An 18-year old white male, a freshman in college, presents with an episode of gross hematuria. He is seen at his college health clinic and a urine culture is taken and he is treated empirically with levofloxacin. 2 weeks later he develops a rash on the dorsal surfaces of his arms and in the pre-tibial area of his lower extremities. He is sent to a local dermatology clinic where a skin biopsy is taken. The biopsy shows a leukocytoclastic vasculitis. No immunofluorescence is available. 1 week later he has another bout of gross hematuria. This time he is referred to a nephrologist. A urine sediment examination reveals dysmorphic red cells and 1 red cell cast. His BUN is 28 mg/dL and his serum creatinine 1.2 mg/dL. His complements are normal and his ANA is negative. A renal biopsy is performed. The EM is shown below. What does the EM show and what is the diagnosis?
The answer to the image quiz of 1/15/12 is: classical Alport's syndrome due to X-linked hereditary nephritis. There is prominent lamination and fraying of the GBM. The lamina densa as such has disappeared. Notice also the distortion and "fusion" of foot processes, shown on top. Lumen of the capillary is on the bottom.
This patient has a family history of hematuria. The urine shows dysmorphic red cells and labs are notable for a BUN of 38 mg/dL and a serum creatinine of 2.1 mg/dL. The pic shows an electron micrograph of the patient's renal biospy. What does the picture show and what is the likely cause for the hematuria.
More information on Alport's is provided here. Other features on renal biopsy of a patient with classic Alport's are shown below:
The answer to the image quiz of 1/15/12 is: classical Alport's syndrome due to X-linked hereditary nephritis. There is prominent lamination and fraying of the GBM. The lamina densa as such has disappeared. Notice also the distortion and "fusion" of foot processes, shown on top. Lumen of the capillary is on the bottom.
This patient has a family history of hematuria. The urine shows dysmorphic red cells and labs are notable for a BUN of 38 mg/dL and a serum creatinine of 2.1 mg/dL. The pic shows an electron micrograph of the patient's renal biospy. What does the picture show and what is the likely cause for the hematuria.
More information on Alport's is provided here. Other features on renal biopsy of a patient with classic Alport's are shown below:
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Classical Alport's syndrome due to X-linked hereditary nephritis.
There is focal and segmental sclerosis of this glomerulus
This is a 22-year-old male patient with significant
proteinuria and moderate renal insufficiency. (PAS)
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| Classical Alport's syndrome due to X-linked hereditary nephritis. Interstitial foam cells are often a clue for the diagnosis in patients without hyperlipidemia. |
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Alport syndrome. Details of the peripheral
capillary wall.
Notice the extreme fragmentation of the lamina densa,
resulting in a characteristic “basket weave” pattern.
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Image Quiz January 20
ReplyDeleteEM micrograph shows mesangial electron dense deposits. In view of clinical history and results of skin biopsy, the patient likely suffers from Henoch-Schonlein Purpura (HSP) with renal involvement (HSP-nephritis)