Renal Flashcards

Question

What is WILMS TUMOUR / NEPHROBLASTOMA ?

Answer 1

Wilms tumour originates from embryonal renal tissue | and is the most common renal tumour of childhood.

Answer 2

Over 80% of patients present before 5 years of age and | it is very rarely seen after 10 years of age.

Answer 3

Haematuria - BRIGHT RED BLOOD -> usually only presenting sx (so initially they thinks it’s UTI) Abdominal mass - incindental find usually ``` Uncommon: Abdominal / Flank pain Anaemia (haemorrhage into mass) Hypertension Anorexia ``` 5% bilateral

Answer 4

Initially (what I saw on wards): • Urinalysis - rule out UTI, gross or microscopic haematouria •US kidney or IV pyelogram +/- renal angiography • CT or MRI : - (staging) - shows an intrinsic renal mass distorting the normal structure. • renal biopsy as it may worsen condition? (Was done when I saw it) -confirms the diagnosis with characteristic pathological appearances • Renal function - normal or elevated creatinine if very abnormal - bilateral • FBC - normal or Anaemia - normocytic n chromic —————— • Genetic studies - WT1 tumour suppressor inactive

Answer 5

* 1 = limited to kidney, not ruptured completely excised * 2 = extends beyond the kidney such as penetration of renal capsule confined to abdo 3 - unresectable non-haematogenous tumour is present and confined to the abdomen. * 4 = haematogenous mets * 5 = bilateral Often staging not done until surgery

Answer 6

Stage 1&2 • Nephrectomy followed by chemotherapy (vincristine AND dactinomycin) Stage 3 + 4 Nephrectomy + chemotherapy (vincristine AND dactinomycin AND doxorubicin). Chemo may be done first to shrink tumour size if tumour large eg 11cm. + post-op radiotherapy (advaned stages) ``` Stage 5 Pre-op Chemo Then surgery - nephrectomy (GOSH) Post-op chemo Kidney transplant? ```

Answer 7

inherited or occur sporadically ; 2 hits needed: inactivation of tumour suppressor genes; WT1 + WT2 genes + Loss of heterozygosity on same genes but diff locus

Answer 8

* With treatment, children survive long term * Survival is 75% for those with metastatic disease * Increased risk of secondary tumours in survivors

Answer 9

disease is extensive bilaterally resulting in renal failure

Answer 10

LEVELS = Normal in typically idiopathic nephrotic syndrome (MCD, focal segmental glomerulosclerosis, membranous nephropathy). C3 levels are LOW in membranoproliferative glomerulonephritis, POST-INFECTIOUS glomerulonephritis, and systemic lupus erythematosus. If C3 level is low, additional tests should be performed to differentiate these conditions, including antinuclear antibody, anti-double-stranded DNA antibody, antistreptolysin O antibody, anti-DNase antibody, and CH50 levels.

Answer 11

HHO: hematuria, hypertension decreased urine output <400 ml/day (oliguria), On urine MCS, will see: red blood cell casts, pyuria, and mild to moderate proteinuria

Answer 12

Diarrheoa + blood Vomiting, Recent gastroenteritis infection -> then eventually AKI Abdominal pain! Common causes: E.coli O157:H7 (Shigella toxin - binds to endothelial cell in glomerulus = platelet activation and microthrombus formation) -> the microthrombus block up the renal vasculature -> infarction -> AKI Triad of haemolytic anaemia, thrombocytopaenia and AKI Children are VERY prone! Risk factors: 1. Contact with animals - went to petting zoo recently 2. Eating undercooked meat Notifiable disease!!

Answer 13

On ivx: Raised creatinine and urea Raised Bilirubin Raised LDH Low platelet Low Hb Blood and stool cultures

Answer 14

``` Supportive: Fluids - replacement and maintenance Weigh daily May need blood transfusion may need dialysis - if very serious Monitor electrolytes closely ``` No antibiotics! Refer to Paeds MDT discussion!

Answer 15

HUS IGA nephropathy Post-infectious /Post-streptococcal glomerulonephritis (PSGN) Membranoproliferative HSP - Henoch schonlein purpura

Answer 16

1. Absence of both kidneys (renal agenesis) – As amniotic fluid is mainly derived from fetal urine, there is severe oligohydramnios resulting in Potter syndrome (fatal) 2. Multicystic dysplastic kidney – It is a non-functioning structure with large fluid-filled cysts with no renal tissue and no connection with the bladder. If bilateral -> potters syndrome 3. autosomal recessive polycystic kidney disease,autosomal dominant polycystic kidney disease, and tuberous sclerosis. - also cause large cystic kidneys BUT some function is retained. 4. Uni or bilateralhydronephrosis - due to urine tract obstruction 5. Horseshoe kidney / pelvic kidney - can predispose to infection or obstruction in drainage 6. Posterior urethral valves - in boys - can lead VU reflux and then to hydronephrosis especially if bilateral

Answer 17

Potters syndrome - cause is renal agenesis or multicystic dysplastic kidneys Potters sequence - obstruction in urine tract causes Both of these leads to intrauterine fetal compression due to oligohydramnios. this leads to: ``` P - Pulmonary hypoplasia O- Oligohydramnios T - Twisted face (Abnormal facies - low set ears, downward slanting eyes) T - Twisted skin E - Extremity malformations R - Renal genesis (billateral) ``` The infant may be stillborn or die soon after birth from respiratory failure.

Answer 18

At worst, this results in a dysplastic kidney which is small, poorly functioning, and may contain cysts. In the most severe and bilateral cases Potter syndrome is present. Renal dysplasia can also occur in association with severe intrauterine vesicoureteric reflux (VUR), in isolation, or in certain rare, inherited syndromes affecting multiple systems. In male fetes, posterior urethral valves may develop severe urinary outflow obstruction resulting in progressive bilateral hydronephrosis, poor renal growth, and declining liquor volume with the potential to lead to pulmonary hypoplasia.

Answer 19

Diagnosed via US in uterus 1. Start prophylactic antibiotics If unilateral hydronephrosis in male (and any abnormality in female) 2. US at 4-6 wks. If abnormal do more ivx If bilateral hydronephrosis in male 2. US within 48hrs birth - normal : stop abx - abnormal : put in catheters then MCUG first then (can confirm with cystoscopy) then surgery if needed.

Answer 20

* urinary tract infection, vesicoureteric reflux, and urinary obstruction have the potential to damage the growing kidney * nephrotic syndrome is usually steroid sensitive and only rarely leads to chronic kidney disease * chronic renal disorders affect growth and development.

Answer 21

About 3–7% of girls and 1–2% of boys have at least one symptomatic UTI before the age of 6 years, and up to 30% of them have a recurrence within a year.

Answer 22

UTI in childhood is important because: • up to half of patients have a structural abnormality of their urinary tract • pyelonephritis may damage the growing kidney by forming a scar, predisposing to hypertension and to progressive chronic kidney disease if the scarring is bilateral.

Answer 23

UTI may involve the kidneys (pyelonephritis), when it is usually associated with fever and systemic involvement - upper UTI or may be due to cystitis, when there may be no fever - Lower UTI ``` Infants - present non-specifically • Fever - may be only sx • Vomiting • Lethargy or irritability • Poor feeding/ faltering growth • Jaundice • Septicaemia - can develop rapidly • Offensive urine • Febrile seizure (>6 months) ``` ``` Children • Dysuria, frequency and urgency • Abdominal pain or loin tenderness • Fever with or without rigors (exaggerated shivering) • Lethargy and anorexia • Vomiting, diarrhoea • Haematuria • Offensive/cloudy urine • Febrile seizure • Recurrence of enuresis ``` Dysuria alone is usually due to cystitis or vulvitis in girls or balanitis in uncircumcised boys. Symptoms suggestive of a UTI may also occur following sexual abuse

Answer 24

• Any child with an unexplained fever > 38C should have a urine sample tested within 24 hours 1. Urine sample – dipstick first AND THEN Microbiology, Senstivity&Culture • Ideally ‘clean-catch’ (otherwise can try urine collection pads) • If urgency consider urethral catheter, if severely ill consider suprapubic aspiration - need to know which bug is growing hence MCS 2. Ultrasound - immediate in atypical/recurrent UTI (regardless of age) - at 6 weeks if normal UTI (if <6months) 3. DMSA - <3y/o - at 4-6months after uti (never done acutely) 4. MCUG - <6months; in atypical/recurrent UTI Refer all children with recurrent UTI to a paediatric specialist for assessment and investigations. Results: 1. Nitrite dipstick - Positive result very likely to indicate a true UTI 2. Leucocyte esterase -ve nitrite +ve Start antibiotic treatment if clinical evidence of UTI Diagnosis depends on urine culture!! - presence of white cells is also specific. - if just leucocytes esterase, dont start abx until further evidence of uti

Answer 25

- If fever > 38 + bacteriuria present OR Loin pain/tenderness present (<2years old)→ consider the child to also have acute pyelonephritis/upper UTI - All other infants with bacteraemia but no systemic symptoms should be considered to have cystitis/lower UTI

Answer 26

3x lower UTIs 1 upper 1 lower UTI 2+ upper UTIs (or acute pyelonephritis)

Answer 27

E.coli - most common when anatomy is normal If atypical organism, consider structural abnormalities: Up to 50% have a structural abnormality of their urinary tract: A. Vesicoureteral reflux (junction doesn’t contract strongly enough = reflux) B. Ureteropelvic junction obstruction C. Posterior urethral valves Circumcision - increases risk in infancy. Neurogenic bladder Constipation - because this causes incomplete emptying - Progression to pyelonephritis is particularly dangerous as it can damage the growing kidney from scarring – predisp to HTN and chronic renal failure

Answer 28

The ureters are displaced laterally and enter directly into the bladder rather than at an angle, with a shortened or absent intramural course risk of: - incomplete bladder emptying which encourages infection - renal damage (bladder voiding pressure transmitted to kidney) - UTIs and pyelonephritis mild reflux only goes into ureter.

Answer 29

``` • infrequent voiding, resulting in bladder enlargement • vulvitis • incomplete micturition with residual postmicturition bladder volumes • obstruction by a loaded rectum from constipation • neuropathic bladder • vesicoureteric reflux. ```

Answer 30

Under 3 months -> seek specialist advice paeds - may require admission and iv abx Over 3 months: - For upper UTI → give oral abx for 7-10 days, ideally low resistant pattern abx (e.g. cephalosporin - CEFALEXIN! co-amoxiclav) - For lower UTI → give oral abx for 3 days – follow local guidance (Examples include: trimethoprim, nitrofurantoin, cephalosporin, amoxicillin) - Safety net – advise parents to return if still unwell 24-48 hours later if younger than 6 months – arrange a follow up US 6 weeks later Regular advice - treatment and/or prevention of constipation Follow up: - surgical repiar of reflux - circumcision - regular urinalysis, BP monitoring for CKD?

Answer 31

DMSA - A DMSA dimercaptosuccinic acid scan 4–6 months following the acute infection should be used to detect renal parenchymal defects - do it late so that if any scarring has formed then it wont be missed in the early stage! MCUG - Used to detect reflux

Answer 32

• high fluid intake to produce a high urine output • regular voiding • ensure complete bladder emptying by encouraging the child to try a second time to empty his bladder after a minute or two, commonly known as double voiding, which empties any urine residue or refluxed urine returning to the bladder * treatment and/or prevention of constipation * good perineal hygiene

Answer 33

haematogenous spread from mum

Answer 34

According to NICE: UTI not caused by E.coli (so klebsiella, Proteus, Pseudomonas, and Streptococcus faecalis) seriously ill (for more information refer to the NICE guideline on fever in under 5s) poor urine flow abdominal or bladder mass raised creatinine septicaemia failure to respond to treatment with suitable antibiotics within 48 hours

Answer 35

Nephritic syndrome ``` Renal conditions: • Tubular: – acute tubular necrosis • Interstitial: – interstitial nephritis • Pyelonephritis, papillary necrosis • FSGS • IgA nephropathy ``` Urine tract: - stones, trauma, cystitis, bladder tumour Haemoglobinuria - Haemolysis Rhhabdomyolysis - myoglobin Drugs - rifampicin Dyes - food Food - beetroot, blackberry Urate crystals Metabolites - porphyria, bilirubin Period blood mixed in

Answer 36

Recent sore thraot orskin infection? - post strep glomerulonephritis Associated fever, dysuria, flank pain? - Pyelonephritis Hx of HTN (+ oliguria) - Nephritic syndrome + renal involvement FH deafness +- Renal disease - Alport syndrome Chronic medical problems - Wengener/Goodpastures, SLE, sickle cell Hx purpuric rash, abdominal pain - Henoch schonlein purpura Hx of trauma - Urine tract bleed

Answer 37

Rochester criteria - there are others too Used to assess risk of bacteraemia, meningitis, UTI, osteomyelitis, suppurative arthritis, soft tissue infections [cellulitis, abscess, mastitis, omphalitis], pneumonia States iif they are low risk, they will meet the following criteria : Infant must appear generally well Infant has been previously healthy: Born at term (≥ 37 weeks' gestation), no perinatal antimicrobial therapy, no treatment for unexplained hyperbilirubinemia, no previous antimicrobial therapy, no previous hospitalization, no chronic or underlying illness, not hospitalized longer than the mother Infant has no evidence of skin, soft-tissue, bone, joint, or ear infection

Answer 38

PAEDS NEPHROLOGY

Answer 39

The onset!! IgA nephropathy: sore throat and renal sx eg haematuria happen AT THE SAME TIME or 1day-1week after onset Normal C3 Adolescents post strep glomerulonephritis: Renal sx eg haematuria come weeks afterwards Low C3

Answer 40

Next step: repeat urinalysis in 6 hours orthostatic proteinuria - where urine protein gets worse throughout day you want one in morning then one after a long day? later you can then do protein:creatinine ratio

Answer 41

transient proteinuria - caused by seizures, infection, heavy exercise or pregnancy resolves within 2 days (as sx go away)

Answer 42

nephrotic syndrome transient proteinuria orthostatic proteinuria (causes persistent proteinuria)

Answer 43

Mild: o Treat the underlying cause o Supportive treatment with close monitoring o Phenoxymethylpenicillin -> if post-strep GN: • Moderate-severe: o ACE inhibitor or ARB (ramipril or losartan) o Phenoxymethylpenicillin if post-strep GN o Furosemide - loop diuretic Anti-GBM antibody: § Plasmapheresis + prednisolone + cyclophosphamide § Prophylactic trimethoprim – for early phases of treatment due to immunosuppression o Immune complex – not SLE: § Prednisolone if SLE: Cyclophosphamide +- Prednisolone

Answer 44

rule of thirds 1/3rd recover 1/3 relapsing nephrotic syndrome 1/3 have risk of progressing to renal failure

Answer 45

Clinical presentation is relatively nonspecific but includes poor growth, fatigue, malaise, weakness, as well as signs of the complications of CKD. The complications include: - uraemia - important in staging and mx! - hypertension - in mx we give lots of anti-htns hyperkalaemia, metabolic acidosis,, anaemia and renal osteodystrophy

Answer 46

1. Treat any reversible causes & complications Initially supportive: Restricted diet and supplementation. - monitor proteins (enoughh for growth but not excess) - Vit D supplements (prevent 2ndary hyperPTH) - Calcium carbonate (bind PO4 - 2nd HPTH) - FeSO4 / EPO stimulator if anaemia - Adress growth/puberty delay: GH Stage 1/2 - no uraemia: 1st - ACE inhibitor/ARB (Lisinopril/Losartan) 2nd - CCB Statin (Nifedipine) -> Additional Antihypertensives Stage 3-4 without uraemia Same as above + Atorvastatin +- Ezetimibe (at each step) Stage 5: If GFR less than 15 -> renal replacement therapy: 1st - Dialysis 2nd - Renal transplantation.

Renal Flashcards

(70 cards)