Paeds Flashcards

1
Q

What happens if VSD uncorrected?

A

Eisenmenger’s syndrome - Left to right shunt leads to compensatory pulmonary vascular hypertrophy. This in turn causes pulmonary hypertension and shunt reversal (right to left). Pulmonary hypertension leads to CHF.

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2
Q

Are newborns with VSD cyanotic?

A

No - often become cyanotic at later age when oxygen demand increases (can be during exercise)

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3
Q

Presumed diagnosis of unwell child with unhealed burn

A

Toxic shock syndrome

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4
Q

Idiopathic pulmonary haemosiderosis features

A

First decade of life (mean 4.5) and more females. Alveolar capillary bleeding causes haemosiderin accumulation causing anaemia. May have haemoptisis. Immunosuppressants/steroids may work. May be linked to gluten sensitivity. May resolve sponateously

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5
Q

Conditions to exclude prior to Idiopathic Pulmonary Haemosiderosis diagnosis

A

Common causes alveolar haemorhage - Goodpastures and SLE

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6
Q

What is Neimann-Pick’s?

A

Glycogen storage disorder - can have lung manifestations - foamy macrophages on biopsy. Multi-system disease

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7
Q

What is vanishing lung syndrome?

A

Found in SLE and idiopathic giant bullous emphysema - common in young male smokers. Starts in upper lobes and lungs appear to be disappearing on X-ray. Chronic and progressive.

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8
Q

Intussusception features, investigation and management

A

Classically presents with slimy or jelly-like (red currant jelly) stools and palpable mass/fullness and abdo pain. Child described as drawing their legs up. Most common in 6-18 months old. Diagnose with ultrasound. Treat with air enema usually - surgery if unsuccessful. Often associated with viral gastroenteritis. Can cause vomiting (bile stained as small bowel).

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9
Q

Hirschsprung’s cause

A

Congenital distal bowel aganglionosis

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10
Q

Hirschsprung’s symptoms and demographics

A

Bowel obstruction - vomiting and not passing stools in very young neonates

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11
Q

Hirschsprung’s diagnosis and treatment

A

Diagnosed via rectal biopsy and treated via surgical removal of the affected part of bowel.

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12
Q

Pyloric stenosis features

A

Occurs within first 4 weeks of life. Projectile vomiting immediately after feeds. Diagnosed with ultrasound and treated with pyloromyotomy

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13
Q

Congenital Adrenal Hyperplasia

A

Autosomal recessive mutation in adrenal hormone/steroid enzymes. 95% secondary to 21-hydroxylase deficiency. They can’t produce cortisol so ACTH production goes up - adrenal hyperplasia and overactivity of steroid-producing cells. Produce excess androgens/oestrogens and sometimes mineralocorticoids. Women develop pcos, men have large penis and scortal pigmentation at birth. Can present hyponatraemia and hyperkalaemia. Can cause hypoglycaemia and hypotension too as involved in adrenaline synthesis. Replace hormones.

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14
Q

Usual presentation age for pyloric stenosis

A

2-4 weeks

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15
Q

Pyloric stenosis features

A

Projectile, non-bilious vomiting, hypochloraemia, hypokalaemia and metabolic acidosis, more commonly in males.

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16
Q

Tracheo-oesophageal fistula presentation

A

Often associated with oesophageal stenosis. presents with cough/choke during feeding and lrti’s. Symptoms can be subtle and not present until later in childhood.

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17
Q

Biliary atresia presentation and treatment

A

Conjugated bilirubinaemia. Surgical treatment with Kasai portoenterostomy

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18
Q

What is a child protection plan?

A

A plan drawn up by local authority to protect children at risk of significant harm. Aims to ensure safety of child, promote childs health and development and support family in looking after welfare of child

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19
Q

Long term complications of ASD

A

Right atrial enlargement and pulmonary trunk dilation. Can present fatigue and exertional dyspnoea

20
Q

Teratogenic effect of lithium during pregnancy.

A

Ebsteins anomaly - poor right ventricular function. Can lead to wolff-parkinson white

21
Q

Age at which hand preference developed

22
Q

Age at which inability to walk becomes red flag

A

18 months - potentially cerebral palsy, muscular dystrophy or other developmental issue

23
Q

Age by which baby should be able to sit unsupported

24
Q

Age at which should be able to stand on one leg

25
Which defects comprise tetralogy of fallot
1. Right ventricular hypertrophy 2. VSD 3. Right sided outflow tract obstruction (pulmonary valve stenosis) 4. Overriding aorta
26
Most common cause of congenital cyanotic heart disease
Tetralogy of fallot
27
If cyanosis is present at birth is tetralogy of fallot or transposition of the great vessels likely the causative defect?
Transposition of the great vessels
28
Clotting factor affected by haemophilia A
Factor 8
29
Treatment of significant bleed in haemophilia A
Recombinant factor 8
30
When is desmopressin used in acute bleeds in haemophilia
Mild-moderate - not severe
31
Clotting factor affected in haemophilia b
Factor 9
32
Common complications of Down's
Congenital heart disease (AVSD), anal atresia, duodenal atresia and increased leukemia risk
33
What is myelomeningocele?
Complex spinal anomaly due to failure of dorsal neural fold fusion. Present with skin defects, lower limb paralysis, sensory loss and bladder and bowel dysfunction. Usually have Chiari ii malformations
34
Features of Edwards syndrome
Congenital heart disease, central nervous system abnormalities, IUGR, rocker bottom feet, single umbilical artery and facial abnormalities
35
Hyper IgE features | aka Buckley or Job syndrome
Recurrent respiratory infections, chronic eczematoid eruptions, Cold abscesses and mucocutaneous candidiasis. Strange teeth and coarse facial features
36
Low CD4 count associations
Advanced HIV, viral infections, pregnancy, malnutrition, TB, drug abuse and severe sepsis
37
Febrile convulsion red flags for urgent admission
``` Children <18months Diagnostic uncertainty Convulsion >15mins Focal features Convulsion recurring within same illness or 24 hours Incomplete recovery after 1 hour No focus of infection identified Exam findings suggest serious cause of fever eg pneumonia Child on/or been on antibiotics ```
38
Are patients at risk of infective endocarditis after VSD repair?
No
39
Up to what age is spontaneous VSD closure common?
2 years old
40
Symptoms that cause suspicion of appendicitis in children
Abdo pain, anorexia, vomiting and low grade fever
41
Initial management if appendicitis expected
Admit, Iv fluids, nil by mouth and surgical review
42
What are Auer rods?
Needle like bodies in the cytoplasm of myeloblast and/orproganulocytes in leukemia
43
What is factitious hypoglycaemia?
A deliberate attempt to induce hypoglycaemia - often via insulin or oral hypoglycaemic agents. C-peptide will be low, glucose low and insulin high
44
Typical age of ASD/VSD manifestation?
Childhood or adulthood - not infancy
45
Encephalitis symptoms
Altered behavious/encephalopathy, decreased consiousness, focal neurology/seizure on a background of viral prodrome of fever and lethargy
46
Main causes of viral encephalitis - children/adults and neonates
Children/adults - HSV-1 | Neonates - HSV-2 from the genital tract during birth