Pathology 6000 Laboratory - Department of Pathology, UCD-School of Medicine

RENAL  UNIT

CASE  3

Clinical History:

A 45-year-old male complained of a constant nagging ache in both right and left flanks, sometimes associated with generalized abdominal pain.  It was not related to eating, urination or bowel movements.  There was no regular variation with time of day or postural position.  He obtained some relief from aspirin.  Past history revealed that he had had an occasional episode of hematuria and his BP had been elevated when checked by his daughter.

Physical examination:  BP 150/100 mm Hg. From the location of the apical impulse and the left border of cardiac dullness to percussion, his heart seemed to be enlarged.  No murmurs were heard. Palpation of the abdomen indicated a renal mass or enlargement of the left kidney.  The examiner thought that there might be a similar mass on the right side but he could not be sure.  The rest of the examination was normal.

Urinalysis: Sp. Gr. 1.008, pH 6.0, sugar 0, protein 1+.  Sediment contained many RBC and a few WBC.  BUN was 52 mg/dl. Creatinine was 4.3 mg/dl.

Questions:

1. If the masses were in fact bilateral, what causes of renal mass or renal enlargement could be excluded?

2. What procedure would you recommend to clarify the diagnosis?

3. Figure 1 shows the results of a study done to determine the diagnosis. How do you interpret it?

4. What other type of study could be done?

5. Over the next 10 years the patient developed chronic renal failure and increasing abdominal discomfort. What other complications might you anticipate? Would you be aggressive in treating the hypertension? Why?

6. The patient then developed uremia and had to be put on the artificial kidney.  Nephrectomy was done to relieve the pain and prepare the abdomen for possible kidney transplantation. The two kidneys were similar, Figure 2 and Figure 3.  What is the diagnosis?  Figure 4 shows an infantile form of a disease with the same name. How do the two diseases differ?

7. What are the genetics of this man's disease? How would you deal with children of this patient who are asymptomatic?

8. Years ago the lesions shown in Figure 2 and Figure 3 were surgically punctured and decompressed in order to relieve pain and discomfort.  Can you see anything wrong with this approach?

9. Summarize this disease with respect to:

A. Manifestations related to the ongoing disease in the kidney.

B. Whether or not it progresses to renal failure.

Answers

  

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