Renal Flashcards
Features of Potter’s Syndrome
Pulmonary Hypoplasia Low set ears Beaked Nose Prominent epicanthic folds Downwards slanting eyes Limb Deformity
Mechanism of Multicystic Dysplastic Kidney
Failure of union of the uterine bud with nephrogenic mesenchyme
Management of Multicystic Dysplastic Kidney
50% involuted by aged 2
Nephrectomy indications: very large/ hypertension develops
Causes of large Cystic Kidneys
Autosomal recessive/dominant polycystic Kidney disease
Multicystic Dysplastic Kidney
Tuberous Sclerosis
Presentation of Autosomal dominant polycystic kidney disease
Childhood hypertension
Renal failure in adulthood
Extra Renal feature of Autosomal Dominant Polycystic Kidneys
Cysts in liver and pancreas
Cerebral Aneurysm
Mitral Valve prolapse
Abnormal Caudal migration of the kidneys results in…
Horseshoe kidney or pelvic kidney
Duplex System is due to?
Premature division of the uterine bud
Complications of duplex system
Lower pole moiety- Reflux
Upper pole moiety- Ectopic drainage to urethra or vagina, prolapse into bladder, obstruction urging flow
Features of Absent Musculature Syndrome (Prune Belly)
Wrinkled appearance of abdomen
Large Bladder
Dilated Ureters
Crytorchidism
Site of urinary tract obstruction
Pelviuretric Junction
Vesicoureteric Junction
Bladder Neck
Urethra
Features of bilateral hydronehritis
Hydronephrosis
Hydroureters
Thickened bladder wall
Diverticula
Management of urinary tract anomalies
Start prophylactic antibiotics
US within 24hours, in males with bilateral hydronephrosis
-Normal: US repeat at 2-3 months, stop Abx
-Abnormal: MCUG and surgery
US within 4-6 weeks, in females or unilateral hydronephrosis
- Normal: US repeat at 2-3 months, stop Abx
-Abnormal: Further Ix
Renal Function Assessment in children
Plasma creatinine concentration: eGFR Insulin/EDTA GFR Creatinine Clearance Plasma Urea Concnetration
Radiological Investigation of Kidneys and Urinary Tract
Ultrasound: anatomy
DMSA scan: renal scarring (2 months after UTI- sensitive)
MCUG: detects reflux and urethral obstruction
MAG3 renogram (Children over 4 years): detects reflux, given with furosemide
Plain abdominal X-ray: spinal abnormalities
Features of UTI in infants
Offensive Urine Septicaemia Jaundice Poor Feeding Lethargy Irritability Vomitting Fever Febrile siezure
Features of UTI in children
Secondary Enuresis Dysuria Frequency Urgency Abdominal Pain Loin Tenderness Fever Anorexia Lethargy Vommitting and Diarrhoea Haematuria Cloudy Urine Febrile Seizure
dDx Dysuria
Girls- Cystitis or Vulvitis
Boys- Balanitis (Uncircumcised)
Collection of Urine Samples
Clean catch sample (recommended method) Adhesive plastic bag Urethral catheter Suprapubic aspiration (fine needle inserted above pubic symphysis under US guidance) Midstream sample- Older children
Dipstick Interpretation
Nitrate- positive indicates UTI
Leukocyte- present in fever without UTI, balanitis, and cystitis
Blood/Protein/Glucose- not UTI specific
Predisposing factors UTI
Renal or urinary tract abnormality
Incomplete Bladder emptying
Vesicoureteric reflux
Incomplete bladder emptying factors
Infrequent voiding Vulvitis Incomplete micturition Obstruction from constipation Neuropathic bladder Vesicoureteric reflux
Anatomy of vesicoureteric reflux
Anomaly of vesicoureteric junctions
Ureters displaced laterally
Ureters enter bladder directly
Shorted or absent intramural course
Complications of vesicoureteric reflux
Incomplete Bladder emptying
Increased risk of infection
Risk of pyelonephritis
Renal damage due to pressure transmission
Features Atypical UTIs
Seriously ill or septicaemia Poor urine flow Abdominal or Bladder Mass Raised Creatinine Failure to respond to Abx within 48hrs Non-E.coli organism
Mx UTI Infants <3 months
Immediate referral to hospital
IV Abx .e.g. Co-amoxiclav for 5-7 days
Oral prophylaxis
Mx Pyelonephritis Children >3m
Oral Abx .e.g. Trimethoprim 7days
OR IV Abx .e.g. Co-amoxiclav 2-4 days + oral (total course 7days)
Mx Simple UTI Child >3m
Oral Abx .e.g. Trimethoprim or Nitrofurantoin 3 days
Prevention of UTIs
High fluid intake Regular voiding Complete bladder emptying- sitting on toilet/double voiding Treatment of constipation Good Perineal hygiene Lactobacillus Acidophilus (pro-biotic) Antibiotic prophylaxis
UTI Abx prophylaxis
Children <2-3years with congenital abnormality/upper UTI/reflux
Continue until out of nappies
Trimethoprim 2mg/Kg NOCHE
Follow-up recurrent UTIs, reflux and scarring
Urine dipstick in any non-specific infection
Long term low dose Abx prophylaxis
Circumcision in boys
Anti-VUR surgery in progressive scarring
Annual blood pressure in renal defects
Urinalysis to check proteinuria (CKD)
Regular assessment renal growth and function
Causes of daytime enuresis
Lack of attention to bladder sensation Detrusor instability Bladder neck weakness Neuropathic bladder UTI/Diabetes Constipation Ectopic ureter
Signs of neuropathic bladder
Distended bladder Abnormal perineal sensation Abnormal anal tone Abnormal leg reflexes and gait Sensory loss in S2-S4
Typical Hx of ectopic ureter
Girls who are dry by day but with wetting on morning waking
Pooling of urine from vaginal ureter opening
Ix for daytime enuresis
Urine dipstick, and MCS US- ?incomplete bladder emptying and thickening of bladder wall Urodynamics X-ray- ?vertebral abnormality MRI- ?cord tethering
Mx daytime enuresis
Star charts Bladder training Pelvic floor exercises Constipation treatment Urine alarm Anticholinergics .e.g. Oxybutynin
Causes secondary enuresis
Emotional upset (most common)
UTI
Diabetes (osmotic diuresis)
Renal concentrating disorder .e.g. Sickle cell, CKD, DI
Ix secondary enuresis
UDS- infection, glycosaria, proteinuria
Early morning osmalality
Formal water deprivation test
US renal tract
Physiological proteinuria
Febrile illnesses
After exercise
Normal protein:creatinine
<20mg/mmol early morning sample
Causes of proteinuria
Orthostatic proteinuria Glomerular abnormality: -Minimal change disease -Glomerulonephritis -Abnormal glomerular basement membrane Increased glomerular filtration pressure Reduced renal mass in CKD Hypertension Tubular proteinuria
Features of nephrotic syndrome
Periorbital odema esp. on waking
Scrotal, vulva, leg, ankle odema
Ascites
Breathlessness: abdominal distension and pleural effusion
Infection .e.g. Peritonitis, septic arthritis, sepsis (loss of Ig)
Systemic disease associated with nephrotic syndrome
HSP
Vasculitides .e.g SLE
Infection .e.g. malaria
Allergens .e.g. bee sting
Epidemiology steroid sensitive nephrotic syndrome
Age 1-10 years
More common in boys
Asian children
Associated with atopy
Biochemistry steroid sensitive nephrotic syndrome
No macroscopic haematuria
Normal BP
Normal complement levels
Normal renal function
Management steroid sensitive nephrotic syndrome
Oral corticosteroids .e.g. prednisolone 60mg/m^2 daily
Reduce dose to 40mg/m^2 after 4 weeks alternate days
Wean after 8 weeks
Histology steroid sensitive nephrotic syndrome
Normal on light microscopy
Electron microscopy- fusion of specialised epithelial cells investing glomerular capillaries
Minimal change disease
Complication of nephrotic syndrome
Hypovolaemia
Thrombosis
Infection
Hypercholestrolaemia
Mx hypovolaemia in nephrotic syndrome
IV 0.9% saline or 4.5% albumin solution
IV 20% albumin with furosemide if severe
Complications of albumin
Precipitates pulmonary odema and hypertension from fluid overload
Prognosis of nephrotic syndrome
1/3 resolve spontaneous
1/3 infrequent relapses
1/3 steroid dependant with frequent relapses
Identified by parents on urine testing
Steroid sparing agents in relapsing nephrotic syndrome
Levamisole- immunomodulator
Cyclophosphamide- alkylating agents
Tacrolimus/cephalosporin A- calcineurin inhibitors
Mycophenolate mofetil- immunosuppressant
Rituximab- Anti B cell monoclonal antibody
Mx steroid resistant nephrotic syndrome
Diuretic therapy
Salt restriction
Angiotensin-converting enzyme inhibitors
NSAIDs
Epidemiology Congenital nephrotic syndrome
Presents in first 3 months of life RARE Recessively inherited Consanguinity and Finnish populations High mortality
Cx Congenital nephrotic syndrome
Hypoalbuminemia- high mortality
Mx congenital nephrotic syndrome
Unilateral nephrectomy for albumin control
Dialysis for stage 5 CKD continued until transplant
Ix nephrotic syndrome
Urine protein FBC and ESR Urea, electrolytes, creatinine, albumin Complement C3 and C4 Anti-streptolysin O or anti-DNAase B titres/throat swab Urine MCS Urinary sodium concentration Hep B and hep C screening Malaria screening (foreign travel)
Steroid resistant nephrotic syndrome
Focal segmental glomerulosclerosis
Mesangiocapillary glomerulonephritis
Membranous nephropathy
Focal segmental glomeruloscleosis features
Most common steroid resistant nephrotic syndrome
30% progress to renal failure
Mx: cyclophosphamide, cyclosporin, tacrolimus, rituximab
Recurrence post-transplant common
Mesangiocapillary glomerulonephritis feature
More common in older children
Haematuria
Low complement level
Decline in renal function over years
Membranous nephropathy
Associated with hep B and SLE
Usually remits spontaneously within 5 years
Non glomerular causes of haematuria
Infection Trauma Stones Tumours Sickle cell disease Bleeding disorders Renal vein thrombosis Hypercalciuria
Glomerular causes of haematuria
Acute nephritis (with proteinuria) Chronic glomerulonephritis IgA nephropathy Familial nephritis .e.g. Alport Thin basement membrane
Indications for renal biopsy in haematuria
Significant persistent proteinuria
Recurrent macroscopic haematuria
Renal function in abnormal
Abnormal complement levels
Glomerular haematuria features
Brown urine
Deformed red cells
Casts
Associated with proteinuria
Lower urinary tract haematuria features
Red urine
Occurs at beginning or end of urinary stream
Unusual in children
Ix haematuria
Urine MCS
Protein and calcium excretion
Kidney and urinary tract US
Plasma urea, electrolytes, creatinine, calcium, phosphate, albumin
FBC, platelet, coagulation screen, sickle cell screen
Ix Glomerula haematuria
ESR, complement levels, anti-DNA antibodies
Hep B and C screen
Throat swab and antitreptolysin O/anti-DNAase titres
Renal biopsy
Test mothers urine and hearing test (Alports)
Causes of Acute nephritis
Post infectious .e.g. strep
Vasculitis .e.g. HSP, SLE, Wegners, polyarteritis nodose, microscopic polyarteritis
IgA nephropathy and mesangiocapillary glomerulonephritis
Antiglomerular basement membrane disease (Goodpastures)
Acute nephritis presentation
Decreased urine output Volume overload Hypertension- seizures Periorbital odema Haematuria Proteinuria
Post-strep nephritis features
Follows sore throat or skin infection
Raise strep titre or culture
Low C3 returning to normal after 3-4 weeks
Henoch-Schonein Pupura Presentation
Purpuric rash affecting extensor surfaces Arthralgia Periarticular oedema Abdominal pain Glomerulonephritis Boys aged 3-10 years
Henoch-Schonein Purpura Pathology
Genetic predisposition and antigen exposure
IgA and IgG complex activate complement and precipitate inflammation
HSP Rash
Symmetrical distribution Buttocks, extensor surfaces of arms and legs Ankles Trunk sparing Urticarial initially Progression to maculopapular Characteristically palpable
HSP Arthralgia
Knees and Ankles
Periarticular oedema
HSP Abdominal Pain
Colicky
Melaena and haematemesis
Mx: corticosteroids
Cx Henoch Schonlein Purpura
Interssusception Ileus Protein loosing eneteropathy Orchitis CNS involvement
HSP Renal Involvement
Common
Haematuria +/- proteinuria
Risk factors for CKD in HSP
Heavy proteinuria
Oedema
Hypertension
Deteriorating renal function
IgA nephropathy presentation
Macroscopic haematuria
URTI
Treat as HSP
Alport syndrome
X linked recessive condition
CKD by early adulthood
Neural deafness and ocular defects
Maternal haematuria
Causes Hypertension
Renal Coarctation of the aorta Catecholamine excess: pheochromocytoma/neuroblastoma Endocrine Essential hypertension
Presentation hypertension
Vomiting Headaches Facial palsy Hypertensive retinopathy Convulsions Proteinuria Faltering growth Cardiac failure Paroxysmal palpations/sweating (pheochromocytoma)
Causes unilateral palpable kidneys
Multicystic kidneys Compensatory hypertrophy Obstructed hydronephrosis Renal tumour .e.g. Wilms Renal vein thrombosis
Causes bilateral palpable kidneys
Polycystic kidney disease
Tuberous sclerosis
Renal vein thrombosis
Predisposing factors renal calculi
UTI esp. proteus
Structural anomalies of urinary tract
Metabolic abnormalities
Hypercalcaemia - calcium stones
Presentation renal calculi
Haematuria Loin pain Abdominal pain UTI Passage of a stone
Mx renal calculi
Passage spontaneously
Lithotripsy
Surgical removal
High fluid intake
Complication of furosemide therapy in neonates
Nephrocalcinosis
Causes Fanconi syndrome- metabolic
Cystinosis Glycogen storage disorders Lowe syndrome Galactosaemia Fructose intolerance Tyrosinaemia Wilson disease
Causes Fanconi syndrome- acquired
Heavy metals
Drugs and toxins
Vitamin D deficiency
Defition Fanconi Syndrome
Generalised proximal tubular dysfunction
Presentation Fanconi Syndrome
Polydipsia Polyuria Salt depletion Dehydration Hypercalcaemic metabolic acidosis Rickets Poor/faltering growth
Urinary electrolyte loss in Fanconi syndrome
Amino acids Glucose Phosphate Bicarbonate Sodium Calcium Potassium Magnesium
Classification of AKI
Pre-renal
Renal: salt and water retention, blood/protiein/casts in urine
Post-renal: urinary obstruction
Management of AKI
Monitoring of circulation and fluid balance
Ix in AKI
US:
- obstruction of urinary tract
- small kidneys in CKD
- large bright kidneys in CKD
Pre-renal acute failure features
Hypovolaemia
Low secretion of sodium
Cx Pre-renal Acute failure
Acute tubular injury and necrosis
Features Acute Renal Failure
Circulatory overload
Most commonly HUS and acute tubular injury
Causes of acute tubular injury
Cardiac surgery
Multisystem failure in ICU
Mx Acute Renal failure
Restriction of fluid intake
High calorie, normal protein feed
Renal biopsy
Causes of Pre-Renal AKI
Hypovolaemia
Circulatory failure
Causes of Renal AKI
Vascular .e.g. HUS, vasculitis, emboli, renal vein thrombosis
Tubular: acute necrosis, ischaemia, toxic, obstructive
Glomerulonephritis
Interstitial nephritis
Pyelonephritis
Causes of Post-renal AKI
Obstruction
Dialysis indications in AKI
Failure of conservative management Hyperkalaemia Severe hyper/hypo-natraemia Pulmonary oedema Severe hypertension Severe metabolic acidosis Multisystem failure
Proximal tubule transport defects
Glycosuria- asymptomatic (glucose)
Cystinuria- renal calculi (cystine amino acids)
Vitamin D resistant rickets (phosphate)
Pseudohypoparathyroidism- obesity, depressed nasal bridge, short fingers (phosphate)
Hyperuricosuria- renal calculi (uric acid)
Renal tubular acidosis- metabolic acidosis, alkaline urine, faltering growth (bicarbonate)
Hypercalciuria- renal calculi (calcium)
Bartter syndrome
Chloride transporter defect Loop of Henle Hypokalaemic metabolic acidosis Hypercalciuria Normal bp Raised renin Polydipsia Polyuria Faltering Growth
Nephrogenic Diabetes Insipidus
Polydipsia
Polyuria
Fever
Faltering growth
Haemolytic Uraemic Syndrome Triad
Acute renal failure
Microangiopathic haemolytic anaemia
Thrombocytopenia
Typical HUS features
Follows GI infection (verocytotoxin E.coli- farm animal or uncooked beef)
Diarrheal prodrome
Persistent proteinuria
Cx: htn and CKD
Mx metabolic acidosis in renal failure
Sodium bicarbonate
Mx hyperphosphataemia in renal failure
Calcium carbonate
Dietary restriction
Mx hyperkalaemia in renal failure
Calcium gluconate in ECG changes Salbutamol (neb or IV) Calcium exchange resin Glucose and insulin Dietary restriction Dialysis
Features Atypical HUS
No diarrheal prodrome
Frequent relapses
High risk htn and CKD
Mx Atypical HUS
Eculizumab: Monoclonal anti-terminal complement antibody
Plasma exchange
Features CKD
Anorexia Lethargy Polydipsia Polyuria Faltering growth/growth failure Bony deformity (renal osteodystrophy) Hypertension Acute-on-Chronic renal failure (dehydration/infection) Proteinuria Normochromic, normocytic anaemia
Staging CKD
I >90ml/min II 60-90ml/min III 30-60ml/min IV 15-30 ml/min V <15ml/min
Mx CKD
Calorie supplementation and NG feeds Phosphate restriction Calcium carbonate as phosphate binder Vitamin D supplementation Salt supplementation Free access to water Bicarbonate supplements Recombinant human erythopoetin- subcut Growth hormone supplementation
Renal Transplant Criteria
Weight >10kg
Risk of renal vein thrombosis
Immunosuppression with transplantation
Tacrolimus
Mycophenolate Mofetil
Prednisolone
