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3A REVISION > PAEDIATRICS 3 > Flashcards

PAEDIATRICS 3 Flashcards

1
Q

How many newborns become visibly jaundiced?

A

Over 50%

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2
Q

Why does jaundice affect so many newborns? (3)

A

There is a release of haemoglobin from breakdown of RBCs at birth, and RBC lifespan is shorter in neonates than adults along with a less effective bilirubin metabolism

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3
Q

What is kernicterus?

A

Encephalopathy resulting from deposits of unconjugated bilirubin in the basal ganglia if at very high levels, can be fatal

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4
Q

What is the level at which clinical jaundice occurs?

A

80umol/L

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5
Q

What is the main cause of jaundice <24 hours?

A

Haemolysis i.e. rhesus haemolytic disease, ABO incompatibility, G6PD deficiency, congenital infection
Bilirubin is unconjugated and can rise rapidly

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6
Q

What is the cause of jaundice at 2 days - 2 weeks?

A

Physiological

Possibly breast milk jaundice, dehydration, infection

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7
Q

What is the cause of jaundice over 2 weeks? (5)

A

Physiological/breast milk, infection, hypothyroidism, obstruction, neonatal hepatitis

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8
Q

How is jaundice investigated? (3)

A

Blanch skin
Transcutaneous bilirubin meter/bloods
Check bilirubin levels against age and check rate of change
Assess risk factors

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9
Q

How is jaundice managed?

A

Phototherapy if moderate

If severe exchange transfusion

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10
Q

What is biliary atresia?

A

Cause of prolonged neonatal jaundice!, is a progressive disease with destruction or absence of the extrahepatic biliary tree and intrahepatic ducts

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11
Q

Symptoms of biliary atresia? (5)

A 
Failure to thrive
Jaundice
Pale stools
Dark urine
Hepatosplenomegaly
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12
Q

How is biliary atresia investigated? (4)

A

LFTs
USS
Liver biopsy
Confirmed with laparotomy

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13
Q

How is biliary atresia treated?

A

Surgical bypass of the fibrotic ducts - success worse with age
Liver transplant

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14
Q

What are red flags in vomiting? (7)

A 
Bile stained
Bloody
Projectile
Painful
Distension
Dehydration
Failure to thrive
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15
Q

Causes of vomiting in infants? (8)

A 
GORD
Infection
Feeding problems
Obstruction
Intolerances
Metabolic errors
Congenital adrenal hyperplasia
Renal failure
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16
Q

Causes of vomiting in young children? (7)

A 
Gastroenteritis or other infection
Appendicitis
Obstruction
Increased intracranial pressure
Coeliac
Metabolism errors
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17
Q

Caused of vomiting in school age children? (10)

A 
Gastroenteritis/infection
H.pylori/ulcers
Appendicitis
Migraine
DKA
Increased ICP
Coeliac
Alcohol
Pregnancy 
Anorexia
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18
Q

What is gastro-oesophageal reflux?

A

Involuntary passage of gastric contents into the oesophagus

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19
Q

Why is GORD common in infancy?

A

Inappropriate relaxation oft he lower oeseophageal sphincter due to immaturity of the muscle, usually resolves by 1 year

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20
Q

What factors worsen GORD in infants? (3)

A

Fluid diet
Horizontal position
Short oesophagus

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21
Q

3 complications of GORD?

A

Failure to thrive
Oesophagitis
Aspiration

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22
Q

How is GORD diagnosed? (4)

A

Clinically
24hr pH monitoring if atypical
Endoscopy and biopsy
Contrast studies

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23
Q

How is uncomplicated GORD treated?

A

Add thickening agents to feeds and head up position maintained

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24
Q

How is severe GORD treated? (4)

A

H2 receptor antagonist ranitidine
Proton pump inhibitor
Investigate for intolerances
Surgery - Nissen fundoplication if refractory to reinforce closing of the sphincter

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25
Q

What does H.pylori infection cause in children? (3)

A

Nodular antral gastritis

Can uncommonly cause duodenal ulceration

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26
Q

Symptoms of gastritis? (3)

A

Abdominal pain
Nausea
Epigastric pain if duodenal

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27
Q

Investigation of gastritis? (3)

A

Carbon 13 breath test
Stool sample
Possible biopsy

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28
Q

How is gastritis infection treated?

A

Proton pump inhibitor

If H.pylori present, add a macrolide and amoxicillin

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29
Q

Management of recurrent gastritis?

A

Endoscopy - if normal, diagnose functional dyspepsia

Can try hypoallergenic diet

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30
Q

Why are childhood UTIs important to diagnose and investigate?

A

Up to 50% have a structural abnormality of the urinary tract

If pyelonephritis develops, can damage and scar kidneys

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31
Q

How do UTIs present in infants? (8)

A 
Fever
Vomiting
Irritability
Failure to thrive
Jaundice
Septicaemia
Smelling urine
Convulsions
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32
Q

How do UTIs present in children? (11)

A 
Dysuria
Frequency
Loin pain
Fever
Anorexia
Vomiting
Haematuria
Cloudy/smelling urine
Convulsion
Enuresis
FTT!
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33
Q

How are UTIs diagnosed?

A

MSU for MC+S, dipstick

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34
Q

What are common organisms causing childhood UTIs? (4)

A

E.coli
Klebsiella
Proteus (esp in boys)
Pseudomonas

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35
Q

Management of initial UTI? (5)

A

Antibiotics
USS of kidneys ureters and bladder
If <1 year also do micturating cystourethrogram (MSUG) and DMSA radionuclide scan!
1-3 years DMSA
Over 3 years nothing else if USS normal and infection wasn’t atypical/severe

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36
Q

How are UTIs treated?

A

<3 months IV cefotaxime
>3 months pyelonephritis 7-10 days oral ciprofloxacin or co-amoxiclav
>3 months cystitis 3 days oral trimethoprim or nitrofurantoin

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37
Q

Except structural, 2 other causes of UTIs in children?

A

Incomplete bladder emptying

Vesicoureteric reflux

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38
Q

What is nephrotic syndrome?

A

Heavy proteinuria, resulting in low plasma albumin and oedema and hyperlipidaemia

Heavy proteinuria. Hypoalbuminaemia. Peripheral oedema.

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39
Q

What causes nephrotic syndrome?

A

Glomerulonephritis

May be secondary to Henoch-Schonlein purpura, SLE, infections, diabetes

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40
Q

What is glomerulonephritis?

A

Inflammation and damage to the glomeruli, allowing protein/blood to leak into the urine, can present with nephrotic or nephritic syndrome

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41
Q

What types of glomerulonephritis primarily case nephrotic syndrome and why?

A

Minimal change glomerulonephritis - abnormal podocytes (80% of nephrotic syndrome)
Focal segmental glomerulonephritis - sclerosed glomeruli
Membranous glomerulonephritis - thickened basement membrane

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42
Q

Signs of nephrotic syndrome?

A

Oedema - periorbital, scrotal/vulval, ankle/leg, ascites

Breathlessness - pleural effusions, abdominal distension

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43
Q

Investigations of nephrotic syndrome? (5)

A 
Dipstick urine for MC and S
FBC, ESR, U+E, autoantibodies
Creatinine and albumin
Throat swab
Hep B and C
Renal USS
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44
Q

What is the treatment for minimal change GN (nephrotic)? (3)

A

Prednisolone at 60mg/m2 for 4 weeks then 40 on alternate days for 4 weeks
Reduce oedema - fluid management, salt restriction, give diuretic and ACE inhibitor
Statin for hyperlipidaemia

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45
Q

What is the treatment of focal segmental GN (nephrotic)? (4)

A

Salt restriction and diuretics
ACE inhibitor
Statin for hyperlipidaemia
Transplant often required - steroids DONT work

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46
Q

What is the treatment of membranous GN (nephrotic)?

A

Prednisolone

1/3 have chronic GN, 1/3 remission, 1/3 renal failure

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47
Q

What are some complications of nephrotic syndrome? (4)

A

Hypovolaemia
Thrombosis
Infection
Hypercholesterolaemia

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48
Q

What is nephritic syndrome?

A

Haematuria with RBC casts in urine, hypertension, oliguria, small proteinuria - due to glomerular inflammation
Can rapidly progress to kidney failure

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49
Q

What causes nephritic syndrome?

A

Glomerulonephritis

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50
Q

What types of glomerulonephritis primarily cause nephritic syndrome? (4)

A

IgA nephropathy
Post infectious (streptococcal) GN
Membranoproliferative GN
Rapidly progressive GN

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51
Q

What are the types of rapidly proliferative glomerulonephritis (nephritic)? (3)

A

Goodpasture’s syndrome
Microscopic polyangitis
Grnaulomatosis with polyangitis

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52
Q

How is nephritic syndrome generally treated?

A

Monitor fluids and electrolytes
Diuretics
Possible steroids
If rapidly progressive - biopsy, immunosuppression and steroids, plasma exchange

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53
Q

What is post streptococcal (infectious) GN (nephritic)?

A

Usually follows weeks after sore throat (strep pyogenes)

Returns to normal <1 month

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54
Q

What is IgA nephropathy (nephritic)?

A

May present with macroscopic haematuria
24-48 hours post URTI infection
20% have end stage renal failure

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55
Q

What is Goodpasture’s syndrome (nephritic)?

A

Autoimmune ant glomerular basement membrane antibodies
Also affects lungs
Treat with steroids, plasmaphoresis

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56
Q

What is microscopic polyangitis (nephritic)?

A

Vasculitic
p-ANCA+ve, affects small vessels
Treat long term steroids and cytotoxics

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57
Q

What is Wegener’s granulomatosis/granulomatosis with polyangitis (nephritic)?

A

Vasculitic
c-ANCA+ve
Affects lungs and kidneys
Treat with steroids, plasmaphoresis, cyclophosphamide

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58
Q

What type of diabetes do children get?

A

Type 1 (rare type 2)

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59
Q

What causes diabetes mellitus?

A

Genetic disposition - family history, HLADR3/4 and other autoimmune association
Environmental - enteroviral infection, diet (cow milk proteins), overnutrition

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60
Q

When does diabetes mellitus present?

A

2 peaks - preschool and late teens

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61
Q

How does diabetes mellitus present? (5)

A 
Polyuria
Polydipsia
Weight loss
Young children - wet the bed, candida infection
DKA uncommon
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62
Q

How is diabetes mellitus diagnosed? (6)

A 
In symptomatic children with random blood glucose >11.1mmol/L
Glycosuria
Ketonuria
Fasting blood test >7mmol/L
HbA1c >48
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63
Q

How are most new cases of diabetes mellitus managed? (5)

A

SC insulin injections with education, BM monitoring
Reduced carb diet
Adjustment for illness, exercise
Hypoglycaemia education

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64
Q

Types of insulin regimes? (4)

A

Rapid acting
Short acting - before meals lasting 8 hours
Intermediate lasting 12 hours
Mixed

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65
Q

How can insulin be delivered?

A

Continuously via a pump

Injections - watch for fatty change

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66
Q

What is the most common insulin regime in children?

A

Pump or 3/4 short acting injections a day

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67
Q

How are hypoglycaemic attacks managed?

A

Glucose tablets/glucogel/glucagon injection

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68
Q

Complications of diabetes mellitus? (7)

A 
Retinopathy
Nephropathy
Hypertension
Neuropathy
Coeliac/thyroid disease
Delayed puberty
Obesity
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69
Q

Signs of diabetic ketoacidosis? (7)

A 
'Pear drop' breath
Vomiting
Dehydration
Abdominal pain
Hyperventilation
Shock
Coma
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70
Q

How is DKA managed? (6)

A 
Fluids slowly over 48 hours
Insulin infusion titrated with BM
Give potassium
Re-establish oral fluids, diet, SC insulin
Identify cause
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71
Q

What causes type 1 diabetes?

A

Autoimmune destruction of the insulin producing beta cells of the islets of Langerhans in the pancreas

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72
Q

What causes type 2 diabetes?

A

Combination of inadequate insulin secretion by beta cells and peripheral insulin resistance

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73
Q

How common is undescended testicles?

A

4% of males at birth, more if preterm

1.5% by 3 months

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74
Q

What are the types of undescended testicles? (4)

A

Retractile - normally descend with age
Palpable in groin but can’t be manipulated in scrotum
Impalpable and may be in inguinal canal/abdomen
Absent

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75
Q

Investigations of undescended testicles?

A

Ultrasound
Hormonal tests if impalpable/absent
Laparoscopy

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76
Q

Treatment of undescended testicles?

A

Orchidopexy - surgery for fertility reasons and to reduce malignancy

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77
Q

When should testicles be descended in the baby?

A

3-6 months

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78
Q

What is torsion of the testes?

A

Spermatic cord rotates and becomes twisted, cutting off testicle blood supply - emergency
Most common in adolescents

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79
Q

Symptoms of testicular torsion?

A

Groin/scrotal/abdominal pain

Swelling

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80
Q

How is testicular torsion treated?

A

Within 6 hours to save testicle

Surgical exploration, and if confirmed fix both testicles in place

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81
Q

What is bell clapper deformity?

A

Tunica vaginalis joins high on the spermatic cord, so the testes are not anchored

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82
Q

What is hydrocoele?

A

Fluid around the testicles, caused by patent processus vaginalis, can occur after illness

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83
Q

Symptoms of hydrocoele? (4)

A

Swelling
Bluish discolouration
Non tender
Transilluminate

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84
Q

How is hydrocoele treated?

A

Most resolve but surgery if persistent after 2 years

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85
Q

What is varicocoele?

A

Varicosities of testicular veins, often around puberty, associated with decreased fertility

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86
Q

How is varicocoele treated?

A

Surgery if painful or impaired growth

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87
Q

What is congenital adrenal hyperplasia?

A

Group of inherited conditions where the adrenal gland is large, with a lack of cortisol and increase in androgens

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88
Q

What effects does CAH cause? (4)

A

Male characteristics to appear early in boys and inappropriately in girls
Less able to cope with stress
Hyponatraemia, hypoglycaemia

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89
Q

What is the mechanism behind CAH? (3)

A

Lack of 21-hydroxylase deficiency for cortisol synthesis
Inability to produce aldosterone
Cortisol deficiency stimulates pituitary to produce ACTH increasing androgens

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90
Q

In what patients is CAH more common?

A

Consanguineous

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91
Q

How does CAH present? (5)

A

Virilisation of female genitalia
Large penis and pigmented scrotum in males
80% salt losing crisis
Male non salt losers - tall stature
Female non salt losers - muscular, BO, pubic hair, acne

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92
Q

What is a salt losing (adrenal) crisis? (6)

A 
Vomiting
Weight loss
Shock
Hyponatraemia, hyperkalaemia, hypogylcaemia
Metabolic acidosis
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93
Q

How is CAH treated? (5)

A

Females - corrective surgery externally
Glucocorticoids, mineralocorticoids
NaCl if salt losing
Additional corticosteroids if ill

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94
Q

How is a salt losing crisis treated? (3)

A

Hydrocortisone
Saline
Glucose IV

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95
Q

What is turners syndrome?

A

45X, female only has one normal X chromosome, normally spontaneous

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96
Q

How is turners syndrome diagnosed?

A

Antenatally
95% miscarry
Clinically

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97
Q

How does turners syndrome present? (9)

A 
Short stature
Lymphoedema of extremities 
Neck webbing
Wide carrying angle
Wide spaced nipples
Coarctation of aorta
Delayed puberty
Infertility
Hypothyroidism
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98
Q

How is turners syndrome treated? (3)

A

Growth hormone
Oestrogen replacement
ART for fertility

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99
Q

What is Edward’s syndrome?

A

Trisomy 18, most die in infancy

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100
Q

Symptoms of Edward’s syndrome? (7)

A 
Low birthweight
Prominent occiput
Small mouth/chin
Short sternum 
Overlapping flexed fingers
Rocker bottom feet
Heart/kidney malformation
101
Q

What is Patau syndrome?

A

Trisomy 13, most die in infancy

102
Q

Symptoms of Patau syndrome? (6)

A 
Brain maldevelopment
Scalp defects
Small defected eyes
Cleft lip
Polydactyly
Heart/kidney defects
103
Q

What is Klinefelter syndrome?

A

47, XXY affects 1-2/1000 males

104
Q

Symptoms of Klinefelter syndrome? (5)

A 
Infertility, hypogonadism
Small testes
Gynaecomastia
Tall
Psychological problems
105
Q

What is William’s syndrome?

A

Deletion of 27 genes on chromosome 7, usually sporadic

106
Q

Symptoms of Williams syndrome? (5)

A 
Short flat nasal bridge
Elf like appearance
Learning difficulties
Heart disease
Happy demeanour
107
Q

What is failure to thrive?

A

Suboptimal weight gain in infants and toddlers

108
Q

Causes of failure to thrive? (5)

A 
Inadequate intake
Inadequate retention
Malabsorption
Failure to utilise
Increased requirement
109
Q

Causes of inadequate intake?

A

Non organic (environmental) or organic

110
Q

Causes of environmental inadequate intake? (5)

A 
Feeding problems
Inadequate milk
Low socioeconomic status
Maternal depression or poor education
Neglect or child abuse
111
Q

Causes of organic inadequate intake? (4)

A

Impaired suck/swallow (oromotor dysfunction)
Neurological problems
Cleft palate
Chronic illness - cystic fibrosis, Crohn’s

112
Q

Causes of inadequate retention? (2)

A

GORD

Vomiting

113
Q

Causes of malabsorption? (5)

A 
Coeliac
Cystic fibrosis
Cow's milk protein allergy
NEC
Short gut
114
Q

Causes of failure to utilise? (4)

A

Chromosomal disorders
Prematurity
Infection
Metabolic disorders

115
Q

Causes of increased requirement? (6)

A 
Hyperthyroid
Cystic fibrosis
Malignany
Chronic infection
Severe combined immunodeficiency
Heart/renal disease
116
Q

Management of failure to thrive?

A

Detailed history and examination for underlying disease - then treat
MDT care
Education and monitoring if no cause

117
Q

Management of severe failure to thrive?

A

If <6 months and severe, admit for refeeding

118
Q

What is a choledochal cyst?

A

Cystic dilatations of the extrahepatic biliary system

119
Q

How does a choledocal cyst present? (4)

A

Abdo pain
Mass
Jaundice
Cholangitis

120
Q

How is a choledocal cyst treated?

A

Surgical excision and anastomosis to biliary duct

121
Q

What is neonatal hepatitis syndrome?

A

Prolonged neonatal jaundice and hepatic inflammation, may have IUGR and hepatosplenomegaly

122
Q

Causes of neonatal hepatitis syndrome?

A 
Congenital infection
Metabolism errors
Alpha antitrypsin deficiency
Cystic fibrosis
Galactosaemia
123
Q

What is acute liver failure?

A

Development of massive hepatic necrosis with subsequent loss of liver function

124
Q

Symptoms of acute liver failure? (7)

A 
Jaundice
Encephalopathy
Coagulopathy
Hypoglycaemia
Electrolyte disturbance
Haemorrhage
Cerebral oedema
125
Q

Management of acute liver failure? (4)

A

Maintain blood glucose
Prevent sepsis - Abx
Vitamin K to prevent haemorrhage
Liver transplant

126
Q

Causes of chronic liver disease in children? (7)

A 
Hepatitis B or C or autoimmune
Drugs - NSAIDs
IBD
Wilson disease
Cystic fibrosis
Alpha antitrypsin deficiency
Neonatal liver disease
127
Q

Symptoms of chronic liver disease?

A 
Acute hepatitis
Hepatosplenomegaly
Cirrhosis
Portal hypertension
Lethargy, malnutrition
128
Q

Management of liver disease?

A 
Treat specific causes
Adequate nutrition
Fat soluble vitamins ADEK
Ursodeoxycholic acid for itching
Liver transplant
129
Q

What is congenital nephrotic syndrome?

A

Inherited disorder, infants present with LBW, oedema, hypoalbuminaemia, proteinuria, often leads to kidney failure

130
Q

What is haemolytic uraemic syndrome?

A

Triad of acute renal failure, microangiopathic haemolytic anaemia and thrombocytopaenia

131
Q

Cause of haemolytic uraemic syndrome?

A

Typically secondary to GI infection with e.coli of shigella
Bacteria cause intravascular thrombogenesis in kidneys
Coagulation pathway activated, platelets consumed
RBC damage through occluded vessels

132
Q

Symptoms of haemolytic uraemic syndrome? (7)

A 
Diarrhoeal prodrome (bloody)
Haematuria
Oliguria
Hypertension
Abdo pain, vomiting
Oedema
Possible purpura
133
Q

Complications of HUS?

A

Acute kidney injury

134
Q

Treatment of HUS? (2)

A

Early supportive therapy

Dialysis

135
Q

What is acute kidney injury?

A

Acute renal failure where there is a sudden reversible reduction in renal function

136
Q

Types of AKI?

A

Prerenal (commonest in paeds)
Renal
Postrenal

137
Q

Prerenal AKI causes? (5)

A 
Gastroenteritis
Burns
Sepsis
Nephrotic syndrome
Circulatory failure
Anything that causes hypovolaemia
138
Q

Renal AKI causes? (4)

A

Haemolytic uraemic syndrome
Acute tubular necrosis
Renal vein thrombosis
Glomerulonephritis

139
Q

Postrenal AKI causes?

A

Obstruction

140
Q

Treatment of AKI? (3)

A

Prerenal - correct hypovolaemia, circulatory support
Renal - restrict fluids, diuretic, high calorie diet
Postrenal - nephrostomy, bladder catheterisation, surgery
Dialysis

141
Q

When is dialysis indicated? (5)

A 
Failure of treatment
Severe electrolyte disturbances
Pulmonary oedema or hypertension
Severe acidosis
Multisystem failure
142
Q

Causes of chronic renal failure? (4)

A

Structural malformations
Glomerulonephritis
Hereditary nephropathies
Systemic diseases

143
Q

Presentation of chronic renal failure? (6)

A 
Anorexia
Lethargy, failure to thrive
Polydipsia and polyuria
Bony deformities - renal rickets
AKI
Anaemia
144
Q

Management of chronic renal disease? (8)

A 
Nutrition 
Vitamin D, restrict phosphate
Salt and fluids
Bicarbonate supplements
Erythropoietin
Give growth hormone
Dialysis
Transplant
145
Q

What is renal agenesis?

A

Absence of both kidneys causing reduced fetal urine and oligohydramnios = fetal compression - Potter syndrome

146
Q

What is multicystic dysplastic kidney?

A

Failure of union of the ureteric bud with the nephrogenic mesenchyme, non functioning, large cysts, no connection with bladder

147
Q

What are autosomal dominant and recessive polycystic kidneys?

A

Large cystic kidneys that maintain some function but predisposed to renal failure

148
Q

What is a horseshoe kidney?

A

Pelvic kidney - abnormal caudal migration, in midline, may predispose to infection or obstruction

149
Q

What are duplex kidneys?

A

Premature division of the ureteric bud, varies from a bifid renal pelvis to complete division with 2 ureters, often have abnormal drainage

150
Q

Management of renal malformations? (4)

A 
Prophylactic antibiotics at birth to prevent UTI
USS 
Treat chronic kidney disease/AKI
Dialysis
Transplant
151
Q

What is vesicoureteric reflux?

A

Developmental abnormality of the vesicoureteric junctions, ureters are laterally displaced and shortened and enter directly into bladder rather than angled

152
Q

What does VUR cause?

A

Reflux into the renal system - can cause distended ureters and renal pelvis

153
Q

Treatment of VUR? (3)

A

Many resolve on own with growth
Treat infections
Surgery - ureteric valve

154
Q

What is Alport syndrome?

A

Familial nephritis, X linked recessive that progresses to end stage renal failure, associated with deafness and ocular defects

155
Q

What is SLE?

A

Systemic lupus erythematosus, autoimmune disease, may present with haematuria or proteinuria

156
Q

Effect of SLE on kidneys?

A

AKI, CKD from lupus nephritis

Immune complex deposition along the glomerular basement membrane

157
Q

What is Henoch Schonlein purpura?

A

Inflammation of small blood vessels - vasculitis, possibly due to IgA and IgG complexes and complement activation
Cause of nephritis

158
Q

Symptoms of Henoch Schonlein purpura? (6)

A

May be preceded by URTI
Purpuric palpable rash over buttocks, extensor surfaces of arms and legs, ankles - TRUNK SPARING
Joint pain in knees and ankles
Joint swelling
Abdominal pain
Glomerulonephritis - haematuria, possible proteinuria

159
Q

Treatment of Henoch Schonlein purpura? (4)

A 
Fluid and electrolyte balance
Diuretics
Possible steroids for abdo pain
Monitor for deterioration after resolution
Self limiting
160
Q

What is impetigo?

A

Localised highly contagious staphylococcal and/or streptococcal skin infection, more common where there is pre-existing skin disease

161
Q

Lesions in impetigo?

A

On the face, neck and hands and begin as erythematous macules which may become vesicular/pustular or blistered, drainage leaves honey-coloured crusted lesions

162
Q

Treatment of impetigo?

A

Topical Abx for mild

Systemic flucloxacillin or co-amoxiclav for severe

163
Q

What is scalded skin syndrome?

A

Caused by staphylococcus causing separation of the epidermal skin from underlying layers, widespread erythema and tenderness, treat with IV abx

164
Q

What is whooping cough?

A

Highly contagious respiratory infection caused by Bordatella pertussis, epidemics every 3-4 years

165
Q

Symptoms of whooping cough? (5)

A

Week of coryza, then development of characteristic paroxysmal cough followed by inspiratory whoop
Cough worse at night, may vomit
Red/blue in face when coughing, may have epistaxis
Infants - apnoea instead of whoop
May decrease but persist for months

166
Q

Management of whooping cough?

A

If severe, isolated in hospital
Erythromycin - only helps symptoms if started in coryzal phase
Close contacts - erythromycin prophylaxis

167
Q

Name 2 diseases eradicated by the vaccination programme?

A

Diptheria, polio

168
Q

What are the major routes of HIV transmission to children?

A

Mother to child transmission - intrauterine, intrapartum, breastfeeding
Infected blood products
Rare - contaminated needles,sexual abuse

169
Q

Diagnosis of HIV in children? (2)

A

Transplacental maternal IgG HIV detection if <18 months and mum has HIV shows exposure
HIV DNA PCR positive shows infection

170
Q

Features of HIV in children? (5)

A

Some progress rapidly to AIDS wihin a year
Some asymptomatic for years before progressing
Lymphadenopathy
Recurrent bacterial infections or candidiasis
Lymphocytic interstitial pneumonitis

171
Q

Indications of severe HIV infection? (4)

A

Pneumocystis jiroveci (carinii) pneumonia (PCP)
Severe failure to thrive
Encephalopathy
Malignancy

172
Q

With what symptoms should you test for HIV? (6)

A 
Persistent lymphadenopathy
Hepatosplenomegaly
Recurrent fever
Parotid swelling
Thrombocytopaenia
Serious, persistent, unusual, recurrent infections
173
Q

Treatment of HIV? (4)

A

Infants should start antiretroviral therapy after diagnosis
Older children based on viral load and CD4 count
Co-trimoxazole for PCP prophylaxis
Up to date immunisations (not BCG as live)

174
Q

What is encephalitis?

A

Inflammation of the brain, caused by viruses such as HSV, post-infectious encephalopathy after VZV, slow virus such as HIV

175
Q

Symptoms of encephalitis? (4)

A

Fever
Altered consciousness
Seizures
Behaviour change

176
Q

Treatment of encephalitis?

A

High dose aciclovir IV

177
Q

What does candida infection commonly cause in children?

A

Nappy rash - erythematous, involves the flexures, treat with topical antifungal

178
Q

What is irritant dermatitis?

A

Nappy rash, affects convex surfaces of buttocks perineum abdomen and thighs but flexures are spared, use emollient or topical steroids

179
Q

What is toxic shock syndrome?

A

Caused by staph aureus and group A strep, gives fever hypotension and a diffuse erythematous macular rash and can cause organ dysfunction

180
Q

Treatment of toxic shock syndrome?

A

Surgical debridement of affected areas

Ceftriaxone and clindamycin

181
Q

What is scarlet fever?

A

Caused by group A strep infection, can lead to rheumatic fever/post strep glomerulonephritis/reactive arthritis

182
Q

Symptoms of scarlet fever? (6)

A

Sore throat, fever, headaches, swollen lymph nodes, and a characteristic rash - red and like sandpaper, starts on face can spread to body
Strawberry tongue

183
Q

Treatment of scarlet fever?

A

Antibiotics - penicillin/amoxicillin

184
Q

What is coxsackie disease?

A

Hand foot and mouth disease - flu like symptoms, blisters

185
Q

How is coxsackie treated?

A

Supportive

Watch for meningitis/encephalitis

186
Q

Types of immune deficiency?

A

Primary - intrinsic defect, mostly inherited
Secondary - caused by another disease/treatment such as infection, HIV, malignancy, immunosuppression, splenectomy, malnutrition

187
Q

Presentation of immune deficiency? (6)

A 
Recurrent bacterial infections
Severe infections
Atypically presenting infections
Failure of treatment for infections
Unexpected causative pathogen
Extensive candidiasis
Severe, Prolonged, Unusual or Recurrent
188
Q

Management of immune deficiency? (5)

A 
Antimicrobial prophylaxis 
Prompt treatment of infection, lower course abx
Screen for end organ disease
Ig replacement therapy
Bone marrow transplantation - for SCID
189
Q

What are febrile seizures?

A

Seizure accompanied by a fever in the absence of intracranial infection due to bacterial meningitis or viral encephalitis, occur in 3% between 6 months and 5 years

190
Q

Features of febrile seizures?

A

Occur early into a viral infection when temp rises
Usually brief generalised tonic clinic
If focal, prolonged or repeated in same illness risk of epilepsy increased

191
Q

Management of febrile seizures? (3)

A

Infection screen
If unconscious/unstable, abx
If prolonged, rescue rectal diazepam

192
Q

What is premature sexual development?

A

Development of secondary sexual characteristics before 8 in girls and 9 in boys

193
Q

What is precocious puberty?

A

Premature sexual development either gonadotrophin-dependent with premature activation of HPG axis or gonadotrophin-independent from excess sex steroids

194
Q

Cause of precocious puberty?

A

Females - premature onset of puberty

Male - more often organic cause such as intracranial tumour, adrenal hyperplasia, gonadal tumour

195
Q

Management of precocious puberty?

A 
Detect and treat underlying pathology
Reduce rate of skeletal maturation
Address psychological/behavioural issies
Gonadotrophin-dependent: GnRH analogues
Gonadotrophin independent: Androgen inhibitors medroxyprogesterone acetate
196
Q

What are thelarche and pubarche?

A

Thelarche - breast development

Pubarche - pubic hair development

197
Q

What is Prader-Willi syndrome?

A

Genetic disorder caused by loss of function of genes, mostly due to paternal partial deletion of chromosome 15 - imprinting disorder

198
Q

Features of Prader-Willi syndrome? (8)

A 
Characteristic facial features
Hypotonia
Feeding problems
FTT
Obesity
Hypogonadism
Developmental delay
Learning difficulties
199
Q

What is Noonan syndrome?

A

Mutation in one of several autosomal dominant genes

200
Q

Features of Noonan syndrome? (6)

A 
Characteristic facial features
Mild learning difficulty
Short webbed neck, trident hair line
Pectus excavatum
Short stature
Congenital heart disease
201
Q

What is Angelman syndrome?

A

Loss of function of part of a maternal copy of chromosome 15 - imprinting disorder

202
Q

Features of Angelman syndrome?

A

Cognitive impairment
Characteristic facial appearance
Ataxia
Epilepsy

203
Q

What is muscular dystrophy?

A

Group of inherited disorders with muscle degeneration that is often progressive

204
Q

What is Duchenne muscular dystrophy?

A

Most common dystrophy, X linked recessive so affects males, deletion of a site of the short arm of the X chromosome that codes for dystrophin

205
Q

What does dystrophin do?

A

Connects the cytoskeleton of the muscle fibre to the surrounding extracellular matrix through the cell membrane - deficiency leads to myofibre necrosis

206
Q

Presentation of Duchenne muscular dystrophy? (7)

A 
Waddling gait
Language delay
Difficulty running, with stairs
Pseudohypertrophy of calves due to fat replacement
Clumsiness
Scoliosis
Learning difficulty
207
Q

Complications of Duchenne muscular dystrophy?

A

Limited life due to respiratory failure or cardiomyopathy

208
Q

Management of Duchenne muscular dystrophy? (6)

A

Exercise and stretching
Night splints to prevent contractures
Orthoses and Achilles lengthening to help walk
Brace for scoliosis/metal rod insertion
Overnight CPAP
Corticosteroids 10 days a month to preserve mobility

209
Q

What are causes of hypogonadotrophic hypogonadism? (4)

A

Systemic disease - cystic fibrosis, asthma, Crohns, anorexia, excess training
Panhypopituitarism
Isolated gonadotrophin deficiency
Acquired hypothyroidism

210
Q

What are causes of hypergonadotrophic hypogonadism? (3)

A

Chromosomal abnormalities - Klinefelters, Turners
Steroid hormone enzyme deficiencies
Gonadal damage - surgery, chemo, trauma

211
Q

What is delayed puberty?

A

Absence of pubertal development by 14 in females 15 in males, more common in males due to constitutional delay

212
Q

Caused of delayed puberty? (3)

A

Constitutional
Hypogonadotrophic hypogonadism - low gonadotrophic secretion, problem with pituitary/hypothalamus
Hypergonadotrophic hypogonadism - high gonadotrophin secretion, problem with gonadal response to hormones

213
Q

Management of delayed puberty? (4)

A

Karyotyping
Thyroid, sex hormone test
Treatment not normally needed - if so, oxandrolone for boys (weak androgen steroid) or IM testosterone
For girls, oestradiol

214
Q

How is childhood obesity defined?

A

BMI >98th centile for age and sex, >91st centile is overweight

215
Q

Complications of obesity?

A

Type 2 diabetes

CV disease

216
Q

2 exogenous causes of obesity?

A

Hypothyroidism

Cushings disease

217
Q

Management of obesity? (4)

A

Sustained changes in lifestyle - healthier eating, increased physical activity, reduction in inactivity
Drug treatment if extreme obesity and over 12 or complications of obesity, after lifestyle changes - orlistat (lipase inhibitor), metformin (increases insulin sensitivity)
Bariatric surgery e.g. gastric banding

218
Q

What is respiratory distress syndrome?

A

RDS - deficiency of surfactant which lowers surface tension in the lungs, more common with increasingly early birth

219
Q

Surfactant physiology?

A

Mixture of phospholipids and proteins
Excreted by type II pneumocytes of the alveoli
Deficiency leads to alveolar collapse and inadequate gas exchange

220
Q

Prevention of RDS?

A

Give mother glucocorticoids if preterm delivery anticipated

221
Q

Signs/symptoms of RDS? (4)

A 
Tachypnoea >60bpm
Laboured breathing, chest recession, nasal flaring
Expiratory grunting
Cyanosis
Within 4hr of birth
222
Q

What is shown on X ray in RDS?

A

Bilateral pneumothorax

223
Q

Treatment of RDS? (2)

A

Oxygen - possibly CPAP, mechanical ventilation

Surfactant therapy into lung via tracheal tube

224
Q

What is bronchopulmonary dysplasia?

A

Infants who still have oxygen requirement at 36 weeks post gestation - chronic lung disease

225
Q

Cause of bronchopulmonary dysplasia? (3)

A

Pressure/volume trauma from artificial ventilation
Oxygen toxicity
Infection

226
Q

Treatment of bronchopulmonary dysplasia?

A

CPAP oxygen at home

Short course corticosteroids for earlier ventilation weaning

227
Q

What is meconium aspiration?

A

Meconium inhaled by the infant at birth, particularly if prolonged gestation or fetal hypoxia due to gasping

228
Q

Complications of meconium aspiration? (4)

A

Meconium is a lung irritant
Results in mechanical obstruction and chemical pneumonitis
Predisposes to infection
High incidence of air leak - pneumothorax
Persistent pulmonary hypertension of the newborn

229
Q

Management of meconium aspiration? (2)

A

Suction airways

Artificial ventilation

230
Q

What are the TORCH infections?

A 
Toxoplasmosis
Other - syphilis, VZV, parvovirus
Rubella
Cytomegalovirus
Herpes
231
Q

What are the TORCH infections associated with?

A

Common viruses associated with congenital anomalies

232
Q

What is hypoxic-ischaemic encephalopathy?

A

Hypoxic-ischaemic injury to the brain due to perinatal asphyxia compromising gas exchange and cardiac output, causing brain damage or death

233
Q

Causes of HIE? (5)

A

Placental exchange failure - prolonged contractions, abruption, ruptured uterus
Interruption of umbilical blood flow - shoulder dystocia, cord prolapse
Maternal hypo/hypertension
IUGR, fetal anaemia
Failure of cardiorespiratory adaptation at birth

234
Q

Symptoms of HIE?

A

Mild - irritable, excessive response to stimulation, impaired feeding, hyperventilation
Moderate - marked abnormalities of tone and movement, cannot feed
Severe - seizures, no normal movements or response to pain, hypo and hypertonic limbs, multi organ failure

235
Q

Management of HIE? (6)

A 
Respiratory support
EEG 
Anticonvulsants
Fluid restriction 
Treat electrolyte imbalances
Cooling if within 6hr of birth
236
Q

Prognosis of HIE?

A

Complete recovery if mild
If abnormalities persist over 2 weeks, full recovery unlikely
Severe has mortality of 30-40% and 80% have disability such as cerebral palsy

237
Q

What is necrotising enterocolitis?

A

Mostly affects preterm infants, associated with bacterial invasion of ischaemic bowel wall, more likely if fed cows milk formula

238
Q

Symptoms of NEC? (6)

A 
Infant stops tolerating feeds
Milk aspiration from stomach
Vomiting may be bile stained
Distended abdomen
Fresh blood in stool
Shock
239
Q

What is shown on X ray in NEC? (4)

A

Distended loops of bowel
Thickening of bowel wall with intramural gas
Air in hepatic portal tract
Air under diaphragm if bowel perforation

240
Q

Management of NEC? (4)

A

Stop feeding orally - parenteral
Broad spectrum antibiotics
Artificial ventilation, circulatory support
Surgery if bowel perforation - resection

241
Q

How is newborn listeria monocytogenes transmitted?

A

To the mother from food such as unpasteurised milk, soft cheese, undercooked poultry

242
Q

Symptoms of listeria monocytogenes infection in the newborn? (8)

A 
Spontaneous abortion
Preterm delivery
Sepsis
Meconium stained liquor
Widespread rash
Pneumonia
Meningitis
243
Q

When is hypoglycaemia in a newborn more likely? (7)

A 
IUGR
Preterm
Mother has diabetes mellitus
Large baby
Hypothermic
Polycythaemic
Ill for any reason
244
Q

Why do diabetic mothers have hypoglycaemic babies?

A

Sufficient glycogen stores (as opposed to preterm/IUGR infants) but hyperplasia of pancreatic islet cells causes high insulin levels

245
Q

Symptoms of hypoglycaemia in the newborn? (6)

A 
Jittery
Irritable
Apnoea
Lethargy
Drowsiness
Seizures
246
Q

Management of newborn hypoglycaemia?

A

Early frequent milk feeding

IV glucose if very low

247
Q

What causes cleft lip or palate?

A

Cleft lip - failure of fusion of the frontonasal and maxillary processes
Cleft palate - failure of fusion of the palatine processes and nasal septum

248
Q

Management of cleft lip/palate? (2)

A

Surgery in first few weeks/months

Special teats/feeding devices

3A REVISION (17 decks)

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