Paediatrics 1 (Cardio, GI, Neuro, Psych, Onco, MSK, Neonatal)

Question 1

Investigation: Fever in baby

Answer 1

• blood culture
• urine dip is not sensitive enough in under 3 months old
• send for urine microscopy
• WBC
• FBC
• U+Es

Question 2

What condition should be checked for in febrile babies?

Answer 2

Meningitis
- weak blood brain barrier
- sample CSF

Question 3

Management: febrile baby

Answer 3

Broad spectrum antibiotics
- cefotaxine or ceftriaxone and amoxicillin

(until culture come back)

Admit babies under 3 months with a fever > may need IV antibiotics

Question 4

What is the most common cause of UTI?

Answer 4

E.coli

Question 5

What antibiotic is used to treat E.coli UTIs in children?

Answer 5

IV cefuroxime
(once meningitis ruled out - doesn’t reach the blood brain barrier)

Question 6

What investigation should be completed with a baby under 6 months old with a UTI?

Answer 6

Renal tract USS
- recurrent infections can cause scarring that can lead to long term renal problems

(or recurrent UTIs in older children)

Question 7

What needs to be ruled out in a fitting child with a fever?

Answer 7

CNS infection
- might need an LP

Question 8

What can be a cause of ineffective antibiotic treatment?

Answer 8

Some bacteria has Extended Spectrum Beta-lactamase producer

• resistant to all penicillins and cephalosporins
• change to meropenem

Question 9

Presentation: meningitis

Answer 9

• Temperature
• Semi-comatose
• Purple rash on skin

Question 10

Management: meningitis

Answer 10

ASAP
(before any investigations)

Bacterial meningitis or meningococcal sepsis:

1. First line intravenous cefotaxime

Question 11

Investigations: meningitis

Answer 11

• blood cultures
• EDTA (acid) blood for PCR
• Lumbar Puncture CSF = might delay if concerns about clotting and raised intracranial pressure

notify Public health

Question 12

What bacteria causes meningitis?

Answer 12

Neisseria meningitidis
- gram negative diplococci

group B streptococcus

Question 13

What are contraindications to a lumbar puncture?

Answer 13

• Findings of increased intracranial pressure
• bleeding diathesis
• cardiopulmonary instability
• soft tissue infection at the puncture site
• shock
• respiratory insufficiency
• suspected meningococcal

Question 14

What is a complication of meningitis that causes petechial haemorrhages to develop on the trunk?

Answer 14

Waterhouse - Friderichsen syndrome

• petechial haemorrhages
• rapidly stops oxygen saturations
• severely hypotensive

Question 15

What conditions should be noticed to public health?

Answer 15

• all meningitis
• all invasive meningococcal
• all encephalitis

Question 16

Presentation: pneumonia

Answer 16

• short of breath
• pyrexial
• miserable

Question 17

Investigations: pneumonia

Answer 17

• sputum culture or nose/ throat swab
• CXR = RLL change
• blood culture

Question 18

What is the main cause of pneumonia?

Answer 18

Streptococcus penumoniae
- Gram positive cocci in pairs

Question 19

Management: pneumonia

Answer 19

IV benzylpenicillin
PO amoxicillin

• if not improving check if child has developed an empyema in the pleural space = CXR

Question 20

Define: prematurity

Answer 20

• under 37 weeks

Question 21

At what gestation is resuscitation of a baby allowed?

Answer 21

from 22 weeks

Question 22

management: premature rupture of membranes

Answer 22

• antibiotics e.g. Erythromycin for 10 days
• steroids
• IV MgSO4 = neuroprotection

Question 23

What is a main problem in premature babies?

Answer 23

lungs are not fully formed

Question 24

When is surfactant and alveoli produced in a foetus?

Answer 24

• surfactant = retained in type 2 pneumocystis (allow lungs to stretch and reduce surface tension)
• alveoli = absent at 24 weeks then exponential increase towards term
  (from type 1 pneumocytes)

Question 25

How does steroids help with lung development in a foetus?

Answer 25

Steroids trigger type 2 pneumocytes to release surfactant

Question 26

Presentation: chronic lung disease of prematurity

Answer 26

- Reduced lung volume - Reduced alveolar surface area - Diffusion defect

Question 27

Presentation: apnoea of prematurity

Answer 27

- brain stem not fully myelinated fully until 32 to 34 weeks - not signal from brain stem to breathe

Question 28

Management: apnoea of prematurity

Answer 28

Physical: - NCPAP - Stimulation Drugs - phosphodiesterase inhibitors e.g. sildenafil (helps with pulmonary hypotension) - caffeine

Question 29

what is the cause of ventricular haemorrhage in premature babies?

Answer 29

- sometimes spontaneously bleeds - worry about blood loss

Question 30

Define: Cystic periventricular leukomalacia

Answer 30

- brain injury most common in premature babies - the white matter (Leuko) surrounding the ventricles of the brain is deprived of blood and oxygen leading to softening (malaria)

Question 31

What cause periventricular leukomalacia?

Answer 31

If the blood supply to an area of the brain is stopped or reduces, this causes tissue damage - uterine infections - early RoM - premature babies - ventricular haemorrhage

Question 32

What are the benefits of breastfeeding for the infant?

Answer 32

Less Infection: Diarrhoea, Otitis media, Respiratory Syncytical Virus, Respiratory Infections, Enhanced Vaccine Response Less immune driven/allergic disease: Wheezing, Childhood cancer, Eczema, Hodgkin's disease, Multiple sclerosis, Crohn's disease, Diabetes mellitus, Enhanced immunologic development - Reduces risk of NEC - Reduced Reduced SIDS - Reduced Gastroesophageal Reflux - Lower risk of Childhood Inguinal Hernia - Higher IQ - Better Cognitive Development

Question 33

What are the maternal benefits of breastfeeding?

Answer 33

Reduces cancer risk for: Breast, Uterine, Ovarian, Endometrial Improved health with less: Post partum haemorrhage, postnatal depression, Decrease insulin requirements in diabetics, Osteoporosis later in life, Less child abuse Promotes postpartum weight loss Optimum child spacing Less food expense Less medical expense More ecological Delays fertility

Question 34

What are the causes of unconjugated and conjugated jaundice?

Answer 34

- haemolysis, - prematurity, - sepsis, - dehydration, - hypothyroid, - metabolic disease Conjugated: prolonged parenteral nutrition, NEC, sepsis, metabolic, anatomical problems

Question 35

How to treat neonatal jaundice?

Answer 35

Unconjugated = high levels cause kernicterus (so need to treat) High levels treated by phototherapy (blue light, 450nm) or exchange transfusions jaundice lasting more than 3 weeks needs investigation

Question 36

Define: Necrotising enterocolitis

Answer 36

acute inflammatory disease leading to bowel necrosis in premature neonates - intestinal disease that affects premature or very low weight birth infants - causes intestinal tissue to die

Question 37

What is the cause of necrotising enterocolitis?

Answer 37

- Very low birth weight or very premature - Formula feeds (it is less common in babies fed by breast milk feeds) - Respiratory distress and assisted ventilation - Sepsis - Patient ductus arteriosus and other congenital heart disease

Question 38

Presentation: necrotising enterocolitis

Answer 38

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

Question 39

Investigations: necrotising enterocolitis

Answer 39

blood tests - Full blood count = thrombocytopenia and neutropenia - CRP = inflammation - Capillary blood gas = metabolic acidosis - Blood culture = sepsis Abdo x-ray - dilated loops of bowel - bowel wall oedema - pneumatosis intestinalis = gas in bowel wall - pneumoperitoneum = free gas in peritoneal cavity + perforation

Question 40

Management: necrotising enterocolitis

Answer 40

- nil by mouth - IV fluids, total parenteral nutrition and antibiotics - surgical emergency

Question 41

What are the complications of necrotising enterocolitis?

Answer 41

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

Question 42

how does prematurity affect immunity?

Answer 42

Last 3 months of gestation active IgG transfer - the more premature you are, the less of this you get - cell mediated immunity is less active

Question 43

Define: retinopathy of prematurity

Answer 43

- too much oxygen given = hyperopic insult - arrest of normal vascular growth - fibrous ridge forms - vascular proliferation cause: - retinal haemorrhages - retinal detachment - blindness

Question 44

Management: retinopathy

Answer 44

Laser therapy - to reduce growth of abnormal blood vessels

Question 45

What are the main symptoms of ADHD?

Answer 45

1) inattention, 2) hyperactivity 3) impulsivity.

Question 46

What are the types of ADHD?

Answer 46

three categories of ADHD: - predominantly inattentive type; - predominantly hyperactive-impulsive type; and - combined type.

Question 47

What is the diagnosis of ADHD?

Answer 47

<17 years 6/9 Inattentive 6/9 Hyperactive/ Impulsive symptoms Present before 12 years Developmentally inappropriate Several symptoms in 2 or more settings Clear evidence symptoms interfere/reduce quality of social/academic/occupational function

Question 48

Presentation: ADHD

Answer 48

Very short attention span Quickly moving from one activity to another Quickly losing interest in a task and not being able to persist with challenging tasks Constantly moving or fidgeting Impulsive behaviour Disruptive or rule breaking

Question 49

Management: ADHD

Answer 49

1. detailed assessment by specialist 2. establish healthy diet and exercise 3. Medication (central nervous system stimulants) - Methylphenidate ‘Ritalin’ (CNS stimulant) - Dexamfetamine (stimulant) - Atomoxetine (SNRI, increase norepinephrine)

Question 50

What are some medications for ADHD?

Answer 50

-Methylphenidate ‘Ritalin’ Dexamfetamine Atomoxetine

Question 51

What are some side effects of ADHD medication?

Answer 51

- headache, insomnia, loss of appetite, stomach ache, dry mouth, nausea - Can stunt growth - Need to Monitor weight, height and BP - Methyphenidate is Not recommended to take during pregnancy

Question 52

What groups are at higher prevalence of ADHD?

Answer 52

Preterm LAC ODD/CD Mood Disorders (Anxiety/ Depression) Close family members with ADHD Epilepsy Neurodevelopmental Conditions Mental Health Substance Use Disorder Youth Justice/ Adult Criminal Justice System Acquired Brain Injury

Question 53

How do ADHD symptoms present differently in girls to boys?

Answer 53

harder to recognise in girls - daydreaming - more chatty - better at masking it in public/school

Question 54

Define: Autism

Answer 54

- genetically based neurological variant of cognition, communication, motivation and interests

Question 55

How to diagnose autism?

Answer 55

To reach the threshold of a diagnosis, patients must meet all criteria A and at least 2 of B in the presence of C,D and E. A) Persistent deficits in social communication and social interaction B) Restricted, repetitive patterns of behaviour, interests, or activities C) Symptoms must be present in the early developmental period D) Symptoms cause clinically significant impairment in social, occupational, or other important areas of current functioning. E) These disturbances are not better explained by intellectual disability (intellectual developmental disorder) or global developmental delay.

Question 56

What does category A for autism diagnosis include?

Answer 56

- social-emotional reciprocity = info dumping, direct answers to questions, abstract language - non-verbal communicative behaviours for social interaction - developing, maintaining and understand relationships

Question 57

What is included in category B for autism?

Answer 57

Need 2 out of 4 1. stereotyped or repetitive motor movements, use of objects or speech 2. highly restricted, fixated interests that are abnormal in intensity or focus 3. preference for sameness, and strong adherence to routines, or ritualised patterns of verbal or non-verbal behaviour 4. hyper or hypo-reactivity to sensory input

Question 58

What are included in category C for a autism diagnosis?

Answer 58

not acquired, (e.g.not TBI or anxiety/mood disorder etc). Can be hard if childhood trauma, attachment. Acknowledgement that might not present until demand exceeds capacity

Question 59

What are included in category D for a autism diagnosis?

Answer 59

significant impairment (adults are usually diagnosed at a period of breaking point)

Question 60

What are included in category E for a autism diagnosis?

Answer 60

not better explained by something else

Question 61

What are factors for an increased risk of autism?

Answer 61

A sibling with autism. Birth defects associated with central nervous system malformation and/or dysfunction, including cerebral palsy. Gestational age less than 35 weeks. Parental schizophrenia-like psychosis or affective disorder. Maternal use of sodium valproate in pregnancy. A learning (intellectual) disability. Attention deficit hyperactivity disorder. Neonatal encephalopathy or epileptic encephalopathy, including infantile spasms. Chromosomal disorders such as Down's syndrome. Genetic disorders such as fragile X. Muscular dystrophy. Neurofibromatosis. Tuberous sclerosis.

Question 62

Define: ALL

Answer 62

Acute lymphoblastic leukaemia (ALL) - affects one of the lymphocyte precursor cells - acute proliferation of a single type of lymphocyte, usually B-lymphocytes. - Excessive accumulation of these cells replaces the other cell types in the bone marrow, leading to pancytopenia.

Question 63

Presentation: paediatric malignant disease

Answer 63

Localised mass - Lymphadenopathy - Organomegaly - Soft tissue or bony mass Problems from disseminated disease - Bone marrow infiltration Problems from localised mass - Airway obstruction from lymphadenopathy

Question 64

What symptoms are present in bone marrow malignant disease?

Answer 64

fever = reduced WBCs fatigue = anaemia (RBCs) easy bruising = reduced platelets Bone pain

Question 65

Presentation: Acute lymphoblastic leukaemia

Answer 65

Fever Fatigue Frequent infections Lymphadenopathy Hepatomegaly and/or splenomegaly Anaemia Bruising,petechiae Bone or joint pain

Question 66

What are the specific features of ALL?

Answer 66

- MC in children - associated with Down's syndrome

Question 67

Investigations: ALL

Answer 67

Blood film Serum chemistry CXR Bone marrow aspirate Lumbar puncture

Question 68

Management: ALL

Answer 68

1. Chemotherapy – 5 phases - Induction, Consolidation, Interim maintenance - Delayed intensification, Maintenance 2. Haemopoietic stem cell transplantation - High risk patients in first remission - Relapsed patients

Question 69

Presentation: CNS tumours

Answer 69

Headache = often worse lying down Vomiting = especially early morning Papilloedema Squint Nystagmus Ataxia (for kids they lose skills e.g. revert back to crawling) Personality or behaviour change

Question 70

When to scan headaches?

Answer 70

If also papilloedema, decreased acuity, visual loss If also other neurological signs (or they develop) If recurrent and/or early morning If associated with vomiting if persistent, more frequent, preceded by headache If also have short stature / decelerated linear growth If have symptoms of diabetes insipidus If age < 3 years If child has neurofibromatosis (NF1)

Question 71

Management: CNS tumours

Answer 71

1. Surgery Resection (can only do in certain parts of the brain) VP shunt 2. Chemotherapy Single agent Combination treatment 3. Radiotherapy For malignant tumours in older children Whole brain not used in very young

Question 72

What are the causes of lymphadenopathy?

Answer 72

Mostly due to self limiting benign cause Other causes: HIV infection Auto immune conditions Storage disorders Malignancy

Question 73

When to biopsy in lymphadenopathy?

Answer 73

- Enlarging node without clear infective cause - Persistently enlarged node - Unusual site e.g. supraclavicular - If have associated symptoms and signs - Fever, weight loss, enlarged liver/spleen - If CXR abnormal

Question 74

Management: paediatric lymphoma therapy

Answer 74

1. Chemotherapy Determined by histology and stage 2. Radiotherapy Hodgkin's – to residual bulk disease NHL - rarely 3. Surgery – mainly limited to biopsy 4. High dose therapy mainly for relapse

Question 75

Presentation: abdominal mass

Answer 75

mass Pain, haematuria, constipation, hypertension, weight loss

Question 76

Investigation: abdominal mass

Answer 76

- CT scans - biopsy

Question 77

What are the differential diagnosis for abdominal mass?

Answer 77

Hepatoblastoma Wilms tumour Neuroblastoma Lymphoma/leukaemia Sarcoma Constipation Enlarged kidneys – polycystic disease

Question 78

Presentation: neuroblastoma

Answer 78

proptosis = can appear like orbital cellulitis

Question 79

Management: neuroblastoma

Answer 79

1. Surgery Primary - if resectable Following chemotherapy 2. Chemotherapy Type determined by stage and biology High dose with HPSC - high risk groups 3. Radiotherapy Mainly for high risk group or at relapse

Question 80

Management: Wilms tumour

Answer 80

1. Chemotherapy Prior to surgery Following surgery 2. Surgery Nephrectomy Partial nephrectomy if bilateral 3. Radiotherapy If residual abdominal or pulmonary disease

Question 81

What are the causes of retinoblastoma?

Answer 81

- RB1 gene on chromosome 13 - familial 40% vs sporadic

Question 82

presentation: retinoblastoma

Answer 82

leukocoria – loss of red reflex (also called cat’s eye) Strabismus pain or redness around the eye poor vision or change in child's vision

Question 83

Management: retinoblastomablastoma

Answer 83

Treatment may include one or more of the following: chemotherapy radiation therapy laser therapy phototherapy thermal therapy cryotherapy surgery - enucleation of eye

Question 84

Limping child: history + examination

Answer 84

- trauma - duration, onset - swelling, bruising, erythema - listen to parents - soles of feet

Question 85

What are the types of limp?

Answer 85

- antalgic - trendelenberg = waddling - vaulting or circumduction = leg length discrepancy

Question 86

Investigations: limping child

Answer 86

Bloods: FBC, ESR, CRP, blood culture X-rays: remember the joint above and below (BONE) USS: Good for infection (SOFT TISSUE) (MRI)

Question 87

What are the features of transient synovitis/irritable hip?

Answer 87

- commonest cause of hip pain in kids - commonly 4-8yrs - can affect any major joint - non-specific inflammation= thought to be related to viral infection - improve 24-48 hr and resolve 1-2 weeks

Question 88

Presentation: transient synovitis

Answer 88

occurs within a few weeks of a viral illness - limp - refusal to weight bear - groin or hip pain - mild low grade temperature they should be otherwise well

Question 89

Management: transient synovitis

Answer 89

- analgesia - exclude septic arthritis

Question 90

Define: Septic arthritis

Answer 90

- intra-articular infection (inside a joint) - <2yrs - 35% hip 35% knee - acute pain - systemic symptoms - flexion abduction and external rotation - fever, look unwell

Question 91

Presentation: septic arthritis

Answer 91

only affects a single joint Hot, red, swollen and painful joint Refusing to weight bear Stiffness and reduced range of motion Systemic symptoms such as fever, lethargy and sepsis

Question 92

What are the main causes of septic arthritis?

Answer 92

mechanism: direct, haematogenous, extension from adjacent bone (osteomyelitis) Staph aureus MC Neisseria gonorrhoea (gonococcus) in sexually active teenagers Group A streptococcus (Streptococcus pyogenes) Haemophilus influenza Escherichia coli (E. coli)

Question 93

Management: Septic arthritis

Answer 93

- require admission to hospital - joint aspiration - send sample for gram staining, crystal microscopy, culture and antibiotic sensitivities - empirical IV antibiotics until sensitivity known for 3 to 6 weeks - may require surgical drainage and washout of joint

Question 94

What is the Kocher criteria?

Answer 94

used for septic arthritis NEWT non- weight bearing ESR>40 WCC>12 Temp>38.5 4/4 = 99% of septic arthritis

Question 95

What are the features of Osteomyelitis?

Answer 95

- infection of the bone and bone marrow - mean age 6.5yrs - more common <10yrs due to the blood supply to the metaphysis - association with (minor) trauma - NB. often have frequent presentation before diagnosis - less likely to have systemic upset

Question 96

What are the risk factors for osteomyelitis?

Answer 96

Open bone fracture Orthopaedic surgery Immunocompromised Sickle cell anaemia HIV Tuberculosis boys <10 yrs

Question 97

Presentation: osteomyelitis

Answer 97

can be acutely unwell or more subtle Refusing to use the limb or weight bear Pain Swelling Tenderness They may be afebrile, or may have a low grade fever.

Question 98

Investigations: osteomyelitis

Answer 98

- x-rays = can be normal, blurry outline - MRI - better - blood tests = raised CRP and ESR and WBCs - blood culture - bone marrow aspiration or bone biopsy

Question 99

Management: osteomyelitis

Answer 99

prolonged antibiotics - may require surgery fro drainage and debridement of the infected bone

Question 100

What are the red flags for non-accidental injury?

Answer 100

Any bruising in an infant <4-6m Injury location (ears, neck, frenulum, cheek) Any fracture in a non-ambulant child Unexplained delays in presentation Multiple presentations

Question 101

what is developmental dysplasia of the hip (DDH)?

Answer 101

- Abnormal growth/development of the hip joint - When the ball does not sit and load the socket, the ball and socket do not grow/mould correctly

Question 102

What are the risk factors for DDH?

Answer 102

Breech presentation Female Family history First baby Packagaing disorders

Question 103

Management: DDH

Answer 103

- Can resolve over time without intervention - Pavlik harness can be used in <6 months old] - Older children may need surgery

Question 104

What are the main clinical findings in DDH?

Answer 104

Red flags on examination 1. Leg length difference (Galeazzi test) 2. Trendelenberg gait

Question 105

Investigation: DDH

Answer 105

Usually picked up on NIPE Exam with Barlow (dislocate) and Ortolani (relocate) tests B before O, dislocate then relocate - Ultrasound for <4.5 months - X-ray for >4.5 months (all breech babies from 36 weeks need scan even if fixed to cephalic with ECV)

Question 106

What is Legg- Calve-Perthes?

Answer 106

- idiopathic avascular necrosis of the proximal femur in children - 4-8 yrs - male:female 5:1 - usually asymmetrical

Question 107

What is slipped upper femoral epiphysis (SUFE)?

Answer 107

- head of the femur is displaced along the growth plate

Question 108

Define: Slipped upper femoral epiphysis

Answer 108

Rare hip condition where the femoral head epiphysis is displaced posteroinferiorly Much more common in males between the age of 8-15 More common in obese children

Question 109

Management: Perthes disease

Answer 109

- Initial management is conservative by maintaining position and alignment in the joint with bed rest, analgesia and casts - If <6 years of age, observation is usually sufficient - Older children may need surgery and physio

Question 110

Investigations: Perthes

Answer 110

Plain X-ray: Widening of the joint space initially then decreased femoral head size Technetium bone scar/MRI if normal X-ray and symptoms persisting

Question 111

Presentation: SUFE

Answer 111

- hip, groin, thigh or knee pain - externally rotated gait (more comfortable for patient) - restricted range of hip movement - painful limp - restricted movement in the hip

Question 112

What are the aetiology of SUFE?

Answer 112

Male obese 12-13 yrs due to rapid periods of growth

Question 113

Investigations: SUFE

Answer 113

X-ray first Blood tests are normal, particularly inflammatory markers used to exclude other causes of joint pain Technetium bone scan CT scan MRI scan

Question 114

management: SUFE

Answer 114

Surgery is required to return the femoral head to the correct position and fix it in place to prevent it slipping further.

Question 115

What are the red flags for bone tumours?

Answer 115

Night pain Pain that doesn't go after treatment/as expected Lump in the soft tissues or bone (Weight loss/other systemic symptoms)

Question 116

What are the features of bone tumours on x-ray?

Answer 116

- bone destruction/hole in th ebone - soft tissue swelling - periosteal reaction - new bone formation

Question 117

What spinal conditions cause a limp?

Answer 117

Spinal tumours Spinal cord problems Nerve root compression

Question 118

What changes occur to foetal circulation when baby is delivered?

Answer 118

Pulmonary resistance falls allowing more blood into the lungs - foramen ovale closes - ductus ateriosis closes - aortic pressure rise due to clamping of umbilical arteries

Question 119

What are the 3 categories of congenital heart disease?

Answer 119

1. holes/connections 2. narrowings 3. complex (mixed) other categories - duct dependent/non-duct dependent - cyanotic/acyanotic

Question 120

What are the features of ventricular septal defect?

Answer 120

- most common congenital defect - L to R shunt causes increased blood flow to lungs, symptoms when PVR falls - Pan systolic murmur, LLSE - tachypnoea, poor feeding, failure to thrive

Question 121

What are the features of Atrial septal defect?

Answer 121

- 2nd most common - L to R shunt (acyanotic) - atria are low pressure chambers, so only get symptoms later in life in early adulthood - fixed splitting S2, pulmonary flow murmur (Not ASD murmur as not high enough pressure to hear)

Question 122

What are the features of Atrioventricular septal defect?

Answer 122

- common defect in Trisomy 21 (Down syndrome) - can lead to pulmonary vascular disease - will not close spontaneously = needs surgery - poor feeding, failure to thrive, tachypnoea - active precordium, thrill, gallop, rhythm - hepatomegaly, oedema - murmur rises from valvular regurgitation rather than septal defects

Question 123

What are the features of Patent ductus arteriosus?

Answer 123

- usually preterm babies (prostaglandins E higher in preterm infants) - poor feeding, failure to thrive, tachypnoea - active precordium, thrill, gallop, rhythm - classical continuous machinery murmur in pulmonary area 'bounding pulse' (in diastolic and systolic phase) - hepatomegaly, oedema

Question 124

Management: ASDs

Answer 124

- only surgery on large defects or if failing to thrive

Question 125

Management: VSD and PDS

Answer 125

If failing to thrive - increase calories, nasogastric feeds - diuretics - surgical closure

Question 126

What are the types of congenital heart defects with stenosis/narrowing?

Answer 126

- coarctation of aorta - aortic stenosis - pulmonary stenosis

Question 127

Aortic Stenosis

Answer 127

- critical AS > acute presentation with shock collapse in newborns - reduced stroke volume (reduced blood flow to the body) - symptoms: reduced exercise tolerance, poor feeding, syncope - signs: femoral, thrill, ejection, systolic murmur loudest in aortic area, radiating to carotids

Question 128

Pulmonary stenosis

Answer 128

- ejection systolic murmur in the left upper sternal edge - murmur often radiates to the back especially if the pulmonary branches are stenosed - right ventricular heave

Question 129

Coarctation of aorta

Answer 129

- critical anomaly, need surgery - impaired perfusion to the body - present = after a week of age (as PDA still open helping with blood flow but once closed baby presents with collapse) - pre and post ductal difference in saturations (only if PDA open) - 4 limb BP, discrepancy between upper and lower limb BP - older children murmur over back (after collaterals develop)

Question 130

Management: coarctation of aorta

Answer 130

in critical cases - prostaglandin E = used to keep ductus arteriosus open while waiting for surgery - surgery = correct coarctation and to ligate the ductus arteriosus

Question 131

Management: obstructive lesions

Answer 131

- keep PDA open with prostaglandins - surgical = balloon valvoplasty or surgical repair

Question 132

What are the 2 Cyanotic heart conditions?

Answer 132

- transposition of great arteries - tetralogy of fallot's

Question 133

Transposition of great arteries

Answer 133

- aorta and pulmonary artery have swapped - this circulation is not compatible with life (foramen open in foetus so can survive in the uterus) - cyanosis - acidosis - collapse/death - emergency cardiac surgery = septosotmy, start prostaglandins

Question 134

What are the 4 features of tetralogy of fallot?

Answer 134

1. ventricular septal defect 2. Pulmonary stenosis 3. overriding of aorta 4. right ventricle has thickened muscle RVH

Question 135

Presentation: tetralogy of fallot

Answer 135

depends on severity of RVOT narrowing - may be cyanotic or pink - murmur - normal pulses - poor feeding - respiratory distress

Question 136

Management: tetralogy of fallot

Answer 136

RVOT/ductal stent implantation Primary surgical repair

Question 137

Fallot spells

Answer 137

Right ventricle muscle goes into spasm preventing blood from flowing to the lungs - baby becomes blue - R to L shunting - short and self resolving Management: - propanolol/morphine

Question 138

What syndromes are associated with congenital heart defects?

Answer 138

- trisomy 21 = AVSD, TOF, VSD (all get cardiac screening) - Turner's (females, webbed neck) = coarctation of aorta, AS, aortic dissection later in life, bicuspid aortic valve - Williams syndrome (chromosome 7) = supravalvular AS, PA stenosis Di George - interrupted aortic arch - truncus arteriosis - TOF - VSD - PDA

Question 139

What is Eisenmenger syndrome?

Answer 139

when blood flows from the right side of the heart to the left across a structural heart lesions, bypassing the lungs - develop after 1-2 yrs with large shunts

Question 140

What are the examination findings for Eisenmenger syndrome?

Answer 140

Right ventricular heave: the right ventricle contracts forcefully against increased pressure in the lungs Loud P2: loud second heart sound due to forceful shutting of the pulmonary valve Raised JVP Peripheral oedema murmur depending on the location of septal defect

Question 141

Management: eisenmenger syndrome

Answer 141

1. oxygen 2. treatment for pulmonary hypertension = sildenafil 3. treatment of arrhythmias 4. treatment of polycythaemia with venesection 5. preventions and treatment of thrombosis with anticoagulation 6. prevention of infective endocarditis using prophylactic antibiotics

Question 142

Define: infective endocarditis

Answer 142

infection of the endothelium of the heart valves

Question 143

What are the risk factors for infective endocarditis?

Answer 143

MC with congenital cardiac disease Intravenous drug use Structural heart pathology (see below) Chronic kidney disease (particularly on dialysis) Immunocompromised (e.g., cancer, HIV or immunosuppressive medications) History of infective endocarditis Structural pathology can increase the risk of endocarditis: Valvular heart disease Congenital heart disease Hypertrophic cardiomyopathy Prosthetic heart valves Implantable cardiac devices (e.g., pacemakers)

Question 144

What are the features of infective endocarditis?

Answer 144

- fever - heart murmur - splenomegaly - petechiae - Osler's nodes - laneway lesions splinter haemorrhages

Question 145

investigations: infective endocarditis

Answer 145

Blood cultures and echo

Question 146

What is the criteria for infective endocarditis?

Answer 146

Modified Duke's criteria Major - typical microorganism on 2 separate blood cultures - evidence of endocardial involvement minor - predisposition - fever >38 - vascular phenomena - immunologica phenomena - micro and bloods that don't meet major criteria 1 major + 3 minor 5 minor

Question 147

Management: IE

Answer 147

IV broad spectrum antibiotics 4-6 weeks surgery - if due to valve pathology - large vegetation's or abscesses - not responding to antibiotics

Question 148

Define: Faltering growth

Answer 148

Failure to gain adequate weight or achieve adequate growth during infancy or early childhood. - a significant interruption in the expected rate of growth compared with other children of similar age and sex during early childhood.

Question 149

What are the thresholds of concern about faltering growth in infants and children?

Answer 149

a fall across 1 or more weight centile spaces, if birth weight was below the 9th centile. a fall across 2 or more weight centile spaces, if birth weight was between the 9th and 91st centiles. a fall across 3 or more weight centile spaces, if birth weight was above the 91st centile. when current weight is below the 2nd centile for age, whatever the birth weight.

Question 150

How to monitor weight for different age groups?

Answer 150

- daily if less than 1 month old - weekly between 1–6 months old - fortnightly between 6–12 months - monthly from 1 year of age

Question 151

What to do if infants in the early days of life lose more than 10% of their brith weight?

Answer 151

1. perform a clinical assessment 2. take a detailed history to assess feeding 3. consider direct observation of feeding 4. perform further investigations only if they are indicated based on the clinical assessment 5. provide feeding support (by a person with appropriate training and expertise).

Question 152

What should you be concerned about linear growth when looking at BMIs?

Answer 152

In a child over 2 years of age determine the BMI centile: BMI< 2nd centile this may reflect either undernutrition or a small build BMI < 0.4th centile probable undernutrition that needs assessment and intervention

Question 153

What are the medical risk factors for faltering growth?

Answer 153

Congenital anomalies (cerebral palsy, autism, trisomy 21) Developmental delay Gastroesophageal reflux Low birth weight (<2.500g) Poor oral health, dental caries Prematurity (<37w) Tongue-tie (controversial)

Question 154

What are the psychosocial risk factors for faltering growth?

Answer 154

Disordered feeding techniques Family stressors Parental or family history of abuse/violence Poor parenting skills Postpartum depression Poverty

Question 155

How to assess a child with faltering growth?

Answer 155

- Perform a clinical, developmental and social assessment - Take a detailed feeding or eating history - Consider direct observation of feeding or meal times Refer if symptoms indicate an underlying disorder

Question 156

What examinations can be found in a child with faltering growth?

Answer 156

Dysmorphic appearance (genetic abnormality) Oedema (renal, liver disease) Hair color/texture change )(zinc deficiency) Heart murmur (anatomic cardiac defect) Hepatomegaly (infection, chronic illness, malnutrition) Mental status change (cerebral palsy, poor social bonding) Poor parent- child interaction (depression, social stress) Rash, skin changes, bruising (HIV infection, abuse) Respiratory compromise (CF) Wasting (cerebral palsy, cancer)

Question 157

What 4 main physiological factors affect weight gain?

Answer 157

Not enough in Not absorbed Too much used up Abnormal central control of growth/appetite

Question 158

What can cause faltering growth in milk-fed infants?

Answer 158

ineffective suckling in breastfed infants ineffective bottle feeding feeding patterns or routines being used the feeding environment feeding aversion parent/carer–infant interactions physical disorders that affect feeding

Question 159

When is enteral tube feeding used for faltering growth?

Answer 159

If there are serious concerns about weight gain. An appropriate specialist multidisciplinary assessment for possible causes and contributory factors has been completed. Other interventions have been tried without improvement.

Question 160

What can cause inadequate nutrient absorption?

Answer 160

Anemia (iron deficiency) Biliary atresia Coeliac disease Chronic GI conditions (infections, IBS) Cystic fibrosis Inborn errors of metabolisms Milk protein allergy Pancreatic cholestatic conditions

Question 161

What are the antibodies of coeliac disease?

Answer 161

1. Tissue transglutaminase antibodies (anti-TTG) 2. Endomysial antibodies (EMAs) 3. Deaminated gliadin peptides antibodies (anti-DGPs)

Question 162

Presentation: coeliac disease

Answer 162

often asymptomatic so low threshold to test Failure to thrive in young children Diarrhoea Fatigue Weight loss Mouth ulcers Anaemia secondary to iron, B12 or folate deficiency Dermatitis herpetiformis is an itchy blistering skin rash that typically appears on the abdomen all new type 1 diagnosis are tested for coeliac disease (often linked)

Question 163

Investiagtions: coeliac disease

Answer 163

- antibody test: * check total immunoglobulin A levels to exclude IgA deficiency * raised anti-TTG antibodies * raised anti-endomysial antibodies - serological test after gluten free first for 6 weeks - Upper Gi endoscopy and small bowel biopsy * crypt hypertrophy * villous atrophy

Question 164

Management: coeliac disease

Answer 164

Gluten free diet

Question 165

What are the complications of untreated coeliac disease?

Answer 165

Vitamin deficiency Anaemia Osteoporosis Ulcerative jejunitis Enteropathy-associated T-cell lymphoma (EATL) of the intestine Non-Hodgkin lymphoma (NHL) Small bowel adenocarcinoma (rare)

Question 166

What are the features and treatment of Giardia?

Answer 166

Only 20% pick up rate on immediate stool examination May last for years: foreign travel not necessary Giardia stool ELISA Practically: treat empirically with metronidazole 15mg/kg 3x a day for 7 days

Question 167

What are the 2 types of cow's milk allergy?

Answer 167

1. IgE mediated - angiooedema - pruritus - colicky abdominal pain - vomiting and diarrhoea - acute urticaria 2. Non-IgE mediated - GOR - loose or frequent stools - blood/mucus in stools - ado pain - food refusal/aversion - perinatal redness - pallor or tiredness - faltering growth - atopic eczema

Question 168

Presentation: Cow's milk allergy

Answer 168

- vomiting - asthma - swelling of the lips or eyelids - wheezing and coughing - iron deficiency anaemia - refusal to feed - blood in poop - diarrhoea - skin rash

Question 169

Management: Cow's milk allergy

Answer 169

- no investigations, diagnosis made on clinical findings = skin prick testing - put on diary free diet for 3 weeks and see if effective

Question 170

What would cause increased metabolism leading to faltering growth?

Answer 170

Chronic infections (HIV, tuberculosis) Chronic lung disease of prematurity Congenital heart disease Hyperthyroidism Inflammatory conditions (e.g. asthma, IBD) Malignancy Renal failure

Question 171

What can cause abornomal growth control?

Answer 171

- GH - thyroid - psychosocial influcence rare

Question 172

Define: avoidant or restrictive food intake disorder (ARFID)

Answer 172

- significant weight loss - nutritional deficiencies (such as iron deficiency anemia) - a dependence on nutritional supplements, (i.e., oral or enteral formulas), to meet energy requirements without an underlying condition necessitating this - significant interference with day-to-day functioning due to the inability to eat appropriately.

Question 173

What are the challenges to management of faltering growth?

Answer 173

Faltering growth is complex and often multifactorial and a specific underlying cause may not be identified. Children may undergo excessively frequent monitoring or unnecessary investigations looking for an underlying disorder. Parents may feel blamed for their child’s slow weight gain, whereas neglect is an uncommon cause of faltering growth. Healthcare professionals should remain alert to the possibility of safeguarding concern, but should be sensitive to the emotional impact of caring for a child with faltering growth.

Question 174

What should always be ruled out in assessing children with faltering growth?

Answer 174

- urinary tract infection - coeliac disease, if the diet has included gluten-containing foods

Question 175

Presentation: Infantile spasms

Answer 175

aka West syndrome - spasms consist of a sudden stiffening. often the arms fling out as the knees pull up and the body bends forward - may cry or after seizure - lasts 1-2 seconds. and occur close together in a series - happen most often just after waking up (rare during sleep) - skin changes, dysmorphism

Question 176

What causes infantile spasms?

Answer 176

- structural change in brain e.g. brain infection, hypoxia, dysplasia - Tuberous sclerosis (growths present in brain) MC

Question 177

Investigations: infantile spasms

Answer 177

- History and see - Classical EEG- hypsarrythmia - Developmental delay/regressing

Question 178

Management: Infantile spasms

Answer 178

Vigabatrin +/- Prednisolone

Question 179

Define: Sleep myoclonus

Answer 179

- benign - jerking movements when asleep - growing well No treatment

Question 180

What are febrile convulsions?

Answer 180

A type of seizure that occurs in children with a high fever (usually after viral infection) - children aged 6 months to 5 yrs

Question 181

What are the 2 types of febrile convulsions?

Answer 181

1. simple febrile convulsions = generalised, tonic clonic seizures that last 15 mins and occur only once 2. Complex febrile convulsions = consist of partial or focal seizures, last more than 15 mins or occurs multiple times during the same febrile illnes

Question 182

How do you diagnose febrile convulsions?

Answer 182

Child around 18months presenting with a seizure during a high fever (once seizure is finished, he acts normal and well again) Good GCS rule out other diagnoses e.g. epilepsy, meningitis, intracranial space occupying lesion, trauma rule out riggers = normal shaking with a fever or delirius

Question 183

Management: febrile convulsion

Answer 183

1. manage source of infection and control fever with analgesia 2. if complex give parents advice on how to deal with seizures

Question 184

What are the features of Reflex anoxic seizure?

Answer 184

- Triggered by pain or strong emotion - goes stiff and then starts jerking - Results in decreased perfusion to brain - Work up includes ECG (?arrhythmia) and EEG (normal) - can happen at any age Prognosis- Most will grow out of it

Question 185

What are the features of Juvenile myoclonic epilepsy?

Answer 185

- myoclonic jerks - absence seizures = become clumsy as LoC for a second - worse when tired

Question 186

Treatment: juvenile myoclonic epilepsy

Answer 186

Sodium valproate (now need 2 neurologist to sign before prescribing) NOT carbamazepine (focal seizures respond well to it)

Question 187

What are the features and investigations for Faints/syncope?

Answer 187

- ask what they were doing - eaten food and dehydrated - completely aware after - last for 30s, LOC Investigations - ECG - BP lying and standing - rule out family history

Question 188

What are the features of Absence seizures?

Answer 188

- vacant, daydreaming episodes - induced by hyperventilation (signifies its epilepsy) - last 30s - behavioural arrest, when aware again will go back to what they were doing - MC in childhood

Question 189

treatment: absence seizures

Answer 189

ethosuximide (zarontin)

Question 190

What does an EEG show for an absence seizure?

Answer 190

3Hz spike and wave discharges

Question 191

Define: status epilepticus

Answer 191

A seizure that lasts for more than 5 mins

Question 192

What is the protocol for status epilepticus?

Answer 192

1. ABCDE 2. 5 mins: Benzodiazepine (midazolam buccal, lorazepam IV, diazepam PR) 3. 10mins later: Further benzodiazepine e.g. IV lorazepam 4. Prepare PR paraldehyde 5. Prepare phenytoin 6. Call for senior support 7. 10mins later: IV phenytoin

Question 193

What is the complication of status epilepticus?

Answer 193

airway is shut off in seizure After 30 mins will start developing irreversible changes to brain

Question 194

What are the 4 categories for child abuse?

Answer 194

- physical abuse - sexual abuse - emotional abuse - neglect

Question 195

What are the risk factors for child abuse?

Answer 195

Parental: Lack of support Generational effect Deprivation Mental health problems Learning difficulty Alcohol and substance misuse Domestic violence ACEs- adverse childhood experiences Child: Difficult to care for Preterm Disabled

Question 196

How do children who've been abused present?

Answer 196

Disclosure Injury observed e.g. At school Found incidentally when attending for other reason Injury presented with or without explanation.

Question 197

What are the types of injuries that present with child abuse?

Answer 197

Superficial: Bruises (age is important, if they can't walk yet why do they have bruises?) Scratches Marks from implements (Patterned injuries) Burns and scalds Fractures Multiple long bones, ribs, metaphyseal Internal Organ Damage Abdominal Intracranial

Question 198

What are the consequences of a shaken baby?

Answer 198

Cerebral Hypoxia* Subdural haematoma* Rib fractures Retinal haemorrhages* May have other fractures, torn frenulum, bruises etc.

Question 199

How does sexual abuse present?

Answer 199

Disclosure or allegation Behaviour change: Sexualised Indicative of distress Physical symptoms: Bleeding Discharge Soreness Wetting or soiling STI Pregnancy

Question 200

What do persistence of primitive reflexes in a infant indicate?

Answer 200

Upper motor neuron abnormality

Question 201

Define: cerebral palsy

Answer 201

permanent neurological problems resulting from damage to the brain around the time of brith - non progressive - variation in severity anf symptoms

Question 202

What are the causes of cerebral palsy?

Answer 202

Antenatal: Maternal infections Trauma during pregnancy Perinatal: Birth asphyxia Pre-term birth Postnatal: Meningitis Severe neonatal jaundice Head injury

Question 203

What are the types of cerebral palsy?

Answer 203

- Spastic: hypertonia (increased tone) and reduced function resulting from damage to upper motor neurones - Dyskinetic: problems controlling muscle tone, with hypertonia and hypotonia, causing athetoid movements and oro-motor problems. This is the result of damage to the basal ganglia. - Ataxic: problems with coordinated movement resulting from damage to the cerebellum - Mixed: a mix of spastic, dyskinetic and/or ataxic features

Question 204

What are the patters of spastic cerebral palsy?

Answer 204

Monoplegia: one limb affected Hemiplegia: one side of the body affected Diplegia: four limbs are affects, but mostly the legs Quadriplegia: four limbs are affected more severely, often with seizures, speech disturbance and other impairments

Question 205

Presentation: cerebral palsy

Answer 205

Might have hypoxic-ischaemic encephalopathy first Become more evident during development: - Failure to meet milestones - Increased or decreased tone, generally or in specific limbs - Hand preference below 18 months is a key sign to remember for exams - Problems with coordination, speech or walking - Feeding or swallowing problems - Learning difficulties

Question 206

What types of gait is present in cerebral palsy?

Answer 206

hemiplegic or diplegia gait - due to increased muscle tone and spascitiy in the legs - leg will be extended with plantar flexion of the feet and toes (swing leg in a semicircle around them)

Question 207

What are the complications of cerebral palsy?

Answer 207

Learning disability Epilepsy Kyphoscoliosis Muscle contractures Hearing and visual impairment Gastro-oesophageal reflux

Question 208

Management: cerebral palsy

Answer 208

permanent condition - physiotherapy - occupational therapy = support with getting dressed, washing, cooking - speech and language therapy - dieticians = peg feeding - orthopaedic surgeons = to release contractors or lengthen tendons medications - muscle relaxants e.g. baclofen - anti-epileptic drugs = seizures - glycopyrronium bromide = excessive drooling

Question 209

Define: syncope

Answer 209

event of temporarily losing consciousness due to a disruption of blood flow to the brain, often leading to a fall - problem with autonomic nervous system regulating blood flow to the brain

Question 210

What are the signs and symptoms of syncope?

Answer 210

Patients often remember the event and can recall how they felt prior to fainting. This is called the prodrome, and involves feeling: Hot or clammy Sweaty Heavy Dizzy or lightheaded Vision going blurry or dark Headache loss of consciousness, fall over ditching, shaking

Question 211

What are the primary and secondary causes of syncope?

Answer 211

Primary syncope (simple fainting): Dehydration Missed meals Extended standing in a warm environment, such as a school assembly A vasovagal response to a stimuli, such as sudden surprise, pain or the sight of blood Secondary causes: Hypoglycaemia Dehydration Anaemia Infection Anaphylaxis Arrhythmias Valvular heart disease Hypertrophic obstructive cardiomyopathy

Question 212

What are the differences between syncope and epilepsy?

Answer 212

syncope - prolonged upright position before event + lightheadedness + sweating + blurred vision - reduced tone

Question 213

Define: disability

Answer 213

someone who has a physical or mental impairment that results in a marked, pervasive limitation of activity

Question 214

Define: impairment

Answer 214

an abnormality or loss of function

Question 215

What are the types of impairments?

Answer 215

- Physical E.g. cerebral palsy, brain injury, neuromuscular disorders, musculoskeletal conditions-ABI * Sensory Impairments = Hearing and visual impairments * Learning e.g. low IQ * Neurodevelopmental disorders E.g.ADHD,Autism - * Emotional/Behavioral E.g. Depression Conduct disorder, attachment disorder D

Question 216

What are the 3 things to assess in disabilities?

Answer 216

1. functioning 2. activity 3. their ability to participate

Question 217

What are the 6 domains of functioning included in WHODAS?

Answer 217

World health organisation disability assessment schedule - Cognition – understanding & communicating * Mobility– moving & getting around * Self-care–hygiene,dressing,eating&stayingalone * Social– interacting with other people * Lifeactivities–domesticresponsibilities,leisure,work&school * Participation– joining in community activities

Question 218

What are factors that affect children with learning disabilities?

Answer 218

* Disabled children are more likely to live in poverty * Parents are more likely to be unemployed * Higher rates of mental health needs * Poorer physical health and lower life expectancy

Question 219

Define + 3 main factors: quality of life

Answer 219

The degree to which a person enjoys the important possibilities of his/her life. * Being –who one is * Belonging –how one fits into the environment * Becoming –How to have purposeful activities in order to achieve ones goals

Question 220

What are 3 ages that diagnosis for vomiting changes?

Answer 220

6 hrs - neonates 6 weeks 6 months likely differential diagnosis changes with each

Question 221

What are the causes of vomiting in neonates?

Answer 221

- GORD (reflux)n= yellow/milky - overfeeding = milky - duodenal atresia/stenosis - sepsis - malrotation =

Question 222

investigations: sepsis

Answer 222

Bloods = FBC, U+Es, CRP, blood gas = lactate Blood culture Lumbar puncture = for meningitis Urine dip = UTI

Question 223

Define: malrotation and volvulus

Answer 223

bowel has herniated into umbilicus and then has not rotated correctly when goes back into the abdomen - reduced surface area so easier for bowel to twist on blood supply - then bowel can infarct

Question 224

Investigations: malrotation

Answer 224

green vomiting > barium meal - x-ray needs diagnosis within 6 hrs to prevent infarction of bowels

Question 225

What is the cause of vomiting in infants?

Answer 225

- overfeeding - GORD - sepsis - pyloric stenosis

Question 226

Define: pyloric stenosis

Answer 226

ring of smooth muscle the forms the canal between the stomach and the duodenum. Hypertrophy (thickening) and narrowing of the pylorus is called pyloric stenosis. This prevents food traveling from the stomach to the duodenum as normal. after feeding, peristalsis in the stomach tries to push food into duodenum and becomes so powerful that it ejects the food in the oesophagus and out the mouth

Question 227

Presentation: Pyloric stenosis

Answer 227

- projectile vomiting - failing to thrive - firm, round mass found I upper abdomen - 4-6 weeks old

Question 228

Investigations: pyloric stenosis

Answer 228

blood gas analysis - hypochloric metabolic alkalosis (as vomiting HCL form stomach) - low potassium (excrete potassium) Abdo USS (then resuscitate baby by giving them fluids)

Question 229

Management: pyloric stenosis

Answer 229

laparoscopic pyloromyotomy (known as “Ramstedt’s operation“) - incision in smooth muscle of pylorus to widen the canal

Question 230

Define: intussusception

Answer 230

bowel telescopes into itself, folding inwards - thickens the overall size of the bowel and narrows lumen leading to obstruction of faeces - mC 6-12 months old due to start weaning > stimulate GI immune system > Peyers patch enlarge as challenges lots of immune cells > peristalsis cause preyers patches to get caught

Question 231

Presentation: intussusception

Answer 231

Severe, colicky abdominal pain Pale, lethargic and unwell child “Redcurrant jelly stool” = blood coming from iscahemic bowel caught Right upper quadrant mass on palpation. This is described as “sausage-shaped” = feeling when bowel has folded Vomiting = milky then green Intestinal obstruction

Question 232

Causes: intussusception

Answer 232

Most idiopathic ileocolic anatomical lead point associated with CF and Meckel's diverticulum

Question 233

Investigations: intussusception

Answer 233

USS - doughnut shape/ target shaped mass Abdominal X-ray - distended small bowel with absence of gas in the large bowel

Question 234

Management: intussusception

Answer 234

Air/water/contrast enema 90% = pushes folded bowel out of the bowel and into the normal position surgical reduction Recur

Question 235

What are the complications of intussusception?

Answer 235

Obstruction Gangrenous bowel Perforation Death

Question 236

Define: acute abdominal pain

Answer 236

less than 7 days duration sudden onset severe

Question 237

What are the most common surgical causes of acute abdominal pain in children?

Answer 237

1. non-specific abdomen pain 2. appendicitis 3. ovarian pathology = ovarian torsion, cysts 4. intussusception 5. Merkel's diverticulum

Question 238

What are 5 non-surgical causes of acute abdominal pain?

Answer 238

1. UTI 2. gastroenteritis 3. constipation 4. pancreatitis = gallstones 5. right sided pneumonia

Question 239

What are the red flags for serious abdominal pain?

Answer 239

Persistent or bilious vomiting Severe chronic diarrhoea Fever Rectal bleeding Weight loss or faltering growth Dysphagia (difficulty swallowing) Nighttime pain Abdominal tenderness

Question 240

What is guarding?

Answer 240

Abdominal wall muscles tense up in response to pain or inflammation inside the abdomen voluntary or involuntary (signifies inflammation in peritoneum causing overlaying muscles to go into spasm > surgery)

Question 241

Investigations: acute abdo pain

Answer 241

- urine dip = exclude UTI - FBC, CRP - U+E - USS abdo

Question 242

What is a abdominal migraine?

Answer 242

children sometimes get this before developing traditional migraines as they get older - central abdo pain lasting more than 1 hr - examination will be normal - associated with = nausea, vomiting, anorexia, pallor, headache, photophobia, aura

Question 243

Management: abdominal migraine

Answer 243

acute attacks - low stimulus environments - paracetamol - ibuprofen - sumatriptan preventative - Pizotifen, a serotonin agonist - Propranolol, a non-selective beta blocker - Cyproheptadine, an antihistamine - Flunarazine, a calcium channel blocker

Question 244

Define: appendicitis

Answer 244

inflammation of the appendix - infection gets trapped in the appendix by obstruction

Question 245

Presentation: appendicitis

Answer 245

MAGNET migration of pain to RIF anorexia (LOA) guarding nausea (not much vomit) elevated temp tenderness in RIF acute onset Rovsing's sign Rebound tenderness percussion tenderness

Question 246

Diagnosis: appendicitis

Answer 246

- clinical presentation - inflammatory markers - CT scan - USS in females to exclude gynae - diagnostic laparoscopy

Question 247

Management: appendicits

Answer 247

appendicectomy - laparoscopic better than laparotomy

Question 248

Complication sof appendicetomy

Answer 248

Bleeding, infection, pain and scars Damage to bowel, bladder or other organs Removal of a normal appendix Anaesthetic risks Venous thromboembolism (deep vein thrombosis or pulmonary embolism)

Question 249

Define: Self harm

Answer 249

act with intent to hurt themselves - no intention to kill self - associated with suicidal ideas therefore check

Question 250

Define: suicide attempt

Answer 250

act with intent to kill self includes overdose, attempted hanging intention includes desire to be dead (its what the intent was, even if the act did not/would not kill them)

Question 251

What are the causes of self harm?

Answer 251

-depression bullying sexual abuse family/friend conflict subculture (self-harm) drugs and alcohol misuse

Question 252

What are the factors that increase the risk of completed suicide?

Answer 252

presence of psychiatric disorder previous suicide attempt alcohol or substance misuse

Question 253

What needs to be checked in an assessment of a suicidal attempt?

Answer 253

Circumstances eg alone, did they tell anyone Planned or impulsive Left letter Continuing ideas What would stop a further episode hope for the Future

Question 254

Management: depression

Answer 254

Mild depression = watchful waits advice, lifestyle changes Moderate to severe: - referral to CAMHS - establish diagnosis - psychological therapy = CBT - 1st line = Fluoxetine - 2nd = sertraline + citalopram - continue medication until 6 months after remission

Question 255

Define: anorexia nervosa

Answer 255

the person feel they are overweight despite evidence of normal or low body weight - involves restricting calorie intake with the intention of losing weight (no longer BMI criteria)

Question 256

What are the risk factors of anorexia?

Answer 256

- low self esteem - complex family dynamics - bereavement - pressure of education - social pressure - genetic risk - abuse - perfectionist/obsessive personality traits - co-morbidities - gender dysphoria, depression, anxiety, OCD, ASD, attachment disorder

Question 257

What are the features of anorexia nervosa?

Answer 257

Excessive weight loss Amenorrhoea Lanugo hair is fine, soft hair across most of the body Hypokalaemia Hypotension Hypothermia Changes in mood, anxiety and depression Solitude Cardiac complications include arrhythmia, cardiac atrophy and sudden cardiac death.

Question 258

How to diagnose anorexia

Answer 258

weight loss is self induced by avoidance of 'fattening foods' 1 or more - self induced vomiting - self induced purging - excessive exercise - use of appetite suppressants and/or diuretics body image distortion

Question 259

Define: bulimia nervosa

Answer 259

recurrent episodes of binge eating: - eating within any 2hr period an amount of food that most would not eat - a feeling that line one cannot stop eating The period of purging by induced vomiting or taking laxatives to prevent the calories being absorbed can have normal body weight

Question 260

What are the features of bulimia?

Answer 260

- Alkalosis, due to vomiting hydrochloric acid from the stomach - Hypokalaemia - Erosion of teeth - Swollen salivary glands - Mouth ulcers - Gastro-oesophageal reflux and irritation - Calluses on the knuckles where they have been scraped across the teeth. This is called Russell’s sign.

Question 261

Management: bulimia nervosa

Answer 261

Guided self-help - monitor what eating - make realistic meal plans CBT - BN family therapy antidepressants e.g. fluoxetine

Question 262

What are the features of a binge eating disorder?

Answer 262

1. eating in discrete period of time, that is defo larger that most people would eat 2. lack of control over eating and marked distress 3. at least 3 of: - eating more rapidly than normal - eating until feeling uncomfortably full once a week for 3 months Usually overweight

Question 263

Management: binge eating disorder

Answer 263

- weight control - guided self help - CBT - meal plan - work not riggers - change negative feelings about body

Question 264

Define: avoidance restricted food intake disorder

Answer 264

1. An eating or feeling disturbance manifested by persistent failure to meet appropriate nutritional and/ or energy needs associated with one or more - sig weight loss - sig nutri def - dependence on enteral feeding - marked interference with psychosocial functioning (e.g. maybe only eats beige food or certain textures) not better explained by lack or food

Question 265

What other conditions or thinking is linked with ARFID?

Answer 265

- linked with high rates of autism - often GI problems like reflux or vomiting - might be a sensory issue or a fear of choking/vomiting - linked with anxiety, OCD and ADHD - not interested in food so genuinely forget to eat

Question 266

what are some other eating disorders?

Answer 266

Orthorexia - eating helpfully that goes too far body dysmorphic disorder Diabulimia = people with diabetes Pica = eating things that aren't food Bigorexia = people who don't think they are big/muscly enough

Question 267

What are the short and long term problems of anorexia?

Answer 267

short term - malnutrition - weight loss - dry skin - atria loss - dizziness/fainting weakness - low BP Low body temp long term - severe dehydration - cardiac problems - infertility - fragile bones = stops releasing oestrogen so can't lay down good bones especially if in teen years - depression, anxiety - risk of relapse post recovery

Question 268

What is the effect of eating disorder on family?

Answer 268

- guilt, blame - anxiety, confusion - shrinking of social/family life - future plans on hold - lives reorganised

Question 269

MAnagement: anorexia

Answer 269

- if medically unstable need to be admitted for stabilisation and re-feeding admission after discharge: - continue meal plan - follow up with community EDS - follow up in medical/physical health monitoring clinics - or transfer to tier 4 bed for further intense treatment (always get to a healthy weight before starting next steps)

Question 270

What are the 4 phases of Family Based Treatment?

Answer 270

1. acknowledge starvation, focus on referring and control given to parents 2. continued focus on weight gain, starting to shift responsibility from parent to young person 3. weight maintenance, focus on family relationships, develop family strengths (may start family therapy) 4. relapse prevention

Question 271

Define: Juvenile Idiopathic Arthritis

Answer 271

Onset before 16th birthday Persistent joint swelling lasting at least 6 weeks with no identified underlying cause

Question 272

What are the 5 key subtypes of Juvenile Idiopathic arthritis?

Answer 272

1. Systemic JIA 2. Polyarticular JIA 3. Oligoarticular JIA 4. Enthesitis related arthritis 5. Juvenile psoriatic arthritis

Question 273

What are the 3 key features of juvenile idiopathic arthritis (JIA)?

Answer 273

- joint pain - swelling - stiffness

Question 274

What are the features of systemic JIA?

Answer 274

Still's disease Subtle salmon-pink rash High swinging fevers Enlarged lymph nodes Weight loss Joint inflammation and pain Splenomegaly Muscle pain Pleuritis and pericarditis

Question 275

What would investigations show for systemic JIA?

Answer 275

Antinuclear antibodies + rheumatoid factor = negative inflammatory markers - CRP, ESR, platelets and serum ferritin = raised

Question 276

What is a key complication of systemic JIA?

Answer 276

Macrophage activation syndrome - severe activation of the immune system - acutely unwell child with disseminated intravascular coagulation DIC, anaemia, thrombocytopenia, bleeding, and non-blanching rash LOW ESR

Question 277

What are the key non-infective differentials when a child presents with a fever for more than 5 days?

Answer 277

1. Kawasaki disease 2. Still's disease 3. Rheumatic fever 4. Leukaemia

Question 278

What are the features of polyarticular JIA?

Answer 278

- idiopathic inflammatory arthritis in 5 joints or more - symmetrical - minimal systemic symptoms = can be mild fever, anaemia and reduced growth - normally rheumatoid factor negative (equivalent to rheumatoid arthritis in adults)

Question 279

What are the features of Oligoarticular JIA?

Answer 279

AKA pauciarticular JIA - involves 4 joints or less - usually only affects single joint - larger joints e.g. ankle or knee - more common in girls under 6yrs - no systemic symptoms - inflammatory markers normal or mildly elevated

Question 280

What is a key feature/complication of oligoarticular JIA?

Answer 280

Anterior uveitis

Question 281

What are the features of Enthesitis-Related Arthritis?

Answer 281

- paediatric version of seronegative spondyloarthropathy = HLA B27 gene - inflammation where the tendon inserts into the muscle MC in males >6 yrs

Question 282

Investigations: Enthesitis Related arthritis

Answer 282

MRI scan - but cannot distinguish between an enthesitis due to stress or an autoimmune process. HLA-B27 testing ANA and RF = negative prone to anterior uveitis = seen by ophthalmologist

Question 283

What are the features of juvenile psoriatic arthritis?

Answer 283

- seronegative inflammatory arthritis associated with psoriasis - symmetrical polyarthritis affecting the small joints

Question 284

What are the signs of juvenile psoriatic arthritis on examination?

Answer 284

- Plaques of psoriasis on the skin - Pitting of the nails (nail pitting) - Onycholysis, separation of the nail from the nail bed - Dactylitis, inflammation of the full finger - Enthesitis, inflammation of the entheses, which are the points of insertion of tendons into bone

Question 285

Management: juvenile idiopathic arthritis

Answer 285

Specialist paediatric rheumatologist - NSAIDs e.g. ibuprofen - Steroids = either oral, intramuscular or intra-artricular in oligoarthritis - Disease modifying anti-rheumatic drugs (DMARDs) e.g. methotrexate, sulfasalazine and leflunomide - Biologic therapy = tumour necrosis factor inhibitors etanercept, infliximab and adalimumab

Question 286

Define: osteogenesis imperfecta

Answer 286

- genetic condition - results in brittle bones that are prone to fractures - mutations affect formation of collagen

Question 287

Presentation: osteogenesis imperfecta

Answer 287

- Hypermobility - *Blue / grey sclera (the “whites” of the eyes) - Triangular face - Short stature - Deafness from early adulthood - Dental problems, particularly with formation of teeth - Bone deformities, such as bowed legs and scoliosis - Joint and bone pain

Question 288

Investigations: osteogenesis imperfecta

Answer 288

clinical diagnosis x-rays Genetic testing but no routinely done

Question 289

Management: osteogenesis imperfecta

Answer 289

- bisphosphates = increase bone density - vitamin D supplementation = prevent deficiency

Question 290

Define: Rickets

Answer 290

condition affecting children where there is defective bone mineralisation causing 'soft' and deformed bones

Question 291

What are the causes of Ricketts?

Answer 291

- deficiency in vitamin D or calcium a rare from caused by genetic defects resulting in low phosphate in the blood = hereditary hypophosphataemic rickets

Question 292

What is the pathophysiology of rickets?

Answer 292

1. vitamin D is a hormone created from cholesterol by the skin in response to UV radiation 2. malabsorption disorders reduce the amount of vit D absorbed 3. vitamin D is essential in calcium and phosphate absorption from the intestines and kidneys 4. reduced calcium and phosphate in the blood means less in bones = defective bone mineralisation 5. low calcium causes a secondary hyperparathyroidism as the parathyroid gland tries to raised calcium level by secreting parathyroid hormone so increased reabsorption of calcium from the bones

Question 293

Presentation: Rickets

Answer 293

Lethargy Bone pain Swollen wrists Bone deformity Poor growth Dental problems Muscle weakness Pathological or abnormal fractures

Question 294

What bone deformities occur in rickets?

Answer 294

- Bowing of the legs = where the legs curve outwards - Knock knees = the legs curve inwards - Rachitic rosary = the ends of the ribs expand at the costochondral junctions, causing lumps along the chest - Craniotabes = which is a soft skull, with delayed closure of the sutures and frontal bossing - Delayed teeth with under-development of the enamel

Question 295

Investigations: rickets

Answer 295

Serum 25-hydroxyvitamin D = <25 is vit D deficient X-ray = for diagnosis Serum calcium may be low Serum phosphate may be low Serum alkaline phosphatase may be high Parathyroid hormone may be high

Question 296

What other tests done to rule out conditions for rickets?

Answer 296

Full blood count and ferritin, for iron deficiency anaemia Inflammatory markers such as ESR and CRP, for inflammatory conditions Kidney function tests, for kidney disease Liver function tests, for liver pathology Thyroid function tests, for hypothyroidism Malabsorption screen such as anti-TTG antibodies, for coeliac disease Autoimmune and rheumatoid tests, for inflammatory autoimmune conditions

Question 297

management: rickets

Answer 297

Prevention - breastfed babies receive less vit D as it is included in formula (breastfeeding women and all children should take vit D supplement) Treatment - vitamin D e.g. ergocalciferol - calcium

Question 298

Define: transient synovitis

Answer 298

irritable hip

Question 299

Define: Osgood-Schlatter disease

Answer 299

- caused by inflammation at the tibial tuberosity where the patella ligament inserts - common cause of anterior knee pain in adolescents - unilateral

Question 300

Pathophysiology: Osgood-Schlatters disease

Answer 300

patella tendon inserts into tibial tuberosity (epiphyseal plate) stress from running, jumping etc. result in inflammation on tibial epiphyseal plate (rare complication) multiple small avulsion fractures where patella ligament pulls away tiny pieces of the bone causing growth of tibial tuberosity initially this lump is tender due to inflammation but as the bone heals and inflammation settles it becomes hard and non-tender

Question 301

Presentation: Osgood Schlatter

Answer 301

Visible or palpable hard and tender lump at the tibial tuberosity Pain in the anterior aspect of the knee The pain is exacerbated by physical activity, kneeling and on extension of the knee

Question 302

Management: Osgood- schlatter

Answer 302

reducing the pain and inflammation. Reduction in physical activity Ice NSAIDS (ibuprofen) for symptomatic relief Physio Symptoms will fully resolve over time. The patient is usually left with a hard boney lump on their knee.

Question 303

What is the definition of regurgitation, rumination and possetting?

Answer 303

Regurgitation: effortless expulsion of gastric contents (healthy infants and older children who eat in excess) Rumination: frequent regurgitation of ingested food (largely behavioural) Possetting – small volume vomits during or between feeds in otherwise well child

Question 304

What are the common cubes of vomiting in neonatal 0-2 days?

Answer 304

-Duodenal or other intestinal atresia -TEF (types A/C)

Question 305

What is the common cause of vomiting in newborns (3 days -1 month)?

Answer 305

-Gastroenteritis -Pyloric stenosis -Malrotation +/- volvulus -TEF (types B/D/H) -Necrotizing enterocolitis -Milk protein intolerance -CAH -IEM

Question 306

What are the common causes of vomiting in infants (1 -36 months)?

Answer 306

-Gastroenteritis -UTI, pyelonephritis -GER -GERD -Ingestion -Intussusception -Milk protein intolerance

Question 307

What are the common causes of vomiting in a child (36 maths - 12 yrs)?

Answer 307

-Gastroenteritis -UTI -DKA -Increased intracranial pressure -Eosinophilic esophagitis -Appendicitis -Ingestion -Post-tussive vomiting

Question 308

What are the common causes of vomiting in adolescents (12-18yrs)?

Answer 308

-Gastroenteritis -Appendicitis -DKA -Increased intracranial pressure -Eosinophilic esophagitis -Bulimia nervosa -Pregnancy -Post-tussive vomiting

Question 309

Define: GORD/GOR

Answer 309

Gastrooesophageal reflux - the passage of gastric contents into the oesophagus - with or without regurgitation or vomiting GORD - the presence of troublesome symptoms and/ or complications of persistent GOR

Question 310

Presentation: GOR

Answer 310

Often babies reflux after larger feeds but is a problem if it causes them distress Chronic cough Hoarse cry Distress, crying or unsettled after feeding Reluctance to feed Pneumonia Poor weight gain Heartburn acid regurgitation epigastric pain

Question 311

What are the red flags of vomiting?

Answer 311

- Not keeping down any feed (pyloric stenosis or intestinal obstruction) - Projectile or forceful vomiting (pyloric stenosis or intestinal obstruction) - Bile stained vomit (intestinal obstruction) - Haematemesis or melaena (peptic ulcer, oesophagitis or varices) - Abdominal distention (intestinal obstruction) - Reduced consciousness, bulging fontanelle or neurological signs (meningitis or raised intracranial pressure) - Respiratory symptoms (aspiration and infection) - Blood in the stools (gastroenteritis or cows milk protein allergy) - Signs of infection (pneumonia, UTI, tonsillitis, otitis or meningitis) - Rash, angioedema and other signs of allergy (cows milk protein allergy) - Apnoeas are a concerning feature and may indicate serious underlying pathology and need urgent assessment

Question 312

Management: GOR/GORD

Answer 312

- Small, frequent meals - Burping regularly to help milk settle - Not over-feeding - Keep the baby upright after feeding (i.e. not lying flat) More problematic cases can justify treatment with: - Gaviscon mixed with feeds - Thickened milk or formula (specific anti-reflux formulas are available) - Proton pump inhibitors (e.g., omeprazole) Surgery - fundoplication

Question 313

Investigations: GORD

Answer 313

- pH - Barium swallow and meal then endoscopy - PPI test

Question 314

What is Sandifer's syndrome?

Answer 314

rare condition causing brief episodes of abnormal movements associated with GOR in infants - Torticollis = forceful contractions of the neck muscles causing twisting of neck - Dystonia = abnormal muscle contractions causing twisting movements, arching of the back or unusual postures usually resolves

Question 316

What is the ROME III criteria of functional constipation?

Answer 316

- Two or fewer defecations per week - At least 1 episode of faecal incontinence per week - Retentive posturing or stool retention. - Painful or hard bowel movements - Presence of a large faecal mass in the rectum - Large diameter stools that may obstruct the toilet

Question 317

What are the red flags of constipation?

Answer 317

- Delayed passage of meconium (within 48 hrs of birth) - Fever, Vomiting, Bloody Diarrhea - Failure to thrive - Tight, empty rectum with presence of palpable abdominal faecal mass - Abnormal anus (anal stenosis, IBD or sexual abuse) - abnormal lower back or buttocks (spina bifida, spinal cord lesion or sacral agenesis) - Abnormal neurological exam - acute severe abdo pain + bloating (obstruction/intussusception)

Question 318

What is encopresis?

Answer 318

means faecal incontinence not pathological until 4 yrs old - usually a sign of chronic constipation where the rectum becomes stretched and looses sensation - large hard stools remain in the rectum and only loose stools are able to leak out > soiling

Question 319

What are some other causes of encopresis?

Answer 319

Spina bifida Hirschprung’s disease Cerebral palsy Learning disability Psychosocial stress Abuse

Question 320

What lifestyle factors contribute to constipation?

Answer 320

- Habitually not opening the bowels - Low fibre diet - Poor fluid intake and dehydration - Sedentary lifestyle - Psychosocial problems such as a difficult home or school environment (always keep safeguarding in mind)

Question 321

What is a complication of constipation?

Answer 321

desensitisation of the rectum - if a habit is developed of not opening the bowels - they loose the sensation - start to retain faeces causing faecal impaction - rectum stretched over time and becomes further desensitised

Question 322

Management: constipation

Answer 322

1. Correct any reversible contributing factors, recommend a high fibre diet and good hydration 2. Start laxatives (movicol is first line) - keep going LT and slowly wean off until normal bowel habit 3. Faecal impaction may require a disimpaction regimen with high doses of laxatives at first 4. Encourage and praise visiting the toilet. This could involve scheduling visits, a bowel diary and star charts.

Question 323

What are the different types of medications for constipation?

Answer 323

Softener: lactulose, liquid paraffin Bulking agent: Fybogel Non-absorbed laxative irrigative: Movicol Stimulant: Senna, Dulcolax Enema Anal fissure: anaesthetic cream +/- vasodilator

Question 324

Define: gastroenteritis

Answer 324

inflammation all the way from the stomach to the intestines and presents with nausea, vomiting and diarrhoea

Question 325

What is the differential diagnosis of diarrhoea?

Answer 325

Infection (gastroenteritis) Inflammatory bowel disease Lactose intolerance Coeliac disease Cystic fibrosis = steatorrhoea Toddler’s diarrhoea Irritable bowel syndrome Medications (e.g. antibiotics)

Question 326

What are the causes of gastroenteritis?

Answer 326

MC - viral Rotavirus and norovirus MC - E.coli = some strains, produces Shiga toxin - Campylobacter jejune = travellers diarrhoea - Shigella = contaminated drinking water, pools or food - salmonella - giardiasis = microscopic parasite, faecal-oral transmission

Question 327

Management: gastroenteritis

Answer 327

1. isolate to prevent spread (stay off school until 48 hrs after symptoms resolved) 2. faeces tested for microscopy to establish antibiotic sensitivities 3. ensure hydrated = fluid challenge 4. slowly introduce food 5. Antidiarrhoeal (loperamide) and antiemetics (metoclopramide) NOT RECOMMENDED 6. antibiotics only given in patients at risk of complications once causative organism confirmed

Question 328

What are post- gastroenteritis complications?

Answer 328

Lactose intolerance Irritable bowel syndrome Reactive arthritis Guillain–Barré syndrome

Question 329

What are the features of Crohn's disease?

Answer 329

(crows NESTS) N – No blood or mucus (these are less common in Crohns.) E – Entire GI tract S – “Skip lesions” on endoscopy T – Terminal ileum most affected and Transmural (full thickness) inflammation S – Smoking is a risk factor (don’t set the nest on fire) Crohn’s is also associated with weight loss, strictures and fistulas.

Question 330

What are the features of ulcerative colitis?

Answer 330

U – C – CLOSEUP C – Continuous inflammation L – Limited to colon and rectum O – Only superficial mucosa affected S – Smoking is protective E – Excrete blood and mucus U – Use aminosalicylates P – Primary sclerosing cholangitis

Question 331

Presentation: IBD different to adults

Answer 331

- poor growth - delayed puberty - reduced final adult height - Catch up growth - persistent poor growth - only sign of disease activity

Question 332

Investigations: IBD

Answer 332

Blood tests - anaemia - infection - thyroid - kidney - liver function - CRP Faecal calprotectin - inflamed bowels Endoscopy = gold standard Imaging = USS, CT and MRI for complications e.g. fistulas, abscesses and strictures

Question 333

Management: Crohn's

Answer 333

Inducing remission 1. steroids e.g. oral prednisolone or IV hydrocortisone 2. alternative = azathioprine, methotrexate, mercaptopurine Maintaining remission 1. azathioprine or mercaptopurine 2. alternative = methotrexate, infliximab, adalimumab Surgery - if only affect distal ileum - to treat strictures and fistulas

Question 334

Management: UC

Answer 334

1. Inducing remission Mild to moderate disease First line: aminosalicylate (e.g. mesalazine oral or rectal) Second line: corticosteroids (e.g. prednisolone) Severe disease First line: IV corticosteroids (e.g. hydrocortisone) Second line: IV ciclosporin 2. Maintaining Remission Aminosalicylate (e.g. mesalazine oral or rectal) Azathioprine Mercaptopurine 3. Surgery - removing colon and rectum will remove the disease - ileostomy or oleo-anal anastomosis (J-pouch)

Question 335

Define: Biliary atresia

Answer 335

Congenital condition where a section of the bile duct is either narrowed or absent - results in cholestasis (bile cannot be transported from the liver to the bowel) - conjugated bilirubin excreted in the bile

Question 336

Presentation: biliary atresia

Answer 336

- persistent jaundice from birth till >14 days in term babies and 21 days in premature babies

Question 337

Investigations: biliary atresia

Answer 337

- check conjugated and unconjugated bilirubin levels

Question 338

management: biliary atresia

Answer 338

Surgery - kasai portoenterostomy = attaching a section of small intestine to the opening of the liver where the bile duct normally attaches often a full liver transplant needed in future

Question 339

Define: Hirschsprung's disease

Answer 339

Congenital condition where nerve cells of the myenteric plexus (Auerbach's plexus) are absent in the distal bowel and rectum - responsible for stimulating peristalsis of large bowel - the section of the colon without innervation becomes aganglionic and does not relax > constricts - proximal to the obstruction the bowel becomes distended and full

Question 340

What are the associations with Hirschsprung's?

Answer 340

- family history of hirschsprung's - Downs syndrome - neurofibromatosis - Waardenburg syndrome = a genetic condition causing pale blue eyes, hearing loss and patches of white skin/hair - multiple endocrine neoplasia type II

Question 341

Presentation: Hirschsprung's

Answer 341

Delay in passing meconium (more than 24 hours) Chronic constipation since birth Abdominal pain and distention Vomiting Poor weight gain and failure to thrive

Question 342

What is Hirschsprung-associated Enterocolitis?

Answer 342

Inflammation and obstruction of the intestine occurring in around 20% of neonates with Hirschsprung's disease. - within 2-4 weeks of birth - fever, abdominal distension, diarrhoea - life threatening > toxic megacolon, perforation of the bowel

Question 343

Investigation: Hirschsprung's

Answer 343

- Abdominal x-ray = diagnose intestinal obstruction - Rectal biopsy = absence of ganglionic cells

Question 344

Management: Hirschsprung's

Answer 344

1. fluid resuscitation 2. manage intestinal obstruction 3. IV antibiotici in HAEC 4. Definitive = surgical removal of the ganglionic section of bowel (most will then a live a normal life and may have LT disturbances in bowel function)

Question 345

Define: Meckel's diverticulum

Answer 345

A malformation of the distal ileum that occurs in around 2% of the population - usually asymptomatic + does not require treatment - it can bleed, become inflamed, rupture or cause a volvulus or intussusception

Question 346

Define: marasmus

Answer 346

A severe undernutrition - a deficiency in all the macronutrients that body requires to function

Question 347

Define: Kwashiorkor

Answer 347

A deficiency in protein predominantly

Question 348

Presentation: Marasmus vs kwashiorkor

Answer 348

Marasmus - wasted, shrivelled appearance kwashiorkor - oedema - swelling with fluid in abdomen and face

Question 349

What happens to the body in marasmus disease?

Answer 349

When the body is deprived of energy from food, it begins to feed on its own tissue - first adipose fat tissue and then muscle - reduced functions to conserve energy = low HR, low BP, low body temp - children stunted in growth

Question 350

What are the main causes of marasmus?

Answer 350

The main causes affecting all ages include: Poverty and food scarcity. Wasting diseases such as AIDS. Infections that cause chronic diarrhea. Anorexia. Additional causes affecting children include: Inadequate breastfeeding or early weaning of infants. Child abuse/neglect. Additional causes affecting adults include: Dementia. Elder abuse/neglect.

Question 351

Presentation: marasmus

Answer 351

Visible wasting of fat and muscle. Prominent skeleton. Head appears large for the body. Face may appear old and wizened. Dry, loose skin (skin atrophy). Dry, brittle hair or hair loss. Sunken fontanelles in infants. Lethargy, apathy and weakness. Weight loss of more than 40%. BMI below 16.

Question 352

Management: marasmus

Answer 352

1. Rehydration and stabilisation - keep warm 2. nutritional rehabilitation - slowly to prevent referring syndrome 3. Follow up and prevention

Question 353

What are the causes of kwashiorkor disease?

Answer 353

- diet of mostly carbohydrates - kwashiorkor = in ghana 'the sickness the baby gets when the new baby comes' > rapidly weaning the toddler off breastfeeding to feed the new baby - lack of dietary antioxidants - parasites and infectious diseases e.g. measles, malaria, HIV

Question 354

Presentation: Kwashiorkor

Answer 354

- oedema - ascites - hair loss - dermatitis - enlarged liver - depleted muscle mass but retained subcutaneous fat - dehydration - loss of appetite - stunted growth in children

Question 355

Management: kwashiorkor

Answer 355

1. treat/prevent hypoglycaemia 2. treat/prevent hypothermia 3. treat/prevent dehydration 4. correct electrolyte imbalances 5. treat/prevent infection 6. correct micronutrient deficiencies 7. start cautious feeding 8. achieve catch up growth 9. provide sensory stimulation and emotional support 10. prepare for follow-up after recovery

Question 356

Define: choledochal cysts

Answer 356

aka biliary cysts - congenital - swellings in your bile duct - usually found in young children but can be diagnosed in adults

Question 357

What are the 5 types of choledochal cysts?

Answer 357

type 1 - a cyst in the bile duct outside your liver - MC type 2 - a cyst that develops as a sac and comes off the side of the bile duct outside your liver type 3 - a cyst at the end of the bile duct, outside your liver, where it joins your intestines - choledochocele type 4 - cysts in the bile ducts inside and outside your liver - like type 1 they are caused by the tubes swelling up type 5 - Carol disease

Question 358

Presentation: choledochal cysts

Answer 358

- abdo pain - bloating - oedema - jaundice - pale coloured poo

Question 359

Investigations: choledochal cyst

Answer 359

- 20 week USS - blood test = LFTs - USS - ERCP

Question 360

Management: choledochal cysts

Answer 360

Surgery - key hole for cysts outside the liver - ERCP for type 3 cysts - surgery for cysts inside your liver

Question 361

What causes liver failure in children?

Answer 361

- viruses e.g. HSV, EBV, CMV, hepatitis - inherited metabolic disorders - toxins e.g. rat poison, insect killer, weed killer - medications e.g. erythromycin, caloric acid, acetaminophen - autoimmune hepatitis - low blood flow to the liver

Question 362

Presentation: liver failure

Answer 362

- fatigue - nausea or vomiting - loss of appetite - abdominal pain - dark urine - jaundice - itching - ascites - eventual brain problems

Question 363

Investigations: liver failure

Answer 363

- high bilirubin levels > jaundice - high liver enzyme levels - problems with blood clotting - signs of encephalopathy

Question 364

Management: liver failure

Answer 364

- watch + wait = often viruses will resolve - medicines = antibiotics, diuretics, BP drugs - liver transplant

Question 365

Define: Colic

Answer 365

Frequent, prolonged and intense crying in a healthy infant - peak at 6 weeks old till 3/4 months

Question 366

Presentation: colic

Answer 366

car for more than 3hrs a day, 3 days a week for at least 1 week but are otherwise healthy - intense crying for no reason - extreme fussiness - predictable timing e.g. more in evening - facial discolouring - body tension

Question 367

What are the causes of colic?

Answer 367

Unknown - digestive system not fully developed - imbalance of healthy bacteria in the digestive tract - food allergies or intolerances - overfeeding, underfeeding - early form of childhood migraine

Question 368

What causes the need for neonatal resuscitation?

Answer 368

- labour causes hypoxia - babies have a large surface area to weight ratio and get cold easily - born wet so lose heat rapidly - born through meconium so may this in their mouth or airway

Question 369

What are the principles of neonatal resuscitation?

Answer 369

1. warm the baby - dry - under heat lamp - < 28 weeks place in plastic bag 2. calculate APGAR score - done at 1, 5 and 10 mins 3. Stimulate breathing - stimulate to prompt breathing 4. Inflation breaths - 2 cycles of 5 inflation breaths - then 30s of ventilation breaths 5. chest compressions

Question 370

What does the APGAR score measure?

Answer 370

out of 10 lowest score = 0 Appearance Pulse Grimmace (response to stimulation) Activity Respiration

Question 371

What are the risk factors for neonatal sepsis?

Answer 371

Vaginal GBS colonisation GBS sepsis in a previous baby Maternal sepsis, chorioamnionitis or fever > 38ºC Prematurity (less than 37 weeks) Early (premature) rupture of membrane Prolonged rupture of membranes (PROM)

Question 372

What are the red flags for neonatal?

Answer 372

- Confirmed or suspected sepsis in the mother - Signs of shock - Seizures - Term baby needing mechanical ventilation - Respiratory distress starting more than 4 hours after birth - Presumed sepsis in another baby in a multiple pregnancy

Question 373

Management: neonatal sepsis

Answer 373

- 1 RF or clinical features = monitor for at least 12 hrs - 2 or more RF or clinical features = start antibiotics - start antibiotics = if any red flag - antibiotics would be given within 1 hr of decision to start them - blood cultures taken before antibiotics given - perform a LP if infection strongly suspected or meningitis antibiotics = benzylpenicillin and gentamycin

Question 374

Define: hypoxic ischaemic encephalopathy

Answer 374

occurs due to hypoxia during birth - lack of oxygen with restriction of blood flow to the brain causing malfunctioning of the brain

Question 375

What are the causes of HIE?

Answer 375

Anything that leads to asphyxia (deprivation of oxygen) to the brain - Maternal shock - Intrapartum haemorrhage - Prolapsed cord = causing compression of the cord during birth - Nuchal cord = where the cord is wrapped around the neck of the baby

Question 376

What are 3 grades for hypoxic ischaemic encephalopathy?

Answer 376

Mild - poor feeding = irritable - resolves within 24 hrs - normal prognosis moderate - poor feeding = lethargic, hypotonic, seizures - weeks to resolve - up to 40% develop cerebral palsy Severe - reduced consciousness, apnoeas, flaccid, reduced/absent reflexes - up to 50% mortality - up to 90% develop cerebral palsy

Question 377

Management: HIE

Answer 377

- supportive care - neonatal resuscitation and optimal ventilation - therapeutic hypothermia = helps protect brain from hypoxic injury

Question 378

Define: meconium aspiration syndrome

Answer 378

varying spectrum of respiratory distress in neonates born through meconium stained liquor - meconium is in feats intestinal tract and if released into amniotic fluid can enter respiratory tract and stimulate an inflammatory response through cytokine release

Question 379

What are the clinical features of meconium aspiration?

Answer 379

- meconium stained liquor - respiratory distress at or shortly following birth - increased oxygen requirements - respiratory distress

Question 380

Investigations: meconium aspiration

Answer 380

bloods = FBC, CRP and blood cultures (raised WBCs,) CXR = hyperinflation, patchy opacification and consolidation

Question 381

Management: meconium aspiration syndrome

Answer 381

Preventative - prevent intrapartum foetal hypoxia and postdates gestation post delivery - oropharyngeal suctioning - admitted to neonatal unit - supportive = oxygen therapy, CPAP - antibiotics started while waiting for blood cultures

Question 382

Define: bronchopulmonary dysplasia

Answer 382

a form of chronic lung disease that affects newborns, most often premature babies that need oxygen therapy - the lungs and airways are damaged causing tissue destruction in the tiny air sacs of the lung

Question 383

What is the cause of bronchopulmonary dysplasia?

Answer 383

damaged to lungs from: - mechanical ventilation - long term use of oxygen (especially susceptible in premature babies lungs)

Question 384

Presentation: bronchopulmonary dysplasia

Answer 384

- Rapid breathing - Labored breathing (drawing in of the lower chest while breathing in) - Wheezing (a soft whistling sound as the baby breathes out) - The need for continued oxygen therapy after the gestational age of 36 weeks - Difficulty feeding - Repeated lung infections that may require hospitalization

Question 385

Management: bronchopulmonary dysplasia

Answer 385

no cure - diuretics = help decrease fluid in and around alveoli - bronchodilators = relax the muscles around the air passages - cortiosteroids = reduce inflammation > decreasing amount of mucus - viral immunisation = increased risk for resp tract infections - cardiac medications = relax blood vessels to reduce strain on heart symptoms usually raced by 2/3 yrs old - should still have regular checkups to prevent future lung problems

Question 386

Define: gastroschisis

Answer 386

abdominal wall defect that occurs when the intestines develop outside the abdomen and are open to the air when the child is born - don't know the cause

Question 387

Management: gastroschisis

Answer 387

many are born prematurely at 35 weeks or induced at 37 weeks 1. wrapped exposed intestines in a types of lying film to reduced the amount of fluid and body heat loss, and protect from further damage 2. surgery = put back in abdomen (sometimes done in stages ) and sewn up 3. ICU = mesh sac over intestines and use gravity to move intestines back inside abdomen

Question 388

Define: oesophageal atresia

Answer 388

the upper part of the oesophagus doesn't connect with the lower oesophagus and stomach - associated with trachea-oesophageal fistula = air passes from trachea into stomach and stomach acid to pass into the lungs

Question 389

What are the risk factors for oesophageal atresia?

Answer 389

- polyhydraminos - more common in babies who have problems with development of kidneys, heart and spine too -

Question 390

investigations: oesophageal atresia

Answer 390

Check ability to swallow pass thin feeding tube down to see if it reaches stomach CXR

Question 391

Management: oesophageal atresia

Answer 391

surgery - closes off fistula - sew together upper and lower oesophagus after surgery - antibiotics - ventilator -tube to drain fluid in chest - oxygen - pain medication

Question 392

Define: bowel atresia

Answer 392

when the jejunum or ileum are not completely connected or blocked

Question 393

Investigations: small bowel atresia

Answer 393

USS - polyhydaminos Not passing meconium when born X-ray = shows blockage Contrast CT enema

Question 394

Management: small bowel atresia

Answer 394

surgery - repairs the atresia

Question 395

What are the risk factors for neonatal hypoglycaemia?

Answer 395

- intrauterine growth restriction in term infants - premature babies - maternal diabetes - macrocosmic babies - infants of mothers taking beta blockers - hypothermia - hypoxia

Question 396

Presentation: neonatal hypoglycaemia

Answer 396

first 48 hours of life Hypotonia Lethargy (excessive sleepiness with or without abnormal tone) Poor feeding Hypothermia Apnoea Irritability Pallor Tachypnoea Tachycardia or bradycardia Seizures Abnormal feeding behaviour (not waking for feeds, not sucking effectively, appearing unsettled and demanding very frequent feeds especially after a period of feeding well)

Question 397

Management: neonatal hypoglycaemia

Answer 397

1. offer additional feed - observe breastfeed and ensure good attachment 2. administer dose of 40% buccal glucose 200mg/kg 3. check blood glucose 4. continue to establish breastfeeding 5. IV access - 2.5ml/kg glucose bolus IV 6. recheck blood glucose after 30mins 7. if below <2 give another bolus

Question 398

What are the 4 domains of child development?

Answer 398

1. Gross motor. 2. Fine motor and vision. 3. Speech, language and hearing. 4. Social interaction and self care skills.

Question 399

What are some developmental milestones for fine motor and vision?

Answer 399

4 months - Grasp an object Uses both hands reaches for things, and brings things to mouth** 12 months scribbles with a crayon, 3 years Tower of multiple cubes

Question 400

What are the developmental milestones for gross motor function? 3 months to 9 months

Answer 400

3m: lifts head on tummy. 6m: chest up with arm support, can sit unsupported. 8m: crawling. 9m: pulls to stand.

Question 401

What are the developmental milestones for gross motor function? 12 months to five years

Answer 401

12m: walking. 2 years: walking up stairs. 3 years: jumping. 4 years: hopping. 5 years: rides a bike.

Question 402

What are the developmental milestones for speech, language and hearing?

Answer 402

3m: laughs and squeals. 9m: can make sounds such as ‘dada’ and ‘mama’. 12m: can say one word. 2 years: can form short sentences and name body parts. 3 years: speech is mainly understandable. 4 years: knows colours and can count. 5 years: knows the meaning of words.

Question 403

Give some developmental milestones for social and self care, 6 weeks to 12 months

Answer 403

6 weeks Smiles spontaneously 6 months Finger feeds 9 months Waves bye – bye 12 months Uses spoon/fork

Question 404

Give some developmental milestones for social and self care 2 -4 years

Answer 404

2 years - can take some clothes off, play w toys, but not sharing, “parallel play” w others 3-4 years can learn to play, take turns, dress themselves mostly

Question 405

Give two examples of concerning child development with regards to gross motor function.

Answer 405

Not sitting by 12 months. Not walking by 18 months

Question 406

Give an example of concerning child development with regards to fine motor function.

Answer 406

Hand preference before 18 months.

Question 407

Give two speech and language examples that may suggest concerning child development.

Answer 407

1. Not smiling by 3 months - blindness? ASD? 2. No clear words by 18 months - ASD? Language problems?

Question 408

Give two examples of concerning child development with regards to social development.

Answer 408

1. No response to carers interactions by 8 weeks. 2. No interest in playing by 3 years.

Question 409

Give some examples of conditions that cause global development delay (child displaying slow development in all developmental domains, Gross motor, fine motor, speach and language, social skills)

Answer 409

Down’s syndrome Fragile X syndrome Fetal alcohol syndrome Rett syndrome Metabolic disorders

Question 410

Give some examples of conditions that cause gross motor delay

Answer 410

A delay that is specific to the gross motor domain may indicate underlying: Cerebral palsy Ataxia Myopathy Spina bifida Visual impairment

Question 411

Give some examples of conditions that cause fine motor delay

Answer 411

A delay that is specific to the fine motor domain may indicate underlying: Dyspraxia Cerebral palsy Muscular dystrophy Visual impairment Congenital ataxia (rare)

Question 412

Give some examples of conditions that cause language delay

Answer 412

Specific social circumstances, for example exposure to multiple languages or siblings that do all the talking Hearing impairment Learning disability Neglect Autism Cerebral palsy

Question 413

Give some examples of conditions that cause Personal and social delay

Answer 413

Emotional and social neglect Parenting issues Autism

Question 414

What is are neuroblastoma tumours?

Answer 414

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

Question 415

Give 2 signs of a neuroblastoma tumour.

Answer 415

1. Abdominal mass - often crosses the midline and envelopes major vessels and lymph nodes. 2. Symptoms of metastases e.g. bone pain, weight loss, pallor, limp, hepatomegaly.

Question 416

Describe the treatment for a neuroblastoma malignancy

Answer 416

Surgery - localised primaries can often be cured with surgery alone. Chemotherapy - can be given before and/or after surgery to control disease. Radiotherapy for high risk groups.

Question 417

What is a hepatoblastoma?

Answer 417

Hepatoblastoma is a malignant liver cancer occurring in infants and children and composed of tissue resembling fetal liver cells, mature liver cells, or bile duct cells

Question 418

What is a discoid meniscus?

Answer 418

congenital anomaly of the knee found in 3% of the population, typically affecting lateral meniscus discoid meniscus is thickened and has a fuller crescent shape and does not taper as much towards the center of the joint and is shaped like a disc.

Question 419

What can having a discoid meniscus lead to ? What is the best way to diagnose it?

Answer 419

- thickness of meniscus - diminished blood supply - weak capsular attachment --> more prone to tears x- ray = rules out bony pathology MRI = tp visualise discoid meniscus

Question 420

Outline what happens in scoliosis

Answer 420

Condition that causes a lateral curvature of the spine This leads to bending of the spine and poor posture, even lead to cardiorespiratory failure

Question 421

What are some symptoms of scoliosis?

Answer 421

Symptoms: – Pain in the shoulders and back – Restrictive lung disease – Limited mobility – Uneven hips, arms or leg lengths – Constipation due to tightening up of the bowel contents

Question 422

What is the management of scolisis?

Answer 422

– If minor –>can self-resolve – If severe –> may require bracing and physiotherapy, else surgery is needed

Question 423

What is Torticollis? What causes it?

Answer 423

This is known as wry neck and is defined by an abnormal, asymmetrical head position – It is due to excessive contraction of the sternocleidomastoid which pulls the ear to ipsilateral shoulder and the face to the other side. Congenital torticollis: – Birth trauma – Also can be due to a sternocleidomastoid tumour Acquired: – Due to muscle spasm (most common) – Also due to ENT infections, antipsychotics

Question 424

What is the treatment for torticollis

Answer 424

– Physical therapy like stretching helps and it usually self-resolves within a few days – If unresolving, surgery may be required

Question 425

What are the common neonatal infections that make up the TORCH acryonym? When is transmission most likely?

Answer 425

TORCH infection – - Toxoplasmosis, - Other (syphilis, varicella-zoster, parvovirus B19), - Rubella, - Cytomegalovirus, - Herpes As a rule, the likelihood of transmission to a fetus is greatest when primary maternal infection occurs.

Question 426

What are some features/consequences of toxoplasmosis infection?

Answer 426

There is a classic triad of features in congenital toxoplasmosis: Hydrocephalus Intracranial Calcifications (calcium deposits in the brain) Chorioretinitis (inflammation of the choroid and retina of eye)

Question 427

What are some features of Congenital cytomegalovirus?

Answer 427

The features of congenital CMV are: Fetal growth restriction Microcephaly Hearing loss Vision loss Learning disability Seizures

Question 428

What can a listeria infection do to a foetus? What are some key signs of it?

Answer 428

- bacteremia = mild, influenza like illness in mother and pass to foetus via placenta - maternal infection may cause spontanous abortion, preterm delivery, or fetal/neonatal sepsis signs - meconium staining of amniotic fluid - widespread rash - septicaemia - pneumonia - meningitis

Question 429

Treatment: listeria

Answer 429

IV Amoxicillin and Gentamicin.

Question 430

What is the most common cause of Encephalitis, in a) Children b) Neonates

Answer 430

In children the most common cause is herpes simple type 1 (HSV-1) from cold sores. Neonates it is herpes simplex type 2 (HSV-2) from genital herpes, contracted during birth.

Question 431

What are some investigations for Encephalitis?

Answer 431

- Lumbar puncture = sending cerebrospinal fluid for viral PCR testing - CT scan = if a lumbar puncture is contraindicated - MRI scan = after the lumbar puncture to visualise the brain in detail - EEG recording = can be helpful in mild or ambiguous symptoms but is not always routinely required - Swabs of other areas can help establish the causative organism, such as throat and vesicle swabs - HIV testing = is recommended in all patients with encephalitis Contraindications to a lumbar puncture include a GCS below 9, haemodynamically unstable, active seizures or post-ictal.

Question 432

What is the management for encephalitis?

Answer 432

Aciclovir = HSV and VZV Ganciclovir = cytomegalovirus Repeat Lumbar puncture to check treatment is successful

Question 433

What is cleft lip? What is cleft palate, and what can it lead to?

Answer 433

Cleft lip is a congenital condition where there is a split or open section of the upper lip Cleft palate is where a defect exists in the hard or soft palate at the roof of the mouth. - This leaves an opening between the mouth and the nasal cavity. - Cleft lip and cleft palate can occur together or on their own.

Question 434

What are some complications of cleft lip/palate?

Answer 434

- problems with: - feeding - swallowing - speech - affect bonding between mum and child - social outcast

Question 435

Managment: cleft lip/palate

Answer 435

- ensure baby can eat using specially shaped bottles and teats - surgery = cleft lip performed at 3 months = cleft palate done at 6-12 months