Nieren und ableitende Harnwege

Weitzel, D.

doi:10.1007/978-3-642-59139-6_12

Cited by 3 publications

(2 citation statements)

References 75 publications

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“…All PKHD1 variants were classified according to criteria of the American College of Medical Genetics (ACMG) 34 . Sonography-based kidney volumes were calculated according to the ellipsoid formula (length × width × depth × π/6) and standardized to standard deviation scores (SDS) to pediatric normal values 35 , 36 .…”

Section: Methodsmentioning

confidence: 99%

Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD)

Burgmaier

Ariceta

Bald

et al. 2020

Sci Rep

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To test the association between bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD) and long-term clinical outcome and to identify risk factors for severe outcomes, a dataset comprising 504 patients from the international registry study ARegPKD was analyzed for characteristics and complications of patients with very early (≤ 3 months; VEBNE) and early (4–15 months; EBNE) bilateral nephrectomies. Patients with very early dialysis (VED, onset ≤ 3 months) without bilateral nephrectomies and patients with total kidney volumes (TKV) comparable to VEBNE infants served as additional control groups. We identified 19 children with VEBNE, 9 with EBNE, 12 with VED and 11 in the TKV control group. VEBNE patients suffered more frequently from severe neurological complications in comparison to all control patients. Very early bilateral nephrectomies and documentation of severe hypotensive episodes were independent risk factors for severe neurological complications. Bilateral nephrectomies within the first 3 months of life are associated with a risk of severe neurological complications later in life. Our data support a very cautious indication of very early bilateral nephrectomies in ARPKD, especially in patients with residual kidney function, and emphasize the importance of avoiding severe hypotensive episodes in this at-risk cohort.

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Section: Methodsmentioning

confidence: 99%

Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD)

Burgmaier

Ariceta

Bald

et al. 2020

Sci Rep

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“…Results of laboratory testing were considered if performed not more than 12 months prior to visit date, results of sonographic examinations if performed not more than 24 months prior to visit date and if examinations were performed at age ≥ 18.0 years. Sonographic kidney enlargement was defined as bilateral pole-to-pole-length (PTP) > mean + 2SD for pediatric and adult measurements according to normal values for children 25,26 and adults 27 . Sonography-based kidney volumes were calculated according to the ellipsoid formula (length × width × depth × π/6).…”

Section: Methodsmentioning

confidence: 99%

Clinical courses and complications of young adults with Autosomal Recessive Polycystic Kidney Disease (ARPKD)

Burgmaier

Kilian

Bammens

et al. 2019

Sci Rep

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Autosomal recessive polycystic kidney disease (ARPKD) is a severe pediatric hepatorenal disorder with pronounced phenotypic variability. A substantial number of patients with early diagnosis reaches adulthood and some patients are not diagnosed until adulthood. Yet, clinical knowledge about adult ARPKD patients is scarce. Here, we describe forty-nine patients with longitudinal follow-up into young adulthood that were identified in the international ARPKD cohort study ARegPKD. Forty-five patients were evaluated in a cross-sectional analysis at a mean age of 21.4 (±3.3) years describing hepatorenal findings. Renal function of native kidneys was within CKD stages 1 to 3 in more than 50% of the patients. Symptoms of hepatic involvement were frequently detected. Fourteen (31%) patients had undergone kidney transplantation and six patients (13%) had undergone liver transplantation or combined liver and kidney transplantation prior to the visit revealing a wide variability of clinical courses. Hepatorenal involvement and preceding complications in other organs were also evaluated in a time-to-event analysis. In summary, we characterize the broad clinical spectrum of young adult ARPKD patients. Importantly, many patients have a stable renal and hepatic situation in young adulthood. ARPKD should also be considered as a differential diagnosis in young adults with fibrocystic hepatorenal disease.

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Bildgebende Diagnostik der Nieren und Harnwege

Wünsch¹,

Tröger²

2002

Pädiatrische Nephrologie

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Nieren und ableitende Harnwege

Cited by 3 publications

References 75 publications

Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD)

Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD)

Clinical courses and complications of young adults with Autosomal Recessive Polycystic Kidney Disease (ARPKD)

Bildgebende Diagnostik der Nieren und Harnwege

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