Complete Nephrology Revision - c25f1267 7e5b 4cbf b13f 699a5696711a

Dr Shubham Upadhyay
© Medi - Lectures Dr Shubham Upadhyay 1

OVERVIEW
• Azotemia & Urinary Abnormalities
• Fluid & Electrolyte Imbalance
• Acid Base Disturbances
• Acute Kidney Injury
• Chronic Kidney Disease
• Renal Replacement Therapy
• Dialysis
• Renal Transplantation
• Glomerular Diseases
• Vascular Diseases of Kidney
• Genetic cystic diseases
• Tubulointerstitial Diseases
• Renal Calculi
1. Which of the following is the first observation in investigations that
helps in arriving at the cause of azotemia?
A. Renal Size
B. Hydronephrosis
C. Cortical thickness
D. Urinary sediment

AZOTEMIA vs UREMIA
• Azotemia - Elevation of BUN & Creatinine values
• Uremia - Azotemia + Clinical Features + Biochemical Abnormalities
• Causes of Azotemia
Chronic Renal Failure Acute Renal Failure
• Usually asso. with Anemia, • Pre Renal- Reduced Renal
Hypocalcemia, Perfusion
Hyperphosphatemia • Intrinsic Renal Disease -
• USG- Increased Echogenecity Damage to Renal
& cortical thinning Parenchyma
• Proteinuria, Isosthenuria • Post Renal- Urinary Tract
© Medi - Lectures Obstruction
Dr Shubham Upadhyay 4
2. Urinary protein excretion of >3.5 grams/day can occur in?
A. Diabetes
B. Amyloidosis
C. Minimal Change Disease
D. All of the above

PROTEINURIA
• Dipstick examination ------> Quantification
• Dipstick Examination
üDetects only Albumin
üFalse Positive Results
üFalse Negative Results
• Albumin Quantification
üSpot Albumin : Creatinine Ratio (First Morning Sample)
• Total Protein Quantification
üSpot Protein : Creatinine Ratio (First Morning Sample)
ü24 Hr Urinary Protein

PROTEINURIA - TYPES
1. Glomerular Proteinuria 3. Overflow Proteinuria
üGlomerular Structure & Function üExceeding Tubular Reabsorption
üMain Protein - Albumin Capacity
üNephritic vs Nephrotic üCauses:
üRBC casts a. Multiple Myeloma
b. Amyloidosis
2. Tubular Proteinuria c. Lymphoma
üMain Protein- Tamm Horsfall
Protein
4. Orthostatic Proteinuria
üMechanism
ü<2 gm/day
üTotal <1 gm/day

3. Mr. Ramprasad is a 61-year-old man who presents to his primary care physician for a
routine physical examination. He says he has been feeling well except for some
intermittent generalized achiness. His only medications are lisinopril and atorvastatin.
He has no abnormalities on his physical examination. His urine dipstick is positive for
hematuria, without proteinuria. On urinalysis, there are no red blood cells seen on urine
microscopy, with no proteinuria or pyuria. Which of the following most likely explains
the discrepancy between the negative urine microscopy and the positive dipstick result?
A. Hypercalciuria is the principal contributor.

B. It was most likely a false-negative urine microscopy
C. Statin-induced rhabdomyolysis causing myoglobinuria is present
D. The patient likely has a urinary tract infection.

HEMATURIA
• Hematuria?
• Dipstick Test?
• Single Urinalysis with Hematuria?
• Persistent or significant Hematuria?
• Isolated Hematuria?
• Isolated Microscopic Hematuria?
• Hematuria with pyuria & Bacteriuria?
• Recurrent/ Episodic Hematuria?
• Renal Biopsy
4. What percentage of total body fluid is found as Interstitial fluid?
A. 5-15%
B. 25-35%
C. 55-75%
D. 45-55%

5. Pseudohyponatremia is defined as the coexistence of
A. Hyponatremia with normal plasma tonicity

B. Hyponatremia with decreased plasma tonicity
C. Normonatremia with increased plasma tonicity
D. Hyponatremia with decreased plasma tonicity

HYPERTONIC ISOTONIC HYPONATREMIA HYPOTONIC
HYPONATREMIA HYPONATREMIA
Posm > 290 mOsmol/kg H2O Posm 275-290 mOsmol/kg H2O Posm < 275 mOsmol/kg
•Results due to presence of • Pseudohyponatremia= laboratory artifact H2O
another effective osmole • Due to marked increase in serum lipids or •True physiologic
that causes free water to proteins hyponatremia that results
move from the ICF to the ECF • Results in reduction of fraction of serum that from excess water
is water and an artificially low serum Na+ eitherdue
• Examples: • Plasma water Na+ concentration and to ADH stimulation or
ü Mannitol (osmotic osmolality are unchanged but the measured impaired water excretion
diuretic), Na+ concentration in the total plasma volume
glycine, marked will be reduced given the specimen contains
hyperglycemia less plasma water
• Only occurs when flame photometry or
• Treatment correct the indirect potentiometry are used
underlying condition or • Direct potentiometry and ion-specific
removal electrodes avoids this misdiagnosis
of the offending agent
ISOTONIC HYPONATREMIA

6. Which of the following are modalities to correct plasma Na+
concentration in patients with SIADH?
A. Oral frusemide
B. Demeclocycline
C. Oral urea
D. All of the above

SIADH
• MCC of Euvolemic hyponatremia
• Generation of hyponatremia requires intake of free water
• Four distinct patterns of AVP secretion
I. Unregulated, erratic AVP secretion
II. Failure to suppress AVP at lower serum osmolalities
III. Reset Osmostat
IV. No detectable circulating AVP
• Subclinically Volume expanded
• s Uric Acid <4 mg/dl

Causes of SIADH

TREATMENT
• Combined Oral Furosemide & Oral salt tablets
• Demeclocycline
• Oral Urea
• AVP antagonists (Vaptans)

• V2 receptor antagonists
• Tolvaptan
• Conivaptan
7. After administering "Desmopressin", urine osmolality should
increase by how much in Nephrogenic Diabetes Insipidus?
A. Atleast 10%
B. Atleast 20%
C. Atleast 50%
D. None of these

8. Mr. Jagdish is a 54-year-old man with nephrogenic diabetes insipidus (NDI) from
lithium therapy. Usually, he is excellent about keeping up his free water intake to
control his sodium level. However, he was involved in a car accident requiring an
operation at an outside hospital. There, they did not know about his NDI, and after
being NPO for 48 hours, he was found to have a serum sodium of 160 mEq/dL. He
is a 100-kg man. To correct his serum sodium over the next 24 hours, at what
approximate rate should the physician run IV 5% dextrose in water (for free water)?
A. 150 ml/hr
B. 250 ml/hr
C. 350 ml/hr
D. 450 ml/hr

• This patient has severe hypernatremia after being denied free water. In the
state of nephrogenic diabetes insipidus, the kidneys fail to respond to ADH
and excrete dilute urine regardless of serum osmolality.
• To correct this hyponatremia, you must first calculate the patient’s total free
water deficit.
• This is calculated as ([Na] – 140/140) × (total body water) where total body
water is roughly 50–60% of body weight (60% in men, or 60 kg for Mr. Jagdish).
• Thus, Mr. Jagdish’s free water deficit is ~8.5 L (or 8500 mL). To replace this in
24 hours requires approximately 350 mL/h of free water administration.

9. Which of the following ECG changes denote severe Hyperkalemia?
A. Tall peaked T waves

B. Loss of P waves
C. Prolonged QT interval
D. Prolonged QU interval

HYPERKALEMIA
• Sinus bradycardia, sinus arrest, Tall peaked T waves
slow idioventricular rhythms, Loss of P waves
ventricular tachycardia, Widened QRS pattern
ventricular fibrillation, and Sine wave pattern
asystole

9. Which of the following ECG changes denote severe Hyperkalemia?
A. Tall peaked T waves

B. Loss of P waves
C. Prolonged QT interval
D. Prolonged QU interval

10. Which of the following reduce plasma K+ levels by shifting K+ into
the cells?
A. Glucose insulin therapy

B. Alkali therapy with NaHCO3
C. Beta 2 adrenergic agonists
D. All of the above

TREATMENT OF HYPERKALEMIA
1. Immediate antagonism of the cardiac effects of hyperkalemia
• iv Calcium
2. Rapid reduction in plasma K+ concentration by redistribution into
cells
• iv Insulin
• β2 -Agonists
• iv Bicarbonate
3. Removal of potassium.
• Cation exchange resins
• Diuretics
• Dialysis
11. Which of the following is an alternative to bisphosphonates in
Hypercalcemia?
A. Glucocorticoids
B. Gallium nitrate
C. IV phosphate
D. Ketoconazole

TREATMENT OF HYPERCALCEMIA
• Mild, asymptomatic Hypercalcemia
• Significant, symptomatic Hypercalcemia
• Volume Expansion
• Loop Diuretics
• Bisphosphonates
• Gallium Nitrate
• Denosumab
• Hemodialysis
• Glucocorticoids
12. Which of the following indicate metabolic acidosis - metabolic
acidosis?
A. Na 140, K 4.0, Cl 106, HCo3 14, AG 20, PaCO2 24, pH 7.39

B. Na 140, K 3.0, Cl 95, HCo3 25, AG 20, PaCO2 40, pH 7.42
C. Na 135, K 3.0, Cl 110, HCo3 10, AG 15, PaCO2 25, pH 7.20
D. Na 140, K 3.5, Cl 88, HCo3 42, AG 10, PaCO2 67, pH 7.42

13. Urine anion gap (UAG) is calculated by the formula?
A. {Na + K}u / {Cl}u

B. {Na + K}u x {Cl}u
C. {Na + K}u - {Cl}u
D. {Na + K}u + {Cl}u

14. All of the following scenarios fit under the criteria for diagnosis of
AKI except?
A. Rise in s. creatinine from baseline 0.5 mg/dL within 48 hrs

B. 50% higher serum creatinine than baseline within 1 week
C. Reduction in urine output to <0.5 mL/kg/hr for >4 hrs
D. Anuria for >8 hrs

DEFINITION OF AKI:
• Rise in s. creatinine from baseline 0.3 mg/dL within 48 hrs
OR
• 50% higher serum creatinine than baseline within 1 week
OR
• Reduction in urine output to <0.5 mL/kg/hr for >6 hrs

15. Mr. Ramesh is a 47-year-old long-haul truck driver with a history of mild chronic kidney
disease (baseline creatinine 1.4 mg/dL) and hypertension. Recently, he was complaining of
some shoulder pain exacerbated by his work. Further, at his primary care appointment, his
blood pressure was elevatedat 150/95. He was started on lisinopril 20 mg daily and advised
to take naproxen 500 mg bid. Two weeks later, he presents to the emergency department
with muscle cramps and is found to have a creatinine of 4.9 mg/dL. His blood pressure is
135/80. Renal ultrasound reveals no hydronephrosis and normal-appearing kidneys. His
urinalysis is bland, and urine microscopy reveals hyaline casts. Urinary sodium is
undetectable. Which of the following is the likely cause of his acute renal failure?
A. Acute Interstitial Nephritis

B. Acute tubular necrosis
C. Glomerulonephritis
D. Glomerular Vasomotor dysfunction

16. Which of the following is the first line of defense against
fluctuations in renal blood flow in autoregulation of glomerular
filtration?
A. Myogenic reflex in afferent arteriole

B. Tubuloglomerular feedback
C. Angiotensin II mediated vasoconstriction of efferent arteriole
D. None of the above

17. All of the following except is a cause of prerenal AKI?
A. Cyclosporine
B. Congestive Heart Failure
C. Amphotericin B
D. Liver Failure

18. A 33-year-old woman with recently treated acute myelogenous
leukemia now in remission is admitted to the hospital with lethargy, fever,
and tachycardia. Blood cultures grow Pseudomonas that is resistant to
cefepime. She is started on IV gentamicin. Five days after starting
gentamicin, her serum creatinine rises from her baseline of 1.0 mg/dL to
2.4 mg/dL. No red or white cell casts are seen on her urinalysis. Her
magnesium level is decreased at 1.5 mg/dL. Renal ultrasound is
unremarkable with no hydronephrosis. Which of the following is the most
likely mechanism of her acute kidney injury?
A. Acute interstitial nephritis

B. Acute Tubular necrosis
C. Glomerulonephritis
D. Ischemic Injury © Medi - Lectures Dr Shubham Upadhyay 42
19. Which of the following is a false statement regarding laboratory
findings in Acute Renal Failure?
A. BUN/Plasma Creatinine ratio is 10:1 in Acute Tubular Necrosis

B. Urine osmolality is >500 mosm/L in prerenal azotemia
C. Fractional excretion of Sodium is >2% in prerenal azotemia
D. Muddy brown casts are commonly seen with acute tubular necrosis

20. Intraabdominal pressure that causes abdominal compartment
syndrome is?
A. >20 mm Hg
B. >30 mm Hg
C. >40 mm Hg
D. >50 mm Hg

BURNS & PANCREATITIS

21. Normal GFR of a 60 yr old person after reducing the annual decline
in GFR should be?
A. 70 ml/min/1.73 m2
B. 80 ml/min/1.73 m2
C. 90 ml/min/1.73 m2
D. 60 ml/min/1.73 m2

• The normal annual mean decline in GFR with age from the peak GFR
(~120 mL/min per 1.73 m2 ) attained during the third decade of life is
~1 mL/min per year per 1.73 m2 , reaching a mean value of 70
mL/min per 1.73 m2 at age 70, with considerable interindividual
variability

22. In stage IV chronic kidney disease, glomerular filtration rate is
below which of the following levels?
A. 90 mL/min/1.73 m2
B. 60 mL/min/1.73 m2
C. 30 mL/min/1.73 m2
D. 45 mL/min/1.73 m2

DEFINITION
• GFR <60 ml/min/1.73 m2 • Albuminuria >30 mg/day
• Urine sediments
• Fluid/Electrolyte imbalance due
to tubular dysfunction
• Abnormal kidneys on USG
• Abnormal kidney biopsy
• Post renal transplant

23. Use of Erythropoeitic Stimulating Agents (ESA) in CKD may be
associated with?
A. Increased risk of stroke in patients with Type 2 diabetes

B. Increase in thromboembolic events
C. Faster progression of renal decline
D. All of the above

ANEMIA in CKD
• Mechanism • Treatment
• MC- ↓ EPO • DOC- Erythropoetin
• Deficiency - Folic Acid/Fe/B12
• Hemolysis
• Bledding Diathesis
• ↓ RBC survival
• Hemodialysis
• ↑ PTH

24. Clear indications of starting dialysis in CKD include all of the
following except?
A. Anorexia & nausea not relieved by protein restriction

B. Bleeding Diathesis
C. Pericarditis
D. Volume overload

INDICATIONS
• Uremic Symptoms
• Hyperkalemia unresponsive to conservative measures
• Persistent extravascular volume expansion despite diuretic therapy
• Acidosis refractory to medical therapy
• Bleeding diasthesis
• Creatinine Clearance or eGFR <10 ml/min per 1.72 m2

SYMPTOMS OF UREMIA SIGNS OF UREMIA
• Nausea • Sallow discoloration of the skin
• Vomiting • Ammonia odor to the breath
• Anorexia • Pericardial friction rub/pericardial
• Dysgeusia (abnormal taste) effusion
• Pruritus • Myoclonus/seizure
• Alterations is sleep (insomnia and • Foot or wrist drop/uremic motor
daytime hypersomnolence) neuropathy
• Sensation of being cold • Prolonged bleeding time/platelet
• Cognitive changes dysfunction

25. All of the following except is an oral phosphate binder?
A. Calcium carbonate
B. Calcium acetate
C. Lanthanum
D. None of the above

26. If bleeding time is increased at the time of renal biopsy, which of
the following is administered immediately prior to the procedure?
A. Protamine
B. Desmopressin
C. N Acetylcysteine
D. IV calcium

27. Which of the following is the most common acute complication of
hemodialysis?
A. Anaphylactoid reactions to dialyzer

B. Bleeding from access site
C. Hypotension
D. Muscle cramps

COMPLICATIONS DURING HEMODIALYSIS
ACUTE CHRONIC
• Hypotension • Anemia
• Infection • Adynamic Bone Disease
• Muscle Cramps • Aluminium Induced dementia
• Anaphylactoid Reaactions • Amyloidoses
• Dialysis Dysequilibrium Syndrome

28. Prosthetic material for AV graft is?
A. Polytetrafluoroethylene (PTFE)
B. Polyethylene (PE)
C. Polyvinylidene fluoride (PVDF)
D. Polytrifluoroethylene

HEMODIALYSIS VASULAR ACCESS
• Arteriovenous Fistula
• Arteriovenous Graft
• Vascular Catheter

29. Most common culprit organism in peritonitis as a complication of
peritoneal dialysis is?
A. Gram positive cocci

B. Gram positive bacilli
C. Gram negative cocci
D. Gram negative bacilli

COMPLICATIONS DURING PERITONEAL DIALYSIS
• Peritonitis
• Catheter associated non peritonitis infections
• Weight Gain
• Hyperglycemia
• Metabolic Disturbances
• Hernia © Medi - Lectures Dr Shubham Upadhyay 64

30. Kidneys from which of the following categories is least preferred for
transplantation?
A. Brought in dead
B. Unsuccessful resuscitation
C. Cardiac arrest in a hospital
D. Cardiac arrest after brainstem death

31. All of the following agents are used for maintenance therapy after
renal transplantation except?
A. Cyclosporine
B. Alemtuzumab
C. Everolimus
D. Belatacept

IMMUNOSUPPRESION AFTER RENAL TRANSPLANT
• Induction Therapy
• Depleting Agents
• Non Depleting Agents
• Maintenance

32. A 21-year-old man is diagnosed with poststreptococcal
glomerulonephritis. Which of the following is likely to be found in his
urine?
A. >3 g per 24-hour proteinuria with hematuria

B. Positive urine culture for Streptococcus
C. Sterile pyuria without proteinuria
D. Microscopic hematuria with leukocytes and 24-hour urinary
albumin of 227 mg

GLOMERULAR DISEASES
FEATURE NEPHRITIC SYNDROME NEPHROTIC SYNDROME
HALLMARK
PROTEINURIA
s. ALBUMIN
HYPERTENSION
CAST
NEPHRITIC SYNDROME
ANTIBODY IMMUNE COMPLEX PAUCI IMMUNE
MEDIATED
GRANULAR PATTERN
LINEAR DEPOSITS ON
GBM NO DEPOSITS
GOOD PASTEUR • WEGENER’S
SYNDROME GRANULOMATOSIS
• MICROSCOPIC
POLYANGITIS

IMMUNE COMPLEX MEDIATED (s. C3 ↓)
MANIFESTATION INVESTIGATION DIAGNOSIS TREATMENT
• CHILDREN (5-15 YRS) • ASO POST STREPTOCOCCAL • SELF LIMITING
• Anti DNAse GLOMERULONEPHRITIS • SUPPORTIVE
• PHARYNGITIS or (DIURETICS)
DERMATITIS with
BETA HEMOLYTIC • Anti ZYMOGEN • ANTIBIOTICS ?ROLE
STREPTOCOCCI
• STEROIDS- NO ROLE
HEMATURIA +
OLIGURIA

33. Which of the following is false about IgA nephropathy?
A. Female preponderance
B. Peak incidence in II & III decades of life
C. Recurrent episodes of macroscopic hematuria
D. Between episodes, urinalysis is normal

IgA Nephropathy
• MC Primary Glomerulopathy in world, Sporadic
• 2nd to 3rd decade, Males > Females
• Pathogenesis
• First Hit : Synthesis of poorly galactosylated IgA1 -> Ab formation against it ->
IC formation and deposition
• Second Hit : Viral or other Ag exposure, hereditary defects in alternate
complement pathway
• Presentation:
• Recurrent macroscopic hematuria (Syn pharyngitic hematuria), proteinuria,
persistent microscopic hematuria
• Rarely, ARF with rapid progression

• Biopsy
• Light
• Immunofluorescence : Mesangial IgA deposits
• Prognosis
• Usually, Benign disease
• MEST-C score
• Treatment
• ACE inhibitors
• Tonsillectomy
• Fish oil
• RPGN
• Steroids
• Cytotoxic agents
• Plasmapheresis

34. Which class of lupus nephritis is labelled as membranous nephritis?
A. 3
B. 4
C. 5
D. 6

35. Most common cause of nephrotic syndrome in elderly is?
A. Minimal Change Disease

B. Focal Segmental Glomerulsclerosis
C. Membranous Glomerulonephritis
D. Mesangioproliferative Glomerulonephritis

ETIOLOGY
MCD MEMBRANOUS GP. FSGS
PRIMARY
INFECTIONS HIV • LEPROSY • HIV
• SYPHILIS • HEPATITIS B
• CHRONIC HEPATITIS B/C • PARVO B19
• MALARIA
• SCHISTOSOMIASIS
• FILARIASIS
NEOPLASIA HODGKIN’S LYMPHOMA PARANEOPLASTIC SYNDROME NON-HODGKIN’S LYMPHOMA
DRUGS RIFAMPICIN, PENICILLAMINE, CAPTOPRIL, GOLD, LITHIUM, HEROINE, PAMIDRONATE,

INTERFERON, NSAID PROBENECID, NSAID, ANTI TNF NSAID
AGENTS
OTHERS • AUTOIMMUNE • RENAL MASS LOSS
-SURGERY
-REPEATED RENAL BIOPSY
• DIABETES MELLITUS -VUR
-CONGENITAL AGENESIS
• SICKLE CELL DISEASE
• SICKLE CELL DISEASE • HYPERTENSION
• OBESITY, DM
CLINICAL FEATURES & COMPLICATIONS
MCD MEMBRANOUS GP. FSGS
MCC of CHILDREN ELDERLY ADULTS
nephrotic
syndrome
in
H/O MCC in MALIGNANCY MCC in HIV
VACCINATION

36. All of the following are typical causes of secondary membranous
glomerulopathy EXCEPT?
A. Hepatitis C
B. Malignancy
C. Nonsteroidal anti-inflammatory drug use
D. Tuberculosis

37. Which of the following is false for HIV associated nephropathy
(HIVAN)?
A. May be the first manifestation of HIV infection

B. More common in Blacks
C. More frequent in IV drug abusers
D. Slow clinical course

RENAL DISEASE IN HIV
• 50% HIV affected individuals with kidney disease - HIVAN (HIV-
associated nephropathy)
• Africans > Whites
• Biopsy - FSGS (Collapsing Glomerulopathy)
• HIVICK?
• Presentation
• Nephrotic Range proteinuria & Hypoalbuminemia
• Uncommon - HTN, Edema, Hyperlipidemia
• Renal USG - Large echogenic kidneys
• Treatment
• RAS inhibitors
• ART
• Renal Transplantation
38. Atypical HUS (aHUS) best relates to?
A. Shiga toxin (verotoxin)

B. Thrombospondin receptor
C. Complement protein deficiencies
D. Plasminogen activator inhibitor 1 (PAI-1)

HUS vs TTP
FEATURE HUS TTP
CAUSE • INFECTION • HEREDITARY (Upshaw Schulman Syndrome)
1. MC - E. Coli (O157:H7) Mutation in ADAMTS 13 -> Large VWF aggregates ->
Platelet thrombus formation
• ACQUIRED
1. Autoimmune (iTTP)
2. Streptococcus pneumoniae 2. Drug Induced
• Dose dependent (Mitomycin C, Cyclosporine,
• AUTOIMMUNE Tacrolimus etc.)
• aHUS : Complement dysregulation • Drug induced Ab (Quinine)
-Factor H, I, B deficiency 3. HIV
-DEAP HUS (AB against Factor H) 4. Pregnancy
CLINICAL • Fever + • Fever +
FEATURES • Acute Renal Failure ++ • Acute Renal Failure +
• Neurological + • Neurological ++
• Thrombocytopenia + • Thrombocytopenia +
• MAHA + • MAHA +
HUS vs TTP
FEATURE HUS TTP
INVESTIGATIO • PT/aPTT : Normal
NS • Coomb’s Test : Negative
TREATMENT • Mainstay: Supportive (Fluid & • Mainstay: Plasmapheresis
Electrolyte balance)
• Plasmapheresis • Immunomodulators (Rituximab, Vincristine)
• Immunomodulators (Rituximab, • iTTP (Caplacizumab)

Vincristine)
• aHUS (Eculizumab, Ravulizumab)

• Strep. pneumonia HUS : Antibiotics
& washed red cells

39. Which of the following drugs is approved for use in acquired
autoantibody mediated TTP (iTTP)?
A. Caplacizumab
B. Eculizumab
C. Ravuliumab
D. Rituximab

40. Which of the following condition presents with Thrombotic
Microangiopathy without Microangiopathic Hemolytic Anemia?
A. POEMS syndrome
B. HELLP syndrome
C. aHUS
D. cTTP

41. Caroli's disease leading to portal hypertension presenting as
Hepatosplenomegaly is a feature of?
A. Autosomal Dominant PCKD

B. Autosomal Recessive PCKD
C. Von Hippel Lindau Disease
D. Nephronophthisis

CYSTIC DISEASES
• Site of Cyst Cortex & Medulla Medulla Only
Polycystic Disease Medullary Cystic Disease
ADTKD

POLYCYSTIC KIDNEY DISEASE
FEATURE AUTOSOMAL DOMINANT AUTOSOMAL RECESSIVE
GENE & Gene- PKD1, PKD 2 Gene- PKHD 1
PROTEIN Protein- Polycystin 1, Polycystin 2 Protein- Fibrocystin
CLINICAL • Age of presentation : 4th - 5th decade • Age of presentation : In utero or <1 month
FEATURES • Renal: • Renal:
- MC: B/L chronic flank pain (Cyst infecton, - MC: B/L enlarged kidneys on palpation
hmg. or nephrolithiasis) - Oligohydramnios, Pulmonary hypoplasia (↓
- Polycythemia (↑ EPO) fetal urine production)
-Hematuria (Cyst rupture) • Extra Renal:
• Extra Renal: - Congenital Hepatic Fibrosis
- MC: Hepatic cyst (others- pancreas, spleen, -Caroli disease (dilatation of intra hepatic bile
ovary) ducts)
-CNS : Berry aneurysms
-CVS : MVP -Present as Portal HTN (HSM,variceal bleed)
-Intestine : Colonic diverticula
• Cause of Death:CV complication>Infection

POLYCYSTIC KIDNEY DISEASE
FEATURE AUTOSOMAL DOMINANT AUTOSOMAL RECESSIVE
DIAGNOSIS • Positive family history • IOC : USG
• IOC : USG -Diagnosis can be made in utero after 24 weeks
-15-29 yrs: Atleast 2 renal cysts of gestation in severe cases
-30-59 yrs: Atleast 2 cysts in each kidney -Blateral enlarged kidney in infant with kidneys
-≥60 yrs: Atleast 4 cysts in each kidney of both parents being normal
-Sensitivity & Specificiy
PROGNOSIS & • No Specific treatent • Mortality ~60% in <1 month age
TREATMENT • BP <140/90 mmHg • Renal Replacement therapy
• Cyst Infection : Lipid soluble antibiotics
(Co-trimoxazole, Quinolones etc)
• Surgical decompresion of cysts
• Renal Replacement therapy
• Everolimus, Sirolimus, Tolvaptan
• - cAMP (↓ growth of cysts)

42. Which of the following is false about Medullary Cystic Kidney
Disease Type II?
A. Absence of Proteinuria
B. Mutation in gene encoding Tamm-Horsfall protein.
C. Benign urine sediments
D. Decreased uric acid levels

ADTKD
• Progressive Renal Failure & Benign urine sediment
FEATURE ADTKD-MUC1 ADTKD-UMOD

Name MCKD I MCKD II
Mutation MUC 1 (Mucin) UMOD (Uromodulin/Tamm
Horsfall)
Uric Acid levels Normal Elevated
Clinical • Adults • Adults (20-50 yr)
Features • Slowly progressive CKD • Slowly progressive CKD
• Minimal proteinuria • No proteinuria
• Occasional renal cysts • Occasional renal cysts (USG)
(USG)
IOC: RENAL • Tubulonterstitial Fibrosis
Throughout Medulla
BIOPSY • Tubular Atrophy
Treatment • Renal Replacement Therapy
43. Renal cell carcinoma is a feature of which of the following inherited
diseases associated with a cystic phenotype?
A. Autosomal recessive PCKD

B. Medullary cystic kidney
C. Nephronophthisis
D. Tuberous Sclerosis

44. Sjogren's syndrome can be associated with?
A. Aristolochic Acid
B. Hyperuricemia
C. Nephrogenic DI
D. Nephrocalcinosis

CLASSIFICATION

• Sjogren’s Syndrome
• Target exocrine glands
• Dry eyes
• Dry mouth
• MC renal presentation : TIN with predominant
lymphocytic infiltrate
• Complications:
• Azotemia
• Distal RTA
• Nephrogenic DI
• Treatment:
• Initial : Glucocorticoids
• Maintenance : Azathioprine, Myophenolate mofetil

45. All of the following metabolic disturbances are associated with
tubulointerstitial disease except?
A. Hypercalcemia
B. Hyperuricemia
C. Hyperoxaluria
D. Hyperkalemia

• Hypercalcemic Nephropathy
• Causes
• Primary Hyperparathyroidism
• Sarcoidosis
• Multiple myeloma
• Vitamin D intoxication
• Metastatic bone disease
• Pathogenesis
• Tubular cell necrosis -> ppt. of calcium salts
• Dilation & atrophy of tubules
• Glomerular & renal arteriole calcium deposition
• Treatment
• ↓ s. Calcium concentra on
• Treat primary pathology

• Hyperuricemic Nephropathy
• Gouty nephropathy
• Pathogenesis
• Crystalline deposits of uric acid
• Intrarenal obstruction
• Inflammatory response -> Lymphocytic infilration.
foreign body giant cell reaction -> Fibrosis
• Treatment
• Allopurinol
• Urine alkalization

• Hypokalemic Nephropathy
• Causes
• Chronic laxative or Diuretic use
• Surreptitious vomiting
• Primary aldosteronism
• Pathogenesis
• Reversible tubular lesion (Vacuolar degeneration)
• Later: Tubular atrophy, cystic dilation, fibrosis--> ESRD
• Treatment
• Timely identification and correction

46. In which of the following cases would treatment with
corticosteroids for biopsy-proven interstitial nephritis be most likely to
impact long-term renal recovery?
A. 37-year-old woman with sarcoidosis

B. 48-year-old man with slowly progressing interstitial nephritis over 2 months
with fibrosis found onbiopsy
C. 54-year-old man with diabetes mellitus and recent Salmonella infection
D. 63-year-old man with allergic interstitial nephritisafter cephalosporin
antibiotic use

47. Which of the following is false about renal stones?
A. Higher potassium intake decreases calcium excretion

B. Higher sodium & sucrose intake increases calcium excretion
C. Magnesium & phytate lower renal stone risk
D. Drinking orange juice is associated with an increased risk of renal
stone formation

RENAL CALCULI
• Types:
• MC- Calcium oxalate(~75%)
• Calcium Phosphate (~15%), Uric Acid (~8%) Struvite, Cystine
• Risk Factors:
• Dietary Factors
• ↑ Risk: Animal protein, Oxalate, Sodium, Sucrose, Fructose
• ↓ Risk: Calcium, Potassium, Phytates
• Non Dietary Factors
• White middle-aged men, Weight gain, Obesity, Hot Environment
• Urinary Risk Factors
• ↓ Urine volume, ↑ Urine Calcium, ↑ Urine oxalate, ↑ Urine Uric acid, ↓ Urine citrate
• Urine pH
• Genetic Risk Factors
• Primary Hyperoxaluria, Cystinuria
48. Renal colic may be confused as acute diverticulitis when the stone
lodges at?
A. Ureteral pelvic junction

B. Pelvic brim
C. Ureterovesical junction
D. All of the above

CLINICAL FEATURES of RENAL CALCULI
• Two common presentations of renal colic:
1. Renal Colic
2. Painless gross hematuria
• Radiation of Pain:
• Stone in upper ureter --> Anterior radiation
• Stone in lower ureter --> Ipsilateral testicle/labium
• Other conditions to be ruled out:
SITE RIGHT SIDE LEFT SIDE
URETEROPELVIC JUNCTION ACUTE CHOLECYSTITIS
PELVIC BRIM ACUTE APPENDICITIS ACUTE DIVERTICULITIS
URETEROVESICAL JUNCTION URINARY FREQUENCY & URGENCY (~BACTERIAL CYSTITIS)

49. Activating mutations occur in which of the following channels in
Liddle's syndrome?
A. Na+
B. K+
C. Cl
D. HCO3-

Na+ CHANNELOPATHIES
MUTATIONS OF Na CHANNELS
Na+K+2Cl- Na+Cl- eNa+
- + - + - +
BARTTER’S X GITELMAN’S GORDON’S PSEUDO LIDDLE’S
SYNDROME SYNDROME SYNDROME HYPOALDOSTERONISM SYNDROME
TYPE 1
LIDDLE’S SYNDROME:
• Na+ gain -> Water gain -> ↑ BP
• Renin ↓ -> Aldosterone ↓
• K+ excre on ↑ -> Hypokalemia
• H+ excretion -> Metabolic alkalosis
• Treatment: Amiloride
50. Which of the following is not a finding in renal tubular defects?
A. Renal calcification
B. Electrolyte disorders
C. Hypoalbuminemia
D. Large Kidneys


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Dr Shubham Upadhyay

© Medi - Lectures Dr Shubham Upadhyay 1

© Medi - Lectures Dr Shubham Upadhyay 3

© Medi - Lectures Dr Shubham Upadhyay 6

© Medi - Lectures Dr Shubham Upadhyay 7

© Medi - Lectures Dr Shubham Upadhyay 8

A. Hypercalciuria is the principal contributor.

© Medi - Lectures Dr Shubham Upadhyay 10

© Medi - Lectures Dr Shubham Upadhyay 13

A. Hyponatremia with normal plasma tonicity

© Medi - Lectures Dr Shubham Upadhyay 14

© Medi - Lectures Dr Shubham Upadhyay 16

© Medi - Lectures Dr Shubham Upadhyay 17

© Medi - Lectures Dr Shubham Upadhyay 18

© Medi - Lectures Dr Shubham Upadhyay 19

• AVP antagonists (Vaptans)

© Medi - Lectures Dr Shubham Upadhyay 21

© Medi - Lectures Dr Shubham Upadhyay 22

© Medi - Lectures Dr Shubham Upadhyay 23

A. Tall peaked T waves

© Medi - Lectures Dr Shubham Upadhyay 24

slow idioventricular rhythms, Loss of P waves

ventricular tachycardia, Widened QRS pattern

ventricular fibrillation, and Sine wave pattern

© Medi - Lectures Dr Shubham Upadhyay 25

A. Tall peaked T waves

© Medi - Lectures Dr Shubham Upadhyay 26

A. Glucose insulin therapy

© Medi - Lectures Dr Shubham Upadhyay 28

© Medi - Lectures Dr Shubham Upadhyay 30

A. Na 140, K 4.0, Cl 106, HCo3 14, AG 20, PaCO2 24, pH 7.39

© Medi - Lectures Dr Shubham Upadhyay 32

A. {Na + K}u / {Cl}u

© Medi - Lectures Dr Shubham Upadhyay 33

A. Rise in s. creatinine from baseline 0.5 mg/dL within 48 hrs

© Medi - Lectures Dr Shubham Upadhyay 34

© Medi - Lectures Dr Shubham Upadhyay 35

A. Acute Interstitial Nephritis

© Medi - Lectures Dr Shubham Upadhyay 37

A. Myogenic reflex in afferent arteriole

© Medi - Lectures Dr Shubham Upadhyay 40

© Medi - Lectures Dr Shubham Upadhyay 41

A. Acute interstitial nephritis

A. BUN/Plasma Creatinine ratio is 10:1 in Acute Tubular Necrosis

© Medi - Lectures Dr Shubham Upadhyay 43

© Medi - Lectures Dr Shubham Upadhyay 45

© Medi - Lectures Dr Shubham Upadhyay 46

© Medi - Lectures Dr Shubham Upadhyay 47

© Medi - Lectures Dr Shubham Upadhyay 48

© Medi - Lectures Dr Shubham Upadhyay 49

© Medi - Lectures Dr Shubham Upadhyay 50

A. Increased risk of stroke in patients with Type 2 diabetes

© Medi - Lectures Dr Shubham Upadhyay 52

© Medi - Lectures Dr Shubham Upadhyay 53

A. Anorexia & nausea not relieved by protein restriction

© Medi - Lectures Dr Shubham Upadhyay 54

© Medi - Lectures Dr Shubham Upadhyay 55

© Medi - Lectures Dr Shubham Upadhyay 56

© Medi - Lectures Dr Shubham Upadhyay 57

© Medi - Lectures Dr Shubham Upadhyay 58

A. Anaphylactoid reactions to dialyzer

© Medi - Lectures Dr Shubham Upadhyay 59

• Infection • Adynamic Bone Disease