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Congenital Nephrotic Syndrome


Guest marcydf

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Guest marcydf

Hello,

I need your help in a case of Congenital Nephrotic Syndrome. The baby, female, is 2 months. We are waiting for genetic diagnosis, but we suspect a finnish form.

We are administrating a very high dose of albumin (2-3 g/kg/die divided in three doses) by a Broviac CVC. We are administrating also Tiroxine, Furosemide, a low dose of captopril (0,015 mg/kg three times per day) and indomethacin (0,4 mg/kg twice per day). We didn't obtain an improvement about proteinuria with these two drugs. We are feeding her with fortified human milk

We would offer a better quality of life to the baby and her parents, so I was thinking to try to infuse a load dose of albumin nightly (3 g/kg of albumin) every three days, hoping to obtain an adequate albuminemia without a daily infusion.

What do you think about optimal management? More doses of albumine per day or less? High or low rate of infusion? How much can I increase dosage of Indomethacin and Captopril?

I thank for any help

Marcello De Filippo

Neonatologist

Grosseto, Italy

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