Paeds renal, endocrine and reproductive Flashcards
What is nephrotic syndrome?
Leaking protein from a damaged glomerulus
What is the triad of symptoms for nephrotic syndrome?
Heavy proteinuria
Oedema
Hypoalbulminaemia
What are the other less common symptoms of nephrotic syndrome?
Periorbital oedema on waking
Breathlessness due to pleural effusions and abdo distension
What causes the heavy proteinuria in nephrotic syndrome?
Damaged podocytes and glomerulus
At what age is nephrotic syndrome most common in children?
Around 4 years
Why is there oedema in nephrotic syndrome?
Loss of protein from the circulation causes water to move from the circulation into the surrounding tissues causing oedema.
WHat investigations should be carried out in nephrotic syndrome?
Urine dip (rule out infection, test for protein)
Urinary protein: creatinine ratio
Bloods
Serum albulmin (low)
What is the management of nephrotic syndrome in children?
High dose steroids for 4 weeks
Then wean and reduce dose
Check urine protein everyday
What is minimal change disease?
A cause of nephrotic syndrome
What happens to the kidney in minimal change disease?
Flattened podocytes
What is the most common cause of nephrotic syndrome in children?
Minimal change disease
When do symptoms start with congenital nephrotic syndrome?
Between birth and 3 months
What is the prognosis for congenital nephrotic syndrome?
Much rarer and leads to irreversible kidney failure
What is the difference between nephrotic and nephritic syndrome?
Nephritic is damage to the capillaries
Nephrotic is damage to the glomerulus and podocytes
What are the clinical features of nephritic syndrome?
Frank haematuria (much more than nephrotic)
Oedema (lesser extent than nephrotic)
Reduced urine output
Hypertension
Name 4 causes of nephritic syndrome?
Post infection (strep infections)
Vasculitis
IgA nephropathy
Anti-glomerular basement membrane disease
What is the pathophysiology of nephritic syndrome?
Inflammation and damage to the capillaries in the glomerulus
Causes a leakage of blood into the renal tubules
What do you see on urine dipstick in nephritic syndrome?
Blood +++
Proteinuria +
On microscopy of the urine of someone with nephritic syndrome what would you see?
Red cell casts
When does post strep nephritis develop?
1-2weeks post sore throat or skin infection
What is the management of post strep nephritis?
Antibiotics to treat strep infection
What is HSP?
Henoch schonlein purpura
What are the clinical features of HSP?
Skin rash on the buttocks, extensor surfaces of arms and legs
Joint pain
Renal symptoms such as haematuria and proteinuria
What type of nephritis are patients with SLE at risk of developing?
Lupus nephritis
How is lupus nephritis treated?
With corticosteroids
What is Alport syndrome?
An inherited form of nephritis
Characteritic signs of alport syndrome?
Haematuria
Proteinuria
Hypertension
Oedema
What is the most common causative organism of UTI’s in children?
E.coli
Klebsiella
From what age do febrile seizures occur?
After 6 months
Symptoms of a UTI in infants?
Fever
Vomiting
Offensive urine
Lethargy/irritability
Poor feeding
Symptoms of a UTI in children?
Dysuria
Frequency
Urgency
Abdo pain or loin tenderness
When is acute pyelonephritis considered instead of a simple UTI?
Temp above 38C with bacteruria
Temp lower than 38C with loin pain and bacteruria
What are the recommended investigations for a UTI?
Clean catch urine sample
Suprapubic sample in very unwell children
How is a UTI managed in infants under 3 months?
2-4 days IV Abx followed by oral
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
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
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
Symptoms of pyelonephritis?
Fever
Pain in R/L iliac fossa radiating to back
N&V
Dysuria
What age on average does puberty begin in girls?
10.5
What is the definition of precocious puberty in girls?
Onset before the age of 8
What is the definition of late onset puberty in girls?
14
What is adrenarche?
Increased production of androgens, leading to:
Acne
Sweating
Hair growth
Body odour
What is thelarche?
Breast bud development
First feature of puberty
What is menarche?
When menstruation begins
Coincides with stage 3 of breast development
What are the 4 stages of testicular development?
Testicular enlargement
Increased pigmentation
Scrotal thickening
Penile growth and thickening
What is the definition of true precocious puberty?
Early activation of the hypothalamic pituitary axis
Name 4 causes of true precocious puberty?
Hydrocephalus
NF
Post sepsis
Brain tumours
What is false precocious puberty?
Gonadotrophin independent. Usually presents with the isolated development of one pubertal characteristic.
Name 3 causes of false precocious puberty?
Increased adrenal activity
Gonadal tumour
Hypothyroidism
If the testes haven’t descended at birth, usually they will descend by what age?
3 months
What ages is testicular torsion most common?
12-25
What are the risk factors for testicular torsion?
Previous testicular torsion
Family history
Undescended testes
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
What is the management of testicular torsion?
Surgery to untwist the spermatic cord and fix the testes in place within the scrotum.
What is hypospadias?
A congenital abnormality that causes the urethral meatus to be located at an abnormal site on the underside of the penis.
What are the clinical features of hypospadias?
Ventral opening of the urethral meatus
Ventral curvature of penis
Hooded foreskin
What investigations would you do in the case of hypospadias?
Karotype
Pelvic USS
U&E
Testosterone, LH, FSH, ACTH
What is the management of hypospadias?
Urethroplasty
Congenital adrenal hyperplasia is deficiency of what enzyme?
21 hydroxylase enzyme
What are the consequences of 21 hydroxylase enzyme deficiency?
Underproduction of cortisol and aldosterone
Over production of androgens
What is the role of glucocorticoids (cortisol) in the body?
Raise blood glucose
Reduce inflammation
Supress the immune system?
What is the role of mineralocorticoids in the body?
Act on the kidney to control the balance of salts and water in the blood
WHat is the action of aldosterone?
Acts to decrease potassium and increase sodium in the blood
What endocrine changes are seen in CAH?
Hyperkalaemia
Hyponatraemia
Hypoglycaemia
Metabolic acidosis
What signs are seen in females with CAH?
Clitoral hypertrophy
Fused labia
What signs are seen in males with CAH?
Enlarged penis
Pigmented scrotum
How is CAH managed?
Cortisol replacement with hydrocortisone
Aldosterone replacement with fludrocortisone
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
What is Kallman syndrome?
Genetic syndrome resulting in hypogonadotropic hypogonadism, and therefore failure to start puberty
Associated with absent sense of smell
Name 3 causes of congenital hypothyroidism?
Maldescent of thyroid
Dyshormonogenesis (permanent thyroid hormone deficiency)
Iodine deficiency
Which test is used to detect thyroid problems in newborns?
The guthrie test
5 clinical feature of hypothyroidism in newborns.
Faltering growth
Feeding difficulty
Prolonged jaundice
Constipation
Pale, mottled, cold skin
