ESPN 50th Annual Meeting

ESPN 2017


 
Unexpectedly hypothyroidism post bilateral nephrectomies in a patient with congenital nephrotic syndrome
Rebecca A Dalrymple 1 Heather Maxwell 1 M Guftar Shaikh 1

1- Royal Hospital for Children, 1345 Govan Road, Glasgow, UK
 
Introduction:

To describe the case of an infant with congenital nephrotic syndrome who was unexpectedly found to have congenital hypothyroidism after thyroxine supplements were stopped post bilateral nephrectomy.

Material and methods:

Case report

Results:

 

An infant with Finnish Type congenital nephrotic syndrome (CNS) was started on thyroxine after having biochemical hypothyroidism detected following routine investigations for CNS. His CNS was managed conservatively with regular albumin infusions and IV furosemide to reduce the oedema, anticoagulation, immunoglobin infusions, antibiotic prophylaxis, ace inhibitors and later indomethocin to reduce his GFR. He went on to have bilateral nephrectomise by 2 years and 10 months of age.

His hypothyroidism was expected to resolve following the reduction in urinary leak of thyroid binding proteins after bilateral nephrectomise, however he became severely biochemically hypothyroid (TSH >500mU/L, fT4 <5.1 pmol/L) after stopping the levothyroxine and he was later confirmed to have thyroid dysgenesis. Patients with nephrotic syndrome (NS) can become hypothyroid due to urinary losses of thyroid binding proteins and our patient was mistakenly thought to be hypothyroid for this reason.

He was quickly restarted on thyroxine and his thyroid function tests stabilised. At the age of 4 years he went on to have a renal transplantation and remains well on his thyroxine replacement.

 

Conclusions:

This case illustrates the importance of monitoring thyroid function post nephrectomies to ensure there are no other unusual causes for their hypothyroidism.