Effect of Galactose on Permeblity Factor in Patients With FSGS and CKD Stage 5

Patients with resistant FSGS have persistent proteinuria and a high risk of progression to
end stage kidney disease (ESKD). A circulating factor that increases glomerular permeability
to albumin (Palb) has been detected in over 50% of these patients. While the molecular
identity of the factor has not been fully established, in vitro studies and limited clinical
experience suggest that galactose can reduce the level of the FSGS permeability factor.

This study is a pilot study to determine if oral administration of galactose can lower the
circulating level of the FSGS permeability factor.

Five patients with biopsy proven primary FSGS - native or transplant kidney, receiving
plasmapheresis - who are resistant to steroids and one other immunosuppressive agents will
be studied.

The only eligibility factor is presence of the FSGS permeability factor.

The experimental intervention is administration of galactose, orally, 0.2 g/kg body
weight/dose twice daily. the duration of treatment will be 28 days,.

Patients will be seen on days 0, 14, and 28 of treatment. They will be seen at week 8, 16
and 24 after discontinuation of the galactose.

Physical examination and routine laboratory tests (SMAC, CBC, urine protein:creatinine ratio
in an early morning sample) will be done at each visit. The FSGS permeability factor will be
assayed at days 0 and 28 of treatment and 6 months after discontinuation of the galactose.
The permeability factor will be tested in the laboratory of Virginia Savin MD (Medical
college of Wisconsin) using previously described methods.

All other treatments will be unchanged during the 28 day oral galactose Treatment Period.

The study will be analyzed based on the number of patients in whom the FSGS permeability
factor is lowered to normal levels.

Inclusion Criteria:

- Primary FSGS

- CKD Stage 5

- Resistance to steroids and another immunosuppressive medication

Exclusion Criteria:

- Secondary FSGS