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ALPORT SYNDROME: A CHALLENGING CAUSE OF NEPHROTIC SYNDROME DURING LATE PREGNANCY
Abstract
BACKGROUND: In pregnancy, proteinuria can be commonly increased and varies from non-nephrotic range proteinuria to nephrotic syndrome. Diagnosis and proper management are very important for both maternal and fetal health. Little is known about pregnancy in women with Alport Syndrome (AS). AS, a hereditary disorder X-linked (85%) or autosomal recessive (15%), associated with mutations in type IV collagen, is characterized by hearing impairment, ocular changes and progressive glomerulonephritis leading to chronic renal failure. In spite of the fact that it is believed that is primarily affecting men, twice as many women seem to be affected. AIM OF STUDY To assess pregnancy outcome in women with nephrotic syndrome, where AS was discovered post-delivery, and the impact of pregnancy on disease progression. MATERIALS AND METHODS. We present a case of a pregnant patient, with no history of disease, where pregnancy was complicated by nephrotic syndrome (proteinuria = 13 g/24 hours) with brutal onset of arterial hypertension during the 36 week of gestation. The patient was monitored by a multidisciplinary team of gynecologists and nephrologists. Termination of pregnancy was performed in the same week due to uncontrolled secondary hypertension associated with anasarca, induced by nephrotic syndrome. She received methylprednisolone, followed by prednisone 1 mg/ kg/ day after delivery, but 3 months postpartum, the patientВ’s renal function was not fully recovered, proteinuria remained over 1.8 g/24 hours, imposing renal biopsy. The electron micrograph of kidney biopsy demonstrated a thinned glomerular basement membrane and the presence of short stretches of lamellation, suggesting Alport syndrome. Patient was monitored in the next five years after delivery, twice per year, and current proteinuria remains between 0.5-0.8 g/ 24 hours under ARBs treatment, without any immunosuppressive drug. Renal function is normal. CONCLUSION Nephrotic syndrome during the last semester of pregnancy may not cause severe complications, usually considered to be preeclampsia and having good evolution after delivery. Women with Alport's syndrome can develop a kidney disease during pregnancy, with worsening of kidney function, which may not fully recover after delivery. Adequate diagnosis and management are required for nephrotic syndrome in late pregnancy, because it can be associated with high risks for the fetus and the mother, as well.
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