Cyanotic Nephropathy in an Adult Patient with Eisenmenger Syndrome: A Case Report and Literature Review

Introduction: Cyanotic nephropathy, a rare disease characterized by proteinuria, decreased estimated glomerular filtration rate, thrombocytopenia, polycythemia, and hyperuricemia, may occasionally be secondary to cyanotic congenital heart disease (CHD). There are currently no detailed diagnostic cri...

Full description

Saved in:
Bibliographic Details
Main Authors: Fanyou Zhu (Author), Rui Wen (Author), Xiangling Tan (Author), Hongjun Nie (Author), Jiali Li (Author), Qi Wang (Author), Jiao Qin (Author)
Format: Book
Published: Karger Publishers, 2024-03-01T00:00:00Z.
Subjects:
Online Access:Connect to this object online.
Tags: Add Tag
No Tags, Be the first to tag this record!

MARC

LEADER 00000 am a22000003u 4500
001 doaj_8e2bde890d8e44f4aa973594debff6f9
042 |a dc 
100 1 0 |a Fanyou Zhu  |e author 
700 1 0 |a Rui Wen  |e author 
700 1 0 |a Xiangling Tan  |e author 
700 1 0 |a Hongjun Nie  |e author 
700 1 0 |a Jiali Li  |e author 
700 1 0 |a Qi Wang  |e author 
700 1 0 |a Jiao Qin  |e author 
245 0 0 |a Cyanotic Nephropathy in an Adult Patient with Eisenmenger Syndrome: A Case Report and Literature Review 
260 |b Karger Publishers,   |c 2024-03-01T00:00:00Z. 
500 |a 1423-0143 
500 |a 10.1159/000538100 
520 |a Introduction: Cyanotic nephropathy, a rare disease characterized by proteinuria, decreased estimated glomerular filtration rate, thrombocytopenia, polycythemia, and hyperuricemia, may occasionally be secondary to cyanotic congenital heart disease (CHD). There are currently no detailed diagnostic criteria or treatments for cyanotic nephropathy, owing to its extremely low incidence. Eisenmenger syndrome (ES) was initially defined by Paul Wood in pathophysiologic terms as "pulmonary hypertension (PH) at the systemic level, caused by a high pulmonary vascular resistance, with a reversed or bidirectional shunt at the aorto-pulmonary, ventricular, or atrial level." It typically develops in the presence of large, unrepaired atrial or ventricular septal defects, arterial shunts, or complex forms of CHD and is the most severe hemodynamic phenotype of pulmonary arterial hypertension associated with CHD. This study aimed to outline the case of an ES patient who developed cyanotic nephropathy and successfully achieved clinical remission through primary disease treatment and symptomatic management. Overall, this case expands our understanding of cyanotic nephropathy and lays a theoretical reference for the treatment of ES. Case Presentation: A 33-year-old Chinese female attended the outpatient department with abnormal urine test results over the past two and a half years. Following a comprehensive medical history collection, she underwent the necessary tests. Cardiac color ultrasound displayed a significant widening of the pulmonary artery and PH (severe), as well as mild tricuspid regurgitation and patent ductus arteriosus. The results of the kidney biopsy, combined with clinical findings, suggested a high risk of polycythemia-related kidney disease. She was eventually diagnosed with cyanotic nephropathy and ES. Her symptoms were relieved following symptomatic treatment, such as the administration of ambrisentan, febuxostat, and home oxygen therapy. Her follow-up visit at 6 months demonstrated improvements in hyperuricemia and a significant increase in physical strength. Conclusion: Cyanotic nephropathy is a rare condition in adults. Kidney biopsy remains the gold standard of diagnosis for various nephropathies. Active treatment of CHD and alleviating hypoxia may be pivotal for the treatment of cyanotic nephropathy. 
546 |a EN 
690 |a cyanotic nephropathy 
690 |a eisenmenger syndrome 
690 |a kidney biopsy 
690 |a polycythemia 
690 |a cyanotic congenital heart disease 
690 |a Dermatology 
690 |a RL1-803 
690 |a Diseases of the circulatory (Cardiovascular) system 
690 |a RC666-701 
690 |a Diseases of the genitourinary system. Urology 
690 |a RC870-923 
655 7 |a article  |2 local 
786 0 |n Kidney & Blood Pressure Research, Vol 49, Iss 1, Pp 211-217 (2024) 
787 0 |n https://beta.karger.com/Article/FullText/538100 
787 0 |n https://doaj.org/toc/1423-0143 
856 4 1 |u https://doaj.org/article/8e2bde890d8e44f4aa973594debff6f9  |z Connect to this object online.