2017 Annual Meeting

Evening Specialty Conference
Renal Pathology

Great Kidney Cases with Key Teaching Points

March 8 2017, 7:30pm to 9:30pm

Moderators:
Vanesa Bijol, MD and Tibor Nadasdy, MD
Panelists:
Priya Mariam Alexander, MD - Christopher Larsen, MD - Cynthia C. Nast, MD - Harsharan K. Singh, MD

Pre-Meeting Materials

Case 1

Faculty:
Priya Mariam Alexander, MD

Clinical History:

An elderly Caucasian man in his late sixties presented with acute renal failure, hematuria and proteinuria. His medical history was significant for arthralgias, cold-induced cutaneous rashes and ulcerations, hypertension, gout and obstructive sleep apnea. He had a remote smoking history. There was no family history of kidney disease. His medications included, among others, allopurinol, calcitriol and furosemide.

Pertinent Laboratory Data:

  • Laboratory investigations include:
  • A creatinine of 1.8 mg/dl
  • Serum albumin of 3.8 g/dL
  • Hemoglobin of 11g/dL

Complement levels were normal and ANA, ANCA and Anti-GBM titers were negative. Viral serologies for hepatitis and HIV were negative. A monoclonal protein, IgG kappa, was detected in the serum. Cryoglobulins were absent. A renal biopsy was performed. The PAS stained slide is scanned. Immunofluorescence studies were negative. A few ultrastructural images are shown. What is your diagnosis?

Morphological Findings (click to enlarge):

Figure 1EM1
Figure 2EM2

Case 2

Faculty:
Christopher Larsen, MD

Clinical History:

The patient is a 12-year-old African American male with nephrotic range proteinuria (5 g by urine protein to creatinine ratio). He presented to clinical attention after he was found to be hypertensive on a physical exam for school sports. He reports feeling well and denies rash, joint pain, abdominal pain, and SOB. Past medical history includes ADHD but is otherwise unremarkable. He currently takes no medications. Family history includes multiple family members with hypertension and a paternal uncle on dialysis. His blood pressure is elevated on physical examination at 152/95 and he is slightly obese (BMI 26.4). There is no evidence of rash or edema.

Pertinent Laboratory Data:

  • Serum creatinine of 0.6 mg/dL
  • Serum albumin 3.3 g/dL
  • Weakly positive ANA antibody (1:40)
  • ANCA, dsDNA, Smith negative C3/C4 normal

Morphological Findings (click to enlarge):

Figure 1
Figure 2
Figure 3
Figure 4
Figure 5
Figure 6
Figure 7 IgG
Figure 8
Figure 9

Case 3

Faculty:
Cynthia C. Nast, MD

Clinical History:

The patient is a 76 year old man with hypertension, diet controlled diabetes and prostatic enlargement. He presented with shortness of breath and was found to be thrombocytopenic without anemia. Bone marrow exam revealed 40% polytypic plasma cells without evidence of myeloma. Serum levels of IgG, IgA, IgM, and both light chains were elevated with a normal kappa:lambda ratio. The serum creatinine was 1.36 mg/dL which, after 3 weeks of prednisone treatment, declined to 1.04 mg/dL with the platelet count and immunoglobulin levels also improved. Two weeks later he had a dental cleaning, and two days after the cleaning developed a pruritic erythematous rash on both thighs, fever, extensive lymphadenopathy, splenomegaly and oligoanuric acute renal failure. He was taking no new medications. (His daughter reported having a similar illness several years previously with lower extremity rash and renal failure requiring dialysis, which resolved over 6 months with steroid treatment.) Over the next four days the patient worsened becoming dialysis dependent and a renal biopsy was performed.

Pertinent Laboratory Data:

  • Creatinine 5.9 mg/dL
  • BUN 72 mg/dL
  • Albumin 2.5 g/dL
  • Glucose 116 mg/dL
  • Calcium 10.6 mg/dL
  • Total bilirubin 0.4 mg/dL
  • Total protein 9.8 g/dL
  • IgG 4008 mg/dL
  • IgA 1138 mg/dL
  • IgM 364 mg/dL
  • AST and ALT normal Hemoglobin 11.0 g/dL
  • Platelets 57,000
  • WBC 11.4 x 103/mcl
  • WBC differential showed decreased lymphocytes and monocytes, and increased neutrophils and plasmacytes
  • Urinalysis - 2+ protein, 1+ blood, 2+ bilirubin, no cells

Morphological Findings (click to enlarge):

Figure 1
Figure 2
Figure 3
Figure 4
Figure 5IgA
Figure 6
Figure 7
Figure 8
Figure 9

Case 4

Faculty:
Harsharan K. Singh, MD

Clinical History:

A 5-year old severely growth retarded child with end-stage renal disease underwent renal transplantation from a cadaveric donor source (13 year old "healthy" boy with self-inflicted gun shot wound; donor kidney 11 cm in length) with intra-abdominal graft placement and anastomosis to the aorta. During year one post grafting under standard triple immunosuppression (mycophenolate mofetil, tacrolimus and prednisone) persistent nephrotic range proteinuria developed in the setting of stable function (serum creatinine: 0.2-0.3 mg/dl, no donor specific antibodies, inactive urine sediment). Post transplantation course: Zero-hour implantation biopsy with moderate acute tubular injury; IF & EM normal Immunosuppression with Cellcept, Tacrolimus & steroids (pat. has been compliant) Immediate and stable renal function with S-Cr 0.2-0.3 mg/dl over entire follow-up period Nephrotic range proteinuria (UP/C ratio up to 8) that developed during 1st year and persisted thereafter Microscopic hematuria A renal biopsy was performed.

Morphological Findings (click to enlarge):

Figure 1 PAS-10x
Figure 2 PAS-20x
Figure 3 Trichrome-20x
Figure 4 C4dIF-10x
Figure 5 EM glomerular basement membranes low power
Figure 6 EM glomerular basement membrane medium power

Meetings

home-circle-inset-1
view more