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Phase 3a Emily > Paediatrics 3 (Derm, Renal, Haem, Infectious) > Flashcards

Paediatrics 3 (Derm, Renal, Haem, Infectious) Flashcards

1
Q

What is the cause of chickenpox?

A

Varicella zoster virus
- highly contagious
- once had develop immunity for life

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2
Q

Presentation: chickenpox

A
  • widespread, erythematous, raised, vesicular (fluid filled), blistering lesions.
  • rash starts on the trunk or face and spreads outwards affecting the whole body over 2 – 5 days.
  • Eventually the lesions scab over, at which point they stop being contagious.

Fever is often the first symptom
Itch
General fatigue and malaise

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3
Q

How long must you stay away from school with chickenpox?

A

5 days after onset of rash/until all lesions have crusted over

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4
Q

What are the complications of chickenpox?

A

Bacterial superinfection
Dehydration
Conjunctival lesions
Pneumonia
Encephalitis (presenting as ataxia)
Necrotising fasciitis = rapidly evolving painful rash

can lie dormant in sensory dorsal root ganglion cells and reactivate later as shingles or Ramsay Hunt syndrome

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5
Q

What is the management for pregnant women who have not had chickenpox?

A
  • first check for immunity
  • varicella zoster immunoglobulins
  • Plus aciclovir if around time of delivery
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6
Q

Managment: chickenpox

A

self limiting

Aciclovir given to:
- immunocompromised
- adults + adolescents over 14yrs presenting within 24 hrs
- neonates

Itching = calamine lotion + chlorphenamine

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7
Q

Define: Kawasaki disease

A

systemic medium sized vessel vasculitis
- unknown cause
- not contagious

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8
Q

Presentation: Kawasaki disease

A
  • High fever > 5 days
  • Strawberry tongue
  • Cracked lips
  • Cervical lymphadenopathy
  • Conjunctivitis
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9
Q

What is the key complication of Kawasaki disease?

A

coronary artery aneurysm

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10
Q

Investiagtions: Kawasaki disease

A
  • Symptoms
  • Blood tests = FBC, LFTs, ESR
  • Urinalysis
  • ECG = to check no heart involvement
  • Echo
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11
Q

Management: Kawasaki disease

A
  1. IV immunoglobulins (+/- prednisolone) = reduced risk of coronary artery aneurysms
  2. High dose aspirin = reduce risk of thrombosis (one of the few times aspirin is given to children -> Reyes syndrome complication)
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12
Q

What are the 3 phases in Kawasaki disease?

A
  1. Acute phase: The child is most unwell with the fever, rash and lymphadenopathy. This lasts 1 – 2 weeks.
  2. Subacute phase: The acute symptoms settle, the desquamation and arthralgia occur and there is a risk of coronary artery aneurysms forming. This lasts 2 – 4 weeks.
  3. Convalescent stage: The remaining symptoms settle, the blood tests slowly return to normal and the coronary aneurysms may regress. This last 2 – 4 weeks.
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13
Q

Define: whooping cough

A

Upper resp tract infection caused by bordetella pertussis
- whooping sound as they forcefully suck in air
- apnoea

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14
Q

How to diagnose whooping cough?

A
  1. nasal swab with PCR testing or serology

if cough lasted longer than 2 weeks can be tested for anti-pertussis toxin IgG

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15
Q

Management: whooping cough

A
  1. notify public health
  2. supportive care
  3. antibiotics (if within 21 days)
    - azithromycin,
    - erythromycin,
    - clarithromycin
  4. prophylactic antibiotics to close contacts
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16
Q

What is a key complication of whooping cough?

A

bronchiectasis

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17
Q

Define + presentation: mumps

A

Viral infection spread by resp droplets

prodrome
- fever
- muscle aches
- lethargy
- reduced appetite
- headache
- dry mouth
Parotid gland swelling
- abdominal pain
- testicular pain + swelling
- confusion

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18
Q

Management: Mumps

A

PCR testing - antibodies
notify public health
supportive with fluids and analgesia

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19
Q

Define and presentation: measles

A

Highly contagious infection caused by a morbillivirus of the paramyxovirus family

contact with someone with measles
recent travel
fever
maculopapular rash
cough
coryza symptoms
conjunctivitis

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20
Q

Investigation: Measles

A

IgM antibodies
Raised LFTs
Measles RNA PCR on oral fluid specimen

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21
Q

Management: measles

A

Usually self-limiting
- stay away from school or work till 5 days after rash onset

fully vaccinated

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22
Q

Define: encephalitis

A

inflammation of the brain
- infective causes = HSV
- non-infective cause = autoimmune

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23
Q

Presentation: encephalitis

A

Altered consciousness
Altered cognition
Unusual behaviour
Acute onset of focal neurological symptoms
Acute onset of focal seizures
Fever

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24
Q

How to diagnose encephalitis?

A
  • 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
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25
Q

When is a lumbar puncture contraindicated?

A

GCS below 9, haemodynamically unstable, active seizures
or post-ictal.

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26
Q

Management: encephalitis

A

IV antiviral medications to treat the underlying cause:

  • Aciclovir = treats herpes simplex virus (HSV) and varicella zoster virus (VZV)
  • Ganciclovir = treat cytomegalovirus (CMV)

Repeat lumbar puncture is usually performed to ensure successful treatment prior to stopping antivirals

Followup, support and rehabilitation is required after encephalitis, with help managing the complications.

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27
Q

What are the complications of encephalitis?

A

Lasting fatigue and prolonged recovery
Change in personality or mood
Changes to memory and cognition
Learning disability
Headaches
Chronic pain
Movement disorders
Sensory disturbance
Seizures
Hormonal imbalance

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28
Q

How is HIV transmitted?

A
  • Unprotected anal, vaginal or oral sexual activity
  • Mother to child at any stage of pregnancy, birth or breastfeeding. This is referred to as vertical transmission.
  • Mucous membrane, blood or open wound exposure to infected blood or bodily fluids. This could be through sharing needles, needle-stick injuries or blood splashed in an eye.
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29
Q

How to prevent transmission of HIV during birth?

A

Mode of delivery will be determined by the mother viral load:

  • Normal vaginal delivery = is recommended for women with a viral load < 50 copies / ml
  • Caesarean sections = are considered in patients with > 50 copies copies / ml and in all women with > 400 copies / ml

IV zidovudine should be given during the caesarean if the viral load is unknown or there are > 10000 copies / ml

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30
Q

What prophylaxis treatment is given to babies of mothers with HIV?

A

Low risk babies, where mums viral load is < 50 copies per ml = zidovudine for 4 weeks

High risk babies, where mums viral load is > 50 copies / ml = zidovudine, lamivudine and nevirapine for 4 weeks

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31
Q

What should mothers with HIV do about breastfeeding?

A

HIV can be transmitted during breastfeeding
- not recommended

if mothers have undetected viral load, can support them during breast-feeding

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32
Q

When should you test for HIV?

A
  • Babies to HIV positive parents
  • When immunodeficiency is suspected, for example where there are unusual, severe or frequent infections
  • Young people who are sexually active can be offered testing if there are concerns
  • Risk factors such as needle stick injuries, sexual abuse or IV drug use
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33
Q

What testing is done in children to HIV positive parents?

A

Babies to HIV positive parents are tested twice for HIV:

  • HIV viral load test at 3 months. If this is negative, the child has not contracted HIV during birth and will not develop HIV unless they have further exposure.
  • HIV antibody test at 24 months. This is to assess whether they have contracted HIV since their 3 month viral load, for example through breast feeding. If the 3 month test is negative and they are not breastfed, this should be negative.
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34
Q

Management: HIV in children

A
  • Antiretroviral therapy (ART) to suppress the HIV infection
  • Normal childhood vaccines, avoiding or delaying live vaccines if severely immunosuppressed.
  • Prophylactic co-trimoxazole (Septrin) for children with low CD4 counts, to protect against pneumocystis jirovecii pneumonia (PCP)
  • Treatment of opportunistic infections
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35
Q

What urine collection techniques are used?

A

*Cotton wool
* Bag urine
* Clean catch (midstream)
* Catheter bag urine
* In/out catheter
* Suprapubic aspirate

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36
Q

What are the signs + symptoms of UTI in <3 months?

A

Fever
Vomiting
Lethargy
Irritability

Poor feeding
Failure to thrive

less common:
Abdominal pain Jaundice Haematuria Offensive urine

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37
Q

What are the signs and symptoms of UTI in pre-verbal >3 months?

A

Fever

Abdominal pain Loin tenderness Vomiting Poor feeding

less common
Lethargy Irritability Haematuria Offensive urine Failure to thrive

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38
Q

What are the signs and symptoms of UTI in verbal >3 months?

A

Frequency Dysuria
Dysfunctional voiding
Changes to continence
Abdominal pain Loin tenderness

less common
Fever Malaise Vomiting Haematuria Offensive urine Cloudy urine

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39
Q

What is the most common cause of UTIs?

A

E.coli

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40
Q

Presentation: pyelonephritis

A

A temperature greater than 38°C
Loin pain or tenderness

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41
Q

Investigations: UTI

A
  1. Urine sample (try to get clean catch)

Nitrites = bacteria breaks down nitrates into nitrites
(better indication of infection)
Leukocytes

  1. Midstream urine = if nitrite or leukocytes present then urine sent to micro
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42
Q

Management: UTIs

A
  1. <3 months - Admit and give IV Cefotaxime
  2. > 3 months with Upper UTI: Oral Cefotaxime/Co-Amoxiclav for 7-10 days
  3. > 3 months with Lower UTI: Oral Trimethoprim/Nitrofurantoin
  4. Safety net advice: Bring child back if still unwell after 48 hours of Abx
  • trimethoprim
  • Nitrofurantoin
  • Cefalexin
  • Amoxicillin
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43
Q

What are the causes of recurrent UTIs?

A
  • vesicoureteric reflux
  • congenital abnormalities
  • duplex left kidney
  • solitary right kidney
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44
Q

What is vesicle-ureteric reflux?

A

urine has a tendency to flow from the bladder back into the ureters. This predisposes patients to developing upper urinary tract infections and subsequent renal scarring.

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45
Q

Investigations: VUR

A

micturating cystourethrogram (MCUG).

  • X-ray which looks at the flow of urine
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46
Q

management: VUR

A

Avoid constipation
Avoid an excessively full bladder
Prophylactic antibiotics
Surgical input from paediatric urology

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47
Q

What imaging is used for UTIs?

A

micturating cystourethrogram

USS

Dimercaptosuccinic acid (DMSA) scan

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48
Q

What is micturating cystourethrogram?

A

(MCUG)

investigate atypical or recurrent UTIs in children under 6 months.
OR
- a family history of vesico-ureteric reflux,
- dilatation of the ureter on ultrasound or poor urinary flow.

A MCUG is used to diagnose VUR.

catheterising the child, injecting contrast into the bladder and taking a series of xray films

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49
Q

What is a DMSA scan?

A

(Dimercaptosuccinic Acid) scans should be used 4 – 6 months after the illness to assess for damage from recurrent or atypical UTIs.

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50
Q

When is imaging offered to children with UTIs?

A

All children under 6 months with their first UTI should have an abdominal ultrasound within 6 weeks, or during the illness if there are recurrent UTIs or atypical bacteria

Children with recurrent UTIs should have an abdominal ultrasound within 6 weeks

Children with atypical UTIs should have an abdominal ultrasound during the illness

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51
Q

What is the imaging schedule for under 6 months with UTIs?

A

Respond well
- USS at 6 weeks

atypical UTI + recurrent UTI
- USS during acute infection
- DMSA scan 4-6 maths after infection
- micturating cystourethrogram

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52
Q

What is the imaging schedule for 6 months to 3 yrs with UTIs?

A

Respond well
- no scans

Atypical UTI
- USS in infection
- DMSA
- MCUG

Recurrent UTI
- USS within 6 weeks
- DMSA
- MCUG

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53
Q

What is the imaging schedule for over 3 yrs with UTIs?

A

Respond to treatment
- no scans

Atypical
- USS in acute infection

Recurrent
- USS within 6 weeks
- DMSA

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54
Q

What is a atypical UTI?

A
  • Septicaemia/requires IV Abx
  • Non-E.coli organism
  • Poor urine flow
  • Abdominal mass/ bladder mass * Raised creatinine
  • Failure to respond to treatment within 48hrs
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55
Q

Define: recurrent UTI

A

≥2 UTIs, at least one with systemic signs or symptoms

≥3 UTIs without systemic signs or symptoms

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56
Q

What children with UTIs are invited to follow up clinics?

A
  • All children under the age of 3 months
  • Children of any age who are systemically unwell
  • Children with recurrent UTI

Why?
* Address dysfunctional elimination syndromes and constipation

  • Include height, weight, blood pressure and routine testing for proteinuria. This should be performed at least on a yearly basis in all infants and children with renal parenchymal defects.
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57
Q

Define: oedema

A

increase in interstitial fluid

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58
Q

Where can oedema occur?

A

pedal oedema
* ascites
* pleural effusions, pulmonary oedema * facial puffiness

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59
Q

What are the causes of oedema?

A
  • Obstruction of lymphatic drainage
  • Obstruction of venous drainage
  • Lowered oncotic pressure
  • Salt and water retention
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60
Q

What are the features of nephrotic syndrome?

A
  1. proteinuria
  2. hypoalbuminaemia
  3. oedema
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61
Q

What are the causes of nephrotic syndrome?

A

MC = minimal change disease (idiopathic cause)
- HSP
- diabetes
- intrinsic kidney disease

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62
Q

Clinical presentation: nephrotic syndrome

A

Periorbital oedema
Scrotal, vulval, leg and ankle oedema
SOB due to pleural effusions
Frothy urine
Pallor
MC in children aged 2-5 yrs

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63
Q

Investigations: nephrotic

A

urine dipstick
urinary protein:creatinine ratio
Bloods = U+Es and LFTs (so can check albumin)

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64
Q

What are the types of nephrotic syndrome

A

Congenital
Steroid sensitive
steroid resistant

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65
Q

management: nephrotic syndrome

A

Standard course of prednisolone for 1st episode
- 60mg for 4 weeks

Other considerations:
- Na + water moderation
- diuretics
- pen V
- measles and varicella immunity

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66
Q

What are the complications of nephrotic syndrome?

A

Risk of relapses
Hypovolaemia
Thrombosis
Infection
Renal failure

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67
Q

What are the features of nephritic syndrome?

A
  • Haematuria visible (made difference with nephrotic)
  • hypertension
  • oedema
    (can have impaired GFR)
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68
Q

What are the causes of nephritic syndrome?

A
  • Post Strep Glomerulonephritis: Occurs 1-3 weeks after a Strep infection
  • IgA Nephropathy: 1-2 days post infection, often related to HSP which is an IgA Vasculitis
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69
Q

What is post-strep glomerulonephritis?

A

Occurs 1-3 weeks after a beta-haemolytic streptococcus infection such as tonsillitis
- immune complexes made up of antigens, antibodies and complement proteins get stuck in the glomeruli of the kidney + cause inflammation
-inflammation leads to acute deterioration in renal function e.g. AKI

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70
Q

Investigations: Post strep glomerulonephritis

A

Blood tests = FBC, U+Es, immunology
Throat swabs

Urinalysis
- haematuria = macroscopic
- proteinuria = protein :creatinine
- microscopy - RBC cast

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71
Q

Management: post strep glomerulonephritis

A

Fluid balance
- measurement of input/output

correction of other balances - potassium and acidosis

dialysis (uncommon)

penicillin = treatment if strep infection

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72
Q

What is IgA nephritis and presentation?

A

vasculitis affecting skin, joints, GI tracts and kidneys

IgA deposition in glomerulus

  • palpable purpuric rash
  • start on back and spread
  • joint pain
  • abdominal pain

(non-blanching like meningitis but not unwell when come in with rash)

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73
Q

How does IgA nephritis present in the kidneys?

A

haematuria/proteinuria
* nephrotic syndrome
* acute nephritis
* renal impairment
* hypertensio

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74
Q

Management: IgA nephritis

A
  • Supportive treatment
  • Immunosuppressant medications to slow disease progression

*IgA nephropathy is actually quite nasty: many patients who suffer long term with this can end up needing renal transplants due to the significant damage

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75
Q

Define: anaemia

A

A condition in which there is a deficiency of red cells or of haemoglobin in the blood to meet the body’s needs.

(in children normal range changes with age)

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76
Q

What are the reasons for anaemia?

A
  1. Blood loss
    Acute haemorrhage
    Chronic gut bleeding leading to iron deficiency
  2. Decreased Production
    Nutritional deficiency e.g. iron, folate, B12, Vitamin C
    Bone marrow failure e.g. DBA, TEC
    Infiltration e.g. Acute Leukaemia, Neuroblastoma, Lymphoma, Osteopetrosis, Storage Disease
  3. Increased consumption
    Acquired e.g. immune, drugs, parasites, MAHA
    Inherited e.g. red cell membrane defects, enzyme defects
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77
Q

What should you check in the blood when anaemia?

A
  1. reticulocytes
    - immature RBCS
    - can signify if RBCs are being made or broken down
  2. MCV size
    - microcytic
    - microcytic
    - normoocytic
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78
Q

What are the causes of microcytic anaemia?

A

Iron deficiency
Thalassaemia
Sideroblastic Anaemia
Chronic disease
Lead toxicity
Copper deficiency
Haemoglobin E trait
Severe malnutrition

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79
Q

What are the causes of macorcytic anaemia?

A

Normal newborn
Aplastic anaemia
Hypothyroidism
Megaloblastic anaemia
Increased erythropoiesis
Fanconi anaemia
PNH
Drugs
Post splenectomy
DBA

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80
Q

what are the causes of normocytic anaemia?

A

Acute blood loss
Infection
Renal failure
Early iron deficiency
Bone marrow infiltration
Haemolysis (enz/mem)
Hypersplenism
Drugs
Dyserythropoietic anaemia

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81
Q

What are the features of iron deficiency anaemia?

A
  • MC anaemia in children
  • LBW dietary - excessive cows milk intake (very low in iron so don’t eat much iron in other foods)
  • malabsorption
  • bleeding
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82
Q

What are the features of the blood film in iron deficiency?

A

microcytic, hypochromic, low-normal retics

Low ferritin and serum iron, Increased TIBC

High ZPP

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83
Q

Management: iron def

A

oral therapy
- 6mg/kg/day of elemental iron
- reticulocytosis in 72 hrs
iron stores replenish by 3 months
- treatment needed for 3-6 months
constipation common

commonest cause of failure is non-compliance
address cause- usually diet

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84
Q

what are the feature of haemolysis?

A
  • Increased RBC turnover, shortened RBC lifespan
  • RBCs are fragile- especially abnormal ones
  • Spleen filters out and breaks down senescent RBCs, and must work overtime, and can result in effective asplenia (e.g. in sickle cell)
  • RBC degradation products must be handled
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85
Q

What is released in increased destruction of RBCs?

A

Intra corpuscular
Haemoglobin
Enzyme
Membrane

Extra corpuscular
Autoimmune
Fragmentation
Hyper splenism
Plasma factors

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86
Q

Complications: haemolytic anaemia

A

Hydrops fetalis
Neonatal hyperbilirubinaemia
Neonatal ascites
Anaemia/failure to thrive
Splenomegaly
Cholecystitis/gall stones
Hyperbilirubinaemia
Leg ulcers
Aplastic crisis
Thromboembolism

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87
Q

What is haemolytic disease of the newborn ?

A

Rh negative mother previously sensitised to Rh pos cells
Transplacental passage of antibodies
Haemolysis of Rh Pos fetal cells

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88
Q

What are the signs and symptoms of haemolytic disease of the newborn?

A

severe anemia
compensatory hyperplasia & enlargement of blood forming organs (spleen and liver)

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89
Q

Management: haemolytic disease of the newborn

A

prevention of sensitization with Rh immune globulin
intrauterine transfusion of affected fetuses

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90
Q

What are the main features of G6PD?

A

x-linked recessive donation due to deficiency of glucose 6 phosphate dehydrogenase

Three main presentations:
1. Neonatal jaundice
2. Chronic non-spherocytic haemolytic anaemia
3. Intermittent episodes of intravascular haemolysis

Sporadic haemolysis

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91
Q

Cause + treatment: G6PD

A

Typically induced by drugs, fava beans, fever, acidosis
Intravascular haemolysis - haemoglobinuria, rigors, fever, back pain

Treated by stopping precipitant, transfusion, renal support

Bite cells

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92
Q

What are the features of hereditary sphereocytosis?

A

Commonest hereditary haemolytic anaemia in Europeans - 1/5000; probably rarer in Africa

Typically autosomal dominant, but no family history in 25% cases

Heterogeneous - deficiencies of spectrin (41.5%), ankyrin (1.5%), band 3 (17%), band 4.2 (21.5%)
Clinical effects vary from mild to transfusion dependence; tends to be similar within families

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93
Q

Blood film appearance; spherocytosis

A

RBCs are spheres and biconcave so clog up and block blood vessels

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94
Q

management: spherocytosis

A

Offer folic acid and transfuse if necessary
- most people once grown up can manage haemolysis

splenectomy if severe

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95
Q

What are the types of sickle cell disease?

A

Sickle Cell Anaemia (HbSS)
Sickle Haemoglobin C disease (HbSC)
Sickle Beta Thalassaemia (HbS/β thal)

96
Q

Pathophysiology: sickle cell disease

A
  • Substitution of valine for glutamic acid on β chain (HbS)
    (single amino acid substitution)
  • HbS polymerises when deoxygenated leading to sickle shape
  • Occlusion of the microvascular circulation producing vascular damage, infarcts, pain
  • Shortened survival of red cells leading to haemolysis
97
Q

Diagnosisng sickle cell disease

A
  • blood film
  • newborn heel prick test
  • sickle solubility
  • HPLC
98
Q

Complications: sickle cell

A
  • painful crisis
  • anaemia
  • dactylitis
  • chest crisis
  • auto-splenect = body shuts it off as blocked
99
Q

treatment: sickle cell disease

A
  1. Education – keeping warm, well hydrated
  2. Prophylactic Penicillin V to prevent infection due to splenic hypofunction (anti malarials when required)
  3. Ensure immunisations are up to date to prevent infection
  4. Screening such as TCD monitoring, G+M
  5. Effective pain management (NICE requirement)
  6. Prompt treatment of infections
  7. Bone Marrow Transplant
  8. Hydroxycarbamide
  9. New agents e.g. Crizanlizumab, L-Glutamine
100
Q

what is haemostasis?

A

Balance of clotting vs bleeding

involved platelets, coagulation factors and vascular integrity

101
Q

What are the causes of thrombocytopenia?

A

Increased plt destruction (Immune)
ITP
Secondary to infection (HIV, Hepatitis, CMV, EBV, Parvovirus, mumps, measles, pertussis)
Drug induced – valproate, ciprofloxacin, ibuprofen, phenytoin, ranitidine, heparin, captopril
Autoimmune –Evans syndrome
SLE
NAIT
Hyperthyroidism

Increased plt destruction (Non Immune)
Microangiopathic (TTP, HUS)
Kasabach-Merritt Syndrome
Drugs
Infection (viral associated haemophagocytic syndrome) (bacterial infection)
Disseminated Intravascular Haemolysis

Decreased Platelet Production
Constitutional (TAR, CAMT)
Ineffective thrombopoiesis ( B12, Folate Deficiency, severe iron deficiency))
Infiltration (Leukaemia, Non haem infiltration, Osteopetrosis, storage disease)
Metabolic disorders (Ketotic glycinaemia, MMA, Acidaemia)
Bone Marrow Failure (Aplastic Anaemia)

Disorders of platelet pooling
Hypersplenism (Portal Hypertension, Gauchers disease)

Pseudothrombocytopenia
Platelet activation during blood collection

102
Q

What is ITP

A

MC form of immunologic thrombocytopenia
- diagnosis by exclusion
- brusing
- petechiae
- important tor review blood film to ensure no evolving bone marrow disorder
- treatment = rarely required unless bleeding e.g. steroids, IVIg, Trop-RA

103
Q

What are coagulopathies?

A

Various errors in clotting cascade

Bleeding disorders:
hemophilia
von Willebrand disease

Hypercoagulable states
antithrombin, protein C, protein S, FVL, PT mutation, APS

104
Q
A

PT = playing tennis = outside
Extrinsic pathway

PTT = playing table tennis = inside
Intirnsic pathway

105
Q

What is von willebrand disease and the types?

A

Commonest inherited bleeding disorder

Deficiency of VWF (type 1)
Dysfunction of VWF (type 2A, B, M,N)
Complete absence of VWF (type 3)

Autosomal recessive or dominant for type 3 or 2N

106
Q

What are the 2 main roles of VWF?

A
  1. Mediates the adherence of platelets at sites of endothelial damage helping form platelet plug
  2. Binds and transports FVIII, protecting it from degradation
107
Q

presentation: VWD

A

Easy bruising
Epistaxis
Menorrhagia
Mucosal bleeding
Following surgery or trauma

108
Q

Diagnosis: VWD

A

Clotting often shows prolonged APTT (not always!)

VWD screen – FVIII, VWF:Ag, VWF activity

Further investigations can be performed to identify type 1 from 2:
Function:antigen ratio <0.6
RIPA
Multimer Analysis
Genetic analysis

109
Q

Management: VWD

A
  1. Tranexamic Acid

An antifibrinolytic useful for menorrhagia or mucosal bleeding
Given pre procedures IV 10mg/kg and orally 15-25mg/kg tds for other bleeding or post operatively

  1. Desmopressin

Used to elevate FVIII and VWF levels by releasing endothelial stores.
Given IV, SC or intranasal
Perform a DDAVP trial if possible to assess response
Excessive fluid intake can reduce sodium so restrict fluid to 1L for 24 hours post dose
Avoid use if <2 years or monitor sodium closely and avoid if know atherosclerosis
Avoid in Type 2B as thrombocytopenia due to clearance of large multimers.

  1. VWF- containing concentrates

Given if DDAVP inadequate or contraindicated
Voncento, Wilate
Given as IV bolus 12-24 hours as required by bleeding and levels.
Plasma products so ensure hepatitis vaccinations given pre where possible

110
Q

What are the features of haemophilia A+ B?

A

A = def factor VIII (MC)
B = def factor IX

X-linked recessive = boys
Prolonged bleeding
Muscle bleeds
Joint bleeds > arthritis and deformity

Treatment Factor VIII/IX
Complications of treatment

111
Q

What are the presentation of different severities of haemophilia?

A

Mild
Severe bleeding with major trauma or surgery.
Spontaneous bleeding rare

Moderate
Occasional spontaneous bleeding, prolonged bleeding with minor trauma or surgery

Severe
Spontaneous bleeding into joints or muscles

112
Q

what is leukaemia?

A

Abnormality in the maturation of a bone marrow cell
- this abnormal white blood cell keeps being made

113
Q

What leukaemia are common in children?

A

Acute myeloid lymphoblastic leukaemia ALL = MC
(80% cure rate)

Acute myeloid leukaemia = next most common

114
Q

Presentation: leukaemia

A

Persistent fatigue
Unexplained fever
Failure to thrive
Weight loss
Night sweats
Pallor (anaemia)
Petechiae and abnormal bruising (thrombocytopenia)
Unexplained bleeding (thrombocytopenia)
Abdominal pain
Generalised lymphadenopathy
Unexplained or persistent bone or joint pain
Hepatosplenomegaly

115
Q

Diagnosis: leukaemia

A

If suspected urgent full blood count within 48 hours.

  • Full blood count, which can show anaemia, leukopenia, thrombocytopenia and high numbers of the abnormal WBCs
  • Blood film, which can show blast cells
  • Bone marrow biopsy
  • Lymph node biopsy

maybe for staging
Chest xray
CT scan
Lumbar puncture
Genetic analysis and immunophenotyping of the abnormal cells

116
Q

Management: leukaemia

A

primarily treated with chemotherapy (long for 2 yrs)

Other therapies:

Radiotherapy
Bone marrow transplant
Surgery

117
Q

Define: Enuresis

A

Involuntary urination
- bed wetting = nocturnal enuresis
- during the day = diurnal enuresis

118
Q

What is primary nocturnal enuresis?

A

The child has never managed to be consistently dry at night
- MC cause is a variation on normal development + family history

119
Q

What are the causes of primary nocturnal enuresis?

A
  • variation on normal development
  • family history of delayed dry nights
  • overactive bladder
  • fluid intake
  • failure to take
  • psychological distress
  • secondary causes e.g. chronic constipation, UTI, learning disability or cerebral palsy
120
Q

Management: primary nocturnal enuresis

A
  • 2 week diary
  • reassure parents children under 5 yrs will resolve
  • Lifestyle changes: reduced fluid intake in the evenings, pass urine before bed and ensure easy access to a toilet
  • Encouragement and positive reinforcement. Avoid blame or shame. Punishment should very much be avoided.
  • Treat any underlying causes or exacerbating factors, such as constipation
  • Enuresis alarms = wakes child up stopping them from urinating
  • Pharmacological treatment
121
Q

What is secondary nocturnal enuresis?

A

where a child begins wetting the bed when they have previously been dry for at least 6 months.

122
Q

what are the causes of secondary nocturnal enuresis?

A

Urinary tract infection
Constipation
Type 1 diabetes
New psychosocial problems (e.g. stress in family or school life)
Maltreatment

123
Q

What is diurnal enuresis and the main 2 types?

A

the child has become dry at night but still has episodes of urinary incontinence during the day

Urge incontinence = is an overactive bladder that gives little warning before emptying

Stress incontinence = describes leakage of urine during physical exertion, coughing or laughing.

124
Q

management: Enuresis

A

Desmopressin = taken at bedtime with the intention of reducing nocturnal enuresis

Oxybutinin = anticholinergic that reduces the contractibility of the bladder

Imipramine = tricyclic antidepressant, relax bladder and tighten sleep

125
Q

Define: hypospadias

A

Urethral meatus (opening of urethra) in males is abnormally displaced to the ventral side (underside) of the penis, towards the scrotum
- congenital
- diagnosed on examination of newborn

126
Q

Management: hypospadias

A
  • referral to specialist urologist

mild cases = may not require treatment
Surgery is usually performed after 3-4 months of age

surgery aims to correct the position of the meatus and straighten the penis

127
Q

What are the complications of a hypospadias?

A

Difficulty directing urination
Cosmetic and psychological concerns
Sexual dysfunction

128
Q

Define: haemolytic uraemia Syndrome HUS

A

Involves thrombosis in small blood vessels throughout the body, usually triggered by Shiga toxins from either E.coli O157 or Shigella

129
Q

What is the triad in HUS?

A
  1. Microangiopathic haemolytic anaemia
  2. Acute kidney injury
  3. Thrombocytopenia (low platelets)
130
Q

Presentation: HUS

A

First have gastroenteritis
= diarrhoea that turns bloody within 3 days

A week after the onset of diarrhoea HUS develops:
Fever
Abdominal pain
Lethargy
Pallor
Reduced urine output (oliguria)
Haematuria
Hypertension
Bruising
Jaundice (due to haemolysis)
Confusion

131
Q

Management: HUS

A

Medical emergency
1. hospital admissions and supportive management with treatment of:
- Hypovolaemia (e.g., IV fluids)
- Hypertension
- Severe anaemia (e.g., blood transfusions)
- Severe renal failure (e.g., haemodialysis)

Self limiting and most patients fully recover with good supportive care

132
Q

define: Phimosis

A

When the foreskin is tight and is difficult to pull back from the end of the penis

  • normal in babies and young boys
  • most boys cannot pul there foreskin back before 5yrs old but sometimes not till 10 yrs old
133
Q

Presentation: phimosis

A

Not a problems unless these symptoms are seen:
- swelling and tenderness
- pain when peeing or a weak flow of pee
- blood in urine
- frequent urinary tract infections (UTIs)
- bleeding or a thick discharge from under the foreskin or an unpleasant smell – these are signs of an infection (balanitis)
- painful erections, which can make having sex difficult

134
Q

Management: phimosis

A

usually loosens as they get older

if its causing problems:
- steroid creams or gels (topical steroids) to help soften the foreskin
- antibiotics if the foreskin or head of the penis is infected
- surgery – removal of the foreskin (circumcision) or a procedure where small cuts are made in the tip of the foreskin so it can be pulled back more easily (usually only suitable for children)

135
Q

Define: eczema

A

Chronic atopic condition cased by defects in the normal continuity of the skin barrier. leading to inflammation in the skin

136
Q

Presentation: Eczema

A

dry, red, itchy and sore patches of skin over the flexor surface
- periods where the condition is well controlled
- and then flares where it worsens

137
Q

Management: eczema

A

maintenance
- emollients = put on as often as possible esp after washing and before bed (use instead of soap)
e.g. E45, hydrofoil ointment, diprobase cream, Aveeno cream

Flares
- thicker emollients
- topical steroids = want to use weeks steroid for shortest period required to control skin
e.g. Hydrocortisone

138
Q

Define: eczema herpeticum

A

Viral skin infection caused by the herpes simplex virus
- usually occurs in a patient with a pre-existing skin condition

139
Q

Presentation: Eczema herpeticum

A
  • patient with eczema
  • develop widespread painful, vesicular rash
  • fever
  • lethargy
  • irritability
  • reduced oral intake
  • lymphadenopathy
140
Q

Investigation + management: eczema herpeticum

A

Viral swabs

Aciclovir
- mild or moderare with oral
- severe with IV

141
Q

Define: Stevens- Johnson syndrome & toxic epidermal necrolysis

A

A spectrum of the same pathology
- disproportional immune response causes epidermal necrosis

SJS = affects <10% of body surface are

TEN = affects >10% of body surface area

142
Q

What are the causes of SJS and TEN?

A

Medications

Anti-epileptics
Antibiotics
Allopurinol
NSAIDs

Infections

Herpes simplex
Mycoplasma pneumonia
Cytomegalovirus
HIV

143
Q

Presentation: SJS + TEN

A

Start with non-specific symptoms of:
- fever
- cough
- sore throat, mouth and eyes
- itchy skin

then develop:
- purple or red rash
- blisters

Then
- skin starts to break away and shed leaving the raw tissue underneath
- pain, erythema, blistering
- eyes can become inflamed and ulcerated
- can affect urinary, lungs and internal organs

144
Q

Management: SJS + TEN

A

Medical emergency
- admitted to dermatology or burns unit

Steroids
Immunoglobulins
Immunosuppressants

145
Q

What are the complications of SJS and TEN?

A

Secondary infection:
The breaks in the skin can lead to secondary bacterial infection, cellulitis and sepsis.

Permanent skin damage:
Skin involvement can lead to scarring and damage to skin, hair, nails, lungs and genitals.

Visual complications: Depending on the severity, eye involvement can range from sore eyes to severe scarring and blindness.

146
Q

Define: Allergic rhinitis

A

Condition caused by IgE-mediated type 1 hypersensitivity reaction

May be:
seasonal = e.g. hay fever
Perennial = (year round) for examples house dust mite allergy
Occupational = associated with school or work environment

147
Q

Presentation: allergic rhinitis

A

Runny, blocked and itchy nose
Sneezing
Itchy, red and swollen eyes

Allergic rhinitis is associated with a personal or family history of other allergic conditions (atopy).

148
Q

What are the triggers for allergic rhinitis?

A

Tree pollen or grass allergy = leads to seasonal symptoms (hay fever)

House dust mites and pets can lead to persistent symptoms, often worse in dusty rooms at night. Pillows can be full of house dust mites.

Pets can lead to persistent symptoms when the pet or their hair, skin or saliva is present

Other allergens lead to symptoms after exposure (e.g. mould)

149
Q

Management: allergic rhinitis

A

avoid the trigger
- hoovering and changing pillows regularly
- good ventilation
- minimise contact with pets

medication:
- oral antihistamines = taken prior to exposure
* sedating = chlorphenamine and promethazine
* non-sedating = cetirizine, loratadine and fexofenadine

150
Q

Define + presentation: urticaria

A

Aka Hives

  • Small itchy lumps that appear on skin
  • Patchy erythematous rash
  • localised or widespread
151
Q

What is the pathophysiology of urticaria?

A

Caused by the release of histamine and other pro-inflammatory chemicals by mast cells in the skin

may be part of an allergic reaction in acute urticaria or an autoimmune reaction in chronic idiopathic urticaria

152
Q

What are the causes of acute urticaria?

A
  • Allergies to food, medications or animals
  • Contact with chemicals, latex or stinging nettles
  • Medications
  • Viral infections
  • Insect bites
  • Dermatographism (rubbing of the skin)
153
Q

What is chronic urticaria?

A

An autoimmune condition = antibodies target mast cells and trigger them to release histamines and other chemicals

  1. Chronic idiopathic urticaria
    - recurrent episodes of chronic urticaria without a clear underlying cause or trigger
  2. Chronic inducible urticaria
    - episodes that can be induced by certain triggers e.g. sunlight, temp change, exercise, strong emotions
  3. autoimmune urticaria
    - underlying autoimmune condition such as systemic lupus erythematous
154
Q

Management: Urticaria

A
  1. Antihistamines
    e.g. fexofenadine
  2. oral steroids = short course for severe flares

If severe:
- Anti-leukotrienes such as montelukast
- Omalizumab, which targets IgE
- Cyclosporin

155
Q

Define: anaphylaxis

A

Life threatening medical emergency
- caused by severe type 1 hypersensitivity reaction
- IgE stimulates mast cells to rapidly release histamine (mast cell degranulation)

156
Q

Presentation: Anaphylaxis

A

Exposure to allergen
Urticaria
Itching
Angio-oedema, with swelling around lips and eyes
Abdominal pain

Additional symptoms that indicate anaphylaxis are:

Shortness of breath
Wheeze
Swelling of the larynx, causing stridor
Tachycardia
Lightheadedness
Collapse

157
Q

Management: Anaphylaxis

A

A – Airway: Secure the airway
B – Breathing: Provide oxygen if required. Salbutamol can help with wheezing.
C – Circulation: Provide an IV bolus of fluids
D – Disability: Lie the patient flat to improve cerebral perfusion
E – Exposure: Look for flushing, urticaria and angio-oedema

IM adrenaline = repeated after 5 mins if required

Antihistamines = Chlorphenamine or Cetirizine
Steroids = IV hydrocortisone

158
Q

LT Management after anaphylaxis event

A
  1. observation = as biphasic reactions can occur
    - confirmed by measuring the serum mast cell try-take within 6 hrs of event
  2. education for child and family
  3. Adrenaline Auto-Injector = given to all children with anaphylactic reactions
159
Q

Define: scalded skin syndrome

A

a condition caused by a type of staphylococcus aureus bacteria that produces epidermolytic toxins
- these toxins are protease enzymes and break down proteins that hold skin cells together

160
Q

Presentation: Scalded skin syndrome

A

under 5 yrs old

  1. generalised patches of erythema on the skin
    - skin looks thin and wrinkled
  2. then fluid filled blisters called bull form
    - burst and leave very sore, erythematous skin
    (looks like burnt/scalded skin)
  3. systemic symptoms: fever, irritability, lethargy, dehydration
161
Q

What is Nikolsky sign?

A

Positive in scalded skin syndrome

Where very gentle rubbing of the skin causes it to peel away.

162
Q

Management: scalded skin syndrome

A

most patients require admission and treatment with IV antibiotics
- manage fluid and electrolyte balance

163
Q

What is the difference between a macule, papule, plaques and vesicles?

A

Macules = flat, non palpable lesions, <10mm

Papules = elevated lesions, <10mm

Plaques = palpable lesions, >10mm, elevated/depressed compared to skin

Vesicles = small, clear, fluid filled blisters

164
Q

Define: scarlet fever

A

occurs as body’s reaction to erythrogenic toxins produced by strep pyogenes
- peak 2-6yrs old
- spread via resp route (sneezing, coughing)

165
Q

Presentation: scarlet fever

A

sandpaper rash
fever
vomiting
abdo pain
strawberry tongue

166
Q

Management:
Scarlet fever

A

10 day course of penicillin
- school exclusion till 24hrs after commencing abs
- notifiable disease

167
Q

Define: rubella

A

German measles
- caused by togavirus
- rare in uk now that MMR has been introduced
- more common during winter

168
Q

Presentation: rubella

A

low grade fever
rash beginning in face and spreading across whole body
palatal petechia
sub occipital and post auricular lymphadenopathy

169
Q

Management: rubella

A

Supportive treatment

170
Q

Define: slapped cheek disease

A

caused by parvovirus B19
occurs in all ages

171
Q

Presentation: parvovirus b19

A

slapped cheek rash
fever
headaceh
coryza
glove and stock rash

clinical diagnosis but can do serology

172
Q

Management: slapped cheek disease

A

supportive, no longer infectious once rash appears

complications
- someone with haemolytic anaemia > infection can trigger aplastic crisis, pancytopenia
- in pregnant women > cause hydros fetalis in baby

173
Q

Define: roseola infantum

A

affects children <2yrs old
caused by HHV-6

174
Q

presentation: roseola infantum

A

Known as ‘3 day fever’
rose coloured rash starts from trunk, spread peripherally
Nagayama spots: enanthem on uvula and soft palate
self resolving within 2-5 days
may have febrile convulsions prior to rash

supportive treatment

175
Q

Define: thalassaemia

A

Genetic defect in the protein chains that make up haemoglobin
- with in Alpha or beta chains
- autosomal recessive

RBCs are more fragile and break down more easily

176
Q

Signs + symptoms: thalassaemia

A

Microcytic anaemia (low mean corpuscular volume)
Fatigue
Pallor
Jaundice
Gallstones
Splenomegaly
Poor growth and development
Pronounced forehead and malar eminences

177
Q

How to diagnose thalassaemia?

A

Full blood count = shows a microcytic anaemia.

Haemoglobin electrophoresis = is used to diagnose globin abnormalities.

DNA testing = can be used to look for the genetic abnormality

178
Q

Management: alpha thalassaemia

A

chromosome 16

  • Monitoring the full blood count
  • Monitoring for complications
  • Blood transfusions
  • Splenectomy may be performed
  • Bone marrow transplant can be curative
179
Q

What is the chromosome and types in beta thalassaemia?

A

Chromosome 11

Minor
- one abnormal and one normal gene
- only requires monitoring

Intermedia
- 2 defective genes OR 1 defective + 1 deletion gene
- blood transfusion and iron chelation

Major
- no functioning beta global genes
- Severe microcytic anaemia
Splenomegaly
Bone deformities
Management:
-regular transfusions,
- iron chelation and splenectomy.
- Bone marrow transplant can potentially be curative.

180
Q

Define: Fanconi anemia

A

rare genetic disorder
- progressive deficiency of all bone marrow production of RBCs, WBCs and platelets
- increases risk of developing AML

181
Q

What are normal haemoglobin ranges for each age group?

A

Birth
150 – 235 grams/litre

2 – 4 weeks
135 – 190 grams/litre

4 – 8 weeks
95 – 130 grams/litre
(normal dip = negative feedback due to high Hb levels at birth)

(transition at 6 months from fetal to adult haemoglobin)

2 months – 6 years
110 – 140 grams/litre

6 – 12 years
115 – 155 grams/litre

Female age 12 – 18
120 – 160 grams/litre

Male aged 12 – 18
130 -160 grams/litre

182
Q

What the causes of anaemia in infancy?

A

MC = physiologic anaemia of infancy

Anaemia of prematurity
Blood loss
Haemolysis
Twin-twin transfusion (blood is unequal distributed between twins)

Haemolysis:
- Haemolytic disease of the newborn (ABO or rhesus incompatibility)
- Hereditary spherocytosis
- G6PD deficiency

183
Q

What is the cause of anaemia of prematurity?

A

the more premature the infant, the more likely they are to require 1 or more transfusions for anaemia

  • Less time in utero receiving iron from the mother
  • Red blood cell creation cannot keep up with the rapid growth in the first few weeks
  • Reduced erythropoietin levels
  • Blood tests remove a significant portion of their circulating volume
184
Q

What is haemolytic disease of the newborn?

A
  • caused by incompatibility between the rhesus antigens on the surface of the RBCs of the mother and foetus

if mother is rhesus D NEGATIVE
- and baby is positive = mother will produce antibodies to the rhesus D antigen

  1. usually fine in first pregnancy = sensitisation
  2. in later pregnancies = mothers antibodies attach to fetal RBCs causing them to attack their own RBCs
  3. haemolytic > anaemia > high bilirubin levels
185
Q

Investigation: haemolytic anaemia

A

direct Coombs test

186
Q

What are the causes of anaemia in older children?

A
  • iron deficiency anaemia
  • blood loss = menstruation
187
Q

How to categorise anaemia?

A

Microcytic anaemia (low MCV indicating small RBCs)

Normocytic anaemia (normal MCV indicating normal sized RBCs)

Macrocytic anaemia (large MCV indicating large RBCs)

188
Q

What are the causes of microcytic anaemia?

A

TAILS
T – Thalassaemia
A – Anaemia of chronic disease
I – Iron deficiency anaemia
L – Lead poisoning
S – Sideroblastic anaemia

189
Q

What are the causes of normocytic anaemia?

A

3 As and 2 Hs for normocytic anaemia:

A – Acute blood loss
A – Anaemia of Chronic Disease
A – Aplastic Anaemia
H – Haemolytic Anaemia
H – Hypothyroidism

190
Q

What are the causes of macrocytic anaemia?

A

Megaloblastic anaemia is caused by:

B12 deficiency
Folate deficiency

Normoblastic macrocytic anaemia is caused by:

Alcohol
Reticulocytosis (usually from haemolytic anaemia or blood loss)
Hypothyroidism
Liver disease
Drugs such as azathioprine

191
Q

Signs + symptoms: anaemia

A

Tiredness
Shortness of breath
Headaches
Dizziness
Palpitations
Worsening of other conditions
Pica = dietary cravings for abnormal
Hair loss = iron deficiency anaemia

Koilonychia = spoon shaped nails (iron deficiency)
Angular chelitis = iron deficiency
Atrophic glossitis = smooth tongue due to atrophy of the papillae (iron deficiency)
Brittle hair + nails = iron deficiency
Jaundice = haemolytic anaemia
Bone deformities = thalassaemia

192
Q

Investigations: anaemia

A

Full blood count for haemoglobin and MCV
Blood film
Reticulocyte count
Ferritin (low iron deficiency)
B12 and folate
Bilirubin (raised in haemolysis)
Direct Coombs test (autoimmune haemolytic anaemia)
Haemoglobin electrophoresis (haemoglobinopathies)

reticulocytes = immature RBCs

193
Q

Define: idiopathic thrombocytopenia purport

A

spontaneous low platelet count causing a purpuric rash (non-blanching)

  • type 2 hypersensitivity reaction
  • caused by the production of antibodies that target and destroy platelets
194
Q

Presentation: idiopathic thrombocytopenia purport

A

Recent viral illness

  • bleeding = from the gums, epistaxis or menorrhagia
  • Bruising
  • Petechial or purpuric rash = caused by bleeding under the skin
195
Q

Investigation: idiopathic thrombocytopenia purpura

A

Urgent FBC
- low platelet count

196
Q

Management: ITP

A

may be required if the patient is actively bleeding

  • Prednisolone
  • IV immunoglobulins
  • Blood transfusions if required
  • Platelet transfusions only work temporarily (antibodies will start destroying them again)
197
Q

Define: Von Willebrand Disease

A

MC inherited cause of abnormal and prolonged bleeding
- autosomal dominant
- glycoprotein called von willebrand factor = needed for platelet adhesion and aggregation in damaged vessels

198
Q

What are the 3 types of von Willebrand disease?

A

Type 1= a partial deficiency of VWF and is the most common and mildest type

Type 2 = reduced function of VWF

Type 3 = complete deficiency of VWF and is the most rare and severe type

199
Q

presentation: VWF

A

easy, prolonged or heavy bleeding

Bleeding gums with brushing
Nosebleeds (epistaxis)
Easy bruising
Heavy menstrual bleeding (menorrhagia)
Heavy bleeding during and after surgical operations

200
Q

Management: VWF

A

Doesn’t require daily treatment
only needed in response to significant bleeding/trauma or in preparation for operations

  • Desmopressin (stimulates the release of vWF from endothelial cells)
  • Tranexamic acid
  • Von Willebrand factor infusion
  • Factor VIII plus von Willebrand factor infusion
201
Q

Define: haemophilia

A

severe inherited bleeding disorder
- haemophilia A = deficiency of factor VIII
- haemophilia B = factor IX

X- linked recessive disease
(all X chromsomes need to have abnormal gene to have haemophilia = so men only need it on their one x chromosome compared to women needing it on both X chromosomes)

202
Q

Management: haemophilia

A

IV clotting factors VIII or IX

203
Q

Define: Diphtheria

A

Potentially fatal contagious bacterial infection
- mainly affects nose and throat and sometimes skin
- spreads through airborne droplets

204
Q

Presentation: Diptheria

A

Usually mild.
Smptoms often develop gradually, beginning with a sore throat and fever.

In severe cases, a grey or white patch develops in the throat, which can block the airway, and create a barking cough similar to what is observed in croup.

May involve lymph node swelling, and can involve skin, eyes and genitals

205
Q

Define + presentation: polio

A

Polio is the common name of poliomyelitis, an acute clinical disease caused by a poliovirus.

  • flu like prodrome
  • Pre-paralytic stage: fever, increased HR, headache, vomiting, neck stiffness, tremor, limb pain
  • Paralytic stage: LMN/bulbar stage +/- respiratory failure
206
Q

What is the morphology of M. TB?

A

Acid fast Rod bacilli
Non-motile + non-spore forming

Acid fast staining (Zeihl Neelsen stain) = red
–> doesn’t take up gram stain due to mycolic acid capsule
Resistant to phagocytic killing.

Slow growing (15-20 hrs)

207
Q

How does TB lead to the formation of Ghon complexes? (Primary/active TB)

A
  1. Macrophages struggle to clear TB due to its waxy mycolic acid capsule.
  2. Instead of being broken down and cleared, A focal caseating granuloma typically forms in the lower lobe known as a Ghon focus.
  3. The Ghon focus can then spread to the Hilar Lymph nodes in the lungs, which together form a ghon complex

These ghon complexes can under go fibrosis and calcification, leading to the appearance of ranke complexes on xray

208
Q

What is latent TB?

A
  • occurs after primary infection, immune system encapsulates sites of infection and stop the progression of the disease.
  • Patients remain asymptomatic and the bacteria remains dormant, resulting in negative sputumcultures but a positive Mantoux test.
  • These patients are
    not infectious.
    However, if patients are immunocompromised, the disease can progress or reactivate at a later stage to become active TB.
209
Q

Outline what happen in secondary TB.

Where in the lung is it most likely to happen and why?

A

Immunocompromised patients may develop secondary TB when latent TB reactivates
- Patients are infectious.
- Reactivation typically occurs in the lung apex where pO2 is highest, as mycobacteria are aerobic.

bacteria can spread locally, to form caseating granulomata, or systemically (miliary TB).

210
Q

Outline what Miliary TB is, and what happens in it.

A

Miliary TB - Where immune system cannot control the infection and it becomes disseminated

Extrapulmonary TB - where TB infects other areas

211
Q

What are some general symptoms of active TB?

A

often nonspecific:
- prolonged fever,
- malaise,
- anorexia,
- weight loss
- focal signs of infection (e.g. lymph node swelling in TB lymphadenitis).

212
Q

What are some screening Tests for TB/diagnosis of latent TB?

A

Latent Disease - Mantoux

Test/tuberculin skin test - can be positive if have had BCG

Interferon Gamma release assay

213
Q

What would Disseminated Miliary TB look like on chest xray?

A

Patchy Consolidation
Ghon Complex
Granulomatous Lesions

Hilar Lymphadenopathy - (enlargement)
Pleural Effusion

214
Q

What is the management of latent TB?

A

Doesnt necessarily need Tx

If risk of reactivation then:
6 months of isoniazid with pyridoxine
or
3 months of isoniazid, pyridoxine and rifampicin

215
Q

What is the Treatment for Active TB?

A

RIPE: Combination Abx for 6-12 months
R – Rifampicin for 6 months
I – Isoniazid for 6 months
P – Pyrazinamide for first 2 months
E – Ethambutol for first 2 months

Pyra zina mide

Etham but ol

216
Q

Give some side effects of
Rifampicin (TB med)

A

Haematuria
(orange urine)

217
Q

Give some side effects of Isoniazid

What disease can it also trigger?
(TB med)

A

Peripheral Neuropathy

Can also be a trigger for SLE

218
Q

Give some side effects of Pyrazinamide

A

Hepatitis,
Also gout, and joint pain

219
Q

Give some side effects of Ethambutol

A

Eye problems
optic neuritis

220
Q

What is impetigo? What are the common causes of it?

A

Impetigo is a superficial bacterial skin infection

Most common cause - staphylococcus aureus,

also streptococcus pyogenes

221
Q

What are some signs and symptoms of impetigo? Whats the pathophysiology behind it?

A

staphylococcus aureus bacteria produces toxins (exfoliative toxins) that breakdown the proteins that hold skin cells together
- cause 1-2 cm fluid filled vesicles to form on the skin.

  • “golden crust” typically occurring around the nose or mouth
222
Q

Whats the difference between bullous and non bullous impetigo?

A

non-bullous impetigo = no blisters

bullous impetigo = fluid-filled blisters (bullae) are present.
- less common

Bullous is more common in <2 year olds, and almost always caused by staph aureus.

223
Q

What is the treatment for Bullous and non bullous impetigo?

A

Non- bullous
= hydrogen peroxide 1% cream
- for people who are not systemically unwell or at a high risk of complications
= topical antibiotic e.g. Fusidic acid

Widespread impetigo
- oral flucloxacillin

224
Q

What can bullous impetigo lead to?

A

Systemic Symptoms
- They may be feverish and generally unwell.

In severe infections when the lesions are widespread, it is called staphylococcus scalded skin syndrome.

225
Q

What causes nappy rash? How should it be treated?

A

Nappy rash is skin inflammation, mainly due to a reaction of the skin to urine and poo.

  • Switching to highly absorbent nappies (disposable gel matrix nappies)
  • Change the nappy and clean the skin as soon as possible after wetting or soiling
  • Use water or gentle alcohol free products for cleaning the nappy area
  • Use a thin layer of barrier cream
  • Ensure the nappy area is dry before replacing the nappy
  • Maximise time not wearing a nappy
226
Q

What are Signs that would point to a candidal infection rather than simple nappy rash?

A
  • Rash extending into the skin folds
  • Larger red macules
  • Well demarcated scaly border
  • Circular pattern to the rash spreading outwards, similar to ringworm
  • Satellite lesions, which are small similar patches of rash or pustules near the main rash
  • oral thrush
227
Q

What is the treatment for candida?

A

Treatment topical antifungal (imidazole).
Cease the use of a barrier cream until the candida has settled
Canesten® cream has been used to treat fungal nappy rash for 25 years.

Active ingredient clotrimazole

228
Q

What is characterized by toxic shock syndrome? What causes it?

A

A severe systemic reaction to staphylococcal exotoxin.
Toxin-producing S. aureus and group A streptococci

Leaving tampons in too long, female barrier contraceptives, any break in the skin, nasal packing for nose bleeds

characterized by:
* fever over 39° C
* hypotension
* diffuse erythematous, macular rash.

229
Q

What is the treatment of toxic shock syndrome?

A

This is an emergency, ABCDE approach.
Oxygen
IV Broad spec Abx + IV IG
IV Fluids
Surgical debridement

230
Q

Common birthmarks - outline what a salmon patch and an infantile haemangioma is

A

Salmon patch - Flat red or pink patches on a baby’s eyelids, neck or forehead at birth.
They’re the most common type of vascular birthmark and occur in around half of all babies.

Infantile Haemangioma - strawberry marks, are raised marks on the skin that are usually red, occur in 5% of birth, more common in girls.
Rapidly increase in size for the first six months before shrinking

231
Q

Common birthmarks - outline what Port wine stain and cafe au lait spots are?

A

Port wine stain - discoloration of the human skin caused by a vascular anomaly (a capillary malformation in the skin). Mikhail Gorbachev famously had one on his forehead

Café au lait spots, = flat, hyperpigmented birthmarks. They are caused by a collection of pigment-producing melanocytes in the epidermis of the skin. Multiple of these birth can be a sign of neurofibromatosis type 1

232
Q

Common birthmarks - outline what Mongolian spots and congenital melanocytic naevi are

What are Mongolian spots now called?

A

Mongolian spots - More common in darker-skinned people and usually occur over the lower back or buttocks.

now called congenital dermal melanocytosis
- patches of darker brown skin

congenital melanocytic naevi - normal moles
- pink patch with rough border

233
Q

What causes hand foot and mouth disease?

A

Coxsackievirus A16 is the most common cause, and enterovirus 71 is the second-most common cause

234
Q

What is the presentation seen in Hand foot and mouth disease?

A

Tiredness, sore throat, cough, temperature
Then small mouth ulcers appear, Then discrete red spots appear on hands, feet and around the mouth, Then spots may blister

235
Q

What is the treatment for hand foot and mouth disease?

A

Diagnosis is made based on the clinical appearance of the rash.

There is no treatment for hand, foot and mouth disease.

Management is supportive, with adequate fluid intake and simple analgesia such as paracetamol if required. The rash and illness resolve spontaneously without treatment after a week to 10 days

236
Q
A

Phase 3a Emily (8 decks)

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