Block 10 week 1 Flashcards
Autosomal Dominant Polycystic Kidney Disease
- Is an example of a familial kidney disease.
- 4th most common cause of pts on dialysis
- caused by a genetic fault that disrupts the normal development of some of the cells in the kidneys and causes cysts to grow
- symptoms: high blood pressure, headaches, abdominal pain and blood in the urine, excessive urination.
- Faults in 1 of 2 different genes are known to cause AKPD.
-PKD1 mutation - more severe, earlier onset (30-40yr old)
- PKD2 mutation - less sever, later onset (70 yr old)
- 2 versions of polycystic kidney disease.
- Autosomal dominant - presents in adulthood
- Autosomal recessive - present in infancy or before birth
Diagnoses: ultrasound scanning and genetic screening
Treatment: surgery, medications to control symptoms.
Advantages vs Disadvantages of being screened for ADPKD
The approach to patient with kidney disease lecture
- Kussmaul breathing - type of hyperventilation that is the lungs emergency response to acidosis
- Kussmaul breathing - laboured deeper breathing
Amyloidoisis
- rare disease when a protein called amyloid builds up in the organs.
- The disorder involves the kidneys 80-90% of patients
- There are amyloid deposits in the glomeruli.
- The commonest presentation is proteinuria or the nephrotic syndrome
Stages for AKI and CKD
AKI has 3 types: prerenal, intrinsic and postrenal
AKI has 4 phases.
1. ONSET phase: Kidney injury occurs
- OLIGURIC (anuric phase): Urine output decreases from renal tubule damage
- DIURETIC phase: The kidneys try to heal and urine output increases, but tubule scarring and damage occurs.
- RECOVERY phase: tubular edema resolves and renal function improves.
Measuring renal function
- changes in serum creatine.
Creatine produced from muscle breakdown. If everything else about a person stays the same and you measure creatine levels 6 month’s apart and it changes. The change would be due to the kidneys function. - Glomerular Filtration Rate (GFR)
- Estimated Glomerular Filtration Rate (eGFR)
Urine Tests
Basic: dipstick test, urine protein:creatine ratio
Special circumstances: urine culture, urine electrolytes, urine ‘stone screen’
Vescio-coli- fistula
-Abnormal connection that develops between the colon and bladder
Haematuria
- painless blood in the urine is alarming and could indicate a cancer
Renal ultrasound imaging
- First lien test in renal impairment
Plain CT
- 1st line for suspected stones
Contrast enhanced CT
CT angiogram:
- Renal artery stenosis
- Suspected bleeds post renal biopsy
CT Venogram:
- renal vein thrombosis
RENAL ANGIOGRAM
Renal MR Angiogram
DMSA scan
- Lets you know the relative function of each kidney separately unlike GFR which would give you the whole view.
-DMSA (dimercapto succinic acid) is a short-lived radioisotope that goes directly to the kidneys once inside the body and only stays radioactive for a few hours.
Overview:
- Ultrasound Scan first line for imaging
Treatment
- Renal transplants have better patient outcomes and are more cost effective
- The problem is the availability of organs
Dialysis
Two types:
- Hemodialysis - patients visit hospital 3 times weekly. Stay on dialysis 4hrs each time
- Peritoneal dialysis. Need to repeat 1-4 times a day
- Peritoneal dialysis is a type of dialysis that uses the peritoneum in a person’s abdomen as the membrane through which fluid and dissolved substances are exchanged with the blood. It is used to remove excess fluid, correct electrolyte problems, and remove toxins in those with kidney failure.
- Peritoneal dialysis can be done at home if patient is capable.
- Assisted peritoneal dialysis. Nurse comes in and helps you change peritoneal dialysis
Advantages of dialysis
Disadvantages of dialysis
- Patients have to be very careful with what they can eat or drink. Anything they eat between dialysis sessions remains in their system as kidneys are not functioning.
- Employment difficulties - due to time needed for dialysis
Juxtaglomerular apparatus:
- Macula densa (DCT) - senses Na+ levels and responds by activating RAAS. Causes reabsorption of Na+ therefore reabsorption of water
- Juxtaglomerular cells: secrete renin
- Mesangial cells: remove debris
Anatomy
- Right gonadal vein drains straight into the IVC
- Left gonadal vein drains into the left renal vain which drains into the IVC
- Something else you should know is the left renal vein is longer than the right renal vein
- Nutcracker syndrome: compression of the left renal vein by the superior mesenteric artery. Presentation abdominal pian, hematuria, varicocele
- Ureters run under the vas deferens and uterine artery and over the common iliac artery (water under the bridge).”Water under the bridge”; the ureter (carrying urine or “water”) traverses deep and inferior to (below) the uterine artery (the bridge).
- Complications can occur during gynecologic procedures to uterine artery or vas deferens because it is so close to the ureters.
- The detrusor muscle is a smooth muscle under autonomic control.
- Parasympathetic innervation promotes bladder contraction and urination. it receives parasympathetic signaling via the pelvic splanchnic nerves (S2-S4).
-Sympathetic innervation promotes bladder relaxation and urine storage.
- The internal urethral sphincter is under involuntary control, while the external urethral sphincter is under voluntary control.
Which of the following renal structures is most susceptible to ischemia?
- Loop of henle
- There are multiple factors that contribute to the loop of Henle’s susceptibility to ischemia.
-Active transport of sodium occurs at the ascending limb of the loop of Henle - this requires a great deal of energy and oxygen. During periods of hypoxia, the oxygen demand of the loop of Henle may be greater than the oxygen supply, leading to ischemia.
-In addition, structures deeper in the renal medulla are more susceptible to ischemia than the renal cortex because the medullary blood flow is kept low to facilitate reabsorption. The loop of Henle is located within the renal medulla.
Wilms tumor and Renal Cell Carcinoma
- Wilms’ tumor is the most common cause of renal malignancy in infants; it results from the malignant overgrowth of the metanephric blastema.
- Malignant overgrowth of the metanephric mesenchyme/blastema in childhood is categorized as a Wilms’ tumor, which is the most common cause of renal malignancy in infants.
- Renal cell carcinoma is the most common cause of renal malignancy in adults; it originates from the proximal convoluted tubule.
Horseshoe kidney
-A horseshoe kidney is associated with the fusion of the inferior poles of the kidney leading to impaired renal ascension and is associated with genetic aneuploidies.
-A horseshoe kidney can be asymptomatic for life.
Multicycstic dysplastic kidney and renal agenesis
Renal agenesis describes the complete absence of one or both kidneys due to failure of the ureteric bud to develop - in most cases, the ureter will also be absent so a duplex collecting system would be highly unlikely.
Active vs passive transport
- Active transport requires ATP
- Passive transport does not require ATP. Make use of electrochemical gradients
- co transport - molecules come in together
- counter transport - one molecule in and another molecule out
Proximal convoluted tubule transporters
- reabsorb 100% glucose by Na+/glucose cotransporter. SGLT2
-reabsorb H2CO3 (bicarbonate). H2CO3 is broken down into H20 and CO2 by carbonic anhydrase in the lumen of the PCT. They cross into the cell.
- In the cell carbonic anhydrase joins H2O and CO2 back together into H2CO3.
- H2CO3 can cross into the peritububular capillaries using a Na+/H2CO3 cotransporter.
- PCT reabsorbs 60-70% off Na+, Cl-, PO43+, K+, HCO3-
- Parathyroid hormone: phosphate trashing hormone. Decreases the function of the sodium phosphate transporter. So less phosphate is reabsorbed back into the blood and more is got rid of in urine
Angiotensin 2
- Angiotensin II increases sodium reabsorption and decreases H+ reabsorption.
- Angiotensin II increases the action of the Na+/H+ antiporter, thereby increasing sodium reabsorption and decreasing H+ reabsorption
- Na+/H+ countertrasnporter
Fanconi syndrome
Fanconi syndrome is a defect in the proximal convoluted tubule (PCT) that impairs reabsorption. Large amounts of potassium excretion will result in hypokalemia.