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An infant case of autosomal recessive polycystic kidney disease-associated dilated cardiomyopathy-like hypertensive cardiomyopathy diagnosed because of urinary tract infection

Published online by Cambridge University Press:  13 September 2024

Takato Akiba
Affiliation:
Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
Noboru Tanaka*
Affiliation:
Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
Mayu Nakagawa
Affiliation:
Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
Kotoko Matsui
Affiliation:
Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
Hideo Fukunaga
Affiliation:
Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
Toshiaki Shimizu
Affiliation:
Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
*
Corresponding author: N. Tanaka; Email: [email protected]

Abstract

We report a case of dilated cardiomyopathy-like hypertensive cardiomyopathy (HTN-CM) with polycystic kidney disease without family history when a 3-month-old boy developed bacteraemia secondary to a urinary tract infection. He was later confirmed as having autosomal recessive inheritance due to the proven PKHD1 gene mutation. The treatment consisted mainly of antihypertensive and anti-heart failure therapies and he was discharged on the 131st day. To prevent the development of heart failure in patients with HTN-CM due to autosomal recessive polycystic kidney disease (ARPKD), it is important to improve the fetal diagnosis rate of ARPKD, detect hypertension early, and strictly control the blood pressure after birth.

Type
Brief Report
Copyright
© The Author(s), 2024. Published by Cambridge University Press

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References

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