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Paediatric Nephrology and Urology

Paediatric Nephrology and Urology. Contents. Balanitis Hypospadias Urinary Tract Infections (Big) Vesico -Ureteric Reflux Renal Scarring Haemolytic Uraemic Syndrome Nocturnal Enuresis Horse-shoe kidney Wilm’s Tumour Nephrotic Syndrome Minimal Change Disease

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Paediatric Nephrology and Urology

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  1. Paediatric Nephrology and Urology

  2. Contents • Balanitis • Hypospadias • Urinary Tract Infections (Big) • Vesico-Ureteric Reflux • Renal Scarring • Haemolytic Uraemic Syndrome • Nocturnal Enuresis • Horse-shoe kidney • Wilm’s Tumour • Nephrotic Syndrome • Minimal Change Disease • Henoch-Schonlein purpura • Nephritic Syndrome • IgA nephropathy • Post-streptococcal glomerulitis

  3. Balanitis • Inflammation of the glans penis • Common in young boys with non-retractable foreskin • The most common cause in children is non-specific dermatitis, due to poor hygiene and build up of smegma. • Presentation: • Irritation/pain • Discharge beneath the foreskin • Inflammation • Recurrent balanitis can cause phimosis, leading to problems with micturition • Management • Advise – Avoid contact with irritants, daily cleaning with warm water/saline • Topical Hydrocortisone 1% OD + imidazole cream for up to 14 days • Oral Flucloxacillin (if bacterial balanitis)

  4. Hypospadias • Congenital disorder of the urethra • Urethral meatus is not at the usual location at the head of the penis • In most cases urethral meatus is on or near the glans of the penis • Others have meatus near or within the scrotum • Affects 1/250 males at birth • Diagnosed at birth through examination • Important to exclude disorders of sexual development • Many require surgery due to functional or cosmetic indications • Surgery is usually performed between 6-18 months

  5. Urinary Tract Infection – Intro • Infection of any part of the urinary tract • Lower = typically bladder/urethra • Upper = typically kidneys/ureters – systemic symptoms (such as fever), or loin pain/tenderness • E. coli is most common cause • Typical and Atypical • Atypical much more worrying

  6. Urinary Tract Infections - Recognising • Younger than 3 months • Fever, vomiting, lethargy, irritability • Poor feeding, failure to thrive • Abdo pain, jaundice, haematuria, offensive urine • Older than 3 months, preverbal • Fever • Abdo pain, loin tenderness, vomiting, poor feeding • Lethargy, irritability, haematuria, offensive urine, failure to thrive • Older than 3 months, verbal • Frequency, Dysuria • Changes to continence, abdo pain, loin tenderness • Fever, malaise, vomiting, haematuria, offensive urine, cloudy urine

  7. Urinary Tract Infection - Diagnosis • Clean catch urine sample recommended (mid-stream) • Younger than 3 years: • Microscopy and culture is preferred method for diagnosing UTI in this group • Older than 3 years: • Dipstick testing for leukocyte esterase and nitrites is just as useful as microscopy and culture in this group • +veLeuk, +veNitr = treat as UTI and send for culture • -veLeuk, +veNitr = Treat as UTI and send for culture • +veLeuk, -veNitr = treat as UTI if urinary symptoms. Send for microscopy and culture • -veLeuk, -veNitr = not a UTI, no culture • Basically, Nitrites are a good indicator of UTI, leukocytes not so much

  8. UTIs and Risk Factors for Serious Underlying Cause • Poor Urine Flow • Previous UTIs • Recurrent fever of unknown origin • Known renal abnormality • Family history of Vesico-ureteric reflux or renal disease • Constipation • Dysfunctional voiding (hard to define? Sounds like a full bladder after voiding) • Enlarged bladder • Abdo mass • Evidence of Spinal Lesion • Poor Growth • High Blood Pressure

  9. UTI – treatment • Serious illness – urgent referral to paediatric specialist • Younger than 3 months with possible UTI – urgent referral to paediatric specialist • Older than 3 months with lower UTI – 3 days of Abx(trimethoprim, nitrofurantoin, amoxicillin, cephalosporin) • Older than 3 months with upper UTI – consider referral, 7-10 days of Abx (cephalosporin or co-amoxiclav) • Always follow local Abx guidelines

  10. UTI – follow-up • Address risk factors and constipation • Encourage good fluid intake • Encourage regular access and use of clean toilets (don’t delay voiding at school!) • Recurrent UTI – can sometimes give antibiotic prophylaxis

  11. Atypical UTIs • Any of the following • Seriously ill • Poor urine flow • Abdominal or bladder mass • Raised creatinine • Septicaemia • Failure to respond to suitable abx within 48 hours • Non E.coli infection

  12. Recurrent UTIs • Two or more episodes of upper UTI • One upper UTI + one lower UTI • Three or more lower UTIs

  13. Imaging Tests for UTIs - Ultrasounds • Ultrasound of the urinary tract to identify structural abnormalities of the urinary tract (such as obstruction) • Can give during acute illness • Some indications: • Atypical UTIs • Recurrent UTIs • Not normally given if not atypical/recurrent • However if younger than 6 months, ultrasound within 6 weeks

  14. Imaging Test for UTIs - MCUG • MicturatingCystourethrogram (or voiding – VCUG) • Catheterise  fill bladder with radiocontrast agent  real-time x-rays to watch fluid and urinary anatomy • Appears to be best test to diagnose Vesico-Ureteric Reflux • Prophylactic abx should be given • Should be used in under 6 months old with atypical or recurrent UTIs • Seems they don’t like to perform MCUG as much after this age

  15. Imaging Test for UTIs – DMSA scans • Radionuclide scan following IV injection of a radioactive chemical that goes to the kidney • Appears to be best for diagnosing renal-scarring • Performed 4-6 months after UTI • Often performed in atypical/recurrent UTIs

  16. Vesico-Ureteric Reflux (VUR) • Retrograde flow of urine from bladder to ureters • More common in children, something to do with the ureters entering the bladder at less oblique angles • Increases risk of UTIs • Can damage upper urinary tract (more pressure?) and lead to renal scarring • Staged from 1 – 4 • Stage 1 – reflux into a non-dilated ureter • Stage 4 – dilatation of the ureter and kidneys in general • Many imaging techniques can pick up on it – but it seems that MCUG is the best at picking it up

  17. Renal Scarring • Often caused by UTIs and VUR • Leads to kidney injury, decreased renal function, proteinuria, hypertension and even end-stage renal disease • DMSA scan appears to be the best at diagnosing

  18. Haemolytic Uraemic Syndrome • Triad • Haemolytic Anaemia • AKI • Thrombocytopenia • Mostly affects children • Most cases preceded by an acute diarrhoea (sometimes bloody) caused by E. coli 0157 • Produces ‘shiga-like toxin’ • Other bacterial causes too. • Test for this bacteria with culture, PCR, serology, antibody tests • Treatment – Unclear? Sounds like renal replacement therapy? Refer to specialists/ITU

  19. Nocturnal Enuresis

  20. Nocturnal Enuresis Overview • Enuresis = bedwetting • Definition – involuntary wetting during sleep, at least twice a week, in children older than 5 years or age with no congenital or acquired defects of the CNS • Generally considered normal in children younger than 5 years of age • Associated with family history • Affects 15-20% of 5 year olds, and 5% of 10 year olds • Most become continent by adolescence

  21. Nocturnal Enuresis - Classification • Primary bedwetting without daytime symptoms • Sleep arousal difficulties, polyuria, bladder dysfunction • Primary bedwetting with daytime symptoms – never achieved continence at night and has daytime symptoms (urgency, frequency, daytime wetting etc.) • Overactive bladder, urinary tract abnormalities, neurological disorders, chronic constipation, UTI • Secondary bedwetting – had night-time continence for more than 6 months, and now doesn’t • Diabetes, UTI, constipation, psychological problems, family problems

  22. Nocturnal Enuresis - Assessment • Assess for classification, as each has different causes • Determine expectation (short, or long term treatment expected?) • Consider assessing for constipation • Pattern of bedwetting • Fluid intake assessment • Assess home situation – easy access to toilet • Urinary history • GI history

  23. CONSIDER CHILD MALTREATMENT • Child reported to deliberately wet the bed? • Parents/carers punishing child for bedwetting • If secondary enuresis not improving despite treatment

  24. Primary Without daytime symptoms- treatment • Reassure parents that is common and usually resolves • Consider diary of fluid intake, bedwetting and toileting patterns • Ensure easy access to toilet • Encourage to empty bladder before sleep • Encourage a positive reward system for helpful behaviour • Consider enuresis alarm (first line long term treatment) • Desmopressin may be considered if alarm not ideal • Can be considered for short term treatment such as sleepovers • Other drugs like oxybutynin and tricyclic antidepressants can be considered later on

  25. Primary With daytime symptoms - treatment • Refer all to a secondary enuresis clinic for further investigations and assessment • (If older than 2 years old)

  26. Secondary - treatment • Manage underlying causes (UTIs, constipation) if present • Some underlying causes need referring • (Diabetes, recurrent UTI, psychological problems, family problems, developmental problems, neurological problems)

  27. General Treatment • Advice and reassurance • Positive reward system always used • Enuresis alarm first line • More commonly used if older than 7 • Desmopressin afterwards • More commonly used if older than 7 • Generally only treat if older than 5

  28. Kidney Disorders

  29. Horse-Shoe Kidney • Born with kidneys fused together • Can be asymptomatic • May experience nausea, abdominal pain, kidney stones, and UTIs • Appears to be seen best on DMSA scan • No curative treatment appears to exist • Most treatment is managing the complications mentioned above

  30. Wilms’ Tumour (Nephroblastoma) • The most common renal malignancy in children (normally below the age of 5) • In around third of cases it is associated with a loss of function in the WT1 gene • Presents with unilateral, painless, abdominal/flank mass • +/- haematuria • Often metastasises early – Usually to the lung • Consider urgent referral (within 48 hours) for an assessment for Wilms’ tumour in children with any the following: • Palpable abdominal mass • Unexplained enlarged abdominal organ • Unexplained visible haematuria

  31. Wilms’ Tumour (Nephroblastoma) • Investigations include • FBC • U&E • Urinalysis • Abdominal USS (sounds like first line imaging) • Sounds like they like to perform Chest X-rays too • CT/MRI Chest/Abdomen • Management • Chemotherapy • Radiotherapy • Surgery – Nephrectomy

  32. Nephrotic and Nephritic Syndromes Ben Ryan

  33. Quick Summary • Nephrotic and Nephritic Syndrome are two syndromes typically caused by damage to the glomeruli • Nephrotic syndrome occurs when damage to the glomeruli causes it to become leaky, so proteins to filter into the urine • Nephritic syndrome occurs when there is inflammation of the glomeruli, leading to blood in the urine • In reality, these syndromes are sort of on a spectrum, and conditions causing nephritic syndrome can also cause nephrotic syndrome • There can be mixed pictures of the two

  34. Nephrotic Syndrome • Nephrotic syndrome is classified as: • Proteinuria • Hypoalbuminaemia • Oedema • But also be aware of hyperlipidaemia due to liver making more lipoproteins • hypercoagulable state due to loss of antithrombin III • Increased risk of infections due to loss of immunoglobulins • Around 80% of cases are due to minimal change glomerulonephritis • Other Causes include: • Henoch Schonlein purpura • Glomerulosclerosis • Glomerulonephritis • Systemic Lupus Erythematous • Diabetes • Sickle Cell Anaemia • Leukaemia, multiple myeloma, lymphoma (rare!)

  35. Minimal Change Disease • Nearly Always Presents as nephrotic syndrome • Occurs when T-cells and cytokines cause damage to the glomerular basement membrane, leading to increased permeability to albumin. • Most cases are idiopathic, but sometimes can be associated with: • NSAIDs, Rifampacin • Hodgkin’s Lymphoma • Infectious Mononucleosis • Management • Corticosteroids – 80% respond to steroids (cyclophosphamide if not) • Fluid Restriction/Low salt diet • Prognosis • 1/3 have just one episode • 1/3 have infrequent relapses • 1/3 have frequent relapses which stop before adulthood

  36. Henoch Schonlein Purpura • The most common vasculitis in childhood • Most cases are self-limiting • Normally preceded by an URTI • Presentation • Purpuric Rash – Classical purpuric rash distributed symmetrically along the lower limbs, extensor surfaces and buttocks predominantly. • Joints – Pain/swelling/reduced ROM of the larger joints • GI – Colicky abdominal pain, N&V • Renal - Microscopic haematuria, nephrotic syndrome • Management • Generally supportive with simple analgesia • Continued monitoring for signs of HSP nephritis (urine dip) – Usually present within 6 months

  37. Nephritic Syndrome • Syndrome consisting of: • Haematuria • Hypertension • Proteinuria also • Caused by glomerular inflammation • Some underlying causes include: • Post-streptococcal glomerulonephritis • IgA nephropathy

  38. Post-Streptococcal Glomerulonephritis • Typically occurs 7-14 days after a Group A beta-haemolytic Streptococcus infection (usually Streptococcus pyogenes) • Typically skin infections • Young children most commonly affected • In a nephrotic syndrome picture, it is good to ask about recent infections and when they were • Is a cause of nephritic syndrome (as well as nephrotic) • Kidney biopsy would likely give diagnosis • Complement profile may also help

  39. IgA Nephropathy (Berger’s Disease) • Typically develops 1-2 days after a non-specific URTI • IgA antibodies get deposited in the glomerulus • Similar to Henoch Schonlein Purpura (HSP) (IgA vasculitis) • Typically presents in young males • Many don’t receive treatment • Unclear which treatments help • Biopsy can be used for diagnosis • Complement levels may help also

  40. Haematuria in a child • Rule out wilm’s tumour – other features? USS? • Imaging can also help rule out kidney stones and identify source of bleeding • If no source of bleeding, nephritic syndrome? • 1-2 days after URTI – IgA nephropathy • 2 weeks after infection (possibly skin, or URTI) – post-streptococcal glomerulonephritis

  41. MCQ Central

  42. MCQ 1 • A 5 year old child presents with a history of 3 day constipation. Normally he has a bowel movement every day which is described as normal by his parents. There are no urinary symptoms however the urine dipstick was ++ for blood. His last appointment was 2 months ago for what sounded like a viral URTI. On abdominal examination there was a palpable mass in the left lumbar region. • Which of the following is your most important next step? • A – Refer urgently to paediatrics • B – Give advice about fluid intake and diet • C – Renal Biopsy • D – Prescribe Movicol as appropriate for constipation • E – Prescribe a 3 day course of trimethoprime

  43. MCQ 1 • A 5 year old child presents with a history of 3 day constipation. Normally he has a bowel movement every day which is described as normal by his parents. There are no urinary symptoms however the urine dipstick was ++ for blood. His last appointment was 2 months ago for what sounded like a viral URTI. On abdominal examination there was a large palpable mass in the left lumbar region. • Which of the following is your most important next step? • A – Refer urgently to paediatrics – painless abdominal mass and haematuria – rule out Wilm’s tumour. • B – Give advice about fluid intake and diet • C – Renal Biopsy • D – Prescribe Movicol as appropriate for constipation • E – Prescribe a 3 day course of trimethoprime

  44. MCQ 2 • A 5 month old female has had 4 lower UTIs over the past 2 months. Which of the following investigations will best diagnose vesico-ureteric reflux? • A – Urinary Tract USS • B – DMSA • C – MCUG • D – Renal Biopsy • E – Urine microscopy

  45. MCQ 2 • A 5 month old female has had 4 lower UTIs over the past 2 months. Which of the following investigations will best diagnose vesico-ureteric reflux? • A – Urinary Tract USS • B – DMSA • C – MCUG – appears to be best at diagnosing VUR, however it doesn’t appear to be used much after the age of 6 months. Ask the paediatric consultants when they would stop using MCUG and what they would use instead. • D – Renal Biopsy • E – Urine microscopy

  46. MCQ 3 • A 5 month old female has had 4 lower UTIs over the past 2 months. Which of the following investigations will best diagnose renal scarring? • A – Urinary Tract USS • B – DMSA – appears to be best at diagnosing renal scarring • C – MCUG • D – Renal Biopsy • E – Urine microscopy

  47. MCQ 4 • A 9 year old child presents with a 2 day history of malaise and swelling in his legs. Initial tests confirm proteinuria and hypoalbuminaemia. Which of the following is the most likely underlying cause? • A – Post-streptococcal glomerulonephritis • B – Systemic Lupus Erythematous • C – IgA Nephropathy • D – Minimal Change Disease • E – Henoch Schonlein Purpura

  48. MCQ 4 • A 9 year old child presents with a 2 day history of malaise and swelling in his legs. Initial tests confirm proteinuria and hypoalbuminaemia. Which of the following is the most likely underlying cause? • A – Post-streptococcal glomerulonephritis • B – Systemic Lupus Erythematous • C – IgA Nephropathy • D – Minimal Change Disease – most common cause of nephrotic syndrome in kids • E – Henoch Schonlein Purpura

  49. MCQ 5 • A 9 year old child presents with a 2 day history of malaise and lethargy. Initial tests confirm haematuria and mild hypertension. On further questioning, he had impetigo a fortnight ago which was successfully treated by his GP. Which of the following is the most likely underlying cause? • A – Post-streptococcal glomerulonephritis • B – Systemic Lupus Erythematous • C – IgA Nephropathy • D – Minimal Change Disease • E – Henoch Schonlein Purpura

  50. MCQ 5 • A 9 year old child presents with a 2 day history of malaise and lethargy. Initial tests confirm haematuria and mild hypertension. On further questioning, he had impetigo a fortnight ago which was successfully treated by his GP. Which of the following is the most likely underlying cause? • A – Post-streptococcal glomerulonephritis – 2 weeks after streptococcal infection – impetigo is most commonly caused by Staph A or Strep pyogenes. • B – Systemic Lupus Erythematous • C – IgA Nephropathy • D – Minimal Change Disease • E – Henoch Schonlein Purpura

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