Kidney

Clinical Correlation. 30th September 2013.

Normal glomerulus (arrow)

Case -1
A six year old boy is brought by his parents with a c/o severe abdominal pain, joint pains and purpuric lesions on the extensor surfaces of arms and legs and on the buttocks. He also complains of passing cola-colored urine. A correct statement regarding this disorder is :
a) Presence of subepithelial deposits. Memb. nephropathy b) Basement-membrane splitting -MPGN c) Immune complex vasculitis. d) Irregular thickening of the GBMDiabetic glomerulonephropathy e) Uniform, diffuse thickening of the GBM- Membranous nephropathy Here is the microscopy:

HSP Clinical photograph-purpuric rashes.

Answer : C : Immune complex vasculitis- HSP. *See p 666

Case-2
A 42 year old male presents with recurrent episodes of purulent sinusitis and two recent attacks of necrotizing pneumonitis. He also has ulcerations of the nasal mucosa that are resistant to healing. Investigations : increased BUN and creatinine . A renal biopsy revealed a necrotizing glomerulonephritis . A correct statement regarding this : a. Ig G and C3 immune deposits along the GBM. b. Paucity of immune complex deposits. c. IgA deposits in the mesangium d. 3 chain of COL4 mutation. e. Deposition of amorphous eosinophilic Congo red positive material in the glomerulus.

Case-2
A 42 year old male presents with recurrent episodes of purulent sinusitis and two recent attacks of necrotizing pneumonitis. He also has ulcerations of the nasal mucosa that are resistant to healing. Investigations : increased BUN and creatinine . A renal biopsy revealed a necrotizing glomerulonephritis . A correct statement regarding this : a. b. c. d. e. Ig G and C3 immune deposits along the GBM. DPGN or membranous nephropathy Paucity of immune complex deposits. IgA deposits in the mesangium Bergers 3 chain of COL4 mutation. Alport Deposition of amorphous eosinophilic Congo red positive material in the glomerulus. Amyloidosis

Answer-B Pauci immune Wegeners Granulomatosis *See p 516.

Wegeners Clinical picture

Massive crusting and inflammation of the nasal mucosa.

Saddle nose

Wegener.
Necrotizing GN- early lesion

Lung lesion

Later-Crescentic GN

Vasculitis.

Case-3
A 8 year old male presents with swelling of his face and also around the ankles. His mother says he had a mild respiratory infection, a few days ago. PE : reveals peripheral edema. Urinalysis : 3 + proteinuria. No red cell casts seen 24 hour protein : 4.0 g protein. A correct statement regarding this disorder is :

a. Non selective proteinuriaFSGS b. Hyperlipidemia c. No change in cholesterol levels. d. Increase in polyanionic content of GBM. e. Hypercellular glomeruli with PMN infiltration on LM Answer : B

Nephrotic syndrome.

MCD
Minimal Change Disease: Features: Absence of immune complexes, but strong evidence of an immunologic basis. On LM and IFM = No changes EM : diffuse loss or fusion of foot processes Dramatic response to prednisone therapy Selective proteinuria

Case - 4
A 68 year-old male who was a heavy smoker c/o fullness in the abdomen and hematuria. Investigations show : Polycythemia and hypercalcemia. Urine : RBC ++ Cytology of the sediment shows suspicious cells. Abdominal CT : mass in the upper pole of kidney extending medially to the hilum. a. b. Eosinophilic fractured casts in the tubules with giant cell reaction. Tumor composed of clear cells with a propensity to invade the renal vein . Small blue, round cell tumor showing triphasic pattern. wilms Asymmetric scarred kidneys, with blunting of calyces. chronic pyelonephritis High incidence in patients with the WAGR syndrome.- wilms

c.
d. e.

A correct statement regarding this lesion is :

Diagnosis-RCC
Clear cells

Case-5

This is the clinical photograph of a 2 year old child brought to the pediatrician by his parents. Surgery was done and the gross and microscopic pictures are shown in the next slide.

Describe the morphology seen here.

What is your diagnosis?

Wilms tumor/Nephroblastoma

Thank you !