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3A REVISION > PAEDIATRICS > Flashcards

PAEDIATRICS Flashcards

1
Q

Describe the haemoglobin concentration at birth and over the first few weeks of life

A

At birth - fetal Hb in term infants is high to compensate for low oxygen concentration in the foetus
Levels decline over first 6 months as adult Hb synthesis is activated and HbF deactivated

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2
Q

What is the difference between fetal and adult haemoglobin?

A

HbF has a gamma subunit instead of beta which decreases binding to 2,3-BPG, a substance that decreases affinity of haemoglobin for oxygen
Therefore HbF has greater affinity for oxygen

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3
Q

Define anaemia

A

Haemoglobin level below the normal range

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4
Q

Give 3 broad causes of anaemia

A

Decreased RBC production
Increased RBC haemolysis
Blood loss

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5
Q

What is anaemia of prematurity?

A

Anaemia affecting preterm infants with decreased RBCs - typically due to repeated blood sampling and reduced erythropoiesis with low EPO levels

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6
Q

What is ineffective erythropoiesis?

A

Premature death of RBCs due to dysfunctional progenitor cells. Normal reticulocytes but lack of mature RBCs

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7
Q

What are the 3 main causes of iron deficiency anaemia?

A

Inadequate intake - common in children as more is needed to accumulate increasing blood volume and to build stores
Malabsorption
Bleeding

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8
Q

What is a cause of inadequate intake of iron in children?

A

Delayed weaning onto solids as milk does not contain enough

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9
Q

Symptoms of iron deficiency anaemia (4)

A

Fatigue
Slow feeding
Pallor
Pica - eating non food

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10
Q

Signs/diagnosis of iron deficiency anaemia (3)

A

MICROCYTIC (small rbcs)
HYPOCHROMIC (pale rbcs)
Low serum ferritin

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11
Q

Management of iron deficiency anaemia (3)

A

Dietary advice
Oral iron supplements
Continue for 3 months after normal Hb level
If refractory, Ixs e.g. for coeliac, Meckel’s

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12
Q

3 types of microcytic anaemia

A

Iron deficiency
Thalassaemia
Anaemia of chronic disease

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13
Q

3 types of normocytic anaemia

A

Blood loss
Aplastic anaemia
Sickle cell anaemia
Haemolytic anaemia i.e. G6PD deficiency

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14
Q

3 types of macrocytic anaemia

A 
Pernicious anaemia (autoimmune)
B12 deficiency 
Folate deficiency
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15
Q

What is sickle cell disease?

A

Collective name for HbS inherited haemoglobinopathies

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16
Q

Cause of sickle cell disease

A

Genetic, point mutation in codon 6 of beta globin gene cusing amino acid from glutamine to valine, forming HbS instead of HbA

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17
Q

4 types of sickle cell disease

A

Sickle cell anaemia (HbSS)
HbSC disease
Sickle trait (one allele)
Sickle beta thalassaemia

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18
Q

Effect of HbS on blood cells

A

Causes irreversibly sickled red blood cells with reduced lifespan
Can get trapped, causing vaso-occlusion and ischaemia

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19
Q

What exacerbates sickle cell anaemia? (3)

A

Hypoxaemia, dehydration, cold

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20
Q

Symptoms of sickle cell anaemia (10)

A 
Anaemia
Jaundice
Susceptibility to infection
Vaso-occlusive crises
Sequestration crises
Splenomegaly
Risk of stroke
Cardiomegaly/heart failure
Renal dysfunction
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21
Q

What are vaso-occlusive crises?

A

Episodes of pain caused by ischaemia of tissue usually preceded by infection - symptoms depend on tissue affected
Can include priapism, dactylitis, abdominal pain, acute chest syndrome

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22
Q

What ethnic groups is sickle cell disease more common in and why?

A

Africa, Hispanic, Mediterranean origin

Sickle shape of RBCs afford some protection against malaria

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23
Q

What is acute chest syndrome?

A

Vaso-occlusive crisis caused by infarction of the lung parenchyma, can be fatal

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24
Q

How does acute chest syndrome present? (6)

A 
Fever
Cough
Pain
Sputum
SoB
Low oxygen
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25
Q

Treatment of acute chest syndrome

A 
Hydroxyurea can prevent
Broad spectrum antibiotics
Pain control
Blood transfusion
Exchange transfusion
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26
Q

What is a sequestration crisis?

A

Acute painful enlargement of the spleen, caused by intrasplenic trapping of RBCs and resulting in fall in Hb level

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27
Q

Complications of sequestration crisis

A

Shock

Circulatory failure and death

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28
Q

How is sequestration crisis treated

A

Supportive

Blood transfusion

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29
Q

General management of sickle cell anaemia (5)

A 
Ensure full immunisation
Daily penicillin in childhood
Folic acid
Avoid crisis triggers
Possible hydroxyurea
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30
Q

Management of severe, refractory sickle cell anaemia

A

HLA identical bone marrow transplant

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31
Q

How would haemophilia present in the neonate?

A

Intracranial haemorrhage

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32
Q

How would haemophilias present in adolescence?

A

Menorrhagia

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33
Q

How are haemophilia A and B inherited?

A

X linked recessive - in males

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34
Q

Symptoms of haemophilia (5)

A 
Bleeding i.e. unexplained from cuts
Large/deep bruises
Blood in urine/stool
Nosebleeds
Severe - bleeding into joints and muscles
40% present neonatally
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35
Q

Management of haemophilia A

A

Recombinant factor VIII for acute bleeds or prophylaxis if severe
Desmopressin if mild

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36
Q

Management of haemophilia B

A

Recombinant factor IX for acute bleeds or prophylaxis if severe

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37
Q

What is von Willebrands disease?

A

Deficiency of von Willebrand’s factor, causing defective platelet plugs

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38
Q

Symptoms of von Willebrand’s disease

A

Bruising
Prolonged bleeding
Nosebleeds
Menorrhagia

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39
Q

Management of von Willebrand’s disease

A

Mild with desmopression

Severe with plasma derived factor VIII concentrate

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40
Q

What is immune thrombocytopaenic purpura?

A

Caused by destruction of circulating platelts by anti-platelet IgG autoantibodies

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41
Q

When do most cases of immune thrombocytopaenic purpura present

A

Between 2-10 years, often 1-2 weeks after viral infection

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42
Q

Symptoms of immune thrombocytopaenic purpura (3)

A

Petechiae
Purpura
Superficial bruising

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43
Q

What is the most common form of immune thrombocytopaenic purpura

A

80% is acute, benign and self limiting, remitting spontaenously in 6-8 weeks

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44
Q

Treatment of immune thrombocytopaenic purpura

A

Most do not need treatment
Treat major bleeds with oral prednisolone, platelet transfusions
Splenectomy if chronic/severe
Anti-D, rituximab to reduce splenectomy rate

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45
Q

What are the 6 things to consider in the febrile child

A 
Temperature and assessment
Age - viral rare < 3 months
Risk factors - travel, family ill
Red flags - high fever, mottled/blue, low GCS
Rash
Focus of infection - URTI
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46
Q

What must be considered in the febrile child if there is no focus of infection

A

Serious bacterial infection i.e. UTI, septicaemia

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47
Q

How do viral infections in the child commonly present

A

Fever and a rash

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48
Q

List the herpes viruses

A 
Herpes simplex virus 1 and 2
Varicella zoster virus
Epstein-Barr virus
Human cytomegalovirus
Human herpesvirus 6-8
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49
Q

What is HSV1 associated with

A

Lip and skin lesions

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50
Q

What is HSV2 associated with

A

Genital lesions

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51
Q

How does primary HSV1 present in children (3)

A 
Gingivostomatitis (fever, painful ulcers in mouth)
Eczema herpeticum (lesions on eczematous skin)
Herpetic whitlow (lesion on finger)
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52
Q

What is the natural progession of varicella zoster infection

A

High temperature
2-500 lesions start on head and trunk, progress to peripheries
Persists for around a week
Normally self limiting

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53
Q

3 serious childhood complications of VZV

A

Serious bacterial infection
Encephalitis
Purpura fulminans (acute thrombotic disorder - blood spots and discolourisation can lead to DIC, skin necrosis)

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54
Q

Action if a pregnant/immunocompromised adult comes into contact with VZV

A

Give human varicella zoster immunoglobulin

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55
Q

What is shingles?

A

Herpes zoster

Reactivation of latent varicella zoster, uncommon in children

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56
Q

Symptoms of shingles (3)

A

Vesicular eruption in the dermatomal distribution of sensory nerves
Headache, fever, paraesthesia
Disseminated can cause hepatitis or encephalitis

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57
Q

What does EBV cause?

A

Causes glandular fever - infectious mononucleosis persists for 1-3 months
Involved in pathogenesis of lymphoma

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58
Q

Symptoms of EBV (5)

A 
Fever
Malaise
Tonsillopharyngitis
Lymphadenopathy
Splenomegaly
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59
Q

How is EBV diagnosed

A

Large T cells on blood film
Positive monospot test
IgM/IgG anti EBV antibodies

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60
Q

How is cytomegalovirus transmitted

A

Saliva, genital secretions, breast milk

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61
Q

What is a serious condition CMV can cause

A

Normally mild

Can cause mononucleosis syndrome - pharyngitis, lymphadenopathy

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62
Q

Transmission of HHV6-7

A

Oral transmission

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63
Q

What does HHV6-7 cause

A

Roseola infantum - high fever, malaise, generalised macular rash

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64
Q

What does parvovirus B19 cause

A

Erythema infectiosum - slapped cheek syndrome

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65
Q

Symptoms of parvovirus B19 (5)

A

Fever, malaise, headache, myalgia

1 week later facial rash progressing to trunk

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66
Q

How does measles spread

A

Highly infectious droplet spread

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67
Q

Symptoms of measles (5)

A 
Fever
Rash
Koplik's spots - white on buccal mucosa
Cough and cold
Conjunctivitis
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68
Q

3 serious complications of measles

A

Encephalitis
Secondary bacterial infection
Subacute sclerosing panencephalitis

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69
Q

How does mumps spread

A

Droplet infection

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70
Q

Symptoms of mumps (4)

A

Fever
Malaise
Parotitis
Transient hearing loss

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71
Q

When is rubella most harmful to develop

A

Mild in childhood

Can cause serious harm if infects a foetus

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72
Q

What is Kawasaki disease?

A

Systemic vasculitis - unknown cause, not contagious

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73
Q

Symptoms of Kawasaki disease (8)

A 
Fever
Red mucous membranes
Non purulent conjunctivitis
Cervical lymphadenopathy
Strawberry tongue
Rash
Red oedematous palms/soles
Peeling of digits
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74
Q

Complications of Kawasaki disease

A

Coronary artery aneurysm

Death

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75
Q

Treatment of Kawasaki disease

A

IV immunoglobulins

Aspirin

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76
Q

General treatment for viral disease in children

A

Supportive

If severe, aciclovir

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77
Q

What are some common malignancies in children? (6)

A 
ALL
Intracranial neoplasm
Lymphoma
Neuroblastoma
Wilms tumour
Retinoblastoma
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78
Q

What is the most common leukaemia in children?

A

ALL

acute lymphoblastic leukaemia

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79
Q

When does ALL present?

A

Mostly between age 2-5

Insidious over several weeks

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80
Q

Diagnosis of ALL (4)

A

Decreased Hb
Decreased platelets
Circulating blast cells
Bone marrow biopsy

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81
Q

What chromosome is associated with poor prognosis of ALL?

A

Philadelphia chromosome

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82
Q

Symptoms of ALL (8)

A 
Malaise
Anorexia
Pallor/lethargy from anaemia
Infection (neutropaenia)
Easy bruising/bleeding
Bone pain
Hepatosplenomegaly
Lymphadenopathy
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83
Q

Treatment of ALL (4)

A

Chemotherapy - induction, intensification, maintenance for 2-3 years (vincristine, dexamethasone, methotrexate common)
CNS protection
Also treat anaemia, give platelet transfusion, treat infection
Allopurinol for renal protection

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84
Q

Treatment of relapsed ALL (3)

A

High dose chemotherapy
Total body irradiation
Bone marrow transplant

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85
Q

What is lymphoma and what are the types?

A

Malignancies of immune system cells - non Hodgkin more common in childhood, Hodgkin in adolescence and rarer

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86
Q

Congenital disease associated with ALL

A

Down’s syndrome

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87
Q

Usual features of non Hodgkin lymphoma (5)

A 
T or B cell malignancy
Localised lymph node swelling
B symptoms - night sweats, weight loss, fever, cough
Abdominal mass
Bone marrow infiltration
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88
Q

Diagnosis of non Hodgkin lymphoma (4)

A

Lymph node biopsy
CT/MRI of nodes
Bone marrow biopsy
CSF sample

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89
Q

Treatment of non Hodgkin lymphoma

A

Multi agent chemotherapy

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90
Q

Symptoms of Hodgkin lymphoma

A

Painless lymphadenopathy in the neck - may obstruct airway

B symptoms less common

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91
Q

Diagnosis of Hodgkin lymphoma (3)

A

Lymph node biopsy
Bone marrow biopsy
CT/MRI of nodes

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92
Q

Management of Hodgkin lymphoma

A

Multi agent chemotherapy

Possible radiotherapy

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93
Q

What are the survival rates for ALL, NHL and HL

A

ALL 90%

Lymphomas ~80%

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94
Q

What is osteomyelitis?

A

Infection of the metaphysis of the long bones, may spread to the joint causing septic arthritis

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95
Q

Most common cause of osteomyelitis

A

Staphylococcus aureus, haematogenous spread

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96
Q

How does osteomyelitis present (5)

A 
Painful, immobile limb
Swelling and tenderness
Red and warm
Febrile child 
Joint effusion
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97
Q

Diagnosis of osteomyelitis

A

Positive blood cultures
High WBC, CRP, ESR
X ray
MRI

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98
Q

What is seen on X ray of osteomyelitis

A

Initial soft tissue swelling, then new subperiosteal bone

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99
Q

How is osteomyelitis treated

A

Antibiotics for several weeks to prevent necrosis, chronic infection, deformity
Surgical drainage if needed

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100
Q

What is septic arthritis?

A

Inflammation of a joint caused by bacterial infection (staph a), most common in knees and hips

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101
Q

Symptoms of septic arthritis

A

Acute onset pain, redness, swelling, heat
High fever
Pain worse on movement

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102
Q

Diagnosis of septic arthritis (3)

A

High WCC, ESR
USS shows fluid in joint
X ray shows widened joint space

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103
Q

How is septic arthritis treated?

A

Joint aspiration

Prolonged antibiotics - IV cefuroxime 2w then 4w oral

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104
Q

What is transient synovitis?

A

Most common cause of acute hip pain in children 3-10

Transient inflammation of the hip joint, possibly due to a recent infection

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105
Q

How does transient synovitis present?

A

Acute limp
No/mild fever
Pain on movement - irritable hip

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106
Q

How is transient synovitis treated?

A

Supportive care
Pain relief (NSAIDs)
Non weightbearing

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107
Q

What is Perthes disease?

A

Avascular necrosis of the femoral epiphysis (femoral head)

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108
Q

What group does Perthes disease commonly affect?

A

Boys aged 5-10

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109
Q

What causes Perthes disease?

A

Can follow transient synovitis or other inflammation, trauma etc.
Interruption of the blood supply followed by slow revascularisation and reossification - can cause permanent deformity.

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110
Q

What is seen on X ray in Perthes disease?

A

Flattening of the femoral head, fragmentation

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111
Q

What is JIA?

A

Juvenile idiopathic arthritis
Most common chronic inflammatory joint disease in children - persistent joint swelling for >6 weeks in the absence of any other cause
Autoimmune.

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112
Q

What are the 5 types of JIA?

A

Oligoarthritis <4 joints, not symmetrical, large joints
Polyarthritis >4 joints, symmetrical, marked finger involvement
Systemic with fever, ‘salmon’ pink rash, myalgia
Psoriatic with psoriasis, joint problems may precede skin
Enthesitis with tendon/ligament swelling, lower limb, back pain

113
Q

What is enthesitic JIA linked with?

A

Ankylosing spondylitis

IBD

114
Q

Symptoms of JIA?

A

Morning stiffness/stiffness after rest
Pain
Limp
Joint swelling/inflammation

115
Q

Complications of JIA? (4)

A

Joint contracture/fibrosis
Chronic anterior uveitis (inflammation of the uvea in the eye - often asymptomatic but can cause glaucoma, cataracts, blindness)
Growth retardation
Osteoporosis

116
Q

Treatment of JIA (7)

A 
NSAIDs and analgesia
Joint injections
Methotrexate
Systemic corticosteroids
Cytokine modulators/immunotherapy
Joint replacement
Therapy
117
Q

What is osteogenesis imperfecta?

A

Disorders of collagen metabolism causing bone fragility, bowing and frequent fractures. Joint laxity.

118
Q

What are the types of osteogenesis imperfecta?

A

I - most common, mildest
II - lethal
III - progressive
IV - moderate

119
Q

What are the features of type I osteogenesis imperfecta? (5)

A

Autosomal dominant
Fragility, some fractures during childhood
Blue sclerae
Hearing loss
Treat with bisphosphonates, splint fractures

120
Q

What are the features of type II osteogenesis imperfecta?

A

Autosomal dominant or new mutation
Severe lethal form
Multiple fractures before birth, many stillborn or die in first year

121
Q

What is the commonest cause of arthritis in children?

A

Reactive arthritis

122
Q

How does reactive arthritis present?

A

Transient joint swelling in ankles/knees

Follows extra-articular infection

123
Q

What bacteria cause reactive arthritis in children?

A

Enteric - salmonella, campylobacter, shigella

124
Q

How is reactive arthritis treated?

A

NSAIDs

125
Q

5 symptoms of growing pains in children

A 
Lower limbs
At night
3-12 year olds
Never on waking
Symmetrical
126
Q

What is genu varum?

A

Bow legs - broad gait, due to rickets

127
Q

What is genu valgum?

A

Knock-knees

128
Q

What is pes planus?

A

Flat feet

129
Q

What is talipes equinovarus?

A

Club foot - inverted and supinated foot

Positional talipes occurs due to intrauterine compression and can be corrected by compression/exercises

130
Q

What is pes cavus?

A

High arches

131
Q

When are hips checked in babies?

A

Neonatal check and at 8 weeks. Extra thigh crease!

132
Q

Risk factor for developmental dysplasia of the hip

A

Female
Breech
Multiple birth
Oligohydramnios

133
Q

How is developmental dysplasia of the hip managed

A

Splint/harness to keep hip flexed and abducted - Pavlik harness

134
Q

What is cerebral palsy?

A

Abnormality of movement and posture causing activity limitation attributed to non-progressive disturbances that occurred in the developing brain. Lesion is non-progressive but manifestations emerge over time as brain matures abnormally

135
Q

General symptoms of cerebral palsy

A

Motor problems often accompanied by cognitive, communication, perception, sensation, behaviour and seizure problems, with possible MSK issues

136
Q

What are the causes of cerebral palsy?

A

80% antenatal due to vascular occlusion, neuronal migration disorder, structural maldevelopment
10% due to hypoxic-ischaemic injury during birth
10% postnatal - trauma, infection, non accidental injury

137
Q

Give 6 early features of cerebral palsy

A 
Abnormal limb/trunk posture and tone
Delayed motor milestones
Feeding difficulties
Abnormal gait
Asymmetric function
Persistent primitive reflexes
138
Q

What are the 3 main subtypes of cerebral palsy? Investigations?

A

Spastic - 90%
Dyskinetic - 6%
Ataxic - 4%
May be mixed

development exam, clinical, possibly MRI

139
Q

Features of spastic cerebral palsy (3)

A 
Damage to UMNs
Increased tone! and brisk reflexes
Babinski sign
Seizures
Can be hemiplegic, quadriplegic or diplegic
140
Q

Features of dyskinetic cerebral palsy (3)

A

Involuntary movements
Variable tone
Primitive reflexes dominate

141
Q

Features of ataxic cerebral palsy (3)

A

Mostly genetic
Symmetrical hypotonia
Incoordinate movements

142
Q

Management of cerebral palsy (3)

A

Symptomatic i.e. improving gait and walking, benzodiazepines, anti-epileptics, analgesia
Botulinum toxin for focal/segmental spasticity in upper or lower limb
Multidisciplinary care i.e. physiotherapy, occupational therapy, speech and language

143
Q

What are the main 5 types of brain tumour in children

A

Astrocytoma 40% - varying from benign to very malignant glioblastoma multiforme
Medulloblastoma 20% - may have spinal mets
Ependymoma
Craniopharyngioma

144
Q

What are symptoms of brain tumours due to?

A

Raised intracranial pressure

145
Q

What are the symptoms of brain tumours in children? (3)

A

Headache worse in the morning
Vomiting on waking
Behaviour/personality change
Visual disturbance/papilloedema

146
Q

What are 3 signs of a brain tumour in an infant

A

Tense fontanelle
Increased head circumference
Developmental delay

147
Q

Rarer symptoms of brain tumours depending on location (4)

A

Hemiplegia if cortex
Pituitary failure if midline
Coordination problems if cerebellar
Cranial nerve defects if brainstem

148
Q

Best investigation for brain tumour?

A

Head MRI

149
Q

Treatment for brain tumours?

A

Surgery to treat hydrocephalus, get a biopsy, resect tumour - some are inoperable due to site
Possible radio/chemotherapy depending on type

150
Q

What is meningitis?

A

Inflammation of the meninges covering the brain, confirmed by inflammatory cells in the CSF

151
Q

Types of meningitis

A

Viral - most common, mostly self limiting

Bacterial - over 80% are <16, 5-10% mortality and 10% serious impairment

152
Q

Damage in meningitis is because of what?

A

The host response to bacteria - cerebral oedema, increased ICP, decreased cerebral blood flow, cortical infarction

153
Q

What is the most common cause of meningitis in neonates

A

Group B strep
E coli
Listeria monocytogenes

154
Q

What is the most common cause of meningitis in children

A

Neisseria meningitidis
strep pneumoniae
haemophilia influenzae

155
Q

Symptoms of meningitis (9)

A 
Fever
Headache
Photophobia
Lethargy
Vomiting
Irritability
Decreased consciousness
Seizures
Drowsiness
156
Q

Signs of meningitis (7)

A 
Fever
Rash (purpuric=meningococcal disease)
Neck stiffness
Bulging fontanelle in infants
Arched back
Brudzinski/Kernig signs
Signs of shock
157
Q

Investigations of meningitis (4)

A

Lumbar puncture and CSF analysis if no raised ICP
Blood cultures
Urine sample
Throat swab

158
Q

Treatment of meningitis

A

Cefotaxine or ceftriaxone
Dexamethasone
Prophylaxis for contacts with rifampicin

159
Q

Complications of meningitis

A 
Hearing loss
Cerebral infarction
Subdural effusion
Hydrocephalus
Abscess
160
Q

What is meningococcal infection?

A

Septicaemia usually accompanied by non-blanching purpuric rash which spreads across whole body

161
Q

Treatment of meningococcal infection

A

IM benzylpenicillin then admitted

162
Q

What is epilepsy?

A

Chronic neurological disorder characterised by recurrent unprovoked seizures, consisting of transient symptoms associated with excessive/abnormal activity

163
Q

What is the cause of epilepsy? (6)

A 
Mostly idiopathic
Cerebral malformation 
Vascular occlusion
Infection
Tumour
Trauma
164
Q

What are focal seizures?

A

Arise from only one hemisphere of the brain and manifestations depend on what lobe is affected (frontal/temporal/parietal/occipital)

165
Q

What are generalised seizures?

A

Arise from both hemispheres, produce a loss of consciousness (absence, tonic clonic, tonic, atonic, myoclonic)

166
Q

Investigations for epilepsy? (4)

A

EEG
MRI/CT
Functional scans
?metabolic/genetic studies

167
Q

Management of first seizure

A

Education and lifestyle advice -driving, alcohol, pregnancy

No treatment after 1 unprovoked seizure

168
Q

Treatment of established epilepsy

A

Focal - carbamazepine, lamotrigine
Generalised - valproate, lamotrigine
Possible surgery

169
Q

What is childhood absence epilepsy? (4)

A

Common between 4-12
Loss of awareness <30s, may be associated with repetitive finger movements/lip smacking automatisms
Can be induced by hyperventilation

170
Q

What is a long term complication of childhood absence seizures?

A

5-10% may develop adult tonic-clonic seizures

171
Q

What is juvenile myoclonic epilepsy? (4)

A

In adolescence
Myoclonic seizures mostly after waking
Also tonic-clonic and absence concurrently in 1/3-2/3
Lifelong prognosis

172
Q

Treatment for childhood absence seizures and JME?

A

Sodium valproate

Lamotrigine

173
Q

How may visual impairment present in infancy? (6)

A 
Loss of red reflex
White reflex
Not smiling by 6 weeks
Visual inattention
Nystagmus
Squint
174
Q

What is squint?

A

Misalignment of visual axes, often intermittent

175
Q

What is squint usually caused by?

A

Failure to develop binocular vision due to refractive errors

Can be caused by cataracts, retinoblastoma

176
Q

What are the types of squint?

A 
Concomitant squint (non paralytic, common) - usually due to refractive error
Paralytic squint (rare) - paralysis of motor nerves, can be due to a space occupying lesion - image head
177
Q

How is concomitant squint treated?

A

Glasses

Possible surgery

178
Q

How are squints detected?

A

Cover test

Light reflex test

179
Q

What is ambylopia?

A

Potentially permanent loss of visual acuity in an eye that hasn’t recieved a clear image

180
Q

What causes ambylopia?

A

Any interference in visual development - refractive errors, squint, ptosis, cataract

181
Q

How is ambylopia treated?

A

Glasses

Occlusion of the ‘good’ eye until vision in affected eye improves

182
Q

What can congenital hypothyroid cause?

A

Severe learning difficulties - one of the preventable causes

183
Q

What causes congenital hypothyroidism? (4)

A

TSH deficiency (rare)
Maldescent of the thyroid and athyrosis - most common in UK
Dyshormonogenesis - most common if consanguineous
Iodine deficiency (rare in UK, most common worldwide)

184
Q

When is congenital hypothyroidism usually picked up?

A

Screening

185
Q

What are the symptoms of congenital hypothyroidism? (8)

A 
Failure to thrive
Feeding problems
Prolonged jaundice
Constipation
Cold/dry skin
Large tongue
Goitre
Delayed development
186
Q

What are the symptoms of acquired (juvenile) hypothyroidism (Hashimoto’s thyroiditis)? (4)

A 
Similar to adults AND:
Short stature
Delayed puberty
Learning difficulty
Increased risk in Down's/Turner's
187
Q

What is the treatment of congenital/acquired hypothyroidism?

A

Oral thyroxine replacement lifelong

188
Q

What is the usual cause of hyperthyroidism?

A

Usually results from Graves disease secondary to production of thyroid stimulating immunoglobulins

189
Q

What are the symptoms of hyperthyroidism? (4)

A 
Similar to adults - eye signs less common
Rapid height growth
Advanced bone maturity
Behavioural problems
Learning difficulties
190
Q

How is hyperthyroidism treated? (6)

A

Carbimazole
Beta blockers if anxiety/tremor
Relapse common after treatment stopped - second course given
Or surgery - subtotal thyroidectomy
Radioiodine
Thyroxine for possible subsequent hypothyroidism

191
Q

What is Down’s syndrome?

A

Trisomy 21 - most common autosomal trisomy and cause of severe learning difficulties

192
Q

What are symptoms of Down’s syndrome? (4+)

A

Face - round face, flat nasal bridge, upslanted palpebral fissures, epicanthic folds, protruding tongue, small ears, flat occiput
Short neck
Single palmar crease
Sandal gap toe deformity

193
Q

Medical complications/signs of Down’s syndrome (4)

A

Hypotonia
Congenital heart defects
Duodenal atresia
Hirschprung’s disease

194
Q

Long term complications of Down’s syndrome (10)

A 
Learning difficulties
Delayed development
Small stature
Increased infection risk
Hearing problems
Visual problems
Increased risk of leukaemia/tumours
Increased hypothyroidism
Increased epilepsy
Increased Alzheimer's
195
Q

Diagnosis of Down’s syndrome (4)

A

Amniocentesis FISH - fluorescence in situ hybridisation
CVS - chorionic villous sampling of placenta
Increased nuchal translucency on USS
Clinical diagnosis

196
Q

What is the most common genetic cause of Down’s syndrome?

A

Meiotic non dysjunction, mostly related to maternal age

197
Q

What is the prevalence of atopic eczema in children and when is it resolved?

A

20%
50% resolved by 12
75% by 16

198
Q

What is eczema caused by?

A

Genetic deficiency of skin barrier function

199
Q

What is eczema associated with?

A

Family history of eczema, asthma, rhinitis

1/3 develop asthma

200
Q

How is eczema diagnosed?

A

Increased IgE
RAST tests if possible allergic cause
Exclude immune deficiency if severe/atypical/infectious

201
Q

Symptoms of eczema? (3)

A

Itching
Erythematous weeping crusted skin
Dry skin may be lichenified

202
Q

Where is eczema rash commonly in infants and older children?

A

Infants - face and trunk

Older children - flexor/friction surfaces

203
Q

What can trigger eczema exacerbations? (6)

A 
Staph/strep infection, herpes
Allergens
Irritants
Heat/humidity
Stress
Medications
204
Q

What may herpes cause in eczema?

A

Eczema herpeticum - blisters commonly on face and neck, give antivirals

205
Q

Management of eczema and exacerbations (5)

A

Avoid soaps, biological detergents, wool/nylon, food allergens
Give emollients
Topical corticosteroids in exacerbations, possible antibiotics
Tacrolimus - immunomodulator
Occlusive bandages if scratching

206
Q

What is sensorineural hearing loss?

A

Caused by a lesion in the cochlear or auditory nerve, usually present at birth and irreversible, can be of varying severity

207
Q

What causes sensorineural hearing loss? (6)

A 
Mostly genetic
Congenital infection
Prematurity
Ischaemia
Meningitis
Trauma
208
Q

What is classified as profound hearing loss?

A

> 95 decibels

209
Q

Management of sensorineural hearing loss?

A

Hearing aid amplification
cochlear implant
sign language (speech may be delayed)

210
Q

What may hearing loss be an underlying cause of?

A

Learning difficulties

Delayed speech

211
Q

What is conductive hearing loss?

A

Caused by abnormalities of the ear canal/middle ear, is intermittent and resolves i.e. after URTI

212
Q

Common causes of conductive hearing loss?

A

Most often from otitis media with effusion (glue ear)

Eustachian tube dysfunction in Down syndrome, cleft palate

213
Q

Which type of hearing loss is more common?

A

Conductive

214
Q

How severe is conductive hearing loss normally?

A

Mild-moderate, usually max of 60 decibels

215
Q

Management of conductive hearing loss?

A

Conservative
Amplification or autoinflation if prolonged - chronic otitis media with effusion
Grommets if not improving

216
Q

What is the treatment of otitis media?

A

Amoxicillin

217
Q

What are grommets?

A

Grommets are tiny tubes which are inserted into the eardrum. They allow air to pass through the eardrum, which keeps the air pressure on either side equal. The surgeon makes a tiny hole in the eardrum and inserts the grommet into the hole

218
Q

What is glue ear?

A

Otitis media with effusion

219
Q

What is otitis media?

A

Inflammatory disease of the middle ear, commonly caused by infection or allergies leading to dysfunction of the eustachian tube

220
Q

Symptoms of otitis media? (4)

A

Ear pain
Fever
Irritability
Accompanying URTI

221
Q

What is periorbital cellulitis?

A

Inflammation and infection of the eyelid and skin around the eye, can be due to sinusitis or other infection with haematological spread

222
Q

Treatment of periorbital cellulitis?

A

Antibiotics - penicillin

223
Q

What is Stephens-Johnson syndrome?

A

Severe blistering form of erythema multiforme involving mucous membranes (conjunctivitis, corneal ulcers) possibly caused by drug sensitivity or infection

224
Q

What is erythema multiforme?

A

Target lesions with a central papule surrounded by an erythematous ring - caused by herpes, drugs, other infections

225
Q

What is utricaria?

A

Hives - flesh coloured weals, usually in response to an allergen/viral infection, may involve deeper tissues to produce angioedema

226
Q

What is angioedema?

A

Swelling of the lips and soft tissues around the eyes

227
Q

What is allergic rhinitis?

A

Can be atopic, seasonal or perennial, with coryza and conjunctivitis or cough with post nasal drip

228
Q

What is atopy?

A

Personal/familial tendency to produce IgE antibodies in response to potential allergens, usually proteins, associated with asthma, eczema, rhinitis

229
Q

Treatment for allergic rhinitis? (5)

A

Non sedating antihistamines - loratidine, cetirizine
Topical corticosteroids
Leukotriene receptor antagonsists - montelukast
Nasal decongestants short term
Allergen immunotherapy

230
Q

What are congenital melanocytic naevi?

A

Moles, congenital moles are present from birth and if >9cm diameter have 4-6% chance of becoming malignant melanoma

231
Q

What is anaphylaxis?

A

Rapid onset, potentially fatal respiratory and cardiovascular compromise, mostly caused by IgE mediated allergies

232
Q

Symptoms of anaphylaxis? (6)

A 
Urticaria
Swelling of lips, tongue, throat
Shortness of breath, stridor, wheeze
Dizziness/fainting
Stomach pain, vomiting
Drowsy, coma
233
Q

Management of anaphylaxis? (6)

A 
Call for help
Give IM adrenaline 1:1000 150mcg <6yr 300mcg 6-12 500mcg >12
Oxygen
IV fluid
Chlorpheniramine antihistamine
Hydrocortisone
234
Q

Long term management of anaphylaxis? (2)

A

Allergen avoidance

Adrenaline auto-injector

235
Q

What is Fanconi anaemia?

A

Most common inherited form (autosomal recessive) of aplastic anaemia (bone marrow failure) - pancytopaenia leading to anaemia, infection, and bruising/bleeding

236
Q

Symptoms of Fanconi anaemia? (40

A

Congenital abnormalities - short stature, abnormal fingers/thumbs, renal malformations, skin lesions
Anaemia
Bleeding
Infection

237
Q

Complications of Fanconi anaemia?

A

Death from bone marrow failure

Acute leukaemia

238
Q

Treatment of Fanconi anaemia?

A

Bone marrow transplant

239
Q

What is haemolytic disease of the newborn?

A

Haemolytic anaemia caused by antibodies destroying the fetal RBC or intrinsic abnormality of the RBC itself

240
Q

Causes of HDN? (4)

A

Rhesus disease
Hereditary spherocytosis
G6PD deficiency
alpha thalassaemia major

241
Q

Explain the mechanism of immune haemolytic disease of the newborn? (4)

A

Commonly, maternal anti-D antibody against fetal rhesus positive antigens
Mother is rhesus negative, baby rhesus positive
Mother makes antibodies against baby’s blood group when sensitisation occurs which cross the placenta
Causes fetal haemolysis of RBC

242
Q

Treatment of immune HDN?

A

Give anti-D to mother at 28 weeks, or if bleeding occurs

Blood transfusion of fetus affected

243
Q

What are the types of thalassaemias

A

Beta thalassaemia major - no HbA produced
Beta thalassaemia intermedia - some HbA producrs
Beta thalassaemia minor (trait carrier)

244
Q

Cause of beta thalassamia?

A

Severe reduction in production of beta globin and reduction in HbA production

245
Q

Symptoms of beta thalassaemia major? (5)

A

Severe anaemia, transfusion dependent
Jaundice
FTT
Extramedullary haemopoiesis - hepatosplenomegaly and bone marrow expansion without transfusions
Can lead to maxillary overgrowth and skull bossing

246
Q

Management of beta thalassaemia major? (3)

A

Lifelong monthly transfusions - repeated causes cardiac failure, liver cirrhosis, diabetes, infertility
Iron chelation to avoid longterm iron effects - dysferrioxamine
Bone marrow transplant

247
Q

What is beta thalassaemia minor?

A

Trait, usually asymptomatic

Mild anaemia if anything

248
Q

What is alpha thalassamia major?

A

Deletion of all four alpha globin genes so no HbA can be produced

249
Q

How does alpha thalassaemia major present?

A

In utero with fetal hydrops which is fatal either then or shortly after delivery
Can be managed with monthly untrauterine transfusions for whole life

250
Q

What are alpha thalassaemia intermedia or minor?

A

Intermedia - 3 globin genes deleted, moderate

Minor - 1/2 globin genes deleted, usually asymptomatic

251
Q

What is Wilms tumour?

A

Nephroblastoma

Originates from embyronic renal tissue, most present before age 5

252
Q

Symptoms of Wilms tumour?

A

Large abdominal mass

Rarely - abdo pain, anorexia, haematuria, anaemia, hypertension

253
Q

Investigations of Wilms tumour? (3)

A

CT/MRI shows cystic and solid mass on kidneys distorting shape
Assess for metastases and staging
Function of contralateral kidney

254
Q

Management of Wilms tumour? (3)

A

Chemotherapy
Delayed nephrectomy
Radiotherapy if advanced

255
Q

What is neuroblastoma?

A

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

256
Q

Presentation of neuroblastoma? (5)

A 
Abdominal mass if adrenal primary
Spinal cord compression if paravertebral
Hepatomegaly
Limp
If over 2 symptoms mainly from metastases - bone pain, bone marrow suppression, weight loss
257
Q

Investigations of neuroblastoma? (4)

A

USS and MRI - large abdo mass, complex relationship with kidney and blood vessels, extending to midline
Raised urinary catecholamine levels
Bone marrow sampling
MIBG scan to map mets

258
Q

Management of neuroblastoma? (5)

A

Surgery to resect localised primaries
Chemotherapy if metastatic
Radiotherapy, autologous stem cell transplant
Immunotherapy

259
Q

What is retinoblastoma?

A

Malignant tumour of retinal cells, accounts for 5% of severe visual impairment in children, some forms are hereditary

260
Q

Symptoms of retinoblastoma? (2)

A

White pupillary reflex replaces red reflex

Squint

261
Q

Treatment of retinoblastoma? (3)

A

Chemotherapy to shrink tumour and local laser treatment to retina
Enucleation of the eye - removal if advanced
Radiotherapy for recurrence

262
Q

What is rhabdomyosarcoma?

A

Most common soft tissue tumour in children, originates from primitive mesenchymal tissue and has varied sites and presentation

263
Q

Symptoms of rhabdomyosarcoma?

A

Head and neck - proptosis, nasal obstruction
GU tumours - dysuria, urinary obstruction
Metastatic - lung, liver, bone marrow

264
Q

Management of rhabdomyosarcoma?

A

Chemotherapy, surgery, radiotherapy

265
Q

2 bone tumours in children?

A

Osteogenic sarcoma

Ewing sarcoma

266
Q

Presentation of bone tumours in children?

A

Persistent limb pain

267
Q

Management of bone tumours? (4)

A

X ray, MRI, bone scan
Chemotherapy before surgery
Resection with prosthesis insertion
Radiotherapy for Ewing sarcoma

268
Q

What is rickets?

A

Failure in mineralisation of the growing bone or osteoid tissue

269
Q

Causes of rickets? (5)

A

Vitamin D deficiency - inadequate intake or sunlight
Diet low in calcium, phosphurus i.e. exclusive prolonged breastfeeding
Intestinal malabsorption - coeliac, cystic fibrosis
Chronic liver/renal disease
Hereditary types

270
Q

Physiology of rickets? (5)

A

Deficient intake/defective metabolism of vitamin D
Causes low serum calcium
Triggers secretion of parathyroid hormone
Normalises calcium but causes loss of phosphate and demineralises bone
Normally, vitamin D3 (cholecalciferol) is hydroxylated in the liver and again in the kidney to 1,25-dyhydroxyvitamin D in response to low calcium

271
Q

Presentation of rickets? (7)

A

Ping pong ball sensation of skull when pressed
Palpable costochondral junctions
Widened wrists and ankles
Harrison sulcus on chest
Bowed legs
FTT
Delayed closure of fontanelle, delayed dentition

272
Q

Diagnosis of rickets?

A

Dietary history
Bloods - calcium low/normal, phosphurus low, alkaline phosphatase activity high, PTH high, vitamin D low
XRay of joints

273
Q

Management of rickets? (4)

A

Balanced diet
Correct risk factors - i.e. malabsorption
Administer daily vitamin D
Complete reversal of bony deformities may take years

274
Q

What is Osgood-Schlatters disease?

A

Osteochondritis of the patellar tendon insertion at the knee, often affecting active teenage boys

275
Q

How does Osgood-Schlatters present? (3)

A

Knee pain after exercise
Localised tenderness and swelling
Hamstring tightness

276
Q

Treatment of Osgood-Schlatters? (3)

A

Reduced activity and physiotherapy
Orthotics
Knee splint

277
Q

What is a slipped capital femoral epiphysis?

A

Displacement of the epiphysis of the femoral head requiring prompt treatment to prevent avascular necrosis, most common in obese teenage boys

278
Q

How does SCFE present? (2)

A

Limp or hip/knee pain

Restricted abduction or internal rotation of the hip

279
Q

Treatment of SCFE?

A

Surgical - pin fixation

3A REVISION (17 decks)

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