I just finished a very educationally rewarding rotation in Nephrology and I do not want to lose the ground I have gained. Nephrology is, in my opinion, a challenging field. I encountered on this rotation many topics that I will need to know well to be a good IM intern (electrolyte disturbances, acidosis, alkalosis, acute kidney injury and chronic kidney disease) as well as some uncommon pathology that the Nephrologist will most likely manage.
02/05/2014
Briefly, a 35 yo female with no sig past medical hx and on no medications came in for an office visit after her urinalysis showed that she had proteinura > 4,028 mg. Labs were negative for secondary causes of nephropathy (ANA, anti-dsDNA, Hepatitis panel, SPEP, UPEP, HIV, ANCAS). Pt's only complaint was mild, generalized swelling in lower legs. Kidney Biopsy was performed and showed fusion of the podocytes on EM. Light microscopy and Immunofluorescence were completely normal.
Minimal Change Disease
Pathology-Simply, the disruption of the negatively charged membrane in the glomerulus allows increased permeability to anions (proteins!!) which then spill out in the urine thus causing proteinuria. Disruption of the podocytes will compromise the membrane permeability.
Epidemiology-It is the most common cause of Nephrotic Syndrome in children and the cause of primary nephropathy 10-20% of the time in adults. 30% of patients will see resolution of the disease with or without treatment. The rest will have relapsing/remitting cases.
Causes-Mostly Idiopathic and was the case with our patient. It may be secondary to NSAID use, leukemia, thymoma, malignancy of the kidney, pancreas or duodenum and Hodgkin's lymphoma.
Diagnosis-Need a kidney biopsy. The Immunofluorescence and Light Microscopy will be normal but you will see effacement, fusion or flattening of the podocytes on Electron Microscopy (our pt had fusion).
Clinical Course-pt will present with sudden proteinuria and may have edema (our pt was complaining of lower leg swelling). You may see acute kidney injury in pts with previously compromised kidneys (atherosclerosis). It is uncommon for the patient to progress to uncontrolled hypertension or end stage renal disease.
Treatment-Steroids are the cornerstone of therapy as well as reducing the blood pressure in the glomerulus. You treat with 60mg of Prednisone every other day for 4 weeks. Then taper down to 40mg every other day for 4 weeks. About 10 % of patients don't respond to steroid treatment and then you progress to cytotoxic drugs such as cyclosporin, mycophenolate etc. Decreasing the blood pressure with an ACE inhibitor or and ARB is a common addition to therapy. In our case, the patient's repeat UA showed an improvement of the proteinuria and we had her on an ACE inhibitor, lisinopril. After weighing the pros and cons of starting steroid therapy, she decided to wait to and see if her proteinuria continued to improve. She would follow up in 3 months and call if she was noticing any worsening of symptoms (rapid weight gain from swelling, high blood pressure readings).
Up until now, I had thought that I would probably not see a case of Minimal Change since it was more common in Pediatrics. Surprise!
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