PROVISIONAL ANSWER KEY
Question Paper Code: 37/2024/OL
Category Code: 520/2023
Exam: Assistant Professor in Nephrology
Date of Test 06-04-2024
Department Medical Education
Question1:-Which is the most common site of absorption of magnesium in the GI tract?
A:-caecum
B:-distal small intestine
C:-colon
D:-duodenum
Correct Answer:- Option-B
Question2:-Which of the following mutation is associated with Hypermagnesemia?
A:-gain of function mutation of claudin 10a
B:-loss of function mutation of claudin 10a
C:-gain of function mutation of claudin 2
D:-loss of function mutation of claudin 2
Correct Answer:- Option-B
Question3:-From which segment of tubule does maximum reabsorption of magnesium take place?
A:-proximal tubule
B:-thick ascending limb of henles loop
C:-DCT
D:-collecting duct
Correct Answer:- Option-B
Question4:-Which among the following mutations cause Familial Hypomagnesemia with Hypercalciuria and Nephrocalcinosis (FHHNC)?
A:-Claudin 16
B:-Claudin 10a
C:-Claudin 14
D:-Claudin 22
Correct Answer:- Option-A
Question5:-A child presented with dysuria, increased frequency of urination with low grade fever. On evaluation found to have plenty of pus cell in urine routine with positive urine culture, for which patient received IV antibiotics. After 2 days patient developed fatigue, generalized weakness. Further evaluation revealed following
S.Na-132
S.K-2.9,
S.Ca-6.8,
S.Mg-1,
S.Phosphate-3.5
ABG-ph-7.53
pco2-41
Hco3-32
AG-10
Which will be the possible antibiotic used in this case?
A:-linezolid
B:-penicillin
C:-amikacin
D:-ceftriaxone
Correct Answer:- Option-C
Question6:-Which type of Bartter syndrome is associated with hypomagnesemia?
A:-type 5
B:-type 3
C:-type 4
D:-type 1
Correct Answer:- Option-B
Question7:-Which is the most appropriate cut off value for fractional excretion of magnesium to confirm the renal loss of magnesium as a cause of hypomagnesemia?
A:-0.5%
B:-1%
C:-2%
D:-5%
Correct Answer:- Option-D
Question8:-Which antidiabetic medication has been suggested to correct hypomagnesemia?
A:-DPP4 inhibitors
B:-metformin
C:-GLP1 agonist
D:-SGLt2 inhibitors
Correct Answer:- Option-D
Question9:-Which among the following oral formulation of magnesium supplement has the highest elemental magnesium per tablet?
A:-magnesium oxide
B:-magnesium carbonate
C:-magnesium chroide
D:-magnesium citrate
Correct Answer:- Option-A
Question10:-At what level of magnesium does a patient develop respiratory paralysis and hypotension?
A:-3 mg/dl
B:-7.2 mg/dl
C:-12 mg/dl
D:-5 mg/dl
Correct Answer:- Option-C
Question11:-Which is the first step in the synthesis of o-linked glycans of circulatory IgA?
A:-Action of alpha-2,3 sialyltransferase on galactose residue
B:-Action of alpha 2,6 sialyltransferase on GAINac
C:-Beta galactosylation by 3-beta galactosyltransferase
D:-Addition of GAINAc to serine or threonine residues by Nacetyl galactosamine transferase
Correct Answer:- Option-D
Question12:-Which immunoglobulin is most clearly associated with the pathogenesis of IgA nephropathy?
A:-hypoglycosylated polymeric IgA1
B:-hypergalactosylated polymeric IgA1
C:-hypogalactosylated polymeric IgA1
D:-hypoglycosylated polymeric IgA2
Correct Answer:- Option-C
Question13:-Which among the following immunologic components is mostly frequently co-deposited in the mesangium along with IgA in the immunofluorescence study of IgA nephropathy specimen?
A:-IgG
B:-IgM
C:-C3
D:-C4
Correct Answer:- Option-C
Question14:-Which of the following disease condition is not usually associated with secondary IgA nephropathy?
A:-Celiac disease
B:-Ankylosing spondylitis
C:-Renal cell carcinoma
D:-Leptospirosis
Correct Answer:- Option-D
Question15:-Which among the following components of MEST-C scoring is associated with a maximum score of 2?
A:-mesangial hypercellularity
B:-endocapillary proliferation
C:-segmental sclerosis
D:-tubular atrophy
Correct Answer:- Option-D
Question16:-Which among the following has no impact on the prognostication of IgA nephropathy?
A:-recurrent macroscopic hematuria
B:-intensity of IgA deposition on immunofluorescence study
C:-gross obesity
D:-systemic hypertension
Correct Answer:- Option-B
Question17:-What among the following is related to VALIGA study?
A:-use of valsartan in IgA nephropathy
B:-validation of Oxford classification of IgA nephropathy
C:-efficacy of combined SGIT2 inhibitor and ARB use in IgA nephropathy
D:-use of Finerenone in IgA nephropathy
Correct Answer:- Option-B
Question18:-Which amongst the following factors has a definite role in deciding treatment of IgA nephropathy?
A:-endocapillary proliferation on biopsy
B:-high serum IgA titres
C:-proteinuria at presentation
D:-low serum C3 levels
Correct Answer:- Option-C
Question19:-Which drug is being studied as a promising candidate for treatment of IgA nephropathy in ENVISION study?
A:-Sibeprenlimab
B:-Bortezomib
C:-Rituximab
D:-Budesonide
Correct Answer:- Option-A
Question20:-Which population has studies proving consistent beneficial role of tonsillectomy in IgA nephropathy?
A:-Chinese
B:-Japan
C:-India
D:-Caucasian
Correct Answer:- Option-B
Question21:-A 16 year old female being evaluated for occasional pedal edema of 3 weeks duration is found to have hypertension, serum creatinine of 1.8 mg/dl, microhematuria and 1.2 gm/day proteinuria. Her initial screen reveals low complement 3 levels. All of the following could be kept in differential diagnosis Except:
A:-Lupus Nephritis
B:-Membranoproliferative Glomerulonephritis
C:-Haemolytic Uraemic Syndrome
D:-Post infective Glomerulonephritis
Correct Answer:- Option-C
Question22:-Glomerular endotheliosis is a characteristic feature of
A:-Preeclamptic toxemia
B:-Lupus nephritis
C:-Anti GBM antibody disease
D:-Sarcoidosis
Correct Answer:- Option-A
Question23:-A 16 year old female being evaluated for occasional pedal edema of 3 weeks duration is found to have hypertension, serum creatinine of 1.8 mg/dl, microhematuria and 1.2 gm/day proteinuria. Her initial screen reveals low complement 3 levels. All of the following could be kept in differential diagnosis. Except:
A:-Lupus Nephritis
B:-Membranoproliferative Glomerulonephritis
C:-IgA Nephropathy
D:-Post infective Glomerulonephritis
Correct Answer:- Option-C
Question24:-All the following anti-neoplastic drugs can cause hemorrhagic cystitis except
A:-Cyclophosphamide
B:-Vincristine
C:-Busulfan
D:-Adriamycin
Correct Answer:- Option-D
Question25:-AURORA trial used which of the following therapeutic agent in lupus nephritis
A:-Voclosporin
B:-Tabalumab
C:-Blisibimod
D:-Bortezomib
Correct Answer:- Option-A
Question26:-LUNAR trial in lupus nephritis studied which of the following agents
A:-Belimumab
B:-Rituximab
C:-Atacicept
D:-Infliximab
Correct Answer:- Option-B
Question27:-Which of the following confer increased risk of development of SLE?
A:-DR3
B:-DR4
C:-DR11
D:-DR16
Correct Answer:- Option-A
Question28:-Conclusion of MAINTAIN trial in LN
A:-MMF is superior to AZA as a maintenance agent
B:-MMF is not superior to AZA as a maintenance agent
C:-MMF is equal to AZA as a maintenance agent
D:-MMF is non inferior to AZA as a maintenance agent
Correct Answer:- Option-B
Question29:-ALMS maintenance trial in LN concluded that
A:-MMF was superior to AZA as a maintenance therapy
B:-MMF is not superior to AZA as a maintenance agent
C:-MMF is equal to AZA as a maintenance agent
D:-MMF is non inferior to AZA as a maintenance agent
Correct Answer:- Option-A
Question30:-Which of the following marker can help in differentiating a flare of lupus nephritis from pre-eclampsia?
A:-Presence of low serum C3 levels
B:-Presence of Proteinuria more than 5 gm/day
C:-Presence of RBCs in urine
D:-Presence of ESR>70 mm/hr
Correct Answer:- Option-C
Question31:-All of the following are true regarding Barter's Syndrome except:
A:-Hypermagnesuria
B:-Response to thiazide diuretic is blunted
C:-Increased incidence of stone disease
D:-Kalemia
Correct Answer:- Option-C
Question32:-While evaluating a child for metabolic alkalosis, the most useful test to differentiate between vomiting and Barter's syndrome is
A:-Presence of Metabolic Alkalosis
B:-Urinary chloride excretion
C:-Hypokalemia
D:-Increased PGE2 excretion
Correct Answer:- Option-B
Question33:-Which of the following syndrome is mimicked by the use of thiazide diuretics?
A:-Bartter's Syndrome
B:-Gitelman Syndrome
C:-Liddle's Syndrome
D:-Fanconi's Syndrome
Correct Answer:- Option-B
Question34:-The major role of connecting segment is
A:- Secretion
B:- Secretion
C:-Water reabsorption
D:- re-absorption
Correct Answer:- Option-A
Question35:-The main site of action of ANP is
A:-Afferent arteriole
B:-Proximal tubule
C:-Inner Medullary collecting duct
D:-Outer medullary collecting duct
Correct Answer:- Option-C
Question36:-Factors affecting the secretion in collecting duct are all except
A:-Transepithelial electrogenic potential
B:-Distal flow rate
C:-Proximal sodium reabsorption
D:-Glucocortcoid hormones
Correct Answer:- Option-D
Question37:-All of the following are true regarding owe syndrome except:
A:-Fanconi Syndrome
B:-Hypertonia
C:-Mental Retardation
D:-Joint Deformity
Correct Answer:- Option-B
Question38:-All are true regarding S3 segment of proximal tubule except:
A:-Is involved in secretion of acid and base
B:-Is damaged most frequently by nephrotoxic drugs
C:-Has most prominent vacuolar lysosomal system
D:-Mitochondria are small and randomly arranged
Correct Answer:- Option-C
Question39:-In an otherwise healthy person with dehydration, free water clearance is
A:-Positive
B:-Negative
C:-Zero
D:-May be positive or negative depending on the urine volume
Correct Answer:- Option-B
Question40:-Urinary anionic gap is a marker of urinary excretion of
A:-Sulphates
B:-Phosphates
C:-Ammonium
D:-Bicarbonate
Correct Answer:- Option-C
Question41:-PLASMIC SCORE criteria include all of the following except
A:-Platelet count less than
B:-No active cancer
C:-INR < 1.5
D:-Creatinine more than 2 mg/dl
Correct Answer:- Option-D
Question42:-True about thrombotic microangiopathy
A:-Normal Prothrombin time
B:-Prolonged aPTT
C:-Fibrinogen reduced
D:-Normal/↓ LDH
Correct Answer:- Option-A
Question43:-The mutation which has least common association with atypical hemolytic uremic syndrome
A:-CFH
B:-MCP
C:-DGKE
D:-CFB
Correct Answer:- Option-D
Question44:-The cells/cell affected in STEC-HUS
A:-Endothelium
B:-Podocyte
C:-Mesangial cell in tubular epithelial cell
D:-All of the above
Correct Answer:- Option-D
Question45:-STEC-HUS has an incubation period after the onset of diarrhea of
A:-2-3 days
B:-6-10 days
C:-14-21 days
D:-Upto 1 year
Correct Answer:- Option-B
Question46:-The antibiotics which can be given safely in HUS
A:-Quinolones
B:-Trimethoprim
C:-Azithromycin
D:-All of the above
Correct Answer:- Option-C
Question47:-Plasma therapy in aHUS is not effective in patients with
A:-CFI mutation
B:-CFB
C:-C3 mutation
D:-MCP mutation
Correct Answer:- Option-D
Question48:-Recurrence after a kidney transplantation in HUS is
A:-60-80%
B:-40-50%
C:-20-30%
D:-<20%
Correct Answer:- Option-A
Question49:-Lowest incidence of recurrence is noted in all except
A:-MCP mutation
B:-DGKE mutation
C:-THBD mutation
D:-Both 1 and 2
Correct Answer:- Option-C
Question50:-Coombs test is positive in aHUS
A:-aHUS with CFB mutation
B:-Streptococcal pneumonia associated aHUS
C:-DGKE mutation associated with aHUS
D:-All of the above
Correct Answer:- Option-B
Question51:-Increased bone turnover is seen in
A:-Osteitis fibrosa
B:-Osteomalacia
C:-Adynamic bone disease
D:-Both 1 and 2
Correct Answer:- Option-A
Question52:-Renal osteodystrophy in CKD starts at eGFR level of
A:-60 ml/min/1.732
B:-50 ml/min/1.732
C:-40 ml/min/1.732
D:--<15 ml/min/1.732
Correct Answer:- Option-B
Question53:-Serum calcium levels in CKD is modified by all except
A:-Vitamin D
B:-PTH
C:-FGF23
D:-Both 1 and 2
Correct Answer:- Option-C
Question54:-FGF23 true statement is/are
A:-Inhibit PTH secretion
B:-Reduction of 1 a hydroxylase
C:-Catabolism of Vitamin D
D:-All of the above
Correct Answer:- Option-D
Question55:-Measure/Measures of osteoblastic activity in CKD-MBD
A:-Aminoterminal of propeptide of type 1 procollagen (P1NP)
B:-Tartarate resistant acid phosphatase
C:-Collagen degradation products
D:-All of the above
Correct Answer:- Option-A
Question56:-Dietary phosphate restriction should start by CKD stage
A:-2 and 3
B:-4
C:-5
D:-All of the above
Correct Answer:- Option-A
Question57:-Identify the false statement regarding the phosphate binding capacity
A:-1 gm of calcium carbonate binds 40 mg
B:-1 gm Sevelamer binds 36 mg
C:-1 gm Lanthanum binds 20 mg
D:-1 gm Sucroferric oxide binds 100 mg
Correct Answer:- Option-C
Question58:-Prevalence of adynamic bone disease in stage 5 CKD is
A:-10%
B:-20%
C:-30%
D:->40%
Correct Answer:- Option-D
Question59:-Adynamic bone disease is characterized by
A:-Low bone turnover/normal mineralization/high osteoid volume
B:-Low bone turnover/normal or high sec mineralization/low osteoid volume
C:-High bone turnover/low mineralization/high osteoid volume
D:-High bone turnover/low mineralization/low osteoid volume
Correct Answer:- Option-B
Question60:-CKD MBD diagnostic criteria for B2 microglobulin amyloidosis include all except
A:-Cystic bone radiolucencies with >10 nm on shoulder with a size more than 5 mm on wrist
B:-Joint space adjacent to cystic lesion is enlarged
C:-Increase of defect (cystic radiolucencies) diameter > 30% per year
D:-Presence of defect in atleast 2 joints
Correct Answer:- Option-B
Question61:-The hemodiafilteration type in which the first part of the blood circuit is operated in post dilution mode and second part in pre dilution mode is called
A:-Post dilution hemodialfiltration
B:-Pre dilution hemodialfiltration
C:-Mid dilution hemodialfiltration
D:-Mixed dilution hemodialfiltration
Correct Answer:- Option-C
Question62:-True about effective convection volume in hemodialfiltration (HDF)
A:-Higher in pre dilution mode of hemodialfiltration
B:-Higher in mixed dilution hemodialfilteration
C:-Equal to total
D:-Relationship with HDF mode
Correct Answer:- Option-C
Question63:-Identify the odd man out trial in the group
A:-CONTRAST
B:-CANUSA
C:-ESHOL
D:-TURKISH
Correct Answer:- Option-B
Question64:-In post dilution HDF, filtration fraction should be limited to
A:-10-15% of blood flow rate
B:-20-25% of blood flow rate
C:-30-35% of blood flow rate
D:-40% of blood flow rate
Correct Answer:- Option-B
Question65:-Higher substitution volumes are needed in all except
A:-Pre dilution hemodialfiltration × 2
B:-Mixed dilution hemodialfiltration × 1.5
C:-Mid dilution hemodialfiltration × 1.5
D:-Post dilution hemodialfiltration
Correct Answer:- Option-D
Question66:-An ideal HDF membrane should have
A:-Seiving coefficient as close to 0 for albumin and 1 for middle molecule
B:-Seiving coefficient 1 for albumin and 0 for middle molecule
C:-Seiving coefficient 0 for both albumin and middle molecule
D:-Seiving coefficient 1 for both albumin middle molecule
Correct Answer:- Option-A
Question67:-Cartoon trial-a RCT compared
A:-Expanded hemodialysis vs high flux dialysis
B:-Expanded hemodialysis vs predilution HDF
C:-Expanded hemodialysis vs post dilution HDF
D:-Expanded hemodialysis vs low flux dialysis
Correct Answer:- Option-C
Question68:-Which among the following modality requires the highest blood flow rate?
A:-SLED
B:-CVVHF
C:-CVVHD
D:-SCUF
Correct Answer:- Option-B
Question69:-Which among the following increases the risk of filter clotting in CVVHF?
A:-High Qb (Blood flow rate)
B:-Pre-filter replacement
C:-Filtration fraction > 25%
D:-Heparin anticoagulation
Correct Answer:- Option-C
Question70:-Which among the following is not a complication of citrate anticoagulation?
A:-Hyponatremia
B:-Hypocalcemia
C:-Hypernatremia
D:-Metabolic alkalosis
Correct Answer:- Option-A
Question71:-Trial which showed that one year post transplant, Daclizumal, 2g MMF, Low Dose Tacrolimus and Steriods resulted in better renal function and lower acute rejections.
A:-Elite Symphony trial
B:-Convert trial
C:-Transform trial
D:-Orion trial
Correct Answer:- Option-A
Question72:-What is the major cause of drug resistance in CMV Post transplant?
A:-Alteration in UL 97
B:-Alteration in UL 94
C:-Alteration in UL 54
D:-All of the above
Correct Answer:- Option-A
Question73:-Absolute contraindication for renal transplantation
A:-Flow cytometry positive
B:-DSA positive
C:-CDC positive
D:-1 and 2
Correct Answer:- Option-C
Question74:-Which is least risk for recurrence, post transplantation?
A:-SLE Nephritis
B:-FSGS
C:-HUS/TTP
D:-MPGN
Correct Answer:- Option-A
Question75:-Stripped interstitial fibrosis associated with arterial lesion is a common finding in
A:-CNI Toxicity
B:-MMF
C:-Steriods
D:-MToR inhibitor
Correct Answer:- Option-A
Question76:-Which statement is true about the mechanism of action of calcineurin inhibitors like Cyclosporine and Tacrolimus?
A:-They enhance the expression of interleukin-2 and promote T-cell proliferation
B:-They are structurally similar to corticosteroids and share the same sideeffect profile
C:-They block the transcription of cytokine genes that promote T-cell activation and proliferation
D:-They are derived from the bacterium Escherichia coli
Correct Answer:- Option-C
Question77:-What is the standard management for a urine leak after kidney transplantation?
A:-Immediate surgical intervention is necessary to repair the urine leak
B:-It is reasonable to place a nephrostomy tube to drain the urine if the leak does not heal on its own
C:-A nephrostomy tube should always be placed immediately after identifying a urine leak
D:-Urine leaks are managed with antibiotics and usually do not require any surgical procedures
Correct Answer:- Option-B
Question78:-Which radio pharmaceutical is primarily used to evaluate tubular secretion in renal imaging?
A:-DTPA
B:-MAG3
C:-DMSA
D:-Ga
Correct Answer:- Option-B
Question79:-What is the primary application of White Blood Cells (WBC) labeled with 111ln or 99mTc in the context of renal transplant function?
A:-Evaluation of glomerular fitration
B:-Assessment of inflammation
C:-Detection of infection
D:-Measurement of renal plasma flow
Correct Answer:- Option-C
Question80:-Assertion (A): peritubular capillary C4d depostition is associated with hyperacute rejection.
Reason (R): C4d is a degradation product that indicates complement activation, typically as a result of antibody-mediated damage to the vascular endothelium in the transplant.
A:-Both A and R are true, and R is the correct explanation of A
B:-Both A and R are true, but R is NOT the correct explanation of A
C:-A is true, but R is false
D:-A is false, but R is true
Correct Answer:- Option-A
Question81:-The NPHS1 gene mutation leads to which of the following conditions?
A:-Congenital Nephrotic Syndrome of the Finnish type
B:-Alport syndrome
C:-Denys-Drash syndrome
D:-Liddle syndrome
Correct Answer:- Option-A
Question82:-Podocin, encoded by the NPHS2 gene, is crucial for the function of which renal structure?
A:-Proximal tubule
B:-Distal tubule
C:-Glomerular basement membrane
D:-Slit diaphragm
Correct Answer:- Option-D
Question83:-In which syndrome are both renal tubulopathy and hearing loss observed due to mutations in a single gene?
A:-Bartter syndrome
B:-Jervell and Lange-Nielsen syndrome
C:-Alport Syndrome
D:-Denys-Drash Syndrome
Correct Answer:- Option-C
Question84:-Which condition is characterized by defective proximal tubular reabsorption and generalized aminoaciduria, glycosuria, phosphaturia, and uricosuria?
A:-Bartter syndrome
B:-Liddle syndrome
C:-Fanconi syndrome
D:-Gitelman syndrome
Correct Answer:- Option-C
Question85:-The genetic mutation in the SLC12A1 gene causes which of the following disorders?
A:-Liddle syndrome
B:-Bartter syndrome type I
C:-Gitelman syndrome
D:-Primary hyperaldosteronism
Correct Answer:- Option-B
Question86:-Which gene is primarily associated with X-linked dominant form of hypophosphatemic rickets, a disorder that can present with Fanconi Syndrome?
A:-PHEX
B:-FGF23
C:-DMP1
D:-CLCN5
Correct Answer:- Option-A
Question87:-Which of the following is a characteristic feature of nephronophthisis?
A:-Excessive calcium excretion
B:-Cyst development at the corticomedullary junction
C:-Hyperkalemia
D:-Increased urine concentration ability
Correct Answer:- Option-B
Question88:-Which statement best describes the mechanism of action of lumasiran in the treatment of primary hyperoxaluria type 1 (PH1)?
A:-Lumasiran increases the excretion of oxalate in the urine
B:-Lumasiran inhibits the intestinal absorption of dietary oxalate
C:-Lumasiran reduces the hepatic production of oxalate by silencing the gene responsible for its synthesis
D:-Lumasiran replaces the defective enzyme that causes oxalate overproduction in patients with PH1
Correct Answer:- Option-C
Question89:-Liddle syndrome mimics the effects of which of the following?
A:-Excessive aldosterone production
B:-Thiazide diuretic use
C:-Loop diuretic use
D:-Antidiuretic hormone deficiency
Correct Answer:- Option-A
Question90:-In which condition is there a risk of developing renal cell carcinoma?
A:-Alport syndrome
B:-Von Hippel-Lindau disease
C:-Bartter syndrome
D:-Gitelman syndrome
Correct Answer:- Option-B
Question91:-What distinguishes Type 1 from Type 2 renal tubular acidosis?
A:-Type 1 involves a defect in bicarbonate reabsorption, while Type 2 involves a defect in acid secretion
B:-Type 1 involves a defect in acid secretion at the distal tubule, while Type 2 involves a defect in bicarbonate reabsorption at the proximal tubule
C:-Only Type 1 is associated with kidney stones
D:-Only Type 2 leads to hypokalemia
Correct Answer:- Option-B
Question92:-18-year-old woman was referred for investigation of progressive generalized muscle weakness and lethargy. She was taking no regular medications. Examination was unremarkable. Her BP was 110/60 mmHg. Her body mass index was 19. Investigations: serum K 2.7 mmol/L (3.5-4.9), serum bicarbonate 34 mmol/L (20-28), serum creatinine 72 μmol/L (60-110), estimated glomerula filtration rate (MDRD) > 90 ml/min (>60), serum magnesium 0.60 mmol/L (0.75-1.05), serum chloride 83 mmol/L (95-107), urinary chloride 60, urinary Na 55.24-hour urinary calcium 1.5 mmol (2.5-7.5). What is the most likely diagnosis?
A:-Bartter's syndrome
B:-Gitelman's syndrome
C:-Hypokalaemic periodic paralysis
D:-Liddle's syndrome
Correct Answer:- Option-B
Question93:-For assessing renal scars in the context of CAKUT, which imaging method is considered the gold standard?
A:-DMSA scintigraphy
B:-Ultrasound
C:-MRI
D:-CT Scan
Correct Answer:- Option-A
Question94:-Which one of the following statements is true regarding ARPKD?
A:-Onset is typically in the third decade
B:-Liver involvement is rare
C:-Is due to a defect on chromosome 16
D:-May be detected on prenatal ultrasound
Correct Answer:- Option-D
Question95:-Denys-Drash Syndrome is characterized by a triad of symptoms, which includes all of the following EXCEPT:
A:-Gonadal dysgenesis
B:-Nephropathy
C:-Wilms' tumor
D:-Hpothyroidism
Correct Answer:- Option-D
Question96:-Which is not seen in ARPKD?
A:-Enlarged echogenic kidneys
B:-Polyhydramnios
C:-Potter facies
D:-Pulmonary hypoplasia
Correct Answer:- Option-B
Question97:-What is the primary kidney-related complication of cystinosis?
A:-Acute Kidney Injury (AKI)
B:-Chronic Kidney disease (CKD)
C:-Glomerulonephritis
D:-Fanconi syndrome
Correct Answer:- Option-D
Question98:-Which of the following is X linked?
A:-Nephronophthisis
B:-Meckel Gruber Syndrome
C:-Jubert syndrome
D:-Oro-facio-digital syndrome type I
Correct Answer:- Option-D
Question99:-Which is not a cause of Fanconi syndrome?
A:-Fabry disease
B:-Cystinosis
C:-Lowe Syndrome
D:-Dent disease
Correct Answer:- Option-A
Question100:-In Diabetes insipidus, all are true except
A:-Congenital diabetes insipidus (DI) is much more common than Acquired Diabetes Insipidus
B:-More than 90% of patients have X linked recessive NDI with pathogenic variants in AVPR2
C:-AVPR2 also mediates vasodilatation and release of Von Willebrand factor
D:-In 10% of the families, congenital NDI has an autosomal recessive inheritance with pathogenic variants in AQP2
Correct Answer:- Option-A
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