The Kidneys

~Sickle Cell Kidneys: Complications, Treatment and Awareness~
Author: Kara Martina, B. Sc. Biopsychology
            Those with Sickle Cell Disease and Trait have a different physiology than the general population. Having to be keenly aware of unique problems, this article aims to cover the common Sickle Cell complications and lingo of the kidney: Sickle Cell Nephrology.  
             Routine Doctor check-ups prevent long-term damage and catch kidney problems before they escalate. Responsible for filtering the waste products from the blood, the kidneys need more than a basal level of functioning to keep the human body healthy. When they don’t work properly, the ramifications can be detrimental or life changing, with treatment ranging from transfusions to dialysis. Those with SCD or SCT fall under a category of at-risk individuals for kidney diseases. Nephrology knowledge should be heeded and spread:   

Sickle Cell Nephrology: A Summary

Glomerulonephritis               

• Glomerular Enlargement: In the Kidney, the tiny ball of blood vessels that filtrates the urine is called the Glomerular. When this enlarges, chronic or acute glomerulonephritis may occur.              
  
• Perihilar Focal Segmental Glomeruloscleosis (FSGS): When only parts or a localized few glomerula are scarred or damaged. 

Symptoms: Often asymptomatic, but Nephrotic and Nephritoc symptoms can occur: proteinuria, hematuria.
Treatments: Transfusions, Angiotensin-converting enzyme (ACE) inhibitor medicines.

Hematuria: Blood in the urine.
Symptoms: Pink, reddish urine, painless.
Treatment: Bed rest and maintenance of high urine flow rate, iron replacement and/or blood transfusions.

Hemosiderosis: Iron overload in the kidneys.
Symptoms: Joint pain, fatigue, unexplained weight loss, abdominal pain, abnormal bronze or gray skin color.
Treatment: Limiting blood transfusions and starting iron chelating therapy. See your doctor about chelating drugs.

Microalbuminuria/proteinuria: An increase in urinary excretion of the protein albumin.  
Symptoms: Sometimes asymptomatic. Detected by measuring aluminum-creatinine ratio (ACR) in a spot urine sample where 30-300mg of albumin will be detected. May cause urine to look foamy, swelling of the hands and feet; edema.
Treatment: angiotension-converting enzyme (ACE) inhibitors or angiotension receptor blockers (ARBs).

Nocturia: low bladder capacity and production of a large volume of urine when you sleep.
Symptoms: Having to wake up during the night to urinate.
Treatment: See your physician. Treatments will include interventions such as restricting fluid in the evening, elevating legs, wearing compression stockings. Medications include: Desmopressin (DDAVP), Bumetanide (Bumex), Furosemide (Lasix), Anticholinergic drugs.   

Papillary Necrosis: Part or all of the kidney’s papillae die. The renal papillae are the areas where the collecting ducts meet the kidney and the urine flows into the ureters.
Symptoms: Back pain, flank pain, bloody urine, cloudy urine, dark urine, tissues in the urine, chills, fever.
Treatment: Exchange transfusions, replacing losses, maintaining hydration, alkalinizing the urine.

Polyuria: Large production of urine.
Symptoms: Increase in urinary frequency, greater than 3 L/day.
Treatment: Admission to hospital, fluid balance and electrolyte disturbance needs to be corrected.

Renal disease/kidney disease/renal failure: kidneys fail to filter waste adequately from blood.
Symptoms: Swelling, lack of urine production, fluid buildup; edema 
Treatment: Dialysis, kidney transplant.

Renal Infarcts: The death of parts of the kidney due to lack of oxygen usually caused by a lack of blood supply.  
Symptoms: Nausea, vomiting, abdominal pain, flank pain, fever.
Treatment: Support Management: dialysis.

Renal Medullary Carcinoma: A tumor in the collecting duct of the nephron. Most common only in those with Sickle Cell Trait.  
Symptoms: 60% experience blood in the urine (hematuria), 50% have abdominal pain and 25% have significant weight loss.
Treatments: When patients present a localized case, a surgical resection can be curative. Unfortunately, most cases are silent, and the cancer spreads past the point of curative care.

Other Words you may hear: 
Hematocrit: A blood test that measures the amount of red blood cells in the blood, (% of whole blood made of red blood cells).
Ischemia: Restriction of blood supply to the tissues. Ischemia of the kidney leads to necrosis, infarction.  
Isosthenuria: Non-concentrated urine, having the same osmolarity as plasma. Isosthenuria is usually a common sign of early renal failure.   

All information has been summarized from Medscape, the American Society of Hematology, and the Mayo Clinic.