Q

How are bone tumours usually managed?

A

combination chemotherapy followed by surgery

204
Q

What is a hepatoblastoma?

A

a primary malignant liver tumour which usually presents with abdominal distension or a mass

205
Q

Which blood protein is suggestive of hepatoblastoma when raised?

A

elevated serum alpha fetoprotein

206
Q

What is the management and prognosis for hepatoblastoma?

A

chemotherapy, surgery and in inoperable cases, liver transplantation

majority of children can be cured

207
Q

What is caput succadaneum?

A

oedema of the scalp at the presenting part of the head, typically the vertex

may be due to mechanical trauma of the initial portion of the scalp pushing through the cervix in a prolonged delivery or secondary to the use of ventouse delivery

resolves within days

208
Q

What is a cephalohaematoma?

A

a swelling on the head of a newborn most commonly in the parietal region which doesn’t cross suture lines

more common following prolonged, difficult deliveries and typically develops several hours after birth

may take months to resolve

209
Q
A

Phase 3A (8 decks)

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