Evaluation of Functional MR-Urography in Complex Obstructive Uropathy of Infants: Comparison to the Conventional Diagnostic Algorithm - A Pilot Study

Furth, Christian; Genseke, Philipp; Amthauer, Holger; Neumann, Grit; Krause, H.; Seidensticker, Max; Wiemann, Dagobert; Rißmann, Anke; Liehr, Uwe-Bernd; Ruf, Juri

doi:10.1055/s-0032-1316295

Cited by 4 publications

(3 citation statements)

References 18 publications

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“…Apart from rare case reports, no reliable data exist about the duration of polyuria necessary to induce hydronephrosis or about reversibility of structural changes. New methods like functional MR-urography might improve the follow-up of these patients (11). Furthermore, our case demonstrates that MRI is not always helpful in the diagnosis of DI.…”

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confidence: 86%

Dilatative Uropathy as a Manifestation of Neurohypophyseal Diabetes Insipidus due to a Novel Mutation in the Arginine Vasopressin-Neurophysin-II Gene

Lindenthal

Mainberger²,

Morris-Rosendahl

et al. 2013

Klin Padiatr

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Polydypsia and polyuria are frequent symptoms in patients with sellar masses caused by neurohypophyseal diabetes insipidus. Autosomal dominant familial neurohypophyseal diabetes insipidus (adFNDI), a disorder caused by mutations in the arginine vasopressin (AVP) -neurophysin II (NPII) gene, should be considered as a rare differential diagnosis. A delayed diagnosis bears the risk of life-threatening electrolyte imbalances and permanent urinary tract damage, leading to impaired quality of life.We present a Caucasian kindred of at least 4 generations with FNDI.Clinical histories, endocrine para-meters, and results of molecular analyses of the AVP gene are presented with a review of the literature on diabetes insipidus (DI) related urinary tract dilatation.Polyuria and polydipsia were only reported based on explicit and thorough interrogation after more than 4 years of clinical follow-up. A novel heterozygous mutation in the AVP gene was found in all examined symptomatic subjects (c.1-33_c.4del37nt). A literature review revealed that non-obstructive hydronephrosis (NOH) is a rare but known complication of DI.Since increased fluid intake is often a typical familial pattern in adFNDI, it is frequently missed as being pathologic in affected patients, therefore a detailed clinical history of drinking volumes is of critical importance. AVP gene testing is an important component in the confirmation of the diagnosis. Otherwise unexplainable NOH should lead to further investigations and evaluation of rare diseases like FNDI.

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confidence: 86%

Dilatative Uropathy as a Manifestation of Neurohypophyseal Diabetes Insipidus due to a Novel Mutation in the Arginine Vasopressin-Neurophysin-II Gene

Lindenthal

Mainberger²,

Morris-Rosendahl

et al. 2013

Klin Padiatr

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“…In the last years, magnetic resonance urography (MRU) has been considered as potential alternative to DRS for assessing OU. It features superior morphological delineation to identify urinary tract abnormalities (2,7,23). In addition, functional analyses (fMRU) can be performed to address the two major landmarks which guide therapeutic decisions including 1. identification of a relevant and fixed obstruction grade O'Reilly II (20) and 2. assessment of DRF to prove significant decrease in renal drainage.…”

Section: Zusammenfassungmentioning

confidence: 99%

“…Data was acquired with a 1.5 Tesla MR device (Achieva®, Philips, Best, The Netherlands) using a standardized protocol (7). Injection of gadoterate meglumine (Dotarem®, 0.1 mmol / kg body weight, Guerbet, Villepinte, France) was followed by a THRIVE sequence for functional assessment:…”

Section: Dynamic Contrast-enhanced Mrumentioning

confidence: 99%

Detection of obstructive uropathy and assessment of differential renal function using two functional magnetic resonance urography tools

Genseke¹,

Rogasch²,

Steffen³

et al. 2017

Nuklearmedizin

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fMRU accurately excluded fixed OU in infants and children, independent from the software used for quantification. However, assessment of DRF with fMRU deviated from DRS especially in infants who may profit most from early intervention. Thus, fMRU cannot fully replace DRS as primary functional examination. If, for clinical reasons, fMRU is performed in first place and it cannot exclude fixed OU, it should be followed by DRS for validation and DRF quantification.

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Magnetresonanzurographie in der Kinderurologie

Schindele

Furth

Liehr

et al. 2012

Urologe

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Magnetic resonance urography (MRU) provides high resolution imaging of the urogenital system and the use of paramagnetic contrast agents enables a functional depiction. This review summarizes existing data concerning this diagnostic procedure in pediatric urology. A systematic search and assessment of the literature was performed.A total of 12 studies were reviewed in detail. In mostly small study populations a great heterogeneity concerning methodology, use of comparative examinations and standards of reference was noted. Besides the quality of anatomical imaging, the functional study of renal excretory function and differential renal function was also assessed. Only a few studies performed statistical analyses.The authors' rating of MRU was mostly positive. Due to methodical weaknesses, lack of independent standards of reference and statistical analyses the overall level of evidence was low. Further high quality studies will be necessary to assess the value of MRU for the diagnostic workup in pediatric urology.

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Evaluation of Functional MR-Urography in Complex Obstructive Uropathy of Infants: Comparison to the Conventional Diagnostic Algorithm - A Pilot Study

Abstract: fMRU has potential to improve therapeutic management of OU in infants. If surgical treatment is advised, the morphological information by fMRU has to be emphasized. Regarding kidney function estimation preliminary results are encouraging.

Cited by 4 publications

References 18 publications

Dilatative Uropathy as a Manifestation of Neurohypophyseal Diabetes Insipidus due to a Novel Mutation in the Arginine Vasopressin-Neurophysin-II Gene

Dilatative Uropathy as a Manifestation of Neurohypophyseal Diabetes Insipidus due to a Novel Mutation in the Arginine Vasopressin-Neurophysin-II Gene

Detection of obstructive uropathy and assessment of differential renal function using two functional magnetic resonance urography tools

Magnetresonanzurographie in der Kinderurologie

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