Renal System Flashcards

1
Q

What type of vasculitis is anti-GBM disease

A

Small vessel vasculitis. antibodies are produced against Type 4 collagen
2x MC in men
Bimodal age 20-30 yrs & 60-70 yrs

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2
Q

Renal biopsy of Anti-GBM disease

A

Linear IgG deposits along Basement memb.

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3
Q

Anti-GBM disease Rx.

A

Plasmapheresis
Steroids
Cyclophosphamide

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4
Q

MCC of Renal vascular disease

A

Atherosclerosis
Fibromuscular disease (FMD)

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5
Q

Ix & Rx. of Renal vascular disease

A

IOC - MR Angio
Balloon Angioplasty

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6
Q

Fibromuscular disease (FMD) features

A

Young Female + HTN + CKD/ ARF (secondary to ACEi initiation) + Flash Pulm. edema

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7
Q

Retroperitoneal Fibrosis C/F

A

Low back/ Flank pain + Fever & LL edema

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8
Q

Association of Retro P Fibrosis

A

Riedal’s Thyroiditis
Previous RT
Sarcoidosis
Drug METHYSERGIDE

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9
Q

Rhabdomyolysis biochemical results??

A

Elevated Cr., CK
Myoglobinuria
Hypocalcaemia (Myoglobin binds to Ca2+)
Elevated K+, PO4-
METABOLIC ACIDOSIS

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10
Q

Rhabdomyolysis Rx.

A

IV fluids
Urinary Alkalinization

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11
Q

Rhabdomyolysis Causes

A

Fall at home for long hrs
McArdles syndrome
ECSTACY

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12
Q

Time taken for AV fistula to develop

A

6 to 8 weeks

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13
Q

Complications of AV fistula

A

Infection
Thrombosis (ABSENCE of Bruit)
Stenosis
STEAL Syndrome

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14
Q

Plasmapheresis Indications

A

GBS, Goodpasture’s,
Myasthenia gravis
ANCA +ve Vasculitis
TTP, HUS
Cryoglobulinaemia
Hyperviscosity synd.

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15
Q

Plasmapheresis Complications?

A

Hypocalcaemia (due to Citrate)
Coagulation factor depletion
IGs depletion
Met. Alkalosis

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16
Q

HLA/ MHC is located on which Chromosome ??

A

Chr. 6
Class 1 antigen - A, B, C
Class 2 antigen - DP, DQ, DR

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17
Q

Renal transplant imp. HLA

A

DR»>B»>A

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18
Q

Graft survival of type of donor??

A

Living donor»>Cadaveric donor

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19
Q

Hyperacute Graft Rejection type of HS reaction???

A

1) Hyperacute - Minutes to Hrs.,
Type 2 HS reaction
Caused by Pre-existing antibodies against ABO or HLA antigens.
Rx. Remove the graft

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20
Q

Type of Graft Rejection

A

1) Hyperacute (min to hrs)
2) Acute (< 6 months)
3) Chronic (> 6 months)

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21
Q

Acute Graft rejection is due to??

A

Mismatched HLA. CMI (cytotoxic T cells)
MONOCLONAL cell infiltrate predominates
CMV infection
Rx. Steroids & Immunosuppresants

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22
Q

Chronic Graft Rejection is due to ??

A

Both Antibody + CMI mechanisms
results in Fibrosis of transplanted kidney (CHRONIC ALLOGRAFT Nephropathy)

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23
Q

Rx. for Renal transplant??

A

Immunosuppression
1) Initial = Ciclosporin/ Tacrolimus + a Monoclonal antibody
2) Maintenance = Ciclosporin/ Tacrolimus + MpMF or Sirolimus
add Steroid if >= 1 steroid responsive acute rejection

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24
Q

Recurrence of original renal disease in Transplanted Kidney ???

A

MCGN»IgA»FSGS

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25
LUTS voiding features??
Hesitancy Poor/ Intermittent stream Straining Incomplete emptying Terminal dribbling
26
LUTS Storage C/F ??
Urgency Frequency Nocturia U Incontinence
27
LUTS post-micturition C/F ??
Post M dribbling Sensation of incomplete emptying
28
Rx. if predominantly voiding C/F???
-Conservative = Pelvic floor exercise, Bladder training, prudent Fluid intake -Moderate - severe symp. = ALPHA Blockers - Prostatomegaly + high risk of progression = 5 alpha reductase inhibitors - Prostatomegaly + moderate to severe symp. = Alpha blockers + 5 alpha reductase
29
Mixed Voiding & Storage symp. Rx. ??
Alpha blockers {If does not respond, Anti-Muscuranic (Anti-cholinergic) eg. tolterodine or darifenacin is added}
30
Predominantly Overactive bladder Rx. ??
-Fluid intake moderation -Bladder retraining -Anti-Muscarinic drugs (Oxybutinin, Tolterodine are immediate release & Darifenacin is once daily prep.) - Mirabegron [if 1st line drugs fail]
31
Nocturia Rx. ??
-Moderate Fluid intake at night - Furosemide 40mg in late afternoon - Desmopressin
32
Types of Haematuria??
Non-Visible (Microscopic or Dip-stick +ve) Visible (macroscopic)
33
Red or Orange Urine discolouration causes???
Beetroot, Rhubarb Doxorubicin, Rifampicin
34
IOC for Haematuria??
Urine dipstick
35
Renal colic analgesia of choice??
NSAIDs (Diclofenac) inpatients = Parenteral Diclofenac (IM)
36
What is Persistent Non-Visible Haematuria??
+ve blood in 2 of 3 samples tested 2-3 wks apart
37
What is the Surgical emergency in renal stones??
Ureteric obstruction (due to stones) + Infection Renals must be decompressed
38
Imaging in Renal Calculi??
-NCCT-KUB (in < 14hrs of admission) - If Fever/ solitary kidney/ Dx. uncertain (to exclude ruptured AAA)== Immediate CT-KUB
39
Oxalate stones prophylaxis??
- CHOLESTYRAMINE (reduces urine oxalate excretion) - PYRIDOXINE (reduces urine oxalate secretion)
40
Radio-Lucent stones??
- Urate stones - Xanthine stones
41
Urate stones prophylaxis??
Allopurinol Urinary Alkalinization (Oral HCO3-)
42
Ca2+ stones prophylaxis??
- High fluid intake - Low salt (NOT low Ca2+ diet) - Low animal protein diet - Thiazides (increases DCT Ca2+ resorption)
43
Semi-opaque or Ground glass appearance of stone cause??
Cysteine stones
44
MC type of renal stone??
Ca. OXALATE (40%)>Mixed Ca Oxalate/ Phosphate sones (25%) >> Triple Phosphate stones (10%) = Ca Phosphate (10%) >> Urate stones (5- 10%) >> Cysteine stones (1%) >> Xanthine (<1%)
45
Risk Factor for Urate stones??
- Gout - ILEOSTOMY (loss of HCO3- & fluids results in Acidic urine, causes ppt. of uric acid)
46
Drugs causing Ca2+ stones??
- Loop diuretics - Steroids - Acetazolamide - Theophylline
47
Stag-horn calculi features??
- Made of Struvite (Mg, NH4, PO4) - aka Triple phosphate or Infection stones - Renal pelvis + involves <= 2 calyces - develops in Alkaline urine - Common in UTIs
48
Rx. of Epididymo-orchitis ??
- Unknown organism = Ceftriaxone 500mg IM single dose + Doxycycline 100mg oral 1-0-1 for 10 to 14 days
49
MCC of Epididymo-orchitis??
- Local spread of infection from genital tract (C trachomatis, N gonorrhoea - STIs) - from Bladder (E coli typically seen in elderly & in low risk sexual history)
50
Organisms predisposing to Stag-horn calculi???
Ureaplasma urealyticum infection Proteus infection
51
Testicular torsion features??
< 20yrs + severe pain + acute onset
52
Ix. for Epididymo-orchitis??
- Young = look for STIs - Elderly + low risk sexual Hx. = MSU for microscopy & culture
53
BPH ethnicity variance ??
Black > White > Asians
54
Ix for suspected chronic retention in BPH ???
U & E
55
Indication for PSA testing ??
- Obstructive symp. - Pt. is worried about Prostate Ca
56
Rx. if Prostatomegaly (significant) + high risk of progression ??
5 alpha reductase inhibitors (Finasteride)
57
Rx. if IPSS >= 8 (moderate to severe voiding symp.) ??
Alpha-1 antagonists (Tamsulosin, Alfuzosin)
58
International Prostate Symp. Score (IPSS) ??
Tools used = Severity of LUTS & Impact of LUTS on quality of life - 0 to 7 = Mildly symptomatic - 8 to 19 = Moderately - 20 to 35 = Severely
59
When is a combination of Alpha 1 antagonist + 5 alpha reductase inhibitor used ??
Moderate to severe voiding symp. + Prostatomegaly
60
Chemotherapy DoC in Prostate Ca ??
Docetaxel
61
When is an Androgen syn. inhibitor (eg. ABIRATERONE) used in prostate Ca. ??
- Hormone relapsed metastatic prostate Ca. + - have no/ mild symp. after androgen deprivation therapy has failed + - Before Chemo is indicated eg.- ABIRATERONE
62
Rx. for Localized Prostate Ca (T1/ T2)??
- Conservative (active monitoring + watchful wait) - Radical prostatectomy - RT (Ext. beam & Brachytherapy)
63
Rx. for Localized advanced prostate ca. ??
- Hormone therapy - Radical Prostatectomy ( erectile dysfunc. is common complication) - RT (Ext. beam + Brachytherapy)
64
IoC to look for Renal scarring ??
DMSA
65
Steroidal anti androgen MoA ??
- Prevents DHT binding from Intracytoplasmic protein complex - eg.- CYPROTERONE ACETATE
66
What do you suspect in - Antenatal USS shows Hydronephrosis - Recurrent UTI in childhood - Chr. Pyelonephritis in children MCC
Vesico- Ureteric Reflux {Renal scarring can cause increased quantity of RENIN ==> HTN}
67
Why do we use Low dose, long term GnRH agonists (Zoladex) for hormonal therapy in Prostate Ca. ??
Low LH levels for long term ==> OVERSTIMULATION ==> disruption of Endogenous hormonal Feedback systems. The testosterone levels initially rise (Tumour Flare) before falling to castration levels
68
Non Steroidal anti-androgen MoA ??
- Blocks Androgen receptor - eg.- BICALUTAMIDE
69
RF for Urothelial (Transitional cell) CA ??
- Smoking (Hazard ratio is 4) - Aniline dyes (Printing & Textile industries) 2-Naphthylamine & Benzidine - Rubber manufacture - CYCLOPHOSPHAMIDE
70
Hormonal therapy for Prostate Ca. ??
Low dose, long term GnRH agonists (Zoladex) + Anti- androgen (initially to prevent Tumour flare)
71
Triad of RCC ??
Haematuria Loin pain Abdominal mass
72
IoC in VUR ??
Micturating Cystourethrogram
73
RF for Squamous Cell CA of Bladder ??
'SSS' - Schistosomiasis - Smoking
74
Grades of VUR ??
G1 - Reflux into ureter only + NO dilatation G2 - Reflux into Renal pelvis on micturition + NO DILATATION G3 - Mild/ Moderate dilatation of Ureter, Renal pelvis, Calyces G4 - Dilatation of Renal pelvis & Calyces + Moderate ureteral TORTUOSITY G5 - Gross dilatation of URETER, pelvis and calyces + ureteral Tortuosity
75
Site of RCC origin ??
Proximal Renal Tubular Epithelium - Clear cell CA (MC histological subtype)
76
What is Stauffer synd.??
- Paraneoplastic disorder a/w RCC - presents as Cholestasis/ Hepatosplenomegaly - Thought to be secondary to INCREASED levels of IL-6
77
Endocrine effects of RCC ??
May secrete- - Erythropoietin (Polycythaemia) - Parathyroid Hormone-related protein (Hypercalcaemia) - Renin - ACTH
78
What sided varicocele does RCC cause ??
- Majority are LEFT sided (due to compression by tumour)
79
RCC T categories of tumour ??
T1 : <= 7cm + confined to kidney T2 : > 7cm + confined to kidney T3 : Tumour extends to major veins or Perinephric tissue + but NOT into I/L Adrenals & NOT beyond Gerota's fascia T4 : Invades beyond Gerota's fascia + extension into I/L Adrenals
80
Rx. of RCC ??
T1 tumour : Partial Nephrectomy Reduce tumour size : Alpha-IFN & IL-2 {Note- Receptor TK inhibitors (Sorafenib, Sunitinib) are superiorly efficacious than Alpha-IFN}
81
Tumour Markers of testicular ca. ??
Germ cell tumour - Seminoma : hCG (elevated in 20%) - Non seminomas : AFP &/or Beta-hCG (elevated in 80%-85%) LDH is increased in 40% 0f Germ cell tumour
82
Types of Testicular Ca. ??
Germ Cell tumors (95% of Ca.) - Seminomas - Non seminomas : includes embryonal, yolk sac, Teratoma & ChorioCA Non-Germ cell tumours - Leydig cell tumour - Sarcomas
83
MCC of scrotal swelling seen in primary care ??
Epididymal cysts (MC found posterior to Testicles)
84
Why do we see Gynaecomastia in Testicular Ca ???
Due to increased Oestrogen : Androgen ratio - Germ cell tumour {hCG => Leydig cell dysfunc. => Increase in both O & T production (but O rise >> T rise)} - Leydig cell tumour {O secretion more + converts additional androgen precursors to O}
85
Features of Wilm's tumour ??
ABDOMINAL Mass (MC presenting feature) - Painless Haematuria - Flank pain - Anorexia, Fever - U/L in 95% cases Metastases in 20% cases (MC site is LUNG)
86
What is Hydrocele ??
Accumulation of fluid within TUNICA VAGINALIS
87
MC type of Hydrocele in Newborn Males ??
Communicating type (Peritoneal fluid drains down to scrotum via PROCESSUS VAGINALIS)
88
Which Testicular swelling occurs - Posterior to testes - Anterior to testes
- Epididymal cyst - Hydrocele (anterior & down)
89
What are the requirements for Maintenance fluids ??
25 to 30ml/kg/day of Water Approx. 1mmol/kg/day of K+, Na & Cl Approx. 50 to 100g/day of Glucose to limit Starvation ketosis
90
MC site of Varicocele ??
LEFT side (> 80%) Bag of worms Subfertility is common
91
0.9% NaCl in large volumes increase the risk of ??
Hyperchloraemic Met. Acidosis
92
Anion gap formulae ??
{(Na+) + (K+)} - {(HCO3-) + (Cl-)} Normal Anion gap is 8 to 12 mEq/L
93
CI for Hartmann's ??
Hyperkalaemia
94
Causes of increased Anion gap (>12 mEq/L) M acdosis ??
GOLDMARK: - Glycols (Ethylene g & Propylene g) - 5-Oxoproline (Chr. Acetaminophen use) - Lactic acidosis - Exogenous Lactic acid - Methanol (& other alcohols) - Aspirin (Late effect) - Renal failure (Urate) - Ketosis (Diabetic, Alcoholic, Starvation)
95
Causes of Normal Anion gap M acidosis ??
SHARDS FU - Spironolactone - Hyperchloremia/ Hyperalimentation - Addison's/ Acetazolamide use/ Ammonium Cl injection - Renal Tubular Acidosis - Diarrhoea - Saline Infusion - Fistula - Uterosigmoidoscopy
96
Types of Lactic Acidosis ??
Type A : Sepsis, shock, hypoxia, burns Type B : METFORMIN
97
Salycylates poisoning ABG changes??
Early- Resp. Alkalosis Late- Met. Acidosis
98
Drugs causing Hyperkalaemia ??
K+ sparing diuretics ACEi & ARBs Ciclosporin, Heparin (UnF & LMW) Beta-blockers in renal failure pts.
99
How does LMWH & UnFH causes Hyperkalaemia ??
by Inhibition of Aldosterone secretion
100
Hyperkalaemia classification ??
Mild = 5.5 to 5.9 mmol/L Moderate = 6.0 to 6.4 mmol/L Severe : >= 6.5 mmol/L
101
Sequential ECG changes in Hyperkalaemia ??
Peaked T waves (occurs 1st) Loss of P wave Broad QRS complex Sinusoidal wave pattern Asystole
102
What is Pseudo-Hyperkalaemia ??
Elevated K+ levels due to - Excess leak of K+ from cells during or after the blood is taken
103
Lab. modification to prevent Pseude-Hyperkalaemia ??
- use Lithium heparin tube - request Slow spin in centrifuge - do not apply excessive vacuum while drawing blood (causes haemolysis)
104
Gynaecomastia is less commonly seen with which K+ sparing diuretics ??
Eplerenone
105
Indication of Spiranolactone in HF (RALES) ??
NYHA 3 + 4 & pt. already on ACEi Low dose Spironolactone reduces ALL CAUSE MORTALITY
106
Indications of Spironolactone ??
- Ascites (Cirrhosis pts. develop Secondary Hyperaldosterone); large doses 100/200mg are used - HTN: Step 4 Rx. - Nephrotic - Conn's
107
Hypokalaemia ECG changes ??
ST depression Flat T wave U wave QT interval prolongation
108
Causes of Hypokalaemia with Alkalosis ??
Vomiting (H+ loss) Thiazides & Loop diuretics Cushing's synd. Conn's synd. (primary hyperaldosteronism)
109
Causes of Hypokalaemia with Acidosis ??
Diarrhoea (HCO3- loss) Renal Tubular Acidosis Acetazolamide Partially treated DKA Fanconi's synd.
110
Causes of Hypokalaemia with HTN ???
Cushing's synd. Conn's synd. Liddle's synd. (disordered Na+ channel in DCT) 11-beta Hydroxylase deficiency Anti-ulcer drug (CARBENOXOLONE) Liquorice excess
111
Causes of Hypokalaemia without HTN
Diuretica GI loss (diarrhoea & vomiting) Barter' synd. Gitelman synd. RTA (type 1 & 2)
112
What is "Fanconi's BaGeLS" ??
Fanconi's : PCT defect Bartter's : Thick Ascending loop of henle (affects Na+/K+/2Cl- triple transporter) Gitelman's : NaCl transporter reabsorption defect in DCT Liddle's : Gain in func. mutation (decreased Na+ channel degradation) --> increased Na+ reabsorption from Collecting duct SAME : Synd. of Apparent Minerelocorticoid Excess (Heriditary 11-Beta HSD deficiency ==> increase Cortisol ==> increase MC receptor activity)
113
AKI Dx. criteria ??
- Rise in S. Cr by >= 26 umol/L in 48hrs (OR) - a >= 50% rise in S. Cr within past 7 days (OR) - UO < 0.5 ml/kg/hr for >= 6hrs in adults & >= 8hrs in children (OR) - >= 25% fall in eGFR in children/ young adults in 7 days
114
What is Thin Basement Memb. disease ??
Inherited Type 4 collagen defect (thinning of BM) Persistent Haematuria + normal KFT without renal failure + Familial haematuria.
115
Acute Interstitial Nephritis features ??
Fever + Rash + Arthralgia EOSINIPHILIA Mild renal impairment + HTN U/R - Sterile pyuria & WBC casts
116
Drugs causing AIN ??
Penicillin Rifampicin NSAIDs Allopurinol Furosemide
117
AIN histology ??
marked Interstitial edema & infiltrates in connective tissue b/w renal tubules.
118
Types of Glomerulonephritis ??
NEPHROTIC (Proteinuria, oedema) -Minimal Change disease - Membranous GN - FSGS - Amyloidosis - Diabetic Nephropathy NEPHRITIC (Haematuria, HTN) - RPGN or Cresentric GN - IgA Nephropathy or Mesangio-proliferative GN) - Alport Synd. MIXED (Nephrotic + Nephritic) - Diffuse Proliferative GN - Membrano-proliferative GN or Mesangio-capillary GN - Post- Streptococcal GN
119
Disorders a/w GN & Low Complement ??
PSGN SABE SLE Mesangio-capillary GN or Memb.-Proliferative GN
120
What is FSGS ??
Is a cause of Nephrotic synd. & Chr. Kidney disease - YOUNG adults
121
Causes of FSGS ??
Idiopathic Secondary to Renal pathology - IgA Nephropathy - Reflux Nephropathy HIV Heroin Alport's & Sickle Cell D High recurrence rate in Renal transplants
122
Renal Biopsy features of FSGS ??
Light Microscopy: Focal & Segmental Sclerosis & Hyalinosis Electron M: Foot process effacement Rx. - Steroids +/- Immunosuppresants
123
What is HIVAN ??
HIV associated Nephropathy - May occur as a consequence of Rx. or the Virus itself Key Features are - Massive Proteinuria - Normal or Large Kidney - FSGS + Focal or Global Capillary collapse - Elevated Urea & Cr - Normotension ART improves the outcome
124
Difference b/w IgA Nephropathy & PSGN ??
IgA Nephropathy (Nephritic) - Develops 1- 2 days after URTI - Young males + Macroscopic Haematuria PSGN (Mixed Nephropthy) - Develops 1- 2 wks after URTI - Proteinuria>Haematuria - LOW Complement
125
PSGN cause ??
Grp. A Beta haemolytic Streptococci infection (Strept. pyogenes) - Caused by Immune Complex (IgG, IgM & C3) deposition in glomeruli - MC among Young Children Features - Proteinuria>Visible Haematuria - Oedema, Oliguria, HTN - Low C3 & Raised ASO titres
126
Renal Biopsy features of PSGN ??
Causes Acute, Diffuse P GN Endothelial proliferation with Neutrophils Electron M: SubEpithelial Humps due to lumpy immune complex deposition Immunoflorescence: Granular or 'Starry Sky' appearance
127
What is HSP ??
Henoch-Schonlein Purpura - IgA mediated Small vessel vasculitis - MC seen in Children after infection - Excellent prognosis without Renal involvement (in Children)
128
Features of HSP ??
- Palpable purpuric rash (with localized edema) over Buttocks & Extensor surfaces of arms & legs - Abd. Pain - Polyarthritis - Features of IgA nephropathy can occur (Haematuria, Renal failure)
129
Rx. of HSP ??
- Analgesia for pain - Rx of Nephropathy is SUPPORTIVE
130
What is HUS ??
Generally seen in Young Children Secondary HUS- Typical (most cases) - Shiga Toxin-Producing E Coli O157:H7 (Verotoxigenic, Enterohaemorrhagic) MCC in children in >90% - Pneumococcal infection - HIV - Rare: SLE, drugs, cancer Primary HUS- Atypical due to Complement dysregulation
131
Features & Ix. done in HUS ??
AKI + MAHA + Thrombocytopaenia FBC: Anaemia, thrombocytopaenia, fragmented blood films U&E: AKI Stool culture - Look for STEC O157:H7 infection - PCR for Shiga toxins
132
Rx. of HUS ??
SUPPORTIVE - Fluids, Blood T, Dialysis (if required) NO role of Antibiotics - Plasma exchange - Eculizumab (a C5 inhibitor monoclonal antibody) has greater efficacy than Plasma exchange in Rx. of Adult HUS
133
Indication of Plasma exchange in HUS ??
Severe HUS + NOT a/w Diarrhoea
134
What is Membranoproliferative GN ??
aka Mesangio-capillary GN: presents as Nephrotic, Haematuria/Proteinuria Type 1 (90% cases) - Cause: Cryoglobulinaemia, Hep.C Type 2 (Dense deposit disease) - Cause: Partial Lipodystrophy (loss of SubC tissue from face), Factor H deficiency Type 3 :- Causes: Hep. B & C Rx.- Steroids may be effective
135
Type 1 & Type 2 Electron microscopy features ??
Type 1: Subendothelium & Mesangium immune deposits of electron-dense material resulting in 'Tram-Track' appearance Type 2: Intramembranous Immune Complex deposits with 'Dense deposits'
136
Features of Type 2 Memb.-Proliferative GN ??
Due to Persistent activation of Alternative Complement pathway - Low circulating C3 C3b nephritic factor found in 70% - Antibody to Alternative-pathway C3 convertase (C3bBb) - Stabilizes C3 convertase
137
What is Membranous GN ??
MC type of GN in adults & is the 3rd MCC of ESRF; Nephrotic picture Causes - Idiopathic (Anti-phospholipase A2 antibodies) - Infections: Hep.B, Malaria, Syphilis - Malignancy: Prostate, Lung, Lymphoma, Leukaemia - Drugs: Gold, Penicillamine, NSAIDs - SLE (Class V), Thyroiditis, RA
138
Renal biopsy features of Memb. GN ??
Electron M: BM is thickened with Sub-epithelial electron dense deposits - This creates 'Spike & Dome' appearance
139
Rx. of Memb. GN ??
All pts.- ACEi or ARBs Immunosuppresion - Only Severe or Progressive cases - Steroids alone are not effective; use a combination of Corticosteroids + another agent like (Cyclophosphamide) Anti-Coagulation in high risk pts.
140
Good prognostic factors of Memb. GN ??
- Female sex - Young age at presentation - Asymptomatic proteinuria of modest degree at the time of presentation
141
What is Minimal Change disease ??
NEPHROTIC picture (75% are Children & 25% are Adults) Causes - Drugs: NSAIDs, Rifampicin - Hodgkin's lymphoma, Thymoma - INFECTIOUS MONONUCLEOSIS
142
Pathophysiology of Minimal Change disease ??
T-cell & Cytokine mediated damage of GBM => Polyanion loss => reduced Electrostatic charge => Increased permeability of GBM (Highly selective Proteinuria)
143
Features of MCD ??
Nephrotic syndrome + Normotensive Only Intermediate-sized proteins such as ALBUMIN & TRANSFERRIN leak through GBM Rx.- - Oral Corticosteroids (80% are steroid responsive) - Cyclophosphamide: if Steroid resistant case
144
Renal Biopsy features of MCD ??
Light M: Normal glomeruli Electron M: Fusion of podocytes & Foot processes effacement
145
What is RPGN ??
Rapid loss of renal function a/w the formation of Epithelial Crescents in the majority of glomeruli Causes- - Goodpasture's syndrome - Wegener's granulomatosis - SLE, Microscopic Polyarteritis
146
Features of Crescentric GN ??
RPGN - Nephritic: Haematuria with Red cell casts, proteinuria, oliguria - Features of the underlying cause
147
MC form of Renal disease in SLE ??
Diffuse Proliferative G N
148
What is Calciphylaxis ??
Complication of End-stage Renal F - Results in deposition of Ca2+ within the arterioles => Microvascular occlusion & Necrosis of supplied tissue - PAINFUL, Necrotic skin lesions
149
RF and Rx. of Calciphylaxis ??
- Hyper Ca2+, Hyper PO4, Hyperparathyroidism - Warfarin Rx.- - Reducing Ca2+ & PO4 levels - Controlling Hyperparathyroidism - Avoid contributory drugs
150
When does EPO therapy fails ??
Iron deficiency anaemia Inadequate dose Concurrent infection/ Inflammation Hyperparathyroid Bone disease Aluminium toxicity
151
Major S/E of EPO ??
- Pure Red cell Aplasia (due to anti-bodies against EPO) DARBEPOIETIN has reduced risk - Iron deficiency secondary to Increased Erythropoiesis
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Which factors are lost in Nephrotic syndrome ??
- Anti-Thrombin 3, Protein C & S + Rise in Fibrinogen levels (predispose to Thrombosis) - Thyroxine-binding Globulin is lost ==> Lowers TOTAL but not Free Thyroxine levels
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Complications of Nephrotic Synd. ??
Thromboembolism (Loss of Anti-thrombin 3 & Plasminogen in urine) - DVT, PE - Renal Vein T => Sudden deterioration in RFT HYPERLIPIDAEMIA (increased risk of ACS & Stroke) Increased risk of infection (IGs loss via urine) HYPOCALCAEMIA (Vit D & Binding protein lost in urine)
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Triad of Nephrotic Synd. ??
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What is Contrast Media induced Nephrotoxicity ??
25% increase in Cr occurring in < 3 days of IV Contrast media administration Risk Factors - Known Renal impairment - Age > 70 yrs - Dehydration - Cardiac failure - Use of Nephrotoxic drugs (NSAIDs)
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How to prevent Contrast induced Nephrotoxicity ??
- IV 0.9% NaCl at 1ml/kg/hr for 12 hrs Pre- & Post procedure - High-risk pts.- STOP Metformin for 48hrs & until U&E is shown to be normal
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What is Renal Papillary Necrosis ??
COAGULATIVE Necrosis of Renal Papillae Causes - Severe Acute Pyelonephritis - Diabetic Nephropathy - Obstructive Nephropathy - Analgesic Nephropathy (Phenacitin, NSAIDs) - TB. - Sickle Cell Anaemia
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Features of Renal Papillary Necrosis ??
Fever + Haematuria & Proteinuria + Loin pain IVU: Papillary Necrosis with Renal Scarring- 'Cup & Spill'
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Common causes of Polyuria ??
Common (> 1 in 10) - Diuretics, Caffeine, Alcohol, Lithium - DM, Heart Failure Infrequent (1 in 100) - Hyper Ca2+ & Hyperthyroidism Rare (1 in 1000) - Chr. Renal Failure - Primary Polydipsia ; HYPO K+ Very Rare (<1 in 10,000) - Diabetes insipidus
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What is Diabetes Insipidus ??
Cranial DI: Decreased ADH secretion from Pituitary Nephrogenic DI: ADH insensitivity Features - Polyuria - Polydipsia
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Causes of Cranial & Nephrogenic DI ??
CRANIAl DI - Idiopathic, Post Head injury, Pituitary Sx., [DI DM OA D- Wolfram] - Infiltrative (Histiocytosis X & Sarcoidosis) NEPHROGENIC DI - Genetic (MC form): Aquaporin 2 ADH channel mutation - Hyper Ca2+ & Hypo K+ - Lithium & Democlocycline -Tubulo-interstitial disease: Obstruction, Sickle-CD, Pyelonephritis
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Ix. & Rx. of Diabetes Insipidus ??
- HIGH Plasma & LOW Urine Osml - Urine Osml of > 700mOsm/kg excludes DI - Water Deprivation Test Rx. - NDI: Thiazides, Low salt/ Protein diet - CDI: Desmopressin
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What are the stages of Diabetic Nephropathy ??
1 : Hyperfiltration- GFR increased (may be reversible 2 : Silent/ Latent- GFR remains elevated + No Microalbuminuria for 10 years 3 : Incipient- Microalbuminuria ( 30 - 300 mg/day) + Dipstick (-)ve 4 : Overt- Persistent proteinuria (>300 mg/day) + Dipstick (+)ve; HTN present + Diffuse GS & Focal GS (Kimmelstiel-Wilson Nodules) 5 : ESRD + GFR < 10 ml/min ; Renal Replacement therapy needed
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WHO classification of Lupus Nephritis ??
Class 1: Normal Kidney C 2 : Mesangial GN C 3 : Focal Segmental Proliferative GN C 4 : DIFFUSE PROLIFERATIVE GN C 5 : Diffuse Membrabous GN C 6 : Sclerosing GN
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What is Class 4 Lupus Nephritis ??
Diffuse Proliferative GN (MC & Most SEVERE form) - Glomeruli: Endothelial & Mesangial proliferation, 'Wire-Loop' appearance - Capillary wall thickened (immune complex deposition) - Electron M: Sub-Endothelial Immune Complex deposits - Immunofluorescence: Granular
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Rx. of Lupus Nephritis ??
Treat HTN Class 3 (Focal) or Class 4 (Diffuse) - Glucocorticoids + Mycophenolate/ Cyclophosphamide MMF preferred over Azathioprine
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What is Fanconi's Syndrome ??
Generalised reabsorptive disorder of renal tubule transport in PCT resulting in - Type 2 RTA (Proximal) - Polyuria - Aminoaciduria - Glycosuria, Phosphaturia - Osteomalacia
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Causes & MCC in Children of Fanconi's synd. ??
Sjogren's syndrome Multiple Myeloma Nephrotic Synd. Wilson's disease MCC in Children: CYSTINOSIS
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What is Alport's Syndrome ??
- 85% cases: X-linked D & rest as A R - Type 4 Collagen defect => Abnormal GBM - More severe in MALES
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Features of Alport's syndrome ??
Usually presents in Childhood - Microscopic Haemturia - Progressive Renal Failure - B/L SNHL ; - Retinitis Pigmentosa - Lenticonus: Lens surface protrusion to Anterior chamber
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Dx. of Alport's syndrome ??
Molecular Genetic testing Renal Biopsy - Electron M: Longitudinal SPLITTING of Lamina densa of GBM resulting in 'Basket weave' appearance
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Typical Alport's syndrome presentation ??
Failing renal transplant (may be caused by the presence of Anti-GBM antibodies leading to Goodpasture's syndrome like picture)
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What is Amyloidosis ??
Extracellular deposition of an Insoluble Fibrillar protein termed Amyloid [Fibrillary + Non-fibrillary components] - Non-fibrillary component: Amyloid-P, Apolipoprotein-E & Heparan sulfate proteoglycan
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Types of Amyloidosis ??
AL amyloidosis (MC form) - L is IG light chain fragment - Due to Myeloma, Waldenstrom's, MGUS - Features: Nephrotic, CVS & CNS involved, Macroglossia, Periorbital eccymoses AA amyloidosis - A: precursor serum amyloid A protein, an acute phase reactant - Seen in Chronic infection/inflam. - eg.- TB, Bronchiectasis, RA - Features: Renal involvement MC Beta-2 Microglobulin amyloidosis - Beta-2 microglobulin is the precursor protein (part of MHC)
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Which type of amyloidosis is a/w - Pts. on Renal dialysis ?? - TB, Bronchiectasis, RA ?? - Myeloma, Waldenstrom's, MGUS ??
- Beta-2 microglobulin amyloidosis - AA amyloidosis - AL amyloidosis
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Dx. of Amyloidosis ??
Congo-red staining: Apple-green birefringence Serum Amyloid precursor scan Biopsy: Skin, Rectal mucosa or Abd. fat
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Which is the most common inherited cause of Kidney disease ??
ADPKD (1 in 1000 Caucasians) 2 types - Type 1 (85% cases); Chr 16; presents with early Renal failure - Type 2 (15% cases) Chr. 4 2 disease loci have been identified, PKD1 & PKD2, which codes for Polycystin-1 & Polycystin-2 respectively
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Ix. done in ADPKD ??
Screening Ix for relatives is USS abd. USS Dx. criteria in pts. with (+)ve FHx - 2 cysts, U/L or B/L if aged < 30yrs - 2 cysts in B/L Kidneys; aged 30 to 59 yrs - 4 cysts in B/L Kidneys; aged > 60yrs
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Rx. of ADPKD ??
TOLVAPTAN (vasopressin receptor 2 antagonist) in adults to slow the progression of cyst devt. & renal insufficiency ONLY if: - CKD stage 2 or 3 at the start of Rx - Evidence of Rapid Progressive disease - The company provides it with discount agreed in pt.'s scheme
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What are the Renal & Extra-renal features of ADPKD ??
- HTN, Recurrent UTIs, Haematuria - Renal Stones, Abd. Pain, CKD Extra-Renal manifestations - Liver cysts: can cause Hepatomegaly - BERRY Aneurysm: causes SAH - CVS: MVP, Mitral/ Tricuspid incompetence, Aortic Root dilation, Aortic dissection - Cysts in other organs: Pancreas, Spleen; very rarely- Thyroid, Oesophagus, Ovary
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MC Extra-renal manifestation of ADPKD ??
Liver Cysts
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Features of ARPKD ??
Gene defect located on Chr. 6 which encodes for FIBROCYSTIN- protein imp. for normal renal tubule devt. Dx. - Prenatal USS or Early Infancy with Abd. mass & Renal failure - Newborns: Potter's syndrome secondary to Oligohydramnios - ESRD in childhood -Liver: Portal or Interlobular fibrosis Renal Biopsy
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Renal Biopsy features of ARPKD ??
Multiple Cylindrical lesions at Rt. angles to Cortical Surface.
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Causes of AKI ??
PRE-RENAL (Ischaemia or Lack of Blood flow to Kidneys) - Hypovolaemia (diarrhoea/vomitting) - Renal artery Stenosis INTRINSIC Renal - Glomerulonephritis - ATN. - AIN. - Rhabdomyolysis - Tumour Lysis Syndrome POSR-RENAL (Obstruction) - Renal stones in Ureter/ Bladder - BPH. - External Ureter compression
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C/F of AKI ??
Early AKI can be asymptomatic - Reduced Urine Output - Pulm. or Peripheral Oedema - Arrhythmias (due to changes in K+ & Acid-base balance) - Uraemia features (eg.- Pericarditis or Encephalopathy)
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Dx. of AKI ??
(p)RIFLE, AKIN or KDIGO criterias - Rise in Cr. of >= 26 umol/L in < 48hrs - Rise in Cr >= 50% known/ presumed to have occurred in past 7 days - Fall in UO to < 0.5ml/kg/hr for >6 hrs in Adults & > 8hrs in Children - Fall of >= 25% in eGFR in Children/ Young adults in 7 days URINALYSIS IMAGING - No identifiable cause or at risk of Urine tract obstruction; Renal USS within 24hrs
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KIDGO staging of AKI ??
STAGE 1 - Rise in Cr. to 1.5- 1.9 times baseline/ - Rise in Cr. by >= 26.5 umol/l or - Fall in UO < 0.5ml/kg/hr for >=6hrs STAGE 2 - Rise in Cr. to 2.0 to 2.9x baseline - Fall in UO < 0.5ml/kg/hr for >=12hrs STAGE 3 - Rise in Cr. to >= 3.0x baseline or - Rise in Cr. of >= 353.6 umol/l or - Fall in UO <=0.3ml/kg/hr for >=24hrs - Initiation of RRT or - In pts. <18 yrs fall in eGFR to <35ml/min/1.73 m2
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When is a AKI pt. referred to a Nephrologist ??
- Renal transplant - ITU pt. + AKI (Unknown cause) - Vasculitis/ GN/ TubuloInterstitial Nephritis/ Myeloma - Inadequate response to Rx. - AKI complications - Stage 3 AKI - CKD Stage 4 or 5 - Qualify for Renal Replacement Hyper K+/ Met. Acidosis/ Uraemia complications/ Fluid overload
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Risk factors of AKI ??
- Emergency Sx (ie. risk of Sepsis or Hypovolemia) - Intraperitoneal Sx. - CKD (if eGFR < 60) - DM, - Heart Failure, - > 65yrs old - Liver diseases - Nephrotoxic drugs (NSAIDs, Aminoglycosides, ACEi/ ARBs, Diuretics
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What is Pre-renal Uraemia ??
Kidneys hold on to Na+ to preserve volume - Urine Na < 20 mmol/l - Urine Osml > 500 mOsm/kg - Normal/Bland sediment urine In ATN - Urine Na > 40 mmol/l - Urine Osml < 350 mOsm/kg - Brown Granular casts in urine
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What is Fractional Na+ Excretion ?? What is Fractional Urea Excretion ??
- (Urine Na/ Plasma Na)/ (Urine Cr./Plasma Cr) * 100 - (Urine urea/Blood urea)/ (Urine Cr./Plasma Cr.) * 100
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Rx. NOT recommended in AKI ??
- Loop diuretics - Low dose Dopamine (to increase renal perfusion) - Loop diuretics can only be used in Fluid overload cases
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CKD classification based on eGFR ??
STAGE 1: > 90ml/min + some signs of Kidney damage on other tests (if all kidney tests normal, NO CKD) STAGE 2: 60- 90ml/min + (same as stage1) normal U&E & No proteinuria STAGE 3a: 45- 59ml/min + Moderate reduction in Kidney function STAGE 3b: 30- 44ml/min + same as 3a STAGE 4: 15- 29ml/min + Severe reduction in Kidney function STAGE 5: < 15ml/min/ Established RF on dialysis/ Kidney transplant needed
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What factors are considered to measure eGFR ??
S. Cr. - Age. - Gender. - Ethnicity Factors that can affect are - Pregnancy - Muscle mass (eg. Amputees, Body builders) - Eating RED Meat 12hrs prior to sample being takes
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Common causes of CKD ??
- Diabetic Nephropathy - Chronic Glomerulonephritis - Chronic Pyelonephritis - HTN - Adult Polycystic Kidney disease
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When does Anaemia becomes apparent in CKD pts. ??
- GFR < 35 ml/min or - GFR > 60ml/min + Other causes of anaemia - Anaemia in CKD predisposes pts. to develop LVH
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Rx. of Anaemia in CKD ??
Target Hb of 10 to 12 g/dl - Iron status should be optimised BEFORE administering Erythropoietin Stimulating Agents (ESA) - Many pts. on Haemodialysis will require IV Iron - ESAs like EPO & Darbepoietin used
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Causes of Anaemia in CKD ??
- Reduced EPO levels (MCC) - Reduced E-poiesis due to toxic effects of URAEMIA on bone marrow - Reduced Iron absorption - Anorexia/ Nausea due to Uraemia - Reduced RBCs survival (due to dialysis) - Blood loss due to Capillary fragility & Poor platelet function - Stress ulceration ==> Chr. Blood loss
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Ca2+ physiology seen in CKD ??
- Low Vit D (1-alpha hydroxylation normally occurs in Kidney) - High PO4 - Low Ca2+ (due to the above 2) - Secondary Hyperparathyroidism (due to the above 3 reasons)
200
Bone diseases seen in CKD ??
- Osteitis Fibrosa Cystica or Hyper-parathyroid bone disease - Adynamic Bone disease - Osteomalacia (due to low Vit D & high PO4 drags Ca2+ from bones) - Osteosclerosis - Osteoporosis
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Bone disease seen with Vit D over Rx in CKD pts. ??
Adynamic bone disease
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Rx. of Mineral Bone disease in CKD ??
Aim: Reduce PO4 & PTH levels - 1st line: Reduce PO4 diet intake - Phosphate binders - Vit D: Alfacalcidol & Calcitriol - Parathyroidectomy in some cases
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Types of PO4 binders ??
Aluminium based (not used) Ca2+ based Binders - Problem: Hyper Ca2+ & Vascular calcification SEVELAMER (MC used now) - Non-Ca2+ based binders - Binds to dietary PO4+ & prevents its absorption - Other beneficial effects: Reduces Uric acid, improves Lipid profile
204
When is Furosemide used as an Anti-Hypertensive in CKD pts. ??
GFR falls to < 45 ml/min & it has a added benefit of lowering S. K+ levels
205
What is Peritoneal Dialysis ??
Form of RRT; Indications - Stop-gap to HD or - Younger pts. who do not want to visit hospital 3x a week
206
Complications of Peritoneal Dialysis ??
PERITONITIS -MCC: Coagulase (-)ve Staph. eg- Staph. Epidermidis >Staph. aureus Rx.- - Vancomycin (or Teicoplanin) + Ceftazidime added to dialysis fluid OR - Vancomycin added to dialysis fluid + Ciprofloxacin by mouth - Aminoglycosides are sometimes used to cover the Gram (-)ve organism instead of Ceftazidime SCLEROSING Peritonitis
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MC form of Peritoneal Dialysis ??
Continuous Ambulatory P D (CAPD) - Involves four 2-lt. exchange/ day
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Approx. Equivalent values of Proteinuria ??
ACR (mg/mmol) : PCR (mg/mmol) : Urinary Protein excretion (g/24hr) = = 30 : 50 : 0.5 g/24hrs = 70 : 100 : 1 g/24 hrs ACR is preferred over PCR as it has greater sensitivity
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How to collect an ACR sample ??
- First-pass morning urine specimen - If initial ACR is b/w 3 to 70 mg/mmol 2nd subsequent early morning sample should be taken for confirmation - If initial ACR >70 mmol/l, repeat sample NOT required
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Clinically significant Proteinuria ??
ACR of >= 3 mg/mmol When to Refer to a Nephrologist - ACR >= 70 mg/mmol unless known to be caused by DM & already appropriately treated - ACR >= 30 mg/mmol + Haematuria (2/3 dipstick shows >= 1+ blood) + UTI excluded - ACR: 3- 29 mg/mmol + Haematuria + RFs (declining eGFR or CVS diseases)
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Rx. of Proteinuria ??
Start ACEi or ARBs - 1st line: HTN & CKD + ACR >30 mg/mmol - ACR >70 mg/mmol regardless of BP
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