Paediatrics Part 1

Question 1

What causes a wheeze

Answer 1

Obstruction of airflow within the thorax (heard in expiration)

Question 2

What is stridor

Answer 2

Noise heard during inspiration

Question 3

What causes stridor

Answer 3

Fixed extra thoracic airway obstruction

Question 4

Investigations for Asthma

Answer 4

1. Peak Expiratory Flow Rate < 80% predicted for height
2. FEV1/FVC <80% predicted
3. Concave scooped shape in flow volume curve
4. Bronchodilator response to beta agonist therapy (15% increase in FEV1 or PEFR)
5. Spirometry
6. FeNO
7. Blood eosinophil levels
8. IgE

Question 5

What is FEV

Answer 5

How much air a person can exhale during a forced breath in one second

Question 6

What is FVC

Answer 6

Total amount of air exhaled during the FEV test

Question 7

Name two short acting beta two agonists

Answer 7

Salbutamol, terbutaline

Question 8

Name two long acting beta-2 agonists

Answer 8

Salmeterol, formoterol

Question 9

Name a short-acting anticholinergic

Answer 9

Ipratropium Bromide

Question 10

Name an inhaled corticosteroid

Answer 10

Budesonide

Question 11

Name an oral steroid used in acute asthmatic attakcs

Answer 11

Prednisolone

Question 12

Is serum IgE measured in an asthma diagnosis

Answer 12

No

Question 13

Treatment pathway for asthma

Answer 13

1. Short acting b2

Step 2: Short acting b2 + low dose ICS

Step 3: Short acting b2 + high dose ICS or low dose inhaled steroid + long acting bronchodilator

Step 4: Theophyllines or ipratropoum +/- alternate day steroid.

Question 14

How is acute severe asthma treated in children >5

Answer 14

Sabultamol via nebuliser

Oral Prednisolone (30-40 mg)

Assess after 15 mins and repeat - then ADMISSION

Question 15

How is acute life-threatening asthma treated

Answer 15

1. Ipratropium via nebuliser (salbutamol 5mg and 0.35mg ipratropium every 20 mins)
2. Oral Prednisolone or IV Hydrocortisone

Repeat and hospital admission

Question 16

Acute severe asthma vs life threatening

Answer 16

PEF: 33-50% vs <33%

Breathless to speak vs silent chest

Question 17

Why is FENO measured

Answer 17

Produced by eosinophils in the lung alveoli

Question 18

When are long acting b2 agonists used

Answer 18

As prophylaxis

In patients on inhaled budesonide or beclometasone (400 msg.day)

Question 19

What type of hypersensitivity reaction is asthma

Answer 19

Type 1

Question 20

When is the value of FEV1 normal

Answer 20

When it is 80% of the normal predicted value of the patient

Question 21

What indicates airways restriction

Answer 21

1. Ratio of FEV1/FVC is greater than 0.7 AND FVC is lower than 80% of predicted value
2. Or just a low FVC

Question 22

What indicates airways obstruction

Answer 22

1. FEV1/FVC < 0.7 (in other words, they can breathe out fast but obstruction stops them from expelling Total capacity)

Question 23

Define type I respiratory failure

Answer 23

Hypoxia but not hypercapnia

Question 24

Genetic factors in asthma

Answer 24

ADAM33

Question 25

Three characteristics of asthma

Answer 25

1. Airflow limitation 2. Airway hyper repsonsiveness 3. Bronchial inflammation

Question 26

RF for asthma

Answer 26

1. Atopy 2. Family history of asthma and atopy 3. Obesity 4. Premature birth

Question 27

Pathophysiology of asthma

Answer 27

1. Inflammed airways react to triggers 2. Airways narrow and produce excess mucous making it difficult to breathe 3. Bronchi spasms 4. Inflammation further narrows airways -> coughing

Question 28

A child has an X-Ray after a bout of choking, however nothing is seen on the X-Ray despite indications of airway obstruction. What is the likely diagnosis

Answer 28

Aspiration of food (food is radiolucent) Objects are radiopaque

Question 29

Investigations for foreign body aspiration

Answer 29

1. Indirect signs of obstruction (subglottic density or swelling) 2. CT Scan 3. Laryngoscopy to visualise objects 4. Nasal speculum

Question 30

Signs of foreign objects in the nose

Answer 30

1. UNilateral, foul-smelling rhinorrhoea | 2. Epixstasis (nose bleeds)

Question 31

Treatment of foreign objects in the nose

Answer 31

1. Blow nose while plugging unobstructed nostril | 2. Positive pressure ventilation through the mouth and blocking unobstructed nostril

Question 32

What is laryngomalacia

Answer 32

1. Congenital abnormality of the laryngeal cartilages

Question 33

What islaryngomalacia

Answer 33

1. Shortened aryepiglottic folds turn larynx into an omega shape, obstructing airflow

Question 34

What is contained within the larynx

Answer 34

The Vocal Cords

Question 35

Symptoms of laryngomalacia

Answer 35

Inspiratory stridor (worse lying supine) Breathing difficulties

Question 36

Diagnosis of laryngomalacia

Answer 36

Laryngoscopy or Bronchoscopy

Question 37

Onset of laryngomalacia

Answer 37

few weeks old, self corrects 12-18 months

Question 38

No infectious causes of rhinitis

Answer 38

1. Allergens 2. Irritants (cold, dry air, smoke) 3. Emotional Stress 4. Excercise

Question 39

Symptoms of rhinitis

Answer 39

1. Nasal Congestion 2. Sneezing 3. Sniffling 4. Allergic Salute 5. Conjunctivitis (e.g., allergic shiners)

Question 40

Diagnosis of rhinitis

Answer 40

Symptoms History of Atopy Skin test for specific allergens Specific IgE measurements

Question 41

Treatment of rhinitis

Answer 41

Avoid allergens Antihistamines Montelukast Intranasal steroids

Question 42

What is montelukast

Answer 42

Leukotriene receptor antagonist = relaxation of smooth muscles and reduces inflammation

Question 43

Causes of infectious rhinitis

Answer 43

Virus 1. Rhinovirus 2. Influenza virus 3. RSV 4. Parainfluenza 5. Adenovirus Bacteria: 1. Strep pneumonia 2. H. influenzae 3. Moraxella cattarhalis 4. Staph aureus Fungi: Aspergillus in immunocompromised

Question 44

What is rhinosinusitis

Answer 44

Bacteria and fungi can spread to the sinuses and cause inflammation there too

Question 45

What is the only sinus present at birth

Answer 45

Ethmoid

Question 46

When do maxillary sinuses develop

Answer 46

1 month - 1 year of age

Question 47

When to sphenoid sinuses develop

Answer 47

1-2 years of age

Question 48

When do frontal sinuses devleop

Answer 48

After age 10

Question 49

Symptoms of rhinitis

Answer 49

1. Low grade fever 2. Low apetite 3. Congestion 4. Sneezxing 5. Nasal discharge

Question 50

Rhinitis vs rhinosinusitis

Answer 50

Low fever vs high grade fever Nasal Discharge > 10 days Facial pain or pressure Voice change

Question 51

Diagnosis of infectious rhinitis

Answer 51

1. Symptoms | 2. Facial X-Ray or CT Scan

Question 52

Treatment of rhinitis

Answer 52

Rest Fluid Nasal Irrigation

Question 53

Treatment of bacterial rhinosinusitis

Answer 53

Amoxicillin 10 days

Question 54

Onset of pharyngitis

Answer 54

3-14

Question 55

Where do pharyngitis infections typically spread to

Answer 55

The Tonsils (tonsillopharyngitis)

Question 56

Symptoms of Viral Tonsillopharyngitis

Answer 56

1. Low grade fever 2. Sore Throat 3. Voice Hoarseness 4. Symptoms of the common cold (nasal congestion, conjunctivitis, cough)

Question 57

Diagnosis of viral tonsillopharyngitis

Answer 57

1. Erythema and oedema of pharynx and tonsils 2. No Exudates 3. Non-Tender Anterior cervical lymphadenopathy

Question 58

Tonsilopharyngitis vs strep throat

Answer 58

Low grade fever vs high grade fever 2. Sore throat in both 3. Voice hoarseness in both 4. common cold symptoms vs none

Question 59

Diagnosis in viral tonsillopharyngitis vs strep throat

Answer 59

Erythema and oedema of pharynx and tonsils in both No exudates vs Grey-white exudates Non-tender anterior cevical lymphadenopathy vs tender anterior cervical lymphadenopathy

Question 60

What criteria is used to identify strep throat

Answer 60

Centor Criteria: C - can't cough E - exudates N - Nodes (tender lymph) T- Temperature > 38 degrees OR - Age 3-14 (1) 15-44 (0) >44 (-1) 2-3 (test for group a strep)

Question 61

Testing for group A strep

Answer 61

1. RADT (rapid antigen detection testing) | 2. Throat culture

Question 62

Treatment of Group A strep

Answer 62

1. Penecillin V | 2. Amoxicillin

Question 63

Bacterial tonsillopharyngitis in Children > 10 vs <10

Answer 63

>10: Peritonsiloar abscess <10: Retropharyngeal abscess

Question 64

Symptoms of tonsilopharyngitis

Answer 64

1. High Fever 2. Drooling 3. Dysphagia 4. Odynophagia (painful chewing) 5. Muffled voice

Question 65

Peritonsilar abscess vs retropharyngeal abscess symptoms

Answer 65

Trismus (difficulty opening the mouth) vs Torticolis (stiff neck)

Question 66

Signs of bacterial tonsillopharyngitis upon examination

Answer 66

1. Enlarged tonsils that move the uvula sideways | 2. Bulge in posterior pharyngeal wall

Question 67

Diagnosis of bacterial tonsillopharyngitis

Answer 67

1. Clinical | 2. X-ray or CT scan to visualise the abscess

Question 68

Treatment of tonsillopharyngitis

Answer 68

1, Incision and drainage 2. Ampicillin

Question 69

Where does a laryngitis infection spread to

Answer 69

Laryngotrachobronchitis (CROUP)

Question 70

Onset of CROuP

Answer 70

6 months - 3 years of age

Question 71

Cause of croup

Answer 71

1. Parainfluenza 2. RSV 3. Adenovirus 4. Infleuzna

Question 72

Symptoms of croup

Answer 72

1. Prodrome fever and nasal congestion (as it spreads) 2. Barking cough 3. Hoarse voice 4. Inspiratory stridor

Question 73

Diagnosis of corup

Answer 73

Anterior neck X-Ray

Question 74

Sign found for croup on an X-Ray

Answer 74

Subglottic narrowing (steeple sign)

Question 75

Treatment of Corup

Answer 75

Dexamthesone Cool, humidified air, antipyretics Repeat doses of racemic epinephrine

Question 76

Causes of bacterial laryngitis

Answer 76

1. Group A strep 2. Strep pneumonia 3. H influenzae Have a preference for superior portion of the larynx and epiglottis

Question 77

Symptoms of epiglottitis

Answer 77

1. Rapid onset of high fever 2. Sore Throat 3. Hot potato voice 4. Severe ARDS 5/ Tripoding to keep epiglottis open

Question 78

Diagnosis of epiglottiitis

Answer 78

1. Lateral neck x-ray

Question 79

Treatment of epiglottitis

Answer 79

EMERGENCY Intubation or Tracheostomy Antibiotics: Ceftriaxone

Question 80

What lung is usually affected by foreign body aspiration and why

Answer 80

Right, because the right bronchi are wide and more vertical.

Question 81

Non-specific symptoms to foreign body aspiration

Answer 81

1. SUDDEN onset of SOB 2. Coughing 3. Gagging 4. Choking 5. Drooling

Question 82

Investigation findings in foreign body aspiration

Answer 82

Diminished breath sounds

Question 83

What anatomical structure usually causes stridor (hint: extrathoracic noise)

Answer 83

Trachea (usually when partially obstructed)

Question 84

What anatomical structures usually cause expiratory wheezing

Answer 84

1. Intrathoracic trachea 2. Bronchi 3. Bronchioles

Question 85

Where will the wheeze be heard

Answer 85

Localised the blocked area

Question 86

In what aspiration cases would no wheeze on stridor be heard

Answer 86

When there is complete obstruction (noises only occur with partial obstruction)

Question 87

Investigations for foreign body aspiration

Answer 87

1. Neck or Chest Radiograph 2. Chest CT 3. Bronchoscopy to visualise and remove the object 4. If it cannot be removed - THORACOTOMY

Question 88

As food can be radiolucent, what other signs can we see on a neck or chest radiograph for foreign body aspiration

Answer 88

1. Complete Obstruction -> Atelectasis (collapse, partial or complete of the entire lobe) distal to obstruction as its blocking the airway 2. Partial Obstruction -> Focal Hyperlucency + reduced pulmonary marking distal to obstruction as its blocking the airway like a one way valve.

Question 89

Symptoms of Asthma

Answer 89

1. Dry Cough 2. Chest Tightness 3. SOB

Question 90

What is characteristic about wheeze findings in Asthma

Answer 90

1. Wheeze is not localised, it happens ALL over both lungs.

Question 91

Diagnosis of Asthma

Answer 91

1. CXR: - Normal but may show diffuse hyper lucency due to airway entrapment PFTs: Decreased FEV1 to FVC FBC: Eosinophils

Question 92

What kids are affected by acute bronchitis

Answer 92

Children UNDER 2

Question 93

What viruses cause acute bronchitis

Answer 93

1. Influenza A and B 2. Parainfluenza 3. Adenovirus 4. RSV 5. Rhinovirus 6. Coronavirus

Question 94

What bacteria can cause acute bronchitis

Answer 94

1. Bordatella pertussis 2. Mycoplasma Pneumoniae 3. Ch;amidyia pneumoniae

Question 95

Symptoms of Acute Bronchitis

Answer 95

Upper respiratory tract infection: 1. Congestion 2. Sore Throat 3. Low Grade Fever Lower tract: GOLD: Cough lasting more than 5 days

Question 96

Ausculattion sounds for acute bronchitis

Answer 96

1. Scattered Rhoncii

Question 97

What is rhoncii

Answer 97

Low pitched wheezing sounds caused by lung secretions

Question 98

In what conditions is rhoncii heard in

Answer 98

COPD 2. CF 3. Bronchiectasis 4. Pneumonia

Question 99

Three stages of whooping cough

Answer 99

Whooping cough is a BRONCHIAL disease: Catarrhal (1-2 weeks): Mild fever, cough and coryza (cold) Paroxysmal (2-6 weeks): Violent coughing characteristic of whooping cough (inspiration causes whooping noise) Convalescent (2-4 weeks): Lessening symptoms that take a whole month to reolve

Question 100

What other diseases can cause a whooping cough-like syndrome

Answer 100

Chlamidyia pneumonia, adenovirus or mycoplasma pneumonia.

Question 101

Examination of whooping cough

Answer 101

Eyes: Subconjunctival haemorrheages CXR: Blood counts: Leucocytosis and lymphocytosis Prenatal swab: bordatella pertussis

Question 102

Treatment for acute bronchitis

Answer 102

1. Just rest and fluid

Question 103

Age range of acute bronchiolitis

Answer 103

6 months to 2 years

Question 104

What virus causes acute bronchiolitis

Answer 104

RSV (MAINLY)

Question 105

Symptoms of acute bronchiolitis

Answer 105

1. URTI symptoms: congestion, sore throat, cough and low grade fever 2. Respiratory Distress: - feeding difficulties - Episdoes of apnoea - 70 breaths/ min (younger than 12 months) - 50 breaths/min (older children) - O2 saturation < 92% - Nasal Flaring - Intercostal, subcostal and suprasternal retractions. - Cyanosis - Lethargic or dehydrated

Question 106

Risk Factors for Acute Bronchiolitis

Answer 106

1. Being PREMATURE 2. Younger than 12 weeks 3. Low birth weight 4. Didn't breastfeed 5. Chronic pulmonary disease 6. Congenital heart disease 7. Immmunodeficiency 8. Neurologic disease that impairs sputum clearance.

Question 107

Investigations for acute bronchiolitis

Answer 107

1. Pulse Oximetry 2. CXR: Hyperinflation, atelectasis and consolidation 3. Nasopharyngeal swab for rapid antigen testing for RSV

Question 108

Management of acute bronchiolitis

Answer 108

1. Oxygen to push over 92% 2. Tachypnoea - use an NGT 3. Nebulised adrenaline for wheeze 4. Mechanical ventilation

Question 109

Prophylaxis for acute bronchiolitis

Answer 109

1. Palivizumab (monoclonal antibody for RSV) in those at risk IM.

Question 110

Onset of early onset Neonatal pneumonia

Answer 110

First 3 days of life.

Question 111

Three main causes of early onset neonatal pneumoniae

Answer 111

1. Group B 2. Escherichia Coli 3. Listeria Monocytogenes

Question 112

Three main causes of late onset neonatal pneumonia (3rd day to 3rd week)

Answer 112

1. Strep pneumoniae 2. Staph aureus 3. Strep pyogenes 4. Adenovirus, parainfluenza, influenza and enteroviruses.

Question 113

Three main causes of 3 weeks to 6 months pneumonia

Answer 113

1. RSV 2. Parainfluenza 3. Adenovirus 4. Chlamidyia trachoma's

Question 114

What distinguishes a C. pneumoniae infection from other strains

Answer 114

1. Afebrile pneumonia of infancy

Question 115

Three main causes of pneumonia from 6 months to 5 years

Answer 115

1. RSV 2. Influenza A and B 3. Parainfluenza

Question 116

Most common cause of pneumonia in children over 5

Answer 116

Atypical bacteria and strep pneumonia

Question 117

How to differentiate between atypical and strep pneumonia

Answer 117

1. Atypical bacteria causes diffuse pneumonia | 2. Strep pneumoniae causes consolidated pneumonia

Question 118

If a child has travelled and symptoms that last a few weeks, what else should e expected other than pneumonia

Answer 118

1. TB

Question 119

If a child has recurrent pneumonias or associated GI symptoms, what should be suspected instead of pneumonia

Answer 119

CF

Question 120

If a child has the symptoms for pneumonia but has exposure to farm animals as a history, what should be suspected

Answer 120

Q Fever, caused by Coxiella Burnetti

Question 121

If a child is exposed to Mouse droppings, what pneumonia strain are they suspectible to

Answer 121

Hantavirus

Question 122

Three types of fungal pneumonias found in children

Answer 122

1. Coccidiodomycosis 2. Histoplasmosis 3. Blastomycosis

Question 123

Symptoms of pneumonia

Answer 123

Younger: 1. Fever 2. Poor Feeding 3. Lethargy 4. Apnoea Older: 1. Fever 2. Cough 3. Tachypnoea Infection near pleural surface: Pleuritic chest pain

Question 124

Symptoms seen in bacterial pneumonia vs viral pneumonia

Answer 124

Sudden vs Gradual More Severe vs Mild No Upper respiratory symptoms vs upper airway symptoms. Focal bronchial breath sounds vs diffuse, bilateral sounds. Crackles in both

Question 125

Auscultation signs for lobar pneumonia

Answer 125

1. Dullness to percussion 2. Crackles 3. Decreased breath sounds 4. Tactile vocal remits 5. Bronchial Breathing

Question 126

What is tactile vocal remits

Answer 126

Increased chest vibrations from talking

Question 127

Pneumonia investigations

Answer 127

1. Sputum 2. Nasopharyngeal aspirate for viruses in infants 3. Blood culture 4. CXR (not routine and only in critical illness) 5. Pleural fluid aspirate culture and antigen testing 6. Viral titres

Question 128

Antibiotic therapy for pneumonia by age

Answer 128

<5: Strep pneumonia: Amoxicillin or Co-amoxiclav >5: Amoxicillin for mycoplasma pneumoniae For staph aureus: Amoxicillin with flucloxacillin Sevre: Co-amoxiclav and cefotaxime

Question 129

What is the curb-65 criteria for pneumonia

Answer 129

C - Confusion U - Urea > 19mg/DL R - Resp Rate > 30 B - Systolic BP <90 or Diastolic < 60 65 - Age

Question 130

Complications of pneumonia

Answer 130

1. Parapneumonic effusion from inflammation | 2. Cavitation (empyema) as the infection is localised and has been walled off by the immune system

Question 131

Atelectasis vs pneumonia

Answer 131

X Rays: Wedge-Shaped Consolidation with Apex at Hilum Ipsilateral shift of structures due to volume loss X Rays: Normal or increased volume Conslidation with no apex at hilum No shift in structure

Question 132

Lab Tests in pneumonia

Answer 132

1. Elevated WBC 2. Elevated CRP (more elevated in bacteria than viral) 3. Blood cultures

Question 133

Treatment of empyema

Answer 133

Urokinase via the chest drain (40,0000 U 12-hourly for three days

Question 134

Management of TB

Answer 134

2 Months: Isoniazid, Rifampicin and Pyrazinamide THEN 4 months: Isoniazid and Rifampicin

Question 135

Pulmonary feature of a child with HIV

Answer 135

Lymphoid Interstitial Pneumoniitis - responds to steroids

Question 136

What types of immunodeficiency can cause recurrent infection of the lower airway

Answer 136

1. IgA deficiency 2. IgG subclass deficiency 3. Defective cell-mediated immunity

Question 137

Diagnosis of COVID-19

Answer 137

1. PCR, rapid antigen testing 2. Oximetry (<91%) 3. Clinical Frailty Scale

Question 138

Types of Non-Invasive Ventilatory support for COVID-19

Answer 138

1. High-Flow Nasal Oxygen 2. Continuous positive airway pressure 3. Non-Invasive ventilation (masks)

Question 139

What corticosteroid is given to COVID patients

Answer 139

Dexamethasone for 10 days

Question 140

Treatment of COVID-19 pneumonia in adults

Answer 140

Remdesivir for 5 days

Question 141

Second line treatment for COVID-19 (corticosteroids)

Answer 141

Tocilizumab

Question 142

How long does it take for a neonate to have their first meconium

Answer 142

48 Hours

Question 143

A neonate has not passed meconium within 48 hours, presenting with abdominal distention and refusing to feed. Name three differentials

Answer 143

Imperforate Anus Meconium Ileus Hirschsprung Disease

Question 144

What is imperforate Anus

Answer 144

A non-patent or tiny anal opening Sometimes there could be a vesicorectal fistula causing drainage of meconium through the pneis.

Question 145

What syndrome is associated with an imperforate anus

Answer 145

VACTERL SYNDROME ``` Vertebral Defects Anal Atresia Cardiac Anomalies Tracheoosophageal Fistula Esophageal Atresia Renal Anomalies Limb Anomalies ```

Question 146

Diagnosis of VACTERL Syndrome

Answer 146

USS of spine, heart, abdomen and Limbs NG Tube and Chest X Ray for tracheoosophageal fistula and oesophageal atresia

Question 147

Treatment of VACTERL syndrome

Answer 147

Decompression and Aspiration through an NG tube - IV Fluids - Nil By Mouth Perineal Anoplasty - low types Colostomy - high type

Question 148

What is Meconium Ileus

Answer 148

A sign of cystic fibrosis - where the meconium gets stuck in the terminal ileum. Might be associated with distended abdomen, tacky, fever if perforated.

Question 149

Diagnosis of Cystic Fibrosis

Answer 149

Abnormal Sweat Test | 2 CF-Related Mutations

Question 150

Investigations for meconium Ileus

Answer 150

DRE: Empty Rectom with No Meconium Surgical Emergency as it can lead to Bowel Perforation -> peritonitis -> sepsis -> death Abdominal X-Ray: 1. Dilated bowel loops proximal to the obstruction. Soap Bubble appearance.

Question 151

Sign of meconium peritonitis on an abdominal x ray

Answer 151

Calcified intraperitoneal meconium

Question 152

Treatment of meconium Ileus

Answer 152

Decompression - Hypertonic contrast enema to break up meconium Laparotomy Perforation = Surgery.

Question 153

What is Hirschsprung's disease

Answer 153

1. Meissner's plexus and Auerbach's plexus are responsible for smooth muscle contraction and relaxation are missing -> stopping function of the rectum and distal sigmoid colon. Meconium builds up at the sigmoid and rectum -> megacolon -> perforation.

Question 154

Diagnosis of Hirschsprung disease

Answer 154

Recta Examination -> tight sphincter and explosive release of gas and stool. Anorectal manometry (dilation of the sphincter with a balloon) Biopsy to see reduced plea in the mucosa and submucosaa

Question 155

Treatment of Hirschsprung's

Answer 155

Resection of ganglionic part of colon and reattachment to the rectum

Question 156

What factors can lead to constipation

Answer 156

Dietary: Low fibre Decreased fluid Behavioural: Anxiety Fear of painful stools Embarrassment

Question 157

What is the problem if a child doe snot poo

Answer 157

Water continues to be reabsorbed -> hard stools/

Question 158

What medications can cause constipation

Answer 158

1. Anticholinergic | 2. Iron supplements

Question 159

What conditions can cause constipation

Answer 159

1. Hypothyroidism 2. Renal failure 3. Hypercalcaemia and Hypokalaemia 4. Spina Bifida and Inflammatory Bowel Disease

Question 160

Examination findings in constipation

Answer 160

1. Anal fissures (might be withholding during pain) | 2. Stool in left abdomen

Question 161

Investigations for constipation

Answer 161

1. TFTs, BUN and creatinine, Calcium and K+, CRP for inflammatory bowel disease 2. Abdo X ray or USS 3. Spinal X Ray or MRI

Question 162

Treatment of functional constipation

Answer 162

1. Disimpaction using a rectal thermometer 2. Rectal Glycerin 3. Prune Juice or osmotic laxatives (propylene glycol) or through an NG tube

Question 163

Diet modifications for constipation

Answer 163

1. Increase fruit, vegetables and whole grains | 2. Ensuring adequate fluid intake.

Question 164

What structure marks if it is an Upper or Lower GI bleeding

Answer 164

Above the ligament of Treitz (suspensory ligament of the duodenum): Oesophagus, stomach and duodenum Below the ligament of treats: small intestines, large intestines and rectal

Question 165

WHAT ARE OCCULT GI BLEEDS

Answer 165

NO VISIBLE EVIDENCE OF A BLEED: detected by a faecal occult blood test

Question 166

Symptoms of Upper GI bleeding

Answer 166

1. Haematamesis - Fresh blood indicating ongoing bleed | 2. Melena (small amounts of blood that's passed through the whole GI system)

Question 167

What is the significance of coffee ground like bleeds in upper GI bleeds

Answer 167

Blood has oxidised and bleeding has stopped (old blood)

Question 168

Symptoms of lower GI bleeds

Answer 168

1. Haematochezia (fresh blood through anus).

Question 169

First management step in a child with an active bleed complaint

Answer 169

1. Evaluate general appearance (activity, tone, level of consciousness and skin colour, haemodynamic instability)

Question 170

Symptoms of mild hypovolaemia

Answer 170

1. Slightly dry mouth 2. Increased thirst 3. Slightly reduced urine output

Question 171

Symptoms of moderate hypovolaemia

Answer 171

1. Significantly reduced urine output 2. Tachycardia 3. Orthostatic Hypotension 4. Reduced skin turgour 5. Dry Mucous Membranes 6. Irritability 7. Delayed Cap refill (2-3 seconds) 8. Deep rests 9. Indants -> Sunken open fontanelle

Question 172

Symptoms of severe hypovolaemia

Answer 172

1. Anuria 2. Hypotension 3. Cap refill > 3 secs 4. Cool and Mottled Extremities 5. Lethargy 6. Deep Resps

Question 173

What liver functions are done for GI bleeds

Answer 173

``` ALT AST GGT Bilirubin Albumin ``` Coagulation studies: Fibrinogen, PT, PTT, INR Nasogastric Lavage

Question 174

In well-appearing children, what investigations might be done in a Upper GI bleed

Answer 174

1. Upper Endoscopy

Question 175

In well-appearing children, what investigations might be done in a Lower GI bleed

Answer 175

1. Colonoscopy

Question 176

In ill-appearing children, what initial investigation must be done in a or Upper Lower GI bleed

Answer 176

1. 2 Peripheral IV Catheters for fluid resuscitation | 2. Blood Transfusion

Question 177

Signs of fluid overload

Answer 177

1. Crackles 2. Gallop Rhythm 3. Hepatomegaly

Question 178

What is the most common cause of GI bleeds in neonates (<1 month)

Answer 178

1. Maternal Blood Ingestion Swallowed during delivery or from fissures during breast feeding

Question 179

How can we check for maternal blood in neonate's faeces

Answer 179

Apt-Downy Test: Haemolysis of newborn's stool + NaOH -> Maternal Blood if yellow If the same = foetal haemoglobin Spetcrophotometric Assay: shows composition of maternal blood

Question 180

What can cause anal fissures

Answer 180

1. rectal Thermometers 2. Birth Injuries 3. Sexual Abuse 4. Constipation

Question 181

Where are anal fissures often found

Answer 181

Posterior Midline (MOST OF THE TIME)

Question 182

What is alarming about a lateral fissure

Answer 182

They're usually caused by infections rather than trauma: Syphilis, HIV or TB

Question 183

Treatment of anal fissure

Answer 183

1. Heal on own | 2. Frequent diaper changes

Question 184

What can cause food protein-induced proctocolitis

Answer 184

1. Cow's Milk 2. Soy Proteins In Infant formula's or even through breast milk if mother's been drinking it

Question 185

Symptoms of food protein-induced allergic proctocollitis

Answer 185

1. Irritability 2. Back Arching during feeding 3. Atopic Dermatitis 4. Urticaria 5. Wheezing 6. Coughing 7. Vomiting

Question 186

Diagnosis of food protein-induced allergic proctocollitis

Answer 186

1. In breastfeeding women: Stop mother from drinking milk 2. In formula feeding: Elemental formulas only with no cow's milk or soy milk Then reintroduce cow/soy after 7 days. If it continues, this is a milk allergy and should be stopped again

Question 187

What can cause GI bleeds if the neonate appears ill and has signs of haemodynamic instability

Answer 187

Necrotising enterocolitis: where the portion of the intestines becomes ischaemic and dies from inflammation from pathogens

Question 188

Where does necrotising enterocolitis usually infect

Answer 188

Distal small bowel and the ascending colon and caecum

Question 189

What babies are most vulnerable to necrotising enterocolitis

Answer 189

Premature babies < 1500 g

Question 190

RF for formula-feeding

Answer 190

1. Loss of protective immunity and bacteria causing flora to be weakened

Question 191

Onset for necrotising enterocolitis

Answer 191

First week of life

Question 192

Symptoms for necrotising enterocolitis

Answer 192

1. Poor Feeding 2. Bloating 3. Bilious Vomiting 4. Bloody Stools

Question 193

What is seen on a physical exam for necrotising enterocolitis

Answer 193

1. Abdominal distention 2. Right lower quadrant mass 3. Abdo wall erythema 4. Tenderness 5. Decreased bowel sounds 6. Visible intestinal loops SHOCK

Question 194

Investigations for necrotising enterocolitis

Answer 194

1. Abdominal Radiograph showing multiple loops of dilated bowel Pathogonomic pneumatosis intestinalis (air in the bowel wall from bacteria) -> extends to portal veins

Question 195

Diagnosis of necrotising Enterocolitis

Answer 195

1. Hyponatraemia due to third spacing of fluid 2. Metabolic acidosis (lactic acid build up from ischaemic bowels) 3. Thrombocytopenia (DIC) 4. Neutropenia (sepsis) Blood cultures for causative pathogens

Question 196

What criteria is used to classify necrotising enterocolitis

Answer 196

Bell's criteria

Question 197

Treatment of necrotising enterocolitis

Answer 197

1. Discontinue enteral feeds and switch to parenteral nutrition (Central venous catheter, gastric decompression w/a NG tube) 2. Benzylpenecillin, metronidazole and gentamicin 3. Systemic Support: Assisted ventilation Fluid replacements, inotropes

Question 198

What is intestinal malrotation with volvulus

Answer 198

Twisting of the mesentery around the mesenteric artery

Question 199

Symptoms of Intestinal Malrotation with volvulus

Answer 199

1. Acute, bilious vomiting 2. Abdo distention 3. Bloody diarrhoea 4. Peritonitis/ sepsis

Question 200

Diagnosis of Intestinal Malrotation with volvulus

Answer 200

1. USS or abdo x ray in upright position: Distended loops of bowel with air fluid levels 2. Upper GI contrast series: Spiral shaped duodenum in right lower quadrant instead of left upper quadrant

Question 201

Treatment of intestinal malrotation with volvulus

Answer 201

1. Stop feeding 2. FLuid resus w normal saline 3. NG tube decompression 4. Vancomycin or Cephalosporins (broad spectrum)

Question 202

Surgical procedures to untwist the volulus

Answer 202

IMMEDIATE laporotomy LADD's procedure: straightening out the intestines

Question 203

What conditions cause GI bleeds between 1 month and 6 years

Answer 203

1. Infectious Colitis 2. Meckel's Diverticulum 3. Intussusception Rarely: Peptic Ulcer disease and juvenile polyposis

Question 204

What organisms can cause infectious colitis

Answer 204

1. Shigella 2. Salmonella 3. Yersinia 4. Campylobacter 5. E. coli 6. Clostridium Difficile. CMV Entamoeba histolytica

Question 205

Symptoms of Infectious colitis

Answer 205

1. Acute, bloody diarrhoea 2. fever 3. Abdo Pain 4. Cramps 5. Ill contacts and history of eating undercooked food or takeaways

Question 206

What complication can result from E. coli

Answer 206

Haemolytic Ureamic Syndrome

Question 207

What is HUS

Answer 207

Microangiopathic haemolytic anaemia Thrombocytopenia Renal Failure

Question 208

What organisms can cause infections from eggs or chicken

Answer 208

Salmonella or Campylobacter

Question 209

What organism can cause infections from milk or pork

Answer 209

Yersinia

Question 210

What organism can cause infections from ground meat, cattle and goats

Answer 210

E coli

Question 211

Where can anteamoeba hystolitca infections arise

Answer 211

1. Recent travel to endemic countries and poor sanitation

Question 212

What organism can cause infections from reptiles

Answer 212

Salmonella

Question 213

Diagnosis for infectious colitis

Answer 213

1. Stool Cultures 2. PCR 3. Microscopy for parasites 4. Rapid stool assay for shigella 5. Blood CUlture 6. Check for hypokalaemia

Question 214

What would be found in HUS

Answer 214

1. Increased BUN 2. Creatinine 3. Schistocytes 4. Haematuria

Question 215

Treatment for Infectious colitis

Answer 215

1. Rehydration (especially for HUS) IV if unable to hold down fluids Oral Rehydration Solution otherwise

Question 216

If the child has no improvements after 24 hours of Rehydration or Persistent diarrhoea, what should be done

Answer 216

Admission to hospital

Question 217

What diseases can infectious colitis mimic

Answer 217

Crohn's and UC

Question 218

What is pseudomembranous colitis

Answer 218

Caused by an overgrowth of C difficile from antibiotic use, causing inflammation in the large intestines. Yellow white plaques form pseudomembranes on the mucosa.

Question 219

What is characteristic of Meckel's Diverticulum

Answer 219

RULE of 2s: - 2% of the population - 2 years age onset - 2 x M > F - 2 inches long - 2 feet of the ileocaecal valve - 2 Types of ectopic tissue (gastric mucosa and pancreatic tissue) The gastric mucosa secretes acid Pancreatic tissue secretes alkaline pancreatic juices - eroding the wall

Question 220

Symptoms of Meckel's Diverticulum

Answer 220

1. USUALLY Asymptomatic Gastric Mucosa: - Occult bleeding = iron deficiency Painless rectal bleeding Ectopic pancreatic tissue: - Abdo Pain Increased serum amylase

Question 221

Diagnosis of mocker's diverticulum

Answer 221

1. Laporoscopy in asymptomacti individuals | 2. Techetium 99 pertechnate scintigram to detect gastric mucosa outside the stomach

Question 222

Treatment of mocker's diverticulum

Answer 222

Surgical Resection

Question 223

What is intussusception

Answer 223

1. Where the proximal portion of the small bowel moves into the distal one. Causes bowel obstruction and occludes blood supply

Question 224

Symptoms of intussusception

Answer 224

Acute, intermittent colicky abdominal pain that REDUCES when drawing the knees up Falls asleep between episodes Nausea and bilious vomiting If blood supply is compromised: Red current jelly stool (blood + stool + mucous) Can become necrotic = sepsis

Question 225

Onset of intussusception

Answer 225

3 months to 6 years

Question 226

Examination findings in intussusception

Answer 226

1. Sausage shaped mass in Right Upper Quadrant

Question 227

Diagnosis of intussusception

Answer 227

. ABDO USS: Shows a target sign (inner circle is the intestines entering the distal segment and outer circle is the distal segment receiving the proximal part ) 2. Abdo X Ray for intestinal obstruction and inteperitoneal gas

Question 228

When do we suspect intussusception

Answer 228

In ANY infant with gastroenteritis who is not getting any better

Question 229

What does intussusception typically associate with

Answer 229

Viral gastroenteritis

Question 230

What part of the small bowel often is involved in intussusception

Answer 230

Enlarged peters patch of the ileum

Question 231

Treatment of intussusception

Answer 231

1. Resus (IV) 2. Antibiotics 3. Analgesia 4. NGT 5. Radiological reduction and laparotomy 6. Barium or water soluble contrast enema

Question 232

What is Jvenile Polyposis Syndrome

Answer 232

SMAD4 gene mutation Causes multiple hamartomas

Question 233

Symptoms of JPS

Answer 233

1. USUALLY SYMPOMATIC but there is GI bleeding

Question 234

Diagnosis of JPS

Answer 234

1. Colonoscpy 2. Biopsy 3. Genetic Testing

Question 235

Treatment of JPS

Answer 235

Regular endoscopic monitoring

Question 236

What condition is duodenal atresia associated with

Answer 236

Trisomy 21

Question 237

Invetigestions of duodenal atresia

Answer 237

DOuble bubble sign (gas in stomach and the duodenum

Question 238

Treatment of duodenal stresia

Answer 238

duodenoduodenostomy

Question 239

What condition may mimic acute appendicitis

Answer 239

Mesenteric Adenitis

Question 240

Two causes of Peptic Ulcers

Answer 240

Helicobacter Pylori Infections | NSAIDs

Question 241

What is a dieulafoy lesion

Answer 241

An abnormally dilated artery lining the stomach, more prone to haemorrhages.

Question 242

Where do mallory-weirs tears often originate

Answer 242

Lower 1/3 of the oesophagus and the proximal stomach

Question 243

Treatment of mallory-weirs tears

Answer 243

1. Thermocoagulation 2. Endosocpic band ligation Metoclopromide for vomiting

Question 244

Treatment of dieulafoy lesions

Answer 244

Epinephrine injections

Question 245

What conditions cause occult GI bleeds

Answer 245

1. IBD 2. Meckel's 3. JP 4. Peptic Ulcer Disease

Question 246

Onset of UC

Answer 246

1. 20-30 year olds | 2. Gradual and progressive over a few weeks

Question 247

Extensive colitis vs distal ulcerative colitis

Answer 247

UC usually effects the end parts of the large intestines. However, distal includes the descending colon - rectum while extensive involves all the large intestines except for the caecum. Pancolitis is all

Question 248

Symptoms of UC

Answer 248

1. Fatigue 2. Fever 3. Weight Loss 4. Dyspneoa 5. Palpitations from iron deficiency anaeia 6. Bloody diarrhoea 7. Colicky Diarrhoea Pain 8. Tenesmus

Question 249

What is tenesmus

Answer 249

The physical feeling you need to shit even if you already just had one

Question 250

Extraintestinal signs of UC

Answer 250

1. Arthritis 2. Uveitis 3. Episcleritis 5. Skin Lesions: Pyoderma Gangrenosum, erythema nodosum 6. Primary Sclerosing CHolangitis 7. Thromboembolisms

Question 251

Crohn's vs UC

Answer 251

Defecation: Porridge, sometimes steatorrhoea vs Bloody No Tenesmus vs tenesmus Continuous involvement vs skip lesions (cobblestone appearance)

Question 252

Which IBD has noncaseating granulomas (and what does this mean)

Answer 252

Crohn's NOT UC

Question 253

Complications of UC

Answer 253

1. Forms strictures in the rectosigmoid colon = bowel obstruction

Question 254

Symptoms of Crohn's

Answer 254

1. Watery diarrhoea or steatorrhoea 2. Fistulas between intestines (mass) or interovesical (bladder) = pneumaturia (gas in the urine) 3. Enterocutaneous fistulas 4. Phlegmon (localised area of inflammation in bowel wall) -> abscess. 5. Apthous Ulcers, gingivitis) 6. Odynophagia, dysphagia 7. Gallstones -> biliary colic

Question 255

Differences in extraintetsinal symptoms found in Crohn's

Answer 255

Also forms kidney stones

Question 256

Most common site of Crohn's

Answer 256

Terminal ileum

Question 257

Treatment of Crohn's and UC

Answer 257

Oral 5-ASA Severe symptoms: Oral Prednisolone or IV methylprednisolone Antibiotics: Metronidazole Maintenance treatment: azathioprine

Question 258

Surgery for Uc

Answer 258

Total Colectomy and ileostomy: can cause short bowel syndrome (diarrhoea and malnutrition disorders)

Question 259

Surgery for CD

Answer 259

Local surgical resection of localised disease

Question 260

How to test for H. pylori infection

Answer 260

C14 urea breath test

Question 261

Treatment of H. pylori

Answer 261

Amoxicillin, Omeprazole and Clarithromycin/Metronidazole

Question 262

One faecal test that is positive in IBD

Answer 262

Faecal Calprotetcin (released during inflammation)

Question 263

Where is gluten commonly found

Answer 263

Wheat and grains

Question 264

What substance in gluten causes an immune response during coeliac's

Answer 264

Gliadin

Question 265

What happens to gluten proteins once reabsorbed at the stomach

Answer 265

Broken down by tissue transglutaminase to deaminated gluten proteins Broken down by macrophages and presented on MHCII

Question 266

What two anti-bodies are produced in coeliac's

Answer 266

Anti-tTG Anti-Gladin

Question 267

Investigations for coeliac's

Answer 267

Villi are flattened Crypt hyperplasia + shallow crypts Duodenal biopsy

Question 268

What virus causes viral gastroenteritis

Answer 268

Rotavirus

Question 269

How is gastroenteritis spread

Answer 269

Faecal-oral route

Question 270

Complications of gastroenteritis

Answer 270

1. Reiter's syndrome (shigella, campylobacter) 2. HUS (E.coli and shigella) 3. Guillain-Barre Syndrome (campylobacter)

Question 271

Treatment for campylobacter infections

Answer 271

Erythromicin

Question 272

Treatment for c difficile

Answer 272

Metronidazole

Question 273

What is pyloric stenosis

Answer 273

Hypertrophy of the circular muscle to produce a tumour

Question 274

Onset of pyloric Stenosis

Answer 274

Third or fourth week of life

Question 275

Clinical features of pyloric stenosis

Answer 275

Non-bilious projectile vomiting or fresh blood from oesophagi's Occurs WITHIN an hour of feeding and the baby immediately becomes hungry 2. Constipation 3. Dehydration

Question 276

Diagnosis of pyloric stenosis

Answer 276

Test feeding: so breastfeed the baby while the doctor palpates the baby's abdomen. We should see visible gastric peristalsis passing along the abdomen and tumours are olive shaped masses just above and to the right of the umbilicus USS Metabolic alkalosis

Question 277

Pre-operative management of pyloric stenosis

Answer 277

1. Rehydrate (IV Saline) and KCL | 2. Withold feeding

Question 278

Surgery for pyloric stenosis

Answer 278

Ramstedt's pyloromyotomy: Splijting the thickened pyloric muscle WITHOLD oral feeds overnight

Question 279

Signs of dehydration in neonates

Answer 279

Sunken fontanelle, dru tongue

Question 280

Cause of cyclic vomtiing

Answer 280

Abdominal Migraines Manipulative behaviour or mental health issues

Question 281

What factors cause GORD in infants

Answer 281

1. Slow gastric emptying 2. Hiatus Hernias 3. Liquid diet (from milk) 4. Gastric hyper secretion

Question 282

Presentation of GORD

Answer 282

1. Regurgitation 2. Heartburn, difficult feeding with crying 3. Painful swallowing 4. Haematemesis Faltering growth Resp: Aponea, Hoarseness, cough, stridor and lower respiratory disease (asthma) Neurobehavioural symptoms: Sandifer's syndrome (extension and lateral turning of the head, torticolis)

Question 283

Complication of GORD

Answer 283

1. Barrett's oesophagus | 2. Lower respiratory disease

Question 284

GORD investigations

Answer 284

1. Upper GI endoscopy 2. Oeopshageal biopsy 3. 24 hour oesophageal pH 4. Barium swallow with fluoroscopy 5. Radioisotope milk scan 6. Oesophageal manometry for dysmotility

Question 285

Treatment of GORD

Answer 285

1. Ranitidine or omeprazole for oesophagi's 2. Gaviscon (antacids) 3. Domperidone

Question 286

Surgery for GORD

Answer 286

Nissen's fundoplication: creating a sphincter at the bottom

Question 287

What is achalasia

Answer 287

1. Obstruction of lower oesophageal sphincter

Question 288

Presentation of achalasia

Answer 288

1. Vomiting 2. Dyphagia with both liquids and solids 3. Aspiration

Question 289

Causes of acute pancreatitis

Answer 289

Hep A Coxsackie B Mumps ``` SLE Kawasaki HUS IBD Hyperlipidaemia ``` Drugs: Metronidazole Pancreatic Duct Obstruction: CF

Question 290

Clinical Features of acute pancreatitis

Answer 290

Hypotension Abdominal Distention Cullen's sign (bruising of peri-umbilical area) grey-Turner's sign: Bruising of the flanks Acites Pleural effusion Jaundice

Question 291

Investigations of acute pancreatitis

Answer 291

Serum amylase (raised) and deranged LFTs Abdominal USS and endoscopic retrograde cholangiopancreatography

Question 292

Complications of chronic pancreatitis

Answer 292

DM

Question 293

Causes of chronic pancreatitis

Answer 293

``` CF COngenital ductal anomalies Sclerosing Cholangitis Hyperlipidaemia Hypercalcaemia ```

Question 294

Describe how bilirubin is metabolised

Answer 294

1. Old red blood cells pass through spleen and broken down by macrophages -> heme and global 2. Heme -> Biliverdin by haemorrhage ocygenase 3. Biliverdin -> unconjugated bilirubin by biliverdin reductase 4. Unconjugated bilirubin is lipid soluble 5. To be water soluble: Bilirubin binds to albumin in blood so it isn't excreted by kidneys 6. Unconjugated bilirubin is conjugated by the sinusoidal membranes of hepatocytes -> bile canaliculi -> gallbladder. 7. Conjugated bilirubin moves into duodenum via ampula of water and is converted by microbes to urobilinogen. 80% -> stercobilinogen which causes brown faeces 8. 20% of urobilinogen is rebosorbed and sent back to the liver, 10% is excreted by the kidneys and causes yellow urine colour.

Question 295

What diseases can causes increased haemolytic

Answer 295

Haemolytic disease of the newborn Sickle Cell Anaemia Glucose 6 phosphate deficiency

Question 296

What children are particularly susceptible to jaundice

Answer 296

Neonates if they're pre term

Question 297

Why are preterm babies at a high risk of neonatal jaundice

Answer 297

Common in asian newborns: Newborns have high haematocrit but shorter life spans of RBCs. High RBCs = high levels of unconjugated bilirubin. Immature UDP glucoronyl transferase so bilirubin conjugation is already imipaured.

Question 298

What medications can cause neonatal jaundice and why

Answer 298

Sulfonamides and ceftriaxone: take up bilirubin binding sites on albumin

Question 299

How is neonatal jaundice treated

Answer 299

non-UV phototherapy: isomerizes unconjugated bilirubin to water-soluble form so it can be excreted.

Question 300

What can cause breast milk jaundice

Answer 300

High levels of glucoronidase in breast milk can deconjugate bilirubin in the intestines. This increases enterohepatic circulation of unconjugated bilirubin -> unconjugated hyperbilirubinaemia

Question 301

When does breast milk jaundice develop

Answer 301

After first 5-7 days of life and peaks after 2 weeks Whereas physiologic jaundice of newborns occurs within 1 week of life.

Question 302

What is Gilbert Syndrome

Answer 302

Impaired hepatic bilirubin uptake and reduced production of UDP glucoronyl transferase. LFTs are normal

Question 303

Consequence of neonatal jaundice

Answer 303

Kernicterus: High bilirubin levels in blood can cause brain damage and learning disabilities.

Question 304

When are intravenous IVIG indicated for neonatal jaundice

Answer 304

If rhesus haemolytic disease is present

Question 305

What is Crigler-Najjar syndrome

Answer 305

Complete absence of UDP glucoronyl Transferase

Question 306

What can cause intrahepatic jaundice

Answer 306

1. Unconjugated +/- conjugated hyperbilirubinaemia ``` Viral Hep, Paracetamol overdose, sodium valproate Wilson's Alpha 1-antitripsin deficiency Hypothyroidism ``` Budd-Chiari Syndrome Right sided heart failure

Question 307

What is budd-chiari yndrome

Answer 307

Hepatic veins blocked by a clot causing liver to grow larger

Question 308

What can cause obstructive jaundice

Answer 308

1. CONJUGATED hyperbilirubinaemia ``` Biliary atresia Choledochal cyst Primary sclerosis cholangitis CF Cholecystitis Tumours ```

Question 309

In emergency treatment of a food allergic reaction, what should be given

Answer 309

IM adrenaline

Question 310

What commonly causes lactose intolerance

Answer 310

Post-viral gastroenteritis lactase deficiency (e.g., rotavirus) This is transient and short lasting

Question 311

Presentation of lactose intolerance

Answer 311

Diarhoea Excessive Flatus COlics Stool pH < 5

Question 312

Presentation of Wilson's Disease

Answer 312

Kayser-Fleischer rings (copper deposits in the eye)

Question 313

Ivesgtigations for wilson's

Answer 313

1. Decreased serum copper and caeruloplasmin 2. 24hr urinary copper excretion 3. Molecular genetic testing

Question 314

Treatment of wilson's

Answer 314

Chleation therapy with penecillamine

Question 315

Presentations of biliary atresia

Answer 315

Jaundiced, dark urine, light stools and hepatocplenomegaly Normal weight and meconium is normal too Failure to thrive

Question 316

Onset of biliary atresia

Answer 316

2 months

Question 317

Treatment of biliary atresia

Answer 317

Kasai portoeneterostomy

Question 318

What is an incarcerated inguinal hernia

Answer 318

The swelling is irreducible

Question 319

Initial management of an inguinal hernia

Answer 319

Resuscitation and an NGT 2. AXR: intestinal obstruction

Question 320

What is a congenital diaphragmatic hernia

Answer 320

A hole in the diaphragm.

Question 321

What are the two types of hiatus hernias

Answer 321

1. Sliding (more common in infants) | 2. Rolling

Question 322

Presentations of inguinal hernias

Answer 322

1. BOYS 2. Common on the right side due to later descent of the right testis No symptoms

Question 323

In what ethnicity are umbilical hernias common

Answer 323

Afro-Caribbean children

Question 324

Marasmus vs Kwshiorkor

Answer 324

Severe deficiency of all nutrients and inadequate caloric intake vs normal caloric intake but protein deficiency Peripheral oedema absent vs peripheral oedema No hair changes vs sparse hair Dry skin vs flaky skin Large appetite vs poor appetite No submit fat vs reduced subset fat No fatty liver vs fatty liver Better prognosis vs worse prognosis

Question 325

Investigations for kwashiorkor and marasmus

Answer 325

Mid-upper arm circumference due to oedema

Question 326

What can cause vet A deficiency

Answer 326

Fat malabsorption states (CF)

Question 327

What vitamin deficiency can cause rickets

Answer 327

Vit D

Question 328

Causes of Vit D deficiency

Answer 328

Low UV light Fat malabsorption Hepatic or renal failure

Question 329

What can cause B12 deficiency

Answer 329

Vegan diets IBD Pernicious Anaemia

Question 330

Presentation of B12 deficiency

Answer 330

1. Paraesthesias 2. Peripheral neuropathy 3. Macroyctic MEGALOBLASTIC anaemia 3. Motor weakness

Question 331

Triad of symptoms in cholecondal cysts

Answer 331

1. INTERMITTENT ABDO PAIN 2. Jaundice 3. Right UQ mass

Question 332

Symptoms of appendicitis

Answer 332

1. Initial periumbilical abdominal pain, with diarrhoea, indigestion and malaise 2. 48 hours later, localised pain and fever

Question 333

Two signs of appendicitis

Answer 333

McBrurney's (1/3rd the way from belly button to right anterior superior iliac spine) Rosving's sign (palpation of left lower quadrant auses pain in the other side) Obturator sign: Irritation of obturator interns muscle Iliopsoas sign: Retrocaecal appendicitis causes pain when extending the right hip

Question 334

Signs of physical abuse

Answer 334

1. Mark/Bruise 2. Fractures, Internal Bleeding and Death 3. Radial Head Subluxation: Nursemaid's elbow 4. Burns

Question 335

Clues of child abuse in patients

Answer 335

1. Repeated hospitalisations with unexplained injuries 2. History of STId 3. Explanation is inconsistent with injury severity

Question 336

What is nursemaid's elbow

Answer 336

Radial Head Sublexation

Question 337

What do abuse burns usually arise from

Answer 337

Forced into hot water: Spared creases in abdomen and palms/soles of the feet Cigrettes being bumped out

Question 338

What clotting factors require vit K

Answer 338

1972

Question 339

What is the function of warfarin

Answer 339

It blocks Vit K from being in its activate state (blocks via K epoxide reductase), this blocks the extrinsic pathway which requires factor 7 to activate Then reduces 10 9 and 2 in the intrinsic pathway Causes 1972 to not be activated

Question 340

What test is used to measure warfarin pathway

Answer 340

PT: extrinsic

Question 341

What is INR

Answer 341

It's patient's PT/ control PT (so if it's 2, it means the patient's blood takes 2 times longer to clot than the general pop)

Question 342

What's a normal INR

Answer 342

<1.1

Question 343

Why is heparin often given at the start of warfarin therapy

Answer 343

Because Warfarin can destroy protein C = congenital protein C deficiency. Causes a state of hyper coagulation (with Va) before anticoagulation occurs.

Question 344

Name four acyanotic heart defects

Answer 344

1. ventricular septal defect (VSD) 2. Atrial Septal Defect 3. Patent Ductus Arteriosus 4. Coarctation

Question 345

Name four cyanotic heart defects

Answer 345

1. Tetralogy 2. Transposition 3. Truncus Arteriosus 4. Total Anomalous Pulmonary Venous return 5. Hypoplastic left heart syndrome

Question 346

Diagnoses for heart defects

Answer 346

1. ECG 2. Chest X Ray for suspicion of heart failure 3. CT or MRI if diagnosis is inconclusive 4. Cardiac Catheterisation + Angiography if diagnosis remains uncertain after non invasive investigations 5. Infective endocarditis Prophylaxis (Cyanotic congenital heart disease not fully replier or fully repaired or with existing leaks)

Question 347

What is the optimal gestational age for screening for congenital heart defects

Answer 347

18-22 weeks

Question 348

What follow up investigation is done if sat levels are <90% in a neonate

Answer 348

Urgent Echocardiography

Question 349

In what conditions should sat levels be repeated hourly

Answer 349

IF it remains 90-95% or there is a difference in 3% between the legs and right hand

Question 350

Pathophysiology of symptoms in Cyanotic defects

Answer 350

1. Oxygenated blood moves from the left ventricle to the right ventricle so no cyanotic symptoms, however this causes pulmonary HTN as extra blood is moving through the pulmonary artery. 2. However, eventually pulmonary pressure > left ventricle pushing blood, that deoxygenated blood from the right to the left - EISENMENGER SYNDROME 3. This eventually leads to cyanosis.

Question 351

What is the most common septal defect

Answer 351

Ventricular septal defects

Question 352

What is different about VSD compared to other cyanotic defects

Answer 352

Not related to increased pulmonary vascular resistance There is just left to right shunting

Question 353

Ausculttaion Symptoms of VSD on examination

Answer 353

1. Loud, harsh, blowing holosytolic murmur 2. Palpable thrill 3. Mid-Diastolic, low pitched rumble from increased blood flow through the mitral valve. 4. Parasternal heave and a displaced apex beat

Question 354

Where is the holosystolic murmur in children best heard

Answer 354

Lower left sternal border

Question 355

What is a holosytolic murmur

Answer 355

1. Begins at the first heart sound and continue to the second heart sound

Question 356

What causes the S1 sound

Answer 356

Mitral valve closure (left atrium -> left ventricle) + tricuspid valve closure.

Question 357

What causes the S2 sound

Answer 357

Aortic and pulmonary valve closure

Question 358

What causes an S2 split sound

Answer 358

Inhalation.

Question 359

Most common cause of an S3 sound

Answer 359

Congetsive heart failure

Question 360

Signs of VSD

Answer 360

1. NO CYANOSIS | 2. LFH symptoms: Peripheral oedema, ascites and liver enlargement

Question 361

Imaging for VSD

Answer 361

1. Transthoracic echocardiography to determine position and size 2. Doppler echocardiography for magnitude of shunt - GOLD 3. CXR for Cardiomegaly 4. ECG for ventricle hypertrophy Last two not diagnostic but initial investigations

Question 362

Treatment for small VSD

Answer 362

1. Spontaneous, leave it

Question 363

Indications for surgical interventions for large VSDs

Answer 363

1. History of endocarditis | 2. Left ventricle dysfunction

Question 364

When are surgical closures of a VSD septum conytraindicated

Answer 364

During Severe Pulmonary Hypertension + Eisenmenger's

Question 365

Treatment for severe pulmonary hypertension + Eisenmenger's

Answer 365

Bosenten or Sildenafil: Pulmonary vasodilator

Question 366

Treatment of a small ASD

Answer 366

<0.5cm = asymptomatic, leave

Question 367

What is a large ASD

Answer 367

>2cm

Question 368

Auscultations of ASD

Answer 368

1. S1: Mid-systolic pulmonary flow 2. Widened split 2nd sound in all respiratory phases because the extra blood return during inspiration equals out across the atrium. Prolongued emptying of the enlarged right atrium delaying closure. 3. Short, rumbling, mmid-diastolic murmur in lower sternal border

Question 369

Signs of ASD

Answer 369

1. Atrial enlargement (chest deformities), Harrison grooves - depression along the sixth and seventh intercostal cartilages. 2. Signs of heart failure 3.

Question 370

Imaging of ASD

Answer 370

1. In children: Transthoracic for size and position of ASD 2. CXR for heart enlargement and increased pulmonary vascular marking 3. ECG: Prolongued QRS complex, p > 2.5mm and right axis deviation from hypertrophy

Question 371

Treatment of ASD

Answer 371

1. Small = spontaneously close in first year of life | 2. >1cm = surgical or percutaneous closure

Question 372

What is the ductus arterioles

Answer 372

Opening between the aorta and pulmonary artery

Question 373

Auscultations of patent ductus arterioles

Answer 373

1. Continuous murmur in second left intercostal space straight after S1 2. Mid-Diastolic low pitched rumble at apex due to increased blood flow moving through mitral valve. 3. Systolic thrill radiating across the midclavicular line

Question 374

Symptoms of patent ductus arterioles

Answer 374

1. COngetsive Failure 2. Impaired Growth 3. Bounding peripheral arterial pulses 4. Wide Pulse Pressure from runoff into pulmonary artery in diastole

Question 375

Imaging for patent ductus arterioles

Answer 375

1. Echocardiography to visualise and measure ductus 2. Doppler to see systolic retrograde turbulent flow in the pulmonary artery 3. Aortic Retrograde flow in diastole 4. Chest XRAY: Prominent pulmonary artery 2. Cardiac enlargement 3. Increased pulmonary vascular markings

Question 376

ECG findings for patent ductus arterioles

Answer 376

1. Deep Q, Tall R

Question 377

Treatment of patent ductus arterioles in heaemodynamically stable infants

Answer 377

1. Acetaminophen

Question 378

Treatment of patent ductus arterioles in unstable infants

Answer 378

Surgical ligation or occlusion percutaneously

Question 379

When in patent ductus arterioles is surgical ligation contraindicated

Answer 379

When severe pulmonary vascular obstructive diseases occurs

Question 380

What is coarctation of aortaa

Answer 380

1. Narrowing of the aorta just where th left subclavian artery branches off, near the ductus arteriosus

Question 381

Auscultations for coarctation of aorta

Answer 381

1. Short systolic murmur at left sternal border, 3rd and 4th intercostal spaces 2. Systolic ejection click from bicuspid valve

Question 382

Symptoms of coarctation

Answer 382

1. Disparity in pulses and blood pressure in arms and legs: Normal: Femoral pulse occurs slightly before radial pulse. Radiofemoral delay: Femoral pulse after the radial pulse. Normal: Systolic in Leggs is 10-20mmHg > arms Here: Arms has higher systolic pressure than in the legs. Heart failure and cyanosis. Tachypnoea Signs of Heart Failure

Question 383

Imaging for coarctation

Answer 383

1. ECHO to locate and check valve abnormality 2. CXR 3. ECG: Normal in young children

Question 384

Treatment of mild coarctation

Answer 384

1. Prostaglandin E1 to open ductus and relax tissues

Question 385

Surgical intervention of coarctation

Answer 385

1. Removing coarctation segment and anastomoses | 2. Transcatheter: Balloon and stent angioplasty

Question 386

Four elements of tetralogy of fallout

Answer 386

1. Pulmonary Stenosis 2. RVH 3. Dextraposition of aorta 4. VSD

Question 387

Auscultation of tetralogy of fallout

Answer 387

1. Systolic murmur: Crescendo - decrescendo at left sternal border. Preceeded by the clock. Cause: Turbulence through right ventricular outflow track because of pulmonary stenosis 2. Systolic thrill

Question 388

Symptoms of tetralogy of fallout

Answer 388

1. Left anterior hemithorax bulge from RVH 2. Signs of heart failure 3. Cyanosis later in life (1st year) 4. Dyspnoea on mild exertion that is relieved on squatting 5. Dusky blue skin ray sclerae, clubbing of fingers and toes 6. PAROXYSMAL HYPERCYANOTIC ATTACKS in 1st -> 2nd years of life. 7.

Question 389

What are paroxysmal hyper cyanotic attacks

Answer 389

1. Hyperpneic and restlessness 2. Cyanosis 3. Gasping resp 4. Syncope

Question 390

Onset of paroxysmal hyper cyanotic attacks

Answer 390

Morning or after crying

Question 391

Complication of paroxysmal hyper cyanotic attacks

Answer 391

Progress to convulsions or hemiparesis

Question 392

Diagnosis of tetralogy of fallout

Answer 392

1. 2D Echocardiography to check extent of overriding aorta, location of RV outflow tract obstruction 2. CXR: BOOTSHAPED heart and enlarged right sided aorta 3. ECG: Right Axis deviation from hypertrophy Prominent R and S

Question 393

What is characteristic about CXR in tetralogy of fallot

Answer 393

BOOTSHAPED heart

Question 394

Treatment of blue spells

Answer 394

1. Knee-chest positioning 2. Oxygen therapy 3. IV Fluid Bolus to increased pulmonary flow 4. Morphine 5. IV beta-blockers 6. IV Phenylephrine

Question 395

Treatment of heart failure in tetralogy of fallt

Answer 395

Digoxin or loop diuretics or complete surgical repair to relieve RV outflow tract obstruction and closure of VSD

Question 396

What is transposition of the great arteries

Answer 396

Usually where the figure of eight systemic/pulmonary blood flow circuit is replacedbby two separate parallel circuits (ie., systemic venous blood possessing through the right side of the heart returns directly to the systemic circulation via a connecting aorta. Pulmonary venous blood returning to the left side of the heart moves back into the pulmonary circulation via a connecting pulmonary artery.

Question 397

Clinical features of transposition of the great arteries

Answer 397

Within a few hours of life with worsening cyanosis. Severe hypoxia

Question 398

What is the treatment for transposition of the great arteries

Answer 398

1. Correction of acidosis 2. Correction of hypoglycaemia 3. Improve arterial oxygenation using prostaglandin E infusion and balloon atrial septostomy

Question 399

What surgery is done to correct transposition of the great arteries

Answer 399

Arterial switch procedure in the first two weeks of life

Question 400

In what condition iss a complete atrioventricular septal defect found

Answer 400

Down syndrome

Question 401

Three features of an AV septal defect

Answer 401

1. Large defect from middle of the atrial septum down to the middle of the ventricular septum 2. Missing bicuspid and tricuspid vallves 3. Just one common atrioventricular valves guarding the atrioventricular junction

Question 402

ECG findings in complete AVSD

Answer 402

Superior axis

Question 403

When is surgery for AVSD offered

Answer 403

3 Months

Question 404

What is tricuspid atresia

Answer 404

No connection between the right atrium and right ventricle causing venous blood to be diverted to the left side through a patent foramen oval.

Question 405

What is the foramen oval

Answer 405

Small opening between the right and left atrium

Question 406

Clinical features of tricuspid atresia

Answer 406

Pulmonary blood flow

Question 407

Auscultation findings in transposition of great arteries

Answer 407

Single and LOUD S2 sound Absent murmurs

Question 408

Why is hypoxia only moderate in transposition of great arteries

Answer 408

Because the ductus may be patent, allowing some shunting of oxygenated blood from the lungs to the systemic circulation

Question 409

Findings on an echocardiography for transposition of great arteries

Answer 409

Egg-Shaped heart Visualises ductus arteriosus, left ventricular outflow tract obstruction VSDs

Question 410

Findings on an ECG for tranpsposition of great arteires

Answer 410

Right axis deviation | RV hypertrophy

Question 411

What is the balloon atrial septostomy

Answer 411

Increases size of foramen ovale Then corrective surgery

Question 412

What is the corrective surgery for transposition of great arteries

Answer 412

Arterial switch operation or rastelli procedure in those with large VSDs

Question 413

What is trunks arteriosus

Answer 413

Blood leaves the heart through one main vessel instead of two (the pulmonary artery and the aorta fuse and form one whole vessel). This causes systemic and pulmonary blood to mix - cyanosis

Question 414

Auscultation findings in truncus arteriosus

Answer 414

Loud and Single S2 sound. Ejection click Systolic murmur

Question 415

Clinical findings of truncus arterioles

Answer 415

Peripheral pulses are accentuated and bounding Mild cyanosis Resp distress

Question 416

Clinical investigations for truncus arterioles

Answer 416

1. Echocardiography 2. Doppler 3. CXR for cardiac enlargement and right aortic arch (common in third) ECGs may be normal

Question 417

Treatment of truncus arterioles

Answer 417

1. Diuretics to reduce preload 2. ACE inhibitors to reduce after load. 3. Positive pressure ventilation Definitive: Primary surgical repair.

Question 418

What is aortic stenosis

Answer 418

1. MOST COMMON CAUSE OF Left ventricular outflow obstruction Thickening of aortic valves

Question 419

RF for aortic stenosis

Answer 419

MOST COMMON IN BOYS

Question 420

DLINIDQL FEATURES OF AORTIC STENOSIS

Answer 420

1. ASYMPTOMATIC 2. MAYBE HEART FAILURE AND COLLAPSE IF SEVERE 3. Child may complain of syncope and chest pain on exertion

Question 421

Management of aortic stenosis

Answer 421

Balloon dilation Avoid competitive sports

Question 422

What is total anomalous pulmonary venous return

Answer 422

This is where the four pulmonary veins are connected to a vessel that is not the left atrium

Question 423

Most common place for total anomalous pulmonary venous return

Answer 423

Superior Vena Cava

Question 424

Auscultation findings in TAPVR

Answer 424

1. Systolic Ejetcion Murmur | 2. Widely Split second sound

Question 425

Symptoms of TAPVR

Answer 425

1. High pulmonary artery pressure | 2. Causes oedema and hypotension -> Symptoms resemble respiratory distress syndrome.

Question 426

TAPVR vs Respiratory distress syndrome

Answer 426

Symptoms occur after first 12 hours of life vs immediately after birth

Question 427

Echo findings in TAPVR

Answer 427

1. Abnormal pulmonary venous attachments 2. Common ascending collecting vein 3. Dilated superior vena cava 4. Direct connection between pulmonary venous system and the RA R->L atrial shunting

Question 428

CXR findings in TAPVR

Answer 428

1. Small Heart 2. Severe Diffuse Pulmonary oedema 3. Cardiomegaly

Question 429

ECG findings in TAPVR

Answer 429

Right axis deviation

Question 430

Treatment of TAPVR

Answer 430

1. Surgical repair: Wide anastomosis with pulmonary venous confluence.

Question 431

What is hypo plastic Left Heart Syndrome

Answer 431

1. Hypoplasia of the ascending aorta 2. Hypoplasia of the left ventricle 3. Maldevelopment of the aortic and mitral valve 4. This leaves a patent ductus arterioles and ASD

Question 432

Symptoms of hypo plastic left heart syndrome

Answer 432

1. Oxyegnated blood reaching left atrium moves into the right atrium. This mixes with deoxygenated venous blood -> exits the right ventricle to the lungs and then into systemic circulation

Question 433

Auscultation findings of hypo plastic left heart syndrome

Answer 433

No murmurs

Question 434

Consequences of hypo plastic left heart syndrome

Answer 434

The ductus arterioles closes in first two days of life, causing cariogenic shock: ``` tachypnoea 2. Dyspnoea 3. Weak Pulse 4. Anuria Oliguria ``` Reduction in coronary and cerebral perfusion

Question 435

Treatment of Total Anomalous pulmonary venous connection

Answer 435

1. Surgical anastomosis of the common pulmonary channel to the left atrium, closing the ASD

Question 436

What is hypertrophic obstructive cardiomyopathy

Answer 436

1. Massive left ventricular hypertrophy, where the myocardium is fibroses, resulting in a stiff muscle with decreased distensibility. This reduces ventricular filling but systolic pumping is maintained til later on.

Question 437

CF for hypertrophic obstructive cardiomyopathy

Answer 437

1. Incidental heart murmur, usually a sudden cause for unexpected death as asymptomatic

Question 438

Treatment of hypertrophic obstructive cardiomyopathy

Answer 438

1. Pacemaker, beta blockers, calcium antagonists | 2. Ventricular septal myotome or transplants

Question 439

What malformations are most associated with dextrocardia

Answer 439

1. Pulmonary stenosis, tricuspid atresia, transposition, anomalous venous drainage

Question 440

Most common infective agent in infective endocarditis

Answer 440

``` Strep viridians (dental) Staph aureus (catheters) Group D strep (enterococcus) - GI ```

Question 441

Examination findings in endocarditis

Answer 441

1. Anaemia 2. Splinter haemorrheages 2. Tender nodules in fingers and toes - Osler's nodes 3. Erythema in palms and soles of feet (Janeway lesions) Finger clubbing later on 5. Retinal infarcts - roth sports 6. Necrotic skin lesions and splenomegaly

Question 442

Treatment of endocarditis

Answer 442

1, IV Oebecillin or vancomycin for 6 weeks minimum

Question 443

What causes rheumatic fever

Answer 443

1, Beta haemolytic streptococcus

Question 444

Ages that typically get rheumatic fever

Answer 444

5-15 years old

Question 445

Clinical features of rheumatic fever

Answer 445

1. 2-6 weeks between onset of symptoms and last strep infection (e.g., pharyngitis)

Question 446

What criteria can features of rheumatic fever be categorised under

Answer 446

Jones criteria

Question 447

What is jones criteria

Answer 447

1. Pancarditis 2. Polyarthritis 3. Erythema marginatum 4. Syndham's corea

Question 448

Management of rheumatic fever

Answer 448

1. Bed rest 2. Aspirin 3. Corticosteroids 4. Diuretics 5. Peecillin V for 10 days

Question 449

What can cause pericarditis

Answer 449

``` Infections: Viral: Coxsackie B, EBV Bacterial: Strep, mycoplasma TB Fungal (Histoplasmosis Parasitic (toxoplasmosis) ``` ``` Connective Tissue: RA RF SLE Sarcoidosis ``` Metabolic: Hyperuricaemia Hypothyroidism

Question 450

Clinical feature distinctive of pericarditis

Answer 450

1. Sharp pain exacerbated when lying down Relieved when sitting or leaning forward - referred to the left shoulder.

Question 451

Management for pericarditis

Answer 451

1. Analgesia for pain relief and ant inflammatory drugs

Question 452

What is constrictive pericarditis

Answer 452

1. Where pericardium inflammation causes the pericardium to become scarred and thickened, making the heart less efficient at pumping blood.

Question 453

Role of the pericardium

Answer 453

Fibrous: Keeps heart in the chest cavity Serous: Forms the cavity which has fluid o let the heart slip as it beats

Question 454

What usually causes pericardial effusion (involving blood)

Answer 454

Trauma to the chest: Stabbing Blunt trauma: Being pushed against an object MI infarctions: Weakened walls break under pressure Heart surgery Aortic dissection

Question 455

Consequence of cardiac tamponade

Answer 455

It reduces the amount of space the heart has to expand before contracting again = less CO and leads to hypotension.

Question 456

Features of cardiac tamponade

Answer 456

1. During inhalation systolic BP drops . Normally: The RV expands and does not affect the left heart volume. In tomponate, the extra volume pushes the ventricular septum to the left, reducing the stroke volume Beck's triad: Distended jugular veins Hypotension Distant Heart sounds

Question 457

What is SV

Answer 457

The volume of blood pumped out of the LV during each systolic cardiac contraction: End diastolic - End systolic volume

Question 458

What drug can cause myocarditis

Answer 458

Adriamycin

Question 459

What infections can cause myocarditis

Answer 459

Coxsackie B and EBV

Question 460

Describe the pathway of electrical impulses through the hear t

Answer 460

SA Node in right atrium -> walls of right atrium and left atrium via backmann's bundles Delayed as it moves to the AV Nodes -> Bundle of His -> purkyje fibres

Question 461

What is the p wave

Answer 461

Atrial contraction

Question 462

What is the PR interval

Answer 462

Delay before moving to the AV Node

Question 463

What is WPW syndrome

Answer 463

Where there is a backdoor entrance from the atria to the ventricles (they usually can only pass through via the AV node) Through the bundle of Kent -> pre excitation Signals travel through bundle of Kent AND the bundle of his to cause v strong contractions This can also result in a reentry circuit, where signals move form bundle of his to the ventricles and back up the bundle of Kent to excite the atrial (atrioventricularr reentrant tachycardia

Question 464

ECG findings for WPW syndrome

Answer 464

1. Short PR interval QRS prolonged

Question 465

What defines a short PR interval

Answer 465

Less than 120 ms

Question 466

What defines a prolonged QRS complex

Answer 466

Over 110 ms

Question 467

Symptoms of WPW

Answer 467

Atrial arrhythmia: 300BPM and cariogenic shock

Question 468

What is heart block

Answer 468

An arrhythmia caused by signals being delayed or blocked (e.g., damage to the nodes)

Question 469

What is a first degree AV block

Answer 469

There is a delay in electric conduction but it still makes it through the AV node to the ventricles PR > 200ms

Question 470

What is Mobitz type 1 second degree block

Answer 470

PR gets progressively longer until electric signals are blocked completely (does not make it to the ventricles) -> there is a dropped beat.

Question 471

What is a mobitza type II second degree AV block

Answer 471

Fairly random dropped beats, no progressive lengthening of PR interval.

Question 472

What is third degree AV block

Answer 472

1. No signals to ventricles

Question 473

Treatment non heart bloick

Answer 473

Atropine to increase heart rate

Question 474

What is the most common abnormal arrhythmia in children

Answer 474

Supraventricular tachycardia.

Question 475

Characteristics of innocent heart murmurs in children

Answer 475

1. Systolic in timing never diastolic 2. Short duration 3. Inetisifies with increased CO (e..g, fever or exercise) 4. May change in intensity when changing positions 5. No thrills 8. No radiation Asymptomatic

Question 476

Types of innocent hear tmurmurs

Answer 476

1. Venous Hum: Machine sound in upper left sternal edge due to great vein flow Flow murmur: Short systolic murmur: Mid-left sternal edge - from fevers and disappears on itself

Question 477

Likely diagnosis with a continuous apex murmur

Answer 477

1. Mitral regurgitation (seen in cyanotic ehaumatic fever)

Question 478

Likely diagnosis if a continuous murmur is heart in the mid-left sternal edge

Answer 478

Tertalogy of fallout

Question 479

Likely diagnosis with an ejection systolic murmur on the upper LSE

Answer 479

ASD secundum, fixed split S2

Question 480

Name four cyanotic CHDs

Answer 480

1. Tetralogy of fallout 2. Transpotiin 3. Tricuspid atresia 4. Total anomalous pulmonary drainage.

Question 481

RECAP: BP management pathway

Answer 481

<55 and not black: ACEI or ARB -> ACEi/ARB and CCB -> ACEi/ARB, CCB and thiazide like diuretic -> all + alpha blocker or spironolactone

Question 482

Management of a STEMI

Answer 482

1. Ticagrelor + Aspirin unless high bleeding risk. High bleeding risk: just aspirin w/without ticagrelor

Question 483

Within how long can a PCI be delivered

Answer 483

Within 12 hours of symptoms present .

Question 484

ECG findings in an NSTEMI

Answer 484

ST depression T wave inversion

Question 485

Initial drug therapy for a STEMI

Answer 485

300 mg aspirin

Question 486

Mechanism of action of ticagrelor

Answer 486

P2y12 inhibitor - stops platelet aggregatio n

Question 487

Mechanism of action of aspirin

Answer 487

COX 1 and COX 2 inhibitor: COX 2 is needed to stop prostaglandin production -> anti-inflammatory

Question 488

What does rheumatic fever commonly succeed

Answer 488

Strep pharyngitis by strep progenies (strep throat)

Question 489

What valve commonly scars in chronic rheumatic heart disease

Answer 489

Mitral -> causes regurgitation and then stenosis

Question 490

Stable vs unstable nagina

Answer 490

Unstable: Pain is at rest whereas stable is usually on exertion

Question 491

What is Klinefelter's syndrome

Answer 491

Where a biological male (XY) inherits an extra X chromosome(or a few others)

Question 492

What is the impact of an extra X chromosome in Klinefelter's

Answer 492

Causes less testosterone to be secreted

Question 493

Role of Leydig Cells

Answer 493

Respond to LH and covert cholesterol to testosterone

Question 494

What is the role of testosterone in spermatogenesis

Answer 494

Testosterone and FSH together cause the Sertoli cells in the seminiferous tubules of the.testes to make more sperm.

Question 495

Describe the negative feedback loop of testosterone

Answer 495

Inhibits gNRH and LH from the pituitary glands

Question 496

What cell enhances this negative feedback

Answer 496

Sertoli cells which produce inhibin (like how granulose cells produce inhibin to enhance the inhibition of progesterone)

Question 497

How does Klinefelter's syndrome impact the negative feedback loop of testosterone-LH-GNRH

Answer 497

1. The extra X chromosome stops leading cells from producing testosterone and Sertoli cells from producing inhibin This causes LH and FSH to increase Lack of testosterone reduces sperm production and stops testes maturation. (Each additional X increases oestrogen:testosterone ratio)

Question 498

What cell division phase causes klinefelter's

Answer 498

Extra X chromosome can happen from errors in meiosis (the duplication to sister chromatids). Meiosis 1: separes the chromosomes into pairs Meiosis 2: pairs into individual chromatids. Meiosis 2 doesn't separate all the pairs Also: Failure in equal mitosis can cause excess chromosomes in one cell, so if meiosis II fails again, there are even more X chromosomes that can end up in the gamete (E.g., XXXXY)

Question 499

Onset and symptoms of Klinefelter's

Answer 499

``` At puberty: Hypogonadism (small testes and penis) Sterile Tall, long legs, short torso, broad hips and gynecomastia Less muscle mass Less facial and body hair Weak bones Low energy levels and IQ points compared to other relatives ``` Some may live atypical or female gender identities.

Question 500

What diseases does Klinefelter put women at a higher risk of getting

Answer 500

Breast Cancer Osteoporosis

Question 501

Diagnoses of Klinefelter

Answer 501

Karyotyping Amniocentesis Testosterone, FSH and LH test

Question 502

What is Patau Syndrome

Answer 502

A chromosomal disorder where someone inherits an extra copy of chromosome 13 (trisomy 13) Robertsonian Translocation

Question 503

What cell division error causes pate syndrome and what is this

Answer 503

Nondisjunction: where the chromosomes do not split apart causing one cell to get both chromosomes while the other gets none. So two cells have a chromosome each (24) and two do not (22) Can also happen in the second step: where the chromosome does not split into individual chromatids.

Question 504

What is mosaicism

Answer 504

Where some cells have an extra chromosome and others do not

Question 505

Symptoms of trisomy 13 | hint, the disease of the celes

Answer 505

Baby: Microcephaly Holoprosencephaly (where the prosencephalon fails to divide into two brain hemispheres) Meningomyelocele ``` Developmental delay (LDs) GI problems: Omphalocele Septal herat defects Polycystic kidney disease Cutis Aplasia: Scalp lesions where skin fails to develop Microthlmia: Small eyes Polydactyly Cell lip and palate Rocker-bottom Feet (smooth feet) Cyclopia (cyclops - one eye from holoprosencephaly) ```

Question 506

What is a meningomyelocele

Answer 506

Sevre spina bifida where the spinal cord and meninges protrude out of a whole in the back. Only held together by a sack of skin

Question 507

What is an omphalocele

Answer 507

Bowel herniates out into the umbilical cord

Question 508

Survival rates of trisomy 13

Answer 508

Most babies die before birth: 3 days 5% pass 6 months of life

Question 509

Serum markers for pate

Answer 509

1st trimester: Reduced HCG and PAPP-A 2nd trimester: AFP and HCG are normal Karyotyping through amniocentesis

Question 510

What is Edward's syndrome

Answer 510

A chromosomal disorder where a person inherits an extra copy of chromosome 18

Question 511

Genetic causes for Edwards syndrome

Answer 511

Nondisjunction Robertsonian Translocation Mosaicism

Question 512

Symptoms of Edwards syndrome

Answer 512

1. LDs 2. Septal Heart Defect and Patent Ductus Arterioles 3. Omphalocele Oesophageal Atresia 5. Horseshoe kidneys (where kidneys fuse together) 6. Pulmonary hypoplasia 7. Frequent infections

Question 513

How does oesophageal atresia lead to polyhydramnios

Answer 513

Swallows less amniotic fluid as its blind ending

Question 514

What tumour are Edwards syndrome people at an increased risk of getting

Answer 514

WILMS TUMOUR (Nephroblastoma) Hepatoblastoma

Question 515

RF for wards syndrome

Answer 515

1. MATERNAL AGE | 2. FH

Question 516

RF in Edwards syndrome vs Klinefelter's

Answer 516

Females more commonly effected in Edwards vs males

Question 517

Survival rates of Edwards syndrome

Answer 517

Death before birth from central apnoea

Question 518

Investigation findings in edwards

Answer 518

Increased nuchal translucency on USS Polyhydramnios USS Reduced HCG and PAPP-A

Question 519

What is fragile X Syndrome

Answer 519

Named as the edges of the chromosome look pedunculate at the site of mutation

Question 520

Gene involvement in FX syndrome

Answer 520

FMR1 - fragile X Mental Retardation 1 Has a trinucleotide repeats where 3 DNA nucleotides are repeated multiple times in a row This causes DNA polymerase to get confused and then just starts again and recopies the nucleotides from the start again.

Question 521

What is the tricnucleotide repeat found in FX syndrome

Answer 521

CGG repeat (>200) Normal: 5-44

Question 522

Developmental problems of Fragile X chromosome

Answer 522

1. LD 2. Delayed Speech 3. Delayed Motor Development (learning to walk at 18 not at 12 motnhs) 4. Autism, ADHD and seizures

Question 523

Physical features of fragile X

Answer 523

1. Long narrow face 2. Prominent Jaw and Forehead 3. Large ears that stick out

Question 524

Why is Fragile X Chromosome less common in females

Answer 524

They have a back u[ FMR1 so less penetrance But can get primary ovarian insufficiency (premature ovarian failure)

Question 525

Symptoms in people with permutation carriers (55-200)

Answer 525

1. Fragile X Associated Tremor ataxia syndrome - Intention Tremour, ataxia, memory and cognitive problems MRI scan

Question 526

Diagnosis of Fragile X Syndrome

Answer 526

1. CGG repeats through DNA test FMR1 test Carrier testing in pregnant women

Question 527

What is muscular dystrophy

Answer 527

MYTONIC (contracted and unable to relax) DYSTROPHY: poorly noursihed

Question 528

How many types of myotonic dystrophy are there

Answer 528

Type 1 (Steinert's disease) Type 2

Question 529

Where is the autosomal gene found in MD T1

Answer 529

Chromosome 19, long arm

Question 530

What is the autosomal gene in MD T1 called

Answer 530

DMPK

Question 531

What genetic error occurs in MD T1

Answer 531

A trinucleotide repeat of CTG

Question 532

What is DMPK responsible for

Answer 532

Myotonic dystrophy protein kinase: communication between muscle cells, heart and Brian cells Shuts off

Question 533

Where is the genetic mutation located in MD T2

Answer 533

Long arm of chromosome 3

Question 534

What is the gene involved in MD T2

Answer 534

CNBP (cellular Nucleic Binding Protein) which controls the genes in muscles of heart and muscles

Question 535

What genetic error causes MD T2

Answer 535

Instead of trinucleotide, it's a tetra nucleotide repeat: CCTG

Question 536

What is repeat expansion in Muscle Dystrophy

Answer 536

Increased CTG and CCTG repeats in the affected genes Caused by slipped ispairing

Question 537

What is slipped mispairing

Answer 537

Where the DNA polymerase gets confused and goes back to the beginning and starts again

Question 538

What is the result of repeat expansion

Answer 538

Increased number of repeats = earlier and more sever symptoms (ANTICIPATION)

Question 539

How many mutations of DMPK or CNBP are needed to cause disease

Answer 539

DMPK: 50 + CNBP: 75+

Question 540

Onset of symptoms in MD T1

Answer 540

Two forms: Congenital and adult form: Symptoms begin at birth Adults: Muscle weakness occurs later in life: facial: Eye dropping, hollow cheeks Distal Hand muscle weakness Muscles of lower leg: Foot Drop

Question 541

Onset of symptoms in MD T 2

Answer 541

Only presents at adulthood: Milder Affects proximal muscles of thighs and hips (Can't walk up stairs or stand up from sitting) Shoulder and elbow weakness: Can't pick things up Myotonia; Sustained muscle contractions, difficulty relaxing muscles after use Cataracts Insulin Resistance

Question 542

Diagnosis of MD

Answer 542

1. genetic 2. EMG 3. Muscle Biopsy

Question 543

Most common chromosomal disorder among live births

Answer 543

Down Syndrome

Question 544

What causes Down syndrome (eugenic error)

Answer 544

Nondisjunction of chromsoomes

Question 545

Signs of Down Syndrome

Answer 545

``` Flat Facial Profile Upward-slanting palpebral fissures Epicanthal Folds Low-Set Ears Excessive Skin at back of the neck k A single Transverse Palmar Crease Clinodactyly (curved fifth finger) Small nose and mouth Large Tongue ``` HAVING AN EXTRA 21 EFFECTS ALMOST EVERY ORGAN SYSTEM IN THE BODY

Question 546

CV complications of down's syndrome

Answer 546

1. Endocardial cushion defects (AVSDs) | 2. VSDs, ASDs

Question 547

Auscultation sounds in VSDs vs ASDs

Answer 547

1. VSDs: Harsh holosystolic murmur | 2. ASDs: Fixed Split S2 Heart Sound

Question 548

Gi Complications of trisomy 21

Answer 548

MOST COMMON: Duodenal Atresia = intestinal obstruction

Question 549

Symptoms of Duodenal Atresia

Answer 549

BEFORE the MAJOR DUODENAL PAPILLA (where the bile contents are pushed in) = NON-BILIOUS VOMITING AFTER: BILIOUS VOMITING

Question 550

Onset of duodenal atresia in down syndrome

Answer 550

Within hours of being born

Question 551

Abdominal X ray characteristic of duodenal atresia

Answer 551

Double Bubble Sign (stomach and duodenum is filled with air)

Question 552

Haematological consequence of trisomy 21

Answer 552

1. Increased risk of Acute Lymphoblastic Leukaemia (ALL) | Acute Myeloblastic Leukaemia (AML)

Question 553

Signs of ALL and AML in children to look out for

Answer 553

RECURRENT respiratory tract infections Anaemia and Laeukopenia on blood tests Increased 20% blast cells in bone marrow biopsy

Question 554

Urogenital symptoms of down syndrome

Answer 554

1. Males have decreased fertility or even sterility

Question 555

Neurological complication of down syndrome

Answer 555

1. LDs 2. Early onset Alzheimer's before 40 Amyloid precursor proteins on chromosome 21, which increases amyloid plaque build up and destroy neurone

Question 556

What is the atlantoaxial instability found in trisomy 21

Answer 556

Posterior transverse ligaments in the neck are lax or floppy - responsible for holding the first (atlas) and second (axis) cervical spines.

Question 557

Signs of atlantoaxial instability

Answer 557

Reduced cervical spine stability = cervical nerve root compression Presents with motor symptoms; Weakness in arms and legs or torticolis (head tilting)

Question 558

Prevention of atlantoaxial instability

Answer 558

Reduce neck extension

Question 559

What does the combined test consist of for down's syndrome

Answer 559

B-HCG (raised) and PAPP-A (low)

Question 560

What blood test is done for down's in the second trimester

Answer 560

``` Alpha feto protein (low) Beta HCG (high) Inhibin A (high) Unconjugated Oestriol (low) ```

Question 561

When are AFP levels raised

Answer 561

1. Multiple gestations 2. Open Neural Tube defects 3. Abdo Wall Defects

Question 562

How is karyotyping for down's conducted

Answer 562

Between 9-11 weeks: Chorionic-villus sampling Between 15-19 weeks: Amniocentesis After 20 weeks: Blood test

Question 563

What genetic mutation causes Angelman Syndrome

Answer 563

A mutation on chromosome 15, where UBE3A is not expressed and transcribed into E6AP

Question 564

What does UBE3A stand for

Answer 564

Ubiquitin-Protein Ligase E3A

Question 565

What is the role of E6AP

Answer 565

It tags other proteins with ubiquitin so they get degraded by the proteasome

Question 566

What mechanism regulates what parental gene expresses the UBE3A

Answer 566

Imprinting: Usually the mother will express her UBE3A gene, silencing the paternal chromosome with the same gene Leaves it vulnerable to mutation

Question 567

What genetic process causes the development of angel man syndrome

Answer 567

Deletion of hundreds of base pairs on chromosome 15 of the maternal gene Mutation within the gene to make it effective Paternal uniparental disomy: Or just an absent maternal chromosome, resulting in two paternal chromosomes: (that one parent contributed all the chromosomes for that one gene). Imprinting Defect: Mother's gene is mistakenly silenced

Question 568

Signs of angel man syndrome

Answer 568

Deletion of a gene nearby: OCA2: Which results in light complexion (compared to the family)

Question 569

Describe how the different mutational processes effect the symptoms seen in Aneglman syndrome

Answer 569

Mutations: Asymptomatic Deletion or Maternal Uniparental disomy causes Prader-Willi syndrome as these also result in changes to nearby genes.

Question 570

Signs of Prader-Willi syndrome in infants

Answer 570

Low muscle tone and poor feeding Delayed development: Never learn how to speak Can't walk properly (walking with arms pronated and flexed legs)

Question 571

Signs of Prader-Willi syndrome in adolescents

Answer 571

1. Overeating 2. Obesity 3. Low IQ 4. Inadequate Genital Development 5. Fair Complexion 6. Seizures 7. Small Head Size 8. Difficulty w/ sleep 9. Scoliosis Triad: LD, Seizures and Ataxia

Question 572

Behaviours in angel man syndrome

Answer 572

1. Happy 2. Excitable 3. Hand-Flapping

Question 573

What choromosome is affected in Williams Syndrome

Answer 573

Chromosome 7 (microdeletion of 26-28 genes including elastin, these happen randomly occur in gametes)

Question 574

Symptoms of Williams Syndrome

Answer 574

Connective Tissue Abnormalities (loss of elastin): - Broad Forehead - Flat nasal bridge - Perioribital puffiness - Short upturned nose - Long medial cleft - Full Lips, Wide Mouth Narrowing of blood vessels (pulmonary artery stenosis, supravalvular aortic stenosis = heart murmurs + hypertension They have a cocktail party personality Cognitive impairement Weak visual motor and spatial Anxiety Phobias OCD traits Increased sensitivity to vitamin D: Hypercalcaemia symptoms Excess calcium then forms kidney stones when excreted

Question 575

Diagnosis of Williams Disease

Answer 575

Microarraqy analysis: to check for elastin | FISH

Question 576

Describe the process of iron absorption at the intestinal level

Answer 576

Fe3+ -> Fe2+ before being absorbed. HCL activates Ferrireductase to do this.

Question 577

Other than iron deficiency anaemia, what is another cause for microcytic anaemia in children

Answer 577

Lead poisoning from ingesting lead-containing paint in toys.

Question 578

What enzymes does lead inhibit to cause anaemia

Answer 578

1. alpha-ALA 2. Ferrochelatase 3. rRNA Degradation This leads to stippling: build up of substances inside the cell. (basophilic stippling on a peripheral blood smear).

Question 579

What type of inheritance is alpha-thalassaemia

Answer 579

Autosomal Recessive

Question 580

Signs of 3 defective alpha genes in thalassaemia

Answer 580

HbH Disease: Causes excess beta chains (4 beta chains in haem). Damage the red blood cell membrane = haemolytic in bone marrow, extravascular haemolytic in the spleen Has a very high affinity for oxygen and causes hypoxia in tissues This causes the bone, live and the kidneys to produce excess RBCs = enlargement of bones, liver and spleen. Pallor, SOB, fatiguability Skeletal deformitie, especially skull or face deformities, making them brittle (increased fractures) Hepatosplenomegaly

Question 581

Signs of 3 defective alpha genes in thalassaemia

Answer 581

1. Hb Bart's Hydrops Foetalis Where gamma chains in foetus form tetramers instead.: Very high affinity for oxygen = hypoxia and death Failed CO Hepatosplenomegaly = Diffuse and severe oedema all over the foetus (Hydrops fettles). So incompatible with life

Question 582

Blood test results n thalassaemia

Answer 582

1. Reduced Haemoglobin 2. Microcutic (so reduced MCV) 3. Reduced mean corpuscular haemoglobin

Question 583

Blood smear result in alpha thalassaemia

Answer 583

Target cells

Question 584

Treatment for thalassaemia

Answer 584

Iron Chelating Agents

Question 585

What is thalassaemia minor

Answer 585

Carriers (heterozygotes)

Question 586

What is thalassaemia mintermedia

Answer 586

3 alpha gene deletion (so not complete thalassamia)

Question 587

Pathophysiology of beta thalassaemia

Answer 587

Lack of alpha chains causes teranomers of beta chains that deposit and cause inclusion inside the RBCs. Damages the red blood cell membrane = haemolytic in the bone marrow or the spleen.

Question 588

Describe the symptoms of beta thalassaemia

Answer 588

Eventually beta chains are broken down and the haemorrhage -> bilirubin + iron (as there is excess beta chains = jaundice and iron causes 2ndary haemochromatosis).

Question 589

Blood tests for beta thalassaemia

Answer 589

Reduced Hb, MCV abd MCH

Question 590

When does beta thalassaemia majorr present

Answer 590

First year to 18 months of life as hbF drops. Frontal bossing of skull, maxillary swelling and hepatosplenomegaly.

Question 591

Management of thalassaeia

Answer 591

Regular blood transfusion Desferrioxamine to chelate iron Splenectoy Bone marrow transplantation is the only cure.

Question 592

What type of anaemia is hereditary spherocytosis

Answer 592

Normocytic

Question 593

What is hereditary spherocytosis

Answer 593

1. Inherited RBC membrane defect causing destabilisation of RBC membrane = excessive haemolytic as the blood cells are faulty

Question 594

Symptoms of hereditary spherocystosis

Answer 594

1. Anaemia, reticulocytosis (lots of premature red blood cells), hyperbilirubinaemia from excessive haemolytic 2. Splenomegaly

Question 595

What people usually have hereditary spherocystosis

Answer 595

Northern European

Question 596

Complications of hereditary spherocystosis

Answer 596

Jaundice

Question 597

Diagnosis of Hereditary spherocystossi

Answer 597

1. Reticulocytosis | 2. Sphere-shaped (spherical) RBCs.

Question 598

Treatment of hereditary spherocytosis

Answer 598

Splenectomy

Question 599

How long do RBCs live for

Answer 599

120 days

Question 600

Two types of immune Haemolytic Anaemia

Answer 600

Warm (where haemolytic occurs at 37 degrees) and Cold (When people's blood is exposed to cold temperatures) - 0-10 degrees so when going out in the snow.

Question 601

What causes warm IHA

Answer 601

IgG - Warm Agglutinins

Question 602

What antigen do warm agglutinins bind to

Answer 602

Rh antigens NK cells and CD8 T cells bind to Fc receptor and destroy the RBC

Question 603

Symptoms of intravscualer acute cold IHA

Answer 603

1. Intravascular haemolytic leads to Raynaud's phenomenon.

Question 604

Signs of intravascular IHA

Answer 604

1. Increased haemolytic = increased reticulocytes produced by bone marrow so reticulocytosis (normocytic anaemia) 2. Lactate Dehydrogenase in killed RBCs leaks = Increased LDH levels Increased bilirubin from head In intravascular haemolysis, haemolytic that is bound to haptoglobin is broken down into bile. Increased haptoglobin and bile Haemoglobinuria if haptoglobin gets overwhelmed. Renal insufficiency

Question 605

Where is haemolytic stored

Answer 605

In renal tubules as haemosiderin

Question 606

Symptoms of extravascular IHA

Answer 606

1. Haptoglobin is normal | 2. No haemoglobin or haemosiderinuria

Question 607

In what children are warm IHAs common in

Answer 607

``` Precedes: Viral infetcions SLE Lymphomas Leukaemia ```

Question 608

What antibiotics can cause warm IHA

Answer 608

Penecillin | Cephalosporins

Question 609

What are causes of acute cold IHA in children

Answer 609

1. Viral pneumonia 2. Mycoplasma 3. Infectious Mononucleosis

Question 610

Symptoms of haemolytic crisis

Answer 610

1. Bounding HR 2. SOB 3. Multiorgan failure from hypoxia Chronic: fatigue, pallor, sob and jaundice.

Question 611

Treatment of warm IHA

Answer 611

Oral Prednisolone

Question 612

What antibody is prevalent in cold IHA

Answer 612

IgM

Question 613

What is cold acute IHA also known as

Answer 613

Paroxysmal Cold haemoglobinuria as its secondary to infections and vaccines

Question 614

Blood test results in IHA

Answer 614

1. Normocytic aenamia 2. Reduced haptoglobin 3. Increased reticulocytes 4. Increased LDH Direct Coombs and Antigen testing also

Question 615

Under what three conditions do sickle-shaped Hb molecules clump together

Answer 615

1. Acidosis 2. Hypoxia 3. Dehydration

Question 616

Two main consequences of sickle cells

Answer 616

1. Vaso-occlusion | 2. Haemolysis: intravascular or extravascular

Question 617

Signs of intravascular haemolyssi

Answer 617

Raised haemoglobin and LDH Increased unconjugated bilirubin as Haptoglobin is reduced

Question 618

Signs of Sickle cell anaemia

Answer 618

1. Increased bilirubin: Scleral Icterus, jaundice and bilirubin gallstones 2. Anaemia 3. Reticuloytosis 4. Expansion of medullary cavities in bone marrow as it increases reticulocyte production (hair on end appaerance on X ray in the skull). 5. Hepatosplenomegaly Vasoocclusion: Tissue ischameia and pain (sickle cell crisis) Pain starts suddenly and excruciating Typically affects the same body parts (e.g., bones in hands and feet - dactlyitis or avascular necrosis in the hips, priapism, strokes and mental status changes). Moyamoya Disease (puff of smoke in lungs) Haematuria or proteinuria Acute Chest Syndrome (Chest pain, SOB and cough) Causes further hypoxia and sickling Splenic Sequestration: LIFE THRETENING

Question 619

Someone gets a blood test and has a rapid drop in haemoglobin and goes into hypovolaemic shock, what anaemia condition has caused this

Answer 619

Splenic Sequestration

Question 620

Chronic complications of sickle cell disease

Answer 620

1. Growth and developmental delay 2. Learning and Behaviour Issues 3. Pulmonary HTN 4. CKD 5. Auto Splenectomy (fibrosis and shrinkage of the spleen - infections from strep pneumoniae COMMON IN CHILDREN)

Question 621

What precipitates encapsulated organism infections from SCD

Answer 621

No vaccination, penecillin V prophylaxis and parvovirus

Question 622

What ENT problems are caused by SCD

Answer 622

Adenotonsillar hypertrophy (COMMON)

Question 623

Diagnosis of SCD

Answer 623

1. Raised reticulocytes | 2. Hb Electrophoressis GOLD STANDARD

Question 624

What type of anaemia is SCD

Answer 624

Microcytic

Question 625

Maintenance treatment of SCD

Answer 625

1. Avoidance 2. Penecillin V prophylaxis 3. Oral folic acid 5. HYDROXYCARBAMIDE to reduce crisis. Bone marrow transplant

Question 626

What is Diamond-Blackfan syndrome

Answer 626

1. A defect that leads to a reduction in bone marrow RBC production

Question 627

Onset of DBS

Answer 627

First year of life as svere anaemia

Question 628

Signs of DBS

Answer 628

Cleft palate, deafness, MSK and short stature

Question 629

Blood test findings for DBS

Answer 629

1. REDUCED reticulocytes

Question 630

Treatment of DBS

Answer 630

Oral prednisolone

Question 631

What causes transient eyrthroblastopenia of child hood

Answer 631

1. From Parvovirus, EBV, Drugs or hereditary spherocytosis)

Question 632

What is transient eyrthroblastopenia of childhood

Answer 632

1. Slow development of pallor and anaemia Treatment; Remove underlying causes and transfusion if needed. Rsolves by itself tho

Question 633

Neonatal causes of polycythaemia

Answer 633

1. Hypertransfusion; Delayed cord clamping, twin to twin transfusion , maternal-foetal transfusion Endocrine: GDM in mother, CAH and neonatal thyrotoxicosis Maternal: GHTN, Heart Disease SYndormic: Down syndrome and Dehydration

Question 634

Causes of polycythaemia in older children

Answer 634

1. Polycythaemia rubra 2. Familial history of high O2 affinity Secondary to anything causing increased erythropoietin: CHD (REALLY COMMON), Respiratory disease, Chronic obstructive sleep apnoea, chronic alveolar hypoventilation, obesity, altitude, renal cysts/carcinoma, dehydration

Question 635

Consequence of polycythaemia in neonates

Answer 635

Hyperviscocity Syndrome: Hypotonia, Congestive heart failure, Tachypnoea, seizures, abnormal renal function and NEC

Question 636

Symptoms of vWD

Answer 636

1. Mucosal bleeding Easy Bruising Excessive bleeding from dental surgery, trauma and menorrhagia

Question 637

Investigations for vWD

Answer 637

1. APTT raised (as Factor VIII is low in activity), platelet count NORMAL

Question 638

Most common cause of acquired aplastic anaemia

Answer 638

Idiopathic

Question 639

What causes aplastic anaemia

Answer 639

Bone marrow suppression of RBCs: usually results in pancytopenia

Question 640

What causes paroxysmal nocturnal haemoglobinuria

Answer 640

Acquired clonal disorder of marrow cells deficient in glycosylophosphatidylinositol (GPI), which prevents against lysis

Question 641

Investigations results in Paroxysmal nocturnal haemoglobinuria

Answer 641

1. Raised reticulocytes, reduced WBCs and platelets.

Question 642

What is Fanconi's Anaemia

Answer 642

1. Affects all three haemopoietic cell precursors (lymphoid and myeloid progenitor cells)

Question 643

Presentations of fanconi's anaemia

Answer 643

1. Bruising or insidious onset anaemia ``` Short stature Skin Hyperpigmentation (cafe au lait), skeletal abnormalities) ```

Question 644

Imvetsigation findings in fanconi's anaemia

Answer 644

1. FBC: Pancytopenia | Bone Marrow: Hypoplastic, dyserythropoietic or megaloblastic change)

Question 645

Treatment in fanconi's anaemia

Answer 645

1. RBC transfusion, hearing aids and orthopaedic Corticosteroids (oxymetholone)

Question 646

What would causes an increase in PT but not in APTT

Answer 646

Deficiency of VII, vit K deficiency and warfarin therapy

Question 647

What would causes a stable PT but an increase in APTT

Answer 647

1. Deficiency of VII, IX, XI, XII, haemophilia A, vWD disease,

Question 648

When is a d-dimer test used

Answer 648

DVTs

Question 649

What is primary haemostasis

Answer 649

Formation of the platelet plug and adhesion to the collagen of the endothelium wall

Question 650

What is haemophilia A

Answer 650

Deficiency in factor 8

Question 651

What is haemophilia B

Answer 651

Defieincy In factor 9 So they both affect the intrinsic pathway

Question 652

What is an acquired cause of haemophilia

Answer 652

Liver Failure. Vit K deficiency DIC

Question 653

Presentation of Haemophilia

Answer 653

1. Ecchymosis 2. Haematomas 3. Prolongued bleeding after cut or surgery 4. GI bleeding 5. Haemturia 6. Haemarthrosis (joint bleeding), painful, swelling and tender. 6. Strokes, Increased ICP

Question 654

Investigations in haemophilia

Answer 654

1. Normal platelet count 2. PT: (7,10,5,2 and 1) 3. APTT: Intrinsic pathway (12, 11, 9, 8) - 9 and 8 means PTT is prolonged and PT is normal.

Question 655

Treatment of haemophilia

Answer 655

Treat with recombinant FVIII product or FVIII inhibitors. For A ONLY: Desmopresssin (DDAVP), stimulates vWF release and stabilises VIII

Question 656

What is wrong with FVIII inhibitors

Answer 656

They diminish the effectiveness over time and cause anaphylaxis

Question 657

What causes Diamond-Blackfan Anaemia

Answer 657

Mutations in ribosomes

Question 658

What is important to remember about the investigation results that indicate diamond-blackfan anaemia

Answer 658

WBCs and Platelets are normal. Just RBCs affected.

Question 659

What is Scarlet Fever

Answer 659

A febrile illness characterised by pharyngitis and a progressive erythematous rash (starting from one area and spreading through the body)

Question 660

Two characteristic symptoms of glomerulonephritis

Answer 660

DARK URINE and perirobital oedema

Question 661

First Line Investigation for Slipped Upper Femoral Epiphyses

Answer 661

X-Ray of BOTH hips (AP and frog leg views)

Question 662

Describe the pattern of rash seen in Pityriasis Rosea

Answer 662

Starts with a Herald's patch which is a small pink or red oval patch This then becomes widespread after 2 weeks. These are small red scaly patches all over the body. The rash may be dark brown or black.

Question 663

What is pityriasis Versicolour

Answer 663

Golden. light coloured spots (cream) found across the whole back and chest

Question 664

What is an infantile Colic

Answer 664

Repeated episodes of excessive or inconsolable crying that lasts for at least 3 hours a day, 3 days a week with no evidence of faltering growth

Question 665

When do infantile colics typically resolve

Answer 665

3-4 months of age

Question 666

What virus is most commonly associated with croup

Answer 666

Parainfluenza virus

Question 667

How is Respiratory Distress Syndrome Treated

Answer 667

DIrect Surfactant replacement

Question 668

Outline the trend of infections caused by poliovirus

Answer 668

Initially invades the intestines before travelling into the spinal cord to cause nerve damage

Question 669

How does polio enter the body

Answer 669

Through contaminated food and water (faeco-oral) Droplet infection

Question 670

Pathophysiology of polio

Answer 670

Enters the ventral horn of motor neurones, causing inflammation when surrounding immune cells come into the area = DAMAGE This causes muscle atrophy and becomes weak

Question 671

Types of polio

Answer 671

Bulbar Polio = Brainstem problems (speaking and swallowing) Also stops diaphragmatic breathing

Question 672

What is post-polio syndrome

Answer 672

After a neurone innervating a muscle gets damaged, neighbouring neurones form collaterals to reinnervate the muscles. However, ageing causes neurones to die anyways which destroys these neurones and causes muscle dystrophy again

Question 673

Symptoms of Polio

Answer 673

1. HIGH GRADE FEVER 2. Intense muscle pain from spasm and weakness 3. Loss of Muscle Reflexes 4. Paralysis 5. Develops over a few days 6. Assymmetric AFFECTS LARGER PROXIMAL MUSCLES OF THE LEG FIRST

Question 674

Symptoms of polio in infants

Answer 674

Develop acute flaccid paralysis Breathing difficulties and death

Question 675

Side-effect of the polio vaccine

Answer 675

Can cause vaccine- associated paralytic polio from re-activation and mutation of the polio in the vaccine Also can cause Vaccine Derived Poliovirus (VDPV)

Question 676

Most common cause of Bacterial Meningitis in Infants (under 3)

Answer 676

Group B strep E. coli Listeria

Question 677

Most common cause of bacteria meningitis in adolescents

Answer 677

N. Meningitidis Strep. pneumoniae H. Influenza

Question 678

Name two diseases that can spread to the brain and cause acute bacterial meningitis

Answer 678

1. Mycobacterium TB | 2. Neurosyphilis

Question 679

What usually causes meningoencephalitis (COMBO)

Answer 679

HSV

Question 680

What causes acute encephalitis only

Answer 680

1. Measles (up to 10 years after first infection) | 2. VZV

Question 681

Signs of meningitis in infants

Answer 681

VERY SUBTLE: | Can just be irritability and poor feeding

Question 682

Name tow signs that are positive in Meningitis and what do they do

Answer 682

Kernig's sign - Flex the hip to 90 degrees and try to extend the knee (pain = positive) Brudzinski's sign - Involuntary flexion of the hip when the neck is flexed (positive = pain)

Question 683

In what types of meningitis is protein levels usually elevated (they are normal in some)

Answer 683

Bacterial | TB

Question 684

In what type of meningitis is CSF glucose levels normal

Answer 684

Viral (low in the others0

Question 685

LONG TERM COMPLICATIONS OF HUS

Answer 685

1. PERSISTENT HYPERTENSION | 2. Proteinuria/ AKI

Question 686

What bacteria causes scalded skin syndrome

Answer 686

Staph Aureus

Question 687

What is Scalded Skin Syndrome

Answer 687

Protease enzymes released by Staph Aureus, causes the skin to be broken and worn down

Question 688

Onset of scalded skin syndrome

Answer 688

5 years

Question 689

Presentation of Scalded Skin Syndrome

Answer 689

1. Starts with a generalised patch of erythema -> skin looks thin and wrinkles -> bullae which then fall off to look like scalded skin Also has b symtoms

Question 690

Management of Scaled Skin Syndrome

Answer 690

Admission to hospital

Question 691

Name some abnormalities that need to be checked for in spina bifida before surgery can take place

Answer 691

Club feet Kyphosis Contractures Airways

Question 692

What causes congenital hyperinsulinaemia

Answer 692

High circulating insulin in foetus' blood because of DM in the mother (remember hyperglycaemia -> hypoglycaemia due to insulin overload)

Question 693

What are tocolytics

Answer 693

Either beta blockers or calcium channel blockers -> delay the contractions in th euterus

Question 694

What is aplasia cutis congenita

Answer 694

Loss of the dermal and epidermal layer in the skin (seen in Pate)

Question 695

REMEMBER, ANTIEPLIEPTIC DRUGS ARE MOST COMMONLY STOPPED BECAUSE OF SKIN SENSITIVITY ReACTIONS

Answer 695

N/a

Question 696

Scalded skin syndrome vs Toxic Ependermal Necrolysis/Steven-Johnson Syndrome

Answer 696

Only affects the upper epidermis and spares the mucous membranes, while toxic epidermal necrolysis affects the dermal-epidermal junction.

Question 697

Are retinoblastoma bilateral or unilateral

Answer 697

Bilateral, will come with both pupils being very white during red reflex test

Question 698

What is the weber and rhino's test and what do they indicate

Answer 698

Rinne: Place tuning fork on the mastoid bone and tell the patient to indicate when the sound is no longer being heart. With conductive loss, bone conduction > air conduction (in that, they will be able to hear it for a longer time than usual) Weber's test: Place tuning fork on forehead. There should be lateralisation of sound to the affected ear

Question 699

What electrolytes do you need to monitor in Diabetes Type I (DKA)

Answer 699

K+! It moves into the cells when insulin is given = hypokalaemia

Question 700

How do we find the volume of IV fluids that are needed to be given in DKA

Answer 700

Weight = L L * 10 (assume 10% fluid deficit in children and young people with DKA) Divide by 48 hours (as this is being given over 48 hours)

Question 701

What cells produce insulin

Answer 701

Beta cells

Question 702

What cells produce glucagon

Answer 702

Alpha cells

Question 703

What types of cells are beta and alpha cells

Answer 703

Secretory cells arising form the islets of langerhand

Question 704

What is the role of insulin

Answer 704

Insulin binds to insulin receptors on muscle and adipose tissues, causeing vesicles (glucose transporters) to fuse with the cell membrane and inserting more glucose channels into the surface = increased uptake of glucose

Question 705

What in the past medical history, would indicate type I Diabetes Mellitus

Answer 705

AUTOIMMUNE (as it's an autoimmune disease): e.g., Hashimoto's thyroiditis, Vitiligo or Lupus

Question 706

Pathophysiology of Type I Diabetes

Answer 706

Immune System attacks the Glutamic Acid Decarboxylase enzymes inside Beta cells = reduction of GABA which is needed to stimulate insulin release. This decreases the number of beta cells in the islet of Langerhans = less insulin = hyperglycaemia So, the tissues remain sensitive to insulin, there's just less insulin secreted

Question 707

Name of the antibodies against beta cells

Answer 707

GAD-antibdoies ANTI-ISLET CELL ANTIBODIES

Question 708

Four clinical symptoms of DM 1

Answer 708

Polyphagia (weight loss + increased appetite) Glycosuria Polyuria Polydipsia

Question 709

What causes weight loss in DM 1

Answer 709

Lipolysis of adipose tissues Proteolysis of muscle tissues This is because glucose can't get inside cells = lack of nutrition

Question 710

What is the difference between DM 1 and 2

Answer 710

Insulin is produced in 2, but tissues do not fully respond So body starts producing more insulin (compensation phase by beta cells) Over time, these overworked bet cells get exhausted and die off = insulin levels start to lower.

Question 711

What other substance is also produced by beta cells in conjunction to insulin

Answer 711

Amyloid Polypeptide (waste products) These increase alongside increased insulin production

Question 712

Histology findings in DM 2

Answer 712

Amyloid polypeptide aggregates in the pancreas

Question 713

Pathophysiology of Diabetic Ketoacidosis

Answer 713

Increased lipolysis in fat tissues = more free fatty acids These are broken down by the liver to form ketone bodies to supply cells for energy However, it increases acidity in the blood

Question 714

Why does DKA not happen in DM 2

Answer 714

Because insulin is still being produced by the islets (even if it's low levels), stopping lipolysis

Question 715

Signs of DKA

Answer 715

1. Dehydration 2. Kussmaul Respiration (sweet and fruity acetone), deep and rapid breaths to reduce acidity Abdominal Pain, Nausea and Vomiting, Delirium and Psychosis

Question 716

Complications of DKA

Answer 716

1. Acute Cerebral Oedema (as excess glucose increases oncotic pressure, causing water to draw out of the cells and into the ECF 2. Cardiac Arrhythmia from K+ imbalance

Question 717

Signs of Metabolic Acidosis

Answer 717

Reduced pH and Bicarbonates

Question 718

Why do we get Hyperkalaemia in DKA/DM

Answer 718

Because insulin is responsible for opening the Na+/K+ co transporter. As there is low insulin, the channels do not open, leaving K+ in the blood

Question 719

Treatment of DKA

Answer 719

1. IV fluids 2. IV Insulin 3. IV K+ to prevent hypokalaemia

Question 720

Complication of DM 2

Answer 720

Hyperosmolar Hyperglycaemic state

Question 721

What is Hyperosmolar Hyperglycaemic state

Answer 721

Where the glucose acts as a solvent, causing water to be drawn out from tissues into the blood = SEVERE dehydration Can cause coma and death

Question 722

What causes GDM

Answer 722

The mother's inability to produce enough insulin to overcome Placental Human Lactinogen (insulin suppressor)

Question 723

Name two systemic disorders that may cause increased insulin resistance or DM

Answer 723

Cushing's | Glucocorticoids

Question 724

Name two DM 2 medications that can cause weight gain

Answer 724

Sulfonylureas (glicazides) Thiazolidinediones

Question 725

Name one DM 2 medication that may cause weight loss

Answer 725

GLP-analogue

Question 726

What DM 2 medication does not cause cardiac problems

Answer 726

Thiazolidinediones

Question 727

How are GLP-1 and DPP-IV inhibitors given and how do they work

Answer 727

SC injections GLP-1: Incretins, so they reducee blood sugar DPP-IV: Stops degradation of GLP-1

Question 728

How much fluid do children need a day

Answer 728

Infant: 150ml/kg/Day Toddlers: 100ml/Kg/Day

Question 729

Name two conditions that may cause dysphagia and thus dehydration in children

Answer 729

Cerebral Palsy | Developmental Delay

Question 730

Indications for enteral feeds

Answer 730

A person on enteral feeds usually has a condition or injury that stops them from eating by mouth but has a functioning GI tract

Question 731

Types of Enteral feeds

Answer 731

NG Tubes: Acute swallowing Pain, Inadequate oral intake (SHORT TERM) Naso-Jejunal (Delayed Gastric Emptying or STomach/oesophageal pathology) - SHORT TERM Percutaenous Endoscopic Gastroscopy Gastrostomy (Head and. neck cancer, Brain Injury) - LONG TERM Jejunostomy (Gastroparesis, same as NJ but long term) - LONG TERM

Question 732

Management of Dehydration in children

Answer 732

Not - Dehydrated: Needs maintenance fluid Dehydrated: Maintenance + Deficit 50ml/Kg Shocked: Maintenance + Deficit 100ml/kg + bolus

Question 733

How much glucose should be given to neonates and why

Answer 733

IV 10% Glucose as they have high metabolism and no fat reserves

Question 734

Rate at how much IV fluids should be given

Answer 734

Day 1 - 60mls/kg/day 2 - 90 3 - 120 4 - 150

Question 735

Formula for weight estimation in children

Answer 735

(Age + 4) x 2 = weight in KG

Question 736

What IV Hydration solution is given to children

Answer 736

0.9% NaCl + 5% Glucose (+/- KCL, if DKA or Sepsis)

Question 737

How do we calculate the daily (ml/24kg) fluid intake to give to a child

Answer 737

First 10kg: 100ml/kg Next 10kg: 50ml/kg Every other kg: 20ml/kg

Question 738

How do we correct Fluid Deficit

Answer 738

REMEMBER: Not Dehydrated - Maintenance Dehydrated (5%) = 50 pls/kg extra Shocked (10%) = 100mls/kg extra Formula: Deficit (%) * 10 * Weight

Question 739

Calculate the rate of rehydration fluids that should be given to an 18 months girl, weighing 12kg - 4 Days diarrhoea + Vomiting - Alert - CRT < 2 Secs - Dry Lips and Mucosal Membranes - Reduced Urine Output

Answer 739

First 10kg = 1000mls Next 2kg = 100mls Appears to be 5% dehydrated so, 5 x 12 x 10 = 600 600 + 1000 + 100 = 1700 1700/24 = 70.8 ml/hr

Question 740

What is the given fluid bolus in severe dehydration

Answer 740

20mls/kg of 0.9% NaCl

Question 741

When would this fluid bolus be reduced to 10

Answer 741

Diabetic Ketoacidosis

Question 742

Prenatal causes of Cerebral Palsy

Answer 742

1. Radiation 2. Infection 3. Hypoxia

Question 743

What is the main type of cerebral palsy

Answer 743

Spastic (70% of cases)

Question 744

What is Spastic Cerebral Palsy

Answer 744

Tight/Stiff muscles from damage to the upper motor neurones - impairs the ability to receive GABA (needed to inhibit movements) Hypertonia

Question 745

Signs of Spastic Cerebral Palsy

Answer 745

Scissor Gait (Adductor muscles are constantly flexed) Tow Walk (As calves are always flexed)

Question 746

What is Dyskinteic Cerebral Palsy

Answer 746

Damage to the Basal Ganglia, resulting in involuntary movement (think Huntington's). Chorea (Slow and uncontrolled movements moving from muscle to muscle) and Dystoonia (slow and uncontrolled movements)

Question 747

What is Ataxic Cerebral Palsy

Answer 747

Damage to the cerebellum, resulting in clumsy and unstable movement and balance

Question 748

Non-Secific symptoms of cerebral palsy

Answer 748

1. Failure to meet milestones 2. Increased or decreased tone 3. Problems with co ordination, speech or walking 4. Feeding or swallowing Problems 5. LD

Question 749

What structure is damaged to cause kernicterus

Answer 749

Basal Ganglia

Question 750

What phototherapy is used in Jaundice

Answer 750

Blue Light, 450nm

Question 751

What factor reduces the risk of necrotising entercolitis

Answer 751

Breast Milk

Question 752

Signs of Retinoppathy of Prematurity

Answer 752

The formation of a fibrous bridge on the retina Retinal haemorrhages and detachment

Question 753

Treatment of Retinpathy of Prematurity

Answer 753

VGEF Laser Therapy

Question 754

Management of Nocturnal Eneuresis

Answer 754

Under 5: Reassurance, that it is likely to resolve without any treatment If it persists: enuresis alarm and then desmopressin for 7 weeks

Question 755

treatment of Crohn's (it's not 5-ASA, that's for UC)

Answer 755

Prednisolone, methylprednisolone or IV Hydrocortisone.

Question 756

First line treatment for GORD in children

Answer 756

Aligini acid (1-2 weeks) If this does not work, a FOUR WEEK trial of a PPI or H2 receptor antagonist should be considered.

Question 757

When are symptoms for croup, the worst?

Answer 757

During the night

Question 758

When is croup more common in th year

Answer 758

Autumn

Question 759

Scarlet fever vs Kawasaki Disease

Answer 759

Scarlet fever also has a strawberry tongue... But affects older children (over 5), while Kawasaki affects under 5. SCARlet Fever -> Scar from lion licks the sand (rough sandpaper rash) with his strawberry tongue. The rash in Kawasaki is also started by exposure to light

Question 760

Septic Arthritis vs Transient Synovitis

Answer 760

Septic Arthritis would exhibit with >12 WCC and over 40 ESR

Question 761

Name three heart conditions in which a pan-systolic murmur happens

Answer 761

Mitral Regurgitation (5th Intercostal space, mid-clavicular line) Tricuspid Regurgitation 9fifth intercostal space, left sternal border) VSD - lower left sternal border

Question 762

What three heart conditions have a ejection-systolic murmur

Answer 762

1. Aortic Stenosis (Second intercostal space, right sternal border). 2. Pulmonary Stenosis (Second Intercostal space, left sternal border) 3. Hypertrophic Obstructive Cardiomyopathy (fourth intercostal space on left sternal border)

Question 763

Characteristic murmur in ASD

Answer 763

Fixed split heart sound

Question 764

Murmur characteristic of patent ductus arteriosus

Answer 764

Continuous systolic murmur (machinery/crescendo-decrescendo)

Question 765

Characteristic murmur in Tetralogy of Fallot

Answer 765

PULMONARY STENOSIS (Ejection systolic murmur at second intercostal space, left sternal border)

Question 766

What should be done if an innocent heart murmurr gets worse on standing

Answer 766

Sent to hospital (innocent murmurs should get quite on standing).

Question 767

In what order does a girl go through puberty

Answer 767

Thelarche -> Pubarche -> Growth Spurt -> Menarche (the last phase of puberty)

Question 768

What is a complication of heart surgery (e.g., percutaneous closure of VSDs)

Answer 768

Pericardial Effusion

Question 769

What stain is used to check for TB

Answer 769

Ziehl-Neelson test

Question 770

How does atopic dermatitis present in the first year of life

Answer 770

Confluent erythema, papular, scaly and crusty rash that is mostly present on the head and upper extremities only

Question 771

What is the gold standard test to diagnose lactose intolerance

Answer 771

Elevated breath hydrogen levels: This is because lactose-containing products cannot be digested so are metabolised by bacteria to produce H+

Question 772

Signs of poliomyelitis

Answer 772

Hypotonia and Hyporeflexia Neck stiffness Remember: FLACCID PARALYSIS

Question 773

What are the complications of rhinosinusitis

Answer 773

Orbital cellulitis Meningitis Osteomyelitis Which is why rhinosinusitis must be treated

Question 774

What is a consequence of Shaken Baby Syndrome

Answer 774

Subdural Haematoma (look out for retinal haemorrhages)

Question 775

What's a major consequence of Rickets that affects children in the first year of life

Answer 775

Seizures: Hypocalcaemic seizures

Question 776

What is the thyroglossal duct

Answer 776

A tiny canal that links the thyroid gland and ducts at the front of the neck. This usually closes in the third month of life (however, this doesn't close in some people)

Question 777

What is a thyroglossal duct cyst

Answer 777

Where the duct fills up with mucous

Question 778

Complication of a thyroglossal duct cyst

Answer 778

The cyst can burst open -> Discharging sinus (thyroglossal fistula Thyroid glands may not migrate down the throat to where they should be = hypothyroidism

Question 779

Examination findings in a thyroglossal duct cyst

Answer 779

Moves up when sticking out the tongue Painless swelling in front, midline of the neck Refer to surgery (remove the hyoid bone as well)

Question 780

What causes a Branchial cyst

Answer 780

Where the second branchial cleft fails to form during foetal development -> Space in the lateral aspect of the neck which can fill with fluid.

Question 781

Where are Branchial cysts found

Answer 781

Between angle of the jaw and sternocleidomastoid muscle

Question 782

Examination findings in Branchial cysts

Answer 782

Transilluminate with light

Question 783

What is a Branchial Cleft Sinus

Answer 783

An abnormal connection fo the branchial cyst to the skin (there will be a small visible hole/discharge from the surface of the skin)

Question 784

What is a normal. healthy foreskin

Answer 784

Retracting the foreskin, forms a rose bud on the end (will not fully retract and that's okay)

Question 785

What is BXO

Answer 785

A type of balanitis (white) - similar to Lichen Sclerosis Affects the glands and scarring of the urethral meatus (urinary retention) Treatment: Circumcision

Question 786

What does a Smegma cyst look like

Answer 786

White swelling under most of the foreskin

Question 787

How do we differentiate between an inguinal hernia or a hydrocele

Answer 787

Hydrocele, you can get above the swelling (there is a gap between the swelling and the external inguinal ring).

Question 788

What type of inguinal hernias are more common in children

Answer 788

Indirect Inguinal Hernia: Usually caused by abdominal wall defects Lateral to the inferior epigastric vessels

Question 789

What side do inguinal hernias usually present

Answer 789

Right

Question 790

When should a child with an enlarged lymph node, be referred to paediatric surgery

Answer 790

> 2cm Inflamed for over 2 weeks Enlarging

Question 791

What is different about managing a dermoid cyst found in the midline of the face

Answer 791

Possibly connected to CSF, needs to have an MRI before draining

Question 792

Management of an umbilical hernia

Answer 792

Leave alone until the age of 5

Question 793

Where are capillary sites taken from: a) under 6 months b) Over 6 months

Answer 793

Under 6 months - foot Over 6 months - 3rd or 4th digit

Question 794

Abdominal USS finding in Biliary Atresia

Answer 794

Absent Gallbladder or Irregular Absence of common bile duct

Question 795

At what age can a baby sit up upsupported

Answer 795

6 Months

Question 796

At what age can a baby pull themselves to stand

Answer 796

9 Months

Question 797

At what age can a baby jump

Answer 797

3 Years

Question 798

At what age to children smile spontaneously

Answer 798

6 Weeks

Question 799

At what age does a child use a fork and spoon

Answer 799

12 month s

Question 800

At what age can a child undress

Answer 800

2 Years

Question 801

At what age can a child dress with no help

Answer 801

4 Years

Question 802

By what age is not sitting up or walking concerning

Answer 802

Not sitting by 1 Year Not walking by 18 Month s

Question 803

By what age is having no hand preference concerning

Answer 803

18 Month s

Question 804

By what age is not smiling concerning

Answer 804

3 Months

Question 805

By what age is making no clear words concerning

Answer 805

18 Months

Question 806

By what age is no response to carer interactions concerning

Answer 806

8 Weeks

Question 807

By what age is still ignoring peers and playing with them concerning

Answer 807

3 Years

Question 808

What is the most common type of ASD

Answer 808

Ostium Secundum

Question 809

What Are the following ASD types: a - Ostium Secundum ASD b - Ostium Primum ASD c - Sinus venous ASD

Answer 809

Ostium Secundum - a hole between two atria Ostium Primum - Split in one of the leaflets of the mitral valve Sinus venous ASD: Drainage of the pulmonary veins into the left atrium rather than the right

Question 810

Pathophysiology of Henoch-Schonlein Purpura and lab results

Answer 810

IgA are made that directly targets the host's own endothelial cells

Question 811

What distinguishes Henoch-Schlonlein Purpura over other rashes

Answer 811

The purport is palpable above the skin

Question 812

Signs of Henoch-Schonlein Purpura

Answer 812

GI Tract: Abdominal Pain Kidneys: Haematuria IgA Nephropathy

Question 813

When should Whooping cough be the first suspected infection in a child

Answer 813

An acute cough lasting 14 DAYS - Paroxysmal cough - Inspiratory whoop - Post-pertussive vomiting - Undiagnosed apnoeitic attacks/cyanosis in young infants

Question 814

What defines compensated shock over late shock

Answer 814

Early shock: BP is normal, HR is fast, responsible is fast and they are blue Late shock: Hypotension, Bradycardiac, Kussmaul respirations and Anuria

Question 815

What defines clinical shock

Answer 815

Pale and cold extremities | CRT >4

Question 816

How do we treat symptomatic hypoglycaemia in neonates

Answer 816

Admit to neonatal unit and IV 10% dextrose Asymptomatic: Encourage normal feeding and monitor glucose

Question 817

When should the PCV vaccine be given

Answer 817

3 Months and 12-13 months

Question 818

Rash in Roseola infantum vs Chicken pox

Answer 818

Similar pattern of rash, however: Roseola: Pale pink, macular rash that DISAPPEARS after 48 hours Chicken Pox: Itchy maculopapular rash that CRUSTS over.

Question 819

When does the Moro Reflex appear and what is it

Answer 819

3-4 months: Head Extension causes abduction followed by adduction of the arms

Question 820

When does the grasp reflex come in and what is it

Answer 820

Flexion of fingers when an object is placed into palm 4-5 months

Question 821

What is rooting and when does it present

Answer 821

Assists in breast feeding 4 months of age

Question 822

What is stepping and when does it present

Answer 822

Walking reflex 2 months of age Remember: Some - Stepping (2m) Meaningful - Moro (4 months) Reflexes (Rooting - 4m) Go (grasp 4-5 m)

Question 823

When are children toilet trained

Answer 823

3 years of age

Question 824

What would show up on an EEG for infantile spasms

Answer 824

Dramatic hypsarrythmia

Question 825

What is an infantile spasm

Answer 825

A type of epilepsy involving repeated flexion of the arm/head/trunks followed by extension of the arms.

Question 826

Onset of infantile spasms

Answer 826

4-8 months life

Question 827

RF for infantile spasms

Answer 827

Male

Question 828

First line therapy of infantile spasms

Answer 828

Vigbatrin and ACTH

Question 829

What heart problem is seen in Fragile X Syndrome

Answer 829

Mitral Valve Prolapse

Question 830

What is the appearance of a dermoid cyst

Answer 830

Multiloculated and heterogenous

Question 831

Where are dermoid cysts usually found

Answer 831

Just above the hyoid bone

Question 832

What is a cystic hygroma and where are they usually found

Answer 832

Formed from malformation of the lymphatic system, found in the posterior triangle (compared to a branchial cyst which is found at the anterior triangle)

Question 833

What are red sign flags for unwell children

Answer 833

1. Moderate or severe chest wall recession 2. Does not wake if roused 3. Reduced skin turgor 4. Mottled appearance 5. Grunting

Question 834

Signs of hypernatraemia

Answer 834

``` Jittery movements Hypertonia Hyperreflexia Convulsions Drowsiness ```

Question 835

What conditions are screened for in neonatal blood spot screening

Answer 835

1. Congenital Hypothyroidism 2. CF 3. SCD 4. Phenyketonuria 5. Medium chain acyl-CoA dehydrogenase deficiency (MCADD) 6. Maple syrup urine disease 7. Isolvaleric acidaemia 8. Glutaric acuduria type I 9. Homocystinuria

Question 836

What is precocious puberty defined as

Answer 836

Under 9 - Male | Under 8 - Female

Question 837

What Genetic condition is associated with Hirschsprung's Disease

Answer 837

Down's Syndrome

Question 838

Duodenal atresia vs Hirschsprung's disease in Down's Syndrome

Answer 838

Duodenal atresia does not present with meconium ileus and presents in first feed Hirschsprung's disease presents with meconium ileus and presents later.

Question 839

Transient Tachypnoea of the newborn vs Neonatal Respiratory Distress Syndrome

Answer 839

NRDS is usually caused by prematurity transient Tachypnoea of the newborn is usually caused by C-Sections

Question 840

In what children should we expect a UTI in

Answer 840

Unexplained fever over 38 degrees | An alternative site of infection but who remain unwell

Question 841

What is the recommended method to sample urine in children

Answer 841

Clean Catch

Question 842

What should be done before a suprepubic catheterisation is attempted

Answer 842

USS of the abdomen to demonstrate the presence of urine in the bladder

Question 843

In what lab results is a suspicion of a UTI excluded

Answer 843

ONLY IF BOTH PYURIA (leucocyte Esterase) and Bacteriuria (Nitrites) are negative. Even if one is positive, you must suspect a UTI

Question 844

In what gender is proteus infection more common

Answer 844

Boys

Question 845

How long should a child be observed following antibiotic treatment for a UTI

Answer 845

24-48 hours

Question 846

Name three signs of an atypical UTI

Answer 846

Poor Urine Flo w Abdominal Mass Raised Creatinine

Question 847

Three indications for a micturating Cystourethrogram

Answer 847

Vesicourethral Reflux Bladder Posterior Urethra

Question 848

Investigation for atypical UTIs

Answer 848

DMSA radionuclide imaging to check for renal function (of one kidney relative to the whole renal function) and scarring Micturating Cystourethrogram

Question 849

When should children be sent to hospital for UTIs

Answer 849

1. Under 3 months 2. Any child who is systematically unwell 3. Recurrent UTIs

Question 850

When should preotine:Creatinine ratio be measured

Answer 850

First thing in the morning

Question 851

Treatment of Steroid Resistant Nephrotic SYndrome

Answer 851

60mg Prednisolone for 4 weeks (standard dose)

Question 852

What is lateral discoid meniscus

Answer 852

Instead of the narrow crescent shape seen in a normal meniscus, the lateral meniscus is thicker and fuller = diminished blood supply and weaker capsular attachment. Because of this, it is more prone to tears When the knee is stretched, it makes as sound of bouncing bones

Question 853

Diagnostic of a discoid meniscus

Answer 853

MRI

Question 854

What is Sever's Apophysitis

Answer 854

Heal pain from sports -> caused by inflammation of the growth plate

Question 855

What can be seen in Accessory Navicular Bone disorders on an X-Ray

Answer 855

Plantar medial enlargement of the navicular

Question 856

What is Chondromalacia Patellae

Answer 856

The softening of tissue on the underside of the knee cap Feels full and aching when the knee is flexed Called 'RUNNER'S KNEE'

Question 857

First line investigation of bone trauma in children

Answer 857

X-Ray (anteroposterior and lateral views)

Question 858

What bone is often broken in chldren

Answer 858

Tibia (from unwitnessed falls) Local tenderness from the tibia shaft - think fracture

Question 859

(ignore trimethoprim card) - what is the first line treatment for upper UTIs

Answer 859

IV Ceforoxime for 7 days (for oral, switch to trimethoprim)

Question 860

A child has a UTI, and was unresponsive to IV Ceforoxime, what treatment may we change to and why?

Answer 860

IV Meropenem/ Gentamycin E.Coli is resistent to most penicillins and cephalosporins This is because E.Coli produces enzymes that destroys beta-lactam antibiotics (penecillins, cephalosporins and Co-Amoxiclav and Tazacin)

Question 861

First line treatment of Osteomyelitis and Septic Arthritis (>3 months)

Answer 861

IV Cefuroxime Treat for 6 weeks ,switching from IV to oral when tolerated

Question 862

What is Usher's Syndrome

Answer 862

Inability of the inner ear and auditory nerves to transmit sensory sound input to the brain (sensorineural hearing loss)

Question 863

Signs of Ushers Syndrome

Answer 863

Type 1: Profound Loss, congenital and absent vestibular response Type 2: Sloping audiogram, congenital and normal vestibular system Type 3: Progressive variable vestibular system and onset Accompanied by retinitis pigments

Question 864

Signs of Wardenbergs' Syndrome

Answer 864

Eyes are different colours Eyes are downwards slanting, making them look wide apart

Question 865

Appearance of teacher collins syndrome

Answer 865

Deafness Followed by underdevelopment of zygomatic complexes (eyes looks sunken) Conductive hearing loss

Question 866

What is Erythroblastosis Fetalis

Answer 866

Rh negative mother previously sensitised to Rh+ cells, causing antibodies to pass through the placenta and haemolytic of the baby's Rh Positive fetal cells

Question 867

Signs of Erythroblastosis Fetalis

Answer 867

Severe Anaemia Hepatosplenomegaly

Question 868

What ethnicity is most commonly associated with G6PD deficiency

Answer 868

Mediterranean and African

Question 869

What is G6PD Deficiency

Answer 869

Glutathione is an antioxidant that neutralises free radicals produced from metabolising glucose G6PD takes the H+ ions away from NADPH that have been reduced by Glutathione reductase

Question 870

How is G6PD deficiency inherited

Answer 870

Exclusively affects men as it is X-Linked

Question 871

Signs of G6PD Deficiency

Answer 871

jaundice Dark Tea-Coloured Urine Back Pain from renal problems ``` Blood Tests: Raised reticulocytes Raised LDH from intracellular hydrolysis Reduced Haptoglobin Raised Bilirubin ```

Question 872

What is seen on a smear for G6PD deficiency

Answer 872

Bite Cells | Heinz Bodies

Question 873

Define Rumination

Answer 873

Refrequent regurgitation of ingested food (behavioural)

Question 874

Define Possetting

Answer 874

Small Volume vomits during or between feeds in otherwise well children

Question 875

What is Boerhaave Syndrome

Answer 875

Oesophageal rupture secondary to forceful vomiting (transmural)

Question 876

Name a bulking agent used for constipation

Answer 876

Fybogel

Question 877

Name a stool softener

Answer 877

Latculose

Question 878

Define Pa

Question 879

What condition in the mother usually causes transposition of the great arteries

Answer 879

Diabetes Mellitus 1

Question 880

What is the main cause of cerebral palsy

Answer 880

NOT kernicterus (never usually happens in hospitals) Hypoxic Ischaemic Encephalopathy (from post term and breech deliveries)

Question 881

What chromosome causes DiGeorge Syndrome

Answer 881

22

Question 882

Pathophysiology of DiGeorge Syndrome

Answer 882

Faulty TBX1 gene: Stops coding for the third and fourth pharyngeal pouches

Question 883

What do the third and fourth pharyngeal pouches code for

Answer 883

Third: Thymus + Inferior Parathyroid Gland Fourth: Superior Parathyroid Gland

Question 884

Symptoms of DiGeorge

Answer 884

Thymic Hypoplasia: Means that T-cells produced in the bone marrow cannot migrate and mature in the thymus = deficiency in mature T cells. Parathyroid hypoplasia: Means less PTH = hypocalcaemia symptoms Truncus Arteriosus + Tetralogy of Fallot Cleft Palate sometimes

Question 885

What type of fractures in babies mayy be non-accidental

Answer 885

Non-Motile bone fractures (leg bones, rib fractures)

Question 886

What is the main side effect of Salbutamol

Answer 886

Tachycardia

Question 887

First line investigation for suspected volvulus

Answer 887

Upper GI contrast study

Question 888

Histology findings of Ewing's sarcoma

Answer 888

Small blue cells with clear cytoplasms on haematoxylin stains

Question 889

What is the onion, osteolytic lesions in Ewing's sarcoma also described as

Answer 889

lamellate periosteal reactions

Question 890

What causes the non-blanching rash in Meningitis and HUS etc

Answer 890

Consumption of clotting factors

Question 891

What is a buckle fracture

Answer 891

Incomplete fracture of the shaft with bulging of the cortex

Question 892

What fracture is commonly seen in child abuse

Answer 892

Spiral Fracture

Question 893

What test is used to differentiate between femoral or tibial length shortening causes in DDH

Answer 893

Gallezi Test

Question 894

What are the steps to managing status epileptics

Answer 894

FIRST: Buccal Midolazepam or RECTUM Diazepam 10mg SECOND: IV Lorazepam Third: IV Phenytoin

Question 895

Changes in doses given of Adrenaline 1:1000 in children suffering from anaphylaxis

Answer 895

<6: 150mcg 6-12: 300mcg >12: 500mcg

Question 896

First line management of a hydrocele

Answer 896

Re-assurance, usually goes on it's own. Incision after 12 months

Question 897

What is one of the most common causes of febrile convulsions in children

Answer 897

HSV- 6 : ROSEALA INFANTUM

Question 898

How long should a patient suffering from anaphylaxis be kept in hospital

Answer 898

For 6 hours observation, as anaphylaxis can sometimes be bi-phasic.

Question 899

What is milia

Answer 899

Loads of white small bumps on the nose of a child or other parts of the face

Question 900

What painkiller is contraindicated in indomethacin treatment for keeping the ductus patent and why?

Answer 900

Ibuprofen - antagonises the affect of prostaglandins

Question 901

In what children is a clean catch sample contraindicated to collect urine

Answer 901

Babies, young children who won't pee on command - Urine collection pads instead.

Question 902

Difference in the treatment of hand, foot and mouth disease vs scarlet fever

Answer 902

Scarlet fever requires antibiotics Hand foot in mouth only requires supportive management.

Question 903

Testes bilateral enlargement = gonadotrophin release from intracranial lesion unilateral enlargement = gonadal tumour small testes = adrenal cause (tumour or adrenal hyperplasia)

Answer 903

N/a

Question 904

What is McCune Albright syndrome

Answer 904

Hyperpigmentation of the skin, contrast to vitiligo

Question 905

What is a complication of chicken pox

Answer 905

Necrotising Fasciitis

Question 906

Croup vs Bronchiolitis

Answer 906

Bronchiolitis - Barkig cough Croup - Inspiratory Stridor

Question 907

When is Transient Synovitis common

Answer 907

Between 3 and 10 years of age Anything under, always suspect Septic Arthritis

Question 908

First Line Management of a sudden limp <3 years

Answer 908

Urgent Hospital Assessment

Question 909

What is Lumacaftor/Ivacaftor in CF treatment

Answer 909

Increases the number of CFTR proteins that integrate into protein surfaces

Question 910

In what children does X-Ray become the first investigation for DDH

Answer 910

>4.5 months

Question 911

When do children talk in small sentences (3-5 words)

Answer 911

2.5-3 Years

Question 912

When do children start to respond to their own name

Answer 912

9-12 months

Question 913

When do febrile convulsions stop

Answer 913

After the age of 5

Question 914

When is the MMR vaccin egiven

Answer 914

1 Year 3-4 Years

Question 915

If a child misses their MMR vaccine, when should it be given

Answer 915

<10 years: Give MMR vaccine + booster in 3 motnsh >10 Years: Give MMR vaccine + Booster in 1 month

Question 916

How does asthma management vary under 5s and over 5s

Answer 916

Under 5s: SABA + 8 week trial of MODERATE-dose ICS SABA + low dose ICS + LRA Referral Over 5s: SABA SABA + LOW-dose corticosteroid SABA + low dose ICS + LTRA SABA + Low dose ICS + LABA SABA + MART SABA + Moderate-dose MART or switch back to moderate dose ICS

Question 917

First line management of cow milk protein allergies

Answer 917

Extensive Hydrolysed formula

Question 918

When does an intractable foreskin resolve

Answer 918

By 2 years

Question 919

Venous vs Still murmur

Answer 919

Venous: Heard below both clavicles Still: Lower left sternal edge

Question 920

What is the main cause of RDS in neonates

Answer 920

Maternal DM

Question 921

How long does a child have to be excluded from school with hand, foot and mouth disease

Answer 921

None

Question 922

Most common cardiac problem associated with Duchenne's

Answer 922

Dilated Cardiomyopathy

Question 923

When can children return to school with scarlet fever

Answer 923

24 hours after starting antibiotics

Question 924

When should children be reviewed for height restriction by the GP

Answer 924

Under 2nd centile And paediatric review at 0.4 centile

Question 925

When is CPAP or BIPAP indicated

Answer 925

When the problem is solely in keeping airways open like RDS.

Question 926

First line intervention of pityriasis Versicolour

Answer 926

Ketoconazole shampoo/soap

Question 927

First line intervention for Pityriasis rosea

Answer 927

None, self limiting

Question 928

Recovery time in a febrile convulsion

Answer 928

1 hour of drowsiness, any more is alarming

Question 929

When is a lumbar puncture in meningitis contraindicated

Answer 929

Meningococcal septicaemia

Question 930

What is the appearance of a candidiasis nappy rash

Answer 930

Beefy, red well-defined patches

Question 931

What characterises a nappy rash from irritant contact dermatitis

Answer 931

Creases are spared

Question 932

Other types of rashes seen in the groin of a neonate

Answer 932

Psoriasis: papule would be seen elsewhere too Seborrhoaic dermatitis: Flaky with scalp involvement

Question 933

Bacterial tracheitis vs Croup

Answer 933

Bacterial Tracheitis - does not improve on dexamethasone Croup does Both have inspiratory stridor

Question 934

What is the last line medication that would be used to treat cyanosis from CHD

Answer 934

Phenylephrine - would cause vasoconstriction, increasing vascular resistance and reducing right to left shunting

Question 935

Symptoms of ADHD

Answer 935

1. Inattention | 2. Hyperactivity

Question 936

What is the first line management of a viral induced wheeze

Answer 936

Inhaled Salbutamol

Question 937

What is the first line management for Pyloric Stenosis

Answer 937

Electrolyte correction Then Surgery

Question 938

What causes RDS in SCD

Answer 938

Aplastic crisis: Tachypnoea, tachycardia in the absence of pain

Question 939

What are morbilliform eruptions

Answer 939

Happens in patients being treated with antibiotics (amoxicillin) for Infectious Mononucleosis - generalised maculopapular rash

Question 940

Sepsis vs Diabetic Ketoacidosis

Answer 940

Fever vs No Fever Always consider sepsis in diabetic paediatric patients as UTI's are more common in them

Question 941

First line treatment for intussusception

Answer 941

Air enema If peritonism is suspected, wanting definitive treatment: Laporoscopic correction/reduction

Question 942

What is the first management step when detecting a murmur in a neonate

Answer 942

Wait 24 hours, if after 24 hours of life they still have the murmur, refer for echo

Question 943

Characteristic signs of GORD

Answer 943

1. Back arching 2. Drawing knees to chest 3. Initially feeding well and then goes to shit

Question 944

Varicocele vs Hydrocele

Answer 944

Varicocele: Pampiniform plexus worm appearance Hydrocele: Transillumination from build up of fluid in the tunica vaginalis

Question 945

At what age do children eat with a spoon and bowl

Answer 945

18 months

Question 946

At what age do children wave goodbye

Answer 946

10 months

Question 947

At what age are children pot

Question 948

Signs of Pierre Robin sequence

Answer 948

Cleft Palate Retracted Tongue Small lower jaw Can only be fed prone

Question 949

What Cardiac disease is common in children who had GDM mothers

Answer 949

Transposition of the great arteries

Question 950

Diagnostic of Duchenne's muscular atrophy

Answer 950

Muscle biopsy

Question 951

What are benign rolandic seizures

Answer 951

children have a tonic seizure overnight Usually found sleeping on the floor or messy sheets

Question 952

EEG finding in benign rolandic seizures

Answer 952

centro-temporal spikes

Question 953

What is the prognosis of rolandic seizures

Answer 953

Most children grow out of it

Question 954

Why is Oxygen contraindicated in neonatal resuscitation

Answer 954

oxygen forms free radicals which can cause increased mortality Just give breaths on air

Question 955

Most common cause of neonatal sepsis

Answer 955

E.coli and Strep B

Question 956

What antibiotics are given in neonatal sepsis

Answer 956

IV Gentamicin and Benzylpenecillin

Question 957

What is an absolute indication for US of the pelvis

Answer 957

ALL babies that were born breach must be given a USS of the pelvis at 6 weeks age To check for DDH

Question 958

At what age is a pavlik harness sindicated

Answer 958

6 weeks + Anything under is just watchful waiting

Question 959

What should be done to children under 6 with Perthes disease

Answer 959

Just observe, keep femoral head within the acetabulum through casts and braces After that, NSAIDs and physio + Surgery

Question 960

When are sub-optimal o2 levels allowed, only observation is indicated?

Answer 960

In the first 10 minutes of life

Question 961

What is a key risk factor for hypoglycaemia

Answer 961

Prematurity (will look jittery and irritable)

Question 962

GOLD STANDARD dignosis of pertussis

Answer 962

Per Nasal Swab and PCR

Question 963

What vaccines are offered to pregnant women 16-32 weeks?

Answer 963

Whooping Cough and Influenza

Question 964

Subdural haemorrhage vs intraventircular haemorrhage

Answer 964

IVH - Premature infants Subdural Haemorrhages - Shaken Baby Syndrome

Question 965

Most common fracture in physical abuse

Answer 965

Mid shaft humeral fracture

Question 966

Treatment of strawberry Naevus

Answer 966

Topical propranolol but otherwise nothing

Question 967

Name three benign skin problems seen in neonates

Answer 967

Erythema neonaturum (white pinpoint papule) Stork bite/salmon patch (blanches on pressure) Congenital Dermal Melanocytosis (benign blue spots on the back)

Question 968

What causes ABO incompatibility

Answer 968

When the mother is O and the baby is Type A or B Mother has anti A and B antibodies so causes destruction to baby's blood

Question 969

Treatment of meconium ileum in CF

Answer 969

Therapeutic contrast enema (gastrhogafin)

Question 970

Surgical management of an undescended testes and at what age is this indicated

Answer 970

Orchidopexy at 1 year

Question 971

When are inguinal hernias more prominent

Answer 971

On crying

Question 972

After what day should medication be given for acute otitis media

Answer 972

After 4 days of symptoms

Question 973

When does crying in infantile colics happen the most

Answer 973

At night/evening

Question 974

Main viral cause of pneumonia

Answer 974

RSV

Question 975

How long should children be off for measles

Answer 975

4 Days from onset of rash

Question 976

How long should a child be off from school with Rubella

Answer 976

5 Days from onset of rash

Question 977

how long should a child be off from school with Mumps

Answer 977

5 Days from onset of swollen glands

Question 978

Conditions that require no exclusion

Answer 978

``` Conjunctivitis Fifth Disease Roseola Mononucleosis Head Lice Threadworm Hand, foot and mouth ```

Question 979

What does honey injection cause in neonates

Answer 979

Infant Botulism

Question 980

What is chorioretinitis

Answer 980

Posterior Uveitis

Question 981

First line investigation for asthma

Answer 981

Spirometry and Bronchodilator reversibility Second Line: FeNO + Paek flow diary

Question 982

At what age do children say bye bye

Answer 982

12 months

Question 983

What drugs can cause G6PD deficiency

Answer 983

Ciprofloxacin Antimalarials: Primaquins Sulfonylureas Sulfonamides

Question 984

Describe the tone of the muscles that characterises Prader-Willi Syndrome

Answer 984

Hypotonia

Question 985

Rocker bottom feet in Edward's vs Patau

Answer 985

Rocker bottom feet in Edward's is usually accompanied by micrognathia

Question 986

Prader willi vs Angelman

Answer 986

No hypogonadism or Diabetes/Obesity Just an ataxic gait with inappropriate laughter (CHARACTERISTIC of Angelman) + Seizures Both have LDs

Question 987

What hearing test is done at 6-9 months

Answer 987

Distraction Test

Question 988

What is the second line treatment for whooping cough if Macrolides are not tolerated

Answer 988

Co-Trimoxazole

Question 989

What do the haematoma’s look like in children

Answer 989

Bilateral rather than unilateral

Question 990

Define Failure to ThriveWhat BMI indicates possible undernutrition

Answer 990

Birthweight < 0.4th Centile

Question 991

How often should children with failure to thrive be monitored

Answer 991

Daily < 1 month Weekly (1-6 months) Biweekly (6-12m) Monthly (1 year)