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PassMedicine > Paediatrics > Flashcards

Paediatrics Flashcards

1
Q

When does pyloric stenosis typically present

A
  • 2nd to 4th weeks of life.
  • Rarely later up to four months
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2
Q

Features of pyloric stenosis

A
  • Projectile vomiting after feed (30min after)
  • Constipation and dehydration
  • Palpable mass in upper abdomen
  • HYPOchloarmic, HYPOkalaemic, metabolic alkalosis due to vomiting
    • Loss of Cl ions and K ions in vomit
    • Alkalosis as loss of H ions in vomit
  • Hypochloraemia leads to elevated bicarbonate
    • Low Cl impairs kidney correction of alkalosis (through bicarbonate excretion)
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3
Q

Diagnosis of pyloric stenosis

A

Ultrasound

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4
Q

Management of pyloric stenosis

A

Ramstedt pyloromyotomy

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5
Q

What kind of pulse is felt in a child with a patent ductus arteriosus

A

Large volume, bounding, collapsing pulse

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6
Q

NICE indications for checking urine sample in a child

A
  • Symptoms or signs suggestive of UTI
  • Unexplained fever of >38
    • Test urine after 24hr the latest
  • Child with an alternative site of infection but who remain unwell
    • Consider urine test after 24 hr at the latest
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7
Q

Methods or urine collection

A
  • Clean catch
  • Urine collection pads
    • NOT cotton wool balls, gauze or sanitary pads
  • Invasive: suprapubic aspiration should only be done if non-invasive methods not possible
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8
Q

Treatment for lower UTI

A
  • 3 day course of antibiotics
    • Trimethoprim, nitrofurantoin, cephalosporin or amoxicillin
  • 5 day if not settled after 48hrs
    • SAFETY NET
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9
Q

Treatment for upper UTI

A
  • 10d course co-amoxiclav
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10
Q

Management of infant (<3months) with UTI

A

Refer immediately to paediatrician

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11
Q

Management of child >3 months old with upper UTI

A
  • Consider admission
  • If not admitted
    • Oral antibiotics
      • Cephalosporin or co-amoxiclav 10d
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12
Q

Management of child with recurrent UTI

A

Prophylactic antibiotics

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13
Q

Major risk factors of Sudden Infant Death Syndrome

A
  • Prone sleeping
  • Parental smoking
  • Prematurity
  • Bed sharing
  • Hyperthermia or head-covering
  • Male sec
  • Multiple births
  • Social class IV and V
  • Maternal drug use
  • Winter (increased incidence)

Odds ratios are additive.

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14
Q

What are some protective factors for sudden infant death syndrome

A
  • Breast feeding
  • Room sharing
    • NOT bed sharing
  • Use of pacifiers
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15
Q

Inheritence pattern of haeophilia A

A

X-linked Recessive

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16
Q

Affected Males can only have what phenotype of children

A
  • Carrier daughters
  • Unaffected sons
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17
Q

Risk factors for Developmental Dysplasia of the hip (DDH)

A
  • Female (6x risk)
  • Breech presentation
  • Family history
  • Firstborn
  • Oligohydramnios
  • Birth weight > 5kg
  • Congenital calcaneovalgus foot deformity
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18
Q

Screening indications for DDH

A

Ultrasound examination: at 6 weeks if

  • Family history (first degree) of hip problems in early life
  • Breech at or 36 week gestation irresepctive of presentation at birth or mode or delivery
  • Multiple pregnancy

Screening with Barlow and Ortolani:

  • ALL infants at newborn check and 6-week baby check
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19
Q

Barlow vs ortolani test

A
  • Barlow
    • Dislocates an articulated femoral head
  • Ortolani
    • Relocate dislocated femoral head
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20
Q

Features seen on clinical examination of infant with DDH

A
  • Barlow and ortolani test positive
  • Asymetry of leg length
  • Level of knees when hips and knees are bilaterally flexed
  • Restricted abduction of hip flexion
  • Skin fold
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21
Q

How do you confirm diagnosis of DDH

A

Ultrasound

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22
Q

Management of DDH

A
  • Pavlik harness
    • ​Must not be removed - advise on how to change clothes, clean harness but dont remove harness
  • Surgerical correction in older children
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23
Q

Causes of obesity in children (5)

A
  • Growth hormone deficiency
  • Hypothyroidism
  • Down’s SYndrome
  • Cushing’s Syndrome
  • Prader-Willi Sydrome
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24
Q

Complications in obese children

A
  • Orthopaedic problems
    • Slipped Upper Femoral Epiphyses
    • Blount’s disease (bowing of legs from tibia abnormality)
    • MSK pains
  • Psychological
    • Poor self-esteem, bullying
  • Respiratory
    • Sleep Apnoea
  • Benign Intracranial Hypertension
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25
Q

Long term complications of obses children

A
  • T2DM
  • Hypertension
  • Ischaemic Heart Disease
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26
Q

Micro-organism causing Hand, foot and mouth disease?

A
  • Picornaviridae family
    • Coxsackie A16
    • Enterovirus 71

Very contagious and outbreaks in nursery

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27
Q

Clinical features of hand foot and mouth disease

A
  • Mild systemic upset
    • Sore throat
    • Fever
  • Oral ulcers
  • Followed by vesicles on palms and soles of feet
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28
Q

Management and advice for hand foot mouth disease

A
  • Symptomatic therapy only
    • Hydration and analgesia
  • Advice
    • No need to exclude from school UNLESS child is feeling unwell, then exclude until feeling better
    • Contact you if there is a large outbreak
    • Reassure no link to disease in cattle
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29
Q

Key features of Osgood-Schlatter Disease (Tibial Apophysitis)

A
  • Sporty teenagers
  • No history of injury
  • Pain, tender and swelling over tibial tubercle
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30
Q

Key features of Chondromalacia patellae

A
  • Teenage girls
  • Softening of patella cartilage
  • Anterior knee pain on walking up and down stairs and rising from prolonged sitting
  • Responds to physiotherapy
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31
Q

Key features of Osteochondritis dissecans

A
  • Pain after exercise
  • Intermittent swelling and locking
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32
Q

Key features of Patellar Subluxation

A
  • Medial knee pain due to lateral subluxation of patella
  • Knee may give way
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33
Q

Key features of Patellar tendonitis

A
  • Athletic teenage boys
  • Chronic anterior knee pain that worsens after running
  • Tender below the patella on examination

Chondromalacia patellae common in teenage girls (anterior knee pain on walking up and down stairs)

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34
Q

List and describe primitive reflexes in neonates

A
  • Moro
    • Head extension causes abduction followed by adduction of arms
    • birth - 3/4 months old
  • Grasp
    • Flexion of fingers when objects placed in palm
    • birth - 4/5 months old
  • Rooting
    • Placing finger or nipple near baby’s mouth causes them to turn towards the stimulus and suck (sucking reflex)
    • birth - 4 months old
  • Stepping
    • Baby walks when held upright and soles of feet touch a flat surface
    • birth - 2 months old
  • ATNR
    • Face is turned to one side, the arm and leg on the side they are facing extend and the opposit arms and legs flex
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35
Q

Diagnostic criteria for whooping cough

A
  • Acute cough lasting >14 days without apprent cause and one or more of:
    • Paroxysmal cough
    • Inspiritory whoop
    • Post-tussive vommiting
    • Undiagnosed apnoeic attack in young infants
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36
Q

Diagnosis of whooping cough

A
  • Nasal swab culture for bordatella pertussis
    • several days or weeks for results
  • PCR and serology
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37
Q

Management of whooping cough

A
  • Infants <6months old = ADMIT
  • Notifiable disease
  • Oral macrolide if cough onset within previous 21 days
  • Houeshold contacts offered prophylaxis
  • School exclusion until 48 hours after commencing antibiotics OR 21 days from onset of illness if no antibiotic treatment
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38
Q

Which macrolides would you use for:

  • Infants <1 month old
  • Children > 1 month old and non-pregnant adults
  • Pregnant women
A
  • Infants <1 month old
    • Clarithromycin
  • Children > 1 month old and non-pregnant adults
    • Azithromycin or Clarithromycin
  • Pregnant women
    • Erythromycin
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39
Q

Complications of whooping cough

A
  • Subconjunctival haemorrhage
    • From cough
  • Pneumonia
  • Bronchiectasis
  • Seizures
    • From anoxia
  • Lymphocytosis
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40
Q

What screening test is used in newborns? What test is used if this is abnormal

A
  • Otoacoustic emmision test
    • Computer generated click in ear piece which meassures babys response
  • Auditory brainstem response test if above is abnormal
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41
Q

Management of neonates iwth hypoxic brain injury

A
  • Therapeutic cooling 33-35 degrees
    • Whole body or head cooling
  • Must be initiated within 6 hours of injury/birth
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42
Q

Triad For Shaken Baby syndrome

A
  • retinal haemorrhages
  • subdural haematoma
  • encephalopathy
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43
Q

Describe the basic physiology behind genitalia development

A
  • Initially gonads in foetus are undifferentiated
  • On the Y chromosome, there is a sex-determining gene (SRY gene) which causes differentation of gonads into testis
  • If absent, then the donads differentiate into ovaries
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44
Q

Three Causes of ambiguous genitalia

A
  • Congenital adrenal hyperplasia
  • True Hermaphodism
  • Maternal ingestions of androgens
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45
Q

What are some risk factors for haemorrhagic disease of the newborn

A
  • No Vitamin K at birth
  • Breat-feeding (breast milk not rich in Vit K)
  • Maternal use of anti-epileptics
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46
Q

How is Vit K administered

A

Once-off IM injection at birth

Or Oral

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47
Q

What are the causative organisms for bacterial pneumoniae in children

A
  • Streptococcus pneumoniae
  • Mycoplasma pneumoniae
  • Chlamydia pneumoniae

Not immunised/immunocompromised

  • Haemophilus influenza
  • Bordatella pertussis

Legionella unlikely in children (unless risk factors -AC)

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48
Q

Management of bacterial pneumoniae in children

A
  • Amoxicillin = first line in ALL children
    • Macrolide if no respones to amoxicillin
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49
Q

What antibiotic is used for mycoplasma or chlamydia poneumonia

A
  • Macrolides
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50
Q

If a bacterial pneumonia is associated with influenza, what antiubiotic should be used

A
  • Co-amoxiclav
    • Amoxicillin + clavulanic acid
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51
Q

Describe the features of ebstain’s anomaly

A
  • Septal and posterior leaflets of tricuspid valves low insertion into ventricle
  • Leads to large right atrium and small right ventricle - atrialisation of right ventricle
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52
Q

What drug is associated with Ebstein’s anomaly

A
  • Maternal use of lithium for BPD
    • First trimester association
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53
Q

What are some clinical features that you may see as a result of ebstain’s anomaly

A
  • Wolff-Parkinson White Syndrome
    • Delta waves
    • Pre-excitation syndrome involving accessory pathway between atrium and ventricle
  • Tricuspid incompetence
    • Giant V waves (JVP)
    • Pan-systolic murmur
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54
Q

What is a common benign cause of noisy breathing in infants

A
  • Laryngomalacia
  • congenital softening of the cartilage of the larynx, causing collapse during inspiration.
  • Laryngomalacia can present at birth, and worsens in the first few weeks of life. It usually self-resolves before 2 years of age.
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55
Q

Generally speaking what anomalies are found in autosomal dominant conditions

A
  • Structural
    • Hereditary spherocytosis
    • Marfan’s syndromes
    • Marfan’s syndromes
    • Achondroplasia
    • Huntington’s Disease
    • Neurofibromatosis
    • Osteogenesis Imperfecta
  • Exceptions - Ataxias
    • ataxia telangiectasia
    • Friedreich’s ataxia
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56
Q

Generally speaking what anomalies are found in autosomal recessive conditions

A
  • Metabolic
  • Exceptions
    • Hunter’s
    • G6PD are X-linked recessive
    • hyperlipidaemia type II and hypokalaemic periodic paralysis are autosomal dominant
    • Cystic fibrosis
    • Sickle Cell Disease
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57
Q

List the features of an innocent murmur

A
  • soft-blowing murmur in the pulmonary area or short buzzing murmur in the aortic area
  • may vary with posture
  • localised with no radiation
  • no diastolic component
  • no thrill
  • no added sounds (e.g. clicks)
  • asymptomatic child
  • no other abnormality
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58
Q

Name and describe two innocent ejection murmurs

A
  • Venous Hums
    • Due to the turbulent blood flow in the great veins returning to the heart. Heard as a continuous blowing noise heard just below bothclavicles
  • Still’s Murmur
    • Low-pitched sound heard at the lower left sternal edge

Ejection murmurs Due to turbulent blood flow at the outflow tract of the heart

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59
Q

Define pathological jaundice

A
  • Jaundice in the first 24 hr of life
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60
Q

Causes of pathological jaundice

A
  • Rhesus Haemolytic Disease
  • ABO Haemolytic Disease
  • Hereditary Spherocytosis
  • G6PD
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61
Q

Descibe physiological jaundice

A
  • Jaundice 2-14 days of life
  • Breastmilk jaundice
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62
Q

Describe prolonged jaundice

A
  • Jaundice lasting >14d
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63
Q

What are some causes of prolonged jaundice

A
  • Biliary atresia
  • Hypothyroidism
  • Galactosaemia
  • UTI
  • Breastmilk Jaundice
  • Congenital infections (toxoplasmosis, CMV)
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64
Q

What are some investigations to order for prolonged jaundice

A
  • Bilirubin
    • Conjugated vs Uncongjugated
  • TFTs
  • DAT (Coombe’s Test)
  • FBC and blood film
  • Urine and MC&S and reducing sugars
  • U&E
  • LFT
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65
Q

Slapped Cheek/Fifth Disease is caused by

A

Parvovirus B19

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66
Q

What is the first-line investigation for DDH in:

Newborn

Infants >4.5 months

A
  • Newborn = Ultrasound
  • Infants > 4.5 months
    • X-Ray
    • because ossification of the femoral head has occurred, meaning that x-rays are better able to visualise the joint.
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67
Q

What is the most common cause of hypothyroidism in the UK (and name two other causes)

A
  • Autoimmune thyroiditis
  • Post total-boyd irradiation
    • Child previously treated for ALL
  • Iodine deficiency (developing world)
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68
Q

Features of childhood sexual abuse

A
  • pregnancy
  • sexually transmitted infections, recurrent UTIs
  • sexually precocious behaviour
  • anal fissure, bruising
  • reflex anal dilatation
  • enuresis and encopresis
  • behavioural problems, self-harm
  • recurrent symptoms e.g. headaches, abdominal pain
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69
Q

When are chest compressions indicated in a newborn baby (after delivery)

A

When HR > 60

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70
Q

How is a newborn who has just been delivered managed if they are not breathing and is thought to be due to fluid in the lungs

A
  • 5 rescue breaths via 250mL face mask
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71
Q

Define Perthes’ Disease

A
  • Degenerative condition affecting hip joints in children (4-8 years).
  • Due to avascular necrosis of the femoral head (specifically the femoral epiphysis)
  • Impaired blood supply causes bone infarction

5 times more common in boys; around 10% cases are bilateral

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72
Q

Features of Perthes’ Disease

A
  • Hip pain that develops progressively over few weeks
  • Limp, antalgic gait
  • Stiffness and reduced range of hip movement
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73
Q

Diagnosis of Perthes’ disease

A
  • Plain X ray
  • Technetium bone scan or MRI if normal x-ray/symptoms persist
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74
Q

Management of Perthes’ Disease

A
  • Cast/Braces - keep femoral head within acetabulum
  • <6 years - observe
  • Older - surgical management
  • Operate on severe cases
  • Heals over 2-3 years
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75
Q

Complications of Perthes’ Disease

A
  • Osteoarthritis
  • Premature fusion of the growth plates
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76
Q

Describe the algorithm used for an unresponsive child

A
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77
Q

Describe the management of Cow’s Milk Protein Intolerance/Allergy

A
  • If Baby is formula-fed:
    • Use exyensive hydrolysed formula (eHF) in mild-moderate
    • Use amino acid-based formula (AAF) in severe or if no response to eHF
  • If baby is breastfed:
    • Continue breastfeeding
    • Eliminate cow’s milk protein from maternal diet (dairy).
    • Consider prescribing calcium supplements for breastfeeding mothers whose babies have/suspected CMPI to prevent deficiency whilst they exclude dairy from diet
    • Use eHF when breastfeeding stops until 12 months of age and at least for 6 months
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78
Q

What is the most common causative organism in gastroenteritis

A

Rotavirus

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79
Q

Causes of chrnoic diarrhoea in infants (<1 yr)

A
  • Cow’s milk intolerance
  • Toddler diarrhoea
  • Coeliac disease
  • Post-gastroenteritis lactose intolerance
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80
Q

What is the advice regarding exclusion for a child with:

  • Scarlet Fever
  • Whooping cough
  • Measles
  • Rubella
  • Chickenpox
  • Conjunctivitis
  • Fifth Disease
  • Mumps
  • D&V
  • Infectiours mononucleosis
  • Impetigo
  • Head lice
  • Threadworms
  • Scabies
  • Influenza
  • Hand, foot and mouth disease
A
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81
Q

What centile of height needs a review by a paediatrician

A

Children below 0.4th centile for height

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82
Q

What are the factors which affect foetal growth

A
  • Environmental
    • ​Maternal nutrition and uterine capacity
  • Placental
  • Hormonal
  • Genetic
    • Primarily maternal
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83
Q

What are the major drivers of growth in:

  • Infancy (birth-2 yrs)
  • Childhood (3-11yrs)
  • Puberty (11-18yrs)
A
  • Infancy
    • Insulin
    • Nutrition
  • Childhood
    • Growth hormone
    • Thyroxine
  • Puberty
    • Growth hormone
    • Sex Steroids
  • Genetic factors most important determinant of final adult height
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84
Q

Patau Syndrome genetic defect and features

A

Trisomy 13

  • Microcephalic, small eyes
  • Cleft lip/palate
  • Polydactyly
  • Scalp lesions
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85
Q

Genetic defect of Edward’s Syndrome and features

A

Trisomy 18

  • Micrognathia
  • Low-set ears
  • Rocker bottom feet
  • Overlapping fingers
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86
Q

Genetic Defect of Down Syndrome and features

A

Trisomy 21

  • Hypotonia at birth
  • Flat nasal bridge
  • Upward palpebral fissure (eyes)
  • Epicanthic fold
  • Single palmar crease
  • Sandal gap toe
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87
Q

Inheritence pattern of Fragile X Syndrome and features

A

X-linked dominant (CGG repeat FMR1 gene on X chromosome)

  • Learning difficulties; delays in S&L
  • Elongated face and broad forehead
  • Large ears
  • High arched palate
  • Prominent jaw
  • Strabismus
  • Macro-orchidism
  • Hypotonia and joint laxity
  • Flat feet (pes planus)

FMR1 = Fragile x mental retardation 1

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88
Q

Inheritence pattern of Noonan Syndrome and features

A

Autosomal dominant

  • Webbed neck
  • Pectus excavatum
  • Short stature
  • Pulmonary stenosis
  • Occular hypertelorism (distance between eyes)
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89
Q

Features of Pierre-Robin Syndrome/Sequence

A

Triad:

  • Micrognathia (small mandible)
  • Glossoptosis (posterior displacement of tongue -> upper airw3ay obstruction)
  • Cleft palate
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90
Q

Features of Prader-Willi

A

Genetic Imprinting

  • Hypotonia
  • Hypogonadism
  • Obesity
    *
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91
Q

Genetic abnormality in Williams Syndrome and features

A

Deletion of genes from chromosome 7s (or autosomal dominant)

  • Short stature
  • Learning difficulties
  • Friendly, extrovert personality
  • Transient neonatal hypercalcaemia
  • Suprclavicular aoritc stenosis
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92
Q

Cri du chat syndrome genetic abnormality and features

A

Chromosome 5p deletion

  • Characteristic cry (hence name) due to larynx and neuro problems
  • Feeding difficulties and poor weight gain
  • Learning difficulties
  • Microcephaly
  • Micrognathism
  • Hypertelorism
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93
Q

What is the classic disease evolution for chicken pox

A

Increased temperature for 2 days then develop clusters of erythematous vesicles of torso and face

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94
Q

Management of chicken pox

A

Supportive measures

Calamine lotion (itch)

Paracetamol

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95
Q

Why should ibuprofen (NSAIDs) be avoided in chicken pox

A

Associated risk of developing necrotising fasciitis

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96
Q

Infectivity of chicken pox

A
  • 4 days before rash and 5 days after rash appears
  • Incubation(10-21d)
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97
Q

Complications of chicken pox

A
  • Secondary infection of lesions
  • Secondary infection from group A Strep -> necrotising fasciitis
  • Encephalitis - rare but serious
    • PC: irritability, confusion, drowsiness, neck stiffness
  • Cerebellar ataxia
    • in recovery phase
  • Pneumonia
    • Chestp ain , SoB, wheeze
  • Myocarditis
  • Transient arthritis
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98
Q

Spot diganosis: A concave abdominal appearance in neonate presenting with dyspnoea and tachypnoea at birth

A
  • Congenital diaphragmatic hernia
  • It is characterised by the herniation of abdominal viscera into the chest cavity due to incomplete formation of the diaphragm.
  • This can result in pulmonary hypoplasia and hypertension which causes respiratory distress shortly after birth.
  • usually represents a failure of the pleuroperitoneal canal to close completely
  • Most common - left-sided posterolateral Bochdalek hernia which accounts for around 85% of cases.
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99
Q

When would you refer a child who is does not know about 2-6 words in voabulary

A

Refer at 18 months for SALT review

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100
Q

List the people involved in the MDT responsible for the care of someone with Cystic Fibrosis

A
  • Doctor
  • Nurse
  • Dietician
  • Social worker
  • Psychologist
  • Physiotherapist
  • Respiratory therapist (PFTs)
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101
Q

Outline the key points in the management of Cystic fibrosis

A
  • Regular (twice daily) chest physiotherapy and postural drainage
  • High calorie diet, high fat intake
  • Vitamin supplementation (DEKA)
  • Pancreatic enzyme supplements with meals
    • Can give PPIs to maintain alkaline conditions
  • Minimise contact with other CF patients to prevent cross infection
  • Symptom management
  • Pharmacological management
  • Bilateral lung transplant
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102
Q

What infections are commonly assoicated with cystic fibrosis

A
  • Pseudomonas aeruginosa
  • Burkholderia cepacia complex
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103
Q

What can be given for symptomatiuc control (as well as prophylacxis)

A
  • Salbutamol
  • Dornase alfa (mucolytic)
  • Antibiotic
    • Inhaled tobramycin (aminoglycoside)
  • Anti-inflammatory
    • Azithromycin/ibuprofen
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104
Q

Name some CFTR modulators used in cystic fibrosis and moa

A
  • Ivacaftor
    • Potentiator
  • Lumacaftor
    • Moves defective CFTR protein to cell surface
  • Tezacaftor

Usually Lum/Iva or Teza/Iva

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105
Q

What is a common complication of viral gastroenteritis

A
  • Post-gastroenteritis lactose intolerance
  • Remove lactose from diet for a few months followed by gradual reintrooduction to resolve
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106
Q

What is the most common cause of inherited neurodevelopmental delay

A

Fragile X Syndrome

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107
Q

Fragile X Syndrome is commonly associated with what disorder

A

Attention Deficit Hyperactivity Disorder (ADHD)

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108
Q

Diagnosis of Fragile X Syndrome

A
  • Antenatally by chorionic villus sampling or amniocentesis
    • Analysis of CGG repeats using restriction endonuclease digestion and southern blot analysis
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109
Q

Features of Cow’s Milk Protein Allergy/Intolerance

Allergy = oimmediate reactions; intolerance = mild-moderate delayed reactions

A
  • Regurgitation and vomitting
  • Diarrhoea
  • Urticaria
  • Atopic eczema
  • Colic symptoms: irritability, crying
  • Wheeze, chronic ocugh
  • Rarely angioedema and anaphylaxis
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110
Q

Diagnosis and investigation of cow’s milk protein allergy/intolerance

A
  • Diagnosis = clinical
  • Investigations
    • Skin prick/patch testing
    • Total IgE and Specific IgE (RAST) for cow milk protein
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111
Q

What are the causes of a cyanotic congenital heart disease

A
  • Tetralogy of Fallot
  • Transposition of the great arteries
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112
Q

Four characteristic features of tetralogy of fallot

A
  • Ventricular Septal Defect (VSD)
  • Right ventricular outflow obstruction - pulmonary stenosis
  • Right Ventricular Hypertrophy
  • Overriding aorta

Degree of RV outflow tract obstruction determines degree of cyanosis and clinical severity

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113
Q

Other features of tetrallogy of fallot

A
  • Cyanosis
  • Right-To-Left Shunt
  • Ejection systolic murmur (due to pulmonary stenosis)
  • CXR: Boot shaped heart
  • ECG: RV hypertrophy
  • Right-sided aortic arch in 25% patients
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114
Q

Difference in presentation of tetralogy of fallot and transposition of great arteries

A
  • TOF: 1-2 months
  • TGA: at birth
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115
Q

Management of Tetralogy of Fallot

A
  • Surgical repair in two parts
  • Beta blockers for reduction of infundibular spasms - less cyanotic episodes
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116
Q

Causative agent for roseola infantum (exanthem subitum or sixth disease)

A

Human Herpes Virus 6 (HHV6)

Incubation 5-15 days ant affect 6 months - 2 year olds

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117
Q

Features of roseola infantum

A
  • High fever followed by:
  • Maculopapular rash
  • Nagayama spots - pauplar enanthem on uvula and soft palate
  • Febrile convulsions 10-15%
  • Diarrhoea and cough

Consequences:

  • Aseptic meningitis
  • Hepatitis
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118
Q

Investigations for biliary atresia

A
  • Serum bilirubin and total bilirubin
    • Conj/unconj
  • LFT
    • Raised GGT (maybe raise AST/ALT)
  • Serum alpha1-antitrypsin
    • Neonatal cholestasis
  • Sweat chloride test
    • Cystic fibrosis
  • Ultrasound of biliary tree and liver
    • Distension and tract abnormalities
  • Percutaneous liver biopsy with intraoperative cholangioscopy
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119
Q

Features of growing pains

A
  • Never present at start of the day when child wakes
  • No limpNo limitation of activity
  • Systematically well
  • Normal physcial exmination
  • Normal motor milestones
  • Intermittent symptoms and worse after vigorous activity
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120
Q

Management of acute asthma

A
  • Oxygen via facemask (in severe and life-threatening)
  • B2 bronchodilator by spacer or nebuliser
    • 1 puff with tidal breathing every 60sec to a maximum of 10 puffs
  • Oral prednisolone 3-5 days
    • 20mg in 2-5 yrs
    • 30-40mg >5 yrs
  • Assess response
    • Poor - repeat B2 bronchodilator and admit
    • Good - continue bronchildator therapy but not exceeding 4 hourly
      • Continue prednisolone until recovery (min 3-5 days)
      • Arrange follow up clinic in next 48 hrs
      • Refer to secondary care if 2nd attack in 12 months
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121
Q

Characteristic features/evolution of roseola infantum

A

Roseola infantum is a common viral illness that causes a characteristic 3 day fever and then emergence of a maculopapular rash on the 4th day, following the resolution of the fever. The fever is typically rapid onset and can often predispose to febrile convulsions. The rash typically starts on the trunk and limbs (this is different to chickenpox which is typically a central rash). HHV6 is neurotropic (attacks the nervous system) and thus a rare complication is encephalitis and febrile fits (after cessation of the fever).

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122
Q

What are the two ways of measuring bilirubin in a neonate

A
  • Transcutaneous bilirubinometer
  • Serum bilirubin
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123
Q

What are the criteria for the use of transcutaneous bilirubinometer

A
  • > 35 weeks gestation
  • >24 hours old
  • Baby visibly jaundiced
  • Baby not on phototherapy
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124
Q

What are the criteria for the use of serum bilirubin in neonates

A
  • < 35 weeks gestation
  • < 24 hrs old and visibily jaundived
  • If transcutaneous bilirubinometer shows >250 micromol/litre, check bilirubiun using SBR
  • Any baby whose bilirubin level is at or above the treatment threshold for their postnatal age or is on phototherapy
  • If baby appears severely jaundiced
  • If haemolysis is likely cause
  • Baby clinically unwell (not feeding well, dehydrated)
  • Baby needs other blood tests other than SBR
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125
Q

When do you perform a neonatal blood spot screening occur in the UK

A
  • 5-9 days of life
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126
Q

What conditions are screened for in the neonatal blood spot screen

A
  • congenital hypothyroidism
  • cystic fibrosis
  • sickle cell disease
  • phenylketonuria
  • medium chain acyl-CoA dehydrogenase deficiency (MCADD)
  • maple syrup urine disease (MSUD)
  • isovaleric acidaemia (IVA)
  • glutaric aciduria type 1 (GA1)
  • homocystinuria (pyridoxine unresponsive) (HCU)
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127
Q

Define febrile convulsion

A
  • seizures provoked by fever in otherwise normal children.
  • Occurs in 6 months to 3 years old, happens in 3% children
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128
Q

What kind of infection usually precedes a febrile convulsion? And what type of seizure is it

A
  • Viral infection
  • Commonly tonic-clonic
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129
Q

Name and describe the differences between the sub-tyypes of febrile convulsions

A

Simple

  • <15 minutes
  • Generalised seizure
  • Typically no recurrence within 24hrs
  • Complete recovery within an hour post-seizure

Complex

  • 15-30 minutes
  • Focal seizure
  • May recurr within 24 hours

Febrile Status Epilepticus

  • > 30 minutes
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130
Q

Management of a febrile convulsion

A
  • Admit children if:
    • 1st seizure OR
    • Features of complex seizure
  • Recurrences:
    • Teach parents how to use rectal diazepam or buccal midazolam
    • Parents to phone ambulance if seizure lasts >5 minutes
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131
Q

What is the overall risk of a further febrile convulsion

A
  • 1 in 3
  • Varies greatly depending on risk factors for further seizure, more likely if
    • age of onset < 18 months
    • Fever < 40
    • Short fever duration before seizure (< 1hr)
    • FH of febrile convulsions
    • child previously had complex febrile seizure
    • Attending nursery - more likely to get viral illness
  • No evidence to show regular antipyretics reducing chance of febrile convulsion recurring
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132
Q

What is the risk, if any, between febrile convulsions and development of epilepsey

A
  • Childen with Hx of febrile convulsion at increased risk of developing epilepsey but risk is VERY SMALL
  • Child with Hx of simple = 1 in 50 (2%)
  • Child with Hx of complex = 1 in 20 (5%)
  • Child with no Hx of febrile convulsions = 1 or 2 in 100 (1-2%)
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133
Q

What are the risk factors for developing epilepsey in a child

A
  • FH
  • Having complex febrile convulsions
  • BAckground of neurodevelopmental disorder
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134
Q

What are the five autism spectrum disorders

A
  • Autism
  • Asperger’s Syndrome
  • Rett Syndrome
  • Childhood disintegrative disorder
  • Pervasive developmental disorder not otherwise specified (PDD-NOS)
    • Diagnosed when criteria not met for specific disorder
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135
Q

In a child with diarrhoea, what are the indications for a stool culture

A

NICE

  • Suspect septicaemia
  • Blood and/or mucus in stool
  • Immunocompromised child

Consider stool culture if:

  • Child recently abroad
  • Diarrhoea not improved by day 7
  • Uncertain about the diagnosis of gastroenteritis
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136
Q

Define caput succedaneum

A
  • Subcutaneous, extraperiosteal, collection of fluid that collects as a result of pressur on the baby’s head during delivery
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137
Q

Define cephalhaematoma

A
  • Haemorrhage between the skull and periosteum.
  • Sweeling is sub-periosteal so limited by the boundaries of the baby’s cranial bones
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138
Q

Compare similarities and differences between capu succedaneum and cephalhaematoma

A

Caput succedaneum

  • Present at birth
  • Typically over the vertex
  • Crosses suture lines
  • Resolves within days

Cephal Haematoma

  • Typically develops several hours after birth
  • Commonly in parietal region
  • Does not cross suture lines
  • May take months to resolve

Both

  • Swelling on the head of newborn
  • More common following prolonged and difficult deliveries
  • Managed conservatively
139
Q

What is the most common cause of hip pain in children

A

Transient synovitis

140
Q

Define transient synovitis and common in what ages

A
  • Transient inflammation of the synovium of the hip
  • Children 2-10 years; most common acute onset non-traumatic limp in children
  • Difficult to differentiate between transient synovitis and septic arthritis as children can still be unwell from preceeding viral illness

Septic arthritis is an orthopaedic emergency

141
Q

What can be used to indicate joint pain as septic arthritis over transient synovitis. Describe this criteria

A

Kocher’s Criteria:

  • > 38.5o
  • Refusal to bear weight on affected limb
  • Raised inflammatory markers
    • ESR > 40 mm/hr; CRP > 20 mg/L
  • Peripheral WCC > 12.0 x109 cell/L
142
Q

Name two acute onset joint pain conditions and two gradual onset joint pain conditions

A

Acute:

  • Transient synovitis
  • Septic arthritis

Gradual:

  • Perthes Disease
  • Slipped Upper Femoral Epiphysis (SUFE)
    • Abnormal X-Ray
143
Q

Describe the clinical features of Benign Acute Childhood Myositis (BACM)

A
  • Presents with severe pain over both calves
  • Raised Creatinine kinase
144
Q

When do children achieve day and night time continence by

A

Age 3 - 4 years

145
Q

Define nocturnal enuresis and two definition

A
  • Involuntary discharge of urine by night in child aged 5 years or older
  • In the absence of conggenital or acquired defects of the nervous system or urinary tract
  • Primary - child has never achieved continence
  • secondary - child dry for at least 6 months before
146
Q

Management of nocturnal enuresis

A
  • Routine and reward system MUST TRY FIRST
  • Enuresis alarm
    • 1st line for <7yrs
  • Desmopressin
    • Considered for children over 5 years
    • may be first line for >7 years
  • Oxybutynin - This is an anticholinergic medication that is used for nocturnal enuresis associated with overactive bladder. It is also used for urinary frequency, incontinence and neurogenic bladder instability.
  • Imipramine - TCA. It can be used in nocturnal enuresis in children who have failed to respond to all other treatments and have undergone specialist assessment.
147
Q

How is desmopressin monitored in the use of nocturnal enuresis

A
  • Iniitiate
  • Review in 4 weeks
  • Continue for 3 months if no response
  • Desmopressin should be withdrawn at regular intervals (1 week every three months) for full assessment
  • Care in avoiding fluid overload
    • Restrict fluid intake 1 hour before taking desmopressin until 8 hours after
  • Gradual withdrawal needed for desmopressin
148
Q

Define plagiocephaly

A
  • Skull deformity producing unilateral occipital flattening, which pushes ipsilateral forehead ear forwards producing a parallelogram appearance
  • Majority improve by age 3-5 due to adoption of upright posture
  • More common since campaigns to encourage babies to sleep on their backs to reduce risk of SIDS
149
Q

How could you manage plagiocephaly

A
  • Re-assure parents
  • Turn cot around to allow other side and take pressure off affected side
  • give baby time to be on their tummy during the day - supervised
  • Ensure all advice is in-line with prevention of SIDS
150
Q

Difference with plagiocephaly and craniosynostosis

A

Plagiocephaly

  • Parallelogram shaped head
  • Unilateral occipital flattening

Craniosynostosis

  • Premature fusion of skull bones
151
Q

Define coeliac disease

A
  • Sensitivity to gluten protein. Repeated exposure leads to villous atrophy leading to malabsorption
  • Present before age 3, following introduction of cereals into diet
152
Q

Genetic associations for coeliac disease

A
  • HLA-DQ2 (95%)
  • HLA-DQ8 (80%)
  • Incidence 1:1000
153
Q

Features of coelica diseaes in children

A
  • failure to thrive
  • Diarrhoea
  • Abdominal distension
  • Older children = anaemia
  • Many cases not diagnosed until adulthood
154
Q

Diagnosis of coeliac disease

A
  • Jejunal biopsy - subtotal villous atrophy and crypt hyperplasia
  • Screening antibodies: anti-endomysial and anti-gliadin
155
Q

Describe how Imprinting works and name an example

A
  • For the disease to occur, patient does not receive the gene from their father
  • The mother’s gene may be normal but it does not prevent the phenotype from occuring
  • Prader-Willi
156
Q

What kind of virus is the measles virus

A

RNA paramyxovirus

157
Q

How is measles spread

A

Spread by droplets

158
Q

Incubation period of measles

A

10-14 days

159
Q

Infective period of measles

A

From prodrome until 4 days after rash starts

160
Q

Features of measles

A
  • Prodrome: irritable, conjunctivits, fever
  • Koplik spots (before rash): white spots on buccal mucosa
  • Rash: starts behind ears then to whole body; discrete maculopapular rash becoming blotchy and confluent
161
Q

Investigations for measles

A
  • IgM antibodies in oral fluids
  • Can be detected within few days of rash onset
162
Q

Management of measles

A
  • Mainly supportive
  • Admission: immunosuppressed or pregnant patients
  • Notifeiable disease - inform public health england
163
Q

Complications of measles (what is most common and what is most common cause of death)

A
  • Otitis media - most common
  • Pneumonia - most common cause of death
  • Encephalitis
    • 1-2 weeks after illness onset
  • Subacute sclerosing panencephalitis
    • very rare, presents 5-10 years post illness
  • Febrile convulsion
  • Keratoconjunctivitis
  • Corneal ulceration
  • Diarrhoea
  • Increased incidence of appendicitis
  • Myocarditis
164
Q

How do you management contacts with measles

A
  • If child not immunised against measels and comes into contact with someone who does, MMR should be offered
    • Vaccine-induced antibodies develops more rapidly than that from natural infection
    • Give within 72 hours
165
Q

Clinical features of transposition of great arteries

A
  • Cyanosis
  • Tachypnoea
  • Loud Single S2
  • Prominent right ventricular impulse
  • Egg-on-side appearance on CXR
166
Q

Management of transposition of great arteries

A
  • Maintenance od ductus arteriosus with prostaglandins
    • Alprostadil (PGE1)
    • Misoprostol is also PGE1 but used to treat missed miscarriage, induce labour and induce abortion
  • Surgical correction is definitive treatment
167
Q

When would you refer a child for developmental problems

A
  • Does not smile 10 weeks
  • Cannot sit unsupported at 12 months
  • Cannot walk at 18 months
168
Q

True or false: Hand preference before 12 months is normal

A
  • False
  • It is abnormal and may indicate cerebral palsy
169
Q

What are the three most common gross motor problems in a developing child

A
  • Variant of normal
  • Cerebral palsy
  • Neuromuscular disorders
    • Duchenne Muscular dystrophy
170
Q

What are some examples of trinucleotide repeat disorders

A
  • Fragile X (CCG)
  • Huntington’s (CAG)
  • Myotonic dystrophy (CTG)
  • Friedreich’s ataxia (GAA)
    • Does not demonstrate anticipation
  • Spinocerebellar ataxia
  • Spinobulbar muscular atrophy
  • Dentatorubral pallidoluysian atrophy
171
Q

In trinucleotide repeat disorders, a phenomenon called anticipation occurs. What does this mean

A
  • Phenomenon which leads to earlier age of onset in successive generations
    • Repeat expansions are unstable and may enlarge
172
Q

What are some risk factors for birth injuries

A
  • Malpositioning
  • Too large for pelvic outlet
  • Manual maneuvres
  • Forcep blades
  • Ventouse deliveries
173
Q

What are the three main injury categories at birth

A
  • Soft tissue injury
  • Nerve palsies
  • Fractures
174
Q

Examples of soft tissue injury at birth and description of each

A
  • Capput succedaneum
  • Cephalhaematoma
  • Chignon
  • Bruising
    • Face, genitalia, buttocks after breech delivery
    • Preterms bruise easliy from mild trauma
  • Abrasions
    • From scalp electrodes applied during labour
    • Accidental scalpel incision at C-Section
  • Forcep marks
    • Face from pressure of blades - transient
  • Subaponeurotic haemorrhage
175
Q

Describe caput succedaneum

A
  • bruising and oedemaof presenting part extending beyond sutures (margins of skulkl bones)
  • Resolves in a few days
176
Q

Describe cephalhaematoma

A
  • Haematoma below the periosteum and confined within margins of skull sutures
  • Usually Parietal bone
  • Centre of haematoma is soft
  • Resolves after several weeks (month or two)
177
Q

Describe a chignon

A
  • Oedema and bruising from ventouse delivery
178
Q

Describe a subaponeurotic haemorrhage

A
  • Very uncommon
  • Diffuse, boggy swelling of scalp
  • Blood loss may be severe and can lead to hypovolaemic shock and coagulopathy
179
Q

How does subaponeurotic haemorrhage (subgaleal) differ to caput succedaneum or a cephalhaematoma

A

Subaponeurotic haemorrhage

  • Diffuse, fluctuant swelling of head which may shift with movement. Palpation of the scalp has been described as a leather pouch filled with fluid.
  • Can enlarge?

Caput succedaneum

  • Does not cross suture lines, SAH does

Cephalhaematoma

  • Both crosses suture lines but cephalhaematoma does not usually fluctuate or shift with movement
180
Q

When do nerve palsies typically occur

A
  • Breech deliveries
  • Shoulder dystocia
181
Q

Examples of nerve palsies in babies with description

A

Erb’s Palsy

  • C5 and C6 (upper nerve root)
  • Can be accopmanied by phrenic nerve palsy causing elevated diaphragm
  • Usually resolves completely (by 2 years) - refer to orthopaedic or plastic surgeon if not resolved by 2-3 months

Facial nerve palsy

  • Compression of facial nerve against mother’s ischial spine or pressure from forceps
  • Unilateral with facial weakness on crying but etye remains open
  • Transient
  • Methylcellulose drops may be needed for eye

Cervical spine damage

  • Rare
  • Laack of movement below level of lesion
182
Q

describe the possible fractures seen at birth

A

Clavicle

  • From shoulder dystocia
  • Snap may be heard at delivery or infant may have reduced arm movement or callus formation (lump) over clavicle at several weeks of age
  • No treatment needed, good prognosis

Humerus/Femur

  • From breech deliveries or shoulder sytocia, midshaft
  • Deformity and reduced limb movement and pain
  • Heal rapidly with immobilisation
183
Q

What are some medical problems that you may see in preterm infants

A

A

  • Need for resuscitation and stabilisation at birth
  • Apnoea and bradycardia

B

  • RDS
  • Pneumothorax
  • Bronchopulmonary dysplasia

C

  • Resuscitation and stbailisation
  • Hypotension
  • Patent ductus arteriosus
  • Intraventricular haemorrhage
  • Periventricular leukomalacia
  • Anaemia or prematurity

D

  • Metabolic
    • Hypoglycaemia, hypocalcaemia, electrolyte imbalances, osteopenia of prematurity
  • Temperature control
  • Nutrition
  • Infection
  • Jaundice

E

  • Necrotising enteroclolitis
  • Retinopathy or prematurity
  • Iatrogenic
  • Inguinal hernias
184
Q

What must you do to stabilise a preterm or sick infant

A
  • Airway and breathing prtoection
    • Manage (clear airway, O2, CPAP)
    • Monitor
  • Temperatrue control
  • Venous/Arterial lines
    • Preipheral IV line
    • Umbilical venous catheter
    • Arterial line
    • Peripherally iunserted central (PIC) lin for parenteral nutrition
  • CXR +/- AXR
  • Investigations
  • Antibiotics
  • Minimal handling
  • Counsel parents and let them see baby
185
Q

What are some investigations you would do when stabilising a preterm or sick infant

A
  • FBC (Hb, Neutrophil, Plt)
  • Blood urea, creatinine, electrolytes, lactate
  • Culture: blood ‎± cerebrospinal fluid ‎± urine
  • Blood glucose
  • CRP/acute phase marker
  • Coagulation screen
186
Q

What would you monitor when stbailising a preterm or sick infant

A
  • O2 saturation (aim 91-95% at preterm)
  • HR, RR
  • Temperature
  • BP
  • Blood glucose
  • Blood gases
  • Weight
187
Q

Compare some features of preterm baby (23-27weeks) to term baby (37-42 wks)

A

*

188
Q

How is a pneumothorax diagnosed in a baby, how is it managed and what are some precautions to prevent this

A
  • Diagnos: cold transillumination of chest or CXR
  • Tension pneumothorax needs urgent decompression with chest drain insertion
  • Prevent: Ventilated with lowest pressure that provide adequate chest movement and satisfactory blood gases
189
Q

What are some underlying causes of apnoea

A
  • Hypoxia
  • Infection
  • anaemia
  • Electrolyte imbalance
  • Hypoglycaemia
  • Seizures
  • Heart failure
  • Aspiration from GORD
  • Premature: immature respiratory control
190
Q

Management of apnoea

A
  • resipratory stimulant: caffeine or gentle physicla stimulation
  • CPAP or mechanical ventilation if frequent apnoeic episodes
  • Often 20-30 s
191
Q

What are some symptoms and signs of patnt ductus arteriosus

A

Symptoms

  • Asymptomatic
  • Apnoea (left to right shunt - breathless)
  • Increased oxygen requirement

Signs

  • Bradycardia
  • Bounding pulse (from increased pulse pressure)
  • HF signs from overload
192
Q

Management of PDA in preterm

A

Symptomatic

  • Prostaglandin synthetase inhibitor
    • Indomethacin
    • Ibuprofen
  • Surgical ligation if pharm does not work
193
Q

Compare the weight gain difference in term and pre term babies

A
  • Term
    • Double weight at 4.5 months
    • Treble at 12 months
  • Preterm (28 weeks)
    • Double in 6 weeks
    • Treble in 12 weeks

Need lots of nutrition to keep up with fast growht!

194
Q

With regards to nutrition in preterm baby, what supplements need to be given

A
  • Breast milk ASAP
  • Fortified with phosphate, protein, calories and calcium and Vit D
  • Formula milk - used but does not provide maternal immunity or breast milk benefits
    • NEC risk with cow-milk based
  • Parenteral nutrition in very imatture and sick infants (<1kg)
    • Risk of infection and thrombosis
  • Iron supplements
    • Iron loaded onto baby usually in last trimester
195
Q

Define necrotising enteroclitis

A

Serious illness caused by inflammation of the gut in preterm babiees

196
Q

Risk factors for NEC

A
  • Ischaemic injury
  • Bacterial invasion
  • Cow’s milk formula
197
Q

Symptoms of NEC

A
  • feeding intolerance
  • abdominal distension
  • bloody stools
  • Progress to: abdominal discolouration, perforation and peritonitis.
198
Q

Signs of NEC on AXR

A
  • dilated bowel loops (often asymmetrical in distribution)
  • bowel wall oedema
  • pneumatosis intestinalis (intramural gas)
  • portal venous gas
  • pneumoperitoneum resulting from perforation
  • air both inside and outside of the bowel wall (Rigler sign)
  • air outlining the falciform ligament (football sign)
199
Q

What is the management of NEC

A
  • Bowel rest - STOP oral feeding
  • Broad spectrum anti-iotics (aerobic and anaerobic cover)
  • Parenteral nutrition
  • Mechanical ventilation and circulatory support
  • SURERY - bowel perforation- resction of bowel
200
Q

What are some considerations when using oxygen therapy to correct hypoxaemia

A
  • Keep sats 91-95%
  • High saturations may lead to increased free radicals
  • sats > 95% increased risk of retinopathy of prematurity
  • sats <91% increased risk of NEC and death

Start neonatal resuscitation on 21-30% oxygen in preterms - avoid saturating to 95%. Air should be used in term infants

201
Q

What are some long-term sequelae for NEC

A
  • Stricture development
  • Malabsorption in extensive bowel resection
  • Increased risk of poor neurodevelopmental outcome
202
Q

Define PPHN

A
  • failure of the normal circulatory transition that occurs after birth - ie there is no decrease in pulmonary resistance at birth
  • characterized by marked pulmonary hypertension that causes hypoxemia secondary to right-to-left shunting of blood at the foramen ovale and ductus arteriosus - these dont close as pulmonary resistance still high3
  • Presents as cyanosis at birth, heart murmurs and signs of HF are often absent
203
Q

What are the conditions that are associated with persitent pulmonary hypertension of the newborn

A
  • Birth asphyxiation
  • meconium aspiration
  • Septicaemia
  • RDS
  • Congenital diaphragmatic hernia
204
Q

Investigations for PPHN

A
  • CXR
    • Normal heart size
    • Oligaemia
  • Urgent echocardiogram
    • Exclude congenital heart disease
    • Identifty signs of pulmonary HTN (raised pulmonary pressures and tricuspid regurgitation)
205
Q

Management of PPHN

A
  • Infants require mechanical ventilaion and circulatory support for adequate oxygenation
  • Vasodilators
    • Nitric oxide
    • Sildenafil
  • ECMO in severe bu reversible cases
206
Q

Define transient tachypnoea of the newborn

A
  • MOst common cause of respiratroy distress in term infants
  • Caused by delay in resorption of lung liquid
  • Most common after C-section birth
  • CXR may show fluid in horizontal fissure
  • MAy need ambient oxygen
  • Usually resolves within first day of life but can take several days to resolve completely
  • Diagnosed after consideration and exclusion of other causes (infection)
207
Q

What are some risk factors for meconium aspiration

A
  • Increasing gestational age
    • 20-25% babies delivered by 42 weeks affected
  • Maternal
    • Hx maternal HTN
    • Pre-eclampsia
    • Chorioamnionitis
    • Smoking
    • Subsatnce abuse
208
Q

Pathogenesis of meconium aspiration

A
  • May be response to foetal hypoxia before or at delivery
  • Infant may be gasipng and then aspirating meconium
  • Meconium causes mechanical obstruciton and chemical pneumonitis predisposing infant to infection and respiratory distress
209
Q

Complications of meconium aspiration

A
  • Lobar collapse and consolidation
  • Pneumothorax
  • Pneumomediastinum
  • PPHN -> difficult to achieve adequate oxygenation despite high pressure ventilation
  • Morbidity and mortality
210
Q

What organism causes ophthalmia neonatorum

A
  • Neisseria gonorrhoea
  • Gram negative diplococcus
  • Obligate anaerobe
  • Conjunctivites
211
Q

What is the most common occular malignancy found in children

A

Retinoblastoma

Average age of diagnosis= 18 months

212
Q

Inheritence pattern of retinoblastoma

A

Autosomal dominant

213
Q

Underlying pathophysiology/genetic defect in retinoblastoma

A

Loss of function of retinoblastoma tumour suppressor gene on chromosome 13

10% cases are hereditary

214
Q

What are some possible features of retinoblastoma

A
  • absence of red reflex (replaced by white pupil, leukocoria)
    • Most common presenting symptom
  • Strabismus
    • Unable to align eyes together normally (cross-eyed)
  • Visual problems
  • Fhx of enucleation
215
Q

Management of retinoblastoma

A
  • Enucleation
  • Dependent on how advanced tumour is:
    • External beam radiation therapy
    • Chemotherapy
    • Photocoagulation
216
Q

Prognosis of retinoblastoma

A

Excellent prognosis, >90% surviving into adulthood

217
Q

Causative agent of roseola infantum

A

Human herpes virus 6

Exanthem subitum, sixth disease

218
Q

Incubation period of roseola infantum (HHV6)

A

5-15 days

Affects 6 months to 2 yo

219
Q

Features of roseola infantum

A
  • High fever: lasts a few days followed by a:
  • Maculopapular rash
  • Nagayama spots
    • Papular enanthem on uvula and soft palate
  • Febrile convulsion (10-15%)
  • Diarrhoea and cough
220
Q

What are some possible consequences of HHV6 infection

A
  • Aseptic meningitis
  • Hepatitis
221
Q

True or false: School exclusion is needed for roseola infantum

A

Fals, school exclusion is NOT needed

222
Q

Define croup

A
  • URTI in infants and toddlers
  • Characterised by stridor (due to laryngeal oedema and secretions)
  • Barking cough
223
Q

What is the common causative agents for croup

A

Parainfluenza

224
Q

What ages are susceptible to corup

A

6 months - 3 years

More common in autumn

225
Q

Features of croup

A
  • Stridor
  • Barking cough (worse at night)
  • Fever
  • Coryzal symptoms
226
Q

What are some features of mild croup with reference to:

  • Cough
  • Stridor
  • Work of breathing
  • General appearance
A
  • Occasional barking cough
  • No audible stridor at rest
  • No/mild suprasternal and/or intercostal recession
  • Child is happy and is prepared to eat, drink, and play
227
Q

What are some features of moderate croup with reference to:

  • Cough
  • Stridor
  • Work of breathing
  • General appearance
A
  • Frequent barking cough
  • Audible stridor at rest
  • Suprasternal and sternal wall recessions at rest
  • No or little distress or agitation
  • Child can be placated and is interested in its surroundings
228
Q

What are some features of severe croup with reference to:

  • Cough
  • Stridor
  • Work of breathing
  • General appearance
A
  • Frequent barking cough
  • Prominent inspiratory (and occassionally expiratory) stridor at rest
  • Marked sternal wall recessions
  • Significant distress and agitation, or lethargy or restlessness (sign of hypoxaemia)
  • Tachycardia occurs with more severe obstructive symptoms and hypoxaemia
229
Q

When should you admit a child with croup

A

Admit if child has moderate or severe croup and any of the features below:

  • < 6 months age
  • Known upper airway abnormalities (laryngomalacia, Down’s Syndrome)
  • Uncertainty about diagnosis
    • Epiglotittis, bacterial tracheitis, peritonsillar abscess, foreign body inhalation
230
Q

Investigations for croup

A
  • clinical diagnosis
  • CXR:
    • PA view shows subglottic narrowing -> Steeple sign
    • Lateral view in epiglottitis shows swelling of epiglottis - thumb sign
231
Q

Management of croup

A
  • Single dose oral dexamethasone (0.15mg/kg) regardless of severity
  • Prednisolone is alternative if dexamethasone not available

Emergency treatment:

  • High flow oxygen
  • Nevulised adrenaline
232
Q

Most common pathogen that casues bronchiolitis

A
  • Respiratory Syncytial virus (RSV) 75-80%
  • Other
    • Mycoplasma
    • Adenovirus
233
Q

What age group are commonly affected with bronchiolitis

A

LRTI in <1 year olds

  • 90% 1-9 months old
  • Peak incidence 3-6 motnsh
  • MAternal IgG provides protection to newborns against RSV
  • Higher incidence in winter
234
Q

Features of bronchiolitis

A
  • Coryzal symptoms FIRST, then
  • Dry cough
  • Breathlessness
  • Wheeze, inspiratory crackles
  • Feeding difficulties associated with dyspnoea - > admit
235
Q

What are some features of bronchiolitis that is recommended by NICE for immediate referal

A
  • Apnoea (observed or reported)
  • Child looks unwell
  • Severe respiratory distress
    • Grunting, recession, RR > 70
  • Central cyanosis
  • Persistent O2 sat <92% on air
236
Q

What factors would indicate that a clinicaian considers refering to hospital

A
  • RR >60
  • Difficulty breastfeeding or inadequate oral fluid intake (50-75%)
  • Clinical dehydration
237
Q

Investigation for bronchiolitis

A

Immunofluorescence of nasopharyngeal secretion may show RSV

238
Q

Management of bronchiolitis

A

SUPPORTIVE

  • Humidified O2 via head box if O2 sats <92%
  • Nasogastric feeding ifinadequate feed
  • Suction sometimes for excessive secretions
239
Q

Define microcephaly

A
  • Occipito-frontal circumference <2nd centile
240
Q

Causes of microcephaly

A
  • Normal variant
  • Familal
  • Congenital infection
  • Perinatal brain injury (HIE)
  • Foetal alcohol syndrome
  • Syndromes : Patau
  • Craniosynostosis
241
Q

Define the following:

  • Neonatal death
    • Early
    • Late
  • Puerperal death
  • Prinatal death
  • Miscarriage
A
  • Neonatal death = death 0-28 days of birth
    • Early = within first week of life
    • Late = after 7th day of life but before 28 days
  • Puerperal death = maternal death within puerperal period (6 weeks post birth)
  • Perinatal death = classify deaths as a result of obstetric events (stillbirths)
  • Miscarriage = death in utero before 24 weeks gestation
242
Q

Define cerebral palsy

A
  • Umbrella term for permanent disorder of movement and/or posture and of motor function due to non-progressive abnormality in the developing brain
243
Q

The motor disorders of cerebral palsy are often accompanied by disturbances of?

A
  • Cognition
  • Communication
  • Vision
  • Perception
  • Sensation
  • Behaviour
  • Seizure disorder
  • Secondary musk problems
244
Q

What are some causes of cerebral palsy

A

Antenatal origin (80%)

  • Cerebrovascular haemorrhage
  • Ischaemia
  • Cortical migration disorders
  • Structural meldevelopment of brain
  • Associations with gene deletions, others to genetic syndromes and congenital infection

Intrapartum (10%)

  • Hypoxic-ischaemic injury before or during delivery

Postnatal causes (10%)

  • Meningitis
  • Encephalitis
  • Encephaopathy
  • Head trauma from AI or NAI
  • Symptomatic hypoglycaemia
  • Hydrocephalus
  • Hyperbilirubinaemia
245
Q

What are some early features of cerebral palsy

A
  • Abnormal limb and/or trunk posture and tone
  • Delayed motor milestones
  • Slowed head growth
  • Feeding difficulties, with oromotor incoordination, slow feeding, gagging and vomiting
  • Abnormal gait once walking achieved
  • Asymmetric hand function before 12 months of age
  • Primitive reflexes may persist
246
Q

Diagnosis of cerebral palsy

A
  • Clinical diagnosis
    • Posture
    • Pattern of tone in limbs and trunk
    • Hand function
    • Gait
247
Q

How is cerebral palsy categorised and what are thes

A

Categorised based on neurological features

  • Spastic (90%)
    • Bilateral, unilateral, not otherwise specified
  • Dyskinetic (6%)
  • Ataxic (4%)
  • Other

In the past description was based on parts of body affected (hemiplegia, quadriplegia, diplegia)

248
Q

How is gross motos function level (functional ability) measured in Cerebral palsy

A

Gross motor function classification system (GMFCS)

  • Level I
    • Walks without limitations
  • Level II
    • Walks with limitations
  • Level III
    • Walks using a handheld mobility device
  • Level IV
    • Self-mobility with limitations; may use powered mobility
  • Level V
    • Transported in manual wheelchair
249
Q

Describe some differences in normal and abnormal development that may be seen in cerebral palsy

A
250
Q

What are some early warning/abnormal signs you may see in cerebral palsy

A
  • Maintenance of primitive reflexes
  • Developmental delay
  • Poor feeding/sucking
  • Irritability
  • Hand dominance before 2 years
  • Toe-walking
  • Epilepsey
  • Scissoring of legs
251
Q

Describe the lesion and some features of spastic cerebral palsy

A
  • Upper motor neurone (pyramidal or coticospinal tract) damage - cortical damage
    • GABA transmission reduced so nerves are over-excited, leading to hypertonia
      • ​Scissor gait (adductor muscles partly flexed)
      • Toe-walk (calves flexed)
  • Really tight or stiff muscles - jerky movements
  • Hemiplegia - unilateral, upper >> lower limb
  • Quadraplegia - all four limbs, upper >> lower limb, most diabling form. Central hypotonia, poor head control
  • Diplegia - all four limbs affected, lower >> upper limbs. Assocaited with prematuriy (periventricular leukoplakia and intraventricular hamorrhage). Cognition often normal (lesion in white matter, so grey matter spared)
252
Q

Describe the lesion and some features of dyskinetic (athetoid) cerebral palsy

A
  • Damage or injury to basal ganglia
    • Helps initiate and prevent movements
    • Damage leads to loss of ability to prevent movements
  • Involuntary movements
  • Dystonia(random, slow, uncontrolled movements in limb and trunk)
  • +/- chorea (dance like movements as small uncontrolled movments seem to move from muscle to muscle
253
Q

Describe the lesion and some features of ataxic cerebral palsy

A
  • Damage to cerebellum
    • Coordination, fine movements
  • Clumsy or unstable movements
  • Poor balance
254
Q

Management of cerbral palsy

A
  • MDT approach
    • Neurologist
    • Rehabilitation specialist
    • OT/PT
    • SALT
  • PT
    • Build strength and improve walking ability
  • Muscle relaxants
    • Botulinum toxin may be injected for hypertonicity
      • Helps with pain associated with hypertonus
  • Surgery
    • Loosening tight muscles
    • Straightening out bones
255
Q

Define hypoxic-ischaemic encephalopathy (HIE)

A
  • Hypoxic-ischaemic injury to organs (braing) due to compromised placental or pulmonary gas exchange
  • Cardiorespiratory depression occurs leading to hypoxia, hypercapnia, metabolic acidosis
  • Leads compromised cardiac output and hypoxic-ischaemic injury
256
Q

What are some causes/risk factors for HIE

A
  • Reduced gas exchange at placenta
    • Excessive/prolonged uterine contractions, placental abruption, ruptured uterus
  • Interrupte umbilical blood flow
    • Cord compression (shoulder dystocia, cord prolapse)
  • Maternal factors
    • Decreased placental perfusion, maternal hypotension or hyeprtension
  • Foetal factors
    • IUGR, anaemia
  • Cardiorespiratory adaptation at birth
    • Failure to breathe
257
Q

When do the clinical manifestations of HIE usually occur

A

Immediately or upt to 48 hours after asphyxia

258
Q

Pathophysiology of HIE

A
  • Failed oxygenation across placenta, umbilicus
  • Cardiorespiratory depression occurs leading to hypoxia, hypercapnia, metabolic acidosis
  • Leads compromised cardiac output and hypoxic-ischaemic injury
  • Neuronal damage may be immediate from primary neuronal death OR
  • delayed damage from reperfusion injury (ie there is a primary energy failure and secondary energy fialure)
  • This window of delay between the two injuries offer opportunity to treat
259
Q

What are some effects on end-organs of HIE

A

Encephalopathy

  • Abnormal neurological signs
  • Seizures

Respiratory failure

  • Persistent pulmonary hypertension of the newborn

Myocardial dysfiunction

  • Hypotension

Metabolic

  • HypOglycaemia
  • HypOcalcaemia
  • HypOnatraemia

Other

  • Renal failure
  • DIC
260
Q

How is HIE graded

A
  • Mild
    • Irritable, responds excessively to stimulation, staring of eyes, hyperventilation, hypertonia, imparied feeding
  • Moderate
    • marked abnormal movements, hypotonic, cannot feed, may have seizures
  • Severe
    • No normal spontatneous movements or response to pain, tone in limbs fluctuate between hypotonia and hypertonia, seizures are prolonged and refractory to treatment, multi-organ failure present
261
Q

How is HIE managed

A
  • Nueroprotection
    • Therapeutic hypothermia (33-35)
  • Monitor
    • aEEG - detect abnormal brain activity and seizures
  • Organ support
    • Respiratory
    • AED
    • Fluid restriction due to transient renal impariemtn
    • Treatment of hypotension by volume and inotrope support
    • Monitor metabolic problems
      • low glucose
      • low Na
      • low Ca
262
Q

Define hydrocephalus

A
  • Accumulation of CSF in the brain
  • Can be congenital, associated with cerebral malformations or obstruction to the flow of CSF leading to dilatation of ventricular system proximal to site of obstruction
263
Q

Name and describe the two types of hydrocephalus (lesions)

A
  • Communicating
    • Obstruction at the arachnoid vili (where CSF absorbed)
  • Non-communicating
    • Obstruction within ventricular system or aqueduct
264
Q

What are some causes of hydrocephalus

A

Non-communicating (obstruction to venticula system)

  • Congenital malformation
    • Aqueduct stenosis
    • Atresia of ouflow foramina of 4th ventricle
    • Chiari malformation (cerebellar tonsils herniation through foramen magnumg)
  • Posterior fossa neoplasm or vascular malformation
  • Intraventricular haemorrhage in preterm infant

Comomunicating (failure to reabsorb CSF)

  • Subarachnoid haemorrhage
  • Meningitis (pneumococcal, tuberculous)
265
Q

Clinical features of hydrocephalus

A
  • Disproportionately large head
    • Skull sutures have not yet fused
  • Sun setting of eyes (fixed downward gaze)
    • advanced sign
  • Signs and symptoms of raised ICP
    • Older children
266
Q

Diagnosis of hydrocephalus

A
  • Can be diagnosed on antenatal US screening or in preterm infants on routine cranial USS
  • Suspected hydrocephalus
    • 1st = cranial USS (in infants) OR MRI
      • Head circumference measured and monitored and plotted oer time
267
Q

Aim of hydrocephalus managmeent

A
  • Symptomatic relief of raised ICP and minimise risk of neurological damage
268
Q

Management of hydrocephalus

A
  • Insertion of ventriculoperitoneal shunt
    • sometimes endoscopic treatment to create ventriculostomy
269
Q

Most common cause of thrombocytopenia in childhood

A

Immune thrombocytopenia (ITP)

incidence 4 per 100,000 children per year

270
Q

Pathophysiology of ITP

A
  • Destruction of circulating platelets by antiplatelet IgG
  • May be some megakaryocytes in bone marrow as compensatory response
271
Q

Clinical features of ITP in children

A
  • Present between 2-10yo
  • Onset 1-2 weeks post viral infection
  • Features:
    • Petechiae
    • Pupura
    • Superficial bleeding
272
Q

What are some complications of ITP in children

A
  • Epistaxis
    • And other mucosal bleeding
    • Profuse bleeding uncommon
  • Intracrainial bleed
    • Serious but rare
273
Q

Differential diagnosis for ITP in children

A
  • Wiskott-Aldrich
  • Bernard Soulier
  • Acute leukaemia
  • Aplastic anaemia
274
Q

Diagnosis of ITP in children

A
  • Diagnosis of exclusion

Careful attention to:

  • History
  • clinical features
  • Blood film
  • Examination
    • Anaemia, neutropenia
    • Hepato-splenomegaly
    • Lymphadenopathy
  • Bone marrow examination if child is going to be treated with steroids
275
Q

Management of ITP in children

A
  • 80% is acute, benign and self-limiting, resoplving in 6-8 weeks
    • Manage at home
  • Do not need treatment even if their platlet count is <10x109/L but treatment should be given if there is evidence of major bleeding or persistent minor bleeds that affect QoL
    • Oral prednisolone
    • IV anti-D
    • IVIG
  • Pletlet transfusions in life-threatening - needs admission
  • Avoid trauma (contact sport) whilst platelet counts low
276
Q

What is chronic ITP

A
  • When platlent count remains low 6 months after diagnosis
  • 20%
277
Q

Management of chronic ITP

A
  • Supportive
  • Screen for SLE if chronic ITP
278
Q

What is the most common malignancy affecting children

A

Acute Lymphoblastic leukaemia (ALL)

80% of childhood leukaemias

279
Q

Peak incidence and boys or girsl more affected in ALL

A
  • 2-5 years
  • Boys >> girls
280
Q

Features of ALL

A
  • Bone marrow failure
    • Anaemia - tired and pallor
    • Neutropenia - frequent and severe infections
    • Thrombocytopenia -easy bruising and petechiae
  • Bone pain (bone infiltration)
  • hepatosplenomegaly (extra-medullary haematopoiesis)
  • Fever in 50% new cases
  • testicular swelling
281
Q

Types of ALL in children

A
  • Common ALL (75%)
    • CD10+ve, pre-B phenotype
  • T cell ALL (20%)
  • B cell ALL (5%)
282
Q

Poor prognostic factors of ALL

A
  • <2 years or >10 years
  • WBC > 20x109/L at diagnosis
  • T or B cell surface markers
  • Non-caucasian
  • Male
283
Q

Pathophysiology of retinopathy of prematurity

A
  • Affects dvelolping blood vessels at the junction of the vascularise and non-vascularised retina
  • Vascular proliferation, which may progress to retinal detachment, fibrosis and blindness
284
Q

Cause of retinopathy of prematurity

A
  • Excessive oxygen use
  • Prematurity
285
Q

How is retiopathy of prematurity managed

A
  • Screen susceptible preterm infants (≤1500g birthweight or <32 weeks gestation)
  • Laser therapy
  • Intravitreal anti-VEGF (being investigated)
286
Q

What are some risk factors for haemorrhages/intraventricular haemorrhages

A
  • Very low birthweight infants (<1500g)
  • Perinatal asphyxia
  • RDS
  • Pneumothorax
287
Q

How is an intraventricular haemorrhage identified

A

Cranial ultrasound scans

288
Q

Difference between a small and large intraventricular haemorrhage

A
  • Small = confined to germinal matrix
  • Large = extends to ventricles
289
Q

What are some complications of a large Intraventricular haemorrhage

A
  • Ventricular dilatation
  • Hydrocephalus
    • separate sutures, tense fontanelle
    • May need ventriculoperitoneal shunt, or remove CSF by LP or ventricular tap
  • Cerebral palsy
  • Periventricular white matter brain injury
    • Periventricular leukomalacia (bilateral loss of white matter)
290
Q

What would you see on imaging (US) of periventricular leukomalacia and what does this signify

A
  • Cystic lesions, bilateral periventricular
  • Loss of white matter - spastic diplegia, with cognitive impairment
291
Q

What is the major route of transmission of HIV in children? What are the other routes of transmission

A
  • Mother-to-child transmission
    • Intrauterine (pregnancy)
    • Intrapartum (delivery)
    • Breastfeeding (post-partum)
  • Infected blood products
  • Contaminated needles
  • Sexual abuse
292
Q

Children less than _____ months of age who are born to infected mothers will have transplacental maternal _____ (3 words,); therefore, at this age a positive antibody test confirms HIV _____ but not HIV _____.

A

Children less than 18 months of age who are born to infected mothers will have transplacental maternal IgG HIV antibodies; therefore, at this age a positive antibody test confirms HIV exposure but not HIV infection.

293
Q

How is HIV diagnosed in children

A

>18 months

  • HIV antibody detection

<18 months

  • HIV DNA PCR
    • All infants born to HIV-infected mothers should be tested for HIV whether or not they are symptomatic
294
Q

What would suggest an infant is not infected in terms of how HIV is diagnosed

A
  • Two negative HIV DNA PCR within first 3 months of life, at least 2 weeks after postnatal ART (antiretroviral therapy)
  • Confirmed by the loss of maternal HIV antibodies from infant’s circulation after 18 months age
295
Q

Describe two phenotypes of children who have been infected with HIV in terms of disease actrivity

A
  • Rapid progression and onset of AIDS in the 1st year of life
  • Asymptomatic for months or years before progressing to clinical disease
    • Some asymptomatic children are onl;y identified in adolescence at routine screening following diagnosis of another family member
296
Q

Clinical presentation of HIV in children varies with degree of immunocompromise. Describe some features of a) mild and b) moderate phenotypes

A

Mild

  • Lymphadenopathy
  • Parotic enlargement

Moderate

  • Recurrent bacterial infections
  • Candidiasis
  • Chronic diarrhoea
  • Lymphocytic interstitial pneumonitis
    • This can look like TB, but its not
    • May be cause by response to HIV infection or related to EBV infection
297
Q

Clinical presentation of HIV in children varies with degree of immunocompromise. Describe some features of severe AIDS diagnoses

A
  • Opportunistic infections
    • PCP
  • Severe growth faltering
  • Encephalopathy
  • Malignancy
    • Rare
298
Q

Management of HIV in children

A
  • Antiretrovirals (ART)
    • 3 or 4 drugs
  • Prophylaxis
    • PCP - cotrimoxazole
      • infants with HIV or older children with low CD4 counts
  • Immunisation
    • Consider influenza, Hep A/B, VZV
    • Do NOT BCG
  • MDT
    • see with family
    • Compliance
    • Planning for future (sex, fertility, pregnancy)
    • Psychological support
299
Q

The decision to start ART in HIV is based on what factors

A
  • Clinical status
  • Viral load
  • CD4 count

Infants should always start ART after diagnosis as they have a higher risk of disease progression

300
Q

What are some factors to help reduce the vertical transmission of HIV

A
  • Maternal ART during prengnacy and intrapartum -achieve undetectable viral load
  • PeP (post exposure) to infant after delivery
  • Avoid breastfeeding
  • Active management of labour and delivery
    • Avoid prolonged ROM and unecessary instrumentation (forceps)
  • Prelabour c-section
    • if maternal viral load is detectable close to expected date

Mothers who are not taking ART and who breastfeed transmit HIV in 25-40% of infants

301
Q

Incidence of pneumonia peaks in infance and old age, but is relatively high in childhood. Major cause of mortality in low and middle income countries. What are some causes of pneumonia in a newborn

A
  • E coli
  • Listeria monocytogenes
  • Group B Streptococcus
302
Q

What are some causes of pneumonia in infants and young children

A
  • RSV
  • Chalmydia trachomitis
  • Streptococcus pneumoniae
  • Haemophilus influenzae
  • Bordatella pertussis
  • Staphylococcus aureus
    • Infrequent but serious
303
Q

What are some causes of pneumonia in children over 5 years of age

A
  • Mycoplasma pneumoniae
  • Streptococcus pneumoniae
  • Chlamydia pneumoniae
304
Q

What are the symptoms of pneumonia in a child

A
  • Fever
  • Cough
  • Rapid breathing
  • Preceded by URTI
  • Lethargy
  • Poor feedgin
  • Unwell child
  • Localised chest, abdo or neck pain
    • Features of pleural irritation -> bacterial infection

Consider pneuimonia in children with neck stiffness or acute abdominal pain

305
Q

What are some signs on examination in a child with pneumonia

A
  • Tachypnoea
    • Very sensitive to pneumonia (unlike in asthma)
  • Nasal flaring and chest indrawing/recession (work of breathing)
  • Decreased oxygen saturations
  • Maybe some end-inspiratory crackles
306
Q

What are some investigations for a child with pneumoina

A
  • CXR to confirm diagnosis
    • Cannot reliably differentiate between viral and bacterial pneumonia
  • Blood cultures, urine cultures, LP (in young)
  • ​Nasopharyngeal aspirate in younger children
    • identify viral causes
  • Blood tests are generally unhelpful in differentiating viral or bacterial cause
307
Q

Most affected children (pnumonia) can be managed at home. What are some indications for admission

A
  • Oxygen saturation <92%
  • Recurrent apnoea
  • Grunting
  • Inability to maintain adequate fluid/feed intake
308
Q

Management of pneumoniaa in a child

A

Choice of antibiotic dependent on childs age and severity of illness:

  • Newborns - IV broad-spectrum
  • Older infants - oral amoxicillin
    • Complicated/unresponsive pneumonia - co-amoxiclav
  • >5 yo - amoxicillin or oral macrolide (erythromycin)
309
Q

Prognosis of pnuemonia in a child

A
  • Full recovery
  • Follow up not generally needed
  • Those with lobar collapse should have repeat CXR in 4-6 weeks to check normal lung fields
310
Q

What are some causes of acute onset limb pain

A
  • Osteomyelitis
  • Bone tumours
  • Septic arthritis
311
Q

Where is the most common site of infection in osteomyelitis

A
  • Distal femur and proximal tibia, but any bone may be affected

Infection of the metaphysis of long bones

312
Q

Spread of osteomyelitis in children

A
  • Haematogenous
  • But may be direct spread from wound
313
Q

Causative organisms of osteomyelitis in children, those with sickle cell anaemia and immunodeficient

A
  • Staphylococcus aureus
  • Streptococcus
  • Haemophilus influenzae (if not imunised)

Children with sickle cell anaemia

  • Staphylococcus
  • Salmonella

Immunodeficient

  • TB
314
Q

How does osteomyelitis present

A
  • Painful and immobile limb (pseudoparesis)
  • Febrile
  • Swelling and tenderness over infected site (erythematous and warm)
315
Q

Investigations for osteomyelitis in children

A
  • Blood cultures
    • Usually positive (due to haematogenous spread)
  • WCC and CRP raised
  • X-ray
  • Ultrasound
  • MRI
  • Radionuclide bone scan if site of infection uncleear
316
Q

Management of osteomyelitis

A
  • Parenteral (IV) antibiotics immediately for several weeks
  • Aspiration or surgical decopmression os subperiosteal spave
    • Atypical presentation or immunodeficient child
  • Surgical drainage
    • Unresponsive to antiboiotic therapy
  • Limb rest in splint

Long antibiotic therapy as poor penetration in joint infection

317
Q

Complications of osteomyelitis

A
  • Bone necrosis
  • Chronic infection
  • Limb deformity
  • Amyloidosis
318
Q

Define retinoblastoma

A
  • Malignant tumour of retinal cells
  • Rare, 5% of severe visual impariment in children
  • Affects one or both eye
319
Q

Retinoblastoma may affect one or both eyes. All bilateral tumours are _____, as are about 20% of unilateral cases. The retinoblastoma susceptibility gene is on chromosome _____, and the pattern of inheritance is autosomal _____, but with incomplete penetrance. Most children present within the first 3 years of life. Children from families with the hereditary form of the disease should be screened regularly from birth.

A

Retinoblastoma may affect one or both eyes. All bilateral tumours are hereditary, as are about 20% of unilateral cases. The retinoblastoma susceptibility gene is on chromosome 13, and the pattern of inheritance is autosomal dominant, but with incomplete penetrance. Most children present within the first 3 years of life. Children from families with the hereditary form of the disease should be screened regularly from birth.

320
Q

What is the most common presentation of retinoblastoma

A
  • White pupillary reflex
  • Squint
321
Q

Investigations for retinoblastoma

A
  • MRI and examination under anaesthetic
  • Tumours are frequently multifocal
322
Q

Treatment/Management of retinoblastoma

A
  • Aim is to cure and preserve vision
  • Management based on opthalmological findings
  • Enucleation of eye
    • Advanced disease
  • Chemotherapy and laser therapy
    • Bilateral disease, chemo to shrink tumours
  • Radiotherapy
    • Advanced disease
    • Reserved for treatment of recurrence
323
Q

Prognosis of retinoblastoma

A
  • Most patients are cured but may be visually imparied
  • Significatn risk of second malignancy among survivors of hereditary retinoblatoma
324
Q

In someone treated with retinoblastoma, what second malignancy has an increased risk of occuringh

A

Sarcoma

325
Q

Clinical Features of malrotation

A
  • Presents fiurst 1-3 dyas of life with obstruction from Ladd bands obstructing the duodenum or volvulos
  • May present at any age with volvulus causing obstruction and ischaemic bowel

Features

  • Billous vomiting
  • Abdominal pain
  • Peritonitis or ischaemic bowel tenderness
326
Q

Investigation for malrotation with billous vomiting

A
  • Upper GI contrast study to assess intestinal rotation
327
Q

Management of malrotation

A
  • Surgical emergency
  • Surgicla correction
328
Q

At what age is considered to be premature sexual development

A

Development of secondary sexual characteristics

  • <8 in females
  • <9 in males
329
Q

What are the four recognised patterns of premature sexual development

A
  • Precocious puberty
  • Thelarche
  • Adrenarche
  • Isolated premature menarche
330
Q

Precocious puberty is categorised according to the levels of the pituitary-derived gonadotrophins, FSH and LH. What are the two categories

A
  • Gonadotrophin dependent - Central or True precosious puberty
    • Premature activation of hypothalamic-pit-gonadal axis (HPG axis).
    • Pubertal development is normal = consonant
  • Gonadotrophin independent - Pseudo or False precocious puberty
    • Excess sex steroids outside of pituitary gland
    • Pubertal development is abnormal = dissonant
331
Q

Female Precocious puberty

The _____ are very sensitive to secretion of gonadotrophins from the pituitary gland, so gonadotrophin-_____ precocious puberty is fairly common in girls. It is usually _____ or familial. Pathological causes of precocious puberty in girls are rare and can be secondary to either:

  • gonadotrophin-independent causes such as excess androgens from _____ _____ _____ (one condition) or _____ tumours, presenting with pubic and axillary hair, adult body odour, acne, and virilization of the genitalia before breast development
  • gonadotrophin-dependent causes such as pituitary _____, where pubertal development will be _____, but perhaps rapid.

_____ examination of the ovaries and uterus
is helpful in assessing the progress of puberty. The uterus will change from an infantile ‘_____’ shape to ‘_____’ shape with the progression of puberty and the endometrial lining can be identified close to menarche.

A

The ovaries are very sensitive to secretion of gonadotrophins from the pituitary gland, so gonadotrophin-dependent precocious puberty is fairly common in girls. It is usually idiopathic or familial. Pathological causes of precocious puberty in girls are rare and can be secondary to either:

  • gonadotrophin-independent causes such as excess androgens from congenital adrenal hyperplasia or adrenal tumours, presenting with pubic and axillary hair, adult body odour, acne, and virilization of the genitalia before breast development
  • gonadotrophin-dependent causes such as pituitary adenoma, where pubertal development will be consonant, but perhaps rapid.

Ultrasound examination of the ovaries and uterus
is helpful in assessing the progress of puberty. The uterus will change from an infantile ‘tubular’ shape to ‘pear’ shape with the progression of puberty and the endometrial lining can be identified close to menarche.

332
Q

Male precocious puberty

The testes are relatively _____ to secretion
of gonadotrophins from the pituitary gland, so
gonadotrophin-dependent PP is _____ in boys. It is important to exclude a pathological
cause. Examination of the _____ may be helpful:

  • bilateral _____ of the testes, with testicular volumes greater than _____ mL, suggests gonadotrophin-_____ PP. This can be caused by an _____ _____ and rarely by secretion of _____ from a liver tumour
  • _____ testes suggest a gonadotrophin _____ cause, e.g. adrenal pathology such as a _____ or _____ _____ _____
  • a unilateral enlarged testis suggests a _____ tumour.

Tumours in the hypothalamic region are best investigated by cranial _____ scan.

A

The testes are relatively insensitive to secretion
of gonadotrophins from the pituitary gland, so
gonadotrophin-dependent PP is uncommon in boys. It is important to exclude a pathological
cause. Examination of the testes may be helpful:

  • bilateral enlargement of the testes, with testicular volumes greater than 4 mL, suggests gonadotrophin-dependent PP. This can be caused by an intracranial tumour and rarely by secretion of B-HCG from a liver tumour
  • prepubertal testes suggest a gonadotrophin independent cause, e.g. adrenal pathology such as a tumour or congenital adrenal hyperplasia
  • a unilateral enlarged testis suggests a gonadal tumour.

Tumours in the hypothalamic region are best investigated by cranial MRI scan.

333
Q

Biochemical abnormalities in gonadotrophin-dependent precocious puberty

A
  • LH ++
  • FSH +
  • Oestrogen from ovary ++
  • Testosterone from:
    • Testes ++
    • Adrenals +
334
Q

Biochemical abnormalities in gonadotrophin-independent precocious puberty

A
  • Low FSH Low LH
335
Q

Causes of gonadotrophin-dependent precosious puberty

A
  • Idiopathic/familial
  • CNS abnormalities
    • Hydrocephalus
    • Acquired (post-irradiation, infection, surgery, brain injury)
    • Tumours
  • Hypothyroidism
336
Q

Causes of gonadotrophin-independent precocious puberty

A
  • Adrenal disroders
    • Tumours
    • congenital adrenal hyperplasia
      • 21 hydroxylase deficiency - salt losing crisis (low aldo, low cortisol, high sex steroids)
  • Ovarian tumour (granulosa cell)
  • Testicular tumour (Leydig cell)
  • Exogenous sex steroids
337
Q

What are the management aims of precocious puberty

A
  • Detection and treatment of underlying pathology
    • MRI for intracranial tumours (males)
  • Reducing rate of skeletal maturation
    • Assess bone age
    • Early growth spurt may result in early cessation of growth and reduction in adult height
  • Address psychological/behavioural difficulties
338
Q

Define delayed puberty

A

Absence of pubertal development by 14 in females and 15 in males

339
Q

In contrast to precocious puberty, delayed puberty is more common in _____ due to relative insensitivity of the _____ to gonadotrophin secretion. Most commonly, this is _____ delay in growth and puberty, often with a _____ history of delayed puberty. It is a variation of the normal _____ of puberty rather than a pathological condition. It may also be induced by dieting or excessive physical training. An affected child will have delayed sexual changes compared with his/her peers, and bone age would show moderate
delay. The legs will be long in comparison to the back. Eventually, the target height will be reached as growth in affected children will continue for longer than in their peers. The condition may cause psychological upset from teasing, poor self-esteem, and disadvantage in
competitive sport.

In boys, assessment includes:
• pubertal staging, especially _____ volume
• identification of long-term systemic disorders.

A

In contrast to precocious puberty, delayed puberty is more common in males due to relative insensitivity of the testes to gonadotrophin secretion. Most commonly, this is constitutional delay in growth and puberty, often with a family history of delayed puberty. It is a variation of the normal timing of puberty rather than a pathological condition. It may also be induced by dieting or excessive physical training. An affected child will have delayed sexual changes compared with his/her peers, and bone age would show moderate delay. The legs will be long in comparison to the back. Eventually, the target height will be reached as growth in affected children will continue for longer than in their peers. The condition may cause psychological upset from teasing, poor self-esteem, and disadvantage in
competitive sport.

In boys, assessment includes:
• pubertal staging, especially testicular volume
• identification of long-term systemic disorders.

340
Q

What is the most common cause of delayed puberty

A

Constitutional delay of growth and puberty (familial)

341
Q

What are the two categories for the causes of delayed puberty

A
  • Low gonadotrophin secretion (hypogonadotrophin hypogonadism)
  • High gonadotrophin secretion (hypergonadotrophin hypogonadism)
342
Q

Managament of delayed puberty

A

Boys

  • Treatment not usually required
  • If needed:
    • Young = oral oxandrolone
      • Catch-up growth - NO secondary characteristics
    • Older = IM testosterone
      • Accelerate growth + secondary characteristics

Girlks

  • Delayed puberty less common so organic cause should be excluded (due to sensitivity of ovaries to pit GnT)
  • Karyotyping
  • TFTs
  • Sex steroid hormone measurement
  • If needed: oestradiol to induce puberty
343
Q
A

PassMedicine (6 decks)

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