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Phase 3a > Paediatrics > Flashcards

Paediatrics Flashcards

1
Q

What is the difference between the median age and limit age?

A

median age is the age when half the standard population of children have reached a milestone

Limit age is the max-age a child should have achieved a milestone by

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2
Q

2 methods of screening hearing in newborns?

A

Evoked Otoacoustic Emission

Automated Auditory Brainstem Response (EEG)

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3
Q

Moro reflex?

A

sudden extension of the neck causes symmetrical extension then flexion of the arms

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4
Q

Recoil reflex?

A

Flexion then sudden extension causes instant flexion again

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5
Q

Grasp reflex?

A

When an object is placed in their hand they flex fingers

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6
Q

Rooting reflex?

A

Head turns to stimulus when touched near the mouth

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7
Q

Asymmetrical tonic neck reflex?

A

Lying supine, infant adopts an outstretched arm to the side which the head is turned

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8
Q

Gallant reflex?

A

when suspended face down, rub the side on the back and the lower extremity should move to that side

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9
Q

Dolls eye reflex?

A

When head is turned, the eyes move in the opposite direction?

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10
Q

Labarythine righting?

A

When body is tilted the head moves in the opposite direction

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11
Q

Postural support?

A

When held upright, legs take weight and push up (Bounce)

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12
Q

Lateral propping?

A

When sitting, the arm extends on the side the child is falling

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13
Q

Parachute reflex?

A

when suspended face down, the arms extend as though to save themselves

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14
Q

Landau reflex?

A

when suspended in prone, when head comes up so do the legs and when head comes down so do the legs

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15
Q

Cerebral palsy?

A

Non-progressive movement and posture disorder caused by brain injury in the first 2 years of life

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16
Q

Types of cerebral palsy and location of damage?

A

Spastic - UMN pyramidal and corticospinal tracts

Dyskinetic - Basal ganglia

Ataxic (Hypotonic) - cerebellum

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17
Q

2 main causes of cerebral palsy?

A

Hypoxic-ischaemic brain injury
Hyperbilirubinaemia

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18
Q

How would a patient with a hemiplegic cerebral palsy present?

A

Unilateral spasticity + brisk reflexes
Face sparing
Fisted hand, Flexed arm + Asymmetrical reaching out between 4 to 12 months

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19
Q

Opisthontos?

A

Spasm of muscle of head, neck, back + spine causing poor head control

Quadriplegic spastic cerebral palsy

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20
Q

Risk factors for autism?

A

Affected sibling
Gestational age <35 weeks
Parental schizophrenia
Birth defect affecting nervous system
LD
Downs
Male

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21
Q

Examples of AD?

A

Ehlers-Danlos syndrome
Familial Hypercholesterolaemia
Familial adenomatous polyposis
Huntington’s disease
Marfan
Osteogenesis Imperfecta
Neurofibromatosis
Noonan syndrome

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22
Q

Examples of AR?

A

Congenital adrenal hyperplasia
Cystic fibrosis
Friedreich Ataxia
Sickle cell
Thalassaemia

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23
Q

What is the difference between penetrance and expressivity?

A

The proportion of people carrying an allele that also exhibit clinical signs

Expressivity - the difference observed in the clinical phenotype between 2 individuals with the same genotype

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24
Q

Anticipation?

A

When the disease presents at an earlier age and more severe in successive generations

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25
Q

What is uniparental disomy and examples of such conditions?

A

When a child inherits 2 copies of a chromosome from 1 parent and non from the other

Angelmann + Prader Willi syndrome

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26
Q

Robertsonian translocation in Downs occurs between which 2 chromosomes?

A

14 + 21

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27
Q

Ways that trisomy 21 occur?

A

Mosaisim
Meiotic non-disjunction
Robertsonian translocaiton between chromosome 14 + 21

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28
Q

Craniofacial features of Down’s syndrome?

A

Flat occiput
Flat nose bridge
Up slanted palpebral fissue
Small mouth
Protruding tongue
Small ears

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29
Q

Non-craniofacial features of Down’s syndrome?

A

Wide sandal grip
Incurved fifth finger
Hypotonia
Short neck

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30
Q

What is atlantoaxial instability?

A

fewer stable bones of the upper neck + base of skull so more at risk of nerve damage

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31
Q

When is the scan done to detect Down, Edwards + Pataeu and what factors are considered?

A

10 to 14 week scan

Nuchal Translucency
Beta hCG
PAPPA

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32
Q

More specific diagnostic investigation of Down’s syndrome?

A

Amniocentesis + Karyotyping

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33
Q

Features of Edwards syndrome?

A

Prominent occiput
Small mouth and chin
Short sternum
Flexed overlapping fingers
Rocker bottom feet (congenital vertical talus)
Cardiac + renal malformations

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34
Q

Features of Patau syndrome?

A

Structural defects of brain
Scalp defects
Small eyes
Cleft lip + palate
Polydactyly
Cardiac + renal malformations

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35
Q

Klinefelter syndrome?

A

47XXY

Hypogonadism, reduced fertility + being taller than expected, Gynaecomastia

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36
Q

Investigations of Klinefelters syndrome?

A

Karyotyping, Testosterone, Sperm Count

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37
Q

Most common cause familial learning difficulties?

A

Fragile X syndrome

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38
Q

Fragile X syndrome?

A

Gap/break in the distal part of the long arm of the X chromosome or >200 trinucleotide repeats

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39
Q

Trinucleotide repeat seen in fragile X syndrome?

A

>200 CGG

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40
Q

Turners syndrome?

A

45X0

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41
Q

Clinical features of turners syndrome?

A

Short stature, Premature ovarian failure, Wide space nipples, Neck webbing, Recurrent otitis media

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42
Q

Neonatal signs of turners syndrome?

A

Lymphoedema of the neck, hands or feet

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43
Q

Management of turners syndrome?

A

GH
Oestrogen replacement to develop secondary sex characteristics

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44
Q

What is the difference between Duchene + Becker’s muscular dystrophy?

A

Duchese in no dystrophyin protein + Becker is misshapen dystrophin making it less severe

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45
Q

Mode of the inheritance of muscular dystrophy?

A

X linked recessive deletion/duplication on the gene that codes for dystrophin protein

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46
Q

Clinical features of muscular dystrophy?

A

Delayed walking
Waddling gait
Calf pseudohypertrophy
Gower’s sign

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47
Q

Complications of muscular dystrophy?

A

Wheelchair-bound
Resp failure
Scoliosis
Dilated cardiomyopathy
Arrhythmia

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48
Q

Diagnosis of muscular dystrophy?

A

High Creatine Kinase
Dystrophin mutation (DNA or western blot)
Muscle biopsy + stain for dystrophin

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49
Q

What causes calf pseudohypertrophy?

A

Cell death of the muscle due to lack of dystrophin
Short term regeneration of muscle fibres of different sizes + long term muscle atrophy
Fat + fibrotic tissue instead of muscle

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50
Q

Classification of mobility in cerebral palsy?

A

Gross motor function classification system

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51
Q

What chromosome is mutated in the conditions Angelmanns syndrome and Prader Willi?

A

Chromosome 15

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52
Q

Clinical features of Angelmann syndrome?

A

Happy puppet
Inappropriate laughter
Unstable ataxic gait
Jerky movements
Microencephaly
Decreased need for sleep
Feeding difficulties

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53
Q

Clinical features of Prader Willi syndrome?

A

Hypotonia + poor feeding in infancy
Overeating, hypogonadism + LD in childhood

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54
Q

Noonan syndrome?
4 HIPA 4

A

4 Major: Short stature, Triangular face, Neck webbing Pectus excavum

Hypertrophic Cardiomyopathy, Infundibular pulmonic stenosis, Pulmonary hypertension, ASD

4 minor: Bleeding disorder, Downward slanting of palpebral fissures, Squint, AD

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55
Q

Syndrome associated with transient neonatal hypercalcaemia?

A

William syndrome

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56
Q

Mechanism of mutation of Williams syndrome?

A

Spontaneous deletion of 26 to 28 genes on chromosome 7

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57
Q

Clinical features of Williams syndrome?

A

Developmental delay
Growth delay
full cheeks
Large mouth usually held open
Broad nasal bridge with nostrils flaring forward
Underdeveloped teeth

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58
Q

Examples of genetic conditions that exhibit anticipation?

A

Myotonic dystrophy
Huntington’s

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59
Q

Mode of inheritance of Haemophilia A?

A

X linked recessive
No father to son transmission
Daughter will be a carrier

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60
Q

Examples of X linked recessive conditions?

A

Haemophilia A
G6PD deficiency

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61
Q

In what situation can a female patient be affected by an X linked recessive mutation?

A

Turners syndrome 45X0

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62
Q

X linked Recessive?

A

Inherited from heterozygous mothers
No male to male transmission
Assuming Het mother + normal father - all male have 50% of being affected and all females have a 50% of being carriers

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63
Q

When a condition is only passed on by females but when it affects a son, they can no longer pass on the mutation. what kind of inheritance is described?

A

Mitochondrial inheritance

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64
Q

CF mode of transmission?

A

AR deletion ٨F508 resulting in a misfolded Cystic Fibrosis Transmembrane Conductdance Regulator

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65
Q

What causes the negative transepithelial potential difference seen in cystic fibrosis?

A

CFTR gene not effective is mucus producing cells so increased intracellular Chloride ions
Compensatory influx of sodium causes a overall native charge on the outside of the membrane

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66
Q

What condition is seen a new born with CF?

A

Meconium ileus (Gastrografin)

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67
Q

CF effects on the pancreas?

A

Avitaminosis A - squamous metaplasia of epithelial lining of pancreatic exocrine ducts - digests it;self resulting in pancreatitis + insulin dependant DM

Steatorrhoea

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68
Q

The causative organism of recurrent pneumonia in children with CF?

A

Staphy A

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69
Q

The causative organism of recurrent pneumonia in teens/adults with CF?

A

Pseduomonas Aruginosa

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70
Q

What organism can cause a CF exacerbation that can lead to resp failure + death?

A

Aspergillus fumigatus

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71
Q

CF effects on the liver?

A 
Small billiary duct obstruction 
Biliary Cirrhosis (Stellate cells)
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72
Q

Metabolic complications of CF?

A

Hypokalaemia
Contraction Alkalosis - Alkalosis associated with hypovolaemia

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73
Q

How does CF cause subfertility/Infertility in adults?

A

Femlae - cervical mucus too thick for sperm to penetrate
Male - Absent vas deferens

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74
Q

4 ways of diagnosing CF?

A

Heel prick - Immunoreacitve trypsinogen
Nasal negative transepithelial potential difference
DNA CFTR gene mutation
Pilocarpine induced sweat test - excess chloride ions

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75
Q

2 mucolytics used to manage CF?

A

Nebulized N-acetylcysteine - Cleaves disulphide bonds in mucus glycoproteins

Dornase - Cuts up nucleic acid

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76
Q

Management of CF?

A

Chest physio
Bronchodilators (Albuterol or theophylline or anticholinergics)
Mucolytics
Anti-inflammaotry
Antibiotics
O2
CFTR modulators (Cafotr family)

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77
Q

LBW, VLBW, EXLBW?

A

<2.5Kg
<1.5Kg
<1Kg

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78
Q

Indications for 5milligrams of folic acid during pregnancy?

A

BMI >30
Previous NTD
Antiepileptics
Coeliac
Sickle cell
Methotrexate on alternate days

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79
Q

What is included in the routine infection screen?

A

HIV
Hep B
Rubella
Syphilis

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80
Q

What can be given if a fetus has supraventricular tachycardia?

A

Digoxin or Flecainide

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81
Q

Effect of anticonvulsants on fetus?

A

Midfacial hypoplasia
CNS, Limb + Cardiac malformation
Developmental delay

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82
Q

Effects of lithium on fetus?

A

Congenital heart defects - Ebstein anomaly (tricuspid is displaced)

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83
Q

Effects of tetracycline on fetus?

A

Enamel Hypoplasia of the teeth

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84
Q

Effects of Thalidomide on fetus?

A

Limb shortening

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85
Q

Effects of Vitamin A on fetus?

A

Increased risk of spontaneous miscarriage + abnormal face

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86
Q

Effects of warfarin on fetus?

A

Interferes with cartilage formation (Hypoplastic nose + stripped epiphysis)
Spontaneous abortion or stillbirth

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87
Q

Perinatal infection associated with anaemia + fetal hydrops?

A

Parvovirus

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88
Q

Effect of rubella infection on the fetus?

A

Deafness, Cardiac malformations + Cataracts

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89
Q

The causative organism of neonatal meningitis?

A

GBS e.g. Strep algalacticae

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90
Q

SE of epidural?

A

Maternal pyrexia
Increased risk of instrumental delivery

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91
Q

What is the first thing done in neonatal resuscitation?

A

Dry the baby, remove wet towels + cover then start the clock

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92
Q

What is the name of the manoeuvre used to correct developmental dysplasia of the hip?

A

Ortolani
Barlow used to identify then Ortiolani dues to correct

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93
Q

What is the recommended compression: ventilation ratio for the newborn?

A

3:1

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94
Q

Recommended regime for Vit K to prevent haemorrhagic disease of newborn?

A

One off IM injection

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95
Q

What is hypoxic-ischaemic encephalopathy?

A

Tissue injury due to lack of perfusion following a period of asphyxia

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96
Q

Some symptoms of hypoxic-ischaemic injury?

A

Irritability, abnormal tone or movement, impaired feeding, hyperventilation

seziures + no response

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97
Q

Causes of HIE?

A

Failure of exchange across placenta
Interruption of umbilical flow
Inadequate placental perfusion
Compromised fetus
Failure to breath

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98
Q

Medication given to the preterm infant?

A

Magnesium sulphate
10 day erythromycin
Steroids

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99
Q

Symptoms of resp distress in infants?

A

Tachypnoea >60bpm
Nasal flare
Grunting
Laboured breathing with chest wall recession
Cyanosis

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100
Q

What can be used to manage preterm apnoea/bradycardia?

A

Caffeine
Phosphodiesterase inhibitor

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101
Q

Management of patent ducuts arteriosus?

A

Neonatal Indomethacin - NSAID that inhibits the synthesis of prostaglandins

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102
Q

Management of patent ductus arteriosus?

A

Neonatal Indomethacin - NSAID that inhibits the synthesis of prostaglandins

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103
Q

Where are most brain injuries found?

A

Germinal matrix above the caudate nucleus

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104
Q

What are porencephalic cysts?

A

When blood has haemorrhaged then gathered into a cyst

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105
Q

What is seen in retinopathy of prematurity?

A

Stevie Wonder - changes in vascular growth + fibrous ridges grow

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106
Q

What is bronchopulmonary dysplasia?

A

When an infant still requires oxygen after 26 weeks due to continuous ventillation

Lung damage from pressure + volume trauma

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107
Q

What can be seen in the CXR of a neonate with bronchopulmonary dysplasia?

A

Widespread opacification
Cystic changes
Destroyed boarders

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108
Q

What enzyme is responsible for maintaining enterohepatic circulation?

A

Beta glucuronidase

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109
Q

What enzyme conjugates bilirubin in the liver?

A

Uridine diphosphatase glucuronyltransferase

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110
Q

FRBC lifespan?

A

70 days

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111
Q

Causes of increased haemolysis?

A

Rhesus disease
ABO incompatibility
Cephalohematoma (Extravasted blood)
G6PD deficiency
Sepsis
Polycythemia

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112
Q

Causes of increased enterohepatic circulation?

A

Breast feeding jaundice
- Poor feeding so the digestive system not fully functioning + dehyadration so no excretion of excess bilirubin

Breast milk jaundice
- increase beta glucuronidase so more enterohepatic circulation

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113
Q

Crigler-Najjar syndrome?

A

Absent/Low uridine diphospho glucuronosyltransferase so increase unconjugated bilirubin

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114
Q

BIND?

A

Bilirubin Induced Neurological Damage due to bilirubin diffusing over the BBB and attaching to basal ganglia + brainstem nuclei

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115
Q

Kernicterus?

A

Encephalopathy due to bilirubin deposits in the basal ganglia + brainstem nuclei
Too much to attach to bilirubin

Choreoathetoid cerebral palsy
Sensorineural hearing loss
Gaze abnormalities
Dental enamel dysplasia

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116
Q

symptoms of acute bilirubin encephalopathy?

A

Lethargy, hypotonia + poor suck then develops into hypertonia, high pitched cry, fever + seziures

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117
Q

Who are most at risk of transient tachypnoea of newborn?

A

Preterm + C section

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118
Q

Causes of resp distress in term infants

A

TTN
Meconium Aspiration
Pneumonia
Pneumothorax
Milk aspiration
Persistent pulmonary hypertension of newborn
Diaphragmatic HErnia

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119
Q

Management of persistent pulmonary hypertension of newborn?

A

Nitric Oxide or Sildenafil

Magnesium sulphate

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120
Q

Herpes simplex effect on newborn?

A

Hepatica lesions on the skin or eye +/- encephalitis or disseminated disease

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121
Q

Effect of toxoplasmosis on fetus?

A

Retinopathy, Cerebral calcifications or Hydrocephalus

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122
Q

Management of toxoplasmosis in newborn?

A

Pyrimethamine + Sulfadiazine for 1 year

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123
Q

Mother 5 weeks till delivery has genital lesions and test +ve for herpes simplex, what is the appropriate management?

A

C section + Oral Aciclovir 400mg tds

IV Aciclovir if PROM or spontaneous delivery

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124
Q

In what situation would the Hep B vaccination be given?

A

If the mother was HBsAg +ve then Hep B vaccine immediately after birth then Hep B immunoglobulin within 24 hours of birth

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125
Q

2 causes of neonatal seizures?

A

Hypoglycaemia + Meningitis

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126
Q

Pierre Robinson Sequence?

A

Underdeveloped jaw + backward displacement of tongue

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127
Q

Causes of small bowel obstruction in neonates?

A

Duodenal atresia
Small bowel atresia (jejunm or ileum)
Malrotation with volvulus
Meconium plug in the lower intestine
Meconium ileus
pyloric stenosis

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128
Q

Causes of large bowel obstruction?

A

Hirschprung disease - missing myenteric nerve plexus in the rectum and may extend to colon

Rectal atresia

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129
Q

VACTERL?

A

Vertebral
Anorectal
Cardiac
Traceho-oesophagal
Renal
Limb abnormalities

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130
Q

When is the fastest period of growth?

  • Fetal
  • Infancy
  • Childhood
  • Adolescent
  • Adult
A

Fetal - 30% of eventual height

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131
Q

What is growth during infancy dependent on?

A

Nutrition and environment

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132
Q

What is growth dependant on during childhood?

A

GH, IGF-1, Environment

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133
Q

What is growth dependent on during puberty?

A

GH, Testosterone + Oestrogen

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134
Q

First signs of puberty in males + females?

A

Male - Increased testicular volume

Female - Breast development

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135
Q

Short stature?

A

Height below the second centile

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136
Q

Height velocity?

A

Rate of growth over time

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137
Q

Causes of short stature?

A

Familial, IUGR, Prematurity, Endocrine, Poor nutrition

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138
Q

What is Laron syndrome?

A

Decreased IGF-1 due to decreased GH receptors

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139
Q

Investigations that can be used to assess growth?

A

Standing height
subischial height - Sitting height - total height
Skeletal survey

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140
Q

Causes of tall stature?

A

Obesity, CAH, Precocious puberty, Marfan, Klinefelter

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141
Q

Craniosynostosis?

A

Premature fusion of the sutures

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142
Q

Premature sex development?

A

showing secondary sex characteristics by 8 in females and 9 in males

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143
Q

What is the function of the SRY gene?

A

AMH + Testosterone + Dihydrotestosterone

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144
Q

Causes of disorders of sexual differentiation?

A

Excessive androgen
Androgen insensitivity syndrome
Ovotesticular disorder
Gonadotrophin insufficiency

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145
Q

Ovotesticular disorder?

A

Some of the cells are XX and some XY so both ovarian and testicular tissue present

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146
Q

Pathophysiology of CAH?

A

AR
Decreased cortisol + mineralocorticoid leadds to increased ACTH which stimulates the adrenal medulla to produce androgens

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147
Q

Signs of CAH?

A

Virilisation (clitoral hypertrophy or variable labial folds)
Enlarged pigmented scrotum
Adrenal crisis
Tall stature
Muscular build, pubic hair + acne

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148
Q

Typical bloods of a patient with CAH?

A

↓Na
↑K
Metabolic acidosis
Hypoglycaemia

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149
Q

What is the precursor to cortisol that is measured to diagnose CAH?

A

High 17α-hydroxyprogesterone

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150
Q

How may an adrenal crisis present?

A

Vomiting, weight loss, floppiness, Circulatory collapse

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151
Q

Features of Marfan?

A

Tall stature
Arahcnodactyl
Pectus excavum
Pes planus
Scoliosis
Cardiac problems
Repeated pneumothoraces
Upward lens dislocation
Dural ectasia - ballooning or widening of the dural sac

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152
Q

What is Marfans?

A

AD connective tissue disorder of the FBN1 gene on chromosome 15 that affects protein fibrillin 1

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153
Q

Risk factors for SIDS?

A

prone sleeping
parental smoking
bed sharing
hyperthermia and head covering
prematurity

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154
Q

What heart mumur is turners syndrome associated with?

A

Ejection systolic murmur

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155
Q

Shaken baby syndrome triad?

A

Retinal Haemorrhages + Subdural haematoma + Encephalopathy

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156
Q

Cardiac abnormalities seen in patients with turners syndrome?

A

Coarctation of the aorta or Bicuspid aortic valves

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157
Q

Features of maternal alcohol syndrome?

A

Microcephaly (small head)
Short palpebral fissures (small eye opening)
Hypoplastic upper lip (thin)
Absent philtrum
Reduced IQ
Variable cardiac abnormalities

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158
Q

what is the difference between weight for height and height for age?

A

weight for height is a measure of wasting and index of acute malnutrition (<3SD below the mean)

Height for age measures stunting an is an index for chronic malnutrition

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159
Q

What is the difference between Marasumus + Kwashiorkor?

A

Marasmus - weight for height <3SD below the mean + significant protein/carb malnutrition leading to wasting, apathy + significant weight loss

Kwashiorkor - Severe protein deficiency that causes fluid retention, protruding abdomen + wasting

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160
Q

Signs of kwashiorkor?

A

Flaky desquamated skin

Distended abdomen

Hepatomegaly (Fatty infiltration)

Spare + Depigmented hair

Diarrhoea, Hypothermia, Bradycardia, Hypotension

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161
Q

What is Rickets?

A

Failure of mineralisation of growing bone or osteoid tissue

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162
Q

Signs of Rickets?

A

Craniotabes

Rachitic Rosary

Harrison Sulcus

Pectus Carinatum (pigeon chest)

Bowed legs

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163
Q

MOA of orlistat and what it is used for?

A

Childhood obesity - it is a lipase inhibitor that reduces the absorption of fat

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164
Q

Fluid deficit calculation?

A

Deficit X 10 XWeight (Kg)

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165
Q

Maintenance doses?

A

First 10kg = 100ml/kg per day

Second 10kg = 50ml/kg per day

Remaining weight = 20ml/ kg per day

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166
Q

Fluid bolus given?

A

20ml/kg of 0.9% sodium chloride or can be 10ml/kg

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167
Q

Common causes of meningitis in newborns?

A

GBS, Listeria + E.coli

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168
Q

Bacterial causes of meningitis in childhood?

A

Neisseria Meningitidis

Strep Pneumonia

Haemophilus Influenzae

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169
Q

Viral causes of meningitis?

A

RSV

Enterovirus

EBV

Adenovirus

Mumps

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170
Q

Meningitis prophylaxis?

A

Rifampicin or Ciprofloxacin

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171
Q

Management of meningitis?

A

Ceftriaxone or Cefotaxime

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172
Q

Signs of encephalitis?

A

Insidious altered consciousness, Altered cognition, Unusual behaviour, Fever, Focal neurological signs, Seizures + fever

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173
Q

Causative organisms of encephalitis?

A

HSV, CMV, EBV, Measles, VZV

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174
Q

Signs of TSS?

A

Superantigens causes vomiting, diarrhoea, fever, hypotension, rash resembling sunburn on palms, soles, finger or toes

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175
Q

Necrotising fasciitis?

A

Severe SC infection involving skin down to the fascia and muscle causing large areas of skin to potentially become necrotic

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176
Q

Management of impetigo?

A

Hydrogen peroxide or fusidic acid cream

Oral flucloxacillin

Highly contagious skin infection common in pre-sensitising skin disease

48 hour after infection or until lesions crust over can they see others

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177
Q

What is this?

A

Impetigo - highly contagious staphy A or GAS infection over pre-existing skin conditions

Erythematous macules then become pustular the bullous containing honey coloured crusted lesions

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178
Q

What is this?

A

Nikolsky sign of scalded skin syndrome due to exfoliate staphy toxins

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179
Q

4 year old patient presentes with sore throat, fever, headache, fatigue, nasuea, pinpoint sandpaper rash over the trunk + pastia lines. How do you investigate and first line management?

A

Scarlet fever

Phenoxymethylpenicillin for 10 days

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180
Q

Causative organism of scarlet fever?

A

Strep pyogenes

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181
Q

Complications of scarlet fever?

A

Otitis media, Peritonsillar abscess, Rheumatic fever, Post-strep glomerulonehritis

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182
Q

complications fo VZV?

A

Secondary bacterial infections, Encephalitis, Pupura fulminans (skin necrosis + DIC)

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183
Q

What is mononucleosis syndrome and guilty culprits?

A

Enlarged glands, Feverm sore throat + fatigue

EBV, CMV

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184
Q

What virus is associated with Roseola infantum, fever + febrile convulsions?

A

HHV 6 + 7

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185
Q

Parvovirus clinical syndromes?

A

Erythema infectiosum (Slapped cheek) + myalgia

Aplastic crisis in patients with haemolytic anaemia

Fetal anaemia causing hydrops

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186
Q

Management of enteroviruses Coxsackir, Echovirs or polio?

A

Ceftriaxone

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187
Q

What can be given to manage measles in immunocompromised patients?

A

Ribavarin

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188
Q

Mumps?

A

Parotitis, fever, malaise, difficulty eating or swallowing

Risk of hearing loss/ meningitis or Encephalitis

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189
Q

Clincial features of kawasaki disease?

A

Fever >5 days

Non-purulent conjunctivitis

Red/dry cracked lips

Strawberry tongue

Cervical lymphadenopathy

Peeling fingers + toes

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190
Q

What is Kawasaki disease?

A

A systemic vasculitis that mostly affects 6 months to 4 year olds

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191
Q

Biggest complication of Kawasaki disease?

A

Giant Coronary Artery Aneurysm

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192
Q

Management of Kawasaki disease?

A

IVIG for 10 days

High dose asprin

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193
Q

TORCH?

A

Toxoplasmosis, Rubella, CMV, HSV

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194
Q

Abx used as prophylaxis against PCP in immunosuppressed patients?

A

Co-trimoxazole

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195
Q

10 year old child went to the farm over summer and developed a dart like rash on their torso. They have muscle and joint pain and a persistent fever. What is the condition and management?

A

Lyme disease

Hard tick

Erythema migrans is the dart-like rash on their torso

because the child is under 12 then Amoxicillin but if over 12 then Doxycycline

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196
Q

SPUR?

A

Spontaneous, Prolonged, Unusual, Recurrent

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197
Q

Common causes of Vomiting in infants?

A

GORD, Infection, Intolerance, Intestinal obstruction

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198
Q

Common causes of vomiting in adolescence?

A

Gastroenteritis, Infection, Appendicitis, Intestinal obstruction

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199
Q

Investigations of GORD in paeds?

A

24 hour OE pH monitoring

24 hour impedance monitoring - measures acidic and non-acidic reflux in OE

Endoscopy + biopsy

+/- contrast

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200
Q

Management of GORD in paeds?

A

Thickening agents + Upright posture

Ranitidine (H2 receptor antagonist)

Omeprazole (PPI)

Domperidone (Increases gastric emptying after eating)

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201
Q

What is pyloric stenosis?

A

Hypertrophy of the pyloric muscle causing gastric outlet obstruction around 2 to 7 weeks of age

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202
Q

Diagnosis of pyloric stenosis?

A

Test feed - while baby is being fed examine gastric peristaltic movements across the abdomen (+/- pyloric mass in RUQ or overdisteneded stomach)

Bloods - metabolic alkalosis

Ultrasound

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203
Q

Management of pyloric stenosis?

A

Correct fluid balance

Pyloromyotomy - remove some of the muscle - feed the baby within 6 hours and discharge within 2 days

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204
Q

What is infant colic?

A

Inconsolable crying between 6 weeks to 4 months that is self-limiting

Baby draws up their knees and arches their back

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205
Q

Management of infant colic?

A

Self-limiting by 6 months

could try hydrolysate formula or gripe water

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206
Q

What is non-specific abdo pain?

A

Abdo pain that resolves within 24 to 48 hours usually associated with URTI or cervical lymphadenopathy

207
Q

Ddx of acute abdo pain?

A

Appendicitis

NSAP

Meckel’s diverticulum

Intussusception

Malrotation with volvulus

Mesenteric lymphadenitis (larrge mesenteric nodes)

208
Q

What is this condition?

Diagnosis?

Management?

A

Intersussception causing obstruction - when proximal bowel invaginates into distal bowel in 3 months to 2 year olds

AXR - Distended small bowel and no clear distal colon

Rectal air insufflation + IV fluids if in shock

209
Q

Clinical features of intussusception?

A

3 months to 2 years old

Redcurrant jelly mucus stool

Paroxysmal abdo pain

Pallor

Drawn up legs

Vomit

Decreased feeds

Palpable sausage-shaped mass

210
Q

Meckel’s diverticulum is a remnant of what and how can it be identified?

A

Vitelline duct - communicated between the yolk sac and midgut of fetus

found 2cm IC, <2%, 2 year olds, 2 types of tissue

Technetium scan - shows uptake by gastric mucosa

211
Q

What is Malrotation?

A

When the small bowel isn’t fixed at the duodenojejunal flexure + ileocaecal region so the small bowel is short and prone to volvulus

Can cause an obstruction as Ladd bands go over the duodenum to attach the caecum to the retroperitoneum

212
Q

Diagnosis of Malrotation?

A

Upper GI contrast study or Laparoscopy

213
Q

Causes of recurrent abdo pain?

A

GI - IBS, constipation, IBD, Coeliac

GYNAE - Dysmenorrhoea, Ovarian cysts, PID

LIVER/PANCREAS - PAncreatiirs, Hepatitis, Gall stones

UT - UTI or obstruction

PSYCHOSOCIAL - bullying, abuse

214
Q

IBS symptoms?

A

Abdo pain worse after eating and made better by opening bowels

Explosive loose stool + mucus stool

Bloating

Feeling of incomplete defecation

Or even constipation

215
Q

When would you suspect a child has a duodenal ulcer?

A

If the pain is waking them up int he middle of the night otherwise uncommon

216
Q

Investigations of H pylori infection?

A

Gastric antral biopsy

Urea breath test

H. Pylori stool antigen test

217
Q

Management of H.pylori infection?

A

O, C + A/M

218
Q

Common causes of gastroenteritis and key features?

A

Rotavirus

Sever abdo pain - campylobacter

Blood, pus +/- fever -shigella or salmonella

Rapidly dehydrating diarrhoea - Cholera

219
Q

Signs of dehydration?

A
220
Q

Management of a dehydrated baby?

A

Breastfeeding + increased fluid intake

Oral rehydration solution (50ml/kg over 4 hours)

NG tube

IV sodium chloride (bolus, Maintenance or correct deficit)

221
Q

What test can be used to diagnose post gastritis intolerance?

A

+ve clinitest - non-absorbed sugar in stool

222
Q

Histological features of coeliac?

A

Crypt hyperplasia, Villous atrophy + intraepithelial lymphocytes

223
Q

Diagnosis of coeliac?

A

Endoscopic biopsy of small bowel

+ve IgA tissue transglutaminase + Endomyseal Antibodies

224
Q

What other conditions are coeliac associated with?

A

T1DM, Autoimmune thyroid disease, Downs

225
Q

Complications of crohns v complication of UC?

A

Crohns - strictures, fistulae, Anal tags

UC - Toxic megacolon

226
Q

Extra abdominal features of crohns?

A

Uveitis, Arthalgia, Erythema nodosum (swollen fat under skin)

227
Q

Management of crohns?

A

Whole protein modular feeds for 6 to 8 weeks

Relapse:

Azathioprine, Methotrexate

Mercaptopurine or Infliximab (anti TNF alpha)

228
Q

Management of UC?

A

Aminosalicylates for induction and maintenance

Azathioprine +/- low dose corticosteroids to maintain remission

229
Q

Causes of constipation?

A

GI- Hirshprung, Coeliac, GI dysmotility, Crohns

Neuro - Lumbosacaral pathology or spina bifida

Endo - hypothyroidism

Metabolic - Hypocalcameia

230
Q

Management of constipation

A

Stool softener - Movicol (Polyethylene glycol) which is an osmotic laxative that draws water into the stool

Stimulant laxative - Senna or Sodium Picosulphate

Enema

Manual evacuation

231
Q

Hirschsprung disease?

A

Absence of ganglion cells in the distal colon causes obstruction

Rectal exam - narrow then stool will come gushing

Suction rectal biopsy - shows absence of ganglion cells

colostomy - anastamose bowel to anus

232
Q

Common cause of coryza in children?

A

Rhinovirus, Coronavirus or RSV

233
Q

2 causative organisms of tonsillitis?

A

GAS + EBV

234
Q

Management of tonsillitis?

A

Penicillin or Erythromycin

Avoid amoxicillin as can cause maculopapular rash if tonsillitis caused by EBV

235
Q

Causative organism of croup?

A

Parainfluenza virus

236
Q

Causative organism of bacterial tracheitis/ pseudomembranous croup and how it differs from normal croup?

A

Staphy A

Thicker mucus + High fever

237
Q

Causative organism of epiglottis?

A

Haemophilus Influenza type B

238
Q

How dose croup differ from epiglottis?

A

Onset - over days v over hours

Preceding coryza - yes

Cough - croup more barking v no

Able to drink? - yes v no

Drooling - no v yese

Appearance - unwell v toxic/ very ill

Fever - <38.5 v >38.5

Stridor - harsh v whispering/ soft

Voice - Hoarse v muffled

239
Q

3 stages of bronchitis and their causative organism?

A

Catarrhal

Paroxysmal

Convalescent

240
Q

Complications of bronchitis?

A

Vomiting, Epistaxis, Sunconjunctival haemorrhages, Pneumonia, Convulsions, Bronchiectasis

241
Q

Management of bronchitis?

A

Erythromycin for 10 days and prophylaxis for close contacts

242
Q

Causative organism of bronchiolitis and it’s symptoms?

A

RSV

Protruding sternum, hyperinflated chest, displaced liver, sharp dry cough, cyanosis

243
Q

Age range most hit by bronchiolitis and prophylaxis that can be given?

A

1 to 9 months, rare in over 1 year

Palivizumab

244
Q

Causative organisms of pneumonia in newborn?

A

GAS, Enterococci, Infection from mothers genital tract

245
Q

Causative organism of pneumonia in infancy?

A

RSV, Strep pneumonia, HI, Staphy A, Chalmydia

246
Q

Clinical features of pneumonia in children?

A

Cough, Dyspnoea, Irritability, Nasal flare, Grunting, Chest recession, Lethargy, Malaise, Decreased feeding

247
Q

Ddx of wheeze?

A

TEW, Atopic asthma, Non-atopic asthma, Anaphyalxis, Aspiration of foreign body, congenital lung defects

248
Q

Signs of asthma?

A

Wheeze (Whistle when the child breathes out), Worse at night and early morning, Interval symptoms, +ve response to bronchodilators, Fx of atopy

249
Q

How does the second line management of asthma change between >5 and <5?

A

<5 - LTRA

>5 Low dose ICS

250
Q

Management of meconium ileus?

A

Gastrografin enema or surgery

251
Q

Pancreatic symptoms seen in CF?

A

Staetorrhoea or Avitaminosis A

252
Q

CF effects on the liver?

A

Small biliary obstruction or Biliary Cirrhosis

253
Q

What conditions are associated with primary ciliary dyskinesia?

A

Dextrocardia or Situs Invertus

254
Q

Aside from obesity, what are other causes of OSA in children?

A

Adenotosillar hypertrophy, Craniofacial abnormalities, Achondroplasia, Neuromuscular disease

255
Q

Left to right shunting causes?

A

ASD, VSD, Partial AVSD

256
Q

Right to left shunting?

A

ToF

Transposition of great arteries

257
Q

Outflow obstruction + asymptomatic well child?

A

Pulmonary stenosis or Aortic stenosis

258
Q

Mixed breathless and blue?

A

Complete AVSD

259
Q

Symptoms of heart failure in infants?

A

Breathlessness, Sweating, Poor feeding, Recurrent chest infections

Faltering growth, Tachypnoea, Tachycardia, Enlarged heart, Peripheral oedema, Cool peripheries

260
Q

Causes of Left heart obstruction in neonates?

A

Hypoplastic LH syndrome (narrow aorta)

AV stenosis

Coarctation of aorta (arterial duct around and collaterals form)

Interruption of the aortic arch

261
Q

Continuous diastolic murmur beneath the clavicle + collapsing pulse + pulmonary hypertension +/- HF?

A

PDA

Failure to close after 1 month

262
Q

Tof?

A

Overriding aorta

RV outflow obstruction/ Pu;moanry stenosis

RV hypertrophy

VSD

263
Q

ToF clinical features?

A

Cyanosis + O2 sats <94% in first weeks of life

Hypercyanotic spells

Clubbing on fingers and toes

Large harsh systolic ejection murmur at the left sternal edge

264
Q

What is transposition of great arteries?

A

Aorta connected to right ventricle and pulmonary artery connected to left ventricle

Cyanosis + large second heart sounds

Narrow upper mediastinum + increased pulmonary flow

Prostaglandin infusion or balloon septostomy - tearing the foramen ovale open allowing the mixture of blood

265
Q

What test is used to determine the presence of heart disease in a cyanosed neonate?

A

Hyperoxia nitrogen washout test

placed in headbox/ 100% O2 ventilator for 10 mins then if PaO2 remains low then congenital heart disease is diagnosed

After excluding persistent pulmonary hypertension
+ chronic lung disease

ABC + prostaglandin infusion

266
Q

Complete AVSD?

A

Hole in the middle of the heart resulting in 5 leaflet valve between atria and ventricles

Cyanosis + breathlessness 2 to 3 weeks of life

Diuretics + Captopril + surgery

267
Q

The difference in clinical features between aortic stenosis + pulmonary stenosis?

A

AS - ejection systolic murmur heard at right upper sternal edge, carotid thrill, slow rising pulse

PS - ejection systolic murmur heard at left upper sternal edge, heave

268
Q

Out of the following 3 options of outflow obstruction in sick infants, which causes circulatory collapse,e absent femoral and absent left brachial?

  1. Coarctation of aorta
  2. Interruption of aortic arch
  3. Hypoplastic left heart syndrome
A
  1. Interruption of aortic arch
269
Q

Management of SVT?

A

CPAP, Vagal Stimulation, Adenosine or DC cardioversion

270
Q

Criteria used for rheumatic fever?

A

Jones criteria

2 major or 1 major + 2 minor

271
Q

The causative organism of rheumatic fever?

A

GAS e.g. Strep pyogenes

272
Q

When does rheumatic fever present?

A

5 to 15 year olds

2 to 6 weeks after pharyngeal infection, arthritis, fever + malaise

273
Q

Major manifestation of rheumatic fever?

A

Pancarditis, Polyarthritis, Sydenham Chorea, Erythema marginatum, Subcutaneous nodules

274
Q

Minor manifestations of rheumatic fever?

A

Fever, Polyarthalgia, History of RF, Prolonged PR interval, Raised acute phase reactions

275
Q

Management of rheumatic fever?

A

Penicillin/ Erythromycin/ Cephalosporin

High dose NSAID or aspirin for arthritis

Diuretics, ACEi, Digoxin for heart failure

Chorea is self-limiting of diazepam

276
Q

What is infective endocarditis and who is at high risk?

A

Strep viridans

Vegetations, fibrin and microorganisms on the valves cause damage

High risk are those with congenital heart disease

277
Q

management of infective endocarditis?

A

6 weeks aminoglycosides + good dental hygiene

278
Q

Management of cardiomyopathy?

A

Diuretics

ACEi

Carvedilol

279
Q

How does eGFR change between infancy and 2 years old?

A

15 to 20ml/min per 1.73m2 to 80 to 120ml/min per 1.73m2

280
Q

Methods of assessing renal function?

A

Urea + Creatinine

eGFR

Plasma Creatinine ratio

Insulin or EDTA - substances freely filtered in glomeruli

281
Q

MAG3 Renogram?

A

Dynamic scan

Renal blood flow by injecting isotope that is excreted from blood into urine and ASSESS URINARY DRAINAGE ( identify obstruction)

282
Q

DMSA scan?

A

Status scan of renal cortex

Looking for functional defects or scars - highly sensitive so best to do 2+ months after UTI

283
Q

Renal ultrasound?

A

anatomical assessment of kidneys + UT but not function

284
Q

Micturating cystourethrogram?

A

Contrast inserted into bladder through urethral catheter

Detect vesicoureteric reflux + urethral obstruction

285
Q

Renal agenesis?

A

Absence of both kidneys

Severe oligohydramnios + potters syndrome

Fetus is compressed - lung hypoplasia, severe talipes, limb deformities

Facies - beaked nose, downward slanting eyes, low-set ears

286
Q

Multicystic dysplastic kidney?

A

Failure of the fusion of the ureteric buds with nephrogenic mesenchyme

Large non-functioning fluid-filled cysts with no renal tissue or connection to bladder

Causes potters syndrome if bilateral

287
Q

Hypertension + haematuria then progress into renal failure?

A

ADPKD

288
Q

Difference between ARPKD and ADPKD?

A

ARPKD - diffuse and bilateral enlargement of the kidneys

ADPKD - Separate cysts of varying size between normal renal parenchyma

289
Q

What 2 conditions are caused by the abnormal caudal migration of kidneys during fetal development?

A

Pelvic kidneys + Horseshoe kidney (fusion of the lower poles of the kidney - increased risk of infection + obstruction)

290
Q

Duplex system of kidneys?

A

Incomplete fusion of the upper and lower pole of the kidney creates 2 drainage systems from the kidney

Lower pole - reflux

Upper pole - into urethra, vagina or prolapse into the bladder

291
Q

Prune belly syndrome?

A

Absent musculature syndrome - no anterior abdominal wall

Large bladder, Dilated ureters + Cryptochordism

292
Q

The common sites for urinary tract obstructions to occur?

A

Pelviureteric junction + Vescioureteric junction + bladder neck (posterior urethral valve)

293
Q

Posterior urethral valves?

A

Gross vesicoureteric reflux

Distended bladder

Dilated posterior urethra

Posterior urethral valve

Bilateral hydronephrosis

Antenatal ultrasound or Micturating Cystourethrogram

294
Q

2 methods of investigating urinary obstruction or vesicoureteric reflux in children?

A

Dynamic MAG3 or Micturating Cystourethrogram

295
Q

Symptoms of UTI in infancy?

A

Fever, Unsettled, Irritable, Poor feed, Lethargy, Jaundice, Septicaemia, Febrile convulsions, Offensive urine

296
Q

Symptoms of UTI in childhood?

A

Dysuria, Frequency, Urgency, Vulvitis or Balinits, Febrile convulsions, Loin tenderness, Suprapubic pain, Offensive urine

297
Q

Investigations of UTI?

A

Urine dipstick + culture (>10^5/ml of single organism)

Ultrasound

Static DMCA + MCUG (<3 months)

298
Q

Causative organisms of UTI?

A

E.coli

299
Q

What organisms is associated with UTI + struvite stones?

A

Proteus mirabilis

300
Q

What organism is associated with UTI + Structural abnormality?

A

Pseduomonas

301
Q

Rfx for UTI?

A

Infrequent voiding

Structural abnormalities

Incomplete micturition

Obstruction due to loaded bladder by constipation

Vesicoureteric reflux

Neuropathic baldder

302
Q

Management of UTI?

A

<3 months - IV cefotaxime

>3 months + acute pylonephritis - Oral co-amoxiclav or IV cefotaxime

LUTI + older child - Oral abx for 3 days

303
Q

UTI prophylaxis?

A

Trimethoprim (2mg/Kg)

Nitrofurantoin or Cephalosporin

Other: Ensure complete bladder empty, Regular voiding, Prevent constipation

304
Q

What is daytime enuresis?

A

Loss of bladder control during the day in 3 to 5 year olds

305
Q

Causes of daytime enuresis?

A

Lack of attention to bladder sensation

Detrusor instability

Bladder neck weakness

Neuropathic bladder

UTI

Constipation

Ectopic ureter

306
Q

Dry at night then waking up in a pool of urine + female?

A

Ectopic ureter with the vagina

307
Q

Management of daytime enuresis?

A

Start chart

Enuresis alarm

Pelvic floor exercises

Treat constipation

Oxybutynin - relax etrusor muscle

308
Q

Secondary enuresis?

A

UTI, Emotional upset, DM

309
Q

What is nephrotic syndrome?

A

Heavy proteinuria that results in decreased plasma albumin and oedmea

310
Q

Causes of proteinuria?

A

Orthostatic postural proteinuria

Glomerulonephropathy

Abnormal glomerular basement membrane

Increase GFR

Renal mass

Hypertension

311
Q

Symptoms of nephrotic syndrome?

A

Periorbital oedema

Lower limb or genital oedema

Ascites

Breathlessness due to distended abdomen and pleural effusion

312
Q

Features suggesting steroid-sensitive nephrotic syndrome?

A

Age 1 to 10

No microscopic haematuria

Normal BP

Normal complement function

Normal renal function

313
Q

Management of proteinuria?

A

60mg/m^2 of prednisolone for 4 weeks then drop to 40 for 4 weeks then should be protein-free within 11 days

314
Q

Complications of nephrotic syndrome?

A

Hypovolaemia

Thrombosis (urinary loss of antithrombin + raised haematocrit creates a hypercoagulable state)

Pneumococcus Infection

Hypercholesterolaemia

315
Q

Features of acute nephritis?

A

Haematuria

Proteinuria

Periorbital oedema

Hypertension

Decreased urine output

Volume overload

316
Q

Causes of acute nephritis?

A

Henoch-scholein purpura

SLE

Post strep

IgA nephropathy

Mesangiocapillary glomerulonephropathy

Anti-basement membrane

317
Q

Investigations of post-infectious nephritis?

A

Culture

Raised Antistreptolycin or Anti-DNAse B

Low C3 complement 3 to 4 weeks post-infection

318
Q

HSP?

A

IgA Vasculitis seen in 3 to 10 year olds after a URTI

A purpuric rash over butt, legs + arms

Haematuria

Knee and ankle joint pain and swelling

Bloody diarrhoea

Self-limiting or NSAIDs

319
Q

commonest familial nephritis?

A

Alport syndrome - X-linked recessive

Male, deaf, ocular defects +/- mama haematuria

320
Q

SLE nephritis?

A

Anti DNA, ANA, low Complement

Haematuria + Proteinuria

321
Q

Paediatric hypertension?

A

BP >95th centile for height, age + sex

322
Q

Causes of paediatric hypertension?

A

Renal: Renal parenchymal disease, Renal artery stenosis, PKD, Renal tumours

Coarctation of aorta

Catecholamine excess: Phaeochromocytoma or Neuroblastoma

Endocrine: CAH, Cushings, Hyperthyroidisim

Essential hypertension

323
Q

Unilateral causes of palpable kidneys?

A

Mutocystic kidney

Obstructed hydronephrosis

Renal tumour (Wilms tumour)

Renal vein thrombosis

324
Q

Causes of bilateral palpable kidneys?

A

PKD (AR/AD)

Tuberous sclerosis

RV thrombosis

325
Q

What is Fanconi syndrome?

A

Substances that are normally reabsorbed are urinated out due to dysfunction of proximal tubule cells

Excess loss of AA, Glucose, Phosphate, Bicarbonate, Sodium, Calcium, Potassium, Urate

326
Q

What kind of acidosis is seen in Fanconi syndrome compared to Bartter syndrome?

A

Fanconi - Hyperchloraemic metabolic acidosis

Bartter syndrome - Hyperkalaemic metabolic acidosis

327
Q

Difference between RTA 1 and RTA 2?

A

RTA 1 causes metabolic acidosis by decreasing the secretions of hydrogen ions

RTA 2 causes metabolic acidosis by decreasing the reabsorption of bicarbonate

328
Q

Prerenal cause of AKI?

A

Hypovolaemia

low excreted sodium as body tries to retain fluid

Secondary to burns, sepsis, haemorrhage, gastroenteritis

329
Q

Management of hyperkalaemia?

A

Calcium gluconate

Salbutamol

Calcium exchange reisn

Glucose + Insulin

330
Q

Hyperphosphatemia management?

A

Calcium carbonate

331
Q

Causes of renal failure?

A

Vascular

Tubular

Glomerular

Interstitial

332
Q

Haemolytic uraemic syndrome?

A

Bloody diarrhoea then AKI + Microangiopathic haemolytic anaemia + Thrombocytopenia

Toxins of E.Coli or shigella cause intravascular thrombogenesis in the endothelial cells of the kidneys decreasing all the platelets then all the RBC that try come to the rescue get damaged from the shearing forces and turbulence of the small vessels

333
Q

Clinical features of CKD in children?

A

Anorexia, Lethargy, Polydipsia, Plyuria, Failure to thrice, Renal osteodystrophy causing bony deformities, Hypertension, Proteinuria, Normochromic normocytic anaemia

334
Q

Management of CKD?

A

Calcium carbonate + decrease phosphate intake

Recombinant Erythropoietin

Growth hormone

Dialysis

Kidney Transplant

335
Q

Phimosis?

A

When the foreskin is too tight to be pulled over the glans penis

Non-retractile foreskin

336
Q

Hypospadias?

A

Where the opening of the urethra is not located at the tip of the penis

Don’t circumcise and check both testes are palpable

Can be Glanular, Coronal, Midshaft or Penoscrotal

337
Q

Blue dot on tesis?

A

Torsion of the testicular appendix - remnant of paramesonephric duct (mullerian)

Tender upper pole

Pre-pubertal

338
Q

Ddx for acute scrotum?

A

Testicular torsion

Torsion of appendix testis

Idiopathic scrotal oedema

339
Q

Smegma pearl?

A

Myertiosu penile swelling

Collection of sebaceous material and dead skin inside the foreskin above the glands

340
Q

How to differentiate between indirect inguinal hernia + hydrocele?

A

Can you get above the swelling and can it be illuminated = hydrocele

Hydrocele - when fluid trickles down an open processes

341
Q

Ddx for head and neck lumps?

A

Thyroglossal cyst - stopping as it goes through it’s natural descent

Branchial fistula

External angular dermoid cysts - the skin gets trapped under another bit of skin near the eyebrow

342
Q

2 indications for circumcision?

A

Phimosis

Recurrent Balanoposthitis - redness of the foreskin + discharge (Warm baths + Abx)

Recurrent UTI

343
Q

Paraphimosis?

A

When foreskin can’t be put back in normal position after being retracted due to swollen glans

344
Q

Causes of biliary tree obstruction + conjugated >20 micromol/L in children?

A

Biliary atresia or Choledochal cyst

345
Q

Symptoms of biliary atresia?

A

Pale stool + dark urine

Failure to thrive

Hepatomegaly/ splenomegaly secondary to portal hypertension

346
Q

Investigations of biliary atresia or choledochal cyst?

A

Ultrasound

Radioisotope scan showing the uptake by the liver but not being excreted into bowel then liver biopsy +/- laparotmy

If dilated on ultrasound - Cholangiogram

347
Q

What is a choledochal cyst?

A

Cystic dilatation of the extrahepatic biliary system causing obstruction + elevation of conjugated bilirubin

348
Q

Causes of neonatal hepatitis?

A

Alpaha 1 AT

Galactosaemia

Neonatal cholestasis of infancy - give ursodeoxycholic acid

Progressive familial Intrahepatic Cholestasis

Intrahepatic biliary hypoplasia (Liver, Heart, Skeletal, Renal)

349
Q

Galactosaemia?

A

Unable to convert galactose to glucose

Poor feeding + vomit + jaundice + hepatomegaly + cataracts

350
Q

Which hepatitis has the greatest risk of causing chronic liver disease?

A

Hep B

351
Q

Which hepatitis is associated with post-transfusion hepatitis and how can it be managed?

A

Hep C

Pegylated interferon + Ribavirin

352
Q

Causes of acute liver disease?

A

Hep A, B, C non-A to G

Paracetamol overdose, Isoniazid, halothene, poisonous mushroom

Wilsons disease

Autoimmune

Reye syndrome

353
Q

Management of acute liver failure?

A

Dextrose keep BM >4

Broad spec abx + antifungals

Vit k to prevent bleeding

Mannitol diuresis - prevent cerebral oedmea

354
Q

Signs of acute liver failure?

A

Jaundice

Encephalopathy

Coagulopathy

Hypoglycaemia

Electrolyte disturbance

355
Q

Reyes syndrome?

A

Non-inflammatory encephalopathy with microvesicular fatty infiltration of the liver

no aspirin to <12

Medium-chain acyl-CoA in heel prick to ID those at risk

356
Q

Causes of chronic liver disease?

A

Chronic hepatitis

Wilsons disease

A1AT

CF

Neonatal liver disease

Bile duct lesions

357
Q

Autoimmune hepatitis?

A

7 to 10 year old

Skin rash, Lupus erythematosus, arthritis, haemolytic anaemia, nephritis

Hypergamaglobulinaemia >20m/L + autoantibodies + low complement

Prednisolone + azathioprine

358
Q

Management of wilsons disease?

A

Penicillamine or Trientine - promote copper excretion

Zinc - reduce copper absorption

Pyridoxine - prevent peripheral neuropathy

359
Q

Complications of cirrhosis?

A

OE varieties that can bleed

Ascites

Spontaneous bacterial peritonitis

Encephalopathy

360
Q

ALL symptoms?

A

Acute Lymphatic Leukaemia

2 to 5 year olds

Insidious onset BM infiltration, Malaise, Anorexia, Bone pain, Hepatosplenomegaly, Lymphadenopathy, Headache, Vomiting, Nerve palsy +/- Mediastinal mass

361
Q

ALL Investigations?

A

FBC - Low Hb, Low Platelets, Leukaemic blast cells

CXR - MEdiastinal mass (central lymphadenopathy)

Bone marrow biopsy

362
Q

Management of ALL?

A

Induction - Consolidation + CNS - Interim maintenance - Delayedd Intensification - Continue maintenance

Overall management takes about 3 years to enter remission

363
Q

What is remission?

A

Eradication of leukaemic blast cells + restoration of normal marrow function

364
Q

What can be used to protect the kidneys against the effect of rapid cell lysis?

A

Allopurinol or Urate oxidase

365
Q

Common location of paeds brain tumours?

A

Infratentorial

366
Q

Most common type of brain tumour in children?

A

Astrocytoma (Glioblastoma Multiforme)

367
Q

Signs of brain tumour?

A

Raised ICP, Focal neurological signs

Spinal - Back pain, limb weakness, bladder/bowel sensation altered

368
Q

Investigations of brain tumours?

A

MRI

MR spectroscopy - biological activity of tumour

369
Q

Which lymphoma is more common in adolescence than childhood?

A

Hodgekin - adolescence

Non-hodgkin - Childhood

370
Q

Hodgkin lymphoma sympotms/signs?

A

Painless lymphadenopathy +/- airway obstruction

Normal FBC

CXR - mediastinal mass

Reed Sternberg cells

Radiological assessment of all nodal sites will show uptake by cervical and axillary nodes

371
Q

Symptoms/ signs of non-Hodgkin lymphoma?

A

B symptoms - fever, weight loss, night sweats, pruritus

Head, neck + Abdo - Abdo obstruction or intussusception

SVC obstruction

372
Q

Signs of raised ICP in infants?

A

Vomit, Tense fontanelle, Separating sutures, Increase Head circumference, Head tilt, developmental delay

373
Q

Signs of raised ICP in children?

A

Headache on waking, Vomit, Personality change, Visual disturbance, Papilloedema

374
Q

Neuroblastoma?

A

Tumours of the neural crest tissue in the adrenal medulla + sympathetic nervous system

<5 year olds - worse prognosis if developed after 1 year old

375
Q

Symptoms of neuroblastoma?

A

Abdo mass, Bone pain, Weight loss, Malaise, Limp, Pallor, Hepatomegaly

may wrap around major vessels if cross midline

376
Q

What scan can be used to identify neuroblastoma + pheochromocytoma?

A

MIBG scan

377
Q

Investigations of neuroblastoma?

A

Increased catecholamines

Biopsy of adrenal medulla

Bone marrow

MIBG

378
Q

Wilms tumour?

A

Nephroblastoma

Embryonal renal tissue

<5 years old

Large abdo mass found incidentally +/- Abdo pain, haematuria, anaemia, hypertension

Ultrasound/ CT/ MRI - intrinsic renal mass altering structure

Chemo + delayed nephrectomy

379
Q

commonest form of soft tissue sarcoma in childhood?

A

Rhabdomyosarcoma

380
Q

Clinical features of soft tissue sarcoma?

A

Head + neck - most common causing proptosis, nasal obstruction or bloodstained nasal discharge

GU - dysuria or urinary obstruction, scrotal mass, blood-stained vaginal discharge

381
Q

Bone tumour most seen in younger children?

A

Ewig sarcoma

382
Q

Symptoms of bone tumour?

A

Bone pain + mass but otherwise well

383
Q

Investigations of bone tumours?

A

X ray - bony lesions

Bone scan

Chest CT - lung met?

384
Q

Retinoblastoma?

A

Malignant tumour of the retinal cells

Hereditary - AD 13

Within 3 years old

White pupillary reflex +/- squint

MRI

Chemo or radio

385
Q

Common liver cancer in children?

A

Hepatoblastoma

386
Q

Liver tumours

A

Abdo distension + mass

Pain and jaundice rare

387
Q

Alpha feto protein associated with which tumours?

A

Hepatoblastoma + Germ cell tumour

388
Q

Germ cell tumours?

A

Primitive germ cells

Alpha FP + Beta HCG

Malignant - gonads + Benign - Sacrococcygeal

389
Q

Langerhans cell histiocytes?

A

Abnormal proliferation of histiocytes

Disorder of antigen-presenting cells/ Macrophages

Bony lesions, Diabetes Insipidus, Seborrhoeic rash,

Soft tissue involvement of gums, ears, lungs, liver spleen, lymph nodes + bone marrow

390
Q

Cancers in children <5?

A

Neuroblastoma

Wilm tumour

Retinoblastoma

ALL

Non-Hodgkin

391
Q

Cancer in school-age children?

A

ALL

Brain tumour

392
Q

Cancer in adolescence?

A

ALL

Hodgkin lymphoma

Soft tissue sarcoma

393
Q

Hb levels that indicate anaemia in different age groups?

A

Neonate <14g/dl

1 to 12 months <10g/dl

1 to 12 years <11g/dl

394
Q

Causes of impaired RBC production?

A

Red cell aplasia

Ineffective erythopoesis

395
Q

4 things that can decrease iron absorption?

A

Cows milk, Vitamin C, Tannin + high fibre foods

396
Q

Causes of red cell aplasia + how can they be investigated?

A

Parvovirus B19

Diamond blackfan anaemia - congenital

Transient erythroblastopenia of childhood

Parvovirus serology + bone marrow aspirate

397
Q

Clinical features of iron deficiency anaemia?

A

Asymptomatic until Hb < 6 to 7 g/dl

Lethargy, Decreased feeding, Pallor, Pica (eating non-food materials)

398
Q

Management of iron deficiency anaemia?

A

Diet

Sytron or Niferex (don’t stain teeth) until Hb normal for 3 months

if no helpful - look at malabsorption or chronic blood loss

399
Q

Diagnostic clues to red cell aplasia?

A

Low reticulocyte count + low Hb

Normal bilirubin

Negative coombs

Absent rec cell precursors in bone marrow

400
Q

What do images A B + C show?

A

A - spherocytosis

B - Sickle cell

C- Hypochromic cells seen in thalassaemia

401
Q

David blackfan anaemia?

A

Congenital mutations in ribosomal protein genes

2 to 3 months

short stature + abnormal thumbs

Oral steroids + monthly blood transfusions or stem cell transplant

402
Q

What is transient erythroblastopenia of childhood?

A

Anaemia triggered by a viral infection

403
Q

Causes of haemolytic anaemia?

A

Red cell membrane disorders

G6PD deficiency

Haemoglobinopathies

404
Q

Signs of haemolytic anaemia?

A

Anaemia

Hepatomegaly or splenomegaly

Increased unconjugated bilirubin

Excess urobilinogen

405
Q

Diagnostic clues of haemolysis?

A

Raised reticulocytes (lilac)

Increased unconjugated bilirubin or urinary urobilinogen

Blood film shos abnormal appearance

Increased Red cell precursors in the bone marrow

406
Q

Hereditary spherocytosis?

A

Spectrin, Ankyrin or band 3 mutation

Red cell loses part of the membrane as it passes through spleen

Less deformable then destroyed in the microvasculature of spleen

407
Q

Management of sphreocytosis?

A

Oral folic acid

Splenectomy after 7

Hib, Men C + strep pneumonia vaccine

408
Q

G6PD deficiency?

A

G6PD - essential for preventing oxidative damage to red cells

X linked

Neonatal jaundice or triggered by infection, medication, fava beans or naphthalene in moth balls

Intravascular haemolysis

409
Q

G6PD drug triggers?

A

Antimalarials

Abx: Co-trimoxazole, Quniolones, Nitrofurantoin

Aspirin

Naphthalene (moth balls)

Fava beans

410
Q

Sickle cell disease pathophysiology?

A

HbS polymerises within RBC causing them to be rigid in structure = sick shape

reduced lifespan and get trapped in microcirculation = blood vessel occlusion + ischaemia of bone or ogan

Precipitated by low oxygen, dehydration + cold

411
Q

Clinical manifestation of sickle cell disease?

A

Anaemia

Infections - microinfarction of the spleen (risk of spesis+ hyposplenism)

Vasooculssive crisis e.g. ductility’s (swelling + pain of hands + feet from vaso-occlusion) can also affect limbs + spine

Acute anaemia (haemolytic crisis, aplastic crisis or accumulation of sickled cells in spleen or liver)

Priapism (long-lasting erection)

412
Q

Management of acute sickle cell crisis?

A

Analgesia, Fluids, Abx, O2

Transfusion if Acute chest syndrome, stroke or priapism

413
Q

Management of sickle cell disease?

A

Vaccinations - Hib, Meningococcal, Pneumococcal

Daily penicillin + folic acid

Avoid cold, dehydration + hypoxia

414
Q

Management of painful vaso-occlusive crisis or acute chest syndrome?

A

Hydroxyurea - increase HbF production

Bone marrow transplant

415
Q

Neonatal screening test used to confirm sickle cell disease?

A

Guthrie test - part of heel prick test

416
Q

Difference between BT major and intermedia?

A

Major - severe + no HbA

Intermedia - small amount of HbA +/- large amount s of HbF

417
Q

Clinical features of BT?

A

Severe anaemia (need repeated BT)

Splenomegaly

Hepatomegaly

Jaundice

Failure to thrive

Bossing of skull or Maxillary overgrowth

418
Q

Management of BT?

A

Blood transfusion + iron chelation

419
Q

Alpha thalassemia?

A

normally 4 alpha genes

Barts fetalis - no alpha genes

Trait - missing 1 or 2 genes

Hb electrophoresis or Hb high performance liquid chromatography

420
Q

Anaemia of newborn?

A

Rhesus disease

ABO incompatibility (Anti A/B/ Kell)

G6PD, Spherocytosis

Blood loss - haemorrhage/ TT transfusion/ placental abruption

421
Q

Diagnosis of rheusus disease?

A

Positive direct antiglobulin test (coombs)

-ve mama + +ve baby

422
Q

Anaemia of prematurity?

A

Decreased EPO

Decreased RC lifespan

Frequent blood sampling

Iron/ Folic acid deficiency

423
Q

Aplastic anaemia?

A

Bone marrow failure

Anaemia, Thrombocytopenia, Reduced WCC

424
Q

Fanconi anaemia?

A

AR inherited aplastic anaemia

Short stature, abnormal radii, renal malformation, microphthalmia (when 1 or both eyes don’t develop properly), pigmented skin lesions

5 to 6 years old

Increased chromosomal breakage of peripheral blood lymphocytes

425
Q

Complications of Fanconi anaemia?

A

BM failure or transformation to acute leukaemia

426
Q

Shwanchman-Diamond syndrome?

A

AR

BM failure + Pancreatic exocrine failure + skeletal abnormalities

Presents with neutropenia or pancytopenia

increased risk of acute leukaemia

427
Q

useful screening tests for bleeding disorders?

A

FBC + blood film

Prothrombin time - 10752

Activated Partial Thromboplastin time - 2,5, 8-12)

Thrombin time - dysfunction of fibrinogen

Quantitative fibrinogen assay

D-dimer - Fibrin degradation products

Renal + Liver function tests

428
Q

Haemophilia?

A

X linked recessive

A is 8 and B is 9

Severe: random muscle or join bleeds

Moderate: Bleed after minor trauma

Mild: bleed after surgery

Evident wen children start to walk or take long to stop bleeding after blood drawn

429
Q

Management of haemophilia?

A

Recombinent 8 or 9

Desmopressin - 8 + vWf

Avoid IM injection, aspirin + NSAIDS

430
Q
A
431
Q

Function of vW factor?

A

Platelet adhesion or damaged endothemlium

Carrier protein for factor 8 to prevent it from being inactivated and cleared away

432
Q

vWD?

A

AD chromosome 12

Bruising, Prolonged bleeding, Mucosal bleeding (epistaxis + menorrhagia)

Uncommon to cause joint to muscle bleeding

Management: Desmopressin or plasma-derived Factor 8 concentrate

433
Q

Causes of acquired abnormal bleeding?

A

Vit K deficiency

Liver disease

Thrombocytopenia (immune or DIC)

434
Q

Vit K uses?

A

1972

Protein C + Protein S anticoagulants

Prolonged Prothrombin time

435
Q

Immune thrombocytopenia?

A

Destruction of circulating platelets by antiplatelet IgG autoantibodies

Reduce platelet + megakaryocytes in BM

436
Q

Immune thrombocytopenia presentation?

A

Platelet count < 150

2 to 10 years old

1 to 2 weeks after viral infection

Petechiae, purpura, superficial bruising, epistaxis, mucosal bleeding +/- intracranial bleeding

437
Q

Management of immune thrombocytopenia?

A

Diagnosis of exclusion after BM biopsy and rule out SLE

self-limiting within 6 to 8 weeks

Oral prednisolone, IV anti-D, IV immunoglobulins

Platelet transfusion

Rituximab - if chronic

438
Q

DIC?

A

Coagulation pathway activation leading to diffuse fibrin deposition in microvasculature

Post sepsis or shock due to circulatory collapse

439
Q

When to suspect DIC?

A

Bruising, purpura + haemorrhage

Thrombocytopenia, prolonged prothrombin time, Low Fibrinogen, raised D dimer, Low anticoagulants protein C + S

440
Q

Management of DIC?

A

Fresh frozen plasma - replace clotting factors

Cryoprecipitate (fibrinogen than clotting factors)

Platelets

441
Q

Purpura fulminas?

A

DIC + skin necrosis

442
Q

Causative organisms of acute otitis externa?

A

Pseudomonas or staphy A

443
Q

Causative organisms of acute otitis media?

A

RSV

444
Q

FeverPAIN score?

A

Do we give Abx for tonsillitis or nah?

Fever

Purulent tonsillitis

Rapid arrival within 3 days

Inflamed

No cough or coryza

2/5 then 30 to 40% strep

4/5 - 60% strep

445
Q

Epiglottitis?

A

Drooling, Dysphagia, Hot potato voice

446
Q

Test to confirm rhesus disease?

A

Kleihaur

447
Q

OA + TOF?

A

Polyhydramnios - unable to swallow

Bubble, salivation, drool, cyanotic episode after feeding, resp distress, aspiration

NG tube + Xray

448
Q

Double bubble + downs?

A

Duodenal atresia

449
Q

2 periods when testicular torsion peaks?

A

Neonates

Puberty

Increasing testosterone

450
Q

Prehn test?

A

Differentiate between torsion or epididymitis

If pain is relieved when elevating the testes then +ve epididymitis

451
Q

Electrolyte imbalance cause by pyloric stenosis?

A

Hypochloramic Hypokalaemic metabolic alkalosis

452
Q

NEC?

A

Indomethacin or bacteria

Pneumostasis intestinalis/ Intramural air + Dilated bowel loop

453
Q

Cows milk allergy?

A

Bloody stool

Type 1 hypersensitivity

Immediate onset of symptoms

Rhinorrhoea

Itchy nose

Urticaria

Pruritus

454
Q

What is this and how is it managed?

A

Bullous impetigo on 2 week old baby

Staphy A

Systemic Abx - penicillin

455
Q

What is this?

A

Ringworm

Dermatophyte fungi

Kerio - severe inflammatory pustular ringworm

Tinea capitis - scalp

UV light to examine + skin scrapings for fungal hyphae + culture

Topical antifungals + treat animal source

456
Q

What is this?

A

Scabies parasite

8 legged Sarcoptes scabiei

itch for 2 to 6 weeks worse in warm and night

infant - soles, palms + trunk

Skin scrape for mites

Secondary bacterial infection is a risk

Permethrin, Benzyl benzoate emulsion or Malathion

457
Q

What is this?

A

Herald patch - beginning of Pityriasis rosea (trunk, upper arm, neck or thigh)

then other pink macules develop and tends to follow the line of the ribs

Self-resolving within 4 to 6 weeks

458
Q

SJS + toxic epidermal necrolysis?

A

The disproportionate immune response that causes epidermal necrosis resulting in blistering and shedding of the top layer of skin

SJS <10% of the body

TEN >10% of body

459
Q

Causes of SJS?

A

Medications: Antiepilpetics, Abs, Allopurinol. NSAIDs

Infection: HS, Mycoplasma pneumonia, CMV, HIV

460
Q

Clinical presentation of SJS?

A

Fever, Cough, Sore throat, Sore mouth + eyes + itch

Blistering

Skin break away leaving raw tissue

Pain, erythema, blister + shed of lips and mucous membrane

Eyes become inflamed and ulcerated

Can affect the urinary tract, lungs and internal organs

461
Q

Management of SJS + TEN?

A

Remove drug

ALDEN (Algorithm for Drug-Induced Epidermal Necrolysis)

SCORTEN (Score for Toxic Epidermal Necrolysis) - asses prognosis

IV fluids, Mouthwash/ Topical anaesthetics, Ophthalmology assessment

ABx + steroids

Immunomodulation e.g. Ciclosporin, Cyclophosphamide, Infliximab

462
Q

Symptoms of T1DM in children?

A

Polyuria, Polydipsia + Weight loss

463
Q

Signs of DKA?

A

Hyperventiallaiton - Kussmaul breathing

Acetone breath

Vomit

Dehydration

Abdo pain

Shock

Downsines

464
Q

T1DM?

A

Random BG >11.1 mmol/L + Glucosureia + ketonuria

Blood ketones >3mmol/L

Fasting BG >7mmol/L

HbA1C >58mmol/L

465
Q

Symptoms of hypoglycaemia?

A

BG <4mmol/L

Abdo pain, Sweat, Dizziness, Wobbly feeling in legs, Seziures, Coma

Glucogel or glucagon injection

466
Q

Management of DKA?

A

Fluids - Blous, Correct deficit + maintenance dose

Insulin (0.05-0.1 U/kg) then 4% dextrose/0.18 saline after 24 hours when blood glucose 14mmol/L

Potassium

467
Q

Antagonist of insulin?

A

Cortisol, Growth hormone, Oestrogen, Testosterone

  • as children grow they increase how much insulin they will need per day
468
Q

Management of hypoglycaemia?

A

2ml/kg of 10% dextrose followed by 10% dextrose infusion

469
Q

Causes of congenital hypothyroidism?

A

Maldescent of thyroid or failure to develop

Error of thyroid hormone synthesis

Iodine deficiency

TSH deficiency as part of panhypopituitarism

470
Q

Test to identify congenital hypothyroidism?

A

Guthrie test

471
Q

Juvenile hypothyroidism?

A

High risk are downs + Turners syndrome

Autoimmune thyroiditis

Growth failure + delayed bone age + goitre

472
Q

Management of hyperthyroidism?

A

Carbimazole or polythiouracil - interfere with thyroid hormone synthesis

BB - anxiety, tremor + tachy

Sore throat + fever - 999 as risk of neutropenia

473
Q

Hyopopathyroidism?

A

Infant - DiGeorge

Children - Addisons disease

474
Q

Pseudohypoparathyroidism?

A

Resistance to PTH - High PTh, Low Calcium, high phosphate

Short stature, Obesity, short 4 metacarpals, enamel hypoplasia, calcification of basal ganglia

Pseduopsedo - the same but no resistance to PTH

475
Q

Adrenal cortical insufficiency?

A

Causes: Autoimmune, Haemorrhage, Infarction, Congenital, TB, Long term corticosteroid therapy

Low Na + Glucose, High K, Dehydrated, Hypotension, Pigmented skin, Lethargy, Vomit, Growth failure

Short synacthen test - measure cortisol before and after synthetic ACTH and cortisol remains low

Saline, glucose + hydrocortisone but long term glucocorticoid + mineralocorticoid

476
Q

Cushing syndrome?

A

Face + trunk obesity, hirsutism, striae, growth failure, short, hypertension, bruising, osteopenia, infection, muscle wasting

High midnight cortisol + high 24 hour urine free cortisol + failure of cortisol to decrease when given dexamethasone (09:00)

+/- adrenal tumour

477
Q

Heel Prick?

A

Congenital hypothyroidism

Sickel cell

Cystic Fibrosis

Phenylketonuria

Medium-chain Acyl-CoA dehydrogenase deficiency

Homocystinuria

Tyrosinaemia

478
Q

Phenylketonuria?

A

deficiency of phenylalanine hydroxylase

6 to 12 months

musty odour, fair hair, blue eyes, eczema, seizures

Restrict dietary phenylalanine + monitor blood concentrations

479
Q

Homocystinuria?

A

Cystathionine synthetase deficiency

developmental delay, subluxation of the ocular lens, LD, psych, convulsion, Marfan like symptoms, fair + thin hair

Coenzyme pyridoxine + low methionine diet

480
Q

Tyrosinemia?

A

AR Deficiency of fumarylacetoacetate

Accumulation of toxic metabolite causing liver damage and renal tubular damage

NTBC - breaks down tyrosine

481
Q

Galactosaemia?

A

Deficiency in enzyme required for galactose metabolism

Poor feeding, vomit, jaundice + hepatomegaly, cataracts

Chronic liver disease+ developmental delay

482
Q

Hyperlipidaemia?

A

Serum cholesterol >5/3 mmol/L + family history

Or secondary to Hypothyroidism, Obesity, DM, Nephrotic syndrome or obstructive jaundice

483
Q

3 causes of in-toeing in children?

A

Metatarsus varus

Medial tibial torision

Persistent anteversion of femoral neck

484
Q

Out-toeing?

A

Hypermobility, Ehler Danlos, Marfan

485
Q

Developmental Dysplasia of the Hip?

A

Barlow + Ortolani

Ultrasound

Splint or harness to keep the hip flexed

Necrosis of femoral head is complication

486
Q

Growing pains?

A

Episodes of generalised symmetrical pain in the lower limbs

Pain wakes from sleep and made better by massage

3 to 12 years old

Never present at start of day after waking

487
Q

Osteomyelitis?

A

Infection of the metaphysis of long bones from haematogenous spread from an infected wound e.g. distal femur and proximal tibia

swollen skin

Staphy A, Strep or HI

Immobile, painful, acute febrile illness, swelling, warm

Bloods +ve for infection, Normal X ray +/- ST swelling + bone rarefaction, Ultrasound - periosteal elevation

IV Abx + surgical apiration/ debridmenet

488
Q

What can be used to differentiate between osteomyelitis + soft tissue swelling?

A

MRI - show subperiosteal abscess

489
Q

Osteosarcoma

A

10 to 20 year olds

Femur > Tibia > Humerus

Nocturnal bone pain wakes from sleep, swelling, mass, Restricted movement

X rays <48 hours = poorly defines, fluffy + destruction, Periosteal reaction (sub burst)

Bloods = Raised ALP

Surgical resection + limb amputation

490
Q

Complications of osetomyelitis?

A

Septic arthritis

Subperiosteal abscess

Bone necrosis

491
Q

Bone Malignancies

A

Osteosarcoma - most common, Prolonged pain + swelling, mostly knee, periosteal reaction + Codman’s triangle – Full body CT for mets

Ewing - Painful, warm mass on long bone diaphysis (midshaft), fever, anaemia, raised ESR + WCC

Chondrosarcoma - Cartilage malignancy most older people- lytic + fluffy popcorn calcifications

492
Q

Osgood-Schlatter disease?

A

Osteochondritis of patellar insertions at the knee

Males who are very active

Knee pain + Tenderness + Swelling over tibial tuberosity + hamstring tightness

Management: Less activity, Quads muscle exercises, Hamstring stretch

493
Q

Transient synovitis?

A

Acute limp + Irritable hip +/- viral infection

2 to 12 year olds

sudden onset, decreased internal rotation, afebrile, no pain at rest

Managed with bed rest and better within 1 week

Risk of developing Perthes disease

494
Q

Perthes disease?

A

Ischaemia of the femoral head

Avascular necrosis of the capital femoral epiphysis of the femoral head due to interruption of blood supply then revascularization and reossification over 18 to 36 months

Male 5 to 10

Insidious bilateral limp/ hip/ knee pain, Fever, Painful at rest, Hip held flexed

>4 week pain

X-ray - Sclerosis + fragmentation of epiphysis

Management: conservative management or plaster cast keeping hip abducted or osteotomy

495
Q

Slipped capital femoral epiphysis?

A

Displacement of the epiphysis of the femoral head posteroinferior (back + down) with risk of avascular necrosis

10 to 15-year-olds + obese + bilateral

Associated with metabolic endocrine abnormalities e..g hypothyroidism + hypogonadism

Limp + restricted abduction + internal rotation

X-ray

Surgical pin fixation

496
Q

Reactive arthritis?

A

Transient joint swelling <6 weeks of ankles or knees

Enteric, viral or STI

Low grade fever + normal X ray

NSAIDs

497
Q

Septic arthritis?

A

<2 years old

following puncture wound or infected skin lesions or osteomyelitis

Hip

Staphy A or HI

Warm, Tender, Reduced movement, Still limb and cry if moved

Aspirate the joint under ultrasound, Raised WWC + ESR, Blood cultures

Wash out + surgical drainage + IV Abx

498
Q

JIA?

A

>6 weeks

Gelling (stiffness after rest), Morning joint pain + Stiffness, Intermittent limp, Swelling or periarticular soft tissue, Thick synovium

Complications: Chronic anterior uveitis, Growth failure, Anaemia, Osteoporosis, Amyloidosis, Genu valgum, Leg lengthening

Management: NSAID, Joint injections, Methotrexate, Systemic Methylprednisolone, Anti-TNF alpha or other cytokine modulators

499
Q

Achondroplasia?

A

Miss Juicy

AD, Short stature, large head, frontal bossing, depression of nasal bridge, lumbar lordosis

500
Q

Osteogenesis imperfecta?

A

Not enough collagen in bones making them fragile and prone to fractures

BITE - Bones, Eyes, Teeth, ears

AD

Bowing + frequent fractures

Bisphosphonates

501
Q

Discoid meniscus?

A

When lateral meniscus remains round instead of becoming cup-shaped

502
Q

Prophylaxis of migraines?

A

Pizotifen, Propanolol, Topiramate

503
Q

Signs of raised ICP?

A

Headahce worse when laying down

Morning vomit

Change in mood or personality

Papilloedema

504
Q

Febrile seizures?

A

seziure + feve4r

6 months to 6 years

Bucccal mid or rectal di

505
Q

Causesof funny turns?

A

Breathe holding after tantrum

Reflex anoxic seizure

Syncope

migraine

Benign paroxysmal vertigo (viral labrythianitis)

506
Q

Reflex anoxic seizure

A

sizure folliwng head trauma, cold, fright or fever

Plae then seizure

Cardiac asystole from vagal inhibition

Brief and rapid recovery

507
Q

Focal seizure types + presentations?

A

Frontal - motor + clonic - jacksonian march

Temporal - aura, altered taste or smell + lip smacking

Occipital - distorted vision

Parietal - contralateral altered sensation or distorted body image

508
Q

Type of generalised seizures?

A

Abscence - day dream, eye flicker, abrupt onset and termination

Mycolonic - isolated movemnts of limb, neck or trunk

Tonic-clonic - typical tonic, cyanosis, clonic, tongue bite + incontinece + post-ictal state

Atonic - myclonic the loss of muscle tone then drop like bricks

509
Q

EEG absence seizure?

A

3/second generalised polyspike + bilaterally synchronous

510
Q

Juvenile Myoclonic Epilepsy?

A

mycolonic/ tonic-clonic of the arms after waking up

3 to 6Hz generalised polyspike

Triggered by lack of sleep, not taking meds, alcohol, flashing lights

Management: Valproate or Lamotrigene

511
Q

Management of generalised and focal seizures?

A

Generalised

  • 1st line majory Valproate and 2nd line Lamotrigene

TC - V, C

A - V, E

Focal

  • Carbamazepine, Valproate, Lamotrigene
512
Q

Side effects of Valproate?

A

Weight gain and hair loss

513
Q
A

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