Nephrology Flashcards

Question

What is the main defect in Type 1 RTA?

Answer 1

Inability of the distal tubule to secrete Hydrogen Ion

Answer 2

Decreased Ability of proximal tubule to reabsorb bicarb.

Answer 3

``` Corticosteroids Decongestants Nonsteroidal anti-inflammatory medications Herbal supplements β-adrenergic agonists Erythropoietin Cyclosporine Tacrolimus Stimulants *Recent discontinuation of antihypertensive medications ```

Answer 4

Age Gender Height

Answer 5

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)

Answer 6

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

Answer 7

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

Answer 8

Cerebral Aneurysm

Answer 9

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

Answer 10

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 ```

Answer 11

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

Answer 12

Hypercalciuria. Usually idiopathic 30%

Answer 13

Alport Syndrome

Answer 14

Most common inheritance: X-linked (*Maternal Uncle) Males affected Female carriers

Answer 15

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

Answer 16

Associated Proteinuria

Answer 17

Orthostatic proteinuria | Transient proteinuria: fever, stress, exercise, cold, abdominal surgery

Answer 18

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

Answer 19

Bright red hematuria is usually indicative of lower urinary tract bleeding

Answer 20

glomerular hematuria (as in nephritis) is commonly described as cola, tea, or brown colored.

Answer 21

YEARLY screening starting at age 12 years in those with T1DM for > 5 years First morning or random ACR

Answer 22

Renal (55%)

Answer 23

1. Hydronephrosis (#1) | 2. Multicystic dysplastic kidney

Answer 24

Mesenteric Cyst

Answer 25

Abdo Ultrasound

Answer 26

A. generally harmless B. less expense C. no sedation D. ability to perform at the bedside

Answer 27

154 meq/L Na 154 meq/L Cl Osm= 154+ 154= 308

Answer 28

square root of height x weight /3600

Answer 29

4-6mg/kg/min

Answer 30

Na-Cl-HCO3

Answer 31

A. Renal tubular acidosis | B. Gastroenteritis

Answer 32

``` Proximal RTA • Urine pH<7 • Acidosis – HCO3 15 • Often hypoNa • Can have hypoPhos • Glucosuria • Mild tubular proteinuria or generalized aminoaciduria • Fanconi’s syndrome ```

Answer 33

* Urine pH>7 * Acidosis – HCO3 can go below 10 * Sodium usually normal * Nephrocalcinosis may be present

Answer 34

Idiopathic ``` Familial Secondary • Ifofamide • Valporate • Amphotericin • Lupus • Obstructive uropathy • Renal dysplasia • Lithium Carbonate ```

Answer 35

``` – Metabolic • Cystinosis = most common • Glycogen storage diseases • Wilson’s • Tyrosinemia • Galactosemia ``` Idiopathic ``` Acquired • Ifosfamide • Carbonic anhydrase inhibitors • Lead • Paroxysmal nocturnal hemoglobinuria ```

Answer 36

``` Aldosterone Deficiency – CAH – Acute GN – ACEI – NSAIDs ``` ``` Aldosterone Resistance – Spironolactone – Dysplasia – CKD – Pseudohypoaldosteroni sm ```

Answer 37

Potassium Citrate Checking for hearing loss Genetic testing

Answer 38

Proteinuria

Answer 39

Transient proteinuria • Exercise • Fever • Infection Prevalence: 5 -15%

Answer 40

Defined as proteinuria on 2 or 3 separate samples take 1-2 weeks apart

Answer 41

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

Answer 42

Protein/creatinine ratio

Answer 43

Normal: Infants (< 2 years of age) – Upc < 50 mg/mmol Children (> 2 years of age) – Upc < 20 mg/mmol

Answer 44

Orthostatic Proteinuria

Answer 45

Assess with two separate night and daytime sample | If AM sample Upc < 20, diagnosis can be made

Answer 46

- X-linked recessive – Proteinuria, hypercalciuria, nephrolithiasis and/or medullary nephrocalcinosis – Also look for glycosuria, aminoaciduria, phosphate wasting

Answer 47

* <18months or >10 years of age * Positive family history * Extra-renal manifestations * Chronic disease * Hypertension * Acute renal failure at presentation • +/- Hematuria

Answer 48

``` 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 ```

Answer 49

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

Answer 50

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

Answer 51

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

Answer 52

Management of Steroid Dependent NS (SDNS) and Steroid Resistant NS (SRNS): ``` – Prednsione – Trial of cyclophosphamide, tacrolimus – Consider MMF or cyclosporine – Biopsy – Consider genetic testing ```

Answer 53

Steroid Resistant (SRNS): Inability to induce remission with 4 weeks of daily steroid therapy

Answer 54

Steroid dependent (SDNS): Relapse during taper or within 2 weeks of discontinuation of steroids

Answer 55

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

Answer 56

``` 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

Answer 57

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

Answer 58

All regular childhood immunizations Live vaccines >3months off prednisone or cyclosporine >6 mo off cyclophosphamide Annual flu vaccine

Answer 59

minimal change disease

Answer 60

hematuria, proteinuria, edema, and often hypertension

Answer 61

Hypercalciuria

Answer 62

ALWAYS DO AN US

Answer 63

Multi-System involvement • Lupus • ANCA vasculitides

Answer 64

``` GN plus pulmonary hemorrhage • Granulomatosis with Polyangitis • Anti-GBM disease • Lupus, HSP, infective endocarditis • AKI and pulmonary edema ```

Answer 65

Onset of GN: – Cellulitis – 5 days after if co-infection with staph aureus otherwise 3 weeks post infection – Pharyngitis – 7-10 days after

Answer 66

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

Answer 67

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

Answer 68

``` Lupus – PSGN – Membranoproliferative glomerulonephritis – C3 glomerulopathy – Shunt nephritis – Subacute bacterial endocarditis ```

Answer 69

Presents WHILE they are sick!! | Gross hematuria occurs with URI

Answer 70

ACE inhibitors, fish oil, steroids and immunosuppressive therapy

Answer 71

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

Answer 72

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

Answer 73

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

Answer 74

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

Answer 75

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

Answer 76

Include: – Granulomatosis with polyangiitis – GPA – Microscopic polyangiitis – Renal-limited Vasculitis

Answer 77

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

Answer 78

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

Answer 79

Plasmapheresis to remove antibodies

Answer 80

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 ```

Answer 81

* Steroids plus immunosuppressive therapy | * Cyclophosphamide versus mycophenolate mofetil

Answer 82

``` 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 ```

Answer 83

Most common cause of secondary hypertension (after 1 year of age): – Renal – parenchymal and renovascular • 34 to 79% • Renovascular disease 13-15%

Answer 84

* The investigation with the greatest yield – Renal US with Doppler * Confirmation requires either CT angio/MR angio or angiogram

Answer 85

Echocardiogram for LVH

Answer 86

``` Weight reduction – Exercise – Decrease in salt intake – Smoking cessation – Monitoring of dyslipidemia ```

Answer 87

• 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.

Answer 88

ACE inhibitor or ARB is recommended as the initial antihypertensive agent unless there is an absolute contraindication.

Answer 89

``` Urinalysis • Electrolytes • Urea, Creatinine • Lipid profile • If obese, (BMI > 95th percentile) consider Hb A1C, AST, ALT ```

Answer 90

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

Answer 91

Mycoplasma pneumoniae – CMV pneumonitis – Pneumocystis jirovecii

Answer 92

• macrolides

Answer 93

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

Answer 94

If just viremia –Reducing immunosuppression and watching PCR closely • IF confirmed PTLD: –stopping immunosuppression particularly MMF and Tacrolimus –May need chemotherapy

Answer 95

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

Answer 96

Live-attenuated vaccines are contraindicated –MMR, varicella, • Travel Vaccines that are considered safe: –Hepatitis A, Japanese encephalitis, inactivated typhoid • NOT Yellow Fever

Answer 97

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 ```

Answer 98

``` CT scan and referral if: – calcification – septae – loculations – thickened walls ```

Answer 99

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

Answer 100

Reflux, PUV, spina bifida

Answer 101

– 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

Answer 102

Normal/increased kidney size ``` AD inheritance Extrarenal • Liver cysts • Cerebral aneurysms • Liver fibrosis ``` Gene PKD1 or PKD2 PCKD1 and TSC2 both on Chr 16

Answer 103

Normal kidney size Inheritance: AD * HypoMg * MODY before 25 yrs * Possible uterine abnormalities HNF1-β

Answer 104

Enlarged and echogenic kidneys Inheritance AR • Liver fibrosis Gene: PKHD1

Answer 105

``` Extremes of age < 5 years and > 75 years Vomiting > 3 days of diarrhea Blood in stool White count > 13 ```

Answer 106

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.

Answer 107

Infection Diarrhea (up to two weeks) THEN HUS presents: thrombocytopenia, MAHA, AKI 70% resolve 30% long term renal or neurologic sequelae

Answer 108

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

Answer 109

Classically thought of as being on LOOP diuretic Polyuria, polydipsia, salt craving

Answer 110

Thought of as being on a thiazide diuretic Tend to present later in childhood/adolescence – Weakness and fatigue – Muscle cramps – palpitations

Answer 111

Measure of osmoles of solute per unit of solvent = 2Na + Glucose + BUN -Affected by water content

Answer 112

Etiology – Enhanced hypothalamic secretion or ectopic production ADH - CNS/Pulmonary/Neoplastic - Drugs : opioids, SSRI, antiepileptics, psychotropic, chemotherapeutic

Answer 113

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

Answer 114

Treat with Fluid Restriction and Remove offending agent

Answer 115

``` Signs of hypovolemia!!!! with CSW Concentrated urine UNa >20meq/l High urinary excretion of urate Hypouricemia!!! ```

Answer 116

Treatment with salt and fluid replacement. Spontaneously resolves

Answer 117

Correcting too quickly can lead to osmotic demyelination

Answer 118

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

Answer 119

- autosomal recessive - lysosomal storage disease - accumulation of cysteine in various organs - measurement of cysteine levels in leucocytes can confirm diagnosis

Answer 120

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 ```

Answer 121

``` Occurs commonly with other renal anomalies multicystic dysplastic solitary duplex ectopic kidney ```

Answer 122

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

Answer 123

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

Answer 124

Diuretic renography – DTPA scan with lasix Examines renal perfusion, uptakes, excretion, drainage Tells you whether there is an obstruction

Answer 125

ANH is present if the APD is ≥4 mm in second trimester and ≥7 mm in the third trimester.

Answer 126

transient then PUJO and VUR

Answer 127

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 ```

Answer 128

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

Answer 129

``` 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 ```

Answer 130

- Partial or complete absence of abdominal wall musculature - Cryptorchidism - Urinary tract malformation Hydronephrosis/hydroureter Bladder distension Urinary tract obstruction VUR

Answer 131

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

Answer 132

``` 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 ```

Answer 133

``` Control pain IV fluids Urology consult Consider oxybutynin Ask family to strain urine/check diaper send any stones for analysis ```

Answer 134

A cidosis E lectrolyte abnormalities – hyperkalemia, hyponatremia, hyperphosphatemia I ntoxication/poisoning – ethylene glycol, ASA, lithium O verload – fluid U remia - mental status change, pericarditis

Answer 135

None! Reassurance

Answer 136

Diagnosis: First morning urine showing albumin:creatinine ratio (ACR) >2.5mg/mmol on two occasions

Answer 137

Treatment: ACE inhibitors Inhibit the renin-angiotensin system (RAAS) which results in decreased urinary protein excretion

Answer 138

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)

Answer 139

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

Answer 140

Common manifestations Microscopic hematuria, macroscopic hematuria Albuminuria Mixed nephritic-nephrotic syndrome (severe cases) Renal impairment Hypertension

Answer 141

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

Answer 142

Low C3 and C4

Answer 143

Multi-disciplinary team Immunosuppressive induction therapy: Corticosteroids, MMF, +/- Rituximab, +/- CNI Maintenance therapy: Corticosteroids, MMF, Hydroxychloroquine

Answer 144

*MAHA - check CBC, LDH, haptoglobin, bilirubin, blood smear (schistocytes) May have elevated LFTs

Answer 145

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

Answer 146

HUS develops 5-13 days after the onset of GI symptoms (bloody stools in 90%)

Answer 147

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

Answer 148

1. Kidney 2. Heart 3. Eye 4. CNS

Answer 149

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