ESPN 53rd Annual Meeting

ESPN 2021


 
Effects of RAAS inhibition and immunosuppressive therapy in pediatric patients with X-linked Alport Syndrome
GULSAH OZDEMIR 1 BORA GULHAN 1 EDA DIDEM KURT SUKUR 1 EMINE ATAYAR 2 ISMAIL DURSUN 3 ZEYNEP BIRSIN OZCAKAR 4 SEHA SAYGILI 5 ALPER SOYLU 6 OGUZ SOYLEMEZOGLU 7 ALEV YILMAZ 8 AYSUN KARABAY BAYAZIT 9 FEHIME KARA EROGLU 10 BELDE KASAP DEMIR 11 SELCUK YUKSEL 12 YILMAZ TABEL 13 AYSE AGBAS 14 ALI DUZOVA 1 MUTLU HAYRAN 15 FATIH OZALTIN 1 REZAN TOPALOGLU 1

1- DIVISION OF PEDIATRIC NEPHROLOGY, HACETTEPE UNIVERSITY FACULTY OF MEDICINE, ANKARA, TURKEY
2- DIVISION OF PEDIATRIC NEPHROLOGY, NEPHROGENETICS LABORATORY, HACETTEPE UNIVERSITY FACULTY OF MEDICINE, ANKARA, TURKEY
3- DIVISION OF PEDIATRIC NEPHROLOGY, ERCIYES UNIVERSITY FACULTY OF MEDICINE, KAYSERI, TURKEY
4- DIVISION OF PEDIATRIC NEPHROLOGY, ANKARA UNIVERSITY FACULTY OF MEDICINE, ANKARA, TURKEY
5- DIVISION OF PEDIATRIC NEPHROLOGY, ISTANBUL UNIVERSITY CERRAHPAŞA FACULTY OF MEDICINE, ISTANBUL, TURKEY
6- DIVISION OF PEDIATRIC NEPHROLOGY, DOKUZ EYLÜL UNIVERSITY FACULTY OF MEDICINE, IZMIR, TURKEY
7- DIVISION OF PEDIATRIC NEPHROLOGY, GAZI UNIVERSITY FACULTY OF MEDICINE, ANKARA, TURKEY
8- DIVISION OF PEDIATRIC NEPHROLOGY, ISTANBUL UNIVERSITY ÇAPA FACULTY OF MEDICINE, ISTANBUL, TURKEY
9- DIVISION OF PEDIATRIC NEPHROLOGY, ÇUKUROVA UNIVERSITY FACULTY OF MEDICINE, ADANA, TURKEY
10- DIVISION OF PEDIATRIC NEPHROLOGY, DR. SAMI ULUS MATERNITY AND CHILDRENS HEALTH HOSPITAL, ANKARA, TURKEY
11- DIVISION OF PEDIATRIC NEPHROLOGY, IZMIR KATIP ÇELEBI UNIVERSITY, TEPECIK RESEARCH AND TRAINING HOSPITAL, IZMIR, TURKEY
12- DIVISION OF PEDIATRIC NEPHROLOGY, PAMUKKALE UNIVERSITY FACULTY OF MEDICINE, DENIZLI, TURKEY
13- DIVISION OF PEDIATRIC NEPHROLOGY, İNÖNÜ UNIVERSITY FACULTY OF MEDICINE, MALATYA, TURKEY
14- DIVISION OF PEDIATRIC NEPHROLOGY, HASEKI TRAINING AND RESEARCH HOSPITAL, ISTANBUL, TURKEY
15- DEPARTMENT OF PREVENTIVE ONCOLOGY, HACETTEPE UNIVERSITY FACULTY OF MEDICINE, ANKARA, TURKEY
 
Introduction:

 

Alport syndrome (AS) is a hereditary nephritis characterized by progressive renal failure. There is increasing evidence that renin-angiotensin-aldosterone system (RAAS) inhibition slows progression to chronic kidney disease (CKD) in AS patients. On the other hand, the effectiveness of immunosuppressive (IS) therapy and the number of studies is limited.  This study aimed to analyze the outcomes of RAAS inhibitors and IS therapy in pediatric patients with X-linked AS (XLAS).

Material and methods:

 

A total of 74 pediatric XLAS patients were included in this multicenter study. Data from medical records were collected retrospectively, other centers were requested to fill out a questionnaire which includes data regarding patient demographic features, clinical and laboratory characteristics, treatment, and follow-up results.

Results:

 

A total of 74 pediatric patients with XLAS (41 male, 33 female) were studied. The median age at first presentation was 6.0 years (interquartile range (IQR) 3.4 – 9.9). The median follow-up duration was 4.0 years (IQR 1.7–7.3). Fifty-two (70.2%) patients received only RAAS inhibitors, 11 (14.9%) patients received RAAS inhibitors and IS, and 11 (14.9%) patients were followed up without treatment. During follow up, glomerular filtration rate (GFR) decreased below 60 ml/min/1.73 m2 in 7 (9.5%) of 74 patients (1 female, 6 male) at the median 16.2 years (IQR 15.2 – 16.6). The patients who were followed up without treatment did not progress to CKD, however, the follow-up period was significantly shorter than other groups (p=0.02). The median age at onset of RAAS inhibitor therapy were significantly higher in patients who progressed to CKD than those who did not (12.3 vs 8.7 years, p=0.007). There was no difference in time to progression to CKD and GFR loss between patients in RAAS and RAAS+IS group (p=0.40 and p=0.87).

Conclusions:

 

Our study showed that early initiation of RAAS inhibitors in patients with XLAS may delay progression to CKD. IS treatment did not improve renal survival in XLAS patients.