Paediatrics Flashcards

Question

How do foetus adapt to extrauterine life?

Answer 1

- Before birth, the blood vessels to and from the lungs are constricted, so most of the blood from the right side of the heart will pass through the ductus arteriosus into the aorta, and some will flow across the foramen ovale - Shortly before and during labour, lung liquid production is reduced - During descent through the birth canal, the infant's chest is squeezed and some lung fluid is drained - Several thermal, tactile and hormonal stimuli (mainly an increase in catecholamine) to initiate breathing - The first breath takes place an average of 6 seconds after delivery - Lung expansion occurs due to intrathoracic negative pressure - Regular breathing is usually established after 30 seconds - After a the infant gasps, the rest of the lung fluid is absorbed into the lymphatic and pulmonary circulation - Pulmonary expansion leads to reduced pulmonary resistance and an increase in pulmonary blood flow - The flow of oxygenated blood through the ductus arteriosus leads to closure of the duct

Answer 2

- In babies born via an elective Caesarean section, their chest would not have been squeezed so it may take longer for the fluid to be drained from the lungs

Answer 3

- May be due to asphyxia (lack of oxygen during labour or delivery)

Answer 4

- If a foetus is deprived of oxygen in utero, the foetus will attempt to breathe - BUT, as they are still in utero, this attempt will be unsuccessful - this is called primary apnoea - During the primary apnoea, heart rate is maintained - If oxygen deprivation continues, primary apnoea is followed by irregular gasping and then a second period of apnoea (secondary or terminal apnoea) - During terminal apnoea, the heart rate and blood pressure will fall

Answer 5

- If the infant is delivered after the terminal apnoea, they will need help with lung expansion (e.g. positive pressure ventilation or tracheal tube) - The foetus rarely experiences continuous asphyxia except in the case of placental abruption or complete occlusion of umbilical blood flow in a cord prolapse - More commonly, asphyxia during labour and delivery is intermittent (e.g. due to frequent uterine contractions) - The Apgar Score is used to describe a baby's condition at 1 and 5 mins after delivery - it is also measured at 5 min intervals thereafter, if the infant's condition remains poor

Answer 6

- Weight or abdominal circumference lies on a lower centile than that of the head - This occurs when the placenta fails to provide adequate nutrition late in pregnancy but brain growth is relatively spared at the expense of liver glycogen and skin fat - This type of growth restriction is associated with utero-placental dysfunction secondary to maternal pre-eclampsia, multiple pregnancy and maternal smoking - These infants tend to put on weight rapidly after birth

Answer 7

- Head circumference is equally reduced - Suggests a prolonged period of poor intrauterine growth starting in early pregnancy - Usually due to a small but normal foetus - It could also be due to a foetal chromosomal disorder, congenital infection, maternal drug/alcohol abuse or a chronic medical condition - These infants are more likely to be permanently small

Answer 8

- Intrauterine hypoxia and 'unexplained' intrauterine death - Asphyxia during labour and delivery

Answer 9

- Hypothermia (because of their large surface area) - Hypoglycaemia (from poor fat and glycogen stores) - Hypocalcaemia - Polycythaemia

Answer 10

- Above the 90th weight centile for their gestation - Macrosomia is a feature of infants of mothers with either permanent or gestational diabetes or a baby with a congenital syndrome (e.g. Beckwith-Wiedemann syndrome)

Answer 11

- Birth asphyxia from a difficult delivery - Breathing difficulty from an enlarged tongue in Beckwith-Wiedemann syndrome - Birth trauma (especially shoulder dystocia) - Hypoglycaemia (due to hyperinsulinism) - Polycythaemia

Answer 12

- Vitamin K

Answer 13

- An abnormality of the hip joint where the socket portion does not fully cover the ball portion, resulting in an increased risk of joint dislocation - Also known as congenital dislocation of the hip (CDH) - DDH is 6 x more common in girls and in breech presentation - Early recognition is important because splinting in abduction reduces long-term morbidity

Answer 14

- Infant needs to be relaxed - The pelvis is stabilised with one hand - With the other hand, the examiner's middle finger is placed over the greater trochanter and the thumb around the distal medial femur - The hip is held flexed and adducted - The femoral head is gently pushed downwards - If the hip is dislocatable, the femoral head will push posteriorly out of the acetabulum - The next part of the test looks at whether the dislocated femoral head can be positioned back into the acetabulum - The hip is abducted and upward leverage is applied - If dislocated, the hip will return with a clunk into the acetabulum

Answer 15

- For infants < 2 months with normal physical examination without instability, observation is recommended with serial examinations and ultrasound on a monthly basis - Hip abduction orthosis (splint) in a Pavlik harness if the dysplasia persists or worsens - Serial follow up and plain X-ray evaluation is recommended after 6 months of age - Breech Delivery: if no DDH at neonatal examination, arrange ultrasound scan at 6 weeks

Answer 16

- Vitamin K deficiency may result in haemorrhagic disease of the newborn - In most affected infants, the bleeding is mild (e.g. bruising, haematemesis, melaena), however, some suffer from intracranial haemorrhage which could cause permanent disability - Breast milk has low vitamin K whereas formula milk has much more vitamin K - Therefore, haemorrhagic disease of the newborn is less likely in infants fed formula instead of breast milk - NOTE: infants of mothers taking anticonvulsants (which impair synthesis of vitamin K-dependent clotting factors) are at increased risk of haemorrhagic disease - Haemorrhagic disease of the newborn can be prevented by giving IM vitamin K injections

Answer 17

- Performed in every baby - A blood sample is usually taken as a heel prick - It is taken once feeding has been established on day 5-9 of life - All infants are screened for - Phenylketonuria - Hypothyroidism - Haemoglobinopathies (sickle cell and thalassemia) - Cystic fibrosis - MCAD deficiency - NOTE: screening for cystic fibrosis is performed by measuring the serum immunoreactive trypsin, which is raised if there is pancreatic duct obstruction - If raised, DNA analysis is also performed to reduce the false-positive

Answer 18

- An earphone is placed over the ear and a sound is emitted which evokes an echo or emission from the ear if cochlear function is normal - If a normal result is not achieved with this test, you move on to the next test

Answer 19

- A computer will analyse the EEG waveforms evoked in response to a series of clicks

Answer 20

- In perinatal asphyxia, gas exchange, either placental or pulmonary, is compromised leading to cardiorespiratory depression - This leads to hypoxia, hypercapnia and metabolic acidosis - Reduced cardiac output leads to hypoxic-ischaemic injury to the brain and other organs

Answer 21

- Hypoxic event immediately before or during labour, delivery such as: - Failure of gas exchange across placenta (excessive or prolonged uterine contractions, placental abruption, ruptured uterus) - Umbilical blood flow disrupted (e.g. cord compression including shoulder dystocia, cord prolapse) - Inadequate maternal placental perfusion - Compromised foetus (anaemia, IUGR) - Failure of cardiorespiratory adaptation at birth (failure to breathe)

Answer 22

- Up to 48 hours after asphyxia

Answer 23

- MILD - infant is irritable and responds excessively to stimulation, may have staring eyes, hyperventilation and impaired feeding - MODERATE - abnormalities of tone and movement, cannot feed and may have seizures - SEVERE - NO normal spontaneous movements or response to pain, limbs fluctuate between hypotonia to hypertonia, seizures are often prolonged and refractory to treatment, multi-organ failure present NOTE: the neuronal injury in HIE may be immediate from primary neuronal death or may be delayed from reperfusion injury causing secondary neuronal death

Answer 24

- Mild HIE: a full recovery - Even infants with moderate HIE, who have recovered fully on neurological examination and are feeding normally by 2 weeks of age have an excellent long-term prognosis - Severe HIE has a mortality of 30-40% - Of the survivors of severe HIE, 80% have neurodevelopmental disabilities, particularly cerebral palsy - If MRI shows significant abnormalities at 4-14 days, there is a very high risk of later cerebral palsy

Answer 25

- Caput Succedaneum - bruising and oedema of the presenting part extending beyond the margins of the skull bones (resolves within a few days) - Cephalhaematoma - haematoma from bleeding below the periosteum, confined within the margins of the skull sutures. Usually involves the parietal bone (resolves over several weeks)

Answer 26

- Oedema and bruising from Ventouse delivery - Bruising - Abrasions - Forceps marks - Subaponeurotic haemorrhage

Answer 27

- Brachial nerve palsies result from traction to the brachial plexus nerve roots - This can occur during breech deliveries or with shoulder dystocia - Upper nerve root (C5 and C6) injury results in Erb's Palsy - NOTE: Erb's palsy can happen with phrenic nerve palsy which results in an elevated diaphragm - Most palsies will resolve completely

Answer 28

- Compression of the facial nerve against mother's ischial spine (this is also usually transient)

Answer 29

- Usually from shoulder dystocia - A snap may be heard during delivery and the infant may show reduced arm movement on the affected side - A lump may be noticed due to callous formation - Prognosis is excellent - no specific treatment needed

Answer 30

- Humerus fractures tend to occur with shoulder dystocia - Femoral fractures are more likely with breech deliveries - Infants might show some deformity, reduced movement of the limb and pain on movement - Fractures heal rapidly with immobilisation

Answer 31

- Also known as hyaline membrane disease - RDS is caused by a deficiency of surfactant (which is responsible for lowering surface tension) - RDS is more common, the more preterm the baby is - However, it can occur in term babies especially if they are born to diabetic mothers

Answer 32

- Surfactant is a mixture of phospholipids and proteins produced by type II pneumocytes - A deficiency of surfactant leads to widespread alveolar collapse and inadequate gas exchange

Answer 33

- Glucocorticoids given if preterm delivery is anticipated | - In babies born with RDS, artificial surfactant can be instilled into the lungs directly or via a tracheal tube

Answer 34

- Tachypnoea (> 60 breaths/min) - Laboured breathing with chest wall recession (particularly sternal and subcostal indrawing) and nasal flaring - Expiratory grunting to try create positive airway pressure during expiration and maintain functional residual capacity - Cyanosis if severe

Answer 35

- Oxygen and ventilation - CPAP or artificial ventilation via a tracheal tube may be necessary - Other options: mechanical ventilation, high-flow humidified oxygen therapy

Answer 36

- Air from overly distended alveoli may track into the interstitium, resulting in pulmonary interstitial emphysema (PIE) - In about 10% of infants ventilated for RDS, air will leak into the pleural cavity and cause a pneumothorax

Answer 37

- An increase in oxygen demands - Breath sounds - Chest expansion on the affected side will be reduced - A pneumothorax can be demonstrated by transilllumination

Answer 38

- Immediate decompression + oxygen therapy + chest drain if tension pneumothorax - To prevent pneumothoraces, infants should be ventilated with the lowest pressures that provide adequate chest movement and blood gasses

Answer 39

- Secondary to: - Meconium aspiration - Respiratory distress syndrome - Complication of ventilation

Answer 40

- These episodes are very common in very low birthweight infants until they reach about 32 weeks' gestational age - It can occur either when an infant stops breathing over 20-30 seconds or when breathing continues against a closed glottis - It tends to be caused by immaturity of central respiratory control and breathing will usually start again after gentle physical stimulation - Caffeine can also help stimulate breathing - CPAP may be needed if apnoeic episodes are frequent - However, underlying causes of apnoea include hypoxia, infection, anaemia, electrolyte disturbances and hypoglycaemia

Answer 41

- Hypothermia ---> increased energy consumption ---> hypoxia and hypoglycaemia, failure to gain weight and increased mortality - Preterm infants are particularly vulnerable to hypothermia, as: - Large surface area to volume ratio (i.e. relatively little mass to generate heat, and lots of surface area from which you can lose heat) - Thin skin that is heat permeable (transepidermal water loss) - Little subcutaneous fat for insulation - Often nursed naked and cannot conserve heat by curling up or generate heat by shivering - Preterm babies are kept in incubators to closely control the temperature and humidity

Answer 42

- This is common in many preterm infants - Shunting of blood across the ductus from the left to the right side of the circulation is most common in infants with RDS - It is sometimes asymptomatic - However, it can cause apnoea and bradycardia, increased oxygen requirement and difficulty in weaning the infant from artificial ventilation - Other signs include a bounding pulse (due to increased pulse pressure), prominent precordial impulse and a systolic murmur - Echocardiography can be used to assess the infant's circulation

Answer 43

- The duct can be CLOSED using: - IV Indomethacin - Prostacyclin synthetase inhibitor - Ibuprofen - If drugs are unsuccessful, surgical ligation or percutaneous catheter device closure may be used

Answer 44

- Preterm infants have a high nutritional requirement - Infants born at 35-36 weeks can suck and swallow milk - Less mature infants will need feeding via an orogastric or nasogastric tube - In very preterm infants, breast milk needs to be supplemented with phosphate and may also need supplementation of protein, calories and calcium - NOTE: there are special formulas made for very preterm infants, however, unlike breast milk, formula does NOT provide protection against infection - If the infant is very immature or sick, parenteral nutrition may be required - this is usually administered via a central venous catheter (e.g. PICC line) - IMPORTANT: PICC lines carry a significant risk of septicaemia (and other risk such as thrombosis) - NOTE: cows' milk based formula increases the risk of necrotising enterocolitis - Poor bone mineralisation used to be common in preterm infants, however, it can be prevented by provision of adequate phosphate, calcium and vitamin D - Iron is usually transferred to the foetus during the last trimester, so preterm babies often have low iron stores and are at risk of iron deficiency - Iron supplements are usually started at several weeks of age

Answer 45

- High risk of infection because IgG is mainly transferred across the placenta in the last trimester - Furthermore, infection in and around the cervix is often a reason for preterm labour - Most infections in preterm infants occur after several days and are nosocomial (often associated with indwelling catheters or artificial ventilation)

Answer 46

- 25% | - Most occur in 72 hours from birth

Answer 47

- Germinal matrix above the caudate nucleus

Answer 48

- Following perinatal asphyxia | - In infants with severe RDS

Answer 49

- Pneumothorax is a major risk factor for haemorrhage

Answer 50

- Unilateral haemorrhagic infarction involving the parenchyma of the brain - usually resulting in hemiplegia

Answer 51

- A large intraventricular haemorrhage may impair the drainage and reabsorption of CSF, leading to an accumulation of CSF - This may resolve spontaneously or may progress to hydrocephalus - Hydrocephalus may lead to separation of cranial sutures, a rapid increase in head circumference and the anterior fontanelle to become tense - A ventriculoperitoneal shunt may be required, but symptomatic relief by relieving CSF via a lumbar puncture or ventricular tap may be sufficient - Around 50% of infants with progressive post-haemorrhagic ventricular dilatation have cerebral palsy - Periventricular white matter brain injury may occur following infarction or inflammation and may occur in the absence of haemorrhage - This may resolve spontaneously - However, if cystic lesions become visible on ultrasound 2-4 weeks later, there is definite loss of white matter

Answer 52

- The presence of multiple bilateral cysts is called periventricular leukomalacia (PVL) - it is associated with an 80-90% risk of spastic diplegia - NOTE: PVL and intraventricular haemorrhage could both occur in the absence of clinical sign

Answer 53

- A form of cerebral palsy causing high tightness or stiffness in the muscles of the lower extremities

Answer 54

- Associated with bacterial infection of ischaemic bowel wall - Preterm infants fed with cows' milk formula more likely to develop this - May rapidly develop shock and require artificial ventilation - May progress to bowel perforation - this can be detected by X-ray or transillumination of the abdomen - Mainly affects preterm infants in the first few weeks of life - Long-term consequences include the development of strictures and malabsorption if extensive bowel resection is necessary

Answer 55

- Infant stops tolerating feeds - Milk is aspirated from the stomach - Vomiting (may be bile-stained) - Abdominal distension - Rectal bleeding (sometimes)

Answer 56

- Stop oral feeding - Broad-spectrum antibiotics - Surgery if bowel perforation/necrosis - Parenteral nutrition - Artificial ventilation and circulatory support

Answer 57

- Distended loops of bowel - Thickening of the bowel wall with intramural gas - Gas in the portal tract

Answer 58

- Affects developing blood vessels at the junction of the vascular and non-vascularised retina - This may progress to retinal detachment, fibrosis and blindness - Uncontrolled use of high concentrations of oxygen - This is found in about 35% of very low birthweight infants

Answer 59

- Infants who still have an oxygen requirement at a post-menstrual age of 36 weeks are described as having bronchopulmonary dysplasia (or chronic lung disease) - Lung damage occurs as a result of artificial ventilation, oxygen toxicity and infection - A chest X-ray may show widespread areas of opacification, sometimes with cystic changes - Some infants will need prolonged ventilation, but most will be weaned onto CPAP and then additional ambient oxygen - Corticosteroids may facilitate earlier weaning (although there are concerns about abnormal neurodevelopment) - Subsequent pertussis or RSV infection could lead to respiratory failure

Answer 60

- Additional iron supplementation needs to be given until 6 months corrected age - They are at increased risk of: - Poor growth - Pneumonia/wheezing/asthma - Bronchiolitis from RSV infection - Bronchopulmonary dysplasia - Gastro-oesophageal reflux - Complex nutritional and gastrointestinal disorders (e.g. following necrotising enterocolitis) - Inguinal hernia - NOTE: about 5-10% of very low birthweight infants develop cerebral palsy, but the most common impairments are learning difficulties

Answer 61

- Over 50% of all new born infants become visibly jaundiced - This is due to: - Marked physiological release of haemoglobin from the breakdown of red blood cells because of the high Hb concentration at birth - Red cell life span of newborn infants (70 days) is considerably shorter than that of adults (120 days) - Hepatic bilirubin metabolism is less efficient in the first few days of life - It is important to notice neonatal jaundice because: - It may be a sign of another disease (e.g. haemolytic anaemia, infection) - Unconjugated bilirubin can get deposited in the brain, particularly the basal ganglia, causing kernicterus

Answer 62

- Encephalopathy due to unconjugated bilirubin in the basal ganglia and brainstem nuclei - May occur when the level of unconjugated bilirubin exceeds the albumin-binding capacity of bilirubin of the blood - Free bilirubin is fat-soluble, so it can cross the blood-brain barrier

Answer 63

- Lethargy | - Poor feeding

Answer 64

- Irritability - Increased muscle tone causing the baby to lie with an arched back (opisthotonos) - Seizures - Coma

Answer 65

- Choreoathetoid cerebral palsy (due to damage to the basal ganglia) - Learning difficulties - Sensorineural deafness

Answer 66

- Usually due to haemolysis - Haemolytic Disorders - Rhesus Haemolytic Disease - Affected infants are usually identified antenatally - Severely affected infants will have anaemia, hydrops and hepatosplenomegaly with rapidly developing jaundice - NOTE: antibodies may develop to rhesus antigens other than D (e.g. Kell and Duffy) - ABO Incompatibility - Most ABO antibodies are IgM and do NOT cross the placenta - However, some group O women have an IgG anti-A-haemolysin in the blood, which can cross the placenta and haemolyse the red cells of a group A infant - Jaundice doesn't tend to be as bad as with rhesus disease and hepatosplenomegaly is absent - Direct antibody test (Coombs' test) is positive - G6PD Deficiency - Parents of affected infants should be given a list of drugs that they should avoid that may precipitate haemolysis - Spherocytosis - Congenital Infection - can cause jaundice < 24 hours after birth

Answer 67

- Physiological Jaundice - Most babies will become mildly jaundiced with no underlying cause - Breast Milk Jaundice - Jaundice is more common and prolonged in breast-fed infants - Hyperbilirubinaemia is unconjugated - Dehydration - Jaundice can be exacerbated if milk intake is poor - In some infants, IV fluids may be needed - Infection - A baby with an infection may develop unconjugated hyperbilirubinaemia from poor fluid intake, haemolysis, reduced hepatic function and an increase in enterohepatic circulation - Other causes - Bruising and polycythaemia can exacerbate jaundice - Crigler-Najjar syndrome (deficient or absent UGT)

Answer 68

- Biliary atresia (disease of the liver with abnormally narrow, blocked or absent bile ducts) - However, most cases of jaundice are unconjugated - In prolonged unconjugated hyperbilirubinaemia: - Breast milk jaundice (MOST COMMON, affected up to 15% of infants) - Infection (especially of the urinary tract) - Congenital hypothyroidism - Conjugated hyperbilirubinaemia will cause pale stools and dark urine - It may also cause hepatomegaly and poor weight gain

Answer 69

- Neonatal hepatitis syndrome | - Biliary atresia

Answer 70

- Jaundice tends to start on the head and face and spread down the trunk and limbs - Bilirubin checked with a transcutaneous bilirubin meter or blood sample - It is recommended in the UK that all babies should be checked clinically for jaundice in the first 72 hours of life, and if clinically jaundiced, a transcutaneous measurement should be made - The rate at which bilirubin rises tends to be linear until it plateaus, so serial measurements can be plotted on a chart and used to anticipate the need for treatment - Drugs that displace bilirubin from albumin (e.g. sulfonamides and diazepam) should be avoided in newborn infants

Answer 71

- Assessment - Visually inspect the baby in natural light - Measure Bilirubin - Use serum bilirubin - if jaundice developed in the first 24 hours of life or if the gestational age is < 35 weeks - In babies born > 35 weeks or with jaundice that develops after the first 24 hours - use a transcutaneous bilirubinometer - If the result is > 250 micromol/L, check the result by measuring serum bilirubin - Assess risk of developing kernicterus Increased risk if: - Serum bilirubin > 340 micrmol/L in babies > 37 weeks gestation - Rapidly rising bilirubin of > 8.5 micromol/L per hour - Clinical features of acute bilirubin encephalopathy - Serum bilirubin should be measured every 6 hours until it drops below the treatment threshold or becomes stable/falling - Investigation of Underlying Cause - Measure haematocrit - Blood group of mother and baby - DAT test (Coombs) - If the mother is Rh-negative, find out whether the mother received prophylactic anti-D immunoglobulin during pregnancy - Consider the following tests - FBC and blood film (e.g. looking for hereditary spherocytosis) - Blood G6PD levels (consider ethnic origin) - Microbiological cultures of blood, urine and/or CSF (if suspected infection

Answer 72

- Use threshold table to determine whether the bilirubin measurement warrants treatment - 1st line: Phototherapy - Light (wavelength 450 nm) form the blue-green band of the visible spectrum converts unconjugated bilirubin into a harmless water-soluble pigment that is predominantly excreted in the urine - Monitor the baby's temperature whilst having phototherapy and protect the baby's eyes - Phototherapy can be stopped once the serum bilirubin level is >50 micromol/L below the threshold for treatment - Check for rebound hyperbilirubinaemia by measuring serum bilirubin 12-18 hours after stopping phototherapy - Other treatment options - Exchange transfusion - IVIG

Answer 73

- Most common cause of respiratory distress in term infants - Cause: delayed reabsorption of lung liquid - More common in birth by C-section - Usually settles within the first day of life

Answer 74

- Meconium = dark green substance forming the first faeces of a newborn infant - Passed before birth by 8-20% of babies - It may be passed in response to foetal hypoxia - Asphyxiated infants may start gasping before delivery and aspirate the meconium - Meconium is a lung irritant and will cause both mechanical obstruction and chemical pneumonitis, and it predisposes to infection - The lungs become over-inflated and have patches of collapse and consolidation - There is a high incidence of air leak, leading to pneumothorax and pneumomediastinum - Artificial ventilation is often needed - Infants may develop persistent pulmonary hypertension

Answer 75

- If normal term infant with meconium-stained amniotic fluid but no history of GBS, observation is recommended - If there are risk factors or laboratory findings that are suggestive of infection, consider antibiotics - IV ampicillin AND gentamicin - Oxygen therapy and non-invasive ventilation (e.g. CPAP) may be used in more severe cases

Answer 76

- Prolonged rupture of the membranes, chorioamnionitis and low birthweight predispose to pneumonia - Broad-spectrum antibiotics are started early until the results of the infection screen are available - NOTE: milk aspiration can also cause respiratory symptoms in the newborn

Answer 77

- Life-threatening - Usually associated with birth asphyxia, meconium aspiration, septicaemia or RDS, but it can occur as a primary disorder - The high pulmonary vascular resistance leads to right-to-left shunting of blood - Cyanosis occurs soon after birth - CXR will show a normal sized heart but there may be some pulmonary oligaemia - An urgent echocardiogram is required to make sure that the infant does not have a congenital heart defect

Answer 78

- Mechanical ventilation and circulatory support - Inhaled nitric oxide (vasodilator) - Sildenafil (viagra) - High-frequency (oscillatory) ventilation may be useful - For severe but reversible cases, extracorporeal membrane oxygenation (ECMO) (where the infants is placed on heart and lung bypass for several days) may be used

Answer 79

- Many are diagnosed during antenatal ultrasound screening - In the newborn, it usually presents with failure to respond to resuscitation or respiratory distress - In most cases, there is a left-sided herniation of abdominal contents through the posterolateral foramen of the diaphragm - This will cause the apex beat and heart sounds to be displaced to the right, with poor air entry in the left - NOTE: vigorous resuscitation may cause a pneumothorax in the normal lung - Diagnosis is confirmed by X-ray - In most infants, the main consequence of a diaphragmatic hernia is pulmonary hypoplasia - compression by the herniated loops of bowel throughout pregnancy prevents the development of the lung in the foetus - If the lungs are hypoplastic, mortality is high

Answer 80

- A large NG tube; suction applied to prevent distension of the intrathoracic bowel - Once stabilised, the hernia will be surgically repaired

Answer 81

- Heart failure (abnormal heart sounds may be heard on auscultation) - IMPORTANT: the femoral arteries must be palpated in all infants with respiratory distress, as coarctation of the aorta and interrupted aortic arch are important causes of heart failure in newborn infants

Answer 82

- Bacteria will have either ascended from the birth canal or invaded the amniotic fluid - The foetus becomes infected because the foetal lungs are in direct contact with the infected amniotic fluid - These infants will develop pneumonia and secondary septicaemia/bacteraemia - On the other hand, with congenital viral infections and early-onset infection with Listeria monocytogenes, foetal infection is acquired via the placenta following maternal infection - Risk of early-onset infection is increased if there has been prolonged or premature rupture of the amniotic membranes and when chorioamnionitis is clinically evident (e.g. mother having fever during labour)

Answer 83

- Respiratory distress - Apnoea - Temperature instability - Other features: vomiting, poor feeding, abdominal distension, jaundice, neutropaenia, shock, irritability, seizures

Answer 84

- CXR - Septic screen - FBC for potential neutropaenia - Blood cultures - NOTE: measuring acute phase proteins (e.g. CRP) is helpful but it can take 12-24 hours to rise, so a single normal result doesn’t rule out infection

Answer 85

- Antibiotics are started immediately without waiting for culture results - IV antibiotics should cover: - Group B streptococci - Listeria monocytogenes - Other Gram-positive organisms (usually with benzylpenicillin or amoxicillin) - Gram-negative organisms (usually with an aminoglycoside such as gentamicin) - Example of suitable antibiotic regimen recommended by NICE: benzylpenicillin + gentamicin - If CRPs are negative and the infant appears to have recovered clinically, antibiotics can be stopped after 48 hrs - If the blood culture is positive or there are any neurological signs, CSF must be examined and cultured

Answer 86

- Source is often the infant's environment - Presentation is fairly non-specific - Common in the neonatal intensive care unit, due to sources of infection such as indwelling catheters, invasive procedures and tracheal tubes

Answer 87

- Coagulase negative staphylococcus (Staphylococcus epidermidis)

Answer 88

- Gram-positive bacteria (e.g. S. aureus and E. faecalis) | - Gram-negative bacteria (e.g. Klebsiella, Pseudomonas and Serratia)

Answer 89

- Examples of suitable regiments: - Ampicillin + gentamicin/cefotaxime - Flucloxacillin + gentamicin - If resistant, specific antibiotics (e.g. vancomycin for coagulase-negative staphylococci or enterococci) or broad-spectrum antibiotics (e.g. meropenem) may be needed - IMPORTANT: the use of prolonged or broad-spectrum antibiotics predisposes to invasive fungal infections (e.g. candidiasis) - Serial measurements of CRP are useful to monitor response to therapy

Answer 90

- Late signs: bulging fontanelle, hyperextension of the neck and back (opisthotonos) - Uncommon but high mortality and morbidity (1/3 of survivors have major sequelae)

Answer 91

- Ampicillin or Penicillin AND - A 3rd generation cephalosporin (e.g. cefotaxime)

Answer 92

- cerebral abscess

Answer 93

- Sepsis 6 - Supplemental oxygen - Gain IV or IO access and order blood cultures, blood glucose and arterial/capillary/venous gasses - IV/IO broad-spectrum antibiotics - IV fluids (be cautious about fluid overload) - Experienced senior clinicians should be involved early - Vasoactive inotropic support (e.g. adrenaline) should be considered early - Considered if normal physiological parameters are NOT achieved after > 40 ml/kg of fluid resuscitation - Take an ABC approach (secure the airway)

Answer 94

- 10-30% of pregnant women have faecal or vaginal carriage of group B streptococci - It can cause early- or late-onset sepsis - In early-onset sepsis caused by group B streptococcus, a newborn baby will present with respiratory distress and pneumonia - It may also cause septicaemia and meningitis - Up to half of infants born to mothers who carry group B streptococcus are colonised on their mucous membranes and skin - Prophylactic intrapartum antibiotics given IV can prevent this infection

Answer 95

- Preterm delivery - Prolonged rupture of membranes - Maternal fever during labour - Maternal chorioamnionitis - Previously infected infant

Answer 96

- Targeted antibiotic therapy - Benzylpenicillin OR ampicillin - AND gentamicin or cefotaxime or ceftriaxone - Supportive therapy

Answer 97

- Uncommon but serious - Organism is transmitted to the mother through food (e.g. unpasteurised milk, soft cheeses and undercooked poultry - It often causes mild influenza-like illness in the mother - It can pass to the foetus through the placenta

Answer 98

- Spontaneous abortion - Preterm delivery - Foetal/neonatal sepsis

Answer 99

- Meconium staining of the amniotic fluid (unusual in preterm infants) - Widespread rash - Septicaemia - Pneumonia - Meningitis

Answer 100

- Amoxicillin OR co-trimoxazole - IMPORTANT: trimethoprim is CONTRAINDICATED in pregnancy - If systemic infection: IV ampicillin or benzylpenicillin with gentamicin

Answer 101

- Common in the neonatal period - usually starting on the 3rd or 4th day - Cleaning with saline and water is all that is required - the condition will resolve spontaneously - Discharge and redness is suggestive of staphylococcal or streptococcal infection and can be treated with a topical ointment (e.g. neomycin)

Answer 102

- purulent discharge, conjunctival injection and swelling of the eyelids - Discharge should be Gram-stained and cultured - Treatment (usually a 3rd generation cephalosporin) needs to be started immediately because loss of vision could occur

Answer 103

- presents with a purulent discharge and swelling of the eyelids - Treatment: oral erythromycin for 2 weeks

Answer 104

- Azithromycin (ophthalmic) - OR erythromycin OR polymyxin B/trimethoprim - NOTE: antihistamines and artificial tears are given for viral conjunctivitis

Answer 105

- If the skin around the umbilicus becomes inflamed, systemic antibiotics are indicated - Sometimes the umbilicus can be prevented from involuting by an umbilical granuloma (an overgrowth of tissue that occurs during the healing process of the belly button) - This can be removed by applying silver nitrate or by applying a ligature around the stump

Answer 106

- Usually transmitted during passage through an infected birth canal or (occasionally) by ascending infection - Often the mother does not know that she is infected - More common in preterm infants

Answer 107

- Localised herpetic lesions on the skin or eye - Or encephalitis - Or disseminated disease

Answer 108

- If primary disease or genital herpetic lesions at the time of delivery, = Caesarean section - Aciclovir or valaciclovir can be given prophylactically to the baby during the at-risk period

Answer 109

- Infants of mothers who are HbsAg positive should receive the hepatitis B vaccination shortly after birth to prevent vertical transmission - Babies are at highest risk of becoming chronic carriers when their mothers are e-antigen positive but have no e-antibodies

Answer 110

- Mothers should receive antiviral monotherapy - Tenofovir disoproxil OR lamivudine - Babies at risk should receive passive immunisation with hepatitis B immunoglobulin AND hepatitis B vaccine

Answer 111

- Within the first 24 hours of those with: - IUGR - Preterm - Born to mothers with diabetes mellitus - Large-for-dates - Hypothermia - Polycythaemia - Ill - IUGR and preterm infants have poor glycogen stores - Infants of a diabetic mother undergo hyperplasia of the islet cells in the pancreas causing high insulin levels

Answer 112

- Jitteriness - Irritability - Apnoea - Lethargy - Drowsiness - Seizures

Answer 113

- > 2.6 mmol/L - During the first 24 hours many asymptomatic infants will have blood glucose below this level - Prolonged, symptomatic hypoglycaemia can cause permanent neurological disability

Answer 114

- Can be be prevented by early and frequent milk feeding - IV Glucose in refractory or severe hypoglycaemia - Glucagon and hydrocortisone as well

Answer 115

- Hypoxic-ischaemic encephalopathy - Cerebral infarction - Septicaemia/meningitis - Metabolic - Hypoglycaemia - Hypo/hypernatraemia - Hypocalcaemia - Hypomagnesaemia - Inborn errors of metabolism - Pyridoxine dependency - Intracranial haemorrhage - Cerebral malformations - Drug withdrawal (e.g. maternal opiates) - Congenital infection - Kernicterus

Answer 116

- Hypoglycaemia and meningitis need to be excluded or treated urgently - A cerebral ultrasound can be performed to identify haemorrhage or cerebral malformations - Anti-convulsants

Answer 117

- Infarction in the territory of the middle cerebral artery may present with seizures - Seizures can be focal or generalised - There are no other abnormal clinical features (unlike HIE) - Diagnosis can be confirmed with MRI - Mechanism: thrombotic (e.g. thromboembolism from placental vessels or inherited thrombophilia) - Prognosis is relatively good (only 20% have hemiparesis or epilepsy)

Answer 118

- May be unilateral or bilateral - Cleft LIP result from failure of fusion of the frontonasal and maxillary processes - In bilateral cases the pre-maxilla becomes anteverted - Cleft PALATE results from failure of fusion of the palatine processes and the nasal septum - These defects can occur as part of a syndrome (e.g. chromosomal disorders) - They are associated with maternal anticonvulsant therapy - Infants are prone to acute otitis media

Answer 119

- Specialised feeding advice may be necessary - Watch out for airway problems (e.g. Pierre-Robin sequence) - Pre-surgical lip tapping, oral appliances or pre-surgical nasal alveolar moulding (PNAM) may be needed to narrow the cleft - Definitive repair is by surgery

Answer 120

- Associated with - micrognathia (small jaw), - posterior displacement of the tongue (glossoptosis) - midline cleft of the soft palate - Can lead to difficulty feeding - Upper airway obstruction as tongue falls back- may cause cyanotic episodes - At risk of failure to thrive - Due to potential airway obstruction, infants may need to lie on their front - Eventually, as the mandible grows, these problems resolve

Answer 121

- Weight for age that falls below the 5th percentile on multiple occasions or weight deceleration that crosses 2 major percentile lines on a growth chart

Answer 122

- Usually associated with a trans-oesophageal fistula - Linkedt to polyhydramnios during pregnancy - If not diagnosed at birth, it typically presents with persistent salivation and drooling - The infant will cough and choke when fed and may have cyanotic episodes - They may aspirate saliva or milk into the lungs from the upper airways, and acid from the stomach - It can be checked by passing a wide-calibre feeding tube through the mouth and checking, using an X-ray, to see if it reaches the stomach - Almost half of babies with oesophageal atresia will have other congenital malformations (e.g. VACTERL: Vertebral, Anorectal, Cardiac, Trachea-oEsophageal, Renal and radial Limb)

Answer 123

- Persistent bile-stained vomiting, unless the obstruction is proximal to the ampulla of Vater - Abdominal distension is more prominent - High lesions will present soon after birth whereas lower lesions may take a few days

Answer 124

- Atresia or stenosis of the duodenum (1/3 have Down syndrome), jejunum or ileum - Malrotation with volvulus (could lead to infarction of the entire midgut) - Meconium ileus (almost all affected neonates have cystic fibrosis) - Meconium plug (a plug of congealed meconium causes lower intestinal obstruction)

Answer 125

- Diagnosed from clinical features and abdominal X-ray - Atresia or stenosis is treated surgically - A meconium plug will usually pass spontaneously - A meconium ileus can be dislodged using Gastrograffin

Answer 126

- Hirschsprung Disease - absence of the myenteric nerve plexus in the rectum which may extend along the colon - Rectal Atresia - absence of the anus at the normal site - Lesions are described as being high or low depending on whether the bowel ends above or below the levator ani

Answer 127

- Abdominal contents protrude through the umbilical ring and are covered by a transparent sac (formed by the amniotic membrane and peritoneum) - Often associated with other major congenital abnormalities

Answer 128

- Bowel protrudes through a defect in the anterior abdominal wall adjacent to the umbilicus and there is no covering sac - It is NOT associated with any other congenital abnormalities - Carries a much greater risk of dehydration and protein loss - So, the abdomen in affected infants should be wrapped in several layers of clingfilm - An NG tube is passed and aspirated frequently and an IV dextrose infusion is set up - Colloid support is often required to replace lost protein - Can be corrected with surgery

Answer 129

- Non-forceful return of milk, but they differ in degree - Posseting refers to small amounts of milk that often accompany the return of swallowed air (burp) - Occurs in nearly all babies at some point - Regurgitation describes larger, more frequent losses - May indicate the presence of more significant gastro-oesophageal reflux

Answer 130

- Vomiting is the forceful ejection of gastric contents - Vomiting is usually benign and is often caused by feeding disorders or mild gastro-oesophageal reflux or gastroenteritis - Potentially serious disorders need to be excluded if the vomiting is bilious or prolonged or if the child is systemically unwell or has faltering growth - In infants, vomiting may result from infection outside the GI tract (especially the urinary tract and CNS)

Answer 131

- The more proximal the obstruction, the more prominent the vomiting and the sooner it becomes bile-stained (unless the obstruction is proximal to the ampulla of Vater) - More distal obstructions are more likely to cause significant abdominal distension

Answer 132

- Bile stained vomit: Intestinal obstruction - Haematemesis: Oesophagitis, peptic ulceration, oral/nasal bleeding, oesophageal variceal bleeding - Projectile vomiting in the first few weeks of life: Pyloric stenosis - Vomiting at the end of paroxysmal cough: Whooping Cough (B. pertussis) - Abdominal tenderness/pain on movement: Surgical abdomen - Abdominal distension: Intestinal obstruction, including strangulated inguinal hernia - Hepatosplenomegaly: Chronic liver disease, inborn error of metabolism - Blood in the stool: Intussusception, bacterial gastroenteritis - Severe dehydration, shock: Severe gastroenteritis, systemic infection (UTI, meningitis), DKA - Bulging fontanelle, seizures: Raised ICP - Faltering growth: Gastro-oesophageal reflux disease, coeliac disease and other chronic GI conditions

Answer 133

- Defined as the involuntary passage of gastric contents into the oesophagus - EXTREMELY COMMON in infancy but nearly all cases will resolve by 1 year - Mechanism: Lower oesophageal sphincter relaxes due to functional immaturity - Gastro-oesphageal reflux is only referred to as gastro-oesophageal reflux disease when it starts causing significant problems

Answer 134

- predominantly fluid diet - mainly horizontal posture - short intra-abdominal length of oesophagus

Answer 135

- Cerebral palsy and other neurodevelopmental disorders - Preterm infants (especially those with bronchopulmonary dysplasia) - Following surgery for oesophageal atresia or diaphragmatic hernia

Answer 136

- Faltering growth from severe vomiting - Oesophagitis - Recurrent pulmonary aspiration (recurrent pneumonia, cough, wheeze) - Dystonic neck posturing - Apparent life-threatening events

Answer 137

- Usually clinical and no investigations are required. However, possible investigations include: - 24-hr oesophageal pH monitoring - 24-hr impedance monitoring - Endoscopy with oesophageal biopsies - Upper GI contrast study

Answer 138

- Referral - SAME DAY referral if haematemesis, melaena or dysphagia present - Arrange assessment by paediatrician if: - Red flag symptoms - Faltering growth - Unexplained distress - Unresponsive to medical therapy - Feeding aversion - Unexplained iron deficiency anaemia - No improvement after 1 year of age - Suspected Sandifer's syndrome - Refer if there are any complications - Recurrent aspiration pneumonia - Unexplained apnoeas - Unexplained epileptic seizure-like events - Unexplained upper airway inflammation - Dental erosion with neurodisability - Recurrent acute otitis media - Treatment - Reassure - It is very common - Begins early (< 8 weeks) and may be frequent - It usually becomes less frequent with time - Treatment and investigation is not usually needed - Review infant or child if: - Projectile regurgitation - Bile-stained vomit or haematemesis - New concerns (e.g. faltering growth, feeding difficulties) - Persistent, frequent regurgitation beyond the first year of life

Answer 139

- Caused by hypertrophy of the pyloric muscle causing a gastric outlet obstruction - Presents at 2-8 weeks of age, irrespective of gestational age - 4 x more common in boys - NOTE: vomiting stomach contents can result in a hypochloraemic metabolic alkalosis with low plasma sodium and potassium

Answer 140

- Vomiting (increases in frequency and forcefulness over time, ultimately becoming projectile) - Hunger after vomiting (until dehydration leads to loss of interest in feeding) - Weight loss if presentation is delayed

Answer 141

- Gastric peristalsis (a wave moving from left to right across the abdomen) - palpable RUQ pyloric mass (like an olive) - NOTE: if the stomach is distended with air, it will need to be emptied using an NG tube to allow palpation - Ultrasound may be useful

Answer 142

- IV fluids resuscitation - Needed to correct the fluid and electrolyte disturbance before surgery - This should be provided at 1.5 x maintenance rate with 5% dextrose + 0.45% saline - Definitive treatment = Ramstedt pyloromyotomy - This involves dividing the hypetrophied muscle down to but not including the mucosa - Can be open or laparoscopic

Answer 143

- Infection (urinary tract, middle ear, meningeal) - Pain from an unrecognised fracture - Oesophagitis - Testicular torsion - Other possible causes of crying: severe nappy rash, constipation, teething

Answer 144

- 'Colic' is used to describe a common symptom complex that occurs during the first few months of life - Typical pattern of symptoms: paroxysmal, inconsolable crying or screaming often accompanied by drawing up the knees and passage of excessive flatus takes place several times a day - The cause is suspected to be gastrointestinal although there is no firm evidence - Colic affects up to 40% of babies in the first few weeks of life - NOTE: if severe and persistent, it may be due to a cows' milk protein allergy - 'colic' type symptoms can result from issues with the feeding/burping technique. Immediately lying a baby down after feeding can cause persistent crying

Answer 145

- Reassure the parents that is common & should resolve by 6 months of age - Sources of Information/Support: NHS Choices leaflet, health visitor (help with feeding techniques etc.) - Strategies to sooth a crying infant: holding the baby, gentle motion, white noise - Encourage parents to look after themselves: get support from family and friends, meet other parents at a similar state (NCT), resting, putting the baby in a safe place to give yourself a time out - NOTE: do not recommend things like Infacol and Colief because there is an insufficient evidence base

Answer 146

- Exclude appendicitis as perforation can occur rapidly - Always check testes, hernial orifices and hip joints - Lower lobe pneumonia may cause abdominal pain - Primary peritonitis may be seen in patients with ascites due to nephrotic syndrome or liver disease - DKA can cause severe abdominal pain - UTI (including pyelonephritis) can cause acute abdominal pain - Pancreatitis can cause acute abdominal pain (serum amylase should be checked)

Answer 147

- Most common surgical cause of abdominal pain in children - It is very uncommon in children < 3 years - NOTE: perforation can occur rapidly in children because their omentum is less well developed and fails to surround the appendix - No laboratory investigation or imaging is consistently helpful in making a diagnosis - Neutrophilia is not always present on the blood count - WCC and organisms can be seen in the urine because the inflamed appendix may be adjacent to the ureter or bladder - Ultrasound may support the clinical diagnosis and show complications (e.g. abscess, perforation, appendix mass)

Answer 148

- Anorexia - Vomiting - Abdominal pain (initially central and colicky (appendicular midgut colic),but then localising to the right iliac fossa (from localised peritoneal inflammation))

Answer 149

- Fever - Abdominal pain aggravated by movement - Persistent tenderness with guarding in the RIF (McBurney's point tenderness) - NOTE: with a retrocaecal appendix, localised guarding may be absent

Answer 150

- SURGICAL EMERGENCY - The patient should be nil-by-mouth from the time of diagnosis - IV fluids should be started - Requires immediate hospital admission - Treatment of choice is an appendicectomy

Answer 151

- Non-specific abdominal pain refers to abdominal pain that resolves within 24-48 hours - The pain is less severe than acute appendicitis - Often accompanied by an upper respiratory tract infection with cervical lymphadenopathy - In some of these children, abdominal signs do NOT resolve and an appendicectomy is performed

Answer 152

- Mesenteric adenitis is often diagnosed in these children as enlarged mesenteric lymph nodes are seen at laparoscopy but with a normal appendix

Answer 153

- Describe the invagination of proximal bowel into a distal segment - Most commonly involves the ileum passing into the caecum through the ileocaecal valve - It is the MOST COMMON cause of intestinal obstruction in infants after the neonatal period - Peak age of presentation: 3 months - 2 years - Usually, there is no underlying intestinal cause - However, viral infection leading to enlargement of Peyer's patches may form the lead point of the intussusception - Other possible lead points include a Meckel's diverticulum (more likely in children > 2 years old) - IV fluid resuscitation is likely to be necessary as there is often pooling of fluid in the gut which could lead to hypovolaemic shock

Answer 154

- Stretching and constriction of the mesentery resulting in venous obstruction, causing engorgement and bleeding from the bowel mucosa, fluid loss, and, eventually, bowel perforation, peritonitis and gut necrosis - Intussusception requires prompt diagnosis, immediate fluid resuscitation and urgent reduction of the intussusception

Answer 155

- Paroxysmal, severe colicky pain with pallor - Child will become pale (especially around the mouth) and draw his/her legs up - There is recovery between episodes but the child may become lethargic - Refusing feeds - Vomiting (may be bile-stained depending on the location of the intussusception) - Sausage-shaped mass palpable in the abdomen - Redcurrant jelly stool comprising of blood-stained mucus - NOTE: this is a 'characteristic sign' but tends to occur later in the illness - Abdominal distension and shock

Answer 156

- X-ray may show distended small bowel with no gas in the distal colon and rectum - Abdominal ultrasound is useful to confirm the diagnosis and check the response to treatment (target sign)

Answer 157

- If no signs of peritonitis, rectal air insufflation (with fluoroscopy guidance) - This is successful about 75% of the time - The remaining 25% require an operation - Clinically stable with no contraindications to contrast enema reduction - Fluid resuscitation - Contrast enema (air or contrast liquid) - Contraindications - Peritonitis - Perforation - Hypovolvaemic shock - Broad-spectrum antibiotics - Clindamycin + gentamicin OR tazocin OR cefoxitin + vancomycin - 2nd line: surgical reduction with broad-spectrum antibiotics - If recurrent intussusception - consider investigating for a pathological lead point (e.g. Meckel's diverticulum)

Answer 158

- 2% of people have an ileal remnant of the omphalomesenteric duct (a long narrow tube that joins the yolk sac to the midgut lumen of the developing foetus) - It contains either ectopic gastric mucosa or pancreatic tissue - Most cases are asymptomatic - However, they could present with severe rectal bleeding (classically neither bright red nor true melaena) - This usually causes an acute reduction in haemoglobin - A technetium scan will demonstrate increased uptake by ectopic gastric mucosa in 70% of cases

Answer 159

- Intussusception - Volvulus - Meckel's diverticulitis (mimics appendicitis)

Answer 160

- Asymptomatic - Incidental imaging finding - NO treatment required - Detected during surgery for other reasons - prophylactic excision - Symptomatic - Bleeding - excision of diverticulum with blood transfusion (if haemodynamically unstable) - Obstruction - excision of diverticulum and lysis of adhesions - Perforation/peritonitis - excision of diverticulum or small bowel segmental resection with perioperative antibiotics

Answer 161

- During rotation of the small bowel in foetal life, if the mesentery is NOT fixed at the duodenojejunal flexure or in the ileocaecal region, its base is shorter than normal and is predisposed to volvulus - Ladd bands are peritoneal bands that may cross the duodenum (often anteriorly) - Two main presentations of malrotation: - Obstruction - Obstruction with compromised blood supply - Obstruction with bilious vomiting is the usual presentation within the first few days of life but it can occur later - Any child with dark green vomiting needs an urgent upper GI contrast study to assess intestinal rotation - If there are signs of vascular compromise, an URGENT LAPAROTOMY is needed

Answer 162

- Ladd procedure- detorting the bowel and surgically dividing the Ladd bands - Laparoscopic (if elective or non-urgent) or during open laparotomy (emergency or urgent) - The bowel is placed in the non-rotated position with the duodenojejunal flexure on the right and the caecum and appendix on the left - NOTE: the appendix is usually removed to avoid diagnostic confusion in case the child presents again with an acute abdomen - Antibiotics (cefazolin)

Answer 163

- Very common - Often defined as pain sufficient to interrupt normal activities and lasts for at least 3 months - An organic cause is found in < 10% - Pain is typically periumbilical and children are generally well - Constipation is a frequent cause of recurrent abdominal pain and should be excluded - It may occur as a manifestation of stress - It can occur as a result of a vicious cycle of anxiety - parents demanding more invasive investigations etc. could worsen the perceive symptoms

Answer 164

- Try identify serious case without unnecessary investigations - Inspect perineum for anal fissures and check the child's growth - Urine MC&S to exclude UTI - Abdominal US to exclude gallstones and pelvic ureteric junction obstruction - Coeliac antibodies and TFTs should be checked (although these are rare causes of recurrent abdominal pain) - Irritable bowel syndrome and functional dyspepsia are diagnoses of exclusion and should be explained

Answer 165

- ~50% of children rapidly become free of symptoms - In ~25%, symptoms can take months to resolve - In ~25%, symptoms continue or return in adulthood as migraine, IBS or functional dyspepsia

Answer 166

- Often associated with abdominal pain and headaches - Midline pain and is associated with vomiting and facial pallor - Often a personal or family history of migraine - The history tends to describe long-periods of no symptoms followed by a shorter period of non-specific abdominal pain and pallor, with or without vomiting - Treatment with anti-migraine medication could help

Answer 167

- Associated with altered GI motility and abnormal sensation of intra-abdominal events - Symptoms may be precipitated by intra-abdominal infection - Abnormally forceful contractions may occur in the small intestines of children with IBS - The disease has a significant psycho-social component (stress and anxiety) - There is often a positive family history - Some people with IBS will also have Coeliac disease (so Coeliac antibodies must always be tested)

Answer 168

- Non-specific abdominal pain (often periumbilical and relieved by defecation) - Explosive, loose or mucousy stool - Bloating - Feeling of incomplete defecation - Constipation (often alternating with normal or loose stools)

Answer 169

- Reassure - Identify sources of stress or anxiety and any foods that aggravate symptoms - Adequate fluid intake

Answer 170

- Helicobacter pylori

Answer 171

- It wakes them up at night - Pain radiates to the back - History of peptic ulceration in a first-degree relative

Answer 172

- Abdominal pain - Nausea - H. pylori causes this

Answer 173

- CLO and C-13 breath test - H. pylori produces urease - Stool antigen

Answer 174

- In children with suspected peptic ulceration, it should be treated with proton-pump inhibitors - If investigations suggest the presence of an H. pylori infection, eradication therapy should be given (amoxicillin AND metronidazole OR clarithromycin) - If they fail to respond to treatment, an upper GI endoscopy should be performed - If this is NORMAL, functional dyspepsia is diagnosed - NOTE: functional dyspepsia is probably a variant of IBS

Answer 175

- Early satiety - Bloating - Post-prandial vomiting - Delayed gastric emptying (due to gastric dysmotility)

Answer 176

- Oesophagus becomes inflamed due to eosinophils within the mucosa and submucosa - May present with vomiting, discomfort when swallowing or bolus dysphagia (when food gets stuck) - More common in children with other features of atopy: - Asthma - Eczema - Hay fever

Answer 177

- Endoscopy - Linear furrows and trachealisation of the oesophagus may be seen (looks like the trachea with rings protruding into the lumen) - Microscopically, eosinophilic infiltration will be identified

Answer 178

- Oral corticosteroids (fluticasone or viscous budesonide)

Answer 179

- Major cause of child mortality in developing countries - In the UK, around 10% of children < 5 yrs annually present with gastroenteritis - It is a common reason for hospital admission in young children - In developed countries, the most frequent cause of gastroenteritis is rotavirus (up to 60% of cases in children < 2 yrs) - It is particularly prevalent in winter and early spring

Answer 180

- Blood in the stools

Answer 181

- Campylobacter jejuni is the MOST COMMON bacterial cause of gastroenteritis in developed countries, is often associated with severe abdominal pain

Answer 182

- Shigella and some salmonellae ---> blood and pus in the stool, pain and tenesmus - Shigella infection may be accompanied by a high fever - Cholera and enterotoxigenic E. coli linked to profuse, rapidly dehydrating diarrhoea

Answer 183

- Dehydration leading to shock

Answer 184

- Infants (especially low birthweight) - If > 6 diarrhoeal stools in the past 24 hours - If vomited > 3 times in the past 24 hours - If unable to tolerate extra fluids - If they have malnutrition

Answer 185

- Greater surface area-to-weight ratio (therefore more insensible fluid losses) - Higher basal fluid requirements - Immature renal tubular reabsorption

Answer 186

- Assess for - Features of DEHYDRATION and shock - Features that might suggest an alternative diagnosis - Severity and possible cause of gastroenteritis - Consider hospital admission - Give rehydration advice - Maintenance fluid volume - 0-10 kg = 100 ml/kg - 10-20 kg = 1000 ml + 50 ml/kg for each kg over 10 - 20+ kg = 1500 ml + 20 ml/kg for each kg over 20 - Modes of rehydration - < 5 years = 50 ml/kg for fluid deficit replacement over 4 hours as well as maintenance with oral rehydration solution - 5+ years = 200 mL ORS after each loose stool - Perform stool sample analysis (if this reveals a causative organism, seek specialist advice regarding antibiotic treatment) - Give advice on: - Preventing spread - Follow up - NOTE: notify the local authority if a notifiable disease is diagnosed or suspected (e.g. cholera)

Answer 187

- Most ACCURATE measure = degree of weight loss during diarrhoeal illness - History and examination is used to classify the degree of dehydration as: - No clinically detectable dehydration (usually < 5% loss of body weight) - Clinical dehydration (usually 5-10% loss of body weight) - Shock (usually > 10% loss of body weight)

Answer 188

- Loss of sodium and water is in proportion and so the plasma sodium remains within the normal range (isonatraemic)

Answer 189

- When a large amount of water is drunk, there is a greater net loss of sodium than water, leading to a fall in plasma sodium (hyponatraemic) - Excess water moves from the extracellular compartment into cells - This increase in intracellular volume leads to an increase in brain volume, which may result in seizures - The marked extracellular depletion leads to a greater degree of shock per unit of water loss - Hyponatraemic dehydration is more common in poorly nourished infants in developing countries

Answer 190

- Less common type of dehydration - Water loss exceeds the relative sodium loss and plasma sodium concentration increases - This usually results from high insensible water loss (e.g. high fever, or hot/dry environment) or from profuse, low sodium diarrhoea - The extracellular fluid becomes hypertonic leading to movement of water into the extracellular compartment from the intracellular compartment - So, the signs of extracellular fluid depletion are less per unit of fluid loss (i.e. depression of the fontanelle, reduced tissue elasticity and sunken eyes are less obvious) - This form of dehydration is more difficult to identify clinically - It is a particularly DANGEROUS form of dehydration because water is drawn out of the brain and cerebral shrinkage within the skull may lead to jittery movements, increased muscle tone and hyperreflexia, altered consciousness, seizures and multiple small cerebral haemorrhages - Transient hyperglycaemia occurs in some patients with hypernatraemic dehydration, but this will correct itself without insulin

Answer 191

- Usually, no investigations are necessary - Stool culture may be indicated if: - Child appears septic - Blood or mucus in the stools - Child is immunocompromised - Recent foreign travel - Diarrhoea has not improved in 7 days - Diagnosis is uncertain - Plasma electrolytes, urea, creatinine and glucose should be checked if IV fluids are required or there are features of hypernatraemia - If antibiotics are started, a blood culture should be taken

Answer 192

- If clinical dehydration is not present, you aim to prevent it - If clinical dehydration is present, oral rehydration solution is the mainstay of therapy - IV fluids are only indicated for shock or deterioration or persistent vomiting

Answer 193

- Oral rehydration solution - If IV fluids are required, a rapid reduction in plasma sodium concentration and osmolality will lead to a shift of water into the cerebral cells and may result in seizures and cerebral oedema - So, the reduction in plasma sodium should be slow - The fluid deficit should be replaced over at least 48 hours and the plasma sodium should be measured regularly

Answer 194

- Are ineffective - May prolong the excretion of bacteria in the stools - Can be associated with side-effects - Add unnecessarily to cost - Focus attention away from oral rehydration

Answer 195

- Only indicated for: - Suspected or confirmed SEPSIS - Extra-intestinal spread of bacterial infection - Salmonella gastroenteritis if < 6 months - Malnourished or immunocompromised children - Specific bacterial or protozoal infections (e.g. C. difficile associated with pseudomembranous colitis, cholera, shigellosis, giardiasis) - Not routinely required to treat gastroenteritis (even if the cause is bacterial)

Answer 196

- In developing countries, multiple episodes of diarrhoea is a major contributing factor to the development of malnutrition - Nutritional intake needs to be increased after diarrhoeal illness - Diarrhoea may lead to zinc deficiency and supplementation may be necessary

Answer 197

- Sometimes, after gastroenteritis, introducing a normal diet results in a return of the watery diarrhoea - Oral rehydration therapy should be restarted

Answer 198

- Abnormal stools - Poor weight gain or faltering growth in most but not all cases - Specific nutrient deficiencies, either singly or in combination - True malabsorption stool is difficult to flush and has a potent smell - NOTE: colour is not a good gauge of abnormality - Some disorders affecting the small intestine mucosa or pancreas (e.g. chronic pancreatic insufficiency) may lead to malabsorption of many nutrients (pan-malabsorption)

Answer 199

- Autosomal recessive metabolic disorder characterised by the malabsorption of zinc which results in: - Diarrhoea - Inflammatory rash around the mouth and/or anus - Hair loss

Answer 200

- Enteropathy in which the gliadin of gluten in wheat, barley and rye provoke a damaging immune response in the proximal small intestine - This result in a massive increase in the rate of migration of absorptive cells moving up the villi from the crypts - However, this is insufficient to compensate for increased cell loss from the villous tips - This leads to villi becoming progressively shorter and then absent, leaving a flat mucosa - Affects around 1% of the population - Age of presentation is partly affected by the age of introduction of gluten into the diet

Answer 201

- Profound malabsorptive syndrome at 8-24 months of age after the introduction of wheat-containing weaning foods. Other features include: - Faltering growth - Abdominal distension - Buttock wasting - Abnormal stools - General irritability - IMPORTANT: this 'classical' presentation is no longer the most common - children are more likely to present less acutely in later childhood

Answer 202

- Mild, non-specific GI symptoms - Anaemia - Faltering growth

Answer 203

- Anti-tTG antibodies - Endomysial antibodies - Duodenal biopsy (required to confirm the diagnosis) - Catch up of growth following gluten withdrawal from the diet is also required to confirm a diagnosis of Coeliac disease

Answer 204

- Remove wheat, rye and barley from the diet - Monitor body weight, height and BMI to assess for signs of malnutrition - Refer to dietician if there are problems adhering to the diet - Arrange annual review - Non-adherence to the gluten-free diet results in: - Micronutrient deficiency (especially osteopaenia) - Small increased risk of bowel cancer (especially small bowel lymphoma)

Answer 205

- Previously known as toddler diarrhoea - Most common cause of persistent loose stools in preschool children - Stools vary in consistency, are sometimes well formed, sometimes explosive and loose - Affected children are well and thriving - In some cases, it might result from undiagnosed coeliac disease or excessive ingestion of fruit juice (especially apple juice) - Occasionally caused by temporary cows' milk allergy after gastroenteritis - Once most probably causes have been excluded, most cases are probably a result of dysmotility of the gut (form of IBS) and fast-transit diarrhoea - These tend to improve with age

Answer 206

- Crohn's disease is more common than ulcerative colitis - Crohn's can occur anywhere from mouth to anus, whereas in UC the inflammation is confined to the colon - IBD can result in poor general health, growth restriction and have adverse effects on psychological well-being

Answer 207

- NOTE: lethargy and general ill health without GI symptoms can be the presenting features in older children - It may be mistaken for psychological problems and can mimic anorexia nervosa - Helpful features in making a diagnosis: - Raised inflammatory markers (platelet count, ESR and CRP) - Iron deficiency anaemia - Low serum albumin - Crohn's most commonly affects the distal ileum or proximal colon - Strictures and fistulae may develop

Answer 208

- Based on endoscopic and histological findings on biopsy

Answer 209

- upper GI endoscopy, ileocolonoscopy and small bowel imaging - May reveal narrowing, fissuring, mucosal irregularities and bowel wall thickening

Answer 210

- Presence of non-caseating epithelioid cell granulomata | - NOTE: this is NOT identified in up to 30% at presentation

Answer 211

- Nutritonal Therapy - Normal diet replaced by whole protein modular feeds (polymeric diet) - Lasts 6-8 weeks - Effective in 75% of cases - Systemic steroids are required if this is ineffective

Answer 212

- Immunosuppressants (e.g. azathioprine, mercaptopurine, methotrexate) - Anti-TNF agents (e.g. infliximab, adalimumab) may be needed if conventional treatments fail - Long-term supplemental enteral nutrition (e.g. NG or gastrostomy feeds) may be needed to correct growth failure

Answer 213

- Obstruction - Fistulae - Abscess formation - Severe localised disease that is unresponsive to medical treatment

Answer 214

- long-term prognosis for Crohn's beginning in childhood is GOOD and most people lead normal lives, despite occasionally relapsing

Answer 215

- Assess impact on daily functioning (anxiety, depression) - Provide info on Crohn's disease and support (Crohn's and Colitis UK) - Encourage stopping smoking (may reduce the risk of relapse) - Assess risk of osteoporosis - Medical Management - Steroids (prednisolone) may be used to induce and maintain remission - Immunosuppressive drugs (azathioprine, methotrexate) - Biologic therapies (e.g. infliximab) - Aminosalicylates (e.g. mesalazine) - Need to monitor certain biochemical measures (e.g. ferritin, B12, calcium and vitamin D) - Educate about features of Crohn's flare up (e.g. unintended weight loss) - NOTE: patients on immunosuppressive therapies should NOT have live vaccines. They are at increased risk of influenza and pneumococcal infection so should receive vaccines.

Answer 216

- recurrent, inflammatory and ulcerating disease involving the mucosa of the colon

Answer 217

- Rectal bleeding, - Diarrhoea - Colicky pain - Weight loss and growth failure may also occur

Answer 218

- Erythema nodosum | - Enteric arthritis

Answer 219

- Endoscopy and histology - Histological Features: - Mucosal inflammation - Crypt damage (cryptitis, architectural distortion, abscesses and crypt loss) - Ulceration - In most ADULTS, inflammation is usually confined to the distal colon - 90% of CHILDREN, have pancolitis

Answer 220

- Severity assessed with the Paediatric Ulcerative Colitis Activity Index (PUCAI) - Severe > 65 points - Mild-Moderate - 10-64 points - Mild Proctitis or Proctosigmoiditis - Oral or topical aminosalicylates or combo of the two - If aminosalicylates are NOT tolerated or are contraindicated: - Topical corticosteroid or oral prednisolone - Mild Left-Sided and Extensive Ulcerative Colitis - Oral aminosalicylate - Also consider topical aminosalicylate or oral beclometasone - If these are NOT tolerated, consider oral prednisolone - If there is no improvement 4 weeks after starting aminosalicylate therapy - Consider oral prednisolone - Still no adequate, consider adding oral tacrolimus - Biological agents e.g. infliximab, adalimumab and golimumab - Resistant disease may require surgery - Maintaining Remission - Aminosalicylates - Consider oral azathioprine or oral mercaptopurine if aminosalycilates are inadequate/frequent exacerbations requiring steroids - Severe fulminating disease- EMERGENCY - Assess likelihood of needing surgery - Increased likelihood of needing surgery if: - Stool frequency > 8 per day - Pyrexia - Tachycardia - AXR showing colonic dilatation - Low albumin, low haemoglobin, high platelets or CRP - Offer IV corticosteroids to induce remission - Consider IV ciclosporin (if IV corticosteroids are contraindicated or ineffective) - Surgical treatment - Colectomy with an ileostomy or ileojejunal pouch - UC is associated with an increased risk of bowel cancer Regular colonoscopic screening performed after 10 years of diagnosis

Answer 221

- The infrequent passage of dry, hardened faeces often accompanied by straining or pain and bleeding associated with hard stools - There may be some abdominal pain, which waxes and wanes with the passage of the stool - On examination, most children will be well with normal growth and a soft abdomen

Answer 222

- Dehydration - Reduced fluid intake - Anal fissures - Anxiety

Answer 223

- Hirschsprung disease - Lower spinal cord problems - Anorectal abnormalities - Hypothyroidism - Coeliac disease - Hypercalcaemia

Answer 224

- Exclude red flag symptoms and underlying causes - Laxatives - may have to be taken for several months - Check for faecal impaction - if present, recommend disimpaction regimen - Start maintenance laxative treatment if impaction is not present/has been treated - Advice behavioural interventions (scheduled toileting, bowel habit diary, reward system) - Diet and lifestyle advice (adequate fluid intake) - Follow up to assess adherence and response to treatment - Fluid requirements - Infants 0–6 months of age: 700 mL, assumed to be from milk. - Babies 7–12 months of age: 800 mL from milk and complementary foods and beverages, of which 600 mL is assumed to be water from drinks. - Children 1–3 years of age: 1300 mL (900 mL from drinks). - Children 4–8 years of age: 1700 mL (1200 mL from drinks). - Children 9–13 years of age - Boys — 2400 mL (1800 mL from drinks). - Girls — 2100 mL (1600 mL from drinks) - Secondary behavioural problems are common - Initial Aim: evacuate the overloaded rectum completely - Achieved using a disimpaction regimen of stool softeners, initially with a macrogol laxative (e.g. polyethylene glycol 3350 + electrolytes (Movicol Paediatric Plain)) - An escalating dose regimen is administered over 1-2 weeks or until impaction resolves - If unsuccessful: a stimulant laxative (e.g. senna or sodium picosulphate) may also be required - NOTE: if polyethylene glycol and electrolytes is not tolerated, an osmotic laxative (e.g. lactulose) can be used instead - Disimpaction must be followed by maintenance treatment to ensure ongoing regular, pain-free defecation - Polyethylene glycol with/without a stimulant laxative are the treatment of choice - Dose should be reduced over a period of months in response to an improvement in stool consistency and frequency - NOTE: dietary changes tend to have little effect in these situations (the addition of more fibre may make the stools larger and more difficult to pass) - Children can be encouraged to sit on the toilet after mealtimes to utilise the physiological gastrocolic reflex and improve the likelihood of success - Behavioural interventions (e.g. star chart) may be useful to aid motivation and keep a record - MAINSTAY OF TREATMENT - Early, aggressive and prolonged use of laxative medication - Should allow the passage of a large, soft stool at least once a day - Emphasise that laxative use is safe, even in the long-term - Underuse is the most common cause of treatment failure - Occasionally, faecal retention may be so severe that evacuation is only possible during: - Enemas - Manual evacuation under anaesthetic

Answer 225

- This condition is characterised by the absence of ganglion cells from the myenteric and submucosal plexuses of the large bowel - This results in a narrow and contracted segment of the large bowel - The region of abnormal bowel will extend from the rectum for a variable distance proximally - The region of abnormal bowel will end with a normally innervated, dilated colon - In 75% of cases, the lesion is confined to the rectosigmoid - In 10% of cases, the entire colon is affected - Occasionally, infants may present with severe, life-threatening Hirschsprung enterocolitis during the first few weeks of life - This is sometimes due to C. difficile infection - In later childhood, Hirschsprung disease can present with chronic constipation, which is usually profound and associated with abdominal distension - The child may also show signs of growth failure

Answer 226

- Usually in the neonatal period with intestinal obstruction - Can't pass meconium within the first 24 hrs of life - Later on, abdominal distension and bile-stained vomiting - DRE: narrowed segment and withdrawal of the examining finger often releases a gush of liquid stools and flatus

Answer 227

- Rectal biopsy: absence of ganglion cells, and presence of large acetylcholinesterase-positive nerve trunks - Anorectal manometry or barium studies may provide useful information about the length of the aganglioinic segment

Answer 228

- SURGICAL - Anorectal pull-through: colostomy followed by anastomosing normally innervated bowel to the anus - Initial management involves bowel irrigation