Nephrology Flashcards
What is the most common renal stone?
Calcium Oxalate
Teenager Found to have struvite stone…what is the next step?
Urine Culture
Struvite calculi
UTIs caused by urea-splitting organisms result in urinary alkalinisation and excessive production of ammonia -> precipitation of magnesium ammonium phosphate (struvite) and calcium phosphate
In the kidney, the calculi often have a staghorn configuration, filling the calyces
Often are seen in children with neuropathic bladder, particularly those who have undergone a urinary tract reconstructive procedure
Bacteria result in alkaline urine -> tx it by acidifying the urine! e. Urologic removal of struvite calculi is generally required to eradicate the underlying infection. Persistent infection, usually due to a urease-producing bacteria (eg, Proteus or Klebsiella), is a risk factor for recurrent stone formation.
A boy with family history of renal stones, presents with 24-48h right flank pain. Passed a 5 mm stone determined to be calcium oxalate.
Name 4 dietary interventions you would recommend to decrease the risk of further stones
- Increase fluids
- Increase citrate in diet (eg add lime/lemon/orange to water)
- Normal calcium intake (do not restrict calcium)
- Low sodium intake (urinary calcium is excreted with urinary sodium)
- Minimize use of furosemide/steroids/excess vit D
- Consider thiazide diuretic to decrease calciuria or potassium citrate to augment stone inhibition
A 12-year-old girl who has had cola-colored urine for 3 days is brought to your office.
Temp 37.7°C, RR 26 breaths/min, HR 110 beats/min, BP 140/90 mm Hg. P/E significant for mild edema. Normal growth parameters.
U/A: >100 RBC, 10-20 WBC, and 2-3 granular casts per HPF.
What diagnosis do you suspect?
Post Infectious GN
PIGN has what complement variations?
low C3 level and normal C4 level
What are the clinical features of glomerularnephritis?
cola-colored urine microscopic hematuria hypertension edema proteinuria
SLE has what complement profile
Low C3…BUT SYSTEMIC ILLNESS
IgA Nephropathy has what complement profile
Normal C3…renal limited disease
HSP has what complement profile
Normal C3…but systemic illness
Alport has what complement profile?
Normal C3 but systemic illness
In PIGN how long does it take for resolution of the following?
How long does it take for resolution of the following?
Gross hematuria
Proteinuria
Low C3
Microscopic hematuria
Gross hematuria: 1-2 weeks
Proteinuria: shortly after gross hematuria
Low C3: 6-8 weeks
Microscopic hematuria: up to 1 year
What type of RTA is this? has HYPERCALCIURIA → renal stones Urine pH = alkalotic >5.5 (can’t excrete H+ so urine alkalotic) Hypokalemia Hypocitraturia
Type 1 = distal
What type of RTA is this?
Hypokalemia
No stones
Urine pH = alkalotic, then acidotic
Can be isolated acidosis
Can be associated with other tubular defects: Fanconi
- Hypophosphatemia
Rickets/osteomalacia
- Aminoaciduria
- Glucosuria
- FTT or short stature
Type 2 = proximal
What type of RTA is this? Urine Acidic pH (<5) Hyperkalemia Congenital – hypoaldo/pseudohypoaldosterone Medications – ACE inhibitors
Type 4
14 month male, FTT, vx, met acidosis, pH 7.31, bicarb 14, K 3.5, Na 140, Cl 118, urine pH 7.3. Dx?
Distal RTA (can’t excrete H ions so urine alkalotic)
What’s the most common condition associated with type II (proximal) RTA?
Cystinosis
What is Fanconi Syndrome?
Excess amounts of things in the urine Amino aciduria Glucosuria (no hyperglycemia) Phosphaturia --> hypophosphatemia Uric aciduria FTT (from acidosis) Hypokalemia
Think genetic disorders Cystinosis Galactosemia Fructose intolerance Tyrosinemia Wilson's Lowe's (oculocerebrorenal syndrome)
CPS UTI mangement
CPS statement first febrile UTI > 2mos of age No known renal/urological pathology Urinary symptoms or unexplained fever Urine culture and analysis Catheter or mid stream Unlikely UTI if analysis has no pyuria Treat 7-10 days oral if can tolerate po
What imaging needs to be done if first febrile UTI 2months- 2 years of age
Ultrasound
During or within 2 weeks of presentation
Less invasive, available, less expensive
VCUG Pursue if renal abnormalities, obstruction, signs of VUR on RUS or second UTI Hydronephrosis, hydroureter Small kidneys Thick walled bladder Renal parenchyma abnormal – scars, cysts
What is cystinosis?
AR Lysosomal storage disease Accumulation of cysteine in various organs Cysteine dimers accumulate in lysosomes 1 in 100,000 children No increased risk of stones
How do you confirm diagnosis of cystinosis?
Leukocyte cysteine
What is the pathophysiology of Type 4 RTA?
Either impaired aldosterone production or impaired renal responsiveness to aldosterone
Can present with growth failure in the first few years of life
Polyuria and dehydration = common
Hyperkalemic non-anion gap MA
What does aldosterone do?
Aldosterone functions (at kidney level):
Secretes H+, secretes K+
Reabsorbs Na
No aldosterone = high potassium
What is the main cause of Type 1 RTA?
Idiopathic
What is the main defect in Type 1 RTA?
Inability of the distal tubule to secrete Hydrogen Ion
What is the main defect in Type 2 RTA?
Decreased Ability of proximal tubule to reabsorb bicarb.
List some medications that can cause Hypertension
Corticosteroids Decongestants Nonsteroidal anti-inflammatory medications Herbal supplements β-adrenergic agonists Erythropoietin Cyclosporine Tacrolimus Stimulants *Recent discontinuation of antihypertensive medications
What is needed to determine normal range of blood pressure?
Age
Gender
Height
What are the BP Stages for children 1-13 y
Normal BP: <90th percentile
Elevated BP: ≥90th percentile to <95th percentile or 120/80 mm Hg to <95th percentile (whichever is lower)
Stage 1 HTN: ≥95th percentile to <95th percentile + 12 mm Hg or 130/80–139/89 mm Hg (whichever is lower)
Stage 2 HTN: ≥95th percentile + 12 mm Hg or ≥140/90 mm Hg (whichever is lower)
What are the BP Stages for Children > 13
Normal BP: <120/<80 mm Hg
Elevated BP: 120/<80–129/<80 mm Hg
Stage 1 HTN: 130/80–139/89 mm Hg
Stage 2 HTN: ≥140/90 mm Hg
What do you do after you identify their BP based on their stage?
Normal/Elevated BP: Repeat q 6 months. Lifestyle counselling.
Stage 1 HTN
A. Repeat 1-2 weeks. up to 3 months. Lifestyle counselling. Treat if persistent at 3 months.
Stage 2 HTN: Repeat in office. Treat. Refer to subspecialist
A 14-year-old boy presents for a health supervision visit. The boy is doing well at school, and his parents have no concerns. His vital signs include a temperature of 37.7°C, heart rate of 65 beats/min, respiratory rate of 14 breaths/min, and blood pressure of 120/74 mm Hg. His growth parameters and physical examination findings are normal. The boy’s mother, age 38 years, was recently diagnosed with autosomal dominant polycystic kidney disease (ADPKD). His 55-year-old maternal grandfather has end-stage renal disease secondary to ADPKD, and is currently receiving dialysis.
Of the following, the extrarenal manifestation MOST likely to be seen with this disease is: Biliary dysgenesis Cerebral aneurysm Lenticonus Portal fibrosis
Cerebral Aneurysm
AR Polycystic Kidney Disease Presentation
Autosomal recessive
Manifests in infancy
Large flank masses
Potter syndrome
Resp distress from pulmonary hypoplasia
Potter faces (flattened ears/nose, micrognathia from oligohydramnios)
U/S
Large echogenic kidneys w decreased “corticomedullary differentiation”
macrocysts are NOT typically seen- Unlike ADPKD
Extrarenal manifestations
Liver fibrosis
Congenital
Extrahepatic portal HTN
AD Polycystic Kidney Disease Presentation
Autosomal dominant
usually asymptomatic in childhood
Dx when renal cysts detected incidentally
Can present with: Persistent microscopic hematuria HTN Flank pain UTI (serum Cr = normal!) Renal function is normal until 4th decade of life
Extrarenal manifestations Cerebral aneurysms (most common) hepatic/pancreatic/seminal vesicle cysts Cardiac valve diseases Abdo wall hernia Colonic diverticula
What is Prune Belly Syndrome
Partial or complete absence of the abdominal wall muscles -> wrinkled abdominal wall
Cryptorchidism
Urinary tract abnormalities
B/L hydroureteronephrosis
Enlarged bladder with thick walls (collagen deposits) and abnormal bladder pressures
Varying degrees of renal dysplasia
~30% develop ESRD requiring renal replacement therapy with dialysis or renal transplant
Hypoplastic or dysplastic prostate
Anomalies of the musculoskeletal, gastrointestinal, and cardiopulmonary systems
NO GENE IDENTIFIED
7 year old with microscopic hematuria most likely dx?
Hypercalciuria. Usually idiopathic 30%
What is this:
An inherited disorder of basement membrane collagen characterized by involvement of the kidneys (always), ears (often), and eyes (occasionally)
Persistent hematuria (microscopic, macroscopic) = earliest symptom and universal symptom
Proteinuria and hypertension can accompany
Can have SNHL and Anterior lenticonus lens
Alport Syndrome
What is the inheritance of Alport Syndrome?
Most common inheritance: X-linked (*Maternal Uncle)
Males affected
Female carriers
What is Alport Syndrome
An inherited disorder of basement membrane collagen characterized by involvement of the kidneys (always), ears (often), and eyes (occasionally)
Persistent hematuria (microscopic, macroscopic) = earliest symptom and universal symptom
Proteinuria and hypertension can accompany
Can have SNHL and Anterior lenticonus lens
What makes the prognosis in a kid with hematuria worse?
Associated Proteinuria
What are some benign causes of proteinuria?
Orthostatic proteinuria
Transient proteinuria: fever, stress, exercise, cold, abdominal surgery
How do you diagnose orthostatic proteinuria?
Benign
Occurs when children are active and disappears when they are supine/asleep for at least 2 hours
Diagnosed with absence of proteinuria in three consecutive first morning U/A
A urine protein-creatinine ratio greater than 0.2 in a first-morning sample is abnormal
Bright red blood in urine usually indicates what?
Bright red hematuria is usually indicative of lower urinary tract bleeding
Cola/tea coloured urine usually indicates what?
glomerular hematuria (as in nephritis) is commonly described as cola, tea, or brown colored.
A 10 year old child has type 1 diabetes which was diagnosed at age 3 years old. When should they start screening for diabetic nephropathy and how?
YEARLY screening starting at age 12 years in those with T1DM for > 5 years
First morning or random ACR
Most likely origin for abdominal mass in newborn?
Renal (55%)
Most likely cause of abdominal mass in newborn?
- Hydronephrosis (#1)
2. Multicystic dysplastic kidney
Most common cause of MIDLINE mass in newborn?
Mesenteric Cyst
First line imaging of abdominal mass?
Abdo Ultrasound
Ultrasound has many advantages over CT or MRI in this situation. They are:
A. generally harmless
B. less expense
C. no sedation
D. ability to perform at the bedside
How much Na, Cl and osm is in a bag of NS?
154 meq/L Na
154 meq/L Cl
Osm= 154+ 154= 308
What is the equation for insensible losses?
400mL/m2
How do you calculate BSA?
square root of height x weight /3600
How much glucose do you need in a day?
4-6mg/kg/min
What is a normal Anion Gap?
<12
How do you calculate Anion Gap?
Na-Cl-HCO3
What are two conditions that present with a non anion gap metabolic acidosis?
A. Renal tubular acidosis
B. Gastroenteritis
What are the characteristics of proximal RTA?
Proximal RTA • Urine pH<7 • Acidosis – HCO3 15 • Often hypoNa • Can have hypoPhos • Glucosuria • Mild tubular proteinuria or generalized aminoaciduria • Fanconi’s syndrome
What are the characteristics of distal RTA?
- Urine pH>7
- Acidosis – HCO3 can go below 10
- Sodium usually normal
- Nephrocalcinosis may be present
Most common cause of Type 1 RTA (distal)
Idiopathic
Familial Secondary • Ifofamide • Valporate • Amphotericin • Lupus • Obstructive uropathy • Renal dysplasia • Lithium Carbonate
Most common cause of Type 2 RTA (proximal)
– Metabolic • Cystinosis = most common • Glycogen storage diseases • Wilson’s • Tyrosinemia • Galactosemia
Idiopathic
Acquired • Ifosfamide • Carbonic anhydrase inhibitors • Lead • Paroxysmal nocturnal hemoglobinuria
Type 4 RTA causes
Aldosterone Deficiency – CAH – Acute GN – ACEI – NSAIDs
Aldosterone Resistance – Spironolactone – Dysplasia – CKD – Pseudohypoaldosteroni sm
How to treat distal RTA?
Potassium Citrate
Checking for hearing loss
Genetic testing
What is the single most important risk factor for future loss of kidney function
Proteinuria
What is the most common cause of proteinuria in pediatrics?
Transient proteinuria
• Exercise
• Fever
• Infection
Prevalence: 5 -15%
Definition of persistent Proteinuria
Defined as proteinuria on 2 or 3 separate samples take 1-2 weeks apart
How can falsely negative and positive dipsticks happen for protein?
Does not measure the actual amount…just the concentration
Concentrated sample - protein may look much higher than it actually is
dilute sample - may look higher than actual
How do we normally assess proteinuria in peds?
Protein/creatinine ratio
What are the normal protein/creatinine rations in children?
Normal:
Infants (< 2 years of age) – Upc < 50 mg/mmol
Children (> 2 years of age) – Upc < 20 mg/mmol
Most common cause of proteinuria in adolescents?
Orthostatic Proteinuria
How do you assess orthostatic proteinuria?
Assess with two separate night and daytime sample
If AM sample Upc < 20, diagnosis can be made
What is Dent’s Disease
- X-linked recessive
– Proteinuria, hypercalciuria, nephrolithiasis and/or medullary nephrocalcinosis
– Also look for glycosuria, aminoaciduria, phosphate wasting
Average age of Idiopathic Nephrotic Syndrome
2-10
Features that should make you think it’s something other than Nephrotic Syndrome
- <18months or >10 years of age
- Positive family history
- Extra-renal manifestations
- Chronic disease
- Hypertension
- Acute renal failure at presentation • +/- Hematuria
Initial Investigations for Nephrotic Syndrome
Laboratory Workup: • CBC, lytes, BUN, Cr, albumin, glucose, cholesterol • Complements, ANA (if < 2 years or older > 10 years of age or other features) • Urine protein:creatinine ratio • Early morning urine for pr:creat ratio • Urine microscopy and C&S • VZVstatus • Hep B an dC serology, HIV \+/- ASOT
Management of Nephrotic Syndrome
Edema
– Albumin/lasix
– Fluid and salt restriction
Low salt diet(thirst)
Avoid hypovolemia (intravascular volume depletion)
– Clinical examination
– Packed cell volume
– Urinary Na (cannot interpret if diuretics)
Risks of hypovolemia
– Pre renal failure
– Thrombosis
Steroids
2 mg/kg per day for 6 weeks (minimum 4 weeks)
• Maximum dose is usually 60mg daily
– 1.5 mg/kg on alternate days for 6 weeks
– No steroid taper is required
– Most children respond within 5-10 days of starting steroids
– 95% with Steroid Sensitive NS (SSNS) are in remission by 4 weeks
What is a remission, relapse and frequent relapse of Nephrotic Syndrome?
Remission: Urine protein/creatinine ratios < 20 or urine dipstick negative-trace for 3 days
Relapse: After remission, increase in proteinuria of 20g/L for 3 days
Frequently relapsing: 2 or more relapses within 6 months or >= 4 in any 12 month period
What is the definition of Nephrotic Syndrome
Nephrotic range proteinuria − Urinary protein excretion greater than 50 mg/kg per day
Pr:creat ratio >250 mg/mmol
Hypoalbuminemia − Serum albumin concentration less than 3 g/dL (30 g/L)
Edema
Hyperlipidemia
How do you treat the steroid resistant or steroid dependent Nephrotic syndrome
Management of Steroid Dependent NS (SDNS) and Steroid Resistant NS (SRNS):
– Prednsione – Trial of cyclophosphamide, tacrolimus – Consider MMF or cyclosporine – Biopsy – Consider genetic testing
What is steroid resistant nephrotic syndrome
Steroid Resistant (SRNS): Inability to induce remission with 4 weeks of daily steroid therapy
What is steroid dependent nephrotic syndrome
Steroid dependent (SDNS): Relapse during taper or within 2 weeks of discontinuation of steroids
In steroid resistant nephrotic syndrome , what percent will need transplant?!
Steroid Resistance:
– Majority of these will be FSGS
– 50% will require dialysis/transplantation within 5 years
– In pediatrics, it is the most common cause of acquired end-stage renal disease
Increased risk of infection with nephrotic syndrome with what Pathogens?
Common pathogens in NS: – S. pneumoniae – Other streptococci – E. coli – Enterobacteriaceae – H. influenzae
Spontaneous bacterial peritonitis must be considered in any child with NS who has severe abdominal pain, fever
Complications of nephrotic Syndrome
Hypercoagulable state-
- intravascularly dry, they have higher Hb, plt AND deficient in different clotting factors
- Loss of anticoagulant factors
Infection and SBP
Hyperlipidemia
• Usually the last abnormal parameter to correct
What to do about vaccinations with nephrotic syndrome
All regular childhood immunizations
Live vaccines
>3months off prednisone or cyclosporine
>6 mo off cyclophosphamide
Annual flu vaccine
Most common etiology of nephrotic syndrome
minimal change disease
Definition of Glomerularnephritis
hematuria, proteinuria, edema, and often hypertension
Most common cause of microscopic hematuria
Hypercalciuria
Most common cause of gross hematuria
cystitis
What to do about gross hematuria
ALWAYS DO AN US
Systemic presentation with GN think of what differential?
Multi-System involvement
• Lupus
• ANCA vasculitides
GN + palm hemorrage is what differential?
GN plus pulmonary hemorrhage • Granulomatosis with Polyangitis • Anti-GBM disease • Lupus, HSP, infective endocarditis • AKI and pulmonary edema
PIGN occurs how long after infection?
Onset of GN:
– Cellulitis – 5 days after if co-infection with staph aureus otherwise 3 weeks post infection
– Pharyngitis – 7-10 days after
What investigations would you do for PIGN and what would the results be?
Low complement 3 – 90% will be low at presentation
• Normal complement 4
• ANA negative
• ASO titer may elevated
• Urine: red cells, red cell casts, positive for leukocytes
Management of PIGN
conservative management.
restrict fluid
may need diuretic
• Treat hypertension – resolves by 2 weeks
• C3 level should be back to normal in 6 to 8 weeks
• Proteinuria may last for 4 months
• Microscopic hematuria may last for 2 years though most cases resolve in 3-6 months
How long does it take C3 to normalize after PIGN?
8 weeks
Differential for low C3
Lupus – PSGN – Membranoproliferative glomerulonephritis – C3 glomerulopathy – Shunt nephritis – Subacute bacterial endocarditis
IgA Nephropathy presentation differs from PIGN how?
Presents WHILE they are sick!!
Gross hematuria occurs with URI
Treatment of IgA Nephropathy
ACE inhibitors, fish oil, steroids and immunosuppressive therapy
Prognosis for IgA Nephropathy
Among patients who develop overt proteinuria and/or an elevated serum creatinine concentration, progression to end-stage renal disease is approximately 15 to 25 percent at 10 years and 20 to 30 percent at 20 years
What is HSP?
IgA Nephropathy
- Usually children 2 to 6 years of age, often under 10 years of age
- Joint and/or abd pain can precede rash by up to 14 days in 30-40%
- 35% of children with HSP will have some renal involvement however 6-7% will have Acute GN or Nephrotic syndrome
- Scrotal Edema
- Hypertension
How to follow your patients with HSP
Weekly BP and Urine x 4 weeks
Every other week BP and Urine x 8 weeks
Monthly BP and urine until 6-12 months
If they develop macroscopic hematuria or hypertension refer to nephro
When to biopsy your patients with HSP
Indications to biopsy:
– Acute renal impairment/Nephritic Syndrome at presentation
– Nephrotic Syndrome with normal creatinine at 4 weeks
– Protein:Creatinine > 250mg/mmol at 4-6 weeks if not improving
– Persistent proteinuria > 100 mg/mmol of Cr at 3 months
1% of all patients with HSP patients will develop ESRD
– 11-13% of children with HSP who develop hematuria/proteinuria will have some degree of renal impairment
– 35-44% of those with nephrotic or nephritic presentation will have renal impairment
What is the inheritance of Alport Syndrome
X Linked
Wha is Alport Syndrome
Disease of collagen type IV that affects the basement membrane of the glomerular capillary as well as causing nerve deafness, eye problems and platelet defects.
Clinical features of microhematuria, hypertension proteinuria and renal failure
Associated with hearing loss
- Electron microscopy: abnormally thin basement membrame
- Need renal replacement therapy 20 to 30 years of age
Anterior lenticonus – localized conical Bulging of the lens into the anterior chamber Will cause abnormal refraction not correctable by specs. 90% associated with Alport’s
Which vasculitides are ANCA positive
Include:
– Granulomatosis with polyangiitis – GPA
– Microscopic polyangiitis
– Renal-limited Vasculitis
Clinical presentation of ANCA diseases
Fever
• Migratory arthralgias
• Malaise, Anorexia, Weight loss
ENT: – Chronic sinusitis – Bloody nasal discharge – Oral and/or nasal ulcers – Bony and cartilage destruction
• Pulmonary:
– Hoarseness, cough, dyspnea, stridor, wheeze
– Hemoptysis, pleuritic pain
• Renal
– 18 % GN at presentation
– 85% with in 2 years of diagnosis
• Cutaneous findings:
– Leukocytoclastic angiitis: Purpura with focal necrosis and ulceration
Anti GBM Presentation
Circulating antibody targeting the alpha-3 chain of a type IV collagen
• GBM in kidneys and pulmonary aveoli
• Usually systemic symptoms such as malasie, fever, weight loss, arthralgias are absent
– If present thing of presence of other vasculitic processes
Anti GBM Treatment
Plasmapheresis to remove antibodies
Lupus Diagnosis
Peak incidence: adolescence/adulthood
• Immune complex-mediated glomerular injury
• hematuria, proteinuria ± acute renal failure
• Bx: classification (WHO)
DIAGNOSTIC CRITERIA 4of 11: – Discoid rash – Malar rash – Photosensitivity – Aphthos ulcers: nares, gums – Arthralgia/arthritis • 2 or more joints – Serositis – pericarditis/plueritis • ANA • Immune markers: dsDNA, false VDRL, anti-Sm, anti-Ro • Nephritis • Neuro – seizures,psychosis • Blood: – Hemolytic anemia or – Leukopenia < 4 or – Lymphopenia < 1.5 or – Platelets < 100 000
Lupus Treatment
- Steroids plus immunosuppressive therapy
* Cyclophosphamide versus mycophenolate mofetil
Who are the high risk populations who have to have HTN Screening?
Prematurity (<32 WGA, SGA, IUGR, UAC) CHD recurrent UTI, hematuria, proteinuria known renal or urologic anomalies FHx of congenital renal disease Solid organ transplant Malignancy or BM transplant Treatment with drugs known to raise BP other systemic illnesses known to cause HTN (TS, NF, SCD) Evidence of ICP
Most common cause of secondary hypertension
Most common cause of secondary hypertension (after 1 year of age):
– Renal – parenchymal and renovascular
• 34 to 79%
• Renovascular disease 13-15%
Investigation of secondary hypertension
- The investigation with the greatest yield – Renal US with Doppler
- Confirmation requires either CT angio/MR angio or angiogram
Best way to assess evidence of end organ injury in children with hypertension
Echocardiogram for LVH
Treatment of HTN: First line
Weight reduction – Exercise – Decrease in salt intake – Smoking cessation – Monitoring of dyslipidemia
What drug to use in HTN
• ACE inhibitor, ARB, long-acting calcium channel blocker, or a thiazide diuretic.
- CCB easy to get in suspension easy to titrate the dose
- Because African American children may not have as robust a response to ACE inhibitors, thiazide is usually the preferred choice
• β-blockers are not recommended as initial treatment in children.
First line med for children with HTN and CKD, proteinuria, or diabetes mellitus
ACE inhibitor or ARB is recommended as the initial antihypertensive agent unless there is an absolute contraindication.
Initial work up in child with Stage 1 HTN
Urinalysis • Electrolytes • Urea, Creatinine • Lipid profile • If obese, (BMI > 95th percentile) consider Hb A1C, AST, ALT
Can children with stage 1 HTN participate in sports?
Children and adolescents with HTN should have target end- organ effects and cardiovascular risk factors assessed before returning to sports – echocardiogram, fundoscopy ???
• If end-organ injury is identified, recommendation is NOT to participate in competitive sports until resolution
• Children and adolescents with HTN should be below Stage 2 HTN definitions before participation in competitive sports even in the absence of end-organ damage
Immunosuppres transplant kid…what resp infections?
Mycoplasma pneumoniae
– CMV pneumonitis
– Pneumocystis jirovecii
What class of antibiotics should generally be avoided in those patients on calcineurin inhibitors (ie tacrolimus, cyclosporine)
• macrolides
What is the concern of EBV in a post transplant patient?
The concern with EBV is the development of Post-Transplant Lymphoproliferative Disorder
• See in the up to 10% of patients
• Mortality has been reported between 40- 60%
• Direct consequence of too much immunosuppression
How to treat EBV in transplant patient?
If just viremia
–Reducing immunosuppression and watching PCR closely
• IF confirmed PTLD:
–stopping immunosuppression particularly MMF and Tacrolimus
–May need chemotherapy
What is the most common infectious complication from kidney transplant?
Most common infectious complication following transplant
–As high as 30% of patients will experience at least 1 UTI –30% risk of developing urosepsis
–Only presenting complaint might be fever
–Increased creatinine often seen
–Higher risk of rejection as well
• Risk Factors:
–Immunosuppression
–Preexisting urological tract abnormalities
–Kidney transplant – short ureter, no tunnel
–Ureteric Stent
What vaccines can be given post kidney transplant?
Live-attenuated vaccines are contraindicated
–MMR, varicella,
• Travel Vaccines that are considered safe:
–Hepatitis A, Japanese encephalitis, inactivated typhoid
• NOT Yellow Fever
What are characteristics of simple renal cysts?
Simple cysts of the kidney are pretty rare in paediatrics
• that is why we follow them annually with ultrasound
• probably around 1-2%
• prevalence increases after 20 years of age to around 5 -7 %
• By definition: – less than 1 cm – usually less than 3 all together – usually unilateral – increase slowly in 25% of cases
When to consider the cyst is more than a simple cyst?
CT scan and referral if: – calcification – septae – loculations – thickened walls
In MCDK what happens to the contralateral kidney
Associated with contralateral:
– Hypoplasia
–UPJ
– Reflux (4-14%) – often mild
Hypertension: rare
Malginancy risk: considerable less than 1 in 1 000
– Natural history: involute 30 months
Associations with Renal Dysplasia
Reflux, PUV, spina bifida
Clinical Triad of Prune Belly Syndrome
– Abdominal muscle deficiency – partial or complete • May also see malrotation, anorectal anomolies
– Severe urinary tract abnormalities – Hydronephrosis, dilated ureters, distended bladder
– Bilateral cryptorchidism
OTHER:
–Skeleton – clubfoot, dislocated hips, kyphoscoliosis, polydactyly, torticollis
–Lungs – hypoplasia
–Heart - rare
Characteristics of ADPCKD
Normal/increased kidney size
AD inheritance Extrarenal • Liver cysts • Cerebral aneurysms • Liver fibrosis
Gene
PKD1 or PKD2
PCKD1 and TSC2 both on Chr 16
Characteristics of Renal Cysts and Diabetes
Normal kidney size
Inheritance: AD
- HypoMg
- MODY before 25 yrs
- Possible uterine abnormalities
HNF1-β
Characteristics of ARPCKD
Enlarged and echogenic kidneys
Inheritance AR
• Liver fibrosis
Gene: PKHD1
Risk factors for developing HUS
Extremes of age < 5 years and > 75 years Vomiting > 3 days of diarrhea Blood in stool White count > 13
What is HUS?
Simultaneous occurrence of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury
Primarily resulted from Shiga toxin-producing Escherichia coli (STEC) infections, and less frequently from Shigella dysenteriae type 1 infection.
Classic Presentation of HUS
Infection
Diarrhea (up to two weeks)
THEN HUS presents: thrombocytopenia, MAHA, AKI
70% resolve
30% long term renal or neurologic sequelae
Management of HUS
Best Supportive Care (BCS)
– Good hydration
• Accurate monitoring of fluids
• If becoming oliguric - UO < 0.5ml/kg/hr X 6 hours – Insensibles 400ml/m2 + 1:1 replacement of losses
What does Bartter’s Syndrome Look like?
Classically thought of as being on LOOP
diuretic
Polyuria, polydipsia, salt craving
What doe Gitelman Syndrome present like?
Thought of as being on a thiazide diuretic
Tend to present later in childhood/adolescence
– Weakness and fatigue
– Muscle cramps
– palpitations
Calculation for plasma osmolality
Measure of osmoles of solute per unit of solvent
= 2Na + Glucose + BUN
-Affected by water content
Causes of SIADH
Etiology – Enhanced hypothalamic secretion or ectopic production ADH
- CNS/Pulmonary/Neoplastic
- Drugs : opioids, SSRI, antiepileptics,
psychotropic, chemotherapeutic
Lab findings for SIADH
Laboratory:
hypotonic…low serum osm
increased ADH…inappropriate elevated Urine osm (> 100)
urine sodium >20meq/l
low serum uric acid and high urine excretion of uric acid
Treatment of SIADH
Treat with Fluid Restriction and Remove offending agent
Biggest difference between SIADH and cerebral salt wasting
Signs of hypovolemia!!!! with CSW Concentrated urine UNa >20meq/l High urinary excretion of urate Hypouricemia!!!
Treatment of CSW
Treatment with salt and fluid replacement. Spontaneously resolves
Side effect if you correct Na too quickly in hyponatremia
Correcting too quickly can lead to osmotic demyelination
How fast can you correct hyponatremia
Treat with 3% saline until symptoms resolve
Na increase by ~5meq/l or serum sodium >120meq/l
Overall aim for slow correction < 0.5mmol/l/hr or =10- 12mmol/day
What is cystinosis
- autosomal recessive
- lysosomal storage disease
- accumulation of cysteine in various organs
- measurement of cysteine levels in leucocytes can confirm diagnosis
What imaging do you do with first febrile UTI 2months- 2 years of age?
Ultrasound
During or within 2 weeks of presentation
Less invasive, available, less expensive
VCUG Pursue if renal abnormalities, obstruction, signs of VUR on RUS or second UTI Hydronephrosis, hydroureter Small kidneys Thick walled bladder Renal parenchyma abnormal – scars, cysts
What other renal anomalies are associated with VUR?
Occurs commonly with other renal anomalies multicystic dysplastic solitary duplex ectopic kidney
Which grades of reflux get prophylactic antibiotics?
No prophylaxis while awaiting RUS
Prophylaxis until VCUG controversial
No prophylaxis grades I-III. Consider Grade IV-V or significant urological anomaly
Minimize use 3-6 mos
TMP/SMX and Nitrofurantoin recommended unless resistant
If resistant to both consider d/c. Broader spectrum leads to more resistance
What does a DMSA scan look for?
Radioactive tracer introduced IV and taken up by active renal tissue
Defects indicate acutely pyelonephritis or late sequelae of scarring (4-6mos post UTI).
Best test to detect renal scars
Invasive, radiation, expense, less available
What does a DTPA with lasixscan look for?
Diuretic renography – DTPA scan with lasix
Examines renal perfusion, uptakes, excretion, drainage
Tells you whether there is an obstruction
Definition of Antenatal Hydronephrosis
ANH is present if the APD is ≥4 mm in second trimester and ≥7 mm in the third trimester.
Most common etiology of antenatal hydronephrosis
transient
then PUJO
and VUR
Treatment of Antenatal hydronephrosis
Start baby on 2mg/kg qd trimethoprim
Mild/moderate isolated ANH
- feeding well and has voided baby may be discharged - Arrange ultrasound after the 3rd day and no later than 14 days old
ANH that is severe, signs of PUV, oligohydramnios, dysplastic kidneys
- Monitor urine output
- Ultrasound before discharge
- Nephrology/urology
1) Renal pelvis < 1cm Stop TMP no more tests, counsel about uti 2) Renal pevis 1-1.2cm Stay on TMP Repeat us 3mo 3) Renal pelvis > or = 1.2cm Stay on TMP Do a vcug Consider lasix washout scan to rule out UPJ obstruction
UPJ Characteristics
Congenital anomaly
Stenosis due to lack of cell apoptosis
Present with progressive hydronephrosis, recurrent UTI, gross hematuria, flank pain
Diagnosis with Renal Diuretic scan – DTPA Lasix scan
Surgical correction with pyloplasty
Post natal management of PUV
TMP prophylaxis Early ultrasound and VCUG Monitor urine output Blood work – lytes/urea/creatinine Call urology Need a catheter but can be hard to put in
What are the common features of Prune Belly Syndrome
- Partial or complete absence of abdominal wall musculature
- Cryptorchidism
- Urinary tract malformation
Hydronephrosis/hydroureter
Bladder distension
Urinary tract obstruction
VUR
How do you monitor/treat solitary kidney
No VCUG if ultrasound is otherwise normal
No antibiotics
Follow by ultrasound to be sure it grows appropriately
Yearly urinalysis, BP
Contact sports – no longer advise against it
What investigations would you do for a renal stone?
Lytes, TCO2, calcium, Mg, Po4, urate, urea, creatinine \+/- PTH, vit D levels Urine spot calcium, creatinine Urine analysis – pH, specific gravity 24 hour urine : calcium, oxalate, citrate, urate, cystine creatinine, volume Renal ultrasound Abdo Xray – spots ureteral stones CT – almost never
Acute Management of Renal Stone
Control pain IV fluids Urology consult Consider oxybutynin Ask family to strain urine/check diaper send any stones for analysis
What are the indications for dialysis
A cidosis
E lectrolyte abnormalities – hyperkalemia, hyponatremia, hyperphosphatemia
I ntoxication/poisoning – ethylene glycol, ASA, lithium
O verload – fluid
U remia - mental status change, pericarditis
Treatment for orthostatic proteinuria
None! Reassurance
How do you diagnose Diabetic nephropathy
Diagnosis: First morning urine showing albumin:creatinine ratio (ACR) >2.5mg/mmol on two occasions
How do you treat diabetic nephropathy
Treatment: ACE inhibitors
Inhibit the renin-angiotensin system (RAAS) which results in decreased urinary protein excretion
Nephritic Syndrome diagnostic criteria
Diagnostic Criteria:
Gross hematuria
Hypertension (due to salt and water overload)
RBC or granular casts
Other common features:
Azotemia (elevated urea and creatinine)
Edema
Proteinuria (usually non-nephrotic range)
IgA nephropathy clinical presentation
First episode usually occurs between 15 -30y of age
Recurrent episodes of gross hematuria concomitant with infection (synpharyngitic)
Gross hematuria lasts <3 days
Microscopic hematuria and/or proteinuria may be the only signs
Not often associated with complete nephritic syndrome
Common HSP NEPHRITIS presentation
Common manifestations
Microscopic hematuria, macroscopic hematuria
Albuminuria
Mixed nephritic-nephrotic syndrome (severe cases)
Renal impairment
Hypertension
How do you treat HSP Nephritis
Treatment is varied:
No support to steroids in prevention of nephritis
ACEi for moderate HSP nephritis
Steroids for severe pain or for persistent nephrotic range proteinuria
What are the complements like in SLE?
Low C3 and C4
Treatment of Lupus Nephritis
Multi-disciplinary team
Immunosuppressive induction therapy: Corticosteroids, MMF, +/- Rituximab, +/- CNI
Maintenance therapy: Corticosteroids, MMF, Hydroxychloroquine
HUS Lab Findings
*MAHA - check CBC, LDH, haptoglobin, bilirubin, blood smear (schistocytes)
May have elevated LFTs
HUS pathogenic bugs
E Coli O157:H7 accounts for 70-90% of all cases of typical HUS; Shigella and S Pneumo are other possible pathogens
*Positive stool culture not necessary for diagnosis
When does HUS present in relation to GI symptoms
HUS develops 5-13 days after the onset of GI symptoms (bloody stools in 90%)
What is part of the initial HTN work up
Blood: CBC, electrolytes (including TCO2, extended), Cr/BUN, (fasting) lipids, optional: LFTs, TSH, renin, aldosterone, catecholamines, Hb A1C, drug screen
Urine: Urinalysis + microscopy, ACR, +/- urine metanephrines
Imaging: Renal US (+ Doppler), CXR
Functional tests: ECG, 24h ambulatory BP monitoring, +/- sleep study
What are the 4 organ systems that should be checked for end-organ damage in a hypertensive paediatric patient?
- Kidney
- Heart
- Eye
- CNS
HTN Treatment
Emergency vs chronic
Lifestyle:
DASH (Dietary Approaches to Stop Hypertension) diet
Moderate - vigorous physical exercise 3-5d/wk for 30-60mins
Stress Reduction
Pharmacologic Management: Start with ACEi, ARB, long-acting calcium channel blocker (Amlodipine), or thiazide
