CASE 24 (February 2008)
The patient is an 8-year-old girl with one year of evolution of nephrotic syndrome, with intermittent remision and exacerbation episodes, and apparent "partial response" to steroids. She has not received other immunosuppressants. The patient has presented, in addition, several episodes of gross hematuria lasting several days. Urinalysis when she did not have gross hematuria revealed microscopic hematuria. Proteinuria: 5.5 g/24 hours, approximately 220 mg/m2/h. Mild hypercholesterolemia. Hypoalbuminemia.
The patient presents mild abdominal pain, diffuse, several days ago. No gastrointestinal symptoms. She does not manifest upper respiratory tract infections or fever in the last month, and she does not have skin lesions.
On physical examination she presents generalized edema. No hypertension or abnormalities of the skin or mucous membranes. There are not visceral size increase nor adenomegalies. There are not other alterations.
What is your clinical diagnosis?
With the diagnosis of nephrotic syndrome and intermittent gross hematuria a renal biopsy was undertaken.
See the images.
Figure 1. There is mild to moderte mesangial hypercellularity in most glomeruli (H&E, X400).
Figure 2. Mesangial proliferation is more evident in this glomerulus (H&E, X400).
Figure 3. In several glomeruli there is also podocyte hypertrophy (Masson's trichrome, X400).
Figure 4. PAS stain, X400.
Figure 5. Methenamine-silver stain, X400.
Figure 6. Diffuse immunostaining for IgA. In the glomerulus of the left, deposits are predominantly mesangial, but in the glomerulus of the right these are mesangial and granular in capillary walls. Immunofluorescence using antiserum to IgA, X400.
Figure 7. Immunofluorescence using antiserum to IgG, X400 (left) and IgM, X400 (right). There are diffuse deposits of IgG in capillary walls and in mesangium. IgM staining is mild and focal (traces).
Figure 8. Immunofluorescence using antiserum to C3, X400 (left) and C1q, X400 (right). There are mild C3 immunostaining and focal and segmental traces of C1q.
Figure 9. Immunofluorescence using antiserum to kappa light chains, X400 (left) and lambda light chains, X400 (right). There are diffuse granular capillary walls and mesangial deposits.
What is your diagnosis?