Publication: A case of alport syndrome with pregnancy-related atypical hemolytic uremic syndrome, andcrescentic glomerulonephritis
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Date
2021-05-01
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BACKGROUND: Thrombotic microangiopathy (TMA) is one of the most important
complications in pregnant patients with chronic kidney disease (CKD) causing clinical
deterioration. However, little is known about the pregnancy course in women with
Alport syndrome (AS).
CASE: A 28-week pregnant, 22-year-old woman was admitted to our clinic because of
widespread edema. Her medical history was notable only for hearing impairment. On
examination, vital signs were normal except for the blood pressure (150/90 mmHg).
There were diffuse crackles at the lung bases, and 3þ pitting edema in both legs. Lab
results revealed heavy proteinuria with 11 gr/day and isomorphic erythrocytes with
granular casts in microscopic urine examination. An emergency c-section was
performed due to severe preeclampsia at 30 weeks’ gestation.
After delivery, her edema did not improve, serum creatinine and lactate dehydrogenase
levels elevated, anemia and thrombocytopenia developed (Table 1). Additional tests
revealed negative Coombs test, schistocytes on peripheral smear and normal
ADAMTS13 level. There was no pathology in serological studies.
She received four sessions of plasmapheresis therapy, and with the diagnosis of aHUS,
eculizumab therapy was started. Despite improving thrombocytopenia and anemia,
serum creatinine levels continued to rise and her urine output decreased. A kidney
biopsy was performed (Figure 1). In the light microscopy, 11 of 15 glomeruli had
circumferential cellular crescents and 4 had partial cellular crescents. The sample had
no findings consistent with TMA. No staining was seen with IgG, IgA, IgM, C3, C1q, j
and k in immunofluorescence.
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BERKE MENTEŞE İ., TUĞCU M., VELİOĞLU A., Nazlı İ., TUĞLULAR Z. S., \"A CASE OF ALPORT SYNDROME WITH PREGNANCY-
RELATED ATYPICAL HEMOLYTIC UREMIC SYNDROME, AND
CRESCENTIC GLOMERULONEPHRITIS\", 56. ERA EDTA CONGRESS, Berlin, Almanya, 5 - 08 Haziran 2021, cilt.36, ss.230-231