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
Q

How is lupus nephritis treated?

A

With corticosteroids

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26
Q

What is Alport syndrome?

A

An inherited form of nephritis

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27
Q

Characteritic signs of alport syndrome?

A

Haematuria
Proteinuria
Hypertension
Oedema

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28
Q

What is the most common causative organism of UTI’s in children?

A

E.coli
Klebsiella

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29
Q

From what age do febrile seizures occur?

A

After 6 months

30
Q

Symptoms of a UTI in infants?

A

Fever
Vomiting
Offensive urine
Lethargy/irritability
Poor feeding

31
Q

Symptoms of a UTI in children?

A

Dysuria
Frequency
Urgency
Abdo pain or loin tenderness

32
Q

When is acute pyelonephritis considered instead of a simple UTI?

A

Temp above 38C with bacteruria
Temp lower than 38C with loin pain and bacteruria

33
Q

What are the recommended investigations for a UTI?

A

Clean catch urine sample
Suprapubic sample in very unwell children

34
Q

How is a UTI managed in infants under 3 months?

A

2-4 days IV Abx followed by oral

35
Q

How is a UTI managed in systemically well children with no systemic features and absence of significant fever.

A

3 days oral antibiotics
Advise to return if not better

36
Q

How is a UTI managed in systemically unwell children with fever above 38C and loin pain?

A

7-10 days oral antibiotics
Clinical judgement as to whether to give IV

37
Q

What is vesicoureteric reflux?

A

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
Q

Symptoms of pyelonephritis?

A

Fever
Pain in R/L iliac fossa radiating to back
N&V
Dysuria

39
Q

What age on average does puberty begin in girls?

A

10.5

40
Q

What is the definition of precocious puberty in girls?

A

Onset before the age of 8

41
Q

What is the definition of late onset puberty in girls?

A

14

42
Q

What is adrenarche?

A

Increased production of androgens, leading to:
Acne
Sweating
Hair growth
Body odour

43
Q

What is thelarche?

A

Breast bud development
First feature of puberty

44
Q

What is menarche?

A

When menstruation begins
Coincides with stage 3 of breast development

45
Q

What are the 4 stages of testicular development?

A

Testicular enlargement
Increased pigmentation
Scrotal thickening
Penile growth and thickening

46
Q

What is the definition of true precocious puberty?

A

Early activation of the hypothalamic pituitary axis

47
Q

Name 4 causes of true precocious puberty?

A

Hydrocephalus
NF
Post sepsis
Brain tumours

48
Q

What is false precocious puberty?

A

Gonadotrophin independent. Usually presents with the isolated development of one pubertal characteristic.

49
Q

Name 3 causes of false precocious puberty?

A

Increased adrenal activity
Gonadal tumour
Hypothyroidism

50
Q

If the testes haven’t descended at birth, usually they will descend by what age?

A

3 months

51
Q

What ages is testicular torsion most common?

A

12-25

52
Q

What are the risk factors for testicular torsion?

A

Previous testicular torsion
Family history
Undescended testes

53
Q

What are the clinical features of testicular torsion?

A

Sudden onset, severe unilateral testicular pain
Associated with N&V related to the pain
Referred abdo pain

54
Q

What is the management of testicular torsion?

A

Surgery to untwist the spermatic cord and fix the testes in place within the scrotum.

55
Q

What is hypospadias?

A

A congenital abnormality that causes the urethral meatus to be located at an abnormal site on the underside of the penis.

56
Q

What are the clinical features of hypospadias?

A

Ventral opening of the urethral meatus
Ventral curvature of penis
Hooded foreskin

57
Q

What investigations would you do in the case of hypospadias?

A

Karotype
Pelvic USS
U&E
Testosterone, LH, FSH, ACTH

58
Q

What is the management of hypospadias?

A

Urethroplasty

59
Q

Congenital adrenal hyperplasia is deficiency of what enzyme?

A

21 hydroxylase enzyme

60
Q

What are the consequences of 21 hydroxylase enzyme deficiency?

A

Underproduction of cortisol and aldosterone
Over production of androgens

61
Q

What is the role of glucocorticoids (cortisol) in the body?

A

Raise blood glucose
Reduce inflammation
Supress the immune system?

62
Q

What is the role of mineralocorticoids in the body?

A

Act on the kidney to control the balance of salts and water in the blood

63
Q

WHat is the action of aldosterone?

A

Acts to decrease potassium and increase sodium in the blood

64
Q

What endocrine changes are seen in CAH?

A

Hyperkalaemia
Hyponatraemia
Hypoglycaemia
Metabolic acidosis

65
Q

What signs are seen in females with CAH?

A

Clitoral hypertrophy
Fused labia

66
Q

What signs are seen in males with CAH?

A

Enlarged penis
Pigmented scrotum

67
Q

How is CAH managed?

A

Cortisol replacement with hydrocortisone
Aldosterone replacement with fludrocortisone

68
Q

What is androgen insensitivity syndrome?

A

Males with the normal XY chromosomes however they are unable to respond to testosterone meaning their genitalia do not develop normally

69
Q

What is Kallman syndrome?

A

Genetic syndrome resulting in hypogonadotropic hypogonadism, and therefore failure to start puberty
Associated with absent sense of smell

70
Q

Name 3 causes of congenital hypothyroidism?

A

Maldescent of thyroid
Dyshormonogenesis (permanent thyroid hormone deficiency)
Iodine deficiency

71
Q

Which test is used to detect thyroid problems in newborns?

A

The guthrie test

72
Q

5 clinical feature of hypothyroidism in newborns.

A

Faltering growth
Feeding difficulty
Prolonged jaundice
Constipation
Pale, mottled, cold skin