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DMD Nephrology Case 2-Nephrotic Syndrome

 

A 40-year-old man has come to your office with the complaint of swelling of his body over the past several months.  He states that approximately 3 months prior to admission he had fallen off his bicycle and bruised his right leg.  He noted swelling in the leg at that time and soon thereafter noted both his legs had become swollen.  Over the past several months he noticed this swelling had increased over his entire body. He reports a 20-lb. weight gain over that period of time.

 

Discussion points:

  • What are the probable causes of total body edema in this patient? (Include a discussion of secondary hyperaldosterone states including: congestive heart failure, cirrhosis, nephrosis, and malnutrition including malabsorption. Additionally, severe hypothyroidism with myxedema or a generalized allergic reaction may give the appearance of body swelling.)
  • Differentiate the between causes of unilateral leg edema and total body swelling.
  • What other historical information will be important in assessing this patient’s problem?

 

He has no history of heart disease and denies PND, DOE, or orthopnea.  He has no history of liver disease and denies a history of jaundice, dark urine or alcohol use.  He does not know of any exposure to any hepatitis and has no history of IV drug use.  He was never told of any problems with his kidney as an adult or a child. His urinary frequency is unchanged over the past two months.

 

Discussion points:

  • Given this patient’s history, is the likelihood of congestive heart failure or cirrhosis likely?
  • What physical exam points should be focused on in this patient with generalized edema? (Signs of heart failure, hepatic dysfunction, hypothyroidism, malnutrition, or nephrotic syndrome including hypertension).

 

On physical exam, he appears grossly edematous and puffy.  His is BP-145/98, P-92, RR-16, and T-98.6F. The patient’s skin shows no signs of jaundice or telangectasias.  He has no signs of palmar errythema.  His face shows swollen eyelids but sclera are not icteric.  Examination of the neck shows no signs of jugular venous distention and no thyromegaly. His lung exams are clear to auscultation and percussion with the exception of decreased breath sounds and dullness to percussion in the bases.  Cardiac exam revealed a non-displaced PMI. Normal S1 and S2 without murmurs, rubs, S3 or S4. Abdominal exams showed the liver to be approximately 9 cm in size. No splenomegaly was appreciated. There were findings of shifting dullness to percussion of the abdomen.  In the periphery the patient had 4+ pitting edema to the level of the sacrum and throughout both legs. The patient had no signs of gynecomastia, testicular atrophy or parotid swelling. The patient’s neurological exam was normal.

 

Discussion points:

  • Given this examination, is it likely that the patient has congestive heart failure as the cause of his generalized edema?
  • Are there signs of cirrhosis present on this examination?
  • What aspect of this examination would suggest this patient has nephrotic syndrome. (Elevated blood pressure, pleural effusions, ascites, and swelling of the eyelids are frequently seen in patients with nephrotic syndrome).
  • What laboratory tests would you order to confirm your diagnosis?

 

A urinalysis revealed 4+ proteinuria with a microscopic exam that revealed several waxy casts, oval fat bodies, no cellular casts, 1 RBC and 1 WBC per high-powered field.  Electrolytes included a BUN of 20 mg/dl, a creatinine of 1.3 mg/dl, other electrolytes, glucose, calcium and phosphorus were normal. A 24-hour urine collection showed 12 grams of protein and a creatinine clearance of 72 ml/min.  LFTs were normal, as was a prothrombin time. The patient’s albumin was 1.9 gr/dl and serum cholesterol was 670 mg/ml. Chest x-rays revealed bilateral pleural effusions without signs of pulmonary venous engorgement or abnormalities in the cardiac silhouette.

Discussion points:

  • Given the above information, what is the diagnosis of this patient?
  • Construct and differential diagnosis of causes of nephrotic syndrome?
  • What diagnostic studies would you send off looking for systemic diseases as a cause of nephrotic syndrome?
  • What would your initial management of this patient be before the results of other tests become available? (Use a salt restricted diet and judicial use of diuretic therapy.)

 

Laboratory tests were sent for antinuclear antibodies, serum complement, and fasting blood sugar which all were normal. A VDRL and Hepatitis B and C serologies were also negative.

 

Discussion point:

  • Would you recommend a kidney biopsy for this patient and why?

 

The patient is informed of his diagnosis of nephrotic syndrome and the potential benefits and risk of a kidney biopsy in looking for the ideology of this problem. He consented and the procedure is done without complications. The results of the biopsy showed evidence of membranous Glomerulonephritis.

 

Discussion points:

  • How common is membranous GN in adult patients with nephrotic syndrome?
  • Can you determine the etiology of the patient’s GN based on the biopsy?
  • What are likely secondary causes of membranous GN in adults? (Include parasitic diseases, viral hepatitis, solid tumors, and certain drugs including gold, penicillamine, NSAIDs.)
  • What is the prognosis for this patient developing end stage renal disease? (Approximately 30% of patients experience complete remission of this disease without therapy. Other patients maintain a stable renal function of 15 years or more while others rapidly progress into chronic renal failure.)
  • Are there predictors regarding which course this patient will take? (Heavy proteinuria for greater than 6 months often portends a poor prognosis.)
  • How would you treat this patient? (Use of corticosteroid and other immunosuppressive agents have been used in improving patients’ initial outcomes.

 

The patient is informed of his prognosis and the diagnosis of membranous GN. He is started on a course of corticosteroid therapy in conjunction with cyclophosphamide. He will be followed up closely for side affects of the medication as well as the effect on the patient’s renal function as well as level or proteinuria.

 

 

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