Paeds renal, endocrine and reproductive Flashcards

1
Q

What is nephrotic syndrome?

A

Leaking protein from a damaged glomerulus

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2
Q

What is the triad of symptoms for nephrotic syndrome?

A

Heavy proteinuria
Oedema
Hypoalbulminaemia

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3
Q

What are the other less common symptoms of nephrotic syndrome?

A

Periorbital oedema on waking
Breathlessness due to pleural effusions and abdo distension

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4
Q

What causes the heavy proteinuria in nephrotic syndrome?

A

Damaged podocytes and glomerulus

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5
Q

At what age is nephrotic syndrome most common in children?

A

Around 4 years

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6
Q

Why is there oedema in nephrotic syndrome?

A

Loss of protein from the circulation causes water to move from the circulation into the surrounding tissues causing oedema.

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7
Q

WHat investigations should be carried out in nephrotic syndrome?

A

Urine dip (rule out infection, test for protein)
Urinary protein: creatinine ratio
Bloods
Serum albulmin (low)

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8
Q

What is the management of nephrotic syndrome in children?

A

High dose steroids for 4 weeks
Then wean and reduce dose
Check urine protein everyday

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9
Q

What is minimal change disease?

A

A cause of nephrotic syndrome

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10
Q

What happens to the kidney in minimal change disease?

A

Flattened podocytes

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11
Q

What is the most common cause of nephrotic syndrome in children?

A

Minimal change disease

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12
Q

When do symptoms start with congenital nephrotic syndrome?

A

Between birth and 3 months

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13
Q

What is the prognosis for congenital nephrotic syndrome?

A

Much rarer and leads to irreversible kidney failure

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14
Q

What is the difference between nephrotic and nephritic syndrome?

A

Nephritic is damage to the capillaries
Nephrotic is damage to the glomerulus and podocytes

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15
Q

What are the clinical features of nephritic syndrome?

A

Frank haematuria (much more than nephrotic)
Oedema (lesser extent than nephrotic)
Reduced urine output
Hypertension

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16
Q

Name 4 causes of nephritic syndrome?

A

Post infection (strep infections)
Vasculitis
IgA nephropathy
Anti-glomerular basement membrane disease

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17
Q

What is the pathophysiology of nephritic syndrome?

A

Inflammation and damage to the capillaries in the glomerulus
Causes a leakage of blood into the renal tubules

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18
Q

What do you see on urine dipstick in nephritic syndrome?

A

Blood +++
Proteinuria +

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19
Q

On microscopy of the urine of someone with nephritic syndrome what would you see?

A

Red cell casts

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20
Q

When does post strep nephritis develop?

A

1-2weeks post sore throat or skin infection

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21
Q

What is the management of post strep nephritis?

A

Antibiotics to treat strep infection

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22
Q

What is HSP?

A

Henoch schonlein purpura

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23
Q

What are the clinical features of HSP?

A

Skin rash on the buttocks, extensor surfaces of arms and legs
Joint pain
Renal symptoms such as haematuria and proteinuria

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24
Q

What type of nephritis are patients with SLE at risk of developing?

A

Lupus nephritis

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25
How is lupus nephritis treated?
With corticosteroids
26
What is Alport syndrome?
An inherited form of nephritis
27
Characteritic signs of alport syndrome?
Haematuria Proteinuria Hypertension Oedema
28
What is the most common causative organism of UTI's in children?
E.coli Klebsiella
29
From what age do febrile seizures occur?
After 6 months
30
Symptoms of a UTI in infants?
Fever Vomiting Offensive urine Lethargy/irritability Poor feeding
31
Symptoms of a UTI in children?
Dysuria Frequency Urgency Abdo pain or loin tenderness
32
When is acute pyelonephritis considered instead of a simple UTI?
Temp above 38C with bacteruria Temp lower than 38C with loin pain and bacteruria
33
What are the recommended investigations for a UTI?
Clean catch urine sample Suprapubic sample in very unwell children
34
How is a UTI managed in infants under 3 months?
2-4 days IV Abx followed by oral
35
How is a UTI managed in systemically well children with no systemic features and absence of significant fever.
3 days oral antibiotics Advise to return if not better
36
How is a UTI managed in systemically unwell children with fever above 38C and loin pain?
7-10 days oral antibiotics Clinical judgement as to whether to give IV
37
What is vesicoureteric reflux?
Ureters are displaced at an angle, leading to reflux of the urine into the ureters and sometimes the kidney Increased risk of infection and recurrent UTI's
38
Symptoms of pyelonephritis?
Fever Pain in R/L iliac fossa radiating to back N&V Dysuria
39
What age on average does puberty begin in girls?
10.5
40
What is the definition of precocious puberty in girls?
Onset before the age of 8
41
What is the definition of late onset puberty in girls?
14
42
What is adrenarche?
Increased production of androgens, leading to: Acne Sweating Hair growth Body odour
43
What is thelarche?
Breast bud development First feature of puberty
44
What is menarche?
When menstruation begins Coincides with stage 3 of breast development
45
What are the 4 stages of testicular development?
Testicular enlargement Increased pigmentation Scrotal thickening Penile growth and thickening
46
What is the definition of true precocious puberty?
Early activation of the hypothalamic pituitary axis
47
Name 4 causes of true precocious puberty?
Hydrocephalus NF Post sepsis Brain tumours
48
What is false precocious puberty?
Gonadotrophin independent. Usually presents with the isolated development of one pubertal characteristic.
49
Name 3 causes of false precocious puberty?
Increased adrenal activity Gonadal tumour Hypothyroidism
50
If the testes haven't descended at birth, usually they will descend by what age?
3 months
51
What ages is testicular torsion most common?
12-25
52
What are the risk factors for testicular torsion?
Previous testicular torsion Family history Undescended testes
53
What are the clinical features of testicular torsion?
Sudden onset, severe unilateral testicular pain Associated with N&V related to the pain Referred abdo pain
54
What is the management of testicular torsion?
Surgery to untwist the spermatic cord and fix the testes in place within the scrotum.
55
What is hypospadias?
A congenital abnormality that causes the urethral meatus to be located at an abnormal site on the underside of the penis.
56
What are the clinical features of hypospadias?
Ventral opening of the urethral meatus Ventral curvature of penis Hooded foreskin
57
What investigations would you do in the case of hypospadias?
Karotype Pelvic USS U&E Testosterone, LH, FSH, ACTH
58
What is the management of hypospadias?
Urethroplasty
59
Congenital adrenal hyperplasia is deficiency of what enzyme?
21 hydroxylase enzyme
60
What are the consequences of 21 hydroxylase enzyme deficiency?
Underproduction of cortisol and aldosterone Over production of androgens
61
What is the role of glucocorticoids (cortisol) in the body?
Raise blood glucose Reduce inflammation Supress the immune system?
62
What is the role of mineralocorticoids in the body?
Act on the kidney to control the balance of salts and water in the blood
63
WHat is the action of aldosterone?
Acts to decrease potassium and increase sodium in the blood
64
What endocrine changes are seen in CAH?
Hyperkalaemia Hyponatraemia Hypoglycaemia Metabolic acidosis
65
What signs are seen in females with CAH?
Clitoral hypertrophy Fused labia
66
What signs are seen in males with CAH?
Enlarged penis Pigmented scrotum
67
How is CAH managed?
Cortisol replacement with hydrocortisone Aldosterone replacement with fludrocortisone
68
What is androgen insensitivity syndrome?
Males with the normal XY chromosomes however they are unable to respond to testosterone meaning their genitalia do not develop normally
69
What is Kallman syndrome?
Genetic syndrome resulting in hypogonadotropic hypogonadism, and therefore failure to start puberty Associated with absent sense of smell
70
Name 3 causes of congenital hypothyroidism?
Maldescent of thyroid Dyshormonogenesis (permanent thyroid hormone deficiency) Iodine deficiency
71
Which test is used to detect thyroid problems in newborns?
The guthrie test
72
5 clinical feature of hypothyroidism in newborns.
Faltering growth Feeding difficulty Prolonged jaundice Constipation Pale, mottled, cold skin