Introduction:
Gordon syndrome is a rare inherited monogenic form of hypertension, which is associated with hyperkalaemia and metabolic acidosis. Since the recognition of this predominantly autosomal dominant condition in the 1960s, the study of families with Gordon syndrome has revealed four genes WNK1, WNK4, KLHL3, and CUL3.Pregnancy in women with Gordon's syndrome appears to be associated with a significant risk of adverse pregnancy outcomes, particularly where there is maternal hypertension preconception. No pregnancy registry exists for Gordon's syndrome. The available data is limited to case reports and small case series and may be affected by bias. However the medication US FDA pregnancy category is not assigned.
Methods:
21-year-old female patient known to have renal tubular acidosis type IV due to Pseudohypoaldosteronism type IID "Gordon syndrome" confirmed by genetic studies (KLHL3- homozygous variant) and chronic hyperkalemia. There is a Strong family history of PHA2 and a similar gene mutation (KLHL3- homozygous variant) with her brother and cousin. Her home medications include hydrochlorothiazide and sodium bicarbonate. She presented to the prenatal clinic for follow-up. She was para 1 +0 at 31 weeks. She had lower limb swelling, and her BP was elevated at 140/100 mmHg. Her K level was 6.8 mmol/L, then dropped to 5.7 mmol/L. She was started on sodium zirconium 10 mg three times a day and a low K diet 24 hours prior to her delivery. Her antenatal ultrasound showed abnormal Umbilical Artery Doppler: absent end diastolic flow, she was given dexamethasone, iv labetalol, and mg sulfate, and she had brisk reflexes. Emergency C/S was performed, and the baby cried immediately with a weight of 1090 g.
Results:
She was started on sodium zirconium 10 mg three times a day and a low K diet 24 hours prior to her delivery. Her antenatal ultrasound showed abnormal Umbilical Artery Doppler: absent end diastolic flow, she was given dexamethasone, iv labetalol, and mg sulfate, and she had brisk reflexes. Emergency C/S was performed, and the baby cried immediately with a weight of 1090 g.
Conclusions:
Sodium zirconium is not systematically absorbed following oral administration, therefore it is not expected to cause fetal exposure. Animal studies did not indicate direct or indirect harmful effects to reproductive toxicity. Rare conditions like Gordon syndrome are difficult to manage during pregnancy. The use of new medications like Sodium zirconium is assumed safe during pregnancy as it is not systematically absorbed however in such a complex case relationship between medication rare risk of edema and patient symptoms cannot be completely excluded.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.