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UROLOGY > PCKD > Flashcards

PCKD Flashcards

1
Q

PKD1 & PKD2 gene
⭐ Produces
⭐ Genes

🧠⚡4² = 16 ⚡

A

⭐ POLYCYSTIN 1 & POLYCYSTIN 2

⭐ PKD1 gene = Ch 16p
⭐ PKD2 gene = Ch 4q

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2
Q

Which gene mutation in ADPKD has BETTER PROGNOSIS

A

PKD2 gene

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3
Q

PKD3 gene
⭐ produces

A

GANAB gene
⭐ POLYCYSTIN 3

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4
Q

Function of POLYCYSTIN

A
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5
Q

Which RECEPTORS are UPREGULATED in ADPKD?

🧠⚡CVS⚡

A
  1. cAMP
  2. V2 Receptors
  3. Somatostatin RECEPTORS
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6
Q

DOC for ADPKD

A

TOLVAPTAN

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7
Q

REVERSE UNIFIED DIAGNOSTIC CRITERIA
⭐ Used for diagnosis of
⭐ Based on

A

🎯 ADPKD
🎯 USG: Kidney

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8
Q

REVERSE UNIFIED DIAGNOSTIC CRITERIA

A
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9
Q

If Adult with FAMILY H/O ADPKD does not develop CYSTS in Kidney (MRI) by 30 years, the

A

Will NOT DEVELOP ADPKD

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10
Q

⚡⚡ MOST COMMON AGE OF PRESENTATION of ADPKD

A

20-40 yrs

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11
Q

🧑🏻‍⚕️ Clinical Features of POLYCYSTIC KIDNEY DISEASE

A
  1. Hypertension
  2. Renomegaly ➡️ Abdominal discomfort
  3. Nocturia (DUE TO: Impaired CONCENTRATING Ability of Kidney)
  4. Anemia: Rare
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12
Q

60/60 rule in ADPKD

A

By 60 years of AGE, 60% patients will develop ESRD

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13
Q

Poor PROGNOSTIC FACTORS in ADPKD

A
  1. Black ♂️
  2. < 30 years
  3. Hematuria
  4. HTN at < 35 years age
  5. PKD1 TRUNCATING mutation
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14
Q

STRONGEST RISK FACTOR FOR RENAL FUNCTION DECLINE in ADPKD

A

Kidney Volume & Cyst Volume
(Diagnosed in MRI)

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15
Q

COMPLICATION of ADPKD

A
  1. Hypertension
    ✨ Target organ damage
    ✨ Biventricular Diastolic Dysfunction
  2. CYST Rupture(rare) ➡️ PERITONITIS
  3. ABDOMINAL PAIN
    ✨ CYST infection
    ✨ CYST hemorrhage
    ✨ Stones (Uric acid > Calcium oxalate)
  4. Necrotizing Pyomyositis
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16
Q

Indication of FDG-PET SCAN in ADPKD

A

Infections

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17
Q

Indication of CT-Urography SCAN in ADPKD

A

Stones

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18
Q

💊💉 MANAGEMENT of ADPKD

🧠⚡3C for Cyst infection ⚡

A
  1. Hypertension
    ⭐ ACE ⛔ (OR) ARBs
  2. Cyst Infection
    ⭐ Carbapenams
    ⭐ Ciprofloxacin
    ⭐ Cotrimoxazole
  3. Stones
    ⭐ Potassium Citrate
  4. Hydration
    ⭐ > 4 liters/day
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19
Q

Target BP in ADPKD

A

SYSTOLIC: 95-110
DIASTOLIC: 60-75

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20
Q

Why HYDRATION is an IMPORTANT TREATMENT MANAGEMENT TOOL in ADPKD?

A

Cyst growth is dependent on ADH
⬇️
Drinking LOTS of WATER ➡️ ADH ⬇️⬇️

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21
Q

NOVEL TREATMENT OPTIONS for ADPKD

A
  1. TOLVAPTAN
  2. SOMATOSTATIN
  3. EVEROLIMUS
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22
Q

Pre-transplant NEPHRECTOMY INDICATIONS in ADPKD

A
  1. Infections
  2. Bleeding
  3. Obstruction
  4. Hypertension
23
Q

Can Son (OR) Daughter of a patient of ADCKD be a donor?

A

Yes
If completed 30yrs age & NO CYST on MRI

24
Q

ASSOCIATIONS OF ADPKD
🧠⚡ Berries Are Very High In Vitamin C²⚡

A

Berries- Berry Aneurysms
Are- Aortic Dissection
Very- Valves: Mitral prolapse/Bicuspid AV
High- Hypertension
In- Intracranial Dolichoectasia
Vitamin- Von Meyenberg Complexes
C- Cysts in liver, pancreas, spleen, ovaries
C-Colonic diverticuli

25
Q

Extra-renal Cysts in ADPKD can be seen in all organs, except

A

Brain

26
Q

Indication for REMOVAL of BERRY ANEURYSMS

A

Size > 10mm

27
Q

Risk for RCC in ADPKD

A

Same Risk as GENERAL PUBLIC

28
Q

If RCC develops in ADPKD, it is

A

✨ B/L
✨ Multicentric
✨ Sarcomatoid type

29
Q

Mutation in ARPKD
⭐ Chromosome
⭐ Most Dangerous

A

PKHD1 (Fibrocystin-Polyductin Complex)

⭐ Chromosome
🎯 6

⭐ Most Dangerous
🎯 missense TRUNCATING MUTATION

30
Q

IN ARPKD, Renal Cysts are seen mainly in

A

MEDULLA
⭐ SIZE of Cyst is SMALLER
⭐ Cortical Cysts are NOT SEEN

31
Q

Which part of the NEPHRON is affected in ARPKD

A

Collecting Ducts

32
Q

PKHD1 is involved in TERMINAL DIFFERENTIATION of

A
  1. Collecting DUCT
  2. Biliary Duct
33
Q

ASSOCIATIONS of ARPKD

A
  1. Congenital HEPATIC FIBROSIS (Periportal Fibrosis)
  2. Caroli’s Disease
  3. Biliary Ectasia
  4. POTTER’S SYNDROME
34
Q

⚡⚡ MOST COMMON CAUSE of DEATH IN CHILDREN with ARPKD

A

Congenital HEPATIC Fibrosis

35
Q

USG of ARPKD

A

Hyperechoic MEDULLA with LOSS of CORTICO-MEDULLARY DIFFERENTIATION (CMD)

36
Q

Hypoplasia of CEREBELLAR VERMIS
➕
NEPHRONOTHISIS
➕
ABNORMAL BREATHING PATTERN

A

JOUBERT SYNDROME

37
Q

🧬 MODE OF INHERITENCE 💉 of JOUBERT SYNDROME

A

AR

38
Q

⚡⚡ MOST COMMON presentation OF JOUBERT SYNDROME

A

Abnormal Breathing PATTERN
⬇️
1. Frequent APNEA
2. Bradycardia & Alternating with HYPERPNEA

39
Q

Copy JOUBERT cards from PEDIA

A
40
Q

Small CONTRACTED KIDNEY
➕
CYSTS @ CORTICO-MEDULLARY JUNCTION
➕
Dilation of PAPILLARY COLLECTING DUCT of 1 (OR) Both Kidney

A

Medullary SPONGE DISEASE

41
Q

MEDULLARY SPONGE DISEASE
⭐ GENE MUTATION
🧬 MODE OF INHERITENCE 💉

A

⭐ GENE MUTATION
🎯 MKCD1 gene
🎯 Ch 1q21

🧬 MODE OF INHERITENCE 💉
🎯 AD

42
Q

🧑🏻‍⚕️ Clinical Features of MEDULLARY SPONGE DISEASE

A
43
Q

Synonyms of MEDULLARY SPONGE DISEASE

A
  1. LENARDUZZI KIDNEY
  2. CACCHI & RICCI Disease
44
Q

IVP findings of MEDULLARY SPONGE DISEASE

A
  1. Paint Brush Appearance
  2. Bouquet of Flower Appearance
45
Q

COMPLICATIONS of MEDULLARY SPONGE DISEASE
🧠⚡DUtCH⚡

A
  1. Distal RTA
  2. Urinary STASIS
  3. Calcium stasis
  4. Hypocitraturia
46
Q

Extra-Renal Manifestations of MEDULLARY SPONGE DISEASE

A
  1. Caroli’s DISEASE
  2. HEMI-HYPERTROPHY
  3. Congenital anomalies of URINARY TRACT
47
Q

Cause of YOUNG ONSET HYPERTENSION

A
  1. PCKD
  2. RENAL ARTERY STENOSIS
  3. PHEOCHROMOCYTOMA
48
Q

⚡⚡ MOST COMMON CLINICAL FEATURE OF ADPKD

A

Hypertension
⬇️
Mass
⬇️
Pain
⬇️
Hematuria

49
Q

Pain in ADPCKD is DUE TO:

A

✨ Hemorrhage inside CYSTS
✨ Renal CYSTS

50
Q

Surgical MANAGEMENT of PCKD

A
  1. Dialysis
  2. Transplant
  3. ROVSING PROCEDURE: Deroofing cyst is NOT Useful
51
Q

ROVSING PROCEDURE

A

Deroofing cyst in ADPCKD

52
Q

⚡⚡ MOST COMMON PRESENTATION OF MULTICYSTIC DYSPLASTIC KIDNEY

A

Abdominal LUMP at BIRTH

53
Q

💊💉 MANAGEMENT of MULTICYSTIC DYSPLASTIC KIDNEY

A

Conservative
⬇️
Dialysis (OR) Transplant

54
Q

MULTICYSTIC DYSPLASTIC KIDNEY is ASSOCIATED with

A

I/L Atresia of URETER

UROLOGY (12 decks)

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