Henny Adriani Puspitasari
1 
, Retno Palupi-Baroto
1,2* , Riski Muhaimin
3 , Mulyadi M Djer
4 , Callen Zulkifli
5 , Sudung Oloan Pardede
1 1 Division of Nephrology, Department of Child Health, Faculty of Medicine Universitas Indonesia – Dr. Cipto Mangunkusumo General Hospital, Jakarta, Indonesia
2 Division of Nephrology, Department of Child Health, Faculty of Medicine, Public Health, and Nursing, Universitas Gadjah Mada, Yogyakarta, Indonesia
3 Division of Hematology-oncology, Department of Child Health, Faculty of Medicine Universitas Indonesia – Dr. Cipto Mangunkusumo General Hospital, Jakarta, Indonesia
4 Division of Cardiology, Department of Child Health, Faculty of Medicine Universitas Indonesia – Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia
5 Faculty of Medicine Universitas Indonesia – Dr. Cipto Mangunkusumo General Hospital, Jakarta, Indonesia
Abstract
Children with cyanotic congenital heart diseases (CCHDs) have several risk factors for the development of several complications, including cyanotic nephropathy (CN). However, due to poor awareness of this problem, CN occasionally progresses to chronic kidney disease (CKD). In the long term, kidney cells subjected to chronic hypoxia undergo further damage, which increases the risk of developing end-stage kidney disease. There are no standardized therapeutic approaches for these cases, especially for children, although phlebotomy is an option. Our case demonstrates a 12-year-old boy with an inoperable, complex CCHD and persistent proteinuria with decreased kidney function. After receiving consecutive treatment with an angiotensin-converting enzyme inhibitor and angiotensin II receptor blocker, the proteinuria did not resolve, and the kidney function deteriorated. Phlebotomy was performed to treat the proteinuria, and the kidney function improved. Within the three months following the first phlebotomy procedure, the patient was less cyanotic and had improved hemodynamic stability. Proteinuria reduction and improvement of estimated glomerular filtration rate were noted; however, the proteinuria gradually worsened after three months. In conclusion, phlebotomy might delay CKD progression in children with CN due to inoperable, complex CCHD. The positive effects in our case were temporary; therefore, repeated therapeutic phlebotomies should be considered.
Implication for health policy/practice/research/medical education:
Children with cyanotic congenital heart diseases are at risk of developing cyanotic nephropathy; thus, serious problems such as end-stage kidney disease could occur. Phlebotomy, as an alternative treatment should be considered as a therapeutic option in severe non-operable children with proteinuria.
Please cite this paper as: Adriani Puspitasari H, Palupi-Baroto R, Muhaimin R, Djer MM, Zulkifli C, Oloan Pardede. Phlebotomy improves kidney function in pediatric cyanotic nephropathy: a case report. J Renal Inj Prev. 2024; x(x): e32286. doi: 10.34172/jrip.2024.32286.