Autosomal Dominant Polycystic Kidney Disease with Emphysematous Polycystic Renal Infection That Required Surgical Treatment

Watanabe, Kentaro; Fujii, Teruhiro; Makabe, Shiho; Nakajima, Haruno; Sato, Masayo; Kataoka, Hiroshi; Tsuchiya, Ken; Nitta, Kosaku; Mochizuki, Toshio

doi:10.2169/internalmedicine.1257-18

Cited by 3 publications

(2 citation statements)

References 19 publications

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“…Perinephric abscess requires prolonged intravenous antibiotic therapy and drainage should be considered. Nephrectomy is rarely needed but may be considered in cases of perinephric abscess or emphysematous pyelonephritis that are resistant to drainage and/or antibiotic therapy 218 .…”

Section: Managementmentioning

confidence: 99%

Polycystic kidney disease

Bergmann

Guay‐Woodford

Harris

et al. 2018

Nat Rev Dis Primers

523

509

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Cystic kidneys are common causes of end-stage renal disease, both in children and in adults. Autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) are cilia-related disorders and the two main forms of monogenic cystic kidney diseases. ADPKD is a common disease that mostly presents in adults, whereas ARPKD is a rarer and often more severe form of polycystic kidney disease (PKD) that usually presents perinatally or in early childhood. Cell biological and clinical research approaches have expanded our knowledge of the pathogenesis of ADPKD and ARPKD and revealed some mechanistic overlap between them. A reduced ‘dosage’ of PKD proteins is thought to disturb cell homeostasis and converging signalling pathways, such as Ca2+, cAMP, mechanistic target of rapamycin, WNT, vascular endothelial growth factor and Hippo signalling, and could explain the more severe clinical course in some patients with PKD. Genetic diagnosis might benefit families and improve the clinical management of patients, which might be enhanced even further with emerging therapeutic options. However, many important questions about the pathogenesis of PKD remain. In this Primer, we provide an overview of the current knowledge of PKD and its treatment.

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Section: Managementmentioning

confidence: 99%

Polycystic kidney disease

Bergmann

Guay‐Woodford

Harris

et al. 2018

Nat Rev Dis Primers

523

509

View full text Add to dashboard Cite

show abstract

“…Common organisms causing EPN are Escherichia coli, Klebsiella pneumonia and Proteus mirabilis. 2 EPN carries a poor prognosis with high mortality, unless prompt antimicrobial therapy is followed by nephrectomy. Both open and laparoscopic nephrectomy may be deemed technically challenging or associated with unacceptably high anaesthetic risks.…”

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confidence: 99%